Textbook of

PAEDIATRIC
EMERGENCY
MEDICINE
Commissioning Editor: Timothy Horne/Jeremy Bowes
Development Editor: Helen Leng
Project Manager: Sukanthi Sukumar
Designer: Kirsteen Wright
Illustration Manager: Gillian Richards
Illustrator: Cactus
Textbook of
PAEDIATRIC
EMERGENCY
MEDICINE
Second edition
EDITED BY

Peter Cameron MBBS MD FACEM

Professor of Emergency Medicine, The Alfred Hospital and Monash University, Melbourne, Australia

George Jelinek MD MBBS Dip DHM FACEM

Director, Emergency Practice Innovation Centre, St Vincents Hospital; Professorial Fellow, The University
of Melbourne, Melbourne, Australia

Ian Everitt MBBS Dip Anat FRACP FACEM

Paediatric Emergency Physician, Princess Margaret Hospital for Children; General Paediatrician, Fremantle Hospital,
Perth; General Paediatrician, Emergency Physician, Kimberley Health Service, Broome, Derby, Fitzroy Crossing,
Australia

Gary Browne MD MBBS MSpMed FRACP FACEM

Professor of Emergency Medicine, The Childrens Hospital at Westmead; Chair of Discipline of Emergency Medicine,
The University of Sydney; Head of Academic Emergency Medicine, The Childrens Hospital at Westmead, Sydney,
Australia

Jeremy Raftos MBBS FRACP

Director of Paediatric Emergency Medicine, Womens and Childrens Hospital, Children, Youth and Womens Health
Service, Adelaide, Australia

Edinburgh London New York Oxford Philadelphia St Louis Sydney Toronto 2012
# 2012 Elsevier Ltd. All rights reserved.

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This book and the individual contributions contained in it are protected under copyright by the publisher
(other than as may be noted herein).

First edition 2006

Second edition 2012

ISBN 9780702033681

British Library Cataloguing in Publication Data

A catalogue record for this book is available from the British Library

Library of Congress Cataloging in Publication Data

A catalog record for this book is available from the Library of Congress

Notices
Knowledge and best practice in this field are constantly changing. As new research and experience broaden our
understanding, changes in research methods, professional practices, or medical treatment may become necessary.

Practitioners and researchers must always rely on their own experience and knowledge in evaluating and using
any information, methods, compounds, or experiments described herein. In using such information or methods
they should be mindful of their own safety and the safety of others, including parties for whom they have a
professional responsibility.

With respect to any drug or pharmaceutical products identified, readers are advised to check the most current
information provided (i) on procedures featured or (ii) by the manufacturer of each product to be administered, to
verify the recommended dose or formula, the method and duration of administration, and contraindications. It is
the responsibility of practitioners, relying on their own experience and knowledge of their patients, to make
diagnoses, to determine dosages and the best treatment for each individual patient, and to take all appropriate
safety precautions.

To the fullest extent of the law, neither the publisher nor the authors, contributors, or editors, assume any liability
for any injury and/or damage to persons or property as a matter of products liability, negligence or otherwise, or
from any use or operation of any methods, products, instructions, or ideas contained in the material herein.

Printed in China
 Preface to
the second
edition

Following the successful launch of the first management protocols where appropriate. further consolidate the convergence of clini-
edition as a companion to the Adult Text- Material has been reviewed by chapter cal knowledge and practice internationally.
book of Emergency Medicine, we have had authors and editors to ensure that it is con- This edition was developed over approxi-
considerable feedback on the content and sistent with best practice internationally. mately 18 months with contributions from
layout from doctors, nurses and paramedics The structure has remained the same to authors around the world including Australia,
working in paediatric emergency practice enable easy access for readers. New Zealand, United Kingdom, Hong Kong,
from around the world. The feedback has Since the first edition, there has been con- and the United States. The commitment
been positive, particularly regarding the solidation of paediatric emergency medicine and effort required to coordinate and cajole
importance of a text with a standardised, eas- as a specialised domain of clinical expertise. the many people involved, required dedica-
ily accessible format. Despite major advances Standards for paediatric patient care in tion from all involved but we would particu-
in computerisation, most clinicians studying emergency departments have been pub- larly like to thank Helen Leng from Elsevier
detailed clinical material, still prefer a well- lished in the United Kingdom and elsewhere who remained focused throughout.
presented book with carefully edited text and training programmes have been devel-
to on-line material. It is likely that further oped in many countries. There is a high P. C. 2012
advances in technology will enable electronic degree of cooperation within the interna- G. J.
versions of this text shortly and it is intended tional emergency paediatric community I. E.
for this to occur after the printed version has and international networks for research G. B.
been released. (e.g. PERN Paediatric Emergency Research J. R.
In this edition we have reviewed each Network) and other activities are being con-
chapter and updated guidelines and sidered. Hopefully texts such as this can

v
Preface to the
first edition

For emergency physicians working in com- basic approach to the initial care of children South-east Asia and the United Kingdom. It
munity hospitals, paediatric presentations with common emergencies whilst giving is hoped that a simple straightforward
are seen as the most challenging. There summary information on more complicated approach to common emergencies will give
are very few critically ill children. However, chronic presentations that may present to the reader a method for managing likely
the possibility of missing an early presenta- community hospitals as these conditions emergency presentations. There are fre-
tion of a potentially life-threatening illness are increasingly treated outside the special- quently many different approaches to man-
is always present. Children are not always ist centres. The contributors to the book aging common medical conditions. Where
capable of communicating their problems have been deliberately selected from both possible, we have tried to take an evidence-
verbally and the parents are inevitably specialist paediatric and emergency back- based approach. However, where limited evi-
anxious. There are numerous anecdotes of grounds, to ensure a broad perspective. dence is available we have reverted to con-
missed diagnoses resulting in catastrophic In Australasia, the United Kingdom, sensus amongst the experts in that area. As
outcomes. With experience, emergency prac- South-east Asia and many other regions, in most areas of medicine, most of our patient
titioners develop systematic approaches to paediatric emergency medicine has fallen care is dependent on consensus rather than
overcome these problems. Unfortunately, between specialist training groups with evidence.
this is often too late for trainees and clini- expertise from specialist paediatricians as For a textbook of this size, an incredible
cians rotating through low-paediatric-volume well as emergency physicians. At times, this amount of work is necessary to ensure that
departments. The reality is that most emer- relationship has been strained with differing content is correct and that the project finally
gency physicians, even when working in a approaches to the management of emer- comes together within a reasonable time
specialised paediatric centre, will have limited gency presentations and training of person- frame. The individual contributors and pub-
exposure to critically ill children. Regular in- nel working in paediatric departments. This lication staff at Elsevier should be congratu-
hospital training, courses such as the is now being improved with joint training lated. We would also like to thank Ms Mimi
Advanced Paediatric Life Support (APLS) programmes and a more collaborative Morgan for her considerable patience in
course, and access to simple reference mate- approach. Hopefully this book will help coordinating this project.
rial are all essential in overcoming this bridge some of the differences between
problem. the specialist groups. P. C. 2006
The purpose of this book is to provide a This text should be seen as a companion to G. J.
reference text for medical, nursing and para- the Textbook of Adult Emergency Medicine, I. E.
medic staff involved in the provision of pae- the second edition of which has been pub- G. B.
diatric emergency care. The book provides a lished and widely distributed in Australasia, J. R.

vi
 Contributors

Jason Acworth MBBS FRACP Tom Beattie MB MSc FRCSE FRCPE Gervase Chaney MBBS FRACP
Director, Paediatric Emergency Medicine, FFSEM(RSPI) DCH Paediatrician and Director, Postgraduate
Childrens Health Services, Queensland; Consultant in Paediatric Emergency Medical Education, Princess Margaret
Senior Lecturer, Paediatrics and Child Medicine, Emergency Department, Royal Hospital for Children, Perth, Australia
Health, The University of Queensland, Hospital for Sick Children, Edinburgh, UK
Nicholas Cheng MBBS BSc(Med) DCH FRACP
Brisbane, Australia
Andrew Berry AM FRACP Paediatric Emergency Physician, Emergency
Navid Adib MBBS FRACP PhD State Director, NETS (Newborn and Department, The Childrens Hospital at
Paediatric Rheumatologist, Queensland paediatric Emergency Transport Service), Westmead, Sydney, Australia
Paediatric Rheumatology Services, Brisbane, NSW, Australia Raymond Chin MBBS DCH FRACP
Australia
Meredith Borland MBBS FRACGP FACEM Clinical Associate Lecturer, Paediatrics and
Philip Aplin MBBS FACEM Deputy Director and Paediatric Emergency Child Health, The Childrens Hospital at
Emergency Medicine, Flinders Medical Physician, Emergency Department, Princess Westmead and The University of Sydney,
Centre, Adelaide, Australia Margaret Hospital for Children, Perth; Sydney, Australia

Franz E Babl MD MPH FRACP FAAP FACEP

Joint Clinical Senior Lecturer, School of Robin Choong MBBS FRACP FCICM
Paediatrics and Child Health and School of Senior Staff Specialist, Paediatric Intensive
Paediatric Emergency Physician, Department
Primary, Aboriginal and Rural Health Care, Care Unit, The Childrens Hospital at
of Emergency Medicine, Royal Childrens
University of Western Australia, Australia Westmead, Sydney, Australia
Hospital; Associate Professor, Department of
Paediatrics, The University of Melbourne; Robyn Brady MBBS DCH MRCPI FRACP FACEM Simon Chu MBBS DCH FACEM MMEd
Research Fellow, Murdoch Childrens Staff Specialist Paediatric Emergency General and Paediatric Emergency
Research Institute, Melbourne, Australia Physician; Director of Emergency Medicine Physician, Emergency Department, Lyell
Training, Paediatric Emergency Department, McEwin Health Service, Adelaide, Australia
Roger Barkin MD MPH FAAP FACEP
Mater Childrens Hospital, Brisbane, Australia
Emergency Medicine Physician, Aurora, Pat Clements BSc BSocWk
Colorado, USA Drago Bratkovic MBBS FRACP HGSA Social Worker, Department of Emergency
Unit Head, Metabolic Clinic, SA Pathology, Medicine and Paediatric Intensive Care
Peter L J Barnett MBBS FRACP MSc FACEM
Adelaide, Australia Unit, Royal Childrens Hospital, Brisbane,
MSpMed
Australia
Deputy Director, Department of Emergency Lindsay Bridgford CSM MBBS DCH
Medicine, Royal Childrens Hospital; DRANZCOG FACEM Jane Cocks MBBS FRACP FACEM FCICM
Clinical Associate Professor, Department of Director of Emergency Medicine Training, Clinical Director, Neonatal and Paediatric
Paediatrics, The University of Melbourne; Maroondah Hospital, Davey Drive, Australia Retrievals, MedSTAR Emergency Medical
Honorary Research Fellow, Emergency Retrieval, SA Health; Paediatric Emergency
Research, Murdoch Childrens Research Andrew Bullock MBBS FRACP Physician, Paediatric Emergency
Institute, Melbourne, Australia Childrens Cardiac Centre, Princess Margaret Department, The Womens and Childrens
Hospital for Children, Perth, Australia Hospital, Adelaide, Australia
Katherine Barton MBBS
Paediatric Emergency Senior Registrar, Danny Cass MBBS David G Cooksley MBChB FACEM DIMC RCSE
Department of Emergency Medicine, Head of Academic Surgery and Director of DRACOG ACCAM PGCertAME
Princess Margaret Hospital for Children, Trauma, The Childrens Hospital at Director, Trauma Service, The Townsville
Perth, Australia Westmead, Sydney, Australia Hospital, Townsville, Australia

vii
CONTRIBUTORS

Elizabeth M Cotterell MBBS DipPaed Linda Durojaiye MBBS BMedSci MRCP FRACP Joanne Grindlay MBBS DA FRACGP FARGP
FRACP MPH Paediatric Emergency Physician, Emergency FACEM EMDM
Associate Professor, Paediatrics, School of Department, Sydney Childrens Hospital, Emergency Physician, Emergency
Rural Medicine, The University of New Sydney, Australia Department, Royal Childrens Hospital;
England, Armidale, Australia Murdoch Childrens Research Institute,
Daryl Efron MBBS FRACP MD Melbourne, Australia
Lisa Coutts BNurs Paediatrician, Royal Childrens Hospital;
Clinical Nurse, Department of Cardiology, Senior Lecturer, Department of Paediatrics, Sonia Grover MBBS FRANZCOG MD
Hollywood Private Hospital, Nedlands; The University of Melbourne; Director, Department of Paediatric and
Emergency Nurse, Emergency Department, Honorary Research Fellow, Murdoch Adolescent Gynaecology and Associate
Joondalup Health Campus, Joondalup, Childrens Research Institute, Melbourne, Professor, Department of Paediatrics, Royal
Perth, WA Australia Childrens Hospital, The University of
Melbourne;
Paul Craven BSc MBBS MRCP FRACP
Ian Everitt MBBS FACEM FRACP Consultant Gynaecologist, Department of
Deputy Director and Senior Staff Specialist, Obstetrics and Gynaecology, Mercy
Paediatric Emergency Physician, Princess
Neonatal Intensive Care Unit, John Hunter Hospital for Women, Melbourne,
Margaret Hospital for Children;
Childrens Hospital, Newcastle, Australia Australia
General Paediatrician, Fremantle Hospital,
Maree Crawford MBBS FRACP Perth; General Paediatrician, Emergency
Naren Gunja MBBS FACEM
Paediatrician and Senior Staff Specialist, Physician, Kimberley Health Service,
Medical Director, NSW Poisons
Child Advocacy Service, Royal Childrens Broome, Derby, Fitzroy Crossing,
Information Centre; Clinical
Hospital, Brisbane, Australia Australia
Toxicologist, The Childrens Hospital at
Nigel W Crawford MBBS MPH Tom Everitt MBBS FRACGP Westmead;
Clinical Research Fellow, Murdoch Childrens General Practitioner, Casey Medical Centre, Clinical Senior Lecturer, Discipline of
Research Institute, Royal Childrens Hospital, Melbourne, Australia Emergency Medicine, Sydney Medical
Melbourne, Australia School, Sydney, Australia
Michael Fairley MBBS FRANZCP
Sarah Dalton BMed FRACP MAppMgtHlth Anthony Harrington MBBS FRACGP
Child and Adolescent Mental Health, Prince
DARCS FACEM
Paediatric Emergency Physician, of Wales and Sydney Childrens Hospitals,
Emergency Department, The Childrens Senior Consultant, Department of
Sydney, Australia
Hospital at Westmead; Retrieval Consultant, Emergency Medicine, Nambour
General Hospital, Nambour,
NSW Newborn and Paediatric Emergency Bruce Fasher MBBS DRCOG DCH FRCP FRACP
Transport Service, Sydney, Australia Australia
Staff Physician, Royal Alexandra Hospital for
Children, Sydney, Australia Andrew Harris MBBS FRACGP
Andrew J Davidson MBBS MD FANZCA
DipSportsMed ACCAM
Senior Anaesthetist, Royal Childrens Toby Fogg BM MRCS FACEM FCEM General Practitioner, Sandringham
Hospital; Associate Professor, Department of Staff Specialist, Emergency Department, Medical Centre, Melbourne,
Paediatrics, The University of Melbourne; Royal North Shore Hospital, Sydney; Australia
Director of Clinical Research, Murdoch Retrieval Specialist, CareFlight, Sydney,
Childrens Research Institute, Melbourne, Australia Wayne Hazell MBBS DipObs FACEM
Australia Emergency Physician, Head of Emergency
Peter Francis MBBS FRACP Medicine Education and Research,
Conor Deasy FCEM FACEM DIMC DCH DipTox
Paediatrician, Frankston Hospital; Middlemore Hospital, Auckland,
MB BAO BCH BMedSc
Paediatric Nuclear Medicine Physician, New Zealand
Emergency Physician, Emergency and
Royal Childrens Hospital, Melbourne,
Trauma Centre, Alfred Hospital; Robert Henning FRCA FJFICM
Australia
Research Scholar, Department of Epidemiology Staff Specialist in Intensive Care, Royal
and Preventive Medicine, Melbourne, Australia Childrens Hospital, Melbourne,
Gary Geelhoed MBBS FRACP FACEM MD
Australia
Ronald A Dieckmann MD MPH FAAP FACEP Clinical Professor, Director, Emergency
Professor Emeritus of Emergency Medicine Department, Princess Margaret Hospital for Malcolm Higgins BMBS FRACP
and Pediatrics, University of California, Children, Perth, Australia Staff Specialist, Paediatric Emergency
San Francisco Department, Womens and Childrens
Padraic Grattan-Smith MBBS MRCP FRACP
Hospital, Adelaide, Australia
Evelyn Doyle MB Bch BAO MRCPCH FRACP Honorary Consultant in Movement
Paediatric Emergency Physician, Emergency Disorders, Department of Neurology, The Andrew J A Holland BSc (Hons) MBBS PhD
Department, Children, Youth and Womens Childrens Hospital at Westmead, Sydney, Grad Cert Ed Studies (Higher Ed) FRCS (Eng)
Health Service, Adelaide, Australia Australia FRACS (Paed) FACS

viii
CONTRIBUTORS

Professor of Paediatric Surgery, The Kevin Mackway-Jones FRCP FRCS FCEM Westmead; Department of Psychiatry,
Childrens Hospital at Westmead, The Professor of Emergency Medicine, Faculty of Medicine, University of New South
University of Sydney, Australia Royal Manchester Childrens Hospital Wales, Sydney, Australia
and Manchester Royal Infirmary,
Jason Hort MBBS MCRP FRACP Matthew OMeara MBBS FRACP
Manchester, UK
Senior Staff Specialist, Emergency Director of Emergency Medicine, Sydney
Department, The Childrens Hospital at Elly Marillier MBBS DCH FACEM Childrens Hospital;
Westmead, Sydney, Australia Emergency Physician, Emergency Conjoint Senior Lecturer, School of Medicine,
Department, Joondalup Health Campus; The University of New South Wales, Sydney,
Andrew Jan MBBS FACEM BA FAMAC MPhil
Clinical Senior Lecturer, The University Australia
Director of Emergency Medicine,
of Western Australia, Perth,
St John of God Hospital Murdoch,
Australia Ed Oakley MBBS
Perth, Australia
Director of Paediatric Emergency Medicine,
Jennie Martin MBBS DipCH FACEM
Andrew Stewart Kemp MBBS PhD FRACP Southern Health, Monash Medical Centre,
Senior Staff Specialist, Department of
Professor of Allergy and Clinical Melbourne, Australia
Emergency Medicine, Royal North Shore
Immunology, The Childrens Hospital at
Hospital, Sydney, Australia
Westmead, Sydney, Australia Kim Lian Ong MBBS FRCS FHKCEM FHKAM
Karen McCarthy MBBch BAO FRACP FAMS
Colin S Kikiros MBBS FRACS Paediatric Emergency Physician, Emergency Consultant, Accident and Emergency, Pok Oi
Consultant Paediatric Surgeon, Princess Hospital, Yuen Long, New Territories,
Department, Starship Childrens Hospital,
Margaret Hospital for Children, Perth, Hong Kong
Auckland, New Zealand
Australia
Helen Mead MBBS FRACP FACEM David Orchard MBBS FACD
Barbara King MBBS FRACP
Emergency Consultant, Emergency Paediatric Dermatologist, Department of
Paediatric Emergency Physician,
Department, Princess Margaret Hospital for Dermatology, Royal Childrens Hospital,
Princess Margaret Hospital for Children;
Children; Melbourne, Australia
Clinical Senior Lecturer, School of
Senior Clinical Lecturer, School of Paediatrics
Paediatrics and Child Health, Colin Parker MBChB DCH MRCPCH FACEM
and Child Health, The University of Western
The University of Western Australia,
Australia, Perth, Australia Emergency Physician, Director of Emergency
Perth, Australia
Medicine Training, Joondalup Health Campus;
Alastair D McR Meyer BSc BMedSci MBBS Emergency Physician, Princess Margaret
Judith Klein MD FACEP FACEM
Assistant Professor of Emergency Medicine, Hospital for Children;
Emergency Physician, Southern Health Clinical Senior Lecturer, The University of
San Francisco General Hospital Medical
(Casey Hospital & Monash Medical Centre); Western Australia, Perth, Australia
Center, San Francisco, USA
Director, Emergency Medicine Research,
David Krieser MBBS FRACP Casey Hospital; Jacqueline E L Parkinson BPharm (Hons)
Paediatric Emergency Physician, Sunshine General Practitioner, Melbourne, Senior Clinical Pharmacist, Pharmacy
Hospital; Clinical Senior Lecturer, Australia Department, Southern Health, Melbourne,
Paediatrics, The University of Melbourne, Alistair Murray FACEM FCEM FRCS(A&E) Australia
Melbourne, Australia DipIMC
Consultant in Emergency Medicine, Donald Payne MD FRACP FRCPCH
Richard Lennon MBBS FRACP FACEM CCPU
St Vincents University Hospital, Dublin, Associate Professor, Paediatric and
MBioeth
Ireland Adolescent Medicine, Princess Margaret
Director of Emergency Paediatrics, Royal
Hospital for Children;
North Shore Hospital, Sydney, Australia Yuresh Naidoo MBChB FACEM Associate Professor, School of Paediatrics
Stuart Lewena BMedSci MBBS FRACP Director of Clinical Training, Emergency and Child Health, The University of Western
Paediatric Emergency Physician, Royal Department, Joondalup Health Campus, Australia, Perth, Australia
Childrens Hospital; Perth, Australia
Research Affiliate, Murdoch Childrens Murali Narayanan FRACP DNB DCH Scott Pearson MBChB FACEM
Research Institute, Melbourne, Emergency Physician, Emergency Medicine,
Head of Department, Paediatrics,
Australia Christchurch Hospital, Christchurch,
Kalgoorlie Regional Hospital, Kalgoorlie,
New Zealand
Australia
Michelle Lin MD
Associate Professor of Clinical Emergency Kenneth Nunn MBBS (Hons) FRANZCP Natalie Phillips MBBS MPhil FRACP
Medicine, Department of Emergency FRCPsych PhD Department of Emergency Medicine,
Medicine, UCSF-San Francisco General Senior Consultant Child Psychiatrist, Acuity Royal Childrens Hospital, Brisbane,
Hospital, San Francisco, USA Reduction Team, The Childrens Hospital, Australia

ix
CONTRIBUTORS

Roderic Phillips MBBS FRACP PhD Mike Starr MBBS FRACP Deputy Medical Director, Newborn and
Paediatric Skin Specialist, Royal Childrens Paediatrician, Infectious Diseases Physician, paediatric Emergency Transport
Hospital, Melbourne, Australia Consultant in Emergency Medicine, Director Service (NETS), NSW, Sydney,
of Paediatric Physician Training, Australia
Susan Phin MBBS FRACP Royal Childrens Hospital, Melbourne,
Paediatric Emergency Physician, Julian White MBBS MD
Australia
The Childrens Hospital at Westmead, Professor and Director, Toxinology
Sydney, Australia Greg Stevens BHB MBChB FACEM Department, Womens and Childrens
Head of Emergency Medicine, Taranaki Hospital, Adelaide, Australia
Colin V E Powell MBChB DCH MRCP FRACP District Health Board, New Plymouth,
FRCPCH MD
New Zealand Richard P Widmer BDSc MDSc FRACDS
Senior Lecturer in Child Health, Department Clinical Associate Professor, Paediatrics
of Paediatrics, Cardiff University; Paul Tait MBBS FRACP and Child Health, The Childrens
Consultant Paediatrician, Department of Senior Staff Specialist and Consultant Hospital at Westmead, Sydney,
Paediatrics, University Hospital of Wales, Paediatrician, Child Protection Unit, The Australia
Cardiff, UK Childrens Hospital at Westmead, Sydney,
Stephen Priestley MBBS FACEM Australia Barry Wilkins MD MA BChir
District Director and Senior Staff Specialist in Senior Intensive Care Physician,
James Tibballs BMedSc MBBS MEd MBA MD
Emergency Medicine, Sunshine Coast Health Paediatric Intensive Care, The Childrens
MHlth&MedLaw DALF PGDipArts(Fr) FANZCA
Service District, Nambour Hospital, Hospital at Westmead, Sydney, Australia
FACLM FCICM
Queensland, Australia
Associate Professor, Intensive Care
Michael Ragg MBBS FACEM DipO&G Physician and Resuscitation Officer,
Gary Williams MBBS FRACP FJFICM
GradCertEBP Staff Specialist in Intensive Care,
Royal Childrens Hospital; Associate
Emergency Physician, Emergency Sydney Childrens Hospital, Sydney,
Professor, Australian Venom Research Unit,
Medicine, Barwon Health, Geelong; Australia
Department of Pharmacology,
Associate Clinical Professor, Deakin The University of Melbourne,
University School of Medicine, Melbourne, Melbourne, Australia Frank Willis MBBS DCCH DCH
Australia GradDipHealthAdmin FRACP FRCPCH
James Tilleard MBBS FACEM Consultant, Emergency Department,
Pamela Rosengarten MBBS FACEM Princess Margaret Hospital for Children;
Emergency Physician, Nambour Hospital,
Associate Professor of Emergency Medicine, Clinical Senior Lecturer, School of
QLD, Australia
Southern Clinical School, Paediatrics and Child Health and School of
Faculty of Medicine, Nursing and Joseph Ting MBBS BMedSci MSc(Lond) Medicine, The University of Western
Health Sciences, Monash University; PGDipEpi(LSHTM) FACEM Australia and The University of Notre Dame
Director of Education and Research in Senior Staff Specialist, Department of (Australia);
Emergency Medicine, Peninsula Health, Emergency Medicine, Mater Public Head of Paediatrics, Fremantle Hospital,
Melbourne, Australia Hospitals; Perth, Australia; Locum Consultant, Renal
John Ryan FCEM FRCSEd(A&E) FFSEM DCH Clinical Senior Lecturer, Division of Unit, Royal Hospital for Sick Children,
DipSportsMed Anaesthesiology and Critical Care, Glasgow, UK
Consultant in Emergency Medicine, School of Medicine, The University of
University College Dublin; Associate Clinical Queensland; Retrieval and Clinical Simon Wood MBBS DipPaed FACEM
Professor, St Vincents University Hospital, Coordination Physician, Careflight Director of Emergency Medicine,
Dublin, Ireland Medical Services Queensland, Brisbane, Joondalup Health Campus, Perth, Australia
Australia
Matt Ryan MBBS DRANZCOG MFM FACLM
FFFLM(RCPUK) FACEM John Walsh MBChB MCHOrth FRCS FRACS Simon Young MBBS DipCrim FACEM
Director, Paediatric Orthopaedics Director, Department of Emergency
Emergency Physician, Emergency
Department, Mater Childrens Hospital, Medicine, Royal Childrens Hospital;
Department, Werribee Mercy Hospital,
Brisbane, Australia Clinical Associate Professor,
Werribee, Australia
Department of Paediatrics,
Kam Sinn MBBS FRACP Christopher Webber MBBS DipRACOG The University of Melbourne;
Emergency Paediatrician and Senior FRACP Research Fellow, Murdoch Childrens
Specialist, Emergency Department, The Paediatric Emergency Physician, Emergency Research Institute, Melbourne,
Canberra Hospital, Canberra, Australia Department, Sydney Childrens Hospital; Australia

x
 Contents

SECTION 1 SECTION 3 5.2 Heart failure 94

Robin Choong
Approach to the paediatric Trauma in children 51
patient 1 Editor: Ian Everitt 5.3 Syncope 96
Editor: Ian Everitt Linda Durojaiye, Andrew
3.1 Introduction to paediatric Bullock
1.1 Approach to the paediatric trauma 51 5.4 Cardiovascular assessment and
patient 1 Danny Cass murmurs 99
Ian Everitt, Andrew Jan, Matthew OMeara
3.2 Paediatric neurotrauma 56
Andrew Harris, Tom Everitt,
David G Cooksley 5.5 Congenital heart disease 103
Lisa Coutts
3.3 Thoracic injuries in Matthew OMeara
1.2 The crying infant 14
childhood 64 5.6 Acute rheumatic fever 107
Barbara King
Philip Aplin Sarah Dalton
3.4 Abdominal and pelvic 5.7 Infective endocarditis 110
trauma 69 Sarah Dalton
Scott Pearson
SECTION 2 5.8 Kawasaki disease 112
3.5 Burns 73 Daryl Efron
Resuscitation 16 Peter L J Barnett
Editor: Ian Everitt 5.9 Cardiac arrhythmias 115
Gary Williams
2.1 Paediatric cardiopulmonary
arrest (CPA) 16
SECTION 4
Christopher Webber
Wound management 79
SECTION 6
2.2 Paediatric basic life
Editor: Ian Everitt Respiratory 123
support 20
Editor: Ian Everitt
Jane Cocks 4.1 Wound management 79
2.3 Paediatric advanced life Ed Oakley 6.1 Upper respiratory tract
support (PALS, APLS) 25 infections 123
James Tibballs Peter L J Barnett
2.4 Specific paediatric 6.2 Inhaled foreign body 126
resuscitation 38
SECTION 5 Peter L J Barnett
James Tibballs Cardiovascular 91
6.3 Acute asthma 128
Editor: Ian Everitt
2.5 Shock 41 Colin V E Powell
Kevin Mackway-Jones
5.1 Cyanotic heart disease and 6.4 Pertussis 134
2.6 Neonatal resuscitation 45 tetralogy of Fallot spells 91 Nigel W Crawford,
Gary Williams Robin Choong Colin V E Powell

xi
CONTENTS

6.5 Community-acquired SECTION 8 10.5 Disorders of fluids, electrolytes

pneumonia 136 and acidbase 248
Mike Starr Neurology 195 Barry Wilkins, Wayne Hazell
Editor: Ian Everitt
6.6 Bronchiolitis 139
Tom Beattie 8.1 CSF shunt
6.7 Croup 142 complications 195 SECTION 11
Gary Geelhoed Richard Lennon Haematology and
8.2 Raised intracranial oncology 259
pressure 199 Editor: Jeremy Raftos
Richard Lennon
11.1 The use of blood products in
8.3 Seizures and non-epileptic children 259
SECTION 7 events 203 Joseph Ting
Gastroenterology Padraic Grattan-Smith
11.2 Anaemia 263
and hepatology 146 8.4 Acute weakness 207 Jane Cocks
Editor: Ian Everitt Richard Lennon
11.3 Disorders of coagulation 270
7.1 Abdominal pain 146 8.5 Acute ataxia 215 Evelyn Doyle
Jason Hort Joanne Grindlay
11.4 Platelet disorders 272
7.2 Gastrointestinal 8.6 Headache 220 Jane Cocks
bleeding 152 Alistair Murray, John Ryan
11.5 Vasculitis 274
Elizabeth M Cotterell 8.7 CNS infections: meningitis Evelyn Doyle
7.3 Gastro-oesophageal and encephalitis 226
Mike Starr 11.6 Acute leukaemia 277
reflux 155
Joseph Ting
Daryl Efron
11.7 Febrile neutropenia 279
7.4 Pyloric stenosis 158
Joseph Ting
Kim Lian Ong SECTION 9
7.5 Bilious vomiting 160 Infectious diseases 230
Andrew J A Holland Editor: Gary Browne
SECTION 12
7.6 Ingested foreign
bodies 163 9.1 Infectious diseases 230 Dermatology 281
Scott Pearson Mike Starr Editor: Jeremy Raftos

7.7 Hepatic failure 165 12.1 Dermatology 281

David Krieser Roderic Phillips, David Orchard,
7.8 Diarrhoea and
SECTION 10 Mike Starr
vomiting 172 Endocrine and
Christopher Webber metabolic 238
Editor: Jeremy Raftos SECTION 13
7.9 Hepatitis 176
Franz E Babl Eyes 323
10.1 Metabolic emergencies 238
Editor: Jeremy Raftos
7.10 Intussusception 182 Drago Bratkovic,
Kim Lian Ong, Peter Francis
13.1 Ophthalmological
Ian Everitt
10.2 Diabetic emergencies in emergencies 323
7.11 Herniae 184 children 242 Toby Fogg
Andrew J A Holland Kam Sinn
13.2 Congenital, developmental and
7.12 Gastroenteritis 186 10.3 Thyroid emergencies 244 neoplastic conditions 327
Susan Phin Malcolm Higgins Greg Stevens
7.13 Constipation 192 10.4 Adrenal crisis 246 13.3 Ocular trauma 328
Bruce Fasher Yuresh Naidoo Jennie Martin

xii
CONTENTS

SECTION 14 16.5 Haemolytic uraemic SECTION 20

syndrome 372
ENT and dental 332 Karen McCarthy Analgesia and
Editor: Jeremy Raftos sedation 409
16.6 Nephrotic syndrome 375 Editor: Gary Browne
14.1 The ear 332 Karen McCarthy
Stuart Lewena, 16.7 HenochSchonlein 20.1 Analgesia and sedation 409
Gervase Chaney purpura 379 Stephen Priestley, Jason
Acworth, Anthony Harrington
14.2 The nose 336 Karen McCarthy
Stuart Lewena,
Gervase Chaney
14.3 The mouth and throat 338 SECTION 17 SECTION 21
Stuart Lewena,
Psychiatric 382
Poisoning 421
Gervase Chaney,
Editor: Gary Browne
Richard P Widmer Editor: Jeremy Raftos

14.4 Retropharyngeal 21.1 General approach to

17.1 Paediatric psychiatric
abscess 343 poisoning 421
emergencies 382
James Tilleard Naren Gunja
Raymond Chin, Michael Fairley
14.5 Foreign bodies and caustic 21.2 Specific poisons 426
ingestion 344 17.2 The treatment of the Naren Gunja, Helen Mead,
Stuart Lewena, behaviourally disturbed Nicholas Cheng
Gervase Chaney adolescent 386
Kenneth Nunn

SECTION 15 SECTION 22
Obstetrics and Environmental 436
SECTION 18 Editor: Gary Browne
gynaecology 347
Editor: Jeremy Raftos Crisis intervention 391
Editor: Gary Browne 22.1 Envenomation 436
15.1 Paediatric gynaecology 347 Julian White
18.1 Sexual assault 391
Pamela Rosengarten, Sonia 22.2 Drowning 453
Grover Matt Ryan
Simon Wood
15.2 18.2 Child at risk 394
Emergency 22.3 Heat-induced illness 459
contraception 354 Simon Young, Raymond Chin,
Michael Ragg
Alastair D McR Meyer, Gervase Chaney
Jacqueline E L Parkinson 22.4 Cold injuries 462
Simon Chu, Nicholas Cheng
22.5 Anaphylaxis 468
SECTION 16 SECTION 19 Andrew Stewart Kemp
Renal 356 Administration
Editor: Jeremy Raftos and EMS 399
Editor: Gary Browne
16.1 Acute kidney injury 356
SECTION 23
Barry Wilkins 19.1 Managing the death of a child Common procedures 472
in the ED: Bereavement Editor: Gary Browne
16.2 Haematuria 362
issues 399
Frank Willis
Paul Tait, Roger Barkin, 23.1 Length-based paediatric drug
16.3 Hypertension 364 Pat Clements dosing and equipment
Frank Willis sizing 472
19.2 Forensic paediatrics and
Ronald A Dieckmann
16.4 Urinary tract infection in the law 404
pre-school children 369 Maree Crawford, Natalie 23.2 Bagmask ventilation 474
Frank Willis Phillips Michelle Lin

xiii
CONTENTS

23.3 Nasopharyngeal, 23.18 Lumbar puncture 515 27.2 Sick child in a rural
oropharyngeal airways Ronald A Dieckmann hospital 583
and the laryngeal mask Murali Narayanan,
airway 476 Robert Henning
Michelle Lin, Conor Deasy
SECTION 24
23.4 Endotracheal tube and
tracheal intubation 479
Orthopaedics and
Michelle Lin
rheumatology 518 SECTION 28
Editor: Gary Browne
23.5 Confirmation of Teaching paediatric
intubation 483 24.1 Orthopaedics and emergency medicine 587
Michelle Lin rheumatology 518 Editor: Ian Everitt
Robyn Brady, Navid Adib
23.6 The surgical airway 485
28.1 Availing web-based
Judith Klein 24.2 Fractures and dislocations 532
resources 587
Robyn Brady, John Walsh
23.7 Needle thoracostomy 488 Colin Parker
Judith Klein 24.3 Spinal injury 544
28.2 Teaching paediatric emergency
Ed Oakley
23.8 Tube thoracostomy 490 medicine 589
Ronald A Dieckmann 24.4 Risk management in acute Colin Parker
paediatric orthopaedics 553
23.9 Removing and replacing a
Robyn Brady
tracheostomy tube 492
Ronald A Dieckmann
SECTION 29
23.10 Central and peripheral
intravenous lines 494 SECTION 25 Paediatric research
Judith Klein Male genitalia 555 in the emergency
Editor: Gary Browne department 593
23.11 Intraosseous infusions 500
Editor: Ian Everitt
Lindsay Bridgford,
Ronald A Dieckmann 25.1 Male genitalia 555
Colin S Kikiros 29.1 Research in children in the
23.12 Rectal drug emergency department 593
administration 502 Franz E Babl, Meredith Borland,
Ronald A Dieckmann Andrew J Davidson
SECTION 26
23.13 Umbilical vessel
cannulation 503 Acute neonatal
Judith Klein problems 558
Editor: Gary Browne SECTION 30
23.14 Electrical countershock 506
Ronald A Dieckmann, 26.1 Acute neonatal Adolescent medicine
Conor Deasy emergencies 558 in the emergency
Paul Craven, Elly Marillier department 599
23.15 Pericardiocentesis 508
Editor: Ian Everitt
Ronald A Dieckmann
23.16 Transurethral 30.1 Adolescent medicine
catheterisation and SECTION 27 in the emergency
suprapubic bladder department 599
Transport and retrieval 574
Katherine Barton,
aspiration 511
Editor: Ian Everitt
Conor Deasy, Judith Klein Donald Payne
23.17 Penile zipper injury 27.1 Emergency medical transport
514 and retrieval 574
Michelle Lin Andrew Berry Index 603

xiv
 1

SECTION
APPROACH TO THE
PAEDIATRIC PATIENT
Section editor Ian Everitt

1.1 Approach to the paediatric patient 1 1.2 The crying infant 14

1.1 Approach to the paediatric patient

Ian Everitt Andrew Jan Andrew Harris Tom Everitt Lisa Coutts

paediatric emergencies. This function may

ESSENTIALS occur through advisory, liaison and educa-
tive processes.
1 Gaining rapport with the child and the confidence of the parents is the Some critically ill children will arrive in a
key to assessing children.
more predictable fashion via ambulance
2 A child needs to be approached according to chronological and developmental and some preparation can occur to plan
age. for their initial treatment. On the other
hand, a child in extremis may well be rushed
3 Observation is a vital diagnostic tool, which is vastly more important in in from a family car, without any prior warn-
children than in adult patients.
ing of their arrival. Systems of preparedness
4 The need for investigations is a balance between an invasive stress on a child for these situations are critical for the imme-
and the potential gain of information to aid decision making. diate assessment and optimal early manage-
ment of children by emergency department
5 Always back up discharge with a concrete action plan and definitive follow up. staff (see Chapter 2).
6 It is often more important to exclude serious illnesses than make a
definitive diagnosis.
Evolution of paediatric
7 Addressing parental concerns is an important part of the therapeutic process. emergency medicine
8 Emergency physicians should stay within their comfort zone and when in In Australia and New Zealand there are cur-
doubt, consult. rently only nine stand-alone tertiary paedia-
tric emergency departments. Hence, the
9 A febrile child should be considered as potentially sick until one can confidently majority of paediatric patients present
conclude that he/she is well following a thorough assessment.
initially to mixed departments where approx-
10 Always reflect on the potential fears of the child and parents. imately 1030% of attendances are paedia-
tric. This is similar to the UK, Canada and
USA. Some of these children will require
subsequent referral to a paediatric tertiary
arrive, due to close proximity in an emer- centre for ongoing specialist care. The role
Introduction gency, at adult departments where staff and functioning of emergency departments
Who sees paediatric may be less familiar with the management has changed dramatically over the past
emergencies? of children. Likewise, paediatric emergencies three decades. Paediatric emergency medi-
It is essential that all doctors are familiar occur remote from emergency departments cine (PEM) has now developed as a speciality
with the recognition and management of and may require stabilisation by general of paediatrics and emergency medicine.
the seriously ill child. The majority of chil- practitioners, paramedical staff or layper- The initial assessment and stabilisation of
dren presenting to emergency departments sons prior to subsequent referral. It is an paediatric patients, which in the past was
are either taken to tertiary paediatric cen- important role of emergency departments often deferred to intensive care or anaes-
tres or mixed departments that see both to be an available resource to support thetic colleagues, is now an important role
adults and children. A small number will the community in the management of of emergency physicians. In Australasia,

1
1.1 APPROACH TO THE PAEDIATRIC PATIENT

the development of PEM as a subspecialty the obviously sick or the potentially sick assess each of these possibilities.2 With
of the College of Paediatrics has seen the child. One of the major tasks for the emer- experience, the ability to appreciate a sick
establishment of a supervised training gency physician is to identify the sick child child improves; however, a good rule, partic-
scheme by the Paediatric Emergency Medi- from a large undifferentiated group of chil- ularly in the younger child, is, if in doubt,
cine Special Interest Group for advanced dren who may present as potentially sick. investigate, admit for a longer period of
paediatric trainees. A joint training It is by a filtering process via history, exami- observation or seek the second opinion
programme overseen by both the Australa- nation, observation, investigation and con- of a colleague.
sian College for Emergency Medicine and sultation that one identifies the potentially
the Royal Australian College of Physicians sick child (Fig. 1.1.1). This group of patients
oversees the training qualifications of includes: those children who have pro-
paediatric emergency physicians. It is para- gressed to a severe form of a usually benign
Children with fever
mount that physicians and trainees in both illness; those with early, subtle signs of a The concept of occult bacteraemia (OB)
specialist and general emergency depart- serious disease; or those who on initial highlights the difficulties in detecting signif-
ments are well trained and the facilities are assessment appear unwell, but require icant illness in febrile young children. With
appropriate for the resuscitation of critically investigation to help rule out serious dis- the recent advent of widespread vaccination
ill children. Hence, the education and train- ease. It is often through observation of a to the common agents of occult bacteraemia
ing of emergency physicians in the manage- child, that one is able to more accurately (Hib, pneumococcus) the prevalence of
ment of common paediatric emergencies is
an important role of both paediatric and Paediatric
emergency colleges. Whether a trainee is patient
seeking a career in a paediatric or a mixed ATS 35
emergency department, experience gained
in both environments with exposure to
the teaching of both paediatricians and History warning bells: Examination warning bells:
Representation Pale, pasty child
emergency physicians is advantageous. < 50% fluid intake Floppy child
Likewise, a rotation in paediatric intensive No smiles or prolonged irritability Drowsy child
care and paediatric anaesthesia provides Prolonged lethargy Non-smiling child
No urine output for 6/24 Altered vital signs and/or O2 sats
additional skills in resuscitation, airway Cyanosis, apnoea, seizures Signs of compensated shock
management, haemodynamic support and BIBA (Brought In By Ambulance) Respiratory distress and/or tiring
Nurses are worried! Sicker than usual, gastro/croup/
monitoring of critically ill children. The APLS bronchiolitis/URTI
(Advanced Paediatric Life Support) and
ATLS (Advanced Trauma Life Support) or
equivalent courses are invaluable additional Well Potentially sick Sick
resources and are requirements to satisfy
fellowship qualifications in the training of
physicians who will be managing paediatric Trial of Warning bells: Trial of time Confounders
observation persistent antipyretic Post-vomit
emergencies. exam warning Fluids/icypoles Sleep
bells Entertainment Post-ictal
Anxious
Identifying the potentially
sick child
Of the vast number of children attending
Investigations Full blood count CXR
emergency departments, approximately CRP LFTs
25% are classified as immediate emergen- U and E/Cr, glucose LP
Urinalysis MSU
cies (Australasian Triage Scale (ATS) 1 and Others BC
2) that require urgent assessment and man-
agement.1 Importantly, children can present
with a less urgent triage category, but may Consultation Colleague
Registrar
rapidly deteriorate from evolving sepsis or Paediatrician
airway compromise. The majority of paedia-
tric presentations consist of less emergent
problems involving a wide spectrum of inju-
Action plan Discharge Review by
ries and illness. Of this group of paediatric What to observe ED
patients there is a subset where the diagno- When to return Paediatrician
What to do General practitioner
sis is not immediately apparent. Thus, pae- Phone call
diatric patients can generally be divided
into three broad groups: the obviously well, Fig. 1.1.1 Algorithm of paediatric decision making.

2
1.1 APPROACH TO THE PAEDIATRIC PATIENT
1

APPROACH TO THE PAEDIATRIC PATIENT

paediatric sepsis has diminished signifi- treatment. The younger the child, the Likewise if a child is to undergo a proce-
cantly and the clinical experience of manag- greater the potential for rapid deterioration dure during which he may become dis-
ing septic children has been diluted in as the early manifestations of a serious ill- tressed, such as intravenous insertion or
developed countries. Hence, one needs to ness may be subtle and non-specific. One laceration repair, it is best performed in a
have a planned approach to the assessment must be vigilant for the early signs of com- closed dedicated procedure room. This will
of febrile children at various ages. Bacterae- pensated shock such as tachycardia, avoid visual or auditory distress to other chil-
mia can in its most manifest form present as decreased capillary refill, mottled skin, cool dren and parents. A mounted television/
a febrile, pale, pasty, mottled child, centrally peripheries, decreased urine output, or video monitor in this setting can be an excel-
warm but with cool peripheries. Some young drowsiness. Early detection and fluid resusci- lent distraction during procedures, as an
children with bacteraemia, however, can tation at this point may prevent hypoten- adjunct to analgesia and sedation. For neo-
appear completely well apart from fever. sion in a child with evolving sepsis. nates and small infants a radiant heater over
Investigations may help identify them with Children with severe and deteriorating respi- the examination bed will aid temperature
a high white cell count or c-reactive protein ratory illness will manifest fatigue. It is the stability, examination and often the discov-
(CRP), but these inflammatory markers are early recognition of children with serious ill- ery of veins for cannulation.
unfortunately often non-discriminatory ness or the potential to deteriorate that is The paediatric resuscitation area should
between benign and serious causes. The critical to the timely initiation of effective include wall charts, which refer to emer-
problem is not so much that children with treatment.2 An important principle in emer- gency algorithms and drug dose guidelines,
OB are sick at that moment of time, but gency paediatrics is to be proactive. One which can be rapidly referred to during the
the possibility of the later development of must be aware of the importance of regu- resuscitation of critically ill children. A white
serious bacterial sequelae necessitates larly reviewing a childs response to a given board is handy for pre-sizing and dosing for
timely treatment. Conversely, many bacter- therapy, escalate treatment if required and the imminent arrival of a sick child. Updated
aemic children will spontaneously clear the be vigilant for subtle signs of deterioration. clinical guidelines in hard copy and elec-
organism without therapy. Therefore, these tronic form in the management of common
children remain in the potentially sick cate- paediatric emergency conditions are a use-
gory of patients and are obligated to have ful resource to be available within the
either admission for observation or dis- department. Clinical pathways initiated in
The environment the emergency department can be useful
charge with frequent planned reviews for
sequelae and a definitive action plan for The physical environment of the emergency in ensuring consistency of management
their parents should the condition change. department needs to reflect a paediatric from all levels of medical and nursing staff,
The evidence for the use of prophylactic milieu with appropriately equipped cubicles as well as improving continuity of care in
antibiotics in these children is controversial. for the reception of children accompanied by children who require admission to an inpa-
The age of a patient and height of the tem- their carers. Despite the noise inherent in a tient unit.
perature are useful risk factors to consider in busy department of sick children, the envi-
the approach to individual patients. In chil- ronment should be as calm and relaxed as
dren less than one month, any fever is signif- possible. Wall or ceiling posters, mobiles, a
icant, whereas older children are more at risk
Triage
selection of toys and books are useful to dis-
of serious illness with higher temperatures. tract younger children from the distress and Paediatric patients arriving in the emer-
Likewise, hypothermia can occur in over- threat of an unfamiliar hospital environ- gency department should undergo triage
whelming sepsis, particularly in neonates ment. Posters of current popular characters according to standardised Australasian Tri-
and infants. such as Teletubbies, Blues Clues, Shrek, age Scale (ATS 15) so that they are seen
Wiggles or Harry Potter are useful. Not only in a prioritised fashion according to acuity.
do these characters make kids feel happy, In mixed emergency departments where tri-
but their active recognition provides a useful age nurses may have had less paediatric
Evolving illness in children
CNS diagnostic tool. A few initial moments experience, there has been a tendency to
Due to differences in anatomy, physiology, gaining a childs confidence with a toy will up-triage paediatric patients.1 The use of
development and psychology, childrens dis- usually reward the doctor with a more rapid scoring systems for specific conditions or a
eases are age specific, with serious illness and thorough assessment of the reluctant Triage Observation Tool may be helpful in
often taking time to evolve.3 Many children child. Supplies of stickers or bravery certifi- improving the reliability of triage in young
present to an emergency department in the cates are excellent rewards to have on hand children, who may present with non-specific
early stage of an illness and making a defin- for young frightened children who have symptomatology.4 A secondary nursing
itive diagnosis may require time. The clinical undergone imaging or blood tests. If possi- assessment should occur when the child is
status of paediatric patients may also ble, in a mixed department, children should admitted to a cubicle, with further observa-
change rapidly. This can occur in response be completely separated from adult patients. tions performed at the bedside, so that any
to prior trauma, evolving sepsis, toxin Adult patients who are behaviourally dis- change in condition can be detected early
absorption or a seizure, and necessitate a turbed will be distressing for a child and and acted on promptly. The senior doctor
change in the initial priority to receive family to see or hear in a nearby cubicle. in the department should immediately be

3
1.1 APPROACH TO THE PAEDIATRIC PATIENT

informed of children triaged as ATS 1 or 2 to The assessment of children in the emer- important to be age appropriate and above
direct timely management. In times of high gency department setting can be both chal- all honest. Never tell a child you wont feel a
workload, children with an ATS 3 may not be lenging and very rewarding. It is a challenge thing! prior to plunging a cannula through
definitively assessed within 30 minutes and to modify the clinical approach according to an EMLA anaesthetised cubital fossa.
should have a senior doctor rapidly assess the chronological and developmental level Rather, explain in age-appropriate terms
status and initiate therapy, if required. It of the individual child. Likewise, treating what it may feel like and that its OK to cry.
may be necessary to modify normal triage paediatric patients is a rewarding area of Maintaining a childs trust at all times is
systems when emergency department num- emergency medicine, as children will often crucial and will positively influence any
bers are affected by surges in demand when respond rapidly to management within the subsequent medical contacts the child may
significant influenza outbreaks or the like time frame of the emergency department have. The demonstration of a procedure on
occur. attendance. a doll may decrease the anticipatory trepida-
tion in a child.
Gaining rapport The assessment of a child should always
Fast tracking Endeavouring to gain initial rapport with a be carried out in the presence of the parent
child and the confidence of the parents is or carer, unless the child arrives by ambu-
Some initiation of treatment is appropriate
the key to assessing children in the emer- lance or other means without the parent/
during the triage process, such as the provi-
gency department setting. An unrushed, carer present, and the childs medical needs
sion of analgesia for pain or an antipyretic in
gentle and caring manner will rapidly settle warrant immediate attention. Otherwise, it
a child symptomatic of fever. It is important
the fears and anxieties of most children and is prudent in the non-urgent situation to
that children with pain are given early and
their parents. This usually allows the exami- provide a staff member to support the child
appropriate analgesia or have injuries
nation to proceed in a non-threatening fash- and defer the assessment until carers are
splinted when required. This will facilitate
ion and improves the reliability of clinical present.
a more comfortable, reliable and expeditious
signs. It will take time, experience and the
assessment. The use of opiates, when
observation of colleagues techniques for Development appropriate
required, will only enhance, rather than
every emergency physician to develop their Infants particularly benefit from the con-
detract from the subsequent physicians
own individual approach to children. A thor- stant presence of their parent in their visual
physical examination.5 The use of visual
ough examination without causing distress field in order to avoid stranger distress and
analogue scales such as the WongBaker
to a child is very reassuring to a parent. Many are often best examined in the parents
faces may assist the assessment of a childs
children arrive at an emergency department arms. Neonates can be examined on the
response to analgesia. A process of fast
miserable, in pain, fearful, or with some examination bed as long as they are kept
tracking appropriate children with single
trepidation of what lies ahead. With a child- warm. Toddlers, despite their evolving
limb injuries for an X-ray prior to definitive
friendly approach by all staff, most will leave autonomy, will usually be less fretful if exam-
medical review may improve efficiency
feeling much better and, hopefully, even ined on a parents lap. It is a useful sign of
through the department. Febrile children
having enjoyed the experience. illness or other cause to note when young
who present with a rash, not clearly due to
children do not exhibit these normal
a viral exanthema or benign phenomena,
Age appropriate stranger anxieties. The preschooler who
should be fast tracked to be seen by a senior
The approach to any child in the emergency enjoys a sense of play and imagination
doctor to consider the possibility of meningo-
department is dictated by the childs age can usually be relaxed during an examina-
coccaemia. It is useful to have documented
and developmental level. It is useful to tion or procedure by storytelling or engaging
management plans for children who may
have a modified approach to suit newborns, in play with a toy. An anxious early school-
recurrently present to the department. This
infants, toddlers, preschoolers, school chil- aged child may respond to participation
includes conditions such as complex children,
dren and adolescents. An understanding of in the examination or being asked about
brittle asthma, cyclical vomiting or recalci-
the concept of the fourth trimester is useful school or other more favoured activities.
trant seizures where a clear plan of manage-
in dealing with crying phenomena in the Adolescents, on the other hand, need to
ment can expedite care by ED staff.
first months of life, which will often precipi- be approached in a more adult fashion
tate emergency department visits (see and should be offered confidentiality and
Chapter 1.2). A preverbal or developmen- the opportunity to choose whether their
The paediatric approach tally delayed child wont tell you of pain parents are present (see Chapter 30.1).
The evaluation process of a child in the which has shifted to the right iliac fossa. In the event of an unatonable child resist-
emergency department involves history, An unwell 14-month-old clinging to mother ing any examination, one may have to mod-
observation, examination and may include may actively resist the initial attempts to be ify the approach to gain essential clinical
relevant investigations. examined by a stranger. The absence of findings in a gentle but firm manner. It is
Each of these components needs to be familiarity with a family or child that their unusual, however, for a child to remain
considered in the formulation of a diagnosis family doctor may have may further impede unexaminable if appropriate analgesia is
and disposition plan. A child needs to be the assessment of anxious children. When given and the child is left for a period of time
considered in the context of the family. explaining procedures to children it is undisturbed.

4
1.1 APPROACH TO THE PAEDIATRIC PATIENT
1

APPROACH TO THE PAEDIATRIC PATIENT

Parental involvement concerns such as a fear their febrile child
Table 1.1.1 History warning bells
In order to provide emotional support, par- has meningococcal disease when commu-
Child taking less than 50% of normal fluids
ents should be encouraged to remain close nity alertness to this condition is height- The child with prolonged lethargy
to their child during any procedures. Parents ened. Addressing this concern may occupy No urine output for six hours
Prolonged irritability or inconsolability
who appear at risk of vagal syncope are best most of the doctors time. The parent of a Report of cyanosis, pallor, seizures or
seated away from the viewing of action but child who has sustained an accidental scald significant apnoea
The child who has not smiled over a period of
still in their childs earshot for verbal sup- or injury may be feeling distressed or guilty hours
port. Childrens behaviour often mirrors that and sensitivity to this is required. Again, Nursing staff feel the child is just not right
Unplanned re-presentations
of their parents, so gaining the confidence of addressing guilt will involve much of the Parental concerns out of proportion to childs
the parent will often make an anxious child doctors time. illness
Brought in by ambulance
relax prior to procedures. The use of a confi- History not compatible with injury/?
dent, calm, caring approach will be rewarded Children-specific issues non-accidental injury
by a child who will allow a more reliable In younger children, certain symptoms are
examination. It is very reassuring to the par- less specific. The report of vomiting in an
ents to see that the doctor is experienced in infant may be due to meningitis, pneumo-
dealing with children and anticipates the nia, tonsillitis or urinary sepsis rather than Table 1.1.2 Important elements
expected anxieties and reluctance to exami- gastroenteritis. The assessment of wellness of the paediatric history
nation that a child may have when unwell. or otherwise in infants can be more chal- Presenting complaint
lenging due to their limited psychomotor
Pregnancy
activities. Indeed, their spectrum of normal
History behaviours involves sleeping, waking to cry Perinatal delivery type, birth weight, need for
resuscitation/special care nursery admission
or demand a feed, followed by a return to
The initial contact with the family should Development in a CNS problem, compatibility
sleep. Hence, it is important to enquire into
include an introduction of who you are. with injury mechanism
their feeding status and sleep/activity pat-
The parents should be addressed and the Immunisation status need to clarify carefully
tern as an indicator of compromise due to ill-
child greeted by name, in an age-appropriate
ness. One needs to carefully clarify what Previous illnesses/surgery/admissions/
manner. It is important to consider ones medications
their current intake is compared to their nor-
approach in terms of the needs of both the
mal breast- or bottle-feeding. An infant who Allergies
childs illness and the parental concerns.
is feeding less than 50% of normal has sig-
The history is generally elicited from the Infectious contacts/recent travel
nificant compromise. It is important to note
parent or caregiver but it is appropriate, in Family history
the report of a young febrile child who
a verbal child, to augment this information
remains lethargic and fails to smile or inter- Social history family circumstances may
by directly questioning the child. influence a childs disposition
act with parents. In the otherwise well-looking
infant, who appears mottled, clarify with Fasting status if relevant
Critically ill child parents whether this may be usual for their
Sometimes the normal routine of history, fol- Feeds normal bottle or breast feeds for
child (i.e. physiological cutis marmoratum comparison
lowed by examination will need to be versus sepsis). In assessing young children
altered in a child who arrives critically ill. with trauma, a thorough history of the timing
The management will need to be expedi- and mechanism of injury, noting the childs
tious and occur simultaneously with the developmental capabilities, is paramount to examined in a systematic fashion similar
gathering of pertinent information from detecting possible non-accidental injuries to adult patients. However, younger chil-
the parents. Parents should be given the (see Chapter 18.2 on NAI). dren usually need to be examined in a less
opportunity to remain at the bedside of their Other useful information to cover in the formalised manner, whilst maintaining a
critically ill child undergoing resuscitation, paediatric patient history is shown in high degree of vigilance. The order of the
with a dedicated support person. Tables 1.1.1 and 1.1.2. examination may need to be adapted
according to the individual childs
Parental issues responses. In a reluctant examinee, clinical
The clarity of the history given by parents findings may be achieved by surreptitiously
can be affected by parental distress, anxiety
Examination examining through play as the opportunity
or sleep deprivation. One should begin the Age appropriate arises. Much can be ascertained in this situ-
history in a focused manner according to The examination technique used in paedia- ation by careful observation rather than a
the presenting complaint. Later it may be tric patients depends on the age and devel- more threatening hands on approach. Enter-
useful to explore individual parental anxi- opmental level of the child. The key is to gain taining a young child in a professional man-
eties. One of the most important questions the confidence and then the co-operation ner during examination will generally allow
to a parent is, What is your biggest worry of the child. Older children are generally the confounding influence of anxiety to
or fear? Some parents may have specific compliant, which allows them to be diminish.

5
1.1 APPROACH TO THE PAEDIATRIC PATIENT

abdomen, which will be altered in the crying child in the ED. Young children with throat
Gentle, distraction, state, prior to disturbing the child to wake- discomfort will be reluctant to volunteer a
painful last fulness. A neonate examined supine needs cough, but a gentle tickle of the axilla or pal-
to be kept warm with a blanket or a radiant pating the anterior larynx will usually pro-
Children are usually reluctant to have any
heater. Hands should be warm. The crying duce a bark to clarify suspicion of croup.
painful area disturbed. Confirming tender-
fractious baby may be settled by offering a Recognising the pattern of respiratory dis-
ness needs to be gentle and unhurried to
feed or a pacifier before the examination. tress in a child from the end of the bed will
minimise any distress, with appropriate prior
The symmetry of normal infant reflexes is often differentiate upper and lower airway
analgesia. Many young children will respond
a useful screen for any focal motor problem obstruction, prior to any auscultation. Chil-
to age-appropriate verbal banter during the
or as a localiser of a painful limb that will dren with upper airway obstruction have
examination, which distracts from the per-
modify normal symmetry of response. slower inspiration, whereas gas-trapped
ceived threat of the examining hand. Alter-
wheezers will have diminished flow and
natively, one may need to gently palpate a
tender right iliac fossa, whilst using distrac-
Index of suspicion speed of expiration on observation.
The routine methods of examination for
tion such as the counting of the childs fin-
gers. Sometimes a child may prefer their
signs of meningism in children are unreli- Abdominal examination
able. Asking the child to look upwards at The abdominal examination needs to
tender abdomen to be palpated with the
an object or down to their umbilicus is a always conclude with the nappy area for
examiners hand through their own hand.
more useful screen to detect nuchal irrita- otherwise occult torsions, hernias, skin pro-
The examination needs to be adapted to
tion. The presence of photophobia is sensi- blems and for stool examination, if present.
the childs responses, deferring distressing
tivity to ambient light as most children will The rectal examination in children is not
phases until the final moment of examina-
be offended by a torch light in the eyes. In routine and only performed with clear indica-
tion. Time used initially to gain a childs con-
children less than two years old, the early tion. One needs to be cognisant to maintain
fidence will make subsequent assessment
signs of meningism are often absent and privacy and dignity, particularly when exam-
more rewarding and the clinical signs more
threshold for cerebrospinal fluid (CSF) exam- ining older children and adolescents. The
reliable. We have all experienced the frustra-
ination adapted accordingly. Other signifi- examination of a child with possible sexual
tion of trying to assess the abdomen of a
cant but subtle features that may be abuse is outlined in Chapter 18.1.
screaming or fractious child who demon-
missed on examination include persistent
strates the pseudo-rigid abdomen! Indeed,
the most reliable method of excluding peri-
tachycardia or tachypnoea that is not clearly ENT last
related to fever. One needs to be alert to the In preschool age and younger children
tonism in a child does not involve any palpa-
spectrum of stigmata of non-accidental inju- examination of the ears and throat is
tion of the abdomen. Asking a child to
ries that may present to the emergency best deferred to last. A gentle, but rapid
cough, walk, jump or climb the trolley are
department (see Chapter 18.2 on NAI). approach is necessary to achieve an accu-
useful manoeuvres to help exclude perito-
rate assessment of the oropharynx, followed
neal irritation.
The examination of ears and throat, ten-
Respiratory examination by a cuddle from the parent. Despite the
Noisy breathing in children can sometimes potential difficulty, the source of fever will
der abdomen or a painful injury, is best left
be difficult to determine if it is due to airway often be overlooked if the throat is inade-
till last in order not to upset a child and
make the remaining routine examination obstruction of intra- (lower airway) or extra- quately visualised in children. In infants,
thoracic (upper airway) origin. The localisa- the throat is best examined with the child
difficult. If one detects that a child has an
tion of airway obstruction to a particular lying supine with both arms abducted along-
unfortunate fearful memory of a stetho-
segment of airway can often be aided by side the head to prevent movement. A
scope or the like, a preliminary auscultation
successive auscultation over the nares, young child who is fearful of throat examina-
of a childs soft toy and warming the dia-
mouth, larynx and peripheral airways. tion needs to be held as still as possible for a
phragm will often allow this to subside. Dis-
Remember, young children may manifest rapid, one gag, one look approach. This is
tracters such as a soft toy placed in the hand
both upper and lower airway involvement best performed after explanation, position-
may alleviate the examiner from the torture
of the curious infant who yanks on the (crasthma, broup, cronchiolitis) with inflam- ing the patient upright on a parents knee,
matory involvement of both segments of the securing arms beside the trunk with the par-
stethoscope tubing during auscultation.
respiratory tract. Younger children are often ents dominant arm and holding the head
easier to auscultate by listening through straight-ahead with the other. Lets count
Improvise clothes (avoiding the stethoscopecry your teeth is a less threatening signal to
The examination of infants and young chil- reflex) from behind whilst being held by most children to open the mouth, rather
dren is best done in the least threatening the parent. Detection of occult asthma in than mentioning the tonsil or throat
position. This is usually best whilst being a child with suggestive symptoms but no words, particularly if parents warn you that,
held securely in the parents arms or on wheeze, may be aided by comparing the Nobody has been able to get a look at his
the knee. If a young child is sleeping, the diminished volume and rate of airflow in throat. Some children require gently insert-
opportunity should be taken to perform aus- expiration compared to inspiration, or alter- ing the spatula between clenched teeth to
cultation, palpation of a fontanelle/ natively re-auscultation after exercising the touch the tongue to initiate a gag. Most

6
1.1 APPROACH TO THE PAEDIATRIC PATIENT
1

APPROACH TO THE PAEDIATRIC PATIENT

LOOK, prior to examination Observing breathing
Table 1.1.3 Examination warning bells
Before the laying on of hands, much can be When observing tachypnoea in a child, it is
The pale, pasty child
gleaned by initially observing a child and useful to determine whether a child has
The floppy child parent in the cubicle, from a distance. Ones quiet tachypnoea (breathing fast and qui-
The child who appears drowsy
first impressions of a child will often direct a etly) with no evidence of increased work of
subsequent approach. The childs state of breathing, such as may occur in conditions
Alteration in vital signs, SaO2
activity and interaction with the environ- of fever, acidosis or cyanotic heart disease.
Early signs of compensated shock ment and parents will often change when Children with noisy tachypnoea (breathing
The tiring child with respiratory distress
a strange doctor approaches. The process fast and hard) demonstrate increased work
of evaluating a child differs from that of of breathing, due to conditions such as air-
The child who never smiles despite appropriate
prompting
an adult by the greater importance of obser- way obstruction, pneumonia or heart failure.
vation. It is important to emphasise the
The child who looks sicker than the usual child
with gastroenteritis/croup/bronchiolitis/URTI
observation of a child to aid diagnosis in Confounders
the emergency department setting. This is There are several observational confounders
Other specific signs particularly important in evaluating children that can influence initial decision-making,
Non-blanching rash petechiae/purpura- during their first three years of life. The end when children can appear transiently sicker
sepsis of bed observations can be an important than they really are. There are many times
Bulging or full fontanelle raised intracranial indicator for differentiating the potentially when the initially sick-appearing child can
pressure sick child. pick up and appear well again over a short
Bilious vomiting bowel obstruction period of time. This situation usually occurs
High pitched cry meningitis
when the paediatric consultant is called and
Observational variables by the time he or she arrives the child is smil-
Grunting respiratory distress The general appearance of a child should ing and running around the department.
include noting level of alertness, eye con- The vomiting child will often look pale and
tact, activity, quality of cry, posture, interac- pasty for up to 20 minutes post vomiting.
tion with the environment, irritability, colour, One-hour post antipyretic the symptomatic
older children, fortunately, will happily vol- hydration, perfusion, general growth and febrile child can look like a different child.
unteer a view of the pharynx. nutrition, respiratory distress and presence Children initially emerging from a simple
Following any distressing procedure it is of any unusual smell (e.g. ketotic). The lack febrile convulsion can look well again in
important to acknowledge bravery in a of normal resistance to examination or a 20 to 30 minutes. A young childs physiolog-
frightened child. Likewise, giving a child an procedure expected of a child is an impor- ical sleep can mimic septic drowsiness or
honest, developmentally appropriate expla- tant observation to note. The sick child somnolence resulting from head trauma. A
nation of what to expect prior to any proce- may make none of the resistance expected fearful child, during examination, can esca-
dure, such as an IV insertion, is to be to examination or venepuncture. Observa- late the examiners perception of his illness.
encouraged. This is best done immediately tional variables have been shown to be more
prior to the procedure so that an anxious predictive of serious disease than historical Re-evaluate
childs fears dont escalate in the intervening information in young children.6 Likewise, This reinforces the power of observation.
period (Table 1.1.3). clinical examination, considered alone, is a It allows time for a trial of fluids, reducing
poor predictor of serious illness. Observation fever with an antipyretic, or seeing if a
of a child needs to be performed as a sepa- child responds to distraction. Subsequent
rate process from the examination and may re-evaluation of the child often allows one
Observation
require a period of time for re-evaluation to to differentiate whether a child is sick or well.
Observation is the distant examination of a detect disease progress. Researchers have The use of observation really allows one to
child that begins prior to introducing oneself used formalised scales such as the McCarthy identify the persistence of the initial abnor-
to the family, and continues after the exami- Observation Scales to aid this assessment in mal examination findings. A child with intus-
nation, whilst one may be writing up notes febrile children.7 In the ED setting, discus- susception may intermittently appear well
or between seeing another patient. It also sion of a child with a colleague can be a and observation may be required to observe
includes the noting of nursing remarks and rewarding aid to decision making. the clues to prompt the appropriate diagnos-
vital signs recorded in order to obtain addi- A childs posture, undisturbed, can be a tic investigation (Table 1.1.4).
tional clues as to the sickness of a child. The useful clue to systemic illness, abnormal
trends of nursing observations over time are neuromuscular function or a painful limb
useful indicators to detect early signs of dis- or joint. Children with sepsis or meningitis
When to investigate
ease progression or the response to therapy. may be floppy or flaccid. In other cases,
This ongoing triage, in effect, is particularly the only sign of meningeal irritation may One needs to be judicious with the use of
important to detect evolving illness that be a child who is holding his neck in a investigations in children in the emergency
may otherwise remain undetected. slightly extended position. department. Investigations serve more than

7
1.1 APPROACH TO THE PAEDIATRIC PATIENT

old, dipstick analysis is inaccurate to exclude parents demand admission, it is usually best
Table 1.1.4 Observation warning bells
infection and microscopy must be perfor- to admit.
Decreased level of alertness, activity, eye
contact
med to exclude pyuria. The appropriate
techniques for obtaining urine are discussed Communication issues
Drowsiness or decreased interaction with the
environment/parents
in Chapter 16.4. Explanations to parents as a general rule
should be appropriate to their level of
Abnormal posture
understanding and education. Provision of
Abnormal quality of cry
The parents handouts (with accompanying verbal expla-
Prolonged irritability or inconsolability nation) or written instructions is useful
Parents who accompany their child to an to reinforce diagnosis and management.
Ongoing pallor emergency department are often anxious Reviewing a parents understanding of
Decreased peripheral perfusion or hydration and fearful regarding the safety of their instructions prior to discharge will allow
appearance child. It is important to consider that the par- clarification and avoid communication pro-
Persistence of abnormal recorded vital signs ents are entrusting the doctor with the well- blems. Parents may have fears related to
being of their most cherished and precious anecdotal advice from family/friends, misin-
Respiratory distress/tachypnoea (quiet or
noisy) possession. The management of the fears terpretation of media reporting, or other
and the identification of the needs and sources, which need to be explored. Gaining
Persistence of examination warning bells
Confounders post vomit/seizure, high fever, expectations of the parents is an important the confidence of parents before they leave
normal sleep, anxiety role of the doctor attending to their child. the department is an essential part of the
Listening to and addressing the parents con- therapeutic process and has a positive effect
cerns in a sympathetic and unhurried fash- on compliance to therapy. It is useful to
one purpose. They help confirm or refute clin- ion is often the main therapeutic strategy explain to parents the likely natural history
ical suspicions. Occasionally, parents appear to reassure an anxious parent that a child of their childs illness and encourage review
to initially want more reassurance than sim- with a relatively minor illness is safe. Many should significant deviations from this occur.
ple clinical assessment and explanation. parents may be sleep deprived due to
attending to their sick child and this will
Blood tests influence their ability to convey a lucid his-
tory. The time spent at triage or in the wait- Management of paediatric
Previous medical visits or advice may prompt
ing area in a busy emergency department patients
a parent to feel that reassurance that their
child is safe can only be confirmed by the can frustrate the most patient parent. This The urgency of management of children can
magic of blood tests. This may be especially needs to be anticipated and acknowledged be graded into the following categories:
so with a young-looking doctor. However, at the start of the consultation. Sensitivity
with clinical experience and clear explana- to potential cultural issues is important in The critically ill child who requires
all interactions with carers. immediate resuscitation and
tion, parents can usually be counselled out
investigations and probable transfer to a
of this option, when it is clearly not war-
tertiary centre.
ranted clinically, by explaining that blood Managing the parents
taking is not always straightforward in a The emergency department visit may follow
The sick child who needs immediate
investigation and IV access.
chubby toddler and that sometimes two, previous medical consultation(s) where their
three or occasionally more attempts are concerns may not have been addressed and
The child who warrants observation and a
trial of fluids or antipyretics in the
required. The potential psychological stress it is important to explore these. Always
department. Consider the early
to the child is not worth the information that acknowledge the parents fears and anxi-
placement of EMLA cream if further
will be obtained in this instance. However, eties; however, medical judgement should
investigation may subsequently be
on the positive side, blood tests such as a full allow an objective decision about whether
necessary.
blood count (FBC) or a CRP may provide a child is sick or not. An exception to this
reassurance in some uncertain situations. is the parents of children with a chronic ill-
The child that requires no investigation
but whose parents need an explanation
At other times they are useless, for example ness or special needs. They are usually cor-
of the diagnosis and management.
to exclude meningitis in a child with clinical rect when they judge that their child is
tonsillitis, as inflammatory markers are sick. Aggressive and unreasonable carers The management of febrile young children
already raised. will usually respond to a professional, polite is a large part of emergency paediatric
Investigations may provide extra time for and courteous senior doctor. There is usually practice. The current approach to those chil-
observation (by clinically reviewing the a reason behind their behaviour that needs dren without a clear focus is controversial
child!) and reassurance; however, if an inves- to be explored. Even the most anxious par- and varies between institution and indivi-
tigation is performed and is abnormal, action ent will usually respond to a thorough duals. The two alternative approaches
must follow! The screening of urine in febrile assessment of their child followed by an are risk- or test-minimising strategy (see
young children is vital to detect occult uri- explanation of diagnosis and management. Chapter 9). The current approach has been
nary infection. In children less than 2 years In unplanned second presentations where modified by the advent of pneumococcal

8
1.1 APPROACH TO THE PAEDIATRIC PATIENT
1

APPROACH TO THE PAEDIATRIC PATIENT

vaccination. Certainly, it is reasonable, in a Phone review the patients family there appears to be a need for ongoing sup-
336-month-old child, to be guided by clini- yourself, later that day or the next port. When in doubt regarding whether or
cal judgement alone with close planned morning. not to discharge a child, err on the side of
review. If the child appears unwell, a sepsis Provide a concrete action discharge caution. It may be prudent to consult, con-
screen is performed according to symptom- plan. sider a period of observation in the emer-
atology. Neonates and small infants less Admit the patient for observation. gency department, or admit the child to
than three months, where threshold for hospital.
investigation is much lower, need to be
When to admit
approached according to departmental
The decision to admit or discharge a child Continuity of care
guidelines. Undifferentiated febrile children
from the emergency department is easily It is important for emergency department
should be reviewed the next day and ongo-
made when the child requires medical care staff to liaise closely with the admitting pae-
ing, until a definitive diagnosis is made or
that is only available in the hospital setting. diatrician to provide continuity of care and
until the child returns to normal. Parents
The receiving ward will need to have appro- to ensure ongoing care is expedited in the
should be instructed to return to the depart-
priate resources for the ongoing manage- emergency department. Ongoing manage-
ment if their child deteriorates. The dis-
ment of the child, which should be clarified ment and monitoring of the patient is an
charge action plan should give clear and
by discussion with the receiving inpatient important role of medical and nursing staff
understandable instructions on when to
unit/paediatrician. Some children may need after this decision has been made, particu-
return. For example, in the febrile child, this
to be discussed with, and transferred to a larly if there is a delay in the transfer process.
should include: if child becomes more
tertiary paediatric environment when they Any significant change in a childs previous
unwell, with decrease in intake to less than
require, or have the potential to require, pae- status or treatment needs to be communi-
50% normal, no urine output for six hours, or
diatric intensive care facilities or paediatric cated to the appropriate receiving team.
becoming drowsy beyond sleeping. Parents
subspecialty management.
should be alerted to potential complications
such as becoming limp, fitting or appear- Observation ward
Factors influencing disposition
ance of a rash, which warrant urgent review. Significant compromise from many child-
However, many other factors need to be
hood illnesses is often transient and will
considered in the disposition decision
Turning the corner often respond rapidly to interventions com-
(Table 1.1.5). The threshold to admit a child
Managing children is often about under- menced in the emergency department fol-
is influenced by the childs age, availability
standing the natural history of the illness lowed by a period of observation. Parents
of appropriate follow up, assessment of par-
and predicting when the child will turn the can often be reassured during this period
ents ability to provide care and ongoing
corner. For example, one needs to be patient of observation in hospital that their child
monitoring, the natural history of the illness
with a child who clearly has gastroenteritis has remained well and will respond to man-
and likelihood to deteriorate, social factors,
who has been vomiting for two hours. Often agement strategies that subsequently can
comorbidity, distance from hospital, time of
a child will vomit hourly for eight hours, per- be continued at home. Studies have shown
day, parental anxiety levels, availability of
haps once or twice more and then improve. that many children admitted to hospital only
an early paediatric opinion, and the possibil-
Advising the parents of the likely natural his- require a limited period of subsequent in-
ity that a child may be at risk. One needs to
tory of a condition and giving a clear action patient therapy and are discharged in less
assess in a non-judgemental fashion the
plan can be reassuring and prevent unneces- than 24 hours.8 In a tertiary paediatric envi-
ability of the parents to carry out any ongo-
sary re-presentation. ronment an effective way to manage these
ing treatment, and consider admission if
children is by admission to a short-stay
observation ward. The emergency depart-
Decision making
Table 1.1.5 Factors influencing ment needs to be appropriately resourced
Making decisions in paediatric emergency admission threshold with staff to provide ongoing care and regu-
medicine is a balance of history, examina-
Age of child lar review of patients to expedite timely dis-
tion, intuition, knowing when to trust the
Availability of appropriate follow up/review
charge. Conditions suitable for consideration
parents and maintaining objectivity. If you
of an observation ward admission will vary
feel uneasy with your diagnosis regarding Parental ability to provide care and monitoring,
social factors
with local resources and may include
a child, respect that feeling and gain support
asthma, croup, gastroenteritis, febrile con-
until you do feel comfortable with your deci- Comorbidity
vulsion, presumptive viral illnesses, non-sur-
sion. There are many strategies to do this:
Distance from hospital gical abdominal pain, minor trauma, post
Consider early consultation with an Time of presentation
sedation recovery or ingestions.9 In mixed
emergency or paediatric consultant. departments, without the facility of a
Organise early follow up with a Parental anxiety levels
short-stay ward, it is often appropriate to
paediatrician or general practitioner. To enable a paediatrician opinion use the paediatric ward to admit patients
 Have a colleague on the floor listen to Possible child at risk outside hospital
who would benefit from a period of observa-
the story or examine the child. tion (see Table 1.1.5).

9
1.1 APPROACH TO THE PAEDIATRIC PATIENT

Making a diagnosis differentiate the possibility of a serious ill- child which cannot always be allayed by
Not every child leaving the emergency ness in a child, particularly during the sea- sound advice from an experienced GP when
department will do so with a specific diagno- sonal peaks of febrile presentations. This a child clearly has a self-limiting viral illness.
sis. The unwell febrile child needs to have child may be non-specifically different to There may be parental demands for
serious diagnoses such as meningitis con- similarly febrile children, but the experi- pathology testing to ensure nothing is
sidered, before making a diagnosis of viral enced GP may just have a gut feeling that missed even though these may be deemed
illness. Many children without a clear diagno- a second opinion may be warranted and inappropriate by the family doctor. Parents
sis can be managed expectantly and safely refer to ED. may also report significant symptoms such
discharged home with organised review by There are two distinct areas where the as fever, an infant not feeding normally,
a local doctor, paediatrician, or return to general practitioner plays a vital role in pae- cough or stridor which may no longer be
the department. Giving the parents clear diatric emergency management:- present at the time of presentation to the
instructions to return should the state of GP. Some auscultatory chest findings are
Assessment, initial stabilisation and
dynamic and therefore have a fluctuating
their child not follow an expected course is
transfer of the child to the paediatric
essential. Children are often seen early in presence, such as wheezing in bronchiolitis,
emergency department of the
the natural history of their illness and a diag- so may vary greatly between the time of
clearly unwell or potentially unwell
nosis will only become clear with time. It is the GP and emergency department visit.
child, and
important to communicate clearly, verbally It is this complex interaction of both the
Ongoing management and follow up of
medical and environmental factors which
or in writing, with the doctor who will be fol-
the child after discharge from a hospital
lowing up the child. Close liaison with a local must be processed by the GP, often in the
encounter.
doctor who has referred a patient to the context of a 15-minute appointment. The
emergency department is essential. One outcome of this assessment may be the
should always respect the concerns raised subsequent referral to hospital level care.
by a referring local doctor who usually has Remember that the GPs decision is carefully
Management prior
the advantage of familiarity with the child considered with all the aforementioned fac-
to hospital care tors coming into play.
and family.
The GP is more often than not the point of Some of the more common reasons for
first contact for the potentially unwell child. referral to the ED may include the following;
The fundamental clinical medical tools of
THE ROLE OF THE history taking and examination are used to
a serious time-critical illness which
GENERAL requires ambulance transfer, such
make an initial assessment of whether the
PRACTITIONER IN as severe asthma, sepsis or
child can be treated in the community or
PAEDIATRIC meningitis;
requires referral to an emergency depart-
EMERGENCY ment for further opinion and management.
non-time critical illness which may not be
responding to community based
MANAGEMENT This can be a challenging task as the GP is
treatment and requires further
not afforded the luxury of observation over
investigation or parenteral antibiotics,
time, readily available ancillary testing such
such as evolving pneumonia;
as pathology and imaging, nor an immedi-
Introduction ate further opinion from a specialist col-
illness or injuries which are beyond the
level of facilities available to the GP to
General practitioners (GPs) are the corner- league. Particularly in the case of early or
manage, for example unstable limb
stone and often the childs first contact undifferentiated illness, the GP will need to
fractures;
within the Australian healthcare system. make a judgement call on whether or not
They are uniquely placed to have an inti- a child can be safely managed at home.
parental concern and anxiety which
cannot be sufficiently allayed by the GP;
mate working knowledge of the biological, Experienced GPs will not only use traditional
and
psychological and social dynamics that methods of history and examination but will
impact on a childs illness. also listen to their gut feeling when asses-
social factors whereby the child cannot be
adequately cared for or progress if
GPs are involved in the long-term care of sing children. This may involve attaching
monitored in the home setting due to lack
family members, often for many years and in importance to red flag symptoms or signs
of family resources.
some cases several generations. It is this or heeding the warning signs reported by
continuity of care and ongoing relationship an anxious, yet appropriately worried par- It is imperative once the decision has been
with a family that is invaluable in assessing ent. This may depend not only on the made to refer the child on to the emergency
and triaging presenting medical conditions medical status of the child but also the department, that the clinical assessment
in children and their subsequent manage- assessment of the social circumstances, and concerns of the GP are adequately com-
ment. This is particularly important when education and competence of the parents/ municated to the physician who will be the
caring for the health of children, who are carers to detect their child is failing to turn next link in the management chain. This is
often seen in the early prodromal phase of the corner or deteriorates. Often there is sig- best done with a phone call to the emer-
serious illnesses. It is the skill of the GP to nificant parental anxiety with an unwell gency department outlining the reasons

10
1.1 APPROACH TO THE PAEDIATRIC PATIENT
1

APPROACH TO THE PAEDIATRIC PATIENT

for referral. In potentially serious illnesses General practitioners can arrange further
Table 1.1.6 Normal milestones in first
the emergency department clinician can monitoring of the recovering child and is two years of life
instruct the GP in any necessary treatment well placed to arrange further tests (for
Neonate Lift head, visually fix for period
prior to transfer (for example, antibiotics example chest X-ray following complicated
and blood cultures in suspected meningitis pneumonia) or specialist follow-up if 6 weeks Smile, follow past midline

or sepsis). A referral letter which contains needed. Often the busy emergency depart- 4 months Roll over
the childs past medical history, allergies, ment is not the easiest place, especially after
6 months Sit, transfer toy between hands
immunisation status, list of current medica- hours, to arrange such important steps in the
tions and any relevant investigations should childs follow up care. The GP is also able to 9 months Stand holding on, crawl,
stranger anxiety
accompany the child to the emergency assess any psychological impact of the
department. This gives the treating doctor childs illness and offer ongoing support to 12 months Walking, single words

a head start in managing the child and the child and the family. These potential 18 months Explorer (trauma/poisons),
avoids wasted time, effort and cost in repeat- issues may not be evident at the time of tantrums, several words

ing already established findings. the emergency department visit. 2 years Combine words, run, jump
Integral to the communication process is
a mutual respect between the GP and the
emergency department physician with both
having a respectful understanding and
may challenge the assessment, so it is impor-
appreciation of the environment and chal-
Management after hospital tant to adapt the approach to these
lenges that each is working under. General
care practitioners have strong attachments to
expected behaviours. Significant deviations
from normal warrant consideration of pae-
Once the child has been managed and dis- their patient and families and will appreci-
diatric referral. Useful early milestones are
charged from the emergency department ate a follow-up phone call and/or letter
shown in the Table 1.1.6.
the circle of communication should include advising of the status of a referred child.
verbal and written feedback to the referring The letter should be timely, with appropriate
GP. This timely discharge communication information including diagnosis, medication
contact has several benefits. First and fore- and results of investigations with an access
phone number for any results pending. It
Growth
most it ensures continuity of care for the
child. If a treatment plan has been com- should be presented in a clear concise form It is essential to measure a childs current
menced by the hospital staff, the GP is then with a structured plan of management. weight on every emergency department visit
responsible for its implementation through Computer-generated letters are often more in order to accurately dose any therapeutic
continuing clinical assessment and adjust- legible than hand written ones and reduce drug and to quantify recent weight loss.
ment of management according to progress. the chance of miscommunication in the dis- Where a child is critically unwell and unable
The natural history of illness and convales- charge process. Some GP clinics now have to be weighed, estimation can be made via
cence are dynamic processes which will vary secure email availability and may prefer to Broselow or other charts. Between the age of
from patient to patient and may require receive information this way. one and ten years an estimation of weight is
vigilant monitoring. This is most likely to be If these strategies are implemented 2
(age 4) kg. Standardised percentile
successful if the discharge plan is well com- within a spirit of co-operation between growth charts are useful to confirm suspicion
municated to the family doctor. It is GPs and emergency departments, this will of failure to thrive or discrepancy in linear or
vital that communication is not mislaid ensure improved continuity of care and cranial growth. The trend of growth plotted
compromising patient care. The GP should therefore better patient outcomes in the on a growth chart over time is more impor-
receive information directly (fax or electronic) care of sick children. tant than a single measurement. As a gen-
as well as via the patient or family as a back eral rule, birth weight doubles by five
up if the usual communication systems fail. months, and triples by one year. Newborns
Secondly, medical practitioners continue are often discharged from hospital in the
to accumulate knowledge and expertise first few days of life and may present in
throughout their careers so that reflective
Developmental milestones the first week to an emergency department.
and sensitive feedback concerning outcomes It is important to have an understanding of Following the expected initial weight loss,
of their referred patients is useful. This helps the major developmental milestones term babies should normally regain their
the GP to analyse and reflect upon their throughout childhood for the provision of birth weight by the end of the first week.
decision making processes and contribute care to paediatric patients. These can be Appropriate neonatal weight gain is an
to their evolving clinical acumen, which is rapidly confirmed by examination or paren- important index of wellness. Head circumfer-
a career long journey for all doctors. This tal enquiry. This allows one to use an appro- ence increases by 2 cm in the first three
is particularly so in the case of paediatrics priate age-modified approach in the childs months, 1 cm in the next three months, fol-
as recognising the potentially unwell child evaluation. Some specific behaviours, such lowed by 0.5 cm per month thereafter
can sometimes be as much art as science. as stranger anxiety in a 12-month-old, (Table 1.1.7).

11
1.1 APPROACH TO THE PAEDIATRIC PATIENT

thought before I went, but I was proved

Table 1.1.7 Estimated normal growth
quite wrong. For a start, the doctors and
Age Weight (kg) Height (cm) Head circumference (cm) nurses actually spoke English (well most
Birth 3.5 50 35 of the time) which made me feel more
comfortable, being actually able to
1 year 10 75 47
understand them. And they were all very
2 years 13 88 49 friendly and smiling at you. In fact, the
>2 years 2 kg yr1 6 cm yr1 N/A complete opposite to the stereotype
storybook of needle brandishing doctors. I
Table adapted from Fleischer and Ludwig, Textbook of Paediatric Emergency Medicine.10
was so glad the corridors werent thickly
caked in white, but were dappled with
different colours and pictures. Entering a
Immunisation REFLECTION ON THE place all white makes you feel quite
It is not the role of the emergency depart- THOUGHTS OF nauseous, so I was glad for the variety.
ment to provide routine immunisations to CHILDREN AND One thing I was a bit overwhelmed by was
children. It is useful, however, to clarify a PARENTS IN ED the size. To me, the ED was massive and I
childs immunisation status with regard to was amazed that it was only part of the
the possibility of a particular infection such The emergency doctor can gain much from hospital. I was so relieved that the ED had
as epiglottitis, whooping cough or measles. the reflection of the insight of children and such a cheerful atmosphere that I wasnt
Immunised children can, however, manifest parents who have experienced the journey really worried at all and I think thats what
a modified form of these infections. In chil- through the emergency department . . . all hospitals should be like.
dren who are found to be incompletely or remember that every emergency depart-
non-immunised, it is opportunistic to provide ment patient may reveal important lessons
information regarding the normal vaccina- in our growth as care givers.
tion schedule and refer to the local doctor Thoughts on the emergency
or appropriate community facility for follow department through the
up (Table 1.1.8). eyes of a parent
The thoughts of a child
Anytime we have to take Aiden in to see
during the emergency
the doctor we have to sedate him before
Vital Signs department experience we go . . .
It is necessary to interpret the vital signs What comes to mind when I hear the No white coats or uniforms. . .
according to the age of a particular child. word hospital? Huge needles, white every Its so nice if the doctor takes time to
A wall chart in the paediatric resuscitation thing, people running around speaking talk to the parents first, not so clinical but
area is a useful reference as a guide to these doctors language. Well, thats what I to chew the fat with the parents so the
parameters. A good rule to remember is any
child with a persistent respiratory rate >60
or heart rate >160 is definitely abnormal Table 1.1.9 Normal vital signs
(Table 1.1.9).
Age Weight (kg) RR (per min) HR (per min) sBP (mmHg)

Birth 3.5 4060 100170 50

Table 1.1.8 Australian standard
vaccination schedule (05 years)* 3 months 6 3050 100170 50

Age Vaccine 6 months 8 3050 100170 60

Birth HepB 1 year 10 3040 100170 65

2 months DTP, OPV, Hib-HepB,7vPCV 2 years 13 2030 100160 65

4 months DTP, OPV, Hib-HepB, 7vPCV 4 years 15 20 80130 70

6 months DTP, OPV, 7vPCV 6 years 20 16 70115 75

12 months MMR, Hib-HepB, MenC 8 years 25 16 70110 80

18 months DTP 10 years 30 16 60105 85

25 years MenC 12 years 40 16 60100 90

4 years DTP, OPV, MMR 14 years 50 16 60100 90

*This varies between states. Adapted from RCH, Melbourne Clinical Guideline Site.11

12
1.1 APPROACH TO THE PAEDIATRIC PATIENT
1

APPROACH TO THE PAEDIATRIC PATIENT

child doesnt get scared when needs before they see them in the helped the doctor with how to explain
approached. . . hospital. . . things to my child without causing all that
Its so much better if the doctor sits at My child was unwell, miserable and fear. . .
the childs level so they dont feel like an clinging to me. The doctor was rough and
adult is standing over them. . . tried to examine him instead of letting
If the doctor is a nervous person, my him settle and coming back later when he
child picks it up and this gets them settled. . .
nervous. . . Its so frustrating when I tell doctors my References
1. Durojaiye L, OMeara M. A study of triage of paediatric
If the child says no during the child wont take oral medications and they patients in Australia. Emerg Med 2002;14:6776.
examination, please dont push anything just give oral medicines for us to struggle 2. Luten RC. Recognition of the sick child. Problems in
paediatric emergency medicine. New York: Churchill
onto them. . .if they say no they mean no with at home, rather than considering a Livingstone; 1988. p. 112.
. . . give them some breathing space one-off injection which has helped in the 3. Browne GJ. Paediatric emergency departments: Old
needs, new challenges and future opportunities. Emerg
unless its life threatening of course . . . past. . . Med 2001;13:40917.
Dont have clinical stuff like needles, It gives us so much more confidence in 4. Browne GL, Gaudry PL. A triage observation tool
improves the reliability of the National Triage Scale in
sharp containers visibly around or the doctor if he explains what hes looking children. Emerg Med 1997;9:283338.
anything the child has a dislike for. . . for during the examination, rather than 5. Browne GJ, Chong RKC, Gaudry PL, et al. Principles and
practice of childrens emergency care. Sydney: McLennan
Perhaps a sedative prior to going to just saying our child is fine when were and Petty; 1997. p. 15.
surgery again, as the next time he went worried. . . 6. Waskerwitz S, Berkelhamer JE. Outpatient bacteraemia:
Clinical findings in children under two years with initial
for surgery he freaked out and it was so I like it when the doctor involves me temperatures of 39.5 C or higher. J Paediatr 1981;99(2):
bad. He remembered everything when he during the examination of my child so I 2313.
7. McCarthy PL, Sharpe MR, Spiesel SZ, et al. Predictive
entered the room and everyone was can distract their fear during the observation scales to identify serious illness in febrile
standing around him in scrubs, Dad had to examination. . . children. Paediatrics 1982;70(5):8029.
8. Browne G, Penna A. Short stay facilities. The future of
hold him down whilst they gave him Some doctors only pretend to be efficient paediatric emergency services. Arch Dis Child
medication just to keep him on the hearing, but not listening to me when I tell 1996;74:30913.
9. Scribano PV, Wiley JF, Platt K. Use of an observation unit
bed. . .. Dad left crying. . . them Im worried about my child. . . by a paediatric emergency department for common
Dont talk in the corridor about the Im so much more confident when the paediatric illnesses. Pediatr Emerg Care 2001;17(5):
3213.
child near the parents if possible. . . doctor is thorough but at the same time 10. Fleischer GR, Ludwig S. Understanding and meeting the
The doctor/nurse talk can be insensitive interacts playfully with my child. . . unique needs of children. In: Fleischer GR, Ludwig S,
editors. Textbook of Paediatric Emergency Medicine.
to the child/parent even if the doctor/ I wish the doctor spoke to me 3rd ed Baltimore: Williams & Wilkins; 1993.
nurse dont realise it at the time. . . separately about the scary operation 11. Royal Childrens Hospital. Clinical practice guidelines
resuscitation: Emergency drug and fluid calculator.
It would be great if the staff have some details and then gave a non-fearful Melbourne, Australia, 2003. [http://www.rch.org.au/
sort of experience with a child with special explanation to my child. I could have clinicalguide/cpg.cfm?doc-id5162].

13
 1.2 The crying infant
Barbara King

crying was no longer tolerable was effective

ESSENTIALS when compared to a non-specific empathic
interview.4,7 Reduction of stimulation advice
1 Define if this presentation is part of a recurrent stereotypical pattern in an includes avoiding excessive patting, winding,
otherwise well infant, or a single acute episode.
lifting, vigorous jiggling and loud noises or
2 A careful history and examination will often lead to an appropriate diagnosis. toys. Carers were advised not to intervene
in the early part of sleep when the infant
3 Screening tests, with the exception of urine culture, have little utility. may appear restless and also given an assur-
4 Review carefully carers coping and supports. ance that a certain amount of crying is nor-
mal.4,7 It is important to remember that
5 Organise appropriate follow up. even if behavioural interventions do not
change the infants temperament, they may
well alter the impact colic has on the carer
3 weeks.4 This pattern typically occurs in
Introduction and on carerinfant interactions.8
the afternoon or evening, ceasing by 3 to
Carers very reasonably assume that there
Crying is an important method of communi- 4 months of age.3
must be something wrong either with the
cation for infants.1 Carers are usually able to
child or their parenting for an infant to cry
identify and manage the cause (e.g. hunger,
Management frequently and excessively. Much reassur-
discomfort) and console the infant. Medical
Current specific dietary, drug and beha- ance that the child is healthy can be gained
advice is sought if the crying is felt to be
vioural strategies meet with limited success; from witnessing the conduct of a complete
unusually intense or persistent or the infant
however, cows milk protein intolerance may history and thorough examination. Similarly,
is unable to be consoled by the usual meth-
have a role in a small proportion of an explanation that this is a common prob-
ods.2 Normal crying was defined in Brazel-
infants.4,5 The positive effects of changing lem that does not reflect on their parenting,
tons 1962 study of 80 infants of American
from cows milk based formula to casein that some babies may be assisted by some
families selected to have minimal psycholog-
hydrolysate formula have been noted to simple behavioural techniques and that the
ical stressors. There was a peak of crying to a
diminish with time, suggesting that colic is carers will be supported by appropriate refer-
median of 23/4 hours per day by 6 weeks, with
not related to allergy.5 A trial of hydrolysate ral will also reduce anxiety considerably.
a wide variation, and a decrease thereafter.1
(e.g. Pepti-Junior, Alfare) may be appropri-
The classification of crying as normal or
ate in a formula-fed baby but its long-term
excessive is highly subjective and will vary
use is likely to be unnecessary. It is notable
according to infant, carer and situational fac- Acute crying
that soy protein formulas may have
tors. All three areas need to be assessed in
similar adverse effects to those of cows The causes of a single episode of excessive
this type of presentation.
milk formulas.5 Mothers of breastfed babies crying in an infant are vast. In an afebrile
Either a single episode of or recurrence of
can exclude all dairy products (but ensure infant without a cause apparent to the
a pattern of excessive crying may precipitate
calcium supplementation) for 2 weeks as a carer, a careful history has been shown to
emergency department (ED) presentation.2
trial. provide clues to the final diagnosis in 20%
Gastro-oesophageal reflux (GOR) is fre- of cases. Physical examination was revealing
quently cited as a possible cause of infant in more than 50% and a period of follow up
crying. Feeding difficulties and frequency often useful in patients where the diagnosis
Recurrent crying was still in question.2
of regurgitation (> five times daily) are asso-
Colic ciated with pathological GOR as defined on
Recurrent excessive crying in an otherwise oesophageal pH monitoring there is a Assessment
healthy infant is often termed colic. This place for an empirical trial of proton pump History includes the timing and amount of
diagnosis can only be made if the pattern inhibitors in this group.6 crying, duration of behaviour, measures
is recurrent and stereotypical3 and a careful Anticolic medications should be avoided, taken to resolve the situation, specific carer
history, examination and period of follow up as they have been shown to at best have concerns, the carers response to crying,
have ruled out important causes. The defini- no effect (simeticone) or risk serious adverse expectations and experience, specific social
tion of colic varies but is frequently arbi- effects (anticholinergics).4 difficulties (including substance abuse), con-
trarily defined as a total of more than Behavioural interventions, including advice tact with child health nurse or other medical
3 hours per day of irritability, fussing and to reduce stimulation in combination with supports. A thoughtful review of the carers
crying on at least 3 days a week for at least permission to leave the infant when the supports and coping is essential.9

14
1.2 THE CRYING INFANT
1

APPROACH TO THE PAEDIATRIC PATIENT

Also important are details of pregnancy, (otitis media), oropharynx (herpes stomatitis, to allow recovery of a sleep-deprived or
labour and neonatal difficulties, feeding tonsillitis, upper respiratory tract infection). poorly supported carer.9 The involvement
activities including volumes of feed (consid- Several additional manoeuvres may also of social work services is appropriate under
erations include under and overfeeding), assist:2 circumstances where the family or child is
dietary changes, past medical history, vomit- considered at risk. Serious injury to children
ing (gastrointestinal reflux, gastroenteritis,
fluorescein staining of cornea (corneal by non-accidental shaking injury is preceded
abrasion);
sepsis, meningitis). Consider changes in by other episodes of abuse or neglect in over
stools (constipation, gastroenteritis, bleed-
palpation of long bones (fractures,
70% of cases.10 Admission and social work
osteomyelitis);
ing anal fissure, intussusception), type of assessment is always warranted if non-acci-
feeding (breast or bottle), type of milk, drug
inspection of skin beneath clothing
dental injury or neglect is suspected.
(bruising);
exposure, recent immunisation, fever, respi- Ensure appropriate follow up is organised
ratory symptoms, rash, contacts with infec-
retinal examination (shaken baby
for infants discharged from the ED partic-
syndrome);
tious illness and growth history. ularly where the diagnosis is not yet clear or
close attention to digits and genitalia
where excessive crying is likely to be
(hair tourniquet).
recurrent.
Examination
A complete set of vital signs is essential, Investigations
including oxygen saturation (tachypnoea Investigations should be guided by initial References
with pneumonia, sepsis or metabolic acidosis, clinical assessment. Screening investiga- 1. Brazelton TB. Crying in infancy. Pediatrics 1962;
tachycardia with supraventricular tachycar- tions, with the exception of urine analysis (April):57988.
2. Poole SR. The infant with acute, unexplained, excessive
dia, sepsis or dehydration, desaturation with and culture, have little utility.2 crying. Pediatrics 1991;88(3):4505.
pneumonia or bronchiolitis). Examination 3. Illingworth RS. Three months colic. Arch Dis Child
1954;16574.
should include tone and activity, alertness/ 4. Lucassen PLBJ, Assendelft WJJ, Gubbels JW, et al.
conscious state (meningitis, encephalitis, Effectiveness of treatments for infantile colic: systemic
sepsis, metabolic crisis, hypoglycaemia, elec-
Disposition review. BMJ 1998;316:15639.
5. Forsyth BWC. Colic and the effect of changing formulas: a
trolyte disturbance), perfusion, hydration, The diagnosis of serious medical conditions double blind, multiple-crossover study. J Pediatr
1989;115:5216.
fontanelle (bulging with infection or trauma, during the initial clinical assessment will 6. Heine RG, Jordan B, Lubitz L, et al. Clinical predictors of
sunken in poor feeding and dehydration), clearly indicate admission. Admission should pathological gastro-oesophageal reflux in infants with
persistent distress. J Paediatr Child Health
assessment of chest for respiratory distress, also be considered in those cases where the 2006;42:1349.
(pneumonia, bronchiolitis, metabolic acido- clinical assessment is normal but the child 7. McKenzie S. Troublesome crying in infants: effect of
advice to reduce stimulation. Arch Dis Child
sis, cardiac failure). An abdominal examina- continues to cry excessively in the ED 1991;66:141620.
tion (herniae, testicular torsion, evidence of beyond the time of the initial assessment. 8. Carey WB. The effectiveness of parent counselling in
managing colic. Pediatrics 1994;94(3):3334.
surgical abdomen), cardiovascular examina- Persistent crying in these circumstances 9. Singer JI, Rosenberg NM. A fatal case of colic. Pediatr
tion (murmurs, femoral pulses, cardiac may be an indicator of serious illness.2 Emerg Care 1992;8(3):1712.
10. Alexander R, Crabbe L, Sato Y, et al. Serial
ischaemia due to aberrant coronary vessels Occasionally it may be necessary to admit abuse in children who are shaken. AJDC
is a reported but extremely rare cause), ears an infant with colic or minor medical problem 1990;144:5860.

15
SECTION

2 RESUSCITATION
Section editor Ian Everitt

2.1 Paediatric cardiopulmonary arrest (CPA) 16 2.4 Specific paediatric resuscitation 38

2.2 Paediatric basic life support 20 2.5 Shock 41
2.3 Paediatric advanced life support (PALS, APLS) 25 2.6 Neonatal resuscitation 45

2.1 Paediatric cardiopulmonary arrest (CPA)

Christopher Webber

achieving return of spontaneous circulation

ESSENTIALS (ROSC), 24% surviving to hospital admis-
sion, and 12% surviving to discharge.6
1 Prevention is the link in the Chain of Survival key to decreasing the incidence of Less than 10% of paediatric OHCA victims
death from Out-of-Hospital Cardiac Arrest (OHCA)1
have a shockable rhythm on arrival of the
2 Paediatric patients who sustain OHCA have similar survival to hospital discharge prehospital care providers.4,5
rates to adults who sustain OHCA.
3 CPA in children usually results from the development of progressive hypoxia and/ Aetiology
or shock, which may be due to a myriad of causes.
The most common causes of cardiac arrest in
4 The goal is to identify evolving hypoxia and shock early in seriously ill children children relate to the development of hypoxia
and proactively instigate appropriate therapy to prevent the progression to cardiac and/or shock, the two pathways which,
arrest. untreated, result in cardiopulmonary arrest.
5 In contrast to adults, the most common arrest rhythm in children is non-VF/VT and Shock is the clinical state of poor tissue
is usually either asystole, pulseless electrical activity (PEA) or electromechanical perfusion. Thus, the primary organ systems in-
dissociation (EMD). volved are the respiratory and cardiovascular
systems, and the central nervous system
6 Resuscitation organisations have developed guidelines for paediatric patients (CNS), which becomes compromised by hyp-
to include nuances of the newly-born, infant, young child and older child. oxia or decreased cerebral perfusion. Injury
Current recommendations simplify the differences in resuscitation approach or diseases progressing to failure of these
between ages. systems may then lead to cardiac arrest. CNS
7 Allowing parents to be present and supported during the resuscitation of their disorders, either with direct dysfunction or
child may be associated with a better long-term psychological outcome. due to raised intracranial pressure, may cause
respiratory arrest with the resulting hypoxia
8 Unless CPA is associated with drug toxicity or some situations with profound leading to cardiac arrest. The primary causes
hypothermia, neurologically intact survival is unlikely after 30 minutes of full CPR and of cardiac arrest are heterogeneous and in-
several doses of adrenaline. clude trauma, sepsis, drug overdose, poisoning,
immersion, critical respiratory illness and
sudden infant death syndrome (SIDS). This
reporting a similar rates of survival to hospi- list is by no means complete, and many other
Epidemiology tal discharge as that of adults who sustain conditions may also progress to cardiac arrest.
Previous studies of paediatric Out-of- OHCA.4,5
Hospital Cardiac Arrest (OHCA) have re- The incidence of out-of-hospital arrest is
ported poor survival rates with severe reported as ranging from 2.6 to 19.7 per
Pathophysiology
neurological sequelae,2,3 however more year per 100 000 paediatric population Significant hypoxic and hypoperfusion
recent publications have challenged this (age < 18 years), in the region of 30% insults to the myocardium are the common

16
2.1 PAEDIATRIC CARDIOPULMONARY ARREST (CPA)
2

RESUSCITATION
pathways that may progress to cardiac or pulseless ventricular tachycardia, hence arrest and to the resuscitation techniques
arrest in children. Hence, fluid or blood loss the time to defibrillation is the single greatest subsequently required (Table 2.1.1).
leading to shock, if left untreated, result in determinant of survival. Thus the phone first
myocardial ischaemia and resultant cardiac principle that applies to adults is not applica-
arrest. Similarly, severe hypoxia also results ble to most infants and children, in whom
Development of
in global myocardial dysfunction and sub- the response should be phone fast (see
sequently cardiac arrest. Thus, preventive Chapter 2.2 on Basic life support).
resuscitation guidelines
strategies must identify hypoxia and shock There are several anatomical and physical In 1992 the American Heart Association
early and instigate appropriate manage- differences between children and adults. It is guidelines for resuscitation were published.
ment to improve oxygenation and organ important to consider these differences in Subsequently representatives of seven resus-
perfusion, thereby preventing progress to relation to the primary event leading to citation councils throughout the world,
cardiac arrest. Poisonings or ingestions may
have a direct effect on the heart, causing an
arrhythmia, coma, with subsequent respira-
tory arrest and hypoxia. Table 2.1.1 Important differences between children and adults

Difference in children Implication

Outcome AIRWAY
Prominent occiput tends to cause neck flexion Neck extension, into a neutral or sniffing position
Generally, the survival from respiratory (slight extension), is required to optimise the airway
for an infant or child respectively
arrest alone is much better than from cardio-
pulmonary arrest. Survival to discharge for Mandible is relatively smaller More difficult intubation
children with respiratory arrest (pulse pres- Tongue is relatively larger Tends to obstruct airway
ent) is around 75%, and of these up to More difficult intubation
88% have a good neurological outcome. Larynx is more cephalad (located almost at base of More difficult intubation - tendency for
Reported survival rates from cardiac arrest tongue) inexperienced operator to insert laryngoscope
blade into oesophagus
in children have varied from 0% to 17%.
Survival to discharge from hospital for Epiglottis is proportionally larger and more floppy Intubation may require straight-bladed
laryngoscope to lift epiglottis forward to allow
paediatric OHCA is 7%4,5 and 36% for in- visualisation of vocal cords
hospital cardiac arrests.7 Survival to dis-
Upper airways are more compliant (i.e. distensible) Tend to collapse during increased work of
charge is a very crude marker of success breathing
as it does not include a measure of neurolog-
ical function. Proactive early resuscitation BREATHING

of the pre-arrest child is important in order Chest wall more compliant (particularly the Less efficient ventilation, when increased work
newborn infant and more so the preterm infant) of breathing
to have the most impact on outcome. Earlier fatigue
Unfortunately, the perception of the pub-
Greater dependence on diaphragm to generate Distended stomach impairs ventilation
lic, and even doctors and nurses, is that the tidal volume Importance of venting stomach with gastric tube
expected survival rate is higher than this.
CIRCULATION
Lay rescuers, physicians and nurses estimate
the survival rate for cardiopulmonary arrest Maintains cardiac output and blood pressure by Diagnose and treat shock before hypotension
tachycardia initially develops
in children as being 63%, 45% and 41% Hypotension usually indicates late
respectively (compared to 53%, 30% and decompensation

24% for adult cardiopulmonary arrest).8 GENERAL

Undoubtedly, fictional medical television Head has proportionally greater component of Loss of body heat during primary event or
programmes contribute to this bias, and body surface area resuscitation
Greater chance of head injury
even non-fictional medical programmes
rarely show death as an outcome. Compliant chest wall allows transmission of energy Pulmonary, hepatic and splenic injury may occur
to underlying organs, resulting in traumatic without overlying rib fractures
damage/rupture, rather than dissipation of energy

Differences compared Development

Language
Must be considered when interacting with the
child and understanding injuries (accidental versus
to adults Motor development (fine and gross) non-accidental)
Social and cognitive development (including
When comparing children to adults in relation abstract thinking)
to cardiopulmonary arrest, there are several Parental and staff considerations Psychosocial issues
important differences. The aetiology of the Presence of family during resuscitation
Staff pressure to continue resuscitation
event is usually different. Adults who collapse Impact on staff from death of child
are more likely to have ventricular fibrillation

17
2.1 PAEDIATRIC CARDIOPULMONARY ARREST (CPA)

including the Australian Resuscitation Coun- this opportunity and despite the occasion- Termination of resuscitative
cil, formed the International Liaison Com- ally chaotic environment, common positive efforts
mittee on Resuscitation (ILCOR). ILCOR perceptions relate to the efforts made by The decision to cease resuscitation efforts in a
advisory statements were produced. A sub- staff. To achieve a positive (psychosocial) child in CPA is influenced by many factors.
committee on paediatric resuscitation with outcome, in relation to the presence of par- These include the total arrest time, clinical
representation from the American Heart ents during resuscitation, the following response to therapy, premorbid state of the
Association and other paediatric represen- issues should be considered: child, potential for any reversible factors, likely
tatives from ILCOR (Paediatric Working neurological outcome, information from col-
Group) further developed guidelines for
Parents being present during the leagues who care for a child with long-term
resuscitation of their child should be
paediatric patients. This group attempted medical problems and the parental wishes.
regarded more as the norm than the
to evaluate the level of evidence for resus- In the initial stage of the arrested child arriving
exception.
citation recommendations. It was impor- in the ED, these details should be rapidly estab-
tant to attempt to avoid confusion
Parents should not be coerced into being
lished whilst resuscitation is continued in
present, but gentle encouragement and
between adult, paediatric and neonatal order to help guide subsequent management.
explanation by a senior member of staff is
algorithms and ultimately the International Termination of resuscitation in a newly
usually helpful.
Guidelines 2000 were published. These born baby is likely to be appropriate if the
were revised and published simultaneously
Senior members of staff engaged in
baby remains in cardiorespiratory arrest at
resuscitation should not feel undue
in the journals Resuscitation and Circula- 15 minutes. Even after 10 minutes of docu-
pressure as a result of the attendance of
tion in 2005 and again in November mented asystole, survival without severe
parents. Occasionally, however, it may
2010.9 The Australian Resuscitation Coun- disability is unlikely.
be preferable for the parents to wait
cil (ARC) and New Zealand Resuscitation For children in established cardiac arrest
outside the immediate resuscitation area
Council (NZRC) released updated guide- the overall outcome is poor. If the child
during challenging or visually disturbing
lines in December 2010. For the first time, requires adrenaline (epinephrine), and fails
procedures.
both Councils published identical guide- to respond to two doses, then survival is
lines on-line, with both Councils logos. Sub-
Parents should have a dedicated support
unlikely. Generally no longer than 30 min-
person, being a health professional, to
sequently resuscitation courses, like the utes of advanced life support resuscitation
remain with them throughout the
Advanced Paediatrics Life Support (APLS) is required to determine whether discon-
resuscitation. The support person should
course have been amended to be consis- tinuation of resuscitation is appropriate.
obtain information from medical staff to
tent with the international recommenda- Recurring/refractory VF or VT, toxic drug
keep the parents informed of their childs
tions. APLS courses in Australia and New exposure or the presence of significant hypo-
progress and explain procedures to the
Zealand are consistent with ARC and NZRC thermia in the setting of ice-cold immersion,
parents.
recommendations. are situations that may require more prolon-
Touching and talking to their child is
ged resuscitation efforts (see Chapter 22.2
often important for the parents where
on Drowning). Many children in cardiores-
this is feasible.
Ethics of paediatric piratory arrest in Australia and New Zealand
Parents need to have a quiet room to
resuscitation retreat to, if necessary. Discussion with
who are hypothermic, however, have lost body
heat due to exposure, without spontaneous
Presence of family family should not occur in a busy
circulation after the arrest, and therefore this
Over recent years, the benefit of allowing emergency department corridor as it will
is unlikely to be neuroprotective.
the family, mainly parents, into the resus- negatively affect the familys retention of
citation room during active resuscitation information.
has become clearer. This practice has Family requests and religious beliefs need Non-initiation of resuscitative
required a cultural change, and mandates to be respected. efforts
appropriate and professional behaviour Obligations mandated by Coronial Law Resuscitation should not be initiated if there
and language during resuscitation. Such need to be explained with sensitivity. are signs of prolonged death, like rigor mortis
practice should occur regardless of the The emotional impact on all staff or post-mortem lividity. Children who are in
presence of any outside witnesses. It is members when a child dies is a palliative phase of care should have an
a professional standard that all medical, considerable and this may be end of life care plan that includes non-
nursing and other health professionals compounded by the distress of initiation of resuscitation should this have
should aspire to and maintain. It is prob- parents during and following the been previously determined by family and
ably even more important that parents resuscitation. the childs coordinating specialist. Children
are offered the opportunity to witness With any resuscitation, particularly those with complex and disabling conditions will
the resuscitation, when the outcome is resulting in death, debriefing of staff is occasionally have life-threatening events
the death of their child. essential, as is organising the appropriate occur. If prior discussions between parents
In hindsight, parents who have witnessed support and medical follow up of the (and child if appropriate) and physicians have
the resuscitation of their child have valued parents. not included limitations of interventions or

18
2.1 PAEDIATRIC CARDIOPULMONARY ARREST (CPA)
2

RESUSCITATION
medical support, then it is difficult to avoid abuse or neglect, appropriate notification Some states have special processes in
commencing resuscitation. Early and urgent should be made. place for the management of sudden unex-
consultation with the childs primary physi- plained death in infancy (SUDI). Any sudden
cian is prudent in these situations. Physicians Organ donation and unexplained death under 12 months of
should not be coerced into undertaking care Organ donation is usually a consideration for age warrants a detailed history as well as
that they believe is morally wrong or fruitless. staff when a child is in an intensive care specific samples collected post-mortem to
There are specific situations in the newly unit and generally not in the emergency identify metabolic and genetic conditions.
born baby that may lead to the non- department. The only situation where it The Forensic Pathologist may need to have
initiation of resuscitation, like extreme may be contemplated in the ED is when this drawn to their attention (e.g. see
prematurity and congenital/chromosomal post-mortem organs (like corneas, heart http://www.health.nsw.gov.au/policies/
abnormalities not consistent with long-term valves) may be retrieved for this purpose. index.asp Search SUDI).
survival. This issue is covered in Chapter 2.6 In cases mandating notification of the coro- In the situation where a death does not
on neonatal resuscitation. ner, then permission is required prior to any require notification to the coroner, it must
organ harvesting. Liaison with the appropri- be clarified who can and will complete the
ate local donor coordinator is essential. death certificate, which includes the cause
Consent
A senior member of staff should have this of death. Consultation with the primary phy-
The philosophy of informed consent should
discussion with the parents and occasionally sician involved with the long-term care of a
be followed during any treatment, including
the parents themselves raise the issue. child with chronic illness is obligatory. Medi-
resuscitation. The patient or the parents/
If resuscitation has resulted in the return cal staff need to be aware of other legal obli-
guardian should have an explanation about
of spontaneous circulation but with likely gations, like the collection of blood alcohol
the management (current and proposed),
brain death, subsequent organ donation specimens in pedestrian and vehicular acci-
risks and potential outcomes. This may be
may be feasible. The determination of brain dents, but these would only be collected
limited during resuscitation but is the
death would usually occur in an intensive ante-mortem by hospital staff. These legal
responsibility of the most senior physician
care unit, as a repeat examination for brain requirements vary between states.
involved with the childs care at the time.
death, after a period of time, is required.
The decision to stop resuscitative efforts in
Consultation with and consideration of
the emergency department setting due to
transfer to a paediatric intensive care unit
a lack of return of spontaneous circulation References
is required. The focus in the emergency
is a medical decision. It is not a choice that 1. Nolan J, Soar J, Eikeland H. The chain of survival.
department at this point is the ongoing care Resuscitation 2006;71(3):2701.
is offered to the parents. The circumstances
of the child and support of the family. 2. Schindler MB, Bohn D, Cox PN, et al. Outcome of out-of-
are completely different to the discussion hospital cardiac or respiratory arrest in children. N Engl
with parents about withdrawal of treat- J Med 1996;335(20):14739.
Death certificates, notification to 3. Young KD, Gausche-Hill M, McClung CD, et al. A
ment, which usually occurs in an intensive prospective, population-based study of the epidemiology
the coroner and other legal issues and outcome of out-of-hospital pediatric cardiopulmonary
care unit in a child who is ventilated but
Death in the ED is, by itself, not an indication arrest. Pediatrics 2004;114(1):15764.
has a spontaneous circulation. 4. Atkins DL, Everson-Stewart S, Sears GK, et al. Epidemiology
to notify the death to the coroner. Coroners and outcomes from out-of-hospital cardiac arrest in
Acts vary from state to state and staff must children: the Resuscitation Outcomes Consortium Epistry-
Cardiac Arrest. Circulation 2009;119(11):148491.
Non-accidental injury be aware of their statutory obligations. 5. Deasy C, Bernard SA, Cameron P, et al. Epidemiology of
It is important that all healthcare providers Remember that if a death is to be notified paediatric out-of-hospital cardiac arrest in Melbourne,
Australia. Resuscitation 2010;81(9):1095100.
involved with children should be aware of to the coroner the body then becomes evi- 6. Donoghue AJ, Nadkarni VM, Elliott M, Durbin D. Effect of
their statutory obligations under state or dence and should be left intact at the termi- hospital characteristics on outcomes from pediatric
cardiopulmonary resuscitation: a report from the national
federal law regarding notification of suspi- nation of resuscitation. Common practices of registry of cardiopulmonary resuscitation. Pediatrics
cion of non-accidental injury or neglect. taking hand/footprints, locks of hair, remov- 2006;118(3):9951001.
7. Tibballs J, Kinney S. A prospective study of outcome of in-
Awareness of development of the child, fea- ing catheters and tubes must not occur or patient paediatric cardiopulmonary arrest. Resuscitation
sibility or compatibility of the history with staff risk being held in contempt of court. 2006;71(3):3108.
8. Brown K, Bocock J. Update on paediatric resuscitation.
clinical signs and any delay in presentation Such mementos can be collected following Emerg Med Clin North Am 2002;20(1):126.
should be considered when assessing this the post-mortem examination. This usually 9. International Consensus on Cardiopulmonary
Resuscitation and Emergency Cardiovascular Care Science.
possibility. Regardless of the outcome of requires liaison with staff in the forensic Circulation 2010;122:18 Supplement and Resuscitation
the resuscitation, if there is a suspicion of mortuary. 2010;81 Supplement.

19
 2.2 Paediatric basic life support
Jane Cocks

The currently accepted international rec-

ESSENTIALS ommendation for the commencement of
external cardiac compressions (ECC) in chil-
1 Paediatric cardiopulmonary resuscitation is different to adult resuscitation in dren of all age groups who are unresponsive
many aspects, but the basic principles remain the same:
with no spontaneous breathing is a heart
Dont panic rate below 60 beats per minute (bpm).

Get help
Anatomy and physiology
Ensure both the patient and you are safe The anatomy and physiology of the human
Initiate basic life support at the earliest possible moment: Airway, Breathing, body changes markedly with age, progressing
Circulation. from the immature neonatal form to the more
adult form of the older child, and these
2 Stabilise the cervical spine if the collapse follows any significant trauma. changes are relevant to the provision of BLS
3 DRSABC is a mnemonic to aid the sequence of events that should be followed when at any particular age. The following differ-
met with any collapsed patient. Check for Danger, Responsiveness, Shout for help, ences from the adult form are of direct impor-
Open the Airway, Check for Breathing and if patient is not breathing normally give tance in the provision of BLS in children:
5 rescue breaths, if there are no signs of life commence Chest compressions.
Airway: Large head (particularly the
4 Quality chest compressions involve compressing the chest to at least one third of occiput) and short neck leading to neck
the antero-posterior chest diameter at a rate of 100 compressions per minute with flexion; large tongue which is
subsequent complete release to allow recoil of the chest wall. predominantly intra-oral; easily
compressible oropharyngeal soft tissues;
5 Lone rescuers should use a 30:2 compression to ventilation ratio, while healthcare larynx lies more anteriorly and higher
professionals performing two-rescuer CPR should use a 15:2 compression to
(C23); cricoid ring is the narrowest
ventilation ratio for all children, except in newly born where it is 3:1.
part of the airway; soft and short
6 The rate of compressions is 100 per minute for all age groups, except in neonates trachea.
where it is 120 per minute. The chest should be seen to fully recoil before the Breathing: Increased respiratory rate;
next compression is commenced. more compliant chest wall; dependence
on diaphragmatic breathing; reduced end
expiratory lung volume.
Circulation: Increased heart rate; more
utilised follow the same general principles,
Introduction but paediatric BLS differs significantly from
dependence on heart rate for delivery of
adequate circulation; small absolute
Basic life support (BLS) or basic cardiopul- adult BLS in some very fundamental ways.
volume of circulating blood.
monary resuscitation (CPR) is a process by
Metabolism: Increased metabolic rate;
which an individuals basic cardiac and respi-
Aetiology of arrests increased body surface area (BSA).
ratory functions can be restored and main-
In adults the most common cause of cardio-
tained through a combination of expired
pulmonary arrest is a sudden, massive cardiac
air resuscitation (EAR) and external cardiac
event with little preceding deterioration.
compressions (ECC). The provision of BLS
Most adults requiring BLS measures are BLS techniques and age
sustains the vital functions of the collapsed
already in a state of full cardiopulmonary
individual without the need for specialised BLS techniques are modified depending on
arrest, defined as a state of pulseless apnoea.
equipment, minimising the potential hyp- the childs age and size to ensure that they
In children, the most common causes of
oxic damage that may occur while advanced will be maximally effective. In view of this,
cardiopulmonary arrest are hypoxic and, as
life support is being activated. children are arbitrarily divided into the fol-
a consequence, children generally undergo
lowing internationally accepted age groups:
a period of deterioration with worsening bra-
Paediatric versus adult dycardia preceding the final arrested state. It newborn/neonate: birth to 1 month old;
basic life support is therefore important that paediatric BLS is infant: between 1 month and 1 year
commenced as soon as this bradycardic dete- of age;
The aim of basic life support in all age rioration is noted and is not withheld until the small child: age between 1 and 8 years;
groups is the same and the techniques patient becomes pulseless. large child: age older than 8 years.

20
2.2 PAEDIATRIC BASIC LIFE SUPPORT
2

RESUSCITATION
and activation of the EMS should occur imme-
Preparation and equipment diately prior to commencement of BLS by a
Basic CPR requires no extra equipment lone rescuer. These cases require rapid access
other than personnel trained in its adminis- to Advanced Cardiac Life Support measures,
tration. It is advantageous, however, to have as is the case with most adult arrests.
available certain basic equipment to assist
in the resuscitation process. Assess responsiveness
Assess the responsiveness of the patient by
Portable suction and suction catheters, gently, but firmly, stimulating the patient
for clearing secretions, improve the ability and asking if they are OK. It is important
to achieve and maintain a patent airway. to remember the possibility of cervical spinal
An oropharyngeal (Guedel) airway may injury and, if this is likely, stabilise the cervi-
assist in achieving a patent airway. cal spine by placing a hand on the patients
A self-inflating bag and mask, suitable for forehead prior to gently shaking their arm. Fig. 2.2.1 Chin lift and head position in infants.
the size of the patient, improves artificial
ventilation and reduces the risk of cross
infection.
Airway
Oxygen for ventilation will further reduce
the risk of hypoxic injury. Open and maintain the airway
Position yourself at the patients head, open
the mouth and remove any obvious debris.
Do not use a blind finger sweep in children
Basic life support sequence as this may damage the delicate palatal tis-
A SAFE approach sues or move a foreign body further into the
It is extremely important to ensure maximum airway. Suction using a large-bore Yankeur
rescuer and patient safety as a priority on first catheter is useful for removing vomitus and
approaching the collapsed child. To this end, secretions, preferably under direct vision.
the Advanced Paediatric Life Support (APLS) Three manoeuvres will assist in opening
course advocates a SAFE approach to col- and maintaining the airway (Figs 2.2.1,
lapsed patients. This acronym stands for: 2.2.2 and 2.2.3), which is most commonly
obstructed by the childs tongue.
S Shout for help. Call for assistance
from surrounding people and request that Head tilt
an ambulance is called, do not leave the Place one hand on the patients forehead Fig. 2.2.2 Head tilt and chin lift in children.
patient as immediate resuscitation may and gently tilt the head back. In children
be required. the sniffing position is desired, as in adult
A Approach with care. Ensure that you resuscitation. In an infant or neonate, a
are not putting yourself at risk. neutral position is required as they have a
F Free from danger. Remove the relatively large occiput and are naturally
patient from immediate danger, positioned in a sniffing position. A small
if present. towel placed under the infants shoulders
E Evaluate the ABCs (airway, will eliminate the excessive neck flexion
breathing, circulation). caused by the prominent occiput. If there
The activation of the Emergency Medical is any possibility of cervical spine injury,
Services (EMS) should be performed as early the head tilt manoeuvre should be avoided.
Fig. 2.2.3 Jaw thrust.
as possible in the sequence of resuscitation,
provided there are adequate numbers of Chin lift
bystanders to allow this to occur without Placing the fingers of the other hand on without head tilt and is indicated particu-
delaying the commencement of BLS. Most the jawbone, lift the chin, taking care not larly if the possibility of cervical injury exists.
paediatric arrests will have an underlying to compress the soft tissues of the floor of The patency of the airway can then be
hypoxic cause and therefore any delay of the mouth under the jaw. assessed by looking for chest movement,
BLS will significantly reduce its effectiveness. listening for breath sounds and feeling for
Commencement of BLS should therefore Jaw thrust exhaled breath. This assessment is best
precede activation of EMS for a lone rescuer. Place two or three fingers under the angle of achieved from the patients side, by placing
In a witnessed sudden collapse of a child, the the mandible bilaterally, and lift the jaw an ear over the patients mouth and nose,
possibility of a sudden cardiac event is higher upwards. This technique can be performed whilst watching the chest.

21
2.2 PAEDIATRIC BASIC LIFE SUPPORT

Oropharyngeal airway seals his or her mouth over the mouth of the Chest compressions
Insertion of an oropharyngeal airway may patient, pinching off the nose with the free The technique of providing ECC varies with
assist in maintaining patency of the airway hand, whilst maintaining the patency of the patients age.
if the above manoeuvres are inadequate. the airway with head tilt and chin lift. The
An appropriately sized oropharyngeal air- mouth-to-mouth-and-nose technique may
Infants and neonates require
compression with two fingers over the
way should reach from the central incisors be necessary for the infant or small child
lower half of the sternum, avoiding the
to the angle of the jaw. In infants and small and in that case the rescuers mouth should
xiphisternum. An alternative technique
children, the oropharyngeal airway should seal around the infants mouth and nose.
involves the rescuer encircling the infants
be inserted under direct vision the right In the hospital setting the rescue breaths
chest with both hands and providing
way up; that is, it should be inserted concave will be delivered utilising bag and mask
compressions with the thumbs at the
down in the position in which it will sit in the ventilation (see Chapter 2.3 Paediatric
same landmark site. This technique is felt
oropharynx. Gentle depression of the childs advanced life support).
to be more effective, but requires two
tongue with a spatula will help facilitate Ensure that the degree of chest excursion
rescuers to provide effective EAR and ECC
airway placement. In children older than is frequently reassessed during the EAR. The
(Figs 2.2.4 and 2.2.5).
8 years, the usual adult oropharyngeal air- chest must be seen to rise as if the child
way insertion technique may be used. This was taking a deep breath. Excessive tidal
All other children require compressions
with the heel of one or two hands over the
involves inserting the airway concave up volumes or force may cause gastric dilata-
lower half of the sternum, avoiding the
and then rotating it through 180 degrees tion and regurgitation. If there is no chest
xiphisternum (Figs 2.2.6 and 2.2.7).
to sit snugly in the oropharynx. Oropharyn- movement, the most likely cause is an
geal airways will not be tolerated in con- obstructed airway due to poor positioning
The emphasis in cardiac compressions is
to minimise interruptions. The depth of
scious or semi-conscious patients because of the childs head. Reposition the patient
compression is relative and should be one
of gagging. If this occurs, the airway should using the above manoeuvres and retry.
be removed. In this situation a nasopharyn- If there is still no chest movement, there
geal airway may be a useful alternative in may be a foreign body obstructing the
the patient who is not completely obtunded. airway, which can be removed with suction
or forceps under direct vision (see below).
Cervical spine
Be aware of the risk of cervical spine injury if
the collapse is following a motor vehicle Circulation
accident (MVA) or other form of significant
trauma. If this is likely, stabilise the cervical Following the initial five rescue breaths,
spine by placing one hand on either side of assess the circulation. Although the pulse
the head and maintaining the head in line check has always been considered the gold
with the body at all times. CPR in this case standard of circulation assessment, the Inter-
generally requires two operators to perform national Liaison Committee on Resuscitation Fig. 2.2.4 Infant with two-finger technique
(ILCOR) recommendations suggest that for of ECC.
successfully. If a second operator is not avail-
able, attempt to immobilise the cervical non-healthcare professionals, the assessment
spine between sandbags or in a hard cervical of pulse has both poor sensitivity and specific-
collar before proceeding. ity and often delays the decision to com-
mence ECC. The current recommendations,
therefore, suggest that lay rescuers assess
for signs of circulation, specifically the pres-
Breathing
ence of normal breathing, coughing or move-
Once the airway is opened and patent, if the ment in response to rescue breaths.
patient is not spontaneously breathing, Healthcare professionals may check for a
deliver two to five slow rescue breaths via pulse as well as assessing for signs of circu-
expired air resuscitation. Each breath is lation. The pulses that are easiest to feel are
delivered slowly over 1 to 1.5 seconds dura- the carotid, brachial or femoral pulses and
tion (inspiratory phase) and up to five may they should be palpated for no longer than
be required to ensure that two effective a period of 10 seconds. The carotid pulse
breaths are delivered. is difficult to feel in small children who
Expired air resuscitation (EAR) is most have relatively short necks. If there is no
commonly performed as mouth-to-mouth pulse or severe bradycardia (heart rate
but may also be delivered using mouth-to- <60 bpm) with signs of poor perfusion, then
mouth-and-nose in the smaller child. In ECC on the lower half of the sternum should
Fig. 2.2.5 Infant with two-thumb technique
the mouth-to-mouth technique, the rescuer be commenced.
of ECC.

22
2.2 PAEDIATRIC BASIC LIFE SUPPORT
2

RESUSCITATION
Table 2.2.1 Rate of ECC and ratio to EAR
Precautions and
Rate of ECC Ratio of ECC:EAR complications
(per min)
Single Two Severe iatrogenic injuries are extremely
rescuer rescuers uncommon in children who have undergone
Newly 120 3:1 3:1 CPR, with an incidence of about 3%. The risk
born
of infection to the rescuer from CPR is mini-
Infant 100 30:2 15:2 mal, with the greatest risk being meningo-
and small
child
coccus from airway secretions. Standard
antibiotic prophylaxis is recommended for
Large 100 30:2 30:2
any rescuer involved in the resuscitation of
Fig. 2.2.6 Child with one-hand technique child and
of ECC. adults the airway of a patient with known or sus-
pected meningococcal infection. There have
been no reported cases of transmission of
for all infants and children (see Table 2.2.1).
hepatitis B or HIV from mouth-to-mouth
Note that the ratio of 30:2 is utilised in
ventilation to date. Airway secretions, tears,
adults regardless of the number of rescuers.
sweat and vomitus are low-risk fluids, but
Neonates require a combination of ECC:
extra precautions should be taken when con-
EAR at a ratio of 3 compressions to every
tact with blood or other bodily fluids is likely.
1 ventilation and a rate of 120 events per
minute. This rate aims to achieve approxi-
mately 90 compressions and 30 breaths
per minute.
Relief of foreign body
It is important to check that the chest
rises normally when the ventilation is
airway obstruction
provided to ensure that an effective breath Presentation
is being delivered. When two rescuers are Foreign body airway obstruction (FBAO) in
delivering BLS, the rescuer providing ECC both adults and children is commonly
Fig. 2.2.7 Child with two-hand technique should pause every 15th compression for caused by the aspiration of food, but in chil-
of ECC. the delivery of the breath. Once the airway dren it may also occur during play with small
is secured with intubation, this pause in objects. In both of these situations, it is likely
ECC is no longer required. that a parent or guardian will be present and
third to one half of the AP diameter of the Any interruption to basic life support mea- the event may have been witnessed. Clinical
chest in all age groups. The chest should sures should last no longer than 10 seconds, signs will include the sudden onset of respi-
be seen to fully recoil before the next after which BLS should be promptly recom- ratory distress, associated with coughing,
compression is commenced. ECC should menced to optimise outcome. gagging and inspiratory stridor. These signs
be performed on a firm flat surface. Mechanical devices to provide chest com- may also be caused by upper airway infec-
The rate of compressions is 100 per pressions during ECC have been designed tions; however, the onset is slower and usu-
minute for all age groups, except in and tested only in adults and are not recom- ally associated with other signs of infection,
neonates, where it is 120 per minute mended for use in children. such as fever, lethargy, or coryza.
(Table 2.2.1). Note that this is the rate or
speed of compressions, not the actual
number delivered per minute. The actual Duration of BLS in the field FBAO management in the
number of compressions delivered per Continue BLS for a period of five cycles and responsive infant or child
minute will be less than 100 as there will then reassess the patient. If there is no In a child or infant who is responsive with
be pauses for the delivery of EAR. return of spontaneous breathing or circula- good respiratory effort and forceful cough-
tion and the rescuer is alone, he/she should ing, do not interfere with the childs sponta-
seek help by activating emergency medical neous efforts. Any attempt to relieve the
Compression to ventilation ratio services. If the patient is small enough, the obstruction in this situation may dislodge
For a lone rescuer, external cardiac compres- rescuer should take the patient with them the foreign body and worsen the situation
sions should be combined with expired air and attempt to continue BLS whilst seeking by turning partial to complete airway
resuscitation in a ratio of 30 compressions help. If the patient is too large to be moved, obstruction. Call for help and get the child
to 2 ventilations for all age groups (except the rescuer should position the patient on or infant urgently to an emergency facility.
neonates see below). Healthcare providers their side and leave to seek help. Once Attempts to relieve FBAO should only be
performing two-rescuer BLS should use a EMS has been activated, BLS should con- commenced when the cough becomes
ratio of 15 compressions to 2 ventilations tinue until EMS arrives or signs of life return. ineffective with increasing respiratory

23
2.2 PAEDIATRIC BASIC LIFE SUPPORT

distress, or the child becomes unconscious response for an unresponsive, apnoeic

or apnoeic. child with FBAO is as for the infant except
the rescuer should alternate between
chest thrusts and abdominal thrusts with
FBAO management in the
each cycle.
unresponsive patient
Abdominal thrusts (Heimlich manoeuvre)
A combination of back blows and chest
may be performed with the victim in the
thrusts are utilised to relieve the obstruc-
upright or supine position.
tion. Abdominal thrusts are not recom-
mended for any age group because of the With the child in the upright position
risk of trauma to abdominal structures. (standing, sitting or kneeling), the
The sequence of response for an unre- rescuer stands behind the victim and
sponsive, apnoeic patient with FBAO is as places the fist of one hand on the
follows: childs abdomen above the umbilicus
and below the xiphisternum. The
Open the airway using chin lift or jaw
rescuers other hand covers the fist and
thrust. Look inside the mouth and
both hands are thrust sharply inwards
remove any visible foreign body. Do not
and upwards into the abdomen
perform a blind finger sweep because of
(Fig. 2.2.10).
the risk of damaging palatal tissues or Fig. 2.2.8 Back blows in an infant.
pushing a foreign body further into the
With the child in the supine position,
the rescuer kneels at the childs feet or
airway.
astride the childs hips. The heel of one
Attempt up to five rescue breaths. If
hand is placed on the childs abdomen
unsuccessful, reposition and try again.
above the umbilicus and below the
 If rescue breaths are still unsuccessful,
xiphisternum, the rescuers other hand
perform a sequence of five back blows
covers the first and both hands are
and five chest thrusts.
thrust sharply inwards and upwards into
Deliver up to five back blows using the abdomen in the midline (Fig. 2.2.11).
the heel of the one hand between the
shoulder blades, deliver five blows
Abdominal thrusts should be repeated
five times unless the foreign body is
with sufficient force to dislodge a
expelled before then.
foreign body. Check after each back
blow to see in the FB has been
dislodged. Infants can be placed in a
prone, head down position lying
along the rescuers forearm with the
jaw supported by the rescuers hand
to deliver back blows (Fig. 2.2.8).
If the obstruction is not relieved
perform up to five chest thrusts
utilising the same position and
Fig. 2.2.9 Chest thrusts in an infant.
technique as for ECC but at a rate of
one per second with sufficient force to
expel the foreign object. Check after
each chest thrust to see if the FB has relief manoeuvres whilst seeking help. If
been dislodged. Infants should be the patient is too large to be moved, the
positioned supine lying along the rescuer should position the patient
rescuers thigh, in a head down appropriately and leave to seek help.
position (Fig. 2.2.9). For management in the emergency depart-
If the obstruction is not relieved, ment setting see inhaled FB (Chapter 6.2).
continue the above sequence until the
obstruction is relieved or for
approximately one minute. If the patient FBAO management in the
remains unresponsive at one minute, unresponsive child
seek help. If the patient is small enough, A combination of abdominal thrusts, back
the rescuer should take the patient with blows and chest thrusts are utilised to Fig. 2.2.10 Abdominal thrusts in a standing
them and attempt to continue FBAO relieve the obstruction. The sequence of child.

24
2.3 PAEDIATRIC ADVANCED LIFE SUPPORT (PALS, APLS)
2

RESUSCITATION
Biarent D, Bingham R, Eich C, et al. European
It is important to take care not to touch Resuscitation Council Guidelines for Resuscitation. Section 6:
the xiphisternum or ribs during this Paediatric life support. Resuscitation 2010;81
manoeuvre due to the risk of internal (10):136488.
International Liaison Committee (ILCOR). Consensus on
damage. Science with Treatment Recommendations for Pediatric
and Neonatal Patients: Pediatric Basic and Advanced Life
Support. Pediatrics 2005;117(5).
Kleinman ME, de Caen AR, Chameides L, et al. Part 10:
Pediatric basic and advanced life support: 2010
International Consensus on Cardiopulmonary
Further reading Resuscitation and Emergency Cardiovascular Care Science
Advanced Life Support Group. Advanced paediatric life With Treatment Recommendations. Circulation 2010;16
support: The practical approach, 4th rev. ed. London: BMJ (Suppl 2):S466515.
Publishing Group; 2004. Richmond S, Wyllie J. European Resuscitation Council
Australian Resuscitation Council Guidelines, sections 12 and 13. Guidelines for Resuscitation. Section 7: Resuscitation of
Fig. 2.2.11 Abdominal thrusts in a supine child. http://www.resus.org.au/; December 2010. babies at birth. Resuscitation 2010;81(10):138999.

2.3 Paediatric advanced life support

(PALS, APLS)
James Tibballs

ESSENTIALS Introduction
1 Diagnosis of cardiac arrest by pulse palpation alone is unreliable by healthcare Definition of ALS
personnel. If the patient is unresponsive, not breathing normally and a pulse cannot Advanced life support is cardiopulmonary
be identified within 10 seconds, give cardiopulmonary resuscitation (CPR) with resuscitation (CPR) with use of specific
external cardiac compression (rate 100/minute) and ventilation in a ratio of 15:2. items of equipment available in the hospital
or ambulance setting and the use of tech-
2 Ventilate initially with bagmask ventilation. Avoid hypoxaemia during attempts niques and skills by specifically trained
at intubation which should be limited to 30 seconds. personnel. It includes the management
3 The approximate uncuffed endotracheal tube size may be chosen for children of critically-ill infants and children in pre-
over 1 year by the formula: size (mm) age (years)/4 4. (Cuffed endotracheal cardiorespiratory arrest (CPA), during arrest
tube: size (mm) age (years)/4 3.5). and post-arrest.
The recommendations for advanced CPR
4 Confirm correct location of endotracheal tube in the trachea immediately after given here are based on publications of
intubation by exhaled CO2 detection and optimize cardiac compression and lung the Australian Resuscitation Council,1 the
inflation by monitoring end-tidal CO2. European Resuscitation Council,2 the Amer-
5 Have a plan to cope with difficult and failed (impossible) intubation when ican Heart Association3 and the Interna-
bag-mask ventilation fails. tional Liaison Committee on Resuscitation
(ILCOR).4 They are intended for use by
6 Obtain intraosseous access if a peripheral vein cannot be cannulated rapidly. medical and nursing personnel in hospital
7 Administer lipid-soluble drugs (adrenaline [epinephrine], atropine, lignocaine and and by ambulance personnel in the field.
naloxone) via endotracheal tube if intravenous and intraosseous access is impossible. To add ability to knowledge, it is advisable
to undertake a specialised paediatric cardio-
8 Restore intravascular volume with a crystalloid solution (0.9% normal saline or pulmonary resuscitation course such as the
Hartmanns solution) or a colloid in aliquots of 20 mL kg1. Advanced Paediatric Life Support (APLS) or
9 Treat shockable rhythms (ventricular fibrillation, pulseless ventricular Paediatric Advanced Life Support (PALS)
tachycardia) with a single direct current shock of 4 J kg1 followed by immediate CPR courses.
for 2 minutes before re-analyzing the rhythm. Distinctions within the term paediatric
are based on combinations of physiology,
10 Institute therapeutic hypothermia (3234 C) if the patient fails to resume physical size and age. Some aspects of
consciousness after return of spontaneous circulation. CPR are different for the newly born,
11 Base decisions to cease CPR on a number of factors including the duration of infant, small (younger) child and large
resuscitation, response to treatment, pre-arrest status of the patient, remediable (older) child. Newly-born refers to the
factors, likely neurological outcome, opinions of personnel familiar with the patient infant at birth or within several hours
and, whenever appropriate, the wishes of the parents. of birth. The management of the newly-
born infant is discussed in Chapter 26.1.
25
2.3 PAEDIATRIC ADVANCED LIFE SUPPORT (PALS, APLS)

In this section, infant refers to an infant point wasting time on pulse palpation airway resistance. Size 6 FG is suitable
outside the delivery room (the newly-born (it is inaccurate and time consuming). for infants, 8 FG for small children and 10
infant) and includes the period starting Instead give CPR immediately. FG for older children. Excessive flow may
from a few hours after birth up to the desiccate mucosal membranes and cause
age of 12 months. Other terms such as gastric distension, which can embarrass
newborn or neonate do not enable that
Epidemiology respiration.
distinction. Small/young child refers to The causes of cardiopulmonary arrest in Oxygen is delivered by nasal cannulae
a child of pre-school and early primary infants and children are many and include (bi-pronged, nasal prongs), which sit at
school from the age of 1 to 8 years. Large/ any cause of hypoxaemia or hypotension, the entrance to the nose or a few centi-
older child refers to a child of late primary or both. Common causes are trauma metres inside, need no humidification and
school from the age of 9 up to 14 years. (motor vehicle accidents, near drowning, do not cause gastric distension. They may
Although ventricular fibrillation occurs in falls, burns, gunshots), drug overdose and become obstructed by mucus and may
children, they are at less risk than adults. poisoning, respiratory illness (asthma, obstruct the nose. Flow rates for infants
One guideline regards children of 8 years upper airway obstruction, parenchymal dis- should be regulated by a low-flow meter,
and over as adults specifically for use eases), post-operative (especially cardiac), graduated 02.5 L min1. Rates of 0.24
of semi-automated external defibrillators septicaemia and sudden infant death 4 L min1 provide 4070% O2 to infants
(sAED) out-of-hospital. syndrome. 110 kg body weight (BW). Improved oxyge-
nation may be caused in part by positive end
expiratory pressure (PEEP).
Diagnosing cardiac arrest Oxygen, ventilation and An oxygen catheter placed in the naso-
Healthcare personnel (doctors and nurses) advanced airway support pharynx a distance equivalent to that from
have difficulty diagnosing cardiac arrest Oxygen ala nasi to tragus provides a small amount
in infants and children if they rely on Oxygen should be administered whenever of PEEP, and indeed may be used for that
pulse palpation alone. Their accuracy is hypoxaemia occurs but evidence from ani- purpose. Oxygen concentrations of 30%,
approximately 80% with a sensitivity of mal and newborn infant studies suggests 40% and 50% approximately are provided
0.85 and specificity of 0.65,5 which means that as soon as oxygenation is achieved by flows of 45, 80 and 150 mL kg1 min1
that in 15% of circumstances they would the inspired oxygen (FiO2) should be regu- respectively. This technique to provide PEEP
not give CPR when needed and would lated to yield arterial oxygen partial- may be useful to temporarily abort central
give it in 35% when not needed. While pressure in the normal range in order to apnoea in the young infant with RSV, whilst
application of CPR is not harmful when limit oxygen-mediated cell damage. The other treatment such as caffeine infusion is
there is a circulation, the withholding of only exception to administration of oxygen established.
CPR when there is none dooms the patient is when it may cause pulmonary vasodilata- Oxygen prongs
to die. The time taken to diagnose cardiac tion and thereby shunt blood to the lungs Non-humidified oxygen is subject to the
arrest is longer than hitherto realised6 away from the systemic circulation, as same flow restriction as nasal catheters
as a group, healthcare personnel take may occur in an infant with a single ventri- (up to 4 L min1). The use of high flow
an average of 15 seconds to exclude cle which pumps blood to both circulations. (
8 L min1) humidified oxygen as a means
cardiac arrest by finding a pulse but 30 Chronic hypoventilation is not an indication to provide CPAP and thereby prevent
seconds to diagnose real cardiac arrest to withhold oxygen therapy during CPR for apnoea, although an attractive technique,
by the absence of a pulse. However, infants and children. has not yet proven to be advantageous in
the accuracy and expediency of diagnosis Numerous devices may be used to supply infants outside the premature age group.
are related to experience and training. supplemental oxygen. The choice is dictated
Only experienced personnel who palpate by the required inspired oxygen concentration Oxygen masks
pulses on a daily basis are able to detect (FiO2), cost, avoidance of CO2 rebreathing, Semi-rigid face masks of the Hudson type can
a real pulse within 10 seconds but they, imposed airway resistance and tolerance by supply approximately 3570% O2 at flow
like inexperienced personnel, are unable the patient. rates of 415 L min1. However, they may
to quickly diagnose cardiac arrest by the not be well tolerated by the infant or small
lack of a pulse and need on average Oxygen catheters child, and the distress they cause may con-
about 25 seconds to confirm it. Clinical These are easy to use, cheap, do not cause sume energy in the tiring child. They may
guidelines advise to spend no more than rebreathing and are well tolerated (permit- cause rebreathing or fail to deliver the desired
10 seconds on pulse palpation and to ting eating and drinking) but are limited FiO2, especially when peak inspiratory flow
combine whatever information is gained to supply of up to 40% inspired O2 because rate (PIFR) is high, thus entraining excessive
with observable signs of circulation such of restriction to gas flow (maximum room air. Masks that incorporate a reservoir
as responsiveness, movement and pre- 4 L min1) and limitation of gas reservoir bag or a Venturi may deliver up to 80% O2
sence or absence of normal respiration. (the nasopharynx). Sizes 6, 8 and 10 FG but, likewise, may cause rebreathing if the
In short, if the patient is unresponsive should be available and placed in the same flow rate does not match PIFR during respira-
and not breathing normally there is no nostril as the nasogastric tube, to limit tory distress.

26
2.3 PAEDIATRIC ADVANCED LIFE SUPPORT (PALS, APLS)
2

RESUSCITATION
Head boxes and incubators devices are available: flow-inflating and that the patient valve opens or the reser-
A clear Perspex head box is the best way of self-inflating bags. They can be attached voir bags deflates in unison with the chest
administering a high concentration of oxy- to a mask, laryngeal mask airway (LMA), movement.
gen to the unintubated infant. It allows a endotracheal tube or tracheostomy. The delivered oxygen concentration is
non-distressing delivery of oxygen and dependent on the flow rate of oxygen, use
allows clear observation of the child. Pre- Flow-inflating bags of the reservoir bag, and the state of the
cise oxygen therapy is possible but may These are designed to give either positive pressure relief valve (whether open or
be expensive and rebreathing, heat loss pressure ventilation or to allow a patient closed). In the Laerdal series, with use of
and desiccation are potential problems. to breathe spontaneously. They are exempli- the reservoir bag and oxygen flow greater
To avoid rebreathing, a large flow rate fied by Jackson-Rees modified Ayres T-piece. than the minute ventilation, 100% oxygen
(1012 L min1) of fresh gas with predeter- Gas flow must exceed 220 mL kg1 min1 is delivered. Without the reservoir bag the
mined oxygen content should be introduced. for children and 3 L min1 for infants, to pre- delivered gas is only 50% oxygen, despite
The practice of introducing a low-flow rate vent rebreathing during manual ventilation. oxygen flow rate at twice minute ventila-
of 100% oxygen into a head box (to gain a It is possible to control precisely the con- tion. At an oxygen flow rate of 10 L min1
lesser concentration) may cause hypercarbia. centration of inspired oxygen, which is an to the infant resuscitator bag, the delivered
If 100% oxygen is the only compressed gas advantage, especially for the premature gas is 85100% oxygen without the use of
available, lesser concentrations of oxygen neonate at risk of oxygen-induced retrolen- the reservoir bag.
may be attained without rebreathing by using tal fibroplasia. However, considerable expe-
a flow of 100% oxygen and a Venturi device. rience is necessary to provide adequate Rates and ratios of external
The relatively large capacity of an incubator ventilation without barotrauma in the intu- cardiac compression and
precludes the attainment of a high con- bated infant. A pressure gauge and a relief ventilation in ALS
centration of oxygen, but limited oxygen valve should be incorporated in the circuit The techniques of external cardiac com-
therapy, up to a maximum of 60%, can be to help prevent barotrauma. pression and expired air resuscitation
achieved at the cost of very high flow rates. (mouth-to-mouth) or rescue breathing are
The concentration of oxygen in the head Self-inflating bags described in Section 2.2. The recommended
box can be monitored with an analyser and These bags are designed only to give posi- ratio of external cardiac compression to
help monitor the progress of the lung disease tive pressure ventilation. Rebreathing is ventilation in basic life support by a
and oxygen requirements. prevented by one-way duck-bill valves, single rescuer is 30:214 which if able to
spring disk/ball valves or diaphragm/leaf be repeated 5 times in 2 minutes, with
valves. The Laerdal bag series (infant, child, pauses for ventilations, would yield approx-
adult) typifies these devices. A pressure-relief imately 75 compressions and 5 breaths per
Ventilation valve (infant and child size) opens at 35 cm minute.
Bagmask ventilation H2O (3.5 kPa). A pressure monitor can be In advanced life support the recom-
As soon as practicable, mechanical venti- incorporated in the circuit. Supplemental oxy- mended ratio of compressions to ventilation
lation with added oxygen should be com- gen is added to the resuscitation bag, with or by 2 rescuers is 15:214 which, if repeated
menced with either a bagmask (bag without attachment of a reservoir bag, whose 5 times in 1 minute, with pauses for ventila-
valvemask) or via endotracheal intubation. movement may serve as a visual monitor of tion by bagmask, would yield approximately
Although intubation is preferred (see tidal volume during spontaneous ventilation 75 compressions and 10 ventilations per
below), valuable time should not be wasted when intubated. However, the valve may minute. When the patient is intubated, it is
in numerous unsuccessful attempts. Initial offer resistance for spontaneous ventilation undesirable and not necessary to pause
effective bagmask ventilation is a neces- and this is important to consider in the spon- cardiac compressions to give ventilations
sary prerequisite for successful paediatric taneously breathing child just prior to the because they can be delivered effectively
CPR but it is a relatively difficult technique effect of relaxants of rapid sequence induc- during cardiac compressions and cardiac
to learn and to perform well in emergency tion prior to intubation. compressions can be given continuously
circumstances. Practice on mannequins or These bags should not be used to provide without interruption. Every time cardiac
in an anaesthetic setting is invaluable in supplemental oxygen to a spontaneously compression is interrupted, cardiac stroke
learning to perform this well for the infre- breathing patient with a mask placed near volume obviously falls to zero and then sev-
quent paediatric resuscitation. or loosely over the face. With Laerdal and eral successive compressions are required
Bags of appropriate sizes should be Partner bags, negligible amounts of oxygen to re-establish the stroke volume achieved
available for infants, small children and (0.10.3 L min1) issue from the patient before interruption. Necessary interruptions
large children. A bag of approximately valve when 515 L min1 of oxygen is to cardiac compressions should be mini-
500 mL volume should be available for introduced into bags unconnected to mized by co-ordinated planning, to for exam-
newborn infants and infants. patients.7 The patient valve is unlikely to ple, analyse the cardiac rhythm or to give DC
Insertion of an oropharyngeal (Guedel) open unless the mask is sealed well on shock. Effort must be directed towards mini-
airway may be necessary to facilitate bag the face. Although not recommended, if mizing hands-off time during requirement
mask ventilation. Two types of resuscitation they are used in this way, it is vital to ensure for cardiac compression.

27
2.3 PAEDIATRIC ADVANCED LIFE SUPPORT (PALS, APLS)

confirmed by capnography or CO2 detection, endobronchial intubation. Assessment of the

Advanced airway support with the realisation that CO2 excretion can depth of insertion during laryngoscopy by not-
Tracheal intubation only occur with effective pulmonary blood ing passage through the vocal cords is not
The trachea should be intubated as soon flow. This implies that CO2 detection cannot completely reliable since alteration of head
as practicable but it can be deferred if success- be expected unless spontaneous cardiac position affects depth of tube insertion. The
ful bagmask ventilation can be given and output returns or external cardiac compres- tube depth increases with neck flexion (goes
should not be undertaken by inexperienced sion is effective. Absent CO2 detection man- in) and decreases (comes out) during exten-
personnel out-of-hospital8 because of compli- dates re-intubation or at least inspection sion. Since intubation is usually performed
cations and poorer outcomes compared with that the tube is indeed passing through with the head extended, the tube depth
use of bagmask ventilation. Nonetheless, the vocal cords. High CO2 indicates poor ven- increases when the laryngoscope is removed
intubation has numerous advantages, which tilation. Oxygenation should be confirmed and the head assumes a position of neutrality
include establishment and maintenance of with use of a pulse oximeter or measure- or flexion. In a neutral head position, an appro-
the airway, facilitation of mechanical ventila- ment of arterial gas tension. priate depth of insertion measured from the
tion, titration of oxygen therapy, minimisation centre of the lips for an oral tube is 9.5 cm
Endotracheal tube size for a term newborn, 11.5 cm for a 6-month-
of the risk of pulmonary aspiration, enable-
(Table 2.3.1) old infant, 12 cm for a 1-year-old. After 1 year
ment of tracheal suction, provision of a
Uncuffed sizes are 2.5 mm for a premature the depth is given by the formula: depth
route for the administration of selected drugs
newborn <1 kg, 3.0 mm for infants (cm) age (years)/2 12. An alternative
and preferred for transport and long-term
13.5 kg, 3.5 mm for infants >3.5 kg and formula for oral tube depth of insertion is:
ventilation. Regurgitation of gastric contents
is common during cardiac arrest.
up to age of six months, size 4 mm for infants depth (cm) size (mm)  3 when an appro-
seven months to one year (Table 2.3.1). The priate tube size for age is used. The appropri-
Hypoxaemia should be avoided during
attempts at intubation which should be
approximate size may be chosen for children ate depth of insertion for a nasal tube in
over one year by the formula: size (mm) this age group is: depth (cm) age (years)/
limited to 30 seconds. If difficulty is experi-
age (years)/4 4. Tubes one size larger 2 15. On a chest X-ray taken with the head
enced, oxygenation should be re-established
and smaller should be readily available. The in neutral position, the tip of the tube should
with bagmask ventilation before a reat-
correct size should allow a small leak on appli- be at the interclavicular line.
tempt at intubation. Initial intubation
cation of moderate pressure but also enable
should be via the oral route, not via the nasal
adequate pulmonary inflation. If the lungs
route. The oral route is invariably quicker, is
are non-compliant, however, it may be neces- Laryngeal mask airway
less likely to cause trauma and haemorrhage
sary to insert a tube without a leak or insert a (LMA)
and the endotracheal tube is more easily
cuffed tube. Appropriate- sized cuffed tubes
exchanged if the first choice is inappro-
may be estimated by the formula: size These have been used for resuscitation by
priate. On the other hand, a tube placed
(mm) age (years)/4 3.5. medical, nursing and ambulance personnel
nasally can be better affixed to the face
trained in their selection and insertion. They
and so is less likely to enter a bronchus or
Depth of tube insertion may be used to maintain an airway and are
be inadvertently dislodged during trans-
(Table 2.3.1) a suitable alternative to the use of airway
port or other procedures. A nasal tube is
The tube is inserted to a specific numerical opening manoeuvres and use of oropharyn-
preferred subsequently for long-term man-
depth to avoid accidental extubation or geal and nasopharyngeal airways. They are
agement. A nasogastric tube should be
inserted after intubation to relieve possible
gaseous distension of the stomach sustai- Table 2.3.1 Endotracheal tube sizes (internal diameter) and orotracheal and nasotracheal
depths for infants and children
ned during bagmask ventilation.
Correct placement of the endotracheal Age/body weight (kg) Size (mm) Oral depth (cm) Nasal depth (cm)
tube in the trachea must be confirmed Newly-born (3.5) 3.0 9.0 11.011.5
immediately. In the hurried conditions of
16 months 3.5 9.511 1213
emergency intubation at cardiopulmonary
arrest, it is not difficult to mistakenly intu- 612 months 4.0 11.512 1314
bate the oesophagus or to intubate a bron- 23 years 4.5 1313.5 1516
chus. There is no substitute for visualising
45 years 5.0 1414.5 1718
the passage of the tip of the endotracheal
tube through the vocal cords, confirmation 67 years 5.5 1515.5 19
of bilateral pulmonary air entry by ausculta- 89 years 6.0 1616.5 20
tion in the axillae, continuous observation
1011 years 6.5 1717.5 21
of rise and fall of the chest on ventilation
and maintenance of a pink complexion. 1213 years 7.0 1818.5 22
In addition, it is recommended that correct 1416 years 7.5 19 23
placement of the endotracheal tube be

28
2.3 PAEDIATRIC ADVANCED LIFE SUPPORT (PALS, APLS)
2

RESUSCITATION
useful to establish an airway in the setting nesses or because of loss of natural mainte- with macroglossia are typical conditions
of airway obstruction or failed intubation.9 nance after administration of drugs, which in which difficult intubation should be
An intubating LMA serves as a conduit for depress consciousness, muscle tone or activ- anticipated.
intubation. ity. Airway difficulties refer to maintenance Difficulty with intubation may be unan-
However, the role of LMA in provision of of the airway, bagmask ventilation and ticipated, but may be predicted with three
mechanical ventilation remains uncertain. endotracheal intubation. Each situation simple observations. One of these is the rel-
Like bagmask ventilation, they do not pro- may rapidly lead to hypoxaemic brain ative tongue/pharyngeal size (Mallampati
tect the airway from aspiration, which occurs damage and death. test): if the faucial pillars, soft palate and
commonly during cardiopulmonary resusci- Resuscitators must be well-skilled in the uvula are obscured by the extended tongue
tation. They are a suitable alternative to a management of the airway and provision on maximum mouth opening, intubation
face mask as a means to give ventilation of bagmask ventilation. It is vital to possess will be difficult. Another predictor is the
before endotracheal intubation and when good basic resuscitation techniques (see extent of possible atlanto-occipital exten-
intubation is difficult. This is a better tech- Chapter 2.2 on basic life support), to be sion. If this is less than 35 intubation will
nique when the operator is unskilled in the familiar with equipment, to have skilled be difficult. This corresponds to the angle
use of LMA and intubation. Although assistance and to have appropriate equip- between the occlusal surface of the upper
insertion of an LMA is easier to learn than ment ready-to-hand. They must also be teeth and horizontal plane when the head
endotracheal intubation, training should familiar with manoeuvres to overcome diffi- is maximally extended. The third predictor
not replace mastery of bagmask ventila- culties with airway maintenance, bagmask is the amount of mandibular space into
tion. They should not be used in semi- ventilation and intubation. Resuscitators which the tongue must be compressed to
conscious patients or when the gag reflex must also have a pre-conceived plan to cope allow a line of sight to the glottis. This
is present and are not suitable for long-term with difficult and failed (impossible) intuba- space can be judged by the distance from
use or use during transport when endotra- tion, especially when bagmask ventilation the thyroid cartilage or hyoid bone to the
cheal intubation is far preferable. They are fails (Fig. 2.3.1). point of the mandible (thyromental, sub-
subject to dislodgment during movement An obstructed airway may be relieved mental distances) or by the horizontal
and transport. Appropriate sizes according by simple repositioning of the patients length of the mandible. In adult-sized
to body weight are given in Table 2.3.2. head and neck, pharyngeal suction, use of patients a thyromental distance of greater
an alternative airway-opening manoeuvre, than 6 cm and a mandibular length of
insertion of an oropharyngeal or nasopha- greater than 9 cm predict easy intubation.
Management of the difficult ryngeal airway and help by an assistant. Unfortunately, no such distances are known
airway Persistent obstruction requires urgent endo- for children and infants.
Unfortunately, unanticipated difficulties tracheal intubation. Thus, the evaluation of intubation difficulty
with the airway and intubation or failures should include pharyngeal examination with
to follow a prepared plan to cope with diffi- Difficult intubation extended tongue, inspection of mandibular
cult and failed intubation continue to cause With good technique, normally, intubation size or submental space and examination
deaths, irrespective of the degree of skill of can be achieved via direct laryngoscopy with of atlanto-occipital angle. These special
the operator. a direct line of sight to the glottis. Some- measures should be in addition to routine
The airway can be difficult to maintain times difficult intubation is a consequence history of airway management, as would have
because of unusual anatomy, injury or ill- of anatomical abnormality, trauma or infec- occurred in previous anaesthesia, inspection
tion but sometimes it is simply the result of of nostrils and history and examination of car-
operator unpreparedness or incorrect choice diorespiratory function.
Table 2.3.2 Laryngeal mask airways or use of equipment or lack of knowledge of
simple techniques to overcome difficulties. Equipment and technical
Sizes are available to suit body weight (kg)
of newborns, infants and children difficulties
Anatomical difficulties Although Magills forceps are very useful
Size Weight (kg) Certain conditions imply that intubation for directing the tube into the larynx, the
1 <5 will not be easy but it is prudent to inspect use of simple tricks may facilitate intuba-
1 12 510 the facial and pharyngeal anatomy of tion. For example, it is important that the
every patient before giving sedation or patients head and neck be correctly posi-
2 1020
anaesthesia. Common anatomical variants tioned in the so-called sniffing position
2 12 2030 in many conditions, which will disallow a (neck flexed, head slightly extended) to
3 3050 direct line of sight to the larynx are an ante- offer the best line of sight to the larynx.
rior larynx, prominent upper incisors, a large In the neonate and infant, however, the
4 5070
tongue or a small hypoplastic mandible. Thus head and neck should remain in the neutral
5 70100 Treacher Collins syndrome and PierreRobin position. The blade of a regular laryngo-
6 >100 syndrome (sequence) with mandibular hypo- scope is designed to be introduced into
plasia and BeckwithWiedemann syndrome the right side of the mouth (not the left)

29
2.3 PAEDIATRIC ADVANCED LIFE SUPPORT (PALS, APLS)

Known difficult airway

or intubation

Airway difficulty
Yes
Seek advice before Optimise headneck position
sedating or Suction oropharynx
anaesthetising Can ventilate by bagmask? Insert oropharyngeal/nasopharyngeal airway
Insert laryngeal mask

Optimise bagging technique

Yes No GET HELP NOW

Can intubate? Can intubate? Rapid sequence intubation

GET HELP NOW No No

Ventilate by bagmask Apply cricoid pressure If

optimise intubation technique, try again unfamiliar

Use most familiar

Use introducing or illuminating stylet
can intubate? Insert laryngeal mask try first Avoid hypoxaemia
Employ two-person intubating technique Attempt reventilation by mask
No Use bougie/intubating bougie but if not possible
Use difficult intubation laryngoscope
Use intubating bronchoscope attempt CPAP with 100% O2
Perform retrograde intubation Try last
If intubation not needed to save life If intubation needed to
abandon attempts save life, proceed
revert to bagmask ventilation If intubation impossible
(repeated intubation attempts may
make ventilation impossible)

Insert trans-tracheal catheter Easiest

Insert IV cannula via cricoid membrane
Perform cricothyrotomy
Mini-tracheostomy Hardest

Organise surgical tracheostomy

Fig. 2.3.1 Management of difficult and impossible intubation.

so that when the blade is subsequently The bevel of an endotracheal tube is larynx is anterior and difficult or impossible
centred the tongue is displaced to the angled so that it easily enters the trachea to see, use of an introducing stylet to create
left and an unimpeded view of the larynx when introduced from the right side of a more acute curve at the tube tip may
and sufficient working space is afforded. the mouth. One should insert the tube achieve intubation and/or application of
The tip of a curved-bladed laryngoscope keeping it oriented in a horizontal plane, posterior cricoid pressure may bring the
(Mackintosh type) is placed in the vallecula rather than vertical, so that the presence anterior larynx into view. A tube may be
(space between base of tongue and epig- of the tube does not impede the intubators placed blindly into the trachea with or
lottis) whereas the tip of a straight- view of the larynx. This may be facilitated without the use of a stylet or bougie over
bladed laryngoscope is placed behind the by an assistant pulling down on the corner which a tube can be railroaded. Skilled
epiglottis. The laryngoscope handle is then of the mouth. When introduced from the anticipatory assistance and good suction
elevated upward and away from the opera- left side, the tip of the bevel may stick at by a dedicated airway nurse who antici-
tor to lift the tongue out of the line of sight the laryngeal inlet but this can be easily pates the needs of the intubator are essen-
to the glottis. The handle should not be remedied by rotating the tube anticlock- tial. Occasionally, a gentle finger of an
elevated directly upwards or elevated wise, thus changing the angle of approach assistant retracting the lip of the child
back towards the operator such actions and directing the tip more toward the cen- may enlarge the oral opening to afford
may not displace the tongue sufficiently tre of the laryngeal inlet rather than to its improved vision and field to place the endo-
and fail to bring the glottis into view, and right side. If the tip of the upward curving tracheal tube down the right side of the
moreover may damage the upper teeth. tube sticks in the anterior larynx, flexion mouth.
The upper teeth should not be used as a of the neck may direct the tube posteriorly Many other items of equipment are avail-
leverage point. and encourage it to enter the larynx. If the able to assist when intubation proves to be

30
2.3 PAEDIATRIC ADVANCED LIFE SUPPORT (PALS, APLS)
2

RESUSCITATION
difficult. These include laryngoscopes that the more-skilled operator when they arrive. while skin over the thyroidcricoid mem-
have a tip independent of the shaft of the In situations where intubation is not abso- brane (between the thyroid and cricoid
blade, which is used to elevate the epiglottis lutely needed (i.e. not life-saving) it is pru- cartilages) is incised with a scalpel held
(e.g. McCoy laryngoscope, Penlon), or which dent to revert to bagmask ventilation (if in the other. Then, bluntly dissect into
incorporate a prism to give a view of an ante- that is possible) rather than persist in intu- the trachea with forceps in the midline or
rior larynx (e.g. Belscope), illuminating stylets bation attempts, which could ultimately incise vertically with the scalpel. Insert a
and intubating bronchoscopes. Illuminating damage the larynx and render bagmask small tracheostomy, preferably bevelled,
stylets are malleable and give a Jack-o- ventilation impossible. If ventilation is not or a small endotracheal tube. Alternatively,
lantern effect when correctly located in the possible by mask, sometimes application of perform percutaneous mini-tracheostomy.
trachea. All items of equipment are useless CPAP with 100% oxygen via an Ayres A formal tracheostomy should be orga-
unless they are ready-to-hand and are famil- T-piece will suffice to maintain oxygenation. nised while these measures are underta-
iar to the operator. Prolonged CPAP cannot be effectively ken. An important part of a contingency
A two-person intubating technique may applied with a mask using a self-inflating plan to cope with unexpected difficulties
achieve intubation in difficult circumstances. resuscitator bag. is to have easily contactable more experi-
In this, one person holds the laryngoscope If the airway is totally obstructed and enced operators.
with their left hand and applies cricoid pres- neither bagmask ventilation nor intuba-
sure with their right to bring the larynx into tion can be performed, the situation is more
view, while a second person applies suction, desperate. Adequate oxygenation (but not Monitoring
holds the lower lip out of the way and normal ventilation) can be obtained by
attempts to pass the endotracheal tube. inserting a trans-tracheal catheter (Mallinck- Vital signs
Another technique is retrograde intuba- rodt Medical Pty Ltd) otherwise intended for Routine monitoring of heart rate, respiratory
tion (translaryngeal-guided) in which a jet-ventilation, or a 14-gauge intravenous rate and blood pressure are essential for
guide wire is threaded through a needle or cannula percutaneously into the trachea infants and children with critical illness. It
intravenous catheter inserted cephalad caudad via the cricothyroid membrane is prudent to have ready access or to have
through the cricothyroid membrane. The (which lies immediately inferior to the thy- displayed the normal age-related values
end of the wire is retrieved from the oral roid cartilage). To do this, the patient should visually available in the paediatric resusci-
or nasal cavity, pulled taut and used as a be lying straight, with the cannula in the tation area of the ED as an aide-memoire.
tightrope over which an endotracheal tube midline and angled towards the feet. After
is railroaded into the trachea. Alternatively, removing the needle, the trans-tracheal Oximetry
a string can be tied to the end of the wire catheter can be connected directly via its Transcutaneous oximetry (SpO2) is essential
which is then drawn from the tracheal punc- standard 22 mm connector to a bagging monitoring in critically ill patients. It equates
ture. The proximal end of the string can be circuit. An intravenous cannula can be well to arterial haemoglobin oxygen satura-
tied to the Murphy eye of a tube, which is connected by various ways to a source of tion (SaO2) but not when the SaO2 is below
then pulled from below into the trachea. oxygen. One of these is direct connection 70%. Note that an SpO2 of 90%, although
A nasal endotracheal tube can be placed by to a resuscitator or a bagging circuit using only 10% below normal, represents a partial
joining a wire (or string) inserted into the oral a connector from a 3.0 mm endotracheal pressure of oxygen in arterial blood (PaO2) of
cavity via the nasal route and joining it to the tube. Alternatively, the cannula can be 60 mmHg, which is 40 mmHg below normal.
wire or string entering the oral cavity via the connected to continuous oxygen supply via
trachea. Retrograde intubation is a useful a three-way intravenous tap (to allow expi- Expired CO2 detection
technique in cases such as facial trauma, tris- ration) and a length of plastic tubing. Confirmation of endotracheal
mus and upper airway masses. Another option is use of plastic tubing intubation
alone that has a side hole cut or a Y-piece End-tidal CO2 (PetCO2) is recommended
Failed intubation inserted, which is intermittently occluded after every tracheal intubation and during
If endotracheal intubation is impossible to cause inspiration (1 second) and unoc- mechanical ventilation to guard against
when needed, the resuscitator must be able cluded (3 seconds) to allow expiration. inadvertent oesophageal intubation and
to oxygenate the patient while preparations With all these techniques care should be inadvertent extubation, particularly when
are made for tracheostomy or until intuba- taken to allow expiration to avoid baro- the intubated patient undergoes transport
tion can be somehow achieved. trauma. Expiration may need to be assisted to, within or between hospitals. Small move-
Attempts at intubation should not be pro- by lateral chest compression as the arrested ments of head and neck, as may occur for
longed, so that hypoxaemia is not caused. patient may not spontaneously expire much example on transfer from one trolley to ano-
Oxygenation should be restored with inter- air. Ventilation is very difficult but oxygen ther or to a bed, may dislodge an endotra-
mittent bagmask ventilation. Likewise, can be supplied by sustained pressure. cheal tube. No CO2 is excreted unless there
intubation attempts should not be overly A semipermanent solution for a totally is pulmonary blood flow so undetectable
repetitive because the larynx will be trauma- obstructed airway is cricothyro(s)tomy. To CO2 after intubation basically represents
tised and this may render bagmask ventila- do this the larynx is stabilised throughout non-tracheal intubation or absence of circu-
tion, previously possible, now impossible for the procedure with fingers of one hand lation, or both.

31
2.3 PAEDIATRIC ADVANCED LIFE SUPPORT (PALS, APLS)

Regulation of cardiac compression It provides rapid, safe and reliable access to Endotracheal route
and ventilation the circulation and serves as an adequate Drugs are absorbed into the circulation from
To achieve optimum CO2 excretion, cardiac route for any parenteral drug and fluid the airways. Lipid-soluble drugs (adrenaline
output and pulmonary ventilation must be administration. Syringing via a three-way [epinephrine], atropine, lidocaine and
matched. A common error in advanced tap is usually needed. The use of purpose- naloxone) may be administered via the
CPR is giving ventilation in excess of the lim- made intraosseous bone injection needles endotracheal tube if either intravenous or
ited cardiac output achievable by external (e.g. Cook Aus Pty Ltd, 16g, 3 cm POWCH intraosseous access is non-existent. Although
cardiac compression, which is likely to be design) is preferable, although a short lum- the optimal doses of these drugs by this
no more than a third of normal cardiac out- bar puncture type of needle with an inner route are not known, work in animal models
put. Ventilation can be safely reduced trocar may suffice. suggests doses should be ten-times the
proportionately. Moreover, excessive ventila- The handle of the device needle is held intravenous doses. The drugs should be
tion not only interferes with performance of in the palm of the hand while the fingers diluted in normal saline up to 2 mL for
external cardiac compression but also grip the shaft about a centimetre from the infants, 5 mL for small children and 10 mL
increases intrathoracic pressure inhibiting tip. It is inserted perpendicular to the bone for large children. It is acceptable and sim-
venous return and cardiac output and may surface and a rotary action is used to tra- plest to squirt the drugs from the syringe
cause hypocarbia which causes cerebral verse the cortex. Sudden loss of resistance directly into the endotracheal tube and
ischaemia by vasoconstriction. On the other signifies entry to bone marrow and the disperse them throughout the respiratory
hand, inadequate ventilation contributes to needle should stand unsupported. Correct tree with bagging. Neither sodium bicarbon-
hypercarbia, acidosis and cerebral vasodila- positioning of the needle is confirmed by ate nor calcium salts should be administered
tion. After tracheal intubation, end-tidal aspiration of bone marrow (which may via the tracheal route because they injure
CO2 should be monitored by capnography. be used for biochemical and haematologi- the airways.
If end-tidal CO2 is low, excessive ventilation cal purposes) but that is not always possi-
and inadequate external cardiac compres- ble. A bone marrow injection gun (Wais Other techniques
sion (rate, depth of compression) should be Med Ltd) which fires a needle a pre-set Surgical cutdown onto a long saphenous,
excluded as causes. distance according to size of the patient saphenofemoral junction or basilic is a valu-
or a bone marrow drill (EZ-IO, Vidacare) able skill sometimes required in traumatic
Electrocardiograph (ECG) enables easy and rapid intraosseous infu- exsanguination. Very occasionally, injection
The ECG should be displayed with either sion/injection for infants, children and into the superior sagittal sinus of an infant1
leads, pads or paddles. Drug therapy or imme- adults. The latter device is preferred. Hav- may be the only vascular access available.
diate direct current shock is administered ing an intraosseous drill available in the Any pre-existing functioning line can be used
according to the existing rhythm. Electrolyte ED can provide extremely rapid access to provided it does not contain any drug or elec-
status, especially that of potassium and cal- the circulation in a child arriving in CPA. trolyte, which may have caused the CPA.
cium, may be indicated by ECG patterns. Although many sites have been used for
bone marrow injection, the easiest to identify
is the anteromedial surface of the upper or
lower tibia. The site of the latter is a few cen-
Fluid therapy
Vascular access
timetres below the anterior tuberosity and the Circulatory hypovolaemia is expected in
Peripheral venous cannulation former a few centimetres above the medial trauma, sepsis, dehydration and anaphylac-
Access to the circulation via a peripheral malleolus. Care should be exercised to avoid tic states. Restoration of intravascular vol-
vein should be attempted immediately on complications, particularly cutaneous extra- ume should be with isotonic crystalloid
CPA. Any site is acceptable. Visible or palpa- vasation, compartment syndrome of the leg, solutions (0.9% normal saline or Hartmanns
ble peripheral veins are to be found on the and osteomyelitis. Contraindications include solution) or a colloidal solution such as
dorsum of the hand, wrist, forearm, cubital local trauma and infection. 4% albumin. There is insufficient evidence
fossa, chest wall, foot and ankle. In infants, to choose between these. Aliquots of
scalp veins are accessible and the umbilical 20 mL kg1 intravenously or intraosseously
vein can be used up to about a week after Central venous cannulation are reasonable volumes to administer in
birth. The external jugulars are usually dis- Cannulation of femoral, subclavian, or inter- shock states with titration against indices
tended during CPR and easy to cannulate nal jugulars or external jugular veins are of vascular volume. It is reasonable to
but this may be impeded by performance options. However, central cannulation is dif- administer blood in traumatic haemorrhage
of intubation. ficult in the setting of cardiorespiratory if 4060 mL kg1 has not restored normal
arrest and fraught with potential serious blood volume (7080 mL kg1). The role of
Intraosseous access complications such as pneumothorax unless hypertonic solutions is not yet defined for
If peripheral intravenous access cannot be the operator is well practised. This technique children with hypovolaemic shock, but these
rapidly achieved, say within 60 seconds, is not recommended in the setting of an solutions are in regular use for patients with
intraosseous access should be obtained. This arrested child as the intraosseous route is severe head injury. Dextrose-containing solu-
route has been used for patients of all ages. more timely. tions are inadvisable in acute resuscitation

32
2.3 PAEDIATRIC ADVANCED LIFE SUPPORT (PALS, APLS)
2

RESUSCITATION
unless hypoglycaemia is proven since they ectopic tachycardia (JET), ventricular tachy- and may sustain hypoglycaemia, whereas
may cause osmotic diuresis. Drugs should cardia (VT) and DC-shock resistant ventri- the stress response may cause hypergly-
be flushed into the circulation with boluses cular fibrillation (VF). It inhibits a- and caemia. Blood levels should be monitored
of isotonic crystalloid solution. b-receptors, slows AV nodal conduction, regularly. Hypoglycaemia (<2.5 mmol kg1)
and prolongs the QT interval and QRS dura- should be treated with a dextrose infusion
Resuscitation drugs tion. It thus may cause torsade de pointes of approximately 68 mg kg1/min or
Adenosine VT. The loading dose is 5 mg kg1 infused equivalent. Bolus injection may be necessary
This endogenous nucleoside is the drug of over several minutes to an hour depending (in situations such as seizure or coma due to
choice for treatment of supraventricular on the dysrrhythmia being treated. Repeated hypoglycaemia) in which case an appro-
tachycardia (SVT) if circulation is adequate, doses to a maximum of 15 mg kg1 may be priate dose is 0.5 g kg1 as provided, for
otherwise DC shock is the preferred first treat- given. Acute side effects are vasodilatation example, by 5 mL kg1 of 10% or 2 mL kg1
ment. It blocks AV node conduction and thus and hypotension and chronic effects are thy- of 25%. Such boluses, however, may cause
re-entry circuits, which are the usual causes roid function disturbance, interstitial pneu- hyperglycaemia and an acute rise in serum
of SVT in infants and children. It has a very monitis, corneal deposits and blue-grey skin osmolality with osmotic diuresis and harm-
short half-life (about 10 seconds) in the blood discolouration. ful rapid changes in brain osmolality. Hypo-
because it is deaminated and inactivated by glycaemia and hyperglycaemia after brain
adenosine deaminase on the surface of red Atropine injury should be avoided.
blood cells. Hence, it must be delivered as a This parasympatholytic drug is used to treat
rapid bolus. The dose is 100300 mcg kg1 bradycardia (<60 min1) caused by exces- Lidocaine (Lignocaine)
(commonly 100 mcg kg1) delivered rapidly sive vagal activity or a consequence of atrio- This is a sodium channel blocker that
intravenously (or intraosseously) and fol- ventricular block (AV). If inadequate decreases automaticity and suppresses ven-
lowed by a bolus of normal saline. If ineffec- circulation or hypotension are present, tricular arrhythmias. However, in infants and
tive, the dose may be repeated and increased severe bradycardia should be treated with children it is considered ineffective unless dys-
to the maximum recommended first and adrenaline. Bradycardia caused by hypoxae- rhythmia is associated with focal ischaemia.
second doses as 6 and 12 mg. mia should treated initially with ventilation Although it has been traditionally used for
and oxygen. The dose is 20 mcg kg1 (mini- shock-resistant VF and pulseless VT, it has
mum dose 100 mcg), which may be repeated no proven efficacy. If used, the recommended
Adrenaline (epinephrine)
after 5 minutes. Unresponsive bradycardia dose is 1 mg kg1 (0.1 mL kg1 of 1%) by
Adrenaline is the most frequently used drug
should be treated with adrenaline. Atropine rapid injection followed by an infusion of
in paediatric advanced life support. Its a-
should be given prior to RSI in young 2050 mcg kg1 min1. A low infusion dose
adrenergic vasoconstrictive actions are
children to prevent the vagal stimulation of is recommended if renal or hepatic dysfunc-
considered the most important by increasing
intubation that may cause bradycardia. tion exists. Adverse side effects include myo-
aortic diastolic pressure and coronary
perfusion pressure. Its b-adrenergic actions cardial depression with hypotension and
enhance contractility and spontaneous Calcium central nervous system depression with de-
contraction. The usual dose is 10 mcg kg1 Calcium should not be administered during pression of conscious state and convulsions.
given intravenously or via the intraosseous acute resuscitation unless the cause of col-
route. It should be administered IV/IO every lapse is due to hypocalcaemia, hyperkalae- Magnesium
35 minutes at the same dosage when mia, calcium channel blocker overdose or Magnesium inhibits calcium channels and
indicated or run as an infusion of 0.05 hypermagnesaemia. Although intimately causes a reduction in intracellular calcium,
0.3 mcg kg1 min1 (0.3 mg kg1 adrenaline involved in myocardial excitation-contrac- thereby causing muscle relaxation, and is
in 50 mL at 1 mL hr1 0.1 mcg kg1 min1). tion coupling, it is not useful and possibly used as a bronchodilator in severe asthma.
Higher bolus doses (up to 200 mcg kg1) or harmful, by causing cell death, in the regular In resuscitation it is used to treat hypo-
infusions may be administered in refractory treatment of asystole, electromechanical magnesaemia and torsade de pointes VT
asystole and bradyarrhythmic states but dissociation and ventricular fibrillation. Its (see earlier) a dysrhythmia associated
there is a risk of severe vasoconstriction, use is associated with poor outcomes10 with prolonged QT interval, which may
ischaemia, hypertension and onset of ectopy and it should not be used without a definite be congenital (e.g. JervellLangeNielson
and tachyarrhythmias. If immediate access indication. If it is indicated, the dose is and RomanoWard syndromes) or acquired.
to the circulation is not available, it can be 0.2 mL kg1 of 10% calcium chloride or The causes of acquired prolonged QT
administered via the endotracheal route 0.7 mL kg1 of 10% calcium gluconate and interval are any cause of hypocalcaemia or
at a dosage of 100 mcg kg1 but absorption it may be repeated after 10 minutes accord- hypomagnesaemia, drugs (notably tricyclics,
is variable. ing to the serum level if possible. phenothiazines, type IA antiarrhythmics
such as quinidine and disopyramide, type
Amiodarone Glucose III antiarrhythmics such as amiodarone
This drug is used to treat a wide variety of Perturbations of glucose metabolism occur and sotalol), central nervous system trauma
atrial and ventricular dysrrhythmias includ- during critical illness. Infants are particularly and myocarditis/ischaemia. The dose of
ing ectopic atrial tachycardia, junctional at risk, as they have limited glycogen stores magnesium sulphate is 2550 mg kg1

33
2.3 PAEDIATRIC ADVANCED LIFE SUPPORT (PALS, APLS)

(0.10.2 mmol kg1) by intravenous or in one hand and never discharged in the been commenced and continued if an ade-
intraosseous infusion over several minutes. air. If after charging, discharge is not quate pulse rate is not detectable. The treat-
needed, the paddles should be replaced in ment of pulseless arrhythmias (ventricular
Sodium bicarbonate their storage holders before discharge. It is fibrillation, ventricular tachycardia, asystole,
Sodium bicarbonate may be used to counter important that no-one is in contact with electromechanical dissociation and pulse-
measured metabolic acidosis in prolonged the patient or the bed or trolley at the time less electrical activity) are summarised in
cardiac arrest but only after hypoventilation of discharge. Fig. 2.3.2.
has been corrected and preferably only Defibrillators should have paediatric Specific causes of arrhythmias should be
during hyperventilation. Although it can paddles of cross sectional area 1220 cm2 treated. For example, calcium channel
neutralise hydronium ions in the blood it for use in children <10 kg. For others, blockade toxicity is treated with calcium
may worsen intracellular acidosis. The prod- adult-sized paddles (5080 cm2) are satis- IV or IO (chloride 10% 0.2 mL kg1, gluco-
uct of bicarbonate and hydronium ions is factory provided the paddles do not contact nate 10% 0.7 mL kg1); hyperkalaemia
carbonic acid, which freely dissociates to each other. Selectable energy levels should treated with calcium salt, sodium bicar-
form water and carbon dioxide (CO2) and, enable delivery of doses 0.55 J kg1. bonate, hyperventilation, insulin and dex-
unless hyperventilation is given, the CO2 Doses should be rounded up to the closest trose. All drugs should be flushed into
may cross into cells, where it reforms weight-based dose. For use in the antero- the circulation with a small bolus of iso-
carbonic acid and librates hydronium ions. lateral positions, one pad/paddle is placed tonic fluid. To prevent inactivation, drugs
If indicated, an appropriate dose is over the mid-axilla opposite the xyphoid or should not be mixed in the syringe or in
1 mmol kg1. Adverse effects include hyper- nipple, the other to the right of the upper infusion lines.
natraemia and hyperosmolality, hypokalae- sternum below the clavicle. Conductive gel
mia, hypocalcaemia and metabolic alkalosis (confined to the area beneath the paddles) Asystole
(which limits oxygen dissociation from or gel pads and firm pressure are needed Asystole should be treated with adrenaline
haemoglobin). It should not be allowed to to deliver optimum energy to the heart with- (epinephrine) 10 mcg kg1 IV or IO. If
mix with catecholamines, which it inacti- out causing skin burns. An anteroposterior these routes are not available, adrenaline
vates, or with calcium salts, which it precipi- position of pads is the preferred positioning 100 mg kg1 should be administered via
tates. The essential treatment of metabolic (one over cardiac apex or anterior chest, endotracheal tube (ETT). Unresponsive asys-
acidosis is treatment of the cause. one over left scapula). Dextrocardia may tole should be treated with similar doses
be present with congenital heart disease (10 mg kg1 IV, IO; 100 mg kg1 ETT) every
Vasopressin and the position of the pads/paddles should 35 minutes. Higher doses, up to 200 mg kg1
The alternative to adrenaline as a vaso- be altered accordingly. IV or IO may be used, but have not altered
pressor, vasopressin, has no better survival In the absence of a manual dose regu- outcome and predispose to complications
advantage for adult victims of in-hospital lated defibrillator, semi-automated auto- (post-arrest myocardial dysfunction, hyper-
cardiac arrest,11 and has not been ade- matic external defibrillation (sAED) may be tension, tachycardia). In newborn infants,
quately investigated for use during CPR used for children but preferably should the initial bolus dose is 1030 mcg kg1
for children. have an attenuated adult dose. A dose of (0.10.3 mL kg1 of 1,10:000 solution).
50 J is appropriate for most infants and chil- Persistent or recurrent bradyarrhythmia or
Direct current shock dren. However, if a machine with such atte- asystole may require an infusion of adre-
Unsynchronised DC shock is required as nuated dosing is not available, the use of an naline at 0.053 mcg kg1 min1. Doses of
first treatment for VF and pulseless VT. If adult sAED delivering 150200 J is accept- <0.3 mcg kg1 min1 are predominantly
effective for VF it is called defibrillation. able for infants and children14 rather b-adrenergic, doses >0.3 mcg kg1 min1
Synchronised DC shock may also be than leave untreated a shockable rhythm. are predominantly a-adrenergic. Infuse
required for pulsatile VT and haemodyna- The use of an sAED with adult doses of DC into a secure large vein. If sinus rhythm
mically unstable supraventricular tachy- shock for children in hospital should not cannot be restored, sodium bicarbonate
cardia (SVT). be considered unless a manual dose regu- 1 mmol kg1 IV or IO may be helpful but do
Operators of defibrillators should be con- lated defibrillator is not available or cannot not allow mixing with adrenaline since
stantly alert to the possibility of inadvertent be used or a body weight specific DC shock catecholamines are inactivated in alkaline
electrocution and cardiac arrest of them- cannot be delivered within 3 minutes when solution. Pacing is not helpful for asystole.
selves and others by misuse. Pads have indicated.
advantages over paddles; they minimise risk Pulseless electrical activity (PEA)
of inadvertent shock to the operator and and electromechanical
Management of pulseless
importantly, allow application of shock with dissociation (EMD)
arrhythmias (Fig. 2.3.2)
minimal disruption to external cardiac A normal ECG complex without pulses
compression. Paddles should be charged The following discussion assumes that is called electromechanical dissociation
after placement on the patient, not before. mechanical ventilation with oxygen and (EMD). If untreated, the ECG deteriorates
Charged paddles should never be carried external cardiac compression (ECC) have to an abnormal but still recognisable state

34
2.3 PAEDIATRIC ADVANCED LIFE SUPPORT (PALS, APLS)
2

RESUSCITATION
During CPR witnessed onset of VF and immediate avail-
Airway adjuncts (LMA / ETT)
Start CPR Oxygen ability of defibrillation (first dose within
Waveform capnography
15 compressions 2 breaths
IV / IO access
30 seconds) is a stack of up to three shocks
Minimise interruptions Plan actions before interrupting compressions (each 4 J kg1)1,2 without intervening CPR
(e.g. change manual defibrillator to 4 J/kg)
Drugs recommended. If ROSC has not occurred
Shockable
within 10 seconds of any of the 3 shocks
Attach Adrenaline 10 mcg kg-1 after 2nd shock
Defibrillator / Monitor (then every 2nd loop) CPR should be given. DC shock may be more
Amiodarone 5mg kg-1 after 3rd shock
Non shockable
successful if front and back placement of pads
Adrenaline 10 mcg kg-1 immediately is used. Whatever position, use of pads rather
(then every 2nd loop)
than paddles enables minimal disruption to
continuous external cardiac compression.
Shockable Assess rhythm Non shockable
Failure of VF or pulseless VT to revert
immediately to a perfusing rhythm with
DC shock (4 J kg1) should be treated with
Adrenaline 10 mcg kg-1
Shock (4 J kg-1) (immediately then every 2nd cycle)
another single DC shock (4 J kg1) after
2 minutes of CPR. Persistent VF or pulseless
VT should be treated with adrenaline
CPR CPR 10 mcg kg1 IV or IO or 100 mcg kg1 ETT
for 2 minutes for 2 minutes followed by another single shock
Return of if necessary. Persistent (refractory) or recur-
spontaneous
circulation? rent VF or VT may be also treated
Consider and correct
Hypoxia with antiarrhythmics (amiodarone, magne-
Hypovolaemia sium) interspersed with single DC shocks fol-
Hyper / hypokalaernia / metabolic disorders
Hypothermia / hyperthermia lowed by 2 minutes of CPR. Irrespective of
Tension pneumothorax
Tamponade other drug therapy, adrenaline should be
Post resuscitation care Toxins administered every 35 minutes. Amiodar-
Thrombosis (pulmonary / coronary)
one is more efficacious than lignocaine
Post resuscitaion care
Re-evaluate ABCDE
for DC shock resistant VF and pulseless
12 lead ECG VT and is the preferred drug. The dose of
Treat precipitating causes
Re-evaluate oxygenation and ventilation amiodarone is 5 mg kg1 IV or IO over sev-
Temperature control (cool)
eral to 60 minutes. It may be repeated
Fig. 2.3.2 Advanced cardiopulmonary resuscitation for infants and children. Adapted from to maximum of 15 mg kg1. If amiodarone
resuscitation guidelines of the International Liaison Committee on Resuscitation, Australian is not available, lignocaine may be used
Resuscitation Council, European Resuscitation Council and of the American Heart Association,
in a dose of 1 mg kg1 IV or IO bolus
December 2010. Australian Resuscitation Council. Key: CPR, cardiopulmonary resuscitation; ECG,
electrocardiograph; IO, intraosseous; IV, intravenous; J, joules; kg, kilogram; mg, milligram; mcg, followed by an infusion if successful
microgram. at 2050 mcg kg1 min1. Magnesium, 25
50 mg kg1 (0.100.20 mmol kg1) is indi-
cated for polymorphic VT (torsade de pointes).

when it is called pulseless electrical activity Either monophasic or biphasic waveforms

(PEA). Both conditions should be treated may be used. The optimum dose of external
as for asystole and their causes ascertained
Management of pulsatile
DC shock in terms of achieving first shock
and treated. success with minimal damage to the myocar-
dysrhythmias
dium is unknown. Some guidelines recom- Bradysrhythmias
mend a dose of 24 J kg1.3,4 The dose of In all age groups, bradycardia is defined as a
Ventricular fibrillation and 2 J kg1 is considered too little by other heart rate <60 min1 or rapidly declining
pulseless ventricular tachycardia guidelines1,2 which advise 4 J kg1. This is rate with poor perfusion. Sinus bradycardia,
In approximately 10% of paediatric cardiac supported by a recent study which showed sinus arrest with slow junctional or idioven-
arrests the initial identified rhythm is VF or that 2 J kg1 converted only about 50% of tricular rhythm and atrioventricular block
pulseless VT. As soon as recognised, VF or patients to a perfusing rhythm.12 In contrast are the most common preterminal arrhyth-
pulseless VT should be treated with unsyn- to previous recommendations, it is now mias in paediatric practice. Untreated,
chronized DC shock. If onset is witnessed recommended to give one shock followed bradycardia will potentially progress to asys-
in a monitored environment, a precordial immediately by uninterrupted CPR for 2 min- tole. The treatment is reversal of the cause
thump may be given but its efficacy has utes without pausing to determine if another (hypoxaemia, hypotension, acidosis, hypo-
not been proven. shock is required. Only in monitored thermia, intracranial hypertension) and, if

35
2.3 PAEDIATRIC ADVANCED LIFE SUPPORT (PALS, APLS)

unresponsive, adrenaline 10 mcg kg1 IV tachycardia, lest drug or other treatment synchronised at
2 J kg1 under sedation/
or IO or 100 mcg kg1 ETT. Bradycardia exacerbate the situation. A history related anaesthesia.
caused by vagal stimulation should be to the tachycardia and a 12-lead ECG should
managed with cessation of the stimulus be analysed carefully. If the diagnosis is not
(e.g. oropharyngeal suctioning, laryngos- obvious, the rate and duration of the QRS Supraventricular tachycardia
copy) and/or atropine 20 mcg kg1 IV or IO are starting points to differentiate sinus SVT is the most common spontaneous
(minimum dose 100 mg). Persistent vagal- tachycardia (ST), ventricular tachycardia arrhythmia in childhood and infancy. Some
mediated bradycardia should be treated with (VT), supraventricular tachycardia (SVT) infants may tolerate this rhythm for long per-
adrenaline 10 mcg kg1 IV or IO. If facilities and wide QRS-complex SVT. iods; however, it may cause life-threatening
are available, pacing (oesophageal, trans- hypotension. It is usually re-entrant with a rate
cutaneous, transvenous, epicardial) may be of 220300 min1 in infants, usually less
effective if sinus node dysfunction or heart in children (approximately 180 min1). The
block exist. Pulsatile ventricular tachycardia QRS complex is usually narrow (<0.08
Haemodynamically stable VT may be treated seconds), making it difficult sometimes to
with an antiarrhythmic agent such as amio- discern from sinus tachycardia. However,
Tachydysrhythmias (Fig. 2.3.3) darone (5 mg kg1 IV over 2060 minutes) whereas ST is a part of other features of
Any heart rate above normal for age or procainamide (15 mg kg1 IV over illness, SVT is a singular entity and whereas
should be considered a tachydysrhythmia, 3060 minutes) or lidocaine (1 mg kg1 the rate in ST is variable with activity or
particularly if associated with poor circula- IV over 24 minutes). Note that both amio- stimulation, it is uniform in SVT and often
tion and hypotension and if the patient darone and procainamide prolong QT inter- of sudden onset and offset. In both rhythms,
has a history of cardiac disease, has had val and should not be given together. a P wave may be discernible.
cardiac surgery or could have been poi- If torsade de pointes (twisting of the peaks) If haemodynamically stable (adequate
soned with cardioactive drugs. Of course, is present, magnesium (2550 mg kg1, perfusion and blood pressure), initial treat-
such tachycardia may be the result, rather 0.10.2 mmol kg1 IV) may be used. If ment of SVT should be vagal stimulation.
than the cause of poor circulation, i.e. sinus pulses are present but accompanied by For infants and young children, application
tachycardia (ST). It is important to deter- hypotension and poor circulation, cardiover- to the face of a plastic bag filled with iced-
mine the type and aetiology of the sion is needed, in which case it should be water is often effective, or alternatively

Treat as pulseless VT No Are pulses palpable?

Yes

History, exam, ECG

What is the QRS duration?
< 0.08s > 0.08s

Sinus tachycardia Supraventricular tachycardia Wide QRS complex Ventricular tachycardia

supraventricular tachycardia

Treat cause What is the BP/perfusion? What is the BP/perfusion? What is the BP/perfusion?

Normotensive Hypotensive Normotensive Normotensive Hypotensive

Vagal stimulation Synchronised DC shock Vagal stimulation Amiodarone 5 mg kg-1

1J kg-1 (biphasic or OR
monophasic) Procainamide 15 mg kg-1
Adenosine Adenosine OR
Try vagal stimulation and
100 mcg kg-1 100 mcg kg-1 Lidocaine 1 mg kg-1 Magnesium 25 mg kg-1
adenosine while preparing
200 mcg kg-1 200 mcg kg-1 Torsades de pointes
but dont delay

Synchronised DC shock Synchronised DC shock

1J kg-1 (biphasic or 2 J kg-1 (biphasic or
monophasic) monophasic)

Fig. 2.3.3 Management of pulsatile tachydysrhythmias.

36
2.3 PAEDIATRIC ADVANCED LIFE SUPPORT (PALS, APLS)
2

RESUSCITATION
submersion of the face into a slurry of ice cardiorespiratory arrest. The cause of the patient, remediable factors, likely outcome
and water in a bowl. Older children may CPA should be investigated and treated if ultimately successful, opinions of person-
be treated with carotid sinus massage or appropriately, e.g. sepsis or drug overdose. nel familiar with the patient and, whenever
asking them to perform a Valsalva manoeu- Particular care should be taken to ensure appropriate, the wishes of informed parents.
vre such as blowing through a narrow adequate cerebral perfusion with well- In general, unless hypothermia or drug tox-
straw. If unsuccessful, give adenosine oxygenated blood. Hyperventilation to hypo- icity exists, survival to normality is most
initially at 100 mcg kg1 IV by rapid carbic levels is contraindicated because of unlikely if there has been a failure to
bolus (max dose 6 mg), increasing to potential harmful cerebral vasoconstriction. respond to full CPR after 30 minutes and
200 mcg kg1 or 300 mcg kg1. In older chil- Survival and neurological outcome is bet- several doses of adrenaline, unless environ-
dren, it is important to describe the transient ter when deliberate hypothermia is used after mental hypothermia was an important
feeling of chest heaviness or breathing cardiac arrest. ILCOR has recommended ther- aetiological or consequential factor. In the
difficulty or fearfulness that may accom- apeutic hypothermia (3234 C) for 1224 newly-born infant, discontinuation of
pany adenosine administration, and/or pre- hours for adults and children1 who remain treatment is appropriate if CPR does not
cede the adenosine with a small amnestic unconscious with spontaneous circulation establish a spontaneous circulation within
dose of midazolam. If unsuccessful, give after out-of-hospital cardiac arrest when the 15 minutes.1 Family members should be
synchronised DC shock (cardioversion)13 initial rhythm was ventricular fibrillation, kept informed, allowed to be present or
initially at 0.51.0 J kg1 but subsequently and suggested that for any other rhythm, asked if they want to be present during
up to 2 J kg1. If at the outset SVT is or cardiac arrest in hospital, such cooling resuscitation (see Chapter 2.1).
accompanied by haemodynamic instability, may also be beneficial. The optimal duration
proceed to cardioversion (synchronised of such requirement, however, is unknown
1.0 J kg1) immediately, although vagal but clinical studies among newborns suggest Post-resuscitation staff
stimulation or adenosine (IV or IO) may be 72 hours.14 Inadvertently hypothermic management
used, provided they do not delay cardiover- patients, provided temperature is above
sion. Verapamil should not be used to treat 32 C or greater should not be actively Unfortunately, CPA occurring in hospital is
SVT in infants and should be avoided in warmed and hyperthermia should be aggres- often unexpected as when, for example, a
children because it induces hypotension by sively treated. If deliberate hypothermia is moribund patient arrives unannounced in
vasodilation and negative inotropic effect. employed, shivering should be prevented the emergency department, or a patients
with sedation and/or neuromuscular block- condition deteriorates rapidly on the ward
ade. Seizures should be actively sought and or when a mishap occurs under anaesthesia.
Wide QRS complex
treated with anticonvulsant. These situations test the readiness, training,
supraventricular tachycardia
Complications of CPR should be sought abilities and skills of individuals and the
SVT with aberrant conduction may cause a
especially if secondary deterioration occurs. organisation of the institution. It is prudent
wide-complex QRS (>0.08 seconds) and
A chest X-ray should be obtained to check to monitor performance with a view to
thus may resemble VT. If the blood pressure
the position of the endotracheal tube, to improvement and not ignore the psycho-
is low or circulation deemed inadequate,
exclude pneumothorax, lung collapse, con- logical impact that such events have on
the rhythm should be regarded as VT and
tusion or aspiration and to check the cardiac individuals. Sensitive debriefing sessions
treated with synchronised DC shock at
silhouette. Invasive blood pressure mea- may be helpful to members of staff involved

2 J kg1. If pulses are absent, the rhythm
surement and periodic echocardiographic post resuscitation.
should be regarded as pulseless VT and
treated accordingly with DC shock. or bedside ultrasound examination are
needed to specifically check contractility
Prevention of
and to exclude a pericardial effusion. Mea-
cardiorespiratory arrest
surement of haemoglobin, pH, gas tensions,
Post-resuscitation
electrolytes and glucose are important. Of course, hospital personnel should be well-
management
trained and organised to treat unexpected
Supportive therapy should be provided until cardiorespiratory arrest, but the far
there is recovery of function of vital organs. preferable course of action is prevention.
This may require provision of oxygen therapy,
Cessation of CPR Some paediatric hospitals have instituted
mechanical ventilation, inotropic/vasopres- Long-term outcome from paediatric CPR is Rapid Response Team systems or Medical
sor infusion, renal support, parenteral nutri- poor, with approximately 510% of patients Emergency Team systems, which can
tion and other therapy for several days or surviving out-of-hospital arrest15 and respond rapidly to deterioration in a
longer. Recovery of infants and children 2550% in-hospital cardiac arrest.16,17 patients condition before cardiorespiratory
is usually slow because cardiorespiratory The decision to cease CPR should be based arrest occurs, with consequent significant
arrest is often secondary to prolonged global on a number of factors including the dura- reductions in unexpected cardiac arrest
ischaemia or hypoxaemia, which implies tion of resuscitation, its quality, response (38%) mortality18,19 (21%) and unplanned
that other organs sustain damage before to treatment, pre-arrest status of the admission to intensive care.

37
2.4 SPECIFIC PAEDIATRIC RESUSCITATION

6. Tibballs J, Weeranatna C. The influence of time on the fibrillation and pulseless ventricular tachycardia. Pediatr
References accuracy of healthcare personnel to diagnose paediatric
cardiac arrest by pulse palpation. Resuscitation 2010;81 13.
Crit Care Med 2011;12:1420.
Tibballs J, Carter B, Kiraly NJ, et al. Biphasic DC shock
1. Australian Resuscitation Council Guidelines. Melbourne. (6):6715. cardioverting doses for paediatric atrial dysrhythmias.
p. 12.112.7. http://www.resus.org.au. 7. Carter BG, Fairbank B, Tibballs J, et al. Oxygen delivery Resuscitation 2010;81:11014.
2. Biarent D, Bimgham R, Eich C, et al. European using self-inflating resuscitation bags. Pediatr Crit Care 14. Kochanek PM, Fink E, Bell MJ, et al. Therapeutic
Resuscitation Council Guidelines for Resuscitation 2010 Med 2005;6:1258. hypothermia: applications in pediatric cardiac arrest.
Section 6. Paediatric life support. Resuscitation 8. Gausche M, Lewis RJ, Stratton SJ, et al. Effect of out- J Neurotrauma 2009;26:4217.
2010;81:136488. of-hospital pediatric endotracheal intubation on survival 15. Deasy C, Bernard SA, Cameron P, et al. Epidemiology
3. Kleinman ME, Chameides L, Schexnayder SM, et al. and neurological outcome: a controlled clinical trial. of paediatric out-of-hospital cardiac arrest in
Part 14: Pediatric advanced life support: 2010 JAMA 2000;283:78390. Melbourne, Australia. Resuscitation 2010;81:1095100.
American Heart Association guidelines for 9. Benumot JL. Laryngeal mask airway and the ASA 16. Tibballs J, Kinney S. A prospective study of outcome of in-
cardiopulmonary resuscitation and emergency difficult airway algorithm. Anesthesiology patient paediatric cardiopulmonary arrest. Resuscitation
cardiovascular care. Circulation 2010;122: 1996;84:68699. 2006;71:3108.
S876S908. 10. Srinivasan M, Morris MC, Helfaer MA, et al. Calcium use 17. Meert KL, Donaldson A, Nadkarni V, et al. Multicenter
4. de Caen AR, Kleinman ME, Chameides L, et al. during in-hospital pediatric cardiopulmonary cohort study of in-hospital pediatric cardiac arrest.
Part 10: Paediatric basic and advanced life support: resuscitation: a report from the National Registry of Pediatr Crit Care Med 2009;10:54453.
2010 International Consensus on Cardiopulmonary Cardiopulmonary Resuscitation. Pediatrics 2008;121: 18. Chan PS, Jain R, Nallmothu BK, et al. Rapid response
Resuscitation and Emergency Cardiovascular Care e114e151. teams. A systematic review and meta-analysis. Arch
Science with Treatment Recommendations. 11. Stiell IG, Hebert PC, Wells GA, et al. Vasopressin versus Intern Med 2010;170:1826.
Resuscitation 2010;81:e213e259. epinephrine for inhospital cardiac arrest: A randomised 19. Tibballs J, Brilli RJ. Pediatric RRSs. In: De Vita MA,
5. Tibballs J, Russell P. Reliability of pulse palpation by controlled trial. Lancet 2001;358:1059. Hillman K, Bellomo R, editors. Textbook of Rapid
healthcare personnel to diagnose paediatric cardiac 12. Tibballs J, Carter B, Kiraly NJ, et al. External and internal Response Systems: Concepts and Implementation.
arrest. Resuscitation 2009;80:614. biphasic DC shock doses for pediatric ventricular New York: Springer; 2011. p. 23143.

2.4 Specific paediatric resuscitation

James Tibballs

Anaphylaxis prognostic indicators are prolonged duration perfusion with well oxygenated blood and
of submersion, lack of bystander CPR, pro- the avoidance of factors that decrease cere-
Persons receiving b-blocking drugs have a longed prehospital resuscitation, pulseless bral perfusion pressure. It is thus vital to
potentiated risk of anaphylaxis. In such arrhythmia on arrival at hospital, fixed dilated restore cardiac output and blood pressure,
patients, it is more resistant to therapy and pupils, severe acidosis and apnoea. Nonethe- to oxygenate blood and to avoid factors that
lasts longer. Hypotension may be refractive less, vigorous resuscitation should be insti- would increase intracranial pressure, such as
to adrenaline. In such cases, glucagon (ther- tuted on arrival in hospital of the pulseless venous obstruction. Hypocapnia, hypercap-
apy for b-blocker toxicity) may be required victim, if not already commenced by ambu- nia, hypoglycaemia and hyperglycaemia
along with infusions of adrenaline and lance personnel, in order to clarify the clinical should be avoided and convulsions treated.
dopamine. See Chapter 22.5 for the detailed details whilst continuing resuscitation. Any pulseless arrhythmia may be encoun-
account of the treatment of anaphylaxis. Intubation and mechanical ventilation with tered and should be managed along
100% oxygen should be instituted immedi- standard lines.
ately. Regurgitation of stomach contents There are no important clinical differ-
Drowning should be anticipated and a rapid sequence ences between fresh and salt-water immer-
Victims of submersion incidents suffer global intubation technique with cricoid pressure sion. Altered levels of serum electrolytes,
hypoxaemia and if arrested, global ischaemia. should be used. Sedative drugs with cardiovas- especially sodium and potassium, may be
Associated injuries are aspiration pneumonitis cular depressive actions should not be used, or detected, but are uncommon and in any case
and hypothermia. Aspiration of water and gas- in minimally required doses only. The lung do not influence acute resuscitation.
tric contents is common (see Chapter 22.2). In compliance is likely to be poor and it may
addition, hypothermia (see Chapter 22.4) be necessary to insert a larger-than-usual
may be present, but unless the victim was sub- uncuffed or a cuffed endotracheal tube Toxicological emergencies
ject to severe environmental hypothermia (preferred) to prevent a leak around the tube, The standard resuscitation protocols may
such as being submersed in ice-cold water to obtain adequate lung inflation in the be inadequate in some toxicological emer-
(<5 C) or has profound afterdrop after setting of acute respiratory distress syndrome gencies, particularly when poisoning has
removal from water, this reflects lack of perfu- (ARDS) in order to achieve oxygenation. After occurred with cardioactive drugs.
sion and is a bad prognostic sign. Hypothermia restoration of cardiac rhythm myocardial con-
should be treated but temperature not permit- tractility should be measured with echocardi- Airway and ventilation
ted to rise above 35 C if cardiac arrest has ography and optimised with inotropic agents. management
occurred (see Chapter 2.3). During resuscitation, the goal is to provide Problems should not be created by treatment.
The outcome is often determined by maximum opportunity for cerebral recovery Gastric lavage should not be undertaken
the extent of neurological injury. Bad and this is achieved by restoring cerebral unless the patient has ingested a significant

38
2.4 SPECIFIC PAEDIATRIC RESUSCITATION
2

RESUSCITATION
toxin in significant amounts within the past preferred antidote because it stimulates Both components of the sodium bicarbonate
hour. There are very few indications for non-catecholamine cAMP. Isoprenaline contribute to its antidotal effect the
gastric lavage. Gastric lavage should not 0.053 mcg kg1 min1 by infusion may sodium counters the sodium channel block-
be performed in the fully conscious or in be used but it may cause b2-induced ade effect of the drugs and the alkalosis
the less-than-fully conscious patient, with- hypotension and should not be used if hypo- reduces bioavailability. Induction of res-
out prior securement of the airway with tension is pre-existing. Alternatively, an piratory alkalosis is also beneficial. The pre-
rapid sequence endotracheal intubation. infusion of adrenalin (epinephrine) at 0.05 ferred antidysrrhythmic agent is lidocaine.
Likewise, activated charcoal should not be 1 mcg kg1 min1 may be used. Phenytoin is no longer recommended.
administered by oro/nasogastric tube in a Digoxin induced bradycardia may be Polymorphic VT (torsade de pointes) of any
patient who is less-than-fully conscious, irre- treated with Digoxin Fab antibody in a dose cause should be treated with magnesium
spective of the interval since ingestion. of 10 vials per 25 of 0.25 mg tablets or per 2550 mg kg1 IV.
The risk of aspiration pneumonitis in these 5 mg elixir ingested. At steady state, the Pulseless VT or VF require DC shock. The
circumstances is significant. If activated number of vials is calculated as serum use of adrenaline (epinephrine) in this situa-
charcoal is to given by naso/orogastruc digoxin (ng mL1)
(kg)/100. tion should be limited to 10 mcg kg1
tube, confirm it is in the stomach, not the boluses while higher doses which may
lungs, prior to instilling the charcoal. Drug-induced tachyarrhythmias cause recurrent VF or VT should be avoided.
The preferred treatment is a benzodiazepine Propranolol is contraindicated.
Drug-induced asystole such as diazepam or midazolam in doses
Infusions of KCl are hazardous. Molar solu- that do not depress CNS function. Other Drug-induced shock
tions of KCl are in common use in intensive treatments are the ultrashort-acting Shock may result from drugs that cause
care units especially after cardiac surgery. cardioselective b1 blocker esmolol IV depression of myocardial contractility, vaso-
They should not be available for regular ward 500 mcg kg1 over 1 minute and then 25 dilatation or loss of intravascular volume, or
use. An inadvertent IV bolus of potassium 200 mcg kg1 min1 by infusion. Treat- combinations of these. An extreme rise in
can cause asystole. Note that a 1 mL bolus ments such as intravenous adenosine and peripheral vascular resistance may also lead
of molar KCl (1 mmol) in a 10 kg child will the- DC shock are not likely to be effective to myocardial failure. Whenever possible
oretically raise the serum concentration by because the toxic drug effect will be persis- preload, contractility and afterload should
2.5 mmol L1 and cause immediate asystole. tent. Calcium channel blockers such as be measured via a central venous line.
Immediately acting treatment (within verapamil may cause severe hypotension Preload deficiencies (hypovolaemia) are
seconds) for hyperkalaemia is either 10% and should not be given. corrected with volume administration, titrated
calcium chloride IV 0.2 mL kg1 (or equiva- against blood pressure, right heart filling pres-
lent) or IV sodium bicarbonate 1 mmol kg1 Drug-induced hypertension sure and against pulmonary artery wedge
or both. Calcium antagonises the cardiac Hypertension may occur with poisoning by pressure.
effects of potassium on the heart, while cocaine, amphetamine and similar substances, Infusion of dopamine is a suitable inotro-
the bicarbonate lowers the serum concen- which cause vasoconstriction, tachycardia pic agent in doses of approximately
tration of potassium by a small amount. and arrhythmias. Benzodiazepines are the pre- 515 mcg kg1 min1, although higher
Better treatments of rapid onset (within ferred treatment. Another alternative is a doses may be needed or other inotropic
minutes) are glucose 0.5 g kg1 IV (e.g. smooth muscle dilator such as sodium nitro- drugs added. If central access is not possible,
5 mL kg1 of 10%) plus insulin 0.05 units kg1 prusside IV 0.52 mcg kg1 min1, but its use infusion of dobutamine in a similar dose
or salbutamol 0.25 mg kg1 by aerosol or mandates intra-arterial monitoring. Another may be given via a peripheral venous
both. Slow treatment (within hours) is by choice is phentolamine IV 0.050.1 mg kg1 access, at least in the short term. Other
resonium, oral or rectal, 0.51 g kg1. and then an infusion of 550 mcg kg1 min1. inotropic agents are infusions of calcium
Propranolol, a non-selective b-blocker is (0.030.1 mL kg1 hr1 of 10% CaCl2, i.e.
Drug-induced bradycardia contraindicated because it may paradoxically 310 mg kg1 hr1), milrinone (50 mcg kg1
Organophosphate and carbamate poisoning worsen hypertension when b2-adrenoreceptors loading dose then 0.3750.75 mcg kg1
with bradycardia should be treated with are blocked. min1) and glucagon. It is desirable to mea-
atropine repeated doses of 2050 mcg kg1 sure cardiac output or to assess contractility
every 15 minutes or until secretions are dry Drug-induced ventricular with echocardiography.
and an acceptable heart rate is restored. tachycardia (VT) and fibrillation Vasodilatation is treated with an a-
In organophosphate poisoning (but not (VF) adrenergic agent such as noradrenaline
carbamate), pralidoxime can reactivate cholin- Haemodynamically stable VT can be treated (norepinephrine) infusion (0.051 mcg
esterase. The dose is 25 mg kg1 IV over 15 with anti-arrhythmic agents. Typical drug kg1 min1) in the higher dose range.
30 minutes then 1020 mg kg1 per hour for causes are tricyclic antidepressants, cocaine Other such agents are phenylephrine
18 hours or more. and amphetamines. These cause a mono- (1-5 mg kg1 min1) and metaraminol
Bradycardia induced by b-blocker poisoning morphic VT by increasing the QRS interval. (0.051 mcg kg1 min1). Vasoconstriction
is treated with glucagon 7 mcg kg1 IV Treatment should be sodium bicarbonate, is also obtained with vasopressin infusion
then 27 mcg kg1 min1. This is the especially in cases of tricyclic poisoning. (0.0020.01 units kg1 min1).

39
2.4 SPECIFIC PAEDIATRIC RESUSCITATION

In selected circumstances, cardiac output a prolonged period. Although paralysis (Chironex fleckeri) can kill rapidly the exact
can be supported with ventricular assist caused by some animals, e.g. blue-ringed cause is still unknown but it may be related
devices, extra-corporeal membrane oxyge- octopuses, may be relatively brief, lasting in part to dysrhythmia caused by hyperkalae-
nation (ECMO) or intra-aortic balloon pump- perhaps several hours (if support is given), mia secondary to haemolysis, or rapid onset
ing, although the last named requires paralysis caused by some snake and funnel- of myocardial cytolysis caused by cell mem-
intrinsic cardiac rhythm. These techniques web spider venoms may be lengthy. For brane pore-forming toxins.5
are sometimes used in toxicological emer- example, a child considered to have been
gencies in centres that use these techniques bitten by a Rough-scaled snake remained Hypertension
for cardiac surgery. They are only indicated ventilator dependent for 10 weeks, although Severe hypertension may occur after bites
when standard supportive measures are antivenom administration in that case was from funnel-web spiders and after stings from
insufficient but the situation is recoverable, delayed.2 jellyfish causing Irukandji syndrome. This is
and organ damage, particularly brain dam- in part caused by release of endogenous cate-
age, has not occurred. The principal risks Coagulopathy and haemorrhage cholamines. Treatment could be with vasodi-
with such techniques are haemorrhage, Snake-bite-induced coagulopathy, and hae- lators, a-and b-adrenergic blockade. The
infection and mechanical failure. morrhage and shock, may be prolonged infusion of magnesium may ameliorate the
and may not respond readily to administra- components of the Irukandji syndrome.6
tion of antivenom and clotting factors. Anti-
Envenomation venom alone does not per se restore normal Adverse reactions to antivenom
coagulation. In the presence of haemor- The treatment of some envenomations
The main principles of resuscitation, restora- rhage, it is prudent to administer clotting by antivenom may be complicated by reac-
tion of airway, breathing and circulation factors if normal coagulation is not restored tions to antivenom. Adverse reactions to
apply to victims of envenomation, but with soon after antivenom administration, since Australian snake antivenoms occur in
additional special requirements related to serum levels of coagulation factors are not approximately 813% of cases which is
the effects of venoms and the treatment of restored by liver function for approximately relatively small compared to some overseas
envenomation.1 6 hours after complete consumption of clot- manufactured antivenoms, but not insignifi-
Numerous venomous Australian terres- ting factors during DIC. In the absence of cant. It is thus prudent to prevent
trial and marine creatures may threaten life any ability to measure the serum concentra- anaphylaxis by premedication with subcu-
(see Chapter 22.1). Snakes, spiders, ticks, jel- tions of venom components it is difficult to taneous adrenaline (epinephrine) 0.25 mg
lyfish, octopuses and cone shells inject lethal judge how much antivenom is required for an adult, 510 mg kg1 for a child. This
venoms whereas the venoms of bees, ants when the sole effect of venom is coagulopa- recommendation stems from high-level sci-
and wasps cause anaphylaxis. The number thy. Hitherto recommended neutralisation entific evidence: A prospective, double blind,
of deaths due to envenomation in Australia doses of antivenom have been found inade- randomised, placebo-controlled trial of
is two to four per year. A similar number of quate in canine and human plasma models 0.25 mg of subcutaneous adrenaline as pre-
deaths, one to three per year, are due to ana- of envenomation and coagulopathy.3,4 medication for snake antivenom in Sri Lanka
phylactic reactions to bee and wasp stings. found that subcutaneous adrenaline
Death and critical illness is due to: Rhabdomyolysis reduced the reaction rate from 43% to
(1) rapid onset neurotoxicity, with respira- Some envenomations cause rhabdomyolysis 11% (p 0.0002) and reduced the severity
tory failure and airway obstruction by bulbar with implications for renal function, electro- of reactions.7 Thus, although adrenaline has
palsy; (2) haemorrhage and shock; and lyte disturbances (hyperkalaemia, hypo- a potential to cause cerebral haemorrhage in
(3) renal failure secondary to rhabdomyoly- calcaemia) and muscle weakness. This is a snake-venom induced coagulopathic state
sis, disseminated intravascular coagulation expected after envenomation by certain when given intravenously or intramuscularly,
(DIC; consumption coagulopathy, defibrin- snakes (e.g. tiger snakes, black snakes, but not when given subcutaneously,8 it
ation), haemorrhage or haemolysis. Rapid car- Beaked sea snake) and funnel-web spiders, should be administered. This controversial
diovascular collapse within minutes after a particularly if antivenom therapy is inade- subject was subject to a systematic review,9
snake bite may be due to anaphylaxis to quate or delayed. Myocardial muscle may which concluded: If clinicians believe local
venom, acute vasodilatation or to dissem- be involved. factors do not justify routine adrenaline,
inated intravascular coagulation-provoked then they should test their belief in a rando-
pulmonary hypertension and cor pulmonale. Cardiac dysrhythmias mised trial. In contrast, more high-level
Particular resuscitation problems en- Cardiac dysrhythmias have been observed evidence, a randomised, double blind,
countered after envenomation include the after envenomation by some creatures. The placebo-controlled trial of promethazine as
following. venom of funnel-web spiders and that of a premedication for snake antivenom in
the Irukandji jellyfish cause massive release Brazil10 did not alter the reaction rate (25%
Respiratory failure and bulbar of endogenous catecholamines, which may placebo, 24% promethazine) and was thus
palsy be responsible for tachydysrhythmia, stress- not beneficial. Moreover, the effects of pro-
Many venoms contains neurotoxins. Respira- induced (Takotsubo) cardiomyopathy and methazine, hypotension and CNS obtundation
tory and airway support may be required for ischaemia. The venom of the Box jellyfish might exacerbate the illness caused by

40
2.5 SHOCK
2

RESUSCITATION
envenomation. Promethazine is therefore con- venom access to the general circulation 4. Sprivulis P, Jelinek GA, Marshall L. Efficacy and potency of
antivenoms in neutralising the procoagulant effects of
traindicated as a premedication. and exerting systemic effects. Australian snake venoms in dog and human plasma.
Unfortunately, application of a bandage is Anaesth Intensive Care 1996;24:37981.
5. Corkeron MA. Magnesium infusion to treat Irukandji
not always performed well or in a timely syndrome. Med J Aust 2003;178:411.
Pressure-immobilisation manner both before and after arrival of 6. Bailey PM, Bakker AJ, Seymour JE, Wilce JA. A functional
bandage comparison of the venom of three Australian jellyfish
the victim at hospital. A critically ill victim Chironex flekeri, Chiropsalmus sp., and Carybdea
Application of a pressure-immobilisation should have a bandage applied in the Emer- xaymacana on cytosolic Ca2, haemolysis and Artemia
bandage is essential in the early manage- sp. Lethality. Toxicon 2005;45:23342.
gency Department, if one is not already in 7. Premawardhena AP, de Silva CE, Fonseka MM, et al. Low
ment of selected envenomations. Elasticised place on arrival and it should not be dose subcutaneous adrenaline to prevent acute adverse
bandages maintain pressure better than reactions to antivenom serum in people bitten by snakes:
removed until antivenom, if indicated, has Randomised, placebo-controlled trial. Br Med J
non-elasticised bandages. Delay in applica- been administered. 1999;318:10413.
tion or premature removal of a bandage 8. Tibballs J. Premedication for snake antivenom. Med J
Aust 1994;160:47.
may severely compromise resuscitation. 9. Nuchpraryoon I, Garner P. Interventions for preventing
Envenomations by snakes, funnel-web spi- reactions to snake antivenom. Cochrane Database Syst
References Rev 2003;1.
ders, blue-ringed octopuses, and cone shells 1. Sutherland SK, Tibballs J. Australian Animal Toxins. 10. Fan HW, Marcopito LF, Cardoso JL, et al. Sequential
all warrant application of a bandage. The Melbourne: Oxford University Press; 2001. randomised and double-blind trial of promethazine
2. Patten BR, Pearn JH, DeBuse P, et al. Prolonged intensive prophylaxis against early anaphylactic reactions to
venoms of these creatures contain compo- therapy after snake bite. Med J Aust 1985;142:4679. antivenom for bothrops snake bites. Br Med J
nents that cause rapid onset of paralysis. 3. Tibballs J, Sutherland SK. The efficacy of antivenom in 1999;318:14512.
prevention of cardiovascular depression and 11. Sutherland SK, Coulter AR, Harris RD. Rationalisation of
The bandage serves to maintain venom at coagulopathy induced by brown snake (Pseudonaja) first-aid measures for elapid snakebite. Lancet 1979;
the bite or sting site11 and hence prevents species. Anaesth Intensive Care 1991;19:5304. I:1836.

2.5 Shock
Kevin Mackway-Jones

The adequacy of circulation (and thereby

ESSENTIALS the adequacy of tissue perfusion) requires
proper functioning of the heart, vessels and
1 Shock is a syndrome that arises because of acute failure of the circulation resulting blood. The heart must pump enough blood
in inadequate tissue perfusion. It may result from hypovolaemic, cardiogenic,
to meet peripheral oxygen demand; the ves-
distributive, obstructive or dissociative causes.
sels that deliver the blood to both the lungs
2 The normotensive child may have profound compensated shock. and the other organs must be patent (that
is not obstructed), be regulated appropriately
3 As tachycardia may be a non-specific sign and hypotension occurs late, it is crucial at a macro level to ensure delivery of blood at
to recognise the early features of shock in a child by assessing indices of peripheral
an appropriate pressure to the organs requir-
perfusion and alterations of end organ function.
ing oxygen, and must function appropriately
4 The initial management of shock should be volume expansion with a bolus of in the periphery so as to allow oxygen diffu-
20 mL kg1 crystalloid. Further boluses are given according to clinical response. sion without fluid loss. The blood must
Occasionally, in the exsanguinating child, universal donor blood is indicated. have sufficient oxygen carrying capacity and
must maintain the ability to allow oxygen
5 The ongoing management of the shocked child will depend on the specific cause exchange. Failure of any aspect of this compli-
and may include interventions such as: oxygenation, ventilation, glucose
cated system will result in inadequate periph-
administration, cardioversion, broad-spectrum antibiotics, inotropic support, ductus
eral tissue perfusion and therefore shock.
arteriosus manipulation, adrenaline (epinephrine), atropine or surgical intervention.
Conventionally, shock is divided into the
following five types:

complex construct, having many causes hypovolaemic

Introduction and many expressions. The clinical diagnosis cardiogenic
Shock, in the sense described in this chapter, and management of shock is complicated distributive
is a syndrome that arises because of acute by the fact that many organ systems become obstructive
failure of the circulation. This acute circula- involved and because many of the signs dissociative.
tory failure results in inadequate tissue of shock actually arise because of the
oxygenation and inability to remove the bodys attempts at homeostasis rather than Hypovolaemic shock arises when there is
waste products of metabolism. Shock is a because of the underlying process. circulatory volume inadequacy. This classically

41
2.5 SHOCK

arises in trauma following haemorrhage or be examined. Oxygen saturation and respi- other area with visible circulation for some
burns but it is also seen following gastrointes- ratory rate should be monitored. 5 seconds and then measuring the length
tinal fluid losses such as diarrhoea and vomit- of time it takes for the blanching to disap-
ing or third space losses such as in volvulus. Circulation pear. Central capillary refill such as over
Cardiogenic shock occurs when the heart fails This is the crux of shock assessment since the sternum is more reliable than the
to pump enough fluid. This can occur because homeostatic mechanisms function particu- peripheries, which can be influenced by
the heart itself is failing secondary to infec- larly well in children and compensation for vasoconstriction due to the ambient tem-
tion, because the heart muscle has been inadequate circulatory function is good. perature effects. A capillary refill time of less
injured (such as in cardiac contusion) or Consequently, in a child it can be difficult than 2 seconds is normal. A capillary refill
because of a problem with rhythm where to detect circulatory failure until a late time of greater than 4 seconds is definitely
the heart is beating too slow or too fast to stage, hence one needs to be vigilant in abnormal. Capillary refill between 2 and
achieve an adequate cardiac output. In distrib- detecting the earlier features of compensat- 4 seconds should prompt further consider-
utive shock, peripheral vascular abnormalities ing shock. ation of the presence of shock.
result in failure to appropriately distribute Circulatory assessment should look at Changes in peripheral circulation (which
pumped blood. This can be because of infec- both circulatory status and also at the are brought about by an increase in cate-
tion (as in the septic child), allergic reaction, effects of inadequate circulation on other cholamines) may also show as an increased
spinal cord injury or drugs. Obstructive shock organ systems. core to periphery temperature difference
arises when there are abnormalities of flow. Heart rate and blood pressure are the (more than 2 C is a sign of poor perfusion)
This can either be because particular vessels classical measures of circulatory status but or as a noticeable centripetal temperature
themselves are obstructed (such as in changes in skin perfusion (as shown by cap- gradient. Reduced skin perfusion may result
pulmonary embolus) or because of extra vas- illary refill and by skin pallor or temperature) in a mottled, cyanosed, pale appearance
cular abnormalities which obstruct the flow of can be more useful in detecting the early and coolness on palpation. Some infants
blood (such as tension pneumothorax or signs of compensated shock. have physiological mottling (cutis marmor-
cardiac tamponade). Finally, dissociative atum) in the first months of life due to
shock arises when the oxygen carrying capac- Heart rate immaturity of the autonomic control of
ity of the blood is too low (as in anaemia) Tachycardia (relative to age norm) is a key skin vessels, which commonly occurs with
or has been reduced, as in carbon monoxide sign of shock (see Chapter 1.1). This tachy- exposure. One must clarify with parents
poisoning. cardia is a homeostatic response to main- whether this mottled appearance is more
taining cardiac output. Bradycardia may than usual in their child, as infants with
occur pre-terminally in the child with cutis marmoratum will become more mot-
Diagnosis and assessment overwhelming shock, and untreated will tled if they have an accompanying illness
progress to asystole. The peripheral pulses causing shock.
In all ill children the first need is to recognise may be weak, thready or absent.
the seriousness, or impending seriousness
of the condition. This requires a systematic Blood pressure Effects of circulatory inadequacy
approach to assessment that involves look- While it is true that a child with hypotension Agitation and altered conscious level (that is
ing at the airway, breathing, circulation, is shocked, it is not true to say that a child response to voice or less) are important signs
neurological status and exposing the child. that is normotensive is not shocked. This of circulatory inadequacy and the resultant
is because maintenance of blood pressure cerebral hypoperfusion. In infants, the
Airway is the key end point of the homeostatic manifestations may be more subtle and
The ability to speak or cry without stridor response, and decompensation (and thereby increased irritability or floppiness or failure
indicates a patent airway. The presence hypotension) occurs late and may be precipi- to recognise and make eye contact with par-
of adequate breathing as assessed by tous. The accurate measurement of blood ents may be the only obvious manifestation.
lookingat chest wall movement, listening pressure in a distressed child can be a Increasing respiratory rate is an early
and feeling for exhaled air also indicates challenge in itself and a judgement as to sign of inadequate circulation. Latterly, as
an adequate airway. whether a particular blood pressure is normal acidosis ensues, deep sighing respirations
Generally, shock affects the airway by or abnormal in a particular age group in any occur due to air hunger.
reducing conscious level. In acute allergic particular circumstances is also challenging. Urine output will decrease with inade-
reaction, airway obstruction (by swelling in Many widely quoted normal ranges by age quate circulation. If a child has a urine col-
the upper airway) and shock due to changes are broad furthermore many of these were lection device in place then a flow of some
in vascular tone can both arise as part of the derived in the resting outpatient child rather 2 mg kg1 hr1 in the child under one, and
same process. than in the seriously ill or injured child. 1 mg kg1 hr1 in older children indicates
adequate renal perfusion. If there is no
Breathing Capillary refill urinary collection device then this can be
The effort of breathing, efficacy of breathing The capillary refill time is measured by difficult to assess, but the weight of wet
and effects of inadequate respiration should applying pressure to the nail bed or nappies may be helpful.

42
2.5 SHOCK
2
An initial bolus of 20 mL kg1 of fluid

RESUSCITATION
Neurological status Hypovolaemia
As mentioned above, initially agitation and should be given. In most cases the initial fluid This diagnosis is usually made because of the
then subsequently depression in conscious will be crystalloid but occasionally universal presence of trauma, fluid loss from vomiting
level are associated with progressing shock. donor blood may be indicated. There is some or diarrhoea or a surgical abdomen.
As in any other case of decreased conscious controversy as to whether colloid should be The further treatment depends on the
level a glucose stick test should be per- administered in cases of sepsis, with propo- response to initial fluid bolus. If there are
formed to exclude hypoglycaemia. nents arguing that maintenance of oncotic still signs of shock then a second 20 mL kg1
pressure is important in this situation. bolus should be given. At this stage a surgical
If a glucose stick test reveals significant opinion should be sought if an underlying
Initial management hypoglycaemia then glucose should be surgical cause is suspected.
administered (5 mL kg1 of 10% dextrose). It would be most unusual for a child with
The child with shock should be managed in In such a case, hypoglycaemia may be the gastroenteritis to require more than two
the resuscitation area with monitoring of primary problem but it may also co-exist boluses of crystalloid and, if this is the case,
heart rate, blood pressure, respiratory rate, with other causes of serious illness and then an alternative diagnosis (such as an
temperature and oxygen saturation. Urine resuscitation must therefore be continued underlying intra-abdominal surgical prob-
output should also be monitored as an indica- if immediate recovery does not ensue with lem or adrenal insufficiency) should be
tor of response to therapy. The airway correction of the blood glucose. considered.
and breathing should be managed as in If a tachydysrhythmia is identified as a If the child shows no further signs of shock
any other case of the seriously ill child. cause of established shock then cardiover- after two fluid boluses and the underlying
Airway patency should be ensured and sion is indicated. This should be undertaken diagnosis is gastroenteritis, then it will
high-flow supplemental oxygen delivered. If without delay. If the child is alert or other- still be necessary to correct any underlying
ventilation is inadequate then this should wise responsive then sedation is usually dehydration and this should be done in
be supported in the first instance with a indicated. If the tachydysrhythmia is supra- the normal manner (see Chapter 7.12).
bag-valve-mask device. Consideration should ventricular tachycardia then it may be
then be given to intubation and ventilation quicker to give a single bolus of adenosine
through an endotracheal tube if there is no while preparing for cardioversion (see Septic shock
improvement. Chapter 5.9). Septic shock arises because of a complex
If no other cause of shock can be found combination of hypovolaemia (relative and
Circulation then it is reasonable to give a broad- absolute), cardiogenic shock (due to myocar-
Once airway and breathing have been man- spectrum antibiotic as part of the initial dial depression) and distributive shock. The
aged, the next priority is to gain intravascu- treatment, as sepsis is the most common underlying cause is, of course, the infection
lar access. This should be obtained rapidly. precipitant of shock in children. A third and this should be treated as a matter of
An initial assessment to see whether there generation cephalosporin should therefore urgency. As previously stated, any shocked
is a vein available to allow the placement be given as soon as the blood culture has child in whom there is not an obvious diag-
of a short, relatively large, peripheral venous been taken, to children beyond the neonatal nosis should receive broad-spectrum antibio-
catheter is made. If it is likely that such a age, where amoxicillin and gentamicin are tics as part of the initial management. If a
catheter can be placed, then up to two used as empirical sepsis cover. specific diagnosis of septicaemic shock is
attempts can be allowed. If these attempts made and antibiotics have not been given
are unsuccessful (or if there is no possibility then these broad-spectrum antibiotics (third
of placing a venous catheter) then intra- generation cephalosporins) should be given
osseous access should be gained. In most Further management immediately. Consideration should also be
cases this is done over the medial aspect given for a specific prescription of anti-
Once the initial assessment and stabilisation
of the tibia just distal to the knee (see staphylococcal antibiotics such as flucloxa-
is complete, then it is usually possible to take
Chapter 23.1). cillin and vancomycin if there is evidence
a more detailed history and undertake a
If neither peripheral venous access nor of cellulitis or a foreign body, or if the clinical
comprehensive examination to try and
intraosseous access is possible or desirable, picture is of toxic shock syndrome (high
establish the underlying condition.
then a Seldinger (guide wire) approach to fever, confusion, scarlatina-form rash with
The following specific conditions are dealt
the femoral vein is probably the next route desquamation and subcutaneous oedema).
with in more detail below:
of choice. In all cases a further bolus of 20 mL kg1
As soon as intravascular access is hypovolaemia of fluid should be given if there is not rapid
obtained then blood should be drawn to sepsis restoration of normal circulation following
be tested for haemoglobin, white cell count duct-dependent congenital heart disease the first bolus. A further fluid bolus should
and platelets together with urea, electro- heart failure be administered (by IV push) if there is not
lytes, acid base and lactate level. A blood acute severe allergic reaction a good response to the second. It is at this
culture should be taken and a glucose stick (anaphylaxis) stage that urgent consideration to rapid
test performed to exclude hypoglycaemia. neurogenic shock. sequence induction and elective intubation

43
2.5 SHOCK

should be given. Many children will develop It will usually be necessary to intubate and diagnosis is clear, then an infusion of adren-
a degree of pulmonary oedema after the ventilate these children during transfer. aline at 0.15 mcg kg1 min1 should be
third fluid bolus and oxygenation can only commenced.
be maintained by positive pressure ventila- Heart failure
tion (often with the addition of positive In babies, heart failure is usually due to Neurogenic shock
end-expiratory pressure). structural heart disease, while in older chil- A child with a spinal injury above T6 will
Cardiogenic shock is also a feature of sep- dren myocarditis and cardiomyopathy are have impaired sympathetic tone below this
sis and this will require specific treatment. the commonest underlying diagnoses. level once the initial catecholamine release
Dobutamine at a rate of 10 mcg kg1 min1 Babies present with breathlessness, feed- that occurs at the time of injury has ceased
should be commenced and adjusted accord- ing problems and failure to thrive and rest- to have an effect. The systemic vascular
ing to the response. Adrenaline (epinephrine) lessness. In older children more general resistance will fall and the reflex tachycar-
should be considered if there is no response complaints such as fatigue, anorexia, exer- dia usually seen as a response to hypovo-
to dobutamine. cise intolerance and cough are common. laemia will not occur. The overall outcome
Features of raised intracranial pressure There are signs of increased effort of is generalised vasodilatation, bradycardia
due to meningitis can occur during treat- breathing with increased respiratory rate, and loss of temperature control. Systolic
ment. This is usually presaged by a recession and positioning. There is an blood pressure will fall below 90 mmHg
decrease in conscious level together with increased heart rate with cool, pale periph- but the skin will appear paradoxically warm
abnormal posturing or focal neurology. eries together with hepatomegaly. On aus- and pink.
Early appropriate treatment should be com- cultation there may be a gallop rhythm Initial management is with fluids and a
menced and this will include consideration and basal crepitations. single bolus of 20 mL kg1 will usually
of diuresis, intubation and ventilation Oxygen should be given in all cases and achieve an acceptable blood pressure.
and appropriate positioning of the patient. broad-spectrum antibiotics administered if Atropine (20 mcg kg1) can be given if heart
In this situation, a lumbar puncture is there is any suspicion of sepsis. As discussed rate falls below 50. Very careful handling is
contraindicated. earlier, alprostadil should be given if the lesion necessary as these children may suffer from
is potentially duct dependent. In other cases postural hypotension if tipped or lifted sud-
Duct-dependent congenital diuretics (furosemide 12 mg kg1 day1 in denly. In the early stages fluid management
heart disease one to three divided doses initially) should is very important and this may require
There are a number of congenital heart be given urgently. A dobutamine infusion the insertion of arterial and pulmonary lines.
defects in which the presence of a patent may be indicated to support the failing heart Urinary catheterisation will be necessary as
ductus arteriosus is essential to the mainte- while urgent cardiological advice is sought. bladder control will be lost.
nance of pulmonary or systemic circulation. Concomitant absolute hypovolaemia due
These conditions include pulmonary atresia, Acute severe allergic reaction to trauma must always be considered, as
hypoplastic left heart syndrome, coarctation (anaphylaxis) neurogenic shock is invariably caused by
and critical aortic stenosis. The ductus A diagnosis of acute allergic reaction will trauma in children. Bleeding sources should
usually closes functionally within 24 hours usually be made because of a history of a be actively sought and managed in all
of birth but may remain patent in the previous similar reaction, or because of the children in whom hypotension persists, with
presence of cardiac abnormality. presence of typical early symptoms such as early surgical consultation.
Babies with critical pulmonary lesions will itching, facial swelling, urticarial rash,
present within a few days of birth with abdominal pain or wheeze.
tachypnoea and apparent breathlessness The initial approach to shock is appropriate
together with cyanosis, while those with administration of fluid boluses. Anaphylactic Further reading
systemic blood flow reduction will present circulatory shock will usually respond to Alderson P, Bunn F, Lefebvre C, et al. Human albumin solution
for resuscitation and volume expansion in critically ill
with failure to feed, apparent breathlessness adrenaline (epinephrine). The recommended patients. Cochrane Injuries Group Cochrane Database Syst
and collapse with poor peripheral circula- dose is 10 mcg kg1 given intramuscularly. Rev 2003;3.
Alderson P, Schierhout G, Roberts I, Bunn F. Colloids versus
tion. The immediate impression, particularly Judicious use of 1:100 000 adrenaline solu- crystalloids for fluid resuscitation in critically ill patients.
in the latter group, may well be of severe tion given intravenously in small incremental Cochrane Injuries Group Cochrane Database Syst Rev
2003;3.
sepsis and empirical antibiotics should be boluses can also be effective. Over enthusias- Bunn F, Alderson P, Hawkins V. Colloid solutions for fluid
given until sepsis is excluded. tic administration of adrenaline in the face of resuscitation. Cochrane Injuries Group Cochrane Database
Syst Rev 2003;3.
On examination, the babies are in heart mild allergic reaction or, indeed, in the face Dark P, Woodford M, Vail A, et al. Systolic hypertension and
failure (usually without a characteristic of an anxiety attack has resulted in severe the response to blunt trauma in infants and children.
Resuscitation 2002;54(3):24553.
heart murmur), often with an enlarged liver dysrhythmias. Caution should therefore be Hartman ME, Angus DC, Clermont G, Kellum JA. Crystalloid
and a gallop rhythm. exercised (see Chapter 22.5). solutions for fluid resuscitation in critically ill patients.
Cochrane Injuries Group Cochrane Database Syst Rev
Immediate treatment is to maintain or If circulatory shock is resistant to the 2003;3.
increase ductus size with an infusion of initial bolus of adrenaline then a further Kwan I, Bunn F, Roberts I. Timing and volume of
fluid administration for patients with bleeding.
alprostodil (0.05 mcg kg1 min1). Immediate intravenous bolus of 20 mL kg1 of fluid Cochrane Injuries Group Cochrane Database Syst Rev
transfer to a cardiological centre is indicated. should be given. At this stage, if the 2003;3.

44
 2

RESUSCITATION
2.6 Neonatal resuscitation
Gary Williams

expansion with an appropriate oxygen-con-

ESSENTIALS taining gas leading to establishment of a
functional residual capacity and adequate
1 Occasionally deliveries occur in the emergency department when labour cannot spontaneous ventilation is of primary impor-
be delayed to enable transfer of the patient to a labour ward setting.
tance. Second, the significance of a vital
2 The emergency department should have available appropriate equipment and sign abnormality depends greatly on the
guidelines for the resuscitation of the newborn. time since birth and the time during which
effective resuscitation measures have been
3 The unique physiological events at birth affect the resuscitative interventions in administered. For instance, bradycardia
the newborn.
immediately after birth prior to any resusci-
4 Ventilation is the main priority in the resuscitation of the newborn. tative manoeuvres likely indicates an intra-
partum stress. The same heart rate after
5 The heart rate is a reliable indicator of the newborn babys degree of compromise 1 to 2 minutes of adequate ventilation sug-
and response to resuscitation. The easiest way to monitor newborn pulses is by
gests a different range of aetiologies and
palpation of the umbilicus.
requires a different resuscitative response.
6 Chest compressions are indicated if: (1) absent pulse; or (2) heart rate <60 min1 The majority of circumstances where
despite adequate assisted ventilations for 30 seconds. newborn resuscitation is needed can be pre-
dicted, allowing opportunity for preparation
7 Compressions and ventilations should be cycled at a ratio of 3 compressions of appropriate equipment and personnel.
to 1 ventilation to achieve approximately 120 events per minute.
Factors placing the newborn at high risk
8 The preferred site of the vascular access during neonatal resuscitation is the for neonatal resuscitation include those
umbilical vein. listed in Table 2.6.1, due to maternal, fetal
and intrapartum circumstances.
9 Drugs are rarely required in the resuscitation of the newly born.
10 Volume expansion should be with crystalloid 10 mL kg1 boluses by slow push.
Aetiology and
pathophysiology
The sequence of physiological changes in
each of which may be altered and require the newborn around birth includes:
Introduction correction. Cessation of alveolar fluid production
Epidemiology There are two important caveats in this and clearance of this fluid from the
Between 5 and 10% of newborns require process. First, the achievement of lung gas-exchanging part of the airway.
some assistance to begin breathing at birth
and, in developed countries, approximately
Table 2.6.1 Risk factors for need for neonatal resuscitation
1% need intensive resuscitative measures
to restore cardiorespiratory function. It has Maternal Fetal Intrapartum
been estimated that birth asphyxia signifi- Premature or prolonged Multiple gestation Fetal distress
cantly contributes to approximately 20% rupture of membranes

of the five million neonatal deaths that Antepartum haemorrhage Preterm (<35 weeks) or Abnormal presentation
occur worldwide each year; outcome might post-term

therefore be improved for more than one Hypertension (>42 weeks) gestation Prolonged or precipitate
million newborns per year through effective labour

resuscitation at birth. Diabetes mellitus IUGR Thick staining of amniotic fluid

Neonatal resuscitation is unique in that
Substance abuse Polyhydramnios Instrumental delivery or
it is required at a time when the newborn emergency caesarean section
is undergoing a predetermined process
Maternal infection or chronic Congenital abnormalities
of transition from a liquid filled intrauterine illness
environment to spontaneous breathing of
Absence of antenatal care
room air. There is an accompanying seq-
uence of dramatic alterations in physiology, IUGR, intrauterine growth retardation.

45
2.6 NEONATAL RESUSCITATION

Spontaneous respirations and abnormalities, hypoxia secondary to

establishment of functional residual pulmonary dysfunction). Assessment at birth
capacity (FRC). At birth the infant should be collected in a
Interventions should occur in a defined
Fall in pulmonary vascular resistance
sequence recognising the primary and warm towel, dried and the umbilical cord
(PVR) (lung expansion, oxygen). clamped. Gentle stimulation may be
crucial role of adequate ventilation and be
Rise in systemic vascular resistance (SVR)
guided by frequent reassessment of other provided (rubbing the back, flicking soles
(umbilical artery constriction). of feet if required) and an assessment of
vital signs.
Reversal of flow from left to right across initial cry, respiratory effort, heart rate,
foramen ovale and ductus arteriosus. colour and tone should be made virtually
Closure of the foramen ovale and ductus
Preparation simultaneously (Table 2.6.3).
arteriosus.
Summon assistance: if it is anticipated
This normal sequence may be interrupted at
that the infant is at high risk of requiring
any point by:
advanced life support resuscitative
Ventilation
Inadequate clearance of endogenous intervention, more than one The initial assessment is an evaluation of
lung liquid (precipitate, emergency or experienced person should be mobilised. the presence and quality of respirations.
operative delivery) or excessive abnormal Communicate with colleagues to seek
airway material (blood, mucus, available antenatal and intrapartum If respirations are adequate, the heart
rate is evaluated.
meconium). data.
Inadequate respiratory effort (maternal  In the case of extreme prematurity (24 If respirations are shallow or slow, a brief
period of stimulation may be provided.
analgesics, central nervous system injury, weeks gestation) or known congenital
sepsis) or excursion (congenital thoracic anomalies, if time allows, the most If the infant has not established
adequate breathing by 30 seconds,
anomalies). senior care provider should seek to
face mask rescue breaths should be
Pulmonary disease preventing discuss with the family their beliefs and
administered and if this is not successful
achievement and maintenance of desires regarding the extent of
with breathing established by 2 minutes,
adequate FRC (parenchymal disease, resuscitation.1
prematurity, space-occupying lesion) Prepare the environment: this includes a
endotracheal intubation should be
performed.
with secondary failure to lower PVR warm, draught-free area with adequate
normally and possible intrapulmonary light, radiant heater, prewarmed There should be no delay in commencing
rescue breaths if the infant is born with,
shunting. blankets and a clock.
Compromised myocardial function Prepare required equipment
or develops evidence of, asphyxia with signs
of flaccidity, pallor and/or bradycardia
(structural or functional cardiac (Table 2.6.2).
(heart rate less than 60 min1).

Artificial ventilation
Various bag and mask systems are available
Table 2.6.2 Equipment and drugs recommended for newborn resuscitation
for neonatal resuscitation. T-piece mechani-
Equipment Drugs cal devices designed to regulate pressure,
Stethoscope Adrenaline (epinephrine) 1 in 1:10000 self-inflating bag or flow-inflating bag are
solution. all recognised as acceptable devices for ven-
Suction catheters (612 French) and suction Naloxone hydrochloride 1.0 or 0.4 mg mL1 tilating newborn infants either via a face
mask or endotracheal tube. Target inflation
8 French feeding tube and 20 mL syringe for Dextrose 5% or 10%
gastric decompression pressures, continuous positive airway pres-
sure (CPAP) and long inspiratory times are
Face masks NaHCO3 4.2% solution
achieved more consistently using T-piece
Oropharyngeal (Guedels) airways Volume expander (0.9% saline or 4% HSA) devices than when using bags but the ability
Resuscitation system for PPV to achieve an increased inspiratory pressure
when required in response to altered compli-
Laryngoscope with straight blade
ance (even for a few breaths) is greatest with
ET tubes 2.5 to 4 mm internal diameter the self-inflating bag. It is suggested in fact
ET stylets that the invariable success of rescue breath-
ing at birth is because the FRC is established
Tape for ETT and IV fixation
by an induced inspiratory effort via Heads
Cannulae, syringes and UV catheterisation paradoxical reflex (inspiratory effort induced
equipment
by any lung inflation). The corollary is that
HSA, human serum albumin; PPV, positive pressure ventilation. face mask rescue breathing is unlikely to

46
2.6 NEONATAL RESUSCITATION
2

RESUSCITATION
lower end of the normal range at
Table 2.6.3 Apgar score
5 minutes of age in a term baby.
Score 0 1 2 Pallor is suggestive of a decreased cardiac
Heart rate Absent Less than 100 Greater than 100 output and may be due to myocardial
dysfunction, severe anaemia,
Respiratory effort Absent Irregular Crying
hypovolaemia, hypothermia or acidosis.
Muscle tone Limp Some flexion Active motion

Reflex irritability Absent Grimace Grimace and cough

Colour Blue, pale Acrocyanosis Pink all over Muscle tone and reflex
irritability
These physical signs are valuable composite
be effective in the severely asphyxiated Once a slow heart rate has increased reflections of the adequacy of cerebral
infant. above 60 min1 and is rising, cardiac perfusion and oxygenation. As such, they
Regardless of these issues, it is generally compressions may be discontinued. constitute two of the five components of
accepted that higher inflation pressures the Apgar score (see Table 2.6.3) used to
(>30 cmH2O) and longer inflation times assess a newborns condition after birth.
Compression technique
(>1.5 seconds) may be required for the first
If required, compressions should be insti-
several (5) breaths. Initial peak inflating
tuted using the two-hand-encircling tech-
pressures required are variable and unpre- Medications
nique (preferred if greater than one
dictable. In general, the minimum pressure
rescuer) or two-finger technique (if only one Medications are rarely required during neo-
required to achieve an increase in the heart
rescuer or if access to the umbilicus is natal resuscitation. One study suggested
rate should be used. Visible chest wall move-
required). The lower third of the sternum (just medications were required in only 0.12%
ment and an increase in the heart rate are
below an imaginary intermammary line) of all births, for severe fetal acidosis or ven-
the best indicators of adequate ventilation.
should be depressed one third of the depth tilatory problems. This reaffirms the primary
Ventilations should be administered at a
of the chest. These should be coordinated and critical importance of achieving optimal
rate of 4060 min1 and after 30 seconds
with ventilations (to avoid simultaneous ventilation before resorting to medications
of effective ventilation, the heart rate should
delivery) in a ratio of 3:1 with about 90 com- in neonatal resuscitation.
be evaluated.
pressions and 30 breaths each minute. The
xiphoid portion of the sternum should not
Vascular access
be compressed because such compression
Heart rate Adrenaline (epinephrine) may be adminis-
may damage the neonates liver.
tered by intravenous (IV), intraosseous (IO)
Assessment of the heart rate can be done by
or endotracheal (ET) routes. If there is respi-
palpating the umbilical stump, brachial or
ratory depression once heart rate and colour
femoral pulse or auscultation of the apical
heart sounds.
Colour have been restored by adequate rescue ven-
tilation, naloxone may be given via the
Once the heart rate has been evaluated, the
The heart rate is a reliable indicator of the infants colour should be assessed by exam-
intravenous (IV) or intramuscular (IM) route.
degree of compromise and response to The other drugs and volume expansion,
ining the trunk and mucosae.
resuscitation. It should be assessed at detailed below, require emergency vascular
least every 30 seconds in the first Peripheral cyanosis (acrocyanosis) is access. Once vascular access is achieved, if
2 minutes if necessary, until the babys common in the first few minutes after the child remains in arrest, an adrenaline
required level of support is established. birth and is not pathological. dose should be immediately administered
If the heart rate is less than 60 min1 Central cyanosis reflects inadequate via the IV route.
despite adequate assisted ventilation, oxygenation and may be pulmonary or The preferred site of the vascular access
compressions are required. When the cardiac in origin. If present despite during neonatal resuscitation is the umbili-
heart rate is greater than 100 min1, only adequate ventilation and a heart rate cal vein, the larger thin walled single vessel
ventilation is continued. If the heart rate greater than 100 beats per minute, (in comparison to the paired thicker walled
is between these two points, the level of supplementary oxygen should be arteries), which appears when the umbilical
intervention should be increased or administered until the cause of the cord is trimmed 1 cm above the skin. A 3.5 or
decreased depending on the serial cyanosis is known.2 5 French catheter flushed with saline (to
change in the heart rate. Using oximetry to guide oxygen remove any air in the tubing) should be
If the heart rate is not rising after 30 administration may be valuable in these inserted only until a good blood return is
seconds of effective ventilation combined minutes to titrate oxygen and limit achieved (usually to a depth of 1 to 4 cm
with chest compressions, then adrenaline hyperoxia, especially in the premature. below the skin). Peripheral veins on extremi-
(epinephrine) should be administered. Remember that a SaO2 of 70% is at the ties or scalp may be attempted or

47
2.6 NEONATAL RESUSCITATION

alternatively an intraosseous cannula may higher dosages. For these reasons the dose should be treated by using a slow bolus of
be placed on the medial aspect of the tibia recommended is 30100 mg kg1 every 3 5 mL kg1 of 10% dextrose IV, avoiding high
just below the tibial tuberosity, if umbilical 5 minutes during arrest. blood sugar levels which have been shown
or other direct venous access is not readily to worsen CNS injury, at least in adults.
obtainable. Bicarbonate
Although correcting acidosis during cardiac Volume expansion
Adrenaline (epinephrine) arrest to improve both myocardial function If hypovolaemia is present because of known
Adrenaline is administered with the aim of and adrenalines effectiveness makes or suspected blood loss or loss of vascular
producing a-adrenergic mediated vasocon- theoretic sense, there are few supportive tone following asphyxia, volume expansion
striction, an increase in coronary perfusion experimental data. Most adult studies have may be appropriate. Isotonic non-dextrose
pressure and myocardial blood flow. Adrena- found either no difference or that bicarbon- containing crystalloid (normal saline or
line is indicated if the heart rate remains less ate had deleterious effects on myocardial Ringers lactate) 10 mL kg1 intravenously
than 60 beats per minute after a minimum performance. There are no neonatal animal over 510 minutes is recommended. Group
of 30 seconds of adequate ventilation and or human studies specifically examining this O negative packed red cells may be indicated
30 seconds of combined ventilation and question. Bicarbonate administration has for replacement of large volume blood loss.
chest compressions. The recommended IV multiple possible adverse effects (metabolic Albumin-containing solutions are used less
dose is 0.10.3 mL kg1 of a 1:10000 solu- alkalosis compromising peripheral tissue frequently because of limited availability, risk
tion (1030 mcg kg1) repeated every 3 to oxygen delivery, paradoxical intracellular of infectious disease and an observed associ-
5 minutes as indicated. ET delivery, though hypercarbic acidosis). Pertinent to neonates ation with increased mortality. A recent ran-
of unproven efficacy, can be considered are studies demonstrating large increases in domised controlled comparison of albumin
and requires a higher dose (up to plasma osmolality and reductions in cerebral versus normal saline for hypotension in pre-
100 mcg kg1); it should be followed by blood flow, both of which may increase the mature newborns showed that those who
1 mL of normal saline and several good infla- risk of IVH in newborns. Therefore, bicar- received albumin required significantly more
tions to achieve optimal delivery to the pul- bonate administration is only recommended volume expander to maintain normal blood
monary vascular bed. Most infant animal during prolonged arrests unresponsive to pressure and had a higher mean percentage
experimental dosing data supporting adre- other therapy after establishment of ade- weight gain in the first 48 hours after birth.3
nalines efficacy have been obtained in VF quate ventilation and perfusion. The dose
models and as such their value may not be is 12 mEq kg1 of 4.2% solution by slow
directly applicable to the apparent pretermi- IV push over at least 2 minutes.
Specific resuscitation
nal bradyarrhythmia in an asphyxiated new-
born with markedly elevated PCO2. Naloxone
situations
Through the early 1990s some experi- Naloxone is a narcotic antagonist, recom- Premature neonate
mental and human data showed that higher mended for the neonate with respiratory Preterm newborns have an increased likeli-
intravenous doses of adrenaline (100 depression secondary to narcotics given to hood of respiratory depression requiring
mcg kg1) were capable of achieving higher the mother within 4 hours of delivery. assisted ventilation at birth. This occurs
plasma adrenaline levels as well as greater Prompt institution of adequate ventilation because of diminished lung compliance,
myocardial and cerebral blood flow. How- is the first priority in such a situation and weak respiratory muscles and immature
ever, several subsequent adult and paedia- naloxone is not recommended for newborns respiratory drive and may make it difficult
tric studies showed no ultimate clinical whose mothers are suspected narcotic abu- to establish and maintain an adequate
benefit in survival or neurological outcome, sers as abrupt withdrawal may be precipi- FRC. For these reasons, infants born at or
with a significant risk of adverse effects tated. Following IV administration, onset of before 32 weeks gestation should receive
from the higher dose (myocardial dysfunc- action occurs in 12 minutes and is variable face mask resuscitation by 30 seconds and
tion or necrosis, hyperadrenergic states, in duration. The recommended IV dose is be intubated at 60 seconds after birth
reduced cerebral cortical blood flow). Speci- 0.1 mg kg1. Since the duration of action if the onset of adequate spontaneous
fically, there is an increase in potential risk of narcotics may exceed that of naloxone, respirations is delayed. A recent study has
of intraventricular haemorrhages (IVH) in continued surveillance and repeat adminis- shown that CPAP applied promptly to pre-
preterm infants. For these reasons, the tration are often required. Naloxone may mature babies who breathe at birth may
currently recommended initial IV dose be administered IM, with some adult data be effective in reducing need for ventilation.
remains 10 mcg kg1 in neonates. suggesting slower onset and more pro- Also, because preterm infants often have
The ET route is likely to be the most acces- longed duration of action via this route. low body fat and a high body surface area
sible route for initial doses of adrenaline. There are no studies examining the ET route to mass ratio, they are more difficult to keep
Again, there is a paucity of both experimen- of administration in the neonate. warm and therefore at increased risk of
tal and human data regarding dosage and cold stress. For this reason babies <1500 g
efficacy of ET adrenaline in neonates. There Dextrose should be covered in food-grade heat-
are data to suggest a slower onset with a Hypoglycaemia is a potential problem for resistant plastic wrapping or placed in a
more prolonged and variable effect at all stressed and asphyxiated babies and polyethylene bag with the head excluded

48
2.6 NEONATAL RESUSCITATION
2

RESUSCITATION
and a hat placed on the head. Rapid boluses 100% oxygen for several minutes (hyper- target may be set higher in the face of
of volume expander and use of hyperosmo- oxia test) will give the best indication of documented pulmonary hypertension).
lar solutions may produce large fluctuations the presence and size of a significant intra- ET tube migration or obstruction should
in blood pressure or osmolarity and there- cardiac right to left shunt (see Chapter 5.1). be vigorously avoided.
fore are not recommended.3 An urgent bedside echocardiogram, if avail- With regard to control of body
able, is indicated to delineate the cardiac temperature the primary goal is to
Meconium aspiration anatomy. Where this is not available, one achieve normothermia and avoid
Approximately one in 20 infants born should consult with the local tertiary neona- iatrogenic hyperthermia in infants who
through meconium-stained amniotic fluid tal unit. If a diagnosis of a duct-dependent require resuscitation (see hypothermia in
(MSAF) develop meconium aspiration syn- cyanotic cardiac lesion is confirmed on the Controversies section below).
drome (MAS) due to aspiration into the dis- echocardiogram, IV alprostadil (PGE1) Blood pressure should be monitored
tal airways either in utero or with the initial 2550 ng kg1 min1 restores and main- meticulously and hypotension treated
breaths following birth. However, of these, tains ductal patency until definitive deci- promptly with fluid resuscitation or
2550% require mechanical ventilation sions regarding surgical correction can be inotropes as required.
and 5% die. Although a long established made. The main side effects of this medica- Close monitoring of body weight, fluid
practice, it is no longer recommended that tion are flushing, fever and possibly apnoea balance, electrolytes, calcium and
infants delivered through MSAF undergo if respiratory support is not already in place. magnesium is indicated.
intrapartum suctioning of the oro- and naso- Alternatively, babies with duct-dependent Evidence of hypoxic insult to major
pharynx once the head is delivered, prior to systemic circulation usually due to some organs other than the brain should be
delivery of chest or shoulders. If the baby structural problem with left ventricular sought (urinalysis, serum creatinine, liver
born through MSAF is not vigorous after outflow (e.g. critical aortic stenosis, severe function tests, serum troponin level,
delivery (depressed respirations, muscle coarctation, hypoplastic left heart syn- coagulation parameters).
tone or heart rate less than 100 min1) drome) may present in the first few days Both hyper- and hypoglycaemia may
direct intratracheal suctioning (using con- of life with heart failure and poor peripheral aggravate injury following hypoxic
tinuous suction directly applied to the perfusion triggered by ductal closure. The ischaemic insult and therefore
tracheal tube as it is withdrawn) should be most reliable physical signs of heart failure normoglycaemia should be targeted.
performed. If meconium is recovered, the are tachycardia, tachypnoea and hepato- There is no evidence in neonatal post-
procedure should be repeated (if the heart megaly. If shock is present (poor pulse resuscitation cerebral care for hypertonic
rate is greater than 60 min1) before pro- volume, pallor, altered conscious state) respi- or osmotic agents to treat or minimise
ceeding with resuscitation. ratory support and fluid volume expansion cerebral oedema. The same applies to
may be required to restore the circulation. glucocorticoids and prophylactic
Congenital heart disease Subsequently, alprostadil or inotrope infu- anticonvulsant treatment. While the early
Central cyanosis at birth apparently unre- sion and continuing judicious fluid adminis- use of barbiturates in the reduction of
sponsive to 100% oxygen particularly in a tration may be required. The most important seizures, neuronal metabolic demands
vigorous baby with adequate spontaneous steps in restoring systemic circulation in and excitatory amino acid production is
respiratory effort and minimal respiratory this situation are artificial ventilation to theoretically sound, there are no solid
distress may indicate duct-dependent cya- normocapnia, together with re-establishing data on which to support their use in this
notic congenital heart disease (primarily and maintaining ductal patency (see way following a perinatal insult.
right heart obstructive lesions, transposition Chapter 5.5). Liaison with and transfer to a neonatal
of the great vessels and anomalous pulmo- intensive care unit (NICU) should be
nary venous return with complete atrial organised as soon as possible with clear
admixture). The major differential diagnoses documentation of the resuscitation
are primary pulmonary hypertension or Post-resuscitation interventions required and responses
major pulmonary structural abnormalities stabilisation achieved.
(e.g. congenital diaphragmatic hernia). In
Post-resuscitation stabilisation should be
The parents should be kept well informed
such a circumstance ventilatory support of the infants condition and efforts made
directed towards preventing any ongoing
requirement should be dictated by degree for them to have contact with the baby.
or repeated primary insults (primarily to
of respiratory distress with a target PaCO2
the brain) as well as limiting any secondary
of 3540 mmHg.
injury and organising a stable transfer to an
Detailed cardiac auscultation should be
appropriate neonatal unit. Prognosis
attempted though there may be no abnor-
mal murmurs audible; simultaneous pre- Artificial ventilation should be continued Predicting outcome at an early stage may be
and postductal oximetry measurements if required to maintain normocapnia difficult but the most reliable early predictors
should be performed. If a cyanotic cardiac (pCO2 3540 mmHg). of adverse outcome are abnormalities in
abnormality is strongly suspected, documen- Oxygenation should be optimised (aiming the clinical examination (i.e. degree of ence-
tation of preductal PaO2 after breathing for pO2 6090 mmHg, although this phalopathy) and electroencephalographic

49
2.6 NEONATAL RESUSCITATION

assessment. A sustained low-voltage EEG

Studies in human neonates are anencephaly or confirmed trisomy
or discontinuous activity on EEG within
accumulating regarding the safety of 13 or 18. Options include a trial of
6 hours of birth are strongly predictive of
induced hypothermia. At present resuscitation, non-initiation or
death or significant adverse neurologic
cerebral hypothermia can not be discontinuation after further assessment.
sequelae.
routinely recommended. However, Initiation of resuscitation does not
hyperthermia should definitely be mandate continued support.
avoided as there is strong evidence that
Controversies this is deleterious.4
International guidelines state that
discontinuation of resuscitative efforts
Air or O2 for newborn resuscitation.  Ethics of resuscitation. The decision may be appropriate if resuscitation of an
Traditionally, 100% oxygen has been
to withdraw or withhold resuscitation infant with cardiorespiratory arrest does
used for neonatal resuscitation, but
from extremely premature newborns or not result in spontaneous circulation in
recent animal studies and human trials
those with severe congenital 10 minutes.
have confirmed that ventilation with
abnormalities requires clear
room air is equally successful in neonatal
communication with the family and
resuscitation. Use of air may have
accurate clinical data. Overall there is References
advantages (reduced oxidative stress,
agreement that overly aggressive 1. Boyle RJ, Mcintosh N. Ethical considerations in neonatal
less delay in onset of spontaneous
treatment is to be discouraged.1 resuscitation: clinical and research issues. Semin Neonatol
respirations). There may still be 2001;6:2619.
situations in which 100% oxygen Recent studies demonstrate 2. Richmond S, Goldsmith JP. Air or 100% oxygen in neonatal
resuscitation? Clin Perinatol 2006;33:1128.
is advantageous (pulmonary extremely poor survival rates for 3. So KW, Fok TF, Ng PC, et al. Randomised controlled trial of
hypertension, diffusion abnormalities, infants less than 23 weeks gestation colloid or crystalloid in hypotensive preterm infants. Arch
Dis Child 1997;76:F436.
alterations in cerebral blood flow). (<1%) with a better but still poor rate 4. Hoehn T, Hansmann G, Buhrer C, et al. Therapeutic
At present best evidence suggests that for infants admitted to the NICU (5%). hypothermia in neonates. Review of current clinical data,
ILCOR recommendations and suggestions for
air should be used initially with Mortality is less at 23, 24 and 25 implementation in neonatal intensive care units.
supplementary oxygen if the infants completed weeks (11%, 26% and 44% Resuscitation 2008;78:712.
5. Wood NS, Marlow N, Costeloe K, et al. Neurologic and
condition does not improve after survival in one large recent study). developmental disability after extremely preterm birth.
effective ventilation. Data suggest that However, severe disability in childhood is N Engl J Med 2000;343(6):37884.

oxygen use in neonatal resuscitation present in 20 to 30% of survivors at

should be individualised with the dose these gestations and some disability Further reading
tailored to clinical response and can be expected in 50%.5 Prognosis is Escobedo M. Moving from experience to evidence: changes in
monitored by oximetry.2 probably better predicted by gestational US Neonatal Resuscitation Programme based on
International Committee on Resuscitation Review.
age (if accurately known) rather than
Hypothermia. Traditionally, post- weight, though both factors have a role
J Perinatol 2008;28:S3540.
International Liaison Committee on Resuscitation.
resuscitation care has involved heat-loss The International Liason Committee on Resuscitation
and gender is also important (lower (ILCOR) consensus on science with treatment
prevention or even external warming on
mortality/morbidity in females). recommendations for pediatric and neonatal
the basis that this facilitates recovery patients: neonatal resuscitation. Pediatrics 2006;117(5):
from acidosis. However, recent animal Non-initiation of resuscitation in the e97888.
Richmond S. ILCOR and Neonatal Resuscitation 2005. Arch
studies have clearly shown that early delivery room is appropriate for infants Dis Child Fetal Neonatal Ed 2007;92:F1635.
modest cerebral hypothermia (3234 C) with confirmed gestation less than 23 Wu TJ, Carlo WA. Neonatal resuscitation guidelines 2000:
framework for practice. J Matern Fetal Neonatal Med
can modulate hypoxic ischaemic injury. weeks or birth weight less than 400 g, 2002;11:410.

50
 3

SECTION
TRAUMA IN
CHILDREN
Section editor Ian Everitt

3.1 Introduction to paediatric trauma 51 3.4 Abdominal and pelvic trauma 69

3.2 Paediatric neurotrauma 56 3.5 Burns 73
3.3 Thoracic injuries in childhood 64

3.1 Introduction to paediatric trauma

Danny Cass

ESSENTIALS Prevention
1 Trauma is the prime cause of death and serious injury throughout childhood, Over the last decades, Australia has done
accounting for 60% of deaths. well in reducing the death rate from approx-
imately 11.5 deaths per hundred thousand
2 Prevention strategies have resulted in most of the improvement in mortality during to about 8.5 (World Health Organization).1
childhood. However, while the death rate has been
3 The advent of trauma teams has led to great improvement in paediatric injury care. almost halved, it is still double that of some
of the best OECD countries. Most developed
4 Delayed management of airway obstruction and inadequate fluid management are countries have significantly reduced injury
the two most common contributors to preventable paediatric deaths in trauma. death rates. Unfortunately this is not the
5 The initial assessment of the seriously injured child should follow a structured approach case in developing countries. While we have
so that life-threatening problems are rapidly identified and managed in rapid sequence. achieved much through prevention strate-
gies, there is still more to be done.
6 It is important to provide early psychological support to other family members who Prevention has involved the work of legisla-
arrive with the child with major injuries. tors (seat belts, baby capsules, cycle helmets)
7 The team leader needs to co-ordinate the resuscitation of the child with multi- through to educators and implementation
system trauma, so that patient care is at all times expeditious and tailored to the groups such as Kidsafe. Generally the com-
specific and prioritised needs of the child. munity has been supportive of the minor
inconveniences that accompany improve-
ments in child safety.
Future progress will depend on campaigns
drowning incidents contributing up to refreshing the messages, as every few years
Prevalence 25%. The third major cause is burns and there is a new generation of young parents
Overall, trauma is the number three cause the remainder includes a range of miscella- and it cannot be assumed that a good
of mortality (6%) and serious morbidity neous causes. In most series, child abuse campaign 3 to 5 years previously will suffice.
throughout life. However, trauma is the contributes less than 10% of all child There needs to be ongoing activity. We also
number one cause of death and disability deaths. need to be aware that we are promoting
between the ages of 1 and 44. Therefore This chapter concentrates on those healthy, safe activities. This does not equate
it is the prime cause of death and serious aspects of trauma management that are to safety above all other considerations. Our
injury throughout childhood rendering it different in children. Overall, paediatric children should not be sitting in front of tele-
the most important health issue in children surgeons and paediatricians have taken a visions and computer games and never going
and adolescents. In most Western societies holistic view of trauma and have been outside because that is perceived as being
road trauma contributes about half (50%) heavily involved in aspects of prevention, dangerous. Rather, through appropriate
of all serious injuries and deaths, with immediate treatment and rehabilitation. research we should further identify problem

51
3.1 INTRODUCTION TO PAEDIATRIC TRAUMA

areas that are key factors in the causation of varying local resources of the individual management are the two most common
road trauma, drowning, house fires, serious department, which vary between institutions. contributors to preventable paediatric
falls and sporting injuries. Our children Regular trauma meetings to review cases deaths in trauma. Chapters 2.2 and 2.3
should be safely riding their cycles rather or videotape evaluation of resuscitation can provide a detailed discussion of basic life
than believing that cycling is unsafe. This will provide education, with lessons learnt on support and advanced life support (ALS)
require much more work by all levels of com- how to do things better. As major paediatric techniques in children applicable in trauma.
munity as individuals, councils and govern- trauma is relatively uncommon, mock paedia- The worst response is to panic and
ments. We do need to take account of tric resuscitation scenarios can provide the freeze. In this situation a child may be left
childrens needs for activity in homes, play- emergency department staff with an oppor- un-intubated, whereas in an adult patient
grounds, skateboard areas, walkways and tunity to improve preparedness. There is intubation would be performed as a reflex
cycle paths in order to plan prevention now a good body of international literature decision. A child with a Glasgow Coma Scale
strategies. available to keep the trauma team up to date (GCS) of less than 8 should not be left with
with the optimal care of paediatric trauma face mask oxygen; in an adult there would
patients. The use of a trauma proforma sheet be rapid intubation. Similarly, intravenous
may be useful for the documentation of the lines can be difficult to insert in the shocked
Succinct treatment
assessment and resuscitation of children with paediatric patient, and children may receive
(salvage) major injuries (see Table 3.1.1). delayed fluid resuscitation if intraosseous
The advent of trauma teams and trauma sys- Clinicians often worry about managing access is not considered as an alternative
tems in hospitals that receive paediatric children with major trauma and wonder where indicated. The supportive management
patients has led to great improvements how much they should treat them as adults of the childs family is a further important
in paediatric injury care. It is estimated and how much they should take note of consideration in the emergency setting.
that there may have been a 20% decrease their differences. In adults it is well estab-
in mortality as a result of these systems. lished that the A, B, C, D, E primary survey
However, it is prevention that has resulted approach is the correct paradigm. In manag-
ing paediatric trauma clinicians sometimes
Primary survey
in most of the improvement in mortality.
Hospitals now have trauma teams ready get confused. They have a vague memory The initial assessment of the seriously
to receive the child. This may occur by that children are different. They are not sure injured child should follow a structured
forward notification by the emergency man- therefore whether to clearly follow the A, B, approach so that life-threatening problems
agement services. Trauma team activation C, D, E or a different approach. are rapidly identified and managed in the
should occur on notification when a child is The best way to remember the acute appropriate rapid sequence. This approach
at high risk of life-threatening injury accord- management of trauma in children is to is based on the prioritised principles as out-
ing to prediction by pre-determined clinical remember the a, b, c, d, e as lower case. That lined in the teaching dictums of courses
and mechanism parameters (Table 3.1.1). is, the sequence is exactly the same as in such as the Advanced Paediatric Life Sup-
The trauma team should include a team adults but there are additional nuances in port (APLS) and Advanced Trauma Life
leader, airway doctor, procedure doctor, and children to optimise their care. However, Support (ATLS) groups, which are invaluable
nursing staff from within the emergency more children suffer or die in the acute man- for practitioners who deal with paediatric
department. Activation may involve alerting agement of trauma by clinicians panicking trauma.
appropriate colleagues from radiology, anaes- and not following the A, B, C, D, E approach The approach includes initially the primary
thetics, intensive care and surgical specialties rather than doctors not being completely survey with securing of (A) airway, (B) breath-
according to local resources and protocols, familiar with these nuances (see primary ing and (C) circulation, and immediate man-
in order to expedite emergent care. The acti- survey below). Delayed management of agement of threats to life. The stabilisation
vation process needs to be adapted to the airway obstruction or inadequate fluid of the cervical spine occurs concurrently with
airway management. Following this rapid ini-
tial stabilisation, a brief neurological assess-
Table 3.1.1 Major trauma predictors at high risk of life-threatening injury ment of the child should occur (D) with
Clinical parameters Mechanism complete exposure for otherwise occult signs
Glasgow Coma Score <13 High impact trauma of injury and a prompt to consider environ-
Systolic BP <90 Fall from significant height mental issues such as hypothermia (E).
Respiratory rate <10 or >30 Crash speed >60 kph
Ejection of child from MVA In the team approach, the management
Injury Rollover MVA should occur horizontally, with simulta-
Penetrating injury to chest, abdomen, head, Death of same-car occupant
neck and groin Pedestrian/cyclist struck at >30 kph neous attention to these priorities by desig-
Significant injury to two or more body areas nated members of the team, overseen by the
Severe injury to head, neck or trunk
Two or more proximal long bone fractures team leader. The role of the primary survey
Burns of >15% or to face or airway is therefore to detect and treat abnormal
MVA, motor vehicle accident.
physiology immediately in order to pre-
Source: Adapted from Cameron P. 2004. Textbook of Adult Emergency Medicine, 2nd edn. vent potential secondary insults due to

52
3.1 INTRODUCTION TO PAEDIATRIC TRAUMA
3

TRAUMA IN CHILDREN
hypoxia or hypovolaemia. A secondary due to facial trauma compromising the air- C. Circulation and stop
survey follows with a head to toe, front way or has high risk of subsequent compro- haemorrhage
and back examination of the child. mise such as from an airway burn. It is important to be aware that a child may
The technique of intubation should be be profoundly shocked from blood loss
rapid sequence with cricoid pressure. The resulting from trauma well before the occur-
control of the cervical spine during intuba- rence of hypotension. A child responds to
Paediatric differences tion should occur by manual immobilisa- hypovolaemia with tachycardia and increas-
A. Airway and cervical spine tion by a dedicated assistant. The patient ing peripheral vascular resistance. There-
control should have continuous monitoring, pre- fore, the assessment of circulation status
In the setting of trauma,A refers to securing oxygenation and difficult airway adjuncts needs to be focused on the heart rate, pulse
of the childs airway with concurrent atten- available, if required. The choice and dosage volume and the parameters of skin perfusion
tion to the possibility of an unstable cervical of the sedation agent (thiopentone, midazo- such as capillary refill time, colour and tem-
injury. The cervical spine should be immobi- lam, propofol, or fentanyl, for example) may perature. Perfusion inadequacy should be
lised using an appropriate-sized hard collar, be individualised according to factors such as proactively corrected with volume resuscita-
sandbags, tape and a spinal board where the level of coma, cardiovascular status of the tion, rather than waiting for hypotension as
appropriate. It may be helpful in an infant child and the presence of other injuries. A rap- an indicator. Hypotension occurs late due to
to place a small towel under the space in idly and short acting muscle relaxant such as cardiac decompensation and indicates that
the shoulder region caused by the elevation suxamethonium usually provides the most a child is nearing collapse. Likewise, brady-
off the bed by the prominent occiput at this optimal intubation conditions, despite the cardia is often a prelude to imminent arrest.
age. As discussed in the ALS section in potential for transient increase in intracra- The persistence of tachycardia in the child
Chapter 2.3, the characteristics of the nial and intraocular pressures with fascicula- with trauma should prompt concern and
paediatric airway make it more vulnerable tion. Children less than 2 years of age are evaluation for occult ongoing blood loss.
to obstruction. This can be exacerbated by prone to significant bradycardia on laryngos- The child with major trauma requires
the necessary supine positioning of the child copy, which may be blunted by the admini- urgent intravenous access with the largest
on the resuscitation trolley. Due to compar- stration of atropine, prior to intubation. practical cannulae into visible or palpable
atively higher oxygen demands and less Confirmation of correct ETT placement should peripheral veins. In the shocked child,
reserve, a child manifests hypoxic decom- occur according to the methods described in cephalic, femoral or great saphenous veins
pensation earlier than adults. This reinforces Chapter 2.3. One needs to be prepared for are usually the most accessible. Where vascu-
the increased importance of airway manage- the possibility of the difficult airway or failed lar cannulation is unsuccessful, after 6090
ment in achieving adequate ventilation. intubation and have a planned algorithm to seconds, the child who requires immediate
The initial airway-opening manoeuvre in deal with this possibility. It is not the failed fluids or drugs to facilitate intubation, should
trauma patients should be by the jaw thrust intubation that causes the problem, but the have a rapid intraosseous needle placed as
technique to maintain cervical spine immo- lack of a plan of action to alternatively described in Chapter 23.11. This is clearly
bilisation. Suctioning of any oropharyngeal oxygenate the patient in this situation, whilst the second line method that should be used
soiling from blood, vomit, teeth or other for- summoning assistance. in children without hesitation, rather than
eign bodies may be necessary. Adjuncts such cutdown techniques that may be applicable
as the placement of an oropharyngeal air- B. Breathing and high-flow in adults. In certain circumstances, such as
way may be required. In children, these oxygen the small child backed over by a motor vehi-
should be placed into the oral cavity directly, All children with severe trauma should cle with obvious major abdominal, pelvic or
using gentle depression of the tongue with a receive high-flow oxygen (1015 L min1) lower limb trauma, it may be prudent to
spatula to allow for atraumatic positioning. by a reservoir face mask, independent of use additional alternative access into a vein
Nasopharyngeal airways are not recom- the need determined by saturation monitor- that drains into the SVC (e.g. external jugular
mended due to the potential presence of ing. Inadequate spontaneous ventilation or subclavian). In this rare situation, lower
cribriform plate fractures, and trauma to is supported by bag and mask assistance. If limb intraosseous infused volume may not
the turbinates may cause bleeding that this need is ongoing, intubation is required. access the circulation effectively due to dis-
may further complicate airway patency. A rapid screen for life threats, such as a ten- ruption of the normal continuity of the
The indications for intubation in children sion pneumothorax, should occur. This may intraosseous and intravascular spaces.
with major trauma are no different than require immediate decompression by needle Fluid resuscitation should initially be
those in adults. Thus, definitive airway aspiration for the child in extremis and a with crystalloid or colloid titrated to clinical
intervention should occur in the child who subsequent formal chest drain, as described response. Boluses of 20 mL kg1 should be
is apnoeic (usually coma related), has per- in the section on chest trauma (see chapter given using pressure infusion. In the hypo-
sistent airway obstruction despite the 3.3). The possibility of developing tension tensive child who arrives in extremis the
above manoeuvres, is requiring bagmask due to a pneumothorax should be borne in early use of uncross-matched O negative
ventilation to achieve oxygenation, has mind as one of the potential precipitants of blood may be indicated. Otherwise, after
respiratory insufficiency from major chest rapid deterioration following the initiation two boluses of crystalloid or equivalent have
injury, has significant ongoing bleeding of positive pressure ventilation. been infused, ongoing hypovolaemia should

53
3.1 INTRODUCTION TO PAEDIATRIC TRAUMA

be with crossmatched blood replacement. Continuous monitoring unconscious child or where the accurate
Whole blood or packed cells should be given This should include heart rate, blood pres- measurement of urine flow is required.
in boluses of 20 and 10 mL kg1 respectively. sure, respiratory rate, oxygen saturation  Investigations appropriate bloods
Fluids should be warmed in order to and clinical assessment of perfusion. should be sent on initial intravenous
reduce the incidence of hypothermic stress access, along with a bedside glucose
complicating the resuscitation. The child measurement. In the multiply injured
Support of family members
with ongoing cardiovascular instability child, initial radiological films should
It is important to provide early psychological
needs urgent surgical intervention. potentially include a resuscitation room
support to other family members who arrive
Part of the circulation phase of manage- chest X-ray, lateral cervical spine and
with the child with major injuries. This
ment includes therapy to limit ongoing pelvis. A 12-lead ECG should be done
should occur via a senior member of the
blood losses from external or fracture sites where a child has sustained significant
team assisted by a nurse or social worker
by direct pressure or splinting. This is of vital trauma to the chest.
who is dedicated to this role. Non-comatose
importance as a childs blood volume is
children are often fearful and distressed and
only 80 mL kg1 and small ongoing losses
it is useful to have the parents available to
contribute to haemodynamic instability. Definitive care and disposition
provide comfort to them.
Although in major trauma the most likely After the secondary survey and reassessment
cause of shock is blood loss, one needs to of the childs physiological response to resus-
consider other possible contributors such citation, the next step is to prioritise injuries
as myocardial injury, pericardial tamponade, Secondary survey to determine the need and timing of further
spinal shock or tension pneumothorax. imaging or any surgical intervention. The
The secondary survey follows when initial
general rule is if the child remains hypotensive
resuscitation has stabilised the child from
despite a 1/2 blood volume resuscitation
D. Disability immediate life threats. Continuous monitor-
over the first 1 to 4 hours, the child needs sur-
The most rapid way to assess a childs ing of vital signs and neurological status is
gery. Persistent fluid and blood resuscitation
conscious level in the primary survey is by paramount, as any deterioration should
can quickly cause a coagulopathic state.
using the AVPU scale (see Table 3.2.2). prompt immediate discontinuing of this phase
It may be necessary to involve multiple surgi-
A child who scores a non-purposeful P or U of the assessment and return to the primary
cal specialists depending on the childs injuries
correlates with a GCS of 8 or less and survey. The secondary survey should include:
and the approach varies according to local
requires intubation and ventilation. The
brief neurological assessment during the pri-
History from ambulance staff, parents resources. The team leader needs to co-
and child where possible. ordinate these inputs so that patient care is
mary survey is completed by checking equal-
A: Allergies at all times expeditious and tailored to the
ity of pupil size and reactivity to light.
M: Medications specific and prioritised needs of the child. In
P: Past history the non-paediatric tertiary environment, early
E. Exposure and environment L: Last ate liaison with appropriate colleagues from a
The child should be undressed fully to allow E: Event in order to clarify mechanism trauma centre with the capacity for definitive
examination of the entire body in order to of trauma and potential for injury. The care of a child with major injuries, will help
detect all external stigmata of injury. Once more detail in the history the more determine the most appropriate means for
this is completed, the child should be accurate the assessment of potential the individual child optimally to reach
covered by warm blankets. The potential injuries. definitive care. Transfer of a child to definitive
for hypothermia can also be decreased by Head to toe, front and back care is discussed in Section 27.
the use of radiant heaters and warm resusci- examination the child should have a Care in paediatric hospitals can result in
tation fluids. methodical and thorough clinical lower rates of non-therapeutic laparotomies.
examination to identify all injuries. The The skill of the paediatric surgical trauma
head, face, neck, chest, abdomen, pelvis, team is often in deciding when not to oper-
spine and extremities (see orthopaedic ate. Repeated examinations identify the
Other issues during initial
trauma below) need to be examined. child who is continuing to bleed and high-
stabilisation A more formal neurological assessment light the child who may have a hollow viscus
Early analgesia should use a paediatric GCS. The spinal injury. Patterns of injury, such as with seat-
Children who have significant pain or dis- immobilisation needs to be maintained belt bruises, are often recognised and are
tress need to have prompt provision of until clinical or radiological clearance, in able to be put in the context of the childs
analgesia as soon as intravenous access is cases where this is possible. During this overall physiology. The optimal care of an
obtained. This is usually best achieved by phase, placement of an orogastric tube injured child is in a paediatric institution
titrating intravenous morphine in doses may occur if gastric decompression is where there is familiarity with children and
of 0.10.2 mg kg1 to effect. Adequate indicated. A urinary catheter (with the a sufficient volume of work to maintain that.
analgesia allows a more rapid and reliable standard precautions for a potential The care of the child throughout the
clinical assessment. urethral injury) is indicated in the hospital stay should include other important

54
3.1 INTRODUCTION TO PAEDIATRIC TRAUMA
3

TRAUMA IN CHILDREN
considerations such as early physiotherapy point to the area of maximal tenderness, experience, so that multiple attempts and
to stop limb contractures, early involvement whereas in a younger child the parent further injury are avoided. Complicated frac-
of the rehabilitation team and schooling. may be able to indicate which areas tures and those in which the reduction will be
cause the child to be apprehensive. hard to maintain require additional expertise.
The function and range of motion of Appropriate orthopaedic follow up of
surrounding joints should always be checked. even minor fractures in children is vital.
Orthopaedic trauma
Vascular complications can be assessed Within 57 days the swelling subsides, and
A discussion of specific injuries and their man- by colour, perfusion and pulses of the the back slab does not hold the fracture,
agement is dealt with in detail in Section 24. distal limb. As pain often limits motor which may lose position. At that stage
Bony injuries are common in children who function, neurological assessment to test a circumferential plaster can usually be used.
have multiple trauma. Once the child is sta- sensory components is important. Because the child frequently falls, a change in
ble, the secondary survey must include Compartment syndrome should be looked fracture position can easily occur. A repeat
assessment of all limbs, clavicles, as well as for when a displaced fracture has X-ray at review is necessary to determine
ribs, pelvis and spine. The splinting of long- significant associated swelling. In if the fracture has maintained position.
bone fractures before moving the patient children the volar surface of the forearm
from the resuscitation room is important, is frequently the site involved. Pain on
even in the face of other serious injuries. This passive stretch of involved muscles is the
avoids ongoing pain and blood loss as well as hallmark. Distal pulses may be preserved.
Rehabilitation
further soft tissue injury to the limb. Neurological dysfunction often develops. Rehabilitation should start from the time of
A careful tertiary survey the following day admission and there should be early invol-
Pain relief involves first immobilising the limb
may reveal minor bony injuries, which were vement of the appropriate teams. There have
with a sling, a temporary backslab or seating
not detected during the initial resuscitation been major improvements in the diagnosis
the child in a wheelchair or bed. The need for
phase. These injuries need to be immobilised and care of cervical spine injuries and manage-
medication for pain relief can then be
and, if necessary, a plan made for reduction ment of severe head injuries. There needs to
assessed. Analgesia is required before radio-
as soon as the general condition of the child be continuing work on the outcome of minor
logical investigation, so that any limb move-
allows. Once the life-threatening injuries head injuries and the debilitating long-term
ment necessary to take the appropriate
heal, a missed displaced bony injury is a major consequences of serious long bone fractures.
films is less distressing for the child.
problem for the child in the longer term. Finally, the childs social situation and pro-
Plaster immobilisation in the Emergency
Isolated orthopaedic trauma is one of the pensity to partake in risky behaviour needs
Department should generally involve using
most common types of trauma presenting to to be assessed, as there is some evidence
a backslab technique, rather than a circum-
an Emergency Department. Review of history that children with injuries tend to have fur-
ferential plaster because of the potential for
and mechanism of injury and an overall ther future injuries. Careful assessments of
early limb swelling. The slab should be of
examination are needed to exclude concur- the childs social history may allow appropri-
sufficient strength to keep the fracture sta-
rent injury. The possibility of a non-accidental ate interventions. A full tertiary history
ble. Plaster of Paris strips usually require
injury must always be considered. The time of should include environmental factors (type
12 layers for the upper limb and 20 layers
last oral intake should be documented and of car / airbags / seatbelts / helmets /
for the lower limb. For displaced fractures
the child should receive nothing orally until details of window falls / pool fence gates,
an initial temporary plaster slab can reduce
the management plan is established. etc.) so as to allow future prevention pro-
movement and therefore pain at the frac-
In order to avoid causing further pain to grams to be appropriately targeted.
ture site during the X-ray. The soft padding
a child, much of the examination of the
can be used to line the plaster slab, rather
fracture area can be done by observation.
than winding it around the limb to reduce
Lack of spontaneous movement gives a movement on application.
Acknowledgement
clue to the location of a painful injury. The reduction of any displaced fractures
Deformity or angulation, swelling and should be done with appropriate analgesia, The contribution of Mary McAskill as author
bruising, or breach of the overlying skin anaesthesia and sedation, which should be in the first edition is hereby acknowledged.
suggests an open fracture. administered by a second doctor. Often this
Gently eliciting localised areas of is best achieved with a general anaesthetic
tenderness helps direct the most efficient where there is significant need for manipu-
use of radiology to identify the site of lation or fracture instability. The reduction of Reference
1. A League Table of Child Deaths by Injury in Rich Nations.
injury. It is best to exclude non-tender any fracture in children should be done by a Innocenti Report Card Issue No. 2, United Nations
regions first. A co-operative child can doctor with the appropriate training and Childrens Fund; February 2001.

55
 3.2 Paediatric neurotrauma
David G. Cooksley

averaging 4:1, but peaking at 9:1 in the

ESSENTIALS 1524-year age group.5
Preventive strategies have made the most
1 Neurotrauma is the leading cause of morbidity and mortality in paediatric trauma. impact on the improved outcomes of pae-
2 The prevention of secondary brain injury is the primary focus of acute medical diatric trauma over the past two decades.
intervention in the emergency department.

3 A careful history and examination is important to assess the potential significance

of the injury and determine the need for further investigation or admission for Pathophysiology
observation.
TBI, like spinal injury, can be divided into
4 Clinicians should not attribute the cause of shock to head injury until other causes primary and secondary trauma. Primary
have been excluded. Infants may become hypovolaemic from the blood loss of an trauma occurs during the initial impact to
intracranial bleed. . the head and only preventive measures such
as using protective equipment (e.g. helmets
5 Any child with a GCS
14, clinical evidence of a skull fracture or penetrating skull and seatbelts), better engineering (e.g. safer
injury should have an emergent cerebral CT scan.
roads), education and legislation can alter
6 The prime role of trauma resuscitation is to rapidly identify and correct hypoxia the extent of this primary injury. Secondary
and hypotension in children with severe TBI, as they are significant contributors to trauma or insult occurs when post-traumatic
secondary brain injury. acute phase response and mediators, or
subsequent physiological insults, such as
7 Rapid sequence oral intubation, with in-line stabilisation of the cervical spine, is hypoxia, hypotension and increased intra-
the preferred technique for securing airway control in the head-injured child.
cranial pressure, occur and cause further
8 Children sent home from the emergency department following a head injury must damage to the already traumatised tissues
be discharged to the care of a responsible adult who is given clear discharge or structures. The prevention of secondary
instructions and a written head-injury sheet containing advice on when to seek review injury, particularly due to cerebral hypoxia
and reduced cerebral perfusion, is the pri-
9 The family of the child with a significant head injury requires appropriate support mary focus of acute medical intervention,
and explanation as an important facet of care whilst in the emergency department.
which begins in the field via ambulance
and continues in the ED setting.
Children have unique anatomical, physio-
aged 1014 years.2 These figures are higher logical and developmental differences when
Introduction than the overall, age standardised, annual compared to adults. They have a large head
Paediatric neurotrauma is a common pre- rate of between 140 and 150 per 100 000 to body ratio, leading to a high centre of
senting problem in emergency medicine population.2,3 It is important to remember gravity (falls) and to the head being the pri-
practice. This chapter will deal principally that these figures only reflect hospital mary target for trauma. The skull is thinner
with paediatric head injury or, more cor- admissions and the true incidence of TBI and more plastic and thus transmits rather
rectly, traumatic brain injury (TBI), and in the community is much higher.4 than attenuates impact.6,7 Skull fractures are
spinal cord injury. Spinal injury is also In Australia the most common cause of therefore more common in children and
covered in Chapter 24.3. TBI is falls, followed by motor vehicle related importantly, serious brain injury can occur
accidents.2 A much smaller percentage without an associated skull fracture.812
result from being struck by objects, crushed, In children, the dura is more closely adher-
assaulted (non-accidental injury) and other ent to the skull compared to adults, making
Epidemiology miscellaneous causes. Males consistently extradural haematomas less common in chil-
TBI covers a spectrum of injury from trivial to outnumber females, with most studies dren, particularly in infants.7,13 Unfused
lethal. It is the leading cause of morbidity reporting approximately a 2:1 ratio for all sutures and an open fontanelle can expand
and mortality in paediatric trauma.1 The age groups. The overall incidence of spinal to accommodate intracranial haemorrhage
number of children admitted to hospital in cord injury in Australia for children aged or cerebral oedema.7 Some authors have
a recent study indicated an annual rate in 0 to 14 years is unknown.4,5 The age stan- thought children to be more prone to malig-
Australia of 232 per 100 000 for those aged dardised incidence in the 15-year-old nant cerebral swelling that can cause rapid
04 years, 158 per 100 000 for those aged population is 14 per million of population and sometimes fatal deterioration even
59 years and 203 per 100 000 for those per year, with a male to female ratio after minor TBI.9,14,15 However, this view

56
3.2 PAEDIATRIC NEUROTRAUMA
3
of 14 or 15.14 Some authors believe that

TRAUMA IN CHILDREN
has recently been questioned and swelling response and motor response (see exami-
may be no more common in children than nation and Table 3.2.1).19 It has proved even this is too liberal and the definition
in adults.16 to be a very useful tool in rating severity of mild TBI should be restricted to patients
Physiologically, children have a lower of TBI and prognosis.7 with a GCS of 15.22,23
systolic and mean arterial blood pressure, The problem with using the GCS on young Approximately 80% of children with TBI
which implies a lower cerebral perfusion children, particularly those aged less than will fall into this category.24 The reported
pressure (CPP). This, in turn, may cause pro- 2 years, is that the best verbal response incidence of intracranial haemorrhage
blems with maintaining adequate cerebral is limited by their language development. (ICH) varies between 4 and 7% in children
perfusion if they have raised intracranial In an attempt to overcome this diffi- with GCS 15, and increases to approximately
pressure. Infants and small children may culty, modified GCSs have been proposed, 10% in children with GCS 14. 10,15,25,26 The
become hypovolaemic with large intracra- including the so-called Child Coma Scale overall mortality in this group is reported to
nial bleeds. This is not seen in larger children (CCS).9,20 It is important to note that, unlike be as high as 2%.15 These figures may be
or adults.6 the GCS, the CCS has never been properly subject to significant selection bias.
Cerebral blood flow is often very low and validated and many studies of head injury The terms minimal or trivial TBI are
may approach ischaemic levels following in children deliberately exclude those aged sometimes used to describe a subgroup of
more severe TBI.17 This may be related to a less than 2 years. mild TBI who meet the following criteria:
low brain metabolic rate in comatose patients, TBI is usually divided into three cate- GCS 15, normal neurological examination
increased intracerebral pressure and vaso- gories: mild, moderate and severe. and no signs of a skull fracture.23,25,27
spasm. Autoregulation of cerebral blood Transient LOC or amnesia does not exclude
flow (CBF) may be lost following TBI, and in patients from this subgroup.
Mild TBI (GCS 14 to 15)
this setting CPP largely determines CBF. This
Mild TBI, sometimes termed minor TBI, was
underscores the importance of maintaining
originally defined as head trauma patients
an adequate CPP in the head-injured patient, Moderate TBI (GCS 9 to 13)
with a GCS from 13 to 15 (and/or varying
especially those with more severe injuries. Approximately 18% of children with TBI
periods of loss of consciousness (LOC) and
Head injury, as opposed to TBI, may be fall into this category.24 The incidence of
amnesia).21 The problem with this definition
described as extra-axial or intra-axial. ICH and overall mortality in this group
is that patients with GCS 13 have a signifi-
Extra-axial injury refers to pathology out- of children is uncertain (almost all research
cantly higher risk of intracranial injury, with
side the brain parenchyma.7,18 Extra-axial focuses on either mild or severe TBI).
subsequent risks for deterioration and neu-
structures include the skull, structures
rosurgery, than patients with a GCS of 14
between the skull and brain and the ventric-
or 15. They more properly belong in the
ular spaces within the brain. Common extra-
moderate head injury group.15,22 Severe TBI (GCS 8 or less)
axial lesions include skull fractures and
The original definition of GCS 13 to 15 Approximately 2% of children with TBI fall
extradural, subdural, subarachnoid and
continues to be used in international and into this category.24 Patients in this category
intraventricular haemorrhages. Extra-dural
Australasian literature but the recognised are, by definition, comatose. Overall mortal-
haemorrhage occurs, as its name implies,
definition of mild TBI in Australasia is GCS ity in this group is between 30 and 40%.28
outside the dura. Medical literature often
refers to this as epidural haemorrhage but
in Australasia the term epidural is generally Table 3.2.1 Adult and child Glasgow Coma Scores (GCS)
used only to describe lesions outside the
Score Adult Child
dura of the spinal cord. Intra-axial injuries
are true TBIs and include contusion, lacera- Eye opening
tion, haemorrhage and diffuse axonal injury 4 Spontaneous
3 To speech only Same
(DAI). DAI may result in considerable 2 To pain only
disability with little to see on radiological 1 No response
investigation.7
Verbal
5 Orientated in person, place and time Happy/smiles/interacts
normally
4 Confused Crying but consolable
Classification 3
2
Inappropriate but intelligible speech
Incomprehensible sounds
Inconsistently consolable
Inconsolable and/or irritable
1 No response No response
The generally accepted method of classify-
ing severity of TBI is by using the Glasgow Motor
Coma Scale (GCS), although other measures 6 Obeys commands
5 Localises painful stimulus
such as duration of unconsciousness or 4 Withdraws to painful stimulus Same
amnesia are sometimes used. The GCS was 3 Flexor posturing to painful stimulus
2 Extensor posturing to painful stimulus
first described for adults in 1974 and scores 1 No response
three variables: eye opening, verbal

57
3.2 PAEDIATRIC NEUROTRAUMA

mechanism is not in keeping with degree neurological observations can be decreased.

ASSESSMENT of injury observed.12 It is important to note that the GCS is
Past history that is particularly relevant intended to score global function not focal
in the context of neurotrauma is: deficit. When using the GCS to classify the
History severity of TBI, the best post-resuscitation
previous head or spinal injury; score should be used.
Careful history and examination is impor- congenital central nervous system (CNS)
tant when assessing the significance of the If a painful stimulus is needed to test
problems;
injury and determining the need for further motor function, apply pressure to the
CNS surgery (such as the insertion of a VP
supra-orbital margin to test for localisation
investigation or admission. shunt);
Initial assessment and treatment may of pain. This is superior to a sternal rub.
bleeding diatheses such as haemophilia
To test for withdrawal or abnormal flexion/
need to occur simultaneously. Ensuring an or thrombocytopenia;
adequate airway, breathing and circulation extension, use a pen or pencil to apply pres-
psychomotor or developmental problems,
sure to finger-nail or toe-nail beds. Abnormal
with resuscitation as required is the first pri- such as autism, that make assessment
ority. The best approach is to undertake a flexion is usually termed decorticate post-
particularly difficult.
primary survey with correction of life uring and abnormal extension termed
threats, followed by a detailed history plus It is important to remember: decerebrate posturing although this was
secondary survey as for any other trauma discouraged in the original description of
medications, and other ingestants that the GCS as it implied a specific physio-
patient. Spinal precautions using immobili- may influence assessment of a head
sation must be maintained until clinical anatomic correlation.19 The best score should
injury including possible illicit drugs or
and/or radiological clearance of the spine be recorded after testing all four limbs and
medications and chemicals to which a
has been completed. any discrepancy between limbs should be
young child may have gained access at
Assessment of the child may be extremely recorded separately.5
home;
difficult, if not impossible, if the child is dis- It should also be noted that it may not be
allergies;
possible to score verbal or motor function in
tressed with pain or is cerebrally irritated. immunisation status;
The early use of a very small dose of paren- some patients, for example those who are
last food or fluid intake.
intubated (verbal score) or have a high-level
teral narcotic such as morphine 0.05 mg kg1
intravenously may make assessment and spinal cord injury or limb injuries (motor
management much easier. Concern about score).
Examination Table 3.2.1 allows comparison of the
masking changes in neurological function
should not prevent the use of adequate Check vital signs, including bedside blood GCS and the CCS used by Hahn et al and
analgesia for children who are distressed sugar level, to immediately exclude: others.9,29 The CCS has never been properly
by pain. validated and assessment of verbal res-
hypoxia (SaO2
90% or PaO2 ponse is somewhat subjective, particularly
The following historical information is

60 mmHg) in children aged less than 6 months.20
relevant and should be ascertained if
hypotension (SBP
5th percentile for age A rapid assessment of a childs neurologi-
possible:
or SBP <90 mmHg) cal disturbance can also be made using the
time and mechanism of the injury; hypoglycaemia (blood sugar level AVPU scale, which denotes the childs
any loss of consciousness or period of <3.0 mmol L1). response to stimuli (Table 3.2.2). The
altered level of consciousness and its child who demonstrates a non-purposeful
These reversible factors may be contributing
duration; response to pain (withdrawal, flexor or
to the childs altered level of consciousness
any post-trauma seizure-type activity;
and also cause secondary brain injury. Ascer- extensor responses) has a level of conscious-
any post-injury vomiting;
tain the childs weight, early, either by mea- ness consistent with a GCS of <9 and the
any headache or other neurological
surement or inference from the childs age, unresponsive child, a GCS of 3.
symptoms such as diplopia, weakness Assess the child carefully for signs of
as it is necessary for calculating drug and
or altered sensation; trauma to the head, neck and thoracolum-
fluid requirements.
any retro- or anterograde amnesia; bar spine. A skull vault fracture may be
progression of any symptoms and signs
from the time of injury;
details of pre-hospital care assessment Glasgow Coma Scale (GCS)
Table 3.2.2 AVPU scale
and therapies. This should be used to define the childs level
A Alert
The possibility of non-accidental injury (NAI) of consciousness and to monitor any change
must always be considered in children with over time. Serial neurological observations V Responds to Voice
skull fractures or intracranial injuries. Be par- should be performed regularly to monitor P Responds to Pain Purposeful
ticularly vigilant with children aged for deterioration or improvement in the Non-purposeful
<2 years, when a parent or carer has childs conscious state. Once a childs GCS U Unresponsive
delayed seeking medical care or the stated is normal and stable, the frequency of

58
3.2 PAEDIATRIC NEUROTRAUMA
3

TRAUMA IN CHILDREN
suggested by scalp haematoma, crepitus or Laboratory without sedation or general anaesthetic and
palpable depression. A basilar skull fracture In patients with mild TBI, laboratory investi- in those in whom possible NAI is sus-
should be suspected in the presence of gations are not indicated unless there is pected.34,35 Skull X-rays may also be useful
racoon eyes, Battles sign (bruising around known or suspected coexistent pathology in screening for depressed skull fractures
the mastoid process), haemotympanum or such as haemophilia or thrombocytopenia. or penetrating skull injury, particularly in
CSF rhinorrhoea/otorrhoea. Any sign of In patients with moderate or severe TBI environments where CTscanning is not read-
trauma above the clavicles increases the the following should be checked: ily available.
likelihood of intracranial pathology being
detected on computerised tomography
Haemoglobin level and platelet count. Cervical and thoracolumbar
(CT) scanning.30
Coagulation profile including fibrin spine X-rays
degradation products (FDPs). Tissue
A careful examination of the cranial These should be obtained for any child with
thromboplastin released from the brain
nerves and peripheral nervous system suspected cervical or thoracolumbar trauma,
can cause coagulopathy and
should follow to assess for any localising or evidence of spinal cord injury, and those in
disseminated intravascular coagulation,
signs. If spinal cord injury is suspected the whom the spine cannot be cleared clinically.
the presence of which denotes a poor
sensory level of injury (dermatome) should Young children (age <10 years) are more
prognosis.31,32
be defined, not forgetting to check for intact prone to high cervical spine injuries whereas
perianal sensation and normal anal tone.
Electrolytes these serve as baseline
older children, like adults, are more prone
during fluid resuscitation and subsequent
Hypotensive bradycardia, altered perspira- to lower cervical spine injuries.36 Spinal
monitoring for hyponatraemia. Transient
tion below the level of the lesion, priapism cord injury without radiological abnormality
hypokalaemia is a well-recognised
in boys and urinary retention all signify (SCIWORA) was thought to be principally a
consequence of TBI and may occur even
autonomic dysfunction, which may occur paediatric phenomenon but is in fact seen
in mild cases. It usually resolves
with spinal injury. Any sensory or motor much more often in adults.37
spontaneously after several hours and no
function below the level of injury implies A low threshold should be held for CT scan-
treatment is required.
that the cord lesion is incomplete and ning the vertebral column in any child with
denotes a better prognosis.
Arterial blood gases should be
high clinical suspicion of spinal injury or pos-
considered, depending upon clinical
Pupil size, equality and reactivity, particu- sible radiological abnormality on plain film.
situation. In ventilated patients arterial
larly in the unconscious child, should be
blood gases (ABGs) should be used to
checked and recorded. A dilated pupil is CT scan
confirm that the end tidal CO2 (ETCO2)
defined as >4 mm diameter, asymmetry as In cases of TBI, CT scan is the modality of
accurately reflects PaCO2.
>1 mm difference between pupil size and choice for imaging the skull for fractures
non-reactivity as <1 mm movement when and brain for acute haemorrhage, oedema,
a bright light is used to test the reflex. Radiological mass effect, pneumocephalus and hydro-
The pupillary size and reaction may be also Skull X-rays cephalus.7,38 It is rapid, inexpensive and
altered by direct trauma to the globe, so Before the widespread availability of CT can accommodate a wide range of life sup-
one needs to consider traumatic mydriasis scanning, skull X-rays were often used to port and monitoring equipment. Any child
when the overall features are not in keeping screen or help risk stratify which children with a persistent GCS
14, clinical evidence
with an intracranial cause of unilateral should be observed in hospital or have a of a skull fracture or penetrating skull injury
pupillary dilatation. Whenever possible, neu- brain CT scan.8,29 While it is acknowledged should have an emergent cerebral CT scan
rological examination should include an that children with a skull X-ray demonstrat- (Table 3.2.3; see also mild TBI). The child
assessment of gait. ing a fracture have a higher incidence of ICH needs to be accompanied by appropriate
compared to those without a fracture, many
children without fractures also have an
Investigations ICH.911,15,29 To compound this problem,
the reported sensitivity and specificity of Table 3.2.3 Indications for CT scan
Bedside skull X-rays in detecting a fracture is GCS <15
Blood sugar level reported to be as low as 21% and 53% Focal neurological deficit
This is better thought of as a vital sign than a Signs of skull fracture or penetrating skull
respectively.33 Emergency department staff injury
bedside investigation and should be measured need to be familiar with the interpretation
at the same time as HR, BP, RR and SaO2. STRONGLY CONSIDER IF . . .
of paediatric skull X-rays in order to mini- Persistent vomiting
mise the misinterpretation of sutures or vas- History of LOC*
Post-traumatic seizure (delayed)
12-lead ECG cular markings as fractures and vice versa. Post-traumatic amnesia
This should be performed in the child who The role of plain films is very limited in Moderate to severe headache
Underlying bleeding risk
has associated chest trauma and considered seriously injured patients but may still have
in any patient with moderate or severe head a role in helping to risk stratify very young *The duration LOC does not correlate well with the risk
of intracranial pathology and anything greater than
injury, as catecholamine release from TBI children (age <2) with head trauma, who transient LOC (i.e. >1 min) should be considered
can cause myocardial effects. can be difficult to keep still for CT scanning significant.12,23

59
3.2 PAEDIATRIC NEUROTRAUMA

staff and monitoring equipment to the CT sedation.12 In addition to the radiation risks, The risk of developing clinically signifi-
scan environment to continue optimal mon- the cost of scanning all children with head cant IC pathology following the initial
itoring and rapidly manage any potential trauma would be considerable. trauma decreases over time. Although cur-
complications. The observation has been made that the rent Australasian guidelines for children
The CT is also used as the initial investiga- greatest benefit in treating patients with suffering mild TBI suggest discharge after
tion of choice to further evaluate suspected TBI is not with aggressive management 4 hours of observation if the child has a
spinal injury although it cannot exclude of the severely injured, but in preventing GCS of 15 and is asymptomatic,14 a large
ligamentous injury and it provides limited deterioration and complications in those study of over 28 000 children admitted to
information on injury to the spinal cord with mild or moderate injuries who appear hospital following head injury demonstrated
itself.39,40 to be at low risk.12 that 6 hours of post-injury observation was
Risk factors for IC injury or deterioration, required to identify all children who were
Magnetic resonance imaging (MRI) such as LOC, amnesia, headache, vomiting, likely to deteriorate.48
MRI is superior to CT scanning for detecting seizures and focal neurological deficit have It has been estimated that a child with
cerebral oedema, contusion and diffuse axo- been studied in an attempt to identify those trivial head injury, who has no LOC and
nal injury in cases of TBI.7 It is also superior children with mild TBI who do not require a who is completely well (i.e. none of the
to CT scanning for visualising the posterior CT scan.11,15,18,26,30,35,42,4447 The presence above mentioned symptoms or signs) has
fossa and brainstem regions. It is the inves- of any of these risk factors increases the a less than 1:5000 chance of significant IC
tigation of choice for spinal cord injury and likelihood of intracranial pathology and it pathology and can be discharged to the
the actual patterns of haemorrhage and is important to note that a child can have care of a responsible adult without further
oedema within the cord carry prognostic sig- all of them and still have a GCS of 15. investigation.23
nificance.39,41 MRI imaging of ligamentous Prospective studies evaluating such clinical Based upon current knowledge, the man-
tissues can be used to investigate possible decision rules for imaging children with agement and disposition algorithm shown in
spine instability. However, clinical correla- TBI are still to be completed. Fig. 3.2.1 is suggested.
tion with MRI findings in mild cases is still
lacking.40
Any of the following?
GCS < 15? No Focal neurological deficit
Signs of skull fracture or
Management penetrating skull injury

Mild TBI (GCS 14, 15)

The management of mild TBI is controver- Yes
Yes No
sial. The debate primarily focuses on the
question of whether or not children in this
group can be risk stratified and managed CT HEAD Any of the following?
clinically without further investigation, or History of LOC > 1/min
whether they need to have a cerebral CT Strongly Post-traumatic seizure
consider Yes Post-traumatic amnesia
scan regardless of clinical findings. Concern Modsevere headache
stems from the observation that even Vomiting
Normal ? Underlying bleeding risk
children with a GCS of 15 and a normal neu-
rological examination can harbour clinically
No
significant intracranial pathology with Yes Neurosurgical
attendant risks for subsequent deterioration consult
and death. One study of 429 children with
mild TBI found that 16% of the children
GCS improves to 15 and child is No
with GCS 15 and no LOC had significant IC completely well apart from mild
injury.42 Of these, 1.4% required neurosurgi- headache
cal intervention and 2% died.
On the other hand, CT scanning involves
No Yes
a significant radiation dose and concern
has been expressed about the subsequent
lifetime risk of radiation-induced malignan-
Observe for 6 hours Discharge to care of
cies which may be as high as 1 per 1500 scans responsible adult. Must have
If still not improved after this time
in very young children.43 Also, if very young, (drowsy, headache, vomiting) admit written head injury advice
the child may require sedation or a general to hospital for further observation sheet
anaesthetic, with their attendant risks of
apnoea, hypoxia, aspiration and prolonged Fig. 3.2.1 Suggested management algorithm for mild TBI.

60
3.2 PAEDIATRIC NEUROTRAUMA
3
total of 40 mL kg1 volume resuscitation,

TRAUMA IN CHILDREN
Moderate and Severe TBI normal range (i.e. aiming for an ETCO2 of
These groups will be considered together. 35 mmHg). Hypercarbia secondary to inade- consideration should be given to the use
It should be noted that the best GCS quate ventilation may result in cerebral of vasopressor support to maintain ade-
after resuscitation is used for classification vasodilation and secondary increase in ICP. quate MAP for cerebral perfusion. In the
of the TBI, and that a child with a GCS Hypocarbia causes cerebral vasoconstriction setting of hypovolaemia due to acute blood

8 is, by definition, comatose. The priorities and therefore decreases ICP, but increases loss, ongoing resuscitation fluid will require
of management include maintenance of the risk of causing or exacerbating cerebral blood product replacement.
the airway, oxygenation and cerebral hypoperfusion with secondary ischaemia.53
perfusion. Routine prophylactic hyperventilation in Hypertonic saline
adults has been shown to worsen outcome Hypertonic saline solutions have been used
and presumably does so in children. There- for initial resuscitation, as maintenance fluid
Airway control, oxygenation fore hyperventilation to a PaCO2 of and as specific treatment for raised ICP. In
and ventilation 25 mmHg should be reserved for the child addition to rapidly restoring circulating vol-
It is important to rapidly identify and who is rapidly deteriorating with signs of ume, increasing blood pressure and reducing
correct hypoxia as this is a significant increased intracranial pressure or cerebral ICP, these solutions appear to have impor-
contributor to secondary brain injury.4952 herniation, such as new onset pupil asymme- tant and beneficial immuno-modulatory and
Supplemental oxygen should be applied in try or rapidly decreasing GCS. neuro-chemical effects that may reduce sec-
order to maximise oxygen delivery to any ondary brain injury.57,59
ischaemic tissue and the childs SaO2 should Circulation A suggested regimen for the use of 3%
be monitored. Hypotension is the single most significant fac- saline for either initial resuscitation or to
Children who are not protecting their air- tor contributing to secondary brain insult.49,51 rapidly decrease ICP is to give a 5 mL kg1
way or maintaining adequate ventilation Hypotension is defined as an SBP <90 mmHg bolus, repeated if necessary according to
should be intubated and ventilated. In gen- in adults and a SBP <5th percentile for age in patient response. It would appear that rapid
eral, this would include any child with a GCS children.4952,54 One or more episodes of changes in serum sodium concentration do

8. In-line stabilisation of the cervical spine hypotension from time of injury through not cause complications such as central pon-
during rapid sequence oral intubation resuscitation at least doubles mortality and tine myelinolysis in humans, and most stud-
should occur to prevent potential secondary significantly increases morbidity.49,51,5456 ies do not place an upper limit on serum
spinal injury. Rarely, the agitated or combat- Despite studies focusing upon SBP, the sodium concentration.59,60
ive child with a higher GCS may require intu- true objective in the patient with TBI is to However, there have been no controlled
bation in order to facilitate imaging or to maintain an adequate CPP. This is the differ- trials demonstrating improved outcomes
stabilise a potentially at risk cervical spine ence between mean arterial pressure with hypertonic saline.
until it can be cleared. (MAP) and intracranial pressure (ICP) [i.e.
Pharmacological adjuncts to intubation CPP MAP ICP]. The normal ICP is
should be used as required. However, care 010 mmHg and an ICP of 20 mmHg is gen- Mannitol
is required to avoid transient hypotension erally regarded as the threshold for initiating Mannitol has been used as both a resuscita-
as this worsens neurological outcome (see specific therapy to reduce it. The optimal tion fluid (plasma expander) and as therapy
below). The endotracheal tube should be CPP is uncertain but consensus opinion for acute deterioration secondary to increas-
securely fastened to the childs face with recommends not less than 50 mmHg and ing ICP. Boluses of 0.25 g kg1 to 1 g kg1
tape rather than using the standard not greater than 70 mmHg, as aggressive body weight have been used successfully
tie around the neck. This avoids the risk of attempts to maintain CPP > 70 mmHg may for short-term reduction of ICP.61 Mannitol
impairing cerebral venous drainage and also worsen outcomes.57 In the pre-hospital acts as an osmotic diuretic and can lead to
causing a consequent rise in intracranial and emergency department settings, the subsequent problems with hypovolaemia
pressure (ICP). Hard collars may also con- ICP is unknown so treatment is purely empir- and acute renal failure via acute tubular
strict the neck and therefore interfere ical. It is reasonable to aim for a MAP of necrosis. A loop diuretic such as furosemide
with venous drainage, so need to be fitted between 70 and 90 mmHg that would main- is sometimes used in addition to mannitol
correctly. tain a CPP between 50 and 70 mmHg (i.e. for treatment of acute rises in ICP. A urinary
An orogastric tube should be passed to allowing for ICPs up to 20 mmHg). catheter is essential in any patient receiving
decompress the stomach. The nasogastric Initial resuscitation should be with mannitol or diuretics.
route should be avoided as inadvertent dextrose-free isotonic solutions such as
intracranial placement can occur if the child 0.9% saline or compound sodium lactate Positioning
has a basal skull fracture. (Hartmanns) solution, although the use of The childs head should be kept in the mid-
SaO2 and PaO2 are not good indicators hypertonic saline may be considered (see line to avoid jugular venous compression.
of adequate ventilation and continuous below). In the hypotensive patient, give a Venous drainage is improved if the head
ETCO2 monitoring should occur if the child 20 mL kg1 bolus (if using an isotonic crys- of the bed is elevated 1530 degrees, with
is intubated. The child should be venti- talloid) and monitor the response. Repeat resultant decrease in ICP. However, CPP is
lated to maintain an ETCO2 in the low if the patient remains hypotensive. After a also reduced by this manoeuvre and there

61
3.2 PAEDIATRIC NEUROTRAUMA

may be no net benefit unless ICP monitor- date no overall improvements have been The recommended dosing schedule is: methyl-
ing is in place and CCP adjusted as required. identified and they may actually worsen prednisolone 30 mg kg1 bolus over 15 min-
outcome. Routine use is no longer recom- utes followed by a 45-minute break, then
Prophylactic anti-seizure therapy mended.6769 5.4 mg kg1 hr1 continuous infusion for 23
Seizures that occur within seven days of hours.14
a TBI are termed early post-traumatic sei-
Thermoregulation: prophylactic
zures (EPTS) and those thereafter are late
hypothermia and prevention of Supportive care
post-traumatic seizures (LPTS).62 The overall
hyperthermia
incidence of seizures in children with TBI is
Mild hypothermia (3235 C) is known to Immobilisation of children at risk of spinal
probably between 5 and 15% but rises with injury is difficult when they are non-
decrease ICP and, in animal models, has
increasing severity of TBI, occurring up to co-operative and needs to include the body
been shown to be neuroprotective.59,70,71
40% of the time in those with a GCS as well as the head. Involvement of parents
Outcomes in humans have not been consis-

8.51,6264 Greater than 95% of PTS are or carers and calming of the child are impor-
tently better and mild hypothermia may
early and approximately 80% of these tant adjuncts. Pressure area care should
increase complications such as sepsis, pneu-
occur within the first 24 hours. occur for all patients who are immobilised.
monia, bleeding and mortality in the TBI
Seizures may cause or exacerbate second- Children who arrive in the emergency
child.59,72 Research is ongoing in this area
ary brain injury by increasing cerebral department on spinal boards should be
and prophylactic hypothermia should be
metabolic demands, increasing intracerebral moved off them as soon as possible as
considered in consultation with local PICU
pressure and by causing or exacerbating pressure areas may develop rapidly.
intensivists regarding individual cases.
cerebral hypoxia.63,64 Hard collars should be checked for
Hyperthermia is associated with a worse
The incidence of EPTS can be reduced by correct fit and potential pressure areas.
outcome in children with severe TBI.72
the use of prophylactic anticonvulsants They should be changed to padded collars
It is not known if actively cooling the patient
such as phenytoin. Their use should be such as Aspen or Philadelphia collars if
alters outcome and further research needs
considered65 in those with: immobilisation is needed for an extended
to be conducted in this area. In the mean-
GCS <10; time it would seem reasonable to attempt period.
seizure within 24 hours of injury; to cool a febrile brain-injured child. A urinary catheter should be placed in
depressed skull fracture or penetrating children who require volume resuscitation,
head wound; mannitol or diuretics, who are unable to
subdural, extradural or intracerebral Spinal cord injury communicate their need to micturate
haematomas; because of TBI, or who have a spinal cord
There is a great paucity of research into the injury.
cortical contusion.
optimal management of acute spinal cord The childs temperature should be moni-
It should be noted, however, that reduced injury in children. Therefore there are insuf- tored and inadvertent cooling avoided (see
EPTS does not translate into reduced ficient data to support diagnostic or treat- comments on thermoregulation above).
mortality65,66 and it remains to be seen ment standards.40 However, the principles The childs analgesic requirements should
whether or not an overall improved level of management of acute spinal cord injury be regularly reviewed and analgesia titrated
of functioning occurs in those survivors are considered to be no different from TBI. as required. Attention should be given to
given prophylactic anticonvulsant therapy. The focus of therapy is to prevent secondary eye protection in children who are sedated
It should also be noted that prophylactic injury. Attention should be paid to the or ventilated.
anticonvulsants do not alter the incidence maintenance of strict spinal immobilisation,
of LPTS and their routine use after 7 days adequate oxygenation, ventilation, blood
is not recommended.65 pressure and good supportive care as per
Family considerations
The management of active seizures moderate-severe TBI.
should be with benzodiazepines and The use of high-dose steroids in acute There are considerable immediate stresses
should follow the guidelines discussed in spinal injury is controversial.73,74 The issue during the initial stabilisation phases on
Chapter 8.3. is further complicated in the paediatric the parents and family of the injured child.
population by the fact that children <13 years The family requires appropriate support
Antibiotics were excluded from the major trials of and explanation whilst in the emergency
Intravenous antibiotic prophylaxis with flu- steroids for spinal cord injury.40 Routine use department. If possible, it is useful to provide
cloxacillin 2550 mg kg1 6-hourly should of high-dose steroids for spinal cord injury a dedicated staff member to be with them.
be given for compound skull fractures or is no longer recommended by the Neuro- The family should be kept well informed
fractures in communication with sinuses. surgical Society of Australasia.14 If steroids of the childs status and its proposed man-
are used, in consultation with local paedi- agement by a designated senior medical
Steroids atric neurosurgical practice, they should member of the resuscitation team and con-
Various steroids have been used in an at- be administered within 8 hours of injury. sideration should be given to allowing
tempt to improve outcome from TBI. To Practice currently varies between institutions. parents into the resuscitation room to be

62
3.2 PAEDIATRIC NEUROTRAUMA
3

TRAUMA IN CHILDREN
with their child (see Chapter 2.1). Premature 10. Lloyd D, Carty H, Patterson M, et al. Predictive value with apparently minor head trauma: proposed guidelines.
of skull radiography for intracranial injury in children with Pediatrics 2001;107:98393.
or vague conclusions of prognosis should be blunt head injury. Lancet 1997;349:8214. 36. Eleraky M, Theordore N, Adams M, et al. Paediatric cervical
avoided until all relevant assessments and 11. Quayle K, Jaffe D, Kuppermann N, et al. Diagnostic testing spine injuries; Report of 102 cases and review of the
for acute head injury in children: When are head literature. J Neurosurg (Spine 1) 2000;92:127.
investigations have been made. computed tomography and skull X-rays indicated? 37. Hendey G, Wolfson A, Mower W, et al. Spinal cord injury
Pediatrics 1997;99:e11. without radiographic abnormality; Results of the
12. Schutzman S, Greenes D. Paediatric minor head trauma. national emergency X-ray utilization study in blunt
Ann Emerg Med 2001;37(1):6574. cervical trauma. J Trauma 2002;53(1):14.
13. Moura dos Santos A, Plese J, Ciquini O, et al. 38. Schwartz D, Reisdorff E. Emergency Radiology. USA:
Disposition Extradural hematomas in children. Pediatr Neurosurg McGraw-Hill; 2000.
1994;21:504. 39. Sledge J, Dain A, Hyman J. Use of magnetic resonance
The disposition for children with mild TBI 14. Neurological Society of Australasia and Royal imaging in evaluating injuries to the paediatric
Australasian College of Surgeons. The Management of thoracolumbar spine. J Pediatr Orthop 2001;21(3):28893.
has been outlined above. Children sent Acute Neurotrauma in Rural and Remote Locations. 40. Hadley M. Management of paediatric cervical spine
home from the emergency department 2nd ed. 2009. and spinal cord injuries. Neurosurgery 2002;50(3):
15. Keskil I, Baykaner M, Ceviker N, et al. Assessment of S8599.
following a head injury must be discharged mortality associated with mild head injury in the paediatric 41. Frank J, Lim C, Flynn J, et al. The efficacy of magnetic
to the care of a responsible adult who is age group. Childs Nerv Syst 1995;11:46773. resonance imaging in paediatric cervical spine clearance.
16. Lang D, Teasdale G, MacPherson P, et al. Diffuse brain Spine 2002;27(11):11769.
given clear discharge instructions and a swelling after head injury: More often malignant in adults 42. Simon B, Letourmeau P, Vitorino E, et al. Paediatric minor
written head-injury sheet containing advice than children? J Neurosurg 1994;80:67580. head trauma: Indications for computed tomographic
17. Bratton S, Chestnut R, Ghajar J, et al. Cerebral perfusion scanning revisited. J Trauma 2001;51(2):2318.
on when to seek review. Children who thresholds. J Neurotrauma 2007;24:S5964. 43. Brenner D, Hall E. Computed Tomography An
have had a significant concussive injury 18. Ratan S, Pandey R, Ratan J. Association among duration increasing source of radiation exposure. N Engl J Med
of unconsciousness, Glasgow Coma Scale, and cranial 2007;357:227784.
need to rest quietly for the next 2448 computed tomography abnormalities in head injured 44. Mandera M, Wencel T, Bazowski P, et al. How should we
hours avoiding television, computers, children. Clin Pediatr July, 2001;3758. manage children after mild head injury? Childs Nerv Syst
19. Teasdale G, Jennett B. Assessment of coma and impaired 2000;16:15660.
music inputs, in order to minimise excessive consciousness. Lancet July 1974;813. 45. Ng S, Toh E, Sherrington C. Clinical predictors of abnormal
visual-auditory stimulation in order for their 20. Simpson D, Reilly P. Paediatric Coma Scale. Lancet August computed tomography scans in paediatric head injury.
1982;450. J Paediatr Child Health 2002;38:38892.
symptoms to defervesce brain rest. Return- 21. Mild Traumatic Brain Injury Committee of the Head Injury 46. Batchelor J, McGuiness A. A meta-analysis of GCS 15
ing to these activities too soon will often Interdisciplinary Special Interest Group of the American head injured patients with loss of consciousness or
Congress of Rehabilitation Medicine. Definition of mild post traumatic amnesia. Emerg Med J 2002;19:5159.
cause a recrudescence of their symptoms. traumatic brain injury. J Head Trauma Rehabil 47. Simon B, Letourneau P, Vitorino E, et al. Pediatric minor
Simple analgesics such as paracetamol can 1993;8(3):867. head trauma: Indications for computed tomographic
22. Culotta V, Sementilli M, Gerold K, et al. scanning revisited. J Trauma 2001;51(2):2318.
be used for mild headache, which should Clinicopathological heterogeneity in the classification of 48. Sainsbury C, Sibert J. How long do we need to observe head
be expected to be short-lived. Any child with mild head injury. Neurosurgery 1996;38(2):24550. injuries in hospital? Arch Dis Child 1984;59:8569.
23. Committee on quality improvement, American 49. Philip S, Udomphorn Y, Kirham F, et al. Cerebrovascular
a suspected NAI should be admitted to hos- Academy of Paediatrics. The management of minor Pathophysiology in Pediatric Trauma Brain Injury.
pital for safety and further evaluation. closed head injury in children. Paediatrics J Trauma 2009;67(2):S12834.
1999;104(6):140715. 50. Chiaretti A, De Benedictis R, Della Corte F, et al. The impact
All children with moderate or severe TBI 24. Murgio A, Patrick P, Andrade F, et al. International study of initial management on the outcome of children with
or spinal cord injury should be transferred of emergency department care for paediatric traumatic severe head injury. Childs Nerv Syst 2002;18:5460.
brain injury and the role of CT scanning. Childs Nerv Syst 51. Chiaretti A, Piastra M, Pulitano S, et al. Prognostic factors
to a paediatric high dependency or intensive 2001;17:25762. and outcome of children with severe head injury: An
care unit for admission under the care of a 25. Davis R, Mullen N, Makela M, et al. Cranial computed 8 year experience. Childs Nerv Syst 2002;18:12936.
tomography scans in children after minimal head injury with 52. Ong L, Selladurai B, Dhillon M, et al. The prognostic value
neurosurgeon. loss of consciousness. Ann Emerg Med 1994;24(4):6405. of the Glasgow Coma Scale, hypoxia and computerised
26. Schunk J, Rodgerson J, Woodward G. The utility of tomography in outcome prediction of paediatric head
head computed tomographic scanning in paediatric injury. Pediatr Neurosurg 1996;24:28591.
patients with normal neurologic examination in the 53. Skippen P, Seear M, Poskitt K, et al. Effect of
emergency department. Pediatr Emerg Care hyperventilation on regional cerebral blood flow in head-
References 1996;12(3):1605. injured children. Crit Care Med 1997;25(8):14029.
1. Martin C, Falcone R. Pediatric traumatic brain injury: an 27. Roddy S, Cohn S, Moller B, et al. Minimal head trauma in 54. Traumatic Brain Injury Guidelines Taskforce.
update of research to understand and improve outcomes. children revisited: Is routine hospitalisation required? Hypotension. J Neurotrauma 2000;17(6/7):5915.
Curr Opin Pediatr 2008;20:2949. Pediatrics 1998;101(4):5757. 55. Winchell R, Simons R, Hoyt D. Transient systolic
2. OConnor P. Hospitalisation due to traumatic brain injury, 28. Johnson D, Krishnamurthy S. Severe paediatric head hypotension. A serious problem in the management of
Australia 199798. Australian Institute of Health and injury: Myth, magic, and actual fact. Pediatr Neurosurg head injury. Arch Surg 1996;131:5339.
Welfare; 2002. 1998;28:16772. 56. Kokoska E, Smith G, Pittman T, et al. Early hypotension
3. Khan F, Baguley I, Cameron D. Rehabilitation 29. Hahn Y, Chyung C, Bartherl M, et al. Head injuries in worsens neurological outcome in paediatric patients with
after traumatic brain injury. Med J Aust children under 36 months of age. Demography and moderately severe head trauma. J Pediatr Surg
2003;178(6):2905. outcome. Childs Nerv Syst 1988;4:3440. 1998;33(2):3338.
4. Research Centre for Injury Studies. Spinal cord injury, 30. Haydel M, Preston C, Mills T, et al. Indications for 57. Banks C, Furyk J. Review article: Hypertonic saline use in
Australia 1995/6. Australian Injury Prevention Bulletin computed tomography in patients with minor head the emergency department. Emerg Med Australas
1998;18. injury. N Engl J Med 2000;343(2):100105. 2008;20:294305.
5. OConnor. December Spinal cord injury, Australia, 31. Vavilala M, Dunbar P, Rivara F, et al. Coagulopathy 59. Walker P, Harting M, Baumgartner J, et al. Modern
1999-00. Australian Institute of Health and Welfare. predicts poor outcome following head injury in children approaches to pediatric brain injury therapy. J Trauma
2001. less than 16 years of age. Journal of Neurosurgical 2009;67(2):S1207.
6. Anderson V, Catroppa C, Morse S, et al. Outcome from Anaesthetics 2001;13(1):138. 60. Peterson B, Khanna S, Fisher B, et al. Prolonged
mild head injury in young children: A prospective study. 32. Keller M, Fendya D, Weber T. Glasgow Coma Scale hypernatremia controls elevated intracranial pressure in
J Clin Exp Neuropsychol 2001;23(6):70517. predicts coagulopathy in paediatric trauma patients. head-injured paediatric patients. Crit Care Med
7. Poussaint T, Moeller K. Imaging of paediatric Semin Pediatr Surg 2001;10(1):126. 2000;28(4):113643.
head trauma. Neuroimaging Clin N Am 2002;12: 33. Homer C, Kleinman L. Technical report: Minor head injury 61. Bratton S, Chestnut R, Ghajar J, et al. Hyperosmolar
27194. in children. Pediatrics 1999;104(6):1380. therapy. J Neurotrauma 2007;24:S1420.
8. Lazar L, Erez I, Gutermacher M, et al. Brain concussion 34. Gruskin K, Schutzman S. Head trauma in children 62. Ong L, Dhillon M, Selladurai B, et al. Early post-
produces transient hypokalemia in children. J Pediatr younger than 2 years: Are there predictors for traumatic seizures in children: Clinical and radiological
Surg 1997;32(1):8890. complications? Arch Pediatr Adolesc Med aspects of injury. J Paediatr Child Health 1996;32:1736.
9. Hahn Y, McLone D. Risk factors in the outcome of children 1999;153(1):1520. 63. Ratan S, Kulshreshtha R, Pandey R. Predictors of
with minor head injury. Pediatr Neurosurg 35. Schutzman S, Barnes P, Duhaime A-C, et al. Evaluation posttraumatic convulsions in head injured children.
1993;19:13542. and management of children younger than two years old Pediatr Neurosurg 1999;30:12731.

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64. Chiaretti A, Benedictis R, Polidori G, et al. Early post- 71. Hutchison J, Ward R, Lacroix J. Hypothermia: Therapy Bratton S, Chestnut R, Ghajar J, et al. Hyperventilation.
traumatic seizures in children with head injury. Childs after traumatic brain injury in children. N Engl J Med J Neurotrauma 2007;24:S8790.
Nerv Syst 2000;16:8626. 2009;358:244756. Bratton S, Chestnut R, Ghajar J, et al. Blood pressure and
65. Bratton S, Chestnut R, Ghajar J, et al. Antiseizure 72. Natale J, Joseph J, Helfaer M, et al. Early hyperthermia oxygenation. J Neurotrauma 2007;24:S713.
prophylaxis. J Neurotrauma 2007;24:S836. after traumatic brain injury in children; Risk factors, Davis R, Hughes M, Gubler D, et al. The use of cranial CT scans
66. Haltiner A, Newell D, Temkin N, et al. Side effects and influence on length of stay, and effect on short- in the triage of paediatric patients with mild head injury.
mortality associated with use of phenytoin for early term neurologic status. Crit Care Med Paediatrics 1995;95(3):3459.
posttraumatic seizure prophylaxis. J Neurosurg 2000;28(7):260815. Mitchell K, Fallat M, Raque G, et al. Evaluation of minor head
1999;91:58892. 73. Hurlbert R. The role of steroids in acute spinal cord injury; injury in children. J Pediatr Surg 1994;29(7):8514.
67. Bratton S, Chestnut R, Ghajar J, et al. Steroids. An evidence-based analysis. Spine 2001;26(24 Suppl.): Smally A. Management of minor closed head injury in
J Neurotrauma 2007;24:S915. S3946. children. Paediatrics 2001;107(5):1231.
68. Alderson P, Roberts I. Corticosteroids for acute traumatic 74. Canadian Association of Emergency Physician Position Temkin N, Dikmen S, Anderson G, et al. Valproate therapy for
brain injury (review). Cochrane Database Syst Rev Statement. Steroids in acute spinal cord injury. www prevention of posttraumatic seizures: A randomised trial.
2009;(4). .caep.ca; 2003 [Revised 22 January 2003]. J Neurosurg 1999;91:593600.
69. Roberts I. Aminosteroids for acute traumatic brain injury. Traumatic Brain Injury Guidelines Taskforce. Glasgow Coma
Cochrane Database Syst Rev 2009;(4). Scale score. J Neurotrauma 2000;17(6/7):56370.
70. Sydenham E, Roberts I, et al. Hypothermia for traumatic Traumatic Brain Injury Guidelines Taskforce. Pupillary
brain injury. Cochrane Database of Systematic Reviews Further reading diameter and light reflex. J Neurotrauma
2009;(4). Beattie T. Minor head injury. Arch Dis Child 1997;77:825. 2000;17(6/7):58390.

3.3 Thoracic injuries in childhood

Philip Aplin

region injury to be almost universal (99%)

ESSENTIALS in cases of severe blunt chest trauma. The
most frequent associated serious injuries
1 Thoracic injuries in children are common, are usually due to blunt trauma and are were head (46%), lower extremity (32%)
often associated with other injuries.
and abdominopelvic injury (30%). By far
2 The pathophysiological differences in children depend principally on their age and and away the commonest chest injuries were
size. These must be appreciated in order to optimise outcome. pulmonary contusion, haemopneumothorax
and rib fractures.
3 The most common injuries are pulmonary contusions, rib fractures, The common mechanisms of injury vary
pneumothoraces and haemothoraces. These can usually be managed by a
with the age of the child. Overall, the majority
combination of adequate oxygenation, IV fluids, thoracostomy tube drainage and
(6080%) are due to blunt trauma and
analgesia.
involve a motor vehicle in over half. In infants
4 Injuries to the aorta and diaphragm may be clinically occult and CXR findings are and toddlers common mechanisms include
often non-specifically abnormal. Further diagnostic evaluation is always indicated in being injured as passengers in motor vehicle
suspect cases. collisions (MVC) or as pedestrians struck or
run over by a vehicle (commonly in the drive-
5 Surgical intervention is uncommonly required, but is indicated in cases of massive way of the family home). Falls (from stairs,
haemothorax, cardiac tamponade, major airway and oesophageal injury, as well as in
balconies, etc.) occur mainly in this age
aortic and diaphragmatic injuries.
group. Child abuse also tends to predominate
6 ED thoracotomy is principally indicated in cases of penetrating chest trauma and in this age group and should always be con-
an arrest or pre-arrest presentation. sidered. In school age children motor vehicle
and bicycle related trauma is common and
sporting ( extreme sports) injuries increase
in frequency with age. With adolescence the
occurrence of penetrating trauma emerges,
abdominal injury to as high as 20%. with an associated increased mortality risk;
Introduction14 Injuries to the great vessels, bronchi, lung also, inexperienced teenage drivers have an
Traumatic injury is the most common lacerations and cardiac tamponade are increased incidence of MVC. Drug and alco-
cause of morbidity and mortality in child- the chest injuries most likely to cause hol intoxication is often associated with
hood and thoracic injuries are secondary early death. Small children provide a small personal/interpersonal violence in this older
to head injuries as a cause of mortality. target in blunt trauma, so multiple injuries age group.
Isolated chest injuries have a mortality should be expected. There are a number of anatomical and
of around 5% but this increases substan- A recent review of an Australian trauma physiological features of small children that
tially when combined with head and/or registry database found multiple body must be appreciated when managing

64
3.3 THORACIC INJURIES IN CHILDHOOD
3

TRAUMA IN CHILDREN
Assisted ventilation is indicated for those
Table 3.3.1 Important pathophysiological Table 3.3.2 Indications for operative
differences between children and adults in intervention in chest trauma with respiratory failure despite optimal
chest trauma non-invasive ventilation and analgesia or
Great vessel injury
Small target multiple injuries common Pericardial tamponade associated injuries, particularly to the head.
Narrow airway prone to obstruction Large haemothoraces Management of rib fractures involves
Anterior larynx difficult intubation Tracheobronchial injuries
Increased surface area increased risk Oesophageal injury analgesia, treatment of associated injuries,
of hypothermia Diaphragmatic lacerations ongoing assessment of the childs respira-
Increased oxygen consumption, Open pneumothorax with major chest wall
diaphragmatic breathing, low functional defect tory status and close observation for compli-
residual capacity prone to hypoxia Penetrating chest trauma that crosses the cations that may arise. Analgesic options
Increased pliability of ribs decreased mediastinum
incidence of rib fractures in the ED include oral paracetamol (also
Response to blood loss hypotension is a Once stabilised, thoracic CT scan may be indicated to available intravenously (IV)) and anti-
late sign of shock further delineate the extent of pulmonary injury,
Developmental considerations paediatric evaluate the great vessels and detect inflammatories, intranasal fentanyl and
coma scoring pneumothoraces. Analgesia should be initiated early in titrated IV narcotics. Prevention of atelecta-
appropriate doses.
sis and pneumonia is a priority.

paediatric chest trauma. These are sum- is not immediately available. Other indica-
marised in Table 3.3.1. tions for operative intervention are listed
in Table 3.3.2. CT imaging of the chest
Pulmonary injury7,8
should be used selectively. It is indicated Contusion
in high impact trauma and when multiple In children it is important to appreciate that
Initial approach in the ED5 injuries are present or suspected, particu- significant lung injury can occur in the
Initial management follows the usual priori- larly severe head injury where there is a high absence of rib fractures or other external
ties. After ensuring airway patency, breath- likelihood of associated severe chest injury. signs of chest wall injury. Pulmonary contu-
ing should be assessed. High-flow oxygen sion most commonly results from blunt
should be applied. Signs of respiratory com- injury and requires significant force. Patho-
promise and tension pneumothorax should logical findings are intra-alveolar haemor-
Chest wall injury6
be managed by needle decompression prior rhage, consolidation and oedema causing
to chest X-ray (CXR) followed by chest tube Rib fractures V/Q mismatch, reduced lung compliance
insertion. A large haemothorax may com- The elasticity and flexibility of the younger and subsequent hypoxaemia. CXR findings
promise ventilation as well as circulation, childs chest wall leads to a lower incidence of increased pulmonary opacity may be pres-
requiring early chest tube placement and of rib fractures. Significant underlying intra- ent in the ED but may evolve over time.
fluid resuscitation, whilst an orogastric tube thoracic injury can occur in the absence of Computerised tomography (CT) scanning
(OGT) should be placed early to decompress rib fractures. In the 0- to 3-year age group of the thorax may reveal contusion that is
the stomach, as gastric distension may com- rib fractures should raise the concern of not evident on the initial plain films.
promise ventilation. Mechanical ventilation abuse: in one study 2/3 of 03-year-olds Approximately half of the CXRs showing
should be instituted for signs of ongoing with rib fractures were victims of abuse, pulmonary contusion show other abnor-
respiratory distress/respiratory failure not and a careful assessment of all aspects of malities, most commonly fractured ribs and
relieved by optimisation of oxygen delivery, the clinical presentation is mandatory. pneumo/haemothoraces.
chest tube insertion, closure of open chest Radiological findings suggestive of abuse Management of pulmonary contusion
wounds and OGT placement. Ongoing include: multiple fractures, fractures of involves:
signs of circulatory compromise without varying ages and bilateral fractures. A bone
evidence of blood loss should raise the pos- scan is a more sensitive test in the setting of
Maintenance of oxygenation.
Supplemental 02 is initially delivered
sibility of cardiac tamponade and myo- potential abuse.
by high flow mask (1015 L min1).
cardial contusion in a child with chest Rib fractures in children are a marker of
Persisting hypoxaemia requires non-
injuries. A portable CXR should be the first potential severe associated injuries. Multiple
invasive or invasive ventilatory support
radiological test ordered. FAST (focused rib fractures (>1) increase the risk of severe
depending on severity and associated
abdominal scan in trauma) scanning should intrathoracic injury, multiple injuries and
injuries.
occur early in the resuscitation of a child, mortality. Fracture of the first rib requires
where available, and imaging of the pericar- significant force, mandating a high degree
Pain relief should be provided as
indicated.
dium should always be included to detect of suspicion of associated injuries to the
haemopericardium. The vast majority of great vessels and the trachea.
Excessive intravenous fluids should be
avoided.
chest injuries in childhood can be managed Flail chest injuries are rare in children and
non-operatively. Drainage of pericardial clearly indicate serious injury, with reduced
Respiratory physiotherapy is useful.
blood may occasionally be performed in ventilatory effectiveness and associated Both acute respiratory distress syndrome
the emergency department (ED) in an lung contusions contributing to the signifi- and pneumonia may complicate pulmonary
unstable patient if operative intervention cant potential for respiratory failure. contusion.

65
3.3 THORACIC INJURIES IN CHILDHOOD

Pneumothorax4,9 decompression with a 16G cannula inserted pericardial tamponade or massive hae-
Traumatic pneumothoraces vary in their size into the 2nd intercostal space in the mid mothorax are the main other possibilities
and clinical significance. They occur in about clavicular line should occur whilst preparing in the chest for such a sudden deterioration.
1/3 of children with significant thoracic for formal intercostal tube insertion via a Lung hyperinflation due to overly aggressive
trauma and associated injuries are common. lateral approach. positive pressure ventilation may also cause
All pneumothoraces should be considered as However, hypotension and/or hypoxaemia cardiorespiratory compromise and risk
having the potential to cause cardiores- may have other causes in the traumatised barotrauma.
piratory compromise. The clinical signs of child. The differential diagnosis of a tension In the setting of deterioration after initia-
pneumothorax (PTX) vary from nothing to PTX includes: haemorrhage; pericardial tion of ventilation, by far the most likely
decreased air entry, hyperresonance and tamponade; haemothorax (which may cause cause is the development of tension PTX,
subcutaneous emphysema. tension); pulmonary contusion; and air embo- which should be managed as above. In the
Small to medium sized pneumothoraces lism. Common, easily preventable/treatable rare event of air embolism, if suspected,
may not be visible on a portable supine causes that may occasionally be confused management includes 100% oxygen, and
CXR. Ultrasound of the chest may be with a tension PTX are intubation of the right reduction of the ventilation pressures.
incorporated into the FAST scan protocol to main-stem bronchus and gastric distension. Emergency thoracotomy with clamping of
detect pneumothoraces and haemothoraces. Endotracheal tubes (ETT) must be inserted the hilum on the affected side and aspira-
The sensitivity of ultrasound in detecting the appropriate distance (age/2 12 cm) tion of ventricular air has occasionally been
these complications of chest trauma is supe- and movement of the childs neck minimised. life saving.
rior to supine CXR but CT scanning remains This is particularly so in small children where
the gold standard. Subtle signs on CXR neck flexion (tube pulled out) or extension
include increased radiolucency on the ipsilat- (tube down the [R] main bronchus) may result
Haemothorax9,10
eral side and a deep sulcus sign. Small pneu- in the malposition of the ETT. An orogastric
Clinically relevant haemothoraces occur in
mothoraces are commonly revealed on CT tube should be placed early.
about 15% of cases of blunt chest trauma
scan of the chest and/or abdomen. The sig- Open pneumothoraces may occur with
but are more common if the injury is
nificance and hence management of these penetrating chest trauma. Respiratory
penetrating. The source of bleeding is most
small pneumothoraces is debated. A small compromise relates to the effects of the
commonly from lacerations to the lung,
uncomplicated PTX in a stable patient with PTX, underlying lung injury and a sucking
intercostal or internal mammary vessels or
isolated chest trauma, who is not likely to chest wound if the defect is large enough.
occasionally from mediastinal vessel injury
require positive pressure ventilation or pro- If the diameter of the chest wound is
(often fatal). Each hemithorax can hold up
longed transport (particularly aeromedical), approximately 2/3 or greater than that
to 40% of a childs blood volume.
can be considered for observation, high-flow of the trachea, air will preferentially be
The clinical presentation is of varying
O2 and analgesia in a high-dependency unit sucked into the chest on inspiration, lead-
degrees of hypovolaemia and respiratory
setting. Most other traumatic pneu- ing to acute severe respiratory compromise.
compromise depending on the amount of
mothoraces require the insertion of a formal Management requires urgent wound cover-
blood lost into the chest, associated pneumo-
chest drain. age on three sides only with an occlusive
thorax and the development of increased
dressing, to prevent the development of
intrathoracic pressure. Chest examination
an iatrogenic tension PTX, and chest tube
reveals reduced air entry and dullness to
Tension pneumothorax placement away from the wound. Once
percussion  signs of tension. Management
This is a clinical condition resulting from the chest drain is in place the wound can
is with a chest drain. Drainage of massive hae-
increasing intrathoracic pressure, lung col- be sealed and arrangements made for
mothoraces may precipitate further bleeding
lapse and mediastinal shift with subsequent definitive surgical care. Significant ongoing
as the tamponade effect is removed.
impaired gas exchange, decreased venous respiratory distress is an indication for
Blood loss from the chest tube, haemody-
return and cardiovascular collapse. The diag- mechanical ventilation.
namic response to resuscitation, mechanism
nosis is clinical and treatment should pre-
of injury (blunt vs. penetrating) and asso-
cede radiology in clear-cut cases. Signs Pulmonary lacerations5
ciated injuries (especially head) are used
are of # air entry, hyperresonance and hyper- Pulmonary lacerations are principally
by cardiothoracic surgeons in determining
expansion plus # movement of the affected caused by penetrating injuries but can occur
the need for thoracotomy.
side. The signs of tracheal deviation and ele- with blunt mechanisms especially associated
Indications for thoracotomy include:
vation of the jugular venous pressure (JVP) with rib fractures. They usually result in a
may be difficult to detect in children who haemothorax (sometimes massive) or PTX initial drainage exceeding >15 mL kg1
have short necks. JVP elevation may also and rarely can be complicated by air embo- of estimated blood volume;
be absent if there is associated hypovolae- lism. Air embolism typically occurs after the continued bleeding greater than
mia. Patients are always tachypnoeic initiation of positive pressure ventilation 12 mL kg1 hr1;
with respiratory distress and tachycardia, and causes sudden haemodynamic deterio- increasing bleeding;
but hypotension is a late sign if solely ration with or without focal neurological significant residual haemothorax post-
due to a tension PTX. Immediate needle signs. Development of a tension PTX, tube drainage.

66
3.3 THORACIC INJURIES IN CHILDHOOD
3

TRAUMA IN CHILDREN
Management involves oxygenation and ven- results. Most of the evidence comes from
Table 3.3.3 CXR signs of aortic injury
tilatory support if indicated, urgent chest adult trauma patients. A normal ECG has a
Widened mediastinum (mediastinum to chest
tube placement of the appropriate size via ratio >0.25)
high negative predictive value for the occur-
the lateral approach directed posteriorly Loss or abnormal contour of aortic knob rence of clinically significant complications
Depression of left mainstem bronchus
and volume resuscitation. The concept of Deviation of the trachea to the right
in suspected myocardial contusion. Evidence
hypovolaemic resuscitation in uncontrolled Deviation of the oesophagus (NG or OG tube) for the value of cardiac markers is lacking.
to the right
traumatic haemorrhage has not been evalu- Left pleural cap
Echocardiography is a very useful modality
ated in children. However, it is important to Left haemothorax in assessing suspected clinically significant
Upper rib fractures
consider early surgical intervention in any myocardial contusion such as the presence
patient who is haemodynamically compro- NG, nasogastric; OG, orogastric. of unexplained hypotension, tachycardia or
mised due to haemorrhage or showing signs new murmurs.
of ongoing bleeding. adolescents involved in high speed MVCs. In the absence of ECG abnormalities,
Longer-term complications of hae- About 80% occur at the aortic isthmus hypotension or new murmurs, ongoing ECG
mothoraces include haematoma organisa- just distal to the origin of the left subclavian monitoring is usually not required.
tion with secondary lung entrapment and artery. Most are rapidly fatal at the scene.
empyema formation. Prophylactic antibio- Diagnosis in hospital depends on clinical Commotio cordis12,13
tics are indicated when chest tubes are suspicion based on mechanism of injury, The phenomenon of sudden cardiac arrest
placed for penetrating trauma. The adult physical signs (often absent) and CXR find- following a localised blow to the chest is well
trauma literature suggests a reduced infec- ings. CXR in cases of aortic rupture is usu- documented in children. In these cases
tion rate even in previously closed traumatic ally abnormal but findings are non-specific autopsy fails to identify myocardial contu-
haemothoraces requiring drainage. (Table 3.3.3) and the suspicion of aortic sion, structural cardiac abnormality, conduc-
injury on clinical or radiological grounds tion system or coronary artery pathology.
requires further diagnostic imaging. The proposed theory is that a blow to the
Tracheobronchial injuries10 In young children the normal thymic chest during the vulnerable phase of the
contour may give the impression of a electrical cycle induces ventricular fibrilla-
These uncommon injuries may occur with
widened mediastinum. Further imaging tion/ventricular tachycardia. Protective
penetrating or severe blunt trauma and
usually involves CT angiography, aortogram chest guards are recommended in at-risk
have a high mortality if not recognised
or transoesophageal echocardiography sports.
and treated rapidly. In blunt trauma
depending on availability, expertise and
intrathoracic airway injuries usually occur
local practices. The absence of signs of Penetrating cardiac trauma14
near the origin of the main stem bronchi.
dissection and mediastinal haematoma on In children this occurs predominantly in the
Typically they present with respiratory
CT angiography is used to exclude aortic adolescent age group. Cardiac lacerations
distress and signs of subcutaneous (SC)
injury. Occasionally, formal aortography or may lead to rapid exsanguination or peri-
emphysema, pneumomediastinum and a
transoesophageal echocardiography will be cardial tamponade. Any penetrating chest
tension PTX. Haemoptysis also occurs. In
performed to exclude possible aortic injury. or upper abdominal wound has the poten-
the case of PTX urgent chest tube place-
Management in confirmed cases is surgi- tial to injure the heart. Clinical signs of tam-
ment is required and typically a large air
cal. b-blockers may be commenced pre- ponade include tachycardia and elevation
leak will continue and there will be failure
operatively in haemodynamically stable of the JVP (in the absence of hypovolae-
of lung expansion on CXR. At this point
patients to reduce vessel wall stress. mia), with subsequent hypotension and car-
an airway injury is usually considered. A sec-
diac arrest with pulseless electrical activity.
ond chest tube should be placed and
The CXR is typically normal in the absence
urgent cardiothoracic surgical consultation
of associated mediastinal or lung injury. In
obtained. Once stabilised, a CT scan may Cardiac injuries11
trained hands and with satisfactory imaging
be helpful in further injury delineation and
As with aortic injury, clinically significant conditions echocardiography has excellent
assessing lung inflation, as massive SC
cardiac injury from blunt trauma in children accuracy in the detection of pericardial
emphysema can make CXR interpre-
is uncommon and is usually associated with blood and can be done rapidly in the emer-
tation very difficult. Bronchoscopy is useful
other intrathoracic injuries. Pericardial tam- gency department (ED). Management
as a diagnostic modality in suspected tra-
ponade can certainly occur with blunt requires urgent cardiothoracic surgical
cheobronchial injury and operative interven-
trauma, though it is more common in involvement. A conscious patient with a
tion is often required.
penetrating injuries (see below). Myocardial perfusing blood pressure requires urgent
contusion may manifest as an arrhythmia or surgery. A rapidly deteriorating patient in
otherwise unexplained tachycardia and/or the ED requires needle pericardiocentesis
Mediastinal injury5,9,10
hypotension. Valvular injury and septal if there is any surgical delay. Cardiac arrest
Aortic transection defects are also reported. Diagnosis suffers with vital signs present at the scene and a
Aortic rupture is a rare event in young from the lack of a gold standard and the short transit time to hospital or arrest in the
children but the incidence increases with questionable clinical relevance of test ED is an indication for ED thoracotomy or

67
3.3 THORACIC INJURIES IN CHILDHOOD

pericardiocentesis depending on the skills can be investigated by laparoscopy/thora-

of personnel available. Pericardiocentesis coscopy or open operation. Controversies
is performed using a long 16 or 18G over The use of intercostal catheters in
the needle cannula via the subxiphoid small traumatic pneumothoraces is
approach at a 35 degree angle to the skin Oesophageal injury5
debated especially with positive
and aiming at the left shoulder with ECG This is essentially only seen in penetrating
pressure ventilation. The maximum size
monitoring. Ultrasound control may assist trauma and in these cases a high index of
that can safely be treated conservatively
where available. Aspiration of 1020 mL suspicion is required, as missed injuries
is not defined.
may result in significant clinical improve- cause inevitable serious morbidity and mor-
ments. The needle should be removed but tality. On initial CXR a finding of mediasti- The role of ED thoracotomy, in low
the catheter should remain in place for nal air is an early clue. Over subsequent volume centres (which includes all
repeat aspirations. Failure to aspirate blood hours an evolving sepsis with pleural effu- Australasian paediatric centres), is not
does not exclude tamponade as the can- sions and mediastinitis ensues. Conscious known.
nula may miss the pericardium or the peri- patients able to verbalise complain of chest
cardial blood may have clotted. and epigastric pain but this is difficult to
interpret in the setting of other chest inju-
ries. Suspicion of an oesophageal injury
must be followed by a Gastrografin study,
oesophagoscopy or both. If positive, References
Diaphragmatic injury9,10,15 broad-spectrum antibiotics and urgent sur- 1. Peclet MH, Newman KD, Eichelberger MR, et al. Thoracic
trauma in children: An indicator of increased mortality.
Diaphragmatic injury is another uncommon gery are required. J Pediatr Surg 1990;25:9615.
2. Black TL, Snyder CL, Miller JP, et al. Significance
paediatric injury, but it is important none- of chest trauma in children. South Med J
theless, as undiagnosed, complications 1996;89:4946.
3. Nakayama DK, Ramenofsky ML, Rowe MI. Chest injuries
eventually will arise, though this may take
ED thoracotomy14 in children. Ann Surg 1989;210:7705.
years. Left-sided injury is more common than 4. Samarsekara S, Mikocka-Walus A, Butt W, Cameron P.
Epidemiology of major paediatric chest trauma.
right-sided in blunt trauma and associated The only definite indication for thoracot- J Paediatr Child Health 2009;45:67680.
intra-abdominal injury is common. omy in the ED is in the scenario of 5. Fleisher GR, Ludwig S. Textbook of paediatric emergency
medicine. 4th ed. Philadelphia: Lippincott Williams &
Upper abdominal penetrating trauma penetrating chest trauma with loss of vital Wilkins; 2000. p. 134160.
that injures intrathoracic structures (and signs shortly before arrival in the ED or dur- 6. Garcia VF, Gotschall CS, Eichelberger MR, et al. Rib
fractures in children: A marker of severe trauma. J Trauma
vice versa) must also have caused diaphrag- ing ED resuscitation, with the purpose of 1990;30:695700.
matic injury and requires repair. Diagnosis pericardial drainage, repairing penetrating 7. Bonadio WA, Hellmich T. Post-traumatic pulmonary
contusion in children. Ann Emerg Med 1989;18:10502.
is difficult unless CXR reveals clear signs injury to the heart or controlling bleeding 8. Allen GS, Cox CS, Moore FA, et al. Pulmonary
of herniated stomach or bowel or the naso- from the hilum or lung. Open cardiac mas- contusion: Are children different? J Am Coll Surg
1997;185:22933.
gastric tube curling up into the thorax. sage can also be performed as well as 9. Bliss D, Silen M. Paediatric thoracic trauma. Crit Care Med
More commonly the CXR is non-specifically cross-clamping of the aorta. The universally 2002;30:113.
10. Wesson DE. Thoracic injuries. In: ONeill JA, editor. Paediatric
abnormal with findings of an abnormal dia- poor outcome of blunt trauma patients who surgery. 5th ed. St Louis: Mosby; 1998. p. 24560.
phragmatic contour with or without lower arrive at the ED with no vital signs/signs of 11. Dowd MD, Krug S. Pediatric blunt cardiac injury:
Epidemiology, clinical features and diagnosis. J Trauma
zone opacity. Often the diagnosis is made life argues strongly against performing 1996;40:112.
at laparotomy or laparoscopy for associated ED thoractomy in these patients. Blunt 12. Cantor RM, Leaming JM. Evaluation and management of
pediatric major trauma. Emerg Med Clin North Am
injuries. Barium studies are normal if bowel thoracic trauma patients who deteriorate 1998;16:22956.
contents are not herniated. CT scanning in the ED despite full resuscitation may 13. Maron BJ. Blunt impact to the chest leading to sudden
cardiac death from cardiac arrest during sports activities.
may miss small tears. Magnetic resonance occasionally survive following ED thora- N Engl J Med 1995;333:33742.
imaging may have a role in diagnosing cotomy but in general these very unstable 14. Polhgeers A, Ruddy RM. An update on pediatric trauma.
Emerg Med Clin North Am 1995;12:26787.
these injuries. Suspected occult diaphrag- patients should go to the operating theatre 15. Jackimczyk K. Blunt chest trauma. Emerg Med Clin North
matic lacerations in penetrating trauma if possible. Am 1993;11:8191.

68
 3

TRAUMA IN CHILDREN
3.4 Abdominal and pelvic trauma
Scott Pearson

derangement.3 Children are generally

ESSENTIALS healthy, with few comorbidities and on
few, if any, medications. In physiological
1 Children may have significant internal injury with little evidence of external terms, they are therefore able to compen-
trauma. The abdominal viscera are less well protected by the musculature and
sate extremely well for blood loss.2
rib cage.
Early surgical involvement in treating
2 Meticulous assessment and reassessment will detect subtle abdominal injuries and children with abdominal injury is vital to
a change in physiological status due to blood loss. care in the emergency department. The
child with multiple injuries requires senior
3 Persistent tachycardia may be the only clue of intra-abdominal haemorrhage in the experienced clinicians involved in decision
child without other overt source of bleeding.
making during the resuscitation. Because
4 Unrecognised abdominal trauma is an important cause of preventable trauma such a patient invariably requires the
deaths in children. involvement of several specialties, a trauma
team approach, with clear leadership of
5 Selective non-operative management of solid visceral injuries is common but the resuscitation, is imperative.
requires institutional support and may necessitate transfer of the child to a regional
centre.
6 Pelvic fractures are a marker of severe injury, and there is a strong association with History
head, abdominal, and chest trauma.
Obtaining details of the exact mechanism of
7 The treatment of children with significant abdominal or pelvic trauma requires a injury cannot be over-emphasised. This
planned team approach and early liaison with a paediatric surgeon and paediatric
often gives a clue to the potential injury
tertiary centre.
pattern. Information can be obtained
8 CT scan is the investigation of choice in stable children with abdominal trauma in from witnesses, ambulance officers, family,
consultation with a paediatric surgeon. Some patients will be safely managed by friends, or care-givers. One member of the
careful serial abdominal examinations without the need for CT scanning. trauma team should be delegated to obtain
this information, so that the primary survey
can occur simultaneously.
Mechanisms of injury in children include
are more compliant, and the abdominal wall pedestrians struck by motor vehicles, falls,
Introduction and musculature frequently thinner and less occupants of motor vehicles involved in
Well over 90% of abdominal injuries in protective. The organs are closely packed crashes, bicycle-related injuries, contact
children are the result of blunt trauma. together and there is less padding soft sports, assaults, and abuse. Falls are the
While penetrating injuries are increasing in tissue to absorb the kinetic energy trans- most common mechanism. The events lead-
incidence in the adolescent population, this mitted by the impact.2 The upper abdominal ing to the fall, and fall height and surface are
remains an unusual phenomenon in most viscera are more at risk of injury, and rela- all pertinent information that can usually be
Australasian communities. Abdominal inju- tively minor forces can be transmitted, obtained rapidly. Information such as the
ries resulting from blunt trauma commonly resulting in a serious disrupting injury.3 aspect of the child when struck and likely
affect the solid organs, particularly the liver The bladder is not as well protected by the speed of vehicles is useful in predicting
and spleen. Overall mortality is generally bony pelvis as in the adult, increasing the injury patterns. Likewise, factors such as
<5%, but obviously this depends on injury risk of bladder injury in lower abdominal the use of restraint devices, type used, and
mechanism.1 In children with multitrauma, trauma. Gaseous distension of the stomach wearing of a bicycle helmet, where appro-
the subtle early clinical findings of intra- from air swallowing during crying or bag priate, are helpful in defining the resulting
abdominal injury may be masked by change valvemask ventilation occurs rapidly and injuries. Lap belts can be associated with
in conscious state, and chest and limb can impair ventilation. Likewise, acute gas- rib and lumbar spine fractures, and upper
injuries, and require repeated abdominal tric dilatation or a large bladder can seriously abdominal organ injuries. Handlebar injuries
examination. impede clinical assessment of the abdomen. may cause serious blunt intra-abdominal
There are unique characteristics of The very compliant body of the child is injuries. The resultant injuries to pancreas
children that predispose them to intra- capable of absorbing considerable amounts and duodenum can be subtle and delay
abdominal injuries. The rib cage does not of kinetic energy without external signs, the diagnosis. Hence the threshold for obser-
extend as far distally as in the adult, the ribs yet be associated with significant internal vation or imaging may need to be varied

69
3.4 ABDOMINAL AND PELVIC TRAUMA

accordingly in children presenting with this The use of the terms unstable and stable with minor trauma who require careful re-
mechanism of injury. is discouraged when conveying information examination. Auscultation of the abdomen
Small children are particularly at risk of regarding the childs status to colleagues. has limited usefulness. Rectal and vaginal
being unsighted and backed over in drive- They are non-specific and are defined differ- examinations are rarely indicated in the
ways by reversing vehicles, and may sustain ently by individual practitioners. It is more child with minor abdominal trauma.
major internal injuries. The recognition of useful, when relaying the circulatory status In the child with only minor injuries, the
abuse as a causal mechanism in younger of a child, to convey the actual vital signs, way the child moves around the emergency
children and infants is important in patients progression over time, and response to fluid trolley or walks can be a useful screening tool
with abdominal trauma. There may be mini- to indicate volaemic state. Other para- as to whether intra-abdominal injury exists.
mal signs of external injury, and the reported meters, such as capillary refill time, have
history may suggest a minor incompatible some limitations but can add to the
mechanism or no history of injury at all. assessment. Investigations
The emergency physician needs to maintain In children with less severe trauma, the
an index of suspicion in the infant who technique of abdominal examination is Laboratory
presents in shock or with an altered level important to reliably exclude significant Blood should be taken for group and hold,
of consciousness (see Chapter 3.2). intra-abdominal injury clinically. Where full blood examination (FBE), liver function
Other information, such as medications, physical examination is to be relied on as tests, amylase, and blood glucose. Urine
allergies, and significant past history, should the major indicator of abdominal injury, it should be obtained for examination. Some
be obtained. should ideally be performed regularly by the studies have demonstrated that elevated
same observer. With serial examination and transaminases in combination with an ab-
vigilance to vital signs, changes are detected normal physical examination are associated
early and appropriate management imple- with intra-abdominal injury (although not a
Examination mented. The aim of the abdominal examina- specific organ injury).4,5 However, at present
Primary survey focuses on the ABCs and may tion is to elicit physical signs, such as there are no laboratory studies that can be
result in early interventions such as intuba- tenderness, rebound, guarding, or rigidity, recommended as a screening tool for intra-
tion or treatment of shock. The examination which may require evaluation by CTscanning. abdominal injury.
of the abdomen is usually delayed until the Pain from injuries and other distress all In the context of pancreatic injury, serum
secondary survey. Where endotracheal intu- add to the difficulty of abdominal assess- amylase often rises, but the initial amylase
bation has already occurred, this invariably ment. Judicious and early use of parenteral may be normal, with increasing values over
involves chemical sedation and paralysis. opiates is safe, decreases a childs distress, 3 days.6
The information obtained by palpation of and allows a more accurate clinical assess-
the abdomen in this situation is somewhat ment. Abdominal examination must be per- Focused assessment by
limited, and these children often require formed by gentle palpation with warm sonography for trauma (FAST)
abdominal computerised tomography (CT) hands. There should be a brief but careful Ultrasound has been promoted as a quick
scanning, provided their vital signs are satis- visual assessment of the abdominal wall and effective initial screening tool in the
factory and not deteriorating. for the distribution of any penetrating evaluation of the abdomen in the trauma-
Vital signs are essential, particularly the wounds, bruising, or marks (e.g. seat belt tised child.710 Ultrasound in this setting is
respiratory rate, pulse rate, non-invasive or handlebar). The presence of these war- aimed at the detection of free fluid in the
blood pressure, and oxygen saturations. rants a prolonged observation period by peritoneal cavity. No attempt is made during
Attention should be given to the childs admission, even for the child with no other this rapid assessment to identify solid vis-
peripheral perfusion to detect early hypovo- positive findings. ceral injury. This examination takes between
laemia and treat prior to the occurrence of Gastric dilatation may greatly impede exam- 3 and 5 minutes, and can be achieved at the
hypotension. The blood pressure needs to ination as well as impair ventilation. Insertion bedside in the resuscitation area. While
be measured with an appropriately sized of a nasogastric tube may facilitate examina- there is much supportive literature for this
cuff for the childs habitus. Automated blood tion by decompressing the stomach, while also modality,79 there is also some cautionary
pressure machines, while useful in freeing reducing the risk of aspiration and improving research.10 Detractors cite concerns of low
staff to attend to other aspects of care, diaphragmatic excursion. Aspiration of blood sensitivities in the detection of free fluid;
can be unreliable when hypotension exists from the nasogastric tube usually signifies however, several studies have documented
and can result in delays in obtaining these the presence of a significant intra-abdominal sensitivities from 89 to 100%. Providing
recordings. Single vital sign recordings are injury. A persistently distended abdomen after the limitations of FAST are appreciated, it
of limited use, but it is the progression of nasogastric tube insertion may signify intra- remains an invaluable tool, particularly in
recordings and the monitoring of perfusion abdominal bleeding. the assessment of the multiply injured child.
that more accurately reflect volaemic state. Information from clinical examination can Identification of free fluid in the stable child
In the critically ill child, pulse and blood usually be obtained by gentle palpation with normal vital signs should be followed
pressure should be measured at 35-minute with occasional use of percussion tender- by CT examination. The detection of free
intervals. ness. This technique is acceptable in children fluid in the child with deteriorating vital

70
3.4 ABDOMINAL AND PELVIC TRAUMA
3

TRAUMA IN CHILDREN
signs supports the decision for operative simultaneously. Oxygen should be adminis-
treatment. FAST ultrasound is operator- tered and vital signs monitored regularly. Surgical issues
dependent and should be performed only Vascular access is obtained early and Selective non-operative management of
by clinicians with appropriate training and appropriate blood samples for blood cross- solid visceral injury in children is now well
credentialing. matching, haematology, and biochemistry. established. It is clear that bleeding from
Where there is delay in obtaining venous an injured spleen, liver, or kidney is generally
CT scan access, intraosseous access remains an self-limiting.11 Success rates in excess of
This is the investigation of choice in stable effective method of resuscitation. Trauma 90% with non-operative care mean that
children with abdominal trauma in con- series films of chest, pelvis, and lateral cer- operative management is an exceptional
sultation with a paediatric surgeon. With vical spine should be obtained, when indi- event at many institutions. Pancreatic inju-
the evolution and common acceptance of cated, during the resuscitation. Views of ries, however, usually result in a higher inci-
non-operative management of blunt abdom- thoracic and lumbar spine may also be dence of operative intervention. Operative
inal injuries, diagnostic imaging is an essen- required if indicated on mechanism or management is the rule for hollow viscus
tial component of the assessment process clinical findings. In the severely injured injuries. Some injuries, such as duodenal
of the injured child. CT has emerged as child, CT scanning may provide much of this haematoma without evidence of perfora-
the gold standard.11 It identifies free intra- information. tion, may be managed without surgery.
abdominal fluid, solid visceral injury and The abdominal examination is usually The decision about operative versus non-
the injury configuration, and loops of reserved until the secondary survey. Atten- operative management is made by the sur-
bowel, and demonstrates free peritoneal tion should be exercised to ensure that the geon who will have ongoing care of the
gas. The retroperitoneum is well visualised. child is warm to prevent the development child. This decision is strongly influenced
CT examination should be performed after of hypothermia during resuscitation. by clear details regarding progression
the administration of intravenous contrast. Fluid therapy should begin with of vital signs, response to fluid therapy,
This enables better visualisation, although 20 mL kg1 of warmed crystalloid (normal and associated injuries. A non-operative
in situations of renal hypoperfusion renal fail- saline) and repeated if required. If further approach must take place only in an insti-
ure can be precipitated, and allergy to the fluid therapy is required after two crystalloid tution with an available surgeon with
contrast is a rare but potentially serious boluses, blood should be used in volumes of a commitment to the injured child and
problem. The addition of oral contrast may 10 mL kg1. The rapidity of blood loss may dedicated paediatric intensive care or high-
increase diagnostic accuracy in detecting rarely dictate the use of O-negative or dependency facility.13 This may necessitate
duodenal or pancreatic injury but this is con- group-specific blood rather than waiting the transfer of the child to a regional centre.
troversial and institutional practice may vary. for full cross-matched blood.
CT scanning carries a radiation risk to the Early consideration of gastric decompres-
child. This needs to be considered when order- sion with a nasogastric tube assists abdomi-
ing such an investigation. CT scanning should nal assessment and aids ventilation. The Penetrating trauma
be reserved for those patients in whom there is insertion of a urinary catheter may be neces- Penetrating abdominal injury in a child usu-
a high index of suspicion of intra-abdominal sary, depending on the requirement to aid ally requires exploration by laparoscopy or
injury. Literature is now emerging that haemodynamic monitoring of fluid resusci- laparotomy. Because the abdominal wall is
addresses the use of CTscanning in children.12 tation and to detect haematuria. Perineal often thinner than in adults, penetration
haematoma and blood at the external into the peritoneal cavity occurs more read-
Formal ultrasound urethral meatus are contraindications to ily. A careful assessment, including a log roll
A detailed upper and lower abdominal ultra- routine catheter insertion and mandate for back examination, to exclude other inju-
sound examination takes longer to perform retrograde urethrogram or cystogram to ries, is necessary. Gunshot wounds should be
and is more observer-dependent than CT assess the urethral integrity. In this situa- explored in theatre. The approach to initial
examination. Abdominal distension caused tion, surgical opinion should be sought. resuscitation is identical to that for blunt
by ileus and luminal distension, and the pres- FAST ultrasound, when available, can be trauma. An erect chest X-ray or lateral decu-
ence of abdominal tenderness in the child, can performed, often within the first 20 minutes bitus film helps to identify the presence of
make ultrasound examination more difficult. of the patients arrival, and may or may free air.
Hence there is little role for ultrasound in not show evidence of free fluid. Ongoing
the definitive diagnosis of abdominal trauma, management is usually dictated by the
except when CT scan is unavailable. haemodynamic response of the child to
Pelvic fractures
fluid resuscitation. CT examination is ideal
but may not be possible in a very small A child who sustains a fractured pelvis has
number of exsanguinating children with been exposed to severe trauma. These are
General management deteriorating vital signs despite fluid resus- uncommon injuries in children, occurring
The assessment begins with primary citation. In this situation, early surgical at half the frequency as in adults.14 There
survey and any life-saving interventions, consultation regarding urgent laparotomy are several major differences in the bony pel-
while historical details are obtained is required. vis between the child and adolescent or the

71
3.4 ABDOMINAL AND PELVIC TRAUMA

adult. There is greater elasticity in the sacro- other injuries must be undertaken. The
iliac joints and pubic symphysis, and plastic- management of those other injuries usu- Disposition
ity of the bone, in the paediatric pelvis, ally takes priority over the pelvic fracture Almost all children with significant abdomi-
therefore greater amounts of kinetic energy management. nal or pelvic injury require admission from
must be involved to cause fracture. Avulsion Bladder injury, while more common than the emergency department. The nature
fractures occur in children and adolescents in the adult, is an infrequent association. and severity of the injuries and intended
because cartilage is weaker than bone. This In a review of 166 children with pelvic frac- management determine the most appropri-
occurs at the physis. Greater laxity of the tures, there was one urethral disruption and ate location for this to occur. Surgical collea-
joints in the paediatric pelvis means that sin- two bladder contusions.15 There is a strong gues involved in the ongoing care of the
gle fractures occur more commonly, as association of these injuries with straddle- child should have input into this decision.
opposed to the adult pelvis, where there is type mechanism. Children commonly receive Younger children who have experienced a
the double-break concept. Fractures occur- fall astride injuries related to playground significant mechanism of injury (e.g. fall
ring through epiphyseal and apophyseal equipment or while riding bicycles. from great height, high-velocity motor vehi-
growth centres may result in growth arrest, In Silbers series, 97% of children were cle crashes, pedestrian hit, or run over by
leg length discrepancy, and deformity. treated non-operatively.15 The majority of motor vehicle) but who are apparently
Children also have increased capacity for these injuries (63%) were type 3 fractures. injury-free or have only minor injuries should
remodelling.15 The remainder were type 2 (17%) and type also be admitted for observation (for 1224
The common mechanisms for pelvic frac- 4 fractures (17%). In this series, six children hours). Abdominal injuries in young children
ture are motor vehicle accidents and motor died; all these deaths were due to associated may initially have minimal or subtle signs,
vehicle-pedestrian collisions, followed by injuries. The mortality rate from paediatric which become more apparent after observa-
falls.15 pelvic fractures is consistently less than tion and serial examination. Because of the
There are several classification systems 6% in recent studies.14 In a study comparing plasticity of the paediatric skeleton, signifi-
for pelvic fractures. None is ideal. Torode paediatric and adult fractures, the mortality cant internal derangement can occur with-
and Zeig described four groups of pelvic rate for children was 5.7% compared with out obvious external evidence of trauma.
fracture but failed to include isolated 17.5 % in the adult group. Vascular injury
acetabular fractures.16 This has been modi- and exsanguination in children is rare, in
fied by Silber et al,15 whose classification contrast to in adults. This is thought to be Controversies
by mechanism of injury and description is due to the greater skeletal flexibility and
useful (Table 3.4.1). the greater ability of paediatric arteries to The necessity to use double contrast
Associated injuries increase in frequency constrict after injury. for CT abdominal examinations. IV
with the increasing severity of fracture In those children in whom blood loss is sig- contrast is required, but the need for oral
type. Other skeletal injuries are common, nificant, early involvement of an orthopae- contrast is controversial.
followed by head, abdominal, and pulmo- dic surgeon and interventional radiologist Defining the haemodynamically
nary injuries. In one large series, 19% is essential to optimise management. Exter- unstable child. When in doubt, discuss
of the total group had visceral injures. nal fixation and angiography have both the child with an emergency or surgical
Therefore, when a pelvic fracture is identi- been used successfully, particularly in ado- colleague. Children can be profoundly
fied on initial X-ray, a thorough search for lescent children. hypovolaemic with normal vital signs or
only tachycardia. Ongoing fluid
requirements and indices of peripheral
perfusion are important indicators of
Table 3.4.1 Classification of paediatric pelvic fractures volaemic status.
Type Mechanism Description  There is debate about which subset
1 Avulsion Separation through or adjacent to an of blunt abdominal trauma patients can
apophysis be safely managed with serial
2 Lateral compression Iliac wing fractures examination and without CT scanning.
3 Anteroposterior compression (usually) Simple ring fractures:
isolated pubic rami fractures
disruption of the pubis symphysis without
disruption of the sacroiliac joint In the older child, however, it may be
isolated acetabular fractures appropriate to discharge the patient who
4 Anteroposterior compression (usually) Ring disruption fractures: is injury-free or has only minor injuries. This
fracture (or diastasis) of both anterior and should occur after several assessments while
posterior structures
pelvic fracture with an acetabular fracture in the emergency department and with
straddle fracture: bilateral superior and arrangements for follow up with a medical
inferior pubic rami fractures
practitioner within 24 hours. Parents should
Originally described by Torode and Zeig16 and modified by Silber et al.15 be instructed to return earlier should a

72
3.5 BURNS
3

TRAUMA IN CHILDREN
childs symptoms change. In general, chil- 4. Holmes JF, Sokelove PE, Brant WE. Identification of 10. Coley BD, Mutabagani KH, Martin LC, et al. Focused
children with intra-abdominal injuries after blunt trauma. abdominal sonography for trauma (FAST) in children with
dren with ongoing abdominal pain after Ann Emerg Med 2002;39:500509. blunt trauma. J Trauma 2000;48:9026.
trauma should not be discharged, regardless 5. Cotton BA, Liao JG, Burd RS. The utility of clinical and 11. Eppich WJ, Zonfrillo MR. Emergency department
laboratory data for detecting intraabdominal injury evaluation and management of blunt abdominal trauma
of negative imaging results. among children. J Trauma 2004;56:106874. in children. Curr Opin Pediatr 2007;19:2659.
6. Sjovall A, Hirsh K. Blunt abdominal trauma in children: 12. Rice HE, Frush DP, Farmer D, Waldhausen JH. APSA
Risks of nonoperative treatment. J Pediatr Surg Education Committee. Review of radiation risks from
1997;32:116974. computed tomography: essentials for the pediatric
7. Akgur FM, Aktug T, Olguner M, et al. Prospective study surgeon. J Pediatr Surg 2007;42:6037.
investigating routine usage of ultrasonography as the 13. Advanced Life Support Group. Advanced Paediatric Life
References initial diagnostic modality for the evaluation of children Support Manual. 3rd ed. London: BMJ Books; 2001.
1. Stafford PW, Blinman TA, Nance ML. Practical points in sustaining blunt abdominal trauma. J Trauma 14. Ismail N, Bellemare JF, Mollitt DL, et al. Death from pelvic
evaluation and resuscitation of the injured child. Surg 1997;42:6268. fracture: Children are different. J Pediatr Surg
Clin North Am 2002;82:273301. 8. Thourani VH, Pettitt BJ, Schmidt JA, et al. Validation of 1996;31:825.
2. Gaines BA. Intra-abdominal solid organ injury in surgeon-performed emergency abdominal 15. Silber JS, Flynn JM, Koffler KM, et al. Analysis of the cause,
children: diagnosis and treatment. J Trauma 2009;67(2): ultrasonography in pediatric trauma patients. J Pediatr classification and associated injuries of 166 consecutive
S1359. Surg 1998;33:3228. pediatric pelvic fractures. J Pediatr Surg
3. Tepas JJ. Paediatric trauma. In: Moore EE, Mattox KL, 9. Corbett SW, Andrews HG, Baker EM, et al. ED evaluation 2001;21:44650.
Feliciano DV, editors. Trauma. 4th ed. New York: McGraw- of the pediatric trauma patient by ultrasonography. Am J 16. Torode I, Zeig D. Pelvic fractures in children. J Pediatr Surg
Hill Education; 2003. p. 107598. Emerg Med 2000;18:2449. 1985;5:7684.

3.5 Burns
Peter L.J. Barnett

inflammables. Chemical and electrical burns

ESSENTIALS are uncommon. One must be alert to the pos-
sibility of burns presenting as a manifesta-
1 Burns are one of the leading causes of injury in children in Australia. tion of non-accidental injury in a young child.
2 The definitive assessment of the depth of the burn may be difficult early on, as the Several preventive strategies can help
appearance can evolve during the first 2448 hours. decrease the riskand degree of burns sustained,
especially with thermal burns. Lowering the
3 The calculated amount of fluid (burn-deficient plus maintenance) to be replaced in temperature of hot water heaters to a maxi-
24 hours is only a guide and should be adjusted according to the childs
mum of 50 C significantly increases the con-
haemodynamic response.
tact time needed to produce deep or full-
4 Children with major or complicated burns should be treated in a paediatric burns thickness burns. Flame-resistant clothing and
unit. smoke detectors in homes have saved many
lives. Spill-proof mugs, guards around wood fire
5 Consider non-accidental injury if the presentation is delayed or if the history given stoves, and child-resistant taps have all been
is inconsistent with the burn sustained.
shown to prevent burns. Further prevention
6 Electrical injuries are more commonly seen in two age groups: toddlers within the strategies will have a far bigger impact on
home setting and male adolescents involved in risk-taking behaviour. High-voltage burns than advances in burn management.
exposures have a more serious outcome, as they are more likely to be associated with
injury to internal structures.
Pathophysiology
The skin is the largest organ in the body, and
whereas late deaths are usually related to its functions include:
Introduction infection. The use of early debridement and
skin grafting has led to an increased survival
preventing heat loss to the environment,
Burns sustained by children are a common thus regulating the bodys temperature;
presentation to emergency departments rate in patients who would previously have
died because of infection.
preserving body fluids by preventing
and often cause significant distress to both water loss from the body;
the child and the parents. The mortality is Most burns are fortunately less serious,
resulting mainly from scalds. These com-
acting as a barrier to infective organisms.
increased in younger children. Deaths are gen-
erally related to flame burns, which may be monly occur in preschool-aged children Therefore children with extensive
complicated by inhalation of smoke and other due to their inquisitive nature precipitating burns have difficulty retaining fluid and
toxic gases (e.g. in house fires). Early fatalities accidents in the home. Flame burns occur in regulating temperature, and are at risk of
are related to respiratory complications, older children often experimenting with infection.

73
3.5 BURNS

The skin is composed of two main layers. Blistering does not occur immediately but causes scarring. Therefore most will require
may over the next few days. The pain and skin grafting.
Epidermis: composed of stratified
swelling generally last only a few days. All significant burns will become colo-
squamous epithelium, which is largely
The skin is erythematous and blanches nised with bacteria. Heat causes coagula-
non-viable. It acts as the barrier to
normally. The epidermis will often peel tion of tissue, which leads to oedema and
infectious agents as well as preventing
within 37 days and is completely healed non-viable skin. This will potentially become
fluid loss from the body.
by 710 days, without scarring. a rich source of nourishment for bacteria.
Dermis: contains the epithelial adnexal
Adequate debridement is required to reduce
structures, e.g. hair follicles, sweat glands,
Partial thickness the risk of infection. Infection will increase
and neural receptors for pain and pressure.
Partial-thickness burns occur when the the depth of skin damage and thus the
It also contains blood vessels, which
whole epidermis is involved and part of degree of scarring.
contribute to temperature regulation of
the dermis. The more of the dermis that is The location of the burn is also important
the body via radiant heat loss.
involved, the increased scarring potential. regarding potential scarring, contracture,
After a burn, injury to the deeper specialised and caring for the acute burn. Facial, hand,
epithelial cells prompts a change into strati- foot, and perineal burns may be difficult to
Superficial partial-thickness burns involve
fied squamous epithelium. These cells pro- dress. Hence the part of the body involved
the papillary layer of the dermis and are
liferate, gradually covering the burn with a by the burn will influence whether inpatient
characterised by erythema with blistering.
non-epithelial barrier. Therefore, if the der- or specialist care is required.
The blisters may remain intact and later
mal structures are damaged, skin grafting
burst due to an external pressure. The skin
is the only means to cover the skin. When
underlying the blister has a pink or red
these deeper layers are involved, scarring
colour and moist appearance. These burns
results and contractures may occur. History
are extremely painful, as the nerve endings
The depth of the burn will depend on the
are exposed. The deeper the burn, the slower The history is generally obtained from the
temperature of the substance in contact
the healing process. Generally, superficial parent to clarify events. Ambulance officers
with the skin, the length of time the sub-
partial-thickness burns heal in 23 weeks. may have important additional information.
stance is in contact with the skin, and the
It is important to know the substance and
extent of subsequent cooling of the burned
Deep partial-thickness burns involve the estimated temperature of the substance
skin area. Hypothermia due to cooling
reticular layer of the dermis. They may be that caused the burn (e.g. hot cup of tea,
occurs quickly in children due to their higher
less painful than their more superficial cooking oil, flame), and the duration of con-
surface area to weight ratio, compared to
counterpart, due to oedema lessening the tact with skin (e.g. was the patient clothed at
adults. Also, children have thinner skin,
exposure of the nerve endings. They are a paler the time, in what, and for how long?).
which leads to deeper burns for a given
colour with a speckled appearance due to With flame burns, was the patient trapped
contact temperature and duration.
thrombosed superficial vessels. The skin is in an enclosed space, therefore at risk of
non-blanching. It may initially be difficult inhalation problems, or was there any loss
to distinguish between full- and deep partial- of consciousness? These features may sug-
Classification thickness burns. Deep partial-thickness burns gest inhalation issues such as carbon mon-
generally heal after 36 weeks. Scarring is oxide poisoning. The historical information
Burns are generally classified into superfi-
common, and skin grafting is sometimes may determine the potential for associated
cial, partial thickness or full thickness. Previ-
necessary. injuries resulting from falls or explosions.
ous nomenclature (first, second, and third
Non-accidental injury should be consid-
degree) has been replaced to give a more
ered if the presentation is delayed or where
accurate description of the burn. In the Full thickness the history given is inconsistent with the
emergency department setting, the defini- Full-thickness burns generally occur after
burn sustained or if the burn has distinctive
tive assessment of the depth of the burn flame burns or after prolonged contact with
distribution (e.g. glove and stocking). Past
may be difficult, as the appearance can a hot surface. Other causes include hot oil,
medical history and immunisation status,
evolve during the first 2448 hours. Like- prolonged immersion, or chemical burns.
particularly for tetanus, should also be
wise, the burn is not generally uniform in They involve the epidermis and all the der-
obtained (see Chapter 1.1).
depth, and it may take time to delineate mis, including epidermal appendages. They
between superficial and deeper areas. have either a dry, hard, white, leathery
Superficial and partial-thickness burns are appearance or may be black in colour. They
the most common burns seen in children. usually have no sensation because the nerve
Examination
endings have been destroyed, and pain is
Superficial due to more superficial burns on the edge Note that children who are distressed
Superficial burns generally involve only the of the full-thickness burn. Full-thickness may require the provision of immediate
epidermal layer. These are commonly seen burns are able to heal only from skin appropriate analgesia at the outset to aid
resulting from sunburn or minor scalds. regrowth from the edge of the burn, which examination.

74
3.5 BURNS
3

TRAUMA IN CHILDREN
Primary survey circumferential burns are apparent. Compar- replacement with blood, protein, and elec-
The initial assessment of the child should ing the pulse wave forms of various digits trolyte may be necessary. Carboxyhaemoglo-
be directed to the presence of any features on the limb using a saturation oximeter bin levels should be obtained in patients
that suggest potential airway involvement. may assist this in questionable instances. with inhalation burns and extrapolated to
These include singed nasal hairs or eyebrow the time of injury. A level of >15% on arrival
hairs, oral or facial burns, coughing up carbon- Evaluation of burn area in emergency suggests significant smoke
aceous sputum, barking cough, altered The extent and depth of the burn should be inhalation. Urine output should be moni-
voice, stridor, wheeze, or respiratory distress. assessed after the patient has been stabi- tored in severe burns, both to guide ade-
Airway compromise (upper or lower) may lised. This is usually done from a body chart quacy of resuscitation and also to screen
have an insidious onset, so frequent re- (Fig. 3.5.1), which can be useful to aid docu- for presence of myoglobin.
examination of the child is vital during mentation of the burn. This chart is used Oxygen saturation monitoring and arte-
the first 1224 hours. If stridor or hoarse because the surface area involved will alter rial blood gases (when indicated) aid clinical
voice is present, this indicates upper airway depending on the age of the child and the assessment of respiratory involvement.
involvement, and early intubation is requi- parts of the body where the burn is located. A chest X-ray should also be obtained if
red prior to evolving oedema causing total A simple method, using the palmar surface inhalation burns are suspected. Inhalation
airway obstruction. Delayed intubation in of the childs hand and fingers, can also be burns will evolve over time, and the initial
this setting can be increasingly hazardous used to estimate the area of burn. This cor- film may be normal. The early appearance
due to distortion of the normal laryngeal relates to approximately 1% of the childs of X-ray changes generally indicates a more
anatomy. Scalds to the face rarely cause total surface area. The adult formula using severe pulmonary injury.
airway compromise, unless the child has the rule of nines can be used in adolescents
ingested hot liquids. older than 15 years.
The circulation status should be assessed
Management
next. Hypovolaemia resulting from third-
space fluid loss will not occur for a few hours Prehospital
after a severe burn. Therefore, if early-onset
Investigations The main aims of prehospital care are
cardiovascular instability is present, then an Patients who require intravenous resuscita- stabilising ABCs, preventing ongoing burn
alternative explanation, such as bleeding, tion should have a baseline full blood count, injury, provision of analgesia, covering the
should be sought. The peripheral perfusion electrolytes, creatinine and urea, and blood area involved, and rapid transfer to an
of limbs should be assessed where group and hold performed. In severe burns, emergency department.
The first priority in any burn is to assess
and stabilise the airway and breathing.
Adult Child
Oxygen should be administered where there
A A is suspected carbon monoxide poisoning
9% with inhalation burns. Circulation is gener-
1 ally not a problem in the first hour after a
1
major burn, and rapid transport to hospital
2 13 2 2 13 2 should occur. If transfer time is greater than
1 hour, and the burn is greater than 20%,
then intravenous fluid replacement should
11/2 11/2 11/2 11/2
9% 9% begin en route where possible.
18% front
11/2
1
11/2 21/2 21/2 The burns should be covered with water-
18% back
B B soaked sterile cloth or the newer tea tree
B B
oil soaked pads. Excessive cooling of major
burns causes hypothermia and worsens the
patients outcome. Recently sustained minor
C C C C burns should be cooled under running water
18% 18% for at least 20 minutes. This acts to minimise
the extent of the burn and also affords some
13/4 13/4 13/4 13/4
pain relief.
Analgesia is generally required early,
Relative percentages of areas affected by growth (age in years)
and a single dose of a narcotic (e.g. fentanyl
0 1 5 10 15 Adult
A: half of head 91/2 81/2 61/2 51/2 41/2 31/2
1.52 mcg kg1 intranasally) is a good choice
B: half of thigh
C: half of leg
23/4
21/2
31/4
21/2
4
23/4
41/4
3
41/2
31/4
43/4
31/2
in burns less than 20%. Greater than 20%,
Second degree and Third degree = Total percent burned
then intravenous access should be obtained
and analgesia titrated to responses as well
Fig. 3.5.1 Lund Browder chart for estimation of percentage size of burn. as administering intravenous fluids.

75
3.5 BURNS

If electrical injury has been sustained, Analgesia should be given early, during ongoing losses (urinary output, respiratory
then cardiac monitoring should occur the stabilisation of the A, B and C. Intranasal loss, etc.).
during transfer. Patients with chemical fentanyl (1.52 mcg kg1) is a good first up The calculated amount of fluid (burn defi-
burns should undergo extensive washing treatment. In severe burns, a morphine cient plus maintenance) to be replaced in 24
of the affected area before transport. infusion should be started after adequate hours is only a guide and should be adjusted
initial intravenous or intranasal analgesia according to the haemodynamic response.
Emergency department has been given. Large doses of narcotics Patients need to be maintained in a positive
The initial priority should focus on stabilisa- are sometimes needed in severe burns to fluid balance for the first 2448 hours. The
tion of airway, breathing, and circulation, control the pain. Intramuscular morphine adequacy of fluid replacement is monitored
with concurrent provision of analgesia. is now obsolete given the use of intranasal by urine output and clinical parameters of
If airway burns are suspected, then early fentanyl. perfusion. In children, 0.51 mL kg1 per
intubation should be considered. Patients A careful secondary survey should then be hour is the recommended urine output and
with obvious stridor due to upper airway undertaken, looking at the extent, depth, should be monitored by a urinary catheter
compromise require urgent intubation. and anatomical relevance of the burns. It in severe burns. A central venous catheter
Supplemental oxygen should be instituted is important to determine if there are any is generally not required in the emergency
and oxygen saturation monitored. circumferential burns to limbs and chest. department phase of management.
Other potential indications for ventilation In superficial or partial thickness burns, care- The type of fluid used varies between
in major burns include: ful monitoring of circulation or ventilatory burn specialists, and it is best to be familiar
compromise is required. In full-thickness with the preference of the local paediatric
Extensive burns (>6070%), to decrease circumferential burns, an urgent escharot- burns unit. In the shocked child, a
the patients work of breathing.
omy may be required to restore circulation 20 mL kg1 bolus of normal saline should
Full-thickness circumferential chest burns, to the limb or allow for adequate ventilation. be given. This can be repeated if necessary
which may compromise chest expansion.
A paediatric burn specialist should be con- to restore peripheral circulation. Ongoing
Severe inhalational lung injury causing sulted in this situation. replacement is generally with crystalloid in
pulmonary oedema and hypoxaemia.
The secondary survey should also include the first 24 hours, as colloid may leak
Inhalation burns generally get worse in
careful examination for any other injuries through the burnt capillaries, causing wors-
the first 1224 hours, and ventilation
requiring attention (e.g. head, neck, chest, ening oedema. After 24 hours, colloid is used
may be needed during this time. Early
limbs, pelvis, intra-abdominal). Burns to the as part of the replacement fluids in the
consultation with paediatric intensive
face should also include fluorescein staining intensive care setting. When calculating
care colleagues in these instances is
of the eyes to check for corneal involvement. the initial fluid requirements, it is important
appropriate.
to subtract the bolus fluids given from this
The fluid losses due to the burn itself do not Fluid resuscitation amount.
cause early circulatory failure, and other Fluid resuscitation should be calculated Additional fluids may be required in
contributing injuries should be sought in based on the weight of the child and the severe electrical burns causing muscle dam-
the patient with early shock. It is also impor- total surface area of the burn. Several formu- age, as myoglobin may cause renal failure
tant to note the time the patient is evalu- las are used to calculate the resuscitation secondary to renal tubular deposition, and
ated, relative to the time of the burn. fluid requirement in the first 24 hours. therefore maintaining adequate glomerular
Children who have delay (e.g. a few hours) The Parkland formula (BSA affected %
filtration is very important.
in presentation may arrive with circulatory weight (kg)
4) gives the number of millili-
compromise from skin fluid losses. tres of resuscitation fluid to be given over
Intravenous access should be placed in all the first 24 hours. Half the fluid is given in
Management of burns
children with burns body surface area (BSA) the first 8 hours and the remainder in the
(Table 3.5.1)
of >20%. Fluid resuscitation rates should subsequent 16 hours. The 24-hour period
be calculated using the time of the burn, should begin from the time of the injury. Major burns
not the time of presenting to the emergency Thus if a patient has received very little fluid These patients should be treated in a
department (see Fluid resuscitation, below). in transfer, and it is 4 hours since initial burn, specialised burns unit. Covering the burn
Peripheral venous access, preferably through then the fluid calculated should be given with a sterile dressing is required prior to
non-burnt skin, is preferred over central over the next 20 hours, and half the calcu- transfer. Specific dressing type is best
venous access for the initial resuscitation. lated fluid given in the first 4 hours. decided after discussion with the receiving
Monitoring of urinary output (via urinary In addition, maintenance fluid for the unit. Within the burn centre, patients are
catheter, weighing nappies) is important in 24-hour period should also be given. This generally dressed with topical silver sulfadi-
determining the adequacy of fluid replace- is calculated as 100 mL kg1 for 010 kg, azine (SSD) cream that should be changed
ment. A nasogastric tube should also be 50 mL kg1 for 1120 kg, and 25 mL kg1 each day. At each change, the wound should
inserted in children with severe burns, as for >20 kg. Thus a 30-kg childs main- be cleaned with warm water and debrided to
gastric dilatation can occur, leading to tenance 1000 500 250 1750 mL remove any avascular tissue (which may
respiratory compromise. over 24 hours. It is also important to monitor lead to infection). The burns are covered

76
3.5 BURNS
3

TRAUMA IN CHILDREN
the first few days. Removal of this type of dressing, it becomes pruritic, which may
Table 3.5.1 Admission criteria
for paediatric burns unit dressing is problematic. Olive oil must be require an antihistamine or cooling of the
applied to the Fixomul several hours before dressing (particularly in hot weather).
Require admission
partial-thickness burns >20% BSA the dressing can be removed, otherwise pull- Tetanus prophylaxis is important in major
full-thickness burns >510% BSA ing the dressing off leads to removal of the burns or minor burns (which are contami-
smoke inhalation or airway burn is suspected
child abuse suspected new granulation tissue. nated). Antibiotics should be used only
when a definite infection is present. Prophy-
Consider admission
burn to hands, feet, face, perineum, More extensive superficial burns lactic use of antibiotics is not recommended.
or joints Most extensive superficial burns should be Pain management for minor burns is
burns <20% BSA and other concerns
e.g. age <12 months, parents not coping, covered with Mepitel, Melolin and crepe generally achieved by the dressing itself.
etc. bandage securing this with Hyperfix. Covering the burn decreases the pain sub-
comorbidity
other significant injuries Mepitel is a low adherent wound contact stantially. Generally, paracetamol with or
dressing made of silicone gel bound to a without codeine should be sufficient during
flexible polyamide net. The dressing is left the first 24 hours.
with a non-stick dressing over the SSD cream intact until the patient is reviewed after Patients with burns involving hands, feet,
(e.g. Melolin), and then wrapped in crepe 57 days. Repeat dressing may be required or face should be referred to a burn specialist
bandages to prevent contamination of the or the healing burn can be covered with for ongoing management.
burn. The face and perineum are generally Tegaderm or Hyperfix and be left on until
left open and covered with a water-based it falls off.
gel. SSD should not be used on the face, as Electrical burns
the patient may spread it into the eyes. Partial thickness/small full
SSD is currently only recommended for inpa- thickness burns Introduction
tient care of patients with significant burns. Use Acticoat, a silver impregnated barrier These are infrequent presentations to emer-
dressing, moistened with sterile water, cov- gency departments but have unique pro-
Minor burns ered with IntraSite Conformable, gladwrap, blems. Electrical injuries are more commonly
Patients with partial-thickness burns <20%  crepe bandage. Secure with Hyperfix. seen in two age groups: toddlers within the
or full-thickness burns <510% can often The exudate from the wound determines home setting and male adolescents involved
be managed on an outpatient basis and the number of dressing changes required. in risk-taking behaviour.
reviewed in a burns clinic. IntraSite Conformable is a soft hydrogel Young children generally sustain electri-
There are several ways to dress wounds in dressing that combines the advantage of cal burns from low-voltage (<1000 V) or
the emergency department. Burns with IntraSite gel with a non-woven dressing. It household currents (240 V). These may be
intact blisters, unless over a joint surface creates a moist wound environment for the due to frayed electrical cords or children
or extremely large, should be left intact continued release of silver from the Acticoat. inserting metal objects into power sockets.
initially. The intact skin acts as a barrier to Patients should be reviewed between 3 and Mouth burns may occur when small children
bacteria, and the skin underneath can heal. 7 days for a change of dressing and to assess chew on power cords. In most states in
After 710 days, the intact blisters should be if skin grafting is needed. Australia, safety switches are installed in
deroofed and the need for grafting assessed. Superficial burns on the face should be all new houses, cutting the current when
If unclear at this stage, then they should be managed non-dressed, and cleaned two or overloaded, thus preventing many severe
redressed and assessed in a further 7 days. three times a day with warm water. Solugel electrical burns. These low-voltage expo-
or vaseline can be applied to the face or it sures rarely result in significant internal
Superficial burns can be left completely non-dressed. As a injury, and most children are asymptomatic
Small superficial burns burn on the face starts to dry up, application apart from distress from the cutaneous burn.
With these small burns, a clear plastic dress- of mild lanolin ointment/cream can be used High-voltage (>1000 V) injuries are seen
ing (e.g. Tegaderm) can be placed over the to aid in healing by softening the skin. The most often in adolescent males as a conse-
burn to provide protection and be left on burn should also be protected from the sun, quence of risk-taking behaviour (e.g. climb-
until it also falls off. The disadvantage of this as sunburn of an already burnt area causes ing electrical poles, train surfing). These
type of dressing is that fluid from the burn increased pigmentation to the skin. high-voltage exposures have a more serious
tends to collect under the dressing, is a rich Superficial burns rarely become infected, outcome, as they are more likely to be
source of food for bacteria, and can lead to but infection should be suspected if the associated with injury to internal structures.
infection. Tegaderm is better applied after a patient: has unexplained fever, or has evol-
few days, when the exudate from the burn ving pain, redness, and tenderness. Foul dis- Clinical effects
has ceased. A more porous adherent dress- charge from the burn does not always Electrical currents preferentially flow along
ing, such as Fixomul or Hyperfix is better indicate that infection is present. In this low-resistance tissues such as blood vessels,
when exudate is going to occur, as it allows case, the dressing should be changed earlier nerves, and muscles, rather than the skin,
some egress of fluid to occur. A covering and the burn inspected, as antibiotics may causing internal injury particularly if extre-
dressing of gauze and crepe is needed for not be indicated. As the skin heals under a mities are involved. There is generally an

77
3.5 BURNS

entrance and exit burn in non-water-related the child is symptomatic (e.g. chest pain or coagulation of the skin, which seems to limit
current injuries, with increased tissue impaired conscious state). A search for an the depth of penetration. Alkali burns
damage at these sites. Wet or moist skin entrance and exit wound should occur to cause liquefaction and thus result in a
increases current flow dramatically by determine the potential for deeper burns. deeper injury. Caustic chemicals tend to give
decreasing the tissue resistance. Analgesia should be provided for the burn deeper burns than thermal injury, as there
Clinical manifestations vary according to or muscle pain. Potential complications and is generally a long duration of contact.
the voltage exposure and may range from associated injuries are treated on their merit. Oedema tends to occur more quickly in
trivial to cardiac arrest, as follows. chemical burns, which may cause a deeper
Specific issues burn to appear more superficial.
Skin Burns
Entry and exit burns: well-demarcated All electrical burns warrant review by a burn Treatment
pale areas with charred centre. specialist. Copious irrigation of the burn is the main-
Arc burns: heat produced can cause stay of treatment. Sufficient analgesia and
extensive tissue injury. Fluids topical anaesthesia is required to achieve
Flame burns: due to ignition of clothing. Fluid requirements for significant electrical this in a child. This can be done simply with
burns are underestimated using the Park- water. Flushing of the burn should continue
Cardiac land formula, as most of the damage is for at least 1015 minutes and sometimes
Arrhythmias: internal. Therefore fluid requirements are longer. Determination of wound pH via lit-
significantly more than estimated, and one mus paper may guide the duration of irriga-
Low voltage: sinus tachycardia, atrial tion. If chemicals are introduced into the eye,
should aim to maintain a urine output of
fibrillation, ventricular fibrillation.
2 mL kg1 per hour. then irrigation should continue until pH is
Myocardial injury is uncommon.
neutral. This usually requires topical anaes-
High voltage: asystole.
thetic to the eye prior to flushing with saline.
Myoglobinuria
Muscle involvement leads to myoglobinuria, A Morgan lens is a good method of flushing
Muscular eyes, as this requires less co-operation from
which may cause renal failure. Adequate
Prolonged tetany of musculature: the patient. Fluorescein staining of the eye
apnoea, fractures and dislocations. fluid resuscitation and alkalinisation may
help prevent renal failure. should then determine the extent of the
Muscle necrosis and rhabdomyolysis. burn. Chemical burns to the eye require
Compartment injury urgent ophthalmological referral. The treat-
Neurological ment of a dermal chemical burn after decon-
Compartment syndrome may also occur due
Acute: altered mental state; seizures; tamination should be the same as for any
headache; speech, motor, or sensory to oedema and burn of the affected muscles.
Fasciotomy may be necessary, with debride- other burn.
disturbances. Some chemicals may cause systemic toxic-
ment of non-viable muscle.
Delayed: spinal cord injury, memory and ity from absorption through the burned skin,
mood disturbance. and these should be managed accordingly.
Disposition
Renal The following are general guidelines for
Renal failure secondary to myoglobinuria. admission and discharge after burns.
Further reading
Admission Klein GL, Herndon DN. Burns. Pediatr Rev 2004;25
Other (12):4116.
Eyes: cataracts. Any high-voltage injury. Holland AJA. Pediatric burns: the forgotten trauma of
childhood. J Can Chir 2006;49(4):2727.
Gastrointestinal tract: ulceration, Any child with evidence of cardiac or Sheridan R. Outpatient burn care in the
perforation. neurological abnormality. emergency department. Pediatr Emerg Care 2005;21
(7):44956.
Trauma following associated falls. Hight DW, Bakalar HR, Lloyd JR. Inflicted bums
Discharge in children: Recognition and treatment. JAMA
1979;242:51720.
Asymptomatic child with low-voltage Dunn K, Edwards-Jones V. The role of Acticoat with
Management injury with normal ECG. nanocrystalline silver in the management of burns. Burns
Assessment of the childs A, B and C is the 2004;30(suppl 1):S1S9.
Smith ML. Pediatric burns: Management of thermal, electrical,
initial priority with electrical exposure, fol- and chemical burns and burn-like dermatologic conditions.
lowed by examining for skin burns and Pediatr Ann 2000;29(6):36783.
potential internal injuries. A baseline 12-
Chemical burns Patel PP, Vasquez SA, Granick MS, Rhee ST. Topical
antimicrobials in pediatric burn wound management.
lead ECG should be done (unless a trivial Many chemical agents can cause burns J Craniofacial Surg 2008;19(4):91321.
Walker AR. Emergency department management of house fire
exposure), and cardiac monitoring only through accidental exposure. They are gen- burns and carbon monoxide poisoning in children. Curr
continued if the initial ECG is abnormal or erally either acid or alkali. Acid burns cause Opin Pediatr 1996;8:23942.

78
 4

SECTION
WOUND
MANAGEMENT
Section editor Ian Everitt

4.1 Wound management 79

4.1 Wound management

Ed Oakley

In children this often requires sedation in

ESSENTIALS addition to adequate local anaesthesia
and analgesia. Universal precautions should
1 The goals of wound management are to avoid infection, minimise discomfort, always be followed when assessing or man-
facilitate healing and minimise scar formation. The care of the patient as a whole
aging any wound. Gloves (preferably sterile),
should be the first management priority.
drapes and eye protection are mandatory.
2 In children, management often requires sedation, adequate local anaesthesia and
analgesia. Sedation should only be undertaken by personnel experienced in its use,
Anatomy of the skin
and able to manage the complications of airway compromise, oxygen desaturation
Skin is composed of two layers: dermis and
and respiratory depression.
epidermis. The epidermis acts as a protective
3 The comprehension level and the co-operation gained from the child influence layer for the dermis, preventing infection
wound examination and the information gained. Distraction techniques, adequate and desiccation. It is avascular and relies
topical anaesthesia and appropriate use of sedation can all aid in wound assessment on diffusion of nutrients from the dermis.
in children. In the child less than 5 years old, observation of posture, symmetry and The dermis is rich in collagen and thus pro-
general movement is required. vides most of the tensile strength of the skin.
It has a rich network of nutrient vessels and
4 Examination of function, sensation and circulation distal to the wound is best capillaries. The subcutaneous fat is com-
performed prior to exploration of the wound and prior to regional anaesthesia.
posed of loose connective and adipose
5 If presence of a foreign body is suspected, radiological investigation is advised. tissues.

6 Surgical debridement of non-viable tissue is vital to prevent wound infection or

delayed wound healing. Pathophysiology of wound
healing
7 Tissue adhesives are for external use only and should not be placed within wounds The stages of wound healing are coagula-
or used on mucous membranes.
tion, inflammation, proliferation and matu-
8 In general, sutures are removed earlier in children than in adults. ration. Wound healing is a sequential
process that begins immediately after tissue
9 Non-accidental injury should be considered, especially when the history and injury injury. Coagulation is initiated by platelet
are inconsistent.
aggregation then by fibrin clot formation.
This supplies haemostasis and allows accu-
mulation of neutrophils and monocytes,
wounds, and about 30% occur on the which herald the inflammatory phase. The
Introduction hands.14 The goals of management of inflammatory phase provides phagocytosis
Open wounds account for up to one third of these wounds are to avoid infection, mini- of bacteria, other foreign matter, and dead
paediatric emergency presentations; two mise discomfort, facilitate healing and mini- tissue in the wound. The macrophages
thirds of open wounds occur in boys, and mise scar formation. Meticulous attention to release factors that stimulate proliferation
40% involve a fall. The scalp and face wound care and repair should ensure the of local fibroblasts in the dermis. These pro-
account for more than 50% of all open best possible outcome and functional result. vide a collagen network and stimulate new

79
4.1 WOUND MANAGEMENT

vessel growth. This phase is characterised the wound. Crush and bite injuries character-
by pink granulation tissue and wound con- Classification of wounds istically cause significantly more surround-
traction. A warm moist environment that Lacerations ing tissue damage and thus are more likely
is supplied either by dressings or scab for- Lacerations are the most common type of to have delayed healing or infection. When
mation aids this process. Collagen synthesis wound seen in the paediatric age group.6 possible, determine the cleanliness of the
reaches its peak towards the end of the The edges are usually ragged. If the wound inflicting object, the amount of blood loss,
first week of healing. Remodelling conti- penetrates into the dermal capillaries it will the presence of a foreign-body sensation,
nues to occur for up to 12 months; thus bleed and if it extends into the subcutane- and the motor function and sensation distal
the scar will usually fade and contract over ous tissue it will gape. Lacerations can be to the affected area. The location of the
the first 2 to 3 months and the final appear- caused by tension on the skin (usually seen wound should be noted and the possibility
ance may not be obvious for up to 6 months in areas with significant subcutaneous tis- of injury to other structures explored.
post injury. sue) or by compression of the skin between The health status of the patient should
A number of factors affect the healing of a an object and bone. There is always damage be explored, especially with regard to
wound. Adequate nutrition (including vita- done to surrounding tissues and healing is chronic illnesses that may impact on wound
mins C and A, which are required for colla- therefore delayed. Compression injuries healing such as diabetes mellitus, obesity,
gen formation) is essential. Corticosteroids usually have more surrounding tissue malnutrition, chronic renal impairment,
and immunosuppressive drugs interfere with damage and thus tend to heal more slowly. cyanotic congenital heart disease, chronic
cellular proliferation and immunity, and respiratory illness, tumours, and bleeding
anticoagulants inhibit clot formation and Incised wounds disorders.3 Immunisation history should be
initial wound stabilisation. Infection inter- A sharp object such as a knife blade or glass obtained and further tetanus vaccination
feres with collagen synthesis and will delay shard makes an incised wound. The wound guided by the recommendations of the
wound healing and cause an increase in scar has margins that are clearly defined and National Health and Medical Research
tissue formation. there is little or no surrounding tissue Council (Table 4.1.1).7 Current medications
Tensile forces of the surrounding skin damage. These wounds heal faster than are important for both drug interactions
affect the healing and scar formation of a lacerations and, in general, have a lower with antibiotics that may be prescribed
wound. The most cosmetically pleasing incidence of infection. and for medications that may interfere with
outcome occurs when the long axis of the wound healing such as immunosuppres-
wound is in the direction of maximum skin Abrasions sive drugs and corticosteroids. A history of
tension along Langers Lines of skin ten- Abrasions are caused by sheering forces on allergies must be determined prior to use
sion. Wounds that have long axis perpen- the surface of the skin. The upper layers of of cleansing agents, dressings and tapes
dicular to the lines of skin tension will heal the epidermis and sometimes dermis are and prescription of medication. A history
with greater scarring, but there is significant scraped away. The depth of injury usually of latex allergy should be specifically sought.
inter-child variability. Dynamic skin tension varies throughout the wound. If the epider- In wounds that require management under
caused by joint movement also impairs mis alone is involved there is no bleeding, general anaesthesia or sedation a history
wound healing and causes increased scar but a transudation of fluid. If the dermis of when the child last ate or drank is impor-
formation, and immobilisation of joints is involved the wound will bleed and there tant. Non-accidental injury should be consid-
while the laceration heals will minimise is said to be an increased incidence of infec- ered, especially when the history and injury
this effect. tion and foreign body retention. are inconsistent.

Examination
Wound infection Evaluation of the patient Once assessment and management of more
Wound infection is relatively uncommon, serious injuries has occurred, the patient
with a laceration
occurring in about 5% of wounds presenting should be assessed for the current severity
to emergency departments (EDs). In general, The care of the patient as a whole should of any chronic illness, and appropriate man-
a wound in a child is less likely to become be the first management priority. The air- agement initiated.
infected than a similar wound in an adult. way, breathing and circulation should be The co-operation able to be gained and
Identified risk factors for infection include assessed and treated as appropriate and a comprehension level of the child influence
severe wound contamination, inadequate thorough secondary survey undertaken in wound examination and the information
wound cleansing, inadequate debridement most patients to exclude or allow manage- gained. Distraction techniques, adequate
of dead tissue (especially in crush injuries), ment of serious injuries as well as detecting topical anaesthesia and appropriate use of
use of subcutaneous sutures, larger lacera- other minor injuries. sedation can all aid in wound assessment.
tion (>5 cm) and site of injury. Specific sites A calm, unhurried, friendly approach, involv-
identified as infection risks include axillae, History ing the parents, will maximise the chances of
perineum or groin, and feet. In general, limb The mechanism of trauma (cut, crush, fall, co-operation. Examination of the wound
wounds are at increased risk compared to bite, burn) and the time of injury are impor- should be done with optimal lighting and
head and neck wounds.3,5,6 tant as they may alter the management of with bleeding minimised. Examination of

80
4.1 WOUND MANAGEMENT
4

WOUND MANAGEMENT
Table 4.1.1 Tetanus prophylaxis in wound management the presence of the foreign body and provide
a guide to its depth and location in the
History of Time since Type of wound DTPa, DTPa- Tetanus
tetanus last dose combinations, immunoglobulin*
wound.911 Other investigations should be
vaccination dT, dTpa, as determined by the findings of possible
appropriate
injuries to adjacent structures, such as bony

3 doses <5 years All wounds NO NO X-rays for fractures.

3 doses 510 years Clean, minor NO NO
wounds

3 doses 510 years All other wounds YES NO Treatment of wounds

3 doses >10 years All wounds YES NO
Wound anaesthesia
<3 doses or Clean, minor YES NO Analgesia and sedation are discussed in
uncertain wounds
more detail in Section 20. Anaesthesia is
<3 doses or All other wounds YES YES required to adequately examine and then
uncertain
treat most wounds. Often, in children, anal-
*Recommended dose for TIG is 250 IU to be given as soon as possible after the injury. If >24 hours has elapsed since gesia and sedation will also be necessary,
the injury 500 IU should be given.
depending on the location of the wound,
A combination vaccine should be used in order to boost community protection against pertussis:
< 8 years old DTPa-IPV (Infanrix-IPV) the involvement of underlying structures,
> 8 years old dTpa (Boostrix)
and the age and anxiety of the child.
Can use a diphtheria/tetanus toxoid vaccine (ADT) if pertussis vaccination is contraindicated.
Please note that CDT and tetanus toxoid vaccine are no longer available. The options for anaesthesia include topical
DTPa, acellular Diphtheria, Tetanus, Pertussis vaccine; dT diphtheria tetanus; dTpa booster diphtheria, Tetanus,
anaesthesia, local infiltration, regional ana-
pertussis vaccine.
esthesia, dissociative anaesthesia, or general
anaesthesia.
function, sensation and circulation distal to be used to examine for sweat beads or the Topical anaesthetics include ALA
the wound is best performed prior to explo- cleaned body of a pen can be run over the (adrenaline, lidocaine and amethocaine
ration of the wound and prior to regional fingers, with less resistance in the dener- (tetracaine)) commonly known as LET (lido-
anaesthesia.810 vated, thus dry, segment. Arterial circulation caine, epinephrine and tetracaine) in North
Functional assessment requires the move- is assessed by palpation of peripheral pulses, America, or EMLA cream (eutectic mixture
ment of all joints distal to the wound. In an capillary return distal to the injury, and skin of local anaesthetics) manufactured by
older child each joint is examined individu- colour and temperature. AstraZeneca. ALA is highly effective on
ally on command and the strength docu- Assessment of the wound should include facial and head wounds but less so on limb
mented. In the child less than 5 years old, site, size, depth, nature of the edges, cleanli- wounds. It has replaced TAC (tetracaine,
observation of posture, symmetry and gen- ness, and presence of foreign bodies. The adrenaline and cocaine) in most institutions.
eral movement is required. In wounds to wound should be explored to determine Due to the vasoconstricting properties of
the flexor tendons of the hand, close atten- the depth and involvement of any underly- adrenaline (epinephrine) these anaesthetics
tion should be paid to the resting position ing tissues including vessels, nerves, ten- should not be used in areas of end arteries
of the fingers (partial flexion). The finding dons, ligaments, muscles, joints, bones and (finger tips, nose, lips, ears, genitalia). EMLA
of extension of one finger at rest and the specialised tissues (especially ducts and has been shown to be safe and effective
failure of the finger to flex at play or after glands). Bones adjacent to the wound should when applied to limb wounds. Topical
application of a noxious stimulus confirms be palpated for deformity or crepitus and the anaesthetics should be applied in the
the tendon injury. wound searched for foreign bodies (including wound either as a liquid dripped onto a
Injury to nerves is classically assessed with the sound of glass on the metal forceps). pledget of cotton wool placed into the wound
two-point discrimination and this should be This assessment and exploration should or as a methylcellulose gel. The wound is then
possible in older children. Using a paperclip take place after appropriate anaesthesia of covered with an occlusive impermeable
bent so that its ends are separated 48 mm the wound and any required sedation. dressing and adequate anaesthesia is usually
is useful in this process. In upper limb inju- obtained within 30 minutes.1217
ries formal assessment of the median, ulna Investigation Local infiltration is the classical method of
and radial nerves is required. In children less If presence of a foreign body is expected anaesthetising a wound. The anaesthetic is
than 5 years old this approach needs to be radiological investigation is advised. In injected into the wound margins. Pain of
modified. A noxious stimulus applied to wounds caused by glass, all but superficial injection can be minimised by using warmed
the fingers will illicit sensation but risks wounds should be investigated with plain anaesthetic, buffering the drug with sodium
losing patient confidence. Another method soft tissue X-ray of the region to exclude a bicarbonate (mix 10 mL of 1% lidocaine
of determining intact innervation is to look glass foreign body. Most glass foreign bodies with 1 mL of 8.4% sodium bicarbonate),
for sweating of the fingers. Since autonomic more than 23 mm in size should be visible. infiltrating slowly, using the lowest concen-
response includes sweating, denervated fin- If a radiolucent foreign body is suspected, tration possible, and using needles sized
gers do not sweat. An ophthalmoscope can ultrasound can be useful to both confirm 25 gauge or smaller. The most commonly

81
4.1 WOUND MANAGEMENT

used local anaesthetic is lidocaine 1 or 2% be undertaken in a manner and with a sub-

Table 4.1.2 Indications for antibiotic
with or without adrenaline (epinephrine) stance that provides adequate antisepsis prophylaxis in wounds
1:100 000. The onset of action is rapid, with without tissue damage or impairing wound
Wound characteristics
duration of action of 30 minutes to 1 hour. defence mechanisms. A solution such as
High risk anatomic site (hands, forefoot, groin,
Addition of adrenaline (epinephrine) is use- aqueous povidone-iodine or aqueous chlor- axilla)
ful to prolong the duration of action and hexidene applied with gauze or cotton Devitalised tissue
Extensive surrounding soft tissue injury
help minimise bleeding; however, adrenaline wool should be used. Care should be taken Stellate lacerations
(epinephrine) should be avoided in regions to minimise the amount of cleanser to pen- Contaminated with body fluids or organic
matter or dirt
of end arteries (fingers, nose, lips, ears, geni- etrate the wound to minimise damage to Large lacerations (> 5 cm)
talia), and its use may increase the risk of wound defences increasing the risk of Closure delayed (> 12 hours)

infection. The safe dose of plain lidocaine infection. High risk for endocarditis
is 3 mg kg1 or 6 mg kg1 for lidocaine Surgical debridement of crushed or non- Prosthetic heart valves
mixed with adrenaline (epinephrine).3 viable tissue is vital to prevent wound infec- Patent ductus arteriosis
Structural heart disease
Regional anaesthesia is useful for facial, tion or delayed wound healing. However, Tetralogy of Fallot
hand and foot lacerations, where nerves as little tissue should be debrided as possi- Ventricular septal defects
Coarctation of the aorta
are readily accessible near bony landmarks. ble. Manual removal with forceps of large Damaged heart valves
A regional nerve block involves anaesthetis- particles of foreign material should also be
Immunocompromised children
ing the nerve or nerves that supply a specific meticulously undertaken. When a heavily
anatomic region. Regional anaesthesia is contaminated wound contains specialised Prior history of endocarditis

especially useful for large lacerations and tissues such as tendons or nerves, consulta- Intravenous drug use
lacerations where local infiltration causes tion is recommended.
distortion of tissue anatomy. Regional Once the wound is adequately anaesthe-
anaesthesia is especially useful for anaes- tised it should be thoroughly cleaned. Irriga- as soon as possible. The initial dose should
thetising digits. Lidocaine or bupivacaine tion is the method of choice for removing be given intravenously and be relatively
hydrochloride 0.5%, which has duration of dirt and bacteria from wounds. In hospital, large to provide rapid reliable high tissue
action of 3 to 6 hours, are the most com- saline (0.9%) is the irrigation solution of concentrations. The first dose should be
monly used agents. The safe dose of bupiva- choice, as it causes no tissue damage, but given before wound closure to ensure an
caine is 2 mg kg1. tap water can be used.20 The ability of irriga- effective concentration of antibiotic in the
Sedation is often required when treating tion to decontaminate a wound is directly wound tissue fluid at the time of wound clo-
lacerations in children. Options for sedation related to pressure of the irrigating stream, sure. When choosing an antibiotic the likely
include benzodiazepines such as midazo- the size of the particles to be removed, and causative organisms should be borne in
lam or diazepam, fentanyl, nitrous oxide, the volume of irrigant. At least 100200 mL mind: the organisms contaminating the
ketamine, or propofol. Sedation should only per 2 cm of laceration are required. The fluid wound and the commensal organisms found
be undertaken by personnel experienced in should be injected from a 3060 mL syringe in that region of the body. In general, bites
its use and able to manage the complications via an 18 to 20 gauge cannula. Higher pres- and wounds in regions with high bacterial
of airway compromise, oxygen desaturation sures should be avoided as they may cause counts (hands, feet, groin) should be treated
and respiratory depression. Adequate equip- tissue damage and increase the incidence with antibiotics to cover Staphylococcus epi-
ment to deal with these complications of wound infection.21,22 The volume and dermidis, S. aureus and Streptococcus sp.
should also be available. Some form of physi- pressure of irrigation should be modified The likelihood of anaerobic bacteria needs
cal restraint may also be necessary to prevent as necessary according to the location and to be considered. Specific circumstances
excessive movement during repair; however, cause of the wound. High-pressure irrigation also need to be borne in mind. Patients at
the aim must be to provide adequate analge- does not enhance the dissemination of bac- risk of endocarditis should have all wounds
sia and anxiolysis.13,18 teria into soft tissue wounds, but excessive treated with antibiotics to cover S. aureus
use can cause local tissue oedema enhanc- and S. epidermidis. Ampicillin/amoxicillin
Wound preparation and cleansing ing risk of infection. Use of a device to mini- is the currently recommended drug in
Hair near the wound should only be mise splashing of the irrigant is desirable Australia. However, in communities where
removed if it interferes with the meticulous and wearing of gloves, goggles and gown the incidence of penicillin resistance is high
closure of the wound. If hair removal is mandatory.21, 22 a cephalosporin and an aminoglycoside are
desired the hair should be clipped, not recommended.
shaved, as shaving disrupts hair follicles Antibiotic prophylaxis Wounds associated with fractures, tendon
and increases the incidence of wound The use of prophylactic antibiotics in or muscle involvement should be considered
infection.19 Eyebrow hair should not be wound care is controversial. Decontamina- for prophylaxis, as should large wounds,
removed because this may lead to abnormal tion with appropriate irrigation techniques wounds with significant devitalised tissue
or delayed regrowth. is more beneficial than the use of prophy- such as crush injuries and stellate lacera-
The surrounding skin and wound edges lactic antibiotics.2,9,23,24 When indicated tions. Wounds contaminated with faeces
should be thoroughly cleaned. This should (Table 4.1.2), antibiotics should be given should be treated with coverage of coliforms

82
4.1 WOUND MANAGEMENT
4

WOUND MANAGEMENT
and anaerobic bacteria. Wounds in children
Table 4.1.3 Characteristics of common suture materials
with a compromised immune system should
all be considered for treatment. Wounds Suture Ease of Tensile Degradation Tissue Infection
material handling strength (d)* reactivity potential
with closure delayed more than 12 hours
should also be considered high risk for Non-absorbable
infection. Treatment should be for 3 to 5 Nylon Average Good Low Very low
(EthilonW,
days with a penicillinase-resistant antibiotic DafilonW)
such as a first generation cephalosporin or Polypropylene Poor Very good Very low Low
(ProleneW)
amoxicillin-clavulanic acid, with consider- Silk Good Poor High High
ation to addition of metronidazole.25
Absorbable
Surgical gut Poor Average 47 High High
(fast absorbing
Polyglactin Average Good 710 Low Low
Wound closure (Vicryl
RapideW)
The aim of wound closure is to reduce Chromic gut Average Average 1014 High High
discomfort, aid healing and produce the best Polyglactin Average Good 1015 Low Low
(VicrylW)
cosmetic result possible.26 The technique Polyglycolic Good Good 2530 Low Low
chosen for wound closure depends on the acid (DexonW)
Polydioxanone Average Very good 2530 Very low Low
type of wound. Most wounds in children (PDS)
can be managed with primary closure, as
*Time to loss of 50% of tensile strength.
the risk of infection is relatively low. Infected,
heavily contaminated wounds and wounds
resulting from high-energy projectiles are ovine or bovine intestines. The collagen is membranes and subcutaneous tissue.
best managed by delayed primary closure, then treated to strengthen the material Heaviest sutures such as 30 should be used
with initial cleansing and packing then and increase resistance to tissue degra- on thick skin (such as the sole of the foot)
closure 3 to 5 days later, once the risk of infec- dation (plain gut). Coating with chromium or over large joints. Small sutures such as
tion has decreased. Wounds with delayed trioxide provides more resistance to absorp- 60 should be used on tissues with light
presentation (>24 hours), or those contami- tion (chromic gut). These suture materials tensions, such as facial skin and subcuta-
nated with saliva or faeces should also be have a somewhat unpredictable absorption. neous tissue.27,28
considered for delayed closure. Some wounds, Synthetic absorbable sutures have
such as puncture wounds or contaminated improved strength and delayed and more Needles
wounds in areas of poor perfusion should reliable absorption characteristics. Absorb- Needles come in varying sizes and shapes
not be closed but allowed to heal by second- able sutures are used for closing deep also. Needles are describes by the arc of cur-
ary intention. Once it is decided to close the layers of a laceration and can be used for vature the needle possesses, and the shape
wound, a technique that allows apposition of skin closure especially where removing of the needle itself. The most commonly
the wound edges that is secure and accurate sutures in a young child may be difficult, used for skin closure is the circle (135 )
and holds the wound edges in apposition or where procedural sedation is required needle or the circle (180 ) needle
until the strength of the wound is sufficient to place sutures. (Fig. 4.1.1). For closure of fascial layers
should be chosen. With improved technology Non-absorbable sutures are made from circumference needles are usually used.
the options for wound closure are growing. either natural (silk, cotton, linen) or syn- Needles that have two circumferences of
Those presently available include sutures, thetic (nylon, DacronW) fibres. They can also curvature (compound needles) are able to
staples, tissue adhesives and tapes. be classified according to their physical char- be passed through the tissue with less rota-
acteristics. Monofilament sutures are made tion of the forearm. Needles come with dif-
Sutures from a single filament (nylon, ProleneW), ferent shapes as well as curvatures (see
Suturing is the traditional method of wound and sutures containing multiple fibres are Fig. 4.1.1). A reverse cutting needle is the
closure. Sutures are divided into two clas- called multifilament (silk, cotton, nylon). Of most common type used for skin closure.
ses on the basis of their degradation prop- these sutures, only nylon is available in both The needle cuts an inverted triangle and
erties. Absorbable sutures degrade rapidly types of filament. Non-absorbable sutures the suture sits on the base of the triangle,
in vivo, and lose their tensile strength are used to close fascial layers (where heal- decreasing the likelihood of cutting out. A
within 60 days. Sutures that degrade more ing is slow) and for skin closure.27,28 conventional cutting needle cuts a triangle
slowly are classified as non-absorbable Sutures come in varying sizes. The size of into the skin and the suture sits in the apex
(Table 4.1.3 for individual suture material the suture to be used depends on the wound of the triangle. For fascia a taper point nee-
characteristics). location and the tensile strength of the tis- dle is used. The cross-section of these nee-
Absorbable sutures are made from either sue to be sutured. Heavy sutures such as dles is a circle that is tapered to a point. It
collagen or synthetic polymers. Gut sutures 40 should be used in the limbs and trunk, does not cut but pushes the tissues aside,
are manufactured from the submucosa of and should also be used on mucous causing less tissue damage and reducing

83
4.1 WOUND MANAGEMENT

Cross section
Table 4.1.4 Example of instruments
required for a simple suture tray Everted wound edges
1  Needle holder (Halsey or Hegar)

1  Toothed dissecting forceps

1  Curved artery/mosquito forceps

Cutting needle 1  Straight artery/mosquito forceps

1  Suture scissors

Correct

be placed so that an equal amount of tissue

Taper needle
is included on each side of the wound, and so Inverted wound edges
that the needle bite includes a broad base
(Figs 4.1.2 and 4.1.3). This is accomplished
Thread
by lifting the wound edge as the needle is
Point Swage passed through the skin on each side, max-
imising the deep tissues included in the
suture.28,29
Body Most wounds sutured in the ED are closed
with interrupted skin sutures. Synthetic non- Incorrect
absorbable sutures are most commonly
used. However, rapidly absorbable sutures Fig. 4.1.2 Normal suture. Suturing technique
Reverse cutting needle for wound edge eversion.
can be used to close the skin in children,
Fig. 4.1.1 Surgical needle characteristics and avoiding the discomfort of suture removal.
types. (From an original drawing by Elaine To place a simple interrupted suture the nee- outcome. In general, the better the blood
Wheildon.)
dle is held so the tip enters the skin at a right supply, the closer together sutures can be
angle, and the hand rotated to ensure the placed.
the chance of the stitch cutting out. For deep needle remains at right angles to the skin There are generally two methods for clos-
tissues that are stronger (such as tendon) a throughout its passage, which aids in maxi- ing a laceration, either suturing from one
tapercut, or combination needle is used it mising the deep tissues captured in the bite. end to the other, or placing sutures that seri-
has a tapered body, but the point is a reverse The stitch should be secured with an instru- ally bisect the wound. A small linear wound
cutting edge.28 ment tie and the knots secured to one side of is easily sutured from end to end, and long
Needles are grasped with a needle holder. the wound to minimise inflammation to the wounds without good landmarks on either
The swage of the needle the region where healing tissue. The initial throw should side are most easily closed by placing the
the needle is hollowed out to join with the include two wraps of the suture material first stitch in the middle and then serially
suture is the weakest point, and grasping around the needle holder; subsequent subdividing the wound. In wounds with defi-
the needle in this region should be avoided. throws should be wrapped once. The knot nite landmarks, such as palmar skin creases
The needle should be grasped in the body should be tied just tight enough to oppose or the vermillion border of the lip, the first
one half or two thirds of the distance from the skin edges. Tying the knot too tightly will suture should be placed to align these
the tip of the needle. cause a reduction in the blood supply to the landmarks.28,30
wound edges and increase the risk of infec- Deep sutures should be placed where
Suturing techniques tion and poor cosmetic outcome. Synthetic there are multiple layers of tissue involved
For closure of a wound with sutures a num- sutures with poor handling should have four and the skin sutures would be under tension.
ber of instruments are needed to maintain a or five throws per knot.2830 They are placed to reapproximate the der-
sterile field and to allow manipulation of the The more sutures placed per centimetre, mal layers of the skin and remove skin ten-
tissues and needle (Table 4.1.4). Finer instru- the finer the control over the wound edge. sion, thus improving cosmetic outcome.
ments should be available for facial lacera- For facial lacerations, the skin sutures should Placing deep sutures inserts a foreign body
tion repair. be placed approximately 3 mm apart and into the wound and increases the risk of
Sutures should be placed to allow apposi- enter the skin about 3 mm from the wound wound infection, so they should only be
tion of all injured layers of the skin. Proper edge. For other areas of the body, sutures placed when necessary and the minimum
suture placement should result in slight should be placed 4 to 5 mm apart and number necessary used. For this reason,
eversion of the wound edges, avoiding a should pierce the skin about 5 mm from deep sutures should be avoided in the hands
depression of the scar when contraction the wound edge. The number of sutures and feet. Deep sutures placed close to the
takes place during wound healing. To ensure used to close a wound should be the minimal skin are sometimes extruded through the
eversion of skin edges the skin suture must number that allows a desired cosmetic wound. To place a deep suture, the needle

84
4.1 WOUND MANAGEMENT
4

WOUND MANAGEMENT
is placed at the depth of the wound and A A continuous suture can be used to close
removed at a more superficial level. The nee- the laceration. It is faster to place than inter-
dle is then placed at the same superficial rupted sutures, removal of sutures is easier
level on the opposite side of the wound and faster, and the tension is spread evenly
and exits deeply so the knot is tied deeply along the wound. The continuous suture can
in the wound (see Fig. 4.1.3).28,30 be percutaneous or subcutaneous and
made with absorbable or non-absorbable
suture material. The disadvantages are that
Special suturing techniques if one part of the suture breaks, the integrity
A variety of special suturing techniques are of the whole wound is lost, and if the wound
available with the sole purpose of aiding the becomes infected, the whole wound needs
provision of skin apposition with everted to be opened to drain the pus. To place a per-
wound edges. The vertical mattress suture cutaneous running stitch an interrupted
is useful in regions with minimal subcutane- suture is placed at one end of the wound
B
ous tissue where the edges are difficult to and only the free end of the suture is cut.
maintain in eversion. The technique is Suturing is continued along the wound in
begun the same way as a simple skin suture, a coil pattern ensuring that the needle
but after the suture loop is made, the skin is passes perpendicularly across the wound
re-entered 1 to 2 mm from the wound edge with each pass. The loop is tightened after
and then tied (Fig. 4.1.4). The horizontal each pass and the last stitch placed beyond
mattress suture reinforces the subcutaneous the end of the laceration. The stitch is tied
tissue and relieves skin tension, but does not using the last loop as the tail.28,30
provide wound edge approximation as well
as the vertical mattress suture (Fig. 4.1.5). Correction of dog ears
The modified or half-buried horizontal mat- A dog ear is the term given to a conical
tress suture (or corner stitch) is the method pucker of redundant skin that may develop
of choice for closing a flap. It relieves tissue at the ends of the wound during suturing.
tension and avoids vascular compromise Fig. 4.1.3 Deep sutures. (A) The buried It is avoided by suturing the wound from
when approximating the tip of the flap subcutaneous suture; (B) the horizontal dermal the middle by sequentially bisecting the
(Fig. 4.1.6).28,30 stitch. wound. Dog ears can be removed in many

A B C D

Fig. 4.1.4 The vertical mattress suture technique is useful to evert wound edges with a natural tendency to roll inwards despite correctly placed simple
sutures. (From an original drawing by Elaine Wheildon.)

A B C D

Fig. 4.1.5 The horizontal mattress suture redistributes tension and everts wound edges. (From an original drawing by Elaine Wheildon.)

85
4.1 WOUND MANAGEMENT

most popular. They are easy and relatively

painless to apply and provide a cosmetic
result that is as good as suturing, with no risk
of causing suture marks. No removal is
required, as they slough off in 7 to 10 days.
They are, however, not suitable for use in
all wounds. If the laceration cannot be
approximated and the wound edges brought
together with minimal tension, then tissue
adhesive is not appropriate. Care should be
taken not to apply too much tissue glue and
to avoid placement over currently bleeding
wounds, as the polymerisation is exothermic
and the patient may notice a heat sensation.
Also, contact with excess blood causes
polymerisation above the skin, limiting the
tensile strength of wound-edge closure.
The tissue glue is applied over the surface
of the wound once its edges have been
approximated by digital pressure. A number
of thin layers of glue are applied across the
wound and the wound held approximated
for about 30 seconds. Care is taken not to
allow glue to spill into eyes or orifices, and
to avoid fixing forceps or gloves to the
patient. The cyanoacrylates also act as their
Fig. 4.1.6 Closure of a flap requires an initial suture of the apex, after which either simple or own dressing providing moist wound healing
horizontal mattress sutures may be used. (From an original drawing by Elaine Wheildon.) conditions under the glue, and have a
degree of intrinsic antimicrobial activity.
ways, the simplest of which is the overlap as sutures, and are slightly more painful to Careful attention to wound cleansing is still
excision technique. The redundant skin is remove. They should not be used on the face needed to avoid wound infections. In gen-
pulled across the wound from one side and or in any other wound where cosmetic out- eral, they are less expensive than sutures
excised along the line of the wound. Redun- come is a high priority.31 or staples and are strongly preferred by
dant skin from the opposite side is pulled patients and families. A comparison of the
across the wound and also excised along Tissue adhesives various methods of wound closure is found
the line of the wound. The wound is then Tissue adhesives have now been in use for in Table 4.1.5.3234
closed (Fig. 4.1.7).28 several decades. The basis of the adhesive
is a cyanoacrylate polymer. The cyanoacry- Skin tapes
Staples late polymerises in the presence of hydroxyl Skin tapes can be used to close small wounds
Stainless steel staples can be applied more ions found in water or blood allowing with low tensile forces. They cannot be used
rapidly than sutures, and they are associated them to bind to the skin. Tissue adhesives over areas of motion such as over joints.
with a lower rate of foreign body reaction are for external use only and should not Wound haemostasis is vital as the tapes will
and infection. Staples are generally consid- be placed within wounds or used on mucous not stick to wet skin. Application of an adhe-
ered especially useful for lacerations of the membranes. sive agent to the skin adjacent to the wound
scalp, trunk, and limbs. However, they do It is in the repair of lacerations to young is necessary to provide adequate adhesion
not allow such meticulous wound apposition children that tissue adhesives have become of the tapes. These adhesive agents (e.g.

Fig. 4.1.7 Dog ear. Direct overlap excision technique. (From an original drawing by Elaine Wheildon.)

86
4.1 WOUND MANAGEMENT
4

WOUND MANAGEMENT
Table 4.1.5 Characteristics of wound closure techniques Table 4.1.6 Timing of suture removal

Technique Advantages Disadvantages Wound location Time of removal (days)

Suture Greatest tensile strength May require removal Face 4

Meticulous closure Painful/requires local anaesthesia
 sedation Scalp 5
Slow application
Costly Upper limbs, trunk 710
Slow to apply
Increased tissue reaction Lower limbs 810

Staples Rapid application Less meticulous closure Over joints 1014

Low cost Painful/requires local anaesthesia
Low tissue reactivity  sedation
Requires removal

Tissue adhesives Painless Lower tensile strength

Rapid application Not for use over joints Treatment of selected
No removal needed Slightly higher incidence of
Low cost dehiscence
injuries
No risk of needle stick
Abrasions
Surgical tapes Least reactive Low tensile strength In abrasions, the underlying tissues are rela-
Rapid application Difficulty maintaining adhesion
Patient comfort May require use of toxic adjuncts tively uninjured, providing a degree of pro-
No risk of needle stick to aid adhesion tection against infection. Cleansing the
Low cost
abrasion is important to flush away bacteria
and remove particulate matter, which should
be removed to prevent infection or tattooing.
Large abrasions or those heavily conta-
Tinc BenzW) are toxic to tissues and cause location of the injury. Removal of the sutures minated may need cleaning under general
pain, so great care should be taken not to too early risks dehiscence; leaving sutures anaesthesia. After adequate cleansing and
spill them into the wound. Tapes are not too long increases tissue reaction and the debridement the wound should be covered
suitable for use in small children as they fre- risk of cross-hatching and wound infection with a non-adherent occlusive or semi-
quently pull them off. Tapes are a useful (Table 4.1.6). In general, sutures are occlusive dressing. A moist environment
adjunct to wound closure, for example after removed earlier in children than in adults. enhances healing, and the environment
suture removal or tissue adhesive applica- Wounds closed with tissue adhesive should under a scab is ideal. However, wounds
tion to decrease tension on the wound. not be covered with an occlusive dressing, without extensive scab formation are more
as the extra moisture will more rapidly comfortable, so a dressing provides the moist
decrease the ability of the glue to maintain environment. Children with large or deep
Post-wound-closure care wound edge apposition. The wound should abrasions should be reviewed in 2 to 3 days
be kept dry for 23 days, after which the to check the wound. Ongoing review should
All patients should be provided with written patients may shower but should avoid bath- be once or twice weekly.
information on care of their wound. Parents ing and swimming. Wounds closed with skin
and children must understand the impor- tapes should be kept dry to prevent prema- Eyelid lacerations
tance of ongoing wound care and be ture removal. A thorough eye examination needs to be
provided with instructions about follow up. performed for all eyelid lacerations. Atten-
tion should be given to the possibility of a
Dressing and suture removal Immobilisation and drains ruptured globe (eccentric pupil) or trauma
After the wound has been sutured it should Wounds that cross joints or are in areas of to the globe (hyphaema, dislocated lens or
be covered with a non-adherent occlusive or highly mobile skin should be immobilised. retinal detachment). Visual acuity should
semiocclusive dressing to protect the wound The joint should be splinted in the position be measured and documented. Any wound
from bacterial invasion and provide a moist of function for 710 days. Plaster of Paris that penetrates the tarsal plate or the inner
healing environment and speed wound heal- can be used to make a cheap and easily canthus requires specialist attention, as do
ing. Ideally, the dressing is left intact until applied splint, or a bulky dressing can be wounds involving the lid margins. Any
suture removal. The dressing should only used to limit motion and prevent the child wound that cannot be adequately assessed
be removed if it becomes saturated or there tampering with the wound. (e.g. in a young child) should be referred
is a risk of infection and inspection is war- In general, drains should not be used in for evaluation under anaesthesia.24
ranted. If the wound is not covered with a wounds that have been closed as they pro- Superficial wounds of the eyelid are rela-
waterproof dressing the dressing can be mote wound infection. If a wound is consid- tively easily repaired with 60 fast absorbing
removed every few days for showering. ered at high risk for infection, delayed gut, with sutures placed close to the wound
Non-absorbable sutures should be removed primary closure should be undertaken rather edge. Care must be taken not to suture into
at the appropriate time, depending on the than closure and drainage.9 the tarsal plate or other deep structures.24

87
4.1 WOUND MANAGEMENT

Lip lacerations be used to secure the sutures and the par- paraffin gauze or the nail replaced to pre-
Inspection of the teeth and oral mucosa is ents warned that while the tongue is anaes- vent adhesions. If a fracture is present, anti-
mandatory in all lip lacerations. Tooth inju- thetised the child may bite through the biotics should be given. If the nail is partially
ries should be documented and referred for stitch.24 avulsed only and is tightly adherent to the
management where necessary; missing nailbed, it is reasonable to leave this intact
teeth warrant investigation to ensure they Fingertip amputation as it will adequately splint and maintain
have not been inhaled or imbedded in the Young children tend to injure their fingertips apposition of any nailbed injury.24
soft tissues of the mouth. Ideal anaesthesia in doors and windows. Most of the injuries in
is via nerve block of the mental or alveolar young children are contused lacerations or Subungual haematoma
nerves for the lower lip or the infra-orbital partial amputations, with complete amputa- A subungual haematoma is a collection of
nerve for the upper lip. Alternatives include tion being less common. Older children are blood between the nail and nailbed. It is
sedation and direct infiltration, with or with- more prone to injury with knives or tools. most commonly seen with blunt fingertip
out application of methylene blue to the Fractures are less common in the older age injuries and may be associated with a frac-
margins of the vermillion border. group. These wounds should be evaluated ture of the distal phalanx. Drainage of the
Wounds that involve the vermillion border for tissue loss and with radiography for bony haematoma usually provides symptomatic
(the junction of the dry oral mucosa and the injury. relief and should be undertaken whenever
facial skin) must be exactly realigned to If the amputated fragment has been the haematoma is causing pain. Generally
achieve acceptable cosmetic results. A 60 retained and involves any of the nailbed no local anaesthesia is required to drain
suture should be used, with the first suture some surgeons reimplant as a graft, with the haematoma with cautery or needle bur-
placed to exactly reappose the vermillion approximately 50% chance of the graft tak- ring using a 19-gauge needle. If there is an
border.24 Further sutures should close the ing. If the tissue is not retained or is small, underlying fracture antibiotics should be
skin and the dry mucosal surface of the lip, and there is no bone on view, it is most administered. Nail removal for inspection
with the wet mucosal surface only closed if appropriate to allow the wound to heal by of the nailbed should not be undertaken,
it is gaping significantly. secondary intention. Fingertips allowed to regardless of the size of haematoma.24
Deep or through and through lacerations heal naturally have greater length and bet-
of the lip require deep sutures to repair the ter sensory outcome than those treated with Puncture wounds to the foot
orbicularis oris muscle: if this is the case, an grafts. These wounds should be covered with Puncture wounds to the foot carry a high risk
absorbable 60 suture should be used. The a non-adherent occlusive or semiocclusive of infection and retained foreign body. All
deep sutures should be placed after the ini- dressing to allow moist wound healing after puncture wounds should be assessed for
tial suture is placed at the vermillion border thorough cleaning and debridement as retained foreign body with radiography,
and before tying that stitch.24 needed. Follow up should be maintained and ultrasound for radiolucent foreign bod-
until the wound is healed. ies. Anaesthesia with local infiltration or a
Injuries involving just the fingertip but posterior tibial nerve block is needed. The
Tongue lacerations not the nail heal very well. Injuries involving wound should be soaked to remove any scab
Most tongue lacerations can be left to heal the nailbed or nail, but sparing bone, heal on the surface, debrided and irrigated. The
without intervention with good results. well. Those that involve the nailbed, nail wound should be left open once any foreign
Large lacerations involving the free edge and distal phalanx heal less well. Any injury material has been removed. The wound
of the tongue should be repaired to avoid with bone on view should be referred for spe- should be cleaned with an antibacterial solu-
healing with a notch, interfering with the cialist care.23,24,35 tion (such as BetadineW) and dressed with a
function of the tongue. Large flaps (that non-adherent dressing. Close review is
gap when the tongue is in the resting posi- Nailbed lacerations important to detect infection early. Prophy-
tion) and lacerations that continue to bleed Trauma to the distal fingers is often asso- lactic antibiotics have not been shown to
should also be repaired. Care should be ciated with nailbed injury. An underlying frac- prevent infection and may predispose to
taken when repairing these injuries because ture of the distal phalanx should be assessed Pseudomonas infection.
of the risk of airway compromise, especially with radiographs. Unrepaired nailbed lacera-
considering moderate to deep sedation is tions can permanently disfigure the growth of Bites
likely to be necessary. General anaesthesia the new nail from the matrix. Animal bites
and repair in the operating theatre should If the nail is lacerated, completely Animal bites are a common presenting prob-
be considered if there is doubt. avulsed, or only loosely attached, the nail- lem for the ED. Dog and cat bites account for
The tongue should be maintained in bed must be explored. This can be done virtually all bites seen in the ED, with dog
position by a gentle pull using a towel clip under local anaesthesia with a ring block bites being about six times more common.
or 40 suture placed through the tip. Inter- of the digital nerves or under general anaes- Rodents and other animals account for less
rupted 40 absorbable sutures should be thesia. The nail must be removed and the than 1 to 2% of bites.
placed using full thickness bites to include nailbed repaired with 50 or 60 absorbable Dog-bite injuries tend to be relatively
both mucosal surfaces and the lingual suture material. The space between the large, relatively superficial crush injuries,
muscle in each stitch. Multiple knots should nailbed and nailfold must be packed with which are seen most commonly on the face,

88
4.1 WOUND MANAGEMENT
4

WOUND MANAGEMENT
neck and scalp in children. The overall infec- documented as adequately treating infec-
tion rate for dog bites is about 10%, with tions of P. multocida, but treatment failures References
facial wound infection rates of about 5%. have been documented for erythromycin 1. Singer AJ, Thode Jr HC, Hollander JE. National trends in
ED lacerations between 1992 and 2002. Am J Emerg Med
Dog-bite wounds are infected with multiple and first-generation cephalosporins. It is 2006;24(2):1838.
organisms on all occasions, with both aero- recommended that all cat bites receive pro- 2. Young SJ, Barnett PL, Oakley EA. 10. Bruising, abrasions
and lacerations: minor injuries in children I. Med J Aust
bic and anaerobic bacteria. It is reasonable phylactic antibiotics (see Table 4.1.7).36,37 2005;182(11):58892.
to cleanse and close most dog bites, with 3. Hollander JE, Singer AJ. Laceration management. Ann
Emerg Med 1999;34:35667.
antibiotic prophylaxis provided only to 4. Capellan O, Hollander JE. Management of lacerations in
Human bites the emergency department. Emerg Med Clin North Am
wounds that are high risk for infection
Most human bites are probably at no more 2003;21:20531.
(Tables 4.1.7 and 4.1.8). Amoxicillin with 5. Hollander JE, Singer AJ, Valentine SM, Shofer FS. Risk
risk of infection than ordinary lacerations factors for infection in patients with traumatic
clavulanate is the most useful drug.
and they are not considered to carry a high lacerations. Acad Emerg Med 2001;8:71620.
Cat bites, on the other hand, are typically 6. Knapp JF. Updates in wound management for the
risk of human immunodeficiency virus trans- paediatrician. Pediatr Clin North Am 1999;46:
puncture wounds with less surrounding tissue
mission. Prophylactic antibiotics have, how- 120113.
injury. They have bacteria inoculated deep 7. NHMRC . The Australian Immunisation Handbook.
ever, been shown to reduce the risk of 8th ed. Commonwealth of Australia; 2008.
into the wound, which is difficult to explore,
infection. Appropriate prophylactic antimi- 8. Singer AJ, Hollander JE, Quinn J. Evaluation and
irrigate or debride. The risk of infection is management of traumatic lacerations. N Engl J Med
crobial choices for human bite injuries 1997;337:11428.
significantly higher than in dog bite at least
include amoxicillin with clavulanate.36 How- 9. Baruch J. Laceration repair. J Gen Intern Med 2005;
twice as likely because of the puncture-type 20(6):5568.
ever, the clenched-fist injury (or fight bite), 10. Weinberger LN, Chen EH, Mills AM. Is screening
wound, the most common location of the bite
which commonly causes a ragged laceration radiography necessary to detect retained foreign bodies
being the hand, and the high incidence in adequately explored superficial glass-caused wounds?
over the fourth or fifth metacarpophalan- Ann Emerg Med 2008;51(5):6667.
(about 80%) of Pasteurella multocida found
geal joint is at high risk of infection. These 11. Anderson MA, Newmeyer WL, Kilgore ESJ. Diagnosis and
in cats mouths. P. multocida is a facultative, treatment of retained foreign body in the hand. Am J Surg
latter wounds should all receive prophylactic 1982;114:63.
anaerobic Gram-negative rod that often
antibiotics, as should human bites (including 12. Zempsky WT, Karasic RB. EMLA versus TAC for topical
results in rapidly progressive cellulitis. It anesthesia of extremity wounds in children. Ann Emerg
self-inflicted bites) that have high-risk prop- Med 1997;30:1636.
is sensitive to the penicillins and variably
erties (see Tables 4.1.7 and 4.1.8).36 13. Kennedy RM, Luhmann JD. Pharmacological
sensitive to macrolides and first-generation management of pain and anxiety during emergency
cephalosporins. All these drugs have been procedures in children. Paediatr Drugs 2001;
3(5):33754.
Controversies 14. Ernst AA, Marvez-Vails E, Nick TG, et al. LAT versus TAC
for topical anesthesia in face and scalp lacerations. Am J

Table 4.1.7 Wound infection risk factors Subcutaneous sutures close deep Emerg Med 1995;13:1514.
15. Blackburn PA, Butler KH, Hughes MJ, et al. Comparison of
wound dead space reducing fluid tetracaineadrenalinecocaine (TAC) with topical
High risk Low risk collection and infection, but deep sutures lidocaineadrenaline (TLE): Efficacy and cost. Am J Emerg
Med 1995;13:31517.
Biting Cat Dog of themselves can cause infection by 16. Loryman B, Davies F, Chavada G, Coats T. Consigning
species Human Rodent brutacaine to history: a survey of pharmacological
acting as a foreign body.
techniques to facilitate painful procedures in children in

Location of Hand Face emergency departments in the UK. [see comment].
wound Over a joint Scalp Dressing practice for all wounds is Emerg Med J 2006;23(11):83840.
Below knee Mucosa changing to promote moist wound 17. Priestley S, Kelly AM, Chow L, et al. Application of
Through and topical local anesthetic at triage reduces treatment
through oral healing; this speeds rate of healing and time for children with lacerations: a randomized
improves wound comfort. controlled trial.[see comment]. Ann Emerg Med 2003;42
Wound type Puncture Large (1):3440.
Extensive crush Superficial
Old Recent
 A number of topical anaesthetic 18. Sectish TC. Use of sedation and local anesthesia to
prepare children for procedures. Am Fam Physician
creams are being used in extremity 1997;55:90916.
wounds despite not being licensed for 19. Seropian R, Reynolds B. Wound infection after
preoperative depilatory versus razor preparation. Am J
this use. Surg 1971;121:251.
20. Moscati RM, Mayrose J, Reardon RF, Janicke DM,

Table 4.1.8 Management of bite wounds

Wound drains are not indicated in Jehle DV. A multicenter comparison of tap water versus
sterile saline for wound irrigation.[see comment]. Acad
the management of wounds in the ED, Emerg Med 2007;14(5):4049.
Species Suturing Antibiotics and delayed primary closure is a 21. Stevenson TR, Thacker JG, Rodeheaver GT, et al.
Cleansing the traumatic wound by high pressure
Dog Yes High-risk preferred technique. syringe irrigation. J Am Coll Emerg Physicians
wound

1976;5:1721.
type only Debridement should remove as little 22. Wheeler CB, Rodeheaver GT, Thacker JG, et al. Side
tissue as possible to maximise cosmetic effects of high pressure irrigation. Surg Gynecol Obstet
Cat Face only All 1976;143:7758.
outcome. 23. Al-Nammari SS, Quyn AJ. Towards evidence based
Rodent Yes No emergency medicine: best BETs from the Manchester

Human No Yes
Prophylactic antibiotics, while Royal Infirmary. Conservative management or suturing
for small, uncomplicated hand wounds. Emerg Med J
hand bites intended to prevent infection, have been 2007;24(3):2178.
shown in some wounds to increase the 24. Brown DJ, Jaffe JE, Henson JK. Advanced laceration
Human Yes Large management. Emerg Med Clin North Am 2007;25
other bites wounds risk of infection with unusual organisms. (1):8399.

89
4.1 WOUND MANAGEMENT

25. Post traumatic wound infections. eTG complete (Internet). 30. Suturing Techniques. eMedicine 2009; http://emedicine. randomized controlled trials. Acad Emerg Med 2003;10
Melbourne: Therapeutic Guidelines Ltd; 2009. http:// medscape.com/article/1128240-overview [accessed (2):1108.
online.tg.org.au/ip/ [accessed 10.12.09]. 16.11.09]. 34. Singer AJ, Thode Jr HC. A review of the literature on
26. Singer AJ, Mach C, Thode HCJ, et al. Patient priorities 31. Khan A, Dayan PS, Miller S, et al. Cosmetic outcome octylcyanoacrylate tissue adhesive. Am J Surg 2004;187
with traumatic lacerations. Am J Emerg Med of scalp wound closure with staples in the (2):23848.
2000;18:6836. pediatric emergency department: a prospective 35. Quinn J, Cummings S, Callaham M, Sellers K. Suturing
27. Lin K, Farinholt H, Reddy V, et al. The scientific basis for randomised trial. Pediatr Emerg Care 2002;18: versus conservative management of lacerations of the
selecting surgical sutures. J Long Term Eff Med Implants 1713. hand: randomised controlled trial. BMJ
2001;11:2940. 32. Holger JS, Wandersee SC, Hale DB. Cosmetic outcomes of 2002;325:299305.
28. Ratner D, Nelson BR, Johnson TM. Basic suture materials facial lacerations repaired with tissue-adhesive, 36. Broom J, Woods M. Management of bite injuries.
and suturing techniques. Semin Dermatol 1994;13:206. absorbable, and nonabsorbable sutures. Am J Emerg Med Australian Prescriber 2006;29:68.
29. Sutures and Needles. eMedicine 2009; http://emedicine. 2004;22(4):2547. 37. Bites, Animal: Treatment & Medication. eMedicine 2009;
medscape.com/article/884838-overview [accessed 33. Farion KJ, Osmond MH, Hartling L, et al. Tissue adhesives http://emedicine.medscape.com/article/768875-
15.11.09]. for traumatic lacerations: a systematic review of overview [accessed 15.11.09].

90
 5

SECTION
CARDIOVASCULAR
Section editor Ian Everitt

5.1 Cyanotic heart disease and tetralogy 5.5 Congenital heart disease 103
of Fallot spells 91
5.6 Acute rheumatic fever 107
5.2 Heart failure 94
5.7 Infective endocarditis 110
5.3 Syncope 96
5.8 Kawasaki disease 112
5.4 Cardiovascular assessment and murmurs 99
5.9 Cardiac arrhythmias 115

5.1 Cyanotic heart disease and tetralogy

of Fallot spells
Robin Choong

ESSENTIALS
1 Cyanosis is dependent on haematocrit and oxygen saturation.
2 Cyanosis is caused by arterial oxygen desaturation or increased capillary oxygen extraction.
3 The hyperoxia test helps differentiate cardiac from other causes of cyanosis:
Measure SpO2 in room air or low FiO2;
Remeasure SpO2 in 100% O2;
If SpO2 remains low consider a fixed intracardiac shunt (cyanotic congenital heart defect);
If SpO2 increases consider other causes, e.g. lung disease.
4 If a cardiac cause is suspected check the pulses, blood pressure, ECG, and chest X-ray. Consult with a neonatologist, paediatrician or
cardiologist early.
5 Cyanosis may be due to a duct-dependent lesion. A prostaglandin infusion may be advised following consultation.
Tetralogy spells
1 Cyanosis is variable and depends on the amount of pulmonary blood flow and right-to-left shunting.
2 Hypercyanotic spells treatment:
Knee-chest position or over parents shoulder with knees bent;
Supplemental oxygen;
Sedation: intravenous or subcutaneous morphine, 0.1 mg kg1;
Intravascular volume expansion.
3 In prolonged cyanosis consider a vasoconstrictor.
4 For prevention of spells, propranolol (0.51 mg kg1 po qid).
5 A BlalockTaussig shunt may be used for palliation before corrective surgery.

91
5.1 CYANOTIC HEART DISEASE AND TETRALOGY OF FALLOT SPELLS

clinically. Other confounding factors may be

Introduction acrocyanosis (a normal finding, which may Management
Cyanosis is a bluish discolouration of skin and last 72 hours), polycythaemia (giving the The presentation of cyanosis in an infant or
mucous membranes due to excessive concen- appearance of cyanosis) and dark child mandates urgent review by a neonatol-
tration of reduced haemoglobin in the blood.1 skin (cyanosis is more difficult to detect). ogist, paediatrician or paediatric cardiolo-
Cyanosis is evident when deoxygenated Cyanosis is better appreciated in natural gist. Echocardiography may be necessary
haemoglobin in the cutaneous veins reaches light than in artificial light. urgently. Early discussion with a neonatolo-
approximately 5 g dL1.2 Deoxygenated hae- Arterial oxygen saturation should always gist or cardiologist can help clarify possible
moglobin may occur either from arterial blood be assessed with pulse oximetry when consid- diagnoses and initial management in the
desaturation or increased oxygen extraction ering cyanosis in the newborn. The hyperoxia emergency department (ED) setting.
by peripheral tissue. Central cyanosis is pro- test may help distinguish cyanosis caused by An important issue in the management of
duced as a result of arterial desaturation, i.e. cyanotic heart defects from other defects.24,8 cyanosis in the newborn period is recognising
aortic blood carrying deoxygenated haemo- Hyperoxia test the duct-dependent lesion. A duct-dependent
globin. Isolated peripheral cyanosis may result Measure SpO2 in room air or low FiO2. lesion is one that results from the ductus arter-
from excessive deoxy-haemoglobin caused by Remeasure SpO2 after 15 minutes in iosus remaining patent so that blood flow is
extensive oxygen extraction.1 Haemoglobin 100% O2. delivered to both the pulmonary and systemic
level affects the presence of cyanosis. Cyano- If SpO2 remains low, consider fixed circuits:
sis is detected at a higher oxygen saturation in intracardiac shunt (cyanotic congenital
children with polycythaemia and is more diffi-
Pulmonary atresia/critical pulmonary
heart defect). Most cyanotic CHDs have a
stenosis. The ductus shunts right-to-left to
cult to detect in children with severe anaemia. PaO2 <60 mmHg (8 kPa).
ensure adequate pulmonary blood flow.
Causes of cyanosis are listed in Table 5.1.1. If SpO2 increases, consider other causes, e.g.
Coarctation of the aorta/critical aortic
lung disease. PaO2 > 150 mmHg (20 kPa)
stenosis/interrupted aortic arch. The
almost completely excludes cyanotic CHD.
ductus shunts right-to-left to ensure
Cyanotic congenital adequate systemic blood flow.
heart disease Transposition of the great arteries. Mixing
Cyanotic congenital heart disease (CHD) Clinical features2,7 is required usually at atrial, ductus and
generally presents in the neonatal period. ventricular levels.
A comprehensive cardiac and respiratory
In the newborn, mild cyanosis (mild hypoxia If a duct-dependent lesion is considered,
examination is essential in any cyanosed
SaO2 >85%) may be difficult to detect consult a paediatric cardiologist or neonatol-
child. The key features are:
ogist before starting an intravenous infusion
pulses rate, rhythm, volume; of prostaglandin E1 (PGE1, 525 ng1 kg1
Table 5.1.1 Causes of cyanosis27 blood pressure all four limbs if the pulse min1). Important side effects are respira-
Differential diagnosis volume is abnormal; tory depression and fever. Avoid PGE1 when
Arterial oxygen desaturation (central cyanosis precordial impulse heaves, thrills; a small heart accompanies cyanosis and
[pO2 <50 mmHg])
1. Airway
auscultation abnormalities of P2, murmurs; pulmonary oedema, because these findings
Airway obstruction pre-and postductal oximetry (right arm suggest TAPVD with obstructed veins (PGE1
2. Breathing versus left arm/legs) if there is
Lung disease will worsen pulmonary oedema).
Central nervous system depression differential cyanosis. Chronic cyanosis stimulates a reactive poly-
(e.g. coma)
Respiratory muscle weakness cythaemia that increases the oxygen-carrying
Reduced respiratory drive (e.g. morbid capacity. When haematocrit reaches 65% or
obesity)
3. Circulation Investigations more, a large increase in blood viscosity occurs.
Intracardiac right-to-left shunt (e.g. Hyperviscosity and coagulopathy often
cyanotic congenital heart disease, Investigations are directed at likely causes,
pulmonary hypertension) occur and in patients with a right-to-left intra-
Intrapulmonary shunt (e.g. pulmonary after history and clinical examination. They cardiac shunt may result in stroke and brain
atrioventricular fistula) may include arterial blood gas, serum elec- abscess.
Increased capillary oxygen extraction trolytes, glucose levels, full blood count, hae-
(peripheral cyanosis) matocrit, and cultures.
1. Circulatory shock, e.g. sepsis The chest X-ray (CXR) can be very useful and
2. Congestive heart failure
should be examined carefully for heart size
TETRALOGY SPELLS
3. Environmental, e.g. cold
4. Acrocyanosis of the newborn (autonomic (normal cardiothoracic ratio in AP film is
may last 72 hours)
<0.6), oligaemic lung field with decreased
Introduction210
Abnormal haemoglobin (not related to pulmonary blood flow, e.g. tetralogy of Fallot,
level of oxygenation) or pulmonary venous congestion with an obs- Hypercyanotic, hypoxic or cyanotic spells occur
1. Methaemoglobinaemia (pO2 >50 mmHg but
low O2 saturation)
tructive lesion, e.g. totally anomalous pulmo- in young infants with tetralogy of Fallot. These
nary venous drainage (TAPVD), cor triatriatum. occur most commonly under 6 months of age

92
5.1 CYANOTIC HEART DISEASE AND TETRALOGY OF FALLOT SPELLS
5

CARDIOVASCULAR
and may be precipitated by agitation from any Chest X-ray
cause. The cause is likely to be multifactorial, The classic coeur en sabot or boot-shaped Disposition
including a component of dynamic infundibu- cardiac silhouette is caused by the elevation Organise hospital admission and paediatric
lar obstruction. The net result is an imbalance of the apex due to right ventricular hypertro- cardiology consultation.
of pulmonary to systemic blood flow. phy, combined with a concavity in the area of Failure to respond to the above treatment
Tetralogy of Fallot consists of anterior the main pulmonary artery. A right-sided mandates intubation, ventilation and deep
deviation of the outlet septum leading to aorta is present in 25% of tetralogy patients. sedation or general anaesthesia and intensive
the characteristic morphological features of: care admission. Emergency surgery is rarely
Echocardiography indicated.
subpulmonary infundibular stenosis

pulmonary valve stenosis
hypoplastic An echocardiogram will accurately confirm
pulmonary arteries; tetralogy of Fallot but is not useful in acute
management of spells.
subaortic perimembranous ventricular References
septal defect (VSD); 1. Anderson DM. Dorlands Illustrated Medical Dictionary.
Philadelphia: WB Saunders; 1994.
aortic root overriding the crest of septum
2. Park MK. Congestive heart failure. In: Park MY, editor.
and VSD; Treatment26,813 Pediatric cardiology for practitioners. 5th ed.
Philadelphia: Mosby Elsevier; 2008.
secondary right ventricular hypertrophy; If possible, the child should be in a quiet, 3. Abelson WH, Garth Smith R. Residents handbook of
calm environment. Being held in pediatrics. 7th ed. Toronto: The Hospital for Sick Children,
Clinical features include: Toronto, Canada, BC Decker Inc; 1987.
parents arms often diminishes crying 4. Guzman MF, Hedley Brown A, Been M, et al. Manual of
intense cyanosis; and helps pulmonary blood flow. cardiorespiratory critical care. Sevenoaks, Kent:
Butterworth; 1989.
hyperpnoea and acidosis; The kneechest or squatting position is 5. Kilham H, Isaacs D. The New Childrens Hospital
quieter and shorter ejection murmur preferred as it increases after-load, thus Handbook. Westmead: RAHC; 1999.
6. Apitz C, Webb GD, Redington AN. Tetralogy of Fallot.
(compared to before the spell); decreasing R to L shunting. Lancet 2009;374:146271.
irritability, lethargy, loss of consciousness,  Supplemental high-flow oxygen 7. Allen HD, Driscoll DJ, Shaddy RE, et al. Moss and Adams
heart disease in infants. children and adolescents:
seizures, death. (1015 L min1 of 100% oxygen via Including the fetus and young adult. 7th ed. Philadelphia:
Lippincott Williams & Wilkins; 2007.
Triggers include dehydration, exertion and mask) should be provided.
8. Nichols DG, Cameron DE. Critical heart disease in
emotional distress associated with crying Monitoring includes continuous ECG infants and children. 2nd ed. Philadelphia: Mosby
monitoring, pulse oximetry, and non- Elsevier; 2006.
(increases pulmonary vascular resistance 9. Siwik ES, Erenberg F, Zahka KG, Goldmuntz E. Tetralogy
and hence decreases pulmonary blood flow). invasive BP. of Fallot. In: Allen HD, Driscoll DJ, Shaddy RE, et al.,

The child may have a history of previous Morphine (0.10.2 mg kg1 editors. 2007 Moss and Adams Heart Disease in
Infants, Children and Adolescents: Including the Fetus
squatting. Spells are usually self-limiting intravenously (IV) or subcutaneously and Young Adult. 7th ed. Philadelphia: Lippincott
Williams & Wilkins; 2007.
though sequelae include hypoxicischaemic (SC)) should be used to treat hyperpnoea
10. Park MK. Tetralogy of Fallot. In: Park MY, editor. Pediatric
encephalopathy and death. Spells are less and decrease systemic catecholamines, cardiology for practitioners. 5th ed. Philadelphia: Mosby
Elsevier; 2008.
common since the advent of early systemic- and often aborts crying, which
11. van Roekens CN, Zuckerberg AL. Emergency management
pulmonary shunts or early corrective surgery. perpetuates the spell. of hypercyanotic crises in tetralogy of Fallot. Ann Emerg
Consider a small fluid volume challenge Med 1995;25(2):2568.
12. Baele PL, Rennotte MT, Veyckemans FA. External
(510 mL kg1) to increase preload and compression of the abdominal aorta reversing tetralogy
Investigations reduce dynamic outflow obstruction. of Fallot cyanotic crisis. Anaesthesiology 1991;75
(1):1469.
Investigations include laboratory testing Propranolol (0.050.2 mg kg1 slow IV 13. Nussbaum J, Zane EA, Thys DM. Esmolol for the
treatment of hypercyanotic spells in infants with
and imaging as follows. push over 10 minutes, repeat once every
tetralogy of Fallot. J Cardiothorac Anaesthesiol 1989;3
15 minutes) can be used to block b- (2):2002.
Laboratory receptors in the infundibulum, thereby
Blood gas analysis usually shows acidosis lessening RV outflow obstruction. Other
with hypoxia. b-blockers may be useful. Further reading
 Phenylephrine (phenylephrine,
ECG Allen HD, Driscoll DJ, Shaddy RE, et al. Moss and Adams heart
510 mcg kg1 IV [maximum SC/IM disease in infants, children and adolescents: Including the
The ECG in tetralogy of Fallot usually shows: fetus and young adult. 7th ed. Philadelphia: Lippincott
dose 10 mg]) increases afterload, Williams & Wilkins; 2007.
right ventricular hypertrophy in the thereby decreasing right-to-left shunt. Apitz C, Webb GD, Redington AN. Tetralogy of Fallot. Lancet
2009;374:146271.
unipolar and standard leads; Alternatives include metaraminol Breitbart RE, Fyler DC. Tetralogy of Fallot. In: Keane J, Fyler D,
right axis deviation (120 to 150 ); (0.01 mg kg1 IV) or methoxamine Lock J, editors. Nadas paediatric cardiology. 2nd ed.
Philadelphia: Saunders Elsevier; 2006.
dominant R wave in the right and a (0.1 mg kg1 IV). Nichols DG, Cameron DE. Critical heart disease in infants and
dominant S in the left precordial leads;  Consider NaHCO3 (12 mmol kg1 IV) children. 2nd ed. Philadelphia: Mosby Elsevier; 2006.
Park MK. Congestive heart failure. In: Park MY, editor.
Right atrial hypertrophy; for correction of acidosis. Pediatric cardiology for practitioners. 5th ed. Philadelphia:
normal PR interval and QRS duration;  Check temperature and blood glucose. Mosby Elsevier; 2008.
van Roekens CN, Zuckerberg AL. Emergency management of
tall, peaked T waves; Correct hypoglycaemia and hypercyanotic crises in tetralogy of Fallot. Ann Emerg Med
reversal of the RS ratio. hypothermia. 1995;25(2):2568.

93
 5.2 Heart failure
Robin Choong

ESSENTIALS Clinical manifestations and

investigations14
1 Congestive heart failure (CHF) is a clinical syndrome and may result from:
Presentation
excessive workload caused by increased pressure or volume, usually increased Infants may present with problems related
pulmonary blood flow, and/or to feeding, such as sweating, tachypnoea,
normal workload faced by a damaged myocardium. reduced volume of feeds leading to failure to
thrive. Respiratory problems, such as cough,
2 Clinical signs may include tachycardia, tachypnoea, increased work of breathing, recurrent respiratory infections, tachypnoea
sweatiness, cardiomegaly and hepatomegaly. In addition, infants may have failure to and increased work of breathing are promi-
thrive, recurrent lower respiratory tract infections and respiratory distress. nent. Lower respiratory tract infection may
3 In older children, new onset heart failure may be less overtly symptomatic. Malaise, have features in common with congestive
decrease in the level of daily activity, abdominal pain, nausea, anorexia and weight cardiac failure (CCF). Cardiac failure should
loss may be present. be considered in atypical, persistent or recur-
rent cases of lower respiratory tract infection
4 Current medications used in heart failure include diuretics, digoxin and (LRTI), particularly in infants.
angiotensin-converting enzyme inhibitors.

General features
First days of life Tachycardia due to fixed stroke volume.
Causes of congestive
Asphyxia, left heart obstruction, metabolic, Tachypnoea due to decreased
heart failure14 sepsis. compliance with increased lung water.
Most cases of congestive heart failure in Gallop rhythm, weak pulse.
childhood result from congenital heart Failure to thrive from decreased energy
defects. One day to one week of age intake, increased energy expenditure.
Left heart obstruction in duct-dependent Cardiomegaly may be due to left heart
Left-to-right shunts with increased lesions, e.g. aortic stenosis, coarctation of obstruction with left-to-right shunt or left
pulmonary blood flow, e.g. ventricular the aorta. ventricular dysfunction.
septal defect (VSD), atrioventricular Large left-to-right shunt lesions, e.g. large
septal defect (AVSD), patent ductus
Sweaty (cold sweat) prominent with
VSD, AVSD, truncus arteriosus. feeding.
arteriosus (PDA), atrioventricular septal
defect, patent ductus arteriosus.
Fatigue/lethargy.
Acute left heart obstruction, e.g. aortic
Hepatomegaly not specific to right
First month
heart failure unlike in adults, may occur
stenosis, coarctation of the aorta, Large left-to-right shunt, e.g. large VSD,
with any aetiology.
interrupted aortic arch, hypoplastic left AVSD, truncus arteriosus.
heart syndrome.
A smaller number of cases may be from: Investigations
First three months
Routine
 Primary myocardial dysfunction, e.g. Large left-to-right shunt, e.g. large VSD,
Chest X-ray
myocarditis, cardiomyopathy, AVSD, truncus arteriosus.
Cardiomegaly (in an AP film the normal
anomalous left coronary artery. Metabolic disease.
cardiothoracic ratio is <0.6). Pulmonary
Other causes, e.g. anaemia, metabolic, Acquired heart disease may occur at any
plethora in left-to-right shunt. Pulmonary
toxic, dysrhythmia. age due to:
venous congestion in left heart
inflammatory/infective, e.g. viral obstruction/left ventricular dysfunction.
Age-based myocarditis, Kawasaki disease, Radiologic features include
The causes of congestive heart failure pleuropericarditis, rheumatic fever; reticulogranular pattern, fluid in lung
change with age. Congenital heart defects metabolic genetic, e.g. inherited fissures, pleural effusions, pulmonary
generally present in the first days or months cardiomyopathy; oedema.
of life. Acquired cardiac disease may occur at dysrhythmia, e.g. sustained SVT, ECG
any age. The following lists the likely causes ventricular tachycardia (VT); Assess rhythm, myocarditis (voltages),
at particular ages. severe anaemia. ischaemia (ST-T wave).

94
5.2 HEART FAILURE
5

CARDIOVASCULAR
Specialized Digoxin adults with severe cardiac failure, improve-
Echocardiography. Severe congestive heart failure treated ment in 31-day survival compared with
Cardiac catheterisation. with initial IV administration. Early failure dobutamine has been shown.
Others: angiography (computerised in less distressed infants can be treated
tomography and magnetic resonance orally. All patients should have oral
imaging), viral studies (blood, throat therapy as soon as possible. IV dosage is References
swab, faeces), chromosomal analysis, only 7080% of amount used orally. 1. Park MK. Congestive heart failure. In: Park MY, editor.
urine metabolic screen, myocardial tissue The total digitalising dose (premature: Pediatric cardiology for practitioners. 5th ed.
Philadelphia: Mosby Elsevier; 2008.
biopsy. 20 mcg kg1; infant: 3040 mcg kg1; 2. Shaddy RE, Tani LY. Chronic Congestive Heart failure. In:
over 2 years: 30 mcg kg1) may be given Allen HD, Driscoll DJ, Shaddy RE, et al., editors. 2007
Moss and Adams heart disease in infants, children and
as 1/2 stat, 1/4 in 612 hr, and 1/4 in adolescents: Including the fetus and young adult. 7th ed.
Referral
another 1218 hr. Philadelphia: Lippincott Williams & Wilkins; 2007.
The diagnosis of cardiac failure in an infant 3. Abelson WH, Garth Smith R. Residents handbook of
Maintenance dose (IV, oral) is 3 to 5 pediatrics. 7th ed. Toronto: The Hospital for Sick Children,
or child mandates urgent review by a paedi-
mcg kg1 12-hourly (maximum of Toronto, Canada, BC Decker Inc; 1987.
atrician or paediatric cardiologist. 4. Guzman MF, Hedley Brown A, Been M, et al. Manual of
200 mcg (IV) to 250 mcg (oral)). If heart cardiorespiratory critical care. Sevenoaks, Kent:
failure is not severe, then may start with Butterworth; 1989.
5. Chang AC, Hanley FL, Wernovsky G, Wessel DL. Pediatric
a maintenance dose only.
Management13,515 Digoxin is contraindicated in complete
cardiac intensive care. Baltimore, MD: Williams & Wilkins;
1998.
6. Shaddy RE. Optimizing treatment for chronic congestive
Current medications used in heart failure heart block, pericardial tamponade, heart failure in children. Crit Care Med 2001;29(Suppl):
include diuretics, digoxin and angiotensin- hypertrophic cardiomyopathy and other S23740.
7. Schwartz SM, Duffy JY, Pearl JM, Nelson DP. Cellular and
converting enzyme inhibitors. outflow tract obstructions. Reduce dose in molecular aspects of myocardial dysfunction. Crit Care
renal failure. Med 2001;29(Suppl.):S21419.
8. Clark IIIrd BJ. Treatment of heart failure in infants and
Acute management Monitor with ECG and serum digoxin level children. Heart Dis 2000;2(5):35461.
Resuscitate with ventilatory support (keep below 2.5 nmol L1 [2.0 ng L1]). 9. Buchhorn R, Hammersen A, Bartmus D, Bursch J. The
pathogenesis of heart failure in infants with congenital
if required. heart disease. Cardiol Young 2001;11(5):498504.
If desaturated consider supplemental Angiotensin-converting enzyme 10. Kay JD, Colan SD, Graham Jr TP. Congestive heart
failure in pediatric patients. Am Heart J 2001;142(5):
oxygen. Caveat: large left-to-right shunt inhibitors 9238.
lesions (e.g. truncus arteriosus) may Angiotensin-converting enzyme inhibitors are 11. Wernovsky G, Hoffman TM. Pediatric heart failure
management: Solving the puzzle. Crit Care Med 2001;
worsen with high FiO2. Consult early in used in heart failure due to large left-to-right 29(10 Suppl.):S21213.
these cases. shunts and heart failure caused by ventricular 12. Shaddy RE. Beta-adrenergic receptor blockers as therapy
in pediatric chronic heart failure. Minerva Pediatr
 Fluid restriction and diuretic therapy. failure. Oral captopril (0.11 mg kg1 dose1 2001;53(4):297304.
Infants with left heart obstruction may 8-hourly) or lisinopril (0.1 mg kg1 daily, 13. Shekerdemian L. Nonpharmacologic treatment of acute
heart failure. Curr Opin Pediatr 2001;13(3):2406.
present with cardiorespiratory failure increasing to 0.20.4 mg kg1 daily over 46 14. De Luca L, Colucci WS, Nieminen HS, et al. Evidence-
when the ductus arteriosus closes. weeks) may be used. based use of levosimendan in different clinical settings.
Eur Heart J 2006;27:190820.
Prostaglandin infusion may reopen the 15. Egan JR, Clarke AJB, Williams S, et al. Levosimendan for
ductus and assist systemic perfusion. b-blockers low cardiac output: A pediatric experience. J Intensive
Care Med 2006;21(3):1837.
b-blockers have a role in paediatric heart fail-
Diuretics ure though doses are extrapolated from adult
Acutely: furosemide 1 mg kg1 dose1 data. However, their role in children with
intramuscular (IM) or intravenous(IV) heart failure remains to be fully defined. Further reading
(anticipate hypokalaemia). Allen HD, Driscoll DJ, Shaddy RE, et al. Moss and Adams heart
Chronically: furosemide 13 mg kg1 day1 Levosimendan disease in infants, children and adolescents. 7th ed.
Philadelphia: Lippincott Williams & Wilkins; 2007.
in divided doses, with spironolactone, Levosimendan is an IV calcium sensitiser Archer N, Burch M. Paediatric cardiology. An introduction.
1 mg kg1 dose1 po q12h. Beware used in acutely decompensated severe con- London: Chapman & Hall Medical; 1998.
Park MK. Congestive heart failure. In: Park MY, editor.
potassium-sparing effect of angiotensin- gestive cardiac failure. It has inotropic, vaso- Pediatric cardiology for practitioners. 5th ed. Philadelphia:
converting enzyme inhibitors. dilatory and cardioprotective properties. In Mosby Elsevier; 2008.

95
 5.3 Syncope
Linda Durojaiye Andrew Bullock

should raise suspicion of an alternate

ESSENTIALS diagnosis.
A complete history should include the
1 The most common cause of syncope in children is vasovagal. following:
2 A careful and detailed history will usually enable the diagnosis of vasovagal A description of the event by a witness, if
syncope to be established with confidence.
possible. Ask specifically about duration
3 The main differential diagnoses of syncope in childhood include cardiovascular of loss of consciousness, seizure activity,
causes, seizures, migraines, hypoglycaemia, drugs, and psychogenic events. incontinence of urine, pallor or cyanosis,
and post-ictal drowsiness or confusion.
4 A 12-lead ECG should be done for all children at the initial presentation with Any antecedent events such as sudden
syncope.
emotion or pain, anxiety, fasting,
5 Any child in whom a cardiac cause of syncope is either suspected or diagnosed intercurrent illness, blood loss, or a hot
should be referred to a cardiologist. environment.
Activity and posture at the time of the
event.
understood. The precipitant for reflex anoxic Prodromal symptoms such as nausea,
Introduction sweating, visual or auditory changes, an
seizures may be a noxious stimulus causing
Syncope is the term used to describe any reflex asystole, which leads to an anoxic aura, perioral paraesthesia and
event of sudden and transient loss of con- seizure. carpopedal spasm, palpitations,
sciousness and postural tone. In older children and in adolescents such dyspnoea or chest pain.
The range of incidences of syncope occur- episodes most commonly present as episodes Relevant medical history including
ring in childhood is described as being from of vasovagal syncope. A combination of hypo- previous episodes of syncope, cardiac
0.150%, with peak incidences occurring tension and profound bradycardia, or either disease, epilepsy, diabetes, drug use and
amongst toddlers and adolescents. The most bradycardia or hypotension alone leads to past sexual activity.
common events seen in the paediatric cerebral hypoxia. Complete understanding Family history of sudden collapse or
setting are episodes of vasovagal syncope. of the underlying mechanisms is lacking. death, cardiac disease, epilepsy, syncope
Differentiating the common, benign vasova- Other terms used to describe these episodes and metabolic disease.
gal event from rare differential diagnoses is include neurocardiogenic syncope, vasode-
essential to the appropriate management of pressor syncope or neurally mediated syncope.
Vasovagal syncope
children who present with syncope. The differential diagnoses of syncope in
The typical history of vasovagal syncope is
By contrast, in the adult population syn- childhood include cardiovascular causes, sei-
that the episode occurs whilst standing or
cope is the cause of 13% of all ED atten- zures, migraines, hypoglycaemia, drugs, and
sitting upright. There may or may not be a
dances, and malignant cardiac arrhythmias psychogenic events. These are listed in more
stressful antecedent event (this occurs less
are commonly the underlying cause. detail in Table 5.3.1. It should be noted that
commonly in frequent recurrent vasovagal
situational syncope (syncope that occurs
syncope). There is a prodrome of nausea,
during micturition, swallowing cold liquids,
dizziness, visual disturbance and a sensation
defecation or coughing), and carotid sinus
of warmth, followed by a period of loss of
Aetiology sensitivity are rare in the paediatric popula-
tone and consciousness. Witnesses will
tion. Mitral valve prolapse has not been con-
The final common pathway that leads to all describe marked pallor. Seizure activity is
clusively proven to be a cause of syncope.
episodes of syncope is a sudden decrease in unusual, but brief tonicclonic activity or
delivery of metabolic substrates, namely stiffening is possible, particularly if the
oxygen and glucose, to the brain. patient fails to fall to a recumbent position.
In childhood and adolescence the major Urinary incontinence may also occur. Recov-
Clinical
cause of syncope is transient autonomic ery to a normal level of consciousness is usu-
dysfunction. History ally prompt once in the supine position.
In toddlers such episodes usually manifest A careful and detailed history will usually Children who recognise the prodrome may
as either blue breath-holding spells or reflex enable the correct diagnosis of the most avoid a syncopal episode by assuming the
anoxic seizures (also called pallid breath- common cause of childhood syncope, vaso- lying position. The child may have a head-
holding spells). The mechanism for the cya- vagal syncope, to be established with confi- ache, or be fatigued for minutes to hours
nosis in blue breath-holding spells is poorly dence. Any unusual features of the history after the event.

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5.3 SYNCOPE
5

CARDIOVASCULAR
needed to make the diagnosis. The QT inter-
Table 5.3.1 Causes of childhood syncope
val is calculated with Bazetts formula:
Abnormality of circulation Vasovagal syncope
Reflex anoxic seizures QT
Blue breath-holding attacks QTc p
Cerebral syncope RR
Acute volume depletion
Chronic hypovolaemia
Orthostatic hypotension
where QTc is the corrected QT interval (nor-
Pregnancy mal <0.44 seconds), QT is the QT interval in
Cardiac causes Tachyarrythmias
seconds, and RR is the RR interval in seconds.
Supraventricular tachyarrhythmias
WolffParkinsonWhite syndrome Reflex anoxic seizures and blue
Ventricular tachycardia
Ventricular fibrillation
breath-holding spells
Blue breath-holding spells are usually asso-
Conduction disturbances
Long QT interval
ciated with a prolonged episode of crying
Atrioventricular block after which the child has a prolonged forced
Sinus node disease
expiration and apnoea, and becomes cya-
Left ventricular outflow tract obstruction nosed. This may be followed by a brief
Valvular aortic stenosis
Coarctation of the aorta
period of loss of consciousness, with a rapid
Idiopathic hypertrophic subaortic stenosis recovery to full normal activity. They occur in
Right ventricular outflow tract obstruction
children between the ages of 1 and 5 years,
Pulmonary stenosis with a peak incidence at the age of 2 years.
Tetralogy of Fallot
Primary pulmonary hypertension
They are more common than reflex anoxic
Eisenmengers syndrome seizures.
Large pulmonary embolism
Reflex anoxic seizures occur when an
Dilated cardiomyopathy infant is suddenly startled (for example by
Myocarditis
Idiopathic
an injury). The infant is seen to give one
Coronary artery anomalies or two cries, quietens and then becomes
Pericarditis with tamponade
pale. There is then an abrupt loss of con-
Vertebrobasilar insufficiency sciousness, during which the infant may
Central nervous system disorders Seizure
have brief tonic posturing and upward eye
Migraine deviation. Tonicclonic movements may
Hypoglycaemia
occur. Episodes usually last less than 1 minute,
and are immediately followed by normal con-
Hypoxia
sciousness and posture.
Drugs and poisons (no QT prolongation) Antihypertensive drugs Of these children, 85% have no further epi-
Antiarrhythmics
Carbon monoxide poisoning
sodes after the age of 5 years, though 17%
Volatile nitrites may get recurrence of syncopal episodes later
Others
in life. There is no increased tendency in these
Psychogenic Hyperventilation children to develop epilepsy, and there are no
Hysteria
Malingering
associated abnormalities found on electroen-
Munchausens by proxy cephalogram (EEG) testing.
Panic disorder

Hypovolaemic states
There will usually be a history suggestive of
fluid or blood loss and obvious signs of shock
Cardiac syncope Long QT syndrome is an ECG diagnosis may be present. Orthostatic hypotension
A cardiac cause of syncope should be sus- that is associated with episodes of syncope and tachycardia may be the only positive
pected in a patient with a history of congen- or seizures caused by episodes of paroxys- clinical signs. These tend to occur immedi-
ital heart disease or with a family history of mal ventricular tachycardia (often torsades ately, as distinct from the changes seen in
sudden unexplained death. Cardiac events de pointes). It may result in sudden death. vasovagal syncope, which occur after more
are more likely to cause episodes of syncope Syncopal episodes in patients with this diag- prolonged orthostatic stress.
that occur with no warning, with associated nosis may be precipitated by exercise or a
chest pain, during exercise, whilst sitting or startle, or may be spontaneous. The condi- Seizures
supine, or in association with palpitations tion may be congenital or acquired. The It may be difficult to differentiate seizures
(though palpitations are frequently ECG in sinus rhythm reveals a prolonged from vasovagal episodes, as they may both
described by individuals with vasovagal syn- QT interval. The QT prolongation may be be associated with brief convulsions as well
cope, and with hyperventilation). minimal, and a high index of suspicion is as a loss of consciousness. A history of

97
5.3 SYNCOPE

significant post-event disorientation is

Table 5.3.2 Indications for referral and
helpful in differentiating seizures from other Investigations further investigation of a child with
causes of syncope. Seizures are also more Clinical investigations within syncope
likely to be associated with cyanosis, tongue the emergency department Atypical history
biting, and a more prolonged period of loss Abnormal cardiovascular or central nervous
A 12-lead ECG should be done for all chil- system examination
of consciousness. dren at the initial presentation with syncope. Suspect cardiovascular cause
Suspect seizure
The primary aim is the exclusion of a pro- Recurrent and problematic vasovagal
Hysterical syncope longed QT interval, and of any arrhythmia syncope
Hysterical syncope is a diagnosis to be made or other abnormality of conduction.
once all other possible causes have been A blood sugar level is an appropriate inves-
excluded, but clues strongly suggestive of tigation for the child with a history of fasting, cardiologist. It should be noted that Holter
the diagnosis are often witnessed during or with a family history of metabolic disease. monitoring is often unhelpful as symptoms
the event. The child will have no hypotension, A bHCG is indicated in fertile and sexually rarely occur whilst the patient is monitored.
bradycardia or pallor, and fluttering of half- active adolescent females. Some children may go on to have electro-
closed eyelids may be seen. Other investigations are usually unneces- physiological studies, exercise stress tests
sary, and if done should be appropriate to or cardiac angiography.
Hyperventilation syncope the clinical history and examination findings Where a history obtained suggests that the
Syncope due to hyperventilation may be for the child. child has had a seizure, an EEG should be
associated with a prodrome of lightheaded- Further investigations performed in the arranged in consultation with the neurologist
ness and blurred vision. It is not as com- emergency department (ED) may include: or paediatrician to whom they are referred.
monly seen as other symptoms associated Head-upright tilt-table testing may be
cardiac monitoring and pulse oximetry for
with hyperventilation, such as feelings of done in children with frequent, recurrent
any child in whom the history is
anxiety, breathlessness and perioral para- syncope, and in those children in whom a
suggestive of an arrhythmia, or who
esthesiae or carpopedal spasm. cause for syncope is not certain. Protocols
remains unwell;
for the test vary, but the requirements are
blood haemoglobin concentration where
that the child has a period of supine rest
a history suggestive of significant blood
before tilting and is then tilted at a defined
Examination loss or anaemia is obtained;
angle for a period of time. The most common
serum electrolytes, urea and creatinine,
A structured approach to the clinical exami- positive response seen is a combination of
and urinary specific gravity where a
nation of all children presenting with syn- hypotension and bradycardia prior to
diagnosis of dehydration is suspected;
cope is essential to ensure that all relevant syncope or near syncope. Other positive
a chest X-ray where a structural cardiac
findings are elucidated. responses seen are either isolated hypoten-
abnormality is suspected;
sion or asystole prior to syncope.
The airway must be examined, and the Urine drug screen for patients with a
Video surveillance with continuous ambula-
work of breathing noted. history or examination that suggests
tory EEG and cardiac monitoring may be indi-
The peripheral pulse rate, rhythm and intoxication with a specific substance;
cated where fictitious events are suspected.
character must be noted, and the this is not a useful investigation when
orthostatic blood pressure recorded (this done routinely.
is abnormal if there is a decrease in Any abnormality found in these investiga- Management of syncope
systolic blood pressure of more than tions should then direct further investi- within the ED
20 mmHg between measurements taken gation and referral. For most children the diagnosis of vasovagal
in the supine and sitting or standing syncope will be made, and the majority of
position). Cardiac auscultation and a these will be able to be discharged well from
12-lead ECG should be performed on all Outpatient investigations the ED. These patients must be given advice
patients. of syncope regarding the precipitants and management
The level of consciousness, posture and Consider referral and further investigation of vasovagal syncope. Avoidance of usual
mental state of the child should be noted, for any child with an atypical history or precipitating events is important. Children
and pupils and fundi should be examined. abnormal examination, or with severe or fre- can also be taught to recognise the typical
The childs temperature should be quent vasovagal syncope (Table 5.3.2). prodrome of the event, and to then attempt
recorded. Any child in whom a cardiac cause of syn- to prevent any loss of consciousness by sitting
The history and the initial assessment cope is either suspected or diagnosed must or lying with their feet elevated. They should
should direct further examination of the be referred to a cardiologist. It may be be discharged into the care of a capable adult.
child. In addition to searching for clues to appropriate to arrange secondary investi- Carers of children with a diagnosis of either
the cause of the syncope, it is important gations such as an echocardiogram or reflex anoxic seizures or blue breath-holding
to examine the patient for any injuries Holter monitoring prior to discharge from spells should be educated and reassured
sustained during the event. the ED after discussion with a paediatric prior to discharge.

98
5.4 CARDIOVASCULAR ASSESSMENT AND MURMURS
5

CARDIOVASCULAR
Children seen with hyperventilation syn- and examination are essential in making Breningstall GN. Breath-holding spells. Paediatr Neurol
1996;14:917.
cope may require treatment by rebreathing. the correct diagnosis. All children who pres- Fleisher GR, Ludwig S, editors. Textbook of paediatric
They should be encouraged to breathe ent with their first episode of syncope should emergency medicine. 4th ed. Philadelphia: Lippincott
Williams & Wilkins; 2000.
slowly and regularly, with a paper bag held have a 12-lead ECG. Hannon DW, Knilans TK. Syncope in children and adolescents.
over their mouth and nose. Once their If there is an atypical history or if there are Curr Probl Paediatr 1993;23:35884.
Johnsrude CL. Current approach to paediatric syncope.
breathing is regular and their symptoms any abnormalities in the clinical examina- Paediatr Cardiol 2000;21:52231.
improved, they should be encouraged to tion the child must be investigated and Lewis DA, Dahla A. Syncope in the paediatric patient. Pediatr
Clin N Am 1999;46:20519.
be calm, and to sit or lie down for a time. referred appropriately. McLeod KA. Dysautomnia and neurocardiogenic syncope.
Both the children and their carers should For children in whom a diagnosis of vaso- Curr Opin Cardiol 2001;16:926.
McLeod KA. Syncope in childhood. Arch Dis Child
be taught the technique prior to discharge. vagal syncope is made, education and reas- 2003;88:3503.
Management of all other children should surance of both the child and their carers Narchi H. The child who passes out. Paediatr Rev 2000;21:3848.
Prodinger RJ, Reisdorff EJ. Syncope in children. Emerg Med
be appropriate for their particular clinical must be done prior to discharge from the ED. Clin N Am 1998;16:61726.
condition. Reuter D, Brownstein D. Common emergent paediatric
neurological problems. Paediatr Emerg Med
2002;20:15576.
Seifer CM, Kenny RA. Head-up tilt testing in children.
Summary Further reading Eur Heart J 2001;22:196870.
Strange GR, et al., editors. Paediatric emergency medicine.
Braden DS, Gaymes CH. The diagnosis and management of A comprehensive study guide. American College of
The most common cause of childhood syn- syncope in children and adolescents. Pediatr Ann Emergency Physicians. New York: McGraw-Hill; 1999.
cope is vasovagal syncope. Careful history 1997;26:4226.

5.4 Cardiovascular assessment

and murmurs
Matthew OMeara

ESSENTIALS History
1 Cardiac problems are uncommon in children, but should be considered in an infant The onset of the symptoms caused by a car-
or child with respiratory distress, cyanosis, collapse or shock. diac problem will depend on the severity of
the haemodynamic disturbance. A child with
2 Key requirements on examination are palpation of the peripheral pulses and liver congenital heart disease may present at
and auscultation of the heart. birth with cyanosis, with symptoms related
3 Murmurs are commonly found on examination. to cardiac failure at days to months of age
or with a murmur heard incidentally during
4 Children with murmurs should be referred if they have: examination. A child with an acquired car-
diac problem may present at any age.
Symptoms which may indicate cardiac disease (e.g. breathlessness, cyanosis,
chest pain) The timing and onset of symptoms should
be carefully noted. Babies with cardiac failure
Abnormalities of the heart sounds (e.g. fixed splitting of the second heart sound) may present with breathlessness, feeding dif-
ficulties, inability to complete feeds and poor
A murmur that cannot be confidently identified as innocent
weight gain. If cyanosis is described, it is
A murmur with a thrill grade 4 intensity or greater. important to determine whether it is persis-
5 Chest X-ray and ECG may help in suspected structural disease but are unlikely to be tent or intermittent and its relationship to
helpful in an asymptomatic child with a murmur. crying, feeding and activity. Normal infants
may appear peripherally cyanosed when cold
or febrile.
Details about the pregnancy are relevant
for infants with cardiac problems. There are
Introduction (see Chapter 5.5). Acquired diseases associations with maternal diabetes (struc-
Approximately 1% of infants and chil- include myocarditis, pericarditis, cardio- tural heart disease, transient cardiomyopa-
dren in developed countries have congeni- myopathies and coronary vascular disease thy) and with maternal lupus (congenital
tal cardiac problems. The majority are such as Kawasaki disease (see Chapters heart block). Teratogenic drugs during preg-
from congenital cardiac abnormalities 5.65.8). nancy may cause heart disease, for instance

99
5.4 CARDIOVASCULAR ASSESSMENT AND MURMURS

alcohol (atrial septal defect (ASD), ventricular paediatric examinations, tact and patience The apex beat should be located and
septal defect (VSD)), amphetamines (VSD, are often required to maintain co-operation palpated for thrills. The presence of a thrill
persistent ductus arteriosus (PDA), ASD, and elicit the signs accurately. Infants and indicates that the murmur is pathological.
transposition of the great arteries (TGA)) lith- young children may be most settled in a Auscultation of the heart starts with lis-
ium (Ebsteins abnormality), retinoic acid parents lap and occupied with a quiet toy tening for the heart sounds, especially
(conotruncal abnormalities) and valproic acid or feeding. One may have to be flexible splitting of the second heart sound. In chil-
(ASD, VSD, aortic stenosis (AS), coarctation of about the order of examination, taking the dren, splitting is usually only audible during
the aorta (CoA)). Infections during pregnancy opportunity to auscultate during quieter inspiration at the upper left border of the
may also be implicated. Rubella is associated moments. sternum. The absence of variation between
with PDA and peripheral pulmonary artery A general assessment of the child comes inspiration and expiration (fixed splitting)
stenosis. Perinatal events such as fetal dis- first. Note whether the child appears well, occurs in atrial septal defects. Third heart
tress and asphyxia may cause an ischaemic has any dysmorphic features and assess sounds are heard in 20% of normal children.
insult and cardiomyopathy. whether growth is appropriate for age. Look Listen for clicks in early systole (in aortic and
A family history of congenital heart dis- at the tongue and mucous membranes for pulmonary stenosis).
ease or a sibling who died suddenly without cyanosis. Central cyanosis is generalised; Murmurs should be assessed with regard
clear cause found at post-mortem (e.g. undi- peripheral cyanosis occurs in areas of poor to their:
agnosed QT syndrome), may be important. tissue perfusion, which are usually cold to
Most congenital cardiac defects are multi- touch. Examine the fingers for clubbing.
timing systolic, diastolic or continuous;
factorial and the risk of another sibling The peripheral pulses should be exam-
localisation the point of maximum
intensity;
being affected is around 13%. Several dis- ined. Assess the rate, rhythm and character
eases have an autosomal dominant pattern of the pulse. Compare the resting pulse rate
loudness increasing loudness from
grades 1 to 6;
of inheritance, including hypertrophic to normal ranges for age (see Chapter 1.1).
obstructive cardiomyopathy (HOCM), supra- Variation of the heart rate with respiration
character ejection, pansystolic, early/
mid/late diastolic;
valvular aortic stenosis, Marfans syndrome, (sinus arrhythmia) in children is more
idiopathic mitral valve prolapse and some marked than in adults. The character of
radiation audible in areas away from
precordium.
cases of ASD and long QT. the pulse may change with a cardiac defect,
The onset of features of cardiac disease surgical treatment or cardiac failure. Bound- Auscultation of the chest should note air
varies with the type of lesion. Most neonates ing pulses are often found in febrile chil- entry and presence or absence of crackles.
with congenital heart disease are asymptom- dren without heart disease but may be Abdominal palpation should pay particu-
atic at birth. Infants with duct-dependent left- associated with patent ductus arteriosus lar attention to the liver span, edge and
sided obstructive lesions usually present in or a systemic-pulmonary shunt for palliative presence of pulsation.
the first 2 weeks of life as the ductus arterio- treatment of cyanotic heart disease with At the completion of the cardiovascular
sus closes. Cardiac output falls and shock decreased pulmonary blood flow. Reduced examination one should be able to deter-
develops. Infants with left-to-right shunting volume or delay of the femoral pulses com- mine whether the child is cyanosed or not
usually present after 4 weeks of age when pared with the right brachial pulse suggests and whether the child has cardiac failure.
pulmonary resistance has decreased and coarctation of the aorta. Diffusely small The pulses, blood pressure and precordial
heart failure develops. pulses are associated with low-output car- findings assist with the assessment of the
The pattern of breathing may provide diac failure or shock. type of cardiac problem, even if a specific
clues. Increased work of breathing and Blood pressure is a routine part of the car- diagnosis is not possible. A chest X-ray and
grunting suggest left-sided obstructive diovascular examination in children. A cuff electrocardiograph (ECG) may assist.
lesions or respiratory illness. Effortless of the correct size should be wide enough
tachypnoea may be found with cyanotic to cover two-thirds of the length of the
heart disease (see Chapter 1.1). upper arm, be centred over the artery and Chest X-ray
Other features in history which suggest a have a bladder encircling at least two-thirds
The chest X-ray is good at detecting volume
cardiac cause, include recurrent respiratory of the circumference of the upper arm. In
overload, but less useful for hypertrophy
infections, exercise tolerance, chest pain general, fit the biggest cuff possible without
without dilatation. Key features to assess
and episodes of palpitations or syncope. covering the cubital fossa.
on the chest X-ray are:
Chest examination starts with looking
at the rate and work of breathing and patient name and date;
Physical examination
comparing the respiratory rate to normal cardiac position (dextrocardia, situs
ranges. Evidence of previous surgery inversus);
Examining a childs cardiovascular system is includes a sternotomy scar or less visible quality of the film centring, penetration,
similar to examining that of an adult, but thoracotomy scar (from repair of coarcta- degree of inspiration;
particular attention should be paid to palpa- tion, patent ductus arteriosus, pulmonary cardiothoracic ratio (usually less than
tion of the peripheral pulses and careful artery banding or insertion of systemic- 50% but up to 60% in AP films in
auscultation of the heart. As with most pulmonary shunt). neonates);

100
5.4 CARDIOVASCULAR ASSESSMENT AND MURMURS
5

CARDIOVASCULAR
cardiac contour especially size of Recognising common innocent murmurs
pulmonary artery and position of the aorta; enables exclusion of organic heart disease
lung fields vascularity. and the need for unnecessary investigation
and referral.
In acyanotic heart disease with left-to-right
There are four characteristic types of
shunting there may be increased pulmonary
innocent murmurs
vascularity.
In cyanotic heart disease, increased pulmo- Vibratory murmur.
nary vascularity occurs in truncus arteriosus, Pulmonary flow murmur.
totally anomalous pulmonary venous drain-  Carotid bruit.
age (TAPVD) and TGA. Decreased pulmonary Venous hum.
vascularity occurs with pulmonary atresia,
The vibratory murmur (Stills murmur) is a
tricuspid atresia, Ebsteins anomaly, tetralogy
short mid-systolic murmur best heard at
of Fallot and critical pulmonary stenosis. A the left sternal border or between the apex
and the left sternal border. The murmur is of
Electrocardiography medium frequency and has a slight musical
The ECG is good at detecting hypertrophy and or vibratory character. It is often softer when
therefore conditions with pressure overload. the child stands or extends the neck and is
The rate, rhythm and axis should be louder when lying supine.
assessed. Normal QRS axis varies with age. The pulmonary flow murmur is a soft
At birth, the range is from 60 to 180 , blowing murmur maximal in intensity in
at 1 year 1 to 100 , at 10 years 1 to 130 . the pulmonary area. Murmurs in this loca-
Systematically look at the P waves, PR tion are common in young children, but
interval, QRS complexes, ST interval and are often difficult to differentiate from an
T waves. atrial septal defect or pulmonary stenosis,
so may require assessment by a specialist.
Atrial enlargement
A carotid bruit produces a rough ejection
Right atrial enlargement peaked P wave
systolic murmur of medium frequency heard
with height of >2.5 mm (Fig. 5.4.1).
B over the base of the neck, but is much softer
Left atrial enlargement P wave >0.08 s, below the clavicle.
may also be plateau or notched Fig. 5.4.2 Left atrial abnormality. Left atrial A venous hum causes a high-pitched,
(Fig. 5.4.2). abnormality/enlargement may produce the
blowing continuous murmur over the neck
following: A, wide, sometimes notched P waves in
Ventricular enlargement one or more limb leads (formerly referred to as or sternoclavicular junction. It disappears
Right ventricular hypertrophy (Fig. 5.4.3): P mitrale pattern); and/or B, wide biphasic P when the child lies flat or when the neck
waves in lead V1. From Goldberger: Clinical veins are gently compressed.
R greater than S in V1 after 1 year; Electrocardiography: A Simplified Approach, 7th
ed. 2006. Copyright # Mosby.
T wave upright in V1 after 1 week;
Disposition
SV6 greater than 15 mm at 1 week, Referral for specialist consultation is indi-
10 mm at 6 months, 5 mm at 1 year.
cated for a child with a murmur who has:
Left ventricular hypertrophy (Fig. 5.4.4).
symptoms that may indicate cardiac disease
SV1 rV6 greater than 30 mm to 1 year; (e.g. breathlessness, cyanosis, chest pain);
SV1 rV6 greater than 40 mm after 1 year. abnormalities of the heart sounds (e.g.
fixed splitting of the second heart sound);
a murmur that cannot be confidently
The child with an identified as innocent;
asymptomatic murmur a murmur with an associated thrill (grade
4 intensity or greater).
Murmurs may be heard in around half of nor-
mal school-aged children and are heard even
Fig. 5.4.1 Right atrial abnormality. Tall narrow more frequently in infants. Many murmurs are
P waves may indicate right atrial abnormality or Investigations
innocent and can be recognised by their spe-
overload (formerly referred to as P pulmonale
pattern). From Goldberger: Clinical cific characteristics. Innocent murmurs occur ECG and chest X-ray are readily available,
Electrocardiography: A Simplified Approach, 7th when there is normal or increased blood but are poor screening tests for children with
ed. 2006. Copyright # Mosby. flow through a normal heart and vessels. asymptomatic murmurs.

101
5.4 CARDIOVASCULAR ASSESSMENT AND MURMURS

I II III aVR aVL aVF

V1 V2 V3 V4 V5 V6

Fig. 5.4.3 Right ventricular hypertrophy. A tall R wave with an inverted T wave caused by right ventricular overload is seen in lead V1 from a patient with
tetralogy of Fallot. Marked right axis deviation is also present. (The R wave in lead III is taller than the R wave in lead II.) From Goldberger: Clinical
Electrocardiography: A Simplified Approach, 7th ed. 2006. Copyright # Mosby.

I II III

aVR aVL aVL

V1 V2 V3

S=
22 mm

V4 V5 V6

R=
26 mm

Fig. 5.4.4 Left ventricular hypertrophy. Tall voltages are seen in the chest leads and lead aVL (R 17 mm). A repolarization (ST-T) abnormality (arrow), formerly
referred to as a strain pattern, is also present in these leads. In addition, enlargement of the left atrium is indicated by a biphasic P wave in lead V1 and a broad,
notched P wave in lead II. From Goldberger: Clinical Electrocardiography: A Simplified Approach, 7th ed. 2006. Copyright # Mosby.

102
5.5 CONGENITAL HEART DISEASE
5

CARDIOVASCULAR
The ECG is unlikely to disclose any unsus- reproducibility. Clinical assessment by
pected heart disease, but may assist in a paediatrician or cardiologist correctly iden- Further reading
reaching the specific diagnosis when there tifies almost all murmurs as innocent or Park MK. Pediatric cardiology for Practitioners. 5th ed.
Philadelphia: Mosby; 2008.
is underlying pathology. needing further investigation. Echocardio-
Similarly, screening asymptomatic chil- graphy is not necessary in children identified
dren with a heart murmur chest X-ray is lim- by a specialist as having an innocent
ited by poor sensitivity, specificity and murmur

5.5 Congenital heart disease

Matthew OMeara

resistance. In general, the more severe the

ESSENTIALS anatomic defect is (i.e., lack of pulmonary
blood flow or lack of systemic blood flow),
1 Congenital cardiac disease is uncommon in children, but should be considered as a the earlier in life these conditions will mani-
possible diagnosis in an infant or child with respiratory distress, cyanosis or shock.
fest with cyanosis and shock.
2 The common congenital cardiac problems have characteristic clinical features that The possibility of a congenital cardiac prob-
assist with clinical diagnosis and initial management. lem needs consideration in a child presenting
with cyanosis, respiratory distress or shock.
3 The radiologic appearance of pulmonary blood flow and electrocardiographic There are a number of general principles that
features of axis deviation and ventricular hypertrophy assist the diagnostic
may indicate a congenital cardiac problem.
evaluation.
Is the child centrally cyanosed?
 central cyanosis with normal breathing
and chest radiograph will help the diagnostic or only mild respiratory distress
Introduction process (Figs 5.5.1 and 5.5.2). suggests a cardiac problem rather than
Incidence a primary respiratory problem;
Approximately 1% of infants and children in  cyanosis that worsens with crying
developed countries have congenital cardiac Clinical features suggests a cardiac cause;
problems. Eight lesions account for 80% of The age, severity of symptoms, and time of  cyanosis that persists with oxygen
all cases of congenital cardiac abnormalities: presentation of a child with congenital heart suggests a cardiac cause.
ventricular septal defect (VSD); patent duc- disease (CHD) vary depending on the spe- Does the heart sound normal?
tus arteriosus (PDA); atrial septal defect cific defect, complexity and severity of the  Is there a murmur that cannot be
(ASD); tetralogy of Fallot; pulmonary steno- defect, and timing of the normal physiologi- confidently identified as innocent?
sis; coarctation of the aorta; aortic stenosis cal changes that occur as the fetal circula- Is the chest radiograph normal?
(AS); and transposition of the great arteries. tion transitions to that of a neonate. The  Is the heart shape abnormal?
more severe or complex CHD lesions may  Is the pulmonary vascularity increased
not be clinically apparent immediately after or decreased?
birth. However, as the ductus arteriosus Is the electrocardiograph abnormal?
Common heart defects in infancy begins to close in the first few weeks of life,  Is the axis abnormal?
and childhood cardiac defects with obstructive lesions of  Is there atrial or ventricular
Heart disease in children may present with the pulmonary or systemic circulations will hypertrophy?
the presence of an abnormal murmur, the be unmasked, and these infants will present
development of symptoms or signs of conges- clinically with acute cyanosis, shock, or both.
tive cardiac failure or central cyanosis. From Even the harsh systolic murmur of a large,
Acyanotic defects
an Emergency Department perspective, it is isolated ventricular septal defect may not
more important to identify the degree of cya- be heard until about the 4th to 6th week These make up about 75% of all congenital
nosis or cardiac failure, rather than identify of life, when the left-to-right shunt across heart defects. They include those associated
the exact anatomic diagnosis. Clinical exami- the ventricular septal defect increases due with isolated left-to-right shunts such as
nation combined with the electrocardiograph to the decrease in the pulmonary vascular VSD, ASD, PDA and those without shunting

103
5.5 CONGENITAL HEART DISEASE

closure is indicated in cases where congestive

Pulmonary blood
flow cardiac failure develops in infancy or if pul-
monary hypertension is present. Repair at a
later age may be required if the defect fails
Increased Decreased or
left-to-right shunting normal to close or aortic valve prolapse develops.

Persistent ductus arteriosus

RVH LVH RVH LVH The ductus arteriosus, connecting the pul-
monary trunk with the aorta, usually closes
ASD VSD PS AS in the newborn period. It may remain open
PDA CoA in premature infants or if the ductus is con-
AVSD genitally abnormal.
Fig. 5.5.1 Diagnostic approach to acyanotic congenital heart defects. AS, aortic stenosis; ASD, atrial
The clinical features depend on the size of
septal defect; AVSD Atrioventricular septal defect; CoA, coarctation of the aorta; LVH, left ventricular the ductus. The only sign of a small ductus
hypertrophy; PDA, patent ductus arteriosus; PS, pulmonic stenosis; RVH, right ventricular hypertrophy; may be a continuous machinery murmur
VSD, ventricular septal defect. Adapted from Marx: Rosens Emergency Medicine, 7th ed. Copyright at the upper left sternal edge. The diastolic
# 2009 Mosby. component is often soft, creating the impres-
sion of a systolic murmur. With a large duc-
tus the pulses may be bounding, the apex
Pulmonary blood displaced and a mid-diastolic murmur audi-
flow
ble due to increased flow across the mitral
valve. An infant with a large PDA will fail
Increased Decreased to thrive, be tachypnoeic and may develop
recurrent chest infections.
In small defects the ECG and chest X-ray
are normal. Larger PDAs cause cardiome-
RVH LVH RVH LVH galy, pulmonary plethora and left ventricular
hypertrophy.
TGA TruncusA TOF TriA Premature infants with PDA may be suc-
TAPVR Severe PS PA cessfully treated with indomethacin. Early
HLHS
closure is indicated in symptomatic term
Fig. 5.5.2 Diagnostic approach to cyanotic congenital heart defects. BVH, biventricular hypertrophy; infants and premature infants following
EA, Ebsteins anomaly; HLHS, hypoplastic left heart syndrome; LVH, left ventricular hypertrophy; PA, unsuccessful medical treatment of a PDA.
pulmonary atresia; PS, pulmonary stenosis; RVH, right ventricular hypertrophy; TAPVR, total anomalous Closure may be delayed in an asymptomatic
pulmonary venous return; TGA, transposition of the great arteries; TOF, tetralogy of Fallot; TriA, tricuspid infant with a small ductus. Closure with cath-
atresia; TruncusA, truncus arteriosus. Adapted from Marx: Rosens Emergency Medicine, 7th ed. Copyright
eter may replace the traditional closure by
# 2009 Mosby.
ligation.

such as AS pulmonary stenosis (PS) and murmur at the left sternal border, often asso- Atrial septal defect
coarctation of the aorta. See Fig. 5.5.1 for ciated with a thrill. The heart sounds are nor- ASDs are most commonly situated in the
an approach to diagnosis based on radiolog- mal. Larger VSDs may cause a parasternal region of the fossa ovale and are called
ical and electrocardiographic appearance. heave, a displaced apex beat, a softer systolic secundum ASDs.
murmur and a diastolic murmur due to Isolated ASDs cause an ejection systolic
increased flow through the mitral valve. An murmur in the pulmonary area, due to
Ventricular septal defect infant with a large VSD will fail to thrive increased flow through the pulmonary valve.
VSDs make up 30% of all cardiac defects. The and become tachypnoeic, sweaty and tired A soft mid-diastolic murmur may be heard at
defect may be small or large and involve the with feeds. the lower sternum due to increased flow
membranous or muscular parts of the septum. The chest X-ray with small VSDs is normal. through the tricuspid valve. The pulmonary
Clinical presentation depends on the With larger defects there may be cardiome- component of the second heart sound is
amount of left-to-right shunting. A murmur galy and pulmonary plethora. The ECG often delayed, causing wide fixed splitting.
may be noted incidentally on examination has features of biventricular hypertrophy. Symptoms are mild. There may be mild
or congestive cardiac failure may develop in Many small VSDs decrease in size or close effects on growth and exercise tolerance.
infants with larger defects and more shunting. spontaneously. In untreated cases complica- Adults with unrecognised or unrepaired
The characteristic clinical features of a VSD tions of pulmonary hypertension and aortic ASD may develop atrial arrhythmias and
are a loud, harsh, high-pitched systolic valve prolapse may develop. Early surgical dyspnoea.

104
5.5 CONGENITAL HEART DISEASE
5

CARDIOVASCULAR
Chest X-ray shows increased cardiac size pulmonary component of the second heart upper and lower limb blood pressures of over
with pulmonary plethora. ECG shows partial sound is soft. An ejection click in early sys- 20 mmHg.
right bundle branch block. tole can be heard at the left sternal border In infants with cardiac failure the chest
Closure is recommended for all cases with with valvular stenosis. X-ray will show cardiomegaly with pulmo-
a significant shunt. Closure by catheter- The chest X-ray may show a bump on the nary congestion. In later years the X-ray
delivered device is suitable in selected cases. upper left heart border from poststenotic may show rib notching due to enlarged
dilatation of the pulmonary artery. The intercostal collateral vessels.
Atrioventricular septal defect ECG shows right ventricular hypertrophy in Infants with coarctation of the aorta
This group includes defects low on the atrial more severe cases. develop cardiac failure from closure of the
septum, abutting on the atrioventricular Most cases can be treated with balloon ductus arteriosus. In addition to supportive
valves and may also involve the ventricular dilatation. treatment, infusion of prostaglandin E1
septum. may help maintain patency of the ductus
Defects involving the atrial septum and Aortic stenosis in the collapsed shocked infant. Surgical
atrioventricular valves (AV) are known as In aortic stenosis the aortic valve is abnor- repair is urgently required. Patients with
ostium primum ASDs. The AV valves are usu- mal with thickened leaflets and fused com- milder degrees of coarctation require moni-
ally abnormal, particularly the left AV missures. The valve is often bicuspid. toring for development of hypertension.
(mitral) valve, which is cleft. Children with Left ventricular outflow tract obstruction
minor mitral regurgitation present with fea- may also occur due to hypertrophic subaortic Hypoplastic left heart
tures like a secundum ASD. Infants with stenosis and supravalvular aortic stenosis. Some infants with severe aortic stenosis or
severe mitral regurgitation present with con- Most children present with an incidentally coarctation of the aorta have associated
gestive cardiac failure. found murmur. Symptoms increase with age gross hypoplasia of the left ventricle. Aortic
When a significant ventricular defect to include syncope, angina and dyspnoea on and mitral valve atresia may also be
coexists (complete atrioventricular septal exertion and sudden death. Infants with crit- present.
defect), infants present with features similar ical aortic stenosis may present with severe Infants with hypoplastic left heart present
to a large VSD with congestive cardiac fail- cardiac failure. with shock in the first few days of life.
ure in early months. This is the commonest The murmur is ejection systolic, maximal Resuscitation and infusion of prostaglan-
cardiac defect associated with Downs at the right sternal edge and radiates to din E1 (alprostadil) may help support. Treat-
syndrome. the carotids. A thrill may be felt over the car- ment options include palliation and heart
The chest X-ray shows marked cardiome- otids, in the sternal notch or aortic area. An transplantation, although donors are rare.
galy and pulmonary plethora. ECG shows ejection click indicates valvular stenosis and
left axis deviation and partial right bundle is best heard at the apex. In more severe
branch block. cases a forceful apical impulse may be pres- Cyanotic cardiac defects
Surgical repair is required to repair the ent and the pulse pressure narrowed.
defects in the atrial and ventricular septa The ECG shows left ventricular hypertro- Cyanotic defects account for about one quar-
and AV. phy in more severe cases. ter of all congenital heart malformations.
Treatment options are open valvotomy or There are three main types of defects
Pulmonary stenosis balloon aortic valvuloplasty but this may cyanotic defects with reduced pulmonary
Pulmonary stenosis (PS) is the commonest worsen aortic incompetence. blood flow (e.g. tetralogy of Fallot), cyanotic
obstructive malformation. The pulmonary defects with bidirectional shunting (e.g.
valve is abnormal with thickened leaflets Coarctation of the aorta truncus arteriosus) and cyanosis with sepa-
and partially fused commissures. The valve There is a discrete narrowing of the aorta, ration of pulmonary and systemic circula-
may be bicuspid. near the site of the ductus arteriosus. There tions (e.g. transposition of the great
The right ventricular outflow tract may be may be associated intracardiac anomalies arteries). See Fig. 5.5.2 for an approach to
narrowed at other sites including infundibu- including bicuspid aortic valve, AS, VSD diagnosis based on radiographic and elec-
lar stenosis, supravalvular stenosis and and mitral valve abnormalities. trocardiographic features.
branch pulmonary stenosis. Coarctation may present in early weeks of
Usually pulmonary stenosis is evident as life with cardiac failure or collapse with pro- Tetralogy of Fallot
an asymptomatic murmur. Moderate steno- found shock. Milder cases may only become The four components of tetralogy of Fallot
sis may lead to dyspnoea and angina with evident with hypertension, a murmur or are PS, VSD, overriding aorta and right ven-
exercise and cardiac failure. More severe reduced femoral pulses in later years. tricular hypertrophy. The pulmonary steno-
cases may present in infancy with cyanosis Physical findings are reduced volume fem- sis is caused by infundibular muscular
due to right-to-left shunting through a pat- oral pulses. Delay of the femoral pulse com- obstruction, often with valvular hypoplasia
ent foramen ovale or ASD. pared to the brachial is evident only in later and commissural fusion, resulting in ele-
There is an ejection systolic murmur max- childhood after collateral vessels develop. vated right ventricular pressure. Although
imal in the pulmonary area, which radiates The upper limb blood pressure may be ele- right and left ventricular pressures are simi-
to the back. A thrill may be present. The vated and a discrepancy occurs between lar, the resistance to ejection into the

105
5.5 CONGENITAL HEART DISEASE

pulmonary circulation results in right-to-left The chest X-ray shows cardiomegaly and palliated cyanotic congenital heart defects.
shunting across the VSD into the aorta. pulmonary plethora. The ECG is often nor- Antibiotic prophylaxis should be carried
Cyanosis is often mild at birth and mal for age. out for procedures (see Chapter 5.7, Infec-
increases during infancy. Cyanosis is usually Surgical correction involves separating tive Endocarditis).
more obvious with crying or exertion. Hyper- the pulmonary artery from the truncus, con- Arrhythmias may develop in children
cyanotic spells due to reduced pulmonary necting it to the right ventricle with a valved with congenital heart defects, particularly
blood flow may occur and are characterised conduit and closing the VSD. Ebsteins anomaly of the tricuspid
by marked pallor or cyanosis with respiratory valve, mitral valve prolapse, and after
distress, particularly after exercise (see Transposition of the great repair of transposition of the great
Chapter 5.1). Children may have reduced arteries arteries with atrial switch and repair of
exercise tolerance and squat on the ground. In this condition the aorta and pulmonary tetralogy of Fallot (see Chapter 5.9, Cardiac
This manoeuvre increases systemic vascular arteries arise from the incorrect ventricles. Arrhythmias).
resistance to reduce right-to-left shunting. Deoxygenated systemic blood is directed Many patients who have undergone total
A systolic murmur due to infundibular ste- through the right side of the heart and back corrective surgery for left-to-right shunts
nosis is maximal at the left sternal edge and into the aorta. Oxygenated blood from the such as ASD, VSD or PDA will have few, if
pulmonary area, radiating through to the pulmonary veins is directed through the left any, haemodynamic problems.
back. The second heart sound is often loud side of the heart and back into the pulmo- Children with repaired coarctation of the
and single as the sound of pulmonary valve nary circulation. Survival is possible only if aorta may develop hypertension and reste-
closure is inaudible. there are flows between each independent nosis. Late problems include coronary artery
In untreated cases cyanosis gradually circuit through a foramen ovale, ductus disease, congestive cardiac failure and rup-
increases, resulting in decreased exercise tol- arteriosus or septal defect. tured aortic aneurysms.
erance, clubbing and growth retardation. Cyanosis is marked from birth. There may Aortic stenosis often progresses with age.
Polycythaemia and thromboembolic compli- be a forceful right ventricular impulse but A gradient over 50 mmHg increases the risk
cations may occur as well as endocarditis. often no murmur. Metabolic acidosis may of arrhythmias, syncope, sudden death,
The chest X-ray shows a normal-sized heart develop due to tissue hypoxia. endocarditis and angina.
with reduced pulmonary vascularity. The The chest X-ray shows a normal or slightly Patients with untreated VSD or atrioven-
heart shape in severe cases has uptilting of enlarged heart with increased pulmonary vas- tricular septal defect may develop Eisen-
the apex, resulting in a boot-shaped heart. cular markings. The abnormal arrangement menger syndrome with associated cyanosis
ECG shows right ventricular hypertrophy. of the great vessels gives the appearance of and polycythaemia. Patients with palliated
Total surgical repair may be possible in an egg on its side. The ECG is normal or cyanotic congenital heart defects and
infancy if the pulmonary valve and vessels may show T-wave changes. patients with Eisenmenger syndrome should
are suitable. If not, palliation to increase pul- An urgent balloon atrial septostomy may avoid situations in which dehydration may
monary blood flow can be achieved with a be performed to increase interatrial shunt- occur and lead to increased viscosity and risk
synthetic shunt between aorta and pulmo- ing. Surgical correction with an arterial of stroke. During illnesses such as gastroen-
nary artery (modified BlalockTaussig shunt). switch operation is usually performed in teritis, diuretics may need to be decreased or
early infancy. This also involves transferring temporarily discontinued in addition to
Persistent truncus arteriosus the coronary arteries to the new aorta. An ensuring adequate hydration status. High
In this rare defect, a single artery arises from alternative is an intra-atrial patch (atrial altitude, airline travel and sudden exposure
the heart and branches into pulmonary switch) to redirect blood flow at an atrial to changes in environmental temperatures
artery and aorta. The truncal valve sits level (Mustard or Senning procedure). should also be avoided.
across a large VSD and receives blood from
both ventricles.
Cyanosis is mild and congestive heart fail-
Long-term complications of
ure appears in the newborn. Systolic and dia- Further reading
stolic murmurs are usually heard from flow
congenital heart disease Park MK. Pediatric cardiology for Practitioners. 5th ed.
Philadelphia: Mosby; 2006.
through the truncal valve, which is often Bacterial endocarditis remains a risk with Rosens Emergency Medicine. Concepts and Clinical Practice.
incompetent. many congenital cardiac defects, especially 7th ed. Mosby; 2009.

106
 5

CARDIOVASCULAR
5.6 Acute rheumatic fever
Sarah Dalton

the presence of three minor manifestations.

ESSENTIALS All diagnoses require evidence of preceding
Group A streptococcal (GAS) infection.
1 Acute rheumatic fever (ARF) is a disease of connective tissue inflammation that
may follow 2 to 4 weeks after a Group A streptococcal throat infection.
2 Diagnosis is based on the presence of carditis, arthritis, skin and neuropsychiatric
sequelae. Carditis
3 The major complication of recurrent disease is progressive destruction of cardiac A pancarditis, manifested as endocarditis,
valves leading to rheumatic heart disease. myocarditis or pericarditis, occurs in nearly
50% of cases of ARF. Clinical diagnosis
4 Treatment is comprised of eliminating streptococci, controlling joint pain and begins with the identification of a new or
managing carditis, heart failure and chorea. changing murmur that must be distin-
5 Antibiotic prophylaxis to prevent further attacks of ARF and episodes of infective guished from an innocent murmur in a
febrile child. The mitral valve is most com-
endocarditis is mandatory.
monly affected, followed by the aortic valve.
Myocarditis may be associated with a dispro-
History portionate sinus tachycardia or rhythm dis-
Introduction Acute rheumatic fever usually presents in turbance, such as first degree heart block.
school-age children but may present at Signs of pericarditis or congestive heart fail-
Acute rheumatic fever (ARF) is an acute
any age. A history of pharyngitis several ure may be present. Recurrent rheumatic
inflammatory disease that may follow group
fever leads to progressive valve damage
A b-haemolytic streptococcal infection. It weeks previously is found in 70% of older
children, but younger children are less likely and RHD.
primarily affects connective tissue, causing
carditis, arthritis and chorea and may follow to recall a sore throat. Typically, ARF pre-
a remitting and relapsing course for several sents with up to a week of high fevers,
years after the primary episode. Long-term followed by several weeks of milder tem-
complications of recurrent disease include peratures and the appearance of a rash (ery-
Polyarthritis
progressive cardiac damage, which is asso- thema marginatum). Specific symptoms The most common and earliest manifesta-
ciated with significant morbidity and mortal- include migratory joint pain, chorea, and tion of ARF is polyarthritis or arthralgia. In
ity in the adult population. neuropsychiatric manifestations such as up to one third of cases it may be the single
abrupt personality change or poor attention presenting problem. Generally, large joints
Epidemiology span. There may be symptoms of congestive are involved, beginning in the lower extremi-
Rheumatic heart disease (RHD) is steadily heart failure or non-specific symptoms such ties. The pain is often asymmetric, migratory
decreasing in the developed world in associ- as weight loss, fatigue, pallor, headache and and shows a marked response to aspirin. In
ation with improved standards of living and abdominal pain. most cases pain resolves within a week
the availability of penicillin. Amongst devel- and virtually never results in permanent
oping countries prevalence is variable, with joint deformity.
one of the highest prevalences worldwide Examination
found in the indigenous Australian commu- The diagnosis of ARF relies upon the identi-
nities. A family history of ARF is common, fication of specific clinical features. The
reflecting genetic predisposition as well as National Heart Foundation of Australia
has developed diagnostic criteria which
Skin
environmental influences.
depend on the stratification of patient risk Erythema marginatum
Pathophysiology (Table 5.6.1). High-risk groups are those Erythema marginatum is a distinctive fea-
The exact pathogenesis of ARF remains who live in communities with high rates of ture of rheumatic fever, but is only found
unclear. It is postulated that certain viru- ARF or RHD such as Aboriginal and Torres in approximately 10% of patients. Lesions
lent strains of Gram-positive Group A Strait Islanders. usually begin as small, pink, non-pruritic
b-haemolytic streptococci (Streptococcus An initial episode of ARF may be diag- macules or papules on the trunk or limbs.
pyogenes) invade the pharynx, leading to nosed where two major or one major and The lesions gradually spread to develop a
the production of anti-streptococcal antibo- two minor manifestations are present. The raised pink serpiginous edge with central
dies, which cross-react with host connective same criteria may be used to diagnose a clearing and remain late into the course of
tissue. recurrent episode, which otherwise requires illness (Fig. 5.6.1).

107
5.6 ACUTE RHEUMATIC FEVER

Imaging
Table 5.6.1 Diagnostic criteria for acute rheumatic fever
To further evaluate ventricular and valvular
High-risk group All other individuals morphology and function a chest X-ray and
Major manifestations echocardiography are indicated. Echocardi-
Carditis (including subclinical echocardiograph Carditis (excluding subclinical echocardiograph ography has an important role in the recog-
evidence) evidence) nition of carditis, which may be clinically
Polyarthritis, aseptic monoarthritis, Polyarthritis
or polyarthralgia Erythema marginatum undetectable in up to one half of cases.
Erythema marginatum Subcutaneous nodules
Subcutaneous nodules Chorea
Chorea Differential diagnosis
Each clinical manifestation of ARF is asso-
Minor manifestations
Fever (documented >38 C) 
Fever (documented >38 C)  ciated with multiple alternative diagnoses,
ESR >30 mm hr1 or CRP >30 mg L1 ESR >30 mm hr1 or CRP hence the need for strict diagnostic criteria.
Prolonged PR interval on ECG >30 mg L1
Prolonged PR interval on ECG
Sydenhams chorea is a diagnosis of exclu-
Polyarthralgia or aseptic monoarthritis sion after eliminating other causes of move-
ment disorder such as drug toxicity, systemic
Amended from National Heart Foundation of Australia; Diagnosis and management of acute rheumatic fever and
rheumatic heart disease in Australia an evidence-based review. 2006. lupus erythematosus (SLE), and Wilsons dis-
ease. Erythema marginatum is not specific
for ARF, also being found in sepsis, glomeru-
Subcutaneous nodules lonephritis and some drug reactions. Subcu-
improve in 12 weeks and resolve completely taneous nodules are also seen in rheumatoid
Subcutaneous nodules are an uncommon but
over 23 months, although the course can be arthritis. Several systemic inflammatory con-
highly specific manifestation of ARF. They are
variable. The presence of chorea is sufficient ditions such as SLE and juvenile arthritis may
firm, non-tender nodules found over the
for the diagnosis of ARF without other man- fulfil all ARF diagnostic criteria and must be
extensor surface of the elbow, metacarpopha-
ifestations, providing differential diagnoses excluded.
langeal joints, knees, and ankles. Up to three
have been excluded.
or four can appear in the first weeks of illness
and may remain for up to a month.
Investigations Treatment
Laboratory Acute management
Evidence of GAS infection Children who present with features of ARF
Neuropsychiatric sequelae
Preceding GAS infection should be demon- should be admitted to hospital under a paedi-
Sydenhams chorea strated using a positive throat culture, rapid atrician for further evaluation and manage-
Chorea occurs in up to 30% of patients with antigen detection test or elevated or rising ment. Management priorities are as follows:
ARF, and is postulated to be due to cross- anti-streptococcal antibody titre. Antibodies
reactivity between anti-streptococcal antibo- rise in the first month post-infection, plateau 1. Group A Streptococcus
dies and basal ganglia neurones. It presents at 36 months, and normalise after 612 eradication
with jerky, purposeless movement of limbs, months (Table 5.6.2). Following identification, GAS must be elimi-
speech impairment, involuntary grimacing Acute phase reactants such as the nated to prevent ongoing antibody forma-
or emotional lability, and may appear several erythrocyte sedimentation rate (ESR) and tion. The National Heart Foundation of
months after the original GAS infection. C-reactive protein (CRP) should be measured Australia recommends phenoxymethylpeni-
Movements may be asymmetric and usually in all cases of possible ARF. cillin 10 mg kg1 up to 500 mg po q12h
disappear during sleep. Generally symptoms for 10 days. Poorly compliant patients or
ECG those intolerant of oral therapy may receive
An ECG should be performed to identify peri- single dose benzathine benzylpenicillin
carditis and first-degree heart block. 450 mg intramuscular (IM) (<20 kg) or

Table 5.6.2 Upper limits of normal streptococcal serology

Age group Anti-streptolysin titre Anti-deoxyribonuclease B titre

(ASOT) (IU mL1) (Anti-DNase B) (IU mL1)

4 to 5 years 120 100

6 to 9 years 480 400

Fig. 5.6.1 Erythema marginatum. From Cohen 10 to 14 years 320 380
& Powderly, Infectious Diseases, 2nd ed.,
Copyright # 2004 Mosby, An Imprint of Amended from National Heart Foundation of Australia; Diagnosis and management of acute rheumatic fever and
Elsevier with permission. rheumatic heart disease in Australia an evidence-based review. 2006.

108
5.6 ACUTE RHEUMATIC FEVER
5

CARDIOVASCULAR
900 mg IM (>20 kg). Erythromycin may be National Heart Foundation of Australia involvement, congestive heart failure or
substituted in cases of penicillin sensitivity. recommends benzathine benzylpenicillin pericarditis was present at diagnosis.
450 mg IM (<20 kg) or 900 mg IM
2. Control of pain and inflammation (>20 kg) every 4 weeks (or 3 weeks for
High-dose aspirin, 80100 mg kg1 day1 in selected high-risk groups). Oral phenoxy- Controversies
four divided doses, is indicated for control of methylpenicillin 250 mg q12h may be used The role of echocardiography to diagnose
pain and inflammation. It should not be com- but is associated with poorer compliance ARF in the absence of a murmur is
menced prior to definitive diagnosis, as discrim- and efficacy. In cases of penicillin sensitivity controversial. It can be difficult to
inating clinical manifestations may be masked. alternatives include oral erythromycin. Dura- distinguish the difference between
tion of therapy should be a minimum of 10 echocardiographic features of pre-existing
3. Treatment of carditis and control years after the most recent episode of ARF or rheumatic heart disease and acute
valvulitis. For this reason the National
of heart failure until age 21 years. Prolonged therapy may
Heart Foundation of Australia excludes
Prednisone may be indicated for severe cardi- be required for moderate to severe RHD
subclinical echocardiographic changes in
tis, but its use is controversial. Recommended and should be discussed with experts. Endo-
diagnostic criteria unless the patient is
dosage is 2 mg kg1 day1 continued for carditis prophylaxis is mandatory for those from a high-risk group.
13 weeks. Antifailure medication such as with residual valve disease (see Chapter
angiotensin-converting enzyme inhibitors or 5.7 on Infective Endocarditis).
corrective valve surgery may be required,
depending on the clinical situation.
Further reading
4. Management of chorea Prognosis Carapetis JR, Brown A, Wilson NJ, Edwards KN, Rheumatic
Fever Guidelines Writing Group. An Australian guideline for
ARF resolves in most patients by 3 months.
A variety of antipsychotics and anti-epilep- rheumatic fever and rheumatic heart disease: an abridged
Complete spontaneous healing of valvulitis outline. Med J Aust 2007;186(11):5816.
tics have been used to control Sydenhams Currie BJ, Brewster DR. Rheumatic fever in Aboriginal
occurs in up to 80% of cases receiving
chorea. Carbamazepine or valproic acid are children. J Paediatr Child Health 2002;38(3):2235.
prophylactic antibiotic therapy. Recurrent National Heart Foundation of Australia (RF/RHD guideline
commonly recommended, but their use is development working group) and the Cardiac Society of
episodes of rheumatic fever are more com-
not universal and management should be Australia and New Zealand. Diagnosis and management of
mon in younger patients and usually occur acute rheumatic fever and rheumatic heart disease in
undertaken with specialist consultation. Australia an evidence-based review. 2006.
within 5 years. Prognostic indicators for Steer AC, Carapetis JR, Nolan TM, Shann F. Systematic review
chronic valve disease include total number of rheumatic heart disease prevalence in children in
developing countries: The role of environmental factors.
of rheumatic fever episodes and time delay
Prevention and prophylaxis between onset of GAS and antibiotics in
J Paediatr Child Health 2002;38(3):22934.
Stollerman GH. Rheumatic fever. Seminar. Lancet 1997;349
(9056):93542.
Continuous anti-streptococcal prophylaxis is each instance. Of all patients with initial Therapeutic Guidelines: Rheumatology, version 1.
recommended in all patients with a docu- mitral valve insufficiency, up to 30% have Therapeutic Guidelines Limited; 2006.
Wilson NJ, Neutze JM. Echocardiographic diagnosis of
mented history of ARF to prevent recur- evidence of RHD at follow up. This number subclinical carditis in acute rheumatic fever. Int J Cardiol
rence with subsequent GAS infections. The increases substantially where aortic valve 1995;50:16.

109
 5.7 Infective endocarditis
Sarah Dalton

deficit or other embolic phenomena should

ESSENTIALS be evaluated for endocarditis. Systemic
embolisation occurs in up to 50% of
1 Infective endocarditis is the microbial infection of cardiac endothelium. patients with IE, and is most frequently seen
2 Risk factors include congenital heart disease, central venous access, intravenous in the first month post-diagnosis. Embolic
drug use and immunodeficiency. events may cause infarction or abscess for-
mation in the brain, lungs, kidneys, spleen,
3 Diagnosis is based on the presence of positive blood cultures with evidence of bone and extremities. Peripheral embolisa-
carditis, peripheral embolisation and immunological phenomena.
tion is often absent in acute endocarditis
4 Acute management includes elimination of causative organisms and treatment of and right-sided heart disease, making their
associated complications such as congestive heart failure. diagnosis more difficult. Endocarditis may
also present with cardiac failure secondary
5 Long-term chemoprophylaxis is recommended for procedures likely to result in to acute valvular dysfunction, fistulous tract
bacteraemia in at-risk patients.
formation or prosthetic dehiscence.

Extracardiac manifestations result from

Introduction Examination
peripheral embolisation of thrombus material
Infective endocarditis (IE) is the microbial with subsequent infarction or infection of The physical signs of endocarditis may be sub-
infection of the endothelial lining of the involved tissue. Mycotic aneurysms are sec- tle. A new or changing murmur may signify
heart. Infection may be bacterial or fungal, ondary to bacterial embolisation causing valvulitis, but can be difficult to distinguish
and may arise in both structurally normal infection and distension of the arterial wall from an innocent murmur or that of a pre-
and abnormal hearts. Presentation can be and are most common in intracranial arteries. existing cardiac abnormality. Other findings
acute or subacute and the long-term morbid- include congestive heart failure or evidence
ity and mortality is high. of peripheral embolisation such as Osler
Microbiology nodes (tender, red nodules of finger pulps),
Streptococcus viridans and Staphylococcus Janeway lesions (non-painful, haemorrhagic
Epidemiology areas of palms or soles) and splinter and sub-
aureus are the most frequently encountered
Over the last decade the incidence of IE has organisms in children with IE. Less common ungual haemorrhages. These stigmata, which
increased owing to the improved survival of aetiological agents include enterococci, Staph- develop late in the course of disease, are
children with congenital heart disease and ylococcus epidermidis and Gram-negative unusual in children. Roth spots (retinal hae-
an increase in patients with central venous HACEK bacilli Haemophilus, Actinobacillus, morrhages with a pale centre) are occasion-
catheters. Up to 75% of children with bacterial Cardiobacterium, Eikenella and Kingella ally seen on funduscopy. Neurological deficit
endocarditis are known to have pre-existing species. Fungal species such as Candida and arises in 3040% of patients from embolic
cardiac abnormalities, most commonly ventric- Aspergillus may cause IE in neonates, immu- infarcts, abscesses or intracerebral haemor-
ular septal defect and tetralogy of Fallot. Other nocompromised patients and those with rhage. Associated findings include spleno-
risk factors include acquired valve disease, long-term central venous lines. Other agents megaly in up to 30% of patients, and new
intravenous drug use, immunodeficiency and such as Coxiella, Pneumococcus and Listeria onset clubbing may occur. Immune-mediated
previous bacterial endocarditis. are rare causes of endocarditis. glomerulonephritis may result in haematuria,
proteinuria and renal impairment.
Diagnosis is based on the modified Duke
Pathophysiology History Criteria for Diagnosis of Infective Endocardi-
tis (see below).
Turbulent cardiac blood flow through a struc- Patients with acute IE are often readily iden-
turally abnormal heart can damage endo- tified by systemic toxicity and high fever of
thelium and lead to overlying thrombus short duration. The presentation of sub-
Modified Duke criteria
formation. Transient bacteraemia may infect acute endocarditis is more non-specific and
the thrombus and a vegetation is formed. usually includes fever, anorexia, malaise, Major criteria
The local effects of vegetations include valvu- cough, headache and arthralgia. A high Typical microorganisms from at least two
lar insufficiency, valvular obstruction, and degree of suspicion is required to identify separate blood cultures or a single
perivalvular extension leading to intracardiac these patients. Any presentation of unex- positive blood culture for Coxiella
fistulae and conduction system abnormalities. plained prolonged fever, new neurological burnetii.

110
5.7 INFECTIVE ENDOCARDITIS
5

CARDIOVASCULAR
Mobile echodense intracardiac mass Imaging and consultation with microbiologists
depicted on echocardiogram. The investigation of possible endocarditis should be undertaken to optimise therapy.
Periannular abscess depicted on should include an electrocardiogram (ECG)
echocardiogram. and chest X-ray (CXR) but findings are Surgical
New partial dehiscence of prosthetic non-specific. A CXR may reveal evidence of The need for surgical management of IE
valve depicted on echocardiogram. cardiac or pulmonary complications but is depends on the severity of complications
New valvular regurgitation depicted on not diagnostic of IE. Transthoracic echocar- and response to medical management. Peri-
echocardiogram. diography is the most definitive investiga- valvular abscesses, obstructive vegetations,
tion and yields excellent images in most cardiac fistulae and prosthetic dehiscence
paediatric patients. often require surgery. Other indications
Minor criteria Accuracy can be increased using transoe- include persistent bacteraemia, recurrent
Predisposing factor history of sophageal echocardiography (TOE), which embolisation, and some cases of fungal
intravenous (IV) drug use or congenital should be considered in high-risk groups. endocarditis.
heart disease. Echocardiographic findings are also used
Fever >38 C. to guide management such as surgical
Vascular phenomena arterial emboli, intervention. Prognosis
pulmonary infarcts, mycotic aneurysm,
The overall mortality of IE remains approxi-
intracranial haemorrhage, conjunctival
mately 25% and morbidity is also consider-
haemorrhage, Janeway lesions.
Differential diagnosis able, with up to 60% of confirmed cases
Immunological phenomena
developing significant complications. Poor
glomerulonephritis, Osler nodes, Roth The presentation of IE is similar to many sys-
prognosis is associated with Staphylococcus
spots, positive rheumatoid factor. temic inflammatory conditions. Positive
aureus infection and fungal disease. Severe
Positive blood cultures or serological blood cultures often distinguish the diagno-
cardiac failure, prolonged clinical symptoms
evidence of infection not meeting above sis, but in suggestive presentations where
and poor response to antibiotics are also
criteria. blood cultures remain negative, other diag-
associated with worse outcomes.
noses must be considered. Rheumatic fever,
These criteria determine three diagnostic
Kawasaki disease, systemic lupus erythema-
categories: definite endocarditis, possible
tosus and leukaemia all have similar presen-
endocarditis and rejected cases. Definite Prevention
tations, hence the need for rigorous diagnostic
diagnosis requires the presence of two
criteria. Antibiotic prophylaxis for IE is recommended
major, one major with three minor, or five
internationally but guidelines are based on
minor criteria. Possible endocarditis is
expert consensus and vary across the world.
defined by one major with one minor, or
Australian guidelines adopt recommenda-
three minor criteria. Rejected cases are Treatment
tions from the American Heart Association
those where manifestations are explained
Medical and consider the underlying cardiac condition
by a clear alternate diagnosis or resolve
The medical management of IE includes and risk of the proposed procedure.
after antibiotic therapy for 4 days or less.
eliminating causative microorganisms and Recommended conditions requiring pro-
treating complications such as congestive phylaxis:
heart failure. The bacterial infection of vege-
tations is relatively protected and must be
prosthetic cardiac valves;
Investigations completely eradicated with prolonged par-
cardiac transplant with subsequent
valvulopathy;
Laboratory enteral therapy. As the aetiological agent
The most definitive test for IE is the identifi- is usually unknown at presentation, empiric
rheumatic heart disease with valvular
pathology;
cation of a known causative organism from antibiotic therapy should be commenced.
more than one blood culture. In all cases Australian antibiotic guidelines recommend
previous infective endocarditis;
of suspected endocarditis three sets of blood benzylpenicillin (45 mg kg1, max. 1.8 g)
congenital heart disease if:
 unrepaired cyanotic defect;
cultures should be collected from different and flucloxacillin (50 mg kg1, max. 2 g)
 completely repaired defect with
peripheral sites prior to antibiotic adminis- intravenously every 4 hours with daily IV
prosthetic material up to 6 months
tration. Up to 10% of all cases of IE remain gentamicin (46 mg kg1, max. 160 mg).
post procedure;
culture negative, usually where organisms Vancomycin should be added if there is a
 repaired defects with residual defect at
are highly fastidious, non-bacterial, or micro- prosthetic valve, penicillin allergy, nosoco-
prosthetic part.
biological techniques are insufficient. Non- mial infection or risk of methicillin-resistant
specific inflammatory markers such as white Staphylococcus aureus. The duration of Chemoprophylaxis depends on the proce-
cell count, C-reactive protein, erythrocyte parenteral therapy is usually 46 weeks, dure being performed. Australian guidelines
sedimentation rate and rheumatoid factor modified by the aetiological agent and ther- suggest dental and upper respiratory tract
may also be abnormal. apeutic response. Monitoring of drug levels procedures (e.g. reimplantation of avulsed

111
5.8 KAWASAKI DISEASE

tooth) receive amoxicillin (50 mg kg1

orally) as a single dose 1 hour before the Controversies Further reading
procedure. Cephalexin or clindamycin are Baddour LM, Wilson WR, Bayer AR, et al. Infective Endocarditis;
There is no published evidence to show that Diagnosis, Antimicrobial Therapy, and Management of
alternatives for penicillin sensitivity. Genito- antibiotic prophylaxis prevents IE. Complications: A Statement for Healthcare Professionals.
urinary and gastrointestinal procedures Endocarditis is thought more likely to result Circulation 2005;111:316784.
Fuster V. The AHA Guidelines and Scientific Statements
require ampicillin (50 mg kg1 IV) just from random bacteraemia than from that Handbook. Wiley-Blackwell; 2009.
before the procedure, or vancomycin associated with dental, GI or GU Milazzo AS, Li JS. Bacterial endocarditis in infants and
procedures. The American Heart children. Paediatr Infect Dis J 2001;20(8):799801.
(25 mg kg-1 IV) if pencillin allergic. Endo- Prendergast BD. The changing face of infective endocarditis.
Association has recently revised guidelines Heart 2006;92:87985.
tracheal intubation and urinary catheterisa-
to reduce the categories of patients for Therapeutic Guidelines: Antibiotics, Version 13. Therapeutic
tion do not require antibiotic prophylaxis. Guidelines Limited; 2006.
whom prophylaxis is recommended and Therapeutic Guidelines: Prevention of Endocarditis.
Current therapeutic guidelines should be Australian guidelines are in keeping with Therapeutic Guidelines Limited; 2008.
consulted to optimise management in these recommendations. Wilson W, Taubert KA, Gewitz M, et al. Prevention of Infective
each case. Endocarditis. Circulation 2007;116:173654.

5.8 Kawasaki disease

Daryl Efron

well as the resemblance of the clinical pre-

ESSENTIALS sentation to other self-limiting infectious dis-
eases, such as measles, adenovirus infection
1 Kawasaki disease is an uncommon ED presentation, but should be considered in and staphylococcal toxic shock syndrome.
any child with an unexplained prolonged fever, particularly in the presence of a rash
However, despite much investigation of a
and red eyes.
variety of viral and bacterial pathogens,
2 The diagnosis is clinical with presence of fever and four out of five other criteria. there is as yet no good evidence to implicate
any known organism in KD. The ethnic vari-
3 Diagnostic criteria can appear sequentially and may not all be present at the same ation suggests a genetic predisposition.
time, which can make early diagnosis difficult.
There is debate as to whether the inflam-
4 Patients with atypical Kawasaki disease are being increasingly recognised, in matory response in KD is initiated by a con-
whom full criteria are not met, but the patient has coronary artery abnormalities. ventional antigen or a superantigen, and
These are often infants. there are some immunological features to
support both hypotheses. Unreplicated
5 Any child with suspected Kawasaki disease should be treated immediately, reports of increased expression of specific
regardless of the duration of symptoms.
T-cell receptor V b-regions suggest toxin
6 Intravenous immunoglobulin (single dose of 2 g kg1) significantly reduces the risk activation, whereas infiltration of paratra-
of coronary artery abnormalities, especially if given in the first 10 days of illness. cheal and vascular tissue with reactive
clonal IgA plasma cells suggests entry of a
conventional antigen via the respiratory
than 5 years), it occurs in all ethnic groups, route.3
Introduction with an annual incidence in the United
Kawasaki disease (KD) is an acute, self-limiting States of approximately 10/100 000 chil-
vasculitic illness predominantly affecting dren younger than 5 years old. The majority
infants and young children. It is now a lead- of cases (85%) occur in children aged less
Pathophysiology
ing cause of acquired heart disease in chil- than 5 years of age. It is 1.5 times more com- The pathophysiology of KD involves vasculi-
dren in Western countries. The diagnosis is mon in boys than girls.2 tis of medium-sized vessels including coro-
made clinically, and effective treatment is Many features of KD suggest an infectious nary, renal, hepatic and splanchnic arteries,
available to reduce the likelihood of poten- aetiology. These include: seasonal variation beginning in both adventitial and intimal
tially fatal coronary vasculitis. KD was first (peak in winter/spring); occasional out- surfaces and proceeding toward the media.
described in 1967 as mucocutaneous lymph breaks; 10-fold higher risk in siblings than Coronary changes occur in approximately
node syndrome in a series of 50 Japanese in the general population; rarity in young 20% of untreated patients.4 Immune activa-
children.1 Although it is most common in infants (suggesting protection from mater- tion involving cytokines and growth factors
Japanese and Korean children (annual inci- nally acquired antibody); low recurrence rate leads to inflammation and aneurysm forma-
dence 90150/100 000 children younger (4%, which suggests acquired immunity); as tion, with the risk of thrombosis. The process

112
5.8 KAWASAKI DISEASE
5

CARDIOVASCULAR
evolves for a long period after the acute ill- halo around the cornea. There is not usually bowel disease, connective tissue disease,
ness. In the majority of patients with echo- an exudate. The changes may resolve rapidly, particularly Stills disease, and malignancy.
cardiographically demonstrable coronary such that this important feature may be pres-
artery lesions, the vessels remodel and have ent only on history rather than being present
a normal appearance within a year or so. when the child is examined. The cervical
However, there is evidence of subtle long- nodes are commonly unilateral and in the
Complications
term changes in coronary artery function, anterior chain. Generalised lymphadenopa- The major concern with KD is coronary
the clinical significance of which remain thy and splenomegaly are absent. The sub- artery disease. Younger children, especially
unclear.5 The risk of early adult coronary acute phase occurs from 24 weeks, with under 12 months of age, are at highest risk.
artery disease in these patients is unknown. resolution of fever. Desquamation (periun- Changes seen include dilatation (ectasia)
A diffuse inflammatory process of a vari- gual) of fingers and toes may occur during and discrete aneurysms. Aneurysms are clas-
ety of tissues has been found in autopsy spe- this time. sified according to the internal diameter into
cimens including lymph nodes, liver and gall- KD is a multisystem disease with many small (<3 mm), medium (36 mm), large
bladder.2 Endothelial changes are promi- and varied clinical manifestations. In addi- (68 mm) and giant (>8 mm). Although
nent, with hyperplasia, necrosis and throm- tion to those in the diagnostic criteria, com- aneurysms rarely form in the first 10 days
bosis. Myocardial abnormalities include mon features include marked irritability, of KD, echocardiographic signs of coronary
hypertrophy of myocytes and fibrosis. diarrhoea, cough, arthralgia/arthritis, ure- arteritis may be seen, including perivascular
thritis with sterile pyuria, otitis media, mild brightness, ectasia and lack of tapering.4
hepatic dysfunction, hydrops of the gallblad- Other findings may include decreased left
der and aseptic meningitis. Diagnosis is ventricular contractility, mitral regurgita-
Clinical features more likely to be delayed in older children, tion, and pericardial effusion.
KD should be considered in the differential in whom less classical manifestations such Coronary artery occlusion is most likely to
diagnosis of all infants and young children as gastrointestinal and joint symptoms often occur in giant aneurysms, through a combi-
with a fever, rash and red eyes, as well as predominate.7 nation of sluggish blood flow and fibrotic ste-
those with a prolonged fever without an nosis at proximal and/or distal ends of the
alternative explanation. The diagnostic cri- aneurysm. Thrombosis of coronary aneur-
teria are outlined in Table 5.8.1. ysms can cause myocardial infarction and
Incomplete KD
The diagnosis can be made earlier than sudden death. Mortality is increased over
day 5 if other features are present. This is Cases of incomplete or atypical KD are the background rate within the first two
important, as there is evidence that earlier being increasingly recognised, in which full months. Rarely, myocarditis can occur, caus-
administration of intravenous immunoglob- criteria are not met, but the patient has cor- ing congestive cardiac failure or arrhythmias.
ulin (IVIG) is associated with a shorter illness onary artery abnormalities. This is more Thrombosis of peripheral arteries can lead to
and reduced risk of coronary disease.6 common in young infants.8 The incidence ischaemia and gangrene.
The fever is often associated with irritabil- of coronary artery aneurysms is at least as
ity, which responds poorly to paracetamol. high in incomplete KD as in classical cases.
Over 90% of patients develop a rash, Given that infants under 6 months appear
to be at increased risk of developing coro-
Investigations
which can take any of a number of forms
scarlatiniform, morbilliform or maculopapular. nary abnormalities;8 a lower threshold for Any child who presents with possible fea-
It often has the appearance of a viral erup- treatment is probably indicated in this tures of KD should be discussed with a
tion or allergic reaction, but can appear urti- group. KD should be considered in all chil- paediatrician.
carial. Petechiae are sometimes seen, as are dren with unexplained fever for 5 days and There is no definitive diagnostic test for
micropustules. The red eyes demonstrate at least two major features of KD, and any KD. Investigation is directed toward exclud-
characteristic sparing of the avascular peri- infant with unexplained fever for over a ing alternative diagnoses, as well as
limbic region, giving the appearance of a week.4 gathering supporting evidence for the diag-
nosis of KD. At least one set of blood cultures
should be taken, and cultures of urine, cere-
brospinal fluid and other sites may be indi-
Table 5.8.1 Diagnostic criteria Differential diagnosis cated. Serological testing for Group A
for Kawasaki disease
Fever (at least 5 days) Depending on which features are present, a Streptococcus, and for specific viruses such
Plus four of the following five features: broad differential diagnosis needs to be con- as measles may be helpful. In KD, the periph-
bilateral conjunctival injection
enanthem dry, cracked lips; oropharyngeal sidered in cases of possible KD. These include eral white cell count and inflammatory mar-
erythema; strawberry tongue viral infections (e.g. measles, adenovirus, kers are generally significantly raised. The
exanthem polymorphous
peripheral changes erythema (palms and enterovirus), streptococcal and staphylococ- erythrocyte sedimentation rate may con-
soles), oedema (hands and feet), cal toxic-mediated illness (scarlet fever, toxic tinue to rise as the child improves clinically.
desquamation (2nd to 3rd week)
cervical adenopathy
shock syndrome), drug allergy (including A marked thrombocytosis is commonly seen
StevensJohnson syndrome), inflammatory in the second to third week of the illness.

113
5.8 KAWASAKI DISEASE

Pyuria, due to sterile urethritis, is often may interfere with immunogenicity, live vac- and tumour necrosis factor-a antagonists
found on a voided sample (not on suprapu- cine administration (e.g. measles, varicella) have been reported in children with refractory
bic aspirate (SPA) or catheter). Serum should be postponed by 3 months in chil- KD. The role of these therapies remains
gamma glutamyl transferase is commonly dren who have been given IVIG. unclear.4
elevated. Exclusion of markers for connec- In the 1980s, high-dose aspirin was
tive tissue disorders may be useful. shown to decrease the incidence of coronary
Echocardiography should be undertaken artery involvement in KD.11 The current role
as soon as the diagnosis of KD is suspected. of aspirin in KD is difficult to determine as it Prognosis
The American Heart Association guidelines has been used in combination with IVIG in
suggest repeat echocardiograms at 2 weeks the major trials. Many centres have used Mortality is less than 1%, being highest in
(time of maximal aneurysm formation), 6 high-dose aspirin (80100 mg kg1 day1 those less than 12 months old. Recurrence
weeks and 12 months (looking for late in 34 divided doses, for anti-inflammatory is most likely to occur in children aged less
sequelae). If abnormalities are demonstrated effect) initially, and then switched to than 3 years who had cardiac involvement
on echocardiography, closer cardiological low-dose (35 mg kg1 day1, antiplatelet initially, and usually within 12 months of
follow up may be required, depending on effect) after the patients fever resolves. the initial episode.19 Patients with recurrent
the lesion. A 12-lead ECG is most often However, in patients treated with IVIG, con- KD appear to be at increased risk for cardiac
normal but may show dysrhythmias, change comitant use of high-dose aspirin initially sequelae.
in PR or QT intervals or non-specific ST does not appear to result in shorter duration Children without demonstrable cardiac
changes. of fever or hospitalisation than low-dose.12 disease appear to have an excellent progno-
Furthermore, the incidence of coronary sis, with long-term follow-up studies demon-
artery aneurysm appears to be independent strating absence of clinical sequelae for up
of aspirin dose.13 Therefore low-dose aspirin to 21 years.20 It is possible, however, that
Treatment seems to be sufficient for initial treatment. individuals who have had KD are at risk of
Treatment of KD is directed towards reduc- Low-dose aspirin is continued for 68 weeks, early atherosclerotic heart disease.
ing the inflammation as rapidly as possible. and then stopped if there is no coronary The prognosis for children who have had
The clinical markers of response to therapy involvement. coronary artery aneurysms is less clear. Most
are the temperature and patients general The role of corticosteroids in KD is a sub- small to medium-sized aneurysms resolve
well-being, supported by the white cell ject of continuing research. Studies of the echocardiographically;21 but healing involves
count and CRP. addition of a single pulsed dose of steroids fibrosis and calcification, with associated loss
IVIG therapy has been demonstrated to to IVIG as initial therapy have yielded of vascular distensibility and reactivity. A
induce resolution of fever as well as signifi- conflicting results.14,15 It is possible that a proportion of coronary artery aneurysms
cantly reduce the risk of coronary artery subset of patients at highest risk of develop- progress to stenosis over time. Therefore chil-
abnormalities (from around 20% to around ing coronary artery aneurysms may benefit; dren with KD who have had coronary artery
35%) if given within the first 10 days of however, risk scores for stratification of aneurysms should have indefinite cardiology
the illness.9 The precise mechanism of patients have not been validated. follow up. These patients will generally
action of IVIG is unknown. Theories include be treated with long-term antithrombotic
saturation blockade of Fc receptors, direct therapy to prevent myocardial ischaemia.
antibody activity against bacterial superan- Children receiving long-term aspirin ther-
tigen, an unidentified causative pathogen or
Refractory KD apy should receive the influenza vaccine
toxin, modulation of cytokine production or Up to 15% of patients with KD treated with annually, to prevent Reye syndrome. New
down-regulation of antibody synthesis. The IVIG and aspirin have persistence or early antiplatelet agents are under investiga-
optimal dose is 2 g kg1 day1 as a single recrudescence of fever, indicative of an tion.22 There have been reports of the use
infusion over 1012 hours. There is some ongoing vasculitic process.16 This is a strong of thrombolytic agents in patients with KD,
evidence that IVIG is also effective if given risk factor for the development of coronary with thrombus seen within coronary aneur-
beyond 10 days;10 however, treatment as artery aneurysms.17 Children with ongoing ysms.23 Anticoagulation with warfarin is
early as possible is optimal.6 IVIG should or recurring fever beyond 36 hours after required for patients with giant (8 mm)
be given to patients with KD after the treatment with IVIG should be given a sec- or multiple aneurysms, and surgical interven-
10th day if they have persistent fever or ond dose of IVIG.4 tion (e.g. angioplasty, bypass grafting) is
other evidence of ongoing systemic inflam- Pulses of methylprednisolone (30 mg kg1 occasionally necessary.
mation.4 The effect of IVIG in patients daily for 1-3 days) have been used in cases
who have already developed coronary artery unresponsive to two doses of IVIG.18 Cortico-
Controversies
aneurysms is unknown, though there may be steroids have been shown to reduce fever in
some benefit.9 Different brands of IVIG may patients with resistant KD; however, their The immunological mechanism of
vary in their clinical effects, due to variation effect on coronary artery abnormalities is the inflammatory response in KD is the
in sterilisation and other manufacturing pro- uncertain.4 A number of other treatments in- subject of continuing research there is
cedures. As passive antibody acquisition cluding plasma exchange, cyclophosphamide, some evidence to support a conventional

114
5.9 CARDIAC ARRHYTHMIAS
5

CARDIOVASCULAR
the Young, American Heart Association. Pediatrics Kawasaki disease: a meta-analysis of 862 children.
antigen, but other evidence suggests a 2004;114:170833. Pediatrics 2005;116:989.
5. Freeman AF, Shulman ST. Recent developments in 15. Newburger J, Sleeper L, McCrindle B, et al. Randomized
superantigen. Kawasaki disease. Curr Opin Infect Dis 2001;14:35761. trial of pulsed corticosteroid therapy for primary

The optimal dose of aspirin in the

6. Tse S, Silverman E, McCrindle B, Yeung R. Early treatment
with intravenous immunoglobulin in patients with
treatment of Kawasaki disease. N Engl J Med
2007;356:663.
acute febrile phase is uncertain. Kawasaki disease. J Pediatr 2002;140:4505. 16. Burns J, Glode M. Kawasaki syndrome. Lancet
7. Stockneim JA, Innocentini N, Shulman ST. Kawasaki 2004;364:533.
 The role of steroids in KD is debated. disease in older children and adolescents. J Pediatr
2000;137:2502.
17. Kim T, Choi W, Woo C, et al. Predictive risk factors for
coronary artery abnormalities in Kawasaki disease.

The clinical significance of subtle

8. Rosenfeld EA, Corydon KE, Shulman ST. Kawasaki disease
in infants less than one year of age. J Pediatr 18.
Eur J Pediatr 2007;166:421.
Wright DA, Newburger JW, Baker A, et al. Treatment
long-term changes in coronary artery 1995;126:5249. of immune globulin-resistant Kawasaki disease with
9. Newburger JW, Takahashi M, Beiser AS, et al. A single pulsed doses of corticosteroids. J Pediatr 1996;128:
function in some patients who have had intravenous infusion of gamma globulin as compared 1469.
KD remains unknown. with four infusions in the treatment of acute Kawasaki 19. Hirata S, Nakamura Y, Yanagawa H. Incident rate of
syndrome. N Engl J Med 1991;324:16339. recurrent Kawasaki disease and related risk factors:
10. Marasini M, Pongiglione G, Gazzolo D, et al. Late nationwide surveys of Kawasaki disease in Japan. Acta
intravenous gamma globulin treatment in infants and Pediatr 2001;90:40.
children with Kawasaki disease and coronary artery 20. Kato H, Sugimura T, Akagi T, et al. Long-term
References abnormalities. Am J Cardiol 1991;68:7967. consequences of Kawasaki disease: A 10- to 21-year
11. Koren G, Rose V, Lavi S, et al. Probable efficacy of follow-up study of 594 patients. Circulation
1. Kawasaki T. Paediatric acute mucocutaneous lymph node high-dose salicylates in reducing coronary artery 1996;94:1379.
syndrome: Clinical observation of 50 cases. Arerugi involvement in Kawasaki disease. JAMA 21. Fukushige J, Takahashi N, Ueda K, et al. Long-term
1967;16:178222 (in Japanese). 1985;254:7679. outcome of coronary abnormalities in patients after
2. Burns JC. Kawasaki disease. Adv Pediatr 12. Hsieh K, Weng K, Lin C. Treatment of acute Kawasaki Kawasaki disease. Pediatr Cardiol 1996;17(2):716.
2001;48:15777. disease: Aspirins role in the febrile stage revisited. MarApr.
3. Meissner HC, Leung DYM. Superantigens, conventional Pediatrics 2004;114(6):e689e693. 22. Williams RV, Minich LL, Tani LY. Pharmacological therapy
antigens and the etiology of Kawasaki syndrome. Pediatr 13. Terai M, Shulman ST. Prevalence of coronary artery for patients with Kawasaki disease. Paediatr Drugs
Infect Dis J 2000;19:914. abnormalities in Kawasaki disease is highly dependent 2001;3:64960.
4. Newburger J, Takahashi M, Gerber M, et al. Diagnosis, on gamma globulin dose but independent of salicylate 23. Tsubata S, Ichida F, Haramichi Y, et al. Successful
Treatment, and Long-Term Management of Kawasaki dose. J Pediatr 1997;131:88893. thrombolytic therapy using tissue-type plasminogen
Disease: A Statement for Health Professionals From 14. Wooditch A, Aronoff S. Effect of initial corticosteroid activator in Kawasaki disease. Pediatr Cardiol
the Committee on Rheumatic Fever, Endocarditis, and therapy on coronary artery aneurysm formation in 1995;16:1869.
Kawasaki Disease, Council on Cardiovascular Disease in

5.9 Cardiac arrhythmias

Gary Williams

ESSENTIALS Introduction
1 Focus initially on airway, breathing and circulation and then recognise and treat Identification and management of the child
arrhythmias compromising cardiac output using the safest possible means. with a cardiac arrhythmia in the emergency
department (ED) requires an initial focus on
2 If an arrhythmia is fast and regular try to assess the width of the QRS complex and, if required, attention to the patients
against age-based normal values. haemodynamic stability, followed by a team
3 Supraventricular tachycardia (SVT) accounts for 90% of all significant approach to diagnose and treat the arrhyth-
arrhythmias in children. mia if necessary. Although arrhythmias
occur less frequently in acutely ill infants
4 Any previous 12-lead electrocardiograph (ECG) may be of enormous value in and children compared to adults, vigilance
diagnosing an arrhythmia. is required, as a correct acute assessment
5 Ventricular tachycardia (VT) has a wide range of potential aetiologies and and management of the arrhythmia will
treatment is based on an assessment of degree of compromise and morphology have significant long-term consequences.
of the tachycardia. The primary aim of this initial assessment
and resuscitation phase in an unstable
6 Continuous recording of a single-lead ECG during acute resuscitation manoeuvres patient is to recognise and treat arrhythmias
(adenosine administration, vagal manoeuvres, cardioversion) may yield very valuable that are compromising cardiac output
information. using the safest means possible so that
7 Any child with a significant arrhythmia should be discussed with a paediatric longer-term management decisions can be
cardiologist at the earliest possible time. made in consultation with a paediatric
cardiologist.

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5.9 CARDIAC ARRHYTHMIAS

tone than adults and are also known to fascicles (anterior and posterior). From these
Normal conduction system have developmental and age-dependent specialised fibres the impulse spreads from
The normal heartbeat is initiated by an differences in action potential amplitude endocardium to epicardium throughout the
impulse which originates from the sinoatrial and conduction speed. Accordingly, there right and left ventricles.
(SA) node located in the wall of the right are age-dependent normal values for resting Abnormally situated embryonic remnants
atrium near the superior vena cava junction. heart rate as well as PR interval and QRS of conducting tissue can persist as accessory
The impulse is then propagated via conduct- duration, in addition to many other electro- tracts. These are most commonly found
ing cells that form a specialised system physiology parameters (Table 5.9.1). around the AV node and are capable of con-
throughout the heart. Initially the impulse Depolarisation initiated in the SA node ducting electrical activity between the atria
travels across the atria and via transatrial spreads rapidly through the internodal path- and ventricles thus bypassing the AV node.
internodal pathways that converge on the ways to converge on the AV node. Usually, These remnants are the anatomical sub-
atrioventricular (AV) node. The impulse pro- the atria and ventricles are electrically sepa- strate for re-entrant tachyarrhythmias.
ceeds down the bundle of His to the right rated from one another by a ring of fibrous
and left bundle branches; the impulse then tissue at the atrioventricular junction (the
finally spreads throughout the Purkinje annulus fibrosis). Accordingly, in usual cir-
fibres to depolarise the ventricular muscle. cumstances, the impulse must pass to the The cardiac action potential
The conducting cells of this specialised His bundle via the AV node. The AV node
The cardiac action potential is divided into
system have a rapid conduction velocity to is located in the inferomedial wall of the
five phases: 0, 1, 2, 3, 4.
rapidly propagate the electrical impulse right atrium near the insertion of the septal
throughout the heart. The various parts of leaflet of the tricuspid valve. Conduction Phase 0 is depolarisation due to voltage
this conduction system are also capable of through the AV node is slow to allow com- gated opening of sodium channels with
spontaneous depolarisation and impulse pletion of atrial systole (and ventricular dias- sodium rushing into the cell. There is an
generation under abnormal conditions. tole). This delay (and therefore potentially intense entry of sodium for a brief period
The SA node is normally the dominant the completeness of ventricular filling) can resulting in depolarisation of the entire
(fastest) cardiac pacemaker but this can be reduced during sympathetic stimulation. cell as well as cell-to-cell propagation.
change if sinus node dysfunction occurs or After leaving the AV node the impulse Phase 1 is a phase of partial repolarisation
if other parts of the conduction system travels along the bundle of His for 12 cm due to several factors but including
develop increased automaticity. As stated along the posteroinferior margin of the chloride entry into and possibly potassium
above, the SA node is located near the junc- membranous portion of the interventricular egress from the cell.
tion of the superior vena cava (SVC) and the septum, before dividing into the right and Phase 2 is the plateau phase thought to be
right atrium. The SA node is innervated by left bundle branches. These extensions of important in coordinated and sustained
both sympathetic and parasympathetic the conducting system spread subendo- ventricular contraction. During this phase
nerve endings. Parasympathetic tone predo- cardially across the ventricular chambers there is slow inward calcium current and
minates during rest. Children are known to to the base of the papillary muscles. slow inward sodium current balanced by a
have relatively greater parasympathetic The left bundle separates into two distinct gradually increasing outward potassium
movement.
Phase 3 is repolarisation and it occurs by
Table 5.9.1 Normal resting heart rates, PR intervals and QRS durations in children inactivation or closure of the slow calcium
Age Heart rate mean Range PR interval (ms) QRS duration
and sodium channels and then a voltage
(beats min1) (ms) gated progressive opening of potassium
<1 day 119 94145 70120 5084 channels leading to outward potassium
ion movement and a progressively more
17 days 133 100175 70120 4079
negative membrane potential. Also
730 days 163 115190 70120 4073 during this phase, the fast sodium
13 months 154 124190 70130 5080 channels are reactivated and the cell is
primed for further depolarisation.
36 months 140 111179 70130 6080
During phase 4, an energy-dependent
612 months 140 112177 80130 5080 membrane NaK ATPase removes the
13 years 126 98163 80150 5080 sodium and restores the potassium to the
cell. Most myocardial cells maintain a
35 years 98 65132 90150 6084
constant level of depolarisation during
58 years 96 70115 100160 5080 this phase but Purkinje or conducting
812 years 79 55107 100170 5084 cells, by way of reduced outward
potassium flow and some inward sodium
1216 years 75 55102 110160 4080
flow, achieve slow depolarisation until the
Source: Adapted from Liebman 1982. AP threshold is reached. These

116
5.9 CARDIAC ARRHYTHMIAS
5
blocking, b-antagonism and calcium chan-

CARDIOVASCULAR
conducting cells have a shorter duration contractility. Verapamil is primarily used in
action potential of lower amplitude, nel blocking effects. Oral amiodarone has the adult setting because of its reduction
thought to be mediated more unusual pharmacokinetics, with clinical of conduction velocity in the AV node,
predominantly by slow calcium flux. effect only apparent after several days of thereby controlling the ventricular response
treatment and a half life of 315 weeks. Side rate to SVT, atrial flutter or atrial fibrillation.
Vaughan Williams antiarrhythmia effects are frequent and serious (corneal Even in that situation it is still a second-line
drug classification photosensitivity, hyper- or hypothyroidism, agent to adenosine for SVT termination in
Antiarrhythmic drugs are classified into five pulmonary fibrosis and pro-arrhythmia). adults, provided there is good left ventricular
classes in the Vaughan Williams classifica- Intravenous amiodarone is used in the acute (LV) function. However, because of serious
tion by the mechanism or channel that they management of post-operative tachyarrhyth- bradycardia, hypotension and cardiac arrest
most reliably affect during in vitro studies. mias (usually junctional ectopic tachycardia, in infants, it is not used in children. Instead,
There is, however, considerable overlap. JET). In the adult ventricular fibrillation pro- b-blockers are used in WolffParkinson
Class 1 drugs block the fast inward tocol, a randomised comparison between White (WPW) syndrome and digoxin in
sodium channels and thereby increase the amiodarone and lignocaine found a greater non-WPW patients.
refractory period. This class is further subdi- chance of successful resuscitation (but not Finally, Class 5 agents are a miscella-
vided into: survival) with amiodarone. Therefore some neous group and include adenosine and
authorities have recommended amiodarone digoxin. Adenosine is an endogenous purine
1a. Those that prolong action potential
as the agent of choice to help effect defibril- nucleoside with a very rapid half life (less
duration (e.g. quinidine);
lation after adrenaline (epinephrine). In hae- than 5 seconds). It acts by depressing slow
1b. Do not change or shorten action
modynamically stable VT the agent of calcium channels and enhancing potassium
potential duration (e.g. lignocaine); or
choice is probably sotalol unless there is conduction. Its main effects are to depress
1c. Produce some mild action potential
impaired ventricular function (EF <40%, sinus node and AV node activity with a
prolongation (e.g. flecainide).
signs of congestive heart failure) when shortening of atrial refractoriness. Because
It is fair to say that these are infrequently amiodarone is recommended. In unstable of its effect in blocking AV nodal activity,
used in paediatrics, though lignocaine is still or polymorphic VT the recommended agent adenosine is superbly suited to block re-
an option in the ventricular fibrillation pro- depends a little on the initial trace. If it looks entry phenomena, the most frequent cause
tocol and flecainide may sometimes be used like torsade de pointes with the trace oscil- of SVT in children. Series show that adeno-
in supraventricular tachyarrhythmias. lating around the baseline (or in polymor- sine terminates 90100% of SVTs but the
Class 2 agents are b-blockers, which act phic VT with a normal QT interval), the arrhythmia re-initiates in approximately
by a combination of b-antagonism and a clear recommendation is intravenous (IV) 25%. Other atrial tachycardias like multi-
quinidine-like membrane stabilising effect. magnesium. If it is not torsade-like and the focal atrial tachycardia, atrial flutter or atrial
There is a slowing of conduction velocity, QT interval can be determined and is not fibrillation are almost always resistant to
prolonging of action potential duration prolonged, amiodarone is a second-line adenosine. Adenosine is given in increasing
and a reduction of automaticity Their main agent to b-blockers. If the QT interval is doses from 50200 mcg kg1 every 2 min-
clinical use is in treating supraventricular prolonged, then IV magnesium, followed if utes, using a two-syringe technique so that
tachyarrhythmias (SVT, atrial fibrillation, necessary by lignocaine, would be the it can be flushed rapidly into the circulation.
atrial flutter) by increasing the refractory recommended approach. Side effects can be facial flushing, broncho-
period of the AV node. Propranolol or aten- Sotalol is classified as a class 3 drug, spasm or sinus arrest. Adenosine is essen-
olol may be used but in acute situations although it is a non-selective b-blocker with tially very safe, although there is a single
many clinicians favour esmolol, a very additional class 3 properties. It therefore case report of VF occurring after a dose of
short-acting parenteral b-1-antagonist (half combines the b-blocking effect on the SA adenosine in a neonate with a concealed
life 9 minutes), which can be useful in supra- and AV nodes with prolongation of AP dura- WPW syndrome previously treated with
ventricular tachycardia (SVT) and possibly tion and lengthening of refractory period digoxin.
ventricular tachycardia (VT). elsewhere in the heart. It is valuable acutely Although adenosine is unlikely to be
Class 3 drugs act primarily as potassium and long term in preventing SVT of all sorts controlling in supraventricular tachycardias
channel blockers and basically prolong the and is the agent of choice in monomorphic other than re-entrant SVT, the acute injec-
phase 3 repolarisation phase, thereby caus- stable VT with apparent normal ventricular tion of adenosine may transiently slow AV
ing a prolongation of the action potential function. Like amiodarone, it does increase nodal activity and allow the flutter wave
and increase in the effective refractory the QT interval and is therefore pro- or P wave morphology to be assessed. This
period. This class of drug is particularly use- arrhythmic as well as having the other risks may be very valuable in diagnosing the spe-
ful in ventricular tachyarrhythmias and of hypotension and bradycardia, presumably cific rhythm. In a haemodynamically stable
includes amiodarone and sotalol. resulting from its b-blocking effects. wide complex tachycardia of uncertain ori-
Amiodarone is one of these antiarrhyth- Class 4 agents are calcium channel block- gin, the differential diagnosis is primarily
mics with a plethora of effects, in that it is ers and the prototype remains verapamil, between VT and SVT with aberrant conduc-
primarily class 3 (i.e. potassium channel which has wide-ranging effects on shorten- tion. Adenosine administration might be dis-
blocker) but also has sodium channel ing the plateau phase and reducing cussed as a way of making this distinction

117
5.9 CARDIAC ARRHYTHMIAS

(i.e. effective in the latter but not in the for- Macro re-entry usually involves the partic- generation if the opportunity arises. These
mer). In the adult literature, unless SVT ori- ipation of an accessory conduction pathway. cells are, however, normally overridden and
gin is strongly suspected, this practice is Accessory pathway conduction characteris- kept refractory by the dominant (faster)
discouraged because of the potential for tics vary widely among patients. The acces- pacemaker of the SA node. Under certain
brief hypotension, and accelerated acces- sory pathway in some patients conducts adverse physiological conditions (e.g. hypo-
sory pathway conduction following adeno- antegrade during sinus rhythm, whereas in kalaemia, hypoxia) the threshold for sponta-
sine injection. others it conducts only retrograde during neous automaticity for these conducting
Digoxin is still very commonly used paren- tachycardia. When anterograde conduction cells may be altered. This potentially creates
terally, acutely, and as a maintenance medi- is possible down the accessory pathway a situation of enhanced automaticity and
cation, primarily to slow AV conduction and the standard ECG will show pre-excitation secondary enhanced pacemakers. One exam-
decrease the ventricular response to atrial with a short PR interval and wide QRS, ple of a tachycardia due to enhanced auto-
dysrhythmia like flutter or fibrillation. It pro- including an initial delta wave resulting maticity is multifocal atrial tachycardia.
vides rate control and sometimes converts from the pre-excitation of the ventricle from
supraventricular tachyarrhythmias. There is the sinus impulse conducting through the After depolarisations
an important caveat with digoxin, that it accessory pathway before the impulse has After depolarisations are due to oscillations
may shorten accessory pathway refracto- passed through the normal conducting sys- of the membrane potential during re-
riness and increase the resulting ventricular tem. Patients with WPW usually manifest polarisation that has reached the thres-
response. Therefore, it should not be used in orthodromic tachycardia (forward excitation hold membrane potential and triggered
accessory pathway dysrhythmias like WPW through AV node and rapid retrograde con- a second complete depolarisation. This
syndrome. Virtually any arrhythmia can arise duction via accessory pathway to create a process may become self-perpetuating.
from intoxication with digoxin. This risk is circuit) but also (rarely) display antidromic Torsade-de-pointes is the classic example
greater with hypokalaemia and the acute tachycardia involving retrograde conduction of such a triggered arrhythmia.
treatment is intravenous Digitalis antibody. up the usual atrioventricular route or via an
alternative accessory connection.
Supraventricular tachycardia is the most General principles for
common sustained tachyarrhythmia in chil- arrhythmia management
Pathogenesis of dren and almost always has a re-entry mech-
arrhythmias anism. Most commonly, particularly in
Direct primary attention to assessment
and correction of ABCs.
Bradyarrhythmias children younger than 12 years of age, this
re-entry is caused by an accessory atrioven-
If patient has an acute arrhythmia with
Two mechanisms are responsible for brady-
haemodynamic compromise (e.g. shock
arrhythmias: tricular connection (resulting in atrioventric-
or loss of consciousness), assess whether
ular (or AV) re-entrant tachycardia). In
Some form of sinus node dysfunction.
adolescents, AV node re-entry is the mecha-
the rhythm is fast and/or disorganised
Conduction system block.
nism in up to one third of patients. Previ-
or slow and/or irregular. If the rhythm is
fast or disorganised, cardioversion or
ously we believed that this was due to
defibrillation should take priority. If slow
Tachyarrhythmias a re-entry circuit within the compact AV
and/or irregular, cardiopulmonary
There are three fundamental mechanisms node but a newer concept involves tissue,
resuscitation should commence.
proposed for the generation of tachyarrhy- most likely atrial muscle, more than several
millimetres outside the compact atrioven-
 Obtain venous access and draw blood for
thmias:
biochemistry, specifically Na, K, Ca, Mg
tricular node.
Re-entry.
The commonest mode of accessory
and blood sugar level.
Enhanced automaticity.
connection-mediated re-entry tachycardia
Record a 12-lead ECG as soon as possible.
 After depolarisations (triggered
is orthodromic tachycardia, with the circular
arrhythmias). Bradyarrhythmias
movement of the electrical impulse going
Normal heart rate varies as a function of
antegrade through the atrioventricular
age. Pathological bradyarrhythmia may
Re-entry node and then retrograde up the accessory
present as fatigue, light-headedness or syn-
Re-entry exists when a closed loop of connection.
cope in an otherwise well child or inappropri-
specialised conducting tissue allows an elec-
ate absence of tachycardia in a critically
trical impulse to travel in a circular fashion Enhanced automaticity
unwell child under stress.
and permits atrial or ventricular electrical The primary pacemaker cells of the SA and
activation with each pass around the circuit. AV nodes usually demonstrate spontaneous
Sinus bradycardia
Re-entry may occur on a large (macro) or depolarisation as well as having somewhat
Sinus bradycardias can be caused in children
small (micro) scale. Atrial flutter and ven- slower action potential propagation. The
by:
tricular fibrillation are examples of micro cells of the conduction system have more
re-entry; paroxysmal supraventricular tachy- rapid action potential propagation but are parasympathetic stimulation (e.g.
cardia is an example of macro re-entry. also capable of spontaneous action potential suctioning);

118
5.9 CARDIAC ARRHYTHMIAS
5
First degree AV block Is present when the

CARDIOVASCULAR
metabolic disturbances (e.g. hypoxia, Bundle branch block
asphyxia, hypothermia); PR interval is longer than usual for age Bundle branch block patterns are unusual in
poisoning (e.g. b-blocker, calcium channel (see Table 5.9.1) but normal sinus rhythm paediatrics but occur when impaired con-
blocker); and 1:1 AV conduction is maintained. This duction is present in the specialised intra-
raised intracranial pressure; is a normal feature of the ECG of ventricular conduction tissue, resulting in
surgical injury to the sinoatrial node (e.g. endocardial cushion defects. It may occur delayed right or left ventricular depolarisa-
following Mustard or Senning procedures in a normal heart during parasympathetic tion and a resulting broad aberrant QRS
for transposition, Fontan, closure of atrial stimulation or digoxin treatment and complex. Right bundle branch block (wide
septal defect or correction of total usually does not require treatment. QRS with rSR pattern in right ventricular
anomalous pulmonary venous drainage); Second degree Mobitz type I AV block leads) may be a normal variant but also
cardiomyopathy. (Wenckebach) Is characterised by occurs in congenital heart disease (espe-
progressive prolongation of the PR interval, cially involving RVH), cor pulmonale and
culminating in a single non-conducted acute pulmonary embolism. Left bundle
Sinus node dysfunction
beat. This usually is a benign normal branch block (wide QRS with RR pattern in
Sinus node dysfunction may manifest as
variant but may represent a temporary left chest leads) is associated with LV strain
sinus pauses (a transient interruption of nor-
pathological prolongation of the or hypertrophy or operated congenital heart
mal sinus mechanism), or sinus exit block
atrioventricular node refractory period. disease.
(abnormal propagation), either of which
Second degree Mobitz type II AV block
may occur with or without an associated
Manifests as a regular intermittent failure
escape rhythm and may evolve to sinus Tachyarrhythmias
of P wave conduction while the PR
arrest. There is a wide range of tachyarrhythmias.
interval remains constant. This is more
Immediate diagnosis and management is
likely attributable to a block within the
best based on the width of the QRS complex
Management His conduction system and as such has
when compared to age-based normal values.
Treatment is required if an adequate greater potential to progress to complete
cardiac output is not being maintained. (3rd degree) atrioventricular block.
If possible, remove the cause (i.e. suction Third degree atrioventricular block (or Wide complex tachyarrhythmia
catheter). complete heart block) Represents The differential diagnosis of a wide or broad
 Ensure adequate ventilation. complete failure of atrial depolarisation complex tachycardia (i.e. QRS duration
If life threatening, commence to propagate to the ventricle. greater than normal for age, usually
compressions and administer atropine Anatomically the block may occur at >0.08 seconds) is between:
20 mcg kg1 IV or EndoTracheal (ET) atrioventricular node or at infranodal
level. ECG will show complete dissociation
Ventricular tachycardia.
(minimum dose 100 mcg).
SVT with intraventricular aberrant
If this is ineffective, or bradycardia of P waves and QRS complex. A narrow
conduction (e.g. pre-existing bundle
recurs, use b-adrenergic agonist or QRS junctional escape rhythm implies a
branch block).
adrenaline (epinephrine) bolus. nodal block whereas a widened slower
QRS escape rhythm suggests an
 Atypical or antidromic SVT (where
Commence adrenaline (epinephrine) or
during the tachycardia antegrade
isoproterenol by infusion while urgent infranodal site for the block.
conduction occurs down the fast
preparations are made for temporary Clinical features Patient may be
accessory pathway and retrograde
external or transvenous cardiac pacing. asymptomatic but will usually
component goes via the atrioventricular
demonstrate an inadequate cardiac
node).
output, particularly in episodes of
Atrially originated arrhythmias
Conduction disturbances: atrioventricular block associated with
conducted via an accessory connection
atrioventricular block slower heart rate. In third degree AV
to the ventricular muscle.
This involves delayed or incomplete conduc- block, cannon A waves are visible in the
tion through the AV node. Causes include a neck and a slow cardiac rhythm with VT in children usually presents as a wide
congenital form (maternal systemic lupus variable first heart sound is present on complex rhythm between 120 and 220 per
erythematosus) and an acquired form. auscultation. minute. There is usually AV dissociation
Acquired AV block occurs in poisoning, met- Management Emergency treatment is only but retrograde VA conduction may occur. It
abolic disturbances, myocarditis, rheumatic required if cardiac output is inadequate. may be mono- or polymorphic, sustained
fever, Lyme disease, fibrosis in the area of Optimise ventilation and initiate b- or non-sustained. Causes include metabolic
the conduction system associated with pre- agonist by infusion while organising abnormalities, poisoning, myocarditis, car-
vious cardiac surgery (particularly ventricu- temporary external/transvenous cardiac diomyopathy, ventriculotomy, ventricular
lar septal defect or atrioventricular septal pacing. This is definitely indicated in tumours and congenital or acquired Long
defect closure, tetralogy repair or aortic symptomatic children with Mobitz type II QT Syndrome.
valve replacement) and inferior myocardial second degree AV block and 3rd degree Most children with VT are symptomatic
infarction. AV block. with lethargy, symptoms of pulmonary

119
5.9 CARDIAC ARRHYTHMIAS

congestion, poor circulation and possibly further assessment of the trace. If the trace is cases, either alone or in combination with
palpitations. torsade-like with complexes that vary in digoxin.
The other three differential diagnoses height and appear to twist around the base- The crucial step in distinguishing between
outlined above are unusual causes of a wide line, intravenous magnesium is clearly the above-mentioned atrial, atrioventricular
complex tachycardia in children. The most recommended. If the QT interval is pro- or nodal tachycardias is an identification of
important differential to think about is longed, IV magnesium followed probably the P wave and its relationship to the
SVT with aberrant conduction, because it by IV lignocaine is recommended. If the QRS complex. This is sometimes best done
should respond to vagal manoeuvres or QT interval can be seen not to be prolonged, with a 12-lead ECG with the paper run at
adenosine administration. A previous electro- b-blockade is the primary approach, with 50 mm s1. If epicardial leads are available
cardiogram is of great value in determining amiodarone again the agent of choice if (i.e. post-operatively), an atrial ECG can be
the presence of an accessory pathway or a LV function is impaired. obtained by connecting the two atrial pacing
pre-existing bundle branch block in that leads to the right arm and right leg electrodes
these features point to SVT with aberrant Narrow complex tachyarrhythmia of a 12-lead ECG. Alternatively, a specialised
conduction. Classically, VT is a wide complex A narrow complex tachycardia is defined as transoesophageal pacing wire can be intro-
tachycardia with a left access deviation and one with a QRS duration normal for age duced, like a nasogastric tube approximately
more frequently a left bundle branch block (approximately 0.08 seconds). The narrow to the level of the nipple and then connected
pattern, whereas a right bundle branch block QRS complex almost always indicates that to lead V1 to display an oesophagoatrial ECG
pattern (with rSR in V1) is more commonly these tachyarrhythmias are supraventricular. in lead V1, with the remainder of the leads
seen in SVT. Again, the P wave morphology SVT refers to a family of tachyarrhythmias showing a normal surface ECG. Because it
is crucial and if P waves can be distinguished, requiring the atrium, AV node or both for is being recorded directly, the P wave will usu-
the loss of a one-to-one relationship between their perpetuation. SVT accounts for 90% ally appear larger than the QRS complex in
P wave and QRS complex is highly suggestive of all significant tachyarrhythmias in chil- the surface ECG and by aligning simulta-
of VT. Sometimes, however, in VT there still dren. It is most useful to subclassify these neously recorded surface and atrial ECGs
may be a one-to-one ventriculoatrial rela- tachyarrhythmias on the basis of site of ori- the position of the P relative to the QRS
tionship. The presence of fusion beats indi- gin and mechanism into primary atrial can be determined.
cating an ectopic focus below the level of tachycardias (including MAT, atrial fibrilla- Atrially-driven tachycardias will have a
the AV node is also strongly suggestive of VT. tion and atrial flutter), atrioventricular P wave preceding each QRS. If the P wave
If there is strong evidence supporting a reciprocating, AV nodal re-entry and junc- is upright in inferior leads, the origin of
supraventricular origin and preserved LV tional ectopic tachycardia. Primary atrial the tachycardia is high in the atrium,
function, vagal manoeuvres and/or adeno- and junctional ectopic tachycardias will be whereas the P wave axis will be negative
sine administration can be tried. The caveat dealt with later. Atrioventricular reciprocat- in inferior leads if there is AV nodal re-entry
is that, in doing this, there is a small but ing and nodal re-entry tachycardias are or a low atrial ectopic focus. The P wave is
important potential for hypotension and the commonest forms of SVT in childhood. usually absent in junctional ectopic tachyar-
accelerated accessory pathway conduction Atrioventricular reciprocating tachy- rhythmia or VT. An abnormal P wave mor-
from the adenosine administration that cardia Occurs due to the presence of an acces- phology usually indicates an origin other
has been responsible for conversion of SVT sory conduction pathway setting up a re-entry than the sinus node and several morpholo-
to VT, or even worse on occasions. circuit. In the commonest variety (orthodromic gies will indicate multifocal atrial tachycar-
If there are no pointers towards a supraven- reciprocating), the impulse travels antegrade dia (MAT). If the P wave follows the QRS
tricular origin, or the above manoeuvres are via the AV node to the ventricles (as usual) complex, the differential diagnosis involves
unrewarding, the diagnosis is most likely VT. and then retrograde via the accessory path- atrioventricular nodal re-entry tachyarrhyth-
If the patient is severely compromised, way back to the atria. If the accessory pathway mia and ventricular tachycardia. If the P
direct current cardioversion is clearly indi- is capable of conducting antegrade, anti- waves are dissociated from the QRS com-
cated. It is debated whether this is better dromic reciprocating tachycardia can occur, plexes, the diagnoses are most likely junc-
synchronous or asynchronous, but most with the impulse travelling in the opposite tional ectopic tachycardia or VT.
authorities recommend starting with synchro- direction, i.e. forward down an accessory path- Regardless of these diagnostic considera-
nous cardioversion if there is a pulse, using a way, returning retrograde via the AV node. tions, the approach is essentially the same. If
1 J/kg shock from a biphasic defibrillator. AV nodal re-entrant tachycardia In this the patient is acutely compromised, basic
If the patient has reasonable perfusion, tachyarrhythmia the two conduction path- life support followed by attempted DC car-
the recommended drug depends on the ways are thought to be within or adjacent dioversion with 0.51 J kg1 of DC shock
appearance of the VT. If the VT is monomor- to the AV node. Beyond 5 years of age, this (under sedation if possible) and with contin-
phic and the patient has preserved LV func- becomes the most common form of SVT. uous ECG monitoring is recommended. IV
tion, intravenous sotalol is the agent of Classically, the P wave is buried within the adenosine is an alternative if this is immedi-
choice, with amiodarone as a second-line QRS complex on ECG. In AV nodal re-entrant ately available. If the patient has adequate
agent. Amiodarone is definitely the drug tachycardia, for long-term prophylaxis ther- perfusion, consider vagal manoeuvres/
of choice if LV function is impaired. If the apy digoxin is the preferred agent. However, eliciting of diving reflex, administer adeno-
rhythm is polymorphic, there should be some b-blockers may be considered in resistant sine in increasing doses as above.

120
5.9 CARDIAC ARRHYTHMIAS
5

CARDIOVASCULAR
The importance of a continuous 12-lead class-3 antiarrhythmic actions and is effec- is avoided in children with long-standing
electrocardiogram during this process cannot tive and safe in resistant SVT. There is also atrial fibrillation, cardiac failure and/or
be over emphasised. This is because just a some evidence supporting a role for enlarged atria.
few beats of sinus rhythm before reversion flecainide (class 1C agent) or amiodarone
back to the tachyarrhythmia might well shed (class 3 agent) as maintenance treatments
considerable light on the diagnosis. If the in resistant cases.
Ventricular fibrillation
arrhythmia is resistant to adenosine, the
In confirmed ventricular fibrillation (VF), the
next step is probably use of a b-blocker, with Atrial flutter
first priority is rapid defibrillation. Basic life
digoxin an alternative for maintenance (see Atrial flutter is an atrial tachycardia that
support should be initiated while a defibril-
below for caveats regarding digoxin in WPW). probably propagates via an intra-atrial re-
lator is sought. There is increasing evidence
Failure of adenosine to convert such a entrant pathway. It is uncommon but can
that shock success is improved if effective
tachyarrhythmia makes unusual diagnoses occur in infancy, when it is usually not asso-
chest compressions are provided for 1 to 3
more likely. For multiple atrial ectopic ciated with structural heart disease. Beyond
minutes before defibrillation, especially if
foci, sotalol is effective but probably only infancy 95% of atrial flutter is associated
VF duration is longer than approximately
as a bridge to radiofrequency ablation. For with structural heart disease (Mustard/Sen-
3 minutes before attempted defibrillation.
JET the agent of choice is amiodarone. ning operation for transposition of the great
Conducting pads or conductive gel should
In atrial fibrillation/flutter, rate control arteries, Fontan, repaired total anomalous
be placed on the chest in the left 5th inter-
can be achieved using digoxin but usually pulmonary venous return).
costal space midaxillary line and second
elective cardioversion is required to termi- Clinical features may include palpitations,
intercostal space to the right of the sternum,
nate the arrhythmia. In post-surgical cases cardiac failure or no symptoms, dependent
being careful that gel areas are not contigu-
recurrence is common and sotalol probably on the rate of ventricular response. The
ous to avoid shorting of current. The paddles
has the strongest demonstrated efficacy in ECG shows a characteristic saw-tooth flutter
are applied, charged on the chest,stand clear
reducing recurrences (and controlling ven- wave at 300 beats per minute, best seen in
is called, and the operator verifies that this
tricular rate) if they occur, though only as II, III and a VF with 2:1 or 3:1 AV block.
direction has been followed by other staff.
a bridge to possible cryoablation. Infants without structural heart disease
Then 2 J kg1 of current is discharged in
usually respond to low energy (0.5 J kg1)
asynchronous mode. CPR is resumed immedi-
WolffParkinsonWhite DC cardioversion, repeated after digoxin
ately after the first shock and continued for
syndrome loading if initially unsuccessful. Atrial flutter
five cycles (about 2 minutes) before the next
WPW is present when the accessory atrio- is more resistant in the older patient who
rhythm check. If VF persists, a second shock
ventricular connection allows ventricular usually has associated structural cardiac dis-
of 4 J kg1 is administered and again CPR
pre-excitation by rapid antegrade conduc- ease. Amiodarone or class 1A agents have
immediately resumed for five cycles. Early
tion of the normal sinus impulse, thereby been shown to be effective. Radiofrequency
during these five cycles adrenaline (epineph-
avoiding the normal delay in the AV node. catheter ablation may be valuable as a more
rine) is administered and if VF persists on
This is present in 2273% of cases of pae- definitive therapy.
rhythm check a third shock of 4 J kg1 is deliv-
diatric SVT and produces a shortened PR
ered. CPR is again immediately resumed and
interval and slurred upstroke on the QRS Atrial fibrillation
if the patient remains in VF, amiodarone
complex (delta waves). It usually produces Unlike in adults, atrial fibrillation (AF) is a
5 mg kg1 by rapid IV bolus is the antiar-
orthodromic reciprocating AV tachycardia relatively rare tachyarrhythmia in infants
rhythmic of choice. This is then followed by
but in a small proportion of cases (approxi- and children.
further attempts at defibrillation while CPR
mately 10%) an antidromic reciprocating Clinical features may include irregular, rapid
is continued. Lignocaine is a second choice
tachycardia can occur. palpitations with cardiac failure if a rapid
agent, magnesium is recommended.
If WPW is known to be present (by past ventricular response is present. ECG reveals
history or presence of delta waves on a absence of discrete P waves with an irregularly
non-tachyarrhythmia ECG) b-blockers (pro- irregular narrow, rapid ventricular complex.
Controversies
pranolol or atenolol) are the agents of first Causes of AF may include atrial distension
choice (slow conduction through AV node or scars, rheumatic heart disease, hyperthy- The appropriate energy dose for
with little, if any, effect on accessory path- roidism, hypocalcaemia, poisoning, or intra- biphasic defibrillation in children is
ways). Digoxin has traditionally been the thoracic pathology. controversial.
drug of choice for prophylaxis against Management should focus on removing
There is debate about the applicability
SVT in WPW but has pro-arrhythmia risks, the cause if possible. Therapy is directed
and safety of automated external
particularly in antidromic reciprocating toward controlling the ventricular rate (pri-
defibrillators for use in young children.
tachycardia (decreases accessory pathway marily using digoxin or b-blockade to
refractory period, thereby facilitating acces- slow AV nodal conduction). Synchronised  Waveform analysis has the potential
sory connection conductance of atrial DC cardioversion is effective in conversion to reliably predict success of
arrhythmias), and should therefore be of atrial fibrillation to sinus rhythm but defibrillation and if so allow treatment
avoided. Sotalol combines b-blocker and because of the embolic risk, the procedure alteration to improve outcome.

121
5.9 CARDIAC ARRHYTHMIAS

Liebman J. Tables of normal standards. In: Liebman J,

Controversy exists around the Further reading Plonsey R, Gillette PC, editors. Pediatric
electrocardiography. 1st ed. Baltimore: Williams & Wilkins;
requirement for ongoing prophylactic Alexander ME, Berul CI. Ventricular arrhythmias: When to 1982. p. 82133.
antiarrhythmic therapy for infants with worry. Pediatr Cardiol 2000;21(6):53241. Luedtke SA, Kuhn RJ, McCaffrey FM. Pharmacologic
Anonymous. The International Liason Committee on management of supraventricular tachycardias in children.
paroxysmal supraventricular tachycardia Resuscitation (ILCOR) consensus on science with treatment Part 1, WPW and AV nodal re-entry. Ann Pharmacother
when the natural history of this condition recommendations for paediatric and neonatal patients: 1997;31:122743.
paediatric basic and advanced life support. Pediatrics Luedtke SA, Kuhn RJ, McCaffrey FM. Pharmacologic
suggests fewer attacks with age beyond 2006;117(5):e95577. management of supraventricular tachycardias in
12 months. Bink-Boelkens MTE. Pharmacologic management of children. Part 2, Atrial flutter, atrial fibrillation and
arrhythmias. Pediatr Cardiol 2000;21(6):50815. junctional and atrial ectopic tachycardia. Ann
The safety of amiodarone as Celiker A, Ayabakan C, Ozer S, et al. Sotalol in the
treatment of pediatric cardiac arrhythmias. Pediatr Int
Pharmacother 1997;31:134759.
McKee MR. Amiodarone - an old drug with new
antiarrhythmic agent of first choice 2001;43:62430. recommendations. Curr Opin Pediatr 2004;15:1939.
in children with shock-resistant Chun TU, Van Hare GF. Advances in the approach to Saul JP, Scott WA, Brown S, et al. Intravenous amiodarone for
treatment of supraventricular tachycardia in the pediatric incessant tachyarrhythmias in children: a randomized,
ventricular fibrillation has been population. Curr Cardiol Rep 2004;6:3226. double-blind, antiarrhythmia drug trial. Circulation
questioned and its replacement by Kudenchuk PJ, Cobb LA, Copass MK, et al. Amiodarone for 2005;112:34707.
resuscitation after out-of-hospital cardiac arrest due to
lignocaine suggested. ventricular fibrillation. N Engl J Med 1999;341(12):8718.

122
 6

SECTION
RESPIRATORY
Section editor Ian Everitt

6.1 Upper respiratory tract infections 123 6.5 Community-acquired pneumonia 136
6.2 Inhaled foreign body 126 6.6 Bronchiolitis 139
6.3 Acute asthma 128 6.7 Croup 142
6.4 Pertussis 134

6.1 Upper respiratory tract infections

Peter L.J. Barnett

affects part of the upper respiratory tract.

ESSENTIALS An URTI is usually caused by rhino- and cor-
onaviruses, but during the winter season,
1 Over 95% of childhood URTIs are viral and do not require antibiotics. parainfluenza, respiratory syncytial virus
2 Upper respiratory tract infections are common; children have between six and (RSV) and metapneumovirus are also com-
eight URTIs per year. mon. These later viruses may progress to
croup, bronchiolitis or pneumonia. Other
3 In the child with presumed URTI who has significant constitutional specific infections may begin with URTI
symptomatology one needs to consider alternative diagnoses.
symptoms and progress to involve the lower
4 Symptomatic nasal obstruction in infants should be treated with saline drops and respiratory tract, e.g. influenza, Bordetella
gentle bulb aspiration. It is uncommon for simple URTIs to compromise feeding in pertussis. If symptoms are persistent, an
infants; in this situation one needs to consider LRT involvement. open mind regarding alternative diagnoses
is required. In their first 6 years, children
5 Stomatitis in children is usually caused by herpes simplex or coxsackie virus and generally have between six and eight URTIs
symptomatic treatment to aid fluid intake is all that is required.
per year and children attending day care
6 Tonsillitis in children under four years is usually viral in aetiology. may have more. Not infrequently these
may occur one after the other, leading to
7 Tonsillitis is a feature of Epstein-Barr virus (EBV), thus always examine for node the impression of persistent symptoms
enlargement and organomegaly and features in the pharynx to suggest possible EBV.
rather than two to three separate infections.
Breast feeding of babies may offer some
protection, particularly in the first 6 months.

History
these infections are mild and self-limiting, This illness is characterised by mild to mod-
Introduction but complications can occur, leading to more erate fever, blocked or runny nose, sneezing,
Infections involving the upper respiratory serious disease. mild cough (generally dry in the first few
tract are the commonest infections seen in days) with an irritating sore throat if the
children and the most frequent reason for child is able to verbalise this symptom.
presenting to emergency departments
Nasopharyngitis
Infants may also become restless and irrita-
(EDs). These infections may involve anato- Introduction ble. Nasal congestion may interfere with
mical structures including the nasopharynx, Nasopharyngitis or the common cold is a feeding in infants under 6 months due to
mouth, ear and upper airway. Specific diag- viral illness of the upper respiratory tract. mechanical obstruction. Over the first few
nosis needs to be made to decide whether This is commonly called an upper respiratory days the watery nasal discharge will become
antibiotic treatment is necessary. Most of tract infection (URTI) even though it only thicker and more mucopurulent. Nasal

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6.1 UPPER RESPIRATORY TRACT INFECTIONS

obstruction leads to mouth breathing and suction device is generally helpful. While the digits in patients who suck their fingers,
increased throat discomfort. The characteris- feeding difficulty may occur due to nasal leading to a herpetic whitlow. Coxsackie
tic of the cough may change, becoming obstruction, infants with a presumed simple virus can produce ulcers involving the oral
moist after a few days. Sputum production URTI who are unable to take normal feeds mucosa and skin areas (hands, feet and
occurs but in small children is generally swal- need to be carefully checked for features buttock areas). Thrush, generally seen in
lowed rather than expectorated and hence of lower respiratory tract (LRT) involvement. infants under 3 months, involves the tongue
will not be reported by parents. The child The routine use of oral decongestants in and buccal mucosa. It can cause feeding
is not particularly unwell. If a child has sig- infants is generally unhelpful and may cause difficulties if severe but other issues need
nificant constitutional symptoms, one needs side effects. In older children symptomatic of to be considered if mild.
to consider alternative diagnoses such as nasal obstruction, topical or oral deconge-
lower respiratory infection or an influenza- stants may be used judiciously and topical Examination
like illness. Other family members may have decongestants never longer than 3 days, Children may be distressed by mouth pain
or be recovering from cold symptoms. to avoid rebound nasal mucosal swelling. and have a high fever. Hydration should
Symptoms usually peak around 13 days There is no indication for antibiotics in be assessed. Herpes causes multiple shallow
and disappear by 710 days although the this setting. Pharyngitis or tonsillitis asso- ulcers of the oral mucosa and associated gin-
cough may persist (up to 8 weeks postviral ciated with a URTI is viral in nature and will gival inflammation. Hence, the oral features
cough). Bacterial complications are uncom- not respond to antibiotics. The inappro- of herpes simplex virus infection tend to be
mon but should be considered if fever priate use of antibiotics in these patients more anterior on the gums, inner lips and
persist for longer than 35 days, ongoing may be a contributory factor to antibiotic tongue initially, and progress in a posterior
mucopurulent nasal discharge for more than resistance. Herbal remedies such as echina- direction. Coxsackie tends to cause fewer
2 weeks, child appears unwell or has lower cea, vitamin C, or zinc have not been shown ulcers in the mouth, more on the palatal
respiratory tract signs (e.g. tachypnoea, to benefit resolution of symptoms. mucosa and is less likely to cause gingivitis.
grunting, wheeze, O2 requirement). An explanation to parents of the expec- It is classically associated with vesicular
ted natural history may decrease the lesions on the hands, feet and buttocks. Oral
Examination likelihood of inappropriate seeking of anti- thrush presents with characteristic white
The child appears well with nasal discharge, biotics. Early review should be encouraged plaques on the buccal mucosa and tongue.
which may cause obstruction (particularly if their childs course deviates from that Clinical confusion may arise in the baby
in babies). Mucopurulent discharge does expected. Paracetamol is indicated if a who has recently taken milk, which may give
not indicate a bacterial cause. The tympanic child is symptomatic of fever or to diminish a similar appearance. Differentiation can be
membranes may be slightly dull or pink in the discomfort of a sore throat. made by trying to scrape off the plaques,
appearance with no evidence of fluid which either causes bleeding or they are
in the middle ear. The throat may be red unable to be removed.
but not associated with exudate or cervical
Stomatitis
lymphadenopathy. The chest is clear to Investigation
auscultation, although there may be trans- Introduction Diagnosis is clinical and usually requires
mitted upper airway sounds, originating Children with stomatitis often present to no investigations. Immunofluorescence for
from the nasal passages. The cough may emergency due to difficulties in drinking. herpes simplex may be required for isolation
be dry or moist depending on the length In children it is most commonly caused purposes or in the immunosuppressed.
of symptoms. by herpes simplex virus (gingivostomatitis)
or Coxsackie virus (e.g. hand, foot and Treatment
Investigations mouth (HFM)). These viral infections cause Viral stomatitis is self-limiting and symptom-
There is no indication for investigations in vesicular lesions, which may involve the atic treatment to reduce pain and allow
a child with common cold. buccal mucosa, gingiva, tongue, palate eating and drinking is the mainstay of
and pharynx. Fungal infection with Candida therapy. Topical anaesthetic treatment such
Treatment albicans (thrush) may be seen in neonates as Xylocaine viscous or gel 2% applied
Therapy is supportive, with explanation or immunosuppressed children. sparingly to the lesions, half an hour prior
and a management plan for parents. Parents to drinking, may be beneficial. In addition,
need to ensure the child has adequate rest History oral analgesics such as paracetamol 
and fluids to maintain hydration. Nasal Primary herpes simplex produces a severe codeine should also be used regularly. Icy
congestion/obstruction in infants may be gingivostomatitis involving most of the poles (ice lollies), cool drinks or a soft diet
improved with saline drops (one teaspoon mouth and characteristically has a few may also be an effective way to achieve
salt in one cup boiled water and allowed lesions on the outer lip area. Fever is promi- an adequate fluid intake. Frequent review
to cool and kept in refrigerator for a few days nent and can last for 710 days. The asso- is required to assess a patients hydration
only or commercial saline preparations) ciated mouth pain can result in drooling, status. The child with severe stomatitis,
into the nares prior to feeds or sleeping. In decreased oral intake and subsequent who is dehydrated, may require admission
addition, aspiration of mucus using a bulb dehydration. Oral herpes can be spread to for intravenous fluids. Aciclovir has been

124
6.1 UPPER RESPIRATORY TRACT INFECTIONS
6

RESPIRATORY
used to hasten the resolution in immuno- Examination monospot or EBV serology may be helpful,
compromised patients, but it has not been Group A Streptococcus is most likely in the although the early monospot (within 1 week
proven to be effective in normal patients older child with fever, swollen tender cervical of onset of symptoms) can be falsely nega-
admitted to hospital for hydration. nodes, isolated sore throat and florid exuda- tive, particularly in younger children.
Treatment for oral thrush is either topical tive tonsillopharyngitis. The presence of a
antifungal gel or oral drops four times a day scarlatiniform rash (red, sandpaper-textured Treatment
for 10 to 14 days. Drops should be applied skin) or a strawberry tongue is supportive Treatment of confirmed Group A streptococ-
after a feed and inserted onto the buccal of streptococcal infection. Enterovirus can cal infection with antibiotics will decrease
mucosa, not into the general oral cavity. Sys- cause, in infants/toddlers, an ulcerative or the length of symptoms approximately by
temic antifungals are only indicated in exudative pharyngitis associated with high 1 day (3 vs. 4 days) and decreases the like-
immunocompromised patients. fever, drooling and may be associated with lihood of rheumatic fever. Oral penicillin is
vomiting/diarrhoea or a fine macular rash. the drug of choice, either 250 mg bd (age
EBV (infectious mononucleosis) is common <10 years) or 500 mg bd (age >10 years)
Pharyngitis/tonsillitis in adolescents and can have a similar pha- given for 10 days. A long-acting parenteral
Introduction ryngeal appearance to bacterial infection. penicillin (benzathine benzylpenicillin) is
Pharyngitis/tonsillitis is a common infection The patient may have general swelling of also effective in children who are refusing
involving the throat, including the tonsils. the neck and face, due to marked cervical oral medication.
In children, particularly in the first 4 years, lymphadenopathy and enlargement of the Most children with a sore throat have a
a viral aetiology is most likely. Viruses that spleen and liver (Table 6.1.1). viral infection and do not require antibiotics.
can cause pharyngitis include adenovirus, Symptomatic relief using paracetamol or sol-
enterovirus, parainfluenza, or EpsteinBarr uble aspirin/salt-water gargles may be help-
virus (EBV). Investigations ful. Topical anaesthetic and oral steroids
Group A Streptococcus is the most com- The use of throat swabs in the ED remains have been shown to decrease the throat
mon bacterial infection of the throat, but controversial due to the Streptococcus A pain. Ensuring adequate hydration improves
only accounts for up to 20% of tonsillo- colonisation that occurs in up to 20% of chil- the patients general well-being. In those
pharyngitis. The incidence is age related dren and since treatment with antibiotics unable to drink and who are dehydrated,
and is uncommon in children under 4 years only decreases symptoms by 1 day. A rapid admission for intravenous fluids may be
of age. Twenty percent of children are strep test is very specific for Streptococcus required. Children with severe tonsillome-
colonised by Group A Streptococcus, hence, A infection, but not very sensitive. If a rapid galy and neck swelling from EBV can
a positive throat swab may be incidental strep test is positive, this obviates the need develop airway obstruction and may benefit
in a child with an acute viral infection. The for further testing. If one suspects EBV, a full from steroids. The threshold for antibiotic
clinical dilemma is distinguishing viral from blood examination (atypical lymphocytes) or treatment needs to be lower in high-risk chil-
acute bacterial infections. dren (indigenous) who have an increased
risk of glomerulonephritis.
History
Table 6.1.1 Features of viral and
Older children may complain of sore throat, bacterial tonsillitis
headache, dysphagia or have referred
abdominal pain. Younger children may pres- Viral Bacterial

ent with less localising symptomatology Age <4 years Age >4 years
Further reading
Dowell SF, Marcy SM, Phillips WR, et al. Principles of judicious
with fever, decreased oral intake, drooling Associated cough, Tender cervical use of antimicrobial agents for paediatric upper respiratory
or clinging to parents. Patients presenting coryza, conjunctivitis, lymphadenopathy tract infections. Paediatrics 1998;101(1):1635.
diarrhoea Dowell SF, Phillips WR, Schwartz B. Appropriate use of
with associated coryzal symptoms (e.g. antibiotics for URIs in children: Part II. Cough pharyngitis
cough, nasal congestion, etc.) are unlikely to Lymphadenopathy, Scarlatiniform rash, and the common cold. Am Fam Phys 1998;58(6):133542.
splenomegaly oedematous tonsils Monto AS. Epidemiology of viral respiratory infections. Am
have a bacterial cause for their pharyngitis. (e.g. EBV) or exudates J Med 2002;112(Suppl. 6A):4S12S.
In high-risk children (e.g. indigenous), one Heikkinen T, Jarvinen A. The common cold. Lancet 2003;36
Oropharyngeal Absence of cough (9351):519.
needs to consider the potential of post- features of EBV Del Mar CB, Glasziou PP, Spinks AB. Antibiotics for sore throat.
streptococcal glomerulonephritis as a sequela. Cochrane Database Syst Rev 2006;(4):CD000023.

125
 6.2 Inhaled foreign body
Peter L.J. Barnett

there may be respiratory arrest with apnoea

ESSENTIALS and cyanosis. Less urgent cases may have
stridor, wheeze, cough and abnormal voice
1 The presentation to an emergency department of a child with airway obstruction or cry. The child may be drooling.
due to an inhaled foreign body can be life-threatening and requires rapid assessment.
2 One needs to have a prepared approach, which differentiates the situation of total Investigations
from partial obstruction. In a stable child with partial obstruction, a
soft tissue X-ray may be useful to localise
3 One needs to be cautious to avoid the potential to convert partial upper airway
the foreign body, if radio-opaque. This is
obstruction to complete obstruction by unnecessary interventions.
best performed portably in the emergency
4 Many children who were initially symptomatic may be asymptomatic by department (ED) so that the airway is clini-
the time they are seen in the emergency department and have minimal clinical cally monitored, with ability to intervene
findings. if necessary.
5 The majority of objects are radiolucent and the radiological findings are those
secondary to the physical presence in the airway.
Treatment
6 Indication for bronchoscopy should be based on history, examination and
radiological investigations. When in doubt, consult a paediatric respiratory physician. Total obstruction
Untreated, total airway obstruction will
rapidly lead to hypoxia, loss of conscious-
ness and subsequently cardiorespiratory
arrest. In the child presenting with complete
obstruction, basic life support should be
commenced (see Chapter 2.4 on Resuscita-
Introduction Upper airway foreign bodies tion). The performance of back blows and
Foreign body (FB) aspiration usually occurs History chest thrusts to dislodge the foreign body
in children less than 3 years of age. Excep- The presentation of a child with upper air- should be followed by attempted ventilation
tions to this can exist, particularly in older way obstruction due to an inhaled foreign with bag and mask. If these methods are
children with developmental or neurological body can be life threatening and require a unable to remove the foreign body and
issues. Foreign bodies may lodge at any rapid careful assessment and management. allow ventilation, then direct visualisation
place along the airway from the hypophar- These children present with acute onset of of the larynx and Magill forceps extraction
ynx to the segmental bronchus. Upper upper airway obstruction which may be of the foreign body should occur if possible.
airway obstruction due to foreign bodies partial, total or fluctuating in severity. Oeso- In the unlikely event of a foreign body visua-
causes several deaths in Australia each year. phageal foreign bodies can cause upper lised below the cords, suctioning may expe-
The reason toddlers are more prone to aspi- airways obstruction due to adjacent airway dite its removal or, if not possible, advancing
ration is due to their general inquisitive compression, usually characterised by par- the object past the carina with an endotra-
nature, the presence of small food or non- tial obstruction and significant drooling cheal tube or bougie may allow life-saving
food objects in the general home environ- and saliva intolerance. There is generally ventilation as a temporising measure.
ment and their inability to efficiently grind no preceding history suggesting an infective
food objects (due to lack of molar teeth). cause such as croup or epiglottitis or allergic Partial obstruction
The most common inhaled foreign bodies phenomena. Occasionally, the aspiration The approach to the child with partial air-
are food materials (e.g. peanuts, carrot or incident is witnessed as a choking episode, way obstruction, but who is able to ventilate
apple pieces) and small pieces of toys. but often not, and a child is only discovered themselves to maintain adequate oxyge-
Inhaled foreign bodies are rarely radio- with breathing difficulty following an unwit- nation, needs to be cautious. One needs to
opaque, therefore, often not visible on a nessed event. avoid the potential to convert the situation
plain X-ray. Foreign bodies in the upper air- to one of complete obstruction by unneces-
way account for only 510% of all inhaled Examination sary interventions. It is best to leave the
objects, but have a higher mortality and mor- A foreign body in the upper airway presents child undisturbed and as comfortable as pos-
bidity due to their potential to cause high- with acute signs of obstruction and respira- sible. Small children are best kept in a par-
grade obstruction of the airway proximal to tory distress. The signs reflect the degree of ents arms. Oxygen should be administered
the carina. airway obstruction. In life-threatening cases if required and tolerated. Causing distress

126
6.2 INHALED FOREIGN BODY
6

RESPIRATORY
and crying will usually worsen the degree of There may be a fever and signs of collapse or
Table 6.2.1 Indications for
airway obstruction. The child needs to be consolidation if a foreign body has resulted bronchoscopy
carefully transferred from the ED to an in a segmental infection in delayed presen-
Two of the following
appropriate environment where the object tations. However, in some cases the inhaled
can be safely removed with anaesthetic, object will be occult and the chest examina- History
Coughing and choking episode and
endoscopic and surgical facilities available. tion may be completely normal. A high index cyanosis or persistent cough after choking
DO NOT attempt to remove the object by of suspicion is required, particularly if the episode

the methods mentioned above as this may history is suggestive. Examination

convert the partial obstruction to complete Unilateral wheeze or unilateral decreased air
entry
obstruction. Investigations
Investigations
The initial investigation of the child with Hyperinflation on expiration chest X-ray or
suspected inhaled foreign body should subsegmental atelectasis or positive
fluoroscopy
Lower airway foreign body include a plain chest X-ray. The majority
History of objects are radiolucent and the radio-
The majority (70%) of patients presenting logical findings are those secondary to the
with an inhaled foreign body have a clear physical presence in the airway. This may Children who have had a minor choking
history to suggest an inhalation. However, be normal or may show signs of unilateral episode but no ongoing symptoms or signs
up to 30% have a delayed presentation hyperinflation. and a normal X-ray can be discharged.
and/or no history of foreign body aspiration. Good quality inspiratory and expiratory Parents need to be instructed to return
Patients generally present with sudden X-rays are helpful. If there is air trapping, should any relevant symptoms evolve
onset of coughing and choking, occasionally then the affected lung or part of the (wheeze, persistent dry cough, etc.).
associated with a brief period of cyanosis. lung remains inflated in expiration. There If a child re-presents with ongoing symp-
After the initial coughing episode, the child may be segmental or lobar collapse distal toms after previous referral to a tertiary
may have an audible wheeze and/or a per- to the foreign body particularly with centre, clarify that an adequate assessment
sistent dry cough. However, many children delayed presentation. Fluoroscopy, if avail- was made to rule out a foreign body at
who were initially symptomatic may be able, shows decreased movement of the that review.
asymptomatic by the time they are seen in diaphragm on the affected side.
the ED. Depending on the size of the foreign Persistent changes on X-ray or recurrent
body the symptoms may be mild. Foreign pneumonia in the same place may signal Prevention
bodies tend to lodge in the right main stem an occult foreign body.
Children under 3 years should be protected
bronchus more than the left, due to its less
from access to toys with small parts or
acute deviation from the lumen of the tra- Treatment
foods requiring molar function, as they are
chea. A patient may also suddenly deterio- Initially, the patients airway and breathing
potential inhalation risks (e.g. peanuts).
rate as the foreign body is coughed from should be assessed and oxygen given if
one bronchus to the other causing increas- required. Referral for bronchoscopy is the
ing degree of lower airway obstruction. definitive treatment for confirmed or suspi-
There may be a history of persistent cough cious cases. Indications for bronchoscopy
Further reading
Digoy GP. Diagnosis and management of upper aerodigestive
or chestiness after a remote choking event. should be based on history, examination tract foreign bodies. Otolaryngol Clin N Am 2008;41:
and radiological investigations 2 of 3 rule 48596.
Heyer CM, Bollmeier ME, Rossler L, et al. Evaluation of clinical,
Examination (Table 6.2.1). radiologic, and laboratory prebronchoscopy findings in
Examination usually reveals a well child with Patients should then be referred to an children with suspected foreign body aspiration. J Ped Surg
2006;41:18828.
mild to moderate respiratory distress. The appropriate service, which can perform a Midulla F, Guidi R, Barbato A, et al. Foreign body aspiration in
patient may have a cough. Auscultation rigid bronchoscopy to remove the foreign children. Pediatr Int 2005;47:6638.
Malick MS, Khan AR, Al-Bassam A. Late presentation
may reveal diminished air entry on one side body. When in doubt, consult with a pae- of tracheobronchial foreign body aspiration in children.
compared to the other  unilateral wheeze. diatric respiratory consultant. J Trop Pediatr 2005;51(3):1457.

127
 6.3 Acute asthma
Colin V.E. Powell

a previous history of asthma or where

ESSENTIALS asthma seems the most likely diagnosis,
one can perform a primary assessment of
1 Acute asthma is one of the commonest paediatric presentations to an emergency severity and institute the initial treatment
department.
at the onset of history taking.
2 It is important to understand the patterns of asthma in children infrequent It is important to understand the patterns
episodic, frequent episodic, and persistent. of asthma in children infrequent episodic,
frequent episodic, and persistent.1 The pat-
3 There are three major groups of factors contributing to high risk asthma in tern of asthma determines the need for pre-
children: previous severe asthma, management issues and psychological factors.
ventive therapy. When a child is discharged
4 The most important parameters in the assessment of the severity of childhood from the emergency department (ED) or
asthma are the general appearance/mental state and work of breathing. ward, consideration of the childs preventa-
tive treatment is essential.
5 Life-threatening asthma is characterised by: silent chest, cyanosis, poor respiratory
effort, exhaustion and altered mental state.
Infrequent episodic asthma
6 The main therapies for paediatric asthma include oxygen, inhaled bronchodilators Infrequent episodic asthma (IEA) is the most
and steroids modified according to severity. common pattern, accounting for 70 to 75%
of children with asthma. In this pattern,
7 Discharged patients should be given a clear asthma action plan and have children have isolated episodes of asthma
appropriate follow up organised.
lasting from 1 to 2 days up to 1 to 2 weeks,
8 There are many areas of contention regarding the management of asthma in usually triggered by an upper respiratory
children and the recommended guidelines vary between countries. One should be tract infection (URTI) or an environmental
familiar with the local paediatric guidelines. allergen. The episodes are usually more than
6 to 8 weeks apart and these children
are asymptomatic in the interval periods.
They require management of the individual
Practice is highly variable, particularly for episode only and regular preventive therapy
Introduction severe to critical acute asthma.13 is unnecessary. Within this group there is
The National Asthma Campaign for Australia a wide range of severity. Most are mild,
updated the 2002 guidelines in 20061 and but this group accounts for up to 60% of
this forms the basis of the chapter. However, History paediatric hospital admissions for asthma.1
there are other best practice guidelines2,3
Consider acute asthma when a child pre-
and national guidelines47 and these are Frequent episodic asthma
sents with signs of increase work of breath-
important resources for cross-reference and Frequent episodic asthma (FEA) accounts for
ing, widespread wheezing and shortness of
comparison to highlight the controversies. approximately 20% of childhood asthma.
breath. There are other causes to consider
Acute asthma is one of the commonest This pattern is similar to IEA but the interval
such as mycoplasma pneumonia, aspiration,
reasons for presentation to an emergency between episodes is shorter, less than 6
inhaled foreign body, and cardiac failure
department and admission to a hospital. to 8 weeks, and the children have only mini-
(Table 6.3.1). In the setting of a child with
A recent review of admissions to nine pae- mal symptoms, such as exercise-induced
diatric emergency departments in Australia wheeze, in the interval period. These chil-
and New Zealand, examining over 300 000 Table 6.3.1 Differential diagnosis dren may benefit from regular preventive
of asthma
presentations, demonstrated that acute therapy such as low dose (not greater than
asthma was the fourth most common Acute Chronic 400 mcg per day) inhaled corticosteroids
presentation, accounting for 3.5% of the Bronchiolitis, Cystic fibrosis
or leukotriene antagonist. Commonly, these
total number of presentations.8 It is well mycoplasma children are troubled through the winter
recognised that in many cases admission Allergy Cilial dyskinesia
months only and may require preventive
to hospital may be preventable9 if managed treatment for that part of the year.1
Aspiration Immune deficiency
effectively by the family and medical team
involved with a childs care. There are still Heart failure Bronchiectasis Persistent asthma
great gaps between best practice guidelines Foreign body Airway abnormalities
Persistent asthma (PA) accounts for 510%
and what actually happens in practice.1012 of childhood asthma. These children can

128
6.3 ACUTE ASTHMA
6

RESPIRATORY
have acute episodes like the categories state and work of breathing (accessory mus- consideration of foreign body. Consider other
above, but they also have symptoms on most cle use, recession), as indicated in Table 6.3.2. causes of wheeze (e.g. bronchiolitis, myco-
days in the interval periods. These symptoms Initial SaO2 in air, heart rate and ability to plasma, aspiration, heart failure, or foreign
commonly include: sleep disturbance due talk are helpful but less reliable additional body). Chronically wheezy children may have
to wheeze or cough, early morning chest features. Wheeze intensity, pulsus paradoxus, a diagnosis other than asthma, such as cystic
tightness, exercise intolerance and sponta- and peak expiratory flow rate are not reli- fibrosis, cilial dyskinesia, immune dysfunction,
neous wheeze. Again, there is a wide range able.2 Clinical signs of acute asthma cor- developmental/congenital abnormality, upper
of severity in this group, ranging from relate poorly with the severity of the airway problems or bronchiectasis. There may
those with mild symptoms 4 to 5 days per asthma attack and none of the signs be clues in the family or perinatal history or
week readily controlled with low-dose corti- in isolation are predictive of severity.4 symptoms and signs that may suggest an
costeroid preventive therapy, to those with Classification of an acute attack, using the alternative diagnosis to asthma.4
frequent severe symptoms and abnormal NAC Australia guidelines1 is as follows:
lung function requiring intensive therapy.1
Investigations Treatment
Chest X-ray is not generally required in Treatment mild
Acute episode children with asthma, unless one has the sus- Salbutamol by MDI/spacer (see below)
Mortality from acute asthma is fortunately picion of an alternative diagnosis or compli- once and review after 20 minutes.
rare confidential enquiry into deaths from cation (air leak or atelectasis). Arterial blood Ensure device/technique appropriate.
asthma suggests that there are three major gas and spirometry are rarely required in the Good response discharge on b2 agonist as
factors contributing to the death. assessment of acute asthma in children. needed. Poor response treat as moderate.
The severity of the disease: most children Oral prednisolone (1 mg kg1 daily for
who have died from asthma have persistent Differential diagnosis 13 days) if on prophylaxis or episode
asthma; however, a minority of children who During an acute episode of wheezing, has persisted over several days.
have died have only mild to moderate asymmetry on auscultation is often found Provide written advice /action plan on
disease.4,14,15 Previous near fatal asthma, due to mucous plugging, but warrants what to do if symptoms worsen.
previous admission in the last year, previous
admission to padiatric intensive care unit Table 6.3.2 Severity of asthma
(PICU), heavy use of b2 agonists, repeat
Symptoms Mild Moderate Severe and life-
attendances to the ED are all risk factors threatening*
associated with risk of a severe attack, near
Altered consciousness No No Agitated
fatal asthma or death.4 Confused/drowsy
Medical management: inadequate
Accessory muscle use/ No Minimal Moderate
treatment steroids, heavy or increasing recession Severe
use of b2 agonists, inadequate
Oximetry on >94% 9490% <90%
monitoring of the asthma and underuse presentation (SaO2)
of written asthma plans and delay in
Talks in Sentences Phrases Words
seeking help, have all been associated Unable to speak
with deaths from asthma.4,14,15
Pulsus paradoxus Not palpable May be palpable Palpable
Psychological factors: non-adherence,
poorly perceived symptoms, failure to Pulse rate <100 100200 >200
attend clinics, conflict between child and Central cyanosis Absent Absent Likely to be present
parent or medical staff, family dysfunction
Wheeze intensity Variable Moderateloud Often quiet
are all risk factors associated with risk of
death from asthma.4,16 Peak expiratory flow >60% 4060% <40%
Unable to perform
A child should be considered as high risk when FEV1 (% predicted) >60% 4060% <40%
attending the ED with an acute exacerbation Unable to perform
of their asthma when there is a combination Arterial blood gases Test not necessary If initial response is poor If initial response is
of features of previous severe asthma and poor
Yes
one or more adverse psychological factors.
*Life-threatening asthma is characterised by:3
silent chest
Examination cyanosis
poor respiratory effort
The most important parameters in the hypotension
exhaustion
assessment of the severity of acute childhood confusion
asthma are general appearance/mental coma

129
6.3 ACUTE ASTHMA

Consider overall control and familys Following loading dose give continuous Emergency attendance or admission
knowledge. Arrange follow up as infusion (19 years: 1.1 mg kg1 hr1, 10 should provide the patient and family
appropriate (see discharge pack). years: 0.7 mg kg1 hr1; commence in ED if with the opportunity to use an
there will be >2 hours before transfer to appropriate size space device and pMDI.
Treatment moderate ward). Make sure the child and family can use
Give O2 if O2 saturation is <92%. Need Magnesium sulfate 50% 0.1 mL kg1 the device adequately and know the
for O2 should be reassessed. (50 mg kg1) IV over 20 minutes then importance of using it for all
Salbutamol by MDI/spacer (see below) 3 0.06 mL kg1 hr1 infusion with serum preventative therapy and treatment for
doses, 20-minutely; review 1020 minutes level 1.52.5 mmol L1. significant exacerbations.
after 3rd dose and decide on admission or Involve senior staff, discuss with local PICU.  Family education.
discharge. Where intubation is indicated, ketamine On discharge from the ED or ward it
Oral prednisolone (1 mg kg1 daily for is the induction agent of choice due to its is important that families understand the
3 days). bronchodilator action. immediate management of their childs
The few children of moderate severity asthma. It is not appropriate to educate
who can go home must be discussed with Disposition them on all aspects of asthma during an
a senior doctor and should not leave ED acute episode. This is best reserved for a visit
until at least 1 hour after their last spacer Each child should have a written action plan to an outpatient clinic or local doctor at a
treatment. on discharge from the ED. Observe the time remote from the acute episode when a
Arrange home treatment and follow up childs inhaler technique before discharge. reasonable amount of time can be allocated
as above. Advise parents to seek further medical and it is more likely that the information will
review should their childs condition deterio- be understood and retained.
Treatment severe rate or if there is no significant improvement Prescription.
Oxygen. within 48 hours. At discharge all patients A prescription for all medications should
Salbutamol by MDI/spacer (see below) or should have an outpatient appointment or be provided at the time of discharge.
nebuliser 3 doses 20-minutely; review appropriate follow up arranged with a pae- Follow up.
ongoing requirements 1020 minutes after diatrician or local doctor as appropriate. All patients should have a clear follow-
3rd dose if improving reduce frequency, Parents should be informed of other sources up plan. For some it will be appropriate
if no change continue 20-minutely, if of information about asthma such as the that they visit their local doctor for an
deteriorating at any stage treat as life Asthma Foundation. The concept of an early review, particularly if their condition
threatening. asthma discharge pack is useful to ensure all deteriorates or fails to improve
Ipratropium bromide (see below) by aspects of discharge are considered. Adult significantly with 48 hours. At discharge
MDI/spacer or nebuliser. Three doses in data suggest that self-monitoring, regular all patients should have an outpatient
the first hour every 20 minutes. review and written action plans can improve appointment or appropriate follow up
Oral prednisolone (1 mg kg1 daily); if outcomes.17 Two paediatric studies suggest arranged with a paediatrician within 4 to
vomiting give intravenous (IV) that an intensive nurse-led discharge con- 6 weeks. This visit will be used for medical
methylprednisolone 1 mg kg1 or centrating on education, written action plans review and, most importantly, appropriate
hydrocortisone 4 mg kg1 6-hourly. and inhaler technique, appropriate follow up education about asthma management.
Involve senior staff. with discharge prescription for steroids can Written action plan.
Arrange admission after initial assessment. reduce readmissions and following mor- All patients should have an individual
bidity.18,19 A child should be ready for dis- written action plan and the discharging
Treatment life threatening charge when it is considered that they can doctor should spend time going over the
Oxygen. be stable on 34-hour inhaled bronchodila- plan with the family.
Continuous nebulised salbutamol tors.20 This is often a subjective decision. Communicate with the family doctor.
(0.5% undiluted). For every emergency attendance or
Nebulised ipratropium 250 mcg three times
Discharge pack
discharge, there should be
in 1st hr (20-minutely, added to salbutamol). communication with the patients local
Methylprednisolone 1 mg kg1 or Review need for preventative treatment. doctor. The local doctor should receive a
hydrocortisone 4 mg kg1 6-hourly. Consider preventative treatment if copy of the action plan.
If deteriorating give IV salbutamol there are wheezing attacks less than
15 mcg kg1 over 10 minutes then 6 weeks apart, the attacks becoming
15 mcg kg1 min1. more frequent and severe or there
Prognosis
If poor response to IV salbutamol, consider are Increasing interval symptoms.
aminophylline 10 mg/kg IV (maximum Initial preventative treatment for Those with episodic asthma tend to improve
dose 250 mg over 60 minutes). If currently frequent episodic asthma is inhaled throughout childhood, with asthma resolv-
taking oral theophylline, do not give IV steroids. ing by their adult years in approximately
aminophylline in ED take serum level. Check inhaler technique. two-thirds. Those who continue to wheeze

130
6.3 ACUTE ASTHMA
6

RESPIRATORY
tend to have very mild asthma and maintain Maternal smoking during pregnancy has similar dosing recommendations for
normal lung function. On the other hand, an effect on lung development and infants nebulised bronchodilator; between
those with persistent asthma in childhood whose mothers smoke are four times more 2.5 mg and 5 mg of salbutamol per
are more likely to continue to wheeze in their likely to develop wheezy illness during the nebuliser every 20 minutes17 or frequent
adult years (about two-thirds), with some first year of life and thus environmental doses of 510 mg of nebulised
impairment of lung function. Available evi- tobacco smoke should be avoided.4 teburtaline.4 There is little evidence for
dence suggests that treatment does not influ-
If an organised discharge from hospital is any benefit for continuous nebulised (0.5%
ence the natural history of childhood asthma.1
completed as per recommendations (as undiluted salbutamol) treatment compared
above), then this is more likely to prevent to frequent intermittent doses of
Prevention readmission or re-presentation and reduce treatment4 although many CPG
morbidity. A number of preventable factors recommend use in life-threatening
There are two areas of prevention to con- associated with admission have been identi- exacerbations.13 Indeed all the sites in
sider but the evidence is mostly inconclusive fied and these issues should be addressed at the PREDICT study used continuous
and confusing.4 discharge: adherence issues, prophylactic nebulised bronchodilator treatment in life-
treatment, action plan use and advice to threatening asthma and 64% of sites
Primary prophylaxis recommended use in severe attacks.13
prevent delay in seeking medical advice.9
These are interventions aimed at preventing There are some interesting data in the
the development of asthma and reduce its adult literature addressing the use of long
incidence. There is insufficient evidence to acting b agonists in acute asthma, e.g.
Controversies
make recommendations about aeroallergen formoterol, which has a rapid onset of action
avoidance during pregnancy or early infancy, Bronchodilator aerosol delivery and long duration of effect6 (see Research/
use of modified infant milk formulae, pre- and Pressurised metered dose inhalers
Future Directions section). Current BTS
probiotic use, fish oil and other nutritional (pMDI) and spacers are an effective way of
recommendation are, however, to stop long
supplement use, immunotherapy and avoid- delivering inhalation medication to treat
acting b2 agonists if short acting b2 agonists
ing air pollutants. However, the only evi- mild to moderate and probably severe
are required more than 4-hourly.4
denced-based recommendations in this attacks of asthma.21 Successful
area are that: implementation22,23 and sustained use of Ipratropium bromide
spacers after changeover from nebulisers Repeated early doses of ipratropium
breast feeding should be encouraged as has been demonstrated.24 There are fewer bromide during the first 12 hours of
this will reduce the likelihood of presentation are associated with improved
side effects such as tachycardia, vomiting
developing asthma, particularly in outcomes;25 efficacy and safety have been
and hypoxia compared to when a drug is
families with an atopic history;4
given via a nebuliser.21 There is still debate demonstrated. It is recommended for
parents and parents-to-be should be
as to cost in different countries and children with severe and life-threatening
advised not to smoke as, particularly attacks17 but often overused in children
whether the pMDI/spacer combination
maternal smoking antenatally and with moderate and mild attacks10 with
use in adult acute asthma is as effective as
postnatally is associated with increased
nebulisers.21 The pMDI/spacer 37% of physicians reporting its use in
risk of infant wheeze.4 moderate asthma exacerbations and 3%
combination is used less frequently in
adult patients.10 For mild and moderate use in mild.13 Although doses of inhaled
Secondary prophylaxis ipratropium bromide are recommended
asthma all of the eleven Clinical Practice
These are interventions aimed at reducing for use with a metered dose inhaler and
Guidelines (CPG) in the PREDICT study
the impact of the disease once developed. spacer13,5 there are no data
recommended MDI and spacers; for severe
A number of non-pharmacological methods demonstrating that these doses are
asthma half of the CPG recommended
of secondary prophylaxis are debated: for appropriate and this is a consensus
spacer delivery and for critical asthma
example, allergen avoidance, house dust
spacers were not recommended at all.13 In statement recommendation. Different
mite control measures, avoiding smoking doses are recommended.
the UK there has been a significant
and air pollution, antioxidant and mineral Standard doses of 250500 mcg per
increase in the use of spacers in the
supplementation, fish oils and fatty acids dose for nebulised treatment seems
hospital setting despite the asthma
use, breathing exercises, chemotherapy universal.17 Repeated doses of
severity remaining stable over the last
and immunotherapy. Recent evidence-based
decade.12 There are no data on the use of ipratropium bromide should be given
recommendations are that: early in children who are not responsive to
spacers in severe to life threatening
In committed families, house dust mite asthma but clearly they are being used in b2 agonists.4 It is not clear whether there
avoidance by use of bed covers, removal the severe group.13 are any benefits from continued use after
of carpets and soft toys, dehumidification, It probably doesnt make much the attack has shown response to
high temperature washing of bed linen, difference what doses are given; if there is treatment. Once hospitalised, the
and use of acaricides on soft furnishing a response, cut down the dose, if there addition of ipratropium to salbutamol
may reduce morbidity from asthma.4 is no response, increase it! There are and steroids appears to add no benefit.26

131
6.3 ACUTE ASTHMA

BTS recommend that the dose should be intravenous magnesium sulfate in severe work in children with intermittent
weaned down to 46-hourly or stopped and critical exacerbations is discussed in the asthma.37 There is no clear evidence that
once admitted.4 BTS guidelines but not fully endorsed4 and there is a role as intravenous therapy in
GINA5 does not recommend its use acute asthma exacerbations; only adult
 Intravenous bronchodilators
routinely in children but mentions it may be data exist.38 Current studies are reviewing
The main problem with deciding which
of benefit in severe asthma (no dose the use of leukotriene antagonists in the
intravenous bronchodilator to use is that
discussed). Magnesium is not mentioned in management of acute asthma in children.
there are no good direct comparisons with
the other guidelines2,3,6,7 for use in children.
the three intravenous treatments
It is clearly being used in EDs in Australia Children less than 2 years of age
commonly used. There are certainly drug This age group can be difficult to assess
and New Zealand in 50% of critical and
versus placebo studies but no head to head and the different phenotypes of acute
18% of severe exacerbations.13
studies to endorse recommendations. Thus wheezing and indeed different labels
Nebulised magnesium may have a role
the referenced guidelines vary with used in different countries can cause
in adult asthma but there are few data on
recommendations.17 The role of problems when examining the
its role in children.6 None of the guidelines
intravenous bronchodilators in addition to literature on appropriate treatment.4 In
recommend its use. A multicentre double
nebulised treatment remains unclear.27 the recent update of the BTS guidelines
blind RCTstudy of nebulised magnesium is
Salbutamol continuous intravenous where asthma is considered to be the
currently under way in the UK.32
infusions are recommended following on likeliest diagnosis, b2 agonists delivered
Adrenaline this bronchodilator is not
from a bolus or initial infusion, if the child by spacer and pMDI is the optimum
mentioned in the paediatric guidelines.15
does not respond, but different doses are delivery device; oral b2 agonists are not
However, the GINA guidelines suggest it
recommended:28 recommended. There is little evidence
may be of use.6 There is no doubt that some
The guidelines from the Global that b2 agonists or ipratropium bromide
acute asthma may be anaphylaxis33 and
Initiative for Asthma (GINA) do not have an impact on wheezy children of this
intramuscular adrenaline (epinephrine)
recommend intravenous b2 agonists, age in regard to their need for
may well have a role.6 The dose suggested
stating there is no evidence for their hospitalisation or length of stay.4,39
is 0.01 mg kg1 up to 0.30.5 mg of
benefit25 and the AAP do not mention Steroid therapy may have a role but this is
1:1000 adrenaline (epinephrine) every 20
intravenous therapy.7 still not clear.4 There are problems
minutes for three doses for acute asthma.5
Aminophylline there is no evidence differentiating between bronchiolitis and
This treatment is very rarely used in acute
that there is a role for aminophylline asthma in younger infants and this is
asthma in a paediatric population.10
in children with mild to moderate outside the scope of this chapter.
exacerbations of their asthma. However, Corticosteroids
if a child is unresponsive to maximal There is no doubt that early use of Intensive care management
inhaled therapy as above, then in the steroids for an acute attack reduces not only The NAC and BTS recommendation is
more severe and life-threatening attacks, the need for admission but also that once a child has severe enough
aminophylline has been shown to have morbidity.4,34 If a child can take a dose orally asthma to require intravenous treatment
an effect on outcome (intubation).29,30 there is no benefit to administering it they should be referred to a PICU or an
This is clearly a controversial area. intravenously.33,35 There are a number PHDU even if they do not require
There is no doubt that vomiting is a of different dosing regimens suggested: intubation.1,4 There are a number of
serious side effect from the higher doses A 2-day course of dexamethasone has been indications for intensive care admission:
of aminophylline and this may be the shown to be as effective as a 5-day course deteriorating lung function, persisting or
reason not to use it.29 of prednisolone and there may be some worsening hypoxia, hypercapnia,
Magnesium intravenous suggestions that this may help adherence.36 exhaustion, drowsiness, confusion, coma
magnesium relaxes smooth muscle and Inhaled steroids there is or respiratory arrest.4 These features are
causes bronchodilation. Its exact place in insufficient evidence in children that clearly all part of a spectrum and the
the treatment of acute asthma in inhaled steroids should replace oral overall picture, plus lack of response to
children has yet to be established. Doses or systemic steroids in acute treatment, should indicate that the child
of 40100 mg kg1 as a 20-minute exacerbations.4 There are some adult should be admitted to a high dependency
infusion have been used with varying data to suggest that high-dose inhaled or intensive care area. They may not
effects on lung function and asthma steroids may have a value.5 necessarily require ventilation. There are
severity scores when compared to no absolute criteria and the decision needs
placebo.31 NAC recommend using 50% Leukotriene antagonists to be made by an experienced physician/
0.1 mL kg1 (50 mg kg1) IV over 20 Initiating oral leukotriene antagonists in anaesthetist.4,6 Non-invasive ventilation
minutes for severe and life-threatening primary care early at the onset of an has had some success in adults but none of
episodes, followed by an infusion of exacerbation can result in reduced the paediatric guidelines recommend its
0.06 ml kg1 hr1 (30 mg kg1 hr1): target symptoms, time off school, healthcare use. Detailed discussion about ventilating
serum levels 1.52.5 mmol L1.1 The use of resource utilisation and parental time off a child with asthma is beyond the scope of

132
6.3 ACUTE ASTHMA
6

RESPIRATORY
24. Cheng NG, Browne GJ, Lam LT, et al. Spacer compliance
this chapter. Helium and oxygen mixtures References after discharge following a mild to moderate asthma
attack. Arch Dis Child 2002;87(4):3025.
have been used in adults but again are not 1. National Asthma Council Australia Asthma Management 25. Plotnick LH, Ducharme FM. Combined inhaled
endorsed in the paediatric guidelines on Handbook. http://www.nationalasthma.org.au/; 2007. anticholinergic agents and beta-2 agonists for initial
2. Royal Childrens Hospital, Melbourne. clinical guidelines.
current evidence.6 Available from http://www.rch.unimelb.edu.au/
treatment of acute asthma in children (Cochrane
Review). In: The Cochrane Library. Issue 3. Oxford:
clinicalguide/; 2010 [accessed 13.10.10]. Update Software; 2001.
3. Sydney Childrens Hospital Randwick NSW. Australia. 26. Goggin N, Macarthur C, Parkin PC. Randomized trial of
Available from http://www.sch.edu.au; 2010 [accessed the addition of ipratropium bromide to albuterol and
13.10.10]. corticosteroid therapy in children hospitalized because of
4. British Thoracic Society and Scottish Intercollegiate an acute asthma exacerbation. Arch Pediatr Adolesc Med
Future directions/ Guidelines Network updated. Available from http://
www.sign.ac.uk/guidelines/published/; 2009
2001;155:132934.
27. Travers AA, Jones AP, Kelly KD, et al. Intravenous beta2-
research [accessed 13.10.10]. agonists for acute asthma in the emergency department.
5. Canadian Association for Emergency Physicians. Cochrane Database Syst Rev 2001;(1). Art. No.:
The following list is a number of Available from http://www.cps.ca/; 2009 [accessed CD002988. doi:10.1002/14651858.CD002988 .
13.10.10]. Cochrane Database Syst Rev 2010 Issue 1, Copyright #
themes needing further development 6. Global Strategy for Asthma Management and 2010 The Cochrane Collaboration.
in acute asthma it is by no means Prevention. Available from http://www.ginasthma.com; 28. Browne GJ, Penna AS, Phung X, Soo M. Randomised trial of
2008 [accessed 13.10.10]. intravenous salbutamol in early management of acute
exhaustive. 7. Hegenbarth MA. American Academy of Pediatrics severe asthma in children. Lancet 1997;49(9048):3015.
Preparing for Pediatric Emergencies. Pediatrics 29. Yung M, South M. Randomised controlled trial of
Prevention of developing asthma and 2008;121:43343. aminophylline for severe asthma. Arch Dis Child
8. Acworth J, Babl F, Borland M, et al. Patterns of 1998;79:40510.
secondary prevention can we reduce presentation to the Australian and New Zealand 30. Ream RS, Loftus LL, Albers GM, et al. Efficacy of IV
asthma morbidity? Pediatric Research Network. Emerg Med Australas theophylline in children with severe status asthmaticus.
2009;21:5966. Chest 2001;119:14808.
Patient education and asthma care 9. Ordonez GA, Phelan PD, Olinsky A, Robertson CF. 31. Rowe BH, Bretzlaff J, Bourdon C, et al. Magnesium sulfate
Preventable factors in hospital admissions for asthma. for treating exacerbations of acute asthma in the
delivery how best to deliver asthma Arch Dis Child 1998;78(2):1437. emergency department. Cochrane Database Syst Rev
care and how best to improve patients 10. Kelly AM, Powell CVE, Kerr D. Snapshot of acute asthma: 2000;(1). Art. No.: CD001490. doi:10.1002/14651858.
treatment and outcome of patients with acute asthma CD001490. Cochrane Database Syst Rev 2010 Issue 1,
and families knowledge? treated in Australian emergency departments. Intern Med J Copyright # 2010 The Cochrane Collaboration.
2003;33:40613. 32. Powell CVE. A randomised, placebo controlled study of
Gaps in practice and delivery of care how 11. Powell CV, Raftos J, Kerr D, et al. Asthma in emergency nebulised magnesium in acute severe asthma in children.
departments: combined adult versus paediatric only Available from http://www.controlled-trials.com/
best to audit asthma care and improve centres. Paediatr Child Health 2004;40:4337. ISRCTN81456894 [accessed 13.10.10].
implementation of guidelines? 12. Davies G, Payton JY, Beaton SJ, et al. Children admitted 33. Rainbow J, Browne GJ. Fatal asthma or anaphylaxis?
with acute wheeze/asthma during November Emerg Med J 2002;19(5):4157.
Improved assessment of acute asthma 19982005: a national audit. Arch Dis Child 34. Smith M, Iqbal SMSI, Rowe BH, NDiaye T. Corticosteroids for
2008;93:9528. hospitalised children with acute asthma. Cochrane Database
what is the best predictor of asthma 13. Babl FE, Sherriff N, Borland M, et al. Paediatric asthma Syst Rev 2003;(1). Art. No.:CD002886 . doi: 10.1002/
severity, need for treatment and management in Australia and New Zealand: practice 14651858.CD002886. Cochrane Database Syst Rev 2010
patterns in the context of clinical guidelines. Arch Dis Issue 1, Copyright # 2010 The Cochrane Collaboration.
hospital admission? Child 2008;93:30712. 35. Barnett P, Caputo GL, Baskin M, et al. Intravenous versus
14. Robertson CF, Rubinfield AR, Bowes G. Pediatric asthma oral corticosteroids in acute pediatric asthma. Ann Emerg
What should be the core outcomes deaths in Victoria: the mild are at risk. Pediatr Pulmonol Med 1997;29:2127.
1992;12:95100. 36. Qureshi F, Zaritsky A, Poirier MP. Comparitive efficacy of
measured for acute asthma research 15. Martin AJ, Campbell DA, Gluyas PA, et al. Characteristics oral dexamethasone versus oral prednisolone in acute
in children? of near fatal asthma in children. Pediatr Pulmonol pediatric asthma. J Paediatr 2001;139:206.
1995;20:18. 37. Robertson CF, Price D, Henry R, et al. Short course
Are there different phenotypes of acute 16. Strunk RC, Mzrazek DA, Wolfson Fuhrmann GS, Montelukast for intermittent asthma in children: A
LaBrecque JF. Physiologic and psychological randomize controlled trial. Am J Respir Critical Med
asthma with potentially different characteristics associated with deaths due to asthma in 2007;175:3239.
treatment modalities? childhood. JAMA 1985;254:11938. 38. Camargo CA, Smithline HA, Marie-Pierre M, et al.
17. Gibson PG, Powell H, Wilson A, et al. Self-management A randomized controlled trial of intravenous montelukast
education and regular practitioner review for adults with
Do long-acting b2 agonists have a role in asthma. Cochrane Database Syst Rev 2002;(3). Art. No.:
in acute asthma. Am J Respir Crit
Care Med 2003;167:52833.
treating acute asthma? CD001117. doi:10.1002/14651858.CD001117. 39. Everard M, Bara A, Kurian M, et al. Anticholinergic drugs for
18. Madge P, McColl J, Payton J. Impact of a nurse led home wheeze in children under the age of two years. Cochrane
Pharmacogenomics are there different management training programme in children admitted Database Syst Rev 2005;(3). Art. No.: CD001279. doi:
to hospital with acute asthma. A randomized controlled 10.1002/14651858.CD001279.pub2.
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treatment? 19. Wessldine LJ, McCarthy P, Silverman M. Structured
discharge procedure for children admitted to hospital
with acute asthma. A randomized controlled trail of
Which is the most effective intravenous nursing practice. Arch Dis Child 1999;80:1104.
bronchodilator? 20. Storman MO, Mellis CM, Van Asperen PP, et al. Outcome
evaluation of early discharge of asthmatic children from
What is the best mode of delivery for hospital: a randomized control trial. J Qual Clin Pract
1999;19:14954.
aerosolised treatments? 21. Cates CJ, Crilly JA, Rowe BH. Holding chambers (spacers)
versus nebulisers for beta-agonist treatment of acute
Non-invasive ventilation where does asthma. Cochrane Database Syst Rev 2006;(2). Art. No.:
CD000052. doi:10.1002/14651858.CD000052.pub2.
this have a role? 22. Powell CV, Maskell GR, Marks M, et al. Successful
implementation of spacer treatment guidelines for acute
What is the role of inhaled magnesium, asthma. Arch Dis Child 2001;84:1426.
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2001;174:3947.

133
 6.4 Pertussis
Nigel W. Crawford Colin V.E. Powell

Stage 1: Catarrhal (12 weeks)

ESSENTIALS Upper respiratory tract infection symptoms,
e.g. rhinorrhoea, conjunctivitis, malaise and
1 Pertussis infection is most common and severe in infants less than 6 months low-grade fever.
of age.
2 All children less than 6 months with pertussis should be admitted for a period Stage 2: Paroxysmal (26 weeks,
of observation in order to assess the risk of significant apnoea. can be longer)
Consists of paroxysms of coughing which
3 Three stages of illness occur catarrhal, paroxysmal and convalescent.
may be followed by an inspiratory whoop.
4 The mainstay of treatment is supportive. Admission allows for apnoea monitoring, Facial suffusion, with prominent eyes and
oxygen and overcoming feeding difficulties. protrusion of neck veins, may be seen during
these paroxysms. The paroxysms can cause
5 Respiratory complications are the most common, with pneumonia the major cause
fatigue, which may impair an infants ability
of mortality (0.51%) in infants <6 months.
to take feeds. Post-tussive vomiting com-
6 Treatment is with macrolide antibiotics (e.g. erythromycin for 2-week course), best monly follows the coughing episode, but
if given early in the course of the illness. between times the child may appear quite
well. It is important to note that young
7 Prevention is by universal immunisation with acellular pertussis vaccine in
infants may present with apnoea as the only
childhood, plus a booster in adolescence/adulthood.
symptom. This can result in a presentation
of sudden collapse (see Chapter 1.2).

Stage 3: Convalescent
(12 weeks)
Introduction Epidemiology The paroxysms of coughing, whooping and
vomiting decrease in number and severity.
Pertussis or whooping cough is a bacterial With the advent of universal vaccination
The cough may persist for a number of
infection of the respiratory tract caused programmes in the 1940s the incidence of
weeks/months and future episodes of upper
by the Gram-negative coccobacillus, this disease decreased markedly. Due to
respiratory tract infections may restimulate
Bordetella pertussis. The word pertussis concerns regarding immunisation, vacci-
the coughing paroxysms.
itself means intensive cough and was first nation levels waned in the late 1970s and
Pertussis is most severe in infants younger
described in 1578, when an epidemic there was a resurgence in the number of
than 6 months of age, particularly the non-
occurred in Paris.1 Pertussis is the preferred cases. Worldwide there are approximately
immunised. It is these high-risk infants that
term because not all cases have the classi- 250 000 deaths attributed to pertussis
particularly need to be identified and admit-
cal paroxysms of coughing, with an inspira- per year. There have been epidemics every
ted for a period of observation to exclude
tory whoop, which occur as a massive 34 years in Australia, with epidemiological
significant apnoea events. In infants, the
respiratory effort forces inhaled air against data showing an estimated incidence of 25
clinical manifestations can mimic those of
a narrow glottis. cases per 100 000 per year in 1996.2,3
bronchiolitis or other infective pneumonitis.
In partially immune adults and adolescents,
who have waning immunity, the presen-
Pathophysiology History tation is less typical and can be that of
a persistent cough.
Humans are the only reservoir and the The possibility of pertussis should be
incubation period is approximately 710 considered in a child presenting to the
days. Transmission is primarily by direct emergency department (ED) who has prom-
Examination
contact with droplets via the airborne inent coughing episodes. The diagnosis of
route. The organism is highly communicable pertussis is usually made on the basis of a The clinical findings depend on the stage of
early in the illness, with attack rates of suggestive clinical history, confirmed by the illness when the child presents and one
75100% from symptomatic individuals to isolation of the organism. needs to look for potential complications.
susceptible contacts. Pertussis is associated There are three characteristic stages of Some children will kindly display a typical
with a significant morbidity and mortality, the illness and presentation is most com- coughing paroxysm in the ED and reinforce
particularly in young infants. monly in the paroxysmal phase. the description given by parents. This may

134
6.4 PERTUSSIS
6

RESPIRATORY
include features of facial colour changes or a Occasionally, the pneumonia can be a severe have not received their five DTPa vaccina-
whoop that will suggest pertussis as the necrotising form, which is the major cause tions. There is no benefit from passive
likely cause. of death. Atelectasis is common, resulting immunisation prophylaxis with human
Other children may have little to find from mucous plugging and air leaks (pneu- pertussis immunoglobulin.3
on examination, apart from mild coryza. In mothoraces, interstitial emphysema or
these, the diagnosis is based on a suggestive pneumomediastinum) may occur secondary Prevention
history and subsequent isolation of the to ruptured alveoli. Bronchiectasis is a rare Immunisation first commenced in the late
organism. There may be visible mechanical late sequela. 1940s with the triple vaccine, Diphtheria
sequelae, due to coughing and vomiting, Subconjunctival haemorrhages, rectal Tetanus Pertussis (DTP). This was initially
such as petechiae or subconjunctival haemor- prolapse or inguinal hernia may occur due a whole-cell vaccine but since the 1990s
rhages. Unless a secondary pneumonia or to increased intra-abdominal pressure. an acellular vaccine has been available. It
atelectasis due to plugging has occurred, the Cerebral anoxia with convulsions can occur has been shown to be as effective, prevent-
chest examination is generally unremarkable. in young children and encephalopathy is ing disease in 85% of cases with fewer side
seen in approximately 1 in 10 000 cases.3,5 effects, and is now recommended.

Investigations
Current immunisation schedule
The clinical diagnosis is traditionally con- Treatment DTPa: 2, 4, 6, and 4 years.
firmed by nasopharyngeal swab, with culture There are also multiple-combination
All infants <6 months of age with suspected
of the organism being the gold standard. vaccines available: e.g. DTPa-hepB. An
pertussis should be admitted with isolation,
The isolation rates for pertussis are best early adult-formulation pertussis-containing vac-
due to the risk of apnoea and other compli-
in the course of the illness, prior to any anti- cine (dTpa) suitable for boosting adults and
cations. This allows for a period of observa-
biotic treatment. More recent investigations adolescents is recommended as a single dose
tion of coughing episodes and monitoring
have focused on more rapid techniques, at 15 to 17 years of age.3 Complications of
for apnoea and desaturations. Older children
which may aid decision making in the ED. the vaccine may potentially present to ED
will require admission should they have
These include fluorescent antibody tests on and include: fever >38 C (uncommonly leads
significant apnoea/cyanosis or feeding
post-nasal aspirate specimens, serology test- to a febrile convulsion), erythema at the
problems. Treatment is mainly supportive
ing to anti-B. pertussis IgA, IgM or IgG, and injection site, persistent crying, drowsiness,
via monitoring and the provision of oxygen
polymerase chain reaction (PCR).4 vomiting, anorexia, systemic allergic reaction,
and fluids.
The full blood examination, at the end and hypotonichyporesponsive episodes
Treatment with erythromycin is most
of the catarrhal or early in the paroxysmal (rare, with no long-term sequelae).
effective if given early and has little effect
phase, can have a leucocytosis with a pre-
in the paroxysmal phase. Recent studies
dominant lymphocytosis.
have looked at the equivalence of the newer
A chest X-ray may show perihilar infil-
macrolides and a 5-day course of azithromy- Prognosis
trates, interstitial emphysema or evidence
cin or 7 days of clarithromycin may be
of a secondary pneumonia. The severity is directly related to age,
as effective at eradicating the organism.6
with the illness normally milder in older
Co-trimoxazole is the drug of choice if
children. Morbidity and mortality (0.51%)
Differential diagnosis macrolides are contraindicated. There is
is still significant in infants under 6 months
minimal chance of transmission after 5 days
of age.
A pertussis-like syndrome may be caused by of treatment. Consider broader-spectrum
other organisms, including: Bordetella paraper- antibiotics if there is evidence of superadded
tussis, adenoviral infections, Mycoplasma bacterial infection. The index case should Controversies and future
pneumoniae and Chlamydia pneumoniae. be excluded from nursery/school until 5 directions
They often lead to a milder, shorter clinical days of treatment is completed.6,7
course and can be isolated by a nasopharyn- An encephalopathy has been
geal sample, or specific serology can be per- controversially linked with the pertussis
Chemoprophylaxis of contacts
formed. In the first few months of life, viral vaccination in the past, but the evidence
Contact tracing is difficult, but the family
bronchiolitis may cause a very similar clinical is inconclusive, as the event is so rare and
and close household contacts should have
picture to that of pertussis and only be distin- no causal link has been proven.8
a course of antibiotics regardless of immuni-
guished on the basis of the microbiology result. Pertussis vaccine does not cause infantile
sation status. There is poor placental trans-
spasms, epilepsy or sudden infant death
fer of B. pertussis antibodies, so pregnant
syndrome (SIDS).
women exposed around the time of delivery
Complications
and their newborn infants should also The drop in vaccination rates in the
Respiratory complications are frequent, receive chemoprophylaxis. The contacts 1970s and the subsequent increase in
with pneumonia the most common. It may should also have vaccination at the same the incidence of this preventable disease
be due to a secondary bacterial infection. time, particularly those under 8 years who highlight the need for ongoing

135
6.5 COMMUNITY-ACQUIRED PNEUMONIA

7. Aoyama T, Sunakawa K, Iwata S, et al. Efficacy of short-

surveillance to monitor the uptake and References term treatment of pertussis with clarithromycin and
azithromycin. J Paediatr 1996;129(5):7614.
efficacy of vaccinations, the frequency of 1. Cone TR. Whooping cough is first described as a disease sui 8. Heininger U. Pertussis: An old disease that is still with us.
adverse reactions and the incidence of generis by Balliou in 1640. Paediatrics 1970;46:522. .
Curr Opin Infect Dis 2001;14(3):32935.
2. Andrews R, Herceg A, Roberts C. Pertussis notifications in 9. Girard DZ. Which strategy for pertussis vaccination today?
epidemics. Further refinement and Australia 1991 to 1997. Commun Dis Intell 1997;21 Paediatr Drugs 2002;4(5):299313.
greater availability of the rapid (11):1458.
3. National Health and Medical Research Council. The
diagnostic techniques will be helpful in Australian Immunisation Handbook 9th edn. Canberra:
making the diagnosis quickly and Australian Government Publishing Service; 2008.
reliably. Progress in the sequencing 4. Heininger U, Schmidt-Schapfer G, Cherry J, Stehr K. Clinical Further reading
validation of a polymerase chain reaction assay for the
of the genome of B. pertussis has also diagnosis of pertussis by comparison with serology, culture Cherry JD, Heininger U. Pertussis and other Bordetella
and symptoms during a large pertussis vaccine trial. infections. In: Feigin RD, Cherry JD, editors. Textbook of
yielded a new field of research.9 Paediatrics 2000;104:312. Pediatric Infectious Diseases. 4th ed., vol. 2. Philadelphia,
There is also a need for more studies 5. Miller D, Madge N, Diamond J. Pertussis immunisation PA: WB Saunders Co; 2004. p. 1588608.
and serious acute neurological illnesses in children. Br
on equivalence testing of Med J 1993;307(6913):11715.
different antibiotics for treatment of 6. Lebel MH, Mehra S. Efficacy and safety of clarithromycin
versus erythromycin for the treatment of pertussis:
pertussis. A prospective, randomized, single blind trial. Pediatr Infect
Dis J 2001;20:114954.

6.5 Community-acquired pneumonia

Mike Starr

investigated with a range of tests, the useful-

ESSENTIALS ness of which is known to be incomplete,
and it is then often treated without knowl-
1 Viruses are the most common cause of pneumonia in children, although up to 40% edge of the aetiology. Fortunately, most
of cases represent mixed infection.
children recover completely with empiric
2 Streptococcus pneumoniae is the most common bacterial cause. treatment.

3 X-ray findings do not correlate with aetiology.

4 Blood tests, including full blood count, inflammatory markers and blood culture are Definition
generally unhelpful. There are various definitions that can be
5 Oral amoxicillin or intravenous benzylpenicillin are appropriate for empiric therapy used for pneumonia. From a pathological
of most children with pneumonia. point of view, it is defined as inflammation
or infection of the lung parenchyma.
6 The addition of a macrolide antibiotic (e.g. roxithromycin) should be considered in In the clinical setting, the diagnosis is typi-
children with atypical pneumonia. cally made on the basis of a constellation
7 The routine use of third-generation cephalosporins provides no additional benefit of clinical features, including fever, cough,
over the penicillins. tachypnoea and auscultatory findings, and
confirmed by radiographic changes.

morbidity and one of the most common rea-

Introduction sons for paediatric hospital admissions.2 The
Aetiology
Pneumonia is a common condition with sig- incidence of pneumonia is approximately 40 In the majority of cases of childhood
nificant morbidity and mortality. It is esti- per 1000 children per year in those under pneumonia, the causative pathogen is not
mated to be responsible for approximately 5 years of age, and 15 per 1000 children identified. Blood cultures are positive in
three million childhood deaths per year, per year in 514-year-olds.35 under 5% of cases of pneumonia.5,6
most of which occur in developing In spite of how common it is, there is no Transthoracic lung aspiration yields a cause
countries.1 Even in developed countries, single clinical or radiological definition in up to 69% of cases,7,8 but is invasive.
pneumonia remains a major cause of acute that is widely accepted for pneumonia. It is It is difficult to obtain adequate sputum for

136
6.5 COMMUNITY-ACQUIRED PNEUMONIA
6

RESPIRATORY
microscopy and culture in young children. most common bacterial cause, with or present. Lateral X-rays do not confer much
Other indirect methods of identifying a without effusion. additional information in most cases.
cause, such as serology or immunofluores- Although, it is not possible to reliably
cence and culture of nasopharyngeal aspi- predict aetiology or differentiate bacterial
rates are neither sensitive nor specific. from viral pneumonia on chest X-ray, pneu-
Although an alveolar or lobar infiltrate on Clinical findings mococcal pneumonia typically presents
chest X-ray is considered by some to be sug- with a lobar infiltrate or round pneumonia.
Pneumonia should be considered in any
gestive of bacterial infection, chest X-ray Pneumatoceles, abscesses and cavities are
infant or child presenting with fever, cough,
changes cannot reliably predict aetiology.9,10 associated most frequently with staphylo-
difficulty breathing, tachypnoea, increased
Nor is any radiological pattern pathogno- coccal pneumonia but they are also seen
work of breathing (nasal flaring, lower chest
monic for viral or Mycoplasma pneumoniae in pneumonias caused by other bacteria.
indrawing or recession), and auscultatory
infection. Bronchopneumonia is more typical of viral
findings consistent with consolidation or
Age is the best predictor of aetiology of or other aetiology.
effusion. However, the auscultatory findings
pneumonia. In neonates, where bacterial Large effusions may be difficult to
may be unreliable in young children, partic-
causes predominate, Group B streptococci differentiate from empyema. In some cases,
ularly in those under 1 year. Infants may
and Escherichia coli are the most common ultrasound may be helpful in determining
present with symptoms not obviously
pathogens. Viruses, particularly respiratory whether loculations are present.
related to a lower respiratory tract infection,
syncytial virus (RSV), parainfluenza, influ- Radiological changes lag behind clinical
such as lethargy, vomiting, poor feeding,
enza, metapneumovirus and adenovirus, signs, and may persist for 46 weeks.
grunting, or poor perfusion. Children less
are the most common cause overall, particu- Follow-up chest X-rays are unnecessary in
than 3 months of age may present with
larly in young children. The occurrence of most cases of uncomplicated pneumonia,
apnoea. Tachypnoea is the most sensitive
recent local outbreaks and the clinical pat- but should be considered if symptoms and
and specific sign12 and may be the only clue
tern may give a clue to the likely causative signs are persistent following treatment.
in some children. Auscultatory findings,
virus. These viruses appear to be responsible Most other investigations to determine
including asymmetrical breath sounds,
for approximately 40% of cases of community- microbiological aetiology are not particu-
crackles and bronchial breathing are less
acquired pneumonia in children who are larly helpful in the emergency department
predictive. The presence of coryza or wheeze
hospitalised, particularly in those under 2 years setting to dictate immediate medical man-
(particularly bilateral) suggests that bacte-
of age, whereas Streptococcus pneumoniae agement. Rapid viral antigen tests, such as
rial pneumonia is unlikely.6,13 Pneumonia
is responsible for 27% to 44% of cases of direct immunofluorescence assay for RSV
located in the apex of the lung may present
community-acquired pneumonia.11 Up to and other respiratory viruses on nasopharyn-
with neck pain and be confused with menin-
40% of infections are mixed.5 Infection with geal aspirates, do not usually alter manage-
gitis, whilst basal pneumonia may cause
Mycoplasma pneumoniae and Chlamydia ment. However, they may inform infection
abdominal pain, suggestive of an acute
pneumoniae is usually considered to cause control strategies for young children admit-
abdomen. Conversely, an infant with grunt-
pneumonia in children of school age and in ted to hospital. Many respiratory pathogens
ing respirations may give the initial impres-
older patients, although more recent studies may be identified using molecular methods,
sion of an intra-abdominal problem.
suggest that preschool-aged children have such as polymerase chain reaction (PCR)
The presentation of pneumococcal pneu-
as many episodes of atypical bacterial analysis of respiratory secretions.
monia is generally abrupt, whilst M. pneu-
pneumonia as older children.11 Staphylococcal
moniae and viral infections more often
and Group A streptococcal pneumonia are
present with a more indolent course, less
uncommon, but should be considered in chil-
dren who are severely unwell with invasive dis-
fever, and other symptoms such as malaise, Management
headache, arthralgia and rash.
ease. These infections are more likely to be Patients should be stabilised as necessary
seen in indigenous and Pacific Islander with high-flow oxygen and fluid resuscita-
children. More recently, infections with com- tion. Many children will not require any spe-
munity-acquired multiresistant Staphylococcus cific treatment. Most children who are not
aureus (CAMRSA) have emerged. CAMRSA
Investigations too unwell and have lobar consolidation
results in a necrotising fulminant pneumonia Posteroanterior chest X-rays are generally on chest X-ray can be managed as outpati-
with increased morbidity and mortality. used to confirm pneumonia where there is ents with oral amoxicillin 25 mg kg 1 (up
Gram-negative pneumonia is uncommon clinical suspicion. However, they need not to 1 g) three-times daily for 7 days. Children
in children; non-typeable Haemophilus be performed routinely in older children with under 1 year of age, and those who are more
influenzae is mainly seen in children with mild disease where the diagnosis may be unwell or hypoxic may require inpatient man-
underlying lung disease, such as cystic made clinically. They are particularly impor- agement (Table 6.5.1) and treatment with
fibrosis and bronchiectasis. tant for confirming the diagnosis of pneu- intravenous benzylpenicillin 50 mg kg1 (up
The presence of a pleural effusion monia in children less than 5 years of age to 1.2 g) 6-hourly. Infants less than 3 months
does not necessarily indicate more severe who present with fever and tachypnoea, of age should also be given intravenous gen-
disease Strep. pneumoniae remains the unless classical features of bronchiolitis are tamicin 7.5 mg kg1 daily.

137
6.5 COMMUNITY-ACQUIRED PNEUMONIA

settings or populations where CAMRSA is 2. Rudan I, Boschi-Pinto C, Biloglav Z, et al. Epidemiology

Table 6.5.1 Possible indications for and etiology of childhood pneumonia. Bull World Health
admission more prevalent, vancomycin 1015 mg kg1 Organ 2008;86(5):40816.
per dose 6-hourly should be added. 3. Jokinen C, Heiskanen L, Juvonen H, et al. Incidence of
Hypoxia, apnoea community-acquired pneumonia in the population of
A short course (3 days) of antibiotic ther- four municipalities in eastern Finland. Am J Epidemiol
Toxic appearance, poor feeding, dehydration apy is as effective as a longer treatment 1993;137(9):97788.
4. Murphy TF, Henderson FW, Clyde WA, et al. Pneumonia:
Age <1 year (5 days) for non-severe pneumonia in An eleven-year study in a paediatric practice. Am J
children under 5 years of age.16 Epidemiol 1981;113(1):1221.
Underlying lung disease or immunodeficiency 5. Juven T, Mertsola J, Waris M, et al. Etiology of community-
acquired pneumonia in 254 hospitalized children. Pediatr
Extensive consolidation Infect Dis J 2000;19(4):2938.
6. Durbin WJ, Stille C. Pneumonia. Pediatr Rev 2008;
Failed response to oral therapy 29(5):14758.
Complications 7. Vuori-Holopainen E, Peltola H. Reappraisal of lung tap:
Review of an old method for better etiologic diagnosis
A child who remains very unwell and febrile of childhood pneumonia. Clin Infect Dis 2001;
after 48 hours of parenteral treatment 32(5):71526.
8. Vuori-Holopainen E, Salo E, Saxen H, et al. Etiological
Antibiotic resistance among pneumococci should be reassessed for the possibility of diagnosis of childhood pneumonia by use of
is becoming more common. However, there empyema or, more rarely, lung abscess. In transthoracic needle aspiration and modern
microbiological methods. Clin Infect Dis 2002;34
is no difference in outcome between cases these cases, input from infectious diseases (5):58390.
caused by susceptible and resistant strains, and respiratory specialists is advised. 9. Bettenay FA, de Campo JF, McCrossin DB. Differentiating
bacterial from viral pneumonias in children. Pediatr
and amoxicillin or benzylpenicillin remains Radiol 1988;18(6):4534.
the treatment of choice.14,15 The use of 10. Swingler GH. Radiologic differentiation between
bacterial and viral lower respiratory infection in children:
third-generation cephalosporins provides A systematic literature review. Clin Pediatr 2000;
no additional benefit over these penicillins.
Prevention 39(11):62733.
11. Ranganathan SC, Sonnappa S. Pneumonia and other
They should be reserved for severe pneumo- The introduction of conjugate H. influenzae respiratory infections. Pediatr Clin North Am 2009;
nia where it may be important to cover beta- type b (Hib) and Strep. pneumoniae vaccines 56(1):13556.
12. Palafox M, Guiscafre H, Reyes H, et al. Diagnostic value of
lactamase producers and Gram-negative into the routine immunisation schedule in tachypnoea in pneumonia defined radiologically. Arch
bacteria. many countries has led to a reduction in Dis Child 2000;82(1):415.
13. British Thoracic Society. Guidelines for the management
Children presenting with coryza, wheeze, the burden of pneumonia caused by these of community acquired pneumonia in childhood 2002.
diffuse crackles and minimal chest X-ray two organisms.17 Thorax 2002;57(Suppl. 1):1124.
14. Friedland IR. Comparison of the response to antimicrobial
changes may have viral pneumonitis. Admis- therapy of penicillin-resistant and penicillin-susceptible
sion may be necessary for supportive care, pneumococcal disease. Pediatr Infect Dis J 1995;
14(10):88590.
but antibiotics should be withheld. A trial 15. Tan TQ, Mason Jr EO, Wald ER, et al. Clinical
of inhaled bronchodilator therapy may be Conclusion characteristics of children with complicated pneumonia
caused by Streptococcus pneumoniae. Paediatrics
useful in children who appear to have signif- Pneumonia is a common illness in children. 2002;110(1):16.
icant associated bronchospasm. A macrolide Viruses are the most common cause, and 16. Haider B, Saeed M, Bhutta Z. Short-course versus long-
course antibiotic therapy for non-severe community-
antibiotic,suchasoralroxithromycin4 mg kg1 many children will not require any specific acquired pneumonia in children aged 2 months to 59
(up to 150 mg) twice daily for 7 days, should treatment. Strep. pneumoniae remains the months. Cochrane Database Syst Rev 2008;16(2):
CD005976.
be considered for those with suspected most common bacterial cause, and amoxicillin 17. Theodoratou E, Johnson S, Jhass A, et al. The effect of
M. pneumoniae infection. remains the antibiotic treatment of choice.18 Haemophilus influenzae type b and pneumococcal
conjugate vaccines on childhood pneumonia incidence,
Only children who are severely unwell severe morbidity and mortality. Int J Epidemiol 2010;
require broader-spectrum antibiotics to 39(Suppl. 1):i172185.
18. Kabra SK, Lodha R, Pandey RM. Antibiotics for
include cover for Staph. aureus and Gram- community-acquired pneumonia in children. Cochrane
negative bacteria: flucloxacillin 50 mg kg1 References Database Syst Rev 2010;17(3) CD004874.
1. Campbell H. Acute respiratory infection: A global
intravenously (IV) (up to 2 g) 6-hourly challenge. Arch Dis Child 1995;73(4):2813
plus cefotaxime 50 mg kg1 IV 6-hourly. In [Review: 38 refs].

138
 6

RESPIRATORY
6.6 Bronchiolitis
Tom Beattie

oedema and muscle spasm, giving the typi-

ESSENTIALS cal clinical picture of bronchiolitis.
How this clinical picture emerges is still
1 The diagnosis of bronchiolitis is clinical, based on history and examination. unclear. The role of pro-inflammatory regu-
2 Bronchiolitis typically affects children under the age of 12 months, but may occur lators interleukin (IL)-6, IL-8, interferon-g,
in children up to 2 years of age. and macrophage inflammatory protein-1b,
as well as of the regulatory cytokine IL-10
3 As feeding by an infant is an important index of bronchiolitis severity, a careful in causing the disease as we know it, as
history of a change in feeds is paramount.
opposed to facilitating healing and repair
4 High-risk patients include those with underlying chronic lung disease, congenital still remains to be elucidated.24
heart disease or corrected age less than 2 months of age. The incidence of concomitant or second-
ary bacterial infection is low, although otitis
5 In the very young infant, apnoea may be the predominant symptom with few media may occur. In the very young infant,
respiratory signs. Likewise, in neonates a non-specific sepsis-like picture with collapse
apnoea may be the predominant symptom,
may occur.
with few other respiratory signs. Likewise,
6 Oxygen saturations fall with disease severity and SaO2 levels below 94% indicate in neonates a non-specific sepsis-like picture
a need for admission. with collapse may occur. In reinfection with
RSV or primary infection in older children,
7 The role of chest radiography is limited and only indicated if the diagnosis is the symptoms are more limited to the upper
unclear or in severe cases.
respiratory tract, causing signs consistent
8 In patients being admitted, perform a rapid NPA or PNA to analyse for RSV or with a severe common cold.
other respiratory viruses for isolation control.

9 Supportive care is the mainstay of treatment of bronchiolitis.

10 The decision to admit to hospital is based mainly on considerations of the childs Clinical assessment
need for oxygen, fluids or cardiorespiratory monitoring for apnoea. History
Bronchiolitis typically presents with a pro-
drome of upper respiratory tract infection
over 12 days.
young children. It is estimated that by the
Introduction When the lower respiratory tract becomes
age of 2, 70% of children have been
involved, the hypersecretion of mucus
Epidemiology exposed to RSV. Despite the frequency, mor-
causes the moist cough, onset of respiratory
Bronchiolitis is a common presentation to tality is low at less than 1% of hospitalised
distress and resultant feeding difficulties. As
emergency departments, with a seasonal babies. High-risk patients include those with
ability to feed in an infant is an important
pattern. It typically affects children under underlying chronic lung disease, congenital
index of bronchiolitis severity, a careful
the age of 12 months, but may occur in chil- heart disease, neuromuscular disorders or
history of a change in feeds is paramount.
dren up to 2 years of age. The peak age is corrected age less than 2 months of age.1
between 2 and 8 months of age, with males
more commonly affected. Approximately Examination
1% of children will require admission for Pathophysiology Examination of the child will reveal a combi-
bronchiolitis, which is the leading cause of Infection with RSV is associated with direct nation of signs of upper respiratory tract
admission for children with lower respiratory invasion of epithelial cells in the respiratory infection (URTI), along with signs indicative
tract disease in the Western world.1 Epi- tract. Primary infection in young children of lower respiratory tract infection (LRTI),
demics of bronchiolitis occur during each and infants involves the lower respiratory which may fluctuate between examinations.
winter, with the peaking of respiratory tract. The bronchiolar epithelium is predom- The fever is usually low grade. A moist cough
viruses. While respiratory syncytial virus inantly affected and an inflammatory is common and wheeze may be audible at
(RSV) is the commonest organism responsi- response follows. Lymphocytes infiltrate the bedside.
ble for bronchiolitis, others include parain- the affected areas and oedema develops in Tachypnoea and tachycardia are usually
fluenza virus, adenovirus, rhinovirus and the submucosa. Smooth muscle spasm in proportion to the illness severity. Infants
influenza. Bronchiolitis may also complicate ensues. The net result is that small airways who are estimated to be feeding less than
exanthems such as measles and varicella in become narrowed by the combination of 50% of normal feeds usually have oxygen

139
6.6 BRONCHIOLITIS

saturations less than 94%. Cyanosis is seen

Table 6.6.1 Bronchiolitis severity and management
in children with severe disease.
Chest examination may reveal hyperinfla- Severity Signs Management
tion and recessions of the chest wall due to Mild Alert Discharge home
increased work of breathing. Paradoxically, Feeding >50% normal
Mild respiratory distress Smaller/frequent feeds
as an infant fatigues, the recessions will SaO2 94% Review in primary care
decrease. In this situation the diminishing NOT high-risk patient
Age >6 weeks
air entry signifies progressive disease.
Auscultation reveals wheezes that are gen- Moderate Lethargic, tired Admit
Feeding <50% normal O2 to SaO2 >94%
erally symmetrical. There may be inspiratory Marked respiratory distress Minimise handling
crepitations. The auscultation findings are Dehydrated Consider NG or IV fluids
SaO2 <94% Close observation
dynamic as coughing will move secretions High-risk patient
to more proximal airways, with resultant
Severe As above but with: Cardiorespiratory monitor
temporary clearing of the wheeze. A short Increasing O2 requirement Consider ABG
time later, as the fluid returns to the more Fatigue Liaise with PICU
Signs of CO2 retention
peripheral airways, the wheeze returns. Apnoeic episode
Hence, babies referred by a local doctor with
ABG, arterial blood gas; NG, nasogastric; PICU, paediatric intensive care unit.
marked wheeze may initially appear to be
wheeze free when seen in the emergency
department (ED) a short time later. Re-
examination later will confirm the presence
have been developed to determine the sever-
of wheeze.5 Table 6.6.2 Differential diagnosis
ity of the disease. Table 6.6.1 shows the of bronchiolitis
Oxygen saturations fall with disease
criteria used to help determine severity and
severity and SaO2 levels below 94% indicate Asthma
management issues.
a need for admission.4 McIntosh graded Cardiac failure
severity of bronchiolitis by simply document-
Pneumonia
ing children as needing no oxygen, requiring Differential diagnosis
oxygen and needing ventilation.6 Certainly, The differential diagnosis of bronchiolitis Neonatal sepsis presenting as collapse
increasing oxygen requirements will be asso- includes cardiac failure, asthma and Happy wheezer persistent wheezing in
ciated with increasing severity of disease. pneumonia (Table 6.6.2). Cardiac failure undistressed baby
can present with many of the features of
bronchiolitis dyspnoea, tachypnoea,
Assessment tachycardia, crepitations and palpable liver.
In helping to assess an infant with bronchi- Feeding may also be poor. However, infants indicated if the diagnosis is unclear or in
olitis and the likely subsequent course, one with bronchiolitis will usually have the URTI severe cases. The typical radiological find-
needs to determine the onset of the respira- prodrome and the onset of poor feeding is ings are hyperinflation of the lung fields,
tory distress or poor feeding phase of the ill- acute. The feeding difficulty in children with bilateral increase in interstitial mark-
ness. Most children have increasing work of with cardiac failure is less acute, leading to ings (particularly perihilar regions). There
breathing for 4872 hours due to increasing poor weight gains. Additional signs, such may be patchy atelectasis secondary to
secretions, before a plateau phase followed as a gallop rhythm or murmur, suggest an plugging or, in severe cases, collapse. Chil-
by resolution over 37 days. underlying cardiac abnormality. Recurrent dren with high fever, or a clinical impression
The cough may persist for a further 710 episodes of wheeze associated with URTIs, of sepsis, may have superimposed bacterial
days after resolution of the respiratory dis- particularly in older infants, can be difficult infection, and a chest X-ray will aid exclusion
tress. In this way one can determine if a child to differentiate from asthma. Pneumonia of this diagnosis.7
is likely to deteriorate further, is probably in infants can mimic bronchiolitis and the Nosocomial infection and cross-infection
stable at the peak of severity or improving differentiation can be difficult. Some infants can occur during bronchiolitis outbreaks. In
at the time of the ED visit. It is the tiring con- have persistent wheezing, which does not patients being admitted to hospital, a naso-
sequence of the tachypnoea of bronchiolitis compromise activity or feeds and is unre- pharyngeal aspirate (NPA) or posterior nasal
that impairs feeding ability, which is the sponsive to inhaled bronchodilators (fat aspirate (PNA) can be done to analyse for
important determinant of whether a child happy wheezer). RSV or other respiratory viruses for isolation
warrants interventions such as oxygen or control. The development of near patient
intravenous fluids. Investigations testing (NPT) kits for rapid diagnosis of
Assessment of the child with bronchiolitis The diagnosis of bronchiolitis is clinical, RSV infection has aided infection control
requires several components to be consid- based on history and examination. The role in some areas.8 The rapid testing for RSV
ered. Several scoring systems for bronchiolitis of chest radiography is limited and only may be useful in cases of neonates to help

140
6.6 BRONCHIOLITIS
6

RESPIRATORY
with decision making where a child presents oxygen may be necessary. In some infants sustained timeframe. Isaacs suggested that
with fever, collapse or apnoea. who have self-limiting apnoeic episodes, con- if wheeze is the predominant sign then a
Other tests such as full blood examination tinuous positive airway pressure may help buy trial of selective b agonist may help.1
are generally not useful in diagnosis. Severe time until the disease ameliorates. However, Systemic or inhaled glucocorticoids are
cases may demonstrate CO2 retention on any persistence of apnoea or failure to main- widely used in some parts of the world,
arterial blood gases or electrolyte disturbance. tain oxygenation would warrant consider- but the evidence for their use is variable.
ation of ventilation using endotracheal A recent meta-analysis has shown some
intubation and admission to an Intensive marginal benefit with systemic glucocorti-
Care setting. Clearly, the choice of treatment coid use.13 The role of ipratropium bromide
Treatment will depend on available expertise and is equally unclear and there is no clear-cut
Supportive care is the mainstay of treatment equipment. In all situations an increase in benefit to use.14
of bronchiolitis. The decision to admit to O2 requirement to maintain saturations Nebulised adrenaline (epinephrine) and
hospital is based mainly on considerations indicates increasing severity and the need to dexamethasone in combination have been
of the childs need for oxygen, fluids or escalate therapy accordingly. shown in a multicentre trial in Canada to
cardiorespiratory monitoring for apnoea. Feeding can be continued orally initially have some potential beneficial effect on
Treatment options depend primarily on the in those with moderate bronchiolitis, with the severity of the illness as determined by
severity of the disease. small frequent feeds as above. If the child hospital admission.15
Mild cases require an explanation to par- cannot tolerate this, nasogastric feeds or Ribavarin and antiviral immunoglobulins
ents and advice regarding feeding to ensure intravenous fluids should be considered. are not used in the ED setting, but may have
adequate hydration. Smaller volume feeds Infants who do not have markedly increased a role in high-risk groups in intensive
offered more frequently to the child will usu- work of breathing may come out of oxygen care. Ribavirin is expensive and has only
ally ensure adequate hydration. Hence, the for feeds if tolerated. marginal benefit when given aerolysed to
child who feeds 34 hourly normally, should There is no indication for antibiotic use for high-risk patients with severe disease.16
be offered feeds every 1.52 hours. Parents either mild or moderate bronchiolitis unless The role of immunisation against RSV is still
should monitor urine output, and should be there is good evidence of secondary bacte- under investigation.
advised to seek review should feeding dete- rial infection.
riorate or urine output fall significantly. Children with severe disease need to be
Some parents become exhausted by the con- admitted to a facility where they can be con- Prognosis
stant demands of infants with bronchiolitis, tinuously monitored by appropriate staff for
The vast majority of children with bronchiolitis
and an assessment of parental coping ventilation should they deteriorate or have
will recover over 710 days. The cough may
should form part of the clinical picture. If significant apnoea. Features of concern
persist for some weeks after the resolution
there are concerns about parental ability include the infant who is tiring, has escalating
of the respiratory distress. A small number
to provide the increased demands of feed- oxygen requirement or develops repeated
may continue to wheeze and cough for several
ing, admission should be considered. and/or prolonged apnoeic episodes. Early dis-
months and an indeterminate number will
Children with moderate bronchiolitis need cussion with the local paediatric intensive care
develop persistent wheeze and/or asthma.
to be admitted. The infant will need to be unit staff will help to determine this need and
The exact relationship between these events
monitored clinically, with regular observation organise appropriate transfer mechanism.
is unclear. Further study is needed to elucidate
of heart rate, respiratory rate, ability to Fluids should be given intravenously and
the role of RSV in the development of
feed and level of fatigue. Oxygen saturation adjusted according to volume status, urine
subsequent reactive airway disease.
should be monitored and supplemental oxy- output and electrolyte results. Children with
gen should be provided to maintain SaO2 underlying disease such as heart disease
above 95% in those with saturations below may need additional specific therapy.
94% or unable to feed. Oxygen may be
Prevention
administered by head box, nasal prong or Drug therapy The transmission of RSV occurs with contact
cannula, isolette, mask or tent. The choice The role of various drug therapies in bron- with infected secretions. In the ED setting,
of method of oxygen delivery needs to be chiolitis is controversial and still undergoing attention to hand washing, stethoscope
tailored to the individual case. In small research. hygiene and cubicle use is important to
infants who are obligatory nose breathers, Two meta-analyses of the benefits of prevent cross-infection between children.
nasal prongs or cannulae may exacerbate inhaled b agonists in bronchiolitis have
the nasal obstruction in babies with copious proved inconclusive. A more recent Cochrane
secretions. Under 6 months of age a head Review suggests some minimal benefit, Controversies
box is usually the best, using an oxygen ana- while Patel has recently shown no benefit Treatment of bronchiolitis has changed
lyser to monitor the O2 requirement. Older with albuterol (salbutamol).912 Nebulised little over the years, with the mainstay being
infants may tolerate an oxygen mask, but if adrenaline (epinephrine) has similarly not supportive care. A considerable amount of
distressed by this then using cot or tent been shown to provide benefit over a research needs to be done on the

141
6.7 CROUP

4. Bennett BL, Garafolo RP, Cron SG, et al. 11. Kellner JD, Ohlsson A, Gadomski AM, Wang EE.
classification of the severity of disease based Immunopathogenesis of respiratory syncytial virus Bronchodilators for bronchiolitis. Cochrane Database Syst
on sound, objective data. Without this, the bronchiolitis. J Infect Dis 2007;195(10):153240. Rev 2000;2: CD001266.
5. Mulholland EK, Olinsky A, Shann FA. Clinical findings and 12. Patel H, Platt RW, Pekeles GS, Ducharme FM. A
debate on treatment with b agonists, severity of acute bronchiolitis. Lancet 1990;335 randomized, controlled trial of the effectiveness
glucocorticoids, anticholinergic drugs and (8700):125961. of nebulized therapy with epinephrine compared
other therapies will persist, with anecdote 6. McIntosh ED, De Silva LM, Oates RK. Clinical severity of with albuterol and saline in infants hospitalized
respiratory syncytial virus group A and B infection in for acute viral bronchiolitis. J Pediatr 2002;141
continuing to supplant scientific data. Sydney, Australia. Pediatr Infect Dis J 1993;12(10): (6):81824.
8159. 13. Garrison MM, Christakis DA, Harvey E, et al. Systemic
7. El-Radhi AS, Barry W, Patel S. Association of fever and corticosteroids in infant bronchiolitis: A metaanalysis.
severe clinical course in bronchiolitis. Arch Dis Child Pediatrics 2000;105(4):E44.
1999;81(3):2314. 14. Everard ML, Bara A, Kurian M, et al. Anticholinergic
8. Mackenzie A, Hallam N, Mitchell E, Beattie T. Near drugs for wheeze in children under the age of two
References patient testing for respiratory syncytial virus in paediatric years. Cochrane Database Syst Rev 2002;1:
1. Isaacs D. Bronchiolitis. Br Med J 1995;310(6971):45. accident and emergency: Prospective pilot study. Br Med J CD001279.
2. Bont L, Heijnen CJ, Kavelaars A, et al. Peripheral blood 1999;319(7205):28990. 15. Plint A, Johnson D, Patel H, et al. Epinephrine and
cytokine responses and disease severity in respiratory 9. Kellner JD, Ohlsson A, Gadomski AM, Wang EE. Efficacy dexamethasone in children with bronchiolitis. N Engl J
syncytial virus bronchiolitis. Eur Respir J 1999;14(1): of bronchodilator therapy in bronchiolitis. A meta- Med 2009;360(20):207989.
1449. analysis. Arch Pediatr Adolesc Med 1996;1150 16. Everard ML, Swarbrick A, Rigby AS, Milner AD. The
3. Smyth RL, Mobbs KJ, OHea U, et al. Respiratory syncytial (11):116672. effect of ribavirin to treat previously healthy infants
virus bronchiolitis: Disease severity, interleukin-8, 10. Flores G, Horwitz RI. Efficacy of b2-agonists in admitted with acute bronchiolitis on acute and
and virus genotype. Pediatr Pulmonol 2002;33(5): bronchiolitis: A reappraisal and meta-analysis. chronic respiratory morbidity. Respir Med 2001;95
33946. Paediatrics 1997;100(2 Pt 1):2339. (4):27580.

6.7 Croup
Gary Geelhoed

airway in small children. Croup is a common

ESSENTIALS childhood problem, with a peak incidence of
60 per 1000 child years in those aged
1 Croup is a common childhood problem with a peak incidence of 60 per 1000 child
between 1 and 2 years, although it may be
years in those aged between 1 and 2 years.
seen up to the teen years. As such, it is by
2 The loudness of stridor is variable and is not a reliable indicator of the severity of far the most common cause of acute upper air-
the airway obstruction. ways obstruction likely to present to emer-
gency departments.
3 All children who present to emergency departments with croup should be treated
The respiratory distress caused by obs-
with steroids.
truction tends to be most marked in younger
4 The steroids of choice are oral dexamethasone 0.15 mg kg1 or prednisolone children due to the small size of their larynx,
1 mg kg1. the presence of loose submucous tissues,
and the tight encirclement of the subglottic
5 A compromised but functioning airway should never be made worse by upsetting
area by the cricoid cartilage. In children
the child.
under 8 years of age, this is the narrowest
6 Children who require adrenaline (epinephrine) may be sent home safely if they region of the airway, hence any inflamma-
have also received steroids and have improved over a number of hours to have no tory swelling in this area results in a sig-
stridor at rest. nificant impingement of the airway. The
younger child, who has a smaller diameter
airway, requires an increased vigilance to
the epidemics of upper respiratory viruses. assess the degree of airway compromise.
Introduction Parainfluenza virus type 1 accounts for around The lower airway involvement of laryngo-
The term croup describes an acute clinical syn- half the cases during winter, with parain- tracheobronchitis may also cause younger
drome of hoarse voice, barking cough, and fluenza type 2, influenza type A, adenoviruses, children to manifest wheeze due to con-
inspiratory stridor usually seen in young chil- respiratory syncytial virus, enteroviruses, and current inflammation producing mucus in
dren. Croup results from swelling of the upper possibly Mycoplasma pneumoniae causing the smaller peripheral airways. Likewise,
airway, in and around the larynx, usually as most of the other cases. Some of the viral occasionally, older children known to have
a result of a viral infection. Croup occurs exanthems, such as varicella, can cause con- asthma may exhibit signs of asthma in the
seasonally, peaking in winter months due to comitant croup by involvement of the upper setting of croup.1

142
6.7 CROUP
6

RESPIRATORY
Examination The use of a croup severity score may be
Presentation
Croup in children can generally be classified helpful for less-experienced staff to assess chil-
History as mild, moderate, or severe (Table 6.7.1). dren with croup and communicate findings
The typical presentation of croup is in a pre- Most children with mild croup are not dis- with a colleague when seeking advice. As pre-
school-aged child with a history of a recent tressed and have only a barking cough with viously mentioned, some children may have
onset of upper respiratory tract infection. no stridor at rest or stridor audible only with concomitant wheeze in addition to the upper
The child subsequently develops a barking physical activity, crying, or agitation. Crying airway stridor and hence their pattern of brea-
or seal-like cough, a hoarse voice and, if causes physiological narrowing of the airway thing may be gas trapped and cause hyperin-
obstruction is severe enough, stridor. The stri- and will increase the respiratory distress. flation of the chest with slow expiration phase,
dor may initially be apparent only when a child Hence the distressed, crying childs obstruc- in contrast to those with pure croup alone.
is distressed, such as during crying. During cry- tion will often defervesce by allowing the child
ing or forced expiration the diameter of the to be cuddled in the parents arms. There may
upper airways physiologically narrows and, be signs due to viral illness, such as mild fever Investigations
hence, stridor will manifest. Stridor, which is and nasal discharge. Children with mild cases
Croup is usually an easy spot diagnosis and
initially inspiratory, indicates obstruction at can have their throats examined, but this
requires no diagnostic tests.
the laryngeal level or higher (i.e. upper air- should be deferred in more severe cases. A
Oximetry is of limited value, as children may
way). Expiratory stridor or biphasic stridor compromised but functioning airway should
maintain near-normal oxygen saturations
indicates more severe laryngeal obstruction never be made worse by upsetting the child.
even when they have significant airway
or alternatively an obstruction occurring lower In more severe cases, the child may have a
obstruction.3 While it may have a role in cases
in the airway. The natural history of airway more pronounced stridor at rest. As airway
of severe croup, this must be balanced with
obstruction, when unmodified by steroids, is obstruction progresses, increased work of
the distress caused by the monitoring probe
to increase slowly to peak over 2448 hours. breathing ensues and the child exhibits
in small children. In stable cases, where
The airway compromise usually then resolves increasing substernal, intercostal, and subcos-
the diagnosis is unclear, a lateral soft tissue
over a few days, although the laryngeal cough tal retractions. Subtle signs of hypoxia causing
X-ray of the neck may be helpful to distinguish
may persist longer. altered consciousness may be reflected as anx-
croup from epiglottitis or retropharyngeal
Less common than infectious croup but iety or restlessness in a child. An obviously
abscess. However, the possible benefits of an
usually more sudden in onset, older children fatiguing child is a worrying sign. The child
X-ray need to be weighed against the risks
may present with recurrent or spasmodic manifesting decreased air entry and respira-
of moving or disturbing the child when the
croup with no viral prodrome. This is thought tory effort, extreme pallor, and cyanosis
obstruction is more than mild. A nasopharyn-
to be allergic in origin. These children may requires immediate intervention.
geal aspirate in croup is not necessary for diag-
have a history of atopy and suffer from The childs preferred position may also
nosis but may be useful for infection control
asthma more than the general population. give clues as to severity of obstruction or
for patients being admitted.
They should, however, be treated in the same to a diagnosis other than croup. Hyperexten-
manner as viral croup. In the smaller child, sion or other abnormal positioning of the
particularly infants, problems with feeding, neck may suggest epiglottitis or a retrophar-
Differential diagnosis
swallowing difficulties, and whether the child yngeal abscess. It is unusual for the child
has been cyanosed should be ascertained. with croup to be saliva intolerant or have It is important to establish that other, more
It is important to enquire whether or not the any tenderness of the neck. sinister, causes of acute upper respiratory tract
child has had croup or other airway problems obstruction masquerading as croup are not
in the past and, specifically, whether the child present (Table 6.7.2). Especially in the youn-
Table 6.7.1 Croup severity
has had any persistence of mild stridor in ger child, one should enquire regarding
between acute attacks. This is important, as Sign or Mild Moderate or longer-term symptoms preceding the present
symptom severe
any child who has a pre-existing narrowing of episode, such as low-grade stridor. This might
the airway (infantile floppy larynx, laryngoma- Stridor None or only Stridor at rest suggest underlying congenital airway or vas-
if agitated
lacia or other upper airway anatomical abnorm- cular anomaly (e.g. tracheomalacia, subglottic
alities) is more likely to proceed to severe Respiratory Usually normal May be
rate decreased
obstruction with a superimposed acute obstruc-
tion. These children need to have a lower Retractions None to

threshold for a period of observation as their Table 6.7.2 Differential diagnoses

Air entry Normal Normal to
of croup
obstruction may be more severe or persistent. decreased
Epiglottitis
An immunisation history is important to Colour Normal May be pallor Bacterial tracheitis
check whether the child has had Hib vacci- Foreign body
Cyanosis None Late sign only Congenital: laryngomalacia, subglottic
nation if there is any suggestion that the stenosis, vascular ring, cord paresis
condition could be epiglottitis; likewise, the Conscious Normal Restless or Retropharyngeal abscess
state decreased Allergic oedema
very rare occurrence of diphtheria in the Airway trauma
non-immunised. After Marks et al 2003.2

143
6.7 CROUP

stenosis, bilateral cord paralysis, laryngeal allowing those with occasional stridor and 30 minutes.12 Combining dexamethasone
web, or vascular ring compression of the tra- barking cough only (mild) to be managed and budesonide is no more effective than
chea). One should also enquire as to possible at home. As many children will improve dexamethasone alone.11,13 There is no place
airway trauma or toxic ingestion. Dysphagia within a few hours of taking steroids, often for antibiotics in a typical case of croup.
and drooling may suggest epiglottitis, they may be discharged home after a short The use of steam or humidified air is
peritonsillar or retropharyngeal abscess, or stay in the emergency department or an unproven,14,15 despite its once common
foreign body in the airway or oesophagus. observation ward. Factors such as the dis- usage. The anecdotal report by parents of
Classic croup and epiglottitis are hard to tance from medical care, the availability of their child improving in the steam-filled
confuse, as the latter usually presents as a transport, the time of day, the childs past bathroom at home is due to the defervescing
pale, toxic, drooling child with a rapidly pro- history with regard to severe airway obstruc- of crying which occurs from cuddling in
gressing course. Cough is generally not a tion, and parental concern and attitude all the room by the parent, rather than any
prominent feature in epiglottitis. Children need to be taken into account when making steam effect.
with epiglottitis may sit forwards, drooling the decision to admit (Table 6.7.3).
saliva, and hold their neck in extension. In Recommended steroid doses are oral dexa- Severe croup
a child presenting with early epiglottitis, methasone in a one-off dose of 0.15 mg kg1,8 Children with manifestations of severe
however, the distinction may be more diffi- or an equivalent dose of prednisolone of obstruction should be given nebulised
cult. Immunisation in developed countries, 0.75 mg kg1. Most children with croup will adrenaline (epinephrine). It is generally con-
however, has made this distinction largely require only one dose, but if the upper airway sidered that adrenaline does not change the
academic. Allergic angio-oedema may mimic obstruction symptoms persist (as opposed to natural history of croup, such as length of
croup after exposure to an allergen such as upper respiratory tract infection symptoms), stay in hospital or need to intubate, due to
peanut. A child with severe croup with a a further dose may be given 18 to 24 hours its short-lasting effects. It will, however,
high fever, who does not respond to adrena- later. It is often more convenient to use pred- buy time while waiting for the effect of ster-
line (epinephrine) and steroids, may have nisolone (rounded off to 1 mg kg1) in the oids to occur. Rarely, in a worst-case scenario,
tracheitis and will need consideration of a community, as it is more readily available. adrenaline can be a useful temporising
diagnostic laryngoscopy to provide a clear While one study9 suggested that children measure while organising the facilities
diagnosis. Likewise, the possibility of an treated with prednisolone may re-present and appropriate personnel for a child who
inhaled foreign body should be kept in mind more commonly than those treated with dexa- may require intubation. The recommended
for children who dont respond to treatment methasone, Fifoot et al10 did not confirm this dose (independent of age and weight) is
or have a prolonged course. While usually finding. Steroids may be administered intra- 5 mL of 1:1000, nebulised with oxygen,
parents will volunteer a history of an acute muscularly or intravenously in the child with which can be used for all children. This may
obstruction or a sudden coughing fit, the severe obstruction, when there is concern be repeated after 10 minutes if needed and
history of an inhaled object may not always that the child may aspirate or vomit, given may help avoid the need for intubation in
have been observed and therefore reported. their degree of respiratory difficulty. children who respond to steroids. Rebound
A definitive diagnosis may need to be made Oral dexamethasone has been found phenomenon may occur, where the upper
by directly viewing the upper airway, but this to be as effective as inhaled steroids airway obstruction may recur as the effect
should be performed only by an experienced such as budesonide,11 and to work as fast, of the adrenaline wears off after 12 hours.
paediatric anaesthetist, intensivist, or emer- at a fraction of the cost. A blinded rando- While in the past it was recommended
gency physician in an appropriate clinical mised trial, submitted for publication, of that any child who received adrenaline for
setting (see below). dexamethasone 0.15 mg kg1 compared croup should be admitted, a number of
to placebo that looked at croup scores studies have now shown that children
at 10-minute intervals after administra- may be sent home safely if they have
tion showed a significant difference at also received steroids and have improved
Treatment and disposition
to have no stridor at rest over a number
Mild or moderate croup of hours.16,17 Children receiving nebulised
All children who present to an emergency adrenaline require close clinical monitoring
department with croup should be treated Table 6.7.3 Possible Indications for of their response, particularly the change
with steroids.4 The mandatory use of ster- admission in air entry, in order to detect any
oids for croup in emergency departments Upper airway obstruction at rest: fails to deterioration.
respond to steroids or adrenaline
results in a reduction in the relapse rate of (epinephrine) and period of observation (4
Intubation needs to be considered in the
those sent home,5 the average length of stay hours) child who has increasing upper airway
Severe croup or moderate croup that does
in hospital falling, and the number of chil- not respond to treatment
obstruction, hypoxia, decreasing conscious
dren needing intensive care and intubation Infants <6 months state, or fatigue despite nebulised adrena-
Previous severe croup
dramatically reducing.6,7 Prior to the regular Underlying airway problem
line. These children should be discussed
use of steroids, a general rule of thumb was Inadequate social factors i.e. transport or lack early with a paediatric intensivist in order
of follow up
to admit children with stridor at rest (moder- Recurrent presentation
to optimise management. The ideal setting
ate) to hospital for observation, while for this to occur is in theatre or a paediatric

144
6.7 CROUP
6

RESPIRATORY
intensive care unit environment via gase- 5. Geelhoed GC, Turner J, Macdonald WB. Efficacy of a small

ous induction, using an endotracheal tube Controversies and future single dose of oral dexamethasone for outpatient croup:
A double blind placebo controlled clinical trial. Br Med J
1.0-mm smaller than predicted by the childs research 1996;313(7050):1402.
6. Geelhoed GC. Sixteen years of croup in a Western
size (see Chapter 2.3). Although once controversial, steroids Australian teaching hospital: The impact of
routine steroid treatment. Ann Emerg Med
are now generally accepted for all 1996;28(6):6216.
children who present to emergency 7. Dobrovoljac M, Geelhoed GC. 27 years of croup: an
Prognosis departments with croup.
update highlighting the effectiveness of 0.15 mg/kg of
dexamethasone. Emerg Med Australas 2009;21:30914.
8. Geelhoed GC, Macdonald WGB. Oral dexamethasone
Most children with croup have mild symp- It is clear that both prednisolone and in the treatment of croup: 0.15 mg/kg is as effective as
toms, do not need hospitalisation, and will dexamethasone are effective in the 0.3 mg/kg or 0.6 mg/kg. Pediatr Pulmonol
recover within a few days. Their symptoms 1995;20:3627.
treatment of croup; however, two direct 9. Sparrow A, Geelhoed GC. Prednisolone versus
will be shortened even further with the dexamethasone in croup: A randomised equivalence trial.
comparisons have not resolved the
use of steroids. It should be pointed out Arch Dis Child 2006;91:5803.
question on whether the shorter half life 10. Fifoot AA, Ting JYS. Comparison between single-dose
to parents that steroids will have no effect oral prednisolone and oral dexamethasone in the
of prednisolone results in more relapses
on the duration of any underlying viral treatment of croup: a randomized, double-blinded clinical
at home. While a once-only dose of trial. Emerg Med Australasia 2007;19:518.
symptomatology. Despite the substantial 11. Klassen TP, Craig WR, Moher D, et al. Nebulized
dexamethasone is sufficient for the vast
impact of steroids, the occasional child budesonide and oral dexamethasone for treatment of
majority of children with croup, a second croup: A randomized controlled trial. JAMA 1998;279
will still follow a prolonged course of cough dose of prednisolone 24 hours later may (20):162932.
and marked stridor for many days. While 12. Dobrovoljac M. Geelhoed G.C. How fast does oral
be needed in some cases. dexamethasone work in croup patients? A randomised
other diagnoses, such as foreign body, need double blinded clinical trial. Emerg Med Australasia
to be considered, most cases will settle  Children with recurrent croup 2009;21(4):30914.
13. Geelhoed GC. Budesonide offers no advantage when
with time. theoretically should benefit from early added to oral dexamethasone in the treatment of croup.
treatment at home, but no formal study Pediatr Emerg Care 2005;21:35962.
14. Neto GM, Kentab O, Klassen TP, et al. A randomized
to date has confirmed this. controlled trial of mist in the acute treatment of
Prevention moderate croup. Acad Emerg Med 2002;9:8739.
15. Bouchier D, Dawson KP, Fergusson DM. Humidification in
viral croup: A controlled trial. Aust Paediatr J
For most children, croup is a one-off episode 1984;20:28991.
and well tolerated, especially if steroids are References 16. Kelley PB, Simon JE. Racemic epinephrine use in croup
and disposition. Am J Emerg Med 1992;10(3):1813.
used. Children who suffer repeated episodes 1. Denny FW, Clyde WA. Acute lower respiratory tract 17. Prendergast M, Jones JS, Hartman D. Racemic
infections in nonhospitalized children. J Pediatr
of recurrent croup, as described above, may 1986;108:63545.
epinephrine in the treatment of laryngotracheitis: Can
we identify children for outpatient therapy? Am J Emerg
benefit from steroid use at home at the first 2. Marks M, Wilkinson D, Munro J. Paediatric Handbook. Med 1994;12(6):6136.
6th ed Victoria: Blackwell Science; 2003.
sign of croup symptoms. Although no trials 3. Stoney PJ, Chakrabarti MK. Experience of pulse oximetry
have evaluated this approach, anecdotal in children presenting with croup. J Laryngol Otol
1991;105:2958.
evidence suggests that this practice appears 4. Geelhoed GC. Croup. Pediatr Pulmonol 1997;23
to have benefit. (5):3704.

145
SECTION

7 GASTROENTEROLOGY
AND HEPATOLOGY
Section editor Ian Everitt

7.1 Abdominal pain 146 7.8 Diarrhoea and vomiting 172

7.2 Gastrointestinal bleeding 152 7.9 Hepatitis 176
7.3 Gastro-oesophageal reflux 155 7.10 Intussusception 182
7.4 Pyloric stenosis 158 7.11 Herniae 184
7.5 Bilious vomiting 160 7.12 Gastroenteritis 186
7.6 Ingested foreign bodies 163 7.13 Constipation 192
7.7 Hepatic failure 165

7.1 Abdominal pain

Jason Hort

ESSENTIALS Introduction
1 Only a small percentage of children presenting with abdominal pain have an Abdominal pain is a common reason for chil-
underlying surgical cause. dren to attend an emergency department
(ED), occurring in up to 5% of all presenta-
2 The age of the child with abdominal pain significantly influences the diagnostic tions in some institutions.1 Most commonly
possibilities. the underlying cause is non-surgical and sur-
3 The younger the child, the less reliable are the clinical signs of acute appendicitis. gery is required in only 17% of children
who present with abdominal pain.1,2 It is
4 The abdominal examination findings, rather than blood tests, are the most not possible to make a definitive diagnosis
important contributor in assessing the need for surgical consultation. in all children with abdominal pain. In one
5 Providing adequate analgesia to the distressed child and an unrushed gentle study, as many as 15% of children present-
approach improve the reliability of physical findings and do not mask the detection of ing to emergency with abdominal pain did
peritoneal findings. not have a specific diagnosis at their dis-
charge. Some children arrive at the ED soon
6 Abdominal pain in children may have intra- or extra-abdominal causes. after the onset of symptoms and it may take
7 Warning bells for a surgical problem include: pain that genuinely requires opiate time, expectant management and review
analgesia, constant pain over a number of hours, inconsolability, a child who remains before a diagnosis becomes clearer or the
motionless despite severe pain (likely peritonism), associated pallor or shock, symptoms of a self-limiting cause resolve.
reproducible localised tenderness, guarding, rebound, abdominal distension, rigidity, It is important, however, to exclude causes
bilious vomiting and representation. of abdominal pain that may require early
surgical consultation, observation or investi-
8 Close observation with serial examination remains very useful in indeterminate cases. gations within the ED.
9 It is not always possible to make a definitive diagnosis in the ED. A practical, The priorities in managing children pre-
systematic approach is required to exclude serious underlying possible causes. senting with abdominal pain are:
Discharged children in whom the diagnosis remains unclear need to have a clear triage and early appropriate analgesia;
action plan with early follow up organised. resuscitation with attention to ABCs as
required;

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GASTROENTEROLOGY AND HEPATOLOGY

diagnosis formulation using history, Table 7.1.1 Causes of acute abdominal pain in children
examination and investigation;
Inflammatory gastrointestinal Gall bladder
consideration of surgical review and Appendicitis Cholecystitis, cholelithiasis
management according to the likely Meckels diverticulum
Mesenteric adenitis Haematological
diagnosis. Gastroenteritis Leukaemia, lymphoma
Food poisoning Haemolytic crisis
If a clear diagnosis cannot be reached Peritonitis Sickle cell disease
in the ED, then exclusion of serious/life- Peptic ulcer, gastritis Neuroblastoma, Wilms tumour
Hepatitis
threatening diagnoses is the priority. Pancreatitis Endocrine
Subsequent disposition and follow up is Inflammatory bowel disease Diabetic ketoacidosis, hypoglycaemia
Adrenal insufficiency
dependent on various factors including: like- Non-gastrointestinal Hyperparathyroidism
lihood of a serious diagnosis; severity of Tonsillitis, pharyngitis
Pneumonia (especially basal) Vasculitic
the pain; availability of review; and psycho- Pericarditis HenochSchonlein purpura
social factors that may be contributory. Serositis Periarteritis nodosa
Pyelonephritis, cystitis Kawasaki disease
Pelvic inflammatory disease
Intra-abdominal abscess Renal
Epididymitis Renal colic
Pathophysiology Hydronephrosis
Generalised Nephrotic syndrome
The sensation of abdominal pain is transmit- Infectious mononucleosis
ted by either somatic or visceral afferent Acute rheumatic fever Miscellaneous
Herpes zoster Constipation
fibres.3 Visceral pain from visceral perito- Colic
neum is poorly localised, whereas somatic Intestinal obstruction Toxic ingestion, e.g. lead
Intussusception Torsiontesticular/ovarian
pain arising from parietal peritoneum or Volvulus Ectopic pregnancy
the abdominal wall is more localised. Adhesions Dysmenorrhea, Mittelschmerz pain
Incarcerated hernia Mesenteric artery occlusion
Referred pain also occurs due to visceral Hypokalaemia
and somatic pathways converging in the spi- Abdominal trauma Acute intermittant porphyria
See Section 3 Familial Mediterranean fever
nal column. Two examples of referred pain Abdominal migraine
are diaphragmatic irritation leading to pain Psychosomatic including abuse
at the shoulder tip due to convergence Source: Adapted from Rudolph 1996.
of visceral and somatic pathways at C4,
and somatic pain from pneumonia leading
examination. A detailed history and thor- Neonates and infants They usually
to T1011 pain sensed in the lower abdo-
ough, gentle, and age-appropriate examina- present with a change in behaviour to sig-
men.3 Abdominal pain may occasionally
tion need to be performed. nify pain.4 This may be persistent crying, irri-
be found to be psychosomatic in origin
tability, inability to be consoled, fussiness,
after a thorough assessment of alternative
sleeplessness, and poor feeding.4 Serious
causes. History or potentially life-threatening conditions
In considering a child who has presented not to miss in this age group are listed in
Aetiology with abdominal pain with no history of Table 7.1.2.
trauma, five important questions have to The diagnoses of acute gastroenteritis or
There is a broad range of causes of be addressed: colic need to be made after excluding more
abdominal pain in children and one needs serious causes.
to initially keep an open mind regarding 1. The age of the child
the diagnosis (Table 7.1.1). The age and The age of the child helps narrow the diag- Preschool Common conditions include
sex of the child need to be considered, as nostic possibilities. The most common diag- acute gastroenteritis, urinary infection,
well as features of the abdominal pain noses to consider according to age are: appendicitis, viral illness (mesenteric
and associated symptoms, and examina-
tion findings to determine the diagnostic Table 7.1.2 Serious conditions not to miss in neonates and infants
possibilities.
Surgical causes Medical causes

Testicular torsion Diabetic ketoacidosis

Assessment Appendicitis
Peritonitis
Toxic, e.g. iron ingestion
Sepsis
Necrotising enterocolitis
Each child needs initial assessment and tri- Volvulus Haemolytic uraemic syndrome
age with attention to securing the ABCs Intussusception Urinary tract infection
Hirschsprung disease
and providing appropriate early analgesia. Incarcerated hernia
This will help achieve a more reliable

147
7.1 ABDOMINAL PAIN

adenitis), pneumonia, constipation and frequency may be reported in older children children, after pain subsides post analgesia,
trauma-related abdominal pain. with a urinary tract infection. A typical rash the child has relaxed and become accus-
or sore joints may indicate HenochSchon- tomed to the examination.
School age Children of school age usu- lein purpura as the cause of related vasculi- One of the keys to the assessment is to
ally have acute gastroenteritis, urinary infec- tic abdominal pain. determine the presence of focal tenderness
tion, trauma, appendicitis, constipation, or true peritoneal irritation. In children this
viral-related, psychosomatic, inflammatory 4. Whether there are any relevant can sometimes be difficult, as many volun-
bowel or in older females consider gynaeco- pre-existing conditions tarily guard the abdomen when examined,
logical causes. The childs past medical and surgical history irrespective of the cause of the abdominal
In the preschool and school-aged child, the should be fully explored. In older females an pain. This is expected when a previous
commonest cause of abdominal pain is consti- adolescent approach (see Chapter 30.1) and examination may have been distressing.
pation. This is a diagnosis of exclusion after a menstrual and sexual activity history may Differentiating the presence of true peri-
carefully considering alternative causes.5 be important. Family history and racial back- toneal signs may be helped by distraction
ground may be relevant, along with a psy- techniques, or by serial gentle examination
Adolescents The causes of abdominal chosocial history that may contribute if over a period of time to determine true
pain in adolescents expand to include vari- there is a suggestion of somatisation. reproducibility of findings.
ous diagnoses of adulthood. In the female, Eliciting rebound should begin with gen-
pregnancy-related conditions must be con- 5. What are the characteristics tle palpation to avoid distress and resultant
sidered, as well as other gynaecological con- of the pain? voluntary guarding. Signs consistent with
ditions (see Chapter 15.1 on gynaecology). These include location (generalised or loca- peritonitis include refusing or being unable
lised), radiation, severity, quality (constant, to walk, slow or stooped walking, or
2. Whether this is the first episodic or of a colic nature), what improves increased pain on coughing or movement,
episode or recurrence of and worsens the pain, along with the timing or the child lying motionless on the bed.
abdominal pain of the pain gradual or sudden onset and Likewise, peritoneal irritation may be
A history of similar pain in a child may help whether the pain wakes the child. Direct detected by asking if the child can expand
to clarify the diagnosis. However, it is impor- questioning of the verbal child often aug- or contract their abdominal wall by asking
tant to remember that children with chronic ments the description of the characteristics them to suck tummy in, then let it out.
abdominal pain due to myriad causes may of the pain as reported by a parent. The younger the child, the less likely they
develop an acute abdomen and this needs Pain that has a sudden onset may occur are to have reliable localising signs of
to be considered at each presentation. with perforated viscus, intussusception or appendicitis and the threshold for surgical
torsion. The pain from appendicitis has a review or observation needs to be adapted
3. Whether there are other more insidious onset and usually increases accordingly.
associated symptoms over a period of hours. In both intussuscep- Important features of examination
Generally children with abdominal pain tion and mesenteric adenitis, the pain may include the following:
have other associated symptoms. A full cause episodic distress. Constant pain over
symptom review is required, with particular
general appearance of the child;
a number of hours in a child usually suggests temperature and vital signs;
reference to gastrointestinal symptoms. This a significant underlying cause.
includes vomiting, and whether it is bilious
jaundice;
or blood stained, and the timing and quality
skin markings, e.g. abdominal bruising;
of stool, including the presence of blood or
abdominal distension;
mucus. The child with fever, voluminous diar-
Examination abdominal tenderness;

rhoea and vomiting is likely to have gastro- The abdominal examination of the young
guarding or rebound and/or rigidity;

enteritis. However, particularly in young child with abdominal pain needs to be per-
abdominal mass;

children, one must keep an open mind to formed in an unhurried and gentle fashion.
bowel sounds;

other possibilities that can mimic or compli- Toddlers may be better examined on the par-
palpation of testes (in the male infant);
cate gastroenteritis (see Chapter 7.8 pp. ents lap. It is important to note the childs
presence of inguinal herniae;
172175 on Diarrhoea and vomiting). general appearance and any features of tox-
urinalysis.
More general questions should assess the icity. Children with colicky pain often writhe Rectal examination in children, when rarely
childs constitution whether the child is around, whereas the child with peritoneal indicated, should be performed once, and
febrile, lethargic, irritable, anorexic or has irritation usually remains still as movement ideally by the surgeon who may require
associated pallor. It is unusual for children exacerbates the abdominal pain. Providing the information. The interpretation of loca-
with appendicitis to be hungry. The child adequate analgesia improves the reliability lised rectal tenderness is often difficult in
with intussusception is often lethargic and of physical findings and does not mask the children, as it is uncomfortable in all children
pale. The presence of upper respiratory tract clinical detection of peritoneal findings. It and therefore does not often add to the
infection (URTI) or viral symptoms may sug- is best to very gently approach the painful assessment. The inguinoscrotal regions
gest mesenteric adenitis. Dysuria or quadrant of the abdomen last in distressed should always be checked for an otherwise

148
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7

GASTROENTEROLOGY AND HEPATOLOGY

occult hernia or torsion referring pain Females of reproductive age should have (23 minutes) with the advantage of not
upwards to the abdomen. A sensitively a bhCG performed. Urinalysis, microscopy requiring venous access, but its short dura-
performed, private, and chaperoned pelvic and culture are necessary to exclude renal tion of action (3060 minutes) means
examination may be indicated in pubertal pathology. longer-acting analgesia will be required if
females. pain is ongoing.6 Oral agents such as para-
The cause of abdominal pain in children Imaging cetamol, codeine or ibuprofen may be used
may be extra-abdominal. The child with The routine use of an abdominal X-ray is in less severe pain. Serial examination of
ileus may have intra- or extra-abdominal unhelpful to screen children with abdomi- the childs abdomen and observation of vital
pathology including sepsis, urinary tract nal pain. Specific situations where it may signs over a period may be important to
infection, pneumonia, or meningitis. be helpful include: demonstrating the signs exclude significant pathology. Children with
Important features in the examination of of bowel obstruction; free air, suggesting a potential surgical cause should be given
other systems that may present with abdom- perforation; an ingested foreign body; nil by mouth, until surgical review.
inal pain include: calcification, including a faecolith in the
respiratory - signs of basal pneumonia; appendix and Meckels diverticulum; thumb-
ENT URTI, tonsillitis or adenopathy; printing, suggesting gut ischaemia or a sen- Disposition
neurological meningitis; tinel loop adjacent to inflammation; or a soft
tissue mass. Calcification may also represent Children with significant abdominal findings
endocrine blood glucose level in
need to have a surgical consultation. Not all
diabetic ketoacidosis; renal stones, neuroblastoma or teratoma.
An erect chest X-ray may be indicated to children who present with abdominal pain
haematological pallor,
will have a clear definitive diagnosis after
lymphadenopathy; demonstrate gas under the diaphragm or
evidence of a basal pneumonia.3 their assessment has excluded a serious
dermatological rash, particularly
cause or the need for a surgical review. Some
purpura/petechiae of HenochSchonlein An ultrasound study can help evaluate
liver, gall bladder and kidneys, as well as children with severe pain, but negative phys-
purpura, zoster;
detect intussusception, features of appendi- ical examination findings, where the diagno-
renal oliguria, haematuria,
sis is not clear, will warrant admission for
hypertension in haemolytic uraemic citis (if able to be visualised) or evaluate a
palpable mass. An abdominal computerised ongoing observation. Children who are dis-
syndrome.
tomography (CT) scan may be indicated in charged home need to have clear instruc-
selected cases. tions given to parents regarding returning,
should they deteriorate, and have a planned
Investigations timely follow up by the local doctor.

Many children have the diagnosis clarified Management

by a physical examination alone. The need
The initial management of the child with
for investigations should be tailored to the
severe abdominal pain should include ACUTE APPENDICITIS
individual case, where the diagnosis is
assessment and securing of ABCs and
unclear and the result of the test is likely
administration of appropriate analgesia to
to rule in or rule out significant pathology.
relieve the childs distress. Children with sim-
Introduction
ilar pathophysiology can have markedly Appendicitis is the most common non-
Pathology varied distress levels and analgesic require- traumatic surgical emergency in children.
A full blood count may demonstrate leucocy- ments need to be individualised.4 Concur- It occurs slightly more commonly in males
tosis, anaemia or film changes of haemolysis. rent anxiety may increase painful stimuli at any age, although it is uncommon in
However, the white cell count/differential is and this can be lessened by involving par- those under 2 years, and very rare in neo-
not particularly useful to determine if a child ents in comforting their child and using a nates. The peak incidence is at 912 years.
has acute appendicitis. Electrolytes, urea child-friendly environment to distract and
and creatinine assess changes resulting from help calm the child. Using a visual analogue
fluid losses and impaired renal function. This scale to evaluate severity of pain may be
may be indicated in the child who has had helpful to assess response to analgesia.
Clinical features
significant losses from diarrhoea or vomit- There is no contraindication to providing The clinical features of classic appendicitis
ing. Liver function tests are indicated in chil- adequate analgesia for any child presenting are well known. Pain is felt initially in the
dren with potential hepatic and gall bladder in pain. It is much easier to perform a reli- periumbilical region due to visceral pain
pathology. The possibility of pancreatitis able examination on a child who is made from obstruction of the appendix. There is
can be assessed by serum amylase or lipase comfortable. For severe distress, intravenous often associated nausea, vomiting, anorexia
levels. A blood glucose level excludes dia- morphine titrated in increments controls and a low-grade fever. Later, there is migra-
betic ketoacidosis in the child presenting most childrens pain and will not mask the tion of the pain to the region of the appen-
with abdominal pain who appears acidotic abdominal signs. Intra-nasal fentanyl is a dix. This more intense right lower quadrant
or has glucose and ketones in the urine. useful alternative for rapid onset analgesia pain results from irritation of the abdominal

149
7.1 ABDOMINAL PAIN

parietal lining. Up to 50% of adults have equivocal cases, imaging of the appendix appendicitis.9 However, several disadvan-
this progression of symptoms but it is less may be helpful or demonstrate alternative tages including high cost, long duration of
common in children. causes of the pain. study, and limited availability mean MRI
Importantly, some children may have false No single test is diagnostic in appendici- has a limited role at the moment. It appears
localising diarrhoea or dysuria caused by irri- tis, with the white blood cell count insensi- potentially useful in pregnant patients
tation from an inflamed appendix. Fever is tive and non-specific.8 C reactive protein with suspected appendicitis in whom ultra-
generally below 39.5 C, unless perforation (CRP) levels >10 mg dL1 have varying sound is inconclusive.8
has occurred. Asking the child to walk or reported sensitivities (4875%) and specifi-
hop to demonstrate pain with right leg cities (5782%) in different studies on Active observation Has also been used
movement may be useful in indeterminate appendicitis. Normal CRP values do not without investigation, with a positive
cases to reveal the presence of true perito- exclude acute appendicitis in children.8 On predictive value of 97.9% in one series
neal irritation. Likewise, manoeuvres such urine microscopy more than five white blood and a normal appendicectomy rate of 2.6%.
as the iliopsoas, obturator or Rovsings signs cells per high power field or the presence of
may help confirm suspicion of appendicitis. red blood cells is found in 725% of children
In children with clear signs of appendicitis, with appendicitis. Abdominal X-rays may
Management
the rectal examination adds little value, is show other pathology (e.g. right lower lobe
distressing for a child and does not alter pneumonia) and occasionally an appendi- The management of acute appendicitis is
management.7 ceal faecolith, but are also insensitive and appendicectomy. Some children require
Under the age of 2 years, vomiting non-specific for diagnosing appendicitis. intravenous rehydration, ongoing analgesia
(8590%) and pain (3577%) are the most Note the rare presence of an appendolith and antibiotics, if perforation is suspected.
common symptoms, with diarrhoea (18 can give a more colicky nature to the pain. The management of the child in whom
46%) and fever (4060%) less common. the diagnosis is not clear, but may be possi-
Sometimes grunting respirations (823%), Ultrasound Has reported sensitivity of ble early appendicitis, is more difficult and
cough or rhinitis (40%) and right hip symp- 7192% with specificity of 8698% and one needs to have a clear approach.
toms (323%) may be misleading. Right is often used when there is initial diagnos- In some children it may be useful to per-
lower abdominal tenderness is present in less tic doubt. However, in one series patients form imaging of the appendix such as ultra-
than 50% so the diagnosis of appendicitis in undergoing sonography before appendicec- sound, particularly in older females who may
this age group is often delayed, leading to per- tomy had a longer delay before operation, a have ovarian pathology as a cause of their
foration rates of 8292%. higher rate of misdiagnosis, and more post- pain. An expectant approach is appropriate
As children become older, right lower operative complications. It is not uncommon in some children with a short history, with a
abdominal tenderness becomes more com- for the appendix to be difficult to visualise clear plan of organised review over the next
mon (age 25 years, 5885%), up to nearly (up to 10% of cases). High positive likeli- 612 hours. This may be achieved by
all children in the school-age group, with some hood ratios and moderate negative likeli- actively observing the child in hospital or,
(15%) having generalised tenderness without hood ratios suggest that ultrasound can be if discharged, by arranging a definite early
perforation. In children 612 years, vomiting used to confirm but not exclude a diagnosis clinical review. Parents need clear instruc-
occurs in 6895% of children, anorexia in of appendicitis.8 tions to return should their childs symptoms
4775%, diarrhoea in 916%, constipation progress.
in 528% and dysuria in 420%. CT scan Abdominal helical CT scan in diag-
nosing appendicitis shows sensitivities from
87100% and specificities 8397% with
MECKELS
signs of distension of the appendix, faeco-
Differential diagnoses DIVERTICULUM
lith, focal caecal wall thickening and fluid
These include mesenteric adenitis (less collections in ruptured appendicitis. Some
severe pain without peritoneal signs is institutions have used a protocol incorporat-
usual and it is rare under 3 years of age), ing ultrasound and subsequent CT scan with
Introduction
bacterial enterocolitis, pelvic inflammatory patients whose diagnosis is equivocal. They Meckels diverticulum is a vestige of the
disease, urinary tract infection, Meckels have shown this to be an accurate and cost- omphalomesenteric duct, occurring in 2%
diverticulitis, intussusception and right effective approach when compared with a of the population, with 2% of those with
lower lobe pneumonia. negative appendicectomy rate of 23%. Meckels diverticulum manifesting symp-
The American College of Radiology in gen- toms. The diverticulum is usually 60 cm
eral advocates the use of ultrasound over proximal to the terminal ileum. Of symptom-
CT scan. atic patients, 45% are under the age of 2,
Investigations
but it can present at any age. These findings
Children with a clear clinical diagnosis of Magnetic resonance imaging (MRI) are known as Meckels rule of twos. The
acute appendicitis do not require investiga- Recent studies suggest high sensitivity majority of Meckels diverticuli contain gas-
tions and delay of surgical consultation. In and specificity in adult patients with tric mucosa and may secrete acid.

150
7.1 ABDOMINAL PAIN
7

GASTROENTEROLOGY AND HEPATOLOGY

of pain occurring over a period of 3 months
Table 7.1.3 Causes of chronic
Clinical features or longer.3 The reported prevalence of abdominal pain
The presenting features of a Meckels diver- abdominal pain interfering with activities is
Gastrointestinal causes
ticulum vary and may include abdominal 1015% in children between 5 and 14 Chronic recurrent functional abdominal pain
pain, bowel obstruction, gastrointestinal years. Causes of chronic abdominal pain Peptic ulcer disease
Irritable bowel syndrome
haemorrhage, or perforation. are diverse and are listed in Table 7.1.3. Inflammatory bowel disease
Meckels diverticuli are the most common Signs and symptoms suggesting organic Chronic or recurrent pancreatitis
Biliary colic
cause of significant lower gastrointestinal disease causing chronic abdominal pain in Appendiceal colic
bleeding in children, usually from peptic school-aged children include: Constipation
Partial bowel obstruction
ulceration within the diverticulum or adja- persistent fever; Parasitic infection
cent ileum. It is classically painless bleeding, poor weight gain or weight loss; Endocrine disease
with stools either bright red or tarry, depend- child awakened from sleep; Hyperparathyroidism
Addisons disease
ing on the site and briskness of bleeding. pain away from the umbilicus; Diabetes mellitus
Diverticulitis causing crampy lower quad- radiation of pain to back, shoulder or
rant pain can occur in older children. A Cardiovascular disease
lower extremities; Superior mesenteric artery syndrome
Meckels diverticulum can be a lead point in persistent regurgitation, vomiting or Coarctation of aorta
intussusception, as well as causing intestinal dysphagia; Neurological disease
obstruction by the formation of intraperito- bloody emesis or stools; Abdominal migraine
neal bands, which can lead to the possibility Migraine headaches
associated altered bowel pattern; Familial dysautonomia
of a volvulus or internal herniation. perianal disease;
Haematological disease
sleepiness following pain attacks;
Sickle cell disease
positive family history of peptic ulcer or Porphyrias
Differential diagnoses inflammatory bowel disease.
Gynaecological disorders
Cystic teratoma of ovary
These include intestinal polyps, intussuscep- Endometriosis
tion, anal fissures, midgut volvulus and bac- Haematocolpos
Mittelschmerz
terial enteritis. Assessment
Musculoskeletal disorders
The assessment of the child with chronic Discitis
abdominal pain involves a detailed history Linea alba hernia
Investigations and examination. Blood pressure should be
Painful rib syndrome
Muscle wall sprain
A Meckels scan using 99mtechnetium can be measured. The child who has no features
Other
used, which is 7585% sensitive and 95% to suggest an organic origin requires no test- Uteropelvic junction obstruction
specific11 at demonstrating the ectopic gas- ing, although some clinicians opt to perform Familial Mediterranean fever
Hereditary angioneurotic oedema
tric tissue, when bleeding is present. a limited screen involving urinalysis, full
blood count and erythrocyte sedimentation Source: Adapted from Rudolph 1996.3
rate, and stool testing for ova and parasites
Management and occult blood.

Management is intravenous fluid support

with blood transfusion for massive bleeding.
A Meckels scan can be done in stable bleed- Diagnosis Management
ing patients, but if unstable or with perito-
The diagnosis of recurrent functional In the ED situation, the management of
neal signs, urgent surgical consultation
abdominal pain is based on history and a chronic abdominal pain depends on the
must be obtained with a view to operative
normal physical examination. Usually the possible diagnoses. Follow up should be
intervention. Definitive treatment is surgical
patient has no worrying features (as listed ensured, particularly in the child with the
excision, which may be done laparoscopically.
above), has periumbilical or mid-epigastric suggestion of organic disease or the child
pain, and rarely wakes at night from the missing a significant amount of school.
pain. Psychosocial stressors may be evident.
CHRONIC ABDOMINAL
There may be secondary gain from the
PAIN childs abdominal pain. Reassurance is the
treatment of choice, although it is important
Controversies
to acknowledge that the child does experi-
Introduction ence pain. Cognitivebehavioural therapy The best approach to
Chronic abdominal pain is defined as the may be useful for children who clearly have investigations in appendicitis has not
presence of at least three discrete episodes recurrent functional abdominal pain.10 yet been settled.

151
7.2 GASTROINTESTINAL BLEEDING

4. DAgostino J. Common abdominal emergencies in 10. Banez G. Chronic abdominal pain in children: what to do
There is probably little need for a children. Emerg Med Clin North Am 2002;20:1. following the medical evaluation. Curr Opin Pediatr
5. Browne GJ, Choong RKC, Gaudry PL, Wilkins BH. 2008;20:5715.
rectal examination in children with Principles and practice of childrens emergency care. 11. Behrman R, Kliegman R, Jenson H. Nelsons Textbook of
abdominal pain, but more research is Sydney: Maclennan and Petty;1997. Paediatrics. 16th ed. Philadelphia: WB Saunders; 2000.
6. Borland ML, Jacobs I, Geelhoed G. Intranasal fentanyl
needed before making definitive reduces acute pain in children in the emergency
conclusions. department: a safety and efficacy study. Emerg Med
2002;14(3):27580.
7. Dunning PG, Goldman MD. The incidence and Further reading
value of rectal examination in children with
suspected appendicitis. Ann R Coll Surg Engl Bachoo P, Mahomed AA, Ninan GK, Youngson GG. Acute
References 1991;73:2334. appendicitis: The continuing role for active observation.
Paediatr Surg Int 2001;17(23):1258.
1. Scholer SJ, Pituch K, Orr DP, Ditttus RS. Clinical outcomes 8. Kwok M, Kim M, Gorelick M. Evidence-based Approach
to the diagnosis of appendicitis in children. Pediatr Emerg Emil S, Mikhail P, Laberge JM, et al. Clinical versus
of children with acute abdominal pain. Paediatrics sonographic evaluation of acute appendicitis in children:
1996;98:6805. Care 2004;20(10):690701.
9. Cobben L, Groot I, Kingma L, et al. A simple MRI A comparison of patient characteristics and outcomes.
2. Simpson ET, Smith A. The management of acute J Paediatr Surg 2001;36(5):7803.
abdominal pain in children. J Paediatr Child Health protocol in patients with clinically suspected
appendicitis: results in 138 patients and effect on Garcia-Pena BM, Taylor GA, Fishman SJ, et al. Costs and
1996;32(2):1102. effectiveness of ultrasonagraphy and limited computed
3. Rudolph A. Rudolphs Textbook of Paediatrics. 20th ed. outcome of appendectomy. Eur Radiol 2009;19
(5):117583. tomography for diagnosing appendicitis in children.
USA: Appleton and Lange;1996. Paediatrics 2000;106:6726.

7.2 Gastrointestinal bleeding

Elizabeth M. Cotterell

Melaena is the passage of black, tarry

ESSENTIALS stool. This is caused by bacterial degra-
dation of haemoglobin and implies that
1 The causes of gastrointestinal (GI) bleeding in infants and children fall into age- the bleeding has occurred over a period
specific diagnostic categories.
of hours.
2 The majority of GI bleeding ceases spontaneously, requiring no treatment or Haematochezia is the passage of bright
treatment on the basis of a presumptive diagnosis. red blood per rectum. Haematemesis and
melaena are usually indicative of an upper
3 A good history is important in determining the likely site of GI bleeding, the GI bleeding source. The passage of fresh
significance and acuity of the bleed and guides appropriate investigations.
blood per rectum usually indicates a source
4 Initial assessment is for signs of haemodynamic compromise due to the blood loss, of blood from the lower GI tract. It can, how-
followed by identification of underlying disease. ever, derive from an upper GI source, espe-
cially in infants less than six months, due
to rapid colonic transit times. Streaks of
blood may be mixed with the stool, usually
The epidemiology of GI bleeding in chil- indicative of colitis, as compared to blood
Introduction dren is very limited. The reported incidence coating a hard or normal stool which may
of GI bleeding of 6.4% in paediatric ICU be due to an anal fissure.
Gastrointestinal (GI) bleeding in infants and
children is an uncommon cause of presenta- patients1 and the most frequent diagnoses
tions to an emergency department (ED) but confirmed endoscopically2 (duodenal and
nonetheless is an alarming symptom that gastric ulcers, oesophagitis, gastritis, and
concerns parents greatly. Fortunately, in varices) represent selected populations and Aetiology
the majority of infants and children, the are not representative of the ambulatory The aetiology of GI bleeding is best consid-
cause is benign or relatively uncomplicated, paediatric population. ered within defined age groups, with some
and not associated with significant morbid- overlap between groups, and the likely loca-
ity or mortality. There are, however, some Definitions tion of the bleed, as guided by history and
less common conditions that occur in infancy Haematemesis is the vomiting of blood that examination (Tables 7.2.1 and 7.2.2).
and childhood that may be a cause of poten- may be either fresh (bright red) or altered The term newborn infant who is breast
tially life-threatening blood loss and require by gastric acidity and described as coffee fed and has GI bleeding is most likely to have
rapid assessment and resuscitation. grounds. ingested maternal blood either at the time

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GASTROENTEROLOGY AND HEPATOLOGY

Table 7.2.1 Causes of upper GI bleeding (causes listed most common to rare) Table 7.2.3 Chart for guided history
taking for GI bleeding
Neonates (<1 month) Infants (1 month to 1 year) Toddlers and school age
Is it blood?
Ingested maternal blood Ingested blood Ingested blood
Consider haemoccult(r) test on stool samples
Gastritis Reflux oesophagitis Reflux oesophagitis
and gastroccult(r) on vomit or NG aspirates
Vascular malformations Gastritis Gastritis
History of ingestion of food colouring or iron
Bleeding disorders MalloryWeiss tear MalloryWeiss tear
supplements
Peptic ulceration Oesophageal varices
Vascular malformation Peptic ulceration
Bleeding disorders Bleeding disorders Is it from the GI tract?
Consider other sources for the blood
Ingestion of maternal blood at delivery or breast
feeding
History of epistaxis, post ENT or oral surgery
or pharyngitis
Table 7.2.2 Causes of lower GI bleeding
Where in the GI tract is it from?
Neonates Infants Toddlers and school age
Upper GI tract haematemesis, melaena or
Ingested maternal blood Anal fissure Anal fissure haematochezia for profuse bleed
Necrotising enterocolitis Protein sensitive enterocolitis Juvenile colonic polyps Lower GI tract haematochezia, redcurrant jelly
Protein-sensitive enterocolitis Hirschsprungs enterocolitis Infectious gastroenteritis stools
Hirschsprungs enterocolitis Ischaemic enterocolitis Meckels diverticulum
Ischaemic enterocolitis Infectious gastroenteritis Intussusception Is it a significant amount?
Infectious gastroenteritis Meckels diverticulum Ischaemic enterocolitis Estimate acuity of bleed by history and assess
Congenital bleeding disorders Intussusception Haemolytic uraemic syndrome clinical signs
Haemolyticuraemic syndrome HenochSchonlein purpura Resuscitate immediately if haemodynamic
Bleeding disorders Inflammatory bowel disease compromise
Vascular malformation Vascular malformation
Inflammatory bowel disease Bleeding disorders Are there other concerning symptoms
or signs?
Paroxysmal abdominal pain and/or lethargy
of delivery or from breast feeding from HenochSchonlein purpura will manifest with intussusception
Bilious vomiting volvulus
cracked nipples. Premature infants are at the typical palpable purpura on extremities History of chronic liver disease oesphageal
an increased risk of necrotising enterocolitis, as well as abdominal pain. Certain diagnoses varices
Renal or haemotological abnormalities
although it can occur in term neonates with also have a recognised age pattern; juvenile
birth asphyxia or cyanotic heart disease. Any polyps have a peak incidence of 1 to 6 years
sick newborn, compromised by hypoxia or of age, intussusception peaks at 5 to 18 A positive family history may be helpful,
hypotension is at risk of GI bleeding from months, and inflammatory bowel disease particularly with the less common causes, such
stress ulcers. An infant who has not received more commonly presents in adolescence, as polyps, inflammatory bowel disease, coagu-
parenteral vitamin K after birth, or has inter- although can occur at any age. lation disorders, haemorrhagic telangiectasia
ference with vitamin K absorption, is at risk and Hirschsprungs disease. A medication his-
of haemorrhagic disease of the newborn. tory, including use of non-steroidal anti-
Formula fed infants may develop cows milk inflammatory drugs (NSAIDs), salicylates
protein intolerance within the first few
History and anticoagulants may provide further clues.
weeks to months. Details of the timing of the blood appearing Inadvertent ingestion of caustic agents and
The bowel habit of the infant or child prior in the vomitus or bowel motions, in relation rodenticides containing warfarin-type agents
to onset of GI bleeding is important to note. to other events, may give a clue to alterna- should be considered in preschool-age
Constipation associated with pain when tive sources of the blood. For example, the children.
straining at stool would make an anal fissure ingestion of substances such as iron or food
a probable diagnosis. The older infant or colourings, onset of epistaxis or recent oral
child with cerebral palsy may have severe or ENT surgery, indicate a source of bleeding
gastro-oesophageal reflux and therefore
Examination
other than from the GI tract (Table 7.2.3).
most likely has an oesophagitis-related A history of recurrent retching or vomiting Initial assessment is for the presence or
source of upper GI bleeding. prior to the appearance of blood in the vomit absence of any haemodynamic compromise
The key factor in identifying the cause of GI suggests a MalloryWeiss tear, but may due to the blood loss. This is uncommon in
bleeding in toddlers and older children is the occur in the absence of these symptoms. children but, if present, clinical assessment
presence of associated symptoms. Crampy Symptoms of epigastric pain and nausea proceeds while resuscitation is commenced.
abdominal pain and diarrhoea with mucus may be elicited with gastritis. Crampy Specific findings in the physical examina-
and fresh blood may be caused by infectious abdominal pain with a passage of loose tion of the GI tract may establish potential
gastroenteritis due to Campylobacter, Shi- stools, with blood and mucus mixed through- causes for the GI bleeding. The abdomen
gella, Salmonella and Yersinia. Intermittent out, suggest infectious or inflammatory coli- should be assessed for any localised tender-
colicky abdominal pain with episodes of leth- tis. Fresh blood coating the stool implies a ness or peritoneal signs. Children with minor
argy occurring in intussusceptions may mani- lesion in the lower rectum or anus, such as bleeding in the setting of gastroenteritis
fest with blood in the stools as a late sign. a polyp or fissure. will have a benign abdomen. The finding

153
7.2 GASTROINTESTINAL BLEEDING

of hepatosplenomegaly with stigmata of Stool testing coagulopathy with fresh frozen plasma, acti-
chronic liver disease may suggest oesopha- Stool samples for microscopy, looking for red vated factor VII or platelet infusions may be
geal varices as the cause. Palpation of a ten- and white cells, as well as for bacterial cul- needed to control the GI bleeding.
der abdominal mass may support a diagnosis ture, should be sent if bacterial enterocolitis
of intussusception. Visualisation of an anal is suspected. Pharmacological treatment
fissure is important to confirm the source Studies addressing the use of H2 antagonists
of blood on stools when the history is sugges- Endoscopy and proton pump inhibitors (PPIs) for acute
tive with the defect in the rectal mucosa usu- The role of endoscopy for investigating GI upper GI bleeding are restricted to the adult
ally superficial and posteriorly located. bleeding in the acute setting in children is population. PPIs have been shown in sys-
Consider beta haemolytic streptococcal infec- limited. It can, however, serve both a diag- tematic reviews to reduce the risk of further
tion if there is marked perianal erythema. nostic and therapeutic role, especially in bleeding and need for surgery for peptic
Skin and mucus membrane examination the case of oesophageal varices. Consulta- ulcers.4 However, the routine use of H2
may reveal signs of a bleeding tendency tion with a paediatric gastroenterologist or antagonists or PPIs in children with acute
due to a coagulation or platelet disorder. surgeon is helpful in determining those GI bleeding cannot be recommended as
Clinical signs of anaemia are more sugges- patients most likely to benefit from this bleeding gastric ulcers are very uncommon.
tive of a chronic picture of GI bleeding. Exam- investigation. Intravenous somatostatin or octreotide
ination of the nose and throat may show a have been shown to be effective for uncon-
nasopharyngeal source for ingested blood. Imaging trolled bleeding oesophageal varices by
In addition, parents may often provide evi- Nuclear medicine studies such as techne- decreasing splanchnic blood flow; however,
dence of the symptoms that they have tium pertechnetate scan for ectopic gastric most variceal bleeding ceases spontaneously.
reported, such as a cloth with blood-streaked mucosa in a Meckels diverticulum may have Bacterial enterocolitis is usually self limit-
vomitus or a nappy with bloody streaks in the a role in investigating the cause of GI bleed- ing and does not require empiric antibiotic
faeces. In the event of a fresh sample of stool ing. However, the Meckel scan has a relatively treatment.Children with gastroduodenal
or vomit being provided, testing for occult low negative predictive value and may not ulceration shown to be associated with Heli-
blood will verify if GI bleeding has occurred. obviate the need for operative evaluation cobacter pylori infection may benefit from
despite a negative scan, where clinical suspi- antibiotic eradication treatment.
cion is high.3 Other imaging techniques Infants with a presumptive diagnosis of
Investigations such as ultrasound and contrast air enema cows milk protein intolerance should be
for intussusception should be considered changed to a hydrolysed protein formula with
The appropriate investigation of GI bleeding
in children under the age of 2 years with expected improvement within days. Mothers
needs to be tailored to the most likely cause
abdominal pain, GI bleeding and lethargy. of breastfed infants can be advised to elimi-
on the basis of history and examination
nate all dairy products from their diet if this
findings.
diagnosis is considered for their infant.5
Treatment Children with bleeding from an anal
Blood tests fissure may require laxatives to ensure regu-
Blood tests for haemoglobin level and red Initial assessment lar soft stools so that the fissure will heal.
cell indices may indicate a hypochromic and stabilisation
microcytic anaemia and point to a chronic In the event of significant GI bleeding, the
source of bleeding. Haematocrit changes child or infant will develop signs of haemo-
in the acute setting are unreliable indicators dynamic compromise. Cool peripheries, dela-
Surgery
of acute bleeding. Coagulation profile and yed capillary refill and tachycardia will be Laparoscopy or laparotomy for investigation
platelet count should be checked if the his- the first detectable signs. Hypotension occurs of lower GI bleeding may be both a diagnos-
tory and examination findings suggest a as a late sign of hypovolaemia in children. tic and therapeutic tool in children with GI
bleeding diathesis or chronic liver disease. Resuscitation should include the adminis- bleeding of obscure origin. A study of 17
If the GI bleeding has occurred in a breast- tration of high-flow oxygen, insertion of two children with GI bleeding and no identifiable
feeding infant, most commonly as streaks of large-bore cannulae and titrated 20 mL kg1 source after upper endoscopy and colonos-
fresh blood in the vomitus, then it may be fluid boluses with crystalloid. Children with copy found that eight patients had Meckels
possible to distinguish if the blood is mater- significant GI bleeding require ongoing mon- diverticulum at laparoscopy, five patients
nal (ingested) rather than from the infants itoring of heart rate, blood pressure, and had other GI pathology that accounted for
gut by performing an Apt test. By diluting urine output in the ED. These observations symptoms and four patients had normal
the blood from the sample with water combined with frequent clinical assessment, findings.6 Laparotomy may be necessary
(1:5), adding 1 mL of sodium hydroxide to help guide ongoing fluid therapy. Trans- for acute abdominal emergencies such as
the centrifuged mixture, foetal haemoglobin fusion of cross-matched blood is indicated intussusception unsuccessfully reduced at
is resistant to denaturisation by alkali, as for ongoing or recurrent bleeding with air enema, midgut volvulus or for manage-
opposed to the brownyellow colour that haemodynamic instability unresponsive to ment of continuous or recurrent active GI
occurs in the presence of adult haemoglobin. initial fluid boluses. Correction of identified bleeding with haemodynamic compromise.

154
7.3 GASTRO-OESOPHAGEAL REFLUX
7

GASTROENTEROLOGY AND HEPATOLOGY

Endoscopic sclerotherapy intensive care or high dependency unit. 2. Cox K, Ament ME. Upper gastrointestinal bleeding
in children and adolescents. Paediatrics 1979;63:40813.
Injection sclerotherapy for oesophageal var- Children who present with recurrent GIT 3. Swaniker F, Soldes O, Hirschl RB. The utility of technetium-
ices in children is well established with a bleeding of unclear cause should be discussed 99m pertechnetate scintigraphy in the evaluation of
patients with Meckels diverticulum. J Pediatr Surg
reported efficacy of controlling active bleed- with a paediatric gastroenterologist regard- 1999;34:7604.
ing and eradication of varices exceeding ing the need for and timing of endoscopy. 4. Leontiadadis GI, Sreedharan A, Dorwrad S, et al.
Systematic reviews of the clinical effectiveness and cost-
90%.7 Complications can occur, including effectiveness of proton pump inhibitors in acute upper
oesophageal ulceration leading to stricture gastrointestinal bleeding. Health Technol Assess 2007;11:
iiiiiv, 1164.
formation. Variceal band ligation may be 5. Maloney J, Nowak-Wegrzyn A. Educational clinical case
limited by size in infants and small children. Controversies series for pediatric allergy and immunology: Allergic
proctocolitis, food protein-induced enterocolitis
The lack of evidence of benefit from syndrome and allergic eosinophilic gastroenteritis with
protein losing enteropathy as manifestations of non-
the use of H2 antagonists and PPIs for IgE-mediated cows mild allergy. Pediatr Allergy Immunol
Dispositon acute gastrointestinal bleeding in children 2007;18:3607.
6. Lee KH, Yeung CK, Tam YH, Ng WT, Yip KF. Laparoscopy for
has not prevented their frequent use. definitive diagnosis and treatment of gastrointestinal
The decision to admit or discharge the infant

bleeding of obscure origin in children. J Pediatr Surg
or child with GI bleeding from the ED is very There is debate about the use of 2000;35:12913.
nasogastric aspiration as a diagnostic tool to 7. Fox VL. Gastrointestinal bleeding in infancy and childhood.
much influenced by the presumptive diagno- Gastroenterol Clin North Am 2000;29:3766.
sis. Given that the majority are self-limiting establish an upper GI tract source of bleeding
or respond readily to treatment, most patients as it has low specificity for active bleeding.
can be managed on an out-patient basis.
Those infants and children suitable for dis-
charge will have no haemodynamic instability
or significant co-morbidities at presentation References Futher reading
1. Lacroix J, Nadeau D, Laberge S, et al. Frequency of upper Boyle JT. Gastrointestinal bleeding in infants and children.
to ED. High-risk patients with significant GI gastrointestinal bleeding in a paediatric intensive care Pediatr Rev 2008;29:3952. http://pedsreview
blood loss will need to be managed in an unit. Crit Care Med 1992;20:3542. .aappublications/org/cgi/content/full/29/2/3.

7.3 Gastro-oesophageal reflux

Daryl Efron

ESSENTIALS Pathophysiology
1 The infant presenting with regurgitation/vomiting needs to have alternative During periods of raised intra-abdominal
causes considered, before concluding that the diagnosis is GOR. pressure such as straining, there are a num-
ber of protective mechanisms which help
2 GOR is a normal physiological phenomenon to which infants are particularly prone. to prevent GOR. These include contraction
3 Infants with benign GOR appear healthy and thriving. If the child appears unwell, of the diaphragmatic crura around the
another cause should be considered. lower oesophagus in the region of the lower
oesophageal sphincter (LES), as well as com-
4 Infants and children with uncomplicated GOR do not need any investigations. pression of the intra-abdominal segment
5 Complications of GOR include failure to thrive, reflux oesophagitis, apnoea, of the oesophagus. Many episodes of GOR
aspiration pneumonia, and recurrent aspiration with chronic lung disease. are associated with transient relaxations
of the LES.
6 Antacids and antisecretory therapies can be helpful in reflux oesophagitis. In infants the LES is located above the dia-
phragm, rendering the above protective
mechanisms ineffective. Therefore, infants
period, usually in infancy. The refluxed are especially prone to GOR, particularly in
Introduction gastric contents are generally rapidly the early months of life. However, with ana-
Gastro-oesophageal reflux (GOR) is the cleared from the oesophagus without tomical and physiological maturation, the
regurgitation of gastric contents into causing any significant clinical symptoms. natural history is of symptom resolution by
the oesophagus. It is a normal physiological Complications warranting medical inter- late infancy in the vast majority. GOR is
phenomenon at all ages. In most cases it is vention are uncommon. When complications unusual in children older than 18 months.1
a benign self-limited condition which spon- do occur, it is referred to as GOR disease Some children are particularly predis-
taneously resolves over a variable time (GORD). posed to develop GORD. These include

155
7.3 GASTRO-OESOPHAGEAL REFLUX

premature infants, children with neurologi- presence of abdominal distension, abnormal Failure to thrive
cal conditions, such as cerebral palsy or tone, or hepatosplenomegaly suggests alter- Some infants with GOR have poor weight
neurodegenerative disorders, and children native diagnoses including anatomical gain, which may extend to failure to thrive
with anatomical anomalies such as tracheo- anomalies, neurodevelopmental dysfunction (crossing two major centiles on a growth
oesophageal fistula. These children have or metabolic disorders. A pyloric tumour chart). This may result from reduced intake
poor oesophageal motility with reduced should be specifically palpated for in young either related to dysphagia or parental
clearance of refluxed gastric contents. infants. Note that occasionally, in a young reduction of feed volumes offered, in an
infant with known GOR, a change in the attempt to minimise regurgitation or from
character of the vomiting may indicate inadequate absorption of ingested food due
History another evolving diagnosis on a background to excessive losses.
of GOR, such as pyloric stenosis or a urinary
The cardinal symptom of GOR is visible
tract infection (UTI). Serial weight measure- Respiratory
regurgitation of milk post-feeds. This is usu-
ments should be plotted on the childs A number of respiratory complications may
ally called vomiting, although in GOR the
growth chart. Respiratory examination is occur with GOR. Recurrent aspiration (often
expulsion of gastric contents is generally
important to identify signs of bronchospasm silent) results in chronic wheeze or cough,
by an effortless spill, whereas true vomiting
or recurrent aspiration, which may compli- and alveolar disease may develop, with signs
involves forceful contraction of abdominal
cate GOR. Conjunctival pallor indicates of tachypnoea and increased work of breath-
wall musculature. In GOR, the regurgitation
anaemia, which may be secondary to reflux ing. Exposure of the oesophageal mucosa to
is not particularly forceful and the milk usu-
oesophagitis. The urine should be screened acid can trigger reflex bronchospasm, and
ally soils the childs clothing. Likewise, the
by microscopy and culture for an occult persistent asthma symptoms may occasion-
physiological posseting of milk, which is
common after feeding, usually just spills
UTI causing vomiting. ally be related to GOR.2 Intermittent (or
onto the childs chin region. This is in con- even isolated) episodes of aspiration can
trast to the more projectile vomiting of pylo- cause pneumonia. Reflux of gastric contents
ric stenosis, where the milk often propels to a Differential diagnosis into the upper airway can result in laryngo-
more distant location. spasm, presenting as an obstructive apnoea
There are many causes of vomiting in chil-
The history is crucial in considering poten- or apparent life-threatening event (ALTE).3
dren, and it is important to consider a num-
tial differential diagnoses and in identifying However, it is unusual to be able to demon-
ber of alternative causes that may mimic
complications. Careful questioning regard- strate an association between GOR and
GOR (see Chapter 7.8 on Diarrhoea and
ing the relationship of vomiting to feeds, ALTE.
vomiting). These include: infection, e.g. uri-
the content of regurgitated material (e.g. nary tract infection, gastroenteritis; surgical
is there blood or bile?), apparent associated Oesophagitis
conditions, e.g. malrotation and volvulus,
distress, and feeding behaviour, is essential. Reflux oesophagitis has become a popular
pyloric stenosis, intussusception; metabolic
Episodic irritability related to feeds may indi- clinical diagnosis given to infants presenting
disorders, e.g. inborn errors, diabetes; food
cate GOR, although the association between with excessive crying behaviour (so-called
allergy and raised intracranial pressure,
irritability and GOR in infants is generally silent reflux). There are many causes for
e.g. hydrocephalus, posterior fossa tumour,
weak. Atopic features such as eczema and infant distress, (including temperamental
subdural haematoma (Table 7.3.1).
a family history raise the possibility of cows factors, food allergy) and peptic oesophagi-
milk protein allergy. A history of associated tis is only responsible for a minority of cases
fever suggests an infective cause. Seizures of infant distress.4 These infants may have
Complications episodes of blood in the vomitus or develop
and poor feeding raise the possibility of a
metabolic or neurological disorder. Inquiry GOR may result in serious complications and iron deficiency anaemia due to the red cell
about associated symptoms such as poor is then referred to as GORD. loss. Rarely, an infant may develop feed aver-
weight gain and respiratory symptoms sion due to the distress of GOR.
including apnoea, or wheezing is important Sandifer syndrome is abnormal trunk and
to screen for potential complications. Onset Table 7.3.1 Differential diagnosis neck posturing of infants in response to
of GOR oesophageal pain due to refluxed gastric
of vomiting after 6 months of age is unusual
in GOR and suggests an alternative cause. Urinary tract infection acid. These uncommon episodes can be
Gastroenteritis
quite stereotypic, such that they may be con-
fused with seizures.
Surgical: volvulus, PS, intussusception
Examination
Food allergy
Infants with benign GOR demonstrate a
Neurological Investigations
robust general condition. They appear well
and are thriving. If the child appears unwell Metabolic: inborn errors In the majority of cases, a careful history and
or lethargic another cause for the symptoms Raised intracranial pressure
examination will clarify the likely diagnosis
needs to be carefully considered. The of GOR. Investigations may be required to

156
7.3 GASTRO-OESOPHAGEAL REFLUX
7

GASTROENTEROLOGY AND HEPATOLOGY

exclude differential diagnoses, when the useful information, e.g. in conjunction In more severe cases, the pharmacological
diagnosis is unclear. with monitoring of respiratory rate, heart options include antacids, antisecretory
Serum biochemistry including an acid rate and oxygen saturations in investi- therapies, and prokinetic agents. High-dose
base, to exclude the evolving metabolic gation of apnoea or episodic hypoxaemia/ antacids can be effective in treating oeso-
hypokalaemic, hypochloraemic alkalosis of bradycardia. phagitis.9 However, prolonged use can be
pyloric stenosis, and urine microscopy and Oesophagoscopy and biopsy may be indi- associated with aluminium toxicity and is
culture to screen for a UTI, are relevant cated for evaluating the presence and sever- not recommended. H2-receptor antagonists
first-line tests in the vomiting infant. If the ity of oesophagitis, as well as to characterise such as ranitidine have been shown to
history or biochemistry is suggestive, a pylo- the changes histologically, e.g. peptic, eosin- improve both histological changes and
ric ultrasound should be performed to rule ophilic (allergic), Crohns disease, Barretts symptoms.10 Proton pump inhibitors have
out pyloric stenosis. oesophagus. well-demonstrated clinical efficacy in chil-
Well and thriving infants and children Aspiration of gastric contents into the dren with reflux oesophagitis11,12 and
with uncomplicated GOR should not be sub- lungs can be identified by nuclear medicine appear to be safe. Prokinetic agents do not
jected to any investigations. There are a study using radiolabelled milk (can detect have clear evidence for symptom reduc-
number of investigations that may be help- aspiration events up to 24 hours following tion;13 however, a trial of domperidone or
ful in selected clinical situations. However, a feed). A chest X-ray  computerised erythromycin may be worthwhile in GORD.
the interpretation of studies of oesophageal tomography scan of the chest can be used
function and their clinical relevance is not to evaluate for chronic lung disease.
straightforward. Surgical
Some children may require admission Antireflux surgery has a limited role in cases
under a paediatrician for observation of of severe GORD, such as neurologically
Treatment
feeding and the presence of regurgitation abnormal infants with recurrent aspiration
and consideration of further tests to clarify Children diagnosed with GOR need follow pneumonia. The operation of choice is usu-
the diagnosis. up organised with the local doctor, to ensure ally the Nissen fundoplication. Complica-
Barium meal and follow through is used to no alternative diagnosis is missed and to tions of the procedure include breakdown
exclude anatomical problems such as malro- monitor for complications. Parents need an of the wrap, dysphagia, bloating and gag-
tation of the small bowel. The observation of explanation of the symptoms and to be reas- ging. Some surgeons are now performing
GOR merely demonstrates that the infant sured that the natural history is for sponta- fundoplications laparoscopically.
experienced an episode of reflux at the time neous resolution over months. It is unusual
of the study. The frequency of GOR episodes, for GOR symptoms to persist beyond 12 to
their correlation with clinical symptoms, or 15 months of age. If there is any doubt Controversies
the presence of complications cannot be about the diagnosis, then a paediatric The role of oesophageal pH studies
determined from a barium study. review should be arranged. in the evaluation for reflux oesophagitis
Oesophageal pH monitoring over 24 is the subject of ongoing debate.
hours provides representative data about Simple measures
the pH at the lower oesophageal mucosa. A number of conservative measures are Antireflux medications are not
commonly used to reduce the symptoms of indicated for uncomplicated GOR, and are
A number of indices can be calculated from
GOR. These include posturing (elevation of probably being overprescribed in infancy.
the monitoring: the frequency of GOR epi-
sodes; the length of episodes; and the reflux cot 30 degrees head up), thickening of  Prokinetic agents have not been
index the proportion of time sampled that feeds,5 and changes to infant formulas. consistently demonstrated to reduce
the oesophageal pH was less than 4 (expo- None of these have been demonstrated to symptoms.
sure to gastric acid greater than 10% is usu- modify clinical outcomes.6 A trial of a par-
ally considered abnormal). However, the tially hydrolysed formula may be indicated
association of GOR with oesophagitis is in GORD, e.g. GOR with failure to thrive.
References
not strong. Infants often have abnormal Prone sleeping position is best for reducing
1. Campanozzi A, Boccia G, Pensabene L, et al. Prevalence
oesophageal pH findings in the absence of GOR7 but is not generally recommended as and natural history of gastroesophageal reflux: pediatric
it as associated with sudden infant death prospective survey. Pediatrics 2009;123:779.
histological features of oesophagitis and, 2. Balson BM, Kravitz EK, McGeady SJ. Diagnosis and
conversely, infants with oesophagitis may syndrome. Suggesting positioning a towel treatment of gastroesophageal reflux in children and
on the carers shoulder may decrease the adolescents with severe asthma. Ann Allergy Asthma
have no evidence of significant GOR on pH Immunol 1998;81:15964.
study.4 These infants presumably have an inconvenience of refluxed milk onto the par- 3. Menon AP, Schefft GL, Thach BT. Apnea associated with
ent during feeding. regurgitation in infants. J Pediatr 1985;106:6259.
alternative cause for their oesophagitis, such 4. Heine RG, Cameron DJS, Chow CW, et al. Esophagitis in
as allergy. The precise role of oesophageal distressed infants: Poor diagnostic agreement between
esophageal pH monitoring and histopathologic findings.
pH studies is contentious. It is a good test Pharmacological J Pediatr 2002;140:149.
to confirm severe GOR prior to embarking Pharmacological treatment of otherwise 5. Horvath A, Dziechciarz P, Szajewska H. The effect of
thickened-feed interventions on gastroesophageal reflux
on antireflux surgery, and there may be well, thriving infants with uncomplicated in infants: systematic review and meta-analysis of
other clinical scenarios where it provides benign GOR is not indicated.8 randomized controlled trials. Pediatrics 2008;122:e1268.

157
7.4 PYLORIC STENOSIS

6. Carroll AE, Garrison MM, Christakis DA. A systematic 9. Cucchiara S, Staniano A, Romaniello G, et al. Antacids Endoscopic healing and twenty-four hour intragastric
review of nonpharmacological and nonsurgical therapies and cimetidine treatment for gastroesophageal acidity. J Pediatr 1996;128:41521.
for gastroesophageal reflux in infants. Arch Pediatr reflux and peptic oesophagitis. Arch Dis Child 12. DeGiacomo C, Bawa P, Franceschi M, et al. Omeprazole
Adolesc Med 2002;156:10913. 1984;59:8427. for severe gastroesophageal reflux in children. J Pediatr
7. Meyers WF, Herbst JJ. Effectiveness of positioning therapy 10. Simeone D, Caria MC, Miele E, et al. Treatment of Gastroenterol Nutr 1997;24:52832.
for gastroesophageal reflux. Paediatrics 1982;69: childhood peptic oesophagitis: A double-blind placebo- 13. Augood C, MacLennan S, Gilbert R, Logan S. Cisapride
76882. controlled trial of nizatidine. J Pediatr Gastroenterol Nutr treatment for gastro-oesophageal reflux in children
8. Khoshoo V, Edell D, Thompson A, Rubin M. Are we 1997;25:515. (Cochrane Review). In: The Cochrane Library. Issue 4.
overprescribing antireflux medications for infants with 11. Kato S, Ebina K, Fujii K, et al. Effect of omeprazole in the Chichester: John Wiley & Sons; 2002.
regurgitation? Pediatrics 2007;120:946. treatment of refactory acid-related diseases in childhood:

7.4 Pyloric stenosis

Kim Lian Ong

after feeding. However, at times, it may occur

ESSENTIALS up to several hours later. The frequency of
vomiting varies: in some infants vomiting
1 Hypertrophic pyloric stenosis rarely occurs before the 1st week of life; it commonly occurs after each feed while in others it
presents at 28 weeks of age.
may be somewhat intermittent.
2 The main symptom is protracted vomiting; the infant is a hungry baby and Shortly after vomiting, the infant is often
metabolic derangements result from extensive and protracted vomiting. hungry and will take another feed immedi-
ately. The vomitus is non-bilious but due to
3 The diagnosis is made by the characteristic clinical manifestations and the finding the frequency and force of vomiting, it may
of a pathognomonic pyloric mass.
occasionally become blood tinged. The
4 Ultrasonography is the diagnostic test of choice; in the absence of amount of stool may be very small and infre-
ultrasonography, barium upper gastrointestinal study is an effective means of quent, depending on the amount of food that
diagnosis. reaches the intestine. The degree of dehydra-
tion, lethargy and metabolic derangement
5 Treatment is directed at correction of derangements before definitive surgical depends on the time interval between the
repair by Ramstedt pyloromyotomy, a straightforward procedure with minimal
onset of symptoms and presentation. In some
complications.
late presentations the infant may be severely
malnourished, dehydrated or even collapsed.
It is important to note that it may take some
cause of pyloric stenosis is unknown.
Introduction time for the clinical features, metabolic distur-
Genetic, familial, gender and ethnic origin
bance and ultrasound findings to become
Hypertrophic pyloric stenosis (HPS) is a com- can influence the incidence rates of HPS. Off-
established and one should not exclude HPS
mon gastrointestinal cause of gastric outlet spring of parents with this condition have a
if symptoms persist / recrudesce after a rela-
obstruction in infants and is one of the most higher risk of developing HPS and in many
tively normal examination of an infant pre-
common surgical conditions of infancy.1 It is series first-born males are seen more fre-
senting very early. A planned review, should
caused by the idiopathic diffuse hypertrophy quently than the other siblings.4
features evolve, is appropriate. Likewise, it is
and hyperplasia of the circular muscle fibres
important to consider HPS in a previously
of the pylorus with the proximal extension
benign posseting or refluxy baby whose
into the gastric antrum resulting in construc-
tion and obstruction of the gastric outlet. In
Clinical presentation regurgitation of milk has changed in intensity
or frequency rather than attributing this to
response to outflow obstruction and vigorous Pyloric stenosis is not present at birth. In the posseting getting worse. In this situation,
peristalsis, stomach musculature becomes early phase, there may just be regurgitation the clue to the possibility of HPS is the change
uniformly hypertrophied and dilated. or occasional non-projectile vomiting. The in the nature of the previous vomiting.
onset rarely occurs before the first week of life
and it commonly presents at 28 weeks of
age; however, it is reported in premature
Epidemiology
babies prior to this corrected age. The peak
Examination findings
Pyloric stenosis has an incidence of 2 to 4 usually occurs at 4 weeks and it is seldom
per 1000 live births in Western populations2 delayed beyond the second to third month. With established HPS, the infant fails to thrive
and it appears to be less common in infants Within a variable time after the onset of due to calorie losses, becomes dehydrated
in African and Asian populations. It is four to symptoms, the vomiting becomes more pro- due to fluid losses and appears hungry un-
five times more common in males.3 The jectile and it generally occurs during or soon less significant dehydration or alkalosis has

158
7.4 PYLORIC STENOSIS
7

GASTROENTEROLOGY AND HEPATOLOGY

started to affect the infants activity level. is close to 100% when performed by experi- the serum potassium and urinary sodium
The infant usually appears bright and enced personnel,9 having a sensitivity and concentration are elevated in adrenal insuf-
active unless significantly dehydrated, com- specificity of 99.5% and 100% respectively.10 ficiency. Recurrent emesis with alkalosis or
pared to the infant with a urinary tract infec- The sonographic appearance of doughnuts or acidosis may be caused by some inborn
tion who may be constitutionally unwell. bulls-eyes on cross-section of the pyloric errors of metabolism but usually there will
On physical examination, gastric distension channel is most characteristic. A muscle thick- be other associated clinical features, such
or visible peristaltic waves may be seen ness of the pylorus greater than 4 mm and a as hypoglycaemia, coma or seizures to sug-
moving from the left upper abdomen toward pyloric channel length of greater than 17 mm gest a metabolic cause. Any vomiting infant
the epigastrium, and right side in some yield a positive predictive value of greater needs to have a clean urine checked to
cases.5 The palpable finding of a firm, mobile than 90%. For infants less than 30 days of exclude infection.
and non-tender ovoid mass (olive) either to age, these limits may be lower.11
the right of the epigastrium or in the midline, In the absence of ultrasonography, bar-
deep to right rectus muscle and under the ium upper gastrointestinal study is an effec- Management
liver edge is diagnostic. This finding of a pal- tive means of diagnosing HPS. This study
The definitive treatment is surgical repair by
pable mass requires much patience as the may be preferred over ultrasound as the
a Ramstedt pyloromyotomy, which is the
success of such a finding is dependent on cost-effective initial imaging study when
procedure of choice in which the pyloric mass
an empty stomach and a relaxed anterior the clinical presentation is atypical for HPS
is split, leaving the mucosal layer intact.
abdominal wall in a non-crying settled infant. and favours other conditions more amena-
This procedure is fairly straightforward, with
If the stomach is significantly distended dur- ble to diagnosis by upper gastrointestinal
minimal complications. The pylorus may be
ing palpation, aspiration of gastric contents study.12 Positive findings include an elong-
accessed by various incision techniques,
using a nasogastric tube may be helpful to ated pylorus with antral indentation from
including laparoscopic means. All methods
increase the likelihood of feeling the hypertro- the hypertrophied muscle. The pathogno-
are considered acceptable practice, with
phied muscle. Also, palpation during a test monic finding is the appearance of a rail-
minimal differences in outcomes noted.13
feed may allow a previously non-palpable road track sign caused by two thin parallel
The preoperative treatment is directed
hypertrophied pylorus to be felt during peri- streams of barium traversing the pylorus.
toward correction of fluid, electrolyte and
staltic contractions. The best position for pal- There is also a vigorously peristaltic stomach
acidbase imbalance which is important to
pation is on the infants left side. The inability with delayed or no gastric emptying.
achieve prior to anaesthesia. The amount
to palpate an olive-shaped mass does not Upper GI endoscopy is performed on the
of fluid resuscitation is based on the degree
exclude the diagnosis of HPS and often an very rare occasions when other imaging mod-
of dehydration. Correction of fluid and elec-
ultrasound is needed to clarify the diagnosis.6 alities are inconclusive and although it would
trolyte imbalance can usually be achieved
With extensive and protracted vomiting, demonstrate pyloric obstruction, it would be
within 24-48 hours.
metabolic derangement will occur. Vomiting difficult to differentiate it from pyloric spasm.
Resuscitation with a bolus dose of intrave-
of gastric contents leads to depletion of sod-
nous normal saline is required for moderate
ium, potassium and hydrochloric acid, which
to severe dehydration and is given at
results in the characteristic finding of hypoka- Differential diagnosis 1020 mL kg
1. This is followed by rehy-
laemic, hypochloraemic metabolic alkalosis.7
dration with N/2 5% dextrose solution at
The kidneys conserve sodium at the expense The diagnosis is made by the characteristic
1.5 times maintenance rate over 24 hours.
of hydrogen ions, resulting in a paradoxical clinical manifestations of projectile non-
Adequate amounts of both chloride and
aciduria. With the increasing degree of dehy- bilious vomiting and the finding of a pathog-
potassium are necessary to correct metabolic
dration, renal potassium losses are accelerated nomic pyloric mass. Other causes of vomiting,
acidosis. The correction of potassium can be
in an attempt to retain sodium and fluid. especially in early life, include achalasia of
achieved by adding 1020 mEq of KCl per
the oesophagus and a symptomatic hiatus
500 mL of intravenous fluid in patients with
hernia. Gastro-oesophageal reflux with or
normal renal function. Chloride can be ade-
without hiatus hernia may have a similar
Imaging studies presentation of persistent vomiting after feed-
quately replaced by normal saline or N/2saline
with 5% dextrose. During resuscitation urine
Some clinicians believe that the palpation of ing. Other non-pathological causes include
output and electrolyte determinations should
an olive-shaped mass may obviate the need feeding technique issues and overfeeding by
be performed regularly. In general, correction
for a confirmatory imaging study, as a posi- an inexperienced enthusiastic carer.
of chloride level to 90 mEq L
1 or greater is
tive examination has high specificity.8 Plain Projectile vomiting may occur in some rare
adequate for surgery to be performed.
radiographs will often have been performed, diagnoses such as pyloric membrane or pyloric
given the history of vomiting, although they duplication where a palpable mass may be felt.
are of no diagnostic value. They may show Metabolic causes may mimic pyloric ste-
gastric distension. nosis. Adrenal insufficiency results in profuse
Complications
Ultrasonography is the now the diagnostic non-bilious vomiting but it is likely to result Pyloromyotomy is associated with a low inci-
test of choice as it can be performed quickly in metabolic acidosis rather than alkalosis as dence of morbidity and mortality. A retro-
and without radiation exposure. The accuracy in pyloric stenosis. Unlike pyloric stenosis, spective review of a large number of

159
7.5 BILIOUS VOMITING

patients from two centres between 1969 stenosis: Do the results match the claims? J Pediatr Surg
It is not easy to palpate a pyloric 1990;25:2626.
and 1994 showed an overall 19% complica- 7. Rice HE, Caty MG, Glick PL. Fluid therapy for the pediatric
mass as infants are irritable in the
tion rate.14 Therefore, complications are surgical patient. Pediatr Clin North Am 1998;45:71927.
presence of protracted vomiting. 8. Godbole P, Sprigg A, Dickson A, Lin PC. Ultrasound
minimal when the pyloromyotomy is per- compared with clinical examination in infantile
formed by experienced hands. The mortality  The threshold for investigation hypertrophic pyloric stenosis. Arch Dis Child
1996;75:3357.
associated with this procedure is less than with ultrasonography varies 9. Hernanz-Schulman M, Sells LL, Ambrosino MM, et al.
0.4% in most major centres.15 considerably. Hypertrophic pyloric stenosis in the infant without
palpable olive: accuracy of sonographic diagnosis.
Radiology 1994;193:7716.
10. White MC, Langer JC, Don S, et al. Sensitivity and cost
minimization analysis of radiology versus palpation for
References the diagnosis of hypertrophic pyloric stenosis. J Pediatr
Controversies 1. Schwartz MZ. Hypertrophic pyloric stenosis. In: JA ONeill, 11.
Surg 1998;33:9137.
Lamki N, Athey PA, Round ME, et al. Hypertrophic pyloric
Rowe MI, Grosfeld JL, et al., Pediatric surgery. St Louis,
There is disagreement as to when USA: CV Mosby; 1998. p. 1117.
stenosis in the neonate diagonostic critical revisited.
Can Assoc Radiol J 1993;44:214.
vomiting becomes significant enough to 2. To T, Wajja A, Wales PW, et al. Population demographic 12. Olson AD, Hernanadez R, Hirschi RB. The role
indicators associated with incidence of pyloric stenosis. of ultrasonography in the diagnosis of pyloric
warrant investigation as infants frequently Arch Pediatr Adolesc Med 2005;159:5205. stenosis: A decision analysis. J Pediatr Surg 1998;33:
3. Poon TS, Zhang AL, Cartmill T, Cass DT. Changing
regurgitate small amounts following a feed 676681.
patterns of diagnosis and treatment of infantile 13. Hingston G. Ramstedts pyloromyotomy what is the
especially if they have caregivers with poor hypertrophic pyloric stenosis: A clinical audit of 303 correct incision? N Z Med J 1996;109:2768.
patients. J Pediatr Surg 1996;31:16115.
feeding technique. An important 14. Hulka F, Harrison MW, Campbell TJ, et al. Complications
4. Murtagh K, Perry P, Corlett M, Fraser I. Infantile of pyloromyotomy for infantile hypertrophic pyloric
distinction may be the general appearance hypertrophic pyloric stenosis. Dig Dis 1992;10:1908. stenosis. Am J Surg 1997;173:4502.
of the infant, as infants with regurgitation 5. Spicer RD. Infantile hypertrophic pyloric stenosis: 15. O Neill JA, Grosfeld JL, Fonkalsrud EW, et al., editors.
A review. Br J Surg 1982;69:12835. Principles of Pediatric Surgery. 2nd ed St. Louis, MO:
generally appear relatively well. 6. Forman HP, Leonidas JC, Kronfield GD. A rational Mosby; 2004. p. 46779 [chapter 45].
approach to the diagnosis of hypertrophic pyloric

7.5 Bilious vomiting

Andrew J.A. Holland

ESSENTIALS Causes
1 Bilious vomiting generally indicates an intestinal obstruction due to surgical The possible causes of bilious vomiting are
pathology. as shown in Table 7.5.1 and are discussed
below:
2 Early referral to a paediatric surgeon is recommended.
3 Rarely, bile-stained vomiting may have a non-surgical medical aetiology.
Intestinal atresia
4 Malrotation with volvulus usually presents in the first few weeks of life, but can The term atresia implies maldevelopment
occur at any age and is always a surgical emergency. of a lumen or opening that is normally
patent. If there is just a narrowing of
5 Small-bowel obstruction secondary to adhesions is less common in children than in
the lumen, this is termed a stenosis. Atresia
adults and rarely settles with non-operative management.
of the intestinal tract can occur at any
level, but in the context of bilious vomiting
the level of obstruction is distal to the
inspection. For practical purposes, bilious ampulla of Vater in the second part of
Introduction vomiting indicates an intestinal obstruction. the duodenum.
Any child presenting to the emergency Some indication of the likely aetiology and As the lesion is congenital, the child will
department (ED) with bilious vomiting has a level of the obstruction may be determined present in the neonatal period, although
surgical cause of intestinal obstruction until from the age of the child, the past medical increasingly, some of these infants are diag-
proven otherwise. Bilious vomiting occurs history and length of illness prior to onset of nosed on antenatal ultrasound scans.1 Prox-
when the vomitus contains bile. Typically, this vomiting. Rapid onset of bilious vomiting imal lesions, such as duodenal or jejunal
is indicated by its bright green colour, and a non-distended abdomen suggest a atresia, will usually present in the first 24
although the discolouration may vary from a proximal obstruction, whereas late onset of hours of life whereas more distal lesions,
pale yellow to a dark greenish brown. Often bilious vomiting and abdominal distension, such as ileal or colonic atresias, can present
the carer is aware of the importance of this a distal obstruction. Immediate referral to later. Duodenal atresia may be associated
sign and will have preserved a towel or item a paediatric surgeon is indicated, as urgent with Downs syndrome and cardiac anoma-
of clothing stained with the vomitus for surgical intervention may be required. lies in up to 30% of cases.2

160
7.5 BILIOUS VOMITING
7
syndrome.7 The cardinal feature of HD is

GASTROENTEROLOGY AND HEPATOLOGY

Inflammatory
Table 7.5.1 Causes of bilious vomiting
the failure to first pass meconium within Complicated acute appendicitis, Meckels
Intestinal atresia
24 hours of birth in a term infant, with the diverticulitis or inflammatory bowel disease
Anorectal anomalies later development of bilious vomiting in leading to the development of an inflamma-
Meconium ileus
the first few days of life. tory mass may result in intestinal obstruc-
tion. This presentation usually occurs when
Hirschsprungs disease
Malrotation with volvulus there has been a diagnostic delay of the
Malrotation with volvulus The mid-gut normally develops within a primary pathology, often in children under
Irreducible inguinal hernia
physiological hernia in utero. As this reduces 5 years of age.
towards the end of the first trimester, the
Intussusception
mid-gut undergoes an anticlockwise rota- Meckels diverticulum
Inflammatory tion around the axis of the superior mesen- This represents a remnant of the omphalo-
Meckels diverticulum
teric vessels.8 Failure of this process to occur mesenteric duct and is present in about
results in malrotation. The onset of bilious 2% of the population.13 For reasons that
Adhesions
vomiting usually indicates that the malrota- are unclear, complications of a MD are more
Non-surgical ted small bowel has obstructed as a result common in males.13 MD may lead to a bowel
of twisting around its pedicle, although obstruction either as a result of acting as the
occasionally, this may occur as a result of lead point for an intussusception, due to an
Anorectal anomalies associated congenital bands. There is a high associated band adhesion with volvulus or
Typically the anus is imperforate and the risk of intestinal ischaemia, so urgent an internal hernia, or rarely an inflammatory
rectum communicates with the urinary tract surgical intervention is required once the mass due to Meckels diverticulitis.
or perineum via a fistula. It may occur as diagnosis has been confirmed.9 Although
part of the VACTERL association of anoma- malrotation most commonly presents in Adhesions
lies (vertebral, anorectal, cardiac, tracheo- the first month of life, it can present at Bowel obstruction due to adhesions is
oesophageal, renal and limb).3 The diagnosis any time and even in adult life.10 uncommon in children following abdominal
should normally be made as part of the surgery.9 This is fortunate as, unlike adults,
routine assessment of a neonate following Irreducible inguinal hernia the obstruction rarely settles with non-oper-
delivery, but is occasionally missed, leading An irreducible inguinal hernia, particularly ative intervention.
to a complete or partial distal obstruction in a male, often contains bowel. The resul-
and late onset bilious vomiting. Infrequently, tant obstruction of the bowel at the level Non-surgical
the anomaly may present outside the neona- of the external inguinal ring may produce A wide variety of medical conditions may
tal period once the child commences solids. bilious vomiting. Untreated, the irreducible occasionally present with bilious vomiting,
hernia is at risk of producing intestinal together with a variable degree of abdomi-
ischaemia. nal distension. Severe gastroenteritis with
Meconium ileus
This condition occurs in about 1020% of prolonged vomiting, sepsis, pyloric stenosis,
Intussusception hypothyroidism, meconium plug syndrome
neonates with cystic fibrosis (CF), although
In intussusception the proximal bowel (the and the cyclical vomiting syndrome may
uncommonly it may occur in the absence
intussusceptum) invaginates or telescopes all lead to a clinical picture similar to intes-
of CF.4 There may be a family history of
into the distal, receiving bowel (the intus- tinal obstruction.14 Any child presenting
CF. The condition results from the occlusion
suscipiens). Initially this involves the ileum with bilious vomiting, however, requires
of the bowel lumen by abnormally viscous
invaginating into itself, but then progresses paediatric surgical consultation to exclude
enteric secretions in the small bowel and
to involve the colon. Most commonly, it a surgical cause.
may be complicated by an atresia.
presents in infants or toddlers with episodic
colicky abdominal pain, which may be asso-
Hirschsprungs disease ciated with vomiting or pallor. As the disease
In this condition the enteric nervous system progresses, vomiting may become bilious,
Complications
is abnormal, leading to a distal physiological resulting from intestinal obstruction. The The major complication of bilious vomiting
obstruction. The condition is more common classical redcurrant jelly stool of intussus- due to intestinal obstruction is the potential
in males by a ratio of 4:1.5 In 75% of cases, ception is a late phenomenon but occult for intestinal ischaemia if the diagnosis is
so called classical Hirschsprungs disease blood can often be detected earlier.11 delayed. Even short periods of ischaemia
(HD), a variable extent of the rectum and In older children above 3 years of age, may lead to loss of the normal gut barrier
sigmoid colon is involved, leading to a distal intussusception is usually associated with a function and a prolonged paralytic ileus. If
colonic obstruction.6 In the remaining 25% pathological lead point such as a Meckels there has been diagnostic delay with irre-
of cases, bowel proximal to the sigmoid diverticulum (MD) or small bowel polyp.12 versible bowel ischaemia, perforation with
colon will also be aganglionic. About 10% It may also complicate CF and Henoch peritoneal contamination can occur. In cases
of children with HD will also have Downs Schonlein purpura. requiring extensive resection, short-bowel

161
7.5 BILIOUS VOMITING

syndrome can result. In unrecognised cases, detection of pathological lead points.15

information obtained from radiological
overwhelming septicaemia and even death Whilst malrotation may be diagnostic of
studies.
can result.9 malrotation, it cannot be relied upon to
exclude the diagnosis.16 The role of laparoscopy in the
assessment of small bowel obstruction in
Investigations children.18 There may be a role for
Treatment laparoscopy as the first manoeuvre in the
A variety of investigations may be required operative management of children with
in the child with bilious vomiting, but should The treatment of intestinal obstruction in
adhesive intestinal obstruction.
not replace a focused history, clinical exami- children involves analgesia, fluid and elec-
nation and surgical assessment. trolyte resuscitation and definitive surgical
Haematological and biochemical investi- management.
gations are often normal unless the child The child should be fasted. Neonates and References
infants require a warm environment to 1. Dalla Vecchia LK, Grosfeld JL, West KW, et al. Intestinal
is moderately to severely dehydrated. atresia and stenosis: A 25-year experience with 277
Acidbase derangement may occur second- ensure temperature stability. Often large cases. Arch Surg 1998;133:4907.
volumes of fluid have been lost from the 2. Grosfeld JL, Rescorla FJ. Duodenal atresia and stenosis:
ary to the volume and content of the vomi- Reassessment of treatment and outcome based on
tus. If there is intestinal ischaemia, there is intravascular space and 1020 mL kg1 fluid antenatal diagnosis, pathologic variance and long-term
boluses of crystalloid solution may be follow-up. World J Surg 1993;17:301309.
usually a marked leucocytosis. The blood 3. Paidas CN, Pena A. Rectum and anus. In: Oldham KT,
glucose level should always be checked required to treat shock. In addition, calcu- Colombani PM, Foglia RP, editors. Surgery of infants and
lated maintenance and deficit fluid using children: Scientific principles and practice. Philidelphia:
and the urine screened for sepsis. Lippincott-Raven Publishers; 1997. p. 132362.
The initial radiological investigations half-strength normal saline with dextrose 4. Fakhoury K, Durie PR, Levison H, Canny GJ. Meconium
should be given. A gastric tube, at least 10 ileus in the absence of cystic fibrosis. Arch Dis Child
should be directed towards demonstrating 1992;67:12041206.
obstruction and include plain films of the or 12F in size should be placed and regularly 5. Russell MB, Russell CA, Niebuhr E. An epidemiological
aspirated to decompress the stomach. study of Hirschsprungs disease and additional anomalies.
abdomen and chest. Free gas from a perfora- Acta Paediatr 1994;83:6871.
tion can be seen on an erect chest, erect Ongoing fluid losses should be charted and 6. Kleinhaus S, Boley SJ, Sheran M, et al. Hirschsprungs
replaced on a mL for mL basis with intra- disease: A survey of members of the surgical section of
abdomen or decubitus abdominal views. the American Academy of Paediatrics. J Pediatr Surg
Fluid levels consistent with intestinal venous normal saline. Hypokalaemia, if pres- 1979;14:58897.
ent, needs to be treated appropriately 7. Goldberg E. An epidemiological study of Hirschsprungs
obstruction may be seen on an erect or decu- disease. Int J Epidemiol 1984;13:47985.
bitus abdominal film, but can be normal and (see Chapter 10.5 on fluids and electrolytes). 8. Rowe MI, ONeill JA, Grosfeld JL, et al. Rotational
If required, intravenous analgesia should anomalies and volvulus. In: Rowe MI, ONeill JA,
may occasionally occur in medical causes of Grosfeld JL, Fonkalsrud EW, Coran AG, editors. Essentials
bilious vomiting. Dilated small bowel is iden- be given in the form of morphine of paediatric surgery. St Louis: Mosby; 1995. p. 492500.
tified by the presence of the valvulae conni- 100 mcg kg1 per dose and titrated to the 9. Madonna MB, Boswell WC, Arensman RM. Outcomes.
Semin Pediatr Surg 1997;6(2):10511.
ventes of Kirkring passing across the entire response of the patient. 10. Powell DM, Otherson HB, Smith CD. Malrotation of the
Most surgical causes of bilious vomiting will intestine in children: The effect of age in presentation
lumen, as opposed to the incomplete haus- and therapy. J Pediatr Surg 1989;24:77780.
tral markings in the colon. In neonates, these require operative treatment following further 11. Losek JD, Fiete RL. Intussusception and the diagnostic
markings are usually not visible. There may investigation.9 Although adhesive obstruc- value of testing stool for occult blood. Am J Emerg Med
1991;9:13.
be features to suggest intussusception (see tion may initially be treated non-operatively 12. Ong NT, Beasley SW. The leadpoint in intussusception.
by a 24 to 48 hour period of gut rest, only J Pediatr Surg 1990;25:6403.
Chapter 7.1 on Abdominal pain). 13. St-Vil D, Brandt ML, Panic S, et al. Meckels diverticulum in
The use of further investigations will be rarely is this effective in children. children: A 20-year review. J Paediatr Surg 1991;
26:128992.
directed by the likely pathology and surgical 14. Li BUK, Balint JP. Cyclic vomiting syndrome: Evolution in
consultation. Contrast studies, either upper our understanding of a brain-gut disorder. Adv Pediatr
or lower, may be both diagnostic of the sur-
Controversies 2000;47:11760.
15. Bhisitkul DM, Listernick R, Shkolnik A, et al. Clinical
gical cause and in the case of intussuscep- Radiological diagnosis of application of ultrasonography in the diagnosis of
intussusception. J Pediatr 1992;121:1826.
tion, potentially therapeutic, although now malrotation. False positives and 16. Millar AJW, Rode H, Cywes S. Malrotation and volvulus in
generally replaced by the safer air negatives may occur with both contrast infancy and childhood. Semin Pediatr Surg 2003;
12:22936.
enema.8,11 Ultrasound (US) has a limited role studies and US assessment.17 Ultimately 17. Dilley AV, Pereira J, Shi ECP, et al. The radiologist says
in the investigation of bilious vomiting, but the clinician needs to make an malrotation: Does the surgeon operate? Pediatr Surg Int
2000;16:459.
may be useful as a screening tool in children assessment based on the patients signs 18. Holcomb GW. Preface. Semin Pediatr Surg 2002;11:19.
with suspected intussusception and the and symptoms in conjunction with

162
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GASTROENTEROLOGY AND HEPATOLOGY

7.6 Ingested foreign bodies
Scott Pearson

intellectual delay has been associated with

ESSENTIALS major morbidity and mortality after foreign
body ingestion.2,6 This is often due to
1 Foreign bodies in the stomach or intestines are expected to pass spontaneously the vague symptomatology and delay in
about 99% of the time if the child has normal anatomy.
presentation.
2 Most complications occur when a foreign body is lodged in the oesophagus but a
significant number of these will pass spontaneously.

3 Children who ingest a metallic foreign body and attend an ED should have X-ray Examination
localisation of the object. For coin ingestions, an alternative is localisation with a
For the majority of children the foreign body
hand held metal detector.
will have negotiated the oesophagus and
4 Removal of disc batteries lodged in the oesophagus should occur urgently as general examination will be unremarkable.
mucosal injury can occur within a few hours. Vital signs should be recorded but once
again are unlikely to be abnormal unless
there is delayed presentation and the devel-
is the time of ingestion. The ingestion may opment of a complication.
Introduction Palpation of the abdomen will usually be
have been witnessed or may have been
Ingestion of foreign (non-food) material is reported (by an older child) or implied by normal. If the history alerts one to abdomi-
common in early childhood and often goes the childs environment at the onset of symp- nal symptoms, the examiner may find loca-
undetected whilst the child is playing and tomatology. It can be extremely useful if a lised tenderness or peritonism suggestive
may not prompt a physician visit. The exact replica of the foreign body can be easily of intestinal perforation. This is rare.
frequency of reported foreign body inges- obtained, especially in determining the type Some children will have symptoms of
tions is uncertain. The literature in this field and size of disc batteries. Determining the proximal oesophageal or pharyngeal foreign
can be divided into three areas: descriptive immediate environment of the child at the body. In the older child or adolescent where
studies of fairly large numbers of ingestion time of ingestion can assist in revealing this is due to bony ingestion (e.g. fish or
cases; studies primarily or exclusively about the possibility of any likely co-ingestants. chicken) there should be a careful examina-
coin ingestion; and studies about disc bat- The symptoms experienced by the child tion of the oral cavity and pharynx by either
teries.1 Common foreign bodies ingested since ingestion help determine the likely site indirect or direct laryngoscopy. Younger chil-
that come to medical attention include of the foreign body but this has limitations. dren are unlikely to co-operate with these
coins, bones (fish, chicken), other metallic Many children are asymptomatic at presen- procedures without sedation and an experi-
objects (pins, screws, keys, batteries), and tation, which usually (but not always) sug- enced examiner.
plastic and rubber foreign bodies. In one gests that the foreign body is lying in the
large series of 1265 reported cases of stomach or more distal part of the gastroin-
foreign-body ingestions, age ranged from testinal tract. Symptoms of vomiting, pain or
7 months to 16 years with a mean of 5.2 discomfort on swallowing, drooling, irritabil-
Investigations
years.2 Most foreign bodies pass through ity and refusal to take food or fluids may All children with a history of ingestion of
the gastrointestinal tract without complica- occur and suggest oesophageal foreign coins or batteries (or other radio-opaque for-
tions. The emergency physician should be body. Several reports note that some chil- eign bodies) should have an X-ray performed
aware of the few instances when emergent dren will be asymptomatic with foreign bod- to localise the foreign body. There is some
or semi-urgent intervention is indicated. Par- ies lodged in the oesophagus, especially the controversy on this issue in relation to the
ents need to have clear guidelines regarding distal oesophagus.3,4 Even in the context of need for X-rays in children who have ingested
the treatment plan for children who are dis- sharp fishbones, a prospective study found coins. Some authors (mainly hospital-based)
charged to outpatient follow up. that symptoms were a poor predictor of note that even previously healthy children
the presence of fishbones, except for a sharp can be asymptomatic with an oesophageal
pricking sensation on swallowing.5 Reports coin and advocate early removal of oesopha-
of abdominal pain or blood in the bowel geal coins to prevent serious sequelae.3,4
History motions should be noted. A history of previ- Other authors (notably in primary care)
In most instances, a thorough history can be ous oesophageal or other gastrointestinal believe that routine X-rays in children having
obtained from parents or caregivers before a disease is significant in determining a man- ingested a coin are unnecessary given that
requirement for intervention. The nature of agement plan and alerting one to potential asymptomatic coin ingestion is rarely, if ever,
the ingested item is obviously imperative, as complications. Significant developmental/ associated with complications in otherwise

163
7.6 INGESTED FOREIGN BODIES

healthy children.7 It has been recognised stomach has been reported to occur in stomach. Battery transit time was known
that those patients presenting to an emer- 3760% of patients.3,4 for 1366 patients. For the great majority
gency department (ED) are a selected group Glucagon has been found to be ineffec- (86.4%) the battery passed through the gas-
and would be expected to have greater tive when studied prospectively in the man- trointestinal tract in 96 hours.
severity of symptoms and higher frequency agement of oesophageal coins. Children Recommendations for ingested disc bat-
of complications.8 Hence the recommenda- should be referred to a specialist with exper- teries are:
tion that all children with a history of coin tise in paediatric endoscopy for endoscopic
ingestion who attend an ED, should have removal under appropriate sedation or
All oesophageal batteries to be removed
urgently by endoscopy.
X-ray localisation of the coin performed. An anaesthesia. This may require transfer of
alternative approach for coin ingestions is the child to another institution, as local
Asymptomatic children with
subdiaphragmatic batteries can be
the use of a handheld metal detector for resources dictate. Other techniques have
observed at home with appropriate
localisation of the coin. Several authors have been described, such as oesophageal bou-
instructions to return if symptoms
confirmed the safety of this approach follow- gienage or Foley catheter extraction, but
develop. It would seem reasonable to
ing a clear algorithm. Handheld metal detec- most emergency physicians will not manage
repeat an X-ray at 4 days and
tors are not reliable at detecting metal sufficient children with this problem to
thereafter as required to detect the
foreign bodies other than coins.9,10 If the develop expertise and appropriate safety
small group where arrested transit has
ingestion is unwitnessed and the object looks of the procedure.
occurred.
like a coin on anteroposterior X-ray, be aware
that a disc battery may have a similar
Symptomatic children with
Disc batteries subdiaphragmatic batteries should
appearance. In this situation, an additional Disc batteries are used for hearing aids, elec- have surgical consultation to consider
lateral view will reveal an asymmetry, as tronic devices and childrens toys and cause surgical or endoscopic removal of the
the two sides of the disc battery have slightly tissue destruction due to their alkaline nature battery, particularly if the battery case
different diameters. On the anteroposterior when in contact with moist membranes. Two has split open or is not progressing along
view one may also see the double ring of deaths have been reported in the literature in the gastrointestinal tract on sequential
both circumferences of the battery. situations of oesophageal batteries in chil- X-rays.1
dren with delayed presentations.12 Children
with a history of possible ingestion of a disc
battery should have an urgent X-ray per-
Other metallic foreign bodies
Treatment formed. If the battery is lodged in the oesoph-
Foreign bodies should be localised by X-ray
and removed if lodged in the oesophagus.
Coins agus, removal should be performed on an
Other objects, including pins, needles and
Coins that reach the stomach almost always emergent basis because mucosal injury can
other sharp objects can be safely observed
pass through the gastrointestinal tract with- occur in a few hours. Litovitz and Schmitz
on an outpatient basis, with intervention
out incident and further management is studied 2382 cases of battery ingestion over
occurring only if symptoms develop. In cer-
unnecessary unless symptoms arise. Coins 7 years (97% were disc batteries) which were
tain situations, where the object is lodged
that lodge in the oesophagus and have been reported to a national registry.13 There were
in the stomach, it may be appropriate to
unrecognised can cause complications such only two major adverse outcomes, both occur-
repeat an X-ray to ensure passage into the
as oesophageal perforation, mediastinitis, ring in children with oesophageal batteries.
intestine. In many situations repeat X-rays
acquired tracheo-oesophageal or aorto- There were no deaths. There were 16 children
are unnecessary unless symptoms develop.
oesophageal fistula formation and death with oesophageal disc batteries. In 88.3%
A more cautious approach is advisable when
has been reported.11 All children who are the battery passed through the gastrointesti-
there is abnormal gastrointestinal anatomy.
symptomatic should have a procedure to nal tract with no symptoms or minor symp-
remove the coin. Asymptomatic children toms only. In 8.4% there were unrelated or
with proximal or middle-oesophageal coins unknown symptoms. As few as 3.1% of Non-metallic foreign bodies
should also have the coin removed as it is patients suffered moderate symptoms Sharp foreign material such as fish or chicken
unlikely to pass spontaneously (2030% (defined as more pronounced or multiple epi- bones will usually impact in the pharynx or
in one series).3 If there is a significant delay sodes of nausea, vomiting, fever, bloody oral cavity and are considered elsewhere.
before the procedure (of more than a few stools or emesis or changes in vital signs). X-rays may assist in localisation but many
hours) the X-ray should be repeated to Forty-four patients had arrested transit foreign bodies are not radio-opaque. Inter-
ensure that spontaneous passage into the defined as identical non-oesophageal site vention is dictated by the presence or persis-
stomach has not occurred or a metal detec- on successive X-rays 48 hours apart: 17 in tence of symptoms. The vast majority of
tor should be used to check if the position stomach; two in small intestine; 16 in the other objects pass through uneventfully.
has clearly changed. Asymptomatic children colon; and in nine at unidentified sites. Plastic bread-bag tags have been associated
with distal oesophageal coins can safely be Although more patients had minor or moder- with small bowel obstruction and perfora-
observed as outpatients (depending on ate symptoms in this group, none had major tion. If there are symptoms of lodgement
social circumstances) for 1224 hours. The adverse effects. Outcome was more severe in the oesophagus the foreign body should
chance of spontaneous passage into the when there was arrested transit in the be removed by gastroscopy, otherwise an

164
7.7 HEPATIC FAILURE
7

GASTROENTEROLOGY AND HEPATOLOGY

observant approach is adequate, with the pre-school-age children can ingest. Great 4. Connors GP, Chamberlain JM, Ochsenschlager DW.
Symptoms and spontaneous passage of esophageal
parents or caregiver receiving clear advice care should be exercised in relation to disc coins. Arch Pediatr Adolesc Med 1995;149:369.
on when to return to the hospital. (button) batteries. 5. Ngan JH, Fok PJ, Lai EC, et al. A prospective study on
fishbone ingestion. Experience of 358 patients. Ann Surg
1990;211:45962.
6. Gilchrist BF, Valerie EP, Nguyen M, et al. Pearls and perils
in the management of prolonged, peculiar penetrating
Disposition Controversies esophageal foreign bodies in children. J Pediatr Surg
1977;32:142931.
Most children will be managed in the ED and Methods of removal of oesophageal coins/ 7. Connors GP, Chamberlain JM, Weiner PR. Paediatric coin
discharged. Some will be asked to return for batteries. There are some advocates of ingestion: a home based survey. Am J Emerg Med
1995;13:63840.
Foley catheter removal or oesophageal
further X-ray imaging but many will be 8. Paul RI, Christoffel KK, Binns HJ, et al. Foreign body
bougienage by emergency physicians or ingestions in children: risk of complication varies with
advised to return only if symptoms or compli- site of initial health care contact. Paediatrics
surgeons. Most centres utilise endoscopic
cations develop. Where admission is 1993;91:1217.
removal by trained endoscopists. 9. Seikel K, Primm PA, Elizondo BJ, et al. Handheld
required this should be to a facility that is metal detector localisation of ingested metallic foreign
skilled in the endoscopic and surgical man- bodies. Arch Pediatr Adolesc Med 1999;153:8537.
10. Lee JB, Ahmad S, Gale CP. Detection of coins ingested by
agement of these children. children using a handheld metal detector: a systematic
References review. Emerg Med J 2005;22:83944.
11. Byard RW, Moore L, Bourne AJ. Sudden and unexpected
1. Brown L, Dannenberg B. A literature-based approach to death: a late effect of occult intraesophageal foreign
Prevention the identification and management of paediatric foreign body. Pediatr Pathol 1990;10:83741.
bodies. Pediatr Emerg Med Rep 2002;7:19. 12. Blatnik DS, Toohill RJ, Lehman RH. Fatal complication
2. Cheng W, Tam PKH. Foreign body ingestion in children: from an alkaline battery foreign body in the esophagus.
Together with child-proofing the home to experience with 1265 cases. J Pediatr Surg Ann Otol Rhinol Laryngol 1977;86:6115.
ensure that children are protected from 1999;34:14726. 13. Litovitz T, Schmitz BF. Ingestion of cylindrical and button
3. Soprano JV, Fleisher GR, Mandl KD. The spontaneous batteries: an analysis of 2382 cases. Paediatrics
access to potential poisons, this should passage of esophageal coins in children. Arch Pediatr 1992;89:74757.
extend to protection from small items that Adolesc Med 1999;153:10736.

7.7 Hepatic failure

David Krieser

hypoalbuminaemia reflect common distur-

ESSENTIALS bances of liver function.1 ALF may be an imme-
diate life-threatening process or a subacute
1 Acute liver failure implies evidence of hepatocellular failure. Synthetic and process, with a spectrum of severity between
metabolic processes may be affected.
those extremes. Medical management is mul-
2 Acute liver failure is a rare but serious problem in the paediatric population. tifaceted and focuses on supporting vital func-
tions while hepatic recovery occurs or liver
3 Many aetiologies require consideration: infectious, toxin-mediated, congenital transplantation can be performed.
(structural or metabolic) or infiltrative (malignancy, storage disease).
ALF has been defined in adults by clinical
4 Infectious hepatitis is the most common cause worldwide, paracetamol toxicity the and laboratory criteria:
most common in the developed world.
Hepatocellular dysfunction (jaundice,
5 Clinical information is vital to target appropriate investigation and treatment. coagulopathy, etc.) of rapid onset, and
Encephalopathy within 8 weeks of
6 Management is largely supportive, and aims to maintain homeostasis until hepatic jaundice.
recovery or transplantation occurs.
The Pediatric Acute Liver Failure Study
7 Liaison with and transport to a paediatric liver transplantation centre is usually Group2 has defined ALF in children as:
required. Consultation should occur early.
Biochemical evidence of liver injury
(usually less than 8 weeks duration)
albumin, while metabolic functions include: No history of known chronic liver disease
Introduction glucose metabolism, and waste product pro-  Coagulopathy not corrected with
Acute liver failure (ALF) is a rare but devastat- cessing (e.g. bilirubin, nitrogenous com- vitamin K
ing presentation in children. The major pounds, drug elimination). ALF in children International normalised ratio (INR)
functions of the liver include synthetic and may be due to many causes (Table 7.7.1). greater than 1.5 if accompanied by
metabolic functions. Synthetic functions in- The manifestations of coagulopathy, hypo- encephalopathy or INR greater than 2 if
clude production of coagulation factors and glycaemia, jaundice, encephalopathy and not accompanied by encephalopathy.

165
7.7 HEPATIC FAILURE

Table 7.7.1 Aetiology of liver failure

Neonates and infants Older children

Cholestasis Biliary atresia Infection: viral Hepatitis AD

Choledochal cyst Enteroviruses
Intrahepatic bile duct paucity (Alagilles syndrome) EBV
Inspissated bile syndrome Varicella
Idiopathic neonatal hepatitis Adenovirus
Cystic fibrosis CMV
Endocrine Hypopituitarism Herpes simplex
Hypothyroidism Rubella
Neonatal Infection: bacterial Listeria
haemochromatosis Tuberculosis
Infection: viral CMV
Herpes simplex Infection: parasitic Toxoplasma
virus/HHV 6/VZV
EBV Toxins and drugs
Parvovirus Malignancy Leukaemic infiltration
Rubella Lymphoma
Reovirus type 3 Neuroblastomaa
Adenovirus Primary hepatic tumours
Enterovirus
Wilsons disease
Infection: bacterial Bacterial sepsis Hepatic venous occlusion
Urinary tract infection Fatty liver Obesity
Tuberculosis Pregnancy
Syphilis Hepatic hypoperfusion Cardiogenic shock
Infection: parasitic Toxoplasma Hypovolaemic shock
Metabolic disease Peroxisome function abnormality (Zellwegers) Septic shock
a1-Antitrypsin deficiency
Bile acid metabolism
Urea cycle abnormalities
Amino acid metabolism abnormalities
Lipid metabolism abnormalities (Gaucher,
Wolman, NiemannPick C)
Carbohydrate metabolism abnormalities
(galactosaemia, fructosaemia, type IV
glycogen storage disease)
Toxins Paracetamol
TPN
Hypervitaminosis A
Tumour Intra- and extra-hepatic

HHV, human herpesvirus; VZV, varicella zoster virus.

Source: Modified from DAgata ID, Balistreri WF 1999. Paediatrics in Review 20(11): 376389.

This definition has been developed Table 7.7.2 Classification of ALF

life. In broad terms, infection, immune dysre-
because the identification of encepha- gulation, toxicity (including medication),
lopathy, especially in infants and young Interval between Classification infiltration, and inborn errors of metabolism
onset of jaundice
children, can be very difficult. In addition, and encephalopathy are the causative pathways that may lead
the onset of the illness may not be to ALF. Cases where the cause is not deter-
7 days or less Hyperacute
clear, particularly in metabolic disorders. mined predominate in children under
For children with chronic liver disease who 8 to 28 days Acute 3 years.
present with features of ALF, management 5 to 12 weeks Subacute
principles are similar, although where Neonates and infants
specific therapy is available for an underly- In the neonatal population, the estimated
ing disease then this should be considered incidence of liver disease is approximately
failure, those with hyperacute liver failure
as well. 1:2500. Biliary atresia and neonatal idio-
had a better prognosis.
ALF classification, using the time interval pathic hepatitis contribute 60% of all cases
between the onset of jaundice and enceph- of cholestasis. In 80 infants under 12
alopathy, has aetiological and prognostic months with ALF, inherited metabolic condi-
importance (Table 7.7.2), despite the diffi- Aetiology
tions were responsible for 42.5% of cases:
culties in identifying encephalopathy men- Table 7.7.1 demonstrates the variety of neonatal haemochromatosis 16%, acute
tioned above. OGrady et al3,4 and Poddar diagnoses that may cause ALF in children. viral hepatitis 15%, and miscellaneous
et al5 found that, in comparison with The aetiology can be grouped according causes (toxins, autoimmune, malignancy)
patients suffering acute or subacute liver to onset prior to or after the first year of 10%. 16% of neonatal cases were

166
7.7 HEPATIC FAILURE
7
undetermined.6 Metabolic causes of liver

GASTROENTEROLOGY AND HEPATOLOGY

adolescents), other medications and nutri- acid synthesis, and abnormal fatty-acid oxi-
failure include: disorders of the mitochon- tional status.8 Treatment of toxicity is dis- dation. Multiorgan involvement (cardiac,
drial electron transport chain; disorders of cussed elsewhere in the text. pulmonary, neurological, renal) with failure
protein, carbohydrate and lipid metabolism; to thrive and hypotonia are major early fea-
and inherited causes of cholestasis. Anticonvulsants tures. Jaundice occurs in 50% of cases. Ther-
Genetic predisposition has been purported apy has not been shown to prolong life
Infectious hepatitis for anticonvulsant induced hepatotoxicity.2 although histological improvement may occur
Worldwide, infectious hepatitis is the great- Sodium valproate causes intracellular fat on liver biopsy. Biopsy demonstrates abnormal
est cause of ALF. Five RNA viruses (hepatitis accumulation within the hepatocyte, and mitochondria and absent peroxisomes. More
A, C, D, E and G) and one DNA virus (hepati- may be related to a primary defect of respi- information can be found at http://www
tis B) can infect the liver. Transmission of A ratory chain enzyme function.2 Impaired .ncbi.nlm.nih.gov/omim/214100.
and E is via the faecal-oral route. The metabolic functions within the cell may lead
remainder are transmitted via body fluids. to necrosis. Children under 2 years and those a1-Antitrypsin deficiency
Acute viral hepatitis is a clinical syndrome on multiple medications are at highest Approximately 1:4000 live births are
with systemic symptoms occurring after a risk. Carbamazepine may cause hepatitis affected by this autosomal recessive disor-
virus-dependent incubation period. Jaundice and/or cholestasis during the first months der. Cholestasis in the neonatal period, in
ensues after hepatocyte necrosis reduces of therapy. Clinically significant hepato- the context of intrauterine growth retarda-
the livers capacity to metabolise bilirubin. toxicity is rare. tion, hepatomegaly and failure to thrive is
Fulminant hepatitis occurs in less than 1% suggestive of this disease. Coagulopathy
of children with hepatitis A and in 12% Total parenteral nutrition may occur in 2% of such infants and is usu-
of cases of hepatitis B. Fulminant disease The aetiology of TPN-associated liver dis- ally responsive to vitamin K. Phenotyping of
occurs with hepatitis D in approximately ease is largely unknown. The associations protease inhibitor (Pi) by isoelectric focus
10% of cases and is more likely with super- of sepsis, surgery, and other medications, classifies children as: Pi ZZ (most commonly
infection. Hepatitis C can cause acute and which often co-exist, cannot be ignored associated with liver disease), Pi SZ, Pi SS, Pi
chronic infection and rarely fulminant hepa- as possible aetiological agents. This form FZ and MZ (carrier state). Up to 50% of chil-
titis. Severe acute hepatitis E infection is a of liver dysfunction will present in the dren with Pi ZZ will develop chronic hepatic
leading cause of ALF in the tropics. intensive-care setting rather than the failure leading to transplantation after
EpsteinBarr virus (EBV), cytomegalovirus emergency department (ED). development of cirrhosis. Bronchiectasis is
(CMV), herpes simplex virus, varicella zoster rare in children. Liver transplantation will
virus, human herpesvirus 6 and parvovirus Aspirin and Reyes syndrome lead to functional restoration and pheno-
B-19 are non-hepatotropic viruses that can Mitochondrial dysfunction leading to acute typic cure.
rarely cause ALF. Consideration of, and encephalopathy, selective hepatic dysfunc-
investigation for, these viruses is important tion and visceral fatty infiltration has been Tyrosinaemia
because specific therapy with antiviral called Reyes syndrome.9 Metabolic disorders Linked to a gene on chromosome 15, tyrosi-
medication is available for some of these have been later identified in some children naemia is an autosomal recessive condition
pathogens.7 initially diagnosed with Reyes syndrome. due to deficiency of fumarylacetoacetase
Mitochondrial oxidative phosphorylation which is the last enzyme in the processing
Toxins and medication and fatty acid b-oxidation are the metabolic of phenylalanine (pathway available at
Paracetamol pathways affected in Reyes syndrome. Pre- http://www.ncbi.nlm.nih.gov/omim/Images/
Paracetamol toxicity is the most common ceding viral infection (classically varicella), tyrosine.html). This autosomal recessive disor-
cause of ALF in the developed world. immune mediators and aspirin (or its meta- der is characterised by progressive liver
Paracetamol is an analgesic and antipyretic bolites) all can limit normal functioning of parenchymal damage and renal tubulopa-
freely available in many countries. It is these pathways. The association of aspirin thy with generalised aminoaciduria. It is
metabolised by the hepatocyte and toxicity with this disorder remains unclear despite particularly common in parts of Quebec.
exhausts hepatic glutathione stores. Gener- a study by Forsyth et al,10 which identified Rapid progression may occur in infancy or
ation of toxic metabolites leads to centri- a doseresponse relationship, and popula- an indolent course leading to hepatic cell
lobular necrosis. Toxicity is unlikely with tion studies that demonstrate that the carcinoma may occur in up to 37% of those
single doses under 150 mg kg1. Children decline in Reyes syndrome mirrors a decline over 2 years.
are often given multiple doses of para- in aspirin usage.9,11
cetamol and this can lead to toxicity if the Galactosaemia
cumulative daily dose is greater than Metabolic diseases associated Occurring in approximately 1 in 40 000 live
60 mg kg1 day1. Factors predictive of with liver failure births, and inherited in an autosomal reces-
hepatotoxicity include: age (lower incidence Zellwegers syndrome sive fashion, this deficiency of galactose-
in children under 5), genetics (cytochrome (cerebrohepatorenal syndrome) 1-phosphate uridyl-transferase leads to
isoenzyme polymorphisms are inherited), Autosomal recessive inheritance of this per- accumulation of galactose-1-phosphate in
alcohol and tobacco use (relevant in oxisomal abnormality leads to abnormal bile liver, brain and renal tubules. It is due to an

167
7.7 HEPATIC FAILURE

abnormality on chromosome 9. Clinically A choledochal cyst may present in identical Production of substances that are
these infants may present with progressive fashion and may co-exist with biliary atresia. neurotoxic; and
jaundice, hepatic failure, hypoglycaemia or Presentation is usually with prolonged neo-  Reduced elimination of neurotoxins.
cataracts. Multifactorial encephalopathy natal jaundice or delayed onset of jaundice
(hypoglycaemia, liver failure) may be present. (age 23 weeks). This occurs in approxi- Contributions from ammonia, inflammatory
These babies have an increased susceptibility mately 1:10 000 to 1:20 000 live births, cytokines, benzodiazepine-like compounds
to sepsis, especially due to Gram-negative with equal gender incidence. Following diag- and manganese16 lead to neuronal dysfunc-
organisms. This disorder is part of the routine nostic testing (ultrasonography, nuclear tion and altered interaction of astrocytes
newborn screening program and prenatal medicine scan, occasionally liver biopsy), with neurons. This leads to the clinical man-
diagnosis is available via chorionic villus sam- management is by surgical hepatoportoen- ifestations of hepatic encephalopathy. The
pling. Treatment is via dietary control. terostomy (Kasai procedure) prior to 8 weeks balance of inhibitory (e.g. GABA) versus
of age, if possible, as earlier surgery excitatory (e.g. glutamate) neurotransmis-
Wilsons disease improves outcomes.12 sion is altered in hepatic encephalopathy.9
An autosomal recessive gene defect leads Ammonia appears to augment inhibitory
to this disorder of copper metabolism that neurotransmission. In addition, the role of
Mushrooms sepsis, either via systemic immune response17
occurs in 1:30 000 live births, linked to
Edible and inedible mushrooms can be diffi- or via lipopolysaccharides directly,18 hypo-
a gene locus on chromosome 13. Symptoms
cult to distinguish. Amanita phalloides pro- glycaemia and raised intracranial pressure is
include: hepatic dysfunction (ALF, chronic
duces amatoxin, which is hepatotoxic. The important in the development of
active liver disease, insidious progression
toxin is a heat stable octapeptide. After a encephalopathy.
to cirrhosis), neuropsychiatric symptoms
period of 648 hours the affected patient
(behavioural disturbance, tremor, dysar-
will start vomiting, complain of abdominal
thria, drooling and deteriorating school
pain, develop diarrhoea, before neurological
performance) renal tubulopathy, haemolysis
and hormonal changes. KayserFleischer
symptoms (coma, seizures) and hepatic fail- Presentation
ure commence, with a mortality of up to
rings are brown bands seen at the History
30%. Cholinergic symptoms via muscarinic
corneairis border. Low serum caeruloplas- History and examination findings are signif-
receptors may also occur and respond to
min and high serum copper with high icantly influenced by the age of the child.
atropine. Charcoal should be given to reduce
urinary copper characterise the laboratory Neonates may present with jaundice and
absorption. Silibinin and high-dose penicillin
abnormalities. care must be taken to differentiate physio-
G may assist in limiting hepatic damage.13
logical jaundice or breast milk jaundice
Identification of the mushroom is important
Alagilles syndrome from pathological jaundice. The presence
and may require referral to local botanists or
This autosomal dominant condition leads to of acholic (pale) stools is characteristic of
mycologists.
intrahepatic biliary hypoplasia in associa- cholestasis. Investigation (see below) will
tion with cardiac, renal, ocular, facial and be directed at identifying the cause of chole-
skeletal abnormalities. Alagilles syndrome stasis. Jaundice in the context of dysmorphic
has an incidence of 1:100 000 live births Pathophysiology features, cardiac murmur and ocular
and is associated with abnormalities of chro- abnormalities suggest Alagilles syndrome.
Exposure to hepatotoxic agents such as:
mosome 20. Peripheral pulmonary arterial The development of jaundice after a change
drugs, products of metabolism or infectious
stenosis, with or without pulmonary valvular in diet may suggest metabolic abnormalities
particles, in addition to immune responses,
stenosis, is the most common cardiac defect. of carbohydrate metabolism such as galac-
initiates hepatocyte injury that may prog-
Vertebral anomalies described as butterfly tosaemia or hereditary fructose intolerance.
ress to necrosis. Biopsy, when performed,
vertebrae constitute the most common Infants and older children may present
reveals multilobular or bridging necrosis
skeletal defect. Retinal pigmentation and with a history of loss of appetite, vomiting,
with reticulin framework collapse. Patterns
posterior embryotoxon (requiring slit lamp fevers, or abdominal pain, prior to the devel-
related to aetiology can be seen, such as
examination) are present in the eye. The opment of jaundice. Infectious hepatitis (e.g.
centrilobular necrosis in paracetamol toxic-
facies are triangular with a broad forehead hepatitis A, EBV or CMV) is the most likely
ity or with circulatory shock. When normal
and a pointed mandible. Failure to thrive cause in this situation. EBV infection may
regenerative processes do not occur, liver
and jaundice are common presentations. be suggested by a history of sore throat
failure follows.14 Astrocyte oedema may be
Hepatic cirrhosis can develop in up to and lymphadenopathy. A dietary and travel
seen and may be due to altered cell wall per-
50% of children. history for hepatitis A may be relevant. Hep-
meability, glutamate, ammonia, and neuro-
atitis B and C infection needs to be consid-
transmitter balance.15
Biliary atresia ered if a history of exposure is obtained.
Hepatic encephalopathy occurs through
Complete absence of all extrahepatic biliary In adolescents, the use of illicit drugs and
the interplay of three factors:
structures is the usual malformation, leading sexual activity must be explored confiden-
to a clinical picture of jaundice, pale (acho- Reduction in synthesis of substances tially. In areas where strict screening is per-
lic) stools and dark urine due to cholestasis. essential for normal brain function; formed, children who receive blood product

168
7.7 HEPATIC FAILURE
7

GASTROENTEROLOGY AND HEPATOLOGY

transfusions (e.g. malignancy, renal failure, encephalopathy, which occurs late, is diffi- where they exist. Therapy needs to be
haemophilia, haemoglobinopathy) are at cult to detect. Cutaneous features such as initiated in the ED prior to transfer to a pae-
very low risk for the acquisition of hepatitis bruising, petechiae or bleeding may indicate diatric intensive-care environment. The ini-
B and C. An infant presenting with enceph- an associated coagulopathy. In children with tial management issues are listed in
alopathy and jaundice may have a meta- chronic liver disease there may be signs such Table 7.7.4. Intubation and ventilation
bolic disease (fatty acid oxidation, as spider naevi, caput medusae or finger may be required due to coma or respiratory
carbohydrate metabolism) and the possibil- clubbing. failure. Respiratory failure itself is multifac-
ity of consanguinity needs exploration. Pre- torial: altered cardiac output; capillary leak;
vious surgery for a choledochal cyst or for possible oliguria; and significant fluid
biliary atresia is important in the context requirements contribute. Fluid management
of a child presenting with jaundice and/or
Investigations may be complex in the face of renal failure
hepatic failure. Exposure to hepatotoxins Investigation of the child with hepatic fail- and electrolyte imbalances. Vitamin K, fresh
such as paracetamol, anticonvulsants, aspi- ure focuses on identifying the extent of liver frozen plasma or cryoprecipitate may be
rin or mushrooms must be identified, as spe- dysfunction and on identifying the cause. required to correct symptomatic coagulopa-
cific treatments can be implemented. History and examination findings must thy. Intravenous fluids containing 10% dex-
Pruritus with jaundice, dark urine and pale guide investigation. ED screen should trose or more are usually required to correct
stools may be the presenting features of include a full blood examination, liver func- hypoglycaemia and then maintain normo-
cholestasis in an older child. tion tests including conjugated and unconju- glycaemia. Lactulose is given in hepatic
gated bilirubin, blood glucose, coagulation encephalopathy to reduce absorption of
Examination screen, renal function and arterial blood nitrogenous wastes. Neomycin and/or met-
Hepatomegaly and jaundice are the most gases. Further potentially relevant investiga- ronidazole, given enterally, reduce the
frequent findings. The character of the liver tions such as viral serology, copper and caer- enteric bacterial load and the production
edge may offer additional information: for uloplasmin levels, Pi typing, lactate level, of nitrogenous wastes. Proton pump inhibi-
example, a firm, nodular surface suggesting drug screens, urine metabolic screen, or tors, H2-receptor blockers and/or sucralfate
cirrhosis or fibrosis; tenderness suggesting imaging should be determined after discus- are given to limit the risk of gastric ulcera-
acute hepatitis. Note that in cirrhosis liver sion with a paediatric hepatologist. Note tion in the context of coagulopathy. Sepsis
span may be reduced. Splenomegaly sug- that the international normalised ratio for is a common and serious complication, and
gests portal hypertension or infiltration coagulation and the serum bilirubin concen- will exacerbate liver failure and requires
(e.g. storage disease, extramedullary haema- tration are not predictive of post-transplant aggressive antimicrobial management.
topoiesis, malignancy). Abdominal examina- survival, while renal dysfunction requiring Raised intracranial pressure and, more spe-
tion will also identify ascites if present. dialysis is associated with a higher mortal- cifically, reduced cerebral perfusion pressure
As discussed above, specific diseases asso- ity.14 Escudie et al19 found a prothrombin are important complications of ALF. Both
ciated with liver failure will have other fea- index below 10% (INR approx 6) 4 days after these effects may be improved by therapeu-
tures. The phenotypes of galactosaemia, Amanita phalloides ingestion was predictive tic cooling. Therapeutic cooling (3233 C)
Alagilles syndrome, a1-antitrypsin defi- of fatal outcome. reduces brain energy metabolism, nor-
ciency and Wilsons disease may be identi- malises cerebral blood flow, reduces ammo-
fied by careful clinical examination. nia delivery, reduces oxidative pressure on
Neurological evaluation is essential and astrocytes and reduces brain glutamate.14
Management A bridge to liver transplantation may be
the presence of asterixis important in a child
with liver failure in order to assess for, and The management of acute liver failure created via the use of liver support devices.
classify a stage of, hepatic encephalopathy, involves supportive care, complication man- These have not undergone extensive testing
if present (Table 7.7.3). In young children agement and specific treatment modalities as yet. Artificial systems using filtration,

Table 7.7.3 Stages of hepatic encephalopathy

I II III IV

IV a IV b

Symptoms Lethargy, euphoria, Drowsiness, erratic behaviour, Stuporous but rousable, Responsive to pain No response
poor concentration disorientation incoherent speech

Signs Reduced cognitive performance Asterixis, incontinence, Asterixis, hyper-reflexia, No asterixis, areflexia, flaccidity
(drawing figures, memory) fetor hepaticus rigidity

EEG Normal Generalised slowing, Markedly abnormal, Markedly abnormal bilateral slowing,
theta waves triphasic waves delta waves, cortical silence

Source: Modified from Suchy FJ 2000. Fulminant hepatic failure. In Behrman RE (ed). Nelson Textbook of Pediatrics, 16 edn.

169
7.7 HEPATIC FAILURE

Table 7.7.4 Initial management tasks Table 7.7.5 The Kings College criteria

Management tasks Features associated with a

Observation of: Vital signs poor prognosis
Pulse oximetry Unknown aetiology
Level of consciousness Toxin associated (other than paracetamol)
Urine output (may need indwelling catheter) Age under 10 years
Age over 40 years
Monitoring of: ECG Jaundice for more than 1 week prior to
Blood glucose encephalopathy
Acid/base status (blood gases) Serum bilirubin over 300 mmol L1
Coagulation INR over 3.5
Liver function tests pH less than 7.3
Serum electrolytes Serum creatinine over 300 mmol L1

Insertion of: Vascular access (peripheral, central, intraosseous) Source: Data from OGrady et al 1989.
Nasogastric tube (for gastric drainage and administration of Gastroenterology 97: 439445.
neomycin and lactulose)

Supplementation of: Glucose (Table 7.7.5), based on multivariate analysis

Potassium
Albumin of 588 patients with fulminant hepatic fail-
Coagulation factors (vitamin K, frozen plasma, cryoprecipitate, platelets) ure, provide clinical and laboratory para-
Oxygen (may require endotracheal intubation)
Intravascular volume if required meters predictive of mortality. A single
adverse factor was associated with 80%
Identification of: Treatable cause if present (toxic, infectious, metabolic)
Evolving encephalopathy mortality; three adverse factors were asso-
Raised intracranial pressure ciated with 95% mortality. Paracetamol tox-
icity was associated with a better prognosis.
If paracetamol toxicity was the reason for
ALF then the presence of a single adverse
risk factor places mortality risk at 55%. It
dialysis or ion exchange or bioartificial sys- transplantation. Certain metabolic diseases
is important to compare these figures with
tems (human or non-human hepatocytes can be managed through dietary manipula-
the 2030% mortality rate of all children
within an artificial framework) are available. tion or metabolic pathway manipulation.
undergoing liver transplantation.
Anecdotal reports of these systems include
The United Network for Organ Sharing
reduction in serum copper in Wilsons
(UNOS) has developed the Model for End-
disease-induced ALF that allowed stabilisa-
tion prior to transplantation.20
Disposition Stage Liver Disease (MELD) for patients over
12 years. The Pediatric End-Stage Liver Dis-
The development of liver transplantation Management of the child with ALF requires
ease score is for patients under 12. Both
has allowed children with irreversible liver a multidisciplinary team of physicians,
assess risks of death while waiting for trans-
failure to survive. The paucity of available nursing staff and allied health personnel
plantation in order to prioritise available
donors and contraindications to trans- in an intensive-care environment. The inva-
organs. The PELD score uses: albumin, biliru-
plantation limit the number of children able sive nature of monitoring and maintaining
bin, INR, growth failure and age at listing
to receive liver transplants in the acute such patients will necessitate transport to
(for transplantation) while the MELD score
setting. Contraindications to transplanta- a paediatric intensive-care unit. Transporta-
uses creatinine, bilirubin and INR in predic-
tion include: uncontrolled systemic infection; tion will require stabilisation and continued
tive modelling (www.unos.org).
extrahepatic metastasis in liver tumours, monitoring of the above parameters,
irreversible neurological injury; and multior- with intervention occurring en route if nec-
gan failure. Living donor transplantation has essary. Liver transplantation will require
increased the number of liver transplants in
Prevention
the involvement of surgeons and anaesthe-
children. Auxiliary partial orthotopic trans- tists in addition to those already involved Viral hepatitis requires public health and leg-
plantation allows the transplanted liver seg- within the paediatric intensive care unit. islative intervention to be controlled. Immuni-
ment to function while a diseased native The transplant service should be consulted sation against hepatitis A and B, improved
liver recovers and regenerates. This tech- early in such cases. hygiene, reduced overcrowding and the pro-
nique also may allow for the discontinuity motion of harm-minimisation through safe
of antirejection medication once the native sex and reduction in the sharing of needles
liver recovers.21 Disease-specific treatment among intravenous drug users may have
is available for paracetamol toxicity. Intrave-
Prognosis
impacts on the incidence of viral hepatitis.
nous N-acetylcysteine (NAC) is given in addi- The mortality of ALF is approximately 60%. Vaccines for hepatitis A and B are available
tion to supportive measures. Herpesvirus- Of children with Stage 4 hepatic encepha- but expense and distribution problems act
induced fulminant hepatitis may respond lopathy, 80% will die. Sepsis is the cause as barriers in the developing world.
to aciclovir. Children with Wilsons disease of death in approximately 10% of children The widespread availability of paraceta-
may respond to chelation therapy pending with ALF. The Kings College criteria mol contributes to its position as the leading

170
7.7 HEPATIC FAILURE
7

GASTROENTEROLOGY AND HEPATOLOGY

cause of ALF in the developed world. Limit- 8. Larsen AM. Acetaminophen hepatotoxicity. Clin Liver Dis
paracetamol hepatotoxicity, arterial pH, 2007;11(3):52548.
ing packet size, child-proofed containers 9. Glasgow J, Middleton B. Reye syndrome - insights
serum lactate and prothrombin time and
and even reducing distribution to pharma- on causation and prognosis. Arch Dis Child
creatinine; in mushroom toxicity, 2001;85:3513.
cies alone may reduce the incidence of para- 10. Forsyth BW, Horwitz RI, Acampora D, et al. New
prothrombin time and creatinine appear
cetamol poisoning. epidemiologic evidence confirming that bias does not
more predictive. A patient with Wilsons explain the aspirin/Reyes syndrome association. JAMA
Screening of embryos or parents after the 1989;261(17):251724.
disease requires transplantation if
identification of a metabolic disease will 11. Belay E, Bresee J, Holman R, et al. Reyes syndrome in the
encephalopathy is present, although United States from 1981 through 1997. N Engl J Med
assist in reducing the incidence of such dis- 1999;340(18):137782.
serum bilirubin, INR, aspartate
eases, if parents consider termination of 12. Serinet M, Wildhaber B, Broue P, et al. Impact of age at
aminotransferase and white cell count Kasai operation on its results in late childhood and
pregnancy an option. Genetic counselling adolescence: a rational basis for biliary atresia screening.
are important predictors of the need for
is mandatory in such cases. Direct gene ther- Pediatrics 2009;123(5):12806.
transplantation. In general, predictors of 13. Schilsky ML, Honiden S, Arnott L, Emre S. ICU
apy for such diseases remains elusive. management of acute liver failure. Clin Chest Med
poor outcome include: poor renal 2009;30(1):7187.
function, a PELD score >25, and age 14. Suchy FJ. Fulminant hepatic failure. In: Kliegman R,
Rudolf M, editors. Nelson Textbook of Pediatrics. 18th ed.
under 24 months and onset of Saunders; 2007.
Controversies and future encephalopathy within 7 days of onset of 15. Sundaram V, Shaikh O. Hepatic encephalopathy:
directions jaundice.4 pathophysiology and emerging therapies. Med Clin North
Am 2009;93:81936.
Transplantation remains the gold 16. Munoz SJ. Hepatic encephalopathy. Med Clin North Am
2008;92(8):795812.
standard therapy and case reports of 17. Shawcross DL, Davies NA, Williams R. Systemic
inflammatory response exacerbates the
auxiliary partial orthotopic liver References neuropsychological effects of induced hyperammonemia
transplantation demonstrated that a 1. DAgata I, Balistreri W. Evaluation of liver disease in cirrhosis. J Hepatol 2004;40:24754.
in the pediatric patient. Pediatr Rev 1999;20 18. Pedersen HR, Ring-Larsen H, Olsen NV.
transplanted liver lobe functioned (11):37689. Hyperammonemia acts synergistically with
satisfactorily until native liver recovery 2. Bucuvalas J, Yazigi N, Squires RH. Acute liver failure lipopolysaccharide in inducing changes in cerebral
in children. Clin Liver Dis 2006;10(1):14968. hemodynamics in rats anaesthetised with pentobarbital.
occurred in a case of mushroom 3. OGrady J, Schalm S, Williams R. Acute liver failure: J Hepatol 2007;47:24552.
toxicity.22 redefining the syndromes. Lancet 1993;342 19. Escudie L, Francoz C, Vinel J-P, et al. Amanita phalloides
(8866):2735. poisoning: Reasessment of prognostic factors and
Cerebral cooling for management of 4. OGrady J. Modern management of acute liver failure. Clin indications for emergency liver transplantation. J Hepatol
2007;46(3): 46673.
Liver Dis 2007;11(2):291303.
raised intracranial pressure associated 5. Poddar U, Thapa B, Prasad A, et al. Natural history and 20. Sen S, Felldin M, Steiner C, et al. Albumin dialysis
with ALF may offer some benefits and risk factors in fulminant hepatic failure. Arch Dis Child and Molecular Adsorbents Recirculating System (MARS)
2002;87(1):546. for acute Wilsons disease. Liver Transpl 2002;8
requires more research. 6. Durand P, Debray D, Mandel R, et al. Acute liver failure in (10):9627.
infancy: a 14 year experience of a pediatric liver 21. Kerkar N, Emre S. Issues unique to pediatric liver
 Prognostic scores have been transplantation center. J Pediatr 2001;139(6):8716. transplantation. Clin Liver Dis 2007;11(2):32335.
7. Fontana R. Acute liver failure including 22. Rosenthal P, Roberts J, Ascher N, Emond J. Auxiliary
developed according to aetiology of ALF acetaminophen overdose. Med Clin North Am liver transplant in fulminant failure. Paediatrics 1997;
and need more prospective research: in 2008;92(4):76194. 100(2):E10.

171
 7.8 Diarrhoea and vomiting
Christopher Webber

condition, it may be important to clarify

ESSENTIALS whether there have been infectious contacts
or recent overseas travel. Review of the past
1 ABC fluids in and out is a useful tool in taking a history. history should include any significant medi-
2 Not all diarrhoea and vomiting is gastroenteritis. cal or surgical problems and whether the
child is thriving.
3 Vomiting may be a non-specific symptom of a more serious medical problem.
4 The differential diagnoses are extensive. ABC fluids in and out
5 Young infants with diarrhoea must be managed with caution. This schema provides a simple structure for
obtaining the history, and may be used to
advise parents about concerning symptoms
Fever is present when the bodys temper- that may develop subsequently. Alertness
Introduction and activity (A) provides information about
ature is elevated above normal. Actual defi-
Common symptoms nitions of fever vary, but a core temperature the neurological state of the child. Lethargy
Vomiting and diarrhoea are common symp- of about 36.537.2
C is normal. The site and poor interactivity are concerning symp-
toms that affect infants and children. These used to make the measurement also affects toms in a young child. The behaviour of the
symptoms may occur separately or in combi- measured temperature. Generally aural baby whilst feeding is also important. Par-
nation. Clinicians must exercise caution (tympanic) temperatures are less accurate. ents may note poor suck and sleepiness dur-
when evaluating infants presenting with Per-axilla temperatures are lower than ing feeding; and the breastfeeding mother
fever and vomiting who do not have diar- rectal temperatures by about 0.51
C. may comment more precisely about the
rhoea, as the list of differential diagnoses Although rectal temperatures more closely quality of the infants suck. Breathing (B),
is extensive, including some conditions with reflect the bodys core temperature, per- specifically rapid or laboured breathing,
significant morbidity and mortality. axilla temperatures are more convenient and poor circulation (C), as indicated by mot-
to measure and socially acceptable to the tling and coolness of the peripheries, are
patient. Needless to say, an infant with car- also worrying signs.
Frequency of presentation The childs fluid intake and urine output as
By far the commonest condition causing diovascular compromise and peripherally
shutdown should have a rectal temperature a percentage of usual or normal provide very
vomiting and diarrhoea in children is gastro-
checked, as a per-axilla temperature may important markers of the unwell infant or
enteritis. A urinary-tract infection (UTI) must
not accurately reflect core temperature child. The number and wetness of the
be considered in all infants with vomiting,
and falsely reassure the physician. nappies is used as the indicator of urine out-
with or without fever. The presence of diar-
put. Thus, using an evaluation of fluids in and
rhoea makes a urine infection much less
out, one should be concerned when these are
likely, and in this situation the diagnosis is
less than 50% of normal for the child.
more likely to be gastroenteritis.
Clinical evaluation
History
Definitions Presenting complaint Examination
and past history
General observation
Vomiting is an active process involving Always listen to the parents or carers of the
In the absence of a life-threatening emer-
muscular contraction expelling the stomach child. Generally, they know the infant or
gency it is always worthwhile to make a
contents orally. In comparison, gastro- child best and physicians are unwise to
careful observation of the child, either whilst
oesophageal reflux is the passive regurgitation ignore their concerns. Explore the symptoms
talking with the carer or to the child. This
of gastric contents, most commonly liquid. further and in more detail, if necessary. Is
general observation phase is invaluable in
In the newborn and young infant gastro- there blood in the vomitus, or blood or
the paediatric assessment, particularly in
oesophageal reflux is common and may be mucus in the stool? Sometimes important
young children. During this observation look
considered normal if it is not associated with associated symptoms are not volunteered
and listen for the following:
consequences like pain or failure to thrive. by parents, and are only alluded to when
Diarrhoea refers to the frequency and con- directed by a focused history. Specific infor- Airway noises (stridor, sturtor, grunt or
sistency of stool being frequent, loose or liq- mation regarding the nature and content wheeze).
uid. The stool may also contain blood and of the vomiting and diarrhoea needs clarifi- Tachypnoea (measure the respiratory
mucous, both of which are abnormal. cation. In the child with a possible infectious rate).

172
7.8 DIARRHOEA AND VOMITING
7


GASTROENTEROLOGY AND HEPATOLOGY

Colour (centrally and peripherally, look chest) and peripheral capillary refill, with infant who is vomiting may have meningitis.
for cyanosis and mottling). other data like heart rate, pulse volume Likewise, raised intracranial pressure (ICP)
Alertness and activity (or inter-activity, and consciousness state, allows an assess- may cause vomiting. Cushings triad includes
particularly with childs carer[s]). ment of the adequacy of the circulation. bradycardia, hypertension and abnormal
The presence of shock indicates inadequate respiration, but is generally associated with
Note that the ABCD approach (airway, tissue perfusion. It is present when there is a a marked elevation of the ICP. On neurologi-
breathing, circulation, disability) is utilised. rapid, thready pulse, delayed capillary refill, cal examination, check for any asymmetry of
Assessment of the state of hydration is especially if central (>2 seconds), and movement, tone and reflexes, as a cerebral
critical. It is well recognised that clinicians abnormal neurological status including agi- abscess may cause fever and vomiting. Focal
tend to overestimate the degree of dehydra- tation, lethargy, or coma. The diagnosis of seizures generally indicate focal pathology
tion, consequently excessive amounts of shock is not reliant on the presence of hypo- and should be considered the same as a
intravenous fluid may be administered. tension, particularly in children. Delay in focal abnormality on examination.
Accurate premorbid and current weight identifying shock until the child is hypoten-
may aid this assessment. The following is sive risks severe compromise and potential Temperature
a revised guide for the assessment of progress to cardiac arrest. Always measure the temperature of the
dehydration: Septicaemia, with or without meningitis, vomiting child. If the childs circulation is
< 3% no clinical signs; reduced urine may cause fever, vomiting and diarrhoea. compromised then consider continually mon-
output; Some bacterial pathogens that cause gas- itoring the temperature with a rectal probe.
3% mild dehydration; mild tachycardia, troenteritis may also cause septicaemia, The bodys temperature varies over time,
dry mucous membranes; such as Salmonella and Shigella. and in the unwell patient it is crucial to con-
5% moderate dehydration; lethargy, Respiratory examination of the infant tinue to monitor for fever over a period of
tachycardia, reduced skin turgor, sunken with fever, cough and vomiting may identify time. Remember that some modes of mea-
eyes/fontanelle; tachypnoea and grunt that alert the surement underestimate the core tempera-
clinician to the possibility of pneumonia. ture or are an inaccurate reflection of the
10% severe dehydration; clinical signs
of shock (tachycardia, thready pulses, Grunting is an expiratory noise, usually core temperature (see above). The child with
reduced perfusion, particularly centrally). intermittent, and generates PEEP (positive overwhelming sepsis may not mount a fever
end expiratory pressure) by partially closing and, indeed, may be hypothermic.
Always check carefully for a rash, as pete- the glottis during expiration. Percussion
chiae and purpura may be inconspicuous or note for dullness may be more valuable
subtle. Examination of the ears, nose and than auscultation for crackles or bronchial Differential diagnoses
throat, is frequently left until the end of breathing. Be aware of the child with
the examination, to avoid potential distress This list is extensive and varies depending
abdominal pain and grunt as they may have
impairing the remainder of the examination. upon the combination of symptoms, vomit-
a lower lobe pneumonia.
ing and/or fever and/or diarrhoea, as well
as the examination findings:
Cardiovascular and respiratory Abdominal examination
status The abdomen is examined for distension, gastro-oesophageal reflux;
It is important to evaluate the cardiovascu- tenderness and guarding. Take note of the viral illness;
lar and respiratory systems in the infant with childs resting posture and how they move gastritis/enteritis;
vomiting for the following reasons: spontaneously or on request, both in the non-specific, generalised viral illnesses;
bed or walking. A gentle, unrushed examina- gastroenteritis viral, bacterial;
shock may be present due to either
septicaemia or fluid loss from vomiting
tion provides more information. otitis media/URTI/tonsillitis/
Presence of guarding and rebound can be pharyngitis/stomatitis;
and/or diarrhoea;
elicited with gentle flexion/relaxation of urinary tract infection (UTI);
septicaemia may be the cause of the
symptoms;
the fingers of the examining hand (at the septicaemia;
metacarpophalangeal joint). Evaluate for meningitis/encephalitis;
pneumonia or other respiratory infections
may cause vomiting;
organomegaly liver, spleen and kidneys. pneumonia;
Are there any masses? The pale, vomiting appendicitis;
effortless tachypnoea may indicate a
metabolic acidosis.
child may have intussusception. Examine metabolic/endocrine problems;
for anal fissures and other perianal abnor- intestinal obstruction including
The pulse rate and pulse volume may iden- mality that may indicate inflammatory malrotation ( volvulus);
tify a rapid thready pulse indicating poor bowel disease. cyclical vomiting;
perfusion. Capillary refill is often thought malabsorption including cows milk
to be a poor indicator of circulatory status, Neurological examination protein enteropathy;
as the peripheral perfusion (hands and feet) The child may have impaired consciousness colitis (including Crohns disease,
may be affected by environmental tempera- either due to systemic illness, shock or a pri- ulcerative colitis, pseudomembranous
ture. The comparison of central (anterior mary neurological problem. The unwell colitis).

173
7.8 DIARRHOEA AND VOMITING

The child with recurrent vomiting warrants glucose. This information can be invaluable In a young, breastfed infant with blood in
particular attention, with consideration in assessing the child with diarrhoea and vomitus or stool it is important to distinguish
of diagnoses like malrotation, UTIs and vomiting. In this context, check the specific swallowed maternal blood from infants
cyclical vomiting. An abdominal X-ray may gravity and for ketones. Glycosuria may blood. An Apt test distinguishes between
be helpful, but after surgical consultation indicate diabetic ketoacidosis. If the UA is the two due to presence of HbF. To perform
an upper gastrointestinal contrast study positive for nitrites or leucocytes, then an the test, add the bloody stool/vomitus to a
may be required. A child thought to have appropriately collected urine specimen test tube with about 5 mL of tap water. This
cyclical vomiting should be referred to a should be cultured. The specimen to be cul- lyses the red blood cells. After allowing to
paediatrician for follow up. Beware of tured should be collected attempting to mini- settle, the supernatant should be pink to
children with a pre-existing condition (or mise contamination, even if re-collection is continue. If so, add 1 mL of 1% sodium
label) such as cyclical vomiting as they required. hydroxide. Remaining pink indicates infants
may develop other acute conditions. These Generally urine culture is unnecessary blood (HbF) whereas changing to a yellow-
children require careful reconsideration at where acute gastroenteritis is likely (i.e. brown colour over 2 minutes indicates
each presentation before concluding another when there is diarrhoea) but is mandatory maternal (adult) blood.
vomiting episode. in the infant or baby with acute vomiting The microscopy of the diarrhoeal stool
and fever. Urinary symptoms like dysuria may be important. The presence of stool
and frequency can be expected in children white cells, without a viral or bacterial path-
Investigations from about 4 or 5 years of age, allowing a ogen, in the young infant, may indicate
more selective approach to those who cows milk protein intolerance.
Remember, only order tests that aid the deci-
require urine culture. Bag urines (collected
sion making and management of the
in an adhesive, sterile plastic bag) have a
patient. Reference ranges are frequently
high contamination rate and are best
based on the standard deviation in a popu-
avoided when performing a urine culture, Management
lation, so a result outside the range may not
but are satisfactory for a simple UA.
necessarily indicate abnormality or pathol- Intravenous fluids
Although more difficult to collect, a clean
ogy. This may inadvertently lead the clini-
catch urine provides a sample less likely to
Bolus.
cian to order more unnecessary tests.
be contaminated. This is collected with an
Rehydration.
Not every child who is medically assessed
open nappy, a poised parent, with an open
Glucose.
requires investigations. The tests required
sterile container, waiting patiently to catch The management will depend on the need
depend on the possible differential diag-
the spontaneously voided urine. Those for immediate resuscitation and subse-
noses and the severity of illness
babies and infants deemed to be at higher quently, the provisional diagnosis. A fluid
(Table 7.8.1). Dont forget simple bedside
risk of a UTI or who are more seriously bolus is used to restore circulating volume
tests like urine analysis (UA) and blood
unwell may require more rapid testing by and is therefore warranted when signs of
either a suprapubic aspirate (bladder tap) shock are present. This must not be confused
or an inout catheter urine collection. Any with strategies of rapid rehydration in dehy-
child who is toilet trained and requires a drated children. Intravenous fluids contain-
Table 7.8.1 Tests that may be useful in
the child with vomiting or diarrhoea urine culture should have mid-stream urine ing dextrose must not be used as volume
collected. expanders. The choice of crystalloid fluid
Common tests:
Urine analysis The child who is previously well and for a bolus is either normal saline (0.9%)
Urine culture thriving who presents with vomiting and or Hartmanns solution. If these crystalloids
Stool
Microscopy, culture hypoglycaemia, if ketotic, is likely to be are not available, and a bolus is required,
Rotavirus antigen hypoglycaemic from starvation. In the then a colloid should be used (e.g. 4%
Giardia (often tested by RIA, avoiding the
need for multiple samples for ova and absence of ketonuria, metabolic and endo- NSA or Gelofusine).
parasites) crine abnormalities need to be considered. In dehydrated, but not cardiovascular
Biochemistry electrolytes, urea/creatinine,
blood glucose It may be necessary to assay growth hor- compromised children, it is safer to com-
Full blood count mone, insulin and cortisol, as well as mence initial intravenous rehydration with
Less common tests: performing a urine metabolic screen. Col- fluids that contain at least 75 mmol L1
Liver function tests lecting appropriate specimens when hypo- sodium, i.e. 0.450.9% NaCl solution.
Blood gas (venous or arterial)
Blood culture glycaemic is important. Blood samples Commonly available fluids include 0.45%
Amylase collected in a lithium heparin tube (plasma) NaCl 5% glucose, 0.9% NaCl (normal
Insulin, growth hormone, cortisol (if
hypoglycaemic) and plain tube (serum) should allow blood saline) and Hartmanns solution. Normal
Lactate glucose to be measured concurrently with saline (0.9% NaCl) with 5% glucose is also
Urine metabolic screen
CXR to exclude pneumonia hormone levels. Other samples may also currently available. Hypoglycaemia is not
AXR to exclude intestinal obstruction be needed. Consultation with a paediatri- infrequent, both at presentation or subse-
AXR, abdominal X-ray; CXR, chest X-ray; RIA,
cian or paediatric endocrinologist may be quently, thus dextrose-containing fluids are
radioimmunoassay. prudent. appropriate in all children, particularly in

174
7.8 DIARRHOEA AND VOMITING
7

GASTROENTEROLOGY AND HEPATOLOGY

the young infant or those who have had the severely unwell, febrile patient broad- clinical data. Dont be distracted by a childs
insufficient caloric intake and are ketotic. spectrum antibiotics should not be delayed previous diagnoses or labels.
Intravenous fluids are commonly manufac- whilst awaiting collection of all cultures. It
tured as isotonic with plasma, by varying would be preferable, but not essential, that General approach
the glucose concentration to balance the a blood culture be undertaken prior to such ABC fluids in and out is a useful tool in tak-
sodium chloride (e.g. 0.45% NaCl 5% glu- administration. ing a history. Caution must be exercised in
cose). The addition of dextrose to many com- the infant who has fever and vomiting, as dif-
mercially available solutions (such as normal ferential diagnoses include serious illnesses,
Consultation
saline) makes them hypertonic compared to like meningitis. The younger the infant, the
Consultation with more experienced staff,
plasma. However, due to rapid equilibration more challenging the evaluation and the less
either emergency physicians or paediatri-
of the dextrose with extravascular space this reliable the clinical examination. If in doubt,
cians should be encouraged, especially for
is not clinically significant. In this context, seek consultation from a colleague. The tele-
complex or severely ill infants or children.
0.9% NaCl 5% glucose is an appropriate phone is a useful and powerful tool for clin-
Assistance may be required for clinical eval-
intravenous fluid to use (may be made by icians and the value of consultation should
uation and technical procedures in critically
adding 50 mL of 50% glucose to 450 mL not be underestimated.
ill children.
of 0.9% NaCl).
Be aware of the potential of SIADH in
unwell children. The rate of fluid administra- Transfer Further reading
Craig JV, Lancaster GA, Taylor S, et al. Infrared ear
tion may need to be altered depending upon Local experienced senior clinical consulta-
thermometry compared with rectal thermometry in
the initial biochemistry, the diagnosis and tion and review should occur prior to trans- children: a systematic review. Lancet 2002;360
(9333):6039 [Review; 48 refs].
the clinical progress of the child. Monitoring fer. The child who requires transfer to a
Craver RD, Abermanis JG. Dipstick only urinalysis screen for
fluid balance is essential in the unwell infant facility that provides a higher level of care the pediatric emergency room. Paediatr Nephrol 1998;11
(3):331333 [1998 erratum appears in Paediatr Nephrol
or child. needs an experienced evaluation regarding
12(5): 426].
Nasogastric rehydration is commonly used the level of escort, urgency and mode of Gorelick MH, Shaw KN. Screening tests for urinary tract
infection in children: a meta-analysis. Pediatrics 1999;104
in dehydrated infants with gastroenteritis. transport required to safely achieve the
(5):54.
See Chapter 7.12. transfer. Gorelick MH, Shaw KN, Baker MD. Effect of ambient
temperature on capillary refill in healthy children.
Paediatrics 1993;92(5):699702.
Antibiotics Hewson PH, Gollan RA. A simple hospital triaging system for
infants with acute illness. J Paediatr Child Health 1995;31
Any specific management like administra-
tion of antibiotics depends on the provi-
Conclusions (1):2932.
Neville KA, Verge CF, OMeara MW, Walker JL. High
antidiuretic hormone levels and hyponatremia in children
sional diagnosis, and the severity of illness. Differential diagnostic with gastroenteritis. Pediatrics 2005;116(6):14017.
Most acute diarrhoeal illnesses are viral possibilities Neville KA, Verge CF, Rosenberg AR, et al. Isotonic is better
than hypotonic saline for intravenous rehydration of
and self-limiting and therefore do not For each patient the differential diagnoses children with gastroenteritis: a prospective randomised
require antibiotics. Clearly it is preferable vary depending on the constellation of symp- study. Arch Dis Child 2006;91(3):22632.
Shaw KN, McGowan KL, Gorelick MH, Schwartz JS. Screening
that appropriate cultures be taken prior to toms (fever, vomiting, diarrhoea or others). for urinary tract infection in infants in the ED: which test is
administration of antibiotics, although in Keep an open mind and always re-evaluate best? Paediatrics 1998;101(6):E1.

175
 7.9 Hepatitis
Franz E. Babl

to specific medical therapy. Persistence of

ESSENTIALS abnormal serum aminotransferase tests
beyond 3 months warrants aggressive evalu-
1 Hepatitis A, B and C are the main viral agents of acute hepatitis in children in ation to define the aetiology of the liver
Australia.
injury. A child with clinical evidence of chronic
2 Aboriginal children in certain areas of Australia are at higher risk of hepatitis liver disease should be referred to a paedia-
A than non-Aboriginal children. tric gastroenterologist for further evaluation
without the 3 month observation period.
3 Acute hepatitis due to the various hepatotropic viruses is clinically
indistinguishable.

4 The younger the child the more likely viral hepatitis will be asymptomatic or only History
mildly symptomatic without jaundice. Acute symptomatic viral hepatitis classically
presents with a 1-week prodromal phase
5 Severe hepatitis, especially with synthetic dysfunction, should be urgently referred
with non-specific symptoms of nausea,
to a paediatric gastroenterologist.
vomiting, malaise, anorexia and low-grade
6 Hepatitis A and B are vaccine preventable and post-exposure prophylaxis is fever, followed by an icteric phase with dark
available for both. urine followed by jaundice and pale stools.
Prodromal symptoms fade with the onset
7 In addition to toxin- and drug-related hepatitis, a variety of metabolic and
of jaundice but anorexia and malaise may
systemic illnesses can present with the clinical picture of acute or chronic
persist, and localised hepatic pain or tender-
hepatitis in children.
ness and pruritus may develop. Important
historical clues indicating an infectious
aetiology are attendance of child care and
Curtis syndrome), syphilis, typhoid
Introduction15 possible disease among peers and childcare
fever or associated with septicaemia;
workers, exposure to highly endemic com-
Hepatitis is defined as inflammation of the amoebic.
munities in Australia (e.g. remote Aboriginal
liver due to a number of possible causes. Drug and toxin related liver injury
communities) or overseas, attendance of
Jaundice or icterus implies a yellow disco- (Table 7.9.1).
schools and residential facilities for the
louration of skin, sclera and mucous mem- Chronic hepatitis.
branes due to hyperbilirubinaemia which Persistent viral infection (hepatitis B, C, D).
may or may not be caused by hepatitis. Autoimmune hepatitis.
Table 7.9.1 Patterns of hepatic
In children, the presentation of hepatitis Sclerosing cholangitis. drug injury
includes a diverse number of viral and Metabolic diseases, e.g. Wilsons disease,
Centrilobar Paracetamol
non-viral causes. Viral hepatitis continues a1-antitrypsin deficiency, cystic fibrosis. necrosis Halothane
to be a major health problem in developing Systemic illnesses, e.g. inflammatory
Microvesicular Valproic acid
and developed countries and can present as bowel disease, cardiac disease, total steatosis
an acute or chronic illness. parenteral nutrition.
Acute hepatitis Isoniacid
Viral hepatitis in children can present General Sulfonamide
with various manifestations, ranging from hypersensitivity Phenytoin
asymptomatic seroconversions especially in
Aetiology infants, mildly symptomatic and anicteric
Fibrosis Methotrexate

Cholestasis Chlorpromazine
Infection presentations with symptoms of a flu-like Erythromycin
hepatotropic viruses, e.g. hepatitis A, or gastroenteritis-like illness, to fulminant Oestrogens
B, C, D, E; hepatic failure as well as chronic hepatitis Veno-occlusive Cyclophosphamide
other viruses with hepatic leading to cirrhosis and hepatocellular carci- disease
involvement, e.g. EpsteinBarr virus noma. In adults, chronic hepatitis is defined Portal and hepatic Oestrogens
(EBV), cytomegalovirus (CMV); as biochemical or histological changes that vein thrombosis Androgens
bacterial infections with hepatic persist for more than 6 months. This defini- Biliary sludge Ceftriaxone
involvement, e.g. leptospirosis, tion in children would inappropriately delay
Hepatic adenoma Oral contraceptives
brucellosis, Q fever, cat-scratch disease, the diagnosis of several childhood causes of or carcinoma Anabolic steroids
gonococcal perihepatitis (FitzHugh non-viral chronic hepatitis which respond

176
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7

GASTROENTEROLOGY AND HEPATOLOGY

disabled, exposure to blood products, injec- antigen (HBsAg), IgM anti-hepatitis B core Epidemiology
tion drug use and high risk sexual activity (HBc) and anti-hepatitis C virus (HCV) anti- The most common mode of transmission is
(men who have sex with men). A history of body should be obtained. If these tests are person to person by the faecaloral route.
acute drug or toxin ingestion or ongoing negative, other viral causes such as EBV In developing countries, and also in certain
therapeutic use of potentially hepatotoxic and CMV should be considered. If no viral Aboriginal communities, where infection is
agents should be elicited. The history should cause can be identified, metabolic and sys- endemic, most persons are infected in the
also explore the possibility of acute or temic causes of acute and chronic hepatitis first decade of life. In developed countries,
chronic presentations of metabolic and sys- should be excluded. Wilsons disease can including much of Australia, infection may
temic illnesses associated with liver disease be detected by measuring serum coerulo- occur at an older age when susceptible
e.g. neurological complaints in Wilsons dis- plasmin levels and 24-hour urinary copper adults travel to endemic areas. In Australia,
ease, respiratory difficulties in cystic fibrosis, excretion, cystic fibrosis by measuring sweat the main patterns of hepatitis A are large,
or a history of cardiac disease. electrolytes, a-antitrypsin deficiency by mea- slowly evolving community outbreaks
suring serum a1-antitrypsin levels. involving childcare centres, residential facil-
ities, communities of intravenous drug users
Examination and male homosexuals, sporadic cases due
Although jaundice is the hallmark of hepati- Viral hepatitis14 to travel to endemic areas and point source
tis, many children will present anicteric. Car- outbreaks from contaminated food, water
Over the past decades the knowledge about
otenaemia, which also causes a yellow- or an infected food handler. Faecaloral
the viruses that cause hepatitis has
orange tinge of the skin due to high intake transmission from asymptomatic infections,
increased and is still evolving. The agents
of carotenoids in carrots and other vegeta- particularly in young children, likely
identified to cause hepatitis as their primary
bles, can be differentiated from jaundice accounts for many of the approximately
disease manifestation include hepatitis A
by the lack of scleral involvement. In addi- 50% cases where the source cannot be
virus (HAV), hepatitis B virus (HBV), hepati-
tion to jaundice, physical examination in determined.
tis D virus (HDV) and formerly non-A, non-B
acute hepatitis may show tender hepato- The incubation period is 1550 days
designated viruses, hepatitis C (parenteral
megaly and mild splenomegaly. Arthralgias (mean 30 days). The highest titres of HAV
transmission) and hepatitis E (enteral trans-
or arthritis and rashes can occur with hepa- in stool occur during 1 to 2 weeks before
mission). HBV is a DNA virus whereas the
titis B. Signs of chronic liver disease should the onset of illness, when patients are most
other hepatotropic agents are RNA viruses.
be specifically looked for including clubbing, likely to transmit HAV.
HAV and HEV are not known to cause
leukonychia, palmar erythema, spider naevi, chronic disease whereas the others can
bruising, scratch marks, asterixis, ascites, develop chronic infection. All five viruses
prominent abdominal veins, oedema and can cause fulminant hepatitis with acute Clinical course
signs of fat-soluble vitamin deficiency. liver failure. A child with evidence of syn- Hepatitis A is an acute self limited illness
thetic dysfunction (prolonged prothrombin associated with fever, malaise, jaundice,
time, hypoglycaemia, hyperammonaemia, anorexia and nausea. In children under
Investigations lactic acidosis) with or without positive viral 6 years of age symptomatic hepatitis occurs
serology should be urgently referred to a in only 30% and few will have jaundice.
Laboratory tests for hepatitis can be expen-
paediatric gastroenterologist. Patients with In older children and adults infection is
sive and many may not be clinically useful
severe hepatitis should be transferred early usually symptomatic, typically lasting
in the emergency setting. Initial studies
before intensive care therapy is required. several weeks, and 70% will have jaundice.
should include a full blood count, total
There are a number of other hepatotropic Prolonged or relapsing disease can occur
and fractionated serum bilirubins, aspartate
viruses which play a less well defined role in though no chronic infection occurs. Fulmi-
aminotransferase (AST), alanine amino-
the pathogenesis of acute and chronic liver nant hepatitis is rare but is more frequent
transferase (ALT), gamma glutamyl trans-
disease such as hepatitis G virus and TT with underlying liver disease.
ferase (GGT) and prothrombin time (PT).
virus. It is virtually impossible to clinically
A serum alkaline phosphatase level may
distinguish an acute infection due to one
be difficult to interpret in growing children
agent from another. Diagnosis is therefore
because levels increase with bone growth. Laboratory tests
dependent on the use of serologic and
Serum AST, ALT and GGT are indicative of HAV specific IgM and IgG antibody tests are
nucleic acid based assays. Many other
hepatocellular damage rather than liver available. IgM is invariably present by the
viruses can cause hepatitis as part of their
function tests. Slight elevations of AST time the patient presents and indicates cur-
clinical spectrum, such as EBV and CMV.
and ALT do not always predict mild disease rent or recent infection and usually persists
and both can fall with end-stage liver for 3 to 6 months. Anti-HAV IgG is detect-
disease. PT is the most useful indicator of Hepatitis A13,69 able shortly after the appearance of IgM.
synthetic function. If the aetiology of the Aetiology The presence of anti-HAV IgG indicates past
hepatitis is unknown, anti-hepatitis A immu- HAV is an RNA virus classified as a member infection (or possibly immunisation) and
noglobulin M (IgM), hepatitis B surface of the picorna virus group. immunity.

177
7.9 HEPATITIS

Management day care setting and in well-defined or fulminant fatal hepatitis. Asymptomatic
There is no specific treatment for hepatitis closed communities. There are insufficient infection is most common in young children.
A. Most patients can be managed at home. data for the use of hepatitis A vaccine alone Symptomatic illness is indistinguishable
Hepatotoxic agents should be avoided. for post-exposure prophylaxis. from other forms of hepatitis. Some patients
No specific dietary restrictions are required. will show extrahepatic manifestations early
Pruritus associated with cholestasis, which Hepatitis B13,6,1012 in the disease including arthralgias, arthritis,
develops in 510%, may be treated with Aetiology macular rash and papular acrodermatitis
cholestyramine, an H1-receptor blocker or HBV is a DNA-containing hepadnavirus. (GianottiCrosti syndrome). The rate of
local therapy. Important components include hepatitis B chronic HBV infection is age dependent. It
Children and adults with acute HAV infec- surface antigen (HBsAg), hepatitis B core occurs in up to 90% of infants infected
tion who are food handlers or work in child- antigen (HBcAg) and hepatitis B e antigen by perinatal infection, 2550% of children
care settings should be excluded for 1 week (HBeAg). 1 to 5 years of age, and 26% of older
after the onset of illness. Hospitalised children and adults with HBV. Chronic HBV
patients require contact precautions for Epidemiology infection can lead to chronic liver disease
1 week after the onset of illness if they are HBV is transmitted through blood or body and cirrhosis and primary hepatocellular
incontinent or in nappies. Patients should fluids such as semen, cervical secretions, carcinoma later in life, though it can occa-
be admitted if warning signs of acute liver wound exudates, and saliva of HBsAg posi- sionally occur in children. The risk of
failure develop (repeated vomiting, deepen- tive persons, in particular if they are chroni- death from HBV related cirrhosis or liver
ing jaundice and increasing prothrombin cally infected. Modes of transmission are cancer is approximately 25% for persons
time), and transfer to a liver transplant through blood and blood products (now rare who become chronically infected in early
centre is indicated at the onset of acute liver due to screening), sharing of needles, percu- childhood.
failure (increased prothrombin time and taneous or mucous membrane exposure to
clouding of consciousness). infective body fluids and homosexual and Laboratory tests
heterosexual activity. Perinatal transmission Antigen and antibody tests for HBV are
usually occurs during delivery rather than available to detect acute infection, resolved
Immunisations and post- intra-uterine. Non-sexual transmission infection, immunity after immunisation and
exposure prophylaxis occurs in household settings primarily from persons at high risk of transmission. HBsAg
Hepatitis A vaccines are highly immuno- child to child, and young children are at is detectable during acute infection. If the
genic in both children and adults, with very highest risk. Although the exact mechanism infection is self limited it disappears before
high efficacy in preventing hepatitis A, is unknown, it is likely due to frequent inter- serum anti-HBs can be detected (the win-
approaching 100%. The duration of immu- personal contact of non-intact skin or dow phase). IgM anti-HBc allows the detec-
nity is not known but there is no current mucous membranes with secretions or saliva tion of acute or recent infections, including
evidence that booster doses are required. or the sharing of inanimate objects, as HBV those in HBsAg negative patients during
There are a number of different vaccines can survive at ambient temperatures for 1 the window phase. Persons with chronic
available, also in combination with other week or longer. HBV is not transmitted by HBV infection are positive for HBsAg as well
vaccines, with variable age, dose and the faecaloral route. Prevalence of HBV as anti-HBc antibody. Both anti-HBs and
vaccination schedules. Vaccination is recom- infection varies markedly from country to anti-HBc antibodies are detected in persons
mended for susceptible persons travelling to country, with low rates in Australia, the with resolved infection, whereas anti-HBs
countries and areas with high endemic rates US, Canada and Western Europe and high alone is detected after hepatitis B vaccina-
of HAV infection, Aboriginal and Torres endemic rates in South-East Asia, China, tion. The presence of HBeAg and HBV viral
Strait Islander children residing in parts of the Pacific Islands and other developing DNA by polymerase chain reaction (PCR)
Australia, those whose occupation or life countries as well as some Australian Aborig- indicates ongoing viral replication,
style may put them at risk of acquiring hep- inal communities. First generation immi- increased disease activity and infectivity.
atitis A, people with intellectual disabilities grants usually retain the carrier rate of
and people with chronic liver disease of any their country of origin, but subsequent gen- Management
aetiology including those with hepatitis B or erations show a decline in carrier rate irre- For acute hepatitis B there is no specific ther-
C. Hepatitis A vaccination of children in spective of vaccination. apy and management is otherwise similar
Aboriginal communities has been shown The incubation period is 45 to 160 days to hepatitis A. Follow-up laboratory studies
to lead to rapid decline in hepatitis cases with an average of 90 days. are important to assess the development
in both indigenous and non-indigenous of carrier state and chronic liver disease.
people. Clinical course Interferon-a, lamivudine and other antiviral
Post-exposure prophylaxis with normal HBV virus infection causes a wide spectrum agents are being used to treat chronic hep-
human immunoglobulin (NHIG) is indicated of diseases ranging from asymptomatic atitis B infection with long-term remission
for household and sexual contacts within seroconversion, subacute illness with non- and reduced complications. Children with
2 weeks of exposure and for unimmunised specific symptoms or extrahepatic symp- chronic HBV infection should be managed
children and employees in outbreaks in the toms and clinical hepatitis with jaundice to with the involvement of a paediatric

178
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7

GASTROENTEROLOGY AND HEPATOLOGY

gastroenterologist and screened periodically are an estimated 125250 children infected (HDAg), both of which are coated with hep-
for disease activity and by abdominal ultra- vertically each year. Incubation time is atitis B surface antigen (HBsAg).
sound for hepatic complications. Liver trans- 2 weeks to 6 months, with an average of
plantation is a possible therapy in end-stage 6 to 7 weeks.
chronic hepatitis.
Epidemiology
Clinical course HDV causes infection only in persons with
acute or chronic HBV infection. HDV
Immunisations and post-exposure The signs and symptoms of HCV infection
requires HBV as a helper virus and cannot
prophylaxis are usually indistinguishable from HAV
and HBV infection. Acute disease tends to produce infection in the absence of HBV.
Hepatitis B vaccines are produced by recombi-
be mild and insidious in onset and in chil- It can cause infection at the same time as
nant DNA technology. They are highly effica-
dren most infections are asymptomatic. Only the initial HBV infection (co-infection) or
cious, safe and provide long-term protection.
15% of patients develop symptomatic acute infect a person already chronically infected
The vaccines are part of universal childhood
hepatitis. Persistent HCV infection occurs in (superinfection). Acquisition of HDV is simi-
immunisations in many Western countries,
the majority of infected children even in the lar to HBV, i.e. by parenteral, percutaneous
including Australia, and should be used
absence of biochemical evidence of liver dis- or mucous membrane inoculation. High
for other persons at high risk of exposure
ease. Most children with chronic HCV infec- prevalence areas include southern Italy,
such as healthcare personnel, haemodialysis
tion are asymptomatic. Chronic liver disease parts of Eastern Europe, South America
and human immunodeficiency virus (HIV)
and cirrhosis can follow and primary hepato- and the Middle East. HDV infection is rare
patients, patients receiving blood products,
cellular carcinoma can occur in these in Australia.
and those with chronic liver disease. Hepatitis
patients in later life. Incubation period for HDV superinfection
A and B combination vaccines are available.
is 28 weeks; in co-infection with HBV the
The vaccine is also used for post-exposure
Laboratory tests incubation period is similar to hepatitis B
prophylaxis in unimmunised household and
HCV infection can be detected using total (45160 days; average 90 days).
sexual contacts of persons with acute HBV
infection, newborns of HBsAg positive anti-HCV antibody assays and HCV RNA
mothers (the baby injection site should be by PCR. However, the diagnosis of HCV is dif- Clinical course
washed prior to injection to decrease needle ficult as antibody assays may be negative The importance of HDV infection lies in its
inoculation contamination), and after acci- early in the course of the acute illness. ability to convert an asymptomatic or mild
dental occupational percutaneous exposure chronic HBV infection into a fulminant or rap-
to blood that contains or might contain Management idly progressive disease. Acute co-infection
HBsAg. To provide immediate protection, All patients with HCV infection should be with HBV and HDV usually causes an acute
the latter two groups should also receive considered infectious and refrain from illness indistinguishable from acute HBV
hepatitis B immunoglobulin. All patients donating blood products and sharing tooth infection alone, except that the likelihood
with hepatitis B infection should also be brushes or razors. They should be counselled of fulminant hepatitis can be as high as 5%.
immunised with hepatitis A vaccine. on avoiding hepatotoxic agents including
medications and alcohol. Susceptible chil-
Hepatitis C2,3,13,14 dren should be immunised against hepatitis Laboratory tests
Aetiology A and B. Infected children do not need to be Diagnosis can be made by detecting IGM-
HCV is a small single-stranded RNA virus excluded from day care. HCV is not a contra- specific anti-HDV antibody and HDAg. If
with multiple genotypes. HCV is a member indication to breastfeeding. markers for HDV infection exist, HBV co-
of the flavivirus family. Interferon-a, ribavirin and other antiviral infection can be differentiated from superin-
agents, usually in combination, have been fection in an established HBsAg carrier by
Epidemiology used for treatment of HCV infection in chil- testing for IgM hepatitis B core antibody.
Infection is spread primarily by parenteral dren and adults, with combination therapy Absence of this core antibody suggests that
exposure to blood and blood products. showing better outcome than single drug the person is an HBsAg carrier.
Screening of blood donors and blood pro- therapy. Liver transplantation is indicated
ducts and inactivation procedures for HCV in end-stage liver disease. HCV infected chil-
Immunisations
have virtually eliminated the risk of post- dren require ongoing care by a paediatric
Because HDV cannot be transmitted in the
transfusion hepatitis due to this agent. gastroenterologist.
absence of HBV infection, hepatitis B immu-
At increased risk are injection drug There is no hepatitis C vaccine and immu-
nisation protects against HDV infection.
users, haemophilia patients who received noglobulin has not shown efficacy for post-
untreated clotting products in the past, hae- exposure prophylaxis.
modialysis patients and persons with high- Hepatitis E2,3
2,3
risk sexual behaviours. The risk of maternal Hepatitis D Aetiology
to infant (vertical) transmission, which is the Aetiology The HEV is an RNA virus and is the only
most likely source of HCV infection in devel- HDV is a particle consisting of an RNA known agent of enterically transmitted
oped countries, is 510%. In Australia there genome and a delta protein antigen non-A non-B hepatitis.

179
7.9 HEPATITIS

Epidemiology children. Excess or prolonged therapeutic inborn errors of metabolism and systemic ill-
Transmission of HEV is by the faecaloral administration of paracetamol combined nesses such as inflammatory bowel disease,
route. It is more common in adults than chil- with reduction in caloric intake may produce cardiac disease and liver disease associated
dren and has a high case-fatality rate in hepatotoxicity in children. When considering with parenteral nutrition. In addition, drugs
pregnant women. Cases have been reported adverse reactions to medications with liver and toxins can produce chronic hepatitis.
in epidemics and sporadically in developing involvement clinicians need to consider all
countries. HEV in Australia has been repor- drugs taken in the previous months includ- Autoimmune hepatitis
ted in returning travellers from endemic ing prescription medications, over the Autoimmune hepatitis (AH) is characterised
regions and in the Northern Territory. counter medications and complementary by progressive destruction of hepatocytes in
Outbreaks have been associated with medicines. In Australia the most commonly association with circulating auto-antibodies,
contaminated water. Chronic infection does reported alternative and complementary but in the absence of other known causes of
not seem to occur. medicines associated with hepatotoxicity chronic liver disease. AH type I occurs mainly
according to the Adverse Drug Reactions in females and presents with lethargy, jaun-
Clinical course Advisory Council (ADRAC) are kombucha dice and the typical findings of chronic
HEV causes a self-limited acute illness with tea, echinacea, evening primrose tea and liver disease. Antinuclear antibodies (ANA)
jaundice, malaise, anorexia, fever, abdominal valerian. are found in the majority. AH II is clinically
pain and arthralgia. Subclinical infection Hepatotoxicity can be predictable or similar but ANA is negative and anti-
also occurs. idiosyncratic. Predictable hepatotoxicity liverkidney microsomal antibodies are pres-
implies a high incidence in exposed ent. Liver biopsy shows piecemeal necrosis
Laboratory tests persons with dose dependence. Examples without involvement of the biliary tract.
Diagnosis can be made by detecting IgM are paracetamol or antimetabolites such Both forms of AH can be associated with
anti-HEV in serum or by detecting HEV as methotrexate. Idiosyncratic hepatotoxic- other autoimmune manifestations such as
RNA by PCR in serum or faeces. ity is infrequent and unpredictable. It is thyroiditis, glomerulonephritis, haemolytic
not dose dependent, may occur at any time anaemia and erythema nodosum. AH gener-
Viral hepatitis due to other viruses during exposure to the agent and may be ally responds to corticosteroid therapy.
EBV, CMV, HIV, herpesvirus, varicella zoster, immunologically mediated as a result of
rubella, adenoviruses and enteroviruses, as prior sensitisation. Sclerosing cholangitis
well as yellow fever and dengue fever in The pathological spectrum of drug Sclerosing cholangitis (SC) is characterised
tropical and subtropical areas, are also induced liver disease varies widely and is by chronic inflammation of the intra- or
known agents causing acute hepatitis in rarely specific (see table). The laboratory extrahepatic biliary tree. Primary SC is asso-
children. EBV and CMV are ubiquitous and features also vary widely. After exclusion ciated with inflammatory bowel disease or
most persons are infected by the time they of other causes of liver disease, the cessation immune deficiency, secondary SC results
reach young adulthood. If tests for hepatitis of a temporally related offending medica- from an obstructive process in the biliary
A, B and C are negative, EBV and CMV are tion with normalisation of liver function system such as stones or post-operative
among the most likely additional agents to tests is usually sufficient to establish a diag- stricture. Primary SC can occasionally be
consider. However, hepatic involvement with nosis. Treatment is mainly supportive. N- associated with antibodies such as ANA.
these viruses is usually only one component acetylcysteine is used as a specific antidote Although histology includes periductal
of a multisystem disease. in preventing paracetamol toxicity. The inflammation, differentiation from other
prognosis of drug or toxin induced liver causes of chronic hepatitis often requires
injury depends on its type and severity. cholangiography demonstrating typical bili-
Injury is usually completely reversible with ary abnormalities. Supportive therapy does
Drug and toxin-induced
the withdrawal of the offending agent. not prevent disease progression to cirrhosis.
liver injury3,15 The mortality of submassive hepatic necro-
The liver is the main site of drug metabolism sis with fulminant liver injury may, however, Metabolic causes of chronic
and is particularly susceptible to structural exceed 50%. hepatitis
and functional injury from drugs and toxins. Wilsons disease is an autosomal recessive
The clinical spectrum varies from asymptom- disorder caused by a defect in biliary copper
atic biochemical abnormalities to fulminant excretion. Excessive intrahepatic accumu-
liver failure. Children may be more or less
Chronic hepatitis lation of copper causes liver cell damage fol-
susceptible than adults to hepatotoxic reac- Overview lowed by fibrosis and cirrhosis. Extrahepatic
tions. For example, liver injury after halo- Chronic hepatitis can be caused by persis- accumulation of copper in brain, cornea and
thane is rare in children and paracetamol tent viral infection (hepatitis B, C and D), kidney accounts for extrahepatic manifesta-
toxicity is unusual in infants compared with autoimmune hepatitis, sclerosing cholangi- tions of neurological abnormalities and
adolescents, whereas most cases of fatal tis, metabolic diseases such as Wilsons KayserFleisher rings. Initial presentation
hepatotoxicity associated with sodium disease, a1-antitrypsin deficiency, cystic of Wilsons disease may resemble acute viral,
valproate use have been reported in fibrosis, Indian childhood cirrhosis and other fulminant or chronic hepatitis with variable

180
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7

GASTROENTEROLOGY AND HEPATOLOGY

extrahepatic findings. The importance in TPN is potentially fatal and is the major fac- Pediatric Gatrointestinal Disease. Ontario, Canada:
BC Decker, Hamilton; 2008. p. 85964.
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mon, lies in the ability to treat patients and is multifactorial. In general, with administra- Paediatrics. 4th ed. Chatswood, NSW: Elsevier Australia;
p. 190252.
other family members with penicillamine, a tion of oral feedings a gradual resolution of 6. Australian Government. Department of Health and
chelating agent. the liver disease occurs. Aging. National Health and Medical Research Council.
National Immunisation Program Schedule. Available from
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deficiency of the major serum protease immunise/publishing.nsf/Content/
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ciency, of which some are more prone to

Improved public health measures to 8. Hanna JN, Hills SL, Humphreys JL. Impact of hepatitis A
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heart failure or cyanotic heart disease. Red Book: Report of the Committee on Infectious Diseases. Australian Immunisation Handbook. 9th ed. Available
27th ed. Elk Grove Village, IL: American Academy of from http://www.health.gov.au/internet/immunise/
Hepatic dysfunction is due to hypoxaemia, Pediatrics; 2006. p. 32661. publishing.nsf/Content/Handbook-home; (accessed
systemic venous congestion and low cardiac 3. Balistreri WF. Part XVII The digestive system. Section 6. 15.10.10).
The liver and biliary system. In: Kliegman RM, American Academy of Pediatrics. Red Book: Report of the
output. Behrman RE, Jenson HB, Stanton B, editors. Nelsons Committee on Infectious Diseases. 27th ed, Elk Grove
Liver dysfunction is the most common Textbook of Pediatrics. 18th ed. Philadelphia: Saunders Village, IL: American Academy of Pediatrics; 2006.
Elsevier; 2007. p. 1657713. Kleinman RE, Goulet O-J, Mieli-Vergani G, et al, editors.
metabolic complication of total parenteral 4. Denson LA. Other viral infections. In: Kleinman RE, Walkers Pediatric Gatrointestinal Disease. Ontario, Canada:
nutrition (TPN). Cholestasis associated with Goulet O-J, Mieli-Vergani G, et al., editors. Walkers BC Decker, Hamilton; 2008.

181
 7.10 Intussusception
Kim Lian Ong Ian Everitt

patients with intussusception are present

ESSENTIALS in less than one half of patients with the dis-
ease.1,2 Intestinal obstruction is often the
1 A high index of suspicion is needed to make an early diagnosis. presenting sign.
2 Most cases are idiopathic. The patient is usually in the infant age
group and is previously healthy and well
3 Intussusception is the most common cause of bowel obstruction in children nourished, with acute onset of symptoms.
between 3 months and 3 years of age.
The presentation is one of sudden onset of
4 Paroxysmal colicky abdominal pain/distress is the most common symptom. intermittent colicky abdominal pain, mani-
festing as episodic bouts (110 minutes)
5 Profound lethargy may be the presenting feature in 10% of cases. of crying. One of the descriptions sometimes
6 Bilious vomiting and redcurrant stools present LATE. given by the caregivers is the drawing up of
the legs to the childs abdomen and then
7 Morbidity and morbidity is increased by a delayed diagnosis. kicking the legs in the air. The child is often
inconsolable during an episode of distress.
Often the child will appear pale due to
Introduction HenochSchonlein purpura (HSP) with
increased vagal tone caused by the telescop-
intramural haemorrhage.
ing bowel. Between the episodes, the child
Intussusception is a common cause of Lymphoma and leukaemia involving the
may be flat, lethargic or fall asleep
paediatric bowel obstruction, particularly bowel wall.
exhausted, whereas some children will
in children less than 2 years of age. Intussus-
resume normal activity until another bout
ception occurs when a bowel segment (usu-
Epidemiology of distress occurs.
ally the small intestine) invaginates into the
There is poor feeding, vomiting, and there
lumen of a more distal lumen of bowel. The Most of the children are younger than 1 year of may be passage of loose or watery stools.
invaginated segment, known as the intus- age, and the peak incidence occurs in infants The child may have one or more episodes
susceptum, is carried distally by peristalsis between 5 and 10 months of age. Intussuscep- of loose stool which may be followed by
while the mesentery and vessels are tion is the most common cause of intestinal blood or mucus per rectum within 1224
squeezed within the engulfing segment obstruction in patients between 3 months hours. The mixture of mucus and shed blood
(intussuscipiens). The resulting venous con- and 3 years. Patients under 3 years of age with described as redcurrant jelly is a late sign.
gestion is the cause of the blood and mucous intussusception usually do not have a mass The diarrhoea, which occurs early, may lead
in the stool, the classic redcurrant jelly stool lesion as the lead point, the telescoping is to a misdiagnosis of gastroenteritis, so intus-
that may result in some cases. Intussuscep- idiopathic and they are usually responsive to susception should be considered in any
tion occurs most commonly at the terminal non-operative reduction. Older children may young child having episodic distress in the
ileum when the terminal ileum is carried have a surgical lead point to the intussuscep- setting of a diarrhoeal illness. Initially the
through the ileocaecal valve into the colon tion and require operative reduction. vomiting is non-bilious but it becomes bil-
(ileocolic 90%) and in some instances The estimated incidence is 1 to 4 per 1000 ious when intestinal obstruction occurs.
the telescoping small bowel may even reach live births. There is an overall male prepon- There may be a preceding upper respiratory
the rectum. derance, with a male-to-female ratio of tract infection, which can sometimes distract
approximately 3 to 1. Mortality with treat- from the true cause of the childs distress.
ment is rare. Morbidity is increased by a delay This condition is unusual in children who
Aetiology in diagnosis and is likely to be due to bowel are malnourished. The child usually appears
wall necrosis and perforation. Delay will chubby and in good health. The child when
Most cases of intussusceptions are idio- cause prolonged intestinal obstruction with observed will be seen to have paroxysmal
pathic without any mass lesion acting as a persistent vomiting, causing resultant dehy- crying spells which represent episodes of
lead point or an apex of the intussusceptum. dration and electrolyte imbalance. abdominal pain between periods of lethargy.
In non-idiopathic intussusception, the fol-
In late presentations, the child may be flor-
lowing may act as lead points:
idly shocked and minimally reactive from
Clinical
Meckels diverticulum or polyp related. collapse.
Haemolyticuraemic syndrome. Clinically, the four classic symptoms and One must be mindful of the small subset
 Cystic fibrosis with inspissated bowel signs of vomiting, abdominal pain, abdomi- of encephalopathic intussusceptions that
content. nal mass and bloody stool described in present without symptoms to suggest a

182
7.10 INTUSSUSCEPTION
7

GASTROENTEROLOGY AND HEPATOLOGY

gastrointestinal problem (painless presenta- exclusion of intussusception ranges from
tion). These children will present with leth- 40 to 90%. In cases of established bowel Management
argy, sweating and pallor which may be obstruction, distended bowel loops and air- The treatment in the emergency depart-
episodic. fluid levels will be present. The presence ment is to initially provide stabilisation
Most children appear pale, but with pink of any free air indicates perforation and pre- depending on the childs clinical condition
conjunctivae. On examination, a right hypo- cludes non-operative intervention. and consult with a paediatric surgeon. Intra-
chondrium or mid-abdominal sausage- Traditionally, the diagnosis of intussus- venous fluids should be started for resuscita-
shaped mass may be palpated and this is ception is made by the use of an enema, tion (normal saline bolus) and subsequent
best felt when the child is quiet between either using air or barium. Contrast enema rehydration as required. The stomach should
spasms of colic. Abdominal palpation may is a quick and reliable investigation and is be decompressed by use of a nasogastric
be soft and appear non-tender in some often also therapeutic. Barium has tradition- tube, if there is a bowel obstruction.
cases, whereas some children will elicit ally been the contrast material used but per- Non-operative reduction by air enema
non-specific guarding. If obstruction has foration can result in barium and faecal (hydrostatic reduction) is now generally
occurred distension and tenderness will be peritonitis.57 The availability of near- accepted as the modality of treatment for
present. The nappy should be checked for isotonic water media and the use of air as most patients. Absolute contraindications
any blood, and in suspicious cases a gentle a contrast medium8,9 have changed the include peritonitis, perforation or profound
rectal swab may reveal otherwise occult traditional therapeutic approach. The shock.18
blood. Rarely, the bowel can progress to advantages of air reduction are its rapidity
present rectally and prolapse. The presence and safety compared with barium. In the
of fever and leukocytosis are late signs and case of perforation during the procedure,
may indicate transmural gangrene and air enema has been shown to result in a
Outcome
infarction. The occurrence of intestinal gan- smaller tear than hydrostatic enema with Various authors have reported reduction
grene and infarction can be suggested by markedly less spillage of faeces.10 In addi- rates of between 80 and 90% using air
the presence of peritonitis, with the physical tion, air has no deleterious consequences enema.1920,2628 The perforation rate is
signs of rigidity and involuntary guarding. within the abdominal cavity.11,12 quoted to be less than 1%.18,19 Factors that
Often patients with intussusception do Findings on contrast examination include are associated with lower reduction and
not present with classic signs and symptoms, the classical coiled spring sign, which is higher perforation rate, especially if more
which may lead to an unfortunate delay in caused by the contrast material tracking than one of the following are present: (a)
diagnosis, with disastrous consequences. around the lumen of the oedematous intes- patients age: younger than 3 months or
Therefore it is essential to maintain a high tine and the meniscus sign is produced older than 5 years; (b) long duration of
index of suspicion for intussusception when by rounded apex of the intussusceptum symptoms, especially if more than 48 hours;
evaluating a child presenting with abdomi- protruding into the column of contrast (c) passage of blood per rectum; (d) signifi-
nal pain, especially those less than 5 years material.13 cant dehydration; (e) small bowel obstruc-
of age or those who have HSP and episodic tion and (f) the presence of dissection sign
severe pain. Ultrasound on contrast study.2025
This is the imaging of choice to demonstrate Surgical reduction is now performed after
intussusception. There are some recent stud- failure to achieve reduction or when it is contra-
Investigations ies to suggest that ultrasound is highly sen- indicated to perform non-operative reduction.
sitive and specific for the diagnosis of The overall mortality rate of intussuscep-
All children should be resuscitated and sta- intussusception. Some authors reported tion is less than 1%.18 Mortality rates
bilised prior to any imaging. the sensitivity to be close to 100% in identi- observed among children in industrialised
fication of intussusception, even in relatively countries are lower than those in developing
Abdominal X-ray inexperienced hands.1416 countries.1,2833 Some of these deaths are
A plain abdominal radiograph may be per- The classical ultrasonographic sign on a preventable and may be related to reduced
formed, as a screen or if a reliable ultrasound longitudinal plane is the pseudokidney sign, access to or delays in seeking health care, fac-
is not readily available. Early in the course of which is an oval or tubular structure with a tors known to be associated with mortality in
the disease it may be normal, or it may show hyperechoic centre surrounded by hypo- children with intussusception.3133 Therefore,
an absence of air in the right upper quadrant echoic periphery. The hyperechoic areas rep- early diagnosis and management play an
and a right-sided soft tissue shadow giving resent mesenteric fat pulled with the vessels important role in the reduction of mortality.
an impression of an intracolonic mass. There and lymph nodes into the intussuscipiens
may be dilated small bowel and an absence while the hypoechoic border is the oedema-
of intraluminal gas in the region of the cae- tous wall of the intussuscepted intestinal References
cum. The presence of stool and air fluid head. On the transverse plane, a target sign 1. Simon RA, Hugh TJ, Curtin AM. Childhood
levels in the caecum makes the diagnosis is classic and appears as a circular mass with intussusception in a regional hospital. Aust N Z J Surg
1994;64:699702.
of intussusception less likely.3,4 The accu- a hyperechoic centre surrounded by a 2. Kim YS, Rhu JH. Intussusception in infancy and childhood:
racy of plain radiography in diagnosis or hypoechoic outer rim.17 analysis of 385 cases. Int Surg 1989;74:1148.

183
7.11 HERNIAE

3. Heller RM, Hernanz-Schulman M. Applications of new Pediatric Radiology. Colorado Springs, Colorado; 1994 24. Stephenson CA, Seibert JJ, Strain JD, et al.
imaging modalities to the evaluation of common Apr 28May 1. Intussusception: clinical and radiographic factors
pediatric conditions. J Pediatr 1999;135(5):6329. 13. del-Pozo G, Albillos J, Tejedor D, et al. Intussusception in influencing reducibility. Pediatr Radiol 1989;20:
4. Sargent MA, Babyn P, Alton DJ. Plain abdominal Children: Current concepts in diagnosis and enema 5760.
radiography in suspected intussusception: a reduction. Radiographics 1999;19:299319. 25. Fishman MC, Borden S, Cooper A. The dissection sign of
reassessment. Pediatr Radiol 1994;24:1720. 14. Bhisitkul DM, Listernick R, Shkolnik A, et al. Clinical nonreducible ileocolic intussusception. AJR
5. Grobmyer A, Kerlan R, Peterson C, Dragstedt L. Barium application of ultrasonography in the diagnosis of 1984;143:58.
peritonitis. Am Surg 1984;50:11620. intussusception. J Pediatr 1992;121:1826. 26. Gorenstein A, Raucher A, Serour F, et al. Intussusception
6. Mahvoubi S, Sherman N, Ziegler M. Barium peritonitis 15. Bowerman R, Silver T, Jaffe M. Real-time ultrasound in children: Reduction with repeated delayed air enema.
following attempted reduction of intussusception. Clin diagnosis of intussusception. Radiology Radiology 1998;206:7214.
Pediatr 1983;23:368. 1982;143:5279. 27. Ein SH, Alton D, Padler SB, et al. Intussusception in
7. Yamamura M, Nishi M, Furubayashi H, et al. Barium 16. del-Pozo G, Albillos J, Tejedor D. Intussusception: US the 1990s: Has 25 years made a difference? Pediatr
peritonitis. Report of a case and review of the literature. findings with pathologic correlation the crescent-in Surg Int 1997;12:4025.
Dis Colon Rectum 1985;28:34752. doughnut sign. Radiology 1996;199:68892. 28. Ein SH, Alton D, Palder SB, et al. Intussusception in
8. de Campo JF, Phelan E. Gas reduction of intussusception. 17. Pendergast LA, Wilson M. Intussusception: a the 1990s: has 25 years made a difference? Pediatr
Pediatr Radiol 1989;19:2978. sonographers perspective. J Diagn Med Sonogr 2003;19 Surg Int 1997;12:3746.
9. Shiels WE 2d, Maves CK, Hedlund GL, Kirks DR. Air enema (4):2318. 29. van Heek NT, Aronson DC, Halimun EM, et al.
for diagnosis and reduction of intussusception: clinical 18. DiFore JW. Intussusception. Semin Pediatr Surg Intussusception in a tropical country: comparison among
experience and pressure correlates. Radiology 1999;8:21420. patient populations in Jakarta, Jogyakarta, and
1991;181:16972. 19. Hadidi AT, El Shal N. Childhood intussusception: a Amsterdam. J Pediatr Gastroenterol Nutr
10. Shiels WE 2d, Kirks DR, Keller GL, et al. John Caffey comparative study of nonsurgical management. J Pediatr 1999;29:4025.
Award. Colonic perforation by air and liquid enemas: Surg 1999;34:3047. 30. Meier DE, Coln CD, Rescorla FJ, et al. Intussusception in
comparison study in young pigs. AJR Am J Roentgenol 20. Katz M, Phelan E, Carlin JB, Beasley SW. Gas enema for children: international perspective. World J Surg
1993;160:9315. the reduction of intussusception: relationship between 1996;20:10359.
11. Hernanz-Schulman M, Foster C, Maxa R, et al. Fecal clinical signs and symptoms and outcome. AJR 31. Stringer MD, Pledger G, Drake DP. Childhood deaths
peritonitis and contrast media: experimental protocol 1993;160:3636. from intussusception in England and Wales, 1984-9.
to assess synergistic effects and to compare relative safety 21. den Hollander D, Burge DM. Exclusion criteria and Br Med J 1992;304:7379.
of barium sulfate, water-soluble ionic media, saline and outcome in pressure reduction of intussusception. 32. Adejuyigbe O, Jeje EA, Owa JA. Childhood
air. Presented at the 35th Annual Meeting of The Arch Dis Child 1993;68:7981. intussusception in Ile-Ife, Nigeria. Ann Trop Paediatr
Society for Pediatric Radiology. Orlando, Florida; 1992 22. Reijnen JAM, Festen C, van Roosmalen RP. Intussusception: 1991;11:1237.
May 1417. factors related to treatment. Arch Dis Child 1990;65:8713. 33. Mangete ED, Allison AB. Intussusception in infancy and
12. Hernanz-Schulman M, Vanholder R, Schulman G. 23. Barr LL, Stansberry SD, Swischuk LE. Significance of age, childhood: an analysis of 69 cases. West Afr J Med
Inhibition of neutrophil phagocytosis by barium sulfate. duration, obstruction, and the dissection sign in 1994;13:8790.
Presented at the 37th Annual Meeting of The Society for intussusception. Pediatr Radiol 1990;20:4546.

7.11 Herniae
Andrew J.A. Holland

ESSENTIALS Types of herniae

1 The key diagnostic steps are detailed history from the carer and a careful clinical Inguinal
examination. The incidence of inguinal herniae in children
has been reported to be between 0.8% and
2 Radiological investigations have little, if any, role in the management of inguinal 4.4%,1 rising to 18.9% to 30% of preterm
herniae in children. infants.2,3 Inguinal herniae are six times more
3 A hernia that is not reducible may be associated with ischaemia and an urgent common in boys and are more common in
surgical opinion should always be sought. twins.1,4 Around 1 in 10 inguinal hernias are
non-reducible at presentation, although a care-
4 In children, unlike in adults, it is the gonad rather than the bowel structures that is ful history from the parents will often elucidate
most at risk from an inguinal hernia. earlier signs and symptoms. In this group, over
two-thirds are under 1 year of age.
In children, inguinal herniae are almost
always indirect.5 The hernia exits the perito-
Herniae are usually classified based on
Introduction two characteristics: anatomical location
neal cavity via the internal inguinal ring to
enter the inguinal canal, leaving the canal
A hernia is defined as the protrusion of a vis- and whether or not the hernia is reducible.
via the external inguinal ring. The sac is inti-
cus or part of a viscus into an abnormal loca- An irreducible hernia may result in ischae-
mately related to the contents of the sper-
tion. The most common herniae occurring in mia of either the contents of the hernia or
matic cord. It is compression of the testicular
children are inguinal and umbilical adjacent structures. Treatment of an irreduc-
vessels by hernial contents that may render
femoral herniae are rare. A variety of ible hernia is thus a surgical emergency. The
the testis ischaemic. In contrast, in females
descriptive terms is applied to herniae in use of the terms incarceration (irreducible)
the risk of ischaemia to the ovary and adnexae
these locations, often incorrectly. This may and strangulation (arrest of circulation)
usually occurs as a result of torsion of these
lead to diagnostic confusion and inappro- should be avoided, as all irreducible herniae
structures within the hernial sac.
priate delays in referral. may be associated with ischaemia.

184
7.11 HERNIAE
7

GASTROENTEROLOGY AND HEPATOLOGY

Rarely, direct inguinal herniae may occur, infants, when the incidence rises to between important complications such as injury to
with some series reporting an incidence of 41.6 and 75%.10,11 The vast majority of the vas and subsequent testicular atrophy.6
up to 5%.6 Typically, these children have umbilical defects appear to close spontane- Laparoscopic correction involves suture
either had previous inguinal surgery, a con- ously with increasing age, largely irrespec- ligation of the hernial sac from within the
nective tissue disorder or were delivered at tive of the size of the defect. Given the abdominal cavity and appears to have a
less than 30 weeks gestation. The clinical very low risk of the hernia becoming non- slightly higher recurrence rate than open
management and surgical approach reducible, surgical repair is only indicated surgery.13 Direct hernias are generally repai-
remains similar to indirect inguinal herniae. for those herniae that persist beyond 3 red primarily without the use of mesh.6
Inguinal herniae usually present as a and 5 years of age, or in the very few that If the hernia does not spontaneously
swelling in the inguinal region first noticed present with symptoms.12 reduce, a paediatric surgeon should be con-
by the carer when changing or bathing the Paraumbilical defects do not close with tacted urgently. In the absence of signs or
child, especially if crying or straining.7 The increasing age and should be referred for symptoms suggestive of ischaemia, manual
swelling may extend into the scrotum in elective repair on diagnosis. reduction by an experienced clinician may
boys or the labia majora in girls. Persistent be attempted. The child should be given
tachycardia, overlying erythema and marked Epigastric herniae appropriate analgesia. A two-handed tech-
tenderness suggest an irreducible hernia These herniae occur as a result of a defect, nique is required: one hand from above dis-
complicated by ischaemia.8 Occasionally, often only a few millimetres in size, in the engages the hernia from the external ring
an irreducible hernia may present with linea alba between the umbilicus and the and the other from below reduces the hernia
vomiting and abdominal distension as a xiphisternum. The child presents with a by gentle direct pressure. If there are signs
result of intestinal obstruction. small swelling in this region that may be suggestive of ischaemia, emergency surgery
A hernia can be differentiated from a associated with pain, usually due to entrap- is required.
hydrocele as the latter transilluminates and ment of extraperitoneal fat. Once diag-
does not usually extend into the inguinal nosed, elective surgical repair is required.
region. In neonates, these differences may Controversies
be less obvious and a hernia may appear to The role of routine contralateral
transilluminate. Encysted hydroceles of the inguinal exploration in children with a
cord and, rarely, testicular torsion may also Complications
unilateral inguinal hernia remains
cause diagnostic confusion. Where there is Complications of an irreducible hernia are controversial. A recent systematic review
any doubt regarding the diagnosis, a paedia- more likely to occur if the child presents late suggested that, as the overall risk of
tric surgeon should be consulted. or there is diagnostic delay. The most com- developing a metachronous hernia in
mon adverse sequelae include gonadal childhood was 7.2%, routine
Femoral ischaemia and bowel obstruction. Enteric contralateral exploration was not
Femoral herniae are rare in children, ischaemia, perhaps leading to perforation, warranted.14
accounting for between 0.4 and 1.1% of remains rare.
all groin hernias.1,9 They are often not diag- The use of intraoperative
nosed prior to surgery and are one cause of laparoscopy for the assessment of the
recurrent inguinal herniae in children. Typi- presence of a contralateral inguinal
cally, most present between 4 and 10 years
Treatment hernia has been advocated but even if
of age and there is an equal sex incidence. All inguinal hernias require surgery due to positive may not necessarily correlate
Clinically, the hernia presents as a swelling the risk of bowel ischaemia and compression with subsequent development of a
that is inferior and lateral to the pubic of adjacent gonadal structures. If the hernia symptomatic hernia on that side.
tubercle. As in adults, femoral herniae are is reducible, the patient should be discussed
associated with a high incidence of compli- with a paediatric surgeon regarding the
cations, so prompt surgical intervention is timing of follow up. Neonates and infants
required. should be assessed within 1 week of presen- References
tation and scheduled for early elective sur- 1. Bronsther B, Abrahams MW, Elboim C. Inguinal hernia in
children a study of 1000 cases and review of the
Umbilical and paraumbilical gery because of the high risk of the hernia literature. J Am Med Womens Ass 1992;27:5225.
herniae becoming irreducible in this age group. 2. Darlow BA, Dawson KP, Mogridge N. Inguinal hernia and
low birth weight. N Z Med J 1987;100:4924.
A true umbilical hernia occurs through the Carers should be advised to re-present 3. Harper RG, Garcia A, Sia C. Inguinal hernia: A common
umbilical ring as opposed to a paraumbilical urgently if signs and symptoms of an irreduc- problem of premature infants weighing 1000 grams or
less at birth. Paediatrics 1975;56:1125.
hernia that results from a defect adjacent to ible hernia develop. 4. Bawkin H. Indirect inguinal hernia in twins. J Pediatr Surg
the umbilical ring. Indirect inguinal hernias have tradition- 1971;6:1658.
5. Rescorla FJ. Hernias and umbilicus. In: Oldham KT,
Umbilical herniae are very common, ally been treated by a herniotomy, with Colombani PM, Foglia RP, editors. Surgery of infants and
occurring in up to 18.5% of infants under removal of the hernial sac following its liga- children. Philadelphia: Lippincott-Raven; 1997.
p. 106981.
6 months of age.5 They are more common tion at the internal ring. This procedure has 6. Brandt ML. Pediatric hernias. Surg Clin North Am
in Afro-Caribbean children and in premature a high success rate with a low incidence of 2008;88:2743.

185
7.12 GASTROENTERITIS

7. Johnstone JMS. Hernia in the neonate. In: Freeman NV, 10. Crump EP. Umbilical hernia: Occurrence of the infantile Redefinition of normal and re-evaluation of indications
Burge DM, Griffiths M, Malone PSJ, editors. Surgery of the type in Negro infants and children. J Pediatr for repair. World J Surg 2001;25:6458.
newborn. Edinburgh: Churchill Livingstone; 1994. 1952;40:21423. 13. Zitsman JL. Pediatric minimal-access surgery: update
p. 32130. 11. Vohr BR, Rosenfeld AG, Oh W. Umbilical hernia in low 2006. Pediatrics 2006;118:3048.
8. Kapur P, Caty MG, Glick PL. Paediatric hernias and birth weight infants (less than 1500 grams). J Pediatr 14. Ron O, Eaton S, Pierro A. Systematic review of the risk
hydroceles. Pediatr Clin North Am 1998;45:77389. 1977;90:8078. of developing a metachronous contralateral inguinal
9. Radcliffe G, Stringer MD. Reappraisal of femoral hernia in 12. Meier DE, OlaOlorun DA, Omodele RA, et al. Tarpley JL. hernia in children. Br J Surg 2007;94:80411.
children. Br J Surg 1997;84:5860. Incidence of umbilical hernia in African children:

7.12 Gastroenteritis
Susan Phin

episodes are due to viruses, with rotavirus

ESSENTIALS being by far the most common pathogen.
The most common bacterial causes are
1 Most children with gastroenteritis and mild-moderate dehydration can be Salmonella and Campylobacter. Shigella,
successfully rehydrated with oral rehydration solutions either by mouth or
Yersinia and Escherichia coli are less
nasogastric tube.
common, while Vibrio cholerae is seen in
2 Oral rehydration solutions (ORS) should be used for oral rehydration, not fruit developing countries. Parasites such as
juices, soft drinks or sports drinks. Giardia and Cryptosporidium are sometimes
the infective agent.
3 It is important to use appropriate intravenous fluids for bolus or maintenance In general a bacterium is more likely to be
phases of rehydration.
the causative agent if:
4 Careful instruction to parents in how to give oral fluids appropriately is vital. There is blood or mucus in the stool.
5 Antidiarrhoeal and most antiemetic medications should not be used in children. There is significant abdominal pain.
 There is a high-grade fever.
6 Early refeeding with age-appropriate foods should be encouraged.
These infective agents cause gastroenteritis
7 Timely reassessment is vital and, if the child does not progress as anticipated, by one or more mechanisms. Some directly
other diagnoses and complications should be considered.
invade the bowel wall, e.g. Salmonella or
8 Other diagnoses should be considered, especially in the presence of: Shigella, some produce toxins prior to
ingestion, e.g. Staphylococcus aureus, and
An infant less than 6 months of age some multiply and produce toxins within
High-grade fever the gastrointestinal tract, e.g. Shigella.
In an uncomplicated acute bout of
Bilious vomiting gastroenteritis, determining the causative
Significant abdominal pain pathogen is usually unnecessary, as this
usually does not alter the management.
No diarrhoea
Blood in vomitus or stool
History
Drowsiness/reduced level of consciousness.
Most children with acute gastroenteritis
present with a history of diarrhoea and
gastroenteritis still remains a significant vomiting. Abdominal pain and fever are
Introduction
cause of morbidity and mortality.1 sometimes accompanying symptoms. It is
Acute gastroenteritis is an inflammation of important to ask for specific details about
the gastrointestinal tract. It is one of the these symptoms, to increase the certainty
commonest reasons for children to present of the diagnosis of gastroenteritis and also
Aetiology
to an emergency department (ED). Most to help assess the risk of dehydration in
children under 5 years of age have experi- Gastroenteritis is caused by a wide range the child.
enced an episode of gastroenteritis and of pathogens including viruses, bacteria Diarrhoea refers to loose or liquid stools.
most can be successfully managed without and parasites (as shown in Table 7.12.1). The frequency, volume (small to voluminous)
admission to hospital. Worldwide, however, In developed countries, the majority of and consistency (semisolid to watery) and

186
7.12 GASTROENTERITIS
7

GASTROENTEROLOGY AND HEPATOLOGY

Table 7.12.1 Common causes of acute gastroenteritis Table 7.12.2 Standard assessment scale
for severity of dehydration
Viruses Bacteria Parasites
Variable Mild Moderate Severe
Rotavirus Salmonella Cryptosporidium
Norwalk virus Campylobacter jejuni Giardia lamblia Thirst
Enteric adenovirus Shigella Entamoeba histolytica
Escherichia coli Dry mucous
Yersinia enterocolitica membranes
Vibrio cholerae
Reduced
urine output
the presence of blood or mucus are all impor- the symptoms, other than gastroente- Lethargy
tant. Similarly, the frequency and nature ritis (see Chapter 7.8) and to assess the
Sunken eyes
(bilious or non-bilious) of the vomiting are degree of dehydration.
also important. The words my child is vomit- Occasionally pathogens causing gastro- Reduced
skin turgor
ing everything he tries to drink can be used enteritis can cause extraintestinal disease.
by parents to describe a child who has had This can occur particularly with Shigella Tachycardia
either two or 20 vomits in the last 24 hours. and Salmonella. Shigella can cause central Poor
It is important, therefore, to be specific in nervous system irritation, which can mani- perfusion
questioning. fest as encephalopathy or seizures. This Tachypnoea
The abdominal pain that can be associated may occur prior to the onset of diarrhoea.
Hypotension
with gastroenteritis is crampy in nature and Salmonella can cause a bacteraemia, which
commonly more pronounced in bacterial can lead to focal infections including osteo-
gastroenteritis. It is not accompanied by focal myelitis and meningitis. The infant under
or significant findings on abdominal examina- 6 months of age is particularly at risk. Gorelick et al5 performed a good study in
tion. If fever is a symptom, the height of the Philadelphia in 1997, which looked at the
fever can be helpful. Although children with Assessment of dehydration 10 clinical signs shown in Table 7.12.3.
rotaviral gastroenteritis are often febrile, it is The accurate assessment of dehydration is Using a gold standard of serial weight gain,
not usually high-grade. Such fevers are more difficult. Studies have shown that medical they found that <3 signs correlated with
likely in bacterial gastroenteritis or in other personnel tend to overestimate the degree <5% dehydration, 36 signs correlated
diagnoses altogether. of dehydration.2 The gold standard in assess- with 59% dehydration and >7 signs
Specific details about the amount of fluid ment of dehydration is a loss of weight correlated with >10% dehydration.
tolerated by the child are vital in assessing compared with a recent pre-illness weight.
the risk of dehydration. Parents are usually able For example, a 1-year-old weighing 10 kg a Laboratory investigations in the
to estimate whether, over a 24-hour period, the week ago who presents with gastroenteritis assessment of dehydration
child has taken one-quarter, one-half or three- for 3 days and now weighs 9.5 kg, is approx- There are few data to support the usefulness
quarters of normal intake. Intake that is con- imately 5% dehydrated. However, a recent of laboratory tests in the assessment of dehy-
sistently less than half of normal is of concern. weight is rarely available in the ED. There- dration due to gastroenteritis. Dehydration
Specific questions about urine output (i.e. fore, tables such as Table 7.12.2 use a com- is thought to typically cause a metabolic
heaviness and frequency of wet nappies) are bination of clinical symptoms and signs to acidosis. It is true that vomiting can cause
also important. Fewer than four wet nappies estimate the degree of dehydration. a metabolic acidosis by several mechanisms
in 24 hours is of concern when assessing It is important to remember that some of including volume depletion, lactic acidosis
hydration status. the signs and symptoms of dehydration can and starvation ketosis. However, isolated
Questions about the level of alertness and be affected by other factors. Mouth breathers vomiting can also cause a metabolic
activity of the child are also important. Leth- have dry mucous membranes. Watery diar-
argy may simply be an indication of signifi- rhoea can make it difficult to assess if the child
cant dehydration in a child. However, more has a wet nappy from urine output. Crying can Table 7.12.3 Ten clinical signs
severe lethargy and drowsiness (particularly of dehydration
cause a sunken fontanelle to appear full.
if out of proportion to that expected for Tachycardia may be caused by a crying, anx- 1. Decreased skin elasticity
2. Capillary refill >2 seconds
the dehydration) may indicate another more ious or febrile child; therefore it is the context 3. General appearance (ill-appearing, irritable,
serious diagnosis as the cause of the childs and trend of the pulse that is important. apathetic)
4. Absent tears
symptoms, such as enteroviral meningitis. Various studies have attempted to 5. Abnormal respirations
validate combinations of these signs and 6. Dry mucous membranes
7. Sunken eyes
symptoms with varying degrees of stand- 8. Abnormal radial pulse
Examination
ardisation and scientific validity.25 Diffi- 9. Tachycardia (HR >150)
10. Decreased urine output
The aim of the clinical examination is to culties arise as some of the signs are
exclude signs of an alternative cause of subjective and gold standards differ. Source: Gorelick et al.5

187
7.12 GASTROENTERITIS

alkalosis through loss of gastric acid. In addi- or bowel obstruction seems more likely than
tion, isolated diarrhoea can cause a meta- Investigations simple gastroenteritis, other investigations
bolic acidosis through loss of bicarbonate In uncomplicated acute gastroenteritis, it is such as abdominal X-rays, ultrasound or air
in the stool, but the child who can increase usually not necessary to perform any inves- enemas may be required.
oral intake to keep pace with the diarrhoeal tigations, as they will not alter management.
losses may not actually be dehydrated. Indications to consider performing investi-
Despite these confounding factors, it has Treatment
gations include:
been shown that serum bicarbonate is Mildly dehydrated
significantly lower in children with moderate uncertainty about diagnosis; Children in developed countries presenting
or severe dehydration (mean 14.5 mEq L1 severe dehydration; to EDs with gastroenteritis are often only
and 10.3 mEq L1) than in children with mild the childs clinical course not progressing mildly dehydrated or not dehydrated at all.
dehydration (mean 18.9 mEq L1).3 Raised as anticipated;
Most can be managed as outpatients with
serum urea levels have also been thought commencement of intravenous oral fluids alone. Educating the parents in
to reflect dehydration, but with even less rehydration.
how to give fluids, and observing them giv-
evidence. Stool cultures do not change management ing a trial of fluids to the child, are important
From a practical point of view, it is not in simple gastroenteritis; therefore they to empower the parents to provide ongoing
vital to put a specific percentage on the are not usually warranted. If bacterial gas- oral rehydration at home.
degree of dehydration, particularly when troenteritis is suspected a stool sample The essential things to ensure are:
the accuracy of such a specific percentage may be performed, although this will not
is questionable. What is important is to allo- the parent knows the appropriate oral
change management in the majority of
cate the child into a broad category of dehy- fluids to give;
cases. A stool sample may be taken for pub-
dration, for example, mild, moderate or the parent knows how, and is able, to give
lic-health reasons, or to reassure the doctor
severe, which determines what treatment the oral fluids correctly to optimise
that there is not another cause for the blood
should be commenced, and then to reassess success;
and mucus in the stool.
the child and the degree of dehydration the parent will have the child reviewed by
Urine culture is usually unnecessary when
within a specific timeframe. the local doctor within 48 hours;
the diagnosis of gastroenteritis is clear.
the parent knows what worrying signs
However, if there is little diarrhoea, particu-
and symptoms to look for that would
larly if the child is under 6 months of age,
Differential diagnosis it may be necessary to exclude a urinary
require representation.

Many other conditions can masquerade as tract infection. It is important that a clean If the above criteria cannot be satisfied, the
gastroenteritis by presenting with a combi- specimen is collected to avoid contamina- child may need to be admitted. This is partic-
nation of vomiting, diarrhoea, fever, or tion by stool contents. ularly so in the younger infant or those with
abdominal pain. These include: Electrolytes, urea and creatinine are profuse gastrointestinal losses, as they have
usually unnecessary in children with gastro- a higher risk of becoming dehydrated. See
other infections, e.g. urinary tract enteritis who are mild to moderately dehy- moderately dehydrated for further treat-
infection, meningitis;
drated and who are to receive oral fluids, ment options in those patients.
surgical diseases, e.g. intussusception, as they will not usually alter management.
bowel obstruction, appendicitis;
If, however, the child does not improve Appropriate oral fluids
raised intracranial pressure, e.g. blocked as expected, or deteriorates despite rehy- Oral rehydration solutions (ORSs) are the
ventriculoperitoneal shunt;
dration, or the child was initially severely appropriate fluids to rehydrate children with
metabolic diseases, e.g. diabetic
dehydrated, these tests along with a blood gastroenteritis. These are specifically
ketoacidosis, inborn errors of metabolism.
glucose level and perhaps other investiga- designed fluids that contain the correct
Features of concern to suggest the possibil- tions, will be required. amounts of sodium, glucose and other elec-
ity of other cause include: A full blood count and blood culture trolytes and are of the appropriate osmolality
are unnecessary in acute gastroenteritis. to maximise water absorption from the gut.
age less than 6 months;
However, if the diagnosis is uncertain it They use the principle of glucose-facilitated
high-grade fever;
may be reasonable to perform these tests. sodium transport, whereby glucose enhances
bilious vomiting;
In the younger infant, particularly the under sodium and secondarily water transport
significant abdominal pain;
3 month age group, if bacterial gastroenter- across the mucosa of the upper intestine.
no diarrhoea;
itis is likely, the possibility of systemic Salmo- The sodium and glucose concentrations and
blood in vomitus or stool;
nella infection should be considered and the osmolality are of vital importance.
drowsiness/reduced level of
blood culture and intravenous antibiotics The WHO ORS has a sodium concentra-
consciousness.
considered. tion of 90 mmol L1. In developed countries
See Chapter 7.8 on diarrhoea and vomiting, If significant abdominal pain is present and with non-cholera diarrhoea, it is generally
for more details on differential diagnosis. surgical pathology such as intussusception thought that 90 mmol L1 is a little high,

188
7.12 GASTROENTERITIS
7

GASTROENTEROLOGY AND HEPATOLOGY

as non-cholera gastroenteritis does not Method of giving oral fluids easy for a parent to detect. They will be
result in the same sodium losses that are The important message is to give small related to two things:
seen in cholera. Many different ORSs with amounts of fluid frequently, for example
The child is, or is at risk of becoming,
varying sodium concentrations have been 0.5 mL kg1 every 5 minutes. The fluid can
more dehydrated
developed. It has been shown6 that water be measured in a syringe and given to the
absorption across the lumen of the human
persistent vomiting despite small
child either by syringe, teaspoon or cup.
amounts of oral fluids frequently; or
intestine is maximal using solutions with a The child is far more likely to tolerate these
sodium concentration of 60 mmol L1 and
no wet nappy for 8 hours; or
small amounts of fluid than a whole bottle
this is the concentration recommended by
increasing lethargy.
at once. If the child tolerates this fluid the
Gastroenteritis may not be the correct
the European Society of Paediatric Gastroen- parent can gradually increase the volume
diagnosis
terology and Nutrition.7 Studies have also and decrease the frequency of the fluid
shown that hypo-osmolar solutions are most
bilious vomiting; or
offered. Success can be optimised in the ED
effective at promoting water absorption.810
significant abdominal pain; or
setting by giving the parents a documen-
Studies have also examined rice-based
drowsiness.
tation chart to fill in, which shows the
ORSs and their effect on stool output and fluid given and any vomits or diarrhoea or
duration of diarrhoea when compared with urine passed.
Refeeding
glucose-based ORSs. Rice-based ORSs It is important to educate the parents that Breast-fed infants breast-feeding
appear to have benefits in cholera diarrhoea should continue throughout the episode
seeing a doctor will not cure the vomiting
but not in non-cholera diarrhoea.11 of gastroenteritis, including the
and diarrhoea. Small, frequent amounts of
The composition of various ORSs and rehydration phase.
fluid will hopefully minimise the vomiting,
other fluids is shown in Tables 7.12.4 and but will not reduce the diarrhoea. The aim Formula-fed infants full-strength
7.12.5. Fruit juices and soft drinks are inap- normal formula should be started as soon
is for the input to exceed the output enough
propriate because of the minimal sodium as the infant is rehydrated. This can be
to rehydrate and then maintain hydration.
content and the excessive glucose content supplemented with an ORS for ongoing
Parents will know if they are succeeding
and hence excessive osmolality. Diluting losses, for example offer 10 mL kg1 after
by observing their child for the following
these solutions will not address the grossly each diarrhoea. However, children with
symptoms and signs.
inadequate sodium content, nor will it result no dehydration and mild gastroenteritis
in an optimal glucose concentration or Indications for re-presentation are the least likely to take an ORS
osmolality. Sports drinks are also inappropri- Parents should be given some indication because of the salty taste. In this instance
ate, with too low sodium levels and too high of symptoms and signs that will prompt re- increased amounts of normal fluids in
glucose levels and osmolalities. presentation. These need to be relatively conjunction with age-appropriate feeding
may be sufficient.
Early refeeding studies have shown that
early refeeding does not worsen or
Table 7.12.4 Composition of oral rehydration solutions
prolong the duration of diarrhoea, nor
increase vomiting or lactose intolerance,
ORSs Na (mm L1) Carbohydrate Osmolality and leads to a significantly higher weight
(mOsm L1)
(mm L ) 1
(%) gain after rehydration.12 Early
reintroduction of age-appropriate foods is
WHO 90 G 111 2 331
now recommended.
Gastrolyte 60 G 90 2 240
1
Gastrolyte-R 60 RSS 6 (g L ) 2.5 226
Moderately dehydrated
Hydralyte 45 G 90 2.5 240 Some children who present to EDs with gas-
(solution or iceblock)
troenteritis are moderately dehydrated.
G, glucose; RSS, ice syrup solids. These children can be rehydrated in several
ways. Some are successfully rehydrated with
oral fluids alone, as previously described.
Some fail this and require additional inter-
Table 7.12.5 Composition of oral fluids vention. Increasing numbers of hospitals in
developed countries are using oral rehy-
Oral fluids Na (mm L1) Carbohydrate (mm L1) Osmolality (mOsm L1)
dration therapy (ORT) via continuous naso-
Apple juice 3 690 730
gastric infusion.13,14 This is where an ORS
Sports drinks 20 255 330 is infused continuously down a nasogastric
Soft drinks 2 700 750
tube with a pump such as a Kangaroo pump.
Nasogastric infusions should not be used

189
7.12 GASTROENTERITIS

when the child has an ileus or is comatose. sodium solutions cause hyponatraemia venous blood gas. Blood cultures can also
Oral rehydration therapy has been the while solutions with a sodium content of be taken at the same time if indicated.
method of choice in developing countries 130154 mmol L1 are protective.28 As in Glucose-containing fluids such as 0.45%
since the 1970s. However, it has taken lon- nasogastric rehydration, it is important to (75 mmol L1) saline 2.5% glucose or
ger to become accepted in developed regularly reassess the childs fluid status. If 0.225% (37.5 mmol L1) saline 3.75%
countries. This is despite numerous studies the child remains on intravenous fluids for glucose should never be used for resuscitation
that show that it is as effective as intrave- >24 hours, it is important to recheck the boluses. Although these solutions are techni-
nous rehydration but less expensive1321 electrolytes. cally isotonic, the glucose is rapidly metabo-
and reduces lengths of hospital stay.13 If the child is significantly hypernatraemic lised, so effectively it is as if a hypotonic
Different regimens are used for continu- the rate of infusion needs to be reduced to solution has been given, with the resultant
ous nasogastric rehydration. The European rehydrate the child over 4872 hours. The risk of rapid fluid shifts and cerebral oedema.
Society of Paediatric Gastroenterology and aim is to avoid a rapid fall in the serum It is important to differentiate these resusci-
Nutrition recommends calculating the fluid sodium level as this can precipitate cerebral tation boluses from rapid rehydration ther-
deficit and replacing that over 4 hours.22 oedema. It is still reasonable to start with apy. The resuscitation boluses are given
The American Academy of Pediatrics 0.9% (150 mmol L1) saline 2.5% (or over several minutes and do not contain glu-
recommends that mildly dehydrated chil- 5%) glucose to prevent a rapid initial fall cose. The rapid rehydration fluids are given
dren receive 50 mL kg1 over 4 hours in serum sodium, but this may need to be over several hours and do contain glucose.
and moderately dehydrated children receive changed to 0.45% (75 mmol L1) saline After the initial resuscitation, replace-
100 mL kg1 over 4 hours.23 Other regimes 2.5% (or 5%) glucose, depending on prog- ment plus maintenance fluids are given as
recommend a fixed volume, for example ress of the electrolytes, which need to be per above. It is important to reassess the
40 mL kg1 over 4 hours for all mild to mod- rechecked frequently. If the child is hypona- childs hydration status regularly and look
erately dehydrated children followed by a traemic, 0.9% (150 mmol L1) saline 2.5% for any indication that another diagnosis is
reassessment and retrial of oral fluids.24 This (or 5%) glucose should be used. Senior the cause of the childs condition. Electro-
takes into account the tendency for medical paediatric advice should be sought if the lytes, urea and creatinine need to be regu-
officers to overestimate the degree of dehy- serum sodium level is significantly abnormal. larly repeated while the child remains
dration2 and the desire to avoid subsequent Rapid intravenous rehydration: Interest in significantly unwell.
over-hydration. The important thing to rapid intravenous rehydration for children
remember is that, whatever regimen is used with gastroenteritis has emerged in recent Other treatments
to rehydrate the child, fluid status should be years in developed countries. It has long Antibiotics
regularly reassessed. been used in developing countries.29,30 Antibiotics are rarely indicated in gastroen-
There have been a number of studies in teritis. They are of proven benefit for treat-
Intravenous rehydration developed countries over the last several ment of only the following situations:
Intravenous rehydration should be used to years that have looked at this issue24,28,3133
rehydrate children with gastroenteritis who and shown that this seems to be a safe and Shigella
fail nasogastric therapy or deteriorate. effective way to rehydrate children with Intravenous ampicillin in severe disease
The volume (mL) of replacement fluids is gastroenteritis who require intravenous Oral ampicillin or
calculated using the formula: weight (kg)  therapy. An example of this type of regime is trimethoprim-sulfamethoxazole in milder
dehydration (%)  10. Maintenance fluids giving 0.9% (150 mmol L1) saline 2.5% disease
are then calculated, with one method being glucose at 10 mL kg1 hr1 for 4 hours.34 It is Shortens illness duration, eradicates
to give 100 mL kg1 for the first 10 kg, important that lower sodium-containing organism.
50 mL kg1 for the second 10 kg and fluids are not used. Invasive Salmonella infections e.g.
20 mL kg1 for each subsequent kilogram. septicaemia, osteomyelitis, meningitis
The volumes for rehydration and mainte- Severely dehydrated Intravenous cefotaxime or ceftriaxone
nance are then added together and divided Although this is a relatively rare occurrence Antibiotics prolong excretion in
by 24 to calculate the hourly rate. in developed countries, it is vital to diagnose gastroenteritis.
The recommended fluid used to rehydrate and institute immediate treatment, as this is Travellers diarrhoea trimethoprim-
children with gastroenteritis has changed a medical emergency. The child is usually sulfamethoxazole.
over recent years. It is now recommended shocked and needs immediate intravenous Campylobacter septicaemia
that for children, excluding neonates and access and resuscitation with a 20 mL kg1 erythromycin or gentamicin. Not
infants <3 months of age, 0.9% bolus of normal saline or Hartmanns solu- indicated in uncomplicated
(150 mmol L1) saline 2.5% (or 5%) glu- tion. If the child remains shocked the bolus gastroenteritis.
cose should be used.25,26 Studies have should be repeated. Senior advice should
shown that children with gastroenteritis be sought about any child presenting in this Anti-diarrhoeal and anti-emetic
have inappropriately high levels of anti- condition. During insertion of the cannula, medications
diuretic hormone and increased incidence blood should be taken for electrolytes, urea, Anti-diarrhoeal medications are not indi-
and risk of hyponatraemia27 and that low creatinine, glucose, full blood count and cated in children with gastroenteritis. Most

190
7.12 GASTROENTERITIS
7

GASTROENTEROLOGY AND HEPATOLOGY

anti-emetic medications are also not indi- 16. Issenman RM, Leung AK. Oral and intravenous

cated. There is little evidence of efficacy and Controversies and future rehydration of children. Can Fam Physician
1993;39:212936.
a high incidence of side effects such as dys- directions 17. Vesikari T, Isolauri E, Baer M. A comparative trial of rapid
oral and intravenous rehydration in acute diarrhoea. Acta
tonic reactions and sedation in infants and Interest has arisen in the use of Paediatr Scand 1987;76:300305.
young children. There have been some recent probiotics such as Lactobacillus GG in the
18. Santosham M, Daum RS, Dillman L, et al. Oral rehydration
therapy of infantile diarrhoea. N Engl J Med
studies3537 that show that ondansetron treatment of acute gastroenteritis. Early 1982;306:10706.
may have some clinical benefit in this setting studies seem to suggest that it may
19. Tamer AM, Friedman LB, Maxwell SRW, et al. Oral
rehydration of infants in a large US urban medical center.
by reducing vomiting, but they do not decr- shorten the diarrhoeal illness.39 Further J Pediatr 1985;107:149.
ease the length of illness and may prolong research is necessary.
20. Listernick R, Zieserl E, Davis AT. Out-patient oral
rehydration in the United States. Am J Dis Child
the diarrhoea.37 Experienced clinicians who 1986;140:2115.
wish to use this medication in this setting The use of anti-emetics such as 21. Nager AL, Wang VJ. Comparison of nasogastric and
ondansetron in children may decrease intravenous methods of rehydration in pediatric
should generally limit its use to a single dose. patients with acute dehydration. Paediatrics 2002;109
the vomiting frequency in (4):56672.
gastroenteritis, but does not decrease 22. Sandhu BK, Isolauri E, Walker-Smith JA, et al. Early
feeding in gastroenteritis. J Pediatr Gastroenterol Nutr
Disposition the length of illness and may prolong the 1997;24:5227.
diarrhoea.3537 Further research is 23. American Academy of Pediatrics, Provisional Committee
on Quality Improvement, Subcommittee on Acute
In the past, many children were admitted to necessary. Gastroenteritis. Practice parameter: The management of
hospital with gastroenteritis when perhaps acute gastroenteritis in young children. Paediatrics
1996;97:42435.
they did not need to be.38 With the emer- 24. Phin SJ, McCaskill ME, Browne GJ, Lam LT.
gence of Short Stay Wards or Emergency Clinical pathway using rapid rehydration in children
with gastroenteritis. J Paediatr Child Health
Observation Units, where patients can be 2003;39:3438.
admitted to a special area within the ED for References 25. Childrens Hospital Australasia working party. Interim
recommendations: Intravenous fluid types for children
a finite time of less than 24 hours, hospital 1. World Health Organization. The treatment of diarrhoea: and adolescents. 2010.
inpatient admission rates for children with A manual for physicians and other senior health workers. 26. National Patient Safety Agency. Reducing the risk of
Geneva: WHO; 1995 (WHO/CDR/95.3 Rev 3 10/95). hyponatraemia when administering intravenous fluids to
gastroenteritis have fallen.24 In these units 2. Mackenzie A, Barnes G, Shann F. Clinical signs of children. 2007. Available from http://www.npsa.nhs.uk/
children can be rehydrated (orally or via rapid dehydration in children. Lancet 1989;2(8663):6057. nrls/alerts-and-directives/alerts/intravenous-
3. Vega RM, Avner JR. A prospective study of the usefulness infusions/ [accessed 15.10.10].
nasogastric or intravenous infusion) over a of clinical and laboratory parameters for predicting 27. Neville KA, Verge CF, OMeara MW, Walker JL. High
period of a few hours and then sent home percentage of dehydration in children. Pediatr Emerg antidiuretic hormone levels and hyponatraemia in
Care 1997;13:17982. children with gastroenteritis. Pediatrics 2005;116
after appropriate education and advice. This 4. Duggan C, Refat M, Hashem M, et al. How valid are (6):14017.
is provided that the medical officer is confi- clinical signs of dehydration in infants. J Pediatr 28. Neville KA, Verge CF, Rosenberg AR, et al. Isotonic is
Gastroenterol Nutr 1996;22:5661. better than hypotonic saline for intravenous
dent about the diagnosis of gastroenteritis 5. Gorelick MH, Shaw KN, Murphy KO. Validity and reliability rehydration of children with gastroenteritis: a
and that the criteria set out in the mildly of clinical signs in the diagnosis of dehydration in prospective randomised study. Arch Dis Child 2006;91
children. Paediatrics 1997;99(5):E6. (3):22632.
dehydrated section are fulfilled. Care needs 6. Hunt JB, Elliott EJ, Fairclough PD, et al. Water and solute 29. Slone D, Levin SE. Hypertonic dehydration and summer
to be taken especially with young infants, absorption from hypotonic glucose-electrolyte solutions diarrhoea. S Afr Med J 1969;34:20913.
in human jejunum. Gut 1992;33:47983. 30. Rahman O, Bennish ML, Adam AN, et al. Rapid
as they become dehydrated more rapidly 7. Booth I, Ferreira R, Desjeux JF, et al. Recommendation for intravenous rehydration by means of a single
than older children and are more likely to composition of oral rehydration solutions for the children polyelectrolyte solution with or without dextrose.
of Europe. Report of an ESPGAN working group. J Pediatr J Pediatr 1988;113:65460.
have another diagnosis. Gastroenterol Nutr 1992;14:1135. 31. Reid SR, Bonadio WA. Outpatient rapid intravenous
Paediatric staff in non-childrens hospitals 8. Ferreira RMCC, Elliott EJ, Watson AJM, et al. Dominant rehydration to correct dehydration and resolve vomiting
role for osmolality in the efficacy of glucose and glycine- in children with acute gastroenteritis. Pediatrics 1996;28
can potentially be utilised to help manage containing oral rehydration solutions: Studies in a rat (3):31823.
these patients in conjunction with ED staff. model of secretory diarrhoea. Acta Paediatr 32. Moineau G, Newman J. Rapid intravenous rehydration
1991;81:4650. in the pediatric ED. Pediatr Emerg Care 1990;6
An admission to, and discharge from, these 9. Hunt JB, Thillainayagam AV, Salim AFM, et al. Water and (3):1868.
Short Stay Wards causes much less disrup- solute absorption from a new hypotonic oral rehydration 33. Brewster DR. Dehydration in acute gastroenteritis.
solution: Evaluation in human and animal perfusion J Paediatr Child Health 2002;38:21922.
tion to the child and family and allows hos- models. Gut 1992;33:16529. 34. NSW Department of Health. Infants and Children: Acute
pital resources to be used for other patients 10. International Study Group on Reduced-osmolarity ORS Management of Gastroenteritis Clinical practice
Solutions. Multicentre evaluation of reduced-osmolarity guideline. 3rd ed. 2010.
requiring inpatient admissions. Some chil- oral rehydration salts solution. Lancet 1995;345:2825. 35. Freedman SB, Adler M, Seshadri R, et al. Oral ondansetron
dren, however, still require an inpatient 11. Gore SM, Fontaine O, Pierce NF. Impact of rice based oral for gastroenteritis in a pediatric emergency department.
rehydration solution on stool output and duration of N Engl J Med 2006;354(16):1698705.
admission for successful management. diarrhoea: Meta-analysis of 13 clinical trials. Br Med J 36. Reeves JJ, Shannon MW, Fleisher GR. Ondansetron
1992;304:28791. decreases vomiting associated with acute gastroenteritis:
12. Walker-Smith JA, Sandhu BK, Isolauri E, et al. a randomized, controlled trial. Pediatrics 2002;109(4):
Recommendations for feeding in childhood e62.
Prognosis gastroenteritis. J Pediatr Gastroenterol Nutr 37. Chubeddu LX, Trujillo LM, Talmaciu I, et al. Antiemetic
1997;24:61920. activity of ondansetron in acute gastroenteritis. Aliment
13. Gremse DA. Effectiveness of nasogastric rehydration in
Gastroenteritis in children is generally a hospitalised children with acute diarrhoea. J Pediatr
Pharmacol Ther 1997;11(1):18591.
38. Elliott EJ, Backhouse JA, Leach JW, et al. Pre-admission
benign, self-limiting disease, with dehydra- Gastroenterol Nutr 1995;21:1458. management of acute gastroenteritis. J Paediatr Child
14. Mackenzie A, Barnes G. Randomised controlled trial
tion being the major potential complication. comparing oral and intravenous rehydration therapy in
Health 1996;32:1821.
39. Guandalini S, Pensabene L, Zikri MA, et al. Lactobacillus
If this is recognised early and the child is rehy- children with diarrhoea. Br Med J 1991;303:3936. GG administered in oral rehydration solution to children
15. Sharifi J, Ghavami F, Nowrouzi Z, et al. Oral versus
drated appropriately, the child should recover intravenous rehydration therapy in severe
with acute diarrhoea: A multicentre European trial.
J Pediatr Gastroenterol Nutr 2000;30:5460.
completely with no adverse sequelae. gastroenteritis. Arch Dis Child 1985;60:85660.

191
 7.13 Constipation
Bruce Fasher

at a socially convenient time. The distending

ESSENTIALS rectum evokes a wave of contraction with
inhibition of the smooth muscle tone of
1 It is important to understand the physiology and development of gut transit to the internal anal sphincter, resulting in a
recognise the normal (often regarded as abnormal).
sensation of the urge to defecate. An urgent
2 Constipation is a common reason for children to present to EDs with abdominal desire to defecate occurs as the stool
pain. stretches the sensitive zone of the upper
anal canal. This urge is overcome by the vol-
3 Management depends on the childs age and whether the problem is acute or untary contraction of the external sphincter
chronic.
and the levator ani muscles. Eventually the
4 The patients and parents real concerns need to be acknowledged. rectum habituates to the stimulus of the
enlarging faecal mass and the urge to defe-
5 Issues to be addressed include: cate subsides. With time this retentive pat-
natural history; tern can become automatic.
It is understandable, therefore, that the
the aims of treatment (empty the bowel and establish pharmacological rhythm child who is afraid to use the non-private,
and then wean to biological rhythm);
wet, smelly school lavatory, and allows his
the likely duration of treatment; rectum occasionally to overcome the exter-
nal sphincter, as he is relieved to arrive
the anticipated setbacks; home, albeit with poo in the pants is really
the need for a coordinator clinician (normally unavailable in an ED). a normal variant rather than true encopresis.
There is a wide variation of physiology
6 Treat from the top, once at night and titrate the dose according to the response. and normal development as can be seen in
Avoid treatment per rectum if at all possible, to draw attention away from any anal
the age range of successful potty training.
obsession to achieve bowel actions.
To produce a stool at will is one of the childs
7 Be able to refer to a resource who is known to be interested in managing the first major achievements and most gain sat-
condition and to ensure appropriate ongoing review and support. isfaction from framing their success in a pot.
If too much persuasion is provided, espe-
cially if full control has never been attained,
the childs profound disappointments are
sympathetic paediatrician in difficult cases compounded by disapproval and hostility
Introduction if deemed appropriate.1,2 from the parents. Like most adults, most chil-
Constipation in childhood engenders in dren seek solitude to defecate.
clinicians unwarranted anxieties about man-
agement. Defining terms and understanding Definitions
normal physiology and its variants, the Constipation is delay or difficulty in Management basics
frequent blurring in paediatric medicine defecation, sufficient to cause
between physical and emotional factors significant distress. Be interested. The patient has often
and their impact on normal develop- Encopresis is the passage of a normal been pushed from pillar to post, with a
ment along with evidence-based data can stool in an abnormal place. quick fix, and reviewed in 3 months.
provide the clinician with an algorithm for  Soiling is the frequent involuntary Recognise that the parents and the
management. passage of loose or semi-loose stools in patient have a concern, which can be the
Management varies according to age and clothing. This is usually overflow cause of major family dysfunction.
whether constipation is acute or chronic. incontinence as the liquid stool escapes Endeavour to treat from the top, orally,
The emergency department (ED) is a diffi- around an impacted rectal faecolith. rather than continue to direct attention
cult place from which to manage constipa- to the rectum and anus with
tion, especially chronic constipation, for suppositories and enemas. The
success requires ongoing maintenance ther- exception is when a fissure needs
apy and contact with a committed and inter-
Pathophysiology managing with ointment or Xylocaine
ested clinician. This is best achieved through The rectum and anal canal have two tasks, ointment if defecation can be
the childs local doctor, with input from a to store faeces temporarily and to evacuate anticipated.

192
7.13 CONSTIPATION
7


GASTROENTEROLOGY AND HEPATOLOGY

Develop a pharmacological sugar (brown sugar is better) or sorbitol
armamentarium of stool softeners or (13 mL kg 1 noct.) will help soften the Chronic constipation
osmotic aperients. Stimulants (e.g. stool. Formula switching is usually unpro- Chronic constipation is defined as persistent
senna) may cause abdominal discomfort ductive, although it is recognised that some delay and difficulty in defecation often asso-
with colic in infants. forms of cows milk intolerance may present ciated with soiling (overflow incontinence).
Be aware that ongoing management by with persistent constipation. Symptoms may be temporarily relieved by
a single interested clinician is desirable Should constipation persist and the clini- laxatives but relapse rapidly. This is why
as the constipation will often relapse, cian is confident that mothers description the ED is not the ideal place for long-term
and gains are often small (three steps is of constipation, red flags should alert management, but may offer acute interven-
forward, two steps back). other considerations. tion for immediate relieve of unpleasant
This makes constipation difficult to Fever, vomiting, bloody diarrhoea, failure symptoms and to gain the familys confi-
manage in EDs. Such departments need to thrive, anal stenosis, abdominal disten- dence by explaining management and natu-
to have appropriate and willing referral sion, history of delayed passage of meco- ral history.
resources. Outpatient appointments nium, polydipsia or polyuria should prompt These children usually present in the
rarely work for the same reasons a search for physical causes. school-age group. There may be a history
difficulties with continuity of care and Conditions to consider are listed in of fissure, pain and withholding then ongo-
the ability to be seen relatively urgently Table 7.13.1. ing constipation for months or years. The
(albeit that encouragement with colon, rectum and upper anal canal become
management, behavioural modification loaded with faeces, insensitive and hypo-
and aperient dose titration is often all Acute constipation tonic. Soiling develops with loose faeces
that is necessary and can often be done escaping around an inspissated hard faeco-
by phone by the interested clinician). Acute constipation often occurs after febrile lith. Faeces are often palpable per abdomen.
The significant risk of relapse dictates illnesses, change in diet or environment, Social rejection in the class, due to the mal-
that a period of maintenance therapy is especially when intake has been low (e.g. odorous state, is often the first impetus for
necessary before weaning off low post-operatively). A fissure in ano, usu- the patient themselves to seek cure. This,
medications. It might be appropriate to ally from the passage of a hard faecolith, with loss of ability to defecate at will, takes
let the patient and family know that if it may cause pain, sphincter spasm, withhold- a toll on the childs emotional stability. They
has taken 2 years to arrive at this stage ing and the start of a vicious cycle leading to have often undergone multiple manage-
it is highly likely it will take 2 years or so chronic constipation with megacolon and ment regimens from dietary manipulation
to regain the rectums natural motility overflow incontinence. Also consider the to manual disimpaction, delivered by a vari-
and sensitivity. management of a fissure-in-ano which may ety of healthcare workers.
be hiding in the anal skin folds.
Most acute constipation resolves sponta-
neously but given the risk of chronic con-
Constipation in babies stipation, especially if there has been a Investigations
Never again in life will the stooling pattern, previous tendency to constipation, it may
When there is doubt regarding the diagno-
and indeed the stools, be so closely exam- be prudent to commence gentle oral therapy
sis, a plain abdominal X-ray is useful to dem-
ined as in the period of nappies being early.
onstrate faecal loading. Demonstration of
changed prior to toilet training. Straining
the X-ray findings to the parents may be
at stool is often marked and then miscon-
additionally useful to aid adherence to pro-
strued as constipation whereas it is a simple Table 7.13.1 Conditions possibly longed therapeutic strategies.
reflection of the urge to defecate sensation. causing persistent constipation
Plantar flexion of the toes is a similarly Anal anomalies (stenosis, stricter,
objective sign (at all ages). malposition)
Spinal anomalies (spinal dysraphism, sacral
Breast-fed infants may pass a stool after agenesis) Management
each feed or as infrequently as once every Hirschprungs disease
Hypothyroidism The management of acute versus chronic
few weeks. As long as the stool is of normal Metabolic hypercalcaemia, hypokalaemia constipation varies mainly in nuance and
quality (often referred to as scrambled eggs) Coeliac disease
Cows milk intolerance the extent and duration of treatment.
and of great volume there is no reason for Lead poisoning The aim of treatment is that in gaining
concern. For a couple of days prior to defeca- Intellectual impairment
Child abuse the familys confidence the clinician recog-
tion the infant may be, not unreasonably, Cystic fibrosis nises that there is a very real concern, and
somewhat unsettled. Dietary inadequate roughage, excessive
cows milk intake a plan can be instituted to:
Just weaned, bottle-fed infants may pro- Psychogenic parent-child and environment
duce dry, hard stools with difficulty and issues Exclude physical treatable causes with
Drug related anticholinergic,
sometimes traces of fresh blood. Attention sympathomimetics, codeine careful history, examination and
to water intake, perhaps addition of extra investigations as warranted.

193
7.13 CONSTIPATION

Empty the rectum, preferably from the Macrogol 3350 which is available in Maintenance therapy
top i.e. through oral agents rather than several palatable commercial Focuses on the prevention of recurrence and
per rectum. presentations. The Macrogol 3350 induces should be instituted straight after successful
 Establish a pharmacological bowel a laxative effect by osmosis, is virtually disimpaction or for the child presenting
rhythm and pattern, which may need unchanged and unabsorbed in the gut and without disimpaction. This involves, as
support for several years. has no known pharmacological activity. above, dietary interventions, behavioural
Allow the enlarged rectum/colon to re- Electrolytes are present in the formulation, modification, laxatives, patience and
establish its own inherent physiological realising virtually no net loss of sodium, enthusiasm.
bowel pattern and tone and to regain its potassium or water.
normal sensation. Only should oral therapy be inadequate or if Dietary changes
Continue maintenance for some time a large impacted stool in rectum is causing Those commonly advised include increased
before considering slowly weaning off significant acute distress: treatment from fluid intake, absorbable and non-absorbable
pharmaceuticals. below may be necessary. carbohydrate, (sorbitol is found in prune,
This will involve: pear and apple juice). No randomised con-
Suppositories of glycerin may be helpful,
especially in acute constipation. trol study confirms the benefit of this. Force-
Enthusiasm on part of managing
Microlax enemas are also helpful in the ful implementation would then seem
clinicians.
acute situation. undesirable.
Non-punitive behaviour on part of the
parents. Phosphate enemas (these should be
Adjunctive behavioural modification avoided in children less than 2 years old;
interventions with achievable goals. persistent use may cause
Controversies and future
Acknowledging the need for patience, hyperphosphataemia, hypocalcaemia
and tetany).
directions
determination and resolution as the most
chronic cases will take years to resolve. Enemas, soap and water (tap water The role of serotonin type 4 receptors in the
and magnesium have potential neurochemical basis of peristalsis may
Stool lubricants, paraffin oil, the taste of
toxicity). provide a more logical pharmaceutical
which has been well masked in Australia
approach to the future management of
by Parachoc, using a nocturnal dose Disimpaction of an obstinate rectal faecolith constipation.
starting at 10 mL and titrating dose may require:
according to response (not recommended
less than 1 year old due to possible Polyethylene glycol electrolyte solution
aspiration pneumonia). lavage, 25 mL kg 1 hr 1 (to 1000 mL hr 1
Osmotic laxative, lactulose or sorbitol, by nasogastric tube); causing nausea,
bloating, cramps, aspiration pneumonia.
References
initially 3 mL kg 1 noct. as a 70% 1. Clayden GS. Constipation and soiling in childhood.
solution, titrating dose according to This will require the child to be admitted Problems of childhood. London: BMA; 1976.
response. to hospital. 2. Baker SS. Constipation in infants and children: Evaluation
and treatment. J Pediatr Gastroenterol Nutr 1999;29
Stimulants, senna starting at 2.5 mL noct. Manual disimpaction under general (5):61226.
titrating dose according to response (side anaesthesia.
effects are possible colic, and if used in There is no evidence-based medicine to
combination with a lubricant it may just suggest the success or otherwise of anal
make the faecolith spin around). dilatation (Lords procedure), and some Further reading
consider permanent external sphincter Liptak GS. Constipation. In: Meyer, et al., editors. Evidence-
Good results have been achieved using based paediatrics and child health. London: British Medical
sachets (according to the directions) of damage may occur. Journal Books; 2000.

194
 8

SECTION
NEUROLOGY
Section editor Ian Everitt

8.1 CSF shunt complications 195 8.5 Acute ataxia 215

8.2 Raised intracranial pressure 199 8.6 Headache 220
8.3 Seizures and non-epileptic events 203 8.7 CNS infections: meningitis and encephalitis 226
8.4 Acute weakness 207

8.1 CSF shunt complications

Richard Lennon

device. There may also be an antisiphoning

ESSENTIALS device. The distal tubing is tunnelled under
the skin to the drainage site, which is most
1 Emergency physicians should consider the possibility of a shunt complication in commonly the peritoneal cavity. The distal
any child with a cerebrospinal fluid (CSF) shunt presenting to an ED because
catheter usually contains valves that pre-
symptoms and signs of malfunction may be non-specific, subtle and of gradual onset.
vent back flow. Variations on the positioning
2 The most common shunt complications are malfunction (undershunting and of the proximal tubing include placement in
overshunting) and infection. the subdural or subarachnoid space and
placement in cystic malformations such as
3 Shunt infection is much more likely to occur in the 6 months following insertion or the Dandy Walker syndrome and also in
revision.
the spinal canal as in lumbo-peritoneal
4 CT scan and plain X-rays of shunt hardware (Shunt Series) are the preferred shunts. Variations in the placement of the
imaging methods for detecting shunt malfunction; however, these tests are not 100% distal catheter include the right atrium,
sensitive and patients should be referred to the neurosurgical service if clinical the pleural cavity and the gall bladder. By
suspicion persists. far the most common is the peritoneal cav-
ity, with these alternatives only being used
5 Before performing any procedure on a shunt, it is best to consult with the treating if the peritoneal site is contraindicated.
neurosurgeon.
Shunt problems that may present to an
emergency department (ED) are listed in
Table 8.1.1.
diagnose those complications, commence
Introduction time critical treatments and refer to a neuro-
The intervention of cerebrospinal fluid (CSF) surgical service when appropriate.
shunting for CSF accumulations has brought
Clinical presentation
about long-term survival and avoidance of Types of shunt The developmental stage of the child with a
disabilities in children suffering from hydro- Many types of CSF shunt may be encoun- CSF shunt can cause considerable variation
cephalus. Unfortunately, the insertion of tered in paediatric emergency practice. in the clinical presentation of shunt compli-
inert non-growing hardware in infants and There are also many different types of shunt cations. The most obvious is the presence of
children who are usually very active and hardware; however, most have the same an open anterior fontanelle in infants up to
can expect to grow 20 to 30 times their birth basic structure, which comprises a proximal the age of approximately 9 to 18 months.
weight leads to a high rate of shunt compli- tube that takes CSF, usually from the lateral Simply looking and feeling the fontanelle
cations. Some studies find that 60% or more ventricle, to the outer surface of the skull. At allows an estimation of intracranial pres-
shunts need revising after several years. The this point there is usually a subcutaneous sure. A bulging fontanelle is a highly specific
task of the Emergency Physician is to one-way valve and possibly a pumping but not very sensitive sign of under-shunting;

195
8.1 CSF SHUNT COMPLICATIONS

recent contact with a case of gastroenteritis)

Table 8.1.1 Complications of CSF Table 8.1.2 Signs and symptoms that on
shunts their own warrant immediate referral to CSF shunt complication is very unlikely. For
neurosurgical service cases where CSF shunt complications are
Infection
Malfunction Bulging fontanelle neither ruled in or out on historical and
Blocked proximal catheter Decreased level of consciousness examination findings, one must resort to
Blocked distal catheter Fluid tracking around shunt tubing
Loculation of lateral ventricle Loss of upward gaze (sunset eyes) investigations and/or observation and/or
Valve dysfunction Signs of local infection (usually <6 m post op) neurosurgical consultation.
Overshunting/slit ventricle syndrome Erythema of site
Disconnection Erosion/ulceration
Abdominal pseudocyst CSF leak
Migration of distal catheter Purulent drainage
History
Invasion of abdominal organs (VP shunt) Meningismus Parents may present very early, reporting
Peritonitis, ascites (VP shunt) Peritonitis
Pulmonary emboli (VA shunt) vague and non-specific symptoms, particu-
Glomerulonephritis (VA shunts) larly if the child has had a similar presenta-
Brain tumour metastases
CSF ascites tion of shunt malfunction previously.
Pleural effusion (pleural shunts) The most common presentation of
comparison with previous scans (see discus- children with shunt obstruction is headache,
sion of CT scanning below). Table 8.1.3 vomiting and/or drowsiness. It is notable that
shows the signs and symptoms with strong seizures occur on neither of the above lists.
a sunken fontanelle can be a sign of positive predictive power but not strong This is because many children with shunts
overshunting. enough to warrant immediate referral if also have epilepsy, therefore seizure alone
The cranial sutures in children are not just one feature is present on its own. Thus has a poor correlation with shunt complica-
fused and can undergo diastasis due to in a patient with a ventriculoperitoneal tion. It is important to remember that distal
raised intracranial pressure. The older a child shunt (VPS) who presents with fever alone, shunt problems may present with abdominal
is, the longer and higher the intracranial an initial general work up for a cause of symptomatology such as pain and distension.
pressure has to be to cause diastasis. This the fever is warranted and then consider- Symptoms suggesting possible infection
also means that the head circumference will ation for referral to neurosurgery made if include fever, lethargy, irritability, or features
rapidly increase when there is inadequate no definite cause for the fever is found. How- of meningism. There may be concurrent
shunting. Therefore it is important to mea- ever, fever with another feature listed in symptoms of shunt obstruction.
sure the occipitofrontal circumference and Table 8.1.3, such as headache, warrants
compare it with previous records if available early neurosurgical referral. The question
and plot it on growth centile charts. A head of whether to send the child home is more Examination
circumference that is rapidly crossing cen- difficult. The absence of any of the symp-
tiles in an upward fashion or a head circum- toms and signs listed in Tables 8.1.2 and For examination features of raised intracra-
ference that is in the very high centile range 8.1.3 does not rule out shunt malfunction nial pressure see Chapter 8.2. Features of
especially when the other parameters, or infection. If concerned about shunt infec- infection e.g. fever, rigors, lethargy, localised
weight and length, are not, is an indicator tion, this is much less likely if the patient is redness, swelling and peritonism (ventriculo-
of undershunting. Another implication of more than 6 months from the last shunt peritoneal shunt) or pleurisy (ventriculo-
unfused cranial sutures is that because this insertion or revision. However, this does pleural shunt) should be sought.
allows an increase in cranial volume it not exclude shunt malfunction. Where a
retards the rise in intracranial pressure. This symptom or sign that is not of high predictive Shunt evaluation
may be the reason that small infants present power is adequately explained by another The extent of the shunt course itself should
with more non-specific signs and symptoms diagnosis (e.g. vomiting with diarrhoea and be examined thoroughly. The older child
than older children. is usually the best at finding the shunt
The most common question an emergency hardware under his/her hair. This should
physician will have to answer when con- be palpated and, if possible, inspected for
Table 8.1.3 Symptoms and signs may
fronted with a child who has a CSF shunt is warrant neurosurgical consultation (see
inflammation of the skin along the shunt
Should I refer this patient to the neurosurgi- text) route. Many have a silicon-pumping bulb,
cal service? A recent study by Piatt et al1 Abdominal pain which is used both for checking the patency
attempted to quantify the power of various of the shunt and for access to sample CSF.
Fever
symptoms and sign to predict shunt malfunc- Most reservoirs can be compressed easily
tion or infection. Table 8.1.2 shows the Nausea/vomiting and rapidly refill in a few seconds. Incom-
strongly predictive signs and symptoms that Irritability pressibility of the bulb is usually due to
allow referral to be made on the basis of that obstruction of the distal catheter. This is
Headache
single finding. This may be done even before the less common site of obstruction. If the
computerised tomography (CT) scanning, as Abnormal shunt pump test bulb compresses easily but does not refill,
the neurosurgeon may prefer to have the Accelerated head growth then the proximal catheter is blocked. This
scan done locally to allow for easier is the shunt pump test. An abnormality of

196
8.1 CSF SHUNT COMPLICATIONS
8

NEUROLOGY
the test did not perform well enough to war- with obtaining a CT scan need to be consid- due to overshunting and may be non-
rant immediate referral in Piatts study.1 This ered. One difficulty is keeping the child still compliant. This leads to sharp fluctuations
is because it can be difficult to tell if a test is long enough to obtain adequate images in ICP, with very little change in CT appear-
abnormal or not. The return of the chamber even with rapid CT scanners. In infants, firm ance. The management of this complication
after depression can take a number of min- wrapping and sucrose on a dummy or paci- is a neurosurgical challenge. The emergency
utes and may take longer if the choroid fier may be sufficient to keep them still. physician needs to be aware that small ven-
plexus is drawn into the catheter causing When these fail procedural sedation or gen- tricles do not mean a normal ICP. Nuclear
partial or complete obstruction. This is the eral anaesthesia are required. This is less medicine shunt function studies are also
reason why shunt pump tests should be kept desirable because of anaesthetic risk, longer used in some centres for diagnosing shunt
to a minimum as multiple tests can not only delays, use of resources, and the potential to blockage. An abdominal ultrasound may
cause blockage with choroid but also other temporarily obscure one of the important be useful to identify a CSF pseudocyst.
debris and the ventricular wall, and can signs of raised intracranial pressure (ICP),
cause low pressure headache. decreased level of consciousness. Another Aspects of some CSF shunt
CSF tracking around the proximal cathe- consideration is the harmful effects of the complications
ter can form a fluctuant swelling around radiation exposure. A protocol designed to Infection
the burr hole in the skull where the shunt minimise radiation exposure in children The diagnosis of shunt infection is not
enters. This indicates a blocked proximal should be used and the head angulated to always straightforward. In a retrospective
catheter or disconnection and the child avoid radiation exposure to the eyes. This case series.3 the presenting symptoms were
requires neurosurgical consultation. reduces the risk of premature cataract forma- as listed in Table 8.1.4.
The entire subcutaneous catheter should tion. These precautions may not be routine The positive predictive value of these
be carefully inspected and palpated for con- at institutions that do not frequently scan symptoms was even greater in the first 9
tinuity and the abdomen examined for evi- children and the clinician may have to months following insertion of the shunt
dence of peritonitis or pseudocyst formation. ensure that these things are done. Some because 80% of all shunt infections occur
studies have examined magnetic resonance in this time. There is also an increase in
imaging (MRI) as an alternative to CT scan- VP shunt infections after laparotomy. Case
ning; however, the scan takes longer, makes reports also mention other symptoms such
Investigations
it difficult to access the child, often requires as rigors whenever a ventriculoatrial shunt
On rare occasions, insertion of a needle into the child to be sedated or anaesthetised is manipulated. The most useful investiga-
a shunt pumping chamber may be useful for and often does not provide images as clear tion is initially the CSF white cell count,
both diagnostic and therapeutic reasons but as a CT. which is elevated in approximately 70% of
should generally be performed by neurosur- Enlarged ventricles on scanning may indi- cases. The blood white cell count is elevated
gical colleagues. The exception is in the rap- cate undershunting due to obstruction. in only 30% of cases. A positive CSF culture
idly deteriorating child, where contact with However, this may be chronic and compari- is the gold standard, although this may be
the neurosurgeon is delayed or where in son with previous CT scans is necessary for negative in those children who have been
remote locations after discussion with the accurate interpretation. Obliteration of the on antibiotics. Bacterial antigen detection
treating neurosurgeon it is considered perimesencephalic cistern is a particularly and polymerase chain reaction testing may
important to get some information to help worrying sign and mandates urgent neuro- be helpful in this situation.
with a decision to transport. In the case of surgical consultation. The scan may also Staphylococcus epidermidis is the most
a moribund child, an emergency physician show the reason for malfunction (e.g. cathe- common bacterium isolated, followed by
may relieve the raised intracranial pressure ter tip embedded in brain tissue) or it may other coagulase negative staphylococci and
by inserting a 25-gauge butterfly needle into show a ventriculitis when the lining of the Staph. aureus. Less commonly Gram-negative
the bulb/pumping chamber at 45 degrees ventricles enhances with contrast. Alterna- bacteria, such as Propionobacter and Strepto-
to the skin under strict aseptic technique.2 tively, neuroimaging may reveal small slit- coccus pneumoniae and Candida infections,
It is important not to advance too deeply like ventricles. These are thought to be have been reported.
as the needle can damage valve mechan-
isms so badly that replacement is necessary.
The pressure can be measured with a similar
technique to that used in a lumbar puncture Table 8.1.4 Shunt infection presenting symptoms2
and then CSF drained off until the pressure
Symptom Percentage of cases of infection
is 10 cmH2O. with symptom
In the stable child, an X-ray of the entire Shunt malfunction 33%
shunt may demonstrate a disconnection or
Fever 26%
kinking causing blockage. The CT scan is
the usual preferred method of imaging, Localised wound or shunt tract inflammation 22%
because it provides clear images in a short Abdominal pain or pseudocyst 19%
space of time. However, difficulties associated

197
8.1 CSF SHUNT COMPLICATIONS

Initial antibiotic therapy can be tailored to Several liver abscesses have been reported. reported. Impacts on distal tubing may
findings on Gram stain from a CSF tap and/or Treatment involves removing the distal part cause shunt penetration of abdominal
cultures from previous infections. If the the tube, possibly repairing damaged organs viscera.
patient has no contraindications flucloxacil- and often a temporary alternative shunting Is there an open wound that may commu-
lin or dicloxacillin should be in all empirical method. nicate with the shunt or the CSF? This would
regimens with Gram-negative cover consid- increase the risk of infection. Thus a scalp
ered after consultation with neurosurgeons wound near the entry point of the tubing
Glomerulonephritis
and an infectious-disease specialist. In the into the cranial cavity may cause a pneumo-
This is a complication of ventriculoatrial
majority of cases the shunt will have to be cephalus and may lead to meningitis. At the
shunts, which are rarely used today. This
removed and replaced at a later date, with other end, a bowel perforation may cause a
is usually a gradually progressive illness
some temporary measure in the meantime. distal shunt infection.
which requires a high level of suspicion
In addition to these questions there is
for diagnosis. Treatment is usually to
Early post-operative the possibility that the presence of the
remove the shunt and replace it with an
complications shunt and/or the underlying pathology
alternative.
Wound dehiscence and purulent discharge may make the child more likely to have
are much more likely in the first few weeks some complication of trauma. The most
after shunt insertion. If there is early over- Trauma in children with common example of this is subdural haema-
shunting this can lead to such a degree of a CSF shunt toma from rupture of bridging veins that
brain shrinkage that the subdural bridging In a child who has a CSF shunt in place and have been stretched by brain shrinkage
veins are broken and subdural haematomas is subject to trauma the emergency physi- after shunt placement.
form. Also, the presence of blood or fibrin in cian should seek to answer the following
the CSF increases the risk of proximal block- questions:
age. At the other end of the shunt peritonitis Was there a direct impact upon the shunt
and wound infection is more likely in the hardware? Relatively minor trauma to the
early post-operative period. shunt may cause breakage and malfunction References
1. Piatt JH, Garton HJ. Clinical diagnosis of
which may not become manifest until some ventriculoperitoneal shunt failure among children
Migration and penetration days or weeks following the trauma; also, a with hydrocephalus. Pediatr Emerg Care 2008;24
(4):20110.
of shunts direct blow may cause shunt movement and 2. Roberts JR. Clinical Procedures in Emergency Medicine.
In various cases the distal end of VP shunts damage to tissues surrounding the shunt. 5th ed. Philadelphia, PA: WB Saunders Company; 2009.
3. Williams DG, Hayes J, McCool S. Shunt infection in children:
have migrated into the thorax, liver, bowel Intracranial haemorrhage following moder- presentation and management. J Neurosci Nurs 1996;
lumen and even out of the anus and mouth. ate impact in sporting events has been 28(3):15562.

198
 8

NEUROLOGY
8.2 Raised intracranial pressure
Richard Lennon

via lymphatics. Intracranial pressure will be

ESSENTIALS raised whenever there is obstruction to the
flow or reabsorption of CSF or in the rare cir-
1 A rapidly expanding head circumference in an infant prior to suture fusion, is an cumstance where CSF production is increased.
important sign of raised ICP.
2 The open anterior fontanelle allows direct palpation of ICP in children up to 918 Measurement of ICP
months of age. Intracranial pressure is usually estimated
3 In rare circumstances an emergency procedure, such as a subdural or a ventricular from lumbar puncture (LP) manometry, when
tap, can be performed through the anterior fontanelle in infants. performed on a child lying in the lateral decu-
bitus position in a relaxed posture. Measuring
4 A brain CT scan or MRI can rule out many causes of raised ICP, but does not exclude CSF pressure in a screaming child is likely to
raised ICP itself and thus should not be the sole basis for deciding whether or not it is be inaccurate because of the temporary rise
safe to perform a lumbar puncture. caused by high venous pressure. Similarly, the
5 Acute severe elevation of ICP is a true emergency often requiring intubation and person holding the child in the fetal position
ventilation, infusion of mannitol 1 g kg1 and early consultation with neurosurgeons. for the LP should relax their grasp and allow
the child to stretch out temporarily while the
pressure is being measured. If the LP is being
done under a general anaesthetic, attempts
hemispheres to be reabsorbed through should be made to normalise the pCO2, so
Introduction the arachnoid villi on the superior sagittal that the pressure reflects awake ICP. If a lum-
Normal physiology sinus. CSF can also be reabsorbed through bar puncture is performed on a child in a sit-
Normal intracranial pressure (ICP) is 618 several other channels, including a small ting posture measurement of hydrostatic
cmH2O. It is the product of the intracranial amount through the choroid plexus and pressure is likely to be elevated compared
contents mainly blood, brain and cerebro- to ICP. Adjustment of the sitting posture LP
spinal fluid (CSF) and the resistance of pressure to reflect intracranial pressure is
Table 8.2.1 Causes of raised intracranial
the cranial vault. Normal ICP has a diurnal difficult because multiple factors affect the
pressure
cycle that is higher in the early hours of the relationship, including; the height of the
morning when one is normally supine during A. Increased CSF (hydrocephalus)
child, their posture and their emotional
1. Decreased absorption
sleep. Therefore the symptoms of raised Obstructive state. Nevertheless, if the child is very co-
ICP, such as headache and vomiting, are Communicating operative, pressure measurement can still
2. Increased production
usually worse in the morning. Intracranial be attempted by carefully laying the child
pressure may be raised by anything that B. Swollen contents on their side straight after the needle has
can cause an increase in the volume of its con- 1. Meningitis entered the subarachnoid space.
2. Encephalitis
tents or a decrease in the size of the cranial 3. Cerebral oedema
cavity (Table 8.2.1). This section will not cover Hypo/hypernatraemia
Post-ischaemic
the diagnosis and management of raised Post-traumatic/diffuse axonal injury Particular issues in children
intracranial pressure associated with trauma.
Those issues are covered in Chapter 3.2. C. Space-occupying lesion (SOL) Infants
Most CSF is made by the choroid plexus in 1. Tumours An infants inability to communicate and
Primary
the lateral third and fourth ventricles. A nor- Secondary smaller repertoire of behaviours makes raised
mal child makes approximately 20 mL hr1 2. Haematomas intracranial pressure more difficult to diag-
Extradural
and the total volume is 50 mL in an infant, Subdural nose and one needs to have a high index of
rising to 150 mL in an adult. It flows through Intracerebral/intraventricular suspicion as the presentation may be subtle.
3. Abscesses/cysts
the foramen of Monro into the third ventri- 4. Arteriovenous malformation The infant does not have a rigid cranial
cle, then down the aqueduct of Sylvius, 5. Congenital cysts vault until fusion of the cranial sutures. This
which is usually only 2 mm wide, and D. Decreased or fixed intracranial volume is a gradual process occurring throughout
3 mm long in a child. This leads into the 1. Depressed skull fracture childhood; therefore ICP can cause diastasis
fourth ventricle and from there via the 2. Premature fusion of cranial sutures of the cranial sutures in children, however,
(craniosynostosis)
foramina of Luschka and Magendie to the this is rare in children over the age of 7 years
basal cisterns. From there the CSF flows over E. Pseudotumour cerebri (benign and even rarer now that computerised
the surface of the cerebellar and cerebral intracranial hypertension) tomography (CT) scans and magnetic

199
8.2 RAISED INTRACRANIAL PRESSURE

resonance imaging (MRI) allow for earlier slower onset conditions such as brain The central herniation syndrome
diagnosis. Because of this flexibility, increas- tumours the most common scenario in Generalised midline or bilateral swelling
ing intracranial contents in the infant will infants is the gradually progressive onset above the tentorium cerebelli causes the
cause a lesser increase in the ICP and a of drowsiness/lethargy, morning irritability midbrain to herniate through the tentorium.
greater increase in the head circumference and vomiting, with an expanding head cir- This leads to dysfunction of the midbrain
than it would in older children or adults. This cumference. In older children there are pro- and higher centres due to compression,
is one of the reasons why acute increases in gressive early morning headaches, but no causing:
ICP in infants are often not easily detected dramatic increase in head circumference.
by the infants subtle change in behaviour Brain tumours may also present with focal
drowsiness;
patterns. neurological signs before there is a signifi-
initially small reactive pupils;
The measurement of head circumference cant rise in ICP due to direct invasion of neu-
decorticate posturing;
(occipitofrontal) is a useful means of detect- ral pathways. Other symptoms may include a
as the process worsens pupils become
midrange;
ing intracranial pathology. The head circum- head tilt, which is due to unilateral 4th cra-
ference should be plotted on centile charts, nial nerve palsy causing a vertical strabis-
posture becomes decerebrate;
using prior measurements, if available, to mus. The child will compensate for this by
bilateral 6th cranial nerve palsies may
become apparent when attempting the
determine if there has been a trend to cross tilting the head. This often occurs with pos-
dolls-eye reflex;
percentiles. The childs length and weight terior fossa tumours. However, there are
should be plotted concurrently, to evaluate other causes of head tilt such as sternomas-
these are accompanied by Cushings triad,
i.e. hypertension, bradycardia and
if the head is disproportionately large or toid tumour in the newborn and benign
abnormal breathing patterns (alternating
small. When the cranial sutures separate torticollis. In obstructive hydrocephalus,
tachypnoea/bradypnoea and fluctuating
due to expansion, percussion of the skull paralysis of upward gaze is common due
depth of respiration).
makes a sound similar to that of a cracked to third nerve dysfunction. This leads to
pot. This is known as Macewens sign. the classic picture in the infant of a big head
Conversely, the open anterior fontanelle and sunset eyes. Some infants will become The lateral mass herniation
allows direct palpation of intracranial pres- irritable on watching TV or looking at books syndrome
sure up to the age of 9 to 18 months. It is because of diplopia. Parents may notice stra- A unilateral supratentorial swelling will give
highly recommended that one closely bismus and older children will complain of rise to a pattern of events formerly known as
observes the fontanelles of normal infants, diplopia. the uncal herniation syndrome. Imaging
to help one identify abnormalities in clinical Often there will be regression in motor studies now show that herniation of the
practice. The normal fontanelle will bulge milestones due to ataxia and/or weakness. uncus happens very late in the process
slightly when the infant is lying down. It will Personality changes may occur. and is not responsible for most of the signs.
become depressed when the child is sat up. As pressure increases, the pressure itself Features in approximate sequence are:
It will bulge more prominently when the may cause focal neurological symptoms contralateral hemiplegia;
infant is crying or straining. The normal fon- and signs. These may be due to several drowsiness;
tanelle will have an arterial pulsation more mechanisms, which include: ipsilateral pupillary dilatation (partial 3rd
apparent when the infant is upright. nerve palsy);
The fontanelle also provides access for
impingement on, or disruption of, a
cranial nerve, cranial nerve nucleus or complete 3rd nerve palsy as pressure
emergency procedures such as the draining increases (can be bilateral);
higher centre;
of a traumatic subdural or a ventricular tap. ipsilateral hemiplegia (midbrain being
Fortunately the need for these procedures in
impingement on a blood vessel supplying
any of the above; pushed against edge of tentorium);
the emergency department (ED) rarely Cushings triad.
arises. When it does, however, it should be
in hydrocephalus, stretching of
corticospinal tract fibres around
done by a neurosurgical specialist (or
enlarging ventricles causes upper motor Cerebellar tonsillar herniation
trainee if sufficiently experienced). In cen-
neuron signs in the legs; syndrome
tres where such help is not rapidly available,
over-the-phone advice from a neurosurgeon
focal seizures may accompany raised A wide variety of signs and symptoms arise
ICP and leave an infant with a Todds from posterior fossa masses. However, some-
may help with both the decision to do the
paresis. times as the cerebellar tonsils are pushed
procedure and the technique.
through the foramen magnum and the
In the more rapid onset conditions, such as
medulla with its respiratory control centres
intracranial haemorrhage, rapid onset of
is compressed, the following sequence
drowsiness and vomiting, with or without
Clinical features of raised occurs:
focal neurology, is the rule. When there is
intracranial pressure a large intracerebral pressure differential patient complains of a stiff neck;
The symptoms and signs that lead to a diag- across a fixed structure, such as the tentor- drowsiness;
nosis of raised ICP (RICP) will vary with the ium, the brain will herniate. There are sev- nystagmoid eye movements;
age, severity and rate of development. In eral herniation syndromes. apnoea.

200
8.2 RAISED INTRACRANIAL PRESSURE
8

NEUROLOGY
In this situation efforts should be directed
Other examination findings Investigations towards maintaining cerebral oxygenation
in raised ICP Imaging the brain will often reveal the and perfusion by supporting the childs venti-
underlying pathology in children with raised lation and circulation, if necessary using rapid
General observation
intracranial pressure. However, there are sequence intubation (RSI) and mechanical
In the unco-operative irritable child, general
several drawbacks that must be borne in ventilation. Some drugs used in RSI and the
observation from a distance is a vital part of
mind when deciding to obtain a scan. manipulation of the airway itself may cause
the examination. It may reveal a large and/
One must be sure that it is safe to move the a further transient rise in intracranial pres-
or an asymmetric head, evidence of trauma,
child to the scanner room where facilities for sure. Several strategies have been used to
gait, speech or visual disturbance. Watching
resuscitation and access to the patient are less avoid this transient rise including administer-
a playful child from a distance will yield a
than ideal. CTor MRI scans may be falsely reas- ing an IV dose of lignocaine just prior to RSI
lot more useful neurological information
suring, especially in the case of pseudotumour and giving a defasciculating dose of a non
than an attempted formal examination of
cerebri (PTC or benign intracranial hyperten- depolarising neuromuscular blocking agent
a screaming unhappy one.
sion) or meningitis, where the appearance just before the Succinylcholine. There are
may be normal even when the pressure is dan- no studies looking at what effect the use of
Fundi gerously high. To keep an unco-operative child these strategies has on patient outcome. This
Although often difficult to visualise in still may require a general anaesthetic, with its authors opinion is that it is more important
children, fundal examination is of vital concomitant risks. However, as scanning tech- to avoid hypotension and hypoxia during
importance. Using a parent as a visual dis- nology improves, the time required for a scan RSI because there is strong evidence that
traction will often help. With patience, the is getting dramatically shorter. Many infants these are harmful. Therefore this author does
disc can usually be visualised through an can often be successfully scanned with the not use these strategies because it is a
undilated pupil. One may be able to visualise use of a dummy (comforter) dipped in a change in the usual practice of the depart-
retinal venous pulsations. The implication of sucrose solution and firm wrapping. Likewise, ment which more likely lead to errors and
these pulsations is that intracranial pressure in older children, the presence of a parent in delays in the RSI process. (See the end of
is less than peak venous pressure. Papilloe- the scanner room may permit the avoidance the chapter for further reading on this issue).
dema is less common in infants with raised of anaesthesia. Otherwise the decision to Intracranial pressure can be reduced by giv-
ICP due to the decompressing presence of use general anaesthesia or procedural seda- ing intravenous mannitol 1 g kg1 and
an open anterior fontanelle. Examination tion depends on the clinical situation, local nursing the patient in a 30 head up position.
of the fundi is less useful in acute severe policy and skill mix, with safety always being Mannitol should not be given if the patient
raised ICP, as papilloedema takes days to the first consideration. has circulatory failure (i.e. hypotension or
evolve. Other retinal findings associated An MRI scan is more likely to require hypoperfusion) as the osmotic diuresis that
with raised ICP include retinal haemor- anaesthesia in younger children, as they it causes could exacerbate the hypoperfusion.
rhages, which have a strong association with usually take longer and patient access is Just as in trauma so in medical illnesses C
non-accidental injury, aneurysms and arte- more difficult. Finally, when obtaining CT (circulation) comes before D (disability)
riovenous malformation that may be asso- scans, one must consider the risks of radia- meaning that a perfusing blood pressure
ciated with similar intracranial lesions, and tion causing cancer, which in a CT scan of must be maintained if there is to be any
subhyaloid haemorrhages which may be the head in a child is estimated to be cerebral perfusion pressure. There is strong
seen in patients with subarachnoid haemor- 1:10001:10 000 over the lifetime of the evidence against prolonged hyperventilation
rhages. In some instances it may be useful to child.1 Head ultrasound is an option for ini- because vasoconstriction impairs cerebral
ask an ophthalmologist to examine the child tial imaging in infants. This is good at imag- perfusion despite the reduction in intracra-
through dilated pupils. ing the lateral ventricles and surrounding nial pressure. pCO2 should be kept between
structures but dependent on operator expe- 35 and 40 mmHg unless the above measures
rience. However, ultrasound is less able to have been unsuccessful or herniation is pro-
Peripheral neurological signs image the subdural space around the vertex gressing, in which case the pCO2 can be taken
The development of handedness is not and the posterior fossa region which may down to 25 mmHg for a brief period while
apparent in normal infants under 1 year of need to be considered in the adequacy of further measures are commenced. Whilst
age. If handedness is apparent in infancy, the clinical scenario. these measures are taking effect consider-
it is usually due to a neurological or muscu- ation and management of the underlying
loskeletal lesion, some of which are asso- Management of raised ICP cause should be undertaken. This will be
ciated with raised ICP. Hydrocephalus may Acute severe increases in ICP are a true aided by a concise history and some rapid
produce lower limb signs with gait distur- emergency and children with features of bedside testing such as blood gas, which
bance in the ambulant child. In infants, this the herniation syndromes should be trea- may reveal conditions such as hyponatraemia
may be apparent when the child is held up ted in the resuscitation area of the ED or any infusions containing free water. If an
by hands around the chest with the legs sus- or the neurosurgical operating theatre. A infant in this situation does not respond to
pended in midair. Spasm of the adductors neurosurgeon should be consulted as early these measures, a neurosurgeon should be
will cause scissoring of the lower limbs. as possible. urgently consulted regarding a ventricular

201
8.2 RAISED INTRACRANIAL PRESSURE

or subdural tap via the open fontanelle. In for disturbances that may lead to cerebral and later progresses with erosion of the
older children who do not respond ade- oedema. Imaging should be considered. If peripheral visual fields. Left untreated the
quately, the options depend on the clinical cerebral oedema is confirmed or not excluded patient with persistent PTC will eventually
situation and the underlying cause. Thera- then the child should be treated as above and develop optic atrophy and blindness.
peutic options include: consulting neurosur- observed in a paediatric intensive care unit. Investigations include CT scan or MRI,
geons and getting an urgent CT scan; followed by lumbar puncture to measure
palliating or treating an underlying cause opening pressure. Lumbar puncture may
urgently (e.g. high-dose dexamethasone for Pseudotumour cerebri (PTC or be both diagnostic and therapeutic. The
vasogenic oedema or hypertonic saline for benign intracranial pressure) CSF has a high pressure, but analysis reveals
hyponatraemia); or maintaining cerebral per- This condition is characterised by sustained normal protein, glucose, cell count and no
fusion pressure by maintaining mean arterial raised ICP causing symptoms similar to a microorganisms.
blood pressure at above normal levels with cerebral tumour but with no anatomical Therapeutic CSF taps should aim to
fluids and inotropes and reducing cerebral abnormality on neuroimaging. It is usually decrease the ICP by 50% under the guid-
metabolic demand with drugs such as thio- due to decreased CSF re-absorption. PTC ance of a paediatric neurologist. This may
pentone. Other neurosurgical interventions has many causes (Table 8.2.2). It is more be curative. However, in most cases repeat
include: craniotomy; insertion of external ven- prone to occur in overweight pubescent lumbar punctures and/or acetazolamide
tricular drain; and insertion of a CSF reservoir girls, in whom no cause is found. The presen- are required. It is believed that acetazol-
or a complete CSF shunt. Where a shunt is tation is usually with headaches and vomit- amide works by reducing CSF production.
already present this may be tapped (see ing, which are worse in the morning. Some In severe cases a lumboperitoneal shunt
Chapter 8.1 on shunt complications). Consid- patients may complain of transient visual or optic nerve sheath fenestration may
eration should also be given to prophylactic obscuration with blurring or darkening of be required. One needs to exclude any
anticonvulsants because seizure will cause vision that lasts less than 30 seconds. treatable underlying cause. Anticoagulants
a further acute rise in ICP. Once initial stabi- Later there may be unilateral or bilateral should be given for venous thrombosis and
lisation is underway and neurosurgeons are 6th nerve palsies causing diplopia on lat- drugs such as glucocorticoids should be
involved referral to a paediatric ICU is eral gaze. Papilloedema is often the only ceased or weaned if possible. Referral to a
mandatory. positive physical finding. The most signifi- paediatric neurologist is mandatory.
Children with less severely raised ICP usu- cant complication of PTC is loss of vision.
ally need investigation and treatment of This begins with an enlargement of the
the cause, usually under the care of paedia- blindspot associated with papilloedema,
tric neurosurgeon whilst avoiding interven- Reference
1. Brenner DJ, Elliston CD, Hall J, et al. Estimated risks of
tions that may increase ICP. One should
Table 8.2.2 Causes of pseudotumour radiation-induced fatal cancer from pediatric CT. AJR
avoid the infusion of hypotonic fluids for a cerebri 2001;175(2):28996.
vomiting child or the administration of cer-
1. Idiopathic
tain drugs. Children with hydrocephalus will 2. Venous obstruction
usually require admission for consideration 3. Metabolic
Hypervitaminosis A
of a shunt, or 3rd ventriculostomy. Hypoparathyroidism
Further reading
Addisons disease Behrman RE, Kliegman R, Jenson HB, editors. Nelson
Obesity Textbook of Pediatrics. 16th ed. Philadelphia: WB
Pregnancy Saunders; 2000.
Galactosaemia Bisan Salhi, et al. In defense of the use of lidocaine in rapid
4 Drugs sequence intubation. Annals Emerg Med 2007;49(1):856.
Some particular causes Oral contraceptive pill Clancy M, Halford S, Walls R, Murphy M. In patients with head
of raised ICP Glucocorticoids
Tetracyclines
injuries who undergo rapid sequence intubation using
succinylcholine, does pretreatment with a competitive
Isotretinoin neuromuscular blocking agent improve outcome? A
Iatrogenic Nalidixic acid literature review. Emerg Med J 2001;18:3735.
Any child receiving intravenous fluids, espe- Nitrofurantoin Roberts JR. Clinical Procedures in Emergency Medicine. 3rd ed.
5 Haematologic Philadelphia: WB Saunders.
cially for treatment of diabetic ketoacidosis Anaemia Robinson N, Clancy M. In patients with head injury
(DKA), who develops headache and/or Polycythaemia undergoing rapid sequence intubation, does pretreatment
6 Infections with intravenous lignocaine/lidocaine lead to an improved
drowsiness and/or seizures during therapy Roseola infantum neurological outcome? A review of the literature. Emerg
should be considered to have raised ICP until Chronic complicated otitis media Med J 2001;18:4537.
7 Others Vaillancourt Christian, et al. Opposition to the use of lidocaine
proven otherwise. A thorough assessment GuillainBarre A syndrome in rapid sequence intubation. Annals Emerg Med 2007;49
should be made and electrolytes checked (1):867.

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NEUROLOGY
8.3 Seizures and non-epileptic events
Padraic Grattan-Smith

seizures, where the seizure arises from one

ESSENTIALS area in the brain. In simple partial seizures
there is no alteration of consciousness. With
1 Seizures are the most common life threatening emergency presentation to EDs complex partial seizures, alteration of con-
requiring immediate management.
sciousness occurs. Partial seizures may
2 The diagnosis of a seizure is based on a careful history. spread and become secondarily generalised.
Status epilepticus is said to occur where a
3 There are many paroxysmal events or funny turns in children, which can mimic seizure lasts more than 30 minutes or there
seizures.
is incomplete recovery between seizures
4 A bedside dextrostix should be performed on the child who is having a seizure. over this time period. In practical terms,
First-ever seizures should have blood sent for formal glucose, electrolytes, calcium, any child who arrives in the ED still fitting,
magnesium and phosphate levels. should be regarded as a medical emer-
gency. Status epilepticus can occur in both
5 The acute management of the child having a seizure involves supporting airway convulsive and non-convulsive forms.
and breathing and the graduated use of antiepileptic drugs to terminate the seizure.
6 Parental reassurance and education regarding seizures, BLS and safety issues are
an important facet of management in the child presenting with a first convulsion.
Febrile seizures
Febrile convulsions are not regarded as a
A family history of premature unexplained form of epilepsy but are discussed because
Introduction of their frequent presentation to EDs.
sudden death in a young relative should also
An epileptic seizure can be defined as a dis- raise this possibility. Typically they occur in children between
turbance in neurological function, resulting Some variants of migraine may be difficult 6 months and 6 years of age. They are com-
from uncontrolled excessive neuronal activ- to distinguish from seizures. Acute confu- mon, affecting around 3% of children. Most
ity in the central nervous system. sional migraine often follows minor head children who have febrile convulsions have
The subject of seizures and their differen- trauma. The episodes may last for a number only one, but 2530% will have a recur-
tial diagnosis is large. This chapter provides of hours. The child is concussed, confused rence. The risk of recurrence is greater in
essential information that may be useful in and often vomiting. Basilar artery migraine infants less than 12 months, where it may
formulating diagnosis and treatment in is associated with confusion or loss of con- be as high as 50%.
the emergency department (ED). There is sciousness and brainstem symptoms and Febrile convulsions most commonly occur
insufficient space for a detailed discussion signs. Neither diagnosis should be made at in the setting of viral illnesses such as an
of the role of the electroencephalogram the first presentation and without further upper repiratory tract infection, pharyngitis,
(EEG) and other investigations. investigation. On the other hand, occipital gastroenteritis, or an exanthem such as rose-
epilepsy can cause vomiting, visual loss and ola infantum. Much less commonly, pneumo-
severe headache. The presence of tonic eye nia or a urinary tract infection may be the
deviation early in the event is a strong clue underlying cause. Febrile seizures typically
General comments
that this is a seizure rather than migraine. occur relatively early in the course of an
There are a number of conditions that must Pseudoepileptic seizures and Munchausens infectious illness and sometimes the convul-
always be considered in children experiencing syndrome by proxy always need to be consid- sion is the first sign that the child is unwell.
unusual events. Hypoglycaemia can cause ered in the differential diagnosis of atypical Most febrile seizures are generalised and
both partial and generalised seizures. It may epileptic seizures. However, the diagnosis is brief, lasting less than a few minutes. The
also illogically produce focal neurological often difficult and the ED is usually not an child returns to normal after a short (usually
signs. Abnormalities of electrolytes, calcium, appropriate setting to make a confident less than 30 minutes) postictal period. The
magnesium and phosphate can present in a diagnosis of either of these conditions. seizures may be clonic, tonicclonic or atonic
similar fashion. Cardiac arrhythmias asso- where the child may simply seem to stop
ciated with prolonged QT interval may cause breathing. Examination should confirm the
epileptic seizures that seem to follow the presence of a fever, identify the source of
Classification of seizures
arrhythmia. Sudden loss of consciousness dur- the fever, and exclude signs of central
ing, or immediately after, intense exercise or Seizures are conceptually divided into gener- nervous system (CNS) infection or of a focal
associated with strong emotion, raises the alised seizures where the onset is from all neurological process. Most children with an
possibility of an underlying cardiac cause. brain regions simultaneously and partial uncomplicated febrile seizure who have

203
8.3 SEIZURES AND NON-EPILEPTIC EVENTS

completely recovered and have a clear obvious limitations to the history, but it Infancy
source of infection require no blood tests remains the single piece of information most Seizure types
or other investigations. likely to provide a diagnosis. It is also worth Infantile spasms Typically take the form
Prolonged (lasting >10 minutes) and talking to the verbal child about what hap- of salaam seizures. There is characteristic
complicated (focal, multiple) febrile seizures pened. Even young children can sometimes sudden flexion of the arms, head and trunk
can occur. Underlying meningitis/encephali- give very helpful accounts if spoken to soon in a brief spasm. Asymmetrical or extensor
tis must always be considered in the child after the event. spasms can occur. The characteristic feature
who fails to return to normal, has multiple It is important to establish whether there of established infantile spasms is that the
or prolonged seizures or has residual neuro- may have been provocative factors, such as episodes occur in runs (clusters) which can
logical signs. The threshold to perform a cer- sleep deprivation, a febrile illness, head continue for 10 minutes or more. In the early
brospinal fluid (CSF) examination is lower trauma or potential exposure to epilepto- phases, the spasms may be mild and rela-
when the child is less than 12 months of genic medications. A past history of similar tively infrequent, making diagnosis difficult.
age or has been on antibiotics, which may events should be sought. It is also important
mask the usual signs of meningism. How- to ask about a family history of seizures or LennoxGastaut syndrome In this syn-
ever, lumbar puncture can be dangerous in unusual turns and to review the childs devel- drome there are multiple seizure types,
the child with focal signs such as a hemipar- opmental milestones. including atypical absences, myoclonic sei-
esis or if there has been a rapid deterioration zures, drop attacks, nocturnal tonic seizures
in conscious level or an abnormality of respi- and generalised tonicclonic seizures. In the
ratory rhythm. Consultation with senior col- early phases, which can be seen in late
leagues is advised if there is doubt about the infancy, the child may present with atypical
safety to perform a lumbar puncture.
Examination absences. These have somewhat slower
Children with seizures are seen in the ED The physical examination is often normal, onset than typical absences and there may
in two broad settings: but it must be carefully performed, looking be obscuration rather than complete loss
for fever, evidence of intercurrent illness of consciousness. Great caution should be
The child who is seen after an event. Was
taken with any child presenting in the first
and abnormal neurological signs. Other
this a seizure?
important diagnostic clues include a Todds 2 years of life with absences, as this may
The child who is still fitting.
be an early manifestation of LennoxGas-
paralysis, skin lesions of tuberous sclerosis
or morphological features of a chromosomal taut syndrome.
disorder or other underlying neurological
THE CHILD SEEN AFTER Complex partial seizures in infancy
abnormality associated with a seizure disor-
THE EVENT der. The finding of lateral tongue trauma or May present as episodes of altered con-
incontinence supports a suspicion of seizure sciousness, with autonomic symptoms and
when the diagnosis is unclear. The febrile also with apnoea. An underlying tumour
History (most often in the temporal lobe) may cause
child needs to have an underlying meningi-
The diagnosis of seizures is based almost tis or encephalitis excluded. this type of seizure.
entirely on the history. In infants, the sudden onset of frequent
The parents or any witnesses of the event seizures and failure to regain consciousness Non-epileptic events of infancy
should be taken through the episode sys- is a common presentation of child abuse and The following events can mimic seizures in
tematically. An exact description of what a careful retinal examination must be per- young children and are usually distinguished
took place is crucial in making a correct formed, looking for retinal haemorrhages. by a careful history.
diagnosis. What was the child doing when
Breath-holding spells
it started? What was the very first sign of
These are always provoked by an unpleas-
a problem, and what followed? If abnormal
ant stimulus. This is usually minor trauma,
movements were present, did they princi-
pally involve one side of the body? How long
Differential diagnosis e.g. a bump on the head, but the child
being scolded or frustrated may provoke
did the event last? What was the child like Tonicclonic seizures, myoclonic seizures
them. Somewhat arbitrarily, breath-holding
afterwards? How long before the child was and clonic seizures, in general, can occur
attacks are divided into:
back to normal? Was there any faecal or uri- at any age. They are well described in stan-
nary incontinence or tongue biting? Was dard textbooks and will not be discussed Pallid breath-holding attacks, where
there any apnoea or colour change? here except to discourage the tendency to after a short cry, the infant loses
Witnessing a sudden loss of consciousness loosely label any event with loss of con- consciousness, becomes pale and there
in a child is an extremely upsetting event for sciousness as a tonicclonic seizure. may be tonic stiffening.
any observer, particularly the parents. Many Although there is overlap, it is helpful to Cyanotic breath-holding attacks, where
parents report that they thought their child approach the problem in terms of age of pre- after vigorous crying, there is breath-
had stopped breathing and commenced sentation. For neonatal seizures refer to holding in expiration and loss of
various resuscitation techniques. There are Chapter 2.6. consciousness.

204
8.3 SEIZURES AND NON-EPILEPTIC EVENTS
8

NEUROLOGY
As in syncopal episodes there may be brief Childhood by sudden bursts of exercise. There is no alter-
clonic jerking after an episode. The story of Seizure types ation of consciousness.
provocation is usually clear, but at times Absence seizures Are usually brief star-
there can be diagnostic confusion, for exam- ing spells characterised by a sudden loss of Late childhood and adolescence
ple, when a child who is playing happily is consciousness and an abrupt end to the sei- Seizure types
found unconscious on the floor and no pre- zure. There is no post-ictal period. With lon- Juvenile myoclonic epilepsy This usu-
cipitating event was observed. ger absences, automatisms may occur. The ally develops in the early teenage years. Typ-
events usually last less than 10 seconds, ically there are myoclonic jerks in the
and multiple episodes may occur per day. morning soon after awakening. These may
Benign neonatal sleep myoclonus
not be mentioned to the parents. The first
This is characterised by myoclonic jerks,
Complex partial seizures There may be presentation is often with an early morning
which can be quite violent and asymmetric,
an aura (actually a partial seizure), a more generalised tonicclonic seizure. A history of
but are confined to sleep. The infant is oth-
prolonged episode of altered consciousness early morning myoclonus should always be
erwise normal.
in which the child may be partially respon- sought in this situation.
sive and a post-ictal period characterised
Benign paroxysmal vertigo by drowsiness and a desire to sleep. Non-epileptic events
Sudden episodes of acute unsteadiness Syncope Is usually a vasovagal response
occur without alteration of consciousness. Benign focal epilepsy of childhood In provoked by a stressful situation, such as
The child typically clutches onto whatever its typical form, presents with the child awak- seeing blood or standing for a prolonged
is nearby. The episodes are usually brief, ing from sleep at around 24 a.m. making a period in hot weather. There is a feeling of
lasting seconds to minutes. The parents glugging or clucking noise. This is followed light-headedness, nausea and then a pro-
may notice nystagmus during an episode. by a clonic seizure involving one side of the gressive fading out of vision. As mentioned
There may be multiple episodes per day. body, including the face. The child may be above, there may be brief stiffening and
awake during the seizure and find it very clonic jerks in the course of syncopal epi-
frightening. Not uncommonly, this seizure sodes. Unusual forms of syncope in adoles-
Shuddering attacks becomes secondarily generalised. cence include stretch syncope provoked by
The infants body shivers as though cold neck hyperextension and shoulder abduc-
water had been poured on its back. tion while stretching and hair grooming syn-
Nocturnal frontal lobe seizures Are rare
but may be mistaken for night terrors and cope, which seems to be provoked by strong
pseudoseizures. Seizures should be consid- pulling of the hair while combing or brush-
Self-stimulatory episodes (infantile
ered if there are multiple stereotyped events ing. Children with intellectual handicap
masturbation)
occurring in the one night, if the episodes may repeatedly provoke episodes of loss of
These are seen in girls. The thighs are gen-
are very brief or if they occur only in the sec- consciousness by hyperventilating and then
erally clenched tight, with the legs crossed
ond half of the night. performing the Valsalva manoeuvre.
at the ankles. There are pelvic undulatory
movements. The infant may look extremely
flushed and upset and may be sleepy Non-epileptic events of childhood: Rage attacks These consist of sudden epi-
afterwards. Night terrors Usually occur within the first sodes of directed anger, usually provoked by
few hours of the child going to sleep. Typically being thwarted in some way. A careful his-
the child screams and is found sitting up in tory will usually differentiate a rage attack
Stereotypies bed with widely dilated pupils and appears from non-directed aggression, which can
There are hand-flapping or other repetitive to be extremely fearful. This may last for half occur in the post-ictal state. This usually
limb movements. These are usually repeated an hour or more. The child is eventually com- occurs when a patient in a confused post-
in exactly the same way each time. Although forted and goes off to sleep. The next morning ictal state is restrained or surrounded by a
common in children with autism, they also the child has no memory of the event. crowd of people.
occur in children who are otherwise normal.
Nightmares In contrast, these usually
occur in the second half of the night. The
THE CHILD WHO IS STILL
Day-dreaming
child goes into the parents bedroom afraid FITTING
Is common at all ages in childhood. The child
may stare straight ahead and at times not because of a bad dream. The child usually
respond to being called. has a good memory of the dream, although
he/she may not want to discuss it.
The child with convulsive
In daydreaming, stereotypic behaviours
status epilepticus
and self-stimulatory episodes, the event
can usually be immediately stopped by Paroxysmal kinesigenic dyskinesia When a child is fitting on arrival at the ED,
physical interaction with the child, e.g. Brief episodes of dystonic or choreoathetoid the seizure is likely to have continued for
by tickling them. movements involving the limbs are provoked at least 15 minutes. There is a risk of brain

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8.3 SEIZURES AND NON-EPILEPTIC EVENTS

damage or death if the seizure is not rapidly Second line metabolic causes have been excluded,
controlled and respiration appropriately If after another 5 minutes the fit con- waking up soon after an IV dose of a benzo-
supported. There needs to be a clear plan tinues, IV phenytoin 20 mg kg1 as an diazepine is a useful diagnostic clue of non-
for the immediate management of the con- infusion over 30 minutes or IV phenobarbi- convulsive status.
vulsing child. The child should be taken tal 1015 mg kg1 as an infusion over
directly to the paediatric resuscitation area 30 minutes should be given. These second-
with ready access to anaesthetic and airway line agents need to be given slowly by
resuscitation facilities. intravenous infusion, and therefore will take Investigations
Airway, breathing and circulation need to time to control the seizure. If continued
be assessed and simultaneously managed. seizure activity is causing respiratory compro- These depend on the clinical setting but
The child should receive high-flow oxygen mise or it is felt their severity may result in some general rules apply.
via mask or assisted by bag and mask, if ven- brain injury, it may be necessary to use
tilation is inadequate. Basic airway man- further benzodiazepines and be prepared
oeuvres and suctioning may be required. to support respiration or to proceed to use Blood tests
Oxygen saturation should be monitored barbiturates, which require intubation. All children having a seizure should have a
continuously. Intravenous access should be bedside Dextrostix followed by a formal
gained and a Dextrostix checked immedi- blood glucose to exclude hypoglycaemia.
ately to exclude hypoglycaemia. Blood Third line At the same time full blood count, electro-
should be taken for glucose, electrolytes, If there has been no response after a further lytes, calcium, magnesium and phosphate
calcium, magnesium and phosphate and full 5 minutes, thiopental 5 mg kg1 and intuba- levels should be checked in order to exclude
blood count. Children on maintenance anti- tion using rapid sequence induction, is the a metabolic cause. These are exceedingly
convulsants should have their drug level next step. The decision to intubate to con- rare beyond the infant age group in the child
taken. trol a seizure needs to involve consideration who has returned to normal and the yield is
At the same time, a history should be rap- not only of the length and severity of the sei- negligible, unless there are other clues to
idly obtained regarding: zure but also the degree of respiratory promote an electrolyte disturbance.
embarrassment to the child. Mild seizure
the duration of the seizure; activity that is not compromising a child
previous episodes (and if there is a can be tolerated longer than a major convul- Neuroimaging
particular drug most likely to be
sion that is impairing oxygenation. These As a general rule, patients with focal seizure
effective);
children will need to be subsequently man- or examination findings, features suggesting
precipitating events;
aged in a paediatric intensive care unit. raised intracranial pressure or seizures in the
medications the child is taking;
There are almost endless variables in this setting of trauma, should have urgent neu-
whether drugs have already been given
situation. If intravenous access is a problem, roimaging. The computerised tomography
for the seizure;
midazolam 0.15 mg kg1 may be given scan of the brain has the advantage of rela-
the presence of underlying neurological
intramuscularly, or into the nose or buccal tive ease of access but it should be remem-
problems.
space, or diazepam 0.5 mg kg1 may be bered that the magnetic resonance scan is a
given rectally. Intraosseous injection can better test in almost all instances except
be used. If the child has already received a traumatic brain injury.
benzodiazepine beforehand, a second dose
may be given on arrival, followed by phenyt-
Drug therapy oin as it is less likely to cause respiratory EEG
If the child has received no treatment for depression than the combination of pheno- An urgent EEG is very useful in cases where
the seizure prior to arrival, the following barbitone and a benzodiazepine. convulsive status epilepticus has been trea-
approach is suggested. In a severely handicapped child who has ted but the patient remains unconscious and
frequent bouts of status epilepticus where there is a suspicion of continuing non-
there is a desire to avoid intubation, paralde- convulsive status. It is also very helpful in
hyde 0.4 mL kg1 rectally may be tried if the child presenting with an altered state
First line there has been no response to benzodiaze- of consciousness and absence or complex
Initial treatment is with benzodiazepines, pines, phenytoin or phenobarbital. partial status is suspected. In the child
which are rapidly acting, using intravenous Absence, atypical absence and complex who has recovered from a brief seizure an
(IV) diazepam 0.25 mg kg1 or IV midazo- partial status are much less common than EEG done soon after presentation may be
lam 0.15 mg kg1. If the seizure has not convulsive status but should be considered useful, e.g. showing focal changes that sug-
stopped within 5 minutes the dose can be in children who are obtunded or have bizarre gest a secondarily generalised seizure. There
repeated. The potential side effects of respi- behaviour without obvious cause. If there is is no reason to delay getting an EEG soon
ratory depression or apnoea need to be doubt an EEG should be performed. If an after a fit but this will be limited by the
anticipated. EEG is unavailable and CNS infection and resources that are available.

206
8.4 ACUTE WEAKNESS
8

NEUROLOGY
with the parents and provide some reassurance
Disposition to be increasingly used in the acute
that it has not caused brain damage. First-aid
management of seizures.
Children who have prolonged or multiple measures should be discussed with the parents
seizures, focal seizures, or abnormal neuro- so that they have a clear plan should their child  Given the efficacy and safety of
logical examination, or where the diagnosis have a further seizure at home. The parents midazolam by the IM or IN route, many
is unclear, should be admitted for observa- should be warned that further seizures can clinicians argue that there is no place for
tion and paediatric or neurological review. occur. It is important to take care that the child rectal diazepam, given its impracticality
Children who are having increased fre- is not in a potentially dangerous situation and unpredictable absorption by this
quency of seizures or are known to have should this happen. A particular risk is swim- route.
prolonged, or clusters of seizures warrant ming without careful adult supervision. This
admission and observation until stabilised. advice should be reviewed at the organised
Not all children presenting with an follow up. Children who are discharged after
uncomplicated first seizure need to be a febrile convulsion should be reviewed by Further reading
admitted to hospital. Those who have a first their local doctor within 24 hours to follow Chin RF, Neville BG, Scott RC. Meningitis is a common cause of
generalised seizure, with normal neurologi- progress and reinforce the safety issues. convulsive status epilepticus with fever. Arch Dis Childhood
2005;90:669.
cal examination and metabolic work up, Maytal J, Shinnar S, Moshe SL, Alvarez LA. Low morbidity and
mortality of status epilepticus in children. Paediatrics
can be followed up on an outpatient basis. 1989;83:32331.
This is usually best achieved by discussion Controversies and future Mitchell W. Status epilepticus and acute repetitive seizures in
children, adolescents and young adults: Etiology, outcome,
with a general paediatrician or neurologist directions and treatment. Epilepsia 1996;37(Suppl 1):S7480.
colleague to determine the timing of imag-
Unlike in other countries, the
Sofou K, Kristjansdottir R, Papachatzakis NE, et al.
Management of prolonged seizures and status epilepticus
ing and an EEG and formal review. Other in childhood: a systemic at review. J Child Neurol
intravenous form of sodium valproate
influences on the decision to admit may 2009;24:91826.
has not been used widely (if at all) in The Status Epilepticus Working Party. The treatment of
include the age of the child, parental anxiety convulsive status epilepticus in children. Arch Dis Child
Australia.
and any intercurrent illness issues. 2000;83:4159.
If the event has clearly been a brief seizure, it In the future, newer intravenous Verity CM, Ross EM, Golding J. Outcome of childhood status
epilepticus and lengthy febrile convulsions: Findings of
is important to discuss the nature of this agents such as levetiracetam may come national cohort study. Br Med J 1993;307:2258.

8.4 Acute weakness

Richard Lennon

ESSENTIALS Introduction
1 The fact that a child has acute weakness may be obscured by an inability to The acutely weak child is challenging to the
communicate and a smaller repertoire of activities. The possibility of a neuromuscular emergency physician because she/he can
problem must be consciously considered by the clinician. present in many different ways with many
different diagnoses. In particular, the prever-
2 Conversely, a small child may not move a limb because of pain rather than bal child may present with only regression in
weakness. A history and examination for evidence of trauma should be performed and motor milestones or bruising on the face
appropriate diagnostic imaging considered. from increased frequency of falls. The diag-
3 Respiratory insufficiency due to weakness may occur with surprising rapidity and nosis in such children can range from a tick
may be compounded by aspiration. Careful assessment and close monitoring for this bite to Duchenne pseudohypertrophic mus-
complication must be made. cular dystrophy.

4 Abnormal reflexes are important in localising the cause of acute weakness. If some
are absent, especially distally, it suggests a lower motor neuron dysfunction. If all are
present it suggests muscle dysfunction and if they are increased it suggests an upper Presentation
motor neuron weakness.
In infants of less than 3 months, parents are
5 The presence of a sensory level indicates a spinal cord lesion. One should consider usually quite sensitive to abnormalities of
investigating this with an urgent MRI to determine whether or not urgent surgery is the infants behaviour. They may bring
needed. a baby in complaining of poor feeding,
decreased activity or being floppy. The older

207
8.4 ACUTE WEAKNESS

infant may be noted to lose milestones such age should be estimated. The size of the adduction of lower limbs. Autonomic dys-
as ceasing to crawl or walk. Ataxia may be childs head and its proportion to the body function may lead to associated fever or
the predominant feature in preschool and should be noted and centiles should be plot- hypothermia. In any child with weakness
school-age children. Sometimes children ted later in the detailed examination phase. one needs to consider whether the findings
may present with an associated feature of ill- could be caused by a toxidrome (e.g. organ-
ness, such as the rash of dermatomyositis or ABC ophosphate poisoning).
an obvious engorged tick. In general, the The effects of weakness may include airway Once the primary survey has been com-
older the child is, the more typical the pre- compromise from bulbar palsy (e.g. hoarse pleted, and any abnormalities corrected,
sentation. However, even in this group, as voice, stridor, and aspiration of secretions). then a detailed history and examination
in adults, there is a wide range of presenta- Severely impaired ventilation will be clini- should be performed.
tions, e.g. cranial nerve palsies in the Miller cally apparent. However, mild or moderate
Fisher variant of GuillainBarre syndrome. impairment can be subtle and can rapidly
progress to cause respiratory embarrass- History
Trauma masquerading ment. Therefore, if possible, respiratory func-
If, on questioning, the weakness has been
as weakness tion tests should be serially performed on all
chronic then the list of possible diagnoses
Trauma is dealt with in detail in other chap- children presenting with neuromuscular
is extensive and is beyond the scope of this
ters of the book. However, it should noted weakness. Arterial or venous blood gases text. Such children should be stabilised and
that non-accidental injury (NAI) can present should also be considered in this assess- referred to the appropriate paediatric ser-
as acute weakness in the infant and young ment. (Table 8.4.1 for predictors of needing
vice for diagnosis. However, it is possible
child. Shaken baby syndrome may present intubation and mechanical ventilation.) that a child with chronic weakness could
as a lethargic irritable child with little or Circulatory defects may arise from distur- undergo an acute deterioration, such as
no bruising. The apparent focal weakness bance of the autonomic nervous system. influenza in a child with a Duchenne muscu-
may be due to an underlying fracture. Like- This is characterised by labile blood pres- lar dystrophy. In infants the distinction
wise, emotional and nutritional neglect sure, heart rate and postural hypotension. between acute and chronic weakness is less
may also lead to weakness. Hence, NAI must relevant. In those children with conditions
be considered in the differential diagnosis of Disability covered by this chapter, the history needs
the acutely weak child (see Chapter 18.2). The neurological part of the primary survey to focus initially on finding treatable causes.
can give early hints as to the site of the Detailed enquiry should be made about any
lesion(s) involved. The classic description possible tick bites or other venomous bites
Primary survey approach of peripheral neuromuscular weakness is or stings. Enquiry should also be made about
an alert, but anxious looking, child that the availability of various medications and
General inspection has a paucity of limb movements. If respira- poisons around the house.
This is done by looking for both cause and tory failure or facial weakness have inter- A precise time course for the illness
effect of the weakness. An abnormality of vened this appearance will not be present. should be obtained, along with the pattern
the primary survey severe enough to cause Conversely, intracranial causes of weakness of evolution of the weakness. Whether it is
generalised weakness (e.g. respiratory dis- are usually associated with some degree of ascending from lower limbs up, whether it
tress) is usually the obvious presenting prob- obtundation. is lateralised and whether it has progressed
lem. Hypoglycaemia in a child can, in rare The weak infant classically lies in a frog or not. A sudden onset may suggest a vascu-
circumstances, present with focal weakness. leg posture with all limbs lying on the bed, lar or epileptic event. Rapid onset weakness
It is important in the weak, floppy infant to abducted at the hips and flexed at the knees. may follow an intoxication or envenom-
consider the possibility of intussusception Asymmetry may be apparent in those with a ation. Other causes are more subacute and
or other intra-abdominal pathology. In an in- focal weakness. With central causes of weak- may have progressed over weeks. The family
fant with intussusception, prostration mim- ness (e.g. acute hydrocephalus) hypertonic history should be reviewed, and whether
icking severe weakness and lethargy may posturing may be seen, with scissoring there is consanguinity. A history of recent
be the only presenting sign. When one comes
infectious illnesses may be relevant. Immu-
to exposure in the primary survey of the weak
Table 8.4.1 Predictors of the necessity nisation history regarding polio vaccination
child a thorough search has to take place for
for ICU/HDU admission is important and diphtheria is still a common
ticks (dont forget to look in deep skin folds),
Bulbar palsy cause of weakness in third world countries.
possible venomous bites and stings, and
Any overseas travel should be noted.
rashes, such as those caused by entero- Vital capacity <20 mL kg1
viruses. Enteroviral rashes are usually scat- >30%reduction in vital capacity from baseline
tered maculopapular but may be petechial.
Flaccid quadriparesis Examination
A close inspection should be made for
bruises and other suggestive signs of NAI. Rapidly progressive weakness A detailed but focused examination is then
A quick assessment of the childs nutritional Autonomic cardiovascular instability
performed, that attempts to establish
state and the appropriateness of weight for the level and the nature of the lesion.

208
8.4 ACUTE WEAKNESS
8

NEUROLOGY
The following lists the important distin- than 17 years of age. It is primarily a lower
guishing features of the different levels of Investigations motor neuron disease affecting the myelin
the neuromuscular system which may pres- Laboratory sheath with variable damage to axons. It is
ent with weakness. Tests that may be useful in the acutely weak an immune-mediated reaction, usually to a
recent infection. Antibodies to various gang-
Muscular: child include:
liosides are found in the serum of patients
usually proximal more than distal a full blood count, which may reveal with GBS and matching antigens are often
weakness; anaemia, nutritional disorders, found in the preceding infectious agent.
reflexes preserved until very late; leukaemia, or signs of infection; Infections known to be associated with
may have tender muscles; abnormalities of electrolytes especially GBS include Campylobacter, Mycoplasma,
these children often have a positive potassium, calcium, phosphate, sodium EpsteinBarr virus, Coxsackie viruses, influ-
Gowers sign (see below). may cause weakness; enza viruses, echoviruses and cytomegalovi-
Neuromuscular junction: urea and creatinine renal failure may rus. The greater the axonal damage, the
fatigability; present as generalised weakness, but more longer and the less complete is the recovery.
reflexes are preserved if the muscle is likely cause an abnormality of electrolytes;
not fatigued or the disease is not creatine kinase is often raised in muscular History and examination
severe. causes of acute weakness; Children usually present with weakness,
 Lower motor neuron weakness: endocrine function tests especially falls, regression of motor milestones or
more peripheral than proximal thyroid and adrenal (see below); ataxia. They also complain of muscle pain
weakness;
lumbar puncture (see below) in the early part of the illness. Cranial nerves
reflexes are lost early in the illness. electrophysiological studies are involved in 4050% of cases, with the
Upper motor neuron weakness: (electromyogram (EMG), facial nerve most commonly involved. The
apart from an initial flaccid phase, electroneurogram (ENG)). MillerFisher variant presents with oculomo-
tone and reflexes are usually
tor palsies, ataxia and areflexia.
increased; Imaging On questioning, the parents often give a
spinal cord lesions are usually Urgent magnetic resonance imaging history of a generalised viral, respiratory or
associated with a sensory level; (MRI) of spine (where progressive spinal gastroenteritis illness in the preceding
intracranial problems are associated cord lesion is suspected); 2 weeks. In the early part of the illness the
with features of encephalopathy, Computerised tomography (CT) or MRI of child may have paraesthesiae. Classically,
decreased level of consciousness, brain (where central cause is suspected); the paralysis is ascending and symmetrical.
speech dysfunction, ataxia, and Chest X-ray (e.g. for suspected aspiration). The majority present with mostly distal
bulbar dysfunction.
weakness; however, about 15% have exten-
It may be difficult to perform a formal neuro- sive proximal muscular involvement.
logical examination in an unco-operative SPECIFIC CONDITIONS Papilloedema is rare, but may occur in
infant or small child. Often in these situations, GBS and is associated with raised intracra-
CAUSING ACUTE
one can get a lot of information just by watch- nial pressure. Paralysis of the respiratory
WEAKNESS
ing the child play. Another useful test is known muscles is common and must be monitored
as Gowers sign. The child is laid on their back carefully. Sympathetic nervous system
Though not exhaustive, the following sec-
on a firm surface and encouraged to stand. A involvement can produce profuse sweating,
tions give some detail on the more common
child with proximal muscle weakness will not hypertension, postural hypotension and dis-
causes of acute weakness seen in children
be able to sit up but will have to roll on to its turbances of sphincter function. Fatal car-
and also the rarer ones that must be diag-
abdomen get up on all fours and then climb diac arrhythmias have been reported in
nosed and treated in emergency.
up its legs using its hands. Although reflexes association with these signs. Although pri-
are a good guide they are by no means fool- marily a motor problem, sensory disturbance
proof. Upper motor lesions will usually have does occur, especially impairment of position
increased reflexes with increased tone. How-
GuillainBarre syndrome sense. As mentioned above, reflexes are usu-
ever, immediately after a spinal cord insult Introduction ally absent though increased reflexes and
there may be a flaccid paralysis below the GuillainBarre syndrome (GBS) is an acute extensor plantar responses are occasionally
level of the lesion with absence of reflexes. polyradiculopathy. It is now considered a found in the early phase of the illness.
Also, in transverse myelitis, there may be group of diseases with two main pathological The weakness may evolve rapidly within
patchy combinations of upper and lower mechanisms; demyelination and axonal hours. However, it usually takes 12 weeks
motor neuron signs. Early in GuillainBarre degeneration. In the paediatric population to reach the maximal weakness. Then, in
syndrome the reflexes may be preserved it is more common in late childhood (49 the 2nd to 4th week of the illness, recovery is
and, likewise, very late in myopathic weak- years of age) and in males (1.5 times higher apparent and most children have recovered
ness, distal reflexes may be lost. Acute myosi- risk). It is uncommon, estimated at less than by 2 months, although some take as long
tis is usually associated with tender muscles. 1/100 000 person years in people less as 18 months. Rarely, GBS will present in

209
8.4 ACUTE WEAKNESS

the newborn and is known as congenital GBS. is usually 2 g kg1 given in divided doses with
Table 8.4.3 Predictors of the necessity
They present as floppy babies that are are- for intubation and ventilation in GBS variable regimes.
flexic and have elevated cerebrospinal fluid
Vital capacity 20 mL kg 1

(CSF) protein. Prognosis

Maximum inspiratory pressure 30 cmH2O Prognosis is usually good in those treated and
Laboratory findings Maximum expiratory pressure 40 cmH2O supported appropriately. Most children reach
An isolated elevation in CSF protein is the maximum weakness in 2 to 4 weeks then
Tidal volume <5 mL kg 1

characteristic clinical finding of GBS. The CSF remain stable for 13 weeks and then recover
A sustained increase of pCO2 to 50 mmHg fully over a period of time that varies from
cell count is usually normal, although 5% of
children may have a pleocystosis of 100 or An increasing respiratory rate 6 weeks to many months. Approximately
so cells. Approximately 10% of children will 85% of children recover completely. Mortality
Increasing oxygen requirement
have a normal CSF protein. The protein may is 2 to 4% and is due to cardiovascular and
An increased use of accessory muscles and respiratory complications.
be raised if a lumbar puncture is done later paradoxical diaphragm movements; these
in the illness as it rises to a maximum after reflect restrictive lung-chest wall movement and
low lung volumes Disposition
45 weeks. The ENG characteristically shows
Children with suspected GBS should be
conduction block, although this may not
admitted and closely observed for the evolu-
become apparent until weeks into the illness.
monitoring of vital signs and cardiac rhythm, tion of severe weakness that can occur. The
frequent examinations and (if possible) lung findings outlined in Table 8.4.1 will assist in
Differential diagnosis deciding who should go to an intensive care
function tests should be performed. As noted
As laboratory tests may be normal in early unit (ICU)/high dependency unit and who
above, progression to respiratory failure can
GBS and signs may be variable, it is impor- should go to the general ward. Children with
be surprisingly rapid and the indicators for
tant to exclude other causes of acute weak- any one of these features should be moni-
intubation listed in Table 8.4.3 should be
ness (Table 8.4.2). tored in a paediatric ICU.
actively sought to avoid respiratory arrest.
The other less emergent treatments of GBS
Treatment are either intravenous immunoglobulin or
Tick paralysis
The main role of the emergency physician in plasma exchange. This is indicated if the child
the treatment of GBS is in monitoring, preven- can not walk unaided, has bulbar palsy, has Ticks that can cause paralysis are found
tion and treatment of cardiovascular and rapidly progressive weakness, or worsening throughout the world. In Australia Ixodes
respiratory complications. In addition to respiratory status. The immunoglobulin dose holocyclus is the main paralysing tick; it is
found on the east coast. Other ticks have
been known to cause paralysis, but they
Table 8.4.2 Differential diagnosis of GBS
are rare. The toxin previously called holocy-
Diagnosis Features/action to exclude clotoxin is now thought to be several toxins.
Puffer fish, Shell fish and History of ingestion or bite often a descending paralysis
The toxins inhibit the release of acetylcho-
Blue ringed octopus line from motor end plates. The paralysis
poisoning, Ciguatera
usually occurs 5 to 7 days after the tick
Tick paralysis Thorough examination of hair/skin creases attaches. The paralysis can resemble GBS
Snake envenomation History; check for bite site; coagulation screen, creatine kinase
in the form of an ascending paralysis and
is an important differential diagnosis
Spinal cord lesion Look for upper motor neuron/mixed features and a sensory level; if suspected
perform MRI
because removal of the tick is necessary
for recovery. The envenomation most fre-
Periodic paralysis Usually a sudden or very rapid onset, reflexes are diminished but preserved;
check family history; measure serum potassium; do ECG
quently presents with cranial nerve palsies.
Although the tick is often located in the
Infant botulism Almost exclusively in infants; ask for history of eating honey; culture stools for
Clostridium botulinum and test for botulinum toxin
vicinity of the palsy, this is not always the
case and in all cases a thorough examina-
Poisoning (e.g. History of exposure; toxidrome; check levels where suspected
organophosphate, lead)
tion should be made for multiple ticks. The
paralysis can be severe and require ventila-
Myasthenia gravis Often cranial nerves involved; fatigability; look for antibodies; perform
Tensilon test
tion. Australian tick paralysis may get worse
in the 48 hours following tick removal, and
Vasculitis (e.g. Check urinalysis; look for autoantibodies if suspected
polyarteritis nodosa)
children should be monitored carefully dur-
ing this time. Conversely, with children pre-
Myositis (e.g. Reflexes preserved; no ophthalmoplegia; look for characteristic rash; check
dermatomyositis) creatine kinase; EMG
senting with paralysis one should ask if a
tick was removed in the last 48 hours.
Poliomyelitis, diphtheria, Usually has fever and sore throat in diphtheria; ask about immunisations; no
other enteroviruses sensory changes
Deaths have usually been due to respiratory
paralysis; however, there have been reports

210
8.4 ACUTE WEAKNESS
8

NEUROLOGY
of myocarditis and autonomic effects on the nature of onset the infants always start one third of cases have gastroenteritis-like
heart. Treatment includes supportive ther- with bulbar palsies because that is where symptoms. The illness usually begins about
apy then careful removal of the tick(s) ensur- the blood supply is greatest; this is a 18 to 36 hours after ingestion, but onset
ing that the mouthparts are removed along descending paralysis; can range from 2 hours to 8 days. Presenta-
with the body. This can be made easier by fatigability, but lack of reversibility with tion is similar to infant botulism but more
applying a pyrethrum spray, which suffo- edrophonium or neostigmine; obvious because of the patients greater
cates the creature and makes it loosen its absence of fever; age. The treatment is similar.
grip. Squeezing the tick is not recommended absence of an altered mental status;
as this may inject more toxin. Human Tick absence of sensory defects; Wound botulism
Antivenom is no longer available. The treat- This is perhaps the rarest form. The differ-
normal CSF.
ment of tick paralysis is supportive; this may ences from the other forms are the presence
Clostridium botulinum may be cultured from of a wound, which may be obviously infected,
well include intubation and ventilation for
the stools and the toxin may be found in stools and the presence of fever. The incubation
several days after tick removal.
or serum through polymerase chain reaction period is 414 days. This requires aggressive
Other envenomations or enzyme-linked immunosorbent assay tests. treatment with antibiotics and antitoxin.
Snake and spider bites are discussed in detail Mouse bioassay is also available where steri-
elsewhere in the text (see Chapter 22.1). Usu- lised stools are injected into a mouse to see
ally the history and accompanying symptoms if it becomes paralysed. If suspicion is high, Spinal cord lesions
will give the diagnosis. Death adder envenom- foods in the childs residence can be tested
These are usually distinguished from periph-
ation is usually a purely neurological presen- for C. botulinum. The EMG is characteristic.
eral nerve disease by upper motor neuron
tation so in the weak non-verbal child a The difficulty is in early diagnosis. A weak
signs. However, in many cases initially the
thorough look for a bite site is indicated. suck is often put down to generalised illness
reflexes and tone are diminished (e.g. spinal
and it is often only the mother who notices
shock after trauma). In other cases, there
the lack of expression on the infants face.
may be patchy upper and lower motor neu-
Botulism A high index of suspicion needs to be
ron involvement such as in transverse myeli-
maintained.
Poisoning with botulinum toxin can present tis. Therefore spinal cord lesions need to be
Treatment is by supportive care and
in three ways: considered in the differential diagnosis of
administration of botulinum antitoxin. Anti-
the weak child. The key to spinal cord lesions
Infant botulism; biotics are not recommended unless there is
is the presence of a sensory level. However,
Food-borne botulism; and secondary infection (e.g. pneumonia). This is
in transverse myelitis and in a preverbal
 Wound botulism. because they may increase toxin release
child this may be difficult to establish. If a
when the bacteria lyse and divulge their con-
Infant botulism spinal cord lesion is suspected, the investiga-
tents. Tube feeding is recommended as it
Although rare, several cases of infant botulism tion of choice is an urgent MRI. This is time-
restores peristalsis, which is essential for
have been reported in Australia in the last few critical as a space-occupying lesion in the
clearing C. botulinum from the gut. If anti-
years. Infant botulism is caused by release of narrow canal can rapidly cause permanent
biotics are used, aminoglycosides should
botulinum toxin into the bloodstream from damage to the surrounding cord.
be avoided because these worsen the paral-
Clostridium botulinum bacteria colonising ysis. About half of the patients end up need-
Transverse myelitis (TM)
the intestines. Part of the toxin enters the ing intubation and ventilation. Hospital stay
The aetiology of this acute spinal cord inflam-
terminal bouton of cholinergic motor nerves is an average of 1 month but varies widely.
mation is still uncertain. Hypotheses include
and enzymatically disables the mechanism Infants seem to recover completely.
microbial antigen cross-reaction with neural
by which acetylcholine-containing vesicles There are two antitoxins available.
elements, bacterial superantigen inflamma-
attach to the cell membrane. This process is Equine-derived antitoxin, a small amount
tion and direct microbial invasion. Rarely, it is
irreversible and recovery occurs by sprouting of which is held in the Commonwealth
associated with systemic diseases such as sys-
new unmyelinated motor neurons. Risk factors Serum Laboratories, and botulism immune
temic lupus erythematosus and multiple scle-
for babies contracting this disease include globulin (BIG), a human-derived hyperim-
rosis. It is likely that TM will be found to be
exposure to honey in the first six months mune globulin, which in a recent rando-
several diseases and perhaps treatment will
(honey is not recommended for babies under mised controlled trial reduced hospital stay
need to be tailored to the specific aetiology.
1 year), decreased frequency of stooling and from 6.6 weeks to 2.6 weeks. It is not avail-
This disease usually has a rapid onset of
lack of breast-feeding. able outside the United States.
predominantly lower limb weakness and
Diagnosis of this condition is charac-
altered sensation. Neck stiffness and fever
terised by:
Food-borne botulism are present early in most cases along with
age group the infants are almost always This is due to consumption of food in which low back pain or abdominal pain. The sensory
less than 6 months; however, there are there is preformed toxin. It is associated with level is usually around the mid-thoracic region
reports in infants up to 1 year and older home-canned foods. It differs from infant bot- below which pain, light touch and tempera-
children with abnormal bowel anatomy; ulism in that it can occur in any age group and ture sensation are impaired. However, joint

211
8.4 ACUTE WEAKNESS

position and vibration sense are more pre- lesion causing compression or by stealing Congenital myasthenia
served. Bladder and bowel disturbance is com- circulation from the nearby cord. The history This is a lasting condition due to a genetic dis-
mon, although this may be difficult to is usually subacute, unless a haemorrhage order. It is not due to antibodies to the AChR.
determine in a child in nappies. Tone is usually or ischaemia has intervened. Clues to the
flaccid early in the illness with decreased diagnosis on examination include a cutane- Acquired myasthenia
reflexes, followed by increased tone and ous angioma over the region and a bruit on The incidence of this is low, though not insigni-
hyperreflexia as the disease progresses to auscultation. MRI/MR angiogram will give ficant. In an Italian study the annual incidence
its peak over the next 23 days. Sixty percent the diagnosis but angiography is usually was 3.3 per million children <15 years; about
of patients recover fully over weeks to months. required to define the lesion fully and to 1% of total MG cases. The presentation is usu-
Urgent MRI usually shows fusiform decide on whether to treat with surgery or ally with ptosis, ophthalmoplegia or bulbar
oedema around the site of the sensory level. embolisation. weakness. The clue on history is the worsening
CSF shows a moderate lymphocytosis and of symptoms as the day progresses due to
mildly raised protein. fatigue. Peripheral muscles, especially limb
Epidural abscess
Treatment is controversial, with case- girdle and hand muscles, are also involved.
This is a rare disease in children, who usually
control studies indicating a benefit with Reflexes are preserved but diminished. Clinical
present with back pain and rigidity, fever,
glucocorticoids, whereas prospective trials tests for fatigability such as getting the child to
leucocytosis and a raised erythrocyte sedi-
show no benefit. Treatment decisions should look up for 6090 seconds and watching
mentation rate. These symptoms may be fol-
be made in consultation with a paediatric for ptosis, or to flap one arm like a bird for a
lowed by spinal neurological signs. MRI is
neurologist. minute and then comparing its strength with
the investigation of choice, but there is con-
the other arm are very useful.
troversy over whether treatment should be
Spinal cord trauma Diagnosis can be made with three tests.
by surgery or antibiotics alone.
This is discussed in Section 3. First, an anticholinergic drug may be given.
Edrophonium, the usual drug used in adults
Spinal cord space-occupying Tethered cord/diastematomyelia may cause cardiac arrhythmias in small chil-
lesions These are two conditions that tend to fix dren, so neostigmine is preferred. Atropine
These include epidural abscess, tumours, the cord of the child, which has to move up is given beforehand to block the muscarinic
syringomyelia and arteriovenous malforma- the canal as the child grows. They rarely pres- effects. These drugs should abolish the fatiga-
tions. Since syringomyelia is almost always ent with acute weakness, although there may bility. Second, the EMG is characteristic and
a chronic condition it will not be dealt with be sudden exacerbations on flexion and exten- usually obviates the need for muscle biopsy.
here. sion. Examination over the spine may reveal Third, AChR antibodies can be measured in
sentinel lesions such as a tuft of hair, a sacral the blood. Early diagnosis is important
Tumours
pit, a lipoma, or a cutaneous haemangioma. because, if untreated, this disease will often
These may be intramedullary (e.g. low-grade
astrocytoma), extramedullary intradural progress to life-threatening severity.
(e.g. meningioma) and extradural (e.g. lym- Associations with MG commonly found in
phoma). They usually have a more gradual Myasthenia gravis (MG) adults, such as thymoma, are rare in chil-
onset than the other diseases mentioned in dren. Differential diagnosis includes botu-
This condition, which is usually due to ace- lism, chronic low-grade organophosphate
this chapter but the early signs may be
tylcholine receptor autoantibodies, leads to toxicity and tick paralysis.
missed and the child may present when signs
easy and rapid fatigability of muscles. This In the ED the child may present as an ini-
are rapidly evolving due to high intramedul-
may occur in children in three forms. tial episode or because of a crisis of weak-
lary pressures. Progressive gait and bladder
disturbance with back pain and absence of ness. These crises may be myasthenic, due
fever are characteristic signs. Recent onset Transient myasthenia to exacerbation of the underlying condition
of scoliosis may be a feature. There may be of newborns or cholinergic due to excessive anticholin-
a mixture of signs with upper motor neuron This occurs where the mother has myasthe- esterase treatment, which leads to over
signs in the lower limbs and lower motor nia. Maternal antibodies to the acetylcho- stimulation and exhaustion of receptors.
neuron signs in the upper limbs. line receptor (AChR) cross the placenta Classically, a cholinergic crisis has the cholin-
Urgent MRI is the investigation of choice, and the baby is born with fatigable muscle ergic toxidrome features of hypersalivation,
followed by consultation with surgeons weakness. These babies may show very little pulmonary oedema and muscle fascicula-
and/or radiation oncologists. If MRI is motor activity for days after birth. Alterna- tion. However, in someone with myasthenia
unavailable, CT or plain X-ray may show tively, they may just have feeding difficulty the cholinergic crisis may only be manifest
abnormalities. However, these should only that worsens during the day. This illness by weakness. Distinguishing between a
be done if they do not delay transfer to an improves within weeks as the maternal anti- myasthenic and cholinergic crisis may be dif-
MRI capable centre. body levels diminish in the childs blood. The ficult. History may give a clue if medications
only treatment is supportive by tube feeding have been missed or an overdose of pyridos-
Arteriovenous malformations and intubation and ventilation, if severe. tigmine has been taken. A therapeutic trial of
These are usually in the thoracic region. They The babies recover completely and have edrophonium may help, but should not be
may cause symptoms as a space-occupying no increased risk of MG. undertaken if there is significant risk of a
212
8.4 ACUTE WEAKNESS
8

NEUROLOGY
cholinergic crisis. In the latter case supportive Diagnosis is based on the immunisation to recommend glucocorticoids in all but the
treatment and measurement of blood cholin- history, the clinical picture and a lumbar punc- mildest cases of Bells palsy. There is even less
esterase activity may be the only option. ture showing a moderate pleocytosis, initially evidence for antiviral agents in the absence
Long-term treatment of MG comprises of neutrophils but then changing to mono- of apparent viral infection (e.g. the Ramsay
anticholinesterases and a variety of immu- cytes. Serology and culture of stool, throat Hunt syndrome). If the eyelid does not
nosuppression, IgG infusion or thymectomy. swab and rarely CSF will often reveal the completely close steps should be taken to
In MG: organism. Treatment is supportive only. Infec- protect the cornea from exposure keratopa-
tion control authorities need to be informed. thy, i.e. artificial tears and eyeglasses during
Do not give neuromuscular blocking the daytime, ointment and a protective eye
agents (e.g. suxamethonium,
chamber at night.
vecuronium) as these may paralyse the
MG patient for days or even weeks. Bells palsy
Do not give aminoglycoside antibiotics A sudden or rapid onset of a unilateral lower
as these exacerbate the weakness. Toxic neuropathies
motor neuron palsyis not a rare occurrence
in children. Estimated annual incidence varies A long list of substances can cause acute
from 3 to 10 per 100 000 children per year. Its weakness. Some of the more common ones
aetiology is uncertain. The disease commonly are listed here.
begins 2 weeks after an infectious illness,
Poliomyelitis and other
which suggests a post-infectious autoimmune Anticholinesterases
enteroviral infections The organophosphates and carbamates are
or allergic aetiology. Lyme disease has been
Poliomyelitis is now exceedingly rare; how- associated and serology should be done if commonly used insecticides, which can
ever, one should always ask about immuni- the patient has been in an endemic area. cause poisoning through skin, oral or pulmo-
sation status in the acutely weak child. If EpsteinBarr, mumps and herpes simplex nary exposure. They inhibit cholinesterases,
the child is not immunised one should ask viruses have also been associated with this allowing acetylcholine to persistently stimu-
about contact with infants recently immu- disease. An association with hypertension late the nicotinic and muscarinic receptors,
nised with Sabin (oral weakened live poliovi- has suggested another aetiology related to which then can become refractory and thus
rus) vaccine. Infants excrete the virus after pressure necrosis of the nerve due to swelling cause weakness. This weakness is usually
the immunisation and this is where most in the narrow facial canal. accompanied by the cholinergic toxidrome
recent cases of poliomyelitis have come The patient often presents with pain (see Chapter 21.2 on toxicology) and,
from. The other source of infection is in around the ipsilateral ear and may also com- indeed, it is usually the respiratory and car-
developing nations, where immunisation plain of abnormal hearing. About half have diac features that predominate. However,
rates are low. The illness itself usually pre- loss of taste sensation to the anterior two- there is an intermediate syndrome where
sents with symptoms related to the viruss thirds of the tongue and there may be hemi- 12 hours to 7 days after the initial poison-
portal of entry through the gut and upper facial dysaesthesia due to the proprioceptive ing, one finds proximal limb weakness that
respiratory tract. Patients have fever, sore fibres to the facial muscles in the facial nerve. is unresponsive to atropine or pralidoxime.
throat, anorexia, nausea, vomiting, general- The differential is extensive but the diag- There may also be respiratory and bulbar
ised non-specific abdominal pain, malaise nosis can be determined by a thorough his- paralysis. Recovery from this is usually com-
and headache. The great majority of poliovi- tory and clinical examination. One should plete. Late neurotoxicity arises 421 days
rus infections end here or are asymptomatic. look for evidence of trauma (be aware of after the acute exposure, causing a mixed
In those patients who progress there is often non-accidental injury), central nervous sys- sensory motor deficit, which may take weeks
an asymptomatic period of 12 days fol- tem dysfunction, aural lesions (e.g. the or months to recover, or may be permanent.
lowed by symptoms of aseptic meningitis Ramsay Hunt syndrome), other cranial Diagnosis is by identification of the poison
with neck and often entire spine stiffness. nerve dysfunctions, hypertension and GBS. at source or in urine or serum drug screens
There may be mild transient neurological Acute lymphoblastic leukaemia and even and also by measuring serum cholinesterase
deficits such as bladder paralysis and loss sarcoidosis have been reported. activity level. Treatment is supportive and
of abdominal and anal reflexes. Those who Treatment is controversial, as the progno- with antidotes atropine and pralidoxime.
progress to paralytic polio will usually do sis in children is better than in adults. Com- Muscle relaxants will have a prolonged
so 812 hours after the superficial reflexes plete recovery occurs in 60 to 80% of effect and should be avoided if possible.
are lost. Poliovirus can infect and destroy patients, with near complete recovery in
neurons from the motor cortex down to the remainder. In one study average time Lead and other heavy metals
the anterior horn cells. However, most com- to recovery was about 7 weeks with a range Lead, mercury and arsenic are all known to
monly the paralytic form presents with of 9 days to 7 months. Uncontrolled studies cause neuropathies, often taking the form
patchy asymmetrical lower motor neuron have claimed a benefit for early corticoste- of mononeuritis multiplex. It is uncommon
weakness. Bulbar weakness is also a fre- roid therapy. However, controlled studies in for these to present as acute weakness. Nev-
quent presentation and often the picture adults have shown a benefit for oral gluco- ertheless, a history of exposure should be
is mixed. Rarely there are also encephalitic corticoids in Bells palsy. This and the less reli- sought and appropriate specimens taken if
and ataxic presentations. able evidence in children prompt this author the cause of the acute weakness is unclear.
213
8.4 ACUTE WEAKNESS

Chemotherapeutic agents Diagnosis is made on the clinical picture by measurement of electrolytes during an
Vincristine, vinblastine and cisplatin are associated with a raised serum creatine attack or response to a metabolic challenge
known to cause a peripheral neuropathy. kinase. There are typical changes on muscle or by gene mutation identification. Hypoka-
Any child who is on, or has recently had, che- biopsy and electromyogram. There may also laemic periodic paralysis usually occurs in
motherapy needs to have this ruled out as a be elevated autoantibodies, liver function adolescence. The patient will have low
possible cause. tests and abnormalities on MRI. potassium during attacks. Conversely, hyper-
Complications include calcinosis of mus- kalaemic periodic paralysis usually comes on
cles, subcutaneous fat and fascia. These in early childhood. The attacks are more fre-
Hereditary neuropathy are more likely if the illness is prolonged quent, occurring up to several times a day
Rarely, a patient with an undiagnosed and are decreased by aggressive treatment. and are associated with an elevated or nor-
hereditary neuropathy presents with an Calcinosis lesions may become infected and mal potassium. The identification of these
acute exacerbation. A thorough history will lead to septicaemia. diseases is important, as they are treatable.
reveal the chronicity of the problem. Such These children may require judicious use
patients should be referred to an appropri- of pain relief and require referral to a spe-
ate paediatric neurology service. cialist paediatric unit. Treatment options
range from sunscreen for the rash through Somatisation disorders/
to immunosuppression with glucocorticoids malingering
Muscular disorders and chemotherapeutic agents, depending
Occasionally, children may present with a
on the severity of the disease. Prognosis is
Juvenile dermatomyositis psychogenic cause of weakness. The find-
generally good in children, with approxi-
Juvenile dermatomyositis is a systemic vascu- ings of a careful neurological examination
mately 80% making a good recovery.
litis thought to be triggered by infection. Both will demonstrate inconsistencies and the
enteroviruses and Group A Streptococcus child will be able to perform manoeuvres
Infectious myositis
have been implicated. This is the most com- that require more strength than normal
Viral infections such as influenza can un-
mon myositis of childhood. Its peak age of inci- walking (e.g. knees giving way but not fall-
commonly cause a myositis that may in its
dence is 6 years. Because of its gradual onset ing). Reflexes will be normal. Usually, no lab-
severest form lead to rhabdomyolysis and
this uncommonly presents as acute weakness. oratory tests or imaging are necessary.
myoglobinuria. Various bacteria including
The child may well present with the rash However, if there is any doubt, consultation
Streptococcus and Mycoplasma species have
before weakness has become apparent. and investigation should be undertaken.
been reported to cause a focal myositis
The rash appears on sun-exposed areas, Once you have made the diagnosis it is
with tender muscles. These usually present
especially the malar region of the face and best to take the parent or carer aside and dis-
with the other features of the infection and
a purple discolouration of the eyelids is cuss the diagnosis with them. Confrontation
myalgia, rather than acute weakness. The
apparent (heliotrope rash). The rash may of the child is rarely helpful and it is often
weakness is only found on close examination,
also be found on the extensor surfaces of best to reassure the child that this is not seri-
if the myalgia permits. The creatine kinase is
the arms and legs, thorax, ankles and but- ous and that they will get better. This gives
raised. The prognosis is generally good
tocks. The fingers develop thickening of the child an exit from the medical review with
although permanent muscle damage may
the skin over joints, called Gottrons papules. dignity intact. An effort should be made to
ensue, especially in focal bacterial myositis.
The weakness comes on about 2 months find the underlying psychological reason for
after the rash and is usually very slow in Metabolic/endocrine myopathies the presentation, which may be anything
onset. Small children may be noted to Most endocrinopathies can produce weak- from school avoidance to sexual abuse. Con-
become gradually inactive and older chil- ness by several mechanisms. Often this is sultation with social work or psychiatry may
dren have increasing difficulty with sport. just myalgia and fatigue, but true myopa- be indicated for ongoing counselling.
Proximal muscle activities, such as climbing thies can develop. This weakness usually
up stairs, reveal the weakness first. The vas- responds to the treatment of the underlying
culopathy can affect any muscle group and endocrine disorder. Endocrinopathies can be Further reading
children may present with aspiration dys- associated with electrolyte disturbances Behrman RE, Kliegman R, Jenson HB, editors. Nelson Textbook of
Pediatrics. 16th ed. Philadelphia: WB Saunders; 2000.
phagia or hoarse voice due to pharyngeal that lead to weakness (e.g. hypokalaemia Emilia-Romagna Study Group on Clinical and Epidemiological
muscle weakness. The affected muscles are in Conns syndrome) or with primary muscle Problems in Neurology. Incidence of myasthenia gravis in
the Emilia-Romagna region: A prospective multicenter
often tender and sometimes swollen. diseases, such as hypokalaemic periodic study. Neurology 1998;51(1):2558 [brief
The vasculitis can affect any organ sys- paralysis with thyrotoxicosis. communications].
Lawn ND, Fletcher DD, Henderson RD, et al. Anticipating
tem. Subclinical myocarditis and conduction The periodic paralyses are a series of mechanical ventilation in GuillainBarre syndrome. Arch
defects are often found at diagnosis. Less genetic ion channel disorders that lead to Neurol 2001;58:8938.
Odaka M, Yuki N, Hirata K. Anti-GQ1b IgG antibody syndrome:
common effects include renal dysfunction, acute episodes of weakness lasting from 1 Clinical and immunological range. J Neurol, Neurosurg
hepatosplenomegaly, retinitis, iritis, seizures, hour up to more than a day. They often come Psychiatry 2001;70:505.
Tang T, Noble-Jamieson C. A painful hip as a presentation of
depression, bowel dysfunction and pulmo- on after rest, during sleep or following exer- Guillain-Barre syndrome in children. Br Med J
nary disease. cise, but never during exercise. Diagnosis is 2001;322:149150.

214
 8

NEUROLOGY
8.5 Acute ataxia
Joanne Grindlay

It has only vestibular connections, which

ESSENTIALS travel in the inferior cerebellar peduncle.
The archaecerebellum controls balance. Dys-
1 The most common causes of acute cerebellar ataxia in children are post-infectious function leads to truncal ataxia charac-
(acute cerebellar ataxia), drug intoxication and posterior fossa tumours.
terised by a drunken gait, with swaying of
2 Clinical features suggestive of a brain tumour and hence warranting further the trunk and titubation when sitting, stand-
investigation include headache, vomiting, papilloedema, cranial nerve dysfunction or ing or walking. Typically there is neither
behavioural changes. alteration in fine movements nor nystag-
mus. Reflexes are normal and there is no
3 Ataxia is not uncommonly a presenting symptom of poisoning or excessive tremor. The anterior lobes of the lateral
therapeutic drug levels, particularly anticonvulsants.
hemispheres, together with uvula and pyra-
4 A number of neurological conditions may present with an unsteady gait mid of the vermis form the palaeocerebel-
(pseudo-ataxia) due to acute weakness. lum. The palaeocerebellum connects to the
spinocerebellar tracts and is involved with
5 Investigations in the child with ataxia are directed by the history and clinical postural reflexes. Dysfunction leads to pos-
features detected on examination.
tural imbalance and increased reflexes.
6 While ataxia may be seen in post-concussion syndrome, conditions requiring acute The posterior lobes of the lateral hemi-
intervention should be sought by CT scan. spheres form the neocerebellum, which con-
nects to the cerebral hemispheres, basal
7 Most children with significant ataxia require admission for investigation and nuclei and the pontine nuclei, via the middle
observation.
cerebral peduncle. It is involved in the coor-
dination of fine, voluntary movements. Dys-
function leads to nystagmus, intention
There are numerous hereditary conditions tremor, dysdiadochokinesis, hypotonia and
Introduction causing chronic ataxia that may present in decreased or pendular reflexes. The vermis
Ataxia is an uncommon, but important pae- childhood. These include: Friedreichs ataxia, and surrounding nuclei of the cerebellum
diatric presentation to the emergency hereditary cerebellar ataxia, spinocerebellar form the roof of the 4th ventricle. An
department (ED). Ataxia is a disorder of ataxia and ataxia telangiectasia. The pro- expanding lesion may obstruct the cerebro-
movement manifest by the loss of coordina- gressive nature of these conditions and their spinal fluid (CSF) flow, with resultant hydro-
tion, most apparent as a disturbance of gait, associated signs differentiate these from the cephalus and truncal ataxia.
with intact muscle strength. It may be asso- acute ataxias.
ciated with a disturbance of balance. Ataxia
Table 8.5.1 Causes of acute ataxia
is most often caused by a loss of function of in childhood
the cerebellum, which controls the coordina- Pathophysiology Post-viral acute cerebellar ataxia
tion of movement. Disease of the peripheral Poisoning/drug intoxication
sensory nerves or the spinal column, particu- Cerebellum Tumours
Posterior fossa, brainstem
larly affecting proprioception, may also lead The cerebellum consists of two lateral hemi- Paraneoplastic syndrome
to ataxia as a result of abnormal inputs into spheres, divided into anterior and posterior Trauma including non-accidental injury
Haematoma
the cerebellum. Cortical ataxia results from lobes by the primary fissure. The hemi- Post-concussion
cerebral cortical dysfunction, particularly of spheres are joined by the vermis in the mid- Metabolic
Hypoglycaemia
the frontal lobe, while vestibular ataxia line. The cerebellum connects to the cerebral Hyponatraemia
results from disease of the inner ear. Rarely, cortex and brainstem via three paired sets of Hyperammonaemia
Inborn errors of metabolism
psychiatric causes of ataxia may also be peduncles, the superior, middle and inferior Infections
seen as a manifestation of conversion reac- cerebellar peduncles. Meningitis bacterial, viral
Cerebral abscess
tion. The most common diagnosis of acute Malaria
Labyrinthitis
ataxia in children is a post-infectious ataxia Cerebral hemispheres and vermis Encephalitis
called acute cerebellar ataxia (see below). There are three functional parts of the cere- Vascular
Stroke
This is a diagnosis of exclusion, made after bellum. These are the archaecerebellum, the Vasculitis
consideration of other causes (Table 8.5.1). palaeocerebellum and the neocerebellum. Immune
Multiple sclerosis
These include poisoning, metabolic disorders The archaecerebellum is formed by the Acute disseminated encephalomyelitis
and organic brain lesion. flocculus, nodule and lingula of the vermis.

215
8.5 ACUTE ATAXIA

Cerebellar peduncles nystagmus occurs in 50% of cases. Findings

Table 8.5.3 Drugs causing acute ataxia
and connections of intention tremor, dysdiadochokinesis, hy-
Anticonvulsants
The cerebellum receives inputs from the ves- potonia and decreased or pendular reflexes Phenytoin
tibular and peripheral nervous systems. Out- are seen in two-thirds of cases, but are Carbamazepine
Benzodiazepines
puts, which coordinate muscle movements less pronounced than the gait disturbance. Alcohols
by modifying tone and contraction, travel Truncal ataxia is uncommon. Unlike acute Ethanol
Isopropanol
in three sets of peduncles (superior, middle, disseminated encephalomyelitis (ADEM) or Ethylene glycol
and inferior) between each cerebellar hemi- multiple sclerosis, there are no focal neuro- Essential oils
Eucalyptus oil
sphere and the brainstem. logical signs. Tea tree oil
Investigations are aimed at excluding an Pine oil
Cough suppressants
alternative diagnosis, if the diagnosis is Codeine
Differential diagnosis unclear. The computerised tomography (CT) Dextromethorphan
Drugs of abuse
scan is normal in acute cerebellar ataxia; how- PCP
The most common cause of acute ataxia in ever, magnetic resonance imaging (MRI) may Solvents, petrol, glue
children is post-infectious, acute cerebellar be abnormal. In one series, inflammatory
ataxia. Drug intoxication and posterior fossa changes were seen in the cerebellum of one
tumours are less common. of nine children.23,24 There is a slight eleva- Carbamazepine toxicity may also lead to
tion of CSF cell count by 450 cells per micro- ataxia. There are usually associated findings
Acute cerebellar ataxia litre in 32%, though occasionally (in 8%) the of drowsiness and nystagmus. There may be
Acute cerebellar ataxia is the most common elevation is higher. There may also be slightly progression to seizures and coma, particu-
diagnosis in acute ataxia in children, partic- elevated protein 410900 mg L1.25 larly if the level is >100 mmol L1.29
ularly between 2 and 7 years of age. It is a Acute cerebellar ataxia usually begins to
diagnosis of exclusion, after consideration improve within a few days, but full recovery Benzodiazepines
of more sinister causes such as tumours. may take from 10 days to 2 months. Patients Ataxia may be the sole presenting feature of
An autoimmune aetiology is likely, with who have a slower recovery are still likely to benzodiazepine ingestion in children. In one
autoantibodies demonstrated in acute recover fully. In one series, 91% recovered series, it was an isolated finding in one-fifth
cerebellar ataxia following infections with fully from their ataxia, including all those with of the cases who demonstrated ataxia. Other
varicella,1,2 EpsteinBarr virus (EBV),3 myco- varicella, EBV or post-vaccination, but 8% findings included lethargy (57%), GCS <15
plasma and human parvovirus B19.4 The had sustained learning problems. Varicella- (35%) and respiratory depression (9%).30
clinical presentation is of a prodromal ill- associated ataxia recovered quicker than
ness, frequently non-specific, with or without non-varicella.24 Alcohols
an exanthema, 510 days prior to the onset Ethanol intoxication produces ataxia, disin-
of acute ataxia, though the timing may show hibited behaviour and slurred speech. A
considerable variation. If a specific aetiology
Poisoning serum ethanol level will aid clarification of
Ataxia is not uncommonly a presenting
is present it is most commonly varicella,5 the diagnosis.
symptom of ingestion or excessive therapeu-
though a number of other viruses have been Ethylene glycol is the main component of
tic drug levels, particularly anticonvulsants.
implicated (Table 8.5.2).622 Acute cerebel- antifreeze. Early symptoms of ingestion are
Accidental poisoning occurs most com-
lar ataxia usually presents with sudden similar to those of ethanol intoxication.
monly in 1 to 4-year-old children. A wide
onset of severe gait ataxia, though a small Delayed features include cardiopulmonary
variety of compounds are implicated, includ-
number of cases have an insidious onset. distress and nephrotoxicity. Ethylene glycol
ing alcohols, substances of abuse and essen-
Most have dysarthric speech. Mild horizontal produces a raised anion gap metabolic aci-
tial oils, as well as medications (Table 8.5.3).
dosis with osmolal gap. Serum levels
In addition to ataxia, these children may
>20 mg dL1 are toxic. The ethanol level
Table 8.5.2 Causes of post-infectious have nystagmus, altered mental status and
acute cerebellar ataxia will be zero. Isopropanol, a component of
vomiting. This is different to acute cerebellar
rubbing alcohol, is used widely as a solvent.
Varicella6 ataxia where consciousness is unimpaired.
Coxsackie A910 Toxicity is evident between 0.5 and 2 hours
EpsteinBarr virus8 post-ingestion and may include vomiting,
Mycoplasma9
Mumps10 Anticonvulsants ataxia, nystagmus, and altered mental state.
Poliomyelitis11,12 Coma and apnoea may occur in severe
Typhoid13
Phenytoin toxicity with serum levels of
Echovirus14 >2030 mcg mL1 may produce signs of poisoning.31
Pertussis15
Human parvovirus16
ataxia, nystagmus on lateral gaze and drows-
Measles15 iness. Onset of symptoms following an acute Essential oils
Herpes simplex virus17
Enterovirus 7118
ingestion is usually within 12 hours and Eucalyptus oil is not an uncommon ingestion
Malaria19 may persist for 45 days. At >30 mcg mL1, by children. In one series of 109 admitted chil-
Immunisation20,21
Human herpesvirus-622
the ataxia and drowsiness become more dren, 41% were asymptomatic. Those who
marked and the nystagmus vertical.2628 were symptomatic demonstrated decreased

216
8.5 ACUTE ATAXIA
8

NEUROLOGY
conscious state (28%), vomiting (37%), or as a result of hydrocephalus. The ataxia Trauma
ataxia (15%) and pulmonary disease (11%). may progress to chronic ataxia. Paraneo- Head trauma is a frequent cause of ED pre-
There was a correlation between ingested plastic syndromes including paraneoplastic sentations. While ataxia may be seen in
dose and toxicity. An ingestion of >5 mL cerebellar degeneration and opsoclonus- post-concussion syndrome, intracranial injury
100% oil was associated with a signifi- myoclonus-ataxia syndrome are uncommon requiring acute intervention, should be
cantly decreased conscious state, whereas causes of acute paediatric ataxia. sought by CT scan. These include haemor-
<23 mL was associated with minor depres- Clinical features suggestive of a brain rhage or cerebral oedema. A base-of-skull
sion of consciousness.32 A second series of 41 tumour, and hence further investigation, fracture may cause ataxia by direct damage
presentations, however, only demonstrated include headache, vomiting, behavioural to the vestibular apparatus. Non-accidental
effects in 20%, with no correlation with changes (particularly with frontal lobe lesions), injury should be considered in all cases of pae-
presumed dose. The clinical effects included papilloedema or cranial nerve dysfunction. diatric trauma.
ataxia (5%), decreased conscious state Posterior fossa tumours include medullo-
(10%) and gastrointestinal symptoms (7%).33 blastoma, astrocytoma and ependymoma. Infections
Other essential oils that may produce nys- Medulloblastoma (2025% of posterior fossa Meningitis and encephalitis may both have
tagmus include tea tree oil34 and pine oil. In tumours) usually presents in a child of less ataxia as a presenting feature.42,43 Other fea-
one small series of pine oil cleaner (35% than 6 years with symptoms of ataxia, which tures to suggest an infective cause, such as
pine oil, 10.9% isopropyl alcohol), symptoms may be truncal, headache, irritability or vomit- headache, vomiting, fever and neck stiffness
developed within 90 minutes of ingestion. ing. The tumour may be located in the 4th in the older child, will usually be evident.
Lethargy was present in all symptomatic ventricle or vermis. Cerebellar astrocytoma In labyrinthitis and vestibular neuronitis,
children and ataxia in four of five cases of (1030% of paediatric brain tumours) is vertigo and vomiting are prominent. Apart
children.35 located in one of the cerebellar hemispheres. from nystagmus and hearing alterations,
It is seen in primary-school-aged children who neurological examination is normal.
Cough suppressants may display ipsilateral limb ataxia, headache
Codeine is contained in a number of cough and vomiting. The head may be held tilted Vascular conditions
medicines as well as in analgesics. In one to one side. Associated raised intracranial Vertebrobasilar stroke is a very uncommon
series of 430 children, ataxia was reported pressure may be life threatening. Ependy- cause of ataxia in children. Ataxia will not
in 9% receiving codeine. Associated symp- moma (810%), located in the 4th ventricle, be an isolated finding. Other findings
toms are somnolence 67%, rash 39%, causes obstruction of CSF flow and may pres- include ipsilateral cranial nerve palsies, con-
miosis 30%, vomiting 27%, itching 10%, ent with headache, vomiting and ataxia, tralateral weakness, vertigo and diplopia.
angio-oedema 9%.36 Dextromethorphan is which may be truncal. Brainstem gliomas Systemic lupus erythematosus vasculitis
a common component of cough and cold (1015% of paediatric brain tumours) may rarely present with ataxia.44
medications, which acts through opiate develop in the pons or medulla. They typically
receptors in the medulla. It may cause occur in early primary-aged children. Present- Other neurological conditions
opisthotonus, ataxia and bidirectional nys- ing symptoms include cranial nerve palsies, In ataxia there is preservation of muscle
tagmus. Fatality is highly unlikely, even with ataxia and vomiting. strength. A number of neurological conditions
one hundred-fold the therapeutic dose.37,38 Hydrocephalus may present with ataxia may present with an unsteady gait (pseudo-
due to stretching of frontopontocerebellar ataxia) because of weakness. These include
Substances of abuse fibres. Associated features are headache, GuillainBarre syndrome in which areflexia
Phencyclidine (PCP) and lysergic acid diethyla- vomiting and the late signs of raised intra- and ophthalmoplegia (in MillerFisher vari-
mide (LSD) are rare causes of ataxia in chil- cranial pressure altered conscious state, ant) distinguish it from acute cerebellar
dren. 1,4-butanediol, which is metabolised raised blood pressure and decreased pulse. ataxia. Tick paralysis should also be consid-
to gamma-hydroxybutyrate (GHB) upon Supratentorial tumours may also present ered in cases where the patient has been in
ingestion, was responsible for ataxia, vomiting with ataxia through involvement of the fron- an appropriate area. Multiple sclerosis/trans-
and seizures in several children following the topontocerebellar fibres. verse myelitis is usually not seen until adoles-
ingestion of Aqua Dots, from toy craft kits.39 An occult neuroblastoma may present cent years. It may present with ataxia, optic or
More common, however, is the abuse of with a triad of acute ataxia, opsoclonus retinal neuritis, regional paraesthesias or
hydrocarbon solvents; toluene in glue, spray (jerky, random, chaotic eye movements) weakness.45 ADEM, a post-infectious enceph-
paints and petrol. Hydrocarbons may pro- and myoclonus (severe myoclonic jerks of alomyelitis, where host myelin components
duce ataxia as a component of acute intoxi- the head, trunk or limbs). The most common become immunogenic, may have ataxia as
cation and also as a chronic central nervous site for the tumour is in the abdomen.40 The one neurological sign. The diagnosis is made
system sequel. triad may also be seen with viral infections, by abnormal CT and MRI findings.
including meningitis, particularly mumps, Other neurological conditions that may
Tumours hence a lumbar puncture should be consid- present with ataxia include seizures and
Cerebellar lesions may present with an acute, ered. Neuroblastoma may present with complex migraine phenomenon. Because
rather than insidious onset of ataxia as a isolated ataxia and should be considered of the episodic nature of these conditions,
result of either haemorrhage into a tumour in cases of persistent or recurrent ataxia.41 there will be a recurrent nature to the

217
8.5 ACUTE ATAXIA

ataxia. Basilar artery migraine may demon- other symptoms, such as headache, vomit-
strate associated headache, blurred vision, Chronic ataxia ing, blurred vision, altered mental status or
visual field defects and vertigo. It tends to Chronic ataxia usually has an insidious onset nausea suggestive of a tumour or meningi-
have a recurrent course. Epilepsy uncom- but it may present as a progression of acute tis? The antecedent history is important;
monly presents with ataxia. Ataxia may be ataxia, or as recurrent episodes. Causes for example, a recent viral illness may sug-
seen post-ictally or in minor motor status or include fixed deficits as in ataxic cerebral gest acute cerebellar ataxia, or there may
partial complex seizures. Other clues to sei- palsy, which makes up 10% of cerebral palsy be a history of trauma or possible drug
zure disorders include altered consciousness and progressive diseases such as the heredi- ingestion. Other symptoms, for example par-
or motor manifestations. Benign positional tary ataxia, inborn errors of metabolism and aesthesia, may suggest an alternative diag-
vertigo is uncommon in children. Ataxia tumours. Some causes are amenable to treat- nosis such as ADEM.
accompanies severe, reproducible vertigo, ment. Table 8.5.4 lists some of the causes.
nausea and vomiting. Cranial nerve examina- Examination This should include a gen-
tion is normal apart from nystagmus. eral examination to look for signs in need
Hereditary ataxias/
of urgent intervention, for example meningi-
spinocerebellar degenerative
Metabolic disorders tis, shock, hypoglycaemia, raised ICP or head
Friedreichs ataxia is an autosomal recessive
A large number of metabolic disorders may injury. A complete examination is required
condition, which manifests in a child less
have ataxia as a feature and should be kept to detect signs that may aid in the diagnosis,
than 10 years of age with ataxia and nystag-
in mind. Hypoglycaemia and hyponatraemia such as abdominal masses, nystagmus, opso-
mus. There is usually rapid progression. On
may present with ataxias but other signs will clonus and myoclonus in neuroblastoma, or
examination there is impaired position and
also be present. The inherited metabolic dis- signs of infection or vasculitis.
vibration sense, positive Rombergs sign
eases will likely demonstrate episodes of A complete neurological examination
and absent tendon reflexes. The plantar
ataxia, together with the other features of should be performed and documented. Cer-
response is up going. Dysarthria is present.
the conditions, which will point towards ebellar signs should be carefully assessed.
Kyphoscoliosis, distal muscle wasting and
the diagnosis. There may be a progression Dysmetria and intention tremor can be
contractures and cardiomyopathy may
to chronic ataxias. Other inborn errors are assessed in the younger child by asking
develop. Ataxia telangiectasia is also an
listed in Table 8.5.4. him to point to the parts of a doll. Dysdiado-
autosomal recessive condition with neurocu-
chokinesis may, however, be difficult to per-
taneous manifestations. Ataxia is predomi-
Table 8.5.4 Causes of chronic ataxia form. Gait should be assessed. If only the
nantly truncal and becomes evident in
anterior lobe or the vermis are involved, gait
Brain tumours early childhood. Ocular and cutaneous telan-
Cerebellar astrocytoma may be affected but the upper limb spared.
Brainstem glioma giectasia become evident between 2 and 6
With truncal ataxia the child may have diffi-
Medulloblastoma years of age. Developmental delay, increased
Ependymoma culty keeping balance whilst seated. This
Cerebellar haemangioblastoma susceptibility to infection, due to thymic
imbalance increases if sitting cross-legged,
(Von HippelLindau) atrophy and IgA and IgE deficiency, and an
Hereditary ataxia standing or walking. Heeltoe walking tests
Friedreichs ataxia increased incidence of neoplasia are seen.
Ataxia telangiectasia
are useful in detecting cerebellar problems,
Dominant hereditary ataxia but unsteadiness may also be due to weak-
Olivopontocerebellar degeneration Congenital malformations
RoussyLevy syndrome
ness or sensory deficits. Sensory ataxia is dif-
Cerebellar aplasia/hypoplasia, Dandy
Hydrocephalus ferentiated from cerebellar ataxia by the
Congenital malformations Walker malformations, ArnoldChiari malfor-
Cerebellar aplasia/hypoplasia (autosomal
presence of impaired position and vibration
mations or vermal aplasia may result in ataxic
recessive) sense and a positive Rombergs test. Lesions
Vermal aplasia, including DandyWalker cerebral palsy which accounts for 10% of all
malformations
of the ipsilateral cerebellar hemispheres
cerebral palsy. Signs include, but are not lim-
ArnoldChiari malformations cause ataxia prominent in one direction.
Inborn errors of metabolism ited to, ataxia, hypotonia and tremor.
Hartnups disease
The child will fall towards the side of the
Wilsons disease lesion. Dysmetria and hypotonia are seen
Refsums disease
on the side of the lesion. Cerebellar dysar-
Abetalipoproteinaemia Evaluation of the patient
Maple syrup urine disease thria (scanning speech) may be detected
Argininosuccinicaciduria The ED assessment includes history and
Ornithine transcarbamoylase deficiency
by repeating sizzling sausage. The speech
Multiple carboxylase deficiencies a thorough examination to detect life- displays a slow onset and is a slurred, jerky
biotinidase deficiency threatening conditions and to identify revers-
Pyruvate dehydrogenase deficiency
sound with an explosive nature.
Juvenile GM2 gangliosidosis ible factors. Investigation is dependent on the Reflexes are often decreased in cerebellar
Juvenile sulfatide lipidosis formulation of a differential diagnosis.
Leighs syndrome
lesions and are absent in GuillainBarre syn-
Sphingolipidoses drome. Pendular knee jerks are seen in
Neuronal ceroid-lipofuscinosis
g-Glutamyl cysteine synthetase deficiency
History Important aspects of the history severe cases of cerebellar dysfunction and
Triosephosphatase isomerase deficiency include the timing of the ataxia. Is it acute, in those with associated pyramidal tract
Glucose transporter 1 deficiency syndrome
Vitamin B12, E or folate deficiency
recurrent or chronic? What is mainly defects. Decreased tone is seen in cerebellar
affected, is it the trunk or limbs? Are there lesions with drift and static tremor on

218
8.5 ACUTE ATAXIA
8

NEUROLOGY
holding up the arms. Rebound may also be 11. Gupta PC, Gathwala G, Aneja S, Arora SK. Acute

detected. Management cerebellar ataxia: An unusual presentation of

poliomyelitis. Indian Pediatr 1990;27(6):6223.
Nystagmus is horizontal in cerebellar 12. Curnen EC, Chamberlin HR. Acute cerebellar ataxia
Treatment is aimed initially at detecting and associated with poliovirus infection. Yale J Biol Med
lesions and maximal to the side of the lesion. treating emergency conditions. For example, 1962;34:219.
It may be positional. In phenytoin intoxica- 13. Thapa BR, Sahni A. Acute reversible cerebellar ataxia in
in hydrocephalus and tumours, raised intra- typhoid fever. Indian Pediatr 1993;30(3):427.
tion, nystagmus is initially horizontal but cranial pressure is treated in consultation 14. Marzetti G, Midulla M. Acute cerebellar ataxia associated
becomes vertical at higher levels. Nystag- with echo type 6 infection in two children. Acta Paediatr
with the neurosurgical unit. In meningitis, Scand 1967;56:547.
mus in vestibular neuronitis is typically urgent antibiotics are given. Hypoglycaemia 15. Batton FE. Ataxia in childhood. Brain 1905;28:487505.
rotational. The cranial nerves should be 16. Shimizu Y, Ueno T, Komatsu H, et al. Acute cerebellar
and other reversible metabolic causes are ataxia with human parvovirus B19 infection. Arch Dis
carefully examined to look for brainstem treated. Intoxications and poisonings are Child 1999;80(1):723.
involvement. 17. Dano G. Acute cerebellar ataxia associated with herpes
generally managed by supportive care. simplex virus infection. Acta Paediatr Scand
Acute cerebellar ataxia usually resolves 1968;57:151.
18. McMinn P, Stratov I, Nagarajan L, Davis S. Neurological
over weeks to months. Occasionally there manifestations of enterovirus 71 infection in children
Investigations is a persistent movement disorder or beha- during an outbreak of hand, foot and mouth disease in
Western Australia. Clin Infect Dis 2001;32(2):23642.
vioural and speech disorders. There is insuf-
Investigations are directed by the history 19. Senanayake N, de Silva HJ. Delayed cerebellar ataxia
ficient evidence to recommend either complicating falciparum malaria: A clinical study of 74
and clinical features detected on examina- patients. J Neurol 1999;241(7):4569.
immunoglobulin or steroid therapy and
tion. Acute cerebellar ataxia is primarily a 20. Sunaga Y, Hikima A, Ostuka T, Morikawa A. Acute
treatment is primarily supportive. Physio- cerebellar ataxia with abnormal MRI lesions after
diagnosis of exclusion and investigation varicella vaccination. Pediatr Neurol 1995;13(4):
therapy, occupational therapy and a wheel-
may be required to detect alternative condi- 3402.
chair for mobility may be required. 21. Deisenhammer F, Pohl P, Bosch S, Schmidauer C. Acute
tions where urgent intervention is required. cerebellar ataxia after immunization with recombinant
Appropriate drug assays may include eth- hepatitis B vaccine. Acta Neurol Scand 1994;89
(6):4623.
anol, ethylene glycol, anticonvulsants or 22. Hata A, Fujita M, Morishima T, et al. Acute cerebellar
benzodiazepines. If the drug screen is posi- Disposition ataxia associated with primary human herpesvirus-6
infection: a report of two cases. J Paediatr Child Health
tive in an appropriate clinical setting, no
Most children with significant ataxia require 2008;44(10):6079.
other tests may be required. 23. Maggi G, Varone A, Aliberti F. Acute cerebellar ataxia in
admission for investigation and observation children. Childs Nerv Syst 1997;13(10):5425.
Imaging by CT and/or MRI is indicated
under a paediatrician. Some children who 24. Connolly AM, Dodson WE, Prensky AL, Rust RS. Course
urgently where there are signs of raised and outcome of acute cerebellar ataxia. Ann Neurol
have mild ataxia, clearly due to post- 1994;35:6739.
intracranial pressure. MRI is indicated in
infectious cerebellitis, are appropriate to 25. Siemes H, Siegert M, Jaroffke B, Hanefield F. The CSF
most cases of acute ataxia unless there is protein pattern in acute cerebellar ataxia of childhood
be followed as an outpatient. and intracranial midline tumours. Eur J Pediatr 1982;137
a clear alternative diagnosis. (1):4957.
A lumbar puncture should be considered 26. Murphy JM, Motiwala R, Devinsky O. Phenytoin
intoxication. S Med J 1991;84:1199204.
when meningitis or encephalitis are possi- 27. Wilson JT, Huff JG, Kilroy AW. Prolonged toxicity following
ble. Differential of a space-occupying lesion References acute phenytoin overdose in a child. J Pediatr
1. Adams C, Diadori P, Schoenroth L, Fritzler M. 1979;951358.
or raised intracranial pressure may indicate Autoantibodies in childhood post-varicella acute 28. Booker HE, Darcey B. Serum concentrations of free
that a CTscan be performed prior to the lum- cerebellar ataxia. Can J Neurol Sci 2000;27:31620. diphenylhydantoin and their relationship to intoxication.
2. Van der Mass AAT, Vermeer-de Bondt PE, de Melker H, Epilepsia 1973;14:177.
bar puncture. Treatment with antibiotics Kemmeren JM. Acute cerebellar ataxia in the 29. Tibballs J. Acute toxic reaction to carbamazepine: Clinical
and antivirals, if indicated, should not be Netherlands: A study on the association with effects and serum concentrations. J Pediatr 1992;121
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Electrolytes, glucose and ammonia are Neurology 2005;65(7):11146. 31. Stremski E, Hennes H. Accidental isopropanol ingestion in
indicated to assess for metabolic causes. In 4. Shimizu Y, Ueno T, Komatsu H, et al. Acute cerebellar children. Pediatr Emerg Care 2000;16(4):23840.
ataxia with human parvovirus B19 infection. Arch Dis 32. Tibballs J. Clinical effects and management of eucalyptus
recurrent ataxia, or where there are features Child 1999;80(1):723. oil ingestion in infants and young children. Med J Aust
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pyruvate, cholesterol and lipoproteins. Mus- 8. Erzurum S, Kalavsky SM, Watanakunakorn C. Acute Baukloh G. Codeine intoxication in childhood. Lancet
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cle biopsy may be required. If occult neuro- infectious mononucleosis. Arch Neurol 1983;40 37. Warden CR, Diekema DS, Robertson WO. Dystonic
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9. Steele JC, Gladstone RM, Thanasophon S, Fleming P. in a toddler. Pediatr Emerg Care 1997;13(3):2145.
vanillic acid and vanillylmandelic acid levels Acute cerebellar ataxia and concomitant infection with 38. Bem JL, Peck R. Dextromethorphan: An overview of
are measured. Other investigations may Mycoplasma pneumoniae. J Pediatr 1972;80(3):4679. safety issues. Drug Saf 1992;7(3):1909.
10. Cohen HA, Ashkenazi A, Nussinovitch M, et al. Mumps- 39. Suchard J, Nizkorodov S, Wilkinson S. 1,4-Butanediol
include an electroencephalogram if seizures associated acute cerebellar ataxia. Am J Dis Child content of aqua dots childrens craft toy beads. J Med
are suspected. 1992;146(8):9301. Toxicol 2009;5(3):1204.

219
8.6 HEADACHE

40. Telander RL, Smithson A, Groover V. Clinical 42. Kaplan S, Goddard J, Van Kleeck M, et al. Ataxia and 44. Yaginuma M, Suenaga M, Shiono Y, Sakamoto M. Acute
outcome in children with acute cerebellar deafness in children due to bacterial meningitis. cerebellar ataxia of a patient with SLE. Clin Neurol
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1989;24(1):114. 43. Iff T, Donati F, Vassella F, et al. Acute encephalitis in Swiss 45. Rust RS. Multiple sclerosis, acute disseminated
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Geneeskd 2006;150(14):799803. 1998;2:2337. Neurol 2000;7(2):6690.

8.6 Headache
Alistair Murray John Ryan

a headache which will run a relatively

ESSENTIALS benign course, from that which may be a
symptom of significant underlying pathol-
1 The primary role of an emergency physician is to exclude a significant underlying ogy with immediate health implications.
cause for a headache, most urgently that of infection, raised intracranial pressure or
The overwhelming majority of headaches
an intracerebral bleed.
will be diagnosed on history and examination
2 While the majority of headaches in children presenting to the emergency alone, with little additional information arising
department (ED) are likely to be benign, appropriate investigation and follow up of from investigations.35 Furthermore, the vast
patients will ensure that serious causes are unlikely to be missed. majority of children that present to the emer-
gency department are likely to have head-
3 A thorough history and examination is likely to provide a diagnosis in the majority aches that are benign, but those that are
of children presenting to an ED with a headache.
not have the potential to be life threatening.
4 Chronic headaches need not necessarily be investigated on an emergent basis The classification of headaches is based
unless they are progressive. on the underlying aetiology.6 The Interna-
tional Headache Society has developed a
5 Red flags prompting further investigation of headache include: occipital headache; classification of headache, the second edi-
meningism; focal neurological signs; seizures; progressive nature; papilloedema;
tion of which was published in 2004 in
persistent vomiting; ataxia; presence of a ventriculoperitineal shunt; age younger than
Cephalgia and is also available on their web-
3 years; or early morning headaches, especially those that wake the child from sleep.
site.7 This classifies headache into three
broad categories most notably, primary or
secondary headaches and cranial neural-
gias central and primary facial pain and
other headaches (Table 8.6.1).
Introduction Incidence The causes of some headaches will be dealt
with in other chapters, e.g. Chapter 8.7 on
The aim of this chapter is to provide the Approximately 1% of all presentations to meningitis, while some of the primary head-
reader with a basic understanding of emergency departments have headache as ache disorders will be discussed in more detail
the aetiology of headaches in a paediatric the presenting complaint.1,2 Headaches in later in this chapter. We recommend an
population presenting to an ED, while offer- children are very common, with up to 75% approach whereby the emergency doctor con-
ing a functional and safe approach to the of children having had a headache of some siders each case by initially excluding the
management of paediatric patients with form by the age of 15.3 Despite the frequency, most sinister causes of the headache
headaches. Some key points should be very few paediatric patients with headaches (Tables 8.6.2 and 8.6.3).
remembered from the outset: ever consult their family physician or an ED.
However, this does not take account of pati-
Children may require different ents who present with a different complaint, Clinical assessment
approaches, depending on factors such as
such as a temperature, who might also have
age, ethnicity or socioeconomic status. History
a headache as part of a concomitant illness.
Children under 3 years rarely complain of The first step in any medical assessment is
headache. This should prompt the history and this is no less important in
consideration of an organic cause. the case of headache. Depending on the
Pathophysiology
The classical history of migraine from a age of the child, the history may be taken
teenager may be more easily elicited The causes of headache are myriad, but the from the child, the parent or indeed both.
than the history of symptoms from a primary aim of the emergency physician Important features of the headache to
shy 5-year-old. should be to differentiate the patient with explore in the history include:

220
8.6 HEADACHE
8

NEUROLOGY
the suspicion of an infectious origin. Other
Table 8.6.1 Summary of International Headache Society classification of headaches
(ICHD-2) inflammatory disorders may also cause a
pyrexia. Equally, the absence of pyrexia does
Primary headaches
not equal the absence of an infection. In the
Migraine
Tension-type headache ED, a febrile child with a headache requires
Cluster headaches and other trigeminal-autonomic cephalgias consideration of the possibility of meningitis
Other primary headaches
or encephalitis.
Secondary headaches
Headache attributed to head and/or neck trauma Progression
Headache attributed to cranial and/or cervical vascular disorders
Headache attributed to non-vascular intracranial disorder The temporal progression is of relevance
Headache attributed to a substance or its withdrawal in children with headaches. For example, a
Headache attributed to infection
Headache attributed to disorder of homeostasis classic migraine will last between 1 and 72
Headache or facial pain attributed to disorder of cranium, neck, eyes, ears, nose, sinuses, teeth or other hours in a child, while a patient with a chronic
facial or cranial structures.
Headache attributed to psychiatric disorder headache that is becoming progressively
more severe may well have an underlying
Cranial neuralgias, central and primary facial pain and other headaches
organic cause. This should prompt the emer-
Cranial neuralgias and central causes of facial pain
Other headache, cranial neuralgia, central or primary facial pain gency physician who encounters a child with
such a pattern, even if incidentally, to ensure
that neuroimaging is performed and that
urgent appropriate follow up is arranged.5

Nature
Onset of the headache
Table 8.6.2 Causes of headache in Headache of a throbbing nature is sugges-
children Sudden onset headaches can be considered
tive of migraine, while band-like headaches
differently in children compared to adults.
Infection are often tension in type. It has also been
The classical history of sudden onset head-
Meningitis, encephalitis, abscess suggested that the inability of a child to
Influenza ache being suggestive of subarachnoid
Systemic infection
describe the nature of the headache may in
haemorrhage in an adult is less relevant in
Sinusitis itself be a predictor of underlying pathology.3
Dental infection the case of the paediatric patient. In chil-
dren, the most frequent underlying cause
Vascular Behavioural change and avoidance
is an upper respiratory tract infection or pri-
Migraine behaviour
Intracranial haemorrhage mary headache.24 There is a significantly
Hypertensive encephalopathy
This is often noted in a collateral history.
higher proportion of underlying pathology
While entirely non-specific, it is particularly
Post lumbar puncture in cases of acute headache, compared to
important in raising suspicion of other
chronic headaches. It should also be noted
Raised intracranial pressure causes of a headache such as a school pho-
that the investigation of headache of acute
Brain tumour bia, drug misuse in the adolescent or indeed
Hydrocephalus onset is more properly the role of the emer-
Benign intracranial hypertension
may be a pointer towards sexual assault.8
gency physician, while chronic headaches
Trauma SDH, concussion
may be best investigated by the childs gen-
Toxic
Sleep disturbance
eral practitioner or paediatrician.
Carbon monoxide
Benign causes of headache rarely wake
Lead patients from their sleep and if a child is
Site being woken by a headache this should
Functional Classic migraine is typically unilateral.
Tension or cluster headache
prompt the consideration of further investi-
However, the case of childhood migraine is gation or referral.
Psychogenic a little different, often being bilateral. The
headache of meningeal irritation due to Postural symptoms
meningitis may involve neck pain. It should A headache that is worse on lying flat or exa-
also be noted that occipital headaches cerbated by coughing may be related to
are relatively uncommon in children and raised intracranial pressure. Early morning
have been reported to be an independent headaches after recumbency of sleep may
Table 8.6.3 Important causes of predictor of underlying pathology, particu- occur with raised intracranial pressure or
non-benign headache larly of the posterior fossa. space occupying lesions.
Brain tumours and hydrocephalus
Benign intracranial hypertension
Intracranial haemorrhage Pyrexia Neurological deficit
Meningitis encephalitis or abscess
Hypertensive encephalopathy The presence of a fever will often be reported A history of neurological deficit, albeit
by a parent and should immediately raise temporary, should be considered highly

221
8.6 HEADACHE

significant and should always be sought. of a thorough ear, nose and throat examina- (CT) scan may be normal and the diagnosis
This is particularly important, as some chil- tion. Occasionally a child with tonsillitis will based on an elevated opening pressure
dren may have subtle objective neurological present with headache, without any com- >20 cmH2O on lumbar puncture. This valu-
findings that could easily be overlooked in a plaint of a sore throat. Dental examination able test is frequently neglected in lumbar
hurried examination. Most mothers will may reveal percussion tenderness of an punctures performed in the ED, but should
have noticed an unusual posture or limp infection that may cause a referred temporal be done if one is considering benign intra-
but may not immediately mention it unless headache. The presence of nasal discharge cranial hypertension. The cerebrospinal fluid
prompted. The importance of this is re- or respiratory symptoms should prompt the (CSF) protein, glucose and cell counts are
inforced by the evidence that it may take clinician to seek evidence of sinus tender- normal in BIH. These children may present
an average of 7 months for a brain tumour ness. Likewise, it should be noted that, in with intermittent headache, vomiting,
to be diagnosed, with as many as three dif- most children, headaches due to uncompli- blurred vision or diplopia.
ferent consultations with a physician. This is cated upper respiratory tract infections can Retinal haemorrhages should be sought
despite the fact that a significant proportion be expected to settle with simple analgesics. on ophthalmoscopy. Their presence is con-
of children with tumours have abnormal The persistence of headache should prompt sidered as evidence of significant trauma,
neurological examinations.9 the consideration of further investigation. in the absence of any other causes such
The temperature should be recorded on as hypertension or diabetes mellitus. It is
Family history (especially more than one occasion. Likewise, it is impor- difficult, if not at times impossible, to
of migraine) tant to measure the blood pressure as head- achieve successful examination of the fundi
Approximately 7090% of diagnosed cases of ache may be the presenting feature of and retina of the younger child. Occasion-
migraine have a positive family history. There- coarctation of the aorta or underlying hyper- ally one may require mydriatic eye drops
fore diagnosing a migraine in the absence of a tension. The blood glucose is relevant if the to dilate the pupils or seek the opinion
family history should be done with caution. history suggests hypoglycaemia. It is useful of a suitably qualified senior colleague.
to measure head circumference and plot this A deferred formal retinal assessment by
Analgesic use on centile charts, even for the older child. A an ophthalmologist may be required in
Abolition of a headache with simple analge- disproportionately large head should cases where the possibility of non-accidental
sics or restoration of normal activities is prompt further investigation5 and the ten- injury exists. Eye movements must be
more common in those with upper respira- sion should be palpated in infants who have carefully examined as subtle nerve palsies
tory tract associated headaches. It should an open anterior fontanelle. may be apparent early in children presen-
also be noted that chronic daily use of A careful examination may reveal subtle ting with a space-occupying lesion or
analgesics is in itself a cause of headaches.10 evidence of trauma, which may or may not hydrocephalus.
have been sustained accidentally. A mild The main consideration in a cardiovascu-
Examination post-concussion headache may persist for lar examination should be directed at the
A number of specific points should be con- a number of days after a head injury, but exclusion of hypertension and the palpation
sidered when examining the child with a may require imaging to exclude a possible of femoral pulses, checking for evidence of
headache. Headache may be a secondary subdural haematoma, particularly where coarctation of the aorta.
symptom of a more generalised illness. A the headache is severe, responds poorly to Abdominal examination is usually normal
comprehensive physical examination is analgesia, is associated with vomiting or is but is nevertheless important, as nausea,
therefore mandatory. Observation of a child prolonged. Headache has been retrospec- vomiting and abdominal pain are frequently
from a distance may be valuable. A child tively described as being more common in associated with headache in children. It is
lying quietly, unresponsive to the environ- victims of sexual abuse. The febrile, toxic- recognised by the International Headache
ment, should prompt earlier assessment appearing child should be examined for Society that cyclical vomiting and abdomi-
than an active child who interacts normally evidence of nuchal rigidity and other signs nal migraine are distinct entities and not
with healthcare staff. One must also con- of meningeal irritation, which may be infrequently progress to the typical pattern
sider other causes of headache that are present in meningitis. In the child who has of migraine in adulthood. Hypertension
not readily discernible. In some cases there a ventriculoperitoneal shunt, the reservoir of renal origin may be suggested by the
may be few clinical signs, such as with car- should be assessed by palpation (see presence of polycystic kidneys.
bon monoxide poisoning from houses with Chapter 8.1).8 A standard neurological examination
poorly ventilated gas boilers.11 Heavy metal Ophthalmological examination including should be performed. Specific features that
poisoning is another rare cause. fundoscopy is particularly important as it should be sought include papilloedema,
A focused examination should be directed may influence immediate management. ataxia, hemiparesis, abnormal eye move-
on the basis of the history. Vital signs may The presence or absence of papilloedema ments and abnormal tendon reflexes.4
provide valuable keys to the aetiology. A sig- should be specifically sought as this may The child should be fully undressed.
nificant proportion of children who present be the only objective finding in cases of In particular, a rash or abnormality of pig-
with headache will have a diagnosis of respi- benign intracranial hypertension (BIH). In mentation should specifically be sought.
ratory tract infection, hence the importance this condition the computerised tomography The most urgent of these is the search for

222
8.6 HEADACHE
8

NEUROLOGY
the petechial rash of meningococcal disease, of an elevated opening pressure is diag-
Table 8.6.4 Red flags prompting further
but examination may also reveal neurofibro- investigation of headache nostic in children with benign intracranial
mas and cafe-au-lait spots associated with hypertension.
Occipital headache
neurofibromatosis or a pigmented patch
associated with tuberous sclerosis. Meningism
Other investigations
Focal neurological signs
Magnetic resonance imaging (MRI) is an
Investigation Chronic progressive headaches investigation that may show increasing util-
The issue of how to investigate a child Persistent vomiting ity in the future as its availability becomes
with a headache in the ED is not clearly more widespread. It has the advantage of
Seizures
defined and remains contentious. It is clear no radiation dose and provides more
that in the majority of cases the cause Papilloedema detailed images of the brain.
of children presenting to the emergency Focal neurological symptoms Other investigations such as electroen-
department with a headache is a respira- cephalogram, electromyogram, SPECT, posi-
Ataxia
tory tract infection or primary headache tron emission tomography, autonomic
syndrome. The key role of the emergency Presence of a VP shunt testing and transcranial Doppler have not
physician is to diagnose those headaches Age younger than 3 years clearly demonstrated their utility in the
that may have significant underlying acute setting.13
Abnormal eye movements
pathology. Investigation should be driven
by the history and by findings on exami- Early morning headaches especially those that
wake the child from sleep.
nation, as the majority of diagnoses should
be suggested by these and confirmed by VP, ventriculoperitoneal. Management
appropriate diagnostic tests. Symptomatic treatment in the ED of most
patients may be better served by redirection headaches in children can be achieved by
CT scanning to their family physician or paediatrician. use of oral analgesics such as paracetamol,
Numerous studies have assessed the value codeine or ibuprofen. Headaches that
of investigating a child with headache, Blood tests require stronger analgesics to control pain
with most focusing on the relevance of Laboratory-based testing has a very low are an indication for admission for further
neuroimaging.3,12,13 yield in children with headaches. Occasion- evaluation.
A CT scan cannot be considered a benign ally they can alert the clinician to more seri-
test as it carries with it a risk in relation to ous underlying pathology, but their value is
the sedation and transfer of a child and with diagnostically limited in the emergency
setting. They should not delay the progres-
Disposition
regard to radiation exposure. A CT head
scan carries a 1:1500 risk of developing a sion to more specific investigation and The disposition of a patient from the ED
subsequent cancer directly related to that treatment. Indeed, with the increasing use should be considered on an individual basis,
radiation exposure.14 of near patient testing much of the useful though some guidelines may be helpful in
In the setting of an acute atraumatic information in this regard can be obtained minimising the risk of missing a significant
headache, the absence of focal neurology with the use of a venous blood gas, which diagnosis. The increasing use of observation
or other red flags (as listed later in this provides information on pH, electrolytes, wards can prove helpful in selected cases.
chapter), a CT scan is unlikely to be of signif- haemoglobin and even carboxyhaemoglo- Such a facility is most useful in the case
icant value (Table 8.6.4). bin levels. where an infective cause of the headache
A guide to CT scanning in the context of is suspected.
trauma is considered separately. Lumbar puncture All patients who present to the ED with
In the context of a chronic, non- Lumbar puncture is mandatory in any case headache should have a follow-up appoint-
progressive headache with a normal neuro- of suspected meningitis or encephalitis. It ment in the short term with either their fam-
logical examination, CT is unlikely to be of is a reassuring test when normal and often ily physician or a paediatrician, depending
value. This is different to a chronic progres- diagnostic when abnormal. It should be on each case. This will ensure that not only
sive headache, which is a concerning feature noted, however, that post lumbar puncture are high standards of care maintained, but
and more likely to harbour underlying headache is also a recognised complication unnecessary acute investigations can be
pathology. However, this artificially selects in children, as it is in adults.15 Such an inva- avoided that would otherwise be ordered
a cohort of patients that are unlikely to be sive procedure may often be emotionally in order to exclude every possible cause of
seen in the emergency department. The traumatic for a child and should not be headache. Furthermore, there may be psy-
investigation of chronic headaches is not ordinarily recommended as a routine inves- chosocial aspects of relevance to childhood
the role of the emergency physician, and tigation in the diagnosis of headache. headaches that may be more appropriately
in the absence of an acute deterioration A lumbar puncture with measurement dealt with by the family physician.

223
8.6 HEADACHE

It is also apparent that serotonin is inte- a headache appear to have a more benign
MIGRAINE grally involved, hence the increasing popu- cause to their headache.
larity of the use of serotonin agonists such Up to 90% of patients with migraine have
as sumatriptan in the acute treatment of a positive family history, although it is note-
ESSENTIALS adult migraine. worthy that only one migraine gene has
been identified and this is associated with
1 Family history of migraine is very
only 50% of the cases of familial
common and a diagnosis of migraine
hemiplegic migraine, a particularly rare
in the absence of a family history Clinical features condition.5
should be carefully considered.
Migraine without aura is more common than A number of other conditions are consid-
2 Unlike adult migraine, simple migraine with aura in children.17 A number ered to be migraine disorders in childhood,
analgesics are often effective in the of key features are listed below: most notably cyclical vomiting and abdomi-
context of childhood migraine. International Headache Society diagnos- nal migraine. In these conditions there may
tic criteria of migraine without aura:7 be recurrent episodes of severe abdominal
3 Childhood migraine is often
pain with associated vomiting but the
bilateral. A. History of at least five attacks, fulfilling
examination and investigation of the chil-
criteria BD
4 Childhood migraine may be shorter- dren is often normal. These conditions are
B. Last 172 hours (untreated or
lived than in adults (172 hours). a diagnosis of exclusion, but their consider-
unsuccessfully treated)
ation may lead to appropriate referral and
5 Migraine is frontotemporal. C. Headache with at least two of the
follow up as they carry significant morbidity.
Occipital headache suggests a following:
Migraine variant disorders may cause
sinister cause of headache. Headaches pulsating in quality transient abnormal neurological findings,
Headaches may be unilateral or such as hemiplegia, ophthalmoplegia or
bilateral (unlike adult migraine
confusional state. These conditions are rare
which is typically unilateral), not
Childhood migraine is a common condition and an abnormal examination should
occipital
presenting to the ED. The diagnosis is often prompt the emergency physician to investi-
based on an adequate history but the diag-
 Headaches exacerbated by routine
gate further.
activities (walking or climbing stairs)
nosis can be a little more difficult to make
in the younger age group. The emergency
The headache is moderate or severe
D. At least one of the following Investigation
physician should not diagnose migraine in
children less than 3 years old. Childhood
Nausea and/or vomiting Migraine as a distinct entity has no specific

migraine also has a wider spectrum of symp-

Photophobia or phonophobia (may diagnostic test. Key to the diagnosis is the
be inferred from childs behaviour). history, as described above, with a normal
toms than adult migraine and should be con- neurological examination. Investigations
sidered in a patient who has recurrent The presence of an occipital headache should be requested on the basis of exclud-
abdominal pain, with normal investigations. suggests a structural abnormality and not ing other pathology as the underlying cause
It is important to remember that although migraine. of headache. It is notable that although a
the diagnosis of migraine is common, it is In migraine with aura the headache is pre- significant number of migraine sufferers
principally a diagnosis of exclusion. ceded by a period of altered perception that have abnormal EEGs, these add little diag-
may take numerous forms. This period of nostic value and on current evidence should
altered perception can last up to 60 min- remain a research tool.13
utes. Examples are positive visual symptoms
(flickering lights, spots, lines), negative
Pathophysiology
visual symptoms (loss of vision, including
Treatment
The exact mechanism of migraine is complex partial), sensory symptoms (pins and nee-
and it has only recently become better under- dles) or fully reversible speech disturbances Most cases of childhood migraine can be
stood. More recently, it is felt to be related to (dysphasia). This period of altered percep- adequately managed with simple analgesics
a hyperexcitable cerebral cortex, leading to tion can last up to 60 minutes prior to the such as paracetamol (15 mg kg 1) and ibu-
cortical spreading depression (CSD), leading onset of the headache.7 profen (10 mg kg 1), although ibuprofen
in turn to activation of the trigeminal nerve Assessing the severity of headache in a appears superior.18,19 As nausea can often
and its associated vessels. CSD can lead to child can prove challenging. Children almost be a significant feature, prochlorperazine
areas of oligaemia of the cortex and may universally point to the unhappiest face on a appears to be of benefit.20
be responsible for the aura associated with visual analogue scale when describing a At present the triptan group of drugs
migraine. Following this, the trigeminal nerve headache. More reliable answers may be remain unlicensed for paediatric use in
afferents appear to be sensitised and may achieved by asking about quality of life many countries but there is now evidence
account for the fact that ordinary activities issues such as ability to continue playing. for the use of nasal sumatriptan in adoles-
can significantly exacerbate the migraine.16 Children who can continue playing during cents and possibly even in those as young

224
8.6 HEADACHE
8
as 8.18,21 The evidence behind the use of

NEUROLOGY
neurological symptoms/signs or evidence 10. Vasconcellos E, Pina-Garza JE, Millan EJ, Warner JS.
Analgesic rebound headache in children and adolescents.
other triptans, or routes of administration, of abnormal physiology should be rapidly J Child Neurol 1998;13(9):4437.
is currently limited in the paediatric assessed, with early referral for diagnostic 11. Hampson NB, Hampson LA. Characteristics of headache
associated with acute carbon monoxide poisoning.
population. facilities and neuroimaging being a priority. Headache 2002;42(3):2203.
There are very limited data regarding pro- Children with chronic symptoms or recur- 12. Lewis DW, Dorbad D. The utility of neuroimaging in the
evaluation of children with migraine or chronic daily
phylactic treatment of migraine in children rence of chronic symptoms should have a headache who have normal neurological examinations.
and hence no clear recommendations can thorough examination and treatment. Fol- Headache 2000;40(8):62932.
13. Sandrinia GFL, Janigc W, Jensend R, et al.
be given, other than ensuring appropriate low up should be arranged with the patients Neurophysiological tests and neuroimaging procedures
follow up of these cases. paediatrician or general practitioner. in non-acute headache: guidelines and
recommendations. Eur J Neurol 2004;11:21724.
14. Brenner D, Elliston C, Hall E, Berdon W. Estimated risks of
radiation-induced fatal cancer from pediatric rCT. Am J
Radiol 2001;176:28996.
Disposition 15. Janssens E, Aerssens P, Alliet P, et al. Post-dural puncture

All patients with a discharge diagnosis of

References headaches in children. A literature review. Eur J Pediatr
2003;162(3):11721.
1. Conicella EMRU, Vanacore N, Vigevano F, et al. The child 16. Lewis D. Toward the definition of childhood migraine.
migraine should be followed up to ensure with headache in a pediatric emergency department. Curr Opin Pediatr 2004;16:62836.
that the headache resolves within the Headache 2008;48(7):100511. 17. Bulloch BTM. Emergency department management of
2. Burton LJ, Quinn B, Pratt-Cheney JL, Pourani M. pediatric migraine. Pediatr Emerg Care 2000;16
expected timeframe and also to ensure that Headache etiology in a pediatric emergency department. (3):196201.
it does not progress. Migraine can carry sig- Pediatr Emerg Care 1997;13(1):14. 18. Lewis D, Ashwal S, Hershey A, et al. Practice Parameter:
3. Kan L, Nagelberg J, Maytal J. Headaches in a pediatric Pharmacological treatment of migraine headache in
nificant impairment to lifestyle, if unrecog- emergency department: etiology, imaging, and children and adolescents. Report of the American
nised. Follow up should be arranged with treatment. Headache 2000;40(1):259. Academy of Neurology Quality Standards Subcommittee
4. Lewis DW, Qureshi F. Acute headache in children and and the Practice Committee of the Child Neurology
the primary care physician, with review of adolescents presenting to the emergency department. Society. Neurology 2004;63:221524.
medication and parental understanding Headache 2000;40(3):2003. 19. Lewis DW, Kellstein D, Dahl G, et al. Childrens ibuprofen
5. Lewis DW. Headaches in children and adolescents. Am suspension for the acute treatment of pediatric migraine
of the condition. Children with frequent Fam Physician 2002;65(4):62532. headache. Headache 2002;42:7806.
migraine warrant referral to a paediatrician. 6. Society IH. International Headache Classification (ICHD-2). 20. Bailey B, Cummins McManus B. Treatment of children
Cephalalgia 2004;24:1160. with migraine in the Emergency Department. A
7. IHS. International Classification of Headache Disorders qualitative systematic review. Pediatr Emerg Care
2 (ICHD-2). 2006. http://ihs-classificationorg/en/. 2008;24(5):32130.
8. Golding JM. Sexual assault history and headache: five 21. Ahonen K, Hamalainen M, Rantala H, Hoppu K.
Conclusions general population studies. J Nerv Ment Dis 1999;187 Nasal sumatriptan is effective in the treatment
(10):6249. of migraine attacks in children. Neurology 2004;62:
Patients with headaches commonly present 9. Mehta V, Chapman A, McNeely PD, et al. Latency 883887.
between symptom onset and diagnosis of pediatric brain
to paediatric EDs. Those children presenting tumors: an Eastern Canadian geographic study.
with new onset symptoms, significant Neurosurgery 2002;51(2):36572; discussion 723.

225
 8.7 CNS infections: meningitis
and encephalitis
Mike Starr

In Australia, meningococcal disease, par-

ESSENTIALS ticularly that caused by serogroup C, has
declined since introduction of the meningo-
1 Bacterial meningitis can be rapidly progressive, and result in substantial morbidity coccal C conjugate vaccine (MenCCV) into
and mortality.
the National Immunisation Program (NIP)
2 A high index of suspicion of the possibility of meningitis must be maintained in any schedule. The most common N. meningitidis
sick infant or child. serogroup causing invasive disease in those
under 19 years is serogroup B (91%),
3 Symptoms and signs are frequently non-specific, particularly with younger age. followed by serogroup C (23%).1 Until
4 Antibiotic treatment must not be delayed, although steroids can improve outcome the introduction of 7-valent conjugate pneu-
if given before the first dose of antibiotic, and continued 6-hourly for 4 days. mococcal vaccine (7vPCV) into the NIP
schedule, the most common pneumococcal
5 Careful management of fluid and electrolyte balance is critical. serotypes causing invasive disease were
6 Careful evaluation for features suggesting raised intracranial pressure should be those contained in 7vPCV (14, 6B, 18C,
done at the initial assessment. 19F, 4, 23F, 9V).2 However, since then, it
appears that serotype replacement is occur-
7 Conjugate haemophilus, meningococcal and pneumococcal vaccines have reduced ring; nasal carriage of 7PCV serotypes has
the incidence of bacterial meningitis significantly.
been replaced by others, namely 19A and
8 HSV encephalitis must be considered in any child with features of meningitis with 16F,3 and the rate of invasive pneumococcal
encephalopathy. disease caused by 7vPCV serotypes has
decreased significantly.4 However, the rate
9 Empiric aciclovir must be given early when encephalitis is suspected. of invasive pneumococcal disease caused by
serotype 19A increased in non-Indigenous
people and in the population overall.4 Antibi-
may be the result of a localised or systemic
Introduction otic resistance is almost exclusively restricted
insult, but is most commonly caused by
to these serotypes (and remaining 7PCV
Meningitis and encephalitis are medical viruses. Fungal meningitis usually occurs in
serotypes).
emergencies that require prompt assess- immunocompromised children.
ment and treatment. Meningitis is inflam-
mation of the meninges that surround the Viruses
Aetiology
brain and spinal cord. Inflammatory cells Enteroviruses, including coxsackie and echo-
Bacteria
invariably spill into the cerebrospinal fluid viruses, cause 8595% of cases of viral
The bacterial causes of meningitis vary with
(CSF) from the meninges, producing an meningitis. Herpes simplex viruses 1 and
the age of the child. In infants less than 23
increased cell count. Encephalitis is inflam- 2 (HSV-1 and HSV-2) and other herpes
months old, organisms acquired from the
mation involving the brain parenchyma, viruses (human herpesviruses 6, 7 and 8,
maternal genital tract predominate: group
which may occur in conjunction with inflam- varicella-zoster virus, cytomegalovirus and
B streptococci, Escherichia coli and Listeria
mation. There is considerable overlap EpsteinBarr virus) tend to cause meningo-
monocytogenes. In older children and adults
between the features of meningitis and encephalitis. HSV-1 and HSV-2 are perhaps
the most common causes are Neisseria
encephalitis, and the two may co-exist. the most important causes to consider, as
meningitidis and Streptococcus pneumoniae.
Encephalitis should be considered if a child meningoencephalitis caused by these
Other causes, including Staphylococcus spe-
has encephalopathy, convulsions or neuro- viruses is associated with high morbidity
cies and Gram-negative bacilli, are occasion-
logical deficits. and mortality, which may be reduced with
ally seen in immunocompromised hosts or
early treatment with antiviral medications.
following trauma or neurosurgery. Haemo-
philus influenzae type b (Hib) is rarely seen
as a cause now because of widespread Fungi
Meningitis
immunisation. Mycobacterium tuberculosis Cryptococcus neoformans is the most com-
Classification is rare, other than in children who have mon fungal cause of meningitis, but occurs
Meningitis is usually broadly classified as spent prolonged periods in countries of high almost exclusively in immunocompromised
bacterial or aseptic. Aseptic meningitis prevalence. hosts.

226
8.7 CNS INFECTIONS: MENINGITIS AND ENCEPHALITIS
8

NEUROLOGY
Clinical findings intracranial pressure (ICP). It may be difficult and this may remain so for more than 24
The classical features of meningitis comprise to determine whether ICP is raised, but the hours.11
fever, headache, vomiting, neck stiffness, following signs may be indicative: Organisms are seen on CSF Gram stain in
photophobia and altered mentation. How- 6080% of cases of bacterial meningitis,
ever, the clinical manifestations are often
coma (absent or non-purposeful response provided that prior antibiotics have not been
to painful stimulus);
non-specific, particularly in infants and given. The sensitivity is highest in pneumo-
young children. They may include fever, irri-
abnormal pupillary responses;
coccal meningitis. Prior antibiotics may pre-
tability, lethargy, poor feeding or vomiting.
abnormal posturing;
clude culture of the causative organism, but
Up to 58% of children with meningitis have
focal neurological signs or seizures;
the biochemistry and white cell count
received antibiotics before the emergency
recent (within 30 minutes), prolonged
remain abnormal for several days whether
(over 30 minutes) or tonic seizures;
department (ED) presentation.5 This may or not antibiotics have been given.
modify the clinical presentation of meningi-
papilloedema although this is an
A traumatic tap occurs in 1520% of LP
unreliable and late sign of raised ICP.
tis.6 It is therefore important to consider the in children.12,13 Several formulae have been
possibility of central nervous system (CNS) A bulging fontanelle, in the absence of other devised for interpretation of CSF contami-
infection in any sick infant or child, particu- signs of raised ICP, is not a contraindication nated with blood, but the safest practice is
larly if they are already taking antibiotics. to LP. to disregard the red cell count and treat if
If the fontanelle is still open, it may be The threshold for performing an LP should meningitis is suspected until culture and
bulging when examined with the infant in be lower in young children with less specific other studies are clearly negative.
a sitting position. Photophobia is difficult signs or those who have been taking antibio- Seizures do not result in increased CSF cell
to ascertain in young children, and other tics prior to presentation. count in the absence of meningitis.
signs of meningeal irritation may be absent Cerebral computerised tomography (CT) Bacterial or viral DNA can be detected in
or difficult to elicit. Resistance to being should not be used to decide whether it is blood and/or CSF using polymerase chain
picked up or distress on walking may be safe to proceed with LP or not. In a prospec- reaction analysis (PCR). Sensitivity and spec-
the only clues. Kernigs sign (inability to tive study of bacterial meningitis, CT find- ificity are high, particularly for N. meningiti-
extend the knee when the leg is flexed at ings obtained during the acute stages dis, HSV and enterovirus. PCR for N.
the hip), Brudzinskis sign (bending the head failed to reveal any clinically significant meningitidis is particularly useful in patients
forward produces flexion movements of the abnormalities that were not suspected on with a clinical picture consistent with menin-
legs) and nuchal rigidity may be present in neurological examination.8 Moreover, cere- gococcal meningitis, but who have received
older children, but have even been shown bral herniation can occur with a normal prior antibiotics.
to have low positive and negative predictive CT9,10 and the true cause of coning and rela- Latex agglutination allows rapid detec-
value in adults with meningitis.7 tionship to prior lumbar puncture is not tion of bacterial antigens in CSF and urine.
Rashes may occur with any bacterial men- clearly established. However, it lacks sensitivity and specificity,
ingitis, although are less common with pneu- Other relative contraindications to LP other than for Hib, and is therefore not clini-
mococcal infection. Petechiae or purpura are include: cally useful.
suggestive of meningococcal sepsis, but may
also occur in Hib and viral meningitis.
cardiovascular compromise or shock
Enteroviral meningitis may even be asso-
respiratory compromise Other investigations
coagulopathy or thrombocytopenia.
ciated with florid purpura fulminans. Culture of blood, throat swab, stool/
It is impossible to reliably differentiate rectal swab or of skin lesion may yield a
between bacterial and viral meningitis on causative organism.
clinical grounds. However, children with Examination of the CSF Gram stain on blood smear may be
enteroviral meningitis are more likely to A CSF specimen should always be sent for positive.
present in summer or autumn with gradual urgent microscopy to help guide empiric Blood glucose should be measured at the
onset of non-specific constitutional symp- treatment. Normal CSF is clear and contains same time as CSF glucose.
toms including diarrhoea, cough and myal- few cells (and no neutrophils). As few as A baseline sodium should be measured.
gia, in addition to the more typical features. 200
106 cells per litre will cause CSF to Hyponatraemia occurs in about one third
appear turbid. The CSF profile may help dif- of children with meningitis, and may be
ferentiate between bacterial and viral men- due to increased antidiuretic hormone
Investigations ingitis, but the findings vary. Table 8.7.1 secretion, increased urine sodium losses,
Definitive diagnosis of meningitis relies on indicates the typical profiles in normal chil- and excessive electrolyte-free water
examination of the CSF with biochemical dren and those with meningitis. However, intake or administration.
analysis, microscopy and culture. Children these should always be interpreted in the Full blood count and acute phase
with suspected meningitis should have context of the clinical picture. In early bacte- reactants (e.g. C-reactive protein) may
a lumbar puncture (LP) performed, unless rial meningitis, the CSF cell count may be provide supportive information, but are
there is a clear contraindication. The normal. In enteroviral meningitis, there is more useful when measured serially to
only absolute contraindication is raised typically a neutrophil predominance early, monitor the response to therapy.

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8.7 CNS INFECTIONS: MENINGITIS AND ENCEPHALITIS

Table 8.7.1 CSF findings in meningitis

White cell count Biochemistry

Neutrophils (
106 L1) Lymphocytes (
106 L1) Protein (g L1) Glucose (CSF:blood ratio)

Normal (>1 month of 0 5 <0.4 0.6 (or 2.5 mmol L1)

age)

Normal (<1 month of 0* <20 <1.0 0.6 (or 2.1 mmol L1)
age)

Bacterial meningitis " (but may be normal) " (usually <100) " (but may be normal) # (but may be normal)

Viral meningitis " (usually <100) " (but may be normal) 0.41.0 (but may be normal) Usually normal

Encephalitis " (usually <100) " (but may be normal) 0.41.0 (but may be normal) # (but may be normal)

*Some studies have found up to 5% of white cells in neonates without meningitis comprise neutrophils.

Management mortality and severe morbidity with pneumo- be monitored closely, and the total fluid
Antibiotics coccal meningitis.16 A recent Cochrane meta- intake adjusted accordingly.
Following initial fluid resuscitation, the analysis including adult and paediatric trials Initial fluid resuscitation to treat shock
emphasis is on prompt commencement of par- concluded that adjuvant steroids are benefi- should be with 20 mL kg1 of isotonic (normal)
enteral antibiotics. Delay in antibiotic therapy cial for children with bacterial meningitis.17 saline. Thereafter, isotonic fluids should be
has been associated with adverse clinical out- Evidence from animal studies shows that given to maintain systemic blood pressure
come in adults with bacterial meningitis.14 dexamethasone reduces penetration of van- (and thereby cerebral blood flow). Previous
comycin into infected cerebrospinal fluid.18 guidelines have emphasised the importance
Age <3 Amoxicillin 50 mg kg1
Thus, there is concern that use of dexameth- of fluid restriction.19 It may be necessary
months: intravenously (IV) 12-hourly
asone with vancomycin could compromise the to restrict fluids if the serum sodium is
(week 1 of life), 8-hourly (week
efficacy of vancomycin in third generation <130 mmol L1, or if there are signs of fluid
24 of life), 46-hourly
cephalosporin-resistant strains. Fortunately, overload. However, fluid restriction does not
(> week 4 of life) plus
the majority of cases of pneumococcal menin- generally improve outcome20 and has even
Cefotaxime 50 mg kg1 IV
gitis are still caused by strains that are suscep- been associated with a worse neurological
12-hourly (week 1 of life),
tible to penicillin and third-generation outcome.21 Thus, most children should receive
6-hourly (>1 week of age).
cephalosporins. normal maintenance fluid volumes.
Because of the morbidity associated with Accordingly, children (>4 weeks old) who
neonatal Gram-negative meningitis, and the are being treated for possible meningitis Prevention
high rates of recrudescence, some neonatol- (who have not yet received parenteral anti- Contacts of patients with meningococcal
ogists add gentamicin to the above regimen. biotics, or who have received their first dose meningitis may require chemoprophylaxis
less than 1 hour previously) should be given to prevent secondary spread. Those who
Age >3 Cefotaxime 50 mg kg1 (max
dexamethasone 0.25 mg kg1 IV (max should receive chemoprophylaxis include:
months: 2 g) IV 6-hourly
10 mg) (followed by 0.25 mg kg1 6-hourly).
the index case if treated only with
Rates of resistance to penicillin and cepha- Steroids should preferably be given 1530 penicillin (does not eradicate carriage);
losporins amongst pneumococci have fallen in minutes before antibiotics, although anti-
all intimate, household or day-care
Australia since the introduction of the conju- biotic administration should not be delayed contacts who have been exposed to the
gate pneumococcal vaccine.3,4,15 Vancomycin for more than 30 minutes. index case within 10 days of onset;
should be added if rates of resistance are high. any person who gave mouth-to-mouth
Fluids
resuscitation to the index case, or had
Careful management of fluid and electrolyte
direct contact with their airway secretions.
Steroids balance is important in the treatment of
Routine administration of steroids as adjunc- meningitis. Over or under hydration are Although nosocomial transmission of
tive therapy has been controversial in the associated with adverse outcomes.1921 meningococcal infection is rare, droplet pre-
past. The evidence that they protect against Many children have increased antidiuretic cautions are recommended until the patient
neurological (particularly audiological) com- hormone secretion, and some will have has received 24 hours of antibiotic therapy.
plications of childhood meningitis is strongest dehydration due to vomiting, poor fluid As the aetiology is usually unknown during
for Hib meningitis, when dexamethasone is intake or septic shock. Assessment of the this period, all cases of suspected bacterial
given before the first dose of antibiotics, clinical signs of hydration, including weight, meningitis should be managed in this way.
and when a third generation cephalosporin measurement of the serum sodium, docu- Preferred placement is in a single room.
is used. A recent large European trial in mentation of urine output, and clinical However, if a spatial separation of >1 metre
adults with meningitis showed reduction in assessment of the neurological state should can be achieved and curtains can be drawn

228
8.7 CNS INFECTIONS: MENINGITIS AND ENCEPHALITIS
8

NEUROLOGY
between the infected patient and other other herpes viruses and Mycoplasma pneu-
patients and visitors, this may be sufficient. moniae. Several other viruses cause seasonal References
epidemics of encephalitis in specific geo- 1. Annual report of the Australian Meningococcal Surveillance
Programme, 2008. Commun Dis Intell 2009;33(3):25967.
Chemoprophylaxis graphic areas. Examples include Murray Val- 2. Liu M, Andrews R, Stylianopoulos J, et al. Invasive
Rifampicin 10 mg kg1 (5 mg kg1 <1 ley encephalitis in the east Kimberley pneumococcal disease among children in Victoria.
Commun Dis Intell 2003;27(3):3626.
month old) orally 12-hourly (max 600 mg) region, and Japanese B encephalitis in the 3. Leach A, Morris P, McCallum G, et al. Emerging
Torres Strait Islands. pneumococcal carriage serotypes in a high-risk
for 2 days or population receiving universal 7-valent pneumococcal
Ceftriaxone 125 mg (12 years)/250 mg conjugate vaccine and 23-valent polysaccharide vaccine
since 2001. BMC Infect Dis 2009;9:1219.
(>12 years) mg intramuscularly as a 4. Giele C, Anthony K, Lehmann D, Van Buynder P. Invasive
single dose or Clinical findings pneumococcal disease in Western Australia: emergence
Ciprofloxacin 500 mg orally as a single dose. There is considerable overlap between the fea- of serotype 19A. Med J Aust 2009;190:166.
5. Kallio MJ, Kilpi T, Anttila M, Peltola H. The effect of a recent
tures of meningitis and encephalitis, and the previous visit to a physician on outcome after childhood
7vPCV and MenCCV vaccines are part of the two frequently co-exist. Encephalitis should bacterial meningitis. JAMA 1994;272(10):78791.
routine NIP schedule for all children and 6. Rothrock SG, Green SM, Wren J, et al. Pediatric bacterial
be suspected if there is encephalopathy, that meningitis: is prior antibiotic therapy associated with an
should have a continuing impact on the is, a change of behaviour, altered conscious altered clinical presentation? Ann Emerg Med 1992;21
number of cases of childhood meningitis. (2):14652.
state or extreme drowsiness. Convulsions (par- 7. Thomas KE, Hasbun R, Jekel J, Quagliarello VJ. The
ticularly focal) and focal neurological deficits diagnostic accuracy of Kernigs sign, Brudzinskis sign, and
Complications are also more common in encephalitis. nuchal rigidity in adults with suspected meningitis. Clin
Infect Dis 2002;35(1):4652.
Bacterial meningitis is associated with a 8. Cabral DA, Flodmark O, Farrell K, Speert DP. Prospective
4.5% mortality rate, and intellectual, cogni- study of computed tomography in acute bacterial
meningitis. J Pediatr 1987;111(2):2015.
tive and auditory impairment in 1020% of Investigations 9. Rennick G, Shann F, de Campo J. Cerebral herniation
survivors.22 The risk for sequelae is greatest CSF biochemistry and microscopy findings are during bacterial meningitis in children. Br Med J
1993;306(6883):9535.
in those who experience acute neurological non-specific (see Table 8.7.1). PCR testing for 10. Shetty AK, Desselle BC, Craver RD, Steele RW. Fatal
complications at the time of their illness.23 HSV and enterovirus is sensitive and specific. cerebral herniation after lumbar puncture in a patient
with a normal computed tomography scan. Pediatrics
Cerebral CT or MRI of the brain and EEG may 1999;103(6):12846.
Viral meningitis be suggestive of HSV encephalitis, particu- 11. Negrini B, Kelleher KJ, Wald ER. Cerebrospinal fluid
Most cases are self-limiting, and treatment is findings in aseptic versus bacterial meningitis. Pediatrics
larly if they show temporal lobe involvement. 2000;105(2):3169.
symptomatic. 12. Mazor SS, McNulty JE, Roosevelt GE. Interpretation of
traumatic lumbar punctures: who can go home?
Pediatrics 2003;111(3):5258.
Management 13. Shah KH, Richard KM, Nicholas S, Edlow JA. Incidence of
Brain abscess It is vital to consider the diagnosis of HSV traumatic lumbar puncture. Acad Emerg Med 2003;10
(2):1514.
Brain abscess classically presents with fever, encephalitis early, because early treatment 14. Aronin SI, Peduzzi P, Quagliarello VJ. Community-
with aciclovir may improve the outcome. acquired bacterial meningitis: risk stratification for
headache and focal neurological deficit. adverse clinical outcome and effect of antibiotic timing.
Although rare, early recognition is vital, as Treatment should be continued for 21 days Ann Intern Med 1998;129(11_Part_1):8629.
unless HSV is excluded clinically or on 15. McMaster P, McIntyre P, Gilmour R, et al. The emergence
early antibiotic treatment and drainage of resistant pneumococcal meningitis implications for
improve outcome. Diagnosis is by cerebral subsequent investigations. empiric therapy. Arch Dis Child 2002;87(3):20710.
16. de Gans J, van de Beek D, the European Dexamethasone
CT or magnetic resonance imaging (MRI). Aciclovir 20 mg kg1 IV 8-hourly in Adulthood Bacterial Meningitis Study Investigators.
Aspiration for diagnosis and neurosurgical (age <3 months) Dexamethasone in adults with bacterial meningitis.
N Engl J Med 2002;347(20):154956.
intervention are usually required. The most 500 mg m2 IV 8-hourly 17. Brouwer M, McIntyre P, de Gans J, et al. Corticosteroids
common causes are oral viridans streptococci, (age 3 months12 years) for acute bacterial meningitis. Cochrane Database Syst
Rev 2010;(9):CD004405.
anaerobes, Gram-negatives and S. aureus. 10 mg kg1 IV 8-hourly 18. Martinez-Lacasa J, Cabellos C, Martos A, et al. Experimental
Empiric treatment is: (age >12 years) study of the efficacy of vancomycin, rifampicin and
dexamethasone in the therapy of pneumococcal
Flucloxacillin 50 mg kg1 (max 2 g) IV meningitis. J Antimicrob Chemother 2002;49(3):50713.
19. Shann F, Germer S. Hyponatraemia associated with
4-hourly plus Complications pneumonia or bacterial meningitis. Arch Dis Child
Cefotaxime 50 mg kg1 (max 2 g) IV Without treatment, mortality of HSV 1985;60(10):9636.
20. Duke T, Mokela D, Frank D, et al. Management of
6-hourly plus encephalitis is up to 80%, and 50% of sur- meningitis in children with oral fluid restriction or
Metronidazole 15 mg kg1 (max 1 g) IV vivors have long-term sequelae.24 Outcome intravenous fluid at maintenance volumes: a randomised
trial. Ann Trop Paediatr 2002;22(2):14557.
stat, then 7.5 mg kg1 (max 500 mg) IV is worse if coma is present initially. 21. Singhi SC, Singhi PD, Srinivas B, et al. Fluid restriction
8-hourly. does not improve the outcome of acute meningitis.
Pediatr Infect Dis J 1995;14(6):495503.
22. Baraff LJ, Lee SI, Schriger DL. Outcomes of bacterial
meningitis in children: a meta-analysis. Pediatr Infect Dis
Encephalitis Conclusion J 1993;12(5):38994.
23. Grimwood K, Anderson P, Anderson V, et al. Twelve year
outcomes following bacterial meningitis: further
Aetiology Meningitis and encephalitis are medical evidence for persisting effects. Arch Dis Child 2000;83
HSV is the most common cause of non- emergencies that must be assessed and (2):1116.
24. Whitley RJ, Lakeman F. Herpes simplex virus infections of
seasonal encephalitis in Australia. Other treated urgently to reduce the likelihood of the central nervous system: therapeutic and diagnostic
causes include enteroviruses, influenza virus, poor outcomes. considerations. Clin Infect Dis 1995;20(2):41420.

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SECTION

9 INFECTIOUS DISEASES
Section editor Gary Browne

9.1 Infectious diseases 230

9.1 Infectious diseases

Mike Starr

Moreover, parents and patients generally

ESSENTIALS prefer other temperature assessments.
Nonetheless, rectal temperature remains
1 Fever is one of the most common reasons for children to present to the Emergency the most widely used measure in infants
Department.
under 3 months of age. Tympanic thermo-
2 Serious bacterial infections need to be identified early and treated aggressively; meters provide the most accurate assess-
these include meningitis, septicaemia, urinary tract infection, pneumonia, and bone ment of core temperature, but the probe
and joint infections. may be too large for an infants auditory
canal. Oral temperature requires patient
3 The majority of febrile children have a viral illness, requiring few if any cooperation, and is generally unsuitable
investigations.
for children under the age of 5 years. Axillary
4 It is important to have a structured approach to the investigation and treatment of temperature measurement is inaccurate
the febrile child without focus. and insensitive.
The definition of fever is
5 Most well-appearing febrile children over 3 months of age without a focus of
infection do not require laboratory testing or treatment, apart from microscopy and 38 C (rectal or tympanic);
culture of urine. 37.5 C (oral); or
37.2 C (axilla).

In this section, general infectious disease

2 years of life, children average four to six Fever: to treat or not to treat?
issues, including the appropriate collection
febrile episodes. Those in child care may The drugs most commonly used for treating
of microbiological specimens, guidelines for
have many more than this. fever are paracetamol, ibuprofen and aspi-
empiric antibiotic therapy, post-exposure pro-
rin. The routine use of these medications
phylaxis and immunisation are addressed.
Defining and measuring in the treatment of fever has been ques-
temperature tioned.5 In particular, there has been con-
There is controversy regarding the most cern that the use of antipyretics may
appropriate thermometer and the best ana- prolong viral shedding, impair antibody
Fever tomical site for temperature measure- response to viral infection, and may increase
Fever is one of the most common presenting ment.3 Parents often use touch to detect morbidity and mortality.57 Moreover, each
complaints in children in both the primary fever in their children. However, touch has of the commonly used antipyretics may have
care and emergency department settings. only 50% specificity.4 It tends to overesti- significant adverse effects such as hepatic
Of all childrens visits to the emergency mate the incidence of fever, and is more dysfunction, metabolic acidosis, Reye syn-
department (ED), 2030% are with acute useful to exclude fever. Rectal temperature drome and gastrointestinal bleeding. Treat-
episodes of fever.1 In children <1 year old has long been considered the gold standard ment should therefore be focused on
presenting to EDs in Australia and New for routine measurement of body tempera- alleviation of discomfort or pain rather than
Zealand, fever without identifiable source ture, but it does not in fact reflect true core on the height of the temperature. Either
is the diagnosis in over 3%.2 In the first temperature within the pulmonary artery. paracetamol or ibuprofen may be used.

230
9.1 INFECTIOUS DISEASES
9

INFECTIOUS DISEASES
It is important to note that the use of anti- Fever with localising signs B breathing difficulty.
pyretics has not been shown to prevent A careful history and examination will C poor perfusion.
febrile convulsions. identify the source of infection in most Fluids in the frequency of feeding over
Paracetamol may be given orally, rectally patients. These children should be managed the 24 hours prior to presentation, <50%
or intravenously at a dose of 1015 mg kg 1 according to the individual condition and its of normal over 24 hours prior to
46-hourly. In an unsupervised, community severity. presentation, suggesting dehydration.
setting, the total daily dose should be limited Fluids out significantly abnormal urine
to 60 mg kg 1, although up to 90 mg kg 1 Fever without focus output of <50% of usual output.
per 24 hrs can be used under medical In a small number of children presenting
Other features on examination that strongly
supervision. Single doses of 30 mg kg 1 with fever, no focus is found. While most will
suggest a seriously ill infant include pallor,
may be used for night-time dosing. Serious have a viral infection, a more serious illness
purpuric rash, high-pitched scream and bulg-
toxicity has been reported in children with such as a urinary tract infection (45%),
ing fontanelle.
chronic daily over-dosage, mostly occurring in occult bacteraemia (<1%) or meningitis
Patients with unexplained fever with a
children who have a febrile illness and (<0.2%) may be present.
higher likelihood for serious infection include
associated anorexia, vomiting and/or dehy- Occult bacteraemia is the presence of
the following patient groups or conditions:
dration.8 A child should be reviewed after bacteria in the bloodstream of a febrile child
48 hours if regular paracetamol has been who has no apparent focus of infection and neonates (<1 month of age);
required for this period. looks well. Diagnosis is by blood culture and incompletely immunised children;
Ibuprofen can be used as an alternative exclusion of focal infection. The incidence immunocompromised (e.g. congenital
to paracetamol at a dose of 510 mg kg 1 of occult bacteraemia in febrile children immunodeficiency, human
(maximum of 500 mg per dose), given 6- has reduced dramatically to <1% since immunodeficiency virus (HIV),
to 8-hourly (maximum daily dose of the introduction of conjugate pneumococcal neutropenic and other oncology patients,
40 mg kg 1 or 2 g). It is recommended that vaccine.10,11 cytotoxic drugs and steroids);
it be used alone, and not in combination Most children who present with fever and asplenic children (congenital, post
with paracetamol, as this practice may lead no identifiable focus appear otherwise well. splenectomy or functional, e.g. sickle cell
to an increase in adverse effects, including History should include details about immu- disease);
gastrointestinal bleeding, renal dysfunction nisation status, infectious contacts, travel, patients who have received prior oral
and anaphylaxis.7 A theoretical risk of diet and contact with animals or insects. A antibiotics; many of these patients have a
aggravating concurrent asthma has also thorough physical examination should be viral infection, but meningitis or other
been described, although these adverse performed, paying particular attention to serious bacterial infection must be
effects are refuted in large prospective stud- general appearance (colour and level of considered;
ies.8 There is also a concern that ibuprofen activity) and vital signs (respiratory rate, children with fever and prolonged
may be associated with an increased risk pulse, peripheral perfusion and blood convulsion;
of necrotising group A streptococcal infec- pressure). children with underlying medical
tions.9 There is no evidence that alternating It is difficult to assess whether a child is conditions (e.g. cystic fibrosis, structural
paracetamol and ibuprofen is any better septic or toxic. A simple and effective cardiac defects, etc.);
at reducing fever or spares the potential approach that is useful in the ED is a combi- children with central venous devices,
hepatotoxicity related to paracetamol nation of ABC, fluids-in and fluids-out.12 An shunts or other foreign material.
administration.9 infant with one or more of these symptoms
When considering management strategies
or signs has a higher risk of serious illness:
for febrile infants, three age groups are gen-
A poor arousal, reduced alertness and erally assigned: <1 month of age, 13
Practical approach to the reduced activity. months and >3 months (Table 9.1.1).
febrile child
The majority of febrile children will have
self-limiting viral infections. The challenge Table 9.1.1 Management of well-appearing febrile child without focus
is to identify those children at risk of serious Age Investigation Management
illness while avoiding unnecessary investiga-
<1 month FBE; blood, urine and CSF Admit
tion and treatment of children with benign cultures; CXR Empiric IV antibiotics: amoxicillin and
viral illnesses. cefotaxime

Fever in children may be classified into 13 months Urine culture Consider admission and observation
three groups:  blood and CSF cultures Discharge with arranged review
 CXR
fever with localising signs; >3 months Consider urine culture Discharge with arranged review
fever without focus;
pyrexia of unknown origin. CXR, chest X-ray; FBE, full blood examination; CSF, cerebrospinal fluid.

231
9.1 INFECTIOUS DISEASES

Infants less than 1 month of age, and those examination and routine investigations cavities and are inactive in an anaerobic
with any of the risk factors above require have not identified a cause. Occasionally, environment).
several investigations including full blood fever appears to occur in a repetitive or peri- For presumed bacterial infection (includ-
examination, culture of blood, urine and odic pattern. In either case, a chronic or non- ing meningitis) in the first 3 months of
cerebrospinal fluid (CSF), and a chest X-ray infectious condition should be considered, life, empiric treatment must cover Group B
if indicated. Empiric antimicrobial therapy such as juvenile idiopathic arthritis and streptococci, Escherichia coli and Listeria
should be based on the patients clinical ill- other connective tissue diseases, inflamma- monocytogenes infections. Recommended
ness, risk factors, and the local epidemiology tory bowel disease or malignancy. Infectious antibiotics are: amoxicillin plus cefotaxime.
of potential pathogens and their antibiotic causes include systemic viral syndromes (such
susceptibility. as infectious mononucleosis), upper or lower Amoxicillin 50 mg kg 1 per dose
Clinical scores, such as the Rochester and respiratory infections (e.g. sinusitis), urinary intravenous (IV) 12-hourly
Boston criteria, have been devised to iden- tract infection, central nervous system infec- (week 1 of life), 8-hourly
tify children at low risk of serious bacterial tion, bone infection, tuberculosis, abscess (week 24 of life), 46-hourly
infection.13 However, their utility has been (e.g. parameningeal, intra-abdominal), endo- thereafter.
questioned in the era of widespread Hib carditis and enteric infections (e.g. typhoid Cefotaxime: 50 mg kg 1 per dose IV
and conjugate pneumococcal vaccination. fever). 12-hourly (week 1 of life),
Febrile infants between 1 and 3 months More extensive investigations are often 8-hourly (week 24 of life),
of age who appear well and do not have required, including specific investigations 6-hourly thereafter.
risk factors may not require blood tests or for mycobacterial, fungal or viral infec-
For presumed bacterial infection (including
a lumbar puncture, although urine micros- tions. Imaging may be required, looking
meningitis) after 3 months of age, potential
copy and culture is advisable. Those over for occult abdominal or central nervous
pathogens include Neisseria meningitidis,
3 months of age do not routinely require system collection, for osteomyelitis or
Streptococcus pneumoniae, Group A strepto-
laboratory testing or treatment, although endocarditis.
cocci and Staphylococcus aureus. Recom-
urine microscopy and culture may still be Kawasaki disease is an important consid-
mended empiric therapy is: flucloxacillin
appropriate. eration in an infant or child presenting with
plus cefotaxime.
There is no evidence that oral or parenteral prolonged fever, and diagnosis is often
1
antibiotics prevent the rare occurrence of delayed. There is a degree of urgency in Flucloxacillin 50 mg kg per dose IV
focal infections from occult bacteraemia; diagnosis, because treatment within 10 days 6-hourly.
1
instead, they result in delayed diagnosis, drug of onset of fever with intravenous immuno- Cefotaxime 50 mg kg per dose IV
side effects, additional costs and the develop- globulin and aspirin reduces the incidence of 6-hourly.
ment of resistant organisms. What is required coronary artery lesions from around 20% to
If meningitis has been excluded, recom-
is a careful clinical assessment, parental edu- around 5%.16
mended antibiotics are flucloxacillin plus
cation and review within 24 hours.
gentamicin.
As urinary tract infection is the most com-
mon serious bacterial infection among Gentamicin 7.5 mg kg 1 24-hourly (<10
Empiric antibiotic therapy
febrile infants and children, urine micros- years), 6 mg kg 1 per dose
copy and culture should be included in the With the possible exception of bacterial 24-hourly (>10 years)
investigation of most such children. In meningitis, where Gram stain results may
Antibiotic choice should also be modi-
infants, a urine sample should ideally be guide therapy, the most appropriate antibi-
fied once relevant culture results become
obtained via suprapubic aspiration or cathe- otic therapy in children must be based on
available.
ter. A negative urinalysis does not exclude a epidemiological grounds. The most impor-
urinary tract infection, which may occur in tant factors determining the likely patho- Antimicrobial resistance
the absence of pyuria.14,15 gens, which should be targeted by empiric Antimicrobial resistance is an increasing
Other rarer causes of fever should also be therapy, are: worldwide problem. Resistance of bacteria
considered: to antibiotics can conveniently be divided
age; into two categories:
Kawasaki disease; presumed focus of infection;
connective tissue disease (e.g. juvenile presence of underlying disease or Intrinsic resistance due to inherent
arthritis); anatomical abnormality; properties of the organism or antibiotic,
inflammatory bowel disease; whether the infection is hospital or and
malignancy (e.g. leukaemia). community acquired. Acquired resistance gained by the
organism either before or during
In addition, the site of infection may have
therapy.
Pyrexia of unknown origin (PUO) implications for the expected penetration
PUO is defined as prolonged fever (usually of the antibiotic chosen (e.g. aminogly- Important examples of intrinsic resistance
greater than 10 days) where history, cosides do not penetrate into abscess (which affects 100% of organisms) include

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9.1 INFECTIOUS DISEASES
9

INFECTIOUS DISEASES
enterococci to cephalosporins, enteric Scarlet fever buccal enanthem called Kopliks spots (white
Gram negatives to penicillin and flucloxacil- This infection is common amongst children spots on a bright red buccal mucosa),
lin and anaerobes to aminoglycosides and 312 years of age. It is caused by Group followed 34 days later by a rash. The
quinolones. A few important examples of A-b-haemolytic streptococci (GAS). The dis- rash is erythematous and blotchy, starting
increasing acquired resistance include: ease is transmitted by direct contact or respi- at the hairline and moving down the
ratory droplets. It has a short incubation body, before becoming confluent. It lasts
penicillin (and cephalosporin)-resistant period of 25 days. The illness is charac- up to a week, and may desquamate in the
Strep. pneumoniae (PRP) of note,
terised by an abrupt onset of fever, vomiting second week.
resistance rates have fallen since
and a sore throat, with abdominal pain. The The average period from exposure
introduction of the conjugate vaccine;
typical rash develops within 1248 hours to appearance of the rash is 14 days. The
methicillin (multidrug)-resistant Staph.
after onset, and is a generalised confluent infectious period is from 1 to 2 days before
aureus (MRSA);
erythematous papular rash giving the skin the onset of symptoms to 4 days after the
Staph. aureus with intermediate
a sandpaper-like texture. The forehead and appearance of the rash.
sensitivity to vancomycin and teicoplanin
cheeks are red, smooth and flushed, with Immunisation to measles using a live
(GISA);
sparing of the area around the mouth (circu- attenuated measles vaccine has been well
community acquired non-multiresistant
moral pallor). Petechiae may coalesce in lin- established since the early 1960s, with an
MRSA (CA-MRSA);
ear form, particularly in skin folds such as uptake rate increasing steadily to over
vancomycin-resistant Enterococcus
axillae and antecubital fossae, forming 95% with the introduction of the measles,
(VRE);
pathognomonic Pastia lines. The tongue in mumps and rubella (MMR) vaccine in
bacteria that produce extended-spectrum
scarlet fever is initially coated by a white 1988. Vaccination should be actively
b-lactamases (ESBL), e.g. some E. coli and
fur, which after a few days is reddened by encouraged, given the potential complica-
Klebsiella spp., which are associated with
the projection of oedematous papillae tions due to measles infection of otitis
cephalosporin (and often gentamicin)
through the coat. This white strawberry media (2.5%), bronchopneumonia (4%),
resistance.
tongue loses its coating after 4 days, reveal- acute encephalitis (0.1% of cases, with
ing a beefy red strawberry tongue. Resolu- a mortality rate of 1015%) and late
tion of the rash and other clinical subacute sclerosing panencephalitis.
manifestations usually occurs by the end MMR may afford some protection if given
Common infectious
of the first week, heralding a period of within 72 hours of exposure to measles to
exanthems those with doubtful immunity, as immunity
characteristic desquamation of skin. Desqua-
Most frequently, the cause of fever in chil- mation progresses from face to trunk and from the attenuated vaccine virus is more
dren is a viral illness. This usually occurs in finally to hands and feet after 34 weeks. rapid than that from natural measles.
a seasonal pattern in Australia particularly Complications of scarlet fever may be If MMR is contraindicated or if more than
the months of April through to September early local upper respiratory tract disease, 72 hours have elapsed since exposure,
when there is an increase of acute infections including cervical adenitis and otitis media, normal human immunoglobulin may be
in the community. Most of these are due to and later immune-mediated disease includ- given within 7 days of exposure, to
respiratory and gastrointestinal pathogens, ing acute glomerulonephritis and rheu- prevent or modify disease.
such as respiratory syncytial virus and rota- matic fever. A 10-day course of penicillin
virus, respectively. However, there is an is effective in eradication of the bacteria;
important group of viral infections that the clindamycin may be added to inhibit toxin Rubella (German measles)
emergency physician needs to be familiar synthesis if there are associated features Rubella is a mild acute infectious disease
with that commonly present with fever of shock. spread by droplets or direct contact. The
and rash. Investigations are often unhelpful acutely. incubation period is 1421 days. Cases are
A rash or other cutaneous manifestation Isolation of GAS from a throat swab may infectious from 5 days before until 7 days
accompanies a large number of presenta- reflect asymptomatic carriage and not nec- after appearance of the rash.
tions for childhood infectious disease. An essarily invasive disease. Elevation of serial Rubella is characterised by a 23-day pro-
exanthem is an acute infectious disease antistreptolysin O or anti-DNAse-B titres drome of fever, malaise, upper respiratory
accompanied by a rash. The most common may aid diagnosis of recent streptococcal tract symptoms, and lymphadenopathy, par-
childhood exanthems are scarlet fever, infection. ticularly postauricular and occipital. The
measles, rubella, erythema infectiosum rash consists of small fine pink maculopa-
and roseola infantum. Rash and fever may pules, starting on the face and spreading
be associated with many other viruses, Measles to the chest and upper abdomen and thighs,
bacteria, and even parasitic infections. In Measles is a highly infectious, acute viral ill- all within 24 hours. However, it is not diag-
addition, a rash and fever may also be asso- ness, spread by respiratory droplets. It is nostic of rubella as a similar rash can be
ciated with a wide variety of non-infectious characterised by fever, coryza, exudative caused by other viruses, including parvovirus
processes. conjunctivitis, cough and a pathognomonic and enteroviruses.

233
9.1 INFECTIOUS DISEASES

The disease itself has rare complications rash, as occurs in Coxsackie and echovirus. vesicular) lesions are located on the face, but-
such as encephalitis and thrombocytopenia, In classical hand-foot-and-mouth disease, tocks, and extensor surface of the extremities
but the risk to the unborn fetus of exposure due to Coxsackie A16, vesicles occur in a with truncal sparing. It is present for 24
in the first trimester (especially 68 weeks typical distribution over the hands, feet weeks, though may take up to 4 months to
gestation) is significant, resulting in severe and buttocks, with lesions in the anterior resolve. It has been associated with a number
developmental and structural damage in mouth often becoming painful ulcers. The of viral illnesses, including enterovirus, rotavi-
90% of affected cases (congenital rubella lesions may be petechial, resembling rus, EpsteinBarr virus (EBV), some bacterial
syndrome). The vaccine is contraindicated those of meningococcal infection. Infections infections, and immunisation. More than
in pregnant women and post-exposure pro- occur particularly through the summer and 90% of patients are <4 years old.
phylaxis with immunoglobulin does not pre- autumn and are usually self-limiting.
vent infection in non-immune contacts, The virus is shed in faeces and saliva for
although it may modify the risk of abnorm- several weeks.
alities to in the fetus. What specimens and when
Varicella (chickenpox)
should they be ordered?
Erythema infectiosum
Slapped cheek disease or erythema infec- Chickenpox is caused by varicella zoster Targeted and judicious use of laboratory
tiosum is caused by parvovirus B19. It is virus (VZV), which has an incubation period investigations facilitates more rapid and
spread by droplets or direct contact, with of 1021 days. Patients are most contagious accurate diagnosis of causes of infection in
an incubation period of 421 days. It is for 12 days before the rash and while new children presenting to the ED.
highly infectious until the rash appears, lesions erupt, which can be up to 5 days. It is
although 5060% of adults are already more severe in adults and can be potentially
immune. Fever occurs in up to 30% with a fatal in immunocompromised patients, with Collection of microbiological
non-specific prodrome. The rash initially con- a mortality rate of 710% in the latter. The specimens
sists of red, flushed cheeks with circumoral clinical features include a 35-day prodrome It is imperative that microbiological speci-
pallor (slapped cheeks), followed after of fever, irritability and lymphadenopathy. mens be collected appropriately, taking care
7 days by a maculopapular rash on the limbs The itchy vesicular rash often begins on not to contaminate samples during collection.
and trunk that clears centrally, leaving a lacy the scalp and spreads rapidly to involve Samples should also be transported to the
(reticular) pattern as it fades. The virus may the whole body. Cerebellitis, aseptic menin- laboratory promptly, stored in appropriate
precipitate a severe aplastic anaemic crisis gitis, transverse myelitis, thrombocytopenia laboratory environments and processed with-
in individuals with underlying haemolytic and pneumonia may complicate the acute out undue delay.
disease, such as hereditary spherocytosis, infection.
sickle cell anaemia and thalassaemia. Most adults, including those with no his-
It can cause fetal hydrops. Pregnant tory of chickenpox, are immune to varicella. Blood cultures
contacts should be offered serology and Staff working in paediatric EDs who have no Collection
specialist review. history of chickenpox should have VZV serol- Proper hand washing technique and use of
ogy to determine their immune status. gloves will avoid contamination of the sam-
Roseola infantum (exanthem Those that spent their childhood in tropical ple. Peripheral blood cultures are usually col-
subitum, sixth disease) countries are more likely to be non-immune lected from veins, either direct puncture or
Roseola infantum is caused by human her- as adults. Any non-immune staff should be immediately aspirated from a cannula after
pesvirus 6 (HHV-6) or less commonly vaccinated. insertion. Arterial samples may also be used,
HHV-7. 95% of children are infected with VZV vaccine can provide post-exposure but heel prick samples are inappropriate. It
HHV-6 by the age of 2, although only prevention of chickenpox if given within is imperative to disinfect the patient site
30% present with roseola. Transmission is 5 days of exposure. Zoster immune globulin for blood collection, usually with topical
by direct contact or (asymptomatically shed) can prevent or attenuate disease if given aqueous chlorhexidine or large alcohol
droplets. Initial presentation is with fever within 4 days of exposure. It is recommended swabs and to allow the disinfecting agent
and few if any other symptoms (possibly a for immunocompromised children, and for to dry, which is an important part of the dis-
febrile convulsion). Fever resolves after newborn infants exposed whose mothers infection process. Swab the rubber bung on
35 days, and a pink macular rash erupts had chickenpox in the 5 days before or the bottle with an alcohol swab and allow
over the trunk and arms, which then fades 2 days after delivery. drying time before introducing blood into
within 12 days. the bottle. Blood for cultures should be col-
Papulovesicular acrolocated lected first, before placing blood into other,
Enterovirus infection syndrome (GianottiCrosti non-sterile bottles for additional investiga-
Enterovirus infections may present in syndrome) tions. Avoiding needle changes to inoculate
a variety of ways, including malaise and Papulovesicular acrolocated syndrome (PALS) the culture bottle minimises the risk of an
fever, often accompanied by a non-specific is an exanthem in which papular (and often accidental needlestick injury.

234
9.1 INFECTIOUS DISEASES
9

INFECTIOUS DISEASES
Volume of blood and collection Bag urines UTIs, and pyuria is not always present in
media Urine collected is usually contaminated, and UTIs, which makes a negative urinalysis less
It is important to remember that a minimum testing is neither sensitive nor specific. likely to exclude a UTI.
amount of blood must be collected, but
also that more than a maximum amount
Clean catch and midstream Microscopy A urinary white cell count
of blood will decrease the sensitivity of the
collection >10  106 L 1 together with a positive
culture and thus the likelihood of identifying
Are both non-invasive and contamination leucocyte esterase is a very sensitive screen-
a microorganism. The optimum blood-to-
is less likely, but care must be taken to ing test for UTIs, but still has a false nega-
culture broth dilution is 1:10 in most blood
culture systems, and up to 5 mL of blood
reduce contamination. The best clean tive rate of 15%. The presence of >10
can be inoculated into older systems, but
catch possible is obtained from boys with leucocytes mm 3 on direct microscopy has
the foreskin retracted, and girls with the been shown to have a low positive predictive
from 0.4 mL to 4 mL in newer collection
labia spread after cleansing with soap value (56%) for UTI but, combined with the
media. Samples for anaerobic bacterial
and water and gentle drying. The patient presence of bacteria, this constitutes the
cultures are not routinely collected, with
or parent should avoid touching skin and most accurate screening test in detecting
few exceptions:
the specimen should be collected in positive urinary cultures.15
febrile neutropenic, midstream.
immunocompromised patients;
patients with suspected intra-abdominal Culture UTI is ultimately diagnosed on
or pelvic sepsis; Suprapubic aspiration demonstration of significant bacteriuria,
neonates with a high risk of anaerobic This is the most reliable means of collection which implies counts of >108 L 1 of a single
sepsis, i.e. those with prolonged rupture of of a sterile specimen, but the infant usually pathogen in a fresh, uncentrifuged clean
membranes, offensive liquor, or maternal needs to have a reasonably full bladder. catch or midstream urine sample. Any growth
fever in labour. Bladder scanning/ultrasound improves the on a sample obtained by urinary catheter or
yield. The technique is safe. suprapubic aspirate is also diagnostic.
Blood culture bottles should be correctly
labelled, and optimally stored in an appro-
priate incubator (never in a fridge). Catheterisation Stool specimens
Quick in-out urinary catheterisation is a rea- Viral gastroenteritis is the most common
sonably reliable collection method and does cause of paediatric hospital attendance dur-
not require the bladder to be filled. However, ing the spring and early summer months.
Cerebrospinal fluid contamination of the specimen is common. The organism involved is usually rotavirus,
See Chapter 8.7, p. 227228, and also Sec- It is very unlikely that infection can be intro- which is usually self-limiting and is readily
tion 23 (Common procedures). duced with an in-out procedure that is done detected by enzyme-linked immunoassay
with sterile technique. Only mild discomfort or latex agglutination. Laboratory analysis
is usually experienced. Catheterisation is usually not warranted during seasonal
Urine examination should not be attempted in girls with signifi- endemic periods as most causes of diarrhoea
A sterile urine sample enables a more accu- cant labial adhesion, nor in boys with phimo- are usually self-limiting and identification
rate diagnosis of urinary tract infection (UTI) sis that prevents vision of the urethral will not alter management in most cases.
and as such the method of collection signifi- meatus. Indications for stool analysis are:
cantly influences the accuracy of the micro-
persistent diarrhoea >7 days;
scopic results obtained. If UTIs are over- Interpretation
blood in stool;
diagnosed, other important diagnoses may Reagent-impregnated dipstick A dip-
recent overseas travel, particularly to
be missed and the child may be subjected stick urinalysis can be used to detect the
typhoid endemic areas;
to unnecessary further investigations. A presence of nitrite-forming bacteria or esti-
immunocompromised children.
negative urinalysis does not exclude a UTI, mate the presence of pyuria using a leuco-
and 16% of UTIs may be missed. The pres- cyte esterase test strip, thereby allowing a
ence of white or red blood cells or protein quick presumptive diagnosis of a UTI and Collection
does not either confirm or refute the diagno- early treatment in an unwell child. Blood, Diarrhoeal stool (510 g) should be col-
sis. All urine specimens should be sent for protein and ketones can also be detected. lected in a sterile container with a secure fit-
microscopy and culture if a UTI suspected, The poor sensitivity for nitrites (40%) and ting lid. Testing the stool pH using a reagent
or if obtained via catheter/suprapubic aspi- pyuria (70%), however, means that a high strip may be a useful indicator for rotavirus
rate in non-toilet-trained children. The labo- false negative rate (of 50% and 20% respec- gastroenteritis as it causes an acidic stool
ratory should always be informed of the tively) for detection of true infection by this (pH < 5). Similarly, testing stool for reducing
collection method as this influences the method exists.14 Non-nitrite-forming bacte- substances may be positive in cases of sec-
interpretation of results. ria such as Enterococcus spp. may also cause ondary lactose intolerance.

235
9.1 INFECTIOUS DISEASES

Throat swab containing the spread of infections in any

Throat swabs may be performed both for healthcare setting. Nowhere more so than Immunisation
bacterial and viral culture, although for the in paediatric EDs, where communicable viral Immunisation of staff
latter a nasopharyngeal aspirate or nasal respiratory and enteric infections are the It is in the best interests of all healthcare
swab is usually more helpful. most common causes of presentations. workers, and their patients, that staff in pae-
Cross-infection can be eliminated by strict diatric centres have a serologically proven
Nasopharyngeal aspirate (NPA) hand-washing measures by both staff and immunisation record against communicable
NPA is the preferred method for recovering parents, as well as avoiding toy sharing by illnesses such as measles, mumps and
viruses from the upper respiratory tract. This patients in ward situations. Prophylaxis with rubella, varicella and HBV, and receive the
may be helpful in children with the clinical normal or specific human immunoglobulin is relevant immunisation as appropriate.
syndrome of bronchiolitis, both for a specific sometimes indicated following significant Pertussis-containing booster and annual
diagnosis and to facilitate infection control exposure to communicable diseases such influenza vaccine should also be given.
procedures, although testing rarely affects as hepatitis A and B, measles and varicella,
patient management per se. Nasal lavage and antibiotic prophylaxis may be indicated
is an alternative to NPA, but swabs of the for significant exposure to meningococcal Opportunistic immunisation
nose and throat are generally unsuitable and Hib disease. The ED visit also presents an invaluable
for rapid immunofluorescence. Newer molec- opportunity to monitor the immunisation
ular techniques may be performed on nasal status of children and offer catch-up immu-
swabs. nisation to those who have missed vacci-
Needlestick injury (NSI)
nations, or commence the appropriate
Interpretation The child presenting with a vaccination schedule. The current immunisa-
Most respiratory viruses can be detected
(community) needlestick injury tion schedule is available in the Australian
by direct immunofluorescence of exfo-
The risk of seroconversion to HIV, hepatitis B Immunisation Handbook.20
virus (HBV) or hepatitis C virus from a com-
liated respiratory epithelium, as viral anti-
munity acquired NSI is very low. Exposed
gens are expressed on the cell surface. If
individuals should be reassured. Immunity
available, this allows rapid diagnosis of Acknowledgement
to hepatitis B should be confirmed, and if
infection. Viruses can also be cultured.
incomplete, hepatitis B vaccine should be The contribution of Neil Smith as author in
The positive and negative predictive
given. Unless the injury is considered to be the first edition is hereby acknowledged.
values of both NPA and nasal lavage in
particularly high risk, no further manage-
diagnosing respiratory syncytial virus
ment is required at the time. Follow up
infection are over 90%. Bordetella pertus-
should be arranged for counselling and
sis may be identified by PCR if a definitive
serology if required. References
diagnosis is required before culture results
1. Browne G, Currow K, Rainbow J. Practical approach to the
of NPA are available. febrile child in the emergency department. Emerg Med
Hospital staff exposure to HBV 2001;13:42635.
2. Acworth J, Babl F, Borland M, et al. Patterns of
Nasal swab In general, NSIs occur more often in the hos- presentation to the Australian and New Zealand
A nasal swab is the classic method for collec- pital environment, where a lack of universal Paediatric Emergency Research Network. Emerg Med
Austral 2009;21:5966.
tion of diagnostic specimens for diagnosis of precautions in handling or discarding con- 3. El-Radhi A, Barry W. Thermometry in paediatric practice.
pertussis. However, a nasopharyngeal aspi- taminated needles and sharps has occurred. Arch Dis Child 2006;91:3516.
4. Ten C, Ng C, Nik-Sherina H, et al. The accuracy of Mothers
rate is also satisfactory. It is advisable that all staff working in the touch to detect fever in children: A systematic review.
hospital environment should be adequately J Trop Pediatr 2007;54:703.
5. Meremikwu M, Oyo-Ita A. Paracetamol for treating fever
Collection immunised against hepatitis B and that in children. Cochrane Database Syst Rev 2002;(2):
A small-tipped nasopharyngeal swab is serological confirmation of immunity is per- CD003676 (2002).
6. Kramer M, Naimark L, Roberts-Brauer R, et al. Risks and
passed into the posterior nasopharynx. Do formed. If significant exposure (percutane- benefits of paracetamol antipyresis in young children
not use a swab with a cotton tip as this ous, ocular, or mucous membrane) to blood with fever of presumed viral origin. Lancet 1991;337
(8741):5914.
may be inhibitory to the organism. The swab or potentially blood-contaminated secretions 7. Russell F, Shann F, Curtis N, Mulholland K. Evidence on the
is left in place for at least 30 seconds, if has occurred in persons who are unimmu- use of paracetamol in febrile children. Bull World Health
Organ 2003;81(5):36772.
possible. nised, testing of the source should be done 8. Riordan M, Rylance G, Berry K. Poisoning in children 2:
as well as of the recipient. A single dose of painkillers. Arch Dis Child 2002;87(5):3979.
9. Lesko S, OBrien K, Schwartz B, et al. Invasive group A
hepatitis B immunoglobulin (HBIG 400 IU streptococcal infection and nonsteroidal
for adults and 100 IU for children) is then antiinflammatory drug use among children with primary
Infection control in the ED varicella. Pediatrics 2001;107(5): 110815.
offered to the recipient if HBsAb-negative, 10. Carstairs K, Tanen D, Johnson A, et al. Pneumococcal
Adhering to strict universal precautions (i.e. or if the source is HBsAg positive or cannot bacteremia in febrile infants presenting to the emergency
department before and after the introduction of the
treating all blood and body fluids as poten- be identified. This should be given within heptavalent pneumococcal vaccine. Ann Emerg Med
tially infectious) is an integral part of 72 hours after exposure. 2007;49:7727.

236
9.1 INFECTIOUS DISEASES
9

INFECTIOUS DISEASES
11. Antonyrajah B, Mukundan D. Fever without apparent in young febrile children? Pediatr Infect Dis J 18. Mazor S, McNulty J, Roosevelt G. Interpretation of
source on clinical examination. Curr Opin Pediatr 1996;15:3049. traumatic lumbar punctures: who can go home?
2008;20:96102. 15. Craig J, Irwig L, Knight J, et al. Symptomatic urinary tract Pediatrics 2003;111:5258.
12. Hewson P, Humphries S, Roberton D, et al. Markers of infection in preschool Australian children. J Paediatr 19. Shah K, Richard K, Nicholas S, Edlow J. Incidence of
serious illness in infants under 6 months old presenting Child Health 1998;34:1549. traumatic lumbar puncture. Acad Emerg Med
to a childrens hospital. Arch Dis Child 1990;65:7506. 16. Burns J, Glode M. Kawasaki syndrome. Lancet 2003;10:1514.
13. Baraff LJ. Management of infants and young children 2004;364:53344. 20. National Immunisation Program Schedule. Available
with fever without source. Pediatr Ann 2008;37:6739. 17. Negrini B, Kelleher K, Wald E. Cerebrospinal fluid findings from: http://immunise.health.gov.au/internet/
14. Hoberman A, Wald E, Reynolds E, et al. Is urine in aseptic versus bacterial meningitis. Pediatrics immunise/publishing.nsf/Content/nips2 [accessed
culture necessary to rule out urinary tract infection 2000;105:3169. 19.10.10].

237
SECTION

10 ENDOCRINE AND
METABOLIC
Section editor Jeremy Raftos

10.1 Metabolic emergencies 238 10.4 Adrenal crisis 246

10.2 Diabetic emergencies in children 242 10.5 Disorders of fluids, electrolytes
and acidbase 248
10.3 Thyroid emergencies 244

10.1 Metabolic emergencies

Drago Bratkovic Peter Francis

There is accumulation of A, which is

ESSENTIALS toxic example: phenylketonuria.
1 Inborn errors of metabolism are individually rare but as a group are not uncommon. There is a deficiency of B, which results
in cellular dysfunction example:
2 The possibility of an inborn error of metabolism needs to be considered in any child SmithLemliOpitz syndrome, a disorder
with unexplained hypoglycaemia, acidosis, altered conscious state, neurological of cholesterol biosynthesis.
presentation or vomiting.  Excess A is converted to C via an
3 Prompt recognition is important to allow appropriate therapy and to avoid further alternate pathway which is toxic
example: tyrosinaemia type 1.
decompensation.
Excess A results in accumulation of
4 Precise identification of the defect in the ED is not generally important as long as metabolite E earlier in the pathway, which is
appropriate initial therapy is commenced and this usually consists of dextrose. toxic example: maple syrup urine disease.
Excess A is excreted by reacting/
conjugating with D, which results in
by screening, thus children and, indeed, deficiency of D example: carnitine
Introduction adults can still present acutely with an undi- deficiency in fatty acid oxidation defects.
Inborn errors of metabolism are a diverse agnosed metabolic condition. The pathology seen in an IEM can result from
group of disorders that result from a defect any of the processes outlined in Figure 10.1.1,
or absence of an enzyme or transport sys- but in fact most IEM are the result of more
Physiology and
tem. The presenting symptoms and signs than one mechanism. Genetic defects can
pathogenesis
are equally diverse; however, there are a also occur in the support processes of meta-
number of common features that can alert The process by which living matter is built bolic pathways such as transport proteins,
the clinician to their presence. This chapter up (anabolism) or broken down (catabolism) enzyme chaperones and enzyme complex
will concentrate on those metabolic condi- is termed metabolism. Strictly speaking, an assembly proteins, which result in the block
tions that present acutely to the emergency inborn error of metabolism (IEM) is an inher- of a metabolic process and thus pathology.
department (ED), but will also touch on ited defect in a metabolic pathway or
those with more chronic presentations enzyme. Generally, there is a defect in an
which could easily go unrecognised. enzyme that catalyses the conversion of
Clinical features
The majority of patients presenting with one organic compound to another. However,
acute metabolic conditions to the emer- not all defects in metabolic pathways give The clinical features and presentations of IEM
gency department will have already been rise to pathology. Figure 10.1.1 shows that are many and varied due to the diverse nature
diagnosed, particularly given the advent of compound A is converted to B. If the enzyme of the enzymes and processes affected; how-
extended newborn screening (see end of that catalyses the reaction is not present or ever, Table 10.1.1 shows that there are a num-
chapter). However, not all conditions can is functioning poorly, this can affect the body ber of common features that should alert the
be screened for and some can be missed in any one of the following ways. clinician to the possibility of an IEM.

238
10.1 METABOLIC EMERGENCIES
10

ENDOCRINE AND METABOLIC

with rapid turn-around times and are
A B thus likely to be available to the physician
in the emergency department.
(a) A B

(b) A B Blood acidbase, ammonia, urea,

creatinine, electrolytes, liver function
(c) C A B tests, laboratory glucose, lactate and
calculated anion gap ([Na K]
(d) D +A B
[C1 HCO3]).
(e) Urine ketone dipstick (will only detect
E A B
acetoacetate).

Fig. 10.1.1 Pathogenic mechanisms in IEM; refer to text for a full explanation. In all cases of hypoglycaemia the following
additional investigations are recommended
Table 10.1.1 Groups of IEM that present to the ED and their common presenting features
and need to be collected before the hypoglycae-
mia is treated. Most tertiary paediatric emer-
IEM group Common presenting features gency departments will have a hypoglycaemia
Glycogen storage disorders,e.g. GSD III, IV, Hypoglycaemia, rhabdomyolysis, investigation kit for use in this situation.
VI, VII and IX cardiomyopathy, hepatomegaly

Aminoacidopathies and organic acidaemia, Vomiting, acidosis, encephalopathy Blood insulin, cortisol, growth hormone,
e.g. maple syrup urine disease (MSUD)
adrenocorticotropic hormone
Urea cycle defects, e.g. ornithine Vomiting, encephalopathy, hyperammonaemia, (ACTH), free fatty acids (FFA),
transcarbamylase deficiency (OTC) respiratory alkalosis
ketones (beta-hydroxy-butyrate
Disorders of gluconeogenesis, e.g. glycogen Lactic acidosis and hypoglycaemia and acetoacetate), acylcarnitine
storage disease type I
profile (collect a newborn screening
Fatty acid oxidation defects,e.g. medium chain Hypoketotic hypoglycaemia, encephalopathy and card; NBS or Guthrie card).
acyl-CoA dehydrogenase deficiency (MCAD) rhabdomyolysis
Urine organic and amino acids.
Mitochondrial respiratory chain defects, Lactic acidosis, seizures, stroke-like events
e.g. Leigh disease, MELAS
Once the results of the initial investiga-
Disorders of ketone production and utilisation, Severe ketoacidosis, hypoglycaemia
e.g. ketolytic defect
tions are available, a possible diagnosis
and further investigations may be suggested,
depending on the profile (Table 10.1.3 and
Table 10.1.2 can be used as a guide to result in an incorrect or no diagnosis, with Figure 10.1.2). However, if this not helpful,
identifying an IEM in the ED: risky provocation testing, such as fasting all of the following second line investiga-
An IEM should be considered if there is or loading studies being the only option. tions should be performed:
both a clinical and a biochemical feature The following investigations are a good
from each of the lists; however, considering starting point when considering an IEM. They
Blood plasma amino acids, plasma
an IEM should never take the place of the are all standard laboratory investigations
ketones, acylcarnitine profile,
work up and treatment for more common
creatine kinase, urate.
causes of the above presentations, the most Table 10.1.2 Guide to identifying an IEM Urine organic and amino acids.
important being sepsis. in the ED
IEM are broken down into a number of
Clinical features Biochemical features Many of these investigations can take
groups of conditions, all of which affect a
Overwhelming illness Acute acidosis (with days for a result, and it is suggested that
common metabolic pathway. The groups of in the neonatal period raised anion gap)
all suspected metabolic cases be discussed
IEM that are likely to present to the ED Recurrent vomiting Hypoglycaemia
Coma or Lactic acidosis with a metabolic physician, so a treatment
are outlined in Table 10.1.1, including the encephalopathy (normal perfusion)
plan can be developed to keep the child sta-
common presenting features of each. Apnoea and/or Ketoacidosis
seizures Acute hepatic ble while awaiting further results. The single
Failure to thrive or dysfunction
malnutrition Coagulopathy
most useful investigation in the suspected
Presence of an IEM work up is the urine organic and amino
Investigation unusual odour
Not responding to
acids, which in some centres is referred to as
The metabolic markers of many IEM are usual treatment the metabolic urine screen. This can be per-
Unusual odour
present only at the time of presentation FH of neonatal or
formed even on a small non-sterile urine
and may disappear with treatment. Thus, infant death, SIDS or sample; however, for technical reasons most
acute life threatening
the timing of investigations is extremely event (ALTE)
laboratories will not perform the test if there
important; if done incorrectly they could is faecal contamination.

239
10.1 METABOLIC EMERGENCIES

Table 10.1.3 Biochemical profiles of the different IEM

Metabolic Lactate Ketones NH3 AST/AST BSL Defect/disorder Further

acidosis investigations

to " to """ N to "" (") N to "" N or # Mitochondrial UO&AA,

respiratory chain pre- and post-
disorder prandial lactate

to N or " "" " to "" N or " N or # Organic aciduria Plasma AA, UO&AA,
ACP

to N """ N N # or N Ketolytic defect Plasma ketones,

UO&AA, ACP

to " to "" N N N to "" ### GSD I Urate, triglycerides,

cholesterol

N " to "" N N to "" ### GSD III CK, triglycerides,

cholesterol

to N to "" N or " (") ("") ### Fatty acid oxidation UO&AA, ACP, CK

() OR N N """ N or " N Urea cycle defect Plasma AA

alkalosis

to N "" N N N MSUD Plasma AA

() N or " N or " N """ N or # Galactosaemia, UO&AA, urine

tyrosinaemia type 1 reducing substances

absent, present, ( ) sometimes, N normal, " elevated, # decreased

BSL blood sugar level, GSD glycogen storage disease, MSUD maple syrup urine disease,
Plasma AA plasma amino acids, UO&AA urine organic and amino acids, ACP acylcarnatine profile, CK creatine kinase.

Initial findings of: Correction of altered

1. Convulsion/coma homeostasis
2. Vomiting Hypoglycaemia and metabolic acidosis
3. Lethargy
are the two most common biochemical
abnormalities seen in IEM.
Metabolic condition Unwell child Differential Hypoglycaemia should always be correc-
suspected sepsis ted immediately with an initial infusion
cardiac, etc. of 5 mL kg1 of 10% dextrose. This should
Check blood be followed with an infusion of 10% dex-
sugar level Low Hypoglycaemia trose at maintenance rates, equating to
work up 56 mg kg1 min1 of glucose, and will be
(see below)
Normal
enough to meet basal endogenous glu-
cose needs, however, to suppress catabolism
Plasma ammonia
higher rates of up to 10 mg kg1 min1 of
glucose may be required. The requirement
High Normal of infusion rates of dextrose higher than
this suggests hyperinsulinism.
Acidbase Acidbase Persistent metabolic or lactic acidosis
can be problematic; however, the manage-
Normal Acidosis Normal ment strategies outlined later in this section
will help reduce acidosis as the patient
recovers; occasionally sodium bicarbonate
Urea cycle defects Organic acidaemias Aminoacidopathies
may be required; however, the effect of
Fig. 10.1.2 Clinical approach to the child with a suspected metabolic disorder. the associated sodium load needs to be
considered and may make other clinical
problems such a cerebral oedema worse.
Correction of altered homeostasis.
Management Reduction of toxic compound Reduction of toxic compound
The management of a child suspected of production. production
having an acute presentation of an IEM  Removal or enhancement of excretion of This is generally achieved through dietary
has three primary goals: toxic compounds. means and varies with the metabolic

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10.1 METABOLIC EMERGENCIES
10

ENDOCRINE AND METABOLIC

condition. A feature common to all dietary
Table 10.1.4 Chronic presenting signs in IEM, the associated condition and appropriate
interventions in the acutely unwell patient investigation
with an IEM is the provision of increased
Sign Disorder(s) Test
calories. Many of the IEM are in catabolic
pathways, and thus measures that help Recurrent abdominal hernia, MPS, oligosaccharidoses Urine MPS screen
especially umbilical Oligosaccharides
suppress catabolism, such as increased
caloric intake, help reduce the production Spinal deformity in infant or toddler MPS, oligosaccharidoses Urine MPS screen
Oligosaccharides
of toxic compounds.
The source of calories will vary depending Recurrent otitis media MPS, oligosaccharidoses Urine MPS screen
Oligosaccharides
on the condition (carbohydrate vs. fat vs.
protein); however, in the acute emergency Persistent nasal discharge MPS, oligosaccharidoses Urine MPS screen
Oligosaccharides
setting, extra calories from glucose are con-
sidered safe and preferable in the majority of Recurrent pain attacks, Fabry disease Lysosomal enzymes
particularly with fevers
IEM. However, protein-containing calories,
which include all regular infant formulas, Recurrent or bilateral avascular Gaucher disease Lysosomal enzymes
necrosis of the femoral head
should be avoided in the undiagnosed IEM
patient, given that most children presenting Hepatospelenomegaly Any lysosomal storage Lysosomal enzymes
disorder including MPS Urine MPS screen
acutely with IEM are in pathways of amino
acid degradation. Fat sources such as MCT Interstitial lung disease Niemann-Pick disease Lysosomal enzymes
oil or intralipid are a dense source of cal-
MPS mucopolysaccharidoses.
ories; however, they should only be used
when a fatty acid oxidation disorder has
been excluded.
Thus in the suspected IEM, dextrose at a use. Thus, patients with a known IEM known as tandem mass spectrometry or
high rate of between 5 and 10 mg kg1 min1 should always be seen promptly (ATS 2) MS/MS to screen for a large number
is the safest and best option while awaiting and those with a suspected IEM should of IEM from the one blood spot. The condi-
results and discussion with a metabolic be discussed with a metabolic physician tions screened vary between programs
clinician. when the suspicion is first raised. but generally cover most of the organic
acidaemias, aminoacidopathies and fatty
acid oxidation defects. The conditions
Removal/enhancement of Chronic presentations a local program screens for affect the
excretion of toxic compounds
There are a number of IEM that do not types of IEM that present both diagno-
There are a number of pathways through
present with acute symptoms, but in a pro- sed and undiagnosed to the ED, remem-
which toxic metabolites are removed from
gressive degenerative manner, frequently bering that these are population based
the body in IEM. Renal excretion, usually
multisystemic. The early symptoms and screening tests and inevitably miss the
conjugated to carnitine is a common path-
signs in these conditions can be common occasional case.
way, but in some conditions there is no
excretory pathway and the only treatment presentations or incidental findings in the
option is haemofiltration or dialysis. This is ED (Table 10.1.4). Appreciation of their
particularly the case in organic acidaemias significance can allow for early diagnosis
such a maple syrup urine disease in order and in a growing number of conditions an
to remove the neurotoxic amino acid, leu- improved outcome for the patient, through Conclusion
cine, and in the urea cycle disorders to help innovative new therapies such as enzyme The child with a metabolic condition
remove ammonia. replacement therapy (ERT) and, in the may present in a variety of ways to an
In the acute presentation of IEM, prompt future, gene and stem cell therapy. ED and the diagnosis should be conside-
institution of appropriate management is red in all children with an unexplained
essential to achieve a good outcome for serious illness. There should be a low
Extended newborn
the patient; previously diagnosed patients threshold for performing blood glucose
should have a care plan for acute presen-
screening in the young and initial investigations
tations, and in some centres pre-made Many newborn screening programmes and management should be instituted
supplements and formulas available for throughout the world now use a technique expeditiously.

241
 10.2 Diabetic emergencies in children
Kam Sinn

given as a bolus. The normal saline bolus

ESSENTIALS (10 mL kg1) should be repeated if the
child remains shocked upon reassessment
1 Diabetes mellitus is one of the most common chronic diseases in childhood. in 10 minutes.
2 The incidence of childhood diabetes has been increasing over the last decade. Careful and frequent monitoring should
continue for the next 2448 hours. Monitor-
3 Diabetic ketoacidosis is defined as BGL >11 mmol L
1, pH < 7.3 and bicarbonate ing should include all vital signs, including
<15 mmol L
1.
neurological assessment, urine output and
4 As obesity becomes more prevalent in childhood, type II diabetes has started to ECG monitoring.
present in younger and younger adolescents.
5 Type I diabetes remains the major (>90%) cause of childhood diabetes. Fluid
After the initial resuscitation, IV fluid consist-
lipolysis, ketosis and acidosis. It may be ing of maintenance fluid and deficit replace-
Diagnosis ment should be calculated and replaced
defined by the combination of:
The classic symptoms of polyuria, polydipsia over 48 hours. The childs degree of dehydra-
and weight loss may be present for a few
hyperglycaemia;
tion should be assessed clinically, including an
weeks before parental concern is raised.
ketosis and ketonuria;
accurate weight.
acidosis (pH < 7.3, bicarbonate <15);
The diagnosis should be confirmed by a ran- The calculation of maintenance fluid is
dom blood glucose level (BGL >11 mmol L1)
dehydration and/or shock.
based on the childs weight or surface area.
in addition to urine analysis for glucose and Management starts with rapid assessment, Dehydration in the form of deficit in per-
ketone. resuscitation and meticulous replacement centage of body weight would allow calcula-
Once the diagnosis is confirmed, initial of fluid, electrolyte and insulin infusion. tion of an estimated volume to be replaced
management is dictated by the severity of Like all medical emergencies, assessment over the next 48 hours. Maintenance and
dehydration, presence of shock, degree of of airway, breathing and circulation (ABC) is deficit replacement should be given as nor-
acidosis, hyperglycaemia and osmolality. vital. Ketotic breath, degree of tachypnoea mal saline (0.9% Na Cl) until the BGL falls
In a child with no past history of diabetes and respiratory distress should be noted. to 1215 mmol L1. Once the BGL falls to
the initial diagnosis may be misled by non- Degree of shock or dehydration should be 1215 mmol L1, the IV fluid should be
specific symptoms such as abdominal pain, assessed. Initial level of consciousness should changed to half normal saline with glucose
weight loss, drowsiness, fever, secondary be noted, and hourly neurological observation 5% (0.45% Na Cl with 5% glucose).
enuresis and dyspnoea. Beware of tachy- commenced. It is also important to look for a Beware of giving deficit replacement too
pnoea due to metabolic acidosis, intercurrent focus of infection and sepsis. rapidly, which decreases intravascular osmo-
infection in a new diabetic, abdominal pain Initial investigations should include venous lality and may contribute towards cerebral
related to diabetic ketoacidosis (DKA) and blood glucose, electrolytes, urea, creatinine, oedema.
drowsiness in a child. In such children, diabe- full blood count, venous or arterial blood
tes should be excluded as a possible cause gases. In addition, if sepsis is suspected, blood
with a random blood glucose. culture, urine culture and chest X-ray may be Insulin
considered.
Insulin infusion should only be started after
shock has been resuscitated with normal
Diabetic ketoacidosis saline intravenously as above. The BGL
Diabetic ketoacidosis is the major cause
Resuscitation would fall rapidly during the resuscitation
of mortality in diabetic children. It often Intravenous access should be established; phase with fluid replacement alone.
presents in newly diagnosed type I diabetic ideally with two intravenous (IV) cannulae Insulin infusion with short-acting insulin
children. In established diabetics, it occa- so that further venous sampling of BGL (soluble or regular) should be started with
sionally presents in the midst of inter- and electrolyte may be undertaken easily. maintenance IV fluid. An initial bolus of
current febrile illness or poor adherence to In children with shock, noted to be hypo- insulin is not recommended, as it may reduce
management. tensive and poorly perfused, resuscitation the BGL and osmolality too rapidly. The infu-
Diabetic ketoacidosis is caused by insulin should start immediately with facial mask sion is made up by diluting soluble or regular
deficiency, leading to hyperglycaemia, oxygen and intravenous fluid bolus. Normal insulin in normal saline to a concentration
osmotic diuresis, hyperosmolar dehydration, saline (0.9% Na Cl) 10 mL kg1 should be of 1 U mL1, to be given in an electronic

242
10.2 DIABETIC EMERGENCIES IN CHILDREN
10

ENDOCRINE AND METABOLIC

IV syringe pump. The infusion is started at a of cerebral oedema. The measured sodium is of oral hypoglycaemic (in type II diabetes),
rate of 0.050.1 U insulin kg1 hr1. The initially affected by hyperglycaemia. intercurrent illness (e.g. gastroenteritis),
insulin infusion rate can be titrated to The corrected sodium can be calculated by: vigorous exercise and other metabolic/
achieve a fall in BGL of 45 mmol hr1. endocrine disorders (e.g. Addisons disease).
corrected Na measured Na BGL=3
Once the BGL falls to the normal range Hypoglycaemia is often defined as a
(510 mmol L
1), the insulin infusion should BGL <2.5 mmol L1. However, symptoms
continue until the child is ready to change If there is hypernatraemia with a corrected of hypoglycaemia may appear with a BGL
over to subcutaneous insulin. As DKA is serum sodium >150 mmol L
1, rate of rehy- <4.0 mmol L1 in a diabetic. Therefore,
caused by Type I diabetes, the insulin infusion dration should be slowed down further to the level of BGL in a child with diabetes
should not be stopped or decreased in rate of 72 hours, to minimise rapid changes in should be maintained above 4 mmol L1.
infusion below 0.05 U kg1 hr1 until subcu- osmolality and fluid shift. Hypoglycaemia may cause symptoms
taneous insulin can be started, despite a related to neuroglycopenia and autonomic
normal BGL. Insulin is required to suppress activation.
Bicarbonate
lipolysis and clear ketosis. If there is a concern In mild to moderate cases, where the
about hypoglycaemia, the glucose con- Bicarbonate is not indicated despite meta- patient remains conscious, treatments
centration in the maintenance IV fluid can bolic acidosis. Bicarbonate may increase include ingestion of rapidly absorbed simple
be increased from 5% up to 10%. central nervous system acidosis and worsen carbohydrate such as sugar or fruit juice,
The change over from IV insulin to subcu- hypokalaemia and hypernatraemia. Blood followed by more complex carbohydrate
taneous insulin is often most conveniently gas should be repeated at frequent intervals and medical review of the cause of the
performed at mealtime, when the child is if the initial pH is below 7.1. hypoglycaemia.
no longer acidotic, is alert and able to toler- In severe hypoglycaemia, the patient may
ate an oral meal. At such time, rapid- or present with seizure or coma. Treatment
short-acting insulin can be given subcutane- Phosphate includes stabilisation of airway, breathing
ously before a meal; the insulin infusion Serum phosphate is often low initially. It is and circulation.
should continue during the meal and then controversial whether replacement of phos-
cease 30 minutes after the meal.
If IV access is available, 25 mL kg1
phate makes any difference to outcome. of glucose 10% can be given as an
IV bolus.
Potassium If IV access is not available; intramuscular
Complications or deep subcutaneous infusion glucagon
The initial serum potassium may be high, Approximately 0.5% children develop cere- should be given.
normal or low, despite a low total body bral oedema as a complication of DKA. The The dose of glucagons is 0.5 U (0.5 mg) for
potassium. Potassium replacement should warning signs are changes in level of con- children up to 8 years of age and 1 U (1 mg)
be given intravenously once resuscitation sciousness, irritability, headache, cranial nerve for older children and adolescents.
is completed and there are no ECG changes palsies and seizures. Treatment includes slow- In the recovery phase of severe hypogly-
of hyperkalaemia, anuria or severe renal ing the rehydration infusion, IV mannitol, caemia, close monitoring of BGL, medical
impairment. intubation and assisted ventilation. review of insulin dosage, diabetic control,
The measured serum potassium goes up by Careful and close observation of vital IV infusion of glucose-containing crystalloid
0.6 mmol L1 for every 0.1 drop in pH. As the signs, frequent review of clinical status and solution and/or additional oral complex car-
acidosis gets corrected with fluid resuscitation laboratory values of BGL, electrolytes, and bohydrate would be required.
and insulin, serum potassium will drop rapidly acidbase status are vital in the first 24 In all children with diabetes, education for
in exchange for H ions. 48 hours of DKA to minimise complications. the parents, patient, teachers and other
The potassium requirement may be as Hypoglycaemia and hypokalaemia can be carers on symptoms and management of
high as 45 mmol kg1 day1 in the first 24 minimised by frequent monitoring of BGL hypoglycaemia, sick-day management, and
hours. Initially, potassium chloride 40 mmol and electrolytes, followed by the adjustment the availability and use of glucagons are vital.
is usually added to each litre of normal saline of rehydration fluid and insulin infusions. Some form of wearable identification
for replacement. It is important that serum
(e.g. Medic-Alert bracelet) for the child
electrolyte is checked frequently every 2 to
would also help in the management of hypo-
4 hours in the first 12 to 24 hours of DKA Hypoglycaemia glycaemic coma.
management.
Hypoglycaemia is a common complication in
all diabetics. Education and ongoing support
Long-term management
for the diabetic child and his/her family is
Sodium
the key to preventing and managing hypo- The key to good long-term management of
Serum sodium is a major determinant of glycaemia when it does occur. children with diabetes is a team and holistic
osmolality. Rapid change and fall in serum Hypoglycaemia can be caused by insulin approach to education for the affected child
sodium should be avoided to minimise the risk excess (e.g. honeymoon period), overdose and his/her parents.

243
10.3 THYROID EMERGENCIES

Regular medical review of insulin dosage, National Collaborating Centre for Womens and Childrens

diet, exercise, medium-term control and Further reading Health. Type 1 diabetes diagnosis and management of type
1 diabetes in children and young people. Available from
Australasian Paediatric Endocrine Group for the Department
complications should be undertaken with a of Health and Ageing. Clinical practice guidelines: Type 1
http://www.nice.org.uk/nicemedia/live/10944/29394/
29394.pdf; 2004 [accessed 19.10.10].
paediatrician, diabetic educator and dieti- diabetes in children and adolescents. Available from
http://www.chw.edu.au/prof/services/endocrinology/
cian. It is beyond the scope of this chapter apeg/apeg_handbook_final.pdf; 2005 [accessed
to deal with the details of long-term man- 19.10.10].
agement of childhood diabetes.

10.3 Thyroid emergencies

Malcolm Higgins

Treatment
ESSENTIALS Further investigation and management of
hyperthyroidism in children and adolescents
1 Life-threatening paediatric thyroid emergencies are rare.
will optimally occur following referral to
2 Children with undiagnosed thyrotoxicosis or hypothyroidism may present to the ED a paediatric endocrinologist or general
with a range of acute symptoms and signs. paediatrician. Therapy will depend on the
cause but may include antithyroid drugs.
3 The identification, appropriate management and referral of children with
b-Blockers are useful for initial control of
congenital hypothyroidism is important as this condition may cause severe
symptoms, particularly in thyroid storm.
neurological impairment if untreated.
Thyroid storm is a medical emergency.
4 The onset of clinical hypothyroidism in Hashimotos thyroiditis may occur in Attention to airway, breathing and circula-
adolescents. tion are the initial priorities. Although
antithyroid medications such as pro-
pylthiouracil (57 mg kg
1 day
1 in three
symptoms in Graves disease is usually insid- divided doses given orally) or carbimazole
Thyrotoxicosis
ious and variable in severity between (0.2 mg kg
1dose
1 in three divided doses,
Hyperthyroidism is generally a disease of patients. Severe ophthalmopathy or dermo- given orally) may be indicated, control of
adult women but occasionally may present pathy is rare in children. Most patients with symptoms may be delayed. In patients
in adolescents. Although there are a number Graves disease will have a goitre charac- with cardiac manifestations, propranolol
of causes the most common is Graves disease, terised by diffuse, non-tender and symmetri- (24 mg kg
1 day
1 in two divided doses
which is thought to have an autoimmune cal enlargement of the thyroid gland. given orally) should be considered.
basis. In approximately 12% of cases the The clinical presentation of thyroid
presentation of hyperthyroidism is acute storm is of abrupt onset of high fever and
and severe. This has been called the thyroid marked tachycardia and hypertension, with Table 10.3.1 Signs and symptoms
of thyrotoxicosis
storm and is potentially life threatening. exaggerated features of hyperthyroidism
Other causes of hyperthyroidism in children (Table 10.3.1). Altered mental state is invari- Symptoms
include thyroiditis, iodine-induced hyper- ably present and may progress to seizures Nervousness/irritability
Heat intolerance and increased sweating
thyroidism, thyroid-stimulating hormone and coma. Weight loss
(TSH) hypersecretion, excessive ingestion of Behaviour problems and poor school
performance
thyroid hormone and thyroid neoplasms. Fatigue
Hyperthyroidism is one of several endocrino- Restless sleep/insomnia
Diagnosis Palpitations
pathies associated with McCuneAlbright Diarrhoea
In most cases, low TSH with raised free T4 Menstrual irregularities
syndrome.
and T3 will be diagnostic of hyperthyroidism.
Other investigations such as thyroid auto- Signs
Tachycardia, hypertension
Clinical features antibodies and radionuclear scans are not Thyroid enlargement with bruit or thrill
Raised levels of circulating thyroid hormone usually part of the emergency department Tremor
Warm, moist skin
have predictable clinical effects depending (ED) assessment. Muscle weakness
on the organ system and are similar to the Thyroid storm is a clinical diagnosis and Eyelid lag and retraction
Brisk tendon reflexes
symptoms and signs of catecholamine treatment should not be withheld while Growth acceleration
excess (see Table 10.3.1). The onset of waiting for laboratory results.

244
10.3 THYROID EMERGENCIES
10

ENDOCRINE AND METABOLIC

Diagnosis of congenital hypothyroidism is
Neonatal thyrotoxicosis Congenital hypothyroidism confirmed by demonstrating decreased levels
This rare condition is usually due to the trans- Inadequate thyroid hormone production in of serum thyroid hormone (total or free T4)
placental transfer of thyroid-stimulating anti- newborn infants can occur from anatomic and elevated levels of TSH. Urgent referral
bodies from a mother with autoimmune defects of the thyroid, an inborn error of for further investigation and initiation of
hyperthyroidism. Importantly, antibody levels thyroid metabolism, or from maternal therapy is important.
sufficient to cause neonatal thyrotoxicosis iodine deficiency. Thyroid hormone is vitally
may not cause clinical hyperthyroidism in important to brain growth and develop-
the mother. ment. Profound mental retardation is the
Hashimotos thyroiditis
The infant may develop clinical features most serious effect of untreated congenital Autoimmune thyroiditis is generally a condi-
of hyperthyroidism. Poor weight gain, hypothyroidism. tion of adult women. Adolescents may be
cardiac dysfunction and hepatosplenome- Fortunately, the majority of infants with affected. The clinical features are of insidious
galy may also be present. Cardiac failure congenital hypothyroidism are diagnosed onset (Table 10.3.3). Diagnosis is confirmed
and airway compression from the goitre soon after birth via the newborn screening with a TSH assay supplemented by thyroid
may occur. Onset of symptoms may be program. A small number of cases will be hormone levels, thyroid autoantibodies and
delayed if the mother is on antithyroid missed and present for medical attention a thyroid scan. Referral to an appropriate
medication. Although symptoms usually because of symptoms of hypothyroidism. specialist is highly recommended.
resolve spontaneously, this condition is Suggestive symptoms and signs are listed
associated with significant morbidity and in Table 10.3.2. Table 10.3.3 Symptoms and signs
mortality. of Hashimotos thyroiditis
Weakness and lethargy
Weight gain
Table 10.3.2 Symptoms and signs Cold intolerance
Hypothyroidism of congenital hypothyroidism

Constipation
Depression, emotional lability and personality
Poor feeding, growth and development changes
Insufficient thyroid hormone leads to slow- Constipation Forgetfulness and poor concentration
ing of bodily functions and can impair the Umbilical hernia, enlarged fontanelle Hoarse/husky voice
Enlarged, protruding tongue Goitre
function of many organ systems. The most Prolonged neonatal jaundice Menorrhagia and menstrual irregularity
important causes in the paediatric patient Hypotonia Bradycardia
Hoarse cry Hypothermia
are congenital hypothyroidism and autoim- Bradycardia, cool extremities Delayed relaxation of deep tendon reflexes
mune (Hashimotos) thyroiditis.

245
 10.4 Adrenal crisis
Yuresh Naidoo

adrenal aplasia/hypoplasia;
ESSENTIALS adrenal infarction secondary to
haemorrhage/sepsis;
1 The prompt recognition of the possibility of adrenal crisis or the risk of adrenal other trauma, tumour, post-surgical.
insufficiency is paramount to early and appropriate management.
Secondary causes include:
2 Adrenal crisis should be considered as a possible contributor in any child with central nervous system (CNS) tumour
acute severe cardiovascular collapse. or trauma;
idiopathic;
3 In children, the majority of cases are due to primary adrenal failure, with exogenous steroid therapy
congenital adrenal hyperplasia the most common cause.
including inhaled corticosteroids.
4 A crisis can be precipitated in a child with known adrenal insufficiency who
develops an intercurrent illness or other physiological stress.
5 Signs of glucocorticoid deficiency include hypoglycaemia, hypotension (absolute Clinical presentation
and postural) and refractory shock.
Clinical features may be subtle; however,
6 Signs of mineralocorticoid deficiency include dehydration (often out of the diagnosis should be considered in all
proportion to estimated fluid losses), hyperkalaemia, hyponatraemia, acidosis, and children with cardiovascular collapse. Fea-
pre-renal failure. tures to look for in history, examination
and investigation findings are listed below,
7 Patients are at risk of hypoglycaemia. with differential diagnosis.
8 The management of adrenal crisis involves immediate fluid resuscitation,
replacement of corticosteroid and treating hypoglycaemia.
History
9 The differential diagnosis of a collapsed neonate (particularly male) in the first Prior steroid use, including inhaled
14 days includes adrenal insufficiency. steroids for asthma or other condition.
Known congenital adrenal hyperplasia or
10 Prevention of adrenal crisis in susceptible children may be possible by following a other adrenal insufficiency on
predetermined action plan during intercurrent illnesses.
replacement therapy.
Severe physiological stress (sepsis,
trauma, burns, surgery).
symptoms of lack of adrenal hormones or On anticoagulants, haemorrhagic
Introduction a child who is at risk due to suppression by diathesis.
prolonged steroid therapy. The prompt rec- Neonate with collapse and/or
Adrenal crisis is a life-threatening emer-
ognition of the possibility of adrenal crisis hypoglycaemic event or ambiguous
gency caused by acute insufficiency of the
or the risk of adrenal insufficiency is para- genitalia.
adrenal hormones cortisol and aldosterone.
mount to early and appropriate manage- Symptoms of glucocorticoid deficiency
This can occur in situations of stress where
ment. These children are at risk of weakness, fatigue, lethargy, anorexia,
the adrenal gland would normally respond
hypoglycaemia and this needs to be antici- vomiting, diarrhoea, weight loss.
by an increase in glucocorticoid secretion.
pated and managed accordingly.
A crisis can be precipitated in a child with
Adrenal insufficiency can be primary, due
known adrenal insufficiency, who develops Examination
to a failure of secretion of the adrenal cortex,
an inter-current illness or other physiological
or secondary to hypothalamic or pituitary
Signs of glucocorticoid deficiency
stress (e.g. burns, surgery, trauma, and sep- hypoglycaemia, hypotension (absolute
dysfunction. In children, the majority of
sis). In this situation, the increased cortisol and postural), refractory shock.
cases are due to primary adrenal failure, with
requirements of the stress or the altered
congenital adrenal hyperplasia the most
Signs of mineralocorticoid deficiency
oral intake of normal replacement therapy dehydration (often out of proportion to
common cause. The incidence of neonatal
results in a relative insufficiency and rapid estimated fluid losses), hyperkalaemia,
congenital adrenal hyperplasia in Australia
clinical deterioration. Alternatively, the hyponatraemia, acidosis, pre-renal
is estimated at 5.9 cases per 100 000 births.
emergency department (ED) visit may repre- failure.
sent a new presentation of adrenal insuffi- Primary causes include: Signs of excess adrenocorticotropic
ciency in a child previously unrecognised congenital adrenal hyperplasia; hormone secretion pigmentation of
to have the subtle, often non-specific Addisons disease (autoimmune); skin, lips, nipples, skin creases.

246
10.4 ADRENAL CRISIS
10

ENDOCRINE AND METABOLIC

Signs of glucocorticoid therapy Remaining estimated deficit plus mainte-
Cushings syndrome. nance volumes should then be replaced with Disposition
Signs of associated hypothalamic/ 5% dextrose and normal saline over a 24- All children with an established adrenal cri-
pituitary abnormality growth hour period. The maintenance fluid require- sis require inpatient admission.
abnormality, midline defects, ments are 1.5 times normal in this setting Discharge may be considered in milder
hypogonadism, diabetes insipidus, and clinical and biochemical (electrolytes cases, after a period of 6 hours observation,
hypothermia. and glucose) reassessment is required to tai- for those susceptible patients who respond
lor fluid therapy to the individual. well to treatment with oral fluids and in-
Investigations creased dose of intramuscular hydrocortisone.
Bedside: finger-prick glucose Replacement of corticosteroid
(hypoglycaemia), electrocardiogram Hydrocortisone should be given intrave-
(ECG) (hyperkalaemia). nously, unless access is significantly delayed, Prognosis
Biochemical: glucose, urea and where it should be administered IM as an In the absence of bilateral adrenal haemor-
electrolytes, arterial blood gas, save interim alternative. The appropriate dose rhage, the survival rate of patients with
clotted blood for cortisol level and 17- can be determined by age: adrenal crisis who are diagnosed and treated
hydroxyprogesterone, if no underlying appropriately approaches that of patients
diagnosis is known.
neonate: 25 mg stat., then 1025 mg
6-hourly; without acute adrenal crisis with similar
severity of illness. Because the true inci-
1 month1 year: 25 mg stat., then 25 mg
Differential diagnosis 6-hourly; dence of adrenal crisis and bilateral adrenal
Other causes of hyponatraemia haemorrhage is unknown, the actual mortal-
13 years: 50 mg stat., then 50 mg
syndrome of inappropriate antidiuretic 6-hourly; ity rate is also unknown.
hormone hypersecretion, nephrogenic or 410 years: 75 mg stat., then 75 mg
cerebral salt wasting, gastrointestinal or 6-hourly;
urinary losses. Prevention
>10 years: 100 mg stat., then 100 mg
Other causes of shock septicaemia, 6-hourly. At-risk children with an intercurrent illness
profound dehydration, duct-dependent may prevent an adrenal crisis with an action
cardiac lesion. Maintenance doses of glucocorticoid and plan for parents:
mineralocorticoid are introduced after the
child has been stabilised as an inpatient. if moderately unwell and/or temperature
Treatment is 3839 C give 3 oral dose of oral
Generally, glucocorticoid replacement dose
The management of adrenal crisis involves
is 1015 mg m2 day1 orally and mineralo- hydrocortisone;
immediate fluid resuscitation, replacement
corticoid dose is 0.10.2 mg day1 for salt- if more unwell and/or temperature
of corticosteroid and treating hypoglycae-
wasting children. >39 C give 4 dose of oral
mia. The underlying illness causing the
hydrocortisone;
stress needs to be treated on its merits.
if vomiting give IM 2 mg kg1;
Potential complications, such as hyperkalae- Hypoglycaemia
Hypoglycaemia is treated in the routine
If gastroenteritis or diarrhoea give 4
mia, may require intervention. In children
dose of oral hydrocortisone.
not previously known to be adrenal defi- fashion using a lower concentration of intra-
cient, blood (prior to administration of gluco- venous dextrose in smaller children. Mainte- NB. Only the dose of hydrocortisone should
corticoid) should be held for analysis to nance fluid will generally require 510% be increased, not fludrocortisone. When
determine if an underlying condition exists. dextrose solution added to normal saline. the stress is over the previous dose should
be resumed without tapering.
neonate/infant: 5 mL kg1 of 10%
Fluid management dextrose stat.;
Patients known to have adrenal deficiency older child: 2 mL kg1 of 25% dextrose Controversies and future
who are not dehydrated or shocked can have stat.;
a trial of enteral fluids in the ED. They should
directions
maintenance fluid: 510% dextrose-
be considered to have vomited or potentially containing solution. The role of screening for congenital
not absorbed normal replacement adrenal adrenal hyperplasia (CAH) is
medication, and should be administered controversial. In Australia there is
Hyperkalaemia
intramuscular (IM) hydrocortisone 2 mg kg1 currently no programme in place.
Hyperkalaemia should be treated if K >7
to ensure delivery.
mmol L1 with ECG changes. Research indicates that early
Children who are dehydrated or shocked
1 screening may reduce the morbidity or
require intravenous resuscitation with crys- 0.5 mL kg of 10% calcium gluconate mortality associated with adrenal crisis.
talloid. Initial boluses of 20 mL kg1 normal over 35 minutes;
saline should be titrated to restore periph- 0.1 U kg1 hr1 of insulin 2 mL kg1  National newborn screening awaits the
eral circulation in those children with shock. hr1 of 50% dextrose infusion. development of a more cost-efficient test.

247
10.5 DISORDERS OF FLUIDS, ELECTROLYTES AND ACIDBASE

Royal Childrens Hospital. Clinical Practice Guidelines.

New research suggests that critically Further reading Adrenal Crisis. Available from www.rch.org.au; 2005
[accessed 19.10.10].
ill children with adrenal crisis may be best Charmandari E, Lichtarowicz-Krynska EJ, Hindmarsh PC, et al. The Childrens Hospital at Westmead. The New Childrens
managed with a single intravenous bolus Congenital adrenal hyperplasia: Management during Hospital Handbook. Acute adrenal insufficiency.
critical illness. Arch Dis Child 2001;85(1):268. Available from www.chw.edu.au; 2005 [accessed
dose of hydrocortisone followed by a Fischer JE, Stalmach T, Fanconi S. Adrenal crisis presenting as 19.10.10].
constant rate infusion. This in preference hypoglycaemic coma. Intens Care Med 2000;26:1058. Todd GRG, Acerini CL, Ross-Russell R, et al. Survey of adrenal
Gassner HL, Toppari J, Quinteiro Gonzalez S, Miller WL. Near- crisis associated with inhaled corticosteroids in the United
to the 6-hourly boluses used currently. miss apparent SIDS from adrenal crisis. J Pediatr 2004; Kingdom. Arch Dis Child 2002;87:45761.
145(2):17883.
A further recent research Kirkland L. Adrenal crisis. eMedicine. www.emedicine.com;
Van der Kemp HJ, Noordham K, Elvers B, et al. Newborn
screening for congenital adrenal hyperplasia in the
development is that CAH can be 2003. Netherlands. Paediatrics 2001;108(6):13204.
Macdessi JS, Randell TR, Donaghue KC, et al. Adrenal crisis in
diagnosed and treated prenatally if a children treated with high-dose inhaled corticosteroids for
mother has previously had a child with asthma. Med J Aust 2003;178(5):2146.
Omori K, Nomura K, Shimizu S, et al. Risk factors for adrenal
CAH. crisis in patients with adrenal insufficiency. Endocr J
2003;50(6):74552.

10.5 Disorders of fluids, electrolytes

and acidbase
Barry Wilkins Wayne Hazell

ESSENTIALS Introduction
1 The most common causes of hypovolaemic shock in paediatric patients are sepsis, Disorders of blood volume, body fluids,
dehydration and trauma. sodium, potassium and acidbase are com-
mon in acutely ill children. Always manage
2 Vigorous restoration of the circulating volume reduces mortality in hypovolaemic shock.
the airway and ventilation first, the ABC
3 Isotonic or near-isotonic fluids such as 0.9% saline, 4% albumin in 0.9% saline, blood principle of resuscitation, even in acute ill-
products, Hartmanns or Ringers solutions are appropriate for volume resuscitation. Hypotonic ness where shock and dehydration are dom-
solutions including 5% glucose, 0.18%, 0.225% or 0.45% saline are inappropriate. inant. In resuscitation of the circulation,
4 After resuscitation of circulating volume, residual dehydration should be corrected slowly vigorous replacement of blood volume
with physiological solutions, such as Hartmanns solution or 0.9% saline, or 0.45% saline in the deficit is urgent, but correction of residual
presence of hypernatraemia. dehydration is not. Maintenance fluids are
the last consideration.
5 The degree of dehydration is easily overestimated.

6 The concept of maintenance fluids applies only after resuscitation of circulating volume and
repair of dehydration or water overload. Even then fluids must be individualised to the patients
particular needs.

7 Hyponatraemia generally reflects water excess, and creates a risk for cerebral and Physiology
pulmonary oedema. Hypotonic fluids are contraindicated until the plasma sodium is corrected.
There are many physiological differences
Cerebral oedema may develop rapidly in hyponatraemic children, especially after administration
of hypotonic fluids. between adults and infants, and a few spe-
cific features that must be taken into
8 Rapid correction of hyper- or hyponatraemia is contraindicated. account in managing fluid therapy. Infants
9 Hyperkalaemia with electrocardiogram (ECG) changes requires urgent potassium-lowering have greater total body water, up to 70%
treatment. of body weight compared with 60% in
adults, the extra fluid being mostly extracel-
10 Metabolic acidosis is common in sick children. The anion gap remains useful in determining
the cause.
lular, 30% of body weight compared with
20%1,2 (Fig. 10.5.1). The ionic composition
11 Treatment of acidosis is aimed at correcting the underlying cause. Alkali therapy is rarely of intracellular and extracellular fluid is
indicated.
shown in Table 10.5.1. Small children drink
12 Metabolic alkalosis is caused by vomiting, especially pyloric stenosis, or as a compensation more to accommodate a higher metabolic
for chronic respiratory failure. rate and excrete a higher solute load, and
thus urine volume is greater.

248
10.5 DISORDERS OF FLUIDS, ELECTROLYTES AND ACIDBASE
10
suggests a 50100 mL kg
1 water deficit;

ENDOCRINE AND METABOLIC

100 Body fluid compartments The capacity of the infants brain and
heart for anaerobic metabolism is greater 34 seconds 100120 mL kg
1, and over
90
than the adult. 4 seconds >150 mL kg
1. However, ambi-
80 The infants kidney is less able to ent temperature affects capillary return.5
concentrate urine and to handle a sodium In children less than 4 years old clinicians
70
Percentage of body weight

TBW load in the first 2 years of life. Thus infants overestimate the degree of dehydration by
60 M
are less able to compensate for fluid loss. 3.2%.6 Other studies have suggested that
F
Renal blood flow and glomerular the sensitivity of clinical examination for
50
ICF filtration rate are less per unit body diagnosing dehydration is 74, 33 and 70%
M
40 surface area. for mild, moderate and severe dehydration
F

30 Infants have less capability to acidify the respectively.7 See Tables 10.5.2 and 10.5.3
ECF urine and are thus less able to deal with a for clinical signs associated with dehydration
20 M
and electrolyte imbalance.8
F hyperchloraemic acidosis caused by too
10 much chloride administration. Plasma bicarbonate concentration may
Total body water and extracellular fluid be the single most useful laboratory test; a
0
Birth 3 6 9 1 3 5 7 9 11 13 15 Adult are proportionally higher in the first 6 level less than 17 mmol L
1 indicates mod-
Months Years erate or severe dehydration. Addition of this
Age months of life. In later childhood
extracellular fluid declines and to the clinical scale improves the sensitivity
Fig. 10.5.1 Body fluid compartments in
intracellular fluid increases due to cell of diagnosing moderate and severe dehydra-
children. Source: Adapted from Henning, 1995.1
multiplication. tion to 90 and 100% respectively. Plasma
Limited glycogen stores exist. bicarbonate was a better predictor than
The following physiological differences
Hypoglycaemia frequently occurs in plasma urea and creatinine.7
apply to children:13
periods of stress in infants and young Shock is a disorder characterised by a
Infants are more dependent on heart rate children. decrease in end-organ oxygenation and/or
for cardiac output. perfusion. This does not solely depend on blood
Infants have less ability to increase pressure and pulse rate. Blood pressure can be
myocardial contractility. maintained in the infant with shock being
Clinical assessment present. Hypotension is a preterminal sign.
The infants ventricle is less compliant.
Higher atrial pressures are required for In assessing the degree of dehydration, End-organ perfusion can best be assessed
the same degree of ventricular filling. sequential body weight measurement is the by the conscious state, capillary return, urine
The child is more dependent on the most accurate measure of water loss. How- output and degree of metabolic acidosis.
extracellular concentration of calcium. ever, previous normal body weight is seldom
Storage and release of calcium from the available in the emergency department (ED).
sarcoplasmic reticulum in the infants Capillary refill times were studied in 32 Haemorrhagic shock
myocardium is less efficient. children, aged 126 weeks, admitted to Clinical signs are of value in assessing
The infant tolerates more tachycardia hospital with dehydration.4 The authors degree of haemorrhage.9 See Table 10.5.4.
and hypotension. recommended a cut-off value of 2 seconds, Hypotension is a preterminal sign.
Degenerative diseases are uncommon in below which minimal or no dehydration
children. exists. A capillary refill time of 23 seconds
Fluid deficit
Table 10.5.1 Ionic distribution of body fluid compartments In the case of dehydration, once the percent-
age loss of body weight (PLBW) is estimated
Cations and anions Intracellular fluid Interstitial fluid Plasma (mEq L
1)
(mEq L
1) (mEq L
1) from clinical and laboratory tests the fluid
deficit can be estimated.
Sodium (Na) 10 145 140

Potassium (K) 150 4 4 Fluid deficit litres PLBW % 

estimated body weight kg
Magnesium (Mg) 30 2 2

Calcium (Ca2) 0 5 5 Thus a 10 kg, 1- year-old who is 10% dehy-

drated has a fluid deficit of:
Chloride (Cl
) 5 114 104

Bicarbonate (HCO3
) 10 24 24 10% of 10 kg 1 kg 1000 g

1 litre or 1000 mL
Protein (Pr
) 65 6 15

Phosphate (PO4
) 95 (organic) 4 (inorganic) 4 (inorganic) As this is an estimate, ongoing clinical para-
Sulfate (SO4
) 20 1 1
meters must be assessed including aiming
for a urine output of >1.0 mL kg
1 hr
1.

249
10.5 DISORDERS OF FLUIDS, ELECTROLYTES AND ACIDBASE

cardiomyopathy;
Table 10.5.2 Degree of dehydration based on percentage of body weight lost*
arrhythmias, especially supraventricular
Variablea Mild (35%) Moderate (69%) Severe (10% or more) tachycardia;
Blood pressure Normal Normal Normal to reduced effusion.
Quality of pulses Normal Normal or slightly Moderately decreased
Liver disease
decreased acute hepatic failure;
chronic hepatic failure.
Heart rate Normal Increased Increasedb
Renal disease
Skin turgor Normal Decreased Decreased nephrotic syndrome.
Fontanelle Normal Sunken Sunken Protein-losing enteropathy
Mucous membranes Slightly dry Dry Dry
Hereditary angio-oedema
Excessive water and sodium intake
Eyes Normal Sunken orbits Deeply sunken orbits Other causes of hypoalbuminaemia.
Extremities Warm, normal Delayed capillary refill Cool, mottled
capillary refill

Mental status Normal Normal to listless Normal to lethargic or

comatose Investigations

1
1
1
1 Most children with gastroenteritis do not
Urine output Slightly decreased <1 mL kg hr <<1 mL kg hr
warrant an IV line or investigations. How-
Thirst Slightly increased Moderately increased Very thirsty or too
lethargic to indicate thirst ever, the following biochemical tests should
be performed in children presenting with
*For each dehydration category, the percentages of body weight lost varied among authors.
a
See Table 10.5.3 for the physical signs of hypernatraemia and hypokalaemia in dehydration. shock or significant problems of water and
b
Bradycardia may occur in children with severe dehydration. electrolyte imbalance that require intrave-
Source: Based on Burkhart, 1999.8
nous treatment or where such disorders
are expected, e.g. respiratory disease, renal
Oedema disease, liver disease and encephalopathy:
Table 10.5.3 Signs of hypernatraemia
and hypokalaemia in dehydration Oedema suggests that considerable water plasma sodium, potassium, urea,
and salt retention has occurred. Mild sodium creatinine, osmolality, glucose,
Hypernatraemia
and water retention do not cause clinical bicarbonate, lactate;
Cutaneous signs
Warm, doughy texture oedema, nor does water retention without plasma calcium, magnesium, phosphate,
Possibly decreased skin-fold tenting in sodium retention, e.g. the syndrome of in-
severe dehydration, thereby giving liver function tests, albumin;
appearance of lower level of dehydration appropriate antidiuretic hormone secretion arterial and/or venous pH and gases if
rarely causes oedema. Oedema is most obvi- shocked.
Neurological signs
ous in dependent and distensible subcutane-
Hypertonia Venous or intraosseous blood samples give a
Hyperreflexia ous tissue such as the genitalia, eyelids,
Lethargy common, but marked irritability lower legs and lower back. Firm pressure satisfactory measurement of electrolytes,
when touched and of respiratory and metabolic acidbase
for at least 1 minute is needed to detect
Hypokalaemia subtle cases. Causes include: status.
Weakness
Ileus with abdominal distension Heart failure urine sodium, potassium, urea, creatinine,
Cardiac arrhythmias
structural cardiac disease; osmolality, glucose, and dipstick test for
myocarditis; protein, red cells and casts (random spot
Source: Based on Burkhart, 1999.8
sample).
A urine sample aspirated from a cotton wool
Table 10.5.4 Classes of haemorrhagic shock9 ball placed at the perineum is satisfactory
for all measurements except urine calcium.
Class of Blood volume Signs
haemorrhage lost (%) Plasma osmolality can be calculated as
well as measured in order to detect an osmo-
I <15 Minimal, slight tachycardia
lar gap.
II 1530 Tachycardia, tachypnoea, diminished pulse pressure, systolic BP
unchanged, prolonged capillary refill, minimal decrease in urine
output, anxiety
Osmolality mosmol kg
1
1:86  Na K glucose
III 3040 Tachycardia, tachypnoea, decreased BP, decreased urine output,
mental status changes urea 10 all in mmol L
1
IV >40 Hypotension, anuria, loss of consciousness
If measured osmolality is greater than this
Source: Based on Morgan and ONeill, 1998.9 figure by 5 mosmol kg
1 or more, then an

250
10.5 DISORDERS OF FLUIDS, ELECTROLYTES AND ACIDBASE
10

ENDOCRINE AND METABOLIC

unmeasured solute is present such as an Ringers solutions are more physiological, con-
alcohol, e.g. ethanol or ethylene glycol. Treatment taining buffer and calcium.1012
Ingestion of alcohols may cause a high Replacement of circulating In dehydration, the loss of fluid and electro-
anion gap ketoacidosis and hypoglycaemia. volume lytes is similar to the composition of extracel-
Accurate timed urine collections are rarely Also called volume resuscitation, this is an lular fluid; this is predominantly where the loss
possible in the ED. A useful indication of urgent priority in any cause of hypovolaemic comes from. Thus the deficit is best replaced
urine flow rate can be obtained from a spot shock, e.g. haemorrhage, sepsis, burns, ana- with a solution that approximates extracellu-
urine sample, based on the relatively constant phylaxis and dehydration. It requires isotonic lar fluid, i.e. 0.9% saline (normal saline).
creatinine excretion between individuals. For fluids (Table 10.5.5). Hypotonic fluids are However, as fluid replacement is not an
example, a urine creatinine of 2000 mmol L
1 inappropriate. Crystalloids are inexpensive exact science, and due to the fact that main-
represents urine flow of 24 mL kg
1 hr
1, and readily available. Colloids have a theoret- tenance fluids also need to be given, a solu-
and 8000 mmol L
1 represents 0.51 mL ical advantage of increasing the colloid onco- tion somewhere between 0.9% saline and
kg
1 hr
1. tic pressure of plasma, thus helping to 4% dextrose plus 0.18% saline is given.
Fractional sodium excretion (FENa) is a maintain fluid in the vascular space. However, Saline (0.45%) with added glucose (2.5%)
useful diagnostic tool. It represents the in a capillary leak syndrome such as septic and sometimes potassium would be appro-
proportion of filtered sodium that is not shock, the colloid may pass into the intersti- priate. Clinical parameters and serum bio-
reabsorbed and is <1% in health. It is given tial space. In practice, albumin in saline solu- chemistry are sequentially measured and
by the formula: tion has been tested against 0.9% saline in the fluid is adjusted accordingly.
Urine Na Plasma creatinine adult intensive care patients. There was no
FENa  Overall 24
hour fluid requirement
Urine creatinine Plasma Na difference in mortality or intensive care unit
(ICU) stay between the two therapies. More fluid deficit maintenance
A high FENa suggests any cause of natriuresis, saline was administered than albumin (ratio ongoing losses
including acute renal failure, a renal salt- 1.38:1), suggesting that albumin may be
superior in patients where excessive water An initial bolus or boluses of 20 mL kg
1 to
wasting disorder or diuretics. Hyperosmolar
administration may be harmful, e.g. pulmo- treat shock may be part of this initial fluid deficit.
urine with high urine creatinine and low FENa
nary oedema, pulmonary hypertension and Saline (but not Hartmanns, Ringers and
suggests dehydration of non-renal cause. Dia-
encephalopathy. There was a suggestion that Plasmalyte) tends to cause a metabolic
betes insipidus causes hypo-osmolar urine
acidosis because of a dilution of bicarbonate
(often <100 mosmol kg
1), low urine creati- albumin may be superior in sepsis but inferior
by chloride, the principal extracellular buffer,
nine (often <1000 mmol L
1) and plasma in traumatic brain injury.10 A problem with
saline is that it has no bicarbonate and and a relative hyperchloraemia.
hyperosmolality. Plasma urea is high in most
relatively high chloride, so it can lead to Those containing lactate, gluconate or
cases of dehydration because of reduced urea
hyperchloraemic acidosis. Hartmanns and acetate are more physiological solutions,
clearance.

Table 10.5.5 Common crystalloid and colloid IV fluids

Content per litre 0.9% NaCl 0.45% NaCl 3% 5% Hartmanns Plasmalyte Albumin Gelatine
(normal (half-normal NaCl glucose solution based
saline) saline) products

Na (mmol L
1) 154 77 500 130 140 140 145154

1
K (mmol L ) 45 5 5


1
Cl (mmol L ) 154 77 500 109 98 134 120145
2 2
1 2 2
Ca or Mg (mmol L ) Ca 2 Mg 1.5 Ca2 06

Buffer (mmol L
1) Lactate 28 Acetate 27 Octanoate 6

Gluconate 23

Glucose (g L
1) 50

1
Osmolarity (mosmol L ) 285 145 900 278 274 280 280293

Number average molecular 69 000 23 00024 500

weight (Mn)

Weight average molecular 69 000 30 00035 000

weight (Mw)

Duration of action 6 hours 34 hours

Survival in the body 21 days 7 days

Starches have not been included in this table because they are very infrequently used in children in Australia and the United Kingdom.

251
10.5 DISORDERS OF FLUIDS, ELECTROLYTES AND ACIDBASE

the buffer being rapidly metabolised to viscosity of the fluid and length of the tube. and effective. It can be effective even in
bicarbonate. The small radius can be overcome by applying the case of vomiting.2126
Glucose may be added to the sodium a larger pressure differential across the tub- Recommendations for oral and intrave-
chloride preparations. ing. Fluid can be drawn up in a 20 or 50 mL nous hydration in gastroenteritis as given
syringe and injected rapidly. It is harder to by the American Academy of Pediatrics
How much fluid? push fluid through a small cannula with a are contained in Table 10.5.7. Oral
Enough fluid should be given in shock to 50 mL syringe.
result in the improvement and disappear- A three-way tap can be connected to a
ance of the signs of shock. normal giving set attached to the IV fluid Table 10.5.7 American Academy of
Pediatrics (AAP) recommendations for
The blood volume of an infant is approxi- of choice. The tap can be turned off to the oral rehydration therapy (ORT) in children
mately 7080 mL kg
1. Decompensation in patient allowing fluid to be drawn into the based on estimated degree of
haemorrhagic shock starts to occur in class 3 syringe. The tap can then be turned off to dehydration*

haemorrhagic shock. At this stage it can be the giving set and fluid can rapidly be No dehydration
presumed that at least 30% or approximately injected into the patient. Oral rehydration therapy: not generally
20 mL kg
1 of blood has been lost. Ideally, blood should be given through the needed unless the child is feeding poorly or is
not taking other fluids well; then give
Thus a bolus dose of 20 mL kg
1 is a logi- largest cannula that can be inserted. As well 10 mL kg-1 of an oral rehydration solution for
each diarrhoeal stool
cal bolus dose of fluid with which to begin as the usual complications of blood transfu- Feeding: continue age-appropriate dieta
resuscitation. sion, the paediatric patient is at risk of
However, in dehydration and sepsis much hyperkalaemia from cell lysis through the Mild dehydration (35% body weight loss)
Oral rehydration therapy: give 50 mL kg-1 of
larger fluid losses and shifts have occurred small cannula. an oral rehydration solution plus replace
and the total body water and extracellular Infants have a higher surface area to body ongoing fluid lossesb over a 4-hour period; re-
evaluate hydration and estimate ongoing fluid
fluid are likely to be depleted far more than weight ratio and lose heat more quickly. losses every 2 hours
20 mL kg
1. If crystalloid is given, not all of Overhead radiant warmers or fluid warmers Feeding: resume age-appropriate dieta as
soon as dehydration is corrected and emesis
the fluid stays in the intravascular space. are recommended for large rapid volume resolves
Capillary leakiness and third space losses replacement when hypothermia is a risk.
in sepsis contribute to the ongoing loss of Moderate dehydration (69% body weight
loss)
fluid from the vascular space. Investigation and management
Oral rehydration therapy: in a supervised
Thus in dehydration repeated boluses of fluids in different conditions setting, give 100 mL kg
1 of an oral
may be necessary. Give a bolus, wait 10 min- Gastroenteritis and dehydration rehydration solution plus replace ongoing
fluid lossesb over a 4-hour period; re-evaluate
utes and reassess the patient again. Do not Dehydration is a common presenting feature hydration and estimate ongoing fluid losses
wait 10 minutes if it is clear that more than in children. Causes are outlined in Table 10.5.6. every hour
Feeding: resume age-appropriate dieta when
20 mL kg
1 will be required.1315 Oral rehydration either by mouth or naso- dehydration is fully corrected
In severe sepsis such as meningococcae- gastric tube is the method of choice for rehy-
Severe dehydration (10% or greater body
mia, 80100 mL kg
1 may be required. dration unless shock exists. It is safe, cheap weight loss; implies shock or near-shock)
Good evidence exists that early and vigorous Intravenous therapy: give a rapid intravenous
resuscitation improves morbidity and mor- bolus of 20 mL kg
1 of normal saline solution
Table 10.5.6 Causes of dehydration or Ringers lactate; repeat as needed until the
tality in paediatric sepsis and meningococ- child is haemodynamically stable and shock
caemia.16 The volume and the rapidity of Gastrointestinal loss resolves; if the child does not respond to one
Diarrhoea or more boluses, consider causes of shock
resuscitation seems to be more important Non-obstructive vomiting other than fluid loss from diarrhoea and
than the type of fluid used.1719 Obstructive vomiting emesis
Third space loss into bowel wall and Oral rehydration therapy: when the child is
In haemorrhagic shock, boluses of peritoneum stable and alert, begin administration of an
10 mL kg
1 of whole blood or packed cells oral rehydration solution; keep intravenous
Renal loss line in place until the child is drinking well
may be given. If fresh frozen plasma (FFP) is Feeding: resume age-appropriate dieta when
required this can also be given in 10 mL kg
1 Renal tubular disease
Acquired tubulopathies
dehydration is corrected

aliquots. Chronic renal failure including obstructive

*These recommendations cover children from 1 month
uropathy
to 5 years of age with no pre-existing comorbid
Adrenal failure
conditions who live in developed countries. Specifically
How to administer fluid in shock Congenital adrenal hyperplasia (salt-losing)
excluded are children who have diarrhoea lasting more
Diabetes insipidus (central and renal)
Intravenous access is often difficult in a Osmotic diuresis (e.g. DKA)
than 10 days, diarrhoea associated with failure to thrive
and/or vomiting in the absence of diarrhoea. If the
shocked young infant, but it is essential that Diuretics
physician is unsure of the dehydration category for a
this is achieved as soon as possible. If intrave- specific patient, the therapy for the more severe
Insensible loss category should be used.
nous access has not been achieved within 90 Burns a
The diet should emphasise complex carbohydrates,
seconds and/or three failed attempts have Sweating lean meats, yogurt, fruits and vegetables. Fatty foods,
Hyperthyroidism and foods and fluids that are high in simple sugars
occurred, intraosseous access should be gained. Hyperventilation with reduced intake should be avoided.
Poiseuilles law states that the flow through Ichthyosis, e.g. Netherton syndrome b
Replacement of ongoing fluid losses should include
an amount for the estimated volume of emesis and
a hollow tube is proportional to the radius to Reduced intake (rarely causes dehydration) 10 mL kg
1 for each diarrhoeal stool.
the power of four and inversely related to the Source: Based on Burkhart, 1999.8

252
10.5 DISORDERS OF FLUIDS, ELECTROLYTES AND ACIDBASE
10

ENDOCRINE AND METABOLIC

Table 10.5.8 Oral rehydration solutions

Product Electrolyte content (mEq L
1)

Na K Cl
Citrate Carbohydrate Calories Osmolality How supplied

(per 30 mL) (mosmol kg
1)

Commercial 4575 2025 3565 3048 2030 g L
1 of 34.2 250310 60 mL freezer pops or
preparations glucose (dextrose)* 2401000 mL ready-to-use,
or rice syrup solids flavoured or unflavoured

WHO oral 90 20 80 30 20 g L
1 of glucose 2.5 310 Dry packets, mixed with 1 L
rehydration water
solution
salts, mixed

Home mix 90 20 80 30 20 g L
1 of glucose 2.5 310 Mix 3.5 g (one half teaspoon) of
(recipe or 40 g L
1 of table salt, 1.5 g (one half
developed sucrose teaspoon) of potassium chloride
by WHO) or potassium salt, 2.5 g (one half
teaspoon) of baking soda and
either 20 g (two tablespoons) of
glucose or 40 g (four
tablespoons) of sucrose in 1 L of
water)

Na, sodium; K, potassium; Cl
, chloride; WHO, World Health Organization.

*Some freezer pops also contain phenylalanine and aspartame.
Source: Based on Burkhart, 1999.8

rehydration should occur with one of the liquid form.27 One study suggested that chil- dehydration (see Table 10.5.5) can cause
recommended oral rehydration solutions dren with a serum bicarbonate greater than hypernatraemia. Rarely, hyperaldosteronism
(Table 10.5.8). No evidence exists to show 13 mmol L
1, and who were vomiting, could or salt poisoning cause sodium excess.
that one formula is more effective than be treated with rapid intravenous rehydra- The clinical features are usually those
another.22 These fluids resemble 0.45% tion (2030 mL kg
1 of isotonic crystalloid of dehydration. Coma and seizures may
saline and 2.5% glucose with added potas- over 12 hours) in the ED.28 They could then occur, especially if plasma sodium is
sium, a useful intravenous solution in this tolerate oral rehydration and be discharged >160 mmol L
1 at any time, and if there
condition.8 home. Shorter stays in the ED were also are rapid changes in circulating volume or
Clear liquids or soft drinks are not to be enabled compared to times reported with plasma chemistry. Some neurological deficit
encouraged. These may be high in carbohy- oral rehydration solution. may result from the encephalopathy of
drate and osmolality and lead to osmotic severe hypernatraemia.
diarrhoea. Hyponatraemia may occur due Hypernatraemia and Fractional sodium excretion is low because
to their low sodium content. Chicken broth hypernatraemic dehydration of a normal physiological response to dehy-
is inappropriate as it may lead to hyperna- Hypernatraemia is usually caused by water dration, but may be high if the cause is an
traemia8 (Table 10.5.9). depletion, or water plus sodium depletion osmotic diuresis, excessive diuretic use or in
Some trials suggest frozen oral rehydra- in dehydration states where there is rela- salt poisoning. The urine is concentrated
tion solution is better tolerated than the tively more water depletion. Most causes of (osmolality >600 mosmol kg
1) except in
diabetes insipidus.
Shock is treated with isotonic fluids until
Table 10.5.9 Electrolyte and carbohydrate content of common clear liquids haemodynamically stable, then 0.225
Liquid Electrolyte content (mEq L
1) 0.45% saline, with potassium as needed
(40 mmol L
1 if not hyperkalaemic). The
Na K HCO3
Carbohydrate (g L
1) Osmolality
(mosmol kg
1)
estimated dehydration should be corrected
over 48 hours with slow correction of plasma
Cola 2 0.1 13 50 to 150, glucose and fructose 550
sodium at no more than 0.6 mmol L
1 hr
1
Ginger ale 3 1 4 50 to 150, glucose and fructose 540 (15 mmol L
1 day
1).
Apple juice 3 20 0 100 to 150, glucose and 700 If salt poisoning is suspected, obtain gas-
fructose tric fluid via nasogastric tube for sodium
Chicken broth 250 5 0 0 450 analysis. Features of dehydration are usually
absent and FENa is high, often >10%. If
Tea 0 0 0 0 5
there is salt excess without dehydration,
Sports drinks 20 3 3 45, glucose and other sugars 330 then give less than maintenance water with
Na, sodium; K, potassium; HCO3
, bicarbonate.
no sodium, aiming for a very slow correction
Source: Based on Gremse, 1995.23 of plasma sodium, <0.6 mmol L
1 hr
1.

253
10.5 DISORDERS OF FLUIDS, ELECTROLYTES AND ACIDBASE

A diuretic may be considered and, in this relates to the severe hyponatraemia A study of severe meningococcaemia17
extreme cases, dialysis.2931 itself or the rapid correction. Convulsions looked at 336 patients retrospectively and
and decreasing level of consciousness may found a decreased mortality, both in the
Diabetes insipidus occur at any time in treatment, especially general population and in high-risk groups,
Diabetes insipidus should be treated with if there are rapid changes in circulating vol- when FFP was not used.
glucose/water, replacing the previous hours ume or plasma chemistry. Some neurological Thus, early vigorous fluid management is
urine volume plus 1020 mL kg
1 day
1 for deficit may result from the encephalopathy the key.
insensible losses. Hyperglycaemia may of severe hyponatraemia.
result. An intravenous vasopressin infusion After treatment of shock, residual dehydra-
tion should be managed by replenishing Haemorrhagic shock
may be needed, but only if the urine output
extracellular space with 0.9% sodium chloride Initial resuscitation can take place with crys-
is stable, which means usually after admis-
or one of the buffered isotonic solutions, talloid or colloid. Whole blood at 10 mL kg
1
sion to a ward or ICU, and after consulting
slowly over 48 hours. Residual hyponatraemia can be given and repeated as necessary until
an endocrinologist.
should correct itself slowly. blood pressure is restored.
Water intoxication needs water restriction FFP should be thawed if a large transfu-
Hyponatraemia and hyponatraemic sion is expected, and platelets requested.
dehydration to half maintenance and sodium supplemen-
tation if there is evidence of natriuresis. FFP only comes in adult-sized bags and doses
Hyponatraemia may be caused by water of 10 mL kg
1 are appropriate with repeat-
excess (e.g. water intoxication, inappropri- ing of coagulation times.9
ate antidiuretic hormone (ADH)), water Syndrome of inappropriate
and salt retention where the water excess antidiuretic hormone secretion
is greater than the sodium excess (e.g. (SIADH) Head injury
nephrotic syndrome, heart failure, renal fail- This condition is over diagnosed. Plasma Vigorous treatment of shock should be
ure or liver failure), dehydration where concentration of antidiuretic hormone, vaso- undertaken to maintain cerebral perfusion
sodium depletion is greater than the water pressin, is elevated, despite physiological pressure.32
depletion (e.g. renal loss, gastrointestinal conditions which should suppress it, namely Interest has been generated in the use of
loss, third-space loss, congenital adrenal hypo-osmolality and water overload. 7.5% saline in head-injured patients. A pro-
hyperplasia, acute adrenal failure), or abnor- Hyponatraemia and hypo-osmolality are spective randomised study of 35 patients
mal solute in extracellular fluid (e.g. glucose accompanied by marked oliguria, but with head injury compared Ringers lactate
in uncontrolled diabetes) causing water shift with high fractional sodium excretion and with 7.5% saline.33 Children treated with
from intra- to extracellular space. very high urine osmolality and sodium (often hypertonic saline had a statistically signifi-
Hyponatraemia may cause nausea, leth- >200 mmol L
1). The child is sodium cant decrease in complications, intracranial
argy, depressed consciousness, raised intra- depleted but water overloaded, so the man- pressure and ICU stay. There was no differ-
cranial pressure and seizures, especially if agement of choice is water restriction, the IV ence, however, in survival or duration of
of rapid onset. fluid being given as 0.9% saline. Furosemide hospital stay. Neurological outcome was
Urine osmolality and plasma urea are causes a diuresis in SIADH and may contrib- not well documented. More evidence is
high in dehydration, but low in water intoxi- ute to management. required.3335
cation. FENa is high in salt-losing states,
inappropriate ADH and often in acute renal Pyloric stenosis
failure. Shock is treated with 20 mL kg
1 boluses of Burns
Treatment of shock is according to stan- 0.9% saline. Saline (0.9%) with 40 mmol L
1 The original Parkland formula of
dard guidelines and can be instigated safely of KCl is the appropriate rehydration solution. 34 mL kg
1 per percentage burn is still
with normal saline. Severely symptomatic Glucose needs to be added. This remedies the applicable to burns patients. This is over
patients with plasma sodium less than loss of chloride and potassium, and slowly cor- the first 24 hours and starts at the time of
120 mmol L
1, such as those with seizures rects the alkalosis. the burn, not admission to ED.
or coma (which is likely to be associated with Originally, 50% of this was to be given in
cerebral oedema), should have the sodium Sepsis and meningococcal disease the first 8 hours and the rest over the
corrected rapidly to 125 mmol L
1 (but no There is no compelling argument for crystal- remaining 16 hours. However, recent evi-
higher), using 3% saline (0.5 mmol mL
1). loid or colloid.1821 A study of septic shock14 dence suggests that restoration of urinary
The following formula may be useful: found that fluid resuscitation in excess of output and vital signs occurs earlier if
40 mL kg
1 in the first hour was associated 50% is given in the first 4 hours. Many burns
Sodium required mmol with improved survival, decreased occur- patients require more fluid than is provided
125
plasma sodium mmoL=L  rence of persistent hypovolaemia and no by the Parkland formula. Aim for urine out-
0:6  weight kg increase in the risk of acute respiratory dis- put greater than 1 mL kg
1 hr
1.35
Excessive rapid correction of hyponatraemia tress syndrome or pulmonary oedema. No Some evidence also suggests that paedia-
can be associated with pontine or extrapon- restriction was placed on the type of resusci- tric burns patients tolerate hypoalbuminae-
tine myelinolysis, but it is not clear whether tation fluid used. mia well and may not require albumin.36

254
10.5 DISORDERS OF FLUIDS, ELECTROLYTES AND ACIDBASE
10

ENDOCRINE AND METABOLIC

Diabetic ketoacidosis promote potassium entry into cells. This is Maintenance rates of 0.18% sodium chlo-
Treatment of shock should include boluses of more effective and longer lasting than glu- ride with 20 mmol L
1 added potassium
20 mL kg
1 of colloid or normal saline. Some cose/insulin (0.1 U kg
1 insulin plus provide 40100 mL kg
1 day
1 of water,
solutions contain potassium and should ide- 0.5 g kg
1 glucose over 3060 minutes). 1.53 mmol kg
1 day
1 of sodium and
ally not be used in hyperkalaemic renal Resonium, 1 g kg
1 every 46 hours oral 0.71.5 mmol kg
1 day
1 of potassium,
failure. or rectal, may eliminate 12 mmol kg
1 of which are normal quantities in healthy
Rehydration over 48 hours is desirable so potassium. children.
as to minimise the risk of cerebral oedema. All cases of hyperkalaemia should be A common formula for calculating main-
Cerebral oedema is almost exclusively a admitted with ECG monitoring. tenance water requirements in a healthy
condition of the newly diagnosed young dia- child is as follows:
betic, with 95% of cases occurring under 20 Hypokalaemia
Hypokalaemia is often an incidental finding
for the first 10 kg of body weight:
years of age.
100 mL kg
1 day
1 or 4 mL kg
1 hr
1;
The fluid of choice is controversial. One on blood chemistry and usually represents
potassium depletion. Causes of potassium
for the second 10 kg of body weight:
article suggests that normal saline be used
50 mL kg
1 day
1 or 2 mL kg
1 hr
1;
until restoration of glucose and then a depletion include vomiting, diuretics,
secretory diarrhoea, ureterosigmoidostomy,
for every subsequent kg of body weight:
change should be made to 0.45% saline.
25 mL kg
1 day
1 or 1 mL kg
1 hr
1.
Potassium replacement should be started renal tubular acidosis, hyperaldosteronism,
when the urinary output is adequate.37 anorexia nervosa and diabetic ketoacidosis. Reduce this by one-third initially, or even
Causes of hypokalaemia without depletion one-half if there is risk of cerebral oedema,
include salbutamol use, alkalosis and famil- e.g. in meningitis or brain injury, especially
Hyperkalaemia ial periodic paralysis. if hyponatraemia already exists. Less than
Hyperkalaemia is often discovered incidentally Mild hypokalaemia is often asymptomatic maintenance water should be given when
on routine chemistry, but may be suggested but tachyarrhythmias, ileus, weakness and water retention is likely, such as in sepsis
by ECG changes. It may be associated with rhabdomyolysis may occur. Hypokalaemia and severe respiratory disease (0.7  main-
renal failure, hypoxia-ischaemia or acidosis. associated with alkalosis usually corrects tenance), cardiac failure (0.5  mainte-
There may not be potassium excess but rather itself as the pH corrects. nance), renal failure or other oliguric
potassium shift from intra- to extracellular Treat potassium depletion by slow states without hypovolaemia (0.3  mainte-
fluid), b-blockers, rhabdomyolysis, haemolysis, potassium supplementation at 2 mmol kg
1 nance plus urine output) (Table 10.5.10).
potassium sparing diuretics, acute adrenal day
1 or greater. Never give 0.5 mmol kg
1 Greater than maintenance is often advo-
failure and potassium poisoning. in 1 hour without ECG monitoring and fre- cated when water losses are expected, e.g.
Mild hyperkalaemia may be asymptom- quent repeat measurement. Concentration spontaneous hyperventilation or fever, but
atic but potassium levels >6 mmol L
1, >40 mmol L
1 of infusion fluid should be it is probably more appropriate to incre-
especially when acute, may cause weakness, given into a central venous catheter. ase fluids only if urine output falls to
peaked T-waves and wide PR interval on 0.5 mL kg
1 hr
1. The most appropriate
ECG, and then loss of P wave, heart block Maintenance fluids maintenance IV fluid in the acutely ill child
and asystole. The concept of maintenance fluids refers to is 0.45% saline because at half-mainte-
Investigations should include a blood gas, healthy children, where the kidneys are able nance rates this provides 13 mmol kg
1
creatine kinase (CK) and glucose. to conserve or excrete water and salt over a day
1 of sodium, but separate consideration
Any plasma potassium >6 mmol L
1, or wide range, in each case according to intake should always be given to sodium and
<6 but rising fast, demands urgent treat- and non-renal losses. Maintenance is a vol- other electrolyte requirements. Do not
ment. If K >7 mmol L
1 or there are ECG ume of water intake which maintains urine include dehydration deficit in maintenance;
changes, consider giving calcium 0.10.15 output in the middle of the normal range consider this separately and use 0.9%
mmol kg
1, which does not change plasma with an osmolality about that of extracellu- saline or a buffered isotonic solution for
potassium but protects cell membranes. lar fluid. However, changes in total body replacement.
Beware of subcutaneous infiltration of the water and sodium and other electrolytes
infusion. Sodium bicarbonate may also be are common in many diseases. Insensible
given, especially if the child is acidotic, skin loss of water may be high because of
13 mmol kg
1 over 3060 minutes, but its fever and the higher surface area:weight
Acidbase disorders
value is not certain. If plasma potassium is ratio in infants. Maintenance is only relevant Disorders of physiological control of acidity
>6.5 mmol L
1, consider arranging early after restoration of circulating volume and of body fluids are common in acutely ill chil-
dialysis. Discuss with the nephrology and total body water, and is therefore relevant dren. The system of defining acidbase state
intensive-care services. to ongoing rather than ED care. A mainte- by changes in pCO2 (respiratory) and stan-
Milder degrees of hyperkalaemia respond nance amount should be a starting amount. dardised base excess (metabolic) accord-
to 510 mcg kg
1 IV salbutamol over a few It may be excessive for any sick child where ing to the Copenhagen school remains the
minutes (or 2.55 mg of inhaled nebulised there may be diminished ability to excrete most familiar way of analysing acidbase
salbutamol if there is no IV cannula), to water. disorders. Base excess is mostly bicarbonate

255
10.5 DISORDERS OF FLUIDS, ELECTROLYTES AND ACIDBASE

Most metabolic acidbase disturbances

Table 10.5.10 Guide to ongoing water therapy
do not need treatment. Compensating
Weight (kg) 0.7  maintenance the mechanisms should generally not be trea-
commonest starting point Full maintenance
ted, otherwise the primary disturbance is

1
1
1
1
(mL hr ) (mL kg day ) (mL h ) (mL kg
1 day
1) exacerbated. It is not necessary to restore
4 11 70 16 100 pH to normal.
6 17 70 24 100

8 22 70 32 100
Metabolic acidosis
Normal physiology maintains extracellular
10 28 70 40 100 pH close to 7.4 in health, but permits meta-
12 31 62 44 88 bolic acidosis at times of anaerobic metabo-
lism without danger. Metabolic acidosis may
14 34 57 48 82
be advantageous because it is thought to
16 36 55 52 78 protect cells against the effects of hypoxia,
18 39 53 56 75 and assists oxygen unloading from haemo-
globin by shifting the oxygen/haemoglobin
20 42 50 60 72
dissociation curve to the right.
30 49 39 70 56 Acidosis is said to cause negative inotropy
40 56 34 80 48 or failure of inotropes to work at pH < 7.2,
but this has little experimental support.
50 63 30 90 43
Acidosis causes pulmonary vasoconstriction
60 70 28 100 40 and may predispose to arrhythmias caused
70 77 27 110 38 by other electrolyte abnormalities.
In metabolic acidosis, bicarbonate deficit
(mmol kg
1) is given by:

Weight kg  24
plasma
deficit, but includes a small amount of buff- way of determining the unmeasured compo-
bicarbonate mmol L
1   0:5
ering by albumin, and a larger amount by nent is from the formula:
haemoglobin. Standardised base excess is Plasma lactate estimation should be per-
Unmeasured anion
provided by blood gas machines derived Na K Mg2 Ca2
Cl

formed if there is significant base deficit or
by microprocessor rather than the original HCO3

albumin  0:123  pH
high anion gap. Urine for drug screen may
nomograms. The philosophy of base excess 0:631
phosphate  0:309  pH
be indicated. For suspected inborn error of
has been challenged because it does not 0:469
lactate
all in mEq L
1 metabolism if the anion gap is greater than
take into account the actual plasma albu- 20, send urine for metabolic screen.
min concentration and assumes a notional Note that Mg2 and Ca2 are divalent and First treat the underlying disease
haemoglobin concentration of 50 g L
1 so are 2  mmol L
1. (Table 10.5.11), including general manage-
across blood and extracellular fluid in all ment of renal, hepatic failure, cardiac failure,
Albumin  (0.123  pH 0.631) is the
patients. It can be argued that the bicar- hypoxia, shock and hypovolaemia. Alkali is
negative charge on albumin, derived from
bonate concentration alone is a sufficient not usually needed for mild acidosis
its known buffer curve.
measure of the degree of metabolic acidosis (pH > 7.2) because the acidosis itself does
Phosphate  (0.309  pH 0.469) is the
or alkalosis. not cause any compromise. Even extreme
negative charge on phosphate, derived
The anion gap remains a useful tool acidaemia (pH < 6.8) can be followed by
from its known buffer curve.
in determining whether any bicarbonate full recovery, and bicarbonate therapy is
Plasma albumin at 4045 g L
1 is
deficit is caused by organic acid (high anion only indicated if there is severe hyperkalae-
0.6 mmol L
1 but contributes 12 mEq L
1
gap) or by chloride excess (normal anion mia or tricyclic poisoning. Sudden changes in
to the anion at pH 7.4. Phosphate at
gap). It is obtained from the formula: acidbase status should be avoided. Sodium
1.5 mmol L
1 contributes 2.7 mEq L
1.
Anion gap Na K
Cl

bicarbonate has many adverse effects,
The normal value for unmeasured anion is especially when given rapidly, such as hypo-
HCO3
all in mEq L
1 56 mEq L
1, including 1 mEq L
1 of lactate. kalaemia, decreased plasma ionised cal-
The normal is 16 mEq L
1 and is essentially Any excess is abnormal. This may be lac- cium, sodium load, osmolar load, increased
the negative charge on albumin and phos- tate in hypoxiaischaemia, acetoacetate or haemoglobin/oxygen affinity, exacerbation
phate. Any excess is accounted for by abnor- b-hydroxybutyrate in DKA, ketones in star- of effects of hypophosphataemia, and late
mal unmeasured acid and/or lactate. Most vation, organic acids in inborn errors of metabolic alkalosis. There is no evidence
modern laboratories, including blood gas metabolism or alcohol poisoning or toluene for increased intracellular acidosis. Sodium
machines, measure lactate. A more accurate inhalation. bicarbonate is especially unhelpful in lactic

256
10.5 DISORDERS OF FLUIDS, ELECTROLYTES AND ACIDBASE
10

ENDOCRINE AND METABOLIC

4. Saavedra JM, Harris GD, Li S, Finberg L. Capillary refilling
Table 10.5.11 Causes of acidosis (skin turgor) in the assessment of dehydration. Am J Dis
Child 1991;145:2968.
High anion gap Normal anion gap 5. Gorelick MH, Shaw KN, Baker N. Effect of ambient
temperature on capillary refill in healthy children.
Diarrhoea (starvation/ketosis) Renal tubular acidosis Pediatrics 1993;92:699702.
Sepsis Other renal tubulopathies which include 6. Mackenzie A, Barnes G, Shann F. Clinical signs of
Diabetic ketoacidosis bicarbonate loss dehydration in children. Lancet 1989;ii:6057.
Renal failure Gastroenteritis 7. Vega RM, Avner JR. A prospective study of the usefulness
Hepatic failure Intestinal fistulae or enterostomies of clinical and laboratory parameters for predicting
Drugs and poisons (e.g. salicylates, ethanol, methanol, Excessive sodium chloride administration percentage of dehydration in children. Pediatr Emerg
ethylene glycol) Hypoaldosteronism Care 1997;13:17982.
Inborn errors of metabolism (especially in babies) 8. Burkhart DM. Management of acute gastroenteritis in
Hypoxia children. Am Fam Phys 1999;60:255563.
Circulatory failure 9. Morgan WM, ONeill JA. Hemorrhagic and obstructive
shock in pediatric patients. New Horiz 1998;6:1504.
10. The SAFE Study Investigators. A comparison of albumin
acidosis, resulting in sodium overload with plus 40 mmol L
1 potassium chloride with and saline for fluid resuscitation in the Intensive Care
Unit. N Engl J Med 2004;350:224756.
a metabolic alkalosis as the lactic acid added glucose. Hypotonic solutions, which 11. Schierhout G, Roberts I. Fluid resuscitation with colloid
or crystalloid solutions in critically ill patients: A
is metabolised to bicarbonate during may exacerbate hyponatraemia, should
systematic review of randomised trials. Br Med J
recovery. not be used. Intravenous hydrochloric acid 1998;316:9614.
12. Emery EF, Greenhough A, Gamsu HR. Randomised
Slow sodium bicarbonate treatment may (or arginine hydrochloride) is indicated in
controlled trial of colloid infusions in hypotensive
have a role in the management of normal rare severe cases when alkalosis may be preterm infants. Arch Dis Child 1992;67:11858.
13. Schexnayder SP. Pediatric septic shock. Pediatr Rev
anion gap acidosis where excessive chloride depressing respiratory drive, and when the
1999;20:3037.
therapy would exacerbate hyperchloraemia. chloride deficit is not accompanied by 14. Carcillo JA, Davis AL, Zaritsky A. Role of early fluid
A suitable amount is 2 mmol kg
1 day
1. In sodium deficit. Give hydrochloric acid resuscitation in pediatric septic shock. JAMA
1991;266:12425.
diabetic ketoacidosis bicarbonate is not (150 mmol L
1 solution) by central intrave- 15. Goh AYT, Chan PWK, Lum LCS. Sepsis, severe sepsis and
septic shock in paediatric multiple organ dysfunction
recommended acutely, even in severe acid- nous catheter. Give half-correction over at
syndrome. J Paediatr Child Health 1999;35:48892.
aemia with pH < 7.0. However, ketoacids least 1 hour. 16. Pollard AJ, Britto J, Nadel S, et al. Emergency
management of meningococcal disease. Arch Dis Child
are excreted by osmotic diuresis in prefer-
Dose of HCl for full 1999;80:2906.
ence to chloride, so it may be helpful to 17. Busund R, Straume B, Revhaug A. Fatal course in
correction mmol severe meningococcemia: Clinical predictors and
include about a fifth of the sodium replace- weight kg  plasma bicarbonate effect of transfusion therapy. Crit Care Med
ment as bicarbonate given just as slowly as mmol L
1 
24  0:5 1993;21:1699705.
the rest of the fluid. This may avoid hyper- 18. McClelland B. Human albumin administration in critically
ill patients. Br Med J 1998;317:882 [letter].
chloraemia and tachypnoea after the meta- Acidifying diuretics such as acetazolamide 19. Cochrane Injuries Group Albumin Reviewers. Human
bolic disorder is corrected. There is, however, albumin administration in critically ill patients:
may be indicated in metabolic alkalosis
Systematic review of randomised controlled trials.
no intravenous fluid preparation available where there is sodium and water retention. Br Med J 1998;317:23540.
that contains bicarbonate. 20. Nadel S, De Munter C, Britto J, et al. Albumin: Saint or
sinner? Arch Dis Child 1998;79:3845.
Correct hypoglycaemia. If an inborn error 21. Moyer VA, Elliott EJ. Evidence-based pediatrics: The
of metabolism is suspected give glucose Controversies and future future is now. J Pediatr 2000;136:2824.
22. Mackenzie A, Barnes G. Randomised controlled trial
at >8 mg kg
1 min
1, correct electrolyte directions comparing oral and intravenous rehydration therapy in
children with diarrhoea. Br Med J 1991;303:3936.
imbalance, and partially correct acidosis There is debate about the use of 23. Gremse DA. Effectiveness of nasogastric rehydration in
with bicarbonate, giving this over at least albumin in sepsis and meningococcal hospitalised children with acute diarrhea. J Pediatr
1 hour (see above). Gastroenterol Nutr 1995;21:1458.
disease. 24. Barnes GL. Oral rehydration solutions in gastroenteritis
before and after admission to hospital. J Paediatr Child
Metabolic alkalosis There is continuing controversy Health 1996;32:167.
25. Eliason BC, Lewan RB. Gastroenteritis in children:
Metabolic alkalosis may be caused by about whether crystalloid or colloid is Principles of diagnosis and treatment. Am Fam Physician
chronic potassium and/or chloride deple- preferable in paediatric resuscitation, 1998;58:176976.
26. Wittenberg DF, Ramji S. Paediatric diarrhoea rehydration
tion, e.g. vomiting, especially pyloric stenosis and which colloid, if any. therapy revisited. S Afr Med J 1995;85:6558.
27. Santucci KA, Anderson AC, Lewander WJ, Linakis JG.
(accompanied by volume depletion) or renal  Hypertonic saline has been Frozen oral hydration as an alternative to conventional
(including diuretic use). It may be compensa- advocated in hypovolaemic shock but enteral fluids. Arch Pediatr Adolesc Med
1998;152:1426.
tory (chronic renal bicarbonate retention) in further research is required. 28. Reid SR, Bonadio WA. Outpatient rapid intravenous
chronic respiratory failure, in which case it rehydration to correct dehydration and resolve vomiting
in children with acute gastroenteritis. Ann Emerg Med
should not be treated. Acute alkalosis caus- 1996;28:31823.
ing tetany by lowering plasma ionised cal- 29. Ng PC, Chan HB, Fok TF, et al Early onset of
hypernatraemic dehydration and fever in exclusively
cium should be treated. References breast fed infants. J Paediatr Child Health
After correction of dehydration, chloride 1. Henning R. Fluid resuscitation in children. Emerg Med. 1999;35:5857.
deficit is approximately 6 mmol kg
1 for Second Australian Symposium on Fluid Replacement
1995;5762.
30. Moritz ML, Ayus JC. The changing pattern of
hypernatremia in hospitalised children. Paediatrics
every 10 mmol L
1 fall in plasma chloride. 2. Cullen P. Fluid resuscitation in infants and children. Curr 1999;104:4359.
Anaesth Crit Care 1996;7:197205. 31. Dunn K, Butt W. Extreme sodium derangement in a
A suitable fluid for treating alkalaemic 3. Tobias JD. Shock in children: The first 60 minutes. Pediatr paediatric inpatient population. J Paediatr Child Health
patients is 0.9% or 0.45% sodium chloride Ann 1996;25:3308. 1997;33:2630.

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32. Scalea TM, Maltz S, Yelon J, et al. Resuscitation of Bickell WH, Wall MJ, Pepe PE, et al. Immediate versus Holliday MA, Segar WE. Reducing errors in fluid
multiple trauma and head injury: Role of crystalloid fluids delayed fluid resuscitation for hypotensive patients therapy management. Pediatrics 2003;111:4245
and inotropes. Critl Care Med 1994;22:16105. with penetrating torso injuries. N Engl J Med [Commentary].
33. Simma B, Burger R, Falk M, et al. A prospective, 1994;331:11059. Ichikawa I, Yoshioka T, editors. Paediatric textbook of fluids
randomised, and controlled study of fluid management in Bohn D. Problems associated with intravenous fluid and electrolytes. Baltimore: Williams & Wilkins; 1990.
children with severe head injury: Lactated Ringers administration in children: Do we have the right solution? Kamel KS, Wei C. Controversial issues in the treatment of
solution versus hypertonic saline. Pediatr Crit Care Curr Opin Paediatr 2000;12:21721. hyperkalemia. Nephrol Dial Transpl 2003;18:22158.
1998;26:126570. Brown WD, Caruso JM. Extrapontine myelinolysis with Kellum JA. Saline-induced hyperchloremic metabolic acidosis.
34. Sheikh AA, Matsuoka T, Wisner DH. Cerebral effects of involvement of the hippocampus in three children Crit Care Med 2002;30:25961.
resuscitation with hypertonic saline and a new low with severe hypernatraemia. J Child Neurol Kelly A, Moshang T. Disorders of water, sodium and potassium
sodium hypertonic fluid in hemorrhagic shock and head 1999;14:42833. homeostasis. In: Nichols DG, editor. Rogers textbook of
injury. Crit Care Med 1996;24:122632. Chan JCM, Gill JR, editors. Kidney electrolyte disorders. New pediatric intensive care. Baltimore: Lippincott Williams &
35. Cocks AJ, OConnell A, Martin H. Crystalloids, colloids and York: Churchill Livingstone; 1990. Wilkins; 2008, p. 161534.
kids: A review of paediatric burns in intensive care. Burns Coulthard MG, Haycock GB. Distinguishing between salt Laureno R, Karp BI. Myelinolysis after correction of
1998;24:71724. poisoning and hypernatraemic dehydration in children. Br hyponatremia. Ann Intern Med 1997;126:5762.
36. Sheridan RD, Prelack MS, Cunningham JJ. Physiological Med J 2003;326:15760. McClure RJ, Prasad VK, Brocklebank JT. Treatment of
hypoalbuminemia is well tolerated by severely burned Doyle JA, Davis DP, Hoyt DB. The use of hypertonic saline in hyperkalemia using intravenous and nebulised salbutamol.
children. J Trauma: Injury. Infect Crit Care the treatment of traumatic brain injury. J Trauma 2001;50: Arch Dis Child 1994;70:1268.
1997;43:44852. 36783. Meyers A. Fluid and electrolyte therapy for children. Curr Opin
37. Edge JA. Management of diabetic ketoacidosis in Duke T, Molyneux EM. Intravenous fluids for seriously ill Paediatr 1994;6:3039.
childhood. Br J Hosp Med 1996;55:50812. children: Time to reconsider. Lancet 2003;362: 13203. Myers CT. Minimal volume, hypotense resuscitation. Emerg
Feld LG. Hyponatremia in infants and children: A practical Med 1995;516. Second Australian Symposium on Fluid
approach. J Nephrol 1996;9:59. Replacement.
Finberg L, Kravath RE, Hellerstein S, editors. Water and Phin SJ, McCaskill ME, Browne GJ, Lam LT. Clinical pathway
electrolytes in pediatrics. Physiology, pathology and using rapid rehydration for children with gastroenteritis.
treatment. 2nd ed. Philadelphia: WB Saunders; 1993. J Paediatr Child Health 2003;39:3438.
Further reading Fraser CL, Arieff AI. Epidemiology, pathophysiology and Rodriguez-Soriano J. Potassium homeostasis and its
Arieff AI. Postoperative hyponatraemic encephalopathy management of hyponatremic encephalopathy. Am J Med disturbances in children. Paediatr Nephrol 1995;9:
following elective surgery in children. Paediatr Anaesth 1997;102:6777 [review]. 36474.
1998;8:14 [editorial]. Gerigk M, Gnehm HPE, Rascher W. Arginine vasopressin and Ronco C, Bellomo R, Kellum J, editors. Critical care nephrology.
Arieff AI, Ayus JC. Treatment of symptomatic hyponatremia: renin in acutely ill children: Implication for fluid therapy. Philadelphia: Saunders Elsevier; 2009.
Neither haste nor waste. Crit Care Med 1991;19:74851 Acta Paediatr 1996;85:5503. Tuthill DP, Hewson M, Wilson R. Paediatric resuscitation by
[editorial]. Halberthal M, Halperin ML, Bohn D. Acute hyponatremia in phone. J Paediatr Child Health 1998;34:5247.
Arieff AI, Ayus JC, Fraser CL. Hyponatremia and death or children admitted to hospital: Retrospective analysis of Wilkins B. Fluid therapy in acute paediatrics: A physiological
permanent brain damage in healthy children. Br Med J factors contributing to its development and resolution. approach. Curr Paediatr 1999;9:516.
1992;304:121822. Br Med J 2001;322:7802.

258
 11

SECTION
HAEMATOLOGY AND
ONCOLOGY
Section editor Jeremy Raftos

11.1 The use of blood products in children 259 11.5 Vasculitis 274
11.2 Anaemia 263 11.6 Acute leukaemia 277
11.3 Disorders of coagulation 270 11.7 Febrile neutropenia 279
11.4 Platelet disorders 272

11.1 The use of blood products in children

Joseph Ting

signs, comorbidities) and laboratory indica-

ESSENTIALS tions; benefitrisk of blood product use
requires careful consideration. In the setting
1 Blood components are appropriately used when clinical benefits of administration of massive haemorrhage whole blood could
outweigh potential hazards, provided the indication is appropriate. Although blood
be used. Otherwise, there is no indication
product administration is relatively safe, potential risks remain.
for the use of whole blood when specific
2 Use specific component(s) appropriate to a clinical problem whenever possible. component(s) appropriate to a clinical
Determine and document urgency for transfusion, the specific blood product to be problem are available.
administered, quantity and duration of transfusion and special instructions (e.g. Emphasis on blood component safety,
premedication). standardisation of appropriate guidelines
for use of blood components and informed
3 For safe use of blood products, hospitals must have a policy for blood product consent for blood component administration
administration, including the staff responsible, credentialing, documentation,
have led to a substantial reduction in the
checking and administration procedures such as identity checks. The majority of
potential risks and complications of their
adverse events result from administrative errors.
use as well as increasing their appropriate
4 Blood loss with poor perfusion or/ and haemodynamic derangement requires rapid usage. Informed consent with regards to
whole blood or packed red blood cell (PRBC) transfusion while a decision is made the risks and benefits of blood component
regarding the need for definitive haemostasis. therapy needs to be obtained in the light
of community concerns about transfusion
5 Anaemia that does not pose a hypoxic or metabolic risk to the asymptomatic child safety, particularly the potential for infec-
may not require transfusion; current guidelines use a Hb threshold <70 g L
1 in
tion transmission. The indication, risks, ben-
critically ill children, although well children without symptoms and/or where
efits, alternatives to transfusion, parental
alternative therapy is available (e.g. erythropoietin) may tolerate lower levels.
consent, response to treatment and any
adverse event should be clearly documen-
ted. In non-urgent situations, parents and
mature children can access publications
Blood components should only be given such as Children receiving a blood transfu-
Introduction when the expected benefits to the child sion: A parents guide2 for more information.
Successful and safe transfusion practice outweigh the potential hazards. A range of Clinical guidelines for use of red blood cells
depends on administering a quality clinical signs and symptoms viewed within (RBC) in children are consensus rather than
blood component of the right type, in the the context of a clinical need is essential evidence based,3 with the latest National
right amount, in the right way, at the for the decision to transfuse. Transfusion Health and Medical Research Council/
right time to the right patient.1 triggers include both clinical (symptoms, Australian Society of Blood Transfusion

259
11.1 THE USE OF BLOOD PRODUCTS IN CHILDREN

Australian practice guidelines for blood 50 mL) with a haemoglobin concentration required for elective top-up transfusion in
product indication and administration of 100150 g L1. PRBC is indicated if the mL is:
available at www.nhmrc.gov.au, including oxygen-carrying capacity of blood is so
Weight kg  haemoglobin

rise
haemoglobin (Hb) and platelet transfusion reduced that the degree of anaemia poses
required gL
1  3
threshold triggers.1 It is recommended a risk to the child or there is ongoing blood
that intravenous (IV) access be 2224G or loss. Transfusion of PRBC in an asymptom-
larger for children receiving blood products atic child is not appropriate in most situa- Alternatives
through a standard blood administration tions. PRBC transfusion is likely to be Autologous blood (patients own blood is
set primed with normal saline or the appropriate when haemoglobin is less than collected before surgery) is suitable for elec-
blood component. A blood warmer is indi- 70 g L1 in critically ill children.5 The hae- tive surgery but has no role in urgent situa-
cated at flow rates >15 ml kg
1 hr
1 in moglobin threshold remains uncertain in tions. Perioperative blood scavenging may
children and for exchange transfusion stable children with anaemia. Use of PRBC be useful in reducing exposure to donor
in infants; very slow rates are recommen- with haemoglobin in the range 70100 g L1 blood. Red cell substitutes such as cell-free
ded in small children if rapid volume is appropriate if the child is at risk of haemoglobins and perfluorocarbon emul-
expansion is not required. Blood products hypoxia (cardiac, respiratory disease) and sions require 100% oxygen to be effective
should not be warmed to above 41 C.4 should be supported by the need to relieve and are still being trialled.
The rate of administration should not be clinical symptoms and signs. Criteria for PRBC
>5 mL min
1 in the first 15 minutes as in patients aged less than 4 months are
Adverse reactions
severe reactions are most likely to occur then; different from those for older children. Infants
Fatal acute haemolytic reactions occur in
all blood components should be infused in the former group have smaller blood
1:250 0001:1 000 000 transfusions, with
within 4 hours unless fluid overload is a risk. volumes, decreased erythropoietin production
half caused by ABO incompatibility as
The child and infusion need to be monitored (especially if premature) and there may be
a result of mismatching the blood with
during blood product administration, more physiological anaemia of infancy.
the patient or other administrative errors.
closely if unconscious or anaesthetised. Additional indications for PRBC include:
Another 1:260 000 patients have a haemoly-
A severe reaction requires suspension
emergency surgery in a child with tic reaction due to minor red-cell antigen
of blood product administration pending
significant preoperative anaemia; incompatibility and 1:1000 patients experi-
further incompatibility or bacterial contami-
anaemia related to chemotherapy or ence delayed haemolytic reactions.
nation checks, consideration of antihis-
radiotherapy;
tamines/steroids, and be reported to the
Australian Incident Monitoring System in
sickle cell disease with ischaemic organ
events to suppress endogenous Hb
Australia or the New Zealand Blood Service.
production;
Platelets
Chronic congenital or acquired anaemia Indications
such as B-thalassaemia. Platelets are appropriate in bleeding chil-
Whole blood dren in whom thrombocytopenia is a major
contributory factor. In haemorrhagic shock,
Whole blood is rarely used, being specifi- Administration
platelet infusion should be considered in
cally used for massive blood loss requiring If stored in optimal conditions, RBC have a
massive transfusion associated with platelet
simultaneous restoration of oxygen-carrying shelf life of 3542 days. In haemorrhagic
count less than 50  109 L1. Platelet trans-
capacity and blood volume, haemorrhage shock, RBC infusion at an initial volume of
fusion may be appropriate in children with
with uncontrolled coagulopathy and ex- 1020 mL kg1 should be considered when
failure of platelet production and platelet
change transfusion. Eight ml kg
1 will incr- loss of blood volume approaches 30% (when
count less than 10  109 L1, as there is a
ease a childs haemoglobin concentration hypotension first appears)3 and shock is
risk of intracerebral bleeding. Platelets
by 10 g/dL
1, and administration must be refractory to non-blood fluid resuscitation.
may be given prophylactically in children
completed within 4 hours to avoid bacterial A single Hb level is not reliable in acute
undergoing invasive procedures or surgery,
contamination. haemorrhage.3 If haemorrhage or haemo-
to maintain platelet count >50  109 L1.
lysis is accompanied by life-threatening
In children with platelet dysfunction (inher-
hypoxia or rapid Hb decline then uncross-
ited or acquired), platelets are administered
matched group O rhesus negative packed
Packed red blood cells to treat active bleeding. Platelets are
cells may be required. In less urgent cases,
(PRBC) indicated in thrombocytopenic premature
type-specific or cross-matched RBC is pre-
infants with active bleeding or prior to an
Indications ferred. Rh type specific blood takes 15 min-
invasive procedure.
The childs Hb level, patient factors, signs utes to cross match. If the child has no
and symptoms of hypoxia, ongoing blood immediate need for RBC replacement and
loss, risk of anaemia and risk of transfusion there is no ongoing bleeding or haemody- Administration
should be considered. Each paediatric red namic instability, cross-matched RBC may Each paediatric unit is 4070 mL and
cell unit is 25100 mL (mean volume be administered over 4 hours. The volume contains at least 5.5  1010 platelets.

260
11.1 THE USE OF BLOOD PRODUCTS IN CHILDREN
11
Infusion of 510 mL kg1 of platelets

HAEMATOLOGY AND ONCOLOGY

for each mL of FFP. FFP is administered at volume paracentesis, severe burns after
will lead to a rise in platelet count of an initial dose of 1020 mL kg1 from a the first 24 hours and plasma exchange.
50100  109 L1. 50 mL paediatric bag. This dose raises coag- There is no evidence for albumin use as
ulation factor level by 20% immediately.3 a nutritional supplement or for the treat-
Adverse reactions and other After thawing, FFP needs to be used imme- ment of ascites or oedema related to portal
problems diately or stored at 26 C for up to 24 hours. hypertension.
Platelets are stored at room temperature
to maintain their ability to aggregate, and Administration
this promotes bacterial growth. Compared Cryoprecipitate Albumin 4% (40 g L1) at 1020 mL kg1
with other blood components, the risk of is used for volume expansion. Albumin
Cryoprecipitate is appropriate to use when
bacterial contamination of platelets is rela- 20% (200 g L1) is used for plasma albumin
clinical or potential bleeding (invasive
tively high at 1:2000 units and sepsis is repletion on a gram-for-gram basis.
procedure, trauma or DIC) is attributable
the most frequent severe complication of
to fibrinogen deficiency.3 It contains factors
platelet use. Platelets older than 5 days lose Adverse effects and risks
VIII, XIII, fibrinogen, von Willebrands factor
their ability to aggregate and are therefore There is evidence that albumin is associated
and fibronectin. Approximately 200 units of
less effective. Alloimmunisation due to with increased mortality and morbidity in
each of these factors are contained in a
the development of platelet alloantibodies critically-ill adults,6 but no such evidence
15 mL bag. This allows more rapid adminis-
from repeated transfusions may lead to in young children. Complications of albumin
tration than FFP, whilst reducing the risk
refractory thrombocytopenia. use include circulatory and sodium overload,
of fluid overload. In infants, a dose of
with the relative risk of viral disease trans-
1015 mL is sufficient to achieve haemo-
mission being less compared with cellular
stasis. Cryoprecipitate is not used to treat
Fresh frozen plasma (FFP) von Willebrands disease, haemophilia and
blood components.

FFP contains all coagulation factors, except deficiencies of factor XIII or fibronectin.
VII, including approximately 150 units of
factor VIII. FFP is used to treat bleeding
Normal human
related to coagulopathy associated with Clotting factor immunoglobulin (NIGH)
cardiac surgery or massive transfusion for concentrates Human immunoglobulin affords passive
haemorrhagic shock when 50% or more of immunity against many infectious agents
Bleeding or thrombosis in children with
circulating blood volume has been replaced. for several weeks. NIGH is derived by Cohn
proven coagulation or antithrombotic factor
FFP may be considered for treatment of fractionation from pooled plasma and
deficiency may require specific factor replace-
acute disseminated intravascular coagu- contains antibodies to viruses that are prev-
ment. These factors have become safer with
lation (DIC) and thrombotic thrombocytope- alent in the general population. Subclasses
the advent of synthesis using recombinant
nic purpura (TTP). Less urgent indications are present in the approximate proportions
genetic technology. Recombinant factor VIII
include children with coagulopathy under- G1 60%, G2 30%, G3 6% and G4 4% but
concentrate is used to treat haemophilia
going invasive procedures/surgery, liver there is no such thing as generic IgG. The
A whereas recombinant factor IX is the
disease-related coagulopathy, replacement new purified commercial products are
treatment of choice for haemophilia B.
of single coagulation factor deficiency where all a little different, with different minor
Antithrombin III, protein S and protein
a specific factor concentrate is not available, reaction rates. NIGH is administered IM for
C concentrates are used to treat patients with
and reversal of life-threatening bleeding pre- or post-exposure prophylaxis against
these specific factor deficiencies.
due to warfarin. FFP should not be used measles, varicella zoster and hepatitis A.
for volume expansion, plasma-exchange IV NIGH (either Intragam or Sandoglobulin)
procedures or treatment of immunodefi- is used to treat idiopathic thrombocytopenic
ciency states. Specific recombinant coagula- Albumin
purpura (ITP), Kawasakis disease, perinatal
tion factors rather than FFP are used to treat Indications acquired immune deficiency syndrome,
specific inherited coagulation disorders. Albumin is derived from volunteer human GuillainBarre syndrome, demyelinating dis-
plasma pools and is indicated for rapid ease, immunological disorders associated
Administration volume expansion in children with evidence with antibody deficiency (such as primary
In life-threatening exsanguination requiring of shock or poor perfusion. In paediatric hypogammaglobulinaemia) and sepsis with
massive transfusion, FFP may be required to emergency practice, albumin may be used secondary immunodeficiency. Allergic reac-
minimise dilutional coagulopathy. One unit in the initial fluid resuscitation for hypo- tions and, rarely, anaphylaxis may occur.
of FFP is required for every four units of volaemic shock, although it is no better NIGH is contraindicated in children with
packed cells but titrate FFP use against than crystalloids or other colloids in this selective IgA deficiency and evidence for
the coagulation profile if possible. FFP setting in adults.3 Other indications in- its role in the treatment and prevention of
contains all coagulation factors including clude the treatment of hypoproteinaemia, neonatal sepsis is conflicting. Immune
approximately one unit of factors VIII and V diuretic-resistant nephrotic syndrome, large response to live virus vaccines (with the

261
11.1 THE USE OF BLOOD PRODUCTS IN CHILDREN

exception of yellow fever vaccine) may be and followed up as a quality assurance Transfusion-mediated
inhibited by NIGH given 3 weeks before or activity with appropriate adjustments made immunomodulation
3 months after immunisation. to hospital transfusion protocols to reduce Allosensitisation, disturbed immunomodu-
risk of recurrence. lation (both due to contamination by
donor leucocytes) as well as infection
Hyperimmune transmission may be reduced by leucocy-
immunoglobulins (IG) Infections todepletion, psoralens and UV irradiation
Even though blood components are the of blood components and using non-
Maternal administration of Rhesus (Rh) Ig
safest they have ever been, infection trans- pooled blood components from a single
prevents the development of Rh antibodies
mission risk remains emotive and highly donor. Immunomodulation is related to
in a Rh-negative mother who gives birth
publicised, especially for human immunode- decreased cell-mediated immunity, with
to a Rh-positive baby. This reduces the risk
ficiency virus (HIV).7 Infection acquired from increased risk of reactivated viral infec-
of maternal Rh sensitisation and maternal-
a blood component is a rare occurrence tion, solid tumour recurrence and post-
neonatal Rh-incompatibility in subsequent
when compared with non-infectious compli- operative sepsis.8
pregnancies. Cytomegalovirus (CMV) Ig is
cations. The estimated risks per actual blood
used in the prevention and treatment of
unit transfused are 1:200 0001:2 000 000
CMV in children at risk of adverse sequelae Transfusion-associated graft-
for HIV, 1:30 0001:250 000 for hepatitis B
from CMV infection, such as with immuno- versus-host disease (TA-GVHD)
and 1:30 0001:150 000 for hepatitis C.
deficiency and following renal and bone- This occurs as a result of transfused lym-
Disease transmission occurs primarily during
marrow transplantation. Zoster Ig (ZIG) is phocytes engrafting and proliferating in
the window period when a blood donor is
advisable within 72 hours after varicella the transfusion recipient. TA-GVHD is
infectious and the infection is immunologi-
exposure in children with cellular immune fatal in 90% of cases because of induc-
cally silent and therefore undetectable on
deficiency, immunosuppression, neonates tion of marrow hypoplasia. Irradiation of
screening tests. Other infectious agents
whose mothers are susceptible to primary cellular blood components is effective
encountered include hepatitis A and G,
varicella infection and infants who are less prevention.
human T lymphotropic virus 1 (HTLV I)
than 28 weeks gestation at birth. Infants
and II, parvovirus B19, syphilis, malaria,
born to HbsAg-positive mothers should
babesiosis, Salmonella and Trypanosoma
receive hepatitis B Ig and commence pri- Other transfusion-related
cruzi. There is no current evidence to sug-
mary hepatitis B vaccination within 12 hours adverse reactions
gest that variant CreutzfeldtJakob disease
of birth. Respiratory syncytial virus (RSV) These include donor-recipient incompati-
can be transmitted by blood transfusion.
Ig is used prophylactically in infants at bility, allergic reactions, anaphylaxis or
Donor selection and exclusion, donated
high risk of severe RSV infection, including anaphylactoid reactions, circulatory over-
blood screening, post-collection leucodeple-
those with bronchopulmonary dysplasia. load, haemolysis (acute/chronic, intra-/
tion and viral inactivation help to minimise
Tetanus Ig passively protects non-tetanus extravascular, associated fever/no fever),
risk of infection transmission. Using pooled
immune children who sustain a tetanus- complications associated with massive trans-
blood components allows contamination of
prone wound and a tetanus Ig infusion is fusion (hypothermia, dilutional coagulopathy
the entire pool from one infectious donor
used to treat clinical disease. and thrombocytopenia) and post-transfusion
and is therefore less safe than components
derived from a single donor. Bacterial con- thrombocytopenia.7
tamination of blood components, either
Risks of blood component
from prolonged/faulty storage or acquired
use Controversies and future
from the donor, is most frequently due
directions
These are categorised as acute or delayed, to occult donor bacteraemia and donor
haemolytic or non-haemolytic, allergy-based skin organisms. Yersinia enterocolitica and The threshold for red cell transfusion
or not. Mild non-haemolytic febrile reactions other Gram negatives are most frequently at which benefits outweigh risk in stable
can occur with any blood component implicated. children and non-critically ill adults
and need to be distinguished from major remains unclear.
reactions. If a major reaction occurs, the
transfusion is discontinued and threats to Transfusion-related acute lung
The role and potential harm of albumin
in adult resuscitation has not been
the airway, breathing and circulation are injury (TRALI)
investigated in paediatric resuscitation
attended to. Specific treatment for anaphy- Acute respiratory distress syndrome with
and the role of immunoglobulins in severe
laxis, sepsis and blood group incompatibility non-cardiogenic pulmonary oedema occurs
sepsis remains uncertain.
may be required, in consultation with a within 4 hours in 1:5000 transfusions, with
haematologist. Recipient and donor blood 90% of patients recovering. This reaction  Oxygen-carrying red cell substitutes
samples are sent for immunological and may be difficult to distinguish from fluid such as modified haemoglobins and
microbiological testing. Critical adverse inci- overload and is related to immune-mediated perfluorocarbon emulsions may offer the
dents should be notified to the blood bank increased pulmonary capillary permeability. same benefits as red blood cells without

262
11.2 ANAEMIA
11

HAEMATOLOGY AND ONCOLOGY

3. Robitaille N, Hume HA. Blood products and fractionated
the infectiontransmission risk, but are plasma products: preparation, indications and
Further reading
administration. In: Arceci RJ, Hann IM, Smith OP, editors.
not in clinical use at present. Pediatric Hematology. 3rd ed. London: Blackwell American Association of Blood Banks. Facts about blood and

Publishing Ltd; 2006. p. 693723. blood banking. Availalble from http://www.aabb.org; 2003
The role of non-blood alternatives 4. National Health and Medical Research Council/Australian [accessed 19.10.10].
such as autologous blood donation, Society of Blood Transfusion. Guidelines for appropriate Behrman RE, Kleigman RM. Intercontinental childhood ITP
use of blood and blood components/Guidelines for the registry. In: Nelson essentials of pediatrics. 4th ed.
perioperative blood salvage and directed administration of blood components. 2004. Available from: Philadelphia: WB Saunders; 2002.
parental transfusion is not clearly www.nhmrc.gov.au; and www.anzsbt.org.au [accessed Christensen RD. Hematologic problems of the neonate.
19.10.10]. Philadelphia: WB Saunders Company; 2000.
defined in children. 5. Canadian Critical Care Trials Group; Pediatric Acute Lung Goodnough LT, Brecher ME, Kanter MH, AuBuchon JP.
Injury and Sepsis Investigators Network. Transfusion Transfusion medicine. Blood transfusion. N Engl J Med
strategies for patients in pediatric intensive care units. 1999;340:43847.
New Engl J Med 2007;356:160919. Kruskall MS. The perils of platelet transfusions. N Engl J Med
6. SAFE Study Investigators, Finfer S, Bellomo R, 1997;337:19145.
McEvoy S, et al. Effect of baseline serum albumin Miller DR, Bachner RL. Blood diseases of infancy and
concentration on outcome of resuscitation with childhood. In the tradition of Carl H Smith. Mosby-Year
References albumin or saline in patients in intensive care units: Book. 7th ed. St Louis, MO: Mosby; 1995.
1. National Health and Medical Research Council/ analysis of data from the saline versus albumin fluid Regan F, Taylor C. Recent developments. Blood transfusion
Australian Society of Blood Transfusion. Topics in evaluation (SAFE) study. BMJ 2006;333(7577):1044. medicine. Br Med J 2002;325:1437.
Transfusion Medicine. Available from: Epub 2006 Oct 13. Sadowitz PD, Ammanullah S, Souid AK, et al. Hematologic
http://www.anzsbt.org.au/publications/TTM.cfm 7. Luban NLC, Wong ECC. Hazards of transfusion. In: emergencies in the pediatric ER. Emerg Med Clin N Am
[accessed 19.10.10]. Arceci RJ, Hann IM, Smith OP, editors. Pediatric 2002;20:17788, vii.
2. National Health and Medical Research Council/ Hematology. 3rd ed. London: Blackwell Publishing Ltd.; Smith H 1996 Diagnosis in paediatric haematology. New York:
Australian Society of Blood Transfusion. Children 2006. p. 72444. Churchill Livingstone.
receiving a blood transfusion: A parents guide. Available 8. Goodnough LT, Shander A, Brecher RE. Transfusion Stainsby D, Jones H, Asher D, et al. Serious hazards of
from:www.nhmrc.gov.au; and www.anzsbt.org.au [accessed medicine: Looking to the future. Lancet 2003;361: transfusion: a decade of hemovigilance in the UK. Transfus
19.10.10]. 1619. Med Rev 2006;20(4):27382.

11.2 Anaemia
Jane Cocks

ESSENTIALS Introduction
1 The normal haemoglobin concentration and type is age and sex dependent. Anaemia is defined as a reduction in the
red blood cell (RBC) volume or haemo-
2 Clinical effects of anaemia depend on the timing of its development, the globin concentration below normal values.
magnitude of the anaemia and the underlying cause. The normal haemoglobin level is age and
3 Iron-deficiency anaemia is the commonest cause of microcytic and hypochromic sex dependent, and racial differences
anaemia. exist. At birth, the haemoglobin level is
159191 g L1 and this falls to a trough
4 Autoimmune haemolytic anaemia (AIHA) is commonly caused by a variety of level of 90114 g L1 between 8 and 12
infective agents, although it can be triggered by a number of medications and weeks of age before rising again toward
systemic illnesses. normal adult range.
5 Non-immune haemolytic anaemia can be due to infective agents, medications Red blood cell production is regulated
and chemical exposures. by erythropoietin, a hormone produced
initially in the fetal liver and, after birth,
6 Haemolytic uraemic syndrome (HUS) is the commonest cause of acute renal in the renal peritubular cells. In normal
failure in children, most frequently secondary to infection with E. coli. erythropoiesis, erythropoietin stimulates
7 Thrombotic thrombocytopenic purpura affects children over the age of 10 years. differentiation of marrow stem cells into
The clinical features are similar to HUS with the addition of neurological deficit. red blood cells. During this process there
is a condensation of the cells nuclear mate-
8 G6PD deficiency is an X-linked enzymatic inborn error in which haemolysis is rial with continual production of the hae-
triggered by exposure to an oxidant. moglobin until it comprises 90% of the
9 Thalassaemia is a relatively common inherited defect in the synthesis of the mass of the RBC. The nucleus is then
globin chain. extruded, leaving the RBC with no synthetic
or replication ability, leading to a limited
10 Sickle cell disease is an autosomal recessive inherited condition with a globin RBC lifespan of 120 days.
chain that is unstable in the deoxygenated state, leading to haemolysis. Haemoglobin is the major functional con-
stituent of the RBC responsible for the task

263
11.2 ANAEMIA

of carrying oxygen to the tissues, with the The adequacy of ventilation should be
Table 11.2.1 Causes of childhood
RBC acting as the carrier through the cardio- anaemia ascertained and supplemental oxygen
vascular system. Haemoglobin is a complex provided. Circulatory compromise in acute
Production defect
protein consisting of iron-containing haem haemorrhage and acute haemolysis requi-
1. Primary bone marrow failure
groups and polypeptide chains, known as Aplastic anaemia (congenital or acquired) res cardiorespiratory monitoring and intra-
globin. The haemoglobin molecule is made Diamond-Blackfan syndrome venous access for fluid resuscitation (see
Transient erythroblastopenia of childhood
up of two pairs of polypeptide chains each (TEC) Chapter 2.5 on shock). Packed red blood
coupled with a haem group. Chemical varia- 2. Erythropoietin production problem cells are used if haemorrhage or haemolysis
Chronic renal disease
tions in the polypeptide chains lead to differ- Hypothyroidism is life threatening. If tissue oxygenation is
ent types of haemoglobin being produced. 3. Abnormal cytoplasmic maturation not critically affected, the circulatory volume
(microcytic anaemias)
At various stages of life, from the embryo Iron deficiency should be sustained with colloid or crystal-
to the adult, there are six different types of Sideroblastic anaemia (congenital or loid until group-specific or cross-matched
acquired)
haemoglobin normally detectable. The nor- Lead toxicity RBCs are available. If the bleeding is con-
mal adult haemoglobin, HbA, is comprised Thalassaemia syndrome trolled, with further bleeding unlikely, the
4. Megaloblastic anaemia
of two a-polypeptide chains and two Vitamin B12 deficiency signs mentioned above are stable and the
b-chains, (a2b2). Haemoglobin F, or fetal Hb Folate deficiency haemoglobin concentration is greater than
Thiamine-responsive megaloblastic
(HbF), is comprised of two a-chains and two anaemia 70 g L1, a cross-match should be performed
g-chains, (a2g2) and constitutes 70% of the Orotic aciduria (rare, associated with and the packed RBCs should be held in
megaloblastic anaemia)
haemoglobin present at birth, reducing to 5. Others reserve for 24 hours. Early consultation
trace levels by 612 months of age. Minor Primary dyserythropoietic anaemias with a haematologist is important in acute
Erythropoietic protoporphyria
amounts of HbA2 (a2d2) are present at all Marrow infiltration, e.g. malignancy or haemolytic anaemia especially if the under-
ages. Pathological variations in the poly- myelofibrosis lying cause has not been diagnosed.
peptide chains can produce disease states, Decreased RBC survival Anaemia is a clinical finding rather than a
known as haemoglobinopathies (see below). 1. Hereditary haemolytic anaemias disease in its own right and symptoms
a. Haemoglobin variants
Anaemia is not a single disease, but may Thalassaemias
depend on the timing of its development,
occur as a result of a variety of pathological Sickle cell disease the severity and the underlying cause. The
b. Abnormal RBC membranes
processes. The anaemias of childhood can be Hereditary spherocytosis
presentation is frequently not specific to
usefully divided into two groups: (1) those Hereditary elliptocytosis anaemia. Common presenting features con-
Hereditary stomatocytosis
caused by inadequate production of RBC c. Abnormal enzymes
sistent with insidious onset anaemia include:
or haemoglobin; and (2) those due to Glucose-6-phosphatase
increased destruction or loss of RBC. dehydrogenase deficiency (G6PD) lethargy, irritability, sleep or settling
Pyruvate kinase deficiency difficulty;
Tables 11.2.1 and 11.2.2 outline the major 2. Acquired haemolytic anaemias
causes of childhood and neonatal anaemias. a. Autoimmune haemolytic anaemia (AIHA) pallor or yellow skin hue;
b. Non-immune haemolytic anaemia
c. Fragmentation haemolysis
loss of appetite  weight loss  pica;
Haemolytic uraemic syndrome (HUS) bruising, bleeding or infection.
3. Haemorrhage
Traumatic Patient factors to note are age, ethnicity and
Overview Non-traumatic acute or chronic
the presence of other significant disease.
The principles of management of anaemia Iron-deficiency anaemia is uncommon in
in the emergency department (ED) are: the first 6 months in term infants and sickle
cell disease (SCD) is unlikely to present
Resuscitate the patient with circulatory
as anaemia under the age of 4 months. Hae-
collapse.
Table 11.2.2 Causes of anaemia in the molytic uraemic syndrome (HUS) mainly
Remove or treat the precipitant if neonate
affects those under 4 years of age, whereas
known. 1. Physiological anaemia thrombotic thrombocytopenic purpura (TTP)
 Treat the cause if treatment can be 2. Abnormal RBC production
affects children from 10 years of age. Iron defi-
a. Nutritional deficiency
initiated in the ED. b. Secondary to infection ciency is common in adolescent females,
3. Decreased RBC survival blood loss
Causes of life-threatening anaemia include a. Overt blood loss whereas G6PD deficiency occurs mainly in
uncontrolled haemorrhage, acute intravas- Iatrogenic during delivery males. The association between ethnicity
Obstetric accidents
cular haemolysis in glucose-6-phosphatase b. Occult blood loss and various types of hereditary anaemia is
deficiency (G6PD) or autoimmune haemoly- Twin-to-twin transfusion shown in Table 11.2.3. Family history of anae-
Fetoplacental haemorrhage
tic anaemia (AIHA), sequestration crisis in Fetomaternal haemorrhage mia may also suggest hereditary anaemia.
sickle cell disease (SCD) and acute decom- 4. Decreased RBC survival haemolysis Dietary history can provide important
a. Immune-related: isoimmunisation
pensation in chronic anaemia. b. Infection-related clues. Iron-deficiency anaemia is likely in
Initial assessment is directed at respira- c. Hereditary haemoglobin variants, prolonged breast-feeding beyond 6 months
enzyme abnormalities, membrane
tion and circulation. The anaemic patient abnormalities of age in term infants and sooner in preterm
may be tachypnoeic with tissue hypoxia. infants if no iron supplementation is

264
11.2 ANAEMIA
11

HAEMATOLOGY AND ONCOLOGY

(MCV) may suggest an underlying cause.
Table 11.2.3 Ethnicity and anaemia
The upper limit of normal in adults is NEONATAL ANAEMIA
1. Thalassaemia syndromes
a-Thalassaemia descendants from
96 fL but MCV in children is less than in
China, Malaysia, Indonesia and Africa adults and average values may be estimated In the term neonate, the haemoglobin at
b-Thalassaemia descendants from birth is in the range of 159191 g L1 and
Africa, Middle East, Mediterranean, India,
by adding 0.6 fL per year of age to 84 fl. The
Pakistan, China reticulocyte count is expressed as a percent- rises in the first 24 hours. Subsequently it falls
2. Sickle cell disease
age of the circulating RBC mass with a nor- to a trough level of 90114 g L1 between
Descendants from Africa, southern Arabia,
Turkey, Greece, central India mal range of 0.51.5%. A low reticulocyte 8 and 12 weeks of age. This decline is gener-
3. Hereditary spherocytosis ally referred to as physiological anaemia of
Descendants from northern Europe
count is found in abnormalities of RBC pro-
4. G6PD deficiency duction and a high reticulocyte count is infancy, although it is an expected normal
Descendants from Africa, China, south- occurrence. The falling haemoglobin is due
east Asia and the Mediterranean
found in increased destruction or from RBC
loss, provided there is no concurrent bone- to a combination of rapid weight gain and
marrow pathology. Morphological features blood volume expansion leading to relative
of RBC may be diagnostic of the underlying haemodilution in the first 3 months of life,
cause as shown in Table 11.2.4. combined with a comparatively shorter life
provided, if there is excessive dependence on There are many additional investigations span of the fetal RBC and a sharp decline in
cows milk as a food source or if food sources apart from full blood count to assist in iden- erythropoiesis a few days after birth due to
are restricted, e.g. food fads. Ingestion of clay tification of the underlying cause. Some that increased arterial oxygenation and a reduc-
and dirt may cause lead toxicity and iron are undertaken in the ED include: tion in the production of erythropoietin.
deficiency. Ingestion of fresh uncooked Following the nadir in Hb, erythropoiesis
broad beans is a potent precipitant of
bilirubin elevated unconjugated recommences and the Hb rises again.
bilirubin suggests haemolysis;
haemolysis in G6PD deficiency, along with
medications.
urea and creatinine to detect renal failure Immune-related
in HUS and TTP;
Recent infections may precipitate AIHA, Immune-related haemolysis is the common-
non-immune haemolytic anaemia, HUS
iron studies (serum iron, total iron binding
est cause of anaemia in the neonate. Mater-
capacity, transferrin saturation);
and TTP. Hyperbilirubinaemia caused by nal antibodies pass through the placenta
haemolysis leads to jaundice, cholelithiasis,
Coombs tests to identify immune-related
leading to fetal cell lysis. Haemolysis occurs
haemolysis;
cholecystitis and dark urine. Mechanical when the target cells are the fetal RBCs. The
destruction of RBCs is caused by vascular
haemoglobin electrophoresis.
commonest antigen responsible is the Rhe-
malformations, shunts, abnormal native or Identification and treatment of the underly- sus antigen on the RBC surface, followed
prosthetic cardiac valves. Septic shock, ing precipitant, where appropriate and pos- by ABO incompatibility. Sensitisation of
bleeding disorders, chronic renal disease, sible, is a crucial part of the management of the mother occurs when Rhesus incompati-
severe burns and hypothyroidism may all anaemia in the ED. Packed RBCs are indi- ble fetal cells gain access to the maternal cir-
cause anaemia. cated in the treatment of severe anaemia culation. Fetal RBCs can be found from
The salient physical findings in the con- with cardiovascular compromise. A dose of 8 weeks gestation onwards. As little as
text of anaemia relate to general appear- 4 mL kg1 of packed RBCs raises haemoglo- 0.050.10 mL is enough to cause primary
ance, cardiovascular status and abdominal bin concentration by 10 g L1. This dose can sensitisation but the volume and risk
examination. Frontal bone expansion and be given hourly or more slowly in the presence increases with obstetric procedures, miscar-
frontal bossing is due to expansion of the of cardiac failure. Furosemide 1 mg kg1 may riages and toxaemia. Fetal harm occurs dur-
medullary spaces and may be seen in severe be given if there is evidence of volume over- ing subsequent pregnancies with a Rhesus
thalassaemias. Some hereditary anaemias load. Involvement of a paediatric haematolo- positive fetus in a Rhesus negative mother.
may also have associated physical abnor- gist is recommended for all but the simple The resultant intravascular haemolysis in
malities (e.g. DiamondBlackfan anaemia). nutritional anaemias. the fetus can be devastating. The clinical
Hepatosplenomegaly is a feature of some significance is due both to the severity of
of the hereditary haemolytic anaemias. the anaemia and kernicterus from the hyper-
Table 11.2.4 Morphological features of
Purpuric or petechial rashes are due to coag- RBCs to assist the diagnosis of anaemia bilirubinaemia. ABO incompatibility pro-
ulation disorders or vasculitis. Dark urine Schistocytes Erythrocyte
duces less severe jaundice and anaemia
from bilirubinuria is consistent with haemo- fragmentation during the second week of life.
syndromes
lysis. Haematuria must be confirmed by Prevention relies on identification of the
microscopy to distinguish haemoglobinuria Sickle cells Sickle cell disease Rhesus status of all pregnant women. This
from haematuria. Spherocytes Immune-meditated
is an essential task for any doctor involved
Anaemia is confirmed by the lowered haemolytic anaemia in antenatal care. Prophylaxis therapy using
Hereditary
haemoglobin concentration and decreased spherocytosis
anti-D immunoglobulin (Ig) is given to all
RBC count. An age-related reference range Rhesus negative women with per vaginal
Blister or bite cells with G6P deficiency
must be used. Abnormalities in the other cell poikilocytosis
bleeding or requiring any obstetric proce-
lines should be noted. The mean cell volume dures that may lead to fetomaternal

265
11.2 ANAEMIA

haemorrhage. The recommended dose is K and, in the breast-fed baby, maternal finding. Breast milk has easily absorbable iron
250 IU if the sensitising event occurs in medications such as warfarin, phenytoin, in modest amounts. The quantity is inade-
the first trimester and 625 IU beyond this rifampicin and isoniazid, may compound quate for the rapid growth in the second
gestation period. This passive immunisation the deficiency. Nutritional vitamin K defi- 6 months. Cows milk has minimal iron con-
is given intramuscularly. If required in the ciency has been associated with prolonged tent, which exists in a poorly absorbable
postpartum period the immunoglobulin is breast-feeding without supplementation, form and is not recommended under the age
administered intravenously. malabsorption, chronic diarrhoea, and pro- of 12 months. Cows milk can cause gastroin-
longed use of oral antibiotics. Vitamin testinal bleeding in the immature gut and
Neonatal infection K prophylaxis is normally given at birth. Hae- children who have an excess intake of cows
Infections cause anaemia by producing mophilia can rarely lead to spontaneous milk have a lower appetite for other foods.
bone-marrow suppression of erythropoiesis haemorrhage in the newborn. More than two servings of milk per day is
or by haemolysis. The infections of relevance not advisable. Iron-fortified cereals should be
in the neonate are cytomegalovirus (CMV), introduced from 5 months of age.
rubella, toxoplasmosis and congenital syphi- ANAEMIAS OF
lis. The human parvovirus may lead to spon- CHILDHOOD Adverse effects
taneous miscarriage in early pregnancy and, Iron deficiency before 24 months can lead
in late pregnancy, can selectively depress to impaired development of cognitive and
erythropoiesis, producing severe fetal anae- Iron-deficiency psychomotor skills, which may be only par-
mia. In the older child, parvovirus is the cause tially reversible when the deficiency, and the
Iron deficiency is uncommon in the first 6
of Erythema Infectiosum (Fifth Disease), associated anaemia, is corrected. Many par-
months in the term infant due to the effi-
which can be complicated by aplastic crisis ents report loss of appetite, irritability and
cient recycling of the iron from the haem
or chronic haemolysis. Vertical transmission inability to concentrate in affected children.
component of haemoglobin when it is meta-
of malaria to the fetus is a major cause of Pica may be present. Iron deficiency affects
bolised. In the preterm and low-birth-weight
anaemia in the neonate in endemic regions. neutrophil and lymphocyte function in vitro
neonate, iron deficiency occurs earlier, often
Human immunodeficiency virus (HIV) infec- and is indicated as one of the investigations
at the time of doubling of the birth weight.
tion in the neonate is predominantly of recurrent infections.
In the second 6 months of life, iron deficiency
acquired via perinatal transmission from
is found in 20% of Australian children, with
the mother. The transmission rate is in the Diagnosis
associated anaemia in 3%. The respective
range of 2040%. The mechanism for Iron deficiency is the commonest cause of
figures in the second year are 35% and
subsequent anaemia is bone-marrow sup- microcytic hypochromic anaemia and is
9%. It is even more common in indigenous
pression and associated pancytopenia as characterised by a low serum iron, a high
children and children from non-English-
well as chronic multisystem disease. total iron binding capacity and a low trans-
speaking backgrounds. In school-age chil-
ferrin saturation. The reticulocyte count is
dren, iron deficiency is present in 12%
Haemoglobinopathies less than 2% unless iron had been reintro-
but is found in up to 9% of adolescent girls.
and enzymopathies duced into the diet. The diagnosis is not
G6PD deficiency is an important global confirmed until iron supplementation is fol-
cause of neonatal jaundice. Haemolysis from Stages lowed by a reticulocytosis within 12 weeks
other enzymopathies and haemoglobin var- The first stage of iron deficiency is a low and a rise in the haemoglobin over 24
iants, such as thalassaemias and SCD, is usu- serum ferritin level. Iron-dependent body weeks. Other conditions producing micro-
ally unmasked beyond the neonatal period functions, including erythropoiesis, are affec- cytic anaemia must be considered if there
when the proportion of fetal haemoglobin ted in stage two. Microcytes and an elevated is poor response to iron supplementation.
falls. It is important to consider congenital erythrocyte protoporphyrin level are detect-
causes of anaemia in children with a family able and the serum iron falls. The third stage Treatment
history of hereditary anaemia or persisting is the presence of a microcytic hypochromic Dietary advice and education are the basis of
neonatal jaundice. anaemia. prevention and treatment in developed
countries. If iron supplementation is required,
Blood loss Causes the ferrous form is more effectively absorbed
Blood loss may arise from twin-to-twin The commonest cause globally is insufficient either as syrup or tablets at 23 mg kg1
transfusion, or obstetric procedures, or as intake of dietary iron. In developing countries, day1. The treatment dose for severe
part of the delivery of the neonate in the the two most significant factors are the failure anaemia is up to 6 mg kg1 day1, but side
perinatal period. Haemorrhage from an un- to obtain iron-rich nutrients and malab- effects of nausea, vomiting, and constipation
derlying haematological disorder must be sorption from intestinal pathology. The are dose-related. Transfusion is only ever
considered. Vitamin K deficiency is normally aetiology is different in developed countries, required if there is cardiovascular com-
present at birth but only a minority of such as Australia, where prolonged breast- promise. Parents should be informed regard-
neonates develop generalised bleeding. feeding beyond 6 months of age without sup- ing the toxicity of iron in overdose so that
Breast milk has a small amount of vitamin plementation with iron-rich foods is a common they store the medication safely.

266
11.2 ANAEMIA
11

HAEMATOLOGY AND ONCOLOGY

peripheral blood film. Spherocytosis and the erythrocytes. They are characterised by
Haemolytic anaemias other characteristic red-cell changes are red cell fragmentation with schistocytes
RBC exist in the circulation for 100120 due to membrane loss as splenic macro- and spherocytes in the peripheral blood film
days with up to 1% of the RBC population phages attack antibody-coated red blood and features of intravascular haemolysis.
being removed by haemolysis and replaced cells. Lactose dehydrogenase, a RBC enzyme, There are two groups.
per day. Pathological haemolysis is that is raised and free haemoglobin and haem-
which occurs outside of this normal process albumin may be found in the plasma. Macroangiopathic haemolytic
leading to reduced RBC survival. In response, In children the clinical pattern is most anaemia
bone marrow activity increases, leading to frequently an acute episode of severe Macroangiopathic haemolytic anaemia
an increased reticulocyte count of >2%. In anaemia with resolution within 3 months. results from RBC damage upon passage
the older age group, the bone marrows Haemoglobin drops to less than 60 g L1 through large vessels and the heart and is
capacity to increase erythropoiesis can com- in over 50%. A slower onset is more likely associated with structural cardiac abnor-
pensate for mild pathological haemolysis. In to follow a chronic and relapsing course with mality or large vessel disease (coarctation of
the infant and young child, the bone marrow guarded prognosis. the aorta). It can follow cardiac surgery but
is maximally active with minimal ability to A reticulocytosis may not be present initi- infection or failure of the prostheses must be
increase erythropoiesis and anaemia accom- ally due to rapid onset. Associated immune considered if it occurs distinct from the sur-
panies pathological haemolysis. thrombocytopenia is present in a third. gical period. It is less likely with xenografts
The haemolytic anaemias of childhood Direct Coombs antiglobulin test is positive. and also when prostheses are covered by
may be classified as hereditary or acquired Free antibodies may be detectable within endothelium. The damage is from a combina-
(Table 11.2.1). The hereditary haemolytic serum. Antibodies that are maximally active tion of shearing forces from turbulent flow
anaemias are caused by inherited traits at 37 C belong to the IgG class and are and interaction between the cells and the ab-
that lead to abnormal haemoglobin chains known as warm antibodies. Antibodies that normal surfaces. The anaemia is usually mild
(haemoglobinopathies), abnormal RBC mem- are maximally active between 0 and 30 C and severe haemolysis is uncommon. Treat-
branes or abnormal RBC enzymes (enzymopa- belong to the IgM class and are known as ment of the underlying cause is important.
thies). The acquired haemolytic anaemias cold antibodies. Serology may be useful in
may be immune or non-immune or as a result identifying the infective agent. Hyperbiliru- Microangiopathic haemolytic
of fragmentation syndromes. binaemia is present. anaemia
IgG-induced AIHA responds to steroid Microangiopathic haemolytic anaemia is due
therapy and variable regimes have been to RBC damage as they pass through diseased
effective. This is not effective in cold aggluti- or partially occluded arterioles and capillaries
Acquired haemolytic
nin disease (IgM induced), which may require and is often acute and severe, with associated
anaemias plasmapheresis. Both may respond to immu- thrombocytopenia. The commonest causes
Autoimmune haemolytic noglobulin, 1 g kg1 IV, and require are HUS under 4 years of age and TTP in
anaemia (AIHA) exchange transfusion if disease is severe. the child 10 years and older but connective
A variety of infectious agents (EpsteinBarr Splenectomy is a last resort in IgG-induced tissue disease, haemangiomas and severe
virus, CMV, mumps, mycoplasma and tuber- AIHA. A haematologist should be consulted burns can all be causes.
culosis) can cause severe anaemia in the regarding specific treatment.
younger child. The antibody involved is Haemolytic uraemic syndrome
mainly IgM, but may be IgG. Other diseases HUS is the commonest cause of acute renal
that trigger autoantibodies include systemic Non-immune haemolytic failure in children. It is classically charac-
lupus erythematosus, rheumatoid arthritis, anaemia terised by the triad of microangiopathic hae-
thyrotoxicosis, ulcerative colitis, malignancy molytic anaemia, thrombocytopenia, and
Non-immune haemolytic anaemia can also be
and immunodeficiency syndromes. Idio- uraemia. In warmer climates it may be
caused by a variety of infectious agents
pathic autoimmune anaemia can be rapidly endemic but in cooler climates it occurs
(malaria, Gram-positive and Gram-negative
progressive over days in the young infant sporadically. More than 90% of affected
organisms), medications (salicylates, sul-
and chronic and relapsing in the older child. children are under the age of 4 years, with
fasalazine, nitrofurantoin) and chemicals
Haemolysis in these conditions occurs peak incidence in children between the
(naphthalene). Clinical features are similar to
within the intravascular space. The Coombs ages of 4 months and 2 years. Numerous
AIHA but Coombs test is negative. Treatment
test in these conditions is positive. This test infections can trigger HUS including Shi-
is supportive, focusing on the identification
detects a coating of immunoglobulins or gella, Salmonella, Yersinia, Campylobacter,
and treatment of the underlying cause.
components of complement on the RBC sur- Streptococcus pneumoniae, echovirus, Cox-
face. During haemolysis, free haemoglobin is sackie virus A and B, and varicella. The
liberated in plasma and combines with hap- Erythrocyte fragmentation commonest organism is Escherichia coli
toglobin resulting in decreased serum hapto- syndromes and verocytotoxin is the toxic agent. HUS
globin. RBC fragmentation, spherocytes and These are haemolytic anaemias due to may also develop in association with
sometimes tear-shaped cells are found on the direct physical or mechanical damage to certain drugs, including oral contraceptives,

267
11.2 ANAEMIA

mitomycin, or cyclosporin, and in diseases Transfusion of packed RBCs is rarely needed

with significant endothelial cell injury such for severe anaemia. Involvement of a Thalassaemias
as systemic lupus erythematosus. A famil- paediatric haematologist is mandatory. Thalassaemia is one of the commonest
ial inherited version has been reported, inherited haemoglobin variants. Defective
although these occurrences are usually synthesis of globin chains leads to abnormal
not associated with diarrhoea.
Hereditary haemolytic pairing of the globin chains in the haemo-
HUS is usually (80%) preceded by an infec- globin molecule. Excess chains tend to aggre-
tion with vomiting and bloody diarrhoea.
anaemia
gate, precipitate and cause damage to the
Fever is present and oliguria occurs within Glucose-6-phosphatase RBCs. The nomenclature depicts the missing
days. Hypertension may be present. The dehydrogenase (G6PD) chain, e.g. bthalassaemia refers to a defi-
thrombocytopenia is mild and severe bleed- deficiency ciency of the b-chain. Haemoglobin electro-
ing is uncommon. Rarely, cardiomyopathy, G6PD deficiency renders the RBC vulnerable phoresis defines the haemoglobin variants.
cerebral infarcts or haemorrhage, bowel per- to haemolysis when exposed to an oxidant.
forations and diabetes may complicate HUS. G6PD is the enzyme in the pathway leading
The peripheral blood film shows the typi- to generation of reduced nicotinamide b-Thalassaemia major
cal schistocytes. A leucocytosis with left adenine dinucleotide phosphate, which Each carrier parent must donate one abnor-
shift often accompanies the anaemia and maintains glutathione in its reduced form. mal gene to the offspring and there is a
thrombocytopenia. Stool samples should Reduced glutathione is an essential factor 25% chance of a homozygote progeny with
be tested for verocytotoxin. Raised urea in the degradation of cellular peroxides that b-thalassaemia major. No b-chains are pro-
and creatinine indicate renal failure. may otherwise damage cellular proteins duced so presentation is in the first month
Treatment is directed at the acute renal including haemoglobin. The enzyme activity of life when the fetal haemoglobin, HbF
failure with early dialysis indicated in some is higher in the younger RBCs but deterio- (a2g2), is falling, with an increasing reliance
cases. Transfusion of packed RBCs may be rates as the cell ages so older RBCs are more on adult haemoglobin, HbA (a2b2). Excess
required. Platelet transfusion is not indi- prone to haemolysis. unpaired a-chains aggregate to form an
cated unless there is active bleeding. Careful G6PD is an X-linked recessive condition unstable tetramer, which is functionally use-
monitoring is essential. with wide variability of expression depending less. Haemolysis is increased and severe anae-
The long-term outcome for the acute on the biochemical type, the oxidant stress mia will occur. Extramedullary haemopoiesis
renal failure is good, with 90% survival rate and the sex of the patient. Homozygous leads to massive hepatosplenomegaly which
in the acute phase. Long-term monitoring is females are rare and the disease is may result in functional hypersplenism. Bone
essential as chronic complications may not predominantly found in males. The severity marrow expansion may result in skeletal
be apparent for several decades. Atypical of the disease varies and levels of enzyme at changes (e.g. bossing of the frontal bone).
HUS following an upper respiratory tract or above 40% of normal will rarely result in The peripheral blood film shows a microcytic
infection may manifest severe hypertension clinically significant haemolysis. A number of hypochromic anaemia with marked poikilo-
and renal failure. The prognosis for the latter different variants are found in patients of cytosis, target cells and reticulocytosis.
is poor. The disease may recur. African, Chinese and Mediterranean descent. Basophilic stippling may be seen in the RBCs.
Viral and bacterial infections can both Iron and folate deficiency may be demon-
Thrombotic thrombocytopenic cause acute haemolysis especially in chil- strated, although iron overload will eventually
purpura dren less than 2 to 3 years of age. Oxidant develop due to increased gastrointestinal
This clinical syndrome is similar to that seen drugs and chemicals that commonly cause tract absorption.
in HUS, with the addition of neurological haemolysis include naphthalene, sulphona- Treatment mainstay is regular (usually
deficit. The latter may take the form of an mides, antimalarials, nitrofurantoin, diazox- monthly) lifelong blood transfusions. Iron
altered level of consciousness ranging from ide and dapsone. Favism refers to the overload complicates this therapy and is
confusion, to seizure or coma. Cranial nerve acute haemolysis resulting from the inges- managed with regular iron chelation therapy,
palsies may also be present. The renal fail- tion of broad bean (Vicia faba) or inhalation but transfusion haemosiderosis is common,
ure is not as severe compared to HUS but of the pollen in those with the Mediterra- with its associated end-organ damage. HLA
the thrombocytopenia is more severe. TTP nean variant but not those with the African matched bone marrow transplantation has
usually affects children older than 10 years. variant. The haemolytic agent in the broad been curative.
Possible triggers include mycoplasma and bean can be passed to the infant by breast
viral infections, HIV and subacute bacterial milk. The anaemia is present within 336 b-Thalassaemia minor
endocarditis but none is evident in the hours and usually lasts for 26 days. Death Individuals with b-thalassaemia minor
majority of patients. Underlying immunolog- can occur within 24 hours of the haemolysis. receive one abnormal gene only, thus
ical disease may be relevant, as is pregnancy, Definitive diagnosis of G6PD deficiency is demonstrating the heterozygote state.
and genetic predisposition. Treatment is sup- via a quantitative assay of the enzyme. There is enough b-chain production and
portive, mainly via exchange transfusion Treatment is non-specific. Prevention is cru- normal haemoglobin to avoid symptomatic
using FFP. If there is no improvement in 24 cial, with avoidance of oxidising medications anaemia. The life span of the RBCs is only
hours then plasmapheresis is considered. and prompt treatment of infections. slightly decreased. The anaemia is microcytic

268
11.2 ANAEMIA
11

HAEMATOLOGY AND ONCOLOGY

and hypochromic with target cells Diagnosis been reported in heterozygous cases. ASS
and elliptocytes. HbA2 level in the range of Diagnosis is made on the family history and can occur suddenly with acute pallor, weak-
3.57.0% is almost diagnostic of the presentation. It is confirmed by haemoglo- ness, abdominal pain and distension with
b-thalassaemia trait. The bone marrow is bin electrophoresis and the sickle prep test features of hypovolaemic shock. The precip-
hyperplastic. Attention to potential iron outside the neonatal age group. The sickle itant is usually an intercurrent infection. The
and folate deficiency is important. The prep uses sodium metabisulphite, which key finding is circulatory shock in a child with
absence of an age-related rise in the MCV extracts oxygen from the RBC and causes atraumatic abdominal pain and distension.
and major histocompatibility complex in sickling. Parents can be tested for further A massive tender spleen is unmistakable.
a patient of the relevant ethnicity should confirmation. The haematocrit is usually less than 50%
prompt further investigations. of baseline. Reticulocytosis and moderately
severe thrombocytopenia are present. Splenic
Clinical features
a-Thalassaemia sequestration of RBCs affects those SCD
The baseline haemoglobin in SCD is in the
Two genes on chromosome 16 code for the sufferers who are young enough to still have
range of 6090 g L1 with a reticulocytosis
a-chain, producing four possible pheno- a spleen, as autosplenectomy from infarc-
of 515%. Children with SCD have abnor-
types. Deletion of one a-gene is asymptom- tion commonly occurs. Hepatic sequestration
mal immune function. They have functional
atic, with a minority having a microcytic may also occur but the volume of trapped
asplenism by age 5 years, although some-
hypochromic anaemia. Deletion of two a- RBCs is unlikely to lead to devastating hypo-
times earlier, as well as abnormal comple-
genes produces mild anaemia. Deletion of volaemia due to the tight hepatic capsule.
ment components. Consequently, fever
three a-genes results in HbH disease due Treatment is resuscitation of the intravas-
should be managed as a medical emergency
to aggregation of b-chains, which leads to cular volume. Prevention of sickling of those
with prompt medical evaluation and deliv-
an unstable haemoglobin. There is chronic RBCs remaining in the circulation is impor-
ery of antibiotics because of the high risk
haemolysis with anaemia in the range of tant as is the prompt treatment of the
of bacterial infection and mortality.
70110 g L1, jaundice, cholelithiasis, hepa- infective precipitant. ASS is recurrent in 50%
Dactylitis is frequently the first manifesta-
tomegaly and leg ulcers. Deletion of four of cases, with the interval between episodes
tion of pain in children with SCD, occurring
a-genes results in intrauterine death and being less each time. Splenectomy is the only
in 50% of children by 2 years of age.
stillbirths, usually after 25 weeks gestation. method of preventing recurrent ASS.
Children present with symmetric painful
Live births have severe anaemia, which may
swelling of the hands and/or feet and
lead to congestive cardiac failure. The fetus Sickle cell trait
require careful management with pain med-
is grossly oedematous. The haemoglobins Sickle cell trait denotes the heterozygous or
ication. Differential diagnosis of osteomyeli-
present are HbH, Hb Barat (four g-chains) carrier state. The complete blood count is
tis should be considered carefully if the
and small amounts of Hb Portland (two z within the normal range. Haemoglobin analy-
presentation is unilateral.
and two g). None of these are functional. sis is diagnostic, revealing a ratio of HbS to
Vaso-occlusive crises are acute episodes of
HbA of 40:60. The RBCs have a normal life-
severe pain from tissue infarction resulting
span. Serious complications in sickle cell trait
from vessel occlusion by the sickled cells.
Sickle cell disease are very rare, but may include sudden death
The major organs involved are bones, lungs,
during rigorous exercise, splenic infarcts at
Sickle cell disease (SCD) is an autosomal reces- liver, spleen, brain and the penis. Painful
high altitude, haematuria, and bacteriuria.
sive inherited condition in which glutamine in bone crisis is the commonest, with minimal
The life span of people with sickle cell trait is
the sixth position of the globin chain is signs on examination. Treatment is directed
normal and children with sickle cell trait should
replaced by valine. In the homozygote with at effective pain relief. Intravenous fluids
not have any restrictions placed on activities.
HbSS the haemoglobin is unstable in the de- should be utilised only if clinical evidence
oxygenated state. It precipitates in the RBC of dehydration exists. Frequently recurrent
and leads to a change in the biconcave shape episodes may be reduced by using hydroxy-
to a configuration resembling a sickle. This urea, but close monitoring is required. Acknowledgement
change is initially reversible. The conse- In aplastic crisis, the reticulocytosis falls to The contribution of Marian Lee as author in
quences are multiple. It is detrimental to the less than 1%. The haematocrit may fall as the first edition is hereby acknowledged.
RBC and the end-organs. Sickle cells have a rapidly as 1015% per day. The precipitant
reduced life-span of 1020 days and occlude is usually an infection. Spontaneous recov-
the microvasculature, leading to end-organ ery is usual. Supportive therapy may involve
ischaemia. The precipitants for the sickling are:
Further reading
transfusion of RBCs.
Chang TT. Transfusion therapy in critically ill children. Pediatr
Neonatol 2008;49(2):512.
tissue hypoxia; Gera T, Sachdev HP, Nestel P, Sachdev SS. Effect of iron
tissue acidosis; Acute splenic sequestration supplementation on haemoglobin response in children:
systematic review of randomised controlled trials. J Pediatr
dehydration; Acute splenic sequestration (ASS) is a life- Gastroenterol Nutr 2007;44(4):46886.
vascular stasis; threatening complication of SCD occurring Kleigman R, Behrman R, Jenson H, Stanton B. Nelsons Textbook
of Pediatrics. 18th ed. Philadelphia: Saunders Elsevier; 2007.
increased 2,3-diphosphoglycerate in 730% of patients under the age of Richardson M. Microcytic anemia. Pediatr Rev 2007;28
(2,3-DPG) levels in the RBC. 2 years with homozygous SCD. It has also (4):151.

269
 11.3 Disorders of coagulation
Evelyn Doyle

recombinant factor concentrates. When

ESSENTIALS available and expense allows, recombinant
factor concentrates should be used. Factor
1 Haemophilia and von Willebrand disease account for the majority of inherited replacement may be given following a con-
coagulation disorders.
firmed bleed or as prophylaxis against
2 A positive family history of a bleeding disorder should be sought when a potential bleeding; for example, prior to den-
coagulation disorder is suspected. tal extraction. Prophylactic factor replace-
ment, usually given two to three times a
3 Establishing disease severity (assayed factor levels), past history of bleeding, the week at home, with increased doses given
nature of this bleed and presence of inhibitors will help guide replacement therapy in
for any breakthrough bleeds, has led to
haemophilia.
a marked reduction in the number and
4 Always consider hidden potentially life-threatening bleeding: intracranial severity of bleeding episodes.
bleeding is a major cause of mortality in the haemophiliac patient. The half-life of factor VIII is 812 hours
and factor XI is up to 24 hours, so repeated
doses may be needed. Many patients now
Other significant and potentially life- initiate treatment for minor bleeds at home.
Haemophilia Early and appropriate treatment reduces the
threatening bleeding can occur and should
Introduction be considered in the haemophiliac patient: risk of deterioration to disabling arthropathy
Haemophilia A and B are coagulation disor- intracranial haemorrhage occurs with incre- and chronic pain. The amount of clotting
ders usually transmitted by X-linked recessive ased frequency after head trauma, retroperi- factor required depends on the patients
inheritance (70% of cases) but can occur as toneal haematoma may occur spontaneously weight, severity of disease, the location of
spontaneous gene mutations (30% of cases). or following trauma and may mimic appendi- the bleed and also previous bleeding in
Haemophilia A results from factor VIII citis, soft tissue haemorrhage in the head or the same area and presence of inhibitors
deficiency and Haemophilia B (Christmas neck is potentially life threatening due to Lacerations generally require factor
disease) is caused by factor IX deficiency. the risk of airway obstruction. Gastrointestinal replacement to prevent excessive bleeding.
Acquired haemophilia is rare and occurs when haemorrhage is a less common manifesta- Head injury requires a high index of suspi-
autoantibodies develop against factor VIII. tion of haemophilia in children. Haematuria cion and a very low threshold for factor
may be macroscopic or microscopic. treatment. Continuous intravenous (IV) fac-
Clinical presentation In the current paediatric haemophiliac tor infusion may be required for major bleed-
Haemophilia A and B are clinically indistin- population, transfusion-borne viruses includ- ing. A negative cranial CT scan does not
guishable. Disease severity correlates well with ing hepatitis B and C and human immunode- preclude the need for factor replacement.
assayed factor levels: severe disease occurs ficiency virus are rare, as most cases of Analgesia should be provided as required
with factor levels <1% of normal, moderate transmission occurred prior to 1985. but aspirin should be avoided. Other non-
and mild disease occur with levels between steroidal anti-inflammatory drugs may be
15% and above 5%, respectively. In the Investigations used on the advice of the treating haematol-
absence of a positive family history, disease Haemophilia A and B both cause a prolonged ogist. Narcotic analgesia has limitations asso-
may go undetected for a variable period, activated partial thromboplastin time (aPTT) ciated with use in treating a chronic condition.
depending on disease severity, with some cases with a normal prothrombin time and platelet Desmopressin (DDAVP), a synthetic ana-
of mild disease being diagnosed in adulthood. count. Individual factor assays can then be per- logue of vasopressin that elevates factor VIII
Bleeding can occur in any tissue and may formed to confirm the diagnosis. Haemophi- levels for several hours after administration
occur spontaneously or with minimal trauma. liacs presenting with suspected intracranial by stimulating release from endothelial
Most neonates, even those with severe dis- or retroperitoneal bleeds need computerised stores, can be used to treat mild haemophilia
ease, are born without significant bleeding, tomography (CT) scanning. Plain radiographs A. In an emergency situation where no clot-
though intracranial haemorrhage may occur. will exclude fractures associated with trau- ting factor is available, fresh frozen plasma
As a child starts to ambulate bleeding epi- matic haemarthroses. (FFP) or cryoprecipitate can be administered.
sodes becomes more frequent. Unlike disor- Inhibitors are alloantibodies that develop
ders of platelet function where mucosal Treatment against clotting factors and are present
bleeding typically occurs, bleeding into joints The primary treatment goal is control of in 1020% of those with haemophilia A
and soft tissues is characteristic, with large bleeding by replacement of clotting factor. and 35% of those with haemophilia B.
joints of the knee, ankle, hip, elbow, wrist Factor concentrates are available as virus- Inhibitors may mean that large amounts
and shoulder involved most frequently. inactivated plasma-derived product and of clotting factor are required, or that

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11.3 DISORDERS OF COAGULATION
11

HAEMATOLOGY AND ONCOLOGY

clotting factors will not work at all. Treat- to be moderate to severe. Type 2N may be children under 3 years of age in whom there
ment options for patients with haemophilia misdiagnosed as haemophilia A until vWF have been case reports of water intoxica-
are prothrombin complex concentrate (pro- testing becomes available. tion and hyponatraemia. An intranasal
thrombinex) or recombinant activated factor Type 3: A total quantitative deficiency of DDAVP preparation is available but may be
VIIa. Elimination of inhibitors is possible in a vWF and decreased factor VIII activity less reliable in children if part of the dose is
proportion of patients using immune toler- occurs in this rare form of vWD. Clinically, swallowed. The dose is 24 mcg kg1.
ance therapy, where daily low-dose factor bleeding is severe and indistinguishable Plasma-derived factor VIII concentrates
infusions will lead to the development of from Haemophilia A, with mucosal, soft contain both vWF and factor VIII. Recombi-
neutralising anti-inhibitor antibodies. tissue and joint bleeding. nant factor VIII does not contain vWF and
is ineffective. These plasma-derived products
Investigations are the treatment of choice for type 2A, 2B
Von Willebrand disease A coagulation screen may be normal and (in which DDAVP is contraindicated) and
does not rule out vWD. In most patients type 3 vWD. They are virus inactivated and
Introduction
aPTT is normal, though a prolonged aPTT effective to control bleeding in most clinical
Von Willebrand disease (vWD) is the com-
can occur in types 2N and 3, with reduced situations. FFP contains both vWF and factor
monest inherited disorder of coagulation
factor VIII levels. Bleeding time may be pro- VIII but very large volumes would be
and is present in 1% of the population in
longed. Screening tests for vWD include: required to raise vWF to satisfactory levels.
screening studies, though only a minority will
vWF assay, ristocetin co-factor assay and fac- Adjunctive treatments include tranexa-
present with clinically significant bleeding. In
tor VIII activity. The diagnosis and typing of mic acid for bleeding involving the oral or
most cases inheritance is autosomal domi-
vWD is difficult and usually requires a spe- nasal mucosa, either alone or in conjunction
nant with variable penetrance and expressiv-
cialist laboratory. Levels are affected by with DDAVP. Synthetic oestrogens cause an
ity, though autosomal recessive inheritance
blood group and physiological factors such unpredictable rise in vWF but may be useful
occurs (Type 2N and 3). Rarely, vWD may
as stress and exercise. The patients personal in controlling menorrhagia.
be acquired and has been described in condi-
and family history of bleeding must also be
tions such as hypothyroidism, and SLE. Von
taken into account in making this diagnosis.
Willebrand factor (vWF) is a large protein that Controversies and future
has two roles in coagulation: it facilitates directions
Treatment
platelet adherence to the vascular endothe-
The main aim of treatment in vWD is Haemophilia is particularly suited to gene
lium enabling a platelet plug to be formed
replacement of deficient or defective vWF therapy intervention because the disease
and also acts as a carrier protein for factor
and factor VIII. Treatment is usually given is due to deficiency of a single gene
VIII. Quantitative or qualitative abnormal-
in response to bleeding or prophylactically product. Gene therapy is still at the stage of
ities of vWF occur. Three types of vWD and
prior to surgical or dental procedures. Treat- small human trials. Von Willebrand disease
three subtypes have been described: Type
ment options currently available are desmo- treatment options for the future include
1, 2 (subtypes 2A, 2B, 2M,2 N) and 3.
pressin and plasma concentrates recombinant vWF, which has shown
Desmopressin (DDAVP), a synthetic ana- promising early results in animal trials,
Clinical presentation
logue of vasopressin releases factor VIII and interleukin-11 has also been shown to
Mucosal and cutaneous bleeding, prolonged
and vWF from endothelial storage sites, caus- increase vWF, but human trials are needed.
bleeding after surgical procedures and a pos-
ing a transient rise in levels. DDAVP is effec-
itive family history of a bleeding disorder
tive in type 1 vWD and will increase plasma
should prompt consideration of vWD. In chil-
levels of vWF between three and five times
dren, bruising and bleeding out of proportion Acknowledgement
from baseline within 3060 minutes. It is
to the severity of the injury is typical. Hae-
usually ineffective in types 2A, M and N as The contribution of Fiona Reilly as author in
marthroses, intracranial and intra-abdominal
vWF is functionally abnormal, and in type 3 the first edition is hereby acknowledged.
bleeds are much less common but can occur
vWD as an appreciable rise in levels may
in more severe types 2N and 3 vWD. vWD is
be clinically undetected. Desmopressin is con-
diagnosed in 10 to 20% of women and ado- Further reading
traindicated in type 2B vWD as it may cause
lescents with menorrhagia and in 15% of Eikenboom J, Van Marion V, Putter H, et al. Linkage analysis in
thrombocytopenia and thus worsen bleeding. families diagnosed with type 1 von Willebrand disease in
children with recurrent epistaxis.
The dose is 0.3 mcg kg1 given intrave- the European study, molecular and clinical markers for the
diagnosis and management of type 1 VWD. J Thromb
Type 1: The commonest form accounts for nously in 3050 mL normal saline over 30 Haemost 2006;4:77482.
6080% of cases, and results from a minutes. The most common adverse effects Fischer K, van der Bom JG, Mauser-Bunschoten EP, et al.
Changes in treatment strategies for severe haemophilia
partial quantitative deficiency of vWF. are facial flushing, headache and tachy- over the last 3 decades: Effects on clotting factor
When bleeding occurs it ranges from mild cardia, which usually respond to slowing the consumption and arthropathy. Hemophilia
2001;7:44652.
to moderate, with only a minority having infusion. Repeat doses can be given at 12 Mannucci PM. Treatment of Von Willebrands disease. N Engl J
clinically significant bleeding episodes. to 24 hours if needed but tachyphylaxis Med 12 2004;351(7):68394.
National Hemophilia Foundation. www.hemophilia.org.
Type 2: (subtypes A,B,M and N) have occurs after approximately 48 hours. Rakel RE, Bope ET. Conns Current Therapy 2009. 1st ed.
qualitative defects of vWF. Bleeding tends DDAVP should be used with caution in Philadelphia: Saunders; 2009.

271
 11.4 Platelet disorders
Jane Cocks

is otherwise normal, with no signs of hepa-

ESSENTIALS tosplenomegaly or lymphadenopathy.

1 Abnormal bleeding can occur from disorders of total numbers of platelets Investigation
or platelet function.
The blood count will demonstrate thrombo-
2 The normal platelet count is between 150 and 400  109 L 1
. cytopenia. Abnormally large and/or abnor-
mally small platelets may be seen on the
3 Idiopathic thrombocytopenic purpura (ITP) is the commonest platelet disorder film. The blood count and film are otherwise
in childhood.
usually normal. Anaemia may be present,
dependent on the extent of previous hae-
morrhage. Eosinophilia may be noted, but
Kawasaki disease, patients with malignancy is not consistent. Coagulation studies will
Introduction and polycythaemia rubra vera. Thrombocyto- be normal, bleeding time prolonged. There
The normal platelet count is between 150 sis of itself is rarely of clinical importance but is no specific antiplatelet antibody test avail-
and 400  109 L1 and platelets usually platelet levels greater than 1000  109 L1 able for routine clinical use. Bone marrow
exist within the circulation for 57 days. In can be associated with acute thrombosis or aspiration is usually performed after 4 weeks
normal haemostasis, blood loss is initially lim- haemorrhage. if there is no evidence of remission, or earlier
ited by the formation of a platelet plug and if the diagnosis is unclear.
cross-linked fibrin at the site of injury. In plate-
let disorders this platelet plug is not formed or Differential diagnosis
is ineffective, and abnormal bleeding occurs. Idiopathic The diagnosis of ITP relies on the presence
Platelet abnormalities can be either quantita- thrombocytopenic purpura of laboratory-demonstrated thrombocytope-
tive or qualitative. Small petechial skin hae- Introduction nia, with a history, clinical examination and
morrhages, less than 3 mm in diameter, are Idiopathic thrombocytopenic purpura (ITP) full blood count that do not suggest another
characteristic of platelet defects. is the commonest platelet disorder in chil- cause. In the presence of a limp, hepatosple-
The commonest platelet disorder is throm- dren. It is an autoimmune disorder charac- nomegaly or lymphadenopathy, consider
bocytopenia (low platelet count). Significant terised by the development of platelet the possibility of an underlying lymphoproli-
thrombocytopenia has a characteristic bleed- autoantibodies leading to decreased plate- ferative disorder. In infants with bruising,
ing profile of spontaneous bruising, pete- let survival. The incidence of ITP peaks in intentional injury must be excluded. In
chiae, epistaxis and mucosal bleeding. winter and spring due to the increased inci- WiskottAldrich syndrome eczema is pres-
Bleeding complications usually only occur dence of viral infections at those times. ITP ent in the first year and the platelets are
at levels of less than 50  109 L1, with spon- in children is usually of the acute form, which smaller than normal. The presence of other
taneous bleeding possible at levels below lasts for less than 6 months, with a high rate congenital abnormalities might be a clue to
10  109 L1. As most platelet counts are of spontaneous resolution. ITP in children is Fanconis anaemia. In older children, partic-
performed by electronic particle counters, most common between the ages of 2 and 6 ularly those with a chronic course, a systemic
an inappropriately low platelet count can years, but can occur in any age group. Up to autoimmune disorder such as lupus or anti-
result from the spontaneous platelet clump- 28% of children with ITP will develop a phospholipid syndrome should be consid-
ing that can occur in EDTA collection tubes. chronic form which lasts longer than 6 ered. In infants, exclude cytomegalovirus
This is confirmed by direct inspection of the months. and consider immunoglobulins and T/B lym-
peripheral blood smear for platelet clumping. phocyte subsets to exclude immune
In qualitative disorders of platelet function disorders.
the abnormal bleeding occurs despite a Clinical presentation
normal platelet count. Platelet dysfunction ITP typically presents up to 3 weeks follow- Management
may occur in hepatic failure, chronic renal fail- ing a viral-type infection or vaccination. Most children recover spontaneously within
ure, myeloproliferative disorders, with some There is generally a short history of bruising, 6 to 8 weeks and have only cutaneous or
drugs (e.g. aspirin) and in rare inherited non-blanching rash (petechiae or purpura) mild bleeding. They require no specialist
disorders of platelet function (Glanzmann, or mucosal bleeding in an otherwise well treatment and can be managed as ambula-
Portsmouth, HermanskyPudlak, May child. The mucosal bleeding is usually from tory patients. Parents should be supported
Hegglin and BernardSoulier syndromes). the gums or nose. Haematuria is also com- with good written advice on the condition
Thrombocytosis (elevated platelet count) mon. The petechial rash may appear in crops and given a telephone contact name and
is seen in inflammatory reactions including over several days. The physical examination number. Written advice should include

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11

HAEMATOLOGY AND ONCOLOGY

information about avoiding contact sports, IVIG infusion will lead to a rapid platelet will occur in up to two-thirds of these
bangs to the head, aspirin and other drugs count rise, usually within 48 hours, lasting patients in subsequent years. A chronic
that interfere with platelet function. They for 24 weeks. As a pooled blood product course is most often seen in older children,
should watch for bleeding and report to hos- IVIG carries the risk of viral transmission particularly adolescent girls. Some may have
pital if new episodes occur. In the initial and is expensive. A range of side effects underlying conditions such as lupus. Most do
stages the platelet count can be reviewed have been reported. not have significant bleeding problems or a
weekly but this can be extended to every High-dose oral steroids may also be con- need for regular treatment.
2 to 4 weeks once it is improving. School sidered, although IVIG has been demon-
can be recommenced when platelets are strated to promote a faster response to
greater than 20  109 L1. By 6 months get platelet numbers above 50  109 L1. Controversies
around 85% will have a normal platelet Anti-D is an effective alternative in Rh-
count. Patients with platelet counts greater positive children only, but the platelet The Intercontinental Childhood ITP
than 150  109 L1 can be discharged but increase is slower, taking 4872 hours. Registry was established in 1997 to
warned that occasional relapses are seen Splenectomy is reserved for life-threatening prospectively investigate the clinical
and to re-present if symptoms recur. haemorrhage or children with significant course, management and outcome of
Consider admission of patients with initial bleeding who have failed to respond to medical children with ITP. Since then a
platelet counts below 20  109 L1. Serious treatment . Post-splenectomy patients have an Splenectomy Register and a prospective
bleeding is rare in ITP, with intracranial hae- increased risk of sepsis despite pneumococcal database on both paediatric and adult
morrhage occurring in 1% of patients with vaccination and antibiotic prophylaxis. patients with chronic ITP have also
platelet counts less than 20  109 L1. All been set up. Information from these
children with counts less than 10  109 L1 databases should provide the evidence
Medical emergencies
during the course of the illness should be base for the optimum treatment strategy
The risk of intracranial haemorrhage is less
admitted. The decision to admit may be for individual children with ITP in the
than 1% but persists as long as the platelet
influenced by the social circumstances and future.
count is very low. Children with ITP and
parents coping ability. severe headache with neurological signs
Consider treatment if bleeding is severe. require urgent investigation with cranial
There are a number of established options computerised tomography scan and immedi-
aimed at increasing the platelet count but ate treatment if indicated with IVIG and Acknowledgement
there is no specific therapy to treat the steroids. Life-threatening haemorrhage is
underlying disorder. the only instance where platelet transfu- The contribution of Kieran Cunningham
sions should be used, as the antibodies in as author in the first edition is hereby
Current treatments ITP target all platelets including donor acknowledged.
Asymptomatic children with acute ITP and transfusions. As a result the benefit of any
platelet count <20  109 L1 should be transfusion is very short-lived. Emergency
carefully observed. In the presence of pur- splenectomy may be used as a last resort.
pura, significant mucosal membrane bleed-
ing or other haemorrhage, intravenous Further reading
Pediatric Blood Cancer 2006;47(S5):649745.
immunoglobulin (IVIG) infusion is recom-
mended and high-dose oral glucocorticoids
Chronic ITP The Intercontinental Cooperative ITP Study Group (ICIS).
website: http://www.itpbasel.ch/Home.90.0.html.
Greer JP, Foerster J, Rodgers GM, et al. Wintrobes Clinical
can be considered with the involvement of Around 1028% of children fail to remit Haematology. 12th ed. Baltimore: Lippincott Williams &
a paediatric haematologist. within 6 months but spontaneous remission Wilkins; 2008.

273
 11.5 Vasculitis
Evelyn Doyle

Skin lesions begin as maculopapules that

ESSENTIALS initially blanch and develop to palpable pur-
pura, which may include macules, papules,
1 The most common childhood vasculidites are HenochSchonlein purpura (HSP) vesicles, bullae, nodules and urticaria.
and Kawasaki disease.
Lesions are symmetrical and maximal on
2 In HSP persistent renal disease occurs in a minority of children. Repeat urinalysis is lower limbs and buttocks and may be pain-
recommended at intervals until resolution of symptoms, as nephritis may be delayed. ful. Local angio-oedema of face, hands, feet,
back, scrotum and perineum is common in
3 Kawasaki disease should be considered in all children with unexplained high fever younger children.
for more than 5 days. All features may not be present simultaneously and a history of
Arthritis occurs in up to 80%, with one or
mucositis, cervical lymphadenopathy, conjunctivitis, rash and peripheral cutaneous
more joints involved, and resolves leaving no
manifestations should be specifically sought.
residual joint deformity. Large joints of the
4 Vasculitis may occur secondary to a primary underlying disease, infections, drug lower extremity are usually affected but
reaction or malignancy and should be excluded. upper extremity joints may also be involved.
Gastrointestinal symptoms are common
5 Vasculitis should be considered in children with a non-specific febrile illness and and include colicky abdominal pain, vomiting
exanthem without explanation that do not resolve as would be expected with a
and bloody diarrhoea. Over half of patients
self-limited infectious illness.
have occult blood in stools. Intussusception
(usually ileoileal) is the most common serious
gastrointestinal complication and results
Introduction Classification from oedema and haemorrhage in the bowel
wall acting as a lead point. Ultrasound is
Childhood vasculitides are a group of disor- The primary vasculitic disorders can be divided
recommended when suspected, as contrast
ders characterised by inflammation of blood according to the size of the vessel involved.
enema will not detect ileoileal pathology.
vessels. Vasculitis is the common end point
Some children present with isolated abdom-
of several different disease processes and
inal symptoms or arthritis and the diagnosis
no one mechanism can explain the patho-
SMALL VESSEL may only become apparent when the rash
genesis in this heterogeneous group of con-
VASCULITIS develops days to weeks later. Nephritis occurs
ditions. Distinguishing a primary vasculitis
in about half and may be delayed for up to 4 or
from a secondary underlying disease or from
HSP, Wegeners granulomatosis and Churg more weeks. Other serious though uncommon
conditions that mimic vasculitis can be diffi-
Strauss syndrome predominantly involve conditions can occur including orchitis cere-
cult. Most forms of vasculitis are rare in child-
small vessels; the latter two conditions are bral involvement, including seizures.
hood. HenochSchonlein purpura (HSP) and
Kawasaki disease (KD) are the two common- extremely rare in childhood.
Investigations
est childhood vasculitides.
There is no diagnostic test for HSP; the diag-
nosis is clinical but may be difficult in the
HenochSchonlein purpura absence of rash. Blood pressure measurement,
Clinical presentation urinalysis and renal function assessment at
HSP is the commonest vasculitis in childhood.
The presentation varies widely according to It is immune mediated, associated with IgA presentation are required in all patients. Renal
the size of the affected blood vessel and the complex deposition in tissues, and though sev- biopsy is reserved for patients with nephritis
extent and nature of organ involvement. eral infective agents, drugs and immunisation complicated by nephrotic syndrome, hyperten-
Constitutional symptoms are common to have been implicated, the cause is unknown. sion or renal failure. Throat swab, C3/C4 and
all vasculitides and include fever, weight ASOT may be useful in distinguishing post-
loss, malaise, irritability and arthralgias. Skin streptococcal arthralgia from HSP where the
manifestations are frequent and often give a Clinical presentation clinical picture is not full-blown.
clue to the size of the vessel involved; lesions HSP is characterised by palpable purpura
are commonly purpuric but may be urticar- which is mandatory for diagnosis with one Prognosis
ial, ulcerative, vesicular or resemble ery- or more of the following: diffuse abdominal It is generally a self-limited condition but
thema multiforme. In severe vasculitis, pain, arthritis or arthralgia, renal involve- can recur in one-third of patients. Resolution
vessel destruction leads to tissue infarction, ment, or a tissue biopsy showing predomi- of symptoms occurs over a period of weeks.
and can result in ischaemia and gangrene. nantly IgA deposition. A small percentage of children develop

274
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11

HAEMATOLOGY AND ONCOLOGY

persistent renal disease, with about 0.1% Some children may present with incomplete Echocardiography for coronary aneur-
developing serious renal disease. Those with or atypical Kawasaki disease: they have ysms should be performed in all children.
isolated haematuria and proteinuria without some but not enough features to meet clas- Screening has shown that a substantial
renal insufficiency completely recover. sical criteria. In these children diagnosis can number of children with KD and coronary
be difficult and consultation with an experi- artery abnormalities are not identified by
Management enced physician should be sought. the classic case definition.
Most children are managed as outpatients
Cervical adenopathy.
with supportive care. Non-steroidal anti- Management
Bilateral non-suppurative conjunctival
inflammatory drugs are effective but use Combined therapy with aspirin and high
injection with perilimbal sparing.
with caution in renal impairment. Steroids
 Polymorphous rash (maculopapular, dose intravenous immunoglobulin (IVIG)
are not routinely used as the majority of has been shown to reduce the rate of coro-
multiforme or scarlatiniform).
patients symptoms resolve spontaneously
Peripheral cutaneous or perianal nary artery aneurysm from 25% to 5%
without sequelae, but may be considered when administered in the first 10 days of
changes including erythema, swelling
in patients with severe renal or extrarenal
and induration. Desquamation may illness. High-dose IVIG 2 g kg 1, is usually
symptoms. Steroids have not been found given as a single infusion. High-dose aspirin
occur as a late sign, usually 23 weeks
to prevent development of renal or gastroin- is given initially: doses varying from
after the onset of illness, beginning in
testinal symptoms but may be effective in
the periungal region. 30100 mg kg 1 day 1 are used. Following
treating symptoms. Renal disease is the
Mucous membrane changes to lips defervescence, aspirin is given for its antipla-
major concern long term and specialist con-
and/or oral cavity (fissuring of the telet action in a dose of 25 mg kg 1 once
sultation should be sought in severe disease. daily.
lips, pharyngitis and strawberry tongue).
Follow up should involve blood pressure sur- Children who do not respond after the
veillance and urinalysis for 3 months as Fever is typically high, >39 with minimal first dose of IVIG may respond to a second
renal involvement can occur later even when response to antipyretics, and usually persists dose. A group of patients remain resistant
initial urinalysis is normal. for 1 to 2 weeks if untreated. Extreme to IVIG therapy and some novel therapies
irritability in a toddler with persisting fever have been tried under specialist supervision.
of unknown origin should always prompt Follow up includes regular echocardiogra-
MEDIUM VESSEL consideration of KD. Other associated find- phy, the duration of which will be deter-
VASCULITIS ings are carditis (pericarditis, myocarditis, mined by the degree of coronary artery
valvular involvement), aseptic meningitis, involvement. Stress testing in adolescence
This group includes Kawasaki disease and arthritis, sterile pyuria, uveitis, hepatitis is required if there has been cardiac involve-
polyarteritis nodosa (PAN). and hydrops of the gall bladder. ment and angiography may be indicated.
Long-term antiplatelet or anticoagulation
Investigations therapy may be indicated.
Kawasaki disease There is no diagnostic test for KD. In the
acute stage, laboratory findings may include Prognosis
KD is an acute systemic vasculitis of leucocytosis with left shift, anaemia, and In children without cardiac involvement
unknown aetiology affecting predominantly thrombocytosis (in the second to third recovery is usually complete though the
medium muscular arteries, with a predilec- week). Mild hepatitis with raised transami- long-term effects are largely unknown.
tion for coronary arteries. It is the second nases and elevated conjugated bilirubin, Aneurysmal coronary artery dilatation
most common vasculitic disease of child- and cerebrospinal fluid analysis may show occurs in 25% of untreated KD, and half
hood after HSP. The disease frequency varies a mononuclear pleocytosis with normal glu- to two-thirds regress over time but stenosis
globally, with highest rates in Japan and in cose and protein and sterile culture. in these vessels may progress independently
Japanese children born overseas. It predom- Mild proteinuria and pyuria may also be of this. The worst prognosis occurs in large
inantly affects children under the age of 5 present. aneurysms (>8 mm), and risks include
but can occur at any age. Coronary artery If atypical KD is suspected laboratory thrombosis, myocardial ischaemia and
aneurysms develop in 1525% of tests, though non-specific, may be useful. infarction; also late sequelae of KD may
untreated children and may lead to ischae- The diagnosis is unlikely if platelet count, manifest in adulthood.
mic heart disease or sudden death. erythrocyte sedimentation rate (ESR) and
C-reactive protein (CRP) are normal after
Clinical presentation day 7 of illness. A moderate to marked ele-
Polyarteritis nodosa
Diagnosis of KD is based on clinical criteria vation in CRP or ESR is almost universal in
and the classic form presents with unex- children with KD. Leucopenia, lymphocyte PAN is a necrotising vasculitis of unknown
plained high fever lasting at least 5 days predominance, and low platelet count in aetiology involving medium-sized muscular
and four of the following five clinical fea- the absence of disseminated intravascular arteries. It is rare in childhood. PAN is a
tures, or fever with coronary artery involve- coagulation are also suggestive of an alter- systemic illness with a wide spectrum of
ment and three other clinical findings. native diagnosis. clinical presentations depending on the

275
11.5 VASCULITIS

organs involved. Constitutional symptoms bacterial endocarditis, the clinical appear-

are common. Skin manifestations may SECONDARY ance resembles a primary vasculitic process
resemble those of HSP or erythema multi- VASCULITIS AND but is secondary to septic emboli.
forme or may be necrotic with peripheral VASCULITIS MIMICS
gangrene. Other features include myalgia,
ischaemic heart pain, nephritis and cerebral Vasculitis is seen secondary to a primary Controversies and future
manifestations of stroke, visual loss and underlying disease, infections, drug reaction directions
psychosis. Diagnosis relies on elevation of or malignancy. Vasculitis occurs in connec- Aspirin, though routinely used to
inflammatory markers (ESR and CRP) and tive tissue disorders such as systemic lupus treat Kawasaki disease, has not been
biopsy showing characteristic histopatholo- erythematosus and dermatomyositis. There shown to reduce the risk of coronary
gical changes or angiographic or magnetic is an increased association with vasculitis artery aneurysm in Kawasaki disease.
resonance angiography evidence of aneu- in patients with familial Mediterranean
rysm or occlusion in affected vessels. Anti- fever, with several patients described with Steroid use in HSP is not routine and
neutrophil cytoplasmic antibodies (ANCA) PAN and HSP features. remains controversial but recent studies
are present in some forms of PAN. Many infectious diseases have been asso- appear to show benefit in terms of
ciated with vasculitis, predominantly with resolution of symptoms and may be
cutaneous manifestations. Papulovesicular considered in patients with severe disease.
LARGE VESSEL acrolocated syndrome (PALS or Gianotti  International collaboration to study
VASCULITIS Crosti syndrome) is a vasculitis associated rare disorders such as paediatric
with multiple viral and other triggers, includ- vasculitis may point toward new
Takayasu disease is a large vessel vasculitis ing hepatitis B antigenaemia, EpsteinBarr treatment approaches.
of unknown aetiology involving the aorta virus, cytomegalovirus, human immunodefi-
and its main branches. It occurs 10 times ciency virus, rickettsial diseases and strepto-
more commonly in young women than coccal infections. Multiple skin-coloured or
men. Morbidity results from stenosis, ischae- red, flat-topped papules occur in an acral dis-
Acknowledgement
mia and aneurysmal dilatation of affected tribution with sparing of the trunk. The contribution of Ruth Barker as author in
vessels. Presentation may be non-specific Hypersensitivity angiitis is a serum-sickness- the first edition is hereby acknowledged.
with constitutional symptoms or specific like reaction in infants that includes a rash,
symptoms relating to inflammation of large arthropathy and fever. It commonly follows
vessels, including congestive heart failure, the use of penicillin or cephalosporins (partic- Further reading
angina, myocardial infarction, stroke, limb ularly Ceclor). Skin manifestations include Brogan PA, Bose A, Burgner D, et al. Kawasaki disease: an
evidence based approach to diagnosis, treatment, and
claudication and renal artery hypertension oedema of the dorsum of the hands and feet proposals for future research. Arch Dis Child
may be found. In children, careful examina- and a nodular, purpuric, urticarial or ery- 2002;86:28690.
Dillon MJ, Ozen S. A new international classification of
tion of pulses and blood pressures in all four thema-multiforme-like rash. Arthralgia and childhood vasculitis. Pediatr Nephrol 2006;21:121922.
extremities, with a search for asymmetry and arthritis are common and respond to non- Newburger JW, Takahashi M, Gerber MA, et al. Diagnosis,
treatment, and long term management of Kawasaki disease:
bruits is essential. High-dose corticosteroids steroidal anti-inflammatory drugs, with short- a statement for health professionals from the Committee on
are first line therapy. In resistant or relapsing term oral steroids reserved for the more severe Rheumatic Fever, Endocarditis, and Kawasaki Disease,
Council on Cardiovascular Disease in the Young, American
patients, cyclophosphamide and metho- cases. The condition is self limiting but recurs Heart Association [Published correction appears in Pediatrics
trexate have been used. Long-term immuno- on re-exposure to the same agent. 2005;115:1118]. Pediatrics 2004;114:170833.
Weiss PF, Feinstein JA, Luan X, et al. Effects of corticosteroid
suppressive therapy is required in some Any process that causes occlusion of a on HenochSchonlein purpura: a systematic review.
patients. blood vessel can mimic vasculitis. In subacute Pediatrics 2007;120:107987.

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HAEMATOLOGY AND ONCOLOGY

11.6 Acute leukaemia
Joseph Ting

ESSENTIALS Differential diagnosis

1 At its most severe, acute leukaemia leads to diffuse bone marrow infiltration and Pancytopenia may result from acute leukae-
failure. Pancytopenia including neutropenia, anaemia and thrombocytopenia as well mia, aplastic anaemia, marrow infiltra-
as extramedullary hematopoiesis give rise to classical symptoms of infection tion by non-haematological malignancy and
susceptibility, easy fatigability, bleeding diathesis and hepatosplenomegaly. collagen-vascular disease. However, hepato-
splenomegaly and adenopathy is unusual
2 ED supportive treatment in conjunction with a paediatric haematologist includes with the alternative diagnoses. Viral infec-
broad spectrum antimicrobial administration or sepsis resuscitation, packed red tions may lead to lymphadenopathy and
blood cell transfusion for symptomatic or critical anaemia and platelet replacement if hepatosplenomegaly accompanied by atypi-
the child has high potential or actual risk of thrombocytopenia-related bleeding. cal lymphocytes rather than blast cells. Leu-
kaemoid reactions with leucocyte counts
>50  109 L1 may rarely occur with acute
receptor, cell antigen and chromosomal stud- infections and inflammatory disease such as
Introduction
ies. Classification of leukaemia is complex, rheumatoid arthritis. Marked lymphocytosis
The leukaemias are a group of diseases char- with the FrenchAmericanBritish (FAB) without leukaemic cells occurs in pertussis.
acterised by the clonal proliferation of malig- categories being useful for AML and immuno- Isolated thrombocytopenia in childhood
nant immature white blood cell precursors. typing better suiting ALL.4,5 may follow a viral infection or be associated
They differ in the lineage and degree of differ- with idiopathic or thrombotic thrombocytope-
entiation of cells involved, being broadly nic purpura rather than leukaemia.
divided into two groups: lymphoid and mye-
loid. These two categories are further subdi-
Clinical presentation
vided into an acute form that progresses
Investigations
more rapidly than the chronic disease, which Signs and symptoms in ALL and AML are
is relatively indolent. Preconceptional germ similar and depend on the degree of marrow Full blood count reveals anaemia and throm-
cell and postnatal environmental exposure infiltration and extent of extramedullary bocytopenia in 80% of cases. The majority
to electromagnetic radiation and carcinogens involvement. The first symptoms are non- of children have leucocyte counts below
may play a role,13 with higher risk in children specific including lethargy, bone pain and 20  109 L1. Those with leucocyte counts
with congenital neutropenia, Downs syn- loss of appetite. Classic findings in acute up to 900  109 L1 usually have extrame-
drome and Fanconi anaemia.4 Previous che- leukaemia include fever/infection, pallor/ dullary involvement with hepatosplenome-
motherapy and radiotherapy are associated lethargy/easy fatigability and bruising/ galy and lymphadenopathy. The neutrophil
with increased risk of a secondary malignancy. mucosal bleeding due to febrile neutrope- count is often depressed to below 1.0 
Acute lymphoblastic leukaemia (ALL) com- nia, anaemia and thrombocytopenia, respec- 109 L1. Circulating blast cells are frequently
prises four-fifths of childhood leukaemia,4 tively. Marrow infiltration leads to bone pain present on blood film examination. Blood
with acute myeloid leukaemia (AML) and and extramedullary spread leads to painless typing is necessary if red-cell transfusion is
chronic myeloid leukaemia (CML) accounting adenopathy (including mediastinal), hepato- anticipated. A screen for atypical antibodies
for 15% and 2%.5 Together they account for megaly, splenomegaly, skin or periorbital is warranted if there have previously been
one-third of all malignancies in children under infiltrates and rash. Difficulty with diagnosis multiple occasions of blood product use.
15 years old. Although children of all ages are arises in differentiating early acute from Cultures of blood and other potential infec-
affected, the peak incidence is between 2 and chronic disease, when symptoms (night tion sites are obtained prior to antimicrobial
6 years. sweats, arthralgia, bone pain, constitutional administration. Blood cultures may be posi-
symptoms) and signs (enlarged liver and tive in up to 25% of cases of newly diagnosed
spleen, lymphadenopathy) are variable leukaemia. Tumour lysis syndrome due to
and non-specific. Central nervous system massive cell death during treatment causes
Classification
(CNS) involvement occurs in 4% of children raised lactate dehydrogenase (LDH), liver
Leukaemogenesis represents clonal prolifera- (cranial nerve palsy, cord compression) and enzymes, hyperkalaemia, hyperuricaemia,
tion of white blood cells arrested at various testicular leukaemia in 10% of boys (pain- hyperphosphataemia, hypocalcaemia and
stages of differentiation, lending itself to less enlarged testes). Hyperviscosity results acute renal failure. Anterior mediastinal
diagnostic and prognostic classification in tissue infarction (CNS, pulmonary) and masses are visible on chest X-ray in 510%
based on the cell line affected, degree of rarely priapism results from leucocyte slud- of cases and pneumonia may require exclu-
differentiation, immunophenotyping, cell ging and cavernosal obstruction. sion. Bone-marrow aspiration and trephine

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11.6 ACUTE LEUKAEMIA

(BMAT) is essential for specific diagnosis and transfusion) and correction of acute or symp-
 Biological (e.g. monoclonal
prognostication, including complex tests tomatic anaemia, which are described
antibodies) rather than cytotoxic agents
such as immunophenotyping, cytogenetics, elsewhere. Chemotherapy may give rise to
and agents with better specificity for
molecular studies and cell-cycle kinetics. bone-marrow suppression, similar to the
leukaemic cells result in less collateral
AML is, rarely, complicated by a coagulopa- sequelae of acute leukaemia. Children who
damage 10 Gene therapy, although
thy that progresses to disseminated intravas- relapse or do not achieve remission are candi-
promising, remains experimental and
cular coagulation, so that a coagulation dates for bone-marrow transplantation.
clinically unproven.11
profile and fibrinogen level may be helpful. Barrier nursing, isolation, antimicrobial and
antiviral chemoprophylaxis, vaccination and
post-exposure administration of immuno-
Prognosis globulin (e.g. varicella zoster virus) and
careful monitoring for early infective compli- References
The cure rate for ALL is 80%,4 compared cations help prevent complications related 1. Schutz J, Ahlbom A. Exposure to electromagnetic fields
with only 3050% for AML,5 depending to immunosuppression. Psychosocial support and the risk of childhood leukaemia: a review. Radiat Prot
on whether adverse prognostic factors are of the child and family is important.
Dosimetry 2008;132:20211.
2. Infante-Rivard C. Chemical risk factors and childhood
present. Poor-risk AML has a dismal outlook, leukaemia: a review of recent studies. Radiat Prot
with less than 20% achieving long-term Dosimetry 2008;132:2207.
3. Greaves M. Infection, immune responses and the
remission. Adverse prognostic factors Controversies and future aetiology of childhood leukaemia. Nat Rev Cancer
include age (ages <1, >10 years), certain directions 2006;6:193230.
4. Smith OP, Hann IM. Clinical features and therapy of
chromosomal translocations (such as
t(4;11) in infant ALL), a very high-presenting The future management of lymphoblastic leukemia. In: Arceci RJ, Hann IM, Smith OP,
editors. Pediatric Hematology. 3rd ed. London: Blackwell
leucocyte count and poor response to first- leukaemia will be influenced by new and Publishing Ltd.; 2006. p. 45081.
5. Kean LS, Arceci RJ, Woods WG. Acute myeloid leukemia.
course treatment. Although the cumulative improved risk stratified therapy to In: Arceci RJ, Hann IM, Smith OP, editors. Pediatric
risk of ALL relapse is 1520%, the risk of improve outcomes and minimise adverse Hematology. 3rd ed. London: Blackwell Publishing Ltd.;
2006. p. 36083.
developing second malignancies after suc- effects. High-risk patients could be 6. Zwaan CM, Kaspers GJL. Possibilities for tailored and
cessful treatment is low.4,5 identified by molecular analysis, in vitro targeted therapy in paediatric acute myeloid leukaemia.
Br J Haematol 2004;127:26479.
pharmacodynamic, pharmacogenetic, 7. Aplenc R, Lange B. Pharmacogenetic determinants of
pharmacogenomic and drug resistance outcome in acute lymphoblastic leukemia. Br J Haematol
2004;125:42134.
Management studies to receive more intensive and 8. Harrison CJ. Cytogenetics of paediatric and adolescent
targeted therapy. 69 acute lymphoblastic leukemia. Br J Haematol
Management requires close collaboration 2009;144:14756.
with a paediatric haematologist. Emergency The increasing use of severely 9. Pui CH, Robison LL, Look AT. Acute lymphoblastic
leukemia. Lancet 2008;371:103043.
department supportive treatment focuses myelotoxic chemotherapy and unrelated 10. Blair A, Goulden NJ, Libri NA, et al. Immunotherapeutic
strategies in acute lymphoblastic leukemia relapsing
on the sequelae of acute leukaemia: febrile bone-marrow transplantation has after stem cell transplantation. Blood Rev
neutropenia and sepsis (broad-spectrum improved prognosis in acute leukaemia 2005;19:289300.
11. Brown P, Smith FO. Molecularly targeted therapies for
antimicrobial agents), actual or potential but has given rise to complications pediatric acute myeloid leukemia: progress to date.
bleeding from thrombocytopenia (platelet related to marrow suppression. Paediatr Drugs 2008;10:8592.

278
 11

HAEMATOLOGY AND ONCOLOGY

11.7 Febrile neutropenia
Joseph Ting

early stages of systemic sepsis. The earliest

ESSENTIALS reliable sign of vascular redistribution is
delayed capillary return, because tachy-
1 Aggressive and early resuscitation of the child with overt or incipient septic shock cardia and tachypnoea can be secondary
is vital to avert hypoperfusion, even in the presence of normal mental state and blood
to the fever. Neutropenic or immunosup-
pressure.
pressed children may have attenuated or
2 Careful physical examination and broad microbiological/radiological altered signs of infection. The absence of
investigation to detect and eradicate the source of infection is essential. neutrophils to localise an infection makes
it difficult to determine the source of infec-
3 Early institution of broad-spectrum antimicrobial therapy, followed by targeted tion by physical examination alone. Pay spe-
culture-guided treatment contributes to improved outcome.
cial attention to body regions vulnerable
to bacterial infection, such as oropharynx,
ears, skin, and perianal area. Evidence of
cytomegalovirus; and 10% due to fungi, with
respiratory, circulatory, renal, and metabolic
Introduction up to a third of episodes due to systemic
derangement indicates severe sepsis.
fungaemia being life threatening.1 Vascular
Infection is a major cause of death in children
catheter-related infections are usually related
with haematological malignancies following
to Gram-positive skin organisms. Although the
bone marrow or solid organ transplantation,
overall mortality rate due to neutropenia-asso- Investigations
iatrogenic immunosuppression, and chemo-
ciated infection is 1%, children undergoing
therapy. The major factor predisposing to Microbiological samples from potential infec-
BMT with febrile neutropenia have a startling
infection in these patients is neutropenia, tion sources such as blood, urine, cerebrospi-
mortality up to 80%.1
which is defined as decreased circulating neu- nal fluid, throat, skin, and central access
Management priorities are:
trophils in the peripheral blood. The normal devices should ideally be obtained prior to,
reference range for neutrophil counts varies early recognition of the child with but not delay, treatment. Similarly, radiologi-
with the age of the child, being highest in compensated septic shock before onset of cal investigation to localise infection source
the neonatal period. In infants, the normal hypoperfusion; should not delay treatment commencement.
threshold is 1000 neutrophils per mL; the aggressive and early resuscitation of the Although no microbiological source is identi-
usual value is 1500 neutrophils per mL up child with overt or incipient septic shock; fied in one-half of febrile neutropenic chil-
to 10 years of age, with an adult threshold careful physical examination and broad dren with cancer, a high proportion have
of 1800 neutrophils per mL applied there- microbiological/radiological culture-positive bacteraemia, respiratory
after.1 Neutropenia is defined as
500 neu- investigation to detect and eradicate the tract infections and central access device
trophils per mL or an anticipated decline source of infection; infections.1 Full blood count with white cell
from 1000 to
500 neutrophils per mL. Infec- Obtaining blood and other cultures prior to differential confirms neutropenia, and when
tion risk increases when the neutrophil count commencing antimicrobial therapy, followed serially tracks progress of neutrophil
is
500 neutrophils per mL and is highest followed by early institution of broad- recovery. Thrombocytopenia and coagulopa-
when
100 neutrophils per mL. Febrile neu- spectrum antimicrobial therapy, followed thy are early markers of disseminated intra-
tropenia requires an associated fever by targeted culture-guided treatment; venous coagulation. Chest X-ray may
38.3 C, or 38.0 C for at least 1 hour.2 This achieving optimal microcirculatory, indicate lower respiratory involvement or car-
chapter focuses on neutropenia associated metabolic and circulatory conditions diac decompensation.
with paediatric cancer or its treatment as this using a combination of intravenous fluids,
is the more frequent type encountered in vasopressors/blood products and
the emergency department (ED). consideration of early mechanical
Neutropenic children receiving chemother-
Treatment
ventilation; however, early goal-directed
apy for leukaemia or undergoing bone-marrow therapy has not been validated in septic IV fluid resuscitation and vasopressor sup-
transplantation (BMT) are at significant risk children. port may be required in septic shock. Urgent
from Gram-negative sepsis, including Pseudo- empiric intravenous therapy with combina-
monas aeruginosa and Escherichia coli. In tion broad-spectrum antimicrobials that
BMT patients, half the infections are bacterial, have excellent Gram-negative cover is
evenly split between Gram-positives (such as
Presentation essential in febrile neutropenia. The pattern
Staphylococcus aureus) and Gram-negative Young children compensate for impending and severity of infection varies according
organisms; 40% are due to viruses like shock and remain normotensive in the to location, clinical context, and level of

279
11.7 FEBRILE NEUTROPENIA

immune suppression. Regularly reviewed days. Although inpatient management is

neutropenia, it is known to reduce
institution- and scenario-specific treatment associated with an excellent outcome, care-
length of hospital stay, infection
protocols, developed in conjunction with fully selected children who are clinically well,
rate and neutrophil recovery
infectious diseases physicians and microbiol- with low-risk criteria, could be considered for
period.9,10
ogists, that take into account local patterns of early discharge or outpatient management
infection and their susceptibilities help to with daily intravenous ceftriaxone or oral The emergence of highly resistant
deliver and standardise optimal treatment. ciprofloxacin and daily reviews.2,46 and virulent organisms is increasingly
The Victorian Drug Committees Thera- Bone-marrow stimulation with colony- recognised as a barrier to improved
peutic Guidelines for Antibiotics3 is an excel- stimulating factors reduces the severity outcomes, especially in the context of
lent resource for centres that do not have and duration of neutropenia and fever, and immunosuppression.
their own febrile neutropenia protocols but antibiotic requirements in children under-
should ideally be adapted to local require- going BMT, and may be effective in prema-
ments. The recommended regimen in eTG ture infants at high risk of infection as well
2010 includes: cefepime or ceftazidime 2 g as children with severe neutropenia and References
(child: 50 mg kg1 up to 2 g) IV, 8-hourly Gram-negative sepsis. Corticosteroids are 1. Behrman RE, Kliegman RM, Jenson HB, editors. Nelson
textbook of pediatrics. 17th ed. Philadelphia: Saunders;
or piperacillin tazobactam 4 0.5 g effective in Pneumocystis carinii pneumonia 2004.
(child: 100 12.5 mg kg1 up to 4 0.5 g) and have been used in conjunction with 2. Holdsworth M, Hanrahan J, Albanese B, Frost J.
Outpatient management of febrile neutropenia in
IV, 8-hourly in children with no immediate immunoglobulin to treat immune-mediated children with cancer. Paediatr Drugs 2003;5:44355.
hypersensitivity to penicillin. Gram-positive neutropenia.7 3. Victorian Drug Committee. Therapeutic Guidelines for
Antibiotics. 14th ed. Victoria: Victorian Drug Committee;
bacteraemia from infected intravascular 2010 (etg32, November 2010).
devices require addition of IV vancomycin 4. Mullen CA. Ciprofloxacin in the treatment of fever and

(<12 years: 30 mg kg1 up to 1.5 g IV, Controversies and neutropenia pediatric cancer patients. Pediatr Infect Dis J
2003;22:113842.
12-hourly concentration monitoring) only challenges 5. Orudjev E, Lange BJ. Evolving concepts of management
of febrile neutropenia in children with cancer. Med
if the child is shocked, known to be Early goal-directed therapy, directed Pediatr Oncol 2002;39:7785.
6. Paulus S, Dobson S. Febrile neutropenia in children with
previously colonised with MRSA or has at optimising cardiac preload, afterload cancer. Adv Exp Med Biol 2009;634:185204.
catheter-related infection in a treatment and contractility to balance oxygen 7. Hann IM. Management of infection in children with bone
environment with high incidence of MRSA marrow failure. Baillieres Clinical Haematology
delivery with oxygen demand as part of a 2000;13:44156.
infection. Vancomycin is also indicated if a sepsis bundle multimodal algorithm, was 8. Rivers E, Nguyen B, Havstad S, et al. Early goal-directed
Gram-positive organism resistant to other therapy in the treatment of severe sepsis and septic
associated with reduced mortality in shock. N Engl J Med 2001;345:136877.
drugs is isolated from blood cultures or adults in a single-centre study which did 9. Sasse EC, Sasse AD, Brandalise S, et al. Colony stimulating
the child worsens clinically despite broad factors for prevention of myelosuppressive therapy
not include children;8 as such, it may not induced febrile neutropenia in children with acute
spectrum antibacterials for 48 h. be relevant in septic children requiring lymphoblastic leukaemia. Cochrane Database Syst Rev
If fevers persist in high-risk children after 2005;(3): CD004139.
further care in ICU. 10. Clark OA, Lyman G, Castro AA, Djulbegovic B. Colony
96 hours of antibacterial therapy, consider stimulating factors for chemotherapy induced febrile
empirically adding antifungal therapy after Granulocyte infusions are no longer neutropenia. Cochrane Database Syst Rev 2003;(3):
CD003039.
consultation with a paediatric infectious used because of questionable efficacy
diseases physician. Antibiotics can be dis- but may have a role in refractory
continued if there is clinical improvement, neonatal sepsis.
no proven infection, cultures remain nega-  Although colony-stimulating factor Further reading
tive, and neutrophil count improves to Chakravorty S, Hann IM. Management of infection in children
does not affect overall mortality in with bone marrow failure. In: Pediatric Hematology. 3rd ed.
500 mL1; this typically occurs at 3 to 7 children with cancer-related febrile London: Blackwell Publishing; 2006.

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 12

SECTION
DERMATOLOGY
Section editor Jeremy Raftos

12.1 Dermatology 281

12.1 Dermatology
Roderic Phillips David Orchard Mike Starr

ESSENTIALS Erythroderma and skin

failure
1 Look at the rash.
Erythroderma is one form of skin failure and
Are there any blisters? Finding fluid-filled lesions greatly narrows the range of refers to any condition that causes most of
possible diagnoses. the skin surface to become red, often with
Is the rash red? Redness is from haemoglobin. Most red rashes blanch, i.e. the some degree of scaling. Skin failure is rarely
redness disappears with pressure. If not, the haemoglobin is outside the blood taught as a concept but is analogous to
vessels giving purpura. failure of any other organ system. Skin fail-
ure refers to the inability of the skin to ade-
Is the rash scaly? If so, the epidermis is involved in the disease process. The quately perform its functions, such as fluid
epidermis may be broken to give weeping, crusting or fissures (eczematous and electrolyte balance, temperature control
rash), or it may be intact (red scaly rash). and protection from infection. Any child pre-
2 Examine all the skin including the anogenital region. senting with extensive areas of skin pathol-
ogy or skin loss has some degree of skin
3 Do not ignore a skin condition even if unrelated to the emergency visit. You may failure. This may be well tolerated in other-
not deal with it yourself but you can organise appropriate follow up for it. wise healthy children but may be life threat-
ening, especially in infants and those
children with associated disorders.
signs of disease, which may be relevant or may Children presenting with erythroderma
Introduction be coincidental to the childs presentation, for will usually be found to have one of the fol-
Approximately 65 000 children a year example, a 5-year-old boy (Fig. 12.1.1) who lowing: atopic eczema (with or without sec-
attend the emergency department (ED) at presents with gastroenteritis. You notice the ondary staphylococcal infection); an allergic
the Royal Childrens Hospital in Melbourne mild comedonal rash on his forehead and rec- reaction; or sepsis with toxin-mediated skin
and about 15 000 of these have some skin ognise that this is unusual at this age. Exami- involvement. Less common causes include:
findings relevant to their visit. For 4000 chil- nation shows that he is tall for his age and has psoriasis; pityriasis rubra pilaris; several
dren (6%), skin findings are a primary reason inappropriate penile (but not testicular) forms of inherited ichthyosis; some other
for their visit. These figures are likely to be growth. His hyperadrenergic state can be genodermatoses; and internal lymphoid
representative of other tertiary teaching investigated and treated years before it might malignancies. In 1020% of children with
hospital EDs in Australasia. Diagnosing otherwise have been recognised. erythroderma, no cause can be found.
and managing these children requires a The discussion of diseases in this chapter is
careful history and astute observation, par- based on the features of the presenting rash
ticularly focusing on the appearance, site and follows the algorithm given in Essentials. Management
and development of their rashes. Diseases are grouped under their main mor- The aim is to restore and maintain the
For children who present to the ED with the phology, e.g. vesicular, papular, eczematous, core functions of skin. Many sensible
primary symptom in another organ, the physi- or purpuric. Mouth, anogenital, hair and nail management decisions can be made
cian has an opportunity to observe cutaneous problems are considered separately. before the diagnosis is clear.

281
12.1 DERMATOLOGY

syndrome, immune-mediated blistering disor- assay for varicella-zoster virus DNA may
ders, arthropod bites, contact dermatitis, fixed be useful in suspected encephalitis.
and bullous drug reactions, mastocytosis, Pneumonia. Cough and respiratory
burns or trauma. distress begin a couple of days after
Many vesicles and blisters are fragile and onset of the rash.
rupture easily. A vesicular disease may present Cerebellar ataxia may appear as the rash
with a number of shallow monomorphic ero- is clearing and resolve within weeks.
sions but no vesicles. A bullous disease may
In neonates, immunocompromised children
present with several larger shallow erosions
and adolescents, varicella can be more
with surrounding loose epithelium but no bul-
severe and occasionally fatal.
lae. Vesicles present for more than a couple of
Fig. 12.1.1 Mild prepubertal acne on the
days become cloudy and vesiculopustular.
forehead of a 5-year-old boy who presented with Management
the unrelated problem of gastroenteritis. Treatment of otherwise well children with
Examination and investigation confirmed that
varicella is supportive with bland
he had a variant of congenital adrenal Varicella (chickenpox)
hyperplasia requiring treatment. emollients and oral antihistamine if
Varicella is caused by infection with varicella necessary. If the child is unwell, look for
zoster virus. It usually occurs in children less secondary complications.
Have a low threshold for admission to than 15 years of age, and is highly contagious. Avoid aspirin or other salicylates because
hospital.
The incubation period is 23 weeks. The con- of the association with Reyes syndrome.
Restore skin-barrier function by using
tagious period begins 2 days before the rash Treat secondary skin infection with
an ointment emollient frequently.
and continues until the lesions are crusted. cefalexin 30 mg kg
1 (max 500 mg)
Monitor temperature, as the child may
Clinical disease begins with a fever and mal- three times daily.
readily become hypothermic or, less
aise but these are usually mild. Lesions on Immunocompromised children should be
commonly, hyperthermic.
the skin appear initially as erythematous treated with intravenous aciclovir
Monitor fluids and electrolytes.
macules, rapidly becoming small vesicles 20 mg kg1 per dose (212 weeks),
Monitor cardiac output, particularly if the
and then crusting. Over the next few days, 500 mg m2 per dose (12 weeks12
child has known cardiac pathology.
anything from a few to hundreds of lesions years), 10 mg kg1 per dose (adult)
Skin and/or systemic sepsis may have
may develop, initially on the face and trunk 8-hourly given over 1 hour.
caused the skin failure or may be
and gradually spreading onto the extremities. Premature neonates (less than 1 month
secondary to it. Temperature control
Superficial ulcers may be seen on all mucosal old) with varicella should be treated with
may be affected, so fever may not be
surfaces. Itch is not always present but may intravenous aciclovir.
present. Have a low threshold for
be marked. Vaccinated children may develop Term neonates should be admitted, and
giving systemic antibiotics, particularly
attenuated disease with fewer lesions and treated with aciclovir if they have severe
to cover Staphylococcus aureus and
without constitutional symptoms. Diagnosis disease.
streptococcal species.
is clinical but can be confirmed by serology Give zoster immune globulin to at-risk
Local or systemic pain relief may be
or immunofluorescence of vesicular scrapings contacts (immunocompromised children,
required.
for varicella-zoster virus antigen. Complete those on prednisolone 2 mg kg1 day1
Cease all non-essential drugs.
resolution occurs in most children but some or more, newborns of mothers who have
Identify and treat the underlying cause.
scarring is common. varicella any time from 5 days before
Manage any associated conditions (e.g.
Children with varicella usually remain sys- until 2 days after delivery). Give within
diabetes, renal failure, congenital heart
temically well and afebrile after the first few 4 days of exposure (6 mL for adults, 4 mL
problems, metabolic disorders).
days. If persistent fever and malaise is present, for children 612 years of age, 2 mL for
search for complications. In previously well children up to 5 years of age).
VESICULOBULLOUS children, the most common complications are: Exclude from school until all lesions are
crusted.
RASHES Bacterial skin infection with group A
Streptococci or Staphylococcus aureus.
Offer varicella-zoster vaccine to non-
immune contacts the vaccine has been
Vesicles are usually caused by infections Look for bullous, indurated, spreading or
shown to prevent or attenuate infection if
(herpes simplex virus, varicella zoster virus, cellulitic lesions. Treat with oral antibiotics.
given within 5 days of exposure.
enterovirus, tinea, scabies or impetigo) or con- Central nervous system (CNS) dysfunction.
tact dermatitis. Also, consider drug reactions, Encephalitis, meningitis, transverse
erythema multiforme and photosensitivity. myelitis, GuillainBarre syndrome or Reyes
Dermatitis herpetiformis is a rare cause of
Zoster
syndrome (sudden onset of vomiting,
itchy vesicles and papules. Larger blisters coma and liver abnormalities) may occur. Zoster is uncommon in childhood but can
occur with staphylococcal infections, tinea, Cerebrospinal fluid (CSF) analysis, occur at any age, even in the neonatal
erythema multiforme, StevensJohnson including polymerase chain reaction (PCR) period. It occurs in children who have

282
12.1 DERMATOLOGY
12

DERMATOLOGY
previously had primary varicella infection. Oral antiviral agents can be considered in function, HSV infection can present as indo-
Zoster in a young child is usually associated older adolescents but are not generally lent, slowly growing, irregular ulcers. Most
with primary varicella having occurred in indicated in an otherwise well child as the HSV infections in children are caused by
infancy. The primary infection may have risk of significant symptoms or post- HSV-1, but HSV-2 may also cause gingival
been mild and may even have gone unno- herpetic neuralgia is very small. and cutaneous infection.
ticed because of maternally-acquired IgG. Primary cutaneous herpes simplex can
Radicular pain may be the first symptom of present at any age. There may be a history
zoster but pain is less common than in adults Hand, foot and mouth of a family member with a recently activated
and may not be present. Vesicles on an ery- disease herpes simplex lesion on the lip (cold sore).
thematous background appear in one or more However, the virus is ubiquitous and trans-
Hand, foot and mouth disease is one pre-
groups aligned in a dermatomal distribution, mission more often occurs from an unknown
sentation of enteroviral infection, usually
frequently with a striking midline cut-off. source. At least 50% of the population have
Coxsackie A16 virus. There may be a mild
The affected area can be an isolated group HSV-1 antibodies, and many of these have
prodrome of fever, malaise and sore throat.
of a few vesicles suggestive of herpes simp- never had an obvious infection. Painful
Discrete vesicles about 6 mm in diameter
lex virus infection. Alternatively, there may grouped vesicles on an erythematous base
on an erythematous base appear first in
be extensive involvement of one or more der- can appear at any site. Multiple lesions rup-
the mouth, sparing the lips and usually
matomes. Lesions continue to appear for a ture, crust and coalesce into larger erosions
sparing the gingiva. One to 2 days later,
few days and then resolve over 2 weeks, gen- with scalloped edges. There may be asso-
37 mm vesiculopustules on an erythema-
erally without sequelae. Postherpetic neural- ciated fever, malaise and lymphadenopathy.
tous base appear on the palms, soles and
gia is very uncommon after childhood zoster.
around the fingers and toes. Lesions are also
Complications include generalised dis- Management
often found on the buttocks. They are often
semination during the first week of the rash, Management consists of the following;
greyish and oval rather than circular in
sometimes with pulmonary or brain involve-
shape. In some cases, the mouth, hands or supportive care;
ment. This can be seen in both normal and
feet may not be involved. Resolution occurs bathing of crusts;
immunocompromised children. Zoster on
within a week. The incubation period is analgesia;
the head is occasionally accompanied by
about 5 days and epidemics are common. aciclovir is not routinely indicated unless
an aseptic meningitis, which resolves fully risk factors for complications exist (e.g.
Virus is excreted in the faeces for weeks
without treatment. Zoster lesions on the underlying disease, immunosuppression).
and exclusion is not recommended. Support-
tip of the nose imply involvement of the
ive care leads to complete recovery.
nasociliary branch of the ophthalmic nerve Recurrent cutaneous herpes simplex can
and may accompany ocular involvement occur weeks or months after the primary epi-
including keratitis and conjunctivitis. Facial sode. Recurrences usually get milder and less
Herpes simplex infection
nerve palsy and ear involvement can occur common with time but exacerbations can
(HSV)
(Ramsay Hunt syndrome). occur throughout childhood and later life
Immunocompromised children are much Herpes simplex infection in childhood is (Fig. 12.1.2). Recurrences can lead to signifi-
more likely to develop zoster. In such cases, zos- common. Intrauterine, intrapartum and neo- cant time off school. Antiviral prophylaxis
ter may be severe, extensive and prolonged. natal infection are considered elsewhere. Pri- may reduce the frequency and severity of
Significant dermatomal scarring can result. mary herpes infection after the neonatal recurrences, and should be used if recur-
period may be either primary herpetic gingi- rences are frequent or debilitating.
vostomatitis or primary cutaneous herpes HSV infection can involve the fingers, espe-
simplex. In children with impaired immune cially the thumbs and index fingers (herpetic
Management
If the diagnosis is unclear, collect epithelial
cells from the base and roof of the vesicles
for immunofluorescence and viral culture.
This is not just a swab and it requires some
persistence to collect enough cells.
In an otherwise well child, the occurrence
of typical zoster does not require
investigation for underlying
immunodeficiency states. However,
unusually severe or extensive zoster
A B
should raise this possibility.
If a child has underlying chronic illness or Fig. 12.1.2 (A) This erythematous, slightly vesicular rash around the eye of a 4-year-old boy had
immunodeficiency, give intravenous appeared over 12 hours. Because his other eye was blind, he was treated with oral aciclovir.
aciclovir or oral famciclovir or valaciclovir. Immunofluorescence confirmed herpes simplex virus. (B) One day later.

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12.1 DERMATOLOGY

whitlow). At these sites, the thick skin does After complete resolution, recurrences
not readily rupture and children present with may occur at the same or different sites, Impetigo (school sores)
painful, coalesced pustular collections, often sometimes as often as every few weeks. This is caused by Staphylococcus aureus or
misinterpreted as bacterial abscesses. Any Recurrences can be widespread and severe group A Streptococcus or both.
history of abscesses occurring at the same but usually decrease in severity over 1 to Non-bullous impetigo presents initially as
site on a finger on more than one occasion 2 years. small erythematous vesicles that rapidly
is suggestive of herpes simplex. Management rupture to form yellow-crusted lesions, com-
includes taking a swab for immunofluores- Management monly on the face.
cence and viral culture to confirm the diagno- Clinical differentiation between herpetic Bullous impetigo is due to Staphylococcus
sis, analgesia and supportive care. and bacterial infection may be difficult; aureus and presents as flaccid blisters on
investigation and empiric treatment for normal skin. Lesions are rounded and well
both may be necessary. demarcated and may be single, grouped or
Eczema herpeticum Collect epithelial cells from the base widespread. Their onset and spread may
and roof of the vesicles for HSV be rapid or occur over days. New blisters
Herpes simplex infection in children with immunofluorescence and viral culture. appear and existing blisters rupture to give
eczema is quite common. It occurs in chil- This requires some persistence to collect shallow moist erosions that can be many
dren with any severity of eczema including enough cells. centimetres in size. There is often loose epi-
children with mild eczema under excellent Local stable disease in an otherwise well thelium and/or brown crusting peripherally
control. Many cases are misdiagnosed either child requires regular observation but and some degree of central healing
as an exacerbation of the eczema or as bac- does not need antiviral therapy. (Fig. 12.1.4). In more chronic cases, lesions
terial infection (Fig. 12.1.3). Grouped vesi- Milder cases demonstrating progression may appear annular.
cles may be prominent, but more often or facial involvement can be managed Impetigo is often secondary to itchy condi-
vesicles are rudimentary or absent and the with oral aciclovir. tions such as scabies infection (especially
infection may present as a group of shallow Admission to hospital and treatment with hand impetigo), atopic eczema and head lice.
24 mm monomorphic erosions on an intravenous aciclovir (20 mg kg1 per Post-streptococcal glomerulonephritis may
inflamed base. In more severe cases, evolu- dose (212 weeks), 500 mg m2 per dose occur in the ensuing 2 months. Contrary to
tion is rapid and large crops of vesicles can (12 weeks12 years), 10 mg kg1 per long-held beliefs, it now seems likely that
arise daily. Ulcers may coalesce into larger dose (adult) 8-hourly should be chronic streptococcal impetigo may also be
erosions with scalloped edges. The infected considered for children with any of fever, a precursor of rheumatic heart disease in com-
area may not be painful or itchy. multiple sites of cutaneous herpes munities where medical conditions are poor
In an atopic child, a high index of suspicion infection, widespread eczema, eye and skin hygiene is suboptimal.1
is needed about any patch of skin where small involvement, immunosuppression or age
erosions or crusts are present and not less than 6 months.
responding to standard eczema therapy with Secondary bacterial infection is
moisturisers and topical cortisone derivatives. common. Swabs for microscopy and
One reason for the under diagnosis of this culture are usually unhelpful. If there
condition is that even without treatment, res- is any suspicion of bacterial infection,
olution usually occurs in 14 weeks. However, add intravenous flucloxacillin
dissemination may occur, leading to multi- 50 mg kg
1 (max 2 g) 6-hourly or oral
focal and extensive erosions, malaise and cefalexin 30 mg kg
1 (max 500 mg)
secondary bacterial infection. In the past, three times daily. Soak to remove
disseminated eczema herpeticum had a sig- excessive crusts.
nificant mortality but this has declined with Monitor for eye involvement, particularly
the recognition and aggressive treatment of scleral redness. If present, this should
secondary bacterial infections. be managed with topical or systemic
aciclovir, or both, and urgent review by
an ophthalmologist.
The underlying eczema can be treated
with moisturiser or wet dressings until the
herpetic scabs have been removed and
then it is important to restart topical
cortisone treatment.
Antiviral prophylaxis may reduce the
Fig. 12.1.3 Eczema herpeticum. Typical
frequency and severity of recurrences and
monomorphic vesicles can be seen away from is often warranted in older children with Fig. 12.1.4 Bullous impetigo in a 7-year-
the central weeping area. recurrent eczema herpeticum. old girl.

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12.1 DERMATOLOGY
12

DERMATOLOGY
Management involved. Increase oral (or intravenous) are an uncommon cause. Erythema multi-
Swab for microscopy and culture if the fluids to increase toxin excretion. Give forme does not evolve into StevensJohnson
diagnosis is unclear or in widespread or analgesia. syndrome. They are different conditions.
complicated disease. Consider alternative diagnoses including
Bathe off crusts. StevensJohnson syndrome and toxic Management
Apply topical mupirocin 2% ointment epidermal necrolysis. If the diagnosis is Fluid maintenance.
8-hourly if very localised, or oral cefalexin unclear, a skin biopsy will confirm the Analgesia if required.
30 mg kg
1 (maximum 500 mg) three diagnosis by showing the split is in the Apply emollient ointment to the lips, if
times daily if extensive. granular layer in the upper epidermis. needed.
Isolate the child from other children or Flucloxacillin 50 mg kg1 (maximum 2 g) If the condition is recurrent, it is highly
from sick adults unless all lesions are IV 6-hourly if there is evidence of sepsis or likely to be related to herpes simplex
covered or antibiotic treatment has systemic involvement. virus. Prophylactic aciclovir prevents
commenced. Look for a focus of infection. Drain any recurrences and should be considered if
Treat any underlying condition such as foci of pus if present. recurrences are frequent, severe and
scabies or eczema. Handle skin carefully and use an affecting the quality of life.
Blisters or erosions often continue to occur emollient ointment. Prednisolone alleviates symptoms but
for a couple of days after antibiotic therapy. probably prolongs the duration of the
If new blisters continue to form after this condition and may cause recurrence of
time, they may be caused by resistant Erythema multiforme herpes simplex virus infection.
Staphylococcus aureus infection, or the
This is a specific hypersensitivity syndrome
child may have an immune-mediated
blistering disorder rather than impetigo.
that occurs at any age, often preceded by StevensJohnson
Children with chronic impetigo should be
facial herpes simplex virus infection. It is over- syndrome/toxic epidermal
examined for any signs of heart disease.
diagnosed in emergency departments. Most necrolysis
children diagnosed with erythema multiforme
Families should be aware that rheumatic StevensJohnson syndrome and toxic epider-
actually have urticaria, often with large
heart disease may be delayed by some mal necrolysis are believed by many to be
lesions with annular or polycyclic borders. In
years in this setting. Older siblings are variants of the one condition. Most cases
erythema multiforme, the primary lesions
likely to have had chronic impetigo in the are thought to be triggered by medications,
are red papules, usually symmetrical and
past and therefore to also be at risk of especially antibiotics (sulfonamides, peni-
involving the forearms, palms, feet, face, neck
heart disease and should be examined. cillins), anticonvulsants (lamotrigine) and
and trunk. They can be found anywhere.
In any child with chronic impetigo where non-steroidal anti-inflammatory drugs. Myco-
There may be few or many lesions. At least
ongoing care may be suboptimal, review plasma and other infections may be the cause
some of the papules will form classical target
with urinalysis to monitor for in some children. Fever, myalgia, arthralgia,
lesions these have an inner zone of epider-
glomerulonephritis is warranted. In other headache and other organ involvement are
mal injury (purpura, necrosis or vesicle), an
cases, families should be aware of the usually present as a prodromal illness for
outer zone of erythema and sometimes a
possibility of renal involvement. several days. The rash evolves suddenly,
middle zone of pale oedema (Fig. 12.1.5).
These papules and target lesions are not characterised by widespread blisters on an
erythematous or purpuric macular back-
Staphylococcal scalded migratory. The involvement of mucous
ground, often with extensive mucous mem-
skin syndrome membranes is common but unlike Stevens
Johnson syndrome, is limited to isolated brane haemorrhagic crusting. Lesions are
This is usually seen in younger children or patches. Most cases are caused by herpes usually on the face and trunk or generalised
children with renal impairment. It is mediated simplex virus, including most of the cases rather than acral, and typical target lesions
by an epidermolytic toxin released from an that occur without prior symptoms. Drugs are not seen. There may be tender erythema-
often insignificant staphylococcal focus (e.g. tous areas with a positive Nikolsky sign
eyes, nose or skin). Fever and tender erythem- (normal skin separates if rubbed). Mucous
atous skin are early features. Exudation and membrane involvement can be extensive,
crusting develop, especially around the severe and painful. Conjunctivitis, corneal
mouth. Wrinkling, flaccid bullae and exfolia- ulceration and blindness can occur. Anogen-
tion of the skin are seen and can be extensive. ital lesions can lead to urinary retention.
Nikolsky sign is present (normal skin sepa-
rates if rubbed). Blisters are very superficial Management
and heal without scarring. See general advice under skin failure.
Admit to hospital. A multidisciplinary
Management approach including dermatology,
Admit to hospital. Monitor temperature, paediatrics, ophthalmology, surgery and
fluids and electrolytes if large areas are Fig. 12.1.5 Erythema multiforme target lesions. paediatric intensive care is required. Good

285
12.1 DERMATOLOGY

nursing care is paramount. In severe Management

cases, nurse in a specialised burns unit. Perilesional skin should be biopsied for Sunburn and
Apply emollient ointment to the skin, lips immunofluorescence to demonstrate the photosensitivity
and anogenital areas this may be diagnostic granular IgA deposition. Excessive solar radiation to the skin causes
required many times a day. Investigate for coeliac disease with total erythema and tenderness commencing at
Regular eye examination with specialist IgA, anti-tissue transglutaminase IgA and least half an hour after the beginning of
review is required. Topical steroid eye anti-deamidated gliadin IgA. Anti- the exposure. Tenderness worsens for a
drops may be needed. transglutaminase antibodies are the day and resolves in 4 days. In more severe
Give IV immunoglobulin 2 g kg1. most specific for dermatitis herpetiformis. cases, oedema and blistering may be wide-
Commence ciclosporin (56 mg kg1 A small bowel biopsy is required but spread. Healing is accompanied by desqua-
day1 orally for a few days, then taper to may be equivocal early in the disease mation and intense itch. Sunburn occurs
35 mg kg1 day1 for 23 weeks). Data course. more rapidly when the sun is overhead (sum-
about the efficacy of any treatment Anaemia, usually megaloblastic mer, closer to the equator, 11 a.m. to 3 p.m.),
regimens are limited but are sufficent to secondary to malabsorption, may be and when the skin is less pigmented, but
justify treatment. StevensJohnson found. can occur with any skin type. Children with
syndrome has a significant mortality and
Skin lesions (but not intestinal symptoms) sensitive skin can burn significantly within
has a high rate of permanent visual
respond well to dapsone, but haemolysis 15 minutes of midday exposure in summer.
scarring including blindness as well as
and other side effects can limit its use. A Any child presenting to the ED with sun-
other problems.
gluten-free diet normalises gut function burn or with a rash in sun-exposed areas
Maintain good nutrition, with nasogastric
and, in most children, leads to eventual may have an underlying photosensitivity dis-
feeding if needed.
clearing of skin lesions. order. These can be considered in four groups.
Intubation may be needed if the child is
unable to protect the airway because of Primary photosensitivity disorders.
severe oropharyngeal involvement. Inherited disorders including porphyrias.
Other immune-mediated  Diseases with a photosensitive
Note: StevensJohnson syndrome is not blistering disorders component (systemic lupus
severe erythema multiforme. They are dis-
As with dermatitis herpetiformis, the many erythematosus, dermatomyositis).
tinct conditions. Permanent sequelae are
diseases in this group are characterised by Exogenous photosensitivity from drugs
rarely seen in severe erythema multiforme
circulating autoantibodies directed against or plants.
and concurrent drug use is unlikely to be
one or other of the structural proteins that
the cause. Skin lesion distribution and mor- A high index of suspicion is needed to diag-
give the epidermis its integrity. All are
phology are the best discriminating factors. nose photosensitivity. There have been
uncommon in childhood, linear IgA dermato-
Mucous membrane involvement can be seen examples of sunburn in children that were
sis (chronic bullous disease of childhood)
in both conditions but is confluent in attributed to parental neglect but were
being the most common. An immune-
Stevens-Johnson syndrome. actually due to a photosensitivity disorder.
mediated blistering disorder should be con-
These diseases are common but diagnostic
sidered in an otherwise well child if vesicles,
traps abound. For example, sun-induced
blisters, or crusted erosions continue to
Dermatitis herpetiformis appear for more than a couple of weeks.
rashes may develop in spring with the first
sun exposure of untanned skin after winter,
Dermatitis herpetiformis is an uncommon If there is a split below the basement
but not later in summer. The rash may
autoimmune blistering disease that may membrane, lesions may be tense, long-
develop on areas usually covered in winter
present at any age as itchy papules or vesi- lasting bullae, as seen in dermatitis herpeti-
such as the neck and arms and not on the
cles. Rapid excoriation means that intact formis, linear IgA dermatosis, systemic lupus
face. Sun-induced rashes may require a few
vesicles are rarely seen. Lesions occur on erythematosus and pemphigoid. If the split
minutes or several days of sun exposure
extensor surfaces of the limbs, buttocks, is higher in the epidermis, lesions are flaccid,
and may commence days after the exposure.
trunk and scalp. Most patients have gluten short-lived blisters that rapidly give crusted In erythropoietic protoporphyria, sun expo-
enteropathy (coeliac disease) and may have erosions with loose epithelium around the
sure induces pain without skin changes initi-
abdominal discomfort, diarrhoea or anae- edges. These are seen in pemphigus var-
ally. Viral exanthems may occur exclusively
mia. Some degree of villous atrophy will be iants. Lesions can mimic local or generalised
or mainly in areas of sun-exposed skin. Chil-
seen on small bowel biopsy in most cases, impetigo. Mucous membranes may be
dren with solar urticaria develop urticaria on
but coeliac disease is often asymptomatic involved. Despite the widespread and often
sun-exposed areas.
at the time of presentation of dermatitis her- dramatic appearance of the rash, the child
petiformis. Growth retardation has been is usually well. Accurate diagnosis always
reported with dermatitis herpetiformis, prob- requires histology and immunofluorescence Management
ably secondary to intestinal involvement. in addition to clinical findings. Management Assess for associated causes (below) of
Recurrence some years after successful requires immunosuppressive therapy and increased sun sensitivity. Many children
treatment has been reported. long-term follow up. presenting to the ED with sunburn have

286
12.1 DERMATOLOGY
12

DERMATOLOGY
an associated photosensitive trigger. Any vesicles on the face, ears, neck or arms. It dorsum of the hands. Progressive scarring
child who experiences symptoms within is recurrent. Recurrences usually occur at can be severe.
half an hour of sun exposure should be the same sites in that child.
assumed to have an underlying condition. Familial porphyria cutanea tarda Pre-
Assess the family situation, especially Juvenile spring eruption Is a specific sents in childhood with chronic blistering
with younger children. Significant variant of PMLE and is common in 412- on hands, arms and face leading to poorly
sunburn in a young child can occur in the year-old boys as a recurrent blistering of healing ulcers, atrophic scarring and mottled
setting of suboptimal child care, although the ears each spring. hypo- and hyper-pigmentation.
usually it is due to an oversight by If a porphyria is suspected, measure
otherwise caring parents. blood, urine and faecal levels of porphyrins.
Hydroa vacciniforme Is rare and can
Within the first few hours of exposure,
begin at any age in childhood as a sun- Management requires intensive avoidance
before blistering has developed, of solar radiation including UVA and UVB.
induced vesicular eruption on the cheeks
application of topical potent steroid b-Carotene is useful in erythropoietic
during spring and summer. The cheeks, ears,
creams can considerably reduce the protoporphyria.
nose, dorsum of the hands and rarely the eyes
severity and duration of symptoms. Many genodermatoses including xero-
can be involved. Recurrences occur for many
Cool compresses and wet dressings will
years. derma pigmentosum, Cockayne syndrome,
alleviate symptoms. Topical anaesthetics Rothmund-Thomson syndrome and tri-
Diagnosis can be difficult and may require
should not be used. Admission and oral chothiodystrophies are associated with photo-
formal evaluation in a photobiology unit
analgesia may be required in severe sensitivity and increased photo-damage to
including assessment of the response to
cases. Maintain adequate fluid intake. skin, but do not usually present with blistering.
different wavelengths of light. Management
Educate about prevention.
requires sun avoidance and often both UVA
and UVB sun protection.
Prevention Photosensitivity and
Prevention of sunburn is important. Epi- bullous reactions to drugs
Solar urticaria Presents as urticaria
sodes of sunburn are linked to later develop-
within minutes or hours of exposure and set-
ment of naevi and melanoma. Blisters may be the presenting feature of a
tles within 1 day. Sharp margins are seen
Parents need to balance the psychological number of different types of drug reactions.
at the edges of clothing. Skin chronically
and physical benefits of activities associated A high index of suspicion is required to diag-
exposed to the sun, such as face and hands,
with sun exposure (including increased fit- nose these conditions. Families must be
is often spared.
ness, increased independence, healthier asked about all ingested and topical pro-
bones and decreased obesity) with the ducts including herbal, recreational and pre-
increased risk of skin cancers of all types. In scription drugs and unusual food patterns
summer, children should minimise sun expo- Porphyrias and other (e.g. daily cups of celery juice). Families will
sure during the middle 4 hours of the day, inherited disorders with occasionally not remember the causative
wear broad-brimmed hats and long-sleeved photosensitivity drug and deny taking anything, only to
shirts in the sun, and use a sunscreen with recall the vital information days later.
The porphyrias are a group of inherited
SPF 30 or greater on exposed parts. Many medicines can cause increased sun
enzymatic defects in haem synthesis leading
Sunburnt infants regularly present to the sensitivity leading to erythema, oedema
to increased levels of porphyrins, some of
ED, partly because of widespread advice and blistering on sun-exposed areas. Doxycy-
which cause photosensitivity.
that sunscreens are not recommended cline (particularly at doses higher than
below the age of 6 months. This recommen- 100 mg day1), tetracyclines, griseofulvin,
Erythropoietic protoporphyria Is the isotretinoin, non-steroidal anti-inflammatory
dation is not based on any evidence of pro-
most common childhood porphyria. Infants drugs, sulfonamides, fluoroquinolones and
blems in infants. It is based on the premise
and young children present after brief sun diuretics are typical causes in children. In
that it is usually easy to protect young
exposure with acute discomfort, burning one series of children receiving naproxen,
babies who cant crawl by keeping them
sensations, itching, erythema, oedema, urti- 12% developed photosensitivity reactions
out of the sun and appropriately dressed.
caria and occasionally vesicles, particularly on the face. Other causes include tars,
However, if a young baby is going to be in
on the face and dorsum of the hands. Epi- perfumes, cosmetics, sunscreens, artificial
the sun, sunscreen should be used.
sodes are recurrent and with time affected sweeteners and many dyes. As with other
skin appears prematurely aged. Erythrocyte photosensitivity syndromes, the rash can
protoporphyrin levels are elevated. occur rapidly or days after the sun exposure
Primary photosensitivity and is most prominent on sun-exposed areas.
disorders Congenital erythropoietic porphyria The photosensitivity can persist for up to
Polymorphous light eruption (PMLE) Is rare and presents in early childhood with 3 months after withdrawal of the medication.
Presents in spring or early summer as skin- extreme photosensitivity leading to painful Prominent hyperpigmentation may persist for
coloured or erythematous itchy papules or blisters filled with red fluid on the face and months after resolution.

287
12.1 DERMATOLOGY

Fixed drug eruptions in children are usu- and a severe generalised itchy vesicular rash
ally caused by paracetamol, non-steroidal Contact dermatitis plants on an adolescent using topical neomycin
anti-inflammatories or sulfonamides. They In children, contact dermatitis reactions ointment.
are quite common and present as single or from agents other than plants usually do
multiple, usually circular red patches that not blister and these conditions are dis-
may blister. With subsequent exposures, cussed under eczematous rashes (p. 297). Isolated blisters
eruptions recur at the same site and some- Many plants, including Rhus and Grevillea
times at other sites. Post-inflammatory For a child who presents with a single blister
species, can cause an allergic contact derma-
hyperpigmentation is prominent and may or a few blisters as an isolated finding,
titis. Some species only cause reactions at
be the only sign of the drug reaction. consider:
specific times of the year and contact with
Many plants and herbal products contain the plant at other times does not cause a Mastocytoma. Usually in an infant with a
chemicals that can induce drug reactions rash. One to 3 days after exposure, erythema, history of recurrent blistering or crusting
including fixed drug reactions, bullous drug oedema and vesiculation develop at sites of at the site of a brownish macule, often
reactions and photosensitivity reactions. contact, often in linear distribution. Lesions misdiagnosed as recurrent localised
may persist for 3 weeks. Periorbital erythema impetigo. There may be one brownish
Management
and vesiculation is often misdiagnosed in lesion or as many as hundreds.
Recognise and stop the causative drug. the ED as cellulitis needing antibiotics. Insect bite. There may be a few lesions in
Institute strict sun protection measures. Clues to the correct diagnosis are that pruri- one area. Non-blistered papules may
Photosensitivity can persist for 3 months
tus is the main symptom, the degree of have a tiny central red bite punctum.
after cessation of the drug.
pain and tenderness is much less than Irritant contact dermatitis.
If the patient is taking multiple drugs, the
timing of the eruption and the frequency
would be expected for cellulitis, systemic Spider bite. These can grow over days to
features, such as fever, are absent, the out- become a non-tender blister with a
of reactions with each of the medications
line of the rash often has irregular patterns diameter of many centimetres. They are
may suggest the causative drug.
corresponding to the contact areas and a uncommon. Regular dressings may be
If continued drug ingestion is necessary,
careful examination will often reveal other required. Debridement is rarely necessary.
reducing the dosage may prevent the
lesions on sensitive parts of the body. In par- White-tailed spiders and huntsman spiders
reaction.
ticular, genital skin may develop lesions do not cause chronic necrotic ulcers.
from secondary spread of the allergen via Scalds or burns from cigarettes or other
the fingers. Patients with allergic contact hot objects. Look for characteristic
Photosensitivity reactions
dermatitis may be extremely uncomfortable. patterns in the burn, as well as other
to plants
Treatment often requires a few days of oral factors on the history or examination
Many plants contain furocoumarins, which prednisolone, topical potent steroid oint- suggestive of non-accidental injury or
are naturally occurring psoralens. UVA light ments, cool compresses and supportive care. suboptimal caring practices.
induces covalent bonding of psoralen into Unlike plant-induced photosensitivity, con- Fixed drug eruptions, e.g. from
DNA, leading to cell death. Typically, a child tact dermatitis from plants does not induce tetracyclines, sulfonamides, non-steroidal
is playing outside in spring or summer and long-term hyperpigmentation. anti-inflammatory drugs or paracetamol.
comes into close contact with a psoralen- Irritant contact dermatitis to components Friction. If minor friction appears to cause
containing species (e.g. species of celery, of stinging nettles, chilli peppers, mustard, more blistering than expected on hands
parsley, parsnip, fig, hogweed, limes and other horseradish and other plant products can and/or feet in children, consider the
citrus fruits). Several hours later, erythema, lead to irritation, stinging, and occasionally possibility of a mild inherited blistering
oedema and occasionally blistering develop. blisters. Burning, oedema and blistering in disorder (e.g. mild epidermolysis bullosa
While contact dermatitis from plants can also the oral cavity can occur in small children simplex). There may be a positive family
present with streaky, often linear vesicular after chewing irritant plants. history of childhood blistering that settled
eruptions on areas of contact after exposure, in later life.
photosensitivity reactions are more often Artefactual lesions caused by either the
painful, are usually sharply limited to sun Contact dermatitis id child or a carer.
exposed areas, and typically heal to leave reactions Bullous Sweet syndrome or pyoderma
striking patterns of hyperpigmentation that gangrenosum.
can persist for months or years. Repeated exposure to an allergen can result
in a papulovesicular eruption at sites distant
Management from the area of contact. This is thought
Chronic erosions or ulcers
Sun protection measures including to be a result of systematised contact sensi-
a UVA sunscreen will prevent tisation. The id reaction may be local or gen- Several primary skin conditions can cause
phytophotodermatitis. eralised. Examples include non-infective chronic erosions or ulcers in children, includ-
Offending plants can be removed. blisters erupting on the hands of a child ing itchy conditions such as scabies and
Treat as for sunburn. with chronic tinea infection of the feet, papular urticaria. Also consider:

288
12.1 DERMATOLOGY
12

DERMATOLOGY
Immunodeficiencies. Recurrent boils can Ichthyosis. Bullous subtypes present as
be seen in chronic granulomatous disease blisters or erosions on red skin, which
and hyper IgE syndrome. Poor wound gradually becomes more scaly.
healing is a feature of leucocyte adhesion Langerhans cell histiocytosis vesicles
defects. and/or purpuric crusted lesions
Skin fragility syndromes. In junctional and (see p. 294).
dystrophic forms of epidermolysis bullosa, Incontinentia pigmenti. This disease is
chronic ulcers related to minimal skin X-linked and fatal in male fetuses. Girls
trauma may be seen in association with present with lesions distributed in linear
failure to thrive, anaemia and patterns. Lesions evolve through vesicular
gastrointestinal tract involvement. and warty phases to eventually leave
Porphyria. Several enzyme defects in permanent hyperpigmented streaks.
haem metabolism are associated with Seizures and developmental, ocular
chronic erosive lesions, photosensitivity and dental problems may occur.
and hyperpigmentation. Episodes of Mastocytosis. Urtication and blistering
acute pain or neurological dysfunction occur spontaneously or on top of oval
are rarely seen in childhood porphyrias. brown macules.
Artefactual lesions caused by either the Fig. 12.1.6 Cystic acne. Acne should be
child or a carer. treated with isotretinoin before reaching this
stage.
Pyoderma gangrenosum.
Mycobacterium ulcerans infection.
PUSTULAR RASHES
Consider acne, folliculitis, drug reactions, Management
pustular psoriasis, scabies, perioral dermati- Most cases are mild and either require no
Neonatal vesicles tis, tinea and localised bacterial infection. intervention or can be managed with
All vesicular rashes can become pustular if topical treatments. A first-line topical
A neonate with vesicles or blisters requires the vesicles persist for more than a couple treatment is benzoyl peroxide 2.5%
urgent assessment, as potential causes of days. Vesicles on areas of thick skin can applied once or twice a day. Warn the
include infection with herpes simplex virus, be white and look like pustules, e.g. on the adolescent that improvement with
varicella zoster virus and Staphylococcus toes in hand, foot and mouth disease. If in topical therapy occurs steadily over
aureus, including bullous impetigo. Neo- doubt, prick one lesion to reveal the clear 14 months (not in a few days).
nates who acquire herpes simplex virus at fluid within. Other topical agents include topical
birth usually present at a few days of age
antibiotics for inflammatory lesions
with grouped vesicles, often on the scalp.
(clindamycin, erythromycin or
Lesions may rapidly spread and coalesce. Acne tetracycline), topical retinoids
Collect epithelial cells from the base and
Acne mainly affects the forehead and face but (isotretinoin, adapalene) or azelaic
roof of a vesicle for immunofluorescence
can involve other sebaceous gland areas acid. These can be used singly or in
and viral culture. This is not just a swab
(neck, shoulders and upper trunk). Early combination and combination
and it requires some persistence to collect
lesions include blackheads, whiteheads and preparations are becoming more
enough cells. CSF must be examined for her-
papules. In more severe cases there may be available. All of these topical agents have
pes simplex virus DNA, as 30% of neonates
pustules or inflammatory cysts that can lead the potential to cause irritation.
with apparently localised skin disease also
have CNS involvement. Empiric intravenous
to permanent scarring. Acne is common in If there are prominent red papules or
adolescence. It is treatable and no person pustules, a course of oral antibiotics for
aciclovir is essential until herpes infection
with acne should be told it is an inevitable 36 months in combination with a topical
has been excluded.
part of adolescence. Several topical and oral treatment is warranted (e.g. tetracycline
Non-infective causes of neonatal vesicles
acne therapies are available and should be 500 mg twice daily, erythromycin 500 mg
include:
used to treat the disease. Under-treated acne twice daily, doxycycline 50200 mg
Epidermolysis bullosa. Over 40 inherited is a major medical cause of significant morbid- day1). Oral hormone therapy can help
diseases are associated with skin fragility. ity in adolescents and has a recognised mor- female patients.
Presentation ranges from a few isolated tality as a factor in teenage suicide. It can If the acne is severe, or if antibiotics and
blisters in otherwise well children to also lead to permanent scarring in 10% of topical treatment have not resulted in
extensive blistering at any site of untreated cases (Fig. 12.1.6). Significant acne considerable improvement in 3 months,
handling, with associated systemic in an adolescent attending the ED for another oral isotretinoin (Roaccutane) is
problems. reason should not be ignored. The adolescent indicated. Prescription of isotretinoin is
Miliaria. Very superficial blistering in hot should be offered information, initial treat- tightly controlled because it is expensive
or occluded areas. ment and referral for ongoing management. and because it is teratogenic, requiring

289
12.1 DERMATOLOGY

absolute avoidance of any pregnancy risk Oral isotretinoin being added to the antifungals may be required (see
in girls. In boys, and in girls who do not regimen at the fourth week, initially at Folliculitis below).
become pregnant, it is safe and highly 0.5 mg kg1 daily and gradually Androgen excess. Other features
effective. Most adolescents with increased to achieve complete depend on the age and sex of the
troublesome acne can be cured with oral clearance. child. In younger children accelerated
isotretinoin. growth, body odour, pubic hair,
Some acne sufferers develop scarring. clitoromegaly (but not breast
This can be quite subtle and can occur development) and penile (but not
with otherwise fairly mild disease. Any Acne with Gram-negative testicular) enlargement may
scarring is an indication for oral folliculitis be present. In teenage females,
antibiotics and early consideration of This can develop in patients treated for acne polycystic ovary syndrome is commonly
oral isotretinoin. Similarly, the presence with long-term antibiotics. It is more com- the cause. Other causes include late-
of any cysts should alert you to the mon in adolescents with an oily complexion. onset congenital adrenal hyperplasia,
likelihood of scarring and the need for It presents as rapidly worsening acne with and virilising tumours. Investigations
oral therapy. pustular lesions on the face. Several com- may include X-rays for bone age,
mon Gram-negative pathogens may be pelvic ultrasound, plasma androgens,
involved. Treat with oral trimethoprim and fasting glucose and insulin, and
oral isotretinoin. response to dexamethasone suppression.
Acne and depression Precocious puberty.
Several drugs can induce an acneiform
Both adolescence and acne can be associated
rash with monomorphic papules and
with depression. Significant depression is an Early onset acne
pustules in post-pubertal children. The
indication to consider oral isotretinoin ther- Acne normally appears after the onset of distribution is different from acne
apy. For several years, the issue of whether iso- pubertal changes such as testicular enlarge- vulgaris, often involving arms, legs or
tretinoin can trigger depression and suicide ment, pubic hair and breast development. trunk, and comedones are not present.
has been investigated. Current medical evi- However, comedonal and occasionally papu- Affected individuals often have a history
dence suggests that treatment of severe acne lar lesions may be the first manifestations of of troublesome acne vulgaris. Possible
with oral isotretinoin may decrease the risk of puberty in a child. These changes can begin causative agents include anti-epileptic
suicide. Assessment and treatment of any up to 2 years before other signs of puberty. medications, especially phenytoin and
depression is required. Early onset of comedonal lesions, even if phenobarbital, glucocorticoids,
mild, is associated with more severe acne testosterone, isoniazid, lithium and
several years later. iodides.
Acne fulminans If comedones or acneiform papules are Aperts syndrome and related craniofacial
noted before puberty (see Fig. 12.1.1), you syndromes can present with more severe
Acne fulminans typically occurs in young should consider the possibility of pathologi- widespread acne.
males being treated for acne. There is sudden cal androgen secretion (see below). Look for
onset of fever, malaise, arthralgia, myalgia, increased growth, genital development and
lymphadenopathy and/or hepatosplenome- advanced radiological bone age.
galy in association with a rapid worsening Folliculitis
of acne over the trunk and shoulders. Many
Infection of the hair follicles is usually due to
painful cystic lesions develop and become
Atypical acneiform rashes Staphylococcus aureus. Predisposing factors
haemorrhagic and ulcerated. Many labo-
include a moist environment, a heavy bacte-
ratory abnormalities have been noted, Acne that is atypical in age of onset, distri-
rial load (e.g. adjacent to wounds or anal
including elevations of white cell count, eryth- bution, morphology or severity may be asso-
region) and excessive occlusive ointment,
rocyte sedimentation rate, C-reactive protein ciated with systemic disease. Consider:
particularly on the trunk. Treatment involves
and liver enzymes. The cause is unknown but
Glucocorticoid excess, either exogenous avoidance of the predisposing factors, use
may be an abnormal immunological response
or endogenous. This can give a of topical cleansers such as chlorhexidine
rather than a primary bacterial infection. The
monomorphic acneiform rash on the face and, rarely, oral antibiotics. Folliculitis often
response to oral antibiotics is slow and indeed
and trunk. Other cushingoid stigmata occurs repeatedly over a period of months
these adolescents are often taking oral anti-
may be present. In children on and may require continued topical measures
biotics at the time of onset.
corticosteroid treatment, the rash may for 36 months. In resistant cases, daily use
appear as the dose of corticosteroid is of topical benzoyl peroxide lotion may be
Management being weaned. Glucocorticoid acne may helpful.
Oral prednisolone 0.51 mg kg1 daily be due to Malassezia folliculitis. If there is Folliculitis can occur in a child with mod-
for 46 weeks (thereafter slowly reduced no response to tetracycline, treatment of erate or severe eczema. Oral antibiotics
to zero). Malassezia folliculitis with topical or oral are usually beneficial in this setting.

290
12.1 DERMATOLOGY
12

DERMATOLOGY
In adolescents, Malassezia folliculitis is arthralgias with widespread pustules coa- infection can occur. Mid-potency topical
common and presents as a persistent itchy lescing into sheets of pus. Local pustular pso- corticosteroids are helpful to control itch.
rash with many tiny monomorphic erythem- riasis can be treated topically (see psoriasis, Milia (white papules on the face) and
atous papules and pustules on the back, p. 295). Generalised pustular psoriasis sebaceous hyperplasia (yellowish
shoulders and upper trunk. It is often asso- requires admission to hospital, rest, skin papules, particularly around the nose)
ciated with occlusion or sweating. Daily emollients and/or wet dressings, monitoring can be misinterpreted as pustules. Both
application of ketoconazole 2% shampoo of fluid balance, electrolytes, renal and car- resolve within a few weeks of birth.
is effective in gaining control. For resistant diac function and oral therapy (see erythro- Neonates with Downs syndrome can
cases, use oral ketoconazole 400 mg weekly derma p. 281 and psoriasis p. 295). develop a leukemoid pustular reaction
for 6 weeks taken with a glass of grapefruit which is self-limiting.
juice followed by indefinite weekly sham-
pooing with selenium sulfide 2% shampoo. Neonatal pustules
Occasionally, pruritic papules and pus- Neonatal pityrosporum
In the neonatal period, pustules may be part
tules can develop many hours after soaking folliculitis
of several transient benign conditions. Pus-
in a hot spa. Lesions usually occur under the
tules or vesicles may also be a marker of seri- This extremely common eruption is referred to
bathing costume and are caused by Pseudo-
ous underlying illness, even in the absence as milk spots, the 6-week eruption or infan-
monas aeruginosa. Oral antibiotics are not
of fever and lethargy. Consider: tile acne. It presents as a widespread papulo-
usually needed.
Infection, either congenital or acquired, pustular follicular eruption on the face and
e.g. Staphylococcus species, Group B torso. This is not true acne and comedones
streptococci, Haemophilus influenzae, are not present. It is due to an inflammatory
Acute generalised reaction to Pityrosporum yeast as the new-
Candida species.
exanthematous pustulosis born sebaceous glands, activated during preg-
Neutropenia from any cause. Superficial
(AGEP) bacterial pustules may be the only sign of nancy, are returning to their quiescent
AGEP appears as a rapidly developing facial congenital neutropenia. childhood state. It is self-limiting and often
or truncal erythema with hundreds of tiny Toxic erythema of the newborn. In this needs no treatment. In more severe cases,
sterile pustules. Oedema is often seen in benign condition, well term babies application of ketoconazole 0.2% solution
the affected areas. Fever and malaise may develop many tiny papules or pustules on (dilute the 2% shampoo) by cotton bud to
be present. Healing is associated with exten- an erythematous macular background in affected areas twice daily leads to rapid
sive superficial peeling. In some cases, chil- the first few days of life. Lesions clear clearing.
dren presenting with acute generalised within several days.
exanthematous pustulosis have an underly- Transient neonatal pustular dermatosis.
ing psoriatic tendency. Loose pustules are present from birth, PAPULAR (RAISED)
Many common medications, includ- and disappear within two days. This RASHES
ing amoxicillin, erythromycin, azithromycin, condition is more common in dark-
sulfonamides, anticonvulsants (especially skinned babies. If a child has itchy papules, consider scabies,
carbamazepine) and non-steroidal anti- Acropustulosis of infancy. Itchy vesicles urticaria, serum sickness, papular urticaria,
inflammatory drugs can cause this. Acute and pustules appear on the hands and molluscum, Malassezia folliculitis, dermatitis
generalised exanthematous pustulosis drug feet, usually commencing in the first herpetiformis or Langerhans cell histiocytosis.
reactions often begin within hours of com- couple of months of life and continuing If papules are not itchy, consider urticaria,
mencing the drug. Other causes include for some months. New crops appear each molluscum, warts, acne, skin appendageal
Group A streptococcal infection. 24 weeks. Sleep disturbance is common. tumours, melanocytic naevi, Spitz naevi, pilo-
Treat by stopping any offending medica- In some infants; it appears to follow matricomas, keratosis pilaris, vasculitis and
tion. Give oral antibiotics for underlying previously treated scabies infection. papular acrodermatitis. For raised red circles
streptococcal infection if required. Pustules contain neutrophils and are or rings, consider urticaria. For softer red, pur-
sterile. Multiple scrapings may be ple or blue swellings, consider haemangiomas
necessary to exclude active scabies or vascular malformations. For haemorrhagic
infection. Potent topical steroids assist papules, consider HenochSchonlein purpura
Pustular psoriasis control of flares, and the condition remits and other causes of vasculitis. If papules are
Psoriasis can present with pustules on an over 12 years. yellowish when blanched, consider juvenile
erythematous background. The pustules Eosinophilic pustular folliculitis (scalp). xanthogranulomas or xanthomas.
are sterile. The affected area may be limited Yellowish pustules appear on the scalp Papules or nodules may also occur in a
to fingers, palms and soles, or local areas and face within the first days of life. number of disorders including acute rheu-
of skin, often with an annular arrange- Lesions crust over and new lesions matic fever, juvenile chronic arthritis, systemic
ment of pustules. Alternatively, there may develop for some weeks. Pustules contain lupus erythematosus and neurofibromatosis,
be erythroderma, high fever, malaise and eosinophils and are sterile but secondary but are rarely the presenting feature.

291
12.1 DERMATOLOGY

benzyl benzoate 25% (too irritant for Post-streptococcal glomerulonephritis may

Scabies children and ineffective if diluted). be seen after chronic impetigo secondary
Scabies infection occurs as a result of close, Apply to dry skin (not after a bath) from to scabies in the indigenous population.
usually repeated, contact with an infected the neck down to all skin surfaces. For Oedema, hypertension and haematuria
individual. Scabies mites eat into the upper infants, apply to the scalp as well (not may occur over the next 2 months. In a
skin forming burrows a few millimetres in face). Use mittens if necessary to prevent child with long-standing infected scabies
length around the fingers, palms, wrists, finger-sucking. where ongoing care may be suboptimal,
elbows, axillae, nipples, penis, and soles. Leave the cream on for at least 8 hours. review and urinalysis to monitor for renal
Early burrows may be vesicular. Usually only Wash the cream off. Wash clothing, involvement is warranted. Examination of
a few mites are present. pyjamas and bed linen at this time. the child and siblings for signs of rheumatic
An intensely itchy secondary papular Remove soft toys from the bed. The heart disease is also warranted in this
eruption develops 26 weeks after first scabies mite cannot live for long periods patient population.
exposure to the Sarcoptes scabiei mite or away from the body, and insecticide
14 days after subsequent reinfestation. sprays and cleaning of furniture and
carpets are not warranted.
This secondary eruption represents an Papular acrodermatitis
immune response to the scabies antigen Exclude from school until after treatment
of childhood
and does not mean that mites are spreading has commenced.
all over the body. Papules can occur any- Treat all family members and any other Papular acrodermatitis of childhood usually
where, including the palms, soles, axillae people who have regular close skin occurs in children aged 13 years but can
and genitalia, but are most prominent on contact with the affected individuals. occur outside this range. It is a reaction pat-
the abdomen, buttocks and thighs. The scalp The itch takes a week or two to settle and tern to many infectious agents, including
and head may be involved in infants and can be treated with topical or oral coxsackie viruses, echoviruses, Mycoplasma
young toddlers. Inflammatory nodules may corticosteroid. Nodules may take months species, EpsteinBarr virus, adenovirus,
also develop, especially on covered areas. to resolve despite successful treatment of respiratory syncytial virus, rotavirus, cyto-
Excoriations and secondary impetigo may the scabies infestation. megalovirus, hepatitis B virus and others.
be present. Scabies is pandemic and affects Reinfestation is common. To minimise the It has also been reported after all standard
both adults and children. risk of reinfestation, the family should childhood vaccines. It is common and a reg-
Not all itchy parasitic rashes are scabies. notify all social contacts (e.g. creche, ular source of confusion in EDs.
Many species of parasite can cause small school or close friends) to ensure that all Papular acrodermatitis of childhood is
itchy papules on the skin, in some cases those infected receive treatment. characterised by the acute onset of mono-
hundreds of lesions. Bird mites, fleas, body Treat secondary impetigo with oral morphic, red or skin-coloured papules mainly
lice, mosquitoes, sand flies, horse flies, bed cefalexin 30 mg kg
1 (max 500 mg) on the limbs, buttocks and face, with striking
bugs, ticks, chiggers, midges and harvest three times daily. sparing of the trunk (Fig. 12.1.7). In any
mites can all masquerade as scabies. Para-
sites can be collected from the skin on trans-
parent adhesive tape. The CSIRO Australian
National Insect Collection provides an iden-
tification service on http://www.csiro.au/
services/InsectID.html.

Management
Treatment of scabies is expensive and
upsetting. If there is any doubt about the
diagnosis, confirm by scraping to find a
scabies mite, or refer before treating.
Permethrin 5% cream.
An alternative, recommended for
pregnant or neonatal cases, is sulfur 6%
in yellow soft paraffin. This is unpleasant
and has no proven safety advantages
over permethrin in these groups. The
following are not recommended: lindane A B
1% (contraindicated in infants or women
who are pregnant or breast-feeding) or Fig. 12.1.7 Papular acrodermatitis of childhood showing almost complete sparing of the trunk.

292
12.1 DERMATOLOGY
12

DERMATOLOGY
given patient, the papules tend to all look may resolve in a week or, if scratched, may the ages of 1 and 12 years. Uncomplicated
the same, typically firm and dome shaped, last for months and may repeatedly flare molluscum lesions are easily recognised as
measuring 24 mm in diameter. However, up after fresh bites elsewhere. Itch is often firm, pearly, 14 mm dome-shaped papules
in different patients, the papules can vary intense and secondary ulceration or infec- with central umbilication. They are some-
from tiny, skin-coloured papules to larger tion can occur. Full resolution usually occurs times misdiagnosed as vesicles on initial
urticarial plaques. Lesions may coalesce into within 9 months. However, scratching can examination. A child may develop a few or
patches on the extensor surfaces of the lead to secondary changes, with non-healing a great many lesions and individual lesions
elbows and knees. Lesions may be papulove- erosions, ulceration, scarring and nodule may last for months. Lesions can come and
sicular (particularly on the limbs) or purpuric formation. These lesions remain itchy and go for up to 3 years without causing any pro-
(particularly on the face). Hundreds of a cycle of scratching and skin destruction blems in most children. Complete resolution
lesions may appear over several days. It is can persist for years or decades if not trea- will not happen until an immune response
usually asymptomatic but may be itchy. ted. Improvements occur with new treat- develops, which may take from 3 months
Complete resolution occurs in 48 weeks. ments but relapses are common without to 3 years.
Affected children may be otherwise well considerable medical support. Rarely, individual lesions can grow to over
or may have features of the underlying infec- 1 cm in diameter. Individual lesions can
tion, such as mild fever, malaise, coryza, sore Management become inflamed and, uncommonly, can
throat, lymphadenopathy and splenomeg- If the diagnosis is unclear, skin biopsy is develop secondary abscesses. More com-
aly. Lymphopenia or lymphocytosis may be useful as the typical histological findings monly, presentation to the ED is triggered
present. In some cases, no history of a pre- are specific for insect-bite reactions. by the development of widespread itchy
ceding or intercurrent illness can be found. Prevent bites, e.g. by adequate clothing, and excoriated eczematous lesions in sur-
Papular acrodermatitis of childhood was modifying behaviour that leads to rounding skin, usually on the lateral chest
formerly known as GianottiCrosti syndrome exposure, occasional use of repellent, and and axillary region or between the thighs.
and was initially described as a manifesta- the treatment of pets and house for fleas In such cases, recognition can be difficult,
tion of hepatitis B infection in Italy. This and mites if necessary. The commonest as the secondary changes can obliterate
association has not been shown to be of source of bites is from mites in roof- the primary lesions. A carefully taken history
general relevance, and papular acrodermati- dwelling animals such as birds, rats and of the initial lesions is usually diagnostic.
tis of childhood has been recognised as possums. Surface spray of ceiling vents Molluscum is common in the anogenital
benign and much more common than previ- can be very useful. area and occurrence at this site does not
ously thought. Treat the itch with an agent such as suggest child sexual abuse. Molluscum is
aluminium sulfate 20% (Stingose), liquor common on the face and occurrence at this
picis carbonis 2% in calamine lotion, site does not suggest underlying undiag-
Management potent steroid ointment or nosed immunodeficiency.
Reassure and advise that clearing can antihistamines. Molluscum may occur on the eyelid mar-
take a few weeks. Protective dressings (e.g. Duoderm) can gin or adjacent area. This can cause a persis-
Children with papular acrodermatitis of speed the healing of lesions. In older tent unilateral conjunctivitis. The molluscum
childhood do not routinely require children, intralesional injection of may be isolated and subtle and go unno-
investigation to determine the underlying corticosteroid is effective. ticed for months during which the child
cause. Treat secondary infection with topical may receive multiple courses of antibiotic
Itch may require topical mid-potency mupirocin ointment 2% or oral cefalexin treatment for conjunctivitis (Fig. 12.1.8).
corticosteroids and/or oral antihistamine. 30 mg kg
1 (max 500 mg) three times
No exclusion from school or creche is daily.
needed. For more persistent and severe lesions, an
intensive regimen may be necessary,
including inpatient admission for
moisturiser, potent topical steroid
Papular urticaria ointment and wet dressings, combined
This is a clinical hypersensitivity to insect with continued intensive care of any
bites and may occur in just one child within relapse for weeks or months. Treatment
a household, even though all family mem- needs to be modified according to
bers may be bitten. New bites appear as response.
crops of asymmetrical, small, red papules, Fig. 12.1.8 This 6-year-old boy had presented
usually in warmer weather. Older bites to the ED four times in 6 months with recurrent
Molluscum unilateral conjunctivitis. Molluscum can be seen
appear as 15 mm papules, sometimes with
on the surrounding skin and a small papule
surface scale or crust, or with surrounding Molluscum is caused by a pox virus and is towards the lateral end of his lower lid margin is
urticaria. Vesicles or pustules may form in very common. Most children get at least also a molluscum. Treatment of the molluscum
the centre of lesions. Individual lesions a few molluscum at some stage between cured the conjunctivitis.

293
12.1 DERMATOLOGY

Management Several treatment options exist for Management

Most children with molluscum require no molluscum around the eye and causing Reassure the patient that this is rarely a
treatment. conjunctivitis. One or two lesions in older problem. Soap avoidance and moisturisers
Children with molluscum should not share non-anxious children can be treated can improve the spiky feeling. Most children
towels but should not be restricted in physically as for elsewhere on the face. need no further treatment. Steroids do not
their activities. Swimming should not Imiquimod 5% cream can be tried. If help.
generally be restricted. It is possible that there are many lesions elsewhere,
spread to other parts of the body and to treating all the other lesions away from
other children may be reduced by the eyes often induces rapid resolution Granuloma annulare
advising the child not to soak in warm of the eye lesions as well. In anxious
water (baths, spas, heated pools). This is a children with significant problems Granuloma annulare is not common in chil-
significant restriction for many children with conjunctivitis, sedation or a brief dren. It begins as a small skin-coloured or red
with long-term sequelae if they miss their general anaesthetic may be warranted. papule that spreads outward over many
school swimming lessons. If restrictions Inflamed lesions rarely warrant antibiotic weeks or months to give an annular, asymp-
are imposed on the child, lesions must treatment or drainage. tomatic ring, usually on the hands or feet.
be actively treated to ensure rapid Although often misdiagnosed as tinea, the
clearing. epidermis is not scaly and discrete subcuta-
Any child having problems with neous papules can be felt all around the
molluscum should be treated. Adnexal tumours ring. It is slow growing and takes months
The treatment depends on the age of the pilomatricoma to reach a size of 2 cm. Resolution usually
child, the location of the lesions and any occurs within a year or two. No investigation
Many different benign tumours of the vari-
secondary changes. Uncomplicated is necessary. Most children should be reas-
ous cell types in hair follicles, sebaceous
lesions not causing problems and not sured and dont need treatment.
glands and sweat glands can present as
spreading can be left alone. papules during childhood. The most com-
If the presenting problem is the itch from mon by far are pilomatricomas. These pres-
secondary eczema, treatment of the ent at any age in childhood as a slow- Langerhans cell
eczema with mild or mid-potency topical growing papule on the head, face or neck
steroid ointment may be all that is
histiocytosis
(or occasionally elsewhere) that can be
required. Try not to overuse as it is 440 mm in size. They may be skin-coloured, Langerhans cell histiocytosis may present
possible that this may encourage white or bluish and are usually firm or hard, in several ways and should be considered
spreading of molluscum lesions. often due to calcification within the lesion. as a possible diagnosis in any unusual,
It is not difficult to destroy molluscum They may be lobulated. The main differen- non-healing rash in infancy.
lesions. Simply deroofing the central tial is an epidermal cyst. An ultrasound In neonates, Langerhans cell histiocytosis
umbilication is almost painless and leads may confirm the diagnosis by demonstrating may present as a congenital self-healing
to the papule becoming inflamed and calcium shadows, which are not present in form. One or a few papules are present at
resolving. In older children, this can be epidermal cysts. Pilomatricomas do not usu- birth or shortly thereafter. They can be many
achieved with superficial needle pricking ally regress. Surgical removal is usually millimetres in diameter and classically have
or light cryotherapy. Benzoyl peroxide recommended because of the appearance a raised border and central necrosis. The
5%, a variety of chemical irritants and but is not urgent. papules usually show complete regression
tape stripping can all be used but within months. Recurrence years later with
these can tend to flare any surrounding visceral involvement or disseminated dis-
eczema. ease has been reported, and long-term mon-
Keratosis pilaris
Topical cantharidin (e.g. Cantharone) is itoring is warranted.
the most rapid and effective way of This is a rough, somewhat spiky papular In infants, Langerhans cell histiocytosis
dealing with molluscum in many rash, mainly on the upper outer arms, thighs can present as recalcitrant cradle cap with
situations. This is painless on application and/or cheeks with variable erythema. scaly, papular and petechial scalp lesions,
and causes small blisters at the site over It affects 5080% of all adolescents often mistaken initially for severe sebor-
the next several hours. Importation of and approximately 40% of adults. About rhoeic dermatitis. Flexural areas, especially
cantharidin requires a personal TGA 3050% of patients have a positive the groin and anogenital area, can be
permit and is not warranted unless you family history. Autosomal dominant inheri- involved, presenting as a chronic weeping
see many children with troublesome tance with variable penetrance has been nappy rash. The presence of papules and/
molluscum. described. It appears in infancy and persists or petechiae, sometimes with ulceration,
Imiquimod 5% cream nocte for several throughout life. However, it tends to improve and the lack of response to treatment for
days is expensive but may be useful for towards adulthood. Rarely, the erythema napkin dermatitis should raise suspicion.
small numbers of lesions at difficult-to- and papules are florid and distressing and In infants and older children, there may be
treat sites. treatment is warranted. widespread, often itchy, small haemorrhagic

294
12.1 DERMATOLOGY
12

DERMATOLOGY
papules mimicking a vasculitis. Erythema- psoriasis can present in many ways, includ-
tous and purpuric scaling and papules may Xanthomas ing isolated thick scaly scalp lesions, scaly
be present on the hands and feet, and nails True xanthomatous papules associated with plaques on the hairline and behind the
may become dystrophic. Mucosal involve- elevated cholesterol levels are rare in child- ears, annular lesions, pustular lesions, pal-
ment can lead to ulceration on the gums hood. They may present as multiple eruptive moplantar psoriasis, guttate psoriasis and
or palate. xanthomas, tuberous xanthomas (usually flexural psoriasis. Therefore, psoriasis must
Extracutaneous disease can involve the distributed over the elbows, knuckles, but- be considered in the differential diagnosis
liver, lymph nodes, marrow, bone and CNS. tocks, knees and heels) and tendinous xantho- of any red, scaly rash, particularly if well-
Diabetes insipidus can occur. mas (usually on tendons around the elbow, demarcated and not particularly itchy.
wrist, hand, or ankle). Investigation to exclude Guttate psoriasis describes the eruption
hyperlipidaemias is mandatory. Consider: of hundreds of small, scaly papules on the
Management trunk and limbs, often following a strepto-
Confirm the diagnosis with a skin biopsy. Primary hyperlipoproteinaemias. Skin coccal throat infection.
Investigate for other organ involvement lesions may present between 5 and Flexural psoriasis can involve any of the
with skull, chest and skeletal X-rays, 15 years of age, sometimes as tendinitis skin folds, particularly the anogenital area,
dental examination, full blood or tenosynovitis. Look for signs of and is more common in children than in
examination and liver function tests. atherosclerotic disease, and a family adults. Psoriasis at flexural sites presents
Skin lesions can be treated with topical history of high cholesterol, early as moist, non-scaly erythema, often painless
corticosteroid application. myocardial infarcts or strokes. but sometimes with secondary fissuring or
While Langerhans cell histiocytosis can be Secondary hypercholesterolaemia. This streptococcal infection.
self limited; it can also be rapidly can occur in hypothyroidism, biliary Minor nail pitting is often seen in child-
progressive with progressive organ cirrhosis, diabetes mellitus, glycogen hood psoriasis.
involvement and a poor prognosis, storage disease and nephrotic syndrome.
requiring intensive chemotherapy. Management
Presentation to the ED is often
Angiofibromas in precipitated by the onset of guttate
Juvenile xanthogranulomas tuberosclerosis psoriasis or by secondary problems with
long-standing anogenital psoriasis. The
Facial angiofibromas may be the first sign of
Juvenile xanthogranulomas are the com- treatment depends on the site and extent
tuberosclerosis. They appear as small, red-
monest cause of yellow papules in children. of disease and the age of the child. Guttate
brown papules (not pustules) on the cheeks
Occurring in early childhood, lesions appear psoriasis is often most easily managed
from about 56 years of age and can be mis-
as low, dome-shaped papules 25 mm in with an extemporaneous tar cream, e.g.
interpreted as flat warts or early-onset acne.
diameter. They may be yellow or red-brown liquor picis carbonis (LPC) 3% and salicylic
lesions that become yellow when blanched. acid 2% in Sorbolene cream, 500 g.
They are often on the scalp but can occur Adolescents are less tolerant of tar creams.
elsewhere. There may be one or multiple
RED SCALY RASHES Minimise skin trauma. Use regular
papules appearing over weeks or months. moisturiser.
Redness and scale indicate a combination of
Biopsy may be required to confirm the diag- Treat isolated skin plaques with either
vasodilatation and epidermal involvement.
nosis. Resolution without treatment usually topical steroids (e.g. mometasone) or
Atopic eczema is the commonest cause of
occurs within a few years. Juvenile xantho- topical calcipotriol, or both, for 4 weeks,
red scaly rashes in children (see p. 297).
granulomas are not associated with lipid with clinical monitoring. Topical steroids
Other red scaly eruptions include sebor-
disorders. are not used for large areas in childhood
rhoeic dermatitis (infants), psoriasis, tinea
Children with neurofibromatosis have a psoriasis because of the possible
corporis, pityriasis rosea and pityriasis versi-
considerably increased chance of having development of rebound pustular disease.
color. If itch is present, consider any of the
juvenile xanthogranulomas. The known Thick scalp plaques can be softened
causes of eczematous rashes (p. 297).
increased risk of myelomonocytic leukaemia overnight with a tar cream and removed
in a child with neurofibromatosis is probably with a tar shampoo. Generally avoid tar
not changed significantly if juvenile xantho- cream on the face, flexures and genitalia as
granulomas are also present, despite state-
Psoriasis it can be irritating.
ments to the contrary in many texts. Psoriasis can occur at any age. Every year in Use hydrocortisone 1% or pimecrolimus
Children less than 2 years old with multi- a city of 2 million people, 400 children will 1% cream on the face, flexures and
ple juvenile xanthogranulomas should have present with psoriasis for the first time. anogenital region.
an ophthalmology assessment. Ocular juve- Lesions typically begin as small, red papules Palmoplantar psoriasis can be resistant
nile xanthogranulomas leading to glaucoma that develop into circular, sometimes itchy, to treatment. Initially treat with topical
have been reported in this subgroup of sharply demarcated, erythematous pla- potent corticosteroid and calcipotriol
patients. ques with prominent silvery scale. However, ointment.

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Secondary infection including perianal A similar eruption may occur in response

streptococcal infection may require to a number of drugs. A pink scaly patch
treatment with oral penicillin. Recurrent 24 cm in diameter near the shoulders or
or persistent streptococcal infection or hips (herald patch) is the first sign in about
carriage should be considered but is 50% of children and is often misdiagnosed
uncommon. as tinea corporis because of its central clear-
A mild normocytic (occasionally ing. However, unlike tinea, the scale is usu-
microcytic) anaemia of chronic disease ally most prominent on the inside edge of
can occur in more severe cases. the inflammatory ring. A few days or weeks
Appropriate medical follow up is later (or as the first feature if no herald patch
essential. Psoriasis will recur. Widespread is present), many pink/red, scaly, oval
or resistant psoriasis may need treatment macules appear, mainly on the trunk, arms
with one or more of dithranol, ultraviolet and thighs. The face, palms, lower legs
therapy, acitretin, methotrexate or and soles are largely spared. There may
ciclosporin, all of which are effective. be thin scale within the lesions and oval
Widespread involvement may lead to lesions may align with skin lines to give a
erythroderma with metabolic and other Christmas tree pattern on the back and
complications (see erythroderma, p. 281). trunk. Pityriasis rosea usually persists for
12 months. It may be mildly itchy but is
A often asymptomatic.
Tinea corporis Pityriasis rosea may be atypical. Lesions
Tinea corporis (often called ringworm) is a may be papular, crusted, vesicular or purpu-
common skin disorder, especially among ric. The distribution may involve neck and
children, but it may occur in people of all extremities.
ages. It is caused by mould-like fungi (derma- Secondary syphilis can appear in a similar
tophytes). Tinea infections can be transmit- fashion, but mucosal and acral lesions are
ted by direct contact with affected usually present. Secondary syphilis should
individuals or by contact with contaminated be excluded in any adolescent at risk of sex-
items such as combs, clothing, shower, or B ually transmitted disease who presents with
pool surfaces. They can also be transmitted pityriasis rosea, particularly if palms and
by contact with pets that carry the fungus Fig. 12.1.9 Inflammatory bullous fungal soles are involved.
infection. (A) The foot with (B) secondary id
(cats are common carriers). The typical reaction involving the hands. Management
lesion is a slow-growing erythematous ring
Reassure the patient.
with a clear or scaly centre. Scale is usually
Treat focal lesions with terbinafine cream Sunlight (or ultraviolet light in
most prominent on the outside of the annu-
troublesome cases) will hasten clearing.
lar ring. However, tinea corporis can present (twice daily for 1 week) or an imidazole
Topical corticosteroids do not help.
in a wide variety of ways. It can be pustular cream (e.g. clotrimazole, miconazole or
Emollients, or occasionally oral
or vesicular, particularly on the soles econazole two to four times daily for
antihistamine, are useful for itch.
(Fig. 12.1.9), or it can spread to many sites 4 weeks).
Rapidly spreading or widespread lesions, In an adolescent at risk for sexually
within days. Between the toes, it presents
transmitted diseases, arrange VDRL test.
as an itchy, white, scaly and macerated rash. or involvement of hair-bearing areas
Previous treatment with steroid ointments usually requires oral griseofulvin
often leads to partial improvement in symp- (20 mg kg1 day1 in divided doses).
Secondary syphilis
toms but causes spreading of the rash, the Combined steroid and antifungal
development of papules and the masking ointments (e.g. Kenacomb) are often Secondary syphilis presents 12 months
of diagnostic features. Tinea should be con- ineffective for clearing tinea corporis. after the primary chancre (which may be
sidered in any red, scaly rash where the diag- Exclude from school until 1 day after unreported). Erythematous, slightly scaly,
nosis is unclear, particularly if there is a treatment has commenced. macules or papules appear mainly on the
gradually spreading eruption with inflam- trunk. There may be darker red macules on
matory edges. the palms and soles, as well as smooth or
eroded papules on the tongue and oral
Pityriasis rosea
Management mucosa, moist perineal papules (condylo-
Confirm the diagnosis by scraping the Pityriasis rosea is common from 110 years. mata lata), annular lesions and pustules.
scale for microscopy and culture. The cause is uncertain, but is thought to Lymphadenopathy is usually present. Sec-
The family should identify and treat the be viral. Human herpesvirus types 6 and 7 ondary syphilis can mimic pityriasis rosea
source animal if any. (HHV-6, HHV-7) have been implicated. and other red scaly disorders.

296
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12

DERMATOLOGY
of tiny flat-topped papules, coalescing into the lesions are more clearly defined, thick-
Seborrhoeic dermatitis scaly lines that may stretch the full length ened discoid areas that may intermittently
The term seborrhoeic dermatitis has been of the limb. It may remain static for a period be itchy. There is usually a cyclical pattern
used to describe a number of different clinical of months or a couple of years, before spon- of improvement and exacerbation.
entities. It is most widely used for a particular taneously resolving. It may be pruritic but Families who present to the ED with
dermatitis that occurs in areas of skin that usually not significantly so. atopic eczema often have children with
have a high density of sebaceous glands, The cause of the eruption is an inflam- chronic and severe disease requiring fre-
namely the scalp, the central T-zone of the matory reaction in a streak of skin that quent, time-consuming applications of topi-
face, and the upper chest and back (not the has a genetic abnormality (mosaicism). cal medicines and wet dressings. These
anogenital area, see p. 316). It is due to a The genetic difference of the streak is so children may have recurrent infections, par-
reaction to a Pityrosporum yeast, which is part subtle that the skin is phenotypically and ticularly with Staphylococcus aureus or her-
of the normal flora at these sites. functionally normal until a trigger, mostly pes simplex. Failure to thrive may be
As true seborrhoeic dermatitis can only a viral infection, induces an inflammatory present. A mild, normocytic (occasionally
occur in the setting of active sebaceous reaction against the abnormal skin. As the microcytic) anaemia of chronic disease can
glands, the diagnosis should only be made condition is self-limiting, no treatment is occur. Severe psychosocial and behavioural
in the first 3 months of life or after puberty necessary if the condition is asymptomatic. problems are common in these children
has begun. The sites of predilection are the Moderate-strength to potent topical steroid although often hidden from medical staff.
scalp, inner eyebrows and paranasal folds. creams can help with any pruritus. Parents may be under enormous stress with
The quality of the scale is more greasy than financial, marital and other problems sec-
that of an eczema at other sites and the ondary to the childs eczema. These pro-
degree of pruritus and discomfort is usually ECZEMATOUS RASHES blems need to be identified and addressed.
minimal. The degree of erythema varies and
mild cases present as neonatal cradle cap or The term eczematous rashes covers several
adolescent dandruff. common conditions that are characterised Atopic eczema general
Atopic eczema is common on the scalp of by erythema, itching and disruption to the
management principles
infants and is differentiated from seborrhoeic epidermis with oozing, crusting, fissures or
dermatitis by increased pruritus and discom- excoriations. The degree of epidermal dis- Education. Parents need to know that
fort, a harsher drier scale and occurrence after ruption may be minimal so that the presen- treatments are effective in controlling the
the age of two months. It is important not to tation of atopic eczema may be with just red disease. They should be aware of the
diagnose scalp eczema as seborrhoeic derma- dry patches. Alternatively, the degree of epi- principles of avoiding relevant triggers
titis as eczema will be further irritated by dermal disruption may be severe, with and settling the inflammation.
shampoos and cradle cap creams. oedema and widespread vesiculation (e.g. Avoid irritants. The following may worsen
plant contact dermatitis, which is discussed atopic eczema: soaps, bubble baths,
under vesiculobullous rashes, p. 282). prickly clothing, including clothing worn
Management by adults carrying babies, seams and
Gently debride any built-up crust with a labels on clothing, car seat covers, sand,
non-irritating product such as olive oil,
carpets, overheating or contact with pets.
bath oil or a soap substitute. Only if very Atopic eczema general
Smooth cotton clothing is preferred.
thick scale is present should salicylic acid issues
Avoid heat. Overheating increases itch.
creams be used.
The term atopic eczema covers a range of Most parents overdress young children.
Settle erythema with 1% hydrocortisone
presentations that depend on the age of Outer clothing should be removed when
cream.
the child and on the childs individual sensi- entering warm environments. Bedding
The use of an anti-yeast shampoo (e.g.
tivities. Several of these presentations are and baths should be kept comfortably
2% ketoconazole) may be helpful in more
best seen as separate diseases. Clinical fea- cool.
recalcitrant cases.
tures and specific management suggestions Keep the skin moist. Use a moisturiser
Adolescents with seborrhoeic dermatitis
are discussed under the individual headings such as sorbolene with 10% glycerine,
may have to wash their hair more
below. However, some generalisations apply aqueous cream or paraffin ointment
frequently.
to most children with atopic eczema subtypes. (50:50 white soft paraffin/liquid
Atopic eczema usually begins in infancy. It paraffin) as often as a few times a day
commonly involves the face, and often the if necessary.
Lichen striatus trunk and limbs as well. In older children, Treat inflammation. In mild or moderate
the rash may be widespread but is often cases, steroid creams can be used
Lichen striatus usually presents on a limb as localised to flexures. Erythema, weeping, intermittently with good effect.
a unilateral linear eczematous rash follow- excoriation and, rarely, vesicles may be seen Hydrocortisone 1% is usually adequate.
ing the developmental lines of the skin. On in acute lesions. Chronic lesions may show If not, moderate potency (e.g.
close inspection, there are often collections scale and lichenification. In some children, betamethasone valerate 0.02%) or

297
12.1 DERMATOLOGY

potent (e.g. mometasone 0.1% or as to whether they warrant admission to Education of the parents in an emergency
methylprednisolone 0.1%) ointment can hospital, oral prednisolone, narrow-band setting is difficult because they speak a
be used for exacerbations. Widespread ultraviolet B therapy or other systemic language other than English.
long-term use of moderate potency immunosuppressive therapy.2 The parents exhibit significant stress or
steroids to the skin can cause atrophy or impending breakdown.
striae, especially on the face, the nappy Outpatient management has not given a
area of infants, and sites of rapid growth Atopic eczema dietary satisfactory response.
such as the thighs of pubertal girls. principles An adolescent has widespread chronic
Although expensive, pimecrolimus 1% is A normal diet is indicated in most children eczema that affects their lifestyle (wears
a useful alternative in some situations. with eczema. If a child has immediate urticar- long clothing in summer, wont swim, etc.).
Oral steroids are rarely indicated in atopic ial reactions to a particular food, that food
eczema. For chronic atopic eczema on the should be avoided. No alteration should oth-
limbs, zinc and tar combinations are erwise be made to an infants or childs diet Atopic eczema use of
alternatives to steroids. Topical unless appropriate conventional therapy, as topical steroid preparations
calcineurin inhibitors (tacrolimus and above, has not worked. In difficult cases, par-
pimecrolimus) are increasingly used as an ticularly in infants, consider a formal allergy The use of topical steroids remains a pivotal
adjunct to topical steroid therapy but are assessment. Restrictive diets without profes- component of eczema management. It is
expensive. sional supervision should be avoided. important to settle the eczema and restore
Control itch. Advise parents to avoid Babies with a first-degree relative with the natural integrity of the skin. If eczema
saying stop itching all the time and to eczema have a 50% chance of developing is left untreated, the natural barrier of the
distract the child instead. Wet bandaging eczema. For these babies, no modifications skin remains disrupted and the skin is more
is very helpful if warranted. to the mothers diet are recommended dur- prone to react to other eczema triggers. Any
Antihistamines are often unhelpful but if ing pregnancy and breast-feeding. Exclusive scratching will further flare the skin and a
the itch is not controlled by other breast-feeding for the baby is recommended worsening cycle is created. Topical cortisone
measures, they may be tried. for a minimum of 4 months for a number of preparations are used to break this cycle and
Wet dressings. Wet dressings are reasons. If exclusive breast-feeding is not heal the skin. They should not be used as
bandages applied over topical possible, supplementation with partially prophylaxis on normal skin. The prepara-
moisturiser or corticosteroid ointments hydrolysed formula may reduce the risk of tions are best prescribed in ointment formu-
two to four times a day. They cool the skin eczema. The use of soy formula has no role lation as these are more moisturising.
and are effective in controlling flares of in the prevention of allergy but is to be con- Creams may be used if ointments are poorly
eczema, settling troublesome focal sidered if there is clinical suspicion of dairy tolerated. As a general rule, topical corticos-
patches of eczema and reducing the need allergy. Peanuts and other nuts should be teroids are extremely safe. Side effects are
for corticosteroid. They are well tolerated avoided until 1 year of age. very uncommon if used appropriately.
by children although cumbersome to Always ask parents if they are treating
apply. However, if the initial application is their children with herbal creams. Any
done by a parent at home, acceptance Atopic eczema admission herbal cream that gives dramatic clearing
of the treatment may be prejudiced by to hospital should be assumed to contain corticosteroid
pre-existing conflict between the child products until proven otherwise. A British
Children with eczema who attend EDs often
and parent over treatment regimens. If study in 2003 looked at a number of herbal
do so because the parents have become
possible, wet dressings should ideally be creams given to children for eczema.3 Over
increasingly desperate to get their childs
commenced by trained staff in hospital. 80% of those tested had illegal potent or
skin under control. Admission to hospital
Treat infection. Weeping and yellow very potent corticosteroid additives. Similar
can be very helpful. You should consider
crusted areas that do not respond results have been found in previous studies.
admitting the child if any of the following
to therapy may indicate secondary
circumstances apply.
bacterial or herpetic (see p. 328) Systemic effects of topical steroid
infection. Take cultures and treat with A child is missing school because of use Suppression of the pituitary-adrenal
simple wet dressings and oral cephalexin atopic eczema. If so, they should axis can occur if potent topical corticoster-
30 mg kg
1 (max 500 mg) three times generally be admitted to hospital for oids are used over most of the skin surface
daily. For recurrent bacterial infection, use intensive treatment. continuously for many weeks, but this is an
bleach baths, e.g. White King Bleach A child has severe impetiginisation or you uncommon clinical scenario. If a child is
(4.2%) 3 tablespoons ( 45 mL) in a have concerns about sepsis. using large amounts of topical corticoster-
quarter full bath (approx 60 L). A child has widespread eczema oids in combination with inhaled corticos-
Severe cases. Do not accept that nothing herpeticum. teroids and/or occasional oral cortisone
more can be done. Children with chronic The family has social, financial or mental therapy, you should consider adrenal sup-
severe eczema need to be referred for health issues that make home treatment pression. Steroid therapy should not be
psychosocial support and for assessment difficult. abruptly stopped in this situation.

298
12.1 DERMATOLOGY
12

DERMATOLOGY
Local effects of topical steroid use skin. Arrange follow up within a week or acute eczema. Management involves
Irritation or allergy to one of the constitu- two if using significant amounts of potent settling the active patches with a brief
ents of the topical steroid occurs in a few topical steroid preparations. Skin prick test- period of topical corticosteroid ointment
children. Apart from this, local side effects ing and appropriate allergen avoidance and then trying to minimise irritation from
rarely occur. Inappropriate use can lead to may be useful in the longer term. saliva. This is difficult in the dribbling infant.
atrophy, striae, telangiectasia, purpura, cat- Thicker moisturisers such as 50% white
aracts and juvenile rosacea. Care is required soft/liquid paraffin (Dermeze) may be
when treating the face, anogenital areas, Atopic eczema facial used both as a moisturiser and as a barrier
flexures and any areas of rapid growth on the skin. In persistent cases, use pime-
where the skin is already under tension Some infants will present with facial lesions. crolimus 1% cream or tacrolimus 0.03%
(e.g. breasts in adolescent females). In these There may or may not be eczema elsewhere. ointment.
situations, permanent striae can occur Facial eczema may be secondarily infected
within weeks. Precipitants of eczema should with Staphylococcus aureus, resulting in
Juvenile rosacea Is often called perioral
continue to be sought if the need for topical weeping and crusting (Fig. 12.1.10). Saliva
dermatitis and confused with perioral
corticosteroids is ongoing. It should be is often a significant exacerbating factor.
eczema but it is not a true dermatitis. It is
explained to patients that once the eczema Herpes simplex virus infection needs to be
better thought of as a subset of rosacea.
has cleared, the topical corticosteroids considered (look for the typical monomor-
The most common clinical setting is follow-
should be ceased. This is more important phic erosions, see p. 284). Treat with oint-
ing the use of topical steroids in a young
in darker-skinned individuals, where post- ment moisturiser several times daily and, if
child who is genetically predisposed to
inflammatory hyperpigmentation may be indicated, oral cefalexin 30 mg kg
1 (max
develop this condition. The morphology is
mistaken by the patient for active disease. 500 mg) three times daily for 1 week. If
quite different from perioral eczema. Ery-
The topical calcineurin inhibitors, tacroli- appropriate follow up can be assured, use
thematous papules occur around the mouth
mus and pimecrolimus, are now available methyl-prednisolone 0.1% ointment for a
but spare the skin immediately adjacent to
as steroid-sparing agents. However, their few days to gain rapid control of the skin.
the lips. Other facial areas may be involved.
expense currently limits their use for wide- Arrange follow up within a week to avoid
The papules are essentially distinct from
spread disease. problems from prolonged steroid use. Avoid-
each other but may coalesce at times. Topi-
ance of irritating factors such as using nap-
cal steroids tend to temporarily settle the
kin wipes on the face and general
inflammation and then cause a rebound
management measures are required to pre-
Atopic eczema flare, and patients are often under the false
vent relapse.
generalised infantile impression that the topical steroids are the
only effective therapy. Treatment involves
Some infants present in the first 6 months of the cessation of all topical steroids on the
life with red, scaly and excoriated lesions Atopic eczema perioral skin in this area and the introduction of an
covering much of the trunk and often the eczema vs. juvenile rosacea appropriate antibiotic. Use erythromycin in
limbs, scalp and face as well. These infants children or tetracyclines in adolescents.
Perioral eczema Refers to eczema round
are typically irritable and often sleeping Pimecrolimus 1% cream may give added
the mouth. This is common in infancy and
and feeding poorly. The parents are often benefit. Resolution usually takes 34 weeks
early childhood. Irritation from saliva is the
highly stressed by the difficulties with man- of therapy. It is important to warn patients
main cause. Occasionally, intolerance or
aging the child. Appropriate treatment usu- who have been actively using topical ster-
allergy to foods can play a role in the older
ally leads to a dramatic change in the babys oids that there will be a rebound flare before
child. Confluent patches of erythema with
behaviour and the family dynamics. they begin to see improvement.
weeping and superficial erosion occur in
A detailed history should be taken to iden-
tify any exacerbating factors in the childs
environment. Examination will often reveal
Atopic eczema periorbital
considerable dermographism and urticarial
change. Allergy to foods is common in this Periorbital eczema is usually associated with
setting and allergy testing is useful. Foods sensitivity to airborne allergens, especially
in the maternal diet can trigger allergy in house dust mites, cat dander and pollens.
a breast-fed baby. Consider whether admis- Consideration of the occasions when the itch
sion for wet dressings is required. Consider is worst will often indicate the causative
whether secondary bacterial or herpes infec- agent. Formal skin prick testing, allergen
tion is present. Treat with a bland emollient avoidance measures, moisturiser, pimecroli-
such as paraffin ointment once or twice mus 1% cream and follow up are required.
daily. Use hydrocortisone 1% ointment on In atypical cases, consider allergic contact
the face and methyl-prednisolone 0.1% to Fig. 12.1.10 Facial eczema with secondary dermatitis, irritant contact dermatitis and
the body daily to gain rapid control of the impetiginisation in a 10-month-old boy. molluscum.

299
12.1 DERMATOLOGY

and regular ointment moisturiser three-

Atopic eczema sudden Atopic eczema flexural times daily should be used. Cotton socks
worsening or urticarial flare While flexural eczema is the most common can be worn over the moisturiser. Minimise
form of atopic eczema in children, it is rarely exposure of cracked skin to carpets and
Children with eczema occasionally present
a cause for attendance at the ED unless other irritants. Topical potent corticosteroid
to the ED with an acute worsening of previ-
sudden worsening has been triggered by ointments may be necessary. Follow up is
ously stable eczema. This should prompt a
secondary bacterial infection, secondary needed.
search for an underlying cause. Look for
any evidence of scabies, which can easily herpetic infection, acute irritant or allergic
be overlooked amongst the background contact sensitivity or molluscum infection.
eczema other family members may be Search for and treat any exacerbating fac- Atopic eczema with
itchy. Look for secondary bacterial infection. tors. General principles of moisturisation, systemic associations
Consider if herpes simplex virus infection reducing overheating and avoiding irritants
Eczematous skin lesions can also be asso-
(p. 283) may be present. Small papules in are usually adequate to regain control.
ciated with:
widespread patches of eczema may be from
subtle molluscum lesions. The child may have Immunodeficiency syndromes.
developed an irritant or allergic contact sensi- Atopic eczema discoid Thrombocytopenia, immunodeficiency
tivity to their topical treatments or other and eczema are seen in WiskottAldrich
agents. Search for and treat any exacerbating Discoid eczema is characterised by chronic syndrome. Petechiae, failure to thrive,
factors. Arrange early follow up for manage- focal circular crusted lesions 15 cm in diam- recurrent infections, diarrhoea or
ment of the underlying eczema. eter. These children may have an associated haematological abnormalities may
Often a sudden worsening with a dramatic psoriatic tendency. The discoid lesions are suggest this or other immunodeficiencies
increase in itch in a child with previously mild thickened, oozing and itchy. They are rela- that are variably associated with eczema.
eczema is caused by viruses or allergy to tively resistant to therapy. Secondary staphy- Nethertons syndrome should be
drugs, food or environmental agents. They lococcal infection is present in most cases. considered if neonatal-onset eczema is
are often dermographic (i.e. they urticate in Intensive and prolonged therapy is needed associated with failure to thrive and
response to pressure on the skin) and it is to break the cycle of itch, lichenification sparse hair.
likely that the cause of the flare is a subtle and infection. Oral cephalexin 30 mg kg
1 Multiple food allergies with consequent
urticaria. When they itch, the scratching (max 500 mg) three times daily, ointment dietary restrictions and secondary
causes more urticaria, thereby causing more moisturiser, potent topical steroid ointment nutritional and psychosocial problems.
itch and escalating problems. These flares will and regular wet dressings for 2 weeks may Metabolic or nutritional disorders.
often abate over a few days or weeks and the be adequate, but therapy may need to be Phenylketonuria often presents with
addition of a regular antihistamine will con- prolonged. Follow up is mandatory. Hospital eczema. Less typical eczematous lesions,
siderably help to control the symptoms. admission, intralesional steroid injections, more prominent in periorificial areas, are
Uncommonly, metabolic triggers such as iron ultraviolet light therapy or oral ciclosporin seen in biotin, essential fatty acid and
deficiency or thyroid disease may present may all be considered. zinc deficiency syndromes. Malnutrition
with increased itch and worsening of eczema. and organoacidaemias, e.g.
methylmalonicacidaemia, can result in
Atopic eczema juvenile similar lesions.
Atopic eczema molluscum plantar dermatosis
Molluscum infection can trigger a marked Juvenile plantar dermatosis is characterised
Irritant contact dermatitis
local or generalised reaction in children. Pre- by erythema, dryness and cracking of the
sentation to the ED follows the development anterior sole and the under surface of the The most common forms of irritant contact
of widespread itchy and excoriated eczema- toes. It is usually seen in mid-childhood dermatitis in children are irritant napkin der-
tous lesions in surrounding skin, usually on and tends to occur in children with a ten- matitis (p. 317), juvenile plantar dermatosis
the lateral chest and axillary region or dency for increased sweating. The child (see above) and lip licking. Lip licking refers
between the thighs. If the child has a previ- may or may not have atopic eczema else- to a dermatitis caused by chronic exposure
ous history of atopic eczema, the molluscum where. Both feet are usually symmetrically to saliva. Usually the application of saliva
lesions may be overlooked or hidden in the involved in the weight-bearing skin on the by the child is presumed by the carers to
eczema. A careful history may reveal that soles. Juvenile plantar dermatosis may per- be secondary to the dermatitis rather than
clear, firm papules were present at some sist for years and exacerbations may be asso- causative, and may not be mentioned. Lip
stage. A careful examination may reveal ciated with painful fissures. The differential licking often presents with a clearly defined
some typical molluscum or some residual includes tinea (which should be excluded ring of dermatitis around the mouth but
papules in the centre of eczematous patches. by culture), psoriasis and contact dermatitis, the pattern of involvement may vary con-
Treat as previously described (under mollus- which can all present with red scaly lesions siderably depending on the behaviour of
cum, p. 293). on the soles. Cotton socks, leather shoes the child. Any habit that spreads saliva in

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12

DERMATOLOGY
a particular direction will affect the pattern for complete resolution to occur. These hair, teeth, nails and sweat glands. The skin
of the dermatitis. Acidic foods, artificial col- children may develop a long-lasting cross- is dry and may be hyperpigmented. Deficient
ours, preservatives and toothpaste may con- sensitivity to sun screens, hair dyes, black sweating may cause overheating in infants
tribute to the irritation. Treatment requires clothing and cosmetics. at any time and in older children in summer.
education, frequent topical ointment barrier Treat with ointment moisturiser and Children may present with undiagnosed
applications (initially many times daily) and potent topical corticosteroid or oral prednis- recurrent fevers or with heat prostration, col-
immediate treatment of any relapse. A short olone if warranted. lapse or death. Teeth are often poorly devel-
period of topical pimecrolimus 1% cream oped or absent. Cleft lip and palate, limb
may be needed. Secondary bacterial or can- abnormalities and mucous retention in the
didal infection is rarely present. upper airway and ear may be present. Accu-
Generalised dry skin
rate diagnosis, genetic counselling, dental
ichthyosis and audiological review, appropriate moist-
Ichthyosis refers to generalised scaly skin. uriser and topical cortisone use if needed,
Allergic contact dermatitis
There are several forms. Some ichthyoses education about avoiding overheating and
Acute allergic contact reactions (e.g. to only affect the skin but several have asso- regular follow up are all required.
plants) often cause vesiculation and are ciated abnormalities in other organs.
considered under vesiculobullous rashes Ichthyosis vulgaris is the commonest of
(p. 282). Chronic exposure to a contact the ichthyoses. It has autosomal dominant RED BLANCHING
allergen presents with erythema, itch and inheritance, and occurs as an isolated RASHES
lichenification. The site of reaction usually finding in 1 in 200 children. (ERYTHEMATOUS)
suggests the cause: axillary rashes from deo- X-linked ichthyosis is less common and
dorants, ears from earrings, lips and eyes may be undiagnosed for years or decades. Erythematous rashes are common in chil-
from make-up, lower abdomen from nickel About one-third of affected boys are diag- dren. They are most commonly caused
buttons on pants, wrist from watch, perioral nosed for the first time by an observant cli- by viral infections (e.g. Coxsackie virus,
from toothpaste and so on. However, the nician during an emergency attendance for echovirus, EpsteinBarr virus, adenovirus,
trigger may be overlooked. For example, an unrelated problem. A fine scaling at birth parainfluenza, influenza, parvovirus B19,
an itchy eczematous patch on the scalp is later replaced by larger brown scales. HHV-6, rubella and measles) or by a drug
may be due to a metal stud in a cap that Diagnosis is confirmed by enzyme analysis reaction. Consider also septicaemia, scarlet
is not being worn at the time of presentation for the affected steroid sulfatase gene. Boys fever, Kawasaki disease and Mycoplasma
to the ED. Consider allergic contact dermati- with X-linked ichthyosis are usually other- infection.
tis if a child has persistent eczema in an wise normal but need to be monitored
unusual distribution. for hypogonadism, cryptorchidism, anosmia,
short stature and mental retardation. Fever and exanthem
Female carriers may have obstetric difficul-
Fever and rash is a common presentation to
ties leading to prolonged labour.
Allergic contact dermatitis the ED. The most common cause is a viral ill-
In SjogrenLarsson syndrome, a yellow-
black henna reactions ness. Some infections have specific clinical
brown lichenified appearance is present
features that aid diagnosis, e.g. measles
In recent years, several children have pre- in infancy. This evolves into a more florid
and erythema infectiosum. However, in most
sented to EDs with an allergic reaction to scaling with a symmetric spastic paralysis
instances, a specific viral diagnosis cannot
black henna. Henna is widely used in Asian and mental retardation. In some trichothio-
be made with certainty. To manage such a
countries as a traditional skin colouring dystrophies, ichthyosis is associated with
child, consider:
agent. It is dark brown. Many street vendors brittle hair and mental and growth retar-
use cheap black hair dye instead, calling it dation. Several ichthyoses are linked with Is the child sick? Is the child lethargic, or
black henna, often mixing this with other deafness, cataracts or other eye problems. peripherally cold? Are they young?
agents such as petrol to enhance absorp- Mild, generalised scaling may be the first Consider meningococcal disease, other
tion. These tattoos last for some weeks. feature in later-onset Refsum disease, before bacterial sepsis, Kawasaki disease. These
These tattoos contain paraphenylenedia- development of multiple visual problems, children require resuscitation if needed,
mine, a strong contact sensitiser that should deafness and neuropathy. urgent investigation and appropriate
not be applied to the skin. Eczema does not usually present as gener- treatment guided by the findings.
Three to 10 days after the application of alised dryness in babies. Unless there is a clear Is the child taking medications? Consider
the tattoo, the child may notice discomfort, history of familial ichthyosis vulgaris, an ceasing any medication.
itch, erythema and swelling. This may prog- infant with significant dryness should be Are there other people at risk? If relatives
ress over the next 2 weeks to blistering and referred for assessment by a skin specialist. are immunosuppressed or pregnant,
scabbing of the tattoo site, sometimes with Ectodermal dysplasias are a heteroge- consider serology, stool viral culture,
ulceration. Generalised itch and urticaria neous group of conditions characterised by and advising the at-risk person to
may be present and it may take months congenital, non-progressive abnormalities of consult their doctor.

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12.1 DERMATOLOGY

Is the rash papular? Consider papular injection and hypotension. Desquamation at the finger tips and spreads to involve the
acrodermatitis (p. 292). of digits, palms, soles and perianal region palms, followed a few days later by desqua-
Is the rash itchy? If so, it may be primarily occurs 721 days later. mation of the toes then soles. Fingertip des-
urticaria or a dermatitis. Initial management should focus on resus- quamation will usually be seen even if no
If the answer to all the above is no, citation. Antibiotic treatment should include palm or sole involvement was noted earlier.
reassurance and review is probably intravenous flucloxacillin 50 mg kg1 (max This is an important feature as palm and sole
appropriate. 2 g) 46-hourly. Clindamycin 510 mg kg
1 involvement in a widespread eruption is oth-
68-hourly oral or IV should be added as it erwise uncommon.
has been shown to inhibit the release of toxin Less common features include arthritis,
Scarlet fever in vitro. diarrhoea, vomiting, coryza, cough, and
hydrops of the gall bladder.
Group A streptococci cause a variety of
In babies under 6 months of age, and
diseases including scarlet fever, pharyngo-
sometimes in older children, Kawasaki dis-
tonsillitis, impetigo, cellulitis, otitis media, Kawasaki disease ease may present as prolonged fever with
streptococcal toxic shock syndrome, necro-
Kawasaki disease (see Chapter 5.8) is mainly only one or two of the above features.
tising fasciitis, glomerulonephritis and rheu-
seen in children between the ages of Untreated, about 25% of children with Kawa-
matic fever. Group A streptococcal infection
6 months and 5 years. It is an acute, self- saki disease develop coronary artery changes.
usually occurs in school-age children.
limiting vasculitic illness and a major cause These can occur up to 68 weeks after the
Scarlet fever begins with a prodrome
of acquired coronary artery disease in onset of fever and may ultimately be fatal.
consisting of sudden onset of high fever,
Australian children. An understanding of Any child with persistent fever of unknown
vomiting, malaise, headache, and abdominal
the timing of clinical features aids diagnosis. cause should be carefully screened for any
pain. This is followed within a few hours by
The cardinal feature is a high fever persist- manifestations of Kawasaki disease. Investi-
rash. The typical rash is a diffuse, pink-red
ing for 14 weeks with unusually severe gations should include full blood count, eryth-
generalised flush with pinhead spots, that
irritability unresponsive to antipyretics. Dila- rocyte sedimentation rate, liver function tests
feels like sandpaper. The rash blanches. It
tation of conjunctival vessels is seen within a and Group A streptococcal and other serology
is first noted on the upper chest, before
few days of the onset of fever in 90% of chil- to exclude possible differential diagnoses. If
spreading to the trunk, skin folds, neck,
dren but may be subtle and evanescent and Kawasaki disease is suspected, echocardiog-
and extremities. The face is often flushed
there is no conjunctivitis or purulent dis- raphy should be performed at least twice:
with circumoral sparing. It does not usually
charge. In 60% of children, cervical lymph at presentation and again at 68 weeks.
involve the palms or soles. Other features
nodes are enlarged as a firm mass at the Treatment with intravenous immunoglobulin
include strawberry tongue (initially white,
onset of fever. In older children, there may (2 g kg1, repeated if fever persists) and aspi-
then red day 45), pharyngotonsillitis and
be striking, unilateral tender lymphadenop- rin (10 mg kg1 8-hourly while febrile,
tender cervical and submaxillary nodes.
athy thought to be cervical adenitis but then 35 mg kg1 orally daily for at least
Confirm diagnosis by throat swab and
unresponsive to antibiotics. 68 weeks) may be life-saving. Regardless
serology. Notification is not required. Trans-
Within a few days of onset of fever, 90% of the initial echocardiogram result, treat-
mission occurs by direct contact. Isolate the
of children develop a widespread erythema- ment should be given as early in the course
child from school or creche for 3 days after
tous rash. This may include urticarial and of the illness as possible, to minimise the risk
the start of treatment. Treat with phenoxy-
maculopapular lesions that increase to sev- and severity of cardiac problems.
methylpenicillin (penicillin V) 250 mg orally
eral centimetres in diameter. There is usually
(under 10 years), 500 mg orally (over 10
involvement of the anogenital area. There
years) twice daily for 10 days. For penicillin-
are no vesicles or crusting but there may Erythema infectiosum
sensitive patients, use roxithromycin.
be a few pustules on elbows or knees. The
Erythema infectiosum, also known as
rash lasts for 17 days.
slapped cheek disease or fifth disease, is
Within a day or two of the rash, 90% of
Toxic shock syndrome caused by parvovirus B19. It begins with a
children develop changes of the lips and
non-specific prodrome of fever (1530% of
Toxic shock syndrome is an acute, febrile ill- mouth. The lips become red, dry, fissured
cases), malaise, myalgia and headache.
ness with a characteristic rash and multiple and occasionally crusted and bleeding. The
The distinctive rash has three stages:
system involvement. It has potential compli- oral mucosa is red without ulceration. These
cations that include shock, renal and myocar- changes can persist for 23 weeks. Slapped cheek appearance (13 days).
dial failure, coagulopathy and respiratory Within a day or two of the development of Maculopapular blanching rash on the
distress syndrome. Toxic shock syndrome has a rash, about 95% of children develop ery- proximal extensor surfaces, flexor
been associated with Staphylococcus aureus thema of the palms and soles, often with surfaces and trunk. This fades over several
and group A streptococcal infection. associated oedema to give a shiny swollen days with central clearing and then
Presentation is initially similar to that appearance to the hands. The swelling can forms a reticular pattern (after 7 days).
of scarlet fever, but other features include persist until the fever resolves. About 2 weeks  Reticular rash reappears with heat, cold
myalgia, profuse diarrhoea, conjunctival after the onset of fever, desquamation begins and friction (weeks/months).

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12

DERMATOLOGY
Arthralgia and arthritis occur infrequently in vesicular and petechial rashes. Hand, foot buccal mucosa). The rash is initially red,
infected children, but are common in adults. and mouth disease represents one particular blanching and maculopapular. It begins
Other potential complications include aplas- syndrome, usually caused by coxsackie A16 around the ears and hairline; and spreads
tic crises in children with haemoglobin virus, and less commonly by other group A to the trunk and the proximal arms and legs.
abnormalities and chronic anaemia in chil- and group B coxsackie viruses and enterovi- It becomes confluent by the third day. High
dren with human immunodeficiency virus rus 71 (EV71) (see p. 283). Pharyngitis is a fever often persists after onset of the
(HIV) infection. frequent feature of enteroviral infection. rash. Uncommonly, a child will develop otitis
Treatment is supportive. Children with Encephalitis is a less-common accompany- media, pneumonia or encephalitis. Subacute
erythema infectiosum are highly infective ing presentation. sclerosing panencephalitis is a rare, fatal,
before the onset of illness and are probably late complication.
not infective once the rash appears. They Measles vaccination gives a transient mild
do not need to be excluded from school or exanthem in about 5% of cases. If a child
childcare. Seek specialist advice if the child
Infectious mononucleosis has been previously immunised with killed
is known to have a blood disorder or to be This syndrome is most commonly caused by measles vaccine and later contracts measles,
immunosuppressed. EpsteinBarr virus, but may also be asso- the presentation may be unusual. This
Pregnant women who contract parvovirus ciated with cytomegalovirus and other atypical measles often has high fever and
B19 have a small risk of fetal anaemia and viruses. Clinical features include fever, malaise, no cough, no eye signs, no Koplik
death but do not have an increased risk of generalised lymphadenopathy, exudative spots, and a rash that is more distal and
fetal abnormalities. It is not practicable to tonsillopharyngitis, palatal petechiae, and often purpuric.
prevent exposure at home, and exclusion hepatosplenomegaly. Children usually have Measles is highly contagious and spread
of pregnant women from work is not recom- milder disease than adults. by airborne droplets or direct contact with
mended.4 Pregnant women in contact with a Rash occurs in up to 20% of children in infected nasal or throat secretions. Sus-
child with parvovirus B19 infection should the first few days of the illness. The rash pected cases presenting to the ED should
be advised to consult the doctor supervising may be erythematous, maculopapular or be managed in a separate area to other
their pregnancy. morbilliform. There is an increased incidence patients in the department, preferably in a
of rash in children with infectious mononu- negative pressure room. Diagnosis should
cleosis if treated with amoxicillin or other be confirmed by immunofluorescence (rapid)
Roseola infantum penicillins. This rash is typically maculopap- and culture of a nasopharyngeal aspirate or
ular and pruritic, and occurs mainly over the throat swab and serology. Laboratory confir-
This common viral exanthem is caused by
trunk. mation and notification to the health
HHV-6, and in some cases by HHV-7.
EpsteinBarr virus has a number of other department is essential to prevent epi-
Ninety-five percent of children have been
less-common dermatological manifesta- demics. The child should be excluded from
exposed to HHV-6 by the age of 2 years.
tions, including papular acrodermatitis, oral school or creche for 5 days.
Up to 30% of all infants will present with
hairy leukoplakia and cutaneous lymphopro- If an unimmunised child over 9 months of
the clinical features of roseola. Typically, an
liferative disorders. age has contact with measles, measles infec-
infant has a high fever for 24 days and is
Diagnosis can be confirmed by full blood tion can be prevented by MMR vaccination
often prescribed oral antibiotics. Despite the
examination, liver function tests and serol- within 72 hours. This is because the incuba-
high fever, the child remains well and active.
ogy. Isolation is not required. Children with tion period of the vaccine strain is shorter
Occipital and cervical lymphadenopathy is
significant throat symptoms may need (46 days) than the incubation period of wild
frequently present. The fever then disappears,
admission for supportive care. Oral predniso- measles virus (1014 days). Older contacts
but at the same time a widespread erythem-
lone for 5 days may assist resolution of who do not have documentation of immuni-
atous rash appears on the face and trunk. This
symptoms. Children with splenomegaly sation with measles vaccine, should be tested
commonly leads to presentation to the ED.
should be advised to avoid contact sports for measles IgG if this can be performed
Recognition of this condition is important so
until improved. within 72 hours. If seronegative, or if serology
that both doctor and family realise that the
cannot be performed, contacts should be
rash is not a reaction to the antibiotics. After
immunised within 72 hours. Infants under
the appearance of the rash, the child remains
9 months old who have contact with measles
well and no isolation is necessary. Measles should be given normal human immunoglob-
As a result of widespread measles immunisa- ulin within 7 days.
tion, this disease is now seen infrequently.
Enteroviruses
However, outbreaks continue to occur in
Coxsackie A, B and echoviruses are all enter- most parts of the world. The hallmarks of
Rubella
oviruses that cause a range of childhood ill- measles are cough, conjunctivitis and rash.
nesses, particularly in the summer months. The rash appears 34 days after the pro- Rubella infection is asymptomatic in
They can cause several types of exanthem, drome of fever, conjunctivitis, coryza, cough 2550% of children. Affected children are
including maculopapular, erythematous, and Koplik spots (white spots on a bright red usually only mildly unwell. The prodrome

303
12.1 DERMATOLOGY

lasts up to 5 days and consists of low-grade Urticarial episodes usually resolve over
fever, malaise, headache, coryza, and post- Urticaria days or weeks and rarely last longer than
auricular, occipital and posterior triangle Urticaria is common. It is characterised by 3 months. Chronic urticaria refers to lesions
lymphadenopathy. The rash is characterised the rapid appearance and disappearance being present virtually every day for more
by small, fine, discrete, pink maculopapules. of multiple raised red wheals on any part than 3 months. Rarely, this can last for many
It starts on the face and spreads to the chest of the body. Circular erythematous macules years. Urticaria may be recurrent with indi-
and upper arms, abdomen and thighs, all or swellings, sometimes with central pallor, vidual episodes resolving quickly but occur-
within 24 hours. appear, migrate and disappear over minutes ring many times over weeks, months or
If a child is over 1 year of age and known or hours. Individual lesions are often itchy years. Recurrent or chronic urticaria is usu-
to have received rubella vaccination, a sub- and resolve within 1 day. There may be cen- ally of unknown aetiology but rarely may
sequent exanthem is highly unlikely to be tral clearing to give ring lesions. (These are be related to underlying inflammatory
rubella and investigation is not required. If not the target lesions of erythema multi- conditions such as systemic lupus erythema-
rubella is suspected in younger children or forme, which always persist for several days.) tosus, juvenile chronic arthritis, other vascu-
you cannot confirm that the child has been In some children the urticarial wheals do litic diseases, and parasitic infection. Painful
previously vaccinated, immunofluorescence not fully blanch with pressure. As the lesions urticaria may be a presenting feature of
(rapid) and culture of a nasal or throat swab migrate or grow circumferentially, they leave erythropoietic protoporphyria.
should be performed. If diagnosis is confirmed, a purplish non-blanching tinge on the skin, In a child who is very unwell with urticaria,
notification to state health departments is indicating some degree of leakage of red cells consider anaphylaxis or Kawasaki disease. If
required in most states. The child should be from capillaries. Frank purpura is not usually individual lesions last longer than 2 days or
excluded from school or creche for 5 days. present. Typically, these children are other- are tender or purpuric, consider investigation
Pregnant women in contact with a child with wise well and are reacting to the same group for cutaneous vasculitis including Henoch
rubella infection must consult the doctor of possible triggers as children with normal Schonlein purpura and urticarial vasculitis.
supervising their pregnancy without delay. urticaria. Occasionally, this may be a presen-
tation of serum sickness or cutaneous or sys- Management
temic vasculitis. Features that raise suspicion Urticaria may be the first sign of
about an underlying vasculitis include weals anaphylaxis. If there is associated angio-
Unilateral laterothoracic
lasting more than 24 hours, bruising within oedema (prominent subcutaneous
exanthem weals, painful lesions or associated fever, swelling) or wheeze, continued
Unilateral laterothoracic exanthem has only arthralgia, abdominal pain or haematuria. observation and appropriate treatment is
been well recognised in the past decade but Urticaria usually occurs in otherwise well required (see Chapter 22.5 on
is another common presentation to the ED. children as an isolated finding. The most anaphylaxis).
It usually occurs between 1 and 4 years. There common trigger for acute urticaria is a viral Investigation is usually not required.
is often a mild fever with gastrointestinal or infection, but it may be triggered by any Identify the cause if possible. Ask about
upper respiratory symptoms 1 to 3 weeks environmental or food allergen, or by pre- illnesses, medications, recent unusual
before the onset of rash. The rash begins in scription and non-prescription drugs. In most foods and environmental contacts.
one axilla or on one side of the chest as ery- cases of short duration the urticaria resolves Document these clearly.
thematous, urticarial, eczematous or papular rapidly and the cause cannot be determined. Treat the itch with oral antihistamine.
lesions. Uncommonly, the rash may start in Occasionally, the parents will strongly sus- Oral prednisolone, 1 mg kg1 per day
the inguinal region or on the thigh. Over a pect some food or environmental agent that (maximum 50 mg), for a few days may be
period of a week, the rash spreads to give a the child contacted before the onset of the warranted by the severity of the itch but
strikingly unilateral involvement of the lateral urticaria. is usually not needed.
chest, axilla and arm. Over the next 2 weeks, Urticarial drug reactions in children are If any purpura is present or the child is
there may be some spread to the other side often associated with penicillin group anti- significantly lethargic or less responsive
and other areas. Itch may be present but is biotics, cefaclor, other antibiotics, aspirin, than usual, assess and investigate to
not marked. Coryza, pharyngitis, and regional other non-steroidal anti-inflammatory drugs exclude meningococcal sepsis and other
lymphadenopathy may occur. Complete reso- and latex. Latex allergy is most common in causes of purpura. Check urine for blood.
lution occurs without sequelae within a total children with recurrent exposure to latex For recurrent or chronic urticaria, look for
of 4 to 6 weeks. such as repeated catheterisations or surgery, trigger factors. Consider mast-cell-
Although the epidemiology of unilateral and may present as local urticaria, oedema degranulating drugs (including opiates,
laterothoracic exanthem suggests an infec- or anaphylaxis. Banana, avocado and kiwi- aspirin, and other non-steroidal anti-
tive cause, no causative agent has been fruit can cross-react with latex and after inflammatory drugs), foods, alcohol,
identified despite exhaustive searching. No ingestion, children may present with itch, animals, parasitic infections, heat, cold,
isolation or restriction of activities is needed. oedema, urticaria and wheezing. sunlight and physical pressure. Consider
Moderate potency topical corticosteroids Urticaria can be precipitated by sunlight, investigating with a throat swab (for
may give some relief but often no treatment pressure, water, cold, heat and other physical Group A streptococcal carriage), full
is needed. factors. blood examination (for eosinophilia and

304
12.1 DERMATOLOGY
12

DERMATOLOGY
anaemia), IgE, antinuclear antibodies, Oral antihistamines may provide cutaneous lesions generally respond well to
urine culture for bacteraemia, nocturnal symptomatic relief. A few days of oral moisturiser, sun protection and potent topical
check for threadworms and a possible prednisolone, 1 mg kg1 day1 corticosteroid preparations. Systemic disease
challenge with any suspected agent. (maximum 50 mg), may be warranted by requires oral corticosteroid therapy initially.
Assessment of erythrocyte protoporphyrin the severity of the symptoms. Regular follow up and monitoring for renal
is warranted in young infants with sun- Hospitalisation and supportive care may and other organ involvement is essential in
induced, painful urticaria. occasionally be needed. all cases.
For recurrent or chronic urticaria that is Rechallenge with cefaclor may result in a
unresponsive to conventional H1 recurrence of serum sickness although
antihistamine antagonists, adding the risk of recurrence is unknown. A Neonatal lupus
cimetidine 1015 mg kg1 (maximum serum sickness reaction to cefaclor is not erythematosus
200 mg) orally 6-hourly, to the a contraindication to having another
antihistamine may help in a few cases. Neonatal lupus erythematosus occurs in
cephalosporin.
For recurrent urticaria, or for chronic infants whose mothers are positive for
urticaria where there is a clear history of anti-Ro(SSA) and/or anti-La(SSB) antibo-
regular exacerbations in the severity of dies. Most of these mothers are asymptom-
the lesions, a diary recording all Lupus erythematosus atic and unaware of their antibody status.
happenings in the 24 hours before each About 5% of infants of antibody-positive
Systemic lupus erythematosus may present mothers develop neonatal lupus.
relapse may be rewarding in identifying as erythematous, well-demarcated facial
the cause. Skin lesions in neonatal lupus can first
lesions, occasionally urticarial or slightly appear from a few days to a few months
scaly. These lesions occur most commonly
after birth. Widespread, erythematous, often
in a characteristic butterfly distribution
Serum sickness over both cheeks and the base of the nose.
annular and growing lesions develop on the
face, scalp, trunk, extremities and neck.
Serum sickness (sometimes called serum- In children, the facial rash may be the only
These can be misdiagnosed as tinea or
sickness-like-reactions) consists of the triad manifestation (cutaneous lupus) or there
eczema. They persist for months before
of fever, urticaria and arthralgias. It can may be systemic involvement. There is usu- clearing as the maternal antibodies clear.
occur at any age. Historically, about 50% ally an epidermal and a dermal component. Moderate potency topical steroid assists
of serum sickness reactions in Australian Therefore the lesion is usually more clearing. Sunlight and ultraviolet light can
children are idiopathic and 50% have been indurated than a dermatitis and more scaly
precipitate or worsen the rash.
associated with a recent course of cefaclor. than an urticaria.
The other major manifestation of neona-
Other medications may also be implicated. In more widespread disease, patchy
tal lupus is complete heart block (from the
Symptoms begin 521 days after com- lesions may be present over the ears, neck
third trimester). This is permanent and often
mencement of the cefaclor and persist for and less commonly the limbs. Erythematous
fatal and requires a pacemaker. Interest-
5 to 10 days. Previous courses of cefaclor macules, petechiae and small infarcts can ingly, about half of all babies with neonatal
may have been taken uneventfully, but more also be seen around the nail beds and on lupus have complete heart block and about
rapid onset can be seen in those children pre- the tips of the fingers and toes. Erythema- half have the characteristic skin lesions but
viously exposed. The child presents with an tous macules may appear on the palms of
only a small proportion have both.
urticarial rash, which can be dramatic in the hands and soles of the feet. Florid urti-
All infants with neonatal lupus presenting
appearance and cause considerable alarm caria, blistering and erythema nodosum
with skin lesions require ongoing follow up
to parents. The lesions may be more fixed are uncommonly seen.
as they appear to have an increased risk
than typical urticaria and may bruise or be Fever, arthralgias and arthritis affecting
of developing autoimmune thyroid or rheu-
tender. Arthralgia and joint swelling, possibly many joints imply systemic involvement. matological disorders during childhood.
with effusion, is observed. Polyarthralgia Lymphadenopathy, anorexia, weight loss, Subsequent siblings may be normal but
occurs in up to 80%. Fever, lymphadenopathy, muscle weakness, pulmonary disease (espe- 25% develop neonatal lupus in either the
malaise, nausea, vomiting and abdominal cially pleuritis), cardiac disease (myocarditis
skin or cardiac form. Subsequent pregnancies
pain are less common. Symptoms resolve fully and coronary artery disease), nephritis and a
should therefore have serial echocardio-
in a few days. Laboratory investigations are wide range of neuropsychiatric symptoms
gram monitoring. Most mothers eventually
unrewarding and infectious studies negative. can be seen in childhood systemic lupus
develop rheumatological disease and they
erythematosus.
also require referral for follow up.
Management (see also urticaria) If lupus is suspected, a detailed history and
If associated with cefaclor, or another examination should look for systemic organ
medication, cease the medication and involvement, and investigations should
give written information about this to the include antinuclear and anti-double-stranded
Dermatomyositis
family and treating doctor. DNA antibodies, erythrocyte sedimentation Childhood dermatomyositis affects skin and
Joint symptoms may require analgesia rate, full blood count, renal assessment muscle. Children may present with skin
and bed rest. and/or brain imaging as indicated. Localised changes years before any muscle involvement

305
12.1 DERMATOLOGY

or may present with acute or subacute devel- recurrent erythema nodosum can persist
opment of fever, rash, pain and weakness. The for months or years, particularly if an under-
earliest features in many children are an ery- lying cause has not been removed.
thematous, sometimes violaceous, rash on
the eyelids, periorbital oedema, a malar ery-
Management
thematous rash, and erythematous papules
Search for any underlying cause.
over the extensor surfaces of the joints of
Investigations may include throat swab,
the hands. In more severe cases, there may Fig. 12.1.11 Palmoplantar hidradenitis. (Photo
full blood examination, erythrocyte
be widespread desquamation and ulceration courtesy of Dr Cremer.)
sedimentation rate, serology for Group A
around the eyes, fingers and skin folds.
Streptococcus, Mycoplasma and Epstein
Muscle weakness may not have been
Barr virus, stool culture, Mantoux testing
noticed at presentation but may be present
and chest X-ray.
Pernio (chilblains)
on testing. Investigations should include
Depending on the severity of symptoms, Pernio usually presents after exposure to
erythrocyte sedimentation rate, full blood
bed rest or limitation of activities for a cold, such as playing outdoor sport in winter.
count, and creatine kinase. Magnetic reso-
few days may be required. Tender red/purple swellings occur on the
nance imaging has replaced muscle biopsy
Non-steroidal anti-inflammatory fingers and or toes. Initially there is no epi-
as a means of assessing muscle inflamma-
treatment will help settle inflammation dermal change but lesions can persist for
tion. Therapy involves high-dose oral corti-
and pain. The use of oral prednisolone is weeks and dusky scaling can develop. Symp-
costeroid therapy initially, maintenance
controversial. toms are usually mild. Most children present-
immunosuppression for a couple of years,
In troublesome, prolonged or recurrent ing with pernio are otherwise well and do
and regular follow up.
cases, potassium iodide (10% solution, not routinely need investigation. In persis-
2.5 mL 250 mg three-times daily for tent and troublesome cases, baseline inves-
older children, taken with milk or juice for tigations may include anti-Ro, antinuclear
Juvenile chronic arthritis 2 weeks) is effective.5 and anti-phospholipid antibodies, rheuma-
toid factor and cryofibrinogens. Recurrences
An erythematous maculopapular rash is often
are common. Manage by avoiding cold expo-
seen in the systemic form of juvenile chronic
sure to hands and using adequate clothing
arthritis. This rash usually has a salmon pink
Necrobiosis lipoidica to stay systemically warm.
colour, and tends to come and go, being par-
ticularly evident at the time when the fever is This presents as large, irregularly shaped,
at its height. It may be urticarial. red-yellow patches on the lower legs, usually
in adolescents. With time, the central area Spider telangiectasia
may become atrophic or sclerosed. Necrobio- A spider naevus presents as a tiny red mac-
sis lipoidica may be the first sign of diabetes ule or papule with a surrounding network of
Erythema nodosum in childhood and a fasting serum glucose fine telangiectasias, best appreciated by
Erythema nodosum can occur at any age should be arranged with follow up. compressing the lesion to make it disappear
and presents with the fairly abrupt on- then watching it reappear in the character-
set of painful and tender subcutaneous istic centripetal pattern, filling from the cen-
erythematous lesions up to 5 cm in diame- tre. These appear during mid-childhood in
ter, mainly on the anterior lower legs. The
Palmoplantar hidradenitis
about 50% of children, typically on the face,
arms, soles and trunk may be affected. Mal- Palmoplantar hidradenitis, (previously hands or arms. Most resolve within a few
aise, fever, and arthralgia may be present. known as childhood neutrophilic eccrine years. Investigation is not needed. If the cos-
Histologically there is a septal panniculitis. hidradenitis or plantar erythema nodosum), metic concern is severe, removal by electro-
In about 50% of children, erythema nodo- occurs in otherwise well children, typically dessication or laser can be arranged.
sum occurs in association with another condi- aged 214 years, during spring or autumn. Multiple spider naevi are of no medical sig-
tion, either as the presenting feature or as There may be a history of considerable phys- nificance in healthy children. Multiple telangi-
part of the evolution of an already diagnosed ical exertion in the days before the lesions ectatic vessels of non-spider type may raise
disease. Causes include chronic streptococcal appear, often with exposure to cold or water. suspicion of hereditary haemorrhagic telangi-
disease, tuberculosis at any site, inflammatory Erythematous, dusky tender lesions appear ectasia or a photosensitivity syndrome.
bowel disease, chronic gastrointestinal in- on the soles (Fig. 12.1.11), and sometimes
fections, sarcoidosis, Mycoplasma infection, on the hands. Pseudomonas infection within
lymphoma, secondary syphilis, deep fungal the sweat glands may be the cause in some
Other erythematous rashes
infections and the oral contraceptive pill. or most cases. Antibiotics are not needed
Lesions pass through colour changes sim- and spontaneous resolution occurs within Many conditions that present with red, scaly
ilar to aging bruises. Resolution occurs in 3 3 weeks. If you are confident of the diagno- or purpuric rashes may appear erythema-
to 6 weeks in most cases. Chronic or sis, no investigation is necessary. tous initially, including guttate psoriasis,

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12

DERMATOLOGY
pityriasis rosea and HenochSchonlein pur- disease. All children with fever and pete- observe for 4 hours;
pura. Sometimes, most of the lesions may chiae should be reviewed by a senior doctor. if the initial test results are normal and
be erythematous but a few may reveal the there is no clinical deterioration or
more typical features of the underlying con- progression of the rash over the 4 hours,
dition, such as scale or purpura. Fever and petechiae in an discharge with review the next day.
Many haemangiomas (vascular tumours) unwell child (including
Children who have received antibiotics prior
will initially present as erythematous meningococcal sepsis)
to presentation can be managed the same
macules. This possibility should be borne
If a child with fever and petechiae meets any way, but the possibility of partially treated
in mind in any infant presenting with an ery-
of the following criteria, they should be pre- meningitis or sepsis may lead to a lower
thematous macule on the face. Early review
sumed to have meningococcal sepsis until threshold for admission or early review.
may show some progression of the lesion. If
proven otherwise and treated accordingly
so, urgent referral for treatment may mini-
(see meningococcal and other septicaemia
mise potentially severe long-term problems
below, this page).
from florid facial haemangiomas (p. 309). Meningococcal and other
impaired conscious state (lack of septicaemia
alertness, lethargy, irritability);
Septicaemia with purpura is usually due to
PURPURIC RASHES abnormal signs (tachycardia, tachypnoea, meningococcal disease. There may be an
desaturation in air, widened pulse
upper respiratory prodrome followed within
Purpuric rashes in childhood may be due to pressure);
hours by high fever, malaise and headache.
vascular dysfunction, coagulation disorder poor perfusion (cold extremities);
Skin lesions may begin as tender erythema-
or a low platelet count. Fever may or may any purpuric lesions greater than 2 mm
tous macules or as petechiae on the skin and
not be present. Purpuric rashes are asso- (unless the clinical picture is suggestive of
mucous membranes. Lesions develop purpu-
ciated with several life-threatening diseases HenochSchonlein purpura, see below);
ric centres and large haemorrhagic areas
and require urgent assessment. abnormal blood indices (including WCC
may form. Purpura may also be due to sepsis
>15  109 L1 and <5  109 L1 or
with Haemophilus influenzae, group A strep-
raised CRP >8).
tococci, Staphylococcus aureus and some
Fever and petechiae Gram-negative organisms. Infective endo-
carditis, typhus, typhoid fever and certain
Petechiae are pinpoint, non-blanching spots, Fever and petechiae
viral haemorrhagic fevers may cause purpu-
less than 2 mm diameter. There are many in a well child
ric lesions.
causes of fever and petechiae. These include:
If a child with fever and petechiae is well and Early management (a detailed protocol
Viral infections including enteroviruses does not meet any of the criteria for menin- for meningococcal management can be
and influenza. gococcal sepsis in the preceding paragraph, accessed online6):
Neisseria meningitidis (meningococcal) the petechiae may be due to an obvious
disease. mechanical cause. These children do not
Give oxygen.
Gain intravenous or intraosseous access.
Other causes of bacteraemia including require investigation and may be discharged
Streptococcus pneumoniae and with review planned within 1224 hours.
Immediate investigations (if blood can be
obtained without delay) including blood
Haemophilus influenzae. Mechanical causes of petechiae include:
culture, blood smear, blood PCR, full blood
Other diseases including Henoch
Schonlein purpura, idiopathic
coughing or vomiting leading to count/differential, glucose, urea and
petechiae around the head and neck; electrolytes, clotting, if appropriate.
thrombocytopenic purpura and
local physical pressure such as a tight Administer cefotaxime (50 mg kg1 per
leukaemia.
tourniquet or being held tightly for dose maximum 3 g, 6-hourly).
Illness associated with vomiting or
coughing causing petechiae around the
procedures; Meningococcaemia is often associated
the practice seen in some ethnic groups with hypovolaemia: give 20 mL kg1 of
head and neck.
of treating a febrile child by rubbing or normal saline. More fluid will often be
The majority of children with fever and pete- suctioning the skin with a variety of needed to improve blood pressure and
chiae do not have a cause identified they implements, producing bizarre circular peripheral perfusion (40 mL kg1 in the
are presumably due to viral infections. Few and linear patterns of petechiae. first hour is common).
children with fever and petechiae (<5%)
If no mechanical cause is identified in a well
Isolate cases (if possible) until they have
will have meningococcal disease. Recogni- had >12 hours antibiotic treatment.
child with fever and petechiae, the child is
tion and early treatment of these children
likely to have a viral infection, and should
Subsequent investigations include throat
with meningococcal disease is paramount. swab, lumbar puncture (unless
be managed as follows:
Clinical signs and laboratory investigations contraindicated). Urinary or CSF rapid
will help determine those who should investigate with a full blood count, antigen testing is not recommended
be treated for suspected meningococcal C-reactive protein and blood cultures; because of poor sensitivity and specificity.

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12.1 DERMATOLOGY

A child with HenochSchonlein purpura purpuric rash anywhere on the body in asso-
Vasculitis and Henoch may warrant a full blood examination to ciation with disseminated intravascular
Schonlein purpura exclude thrombocytopenia. Check blood coagulation.
pressure and urine for blood or protein.
Vasculitis is a reaction pattern that presents
Bed rest and analgesia may be needed if
as non-itchy, painless macules, papules or
discomfort is considerable. Oral prednisolone Coagulation disorders
urticarial lesions with purpuric centres.
may reduce the duration of abdominal pain
There are many causes of vasculitis includ- Extensive purpuric lesions in a well child with-
and may reduce the likelihood of renal
ing infections (streptococcal, hepatitis out a history of significant trauma may be due
problems. Severe renal involvement may
viruses and others), autoimmune diseases to an underlying coagulation disorder. This
require immunosuppression, plasmapheresis
(systemic lupus erythematosus, Behcet dis- may be inherited or acquired. There may be
or hypertensive therapy but this is rare. In
ease), allergy (drugs) or idiopathic. a history of joint pain or swelling, or bleeding
children with no renal involvement at pre-
Henoch-Schonlein purpura is an IgA- from other sites.
sentation, blood pressure and urinalysis
mediated condition and is the commonest
should be done weekly for 1 month, then
cause of vasculitis in children. It can occur
each 2 months for 6 months. Children who
at any age but is most common between
the ages of 2 and 10 years. There is some-
have not developed urinary abnormalities Child abuse
within 6 months do not require further reg-
times a preceding streptococcal or non- Twisting, compression, pinching and hitting
ular follow up.
specific viral illness. Over a couple of days, can all cause petechial or purpuric lesions.
lesions occur in a symmetrical distribution, Look for bruises of bizarre shapes and differ-
mainly on the buttocks and lower legs, and ent ages or evidence of fractures or other
occasionally on the genitalia, arms and else- Thrombocytopenic purpura injuries. Assess the childs affect and rela-
where (Fig. 12.1.12). In many children, most tionship with carers. A complete examina-
Idiopathic thrombocytopenic purpura (ITP) is
lesions are purpuric, ranging from pinpoint tion is mandatory. If child abuse is
the most common cause. This is an acquired
petechiae to 2 cm in diameter. In other chil- suspected, admission or immediate referral
thrombocytopenia due to immune-mediated
dren, only a few purpuric areas may be noted to a paediatrician may be needed for com-
shortened circulating platelet survival in the
amongst many erythematous or urticarial plete assessment and further investigations
absence of other disturbances of haemostasis
lesions. There may be associated abdominal (see Chapter 18.2 on child abuse).
or coagulation. Most children present with
pain, arthralgia, arthritis or haematuria.
bruising and petechiae alone. Oral bleeding,
Renal involvement leading to chronic renal
epistaxis, rectal bleeding or haematuria are
failure is rare, but can occur irrespective of Artefactual purpura
less common. There is often a history of a
the severity of the rash and other symptoms,
recent viral infection. The child is otherwise Older children sometimes present with self-
and may be delayed until weeks or months
well. More serious causes, such as leukaemia induced purpuric lesions. These are often
after the onset of the illness.
or aplastic anaemia should be excluded. Full in bizarre, non-physiological shapes, some-
blood examination will be normal apart from times with obvious marks of finger trauma.
a low platelet count. They occur in exposed areas. Lesions may
Thrombocytopenia may be drug-induced have recurred for months or longer in an oth-
(e.g. secondary to chloramphenicol, antithy- erwise well and generally unconcerned child
roid medications). or adolescent. The history typically has inap-
propriate or contradictory details and a lack
of concern about the lesions. The diagnosis
Leukaemia and management of artefactual disease in
children may be difficult and requires confi-
Leukaemia should be suspected in a child dence in interpretation of the skin findings
with generalised petechiae or purpura in and ongoing paediatric and psychological
the absence of trauma. Features may follow up of the child and family. Occasion-
include tiredness, pallor, limb pain, malaise ally, admission to hospital may be required.
and gum hypertrophy. Thrombocytopenia
is usually present. An urgent full blood
examination should be obtained (see
Papular-purpuric gloves
Chapter 11.6 on acute leukaemia).
and socks syndrome
Two subtypes of acute myeloid leukaemia
typically present with skin lesions. Acute Papular-purpuric gloves and socks syndrome
monocytic leukaemia may present with skin is caused by parvovirus B19 and probably
Fig. 12.1.12 Vasculitis. HenochSchonlein infiltrates and gum hypertrophy. Acute pro- by several other common viruses. Adolescents
purpura. myelocytic leukaemia often presents with a develop vague symptoms of fever, fatigue and

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12

DERMATOLOGY
pains. Redness and swelling of the palms and congenital infection including rubella, redundant skin. Some haemangiomas show
soles progresses rapidly to petechial and pur- cytomegalovirus, toxoplasmosis, and little resolution.
puric lesions that do not extend onto other herpes simplex. Most haemangiomas never cause pro-
body parts. There may be oral vesicles or ero- haemolytic disease of the newborn. blems and are best left alone and allowed
sions. The adolescent remains generally well malignancy, including neuroblastoma, to involute spontaneously. However, hae-
and the eruption clears in 2 weeks. Interest- Langerhans cell histiocytosis and mangiomas can rapidly lead to problems
ingly, unlike erythema infectiosum, adoles- leukaemia. such as extreme disfigurement, ulceration,
cents with papular-purpuric gloves and iatrogenic injury, including birth trauma, blindness, destruction of cartilage, respira-
socks syndrome are thought to be infective extravasation of drugs and arterial injury tory obstruction, coagulation failure or
during the period of rash. during catheterisation. death. Urgent assessment by a clinician
protein C or S deficiency. experienced in this field is needed in the fol-
lowing circumstances:
Dusky purple nodules
on hands and feet VASCULAR TUMOURS Association with stridor Stridor, espe-
For tender red/purple areas (not usually
HAEMANGIOMAS AND cially increasing stridor of recent onset in a
purpuric) on hands and feet in otherwise HAEMANGIOMA child of a few months of age, may be due
well children, consider pernio (p. 306) and VARIANTS to a laryngeal or tracheal haemangioma.
palmoplantar hidradenitis (p. 306). This risk is greatest in children with a super-
ficial haemangioma involving the beard
Haemangiomas of infancy area. About half of all children with an air-
way haemangioma will have a visible super-
Chronic pigmented purpura Haemangiomas are tumours of vascular
ficial haemangioma.
endothelial cells. While haemangiomas
Chronic petechiae in childhood are usually
may be present at birth, most appear in
due to chronic pigmented purpura. This typ-
the first days or weeks of life. The appear- Eye involvement Even quite small hae-
ically presents as several 23 cm patches of
ance depends on whether the haemangioma mangiomas on the eyelid or adjacent to
petechiae. In each patch, both new and old
involves the skin. If the skin is involved, the the globe of the eye can cause impaired
petechiae can be seen giving red, purple and
first sign is often a patch of pallor, followed vision on that side, either by directly
brown dots (like cayenne pepper). Patches
by the development of an erythematous obstructing the visual axis or more com-
may be subtle, the child is otherwise well,
macule. Over a few weeks, this may grow monly by pressing on the globe to cause
the condition is benign, but lesions recur
and thicken into a soft, partly compressible, astigmatism. In both cases, amblyopia may
for months or years.
well defined, crimson or purple nodule (the result and may lead to permanent blindness
so-called strawberry haemangioma). There if not treated.
may be a substantial subcutaneous mass.
Other causes of childhood Haemangiomas that do not involve the skin
purpura Involvement of facial structures Hae-
are often first noticed as firm, blue or skin-
mangiomas may deform structures such as
Unusual patterns or presentations of pur- coloured swellings. Haemangiomas can
the lip, ear cartilage, nasal cartilage, denti-
pura in childhood may be associated with occur anywhere on the body and may
tion or jaw growth. This deformity will
vasculitic diseases, glucocorticoid excess, involve internal organs such as the liver or
remain after resolution of the haeman-
any cause of abnormal skin elasticity and trachea.
gioma. Deforming lesions require aggressive
scurvy. In children with scurvy, irritability, Haemangiomas may remain as tiny 1-
treatment.
bone pain, gum sponginess and bleeding millimetre lesions or involve extensive areas
may be present. Wrist X-rays are diagnostic. of the body. Growth may be slow but can be
Langerhans cell histiocytosis may present rapid and frightening, even with intensive Ulceration Ulceration is more common in
with persistent anogenital or scalp pete- treatment. haemangiomas at sites exposed to trauma,
chiae in infants, or with widespread, itchy, Haemangiomas usually grow for 39 including areas of friction, anogenital
haemorrhagic papules, including on the months and then slowly involute over the lesions and lesions involving the lips. Ulcers
hands and feet (see p. 294). next 210 years. Involution is first noticed may be very painful and can spread within
as a decrease in the intensity of the surface days to give necrotic lesions several centi-
crimson colour with development of small metres in size. There may be full tissue loss
islands of greyish skin. The haemangioma of lips, nose and eyelids with permanent
Neonatal purpura becomes softer and gradually shrinks in size. disfigurement and loss of function. Early
Purpuric rashes in the neonatal period may Half resolve by age 5, and 90% by age 9. treatment with topical metronidazole and
be an early presentation of any cause of Resolution may not be complete. Particularly paraffin ointment several times daily usually
childhood purpura (see above). In addition, with larger lesions, there may be residual leads to healing. Laser to the ulcer base or
consider: telangiectatic vessels, fibro-fatty tissue or surgical excision may be warranted.

309
12.1 DERMATOLOGY

Macular facial lesions A macular (flat) more experience is gained with

capillary lesion on the face of a neonate is propranolol treatment, optimal protocols
likely to be a capillary malformation, not a for pre-treatment investigations and for
haemangioma, and does not require urgent monitoring while on treatment will be
treatment. However, it may be the first sign better understood. Intralesional
of an extensive facial haemangioma. This corticosteroids, intravenous vincristine,
can commence as a large macular capillary subcutaneous a-interferon, vascular laser,
lesion that is indistinguishable from a capil- surgery and embolisation may all be
lary malformation until thickening and rapid warranted in individual patients, either at
growth occurs (Fig. 12.1.13). These haeman- presentation or if the response to initial
giomas cause extreme disfigurement and treatment is insufficient. Early treatment
A
after thickening has commenced, treatment will prevent or minimise complications in
is difficult. Treatment with vascular laser can most cases.
dramatically help if given during the brief Any infant with a midline lumbosacral or
macular phase, but is of little value after anogenital haemangioma or other
thickening has commenced. If you are suspi- vascular malformation should be checked
cious about a large macular capillary lesion for genitourinary abnormalities and have
in a neonate (e.g. onset after birth, changing imaging to exclude a tethered cord. To
colour, any sign of thickening), monitoring image the spine prior to 3 months of age,
and/or referral should be done with an aware- an ultrasound examination is generally
ness of the possible urgency of treatment. satisfactory. In older children, a magnetic
resonance imaging (MRI) examination is
Sudden onset of swelling and bruising warranted.
in a large haemangioma This may
presage the development of a consumptive
B
coagulopathy see Kaposiform haeman-
Rapidly involuting
gioendothelioma/tufted angioma (p. 310).
congenital haemangioma
Multiple haemangiomas (diffuse Rapidly involuting congenital haemangiomas
neonatal haemangiomatosis) Infants have recently been recognised as a distinct
with five or more cutaneous haemangiomas type of haemangioma. They are present at
have a considerably higher risk of haeman- birth as raised violaceous vascular tumours,
giomas at other sites, including liver, gut often many centimetres across. They can be
and lungs. Investigations should include a associated with high-flow cardiac failure,
hepatic ultrasound, full blood examination, and can be mistaken both on clinical and
platelet count, fibrinogen level, clotting pro- radiological assessment for arteriovenous
file, urinalysis, liver function tests, and stool malformations or vascular sarcomas. The dis-
analysis for blood. An electrocardiogram tinction is important because they resolve
and chest X-ray may be needed. Liver hae- within 1218 months without treatment.
mangiomas in particular are associated with
high flow cardiac failure and a significant
mortality. If small hepatic lesions are found Kaposiform
on imaging, follow up is required to ensure C haemangioendothelioma/
that undetected rapid growth does not occur.
Fig. 12.1.13 Untreated segmental facial
tufted angioma
Management haemangioma. (A) At 1 week; (B) at 2 weeks These tumours may be present at birth.
(easily misinterpreted as a capillary
Most parents just need education and
malformation); and (C) at 6 weeks, by which time
Growth can be rapid and extend over much
reassurance about the inherently benign thickening has occurred. Urgent assessment for of a leg and buttock. The texture is often
nature of these lesions. possible laser and oral propranolol treatment firmer than felt with typical infantile haeman-
If complications, as described above, are can minimise morbidity. giomas. Kaposiform haemangioendothelioma
considered likely, the initial treatment and tufted angioma are associated with
option was oral prednisolone for 6 weeks, types of lesions, such as large facial platelet trapping and a consumptive coagulo-
35 mg kg1 day1 for some weeks tumours, propranolol has led to pathy (KasabachMerritt syndrome). This has
before slow weaning. Since 2008, the remarkable shrinking and fading. Not all a significant mortality if untreated. Crises usu-
initial treatment of choice has been oral haemangiomas respond as well. Other ally manifest clinically as rapid swelling of the
propranolol (e.g. 1 mg kg
1 bd). For some beta blockers may also be effective. As lesion with the lesion appearing bruised and

310
12.1 DERMATOLOGY
12

DERMATOLOGY
becoming hard and tender. Assess platelet malformation leading to its appearance for with capillary malformations involving the
count, clotting profile and fibrinogen level. the first time, or leading to problems such forehead and upper eyelid have a 10% risk
High-dose oral corticosteroids may not be as pain in a previously asymptomatic lesion. of intracranial involvement and a smaller
effective and vincristine for many months The appearance depends on the nature risk of developing epilepsy, strokes, hemiple-
is commonly required to achieve and main- and site of the malformation. Purely capil- gia or mental retardation (SturgeWeber
tain control and reduction in tumour size. lary malformations appear as red macules syndrome).
Alpha-interferon may be needed, sometimes on the skin. They blanch partially or fully. Beware of any facial capillary malforma-
for years. Venous involvement manifests as dilated tion that appears after birth or becomes dar-
veins or soft compressible bluish lumps that ker or thicker in the first weeks of life. This
empty when elevated. Phleboliths are com- may actually be a haemangioma requiring
Pyogenic granuloma mon and may be palpable. Significant arte- urgent treatment (see macular facial
Pyogenic granulomas are benign acquired rial involvement may allow a thrill to be lesions, p. 310).
vascular tumours that often present to EDs felt on examination. Lymphatic lesions give
with a history of bleeding. They usually occur diffuse swelling of a limb or soft subcutane-
after infancy, on the face and neck, hands, or ous masses. Lymphatic involvement on the Pain, swelling
elsewhere on the body. There may be a his- skin surface results in a mass of firm warty Chronic discomfort and pain is common with
tory of preceding trivial trauma to that site. 2 mm papules that may be clear but often large venous malformations, particularly
Over several days, a dark red/bluish papule are dark blue or black because of haemor- those involving the legs. Pain is worse after
appears and grows to 10 mm in height. The rhage into the lesions. standing for prolonged periods. Sudden onset
surface may be moist, scaly or warty. Bleed- Vascular malformations can be associated or worsening of pain is also common. In
ing is common and may be recurrent. with many chronic and acute problems that venous malformations, it may arise from loca-
can precipitate attendance at an ED. lised microthrombi and microinfarction. In
Chronic problems include concerns about venous and lymphatic malformations, it may
Management the appearance, psychological problems, be due to secondary infection. Sudden pain
social isolation, pain, deformity, dental pro- may be accompanied by a sudden increase
Bleeding will stop with the application of blems, overgrowth, leg length discrepancy,
pressure. Pedunculated lesions can be in swelling and may lead to a rapid compro-
arthritis and bone erosion. Acute problems mise of a body function, depending on the site.
ligated at the base without anaesthetic. may be related to any of these and also
Alternatively, or if recurrence occurs, surgical Sudden, severe pain in an adolescent, with or
include pain, haemorrhage, infection, throm- without fever, may be the presenting feature
curettage is curative. Usually, local anaes- bosis and pulmonary emboli.
thesia is adequate. Any solitary feeding arte- of Fabry disease (see below). Complex regional
riole beneath the papule may need cautery. pain syndrome should also be considered.
A protective dressing will minimise the risk
of rebleeding before surgery. Neonatal vascular
malformations High flow lesions
A neonate with a substantial vascular mal- Arteriovenous malformations are danger-
Vascular malformations formation should be examined thoroughly ous. Any vascular lesion with a thrill should
Vascular malformations are developmental for associated malformations, for high-flow be presumed to be an arteriovenous malfor-
lesions composed of dilated blood vessels cardiac compromise and for other abnormal- mation. They can present in many ways to
and/or lymphatic channels. They are present ities in any system. Investigation includes the ED as a new swelling, an area of skin
at birth but may not become evident until ultrasound and radiological imaging. necrosis, skin ulceration, bleeding, or with
infancy or occasionally later in childhood. the sequelae of internal bleeding, including
Malformations include common capillary mal- intracranial bleeding. They can expand and
formations on the face (port-wine stain), more erode surrounding structures. If a high-flow
Facial capillary lesion is suspected, Doppler ultrasound
extensive capillary lesions, localised venous
malformations (port wine should be arranged to confirm this. Expert
malformations, venous malformations involv-
ing large areas with associated overgrowth
stain) follow up is essential.
and other changes, arteriovenous malforma- Facial capillary malformations are usually
tions, lymphatic malformations (e.g. cystic present at birth and persist throughout
Bleeding, coagulopathy
hygroma) or any combination of these. childhood, becoming darker and thicker dur-
They are not tumours and do not have a ing adolescence. Treatment with vascular Large venous malformations are associated
growth phase or resolution phase as hae- laser in early childhood leads to clearing of with chronic consumption of clotting factors,
mangiomas do. They are generally static most of the lesion in most children. low fibrinogen, somewhat reduced platelet
lesions. However, changes can occur over Children with vascular malformations count and bleeding. (This is not Kasabach
time, leading to increasing problems. Partic- around the eye should be screened for glau- Merritt syndrome, which is associated with
ularly at puberty, there may be growth of the coma and other eye abnormalities. Children vascular tumours.)

311
12.1 DERMATOLOGY

and thighs. Tiny vascular lesions may be Endocrine disease. Addison disease (also
Intestinal bleeding seen on the lips or in the mouth. Corneal thickening of the skin and signs of
Multiple cutaneous and visceral vascular opacities are invariably present. Children increased androgen production), Cushing
malformations occur in blue rubber bleb syn- or adolescents may present to the ED with syndrome of pituitary origin (also acne,
drome and may cause intestinal bleeding. In paraesthesia of the hands and feet, or with hirsutism, striae, purpura), exogenous
hereditary haemorrhagic telangiectasia, tel- sudden severe pain involving the limbs. adrenocorticotropic hormone
angiectasias usually appear on the face, Renal, cardiac and central nervous system administration, acromegaly (thickened,
mouth and nose. Nose bleeds become fre- problems occur later. greasy, more hairy and often with many
quent in late childhood and gastrointestinal skin tags) and hyperthyroidism can all
bleeding occurs in adult life. cause hyperpigmentation.
Renal failure may cause greying
Management of vascular
of the skin.
malformations
Bladder or bowel Haemochromatosis. In children, this is
Management of vascular malformations usually secondary to transfusions.
dysfunction in older can be demanding and complex. For
children Lipoidoses. A yellow-brown darkening of
example, interventional embolisation of a
skin, most prominent in sun-exposed
large venous malformation of a limb may
Congenital lesions over the lumbosacral areas, can occur in the NiemannPick
achieve an excellent result but only after
area may be associated with occult spinal diseases. Look for waxy indurated skin,
perhaps 20 or more general anaesthetics
abnormalities such as a tethered cord. These purpura, hepatosplenomegaly and
each of a few hours duration.
spinal anomalies may not cause problems neurological deterioration. Although a
until later in childhood when they can pres- Management requires a multidisciplinary
similar colour can be seen in adult-onset
approach using expertise from surgical,
ent insidiously with irreversible bladder, Gauchers disease, it is not a feature of the
paediatric, dermatological, radiological
bowel or limb dysfunction. These problems earlier onset forms.
and psychological fields.
can be prevented by early screening with
MRI and surgical correction. Congenital Children with lumbosacral vascular
malformations should have magnetic
lesions that have been associated with
resonance imaging to exclude a tethered
Localised macular
underlying spinal problems include haeman- hyperpigmentation
spinal cord.
giomas, capillary malformations, lipomas, including cafe-au-lait
dimples, sinuses and hairy patches. Compression stockings may reduce
chronic pain in venous malformations. macules
Low-dose aspirin may reduce episodes of Many normal children have one or two well-
pain in venous malformations. Heparin defined pigmented macules, generally not
Leg length discrepancy will minimise the coagulopathy from present at birth but appearing in the early
Children with a significant vascular malfor- large venous malformations (but does not years. In a child with pigmented macular
mation involving a leg need to be monitored help the coagulopathy associated with lesions of normal texture, consider:
for unequal leg growth. This can present as a KasabachMerritt syndrome seen with
limp or as a secondary problem including some vascular tumours). Neurofibromatosis Six or more cafe-au-
scoliosis, back pain, joint pain and headache. Partial or complete surgical resection may lait spots greater than 0.5 cm in diameter
be needed for venous, lymphatic or are strong evidence for neurofibromatosis.
arteriovenous malformations. Examine for other features including axil-
Multiple telangiectatic Orthotic or surgical correction may assist lary freckling (usually not seen until mid-
vessels with a leg length discrepancy. childhood), pigmented or thickened skin
over plexiform neurofibromas (present in
Multiple telangiectatic vessels of non-spider
early childhood), iris pigmentation, optic
type may raise suspicion of hereditary hae-
tumours, skeletal abnormalities, short
morrhagic telangiectasia or some photosen- HYPERPIGMENTATION stature, skin neurofibromas, hypertension,
sitivity syndromes. Multiple spider naevi do
macrocephaly and learning difficulties. The
not require investigation.
diagnosis may be uncertain early in life
Diffuse hyperpigmentation
but full penetrance of neurofibromatosis is
Diffuse hyperpigmentation is rare in chil- usually seen by 8 years of age. Regular fol-
Fabry disease dren and investigation to identify the cause low up is needed, including assessment of
Fabry disease is X-linked and primarily is required. Generalised darkening of the intellectual progress and audiological and
affects males. Females can be variably skin is often most obvious on the palmar ophthalmological review. In children under
affected. Angiokeratomas (flat or raised, creases, linea alba and areola, on the buccal 5 years old, magnetic resonance imaging
slightly warty, red/purple lesions) appear mucosa and on the sun-exposed areas of the of optic tracts to exclude optic glioma is war-
in mid-childhood on the lower trunk, pelvis face, neck and extremities. Consider: ranted. Other investigations are not required

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12

DERMATOLOGY
unless suggested by clinical findings. The Post-inflammatory hyperpigmentation Management
risk of myelomonocytic leukaemia in a child This occurs particularly in dark-skinned peo- The risk of malignancy in isolated small
with neurofibromatosis is several hundred ple. Many inflammatory skin disorders may congenital melanocytic naevi is very
times higher than in other children. It is still heal leaving diffuse, hypo- or hyperpig- small and does not require surgical
sufficiently low (0.05%) that routine testing mented macules that can persist for months excision. Decisions about removal are
is not warranted but any unexplained hepa- or years. based on the site and potential effects of
tomegaly, splenomegaly, lymphadenopathy, the childs psychosocial development.
pallor or infiltrative skin lesions requires Pellagra Hyperpigmentation and ery- Excision, if desired, can be done early in
investigation. Most children with neurofibro- thema on sun-exposed areas, cheilitis, peri- life, or later if undecided.
matosis never develop any of the significant neal inflammation and diarrhoea may be Giant congenital melanocytic naevi are
medical associated features. seen in niacin deficiency. associated with major psychosocial
problems. Early dermabrasion can
Naevus of Ota This presents at birth or
markedly decrease the colour of the
McCune-Albright syndrome A child during childhood as a bluish brown patch
naevus and is usually warranted. Results
with one or more large unilateral brown around the eye, sometimes involving the
may be better if surgery is performed
macules may have McCuneAlbright syn- sclera. Glaucoma has been reported with
within the first 3 months of life. Urgent
drome. Endocrine or bony abnormalities naevus of Ota.
contact with a plastic surgeon
should be looked for on examination. A bone
experienced in this area is necessary.
scan should be performed at 3 years of
Pigment lasers have also recently been
age, and the child should be monitored for Localised raised used and may be useful for some lesions.
any signs of increased hormonal secretion. hyperpigmentation A second concern is malignancy. The
Routine endocrine investigations are not
If local areas of hyperpigmentation are lifetime risk of melanoma in a giant
warranted in the absence of abnormal
roughened, raised or warty, consider: congenital melanocytic naevus is small
clinical signs.
(less than 5%). Parental instruction
Congenital and acquired pigmented about reporting changes in the
naevi (see below).
Incontinentia pigmenti If the earlier naevus will allow early biopsy of
phases have occurred in utero, hyperpig- Genodermatoses such as dyskeratosis changing areas.
congenita. Congenital nail dystrophy,
mented linear streaks may be present Giant congenital melanocytic naevi of the
pancytopenia, skeletal and eye anomalies
at birth. scalp or spine can be associated with
may be present.
neurocutaneous melanosis and other
Acanthosis nigricans. Rough dirty skin on
CNS abnormalities. Most of these
PeutzJeghers syndrome Small, pig- the neck or axillary folds is associated
abnormalities remain asymptomatic, and
mented macules present on the lips and with obesity, polycystic ovary syndrome,
screening with MRI is controversial.
mucosa from birth are associated with intes- other insulin resistance syndromes and
A reasonable approach is to screen if
tinal polyposis. Care must be taken when hypothyroidism. Early acanthosis
there is any suggestion of obstructive
assessing any episodes of abdominal pain nigricans can be seen in many overweight
hydrocephalus, if there are large lesions
as these children are at higher risk of intus- children. If weight continues to increase,
involving the axial spine and posterior
susception and collapse. Intestinal bleeding the acanthosis nigricans will progress to
scalp or multiple cannonball-shaped
may be the presenting feature. become cosmetically obvious and
lesions (look for DandyWalker
widespread. Early intervention and follow
malformations), or if there are lesions
up is required.
Segmental pigmentary disorder over the lower spine (look for tethered
(including linear and whorled hyper- cord). Early imaging in the first weeks of
pigmentation and hypopigmentation life allows the use of ultrasound where
of Ito) Streaks, lines and whorls of hyper-
Congenital pigmented naevi
possible and the use of MRI without
pigmentation and/or hypopigmentation Congenital melanocytic naevi are thickened, the need for a general anaesthetic.
may be present from birth. These patterns sharply defined, tan, dark brown or black Regular follow up is needed throughout
reflect mosaicism. Usually these children birthmarks. They range in size from common childhood to monitor the naevi and
are otherwise normal but a wide range of small lesions less than 2 cm in diameter to psychosocial development.
associated malformations in other systems rare giant lesions that may cover entire parts
have been reported, particularly eye, teeth, of the body, such as the trunk. Larger lesions
brain and skeletal malformations. Apart may have irregular colour, texture and hairi-
from audiological and ophthalmological
Acquired pigmented naevi
ness with areas of thick redundant skin.
examination, investigations are not required Large congenital melanocytic naevi often During childhood, most children develop
unless suggested by clinical findings, but are associated with many smaller lesions multiple acquired melanocytic naevi. Sun
these children should be reviewed until set- elsewhere on the body, some of which exposure in white children is associated with
tled in school. appear in the first 2 years of life. the development of an increased number of

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12.1 DERMATOLOGY

naevi. Acquired melanocytic naevi begin as

small, flat, well-demarcated pigmented Halo naevi HYPOPIGMENTATION
lesions (junctional melanocytic naevi). Halo naevi appear as a sharply defined area
A small percentage of junctional melanocy- Localised patches of depigmentation (total
of depigmentation 515 mm in diameter,
tic naevi become raised and dome shaped loss of pigment of skin or hair) can be seen
centred on a regressing mole. They are com-
(compound melanocytic naevi) often with in vitiligo, halo naevi, naevus depigmentosis
mon in childhood and benign.
a darker centre. Enlargement and darkening and piebaldism. Localised patches of hypo-
of naevi just before and during puberty is pigmented skin may be due to pityriasis ver-
common. sicolor (usually in adolescence), pityriasis
Spitz naevi alba (representing post-inflammatory
Melanoma is very rare in childhood and
uncommon in adolescence. Children and Spitz naevi are fairly common, benign mela- change following mild eczema, usually in
adolescents continue to acquire new mela- nocytic lesions. They usually present prior to mid-childhood) and post-inflammatory loss
nocytic naevi until age 20. Therefore, the puberty as single, red or red/brown papules of pigment. Focal pale patches may be the
presence of a new naevus is not in itself sus- that can grow to 1 cm in diameter. They are only marker of leprosy in an individual from
picious. You do need to be concerned if atyp- vascular and blanch to reveal the underlying an endemic area.
ical features are present, particularly in pigmentation.
adolescents. Any rapid change over a few Management is controversial. Spitz naevi
weeks leading to an increase in asymmetry, are benign but usually do not resolve.
Removal may be indicated because of uncer-
Pityriasis versicolor
an irregular border, multiple colours espe-
cially with non-brown areas within brown tainty about the clinical diagnosis or for cos- This is common in adolescents, and caused
areas, and growth beyond 10 mm may sug- metic reasons. Lesions on the central face that by an increased activity of commensal
gest malignancy. are only a few millimetres in diameter at pre- yeasts. Multiple oval macules, 310 mm in
Immune-suppressed children and those sentation are likely to grow considerably and diameter and usually covered with fine scale,
who have had chemotherapy are at greater early surgical excision may be desirable. His- appear on the trunk or upper arms. The
risk of skin malignancy and should have reg- tological interpretation is sometimes difficult lesions coalesce and may appear paler or
ular skin review. with findings that overlap with malignant darker than the surrounding skin. It is distin-
Children with a great many lentigines melanoma. Assessment by an experienced guished from vitiligo by the presence of
(small, dark brown 12 mm macules) may dermatopathologist is required. scale and residual pigment. Scrapings can
have associated findings (e.g. LEOPARD syn- be sent for microscopy to confirm the diag-
drome) and require screening for hearing nosis. Treat with topical imidazole creams
and cardiac problems and regular follow up. Naevus of Ota or with anti-yeast shampoos. For example,
apply selenium sulfide 2% shampoo or keto-
Naevus of Ota is present at birth in 50% of
conazole 2% shampoo. Leave on for 30 min-
cases and appears at puberty in most others.
utes, if tolerated, rinse, and treat daily for
Dysplastic naevi It can occur in any racial group and is one
1 week and then monthly. If there are
manifestation of dermal melanocytosis. It
These are a subtype of acquired pigmented cosmetic concerns, a short course of oral
presents as a unilateral, well-demarcated,
naevi. Dysplastic naevi are usually macular, ketoconazole followed by the above mea-
blue-black patch of skin on the cheek, fore-
greater than 5 mm in diameter, with a less- sures may be warranted. The yeast is readily
head and periorbital area. The sclera of
regular border, some erythema, and some treated, but the post-inflammatory hypopig-
the associated eye may also be pigmented.
variability in colour. They may develop dur- mentation takes months to resolve. Relapses
Cover-up cosmetics or laser depigmentation
ing childhood or after puberty. The presence are common unless ongoing monthly treat-
have been used to treat the discolouration.
of multiple dysplastic naevi is associated ments are used for a year or so.
with a significant increased risk of mela- Pityriasis versicolor is extremely uncom-
noma. However, the great majority of mela- mon in young children but, when it occurs,
Mongolian spots lesions may be on the face or neck. If found
nomas that occur in this population are
found in unaffected skin or normal moles Mongolian spots are another manifestation in a young child, examine the child for other
rather than in pre-existing dysplastic naevi. of dermal melanocytosis. They are extremely markers of an underlying congenital adrenal
Removal of a dysplastic naevus is only indi- common and can be found on most Asian hyperplasia.
cated if there is concern that a naevus may and dark-skinned babies as poorly-
represent a melanoma. Regular photogra- circumscribed, blue-black patches. The most
phy and follow up are indicated to aid in common site is on the lower back, but they
Pityriasis alba
the detection of melanoma at an early stage. can occur elsewhere, and may be multiple
Children with a family history of malig- and widespread. They are benign and usu- This condition is common in prepubertal
nant melanoma and/or multiple dysplastic ally fade over many years. No treatment is children and simply represents post-
naevi are at a higher risk of developing mel- required. When at unusual sites, they can inflammatory hypopigmentation secondary
anoma and require regular skin review. be mistaken for bruises. to mild eczema. Single or multiple, poorly

314
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12

DERMATOLOGY
demarcated hypopigmented (but never a history of epilepsy. Look also for forehead confirmed by skin biopsy showing mucopoly-
completely depigmented) 12 cm macules plaques (pink, initially flat but later slightly saccharide intradermal staining. Resolution
are seen on the face or upper body. Lesions raised) and shagreen patches (rough, usually occurs over months.
are not itchy but often have a fine scale. slightly thickened skin usually over the Unusually firm skin is an early feature of
Usually no treatment is needed. If desired, back). Other skin findings such as periungual systemic sclerosis. In children, there is usu-
treat with emollients. Treat any erythema fibromas and facial angiofibromas usually ally widespread skin involvement. Raynaud
with hydrocortisone 1%. Resolution takes appear in older children. The diagnosis of phenomenon may be present and involve-
weeks and requires sun exposure for full tuberous sclerosis may require imaging of ment of lungs, heart, kidneys and gastroin-
repigmentation. eyes, brain, heart and kidneys and investiga- testinal tract usually occurs within a few
tion of relatives. Regular paediatric follow years.
up is required. Waxy indurated skin may accompany
Vitiligo neurological degeneration and hepato-
Generalised hypopigmentation splenomegaly in type A NiemannPick
This condition is characterised by sharply disease. Thickened skin may be seen in
Generalised hypopigmentation, blonde hair
demarcated, often symmetrical areas of acromegaly and Addisons disease.
and grey-blue eyes are seen in several geno-
complete pigment loss. Eventual repigmen-
dermatoses involving chromosomes 11 or
tation in childhood vitiligo is common and
15. The clinical presentations vary from mild
is helped by topical steroids or pimecrolimus
to complete loss of pigmentation and indivi- MOUTH DISORDERS
cream. In troublesome cases, corrective cos-
duals may go undiagnosed unless compared
metics or ultraviolet therapy may be
to their siblings and parents. Early diagnosis Most of the conditions covered in the section
required. The association in children
and investigation is important to ensure on vesicular disease can involve the oral
between vitiligo and other organ-specific
early ophthalmological intervention and rig- mucosa. Oral vesicles break rapidly so that
autoimmune conditions (e.g. diabetes, thy-
orous sun protection. Look for: usually only erosions or small ulcers are
roid disease) is very small. Investigations
seen. Oral involvement is often the first fea-
for these are not necessary unless there is Poor vision, photophobia and nystagmus. ture of hand, foot and mouth disease, man-
a clinical suspicion of an associated disorder. Type 1 oculocutaneous albinism usually
ifesting as a few lesions involving the
results in more severe disease than
tongue and palate, followed a day or two
type 2.
later by vesicles on the extremities. Oral ero-
Post-inflammatory Bleeding diathesis, due to a platelet
sions may be seen occasionally in varicella
hypopigmentation defect in HermanskyPudlak syndrome.
and rarely in zoster.
Recurrent infections in ChediakHigashi
This condition occurs particularly in dark- syndrome.
skinned people. Many inflammatory skin dis- Mental retardation and obesity. Both
orders may heal leaving diffuse, hypo- or Angelmans and PraderWilli syndromes Primary herpetic
hyperpigmented macules that can persist can present with albinism. gingivostomatitis
for months or years. Repigmentation eventu-
ally occurs. Primary herpetic gingivostomatitis (see
p. 283) presents in infants and young chil-
SKIN TEXTURE dren with malaise, high fever, soft-tissue
swelling, lymphadenopathy and vesicles
Linear and whorled and erosions on the buccal mucosa, gingi-
hypopigmention Lax skin vae, lips and adjacent face. Drinking and
(hypomelanosis of ito) Lax, hyperextensible skin is seen in the sev- eating is painful. Management includes sup-
See linear and whorled hyperpigmentation eral EhlersDanlos syndromes. There may portive care, bathing of crusts and analgesia.
and hypopigmentation, p. 313. be bruising, scarring at sites of minor Aciclovir is not routinely indicated unless
trauma, joint hyperextensibility and arthri- risk factors for complications exist (e.g.
tis, and recurrent urinary infections. underlying disease, immunosuppression).
Tuberous sclerosis
Hypopigmented patches may be the first
Firm or thickened skin Herpangina
sign of tuberous sclerosis. These patches
can be regular or irregular in shape. A couple Scleredema (do not confuse with sclero- Several echoviruses and Coxsackie viruses
of isolated hypopigmented patches in a derma) is a rare condition in childhood, can cause the sudden onset of malaise, high
young child are far more likely to be simple which presents over some weeks with grad- fever, sore throat and vomiting, sometimes
achromic naevi than to be the first sign ual thickening of the skin of the head, neck with abdominal pain. Examination of the
of tuberous sclerosis. Tuberous sclerosis and upper trunk. It may be related to preced- pharynx and posterior mouth reveals a large
becomes much more likely if the child has ing streptococcal infection. Diagnosis can be number of small, 2-mm vesicles or erosions

315
12.1 DERMATOLOGY

coalescing on an erythematous background Rarely, angular cheilitis may be the pre- (the softer areas) rather than the gums or
to form painful ulcerations. Diagnosis can be senting feature of nutritional deficiencies hard palate. They resolve in a few days. Less
confirmed by throat or faecal swab. Com- including riboflavin, iron, zinc, pyridoxine, commonly, ulcers can be larger and persist for
plete resolution occurs with supportive care biotin and protein metabolic disorders. Mal- weeks. Any atypical features should lead to a
over 5 days. absorption, malnutrition, oral corticosteroids consideration of less-common causes of recur-
and oral antibiotics may contribute. rent mouth ulcers including:

Management requires avoidance as much Iron, folate or vitamin B12 deficiency.

Transient lingual papillitis as possible of any factors that may be Recurrent erythema multiforme.
Transient lingual papillitis is probably the
contributing. Pemphigus, pemphigoid and other
same condition as eruptive familial lingual
Minimise acidic foods such as tomatoes immune-mediated blistering conditions.

papillitis. It was first described in 1996 and

and citrus fruit. Gastrointestinal disorders. Coeliac
is not mentioned in major paediatric or der-
A trial of non-fluoride toothpaste and disease, Crohns disease and ulcerative
supervised rinsing after teeth cleaning colitis are all associated with mouth
matology texts but is much more common
helps in some children. ulceration. Recurrent abdominal pain,
than generally realised. Most reported cases
have been in the families of hospital staff.
Topical unmedicated ointment or paste intermittent diarrhoea and failure to
up to several times a day will assist thrive may be present.
In one survey of 200 hospital staff, 50% of
families appeared to have experienced this
recovery. Topical 1% hydrocortisone Connective tissue disorders. Patients
ointment is useful. If microscopy reveals with Behcets disease usually present
condition.7 In most cases, an infant appears
evidence of Candida or bacterial in late childhood with ulcers at one
to be the index case and many other family
pathogens, topical miconazole or site (mouth or genitals) and it may
members are then affected. Older children
mupirocin, respectively, can be added. be many years before a second site
present with a burning sensation on the tip
or sides of the tongue. Younger children pres-
Allergy to a component of the treatment is involved. Systemic lupus
schedule should be suspected if the erythematosus and juvenile rheumatoid
ent with irritability and poor feeding of
condition persists despite treatment, and arthritis may cause recurrent mouth
unknown cause. The tongue is normal apart
patch testing may be warranted. ulcers.
from small papules or scalloping along the
anterior and lateral tongue margins without
In difficult cases, consider an uncommon Immunodeficiency states, HIV infection,
underlying cause as above, and a trial of cyclic neutropenia and periodic fevers.
ulceration. Affected individuals are otherwise
well without any fever, lethargy, respiratory or
iron, vitamin and mineral Malignancy and chemotherapy.
supplementation. Lymphoma and Langerhans cell
gastrointestinal problems. Symptoms and
histiocytosis can present with non-
signs last from 220 days. Recurrent episodes
healing mouth ulcers.
may occur for many weeks and again years
later. Manage with symptomatic treatment Geographic tongue PFAPA syndrome of periodic fever,
aphthous stomatitis, pharyngitis,
and education. Geographic tongue is typically seen under 4 cervical adenitis.
years of age but can occur in older children.
Most children are asymptomatic but some Troublesome episodes of aphthous ulcera-
complain of tenderness or pain with salty tion can be managed by:
Angular cheilitis
foods. Irregular smooth patches are seen investigating for an underlying cause as
Angular cheilitis can occur during child- on the tongue in a pattern that changes above;
hood and is common in older children. It pre- from day to day. The cause is unknown. Geo-
sents as persistent erythema, scaling and
topical anaesthetic gels;
graphic tongue is more common in psoriasis topical steroid paste (e.g. triamcinolone
fissuring at the corners of the mouth and sur- but most children (>90%) who have geo- acetonide);
rounding skin, sparing the buccal mucosa. graphic tongue never develop psoriasis. No
Discomfort and pain may be worsened by
oral colchicine.
treatment is needed unless some foods
stretching, during yawning for example, cause discomfort, in which case avoidance
and by salty or tart foods. Most cases are of those foods for a while is usually suffi-
primarily attributable to atopic eczema cient. If needed, topical corticosteroid can ANOGENITAL RASHES
but are multifactorial. Contributing factors alleviate symptoms.
include saliva, licking, drooling at night, sig- Anogenital rashes are common in infancy.
nificant dental malocclusions, irritant and Most anogenital rashes seen in infants that
allergic dermatitis (e.g. cosmetics, medicated wear nappies are primarily caused by reac-
Recurrent mouth ulcers
lip balms, sunscreen lip creams, toothpaste, tion to urine or faeces (irritant napkin derma-
fluoride and sucked or chewed lollies). These are most often due to aphthous stoma- titis). Soaps, detergents and secondary yeast
Staphylococcus aureus, Candida albicans or titis. Aphthous ulceration usually presents as infection may contribute. In atypical or persis-
streptococcal species may all be cultured a few, painful, 48 mm ulcers on an erythem- tent anogenital rashes in infancy, consider
from affected skin. atous base on the non-keratinised mucosa psoriasis, Langerhans cell histiocytosis, zinc

316
12.1 DERMATOLOGY
12

DERMATOLOGY
deficiency and malabsorption syndromes. In cotton wipes. Avoid commercial wipes,
older children, consider perianal streptococ- which can be irritating.
cal infection (if painful), psoriasis, lichen scle- Gel-based disposable nappies or a non-
rosis, and atopic eczema. Kawasaki disease wettable under-napkin can be helpful.
may be associated with a tender anogenital Cloth nappies should be thoroughly
rash in an unwell child. washed and rinsed.
Treatment of rashes in the anogenital Use topical zinc cream or paste for mild
area requires caution. The moistness and eruptions. This functions as a barrier and
occlusion of these areas increases the pen- should be applied thickly so that some is
etration and effectiveness of topical agents. still present at the next nappy change.
For example, the absorption of topical The cream or paste does not need to be
hydrocortisone per square centimetre of completely removed at each change. Fig. 12.1.14 Anogenital psoriasis in a 1-year-
old boy.
anogenital skin can be several times Add hydrocortisone 1% cream if
greater than trunk skin. Adrenal suppres- inflamed. Do not use stronger steroids.
boys and girls (Fig. 12.1.14). Symptoms are
sion may occur after use of excessive topi- Consider mupirocin 2% cream if not
cal hydrocortisone under the nappy area settling. Antifungal therapy is usually not often surprisingly minimal. If pain and/or fis-
in small infants. Anogenital skin is also needed even if Candida is present. suring are present, swab for secondary strep-
more likely to develop local steroid side tococcal infection. This leads to a marked
effects, such as atrophy and striae. Any increase in discomfort and pain and is often
child using cortisone products in this area the cause for presentation in an older child
should have written instructions and appro- Candida napkin dermatitis who may have had months or years of asymp-
priate medical follow up. tomatic redness. Anogenital psoriasis may be
This occurs secondary to irritant napkin der- an isolated finding or there may be similar
matitis and antibiotic use, leading to ery- lesions in other intertriginous areas, such as
thema and white material in the folds and
the axillae, or more typical signs of psoriasis
satellite pustules. Treat the underlying cause
Irritant napkin dermatitis elsewhere. Occasionally, in infants, anogenital
as above and add topical imidazole cream if psoriasis will lead to a few lesions on the lower
Irritant napkin dermatitis is the most com- necessary. trunk and then a rapid eruption of lesions
mon cause of napkin dermatitis in infants
elsewhere including scalp, face and trunk.
and typically presents as confluent ery-
This settles within weeks.
thema that spares the groin folds. Variant Anogenital seborrhoeic Manage by treating streptococcal infec-
presentations include multiple erosions in
dermatitis tion, if present, and then treat as for irritant
the natal cleft secondary to diarrhoea, scaly
napkin dermatitis (this page). Medical follow
or glazed erythema, and satellite lesions at The term seborrhoeic dermatitis has been
up is essential to ensure clearing, as children
the periphery. Satellite pustules are sugges- used to describe an anogenital eruption in
and adolescents are often unaware of quite
tive of secondary Candida infection. Can- infants, with or without scalp or flexural
significant asymptomatic anogenital psoriasis
dida infection is much less common with involvement. Traditionally, it is differen-
or fail to report it because of embarrassment.
breathable disposable nappies. tiated from irritant napkin dermatitis by
Gluteal granulomas are purple/brown accentuation in the skin folds. However, this
papules or nodules, often oval shaped, which distinction is far from clear. The napkin area
can develop around the skin folds in infants is not a seborrhoeic site. Many presentations Perianal streptococcal
with troublesome napkin dermatitis. Most with anogenital seborrhoeic dermatitis rep- dermatitis
cases are associated with the inappropriate resent either atopic eczema with irritant
use of potent topical steroid in this area. napkin dermatitis or psoriasis with anogen- A painful, red, perianal rash in a child
These lesions slowly resolve over several ital involvement, or a combination of the between the ages of 1 and 10 is likely to
months provided the underlying irritant nap- two. Treat as for irritant napkin dermatitis, be due to perianal streptococcal dermatitis.
kin dermatitis is treated. or anogenital psoriasis if suspected. The usual presentation is a child who has
Irritant or traumatic anogenital rashes had a sore anal region for weeks or months.
may be seen in circumstances of suboptimal Defecation is painful and constipation is
care, emotional abuse or physical abuse. present, but treatments aimed at relieving
Anogenital psoriasis
the constipation have not helped. Bright
Anogenital psoriasis can present at any age red blood may be present on the stool or toi-
Management from infancy to adolescence. Sharply demar- let paper. On examination, a localised, well-
Keep the area clean and dry. Leave the cated, non-scaly, brightly erythematous pla- demarcated erythema that covers a circular
nappy off whenever possible. ques can be seen and may involve any or area of 12 cm radius around the anus
Clean the anogenital area by hand under all of the perianal and intragluteal region, can be seen. Fissures or macerated skin
warm water or with diluted bath oil on inguinal folds and the genital area in both may be seen.

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12.1 DERMATOLOGY

Perianal group A streptococcal dermatitis an erythematous base may be seen but

may be secondary to any cause of perianal these rapidly become small monomorphic Vulval itch/vulvitis in
itching. Concurrent pinworm infestation erosions, crusting and coalescing, sometimes prepubertal girls
should be considered. Constipation usually into an extensive, painful, erosive rash. Her-
Prepubertal girls with vulval itch usually
follows painful defecation, rather than being pes simplex virus infection in the anogenital
have an identifiable dermatological condi-
the cause of it. It takes a long while for the area of young children is usually innocent,
tion. (Do not call this vulvovaginitis as the
child to recover confidence in defecation contracted from a cold sore on a carer but
vagina is not involved.) Girls between the
after successful treatment. the possibility of child sexual abuse should
age of 2 and 10 present with months or
be considered. The possibility of child sexual
years of vulval itch, occasionally with sting-
Management abuse is higher in older children. For man-
ing or pain on micturition. On examination,
Take perianal and throat cultures to agement see p. 283.
the labia majora may appear normal or may
confirm the presence of group A show erythema, scale, lichenification and
Streptococcus. excoriation. The introitus is normal. The
Apply paraffin ointment three-times daily Varicella great majority of these girls have atopic
to the perianal area for symptomatic eczema. There may be signs of this else-
Varicella lesions may initially be localised to
relief. Topical mupirocin three-times daily where or signs may be limited to the vulval
the anogenital area in young children.
has a role. Treat with area. Management is as for atopic eczema at
Immunofluorescence and culture may be
phenoxymethylpenicillin (penicillin V) other sensitive sites and involves avoidance
required to avoid an incorrect diagnosis of
orally BD 250 mg (under 10 years), of chemicals, soaps, bubble baths and other
herpes simplex.
500 mg (over 10 years) for a minimum of irritants, and use of cotton underwear and
2 weeks. Several weeks of therapy may be ointment moisturiser. Use of corticosteroid
required. Intramuscular benzathine or pimecrolimus creams is appropriate to
benzylpenicillin can be used if there are Threadworms
settle the erythema and itch. Measures to
concerns about compliance. In older children, threadworms (Enterobius oestrogenise the vagina are either irrele-
Consider treatment for pinworm with vermicularis) are a common cause of an itchy vant or counterproductive and oestrogen
mebendazole or pyrantel. anogenital rash. Look for the worms at night creams should not be used.
Keep stools soft with oral liquid paraffin. and treat with oral mebendazole 50 mg Less common causes of vulval itch include
Perianal emollient ointment and stool (<10 kg), 100 mg (>10 kg) (not in preg- psoriasis, lichen sclerosis, staphylococcal fol-
softeners should be continued for a nancy or less than 6 months) or oral pyrantel liculitis or infection with group A strepto-
further 2 months after resolution of 10 mg kg1 (maximum 500 mg) as a single cocci. In all these cases, clinical signs
symptoms to prevent relapse, which is dose. A repeat dose 2 weeks later helps suggestive of the associated condition are
otherwise common. reduce the high rate of reinfestation. likely to be present.
Emphasise the importance of wearing clean
underwear at night, preventing perianal
Staphylococcal-mediated scratching at night, hand washing immedi-
anogenital rashes ZINC AND OTHER
ately on awakening, avoidance of thumb
sucking and good toileting hygiene.
NUTRITIONAL
Bullous impetigo may present in the ano- DEFICIENCIES
genital area as small vesicles or pustules
that coalesce to give areas of peeling skin Zinc deficiency may be due to an inherited
with peripheral margins of loose epidermis. Lichen sclerosis defect in zinc handling or may be an
These may be asymptomatic and the infant Lichen sclerosis presents with chronic itch, acquired deficiency as a result of prematu-
is otherwise well. Provided the baby is well, discomfort or pain in the vulval area of girls rity, malabsorption syndromes or abnormally
the disease is localised and the parents will aged 3 years or older. On examination, there low maternal breast-milk zinc levels. Breast
follow instructions regarding follow up, an may be an area of atrophy with white shiny milk normally provides some protection from
alternative is to treat with topical mupirocin skin, purpura or telangiectasia in the peri- the inherited form, so presentation of inher-
2% and general measures for napkin care. vulval region. Cases have been misdiag- ited zinc deficiency is usually after breast-
Oral cefalexin 30 mg kg
1 (max 500 mg) nosed as sexual abuse. Management is feeding has ceased. By contrast, acquired
three times daily may be required for more with moisturisers and brief courses of moder- zinc deficiency can present earlier, in the
widespread disease. ately potent or potent steroid ointment. neonatal period.
About 50% of cases resolve spontaneously. Zinc deficiency causes acrodermatitis
Relapses after successful treatment are com- enteropathica. A sharply defined, red, often
Herpes simplex virus extensive, anogenital rash is an early and
mon and children with this condition may
Primary herpes simplex virus infection and have ongoing troublesome symptoms and prominent manifestation. Look for perioral,
eczema herpeticum can both occur in the vulval abnormalities into adulthood. Long- perinasal and acral (hand and foot) dermati-
anogenital area. Initially, small vesicles on term follow up is required. tis, alopecia, diarrhoea, and failure to thrive.

318
12.1 DERMATOLOGY
12

DERMATOLOGY
Serum zinc levels do not correlate well with Management options include awaiting reso- traumatic alopecia, tinea capitis and kerion.
body zinc status. Oral zinc leads to a rapid lution, topical podophyllotoxin, imiquimod, For diffuse hair loss, consider telogen efflu-
improvement of the rash within days. curettage and diathermy and carbon dioxide vium and nutritional causes. Longstanding
Long-term follow up to monitor the childs laser. sparse hair can be associated with ectoder-
zinc and copper status is essential. The presence of genital warts in a young mal dysplasias and with many primary
Biotin deficiency and various other meta- child is not an indication for mandatory genetic abnormalities of hair and hair
bolic deficiency syndromes can give a similar reporting to government protective services. anchoring.
picture, although usually in the setting of an Genital warts in young children should
unwell acidotic baby. prompt consideration of the possibility of
sexual abuse, but transmission is usually
Head lice
by normal intimate parent-child contact,
Malabsorption including at birth. Unless there are other risk Head lice infestation is common. Epidemics
factors for sexual abuse on history or exami- of head lice regularly sweep through primary
Malabsorption from any cause (e.g. cystic nation, referral for sexual abuse investiga- schools in all areas. Nits (eggs) can be seen
fibrosis) can present with diarrhoea, erosive tion is inappropriate. If in doubt, seek as small white specks firmly attached (unlike
dermatitis and failure to thrive. There may paediatric advice. In children older than dandruff flecks) to hairs. Adult lice may occa-
be a progressive intractable napkin rash 5 years, the association between anogenital sionally be seen as small brown insects,
contributed to both by the diarrhoea and warts and sexual abuse increases and about 4 mm long, walking across the scalp.
by secondary nutritional deficiencies. If mal- assessment by a paediatrician is warranted. Itching and excoriation are common. Presen-
absorption continues, the rash may become
tation to an ED usually follows secondary
glazed and generalised in association with eczematisation and infection causing
oedema and malaise. Identify and treat Molluscum In the anogenital area are
often misdiagnosed as warts. Close exami- extending weeping and crusted scalp lesions
the cause of the malabsorption. Manage and neck lesions.
the anogenital area as for irritant napkin nation of all lesions usually reveals a few
to have the classical umbilicated pearly Suitable treatments include pyrethrin
dermatitis. Topical pastes are thicker than 0.165% (e.g. Pyrifoam), maldison 0.5%
ointments and may be required. appearance. Molluscum in the anogenital
region are common and are not a marker and permethrin 1% (e.g. Nix and Lyclear
of child sexual abuse. cream rinse). Wash the hair with soap and
water. Thoroughly moisten the hair with
Langerhans cell the treatment and leave for 10 minutes.
histiocytosis Congenital syphilis Erosions and moist Rinse well and comb out with a fine-toothed
warty lesions in the perianal area may be seen lice comb. Reapply 1 week later to kill any
Langerhans cell histiocytosis may present in
in infants with congenital syphilis. There may eggs that have subsequently hatched. Rein-
infancy as a chronic inguinal or anogenital
be erythema on the palms and soles, fever, festation is common. A regular physical
rash, with brownish/red scale and pete-
failure to thrive and hepatosplenomegaly. inspection, use of conditioner and combing
chiae, which is often erosive and unres-
ponsive to treatment. A scaly, papular, of the hair are as important as chemical
eruption on the scalp or trunk may appear. treatment. Resistance to chemical prepara-
IMMUNODEFICIENCY tions is increasingly common and a combina-
Purpura, fever, diarrhoea or hepatospleno-
megaly may be present, see p. 294.
STATES tion of chemical and physical therapies is
generally required. A shorter hair cut can
Immunodeficiency from any cause may pres- make this more manageable.
ent in infancy as diarrhoea with a chronic
Constipation erosive napkin dermatitis, widespread sebor-
Constipation by itself does not cause peria- rhoeic dermatitis or neonatal erythroderma.
nal erythema and pain. Look for another Secondary infection with bacteria and/or
Tinea capitis
cause, e.g. eczema, psoriasis or streptococcal viruses can complicate the clinical appear- In Australia, tinea capitis is usually caused
infection. ance. Look for other features of immuno- by Microsporum canis contracted from cats
deficiency including failure to thrive, or dogs or by Trichophytum species con-
erythroderma, lymphadenopathy, unusual tracted from other infected children or ani-
ANOGENITAL PAPULES infections and petechiae with eczema. mals. The incidence of tinea capitis is
AND LUMPS much higher in children with racially deter-
mined tightly curled black hair. Any child
Anogenital warts Are soft fleshy warts HAIR PROBLEMS of African background with an itchy scalp
that occur at the mucocutaneous junctions, should be assumed to have tinea until
especially around the anus. They may be For an itchy scalp, consider tinea capitis, proven otherwise. Apart from itch, tinea
isolated, flesh-coloured nodules or may atopic eczema and head lice. For patches capitis is characterised by patches of hair
coalesce into large cauliflower-like masses. of hair loss, consider alopecia areata, loss with some short, lustreless, broken hairs

319
12.1 DERMATOLOGY

a few millimetres in length. Redness and to total scalp or body-hair loss or recurrent
scaling are present in the patch. Occasion- alopecia can occur. Regrowth can occur dec- Diffuse hair loss
ally, inflammatory pustular swellings may ades later. Diffuse hair loss in children is usually from
be present (see kerion below). Hair loss with- telogen effluvium. This is characterised by
out any epidermal changes is not likely to be an increased number of hairs going synchro-
fungal. Management
nously into the resting (telogen) phase and
Confirm the diagnosis by greenish fluores- For isolated small patches present for
weeks without further progression, no subsequently being shed several weeks later.
cence of the hair shafts (not present with Shed hairs have a small club-like appearance
some fungi) with Woods light and with treatment is needed.
at the proximal end. Telogen effluvium pre-
microscopy and culture of hair and scale. In older children who are bothered
cosmetically, intralesional corticosteroid sents as diffuse, often quite dramatic hair
Treatment usually comprises griseofulvin loss 416 weeks after the causative event.
orally 20 mg kg1 in divided doses (maxi- injections are the treatment of choice.
In children, this is commonly a high fever
mum 1 g, after meals with milk) daily for For recent or progressive hair loss,
treatment with potent topical steroids for a few days. Other causes include car acci-
812 weeks or until non-fluorescent. Terbi- dents, operations and acutely stressful
nafine and itraconazole are also effective. may be trialled but is rarely useful as
penetration into the deeper dermis to the events. Complete regrowth always occurs.
Ketoconazole 2% shampoo twice-weekly Dramatic widespread diffuse hair loss may
can be used by all family members for hair follicle bulb is small. In difficult cases,
other therapies, including contact be from alopecia areata.
6 weeks to reduce cross-infection. Exclude Diffuse hair loss occurs after cancer che-
from school until 2 days after treatment sensitisation, irritant agents and pulsed
corticosteroids, need to be considered. motherapy. More chronic, diffuse hair loss
has commenced.2 without an obvious cause may require inves-
tigation to exclude treatable causes, such as
zinc or iron deficiency, malnutrition, excess
Kerion (inflammatory Traumatic alopecia
vitamin A, oral retinoids for acne, hypothy-
ringworm) roidism or antithyroid medications, hypopitu-
This condition is usually caused by rubbing itarism, hypoparathyroidism, diabetes, and
This represents an inflammatory scarring (as on the occiput of many babies), cosmetic thallium poisoning.
immune response to tinea capitis. It is an practices (e.g. tight braiding) or hair pulling Long-standing sparse or irregular hair is a
erythematous, tender, boggy swelling, often as a habit (trichotillomania). Trichotilloma- feature of many genetic diseases. In some,
several centimetres in diameter, which dis- nia may be largely nocturnal and parents such as monolethrix, the child is otherwise
charges pus from multiple points. The swel- are often unaware of it. The affected areas normal. In others, such as ectodermal dys-
lings appear fluctuant and are often are usually angular and on the anterior or plasias, many associated features may be
misinterpreted in EDs as pyogenic abscesses. lateral scalp. The areas contain hairs of dif- present. These include decreased or absent
Skin incision and drainage should be ferent lengths and are never completely sweating, pointy or missing teeth, dysplastic
avoided as it leads to delayed healing and bald, unlike alopecia areata. Presentation nails and dry skin. Clinical findings may be
increased scarring. Scrapings and/or some to EDs may be triggered by the hair loss or subtle. In any young child with unusual nails,
extracted hairs should be sent for micros- by the sequelae of eating the hair, including skin or teeth who presents with fever and
copy and culture. Treatment is as for tinea acute intestinal obstruction (trichobezoar) in collapse, ectodermal dysplasia with inability
capitis combined with a brief course of oral severe cases. to sweat and dysfunctional temperature
steroids to suppress the immune response. control should be considered. Treatment
Antibiotics for secondary infection may includes cooling to restore normal body
occasionally be useful. Other inflammatory Management temperature, ongoing parental education
granulomas can mimic kerions. Exclude from Recognition of the problem and a careful and support to avoid similar overheating
school until 2 days after treatment has explanation to the family is often crises.
commenced. sufficient. Alert the family to the risk of
trichobezoar.
Trichotillomania in younger children does
Hypertrichosis
not usually indicate that significant
Alopecia areata psychological problems are present. It is Generalised hypertrichosis (increased hair in
Typically one or more oval patches of hair a habit similar to thumb-sucking or all areas) may be an isolated finding or
loss develop over a few days or weeks. These nail-biting and a low-key approach similar may be related to inherited syndromes
patches are usually completely bald but to that used in those conditions is (including Hurlers and De Langes syn-
some hairs may remain within the patches. appropriate. In older children, it may be dromes), medications (especially minoxidil,
Rarely, the hair loss is diffuse. The scalp part of an obsessive trait or other phenytoin and ciclosporin), gastrointestinal
appears normal and does not show scaling, psychological problems that require disease (including coeliac disease), anorexia
erythema or scarring. Most cases in child- professional and pharmacological nervosa, hypothyroidism, and porphyria (look
hood resolve spontaneously but progression support. for photosensitivity and blisters).

320
12.1 DERMATOLOGY
12

DERMATOLOGY
Local patches of hypertrichosis can be can be seen during therapy with oral isotret- cause such as mucocutaneous candidiasis
seen as an isolated finding, or in association inoin for acne. One or more nail folds become (especially if oral Candida infection is
with lichenified eczema (temporary), pig- red painful and swollen. Pus may be present present), hypoparathyroidism and
mented lesions (congenital pigmented at the nail margin. Any cause of prolonged malignancy.
naevi, acquired naevi, Beckers naevi), plexi- wetness or minor trauma (e.g. finger sucking,
form neurofibromas, other naevoid lesions, nail picking, finger eczema, preceding
chronic rubbing or over the sacral area in chronic paronychia) predisposes to acute
association with underlying spinal anoma- infection by breaching the cuticle between Nail psoriasis
lies (Fauns tail). A small degree of increased nail and skin. Treatment requires identifica- Psoriasis can present as isolated nail disease
hair growth over the sacral area is common tion and avoidance of the trigger factors, and can mimic acute or chronic paronychia
in many racial groups and this is not a warm compresses, drainage if needed, oral and onychomycosis. Features of nail psoria-
marker of spinal anomalies. cephalexin 30 mg kg
1 (max 500 mg) three sis include pitting, thickening and brownish
times daily, topical paraffin ointment and subungual discolouration. There may be
ongoing nail care. If due to oral isotretinoin, repeated episodes of pain associated with
Hirsutism dose reduction and treatment as above usu- the collection of pus under the nail. Drain-
Hirsutism refers to an increase in male pat- ally resolves the problem. age leads to relief but antibiotics dont help.
tern terminal hair. Increased pubic or axillary Cultures are sterile. Detailed history and
hair in young children may be due to adrenal, Chronic paronychia Is traditionally examination of the rest of the child may
gonadal or CNS disease and requires investi- associated with Candida albicans, which reveal other features of psoriasis. Initially
gation. Hirsutism in adolescent females may can be found in specimens from most cases. treat with potent topical steroid ointments.
be an isolated finding or may be seen with However, Candida is now seen by many
obesity and amenorrhea in polycystic ovary authorities as a secondary feature that
syndrome. Cushing syndrome, mild congenital may not be particularly relevant to the onset
and maintenance of the condition. The pri-
Ingrown toenail
adrenal hyperplasia, virilising adrenal and
ovarian tumours, exogenous androgens and mary event is disruption of the nail cuticle The nail of the large toes can grow into the
thyroid dysfunction may cause hirsutism. resulting in inflammation of the proximal lateral nail fold and cause pain, redness,
nail fold. Chronic paronychia may be a pri- swelling and occasionally pus secondary to
marily eczematous condition caused by Staphylococcus aureus. The chronic inflam-
NAIL PROBLEMS many factors including finger sucking, nail matory process leads to progressive granula-
picking, finger eczema and any increased tion tissue formation. Management involves
Infants nails are often thin and exhibit a exposure to moisture. Affected nail folds avoidance of shoes where possible, avoid-
degree of koilonychia (spoon shape) that are often dusky red or purple with loss of ance of tight-fitting shoes, careful cutting
requires no investigation. Congenitally the cuticle and without significant pain. of the nails and use of pledgets between
abnormal nails are usually atrophic and The nail often has several horizontal ridges the nail and lateral nail fold. Oral antibiotics
can be the presenting feature of rare inher- corresponding to episodes of inflammation are not usually required. Surgical removal of
ited conditions, such as ectodermal dyspla- of the proximal nail fold. The nails may granulation material is helpful. Trimming of
sias (these children may present with fever become increasingly dystrophic with time the lateral nail alone usually results in recur-
and heat prostration), dyskeratosis conge- but do not tend to thicken. Chronic parony- rence of the problem. In troublesome cases,
nita, pachyonychia congenita (thickened chia may be present for months or years with surgical ablation of the lateral nail plate
nails), congenital malalignment of the great occasionally acute exacerbations that may be warranted.
toenails and the nail-patella syndrome. Nails respond to oral antibiotics without clearing
may have appeared normal at birth and the underlying problem.
become dystrophic over the first year of life.
Tinea unguium
Acquired nail disease is usually a result of Management
(onychomycosis)
paronychia, fungal infection, psoriasis, Management requires topical potent
ingrown toenails or 20-nail dystrophy. It corticosteroid application daily for several Dermatophyte infection may affect one or
may also be seen in association with dis- weeks. more nails. White or yellow patches develop
eases, such as alopecia areata and lichen Avoid all factors that predispose to at the distal and lateral nail edges. The rest
planus. Nail-biting and picking can lead to continuing irritation or allergen exposure. of the nail may become discoloured, friable
marked deformity of involved nails. Nails should be kept dry. Repeated and deformed with accumulation of subun-
applications of Vaseline or even a gual debris. Multiple nails may become
hydrocolloid dressing over the cuticle can involved over years. Tinea may also present
Paronychia assist in this. Cotton gloves under rubber on the adjacent skin, particularly in the
Acute paronychia (inflammation of the gloves should be used for wet work. interdigital folds. It can be difficult to clini-
nail fold) Is usually caused by Staphylo- In difficult or unresponsive cases, consider cally distinguish between psoriasis and fun-
coccus aureus or group A Streptococcus. It an unusual infection or rare underlying gal infection in nails. Always confirm the

321
12.1 DERMATOLOGY

diagnosis by microscopy and culture of nail systemic disease, including hepatitis C Swabs for Chlamydia trachomatis
clippings before commencing treatment. If infection, diabetes, uraemia, conjugated Dry swab for PCR.
cultures are negative, repeat the cultures hyperbilirubinaemia, paraproteinaemia, For culture, swab lesion with two
but do not start oral treatment. In very mild polycythaemia and thyroid disease. dacron or rayon-tipped swabs. Smear
cases only, treatment with physical debride- one onto microtrak well and break
ment and antifungal nail lacquer (e.g. amiol- tip of other into Chlamydia transport
farone) may be effective. Most cases require Collection of specimens medium.
oral therapy with terbinafine or griseofulvin
Bacterial swabs (Gram stain Skin scrapings for fungal/
for months. School exclusion, although spe-
and culture) dermatophyte culture
cified in some states, is not medically war-
Swab lesion with a plain dry swab and roll Use scalpel blade to scrape the surface
ranted or appropriate.
over a glass slide. Discard swab and air dry of the lesion. Collect flakes of skin in
slide. specimen jar.
Swab lesion with second swab. Wet swab
ITCH WITHOUT RASH with sterile saline if lesion is dry. Place
swab in charcoal medium (maintains References
Some children present with an itch without organism viability). 1. McDonald M, Currie BJ, Carapetis JR. Acute rheumatic
an obvious rash. Other children may have fever: a chink in the chain that links the heart to the
Swabs for HSV or VZV throat? Lancet Infect Dis 2004;4:2405.
multiple excoriations without obvious cause, 2. Borchard KL, Orchard D. Systemic therapy of paediatric
or they may have much more severe pruritus Roll dry swab over base of suspicious atopic dermatitis: an update. Australas J Dermatol
lesion (to collect cells for 2008;49:12334.
than would be expected for the eruption 3. Ramsay HM, Goddard W, Gill S, Moss C. Herbal creams used
present. All such children need to be evalu- immunofluorescence). Roll over for atopic eczema in Birmingham, UK, illegally contain
appropriate slide (3 wells). Swab can potent corticosteroids. Arch Dis Child 2003;88
ated for: (12):10567.
then be cut off and placed in viral 4. Gilbert G, Starr M. Parvovirus. In: Palasanthiran P, Starr M,
atopy; transport medium for culture. Jones C, editors. Management of perinatal infections.
Australas Soc Infect Dis Sydney; 2002. p. 302.
scabies; Fluid from vesicles is not suitable for 5. Sterling JB, Heymann WR. Potassium iodide in
symptomatic dermographism; immunofluorescence, but may be collected dermatology: A 19th century drug for the 21st century
uses, pharmacology, adverse effects, and
dermatitis herpetiformis; into viral transport medium for culture. contraindications. J Am Acad Dermatol 2000;43
iron deficiency; (4):6917.
6. Royal Childrens Hospital Melbourne. Clinical Practice
food allergy; Swabs for PCR (for pertussis, Guidelines. Available from http://www.rch.org.au/
drug use. Most pharmaceutical, VZV, HSV, Mycobacterium clinicalguide [accessed 20.10.10].
recreational and herbal drugs can ulcerans, etc.) 7. Whitaker SB, Krupa JJ, Singh BB. Transient lingual
papillitis. Oral Surg Oral Med Oral Pathol Oral Radiol Endod
cause itch; Dry (flocked) swab 1996;82:4415.

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 13

SECTION
EYES
Section editor Jeremy Raftos

13.1 Ophthalmological emergencies 323 13.3 Ocular trauma 328

13.2 Congenital, developmental and
neoplastic conditions 327

13.1 Ophthalmological emergencies

Toby Fogg

extraocular movements follow a finger,

ESSENTIALS a small toy or a face;
visual fields;
1 A history of contact lens use should always be sought. lids, lashes and lacrimal apparatus;
2 Visual acuity should always be assessed. conjunctiva and sclera;
cornea;
3 Neonatal conjunctivitis may have severe sequelae if inadequately treated. anterior chamber;
4 The suspicion of keratitis should prompt urgent ophthalmic review. pupil and iris;
lens;
5 The distinction between orbital and preseptal cellulitis should always be made. red reflex, posterior chamber and retina.
6 Local anaesthetic drops should never be given to a patient to take home. All drops will sting, except for fluorescein. How-
ever, once the pain has subsided, the effects of
local anaesthetic may allow for a much more
Introduction Examination meaningful examination than would other-
wise have been possible. If, however, this
Few ophthalmological emergencies relate The approach to the examination of a paedia- remains impossible, consideration should be
exclusively to children. Although the pathol- tric eye may differ from that of an adult in that given to examination under anaesthetic.
ogy may be the same, the general approach, an opportunistic strategy should be taken.
examination and compliance with treatment Leave touching the child until last, particu-
is very different depending on the age and larly if it will result in fear, pain or discomfort. Eyelid disorders
development of the child. Also, the long- Some form of assessment of acuity must be
term sequelae of serious eye injury or dis- made. In pre-verbal children, assess their abil-
Blepharitis
ease are much greater. Blepharitis is an inflammatory process of the
ity to fix and follow interesting objects and
eyelids that is characterised by crusting at
then reach for them. In older children, monoc-
the base of the eyelashes. There may be
History ular testing should be carried out using
oedema of the lids themselves and occasion-
picture cards, single letter matching or a stan-
As with other paediatric encounters, it is ally the conjunctiva is also injected. Staphy-
dard Snellen chart. Use of the slit lamp is not
important to gain the childs confidence in lococci are the usual organisms. Treatment
essential for a detailed examination, but even
you whilst obtaining the history from the should involve the removal of the crusts
small children may co-operate if they remain
parents or carers. A detailed history should using a warm flannel, cleansing with dilute
seated on their parents knees.
be obtained from an adult witness. If this baby shampoo and application of antibiotic
If the child is compliant, a logical sequence
is unavailable, injury will be the likely cause ointment to the lid margin.
of examination would be from outside to
of a painful red eye. Other conditions pre-
inside thus:
senting with a red eye are listed in Hordeolum (stye)
Table 13.1.1. Always enquire about the visual acuity using Snellen chart with A hordeolum is an acute infection, usually
use of contact lenses or glasses. letters or pictures; staphylococcal, of an eyelash follicle. It causes

323
13.1 OPHTHALMOLOGICAL EMERGENCIES

be chemosis, conjunctivitis and a mucopuru-

Table 13.1.1 Differential diagnosis of the red eye
lent discharge. Rarely there is proptosis,
Normal visual acuity fever, malaise and a reduction in ocular
Conjunctivitis (bacterial, viral, Gritty, itchy, injected conjunctiva, discharge motility, making clinical differentiation from
allergic, chemical)
orbital cellulitis difficult, and thus requiring
Foreign body Pain, grittiness, epiphoria, photophobia a computerised tomography (CT) scan or
Episcleritis Mild pain, localised conjunctival injection
magnetic resonance imaging (MRI).
The disease is usually viral in origin but a
Scleritis Severe pain, diffuse conjunctival injection
mucopurulent discharge makes a bacterial
Subconjunctival haemorrhage Consider trauma or pertussis infection more likely so swabs should be
taken before starting cephalexin.
Reduced visual acuity
Corneal abrasion Pain, grittiness, epiphoria, photophobia. Corneal defect seen with
fluorescein staining

Keratitis Photophobia, epiphoria, ciliary injection, flare in anterior chamber, Conjunctival and scleral
corneal infiltrate disease
Corneal ulcer Photophobia, epiphoria, corneal injection, flare in anterior chamber,
defect on fluorescein staining. History of contact lens use? Neonatal conjunctivitis
(ophthalmia neonatorum)
Anterior uveitis Photophobia, epiphoria, ciliary injection flare in anterior chamber,
miosis, posterior synechia This condition is defined as conjunctivitis
occurring within the first 30 days of life. The
causative organisms are usually passed from
a small, tender swelling, often with a head of spontaneous resolution not occur, the child mother to fetus during passage through the
pus, which is seen at the lid margin. Treat- should be referred for probing of the duct birth canal and are commonly Neisseria
ment is with warm compresses and chloram- after 6 to 12 months of age. If there is a gonorrhoeae, Chlamydia trachomatis, staphy-
phenicol ointment until resolution occurs. mucopurulent discharge, topical antibiotics lococci, streptococci or herpes simplex.
should be added for 2 weeks. Gonococcal infection presents within 24
Chalazion (meibomian cyst) to 48 hours of birth with acute eyelid
This is caused by inflammation of the lipid- Dacryocystitis oedema, bulbar conjunctivitis, chemosis
secreting meibomian glands of the lid, prob- This is defined as infection of the nasal lacri- and a profound purulent discharge. Both
ably due to ductal obstruction. It presents as mal duct and presents with pain, redness, eyes are usually affected. As the presenta-
a slowly progressing, mildly painful red lump swelling and tenderness of the overlying skin tion may overlap with other infectious
in the eyelid. Most settle with conservative and the adjacent nasoperiorbital area. There agents, a swab should be taken before treat-
treatment, namely warm compresses for 10 may also be tearing, discharge and fever. Pres- ment is commenced. An urgent Gram stain
minutes four times a day with light massage sure over the lacrimal sac may express pus may show Gram-negative intracellular diplo-
and chloramphenicol ointment for up to 4 from the punctum. This should be sent for cocci. Urgent ophthalmology consultation
weeks. If this treatment fails, or the cyst is culture. Staphylococcus aureus and Staphylo- should be sought and treatment should
excessively large causing visual disturbance, coccus epidermidis are the commonest organ- not be delayed due to the risk of rapidly pro-
it can be incised. It may become complicated isms. Treatment should be with cefalexin gressive corneal ulceration and perforation.
by preseptal cellulitis that may require sys- orally or cefazolin intravenously (IV) if unwell. Ceftriaxone 50 mg kg 1 IV for 7 days is used,
temic antibiotics. Gentle massage in the more minor cases may with the addition of erythromycin orally to
well aid in clearing the infection and unblock- cover for infection with C. trachomatis until
ing the duct, as dacryocystitis is invariably cultures are negative.
associated with ductal obstruction that may Chlamydial infection is classically asso-
Disorders of the lacrimal
be congenital or acquired. Probing or irriga- ciated with a watery then mucopurulent dis-
apparatus tion of the lacrimal system should be avoided charge 5 to 14 days after delivery. There is
Nasolacrimal duct obstruction in the acute setting and in infants less than 6 also palpebral conjunctival injection, but less
This condition occurs in 6% of infants and months old. Complications include preseptal lid oedema is seen than with gonoccocal
almost invariably resolves within the first cellulitis; orbital cellulitis and cavernous sinus infection. Swabs are sent for culture and
year. It may cause epiphoria (watering), a thrombosis occur very rarely. polymerase chain reaction and then treat-
mucoid discharge or some erythema of the ment is commenced with oral erythromycin
lids. There may be a minor mucopurulent Dacryoadenitis 10 mg kg 1 qid for 21 days. This disease
discharge and rarely a bluish palpable mass Infection of the lacrimal gland, found in the is complicated by pneumonitis in 1020%
will be seen overlying the drainage system, superotemporal orbit, is called dacryoadeni- of cases.
which represents a dacryocele. Treatment tis. Acute disease is characterised by abrupt For both of these conditions, the mother
of simple obstruction consists of gentle mas- onset of pain, swelling and erythema in will also need treatment and her partner will
sage four times a day and cleansing. Should the superotemporal region. There may also need screening.

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13.1 OPHTHALMOLOGICAL EMERGENCIES
13

EYES
The same organisms that affect older chil- The diagnosis of adenoviral conjunctivitis older children and adults use azithromycin 1 g
dren can also cause neonatal conjunctivitis. is clinical but swabs should be taken in most (20 mg kg 1 up to 1 g for children) as a single
These organisms typically present from day cases to exclude a bacterial cause. It is a dose. In prevalent areas, consideration should
5 to 7. Clinical findings do not distinguish highly contagious disease so household or be given to treating all household contacts.
the pathogen so cultures should be taken other social contact spread is likely. Treatment
and treatment commenced with broad- should be with cold compresses several times Episcleritis
spectrum antibiotic ointment. Herpes simplex a day, artificial tears and good hygiene. If This condition presents with acute redness to
virus (HSV) conjunctivitis is the exception and there is difficulty ruling out bacterial conjunc- a section of the sclera. There is usually mini-
should be suspected if there is a maternal his- tivitis clinically, antibiotic ointment should be mal discomfort or tenderness and it is only the
tory of infection, vesicular blepharitis or den- used. Parents should be advised that the child more superficial episcleral vessels that are
dritic ulceration. In this instance, treatment will be contagious for 12 days from onset. engorged. Although it is associated with con-
should be with topical and systemic aciclovir nective tissue disorders, HSV, varicella zoster
after urgent ophthalmological consultation. Allergic conjunctivitis, e.g. hay fever virus (VZV) and inflammatory bowel disease,
Presentation is similar to viral conjunctivitis most presentations are idiopathic in nature.
although a preauricular lymph node is not Treatment is symptomatic with artificial
Conjunctivitis in older children
present and there is usually a good history tears and oral non-steroidal anti-inflammatoriy
Conjunctivitis represents the commonest
of atopy. Treatment should consist of artifi- drugs (NSAIDs) but if severe, topical steroids
paediatric ophthalmic emergency presenta-
cial tears, oral antihistamines and removal can be used. Ophthalmic follow up should be
tion. Unlike the adult population, bacterial
of the trigger. arranged to ensure resolution.
infections predominate.

Atopic conjunctivitis Scleritis

Bacterial conjunctivitis This is commonest in male adolescents with In contrast to episcleritis, scleritis usually
This is characterised by epiphoria and a for- a history of atopy. It occurs in spring and presents with pain that gradually becomes
eign body sensation that progresses to diffuse summer and causes itching and a thick, ropy severe and a diffuse redness due to injection
conjunctival injection and a purulent dis- discharge. Large subtarsal papillae may also of the scleral, episcleral and conjunctival ves-
charge that may glue the lids together. The be seen. Treatment should be as for allergic sels. When viewed in daylight, the affected
onset is usually abrupt and spreads to the conjunctivitis, with the addition of sodium sclera may have a bluish hue and vision
other eye within 48 hours. Staphylococci, cromoglycate drops. may be reduced. There is a strong association
Streptococcus pneumoniae and Haemophilus with connective tissue disorders. Treatment
influenzae are the commonest organisms. Herpes simplex conjunctivitis consists of oral NSAIDs and steroids and
After swabbing, therapy is with chloramphen- Patients complain of a foreign body sensa- should be initiated by an ophthalmologist.
icol 0.5% 1 to 2 drops every 2 hours initially, tion, pain or burning and may give a history
tapering to 6-hourly as symptoms improve. of past ocular or perioral herpetic lesions.
In sexually active teenagers and iso- Examination will reveal a unilateral conjunc-
Corneal disease
lated communities in central and northern tivitis with vesicles on the eyelid or periorbi- Keratitis
Australia, N. gonorrhoeae or N. meningitidis tal skin and a palpable preauricular lymph Keratitis is defined as inflammation of one or
may be the causative organism and will node. Treatment is with topical antiviral more layers of the cornea. It appears as a focal
require systemic treatment with ceftriaxone medication and close ophthalmic follow up. white corneal opacity without an overlying
1 g intramuscularly for 3 to 5 days.
defect. If ulceration is also present it will stain
Trachoma with the use of fluorescein. Symptoms include
Viral conjunctivitis Trachoma results from recurrent infections pain, redness, photophobia and poor vision.
This is usually adenoviral in origin and find- of Chlamydia trachomatis. In Australia, it The lids may be erythematous or oedematous
ings include itching or grittiness, tearing, affects remote indigenous communities, whilst chemosis, tearing, conjunctival injec-
oedematous lids and a characteristic palpa- whilst internationally it is a leading cause tion, hypopyon or flare in the anterior cham-
ble pre-auricular lymph node. It usually of blindness in the third world. ber may all be seen on examination.
spreads to the other eye after a few days. Infection manifests as a mild mucopuru-
There may be corneal involvement as well, lent conjunctivitis that is self-limiting. How- Causes of keratitis
when it is known as epidemic keratoconjunc- ever, recurrent episodes result in a chronic Bacterial infection is the commonest cause of
tivitis. Adenovirus can also cause pharyngo- inflammatory state affecting the palpebral keratitis and this is considered to be one of the
conjunctival fever that, intuitively, comprises conjunctiva and cornea. Eyelid deformities leading causes of blindness in the developing
pharyngitis, conjunctivitis and fever. It is a due to scarring include entropion and ectro- world. Organisms include Gram positives, such
self-limiting disease but may very rarely dis- pion whilst corneal complications include as staphylococci, streptococci and bacilli, as
seminate and cause multiorgan failure. scarring (known as cicatrization), vasculari- well as Gram negatives. Of these, Pseudomo-
Other viral infections known to cause con- sation, ulceration and perforation. nas aeruginosa is of significance as it is the
junctivitis include rhinovirus, enterovirus, Treatment for children under 6 is by eryth- commonest cause of keratitis in the contact
influenza and respiratory syncytial viruses. romycin 10 mg kg 1 orally qid for 21 days. For lens wearer. Other organisms include:

325
13.1 OPHTHALMOLOGICAL EMERGENCIES

Fungi, particularly after trauma from inflammatory bowel disease, and psoriasis. admission for IV antibiotics, as for orbital cel-
vegetable matter. The course is usually Referral to an ophthalmologist should be lulitis, for all cases of presumed preseptal
more gradual in comparison to bacterial made to rule out any involvement of poste- cellulitis, particularly in the under 5s.
keratitis. rior structures. Treatment is with a cyclople-
gic agent and steroid drops. Orbital cellulitis
Acanthamoebic keratitis is a parasitic
Intermediate and posterior uveitis may The orbit is surrounded on three sides by
infection seen in contact lens wearers,
present with flashes, floaters and decreased paranasal sinuses. The migration of organ-
especially if they have poor lens hygiene.
vision. isms from an infected sinus into the orbital
It is also a complication of a corneal injury
cavity causes orbital cellulitis in the majority
contaminated by soil. Patients often
of cases. Haematogenous or dental spread
present with pain out of proportion to
Glaucoma can also occur and there may be an asso-
early clinical findings which progress over
ciated subperiosteal abscess. Infection is
several weeks. Glaucoma is very rare in children. An usually poly-microbial and causative organ-
HSV may cause a punctate keratitis enlarged, clouded cornea is seen associated isms are as for preseptal disease, with the
before the classical dendritic ulcer with tearing (epiphora) and photophobia. It addition of Gram negatives and anaerobes.
manifests. It may affect both eyes and may be associated with a hyphaema, lens Orbital infection presents with swollen, ery-
corneal scarring may be permanent. subluxation, retinoblastoma or systemic dis- thematous lids but, unlike preseptal celluli-
VZV is associated with a dermatomal ease (neurofibromatosis or SturgeWeber tis, the swelling should be limited to the
skin rash. syndrome) or high dose steroid use. Ophthal- orbital rim due to the septum preventing
mological referral should be made immedi- anterior spread. The fever is likely to be
Management ately as prompt diagnosis is essential to higher than that seen with preseptal celluli-
All patients with suspected infectious kerati- prevent visual loss. tis and the child usually appears toxic. There
tis should be urgently referred to an ophthal- may be chemosis and proptosis and eye
mologist for culture by corneal scraping movements are likely to be restricted and
before starting antibiotic treatment. Orbital and preseptal painful. A raised white count is expected
(periorbital) cellulitis but will not aid differentiation from presep-
Recurrent corneal erosion The fibrous orbital septum is a continuation tal infection. Blood culture should be taken
This manifests with recurrent attacks of pain, of the periosteum of the orbital rim. It con- and then a CT scan or MRI will confirm the
photophobia, grittiness and tearing, which nects to the tarsal plates and separates the diagnosis and guide drainage of the offend-
occur on waking or rubbing the affected eye. eyelids and other preseptal structures from ing sinus or subperiosteal abscess. Therapy
There is often a prior history of corneal abra- the orbital space, acting as a barrier to the should be with IV flucloxacillin and ceftriax-
sion. Examination may often reveal a corneal spread of infection. It is important to distin- one and urgent referral should be made to
defect but minor epithelial changes may have guish clinically or radiologically between the ophthalmology as well as ear, nose and
resolved by the time the patient is reviewed. two conditions, as missing the diagnosis of throat for consideration for a sinus drainage
Treatment consists of antibiotic ointment and orbital cellulitis may have grave sequelae. procedure. Complications include optic
then artificial tears once the corneal defect is nerve compression, reduced visual acuity,
healed. Ophthalmic follow up is recommended. cavernous sinus thrombosis, osteomyelitis,
Preseptal cellulitis
Of these two conditions, preseptal cellulitis meningitis and cerebral abscess.
is far more common, accounting for 95%
Uveitis of infections around the eye. It presents with
Acknowledgement
Inflammation of the uveal tract is subdivided lid oedema and erythema, mild pain and a
according to location of disease (anterior, low-grade fever. The child is unlikely to The contribution of Adrienne Adams as author
intermediate or posterior) or structure appear ill. There is usually a portal of entry in the first edition is hereby acknowledged.
involved (iris, choroid, retina). for the organism such as a nearby wound or
The most frequently encountered of insect bite, lid lesion or a pre-existing dacryo-
these conditions is that of traumatic iritis. cystitis. Rarely, haematogenous spread can Further reading
Bravermann RS. Eye. In: Hay WW, Levin MJ, Sondheimer JM,
This presents with photophobia, epiphoria, occur. Staph. aureus, Strep. pneumoniae or Deterding RR, editors. Current pediatric diagnosis &
blepharospasm, perilimbal injection and H. influenzae in the unvaccinated are the treatment. 19th ed. New York: McGraw Hill; 2008.
Christiansen K, Currie B, Ferguson J, et al., editors. Antibiotic
blurring of vision 1 to 3 days after a blunt commonest organisms in the under 5s. Cul- Guidelines 2006. Version 13. Therapeutic Guidelines
globe injury. The pupil may be miotic or tures should be taken as appropriate and Limited.
Ehlers JP, Shah CP. The Wills Eye Manual. 5th ed. Baltimore:
irregular and cellular flare may be seen in if the child is well and the symptoms minor, Lippincott Williams & Wilkins; 2008.
the anterior chamber upon slit lamp exami- treatment can be as an outpatient with Greenberg MF, Pollard ZF. The red eye in childhood. Paediatr
Clin N Am 2003;50:10524.
nation with an oblique beam. Relief of pain augmentin or cefalexin for 7 days as long Prentiss KA, Dorfman DH. Pediatric ophthalmology in the
with topical local anaesthetic is a relatively as appropriate follow up will occur to en- Emergency Department. Emerg Med Clin N Am
2008;26:18198.
sensitive way of excluding the disease. Other sure that symptoms are improving. This is, Rubenstein JB, Virasch V. Conjunctival disease. In: Yanoff &
causes include juvenile rheumatoid arthritis, however, contentious and some advocate Duker: Ophthalmology. 3rd ed. New York: Mosby; 2008.

326
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EYES
13.2 Congenital, developmental
and neoplastic conditions
Greg Stevens

ESSENTIALS Ocular tumours

1 These conditions are most likely to present with a concern from a parent or Tumours may present with an evident defor-
caregiver who has noted that there has been a change in the appearance of their mity, with proptosis, or with an alteration in
childs eyes, or that they are not responding to visual cues in an expected way. the visual axis. Any of the tissues in the orbit
and eyelids may give rise to tumours. Chil-
dren treated in infancy with radiotherapy
for an intraorbital tumour are at increased
A detailed examination will help to further image provided to the retina. It is usually
risk of developing malignancies in the
define the abnormalities or deficits noted. unilateral. Strabismus may also be an early
radiated area in later life.
These conditions require specialised investi- sign of significant visual pathology, e.g. reti-
gations and management, and as such con- noblastoma, retinitis of prematurity and
sultation with a paediatric ophthalmologist Coats disease.
is advised. Retinoblastoma
This is the most common primary intraocular
Paediatric cataracts tumour in childhood.
Deficits in visual acuity
Hereditary and non-hereditary forms
These can be detected as an abnormality of
This may reflect a deficit anywhere from exist. The heredity forms are usually bilat-
the red reflex. There is an association with
the visual cortex to the cornea and a care- eral and multifocal. There is a defect of a
several chromosomal, metabolic and intra-
ful examination including pinhole testing tumour suppressor gene located on chromo-
uterine infective causes.
in children old enough to co-operate will some 13.
help localise this and guide appropriate When the tumour is confined to the eye
referral. there is a good survival rate (>90%).
Congenital nasolacrimal
duct obstruction
Strabismus This is a common disorder, occurring in up to Rhabdomyosarcoma
Abnormalities of the binocular alignment of 6% of children. It presents with excessive
the eyes, strabismus, may present with a overflow of tears. It has a good outcome, This is the most common primary intraorbi-
concern from a parent that their child has with 96% resolving by the age of 1 year. tal tumour in childhood and usually occurs in
a squint, and this may be confirmed by care- the first decade.
ful examination of the corneal reflex and use Presentation may be rapid with asso-
of the cover-uncover test. ciated eyelid oedema, and can lead to confu-
Strabismus may be convergent (esotro-
Infantile glaucoma sion with an infective process.
pia), divergent (exotropia), upwards (hyper- Glaucoma in children is rare, but if unde-
tropia) or downwards (hypotropia). tected may cause blindness.
Strabismus may be a dynamic process, Because of the elasticity of the paediatric
occurring in relation to alterations in the eye, significant enlargement of the cornea
Neuroblastoma
accommodative reflex with refractive errors. may occur, with subsequent clouding of The orbit is a common site of metastasis of
The importance of strabismus lies in the the cornea. Excessive lacrimation and photo- neuroblastomas. The primary is usually
development of amblyopia, which is the phobia may be present. It is a cause of irrita- abdominal and usually known at the time
decrease in visual acuity occurring in visually bility in the infant. of orbital metastasis. Most occur in the first
immature children due to the lack of a clear The treatment is usually surgical. 5 years of age.

327
 13.3 Ocular trauma
Jennie Martin

the pupillary shape, size and reaction to

ESSENTIALS light;
ocular movements in all directions of gaze
1 Anxiety is pronounced in the carer, be calm in your approach. (nystagmus, diplopia, pain or limitation of
2 Consider hidden injury. movement);
inspect the lids;
3 Never apply pressure to an eye that may be ruptured or penetrated. palpate orbital rims for steps or
4 Commence immediate treatment with chemical injury. tenderness;
test for infraorbital nerve sensation;
the position of the globes (exophthalmos,
enophthalmos);
Begin by taking a careful history. In addi- the conjunctival surface of eye lids (the
Introduction tion to aspects of history common to all pre- deep superior fornix requires lid eversion
Injury is the leading cause of visual disability sentations, ask specifically about existing for viewing);
and blindness in children and has the follow- eye disorders, the mechanism of injury and conjunctiva and sclera for laceration or
ing features subsequent events. foreign body;
Often the injury is unwitnessed or the red reflex;
severe eye injury is not uncommon; child may be frightened and so the history corneal surface (use fluorescein staining
all age groups may be affected, peak may be vague or concealed. Have a high where possible);
1115 years;
index of suspicion for hidden injury. Specifi- examine the anterior chamber depth and
male predominance after infancy; cally ask for visual symptoms of reduction clarity.
usually unintentional, age-related or change in vision. Children are prone to
patterns;
the oculocardiac reflex and a history of bra-
causes include sports activities (balls or dycardia, nausea, somnolence or syncope
clashes); projectiles; plant thorns; pet
strongly suggests a significant injury. Trauma
bites/scratches; elastic/bungee cords;
Perform the non-invasive aspects of the
fireworks;
examination first. Reassure the patient
Lid lacerations
education is important as up to 90% of
and carer that you will not hurt them. Dim
A laceration to the eyelid may be partial or
injuries are preventable; full thickness and may involve the lid mar-
the room lights if possible, keep the ophthal-
visual impairment results in an
moscope light to a minimum. Be systematic
gins, canthal tendons, levator complex or
emotional, social and economic cost; canalicular system.
and touch last. Importantly, know when to
there are unique challenges to obtaining
stop and refer.
Perform a thorough and complete eye
a history and examination in the child. examination to exclude an injury to the
Document the visual acuity in each eye.
globe (see ruptured globe below). Pressure
Trauma to the eye engenders a marked anxi- Visual acuity testing should be adjusted to
exerted by attempts at cleaning and repair
ety reaction in the carer who is always con- the age and ability of the child. Fix and fol-
may apply pressure to a potentially ruptured
cerned about long term visual impairment. low testing, ability to reach for a small toy
globe. Children who are unable to co-operate
Use a careful and calm approach to enable with one or the other eye covered, an Allen
enough to allow accurate assessment of
co-operation so a thorough examination chart using pictures (allow the child to iden-
wound depth should be referred for examina-
may be performed. Ensure the parent/carer tify the pictures closely first), a Tumbling E
tion under anaesthesia. If a globe injury is
is with the child at all times. chart (described as table legs pointing in
suspected, apply a rigid shield, fast the
Always consider what may lie beneath an different directions) or a formal Snellen
patient and refer immediately.
injury that appears to be superficial. Even in chart. A difference of two or more lines is sig-
The mechanism of injury should be deter-
an unco-operative child, extensive informa- nificant. Remember the child has a low
mined to assess the risk of a foreign body
tion can be obtained by observation alone. attention span, so do not insist on them
(e.g. windscreen shattering), whether a bite
Uncommonly, mild sedation may be required; reading every line. If acuity is markedly
(human or animal) and whether significant
however, when there are genuine concerns, reduced, use finger counting or light percep-
contamination may have occurred.
referral for general anaesthesia to enable tion at close range.
Indications for emergency ophthalmo-
adequate examination is preferred. If gentle Follow with a visual inspection. If appro-
logic consultation include:
restraint has not facilitated examination or priate, relieve pain with topical anaesthesia
a particular procedure, repeated and forcible early to assist examination. bites and those with significant
restraint should not be performed. Examine and document: contamination requiring debridement;

328
13.3 OCULAR TRAUMA
13

EYES
lacerations involving the eyelid margin to not be forcibly opened. If a strong index of bleeding diathesis;
prevent notching; suspicion exists, place a rigid shield and refer. Valsalva (cough, heaving lifting,
lacerations on the nasal side of the eyelid When swelling limits direct visualisation of straining).
punctum due to the risk of injury to the the eye, ocular ultrasound is used in some
Pain with extraocular movement, reduced
canalicular system; centres to allow non-invasive assessment.
vision, hyphaema, pupil abnormality and /or
lacerations with involvement of the After other injury is excluded, the eyelid
bloody chemosis raise suspicion of a globe
levator aponeurosis of the upper eyelid haematoma requires no specific manage-
injury. A 360 degree subconjunctival hae-
(which produces ptosis) or the superior ment. Cold compresses may provide analge-
morrhage should be referred urgently for
rectus muscle. sia for the first 24 hours. The eye should be
ophthalmological review as the globe may
exposure of orbital fat this suggests the re-examined in 24 hours to ensure an injury
be ruptured posteriorly. No specific treat-
orbital septum has been lacerated as has not been missed. Swelling and bruising
ment is required for an isolated subconjunc-
there is no subcutaneous fat in the lids may appear to extend down the cheek or
tival haemorrhage. The haemorrhage will
themselves; to the other eye.
clear spontaneously within 12 weeks.
lacerations with significant tissue loss.
Conjunctival lacerations present as a red
Wounds requiring referral should be cleaned Orbital fracture eye with a foreign body sensation and usually
with normal saline and have foreign mate- Suspect fracture when there is tenderness of a history of trauma. Conjunctival and subcon-
rial removed as much as possible. Following the orbital rim or crepitus of the lid indicat- junctival haemorrhages are often associated.
cleansing, the wound should be covered ing a sinus fracture. There is often a haema- The conjunctival edges can be separated
with a saline soaked dressing, prophylactic toma of the eyelid. Blow-out fractures gently with a moist cotton tipped applicator
antibiotics commenced for bites or signifi- (fracture of the orbital floor
the medial following topical anaesthesia to assess the
cantly contaminated wounds and tetanus wall) from blunt trauma are typically seen depth of injury. If the diagnosis is uncertain,
status considered. in adolescents. refer to ensure a scleral perforation or sub-
If the laceration is suitable for repair in Look for conjunctival foreign body is excluded. If the
the emergency department, the eyebrow restricted ocular movements which may conjunctival laceration is isolated, treatment
should not be shaved as long-term cosmetic produce diplopia, usually on upward with antibiotic ointment or drops is required
alterations may result and the hair direction gaze; for 47 days. They rarely require repair.
assists in correct alignment of the wound. enopthalmos; and Corneal lacerations may be partial (not
Tissue should not be removed, as the good infraorbital nerve anaesthesia. into the anterior chamber) or full thickness
blood supply of the eyelid generally ensures (ruptured globe). Complete examination is
viability. Partial thickness lacerations should Nausea and vomiting may occur. Facial com- required to exclude a penetrating injury of
be repaired with 6/0 synthetic suture and puterised tomography (CT) with axial and the cornea or sclera. Ensure the anterior
full thickness lacerations should be repaired coronal views, ophthalmological and facio- chamber is of normal depth and there is no
in layers. In general, non-absorbable sutures maxillary referral is required. Ensure a globe hyphaema. Superficial partial thickness
should be removed in 47 days. Tissue glue or central nervous system (CNS) injury is not lacerations will heal spontaneously with anti-
is not advised due to proximity to the lashes present, as this will have greater priority. Eye biotic cover; however, daily review is necessary
and cornea. injury may occur in over 50% of these frac- to exclude the development of infection until
tures, with globe rupture in 510%. If prom- healing is complete. Deep partial thickness
inent symptoms of the oculocardiac reflex lacerations should be referred for consider-
Ecchymosis are present, immediate repair is indicated. ation of repair. Seidels test can be used (see
A black eye is a common injury and may be If not, surgery may be arranged non- ruptured globe). For management of full thick-
limited to a minor ecchymosis of the lid or urgently. Instruct the patient not to blow ness lacerations refer to ruptured globe.
extend to a periorbital haematoma with sig- the nose, as orbital emphysema may occur. Corneal abrasions: 75% of ED visits are
nificant oedema. Always consider a blow-out
related to corneal abrasions, conjunctival
fracture of the orbit, globe injury and/or
Conjunctival haemorrhage, or corneal foreign bodies and conjunctivitis.
base of skull fracture or anterior fossa frac-
lacerations Corneal abrasions are very painful. Finger-
ture (suggested by a subconjunctival hae-
Subconjunctival haemorrhage presents as a nails, sticks or foreign body may be the cause.
morrhage without posterior limit). Always
red eye with a painless collection of bright, They may present with:
document the ocular motility. Care must be
smooth blood confined to a sector of the bul-
exercised when opening the lids so that pres-
bar conjunctiva which is sharply demarcated
tearing;
sure is not exerted on the globe until a rup-
at the limbus and does not pass beyond the
a foreign body sensation;
tured globe is excluded. If the eyelids are
limbus. The visual acuity is normal.
photophobia;
markedly swollen, examination of the eye
Causes include:
visual acuity may be reduced, dependent
is difficult. Placing ones thumb on the infra- on the size of the abrasion and the
orbital and supra orbital rims, the swollen trauma; position over the visual axis;
eyelids can be separated without placing idiopathic; conjunctival injection, iritis and eyelid
pressure on the globe. The eyelids should hypertension; swelling may be associated.

329
13.3 OCULAR TRAUMA

The application of a topical local anaes- 13-mL syringe, bevel angled away from the Assess with a slit lamp and fluorescein stain.
thetic provides temporary pain relief and will eye, to gently scrape the foreign body from Moderate or severe burns are suggested by
assist in allowing the eye to be opened for the cornea. Referral should be made if the significant epithelial loss, chemosis, corneal
examination. In a non-compliant child, place foreign body is central or deep or if the child oedema or haziness, blanching of the con-
a drop at the medial canthus and when the is not co-operative. Post removal, complete junctival vessels or opacification. Refer
eye is opened, the drops flow in. The non- examination of the eye using fluorescein. immediately. All patients require treatment
verbal child may present simply with undif- If a rust ring or residual foreign body with antibiotic drops/ointment and analge-
ferentiated distress with or without refusal remains, next day referral should be arranged. sia
cycloplegics.
to open the eye and topical anaesthetic Refer patients with central or large abrasions Thermal burns are managed similarly to
may be diagnostic. for daily review. Topical antibiotic treat- abrasions. UV keratitis may result from weld-
Topical anaesthesia should never be ment should be commenced. A topical non- ing, sun lamp exposure or excessive sunlight.
provided to the patient for continued instal- steroidal anti-inflammatory drug (NSAID) The symptoms develop several hours after
lation and home use as this may impair heal- provides effective analgesia and if there exposure with pain, tearing and red eye.
ing, inhibit protective reflexes and permit is severe pain, a cycloplegic (tropicamide There are usually bilateral superficial cor-
further injury. 0.51%, cyclopentolate 0.52%) may be neal defects seen on fluorescein staining.
Abrasions may be associated with a for- prescribed to relieve ciliary spasm (avoid in Treatment is with topical antibiotics
a
eign body on the lid conjunctiva, which must infants). Ensure tetanus prophylaxis. Eye cycloplegic.
be everted to be examined fully. An upper lid patching does not reduce pain or aid healing, Traumatic iritis presents with the onset of
foreign body is suggested by a linear vertical may cause difficulty walking in children and a dull, aching pain, photophobia, and tearing
abrasion. The inner surface of the upper lid thus should be avoided. within 3 days of trauma. Possible signs: small
is examined by asking the patient to look Contact lens wearers should be referred pupil; perilimbal injection of the conjunctiva
down, applying a cotton bud to the lid for follow up and require pseudomonal cov- and pain in the affected eye when a light is
crease and applying light pressure. Use the erage (tobramycin or ciprofloxacin). The lens shone into either the affected or non-affected
eyelashes to pull the everted lid over the should not be worn until the defect has been eye; reduction in visual acuity; hyphaema.
bud, away and up from the globe. Hold healed for a week. White blood cells and a flare are seen within
the lashes against the orbital rim to keep Chemical burns are unusual in childhood, the anterior chamber when examined under
the lid everted. To return the lid, release but are potentially very serious. For all chem- the slit lamp. This is best seen by placing
the pressure and ask the patient to look ical burns, irrigation should begin immedi- the slit lamp beam at 45 degrees with full
up. The lower fornix is easily inspected by ately. Anaesthetise the eye (procedural intensity and a short narrow slit. The appear-
applying downward pressure to the lower sedation may be required in young children) ance is like dust in a room illuminated with a
lid while the patient looks up. and then copiously rinse the eye with at torch. Refer immediately.
Diagnose the abrasion by demonstrating least 12 litres of Hartmanns or normal Hyphaema is blood in the anterior cham-
a staining defect with fluorescein using saline (warm if possible) for at least 30 min- ber and may result from blunt or penetrating
either an ophthalmoscope (12 magnifica- utes. Use IV tubing connected to the bag of trauma. Presents with pain, photophobia,
tion) or a slit lamp. Use only a small amount solution and direct the flow from medial to mydriasis/miosis, reduced visual acuity or
of fluorescein as excessive dye can mask the lateral onto the conjunctiva, not cornea. blurred vision. The red reflex will be intact.
defect. The abrasion will appear bright Ensure the fornices are irrigated by everting If the pupil is dilated, it is important to
green when viewed under a blue light. the lid. A Morgan lens (a moulded lens differentiate between traumatic mydriasis
Conjunctival foreign bodies (palpebral or applied to the eye) allows continuous irriga- (present only in the affected eye) and an
bulbar) should be removed after topical tion if available. Five minutes after irrigation afferent pupillary defect (swinging light
anaesthesia, with gentle irrigation or a has been completed, check the pH with lit- test paradoxical initial dilatation of the
moist cotton tipped swab. mus paper and continue irrigation until a affected pupil occurs when light is shone
Corneal foreign bodies: Assess for and pH > 7 is achieved A urine dipstick can be quickly from the unaffected to the affected
refer those with an intraocular foreign body used (trim with scissors to retain the pH eye); this may suggest an optic nerve or
immediately (see penetrating injury). Always section). Check the pH again 20 minutes severe retinal injury. The size may vary from
document the visual acuity. Topical anaesthe- after irrigation to ensure there is no rebound microscopic hyphaema to blood involving
sia is usually required to relieve pain and fall. Once irrigation is completed, examine the whole anterior chamber. Urgent ophthal-
blepharospasm, enabling examination. for retained foreign bodies. Particulate mat- mological referral is required for all children.
Removal requires adequate magnification ter requires removal (moist cotton bud or Treatment includes restricted activity, eye
and illumination. Foreign bodies may be toothed forceps) and this may require gen- shield, anti-emetic, cycloplegic and topical
removed by irrigation; a cotton tipped appli- eral anaesthesia. Alkali burns (dishwasher steroid in some children. NSAIDs and aspirin
cator or needle removal. Needle removal of a liquid, oven cleaner) produce liquefactive should be avoided. Always consider non-
superficial foreign body must occur at the necrosis and are often more severe than acid accidental injury. Rebleeding occurs in up to
slit lamp and this will require a co-operative burns. one-third of patients, usually after a few days.
child. Approaching from the temporal side, Once irrigation has been completed, Ruptured globe and penetrating eye
use a 25-gauge needle attached to a assess injury severity and document acuity. injury: A ruptured globe occurs when the

330
13.3 OCULAR TRAUMA
13

EYES
integrity of the sclera or cornea is disrupted DO NOT apply pressure to the globe if inadequate. Retinal haemorrhage due to
by blunt trauma or direct perforation. There penetration or rupture is suspected. inflicted injury is typically seen in the child
may be surprisingly few signs. Suspect if NEVER attempt to remove a protruding <1 year and often there are associated
there is a peaked pupil (apex of the teardrop foreign body from the globe. injuries.
points to the perforation), which may be NEVER force the lids open.
the only clue to occult rupture. Chemosis Do not use further eye drops and never
overlying the laceration, subconjunctival use eye ointment on an open globe.
haemorrhage, corneal or scleral laceration, Further reading
American Academy of Pediatrics. Policy Statement.
distortion of the anterior chamber (deep Rest the child in bed, head up. Protect the Eye Examination in Infants, Children, and Young Adults
or shallow), bubbles in the anterior cham- eye with a rigid eye shield. If a formal eye by Pediatricians. Pediatrics 2003;111(4):9027.
Babineau MR, Sanchez LD. Ophthalmologic procedures in the
ber, extrusion of the intraocular contents, shield is unavailable, one can be created emergency department. Emerg Med Clin N Am
hyphaema, or loss of ocular motility may from the base of a polystyrene cup. Keep 2008;26:1734.
Curryn KM, Kaufman LM. The eye examination in the
be indicative. A Seidels test can be used if nil by mouth. Commence an anti-emetic pediatricians office. Pediatr Clin North Am 2003;50
the diagnosis is unclear. Apply a moistened and analgesia, ensure tetanus prophylaxis (1):2540.
Ehlers JP, Shah CP, editors. Wills Eye Manual: Office and
fluorescein strip over the potential site of and systemic antibiotic prophylaxis. Contact Emergency Room Diagnosis and Treatment of Eye Disease.
perforation. Use the blue light of the slit the ophthalmologist urgently. If an intraocu- 5th ed. Baltimore: Lippincott Williams & Wilkins; 2008.
p. 1248.
lamp and the leak from a perforation will lar foreign body or globe rupture is sus- Danis RP, Neely D, Plager DA. Unique aspects of trauma in
manifest as a green dilute aqueous stream pected CT will usually be required. children. In: Kuhn F, Pieramici DJ, editors. Ocular Trauma:
Principles and Practice. Chap 30. Stuttgart: Thieme; 2002.
within the darker, concentrated orange dye. Non-accidental injury may result in any p. 30717.
Once perforation or penetration of the eye injury. A high index of suspicion is Levine LM. Pediatric ocular trauma and shaken infant
syndrome. Pediatr Clin N Am 2003;13748.
eye is suspected, further emergency depart- required if the injury is inconsistent with NSW Health. Eye Emergency Manual, An Illustrated Guide.
ment examination is unnecessary. the given history or the history is NSW Health; 2007.

331
SECTION

14 ENT AND DENTAL

Section editor Jeremy Raftos

14.1 The ear 332 14.4 Retropharyngeal abscess 343

14.2 The nose 336 14.5 Foreign bodies and caustic ingestion 344
14.3 The mouth and throat 338

14.1 The ear

Stuart Lewena Gervase Chaney

Examination
ESSENTIALS Oedema and erythema of the canal with
serous or purulent discharge is usual. The tra-
1 Otitis externa usually results from excessive exposure to heat and moisture gus is tender to manipulation. With increased
and is usually very painful. Treatment involves cleaning, keeping dry and topical severity, the canal becomes occluded with
antibiotics. periauricular oedema and may progress to
2 Acute otitis media is a very common emergency presentation, but not all red ear otitis externa with cellulitis when the child
drums are due to otitis media. Management with adequate analgesia is essential. becomes febrile with a toxic appearance.
However, antibiotic use should be restricted to specific circumstances. Differentiation from acute otitis media
with perforation or chronic suppurative oti-
3 Discharging otitis media due to chronic suppurative otitis media usually presents tis media is important. Usually in these con-
with painless and offensive discharge. Treatment is with ear toilet and topical ditions, the tragus and canal are not tender
antibiotics. Oral antibiotics have little or no role. and there is no erythema and oedema of the
4 Otitis media with effusion is very common in children, but treatment is unnecessary canal.
in the majority, with resolution over 3 months. Acute localised otitis externa (furuncu-
losis) occurs in the posterosuperior aspect
5 Mastoiditis continues to be a problem in the antibiotic era and in many cases is the on the ear canal. Otomycosis has only mild
first presentation of ear disease. Admission, myringotomy and IV antibiotics
canal wall inflammation and thick otorrhoea.
constitute the mainstays of medical management.

6 Ear trauma is uncommon. Accidental ear injuries are usually unilateral and Investigations
isolated. Ear trauma is rare in the first year of life and may indicate non-accidental Investigations are largely unnecessary and
injury. Haematomas should be removed by aspiration or excision. rarely alter empiric treatment. The organ-
isms found in diffuse otitis externa are most
commonly Pseudomonas aeruginosa and
Staphylococcus aureus. Furunculosis is usu-
of the protective coating of the ear canal, ally S. aureus and otomycosis Aspergillus
Otitis externa including cerumen and obstruction of the or Candida species. Consideration of cul-
Introduction ear canal. Allergy may also play a role. tures aerobic, anaerobic and fungal is
Otitis externa includes various conditions worthwhile in cases resistant to routine ther-
from the most common acute diffuse otitis History apy or if there is more extensive disease
externa (swimmers or tropical ear) to otomy- It may initially present with aural fullness such as associated cellulitis.
cosis, localised (furunculosis) or chronic otitis or itch, but usually progresses to pain with
externa. It occurs commonly in hot, humid or without discharge. The pain is often Treatment
climates or in the summer of temperate cli- severe and is worse with chewing. Acute diffuse otitis externa is managed
mates. Risk factors also include swimming Otomycosis or fungal otitis externa makes up with frequent gentle cleaning of the canal,
and other water exposure, local trauma, loss 10% of cases and has a more insidious onset. along with avoidance of water (swimming

332
14.1 THE EAR
14
is 45 to 90 mg kg
1 per day. Amoxicillin

ENT AND DENTAL

prohibited). Ototopical medications are the may occur (see complications). These symp-
mainstay of treatment, usually topical anti- toms may present as a primary complaint or clavulanate is the next choice for poor
biotics such as framycetin/gramicidin/ frequently occur in the course of an upper responders. Cefaclor has a significant rate
dexamethasone combinations. Ciprofloxacin respiratory infection. of serum sickness reactions in children and
is also effective. Insertion of a wick or ribbon should not be used.
gauze is also helpful. Close follow up for Examination Topical otic antibiotic preparations may
repeated cleaning with or without wick rein- A red, bulging, non-mobile tympanic mem- be used instead of oral antibiotics in cases
sertion may be required. brane is the most reliable constellation of with tympanic membrane perforation or
Furunculosis treatment is by local heat signs. Redness of the eardrum is a non- those with intact tympanostomy tubes when
application and oral antibiotics (flucloxacil- specific finding and may be seen with a high purulent otorrhoea is the prominent finding.
lin or cephalexin) or incision and drainage. fever or following crying. Alone, therefore, it
Otomycosis will require canal cleaning and is not diagnostic of otitis media and is an Complications
antifungal drops or gentian violet. inadequate finding to make the diagnosis. The most common complication is perfora-
tion of the drum and otorrhoea. Other com-
Prevention Investigations plications are very unusual but potentially
Keeping the ear canal dry and avoidance of The diagnosis of otitis media is made solely severe. Most are due to bacterial spread
trauma to the canal are the mainstays of on clinical grounds and investigations are and include extracranial complications such
prevention. rarely performed. In cases where tympanost- as mastoiditis, cholesteatoma and facial
omy has been performed, two-thirds are nerve paralysis and intracranial complica-
Complications bacterial-culture positive, with a predomi- tions such as epidural abscess, meningitis
Progression to cellulitis of the nearby skin/ nance of Streptococcus pneumoniae, Morax- and lateral sinus thrombosis.
soft tissue and/or lymphadenitis may occur. ella catarrhalis and Haemophilus influenzae. Persistent middle ear effusions are almost
Oral antibiotics are usually then indicated, universal after an episode of acute otitis
with cephalexin a reasonable first choice, Treatment media and should not be viewed as a compli-
but antipseudomonal antibiotics may be The administration of adequate analgesia is cation. Complete resolution over several
necessary. Progressive cellulitis and a toxic- paramount to the management of acute oti- months occurs in 90% of cases.
appearing child will require admission for tis media. Paracetamol alone may not be
intravenous antibiotics, including pseudo- adequate and the combination with codeine Prevention
monas cover. Less commonly, involvement may be required. Topical instillation of lido- Prophylactic antibiotics confer a small
of the parotid gland, temporomandibular caine 2% has been shown to be a useful decrease in recurrence at best, and are likely
joint or base of skull may occur. adjunct for rapid pain control but should to contribute to increasing antibiotic resis-
Chronic otitis externa may occur, and may be combined with longer-acting oral analge- tance and generally are not recommended.
be a sign of an underlying dermatological dis- sia. Decongestants and antihistamines have More appropriate means of reducing recur-
ease such as seborrhoeic or atopic dermatitis. not been shown to be effective and are not rence include avoidance of passive smoke
recommended. exposure, reducing day-care attendance and
The majority of cases of otitis media will immunisation (pneumococcal and influenza).
Acute otitis media resolve spontaneously. However, antibiotics
Introduction continue to be widely used. In an otherwise
Acute otitis media (AOM) is one of the most healthy child over 2 years, most authorities Discharging otitis media
common primary care paediatric presenta- now recommend deferring antibiotic use chronic suppurative otitis
tions. It occurs as a result of infection of for 2 to 3 days, and to commence treatment
media
the middle ear cavity by both viral and bac- only if the child remains symptomatic at
terial organisms. It is frequently over diag- review. Approximately 80% of children will Introduction
nosed and remains a common cause for avoid antibiotic use with this approach. Pro- Persistently discharging otitis media is most
excessive antibiotic use. Adequate analgesia vision of a prescription upfront, with advice common in developing countries and certain
and a selective approach to antibiotic use to commence antibiotics in 2 to 3 days if the high-risk populations in developed nations,
are the mainstays of management. child remains unwell, has been shown to such as Aboriginal Australians. It generally
result in approximately 50% of children occurs following perforation of the eardrum
History avoiding antibiotics. Both strategies are rea- from acute otitis media. It may also occur as
Classic symptoms include fever, malaise and sonable, with the latter chosen in cases a complication of tympanostomy tube
ear pain. The pain can be severe and during where access to timely medical review is (grommet) placement.
the night may wake the child. Other systemic uncertain. Early antibiotic therapy continues
features such as nausea and vomiting can to be advocated in the very young or those History
occur. In younger children, presentation is with comorbidity. There is usually an absence of pain and a var-
often non-specific, with crying and irrita- Amoxicillin is a reasonable first-line anti- iable history (often weeks) of discharge that is
bility. Eardrum perforation and otorrhoea biotic choice. The usual recommended dose purulent and offensive. It is often recurrent.

333
14.1 THE EAR

Examination Investigations inflammation and tenderness, and narrow-

The canal is usually non-tender and there is No acute investigations are indicated. In ing of the external auditory canal. The tym-
usually no inflammation or some chronic persistent cases, referral for audiology is panic membrane is usually abnormal and
inflammation. If the tympanic membrane recommended to determine any significant may be perforated.
can be visualised, usually only after ear toi- hearing loss.
let, there will be a perforation or tympanost- Investigations
omy tube in situ. Treatment Increased routine use of computerised
As the majority of OME cases will resolve tomography scanning is due to the difficulty
Investigations spontaneously (90% by 3 months) a period in diagnosis of subperiosteal abscess by clin-
Investigations are largely unnecessary. If of observation is recommended. Persistent ical examination alone. Magnetic resonance
performed, the organisms found on ear OME is more likely to follow acute otitis imaging may also be valuable. Bacteriologi-
swabs are most commonly Pseudomonas media in the first year of life. cal diagnosis can be made at the time of
aeruginosa and Staphylococcus aureus. Trials of many different treatments includ- operative treatment. Cultures show Strepto-
ing antibiotics, nasal decongestants, nasal coccus pneumoniae, Strep. pyogenes and
Treatment insufflation, and corticosteroids have failed Staph. aureus to predominate but many
Ear toilet (using a dry tissue spear) and topi- to show benefit. Persistent OME with con- organisms are possible. P. aeruginosa can
cal antibiotics, particularly quinolones (such cerns of significant hearing loss is an indica- be seen in chronic or recurrent cases.
as ciprofloxacin with hydrocortisone) or fra- tion for audiology and referral to an ear,
mycetin/gramicidin/dexamethasone com- nose and throat (ENT) surgeon for consider- Treatment
binations, have been demonstrated to be ation of tympanostomy tubes (grommets). Management has historically been cortical
effective in acute resolution of otorrhoea. mastoidectomy. However, a number of series
Long-term outcomes are still to be deter- Complications report successful treatment in the majority
mined. Systemic antibiotics alone are not The principal concern for persistent OME is of cases with myringotomy and intravenous
as effective and addition to topical treat- conductive hearing loss and potential impact antibiotics. Broad-spectrum antibiotics such
ment does not improve outcome. on language and cognitive development. as third-generation cephalosporins are gen-
There are potential long-term changes to the erally recommended, although antipseudo-
Complications tympanic membrane and middle ear that monal antibiotics may be required.
Chronic perforation and discharge are the may cause hearing loss (e.g. tympanosclerosis).
main issues, although hearing impairment Complications
may be a problem. Cholesteatoma occurs Complication rates are significant (1335%)
in a small proportion of affected children. Mastoiditis and include subperiosteal abscess, facial
nerve paralysis, sigmoid sinus thrombosis,
Introduction epidural abscess and meningitis.
Mastoiditis is the infection of the mastoid air
Otitis media with effusion
cells. It is an infrequent illness with a rate of
Introduction between 1.2 and 4.2 per 100 000 person
Trauma
Otitis media with effusion (OME) is the pres- years in developed nations. Presentation
ence of a middle-ear effusion in the absence can occur at any age, with a median of Introduction
of acute inflammation. It is unlikely to be 1248 months. There is some evidence that Paediatric ear trauma is an uncommon pre-
a presenting complaint in an emergency decreased use of antibiotics for AOM has sentation to an ED. Accidental ear trauma
department (ED) setting. It is more likely resulted in a small increase in cases of mas- is almost always unilateral. There is the usual
to be an incidental finding. It is extremely toiditis. However, it is estimated that approx- male predominance, with a majority between
common, particularly in pre-school children. imately 5000 children with otitis media 1 and 7 years of age. Accidental ear trauma is
Its significance is in relation to its effect on would need to be treated with antibiotics rare in the first year of life and such presenta-
conductive hearing. to prevent 1 case of mastoiditis. tions should be assessed for possible non-
accidental (inflicted) injury. Other suggestive
History History findings are bilateral ear injuries and asso-
Older children may present with aural full- Symptoms at presentation with mastoiditis ciated injuries, particularly retinal haemor-
ness or reduced hearing. OME is usually are very similar to AOM, with pain, irritabil- rhages and subdural haematoma.
asymptomatic in young children. ity and fever. Mean time from onset of illness
to signs of acute mastoiditis has been History and examination
Examination reported as just over 4 days. The most common mechanism is falls, fol-
The eardrum may appear dull and non- lowed by blows from an object and self-
erythematous and the effusion is most easily Examination inflicted penetrating injuries which may
recognised by the presence of bubbles or a Examination findings differentiating mas- result in perforation of the tympanic mem-
fluid level. If these are not present, pneumatic toiditis from AOM include protrusion/ brane. The most common objects inserted
otoscopy will demonstrate impaired mobility. displacement of the auricle, post-auricular are cotton buds. Dog bites of the ear also

334
14.1 THE EAR
14

ENT AND DENTAL

occur. Lacerations are the most common Dagan R, McCracken Jr GH. Flaws in design and conduct of
increased complications (see clinical trials in acute otitis media. Paediatr Infecti Dis J
injury, followed by bruising, abrasions and 2002;21(10):894902 [comment].
mastoiditis).
haematomas. Blood in the canal is a com- Del Mar CB, Glasziou PP. Should we now hold back from

mon finding in the penetrating injuries, Discharging otitis media chronic

initially prescribing antibiotics for acute otitis media?
J Paediatr Child Health 1999;35(1):910.
making assessment of the eardrum difficult suppurative otitis media: Dowell SF, Marcy SM, Phillips WR, et al. Principles of judicious
use of antimicrobial agents for pediatric upper respiratory
at the time of initial presentation. Burns
are rare and are likely to be associated with
Concern has arisen with the use of tract infections. Paediatrics 1998;101(1):1635.
Dowell SF, Marcy SM, Phillips WR, et al. Otitis media
potentially ototoxic antibiotic ear Principles of judicious use of antimicrobial agents.
more extensive burns. Barotrauma from Paediatrics 1998;101(1):16571.
drops in the presence of a
explosions and loud noises are uncommon Flynn CA, Griffin G, Tudiver F. Decongestants and
tympanic-membrane perforation, antihistamines for acute otitis media in children. Cochrane
compared to industrial injuries in adults. Database of Syst Rev 2002 2001;1 [update of Cochrane
despite routine use by ENT Database Syst Rev 2:CD001727, PMID: 11406002].
Assessment should include assessment of
specialists with few reported Froom J, Culpepper L, Green LA, et al. Antimicrobials for acute
the facial nerve and hearing. otitis media? A review from the international primary care
cases of ototoxicity. network. Br Med J 1997;315(7100):98102.
Investigation A series of nine cases of Garbutt J, Jeffe DB, Shackelford P. Diagnosis and treatment of
acute otitis media: An assessment. Paediatrics 2003;112
Acute investigations are rarely required. How- iatrogenic topical (1):1439.
ever, audiology and ENT referral is indicated vestibulotoxicity, all secondary to Glasziou PP, Hayem M, Del Mar CB, et al. Antibiotics for acute
otitis media in children. Cochrane Database Syste Rev 2000
in penetrating and barotrauma injuries. gentamicin-containing eardrops, 2000;4: [update of Cochrane Database Syst Rev 2:
has been reported. As a result, CD000219, PMID: 10796513].
Treatment agents such as ciprofloxacin are Holmes RE. Management of traumatic auricular injuries in
children. Paediatr Ann 1999;28(6):3915.
Minor lacerations may be managed with being increasingly used. Hughes E, Lee JH. Otitis externa. Paediatr Rev 2001;6:1917.
steristrips, glue or suturing under local Kozyrskyj AL, Hildes-Ripstein GE, Longstaffe SE, et al. Short

anaesthesia. Complex or larger lacerations  Otitis media with effusion course antibiotics for acute otitis media. Cochrane
Database Syst Rev 2000;2.
will often require a general anaesthetic Ventilation-tube (grommet) Little P, Gould C, Williamson I, et al. Pragmatic randomised
controlled trial of two prescribing strategies for
and surgical repair. Haematomas can lead insertion remains one of the most childhood acute otitis media. Br Med J 2001;322(7282):
to cartilage necrosis, infection and chondritis common surgical procedures 33642.
Little P, McCormick DP, Chonmaitree T, et al. Predictors of poor
or fibrous organisation. All of these have performed in children. outcome and benefits from antibiotics in children with
potential to cause significant deformity. It is effective at improving acute otitis media: Pragmatic randomised trial. Br Med J
2002;325(7354):22 [commentary: research directions for
Therefore removal of the haematoma is indi- hearing in the short term. There treatment for acute otitis media].
cated. Smaller haematomas can be aspirated has been conflicting evidence for Pond F, McCarty D, OLeary S. Randomized trial on the
treatment of oedematous acute otitis externa using ear
and larger or recurrent collections should be its long-term benefit on language wicks or ribbon gauze: Clinical outcome and cost. J Laryngol
evacuated. Appropriate contoured pressure and cognitive development. Otol 2002;116(6):4159.
Rovers MM, Straatman H, Ingels K, et al. The effect of
dressings are then required to avoid reaccu- ventilation tubes on language development in infants with
mulation. Penetrating injuries will often otitis media with effusion: A randomized trial. Paediatrics
2000;106(3):e42.
require ENT referral. Unless minor, burns will Rovers MM, Straatman H, Ingels K, et al. Randomised
require evaluation by a burns specialist. Further reading controlled trial of the effect of ventilation tubes (grommets)
on quality of life at age 12 years. Arch Dis Child 2001;84
Acuin J. Extracts from concise Clinical evidence: Chronic (1):459 [comment].
Complications suppurative otitis media. Br Med J 2002;325(7373):16. Ruohola A, Heikkinen T, Meurman O, et al. Antibiotic
The main concerns are cosmetic deformity Acuin J, Smith A, Mackenzie I. Interventions for chronic treatment of acute otorrhea through tympanostomy tube:
suppurative otitis media. Cochrane Database Syst Rev Randomized double-blind placebo-controlled study with
from haematomas, lacerations and burns, 2000;2. John Wiley & Sons, Chichester. daily follow-up. Paediatrics 2003;111(51):10617.
and hearing loss from penetrating injuries. Ah-Tye C, Paradise JL, Colborn DK. Otorrhea in young children Segal S, Eviatar E, Lapinsky J, et al. Inner ear damage in
after tympanostomy-tube placement for persistent middle- children due to noise exposure from toy cap pistols and
ear effusion: Prevalence, incidence, duration. Paediatrics firecrackers: A retrospective review of 53 cases. Noise &
2001;107(6):12518 [comment]. Health 2003;5(18):138.
Controversies and future Bitar CN, Kluka EA, Steele RW. Mastoiditis in children. Clin Spratley J, et al. Acute mastoiditis in children: Review of the
Paediatr 1996;35(8):3915. current status. Int J Pediatr Otorhinolaryngol 2000;56
directions Bolt P, Barnett P, Babl FE, Sharwood LN, et al. Topical (1):3340.
Steele BD, Brennan PO. A prospective survey of patients with
Acute otitis media: lignocaine for pain relief in acute otitis media: results of a
double-blind placebo-controlled randomized trial. Arch Dis presumed accidental ear injury presenting to a paediatric
Child 2008;93(1):404. accident and emergency department. Emerg Med J
Antibiotic usage for acute otitis Brook I. Treatment of otitis externa in children. Paediatr Drugs 2002;19(3):2268.
1999;1(4):2839. Straetemans M, Sanders EA, Veenhoven RH, et al.
media in Australia, the UK and Butler CC, Van Der Voort JH. Oral or topical nasal steroids for Pneumococcal vaccines for preventing otitis media.
USA remains high. Due to hearing loss associated with otitis media with effusion in Cochrane Database Syst Rev 2002;2.
children. Cochrane Database Syst Rev 2002 2000;4 Thompson PL, Gilbert RE, Long PF, et al. Effect of antibiotics
concerns over the emergence of [update of Cochrane Database of Syst Rev 4:CD001935, for otitis media on mastoiditis in children: a retrospective
antibiotic resistance, the PMID: 11034736]. cohort study using the Unitied Kingdom general practice
Byington CL. The diagnosis and management of otitis media research database. Pediatrics 2009;123(2):42430.
Netherlands has limited antibiotic with effusion. Paediatr Ann 1998;27(2):96100. Van Zuijlen DA, Schilder AG, Van Balen FA, Hoes AW, et al.
use (31%), with good results Cantor RM. Otitis externa and otitis media. A new look at old National differences in incidence of acute mastoiditis:
problems. Emerg Med Clin N Am 1995;13(2):44555. Relationship to prescribing patterns of antibiotics for acute
leading to a review of use in high- Cohen-Kerem R, Uri N, Rennert H, et al. Acute mastoiditis in otitis media? Paediatr Infect Dis J 2001;20(2):1404
prescribing countries. The children: Is surgical treatment necessary? J Laryngol Otol [comment].
1999;113(12):10815. Vassbotn FS, Klausen OG, Lind O, Moller P, et al. Acute mastoiditis
concern about lower rates of Combs JT. Diagnostic accuracy of otitis media. Paediatrics in a Norwegian population: A 20 year retrospective study.
antibiotic use is the potential for 2003;112(1):2056. Int J Paediatr Otorhinolaryngol 2002;62(3):23742.

335
 14.2 The nose
Stuart Lewena Gervase Chaney

eyes and/or nose. Unilateral nasal dis-

ESSENTIALS charge, particularly offensive, is suggestive
of a nasal foreign body.
Rhinitis and sinusitis
1 Acute rhinitis is common and largely due to viral infections. Examination
In rhinosinusitis, nasal discharge is the usual
2 Mucopurulent discharge does not mean bacterial infection. finding. This may be clear or mucopurulent
3 Antibiotics might be indicated in prolonged cases of nasal discharge (> 1014 and, as already discussed, is not indicative
days duration), indicating possible sinusitis. of cause. A red throat is a common associa-
tion. In allergic rhinitis, the nasal mucosa
4 Constant nasal discharge may be due to perennial allergic rhinitis. will be pale and swollen. Facial swelling,
Epistaxis such as orbital/periorbital and/or facial
tenderness may occur in sinusitis.
1 Epistaxis is common and largely responds to simple first aid compression of the
nose.
Investigations
2 Severe cases are uncommon, but are a medical emergency, requiring nasal packing Investigations are not indicated for the com-
and urgent ENT referral. mon cold. Sinusitis is also a clinical diagnosis.
Radiological investigations for acute sinusi-
3 Underlying bleeding tendency should be excluded in cases with a family history of tis are difficult to interpret, as similar find-
bleeding tendency or those with severe recurrent epistaxis.
ings may be found in the common cold or
Nasal trauma even asymptomatic children. Also, the
sinuses of young children are poorly devel-
1 Nasal trauma is common. oped, with frontal and sphenoid sinuses
2 Most injuries will require little or no intervention. However, a careful examination not appearing till 56 years of age. Radio-
to exclude septal haematoma is essential. logical investigations should be reserved
for those cases with suspected complications
3 Septal haematoma requires urgent ENT referral. of orbital/intracranial extension, or possibly
4 Acute assessment can be difficult and a review, once the swelling has settled, is in recurrent cases.
required to manage any deformity. If aspirated under general anaesthesia,
sinusitis fluid most commonly cultures Pneu-
mococcus, followed by Haemophilus influen-
zae, Moraxella catarrhalis and then viruses.
History Nasopharyngeal cultures are not predictive
Rhinitis and sinusitis The common cold usually commences with a and therefore not useful.
Introduction throat irritation and clear, thin nasal dis-
Viral upper respiratory tract infections caus- charge and progresses to a thick, mucopuru- Treatment
ing rhinitis and inflammation of the upper lent discharge after a few days. This does not Antibiotics are not indicated for the com-
respiratory tract are a frequent cause of indicate bacterial infection. Sneezing, nasal mon cold and have been shown neither to
febrile illness presentations amongst children obstruction, systemic features such as alter the course of the illness nor prevent
to emergency departments. Children will com- malaise and low-grade fever, and cough the development of complications. Perennial
monly experience three to eight of these are common. Generally the duration is allergic rhinitis responds best to intranasal
infections per year, even more if attending about 7 days. corticosteroids, but these are often not toler-
day care. After exclusion of more significant Persistence of mucopurulent nasal dis- ated well by children.
illness, education and symptomatic treatment charge without improvement for longer than Even in bacterial sinusitis, the majority of
is all that is required. Despite recommenda- 1014 days, or more severe acute symp- cases will resolve spontaneously. Nasal
tions against such practice, many children will toms, such as high fever, headache, facial decongestants may provide some short-term
have been prescribed antibiotics. Bacterial pain and/or swelling are more indicative relief. If antibiotics are used, amoxicillin is
sinusitis has been estimated as complicating of bacterial sinusitis. recommended as first-line treatment for
only 0.55% of upper respiratory tract infec- Persistent clear nasal discharge is more 1014 days duration. Amoxicillin clavula-
tions. Most of these will resolve without anti- likely due to perennial allergic rhinitis. This nate can be kept for those cases failing to
biotic treatment. may be associated with sneeze, and itchy respond in 4872 hours.

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ENT AND DENTAL

Complications Treatment may indicate a septal haematoma and
Bacterial complications of the common cold First aid treatment of epistaxis is firm com- urgent ENT consultation for evacuation is
do occur, such as otitis media, but cannot be pression of the alar nasae, applying pressure indicated.
prevented by antibiotic use. Asthma is fre- over Littles area. This will result in cessation
quently precipitated by upper respiratory of bleeding in the majority of cases. The Investigations
tract infections. use of anaesthetic/vasoconstrictor spray or Nasal X-rays offer no benefit. A young childs
Ethmoid sinusitis can spread to cause soaked pledgets may assist in haemostasis. nose is predominantly cartilage, and even
orbital cellulitis or intracranially, causing If these measures fail, nasal cautery can be when a fracture of the nasal bones is identi-
an abscess or cavernous sinus thrombosis. used for a well-identified bleeding site or fied, this information does not influence
an anterior nasal pack inserted. Purpose- management. Facial X-ray or computerised
made self-expanding nasal packs have made tomography (CT) should be limited to those
Prevention
this procedure much easier. These packs suspected of more serious facial bony injury.
Breast-feeding has a protective effect on the
should be lubricated with antibiotic oint-
number of upper respiratory tract infections Treatment
ment before insertion. Children with epi-
experienced by young children. Day care Most nasal injuries require no acute interven-
staxis that has required packing should be
attendance and environmental tobacco tion beyond analgesia. However, follow up
considered for investigation of underlying
exposure increase the number of episodes. may be required to determine the extent of
bleeding problems and have an ear, nose
and throat (ENT) referral. Posterior nasal deformity. The timing of this is recommended
packing or other more invasive procedures to be about 1 week post-injury, as the swelling
Epistaxis are rarely required in children, but may be has usually resolved by then, and, if required,
needed in severe epistaxis in the context of further treatment can be instituted.
Introduction
resuscitation and urgent ENT consultation.
Although epistaxis is common in childhood Complications
(affecting about 69%), it is usually mild Septal haematoma,  abscess formation, is
and usually does not require any medical
Complications
Rarely, recurrent epistaxis is sufficient to the main complication that causes long-term
intervention, let alone presentation to an damage and therefore concern. It may pres-
cause anaemia. Care should be taken when
emergency department (ED). ent later down the track and may have been
applying nasal cautery to avoid damage to
the underlying septal cartilage. Bilateral missed on earlier assessment. Haematomas
History cautery should be avoided for this reason. can be very painful and frequently cause
The frequency and severity of episodes nasal obstruction. On its own, a septal hae-
should be determined together with first matoma can cause nasal cartilage damage,
aid measures undertaken. A history of including aseptic necrosis. However, the
Nasal trauma development of an abscess is almost always
abnormal bleeding/bruising, infections or
trauma should be sought. Introduction associated with cartilage damage and
Nasal trauma is common in children. It can potentially severe nasal deformity. Staphylo-
occur at any age, but is most frequent in coccus aureus, Streptococcus pneumoniae
Examination early primary-age groups. Like most injuries, and Strep. pyogenes have all been cultured.
In the majority of cases, positive examina- there is a male predominance. Meningitis or cavernous sinus thrombosis
tion findings are limited to the nose. Bleed- may also complicate a septal abscess.
ing can be unilateral or bilateral. Crusting
History and examination
and vessels in Littles area are commonly
The most common mechanism of injury is
seen. The remainder of the examination Controversies
falls, usually at home, followed by sports
should exclude hypertension, abnormal
injuries. Child abuse (inflicted injury) should Epistaxis
bruising or petechiae and findings of lymph-
be considered, particularly in the younger The choice of which children to test and
adenopathy or hepatosplenomegaly.
(non-mobile) child. A careful history is there- extent of investigation for bleeding
fore required. disorders is still uncertain. For the
Investigations The types of injury are varied, from abra- severe and recurrent cases, thorough
Investigation for bleeding disorders is fre- sions and lacerations of the soft tissues to coagulation testing, including for von
quently performed. However, the vast major- fractures, dislocations and cartilage damage. Willebrand disease, is appropriate.
ity of these will be normal unless there are More than minor epistaxis is unusual. Due to
other indications of bleeding tendency or swelling it is often difficult to make a thor- Nasal trauma
family history. A more severe history of epi- ough assessment of the extent of injury in Open rhinoplasty for nasal deformities
staxis, with frequent bleeds of greater vol- the acute setting particularly for defor- may affect nasal growth in itself and
ume and duration, is more suggestive of a mity. Examination must include an examina- should be used as conservatively as
bleeding disorder such as von Willebrand tion of the nasal cavities. Deviation, swelling possible.
disease and warrants appropriate testing. and/or discolouration of the nasal septum

337
14.3 THE MOUTH AND THROAT

Royal Infirmary. Cautery or cream for epistaxis in children. tract infections, and bronchitis. JAMA 1998;279(11):
Further reading Emerg Med J 2001;18(3): 210.
Ginsburg CM. Nasal septal hematoma. Paediatr Rev 1998;
875877.
OBrien KL, Dowell SF, Schwartz B, et al. Acute sinusitis
Canty PA, Berkowitz RG. Hematoma and abscess of the nasal 19(4): 142143. Principles of judicious use of antimicrobial agents.
septum in children. Arch Otolaryngol Head Neck Surg Katsanis E, Luke KH, Hsu E, et al. Prevalence and significance Paediatrics 1998;101(1): 174177.
1996;122(12): 13731376. of mild bleeding disorders in children with recurrent Rosenstein N, Phillips WR, Gerber MA, et al. The common
Crockett DM, Mungo RP, Thompson RE. Maxillofacial trauma. epistaxis. J Paediatr 1988;113(11): 7376. coldPrinciples of judicious use of antimicrobial agents.
Paediatr Clin N Am 1989;36(6): 14711494. Makura ZG, Porter GC, McCormick MS. Paediatric epistaxis: Paediatrics 1998;101(1): 181184.
East CA, ODonaghue G. Acute nasal trauma in children. Alder Hey experience. J Laryngol Otol 2002;116(11): Ruddy J, Proops DW, Pearman K, et al. Management of
J Paediatr Surg 1987;22(4): 308310. 903906. epistaxis in children. Int J Paediatr Otorhinolaryngol
Ghosh A, Jackson R. Towards evidence based Nyquist A-C, Gonzales R, Steiner JF, et al. Antibiotic 1991;21(2): 139142.
emergency medicine: Best BETs from the Manchester prescribing for children with colds, upper respiratory

14.3 The mouth and throat

Stuart Lewena Gervase Chaney Richard P. Widmer

ESSENTIALS Stomatitis
Stomatitis Introduction
Acute herpetic gingivostomatitis is the
1 Acute herpetic gingivostomatitis is the most common cause of stomatitis in young most common cause of stomatitis in young
children. children (13 years). It is also the most
2 Early treatment with aciclovir is effective if commenced in the first 72 hours. common clinical presentation of primary
herpes simplex infection in young children.
3 Aphthous stomatitis or ulcers are more common in young adults, but do occur in It can also occur in older children and adults.
children. Untreated, the course of the illness is 1014
Pharyngitis/tonsillitis days. Aphthous ulcers usually occur as single
ulcers and are often recurrent.
1 Most sore throats in children are due to viral infections.
2 Bacterial tonsillitis is more likely in older children with isolated sore throat, high History
fever and tender cervical lymphadenopathy. Acute herpetic gingivostomatitis may pres-
3 Ten days of penicillin is the recommended course of treatment for bacterial ent suddenly or insidiously initially with
infection. fever and irritability. Mouth pain, often
severe, plus drooling and refusal to eat will
Peritonsillar abscess usually follow. Dehydration can occur if the
1 Peritonsillar abscess is the most common abscess of the head and neck. child refuses to drink. Gingival bleeding
can occur
2 Diagnosis is not always straightforward. Aphthous ulcers present as recurrent
3 Management is analgesia, antibiotics, fluids and draining the abscess. painful lesions of the oral mucosa, usually
single and less than 1 cm diameter.
Post-tonsillectomy haemorrhage
1 Tonsillectomy remains a relatively common procedure. Examination
2 Post-tonsillectomy bleeding occurs in about 1.5% and although most may be Early herpetic lesions are vesicles, but usu-
managed conservatively, for some, transfusion and/or surgery are necessary. ally not seen, due to early rupture. Multiple
ulcers up to 1 cm then occur on any part of
Oral/dental trauma the oral mucosa and initially are covered
1 Oral/dental trauma is common in children. with a yellow-grey membrane. Associated
gingivitis is usual.
2 A careful examination of the orofacial structures is required.
3 Dental consultation is indicated for all but the minor cases.
Investigations
Oral/dental infection Viral swabs and immunofluorescence or viral
culture for herpes simplex can confirm the
1 Dental infections are the most common cause of facial cellulitis.
diagnosis if required. However, if this is not
2 There are usually underlying dental caries and tooth extraction is often required. readily available, clinical diagnosis is reason-
ably accurate.

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ENT AND DENTAL

For recurrent aphthous ulcers, checking a to the value of throat swabs and they should
neutrophil count to exclude cyclical neutro- Pharyngitis/tonsillitis probably only be performed in cases with a
penia is appropriate. Introduction high clinical index of bacterial aetiology.
Sore throat is an extremely common presen- (Child older than 4 years, significant fever,
tation and is predominantly viral in cause. pharyngitis in the absence of other upper
Differential diagnosis Group A streptococcal infection accounts respiratory tract infection signs, tender ton-
for 1020% of cases, and is even less fre- sillar lymph nodes.)
Initially presentation is non-specific and Blood tests are usually done for investiga-
quent in young children (infants < 1 year
can be confused with a general viral infec- tion of infectious mononucleosis, although
< 5%). The concern over potential compli-
tion. If the tonsils are involved early, acute in children, the monospot/monotest has a
cations from streptococcal infection, such
tonsillitis or herpangina may be sus- high false-negative rate and serology is
as rheumatic fever and glomerulonephritis,
pected. Some cases are misdiagnosed as more reliable.
has led to many children being prescribed
oral candidiasis.
unnecessary antibiotics. Most people do
Aphthous ulcers are easily distinguished,
not seek medical care for sore throats and Treatment
as these are usually single. Recurrent
the problem resolves spontaneously. For the majority of sore throats due to viral
aphthous ulcers can be seen in cyclical or
pharyngitis/tonsillitis, no antibiotics are
congenital neutropenia and PFAPA syn-
necessary. Analgesia in the form of paracet-
drome (fever, malaise, aphthous stomati- History
amol or ibuprofen will provide symptomatic
tis, tonsillitis, pharyngitis and cervical Older children will present with a complaint
relief. For streptococcal pharyngitis, phenoxy-
adenopathy). of sore throat, while younger children may
methylpenicillin (250 mg or 500 mg) twice
be non-specifically unwell. A reluctance to
daily for 10 days is the recommended treat-
Treatment take food or drinks may indicate a sore
ment, with a cure rate of approximately 90%.
Traditional treatment has been symptom- throat. Associated symptoms include fever,
Antibiotics should be routine in groups at
atic, in the form of analgesia and hydration. headache, vomiting and abdominal pain,
high risk of rheumatic fever, those with exist-
Analgesia usually requires a combination of but are not predictive whether bacterial
ing rheumatic heart disease and those with
topical anaesthetic agents and oral analge- or viral.
scarlet fever.
sics, such as paracetamol or ibuprofen. Topi-
cal lidocaine gel can be very effective. Examination
Complications
Rehydration may require the use of nasogas- Ulcerative pharyngitis (herpangina) is a
Complications from sore throats are uncom-
tric or intravenous fluids. helpful finding indicating viral infection,
mon. Suppurative complications of strepto-
Recent studies, including a placebo- such as coxsackie. Otherwise, it is difficult
coccal infection include peritonsillar
controlled study, have demonstrated the to differentiate between bacterial and viral
abscess, sinusitis and otitis media. The prin-
efficacy of oral aciclovir, if commenced in causes. Features that suggest bacterial
cipal concerns, however, are with the non-
the first 72 hours of the illness. They have infection are tender cervical lymphadenopa-
suppurative complications: rheumatic fever
shown a significant reduction in duration thy and absence of other symptoms such as
and glomerulonephritis. Acute tonsillitis
of fever, feeding and drinking difficulties coryza, cough, conjunctivitis and diarrhoea.
can cause airway obstruction, particularly
and viral shedding. A dose of 15 mg kg1 Tonsillar exudate or pus is not an accurate
with pre-existing tonsillar hypertrophy and
per dose (up to 200 mg) five times a day predictor of bacterial infection and is fre-
may even warrant admission for monitoring.
for 57 days is recommended. quently seen in viral causes such as infec-
Aphthous ulcers with an adherent/dental tious mononucleosis, EpsteinBarr virus
base can be treated with topical corticoster- (EBV) and adenovirus. EBV infection may
oids (e.g. triamcinolone). be suggested by the presence of more wide- Peritonsillar abcess
spread lymphadenopathy, particularly if Introduction
Complications there is associated splenomegaly. Peritonsillar abscess is the most common
Acute dehydration has been mentioned. Scarlet fever is suggested by a wide- deep space head and neck infection in chil-
Secondary bacterial infection is uncommon. spread, fine, maculopapular rash with a dren. It is likely to be an extension of acute
Primary herpetic infection may progress to sandpaper-like feel, with associated pharyn- tonsillitis. However, it has been suggested
generalised vesicular eruption. Also, auto- gitis and possibly a strawberry-tongue. that some cases may arise from obstruction
inoculation can occur, particularly to the Although the scarlatiniform rash is highly and infection of Webers glands (mucous
eye. specific for streptococcal infection, it only glands located in the superior tonsillar pole).
Recurrent labial herpes is common and occurs in a minority of cases. It can occur at any age.
more of an inconvenience. It occurs follow-
ing exposure to sunlight, stress, trauma Investigations History and examination
or cold. Topical aciclovir may be of use, Throat swab and culture may be used to Presentation is usually with sore throat
when applied with the first evidence of assist differentiation between viral and bac- or neck pain, which is often severe. Odyno-
symptoms. terial aetiology. Opinion varies with regard phagia, dysphagia, and fever are also

339
14.3 THE MOUTH AND THROAT

common. A muffled (or hot potato) voice about 7 days, with a range up to 15 days. relatively minor. Occasionally, higher impact
and trismus occurs in about one-third of Rarely, haemorrhage is catastrophic and life trauma including motor vehicle accidents
cases. Only a minority will have a prior his- threatening. will produce severe injury requiring max-
tory of tonsillitis (2030%). illofacial reconstructive surgery and early
Examination findings include cervical History problems of haemostasis and airway
lymphadenopathy, unilateral tonsillar ery- Bleeding is usually obvious, although occa- compromise.
thema, bulging of the superior aspect of sionally will be swallowed and not immedi-
the tonsil and uvular deviation. The differen- ately apparent. A history of bleeding or History
tial is peritonsillar cellulitis, where there is bruising tendency should be sought, Make specific enquiry with regard to the
no abscess. Clinical differentiation between although ideally will have been identified time and mechanism of trauma and the
the two is difficult. prior to surgery. nature of the dentition prior to injury. This
includes the number of deciduous teeth that
Investigations Examination were present and the presence of any sec-
The definitive investigation is needle aspira- Initial assessment should focus on signs of ondary dentition. Determine whether any
tion, but not all children will be co-operative. shock or haemodynamic compromise. Exam- teeth have been avulsed and their current
Computerised tomography scan or ultra- ination of the tonsillar fauces for evidence of location and method of storage. Consider
sound may assist diagnosis. Microbiological active bleeding is then important. the possibility of aspiration if a tooth is
identification of abscess contents probably missing.
does not alter management. If performed, Investigations
the predominant organism found is Strepto- Full blood examination to monitor for a drop Examination
coccus pyogenes. in haemoglobin and taking blood for cross The physical examination should review all
match is recommended in any significant orofacial structures with careful scrutiny,
Treatment post tonsillectomy bleed. Coagulation pro- digital palpation and observation of normal
Initial treatment is rehydration, analgesia file testing rarely demonstrates abnormality, function. Externally and internally the orofa-
and antibiotics (penicillin). Acute drainage but is warranted in major haemorrhage. cial region should exhibit consistent symme-
is generally recommended, with either try and any departure from this, be it an area
intraoral drainage, abscess tonsillectomy or Treatment of altered facial soft tissue architecture asso-
needle aspiration. Needle aspiration alone Immediate, preferably large-bore, intrave- ciated with a swelling or altered bony archi-
would appear to be effective in a majority nous access is indicated with cross-matching tecture associated with a fracture, will be
of cases (>90%). Sedation or general anaes- of blood and commencement of fluid resusci- important. For example chinpoint trauma
thesia may be required. tation as required. Early involvement of an is a common injury associated with mandib-
Abscess tonsillectomy should be reserved ear, nose and throat surgeon for children with ular condylar fracture. Assessment of man-
for recurrent tonsillitis abscess, but many of active bleeding is indicated. Transfusions dibular opening and malocclusion is vital.
these can still be managed with aspiration have been reported as being required in In cases where the child has fallen with
and delayed tonsillectomy. 1012% of secondary haemorrhage. How- an object in their mouth, careful assess-
ever, a more conservative approach is proba- ment for palatal or pharyngeal penetrating
Complications bly possible with the use of other intravenous trauma is required.
Dehydration is common and will require fluids and close monitoring. Surgical inter- The key points in recognition of a facial
medical intervention. Uncommon, but dan- vention under general anaesthesia is less fre- fracture are pain, facial swelling, stepping
gerous, complications include infection quently required according to a retrospective (of the bony border), limited jaw opening,
spread, such as to the parapharyngeal Australian study (4.3%). palatal or sublingual haematoma, malocclu-
space, airway obstruction and aspiration of sion and paraesthesia. The need for tetanus
pus causing pneumonia. Complications prophylaxis and antibiotics should be con-
Post-tonsillectomy bleeding is potentially sidered. Usually, antibiotics (penicillin and
life threatening, largely from hypovolaemic metronidazole) should be given for a com-
pound fracture in the mouth.
Post-tonsillectomy shock. Massive bleeding may also cause air-
A common error when assessing a childs
haemorrhage way obstruction.
occlusion is to fail to recognise that an ante-
Introduction rior open bite (associated with a prior digit
Tonsillectomy remains a very commonly or object sucking habits) is a normal anterior
Oral/dental trauma
performed procedure. The most common relationship for that child and not necessar-
complication of tonsillectomy is bleeding. Introduction ily evidence of a fracture in the maxilla/
Secondary (delayed) haemorrhage, which The most common reasons for infants and mandible. Check the posterior teeth for
occurs after the first 24 hours following children to present to the emergency depart- appropriate occlusion in this situation. The
surgery, complicates about 1.5% of tonsil- ment (ED) with oral/dental trauma are falls older child will usually be able to tell you
lectomies. The mean time of presentation is and sporting injuries. Most of these are if the teeth feel right when they bite.

340
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ENT AND DENTAL

Investigations re-implanted. Reasons include the potential that displaced soft tissues are adequately
The most relevant extraoral radiograph is an damage to the developing permanent teeth, repositioned and sutured. Alveolar bone
orthopantomogram (OPG). This radiograph difficulties in securing the tooth/teeth in should not be left exposed to granulate over.
will give an excellent view not only of the place and the level of co-operation that is
body of the mandible, the dentoalveolar required. Prevention
area, but also of the temporomandibular Ideally, avulsed permanent teeth should Advice on prevention is never misplaced and
joints (TMJ). However, the childs co- be repositioned/reimplanted in the tooth recommendation of well-made and fitted
operation is required. If a TMJ view is specif- socket as soon as possible. It is crucial not mouth guards is encouraged.
ically required it will be important to notify to handle the root of the tooth. Manipulate
the radiographer. using the crown only. This avoids damage
to the periodontal ligament cells, which Oral/dental infection
Treatment line the root surface and are critical in re-
establishing the tooth back in the mouth Introduction
Management will obviously depend upon Dental infections are the most frequent
the exact diagnosis, which may include the as a functioning unit. Once re-implanted,
avulsed teeth need to be splinted in place. cause of acute facial cellulitis, accounting
following five factors: for about 50% of all cases. Therefore, for
Temporary splints can include moulded
aluminium foil around the teeth or a fishing children presenting with a facial swelling
Luxation line and super glue. It will be crucial to stress associated with fever and general malaise,
This is defined as a slight movement of the dental follow up to try and avoid the a dental cause must be high on the list of
teeth as a result of trauma, with or without sequelae that lead to tooth loss. differentials.
associated gingival bleeding. These injuries
can occur in either the primary or permanent Root factures History
teeth and can be in any direction, i.e. inter- The root of the tooth, which is buried in Facial cellulitis will present as above, with
nal, external or lateral. Active intervention the alveolar bone, can also be fractured, facial redness and tenderness. The upper
may or may not be needed, depending on often with minimal damage to the crown face is affected more frequently, particularly
the extent of movement and interference of the teeth. This may present at review, if in younger children. The lower face is
with the occlusion or risk to the develop- not initially obvious. A particularly mobile affected relatively more often in older chil-
ment of the underlying secondary dentition. or loose tooth/teeth, that is/are slightly dren, probably related to caries patterns.
The appropriate treatment in luxation extruded are classic signs of associated root
may be conservative in minor cases or fractures. Examination
involve extraction of the damaged primary A thorough examination, particularly intra-
tooth/teeth or repositioning of displaced Hard tissue injuries orally is required and early dental involve-
teeth and placement of a dental splint with This involves a chip off the tooth, whether it ment is very helpful in determining a dental
associated suturing of damaged soft tissues. is just enamel or involves the deeper layers source.
such as the dentine. It may involve all
Avulsion three layers of the tooth and expose the Investigations
This is defined as the complete loss of a dental pulp. If a tooth is exposed, then it Appropriate radiographs are frequently indi-
tooth, primary or permanent. In all situa- is important that the exposure is treated cated to determine a dental aetiology. These
tions, if there is any doubt about the nature immediately so as to preserve the vitality may be extraoral, e.g. an OPG, and/or
of the tooth (that is, whether it is primary of the dental pulp and thus enable the root intraoral.
or not) and given that permanent teeth to grow to its full length and thickness. This
are always re-implanted, it is prudent, as is very important for the tooths long-term Treatment
first-line advice, to suggest that all avulsed maintenance as a useful member of the The usual treatment involves the use of anti-
teeth are placed in milk or an appropriate dentition. biotics (usually penicillin will be adequate)
tissue-culture medium, if available. The and repair or removal of the offending
appropriate tissue-culture medium is Hanks Soft-tissue injuries tooth/teeth. Metronidazole may be added
balanced salt solution. This can be kept fro- The soft tissues of the mouth involve the in cases of significant infection requiring
zen in the ED in a small vial. It will provide gingival tissues, mucosa and muscle. The hospitalisation.
up to 12 hours working time before replant- soft tissue lacerations, especially of the gin-
ing the avulsed tooth is necessary. It is gival tissues, need to be carefully assessed Prevention
important not to store the tooth in water. for degloving and may need to be reposi- Lifting the upper lip in all children seen is a
If milk or tissue-culture medium is not avail- tioned and sutured. Where teeth are signifi- useful way of detecting early dental decay,
able, it is crucial to keep the tooth moist by cantly displaced there will be associated which presents with whitening of the teeth
wrapping it in clingfilm or a gauze that is displacement and laceration of soft tissue at the gum margins and later staining. A sig-
kept moist with saline or the patients saliva. and it is very important for the long-term nificant cause of tooth decay in infants and
As a general rule, the primary teeth are not periodontal health of the traumatised teeth young children is inappropriate feeding

341
14.3 THE MOUTH AND THROAT

habits based on the use of a night-time Dawes LC, Bova R, Carter P. Retropharyngeal abscess in
Tonsillectomy children. Aust N Z J Surg 2002;72(6):41720.
feeding bottle containing sweetened liquids, Del Mar CB, Glasziou PP, Spinks AB. Antibiotics for
which end up causing extensive dental Antibiotics post-tonsillectomy do lessen sore throat. Cochrane Database Syst Rev 2000;4 [update
of Cochrane Database Syst Rev 2000;2:CD000023; PMID
damage. Advice should be given to parents morbidity, in particular pain, analgesic 10796471].
to go from breast to cup with their babies use and delay in resumption of oral diet. Delaney JE, Keels MA. Paediatric oral pathology Soft tissue
and periodontal conditions. Paediatr Clin N Am 2000;
or to wean from the bottle by 12 months. Amoxicillin and amoxicillin 47(5):112547.
Also, infants should not be put to bed with clavulanate have been shown to be Edmond KM, Grimwood K, Carlin JB, et al. Streptococcal
pharyngitis in a paediatric emergency department. Med J
a bottle. effective. The studies have not had the Aust 1996;165(8):4203.
numbers to demonstrate whether this is Febres C, Echeverri EA, Keene HJ. Parental awareness, habits,
and social factors and their relationship to baby bottle
also true for post-tonsillectomy tooth decay. Paediatr Dentistry 1997;19(1):227.
haemorrhage. Gianoli GJ, Espinola TE, Guarisco JL, Miller RH.
Retropharyngeal space infection: Changing trends.
Other dental issues Oral/dental trauma Otolaryngol Head Neck Surg 1991;105(1):92100.
Goldenberg D, Golz A, Joachims HZ. Retropharyngeal
Spontaneous oral haemorrhage The management of avulsed
abscess: A clinical review. J Laryngol Otol 1997;111
(6):54650.
Oral bleeding is still a very important sign for permanent teeth centres on the Graham DB, Webb MD, Seale NS. Paediatric emergency room
the diagnosis of underlying generalised visits for nontraumatic dental disease. Paediatr Dentistry
prescription of routine antibiotics post- 2000;22(2):13440.
bleeding disorders. It is important to deter- re-implantation in an effort to limit the Herzon FS, Nicklaus P. Paediatric peritonsillar abscess:
mine the actual site of the bleeding and con- Management guidelines. Curr Probl Paediatr 1996;26
unwanted side effects associated with (8):2708.
sequently a through oral examination will re-implantation, such as an ankylosis of Irani DB, Berkowitz RG. Management of secondary
be required. Try to rinse the childs mouth hemorrhage following paediatric adenotonsillectomy.
the teeth and inflammatory root Int J Paediatr Otorhinolaryngol 1997;40(23):11524.
with water or saline and use gauze to resumption. This prescription of Myssiorek D, Alvi A. Post-tonsillectomy hemorrhage:
remove any blood clots and identify the An assessment of risk factors. Int J Paediatr
antibiotics is not universally accepted. Otorhinolaryngol 1996;37(1):3543.
source of the oral bleeding. In many Nussinovitch M, Finkelstein Y, Amir J, Varsano I. Group A beta-
instances this may be a tooth socket asso- The immediate disimpaction of hemolytic streptococcal pharyngitis in preschool children
aged 3 months to 5 years. Clin Paediatr 1999;38
ciated with a recent extraction. However, the traumatically intruded permanent (6):35760.
life-threatening disorders such as haeman- tooth/teeth using orthodontic Ripa LW. Nursing caries: A comprehensive review. Paediatr
Dentistry 1988;10(4):26882.
giomas and arteriovenous malformations appliances is proposed as the most Schraff S, McGinn JD, Derkay CS. Peritonsillar abscess in
may present in the same fashion. appropriate care, especially when trying children: A 10-year review of diagnosis and management.
Int J Paediatr Otorhinolaryngol 2001;57(3):2138.
If a tooth-extraction socket is identified to limit the unwanted sequelae of poor Schwartz B, Marcy SM, Phillips WR, et al. Pharyngitis:
as the cause of bleeding, local measures to alveolar bone height and gingival Principles of judicious use of antimicrobial agents.
Paediatrics 1998;101(1):1714.
control the bleeding will usually be suffi- attachment. However, there is a school Schwartz SS, Rosivack RG, Michelotti P. A childs sleeping habit
cient. This involves applying digital pres- of thought that proposes to partially as a cause of nursing caries. J Dentistry Child 1993;60
(1):225.
sure to the bleeding socket or having disimpact surgically, and then complete Sharma HS, Kurl DN, Hamzah M. Retropharyngeal abscess:
the child bite down on a gauze pad for the job of realignment orthodontically. Recent trends. Auris, Nasus. Larynx 1998;25(4):4036.
Spruance SL, Stewart JC, Rowe NH, et al. Acyclovir cream for
15 minutes. Bleeding disorders such as Oral/dental infection treatment of herpes simplex labialis: Results of two
von Willebrands disease and haemophilia randomized, double-blind, vehicle-controlled, multicenter
clinical trials. Antimicrob Agents Chemother 2002;46(7):
should be considered in cases that con- Antibiotic choice is not universally 223843.
tinue to bleed. agreed in either delivery (oral vs. Tsevat J, Kotagal UR. Management of sore throats in children:
A cost-effectiveness analysis. Arch Paediatr Adolesc Med
intravenous) or type (penicillin vs. 1999;153(7):6818 [comment].
cephalosporins). Unkel JH, Mckibben DH, Fenton SJ, et al. Comparison of
odontogenic and nonodontogenic facial cellulitis in a
paediatric hospital population. Paediatr Dentistry 1997;19
Controversies (8):4769.
Pharyngitis/tonsillitis van Everdingen T, Eijkman MA, Hoogstraten J. Parents
Further reading and nursing-bottle caries. J Dentistry Child 1996;63(4):
2714.
Although antibiotics have a protective Amir J, Harel L, Smetana Z, et al. Treatment of herpes simplex Wilson S, Smith GA, Preisch J, Casamassimo PS. Nontraumatic
gingivostomatitis with aciclovir in children: A randomised
effect in the prevention of rheumatic double blind placebo controlled study. Br Med J 1997;314
dental emergencies in a paediatric emergency department.
Clin Paediatr 1997;36(6):3337.
fever, the evidence does not support a (7097):18003 [comment]. Windfuhr JP, Chen YS. Hemorrhage following
Cmejrek RC, Coticchia JM, Arnold JE. Presentation, diagnosis,
similar effect on prevention of and management of deep-neck abscesses in infants. Arch
paediatric tonsillectomy before puberty. Int J Paediatr
Otorhinolaryngol 2001;58(3):197204.
glomerulonephritis. Otolaryngol Head Neck Surg 2002;128(12):13614.

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 14

ENT AND DENTAL

14.4 Retropharyngeal abscess
James Tilleard

ESSENTIALS Treatment
1 The potential for airway compromise and other complications mandates early The initial priority in treatment is to ensure
diagnosis and treatment. an adequate and stable airway. The poten-
tial for upper airway obstruction and a diffi-
2 Retropharyngeal abscess should be suspected in children with sore throat, neck cult intubation should be planned for.
pain, fever, dysphagia, dysphonia, neck swelling or torticollis. Consideration should be given to performing
3 The differential diagnosis includes meningitis and epiglottitis. intubation in the operating theatre with
both anaesthetic and surgical assistance
4 Antibiotics must be initiated early. available.
5 Patients should be managed in an institution with appropriate paediatric medical, Once resuscitation is complete, antibiotics
surgical, anaesthetic and intensive-care facilities. must be commenced as soon as possible.
Empiric treatment must cover the most likely
causative organisms and account for possible
resistance. Antibiotic combinations include a
Introduction Examination third-generation cephalosporin, flucloxacillin
Retropharyngeal abscess is an uncommon Examination may reveal fever, a neck mass, and metronidazole. Clindamycin, penicillin,
condition with the potential for significant lymphadenopathy, limitation of neck exten- vancomycin and ticarcillin clavulanate are
complications including airway compromise. sion, torticollis, dysphonia or bulging of the also considered in some references. Atten-
Retropharyngeal abscess formation occurs pharyngeal wall. There may be evidence of tion must also given to analgesic needs, fluid
in the space between the fascia covering complications, including respiratory compro- and metabolic resuscitation. Complications
the posterior pharyngeal wall and the pre- mise, dehydration or spread of infection. should be excluded and these include airway
vertebral fascia. The related parapharyngeal compromise, septic shock, aspiration, med-
abscess occurs in the deep neck space lateral iastinitis, septic emboli, osteomyelitis and
to the pharynx containing the carotid involvement of vessels or cranial nerves.
sheath and cranial nerves. Investigations
Retropharyngeal abscess in the paediatric Computerised tomography (CT) scanning is
population is usually secondary to upper the investigation of choice. It is essential Controversies and future
respiratory tract infection and suppuration to ensure the patient is stable and safe for directions
in retropharyngeal lymph nodes. Other this procedure, with special attention to
causes include infection following trauma, the airway. The CT result will assist with Current debate centres on the need for
foreign body ingestion or dental problems. diagnosis and the differentiation of cellulitis surgery or initial medical treatment with
While abscess formation may occur at any from abscess formation which is essential in antibiotics and close observation.
age, the peak incidence is in children aged determining the need for surgery.
less than 6 years. Lateral soft tissue neck X-ray may reveal
The most common causative organisms prevertebral swelling, gas, foreign body, air
are group A b-haemolytic streptococci and fluid level or bony erosion but is not sensi-
Staphylococcus aureus. Anaerobes, espe- tive or specific. Both magnetic resonance Further reading
cially Bacteroides, Gram-negative and mixed Brook I. Microbiology and management of peritonsillar,
imaging and ultrasound have also been retropharyngeal and parapharyngeal abscess. J Oral
infections also occur. used. A chest X-ray may reveal evidence of Maxillofacial Surg 2004;62(12):154550.
Cmejrek RC, Coticchia JM, Arnold JE. Presentation, diagnosis
aspiration or mediastinitis. and management of deep-neck abscesses in infants. Arch
Laboratory studies typically reveal an Otolaryngol-Head Neck Surg 2002;128(12):13614.
History elevation of white blood cell count and Craig FW, Schunk JE. Retropharyngeal abscess in children:
Clinical presentation, utility of imaging and current
Children with retropharyngeal abscess may inflammatory markers. Microbiology speci- management. Paediatrics 2003;111(6):13948.
Dawes LC, Bova R, Carter P. Retropharyngeal abscess in
present with a history of recent upper respi- mens, including blood cultures and culture children. ANZ J Surg 2002;72(6):417.
ratory tract infection, fever, neck pain, sore of pus obtained at the time of surgery, are Shefelbine SE, Mancuso AA, Gajewski BJ, et al. Paediatric
retropharyngeal lymphadenitis: differentiation from
throat, dysphagia, poor oral intake, trauma important in guiding subsequent antibiotic retropharyngeal abscess and treatment implications.
or other infective focus of the head and neck. therapy. Otolaryngol Head Neck Surg 2007;136(2):1828.

343
 14.5 Foreign bodies and caustic ingestion
Stuart Lewena Gervase Chaney

Positive pressure ventilation using bag

ESSENTIALS valvemask is effective, particularly for
larger objects that occlude the nasal
Nasal foreign bodies cavity. An alternative to a bagvalvemask
1 Foreign body removal from the nasal passage requires good preparation and is for the parent to blow into the childs
lighting. mouth with their own. Air is bagged or
blown rapidly through the mouth while
2 The appropriate method of removal depends upon the type of object and its the unaffected nostril is occluded with a
location.
finger. The high pressure generated in the
Aural foreign bodies upper airway expels the foreign body from
the nose. There is an unreported
1 Foreign bodies in the medial two-thirds of the ear canal are difficult to remove theoretical risk of barotrauma.
and frequently require sedation or general anaesthesia.
Suction catheters can be used. Generally
Caustic ingestion a soft, pliable end is required, and this can
be made up using soft tubing, although
1 Caustic ingestion is usually accidental in children. commercially available kits are now
2 The children are often asymptomatic and are at minimal risk of complications available.
such as stricture. A balloon catheter can be used if passed
beyond the foreign body, inflated and
3 Airway management is the priority in resuscitation. drawn back.
4 There is no proven treatment to prevent stricture formation. Application of cyanoacrylate glue via a
wooden swab stick or similar is described,
but requires patience and a very steady
Investigations hand. It may adhere to the mucosa.
Nasal foreign bodies Investigations are not usually required.
General anaesthesia and removal by ear,
Introduction Imaging is usually indicated for other possi-
nose and throat (ENT) staff is required for
Nasal foreign bodies are a relatively com- ble causes such as tumour.
the failed removal and the unco-operative
mon presentation to a paediatric emergency
child.
department (ED), although there is little
Treatment
published literature on the subject. They
Except for button batteries (see also Complications
are not life threatening, but can cause signif-
Chapter 7.6), removal of the foreign body is Possible complications include epistaxis, local
icant morbidity.
not urgent. Most foreign bodies can be suc- and more widespread infection. Uncommonly,
cessfully removed in the ED with adequate septal perforation has occurred secondary to
preparation and planning. Prior to the a button battery. Mucosal injury may occur
History
attempted removal, use of a topical anaes- within hours.
Nasal foreign bodies predominantly present
thetic nasal spray, such as lidocaine phen-
in children from 2 to 5 years of age. Children
ylephrine, is recommended. Sedation and/or
with nasal foreign bodies are usually
appropriate restraint of the child may be
brought in within 24 hours of insertion/
required. A good light source and an assistant Aural foreign bodies
impaction. Delayed presentation is mostly
to hold the childs head still are essential. Introduction
with unilateral purulent nasal discharge,
The appropriate procedure for removal Aural foreign bodies in children are also a
which is usually offensive. Other possible
will depend upon the foreign body size, relatively common presentation to EDs.
symptoms include foul breath, nasal pain,
shape, consistency and location in the nares. Again morbidity can be significant.
sneezing, snoring and mouth breathing.
These include:

Right-angle hooked probes can be History

Examination passed alongside and past larger objects, A greater proportion of children present
The type of foreign body is extremely vari- rotated and then drawn back gradually, beyond 24 hours from insertion compared
able, although it is most commonly a plastic removing the object. with nasal foreign bodies and in many cases
toy or bead. The foreign body is usually read- Forceps (alligator or bayonet) are useful for the time frame may not be determined. It
ily visible. smaller objects near the anterior nares. may be a result of the event being witnessed,

344
14.5 FOREIGN BODIES AND CAUSTIC INGESTION
14

ENT AND DENTAL

reported by the child due to irritation or pain, inflammation. Aural antibiotic drops with ste- least half of these, that will be the limit of
or as an incidental finding. The object may roid are often recommended, particularly if injury. Occasionally, significant burns occur
have been placed due to existing ear canal there is evidence of trauma or inflammation. beyond an unaffected oral cavity.
irritation due to wax impaction or otitis. Live
insects are a common finding, and may result Complications Investigations
in marked discomfort. Trauma to the ear canal is common as a Chest X-ray has demonstrated pulmonary irri-
result of the foreign body and/or its tation in up to 13% in some series. However,
Examination removal. More seriously, trauma to the tym- there is evidence that radiological investiga-
Visualisation of the foreign body is usually panic membrane or ossicles can occur. Otitis tions have little impact upon management
by otoscopy. The type and location of the externa can occur as a result of trauma. and therefore cannot be recommended rou-
object has a significant role in the difficulty tinely, except to localise a button battery.
of removal. Oesophagoscopy may be performed
Caustic ingestion according to symptoms, particularly ongoing
Investigation drooling and dysphagia, vomiting/haema-
Investigations are not indicated. Introduction temesis and stridor. The type of caustic sub-
Caustic ingestion in children is an uncom- stance, its form and pH should be considered.
Treatment mon presentation and significant injury with
Removal of foreign bodies from the lateral stricture formation is rare because the vol- Treatment
one-third of the external auditory canal is ume ingested is usually small. The ingested Resuscitation, involving airway and breath-
much easier and more successful than from agents are variable and include alkalis (more ing management, is the priority in severely
the medial two-thirds. The latter is the osse- common) and acids in liquid, granule or solid affected cases. Upper airway obstruction
ous portion and is narrower, more vascular form. The likelihood of significant injury is may warrant intubation. However, there is
and very tender. Therefore removal is more dependent upon how alkali or acidic the a case report of successful use of nebulised
likely to require sedation or general anaes- agent is (i.e. its pH). Strong liquid alkali is adrenaline (epinephrine) for acute marked
thesia and ENT expertise. particularly dangerous. stridor following hypochlorite ingestion.
Dilution using water or milk may be useful
Appropriate, restraint of the child and a History for solid or granule ingestions. Children who
good light source are always necessary;
In children, in contrast to adults, ingestion is remain asymptomatic may be discharged.
Sedation may have a role in some
largely accidental and, as a result, significant Button batteries should be removed endo-
children;
injury far less common. Most cases occur in scopically, followed by a period of observation.
Choice of method of removal will depend
children less than 3 years of age and, like acci- There is no proven effective treatment for
upon the object and its location;
dents in general, occur in boys more fre- oesophageal burns, especially in the preven-
Irrigation is the simplest method, but is
quently than girls. Most ingestions occur at tion of stricture development. Antibiotics
contraindicated if there is a tympanic
the home of the child. The most common and corticosteroids have been commonly
perforation, or for soft objects or
alkali agent ingested is dishwasher detergent used, but have not been shown to be of
vegetable matter that may swell;
and commonest acidic agent is acetic acid. any value.
Suction via a small catheter;
Button batteries have a significant corrosive
Forceps alligator, Hartmann;
and burn risk if lodged or impacted in the Complications
A right-angled hook passed beyond the
oesophagus (see Chapter 7.6). Stricture formation is rare and, in a combina-
object and pulled out.
Many children are asymptomatic. If they tion of nine series giving a total of 1961 chil-
Button batteries are again a risk (see also do occur, symptoms include vomiting, drool- dren who had caustic ingestions, occurred in
Chapter 7.6), causing necrosis of the ear ing, pain on swallowing and dysphagia, only 3.2%. This was despite a mean inci-
canal or tympanic membrane and should chest pain and refusal to drink. Less com- dence of oesophageal burns of 21.4% and
be removed as soon as possible. A live insect monly, stridor may occur secondary to upper of deep burns of 4.7%. Upper airway
should be killed or immobilised with micro- airway burns and obstruction. obstruction has been mentioned and is
scopic immersion oil, mineral oil or local In one series, the most predictive symptoms potentially life threatening.
anaesthetic solutions (2% lidocaine). It of significant injury and scar formation were
can then be removed using irrigation or for- prolonged drooling and dysphagia (100% Prevention
ceps. Putty is often very difficult to remove sensitivity and 91% specificity). In another, Labelling, formulation, and child-proof con-
and may require otomicroscopic removal. haematemesis and respiratory distress were tainers and caps, have proved effective in pre-
Sharp objects also usually require ENT always associated with severe injury. vention, when legislated. Storage of strong
removal. caustics should be in locked cupboards or sim-
Failure to remove in the ED will require ENT Examination ilar, and handling should occur out of the
referral. Inspection following removal is Many children will have normal examination reach of children. More hazardous caustics
advised to ensure there is no persisting findings, but oral burns may be demonstrated are available in developing countries and
foreign body and to assess for trauma or by inflammation, oedema or white areas. In at severe injury occurs more frequently.

345
14.5 FOREIGN BODIES AND CAUSTIC INGESTION

Lamireau T, Rebouissoux L, Denis D, et al. Accidental caustic

Controversies Further reading ingestion in children: Is endoscopy always mandatory?
J Pediatr Gastroenterol Nutr 2001;33(1):814.
Anderson KD, Rouse TM, Randolph JG. A controlled trial of Lovejoy FH Jr, Woolf AD. Corrosive ingestions. Pediatr Rev
In caustic ingestion, acute corticosteroids in children with corrosive injury of the 1995;16(12):4734.
oesophagoscopy has been advocated as esophagus. N Engl J Med 1990;323(10):63740 [comment]. Mantooth R. Foreign bodies, ear. eMedicine 2001.
Ansley JF, Cunningham MJ. Treatment of aural foreign bodies Nuutinen M, Uhari M, Karvali T, Kouvalainen K. Consequences
critical to the early diagnosis and in children. Pediatrics 1998;101(4):63841. of caustic ingestions in children. Acta Paediatr 2001;83
management of burns. This may help Christesen HB. Epidemiology and prevention of caustic (11):12005.
ingestion in children. Acta Paediatr 1994;83(2):2125. Samad L, Ali M, Ramzi H. Button battery ingestion: Hazards
assess severity of injury and likelihood Cox RJ. Foreign bodies, nose. eMedicine 2001. of esophageal impaction. J Pediatr Surg 1999;34
of stricture formation. However, as de Jong AL, MacDonald R, Ein S, et al. Corrosive esophagitis in (10):152731.
children: A 30-year review. Int J Pediatr Otorhinolaryngol Tong MC, Ying SY, van Hasselt CA. Nasal foreign
there is no clearly effective 2001;57(3):20311. bodies in children. Int J Pediatr Otorhinolaryngol 1996;35
treatment and stricture formation is Karnak I, Tanyei FC, Buyukpamukou N, et al. Pulmonary (3):20711.
effects of household bleach ingestion in children. Clin Ziegler DS, Bent GP. Upper airway obstruction induced by
uncommon, it is difficult to recommend Pediatr 1996;35(9):4712. a caustic substance found responsive to nebulised
this routinely. Kiristioglu I, Gurpinar A, Kilic N, et al. Is it necessary to adrenaline. J Paediatr Child Health 2001;37
perform an endoscopy after the ingestion of liquid (5):5245.
household bleach in children? Acta Paediatr 1999;88
(2):2334.

346
 15

SECTION
OBSTETRICS AND
GYNAECOLOGY
Section editor Jeremy Raftos

15.1 Paediatric gynaecology 347 15.2 Emergency contraception 354

15.1 Paediatric gynaecology

Pamela Rosengarten Sonia Grover

in this age group is non-infectious in origin

ESSENTIALS and results from irritation of the vaginal
and vulval skin, particularly where there is
Prepubescent girls contact between the labial surfaces.2,3 Fac-
1 Vaginal examination is inappropriate in paediatric patients and usually provides tors that contribute to genital inflammation
little information. Ultrasound may provide further information or, if necessary, an include the vaginal and vulval skin of chil-
examination under anaesthesia (EUA) is more appropriate. dren being thin and atrophic due to low oes-
trogens and therefore easily irritated. In
2 Vulvovaginitis is the most common gynaecological problem in prepubertal girls addition, lack of protective labial hair and
and in most cases a specific infectious cause cannot be identified.
fat pads as well as moisture from wet swim-
3 Vaginal bleeding in the prepubertal female is abnormal after 34 weeks of age. ming costumes, obesity and poor hygiene
may play a role.24 Additionally, the pres-
Adolescents ence of bowel flora, which is the normal flora
1 Laboratory confirmation is required to make an accurate diagnosis of a vaginal in the atrophic vagina, may contribute to
discharge. skin irritation and the other related common
symptom of offensive smell.
2 Pregnancy-related conditions need to be considered for all adolescents with pelvic Infectious vulvovaginitis is less common
pain or abnormal vaginal bleeding.
and is due to an overgrowth of one organ-
3 Abnormal vaginal bleeding may be secondary to an underlying haematological ism, for example Group A Streptococcus,
condition. Staphylococcus, enterococci and Escherichia
coli. In this instance, profuse discharge is usu-
ally present with marked skin inflamma-
tion, often beyond the contact surfaces
occur is at the time ovarian activity com- of the labia.3 Isolation of an organism
PREPUBESCENT mences with the onset of puberty. that has strong sexual transmission, such
GYNAECOLOGY Other conditions that cause vaginal dis- as Neisseria gonorrhoeae or Chlamydia tra-
charge in the prepubertal girl include vulvo- chomatis, generally indicates sexual abuse
vaginitis, lichen sclerosis, foreign body, or sexual activity and therefore warrants
Vaginal discharge eczema and pinworms. further investigation.3,4
Introduction Lichen sclerosis is an uncommon condi-
Vaginal discharge in neonates and prepu- tion of unknown aetiology. It may present
bertal girls can be physiological. In neo- in childhood with vulval irritation, pruritus,
Vulvovaginitis dysuria or bleeding.1,2 Examination reveals
nates, a white mucoid vaginal discharge is
present in most baby girls and is due to This is the most common gynaecological pale atrophic patches on the labia and peri-
the effects of maternal oestrogens; it disap- problem in childhood, usually occurring in neum. The patches can be extensive and
pears by about 3 months of age. The second girls aged between 2 years and prior to coalesce, and with scratching lead to chronic
period where physiological discharge may the onset of puberty.1,2 Most vulvovaginitis inflammation and purpuric haemorrhage

347
15.1 PAEDIATRIC GYNAECOLOGY

into the skin. The condition usually persists avoidance of potential irritants such
with intermittent exacerbations. Most Examination as soaps and bubble baths and other
resolve before puberty although some may A general examination including sexual causal factors, such as tight/
continue to have problems into adult life.5 development is required. Perineal, vulval synthetic clothing and wet bathers;
Eczema may contribute to the symptoms and introital examination may be required vinegar (add one cup white vinegar in
of vulvovaginitis with the addition of itch. for the above conditions. Attempts must a shallow bath and soak) or the use of
In these cases eczema is usually present be made to ensure it is not a traumatic event a simple barrier cream to the labial
elsewhere on the body and can be superim- for the child. The perineum is best examined area (e.g. zinc-castor oil or nappy rash
posed on the irritation due to the discharge. either with the girl supine with heels cream) or both.
Foreign bodies are a potential cause for a together and knees flexed and hips Rarely, if the problem persists, further action
persistent, unresolving, often bloodstained abducted or in the lateral position with may be required. The natural history of vul-
offensive discharge. knees drawn up to the chest.6 Vaginal exam- vovaginitis is for recurrences to occur up
Candida is very uncommon in the prepu- ination is inappropriate in paediatric until the age when oestrogenisation begins.
bertal girl unless there has been significant patients and usually provides little further If a primary bacterial cause is suspected, cul-
antibiotic use or they are still in nappies.2,4 information. Specific external examination tures should be taken and treatment com-
(Thrush thrives in an oestrogenised environ- of the perineum usually reveals mucoid dis- menced with the appropriate antibiotics,
ment, not in the atrophic setting.) In this age charge and reddened introitus, particularly e.g. initial amoxicillin, and adjusted when
group, recurrent or unexplained candida on the contact surfaces between the labia. culture results are available.
requires exclusion of diabetes mellitus or The presence of a profuse discharge or Where itch is a dominant symptom
other causes of diminished immune function. marked skin inflammation, especially if it consider:
extends beyond the contact surfaces of the
History labia, suggests an infectious cause. An offen- Pinworms, especially if perianal
The child or parent/caregiver usually sive discharge can occur with vulvovaginitis excoriation is present, and treat the
describe: or foreign body. A bloody discharge can occur child and their family with pyrantel
with vulvovaginitis (particularly with Shigella 10 mg kg-1 (maximum 500 mg) orally
an offensive vaginal discharge;
or Group A streptococci).6 The presence of or mebendazole 50 mg (<10 kg), 100 mg
erythema (redness)/irritation of the labia
perianal excoriation suggests pinworm. (> 10 kg) orally (contraindicated in
and perineal skin;
pregnancy and <6 months of age)
itch, dysuria and urinary frequency may
Investigations as a stat. dose and repeat 2 weeks
also be present.
Swabs are generally not required. If taken in later.7
These symptoms often fluctuate in severity. Lichen sclerosis in the asymptomatic
mild cases, they usually reveal a growth of
A general medical history is required, with patient does not require treatment other
mixed coliforms.3 If discharge is visible or
specific history of the symptoms and their than
profuse or marked erythema is present,
duration including nature of discharge as reassurance. In symptomatic patients,
introital swabs should be taken for culture.
well as any previous treatments. Also impor- avoidance of irritants, improved hygiene,
Vaginal swabs are painful and distressing
tant is past history of urinary tract infection, reduced trauma and barrier ointments,
and are not required.
encopresis, constipation, enuresis, the pres- e.g. nappy rash creams. In more severe
If urinary symptoms are present, the urine
ence of skin disorders, and any other illness, cases, a brief course of topical steroid (1%
(midstream urine; MSU) should be checked
including antibiotic use in the previous 4 hydrocortisone) and referral to a
to exclude urinary tract infection. A pinworm
weeks. Although a history of perineal dermatologist or paediatric
test should be taken if itch is a prominent
hygiene (e.g. wipe front to back, frequency gynaecologist is required.
symptom. This requires briefly placing clear
of bathing, type of underwear/clothes, spe- Eczema, especially if skin disease is
sticky tape on the perianal skin in the morn-
cific irritants, such as bubble baths or use of present elsewhere. Combined treatment
ing and viewing under a microscope for
feminine hygiene sprays in the adolescent of the vulvovaginitis (as above) and
eggs. Otherwise worms may be seen in the
population, etc.) should also be established, hydrocortisone or Diprosone may be
perianal region at night.
there is limited evidence to support the role indicated.
Ultrasound may provide further informa-
of this in the pathogenesis of vulvovagini-
tion, especially if there is a possibility of for-
tis.3 Where itch is the dominant symptom, Foreign body
eign body, or if necessary an EUA may be
pinworms should be considered and ques- If foreign body is suspected then an exami-
more appropriate.
tions asked about family symptoms. nation under anaesthesia with vaginoscopy
Although uncommon, the possibility of is usually required. If the presence of a for-
Management
sexual abuse should always be considered eign body is questionable then an ultra-
Vulvovaginitis
in a child that presents with genital symp- sound may be beneficial to visualise the
Management consists of:
toms, and check for other signs that may object. The most common foreign bodies
be present including alterations in behaviour Explanation and reassurance. are tiny pieces of tissue paper. The possibil-
such as phobias, eating or sleeping disorders. Toileting/hygiene advice: ity of sexual abuse should be borne in mind.

348
15.1 PAEDIATRIC GYNAECOLOGY
15

OBSTETRICS AND GYNAECOLOGY

genital enlargement, premature sexual mat- A urethral prolapse will be evident on
Vaginal bleeding uration or virilisation may be associated with examination of the perineum as a friable,
Introduction hormonally active ovarian or adrenal tumour. red-blue (doughnut-shaped) annular mass.
Vaginal bleeding in the prepubertal child is
abnormal after 34 weeks of age.6 Investigations
Non-vaginal causes of bleeding
Causes of vaginal bleeding in the prepu- Full blood examination should be performed if
Haematuria may stain the underwear or
bertal girl can be classed as hormonal and the child appears clinically anaemic or bleed-
nappy and be reported as vaginal bleeding.
non-hormonal. ing has been prolonged. Coagulation studies
This will become evident upon urinalysis.
may be indicated. Perineal swabs should be
Urethral prolapse often presents with bleed-
Hormonal causes ing thought to be vaginal in origin. The peak
taken for microscopy and culture where there
Neonates A withdrawal bleed from mater- is evidence of severe vulvovaginitis.
age is 58 years. Bleeding disorders are an
nal oestrogens is common and does not In trauma, where there is a history of
uncommon cause of vaginal bleeding but
require investigation or treatment. This will penetrating injury or where it is not possible
should be considered when there are other
cease after approximately 4 weeks of age. to perform adequate perineal examination,
systemic signs of a bleeding tendency, e.g.
an examination under anaesthesia is
bruising and petechiae.
Onset of menstruation Consider as pre- required. Midstream urine for micro and cul-
mature (precocious puberty) if this occurs ture should also be performed where haema-
at less than 8 years of age.7 History turia is present.
A general medical history including family In the child less than 8 years of age who
Non-hormonal causes and past history should be taken. has signs of puberty evident, referral to a
Vulvovaginitis In prepubescent girls, vagi- The history of vaginal bleeding should paediatric endocrinologist or gynaecologist
nal bleeding in this age group is most com- focus on the amount and circumstances of is required.
monly due to more severe vulvovaginitis bleeding, the times of recurrences and the
(the most common pathogens are Shigella presence of associated pain. A history Management
or Group A b-haemolytic streptococci).6 regarding any perineal trauma should be eli- Trauma
The discharge is brown and often offen- cited as well as the possibility of a foreign A warm bath several times a day to help
sive (see above under vaginal discharge, body. Other medical disorders and symptoms voiding as well as assist in keeping the peri-
vulvovaginitis). of blood dyscrasias, such as epistaxis and neum clean is all that is required. The girl
bruising should also be noted. may find voiding in a bath more comfort-
Trauma Accidental injury usually results able. Antibiotics are rarely required. Exami-
from straddle injuries where the girl falls nation under anaesthesia is only required
Examination
on a narrow object (e.g. bicycle cross bar, if there is persistent bleeding, uncertain ori-
Vital signs and haemodynamic stability
jungle gym). Non-accidental injury/sexual gin of bleeding, massive bruising or history
should be established. General examination,
abuse should be considered in trauma, espe- of a fall on a potentially penetrating object.
including sexual development, should be
cially where there is a hymenal tear without Suspected sexual abuse must be referred.
performed. On examination, excessive bruis-
a history of accidental penetrating injury. ing or petechiae and generalised skin disor-
ders, such as eczema, should be noted. Urethral prolapse
Foreign body Vaginal foreign body may Specific examination requires abdominal The management is conservative as long as
also result in bloodstained discharge and and vulval/perineal examination. Abdomi- voiding is normal. Warm baths may be of
should be considered if no infectious cause nal examination should note abdominal ten- assistance. Topical oestrogen cream assists
is identified or there is failure to respond derness and the presence of a mass. Perineal in the resolution.
to appropriate treatment. In bleeding sec- examination should note the presence of
ondary to excoriation from pinworms, lichen vulvovaginitis, other skin disorders, such as Labial adhesions
sclerosis or eczema, examination should eczema, or traumatic injury. Labial adhesions are seen in infancy, and
demonstrate eczema and lichen sclerosis. The common perineal injuries in trauma usually resolve by about 8 years, although
The presence of anal and perineal excoria- are bruising/haematoma of vulva and peri- they may occasionally persist through to
tion suggests pinworms and treatment as clitoral folds and superficial lacerations of puberty when they will resolve around the
previously described under vulvovaginitis. the labia minora and periurethral tissue. If time of menarche. The adhesion is not
a hymenal tear is present, a history of congenital, but it is acquired from a second-
Tumours Although rare, the possibility of a penetrating injury, e.g. broom handle or ary adherence of the atrophic surfaces of
genital tumour should be considered when bed post should be sought. If there is no his- the labia minora, presumably as a result
there is chronic genital ulceration, non-trau- tory of penetrating trauma then sexual of irritation.1,2
matic swelling, tissue protruding from the abuse needs to be considered. Labial adhesions are usually asymptomatic
vagina or foul smelling, bloody, vaginal dis- A foreign body in the vagina may be evi- in children and do not need to be divided
charge. Vaginal bleeding associated with dent on examination of the perineum. as long as the child is able to void. When

349
15.1 PAEDIATRIC GYNAECOLOGY

symptoms occur they usually relate to diffi- bacteria seen in the prepubertal female. warts, herpetic lesions, the nature of the
culty with urination or pooling of urine behind Oestrogen also influences the cervix, result- discharge and evidence of excoriation.
fused labia, which results in irritation.1,2,5 ing in increased mucous production, which is Adolescent girls who have never been
Urinary tract infections are seldom a compli- largely responsible for the physiological sexually active and/or who have not used
cation.5 Parents should be reassured that vaginal discharge.8 tampons should not be examined internally.
separation of the labia will occur when oestro- Vaginal infections include candidiasis, If there is a suspicion of a vaginal foreign
genisation commences as the child grows. bacterial vaginosis and trichomoniasis, with body, vaginal trauma or PID in a girl who
Although it is possible to divide the adhesions only the latter being sexually transmitted. is sexually active or uses tampons, a gentle
with lateral traction this is frequently distres- Chlamydia and gonorrhoea are sexually vaginal examination may be considered.
sing for the child and the parents, and unnec- transmitted infections of the cervix and/or However, even sexually experienced girls
essary. There is a considerable recurrence rate the upper genital tract, which may also may be unable to proceed with the examina-
after this approach.5 result in a vaginal discharge. tion and alternative forms of assessment
The use of topical oestrogen cream is like- may need to be considered.
wise unnecessary, and is associated with sig-
nificant failure and recurrence rates, but
may occasionally be indicated when cathe-
History Investigations
Microscopy, culture and wet-preparation
terisation is required for a micturating Diagnosis can often be suspected from the
analysis of a vaginal discharge will help to
cystoureterogram. history and the appearance of the discharge.
confirm the diagnosis of candidiasis, bacte-
Associated nappy rash or vulvovaginitis is
managed as described above.
Candida infections typically present as a rial vaginosis and trichomoniasis.
cheesy white discharge and are The gold standard for diagnosis of both
associated with vulval and vaginal Chlamydia and gonorrhoea is culture from
Distressing vaginal or itching, dysuria or superficial dyspareunia a cervical sample. However, both infections
perineal pain in sexually active girls. They are can readily be detected with a high degree
Severe perineal or vaginal pain, often commonly associated with antibiotic use, of sensitivity using a molecular replication
described as a vaginal shooting pain that stress and diabetes mellitus. technique (either PCR or LCR) performed
is very distressing, is reported in the prepu- Bacterial vaginosis generally presents as on samples collected from either the cervix
bertal girl. It predominantly occurs in the a homogenous white discharge or a first-catch urine.
evening waking from sleep. The likely associated with a fishy odour. Symptoms
cause for this is pinworms (Enterobius vermi- of vaginal irritation are uncommon.
cularis). The worms, usually found in the
Management
Trichomoniasis is a sexually transmitted
perianal region, can become lost within Candida albicans is the most common
infection (STI) and typically presents with
cause of vaginal thrush. One treatment
the vagina. When they crawl onto the thin a frothy, green vaginal discharge.
option is clotrimazole 10% cream
atrophic hymen in the prepubescent girl Chlamydia and gonorrhoea infections
inserted vaginally as a single dose at
they cause the distressing pain. Treatment may present as vaginal discharge,
night. An alternative, if the patient, is
with mebendazole, with repeated treat- intermenstrual or post-coital bleeding,
intolerant of topical therapy and not
ments (usually 3 at weekly intervals), is urethritis or pelvic inflammatory disease
pregnant, is fluconazole 150 mg orally as
required to ensure clearance. (PID). Chlamydia and gonorrhoea may
a single dose.10
also be identified incidentally due to the
Bacterial vaginosis should be treated in
high incidence of asymptomatic
symptomatic patients and in those at
ADOLESCENT infections. The presence of lower
high risk of acquiring other STIs. Oral
GYNAECOLOGY abdominal pain and/or dyspareunia in
metronidazole 400 mg bd for 5 days is
the history increases the likelihood of PID,
recommended in non-pregnant
with a positive and negative predictive
patients.10
Vaginal discharge value of 17% and 100% respectively.9
Trichomoniasis can be treated with oral
STIs may be contracted by oral or digital
Introduction metronidazole 2 g orally as a single
contact and do not necessarily require
Vaginal discharge in an adolescent female dose.10
penetrative intercourse for transmission.
may be physiological, a symptom of vaginal Chlamydia treatment is with either a 7-
or cervical infection or secondary to a vagi-
The most common vaginal foreign body
day course of doxycycline (100 mg bd) or
in adolescent girls is a retained tampon.
nal foreign body. Under the influence of oes- a single oral dose of 1 g azithromycin.10
The presenting symptom is often a
trogen there is an increase in the glycogen Gonorrhoea has developed resistance
malodorous discharge.
production by the vaginal epithelial cells, to a number of antibiotics and therefore
which supports the growth of lactobacilli treatment should be based on local
in the vagina, which leads to lowering of Examination sensitivities. In urban Australia a single
the vaginal pH to 3.84.5. The acidic envi- Inspection of the external genitalia should be dose of 250 mg ceftriaxone
ronment helps to inhibit the growth of performed, specifically looking for evidence of intramuscularly in combination with

350
15.1 PAEDIATRIC GYNAECOLOGY
15

OBSTETRICS AND GYNAECOLOGY

cover for Chlamydia is currently patients admitted with menorrhagia have line, regimens include oral norethisterone
recommended.10 been found to have an underlying haemato- 510 mg every 2 hours for four doses,
A retained tampon can generally logical disease, the most common of which is followed by 5 mg two to three times a day
be removed in the ED. However, a coagulopathy, half of which are von Wille- for 14 days.8 Commencing the oral con-
occasionally the swollen tampon brands disease and the other half due to traceptive pill once the bleeding has ceased
cannot be extracted due to a hymenal platelet problems or dysfunction other fac- may be sensible, as cessation of the proges-
ring and removal under general tor deficiencies are very rare.11,14 togens will almost invariably result in bleed-
anaesthesia is required. If the tampon Chronic untreated menorrhagia may also ing recommencing.
is removed in the ED it should be present with signs and symptoms of anaemia. Alternatively, treatment may include oral
immediately placed into a container of conjugated oestrogens 0.6251.25 mg
water to limit exposure to the often Examination every 46 hours or oral oestradiol 12 mg
offensive odour. The haemodynamic status of the patient every 46 hours until the bleeding stops,
and the severity of the bleeding should be which is usually about 24 hours. The dose
Disposition assessed. A general examination should be is then reduced to once or twice daily. Intra-
Adolescents with a sexually transmitted infec- performed, particularly noting evidence of venous conjugated oestrogens are no longer
tion should have a follow-up communicable a haematological disorder or anaemia. available in Australia and have been asso-
disease consultation to discuss prevention ciated with thromboembolic complications,
of further STIs. Ongoing contraception and consequently oral regimens are generally
Investigations
contact tracing should also be addressed. preferred.17 This approach is worth taking
A full blood count, iron studies and coagula-
Gonorrhoea and Chlamydia are notifiable when progestogens have already been used
tion screen will assist in identifying anaemia,
diseases in some Australian states. and failed, but can be used first line.
an underlying coagulopathy or haematolo-
Again, the oral contraceptive pill should be
gical cause for the bleeding( although cau-
commenced for ongoing control, particularly
tion needs to be taken in interpreting
Abnormal vaginal bleeding these results as stress may result in apparent
if there is a need to allow some time before
further menses in the context of a low haemo-
Introduction normalising of the test results, particularly
globin. Supplement with iron therapy.
Abnormal vaginal bleeding may be caused by in von Willebrands disease11). A pregnancy
Menorrhagia, in the absence of a coag-
a complication of pregnancy, trauma, infec- test should also be performed.
ulation disorder or other underlying pa-
tion or it may be secondary to contraceptive Ultrasound may be helpful when ovarian
thology, can be treated with non-steroidal
use. The most common causes of heavy vagi- pathology is suspected and can sometimes
anti-inflammatory agents or tranexamic
nal bleeding are anovulatory bleeding or be used to assess the thickness of the endo-
acid, which reduce the menstrual loss by
an underlying haematological condition.11 metrium, although transabdominal views
33% and 50% respectively,11,18 or, alterna-
Menstrual cycles in adolescents are often may be limited. An STI screen should be
tively, low-dose oral contraceptive therapy.12
anovulatory due to the gradual maturation obtained if clinically indicated, as Chlamydia
of the hypothalamicpituitaryovarian axis, in particular can cause intermenstrual or
which can take up to 5 years after the men- heavy bleeding. Disposition
arche.1114 Anovulatory or dysfunctional Dilation and curettage is very rarely The identification of a coagulation disorder
uterine bleeding generally presents as irreg- indicated for a diagnosis and is not a recog- will necessitate review by a haematolo-
ular, often heavy, blood loss. Anovulatory nised treatment for dysfunctional uterine gist. Pregnancy-related bleeding should be
bleeding may result from a relative deficiency bleeding.15 reviewed by an obstetrician/gynaecologist.
of either oestrogen or progesterone. Relative Anovulatory bleeding and simple menorrha-
oestrogen deficiency is more common in thin Management gia can be managed by either a primary care
young women who have just commenced Heavy bleeding, either acute or chronic, physician or a gynaecologist.
their menstrual cycles. The relative oestro- should be treated with fluid and blood pro-
gen deficiency results in a thin, atrophic ducts as clinically indicated.
endometrium, which can bleed profusely. Oestrogens and progestins are generally
In girls with higher endogenous oestro- used in the management of anovulatory
Pelvic pain
gen levels, anovulatory menstrual cycles bleeding. Suggested regimens vary with Introduction
are more likely to result in a relative proges- respect to the route of administration, the The differential diagnosis of acute pelvic
terone deficiency due to failure of the luteal dose and the type of hormone used. Cur- pain in an adolescent includes both gynae-
phase. In this case the unopposed oestrogen rently there is no convincing evidence in cological and non-gynaecological causes.
results in thickening of the endometrial lin- favour of any particular regimen.16 Common gynaecological causes include
ing, which can bleed erratically. For both the acute and non acute bleed pregnancy-related pain, dysmenorrhoea,
Menorrhagia, whether associated with tranexamic acid should be used. This can ovulation pain (Mittelschmertz), ovarian
ovulatory or anovulatory cycles, may be a be used in combination with hormonal cysts and their complications, torsion of
marker of systemic illness. Up to 20% of approaches. If using progestogens first the ovary, pelvic inflammatory disease

351
15.1 PAEDIATRIC GYNAECOLOGY

(PID), endometriosis and congenital uterine haemoperitoneum.8 Bleeding disorders several days after the onset of menses
abnormalities, such as imperforate hymen. need to be considered if recurrent and persists beyond the end of the
haemorrhagic cysts occur or a significant menstrual period.
haemoperitoneum.
History
A general gynaecological history should be
Complex ovarian cysts include dermoid Examination
cysts, which are often asymptomatic but Examination should include vital signs and
taken, with particular note made of the rela-
may cause local pressure symptoms. abdominal examination. Pelvic examination
tionship of the pain to the menstrual cycle.
History should also include the nature of
Ovarian torsion is an uncommon can give additional information regarding
condition and is almost exclusively seen the location of the pain and its relationship
onset of the pain and a history of similar epi-
in association with ovarian pathology. to pelvic organs. Uterine, adnexal or cervical
sodes. The pain of dysmenorrhoea, Mit-
The history is typically an acute onset of motion tenderness should be sought if pelvic
telschmertz, endometriosis and imperforate
colicky iliac fossa pain, with the right examination is performed. The identification
hymen may be cyclical. However, they may
being affected more than the left. There of an adnexal mass is clinically imprecise.
also present during the first episode or as
may be associated nausea, vomiting and However, it should prompt consideration of
an unusually severe exacerbation. An
a leucocytosis. Ultrasound has a high such conditions as ovarian torsion, tubo-
ectopic pregnancy is not excluded by the his-
degree of sensitivity and specificity in ovarian abscess or ectopic pregnancy.
tory of a recent menstrual period.
making the diagnosis of an enlarged
Primary dysmenorrhoea is common in the ovary however, doppler studies are not Investigations
adolescent population; it refers to pain necessarily conclusive and presence of A pregnancy test and a pelvic ultrasound
associated with menstruation in the flow does not exclude torsion.20,21 are the most valuable tests for excluding
absence of underlying pathology. The Pelvic inflammatory disease can have a pregnancy-related conditions. Ultrasound is
pain is generally described as crampy, range of clinical presentations, the investigation of choice for the definition
occurring in the lower abdomen, often depending on the location and the of masses and in the identification of ovar-
with radiation to the back or to the inner severity of the infection. The pain can ian cysts.23 If a congenital anomaly is sus-
thighs. Associated features may include range from mild to severe; systemic pected, information regarding the kidneys
nausea, vomiting, diarrhoea, headache features of infection are not always at the time of the ultrasound can be helpful.
and syncope. The symptoms have been present. Due to the significant Laparoscopy is reserved for pain that
attributed, in part, to an increased release complications associated with untreated cannot be adequately explained or has failed
of, or increased sensitivity to, PID, the isolated findings of adnexal, to respond to appropriate treatments. Lapa-
prostaglandins. The diagnosis is generally uterine or cervical motion tenderness roscopy may be required to exclude torsion
made on the basis of history and limited in the young woman who is sexually where a cyst is present combined with a sus-
physical examination findings. active, for which no other cause can picious history.21 It may also have a role in
Mittelschmertz is pelvic pain related to be found, have been suggested as a the investigation of chronic pelvic pain.
ovulation. The pain is generally dull in basis for the diagnosis and treatment
nature and located in either of the iliac of PID.22 Management
fossae. The pain may last from a few Endometriosis is reputed to cause a range Dysmenorrhoea will generally respond to
hours to 23 days. The pathological basis of menstrual disorders and pain antiprostaglandin agents, such as
of the pain is unclear but may be due syndromes. However, the symptoms do not mefenamic acid 500 mg orally 8-hourly
either to distension of the capsule of the appear to correlate well with the degree of or ibuprofen 400 mg orally 8-hourly. The
ovary prior to ovulation or to spillage of a disease identified at laparoscopy. The response is improved by commencing
small amount of follicular fluid at the diagnosis should be made with caution treatment prior to the onset of
time of ovulation.8,19 due to the risk of labelling a patient with symptoms.24 Lifestyle issues, such as
Simple ovarian cysts are a normal what may be seen as a sentence for increasing physical exercise and
physiological consequence of ovulation. chronic pain and infertility. decreasing stress, may also improve
They can be up to 6 cm in diameter. The Congenital obstructive abnormalities symptoms.25 For patients who fail to
pain may be from irritation of include an imperforate hymen or respond to treatment, combined oral
surrounding structures or stretching of obstruction of one side of a double contraceptive therapy is considered
the wall of the cyst. Corpus luteal cysts uterine, cervical or vaginal system. second-line therapy and further
can reach up to 10 cm in size and may An imperforate hymen may present investigation, such as ultrasound and
present as acute pain secondary to as cyclical or irregular lower abdominal laparoscopy, may be considered to
haemorrhage into the cyst. Both simple pain in a girl with well-developed identify other pathology, for example
and corpus-luteal cysts can present as secondary sexual characteristics and significant endometriosis.24
acute pain they rupture. Although the absence of menstruation. Unilateral Mittelschmertz and simple ovarian cysts
uncommon, a ruptured corpus-luteal obstruction in a double system can often be treated by reassurance.
cyst may continue to bleed and be more typically presents with However, if this is not adequate, simple
associated with significant dysmenorrhoea that commences analgesia and non-steroidal anti-

352
15.1 PAEDIATRIC GYNAECOLOGY
15

OBSTETRICS AND GYNAECOLOGY

inflammatory drugs may be required. If 14. Claessens EA, Cowell CA. Acute adolescent

the pain is recurrent then consideration Acknowledgement menorrhagia. Am J Obstet Gynecol 1981;139(3):
27780.
should be given to the use of the The contribution of Sheila Bryan as author in
15. Duflos-Cohade C, Amandruz M, Thibaud E. Pubertal
metrorrhagia. J Paediatr Adolesc Gynaecol 1996;9
combined oral contraceptive pill in an the first edition is hereby acknowledged. (1):1620.
attempt to suppress ovulation. Failure of 16. Hickey M, Higham J, Fraser IS. Progestogens versus
oestrogens and progestogens for irregular uterine
resolution of a cyst after 2 months or a bleeding associated with anovulation. In: Cochrane
simple cyst greater than 8 cm diameter Review. The Cochrane Library. Issue 3. Oxford: Update
software; 2003.
may require surgical exploration.8 17. Richlin SS, Rock JA. Abnormal uterine bleeding. In:
Complex ovarian cysts, including References Carpenter SEK, Rock JA, editors. Paediatric and
adolescent gynaecology. 2nd ed. Philadelphia: Lippincott
dermoids and endometriomas, are 1. Mroueh J, Muram D. Common problems in paediatric Williams & Wilkins; 2000. p. 20724.
gynaecology: New developments. Curr Opin Obstet
generally treated surgically. 18. Prentice A. Fortnightly review: Medical management of
Gynecol 1999;11(5):4636. menorrhagia. Br Med J 1999;319:13435.
Ovarian torsion requires urgent operative 2. Fiorillo L. Therapy of paediatric genital diseases. 19. Hann LE, Hall DA, Black EB, Ferrucci JT. Mittelschmerz.
Dermatol Ther 2004;17:11728.
intervention with the aim to preserve Sonographic demonstration. JAMA 1979;241
3. Jaquiery A, Styianopoulos A, Hogg G, Grover S. (25):27312.
ovarian tissue. 21 Vulvovaginitis: Clinical features, aetiology, and 20. Ben-Ami M, Perlitz Y, Haddad S. The effectiveness of
microbiology of the genital tract. Arch Dis Child
PID can be treated with either oral or
1999;81:647.
spectral and color Doppler in predicting ovarian torsion.
A prospective stud. Eur J Obstet Gynecol Reprod Biol
parenteral antibiotics. However, there 4. Farrington P. Paediatric vulvo-vaginitis. Clin Obstet 2002;104(1):646.
Gynaecol 1997;40(1):13540.
should be a low threshold for admission in 21. Grover S. Pelvic pain in the female adolescent patient.
5. Fischer G. Vulval disease in pre-pubertal girls. Australas J Aust Fam Phys 2006;35(11):8503.
adolescents.26 Dermatol 2001;42:22536. 22. Centers for Disease Control and Prevention. Sexually
6. Quint E. Vaginal bleeding and discharge in the paediatric transmitted diseases treatment guidelines. MMWR
and adolescent age groups. In: Pearlman M, Tintinalli J, 2002;51(NoRR-6):326, 6971.
Disposition editors. Emergency care of the woman. New York: 23. Arbel-Derowe Y, Tepper R, Rosen DJ, et al. The
McGraw-Hill; 1998. p. 395407.
Most forms of pelvic pain will require further contribution of pelvic ultrasound to the
7. Grover S. Gynaecological conditions. In: Smart , editor. diagnostic process in paediatric and adolescent
management or investigation and should be Paediatric handbook. 6th ed. Melbourne: Blackwell gynaecology. J Paediatr Adolesc Gynaecol 1997;10:
Science Asia; 2000. p. 34250.
referred accordingly. 312.
8. Mackay E, Beischer N, Pepperell R, Wood C. Illustrated 24. Sanfilippo J, Erb T. Evaluation and management of
textbook of gynaecology. 2nd ed. London: WB Saunders; dysmenorrhoea in adolescents. Clin Obstet Gynecol
2000. 2008;51(2):25767.
9. Blake DR, Fletcher K, Joshi N, Emans SJ. Identification of
Controversies symptoms that indicate a pelvic examination is necessary
25. Bolton P, Del Mar C, OConnor V. Exercise for primary
dysmenorrhoea (Protocol for a Cochrane Review). In:
The management of anovulatory
to exclude PID in adolescent women. J Paediatr Adolesc The Cochrane Library. Issue 3, Oxford: Update Software;
Gynaecol 2003;16(1):2530. 2003.
vaginal bleeding. 10. Therapeutic Guidelines. Antibiotic Version 13. 26. Hemsel DL, Ledger WJ, Martens M, et al. Concerns
Therapeutics Guidelines Ltd; 2006. regarding the Centers for Disease Controls published
The diagnosis and management of 11. Grover S. Bleeding disorders and heavy menses
in adolescents. Curr Opin Obstet Gynecol 2007;19:
guidelines for pelvic inflammatory disease. Clin Infect Dis
2001;32:1037.
ovarian torsion. 4159.
12. Sandofilippo JS, Lara-Torre E. Adolescent gynaecology.
 The diagnostic criteria and optimal Obstet Gynaecol 2009;113:93547.
13. Apter D, Viinikka L, Vihko R. Hormonal pattern of
management of PID. adolescent menstrual cycles. J Clin Endocrinol Metab
1978;47(5):94454.

353
 15.2 Emergency contraception
Alastair D.McR. Meyer Jacqueline E.L. Parkinson

first dose of 0.75 mg LNE is taken as

ESSENTIALS soon as possible after unprotected sexual
intercourse. This dose is repeated 12 hours
1 Emergency contraception (EC) should be made available to an adolescent who has
later.
had unprotected sexual intercourse within 72 hours prior to presentation, regardless
While the precise mechanism by which
of the stage of her menstrual cycle.
LNE prevents pregnancy is not clear, it is
2 Pregnancy must be excluded by urinary b-HCG testing. believed to work by preventing ovulation
and by altering the tubal transport of sperm
3 Commonly prescribed treatment is with levonorgestrel (LNE) EC, two 0.75 mg
and ova. Fertilisation is thus prevented.
tablets, 12 hours apart.
When used within 72 hours of unprotected
4 Follow up is essential. sexual intercourse, LNE EC prevents 85%
of expected pregnancies. Efficacy falls with
time (Table 15.2.2).
involved a high-dose oestrogen/progesto- LNE EC is contraindicated in unexplained
Introduction gen preparation. Two doses of 100 mcg of vaginal bleeding, current breast cancer and
Emergency contraception (EC) or post-coital ethinylestradiol combined with 500 mcg of pregnancy. Care should be taken with
contraception can be defined as preventing levonorgestrel given 12 hours apart resulted patients taking anticoagulants. Adverse
pregnancy after sexual intercourse. Every in withdrawal bleeding within 21 days for reactions include fatigue, abdominal pain,
year over 3.5 million unintended pregnan- 98% of women.3 gastrointestinal discomfort, dizziness, head-
cies occur in the United States alone, mostly Simple anti-emetics such as metoclopra- ache, breast tenderness and vaginal bleed-
to teenage mothers. It is believed that half mide were routinely prescribed for nausea ing. LNE is primarily metabolised in the
of these unintended pregnancies could be associated with the high oestrogen dose. liver.
avoided by the judicious use of EC. Indica- This method of EC disrupted the natural Conditions regarded as relative con-
tions for the provision of EC are shown in hormone patterns necessary to sustain preg- traindications include: severe hypertension;
Table 15.2.1.1 nancy. The high oestrogen and progestogen diabetes with nephropathy; retinopathy;
Many of these unintended pregnancies levels were believed to alter the endome- neuropathy; ischaemic heart disease; and
are later surgically terminated. Estimates trium, thereby preventing implantation. past history of breast cancer.
of as many as 170 000 such terminations The fertilised ovum had implantation pre- LNE contraception has fewer side effects
are performed in England and Wales annu- vented by blocking oestrogen and progesto- than the traditional Yuzpe method. Only
ally.2 These surgical terminations are not gen receptors, which made the endometrium 20% of patients experience nausea, which
without clinical risk and cost, as well as being hostile to implantation. Endometrial bio- usually responds to standard anti-emetics.
a major social, religious and political issue. chemistry and glandular and stromal devel- LNE EC results in 60% of women commenc-
opment were affected such that the ing their next menstruation within 3 days of
environment was hostile to the fertilised their expected date. Fifteen percent of
Clinical assessment ovum. The therapy therefore prevents women can be up to 7 days late with their
implantation and can consequently be period. It is essential that women have preg-
EC should be made available to an adoles-
described as a contraceptive rather than nancy testing if their period is more than
cent who has had unprotected sexual inter-
an abortifacient. 7 days late.6
course within 72 hours prior to presentation,
In June 2002, levonorgestrel (LNE) If unprotected sexual intercourse has
regardless of the stage of her menstrual
became the first drug licensed for use in occurred more than 72 hours but less than
cycle. Pregnancy must be excluded by uri-
Australia specifically as an EC agent. The 5 days before presentation, insertion of a
nary b-human gonadotrophin hormone (b-
HCG) testing.
Depending on the age of the young per-
son, issues of child protection and consent Table 15.2.2 Efficacy of LNE EC4,5

to treatment may need to be explored. Table 15.2.1 Indication for providing EC Hours Pregnancies
post-coital prevented (%)
Unprotected intercourse
Barrier contraception failure <24 95
Available medications Intrauterine contraceptive device (IUCD)
expulsion 2448 85
Missed contraceptive pills
Historically, the Yuzpe method of EC was Sexual assault 4972 58
commonly practised in Australasia. This

354
15.2 EMERGENCY CONTRACEPTION
15

OBSTETRICS AND GYNAECOLOGY

copper-based intrauterine contraceptive
device (IUCD) should occur. These devices Controversies References
prevent implantation. IUCDs should be Is the ED an appropriate site for 1. Trussel J, Stewart F, Guest F, et al. Emergency
contraceptive pills. Simple proposal to reduce
inserted in consultation with the gynaecol- dispensing EC, as these patients are unintended pregnancies. Family Planning and Prevention
ogy service. 1992;24:26973.
essentially well? 2. Anonymous. Hormonal emergency contraception. Drug
If inserted up to 5 days after predicted Ther Bull 1993;31(7):278.
ovulation, IUCDs can prevent 99% of There may be a place for non-medical 3. Yuzpe AA, Percivalsmith R, Rademaker AW. A multicentre
providers of the EC, given its safety. clinical investigation employing ethinylestradiol combined
expected pregnancies.5 Relative contraindi- with dinorgestrel as a post-coital agent. Fertil Steril
cations are nulliparity and patients who  The place of follow-up termination,
1982;37:50813.
4. Rodrigues I, Grou F, Joly J. Effectiveness of emergency
are at high risk of sexually transmitted should EC fail, is important to consider. contraceptive pills between 72 and 120 hours after
infection. unprotected sexual intercourse. Am J Obstet Gynecol
All patients receiving EC from EDs 2001;84:5317.
All women presenting to EDs following 5. Bryan S. Female sexual health. Emerg Med 2003;15:
unprotected sexual intercourse should be must have clear follow-up arrangements 2236.
made. The patients usual doctor or the 6. Task force on postovulatory methods of fertility
counselled regarding sexually transmitted regulation. Randomised controlled trial of
infections, ongoing contraception and the hospital female health clinic are the most levonorgestrel versus the Yuzpe regimen of combined
appropriate facilities. oral contraceptives for emergency contraception.
need for follow up. Lancet 1998;352:42833.

355
SECTION

16 RENAL
Section editor Jeremy Raftos

16.1 Acute kidney injury 356 16.5 Haemolytic uraemic syndrome 372
16.2 Haematuria 362 16.6 Nephrotic syndrome 375
16.3 Hypertension 364 16.7 HenochSchonlein purpura 379
16.4 Urinary tract infection in pre-school children 369

16.1 Acute kidney injury

Barry Wilkins

(e.g. hypoxicischaemic cause) or days (e.g.

ESSENTIALS glomerulonephritis). AKI caused by acquired
renal disease presenting to the emergency
1 Acute kidney injury (AKI, or acute renal failure) is acute reduction in glomerular department (ED) is rare. However, it is com-
filtration rate.
mon in hospitalised critically ill children,
2 A high or rising plasma creatinine is the hallmark, but this does not necessarily where it is associated with significant mor-
distinguish it from chronic renal failure. tality. AKI caused by shock states may be
preventable by attending to circulating
3 A normal plasma creatinine does not exclude AKI. volume.
4 The commonest causes of AKI presenting to the ED are haemolytic uraemic Classically, AKI has been divided into
syndrome and post-streptococcal glomerulonephritis. three broad categories, but pathophysiolog-
ical processes overlap, and the first and sec-
5 Not all AKI is oliguric or anuric. ond groups can lead to the third when
6 Most cases of oliguria are not AKI. severe or prolonged.

7 Most cases of elevated plasma urea are not AKI. Prerenal. Hypovolaemia, hypotension
and low cardiac output reduce renal
8 The most important treatment areas are circulating volume, potassium, and blood flow and cause elevation of plasma
blood pressure. urea and a mild reduction in GFR and
9 Hyperkalaemia in acute kidney injury is not necessarily caused by potassium elevation in creatinine. This is not true
retention, especially when of short duration, but by potassium redistribution from renal failure, but a normal physiological
cells. adaptation to reduced renal blood flow,
which responds to rehydration or other
10 Posterior urethral valves should be suspected in male infants with unexplained treatment of the cause.
acute kidney injury. Postrenal. Obstruction in the renal tract
11 Do not continue diuretics if two IV doses of furosemide of 1 mg kg , then 1 anywhere from the pelvicalyceal system
5 mg kg1 (over 1 hour) produce no urine. to the urethra may lead to AKI or chronic
renal failure (CRF). Crystalopathy
(obstructing the tubules) is usually
regarded as a renal cause.
Introduction
excretory function of the kidneys, with oli-  Renal. Primary disease in the
guria (<0.5 mL kg1 hr1 in children or glomerulus, which may be intrinsic
Acute kidney injury (AKI), or acute renal fail- <1 mL kg1 hr1 in infants) or anuria, and (e.g. glomerulonephritis (GN)) or
ure (ARF), means any acute reduction in a rise in plasma creatinine. Occasionally it imposed (e.g. hypoxia, ischaemia,
glomerular filtration rate (GFR), i.e. in the is non-oliguric. It may evolve over hours toxins).

356
16.1 ACUTE KIDNEY INJURY
16

RENAL
Table 16.1.1 Causes of AKI Table 16.1.2 Causes of oliguria
1. Reduced renal blood flow AKI
Severe shock from hypovolaemia or myocardial failure Hypovolaemia
Rarely, the following may cause acute tubular necrosis (ATN) or cortical necrosis, and are more Dehydration
common in children with pre-existing cardiac, renal or liver failure: Low cardiac output

Severe gastroenteritis Intrarenal vasoconstricting drugs when renal

Nephrotic syndromea impairment is mild

Burns Syndrome of inappropriate antidiuretic

Diuretic use hormone secretion (SIADH)

Haemorrhage

Salt-wasting tubulopathies

Hypoaldosteronism
Cardiogenic shock
Table 16.1.3 Causes of polyuria
Pericardial tamponade
Coarctation of the aorta or renal artery stenosis AKI
Septic shock CRF
Anaphylactic shock Diuretics
Systemic inflammatory response syndromes (e.g. severe trauma, pancreatitis) Osmotic diuresis, e.g. diabetic ketoacidosis
Hepatorenal syndrome (rare) Obstruction of the urinary tract
Malignant hypertension Nephrogenic diabetes insipidus (DI)
Arterial or venous thrombosis Nephrocalcinosis
Disseminated intravascular coagulation (DIC) Interstitial nephritis
Drugs (e.g. angiotensin-converting enzyme (ACE) inhibitors, non-steroidal anti-inflammatory drugs Renal tubular disorders, e.g. Bartters
(NSAIDs), tacrolimus, ciclosporin) syndrome
Hypoaldosteronism, e.g. Addisons disease,
2. Intrinsic renal disease congenital adrenal hyperplasia
Haemolytic uraemic syndrome (HUS)b Cerebral salt wasting
Thrombotic thrombocytopenic purpura (TTP) Sickle cell disease
Acute proliferative glomerulonephritis (GN), including post-streptococcal and other post-infectiousc Psychogenic polydipsia
glomerulonephritis
Acute tubular necrosis (ATN) caused by hypoxia plus ischaemia
Systemic lupus erythematosus (SLE) or vasculitis
IgA nephropathy
Wegeners granulomatosis Table 16.1.4 Causes of water retention
Anti-glomerular basement membrane (anti-GBM) disease and oedema
Pyelonephritis
Toxins (e.g. petrol, carbon tetrachloride, heavy metals) AKI
Drugs (e.g. aminoglycosides, vancomycin, cisplatin, tacrolimus, ciclosporin, ACE inhibitors, non- CRF
steroidal anti-inflammatory drugs (NSAIDs), amphotericin B) Nephrotic syndrome (also has hypovolaemia
Acute tubulointerstitial nephritis (TIN); drugs (e.g. furosemide, NSAIDs, penicillin, rifampicin, and oliguria)
thiazides, allopurinol), immune-mediated diseases (e.g. IgA nephropathy, SLE) and infections Chronic liver disease
(bacterial including Yersinia, viral, fungal, rickettsial) are causes Other causes of hypoproteinaemia (e.g.
HIV nephropathy protein-losing enteropathy)
Snake or arachnid envenomation Inappropriate ADH secretion or
Renal trauma administration (oedema rare)
Myoglobinuria from crush injury or rhabdomyolysis Congestive heart failure (congenital or
Haemoglobinuria (acute intravascular haemolysis, e.g. in G6PD deficiency) acquired structural heart disease,
Sickle cell disease tachyarrythmias, pericardial effusion)
Leukaemic infiltration Excess sodium and water intake
Shunt nephritis (ventriculoperitoneal shunt infection)
Radiographic contrast agents (rare in children)
Renal dysplasia, hypoplasia, and agenesis in neonates (chronic but acute presentation)
Table 16.1.5 Causes of uraemia
3. Urinary tract obstruction
Posterior urethral valves (chronic but acute presentation) AKI
Urethral stricture Increased dietary protein load
Ureterocele Catabolic states with fever, e.g. sepsis,
Pelvic or retroperitoneal tumours rhabdomyolysis
Neurogenic bladder Gastrointestinal or internal haemorrhage
Retroperitoneal fibrosis (rare) Hypovolaemia, dehydration, low cardiac
Stones, sludge (metabolic disease), pus, fungal ball, sloughed renal papilla, clots, proteinaceous output (increased reabsorption by the renal
material or crystals (uric acid, calcium oxalate, aciclovir, methotrexate, purines, sulphonamides) in tubules)
the ureters or renal tubules

4. Acute presentation of end-stage renal failure

a
Sometimes nephrotic syndrome caused by mesangiocapillary GN may present with features similar to acute post- There are many causes of oedema other
streptococcal GN.
b
In developed countries HUS and post-streptococcal GN are the commonest causes presenting to the ED, whereas in than AKI (Table 16.1.4).
tropical countries prerenal causes predominate outside the hospital environment. There are many causes of elevated plasma
c
Various infectious agents can produce nephritis similar to that of post-streptococcal GN (e.g. Mycoplasma,
Leptospira, atypical Mycobacterium, Varicella, cytomegalovirus, EpsteinBarr virus, Toxoplasma, Rickettsia, hepatitis urea (PUrea) other than AKI (Table 16.1.5).
B and C).

Causes blocked bladder catheter before assuming

Pathophysiology
See Table 16.1.1. true oliguria (Table 16.1.2). Physiology
There are many causes of oliguria other There are many causes of polyuria other The kidney has three principal areas of
than AKI. Beware of urine retention or a than AKI (Table 16.1.3). function:

357
16.1 ACUTE KIDNEY INJURY

Excretion of waste products of to effect glomerular filtration. It is auto regu- In snake and arachnid envenomation,
metabolism (e.g. urea and non-volatile lated over a wide perfusion pressure range. haemolysis, disseminated intravascular
acid) and excess ingested elements (e.g. Normally 20% of the renal plasma flow is fil- coagulation (DIC) and the venom itself
potassium). This is effected by glomerular tered. Up to 50% of urea is reabsorbed, may contribute to renal injury.
filtration, measured by the GFR. more in oliguric states; hence plasma urea Sickle cell haemoglobinopathy causes
Regulation of the volume and mineral may reflect dehydration more than GFR. papillary necrosis by red cell sickling in
composition of body fluids. This is The high metabolic rate (for tubular reab- medullary blood vessels. This may cause
effected by tubular function, by sorption) and high endothelial mass (greater gross haematuria. Sepsis and volume
absorption of >90% of filtered water, than any other organ by weight) make the depletion may contribute to renal injury.
>99% of filtered sodium and 100% of kidney vulnerable to hypoxia and ischaemia. Recurrent episodes often lead to a
glucose. nephropathy with an irreversible urine
 Endocrine functions, e.g. erythropoietin, Pathogenesis of AKI concentration defect in teenagers.
renin. Most AKI presenting to EDs has a single Approximately 10% of sickle cell crises
identifiable cause, in contrast to hospital have AKI.
Normal GFR is around 1 mL kg1 min1 in acquired AKI, which may be multifactorial. In hypovolaemia, hypotension and low
newborn infants, rising to 2 mL kg1 min1 In acute tubular necrosis (ATN), there is a cardiac output states the combination
in adults, but there is wide variation generalised hypoxic or ischaemic insult affect- of decreased renal perfusion pressure
between and within individuals, reflected ing the glomerulus and tubules. Tubular injury and afferent arteriolar constriction
in wide variations in plasma creatinine leads to failure of electrolyte absorption and a reduces GFR.
(PCr) in normal children. Measurement of tendency to loss of extracellular fluid, which In ATN, increased tubular hydrostatic
GFR is unhelpful in the acute situation. Cre- would be rapidly fatal if not limited by a reduc- pressure due to obstruction caused by
atinine clearance approximates to GFR. tion in GFR. ATN is a term that is not strictly casts and injured tubular cell debris, and
Plasma creatinine reflects GFR because cre- correct. Rarely is there histological necrosis, back-leak of filtrate through injured
atinine production is remarkably constant, though individual tubular cell apoptosis does tubular epithelium reduces effective GFR
both within and between individuals, and occur. Acute tubular dysfunction (at a subcel- further. Influx of calcium into renal tubular
is related to muscle mass. It is around lular level) is a better term. This process was cells may activate enzyme systems, leading
100 mmol kg1 day1 in infants, rising to once termed acute renal success because of to membrane and organelle dysfunction.
around 200 mmol kg1 day1 in adults. the saving of the patients life by the kidney Birth asphyxia may precipitate renal vein
There is a little absorption from, or secretion taking itself out. Pre-existing haemodynamic thrombosis in neonates, commencing
into, the tubule and this is abolished by compromise, CRF, age, hypovolaemia, diure- within the kidney, spreading into larger
Histamine-2 (H2) receptor antagonists. In tics, diabetes, or proteinuria favour the induc- veins, but thrombogenic states (e.g.
AKI with complete cessation of glomerular tion of ATN with any insult. protein S or protein C deficiency) are rare
function, plasma creatinine (PCr) increases Vasoconstricting local mediators, includ- causes. There are firm palpable kidneys
by up to 150 mmol L1 day1 in infants and ing adenosine, angiotensin II, endothelin, with haematuria.
300 mmol L1 day1 in adults. In less severe thromboxane, leukotrienes, urotensin, plate- Renal function may deteriorate suddenly
AKI, PCr rises by >50 mmol L1 day1. In let activating factor, heat shock proteins, in some patients without an oliguric
stable renal function, GFR can be estimated oxygen free radicals and superoxides, phase. Obstruction, sepsis,
by the formula: 70/PCr mL kg1 min1 in together with deficiency of vasodilating hypercalcaemia and some drugs and
infants or 140/PCr mL kg1 min1 in adults, mediators, including prostacyclin, atrial other nephrotoxins may be causes.
but this is an approximation. The formula natriuretic peptide (ANP) and nitric oxide, Functioning nephrons have reduced
does not hold true if GFR is changing. are causes of the intense glomerular vaso- reabsorption capacity, thereby
Although there are published tables of PCr constriction in ATN. In future, endothelin A maintaining urine output. It has a better
and GFR according to age, gender and size, receptor antagonists may play a role in mod- prognosis than oliguric renal failure.
the variability and different PCr assays in erating renal injury. Although minimal change nephrotic
use preclude their being of much use in inter- Inflammatory infiltrate, interstitial syndrome rarely causes AKI,
preting isolated creatinine results. A normal oedema, up-regulation of adhesion mole- hypovolaemia, peritonitis, septicaemia,
plasma creatinine does not exclude AKI. cules, tubule cell swelling, loss of epithelial renal vein thrombosis and drug toxicity
Generally any creatinine >100 mmol L1 is surface area, loss of epithelial cell polarity, may precipitate AKI that is histologically
abnormal, but in infants > 50 should raise compression of peritubular capillaries, impac- ATN and may be reversible.
a suspicion of impaired GFR, in a 5-year-old tion of debris in tubules, increased intratubu- Human immunodeficiency disease (HIV)
>70, in a 10-year-old >80, and in a lar pressure, back-leak of urea, aggregation disease may first present as AKI, nephrosis
15-year-old >100. More important is the of erythrocytes, leucocytes and fibrin in peri- or CRF. Minimal change, mesangial
rate of increase of plasma creatinine in AKI, tubular capillaries, all contribute to the estab- proliferation and focal segmental
which takes 1224 hours to become evident. lished state of reduced GFR. glomerulosclerosis (FSGS) may be seen.
Renal blood flow (RBF) is normally very The following pathological processes may After initiation of AKI by glomerular or
high (one quarter of cardiac output) in order contribute to other causes of AKI. tubular injury there is a maintenance

358
16.1 ACUTE KIDNEY INJURY
16
Normal is 16 mEq L1.

RENAL
phase where GFR remains relatively low on the shin for at least 1 minute. Haema-
for several days or weeks, then a recovery turia may suggest renal parenchymal dis- Calculate the plasma osmolal gap:
phase characterised by gradual and ease. Poor urinary stream and palpable
progressive restoration of GFR and bladder points to obstruction but in the very Osmolal gap Na K  1:86
tubular function. young this can be only excluded by thorough Urea Glucose 10  Osmolality
investigation. Monitor cardiac electrical
NB. Electrolytes, urea and glucose are in
rhythm and oximetry.
mmol L1, osmolality in mosmol kg1.
Presentation If osmolal gap is >5 mosmol kg1 then
See Table 16.1.6. Investigations unmeasured solute such as ethanol or
A careful history and examination may ethylene glycol may be present.
Tests in AKI include the following. Chest X-ray will detect pulmonary oedema,
give clues to the cause of AKI. Ask about
diuretic and other drug use, recent hospita- Full blood count. pleural effusions or cardiomegaly.
lisation or radiographic investigations, diar- Plasma Na, K, Cl, Ca, PO4, Mg, osmolality. Abdominal X-ray may detect
rhoea or vomiting (may be the presenting Plasma glucose. nephrocalcinosis and spinal abnormalities.
symptom of UTI or HUS), weight change, Plasma uric acid.
All children with AKI should have a urinary
musculoskeletal symptoms, abdominal or Plasma urea and creatinine.
tract ultrasound examination to check for
flank pain, urinary dribbling, arthralgia and Plasma albumin.
two kidneys. This may diagnose polycystic
myalgia, recent upper respiratory tract Plasma pH, bicarbonate and lactate.
kidneys, renal vein thrombosis, nephrocalci-
infrction. Enquire for family history of renal Liver function tests.
nosis, hydronephrosis or other causes of
disease or hypertension. Examine for signs Coagulation screen.
obstruction. The kidneys are likely to be nor-
of heart failure. Look for fever and rash that Blood cultures.
mal in size or enlarged in AKI, but small in
might be signs of GN or tubulointerstitial Plasma creatine kinase (CK) and troponin.
CRF. A non-dilated system does not neces-
nephritis (TIN), vasculitic signs, palpable Urinalysis for protein and blood by
sarily exclude an obstructive uropathy in
bladder. Important features of hypovolae- dipstick.
anuria; hence retrograde pyelography may
mia or low cardiac output include tachycar- Urine microscopy, Gram stain and culture.
be needed.
dia (for normal pulse rates, see Chapter 1.1), Urine chemistry (Na, K, urea, osmolality,
Further tests if there is any suspicion of
prolonged capillary refill time >2 s (but creatinine). Only 1 mL is needed and can
GN or vasculitis include:
beware of cold ambient temperature), pos- be aspirated from a cotton-wool ball
tural hypotension (remember that supine placed in the nappy (calcium and Antistreptolysin O titre.
blood pressure can be preserved until late phosphate may be falsely elevated). Complement C3, C4, CH50.
in hypovolaemia and dehydration for nor- Urine protein (quantitative protein: Serology for hepatitis.
mal blood pressures, see Chapter 16.3) and creatinine ratio >20 mg mmol1 is Autoantibodies, especially anti-nuclear
possibly a gallop rhythm. Oedema, when abnormal). antibodies (ANA), anti-double-stranded
present, can be hard to find press firmly Urine urate. DNA (anti-ds-DNA) antibodies, anti-
Examination of urine sediment by microscopy neutrophil cytoplasmic antibodies
Table 16.1.6 Possible presenting is often neglected. Normal urine indicates a (ANCA), anti-glomerular basement
features of AKI pre- or post-renal cause of AKI. Cells, casts membrane (anti-GBM) antibody.
Oliguria or protein suggest intrinsic renal pathology. Consider HIV serology.
Anuria
In ATN there are granular and epithelial cell Consider isotope renal scans and
Polyuria
Haematuria casts. White cell casts or eosinophils occur angiography. Dimercaptosuccinic acid
Brown urine
in acute TIN. Red blood cell casts and heavy (DMSA) scan assesses functioning renal
Hypertension
Pulmonary oedema proteinuria occur in GN or vasculitis. A dip- tubular mass and shows scars, while
Water retention (but oedema less common) diethylene-triamine-penta-acetic acid
Recent weight gain or weight loss stick positive for blood in the absence of red
Acidosis cells in urine is suggestive of haemoglobin (DTPA) and mercaptoacetyl
Electrolyte abnormalities, especially
or myoglobin. Nitrite in urine may suggest glycylglycylglycine (MAG3) scans assess
hyperkalaemia, hyperphosphataemia
Arrhythmias UTI. Obstruction and UTI may cause pyuria. glomerular filtration and renal blood flow.
Pericarditis
Calculate the plasma anion gap, corrected They may be useful in distinguishing ATN
Gastrointestinal symptoms (e.g. anorexia,
nausea, vomiting, haemorrhage) for hypoalbuminaemia: from other causes of AKI.
Neuro-psychiatric manifestations (confusion, Echocardiography assists in assessing
agitation, seizures, headache, visual
disturbances)a Anion gap corr Na K  Cl  myocardial function or in excluding
Itching (uraemia)
45  Albumin bacterial endocarditis.
Generalised systemic illness with malaise, Bicarbonate
anorexia and fatigue 4 If the cause of renal failure still remains
Acute respiratory distress syndrome (ARDS) obscure, the paediatric nephrologist will
a
Neurological manifestations are usually late and may
NB. Albumin is in g L1, the electrolytes in consider a percutaneous renal biopsy,
represent hypertensive encephalopathy. mmol L1. generally after admission. This may show

359
16.1 ACUTE KIDNEY INJURY

various types of acute GN, vasculitis or bicarbonaturia or use of diuretics. Also, in syn- in small doses initially, because acute hypovo-
TIN that may need immunosuppression drome of inappropriate antidiuretic hormone laemia can occur. Furosemide 1 mg kg1 IV is
and/or plasmaphaeresis. there is oliguria with a high FENa. High FENa appropriate, and then 5 mg kg1 over 60 min-
and polyuria suggest natriuresis, possible utes if there is poor response. Give no more if
Urine chemistry is often neglected. Protein:
causes being a renal salt-wasting disorder, there is no response. Remember that furose-
creatinine ratio >20 mg mmol1 diagnoses
polyuric AKI, diuretics (including osmotic mide is itself nephro- and ototoxic. Mannitol
significant proteinuria, but may be falsely
diuretics) and volume overload (mediated via is not recommended as it may worsen the sit-
elevated by fever, trauma, recent surgery,
natriuretic hormones). FENa is also unreliable uation by acute intravascular expansion,
upright posture and exertion; early morning
in preterm newborns and in obstructive uropa- especially if there is no diuresis.
sample is best. Haematuria plus proteinuria
thy. Fractional urea excretion (FEUrea) may be
strongly suggests a renal parenchymal
more useful in distinguishing low renal perfu- Remove the cause Treat sepsis. Remove
lesion. Urate:creatinine ratio >1 mmol
sion forms of oliguria from ATN, especially after nephrotoxic agents (e.g. drugs and poisons).
mmol1 suggests catabolic state, or tumour
diuretics. FEUrea is <35% in prerenal syn- Treat low cardiac output with inotropes, e.g.
lysis, and uric acid nephropathy.
dromes even after diuretic use, and >50% in dopamine or dobutamine at 510 mcg kg1
Urine electrolytes and osmolality are
ATN. FEUrate is >15% in ATN and many tubu- min1 IV, but note that there is no evidence
helpful in differentiating causes of uraemia.
lopathies, and <7% in prerenal states. that low-dose dopamine either improves
Always measure the urine creatinine. Calcu-
The urine calcium:creatinine ratio will prognosis or reduces the incidence of AKI in
late fractional excretion of sodium (FENa).
help identify hypercalciuria as a cause of cal- patients at risk. Avoid potentially nephrotoxic
This is the fraction of filtered sodium that
culi or nephrocalcinosis (normal <0.7 mmol drugs, e.g. aminoglycosides, peptidoglycans,
ends up in the urine, and is calculated by:
mmol1). ACE inhibitors. Pure obstructive AKI is com-
Urine Plasma monly reversible and always treatable. It is
Na creatinine usual to treat urethral obstruction (posterior
FENa  Treatment
Urine Plasma urethral valves) by urethral catheterisation
creatinine Na (by urologist), or suprapubic catheterisation,
The principles of treatment of AKI involve:
initially. In proven vascular thrombosis,
N.B. this formula does work even if the
tissue plasminogen activator (TPA) may be
GFR is increasing or decreasing rapidly Treat established causes and compli-
of benefit, but renal vein clot removal is
(Table 16.1.7). cations First, pay attention to the airway,
unlikely to speed recovery.
In health, FENa is <1% except in high-salt breathing and circulation. Treat life-threat-
diet. In dehydrated patients, hypovolaemia ening conditions (e.g. hypoxia, hypovolae-
Compensate for the reduced GFR
or low cardiac output it is <0.1% (represent- mia, hyperkalaemia and seizures).
and impaired tubular function Avoid
ing normal renal physiology). In general, FENa Ventilation may be needed for pulmonary
or reduce the dose of renally excreted drugs.
>2% is seen in most cases of intrinsic renal fail- oedema not responsive to diuretic. Monitor
Pay strict attention to water and electrolyte
ure. However, not all low FENa represents nor- continuous electrocardiogram.
balance.
mal physiology; it may also be low in some
causes of oliguric AKI (e.g. ciclosporin toxicity). Minimise further renal injury Avoid Remove accumulated water and
Not all high FENa with oliguria represents AKI excessive fluids and electrolytes (especially minerals Water and sodium input should
with tubular injury. In prerenal uraemia, FENa Na and K). Attempt to establish a diuresis be reduced. Begin an accurate water balance
may be higher in the presence of glycosuria, to wash out intra-tubular debris. Use diuretics chart. Replace each previous hours urine

Table 16.1.7 Using plasma and urine biochemistry to distinguish causes of oliguria

Cause Plasma urea Plasma creatinine Plasma Na Urine Na (FENa) FEUrea Urine creatinine Urine osmolality

Low cardiac Moderatemarked Mildmoderate Normal, <10 mmol L 1

<35% >4 mmol L 1
> 400 mosmol kg1
output increase increase low or high (<0.5%)
Hypovolaemia
Dehydration
Renal
vasoconstriction
(e.g. ciclosporin
toxicity)

ATN High Mild increase on 1st Normal >20 mmol L1 >50% <1 mmol L1 Similar to plasma
day, then progressive or low (>3%) osmolality
increase to high

SIADH Normal or low Normal or low Low Often Very high, often Inappropriately high
100200 mmol L1 >10 mmol L1 for plasma
(>1%) osmolality, > 300,
often > 800
mosmol kg1

360
16.1 ACUTE KIDNEY INJURY
16
output plus insensible losses (1020 mL kg1

RENAL
family history of kidney failure, dialysis as a transplant carries high stakes. AKI in
day1). Refer early to the nephrology service machine, deafness, and blood or protein in the transplanted kidney is often reversible
for renal substitution therapy if diuretic is urine. with prompt management. Contact the
ineffective, for control of water, urea (plasma The same investigations as in AKI are transplant/nephrology service urgently for
urea >30 mmol L1), phosphate, metabolic mandatory. all transplants presenting to the ED for
acid and potassium retention, and to remove Acute problems in chronic renal failure any cause. Prerenal uraemia must be
any toxic drugs or poisons. Oral calcium car- include the following. excluded in any intercurrent illness.
bonate, calcium acetate (6001800 mg ele- Possible causes include:
mental calcium per meal) or phosphate
Hypertensive crisis, possibly hypertensive
binding resin helps to control hyperphospha-
encephalopathy with convulsions and volume depletion the transplanted
coma. kidney compensates less well to
taemia. Aluminium is now avoided.
Salt depletion, especially with dehydration;
superimposed gastroenteritis. delayed, antibody mediated rejection;
Optimise nutrition Supply adequate
Dehydration can lead to irreversible ciclosporin or tacrolimus toxicity (a
energy.
deterioration in GFR caused by glomerulopathy with hyaline arteriolar
hypovolaemia. Urine output cannot be thickening);
used to judge circulating volume. urine leak at the ureteric-vesical
Disposition Hypertension may be normal for the anastomosis;
All children with AKI should be admitted to a child. ureteric obstruction;
paediatric intensive-care or high-depen- Volume overload and heart failure. ATN (rare in the child presenting to ED);
dency facility under joint care of intensivist, Hyperkalaemia. See hyperkalaemia vascular thrombosis, including
nephrologist and any other relevant special- protocol in Chapter 10.5. microangiopathic thrombosis diagnosed
ties. This may involve discussions with those Arrhythmias. only on renal biopsy;
specialties in a childrens hospital and with Acidosis. Chronic acidosis may be infection;
an emergency retrieval service. managed by oral alkali administration aciclovir.
(23 mmol kg1 day1).
Hyper- or hypocalcaemia.
Acute presentation of CRF Urinary tract infection.
Urinary tract obstruction. Controversies and future
Occasionally a child with CRF (also called
Haemodialysis catheter or shunt, or directions
chronic kidney insufficiency), that may have
peritoneal dialysis catheter malfunction. Acetylcysteine has been shown to
been previously undiagnosed, may present
acutely with any of the symptoms of AKI.
Haemodialysis catheter or shunt ameliorate the acute progression of
infection. some causes of AKI, but its role is not yet
Anaemia, poor growth, clinical or radiologi-
cal evidence of osteodystrophy, or small
Peritonitis in a child on continuous defined.
cycling or ambulatory peritoneal dialysis.
kidneys on ultrasound may suggest CRF Low-dose vasopressin is being
(though polycystic kidneys are large). Ele-
Acute gastrointestinal haemorrhage
evaluated for a role in improving renal
caused by platelet dysfunction.
vated PCr rising by <40 mmol L1 day1 is blood flow and GFR.
Desmopressin (DDAVP) is a therapeutic
also suggestive that the renal failure is
agent that may be useful, together with  Dopamine A1 receptor agonists,
chronic. AKI can occur in a child with CRF,
platelets, fresh frozen plasma or e.g. fenoldopam, are being evaluated
which may be a flare of the original disease
cryoprecipitate. for a role in improving renal blood flow
or genuine AKI. Most CRF patients will
already be under the care of a nephrologist,
Anaemia is often tolerated very well, and GFR.
even down to haemoglobin levels of
who should be contacted early. Symptoms Adenosine A1 receptor agonists are
3050 g L1. Do not transfuse without
such as anorexia, nausea, vomiting, malaise being evaluated for a role in improving
consulting the nephrologist, except in
and sleep disturbances are common, but be renal blood flow and GFR, especially in
emergency. Transfusion, especially of
alert for acute deterioration in any of these. association with furosemide.
platelets, may actually worsen prognosis
History should be sought regarding med-
in HUS (see Chapter 16.5). Anaritide (atrial natriuretic peptide)
ications (diuretics, antihypertensive agents,
steroids, erythropoietin, salt supplements,
Fractures secondary to renal may improve renal perfusion, particularly
osteodystrophy. in oliguric renal failure.
alkali, vitamin D, calcium carbonate phos-
phate binder), type and frequency of renal Melanocyte stimulating factor,
support (if appropriate), other illnesses and insulin-like growth factor, epidermal
AKI in the renal transplant
usual follow up. When CRF is previously growth factor and hepatocyte growth
undiagnosed, a family history of Alport dis-
recipient factor, anti-adhesion molecule
ease, haemophilia, polycystic kidney disease, The cause of a rising creatinine or sudden compounds, free radical scavengers and
sickling or stones should be sought. Ask for oliguria must be diagnosed without delay antioxidants are also being evaluated.

361
16.2 HAEMATURIA

Benfield MR, Bunchman TE. Management of acute renal Ronco C, Bellomo R, Kellum J, editors. Critical care nephrology.
Endothelin A receptor antagonists failure. In: Avner ED, Harmon WE, Niaudet P, editors. Philadelphia: Saunders Elsevier; 2009.
Pediatric nephrology. 5th ed. Baltimore: Lippincott, Schrier RW. Acute renal failure. In: Schrier RW, editor.
may in future play a role in moderating Williams & Wilkins; 2003, p. 125366. Section VII Diseases of the kidney and urinary tract. 8th ed.
renal injury. Better OS, Stein JH. Early management of shock and Lippincott, Williams and Wilkins; 2007. p. 9301209.
prophylaxis of acute renal failure in traumatic Siegel NJ, Van Why SK, Devarajan P. Pathogenesis. of acute
 Stem cells to produce new renal rhabdomyolysis. N Engl J Med 1990;322:8259 renal failure. In: Avner ED, Harmon WE, Niaudet P, editors.
[review]. Pediatric nephrology. 5th ed. Baltimore: Lippincott,
tissue. Bidani AK, Griffin KA. Calcium channel blockers and renal Williams & Wilkins; 2003. p. 122332.
protection: Is there an optimal dose? J Lab Clin Med Siegler RL. The hemolytic uremic syndrome. Pediatr Clin N Am
 More accurate markers of GFR may 1995;125:5535 [review]. 1995;42:150529 [review].
Chan JCM, Gill JR, editors. Kidney electrolyte disorders. New Soni SS, Ronco C, Katz N, Cruz DN. Early diagnosis of acute
become available, e.g. cystatin C. York: Churchill Livingstone; 1990. kidney injury: the promise of novel biomarkers. Blood Purif

 Plasma and urinary biomarkers of

Cronan K, Kost SI. Renal and electrolyte emergencies. In:
Fleisher GR, Ludwig S, Henretig FM, editors. Textbook of
2009;28(3):16574.
Stein JH. Acute renal failure. Lessons from pathophysiology.
AKI, e.g. NGAL, NAG, KIM 1. pediatric emergency medicine. 5th ed. Philadelphia: W J Med 1992;156:17682 [review].
Lippincott, Williams & Wilkins; 2006. p. 873920. Taylor CM, Chapman S. Handbook of renal investigations in
Finney H, Newman DJ, Thakkar H, et al. Reference ranges for children. London: Wright; 1989.
plasma cystatin C and creatinine measurements in Thadani R, Pascual M, Bonaventre J. Acute renal failure.
premature infants, neonates and older children. Arch Dis N Engl J Med 1996;334:144860.
Further reading Child 2000;82:715. Thurau K, Boylan JW. Acute renal success. The unexpected logic
Griffin KA, Bidani A. Guidelines for determining the cause of of oliguria in acute renal failure. Am J Med 1976;61:30815.
Andreoli SP. Acute renal failure. Curr Opin Pediatr acute renal failure. J Crit Illness 1989;4:32. Venuto RC. Pigment-associated acute renal failure: Is the water
2002;14:1838. Mathew A, Berl T. Fractional excretion of sodium: Use early to clearer 50 years later? J Lab Clin Med 1992;119:4524.
Andreoli SP. Clinical evaluation and management. In: assess renal failure. J Crit Illness 1989;4:45. Wilkins BH, Goonasekera CDA, Dillon MJ. Chapters 2.10 and
Avner ED, Harmon WE, Niaudet P, editors. Pediatric Miller PD, Krebs RA, Neal BJ, McIntyre DO. Polyuric prerenal 3.5. In: Macnab AJ, Macrae DJ, Henning R, editors. Care of
nephrology. 5th ed. Baltimore: Lippincott, Williams & failure. Arch Intern Med 1980;140:9079. the critically ill child. London: Churchill Livingstone; 1999.
Wilkins; 2003. p. 123352.

16.2 Haematuria
Frank Willis

disease (hypertension, oedema,

ESSENTIALS proteinuria, urinary casts, poor growth or
renal impairment) is also relatively
1 In children haematuria usually originates from the kidneys. common.
2 Haematuria can be caused by urinary infection, glomerular disease, tumours,  Consider idiopathic thrombocytopenic
congenital abnormalities and coagulopathies. purpura (ITP), HenochSchonlein
purpura (HSP) and coagulation
3 All paediatric haematuria should be investigated. disorders.
4 All patients with haematuria require follow up. Causes of glomerular haematuria include:

glomerulonephritis (GN) (including

Macroscopic haematuria exists when visi- nephritis in multisystem disorders, e.g.
Introduction systemic lupus erythematosus (SLE));
ble to the naked eye and confirmed on test-
Blood in the urine (haematuria) may be visi- ing as being blood. familial nephritis (Alports disease);
ble to the naked eye (macroscopic) or be Haematuria can originate at any site in the thin basement membrane disease
detected only on dipstick testing and/or urinary tract but, in contrast to adults, lower (benign familial haematuria);
urine microscopy (microscopic). It may be tract haematuria is relatively uncommon in IgA nephropathy;
found in isolation or associated with other children (and therefore cystoscopy is rarely polycystic kidney disease.
urine abnormalities such as proteinuria, indicated).
Causes for non-glomerular haematuria
crystals and casts. It is essential to consider Remember:
include:
urinary tract infection (UTI) as a possible
cause and, if confirmed, to manage accord- Microscopic haematuria in the setting
UTI;
of an acute febrile illness can be
ingly (see Chapter 16.4). idiopathic hypercalciuria;
normal. UTI should be excluded by
Microscopic haematuria may be defined stones;
as >10 red blood cells (RBCs) per high- urine culture and the urine tested
anatomical abnormalities;
power field, or >50 RBCs mL1 of urine (con- again after the acute illness has
tumours;
passed.
firmed on three separate occasions). trauma;
Note that small numbers of red cells are Asymptomatic micro-haematuria in sickle cell disease (in relevant ethnic
normally excreted in urine. children without other signs of renal groups).

362
16.2 HAEMATURIA
16

RENAL
Features suggestive of upper tract hae- the urinary tract, e.g. vaginal haemorrhage,
History maturia include the following: rectal fissure or contamination by the child
As in all cases attending the emergency or another person (such as in Munchausen
protein is often present; syndrome by proxy).
department (ED), a relevant and thorough RBCs are often small and misshapen
general and specific history should be taken. Not everything staining the urine pink,
(dysmorphic);
It is important to obtain a family history of brown or red is haematuria.
RBC casts and tubular casts may be
Urine dipstick tests for haematuria are
renal tract disease in patients with isolated seen.
micro-haematuria. Considerations include very sensitive and will also be positive in
the possibility of familial haematuria. Check Features suggestive of lower tract haema- the presence of haemaglobinuria and myo-
for a family history of renal tract stones (sug- turia include: globinuria. Dyes and foodstuffs (e.g. beet-
gesting hypercalciuria) or sensorineural deaf- root, blackberries) can colour the urine
RBCs of normal shape (non-dysmorphic); pink/red. Urates in the urine of neonates
ness and nephritis (with Alports disease). no proteinuria.
Ask about previous episodes of haema- may also stain the nappy pink. Drugs (e.g.
turia and/or features of HSP or SLE. NB. Urinary RBC morphology alone is an rifampicin, phenothiazines, phenolphtha-
Historical features of upper tract haema- inaccurate method to determine the site of lein) and porphyria may also discolour the
turia include: origin of haematuria and should not be urine.
relied upon in isolation.
brown urine; The extent and type of other investiga-
frothy urine, which may indicate tions done while in the ED will be determined
proteinuria. by the clinical scenario. The following tests
may be considered:
Treatment/disposition
Features of lower tract haematuria include:
urine calcium:creatinine ratio Treatment of patients presenting to
pink or bright red colour; ED with haematuria will obviously
blood in the initial part of the urine >0.7 mmol nmol (hypercalciuria);
1
depend on clinical factors such as the
stream suggests a urethral origin; urine protein:creatinine ratio
presence or absence of hypertension,
blood towards the end of the urine stream > 20 mg nmol1 (glomerulonephritides,
anaemia, problems of fluid balance
suggests bladder origin. chronic renal failure);
and fluid distribution and pain or
plasma urea, creatinine electrolytes
discomfort. Biochemical, haematological
(abnormal renal function);
Examination and imaging results may also be
plasma calcium, PO4, albumin (chronic
important.
renal failure);
Physical examination should seek to elicit Whatever the ED management, all
signs of renal disease. A thorough general FBC, platelets, film (thrombocytopenia,
cases of haematuria should be
anaemia);
examination is essential, concentrating on followed up by the family doctor, a
the following in particular: coagulation screen (coagulopathy);
paediatrician or paediatric nephrologist
streptococcal serology (post-
(as appropriate).
cardiovascular status (pallor, BP, streptococcal GN);
hypovolemia and/or fluid overload pharyngeal swab culture (post-
including gallop rhythm, pulmonary streptococcal GN);
oedema, ascites, peripheral, scrotal/ complement C3, C4 (post-streptococcal
perineal oedema); GN, mesangiocapillary GN (MCGN), SLE);
abdominal examination (loin tenderness, anti-nuclear antibody (ANA), (screening Further reading
ascites, petechiae or purpura); Andreoli SP. Management of acute renal failure. In:
for SLE); Barratt TM, Avner ED, Harmon WE, editors. Pediatric
neurological status (anaemia, abdominal X-ray (renal calculi, nephrology. 4th ed. Baltimore: Lippincott, Williams &
hypertensive encephalopathy, uraemia). Wilkins; 1999. p. 111934.
nephrocalcinosis); Renal Unit Royal Hospital for Sick Children. Guidelines on
renal ultrasound (structural the management and investigation of haematuria. Glasgow,
UK: Yorkhill. Available at
abnormalities, stones, etc.);
Investigations http://www.clinicalguidelines.scot.nhs.uk/Renal%20Unit
sickle screen (sickle cell disease); %20Guidelines/Haematuria.pdf; [accessed 21.10.10].
Leticia UT, Fildes RD. Hematuria and proteinuria. In:
In children presenting to the ED with schistosomiasis serology (schistosomiasis Barakat AY, editor. Renal disease in children clinical
haematuria, investigations should focus on infection). evaluation & diagnosis. New York: Springer-Verlag; 1990.
p. 13356.
identifying the anatomical source of the hae- Royal Childrens Hospital Melbourne Clinical Practice
maturia as well as its clinical significance. Guidelines Haematuria. Available at http://www.rch.org.
au/clinicalguide/cpg.cfm?doc_id5208; [accessed
Urine dipstick testing, microscopy and
culture should always be performed as part
Differential diagnosis 21.10.10].
Willis FR, Geelhoed GC. Haematuria. In: Management
guidelines Emergency Department. Perth, Western
of the initial evaluation of a child with It is important to remember that blood in Australia: Princess Margaret Hospital for Children;
haematuria. urine may come from somewhere other than 2002.

363
 16.3 Hypertension
Frank Willis

regular service and calibration. Elevated

ESSENTIALS measurements should (if possible) be con-
firmed with a mercury sphygmomanometer.
1 Hypertension is blood pressure that is consistently above the 95th BP should be checked in both arms and at
percentile.
least one leg to exclude the possibility of
2 Severe hypertension is blood pressure that is consistently above the 99th aortic coarctation. BP should be repeated
percentile (i.e. 1% of children). after an interval in an attempt to exclude
white coat hypertension.
3 All hypertension should be investigated. Ambulatory blood pressure monitoring is
4 The most common secondary cause is renal disease. available for children and is a useful method
of further evaluating BP as well as for moni-
5 Accelerated hypertension with encephalopathy is an emergency. toring treatment effects.
6 In malignant hypertension blood pressure should be lowered slowly. Feel all pulses. As well as a thorough
general examination, physical assessment
7 Borderline hypertension is of uncertain significance, but needs follow up. following identification of elevated blood
pressure should consider specifically:
presence or absence of encephalopathy,
growth, hydration, signs of chronic disease
and presence of abdominal masses or
Introduction History and examination
bruits (cardiac, cranial and abdominal).
In children, blood pressure (BP) is a continu- History The optic fundi must be visualised, speci-
ous variable that is influenced by age, sex, Ask about signs or symptoms of underlying fically looking for papilloedema and vas-
body size, and genetic, circadian and envi- disease, urinary tract infection (UTI), drugs, cular changes.
ronmental factors. family history of renal disease or hyperten-
Hypertension is defined as BP that is sion (essential or otherwise).
consistently above the 95th percentile for Non-specific signs and symptoms of
age, gender and height (nomograms hypertension in infants may include vomit- Investigations
are available with these data and should ing, irritability, respiratory distress, failure
Investigations to be considered in ED
be present in both the emergency depart- to thrive and congestive heart failure.
include:
ment (ED) and paediatric wards) (Table In older children, also consider polyuria,
16.3.1). polydipsia, fatigability, headache, epistaxis, full blood count, electrolytes, urea and
Severe hypertension is BP consistently chest pain, abdominal pain and neurological creatinine, liver function tests (LFTs),
above the 99th centile with signs of end- abnormalities such as Bells palsy or impaired calcium, phosphate, magnesium, alkaline
organ damage such as retinopathy, nephro- vision. Enquire regarding any noted deterio- phosphatase;
pathy or left ventricular hypertrophy. ration in school performance and/or change urinalysis by dipstick for protein and
Accelerated hypertension occurs with in personality, as these can be seen in chronic blood;
severe hypertension plus neurological signs hypertension. urine microscopy and culture;
and symptoms (hypertensive encephalopa- urine catecholamines;
thy) and severe hypertensive retinopathy. Examination chest X-ray, electrocardiogram and
Prevalence of hypertension is around 1 Measurement of BP is classically per- echocardiography;
3% of children (note that this is to some formed using a mercury sphygmomanome- abdominal and renal ultrasound scan,
extent a statistical issue related to use of ter (though these are no longer universally including grey-scale sonography to
BP centiles in defining hypertension). The available, e.g. in the United Kingdom), with identify parenchymal and collecting
most common secondary cause of hyperten- the largest possible cuff (do not be misled system disease and tumours, colour and
sion in children is renal disease, though with by labels on cuffs). The inflatable part should duplex Doppler sonography to identify
increasing age (and the pandemic of child- encircle the arm and the width should cover vessel disease;
hood obesity) essential hypertension assumes 75% of the length of the upper arm. plasma renin and aldosterone (refer to
more significance. Increasingly, oscillometric BP machines lab regarding collection);
Ideally, all children presenting to ED (e.g. Dinamap) are routinely used in EDs complement C3, C4, anti-nuclear
should have a BP measurement. and elsewhere. Automated devices need antibody (ANA);

364
16.3 HYPERTENSION
16

RENAL
Haemolytic uraemic syndrome. investigation of the underlying cause. Such
Table 16.3.1 Upper limit of systolic
blood pressure by age Nephrotic syndrome. patients should be discussed with a paedia-
Renal artery stenosis. tric intensivist and nephrologist and will
Age Upper limit for normal
systolic BP (95th percentile) Renal vein thrombosis. generally be admitted to the paediatric
Acute and chronic renal failure. intensive care unit.
Males Females Aim initially for 25% reduction towards
Polycystic disease.
1 month 104 102 Renal tumours. target BP (upper limit of normal systolic
Renal dysplasia. see Table 16.3.1) in the first 1224 hours,
2 months 109 106
then the reduction should be slow, over
3 months 111 108 2448 hours. Neglecting to lower the blood
Endocrine
4 months 110 109 Catecholamine excess (neuroblastoma, pressure can lead to permanent disability or
phaeochromocytoma). death. Acutely lowering the blood pressure
6 months 110 110
Corticosteroid excess (congenital adrenal to normal is also contraindicated, because
1 year 110 110 a rapid drop in blood pressure to normal
hyperplasia).
2 years 110 110 Hyperthyroidism. can produce tissue ischaemia. This may
manifest as shock (despite normal to slightly
3 years 111 110
Vascular elevated blood pressure values), encepha-
4 years 112 111 lopathy leading to cerebral injury, retinopa-
Aortic coarctation.
5 years 113 112 thy or optic nerve infarction leading to
Arteriovenous fistula.
blindness, hypoventilation and apnoea.
6 years 115 114 Takayasu arteritis.
7 years 117 116
SLE.
Peripheral vasodilators
8 years 118 117 Intravenous (IV) nitroprusside infusion.
Metabolic
9 years 120 119 Diabetes. Starting dose 1 mcg kg 1 min 1,
Porphyria. increment by 1mcg kg 1 min 1 every
10 years 122 121
Hypercalcaemia. 510 minutes to maximum
11 years 124 123 5mcg kg 1 min 1. Beware of cyanide
12 years 127 125 Neurological toxicity above this dose, or with lower
GuillainBarre syndrome. doses in renal failure. Check a plasma
13 years 129 128
Raised intracranial pressure. thiocyanate level every 12 hours. Half-life
14 years 130 129 is very short.
15 years 133 130 Drugs IV nitroglycerine infusion. Starting dose
1 mcg kg 1 min 1, increment by
16 years 136 131 Steroids.
1mcg kg 1 min 1 every 510 minutes to
Oral contraceptives.
Data obtained with permission from the National Heart, maximum 5mcg kg 1 min 1. Half-life is
Lung and Blood Institute of the United States National Stimulant medications (methylphenidate
Institutes of Health. and dexamphetamine). very short.
IV hydralazine. 100200 mcg kg 1 IV
Recreational drugs.
bolus followed by 46mcg kg 1 min 1
Liquorice.
drug screen, (e.g. amphetamines); infusion.
cranial computerised tomography (CT) IV clonidine. 36 mg kg 1 as slow
Toxins
scan. injection followed by 0.52 mg kg 1 hr 1
Heavy metals.
Subsequent investigations will depend on infusion. Clonidine is a centrally acting
results of preliminary investigations and on vasodilator acting on spinal cord
Miscellaneous
the underlying diagnosis. a2-receptors.
Anxiety and pain.
Burns. Avoid diazoxide because it has a too-rapid,
Essential hypertension. large and sustained effect.
Causes
There are many possible causes of hyper- Beta-blockers
tension:
Treatment IV labetalol infusion. Starting dose
1 mg kg 1 hr 1, increment by
Renal Hypertensive encephalopathy 0.250.5 mg kg 1 hr 1 (to maximum
Reflux nephropathy. Treatment for encephalopathy/accelerated 3 mg kg 1 hr 1) every 30 minutes
Obstructive uropathy. hypertension is urgent. The blood pressure until desired rate of reduction in BP is
Glomerulonephritis. should be treated before proceeding with achieved. Monitor BP every 15 minutes

365
16.3 HYPERTENSION

initially. Half-life is approx 4 hours and isradipine 0.1 mg kg1 8-hourly,

Speed of reduction in hypertensive
not altered in chronic renal insufficiency. amlodipine 0.050.3 mg kg1 once daily.
encephalopathy/accelerated
Be aware of negative inotropic and Angiotensin converting enzyme (ACE)
hypertension.
chronotropic effect in patients with inhibitors. Captopril, initial test dose
cardiac failure or bradycardia, and risk of 0.1 mg kg1, then 0.34 mg kg1 day1. The role of ambulatory BP
asthma exacerbation. Lower acceptable Be aware that ACE inhibitors may measurements.
limit of heart rate is 120 in neonate, decrease intrarenal blood flow and induce
CT and MR angiography to identify
100 in children up to 3 years, 80 in or worsen renal failure.
small intrarenal vessel disease.
children 310 years, 60 in children Angiotensin II receptor (AT1) blockers,
>10 years old. e.g. losartan 0.52 mg kg1 as a once-  Angiotensin converting enzyme
IV esmolol infusion. Starting dose daily dose. inhibitor renography to identify small
200 mcg kg1 min1, increment by Clonidine, 16 mcg kg1 8-hourly. vessel disease.
50mcg kg1 min1 every 510 minutes to
 Assessment of the impact of rigorous
maximum 1000mcg kg1 min1. Half-life Beta-blockers treatment of hypertension in childhood
is very short only a few minutes, because Metoprolol, initial dose 1 mg kg1 on the later development of vascular
of plasma esterase metabolism. 12-hourly, incrementing to 2.5 mg kg1. disease, stroke, renal disease and
Beta-blockers and vasodilators may be used Atenolol, initial dose 1 mg kg1 congestive heart failure.
in combination. Beta-blockers and calcium 1224-hourly, incrementing to 2 mg kg1
12-hourly.  Evaluation of the role of dietary salt
channel blockers together are absolutely
intake in childhood on the development
contraindicated. The combination is seve- NB. Where available, local guidelines should of hypertension.
rely negatively inotropic. be referred to when using antihypertensive
agents, especially when given by the intra- Further evaluation of genetic factors
Diuretics venous route. Early consultation (as appro- and the link with obesity and insulin
In situations of clinical fluid overload, diuretics priate) with a paediatric intensivist or resistance.
can be helpful. Furosemide 0.51 mg kg1 nephrologist is wise. The role of newer angiotensin II
IV bolus, which may be repeated after 4 hours, Treatment is less urgent in severe hyper- inhibitor and angiotensin converting
is first choice. If no response in patients tension (without encephalopathy), but such enzyme inhibitor drugs.
with renal failure, try 2 mg kg1, then patients should be admitted for investiga-
5 mg kg1 infusion over 1 hour once only. tion and treatment. Early liaison with the
Avoid inducing hypovolaemia. admitting consultant is prudent.
What to do about borderline hypertension
Less severe hypertension and is less clear, though such children should be
oral management after control referred for follow up by a paediatrician or
of malignant hypertension paediatric nephrologist.
When target blood pressure is reached, Further reading
nitroprusside can be slowly withdrawn and Flynn JT. Whats new in pediatric hypertension. Curr
Hypertens Rep 2001;3:50310.
other intravenous agents replaced by oral Julius S. Clinical and physiological significance of borderline
drugs as follows. Oral antihypertensive Controversies and future hypertension in youth. Paediatr Clin N Am 1978;25:
3545.
agents should be used to treat less severe directions Mouin GS, Arant BS. Hypertension. In: Barakat AY, editor.
hypertension because of their slower and Renal disease in children clinical evaluation and

more sustained effect. Drugs that may be Significance of borderline diagnosis. New York: Springer-Verlag; 1990. p. 30728.
Report of the second task force on blood pressure control in
used include: hypertension. children. Paediatrics 1987;79:125.
Report of the task force on blood pressure control in children.
Routine or selective BP measurement Paediatrics 1977;59:797820.
Rocchini AP. Pediatric hypertension 2001. Curr Opin Cardiol
Vasodilators in paediatric EDs. 2002;17:3859 [review].
Calcium channel blockers.  Obesity and blood pressure in
Sorof JM, Portman RJ. White coat hypertension in children
with elevated casual blood pressure. J Paediatr 2000;137:
oral or sublingual nifedipine, initial 4937.
paediatric populations.
dose 0.25 mg kg1, then Vogt BA. Hypertension in children and adolescents. Curr
0.51.5 mg kg1 day1 in 34 doses Pharmacology and pharmacokinetics Thera Res Clin Exp 2001;62:28397.
Wells TG. Trials of antihypertensive therapies in children.
per day, or of antihypertensive drugs in children. Blood Pressure Monitor 1999;4:18992.

366
16.3 HYPERTENSION
16

RENAL
Appendix
Age-specific percentiles of BP measure-
ments
130 95th

125 90th
140 95th
90th 120
135 75th
115

Systolic BP
130 75th
110 50th
Systolic BP

125
50th 105
120
115 100

110 95
105 90

90 95th
85 95th
85 90th 90th
80
75
Diastolic BP (K5)

80 75th

Diastolic BP (K4)
75th
70
75 50th
50th 65
70
60
65 55

60 50
13 14 15 16 17 18 1 2 3 4 5 6 7 8 9 10 11 12 13
Years Years

Fig. 16.3.1 Boys 1318 years of age. Fig. 16.3.2 Boys 113 years of age.

130
115 95th
110 95th 125 90th
105 90th
120
100 75th 75th
Systolic BP

95 115
Systolic BP

90 50th
110 50th
85
80 105
75
100
70
65 95
90

75
95th
85 95th
70 90th
80 90th
Diastolic BP (K4)

65
Diastolic BP (K4)

75th 75 75th
60 70
50th
50th 65
55
60
50
55
45 50
0 1 2 3 4 5 6 7 8 9 10 11 12 1 2 3 4 5 6 7 8 9 10 11 12 13
Months Years

Fig. 16.3.3 Boys from birth to 12 months of age. Fig. 16.3.4 Girls 1-13 years of age.

367
16.3 HYPERTENSION

140 115
110 95th
135 90th
95th 105
130 100 75th
90th
Systolic BP

125 95

Systolic BP
50th
75th 90
120
85
115 80
50th
110 75
70
105
65

90
75
85 95th 95th
70
Diastolic BP (K5)

90th 90th
80

Diastolic BP (K4)
65
75 75th 75th
60
70
50th 55 50th
65

60 50
13 14 15 16 17 18
45
Years
0 1 2 3 4 5 6 7 8 9 10 11 12
Fig. 16.3.5 Girls 13-18 years of age. Months

Fig. 16.3.6 Girls from birth to 12 months of age.

All charts reproduced with permission from the National Heart, Lung and Blood Institute of the United States National Institutes of Health.

368
 16

RENAL
16.4 Urinary tract infection in
pre-school children
Frank Willis

midstream sample. Difficulties arise in chil-

ESSENTIALS dren too young to have been toilet trained,
who are also the group at highest risk for
1 Urinary tract infection is common and potentially serious. pyelonephritis and renal scarring.
2 Septicaemia may occur in babies. In infants and toddlers, urine bag samples
are unreliable (very high false-positive rate)
3 Renal scarring is a serious and potentially preventable complication. and should not be used. Clean catch samples
4 Urinary tract infection should be considered in all febrile infants and young are more reliable and are the preferred
children, as well as babies who are non-specifically unwell. method for non-invasive urine collection.
If samples are required urgently, bladder
5 In young children, urinary tract infection cannot be diagnosed reliably or excluded catheterisation is the most reliable method,
on clinical grounds.
though those familiar with the technique
6 Dipstick urinalysis is an unreliable method of ruling out urinary tract can consider suprapubic aspiration (SPA).
infection in infants. The yield from SPA is markedly improved
by using ultrasound to confirm a full bladder.
Samples should be sent to the laboratory
presence of vesicoureteric reflux (VUR) or for urinalysis, microscopy and culture. Find-
Introduction other anatomical abnormality. ings supportive of the diagnosis of UTI
include presence of leucocytes and organ-
Bacterial infection of the urinary tract (UTI) is Long-term complications of renal scarring
isms on microscopy and leucocyte esterase
common in the paediatric age group. Its sig- include: pregnancy-associated problems;
and nitrites on dipstick urinalysis. Organisms
nificance is greatest in young children, partic- hypertension; and, rarely, chronic renal
may be seen on Gram stain. In centres with-
ularly in the first year or two of life, where the insufficiency (see prognosis section below).
out 24-hour laboratory services, after-hours
high incidence of upper tract infection (pyelo-
samples should be sent using a urine dip-
nephritis) and the presence of immature
slide.
kidneys lead to significant potential for renal History and examination Dipstick urinalysis may be helpful in
scarring (reflux nephropathy). It is unlikely
In infants and young children with UTI, the making a provisional diagnosis of UTI.
that new scarring occurs after age 5 years.
clinical history is frequently non-specific and However, a negative result does not rule out
Data from Sweden suggest that in the first
may include irritability, jaundice (neonates), UTI in infancy. One study showed that urinal-
2 years of life, up to 3% of infants may suffer
poor feeding or fever without apparent ysis was normal in 50% of infants <8 weeks
UTI. Between 1 and 10 years of age 38% of
source. Symptoms and signs become more with confirmed UTI. Another study suggested
girls and < 1% of boys will have at least one
specific with increasing age (Table 16.4.1). that dipstick urinalysis was a reliable method
urine infection. It is important to remember
Dont forget to obtain a family history of of ruling out UTI only after age 2 years.
that recurrences are common. UTI is more
renal tract disease, in particular regarding The traditional definition of pyuria is >5
frequent in boys than girls in the first months
UTIs, VUR (probably an autosomal domi- white blood cells (WBC) per high-power field
of life, partly because of a higher incidence of
nant condition) and renal impairment. (centrifuged urine). Another definition is
obstruction including pelviureteric junction
Physical examination of young children with >10 WBC mm3 (uncentrifuged urine).
obstruction, thereafter occurring signifi-
UTI is often unremarkable or non-specifically The definition of significant bacteriuria is
cantly more often in girls (Fig. 16.4.1).
abnormal. Septicaemia, however, does occur guided by the method by which the urine
UTI is caused by organisms normally resi-
with UTI in infancy and must be considered specimen was collected (Table 16.4.2),
dent in the gut. It is thus an ascending infec-
in babies up to around 6 months of age. Fever though on occasion genuine UTI may be
tion that may affect the bladder (cystitis) or
is the best clinical marker of pyelonephritis in present with lower colony counts than would
upper renal tract (pyelonephritis), which may
infants with UTI, but is non-specific. usually be considered significant, especially
in turn result in scarring. Neonates are unusual
in babies interpret results in light of his-
as they may also develop UTI following hae-
tory and clinical findings.
matogenous dissemination of organisms.
Renal involvement is associated with: Diagnosis
Treatment
young age (especially <1 year); A reliable urine sample is required to estab-
symptoms > 5 days; lish the diagnosis of UTI. In older children ED treatment recommendations for UTI vary.
systemic upset, fever, leucocytosis; this is usually accomplished by obtaining a However, one approach is as follows.
369
16.4 URINARY TRACT INFECTION IN PRE-SCHOOL CHILDREN

140 Arrange GP or ED follow up at

2448 hours.
120
Girls Ensure urine culture and sensitivity
Boys results are checked at 4872 hours.
100
Number of cases

Age >12 months

80
Treat on clinical merits, i.e. admit or allow
60
home, parenteral or oral antibiotics.
Overnight admission to observation
40 ward for IV/IM antibiotics may be
worth considering if unwell.
20 Ensure urine culture and sensitivity
results are checked at 4872 hours.
0
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16
Year of life
Which antibiotic?
Empirical antibiotic choice, dose and dura-
Fig. 16.4.1 Epidemiology of UTI in childhood. Cases recorded in Gothenburg 19601966. tion should be guided by local sensitivity
Source: Winberg J, Andersen HJ, Bergstrom T, et al. Acta Paediatrica Scandinavia Supplement 1974;63
(Suppl. 252):120.
patterns and antibiotic guidelines. However,
consideration may involve the following:
Table 16.4.1 UTI symptoms by age
Parenteral treatment (IV/IM)
Age 02 years 25 years 512 years Ceftriaxone 25 mg kg1 twice daily IV
Failure to thrive (may not cover enterococci in young
infants), or
Feeding problems
Amoxicillin 1025 mg kg1 8-hourly IV
Screaming gentamicin 7.5 mg kg1 once daily IV/IM
Irritability (reduce gentamicin to 2.5 mg kg1 as a
single dose in patients with known or
Diarrhoea
suspected renal impairment and check a
Vomiting level before repeat dosing).
Fever
Oral treatment
Convulsions
Amoxicillin 20 mg kg1 with clavulanic
Haematuria acid 8-hourly, or cefalexin 15 mg kg1
twice daily.
Urinary frequency
Cotrimoxazole (1.53 mg kg1
Dysuria trimethoprim) twice daily is generally
Enuresis less good.
Abdominal pain
Amoxicillin is not recommended.

Loin pain Duration of treatment?

Infants up to 12 months: treat for 10 days
at full dose.
Admit to medical ward (observation ward
Table 16.4.2 Definition of significant Older children: generally treat for 510
bacteriuria only if systemically well).
days at full dose.
If discharged from observation ward,
Method of Colony forming Courses as short as 3 days may be used in
unit count (CFU mL1)
arrange general practitioner (GP) or ED
collection older children with uncomplicated lower
follow up at 2448 hours.
Clean catch > 105 tract UTI.
Ensure urine culture and sensitivity
Catheter >5  104 results are checked at 4872 hours. Management after discharge
Suprapubic >0 from ED or observation wards
Age 612 months Most centres will have a referral, manage-
If systemically unwell, admit to observation ment and investigation protocol which
Age <6 months ward or medical ward for IV/IM antibiotics. should be followed. Many of these are
Admit all for intravenous (IV)/ If not systemically unwell, consider single based on the National Institute for Clinical
intramuscular (IM) antibiotics IM/IV dose of ceftriaxone or gentamicin, Excellence Guidelines from the United
(septicaemia occurs in this age group). followed by oral antibiotics. Kingdom,1 though significant variation

370
16.4 URINARY TRACT INFECTION IN PRE-SCHOOL CHILDREN
16

RENAL
exists from place to place. In the absence of 515% of end-stage renal disease (ESRD) Post-UTI imaging protocols.
local guidelines, the following approach is in adults <50 years is due to reflux
The role of antibiotic prophylaxis.
reasonable. nephropathy.4
Arrange a proof of cure urine culture
Diagnosis and management of
At least some of these consequences may be vesicoureteric reflux.
after stopping antibiotic.
In pre-school children (both sexes), follow-
preventable by early and effective diagnosis Complementary therapies such
and treatment of UTI in very young children as lactobacillus and bifidus
ing the first documented UTI (or if previous
(though even this has been questioned by consumption.
UTI not investigated), start low-dose noctur-
nal prophylactic antibiotics. Co-trimoxazole
some authors5). Cranberry juice as prophylaxis.

(because of its long shelf life) and nitro-

Recognition of dysfunctional
voiding.
furantoin are reasonable choices. The dose
is 12 mg kg1 at night for both drugs
Recognition of sphincter and
Prevention detrusor dyssynergia.
(expressed as the trimethoprim component
in the case of co-trimoxazole). Trimethoprim The basics of good fluid intake, regular and
suspension is unavailable commercially in complete voiding, avoidance of constipation
Australia, but is a better choice than co- and proper (front to back) bottom wiping (in References
trimoxazole in countries where it is readily toilet-trained females) should be encour- 1. NICE Guidelines. Urinary tract infection: diagnosis,
treatment and long-term management of urinary tract
obtainable. aged in order to reduce the likelihood of infection in children. Available at http://www.nice.org.
A practical note after the full course of repeat infections. There is also some evi- uk/CG054 [accessed 21.10.10].
2. Smellie J, Edwards D, Hunter N, et al. Vesico-ureteric reflux
treatment, if any antibiotic is left over, con- dence of benefit from the regular use of and renal scarring. Kid IntSuppl 1975;4:6572.
tinue this as a nocte dose until finished, and cranberry juice (which acidifies urine and 3. Malek RS, Svensson J, Neves RJ, Torres VE. Vesicoureteral
reflux in the adult. III. Surgical correction: benefits. J Urol
then change to a nocte dose of the chosen reduces bladder wall adhesiveness). Prophy- 1983;130:8826.
prophylactic antibiotic. lactic antibiotics should be considered in 4. Noe HN. The current status of screening for vesicoureteral
reflux. Paediatr Nephrol 1995;9:63841.
Arrange a renal tract ultrasound cases of VUR in pre-school children, recur- 5. Hewitt IK, Zucchetta P, Rigon L, et al. Early treatment of
(although some would restrict this to chil- rent pyelonephritis, recurrent symptomatic acute pyelonephritis in children fails to reduce renal
scarring: data from the Italian renal infection study trials.
dren <3 years), and refer to medical out- UTI in older children and in pre-school chil- Pediatrics 2008;122(3):48690.
patients, hospital UTI clinic or paediatric dren awaiting renal tract imaging.
nephrology clinic (depending on local orga-
nisation) all those discharged home from
ED/observation wards who have not previ- Further reading
ously been investigated.
Controversies and future Bachur R, Caputo GL. Bacteraemia and meningitis among

It is generally not necessary to book other

directions infants with urinary tract infection. Paediatr Emerg Care
1995;11:2804.
imaging studies as consultants will differ in There is no subject in which there Crabtree EG, Cabot H. Colon bacillus pyelonephritis: Its nature
and possible prevention. In: Transactions of the Section of
the investigations that they prefer, e.g. mic- is so little uniformity of opinion and so Genitourinary Diseases of the American Medical
turating cystourethrogram (MCUG) and/or much confusion. This statement made in Association, vol 57.1916. p. 20917.
Crain EF, Gershel JC. Urinary tract infections in febrile infants
dimercaptosuccinic acid (DMSA) isotope 1916 is seemingly just as pertinent in the younger than 8 weeks. Paediatrics 1990;86:3637.
scan, though this can be expedited by early 21st century, with many questions Doley A, Neligan M. Is a negative dipstick urinalysis good
enough to exclude urinary tract infection in paediatric ED
speaking to the consultant or unit to whom about paediatric UTI management still patients? Emerg Med 2003;15:7780.
the child has been referred. the subject of debate and controversy. Hodson EM, Wheeler DM, Vimalchandra D, et al.
Interventions for primary vesicoureteric reflux. Cochrane
On the specific subject of VUR, one Database Syst Rev 2007;(3):CD001532.
author, considering the effect of Jakobsson B, Esbjorner E, Hansson S. Minimum incidence and
diagnostic rate of first urinary tract infection. Paediatrics
scientific evidence on clinical decision 2000;106:6201.
Prognosis making in children with VUR during the Kinney AB, Blount M. Effect of cranberry juice on urinary pH.
Nurs Res 1979;28:28790.
UTI in preschool years may be associated past 40 years (since its description), Montini G, Zucchetta P, Tomasi L, et al. Value of imaging
with renal damage. Risk factors are listed suggests that reported data have been studies after a first febrile urinary tract infection in young
children: data from Italian renal infection study 1. Pediatrics
in the introduction to this section. While overlooked in favour of unfounded 2009;123(2):e23946. Epub 2009 Jan 12.
most children with UTI will not suffer any speculation or clinical tradition.5 Pollack CV, Pollack ES, Andrew ME. Suprapubic bladder
aspiration versus urethral catheterization in infants:
long-term sequelae, a number will do so.
Examples include:
Current controversies and areas for Success, efficiency and complication rates. Ann Emerg Med
1994;23:22530.
potential investigation in paediatric UTI Shihab ZM. Urinary tract infection. In: Barakat AY, editor.
Renal disease in children clinical evaluation & diagnosis.
1020% of children with VUR and scars include: New York: Springer-Verlag; 1990. p. 15770.
develop hypertension during childhood;2 Parenteral or oral antibiotic Sobota AE. Inhibition of bacterial adherence by cranberry
juice: Potential for treatment of urinary tract infections.
34% of adults with reflux nephropathy treatment. J Urol 1984;131:10136.
are hypertensive;3 Duration of antibiotic treatment. Willis FR, Geelhoed GC. Urinary tract infection. In:
3050% of children with VUR have Outpatient or inpatient Management guidelines Emergency Department. Perth,
Western Australia: Princess Margaret Hospital for Children;
scarring at initial evaluation;4 management. 2002.

371
 16.5 Haemolytic uraemic syndrome
Karen McCarthy

ileum and large intestine by complex

ESSENTIALS mechanisms. Cytotoxins are produced and
they damage the microvasculature of the
1 Diagnosis is based on the presence of microangiopathic haemolytic anaemia, intestinal wall, causing haemorrhagic and
thrombocytopenia and renal impairment.
ulcerative intestinal lesions. Once the
2 Diarrhoea-associated haemolytic uraemic syndrome accounts for 90% of cases. intestinalblood barrier has been compro-
mised, the toxins gain entry to the circulation.
3 The cornerstone of treatment is careful attention to fluid and electrolyte balance. It is presumed that the toxin then initiates
4 Most patients with haemolytic uraemic syndrome have some degree of microvascular damage in the glomerular
encephalopathy. endothelium of the kidneys. Intravascular
and intraglomerular fibrin clot is formed
5 Hypertension is present in up to 50%. and at the same time there is activation of
6 Long-term follow up is necessary. the coagulation system, which leads to occlu-
sion of the vessels and glomerular loss. Ery-
7 Use of antibiotics, antimotility agents and non-steroidal anti-inflammatory drugs throcytes passing through these vessels
should be avoided in children acutely infected with Shigatoxin Escherichia coli.
become damaged. Microvascular endothelial
cell injury is central to the renal thrombotic
microangiopathy that is characteristic of
DHUS occurs in epidemics as well as
Introduction HUS. Thrombocytopenia results from intrare-
sporadically. Outbreaks can be traced to con-
nal platelet adhesion and platelet survival is
Definition taminated food, especially undercooked
shortened. Renal dysfunction results from this
Haemolytic uraemic syndrome (HUS) is the hamburger and contaminated water.
process.
commonest cause of acute renal failure in There are over 100 different serotypes
infants and young children <5 years of age of STEC with different phage typing and
in developed countries. There is multisystem subtyping. STEC O157:H7 is the common-
History
involvement in this syndrome, which is char- est subtype producing disease in North
acterised by sudden onset of non-immune America, the British Isles and Japan. It is rare A child who has previously been healthy pre-
microangiopathic haemolytic anaemia, throm- as a causative agent of HUS in Australia, sents with a history of gastroenteritis within
bocytopenia and acute renal failure. Various where other types, including O111:H, are the previous 2 weeks. There is subsequent
degrees of severity occur. One-third of pathogenic. E. coli can be spread by person development of vomiting, bloody diarrhoea
patients are anuric at presentation. to person contact. The incubation period to and crampy abdominal pain. The child is usu-
An inherited form, which is transmitted in onset of diarrhoea is 18 days. ally afebrile at presentation. The presenta-
an autosomal dominant manner, may pres- Non-diarrhoeal-associated HUS (DHUS) tion may resemble an acute surgical
ent in the newborn period. cases account for 10% of cases. These are abdomen. The child then becomes pale and
secondary to: develops haematuria and oliguria and is
noted to become lethargic. Not uncommonly,
Epidemiology non-enteric infections, e.g. streptococcal the patient appears to be improving from
The commonest form of HUS in children is pneumonia;
his/her gastroenteritis but then deteriorates
the one that occurs following a prodromal ill- immunosuppression: malignancy- suddenly, becoming quite ill.
ness of acute gastroenteritis with bloody associated, following bone-marrow
diarrhoea. This diarrhoea-associated haemo- transplant, and drug-related, e.g.
lytic uraemic syndrome (DHUS) accounts mitomycin, ciclosporin and FK-506
for 90% of cases. DHUS is most often
Examination
(tacrolimus);
caused by Shiga toxin (verotoxin) producing immunodeficiency and pregnancy may The child appears pale, lethargic and dehy-
Escherichia coli (STEC, also called VTEC). also be associated with HUS. drated. Petechiae may be noted on the skin,
Other cytotoxin-producing bacteria such as which may also appear jaundiced.
Shigella dysenteriae type 1, Salmonella typhi Most patients have some degree of
and Campylobacter are less common causes Pathophysiology encephalopathy. Central nervous system
of HUS. Streptococcus pneumoniae also The primary process in the pathogenesis of involvement can range from irritability and
causes HUS but does so via a completely dif- HUS is vascular endothelial cell injury. E. coli restlessness to seizures or coma. Seizures
ferent mechanism, which involves neuramin- is ingested and colonises the gut. These bac- can be present in up to 40%. Hemiparesis
idase rather than cytotoxin production. teria attach to the epithelium of the distal and cranial nerve dysfunction may be seen.

372
16.5 HAEMOLYTIC URAEMIC SYNDROME
16

RENAL
Hypertension is present in up to 50% and dysmorphic. There can be evidence of No added potassium is required unless
is likely secondary to increased renin levels. proteinuria (mild to severe) and serum levels are below normal values.
Evidence of cardiac involvement may present leucocytes. Granular and hyaline casts Hyperkalaemia must be anticipated and
as myocarditis, cardiomyopathy or high- may be present. treated in a timely fashion (refer to manage-
output failure. Oliguria occurs often. Hepato- ment of hyperkalaemia in Chapter 10.5 on
Renal biopsy is rarely indicated in children
megaly and oedema develop (reflecting fluid electrolytes).
who have the characteristic features of HUS.
overload) if the oliguria is not recognised and Anaemia should be treated with packed
fluid intake is not restricted. Splenomegaly is red blood cell transfusion (10 mL kg1) when
not as common as hepatomegaly. anaemia is severe, the patient is symptom-
Differential diagnosis atic or the haematocrit is falling rapidly.
In general, an attempt is made to maintain
Investigations Intussusception. Hb > 70 g L1 when possible.
Disseminated intravascular coagulation Platelet transfusion is avoided because of
Diagnosis of HUS is based on the presence of (DIC) in patients with sepsis and multi-
precipitous worsening of the patients clini-
microangiopathic haemolytic anaemia, organ failure.
cal status. This results from the aggregation
thrombocytopenia and renal failure. Idiopathic thrombocytopenic purpura
of platelets, which are a major constituent of
(ITP).
Anaemia is usually severe and is microthrombi and thus induce further dam-
normochromic normocytic in type with a
Systemic lupus erythematosus (SLE) with
age. Newly formed platelets in HUS function
nephropathy.
haemoglobin level of 5090 g L1. very well and platelet transfusion is not
Leukaemia.
White cell count is non-specific and often required, even prior to the surgical insertion
raised with increased number of
Toxic syndromes and encephalitis may
of a central venous catheter or peritoneal
have similar presentation.
immature forms. dialysis catheter.
Post-infectious glomerulonephritis.
Platelet count is low in 95% and can be Hypertension responds well to treatment
as low as 20  109 L1. Subsequent Findings on blood film, coagulation profile, with short-acting calcium channel blockers,
increase in platelet count may be the first urinalysis, stool and blood cultures assist in e.g. nifedipine. Intravenous nitroglycerine
indication of resolution of the differentiating all these conditions from can be used if oral medication is not toler-
microangiopathic process. HUS. There is no evidence of haemolysis in ated. Nitroprusside is not favoured because
Reticulocyte count is mildly elevated. intussusception. The complete blood count of the danger of cyanide poisoning in renal
Evidence of schistocytes, burr cells and in leukaemia would reveal low haemoglobin, failure. Labetalol by intravenous bolus or
helmet cells on blood film. thrombocytopenia and a white cell count continuous infusion can also be used to
Coombs test is negative indicating that was depressed or markedly elevated manage hypertension (see Chapter 16.3).
anaemia is not immunologically with the presence of blasts. Treatment of hypertension can prevent
mediated. Patients with DIC have sepsis and prolon- development of encephalopathy and con-
Coagulation tests are usually normal but gation of the prothrombin time and partial gestive heart failure.
may show mild disseminated thromboplastin time. ITP has isolated throm- Seizures should be treated with short-
intravascular coagulation. bocytopenia with a normal coagulation acting benzodiazepines initially, followed
Serum haptoglobin is low, lactic profile. by intravenous infusion of phenytoin or
dehydrogenase (LDH) is elevated along phenobarbital.
with indirect bilirubin level all There is some evidence that early dialysis
indicators of intravascular haemolysis. may improve outcome in HUS. Dialysis should
Treatment
Elevated serum blood urea, creatinine be commenced in the following situations:
and phosphate reflect renal involvement, There is no specific therapy for Shiga toxin-
occurring at the same time as the urine producing E. coli and prevention of the dis-
severe hyperkalaemia uncontrollable by
medical means;
output is declining and the haemolysis ease is therefore of utmost importance.
developing. Supportive therapy may include dialysis,
fluid overload and pulmonary oedema;
significant uraemic symptoms;
High triglycerides. antihypertensive therapy, blood transfusions
blood urea >36 mmol L1 even if
High lipase/amylase reflecting and management of neurological complica-
electrolyte and water balances are
pancreatic involvement. tions. With supportive therapy, 85% of chil-
satisfactory;
Low albumin even without nephrosis. dren recover renal function.
anuria.
Electrolyte derangement may result from The cornerstone of treatment is careful
the renal abnormalities, including attention to electrolyte and fluid balance. Peritoneal dialysis is generally used in
hyponatraemia, hyperkalaemia, Once intravascular volume has been infants and preschool-age children unless
hypocalcaemia and metabolic acidosis restored, the amount and type of fluid admi- there is evidence of severe colitis or abdomi-
(which can be severe). nistered should be limited to ongoing losses, nal tenderness is present. This management
Urinalysis shows haematuria that can be i.e. insensible loss, urine output and gastro- strategy is dependent on the resources and
gross and the red blood cells are intestinal losses. preferences of the managing team/unit.

373
16.5 HAEMOLYTIC URAEMIC SYNDROME

There are no controlled clinical trials long-term studies have shown late complica-
E. coli have been undertaken in a
demonstrating efficacy of antibiotic therapy tions developing in patients who had fully
preclinical study.
on the prevention and amelioration of HUS. recovered initially. These complications
In fact, the use of antibiotics should be avoided include hypertension, proteinuria, reduced  An oligovalent water-soluble
in children infected with Shiga toxin E. coli. glomerular filtration rate and late develop- carbohydrate pentamer with five pairs
There are no controlled randomised ment of end-stage renal disease (ESRD). ESRD of trisaccharide ligands linked to a
studies on plasmapheresis as treatment for was shown to develop in 1015% as late as central dendrimer, and called Starfish,
DHUS. Plasmapheresis may be beneficial 1525 years following recovery. neutralises all five B subunits of Shiga
in patients with drug-induced HUS. Vincris- The frequency of complications is higher toxin and protects human cells from
tine and ciclosporin A are known to cause in DHUS patients. Shiga toxin in culture.
drug-induced HUS. HUS can recur in transplanted kidneys
Administration of intravenous
Nutritional requirements must be regardless of the aetiological agent. Recur-
g-globulin IgG and gabexate mesilate,
addressed aggressively, as these patients rence of disease following renal transplant
a synthetic serine protease inhibitor
are catabolic and hypoalbuminaemic. is infrequent in DHUS in comparison with
used as an anticoagulant resulted in
Enteral feeds can be commenced once the DHUS.
amelioration of disease in five children
diarrhoea has settled. Total parenteral nutri- Occasionally, children can be left with
with DHUS. The IgG itself has
tion is required in some cases. chronic sequelae due to HUS, which are not
no proven benefit on the course of
No evidence exists for the use of aspirin, renal related. Almost any organ system can
DHUS.
heparin, warfarin, streptokinase, urokinase, be involved. Stroke is the most serious sequela
vitamin E or immunoglobulin G in the treat- and occurs in 35% of patients. Colitis can The use of angiotensin-converting
ment of HUS. result in bowel infarction, leading to colos- enzyme inhibitors in conjunction with
tomy/colectomy or stricture formation. controlled intake of protein (to
Pancreatitis can develop, sometimes recommended daily allowance) may
Prognosis with diabetes, and hepatic involvement help preserve renal function in
Overall prognosis for DHUS is better than can occur. children with persistent proteinuria
that for DHUS. However, long-term progno- during the chronic stages of Shiga
sis for those DHUS who recover, but toxin HUS.
have persistent proteinuria 1 year following Prevention New DNA probes to detect Shiga
recovery, is guarded. There is an early mortal- toxin in stool have been developed but
DHUS is an infectious disease and the
ity rate of about 5%. Another 5% require are not yet clinically available.
most effective prevention strategy would
lifelong dialysis because of the development
be to prevent ingestion of the E. coli. Avoid-
of chronic renal failure and anuria.
ance of undercooked meat can assist in this
The improved prognosis has resulted from
area. There are, however, other vectors for
careful correction of electrolyte abnormal-
the transmission of the E. coli and these Further reading
ities, judicious fluid management and earlier Corrigan Jr JJ, Boineau F. Hemolytic uremic syndrome. Pediatr
include contaminated water and beverages. Rev 2001;22:3658.
institution of dialysis.
Food handlers, vendors and consumers must Cronan K, Norman M. Renal and electrolyte emergencies. In:
The risk of renal sequelae in children is Fleisher GR, Ludwig S, editors. Textbook of pediatric
be made aware of proper food-handling emergency medicine. 4th ed. Philadelphia: Lippincott,
higher in males, and those with hyperten-
techniques. Williams & Wilkins; 2000. p. 8478. Chapter 86.
sion, prolonged anuria and haemoglobin Elliott EJ, Robins-Browne RM, OLoughlin EV, et al. Nationwide
<100 g L1 at onset of disease. study of haemolytic uraemic syndrome: Clinical,
microbiological, and epidemiological features. Arch Dis
Other poor prognostic indicators include: Child 2001;85:12531.
Controversies and future Garg AX, Suri RS, Barrowman N, et al. Long-term renal
elevated white cell count (>20  109 L1) directions prognosis of diarrhoea-associated hemolytic uremic
syndrome: a systematic review, meta-analysis and meta-
at presentation;
In one study, a strategy was
regression. JAMA 2003;290(10):136070.
elevated white cell count at presentation, Kaplan B, Meyers K. The pathogenesis of hemolytic uremic
developed in which a recombinant E. coli syndrome. J Am Soc Nephrol 1998;9:112633.
which remains elevated; Ray P, Liu X. Pathogenesis of Shiga toxin-induced hemolytic
age < 1 year or > 5 years; was generated that had on its surface a uremic syndrome. Pediatr Nephrol 2001;16:82339.
lipopolysaccharide (LPS), which Ring GH, Lakkis FG, Badr KF. Microvascular diseases of the
central nervous system involvement kidney. In: Brenner B, editor. The kidney. 6th ed. vol. 2.
(seizures, coma, etc.); mimicked the Shiga toxin receptor. These Philadelphia: WB Saunders; 2000. p. 1597603.
recombinant E. coli bind to the Shiga Chapter 35.
the degree and duration of renal Siegler RL. The hemolytic uremic syndrome. Pediatr Clin N Am
dysfunction. toxin thus preventing the binding of 1995;42(6):150522.
Shiga toxin to target cells. Stewart CL, Tina LU. Hemolytic uremic syndrome. Pediatr Rev
1993;14(6):21824.
Strategies for immunisation have Tarr PI, Gordon CA, Chandler WL. Shiga-toxin-producing
Complications been explored. The first steps in
Escherichia coli and haemolytic uraemic syndrome. Lancet
2005;365(9464):107386.
Trachtman H, Christen E. Pathogenesis, treatment and
Children with DHUS should be followed for evaluation of development of a vaccine therapeutic trials in hemolytic uremic syndrome. Curr Opin
many years following full recovery as some against Shiga-toxin-producing strains of Pediatr 1999;11:1628.

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16.6 Nephrotic syndrome
Karen McCarthy

Aetiology
ESSENTIALS The cause of primary nephrotic syndrome is
unknown and hence is referred to as idio-
1 Nephrotic syndrome refers to the clinical findings of hypoalbuminaemia, heavy pathic. The commonest form is minimal
proteinuria, oedema and hyperlipidaemia. Every child has albuminaemia but not all change disease (85%). Renal biopsy find-
features are necessary for diagnosis. ings in this form show no abnormality of
2 Despite the presence of oedema, shock still needs to be treated with 20 mL kg1 the glomeruli on light microscopy. Electron
0.9% saline or albumin boluses. microscopy shows effacement of the epithe-
lial foot processes. Biopsy findings of FSGS
3 Renal function is usually normal though serum creatinine may be slightly elevated (10%) reveal normal appearing glomeruli
at presentation. but may show some mesangial proliferation.
4 There is 12% mortality despite good long-term prognosis. Some biopsy specimens may demonstrate
segmental scarring. Mesangial proliferative
5 The physical findings of peritonitis in nephrotic syndrome may be very subtle, thus disease (5%) shows diffuse increase in
the emergency physician must have a very high index of suspicion for peritonitis. mesangial cells and matrix on biopsy.
6 Death from peritonitis and thrombosis can occur despite very good care.
7 All children with nephritic syndrome should receive pneumococcal vaccine.
Pathophysiology
8 All non-immune children on steroid therapy who are exposed to varicella require
prompt administration of zoster immune globulin. Should they develop lesions, they An immune basis for MCNS was proposed in
require treatment with intravenous aciclovir. 1974 but no primary immune abnormality
has been identified. Studies have shown
increased levels of circulating soluble
interleukin-2 receptor in patients with active
24 hours), generalised oedema and hyperlipi- MCNS and FSGS. This soluble interleukin-
Introduction daemia (triglycerides and cholesterol). 2 receptor is shed by activated lymphocytes
Definition Primary nephrotic syndrome (idiopathic) and may therefore indicate generalised acti-
Nephrotic syndrome (NS) refers to the find- represents disease limited to the kidney. Dis- vation of the immune system. However, this
ings of heavy proteinuria, hypoalbuminae- ease is classified by responsiveness to steroid circulating soluble interleukin-2 receptor
mia, oedema and hyperlipidaemia, which therapy (steroid sensitive, steroid dependent could serve as a neutraliser of circulating
result from a massive loss of protein in the or steroid resistant) and histology on renal interleukin-2. It is unknown whether circu-
urine secondary to glomerular disease. All biopsy (minimal change, mesangial prolifera- lating levels represent evidence of activa-
components of the disease do not need to tive glomerulonephritis (GN) and focal seg- tion or an attempt to down-regulate the
be present for diagnosis. The protein lost mental glomerulosclerosis, FSGS). immune responses.
in the urine includes plasma proteins of Secondary nephrotic syndrome represents There is hypercoagulability resulting from
molecular weight up to and including albu- multisystem disease that has kidney involve- serum protein abnormalities introduced by
min. Generalised glomerular leak to macro- ment, e.g. lupus nephritis, hereditary nephri- renal protein wasting. All children with
molecules does not occur in most cases, tis and HenochSchonlein purpura. acute NS have increased platelet aggrega-
but can occur in severe disease. In severe Certain drugs and chemicals can cause tion. Blood viscosity is increased and blood
disease, as there is progressive loss of glo- nephrotic syndrome, e.g. phenytoin, non- flow is reduced. Fibrinogen concentration
merular permselectivity, renal clearance of steroidal anti-inflammatory drugs, and is increased and antithrombin III is lost in
IgG approaches that of albumin. Protein captopril. the urine. All these factors contribute to this
selectivity is seen mainly in minimal change problem in NS.
nephrotic syndrome. The determination of Epidemiology The cause of cholesterolaemia and hyper-
protein selectivity has little clinical value Minimal change nephrotic syndrome triglyceridaemia remains uncertain. One
but does increase the likelihood of response (MCNS) is more common in males. Mean theory is that reduced plasma oncotic pres-
to steroid therapy. age of onset is <6 years for primary ne- sure may stimulate lipoprotein synthesis
The hallmarks of nephrotic syndrome are phrotic syndrome, and >6 years for second- and perhaps lipolytic factors may be lost in
hypoalbuminaemia (serum albumin <30 g ary. It is usually sporadic. A congenital the urine.
L1), heavy proteinuria (>50 mg kg1 body form, inherited in an autosomal recessive Oedema reflects retention of salt and
weight per 24 hours, or >1000 mg m2 per manner, presents in the newborn period. water. Reduced serum albumin causes a

375
16.6 NEPHROTIC SYNDROME

reduction in plasma oncotic pressure. When protein to urine creatinine. Spot urine
plasma oncotic pressure falls, intravascular protein:creatinine ratio is Treatment
volume is reduced, which stimulates proxi- >300 mg nmol1 in nephrotic syndrome.. It can be difficult to assess volume status
mal tubular reabsorption of sodium. The Normal is <0.2 mg protein per mg in patients with NS. The patient may be in
reninangiotensin system is also stimulated, creatinine (or protein per mmol shock despite the presence of oedema.
raising the serum aldosterone, which further creatinine). Attention to detail on clinical
increases distal tubular reabsorption of Microscopic haematuria is seen in < 15% examination can assist with the
sodium. There is an increase in filtration but macroscopic haematuria is rare. assessment. Clues to hypovolaemia are:
capacity in patients with nephrotic syn- Urinalysis by dipstick reveals 3 or 4 tachycardia; peripheral vasoconstriction
drome along with an increase in glomerular proteinuria. with poor capillary refill time; and
permeability, resulting in proteinuria. Urine specific gravity is usually high at orthostatic hypotension, i.e. positive tilt
There is also evidence in some patients >1.020, but may be lower. test. Other clues to volume status include
for primary salt and water retention by the Haematocrit is often elevated due to a history of oliguria and evidence of renal
kidney. intravascular depletion and can be insufficiency on laboratory investigations.
followed to assess fluid status. Albumin infusions transiently increase
Hyperlipidaemia is present with elevated plasma volume and are most useful in
History serum cholesterol and triglyceride levels. patients with profound volume depletion.
The presence of the following may be noted Serum sodium can be low due to Plasma volume is increased using 4%
on history: decreased free water excretion but is albumin rather than 0.9% saline. It is
rarely symptomatic and does not require important to note that this is only a
recent flu-like illness; treatment. Total body sodium is high. temporising measure. The infused
anorexia; Hypocalcaemia is a common finding and albumin is rapidly lost in the urine,
abdominal pain; reflects the low serum albumin. The especially when the original albumin
diarrhoea; ionised calcium is usually normal, however, level is very low because of high renal
recent ant bites or bee stings. and the hypocalcaemia is asymptomatic. albumin clearance (rate of loss is
Plasma urea and creatinine are elevated proportional to albumin clearance and
in approximately 25% of patients. serum albumin level). The patient can
Examination
Serum complement levels are normal in develop acute pulmonary oedema
Periorbital oedema is noted initially, but in MCNS, FSGS and mesangial proliferative requiring admission to an intensive-care
time oedema becomes generalised. Oedema glomerulonephritis. unit.
of the lower extremities is usually pitting in In a patient symptomatic from massive
Steroids can be used as a diagnostic tool in
type and accumulates in dependent sites. oedema, a trial of diuretics may be given,
the management of NS. The patient is said
The rate and degree of oedema formation is provided the patient is not hypovolaemic,
to be steroid responsive when the urine
directly related to salt intake and to the degree and only after discussion with a
becomes free of protein. If the child con-
of hypoalbuminaemia and therefore can vary nephrologist. Diuretics may induce
tinues to have proteinuria (>2 ) after
from child to child. Ascites may be present. hypotension, severe volume depletion,
48 weeks of compliance with continuous
Pleural effusions or pulmonary oedema may ARF and hyperkalaemia if there is already
daily prednisone, the nephrosis is termed ste-
develop. Scrotal or perineal oedema may be incipient hypovolaemia. An infusion of
roid resistant and renal biopsy is indicated
very distressing to the patient. Blood pressure 20% salt-deficient albumin 0.51 g kg1,
to determine the precise cause, e.g. FSGS.
may be decreased when hypovolaemia exists followed 30 minutes later by furosemide
Renal biopsy is not indicated during the
or may be elevated if there is significant renal 0.51 mg kg1 intravenously can be
initial episode of acute nephrotic syndrome.
disease by activation of the reninangiotensin effective. In occasional circumstances,
The need for, and timing of a renal biopsy is
system or primary salt retention. ultrafiltration or haemofiltration may be
determined by the subsequent disease
course, e.g. poor or no response of initial epi- used to manage fluid overload.

Investigations sode to steroid therapy after 48 weeks of The natriuretic effect of furosemide is
treatment or if other signs or symptoms thought to be diminished in NS.
Hypoalbuminaemia (serum albumin are present that indicate a systemic disease. Increasing the dose or using furosemide
<30 g L1) occurs in every child with in combination with a diuretic acting at
nephrotic syndrome. another site, such as a thiazide diuretic
Reliably timed 24-hour urine collections (hydrochlorothiazide 1 mg kg1 day1 in
are difficult to obtain in children (protein
Differential diagnosis two divided doses) is recommended to
excretion exceeds 50 mg kg1 per 24 Other causes of oedema need to be excluded: overcome the hyporesponsiveness.
hours or 1000 mg m2 per 24 hours in congestive heart failure; starvation; protein- Metolazone (diuretic activity on the distal
nephrotic syndrome), so the proteinuria is losing enteropathy; cystic fibrosis; hypothy- tubule) is a powerful diuretic and is
generally quantified by the ratio of urine roidism; vasculitis; and steroid therapy. occasionally employed but must be used

376
16.6 NEPHROTIC SYNDROME
16

RENAL
with caution. If serum albumin is agents cyclophosphamide or
<15 g L1, diuretic therapy is ineffective. chlorambucil can produce prolonged and Disposition
Oral fluids, when offered, should be given sometimes permanent remission and are Outpatient management is appropriate for
in small aliquots, especially in considered to be steroid-sparing. These children with the first presentation of NS, if
hyponatraemic patients, in whom any alkylating agents are not commonly the oedema is not massive and there is no sus-
free water will tend to lower the plasma needed in patients with MCNS but when picion of infection or life-threatening compli-
sodium further. needed they are very effective. Some cations and the physician can be assured of
Therapeutic paracentesis may be patients with MCNS may not respond close follow up. The rest are best managed
indicated for acute respiratory distress to the alkylating agents and will respond as inpatients to permit further work up and
but is rarely necessary. Respiratory to ciclosporin (<5.5 mg kg1 per 24 treatment and to provide education and sup-
distress is due to reduced lung capacity hours or <200 mg m2 per 24 hours) if port for the patient and family.
secondary to the increased abdominal there is steroid responsiveness. Admission is warranted in a child with
pressure. Paracentesis should only be Dependence on ciclosporin is common, known history of nephrotic syndrome if there
used when there is no other way to the effect lasting only as long as are signs of severe dehydration, unexplained
support respiration, because of the treatment continues. Some nephrologists fever, renal insufficiency (i.e. elevation in
association of complications with the consider the limited risk of ciclosporin serum creatinine), refractory oedema (e.g.
procedure, e.g. infection and the re- nephrotoxicity in this population to be respiratory distress) or suspected peritonitis.
accumulation of the fluid. preferable to the side effects of chronic Discuss admission of frequent relapsers, or
Prednisolone is started at 2 mg kg1 steroid toxicity or the risk of sterility from > 1st relapse, with the nephrologist.
day1 (60 mg m2 day1) in two divided a second alkylating agent when one
doses. A divided daily dose is used in course of an alkylating agent is
preference to a single dose as some insufficient therapy.
Prognosis
children who fail to respond to a single In FSGS ciclosporin is more effective in
daily dose respond to divided doses. steroid-dependent rather than in steroid- Long-term prognosis is good for children
Disappearance of proteinuria is seen resistant disease and may even be more with steroid-responsive primary nephrotic syn-
in 90% within 46 weeks. nephrotoxic in this latter group. drome, though 12% mortality is associated.
If the child is unwell or peritonitis is Whilst on steroid therapy, non-immune Most deaths are due to peritonitis or sepsis
suspected, antibiotics are commenced children exposed to chickenpox require (pneumonia), and thrombus, which may occur
after appropriate cultures are taken. prompt administration of zoster immune despite good care. Relapses may recur for many
Prophylactic antibiotics are generally globulin. years, often until puberty, with a small number
recommended for all patients whilst Immunisations: Live-virus vaccines continuing to relapse through to adulthood.
nephrotic and on high-dose steroids. should not be administered to children These relapses are not usually severe and
Some units advocate routine use of low- on high-dose corticosteroid therapy. can be treated with short courses of oral ster-
dose aspirin, though this is not commonly Children receiving <2 mg kg1 per 24 oids. Though these children with steroid-
done. hours of prednisolone or its equivalent, responsive disease may require steroid therapy
Angiotensin-converting enzyme (ACE) can be immunised. Those children intermittently throughout childhood, serious
inhibitors are good antihypertensive receiving >2 mg kg1 per 24 hours daily sequelae of steroid toxicity are uncommon.
drugs in those patients who have or alternate daily prednisolone or its When in remission, the child should have
hypertension from their primary disease equivalent for more than 14 days, should unrestricted diet and activity.
or as a result of steroid therapy. ACE have live-virus vaccine administration Recovery is considered permanent if the
inhibitors are useful as most of the deferred for at least 1 month after child remains asymptomatic and has no
hypertension is due to activation of the discontinuation of the steroids. Resonse requirement for medications for more than
reninangiotensin system. If the to treatment see Table 16.6.1. 2 years.
hypertension is a result of steroid therapy,
the patient may respond better to a b-
blocker. Table 16.6.1 Definitions of response to treatment

Some children with MCNS who are Remission Urine protein excretion <4 mg hr1 m2 or urine protein negative/trace for
3 consecutive days
steroid-responsive either relapse
frequently or require a high daily dose of Relapse Urine protein on Dipstix 2 for 3 consecutive days having previously been in
remission. This occurs in up to 75% of patients
steroids to maintain remission. These
children are at risk of development of Frequent relapse 2 more relapses within 6 months or >4 relapses in 12 months
serious side effects from the steroids, Steroid dependence 2 consecutive relapses occurring during steroid treatment or within 14 days of
including growth failure, hypertension, its cessation
osteoporotic bone disease and posterior Steroid resistance Failure to achieve response after 28 days of steroid at 60 mg m2 day1
sublenticular cataracts. The alkylating

377
16.6 NEPHROTIC SYNDROME

Thromboembolism from hypercoagula- Growth can be impaired in nephrotic syn-

Complications bility is the second major contributor to mor- drome. Insulin-like growth factor (IGF-1)-
As MCNS is a self-limited disease in most bidity and mortality. The thrombus is usually binding protein is lost in the urine, which
children, a distinction must be made venous and is located in the deep vessels may account for the reduced serum levels
between the complications of the disease of the extremities, renal vein, pulmonary of IGF-1 and IGF-2. Growth impairment is
itself and those that are related to the treat- venous system and cerebral cortical system. also a consequence of steroid therapy.
ment. Major complications of the disease For this reason, it is recommended that fem-
oral or deep venepuncture is avoided if pos-
itself are infection, dislipidaemia and throm- Prevention
bosis. Treatment with steroids may increase sible in children with nephrotic syndrome.
The risk of thromboembolus is real and As the primary causes of idiopathic ne-
body mass index and impair growth.
exists even in the first acute presentation phrotic syndrome are unknown, there is
Acute complications occur in two groups
with NS. This is due to increased platelet no means of prevention.
of patients:
aggregation, and in part also due to the uri- There is some evidence supporting a role
Those who present de novo or in relapse nary loss of several proteins that inhibit of the immune system in paediatric minimal
but not taking steroids. blood coagulation, especially antithrombin- change disease. Relapses can be triggered
Those who present in relapse or III, protein C and protein S. Increased blood by minor infections.
remission while still receiving viscosity and reduced blood flow also play Vaccination against varicella, influenza
pharmacological doses of steroids. a role. Renal vein thrombosis should be and pneumococcus would seem prudent,
suspected if flank pain, haematuria and given the relapsing nature of nephrotic syn-
The tendency to develop infections is a true
decreased renal function occur. Occlusion drome. Some susceptibility to infection may
complication of the disease.
of the arterial system is uncommon. result from loss of opsonising factors in the
urine so even if the child has been immu-
Hypogammaglobulinaemia Is a factor nised, vigilance is warranted against the
Hyperlipidaemia associated with NS possibility of any bacterial infection.
in the reduced defences against bacterial
rarely gives rise to clinical complications as
infections. IgG is the immunoglobulin most
it usually reverses with steroid therapy. It
severely depleted. This may be a conse- Controversies and future
may become problematic in patients with
quence of it being the smallest of the immu-
chronic NS. directions
noglobulins and the one with the greatest
renal excretion. Cyclo-oxygenase inhibitors are a class of
Symptomatic hypovolaemia Despite drugs that may be of assistance in
the presence of oedema, symptomatic hypo- reducing proteinuria in patients with NS,
Bacterial infections occur in both volaemia leading to shock can develop if although, unlike ACE inhibitors, they
groups but are more common in the ste- fluids are restricted injudiciously with or generally cause a reduction in GFR.
roid-treated group without diuretic therapy

Spontaneous bacterial peritonitis is a Respiratory compromise can rarely be Further reading

caused by massive ascites, warranting emer- Bergstein J. Conditions particularly associated with
major source of morbidity in children with proteinuria. In: Behrman R, Kliegman R, Jenson H, editors.
NS. Main causative organisms are Strepto- gency paracentesis in some cases Nelsons textbook of pediatrics. 16th ed. Philadelphia: WB
Saunders; 2000. p. 15926.
coccus pneumoniae and Escherichia coli. Cronan K, Norman M. Renal and electrolyte emergencies. In:
Diagnosis of peritonitis should always be Fleisher G, Ludwig S, editors. Textbook of pediatric
Hypertensive encephalopathy Acute emergency medicine. 4th ed. Philadelphia: Lippincott
considered in a child with nephrotic syn-
increase in blood pressure may occur in Williams & Wilkins; 2000. p. 8325.
drome who complains of abdominal pain. Falk RJ, Charles Jennette J, Nachman PH. Nephrotic syndrome.
steroid-treated children at any stage of In: Brenner B, editor. The kidney. 6th ed. vol. 2.
The physical findings of peritonitis in NS
their illness. The diagnosis of hypertensive Philadelphia: WB Saunders; 2000. p. 126683.
may be very subtle. The patient does not Gipson DS, Massengill SF, Yaol L, et al. Management of
encephalopathy is based on the degree of childhood onset nephrotic syndrome. Pediatrics
necessarily have a rigid abdomen. The phy-
change in the blood pressure and rate 2009;124:74757.
sician must have a high index of suspicion Grimbert P, Audard V, Remy P, et al. Recent approaches to the
of increase rather than on a specific level pathogenesis of minimal-change nephrotic syndrome.
when assessing these patients. Steroid ther-
of systolic and/or diastolic blood pressure. Nephrol Dial Transpl 2003;18:2458.
apy, especially high dose (2 mg kg1 day1 Kaysen GA. Proteinuria and the nephrotic syndrome.
In: Schrier RW, editor. Renal and electrolyte disorders.
of prednisolone) can mask the typical signs 6th ed. Philadelphia: Lippincott, Williams & Wilkins; 2003.
and symptoms of infection. Children with Benign intracranial hypertension can p. 580614.
Roth KS, Amaker BH, Chan JCM. Nephrotic syndrome:
NS can develop other infections such as be precipitated by abrupt reduction in Pathogenesis and management. Pediatr Rev 2002;23:
pneumonia, cellulitis and sepsis. steroid doses 23747.

378
 16

RENAL
16.7 HenochSchonlein purpura
Karen McCarthy

abdominal pain, it is extremely uncommon

ESSENTIALS for nephritis to precede the appearance of
the rash. The onset of nephritis may in fact
1 There is no diagnostic laboratory test for the diagnosis of Henoch-Schonlein be delayed for weeks or months after the
purpura.
onset of other symptoms. Approximately
2 The diagnosis is clinical and consists of the tetrad: palpable purpura in patients 80% of patients manifest nephritis within
with neither thrombocytopenia or coagulopathy, arthritis/arthralgia, abdominal pain 4 weeks and 95% within 3 months of the
and renal disease. onset of other symptoms.

3 Urinalysis, and blood pressure should be measured at presentation.

4 Clinical hallmark of HenochSchonlein nephritis is haematuria.
Examination
5 Onset of nephritis may be delayed for weeks or months after onset of other
symptoms. The clinical features of HSP may be atypical
in the extremes of age. The clinical manifes-
6 All patients with HenochSchonlein nephritis need follow up for 6 months even if tations can develop over days or weeks. The
urinalysis is normal at presentation.
characteristic rash consists of crops of palpa-
7 There is no need for follow up beyond the first 6 months if urinalysis remains ble purpura and is symmetric in distribution
normal. and located on the extensor surfaces of the
arms, legs and buttocks. It is worth noting
8 Recurrence of HenochSchonlein purpura is common and is normally observed that the rash is not restricted to these areas.
within 4 months of resolution of original symptoms.
The rash may begin with urticaria that pro-
gresses over 24 hours to palpable purpura.
The arthritis involves the knees, ankles or
The reported incidence of nephritis is feet and may result in refusal to weight bear.
Introduction 20-50% but may in fact be higher. Renal Periarticular swelling and tenderness are
involvement may manifest with haematuria often noted.
HenochSchonlein purpura (HSP) is the
 proteinuria and occasionally with nephritic Gastrointestinal symptoms of nausea,
most common acute vasculitis affecting chil-
or nephrotic syndrome or with renal failure vomiting and abdominal pain occur in
dren. It has a prominent cutaneous compo-
associated with rapidly progressive glomer- approximately 50%. These symptoms are
nent. It is a leukocytoclastic vasculitis that
ulonephritis. Risk factors for renal pro- caused by submucosal haemorrhage and
affects small vessels (arterioles and venules).
gression are nephrosis, hypertension, renal oedema. Asymptomatic microscopic haema-
The aetiology is unknown. The disease is
impairment at presentation, and haematuria. turia occurs in most but up to 40% have
more common in males, with mean age of
gross haematuria. Proteinuria occurs in con-
occurrence 47 years. Infants tend to have
junction with haematuria in two-thirds of
milder disease and those less than 2 years
patients but the occurrence of proteinuria
of age are less likely to have nephritis and
History alone is rare.
abdominal pain in comparison with older
children. With the exception of nephritis, it There is usually a history of antecedent
is generally an acute self-limited illness last- upper respiratory tract infection. The charac-
ing from several days to several weeks. teristic rash is the most frequent presenta-
The characteristic histological finding on tion. Arthritis is the second most frequent
Investigations
skin biopsy is neutrophil infiltration in and manifestation, occurring in approximately Diagnosis of HSP is straightforward when
around dermal vessels. Histological changes 80% and though it may be incapacitating, the typical rash is present.
in the kidney range from minimal change to it is self-limiting and non-deforming. Gastro- Diagnosis of HSP requires a normal
focal or diffuse mesangial proliferation that intestinal involvement occurs in 5075% of platelet count by definition.
may be accompanied by glomerular crescent patients and is potentially the most serious There is no diagnostic laboratory test for
formation. The characteristic finding on complication of HSP due to the possible HSP routine laboratory tests are neither
immunofluorescence is diffuse mesangial development of gastrointestinal bleeding specific nor diagnostic.
deposition of immunoglobulin A (IgA) and and intussusception. Urinalysis should be performed to
complement 3 (C3). IgA plays a central role The clinical hallmark of HSP nephritis establish the presence of microscopic
in the pathogenesis of HSP. is haematuria. In contrast to arthritis and haematuria, with or without proteinuria.

379
16.7 HENOCHSCHONLEIN PURPURA

Serum creatinine should be checked when immunosuppressive agent, either azathio- in recipients of living related-donor kidneys
HSP nephritis is present. prine or cyclophosphamide. than of cadaveric kidneys. It is recommended
Blood pressure should be measured and Plasmapheresis has been reported to be that transplantation is delayed for 1224
recorded at presentation for baseline of benefit in a few patients with rapidly pro- months following disappearance of the pur-
evaluation. gressive HSP glomerulonephritis i.e. severe puric lesions.
disease.
Hypertension angiotensin blockade by
use of angiotensin-converting enzyme Complications
Differential diagnosis (ACE) inhibitors like enalapril may decrease
proteinuria and result in slower deteriora-
Acute renal insufficiency with elevation
The differential diagnosis of a purpuric rash of serum creatinine can develop in
is broad. Purpura may be the initial manifes- tion in renal function as well as providing
patients with HSP nephritis.
tation of an infectious process. effective control of blood pressure.
Patients can also develop nephrotic
syndrome. In this situation the urine
Meningococcaemia Palpable purpura protein excretion is >50 mg kg1 per
(as opposed to the usual non-palpable pur- Disposition 24 hours, with oedema and
pura) can occur if there are pre-existing coag- hypoalbuminaemia.
Outpatient management is appropriate for
ulation abnormalities (such as protein S or
most patients with HSP.
End-stage renal disease (ESRD) is a
protein C deficiency). This diagnosis must possible complication of HSP nephritis.
Inpatient management may be required
be considered in any child with purpura.
for management of abdominal pain and
Orchitis is a well-described complication
of HSP and it may mimic testicular
the arthritis/arthralgia, especially if the
torsion.
Kawasaki disease Considered in the child is unable to ambulate. Urinalysis
patient with an unremitting fever with a should be performed weekly whilst the dis-
Testicular torsion can be an infrequent
complication of scrotal oedema.
maculopapular rash that is prominent on ease is active. Provided there is no evidence
the lower extremities. This rash, however, of nephritis during this period, patients
Gastrointestinal bleeding and
intussusception can complicate HSP.
does not appear in crops and is not asso- should have a urinalysis on a monthly basis
ciated with arthritis. The eye findings in for 6 months after all other manifestations
Kawasaki disease are usually absent in HSP. have resolved.
Bacterial endocarditis, infectious mononu- Patients who develop nephritis at any
Prognosis
cleosis, rubeola and streptococcal infection time should have blood pressure and serum Though the overall prognosis is excellent for
may present with a purpuric rash. HSP can creatinine monitored periodically until the HSP, nephritis is the one manifestation that
occur with other forms of vasculitis or autoim- nephritis has resolved. can become chronic. Renal disease develops
mune disease, such as inflammatory bowel Children with overt renal disease, e.g. in 5%. Prognosis depends on the severity
disease or familial Mediterranean fever. complicated by nephrotic syndrome, hyper- and extent of renal involvement. In general,
Rocky Mountain spotted fever should be tension or renal insufficiency at presentation patients with isolated haematuria, with or
considered in endemic areas when there is should be referred to the renal physicians without trivial proteinuria, have a uniformly
a history of tick exposure. for management, as a renal biopsy may be good prognosis. However, up to 50% of
required to predict prognosis more precisely. children with nephritis associated with
These patients usually require prolonged nephrotic syndrome may progress to renal
follow up. insufficiency. Approximately 2050% will
Treatment have persistent urinary abnormalities on
Treatment is mainly supportive with hydra- long-term follow up but only 1% of patients
tion, rest and analgesics. progress to ESRD.
Recurrence
There is suggestive evidence that corti- Clinical presentation is helpful but is not a
costeroids enhance the rate of resolution A recurrence rate of 40% has been reported precise determinant of prognosis. Severity of
of the arthritis and abdominal pain but do for symptoms of HSP. The recurrences tend renal disease and the ultimate outcome cor-
not appear to prevent recurrence of disease. to resemble the original episode but are usu- relate more closely with histopathological
Available data, though considered some- ally milder and of shorter duration. changes on renal biopsy and the degree of
what controversial, suggest that patients Recurrences appear to be more common proteinuria at presentation. Poor prognosis
with severe nephritis (haematuria, nephrotic in those patients with nephritis. is associated with crescent formation involv-
syndrome, decreased glomerular filtration With each recurrence of disease, patients ing more than 50% of the glomeruli.
rate (GFR) and crescent formation involving should have urinalysis and blood pressure Regardless of clinical presentation or his-
>50% of the glomeruli) should receive checked. tological changes, all patients with nephritis
a combination of high-dose intravenous HSP nephritis may recur in transplanted need prolonged follow up since deteriora-
methylprednisolone (30 mg kg1 day1) for kidneys. Recurrence of the nephritis and tion in renal function may occur in some
3 days followed by oral steroids plus an extra-renal manifestations are more likely patients many years later.

380
16.7 HENOCHSCHONLEIN PURPURA
16

RENAL
Prevention Further work is needed to explore the Further reading
role of cytokines in determining the Chang W-L, Yang YH, Wang LC, et al. Renal manifestations
There appears to be no single pathogen or exclusive involvement of IgA1 in the in henoch Schonlein purpura: a 10 year clinical study. Ped
environmental agent that has emerged as Nephrol 2005;20:126972.
immunopathogenesis of HSP. IgA Cronan K, Norman M. Renal and electrolyte emergencies. In:
an important precipitating cause of HSP, has two subclasses, IgA1 and IgA2, Fleisher G, Ludwig S, editors. Textbook of pediatric
thus there is no means of preventing HSP. emergency medicine. 4th ed. Philadelphia: Lippincott
but HSP is associated with Williams & Wilkins; 2000. p. 851.
abnormalities involving IgA1 rather Meulders Q, Pirson Y, Cosyns JP. Course of HenochSchonlein
nephritis after renal transplantation. Transplantation
Controversies and future than with IgA2. 1994;58:117986.
Narchi H. Risk of longterm renal impairment and duration of
directions  The fundamental abnormality follow up recommended for Henoch Schonlein Purpura with
normal or minimal urinary findings: a systematic review.
A number of studies have addressed responsible for HSP and IgA
Arch Dis Child 2005;90:91620.
nephropathy needs to be Oner A, Tinaztepe K, Erdogan O. The effect of triple therapy
the question of whether early on rapidly progressive type of HenochSchonlein nephritis.
elucidated.
corticosteroid treatment prevents the Paediatr Nephrol 1995;9:610.
delayed appearance of nephritis but the Role of other immunosuppressives?
Saulsbury FT. HenochSchonlein purpura in children Report
of 100 patients and review of the literature. Medicine
results are conflicting. 1999;78(6):395406.

381
SECTION

17 PSYCHIATRIC
Section editor Jeremy Raftos

17.1 Paediatric psychiatric emergencies 382 17.2 The treatment of the behaviourally disturbed
adolescent 386

17.1 Paediatric psychiatric emergencies

Raymond Chin Michael Fairley

Preconceptions belief system,

ESSENTIALS expectations from medical consultation.
What the child and parent really want.
1 Children with psychological problems often present with somatic complaints and
at times of family crisis. The treating emergency physician should be aware of this. Synthesis of assessment
2 Early identification of psychological illness can reduce prolonged investigations It should be possible for the emergency phy-
and expedite specific psychiatric treatment. sician to determine why the child is attend-
ing, who wants something done, and where
the main pathology rests; in the patient, the
be provided that the visit is not a punish- parent or their relationship.
Introduction
ment. Playing and drawing may put the child
Paediatric patients and, at times, their par- at ease. Examination
ents can provide complex diagnostic and In adolescents, self-referral for psycholog- Examination includes obtaining vital signs,
logistic challenges to staff in a busy emer- ical problems is more likely, but is usually and a physical and mental state exam.
gency department (ED). They often present associated with reluctance to identify and
in a time of life crisis. However, the com- discuss the problem. Physical complaint
Investigations
Special tests should be confined to the differ-
plaint may be primarily emotional. The may point to a psychological issue, e.g. head-
ential diagnosis of assessment.
majority of cases have no clear diagnosis ache and panic attack. As the patient is usu-
and may be complicated by multiple visits ally unco-operative, reassurance should be
to previous medical practitioners. provided about confidentiality.
Assessment requires time and patience COMMON PAEDIATRIC
and unique clinical skills. Intent listening History PSYCHIATRIC
with directed questioning is required. It is A thorough medical history is imperative. In PRESENTATIONS
important to avoid drawing premature con- addition, the six Ps of collateral history
clusions, and emotional factors should be should be explored.
considered, even when the problem appears The acutely disturbed child
predominantly organic. Collateral history See separate chapter dealing with this
Pre-existing psychological disorders, life presentation.
experiences, medical conditions, family
vulnerability.
General approach
Precipitants trigger for presentation. Suicidal patients
Assessment  Presentation why now, who is most
It is important to establish:
In the case of children, parents generally ini- troubled by the symptom.
tiate medical contact, so it is important to Perpetuation factors that operate to Risk of suicide or repeat attempt.
seek the childs opinion early. If possible, a prevent recovery. Nature of current crisis and meaning of
history from both the parent and the child Positives strengths and resources, the event.
should be obtained. Reassurance needs to coping with previous problems.  Presence of psychiatric disorder.

382
17.1 PAEDIATRIC PSYCHIATRIC EMERGENCIES
17

PSYCHIATRIC
Background history, personality style, are from a socially disadvantaged pervasive hopelessness;
coping mechanisms. background characterised by low quality of interaction with the
Social supports. socioeconomic status, limited educational interviewer.
Source of appropriate help. achievements, low income, poverty and
Document:
associated factors;
experience parental loss through appearance;
Introduction separation and divorce; behaviour during interview;
The presentation of a child or adolescent have experienced physical or sexual abuse; communication (content, themes,
with self-directed harm or injury constitutes have experienced impaired parentchild structure);
a medical and psychiatric emergency, even if relationships; affect (mood);
the need for medical urgency appears low. have parents with a mental illness such as perception;
The presentation of a child or adolescent mood disorders, harmful drug use cognition (orientation level, formal
with suicidal ideation but no apparent phys- (including alcohol) or problems involving thought disorder);
ical injury similarly constitutes a psychiatric violence; insight into present situation;
emergency. More people die in Australia by experienced loss through suicide in their judgement;
suicide than by motor-vehicle accidents. The family or peer group; rapport.
ratio of male to female suicides is currently experience a greater number of life
approximately 4:1 and the figures repre- stresses than normal. Hazards during assessment
senting ratios of male to female attempted
suicides vary generally from being equal to
Use of denial by parents, doctors and
Evaluation of the suicidal crisis nurses.
up to 15:1 in favour of females.1
Suicidal act Refusal to be interviewed.
The rate of suicide in older teenagers has
increased, especially over the past 30 years.
Intent (wish to die). Lack of insight.
Although rare in prepubertal children, it is
Lethality (danger to life). Negative influence of relatives.
always indicative of a severe disorder.
Mitigating circumstances (factors Pressure and hostility from other staff.
interfering with the persons ability to
In young persons who commit suicide, up
assess the consequences of the act). Management following
to 46% have made a prior threat or attempt,
and of those who attempt suicide, some 10% Specific enquiry into: an attempt
eventually kill themselves, often within Comprehensive medical assessment for
2 years.
Degree of isolation, probability of extent of injury as soon as possible. This
intervention, precautions against discovery. should occur prior to psychiatric
The most important contribution that can
be made to the prevention of suicide is the
Actions to gain help during, or after, an examination.
recognition of the early signs of mental ill-
attempt. Admission to a medical unit if outcome
ness, in particular, depression and schizo-
Degree of planning, level of impulsivity. of assessment indicated that physical
phrenia. Depression can present as school
Communication of intent, suicide note. safety has been adversely affected.
failure after normal achievement, hypochon-
Expectations of methods lethality.  If medical status is stable, referral to
driasis, social withdrawal and aggressive
Conception of reversibility with medical psychiatry for assessment is required in
attention. all cases.
behaviour, more often than verbal com-
plaints of depression and anxiety.
Use of alcohol or other drugs.

Energetic, persistent and effective treat-

ment is required once a suicide attempt Circumstance of the act
has been made. Precipitants bereavement, separation, Anxiety disorders
custody, disciplinary crisis.
Anxiety is a complex feeling of apprehen-
Immediate past history.
sion, fear, and worry often accompanied
Risk factors Interpersonal relationships.
by pulmonary, cardiac, and other physical
Theories abound to explain these high sui- Social support systems.
sensations. It is a ubiquitous condition that
cide rates although many of the assump- Impact on others e.g. rescue or
varies from the physiological to the patho-
tions about causality are not proven but rejection.
logical in its presentation. Panic disorder
based only on occasions. Nevertheless, a Family diseases.
has a lifetime prevalence of 1.53.5%.
thorough review of the literature2 leads to
In school refusal, the child often presents
the conclusion that the risk of suicidal beha- Mental state examination
with a somatic complaint such as abdominal
viour is increased among young people who: Look for:
pain. Cases of syncopal collapse may mani-
have previously attempted suicide; suicidal ideation; fest as a panic attack. Early identification
have a mood disorder or are involved in ongoing depression; can reduce the number of medical investi-
harmful use of drugs (including alcohol); psychotic features (delusions, gations and commence early psychiatric
have problems involving violence; hallucinations); referral.

383
17.1 PAEDIATRIC PSYCHIATRIC EMERGENCIES

Acute anxiety can effectively be treated thought control. Diagnosis is based on QT syndrome, bradycardia, orthostatic
with a comprehensive approach utilising descriptive clinical phenomena. hypotension, and shock due to congestive
psychotherapy, counselling, and a wide spec- heart failure.
trum of anxiolytics (e.g. benzodiazepines, Treatment Renal disturbances include decreased glo-
buspirone, and antidepressants). Combined psychotropic and psychothera- merular filtration rate, elevated creatinine,
peutic treatment is required. Phenothiazines oedema, acidosis with dehydration, hypoka-
(e.g. thiothixene 0.100.40 mg kg1 day1) laemia, hypochloraemic alkalosis with
and butyrophenones (e.g. haloperidol vomiting, and hyperaldosteronism.
Autism 0.050.15 mg kg1 day1) may be effective Gastrointestinal findings include consti-
A syndrome of early childhood characterised in controlling acute psychotic symptoms, pation, delayed gastric emptying, gastric
by: abnormal social relationships; language but relapse is common. Hospitalisation is dilation and rupture, dental enamel erosion,
disorder with impaired understanding, echo- useful in managing acute exacerbations; palatal trauma, enlarged parotids, oesopha-
lalia; rituals and compulsive phenomena; some children require continuing inpatient gitis, MalloryWeiss lesions, diminished gag
and uneven intellectual development with psychiatric care. reflex, and elevated transaminases.
mental retardation in most cases. Finally, bone marrow suppression may
Autism is two to four times more common occur, leading to platelet, erythrocyte, and
in boys than girls. Eating disorders leucocyte abnormalities.
Autism usually is manifest in the first year
Anorexia nervosa occurs in approximately History
of life; its onset is not later than age 3 years.
one out of 100 adolescent females and is Patients may present to the ED with
most frequently found in middle-to-upper extreme weight loss, food refusal,
Treatment socioeconomic groups. Most recent studies dehydration, weakness, or shock.
For the most severely impaired children, sys- have suggested no increased incidence of Affect may be flat or nearly catatonic.
tematic application of behaviour therapy, a anorexia nervosa over the last four decades. Patients may be depressed and should be
technique that can be taught to parents, helps A familial component appears to be present. questioned to gauge risk of suicide.
manage the child in the home and at school. Obtain a mental history because there is
Butyrophenones provide limited benefit, Mortality/morbidity The mortality rate for a strong association with depression and
mainly in controlling the most severe forms anorexia nervosa ranges from 510% and is substance abuse.
of aggressive and self-destructive behaviour greater (540%) among bulimic patients. Often, the family will bring in the patient
they do not resolve the psychosis. Fenflur- because many patients may refuse to
amine, a serotonergic antagonist, is no longer Race Anorexia nervosa is significantly more seek help or may have no insight into
available. frequent in white populations than in people their problem.
Speech therapy should begin early. For of other races. The co-existent effect of socio- Recurrent patients may recognise their
mute children, the value of learning sign lan- economic class, however, is difficult to isolate. problem and present spontaneously for
guage is not yet established. Children in the therapy.
near-normal or higher IQ range often benefit Sex Female to male ratio is 1050:1 in
from psychotherapy and special education. developed countries. Physical
Physical examination may reveal
Age Anorexia nervosa is primarily a phe- hypothermia, peripheral oedema,
nomenon of puberty and early adulthood, thinning hair, and obvious emaciation.
Childhood schizophrenia although premorbid determinants and long- Behaviourally these patients often have
Psychotic states usually have onset after age term outcomes are still being defined. a flat affect and display psychomotor
7 years and with behavioural similarities to Anorexia nervosa is an eating disorder retardation.
adult schizophrenia. Evidence suggests that characterised by severe weight loss to the
environmental stress precipitates manifest ill- point of significant physiological conse- Care
ness in children with a genetic predisposition. quences. Patients with anorexia nervosa ED care may include rehydration, correction
The prevalence of this disorder increases generally fall into two categories: (1) those of electrolyte abnormalities (e.g. hypokalae-
with age. Whereas autism and pervasive who practise extreme food restriction mia), and institution of appropriate disposi-
developmental disorder are distinctly differ- behaviours; and (2) those who display food tion for continuing medical and psychiatric
ent from the adult schizophrenias, childhood binge/purge behaviour. treatment.
schizophrenia forms a continuum with the
adolescent and adult forms. Complications Consultations
It is characterised by withdrawal, apathy, Complications of anorexia nervosa may As appropriate, consult with psychiatry and
flat affect, thought disorder (blocking and result in ED presentation. adolescent medicine specialists in order to
perseveration), ideas of reference, hallu- Cardiovascular effects include supraven- optimise inpatient care and facilitate out-
cinations and delusions, and complaints of tricular and ventricular dysrhythmias, long patient follow-up care.

384
17.1 PAEDIATRIC PSYCHIATRIC EMERGENCIES
17

PSYCHIATRIC
in children, one parent, usually the father, life and cause the child significant distress
Problem parents is absent during hospitalisation; and impaired everyday function. Usually
Parents with a mental illness history of sudden infant death syndrome onset is in children aged 312 years; the dis-
Depression in siblings is noted; order generally resolves during adolescence.
Mothers with postnatal depression may parent is overly attached to patient; An estimated 16% of children experi-
present frequently in a cry for help. patient has poor tolerance of treatment ence night terror episodes. Recurrent night
(e.g. frequent vomiting, rash, and terror episodes accompanied by significant
problems with intravenous lines); distress and impairment are less frequent.
Munchausens syndrome by proxy general health of patient clashes with Peak frequency in children younger than
In 1977, Englishman Roy Meadow pub- results of laboratory tests; 3.5 years is at least one episode per week;
lished3 the first report of a new form of child parent shows inordinate concern for among older children, peak frequency is
abuse. He coined the term Mu nchausens feelings of the medical staff; 12 episodes per month.
syndrome by proxy (MSBP) after the syn- seizure activity is unresponsive to
drome that first had been reported by Asher anticonvulsants and is witnessed only by
History
in 1951. This term is applied when an adult, parent or caretaker.
The most important step toward diagnosing
usually the mother, presents a false history Tests that emergency physicians may con- this disorder is to obtain a detailed history.
to the physician regarding a child who is sider include the following:
not suffering from any of the fabricated Approximately 90 minutes after falling
symptoms. This history causes the physician urine toxicology screening; asleep, the child sits up in bed and
to perform unnecessary diagnostic proce- chemistry panels; screams. Prominent autonomic activity
dures that do not result in any specific diag- electrocardiogram; (e.g. tachycardia, tachypnoea,
nosis. (MSBP has also been called Polle drug levels for suspected poisoning diaphoresis, flushing) occurs. The child
syndrome, after Baron von Mu nchausens agents (e.g. aspirin, paracetamol appears awake but confused, disoriented,
only child, who died when aged 1 year.) (acetaminophen), anticonvulsants); and unresponsive to stimuli.
In 1994, the DSM-IV4,5 included a defini- cultures; Most episodes last 12 minutes, but the
tion for factitious disorder by proxy, which is coagulation tests; child may remain inconsolable for 530
now the accepted psychiatric category for head CT scan. minutes before relaxing and returning to
MSBP. The definition includes the following: quiet sleep.
Parents with substance abuse If the child awakens during the night
Intentional production or feigning of Parents with significant substance abuse, i.e. terror, only fragmented pieces of the
physical or psychological signs or alcohol and other drugs have significant episode may be recalled.
symptoms in another person who is impact on the childs presentation. The In the morning, the child typically has no
under the individuals care. parental behaviour can be inappropriate, memory of the experience.
The motivation for the perpetrators threatening and aggressive towards the
behaviour is to assume the sick role by practitioner. This makes the assessment of
proxy. the child much more difficult and unreliable.
Management
 External incentives for the behaviour, This consists of educating the family about
such as economic gain, avoiding legal the disorder and reassuring them that epi-
responsibility, or improving physical sodes are not harmful.
well-being, are absent.
Miscellaneous
Night terrors
Children at risk for MSBP abuse are aged
Sleep disruption is a parents most frequent
15 months to 6 years. The emergency physi- References
concern during the first 2 years of a childs
cian often is confronted with baffling symp- 1. Aghababian RV, Allison EJ, Braen GR, et al., editors.
life. Half of all infants develop a disrupted Emergency medicine: The core curriculum. Philadelphia,
toms. Frequently, the child has been taken to
sleep pattern serious enough to warrant PA: Lippincott, Raven; 1998.
many care providers before the diagnosis is 2. Baeutrias A. Risk factors for suicide and attempted
physician assistance. Night terror disorder suicide among young people. Setting the evidence-based
finally established. Warning signs that are
is characterised by recurrent episodes of research agenda for Australia: A literature review.
suggestive of MSBP include the following: Canberra: National Health and Medical Research Centre,
intense crying and fear, and by difficulty Commonwealth Department of Health and Aged Care;
illness is multisystemic, prolonged, arousing the child. Children also can ex- 1999.
3. Meadow R. Munchausen syndrome by proxy. The
unusual, or rare; perience signs of autonomic arousal (e.g. hinterland of child abuse. Lancet 1977;2(8033):3435.
symptoms are inappropriate or tachycardia, tachypnoea, sweating) during 4. DSM-IV. Diagnostic and statistical manual of mental
disorders. 4th ed. Washington, DC: American Psychiatric
incongruent; episodes. Children do not recall a dream after Association; 1994.
patient has multiple allergies; a night terror and typically do not remember 5. ICD-10-AM. The international statistical classification of
diseases and related health problems, 10th revision,
symptoms disappear when parent or the episode the next morning. Night terrors Australian modification. 2nd ed Lidcombe, NSW: National
caretaker is absent; are frightening episodes that disrupt family Centre for Classification in Health; 2000.

385
 17.2 The treatment of the behaviourally
disturbed adolescent
Kenneth Nunn

domains of risk), the level of the threat,

ESSENTIALS the emotional and behavioural identifiers
of risk and the performance indicators for
1 Emergency psychiatry is treating the underlying neurobehavioural processes risk management, all within a rapid time
NOT the cognitive content or psychiatric diagnosis.
frame, requires training, skill and experience.
2 When thinking about management it is worthwhile to move back through the
ABCC from the most disrupted young people, through to the early levels of distress Time is risk
and dysfunction. The time taken before treatment begins in
psychiatric triage is related to increasing risk,
C If cognitive processes are very disrupted, hospital admission will be necessary as impulsivity, agitation and lack of relief
irrespective of the eventual diagnosis.
of distress converge with the sensory over-
C If containment is threatened - offer co-operative sedation early and put stimulation of most ED waiting areas.
security on notice. If it is actually being breached, all other treatment must Time passing constitutes an escalating risk
wait until containment is addressed voluntarily or involuntarily. of a loss-of-control, or loss-of-containment,
event. Where agitation is present, motor
B If behaviour is extreme, actively offer relief with calm reassurance, nursing activity increased and cognitive processes
presence and medication while consciously preparing for escalation to a
clearly altered, the risk rises dramatically
containment breach.
after even short periods of waiting. The com-
A If arousal is high, establish whether this can be readily managed by calmness monly encountered slide down the spectrum
and co-operative use of medication. If calm is not forthcoming, be prepared for of distress to disruption anxiety, agitation,
behavioural escalation. anger, together with demanding, impatient,
impulsive and explosive behaviour leaves
few options once in full progress. On the
other hand, the rapid initiation of triage
supply, sometimes not trained in Emergency and the commencement of an altogether
Introduction Psychiatry, or unaccustomed to adolescent less disruptive process seen, relieved, trea-
Emergencies fragment clinician thought, psychiatry. For the present, emergency ado- ted, monitored, transitioned are each asso-
unless they are transformed into routine. lescent psychiatry largely falls to emergency ciated with a reduced risk of loss-of-control.
No area of medicine is more difficult to con- physicians.
form to routine without practice than psychi- Pre-triage
atry and no area of psychiatry is more Early warning signs subjective
inconvenient in this regard than adolescent
The principles of psychiatric Identifying a safety threat to staff and
psychiatry. others.
triage
Just as in paediatrics, the first question is Questions such as Do I feel unsafe? Am I
not which illness? but how sick are they? Purpose of triage anxious for their welfare? Is there a
There is currently too much emphasis on Triage aims to enable the earliest possible sense of threat? may all provide useful
whether or not there are signs of a particular identification and treatment of factors that clues, if considered (Table 17.2.1). It is
mental illness, not whether there are the cru- may threaten the immediate safety and worthwhile acting on this as a given until
cial elements of neurobehavioual disorder well-being of the patient, others and staff reassured otherwise.
and the disruption of basic neurobehavioural in triage and the emergency department
systems. Despite this, once the essentials are (ED). The aim is to put safety first, manage- Early warning signs observed
understood and applied, the emergency phy- ment of distress and behaviour second, and Some behaviour stands out as out of
sician can provide consistent, high quality diagnosis third. It is critical that the task of control, unusual, potentially dangerous,
care in triage, initial stabilisation and man- definitive diagnosis does not delay acute conflicting, or distressing, before the patient
agement of immediate risks before prepar- management. Whether or not a young per- and those attending them sit down and you
ing for transfer for definitive management son has a particular psychiatric disorder is begin to listen. Sitting down is a helpful first
and ongoing treatment where needed. of secondary importance. Accurately identi- step to reducing arousal and activation.
This chapter is written with the underly- fying the components of threat, the com- A louder than usual voice, an argument
ing premise that psychiatrists will be in short plexity of the threat (that is the number of between parent and child, a surly withdrawn

386
17.2 THE TREATMENT OF THE BEHAVIOURALLY DISTURBED ADOLESCENT
17

PSYCHIATRIC
are sustained, they may overflow and have
Table 17.2.1 A triad of subjective early warning signs
an impact on the other patients, staff and
Threats to safety Syndromes of distress Organic flags
the functioning of the ED. If this impact is suf-
If you feel unsafe or If you feel distressed If you cant make sense and ficiently disruptive, they may constitute a con-
moved to protect others at watching the child looks unwell
tainment threat or demand for containment.

father, a seemingly drunken teenager, may Cognitive processes the coherent Containment Behavioural control with-
all catch our attention. communication of reality-based thinking in a social setting so as to reduce major threat
An immediate threat to others, an imme- and feeling. and disruption, that is in an acute medical
diate threat of patients to themselves or an setting, is termed containment. While contain-
Identifying the triad of early changes, rate ment is usually a physical process, which
immediate threat to medical safety, all
of change and the extremity of change to reduces the capacity of a patient to disrupt
require immediate action analogous to a
each component of assessment will be used the ED, it is primarily aimed at reducing risk
cardiac arrest. Practising commonly encoun-
throughout. to others, risk to self and risk to the environ-
tered scenarios with acceptance of error and
inefficiency is essential. ment in the ED. The acute and open nature
Arousal The triad of early signs of of the ED means that any disruptive threat
change in arousal, volatility of arousal and may constitute a broader threat to the provi-
The hierarchy of needs
extremes of arousal are our focus. Thus, early sion of urgent medical treatment to the young
All triage involves addressing a hierarchy of
autonomic signs of either sympathetic or person in question or to others.
needs (Table 17.2.2).
parasympathetic shift include pallor, flush- It follows that, since changes in arousal
Safety, symptom relief and initial investi-
ing, tachycardia, bradycardia, tremulousness, normally precede changes in behaviour,
gation (SSRI) and working or provisional
mydriasis and tachypnoea. Marked hypervig- which precede changes in the likelihood of
diagnosis (PD) form the underpinnings of
ilance and clouding of consciousness or rapid containment, the early warning signs of a
emergency psychological care. The estab-
excursions between the two are very helpful loss of containment event have already been
lishment of mental state monitoring and
in identifying the need to intervene rapidly. covered under the headings of arousal and
the active exclusion of medical contributors
Anxieties may be more difficult to assuage behaviour. However, frequently these early
commence immediately upon completing
with lack of fine social and emotional tuning features will have happened prior to being
the initial stabilisation.
with either unresponsiveness, or exquisite seen in the ED and the demand for contain-
Signs of threat to safety responsiveness, to the environment, or a rap- ment is the presenting request.
Behaviour potentially injurious to others idly changing mixture of both. If the arousal Running away, disrupting the ED, damag-
requiring PROTECTION & RESTRAINT. is sufficiently disruptive, the child or young ing property and creating an atmosphere of
Disruptive behaviour with a threat to person may have a functional overflow of threat and menace would all constitute a
safety requiring CONTAINMENT. their arousal into their behaviour. containment threat or a containment failure
 Out of control behaviour that requires event. In extreme cases this may involve
CONTAINMENT. Behaviour Again, early signs of change weapons, the police and the clearing of the
in behaviour, volatility of behaviour and
ED, with cessation of medical activities while
Signs of intense distress extremes of behaviour are our focus. Sitting
a local Disaster Response is put into place. In
By way of analogy with the provision of with one leg constantly shaking, wringing of
most cases, some form of containment was in
acute life support, it is helpful to employ hands or stroking of hair as part of anguish
place before being brought to the ED and it
the ABCC mnemonic. or anger all portend an imminent deteriora-
is important that this containment is not lost
tion in behaviour. Constant pacing or refusal
in the transition process into the ED or in
The ABCC of rapid psychiatric to move, a loud voice or speaking very quietly,
transfer from the ED. If containment failure
assessment swearing excessively and particularly offen-
is the external manifestation of maximal dis-
Arousal autonomic and cortical arousal sively, or refusing to talk as part of a broader
ruption to the individual, the fragmentation
Behaviour behavioural activation picture of social disinhibition or extreme inhi-
of thought processes or pathological coales-
Containment behavioural control within a bition, reflect extremes of response and lack
cence of thinking into paranoid or self-
social setting of fine social and emotional tuning. If these
destructive ideation is the inner manifesta-
tion shown in arousal, behaviour, including
Table 17.2.2 The hierarchy of patient needs speech, and containment.

Threat Need Sign for observation

Cognitive processes The early mani-
Danger Safety Signs of threats to safety festations of thought disruption are changes
Distress Relief Signs of intense distress in arousal, behaviour, especially speech, and
containment that belie a loss of coherence
Disease Treatment Signs of organic cerebral, and long-term psychiatric dysfunction
in the process of thought and other cognitive
processes. Other cognitive processes such as

387
17.2 THE TREATMENT OF THE BEHAVIOURALLY DISTURBED ADOLESCENT

attention, executive function (planning, judg- Identify the immediate issue of concern Target behaviour and agitation for
ment, problem solving and insight) and per- to the young person and to others while example, the inability to be able to move
ception, underpin thinking and consequent establishing a formulation in medical in an emotional paralysis or to sit still due
action. This is not to say that thought or terms. to agitation, despite sustained reassurance.
speech content is irrelevant. The young per-  Identify risks to self and others,
son may be worried, fearful, self-loathing, including, maltreatment, medical and Tracking the seven stages
bizarre, threatening and angry. However, the reputation risks. of sedation
processes of perceived threat (in the extreme, Symptomatic relief provide immediate Fixation immobilising the body to
paranoia) and the pursuit of relief (in the treatment with whatever biological, enable vascular access and a safe
extreme, self-harm and suicide) are the under- psychological and social supports and medical procedure.
pinning cognitive processes foremost in any
emergency assessment.
resources are available. The provision of Induction commencing sedation with
relief is the single biggest factor in the steady reduction of consciousness.
Extremes of incoherence or coherence reducing risk.  Disinhibition loss of emotional and
apply here as well. Jumbled, accelerated, Identify psychosocial supports and behavioural control associated with loss
guarded, slowed down, rigidly preoccupied, stressors (immediate), i.e. who is the of cortical inhibition before limbic and
fixedly convinced or non-communicative consenting authority for the young basal nuclei are similarly inhibited.
speech and thought are all more worrying person and where do they normally Stabilisation of arousal depth titrating
process variables, irrespective of what or live. None of these may be available in the to a level of sedation that maintains gag
who is upsetting the patient. acute situation. However, they will save reflexes, pharyngeal patency and
a great deal of time in determining the adequate breathing and oxygenation.
Managing the ABCC, including disposition of the patient. Maintenance high-level observation
restraint and acute sedation A plan of action for the next 24 hours. with clear parameter thresholds and
It is inappropriate to attempt to obtain a Sedating the adolescent brain specification of appropriate responses
detailed developmental history, systems When attempting to provide reduction in when thresholds are breached.
review or full exploratory psychiatric history arousal and behavioural agitation, or to man- Emergence the period of decremental
from an acutely distressed and very ill young age a breach of containment, it is helpful to lowering of medication to allow a
person. When thinking about management consider which parts of the brain or neuro- transition to the fully conscious state
it is worthwhile to move back through the behavioural systems are being targeted. with an awareness that disinhibition
ABCC from the most disrupted young peo- The neurobehavioural systems are referred may occur during this process as during
ple, through to the early levels of distress to here as subbrains as a useful shorthand. the establishment of sedation.
and dysfunction. After care the psychological
The thinking brain (the cortex) easiest to
explanation and support required
C If cognitive processes are very disrupted, sedate, especially in children inhibits
specifically in relation to the sedation
hospital admission will be necessary, the other two brains. When the cortical
process.
irrespective of the eventual diagnosis. brake is sedated, the two lower brains
C If containment is threatened, offer (disinhibition) are released.
co-operative sedation early and put Benzodiazepines are often enough to Five tips on sedating adolescents
security on notice. If it is actually being sedate this brain. Start high (without bolus) and titrate
breached, all other treatment must wait down in an emergency.
until containment is addressed voluntarily Target arousal and preoccupation for If there is definite motor agitation, do not
or involuntarily. example, the inability to stop thinking about use a benzodiazepine alone, which may
B If behaviour is extreme, actively offer distressing events despite sustained talking disinhibit the patient. Add haloperidol
relief with calm reassurance, nursing through and reassurance. and monitor for extrapyramidal side
presence and medication while The feeling brain (the limbic system) takes effects such as dystonia.
consciously preparing for escalation to a longer and more medication to sedate,  If re-sedation is likely, move to regular
containment breach. especially when distress is established. doses rather than p.r.n., ebb and flow
A If arousal is high, establish whether this Benzodiazepines may be enough, but prescribing. Less medication is needed if
can be readily managed by calmness and often antipsychotics are needed. non-p.r.n.
co-operative use of medication. If calm is Initiate sedation away from the main
Target arousal and distress for example,
not forthcoming, be prepared for ED (more private and less disruptive)
the inability to find emotional relief despite
behavioural escalation. and maintain and monitor close to the
sustained and skilled reassurance.
main ED.
There are only six essential things which
must be attempted:-
The moving brain (the basal nuclei) takes Plan for emergence risks ensure
longest to sedate and is first to awaken security presence during emergence
Rapport and respect even where they from sedation. Antipsychotics are almost from sedation or be prepared to
cannot be achieved. always needed. re-sedate.

388
17.2 THE TREATMENT OF THE BEHAVIOURALLY DISTURBED ADOLESCENT
17

PSYCHIATRIC
Signs of organic dysfunction Work closely with security staff. Issue ultimatums do this or else
syndromes Ask early and provide sedation almost always leads to or else in this
Elevated temperature in a psychotic back-up. population.
patient. Back into corner either physically or
Appearance Looking lost or out of it psychologically (unless everything else
with lowered eyelids, glazed eyes and in a Behaviours that usually de-escalate
has failed and safety demands we must).
aggression
world of their own. Adopt a thou shalt tone of voice instead
Behaviour Recent personality change Nothing works all the time and nothing
of a reasonable request.
works in every case, but some of these are
with loss of social fine tuning and reversal Raise voices in a counter arc-up to the
of sleep rhythms. likely to be helpful.
patient.
Speech Muddled speech and thinking,  Mock, criticise or accuse the patient at
often with paranoid but fragmented Ten DOs any time.
themes.  Rush the process or create time pressure.
Perception Visual illusions, i.e. Do be respectful, friendly and open  Hark back to previous behaviour or try to
misidentifying actual stimuli, e.g. (single most important strategy, sort out longstanding issues unless this
shadows mistaken for people or frank especially respect). is a previously agreed treatment goal.
visual and tactile hallucinations Be quicker to listen than to speak.
(especially with antihistamine or  Do speak clearly, quietly, gently and The four main themes are to
anticholinergic overdose). calmly with an expectation that they will
Ideation Often paranoid in thinking. respond. Communicate respect.
Cognition Clouded and fluctuating Use humour that shows we accept we Many of them have not been treated
levels of consciousness with have things about us that are not ideal, with respect.
disorientation. perfect or completely respectable, Communicate our desire to keep
Judgement and insight Impaired markedly. especially when we are derided or everyone safe.
spoken to rudely. We want them, and us, to be safe. Many
Do declare desire to be helpful even if it of our patients have not lived in safe
Differential diagnosis is not always known how to help or what environments.
Specific toxidromes and syndromes of it is they want.  Be willing to acknowledge we may have
adverse drug reaction Do relax posture, voice and face, even if contributed to their distress by what we
substance abuse preparing internally for fight and/or have done or not done.
accidental ingestion. flight. Many of the young people who will
Delirium generalised acute brain Move slowly, predictably and with due present have lived with people who
syndrome. respect for distance. have never accepted blame, use denial
 Frontal lobe syndromes (including TBI  Distract to details that they might be a great deal and blame others.
and intellectual disability). interested in as well, especially things Appreciate their distress even though it
Seizure-related disorders. about them and what they like. may not be fully understood why they
Starvation-related syndromes.  Acknowledge any faults in our feel like this or how bad they feel.
Cerebellar dysfunction behaviour (not someone elses) that Many of the patients have been told
slurred speech; might be contributing to them being they dont or do feel a certain way (even
ataxic gait; upset. if they did not) and had the legitimacy of
incoordination;  Acknowledge tiredness, a hearing their distress invalidated. They may
substance abuse, e.g. benzodiazepine; problem (if they are withdrawn, hostile, never have been in a situation where
toxicity from prescribed medications, talking quietly), irritability or crabbiness high distress is tolerated, validated and
e.g. anticonvulsants; after a busy shift, which emphasise our appreciated so long as safety is high as
ingestion of poisons, e.g. alcohol, humanity. well.
phenytoin.
Dementia generalised chronic brain
Identify patients threat
syndrome. Ten DONTs
to themselves?
Try to shame them into good behaviour If the patient or those with them feel
The management of acute risk by telling them they are childish, silly or unsafe, they probably are unsafe.
including medical risk stupid. If your ABCC evaluation says they are
Managing threat to staff and others Try to get above them ( so-called unsafe, they are unsafe.
Do not ask medical or nursing staff to towering) physically, by position, If a colleague outside the hospital says
carry out unsafe practice. verbally, intellectually or socially. they have been behaving unsafely, be
Offer co-operative oral sedation early,  Talk without listening or nag. Dont all very wary of not honouring their
such as olanzapine or quetiapine. talk at once one person only. assessment.

389
17.2 THE TREATMENT OF THE BEHAVIOURALLY DISTURBED ADOLESCENT

Managing a threat to themselves  0.050.20 mg kg 1 per dose;

Remain calm, firm, enlist security, Sentinel nursing  give slowly. Dilution enables greater
maintain constant observation and, observations post control of injection, monitoring
if actually attempting to hurt themselves intramuscular or respiration for bradypnoea, pause
during the assessment, intervene intravenous sedation between inspiration and expiration
immediately. and paradoxical respiration;
Respiratory rate, O2 saturation, pulse, blood
Identify treatable disorders and social  can be given in divided doses (2.5 mg
pressure and level of consciousness should
predicaments while waiting for suicidality quanta) alternating with haloperidol
be checked continuously during the mainte-
to settle (attenuates over several days and flush;
nance of sedation each 15 minutes for
usually).  several minutes between doses
2 hours, then each 30 minutes for 2 hours.
initially;
An electrocardiogram should be performed
Identify medical risks  4-6 hours between doses once initial
as soon as possible after any sedation with
The broader medical needs of the patient sedation has been established.
zuclopenthixol acetate or droperidol and
need to be recognised.
the medical officer contacted if QTc is above
Psychiatric disorder does not protect 440 ms in males and 460 ms in females. Transfer is a potential escalation
against medical disorder. of risk
These are conservative figures and represent
It is possible to do a great deal with
nursing thresholds for alerting doctors, not The leaving are they stable?
a non-co-operative patient to clarify The transfer are they monitored with an
thresholds for medical algorithms, which
medical status. adequate response to thresholds
may be considerably higher.
strategy?
Manage specific medical risks The arriving are the recipients prepared?
Obtundation - due to panic sedation in Three tips for monitoring
response to previous failed sedation in adolescents
adolescents who became disinhibited. Expect the medication to be
Extrapyramidal side effects nuchal metabolised quicker. Conclusion
spasm with headache is common in the They become disinhibited more easily
young as a form of dystonia. going into sedation and coming out of Emergency psychiatry is treating the under-
Respiratory laryngeal dystonia sedation. lying neurobehavioural processes not the
especially in younger patients but still  Maintaining a sedation intravenously cognitive content or psychiatric diagnosis.
rare, especially if benzodiazepines are not (IV) should be done with a slow injection Process psychiatry requires a different
used with butyrophenones. over several minutes avoid boluses to mind-set. Emergency physicians are ideally
Cardiac prolonged QTc, especially in avoid respiratory depression trained to adopt that mind-set.
poor 2D6 metabolisers (5% of normal (benzodiazepines) or cardiac
population). (butyrophenones).
Neurological delirium (common, Further reading
especially substance abuse), serotonergic
Specific medications Glick RL, Berlin JS, Fishkind AB, Zeller SL. Emergency
syndrome, neuroleptic malignant Psychiatry Principles and Practice. Philadelphia:
Intramuscular injection (IMI) will often be Lippincott Williams & Wilkins; 2008.
syndrome (rare) (NMS). Hillard R, Zitek B. Emergency Psychiatry. New York: McGraw
the route of choice and the agent should
Concurrent medical disorder asthma,
be relatively quick, effective and safe:
Hill Professional; 2004.
Nunn KP, Dey C, editors. The Clinicians Guide to Psychotropic
diabetes, traumatic brain injury, epilepsy, Prescribing in Children and Adolescents Second Edition.
1
atopy and anorexia. Haloperidol IMI 0.050.20 mg kg Child and Adolescent Mental Health Statewide Network,
(CAMHSNET) Publications; 2004.
Dermatological lamotrigine-induced per dose with doses up to 6-hourly. Petit JR. Handbook of Emergency Psychiatry. Philadelphia:
1
StevensJohnson syndrome. Midazolam IMI 0.050.20 mg kg per Lippincott Williams & Wilkins; 2004.
Slaby AE, Lieb J, Tancredi LR. Handbook of Psychiatric
Metabolic lithium toxicity especially dose with doses up to 6-hourly. Emergencies. New York: Medical Examination Publishing
chronic. Diazepam IVI Co; 1975.

390
 18

SECTION
CRISIS
INTERVENTION
Section editor Gary Browne

18.1 Sexual assault 391 18.2 Child at risk 394

18.1 Sexual assault

Matt Ryan

of activities that vary from exposing the

ESSENTIALS child to sexually explicit materials to anal
or vaginal penetration of the child. Central
1 Sexual assault occurs when a child is engaged in sexual activity that the child to the definition is the limitation of the child
cannot comprehend, for which the child is developmentally unprepared and cannot
to provide truly informed consent for sexual
give consent, and/or that violates the law or social taboos of society.
activity with adults.
2 Sexual assault includes a spectrum of activities ranging from rape to physically less Sexual play between children of similar
intrusive sexual activity.1,2 age does not fit into this description.
The term assault is preferred over abuse
3 Assessment and management of children following alleged or suspected sexual assault as it highlights the criminal nature of the
is a highly specialised area and requires a multidisciplinary, multiagency team approach.
activity and avoids minimisation of such
abusive acts.

Introduction treatment of acute physical injury;

provision of emergency contraception Attitudes/myths
Assessment of child sexual assault (CSA) and/or antibiotic and antiviral
requires a dedicated, well-trained and experi- prophylaxis;
surrounding CSA
enced doctor who is able to spend a signifi- protection of the child and referral to The subject of CSA is an emotive one. Emer-
cant amount of time making an unhurried local child protection agencies; gency physicians will often have strongly
and thorough assessment and detailed docu- referral to a paediatrician; held opinions and attitudes on this subject.
mentation of history and examination find- ensuring appropriate psychological These attitudes may be shaped by past
ings. The doctor must have an accurate support is provided to the child and family. experience and/or social taboos. In order
knowledge of genital anatomy, and experience to approach CSA in a calm, non-judgemental
In the majority of cases, determination of
in performing gynaecological examinations. and objective manner it is important that
whether or not sexual assault has occurred
Skills and experience in this field are developed emergency physicians are cognisant of their
is not possible within the emergency depart-
through postgraduate studies, significant case own opinions and emotional responses. In
ment (ED). In the majority of cases, physical
numbers, a knowledge of current literature dealing with victims of CSA, expressions of
examination will neither confirm nor refute
and involvement in peer-review practices.3 anger, sadness or surprise are not helpful
an allegation of sexual assault. The most
Inexpert assessment of such cases may and potentially stigmatising and harmful
important indicator of possible CSA is disclo-
have a profound negative influence on the to the child. With emergency physicians
sure by the child.
child and family. It may potentially lead to infrequently encountering CSA, it is useful
inappropriate removal of the child from to reflect on the following, sometimes poorly
the family or wrongful imprisonment.4 understood, statements.
The roles of the emergency physician in Definitions
this process are:
A broad range of sexual behaviours has
CSA is the use of a child for sexual grati- been observed in normal children.
recognition of the possibility of sexual fication by an adult or significantly older Most children are not abused by
assault; child/adolescent.5 It may involve a range strangers.6

391
18.1 SEXUAL ASSAULT

As historians, children are no less reliable  They are brought to the ED by social bleeding or acute injury, the examination
than adults. services or the police for a medical should be performed immediately. In this sit-
CSA is not normally an isolated incident. evaluation for possible sexual abuse as uation, protocols for CSA victims should be
CSA uncommonly produces severe part of an investigation. followed to secure biological trace evidence
genito-anal injury.3,710 They are brought to an ED after a such as epithelial cells, semen, and blood,
CSA often occurs in the context of other suspected acute sexual assault for as well as to maintain a chain of evidence.
family problems, including physical evaluation, evidence collection, and When more than 72 hours has passed and
abuse, emotional maltreatment and crisis management. no acute injuries are present, an emer-
substance abuse.11 gency examination usually is not necessary.
An evaluation, therefore, should be sched-
Recognition of CSA uled at the earliest convenient time for the
Epidemiology of CSA child, physician, and investigative team.17
Recognition of the possibility of CSA is depen- In the child presenting with genitoanal
There has been a significant increase in the dent on history and examination findings, injury or abnormality, CSA is only one of a
recognition of CSA,3,11,12 which has been both of which are normally non-specific. number of diagnoses that should be consid-
reflected by a substantial increase in the History from the child remains the single ered. The differential diagnosis of genito-
number of reports made to child protection most important diagnostic feature in coming anal injury includes:
services across Australia and overseas, par- to the conclusion that a child has been sex-
ticularly in the last 5 years. ually abused.13 accidental injury;
Sexual assault has been documented as falls astride;
occurring on children of all ages and both sexual assault;
sexes, and is committed predominantly by Signs and symptoms medical/dermatological condition, e.g.
men, who are commonly members of the lichen sclerosis/drug reaction.
childs family, family friends or other trusted
Non-specific
Genital findings in children are difficult to
Children who have been sexually assaulted
adults in positions of authority.11 interpret. Such interpretation is generally
may develop a variety of emotional and
Sexual abuse by family members or beyond the expertise of most emergency
physical complaints, often unrelated to the
acquaintances usually involves multiple epi- physicians.14 Whilst acute trauma may be
genital area. These include:
sodes over periods ranging from a week to easily recognised, interpretation of such
years. developmentally regressive behaviour; findings may be problematic for the occa-
Victims of unknown assailants tend to be deterioration in school performance; sional examiner.14
older than children who are sexually abused sleep disturbances;
by someone they know and are usually only abdominal pain;
subjected to a single episode of abuse. enuresis, encopresis;
The estimated proportion of children phobias; Genitoanal anatomy
exposed to some form of sexual assault var- sexualised behaviour.
Knowledge of what constitutes normal and
ies depending on the definition of sexual
abnormal anatomy has evolved over recent
abuse and methodology used. In the United Specific years. This has been driven partly by several
States, literature surveys provide estimates Disclosure by child.
highly publicised cases where misinterpreta-
of 952% for females and 310% for Genitoanal injury.
tion of normal findings led to inappropriate
males.6 There is no comprehensive compar- Sexually transmissible disease.
separation of children from parents and
ative Australian literature. Pregnancy.
wrongful conviction.

CSA and emergency Hymen

Genitoanal injury
medicine There is considerable variation in the shape
Only 4% of all children referred for medical of the hymen. In the prepubertal girl it is
Children who are victims of sexual assault
evaluation of sexual abuse have abnormal thin and relatively inelastic. In this age
may present to EDs in a variety of
examinations at the time of evaluation. group, blunt penetrating trauma to the
circumstances:
Even with a history of severe abuse, such vagina may result in tearing of the hymen.
They may be seen for an unrelated as vaginal or anal penetration, the rate of Such tears when healed may manifest as a
matter when routine history and abnormal medical findings is only 5.5%.13 notch or defect in the hymenal tissue.
physical examination produce The physical examination of sexually As an oestrogen-dependent/responsive
information where sexual assault forms abused children should not result in addi- tissue, at puberty the hymen becomes thick,
part of the differential diagnosis. tional emotional trauma. irregular and elastic and distensible. It is less
They are brought by a parent or carer to When the alleged sexual abuse has likely to sustain injury during penetration
the ED for evaluation of suspected abuse. occurred within 72 hours, or there is than in the prepubertal state.

392
18.1 SEXUAL ASSAULT
18

CRISIS INTERVENTION
Table 18.1.1 Guidelines for decision making

Data available Response

History Physical examination Laboratory findings Level of concern Report decision

about sexual abuse

None Normal None None No report

Behavioural changes Normal None Variable depending Possible report; follow-up closely
upon behaviour (possible mental health referral)

None Non-specific findings None Low (worry) Possible report; follow-up closely

Non-specific history by child Non-specific findings None Intermediate Possible report; follow-up closely
or history by parent only

None Specific findings None High Report

Clear statement Normal None High Report

Clear statement Specific findings None High Report

None Normal, non-specific Positive culture for sexually Very high Report
or specific findings transmissible disease, presence of
semen, sperm acid phosphatase

Behaviour changes Non-specific findings Other sexually transmitted diseases High Report

injury to, or scarring of, the posterior Documentation

Sexually transmitted fourchette; Because the likelihood of civil or criminal
diseases anal lacerations.17 court action is high, detailed records, and/
The diagnosis of a sexually transmitted or drawings should be kept.
Many cases of alleged sexual abuse involve
disease in a child may be highly suggestive parents who are in the process of separation
but is not diagnostic of sexual contact. or divorce and who allege that their child Mandatory reporting legislation
Rectal and genital Chlamydia infections in is being sexually abused by the other Some form of mandatory reporting legisla-
young children may be due to a persistent parent during custodial visits. Although tion exists in all Australian jurisdictions.
perinatally-acquired infection, which may these cases are generally more time consum- Although this legislation varies from state
last for up to 3 years.15,16 ing, they should not be dismissed because a to state, the basic principles are similar.
custody dispute exists. Allegations of abuse Doctors are mandated to report cases
that occur in the context of divorce proceed- where there is reasonable suspicion that
ings should either be reported to the child CSA will occur, or is occurring, and the
Diagnostic considerations guardian is unlikely to prevent such acts
protective services agency or followed-up
The diagnosis of CSA can often be made closely. (Table 18.1.1). The reporting practitioner
based on a childs history. Physical examina- has statutory protection from prosecution
tion is infrequently diagnostic in the if a report is made in good faith.
absence of a history and/or specific labora-
tory findings. Physical findings are often Role of the emergency
absent even when the perpetrator admits physician References
to penetration of the childs genitalia. Many 1. American Academy of Pediatrics. Committee on
The emergency physician (EP) has many Adolescence. Sexual assault and the adolescent.
types of abuse leave no physical evidence,
roles in CSA. The EP should ensure that Paediatrics 1994;94:7615.
and mucosal injuries often heal rapidly. 2. American Academy of Child and Adolescent Psychiatry.
any physical injuries are detected, accu- Practice parameters for the forensic evaluation of
On examination, findings which are sug-
rately documented and correctly treated. children and adolescents who may have been physically
gestive, but not diagnostic, of CSA include: or sexually abused. J Am Acad Child Adolesc Psychiatry
Medical issues, such as sexually transmit- 1997;36:42342.
abrasions or bruising of the inner thighs ted diseases and emergency contraception, 3. Donald T, Wells D. Graduate Diploma in Forensic
Medicine, Subject guide. Melbourne: Monash University
and genitalia; should be discussed and managed. Centre for Learning and Teaching Support; 2000. p. 253.
scarring or tears of the labia minora; The EP should collect, or provide oppor- 4. Butler-Sloss E. Report of the Enquiry into Child Abuse in
Cleveland 1987. London: HMSO; 1988.
enlargement of the hymenal opening. tunity for collection of, forensic specimens, 5. Kempe CH. Sexual abuse, another hidden paediatric
ensure appropriate psychological support problem: The 1977 C. Anderson Aldrich lecture.
Findings that are of greater concern include: Paediatrics 1978;62:3829.
is provided to the child and family, and 6. Tomison A. Update on child sexual abuse. National Child
scarring, tears, or distortion of the hymen; report the case to protective agencies Protection Clearinghouse; 1995. Issues in child abuse
prevention number 5. Available from http://www.aifs.
a decreased amount, or absence, of within the legislation of the local juris- gov.au/nch/pubs/issues/issues5/issues5.html
hymenal tissue; diction. [accessed 26.10.10].

393
18.2 CHILD AT RISK

7. Adams JA, Harper K, Knudson S, Revilla J. Examination 12. Leventhal JM. Epidemiology of child sexual abuse. In: 15. Hammerschlag MR. Sexually transmitted diseases in
findings in legally confirmed child sexual abuse: Its Oates RK, editor. Understanding and managing child sexually abused children. Adv Pediatr Infect Dis
normal to be normal. Paediatrics 1994;94:3107. sexual abuse. Sydney: Harcourt Brace Jovanovich; 1990. 1988;3:118.
8. Finkel MA. Anogenital trauma in sexually abused 13. Heger A, Ticson L, Velasquez O, Bernier R. Children 16. Hammerschlag MR, Doraiswamy B, Alexander ER, et al.
children. Paediatrics 1989;84:31722. referred for possible sexual abuse: Medical findings in Are rectogenital chlamydial infections a marker of
9. McCann J, Voris J, Simon M. Genital injuries resulting from 2384 children. Child Abuse Negl 2002;26(6 sexual abuse in children? Pediatr Infect Dis J
sexual abuse: A longitudinal study. Paediatrics 7):64559. 1984;3:100104.
1992;89:30717. 14. Makoroff KL, Brauley JL, Brandner AM, et al. Genital 17. American Professional Society on the Abuse of
10. McCann J, Voris J. Perianal injuries resulting from sexual examinations for alleged sexual abuse of prepubertal Children. Guidelines for psychosocial evaluation of
abuse: A longitudinal study. Paediatrics 1993;91:3907. girls: Findings by pediatric emergency medicine suspected sexual abuse in young children. Chicago, IL:
11. Finkelhor D. The international epidemiology of child physicians compared with child abuse trained physicians. American Professional Society on the Abuse of
sexual abuse. Child Abuse Negl 1994;18(5):40917. Child Abuse Negl 2002;26(12):123542. Children; 1990.

18.2 Child at risk

Simon Young Raymond Chin Gervase Chaney

child protection. Doctors, nurses and other

ESSENTIALS healthcare workers who deal with children
are an integral part of this system that acts
1 Non-accidental injury occurs when an adult responsible for the care of the child to protect children and adolescents. Every
either harms the child or fails to protect the child from harm.
health professional that has contact with
2 A child may be at risk from physical abuse, sexual abuse, emotional abuse or children needs to be aware of the possibility
neglect. of non-accidental injury, must be able to
detect when it is occurring and know how
3 Medical and nursing practitioners are often mandated by law to report suspicions to act in the best interests of the child once
of non-accidental injury.
it is suspected.

Introduction
these factors and the recognition that nega- Definition
tive experiences often have serious short-
Non-accidental injury is increasingly recog- and long-term implications for the child has The child at risk is not a medical diagnosis
nised as a major public health and social led to a general acknowledgment that chil- but rather a description of certain forms of
welfare problem with important short-term dren and adolescents need protection. behaviour displayed by adults responsible
and long-lasting effects for children and ado- The United Nations Convention on the for the care of a child. The child is at risk
lescents. As Australian and New Zealand Rights of the Child recognises that: when an adult responsible for the care of
Emergency Departments (ED) provide care the child harms, threatens to harm or fails
. . . for the full and harmonious to protect the child from harm. Harm may
for many hundreds of thousands of children
development of his or her personality, be either physical (e.g. inflicting an injury,
and adolescents each year, departments and
[a child] should grow up in a family causing pain or poisoning), psychological
their staff play an important role in the
environment, in an atmosphere of (e.g. causing feelings of being unloved or
detection of abuse and initiation of a medi-
happiness, love and understanding . . . worthless) or both.
cal and community response. This response
is aimed primarily at treating the child, mini- The Convention continues, stating in Article A child may be at risk from:
mising psychological effects and ensuring 19 that governments shall: physical injury;
their safety. sexual abuse;
. . . take all appropriate legislative,
Children and adolescents are, by virtue of emotional abuse;
administrative, social and educational
their developing intellectual, emotional and neglect.
measures to protect the child from all forms
physical state, a vulnerable group. The envi-
of physical or mental violence, injury or These are not exclusive and a child may be
ronment within which they develop is influ-
abuse, neglect or negligent treatment, subjected to more than one type of abuse.
enced by many factors outside their control:
maltreatment or exploitation, including
the economic and social status of their family,
sexual abuse, while in the care of parent(s),
the personality and values of family members Physical injury
legal guardian(s) or any other person who
and friends, and the extent of physical and This is the commonest type of abuse that is
has the care of the child . . .
intellectual stimulation that they receive reported to child protection agencies. Many
may all have profound influence upon their It is this philosophy that has driven the cre- thousands of children present to EDs around
development. The potential variability in ation of the social and legal framework of Australia each year with a wide range of

394
18.2 CHILD AT RISK
18

CRISIS INTERVENTION
physical injuries, the vast majority of which child sustaining the injuries. Specifically, Bruising of the skin Bruises are
are caused accidentally. It is a difficult but enquire as to when, where and how the extremely common in children. In the
essential task to identify children within this injury happened, who was present at the absence of a documented bleeding tendency
group who have been injured as a conse- time and what happened after the injury. they are evidence of blunt trauma and may
quence of abuse. While medical and nursing The childs medical, developmental and provide some information on the site, the
staff must be alert for the possibility of any social history, with specific information on implement or force of an impact. Accidental
non-accidental injury, there are specific cir- past injuries, is important. bruises are commonly found in children once
cumstances that may raise suspicion. These This history must be sought in an open they have learnt to crawl, occurring over
include: and non-judgemental fashion, which en- bony prominences, usually on the front of
courages the participants to reveal all the the body and are directly related to a childs
situations where there are direct important information. Unfortunately, the increasing motor activity. Babies who are
allegations of violence directed against
ED is often not the ideal place to conduct not yet crawling rarely have accidental
the child made by the child or any other
a lengthy and in-depth forensic interview bruising.
person;
with the parents or carers and it may be Bruises caused by abuse may occur any-
the type and pattern of injury observed at
prudent to limit the information gathering where on a childs body. Specifically, look in
the examination;
to items that will enable specific issues to places where accidental bruises are uncom-
an explanation being offered for an injury
be addressed. The interview can always mon such as the mouth, behind the ears, on
that does not fit the type or pattern of
be completed by trained investigators at a the inner aspect of the upper arm and
injury;
later time. around the buttocks. Observe the shape
delayed presentation for medical care with
and pattern of bruises, looking for features
an injury that a reasonable person would
Physical examination that may suggest a blow from an open hand
have recognised as needing care sooner;
Prior to commencing the examination the or a single or multiple blows from an imple-
multiple presentations, often to different
doctor must ensure that the parents and, ment. Look for a pattern within the bruise
healthcare providers, seeking medical
where appropriate, the child are informed that may suggest contact with a specific
attention.
of the nature and extent of the exami- surface.
Once the possibility of non-accidental injury nation and that valid consent has been Whilst it is important to describe the
has been raised the priorities of the treating given. In addition it is ideal to have spent appearance and colour of the bruising, it is
doctor are: some time with the child, to gain their not possible to be accurate about the age
confidence and thus increase the chances of a bruise. If a bruise is yellowing in colour
To diagnose, treat and document the
of keeping their co-operation during the then it is likely to be more than 18 hours old.
childs injuries.
examination.
To interpret the pattern of injury or
Consent from the parent or legal guardian Laceration and abrasion of the skin
behaviour as to the possible causes.
is necessary to conduct a physical examina- Lacerations are the tearing of tissues caused
 To notify and involve the agency
by a blow from a blunt object. Abrasions are
tion, to perform investigations (including
responsible for ensuring safety of the
photographs) and to release clinical infor- the disruption of the outer layers of the skin
child.
mation in the form of a report to a third caused when the skin contacts a surface at
To provide a written or verbal report and
an angle. They occur at or close to the site
party. If consent is refused the protective
advice to that agency or the police.
agency or police must seek a court order. of impact and may occur after a blow with
If there is an urgent medical problem that an object or after a fall on to a surface. They
Presentation needs intervention and such intervention is are frequently associated with bruising.
Physical abuse may present to the ED in clearly in the best interests of the child, then Examine the wound for neurovascular and
many different ways. Most commonly it will the examination and treatment should tendon injury and for foreign bodies. The
present as a child with an obvious injury and proceed and not be delayed by the lack presence of foreign material such as glass,
a suggestive history but in some situations it of consent. dirt or gravel should be noted as they may
will be more subtle, such as a younger child An adolescent may be able to give con- be important in evaluating the injury.
who presents not using a limb. sent for their own examination as long as
Children who present with a decreased they are capable of understanding what Burns or scalds The appearance of a
conscious state with no obvious cause may the examination entails, what the results burn on a child is influenced by many fac-
have a head injury from a blow or a fall or will be used for, and the implications that tors: the temperature, size and shape of
may have been poisoned. If the child is very this may have for them. the causative agent, the depth of skin at
young, the decreased conscious state may A thorough physical examination of the the contact site, the length of time of con-
be a result of being shaken. child should be performed, with observation tact and the application of first aid measures
and palpation of skin, soft tissues, bones and all have the potential to modify its charac-
History joints, and giving specific attention to the teristics. Whilst information on all of these
It is necessary to collect as much information eyes, ears and mouth. The examination should factors should be sought from the care-
as possible on the events that led to the look for the following physical findings: givers it is often difficult to draw accurate

395
18.2 CHILD AT RISK

conclusions from examination of the wound The ear drum may rupture due to the air of emotional abuse have been described.
itself. Associated injuries such as bruises or pressure changes. These are the following behaviours:
fractures may help. Although classically
described as associated with non-accidental Head injuries Head injuries are a major
rejecting;
injury, cigarette burns are rare. These appear source of mortality and morbidity in non-
isolating;
as small, deep, round burns, usually on the accidental injury. Young children have a
terrorising;
limbs or back. large head to body size ratio, relatively weak
ignoring;
Healing burns can sometimes be espe- neck musculature and compliant skull bones
corrupting.
cially difficult to diagnose and interpret. that predispose them to intracranial injuries. The incidence of emotional abuse is
Inflammation can extend beyond the mar- The child may be struck, dropped, thrown or unknown but it is likely to be common and
gins of the burn, obscuring the shape and shaken, producing an open or closed head under-diagnosed.
increasing the size of the lesion. The healing wound.
area may become flaking or exudative, caus- Skull fractures, cerebral contusion, intra- History and examination
ing confusion with skin conditions such as cerebral haemorrhages, extradural haema- Presentation is subtle and depends upon the
impetigo. toma and subdural haematoma are all age of the child. An infant may present with
Scalds are a common form of accidental possible sequelae. sleep or feeding problems, irritability or apa-
injury in infants, often caused by a hot liquid thy. Older children may present with atten-
being tipped over the upper torso, arms or Intra-abdominal injury Blows to the tion deficit, attention seeking, aggression,
hands: some features of a scald may suggest abdomen may cause laceration or rupture school failure, truancy, anxiety, depression
an intentional cause. Look at the position, of either solid abdominal organs, such as and psychosomatic disease. It is likely that
shape and depth of the burn. Circumferen- the liver or spleen, or of the hollow organs, emotional abuse accompanies other forms
tial scalds of the hands or feet may be such as the duodenum. There may or may of abuse, such as physical and sexual abuse.
caused by forced immersion. Small round not be accompanying bruising of the Consideration of its possible role is impor-
bruises above the burn may represent forc- abdominal skin to alert you to this possibil- tant in all assessments of a child at risk.
ible gripping by a hand. Scalds of the but- ity. Liver function tests and amylase may be Detection by all health workers looking
tocks extending on to the lower back or helpful if this is suspected. after children, including emergency staff,
upper thighs with sparing of the natal cleft requires a high index of suspicion and vigi-
may indicate the child has been lowered into Investigations Primarily, the findings of lance. Diagnosis is suggested by the conse-
hot water. the clinical examination dictate the extent quences in the child, as above.
and type of investigations necessary. As Assessment should include that of beha-
Fractures Fractures of long bones, ribs would be the case in the investigation of vioural, emotional and physical signs and
and skull may occur when a child is inten- any injured child, plain X-rays, computerised the childparent interaction. This will usu-
tionally struck, pushed, squeezed or tomography scans, magnetic resonance ally require time not available to emergency
dropped. While any fracture may be caused imaging, ultrasound and other imaging staff and therefore, when emotional abuse is
by non-accidental injury, certain fractures should be directed at areas where there is suspected, referral is necessary. This may be
have an association with abuse that should clinical suspicion of injury. to the hospital child protection/abuse unit
alert the ED clinician and prompt further Other investigations, such as a skeletal and/or community social services.
action. Specifically, fractures in children survey and bone scan, are used in an
under the age of 18 months, rib fractures, attempt to detect injuries that may not be Neglect
metaphyseal fractures, multiple fractures clinically apparent but which will assist in Neglect may also cause the child to be at risk
and fractures of differing ages should be establishing the likelihood of non-accidental and can be difficult to define and diagnose.
carefully evaluated. injury. These are especially useful in children A broad definition is anything that indivi-
A bone scan and skeletal survey may be under the age of 2. duals, institutions or processes fail to do,
extremely useful in gathering evidence of In a child with multiple bruises, the possi- which directly or indirectly harms children
multiple injuries when a child under the bility of a bleeding disorder such as idio- or damages their prospects of a safe and
age of 2 years presents with suspicious pathic thrombocytopenic purpura should healthy development into adulthood. Other
bruising or other features of abuse. be considered. A full blood examination definitions have tended to be narrower and
and coagulation profile may be necessary therefore target more severe or persistent
Eye injuries Direct blows to the face may in these circumstances. neglect. It is important to differentiate
cause subconjunctival haemorrhages or neglect from poverty or ignorance, as these
intraocular injuries such as retinal haemor- Emotional abuse will require a different intervention.
rhages. A careful examination including Emotional or psychological abuse is difficult The true incidence of neglect is unknown
visual acuity and funduscopy is necessary. to define and even harder to detect, partic- and it is probably the most common reason
ularly in the ED, when frequently the child for the child to be at risk. Its diagnosis usu-
Ear injuries Blows to the side of the face presents to a particular health worker on a ally only occurs when harm has occurred, but
may cause bruising of and behind the pinna. single occasion. Five possible components consideration should be made of potential

396
18.2 CHILD AT RISK
18

CRISIS INTERVENTION
for harm and long-term effects. Types of symptoms in another person who is Children (Care and Protection) Act 1987,
neglect include medical neglect, safety under the individuals care. medical practitioners were required to report
neglect, educational neglect, physical The motivation for the perpetrators physical and/or sexual abuse of children
neglect and emotional neglect. Non-organic behaviour is to assume the sick role by under the age of 16 years. Mandatory noti-
failure to thrive is likely to be due to a com- proxy. fiers who failed to notify under Section 22
bination of a lack of calories and affection.  External incentives for the behaviour, of the Act were guilty of an offence.
such as economic gain, avoiding legal All healthcare staff are now mandatory
History and examination responsibility, or improving physical reporters under the Children and Young Per-
Some possible features to look out for are well-being, are absent. sons (Care and Protection) Act 1998. It is a
frequent presentation and admission to hos- criminal offence not to report.
Children at risk for MSBP abuse are aged 15
pital with accidents or illness, delay or failure Notification involves contacting the child
months to 6 years. The emergency physician
to access health care, malnutrition, failure to protection authority relevant to the state.
is often confronted with baffling symptoms.
immunise, poor physical presentation, poor The Children (Care and Protection) Amend-
Frequently, the child has been taken to many
compliance, behaviour disorders, develop- ment (Disclosure of Information) Act 1996
care providers before the diagnosis is finally
mental delay and, very importantly, failure extends voluntary reporting to any person
established. Warning signs that are sugges-
to thrive. The assessment of growth and who believes on reasonable grounds that a
tive of MSBP include the following:
development is clearly an essential part of child who is aged 16 or 17 years has been,
child health assessment, even in the emer- illness is multisystemic, prolonged, or is, in danger of being abused.
gency setting. Measurement of height, unusual, or rare; Once notification is made a process of risk
weight and head circumference and their symptoms are inappropriate or management is commenced.
plotting on standardised growth charts can incongruent;
be very useful in assessment and follow patient has multiple allergies; What to do as the medical
up. Emotional and behavioural assessment symptoms disappear when parent or practitioner, in suspected cases
is difficult in the emergency setting. caretaker is absent; Take a concise history of events and
in children, one parent, usually the father, physical examination.
Treatment is absent during hospitalisation; Document injuries and order
The diagnosis of non-organic failure to thrive history of sudden infant death syndrome appropriate investigations.
usually requires admission to hospital to in siblings is noted;  Manage injuries as usual practice.
assess the childs ability to grow with ade- parent is overly attached to patient; Notify relevant child protection agency.
quate nutrition and an interdisciplinary patient has poor tolerance of treatment (Clients consent is not required in child
approach. Other forms of neglect may require (e.g. frequent vomiting, rash, problems protection notification.)
admission or, if not, referral to child protec- with intravenous lines);
general health of patient clashes with This may be facilitated by social worker,
tion/abuse unit or community social services.
results of laboratory tests; nurses and paediatric specialists.

Munchausens syndrome
parent shows inordinate concern for
feelings of the medical staff; Legal responsibilities
by proxy
Munchausens syndrome by proxy (MSBP) is
seizure activity is unresponsive to By law, health services staff must provide
anticonvulsants and is witnessed only by all relevant information that they have
an unusual presentation of the child at risk
parent or caretaker. available when asked (in writing) to do
that may present to an ED. This condition
so by the child protection authority.
occurs when an adult, usually the mother,
presents a false history to the physician
Staff do not have to get permission of
the client in order to forward the
regarding a child who is not truly suffering The community response
relevant information.
from any of the reported symptoms. This his- to the child at risk
tory may cause the emergency physician to
 Protection for notifier. The Children (Care
Responsibilities to report and Protection) Act 1987 made provision
perform unnecessary diagnostic and thera-
For medical practitioners working in acute for the safeguarding of the identity of
peutic procedures that do not result in any
medicine, often the first point of contact with the person who makes the report.
specific diagnosis.
a child at risk is when they present for treat- Neither the report nor its contents are
In 1995, the Diagnostic and Statistical
ment. Statistics suggest that doctors only admissible as evidence in any proceedings
Manual of Mental Disorders, 4th edition-
diagnose cases in about 2% of notifications. against the person who made the report.
included a definition for factitious disorder
by proxy, which is now the accepted psychi-
Mandatory reporting legislation brought in If, as a result of making a report, a
across all states of Australia has made it person is threatened or fears personal
atric category for MSBP. The definition
compulsory to report cases of non-accidental violence, this should be reported to
includes the following:
injury and suspected cases of neglect to the the police, who may apply for, and
Intentional production or feigning of State Child Protection Agency. For example, pursue on their behalf, an apprehended
physical or psychological signs or in New South Wales under Section 22 of the violence order.

397
18.2 CHILD AT RISK

What happens after notification? Actions based on risk child be placed in agency care for a
The Child Protection Agency must assessment period of time. The court can also
respond when someone reports that If the child is in immediate danger, order counselling and other types of
they think a child or young person under steps will be taken to reduce the level of support services including health
the age of 16 years has been, or is being, risk or move the child to a safe place. services.
injured or neglected. This may mean admission to hospital or
When the agency receives information foster care.
about non-accidental injury, it makes In many cases, this might mean giving
decisions about how to go ahead with the family practical help, such as Further reading
investigating the claims and how others organising child care, emergency Browne K, Hanks H, Stratton P, Hamilton C. Early prediction
and prevention of child abuse: A handbook. Chichester:
may be able to help. For example, it may finance, providing a referral for John Wiley & Sons; 2002.
contact the childs teacher, child-care counselling or information on health Gabarino J, Guttman E, Seeley J. The psychologically battered
child. San Francisco: Jossey-Bass; 1988.
worker, relatives or the police. or other services. Hobbs C, Hanks G, Wynne J. Child abuse and neglect:
 The police will be contacted and may  In some cases, the child protection A clinicians handbook. London: Churchill Livingstone;
1999.
become involved if the agency thinks the agency takes the matter to the Oates KR. The spectrum of child abuse: assessment, treatment
law has been broken. Childrens Court. The court can order a and prevention. New York: Brunner/Mazel; 1996.

398
 19

SECTION
ADMINISTRATION
AND EMS
Section editor Gary Browne

19.1 Managing the death of a child in the ED: 19.2 Forensic paediatrics and the law 404
Bereavement issues 399

19.1 Managing the death of a child in the ED:

Bereavement issues
Paul Tait Roger Barkin Pat Clements

of self, the grief response of parents may be

ESSENTIALS very painful and prolonged. The death of a
child must be viewed as a tragedy for the
1 The death of a child under any circumstances is likely to lead to a significant crisis entire continuum of family and friends. Addi-
and grief response in parents.
tionally, paediatric deaths are frequently per-
2 Emergency physicians should be prepared for parental presence in the sonalised by ED staff, and hence have broad
resuscitation room, anticipate their high level of distress, and ensure that they are implications for the whole ED clinical team.
kept informed. In large hospital EDs, particularly in urban
areas, there is rarely a pre-existing relation-
3 It is important that the family knows that everything that could have been done ship between the health professionals and
was done.
the patient/family. While this facilitates the
4 Parental questions should be answered honestly and directly, allowing humanity professional detachment needed for ED staff
and empathy to show. to function effectively, it creates inherent voids
in the ability to support grieving relatives and
5 Personal, compassionate and individualised support should be provided for friends. In smaller hospitals like those found in
families, respecting their cultural, religious and social values.
rural and regional communities, a pre-existing
6 Family members are likely to have impaired decision making and communication relationship may exist, potentially lowering
abilities, and this needs to be taken into consideration around informed consent issues. communication barriers but bringing out
other stresses and strains for ED staff.
7 The needs of the grieving family must be balanced with the legislative Good communication with family mem-
requirements of the Coroners Act when this is relevant.
bers must be established early and main-
8 Relatives should be allowed to spend time with the deceased child if they want tained throughout. This is best left to an
to, preferably in a quiet suite. experienced member of the staff. There is
evidence to suggest that junior medical staff
9 It is important to be available in the weeks following the death to clarify and do not feel adequately trained in talking
answer any further questions from the family.
with parents in regards to end-of-life care
10 Team members need to be aware of their own likely emotional responses to the matters.4 Due consideration for the comfort
death of a child. of the family should be at the forefront of
the minds of clinical staff at all times.

The unexpected death of a child undoubt-

Introduction The resuscitation process
edly brings about the most severe and shat-
Deaths occurring in the emergency depart- tering grief response for the childs parents.3 Parents usually benefit from being pre-
ment (ED) present unique challenges for the Because the loss is unexpected and involves sent during the resuscitation process.5 It is
clinician, particularly if the patient is a child.1,2 someone so young and so intrinsically a part therefore unacceptable to discourage their

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19.1 MANAGING THE DEATH OF A CHILD IN THE ED: BEREAVEMENT ISSUES

presence unless they are interfering with, appropriate language. Answer questions It is important to allow parents and family
and compromising, the resuscitation itself. and be responsive to needs and concerns. members time to examine the implications
Family members watching monitors and see- When death has occurred, or is imminent, of the loss, and to begin the process of
ing the trace go flat experience much alarm it is essential to have identified the relevant searching for some answers and meaning
and distress, but this should not be seen as a family members so that discussions are with in the midst of the event. It is also important
reason to exclude them.6 the appropriate individuals. At the point of to assist them to mobilise resources from
The resuscitation process can be trau- death, the medical officer in charge of the their social, cultural and religious commu-
matic for parents and family members, resuscitation should advise those family nities to help them to deal with their grief.
requiring ongoing communication and inter- members present in the resuscitation room There can be a temptation to offer seda-
pretation of events. It should be expected or in a private, quiet location. Research tion to grief-stricken parents. This is often
that parents will be visibly upset and dis- has indicated that families appreciated a requested by relatives distressed by observing
tressed during this period. A staff member, high level of physician involvement.8 the parents pain. Grief is a normal process,
often a social worker, should be assigned Clear, distinct and accurate information which is rarely helped by pharmacological
to support the family, to answer any ques- is essential, and medical jargon should be intervention.
tions about the procedures and responses, avoided. It is very important to state ini- Junior medical staff are often involved in
and to prevent distraught family members tially that the child has died. This is the resuscitations, and it is essential that they
from impeding the resuscitation.7 The ED piece of information that the parents will have received some training/education to
medical officer in charge must communicate most want clarified. It is then desirable help them handle the unexpected death of
with this staff member and family members to provide a brief chronology of events, a child. A number of programs have been
about the progress of the resuscitation. while reassuring the family that every- described, which have been found to be use-
Viewing the resuscitation efforts allows thing was done and that the child did not ful in preparing staff to deal with loss in an
the family to see a caring and competent suffer pain. effective manner, from the perspective of
staff, in control of their emotions, doing their Sometimes family members are not pres- both the family and staff members.1114
best to save the childs life. ent at the time of death. If practicable it is
Where parents choose not, or feel unable, best to delay notification of death until it
to be in the resuscitation room, it is essential can be done in person.9 If the family cannot
Laying out of the child
that they be kept informed of progress. Panic, readily access the ED, telephone notification
fear and a sense of isolation have been noted may be necessary. A survey of survivors sug- Where parents want to view or spend time
as the main responses of relatives who remain gested that if delay in personal notification with their deceased child, it is important to
outside the resuscitation room.6 Small, dull was greater than 1 hour, telephone notifica- facilitate their wishes (having due regard
rooms with no windows or natural light were tion may be appropriate.10 However, it is to the possibility that the death may need
seen as heightening the sense of isolation, obviously difficult to be sensitive to the to be referred to the coroner and hence care
disconnectedness and fear for those family familys response via a telephone, and there not to interfere with evidence). All tubes
members unable to bring themselves to view may be limited ability to provide immediate inserted during the resuscitation process
the resuscitation. support. Ensure that the family is safe to (endotracheal tubes, intravenous cannulae,
It is important to be skilled in early recog- transport themselves and that ongoing drains, etc.) should be removed, unless the
nition of the signs of trauma responses by support options have been explored for medical officer in charge considers that
parents, such as dissociation, as this can those family members unable to make it the placement of a tube may have been
affect long-term adjustment. A social worker to hospital. associated with an adverse event. All
or other designated professional should If family members were not present at the wounds and cannula sites should be dressed
ideally be available to provide support for hospital it is likely that they will have many to avoid leakage of bodily fluids. The childs
parents and act as an advocate during what questions related to the process, potential face and exposed areas should be bathed/
is likely to be an overwhelming and bewil- suffering, and any awareness by the child cleaned and any soiling removed.
dering process. The social worker is also of the event. These may be asked either over The impact of the death can often cause
likely to be the main staff member to have the telephone or upon arrival. If parents an overwhelming sense of numbness and
an ongoing role after death has occurred arrive too late, this can create a further helplessness, diminishing the ability to self
and the family has left the hospital. burden of guilt because they were not advocate. Therefore, it is important to be
present. proactive with family members and ask
Family members experiencing significant how much they want to be involved with
grief are likely to struggle with the integra- the bathing and laying out of the child,
Talking to parents and
tion of the information that they are being and about any specific cultural or religious
families given and with the communication of any practices that they would like observed.
When talking with the family about the questions that they might have. They may It can often be useful to obtain mementos
childs deteriorating condition, give details need to revisit the same questions and infor- of the child. Photographs, a lock of hair, or a
in a simple, straightforward and accurate mation repeatedly in order to try to make foot/hand print may become important
manner. Provide the information using sense of the event.6 mementos along the grieving journey. It is

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19.1 MANAGING THE DEATH OF A CHILD IN THE ED: BEREAVEMENT ISSUES
19

ADMINISTRATION AND EMS

recommended that hospital EDs have access Perhaps the most well-known model of will be less emotionally available. The cogni-
to such items as a camera, memento books describing the process of grief is the stages tive developmental level of a sibling has a
and bereavement packs to give to families. model with its clearly defined stages of significant bearing on their capacity to
There are specific requirements in place shock, denial and isolation, anger and envy, understand concepts of death like perma-
for deaths that must be referred to the coro- bargaining, depression and acceptance.17 nent, irreversible, inevitable, universal.28
ner. These may limit the process of laying These stages should not be seen as linear Regardless of how siblings understand and
out the body, and require that family mem- or rigid. Individuals can move back and forth express their grief, it is critically important
bers may not be left unsupervised with the between the stages or may appear stuck to remember that they are part of the social
child. ED staff need to balance the needs in a stage. Although the stages model is context in which the death has occurred.
of grieving family members with their legal the most well known and can be a useful Their needs for explanation and support
responsibilities to the coroner. guide, there are a number of other models are just as important as the needs of their
of grieving including psychodynamic,18 parents.
attachment,19,20 social constructionist,21 The death of a child does not occur in iso-
cognitive/behavioural,22,23 and personal lation, but rather it occurs in a social context
Viewing the body quiet
construct.24 Good practice requires being that includes many variables. The main ones
suite open and flexible, adapting to the needs are parental coping capacity and skills,
Most available evidence strongly suggests of the grieving family as opposed to trying family and relationship functionality, social
that seeing the body of the deceased is an to fit the family into any particular model. networks, parental physical and mental
important part of accepting the reality of It is important not to pathologise individuals health issues, education, socioeconomic
death.15,16 This includes not only seeing, whose grief response does not fit neatly into status, and, importantly, any real or per-
but also being able to touch and hold the a particular model of grief.25 ceived parental responsibility in the death
loved one. It is helpful to describe to rela- The death of a child provokes the most of the child. Thus the broader social context
tives what they are going to see prior to intense form of bereavement. It is certain will have relevance to how parents and
viewing the body, especially if there are to alter the course of the parents lives, their extended family members manage the
trauma-related injuries.6 Viewing the body relationship with each other and with others. impact of the childs death.26 Any available
can also relieve anxieties about mutilation, Losing a child is more than losing a relation- psychosocial assessment or information,
signs of trauma, or that the person was in ship. For a parent it is losing part of their self, such as that provided by the ED social
pain when they died.16 A parent or family their present and their future. Many parents worker, should be factored into the manage-
members preference not to spend time with experience a loss of meaning in their lives ment of the family.
the child should also be respected.. and may never fully recover from the impact
Most large paediatric hospitals have a of their childs death.20 A childs death is not
quiet suite or family room to facilitate par- a singular loss, but produces a ripple effect
overwhelming all aspects of the family and
Support of the family
ents spending time with their deceased
child. This can allow a private good bye environment. Parents, and even the Generally, parents are completely unpre-
and time to reflect. It can also allow time extended family, may feel that they have pared for the impact of their childs death
to create an image of the child as dead, failed, irrespective of the nature of the death as they have no prior knowledge or experi-
altered from the image of the living child.16 and level of love, nurturing and caring that ence to draw on.29 Arranging support is
The importance of the family/relatives existed during the childs life.16,26,27 essential, and early social worker involve-
room cannot be overemphasised privacy The parental relationship faces severe ment is highly desirable. Parents and other
and basic facilities are essential. stress following the death of a child. It can family members must be provided with infor-
Subsequently, additional relatives/ friends pull a dysfunctional relationship further mation about normal grieving, and should
may arrive at the hospital. This can often lead apart, or glue a functional one closer be linked to appropriate resources. This
to heightened distress for parents as they try together. Adverse impacts on the relation- can take the form of written information
to explain the events that have led to the ship can occur through the real or perceived packs that parents can take away, and which
childs death, hence taking them back to apportioning of blame by one parent to the they may choose to read at a later time.30,31
the initial traumatic stages. It can also be a other. This can occur where a child died Referral information should be readily avail-
useful process. By reviewing events, parents while under the specific supervision of one able for support groups with particular
may build a clearer picture and fill in the parent, or one parent was simply not present expertise relating to the death of a child
blanks as they retell their story. when a critical event occurred. such as Sids & Kids,32 SANDS Australia,33
Siblings of the deceased child will also Compassionate Friends,34 and other rele-
experience a significant grief reaction. Not vant organisations. The extent of involve-
only must they manage the actual loss of ment of support by ministers of religion
The grief response their deceased sibling, but they must also will depend on the wishes of and the reli-
Grief is a normal reaction accompanying cope with the loss of their normal family gious commitment of the family. Ideally
death. The severity of the grief response par- environment. Their parents will be strug- there should be a protocol that facilitates
allels the severity of the loss. gling to cope with their own grief, and thus ready access to this material.

401
19.1 MANAGING THE DEATH OF A CHILD IN THE ED: BEREAVEMENT ISSUES

Practical assistance with arrangements After a year, there may be some ritual unsupervised with the body. It is desirable
at the time of the childs death, including (i.e. tombstone opening) associated with for the family to formally identify the childs
organising family support, funeral and finan- the end of the mourning process, which body in the presence of the police. Other-
cial assistance, should be offered to families requires community members to return wise identification will have to be performed
as appropriate, while being sensitive to home even if in hospital themselves. later and probably at the morgue, a process
the social and cultural environment of the likely to increase family distress. All medical
The Maori culture of New Zealand tradition-
family. notes, investigations, observation sheets,
ally has family members present with the
etc., should be provided to the police when
body from the time of death through inter-
they depart with the childs body for the
ment. This maintains the harmony of the
Cultural implications morgue. Full and accurate documentation
child, assisting the decedent to join their
of all events in the patients hospital chart
Many cultures have specific rituals and prac- ancestors. Family members will want to be
is essential. This should include the date
tices concerning death. It is critical to listen part of the laying out of the body, washing,
and time of death, the observations that
to the family members and be guided as dressing, etc.
specify that the child is clinically deceased,
much as possible by their requests. Some Other practices reflecting different cul-
any relevant history surrounding the circum-
of these rituals may require modification tural belief systems that may need to be
stances of the childs death and any relevant
when the death of a child has been referred considered include parents needing to
conversations held with the parents or fam-
to the coroners office. Sensitivity is essential. remain with the body 24 hours after the
ily members. There are potentially legal
It is difficult to make broad statements death, caring of the body by staff of the
consequences following any death and the
about the cultural practices related to death same gender as the deceased child, laying
forensic issues need to be considered. For
and dying in indigenous (Aboriginal and the body to face a certain direction (Mecca),
example, child abuse remains an important
Torres Strait Islander) communities, because special roles for specific religious/spiritual
cause of deaths in infancy.
across Australia there are different practices leaders, and the burning of incense/candles.
and rituals. Examples of the kinds of cultural When working with families from differ-
practices and rituals to be aware of include: ent cultures following the death of a child,
it is important to be guided by custom, rit- Organ and tissue donation
When a child is dying many families will and collection
ual, experience, and the familys cultural
want any extended family present to be
environment.
in attendance. Organ donation (e.g. heart, lungs, liver,
During the grieving process pre- and post- kidneys) requires intact cardiorespiratory
death, loud crying/wailing may need to function but brain death. Because of the pre-
occur as part of the communitys customs.
Legal issues conditions required by Transplant Acts
Privacy in these circumstances is Each state and territory will have subtle var- before brain death can be declared, organ
desirable. iations as to the legal requirements for the donation discussions are commonly deferred
Senior female or male figures may documentation and handling of the body until admission to the intensive care unit.35
wish to take a lead role in mourning of a deceased. An up to date protocol should Tissue donation (e.g. corneas, heart valves)
rituals after death has occurred. This be available to ensure that proper proce- can occur from cadavers, and hence theoreti-
can include ceremonial cleansing dures are followed. cally this issue could arise for children who die
(washing the childs body), dressing A life extinct form will need to be com- in the ED. However, deaths of children in the
and handling. pleted by one of the attending ED medical ED are usually coroners cases. For parents
Funeral arrangements may have to be staff. However, it will also be necessary to faced with the extreme distress of the sudden
organised by a specific extended family decide whether or not a death certificate death of a child and the need for coroners-
member or a senior member of the can be completed. If the patient was case status, it may be potentially too distres-
community. known to the hospital and the death was sing to parents for ED staff to raise the further
House smoking (burning leaves in order not unexpected, the childs usual physician issue of tissue donation in this setting. This
to prevent the spirit from rising up) may may be prepared to sign a death certificate. can come a little later at the Forensic Pathol-
need to occur relatively quickly after This physician may also discuss with the ogy Institute level, when parents have had a
death has occurred. This can mean that parents the option of performing a hospi- little time to regain some degree of compo-
the family will want to return to their tal-based autopsy. sure and hence may be better able to give
community quickly. Usually the death of a child in the ED is informed consent. On the rare occasion when
Many communities forbid the use of the not anticipated, and hence becomes a cor- the issue of tissue donation is spontaneously
deceased persons name after death (for oners case (see Chapter 19.2 on Forensic brought up by parents in the ED setting, con-
up to one year). Nicknames or aliases may paediatrics and the law). For a coroners case, tact with the transplant coordinator can be
be used after death has occurred. A family only a life extinct form can be completed, initiated if there are no potential medical con-
member with the same name as the laying out of the body will be restricted traindications to tissue donation. Consent by
deceased may use their second name to spot cleaning, the local police must be the coroner must be obtained prior to tissue
during the mourning period. notified, and parents must not be left removal.

402
19.1 MANAGING THE DEATH OF A CHILD IN THE ED: BEREAVEMENT ISSUES
19

ADMINISTRATION AND EMS

When children die suddenly and unex- failed to reduce the incidence of post-trau- 6. Wright B. Sudden death: Intervention skills for the caring
professions. New York: Churchill Livingstone; 1996.
pectedly there may be merit in considering matic stress disorder but actually increased 7. Tsai E. Should family members be present during
collecting perimortem samples in order to the risk of developing it.37 In addition, there cardiopulmonary resuscitation? N Engl J Med
2002;346:101921.
obtain as much information as possible. This was no evidence of reduction in general psy- 8. Scott JL, Sanford SM, Strong L, Gable K. Survivor
might include urine and blood for metabolic chological disturbance, depression or anxi- notification of sudden death in the emergency
department. Acad Emerg Med 1995;2:4089.
profiling, genetics screening and other pos- ety. This is an area where more research is 9. Stewart AE. Complicated bereavement and post-
sible investigations such as liver of other tis- required. traumatic stress disorder following fatal car crashes:
Recommendations for death notification practice. Death
sues samples that may contribute to the Stud 1999;23:289321.
understanding of cause of death. This will 10. Leash RM. Death notification: Practical guidelines for
health care professionals. Crit Care Nurs Q
depend on location and is more likely to Conclusion 1996;19:2134.
be valuable in a major centre where appro- 11. Schmidt TA, Norton RL, Tolle SW. Sudden death in the ED:
Educating residents to compassionately inform families.
priate pathology facilities are immediately The death of a child has the most profound J Emerg Med 1992;10:6437.
available. effect on parents, family and friends. It can 12. Bagatell R, Meyer R, Derron S, et al. When children die:
A seminar series for paediatric residents. Pediatrics
also have a profound effect on staff involved 2002;110:34853.
in the resuscitation process. It requires the 13. Swisher LA, Nieman LZ, Nilsen GJ, Spivey WH. Death
notification in the emergency department: A survey of
Debriefing and support sensitivity and strength of clinical staff to residents and attending physician. Ann Emerg Med
for ED staff help relatives through this difficult time 1993;22:131923.
14. Rutkowski A. Death notification in the emergency
and to assist in the initiation of a healthy department. Ann Emerg Med 2002;40:5213.
Much of what is written about the family grieving process. A thoughtful and sensitive 15. Jones WH, Buttery M. Sudden death. Survivors
grief reactions applies equally to the ED approach is likely to have profound and pos- perceptions of their emergency department experience.
J Emerg Nurs 1981;1:7.
staff and due consideration of staff reactions itive long-term implications for all those 16. Raphael R. The anatomy of bereavement: A handbook for
is very important. A healthy approach is to impacted upon by the death of a child. the caring professions. London: Hutchinson; 1984.
17. Kubler-Ross E. On Death and Dying. London: Tavistock;
factor the reality of day to day exposure of 1970.
grief and loss into the culture of a busy 18. Freud S. Mourning and melancholia. Standard Edition XIV.
London: Hogarth Press; 1917.
ED. There is a paucity of literature on the 19. Bowlby J. Attachment and loss. Vol. 1. Attachment.
reactions of staff and grief management Acknowledgements London: Hogarth Press; 1969.
20. Parkes CM. Bereavement: Studies of grief in adult life.
among ED staff members. The authors gratefully acknowledge the London: Tavistock; 1972.
Identifying abnormal psychological symp- assistance of the Indigenous Liaison Service, 21. Glick IO, Weiss RS, Parkes CM. The first year of
bereavement. New York: John Wiley & Sons; 1974.
tomatology in ED staff (flashbacks, sleep Herston Hospitals Complex, Brisbane. 22. Attig T. The importance of conceiving of grief as an active
disturbance, bad dreams, absenteeism, process. Death Stud 1994;15(4):585647.
23. Worden JW. Grief counselling and grief therapy:
detachment, intensified emotions, etc.) and A handbook for the mental health professional. London:
making ongoing psychological counselling Controversies Routledge; 1983.
24. Neimeyer RA, Neimeyer GJ. Advances in personal
available to affected staff is clearly impor- There remains some controversy construct psychology. Science & Technology Books.
tant. Such symptomatology may occur as a about actively encouraging parents to be
New York: Jai Press; 1997.
25. Dubin WR, Sarnoff JR. Sudden unexpected death:
result of either a single exposure or cumula- in the resuscitation room. Intervention with the survivors. Ann Emerg Med
tive exposures to traumatic situations. It is 1986;15:547.
important for senior ED staff to promote There is little evidence to support the 26. Murray J. Loss as a universal concept: A review of
literature to identify common aspects of loss in diverse
the concept of self-care, to guarantee confi- widespread practice of mandatory single- situations. J Loss Trauma 2001;6:21941.
session psychological counselling of 27. Murray J. Children, adolescents and loss. Loss and Grief
dentiality to staff experiencing problems, Unit. Brisbane: University of Queensland; 2002.
and to ensure staff are made aware of distressed staff who attended the child. 28. Murray J. Understanding loss in the lives of children and
adolescents: A contribution to the promotion of well
counselling options available to them  Raising the issue of tissue donation being among the young. Aust J Guid Counsel 2000;
should they experience problems.36 with parents in the ED setting is difficult.
10(1):95109.
29. Heiney S, Hasan L, Price K. Developing and implementing
Performing an operational debriefing of a bereavement program for a childrens hospital. J Pediatr
the resuscitative process with a view to clar- Nurs 1993;876:38591.
30. Johnson L, Rincon C, Gober C, Rexin D. The development of
ifying events for attending staff and identi- a comprehensive bereavement program to assist families
fying areas for potential improvement is References experiencing paediatric loss. J Pediatr Nurs 1993;8:3.
1. ACEP. Death of a child in the emergency department: 31. Murray J. An ache in their hearts. Brisbane: University of
essential. A joint statement by the American Academy of Pediatrics Queensland Press; 1993.
The same cannot be said for psychological and the American College of Emergency Physicians. 32. SIDS and Kids www.sidsandkids.org.
Ann Emerg Med 2002;40:40910. 33. SANDS Australia www.sands.org.au.
debriefing sessions. It has become a popular 2. Olsen JC, Buenefe ML, Falco WE. Death in the emergency 34. Compassionate Friends www.thecompassionatefriends.
and widespread practice to conduct single department. Ann Emerg Med 1998;31:75865. org.au.
3. Seecharan GA, Andersen EM, Norris K, Toce SS. Parents 35. Rivers EP, Buse SM, Bivins BA, et al. Organ and tissue
session psychological counselling for person- assessment of quality of care and grief following a childs procurement in the acute care setting: Principles and
nel attending traumatic critical incidents. death. Arch Pediatr Adolesc Med 2004;158:51520. practice, part 1. Ann Emerg Med 1990;19:7885.
4. McCabe ME, Hunt EA, Serwent JR. Pediatric residents 36. Everly GS, Flannery RB, Mitchell JT. Critical incident stress
ED staff in attendance at an unsuccessful clinical and educational experiences with end-of-life care. management: A review of the literature. Aggr Violent
resuscitation fit into this situation. A recent Pediatrics 2008;121(4):e7317. Behav 1999;5(1):2340.
5. Doyle CJ, Post H, Burney RE, et al. Family participation 37. Rose S, Bisson J, Wessely S. Psychological debriefing for
Cochrane Review concluded that single ses- during resuscitation: An option. Ann Emerg Med 1987;16 preventing post traumatic stress disorder (PTSD).
sion psychological debriefings have not only (6):6735. Cochrane Database Syst Rev 2002;(2):CD000560.

403
 19.2 Forensic paediatrics and the law
Maree Crawford Natalie Phillips

to develop an understanding of the mecha-

ESSENTIALS nism of an injury, and the nature of the
forces required. This then allows consider-
1 Emergency physicians will frequently be involved in the area of forensic medicine ation and evaluation of the provided history
and hence require skills and knowledge of their responsibilities with regard to the
to determine whether it adequately and
legal system.
plausibly accounts for the injuries seen. An
2 Thorough history, examination and documentation provide the basis for well- opinion can then be formulated that an
prepared legal reports. This, in turn, greatly assists in the presentation of evidence injury is truly accidental, resulting from an
to courts. unpredictable or unavoidable event; that it
has been inflicted or imposed on the child;
3 Emergency physicians who deal with children must be aware of their legal or that it is accidental but involves caregiver
obligations with regards to reporting of child abuse, reporting of criminal matters
neglect, or failure to protect from harm.
and management of deaths.
As in all areas of medicine, a forensic medi-
4 Doctors providing evidence in court must be seen to be independent, and cal opinion must be based on the information
competent within their level of expertise. available to the clinician. This is obtained
using standard clinical tools of history, exami-
nation and investigation, but also incorpo-
rates additional standards and procedures
in their investigation of such an event. There and may require more expansive investiga-
Introduction are also obligations with regard to the man- tions to consider all differential diagnoses.
The emergency department (ED) is the ini- agement and notification of deaths to the
tial point of contact for many children pre- state coroner. Accurate history and examination
senting with acute injuries. While the By virtue of this front-line contact, emer- and appropriate investigation
majority of injuries are accidental, within gency physicians will at times be required The following points may assist in forensic
this large group will be a number of children to provide evidence in a variety of legal jur- matters.
with inflicted injury or with findings result- isdictions. Familiarity with these systems,
ing from abuse or neglect, on whose behalf good preparation, and a neutral unbiased Physical injuries
legal proceedings may be initiated in a vari- approach will allow doctors to discharge Key questions that should be asked and
ety of legal jurisdictions. Hence ED staff their duty to provide the courts with accu- recorded in all cases of injury include:
require some expertise in forensic medicine rate representation of facts and balanced Who was present at the time of
or medicine as it relates to the law. expert opinion. injury?
The role of medical staff in the ED is to When and where did it occur?
carefully assess and treat children with such How did it occur?
injuries. A number will present with a clear
Forensic medical What happened after the injury?
history or findings to indicate that injury It is important to document from whom
or abuse has been inflicted. Many others will
assessment each part of the history is obtained and
present with non-specific injuries or findings Forensic medicine, also known as medical any differing accounts. Any explanation
that will not be flagged as abusive, but at a jurisprudence, deals with the interaction of that the child gives for the injury should
later date may evolve to require legal medicine and the law. All specialities or be recorded.
involvement. It is therefore essential that areas of medicine will have overlap with  A childs developmental abilities should
doctors in the front line adopt high stan- forensic matters at some time, and this is be evaluated to ensure that any actions
dards of history, examination and documen- particularly so in paediatric emergency med- the child has allegedly taken are within
tation for all cases of injury, both to allow icine. The aims of a forensic medical assess- their developmental ability, e.g.
detection of abuse and also to meet the ment are diagnosis and management that standing and turning on hot-water taps.
standards required should a matter progress incorporates the requirements of the legal Any injury must be accurately described
into the legal system. system. The qualities required include preci- including site, colour, size and pattern.
Medical staff have a number of legal obli- sion and thoroughness, objectivity, neutral- Accurate terminology for injuries should
gations in such matters. These include man- ity, clarity of thought and expression, and be used e.g.:
datory notification of suspected abuse or logical formulation of opinion. Abrasion superficial denuding of
neglect, a need to advise the police of a Forensic paediatric medicine largely the skin confined to the epidermis,
potential criminal matter and to assist police revolves around the assessment of trauma often called a graze or scratch

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19.2 FORENSIC PAEDIATRICS AND THE LAW
19


ADMINISTRATION AND EMS

Bruise or contusion extravasation of Additional testing may also be are an excellent aid and should be used.
blood into surrounding tissue caused indicated to exclude differential Any injury thought to be suspicious of being
by blunt trauma diagnoses and to assess for other inflicted or of being caused by abuse should
Laceration tearing wound through factors that may impact on the extent be photographed. This can be done using
the full thickness of the skin, or of any injuries for a given history hospital photographic services or ED photo-
through other tissues and organs e.g. testing for bleeding tendency graphic equipment if available. However, if
caused by blunt trauma (see Table 19.2.1). there are suspicions of abuse, staff will
Incised wounds sharply cut injuries Specific forensic sampling may be be notifying child protection agencies and
from any object with a cutting edge, required in some instances, e.g. the resources of the police may be used
e.g. stab or slash wounds. toxicology, swabbing for DNA in for photography. These are usually of higher
If an injury or injuries are present, suspected bites, or specimen collection quality, and police involvement overcomes
there must be careful examination in sexual abuse. Child protection or problems of confirming a chain of evidence
for other abnormalities that might forensic physicians should be involved, if the material is later used in a legal setting.
not be immediately obvious, e.g. a and a clear chain of evidence must be Notes made at the time of consultation will
child with facial bruising should maintained from clinician to police to provide the doctor with information from
have careful examination of mouth forensic pathologist. which to prepare a statement or report for a
and ears. court if this is required at a later time (some-
In addition to a childs injuries, notice Other circumstances times years later). They also will assist the doc-
tor in providing evidence if asked to appear in
should also be taken of their general Sexual abuse allegations should be
presentation, appearance and court proceedings. For this reason documenta-
discussed with a child protection
demeanour, and of any non-concerning tion must be detailed and accurate.
paediatrician as soon as possible
injuries or skin markings. and generally prior to any further
 When ED medical staff encounter assessment and physical Opinion formulation
injuries or findings that are suspicious of examination. The next step is to infer the mechanism of the
abuse or neglect they should, in the first Child protection concerns not specifically injury, if possible, from the clinical findings,
instance, involve more senior staff, related to injury may arise in an ED e.g. a patterned bruise is due to blunt trauma
either a senior paediatric emergency context. These may include concerns of from contact with a specific object, and
physician, or a child protection suspected emotional/psychological posterior rib fractures are due to chest com-
paediatrician, for guidance. abuse, factitious illness and neglect e.g. pression. Frequently there are no discriminat-
 In consultation with senior staff, non-organic failure to thrive, significant ing features to assist with determining the
further testing for occult injury may be hygiene concerns, failure to attend to mechanism, e.g. a child with multiple non-
required when injuries or findings are medical needs, concerning parentchild specific bruises. In all cases the adequacy of
suspicious for abuse or neglect e.g. interactions. the parent or carers explanation of the injury
skeletal survey in infants with bruising must be considered.
to look for occult fractures, or Any opinions as to causation and mecha-
funduscopy with dilated pupils in Documentation nism must be based on the medical findings
infants with rib fractures, looking Both history and examination findings and their compatibility with the history, and
for retinal haemorrhages should be recorded clearly, and in detail. not on extraneous factors, e.g. adverse psy-
(Table 19.2.1). Accurate printed diagrams of body parts chosocial history. Additional information

Table 19.2.1 Further investigations

I. For occult injury

Skeletal survey: to examine for occult fractures. Generally indicated in any child under 2 years of age where inflicted physical injury is suspected. This may be done
at older ages in specific cases. A second skeletal survey, 1114 days after the original, may be helpful in detecting healing fractures not easily visible in the very acute
phase.
Funduscopy with dilated pupils: to examine for retinal haemorrhage and define nature and extent. Generally indicated if suspected shaking, or concerns for
inflicted head and neck trauma.
Neuroimaging: CT is generally indicated in the acute and hyperacute settings, with MRI preferred in the subacute and chronic phases, after suspected injury. MRI is
increasingly sensitive for intraparenchymal injury and often allows more accurate delineation of subacute and chronic haemorrhage.
Bone scan: may be indicated to examine for acute fractures (prior to callus formation). Any positive findings still require confirmatory X-rays at a later date.

II. For underlying medical conditions:

Full blood count and film, coagulation screen
Extended coagulation screen: may include von Willebrands factor testing, factor assays, platelet function tests. These tests should be discussed with a
haematologist prior to collection.
Further testing as advised and according to injury e.g. calcium, phosphate, PTH, vitamin D, urine metabolic screen including organic acids, copper, ceruloplasmin,
bone mineral density imaging, skull X-ray for wormian bones.

CT, computerised tomography; MRI, magnetic resonance imaging; PTH, parathyroid hormone.

405
19.2 FORENSIC PAEDIATRICS AND THE LAW

from site visits may be provided by Police wellbeing. In many states, it is mandatory to surrounding the death. The coroner has
investigators and should be incorporated in report not only actual suspected harm but also the responsibility to investigate and report
formulating an opinion. Opinions must be log- significant risk of harm. Practitioners should on these matters.
ical and not based on speculation. An attempt be familiar with the requirements and
should be made to differentiate whether inju- mechanisms for notification within their state
ries are truly accidental, accidental but with a and locality. Doctors are free of any liability if
component of neglect, or inflicted. In many such reports are made in good faith. Legal jurisdictions
cases, no definite conclusion can be drawn.
At times, emergency physicians will be
Notification of suspected criminal involved with children whose injuries or
matters other findings will result from physical
Legal obligations If a medical officer considers that an injury is assault or abuse, sexual assault or abuse,
Mandatory reporting of child not accidentally caused or that a crime has or emotional abuse or neglect. Some of
abuse been attempted or completed, there is also these cases will end with involvement in
Medical practitioners in all states and terri- an obligation to notify the police. All the legal system in various jurisdictions.
tories of Australia are now mandated to notify involved individuals, including medical prac- Inflicted injury in which there is a single
suspicions of abuse and/or neglect to the rel- titioners, are required to assist police in their alleged offender may lead to criminal
evant statutory authority in their state, investigations. charges of assault or of torture. The child
although the forms of abuse/neglect to which protection statutory agency may look to pro-
mandatory reporting applies vary bet- Notification to the coroner vide safety for a child and their siblings via a
ween states e.g. only sexual abuse in Western of deaths under certain range of orders in the Childrens Court or
Australia, sexual/emotional/physical abuse circumstances information may be sought regarding a
and neglect in New South Wales Medical practitioners are required to notify childs well-being by the Family Law Court
(Table 19.2.2). Definitions of abuse com- the coroners office of deaths that occur in in matters of contested custody or residency
monly relate to the concept of harm, which, a variety of circumstances. These include, between parents.
for example, under the Queensland Child Pro- in broad terms, situations of any sudden, To assist medical practitioners in their
tection Act 1999, is defined as any detri- unexplained death, situations where cause interaction with the legal system a brief
mental effect of a significant nature on the of death is unknown, and situations where outline of the different court systems
childs physical, psychological or emotional there are any suspicious circumstances follows.

Table 19.2.2 Mandatory reporting requirements for medical professionals (2009) in children and young people by state

State Maltreatment types for which Legislation Statutory agency

mandatory reporting applies

Australian Capital P, S Section 356 of Children & Young People Act Office for Children, Youth & Family Support-Dept
Territory 2008 (ACT) of Disability, Housing and Community Services

New South Wales P, S, E, Neglect, Exposure Section 23 & 27 of Children & Young Person Department of Community Services
to family violence Act 1998 (NSW)

Northern Territory P, S, E, Neglect, Exposure Section 15 & 26 of Care &Protection of Children, Youth and Families- Department of Health
to family violence Children Act 2007 (NT) and Families

Queensland P, S, E, Neglect, Sexual Section 191-192 & 158, Public Health Act Child Safety Services - Department of Communities
exploitation 2005 (QLD)

South Australia P, S, E, Neglect Section 11 of Childrens Protection Act 1993 Families SA- Department of Families &
(SA) Communities

Tasmania P, S, E, Neglect, Exposure Section 13 & 12 of Children, Young persons Child Protection Services Department of Health
to family violence and their Families Act 1997 (Tas) and Human Services

Victoria P, S Section 182 (1) a-e, 184 and 162 c-d of the Child Protection and Family Services- Department
Children, Youth and Families Act 2005 (Vic) of Human Services

Western Australia S Section 124 B of Children & Community Department for Child Protection
Services Act 2004

Notes:
Mandatory reporting requirements apply to all individuals under 18 years of age, except in New South Wales where they apply to individuals under 16 years old.
States vary on whether it is mandatory to report only actual suspected harm, or also significant future risk of harm.
Key: P, physical abuse, S, sexual abuse, E, emotional/psychological abuse.
Source: Adapted from Higgins et al. 2009. National Child Protection Clearinghouse Resource Sheet No 3. Mandatory reporting of Child Abuse and Neglect. Australian Institute of
Family Studies, Australian Government.

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19.2 FORENSIC PAEDIATRICS AND THE LAW
19

ADMINISTRATION AND EMS

Criminal court
Table 19.2.3 Terminology of criminal charges
In a criminal matter, a person is charged with
Bodily harm an injury which interferes with health or comfort (e.g. causes moderate pain or discomfort)
committing an offence under the criminal but does not endanger life or cause permanent disability
code of the state in which the offence Grievous bodily harm any bodily injury of such a nature as to endanger or be likely to endanger life, or to
cause or be likely to cause permanent injury to health if left untreated
occurred. Criminal courts are adversarial sys- Unlawful wounding an injury in which the true skin has been broken, e.g. laceration or incised wound
tems where the Crown must prove that the Torture an intentional infliction of severe pain or suffering on a person by an act or series of acts done
on one or more than one occasion
accused is guilty of the offence with which
they have been charged. The level of proof Source: Derived from Queensland Criminal Code Act 1899.
required is beyond reasonable doubt.
Police are responsible for investigating
any alleged crime and for laying charges
against an individual if grounds exist. They comments as to effects on the child with case before a judge for ruling. Proof is on
are also responsible for assisting the Direc- regards to pain and suffering, and prognosis the basis of the balance of probability.
tor of Prosecutions in obtaining witness assuming medical intervention had not
statements. Initial evidence is heard in a occurred. It is not appropriate to describe
Coroners inquest
committal proceeding before a magistrate. injuries as, for example, constituting griev-
The coroners role is to investigate and
In this court, the evidence is tested to ensure ous bodily harm as this is for the court to
report on the circumstances surrounding a
that there is sufficient for the matter to pro- determine.
persons death. The coroners powers are
ceed to a higher court. defined by state or territory legislation. This
Whether the matter is heard in a higher Childrens court child protection legislation also defines a medical practi-
court is determined by the nature of the proceedings tioners obligations to notify reportable
offence. Summary offences (usually relatively Childrens courts deal with criminal charges deaths and to co-operate in any inquiry.
minor offences, e.g. traffic offences, some brought against children up to 17 or 18 The precise definition of reportable death
assaults) are dealt with solely in the Magis- years. They also handle matters related to varies between states but, in general,
trates Court. Indictable offences proceed to childrens welfare. includes any death for which the cause is
a higher court before judge and jury in either An ED medical officer who provides unknown or where the cause of death
District or Supreme Courts. assessment and treatment of a child with appears to be by violent, unnatural or acci-
Doctors may be called to provide evidence inflicted injury or abuse may be required dental means, or where there are suspicious
either as a professional witness providing to provide information and expertise to circumstances.
factual evidence of something that occurred assist this court in making decisions regard- Emergency physicians will sometimes
in the clinical setting, or as an expert wit- ing a childs need for care and protection by encounter children dying or presenting dead
ness, that is, a specialist or senior doctor the state or the need for other protective to the ED and must be familiar with their
who can provide expert opinion on certain orders. This court is conducted before a mag- state or territory requirements. The death
facts. istrate and is adversarial; however, it should be notified to the coroners office
The first involvement that a doctor may requires a lower standard of proof than crim- and also to the police. Police will assist the
have with the proceedings is via a request inal court, i.e. on the balance of probability. coroner in the investigation, and a forensic
from police for a statement of witness. This The nature of evidence that can be admitted pathologist will conduct an autopsy and
statement, which must be in a format is more lenient than in criminal proceedings, report back to the coroner.
acceptable to the court, will provide the allowing hearsay (second hand) information. At times doctors will be called to provide a
basis for oral evidence to be provided. The As in criminal proceedings, a sworn report or statement and may be required to give evi-
report must be clear, truthful and unambig- affidavit will often be sought prior to dence at a coronial inquest. This is not a trial
uous, should avoid the use of medical jargon appearance in court, and should follow the but a fact-finding inquiry. A mix of inquisito-
and should clearly describe terminology in general principles for statements for crimi- rial and adversarial procedures is used and
such a way that it may be understood by a nal court. hearsay evidence can be accepted.
lay person. The coroner will make public the findings
The statement should include details of Family law court of the inquest and may comment on inade-
professional qualifications and experience, This is a federal court created to administer quacies in systems and management. The
a brief history provided to the doctor, the Family Law Act 1975. In this jurisdiction, coroner does not directly recommend crimi-
and an accurate description of clinical find- medical practitioners may be required to nal proceedings.
ings. Reference should be made to any other provide evidence on behalf of children in
documentation or investigation, e.g. photo- contested matters between parents, related
graphs, so that they can be admitted to guardianship and residency of involved
as evidence. It is important to address
Providing evidence in court
children. Again, the system is adversarial,
whether the injuries found could produce with legal representatives for both parental Doctors may be called to give evidence in
effects consistent with the definition of the parties, and often an independent represen- any of the courts outlined. This will be either
charges (Table 19.2.3). This usually requires tative for the child or children, outlining a as a professional witness providing evidence

407
19.2 FORENSIC PAEDIATRICS AND THE LAW

in fact, related to what they saw or did as to assist the court in its deliberations and medical negligence involved, prior contact
part of their work, or, alternatively, to pro- is not personally on trial. The doctor must with their medical indemnity organisation
vide an expert opinion on a matter. adopt an independent neutral role and be should be made.
Prior to attendance at court, the doctor prepared to consider objectively any state-
may have been requested to provide a state- ments put to them. Doctors are not there
ment of witness, affidavit, or medical report to support a particular side. Further reading
that is composed from the doctors notes. When appearing as an expert witness, a Breen K, Plueckhahn V, Cordner S. Ethics, law and medical
The statement must include professional doctor will be asked initially to describe their practice. Allen & Unwin, St. Leonards: NSW;
1997.
qualifications and, in the case of an expert qualifications and expertise. They will then Dix A, Errington M, Nicholson K, Powe R. Law for the medical
witness, must establish that they have be questioned by one counsel and cross- profession in Australia. 2nd ed. Port Melbourne:
Butterworth-Heinemann; 1996.
specialised skills and knowledge in their area examined by the other. They should listen Higgins D, Bromfield L, Richardson N, et al. National Child
of expertise. carefully to all questions and answer as Protection Clearinghouse(NCPC) Resource Sheet No 3.
Mandatory reporting of Child Abuse and Neglect.
The report should include a brief history accurately as possible. They should have Australian Institute of Family Studies, Australian
of the reasons for seeing the child, the date, their notes of the consultation available Government; 2009.
Queensland Child Protection Act. 1999.
time and place the child was seen, and the and ask leave of the court to refer to them. Queensland Criminal Code Act. 1899.
person accompanying the child. Any sponta- It is important to remain calm and profes- Royal College of Paediatrics & Child Health (RCPCH). Child
Protection Companion. 2006.
neous disclosures that the child has made to sional in all ways and never be tempted to Royal College of Paediatrics & Child Health (RCPCH). Child
the doctor should be included. The general outsmart the lawyers in their domain. Care- Protection Reader. 2007.
Royal College of Paediatrics & Child Health (RCPCH).
state and demeanour of the child, and ful preparation and a sound knowledge of Standards for Radiological Investigation in Suspected
detailed specific findings are important. the relevant medical problems will assist Non-Accidental Injury. Intercollegiate Guideline RCPCH
and Royal College of Pathologists; 2008.
Lastly, an opinion regarding the mechanism the doctor greatly in this whole process. If Shepherd R. Simpsons forensic medicine. 12th ed. London &
of any injury, and the effects on the child of questioned about a matter outside their Baltimore MD, USA: Hodder Arnold; 2003.
that injury with regards to pain and disabil- level of experience or expertise, a doctor
ity, are important. should indicate that this is the case and References available
Court appearances can be stressful for not attempt to speculate. online (2010)
doctors. However, it is important to remem- Doctors providing evidence to a coronial RCPCH guidelines http://www.rcpch.ac.uk/Publications/
Publications-list-by-title.
ber that an individuals evidence is but a inquest will normally be independent parties. NCPC resource sheet http://www.aifs.gov.au/nch/pubs/
small part of any case. The doctor is there However, if there are issues of possible sheets/rs3/rs3.pdf.

408
 20

SECTION
ANALGESIA AND
SEDATION
Section editor Gary Browne

20.1 Analgesia and sedation 409

20.1 Analgesia and sedation

Stephen Priestley Jason Acworth Anthony Harrington

and benchmarked standards for time-to-

ESSENTIALS analgesia within the ED.
Definitions of terms are outlined in
1 Acute pain is one of the most common emergency department presenting Table 20.1.1.
problems.
2 Pain rating scales suitable to the age and development of the child are useful in
establishing a childs level of pain and assessing the adequacy of analgesia. Assessment and
3 Adopt a multimodal approach to pain management (pharmacological, cognitive, measurement of pain
behavioural and physical therapies).
Pain is a subjective multifactorial experience
4 Involve families and tailor interventions to the individual child. and should be treated as such.2 Pain may be
influenced by age, race, gender, culture,
5 Minimise the number of drugs used and become familiar with dose, administration emotional state, cognitive ability, expecta-
and potential complications of each.
tions and prior experience. Assessment of
6 Combining drugs without detailed knowledge and training risks serious adverse pain should be individualised, continuous,
outcomes. measured and documented. Over the last
20 years, pain assessment and measurement
7 Establish educational programs and policies for Emergency Department tools have been developed that are suitable
Procedural Sedation (EDPS).
for children of different ages and develop-
mental stages. A definitive review of pain
measurement in infants and children has
patients at the highest risk of receiving inad- not been published.
Introduction equate analgesia.2,3 Accurate assessment requires a detailed
Acute pain in children is one of the most Childrens pain is underestimated because pain history and consideration of the com-
common reasons for presentation to the of a lack of adequate assessment tools and plexity of the childs pain perception and
emergency department (ED).1 Pain resulting the inability to account for the wide range the influence of situational, psychological
from injury, illness or necessary medical pro- of childrens developmental stages. Pain is and developmental factors. Because of its
cedures is associated with increased anxiety, often undermedicated because of fears of subjective nature, pain is best assessed
avoidance behaviour, systemic symptoms oversedation, respiratory depression, addic- using the childs self-report, especially in
and parental distress. Painful experiences tion, and unfamiliarity with use of sedative older childrens pain. Observation of the
involve the interaction of physiological, psy- and analgesic agents in children.3 childs behaviour should be used to comple-
chological, behavioural, developmental and Emergency department staff should be ment self-report tools. Four useful means of
situational factors. Children with painful proficient in the assessment and safe man- recognising pain in children are outlined in
conditions can be difficult to assess and agement of pain in children. Early and Table 20.1.2.
are often still underassessed and under- appropriate analgesia may be best achieved Observational assessment scoring may be
treated. Children often receive less analge- by using a systematic approach with well- useful when the child is too young or self-
sia than adults and the administration of developed pain management educational report is not possible, e.g. children with cog-
analgesia varies by age, with our youngest programs, specific pain management policies, nitive impairment. Pain ratings provided by

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Table 20.1.1 Definitions of terms Pre-procedure assessment.

Procedure management.

Analgesia Relief of the perception of pain without sedation
Post-procedure management.
Anxiolysis Relief of apprehension without sedation or analgesia

Sedation Lessening of awareness of the environment and or pain perception

Pre-procedure assessment
Anaesthesia (general) Complete loss or awareness of the environment accompanied by loss of
protective reflexes
and management
Health status of the patient is essential to
Procedural sedation A technique of administering sedative or dissociation agents, with or without
analgesics, to induce a state that allows the patient to tolerate unpleasant
identify elements of medical history or any
procedures while maintaining cardio-respiratory function current illnesses that may impact upon the
PSA Procedural sedation and analgesia is intended to result in a depressed level of
safety of procedural sedation, choice of agent
consciousness but one that allows the patient to maintain airway control or consideration of alternative techniques.
independently and continuously
Important factors to consider are listed in
EDPS Emergency Department Procedural Sedation Table 20.1.4.
Children aged less than 1 year, and partic-
ularly the very young baby, have a higher
incidence of hypoxic complications with pro-
cedural sedation and in very young infants
Table 20.1.2 Recognition of pain Procedural sedation and consideration should be given to general
in children anaesthesia with formal airway management.
analgesia: a structured
1. The childs self-report of pain While the presence of active asthma or
2. Behavioural changes, e.g. crying, guarding, approach upper respiratory tract infection is asso-
facial grimacing
3. Physiological changes, e.g. pallor, The goal of procedural sedation in the ED ciated with a higher risk of complications,
tachycardia, and tachypnoea is to provide a suitable level of sedation including laryngospasm, in patients under-
4. Pathophysiological process, e.g. fracture or
burn that enables successful completion of a going general anaesthesia17 it is unclear
necessary procedure whilst minimising the whether this increased risk also applies to
risk of adverse events to the patient and procedural sedation. Most authorities
enabling early safe discharge home from assume that it does and tailor the sedation
parents or regular carers may be used;4 the department. In order to achieve this plans accordingly.
however, whilst there is good correlation goal, consideration needs to be given to Further history that needs to be obtained
between the childs and the parents assess- the health status of the patient, the proce- includes current medications, allergies and
ment of pain intensity, parents tend to dure to be performed particularly the dura- fasting status.
underscore more severe pain being experi- tion and likely amount of pain selection Pre-procedure fasting guidelines are a
enced by their children.5 Physiological mea- of an appropriate drug or combination of feature of most protocols developed for pro-
sures (e.g. heart rate and respiratory rate) drugs, provision of non-pharmacological cedural sedation in children and aim to min-
may be useful in pain assessment in non- techniques, patient preparation and the imise the risk of pulmonary aspiration. Both
verbal or sedated children but may be con- informed consent and understanding of the American Academy of Pediatrics and the
founded by stress reactions. For example, the parent(s) or carer(s). American Society of Anesthesiologists (ASA)
the infant who is hungry or frightened Critical incident analysis of adverse seda- list specific fasting times for solids and
may give an inappropriately high score. tion events in paediatrics has identified liquids that vary from 2 hours to 68 hours,
Specific pain assessment tools using inadequate medical evaluation, inadequate depending on the age of the child.18,19 How-
behavioural observation have been devel- monitoring during or after the procedure, ever, these recommended times were a
oped for neonates and non-verbal or cogni- inadequate skills in problem recognition result of expert consensus opinion and are
tively impaired children (e.g. FLACC and and timely intervention and lack of experi- not specifically directed at patients in the
NCCPC-R tools). Common behavioural indi- ence of the practitioner with a particular unique ED setting. In fact, aspiration asso-
cators of pain include: crying or vocalisation; age group or with an underlying medical ciated with paediatric procedural sedation
facial expressions; body language and pos- condition as factors associated with adverse has not been reported in the literature and
ture; agitation and consolability. events in paediatric sedation.6 there is no compelling evidence to support
An understanding of the pathophysiolog- The development and implementation specific pre-procedure fasting periods for
ical process can also give the clinician a sur- of procedural sedation guidelines in emer- either liquids or solids.2023
rogate indication of severity of pain. gency departments, addressing quality of Whilst some EDs use a general fasting
Pain scores are typically standardised on a care for the patient, are associated with guideline of 2 hours for clear liquids and
010 scale. Pain scores are documented with practice improvements7 and the lessening 46 hours for solids or liquids that are not
other observations and vital signs. Some of adverse events and complications.816 clear (including milk), a number of EDs do
common pain rating scales appropriate to The formulation of a sedation plan and not have strict fasting guidelines. The risk
age are outlined in Table 20.1.3. sedation policies can be divided into: of aspiration with ED procedural sedation

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ANALGESIA AND SEDATION

Table 20.1.3 Pain scoring

FLACC scale
The FLACC Scale is a behavioural scale for scoring pain in children between the ages of 2 months and 7 years or in persons unable to communicate. Each of the 5
categories is scored from 0-2 and the scores are added to get a total score from 0 to 10.

0 1 2

Face No particular expression or smile Occasional grimace or frown, withdrawn, Frequent to constant frown, clenched jaw,
disinterested quivering chin

Legs Normal position or relaxed Uneasy, restless, tense Kicking, or legs drawn up

Activity Lying quietly, normal position, moves easily Squirming, shifting back and forth, tense Arched, rigid, or jerking

Cry No cry (awake or asleep) Moans or whimpers, occasional complaint Crying steadily, screams or sobs, frequent
complaints

Consolability Content, relaxed Reassured by occasional touching, hugging or Difficult to console or comfort
talking to, distractible

The FLACC behavioural pain assessment scale # University of Michigan Health System can be reproduced for clinical or research use.

Faces rating scales (FRS)

These scales can be used with young children (as young as 4 years of age). They also work well for older children and adolescents, including those who speak a
different language.
Ask the patient to choose the face that best describes how they feel. The far left face indicates no hurt and the far right face indicates hurts worst.

The Faces Pain Scale Revised (FPS-R) can be downloaded (including instructions in multiple languages) from the Pediatric Pain Sourcebook at www.painsourcebook.ca.

Numerical rating score (NRS)

This tool may be used for children over the age of 6-8 years. Instruct the patient to rate their pain intensity on a scale of 0 (no pain) to 10 (the worst pain imaginable).

generally used for ED procedural sedation

Table 20.1.4 Patient factors to consider in procedural sedation
as it is unclear how they extrapolate to this
Current illness URTI, active asthma, altered conscious state, haemodynamic compromise
Age under 12 months (especially <3 months)
setting.24 Following risk assessment and
Airway abnormalities craniofacial deformities, tracheomalacia, predicted difficult airway generation of a sedation plan, this plan
Cardiac conditions congenital heart disease, arrhythmias, cardiac failure
Neurological conditions seizure disorders, raised intracranial pressure
should be discussed with the childs parent
Respiratory conditions active asthma, upper or lower respiratory tract infections, respiratory failure or carer to obtain informed consent. A clear
Hepatic and renal disorders
Prior history of failure of sedation
explanation of the sedation plan and what is
Prior adverse event due to sedation, analgesia or anaesthesia to happen for the older child is often useful
in an effort to allay anxiety and optimise co-
operation.
is likely to be significantly lower than that Physical examination should include The expected effects on the child, risks,
associated with general anaesthesia and patient weight, baseline vital signs including benefits, alternatives and need for monitor-
the requirement for fasting remains a sub- oxygen saturation, assessment of conscious ing and observation during and after the pro-
ject of debate. Fasting requirements should state, evaluation of the airway and examina- cedure prior to safe discharge must be
be adjusted for individual cases, following tion of the cardiovascular and respiratory discussed with the parent or guardian. As
consideration of an individual patients risks systems. the expected effects, risks and recovery times
of aspiration and the nature and urgency of The ASA physical status categories devel- prior to discharge will vary greatly between
the sedation.23 oped for general anaesthesia are not different agents, the use of a standardised

411
20.1 ANALGESIA AND SEDATION

procedural sedation consent form for all management, vascular access and cardio- airway and respiratory adverse events and
sedative drugs is suboptimal. It is recom- respiratory resuscitation. be proficient in paediatric resuscitation. The
mended that drug or drug combination- Sedation in environments where the area role of sedationist may be filled by a nurse or
specific consent forms should be generated does not meet these requirements is asso- doctor with appropriate knowledge and
that reflect the individual features of the dif- ciated with an increased level of serious training.
ferent agents, to clearly identify that these adverse events.6 The second staff member (the procedural-
issues have been discussed with the childs ist) is responsible for performing the proce-
parent. An alternative to this involves stan- Equipment dure, e.g. fracture reduction or suturing.
dard documentation in the medical record Equipment must be suitable for children of When performing deep sedation (e.g.:
that agent-specific issues were discussed with all the ages and sizes being treated. The propofol, thiopentone or other intravenous
the parent(s)/carer(s). ability to recognise respiratory or circulatory anaesthetic agents), three staff members
In most EDs there is a policy that informed compromise early with the use of monitoring should be present a trained sedationist
consent for procedural sedation is obtained equipment is essential. Devices for airway with paediatric resuscitation skills, a proce-
in written form unless it is employed in a life- management and techniques of advanced duralist and another staff member to record
or limb-threatening situation. life support are recommended, along with observations, assist the other staff as needed
sedative drug-reversal agents and life- and liaise with the childs parent or carer,
Medications support drugs. who may often be present during the
A medication order containing all sedative procedure.
drugs to be used should be recorded on Intravenous access Finally, it is recommended that there is a
the appropriate medication chart or seda- In patients receiving intravenous (IV) seda- supervising senior doctor generally at spe-
tion record, and the doses, route and time tion, the IV access should be retained until cialist or advanced trainee level, that has
of administration should also be recorded. the patients conscious state has improved. specific experience and competence in pae-
Reversal drugs and life-support drugs Patients receiving sedation by a non-IV diatric airway management and resuscita-
should also be available (Table 20.1.5). route may benefit from the safety of having tion. This person may be taking the role of
an IV line placed, particularly when the level sedationist but if not directly involved in
of sedation is deep or if there is a higher the procedure it is appropriate that he or
Environment than usual probability of complications as she is aware that the procedure is occurring
Paediatric procedural sedation must be per-
a consequence of current illness or injury and has guaranteed that they will be in the
formed in a facility that is adequate in size
or comorbidities. Where an IV line is not department and available to assist rapidly
and has equipment available for drug
established prior to sedation, a practitioner should complications arise.
administration, clinical monitoring, airway
with skills in establishing vascular access
in children must be readily available. Management during
Table 20.1.5 List of drugs and Recommended equipment and drugs are the procedure
equipment recommended for safe listed in Table 20.1.5. A number of recommendations and state-
procedural sedation
ments from clinical authorities have been
Equipmenta Personnel published detailing particular aspects of
Oxygen source, masks and tubing There must always be at least two clinical required personnel, monitoring equipment,
Suction source and suction devices
Self-inflating bagmask device staff involved in procedural sedation, with patient preparation for procedural sedation
Oral and nasal airways at least one doctor. in children both inside and outside of the
Endotracheal tubes and difficult airway
management devices One acts as the sedationist and is res- operating theatre.2527
Laryngoscopes ponsible for giving drugs and monitoring It is essential that the team administering
Defibrillator with paediatric paddles
ECG monitor the patient particularly from a respiratory procedural sedation is aware that it is impos-
Non-invasive blood pressure measurement and circulatory perspective. The sedationist sible to reliably predict in advance the level
device
Intravenous cannulation equipment should have no other responsibilities in the of sedation that will occur in an individual
Oxygen saturation monitor procedure thus enabling that person to be patient given a sedative drug or combination
Delivery system and scavenger device for
continuous nitrous oxide administration able to respond as rapidly as necessary to of drugs. Regardless of the intended level of
End-tidal carbon dioxide monitor (ETCO2) changes in the patients vital signs or clinical sedation or route of administration, the seda-
Drugsb state. If another staff member is not present, tion of a patient represents a continuum, and
Epinephrine (adrenaline) the sedationist should also be responsible may result in the loss of the patients protec-
Atropine for documenting observations, times and tive reflexes a patient may move easily from
Lidocaine
Dextrose 10% doses of drugs administered and recording a light level of sedation to deep sedation. As a
Reversal agents naloxone and flumazenil specific interventions. consequence, it is imperative to plan for situa-
Hydrocortisone
Staff providing and monitoring sedation tions where the child becomes sedated to a
a
All equipment must be available in varying sizes must be knowledgeable about the drugs far greater level than intended or experiences
appropriate to patient population.
b
Correct weight-based dose information should be
being used and be trained to identify and significant cardio-respiratory complications of
readily available. rapidly manage complications particularly sedation.

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ANALGESIA AND SEDATION

procedure, after sedative drug administra- 4:drowsy, eyes open or closed, but easily
Table 20.1.6 Elements of safe
procedural sedation management tion and at 510-minute intervals during arouses to consciousness with verbal
the procedure, depending on the level of stimulus;
1. Personnel
2. Environment sedation achieved. Times of administration, 3:arouses to consciousness with moderate
3. Medications drugs given, dosages and routes of adminis- tactile or loud verbal stimulus;
4. Risk assessment
5. Patient preparation and consent tration of drugs should be clearly recorded 2:arouses slowly to consciousness with
6. Monitoring on a time-based observation chart. sustained painful stimulus;
7. Recovery
8. Documentation Interventions performed, such as provi- 1:arouses, but not to consciousness, with
9. Discharge criteria sion of supplemental oxygen or basic airway painful stimulus;
management should also be recorded. 0:unresponsive to painful stimulus.
Deep sedation is commonly defined as a
Accordingly, well-understood policies and
score of 02 and moderate sedation as a
procedures detailing the requirements for Supplemental oxygen score of 3.
procedure management should be devel- The use of supplemental oxygen is largely The Modified Ramsay Sedation Score36 is
oped by EDs providing procedural sedation unstudied in children undergoing proce- also commonly used and ranges from a score
to children. Table 20.1.6 details the impor- dural sedation in the emergency depart- of 1: awake and alert, through to a maxi-
tant elements of the conduct of procedural ment.32,34,35 mum score of 8: unresponsive to external sti-
sedation management. The addition of supplemental oxygen has muli, including pain.
the potential to mask respiratory depression
Monitoring, observations as hypoxia may not manifest even in the
and recording Bispectral index monitoring
presence of significant hypoventilation.
The most important element of monitoring The advent of bispectral index (BIS) monitor-
Use of ETCO2 monitoring is recommended
during sedation is close, continuous observa- ing to measure the depth of anaesthesia in
when supplemental oxygen is administered
tion of the patient. patients in the operating room may poten-
as changes in ETCO2 associated with respira-
Recommended monitoring equipment tially be a useful adjunct in procedural seda-
tory depression are detectable before the
includes electrocardiogram (ECG), non- tion in the ED. 29,3739
onset of hypoxia.34
invasive blood pressure measurements, Further studies using this index will better
Many physicians use supplemental oxy-
oxygen saturations and end-tidal carbon define what role an objective measure of
gen coupled with ETCO2 monitoring with
dioxide monitoring (ETCO2) (Table 20.1.5). sedation level may play in reduced complica-
patients who are undergoing deep sedation.
Continuous oxygen saturation monitoring tions of oversedation along with avoidance
is recommended in all forms of procedural of inadequate sedation during procedures.
sedation as is continuous observation of col-
Sedation scores
our, airway and rate and depth of ventilation. Post-procedure management
There is no universally validated and
It is recommended that intermittent heart It is imperative that children are closely
accepted score for recording the depth of
rate and blood pressure monitoring is used observed and monitored in an appropriate
sedation in patients undergoing procedural
when the level of sedation is deep. environment following procedural sedation.
sedation in the emergency department.
Children vary greatly in their speed of recov-
Benefits of the use of a valid scoring
Capnography ery from sedation. This is also influenced by
method include the monitoring and record-
Capnography provides a more sensitive the drug(s) used, the route of administration
ing of depth of sedation during episodes of
means of identifying respiratory depres- and the total dosage given.
procedural sedation along with assisting in
sion or airway complications resulting from The period immediately following the com-
standardisation of terminology and classifi-
sedative agents than conventional moni- pletion of the procedure with cessation of
cation of sedation depth, particularly in clin-
toring and observation, but has been the painful or unpleasant stimulus is a
ical audit and research, looking at the
underutilised.28 period during which cardiorespiratory depres-
effectiveness and adverse event profiles of
With abnormalities in ventilation that are sant effects of the sedation drugs may be
different drugs at different doses.
detectable by capnography and then only most apparent and it is during this time that
These scores are observational in nature
later evolve into the typical clinical manifes- there is an increased risk of complications.40
and are recorded during the episode of
tations of respiratory depression, apnoea, If the child is moved to another area follow-
sedation.
or airway obstruction.2933 Oxygen desa- ing the completion of the procedure, they
A commonly used sedation score is the
turation is often the last sign of the com- should continue to be closely observed and
Childrens Hospital of Wisconsin Sedation
plication, particularly when supplemental monitored by a suitably experienced nurse
Scale.9
oxygen has been administered.29 and resuscitation equipment should still
The scale has seven levels of sedation
Observation and recording of heart rate, be immediately available if needed. The fre-
ranging from 6 to 0:
blood pressure, respiratory rate, conscious quency of vital signs observation can gra-
level or sedation score, O2 saturation and 6:anxious, agitated or in pain; dually lessen as the child awakens and
ETCO2 should be recorded prior to the 5:spontaneously awake without stimulus; the risk of cardiorespiratory complications

413
20.1 ANALGESIA AND SEDATION

of 2.325%, with the incidence of serious the incidence and nature of paediatric seda-
Table 20.1.7 Discharge criteria post-
procedural sedation complications such as laryngospasm, pulmo- tion with propofol outside the operating room
nary aspiration or cardio-respiratory arrest using a large database of 49 836 propofol
Normal age-specific vital signs
Child has regained pre-sedation conscious being extremely low.4345 sedation encounters.44 There were no deaths
state and communication skills Establishing accurate adverse event and reported. Cardiopulmonary resuscitation was
Child is able to ambulate independently
(relative to age and pre-sedation ability) complications rates of different agents from required twice and there were four episodes
Child is able to tolerate oral food or fluids the available literature has been difficult of pulmonary aspiration. Less serious events
Post-procedure analgesia is satisfactory
Parent or carer has been provided with post- because of the difficulty in aggregating were reasonably common, with oxygen
discharge instructions, understands them results from previous studies that have used desaturation below 90% for more than 30 s
and is capable of following them
varied terminology to describe the same having an incidence of 1.5% and apnoea,
adverse events and outcomes.46,47 airway obstruction, wheezing, stridor or laryn-
diminishes. Generally, the frequency of vital In 2009, a Consensus Panel on Sedation46 gospasm occurring at a rate of 1.6%, with
signs observation will lessen from 5-minutely proposed a standardised terminology and a reported rate of unplanned airway inter-
to 30-minutely as the child returns to their reporting methodology for adverse events in ventions (from simple airway manoeuvres
pre-sedation state. EDPS in children. Moving away from the tradi- to emergency intubation) of 1.5%, which
Post-procedural observations should be tional event- and threshold-based definitions equates to 1 in 70 propofol sedations requir-
recorded on the same time-based observation of an adverse event (e.g. oxygen saturation ing airway and ventilation interventions.
sheet as used during the procedure and <92%), the Panel proposed reporting based Metanalyses of predictors of adverse
evidence of improvement in conscious level on whether a particular event required an events in procedural sedation with ketamine
and activity should also be recorded. intervention to be performed by the clinician, in children have reported an overall incidence
It is important to have standard discharge i.e. whether the event was clinically relevant of airway and respiratory adverse events of
criteria, which must be met by the child prior rather than simply transient, self-limiting 3.9% from 8282 episodes of ketamine seda-
to leaving the ED, and nursing staff caring and without clinical sequelae. Adoption of tion. The rate of unplanned airway interven-
for the child must be familiar with these. such standardised reporting guidelines by tions was not reported and many of these
Suggested discharge criteria are listed in researchers will provide data that may be observed adverse events required no inter-
Table 20.1.7. readily compared and aggregated across a vention. The overall incidence of emesis,
Development of a standard post- variety of drugs, drug combinations, sedation any recovery agitation, and clinically impor-
procedural sedation discharge instruction providers and sedation locations. tant recovery agitation was 8.4%, 7.6%,
sheet, which includes advice regarding diet, A number of recent studies have reported and 1.4%, respectively.45,48
activities, observation, and sleeping in the on large cohorts of children undergoing pro- The majority of significant adverse events
24 hours after discharge, along with specific cedural sedation. in paediatric procedural sedation are re-
signs to watch for and a direct ED contact The Paediatric Sedation Research Consor- lated to airway obstruction, hypoventilation
number, is also valuable. The adult taking tium reported on the nature and frequency of requiring stimulation, supplemental oxyge-
responsibility for the child after discharge adverse events in 30 000 children receiving nation and/or a short period of assisted ven-
should receive a copy prior to leaving and sedation and/or anaesthesia for diagnostic tilation by bagmask device. Paradoxical
staff should both check that they under- and therapeutic procedures outside of excitation (benzodiazepines) or clinically sig-
stand it clearly and answer any questions the operating room.43 The overall reported nificant recovery agitation (ketamine) may
about it that they may have. adverse event rate was 1 in 29 cases require specific management.
Finally, follow-up arrangements relating (3.4%) and the rate of unplanned treatments Table 20.1.8 lists adverse events and com-
to the procedure performed (e.g. review of was 1 in 89 cases (1.1%) Serious adverse plications associated with paediatric proce-
wound, suture removal) should also be events were rare and there were no deaths. dural sedation.
provided before discharge. One case of cardiac arrest and one case of pul- Adherence to published guidelines recom-
monary aspiration were reported. Conversely, mending minimum requirements for person-
more minor but potentially serious adverse nel, patient monitoring and assessment for
events were not rare. Oxygen desaturation safe discharge has been shown to reduce
Complications of paediatric
below 90% was the most common adverse adverse events associated with procedural
procedural sedation event reported, with an incidence of 1.5%. sedation in children.9
Paediatric procedural sedation can be haz- Vomiting was common and occurred in 1 in In particular, the generation of an individ-
ardous and both mortality and significant 200 sedations (0.5%). Approximately 1 in ual sedation plan comprising an individual
morbidity have been reported in the litera- 400 procedures were associated with stridor, risk assessment with documentation of vari-
ture.4143 laryngospasm, wheezing or apnoea and 1 in ables such as fasting status, quantitative
Studies of procedural sedation in EDs with 200 sedations required airway and ventila- sedation scoring, time-based recording of
adherence to published guidelines and tion interventions ranging from bagmask vital signs and pulse oximetry combined
involvement of staff trained in sedation ventilation (0.6%), to oral airway placement with standardised recovery and discharge
and paediatric resuscitation techniques (0.3%) to emergency intubation (0.1%). criteria and use of a standardised record
have yielded variable rates of complications The same research group has also reported has been shown to progressively reduce

414
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ANALGESIA AND SEDATION

prodrug of morphine and nine percent of
Table 20.1.8 Adverse events and Table 20.1.9 Supportive and distractive
complications in procedural sedation techniques children do not have the liver enzyme
CYP2D6 to convert the inactive codeine
Sedation failure Environment
Paradoxical excitation and therefore in this group it provides no
Recovery agitation Calm friendly non-clinical atmosphere
Toys, mobiles, pictures and videos analgesia.
Vomiting
Airway obstruction requiring suction,
repositioning or use of an airway adjunct. Psychological
Hypoxia
Oral sucrose for infant analgesia
Parental presence
Hypercarbia Age-appropriate communication Oral sucrose (25%) has been shown to be a
Apnoea Clear confident instructions simple, safe and effective means of
Hypoventilation requiring supplemental
oxygenation and/or assisted ventilation Cognitivebehavioural providing analgesia for young infants (up
Laryngospasm to 2 months of age) for short painful events
Bronchospasm Distraction techniques
Pulmonary aspiration Hypnosis and biofeedback (e.g. venepuncture, lumbar puncture).5254
Hypotension Art/stories
Music/video/TV It stimulates endogenous opioid and non-
Bradycardia/tachycardia/cardiac
arrhythmias Interactive computer games opioid pathways in the brain. Up to 2 mL
Cardiorespiratory arrest Guided imagery
Muscle relaxation and deep breathing may be administered via oral syringe or on
Allergic reactions
Over sedation techniques a pacifier approximately 2 minutes prior to
Seizures Reinforcement of coping behaviours
the painful event.
Physical
risk in procedural sedation, particularly in Massage/rubbing Parenteral opioid analgesics
Comfort swaddling (infants)
those children requiring a deep level of Heat/cold techniques Opioid analgesics are the mainstay of treat-
sedation.9 Immobilisation and elevation (injured part) ment of severe pain. Infants and neonates
Breast-feeding require lower doses of opioid medication.
Comforter (favourite blanket/soft toy) Opioids are ideally administered IV in a
Non-pharmacological dilute solution, e.g. morphine (1 mg mL1)
methods given slowly over a few minutes and titrated
against response. A further dose may be
A balanced multidisciplinary approach using
given after 510 minutes. Fentanyl is a
pharmacological and non-pharmacological
Pure sedative agents potent rapid-acting analgesic with a shorter
strategies is essential to providing optimal
Sedative agents relieve anxiety but not duration of action and used in combination
analgesia and sedation for the child. Non-
pain. They may reduce the childs ability to with midazolam produces a state of dissoci-
pharmacological techniques can be particu-
communicate pain and discomfort and a ation and deep sedation.
larly useful in pain management (whether
common side effect is paradoxical hyper-
or not medications are used as well) as they
excitability. They are useful for painless Intranasal fentanyl
are free of side effects and may be utilised
diagnostic studies and, when used in combi- Intranasal fentanyl provides safe and effec-
before, during and after painful procedures.
nation with opioids or nitrous oxide, can tive analgesia equivalent to parenteral
The planning of procedures for children in
produce a state of deep sedation. morphine in children as young as 1 year of
the ED should include age-appropriate psy-
age.5558 It offers a quicker onset, is less
chological interventions, such as distraction
invasive, and its duration of action, although
techniques. Distraction reduced self-reported Oral analgesic agents
short, allows time for topical anaesthetic
pain following needle-related procedural Paracetamol is usually the first line of
application prior to intravenous cannulation
pain.49 Age-appropriate distraction techni- therapy for mild to moderate painful condi-
for ongoing analgesia. It is particularly use-
ques reduced situational anxiety in older tions. It can be given orally and rectally. Non-
ful for analgesia for fractures or burns dres-
children and lowered parental perception steroidal anti-inflammatory drugs (NSAIDs),
sings but its utilisation is spreading into
of distress in younger children undergoing such as ibuprofen, are usually second-line
other areas.
laceration repair.50 A childs anxiety and co- therapy and have excellent analgesic and
operation are affected by age, anxiety of anti-inflammatory properties, which may
Nitrous oxide
the parent and previous medical experiences. enhance the effects of paracetamol.
Nitrous oxide mixed with oxygen has a
Toddlers are very distractible and storytelling
potent analgesic action with rapid onset
and guided imagery are very effective meth-
Oxycodone and codeine and offset. It is an excellent analgesic seda-
ods. Some useful non-pharmacological strate-
Oral codeine has traditionally been used for tive for gaining rapid IV access, injecting
gies are outlined in Table 20.1.9.
moderate to severe pain; however, evidence local anaesthetics, performing a nerve block
from recent work shows that oral oxycodone or splinting a fractured limb.5961
produces greater pain relief compared with EntonoxW (50% nitrous oxide, 50% oxy-
Pharmacological methods codeine.51Oxycodone also has a better side gen) is usually delivered via a demand-
Some of the most commonly used agents are effect profile with less itching, less nausea, valve system. This limits its use in younger
listed in Table 20.1.10. and fewer allergic reactions. Codeine is a or unco-operative children. Machines that

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20.1 ANALGESIA AND SEDATION

Table 20.1.10 Commonly-used agents for sedation and analgesia

Classification Doses Comments

Pure analgesics
Paracetamol 1530 mg kg1 PO/PR (<3 months 10 mg kg1) 30 mg kg1 stat. only as a single dose (check no recent
doses of paracetamol)
Ibuprofen (NSAID) 510 mg kg1 PO
Liquegesic Co paracetamol/ Dose based on paracetamol content
codeine mixture (e.g. PainstopW)
Oxycodone 0.1 mg kg1 PO Better analgesia and fewer side effects than codeine

Pure sedatives
Midazolam 0.050.15 mg kg1 IV/IM; 0.50.75 mg kg1 PO; Paradoxical excitement reaction in 10% of children when
0.30.5 mg kg1 IN used PO or IN

Sedative/analgesics
Morphine 0.10.2 mg kg1 per dose (>3 months) IV, IM Cardio-respiratory depression with IV/IM doses IV dose
0.050.1 mg kg1 per dose (<3 months) IV, IM Infusion preferred
(>3 months) 0.010.04 mg kg1 hr1
Fentanyl 1.5 mcg kg1 per dose
Nitrous oxide 50:50 mix inhalation agent (ENTONOXW); Rapid onset; continuous flow delivery system allows use in
N2O continuous flow variable mix 3070% with oxygen young children/short duration of action. Vomiting can occur;
contraindicated with pneumothorax/chest injuries

Dissociative agent
Ketamine 12 mg kg1 IV; 2.54.0 mg kg1 IM; 510 mg kg1 PO Excellent safety profile in selected patients undergoing
painful procedures; contraindicated in head injury, seizure
disorders, ocular injuries

Sedative/hypnotic agent
Propofol 0.5-3.0 mg kg 1 induction dose (titrate slowly to achieve Median dose 0.75 mg kg 1 when used with adjunctive
desired sedation depth), halve the induction dose for top-up analgesia. Respiratory depression is the most common
dosing adverse effect

Local anaesthetic agents

Injectable
Lidocaine 1% or 2%; 3 mg kg1 maximum dose without adrenaline Pain of injection can be minimised by using small needles
(epinephrine); 7 mg kg1 maximum dose with adrenaline (e.g. 31G), slow infiltration, infiltrating through wound edges,
pre-treatment with topical LA, buffering and warming
Levobupivicaine or bupivacaine 3 mg kg1 (maximum dose) Slower onset, longer duration of action compared with
lidocaine; useful for nerve blocks
Prilocaine 0.5% solution common; 2.53.0 mg kg1 Safe agent for intravenous regional anaesthesia (Biers
blocks)

Topical
Surface EMLAW Eutectic mix of lidocaine 2.5% and prilocaine 2.5% Requires 60 minutes post-application to achieve
satisfactory dermal anaesthesia
W W
AnGel /Ametop Amethocaine 4% Requires 3045 minutes post- application to achieve
satisfactory dermal anaesthesia
W
Laceraine LaceraineW solution is a mixture of 4% lidocaine, 0.5% Contains adrenaline so cannot be used in end arteriole
tetracaine and adrenaline 1:1000 regions, e.g. digits, penis; requires > 20 minutes of good
contact with wound to provide anaesthesia; may require
supplementation with small doses infiltrated LA

Reversal agents
Naloxone 10 mcg kg1 IV May need to repeat doses
Flumazenil 0.02 mg kg1 IV

LA, local anaesthetic; IM, intramuscular; IN, intranasal; IV, intravenous; PO, per oram; PR, per rectum.

deliver variable concentration (3070%) painful conditions but may require the use Ketamine
nitrous oxide via a continuous flow system of adjunctive analgesics for very painful Ketamine is a unique analgesic dissociative
allow the use of this agent down to age procedures.61,62 agent. Its action produces a near ideal state
1 year, where it has been shown to be safe Oxygen should be administered for 35 of sedation, amnesia, analgesia and motion
when embedded in a comprehensive seda- minutes after cessation of nitrous oxide to control with few side effects. It has been
tion programme.61 prevent diffusion hypoxia. Nitrous oxide is extensively studied, with safety and efficiency
Nitrous oxide alone is effective in achiev- contraindicated in conditions involving documented in several large paediatric stud-
ing moderate levels of procedural sedation closed air spaces (e.g. pneumothorax, bowel ies. It is particularly suitable for laceration
for a high percentage of children with obstruction). repair and orthopaedic procedures in the

416
20.1 ANALGESIA AND SEDATION
20
EMLAW (2.5% lidocaine, 2.5% prilocaine)

ANALGESIA AND SEDATION

emergency department. Emergence reac- painful procedures in children (e.g. fracture
tions, common in adults during the recovery reduction). It is associated with a signifi- is a well-established topical anaesthetic for
phase, are rare in children. Co-administration cantly higher rate of adverse airway events use on intact skin prior to venepuncture,
of midazolam has not been shown to reduce (oxygen desaturation and need for airway intravenous cannulation or lumbar puncture.
the rate of emergence phenomena but may repositioning) when compared to keta- Its use in the ED is, however, limited, due to its
be associated with less post-procedure eme- mine,67 emphasising the importance of hav- long onset to peak effect (at least one hour)
sis. Intravenous ondansetron significantly ing skilled and experienced practitioners and its vasoconstrictive effect, which may
reduces the incidence of vomiting associated available when it is being used. make cannulation more difficult. EMLAW
with ketamine sedation.63 It does offer quick recovery to pre-sedation has a theoretical risk of methaemoglobinae-
Airway complications (including stridor, state, allowing more rapid discharge. It may mia and is not recommended in infants less
laryngospasm, respiratory depression, and be delivered in a variety of manners from than 3 months of age.79
apnoea) very rarely occur and have been repeated boluses to continuous infusion. Amethocaine (e.g. AnGelW 4% ametho-
reported as being associated with high intra- Despite initial barriers to the use of propofol caine) has a quicker onset of action (3045
venous dosing (initial dose >2.5 mg kg 1 or in the ED, a large number of studies have pro- minutes) than EMLAW and its vasodilating
total dose 5.0 mg kg 1), administration to duced evidence that propofol can be given in effect may facilitate cannulation. Tetracaine
children younger than 2 years or aged 13 a safe and effective manner in ED for chil- is superior to EMLAW in terms of lessening
years or older, and the use of co- dren.6872 Titrating and optimising the dose pain associated with intravenous cannulation
administered anticholinergics or benzodiaze- will enhance control of depth of sedation and is more effective than EMLAW when
pines.45 There are few data on EDPS in and recovery time.73 Predictable cardiovascu- application time is less than 60 minutes.80
relation to infants under the age of 3 months lar and respiratory events respond to reposi- Itching and erythema are side effects of
and the traditional contraindication to keta- tioning the airway, increasing oxygen tetracaine, whilst skin blanching is seen with
mine in this age group should continue until delivery or limited BVM-assisted ventilation EMLAW as a consequence of vasoconstric-
there is evidence of safety in this group. without adverse sequelae.73 Capnography is tion.80
Multiple routes of administration are now considered essential for early detection Topical wound anaesthetics, e.g. Lacerai-
available but IV and IM routes are preferred. of hypopnoea and apnoea.31 neW (adrenaline [epinephrine] 1:1000, lido-
Both routes display similar risk of airway An evidence-based Clinical Practice Advi- caine 4%, tetracaine 0.5%) and Adrenaline
and respiratory adverse events, and of clini- sory describing propofol use in EDPS was Cocaine Gel (adrenaline 1:1000, cocaine
cally important recovery agitation. The IM published in 2007.74 12%) permit wound management with
route is associated with a higher rate of minimal to no discomfort.81,82
Ketafol
vomiting.45,48 Cocaine-containing topical wound anaes-
Ketafol refers to a mixture of ketamine/
Comprehensive ketamine clinical practice thetics (e.g. TAC tetracaine, adrenaline,
propofol in a 1:1 ratio (i.e. 1 mg ketamine:
guidelines were published in 2004.64,65 cocaine) were originally found to be very
1 mg propofol). A median dose of 0.75 mg
effective in providing good wound anaes-
kg 1 of each drug (range 0.22.0) has been
Methoxyflurane thesia but non-cocaine-containing topical
shown to be safe and effective for procedural
A recent review has shown that the inhala- wound anaesthetics have now been shown
sedation in children.75 Fentanyl is usually
tional agent Methoxyflurane, long used by to be equivalent or superior to cocaine-
used as adjunctive analgesia but a study
ambulance services, is also safe and effective containing preparations, Such preparations
looking at combining propfol with a sub-
in the ED setting.66 The commonly-used are significantly cheaper, are not associated
dissociative dose of ketamine (0.3 mg kg 1
Penthrox inhaler is now available with an with the requirement for secure storage
IV) compared with fentanyl suggested that
activated charcoal scavenging chamber to and avoid the potentially serious systemic
the ketamine/propofol combination was
reduce environmental contamination. side effects that have been reported with
safer.76 Further research is required to deter-
cocaine-containing preparations.8385
mine if combining the two agents together is
Propofol There are no commercially available
associated with fewer cardiovascular and
Propofol is an excellent ultra-short acting cocaine-based wound anaesthetics available
respiratory adverse effects than using pro-
sedative/hypnotic agent, very useful for in Australia currently.
pofol alone.77
EDPS. It provides deep sedation and has Local anaesthetic combination solutions
anti-emetic and euphoric properties. Propo- Local and topical anaesthetic such as LaceraineW instilled in a wound for
fol has no analgesic properties and therefore agents 2030 minutes provide sufficient anaesthe-
needs adjunctive analgesic agents, e.g. fen- The expanded use of topical anaesthetics has sia for suturing 7590% of scalp and facial
tanyl or ketamine when used for procedural revolutionised the management of simple lacerations82,86 and 4060% of extremity
analgesia and sedation (PSA) for short lacerations in the ED and has also greatly wounds. Its vasoconstrictive effect is also
painful procedures. Used alone, it is effective improved conditions for intravenous cannula- useful prior to application of cyanoacrylate
in producing co-operation for painless tion and lumbar puncture. These agents pro- tissue adhesive.
diagnostic studies in emergency depart- vide a non-invasive means of producing local Wound infiltration with injectable local
ment patients. When combined with opiate anaesthesia and can be applied at triage to anaesthetics can be carried out with less-
agents, it has been used effectively for facilitate timely management in the ED.78 ened pain by buffering the local anaesthetic,

417
20.1 ANALGESIA AND SEDATION

warming the solution to body temperature,

Table 20.1.11 Analgesia options
using fine-bore needles and slow administra-
WongBaker faces score 2 3 4 5
tion techniques through the wound edges
rather than through intact skin.8791 Nitrous Pain score 1 2 3 4 5 6 7 8 9 10
oxide and distraction techniques during the Mild pain Moderate pain Severe pain
process of injection often provide good anal-
Paracetamol Ibuprofen Morphine
gesia. Safe dosage regimes must be adhered Paracetamol- Nitrous oxide
to by the clinician. Codeine mix
Oxycodone IN Fentanyl

Regional anaesthetic techniques

Regional nerve blocks may be used for either
lessen epistaxis and assist passage of the
pain relief (e.g. femoral nerve block for femur Table 20.1.12 Balanced analgesia and
sedation tube by decreasing swelling.
fracture) or to facilitate suturing, fracture
or dislocation reduction (e.g. digital or meta- The four components
1. Sedation Suprapubic aspiration
carpal blocks).79,92 Nerve blocks have tradi- 2. Analgesia
tionally been performed using anatomical 3. Amnesia Topical application of EMLAW prior to the
4. Motion control procedure will help alleviate pain. As this
landmarks, with variable results. The use of
ultrasound has been shown to increase the procedure is usually only performed on
success of regional anaesthetic techniques. young infants, oral sucrose should be
Ideally, routes of administration should offered.
Intravenous regional anaesthesia (Biers
be non-invasive. Extensive knowledge and
block) is useful and effective for some older
experience of the synergistic effects of
children. Tired children, reluctant children, Urinary catheterisation
analgesics and sedative agents can produce
parental concern, or the lack of ED resources Oral sucrose may be useful in young infants
a balanced state of sedation and analgesia
may make deep sedation or a general anaes- and nitrous oxide in older children. Ligno-
(Table 20.1.12).
thetic the preferred approach. caine gel should be used as lubricant.

Discharge analgesia Lumbar puncture

Recommendations for EMLAW should be applied in any child for
Lack of appropriate or inadequate dosing of some common
discharge analgesia is an ongoing problem. whom a lumbar puncture may be considered
procedures but the procedure should not be delayed
Pain experienced at home post acute injury
or PSA in the ED can place considerable Painless diagnostic study while waiting for it to work. Instillation of
extra burden on family physicians for pain- Motion control for imaging procedures, such 1% lidocaine (using a small needle e.g.
related issues. It is essential to include as CT scan or even plain X-ray, can be a chal- 27G) into the skin overlying the interspace
adequate discharge analgesia in ED pain lenge. Oral anxiolytics given prior may be will reduce the pain of the procedure. Oral
management guidelines. Ibuprofen was useful. It is important to apply topical local sucrose on a pacifier should be offered to
found to be preferable to paracetamol and anaesthetic (AnGelW or EMLAW) 4560 young infants and inhaled nitrous oxide
codeine for outpatient management for chil- minutes prior to the procedure to facilitate may be offered to older children.
dren with uncomplicated arm fractures.93 intravenous cannula placement if adjunctive
intravenous sedation becomes necessary. Small laceration
A pre-emptive approach using a combina-
Venepuncture and IV cannulation tion of oral anxiolytics/analgesic medica-
Selection of agents by Topical local anaesthetic agents are useful tion will improve co-operation during the
procedure and age for semi-urgent IV access. Rapid access is procedure. Topical wound anaesthetics
Pain management can be simplified by divid- best gained using nitrous oxide analgesia/ allow wound cleaning and closure with min-
ing pain into mild, moderate and severe cate- sedation and distraction techniques. Oral imal or no pain. Tissue adhesives are helpful
gories and matching this with the appropriate sucrose is useful in young infants. for smaller wounds. Nitrous oxide and dis-
analgesic agent(s) (Table 20.1.11). It is best to traction are again useful.
minimise the number of agents used and Nasogastric tube insertion
to be familiar with doses, duration of action, Although nebulised lidocaine has been Complex wound management
adverse effects and contraindications. Choice shown to be a useful analgesic method for Infants and toddlers may require dissocia-
of agent should be individualised for the this distressing procedure in adults, it has tive methods using a choice of ketamine
childs level of pain and the procedure. Disso- been shown to be ineffective in children.94 IM or IV, fentanyl and midazolam IV,
ciative techniques using various combina- Oral sucrose may be useful in neonates or nitrous oxide. With appropriate pre-
tions of agents are useful for very painful requiring nasogastric tubes. Topical Cophe- medication older children can be managed
procedures or in infants and toddlers. nylcaine ForteW may help alleviate pain, with local or regional anaesthetic blocks.

418
20.1 ANALGESIA AND SEDATION
20

ANALGESIA AND SEDATION

Foreign body removal simulation-based training of non-anaesthesiologists.
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Anxiolytic pre-medication may be all that 15. Borland M, Esson A, Babl F, Krieser D. Procedural
further improve the safety profile of our sedation in children in the emergency department:
is needed for the older child; however,
practices. Clinical audit of sedation/ A PREDICT study. Emerg Med Australas 2009;21:719.
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analgesia practice should be routine. Our
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practices will be made more efficient by 626.
17. Olsson GL, Hallen B. Laryngospasm during anaesthesia -
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splinting and manipulation 18. American Academy of Pediatrics, American Academy of
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Severe pain associated with these injuries is Pediatric Dentistry, Cote CJ, Wilson S, the Workgroup on
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with propofol for procedures outside the operating room: sedation and analgesia in the emergency department. in children aged 5 to 17 years. Pediatrics
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Consortium. Anesth Analg 2009;1083:795804. 65. Green SM, Krauss B. Clinical practice guideline for 87. Mader TJ, Playe SJ, Garb JL. Reducing the pain of
45. Green SM, Roback MG, Krauss B, et al. Predictors of emergency department ketamine dissociative sedation in local anesthetic infiltration: Warming and buffering
airway and respiratory adverse events with ketamine children. Ann Emerg Med 2004;44:46071. have a synergistic effect. Ann Emerg Med 1994;23:
sedation in the emergency department: an individual- 66. Grindley J, Babl FE. Review article: Efficacy and safety of 5504.
patient data meta-analysis of 8282 children. Ann Emerg methoxyflurane analgesia in the emergency department 88. Bartfield JM, Gennis P, Barbera J, et al. Buffered versus
Med 2009;54:15868. and prehospital setting. Emerg Med Australas plain lidocaine as a local anesthetic for simple laceration
46. Bhatt M, Kennedy RM, Osmond MH, et al. Consensus- 2009;2:411. repair. Ann Emerg Med 1990;19:138790.
based recommendations for standardizing terminology 67. Migita RT, Klein EJ, Garrison MM. Sedation and analgesia 89. Scarfone RJ. Pain of local anesthetics: Rate of
and reporting adverse events for emergency department for pediatric fracture reduction in the emergency administration and buffering. Ann Emerg Med
procedural sedation and analgesia in children. Ann department: a systematic review. Arch Pediatr Adolesc 1998;31:3640.
Emerg Med 2009;53:42635. Med 2006;160:4651. 90. Kelly AM, Cohen M, Richards D. Minimizing the
47. Green SM, Yealy DM. Procedural sedation goes Utstein: 68. Bell A, Treston G, Cardwell R, et al. Optimization of pain of local infiltration anesthesia for wounds by
the Quebec guidelines. Ann Emerg Med propofol dose shortens procedural sedation time, injection into the wound edges. J Emerg Med 1994;12:
2009;53:43668. prevents resedation and removes the requirement for 5935.
48. Green SM, Roback MG, Krauss B, et al. Predictors of post-procedure physiologic monitoring. Emerg Med 91. Davies RJ. Buffering the pain of local anaesthetics:
emesis and recovery agitation with emergency Australas 2007;19:4117. A systematic review. Emerg Med Australas 2003;15:
department ketamine sedation: an individual-patient 69. Burton JH, Miner JR, Shipley TD, et al. Propofol for 818.
data meta-analysis of 8,282 children. Ann Emerg Med emergency department procedural sedation and 92. Peutrell JM, Mather SJ. Regional Anaesthesia in Babies &
2009;54:17180. analgesia: a tale of three centers. Acad Emerg Med Children. Oxford: Oxford University Press; 1997.
49. Uman LS, Christine T, Chambers P, et al. Systematic 2006;13:2430. 93. Drendel AL, Gorelick MH, Weisman SJ, et al. A
review of randomized controlled trials examining 70. Green SM. Propofol in emergency medicine: further randomized clinical trial of ibuprofen versus
psychological interventions for needle related procedural evidence of safety. Emerg Med Australas acetaminophen with codeine for acute pediatric arm
pain and distress in children and adolescents: An 2007;19:38993. fracture pain. Ann Emerg Med 2009;54:55360.
Abbreviated Cochrane Review. J Pediatr Psychol 71. Patel DK, Keeling PA, Newman GB, Radford P. Induction 94. Babl FE, Goldfinch C, Mandrawa C, et al. Does
2008;33:84254. dose of propofol in children. Anaesthesia nebulized lidocaine reduce the pain and distress of
50. Sinha M, Christopher NC, Fenn R, Reeves L. Evaluation of 2007;43:94952. nasogastric tube insertion in young children? A
nonpharmacologic methods of pain and anxiety 72. Hohl CM, Mohsen S, Nosyk B, et al. Safety and clinical randomized, double-blind, placebo-controlled trial.
management for laceration repair in the pediatric effectiveness of midazolam versus propofol for Pediatrics 2009;123:154855.
emergency department. Pediatrics 2006;117:11628. procedural sedation in the emergency department: a
51. Charney RL, Yan Y, Schootman M, et al. Oxycodone versus systematic review. Acad Emerg Med 2008;15:18.
codeine for children with suspected forearm fracture: a 73. Bell A, Treston G, McNabb C, et al. Profiling adverse
randomized controlled trial. Pediatr Emerg Care respiratory events and vomiting when using propofol
2008;9:595600. for emergency department procedural sedation. Emerg
52. Stevens B, Yamada J, Ohlsson A. Sucrose for analgesia in Med Australas 2007;19:40510.
Further reading
newborn infants undergoing painful procedures. 74. Miner JR, Burton JH. Clinical practice advisory: Cote CJ, Notterman DA, Karl HW, et al. Adverse sedation
Cochrane Database Syst Rev 4:CD001069. Emergency department sedation with propofol. Ann events in pediatrics: A critical incident analysis of
53. Le Frak L, Knoerlein N, Duncan J, et al. Sucrose analgesia: Emerg Med 2007;50:1827. contributing factors. Paediatrics 2000;105:80414.
Identifying potentially better practices. Paediatrician 75. Willman EV, Andolfaltto G. A prospective evaluation of Green SM. Research advances in procedural sedation and
2006;118:197202. kefofol (ketamine/propofol combination) for procedural analgesia. Ann Emerg Med 2007;49:316.
54. Carbajal R, Verapen S, Coudere S. Analgesic effect of sedation & analgesia in the emergency department. Ann Green SM, Krauss B. Pulmonary aspiration risk during
breast feeding In term neonates: randomised controlled Emerg Med 2007;49:2330. ED procedural sedation An examination of the role of
trial. Br Med J 2003;326:13. 76. Messenger DW, Murray HE, Dungey PE, et al. fasting and sedation depth. Acad Emerg Med
55. Cole J, Shepherd M, Young P. Intranasal fentanyl in 1-3- Subdissociative-dose ketamine versus fentanyl for 2002;9:3542.
year-olds: a prospective study of the effectiveness of analgesia during propofol procedural sedation: a Mace SE, Brown LA, Francis L, et al. Clinical policy:
intranasal fentanyl as acute analgesia. Emerg Med randomized clinical trial. Acad Emerg Med Critical issues in the sedation of pediatric patients
Australas 2009;21:395400. 2008;16:877886. in the emergency department. Ann Emerg Med
56. Borland M, Jacobs I, King B, OBrien D. A randomized 77. Arora S. Combining ketamine and propofol (Ketofol) 2008;51:37899.
controlled trial comparing intranasal fentanyl to for emergency department procedural sedation and Paediatrics & Child Health Division, The Royal Australasian
intravenous morphine for managing acute pain in analgesia: a review. West J Emerg Med 2008;9:2023. College of Physicians. Guideline Statement: Management
children in the emergency department. Ann Emerg Med 78. Priestley SJ, Kelly AM, Chow L, et al. Application of topical of procedure-related pain in children and adolescents.
2007;49:33540. local anesthetic at triage reduces treatment time for J Paediatr Child Health 2006;42:S1S29.

420
 21

SECTION
POISONING
Section editor Gary Browne

21.1 General approach to poisoning 421 21.2 Specific poisons 426

21.1 General approach to poisoning

Naren Gunja

that paracetamol is present in many pre-

ESSENTIALS parations as well as in combination products
(e.g. with codeine, pseudoephedrine, doxyla-
1 Poisoning in children is usually accidental, particularly in the under-6 age group. mine, dextromethorphan).
Deliberate self poisoning may become apparent as they mature into teenage years.
2 Poisoning in most children runs an uneventful course and emergency department
(ED) observation is often the only management needed. Diagnosis
3 The potential for non-accidental poisoning (either deliberate or due to neglect) As opposed to overdose in the adult popula-
should be considered, particularly in children under the age of 1 year. Where non- tion, exposures in children are nearly always
accidental poisoning is suspected, the child should be referred to the relevant child accidental or unintentional. The circum-
protection authorities. stances around the exposure or ingestion
are often unknown or difficult to elucidate.
4 A focused history and examination should lead to a risk assessment on the likely
Parents and carers are usually uncertain
outcome, and worst-case scenario.
about time of exposure or dosage of drug
5 Advice regarding management of poisoned children can be sought from Australasian ingested. As such, the clear history required
Poisons Information Centres (Australia: 13 11 26; New Zealand: 0800 764 766). to make an accurate risk assessment is diffi-
cult or sometimes impossible. Regardless of
6 Poisoning in children manifests clinically in a similar manner to adults. The
whether the entire history and circum-
management of poisoning in children is also similar.
stances surrounding the exposure are avail-
7 Gastrointestinal decontamination is not necessary in the majority of cases. able, it is prudent to plan for a worst-case
scenario, assuming maximal exposure.
8 Parents and carers should be advised to keep medicines and chemicals away from
Important elements of the focused history
the reach of children medicines should be stored in locked containers or cupboards
include:
at a height of at least 1.5 metres.
age and gender of child;
agent involved drug, chemical or plant;
teens. The latter peak relates to deliberate approximate time of exposure;
Introduction and dose ingested, including maximum
self-poisoning in adolescents. Over eighty
epidemiology percent of poisons centre calls relating possible ingestion;
In 2009, the NSW Poisons Information to childhood exposures are advised to circumstances around exposure;
Centre received in excess of 50 000 calls stay at home as no acute management is symptoms, in particular vomiting;
from around Australia regarding paediatric necessary. Pharmaceuticals are by far the first aid and pre-hospital management.
exposures to pharmaceuticals, chemicals, commonest exposure in children as per Deliberate self-poisoning in adolescents
plants and animals. There is a bimodal American Poison Control Center data. The warrants further enquiry into previous inges-
distribution in the frequency of exposures, top ten unintentional exposures in children tions, pre-existing psychiatric illness and
with the larger peak occurring in the tod- (under the age of 18 years) reported to management, drug use and social circum-
dler age group (ages 13 years) and a Australian Poisons Information Centres are stances. In cases of unknown drug exposure,
much smaller peak in the mid to late listed in Table 21.1.1. It is important to note it is important to explore the availability of

421
21.1 GENERAL APPROACH TO POISONING

Table 21.1.1 Top 10 exposures Table 21.1.2 Common toxidromes

in children
Toxidrome Agents Clinical features
No. Agent
Sympathomimetic Amphetamines Tachycardia
1 Paracetamol Pseudoephedrine Hypertension
Caffeine Mydriasis
2 Detergents Sweating
Agitation
3 Household cleaning agents Delirium
Fever
4 Dessicants (e.g. silica gel)
Anticholinergic Atropine Tachycardia
5 Ibuprofen Hyoscine Mydriasis
Antihistamines Loss of visual accommodation
6 Cough & cold preparations Plants Flushed skin
Mushrooms Dry skin/mouth/eyes
7 Rodenticides Fever
Delirium
8 Light sticks/glow toys
Opiate Opiates Sedation
9 Zinc-containing barrier creams Tramadol Respiratory depression
Clonidine Hypotension
10 Bleach (containing hypochlorite) Miosis

Cholinergic Organophosphates Delirium

Carbamates Coma
pharmaceuticals and/or chemicals to which Seizures
Excess secretions (DUMBELS)
the child may have had access. Plant and Weakness
mushroom ingestion is common in children Fasciculations
and needs to be considered in the acutely Serotonergic SSRIs Delirium/agitation
unwell child who has been outdoors. Cyclic antidepressants Hyperreflexia
Opiates Hypertonia
Non-accidental (or deliberate) poisoning Tramadol Tremor
of a child requires mandatory reporting to Lithium Clonus
MDMA (ecstasy) Diaphoresis
child protection authorities in all jurisdictions Fever
within Australia. The index of suspicion is
higher in children under the age of 1 year, SSRI, selective serotonin reuptake inhibitor.

or where the circumstances of the exposure

do not fit the capabilities of the child in ques- neurological signs: abnormal tone, from expert clinicians in paediatric toxicology,
reflexes, clonus, seizures; such as through the Australasian Poisons
tion. Rare cases of Munchausens syndrome
by proxy are also reported in the literature, external signs of trauma, bruising, bite Information Centres (Australia: 13 11 26;
marks; New Zealand: 0800 764 766). These centres
involving deliberate poisoning of children
by their parent/carer. saliva or vomitus, pill fragments. are available 24/7 and provide expert advice
on the assessment and management of poi-
Physical examination of the potentially Children may also present with a cluster of
soning in children. The majority of paediatric
poisoned child is usually unremarkable, par- symptoms and signs suggestive of poison-
exposures do not present to hospital, and
ticularly in asymptomatic children or in the ing, i.e. a toxidrome. Although most cases
even those that do, require observation only.
early stage of ED presentation. However, do not manifest the full spectrum of signs
However, there are a few highly toxic pharma-
in children presenting with symptoms or and symptoms, pattern recognition amongst
ceuticals and chemicals that, even in small
patients with altered level of consciousness, clinicians may provide a clue to diagnosis.
doses, can cause severe toxicity. Patients
a thorough physical examination is vital. Key Toxidromes and corresponding causative
exposed to these select few agents may
elements of the toxicological examination agents commonly seen in children are listed
require close monitoring and potentially
include: in Table 21.1.2.
aggressive resuscitation these are discussed
vital signs: heart rate, blood pressure, in Chapter 21.2.
temperature, respiratory rate, oxygen
saturation;
Risk assessment
odour suggesting intoxication or Following the history and examination of
Investigations
poisoning; the potentially poisoned child, the clinician
airway patency and adequacy of must undertake a risk assessment of the likely The vast majority of children exposed to a
ventilation; exposure and probable course of toxicity, substance require no investigations at all.
cardiovascular status and end-organ if any. This requires knowledge of the toxi- There are some instances where a specific
perfusion; codynamics and kinetics of the agents, an agent is ingested and a specific investigation
level of consciousness or altered mental understanding of potential complications may aid diagnosis and/or management.
status, presence of delirium or psychosis and experience with previous similar cases. Screening tests in the poisoned child should
(including blood glucose level); At this stage, it is prudent to obtain advice be performed based on the risk assessment.

422
21.1 GENERAL APPROACH TO POISONING
21

POISONING
Table 21.1.3 Investigation of the poisoned child
Resuscitation
Investigation Potential toxicological indication(s)
In the severely poisoned child, timely and
Screening tests effective resuscitation is the key to better
Blood glucose level Altered mental status outcomes. Thankfully, this scenario is
Deliberate self-poisoning
All exposures to insulin or oral hypoglycaemic agents uncommon in Australasia. Resuscitation of
b-HCG pregnancy test Female patients of childbearing age presenting with overdose the poisoned child should follow standard
Paracetamol level Deliberate self-poisoning (of any substance) advanced paediatric guidelines with regards
Urine drug screen Known or suspected exposure to illicit substances/drugs of abuse to promoting haemodynamic stability, pre-
Altered mental status (delirium, psychosis, coma)
venting secondary brain injury and best
ECG Heart rate outside normal parameters for age
Haemodynamic instability or shock practice supportive care.
Poisoning with specific agents: The majority of cases require no more
Cardiovascular drugs
Sedatives than oxygen therapy and intravenous fluid
Neuroleptics boluses. In the presence of coma or cardio-
Antidepressants
Amphetamines and sympathomimetics vascular collapse, resuscitation involves air-
Clonidine and baclofen way protection and ventilatory support as
Metals (e.g. potassium, lithium, iron)
well as the potential use of agent-specific
Blood gas measurement Known or suspected acidbase abnormality
Poisoning with specific agents: antidotes. In cases where early decontami-
Salicylates nation is required, such as exposure or inges-
Tricyclic antidepressants
Ethanol tion of chemicals, decontamination should
Toxic alcohols occur concurrently with, and not to the det-
Iron
Isoniazid riment of, active resuscitation.
Carbon monoxide
Cyanide
Metformin
Methaemoglobinaemia
Decontamination
Specific investigations
Decontamination involves the removal of a
Chest/abdominal X-ray Radio-opaque tablet or foreign body ingestion
Known or suspected aspiration pneumonitis toxic substance to which a child has been
Tube (e.g. endotracheal, gastric) placement exposed in order to minimise its absorption
Liver function tests Known or suspected paracetamol poisoning into the systemic circulation. In a child with
Suspected hepatotoxicity from any systemic poisoning
Coagulation panel Snake bite
dermal, eye or mucosal exposure to a sub-
Suspected coagulopathy from poisoning/envenoming stance, decontamination simply involves
Poisoning with specific agents:
Paracetamol
removal of the toxic substance and irrigation
Anticoagulants (e.g. warfarin, rodenticides) or washing of the contaminated skin, eye or
Salicylates
Iron and heavy metals
mucosa. Inhalational injury to toxic fumes
Paracetamol level In all cases of paracetamol ingestion (incl. deliberate self-poisoning, or gases should include the removal of
accidental, supratherapeutic, chronic) the patient from the source of exposure
Specific drug levels Known or suspected poisoning from: and, if necessary, administration of supple-
Anticonvulsants (carbamazepine, valproic acid, phenytoin)
Aspirin/salicylates mental oxygen. In extreme situations, these
Digoxin patients may need advanced airway and
Metals (potassium, lithium, iron)
Ethanol ventilatory support. The use of universal pre-
Toxic alcohols (ethylene glycol, methanol) cautions (gown, gloves, goggles) by staff
Methotrexate
Theophylline during the process of decontamination is
Phenobarbital sufficient for the vast majority of poison-
Notes:
ing situations, including hydrocarbons and
Salicylate levels should not be routinely ordered as their screening value is negligible organophosphate insecticides.
Tricyclic antidepressant levels are not useful in the management of poisoning from these agents, nor are they a useful
screening test
Oral exposure to pharmaceuticals, chemi-
Rarely performed investigations for specific toxins include cholinesterase levels, carboxyhaemoglobin and cals or plants requires a risk assessment-based
methaemoglobin amongst others.
Computerised tomography of brain is not routinely required in the comatose child with a reliable history of poisoning; it
approach as to whether decontamination is
may be warranted when the history is unclear, or there is suspicion of trauma or non-accidental injury. deemed worthwhile. The need for active oral
HCG, human chorionic gonadotrophin.
decontamination in paediatric poisoning is
Specific investigations, which may be invasive and specific investigations in paediatric poi- rare. Clinicians are advised to seek expert
or time-consuming to return a result, should soning. Baseline blood investigations (blood advice prior to decontamination in children
be discussed with an expert toxicologist prior counts, electrolytes, renal function test) should with oral exposures.
to embarking on these tests. Table 21.1.3 sum- be performed if any of the screening or specific Syrup of ipecacuanha (derived from the
marises the potential indications for screening laboratory investigations are ordered. root of a South American plant) is no longer

423
21.1 GENERAL APPROACH TO POISONING

Table 21.1.4 Antidotes

Antidote or specific therapy Dose Potential indications

Atropine 0.020.05 mg kg1, repeat every 510 Known or suspected cholinergic toxidrome from organophosphate or carbamate
minutes (use doubling regimen) pesticide poisoning. Titrate to pupil size, heart rate, blood pressure and drying of
chest secretions.

Calcium Calcium gluconate Calcium-channel blockers:

10%, 0.6 mL kg1 Bradycardia or heart block
Calcium chloride 10%, 0.2 mL kg1 Hypotension

Desferrioxamine 15 mg kg1 hr1 Iron toxicity:

Serum iron >90 mmol L1
Clinically severe systemic toxicity

Digoxin Fab antibodies Empiric dose: Digoxin toxicity:

Acute poisoning - 5 vials Life-threatening arrhythmias
Chronic poisoning - 12 vials Clinical signs of severe digitoxicity

Ethanol PO/NG route preferred Known or suspected poisoning from toxic alcohols (ethylene glycol, methanol):
Loading dose: 750 mg kg1 Elevated osmolar gap
Infusion: 80150 mg kg1 hr1 Metabolic acidosis
Maintain blood ethanol at 100 mg dL1

Flumazenil 0.0050.01 mg kg1 Benzodiazepine poisoning:

(max 2 mg) Reduced level of consciousness
Bradypnoea
N.B. Titrate to respiratory rate

Glucagon 0.050.1 mg kg1 b-Blockers

Bradycardia
Hypotension

Insulin (high dose dextrose) Initial dose: 1 unit kg1 b-Blockers

Infusion: 12 units kg1 hr1 Calcium channel blockers
Bradycardia or heart block
Hypotension

N-Acetylcysteine 1st: 150 mg kg1 over 1530 min, Paracetamol poisoning:

2nd: 50 mg kg1 over 4 hours, Patients at risk of, or with established, hepatotoxicity
3rd: 100 mg kg1 over 16 hours

Naloxone Bolus: 0.005-0.01 mg kg1 (max 2 mg); Known or suspected opiate toxidrome. Titrate to respiratory rate
Infusion: 0.01 mg kg1 hr1

Octreotide Bolus: 1 mcg kg1 IV or SC Sulfonylurea poisoning with recurrent hypoglycaemia

Infusion: 0.5 mcg kg1 hr1 IV

Physostigmine 0.02 mg kg1 (max 0.5 mg) Anticholinergic poisoning with delirium
1
Sodium bicarbonate 12 mmol kg IV bolus (serum Cyclic antidepressants (and other cardiac sodium channel blocking agents):
alkalinisation) Cardiac arrest
Wide-complex tachyarrhythmias
Hypotension
Seizures

Vitamin K 510 mg, PO Poisoning from warfarin (or other coumadin anticoagulants) with established, or risk of,
12 mg, IV or IM coagulopathy

IM, intramuscular; IV, intravenous; PO, per oram; SC, subcutaneous.

recommended in the management of poi- polyethylene glycol solution through the charcoal in children is uncommon, it is
soning. The induced emesis does little to gastrointestinal tract for the purpose of pro- potentially indicated when a child ingests
prevent drug absorption and potentially moting tablet residue in effluent and thus a highly toxic substance, which is bound
can cause a myriad of complications in- preventing drug absorption. It is reserved by charcoal, and the charcoal can be ad-
cluding protracted vomiting, aspiration for specific ingestions such as sustained- ministered within an hour post-ingestion to
and oesophageal tears and haemorrhage. release preparations or metals; expert an alert child (or in the case of intubated
Gastric lavage (stomach pumping), involv- advice should be sought prior to instituting children, via a gastric tube). Clinicians should
ing the injection of fluid into the stomach whole bowel irrigation. avoid inserting a gastric tube for the pur-
via a tube and aspirating gastric contents, Activated charcoal is a colloidal suspen- pose of administering charcoal to a patient
is also a discontinued practice which has sion of charcoal particles able to bind to with altered level of consciousness. The pres-
little or no role in the management of most pharmaceuticals. Charcoal does not ence of bowel sounds should always be
poisoned children. Whole bowel irriga- adsorb metals, hydrocarbons, corrosives or confirmed prior to the administration of oral
tion involves the administration of a alcohols. Although the need for activated charcoal. When indicated, the activated

424
21.1 GENERAL APPROACH TO POISONING
21
charcoal dose is 1 g kg1. Expert advice

POISONING
used in childhood poisonings and expert include rhabdomyolysis (seen in snake bite,
should be sought regarding the use of char- advice should be sought prior to their prolonged coma), aspiration pneumonitis,
coal in situations beyond an hour post-inges- institution. and persistent delirium (commonly due to
tion or multi-dose activated charcoal anti-cholinergic drugs or plants).
(discussed below). Common side effects of
charcoal administration include vomiting Supportive care
and the passage of black stools. Aspiration Consultation and
of charcoal can lead to chemical pneumoni- Active resuscitation of the severely poisoned disposition
tis and potentially acute respiratory distress child is paramount and should be followed
by meticulous attention to supportive care Poisoning in children, as in adults, is a symp-
syndrome.
in a high dependency or intensive care tom of an underlying issue, be it psychologi-
environment. Although specific poisoning cal, parental neglect or accidental access to
scenarios are dealt with in the next chapter, harmful substances. The mainstay of man-
Antidotes
the guiding principles of excellent support- agement involves observation in the ED.
The need for agent-specific antidotes in chil- ive management of the poisoned child are Occasionally the child is exposed to an
dren is uncommon. Knowledge of antidotes likely to be more crucial. unusual substance or develops severe toxic-
and their potential utility may, in rare cases, All children with altered level of con- ity that requires input from clinical toxicolo-
be life-saving. Table 21.1.4 lists select anti- sciousness should have close glucose moni- gists. Clinicians should seek advice from
dotes used in paediatric poisoning and their toring. Coma from drug overdose should local experts in the field and/or their local
indications. The use of these agents should be managed with advanced airway and Poisons Information Centre.
be discussed with a toxicologist. ventilatory manoeuvres. In general, non- The underlying issue or disease process
invasive ventilation does not have a role in also requires attention, such as mental
the poisoned child. Drug-induced seizures health assessment or counselling. Children
Enhanced elimination who are overdosed with analgesics or anti-
from all causes should be treated with par-
Techniques used to promote drug elimina- enteral benzodiazepines as the first-line pyretics may warrant investigation into the
tion from the body are employed in a limited agents of choice. Phenytoin should be cause of pain or fever. In the case of neglect
number of poisonings. These methods avoided as its sodium channel blocking prop- or deliberate poisoning the child is likely to
include haemodialysis, multidose activated erties may exacerbate the problem. need referral to relevant child protection
charcoal and alkaline diuresis. Various forms Cardiovascular collapse and asystole in authorities. All carers involved in accidental
of haemodialysis methods are utilised in the poisoned child should be managed as poisoning should have counselling with
paediatric poisoning after insertion of a tem- per standard advanced paediatric life sup- regards to safe storage of medicines and
porary vascular catheter. Agents that are port guidelines. Drug-induced arrhythmias chemicals in the home.
potentially dialysable include potassium, may warrant agent-specific strategies, such
salicylates, toxic alcohols, theophylline and as antidotes. Wide QRS complex tachyar-
carbamazepine, amongst others. Multidose rhythmias, usually due to sodium channel Further reading
activated charcoal is rarely used when pro- blocking agent poisoning, should in the first Bronstein AC, Spyker DA, Cantilena LR Jr, et al. 2008 Annual
moting charcoal adsorption and elimination instance be treated with boluses of sodium report of the American Association of Poison Control
Centers National Poison Data System (NDPS): 26th Annual
of poisons that undergo enterohepatic bicarbonate (12 mmol kg1). Report. Clin Toxicol 2009;47(10):9111084.
circulation, such as carbamazepine. Urinary Close monitoring and maintenance of Including beta-blockers, calcium channel blockers, clonidine,
chloroquine, salicylates, phenothiazines, sulfonylureas,
alkalinisation with sodium bicarbonate normothermia, euglycaemia, acidbase bal- opiates and others. J Emerg Med series on paediatric
is indicated in salicylate poisoning to ance and electrolyte levels are vital in the exposures of 12 pills 2004;26.
NSW Poisons Information Centre Annual Report. Westmead,
promote the renal excretion of salicy- severely poisoned child. Other potential NSW Australia: The Childrens Hospital; 2009. www.
late ions. These techniques are seldom complications in this group of patients poisonsinfo.nsw.gov.au.

425
 21.2 Specific poisons
Naren Gunja Helen Mead Nicholas Cheng

ingestion in these children, a 2-hour level

ESSENTIALS above 1500 mmol L1 suggests risk of hepa-
totoxicity. Other relevant investigations
1 Medically-important paediatric poisoning usually falls into two categories: include electrolytes, renal function, liver
common ingestions, and rare or dangerous toxins.
function tests and coagulation panel.
2 The Emergency Physician plays a crucial role in forming an appropriate The paracetamol nomogram (Fig. 21.2.1),
management strategy based on the risk and knowledge of the relevant drug(s) based on adult toxicity profiles, is extrapo-
toxicity. lated to children and predicts the potential
for significant hepatotoxicity. The nomo-
3 The management of common ingestions is essentially supportive, with the gram is applicable when a paracetamol level
potential utility of a specific antidote. Gastrointestinal decontamination is rarely
is taken between 4 and 16 hours post-acute
indicated in these cases.
ingestion. The nomogram cannot be utilised
4 Although most substances are harmless to children in small amounts, a few when the time of ingestion is unknown or in
pharmaceuticals and chemicals are extremely toxic in minute quantities these the case of staggered or chronic ingestions.
substances are potentially lethal in a toddler even if only one to two pills or one to two Paracetamol treatment guidelines were
mouthfuls are ingested. reviewed by a representative panel of Clini-
cal Toxicologists to the Australasian Poisons
Information Centres and published in 2008.
A major shift in management was the
Children appear less sensitive to the hepa- amalgamation of the high- and low-risk
Common poisons totoxic effects of acute paracetamol overdose nomogram treatment lines into a single
Paracetamol than adults. This may be related to metabolic curve for both adults and children. This cur-
Paracetamol is by far the commonest paedia- differences, age-related clearance rates and rent line commences at 1000 mmol L1 at
tric poisoning presentation to the Emergency to the increased propensity for children to 4 hours post-ingestion and has a half-life
Department (ED), but the large majority will vomit after acute paracetamol ingestion. of 4 hours.
have no toxic effects. Absorption from the The peak serum concentration is reached in N-acetylcysteine (NAC), a glutathione pre-
gastrointestinal (GI) tract is rapid, particularly <2 hours in the majority of children having cursor, is the antidote of choice in paraceta-
with the liquid formulation (approx. 30 min- a single ingestion of paracetamol elixir. mol poisoning. NAC is indicated in the
utes). Most of the paracetamol is conjugated In the early phase, <24 hours, a child may setting of acute paracetamol ingestion,
by the hepatic pathways of sulfation and glu- be totally asymptomatic or complain only of where the measured paracetamol level falls
curonidation to inactive metabolites, which mild abdominal pain, nausea and vomiting. above the nomogram treatment line. The full
are excreted in the urine. Children under Following a period of latency, hepatoxicity course involves a loading dose of 150 mg
the age of 912 years have a more active sul- progresses to multiorgan failure. Paraceta- kg1 in dextrose over 1560 minutes, fol-
fation pathway. Less than 5% is excreted mol can directly cause renal impairment lowed by a second infusion of 50 mg kg1
unchanged by the kidney and 515% is and coagulopathy with prolonged prothrom- over 4 hours, and finally a 100 mg kg1 infu-
oxidised by the hepatic cytochrome P450 bin time. The hepatorenal syndrome can sion over 16 hours. NAC is preferably com-
enzyme system to form a highly reactive inter- also complicate severe hepatotoxicity. menced within 8 hours of ingestion, but
mediate metabolite NAPQI (N-acetyl-p- Finally, fulminant hepatic failure may occur has been documented to be effective in adult
benzoquinone imine), which binds to hepato- or the patient may enter the recovery phase patients when given up to 48 hours after a
cytes and leads to oxidative stress and cell with return to normal hepatic function serious ingestion, and can even be consid-
death. With therapeutic dosing, NAPQI is within 4 weeks. Rare consequences of para- ered later when hepatic failure is established.
metabolized to a non-toxic metabolite with cetamol toxicity include myocardial necrosis, Anaphylactoid reactions, including rash,
glutathione as a sulfhydryl donor. In over- haemolytic anaemia, methaemoglobinae- bronchospasm, pruritus, hypotension and
dose, when glutathione reserves are depleted, mia, skin rashes and pancreatitis. tachycardia, occur in up to 15% of cases,
NAPQI accumulates and causes hepatotoxic- In acute overdose, a serum level should be and are most common during the second
ity. Acute toxicity from accidental ingestion is obtained 4 hours post-ingestion in all chil- infusion. These reactions are managed simi-
extremely rare in children. Paracetamol toxic- dren with potential paracetamol ingestions larly to other hypersensitivity reactions; the
ity is more likely to be problematic in children of greater than 200 mg kg1. Children with NAC infusion should be temporarily ceased
taking standard doses of paracetamol chroni- acute single ingestions of liquid paraceta- and recommenced at half the rate.
cally or in repeated supratherapeutic doses, mol preparations are likely to have reliable As children usually ingest the elixir formu-
rather than after a single ingestion. post-peak levels earlier than 4 hours post- lation rather than tablets, rapid absorption

426
21.2 SPECIFIC POISONS
21

POISONING
at benzodiazepine receptors. Drowsiness,
160 slurred speech and ataxia are the most com-
1000 150 mon manifestations. This may progress to
140 coma and hypotension, hypothermia and
900
130 respiratory depression in more significant
800 120
ingestions. Death is rare unless other CNS
Serum paracetamol (mol L-1)

Serum paracetamol (mg L-1)

110
700 depressants have been co-ingested.
Hepatotoxicity likely 100
600 90
Management is entirely supportive. Hypo-
80 tension usually responds to fluid administra-
500 tion. GI decontamination is not indicated in
70
400 60 pure benzodiazepine poisoning. A blood
50 sugar level should be checked in all children
300
Hepatotoxicity unlikely 40 with an altered level of consciousness. Other
200 30 laboratory investigations are not routinely
20 indicated. Chest radiography is only indi-
100
10 cated if aspiration is suspected. A qualitative
0 0
4 5 6 7 8 9 10 11 12 13 14 15 urine test for benzodiazepines may provide
Time elapsed since paracetamol ingestion reassurance as to the aetiology of drowsi-
ness in the setting of an unconscious patient
without a clear history of ingestion. In some
clinical scenarios, flumazenil may avert the
Fig. 21.2.1 Paracetamol nomogram.
need for intubation and mechanical support
but its use should be discussed with a toxi-
cologist. Flumazenil should be administered
precludes the utility of oral activated char- NSAIDs are generally of low toxicity, produc-
in boluses of 510 mcg kg-1 and titrated to
coal. Activated charcoal can be adminis- ing gastrointestinal upset, headache, dizzi-
clinical effect (respiratory rate and effort).
tered for potentially toxic doses of the ness, tinnitus, and visual disturbance.
Most children can be discharged after 46
tablet formulation, if given within 1 hour. Hypotension, tachycardia, and hypothermia
hours if vital signs are satisfactory and the
Children who present with established and coagulopathy have been reported when
child can walk unaided.
hepatic failure must receive prompt resusci- NSAIDs were consumed in large amounts. In
tation and stabilisation. NAC is still indi- massive overdose, electrolyte disturbances,
cated when hepatotoxicity is established. metabolic acidosis, central nervous system
Opioids
Coagulopathy and encephalopathy should depression, and respiratory failure can occur.
Opioids are frequently available where fam-
be managed as from other causes of liver Asymptomatic children can be discharged
ily members suffer chronic pain, abuse drugs
failure. These children are managed in the at 4 hours post-ingestion. Symptomatic chil-
or are on drug-rehabilitation programmes.
intensive care and should be assessed for dren require hospital admission. Oral fluids
Iatrogenic intravenous overdosing of chil-
potential liver transplantation. should be encouraged and dehydration cor-
dren is commonly a result of 10-fold errors
Chronic over-dosing or repeated suprather- rected. Electrolytes, blood sugar level, renal
in dose calculation.
apeutic ingestion is a significant problem. The function and acidbase status should be
Morphine and codeine are natural opium
treatment nomogram is not applicable to monitored. Activated charcoal may be indi-
alkaloids. Other opioids are synthetic or
these situations and cases should be discussed cated in massive ingestions that present
semisynthetic analogues. The opioids act
with a toxicologist. Children with paracetamol early. Methods of enhanced elimination
on various receptors on the brain, spinal cord
levels below the nomogram treatment line are not usually beneficial.
and gastrointestinal tract as full or partial
after acute single ingestions can be cleared
agonists or antagonists. Opioids are well
from a toxicological point of view. Parents or
Benzodiazepines absorbed by all routes except skin, are meta-
carers should be educated on correct paracet-
Benzodiazepine overdose is commonly seen bolised by the liver and are renally excreted.
amol dosing and safe storage. Children with
in toddlers who ingest 12 tablets, or as Some opioids (e.g. pethidine and diphenoxy-
deliberate self-poisoning should be assessed
part of a mixed overdose in adolescents. late) have potent metabolites. Opioids vary
by the mental health team.
Benzodiazepines bind predominantly to the in their duration of action and some are
g-aminobutyric acid (GABA) type-A receptor available as sustained release prepara-
NSAIDs complex in the central nervous system (CNS) tions (e.g. morphine). Toxicity is enhanced
Of the many non-steroidal anti-inflammatory and enhance GABA activity to produce by co-ingestion of other sedative medica-
drugs (NSAIDs) available, some obtainable sedative, anxiolytic and anticonvulsant tions, which may be found in some cough
as over-the-counter medicines without pre- activity. The duration of sedation ranges remedies or analgesic preparations. Children
scription, ibuprofen is the most significant from 436 hours, depending on the agent. are especially sensitive to the depressive
NSAID ingested by children. As a group Flumazenil is a competitive antagonist effects of opioids.

427
21.2 SPECIFIC POISONS

Paediatric overdose of a parents metha- many of which can be bought over the observed for a minimum of 6 hours following
done (long-acting opioid) syrup requires over- counter. Pharmaceuticals with prominent ingestion. Management is supportive and
night admission for extended observation anticholinergic properties include first- the anticholinergic effects of antihista-
and monitoring. Antidiarrhoeal preparations generation antihistamines, antipsychotics mines, while unpleasant, are generally not
contain diphenoxylate (with atropine) and and tricyclic antidepressants. Some plants, life threatening.
produce delayed onset of symptoms, with e.g. Jimsonweed, angels trumpet, and Hypotension should be treated with intra-
numerous paediatric deaths reported. A mushrooms contain alkaloids with potent venous fluids. Convulsions and anticholiner-
major metabolite of diphenoxylate is more anticholinergic effects. gic delirium are best managed with
potent than the parent compound and under- The anticholinergic toxidrome is caused benzodiazepines. Continuous ECG monitor-
goes enterohepatic circulation. Dextropropoxy- by competitive inhibition of the muscarinic ing is advised for symptomatic children
phene has a membrane-stabilising effect on receptor in the autonomic nervous system. and those with persistent tachycardia. Ven-
cardiac conducting tissue and may induce The classic anticholinergic toxidrome is well tricular arrhythmias should be managed
ventricular arrhythmias and heart block. described by the following rhyme: with sodium bicarbonate as the drug of
The classic features are of nausea and choice for QRS prolongation with sodium-
vomiting, drowsiness, pinpoint pupils, respira-
Hot as a hare hyperthermia from channel blocking antihistamines.
inability to sweat.
tory depression, and occasionally bradycardia
and hypotension. Respiratory depression may
Blind as a bat dilated pupils with loss of
Corrosive ingestions
accommodation.
lead to hypoxia and respiratory arrest. The Most serious caustic ingestions involve
histamine-releasing effects of some opioids
Dry as a bone hot, dry skin and mucous
strong acids and alkalis which account for
membranes with paucity of secretions.
may cause urticaria and hypotension. a high number of presentations to the ED.
Management of the opioid toxidrome is
Red as a beet erythematous skin.
The initial presentation and treatment are
essentially supportive, with attention to
Mad as a hatter central anticholinergic
similar to other burns. Domestic bleaches
delirium manifest by agitation, confusion,
airway and ventilation. All children with and ammonia products generally cause
hallucinations (often visual), dystonic
altered level of consciousness should have minor injuries. Serious injuries result most
movements and seizures.
a blood sugar level checked. Activated char- often from the ingestion of drain and oven
coal may be considered for massive inges- Other anticholinergic effects include tachy- cleaners (NaOH, KOH). Dishwashing powder
tions or long-acting preparations. Insertion cardia, gastrointestinal ileus and urinary residue left in the dispenser of machines is a
of gastric tubes for charcoal administration retention. commonly accessed alkali that may cause
is not recommended unless the child is intu- The management of anticholinergic poi- serious injury.
bated for other indications. soning includes attention to the ABCs with The severity of burn depends on the
Naloxone, the antidote for opiate toxi- appropriate supportive therapy and moni- nature, volume, pH and concentration of
city, is a competitive antagonist at opioid toring of vital signs and mental state. Symp- the agent and the duration of contact. Stom-
receptors. Naloxone (intravenously (IV) tomatic patients should have IV access, a ach contents may afford some protection
0.01 mg kg
1, maximum 2 mg, as a bolus, 12-lead electrocardiogram (ECG) and contin- from injury, but pylorospasm, oesophageal
repeated every few minutes till appropriate uous cardiac monitoring. Benzodiazepines reflux and vomiting may exacerbate injury.
response) may be useful to reverse the neu- are useful in managing agitation or seizures. Liquids may cause a circumferential injury
rological and respiratory depression, and Physostigmine is a cholinesterase inhibitor and powders/granules or tablets may cause
should be titrated to respiratory rate and which enters the CNS and is effective at prolonged contact with a mucosal surface,
effort. Naloxones short therapeutic half-life reversing central anticholinergic delirium. with potential for linear burns, deep erosion
of 3060 minutes may necessitate a contin- Due to concerns regarding adverse cardiac and penetration. Acids cause superficial cor-
uous infusion, in order to maintain the rever- side effects, the use of physostigmine should rosion and a coagulative necrosis, and the
sal and obviate the need for mechanical be discussed with a toxicologist. The initial extent of tissue penetration is limited by
ventilation, particularly for ingestions of dose in children is 0.02 mg kg
1 (maximum eschar formation. Alkalis start to burn imme-
long-acting opiates. 0.5 mg) by slow IV push; doses may be diately on contact and cause a liquefaction
All patients should be observed for a mini- repeated every 15 minutes. necrosis of fat and protein, penetrating
mum of 6 hours. Methadone, dextropropoxy- Aggressive cooling measures may be deeply into tissues. Acids typically injure
phene and sustained-release morphine required in severe hyperthermia. Urinary the stomach while alkalis damage the oro-
sulfate may cause symptoms persisting for catheterisation is required for patients with pharynx and oesophagus.
2448 hours so prolonged observation is urinary retention. Asymptomatic patients Many children will be asymptomatic,
necessary after ingestion of these agents. may be discharged after 6 hours. Patients especially if low-concentration household
with moderate or severe toxicity should be products are involved. Pain, drooling, dys-
Anticholinergics and admitted to an intensive-care facility. phagia, vomiting and abdominal pain and
antihistamines Antihistamine poisoning in children is of haematemesis may occur. Airway compro-
Anticholinergic (antimuscarinic) poisoning concern only for first-generation agents mise with laryngeal oedema, cough and
can result from a diverse range of therapeu- (e.g. promethazine), which have significant bronchospasm may be seen after ingestion
tic substances, plants and natural remedies, anticholinergic effects. Children should be of high-concentration agents. Endoscopy

428
21.2 SPECIFIC POISONS
21

POISONING
provides the best guide to prognosis and antibiotics are required if perforation or pen- the clinical state. Hypoglycaemia should
management. The extent of injury is graded etration is suspected clinically, on endoscopy be corrected with 5 mL kg
1 10% dextrose.
by the depth of ulceration and the presence or contrast radiography. Steroids have no Hypotension will usually respond to intrave-
of necrosis. Typically, after ingestion the proven benefit and may possibly increase nous fluids and acidosis usually responds
mouth or oesophagus is red and ulcera- the risk of perforation. to correction of hypovolaemia and hypogly-
tion follows within 24 hours. One-third of Asymptomatic patients should be advised caemia. Hypothermia should be corrected.
patients with oral burns have associated to return if they develop respiratory diffi- Activated charcoal does not bind ethanol
oesophageal lesions, whereas 1015% of culty, pain or dysphagia. All symptomatic but may be considered if co-ingestants are
patients with oesophageal lesions have children should be admitted for observation suspected, provided the airway is protected.
no oropharyngeal burns. Asymptomatic and potential endoscopy. Gastric lavage is likely to be ineffective due
patients with no oral burns may have signif- to the rapid absorption from the stomach.
icant oesophageal injuries. Drooling and Ethanol Haemodialysis may be indicated in the
dysphagia persisting beyond 1224 hours Ethanol is available in numerous household extremely intoxicated child who is haemody-
are reliable predictors for oesophageal scar medicinals, mouthwashes and perfumery namically unstable, but this is uncommon.
formation and suggest the need for upper products as well as alcoholic drinks. All pro- Admit all children who are clinically intoxi-
GI endoscopy. ducts marketed in Australia as methylated cated until asymptomatic.
Oesophageal perforation and mediastini- spirits contain ethanol. Although frequently
tis may be suspected by chest pain, fever, ingested by children, serious toxicity is
pleural rub, dyspnoea. Abdominal pain, uncommon. Rare and dangerous
fever, peritonism and ileus may indicate Ethanol is well absorbed across gastroin- poisons
gastric or abdominal oesophageal perfora- testinal mucosa and respiratory tract, most
tion. These signs may progress to septic within 3060 minutes, and distributes to Although most substances are harmless to
shock, multiorgan failure and death. Large total body water. Children metabolise alco- children in small amounts, a few pharmaceu-
acid ingestions may be associated with hol faster than adults. Only very small ticals and chemicals are extremely toxic in
hypotension, metabolic acidosis, haemolysis, amounts are excreted unchanged in the minute quantities. Table 21.2.1 lists drugs
nephrotoxicity and pulmonary oedema. urine and the breath. Hypoglycaemia is where one pill can kill and chemicals where
Late complications are infection, achlor- caused by depressed gluconeogenesis. The a sip or mouthful is potentially lethal.
hydria and stricture formation in 13%. potentially fatal dose of alcohol for children
All patients with full-thickness and 70% is 4 mL kg
1 of absolute alcohol (e.g. Salicylates
with deep ulceration will develop strictures. 10 mL kg
1 for a 40% alcohol spirit), about The incidence of acute salicylate poisoning
Eighty percent of all strictures occur within half the dose required for adults. Quite low has declined due to improvements in medi-
2 months of ingestion and 99% within serum levels (>10 mmol L
1, >0.05% or cation packaging, removal of aspirin from
1 year. >500 mg L
1) may produce clinically signif- oral paediatric formulations and due to
The management of caustic ingestions is icant effects in children. paracetamol now being the favoured over-
aimed at limiting the extent of injury and pre- Ethanol acts on the reticular activating the-counter analgesic. Methylsalicylate and
venting strictures and other complications. system to cause CNS depression. Low con- salicylic acid are common in many topical
Immediate management consists of rinsing centrations result in alterations of mood preparations. Oil of Wintergreen containing
the skin with water or drinking water unless and thought processes, whereas higher con- methylsalicylate can be significantly toxic
respiratory distress is notable or visceral per- centrations affect cerebellar function, caus- when ingested. Choline salicylate is a con-
foration is suspected. Acids and alkalis do not ing ataxia and slurred speech. Higher stituent of many teething gels.
bind to charcoal. Attempts to neutralise the levels still depress all cortical function and Aspirin is rapidly absorbed from the upper
substance are contraindicated, but dilution brainstem activity, depressing respiratory gastrointestinal tract, with peak plasma con-
with water may possibly be helpful for acids drive and protective airway reflexes. Respira- centrations at 13 hours after therapeutic
and may reduce mucosal contact time in tory arrest or aspiration is a frequent cause doses. An ingestion of 150300 mg kg1
ingestion with particulate alkalis. Early of death. Facial flushing, excessive sweating may result in mild to moderate toxicity.
treatment focuses on ensuring an adequate and vomiting are common. Ingestions over 500 mg kg1 are potentially
airway, intravenous fluid replacement, moni- Management depends on the time lethal. Oil of Wintergreen is 100% methylsa-
toring fluid balance, avoiding vomiting and elapsed since ingestion. Assess and secure licylate and extremely small amounts may
adequate analgesia. Oesophagoscopy in the the ABCs and correct electrolyte abnormal- be lethal.
symptomatic patient guides further manage- ities and dehydration. Patients with severe Aspirin is hydrolysed to form salicylic acid
ment. Patients with deep, especially circum- CNS depression are at risk of aspiration (salicylate). In large overdoses, the potential
ferential burns of the oesophagus should be and require airway protection. Blood glu- for pharmacobezoar formation in the gut
admitted to an intensive care unit and may cose should be monitored. A blood alcohol may alter absorption kinetics. In therapeu-
require prolonged parenteral feeding and level may be taken at least 1 hour post- tic doses, salicylate is 8595% plasma
repeated endoscopic stricture dilatations. ingestion if symptoms are present, although bound, but in overdose, free salicylate
Early surgical intervention and prophylactic management is generally determined by concentration rises as plasma protein

429
21.2 SPECIFIC POISONS

hyperkalaemia. The slowing of conduction,

Table 21.2.1 Potentially lethal ingestions in small amounts for a toddler
as well as increased refractory period,
Pharmaceuticals Chemicals through the AV node, enhanced automatic-
CCBs (diltiazem & verapamil) Organophosphate & carbamate pesticides ity of the Purkinje fibres and enhanced vagal
tone leads to a multitude of arrhythmias
Chloroquine & hydroxychloroquine Paraquat
including sinus bradycardia, sinoatrial
Clonidine Camphor arrest, conduction blocks, ventricular tachy-
Tricyclic antidepressants Naphthalene cardia and fibrillation.
Early signs of chronic toxicity include nau-
Sulfonylureas Toxic alcohols
sea, vomiting and diarrhoea. The child is
Amphetamines & ecstasy Essential oils often asymptomatic in acute poisoning, until
haemodynamic instability from cardiac tox-
icity becomes clinically apparent. Patients
binding is saturated. As metabolic pathways maintenance bicarbonate doses ranging can deteriorate suddenly and digitoxicity
in the liver become saturated, the kidney from 12 mmol kg
1 hr
1. Haemodialysis is can produce a myriad of both brady- and
becomes the main route of elimination. indicated in severe toxicity with acidaemia, tachyarrhythmias. Assess ABCs, secure IV
Clearance is markedly enhanced by an alka- cardiorespiratory failure, renal impairment access and continuously monitor blood pres-
line urinary pH. or CNS manifestations (coma, seizures). sure (BP) and ECG. Although digoxin is well
Salicylate poisoning leads to the uncou- Complications of salicylate poisoning require bound by activated charcoal, repeated
pling of oxidative phosphorylation and aggressive supportive management. The vomiting may reduce its effectiveness.
anaerobic metabolism. The resulting lactic development of non-cardiogenic pulmonary Obtain a serum digoxin concentration and
acidosis is more prominent in young chil- oedema often signals the need for invasive electrolytes. Serum potassium concentration
dren. Early signs of salicylism include a ventilation and haemodialysis. Seizures war- should be monitored every 4 hours. Hyperka-
respiratory alkalosis from centrally driven rant benzodiazepine therapy and correction laemia should be corrected to within upper
hyperventilation and tinnitus. With increas- of any glucose or electrolyte derangement. limits of normal with sodium bicarbonate
ing toxicity, confusion, hallucinations and Observe all symptomatic children and and insulin/dextrose. Calcium is relatively
seizures are reported. Metabolic derange- those with ingestions of greater than contraindicated due to potential for myocar-
ments include temperature dysregulation, 150 mg kg
1. Most patients will require dial destabilisation.
impaired glucose metabolism and trans- admission for 612 hours for observation Digoxin Fab antibodies are a specific and
port as well as electrolyte abnormalities. of clinical state and serum salicylate level. highly effective antidote in digoxin poison-
Mixed picture acidbase derangement is ing. Intravenous Fab fragments of digoxin-
a hallmark of severe salicylate poisoning, Digoxin specific antibodies are first-line therapy
with serum pH below 7.3 being a late Digoxin is a cardiac glycoside used for man- for patients with cardiac arrhythmias with
and ominous sign. Coagulopathy may result agement of heart failure and supraven- haemodynamic instability. The dosage is
from competitive inhibition of synthesis tricular arrhythmias. Many plants contain based on total body load, estimated from
of vitamin-K dependent factors. Non- digitalis glycosides (e.g. foxglove, oleander) the serum digoxin concentration or from
cardiogenic pulmonary oedema has been and poisoning from these plants should be the ingested dose. Alternatively, a dose
reported, but the mechanism is unclear. managed in a similar manner to digitoxicity. estimation can be made on the presump-
Investigations include baseline blood Acute digoxin poisoning in children is more tion that one vial of 40 mg will bind
sugar level, electrolyte and renal function, often seen in the context of toddlers who 0.6 mg of digoxin. A clinical response is
acidbase status, coagulation panel and obtain access to grandparents medication. seen in 2030 minutes, with maximum
6-hour salicylate level. Decontamination Rarely, children with underlying cardiac dis- effect at 24 hours. An empiric dose of
with activated charcoal is indicated in early ease on maintenance digoxin therapy can 5 vials of digoxin Fab may be given IV over
presentations and may be considered in late develop chronic toxicity and therapeutic 20 minutes in severe life-threatening toxic-
presentations of enteric-coated prepara- drug monitoring is crucial. Chronic overdos- ity. It is important to note that subse-
tions. Meticulous monitoring of fluid bal- ing or renal impairment can lead to chronic quent digoxin levels post-treatment with
ance, temperature, glucose and electrolyte digitoxicity. antibodies are not interpretable and
levels is recommended. In particular, potas- Digoxin is well absorbed from the gastro- should not be performed.
sium replacement is often required, along intestinal tract and has a relatively large Acute overdoses should be observed for a
with maintenance of urine output. volume of distribution. Digoxin is predomi- minimum of 12 hours or overnight. Symp-
Methods of enhancing elimination should nantly excreted unchanged by the kidney, tomatic patients should be monitored in
be instituted following consultation with a with an elimination half-life of about an intensive care unit or coronary care facil-
toxicologist. Urinary alkalinisation with intra- 36 hours. Digoxin inhibits the action of ity. Patients treated with Fab fragments
venous sodium bicarbonate infusion is known the cardiac Na/K ATPase pump and accu- should be monitored for subsequent hypoka-
to enhance renal excretion of salicylate ions. mulation of sodium and calcium ions leads laemia and for deterioration of pre-existing
The target urinary pH is at least 7.5 with to intracellular depletion of potassium and cardiac disease.

430
21.2 SPECIFIC POISONS
21
units kg
1 hr
1 infusion) and glucagon

POISONING
Calcium-channel blockers myocardial contractility independently of
Calcium-channel blockers (CCBs) are widely (0.050.1 mg kg
1 IV bolus). Rarely, more b-receptor activation and is currently the
used in the treatment of hypertension, coro- extraordinary measures may be necessary preferred inotrope over glucagon. Doses
nary artery disease and supraventricular such as transvenous pacing, cardiopul- are similar to those above in calcium chan-
tachyarrhythmias. monary bypass or aortic balloon pumps. nel blocker poisoning. Extreme measures
CCBs are rapidly absorbed from the gas- Prolonged resuscitation and aggressive sup- such as transvenous pacing and cardiopul-
trointestinal tract and have peak plasma portive care may allow the peak toxicity to monary bypass may be required in cases of
concentrations ranging from 30 minutes pass and improve survival. intractable hypotension. Importantly, pro-
(nifedipine) to 90 minutes (verapamil), but Symptomatic children should be moni- longed resuscitation and aggressive sup-
sustained-release preparations are asso- tored in an intensive-care setting. Obser- portive care may allow the peak toxicity to
ciated with longer times to peak concentra- vation for 24 hours is warranted for pass and improve survival. Hypoglycaemia
tion and prolonged clinical effect. These ingestion of sustained-release formulations. should be treated in the usual manner with
agents inhibit the entry of calcium into the 2.55 mL kg
1 10% dextrose. Seizures may
cells of cardiac and smooth muscle, decreas- b-Blockers respond to IV dextrose, even if blood glucose
ing the activity of the calcium-dependent b-Blockers have wide clinical use in the is normal. Benzodiazepines are the preferred
actin-myosin ATPase. Dihydropyridines treatment of cardiac conditions, hyper- anticonvulsant. Bronchoconstriction should
(represented by the prototype agent nifedi- tension, thyrotoxicosis and prophylaxis for be treated with inhaled b2-agonists. Symp-
pine) are more potent at peripheral vascular migraine. tomatic children should be monitored in
calcium channels and have little cardiac tox- b-Blockers are class II antiarrhythmics, an intensive-care setting.
icity. Verapamil and diltiazem are highly which act by competing with catecholamines
toxic drugs where a single large dose tablet at b-receptor sites. Different b-blockers Clonidine
can cause profound cardiogenic shock in a have differing cardioselectivity, membrane- Until recently clonidine was used primarily
toddler. Dihydropyridine CCBs are unlikely stabilising activity, partial agonist activity as an anti-hypertensive agent and accessi-
to cause hypotension or cardiac conduction and lipid solubility. They are well absorbed bility to children was limited. The drug is
abnormalities in small doses. from small intestine, with peak serum levels now widely prescribed in the treatment of
The features of cardiotoxicity include within 14 hours. The elimination half-life is attention deficit hyperactivity disorder, con-
brady-arrhythmias, such as sinus bradycar- less than 12 hours. They have a moderate duct disorders, Tourettes syndrome and for
dia, varying degrees of AV block, and asys- to large volume of distribution. Highly lipid narcotic and alcohol withdrawal symptoms.
tole. Myocardial depression may cause soluble drugs, such as propranolol, cross the Clonidine overdose is commonly seen in chil-
congestive failure or cardiogenic shock. bloodbrain barrier and thus have more dren with behavioural disorders, and their
Dose-dependent peripheral vasodilatation potent CNS effects. Propranolol is also known siblings who have access to clonidine.
with hypotension may occur. Other manifes- for its cardiac sodium channel blocking prop- Clonidine is a central a2-adrenoceptor
tations include nausea and vomiting, leth- erties, which cause prolongation of the QRS agonist that acts on brainstem receptors,
argy, coma, seizures, hyperglycaemia and complex. causing inhibition of sympathetic outflow.
lactic acidosis. The major clinical effects are the cardio- Its stimulation of peripheral a2-receptors
Good supportive management is essen- vascular effects. Bradycardia may be sinus, on vascular smooth muscle may cause tran-
tial. Intravenous access should be secured, junctional or ventricular and may progress sient hypertension, but hypotension usually
and blood glucose and electrolytes to cardiac arrest. Hypotension results from occurs subsequently. Clonidine also has
measured. ECG and BP should be continu- bradycardia, myocardial depression and opiate-like effects which may be mediated
ously monitored. Activated charcoal is worth vasodilatation. Deterioration can be sudden through mu receptors. It is rapidly absorbed
considering in patients that present early and precipitous, particularly with proprano- and distributed, with peak plasma concen-
with a significant ingestion. Whole bowel lol, which can cause seizures, coma and wide trations 6080 minutes post-ingestion.
irrigation with polyethylene glycol should complex arrhythmias. Hypoglycaemia may The elimination half-life is 624 hours.
be considered for large ingestions of sus- occur due to impaired gluconeogenesis Clinical effects are seen 3060 minutes
tained-release preparations. and glycogenolysis. Bronchospasm is more after ingestion. Depression of the CNS with
Calcium is the initial antidote for hypoten- likely in atopic subjects, and more prominent lethargy and impaired conscious state is
sion and bradycardia (bolus: 10% calcium with non-selective agents. the most frequent manifestation. Miosis
chloride 0.2 mL kg
1 or 10% calcium gluco- Good supportive management is essen- and hypothermia may be observed. Symp-
nate 0.6 mL kg
1). Atropine is likely to be tial. Activated charcoal is the decontamina- toms are minimal with ingestions of under
ineffective. Catecholamine infusions (e.g. tion method of choice. Intravenous access 10 mcg kg
1, but cardiovascular compromise
adrenaline (epinephrine) commencing at should be secured, and blood glucose and with hypotension and bradycardia may
1 mcg kg
1 min
1) may be required. Other electrolytes measured. ECG and BP should occur after ingestion of 1020 mcg kg
1.
inotropes that do not require calcium influx be continuously monitored. Cardiovascular Respiratory depression and apnoea may
are potentially useful in managing intracta- effects should be treated with atropine, be seen after ingestions of 20 mcg kg
1.
ble shock from CCBs. These include high- volume expansion and catecholamines. There have been no reports of in-hospital
dose insulin (12 units kg
1 IV bolus; 12 High-dose insulin enhances heart rate and paediatric deaths.

431
21.2 SPECIFIC POISONS

Treatment is largely supportive. Activated The management of TCA poisoning Patients who ingest >5 mg kg
1 TCA
charcoal is only useful if given under 1 hour includes attention to the ABCs, good sup- should be admitted for observation for at
post-exposure. Hypotension should be trea- portive therapy and GI decontamination least 6 hours, but may be discharged at that
ted with volume expansion and vasopres- for potentially serious ingestions. Continu- time if the ECG remains normal and the child
sors. Hypertension is usually transient and, ous cardiac monitoring, serial 12-lead ECGs is well. TCA ingestions with significant CNS
if treatment is required, a short-acting agent and close observation of vital signs and men- depression, seizures or significant cardiotoxi-
such as nitroprusside should be used. Atro- tal state are required for all ingestions of city should be admitted to an intensive-care
pine may be useful in the treatment of bra- >5 mg kg
1. Secure IV access. Airway pro- facility.
dycardia. Naloxone therapy in clonidine tection should precede administration of
poisoning is controversial and unreliable. charcoal if the patient is less than fully
There may be inconsistent reversal of the conscious. Iron
neurological, cardiovascular and respiratory Depressed conscious state is the best pre- Iron tablets are commonly available in the
effects after administration of naloxone. dictor of serious toxic complications (sei- homes of toddlers but severe poisoning is
Maximal toxicity is expected in the first zures, ventricular arrhythmias, hypotension uncommon.
6 hours and children who show no symptoms and the need for mechanical ventilation) The amount of elemental iron varies
at that stage can be discharged. Children and the ECG limb lead QRS duration of according to the formulation. Initial toxicity
with significant respiratory and cardiovascu- 100 ms or greater is associated with an is due to the corrosive effects on the gastroin-
lar compromise may require admission to an increased incidence of seizures and cardio- testinal tract. Iron is absorbed in the ferrous
intensive-care unit for up to 24 hours. toxicity. Early intubation and hyperventila- state and after oxidation to the ferric state
tion to a serum pH of 7.457.55 may becomes bound to ferritin. Toxicity occurs
attenuate or prevent seizures and ventricu- when ferritin and transferrin are saturated
Tricyclic antidepressants lar dysrhythmias. Intubate and mechanically and serum iron exceeds the total iron-binding
Despite the declining prescription of tricyclic ventilate all patients with rapidly decreasing capacity (TIBC). High concentrations of intra-
antidepressants (TCAs), the low therapeutic conscious state, seizures and ventricular dys- cellular iron cause mitochondrial dysfunction,
index and potential for lethal toxicity remain rhythmias. Hypotension should be treated interfering with mitochondrial processes,
a concerning cause of paediatric morbidity with volume replacement and adrenaline causing lactic acidosis and cell death.
and mortality. (epinephrine) or noradrenaline (norepineph- Ingestions of less than 20 mg kg
1 ele-
TCAs are rapidly absorbed. TCAs have a rine) infusion if required. mental iron usually remain asymptomatic.
high degree of protein binding and a large Sodium bicarbonate is regarded as a Significant symptoms usually only occur in
volume of distribution. Although different specific antidote in the treatment of the ingestions above 60 mg kg
1. Potentially
TCAs have different pharmacokinetic para- cardiovascular effects of TCA toxicity. It lethal systemic toxicity may follow inges-
meters, the effects in overdose are similar. competitively overcomes sodium-channel tions of greater than 100 mg kg
1 elemen-
Dothiepin is associated with the greatest blockade and its effect on serum pH appears tal iron. Serum iron peaks at 46 hours.
lethality. Minor TCA toxicity is generally to improve sodium-channel function. Ven- Serum iron levels should be considered in
manifest by central and peripheral anticho- tricular dysrhythmias will usually respond conjunction with the clinical state.
linergic signs and the antiadrenergic effect to treatment with sodium bicarbonate Although four stages of iron poisoning
of vasodilatation. More serious toxicity (12 mEq kg
1 IV bolus, repeated till are classically described, distinct phases
results from fast sodium-channel blockade the QRS narrows or serum pH reaches may not be apparent with severe poisoning.
in the myocardium, causing a wide variety 7.457.55). Refractory ventricular arrhyth- In the initial 6 hours the gastric irritant
of atrial and ventricular dysrhythmias, mias should be treated according to stan- effects predominate, with vomiting, diar-
impaired contractility and impaired conduc- dard ACLS protocols avoiding type 1a and rhoea and haematemesis or melaena. Circu-
tion with ECG changes of prolonged QT 1c antiarrhythmics. Lidocaine is safe. There lating free iron may damage blood vessels
interval and widened QRS complexes. is no documented evidence to support and cause a transudate of fluid and hypoten-
Tricyclic ingestions of <5 mg kg
1 result the use of phenytoin, which may aggravate sion. There may be a quiescent phase when
in minimal toxicity and no treatment is hypotension and conduction problems due the patient may appear to be improving, but
required. Ingestions of 510 mg kg
1 may to its effect on fast sodium channels. about 1224 hours after ingestion the phys-
cause mild anticholinergic symptoms of Seizures may be averted or attenuated by iological processes of cells are disrupted,
drowsiness, ataxia, dilated pupils, ileus and bicarbonate therapy. Benzodiazepines are leading to metabolic acidosis, gastrointesti-
urinary retention but life-threatening toxic- the preferred agents to treat seizures but nal haemorrhage, altered mental state, pul-
ity is unlikely. Ingestions over 10 mg kg
1 barbiturates may be required to treat refrac- monary oedema, cardiovascular, hepatic and
may cause life-threatening coma, seizures tory seizures. TCAs are not amenable to renal failure. The liver is particularly vulner-
and cardiac dysrhythmias. There may be aci- removal by extracorporeal methods due to able and fulminant hepatic failure may
dosis, hypokalaemia and inappropriate anti- their large volume of distribution. Quantita- cause hypoglycaemia, coagulopathy and
diuretic hormone secretion. Onset of tive analysis of TCA levels does not aid man- death. At 46 weeks there may be stricture
symptoms is usually within 2 hours and per- agement but screening for other drugs formation in the gastrointestinal tract due
sists for less than 1224 hours. should be considered in deliberate self-harm. to scarring.

432
21.2 SPECIFIC POISONS
21

POISONING
Symptomatic patients and those with have a combination of short-acting warfarin glycolic acid, glyoxylate and oxalate are
ingestions greater than 60 mg kg
1 of ele- and long-acting superwarfarin these pose responsible for toxicity. Formation of glycolic
mental iron require laboratory investiga- a management challenge. acid and some lactic acid is the primary
tions and an abdominal X-ray. Baseline Short-acting warfarin ingestion in chil- cause of the delayed metabolic acidosis,
electrolytes, renal function and a 46 hour dren is of concern at doses greater than which can occur 412 hours after ingestion.
iron level is recommended. Iron does not 0.5 mg kg1. Prolongation of the prothrom- Oxalate is highly toxic, causing myocardial
bind well to activated charcoal. Patients bin time usually occurs at 1236 hours. depression and acute renal tubular acidosis.
with ingestions potentially in excess of Treatment with vitamin K is dependent on Calcium oxalate crystals may be noted on
60 mg kg
1 of elemental iron should have dose ingested, prothrombin time and pres- examination of the urine. Ethanol has a
IV access and a blood glucose level. IV fluid ence of bleeding. 100-fold greater affinity for alcohol dehydro-
resuscitation may be required and electro- Acute ingestion of a few pellets of super- genase than ethylene glycol, hence it is used
lytes and glucose should be monitored. warfarin is usually not a problem. Coagulo- to prevent metabolism of ethylene glycol
Whole bowel irrigation with polyethylene pathy is likely to occur in repeated or into toxic metabolites. The initial symptoms
glycol at 20 mL kg
1 hr
1 via a nasogastric chronic ingestions of rodenticides. In these of an acute ethylene glycol poisoning
tube is reserved for massive ingestions. A cases, prothrombin time should be measured include those of alcohol intoxication with
venous bicarbonate level should be per- and, if prolonged, treatment with vitamin K lethargy, slurred speech, nystagmus, ataxia
formed and serum iron concentration is indi- instituted; follow up with serial coagulation and vomiting. Papilloedema occurs less fre-
cated at 46 hours after ingestion. The tests is usually necessary. quently than with methanol poisoning. An
peripheral blood white cell count greater elevated anion osmol gap acidosis may
than 15  10 L
1and hyperglycaemia are Toxic alcohols occur. Seizures, myoclonic jerks and tetanic
suggestive of systemic toxicity. Ethylene glycol is encountered in antifreeze contractions reflect hypocalcaemia. At 12
Desferrioxamine (desferoxamine) binds compounds and radiator additives. Metha- 36 hours post-ingestion, progressive pulmo-
unbound iron in the intravascular and extra- nol is found in model aeroplane fuel and nary oedema and congestive heart failure
cellular space and the chelated complex is in home brewing concoctions. Toxic alcohols occur and may be followed by death due
eliminated in the urine, imparting a pink- are rapidly absorbed from the GI tract and to cardiovascular collapse. If the child sur-
brown colour (vin-rose urine). The decision distribute to total body water. vives, renal insufficiency may ensue over
to use chelation therapy should be based Methanol is oxidised to formaldehyde by the next 23 days.
on the combination of the patients clinical the rate-limiting enzyme alcohol dehydroge- Fomepizole and ethanol block alcohol
condition and serum iron concentration. nase, and then aldehyde dehydrogenase dehydrogenase, which is involved in the
IV desferrioxamine (15 mg kg
1 hr
1 to a converts the formaldehyde to formic acid metabolism of the parent compounds of
maximum of 80 mg kg
1 per 24 hours) is (formate). Approximately 2% of methanol methanol and ethylene glycol to their toxic
indicated in patients with hypotension, is excreted unchanged by the kidneys and metabolites. These antidotes do not prevent
shock, coma, convulsions or potentially if a small amount is excreted via the lungs. the toxic effects of the acid metabolites and
serum iron concentration is greater than The optic nerve is particularly susceptible are only useful if an osmolar gap exists.
90 mmol L
1. Desferrioxamine infusion is to the toxic effects of formic acid. Lactate Fomepizole, which is difficult to source in
usually required for 612 hours. The end- is produced from anaerobic glycolysis as a Australasia, is expensive but easier to
points of chelation therapy are clinical result of tissue hypoxia and a formate- administer and monitor than IV ethanol,
improvement and a reduction in free iron induced inhibition of mitochondrial respira- without the complications of profound hypo-
levels. Acidbase and electrolyte balance tion. Ingestion of 1.5 mL of 100% methanol glycaemia, hepatotoxicity and inebriation
should be maintained and hepatic and renal in a child weighing 10 kg would produce a that may occur with ethanol infusions.
function monitored. The chelated complex potential peak plasma level of 6 mmol L1 The target serum ethanol concentration
can be removed with haemodialysis should (0.02%, 20 mg dL
1), so a single mouthful of 20 mmol L
1 (100 mg dL
1, 0.1%) will
renal function be significantly impaired. is potentially lethal. Symptoms of methanol fully inhibit alcohol dehydrogenase. This
Asymptomatic children with ingestions of poisoning may be delayed with a 1224 can be difficult to achieve in children with-
under 60 mg kg
1 may be observed at hour latent period because of the slow out advanced support of airway and ventila-
home. Symptomatic patients, or those with metabolism to formate. The most common tion. Ethanol is preferably administered
ingestions greater than 60 mg kg
1 of ele- presentation of intoxication consists of a orally or via gastric tube. The loading dose
mental iron, require further evaluation in triad of findings related to the GI tract, eyes is 7.5 mL kg
1 of 10% ethanol in 5% glu-
hospital and an admission of 1224 hours. and metabolic acidosis. Nausea and vomit- cose water over 30 minutes, followed by a
ing, epigastric abdominal pain, pancreatitis maintenance dose of 0.81.5 mL kg
1 hr
1
Warfarin and rodenticides and GI bleeding may occur. Visual distur- of 10% ethanol. Serum ethanol and glucose
Domestic rodenticides are widely available in bance including blurred vision, central sco- levels should be monitored after the loading
almost every household. The majority of pro- toma, yellow spots and complete blindness dose and frequently thereafter. Haemodialy-
ducts available use superwarfarins, long- offer an important diagnostic clue. sis enhances elimination and is indicated for
acting anticoagulants, as their base (e.g. bro- Ethylene glycol depresses the CNS, but renal failure, visual impairment or severe met-
difacoum). A limited number of rodenticides the hepatic metabolites glycoaldehyde, abolic acidosis. Asymptomatic children should

433
21.2 SPECIFIC POISONS

have blood taken for determination of acid As management is entirely supportive, soap and water. Contaminated clothing
base status, presence of osmolar gap and assess and secure the ABCs. Benefit from should be removed and disposed into biohaz-
electrolytes. Admit all children who are clini- oral activated charcoal or gastric lavage is ard bins. The main treatment for organo-
cally intoxicated until asymptomatic. dubious and not routinely recommended. phosphate and carbamate poisoning is
A chest X-ray is indicated only if respiratory anticholinergic therapy with atropine. Char-
Essential oils symptoms are apparent. Aspiration pneu- coal decontamination or gastric lavage has
Essential oils are complex aromatic mixtures monia is treated with respiratory support if not been proven to be effective. Atropine is
of alcohols, esters, aldehydes, ketones and required. Benzodiazepines are preferred for indicated for the muscarinic symptoms of bra-
turpenes widely used in perfumery, food fla- managing seizures. dycardia and excess secretions. Bolus doses of
vourings, massage and alternative remedies. With regards to eucalyptus oil, asymptom- atropine (0.05 mg kg
1, max 12 mg)
Eucalyptus oil is an essential oil commonly atic children can be discharged after 2 hours should be repeated every 23 minutes until
implicated in hospitalisations for childhood observation. Patients with impaired con- the end-points of normal heart rate, blood
poisoning. Incidents usually involve vapori- scious state or respiratory distress on presen- pressure and drying of secretions are reached.
ser solutions, eucalyptus oil preparations tation should be admitted to an intensive- An atropine infusion may be necessary and
and other medicinal preparations, which care unit. should be discussed with a toxicologist.
are freely available over the counter. Citro- Tachycardia and dilated pupils following atro-
nella oil is used as an insect repellent. Oil Organophosphates pine therapy may indicate atropine toxicity.
of turpentine has been largely replaced by and carbamates Oxime therapy in organophosphate poison-
white spirit and turpentine substitutes, Pesticide poisoning in children is a rare event ing is controversial and unproven. Pralidox-
which are less toxic. in Australasia. Organophosphates and carba- ime, a cholinesterase reactivator at the
Essential oils are complex mixtures of sub- mates inactivate the enzyme acetylcholines- neuromuscular junction, may be effective in
stances distilled from plant species, includ- terase at cholinergic nerve terminals and re-establishing respiratory muscle and dia-
ing oil of cloves, eucalyptus, citronella, neuromuscular junctions, resulting in the cho- phragmatic function in some types of organ-
lavender, peppermint, melaleuca (tea tree) linergic toxidrome. Plasma (butyl) cholinester- ophosphate poisoning. The loading dose is
and turpentine. The oils probably differ in ase and red blood cell cholinesterase levels 2550 mg kg
1 (maximum 2 g) infused IV
the degree of toxicity but comparative data are surrogate markers for exposure and over 30 minutes, followed by an infusion at
are lacking. The irritant effects are manifest toxicity. 1020 mg kg
1 hr
1 for up to 48 hours.
by vomiting after ingestion and aspiration The cholinergic toxidrome involves excess All children with possible or potential
causing a chemical pneumonitis. secretions from muscarinic overstimulation, organophosphate or carbamate ingestion
Essential oils are potentially very toxic. neuromuscular dysfunction and paralysis should be admitted for prolonged observa-
The breath, vomitus, urine and faeces smell from nicotinic over-stimulation and central tion. Patients who remain asymptomatic in
strongly of the oil. Skin irritation may occur. effects including delirium, seizures and even- the ED after 12 hours, or overnight, may
Oil of turpentine has been reported to cause tually, coma. The onset, peak and duration of be discharged home for observation.
gastrointestinal irritation, central nervous toxicity varies with each organophosphate.
system toxicity, hepatic and renal failure In children, lethargy, coma and hypotonia
and metabolic acidosis. Eucalyptus oil toxic- are common early features of organophos- Oral hypoglycaemics
ity has been reported to involve all major phate toxicity. Excess secretions can be Sulfonylurea poisoning is uncommon in chil-
body systems and death has been reported absent in children. Severe cases can progress dren, but even ingestions of a single tablet
in an adult after ingestion of 4 mL. CNS rapidly to generalised weakness, coma, have led to significant morbidity and mortal-
depression, seizures and gastrointestinal convulsions and respiratory failure. Organo- ity, and the onset of symptoms may be
effects generally occur within 1 hour after phosphates do not off-gas, unlike nerve delayed and prolonged. Children may access
ingestion and respiratory complications agents, and do not cause secondary respira- the tablets in the home of diabetic relatives.
including respiratory depression, broncho- tory contamination of treating staff. They do, In Australasia, gliclazide, glipizide and glib-
spasm, aspiration pneumonitis and pulmo- however, warrant the use of universal pre- enclamide are responsible for the majority
nary oedema have been reported. In a cautions including gown, gloves and gog- of poisonings. Ingestion of the newer sus-
retrospective hospital-based series of 41 pae- gles. Pesticides are often dissolved in tained release preparations of gliclazide
diatric cases of eucalyptus ingestion, 80% hydrocarbon solvents and these chemicals warrant prolonged monitoring of blood glu-
remained asymptomatic, eight children had give the characteristic odour, as well as caus- cose levels.
transient symptoms prior to attendance at ing symptoms in clinicians such as head- Sulfonylureas induce hypoglycaemia by
ED (vomiting in seven patients, respiratory aches and dyspnoea. Staff should be stimulating endogenous insulin secretion.
distress in one patient) but only two children rotated regularly and the patient should be In contrast, biguanides do not cause hypo-
remained symptomatic on presentation to placed in a well ventilated resuscitation glycaemia, but can induce severe lactic aci-
ED (one with drowsiness, hypertonia and area. dosis. Children are more susceptible to
hyper-reflexia and another with drowsiness Resuscitation and decontamination should hypoglycaemia than adults because of their
and rash). Both children were discharged be carried out concurrently. Vomitus and increased metabolic rate and limited ability
the following day. secretions should be washed off the skin with for gluconeogenesis.

434
21.2 SPECIFIC POISONS
21

POISONING
Hypoglycaemic manifestations occur with haem proteins, such as mitochondrial cyto- psychoactive effects that alter perception
palpitations, shaking, hunger, sweating, chromes, to disrupt cellular metabolism. and mood.
weakness and with increasing neuroglycope- Although carboxyhaemoglobin (COHb) Complications of amphetamine and
nia, confusion, coma and seizures occur. levels poorly correlate with symptoms or ecstasy ingestion include coma, convulsions,
Long-term neurological disability and death prognosis, patients with up to 20% of hae- arrhythmias, malignant hyperthermia, rhab-
may occur. moglobin affected complain of headaches domyolysis, hypertension, and multiorgan
Following assessment and management and nausea. At 2040%, patients tire and failure. Cocaine also has sodium-channel
of the ABCs, a blood glucose level should become confused. COHb greater than 40% blocking properties which can induce ven-
be performed immediately and checked can result in ataxia, collapse, and coma. tricular tachyarrhythmias. Hyponatraemia
hourly. An initial serum insulin level may Death is preceded by cardiac arrhythmias, can be seen in ecstasy ingestion, leading
be helpful in guiding subsequent manage- cerebral oedema, and severe metabolic aci- to intractable seizures.
ment. Gastric decontamination with acti- dosis. Standard oxygen saturation monitors Patients who are asymptomatic should
vated charcoal is not routinely warranted. are unreliable in the presence of COHb, receive activated charcoal if ingestion has
Hypoglycaemia should be treated with dex- with saturations of 100% occurring in the occurred within 1 hour. Blood pressure, tem-
trose 10% 5 mL kg
1 IV bolus. Glucagon is presence of significant hypoxia. Accurate perature, and ECG monitoring should be insti-
not recommended for sulfonylurea-induced oxyhaemoglobin saturation requires mea- tuted. Symptomatic patients and those with
hypoglycaemia. surement with a co-oximeter. Conventional persistent tachycardia should be admitted
Octreotide, a long-acting synthetic blood gas analysers can also be misleading. to a monitored environment. Asymptomatic
somatostatin analogue, inhibits secretion Cyanide binds to ferric iron (Fe3) in the children may be discharged after 24 hours.
of insulin from the pancreas and may be cytochrome a-a3 complex, inhibiting its Patients with signs of cardiac or central
the most appropriate method of stabilising action and blocking the final step in oxida- nervous system toxicity require admission
blood glucose levels. Patients with persistent tive phosphorylation. Aerobic metabolism to the paediatric intensive care unit. Careful
or recurrent hypoglycaemia requiring repeat is halted and carbohydrate metabolism is monitoring of haematological and biochem-
bolus of dextrose should be given octreotide diverted to the production of lactic acid. ical parameters is essential. Hyperthermia
(1 mcg kg
1 IV bolus), followed by an The diagnosis of cyanide poisoning may respond to fluid resuscitation and sim-
octreotide infusion (250 mcg in 250 mL requires a high index of suspicion as clinical ple cooling measures; however, intractable
5% dextrose at 1 mcg kg
1 hr
1, to maxi- signs are limited and made even more diffi- cases should receive muscle paralysis and
mum of 25 mcg hr
1). If octreotide is not cult with co-existing CO poisoning. Cardinal be ventilated in an intensive-care setting.
available, a dextrose 10% infusion should features are the presence of cyanosis with Convulsions and agitation should be treated
be commenced at 12 mL kg
1 hr
1. Hourly severe high anion gap metabolic acidosis with benzodiazepines; phenytoin and neu-
blood glucose measurements are required and elevated lactate. Complications include roleptics should be avoided. Ventricular
until octreotide and/or dextrose infusions coma, seizures and myocardial ischaemia. tachyarrhythmias are managed with sodium
are ceased. Serum insulin levels may have Treatment usually cannot wait until defini- bicarbonate and benzodiazepines.
a role in ongoing management. tive diagnosis is made with cyanohaemoglo-
Asymptomatic children should be bin levels.
observed for at least 8 hours, longer for Management of CO and cyanide poison-
sustained release preparations. Symptom- ing involves high flow oxygen, supportive Further reading
Daly FFS, Fountain JS, Murray L, et al. Guidelines for the
atic children require intensive monitoring care and the potential use of cyanide anti- management of paracetamol poisoning in Australia and
of blood sugar and clinical condition. dotes. Unconscious patients require airway New Zealand explanation and elaboration. Med J Aust
2008;188:296301.
and ventilatory support and may warrant Erickson SJ, Duncan A. Clonidine poisoning - an emerging
House fires cerebral imaging in the event of trauma. problem: Epidemiology, clinical features, management and
preventative strategies. J Paediatr Child Health
From a toxicological point of view, the main Current evidence suggests that hydroxoco- 1998;134:2802.
exposures relate to carbon monoxide (CO) balamin is the most effective cyanide anti- Kerns W, Kline J, Ford MD, et al. Beta-blocker and calcium
channel blocker toxicity. Emerg Med Clin N Am 1994;12
and cyanide, derived from combustion pro- dote with the fewest side effects. (2):36590.
ducts of nitrogen-containing polymers, both Lifshitz M, Shahak E, Sofer S, et al. Carbamate and
organophosphate poisoning in young children. Pediatr
natural (wool and silk) and synthetic (poly- Psychostimulants Emerg Care 1999;15:1023.
urethane and polyacrylonitrile), which are Amphetamines and cocaine are psychomo- Nuutinen M, Uhari M, Karvali T, et al. Consequences of caustic
ingestions in children. Acta Paediatr 1994;83:12005.
used extensively in domestic furnishings. In tor stimulants that promote central and peri- Quadrani DA, Spiller HA, Widder P. Five-year retrospective
children, carbon monoxide poisoning is pheral sympathetic outflow. Ecstasy, 3,4- evaluation of sulphonylurea ingestion in children. Clin
Toxicol 1996;34:26770.
often associated with other injuries, such methylenedioxymethamfetamine (MDMA), Riordan M, Rylance G, Berry K. Poisoning in children 1-5. Arch
as burns or smoke inhalation. The affinity is an amfetamine derivative and com- Dise Child 2002;87:392410.
Tibballs J. Clinical effects and management of eucalyptus oil
of haemoglobin for carbon monoxide is mon drug of abuse. It produces typical ingestion in infants and young children. Med J Aus
210-times its affinity for oxygen. Carbon amfetamine effects, such as locomotor stim- 1995;163:17780.
Woolf AD, Wenger TL, Smith TW, Lovejoy FH Jr, et al. The use
monoxide dissolved in the plasma acts as ulation, euphoria, excitement and stereo- of digoxin-specific Fab fragments for severe digitalis
a direct cellular poison reacting with other typed behaviour. Ecstasy has additional intoxication in children. N Engl J Med 1992;26:173944.

435
SECTION

22 ENVIRONMENTAL
Section editor Gary Browne

22.1 Envenomation 436 22.4 Cold injuries 462

22.2 Drowning 453 22.5 Anaphylaxis 468
22.3 Heat-induced illness 459

22.1 Envenomation
Julian White

estimated more than 2.5 million venomous

ESSENTIALS
snakebites per year, with more than 125 000
1 Snakebite is the most important form of envenoming globally, causing significant deaths. Accurate data to confirm such esti-
morbidity and mortality. mates are unavailable, but small regional
2 Envenoming can cause rapid and severe medical problems in children, such as studies point to the broad veracity of such
statements. It is not just the number of
shock, collapse, convulsions, bleeding and respiratory failure due to either neurotoxic
fatalities that are of concern in snakebite;
paralysis or neuroexcitatory pulmonary oedema.
many survivors are left with permanent
3 Stabilisation of vital systems takes priority, followed by specific antidote therapy physical impairment, sometimes severe.
(usually antivenom) when indicated and appropriate fluid management. Paediatric cases represent 2025% of the
4 Not all patients bitten/stung by venomous animals develop major envenoming, so total, but a higher proportion of fatalities.
Snakes are ectothermic (coldblooded)
antidote (antivenom) therapy is only appropriate where significant envenoming occurs.
reptiles, comprising around 3000 species.
5 In most cases antivenom, if required, should be given IV, but always with adrenaline Venomous snakes are restricted to just four
and resuscitation facilities immediately to hand. families: Colubridae, Elapidae, Atractaspidi-
6 Assessment of degree of envenoming is critical in determining the need for dae, Viperidae (Table 22.1.1 and Figs
22.1.122.1.4). Fang structures and venom
antivenom.
types vary between families, but the common
7 Observe all patients for long enough to exclude late-developing envenoming; theme is a bite resulting in injection or inocu-
duration of observation varies dependent on the type of venomous animal. lation of venom through a break in the victims
skin. In most cases, venom is injected by fangs,
paired teeth evolved to deliver venom, usually
worldwide each year, it is not surprising through a venom groove or enclosed channel,
Introduction that deaths may exceed 100 000 per year. exiting near the tip. The act of biting can leave
Envenoming is a significant global problem, For most major causes of envenoming, anti- a variety of bite marks, which may be highly
particularly in the rural tropics, but more tem- venom remains the definitive treatment, visible or almost invisible. Venom need not
perate and urban environments are not when available. Dosage is based on extent be injected (dry bites).
immune. Children represent 25% or less of of envenoming, not patient size, so there Venom varies between snake families,
all cases, but because of their lower body is no paediatric dosage children receive within families, between genera, within
mass, are disproportionately represented the same dose as adults. genera, between species, within species,
in cases of severe envenoming. In general, between individual snakes, and even over
early diagnosis and treatment is required to time for a particular snake. It follows that
Snakebite while broad patterns of envenoming can
optimise outcomes, but diagnosis is not
always easy and specific treatments fre- Introduction be stated, there is always the possibility of
quently unavailable. With estimates as high Snakebite is the single most important an atypical pattern of effects occurring,
as several million cases of envenoming cause of envenoming. Some experts have because of venom variability. This is just

436
22.1 ENVENOMATION
22

ENVIRONMENTAL
Table 22.1.1 Families of venomous snakes and their principal characteristics

Family Fang type Common names of selected Geographic range of Family

medically important species

Colubridae (colubrids) Back-fanged (opisthoglyphs) (see Boomslang (Africa) Global

Fig. 22.1.1) Vine snakes (Africa)
OR Yamakagashi and red-necked
No fangs (aglyphs)a keelbacks (Asia)

Elapidae (elapids) Front-fanged (proteroglyphs) Cobras (Africa, Middle East, Asia) Global
(see Fig. 22.1.2) Coral snakes (Americas, Asia)
Fangs fixed or with minimal rotation Kraits (Asia)
Mambas (Africa)
Tiger snakes (Australia)
Brown snakes (Australia and
New Guinea)
Taipans (Australia and New Guinea)
Death adders (Australia, New
Guinea, and eastern Indonesia)
Mulga and black snakes (Australia
and New Guinea)
Small-eyed snake (New Guinea)
Sea snakes (Pacific and Indian
Oceans)

Atractaspididae (atractaspids) Front-fanged (proteroglyphs) Side-fanged and mole vipers Africa and Middle East
(see Fig. 22.1.3)
Fang placed to exit mouth through
side (side-fanged)

Viperidae Front-fanged (solenoglyphs) Common vipers and asps (Europe) Global, except New Guinea
Subfamily Viperinae (viperids) (see Fig. 22.1.4) Puff adders and Gaboon vipers and Australia
Subfamily Crotalinae (crotalids) Fangs on mobile maxilla, with Night adders (Africa)
considerable rotation possible Carpet or saw-scaled vipers (Africa,
Middle East, and western Asia)
Russells vipers (Asia)
Rattlesnakes (Americas)
Lance-headed vipers (Americas)
Bushmasters (Americas)
Green tree vipers (Asia)
Mamushis (Asia)

a
The absence of fangs in most colubrids does not exclude the possibility of at least local envenoming from toxic salivary secretions inoculated into the wound in the act of biting.

Fig. 22.1.3 Diagramatic representation of

the head of a side fanged atractaspid snake.
Reproduced with permission of Dr Julian White.
Fig. 22.1.2 Diagramatic representation of
the head of a proteroglyph (front fanged) snake
of the cobra type. Reproduced with permission Venom actions are diverse. Some major
of Dr Julian White. actions are listed in Table 22.1.2. From a
Fig. 22.1.1 Diagramatic representation of clinical perspective, venom effects can be
the head of an opisthoglyph (back fanged) divided into three major groups:
snake. Reproduced with permission of Dr Julian
assist prey capture by promoting
White. immobilisation; Local effects.
assist prey death, to avoid injury to the Non-specific general effects.
snake;  Specific systemic effects.
one aspect of the potential difficulties in
diagnosing and treating snakebite.
assist prey digestion; For many snakebites, in most regions of the
Venom has evolved from digestive juices.
act as a deterrent to predators, by causing world, local effects are a major, often the
rapid, unpleasant effects.
It has a variety of functions, which vary bet- principal, medical problem. In these cases
ween species, but, in broad terms, venom As humans, we tend to focus on the last there may be local pain, swelling, which
has evolved to fulfil one or more of the function, but it is the other three functions may be severe, involving much or all of
following: that cause major medical problems. the bitten limb, resulting in fluid shifts,

437
22.1 ENVENOMATION

of major local effects and secondary sys- History

temic effects can be seen following bites There may be a clear history of snakebite,
by many, but not all, species of viper and or an encounter with a snake, where an
atractaspids but not colubrids and only actual bite is uncertain, or no history of
selected African and Asian cobras amongst a snake or bite. Particularly in young chil-
the elapids. Such severe local effects are dren, there may be no possibility of obtain-
generally absent from snakebite in New ing a history. Listen carefully to the story
Guinea and Australia, where the only medi- from young children, because relevant
cally important venomous snakes are all information may be disguised by rudimen-
elapids. tary language. Some key points are listed
The non-specific general effects of enve- in Table 22.1.3.
Fig. 22.1.4 Diagramatic representation of the
noming vary between species, but usually The environment and circumstances can
head of a solenoglyph (front fanged) snake of
the viper type, with maxillary rotation to allow include one or more of the following: be of great importance in deciding if a
folding of the fang against the mouth. snakebite is likely. Do not assume that bites
Reproduced with permission of Dr Julian White. nausea or vomiting;
are unlikely indoors; snakes do enter houses,
abdominal pain or cramps;
commonly in the rural tropics, but even in
headache;
temperate urban areas such as Australian
secondary hypovolaemic shock and the risk
dizziness;
cities.
of compartment syndrome. There may be
non-paralytic blurred vision;
If there is a history of an encounter with
local blistering, bruising or development of
tender or enlarged draining lymph nodes;
a snake, note if the snake struck, how
skin necrosis. Systemic coagulopathy may
brief period of collapse;
many times, if bites were through clothing,
manifest locally as persistent oozing or
hypertension (occasionally hypotension).
as well as noting the apparent length and
bleeding from the bite or damaged areas As most of these can be the result of anxiety colouration of the snake. For selected
of the affected limb. The extent of local as well as envenoming, they may not be cobra attacks in Africa and Asia, the snake
necrosis may be significant, with potential, reliable indicators of systemic envenoming. may spit first, particularly aiming for the
often realised, for long-term tissue injury The specific systemic effects of snake eyes, before either retreating or pressing
and dysfunction. Secondary infection may venoms are the most intensely studied, home an attack with actual bites. A chew-
develop in the injured limb. For some species partly because they can usually be ascribed ing bite, where the snake hangs on is also
(e.g. lance-headed vipers, such as Bothrops to particular venom components that can important, as there is more opportunity for
spp. in South America) there may be local be isolated and studied in detail. An over- venom injection. Similarly, multiple bites
abscess formation. Long-term disability is a view of these components is listed in are associated with higher rates of major
frequent outcome. In some cases, amputa- Table 22.1.2. Specific clinical findings envenoming.
tion is required. In cases with ongoing skin for the effects of these components will be A description of the snake and geograph-
damage, never fully healed, skin tumours discussed in the sections on history, exami- ical location may help narrow the range
can develop after some years. This range nation and investigations. of possible culprit species. This can be

Table 22.1.2 Broad overview of major clinical actions of snake venoms

Type of venom action General site of action Type of venom component Clinical effects

Local toxins Bite site and bitten limb Necrotoxins, cytotoxins, etc. Local effects; may include pain,
swelling, blistering, bruising,
necrosis

Paralytic toxins Specific systemic (neuromuscular Neurotoxins (presynaptic, Progressive flaccid paralysis of
junction) postsynaptic, dendrotoxins, skeletal muscle and diaphragm
fasciculins)

Myolytic toxins Specific systemic (skeletal muscle) Myotoxins Destruction of skeletal muscle
throughout body (or locally in bitten
limb only for some crotalids)

Haematological toxins Specific systemic (interfere with Procoagulants Varies, depending on type of toxin;
haemostasis in a variety of ways, or Fibrino(geno)lytics may cause consumption
may damage vessel walls, promote Anticoagulants coagulopathy, complete
bleeding) Haemorrhagins defibrination, active haemorrhage
Various other toxins affecting promoting, or even thrombosis and
haemostasis infarction or embolism (Martinique
vipers only)

Nephrotoxic toxins Specific systemic (kidneys) Nephrotoxins Renal damage, failure, or necrosis

Cardiotoxic toxins Specific systemic (heart) Cardiotoxins Cardiac arrhythmias, failure or arrest

438
22.1 ENVENOMATION
22

ENVIRONMENTAL
Table 22.1.3 Summary of principal points in history for snakebite

Broad category Question Significance

Details of bite Was snake seen? Increases likelihood of snakebite

Description of snake? May assist identifying type of snake, so possible problems
can be anticipated
Size of snake? May indicate potential for severe bite if large specimen, but
beware, even juvenile snakes can inflict a severe bite
Geographical location? May limit types of snake to be considered
Environment? May indicate likelihood of snake encounter, if no clear history
of snake being seen
Number of bites? Multiple bites increase the likelihood of severe effects
Was bite through clothing? Clothing may soak up some venom, reduce the chance of an
effective bite. This may also be a source for venom detection
(Australia)

Details of first aid Was first aid used? If no first aid then nothing to impair development of effects of
venom
What type of first aid? Some types of first aid (e.g. tourniquets, cut and suck, suction
devices, snake stones, electric shock/stun guns) may make
matters worse or be ineffective
Effective first aid (e.g. immobilisation of the bitten limb, or full
pressure immobilisation bandage) may delay onset of
envenoming, thus the patient may present well, yet
deteriorate after removal of first aid
When was first aid applied? If applied promptly, it may be effective, delaying envenoming
If applied late or after physical activity (e.g. chasing snake,
running for help) it may be ineffective

Local effects of bite Were any bite marks, etc. noted If bite marks present, snakebite more likely, but absence of
prior to application of first aid? visible bites does not exclude snakebite (especially for some
Australian elapids, notably brown snakes)
Is there any local pain, swelling, May indicate likelihood of effective bite and possibly even
bleeding, blistering, skin discolouration type of bite
or other local effect?

General symptoms Headache, nausea, vomiting, abdominal pain? Non-specific indicators of possible systemic envenoming
(or anxiety)
Collapse? If in association with a definite bite, is suggestive of systemic
envenoming
Convulsion? If in association with a definite bite is strongly suggestive of
major systemic envenoming
Blurred or double vision experienced Common effect, not likely to indicate developing paralysis
within a few minutes of the bite?
Specific systemic effects
Paralytic effects Presence and time of onset of Cranial nerves affected first, usually ptosis. Important to pick
paralytic symptoms? this up, before paralysis advances too far. May also help
(Early ptosis may be described as indicate the most likely type of snake
heavy or sleepy eyes/eyelids)

Myolytic effects Presence and time of onset of Usually takes several hours to manifest. May indicate most
myolytic symptoms? (muscle pain, likely type of snake
tenderness, weakness; urine becoming pink, red,
brown or black)

Coagulopathic and Presence of coagulopathy effects, such as Indicates coagulopathy likely and probably significant. May
haemorrhagic effects persistent bleeding from bite site or cuts, gums, indicate most likely type of snake
or bruising, haemoptysis, haematemesis,
haematuria?

Renal effects Presence of anuria or oliguria or polyuria Indicates likely significant renal damage

General history
Medications Anticoagulants or NSAIDS? May affect coagulation test results or increase likelihood of a
major bleed if coagulopathy present
Antihypertensives? Though not proven for antivenoms, it is suspected that
b-blockers and ACE inhibitors may increase the
chance of and severity of anaphylactic reactions to
antivenom

Past history Past bites requiring antivenom? Past exposure to antivenom may increase the likelihood of
reactions to subsequent antivenom therapy
Past renal problems? May increase the likelihood of envenoming causing renal
damage
Other past medical history? Evaluate as appropriate

439
22.1 ENVENOMATION

combined with clinical features to assist in indicate developing significant envenoming instance, a small child seeking a parent
identifying the most likely culprits using (see Table 22.1.3). because they are upset, then collapsing,
diagnostic algorithms (Figs 22.1.5 and In children it may prove difficult, even having a convulsion, then recovering, but
22.1.6). impossible, to obtain any history from the remaining miserable is a classic presen-
It is important to ask about any local, child; however, parents, siblings or bystan- tation for significant snakebite in some
general or specific symptoms that might ders may have useful information. For regions (e.g. Australia).

Examination
While examination must be thorough, time
Examine the bite site
is of the essence in major envenoming.
Therefore, if snakebite is suspected, exami-
Minimal local effects, Obvious redness nation should be directed initially to deter-
no significant redness swelling, bruising mine if there is evidence for snakebite and
swelling, or bruising
the extent of any envenoming.
It is clearly important to look at the bite
Moderate to severe Minimal or no Marked swelling Only mild swelling
after 3+ hours
site, or look for a bite, if no site is indicated
local pain local pain after 3 + hours
from the history. Snakebites may result in
single or paired fang punctures, multiple
Consider Consider Consider Consider teeth punctures or even scratches, as
Death adder Brown snake Mulga snake Tiger snake fangs are dragged through the skin during
Taipan Red bellied Rough-scaled
black snake snake release (Figs 22.1.722.1.10). If there is a
Colletts snake Broad-headed bandage over the bite site, as first aid, cut
Spotted black snake
snake Stephens a window only to inspect. Keep the removed
Yellow-faced banded snake bandage portion, if in Australia, for possible
whip snake Taipan
venom detection later. If venom detection
is available (Australia, New Guinea), swab
Fig. 22.1.5 Diagnostic algorithm for Australian snakes using the bite site effects. Reproduced with
permission of Dr Julian White.

Is there a coagulopathy?

Yes No

Defibrination Anti-coagulation Is there paralysis?

Coagulation
Low fibrinogen Normal fibrinogen
Raised FDP/XDP Normal FDP/XDP
Yes No
Fig. 22.1.7 Brown snake bite. Note scratches
rather than punctures and lack of local
Is there major Is there major Is there major Is there major reaction. Reproduced with permission of
paralysis paralysis? myolysis? myolysis? Dr Julian White.
myolysis?

Yes No No Yes No Yes No

Consider Consider Consider

Tiger snake Death adder Red bellied
Rough-scaled Copperhead black snake
snake Yellow faced
Taipan Consider Consider whip snake
Brown snake Mulga snake
Broad-headed Colletts snake
snake Spotted black
Stephens snake
banded snake Small-eyed
snake
Fig. 22.1.8 Tiger snake bite. Multiple bite
Fig. 22.1.6 Diagnostic algorithm for Australian snakes using the systemic effects of the bite. with two sets of marks and local bruising.
Reproduced with permission of Dr Julian White. Reproduced with permission of Dr Julian White.

440
22.1 ENVENOMATION
22

ENVIRONMENTAL
available in Australia (most reliable sample
is bite site swab; urine can be tested if
there is systemic envenoming; blood is
unreliable). However, venom detection will
not always provide a useful answer, even if
available, so it is important to be aware
of other diagnostic tools in determining
the type of bite and clinical effects. These
Fig. 22.1.11 Early stage flaccid neurotoxic are discussed further under differential
paralysis with mild ptosis. An important early diagnosis.
sign, easily missed (tiger snake bite). Laboratory or similar investigations are
Fig. 22.1.9 Persistent bleeding from bite site, Reproduced with permission of Dr Julian White.
a sign of coagulopathy. Reproduced with often crucial to the management of snake-
permission of Dr Julian White. bite. The key areas are coagulation, renal
function and muscle integrity.
Many snakes, especially vipers but also
some colubrids and many Australian elapids,
can cause coagulopathy, which in many cases
is potentially lethal. Coagulopathy can
develop early or gradually over many hours.
The type of coagulopathy is determined by
the type of venom components, but just a
few tests are adequate in most situations
to determine the extent of pathology. For
rural areas or hospitals without laboratory
facilities, including outback Australia, the
Fig. 22.1.10 Extensive bruising of bitten limb.
Typical of viper bites causing coagulopathy whole blood clotting test (WBCT) is the only
(green pit viper bite). Reproduced with practical test. 510 mL of venous blood is
permission of Dr Julian White. placed in a glass test tube or similar and
allowed to clot. If possible, the time to clot is
measured. Normal blood should clot in under
the bite site with the stick provided in Fig. 22.1.12 Flat facial appearance. Caused by 10 minutes. If there is minimal or no clot at
the test kit. Do not allow anyone to clean progressive involvement of cranial nerves in 20 minutes, this strongly suggests a coagulo-
the bite area until it has been swabbed flaccid neurotoxic paralysis. Ptosis is also pathy. If possible, perform a parallel test on
for venom. Look for bite marks and parti- present (tiger snake bite). Reproduced with blood from a normal control (e.g. relative or
permission of Dr Julian White.
cularly for multiple bites. Observe for local staff member). If laboratory facilities are avail-
bruising, bleeding, blistering, swelling or able, the key tests are prothrombin time (PT)
necrosis. If there is significant local tissue or international normalised ratio (INR), acti-
injury or swelling, check pulses, etc., to vated partial thromboplastin time (aPTT),
exclude compartment syndrome in affected fibrinogen titre, fibrin (ogen) degradation
compartments. Compartment syndrome, products (or d-dimer) titre, and platelet count.
if suspected clinically, must be confirmed Renal function tests are usually urea and
by measuring intracompartmental pres- creatinine levels. In the absence of a labo-
sure, before any consideration of surgical ratory, monitoring renal output is all that
intervention. is practical.
Check draining lymph nodes; if they are Muscle integrity relates to myolytic-
tender or swollen it may indicate venom venoms, the best measure being creatine
absorption and movement. phosphokinase level (CK, CPK). In the
Fig. 22.1.13 Persistent blood ooze from IV site
Examine for specific effects, notably neuro- indicative of coagulopathy (taipan bite). absence of a laboratory, the presence of
toxicity (flaccid paralysis; check for cranial Reproduced with permission of Dr Julian White. red, brown or black urine is suggestive of
nerve paralysis first, starting with ptosis; myolysis and myoglobinuria. However, red
Figs 22.1.11, 22.1.12), myolysis (muscle (arrhythmias),allergy (angioneurotic oedema; urine can also be caused by haematuria. If
tenderness and weakness), coagulopathy particularly European vipers). in doubt, spin down the urine and examine
(persistent bleeding from bite site, needle under a microscope, looking for evidence
punctures, etc.; Fig. 22.1.13) or deep vein Investigations of red cell casts. Both haemoglobin and myo-
thrombosis (DVT, pulmonary embolism; The most specific investigation is venom globin test positive for blood with dipstix
Martinique crotalids only), cardiotoxicity detection, but currently this is only routinely testing of urine.

441
22.1 ENVENOMATION

If there is evidence of infection around the Patient presents with a history of snakebite BUT
bitten area, culture and sensitivity should be no dead snake and little/no description of the snake
performed on wound swabs.
In cases where there is clinical evidence of Yes No
Neurotoxic Marked local Neurotoxic
cardiovascular effects of envenoming, pri- signs swelling signs
mary or secondary, or for bites by snakes No Yes Yes No
known to be cardiotoxic, ECG monitoring is TREATMENT*

appropriate, but in other cases it may be Non-clotting blood In Sri Lanka OR Bitten Observe in
(20WBCT) OR South India on land hospital for
unnecessary. spontaneous 24 hours
systemic bleeding No
Chest X-ray (CXR) is only required if there Yes Yes No

are clinical grounds to suspect respiratory

pathology. Similarly, arterial blood gas No Yes Non-clotting blood Cobra or In the Myalgia AND/OR
(20WBCT) OR king cobra Philippines black urine or
examination is not routinely required, but spontaneous bite renal failure
Early blistering systemic bleeding
could be considered if there is respiratory or necrosis or black urine Yes No
impairment, particularly if there is respira- No Yes TREATMENT*
tory paralysis developing. In the later stages, TREATMENT* Yes No Sea snake
after extensive intravenous (IV) fluid ther- bite: give
Observe in Viper bite Local necrosis appropriate
apy, secondary pulmonary oedema is a risk, hospital for evident antivenom
24 hours
especially in young children; if suspected, a Renal failure Yes No
TREATMENT*
CXR may be diagnostic. TREATMENT* TREATMENT*
Bite by very Philippine cobra
Envenoming does not always manifest Cytotoxic cobra Russells large snake bite: give anti- Krate bite:
bite: give viper and King cholinesterase give
early. It is therefore appropriate to retest appropriate present in cobra occurs in and appropriate appropriate
for coagulopathy, renal impairment and antivenom this area the area antivenom antivenom
elevated CK, if the initial tests are normal. No Yes No Yes
TREATMENT* TREATMENT* TREATMENT*
In general, a useful protocol is to retest 2
Patient describes Russells viper bite: Cobra bite: give King cobra bite:
3 hours and 56 hours after the initial green snake OR give appropriate anticholinesterase and give appropriate
test, or earlier if symptoms or signs of tree snake antivenom appropriate antivenom antivenom
envenoming develop and at 12 hours Yes No
TREATMENT*
or later post bite, prior to any decision to Malayan pit Probable Malayan pit Blood still non-clotting after
Yes
discharge. viper present viper bite: give four 6 hourly doses of
in this area appropriate antivenom Malayan pit viper antivenom

Differential diagnosis TREATMENT*

Yes No

A full discussion of all possible differen- Russells No Try green pit viper TREATMENT*
tial diagnoses for snakebite is beyond the viper present antivenom
in this area Observe in
scope of this chapter. It is important to TREATMENT*
hospital
TREATMENT*
include snakebite in the differential diagno- Green pit viper bite: give Yes Try Russells viper
sis for patients with unexplained collapse, appropriate antivenom or antivenom * Observe patient closely for emerging signs
conservative treatment of envenoming and take appropriate action
convulsions, bleeding, coagulopathy, throm-
bosis (in Martinique, specifically), myolysis, Fig. 22.1.14 Diagnostic algorithm for snakebite in southeast Asia. After Warrell et al. Reproduced
flaccid paralysis, muscle fasciculation with permission of Dr Julian White.
(mamba bites in Africa), renal failure or
impairment, or local tissue injury.
Differential diagnosis can also be applied can be targeted appropriately. A similar specific (antivenom) and non-specific
within snakebite, in determining the type of situation applies in some other regions, treatment.
snake most likely to have caused the bite. where specific antivenoms are available. First aid for snakebite is controversial.
Diagnostic algorithms have been developed In regions such as North America, this is less Many techniques have been advocated and
for Australia (see Figs 22.1.5 and 22.1.6) important, because there is only one poly- are in use throughout the world. Almost
and South-East Asia (Figure 22.1.14). These valent antivenom covering all venomous none meet the critical criteria of safety
are based on cases with significant enve- species, except coral snakes. and effectiveness. For snakes not likely to
noming and will not function if the patient cause major tissue injury in the bitten
is not envenomed, though this hardly area, the Australian-developed pressure
matters, as such a patient will not require Treatment immobilisation method is appropriate. A
antivenom therapy. In some regions, notably Snakebite treatment can be divided into broad bandage is applied over the bite
Australia, it is important to know the type of several areas: first aid; diagnosis; and site, then the rest of the bitten limb, at
snake involved, because antivenom therapy treatment, the latter further divided into the same pressure as used for a sprain,

442
22.1 ENVENOMATION
22

ENVIRONMENTAL
that is firm but not occlusive. The limb is the only available snake antivenom is poly- fatality of more than 100 000 per year,
then immobilised using a splint. Correctly valent, covering all endemic pit-viper spe- death is clearly a significant risk. This is
and promptly applied, this method is both cies, so identifying the snake is less especially true for children. Their lower body
safe and effective. However, for snakes important. In general, antivenom will be mass and often delayed application of
likely to cause local tissue injury, even more effective than any other therapeutic appropriate first aid puts them at greater
the pressure of this technique may agent at reversing envenoming. Used appro- risk. With such a wide variety of snake spe-
cause further tissue damage, at least theo- priately it is life saving and the old wisdom cies, it is beyond the scope of this chapter
retically. For this reason, the pressure that the antivenom is more dangerous to define prognosis for all snakes. However,
immobilisation method has not been than the venom is outdated, inappropriate some general principles apply.
recommended for all snakebites. The theo- and dangerous. The more rapid and severe the onset of
retical danger from this method has been Fourth, antivenom is generally disappoin- envenoming, the more grave the prognosis,
challenged by recent research and it may ting as therapy for local effects of envenom- but this is not absolute. For instance, in
be that extension of this research will show ing, but is still better than other therapies in Australia, a small child (under 5 years)
that the pressure immobilisation method is most cases. Equally, do not overlook adjunc- may show early irritability, collapse, even
safe and effective for all snakebites. tive therapies, in particular adequate IV convulsions following a snakebite (espe-
Other popular first aid methods enjoy no hydration if there is massive local or limb cially bites by brown snakes, tiger snakes,
such success and are either unsafe or ineffec- swelling following the bite, as untreated taipans), yet will usually spontaneously
tive, or both, and should never be used. hypovolaemic shock secondary to such recover consciousness. Such a presentation
Amongst these are tourniquets, cut & suck, fluid shifts is potentially lethal, especially is indicative of major envenoming, but with
patent venom extraction devices (suction), in children. correct treatment, is survivable. Thus prog-
electric shock (stun guns etc), application Fifth, for coagulopathy caused by venom, nosis is determined by several factors, not
of chemicals to the bite, snake stones and antivenom is the best treatment to reverse just the type and toxicity of the snake, but
witch doctor treatments. The application effects, and factor replacement therapy, also the treatment response. It is likely that
of certain plant extracts is still undergoing including even whole blood, is best if high standard treatment were universally
evaluation. reserved for those cases with catastrophic available, the global toll from snakebite
Diagnosis of snakebite has been discussed bleeding, or no available antivenom, or would be far lower.
earlier. where sufficient antivenom has already For local effects of envenoming, after
Definitive treatment for snakebite will been given to neutralise all venom. Giving bites by snakes causing local tissue injury
vary depending on the type of snake, but factor replacement therapy while active (most vipers, many African and Asian
some general principles apply. venom is still circulating is to invite worsen- cobras), the more rapid the swelling, the
First, not every bite will result in enve- ing of the coagulopathy. Heparin is gener- more extensive blistering, the more serious
noming, but the extent of envenoming, if ally ineffective in these cases and should the bite. Similarly, development of disco-
any, may not be immediately apparent, be avoided. louration of the skin with a well-demarcated
therefore all bites should be treated with Finally, for cases with flaccid paralysis, edge often indicates an area of impending
caution. consider anticholinesterase therapy as an necrosis.
Second, in many regions, the bulk of adjunct to antivenom, if a Tensilon test has For general systemic effects, the more
snake fauna is non-venomous, so many shown benefit (there is likely benefit for cobra severe the symptoms, such as vomiting, abdo-
snake bites may be trivial. However, it is nec- bite causing paralysis, also death adders (but minal pain, headache, often, though not
essary to be sure of the snakes identity as only some cases), possibly some kraits, sea universally, the more severe the envenoming.
non-venomous, before dismissing the case snakes, possibly some coral snakes). Venoms For specific systemic effects, rapid onset
and identification is rarely easy, especially with presynaptic neurotoxins will not show often indicates severity. Early development
in paediatric cases where history is scant. response to anticholinesterase therapy (most of progressive flaccid paralysis is usually
An exception is Australia, where almost all Australian snakes causing paralysis; excep- indicative of severe envenoming, while
snakes causing bites are potentially lethal. tion is the death adder, which sometimes paralysis of very limited extent after 6
Third, if significant envenoming has has only postsynaptic neurotoxins). hours is often indicative of a less severe
occurred, with few exceptions, antivenom, From the above it will be clear that anti- bite. However, this may not always be the
if available, is the treatment of choice and venom is the key treatment for snakebite, case. Occasionally flaccid paralysis may
should always be given IV. Choice of anti- when available. The latter is a real issue, not be evident; even a patient with just pto-
venom will be determined by the type of because for many species and substantial sis, until 24 hours post bite, yet may prog-
snake and the availability of methods to areas of the rural tropics, antivenom is not ress to severe paralysis without treatment.
determine snake identity. Thus, in Australia, available. This may occur with many snakes causing
specific antivenoms are available, together paralysis, but notably Australian death
with venom detection and diagnostic algo- Prognosis adders. This is an important reason why
rithms, so polyvalent antivenom is often The majority of all snakebites globally prove early discharge of an apparently well
not required. In contrast, in North America non-fatal, but with an estimated global patient is ill advised. Once flaccid paralysis

443
22.1 ENVENOMATION

is extensive, with respiratory failure, the renal cortical necrosis has occurred. This
medical expert support (see earlier
patient can still survive if ventilation is sup- severe complication with a poor prognosis
discussion on first aid).
ported. However, for snakes with presynap- is not easily predicted and is generally
tic neurotoxins, reversal of paralysis will not only discovered at renal biopsy in patients Type of antivenom The role of
occur until the damaged terminal axons at who have failed to recover from early renal antivenom in treatment should no longer
neuromuscular junctions have recovered, failure. It has been reported after bites by be considered controversial, as where
which may take days to weeks. Antivenom only a few species, such as the Australian available, if of reasonable quality, it is the
will not reverse such paralysis. In contrast, taipan and South American lance-headed treatment of choice. Less certain is the role
paralysis caused by snakes with purely post- pit vipers (jararacusu, jararaca), but other of antivenom in treating purely local
synaptic neurotoxins will usually reverse species could potentially cause this effects of envenoming, especially in
with adequate antivenom therapy. The outcome. preventing necrosis. Recent clinical
Tensilon test will generally predict such experience suggests that high-quality
responses. antivenoms, particularly Fab0 antivenoms,
Prevention
For coagulopathy, rapidity of onset and are at least able to lessen the extent of
Prevention of snakebite can be considered
presence of signs such as persistent oozing local tissue injury, if used promptly. No
in two ways. First, there is prevention of
from the bite site and bleeding gums indi- antivenom can be expected to reverse
bites, by educating the population about
cate significant coagulopathy. With appro- established local necrosis. However,
ways to avoid contact. These will vary from
priate treatment, this does not imply a debate continues on the relative merits of
region to region and are beyond the scope
poor prognosis, but is a warning that more different types of antivenom. The most
of this chapter. Second, there is prevention
critical haemorrhaging is possible. Any recent Fab antivenoms have proven
of the more severe effects or complications
bleeding into a vital organ, most commonly clinically effective, but are rapidly cleared,
of envenoming, by prompt diagnosis and
the brain, indicates a poor prognosis, with a requiring higher and repeat doses. F(ab0 )2
appropriate treatment. This commences
fatal outcome most likely with intracranial antivenoms are less rapidly cleared and
with early application of appropriate first
bleeds. For Russells viper in Myanmar are effective. Whole IgG antivenoms have
aid pre-hospital, to minimise the chance
(Burma) and southern India, there may be the highest rate of adverse reactions, but
of severe envenoming developing before
haemorrhagic infarction of the anterior pitu- are cheaper and may be the most potent.
treatment can be instituted. Once in hospi-
itary gland, with resulting Sheehans syn- New methods of producing whole IgG
tal, urgent triage and assessment will permit
drome developing, though this may not be antivenoms are proving cost effective and
prompt IV rehydration, for cases where
rapidly apparent. appear to significantly reduce the adverse
there is a major fluid shift into the bitten
Myolysis is most often measured as effect profile, so there is a likely resurgence
limb, or rapid commencement of IV anti-
profoundly elevated CK levels, and may of safe, effective IgG antivenoms. The
venom, if indicated, before more delayed
progress to secondary renal failure and choice of animal is also contested. Horses,
forms of envenoming (e.g. flaccid paralysis,
hyperkalaemia, the latter indicating a poor while traditional and easy to use in most
myolysis) have progressed too far, as well
prognosis, as cardiac complications may regions, produce an antivenom with
as instituting any life-support measures
ensue. In general, the more rapid the rise higher rates of adverse reactions than
required.
in CK levels, the more severe is the myolysis, sheep, but sheep can only be used safely if
Many deaths or cases with long-term
but this is not always the case. In some cases raised in regions free from prion diseases,
morbidity after snakebite are the result of
the CK rise may initially be slight, but essentially limiting them to Australia and
either delays in commencing treatment, or
become more significant after 24 hours, New Zealand. IgY antivenoms from egg
inadequate or inappropriate treatment.
rising to high levels over several days. Early yolk, produced by immunising chickens,
The latter may be the result of poor training
muscle pain and myoglobinuria is also are potentially easy and cheap to produce,
of health personnel. It follows, therefore,
suggestive of more severe myolysis. but their safety and effectiveness is not yet
that adequate training of staff will be
Renal damage may be primary or sec- established and recent research indicates
preventative.
ondary and can prove lethal if untreated. both limits on effectiveness and a likely
Rapid development of anuric renal failure unacceptable adverse effects profile.
indicates a poor prognosis, unless dialysis
can be instituted. A slower rise in creati-
Controversies  Premedication There remains
nine levels, without anuria, usually indi- First aid Possibly the most debate on the value of premedication
cates less severe renal damage, but controversial aspect of snakebite prior to antivenom therapy to reduce the
still may take a week or more for return management is the recommended type of likelihood of acute adverse reactions,
of normal renal function. In most cases, first aid. Many types of first aid have been especially anaphylaxis. Antihistamines
even acute renal failure after snakebite advocated and remain in widespread use, have been shown to be ineffective at
is reversible, over days to weeks. A small but only immobilisation, or pressure preventing such reactions. They have
minority of cases will develop more lasting bandaging and immobilisation have adverse side effects (drowsiness and
renal failure, generally because bilateral consistently enjoyed both scientific and occasionally hyperexcitability) and

444
22.1 ENVENOMATION
22

ENVIRONMENTAL
quinquestriatus, Androctonus spp., Buthus
should not be used. Hydrocortisone epidemiological studies that fail to
spp.), Western Asia and India (Buthus
carries no proven benefit, but no great relate bite effects to particular species
spp., Mesobuthus spp.) and in Iran, the
risk. Adrenaline (epinephrine) remains of snakes, rendering these studies
unique Hemiscorpius lepturus.
the most controversial, as it can reduce almost useless. It is essential that
In general, it is not the larger scorpions
the incidence of reactions for poor- accurate profiles of the clinical spectrum
with robust front pincers that are most con-
quality antivenoms, but has a significant of envenoming be documented for every
cerning, but the smaller, more delicate species
risk profile, so for most antivenoms its use species biting humans, preferably in
with unimpressive front pincers, because
is not recommended. It is particularly controlled prospective studies.
they rely on the toxicity of their venom.
dangerous for bites likely to cause
coagulopathy (e.g. many Australian
Controlled studies to establish Scorpion venoms contain a wide array of
antivenom effectiveness and dosage are ion-channel toxins of great potency, causing
snakebites, most viper bites). The
required. Use of modern techniques to an excitatory neurotoxic reaction (not paral-
practice of pre-testing for allergy to
measure venom and component levels ysis), not dissimilar to an autonomic storm.
antivenom, using a small subcutaneous
in serial patient blood samples should Only a matter of minutes, not hours, may
dose of the antivenom is to be
greatly assist in such research. elapse from the time of the sting to major
discouraged. It has been shown to have
Antivenoms need to become safer, more systemic envenoming. Once the systemic
no reliable predictive value, but carries a
effective and much more widely toxicity is established, antivenom therapy
significant risk, without benefit, and will
available, particularly in the rural has less chance of success, though it may
delay commencement of antivenom
tropics. still save lives. In Mexico, with >280 000
therapy.
cases per year, death rates in children
Coagulopathy The treatment of following scorpion sting have fallen from
coagulopathy remains contentious, thousands per year to a handful following
Scorpion stings the introduction of antivenom.
though most experts agree antivenom is
the best therapeutic choice, if available. Introduction Scorpion venoms do not contain paralytic
Factor replacement therapy is often the Scorpion stings are the second most impor- neurotoxins, myolysins, components affect-
only option if no antivenom is available, tant form of terrestrial envenoming, after ing coagulation or renal function, nor do
as for some colubrid snakes, but is not snakebite, with global cases probably excee- they contain local necrotic toxins (except for
without hazard. Heparin has been ding one million per year, and deaths num- one species in the Middle East; Hemiscorpius
advocated, but most evidence suggests bered in the many hundreds, to possibly as lepturus in Iran).
that it is both ineffective and dangerous high as 5000 per year, nearly all in children.
in this setting. Scorpion envenoming is unpleasant for
adults and occasionally is severe enough History
Local necrosis The treatment of to threaten life. In children, however, it can Often a scorpion will have been seen. There
local swelling should be standard, with be a rapidly severe and lethal disease, will usually be a clear history of an immedi-
fasciotomy reserved for those few cases with some centres still reporting paediatric ately painful sting (except Hemiscorpius
with proven compartment syndrome fatality rates in excess of 10%. lepturus), followed by development of
(intracompartmental pressure Scorpions vary in size, with over 1600 systemic envenoming with effects that
measurement). Fasciotomy should be species known. All have a sting in the tail may include some of the following:
avoided, if possible, in cases with active (telson) with associated venom glands.
coagulopathy. However, early Most scorpions either rarely sting humans,
tingling of the lips;
fasciotomy is still practised in some or are too small to cause envenoming, or
nausea, vomiting;
areas, often with distressing and have venom of little potency in humans.
abdominal pain;
unacceptable functional and cosmetic Unfortunately, a small number of scorpions
collapse;
sequelae. do possess potent venoms and these spe-
convulsions;
cies predominate in parts of the world
hypertension or labile blood pressure;
where humans exist in large numbers, often
increased sweating, salivation or
lacrimation;
in less than affluent conditions. The combi-
nation of warm to hot evenings, sandy soils,
piloerection;
Future directions dyspnoea;
a tendency to walk around barefoot and
Even for many common species of dwellings that do not exclude scorpions
pulmonary oedema;

venomous snakes, known to cause leads to the large number of stings. Major
cardiac collapse;

significant numbers of bites, reliable risk areas include South and Central Amer-
multiple organ failure.
clinical studies of envenoming are scant ica, particularly Brazil (Tityus spp.), Mexico Symptoms appropriate to each of these
or lacking. The medical literature on and adjacent USA (Centruroides spp.), effects may be described. It is important to
snakebite is replete with North Africa and the Middle East (Leiurus note the time of onset for symptoms a

445
22.1 ENVENOMATION

rapid onset and escalation in severity indi- Treatment

Indian doctor (where no antivenom is
cates a severe sting. Treatment of major scorpion envenoming is
available). This technique is not favoured
controversial, particularly centring around
by most experts, considering it both
the role of antivenom. Most evidence sug-
highly risky and of most uncertain
Examination gests that antivenom use has resulted in
theoretical and practical benefit.
The local effects of the sting are not gene- greatly reduced fatality rates in children,
rally impressive, though there may be local but a few doctors argue that pharmacother-  Despite the frequency of scorpion
sweating and piloerection. It is the systemic apy is more effective than antivenom, partic- stings, there are still few published
effects that will be most important, so ularly focusing on the cardiac failure seen in studies of series of stings by particular
particularly check blood pressure, look fatal cases. Prazosin, in particular, has species and few trials of various methods
for signs of neuroexcitation, pulmonary enjoyed success and should be considered, of treatment. There is a need for more
oedema and cardiac collapse. In small chil- both as an adjunct to antivenom and as intensive systematic study of scorpion
dren there may be a nystagmus. The excep- first-line therapy in the absence of anti- envenoming and controlled trials of
tion is Hemiscorpius lepturus in part of Iran. venom (i.e. in India). If antivenom is avail- treatment alternatives.
This species causes severe local effects, plus able it should be used IV without delay.
systemic effects including multiorgan failure Dose will vary depending on product.
and shock. Stings by this scorpion particularly Prevention
affect children with a significant fatality rate. Most scorpion stings occur because of the
Prognosis patterns of human living, and thus are the-
The prognosis in scorpion envenoming
oretically avoidable. It is possible to scor-
depends on several factors. More severe
Investigations pion-proof houses, by use of tiles around
envenoming is likely in smaller children, with
There are no specific tests for scorpion the lower walls, preventing scorpion entry.
more rapid development of effects and a
venom, nor specific indicators of envenom- Use of enclosed footwear and avoidance of
shorter window for effective antivenom ther-
ing, but in severe cases it is important to sitting down or lying down outside after
apy. If multiple organ failure develops then
exclude secondary effects of envenoming dusk can also reduce sting incidence.
prognosis is generally poor.
and multiple organ failure.

Controversies and future

Differential diagnosis directions Spiderbite
Full differential diagnosis of scorpion sting is Introduction
beyond the scope of this chapter. The auto-
The major controversy in
Spiderbite is probably very common, but
management of scorpion sting is the
nomic storm clinical picture seen in severe most bites are trivial, with only a few species
issue of antivenom effectiveness, as
scorpion envenoming can also be caused likely to cause major harm to humans
discussed earlier. Of the various types of
by some other venomous animals, particu- (Table 22.1.4). For these species, morbidity
antivenom, animal studies have
larly funnel web spiders (in Australia, where can be significant, but mortality is low, with
indicated that Fab antivenoms are not
major scorpion stings do not occur), banana global deaths directly related to spiderbite
more effective than F(ab)2 or IgG
spiders (in Brazil; typically also cause pria- probably measuring 20 or less per year. Even
antivenoms.
pism in boys), and some jellyfish (irukandji the worlds most dangerous spiders, the
type). Accidental or deliberate exposure to Among non-antivenom therapies, Australian funnel web spiders, have only
certain pesticides and pharmaceuticals the most controversy has surrounded the caused one known fatality in the last
should also be considered. proposal to use insulin, suggested by an 20 years. As with other venomous animals,

Table 22.1.4 Medically important spider groups

Family Genera Common name Distribution Clinical effects

Hexathelidae Atrax Australian funnel web spiders Eastern Australia, from Cape Severe neuroexcitatory (autonomic storm)
Hadronyche York to Tasmania envenoming; about 10% of cases develop major
envenoming, which untreated, carries a significant
risk of fatality

Theriidae Latrodectus Red back or widow spiders Global Moderate, generally non-lethal neuroexcitatory
Steatoda envenoming

Ctenidae Phoneutria Banana spiders Central and South America, Moderate to severe neuroexcitatory envenoming,
especially Brazil rarely lethal

Loxoscelidae Loxosceles Recluse or fiddleback spiders Global, particularly the Severe local tissue injury, with occasional major,
Americas potentially lethal systemic effects

446
22.1 ENVENOMATION
22

ENVIRONMENTAL
spiderbite is more likely to be severe in
small children.
Approximate range of funnel
web spiders, genera Atrax
History and Hadronyche
Spiderbite is not always initially painful, Approximate location of
and spiders are small and easily misidenti- confirmed cases of
significant systemic
fied, so most commonly there will be no cer- envenoming by funnel web
tainty from the history about the species spiders from literature
involved. However, particular spiders cause Fatal cases (each cross
represents location, not
quite specific envenoming syndromes, number of cases)
making diagnosis possible even without a
1 possible death
spider being available. The common pre- (Sutherland)
sentations for the medically important spi-
ders are listed below. In general, however, it 13 deaths in Sydney area
is important to note the circumstances
of definite or possible exposure to spider-
bite, a description of the spider, if seen,
and the timing of onset for any symptoms
that develop.

Australian funnel web spiders

These large mygalomorph spiders are
robust in appearance (Fig. 22.1.15), gener-
Atrax robustus Hadronyche formidabilis
ally ground dwelling (there are tree-dwelling Atrax sp. 1 Hadronyche cereberea
species) and are found only in eastern Atrax sp. 2
Australia (Fig. 22.1.16). Their large fangs
and acidic venom generally cause immedi-
ate local pain on biting and they may hang
on, being difficult to dislodge. Apart from
pain, other local effects are not prominent.
In about 10% of cases, systemic envenoming
will develop, often rapidly, and can be lethal
in less than 60 minutes in children. First
symptoms are tingling of the lips and twitch- Hadronyche infensa Hadronyche sp. 7
ing of the tongue, followed by non-specific Hadronyche versuta
Hadronyche sp. 4
symptoms, which may include headache,
nausea, vomiting and abdominal pain.
There is frequently evidence of neuroexcita- Fig. 22.1.16 Distribution of Australian funnel web spiders (Atrax spp. and Hadronyche spp.).
tion, with sweating, salivation, lacrimation Reproduced with permission of Dr Julian White.
and piloerection. Hypertension is usual
and dyspnoea secondary to pulmonary
oedema can develop rapidly. Without
treatment this can progress to hypoxia,
coma and death.

Widow spiders including Australian

redback spiders
Widow spiders have a classic appearance
(Fig. 22.1.17) with a globular abdomen,
comparatively small cephalothorax and long
thin legs. They utilise a drop-line web struc-
ture to capture prey. Humans most often
receive bites when they either come in con-
Fig. 22.1.15 Male Sydney funnel web spider
tact with the web or occasionally when Fig. 22.1.17 Female widow (redback)
(Atrax robustus). Reproduced with permission touching a wandering spider (i.e. caught in spider. Reproduced with permission of
of Dr Julian White. footwear, clothing or similar). Widow spider Dr Julian White.

447
22.1 ENVENOMATION

venom, though neuroexcitatory, is rarely unexplained acute abdomen or chest pain,

lethal in humans. A typical significant bite before laparotomy is scheduled.
causes local pain, becoming more severe,
sometimes with local sweating, then pro-
gressive proximal movement of pain and Treatment
sweating, ultimately becoming severe Treatment for Australian funnel web spider-
regional or generalised pain, sweating, plus bite is principally the use of specific IV anti-
hypertension, nausea and malaise. Enve- venom, available in Australia only. Initially
noming can mimic acute abdomen and car- 24 vials are needed, but severe cases
diac chest pain. Untreated, pain may take require more. All cases with any evidence
days or weeks to resolve and often gravi- of venom spread require antivenom
tates to the lower limbs, causing burning Fig. 22.1.18 Recluse spider, with violin-shaped urgently, before life-threatening envenom-
pain in the feet and legs, often with asso- marking on the cephalothorax. Reproduced with ing develops, which may occur rapidly in
ciated sweating. Rarely, severe systemic permission of Dr Julian White. children.
envenoming can cause pulmonary oedema Widow spiderbite only requires treat-
or a secondary rise in creatine kinase. In as necrosis develops over the following ment in those cases with significant local,
infants, a generalised rash is common, the 47 days. The area finally involved can be regional or systemic envenoming, which is
infant presenting as highly distressed and local to extensive, with skip lesions, and is a minority of cases. Antivenom is the most
irritable, not consolable, erythematous, but usually painful. There is commonly an effective treatment and may be given
with no obvious cause apparent. A widow associated non-specific, self-limited, sys- intramuscularly (IM, in Australia), but is
spider may be found in the bedding or temic illness. In a few cases, a more severe more rapidly effective if used IV (even in
underneath the bed. Less than 20% of bites systemic illness can occur, potentially lethal, Australia). Experience has shown that only
cause major envenoming. with haemolysis, shock, coagulopathy, antivenom reliably reverses envenoming
or renal failure. This is viscerocutaneous and is more effective than narcotic analge-
loxoscelism. sics in treating pain. It can be effective for
Banana spiders
up to days post-bite. It is usually given as
Banana spiders are large aggressive
Examination single vials (2 vials in Australia), waiting
spiders, well known within their range. They
Initial examination may show little locally 2 hours before giving a further dose,
are active hunters and invade houses, with
at the bite site, depending on the type of if symptoms warrant. Some recent research
bites occurring year round, but especially
envenoming involved. For those spiders in Australia has questioned the effective-
in autumn. They are a common cause of
causing predominantly regional or systemic ness of antivenom, but this is in contrast
bites in Brazil, accounting for 20% of all
effects, these will dominate examination to decades of positive experience using
presentations in some hospitals. The neu-
findings. this antivenom.
roexcitatory venom has effects similar to
Banana spiderbite is most often managed
widow spider bites in many ways, with
without use of antivenom, the latter
pain being a predominant feature, as is Investigations reserved for severe cases, where it should
sweating, hypertension and nausea. How- There are no clinical diagnostic tests be given IV, most of these cases being
ever, unlike widow spiders, there is often specific for spiderbite. Most bites will result children under 7 years of age. In the
local swelling of the bite area and priapism in leucocytosis. For suspected loxoscelism, less severe cases, local anaesthetic block is
is a classic feature of envenoming in boys. it is important to look for haemolysis, usually adequate.
Death is a rare outcome, but severe cases disseminated intravascular coagulation Loxoscelism is difficult to treat. Specific
can develop pulmonary oedema or cardiac and renal failure, especially in children. antivenom is only available in Brazil and
arrhythmias.
its place in management is controversial,
Differential diagnosis though it is widely used in Brazil and
Recluse or fiddleback spiders Detailed differential diagnosis is beyond considered effective. In general, patients
These small, delicate spiders are generally the scope of this chapter, but the geogra- present late, after tissue injury has com-
brown with long spindly legs and a darker phical location and pattern of local and menced, requiring good wound care.
brown pattern on the cephalothorax in the systemic effects makes differentiation Secondary infection requires appropriate
rough shape of a violin (Fig. 22.1.18). They between these types of spiderbite straight- antibiotic therapy. Early surgical debride-
have a venom that is predominantly dermo- forward. For severe neuroexcitatory enve- ment can extend the area of injury and
necrotic. The most common effect of bites noming, as seen with Australian funnel should be avoided. Steroids have not been
is cutaneous loxoscelism. The bite is rarely web spiders, consider pesticide or pharma- shown to be effective. Dapsone, given
noticed and often occurs in bed at night. ceutical poisoning in the differential. Widow early, can reduce injury, but is toxic and
However, hours later the area becomes spiderbite should be considered in the not widely favoured as therapy. Hyperbaric
red, painful, may blister or bruise and darken differential for selected cases of apparent oxygen therapy is controversial. It may

448
22.1 ENVENOMATION
22

ENVIRONMENTAL
benefit some patients, but is unsuitable for flaccid paralysis, especially in children. In The more rapid the onset of paralytic
most children. Australia, for instance, paralysis ticks features, the more likely is major paralysis.
(Ixodes spp.) have caused more deaths than
funnel web spiders.
Prognosis
Prevention
Prognosis varies depending on the type of
History If visiting tick-infested areas, it may be diffi-
spider, but only for Australian funnel web
There may be a clear history of a tick cult to exclude all tick contact risk, so routine
spiders is death a likely outcome unless
being found, but often in children presen- checking for ticks after departure and their
specific treatment is urgently instituted.
tation is as an unexplained progressive removal is advisable.
flaccid paralysis, first manifesting as an
Prevention ataxic gait. Occasionally the paralysis
Spiders are ubiquitous and it is not practi- may be purely local, notably a Bells palsy. Controversies and future
cal to avoid human contact. In areas Without treatment, the envenoming may directions
where the potentially deadly funnel web cause complete respiratory paralysis. For
spiders are common, such as parts of Syd- The major controversy in the past was
Australian paralysis ticks only, the paraly-
ney, residents should avoid walking bare- the value of tick antivenom. This
sis may worsen for up to 48 hours after
foot, leaving clothes on the ground or antivenom is no longer available.
removal of all ticks. It is important to
putting on footwear without first checking ascertain if the patient had exposure to
for spiders. ticks, such as walking in scrubland in east-
ern Australia. Jellyfish stings
Introduction
Controversies and future Examination Jellyfish are numerous in all seas and
directions Examination is crucial, both to document the oceans and stings, mostly trivial, are com-
extent of paralysis and to locate every
Major controversies for spiderbite attached tick. These may be hiding in the
mon. A few jellyfish can cause more severe
centre around adequate treatment, stings and an even smaller number can
scalp, behind or in the ears or in body skin cause potentially lethal envenoming. Of
particularly the role of antivenom, with folds.
attendant risks, in envenoming unlikely the many species that cause some effects,
to prove lethal. This concern has held only the three groups of most medical sig-
Investigations nificance will be discussed here. All jelly-
sway in North America for widow There are no specific investigations for tick
spiderbites, with few cases offered fish have a common mechanism of
envenoming.
antivenom therapy. As a result countless envenoming, using individual sting orga-
patients probably suffer prolonged nelles (nematocysts) that both produce
Differential diagnosis
periods of eminently treatable major and inject the venom. This can result in
Apart from ticks, at least in Australia, flaccid
discomfort. The opposite situation occurs some venom directly entering small blood
paralysis can be caused by snakebite. Ataxia
in Australia where widow (redback) vessels in the skin, causing rapid envenom-
only can also be caused by exposure to
spiderbites routinely receive antivenom, ing. In the case of large jellyfish, like the
pesticides or some pharmaceuticals.
with apparently good results and little box jellyfish, with millions of nematocysts
risk. discharging simultaneously through the
Treatment skin, very rapid and severe envenoming
Bites by most spiders remain poorly The principal treatment for tick envenoming is can develop.
documented and, even for major prompt removal of all ticks. Care must be
species, treatment is controversial. For taken to lever the tick off, including mouth-
these latter, controlled trials are Box jellyfish
parts, and not squeeze it between fingers,
required to establish best treatment The Australian box jellyfish, Chironex fleck-
which forces in more saliva and often leaves
practice. eri, is found in northern Australian marine
the head embedded, when secondary infec-
waters and areas to the north, including
tion can ensue. The previously available tick
Borneo. It is the most dangerous of all jelly-
antivenom (Australia) is no longer produced.
fish and can even kill an adult human in less
Tick bite paralysis The paralysis resolves after several days,
than 5 minutes, from cardiac arrhythmia
during which time ventilatory support may
Introduction and arrest. Most stings are minor, not a
be needed.
Tick bite is probably common in some threat to life, and cause local pain only. In
regions, but rarely causes major harm to cases with extensive and severe stings,
humans. However, a few species of ticks Prognosis there is immediate excruciating pain, with
have toxic saliva, containing paralytic neu- With removal of all ticks and respiratory a ladder mark present, often with adherent
rotoxins, which can cause potentially lethal support, the prognosis should be optimistic. tentacles. Collapse may follow rapidly,

449
22.1 ENVENOMATION

either due to the pain or to cardiac effects. conditions and medications that might so treatment should be supportive and
Respiratory failure can develop, but it is increase the risk from envenoming. symptomatic. Narcotic analgesia (excluding
cardiac toxicity that is most likely to prove pethidine) is often required, with hyper-
lethal. tension most often treated with glyceryl
Examination
trinitrate (beware hypotension) and pulmo-
The sting area should be examined and the
nary oedema managed with oxygen, dopa-
Irukandji syndrome extent determined. For box jellyfish, stings
mine, adrenaline (epinephrine) and positive
This is caused by envenoming by a variety covering half or more of one limb or an
pressure ventilation.
of jellyfish, including Carukia barnesii, equivalent area on the trunk should be
Bluebottle envenoming is treated with
some of which are very small. The initial considered as potentially lethal. Such a sting
supportive and symptomatic care only.
sting may be trivial and may be from the area is easily achieved in small children,
bell rather than tentacles. However, 20 who are those most often involved in fatal
40 minutes later systemic envenoming envenoming. Evidence of systemic effects Prognosis
develops, with muscle and back pain, often should be sought. For box jellyfish envenoming, the larger
severe, hypertension and malaise and pul- the area of sting, the more severe the
monary oedema, consistent with an auto- envenoming, with half or more of one limb
Investigations
nomic storm. While death is very rare, it involved being potentially lethal. For Iru-
There are no specific tests for jellyfish
has been recorded as a result of intracranial kandji, the prognostic indicators are less
envenoming.
haemorrhage associated with severe hyper- clear.
tension, though the actual contribution of
envenoming is controversial. Differential diagnosis
Prevention
Except for Irukandji syndrome, which can be
The only sure way of preventing jellyfish
confused with non-envenoming illness,
Portuguese-man-o-war stings is to avoid using the sea. Stinger suits
jellyfish stings are usually easily diagnosed,
Blue bottles or Portuguese-man-o-war are can greatly reduce the chance of contact
the major differential diagnosis being other
global oceanic jellyfish (actually hydro- and will prevent potentially lethal box jelly-
types of marine envenoming, particularly
zoan colony organisms), which swarm fish stings, by limiting the area stung, but
stings from venomous fish, but these usually
and can cause stings from their tentacles. since only a small contact area is required
show only a few spine penetration points,
In most cases the stings are minor, with for Irukandji jellyfish, exposed face, hands
not the widespread tentacle tracks of
local pain and wheal formation, but rarely or feet may permit major envenoming.
jellyfish.
more severe envenoming is reported, with Similarly, stinger exclusion nets on beaches
a very few cases of vascular injury locally. will prevent large box jellyfish from entering,
More common is an allergic reaction to Treatment so preventing major stings, but do nothing
stings, occasionally resulting in lethal Most jellyfish stings require either no treat- to prevent Irukandji stings.
anaphylaxis. ment or simple symptomatic relief of
pain. Hot water (45 C, usually as a hot
shower) has proved the most effective first Controversies and future
History aid for reducing local pain from jellyfish directions
For most jellyfish there will be a clear his- stings, though its applicability to box jelly-
tory of being stung while in the sea and fish stings remains untested, so for these
There are a number of current
controversies for jellyfish sting
tentacles may still be adherent. These stings a cold pack is preferred. For box
management. For box jellyfish, while
may still be active, so caution is advisable jellyfish stings, where either an extensive
vinegar is widely accepted as first aid
on removal. For box jellyfish only, the first area has been stung or systemic envenom-
to inactivate unfired nematocysts,
aid application of copious amounts of vine- ing is evident, then box jellyfish antivenom
the use of pressure immobilisation
gar may inactivate the tentacles, so they is required urgently, either IM if pre-hospital,
bandages in severe cases has been
may be removed safely. The geographical or preferably IV in hospital, 13 vials.
questioned, research showing that
location is important, as is the time of In addition full cardiorespiratory support
this technique may actually increase
day and season, as this affects the likely should be instituted, where needed. Local
envenoming. As a consequence,
local jellyfish fauna. infection of the tentacle tracks can occur,
most authorities now do not
For stings by Irukandji-type jellyfish, the requiring antibiotic therapy. Many cases
recommend using pressure
presentation may be one of unexplained develop delayed allergic reactions locally,
immobilisation bandages for any
severe pain following a swim in the sea, at responding to antihistamines and topical
jellyfish stings.
any time of the year (but only in northern corticosteroids.
Australian waters and adjacent areas). It is Irukandji envenoming cannot be treated The role of antivenom for box
important to note pre-existing medical with antivenom (there is none suitable), jellyfish stings remains clouded in

450
22.1 ENVENOMATION
22

ENVIRONMENTAL
vessels, nerves, tendons and direct pene- determine the extent of any mechanical
uncertainty, but most authorities still
tration of the chest and abdomen, in- trauma is essential.
recommend its use. Verapamil, once
cluding direct cardiac puncture. Such
suggested for treating severe cardiotoxic
mechanical wounds can pose a great Investigations
box jellyfish envenoming, is no longer
threat to life, but are beyond the scope There are no specific investigations for fish
recommended by most authorities and
of this chapter, as envenoming is not the sting envenoming.
has never undergone clinical testing in
significant problem in such cases.
this setting.
Venomous fish exist in many families,
Differential diagnosis
 Irukandji envenoming remains representing hundreds of species, with ven-
The sharp and localised pain of a fish sting is
problematic. There are still calls for omous spines in a number of different loca-
distinctive and can be separated from jelly-
antivenom development, but the tions, depending on species, including on the
fish stings due to the tentacle tracks caused
delayed nature of envenoming and the back (dorsal as in stonefish), pectoral,
by the latter.
increasing and varied range of the behind the head (particularly catfish) and
species of jellyfish involved make even on the tail. A primitive venom gland
development of an effective surrounds the spine and as the spine is Treatment
antivenom problematic and forced into the skin by mechanical pressure With the exception of stonefish, for which
economically unviable. (such as by stepping on a stonefish or there is an antivenom, fish stings must be
handling a fish) the gland is compressed, treated symptomatically and supportively.
Research is required into the Both for first aid and in-hospital care, hot
forcing venom up groves in the spine and
mechanisms of major jellyfish water immersion appears effective at reduc-
into the victim.
envenoming in humans, as these are still ing pain in the short term. The contralateral
Most venomous fish species have never
not fully understood, mirroring the limb should first be immersed in water that is
had their venom studied. For those few that
difficulties in collecting and studying hot, but not so hot that thermal injury might
have been investigated, notably the stone-
jellyfish venom. occur. The affected limb is then immersed,
fish, while the venom may contain a variety
Research is required to determine of components, in the clinical setting it is usually bringing rapid relief of pain. Unfortu-
most effective treatment strategies for toxins causing pain and swelling that pre- nately, pain may recur on removal from the
jellyfish envenoming. Irukandji dominate. There is no evidence that the neu- water and if this persists, other analgesia
syndrome, in particular, requires more rotoxin found in stonefish venom has any must be considered, often a local or regional
research at all levels, starting with a clinical effect in humans. anaesthetic block in more severe cases. The
concerted effort to identify and describe wound must be examined for remnants of
all likely culprit species. stings, which should be removed. The wound
History
should be allowed to heal by secondary
The shift from cold pack to a hot There is always a history of definite or likely
intention and the temptation to surgically
shower as first aid for most jellyfish exposure to a stinging fish, in a marine or
close the wound resisted. For any wound
stings, though supported by evidence freshwater environment, or of handling a
that is extensive (i.e. many stingray injuries)
(unlike cold packs), is still questioned by fish out of water. This includes sudden pain
a course of antibiotics should be considered.
a few authorities. in a foot after walking in water, such as reef
For stingray injuries with extensive trauma,
walking, usually indicative of stepping on a
surgical input on managing this injury
fish, notably stonefish. The pain may be very
should be paramount.
severe, sufficient to cause collapse, but sys-
For stonefish, there is a specific anti-
Venomous fish stings temic symptoms are related principally to
venom in Australia, which can be given IM
local pain, not general toxicity. There are
or IV, the dose depending on the number
Introduction very rare reports of pulmonary oedema
of stings. It is effective at reducing the
There are numerous species of fish, in following stonefish stings (in Madagascar).
severe pain and should be considered in
both marine and freshwater environments, Portions of the sting may have been seen
all cases of stonefish sting with more than
both bony and cartilaginous, capable of in the wound.
trivial symptoms.
inflicting venomous stings, but envenom-
ing, though often distressing, is rarely
likely to be lethal, even in children. More Examination Prognosis
concerning, in the case of cartilaginous For venomous fish stings, there may be sting With the exception of severe mechanical
fish, specifically stingrays, is the potential present in the wound(s). The number of trauma from stingray injuries, the prognosis
for major, even lethal, mechanical injury wounds can be significant (i.e. for stonefish, for fish stings is generally optimistic, with
during the act of stinging. Stingray spines as it determines antivenom dose). For sting- recovery likely. The greater the number of
on the muscular tail can inflict major ray wounds, apart from residual sting left stings, the more likely that symptoms will
trauma, with cases of transection of in the wound, careful examination to be severe and possibly prolonged.

451
22.1 ENVENOMATION

Prevention Cone snails fire poisoned darts at their Prognosis

Avoidance of contact with stinging fish is prey, or human victims, the venom being If respiratory support is instituted early,
the obvious preventative measure. When both complex and incredibly potent, immo- before irreversible hypoxic organ injury
reef walking or on sandy bottoms where stin- bilising fish prey in a few seconds. In humans occurs, ultimate prognosis is good. Cases
grays may hide, avoid sudden movements, the sting may be painless or quite painful. without evidence of significant systemic
running into water, wear strong-soled reef Systemic envenoming, with collapse and envenoming 6 hours post-exposure are
shoes and observe and choose carefully flaccid paralysis, rapidly follows. unlikely to develop envenoming.
when placing feet. Despite all such precau-
tions, stings may occur, particularly if reef
walking, by stepping on larger stonefish. History Prevention
There is usually a clear history of picking up The principal preventative measure is absti-
and handling a blue ringed octopus or cone nence from contact with and certainly
snail. In significant cases, which are rare, handling these molluscs. This may require
Controversies and future
envenoming is rapid, with neurotoxic symp- local education programmes, particularly
directions toms quickly evident. The earliest feature of directed at children, who will find these
There are no major controversies for envenoming is often tingling of the lips. small and attractive animals tempting to
fish stings. There is uncertainty if pick up.
stonefish antivenom is useful for stings
by other types of fish. There is no formal Examination
study to validate such non-specific use, The sting or bite site may not be readily Controversies
but anecdotal clinical experience evident. The key effects to exclude are those
There are no major controversies in
suggests that this antivenom may work of progressive flaccid paralysis and cardio-
managing marine mollusc envenoming,
for some related species, including the vascular collapse, notably hypotension.
in part reflecting the rarity of this clinical
bullrout.
problem.
There is great scope for studies on
Investigations
fish sting venoms and for research into
There are no investigations specific to enve-
more targeted treatment strategies. Further reading
noming by molluscs.
While there are numerous papers on various aspects of
envenoming, covering different regions, there are few texts
giving a detailed overview of snakebite or other types of
Differential diagnosis specific envenoming or clinical toxinology. Some recent key
texts are noted here. A major source of information is the
The onset of progressive flaccid paralysis Clinical Toxinology Resources Website (www.toxinology.com),
Venomous marine molluscs after a marine sting or bite is uncommon a detailed site, initially developed with public funds, global
in scope.
Introduction and apart from mollusc envenoming, the
Chippaux J-P. Venins de Serpent et Envenimations. IRD ed.
There are two groups of marine molluscs likely differential is sea snake bite, which Paris: 2002. p. 288 [French handbook on global snakebite].
(snails) that are able to inflict major, even may manifest as either paralysis and/or Covacevich J, Davie P, Pearn J. Toxic plants and animals:
A guide for Australia. Brisbane: Queensland Museum;
lethal, envenoming on humans. These are myolysis. However, the development of 1987. p. 501 [a comprehensive textbook with a
the blue-ringed octopus, from Australian paralysis is much less acute than with clinical focus].
Gopalakrishnakone P, Chou LM. Snakes of medical importance
and adjacent waters and selected cone mollusc envenoming. (Asia-Pacific Region). Singapore: National University of
snails, found widely in the Indo-Pacific area. Singapore; 1990. p. 670 [covers snakes in the designate
region].
Neither are a common cause of envenoming, Goyffon M, Heurtault J. La Fonction Venimeuse. Paris: Masson;
surprisingly, since both groups are common Treatment 1995. p. 284 [French handbook covering many aspects of
toxinology].
the blue-ringed octopus particularly, with There are no antivenoms for venomous Junghanss J, Bodio M. Notfall-Handbuch Gifttiere:
its various species common all around the molluscs, so both first aid and treatment Diagnose, Therapie, Biologie. Stuttgart: Georg Thieme
Verlag; 1996. p. 646 [this German textbook covers a global
Australian coast. are supportive and symptomatic. Pressure spectrum of envenoming with a focus on management
The blue-ringed octopus has a potent immobilisation bandaging may reduce the in EDs].
Mebs D. Venomous and poisonous animals. Boca Raton, FL:
paralysing neurotoxin, tetrodotoxin, in its rate of development of systemic envenom- Medpharm/CRC Press; 2002. p. 339 [this textbook,
saliva. Bites, which usually occur when the ing, if applied early enough. The key require- beautifully illustrated in colour, covers the broad scope
of venoms, the animals that produce them and their clinical
octopus is removed from the water and ment is to support respiratory function and effects and treatment the focus is more on toxinology than
placed in contact with skin, are often pain- blood pressure. The latter may require pres- detailed medical advice, but it is a valuable source of
information].
less and may go unnoticed until 520 min- sor therapy. The former may require intuba- Meier J, White J, editors. Handbook of clinical toxinology of
utes or so later, when paralytic features tion and mechanical ventilation, but this animal venoms and poisons. Boca Raton, FL: CRC Press;
1995. p. 752 [this is the standard textbook for clinical
start to develop. These may rapidly progress may not be for a prolonged period, often toxinology and contains chapters covering all aspects of
to respiratory paralysis and collapse, with only for 612 hours, unlike snakebite, where envenoming, especially snakebite, scorpion stings,
spiderbite, tick envenoming and marine envenoming the
death possible in under an hour. The paraly- ventilation may be needed for days, weeks, coverage is global, but with considerable detail for most
sis is a general flaccid paralysis. even months. regions and animal types].

452
22.2 DROWNING
22

ENVIRONMENTAL
Sutherland SK, Tibballs J. Australian animal toxins. Warrell DA, et al. Oxford textbook of medicine. Oxford: Oxford is available in its entirety on the toxinology website:
Melbourne: Oxford University Press; 2001. p. 856 [a major University Press; 1996 [this standard medical text includes www.toxinology.com].
work covering the Australian venomous fauna]. an extensive chapter on envenoming]. Williamson JA, Fenner PJ, Burnett JW, Rifkin JF. Venomous and
Warrell DA. WHO/SEARO guidelines for the clinical White J. CSL Antivenom handbook. Melbourne: CSL; 2002. p. poisonous marine animals: A medical and biological
management of snake bites in the Southeast Asian region. 69 [a concise handbook on the diagnosis and handbook. Sydney: University of NSW Press; 1996. p. 504
SE Asian Journal of Tropical Medicine and Public Hygiene treatment of envenoming by Australian fauna, which [this is the major textbook covering marine toxinology].
1999;30(Suppl 1):185.

22.2 Drowning
Simon Wood

submersion/immersion in a liquid
ESSENTIALS medium. Implicit in this definition is that a
liquid/air interface is present at the
1 Drowning is defined as the process of experiencing respiratory impairment from entrance of the victims airway,
immersion in liquid, regardless of the outcome.
preventing the victim from breathing air.
2 Drowning is a leading cause of accidental death in Australian children. Children The victim may live or die after this
aged 04 years are the most vulnerable. The most common site of drowning in this process, but whatever the outcome, he or
age group is the domestic swimming pool. she has been involved in a drowning
incident. 2
3 There is no clinical or therapeutic difference between submersion in fresh or salt
water. The term submersion is generally accepted
to indicate an incident in which the victims
4 The major pathophysiological consequence of submersion is hypoxic brain injury. body is totally covered by water, while the
5 Pulmonary injury due to aspiration of water is an important clinical consideration term immersion refers to an incident in which
in all submersion victims. the victim is only partially covered by water,
although for drowning to occur the face and
6 The mainstay of treatment is early effective oxygenation. airway must at least be covered.2
7 Whilst hypothermia may be protective in small children who suffer a submersion ILCOR recommends that other terms such
event, it does not reliably predict good outcome. as dry drowning versus wet drowning, active
versus passive versus silent drowning,
8 Response to resuscitation is the single most important predictor of outcome in secondary drowning and near drowning be
children who have suffered submersion.
abandoned.2
9 Isolation pool-fencing with a self-locking gate has been shown to effectively
reduce submersion incidents in pre-school-age children.
Epidemiology
In Australia drowning is a leading cause of
accidental death in children. Its incidence
Introduction use of a time limit for survival is not a scien- peaks in early childhood and again in adoles-
Definition tific concept and is not in accordance with cence. Males outnumber females in both
Traditionally drowning has been defined as outcome parameters as used in the interna- groups. Children under 5 years of age are
death due to suffocation within 24 hours tionally accepted Utstein style.1 the most vulnerable to drowning in Austra-
of submersion in a liquid medium and To address this issue the Utstein Taskforce lia.3 In the period between 1 July 2008 and
near-drowning as survival for 24 hours or on Drowning was convened in Amsterdam 30 June 2009 there were 302 drowning
more following such an incident.1 Consider- as part of the 2002 World Congress on deaths in Australian waterways. Children
able confusion has surrounded the use of Drowning. The International Liaison Com- aged 0-4 years accounted for 11% of deaths
these terms. In part this is because the dis- mittee on Resuscitation (ILCOR) has since overall and 74% of deaths under the age of
tinction between drowning and near- endorsed a review of the terminology and 14 years. 59% of drowning deaths in the
drowning often cannot be made before 24 defines drowning in the following way: 04 years age group occurred in swimming
hours, making the terms clinically irrelevant. Drowning. Drowning is a process resulting pools, with around 84% of cases occurring
In addition it has been suggested that the in primary respiratory impairment from from wandering or falling in.4

453
22.2 DROWNING

Risk groups for childhood drowning are of drowning in infants and smaller children, event. Most paediatric drowning deaths in
children aged 04 years, children living in particularly in events that occur in the home, hospital are due to hypoxic cerebral injury
cities with high swimming pool to popula- such as in baths and buckets. Up to 8% of rather than pulmonary complications.8
tion ratios, children living in hot climates, drownings presenting to tertiary paediatric The average volume of water aspirated
children living in areas with lack of isolation centres may be attributed to child abuse.8 in human drownings is 1015 mL kg1. Aspi-
pool fencing, and Indigenous children.5 The immersion syndrome is sudden loss ration of volumes as little as 13 mL kg1
More toddlers drown in swimming pools of consciousness secondary to a bradycardia, of water can cause profound alterations in
than from any other cause.6 Most children or tachyarrhythmia induced by contact gas exchange and subsequent ventilatory
who drown in pools are out of sight for with water at a temperature of at least abnormalities.10 Laryngospasm is thought to
less than 5 minutes and are in the care of 5 C below body temperature. This can lead occur in 1015% of drowning victims and a
one or both parents.7 Around the home secondarily to drowning. The immersion syn- subset of patients who drown without evi-
small children can also drown in baths, buck- drome can occur in water with temperatures dence of significant aspiration of water at
ets, and garden ponds. Up to 8% of cases of as warm as 31 C, although it is more likely to post-mortem, so-called dry-drowning, has
drowning in small children in the domestic occur in much colder water. Wetting the face been described. This concept has recently
setting may be secondary to non-accidental before entering the water may reduce its been questioned and it has been suggested
injury.8 incidence.1 that in these cases death has occurred prior
While pool-fencing legislation has proven to submersion.1 Regardless of whether dry-
to be effective in reducing the incidence drowning is a true clinical entity, or whether
of drowning in small children it has had laryngospasm has occurred at the time of sub-
little impact on rates of drowning in older
Pathophysiology mersion, aspiration of water into the lungs
children and adolescents. In this group alco- The two most significant pathophysiological remains a clinically important consideration
hol, suicide, and risk-taking behaviours are consequences of submersion are hypoxia in the management of all drowning victims.
important factors that lead to increased risk from asphyxiation during the submersion Despite the large literature dedicated
of drowning.8 itself, and aspiration of water into the lungs. to the subject, there are no clinically or
It is the severity of the initial hypoxic insult therapeutically important differences
that is the major determinant of outcome. between drowning in fresh or salt water.1,8
If the initial hypoxic event is survived, the Pulmonary injury is related more to the
Aetiology amount of water aspirated than to the
degree of hypoxic organ injury and pulmo-
Drowning is most commonly a primary nary injury secondary to aspiration become composition of the water itself. Both fresh
event. In children it most often occurs when the clinically important factors. and salt water cause loss of pulmonary sur-
the victim is unable to rescue him or herself Much that is known about the sequence factant, non-cardiogenic pulmonary oedema,
after entering the water, as in the case of of events following submersion has come impaired alveolar-capillary gas exchange, and
a toddler falling into a swimming pool, or from animal models. Aspiration of water increased intrapulmonary shunting with the
an infant drowning in a bath whilst unat- initially causes breath-holding or laryngos- potential for profound hypoxia.1 Aspiration
tended. In older children and adolescents pasm and the resultant asphyxiation leads of water that is contaminated with particu-
fatigue while swimming may play a role, to progressive hypoxia. Active and passive late matter or bacteria can lead to compli-
but drowning in these age groups is more swallowing of water follows and, as hypoxia cations from obstruction of small airways
likely to be secondary to other causes. worsens, breath-holding and laryngospasm or increased risk of pulmonary infection,
Drowning can occur secondarily to a num- are terminated, resulting in aspiration of although neither is seen in the majority of
ber of underlying causes. These should be water into the lungs.1 patients.11 If present, evidence of significant
considered during assessment of the sub- Anoxia lasting 13 minutes can shut pulmonary injury due to aspiration will usu-
mersion victim. Individuals with seizure dis- down both the brain and the heart, causing ally manifest or progress within hours of res-
orders have up to 19-times higher risk for loss of consciousness and hypoxic cardiac cue. Delayed onset of respiratory distress and
drowning accidents, regardless of age.3,8,9 arrest. Rescue and early institution of hypoxia, the so-called delayed immersion
Prolonged QT-syndrome leading to dysrhyth- cardiopulmonary resuscitation can salvage syndrome or secondary drowning has been
mia has been implicated as a significant myocardial function, but the brain is more refuted by recent evidence.8
cause of drowning, although the true inci- sensitive to hypoxic injury and it is the sever- Clinically significant electrolyte and fluid
dence of this condition in drowned children ity of this injury that determines outcome. volume abnormalities are rarely seen in
is unknown. Ethanol is an important risk fac- Effects of hypoxia on other organ systems cases of drowning in humans despite
tor for drowning injury, particularly in ado- are delayed. Profound hypoxia can cause being demonstrated in animal models.1,3,8,11
lescents. Elevated serum ethanol levels are an acute respiratory distress syndrome, Occasionally a mild hyponatraemia, which
documented in 1050% of adolescent which develops within hours and further self-corrects without specific therapy, is
drownings.8 Head and cervical trauma from worsens hypoxic injury. Posthypoxic cerebral observed.8 Theoretical exceptions are
diving and boating-related accidents may oedema is a major complication and can drownings occurring in hypertonic solutions,
also lead to drowning as a secondary event. develop 612 hours following successful ini- such as the Dead Sea, or water contami-
Non-accidental injury is an important cause tial resuscitation from a serious submersion nated with industrial waste.

454
22.2 DROWNING
22

ENVIRONMENTAL
Hypothermia is an important issue patients. It is important to detect hypother-
following drowning, particularly in small History mia, particularly in patients who have failed
children who have a large body surface area Key points in history are summarised in to respond to resuscitation efforts. Small
to weight ratio. Cooling can occur at the Table 22.2.1. Broad areas in history include children may rapidly become hypothermic
time of submersion, but can also continue details of the drowning event itself, details due to evaporative heat loss during resusci-
following rescue and during attempted of rescue and resuscitation, response to tation efforts and transfer to the hospital.
resuscitation due to heat loss through resuscitative efforts, possible underlying Apparent lifelessness and severe brady-
evaporation. Hypothermia can confer some causal factors, and medical conditions that cardia due to profound hypothermia need
degree of protection from cerebral hypoxia, may influence recovery. Features of history to be distinguished from asystole and
particularly in small children. Multiple case that alert to the possibility of non-accidental brain death. Assessment of cardiac rhythm
reports in the literature attest to intact injury should be recognised. These include a requires observation of the continuous elec-
survival of both children and adults follow- history that is inconsistent or a history that is trocardiograph (ECG) monitor for up to a
ing prolonged (>15 minutes) drownings incompatible with the victims developmen- minute to detect very slow heart rates that
in icy water (water temperature <10 C).12 tal level. can occur in profound hypothermia. Simi-
Profound hypothermia and subsequent larly, lack of response to painful stimulus,
intact survival has also been documented along with fixed and dilated pupils should
in children suffering drowning in non-icy not be interpreted as brain death in the
water and in temperate climates. Examination presence of profound hypothermia.
The mechanisms of temperature drop and Depending on the clinical condition of the
Examination is dictated by the clinical condi-
cerebral protection remain unclear. Surface victim at arrival, neurological evaluation
tion of the victim on arrival in the emergency
cooling at the time of submersion is thought may range from an assessment of level of
department (ED) and is mainly directed
to be insufficient on its own to provide cen- responsiveness as graded by the AVPU or
at assessing the degree of neurological
tral cooling of a degree that confers cerebral Glasgow coma score (GCS) and pupillary
impairment due to hypoxic cerebral injury,
protection. Other mechanisms of heat loss, reaction, to a focused neurological examina-
the severity of respiratory embarrassment
such as via ingestion and/or aspiration of tion looking for focal deficit or spinal-cord
due to aspiration, and cardiovascular insta-
cold water, are not supported by quanti- injury. An assessment should always be
bility due to a combination of the initial
tative evidence. Some authors suggest that made for the possibility of cranial or cervical
insult and/or ongoing hypoxia. In broad
core temperature drop is insufficient on its spine trauma, particularly in diving-related
terms drowning victims arriving in the ED
own to explain the cerebral protection accidents. Trauma to other areas of the body
will fall into two categories: (1) those who
afforded by hypothermia.13 The diving should also be sought.
respond to minimal resuscitation and who
reflex, in which blood is shunted from the In the awake child, examination of the
will generally do well with minimal com-
limbs and splanchnic circulation to the brain respiratory system can establish a clinical
plications; and (2) those high risk patients
and heart alongside slowing of the heart baseline against which subsequent dete-
who fail to respond to resuscitation and
rate and reduction of the basal metabolic rioration can be measured. Work of breath-
who will require ongoing resuscitation
rate, has been suggested as being an impor- ing should be assessed, and abnormalities
and/or monitoring.8
tant mechanism for cerebral protection in found on auscultation, such as crackles
Vital signs, including oxygen saturation,
children.13 There is little clinical evidence and wheezes, should be noted. The finding
a bedside blood glucose estimate, and a
to indicate that the diving reflex is suffi- of signs on an initial examination indicates
temperature should be recorded on all
ciently active in humans, even small chil- the possibility of significant aspiration
dren, to confer any benefit on its own.1,8 and dictates close observation to identify
It is most likely that a combination of the deterioration. A clear chest initially does
effects of the diving reflex initially, followed Table 22.2.1 Key points in history not exclude aspiration and a period of
by rapid and continued cooling, is what The circumstances leading to the submersion
observation and re-examination is necessary
underlies the cerebral preservation that The location of the submersion in all children who have been symptomatic
Water and environmental temperatures
is sometimes seen in small children who Duration of submersion
following drowning.
suffer submersion and who are profoundly Time from when the victim was last seen to the
time when found (if the submersion was
hypothermic. unwitnessed)
By whatever mechanism cooling occurs, Time from rescue to effective CPR
and whether the diving reflex plays a signifi- Time to first gasp or return of spontaneous Investigations
circulation
cant role in cerebral protection or not, hypo- Elements of basic and advanced life support Investigations will also be dictated by the
employed
thermia in the drowning victim, particularly Symptomatology following successful
clinical condition of the patient on arrival
if the victim is an infant or young child, resuscitation in the ED. A child who has suffered a drown-
Medical history particularly with regard to
should be considered to be an indication possible predisposing factors (i.e. neurological
ing injury and who is alert and asymptom-
for aggressive and prolonged resuscitation disorders, such as epilepsy) atic on arrival requires little in the way of
Factors that may complicate recovery (i.e.
efforts. This issue is discussed further in respiratory illness, such as asthma)
laboratory or radiological evaluation. The
Chapter 22.4 on cold injuries. more symptomatic or seriously unwell child

455
22.2 DROWNING

may benefit from further evaluation with Non-accidental injury should be suspected clothing should be removed and the
laboratory and radiological investigations. when there are inconsistencies in the history. patient should be dried if possible. Exposure
Useful investigations include arterial Severe hypothermia (core temperature during cardiopulmonary resuscitation (CPR)
blood gas (ABG) analysis, serum glucose, less than 29 C) can mimic irretrievable should be minimised as much as is practica-
and chest radiography. ABG analysis may cardiorespiratory arrest and brain death. ble. If hypothermia is severe at the scene
demonstrate a metabolic acidosis, which The profound bradycardia associated with (<30 C), rewarming should probably be
confirms a significant drowning injury.8 very low core body temperature can easily delayed until adequate ventilation and
The severity of the acidosis reflects the be confused with brady-asystole secondary oxygenation has been instituted.15 Invasive
severity of the hypoxic insult as well as to hypoxic injury and will not respond to rewarming techniques should be reserved
ongoing hypoxic injury. Profound acidosis usual resuscitation measures until hypo- for the hospital phase of management.
(pH <7.10) implies a poorer prognosis but thermia is corrected. Similarly, severe hy- Treatment in the ED involves provision of
needs to be interpreted in the clinical con- pothermia can cause depression of cerebral adequate oxygenation, stabilisation of the
text.14 ABG analysis can be used to guide function leading to unresponsiveness and body temperature, prevention of complica-
decisions regarding oxygenation and venti- fixed, dilated pupils, indistinguishable from tions such as aspiration, and assessment of
lation, and serial determinations may be irreversible hypoxic cerebral injury.15 Failure the patients clinical status in order to make
useful in monitoring and quantifying deteri- of the core temperature to rise despite decisions regarding ongoing management
oration of pulmonary function due to non- aggressive, active rewarming may be the only and disposition.
cardiogenic pulmonary oedema secondary indication that death has occurred. Supplemental oxygen should be adminis-
to hypoxia or aspiration. Determination of tered at concentrations and via delivery
the blood glucose level is important in any systems appropriate to the patients oxygen
critically ill child as hypoglycaemia can requirement. Continuous pulse oximetry and
complicate physiological stress and should
Treatment serial estimations of respiratory rate and
be actively treated. Hyperglycaemia in Treatment of the drowning victim occurs in work of breathing should be undertaken
a comatose child, although not requiring three major phases: (1) rescue and resus- during observation in the ED to determine
treatment, implies a poor prognosis.8 Initi- citation at the scene; (2) initial assessment worsening or improvement of the patients
ally the chest X-ray (CXR) may be normal, and stabilisation in the emergency depart- respiratory status. If available, non-invasive
may demonstrate pulmonary infiltrates, or ment; and (3) subsequent observation and ventilation techniques, although not exten-
may display frank pulmonary oedema. supportive care in the hospital ward, inten- sively evaluated in paediatric drowning
Abnormalities detected on an early CXR sive-care unit (ICU), or after discharge. victims, may be considered if the oxygen
mandate close observation and the patient Early institution of effective cardiopul- requirement outstrips conventional mechan-
should be monitored for clinical deteriora- monary resuscitation with an emphasis on isms of oxygen delivery. Intubation to isolate
tion. Repeated CXR may be required but providing adequate ventilation is the key the airway and provide ventilation with
should be dictated by the patients clinical task in pre-hospital management. Man- positive end-expiratory pressure (PEEP)
condition. oeuvres to drain the lungs of water have may be required in patients with depressed
Other investigations that may be helpful not been shown to be clinically effective neurological status or if respiratory compro-
include baseline electrolytes and full blood and may increase the risk of aspiration of mise progresses despite supplemental oxy-
count, although rarely are any clinically or gastric contents. The Heimlich manoeuvre gen therapy. Failure to maintain an SaO2
therapeutically significant abnormalities should be reserved for cases in which of greater than 90% with an FiO2 of 0.50
found on initial determinations. Blood repeated attempts to position the airway or higher, a PaCO2 of more than 35 mmHg,
ethanol levels may be relevant, depending and provide ventilation have been unsuc- an abnormally high respiratory rate (>50
on the age of the patient. A 12-lead ECG cessful or when foreign body aspiration bpm) or inadequate spontaneous ventila-
may be helpful in excluding prolonged QT and obstruction is suspected.1 Emesis is tion, are all indications that mechanical
syndrome as a cause for the drowning common in drowning victims, both sponta- ventilation is likely to be required.1,10 Pas-
event.10 Cervical spine films should be con- neously and as a complication of resuscita- sage of a nasogastric or orogastric tube,
sidered if cervical injury is suspected or the tion, and aspiration of gastric contents is if cranial trauma is suspected, should be
drowning is secondary to a diving accident. a major potential complication following performed following intubation to decom-
rescue. Spontaneously breathing patients press the stomach and facilitate mechanical
should be managed and transported in ventilation.
the right lateral decubitus position. Cricoid Diuretics are not recommended in the
Differential diagnosis
pressure may reduce the risk of gastric management of non-cardiogenic pulmonary
The major issues in differential diagnosis distension and aspiration during cardiopul- oedema.1,8,10 Steroids have not been shown
relate to the cause of the drowning event. monary resuscitation but requires an addi- to be useful in the management of aspira-
Underlying medical conditions, such as tional rescuer.1 tion pneumonitis and the role of antibiotics
epilepsy, should be considered. Trauma, Hypothermia can be exacerbated by in this setting remains controversial.1,8,10
either leading to or as a consequence of ongoing evaporative heat loss during Prophylactic antibiotics are generally not
the drowning, should be recognised. resuscitation efforts following rescue. Wet recommended, although they are sometimes

456
22.2 DROWNING
22

ENVIRONMENTAL
used when there is a history of drowning in of hypoxic organ injury, and management of pulmonary oedema up to 12 hours after
heavily contaminated or polluted water. In the complications of pulmonary aspiration. drowning have been reported as occurring
general, antibiotics should be reserved for The use of PEEP in drowning victims venti- in patients who appear well and display nor-
patients who develop signs of pulmonary lated in the ICU is controversial. Although mal chest radiography in the ED.1 Any child
infection, such as fever or leukocytosis.1 PEEP may be necessary to maintain ade- discharged from the ED following a drown-
Fluid therapy should be judicious and should quate oxygenation, some authorities express ing event should be in the care of a reliable
be aimed at maintaining adequate circula- concern regarding the potential reduction and responsible adult. Instructions should be
tory status and euglycaemia without over- in cerebral venous outflow and subsequent given to re-present for further medical
loading the patient. increase in intracerebral pressure associated assessment in the event of any change in
Hypothermic patients should be warmed with its use. PEEP may have unfavourable the childs respiratory status.
once adequate ventilation and oxygenation effects on cardiovascular status and may Patients who have suffered trauma may
has been assured. Awake and spontaneously increase the risk of barotrauma in already require admission for the management of
breathing patients with mild to moderate insulted lungs.8 Other ventilation strategies their injuries. Children with underlying
hypothermia (core temperature >32 C) to minimise non-cardiogenic pulmonary cardiac or respiratory disorders, those who
require passive or external active rewarming oedema and barotrauma, such as pressure- suffer drowning secondary to a pre-existing
techniques only. Patients with severe hypo- control ventilation with low-peak airway illness, and victims of suspected non-
thermia (core temperature <32 C) who pressure and prolonged expiratory time, accidental injury also warrant inpatient
are obtunded or in cardiorespiratory arrest and permissive hypercapnia have been sug- evaluation.
will require invasive active rewarming tech- gested, although again the trade-off is the Asymptomatic children and children with
niques. These are discussed fully elsewhere risk in adversely influencing intracerebral mild symptoms who are admitted for obser-
in this text (see Chapter 22.4 on cold inju- pressure.8 Various measures to provide cere- vation can be managed in a general ward
ries). Whilst there is increasing support for bral resuscitation and control intracranial environment, provided that there is the facil-
the use of therapeutic hypothermia (cooling pressure have been evaluated in the litera- ity to increase the level of monitoring and
to a core temperature of 33 C) following car- ture and have generally been found to be care should it be required. Children who have
diac arrest in adults, and the technique has not helpful in influencing outcome.1 In required CPR, who have abnormal chest radi-
been used in adults following drowning, its general, the aim should be to maintain ade- ography or ABGs on arrival in the ED, or who
role in the management of children remains quate cerebral oxygenation and to minimise have required ventilatory support, should be
to be established and may, in fact, be asso- causes of secondary cerebral injury such admitted to a high-dependency unit or ICU
ciated with harm.16 as hypotension, hypercapnia, and hypo/ for observation and management.1 Transfer
In the setting of cardiorespiratory arrest hyperglycaemia. to a facility that provides paediatric ICU
and severe hypothermia, resuscitation should be considered for all such patients
efforts should be continued until the core prior to clinical deterioration.
temperature has risen to around 32 C
Disposition
before a decision to cease resuscitation is
made. Although there are no clear guide- Disposition is dictated by the clinical condi-
lines to determine how long efforts at
Prognosis
tion of the patient in the ED, the nature
rewarming should be continued, failure to of the drowning event, the need for resus- Most children who suffer drowning injury
effect rewarming despite maximal invasive citation, and the presence or absence of will either survive intact or die. Death
efforts may be an indication that continuing other factors, such as trauma, suspicion occurs in 3050% of drowning victims,
resuscitation is futile. The decision to cease of non-accidental injury, and underlying or most of these not surviving to treatment
resuscitative efforts in the presence of per- complicating medical conditions. In general, in the ED. A small proportion of victims
sistent severe hypothermia should follow a all victims of drowning will require some will be left with severe neurological deficit,
multidisciplinary approach involving the period of observation. either persistent vegetative state or spas-
emergency physician, paediatrician and/or The alert, otherwise healthy, patient who tic quadriplegia. Mild learning deficits
paediatric intensivist and other members is asymptomatic or who has suffered only may occur in apparently intact survivors,
of the resuscitation team, together with mild, transient symptoms following a brief although the extent of these and the
the parents and family. Cardiorespiratory drowning can be safely discharged if they impact on subsequent function have not
arrest on arrival in the ED in a patient with remain well after 68 hours of observation.8 been clearly quantified.8 In general, how-
a core temperature above 32 C carries a uni- Patients with a history of drowning for lon- ever, the prognosis of children who suffer
formly poor prognosis and prolonged efforts ger than 1 minute, a period of cyanosis or drowning events and survive to hospital
at resuscitation are generally not indicated. apnoea, or who required pulmonary resusci- admission is excellent.17
Treatment in the ICU involves provision of tation, should be admitted for observation Despite a large body of literature dedi-
ventilatory support to ensure adequate oxy- for 24 hours, or at least overnight, even if cated to the subject, the factors that predict
genation, maintenance of cardiovascular they are well in the ED. Although recent evi- prognosis in the ED following childhood
stability, minimisation of secondary brain dence discredits the idea of the delayed drowning injury remain poorly defined.
injury due to cerebral oedema, management immersion syndrome, cases of fulminant There are no prospectively validated scoring

457
22.2 DROWNING

systems and most of the literature is based patients lack of response to aggressive
on retrospective data. Individual case rewarming and resuscitation becomes a Controversies and future
reports of children surviving prolonged surrogate indicator of poor outcome. In directions
resuscitative efforts with good outcome con- non-hypothermic patients who are in cardio- The role of therapeutic hypothermia
tinue to arise in the popular press and medi- respiratory arrest, a lack of response to following cardiac arrest due to drowning
cal literature. 25 minutes of effective advanced life in children is controversial.
Factors elicited in history that influence support measures is almost universally
prognosis are duration of submersion, time associated with poor outcome.8 It should Prospective collection of data on
to institution of effective CPR, and time to be noted, however, that studies of outcome drowning victims is needed to generate
first spontaneous gasp, all three reflecting following out-of-hospital cardiac arrest more useful information regarding
the duration of the hypoxic cerebral insult. in children indicate that children who prognosis. Scoring systems for prognosis
Duration of submersion can often be diffi- suffer cardiac arrest secondary to drowning should also be prospectively validated.
cult to determine but submersion for have significantly better outcomes than  Further research regarding
longer than 5 minutes is associated with those who suffer cardiac arrest from other preventative strategies is required. Areas
a poorer prognosis.14 Although time to causes. of interest would be the relationship
institution of effective CPR can be similarly between swimming lessons and
difficult to ascertain accurately, delays of drowning and the development of
longer than 1020 minutes are also asso- standardised and uniform legislation
ciated with poorer outcomes, while early pertaining to pool fencing in Australia.
effective CPR has been shown to be an
Prevention
important factor in improving survival after Drowning remains a leading cause of acci- Preventive educational programmes
rescue.14 dental death in toddlers and adolescents aimed at older children and adolescents
As already discussed, hypothermia may in Australia. Most drowning-prevention are lacking, and are an area of possible
be protective in small children who suffer strategies are aimed at the small child future development in public health.
drowning, particularly, but not exclusively, who drowns after falling into the domestic
if the drowning has occurred in cold or icy swimming pool, with few or none aimed at
water. However, neither hypothermia nor older children and adolescents.
water temperature can be reliably used to Although programmes to prevent drown-
predict outcome.18,19 ing by teaching children under the age of References
Response to resuscitation following res- 4 to swim remain unproven, there is increas- 1. Orlowski JP, Spilzman D. Drowning: Rescue, resuscitation,
reanimation. Pediatr Clin North Am 2001;48(3):62746.
cue and prior to arrival in the ED is the single ing evidence that such programmes may 2. Idris AH, Berg RA, Bierens J, et al. Recommended
most important indicator of outcome in chil- be effective and at least are not asso- guidelines for uniform reporting of data from drowning.
The Utstein style. ILCOR Advisory Statement. Circulation
dren who have suffered drowning. Children ciated with an increased risk of drowning. 2003;108:256574.
who arrive conscious in the ED after success- One recently reported casecontrol study 3. Mackie IJ. Patterns of drowning in Australia, 19921997.
Med J Aust 1999;171:58790.
ful resuscitation have almost universally demonstrated an 88% reduction in the risk 4. Royal Life Saving Society Australia. The National
excellent outcomes. Similarly, lack of of drowning in children aged 1 to 4 years, Drowning Report. Sydney: RLSSA. Available from http://
www.royallifesaving.com.au; 2009 [accessed 26.10.10].
response to early resuscitation efforts and although the 95% confidence intervals 5. Edmond KM, Attia JR, DEste CA, Condon JT. Drowning
coma on arrival in the ED are associated were wide (399%).22 and near-drowning in Northern Territory children.
Med J Aust 2001;175:6058.
with a poor outcome.10 In a classification Public-education programmes outlining 6. Pitt WR, Cass DT. Preventing children drowning in
system based on the neurological status of the need to provide adult supervision when Australia. Med J Aust 2001;175:6034.
7. Moon RE, Long RJ. Drowning and near-drowning. Emerg
the patient on arrival in the ED, it was dis- children are near water may be helpful. Pool Med 2002;14:37786.
covered that less than 15% of patients alarms, pool covers, and fencing that does 8. Quan L. Near-drowning. Pediatr Rev 1999;20(8):25560.
9. Bell GS, Gaitatzis A, Bell CL, et al. Drowning in people
who were unresponsive and flaccid to pain- not isolate the pool from the home are with epilepsy. How great is the risk? Neurology
ful stimulation had intact survival. This is probably not effective, although evidence 2008;71:57882.
10. Sachdeva RC. Environmental emergencies. Near
supported by the findings of other investiga- is lacking. Isolation or four-sided fencing drowning. Crit Care Clin 1999;15(2):8196.
tors that a GCS of 5 or less on arrival in the with a self-locking or dynamic gate, on the 11. Modell JH. Drowning. N Engl J Med 1993;328(4):2536.
12. Orlowski JP. Drowning, near-drowning, and ice-water
ED is associated with a dismal outcome, other hand, has been shown to effectively submersions. Pediatr Clin North Am 1987;34(1):7592.
either death or severe neurological disabil- reduce drowning incidents by up to 50%, 13. Gooden BA. Why some people do not drown.
Hypothermia versus the diving response. Med J Aust
ity.1,20 Lack of pupillary response in the ED in both Australia and New Zealand, and in 1992;157:62932.
has similarly been identified as a predictor the United States.8 Pool-fencing legislation 14. Orlowski JP. Prognostic factors in pediatric cases of
drowning and near-drowning. J Am Coll Emerg Phys
of dismal outcome.21 The caveat concerning exists in Australia, but is not uniform across 1979;8(5):1769.
the use of level of responsiveness and pupil- states. Compliance with legislation remains 15. Theilade D. The danger of fatal misjudgement in
hypothermia after immersion. Successful resuscitation
lary reaction to predict poor prognosis and incomplete. Further research is required following immersion for 25 minutes. Anaesthesia
withdraw treatment applies to the child to aid in establishing uniform pool-fencing 1977;32:88992.
16. Williamson JP, Illing R, Gertler P, Braude S. Near-drowning
who presents with severe hypothermia, requirements based on Standards Australia treated with therapeutic hypothermia. Med J Aust
which may mimic brain death. In these specifications.6 2004;181(9):5001.

458
22.3 HEAT-INDUCED ILLNESS
22

ENVIRONMENTAL
17. Pearn J. Neurological and psychometric studies in 19. Conn AW, Montes JE, Barker GA, Edmonds JF. Cerebral 21. Graf WD, Cummings P, Quan L, Brutaco D. Predicting
children surviving freshwater immersion accidents. salvage in near-drowning following neurological outcome in pediatric submersion victims. Ann Emerg Med
Lancet 1977;1(8001):79. classification by triage. Can Anaesth Soc J 1980;27 1995;26(3):3129.
18. Suominen PK, Korpela RE, Silfvast TGO, Olkkola KT. (3):2019. 22. Brenner RA, Saluja Taneja G, Haynie DL, et al.
Does water temperature affect outcome of 20. Dean JM, Kaufman ND. Prognostic indicators in pediatric Association between swimming lessons and drowning in
nearly drowned children? Resuscitation 1997;35: near-drowning: the Glasgow coma scale. Crit Care Med childhood. A case-control study. Arch Pediatr Adolesc
1115. 1981;9(7):5369. Med 2009;163(3):20310.

22.3 Heat-induced illness

Michael Ragg

ESSENTIALS Causes of heat-related

illness
1 Most heat-induced illness in children is due to overheating from exogenous
sources. Overheating
Usually seen in the setting of high
2 Children are particularly susceptible to heat-related illness due to their unique ambient temperature and humidity.
physiology. Examples are athletics, summertime
3 Certain genetic and dermatological disorders in children put them at high risk of sporting events and during heat waves.
heat-related illness. Young children left in cars are also
particularly at risk. A review of 171 cases
4 Genuine heat stroke is a true medical emergency. It is characterised by a core body of heat-related car deaths found that in
temperature above 40.5 Celsius in association with acute mental state changes. approximately 25% of cases the child
5 Immediate aggressive cooling methods should be instituted for heat stroke. gained access to an unlocked vehicle
and in 75% of cases the child was left by
6 Children account for almost one-fifth of all cases of malignant hyperthermia. an adult.4
Dantrolene should be given early for suspected cases. Approximately 50% of cases Increased heat production:
have a gene defect of the ryanodine receptor. Metabolic (e.g. hyperthyroidism)
Drugs (e.g. anticholinergics such
as atropine, sympathomimetics, aspirin,
neuroleptic agents, selective serotonin
Introduction Children have fewer cardiovascular reuptake inhibitor drugs)
compensatory mechanisms to deal with Seizures.
Hyperthermia in the paediatric patient heat insult.  Decreased heat loss:
differs physiologically from fever. Fever is Young children, particularly, produce less Over-wrapped babies
caused by an elevation of body temperature sweat, which limits their ability to lose Excess clothing on hot/humid days
secondary to regulation by the hypothala- heat through evaporation. Disease states (e.g. cystic fibrosis)
mus. High body temperature not caused Children depend on parents or adult Heart disease
by hypothalamic thermoregulatory mechan- caregivers to protect them from Drugs (e.g. phenothiazines,
isms is usually caused in children by one exogenous heat sources and cannot anticholinergics).
of the following: prolonged exposure to manipulate their environment to cool
high ambient temperature (overheating), themselves.2
Clinical syndromes
increased heat production or reduced heat
Certain genetic disorders such as ecto- The neonate/infant
loss. In the neonate and small infant, over-
dermal dysplasia and Fabrys disease Overheating is the most common cause in the
heating is almost always the cause.
also put children at risk of heat-related neonate and infant. It is important to distin-
In children, heat-related illness is the sec-
illness.3 In these conditions, there is an guish the healthy infant who is overheated
ond most common cause of non-traumatic
impaired ability to dissipate heat through from the febrile infant (Table 22.3.1).5
death after heart disease.1 Children are
sweating. Mild overheating is usually not dangerous
particularly at risk because of the following:
Rising temperatures and more extreme to infants though there may be some asso-
Children have a greater body surface area weather events due to climate change have ciation with apnoeic episodes in premature
relative to mass and therefore are more the potential to increase the incidence of babies.5 Hyperthermia from overheating,
susceptible to radiant heat. heat-related illness in children. however, is dangerous and has been linked

459
22.3 HEAT-INDUCED ILLNESS

Table 22.3.1 The overheated and febrile infant

Other heat-related syndromes
Malignant hyperthermia (MH) is usually
Overheated infant Febrile infant seen in the paediatric patient after general
High rectal temp High rectal temperature anaesthesia. 75% of victims have no family
history and 20.9% have had a previous nor-
Warm hands and feet Cool hands and feet
mal anaesthetic.9 Mutations in the gene
Pink skin Pale skin that programmes the ryanodine receptor,
Extended posture Lethargic which is a tetrameric calcium release chan-
nel in the sarcoplasmic reticulum, are asso-
Healthy appearance Looks unwell
ciated with malignant hyperthermia.10
Abdomen temperature > hand temperature Abdomen exceeds hand skin temperature This gene defect of the ryanodine receptor
by <2 degrees by >3 degrees
is present in 50% of cases. Approximately
18% of all cases of malignant hyperthermia
with sudden death,6 particularly in families salt are lost in their sweat), heat exhaus- occur in children, and their mortality rate is
with a history of malignant hyperthermia. tion may occur predominantly due to salt lower as compared to adults.11
Babies with heat-related illness may depletion.8 Initially, an increase in heart rate is fol-
present like any seriously ill infant. Hewsons lowed by an elevation of blood pressure.
work has summarised this as A,B,C, fluids Heat stroke Because these children are often paralysed,
in and out approach.7 Heat stroke is defined as a core body tem- tachypnoea may not be seen. Elevation of
perature >40.5
C acute mental state the end tidal CO2 is also an early sign. Mus-
Heat syncope changes. There are two types: exertional cle rigidity or increased tone may become
Heat syncope commonly involves children and classical. In the paediatric population, apparent and the body temperature rises
who have been standing for prolonged exertional heat stroke is most likely to occur at a rate of 12 degrees Celsius every 5 min-
periods in hot/humid weather or who have in the adolescent who is exercising vigor- utes. When succinylcholine is used, however,
undergone strenuous exertion. ously in a hot and humid environment. an acceleration of the syndrome may occur.
Sweating is usually present in this group. Hyperkalaemia, hypercalcaemia, metabolic
Heat cramps Classical heat stroke, however, may be seen acidaemia and myoglobinuria follow.
Heat cramps occur in the setting of strenu- during heat waves or when children are left The condition of masseter muscle rigidity
ous exercise in hot/humid conditions. in cars in hot weather. (MMR) is said to be associated with malig-
A relative lack of body salt plays a part. Heat Irritability, confusion, ataxia are common. nant hyperthermia. The peak age group is
cramps can be very painful; however, most Seizures may be seen, particularly during 812 years. It occurs after administration
last for less than 1 minute. Abdominal cooling. Coma may be the most common of succinylcholine. In approximately 50%
muscle cramps have been known to simulate presentation; however, the childs conscious of patients MH occurs after MMR is first
an acute abdomen.8 state may improve in the pre-hospital seen. The rigidity can usually be overcome
environment. with effort and resolves after 23 minutes.
Heat exhaustion Other serious complications of heat Repeat doses of succinylcholine do not help.
Heat exhaustion in children presents as stroke include cerebral oedema, liver injury, The association between MH and skeletal
hyperpyrexia, vomiting, headache, lethargy renal failure secondary to rhabdomyolysis, abnormalities such as osteogenesis imper-
and weakness with a normal mental state. non-cardiogenic pulmonary oedema and fecta is unclear.9
The major problem is body water depletion; disseminated intravascular coagulation. Serotonin syndrome, neuroleptic malig-
however, in some paediatric patients (e.g. See Table 22.3.2 for comparison of signs nant syndrome (NMS) and anticholinergic
cystic fibrosis where greater amounts of and symptoms of heat-induced syndromes. syndrome: Serotonin syndrome is the clinical
syndrome seen in the setting of excessive
serotonin neurotransmission as a result of
Table 22.3.2 Comparative table of the signs and symptoms of heat-induced syndromes
the ingestion of serotonergic agents. The
Condition Symptoms Signs triad of clinical features of the serotonin
Heat syncope Dizziness, feeling faint, may have brief Pallor, sweating, moist and cool skin, syndrome can be grouped under the head-
loss of consciousness normal body temperature ings of: central nervous system (CNS), auto-
Heat cramps Painful, brief muscle cramps during or Spasm of affected muscle group, body nomic and neuromuscular. NMS is a rare but
after strenuous exercise temperature usually normal potentially lethal syndrome, the exact
Heat exhaustion Vomiting, headache, lethargy, weakness, Signs of dehydration, tachycardia, aetiology of which is unclear. It is seen in
normal mental state orthostatic hypotension, core children and adolescents taking antipsy-
temperature <40
C
chotic medication. It has been suggested
Heat stroke Episode of loss of consciousness Core temperature >40.5
C, acute that the duration of NMS was one-third
common, neurological symptoms, change in mental state, sweating often
irritability, confusion present if exertional heat stroke shorter in children taking atypical anti-
psychotics when compared with older, more

460
22.3 HEAT-INDUCED ILLNESS
22
typical agents.12 Methylphenidate (Ritalin)

ENVIRONMENTAL
intravenous) and rest in a cool environment. drug combinations are used, whereas
has been implicated in NMS. The anticho- The decision to admit will depend on a NMS is most commonly an idiosyncratic
linergic syndrome is usually seen in the number of factors. reaction to a single agent. In patients with
setting of deliberate self-poisoning with the serotonin syndrome, most resolve
potent anticholinergic agents.13 Clinically Heat stroke within 2448 hours with supportive man-
there are both central and peripheral fea- Heat stroke in children is a medical emer- agement and stopping the serotonergic
tures. The latter are an extension of the gency. The ABC approach is important and agent/s. In severe cases, attention to
physiological effects of blocking cholinergic all children with heat stroke should be ABCs, continuous core temperature moni-
receptors. triaged to a resuscitation environment. toring and titrated intravenous benzodiaze-
Drug-related: amphetamine use (e.g. Rapid external cooling is the next immediate pines are often necessary.13,16 Specific
ecstasy at rave parties) and other drugs goal. Evaporative methods are probably serotonin antagonists have also been
(e.g. salicylates, anticholinergics) can cause best. Initial cooling should aim for a core used such as cyproheptadine (0.1 mg kg1
heat-related emergencies in the adolescent temperature of 38.5
C. Removing the childs per dose) or chlorpromazine (0.25
group. Vigorous activity, high ambient clothes, spraying with water and fanning 1.0 mg kg1 per dose). In NMS, benzodiaze-
temperatures and ingestion of amphet- (wet and windy approach) and applying pines are useful in mild cases. In more
amine compounds contribute to the risk of ice packs to the neck, groin and axillae are severe cases, bromocriptine and dantrolene
hyperthermia at rave parties.14 all indicated. It is important to prevent shiv- may have a role. The treatment of the anti-
ering, which can generate heat, by the cholinergic syndrome is similar. Physostig-
administration of diazepam or chlorproma- mine (0.02 mg kg1 per dose up to a
Investigations zine. Antipyretics such as paracetamol or maximum of 0.1 mg kg1) is a centrally act-
ibuprofen have no role. ing acetylcholinesterase inhibitor. Its exact
Heat-related illness in children is diagnosed role is controversial, but one review has
All children with genuine heat stroke
on clinical criteria and rectal temperature. suggested that in confirmed cases of
require intravenous fluids, an indwelling
Investigations are directed at the complica- anticholinergic poisoning in children, phy-
urinary catheter and admission to the in-
tions of heat-related illness in the paediatric sostigmine might be more effective than
tensive care unit. Standard treatment of
patient.
shock, non-cardiogenic pulmonary oedema benzodiazepines.17
Children who present with heat syncope
and cerebral oedema, disseminated intra-
or heat cramps usually do not require any
vascular coagulation and renal/hepatic
investigations. Those children with more
failure should be given, should these life-
severe heat-related illness, however, re-
quire further work up. In heat exhaustion,
threatening complications occur. Prognosis and
basic bloods including full blood exami- disposition
nation, urea and electrolytes, as well as Malignant hyperthermia Apart from children with mild heat-related
serum creatinine kinase and glucose The principles of treatment of malignant illness (i.e. heat syncope, heat cramps and
should be performed. Urinalysis would also hyperthermia are similar to those of heat mild heat exhaustion), who can be managed
be indicated. stroke. The antidote is dantrolene which in the emergency department, all others
The paediatric patient with heat stroke or inhibits the release of calcium into muscles. should be admitted to hospital. Any child
other life-threatening hyperpyrexial illness Several regimes have been suggested. with suspected heat stroke should be admit-
requires full investigation: full blood examina- Shann suggests an initial dose of 1 mg ted to intensive care.
tion, electrolytes, liver function tests, total cre- kg1 min1 until improvement, with a max- Prognosis in heat-related illness is related
atine kinase, coagulation studies, urinalysis, imum dose of 10 mg kg1 total.15 Another both to the absolute temperature level and
arterial blood gases and 12-lead electro- regime suggests giving 2.5 mg kg1 intrave- the duration of the elevation.18 Persisting
cardiogram. nously and repeating every 10 minutes to a coma and multiorgan failure are poor prog-
maximum of 10 mg kg1.9 If the MH has nostic signs. As prognosis correlates with
been caused by an inhalational anaesthetic, duration of hyperthermia, immediate cool-
the agent should be stopped immediately. ing is critical. The mortality rate for heat
Management Other manoeuvres include hyperventilation stroke is approximately 10% and malignant
Heat syncope and heat cramps with 100% oxygen, bicarbonate in fulmi- hyperthermia, 7%.
For children with heat syncope and heat nant cases (24 mEq kg1), lidocaine to Prevention, particularly in babies and
cramps, external cooling and oral fluids treat arrhythmias and aggressive cooling infants, centres around educating new par-
(e.g. gastrolyte) is usually sufficient. These of the child. ents about the risks of sun exposure, cloth-
patients usually do not require admission. ing and leaving children in cars in hot
Serotonin syndrome, NMS and weather even for brief periods. Sports medi-
Heat exhaustion anticholinergic syndrome cine guidelines regarding older children tak-
The child with heat exhaustion needs These conditions are rare in children. The ing part in sporting activities in hot, humid
fluids (oral if able to tolerate, otherwise serotonin syndrome is usually seen when weather need to be in place.

461
22.4 COLD INJURIES

10. Neuromuscular disorders. In: Nelson Textbook of

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7. Hewson P, Oberklaid F. Recognition of serious illness in
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18. Rogers IR, Williams A. Heat-related illness. In: Cameron P,
susceptibility of infants and young children Malignant hyperthermia and other pharmacogenetic Jelinek G, Kelly A-M, Murray L, Heyworth J, editors.
disorders. In: Barash PG, Cullen BF, Stoelting RK, editors. Textbook of Adult Emergency Medicine. Edinburgh:
to heat-related illness compared to adults. Clinical Anaesthesia Textbook. Baltimore: Lippincott Churchill Livingstone; 2000. p. 60710.
Williams & Wilkins; 2001 [chapter 20].

22.4 Cold injuries

Simon Chu Nicholas Cheng

common problem potentially complicating

ESSENTIALS any other severe illness (especially trauma)
and must be prevented.2
1 Hypothermia is defined as a core body temperature less than 35
C.
2 Cold injury is uncommon in Australia, but when cold injuries do occur they are more Normal physiology: a review
significant in children than in adults.
Heat production is derived from basal
3 The major complications of hypothermia are altered mentation and arrhythmias. metabolism, digestion, and muscular activ-
4 Hypothermia is treated with passive external, active external, active internal, ity, which may be voluntary (exercise) or
and/or extracorporeal rewarming, depending on severity. In addition, hypothermia involuntary (shivering). Emotional factors
should be rigorously prevented in any patient with a severe illness. and hormonal fluctuations influence heat
production. The main mechanisms by which
5 The major complications of rewarming are an after-drop in temperature and the body compensates for low core body
vasodilatory shock. temperature are by increasing its metabolic
6 Severe local cold injuries are treated with rapid immersion rewarming, then care rate, primarily through shivering, and by
given as for burns. shunting blood away from non-essential
organs to preserve vital organs. The capacity
7 In Australia patients are almost always dead, then cold. Situations where patients to shiver is dependent on local glycogen
become cold enough to apply warm and dead criteria are very rare. stores and the rate of change of core and
external temperature.1,3
Neonates are the patients most prone to
Introduction underdeveloped behaviour responses to hypothermia. They are unable to shiver and
cope with extreme cold (young have limited stores of energy. Because of
Cold injury is uncommon in Australia due children cant put on more clothes). this, newborn children utilise catabolism of
to its warm climates. However, its signifi- When cold injuries do occur, they can be brown fat to generate heat. This is an ineffi-
cance is more important in children, as they subdivided into generalised injury (namely cient process that consumes oxygen, thus
have: accidental or environmental hypothermia) exacerbating hypoxia. In addition, the large
a larger surface area:weight ratio;1 and localised injury. Cold injury is also a surface area to weight ratio, due to a

462
22.4 COLD INJURIES
22

ENVIRONMENTAL
relatively large head, contributes to heat Evaporation from the skin accounts for of this pathophysiology comes from con-
loss. At birth, neonates are covered in amni- only 7% of heat loss at rest. This may be trolled hypothermia in cardiac surgery. Note
otic fluid, and evaporative losses are signifi- increased in cold, dry conditions and by that there is a huge variation of the onset of
cant. An overhead radiant heater is not sweating. Evaporation from the respiratory certain clinical signs based on temperature
adequate to compensate for this evapora- tract removes another 7%. This can increase level. For instance, some patients may
tive loss.4,5 by breathing faster (such as at high altitude exhibit confusion at higher temperatures
Heat loss from the human body is by four or during exercise). compared with others. Note that in children
methods: Temperature is perceived through central clinical manifestations of altered conscious-
and peripheral mechanisms. Heat sensors in ness may be subtle.
radiation;
the central hypothalamus receive input from Note that only during severe hypothermia
conduction;
the skin, central arteries, and viscera. It is does protection from hypoxia occur, due to
convection;
this central thermostat that is reset, which decreased demand for oxygen by tissues,
evaporation.1
causes fever. Skin receptors respond to and even then only at extremely low tem-
Radiation occurs when heat energy leaves a change in skin temperature but do not peratures (patients <20 C can tolerate
the skin at the speed of light. Patients with themselves indicate the patients core tem- anoxia for up to 60 minutes). Metabolic pro-
more fat become more hypothermic than perature. A result of all this input is that cesses slow by approximately 6% for each
thinner patients, due to the formers larger the body responds by those autonomic 1 C drop in body temperature.1 Thus at
surface area for radiation heat loss. In chil- reflexes listed below to increase or decrease 28 C the basal metabolic rate is about
dren, who have a higher surface area to core body temperature.3 50% of normal. This leads to hypoventila-
weight ratio, it accounts for up to 50% of tion and hypoxia. However, at this tempera-
all heat loss; indeed, up to 75% in neonates. ture the decreased cellular metabolism
This higher number in neonates is due to a affords some protection against hypoxia.
proportionally larger head increasing the
Hypothermia Cold diuresis is an initial brisk diuresis; this
surface area:weight ratio.1,4 Radiation This is defined as a core temperature of is due to decreased tubular reabsorption and
losses decrease when a patient is clothed. <35 C.1 Hypothermia is classified on the also a decreased production of antidiuretic
Conduction of heat is poor in air and basis of severity. The reason for this classifi- hormone. There is also an increased central
therefore does not contribute much to hypo- cation is that it influences the rewarming blood circulating volume as blood is shunted
thermia in normal circumstances. However, mechanisms that are most often deployed. away from the periphery, thus presenting
water-conductive heat loss is 24 times more It is also related to the physiological ability the kidneys with an apparent increased
than that of air. It is this method by which of the patient to compensate for hypother- blood volume for filtration.1
patients suffering from water immersion mia. An easy way to remember these tem-
become profoundly hypothermic, and how perature ranges is:
patients in wet clothes become hypothermic
quickly. The surface on which the patient is
acceptable low temperature: 2 C below History
37 C (>35 C); Important points in history are:
lying also contributes to conduction heat
true mild hypothermia: 3 C below that
loss. For instance, a patient lying on snow
(3235 C);
approximate time of exposure, if known;
is likely to become more hypothermic than
moderate hypothermia: 4 C below that
environment in which patient was found;

a person lying on sand.
(2832 C);
resuscitation at the scene, including
Convection occurs as warm air next to the duration of time with no cardiac output
skin is replaced by cool air. This can contrib-
severe hypothermia: anything below that
(downtime);
(<28 C).
ute to 25% of total body heat loss in still air. pre-existent illnesses (e.g. thyroid
In a wind of 63 km hr1, this increases by 14 Tables 22.4.1 and 22.4.2 show the main disease);
times. This is described as the wind chill consequences of hypothermia at a given drugs, medications, allergies, and
factor. temperature.1,3,6 Much of our understanding immunisation status.

Table 22.4.1 Compensatory mechanisms at different severities of hypothermia

Mild (3235 C) Moderate (2832 C) Severe (<28 C)

Increased basal metabolic rate by shivering Limits of increasing basal metabolic rate reached Complete loss of thermoregulation
Vasoconstriction peripherally, leading to fluid shift Shivering stops Stupor, coma
Mild tachycardia Decreasing cerebral blood flow, causing delirium and Pulseless
Cold diuresis (see text) gradual decreased level of consciousness Fixed dilated pupils
Apathy, ataxia, amnesia, dysarthria Decreased rate of neural impulse transmission, Absent reflexes
causing clumsiness and numbness Dysrhythmias, initially slow atrial fibrillation then
Muscle rigidity due to increasing acidosis ventricular fibrillation
Appearance of death at < 25 C with asystole
Falling blood pressure
See text for more specific changes

463
22.4 COLD INJURIES

wave, interval (PR, QRS, QT) prolongation,

Table 22.4.2 Findings at low body temperatures
atrial dysrhythmias, and ventricular dys-
Temperature ( C) Findings rhythmias.10 Other less well-known changes
27 Reflexes absent, no response to pain, comatose include abnormalities similar to myocardial
infarction. Hypothermia can also blunt the
25 Cerebral blood flow one-third of normal, cardiac output one-half of normal
ECG changes with hyperkalaemia.6 Note
23 No corneal reflex, ventricular fibrillation risk is maximal that all these are not present in all hypother-
19 Asystole, flat EEG mic patients.
Once temperature is measured, the
15 Lowest temperature survived from accidental hypothermia
severity of hypothermia determines the
methods of rewarming. Specific methods of
Examination precipitate arrhythmias, particularly ventric- rewarming are classically divided into four
Full primary and secondary assessment of ular fibrillation.6 While this is occurring, one categories:6,8,11
patient. should attend to the patients airway, breath-
passive external rewarming;
Accurate measurement of core body ing, and circulation, as per any resuscitation.
active external rewarming;
temperature. Active rewarming should be avoided until
active internal (core) rewarming;
the patient reaches the emergency depart-
extracorporeal rewarming.
ment. This is because of the complications of
Diagnosis
rewarming, namely after-drop and shock.1,6,8
This requires only two essentials:1 Passive external rewarming These are
methods used to prevent excessive endoge-
a thermometer able to record low core
Treatment in the emergency nous heat loss and promote the patient to
temperatures accurately;
department self-warm to normal core temperature. They
a high index of suspicion.
Once in the emergency department, the include placing the patient in a warm room
Core temperatures can be measured best to prevent excessive convection, evaporation,
patient should be triaged to an appropriate
with oesophageal or rectal probes. The most and conduction heat losses. Sheets of foil (foil
area, which is warm. In the very young, a
direct method of measurement is with a car- space blankets) are placed over the patient to
radiant warmer bed and heating lamps
diac catheter such as a SwanGanz, but this decrease loss of heat through radiation.
should be available when the patient
is impractical in the emergency setting. Rec-
presents.4,9 Patients should have their air-
tal probes are often used,7 but care must be Active external rewarming These are
way, breathing, and circulation reassessed
taken when using these. The probe must be methods used to transfer heat energy to
and appropriate resuscitation commenced.
at least 10 cm into the rectum in older chil- the patient from the external environment.
Appropriate monitoring should be insti-
dren (more than 8 years old) and 5 cm in They include:
tuted, including electrocardiogram (ECG),
younger children. Inaccuracies may occur
and core temperature, either by rectal
due to the presence of faecal material,1 warmed blankets;
or oesophageal means. Oxygen saturation
and the probe must be left in until the tem- chemical hot packs and warm water
monitoring should be attempted, whilst bottles;
perature equilibrates. Tympanic measure-
understanding that initial vasoconstriction
ments are well known to be unreliable in forced warm air blankets;
will give inadequate readings. Urine output
the very young,7 but they are a good indica- radiant heaters and lights;
should be monitored. Gentle handling
tor of therapy progression in the older child. warm-water body immersion.
should be continued to avoid precipitation
Oral and axillary temperature probes are
of arrhythmias.6 Patients should continue
unreliable and impractical in the setting of Active internal rewarming These are
100% oxygen on arrival in emergency.
true hypothermia. methods used to transfer heat energy to
Blood tests taken should include arterial
the patient that deliver heat internally. They
blood gas (ABG); full blood count; electrolyte,
can be subdivided into simple methods (the
Treatment urea, creatinine (EUC); liver function tests;
first two methods below) and invasive meth-
Pre-hospital treatment amylase; comprehensive metabolic panel
ods. They include:
This is mainly the realm of passive external (CMP); glucose; thyroid function tests (TFTs);
rewarming methods (see below). Patients coagulations; tests for infection; and, if sus- warmed intravenous (IV) fluids (normal
should be carefully removed from the precip- pected, a screen for sedative drugs and etha- saline 40 C);
itant cold environment to a dry, sheltered nol. Hypothermia causes measured pH to fall inhalation of humidified warm oxygen
area. If clothes are wet, they should be and pO2 and pCO2 to be higher. It is recom- (40 C);
removed, and the patient dried and covered mended that these ABG values should not gastric lavage;
with a warm dry blanket. All patients should be corrected for temperature to better reflect bladder lavage;
be gently handled, especially during trans- the physiological state of the patient.6 colonic lavage;
port, as there is evidence that sudden move- A 12-lead ECG should be taken. Classic pleural and peritoneal lavage
ments to a body in severe hypothermia can changes include the presence of a J (Osborn) (1020 mL kg1 of 40 C saline).

464
22.4 COLD INJURIES
22

ENVIRONMENTAL
Extracorporeal rewarming These are done (see Controversies). Note that, accord- resuscitation guidelines of their country
methods by which the patients blood is ing to criteria for diagnosing brain death which were released following the latest
removed, rewarmed outside the body, and as quoted from the Australian and New ILCOR guidelines released in late 2010.
replaced. They include: Zealand Intensive Care Society,13 the
patient must have a core temperature above Complications
haemodialysis; 35 C, whereas other sources say 32 C.14 Complications can be classified as due to
venovenous transfer of blood; The decision of who to rewarm continues the hypothermia itself and complications
extracorporeal (coronary bypass) to evolve. There is a case report of a 26- as a result of rewarming.
circulation.
month-old patient with a core temperature Complications from hypothermia in-
Due to the childs larger surface area to of 15 C who, after rewarming, recovered clude:1,3
weight ratio,1,5 emergency physicians neurologically intact.15 For patients without
should start to institute limited active exter- submersion, extreme duration of exposure is
cardiac: arrhythmia;
nal rewarming methods, such as radiant not incompatible with life. In immersion
haematological: platelet dysfunction,
thrombocytopenia and disseminated
light warmers and forced warm air blankets, patients, successful recovery is very rarely
intravascular coagulopathy;
even in mild hypothermia.11 If the child is seen unless patients are immersed in ice-
unable to produce extra heat, then the insti- cold water (< 10 C) for long periods of time
pulmonary: pneumonia, pulmonary
oedema and (adult) acute respiratory
tution of simple active internal rewarming (see Chapter 22.2). However, further
distress syndrome;
methods (warm humidified oxygen and attempts to resuscitate after failure to
warmed IV fluids) is appropriate. restore a circulating cardiac rhythm within
infection: immune complex suppression;
In older children, asking them to drink 30 minutes of rewarming to above 32 C
renal: acute tubular necrosis and
rhabdomyolysis;
some warm liquids (hot chocolate or soup) are likely to be ineffective.14 In most Austra-
will in effect give them the effects of a warm lian cases, however, the adage youre not
neurological: cerebral oedema, prolonged
coma and slow neurological recovery (up
gastric lavage. However, patients should dead till youre warm and dead does not
to 6 months);
only have warmed fluids if they are fully con- apply. The above only relates to patients
scious, can protect their airway, and have no that are snap frozen in snowy weather.
gastrointestinal: pancreatitis;
evidence of gastroparesis. With the recent evidence of inducing
biochemical and metabolic
derangements (including glucose, sodium
Moderate hypothermia (2832 C) should hypothermia for out-of-hospital arrests, it
and potassium).
have all active external rewarming techni- would make sense for patients who present
ques instituted except immersion in warm hypothermic due to out-of-hospital arrests There are two main complications that
bath therapy, which is limited to localised to be warmed to 34 C. In this way, hypother- result directly from the rewarming of
cold injury in the emergency setting (see mia has been treated, whilst giving the patients: after-drop and shock.1,6,8 After-
frostbite below). It is almost impossible to patient admitted to the paediatric intensive drop is a drop in core temperature after
adequately monitor patients while they care unit an opportunity to recover with the rewarming therapies have commenced.
are in an immersion bath. Forced air warm- best possible neurological outcome.16 There are two proposed mechanisms for
ing blankets can increase core temperature after-drop:
by up to 1.5 C hr1. Patients should also Neonatal resuscitation
have warmed IV fluids and heated humi-
Cold peripheral blood re-enters the
In neonatal resuscitation, a warm ambient
circulation once peripheral
dified oxygen for inhalation. Together, environment for the newborn child is
vasodilatation occurs with rewarming.
they can increase core temperature by prepared. A heated room with an overhead
12 C hr1. Normal saline bags can be warmer is required. Warmed towels and blan-
After-drop is due to ongoing conduction
of heat from the warmer core into colder
safely warmed in a microwave oven. The kets are used to rapidly dry the newborn to
peripheries and surface layers of the body.
optimum operating system for warming prevent evaporative heat loss. Unwell neo-
500-mL bags of crystalloid is 400-W micro- nates should be admitted to special care In reality, after-drop is most probably a com-
wave for 100 seconds or 800-W microwave nurseries or neonatal intensive care within bination of the two mechanisms described.
for 50 seconds.12 Alternatively, if a heat humidicribs or transport cribs with radiant This can be minimised by ensuring that
infusion pump is available, this should be heat. If ventilated, humidified warm gases rewarming only occurs over the core of the
used. Intravenous infusion tubing should should be used.4,5 body, while the peripheries of the body are
be as short as possible, as longer tubing There is now increasing evidence that in not actively rewarmed. For instance, a forced
loses more heat to the atmosphere. neonates suffering hypoxic ischaemic en- warm air blanket is placed over the patients
Severe hypothermia requires institution cephalopathy a period of hypothermia may body, but their hands and feet are left out-
of invasive core-rewarming techniques. All be beneficial (see Controversies). Some side the blanket.
can raise core temperature by 2 C every 5 centres in Australia have already changed Shock occurs from the same mechanism,
minutes.6 In cardiac arrest, cardiopulmonary their practice to cool such neonates. The where peripheral vasodilatation increases
resuscitation (CPR) should be commenced reader is advised to consult local guidelines the intravascular space to be filled, causing
until core temperature has reached 35 C, and their referring neonatal intensive care a consequent drop in blood pressure and rise
and then a further assessment of the patient unit for more information, and the latest in heart rate. In addition, the cold diuresis

465
22.4 COLD INJURIES

experienced has already decreased the circu- of 1812. It is due to prolonged exposure to anaesthetic limb, loss of fine motor function,
lating blood volume (up to 35%). In immer- wet and cold, most common in the feet when loss of gross motor function, and finally
sion patients, there is also a hydrostatic poorly ventilated cold shoes are worn. There severe joint pain. Examination of the
squeeze effect, which further decreases is peripheral neurovascular damage, but no affected limb will reveal varying degrees of
blood volume. Rewarming should therefore ice crystal formation within tissues. frostbite.18 In the past, they have been clas-
occur only once vascular access is estab- Perniosis or chilblain is injury due to sified similarly to burns, from first-degree to
lished and warmed normal saline is insti- repeated exposure to dry cold. Bullae form fourth-degree injury. It is much easier to clas-
tuted if active rewarming occurs, starting 12 hours post injury. These bullae burst to sify them as superficial or deep.8 Superficial
with usual shock doses of 20 mL kg1. Inser- form ulcers, which are painful and pruritic. injuries go only to the skin and subcutane-
tion of a central venous pressure (CVP) line It is possibly due to repeated vasoconstric- ous tissues, whereas deep frostbite also
would be useful; however, this needs to be tion. Common areas involved are the feet, affects bones, joints, and tendons.
balanced against the increased rough lower legs and face. Most investigations are unhelpful. How-
handling of the body during insertion. Panniculitis consists of red lesions due to ever, a full blood work up with full blood
Medications in hypothermia behave cold. Treatment is with non-steroidal anti- count, EUC, liver function tests, glucose,
unpredictably. Metabolism of most drugs inflammatory drugs (NSAIDs), and the and creatine kinase to look for rhabdomyol-
will be slowed due to hypothermia. Some lesions resolve in 1021 days. ysis should be done. Urinary myoglobin
drugs have decreased effectiveness; others, should be checked. Imaging is unhelpful
such as morphine, have increased effects. initially; however, there is some evidence
Drugs not used in treatment include sodium that a bone scan will help surgeons later
bicarbonate, insulin, corticosteroids, empiri-
Frostbite determine how much limb is still viable,
cal antibiotics, and ethanol (contrary to pop- Frostbite is the most dangerous of the local and whether superimposed osteomyelitis
ular belief).3,6 cold injuries. It is caused by freezing and ice has developed.8,18,19
Electrical defibrillation and antiarrhyth- crystal formation within the interstitial and
mics may be administered at any tempera- cellular spaces due to prolonged exposure
ture, but most efforts do not succeed until to freezing temperatures. It tends to occur Treatment
the temperature reaches above 2830 C.3 more if the skin is directly exposed to tem- Premedical treatment involves preventing
peratures less than 10 C. Several patho- further hypothermia and initiating resuscita-
Disposition genic phases evolve, called the frostbite tion. As for hypothermia, removing the
A patient with mild hypothermia, once trea- injury cascade:8,18 patient from the cold environment is para-
mount. Rubbing the limb to try to warm it
ted, may be observed in the emergency Prefreeze phase. Superficial tissue
should be avoided, as this increases tissue
department for a few hours before discharge cooling occurs, which leads to increased
if the patient is well. Moderate and severe damage.8,18
blood viscosity, microvascular
hypothermia are an indication for ward or Once in emergency, the mainstay of treat-
constriction, and endothelial plasma
intensive care unit admission, depending on ment is rapid immersion rewarming.8,18 The
leakage.
affected limb should be placed in a whirlpool
if cardiac abnormalities are present during Freeze phase. Ice crystals form in the
of water about 40 C for 2040 minutes for
assessment. Discharge should include educa- extracellular space, leading to disruption
tion to patients on prevention of further superficial frostbite, 1 hour for deep frost-
of endothelium, disruption of cell
occurrences, for example the proper use of bite. This procedure will be painful, some-
anatomy, and hyperosmolality within
clothing, checking weather reports, etc.1 times exceedingly so, hence narcotic and
cells due to crystals osmotically drawing
NSAID analgesia should be started before
water out of cells. This leads to protein
treatment is commenced. Rewarming is
denaturation and DNA synthesis
complete when the distal area of the limb
Localised cold injuries inhibition.
is flushed, soft, and pliable. The patient
Frostbite is the most severe of the injuries,
 Vascular stasis. There is arteriovenous
should start to move the limb during
shunting within damaged tissue, leading
but others include chilblain (perniosis), rewarming to encourage blood flow in the
to stasis coagulopathy and thrombus
cold-induced fat necrosis (panniculitis), frost limb. The main reason for suboptimal results
formation.
nip, and trench foot.17,18 is premature cessation of rewarming. After
Frost nip is transient blanching and numb-
Late progressive ischaemic phase. The
rewarming, the limb is dried and placed in
thrombus induces inflammation, distal
ness of peripheries that resolves with a splint and elevated, and dressings applied
hypoxia, and anaerobic metabolism,
rewarming. Only the skin surface is dam- four times a day.
which eventually leads to tissue necrosis.
aged. No ice crystals form within tissues, Blistering of the limb will occur. The blis-
as opposed to frostbite (see below). ters are usually clear in superficial frostbite,
Trench foot, also known as immersion Clinical features and diagnosis and haemorrhagic blisters in deep frostbite.
foot, is historically the most interesting, with Symptomatically, frostbite begins as an ini- Controversy exists as to whether to aspirate
the most famous cases occurring at Napo- tial coldness of the skin. It then progresses these blisters. Haemorrhagic blisters should
leons failed invasion of Russia in the winter to a stinging or burning pain, then to an not be aspirated, as this increases trauma to

466
22.4 COLD INJURIES
22

ENVIRONMENTAL
the wound. The treatment of clear blisters is Once the hypothermia is treated, then
general consensus is that, to promote
not as well defined. non-environmental causes should be investi-
brain perfusion, CPR should be continued
All patients should have their tetanus sta- gated and, if found, appropriate treatment
till the core body temperature reaches
tus updated and, as 30% of wounds become instituted. Iatrogenic causes need to be pre-
35 C.13
infected, IV prophylactic antibiotics may be vented. Always prevent hypothermia in
of use. IV penicillin G is most commonly trauma patients after full assessment, as  Continuing CPR in the absence of
used.18 hypothermia worsens the diagnosis. cardiac output in a hypothermic patient
has traditionally been mandatory.
Disposition However, as survival occurs only in
All patients should be admitted under a Controversies patients who have been immersed in
specialised (burns) unit.8,18 Patients are In certain countries, active water <5 C prior to cardiac arrest,1 it is
observed for up to 6 weeks, which is usually rewarming is commenced prior to arrival more likely that patients arrest in
the time the gangrenous parts of the limb in the emergency department.8 This is Australia before they become
are fully delineated so that safe amputation, due to well-organised emergency hypothermic. Therefore when to stop
if necessary, will occur. Good prognostic fac- medical systems catering for such cardiac resuscitation is an issue.
tors are patients with superficial injuries emergencies, like in Canada. In Australia, The use of hypothermia in treatment,
only, clear blisters, and sensation still pres- this is more of a contentious issue, as we particularly in trauma and head injury,
ent after rewarming.18 dont see hypothermia as often. has always been of some debate.11
Controversy exists regarding CPR Hypothermia causes a reduction in
causing arrhythmias when a patient oxygen consumption and is theoretically
Hypothermia not due to cerebroprotective. This contradicts the
loses spontaneous circulation.1 However,
environmental causes the evidence that CPR causes ventricular results that trauma patients presenting
There are many other causes of hypother- fibrillation in hypothermic patients is at with hypothermia have a worse
mia,1,3 as seen in Table 22.4.3. best circumstantial, and therefore the prognosis.2

In neonates with hypoxicischaemic

Table 22.4.3 Hypothermia not due to environmental causes
injury, cooling of the head is now
accepted in many centres as best-
Cause Details practice treatment, ahead of changes in
Metabolic or endocrine Hypoglycaemia the ILCOR guidelines. However, the
Diabetic ketoacidosis
Hypopituitarism
means to do so is not fully determined.
Hypothyroidism This is an area in which there are rapid
Addisons disease
Uraemia
developments and the recent ILCOR
Malnutrition guidelines in 2010 are likely to reflect
Toxicological Alcohol
this new evidence.
Barbiturates
Anaesthetic agents
Carbon monoxide
Cyclic antidepressants
Narcotics
References
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(5):6719.
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10. Mattu A, Brady WJ, Perron AD. Electrocardiographic Cardiac arrest in special circumstances. Resuscitation 1994, 55-68. Online. Available http://www.vnh.org/
manifestations of hypothermia. Am J Emerg Med 2005;67S1:S135S170. MilitaryDerm/Ch14.pdf 11 Sep 2003 a comprehensive
2002;20(4):31426. 17. Herrin J, Antoon A. Cold injuries. In: Behrman R, text on military dermatology, part of a large tome on
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thermal safety of microwave warming of crystalloid fluids. Jan 2003. Available from http://www.emedicine.com/ treatment. Available from http://www.hypothermia.org;
Anaesthesia 2000;55(3):2514. ped/topic803.htm; [accessed 27.10.10]. 2003 [accessed 27.10.10] an excellent web site with up-
13. Australian and New Zealand Intensive Care Society. 19. Cauchy E, Chetaille E, Lefevre M, et al. The role of to-date insight on hypothermia and future directions in
Recommendations on brain death and organ donation. bone scanning in severe frostbite of the extremities: management.
3rd ed. Melbourne: Australian and New Zealand A retrospective study of 88 cases. Eur J Nucl Med Meteorological Service of Canada. Wind chill charts and
Intensive Care Society; 1998. Available from http:// 2000;27(5):497502. tables. Ottawa: Meteorological Service of Canada; 2002.
www.anzics.com.au/downloads/cat_view/12-death- Online. Available: http://www.msc.ec.gc.ca/education/
and-organ-donation [accessed 27.10.10]. windchill/charts_tables_e.cfm 11 Sep 2003 an excellent
14. Wijdicks EFM. The diagnosis of brain death. N Engl J Med article with excellent graphical depictions of wind chill
2001;344:121521. effects; the reader should also peruse the rest of the
15. Kelly K, Glaeser P, Rice T, et al. Profound accidental website.
hypothermia and freeze injury of the extremities in a
Further reading
child. Crit Care Med 1990;18(6):67980. Adnot J, Lewis CW. Immersion foot syndromes. In: James WD,
16. Soar J, Deakin CD, Nolan JP, et al. European Resuscitation editor. Textbook of Military Medicine: Military Dermatology.
Council Guidelines for Resuscitation 2005. Section 7. Washington: United States Government Printing Office;

22.5 Anaphylaxis
Andrew Stewart Kemp

ESSENTIALS Pathophysiology
1 Adrenaline (epinephrine) is the treatment of choice and should be administered if Anaphylaxis is due to mast cell mediator
there are any respiratory symptoms. release. Those mediators with vasoactive
and/or bronchoconstrictive activities (his-
2 Biphasic reactions occur, with relapse 410 hours after initial successful tamine and the sulfidopeptide leukotrienes)
treatment. are principal factors in the development
3 A full history should be obtained with the aim of identifying the triggering factor. of anaphylaxis. An increase in vascular
permeability causes loss of fluid from the
4 If repeated exposure is considered possible, consider provision of an anaphylaxis circulation into the interstitial space. The
action plan and instruction in use of adrenaline injector. relative contributions of the mediators to
5 An allergy referral is indicated for definitive diagnosis of precipitating factors. the various clinical features are not
completely defined. Mediators may directly
compromise cardiac muscle function, and
the intravascular volume depletion and
4-fold rise in anaphylaxis hospital admissions haemoconcentration further compromises
Introduction over an 11-year period from 1994.2,3 The the cardiac output. Secondary cardiac
Acute allergic reactions resulting from the most dramatic rise was reported in children arrhythmias can occur. Both upper and
degranulation of mast cells present as a less than 5 years of age, with an almost 7-fold lower airways are affected. Laryngeal
continuum of responses from mild cutane- increase in hospital admissions from anaphy- oedema results in a variable degree of
ous erythema and urticaria to severe laxis. The increasing reactions are predomi- upper airway obstruction, and broncho-
hypotension, collapse, and death. Differ- nately due to foods. Despite the increase spasm and increased mucus secretion com-
ent authorities include varying compo- in admissions, death from anaphylaxis in promise the lower airways. Bronchospasm is
nents of this continuum in the definition childhood remains rare (the Australian more marked in asthmatic patients or in
of anaphylaxis.1 Anaphylaxis may be mortality rate has remained stable at 1 per those taking beta-blockers.
defined as a severe acute allergic reaction million population per year over an 11-year
that involves the respiratory tract and/or time period).3 Thus there is a paradox
results in circulatory compromise with that although anaphylaxis admissions are
hypotension. increasing, particularly in children less than
5 years of age, death from anaphylaxis
Aetiology
Along with the increasing incidence of
allergic diseases, anaphylaxis admissions to remains rare, with the majority of deaths The triggering agent can be identified in
Australian, USA and UK hospitals have occurring in teenage or adult years rather about three-quarters of patients presenting
increased. Australian studies reported a 2- to than in early childhood. to emergency departments.4

468
22.5 ANAPHYLAXIS
22
In one childhood series the triggers were:5

ENVIRONMENTAL
Table 22.5.1 lists the frequency of the pre- 10 minutes and decline rapidly to baseline
senting symptoms and signs in children by 1560 minutes. Tryptase should be col-
food: 50%;
admitted to hospital for anaphylaxis. lected not later than 6 hours after the initial
medications: 25%;
Biphasic anaphylactic reactions are defined reaction. Peak levels of beta-tryptase occur
insect bites: 10%;
as worsening of symptoms, requiring new at 12 hours and decline with a half-life of
immunotherapy: 1%;
therapy, after the resolution of anaphylaxis, approximately 2 hours. If blood is collected
immunisations: 1%.
and occur in 320% of anaphylactic presen- in the initial 30 minutes after a reaction,
In a more recent series, food anaphylaxis tations.5,6 The reaction usually occurs 410 tryptase elevation may not be detected.
comprised 85% of presentations to a hours after the initial event; however, it has Tryptase is stable and can be identified in
tertiary paediatric hospital emergency been described up to 48 hours later. Biphasic plasma or serum stored at room tempera-
department.6 reactions are not accurately predicted from ture for several days. Interpretation of tryp-
the initial clinical features. The more severe tase levels is often difficult and is improved if
the initial anaphylactic event and/or its inad- baseline levels are available; however, in the
Clinical features equate treatment with adrenaline, the more majority of cases this is unlikely to be pres-
Symptoms occur along a continuum, from likely a biphasic reaction will occur.6 ent. Comparison with a baseline may be
reactions that are primarily cutaneous in helpful in cases with recurrent presentation
nature, through mild to moderate anaphy- where the diagnosis is uncertain.
lactic reactions that may have respiratory The reliability of measuring mast cell tryp-
symptoms but without tachypnoea or hypo-
Investigations tase post mortem has been questioned,
tension, to severe life-threatening anaphy- Anaphylaxis is a clinical diagnosis, and inves- because elevation of tryptase may be seen
laxis with hypotension and hypoxia. tigations do not have a role in the acute man- in control cases where death has occurred
In children, cutaneous (90% of cases) and agement. On occasions, it may be difficult to from other causes. Constitutionally raised
respiratory (80% of cases) manifestations differentiate anaphylaxis from other cardiac, levels of mast cell tryptase in the non-acute
occur earlier and are more common than respiratory, or neurological episodes. In this phase have been associated with an
gastrointestinal and cardiovascular manifes- situation, determination of plasma levels of increased incidence of severe reactions fol-
tations. In food anaphylaxis, gastrointesti- mast cell mediators (histamine and mast cell lowing insect stings, suggesting that the
nal symptoms are more frequent, whereas tryptase) may provide additional diagnostic patients most likely to develop anaphylaxis
cardiovascular symptoms are rare. Gastroin- help.7 Mast cell tryptase occurs in an alpha may have either an increased mast cell mass
testinal symptoms include abdominal dis- form that is constitutively released and a beta or an increased mast cell releasability.
comfort and vomiting. Gastrointestinal form that is released only following mast cell The investigation of allergic triggers
features are associated with cardiovascular activation. In anaphylaxis, mediators are ele- requires referral to a consultant allergist
rather than respiratory manifestations. The vated in approximately 50% of patients pre- for performance of appropriate skin prick
cutaneous features of pruritus, erythema, senting to emergency departments and in and blood tests to determine the presence
urticaria, and angio-oedema occur in nearly approximately 80% of fatal cases. Histamine of specific IgE antibodies. Serum allergen
all children. These are commonly the first elevation is better correlated than tryptase specific IgE levels determined via UniCAPW
symptoms experienced, occurring within with the severity of the symptoms. However, above which patients have a >95% chance
minutes following allergen exposure. Life- histamine and tryptase may also be elevated of having an immediate IgE-mediated reac-
threatening symptoms and signs include in milder cases of acute allergic reactions with tion8 have been determined for some foods
loss of consciousness, syncope, dizziness, cutaneous reaction alone. (e.g. cows milk, egg, peanut, wheat). It is not
light-headedness, cerebral dysfunction, It is necessary to collect blood for hista- possible to predict the severity of a future
hypotension, hypoxia, stridor, cyanosis, and mine within 10 minutes to 1 hour following allergic reaction based on the skin prick test
laryngeal oedema. the reaction, as histamine levels peak at 5 size or allergen specific IgE levels.

Table 22.5.1 Presenting features of children with anaphylaxis5

Treatment
Presenting feature Per cent

Cutaneous (urticaria, angio-oedema, flushing, or warmth 90

Adrenaline (epinephrine)
Adrenaline should be given via the intra-
Upper airway (throat tightness or itchiness, drooling, stridor, oropharyngeal swelling) 80
muscular rather than the subcutaneous
Lower airway (chest tightness, wheezing) 60 route, due to better absorption from mus-
Gastrointestinal (abdominal discomfort, vomiting) 40
cle.9 In children, peak adrenaline levels were
reached 8 minutes after intramuscular and
Cardiovascular (arrhythmias, hypotension, poor capillary refill, weak pulses) 30
34 minutes after subcutaneous injection.
Neurological (confusion, decreased conscious state) 25 Peak levels were 20% higher after intramus-
Generalised (diaphoresis, tingling, an impending sense of doom) 15
cular injection. The preferred injection site
for intramuscular administration is the

469
22.5 ANAPHYLAXIS

upper outer side of the thigh, which gives In unrelenting hypotension, pressors NonIgE-mediated anaphylactoid reactions
significantly better absorption as compared such as dopamine or isoproterenol may are clinically indistinguishable from anaphy-
with the deltoid muscle. be indicated. laxis. Drugs that can cause anaphylactoid
reactions include opiates, muscle relaxants,
The dose of adrenaline is 0.01 mL kg1 radiocontrast media, non-steroidal anti-
of 1 in 1000 intramuscular injection. Supplemental treatment
The rationales for supplementary inflammatory drugs and quinolone and van-
Improvement should be seen within
treatment with steroid and comycin antibiotics.
minutes. The dose should be repeated after
515 minutes if the effect is incomplete. antihistamines are less well defined.
If steroid is used, give Stings Stings are most commonly due to
Approximately one-third of patients will
methylprednisolone 1 mg kg1 bees and less often wasps. In Australia,
require more than one dose of adrenaline.
intravenously. jumper ant (Myrmecia spp.) stings are a com-
In situations where there is severe
circulatory compromise, adrenaline 1 in Antihistamine: promethazine 1.0 mg kg1 mon cause of anaphylaxis in endemic areas.
10000 0.1 mL kg1 should be given per dose (maximum 25 mg) orally
or intravenously (slow) is given for Latex Sensitisation occurs particularly in
by slow intravenous injection over
symptomatic relief of urticaria. children with multiple exposure to latex-
10 minutes. Patients given intravenous
containing items during medical procedures.
adrenaline require cardiac, respiratory,
Duration of treatment Children with spina bifida are particularly at
and blood pressure monitoring.
Duration of treatment is determined by clin- risk due to multiple exposures following
If there is an inadequate response,
surgery and urinary tract catheterisation.
administer continuous intravenous ical response. Repeated doses of adrenaline
adrenaline using a 1:100 000 (epinephrine) are often required.
Less common
(0.01 mg mL1) dilution at a rate of
Admission Idiopathic No triggers are identified,
0.1 mcg kg1 per minute, to a maximum
Admit or observe for at least 12 hours all despite full investigation. Cases may present
of 1 mcg kg1 per minute, titrated
patients with significant anaphylaxis, as a with laryngeal oedema as the only manifes-
according to the response.
biphasic reaction with deterioration may tation. The episodes can usually be con-
For refractory cardiorespiratory arrest, the
trolled by regular antihistamine and, if
initial intravenous dose is 10 mcg occur following the initial episode.
necessary, the addition of alternate day ster-
(0.01 mg) per kg body weight of a 1 in
The role of H1 and H2 blockers oids.10 Psychogenic anaphylaxis has been
10 000 (wt/vol) dilution. Subsequent
The role of antihistamines is unclear. Addi- classified as a variant of idiopathic anaphy-
doses are 100 mcg kg1. (1 mL of 1 in
tion of H2 to H1 antagonists produces no dif- laxis and should be considered in the differ-
10 000) (wt/vol) every 35 minutes and,
ferences in blood pressure and symptoms; ential diagnosis.
if still refractory, the dose may be
increased to 200 mcg kg1. however, there is less urticaria at 2 hours
in patients treated with combined H1 and Uncommon
Adverse effects of adrenaline include
Exercise-induced Symptoms of urti-
transient pallor, tremor, anxiety, H2 blockers. Antihistamine is not a substi-
tute for adrenaline. caria, angio-oedema, and stridor plus or
palpitations, cardiac arrhythmias,
minus hypotension develop during or soon
headache, and nausea.
Patients on beta-blockers after cessation of vigorous exercise.
Glucagon 0.02 mg kg1 intravenously has
Airway and breathing Food-dependent exercise-induced
been used in an attempt to reverse beta
Give high-flow oxygen by mask. In this situation, exercise induces symptoms
blockade.
For bronchospasm, give continuous only following ingestion of the relevant
nebulised salbutamol (0.5%). foodstuff, which has included wheat, celery,
Nebulised adrenaline (epinephrine) shellfish, oranges, and peaches. In some but
0.5 mL of 1% may be used in conjunction Diagnosis
not all cases, IgE sensitisation to the rele-
with systemic administration or alone for It is important to determine the cause of the vant food can be demonstrated.
isolated mild upper airway obstruction. anaphylactic reaction whenever possible.
Intubate if obstruction is severe. Recurrent anaphylaxis
Types of anaphylaxis Anaphylaxis is frequently multiple, and in
Circulation Common one series two-thirds of patients had three
Achieve intravascular access with large- Foods Severe life-threatening reactions or more anaphylactic episodes. Efforts
bore cannula. are predominantly due to peanut and tree should be made to identify unrecognised
Treat hypotension with normal saline nuts. In pre-school-age children, they may triggers. In children, this is often due to foods
20 mL kg1. be due to egg and cows milk proteins. (generally peanut or tree nut products)
If hypotension continues, give further contained in manufactured or processed
colloid boluses of 10 mL kg1 and repeat Drugs Many reactions, in particular foods. Some cases without an identifiable
adrenaline (epinephrine) dose. those due to antibiotics, are IgE-mediated. cause are due to idiopathic anaphylaxis.

470
22.5 ANAPHYLAXIS
22

ENVIRONMENTAL
Differential diagnosis
fatalities are recorded in children over significantly reducing the risks
Anaphylaxis should be distinguished from
5 years of age, despite the fact that and increasing the efficacy of
other presentations that may cause confu-
food-allergic reactions are more immunotherapy.
sion. These include:
common in pre-school children and
 The promotion of desensitisation or
acute asthma; frequently lessen with time. As the
tolerance in food allergy by graded oral
vasovagal syncope; prescription of an EpiPen is primarily
administration of food allergens such as
urticaria or angio-oedema; concerned with risk management, it is
peanut or egg.
psychogenic stridor; necessary to consider the factors that
cardiovascular events; point to the likelihood of developing
seizure disorders; a severe life-threatening reaction.12
mast cell mediator release in These are: Acknowledgement
mastocytosis;
age over 5 years; The contribution of David Singh as author in
hereditary angio-oedema.
a history of respiratory tract the first edition is hereby acknowledged.
involvement with the initial or
Prevention subsequent reactions;
a history of asthma requiring References
Advice concerning the avoidance of the trig- preventive medication; 1. Sampson HA, Munoz-Furlong A, Campbell RL, et al.
gering allergen is critical. This will usually Second symposium on the definition and management of
peanut or tree nut sensitivity; anaphylaxis: summary reportSecond National Institute
require referral to a consultant allergist.
reactions induced by traces or small of Allergy and Infectious Disease/Food Allergy and
The provision of self-injectable or carer- amounts of allergen;
Anaphylaxis Network symposium. J Allergy Clin Immunol
2006;117(2):3917.
administered adrenaline should be con-
a strongly positive skin prick test 2. Poulos LM, Waters AM, Correll PK, et al. Trends in
sidered for all children with anaphylaxis. (>8 mm).
hospitalizations for anaphylaxis, angioedema, and
urticaria in Australia, 19931994 to 20042005.
Inadvertent re-exposure is most likely in J Allergy Clin Immunol 2007;120(4):87884.
the case of insect stings and foods, and least Each factor should be considered, 3. Liew WK, Williamson E, Tang ML. Anaphylaxis fatalities
and the greater the number that are and admissions in Australia. J Allergy Clin Immunol
likely for drugs. The prescription of a self- 2009;123(2):43442.
injectable adrenaline also requires instruc- positive, the lower the threshold for 4. Kemp SF, Lockey RF. Anaphylaxis: A review of causes
prescribing an EpiPen. In addition, and mechanisms. J Allergy Clin Immunol 2002;1103:
tion in the indications for and demonstration 3418.
of use and the provision of a clear and simple these factors need to be weighed in 5. Lee JM, Greenes DS. Biphasic anaphylactic reactions in
the light of the parental wishes and pediatrics. Pediatrics 2000;1064:7626.
written anaphylaxis action plan. 6. Mehr S, Liew WK, Tey D, Tang ML. Clinical predictors for
Self-injectable adrenaline is available in environmental circumstances. biphasic reactions in children presenting with
Providing the parents with a rational anaphylaxis. Clin Exp Allergy 2009;39(9):13906.
two fixed-dosages: (0.15 mg of adrenaline) 7. Lin RY, Schwartz LB, Curry A, et al. Histamine and tryptase
for children 1530 kg and (0.3 mg of adren- perspective on the remote risk of death levels in patients with acute allergic reactions: An
is essential. emergency department-based study. J Allergy Clin
aline) for children greater than 30 kg. The Immunol 2000;106(1 Part 1):6571.
American Academy of Asthma Allergy and 8. Sampson HA. Utility of food-specific IgE concentrations
in predicting symptomatic food allergy. J Allergy Clin
Immunology recommends the 0.3 mg dose Immunol 2001;107(5):8916.
for children >20 kg.11 9. Simons FE, Roberts JR, Gu X, et al. Epinephrine absorption
in children with a history of anaphylaxis. J Allergy Clin
In venom-induced anaphylaxis referral to Immunol 1998;101(1 Part 1):337.
an allergist for desensitisation should be con- Future directions 10. Ditto AM, Krasnick J, Greenberger PA, et al. Pediatric
idiopathic anaphylaxis: Experience with 22 patients.
sidered for life-threatening reactions with and research J Allergy Clin Immunol 1997;1003:3206.
respiratory or cardiovascular involvement if 11. AAAI Board of Directors. The use of epinephrine in the
there is an appropriate reagent available, Humanised monoclonal anti-IgE treatment of anaphylaxis. J All Clin Immunol
1994;944:6668.
such as honey bee (Apis mellifera), paper antibodies increase the threshold dose of 12. Kemp AS. EpiPen epidemic: suggestions for rational
wasp (Polistes spp.) or European wasp food required to trigger symptoms in prescribing in childhood food allergy. J Paediatr Child
Health 2003;39(5):3725.
(Vespula spp.). This will involve a series of food-induced anaphylaxis. Regular
injections with venom for a duration of 3 or administration is required. Unlike
more years. In general, venom desensitisation desensitisation, the treatment is not
Further reading
is not recommended for children with gener- allergen-specific, and therefore offers
Brown SG. Anaphylaxis: clinical concepts and research
alised cutaneous reactions in the absence of promise to individuals with life- priorities. Emerg Med Australas. 2006;18(2):15569.
threatening reactions to multiple Joint Task Force on Practice Parameters, American Academy
respiratory or cardiovascular involvement. of Allergy, Asthma and Immunology, American College of
allergens. Allergy, Asthma and Immunology, and the Joint Council of
Allergy, Asthma and Immunology. The diagnosis and

Controversies Characterisation of the molecular management of anaphylaxis. J Allergy Clin Immunol

1998;101(6 Part 2):S465528.
structure of allergenic epitopes may Muraro A, Roberts G, Clark A, et al. EAACI Task Force on
Currently, there are no clear guidelines allow the construction of peptides for Anaphylaxis in Children. The management of anaphylaxis
in childhood: position paper of the European academy of
on which children should be prescribed immunotherapy that trigger T-cell allergology and clinical immunology. Allergy. 2007;62
an EpiPen. The great majority of responses without binding IgE, thus (8):85771.

471
SECTION

23 COMMON
PROCEDURES
Section editor Gary Browne

23.1 Length-based paediatric drug dosing and 23.10 Central and peripheral intravenous lines 494
equipment sizing 472
23.11 Intraosseous infusions 500
23.2 Bagmask ventilation 474
23.12 Rectal drug administration 502
23.3 Nasopharyngeal, oropharyngeal airways
23.13 Umbilical vessel cannulation 503
and the laryngeal mask airway 476
23.14 Electrical countershock 506
23.4 Endotracheal tube and tracheal
intubation 479 23.15 Pericardiocentesis 508
23.5 Confirmation of intubation 483 23.16 Transurethral catheterisation and suprapubic
bladder aspiration 511
23.6 The surgical airway 485
23.17 Penile zipper injury 514
23.7 Needle thoracostomy 488
23.18 Lumbar puncture 515
23.8 Tube thoracostomy 490
23.9 Removing and replacing a tracheostomy
tube 492

23.1 Length-based paediatric drug dosing

and equipment sizing
Ronald A. Dieckmann

There are two methods of rapid drug dos- additional comprehensive drug reference is
Background
ing and equipment sizing: (1) a software imperative to address the multiple pharma-
Emergency treatment of infants and children paediatric decision support program; (2) a cological needs of children.
is sometimes difficult because children of dif- length-based resuscitation tape. Software
ferent ages require different sizes of equip- decision support programs for desktop, or
ment, doses of medications, and volumes of laptop computers or PDA are now available:
fluids. Errors are common when selecting by imprinting a childs length, the programs Indication
appropriate equipment and medications in provide exact drug doses or equipment
Children requiring equipment, medication,
critical paediatric emergencies, and mistakes sizes for a vast range of medications and
or fluids, weighing 334 kg body weight
are especially frequent with doses of drugs equipment.
(about age 1012 years).
that are powerful cardiovascular agents, such The paediatric resuscitation tape or
as adrenaline (epinephrine). length-based resuscitation tape is a simple
Length-based drug dosing and equip- visual tool to measure lengths and to
ment sizing is an effective method for rapid approximate weights in children. There are
selection of emergency treatments. Using several commercial products available. One
Contraindications
length as a basis for ordering drugs and type of tape utilises colour zones to cluster Premature infant weighing less than
equipment is at least as accurate as closely matched sizes of children into cate- 3 kg.
weight, and length may be more accurate gories that have the same drug and equip- Child older than 1012 years of age
than weight for equipment sizing. It is also ment requirements. A disadvantage of the or weighing more than 34 kg body
less likely to cause error in high-stress resuscitation tape is that only a few drugs weight (use adult equipment and drug
circumstances. are listed on the tape. Hence, a current dosages).

472
23.1 LENGTH-BASED PAEDIATRIC DRUG DOSING AND EQUIPMENT SIZING
23

COMMON PROCEDURES
weight in kilograms that corresponds
to the childs measured length at
the heel.
If the child is longer than the tape,
use adult equipment and medication
doses.
 From the tape, identify appropriate
equipment sizes (see Fig. 23.1.2).
From the tape, identify appropriate
medication doses (see Fig. 23.1.2).

Complications
None.

Tips
Use the decision support software or
resuscitation tape instead of attempting
to estimate weight or calculate
equipment sizes or drug doses.
Fig. 23.1.1 Decision support software allows instantaneous calculations of drug doses and Measuring to the childs toes (instead of
equipment sizes, based upon patient length.
heel) will add a number of kilograms to
the estimated weight and may result in
over-sized equipment and over-large
Equipment Preparation
drug doses.
Computer or PDA based paediatric Place the patient in a supine position.
decision support software Extend the patients legs.
(Fig. 23.1.1).  Measure the child in centimetres. Further reading
Colour-coded paediatric resuscitation Black K, Barnett P, Wolfe R, Young S. Are methods used to
tape (Fig. 23.1.2). Store the tape estimate weight in children accurate? Emerg Med
in a place that is easily accessible, Procedure decision (Fremantle) 2002;14(2):1605.
Davis D, Barbee L, Ririe D. Paediatric endotracheal tube
such as in the paediatric equipment support software selection: A comparison of age-based and height-based
criteria. Am Ass Nurse Anesth J 1998;66(3):299303.
kit, on the paediatric code cart, or
After measuring the childs length, input Hofer CK, Ganter M, Tucci M, et al. How reliable is length-
on a wall hook. Lamination of the based determination of body weight and tracheal tube size
into computer program and read drug doses in the paediatric age group? The Broselow tape
tape may preserve longevity. There
and equipment sizes (see Fig. 23.1.1). reconsidered. Br J Anaesth 2002;88(2):2835.
are several different brands of Lubitz DS, Seidel JS, Chameides L, et al. A rapid method for
estimating weight and resuscitation drug dosages from
length-based paediatric resuscitation length in the paediatric age group. Ann Emerg Med
tapes, and these have not been Tape 1988;17(6):57681.
Luten RC, Wears RL, Broselow J, et al. Related articles, links
compared for speed, accuracy,
or safety. Measure childs length from head to abstract length-based endotracheal tube and emergency
equipment in paediatrics. Ann Emerg Med
heel with the tape. Note and say 1992;21:145460.

Fig. 23.1.2 Colour-coded paediatric resuscitation tape.

473
 23.2 Bagmask ventilation
Michelle Lin

Hypoxia despite high-flow oxygen

Background administration via a non-rebreather Positioning
Bagmask (BM) ventilation is the most mask. Patient positioning is essential for successful
important skill in paediatric airway manage- BM ventilation. Maintain a supine, neutral
ment. This non-invasive manoeuvre for neck position to keep the airway patent.
assisted, positive-pressure ventilation is Because of their large occiputs, infants and
Contraindications
effective treatment for most children with toddlers are prone to hyperflexion of the
hypoventilation and hypoxia. A child with Do not perform BM ventilation in the setting neck and consequently benefit from a small
respiratory insufficiency may require only of a complete airway obstruction. If this exists, towel roll under their shoulders to achieve a
temporary assisted ventilation with BM. first perform airway clearance and basic life- neutral sniffing position. Both hyperflexion
Some children in respiratory failure who support manoeuvres. Then attempt foreign- and hyperextension of the neck may worsen
require prolonged ventilation or airway pro- body removal of the obstruction, if necessary, airway obstruction, compromise ventilation,
tection may need tracheal intubation under direct laryngoscopy with Magill forceps. and increase risk of spinal injury.
(Chapter 23.3).
In the BM setup, oxygen flows into a bag
Relative contraindication
reservoir, through a pop-off valve, and into a
In the presence of a congenital diaphrag- Procedure
mask, which forms a tight seal around the
matic hernia or a tracheo-oesophageal fis-
childs nose and mouth. Squeezing the bag
tula, BM can cause insufflation of the Perform the head-tilt and chin-lift
administers oxygen under positive pressure manoeuvre to open the airway and lift
stomach and subsequent extrapulmonary
to the lungs. While this manoeuvre does the tongue away from the soft palate
compression of the lungs. This may compro-
not fully protect the airway, as tracheal of the oropharynx. Proper positioning
mise adequate oxygenation and ventilation.
intubation does, BM ventilation adequately and suctioning of excess secretions
provides emergent airway support during will often relieve the respiratory
the acute decompensation period (Table compromise without BM ventilation.
23.2.1). Additionally, Gausche et al demon- Equipment In the setting of trauma where spinal
strated that BM ventilation is as effective
as tracheal intubation in the pre-hospital
Appropriately-sized mask (Fig. 23.2.1).
injury is a consideration, do not perform

setting for airway management, regardless

Self-inflating bag reservoir (Fig. 23.2.2).
the head-tilt/chin-lift manoeuvre.

of the underlying aetiology.1 Thus, both

 Oxygen saturation monitor.
Instead, provide in-line spinal
immobilisation and perform a jaw-thrust
pre-hospital and in-hospital practitioners manoeuvre to create a patent airway.
must be comfortable and proficient in  If airway patency is still suboptimal,
performing BM ventilation. Preparation reposition and suction the patient. If still
inadequate, insert an oropharyngeal
Select an appropriately-sized mask and
or nasopharyngeal airway underneath
ventilation bag.
the mask to lift the tongue from the
Connect the oxygen tubing to the
Indications ventilation bag, and attach to the face
posterior oropharynx (Chapter 23.3).
In the one-person BM technique place
Hypoventilation or apnoea. mask.
the mask on the patients face and
Respiratory failure.  Begin 15 litres of oxygen per minute.
achieve an airtight seal over the mouth
and nose. Using the non-dominant
Table 23.2.1 Choosing BM ventilation versus tracheal intubation hand, place the thumb and index finger
on the superior and inferior parts of
Sample patient case BM Intubation
the face mask, respectively. Cradle the
Hypoventilation during procedural sedation
tips of the other three fingers along the
Hypoventilation during post-ictal stage mandible and lift the jaw up toward
Hypoxia during asthma exacerbation despite non-rebreather oxygen mask
the mask to create the seal. This is the
EC clamp manoeuvre, based on the
Persistent hypoxia or hypoventilation despite BM
E-shape of the three fingers along
Partial airway obstruction (laryngeal burn, angio-oedema) the jaw and the C-shape of the thumb
Cardiopulmonary arrest
and index finger along the face mask
(Fig. 23.2.3). In creating the seal, pull

474
23.2 BAGMASK VENTILATION
23


COMMON PROCEDURES
the jaw anteriorly rather than push In addition to visualisation,
the mask posteriorly into the patients auscultation of bilateral breath
face. To ventilate, squeeze the oxygen sounds in the mid-axilla and
reservoir bag using the dominant hand. improvement of the oxygen saturation
For prolonged BM ventilation, use the both corroborate with adequate
little finger of your non-dominant hand ventilation.
(used for the EC clamp) and apply  With prolonged BM ventilation,
cricoid pressure to reduce gastric air decompress the likely over-distended
insufflation. stomach with an orogastric (OG) or
In the two-person technique place the nasogastric (NG) tube. This reduces
mask on the patients face and achieve the risk of emesis and consequent
Fig. 23.2.1 Choosing the appropriately sized
an airtight seal over the mouth and aspiration.
bagmask. Variously-sized, clear masks are
available for all ages, ranging from neonates to nose. For operator #1, place the thumb
adults. These masks have an inflated and index finger of both hands on the
circumferential rim, which provides a tight seal to superior and inferior parts of the face
the patients lower face. With an appropriately- mask, respectively. Cradle the tips of Complications
sized mask, the superior aspect of the mask should
the other three fingers of both hands
rest over the patients nasal bridge and the inferior
along either side of the mandible and Incorrect mask sizing causing
aspect should rest over the cleft of the chin. Too inadequate ventilation or trauma to
large a mask may compress the patients eyes, and symmetrically lift the jaw up toward
the eyes.
too small a mask may occlude the nostrils and the mask to create a seal. This is a
impede adequate oxygenation and ventilation. double EC clamp manoeuvre Gastric insufflation and distension.
(Fig. 23.2.4). Again, pull the jaw Aspiration from emesis.
anteriorly rather than push the mask Pneumothorax from poor lung
compliance or excessive tidal volumes.
posteriorly into the patients face.
Operator #2 ventilates the patient by Hypoxia.
squeezing the oxygen reservoir bag,
and applies cricoid pressure for
prolonged BM ventilation to reduce
Tips
gastric air insufflation.
Provide BM ventilation at a rate of 20, 30, In paediatric trauma cases requiring
and 40 inspirations per minute for the in-line spinal immobilisation, perform
child, infant, and neonate, respectively. BM ventilation with the two-person
Fig. 23.2.2 Paediatric oxygen bag. Squeeze and release the bag at an technique. One operator focuses on
Supplemental, high-flow oxygen flowing into inspiratory-to-expiratory ratio of 1:2. providing an adequate mask seal,
a bag serves as the oxygen reservoir for BM A common error is to over-ventilate the while the other operator focuses on
ventilation. A paediatric bag, which has a volume
patient with too rapid a rate. squeezing the bag and maintaining
of 450750 mL, is adequate to oxygenate
and ventilate a small child. Alternatively, if a Provide a BM tidal volume of 8 mL kg1 spinal alignment. In comparison, a
paediatric bag is not readily available, an adult to oxygenate and ventilate the patient. one-person BM technique often
bag, which has a volume of about 1200 mL, As an equally effective alternative inadvertently extends the patients
can also provide adequate oxygenation and measure of appropriate ventilation neck while trying to achieve an
ventilation. Be careful not to administer excessive
volume, watch for bilateral chest rise adequate mask seal.
tidal volumes with this larger bag.
and fall. When elevating the jaw anteriorly to
form a mask seal with the third, fourth
and fifth fingers, be sure to lift up
along the bony mandible rather than
the submandibular soft tissue. In
addition to trauma, compressing the
submandibular soft tissue may
inadvertently occlude the airway.
Some BM reservoir bags have a pop-off
valve to prevent excessive positive
pressure ventilation. This may
inadequately ventilate a patient with low
lung compliance. Occlude this valve to
allow higher positive pressures during
Fig. 23.2.3 EC clamp BM technique. Fig. 23.2.4 Double EC clamp BM technique. inspiration, while watching for chest rise.

475
23.3 NASOPHARYNGEAL, OROPHARYNGEAL AIRWAYS AND THE LARYNGEAL MASK AIRWAY

For the neonate and infant, assessing This approximates the correct ventilatory
chest rise and fall for adequate BM tidal rate and inspiratory-to-expiratory ratio Further reading
for the patient. Brown RE. Bag and mask ventilation. In: Dieckmann RA, et al.,
volume is subtle. The optimal viewing editors. Paediatric emergency and critical care procedures.
angle is from the patients side at the St Louis, MO: Mosby; 1997.
Chameides L, Hazinski MF, editors. Paediatric advanced life
level of his or her bed. support. Dallas, Texas: American Heart Association; 1997.
In order to prevent the common Dieckmann RA, Brownstein DR, Gausche-Hill M, editors.
Paediatric Education for Prehospital Professionals. Sudbury,
complication of over-ventilating a Reference MA: Jones & Bartlett Publishers; 2000.
patient, say aloud squeeze release 1. Gausche M, Lewis RJ, Stratton SJ, et al. Effect of out-of- Lee BS, Gausche-Hill M. Paediatric airway management. Clin
hospital paediatric endotracheal intubation on survival Paediatr Emerg Med 2001;2(2):91106.
release repeatedly while correspondingly and neurological outcome: A controlled clinical trial. JAMA Zideman D, et al. Airways in paediatric and newborn
squeezing and releasing the bag. 2000;283(6):78390. resuscitation. Ann Emerg Med 2001;37(4 Suppl):S12636.

23.3 Nasopharyngeal, oropharyngeal

airways and the laryngeal mask airway
Michelle Lin Conor Deasy

the curvature of the oropharynx. The distal OP airway

Background end rests along the posterior tongue, which Intact gag reflex because the OP airway tip
Children have relatively large tongues, would trigger the gag reflex in an awake rests on the posterior tongue, a patient with
which may fall back and cause airway patient, and the proximal end has a wide an intact gag reflex will likely vomit and
obstruction when there is loss of nasopha- flange to anchor the tube in place at the lips. aspirate gastric contents.
ryngeal muscle tone, for instance, in patients Airway adjuncts may allow adequate
who are post-ictal or overly sedated by spontaneous ventilation and avert the need
drugs. Hypoxia and hypercarbia may for bagvalvemask ventilation or the more
develop. Non-invasive airway adjuncts, such invasive technique of tracheal intubation. Equipment
as nasopharyngeal (NP) and oropharyngeal
(OP) airways, can maintain a patent airway Lubricating jelly (for NP airway).
even when the tongue falls back against Tongue blade (optional for OP
Indications for OP or NP airway).
the posterior pharyngeal wall. The laryngeal
mask airway (LMA) is designed to provide a Airway obstruction.  NP airway (Fig. 23.3.1).
seal around the laryngeal inlet when Respiratory decompensation. OP airway (Fig. 23.3.2).
inserted and the cuff inflated, and is used Seizures.
in situations involving a difficult bag
valvemask fit in an unconscious patient
and as a back-up device where tracheal Preparation
intubation is not successful.
Contraindications
NP airway
Structurally, an NP airway is hollow, made NP airway
Determine the correct NP airway size by one
of latex or a latex-like substance, and has a Age less than 1 year old the nares of three methods:
slight curvature to approximate the curva- diameter is too small to introduce an NP
ture of the nasopharynx. The distal end airway. Follow the recommendations on a
has a bevel, which helps to tunnel through Nasal obstruction attempting to length-based resuscitation tape.
the nasopharyngeal soft tissue, but which introduce an NP airway into a nasal Choose an NP airway the length of
may also cause inadvertent shearing trauma passage that is obstructed will be which is equivalent to the distance from
to the nasal septum. The proximal end of the unsuccessful and may cause traumatic the patients nasal tip to the tragus of
NP airway has a wide flange to anchor the epistaxis. the ear (Fig. 23.3.3).
tube in place at the nostril orifice. Patients Severe facial injury or suspicion for basilar  Choose an NP airway the outer
tolerate this airway adjunct better than an skull fracture a fractured cribriform diameter of which is equivalent to
OP airway because it does not trigger the plate may allow an NP airway to the nostrils inner diameter. Cut the
gag reflex. traverse incorrectly into the intracranial length of the tube according to
The OP airway is hollow, made of plastic, space rather than into the posterior the length-measurement guide in
and has a slight curvature to approximate oropharynx. Method 2.

476
23.3 NASOPHARYNGEAL, OROPHARYNGEAL AIRWAYS AND THE LARYNGEAL MASK AIRWAY
23

COMMON PROCEDURES
that the tip passes the septal border.
When resistance is felt, re-rotate the NP
airway 180 degrees into its original,
correct orientation. Finish inserting the
NP posteriorly into the nostril until it rests
just external to the nostril orifice.

OP airway
The most common mistake in OP airway
insertion is causing damage to the soft pal-
ate or pushing the tongue further back while
inserting it and thus worsening airway
Fig. 23.3.4 Sizing the OP airway: corner of obstruction. Two methods will prevent this:
mouth to mandibular angle.
With tongue blade after pushing the
tongue inferiorly with a tongue blade,
OP airway slide the OP airway directly over it
Determine the correct OP airway size by one concave down, i.e. with the tip pointed
Fig. 23.3.1 Nasopharyngeal airway. of two methods: inferiorly. Continue until the flange
gently rests against the lips.
Follow the recommendations on a
Without tongue blade rotate the OP
length-based resuscitation tape.
airway 180 degrees such that the tip
Choose an OP airway the length of which
points superiorly and insert it into the
is equivalent to the distance from the
mouth. When it contacts the hard palate,
patients incisors to the mandibular
depress the tongue with the OP airway
angle, as measured from the side of the
curvature. Insert the OP airway completely
patients face (Fig. 23.3.4).
until the flange reaches the lips. Then re-
rotate the airway 180 degrees so that the
tip correctly points inferiorly and rests
Positioning along the posterior tongue.
Place the patient in a supine, neutral
sniffing position.
To prevent the patients tongue from Complications
worsening the airway obstruction, Emesis and aspiration.
perform a chin-lift manoeuvre. Use a jaw- Laryngospasm.
thrust manoeuvre for the trauma patient Local trauma and bleeding.
in whom spinal immobilisation is crucial. Worsening airway obstruction when the
OP airway is inappropriately long, it may
directly occlude the posterior oropharynx;
Procedure when it is inappropriately short, it may
push the tongue further back, causing
NP airway
more airway obstruction.
Prelubricate the NP airway before
Fig. 23.3.2 Oropharyngeal airway. insertion. NP airway plug the relatively small
diameter of the NP airway makes it easily
When inserting the NP airway in the right
prone to occlusion with mucus, secretions,
nostril, the bevel already points toward
and blood.
the septum and is of minimal risk for
nasal septal trauma. Gently introduce the Intracranial placement of the NP airway.
NP airway directly posteriorly into the
patients nostril until the flange rests just
external to the nostril orifice.
Tips
 When inserting the NP airway in the left Insert the NP airway directly posteriorly
nostril, the bevel is pointed away from the along the floor of the nose and not
septum. To avoid septal trauma, rotate superiorly.
the NP airway 180 degrees so that the Do not select too wide an NP tube,
Fig. 23.3.3 Sizing the NP airway: nasal tip to bevel now points towards the septum. which may cause pressure necrosis to
tragus of ear. Insert the airway approximately 2 cm so the nasal ala.

477
23.3 NASOPHARYNGEAL, OROPHARYNGEAL AIRWAYS AND THE LARYNGEAL MASK AIRWAY

Table 23.3.1 Recommended weight-based sizing and inflation volumes

Weight of patient Recommended Maximum air

size guidelines in cuff (mL)

5 kg Size 1 4

510 kg Size 1.5 7

1020 kg Size 2 10

2030 kg Size 2.5 14

30 kgsmall adult Size 3 20

Adult Size 4 30

Large adult Size 5 40

Fig. 23.3.5 Endotracheal tube cut into an NP
airway.

 If an NP airway is not immediately Preparation Procedure (Fig. 23.3.6)

available, an alternative is to use a
endotracheal tube, normally used for
Select correct size (Table 23.3.1). The mask is held like a pen and inserted
intubations. First, choose the tube where
Check the LMA testing inflation and while pressing against the palate and
deflation of the cuff. posterior pharyngeal wall using the index
the outer diameter is equivalent to the
inner diameter of the patients nostril.
Lubricate the back of the LMA finger until resistance is felt when the
thoroughly using a water soluble mask tip reaches the triangular base of
Second, trim the tube from the proximal
lubricant; however, avoid excessive the oropharynx.
end to the appropriate length as
measured from the patients nasal tip
amounts as inhalation of the The lumen of the LMA should be facing
lubricant result in coughing or the patients tongue and not the hard
to the tragus of the ear. Leave the
obstruction. palate.
proximal ventilator adapter on the
endotracheal tube in place so that the
Inflate the mask with the recommended
volume of air (see Table 23.3.1), avoiding
tube is anchored at the nasal tip and
over inflation.
does not accidentally slip beyond the Positioning
nares (Fig. 23.3.5).
Normally the mask should be allowed
Extend the head and flex the neck if to rise up slightly out of the hypopharynx as
Be sure the patient does not have a gag
possible. it is inflated to find its correct position.
reflex before placing an OP airway.

Indications for laryngeal

mask airway
Situations involving a difficult mask
(bagvalvemask) fit.
May be used as a back-up device where
tracheal intubation is not successful.
May be used as a second-last-ditch
airway where a surgical airway is the only (a) (b) (c)
remaining option.

Equipment
Appropriate size LMA.
Syringe with appropriate volume for LMA
cuff inflation.
Water soluble lubricant.
Bagvalvemask to ventilate.
Stethoscope to check for adequate air (d) (e) (f)
entry into both lungs once in place.
Tape or other device(s) to secure LMA. Fig. 23.3.6 Placing a laryngeal mask airway.

478
23.4 ENDOTRACHEAL TUBE AND TRACHEAL INTUBATION
23

COMMON PROCEDURES
Connect the LMA to a bagvalvemask pushing the epiglottis into its down-folded Insert a bite-block or roll of gauze to
device or low pressure ventilator. position, causing mechanical obstruction. prevent occlusion of the tube should the
Ventilate the patient while confirming patient bite down.
equal breath sounds over both lungs in all
fields and the absence of ventilatory Tips
sounds over the epigastrium.
Secure the LMA in position using the same The insertion of the LMA by the standard
technique is not always easy owing to the
techniques as for an endotracheal tube. Further reading
anatomy of the paediatric airway. Some
Chameides L, Hazinski MF, editors. Paediatric advanced life
advocate a rotational technique: with a support. Dallas, TX: American Heart Association; 1997.
partially inflated cuff, the mask is inserted Dieckmann RA, et al., editors. Paediatric education for
Complications prehospital professionals. Sudbury, MA: Jones & Bartlett
with its lumen facing backwards and then Publishers; 2000.
Inadequate lubrication or lack of pressure on rotated through 180 degrees, when the Greenberg RS. Facemask, nasal and oral airway devices.
Anesthesiol Clin N Am 2002;20(4):83361.
the deflated mask up against the hard pal- resistance of the posterior pharyngeal Shifrm SW. Insertion of oral and nasal airways. In:
ate on placement can cause the mask tip wall is felt, and then passed downwards Dieckmann RA, et al., editors. Paediatric emergency and
critical care procedures. St Louis, MO: Mosby; 1997.
to fold back on itself. This may progress, into position behind the larynx.

23.4 Endotracheal tube and tracheal

intubation
Michelle Lin

Background  A paediatric patient has more anterior In addition to proper positioning and equip-
and cephalad vocal cords. A straight ment selection, successful emergent intuba-
Tracheal intubation (TI) provides a definitive laryngoscopic blade provides the best tion often requires the administration of
airway. Insertion of a tube between the direct line-of-sight to the anteriorly- rapid sequence induction (RSI) medications.
vocal cords and into the trachea allows opti- positioned cords. These medications provide transient seda-
mal management of the patients oxygena- The cricoid ring is the narrowest part of tion and neuromuscular relaxation during
tion and ventilation, while also protecting the airway in patients less than 8 years the procedure. Induction agents include
the airway from aspiration. Depending on old. This anatomical narrowing naturally ketamine, fentanyl, and midazolam, and
the preparation and skill of the practitioner, secures the endotracheal tube in place neuromuscular paralysing agents include
this procedure can be either life saving or life at the cricoid ring level without an air succinylcholine, rocuronium, and vecuro-
compromising. TI is the standard rescue pro- leak. Endotracheal tubes therefore do nium. The advantages of RSI are twofold.
cedure when bagmask (BM) ventilation is not need to be cuffed. In contrast, First, the patient will not instinctually strug-
ineffective or insufficient. adolescents and adults have a gle against noxious stimuli, such as the lar-
The anatomy of the paediatric airway cre- cylindrical-shaped rather than a funnel- yngoscopic blade and endotracheal tube
ates unique considerations during intuba- shaped airway, and they do require insertion. This optimises the chances of
tion as compared to adult intubation. cuffed endotracheal tubes to help secure visualising the vocal cords. Second, the
Specifically, the differences are as follows: the tube and prevent an air leak. induction agents affect the following auto-

A paediatric patient has a relatively

A paediatric patient has large adenoidal nomic responses.

larger tongue, making visualisation of

tissue and relatively small nares. Bradycardia patients less than 5 years
Consequently, nasotracheal intubation old have a higher risk for
the vocal cords more difficult. Hence,
in the paediatric population is bradyarrhythmias with direct
preintubation positioning of the patient
technically difficult and has a high laryngoscopy because of vagal nerve
plays a crucial role in TI success.
complication rate from traumatic
A paediatric patient has a wider and stimulation. Consequently, they may
bleeding, aspiration, and oesophageal require atropine prophylactically in their
floppier epiglottis. Thus, in neonates and
intubation. These intubations also RSI drug regimen.
infants, use a straight laryngoscopic
blade to achieve the best visualisation of
generally take a longer time to perform Tachycardia and hypertension
and require a patient who is awake and induction medications attenuate this
the vocal cords. The blade moves the
co-operative. The orotracheal route is catecholamine response to laryngoscopy
posteriorly-hanging epiglottis anteriorly,
the preferred approach. and intubation.
exposing the vocal cords.

479
23.4 ENDOTRACHEAL TUBE AND TRACHEAL INTUBATION

Gag reflex patients requiring

emergency intubation benefit from RSI
medications because patients are
assumed to have a full stomach, the
contents of which may regurgitate
during intubation. By blunting the gag
reflex using RSI, the risk of oesophageal
reflux and pulmonary aspiration
decreases. The process of RSI includes
Fig. 23.4.3 Endotracheal tubes, uncuffed (top)
cricoid pressure, which also limits
and cuffed (bottom).
regurgitation and aspiration.

The advantages of RSI agents, however,

must be balanced against the primary disad- Fig. 23.4.1 Curved (top) versus straight  Strong adhesive tape for securing the
(bottom) laryngoscopic blades. endotracheal tube.
vantage of persistent apnoea after a failed
intubation. The practitioner must be aware  Bite block or oropharyngeal (OP)
that when patients receive these drugs, airway.
unsuccessful intubations may cause the
patient to drop their oxygen saturation Epiglottis
Trachea
precipitously. Preparation
Because many pieces of equipment require
preparation, the SOAP ME mnemonic is a
Indications helpful checklist reminder for the provider
Cardiopulmonary arrest. (Table 23.4.1).
Respiratory failure or obstruction.
Excessive work of breathing refractory Laryngoscope There are two types of
to BM ventilation. A
blades to visualise the vocal cords straight
Loss of the gag reflex. and curved. For the neonate or infant, use a
Need for prolonged ventilation or straight blade to more easily visualise the
hyperventilation. Vallecula anteriorly located vocal cords. For older
Epiglottis
patients, whose anatomy more resembles
Trachea
that of adults, use either a straight or a
Contraindications curved laryngoscopic blade.
Additionally, the laryngoscopic blades
Adequate response to bag mask (BM) come in different sizes. To determine the cor-
ventilation with anticipated short
rect size to use for a specific patient, either
requirement for assisted ventilation.
(1) use the length-based resuscitation tape
Structural abnormalities, such as a large
tongue haematoma, or massive facial
injuries that require a tracheostomy or B Table 23.4.1 Preparing the equipment
cricothyrotomy. SOAP ME mnemonic
Fig. 23.4.2 Insertion of laryngoscopic blades.
Functioning tracheostomy.
Laryngoscopes are available with either a straight 1. Suction turn the suction apparatus on and
blade end (A) or a curved blade end (B). The straight test the suction catheter.
2. Oxygenation equipment test the self-
laryngoscopic blade should slide just under or inflating reservoir bag and bag mask (BM)
Equipment posterior to the epiglottis and elevate the tongue setup.
and epiglottis as a unit to visualise the vocal cords. 3. Airway equipment select the appropriately
Cardiopulmonary and oxygen saturation Because of their floppy and large epiglottis, sized endotracheal tube and insert a stylet.
Check the endotracheal tube cuff integrity
monitor. neonates and infants less than 5 years old require with a syringe, if applicable. Also obtain tube
this straight blade for better visualisation of the
Rigid-tip suction catheter. cords. For older patients, both straight and curved
sizes slightly larger and smaller than
expected, in case of unexpected anatomy.

BM and self-inflating bag. blades are acceptable. When the curved Obtain laryngoscopic blades slightly larger
Laryngoscope (Figs 23.4.1, 23.4.2). laryngoscopic blade is used, the blade should rest in
and smaller than anticipated, and test the
blade lights. Finally, check the rescue airway
Endotracheal tube (Fig. 23.4.3). the vallecular space just anterior to the epiglottis devices in case of a failed intubation.
Endotracheal tube stylet. and posterior to the base of the tongue. Lifting the 4. Pharmacological agents prepare rapid
sequence intubation drugs.
laryngoscope up towards the ceiling elevates the
Syringe for cuffed endotracheal tube. vallecular space and the tongue as a unit, which
5. Monitoring equipment turn on the
 Device for confirmation of intubation indirectly lifts the epiglottis anteriorly to allow
cardiopulmonary monitor, oxygen saturation
monitor, and tracheal confirmation device.
(Chapter 23.5). visualisation of the vocal cords.

480
23.4 ENDOTRACHEAL TUBE AND TRACHEAL INTUBATION
23


COMMON PROCEDURES
Table 23.4.2 Laryngoscope size selection Suction any excessive secretions or
gastric contents that are obscuring
Age Blade type and size adequate visualisation of the epiglottis
Premature infant Straight blade 0 and vocal cords.
Newborn to 2 years Straight blade 1
 Once the vocal cords are identified,
use the right hand to insert the
26 years Straight blade 2
endotracheal tube into the right side of
612 years Straight blade 2, Curved blade 2 the mouth, aiming between the vocal
Adolescent Straight blade 2 or 3, Curved blade 3
cords.
 Watch the tube actually pass
between the vocal cords and enter
(Chapter 23.1) or (2) use an age-based table neutral position of the head and neck by the trachea. The best way to
(Table 23.4.2). cupping the patients ears with both determine whether a TI is successful
hands (Fig. 23.4.4). (i.e. not an oesophageal intubation)
Endotracheal tube Cuffed endotracheal Pre-oxygenate the patient by BM is by direct visualisation. Other
tubes serve to prevent an air leak around the ventilation with 100% oxygen for 1 confirmatory modalities may
tube. Because of the relatively funnel- 2 minutes. Be aware that the smaller the provide false and misleading
shaped airway in patients less than 8 years child, the faster the rate of oxygen information.
old, an uncuffed tube should be used. desaturation during the intubation Insert the tube to the appropriate length
Conversely, for older patients and adults, procedure. If time permits, fully pre- (Table 23.4.4).
cuffed tubes are essential because of their oxygenate the patient. Inflate the cuff, if applicable.
cylindrical-shaped airway. Without a balloon
During preoxygenation and intubation, Attach the oxygen bag to the
cuff, this airway shape is susceptible to have an assistant apply gentle posterior endotracheal tube and ventilate the
significant air leaks. After choosing a cuffed pressure to the cricoid cartilage. This patient, watching for equal chest rise
or uncuffed endotracheal tube, select the reduces oesophageal reflux of gastric with each bag insufflation.
appropriate size of the tube for the patient contents and the risk of aspiration. Listen for equal breath sounds in
(Table 23.4.3). The tube sizes range from Administer RSI medications and bilateral axilla and the absence
1.0 to 9.0, representing the tube inner diam- continue BM ventilation until of breath sounds in the
eter in millimetres. neuromuscular paralysis occurs. epigastrium.
Grasp the laryngoscopic handle with Check for successful placement of
the left hand and engage the blade the endotracheal tube by a confirmatory
Positioning so that the attached light bulb device (see Chapter 23.5).
The patient should be placed in a supine, illuminates. Obtain a postintubation chest
neutral sniffing position. For infants and Gently insert the laryngoscope, starting radiograph to check for successful tube
small children, placing a small towel roll from the patients right side of the placement and adequate depth of tube
under the shoulders prevents the occiput mouth and sweep the tongue towards insertion. The tube should end 12 cm
from hyperflexing the neck and occluding the left. above the carina.
the airway. Continue inserting the blade deep while Secure the tube in place with adhesive
lifting the laryngoscope anteriorly and tape.
inferiorly, trying to visualise the vocal Insert a bite block or OP airway to
Procedure cords. prevent the patient from chewing off the
endotracheal tube.
Provide in-line spinal immobilisation, if
necessary. Have an assistant stand at
the side of the bed, and maintain the

Table 23.4.4 Determining the correct

Table 23.4.3 Endotracheal tube size endotracheal tube insertion depth
selection
Option 1 insert the endotracheal tube (3
Option 1 use a length-based resuscitation endotracheal tube size) centimetres as
tape. marked at the patients teeth. For instance, a
Option 2 the inner diameter of the 5.0 tube correlates with a 15 cm insertion
endotracheal tube should equal the patients depth.
fifth fingernail. Option 2 there exists a black double line
Option 3 the tube size should equal the sum drawn circumferentially about 25 cm from
of the patients age in years divided by four the tubes distal tip. Insert the endotracheal
and added to four. For instance, an 8-year-old tube until this double line rests at the level of
child requires a (84) 4 tube, or a 6.0 tube. the vocal cords.
Fig. 23.4.4 Spinal immobilisation by assistant.

481
23.4 ENDOTRACHEAL TUBE AND TRACHEAL INTUBATION

During preoxygenation and intubation, during intubation is inserting the

Complications applying cricoid pressure too aggressively endotracheal tube too deep, resulting in
Traumatic injury to lips, teeth, may not only occlude the oesophagus but a right mainstem intubation.
oropharynx, larynx, vocal cords and also the airway. Also, because of the short trachea in the
oesophagus While looking for the vocal cords with the paediatric population, the endotracheal
Incorrect insertion of tube into laryngoscope, a common mistake is to use tube may easily dislodge from its position
oesophagus or intracranially the patients teeth as a fulcrum for the in the airway. Be vigilant about securing
Emesis and aspiration blade. Beware of fracturing the patients the tube at all times, but especially during
Increased intracranial, intraocular, and teeth. transport or with any movements.
intragastric pressures To help visualise a patients vocal cords, Frequently recheck its position.
Cervical spinal injury which are being obscured by a floppy
Dysrhythmia epiglottis, use the BURP manoeuvre
Hypertension or hypotension (Fig. 23.4.5).
Hypoxia and/or hypercarbia Because of the short trachea in the
Pain and anxiety paediatric population, a common mistake Further reading
Dislodgement of endotracheal tube Chameides L, Hazinski MF, editors. Paediatric advanced
life support. Dallas, TX: American Heart Association;
during movement 1997.
Dieckmann RA, Brownstein DR, Gausche-Hill M, editors.
Paediatric education for prehospital professionals. Sudbury,
MA: Jones & Bartlett Publishers; 2000.
Tips Gnauck K, et al. Emergency intubation of the paediatric
medical patient: Use of anesthetic agents in the ED. Ann
Up Back
Emerg Med 1994;23(6):12427.
Equipment failure is an unnecessary Knill RL. Difficult laryngoscopy made easy with a BURP. Can J
complication with potentially Anaesth 1993;40(3):27982.
Lee BS, Gausche-Hill M. Paediatric airway management. Clin
devastating consequences. Be sure to test Paediatr Emerg Med 2001;2(2):91106.
Right
the functionality of the suction McAllister JD, Gnauck KA. Rapid sequence intubation of the
paediatric patient. Fundamentals of practice. Paediatr Clin
apparatus, the brightness of the N Am 1999;46(6):124984.
laryngoscope light bulb, and the integrity Moynihan RJ, Brock-Utne JG, Archer JH, et al. The effect of
cricoid pressure on preventing gastric insufflation in infants
of the endotracheal tube cuff prior to and children. Anesthesiology 1993;78(4):6526.
intubation. Fig. 23.4.5 Improving vocal cord Stoelting RK. Circulatory changes during direct laryngoscopy
and tracheal intubation: Influence of duration of
Anticipating a failed initial intubation visualisation BURP manoeuvre. Using your laryngoscopy with or without prior lidocaine.
reduces the complication rate in tracheal right hand (non-laryngoscope hand), manipulate Anesthesiology 1977;47(4):3814.
intubation. Prepare variously-sized the external cricoid cartilage with Back (posterior), Thompson A. Paediatric emergency airway management.
Up (superior), and Rightward Pressure. This In: Dieckmann RA, et al., editors. Paediatric
laryngoscopes, slightly larger and smaller emergency and critical care procedures. St Louis, MO:
essentially moves the vocal cords towards the Mosby; 1997.
endotracheal tubes, and failed-airway right and out from under the overhanging Tintinalli JE, Claffey J. Complications of nasotracheal
alternatives at the bedside. epiglottis. intubation. Ann Emerg Med 1981;10(3):1424.

482
 23

COMMON PROCEDURES
23.5 Confirmation of intubation
Michelle Lin

Background significant airflow resistance suggests Tracheal intubation. With each

an oesophageal intubation, because exhalation and inhalation, a
Confirming correct placement of the endotra- of oesophageal wall collapse. The characteristic waveform, showing the
cheal tube is crucial because of the high mor- large syringe or self-inflating bulb will rise, plateau, and fall of CO2 levels,
bidity and mortality of an inadvertent and not fill with air to full capacity. confirms correct positioning of the
unrecognised oesophageal intubation. The
Tracheal intubation. When air is tube in the trachea.
most accurate means of assuring success is aspirated from the endotracheal tube, Oesophageal intubation. With each
by visualising the endotracheal tube between there will be no resistance to 30 exhalation and inhalation, an
the vocal cords and into the trachea. A suc- 40 mL of airflow if the tube indeed unchanging, flat waveform
cessful tracheal intubation (TI) is also highly lies in the trachea. demonstrates the absence of CO2
likely when condensation appears in the End-tidal colorimetric capnometry and thus the misplacement of the
plastic endotracheal tube during assisted (Fig. 23.5.2). The capnometer attaches endotracheal tube into the
ventilation, when breath sounds are heard to the proximal end of the endotracheal oesophagus.
in both axillae but not in the epigastrium, tube and detects the presence of CO2
when the pulse oximetry is 100%, and when within the tube. The capnometer will
the chest cavity rises and falls with positive- display a yellow (CO2 present) or purple Indications
pressure ventilation. At times, however, these (CO2 absent) colour in the indicator
findings can be equivocal, especially in chil- All intubated patients.
window, which generally correlates with
dren. The clinical exam is notoriously decep- a tracheal or oesophageal intubation,
tive in determining correct endotracheal tube respectively. Multiple studies find that a Contraindications
placement, and it is imperative to use at least yellow colour change has a 100%
one of the following techniques for every positive predictive value for correct
Do not use an adult-sized colorimetric
intubated patient. capnometry device on intubated patients
endotracheal tube placement. When
weighing less than 15 kg, because the
Oesophageal aspiration. An oesophageal used for a poorly perfused patient (e.g.
device adds a significant dead-space
aspirator is a large syringe or a self- cardiac arrest), however, there will often
volume for the neonate to rebreathe CO2.
inflating bulb (Fig. 23.5.1), which attaches be no yellow colour change because of
Instead, attach a paediatric-sized
to the proximal end of the endotracheal expected low CO2 levels. This is the
capnometer for these patients.
tube. The aspirator differentiates between primary limitation when using this
modality. Colorimetric capnometry is not
Use caution with oesophageal aspiration
an oesophageal versus a tracheal
on patients weighing less than 20 kg.
placement because the oesophagus is a commonly used in hospital. Table 23.5.1
Several studies, however, suggest that
collapsible structure under negative provides a mnemonic to help remember
pressure, while the trachea is not. the colour scheme.
Successful air aspiration is highly  Digital capnography (Fig. 23.5.3). This
associated with trachea placement. It has latest technology continuously detects
been determined that the oesophageal and displays the partial pressure of CO2
aspiration modality has a sensitivity of at the proximal end of the endotracheal
99% and specificity of 100% in confirming tube. In adult cardiac arrest patients, a
endotracheal tube placement in patients pCO2 < 5 mmHg correlates with an
weighing more than 20 kg. extremely poor prognosis. Digital
Oesophageal intubation. When air is capnography is the standard for A
aspirated from the endotracheal tube, confirmation of intubations.
PCO2 Exhalation Inhalation

Time
B

Fig. 23.5.1 Oesophageal bulb (left) and Fig. 23.5.3 Digital capnography instrument
aspirator (right). Fig. 23.5.2 Colorimetric capnometer. (A) and typical waveform reading (B).

483
23.5 CONFIRMATION OF INTUBATION

oesophageal aspiration may still be safe If the endotracheal tube is incorrectly

at a lower weight limit of 4 kg, because positioned in the oesophagus, there will
Tips
the resting lung volume is still greater be resistance during aspiration. Reposition Check tube placement after every
than 50 cm3, which is the typical volume the tube under direct laryngoscopy. intubation and after any patient
in an oesophageal aspirator. movement, such as during transport,
because the endotracheal tube can
Equipment easily shift into the oesophagus.
End-tidal colorimetric
Use a paediatric-sized colorimetric
Oesophageal aspiration syringe or bulb capnometer
capnometer for patients less than 15 kg
(see Fig. 23.5.1). Attach the capnometer in series with the
to decrease the volume of dead space
End-tidal colorimetric capnometer entire ventilation system between the
in the ventilatory circuit.
endotracheal tube and the bagvalve
(see Fig. 23.5.2).  When intubating a poorly perfused
 Digital capnography (see Fig. 23.5.3). device. Ventilation can continue through
patient, using an oesophageal
the capnometer.
aspirator is more accurate than the
After ventilating the patient over six
end-tidal colorimetry capnometer or
times, check the colour of the
Preparation and positioning digital capnography, because the
capnometer indicator window during the
endotracheal tubes CO2 level is
Prepare at least one confirmation device exhalation phase. Initially purple in
for bedside use before the patient is extremely low. In this case, the latter
colour when attached, exhalation phases
intubated so that immediate instruments, which rely on CO2 levels,
correlate with a yellow colour change.
may only give equivocal confirm of
confirmation is available.  Recall that a yellow colour confirms
endotracheal tube placement. The
Specific for the colorimetric capnometer, correct TI because it indicates the
open the packaging for this disposable oesophageal aspirator, however,
presence of CO2.
relies on the anatomical differences
device. Check that the unit is dry and Secure the capnometer in place for
initially displays a purple colour in the between the collapsible oesophagus
continuous monitoring while the patient
indicator window to ensure proper and the semi-rigid trachea, independent
transfers to the intensive-care unit.
functioning. of expired CO2 levels. Thus, with
low perfusion states, the aspirator
Specific for digital capnography, be sure
that the device is plugged in and turned on. provides a more accurate
Digital capnography
Blowing across the capnography tubing Connect the digital capnography tubing
confirmation of correct endotracheal
tests the monitoring and sensing function. tube placement.
to the distal end of the bagvalve device.
The air in the endotracheal tube is now
contiguous with the air in this tubing.
Procedure Ventilate the patient and observe the
live-time waveforms of CO2 return Further reading
Oesophageal aspiration Anderson KH, Schultz-Leban T. Oesophageal intubation can
during exhalation. The absence of CO2
Expel all the air out of the oesophageal
indicates a failed intubation.
be undetected by auscultation of the chest. Acta
Anaesthesiol Scand 1994;38:5802.
syringe or bulb, and attach it to the Bhende MS, Thompson AE, Orr RA. Utility of an end-tidal
proximal end of the endotracheal tube carbon dioxide detector during stabilization and
transport of critically ill children. Paediatrics 1992;89:
just after intubation. Inadvertently 10424.
instilling air into the endotracheal tube Bhende MS, Thompson AE, Orr RA. Validity of a disposable
Complications end-tidal CO2 detector in verifying endotracheal tube
with the syringe or bulb may cause a placement in infants and children. Ann Emerg Med
falsely reassuring aspiration result. There are no direct complications to the 1992;21:1425.
Burnett YL, et al. Efficacy of the self-inflating bulb in verifying
Slowly aspirate air over 5 seconds. patient by using these devices. Indirectly, tracheal tube placement in children. Anesth Analg
 If the endotracheal tube is correctly however, the misinterpretation of their find- 1995;80:S63 [abstract].
Dieckmann RA, Brownstein D, Gausche-Hill M, editors.
positioned in the trachea, there will be ings may cause the practitioner to incorrectly Paediatric education for prehospital professionals. Sudbury,
no resistance during aspiration. change patient management. MA: Jones & Bartlett Publishers; 2000.
Marley CD Jr, et al. Evaluation of a prototype oesophageal
detection device. Acad Emerg Med 1995;2(6):5037.
Sharieff GQ, et al. The self-inflating bulb as an oesophageal
Table 23.5.1 End-tidal colour capnometer interpreting the colour detector device in children weighing more than twenty
kilograms: A comparison between two techniques. Acad
If you see Yellow, then Yes there is CO2 return. At least 20 mmHg of CO2 is detectable. Several Emerg Med 2003;41(5):6239.
studies show a 100% positive predictive value of a yellow indicator colour with a tracheal space Ward KR, Yealy DM. End-tidal carbon dioxide monitoring in
intubation (rather than an oesophageal). emergency medicine. Part 1. Basic principles. Acad Emerg
If you see Purple, then there is a Problem - there is no CO2 return. A TI is unlikely, unless the patient has Med 1998;5(6):62836.
extremely poor perfusion, such as during asystole. If a purple colour is displayed, less than 4 mmHg of Wee MYK. The oesophageal detector device: Assessment of a
CO2 is detectable. new method to distinguish oesophageal from tracheal
If you see an intermediate Tan colour, then Think about it, because the tube could be sitting in the intubation. Anaesthesia 1998;43(1):279.
trachea or the oesophagus. The capnometer is sensing between 415 mmHg of CO2. This equivocal Zideman D, et al. Airways in paediatric and newborn
finding requires direct laryngoscopy re-visualisation of the tubes placement to help determine placement. resuscitation. Ann Emerg Med 2001;37(4 Suppl):
S12636.

484
 23

COMMON PROCEDURES
23.6 The surgical airway
Judith Klein

small. For this group, needle cricothyroidot- presence of massive nasopharyngeal

Background omy is easier than surgical cricothyroidot- haemorrhage, large tongue haematoma,
The paediatric airway is distinct from that of omy. While the needle technique will laryngeal spasm, laryngeal stenosis, or
the adult. The epiglottis is floppy and the provide emergency oxygenation, it may obstruction of the airway by a foreign body
larynx is shorter, more anterior, and more not provide adequate ventilation, especially that cannot be removed.
cephalad. The larynx is narrower and there- over time. Even a jet ventilator, a rescue
fore more prone to obstruction by oedema, device that attaches to the needle and pro-
scarring, fluids, or foreign bodies. The carti- vides intermittent bursts of high-pressure
Contraindications
lage of the larynx is also softer, which makes oxygen to simulate the normal respiratory
palpation of landmarks on the skin more dif- cycle, may not allow for adequate ventila- Presence of a secure airway.
ficult. The cricoid ring is the only spot where tion and clearance of CO2. Traumatic destruction of the cricothyroid
cartilage encircles the paediatric trachea, In children 5 years and over, surgical cri- membrane.
and it is also the narrowest portion of the air- cothyroidotomy is easier and more effective, Transection of the trachea with retraction
way (Fig. 23.6.1). because it creates a larger conduit and of the distal segment.
The vast majority of paediatric airway allows both immediate oxygenation and Surgical cricothyroidotomy is relatively
emergencies can be readily managed with ventilation via a bagvalve device. Tracheot- contraindicated in children under the age
bagvalvemask (BVM) ventilation and/or omy in a young child is extremely difficult to of 5 years. In this group, perform needle
tracheal intubation (TI). Rarely, a child pre- perform emergently and is rarely indicated cricothyroidotomy.
sents with complete airway obstruction or in the emergency derpartment.
anatomic abnormalities, and ventilation with
BVM or TI is impossible. In these situations, a Needle cricothyroidotomy
surgical airway may be necessary to provide Indications
life-saving oxygenation and ventilation to Equipment
Failure to ventilate with BVM and/or TI. Table 23.6.2 lists the equipment for this pro-
avoid anoxic brain injury. Surgical airways in
Obstructed or disrupted larynx. cedure, which can be divided into three
children include: needle cricothyroidotomy;
surgical cricothyroidotomy; and, rarely, trache- Table 23.6.1 lists situations in which these categories:
indications occur. The most common circum-
ostomy. Placing a needle, catheter or tube Oxygen source. A transtracheal jet
directly into the trachea through the neck will stances in which intubations fail are the
ventilator device is ideal. It provides
temporarily relieve airway obstruction or high-pressure 100% oxygen (50 psi) in
bypass anatomic abnormalities. Table 23.6.1 Situations requiring intermittent bursts, which allows more
In children under the age of 5 years, the cricothyroidotomy time for passive CO2 exhalation
membrane between the thyroid cartilage Trauma (Fig. 23.6.2). Alternatively, use a bag
and the cricoid cartilage, the cricothyroid Laryngeal fracture valve device connected to 15 L min1 of
Airway burns
membrane (see Fig. 23.6.1), is extremely Massive haemorrhage wall oxygen.


Foreign body
Subglottic stenosis (late)
Adaptors. The jet ventilator device
Burn contractures (late) connects directly to the catheter at the
A B
neck. To use the bagvalve system,
Congenital abnormalities
Laryngeal atresia/stenosis/clefts
Tracheoesophageal fistula
PierreRobin syndrome Table 23.6.2 Equipment for needle
TreacherCollins syndrome cricothyroidotomy
P A P A
Cervical spine abnormalities Oxygen source
Thyroid Trisomy 21 syndrome Jet ventilator (50 psi)
cartilage KlippelFeil malformation Bagvalvemask device attached to
Torticollis 15 L min1 wall oxygen

Cricoid Adaptor
Inflammatory/infectious
Severe croup 3 mm endotracheal tube adaptor
Epiglottis 3 cm3 syringe barrel and 8.0 mm
Bacterial tracheitis endotracheal tube
Fig. 23.6.1 Locating the cricoid membrane. Retropharyngeal abscess
Cricoarytenoid arthritis Catheter
Comparison of adult (A) and paediatric (B)
14-gauge catheter over a needle
airways. Note funnel shape of paediatric airway Laryngeal spasm 3 cm3 syringe
with narrowest portion at cricoid ring.

485
23.6 THE SURGICAL AIRWAY

passive exhalation. If a bagvalve

device is used, provide 20 breaths per
minute with the same I:E ratio.

Complications
Bleeding, but it is rarely massive.
Inappropriate placement of a needle can
A Cricothyroid membrane cause injury to:

the larynx and vocal cords

great vessels of the neck

nerves

oesophagus.
High-pressure oxygen can also cause
significant barotrauma resulting in
extensive subcutaneous emphysema,
Fig. 23.6.2 Transtracheal jet ventilator device. pneumothorax, and
B pneumomediastinum.
attach a 3-mm paediatric tracheal tube Fig. 23.6.4 Inserting the needle through the
adapter with one end connected to the membrane.
bagvalve device and the other to the
Tips
end of the cricothyroidotomy catheter. Needle cricothyroidotomy is a
Alternatively, set up a ventilation system Stabilise the cartilage using the thumb temporising measure that may provide
and index finger of the non-dominant enough ventilation for about 30 minutes.
with a 3-mL syringe and an 8-mm
hand. Immediately consider other airway
tracheal tube that attaches to the
bagvalve device (Fig. 23.6.3).  Insert a 14G over-the-needle catheter options.
 Needle/cannula. A 14G over-the-needle attached to a 3-cm3 syringe just superior Remember to allow adequate time for
to the cricoid cartilage (Fig. 23.6.4B). passive exhalation to prevent
catheter.
Angle the catheter caudally at about a barotrauma.
45
angle to the skin. Do not let go of the catheter until an
Preparation
Since children have relatively large Apply continuous backpressure to the alternative airway is established.

occiputs, place a shoulder roll syringe barrel. A rush of air into the
transversely to prevent hyperflexion of syringe confirms entry into the trachea.
the neck and improve visualisation of Alternatively, fill the syringe with saline
the anterior neck anatomy. to use as an indicator. The marker of Surgical cricothyroidotomy
If time allows, prepare the skin with entry into the trachea is the presence of
Equipment
povidone-iodine solution. air bubbles in the syringe.
Table 23.6.3 lists the equipment for surgical
Advance the catheter into the trachea
cricothyroidotomy.
Procedure and remove the needle and syringe. Do
Several commercial kits contain all equip-
Palpate the hyoid bone high in the neck not let go of the catheter until it is
ment for a cricothyroidotomy tube using the
and move caudally to identify the secured or an alternative airway is
guidewire or Seldinger technique. Other use-
thyroid and cricoid cartilages obtained.
ful instruments include a tracheal hook and
(Fig. 23.6.4A). If a jet ventilator device is available,
a tracheal dilator.
attach it directly to the catheter.
If this device is not available, attach a
3 3

21/2211//2

2 2

11/1211//2

1 1

1/21//2

bagvalve system to high-flow Table 23.6.3 Equipment for surgical

OR (15 L min1) wall oxygen. Place an cricothyroidotomy
adaptor between the catheter and the Frazier tip suction
Scalpel
bagvalve device. Either attach a 3-mm Haemostat
endotracheal tube adaptor directly Tracheostomy or endotracheal tube
Oxygen delivery system
1 1

1/21//2

onto the catheter or attach an 8-mm Tracheostomy suction

OR endotracheal tube in a 3-mL syringe
barrel. Optional
Povidone-iodine solution
Fig. 23.6.3 Using a 3.0-mm tracheal tube or an
With a jet ventilator, administer 100% Lidocaine with adrenaline (epinephrine)
Tracheal hook
8-mm tracheal tube into a 3-mL syringe hub as a oxygen at 20 bursts per minute with an Tracheal dilator
connector. approximate I:E ratio of 1:4 to allow for

486
23.6 THE SURGICAL AIRWAY
23

COMMON PROCEDURES
Preparation Make a horizontal incision in the Make a stab incision over the needle
Expose the thyroid and cricoid cricothyroid membrane, carefully through the skin and membrane.
cartilage by placing a roll under avoiding inserting the scalpel too far Insert the catheter and dilator over the
the childs shoulders. and lacerating the posterior aspect of guidewire into the trachea and remove
If time permits, prepare the skin with the larynx. both the wire and the dilator.
povidone-iodine solution and infiltrate Insert the haemostat into the airway  Provide ventilation via a bagvalve
the skin with lidocaine with adrenaline next to the scalpel to hold the space and device.
(epinephrine) to provide local widen the opening.
anaesthesia and haemostasis. Use a tracheal hook, if available, to
Complications
maintain the opening and provide
traction cranially while inserting the
Bleeding.
Procedure Tube misplacement, resulting in hypoxia
tube caudally (Fig. 23.6.6).
Palpate the hyoid bone high in the neck and subcutaneous emphysema.
If a tracheostomy tube of the
and move caudally to identify the
appropriate size is not available, use an
Laryngeal, oesophageal, or neurovascular
thyroid and cricoid cartilages injury.
endotracheal tube of the same calibre.
(see Fig. 23.6.4A).
Trim the length of the tube to 3/2 of its
Barotrauma, which can result in
Stabilise the larynx by placing thumb pneumothorax or pneumomediastinum.
diameter in centimetres (e.g. 4.0 ETT
and forefinger of the non-dominant
(cut to 3/2  4 6 cm).
Significant late complications include
hand on either side of the thyroid voice change due to vocal cord damage
cartilage.
 Attach a bagvalve device and ventilate
and subglottic stenosis.
the patient. Do not let go of the tube
 Make a midline vertical incision in the
until it has been secured to the patients
skin from thyroid cartilage to cricoid
neck. Tips
cartilage (Fig. 23.6.5). Expose the
cricothyroid membrane via blunt The guidewire or Seldinger technique is an
If the cricothyroid membrane is not
visible with the initial vertical incision,
dissection with haemostats. alternative to surgical placement of a cri-
extend it.
cothyroidotomy tube.
Do not cut into the larynx blindly
Position the patient for surgical as this will increase the rate of
cricothyroidotomy. complications.
Identify the cricothyroid membrane and Have suction readily available for any
prepare the skin with povidone-iodine bleeding into the trachea.
solution. Avoid using too large a tube. This can
 Insert the needle mounted on a lead to laryngeal fracture.
syringe through the cricothyroid Size the catheter by using a length-based
membrane. resuscitation tape.
Angle caudally at 45 degrees to the skin If a child requires prolonged ventilation,
with the bevel of the needle up. establish a definitive airway (tracheal
Once air rushes into the syringe, pass the intubation or tracheostomy) as soon as
J-tipped guidewire through the needle possible.
A and remove the needle.

Further reading
Bower CM. The surgical airway. In: Dieckmann RA, Fiser DH,
Selbst SM, editors. Paediatric emergency and critical care
procedures. St Louis, MO: Mosby; 1997. p. 11622.
Granholm T, Farmer DL. The surgical airway. Resp Care Clin N
Am 2001;7(1):1323.
Mace SE. Cricothyrotomy and translaryngeal jet ventilation.
In: Roberts JR, Hedges JR, editors. Clinical procedures in
emergency medicine. 3rd edn. Philadelphia, PA: WB
Saunders; 1998. p. 5774.
Peak DA, Roy S. Needle cricothyroidotomy revisited. Paediatr
B Emerg Care 1999;15(3):2246.
Yealy DM, Plewa MC, Steward RD. An evaluation of cannulae
Fig. 23.6.5 Making the surgical incision in Fig. 23.6.6 Inserting the cricothyroidotomy and oxygen sources for paediatric jet ventilation. Am J
the skin. tube. Emerg Med 1991;9(1):203.

487
 23.7 Needle thoracostomy
Judith Klein

Needle evacuation of the air between the Suspected tension pneumothorax in an

Background visceral and parietal pleura and conversion intubated patient who is rapidly
Normally, the visceral and parietal pleura of the tension pneumothorax to an open deteriorating (e.g. hypotension,
are closely adherent to one another. How- pneumothorax will re-expand the lung and hypoxia).
ever, if either surface is violated, air enters improve venous return and cardiac output. Pulseless electrical activity (PEA) in the
into the potential space between visceral Needle thoracostomy is a temporising proce- setting of trauma or asthma.
and parietal pleura, creating a simple pneu- dure. Immediately after needle thoracostomy,
mothorax (Fig. 23.7.1). This typically occurs insert a chest tube to provide ongoing drain-
in the setting of blunt or penetrating age in a closed system (Chapter 23.8). The Contraindications
trauma. Occasionally, spontaneous pneumo- risk of not performing needle thoracostomy
thorax occurs, as with excessive air trapping in a child spiralling towards death from a ten- A stable child with a simple
in an asthmatic child. If enough air collects, a sion pneumothorax is far greater than the risk pneumothorax, not under tension.
tension pneumothorax can develop in which of performing the procedure in a child without There is no absolute contraindication to
a pneumothorax. needle thoracostomy in a child with
pressure in the space shifts the mediasti-
possible tension pneumothorax and
num, impedes venous return to the heart,
and decreases cardiac output (Fig. 23.7.2). shock. While preparing for open
thoracotomy in the emergency
Another pathophysiological mechanism for Indications
tension pneumothorax occurs when a department, placing a needle in the
patient with a simple pneumothorax is intu-
Blunt or penetrating trauma with signs of pleural space will not cause further
tension pneumothorax. deterioration.
bated positive pressure ventilation will
force air into the pleural space resulting in
Chest X-ray evidence of a tension If a skin infection is present at the
pneumothorax in a child in respiratory preferred needle site, select an
a tension pneumothorax. Tension pneumo-
distress for any reason. alternative location.
thorax can progress to shock and cardiopul-
monary arrest.
The typical clinical scenario for tension
pneumothorax is the child who experiences Equipment
a penetrating injury to the chest wall or 14-gauge catheter over a needle.
back. A sucking chest wound (open pneumo- 30-mL syringe.
thorax) may be present. The classic adult  Povidone-iodine solution.
clinical findings of unilateral decreased air
movement and trachea shift may not be
readily detectable in children. More com-
Preparation
monly, the clinical signs are tachypnea, oxy-
gen desaturation, retractions, flaring, Inspiration Inspect the anatomy of the ribs and
grunting and shock. Once tension pneumo- select an entry site above the rib
thorax develops, progression to shock and that avoids injuring the intercostal
A
cardiopulmonary arrest can be rapid. neurovascular bundle (Fig. 23.7.3).
Identify a site to insert the needle
over the rib.
 Position the child supine with the head
of the bed angled up at 30 degrees.
Have an assistant gently restrain the
conscious child.
Use a local and/or intravenous
analgesic if time allows and if the child
is conscious.
Identify the 2nd intercostal space
Expiration
(above the 3rd rib) at the
B midclavicular line. This space is ideal
Fig. 23.7.2 Tension pneumothorax. since air in the pleural space rises
Fig. 23.7.1 Simple pneumothorax. (A) Inspiration, (B) Expiration. and typically collects toward the top

488
23.7 NEEDLE THORACOSTOMY
23

COMMON PROCEDURES
A

Parietal pleura
B

Fig. 23.7.4 Inserting the thoracostomy needle.

Vessels and
nerves
Remove the syringe and the needle, and Fig. 23.7.5 Treating a sucking chest wound.
leave the catheter in place
(Fig. 23.7.4B).
Place a chest tube immediately. diaphragm penetration, bowel
penetration, haemopericardium or
If there is an open connection between the
coronary vessel.
chest wall and the pleural space from a
penetrating injury an open pneumothorax
If the catheter becomes occluded and
fails to drain air, the tension
Fig. 23.7.3 Anatomy of the ribs and intercostal or sucking chest wound cover with a petro-
pneumothorax can reoccur.
neurovascular bundle. latum gauze, extending 68 cm from the
wound edges on all sides and taped on three
sides, to stop further air entry during inspira-
of the lungs. A lateral approach is tion (Fig. 23.7.5). Leave one side free to
Tips
an alternative, but may have more allow for the ongoing egress of air from
complications, including lung the pleural space. Perform tube thoracostomy as soon as
penetration and subsequent possible in the setting of suspected
adhesions. tension pneumothorax. This relieves
Prepare the skin of the entry site Complications tension and creates an open
with povidone-iodine solution. pneumothorax.
Performing needle thoracostomy in a Always perform tube thoracostomy after
patient without a pneumothorax who is needle thoracostomy to prevent tension
subsequently intubated can cause a pneumothorax from developing.
pneumothorax or injury to the lung
Procedure parenchyma. Under positive pressure
Attach the catheter over the needle ventilation, this pleural penetration can
to the 30-mL syringe. result in an ongoing air leak, which can
Insert the catheter into the chest then lead to a tension pneumothorax. Further reading
Barton ED, Epperson M, Hoyt DB, Rosen P. Prehospital needle
wall at a 90 degree angle above Always place a chest tube after needle aspiration and tube thoracostomy in trauma victims: A six-
the 3rd rib (2nd intercostal space) thoracostomy to prevent this year experience with aero medical crews. J Emerg Med
1995;13(2):15563.
at the midclavicular line complication. Bliss D, Silen M. Paediatric thoracic trauma. Crit Care Med
(Fig. 23.7.4A). Placing the needle below the rib can 2002;30(11 Suppl):S40915.
 Provide back pressure on the syringe. cause laceration of an intercostal artery Wright SW. Tube thoracostomy. In: Roberts JR, Hedges JR,
editors. Clinical procedures in emergency medicine. 3rd ed.
Once there is a free flow of air in the and, subsequently, a haemothorax. Philadelphia, PA: WB Saunders; 1998.
Zimmerman KR. Needle thoracostomy. In: Dieckmann RA,
syringe, continue to pull back on the Placing the catheter lower in the chest or Fiser DH, Selbst SM, editors. Paediatric emergency and
plunger to evacuate the air. closer to the mediastinum can result in critical care procedures. St Louis, MO: Mosby; 1997.

489
 23.8 Tube thoracostomy
Ronald A. Dieckmann

adults and children are the need for more

Background meticulous patient preparation and smaller Preparation
Traumatic pneumothorax and haemothorax catheters in children. Sometimes, detection Identify the presence of pneumothorax/
are the most frequent paediatric indications of pleural air or fluid is more difficult in chil- haemothorax and the affected side by
for tube thoracostomy. Pneumothorax is a dren, requiring computerised tomography clinical examination and/or chest X-ray.
serious sequela of major chest trauma in (CT) imaging to localise and quantify. Establish secure vascular access and
children and may occur as a result of blunt In the setting of tension pneumothorax, apply cardiac monitor and pulse
injury without rib fractures or chest pene- needle thoracostomy (Chapter 23.7) may oximetry.
tration. Sometimes, spontaneous pneumo- precede tube thoracostomy, but tube thora-  If the child is conscious, provide
thorax occurs without trauma, from rapid costomy must always follow needle thora- parenteral sedation/analgesia.
increase in intraluminal pressure, especially costomy. Stable, small pneumothoraces not If the child is stable, consider an
with patients who have pre-existing broncho- under tension (e.g. <20%), are usually man- intercostal nerve block.
pulmonary disease. Air or fluid in the pleural aged with observation alone, without a Explain the procedure to the verbal child.
space can cause significant impediments to chest tube. There is an increasing tendency Place a nasogastric tube to decompress
oxygenation/ventilation and drastically to manage even moderate sized pneu- the stomach, when there is abdominal
reduce cardiac output, usually when the hae- mothoraces without tension with observa- distension.
mothorax is large or the pneumothorax is tion only. Massive haemothorax occurs Place the child in the supine or upright
under tension. Tension pneumothorax and when a large vascular structure under sys- position with the arm raised on the
large haemothorax are potentially lethal temic pressure bleeds into the pleural space. affected side.
emergencies that require immediate chest This is usually the internal mammary artery  Identify the entry site (Fig. 23.8.1), on
decompression. Rarely, large pleural fluid col- or an intercostal artery. In this setting, tube the middleanterior axillary line
lections from non-traumatic aetiologies, such thoracostomy is the only treatment likely to between the fourth and fifth rib, at the
as empyemas, that are causing respiratory salvage the child. Occasionally, recirculation nipple level.
distress, require tube thoracostomy for or autotransfusion of evacuated blood from  Drape the area and prepare the area
drainage. the pleural space is indicated to treat mas- widely between the fourth and sixth ribs
The typical clinical presentation for ten- sive haemothorax. In the overwhelming with povidone-iodine solution.
sion pneumothorax and/or large hae- majority of children with significant chest-  Infiltrate the entry site above the sixth
mothorax is the child who experiences a wall penetrations, tube thoracostomy is rib generously with lidocaine in
penetrating injury to the chest wall or back. the only treatment indicated. conscious patients, and also infiltrate
A sucking chest wound may be present. along the insertion site superiorly to the
Sometimes, these conditions result from fourth interspace.
penetrations below the nipple line or tip of Select a properly-sized standard chest
Indications
the scapula that may also involve penetra- tube or pigtail catheter (Table 23.8.2).
tion into the abdomen, or from remote pene- Tension pneumothorax.
trations with gunshots that traverse multiple Haemothorax.
anatomic regions. Less frequently, blunt Rapid pleural fluid accumulation with Table 23.8.1 Chest tube tray
trauma is the mechanism. The classic adult respiratory distress.
Kelly clamps (2)
clinical findings of unilateral decreased air Mayo scissors
movement and trachea shift are rarely pres- Suture scissors
Needle holder
ent in children. More commonly, the clinical Scalpel
signs are tachypnoea, oxygen desaturation, Contraindications Forceps
Silk suture
retractions, flaring, grunting and shock. Need for immediate open thoracotomy. 10 mL syringe
Tube thoracostomy is the insertion of an 25-gauge needle
20-gauge needle
intrapleural tube to evacuate air and/or Sterile towels
fluid from the pleural space. The procedure 4
4 sterile gauze
is a time-honoured evacuation technique
Equipment Vacuum device
Drainage apparatus with water seal
for all age patients, and it may be life saving. Chest tube tray (Table 23.8.1). Plastic connectors (straight and Y type)
The technique is effective for removal of Chest tube sizes French 1236.

Chest tubes
Petrolatum
most pleural air or fluid collections and  Pigtail chest tubes. Lidocaine or bupivacaine for local injection
has a good safety record in children. The Chest tube drainage device.

Antiseptic prep solution
Wide cloth tap
main differences in the procedure between Wall suction.

490
23.8 TUBE THORACOSTOMY
23

COMMON PROCEDURES
1
2
3
4
5

6
Second intercostal 7 Nipple
space midclavicular line 8
9
Fourth intercostal
space anterior axillary line 10
Needle at 60angle

Fig. 23.8.1 Entry site for chest tube.

into the pleural space. Assure the tube

Procedure is inserted beyond the last hole.
Inserting the tube  Attach the tube to the drainage system.
Incise the skin above the sixth rib
13 cm horizontally.
Insert a curved Kelly into the incision, Securing the tube
with tips away from the chest, and Use 20 silk to secure the tube.
dissect the track superiorly to the fourth Employ a purse string suture, and wrap Fig. 23.8.3 Securing the chest tube.

interspace (Fig. 23.8.2). the suture material around the tube

 Rotate the Kelly and puncture through (Fig. 23.8.3). This will allow removal of
Complications
the chest wall. the tube and closing of the incision
Enter over the rib to avoid the without additional suturing. Haemopericardium.
neurovascular bundle.  Place a gauze, slit in half and coated Tension pneumothorax.
Widen the hole with the Kelly then insert with petrolatum jelly over the tube and Haemothorax.
a gloved finger into the hole to assure entry site. Myocardial injury.
the track is clear to the pleura. Secure all tube connections with tape. Diaphragm perforation.
Insert the appropriately-sized Obtain an X-ray to confirm tube Solid organ injury.
thoracostomy tube into the hole and placement and decompression of air or Viscus injury.
direct the tube posteriorly. fluid. Cardiopulmonary arrest.
Facilitate tube entry by grasping the
tube with the Kelly to guide tube entry

Fourth rib
Table 23.8.2 Sizing chest tubes
NV bundles
Age Chest tube
size (Fr) Fifth rib
Newborn 812

Infant 1420 Sixth rib

Child 2028

Adolescent 2836
Chest tube
Formula: chest tube size (in Fr) 4
endotracheal tube
size (mm).
Use larger tube size for haemothorax. Fig. 23.8.2 Inserting the chest tube.

491
23.9 REMOVING AND REPLACING A TRACHEOSTOMY TUBE

Dieckmann RA, Fiser D, Selbst S. Illustrated textbook

Avoid trocars, to minimise lung lacerations.
Tips If a child with a penetrating chest injury
of paediatric emergency and critical care procedures.
St Louis, MO: Mosby; 1997, 57983.
is in shock or respiratory failure, do not Dull KE, Fleisher GR. Pigtail catheters versus large-bore chest
Sizing a chest tube involves estimation of tubes for pneumothoraces in children treated in the ED.
the size of the child. If an exact size is not wait for a chest X-ray before performing Paediatr Emerg Care 2002;18(4):2657.
tube thoracostomy. Genc A, Ozcan C, Erdener A, Mutaf O. Management of
known, use the following formula: pneumothorax in children. J Cardiovasc Surg (Torino)
chest tube size 2 x nasogastric tube Use of CT or video imaging may assist 1998;39(6):84951.
chest-tube placement. Sarihan H, Cay A, Aynaci M, et al. Empyema in children.
size or 4 x endotracheal tube size. J Cardiovasc Surg (Torino) 1998;39(1):1136.
Use a smaller pigtail catheter if the Wilcox DT, Glick PL, Karamanoukian HL, et al. Spontaneous
pneumothorax: A single-institution, 12-year experience in
pneumothorax needs to be evacuated but patients under 16 years of age. J Paediatr Surg
is not causing significant tension. 1995;10:14524.

Use a larger tube for haemothorax. Further reading

Monitor for air leak for evidence of system Cullen ML. Pulmonary and respiratory complications of
air leak or tracheobronchial injury. paediatric trauma. Respir Care Clin N Am 2001;7(1):5977.

23.9 Removing and replacing

a tracheostomy tube
Ronald A. Dieckmann

Background Indications for emergent Water-soluble lubricant.

Scissors.
Children with tracheostomy tubes are replacement
Sterile saline.
increasingly common in out-of-hospital and Respiratory distress or failure in the pres- Stethoscope.
emergency department settings. Laryngeal ence of:
trauma, cervical cord injury, subglottic
stenosis, and conditions requiring prolonged decannulation; Preparation
ventilation often need short- or long-term tube obstruction.
Ask the caregiver if there are any special
tracheostomy-tube placement. Most of
problems with the childs trachea or
these children live at home and have trained
special requirements involving the
caregivers, often parents. Because the tra- Contraindications
childs tracheostomy.
cheostomy tube may be the primary air-
Inadequately sized tract or stoma for inser- If the child has an obstruction of the
way for the child, if the tube comes out
tion of a new tracheostomy tube. In this case, airway as a reason for tracheostomy
(decannulation) or becomes obstructed
insert an endotracheal tube or replacement placement, rescue breathing with a BVM
(mucus plugging), immediate action is nec-
tracheostomy tube that is smaller in diame- may be difficult or impossible.
essary to secure the airway and preserve
ter than the original tracheostomy tube.  Ask the caregiver if a replacement
gas exchange.
tracheostomy tube is available.
Treatment of most tracheostomy problems
Speak directly to the child about what to
requires only simple techniques to establish a
patent airway, such as suctioning of the exist-
Equipment expect and attempt to enlist
co-operation.
ing tube, or removal of the old tracheostomy Suction device.
tube and replacement with a new tube. The Sterile suction catheters.
childs major ongoing risk is airway obstruc-  Oxygen. Procedure
tion from clogging of the old tube with secre- BVM, standard paediatric and adult
Removing an old tracheostomy tube:
tions or foreign bodies. Occasionally, it is mask sizes.
impossible to ventilate a child through an Tracheostomy cannulas, appropriately Position the child with the head and
existing tracheostomy tube because of frank sized for patient (Fig. 23.9.1). neck hyperextended to expose the
decannulation or complete tube obstruction. Endotracheal tubes, standard paediatric tracheostomy site.
Under these conditions, ventilating through and adult sizes. Apply oxygen over the mouth and nose,
the nose and mouth with a bagvalvemask Laryngoscope handle with blades. and occlude the stoma or tracheostomy
(BVM) device, or inserting a new tracheos-  Tape or tracheostomy ties. tube. Most children with tracheostomies
tomy tube or another temporary airway will  Gauze pads. have an intact airway from the mouth
save the childs life.  Syringes, 5 mL or 10 mL. and nose to the trachea.

492
23.9 REMOVING AND REPLACING A TRACHEOSTOMY TUBE
23

COMMON PROCEDURES
Without applying suction, insert the
suction catheter into the stoma.

Slide the tracheostomy tube along the
suction catheter and into the stoma,
until it is in the proper position.
Remove the suction catheter.
Assess ventilation through the
tracheostomy tube.

Option 4. Endotracheal intubation

If still unsuccessful, consider either
orotracheal intubation or ventilation
through the stoma, using a stoma mask
or newborn mask.
Alternatively, do BVM over the nose and
Fig. 23.9.1 Tracheostomy cannulas. mouth while covering the stoma with a
sterile gauze.
Fig. 23.9.2 Inserting a new tracheostomy tube.

If the existing tube has a cuff, deflate it:
Connect a 510-mL syringe to the
cannula, insert to allow mechanical Securing the tracheostomy tube
valve on the pilot balloon.
ventilation with a BVM device. After proper placement, cut the ends of the
Draw air out until the balloon
collapses.
Check for proper placement by watching tracheostomy ties or tape diagonally (allows
for bilateral chest rise, listening for for easy insertion), pass through eyelets
Cutting the balloon will not deflate
equal breath sounds, and observing the (openings) on the flanges, and tie around
the cuff.
patient. Signs of improper placement the patients neck, so that only a little finger
Cut or untie the cloth ties that hold the
include lack of chest rise, resistance to can pass between the ties and the neck.
tracheostomy tube in place.
assisted ventilation, air in the surrounding
Withdraw the tracheostomy tube using a
tissues, and patient agitation.
slow, steady, outward and downward
motion.
If the tube cannot be inserted, withdraw Complications
the tube, administer oxygen, and
Assess airway for patency and adequate Creation of a false lumen.
ventilate as needed.
ventilation.
Provide oxygen and ventilation through
Use a smaller size tracheostomy tube for
Subcutaneous air.
the second attempt. Pneumomediastinum.
the stoma as needed. Pneumothorax.

Option 2. Using an endotracheal

Bleeding at insertion site.
Replacing the tracheostomy tube tube
Bleeding through tube.
Option 1. Using a new tracheostomy If a replacement tracheostomy tube is not avai-
Right main-stem intubation with
tube endotracheal tube.
lable, use an endotracheal tube of the same
Insert a tracheostomy tube of the same size
outer diameter as the tracheostomy tube.
and model whenever possible. If the tube uses
Check the length of the original tracheos-
an insertion obturator, place this in the tube. If
tomy tube, note the markings on the endo-
Tips
the tube has an inner and outer cannula, use
tracheal tube, and advance it to the same Talk to the caregiver about the size and
the outer cannula and obturator for insertion.
depth as the original tube. type of tracheostomy tube and about
Moisten or lubricate the tip of the
The inserted portion of the endotracheal known problems with the stoma, trachea
tube (and obturator) with water, sterile or tube before proceeding.
tube will be approximately half the
saline, or a water-soluble lubricant.
distance needed for oral insertion. Most children who have lost a
Hold the device by the flange (wings) or
Do not advance the tube too far, or it may
tracheostomy tube can be ventilated by
hold the actual tube like a pencil. BVM over the nose and mouth, with the
go into the right main-stem bronchus.

Gently insert the tube with an arching stoma occluded.
motion (follow the curvature of the tube)
Option 3. Using a suction catheter
If unable to reinsert a tracheostomy tube,
posteriorly then downward. Slight use a similarly sized endotracheal tube.
as a guidewire
traction on the skin above or below the
If unsuccessful with the initial replacement
Do not force a large tracheostomy tube
stoma may help (Fig. 23.9.2). through a new stoma site.
attempt, use a suction catheter as a guide:
Once the tube is in place, remove the Do not advance an endotracheal tube too
obturator, attach the bag, and attempt Insert a small, sterile suction catheter far through the stoma, or it may intubate
to ventilate. If the tube has an inner through the tracheostomy tube. the right main-stem bronchus.

493
23.10 CENTRAL AND PERIPHERAL INTRAVENOUS LINES

Bosch JD, Cuyler JP. Home care of the paediatric Dieckmann RA, Brownstein D, Gausche M. Textbook for
Further reading tracheostomy: Our experience. J Otolaryngol 1987;16
(2):1202.
paediatric education for prehospital professionals. Sudbury,
MA: Jones & Bartlett; 2001. p. 3002.
Bahng SC, VanHala S, Nelson VS, et al. Parental report of Dieckmann RA, Fiser D, Selbst S. Illustrated textbook of
paediatric tracheostomy care. Archives Physiology of paediatric emergency and critical care procedures. St Louis,
Medical Rehabilitation 1998;79(11):13679. MO: Mosby; 1997. p. 1212.

23.10 Central and peripheral

intravenous lines
Judith Klein

Background catheters by consulting a length-based

Indications resuscitation tape.
Most children who present to an emergency
department do not need vascular access for Peripheral IV access is indicated if oral, If time permits, consider anaesthetising
transmucosal, intramuscular or the skin with an anaesthetic drug or by
drug or fluid therapy. Medications can usu-
ally be administered orally, transmucosally, inhalation routes are not adequate to iontophoresis. To anaesthetise skin,
intramuscularly or by inhalation. Even fluid meet the patients needs for fluids and/or apply a topical anaesthetic, such as the
administration in dehydrated children does medications. eutectic mixture of local anaesthetics
not ordinarily require vascular access. Oral Use a central IV for: (EMLATM) cream, under an occlusive
rehydration, performed slowly and methodi- Central venous pressure monitoring. dressing at the selected entry site at
cally, is often successful in children with Administration of hypertonic solutions. least 45 minutes prior to any IV attempt.
vomiting and/or diarrhoea. However, when Essential access when peripheral and IO The alternative technique of
attempts have failed. iontophoresis requires specialised
oral rehydration is unsuccessful or when
a child presents critically ill or injured, intra- equipment for delivery of a low
venous (IV) or intraosseous (IO) access amperage, painless electrical charge to
Contraindications
(Chapter 23.11) becomes essential. the skin. The technique is safe, non-
Finding veins to cannulate in infants and Peripheral IV catheters are invasive and usually quite effective.
small children can be quite a challenge. The contraindicated if other, non-invasive, Iontophoresis takes 1020 minutes to
higher ratio of subcutaneous fat and smaller routes can meet the childs needs. numb skin for peripheral IV insertion.
vessel size in young patients pose significant Central venous catheters are Use over-the-needle catheters whenever
barriers to rapid venous cannulation. A contraindicated if peripheral venous possible. Butterfly needles are far less
peripheral venous site, rather than a central access sites are available and there are no stable but are acceptable for short-term
site, offers the highest benefit:risk ratio of special indications for central venous infusions. Use armboards/legboards
any vascular access option. Fig. 23.10.1 illus- catheter placement. and a plastic container to cover the IV to
trates common sites for peripheral IV line If possible, do not pass peripheral IV avoid unintentional (or deliberate!)
insertion. catheters: dislodgement of the catheter. Prime the
When a peripheral site is unavailable, Through cellulitic skin. tubing, and set up the IV fluid chamber
an IO site provides a second option In an extremity that has a wound, e.g. with microdrip and an infusion pump in
(Chapter 23.11). When peripheral venous burn or laceration. advance. Carefully monitor infusions in
and IO access cannot be obtained or if cen- Distal to unstable fractures or injured infants and small children to prevent
tral venous pressure monitoring is required, veins. over-administration of fluids. Select the
consider cannulating central veins, such as Avoid placing central lines in non- smallest-sized catheter that will meet
the femoral vein, subclavian vein, or internal compressible sites in patients with the patients needs for drug and fluid
jugular vein. Avoid saphenous vein cut- bleeding diatheses. administration.
downs in children, because they are techni-
cally difficult to perform and the technique Preparation
Peripheral venous line
is time consuming in infants and young chil-
placement Prepare all equipment and place near
dren, even for experienced operators. Make the child. Avoid using the bed as an
every effort to provide the least invasive Equipment equipment table as the equipment may
form of access required by the degree of Table 23.10.1 lists peripheral IV equip- find its way quickly to the floor with an
the childs illness. ment. Determine appropriate sizing of IV active child.

494
23.10 CENTRAL AND PERIPHERAL INTRAVENOUS LINES
23

COMMON PROCEDURES
Locate a straight segment of the vein
Frontal
and provide in-line traction away from
Posterior Superficial
auricular temporal
the direction of catheter insertion.
External jugular Insert the catheter at about a 1020
Axillary degree angle to the skin. Once there is a
Basilic flash of blood in the hub of the catheter,
Cephalic advance the catheter with the needle in
place another 12 mm. Then advance
the catheter over the needle into the
Superficial Umbilical
vein.
dorsal
Draw any blood samples needed,
remove the tourniquet, and connect the
Great IV line.
saphenous Fig. 23.10.2 Restraining an infant. Secure the catheter and IV line to the
patient with tape and a plastic container
Dorsal over the IV (Fig. 23.10.4).
If insertion is unsuccessful at traditional
peripheral IV, consider two alternative sites
Fig. 23.10.1 Common peripheral IV sites.
prior to moving to a central line: the external
jugular vein and the deep brachial vein. To
cannulate the external jugular vein, place
Table 23.10.1 Equipment for peripheral the patient in Trendelenburg position to
IV insertion
dilate the vein. Restrain the patient well
Gloves and turn his/her head slightly away from
Arm or leg board
Tourniquet the cannulation site (Fig. 23.10.5). Do not
Alcohol pads use a tourniquet for placement of an IV at
Gauze pads
2224-gauge venous catheters this site. Clean the area with isopropyl alco-
Saline flush hol or povidone-iodine solution and use an
IV tubing, solution and pump
Protective covering for IV (small plastic cup, Fig. 23.10.3 Restraining a child. 18G needle to make a nick in the skin at
roller gauze) the catheter site. This will facilitate passage
Tape
of the catheter through the skin. Use the
Procedure peripheral insertion technique but apply
There are a number of possible sites for
Flush the catheter with saline solution if firm counter-traction at the entry site to help
placement of peripheral IV lines in infants stabilise the vessel. Secure the needle care-
diagnostic blood samples are not
and children. The easiest sites are the dor- fully to avoid dislodgement.
necessary during IV insertion. This will
sum of the hands and feet, the antecubital
reduce the risk of air embolisation.
fossa and, in infants less than 1 year, the
 Remove any topical anaesthetic at the
scalp. In non-emergent situations, start dis-
IV site or the apparatus for
tally and move proximally when initial
iontophoresis.
attempts fail. Consider using a warm com-

CC
12
11
10
9
8
7
6
5

2
1

Have an assistant restrain an infant or

press to dilate constricted veins and make
toddler with a sheet (Figs 23.10.2 and
them more visible. In emergent situations,
23.10.3).
cannulate the largest vein available and
Attempt to talk an older child through
consider less commonly used peripheral
the procedure with a parent or caregiver
sites, such as the external jugular vein and
present.
deep brachial vein.
Have the assistant gently restrain the
extremity/body part selected for IV Make sure that the child is well
access. Use padded boards to stabilise immobilised.
arm or leg access sites in infants and Don gloves and clean the insertion site
young children. with isopropyl alcohol or povidone-
Locate landmarks and determine iodine solution.
insertion sites first with an ungloved  Apply a tourniquet just proximal to the
hand that is more sensitive in detecting insertion site. Use a rubber band around
surface veins and palpating landmarks the scalp caudad of the insertion site to
than the gloved hand. cannulate scalp veins. Fig. 23.10.4 Securing the catheter to the skin.

495
23.10 CENTRAL AND PERIPHERAL INTRAVENOUS LINES

Infiltration.
Bleeding.
Pressure necrosis due to IV stabilisation
methods.
To avoid these complications, clean insertion
sites well prior to passing the needle
through the skin. Make sure blood can be
easily withdrawn from and fluids easily
External
jugular vein infused through the IV. Avoid infusing irri-
tant or hyperosmolar solutions through
Sternocleidomastoid peripheral lines. Tape IV lines securely but
Internal muscle
jugular vein do not create a tourniquet with tape and
remember to pad all joints. Replace periph-
eral lines every 4872 hours.

Systemic complications
Thrombosis.
Air embolism.
Catheter tip embolisation.
Subclavian vein
Flush catheters regularly but not forcefully.
Fig. 23.10.5 Positioning for external jugular vein puncture.
Do not re-insert the needle stylet into the
The second alternative site is the deep artery and slowly advance the catheter. If catheter once it has been removed. Doing
brachial vein. In contrast to the other sites, arterial blood appears or the patient com- this can shear the catheter and cause cath-
identify the likely location of the deep bra- plains of hand paraesthesiae (median nerve eter fragment embolisation.
chial vein by anatomic landmarks, not by irritation), remove the catheter and hold
visualisation or by palpation. Ultrasound pressure at the site. There is anatomic varia-
can assist in localisation of the vein. Consider bility in the position of the vein, so try mar- Tips
this option in older children. Palpate the bra- ginally different slightly medial insertion
Whenever possible, use a topical
chial artery medial to the biceps just proxi- sites if initial attempts are unsuccessful.
anaesthetic at several possible IV sites
mal to the antecubital fossa (Fig. 23.10.6).
at least 45 minutes prior to line
Place a tourniquet and prepare the skin with
placement, or consider iontophoresis.
isopropyl alcohol or povidone-iodine solu-
tion. Insert the catheter at a 30 degree angle
Complications Avoid joint surfaces and the patients
dominant hand. If a large-bore catheter
approximately the patients finger breath Local complications
is required, use more proximal sites,
medial to the brachial artery. Angle the cath- Phlebitis. such as those at the antecubital fossa
eter parallel to the course of the brachial Site infection. or external jugular vein.
Triceps muscle Do not let go of recently-placed IV
Biceps muscle
Incision
catheters until they are well secured.
Deep brachial vein Monitor newly-placed IVs closely,
Cephalic vein particularly in infants and small children
Brachial artery
with persistent crying. A swollen
Median cutaneous extremity may be the only marker of an
nerve
infiltrated catheter.

Lateral epicondyle
Medial epicondyle
Lateral antebrachial
cutaneous nerve
Central venous line
Distal flexor
crease placement
Median antebrachial
vein Equipment
The equipment for placement of a central
venous line is usually in a prepackaged kit.
Cephalic vein Median cubital
vein The simplest and most straightforward tech-
nique for central-line placement is the guide-
Fig. 23.10.6 Cannulating the deep brachial vein. wire or Seldinger technique. Pre-packaged

496
23.10 CENTRAL AND PERIPHERAL INTRAVENOUS LINES
23

COMMON PROCEDURES
the neck extended and the head turned
Table 23.10.2 Sizing central IV catheters
slightly away from the insertion site. A roll
Weight (kg) Age Catheter size (French) placed under the ipsilateral shoulder will
<5 Newborn to 6 months 3, 4 facilitate neck extension, particularly in
infants. Fig. 23.10.8 illustrates the relevant
515 6 months to 5 years 5, 7
anatomy and landmarks.
15 >5 years 511
Identify the triangle formed by the
sternal and clavicular heads of the
kits will contain, among other things, a Procedure sternocleidomastoid muscle and the
needle, syringe, J-wire, scalpel, dilator and Sites for central venous line placement in clavicle.
catheter. Determine appropriate catheter children include: the femoral vein, the inter- Palpate the carotid artery just medial to
sizes by measuring the child next to a nal jugular vein, and the subclavian vein. the triangle.
length-based resuscitation tape or by consult- The external jugular vein is also a possible  The insertion site is either at the apex of
ing Table 23.10.2. Estimate insertion distance central venous insertion site. Passage of the triangle or halfway (from mastoid to
by measuring from the insertion site exter- the catheter centrally via the external jugu- sternum) along the medial border of the
nally to the desired tip position. lar vein is difficult because of the acute sternal head. In either case, aim towards
angle of entry of the external jugular into the ipsilateral nipple and away from the
the subclavian vein. It is, therefore, the least carotid artery. The right side is
desirable site. preferable because of the straighter
Preparation Fig. 23.10.7 illustrates the essential anat- course to the superior vena cava, the
If time allows, sedate the child prior to omy for placement of a femoral venous lower pleural dome, and the absence of
insertion of a central venous catheter catheter. the thoracic duct.
and/or use a topical anaesthetic such as Place the catheter tip at the junction of
EMLA cream. If the situation is Place the patient supine in a mild
the superior vena cava and the right
emergent, use a sheet wrapped around reverse Trendelenburg position.
atrium.
the child and an assistant to restrain the Maintain the hip in slight external
child. Excessive patient movement will rotation and abduction. Positioning and anatomy relevant for place-
increase the rate of complication and Palpate the femoral artery at the ment of a subclavian central venous line is
decrease the likelihood of procedure inguinal ligament. The insertion site is similar to that for placement of an internal
success. about a patients finger breadth medial jugular venous line (Fig. 23.10.9).
Prepare all equipment on the tray so to the artery and 23 cm distal to the
Sedate conscious patients before
that items can be easily located. Do not inguinal ligament. The right side is
attempting a subclavian line, as
use the childs bed as an instrument easier for a right-handed operator.
patient movement can result in a
table as the instruments will soon find  Measure from insertion site to the
high rate of complications. Place the
their way to the floor. umbilicus to estimate catheter insertion
patient in Trendelenburg position with a
 Flush the catheter with saline solution in distance.
towel roll underneath the ipsilateral
advance unless diagnostic blood To cannulate the internal jugular vein, place shoulder to help keep the neck in
specimens are required. the patient in Trendelenburg position with extension.
Mentally divide the clavicle into
thirds.
 The insertion site is just inferior to the
junction of the middle and medial thirds
Femoral nerve, artery
and vein of the clavicle aiming towards the
sternal notch. Aim the J curve of the
Inguinal ligament
guidewire caudally during insertion.
Needle enters femoral vein
12 cm below inguinal ligament Also, turn the patients head toward the
Needle enters skin 23 cm site of insertion while advancing the
below inguinal ligament wire to avoid passage of the wire up the
internal jugular vein.
Place the catheter tip at the junction of
the superior vena cava and the right
atrium.
The general procedure for placement of a
central venous catheter is the same regard-
Fig. 23.10.7 Essential anatomy for placement of a femoral venous catheter. less of location.

497
23.10 CENTRAL AND PERIPHERAL INTRAVENOUS LINES

insertion of the needle underneath

the clavicle aiming towards the
sternal notch.
Maintain constant negative pressure on the
syringe while inserting or withdrawing the
needle as vessel entry may occur during
withdrawal. Once blood flows freely into
the syringe, stabilise the needle with a hand
External resting on the patient. Remove the syringe
jugular vein
carefully, trying not to alter the position of
Sternocleidomastoid the needle. Insert the J-wire through the
Internal muscle
jugular vein needle, J end first (Fig. 23.10.10). Point the
J of the guidewire in the direction the cath-
eter is intended to go. The wire should pass
easily. Do not force it if it does not. Removing
the wire and repositioning the needle can
often resolve gentle resistance. If strong
resistance is met, remove the needle and
wire together to avoid shearing off the wire
Subclavian vein into the vein.
Once the wire is passed to the pre-marked
Fig. 23.10.8 Cannulating the internal jugular vein.
distance, remove the needle. Never let go of
the wire. Make a small stab incision with a
Clean the skin with a povidone-iodine or Insert the needle at the selected scalpel over the wire. Insert a dilator over
chlorhexidine solution. insertion site with the bevel pointed the wire, once again, remembering to hold
Anaesthetise the site with 1% lidocaine. anteriorly (femoral), medially (internal onto the wire at all times. Remove the dila-
 Attach a syringe to the insertion needle jugular), or caudally (subclavian). tor and insert the catheter over the wire to
so that the bevel lines up with the Angle the needle at approximately the pre-measured distance (Fig. 23.10.11).
numbers on the syringe. This allows 30 degrees to the skin, except when Remove the wire. Draw off any diagnostic
for identification of bevel direction placing a subclavian line. With blood specimens. If the catheter has not
even when the needle is inside the subclavian line placement, use the been flushed in advance, withdraw blood
patient. shallowest angle possible that allows

J wire

Needle

Vein

Fig. 23.10.10 Inserting the J-wire through the

needle.

Internal jugular vein J wire

Sternocleidomastoid
Lateral one third of muscle
clavicle
Distal lumen
Double
lumen
catheter

Subclavian vein
Fig. 23.10.11 Inserting the catheter over the
Fig. 23.10.9 Cannulating the subclavian vein. wire to the pre-measured distance.

498
23.10 CENTRAL AND PERIPHERAL INTRAVENOUS LINES
23

COMMON PROCEDURES
from all lumens of the catheter prior to infus- A shallow angle of insertion with If central venous pressure monitoring is
ing any fluids or medications to avoid air subclavian lines can help prevent this required, use an access site above the
embolisation. Suture the catheter in place complication. Obtain an X-ray after all diaphragm. While the internal jugular
and apply a sterile dressing. Verify location line placements, not only to verify vein is larger, the subclavian site is more
of the catheter tip with an X-ray. catheter position but also to detect a comfortable for the patient in the long
pneumothorax. term.
Suspect catheter thrombosis if If the patient is pulseless, the subclavian
withdrawal of blood or infusion of vein is the preferred site for central
Complications fluids becomes difficult. Eventually venous catheterisation as it does not
pain and swelling of the extremity require a pulse for localisation and can be
Table 23.10.3 lists the most common compli-
will tip the clinician off to the used for central venous pressure
cations of central venous line placement.
presence of the clot. Manage monitoring.
Bleeding can occur during traumatic line thromboses expeditiously with
placement or after arterial puncture. catheter removal.
Patients with bleeding diatheses are at Infection is equally likely to occur at any
great risk for bleeding complications. of the central venous sites. Remove
Compress bleeding sites from femoral or infected catheters as soon as possible and
internal jugular sites. These sites are safer treat with antibiotics. If possible, avoid Further reading
than the subclavian route where the insertion of a new central catheter for Bagwell CE, Salzberg AM, Sonnino RE, Haynes JH. Potentially
lethal complications of central venous catheter placement.
internal bleeding site is non- 2448 hours. J Paediatr Surg 2000;35(5):70913.
compressible. Avoid subclavian Chiang VW, Baskin MN. Uses and complications of central
venous catheters inserted in a paediatric emergency
catheterisation in patients who are department. Paediatr Emerg Care 2000;16(4):2302.
moving around or who have clotting Cunningham FJ, Engle WA. Paediatric vascular access and
blood sampling techniques. In: Roberts JR, Hedges JR,
problems. editors. Clinical procedures in emergency medicine. 3rd ed.
Pneumothorax can occur with internal Tips Philadelphia, PA: WB Saunders; 1998.
Fernandez EG, Sweeney MF, Green TP. Central venous
jugular or subclavian line insertion. catheters. In: Dieckmann RA, Fiser DH, Selbst SM, editors.
In the awake patient, restraint and proper Paediatric emergency and critical care procedures. St Louis,
sedation and analgesia will improve MO: Mosby; 1997.
King D, Conway EE Jr. Vascular access. Paediatr Ann 1996;
procedural success and avoid 25(12):6938.
Table 23.10.3 Complications of central
venous line placement complications. Macnab AJ, Macnab M. Teaching paediatric procedures: The
Vancouver model for instructing Seldingers technique of
Bleeding Use ultrasound, if possible, to identify central venous access via the femoral vein. Paediatrics
Arterial puncture/cannulation/laceration vessels prior to line placement. 1999;103(1):E8.
Infection Stoyroff M, Teague WG. Intravenous access in infants and
Catheter thrombosis If the patient is moving and there is no children. Paediatr Clin N Am 1998;45(6):137393.
Pneumothorax time for sedation, the femoral site is the Webster PA, Salassi-Scotter MR. Peripheral vascular access. In:
Haemothorax Dieckmann RA, Fiser DH, Selbst SM, editors. Paediatric
Thoracic duct laceration
safest and most easily compressible vein emergency and critical care procedures. St Louis, MO:
if bleeding occurs. Mosby; 1997.

499
 23.11 Intraosseous infusions
Lindsay Bridgford Ronald A. Dieckmann

transmucosal, intramuscular or
Background inhalation routes are not adequate to Preparation
Peripheral intravenous cannulation in a meet the patients needs for fluids and/or Identification of the entry site
critically ill or injured child can be difficult, time medications. The best site in children is the anteromedial
consuming, and sometimes impossible. Small aspect of the proximal tibia lateral to
veins collapse or disappear during shock, and the tibial tuberosity. Alternative sites are the
increased body fat may camouflage super- Contraindications distal end of the femur 23 cm above the
ficial skin veins. Central venous access and sur- patella in the midline, and the medial
Do not use an IO infusion if the child is
gical cut-down are also technically difficult stable. malleolus at the ankle (Fig. 23.11.2). The
procedures that may be risky or impossible in sternum is not recommended in children.
Do not place an IO needle below a
critical situations. Although the endotracheal fracture site. Use the other side. In adults, the medial malleolus may be the
route is an alternative to vascular access in best site, although studies are lacking.
Avoid placement of an IO below any
cardiopulmonary arrest, endotracheal intu- open injury on an extremity. Use the
bation may be delayed, drug absorption may other side.
not be reliable, and large fluid administration
is contraindicated by this route. Positioning the child
Relative contraindications Place the child supine with the knee
The intraosseous (IO) or intramedullary
Avoid IO needle insertion in children slightly flexed and a small towel roll or
route for the delivery of resuscitation fluids
with osteoporosis and osteogenesis other bulky material under the popliteal
and medications has been used for over
imperfecta, due to the high fracture fossa. Make sure the conscious child is well
50 years in children and adults. Many stud-
potential. immobilised.
ies have confirmed that the highly vascu-
Recent prior use of the same bone for IO
larised IO space is an excellent route for
infusion, due to the potential for
medications and fluids. The only technical
extravasation from previous IO sites.
problem is successfully piercing the bony
cortex in older children. The bones of
neonates and infants are usually soft and
Equipment
the IO space is relatively large, so needle
insertion is easy in children of youngest The EZ IO or intraosseous gun is an
age. Good equipment, preparation, and excellent alternative to the manual
effective technique are especially important needle.
for success in IO needle insertion. While IO A variety of needles will work. Butterfly
access is easy, quick, and safe, it is painful needles and spinal needles may be
in a conscious child and therefore is only effective, especially in neonates and Fig. 23.11.1 IO needles.
practical in a critically ill or injured child. infants, but these needles bend too
The IO space functions as a non- easily in the more calcified bones of
collapsible vein. There are several possible children and adolescents. 60 degrees
sites for insertion; but the easiest location  Commercially available IO needles have
Medial flat surface
of the anteror tibia
in children is the proximal tibia. The emis- more durable parts intended for
sary veins of the IO space absorb all paren- penetration of bone. There are several
teral medications, crystalloid fluids, or blood styles (Fig. 23.11.1). A central stylet is
products which move quickly into the universal. There are short, 2.5-cm,
Growth Tibial
central circulation. Complications are minor needles for neonates and infants and plate tuberosity
and infrequent. Out-of-hospital emergency- longer, 3.0 and 3.5-cm, needles for older
7580 degrees
care professionals have also employed the children. Some needles have stylets with
IO technique with a high rate of success. multifaceted cutting edges intended for
a rotary insertion, others have bevels,
and others can be screwed in place. The
Indications
shaft may have side ports. Femur
Cardiopulmonary arrest. 10-mL syringe.
Any critical emergency when a peripheral Normal saline.
cannulation site is unavailable and oral, Stopcock (optional). Fig. 23.11.2 IO needle entry sites.

500
23.11 INTRAOSSEOUS INFUSIONS
23

COMMON PROCEDURES
Selecting the correct side Growth-plate injury.
A right-handed operator will more easily Bone infection.
insert the needle in the childs right leg, Skin infection.
and vice-versa for the left-handed operator. Skin necrosis.
Bony fracture.

Procedure
Introduce the IO needle in the skin, Tips
directed slightly away from the growth
plate. Grasping the limb with the Be extremely careful using an EZ IO in a
non-dominant hand beside the insertion young infant, because it is quite easy to
Fig. 23.11.3 EZ-IO.
site (e.g. behind the knee for proximal penetrate through the bone into the soft
tibia insertion) helps to steady the tissues and cause a compartment
bone during placement. syndrome.
Pierce the bony cortex with a firm, twisting If the child is conscious, provide generous that operates like a small drill. The driver
motion from a position directly above the local anaesthesia at the entry site. itself is a sealed unit that is good for about
entry site. A pop may be felt as the needle In addition to serving as a route for drug 700 insertions. The EZ-IO uses a bevelled
passes through the bony cortex and into and fluid administration, blood samples drill tip that rotates into the IO space at a
the marrow cavity. Do not push too hard drawn from the site can be used for preset depth (Fig. 23.11.3).
on the needle. Too much force may push culture, haematological, and biochemical The procedures for insertion of the EZ-IO
the needle all the way through the bone analysis, but there are limitations. needle are similar to those described above
and into the soft tissues. Emergency type and crossmatch is for the manual device. The difference is in
 Remove the stylet and aspirate marrow reliable with an adequate sample. the preparation of the EZ-IO driver and pae-
contents with a 10-mL syringe. Keep any A complete blood cell count may not be diatric needle set, with a 15-gauge, 15-mm
bone marrow aspirate for glucose check reliable, as it reflects the marrow cell long needle generally used for children
or for other tests in the emergency count rather than the cell count in the weighing between 3 kg and 39 kg, a
department. Sometimes, marrow cannot peripheral circulation, and also because 15-gauge, 25-mm long needle for children
be aspirated. blood aspirated from the marrow usually weighing greater than 40 kg and a new
Confirm correct placement by infusing clots rapidly, even if it is placed in a tube 45-mm length needle is available for larger
10 mL of normal saline without that contains heparin. Blood chemistry children with significant tissue or oedema
resistance. Once the IO needle has been determinations are possible, but they may overlying bone.
placed, attach a stopcock (if available) not correlate with blood samples taken
to the end of the needle before from central veins.
attaching the intravenous (IV) line. All commonly used intravenous fluids and
Further reading
Attach IV line to the hub and infuse drugs can be administered via the IO
Brunette DD, Fischer R. Intravascular access in paediatric
fluids or drugs directly into IO space. route. cardiac arrest. Ann Emerg Med 1988;6:577.
Pushing the fluids too hard may force The rate of infection from IO needle Fiser DH. Intraosseous infusion. N Engl J Med
1990;322:1579.
the fluids out of the IO space. The rate of placement is low and comparable to IV Johnson L, Kissoon N, Fiallos M, et al. Use of intraosseous
fluid infusion may be limited by the cannulation. blood to assess blood chemistries and haemoglobin during
cardiopulmonary resuscitation with drug infusions. Crit
small marrow space. A slow infusion rate does not mean the IO Care Med 1999;27:114752.
Secure the needle to the overlying skin needle is misplaced, and pressure infusion Phillips B, Zideman D, Garcia-Castrillo L, et al. European
Resuscitation Council guidelines 2000 for
with tape. may be necessary to maintain flow. advanced paediatric life support. Resuscitation
Monitor the calf to ensure that there is 2001;48:231324.
Vidal R, Kissoon N, Gaylor M. Compartment
no swelling to indicate leakage of fluid. The EZ-IO syndrome following intraosseous infusion. Paediatrics
1993;91:12012.
Mechanical devices such as the EZ-IO have Wenzel V, Lindner KH, Augenstein S, et al. Intraosseous
vasopressin improves coronary perfusion pressure rapidly
simplified the insertion of an IO needle as
Complications during cardiopulmonary resuscitation in pigs. Crit Care Med
they are not dependent on the manual pro- 1999;27:15659.
Tobias JD, Ross AK. Intraosseous infusions: a review for the
Compartment syndrome. cess. The EZ-IO (Vidacare, San Antonio, TX, anesthesiologist with a focus on pediatric use. Anesth
Failed infusion. USA) is a reusable battery-powered device Analg 2010;110(2):391401.

501
 23.12 Rectal drug administration
Ronald A. Dieckmann

tuberculin syringe or into a 35-mL

Background Equipment syringe.
Status epilepticus is a major paediatric Diazepam gel preparation or IV solution Lubricate the syringe or catheter:
medical emergency that requires emergency of diazepam or lorazepam. a. If using the tuberculin syringe as the
treatment. Although the first priority is Lubricant. administration device, remove needle
airway and breathing, additional therapy  Tuberculin syringe, or 1420-gauge and apply lubricant to the tip of
includes termination of the seizure. Cannu- over-the-needle catheter with 35-mL the syringe.
lating a peripheral vein in a child in status syringe. b. If using a 35-mL syringe, remove
epilepticus limits timely delivery of essential Tape (optional). needle, attach over-the-needle
advanced life support (ALS) drugs, especially catheter (plastic portion only), and
in infants and toddlers. However, intra- lubricate catheter.
venous (IV), intramuscular (IM), or intraoss-
eous drug administration is not necessary
Preparation
because rectal benzodiazepine admini- Determine the weight of the child using a
stration is probably as effective and has no decision support software program or pae-
added complications. Rectal drug adminis- diatric resuscitation tape or other accurate
Procedure
tration is a time-honoured drug-delivery estimate of size. Or obtain the patients Position the patient in the decubitus
technique in children and is useful for weight from the caregiver. position, kneechest position, or
many medications, including antipyretics supine position (Fig. 23.12.2) and
and anticonvulsants. The rectum is highly have a second person hold the legs
Option 1. Diazepam gel
vascularised, and lipid-soluble drugs are rap- apart.
preparation
idly absorbed when correctly administered
Remove the rubber-tipped gel container Carefully introduce the rubber tip
(Fig. 23.12.1). Diazepam is a lipid-soluble of the gel container, or the syringe
from the sterile package.
benzodiazepine that is reliably absorbed or over-the-needle catheter
through the rectum and will terminate most approximately 5 cm (2 inches) into
seizures without further treatment. Loraze- Option 2. IV diazepam the rectum.
pam is an effective alternative drug choice. preparation  Inject the gel or solution into the
Rectal drug administration is a technique Draw up the calculated dose of rectum. Remove the syringe.
that allows delivery of an absorbable benzodi- IV medication into a disposable Hold buttocks closed for 10
azepine in the setting of status epilepticus. seconds.
The relative effectiveness and safety of Tape buttocks closed (optional).
rectal diazepam versus IM midazolam, loraze-
pam or other benzodiazepines for treatment
of status epilepticus are not known. Rectal
diazepam or lorazepam administration may
take a few minutes longer to stop the seizure,
compared to IV administration, and drug
levels may be more variable. Occasionally,
as with the IV diazepam preparation, more
than one dose of rectal diazepam is necessary.

Indications
Repetitive seizures.
Status epilepticus.

Contraindications
Newborn age (a month or less).
Middle rectal vein Inferior rectal vein
Recent prior rectal surgery (e.g. for
Hirschsprungs disease, imperforate anus). Fig. 23.12.1 Anatomy of the rectum. Fig. 23.12.2 Administering rectal drugs.

502
23.13 UMBILICAL VESSEL CANNULATION
23

COMMON PROCEDURES
depression occurs after diazepam
Complications Tips administration. The drug may precipitate
The most serious potential complication The rectal dose of diazepam is 0.5 mg seizures by interfering with the effect of
of rectal diazepam is respiratory kg1, or five-times the IV dose, to a the diazepam in the brain.
depression, which is usually from the maximum dose of 10 mg. Onset of action
drug, but may be from the prolonged for rectal diazepam is slower.
seizure, or the underlying cause of the Do not give additional rectal diazepam until
seizure. approximately 5 minutes after the first Further reading
Administration of diazepam too high dose, to avoid stacking doses and Appleton R, Martland T, Phillips B. Drug management for
or too proximal into the rectum may increasing the risk of respiratory depression. acute tonic-clonic convulsions including convulsive status
epilepticus in children. Cochrane Database Syst Rev
decrease its anticonvulsant effect. Rectal lorazepam may be at least as 2002;4:CD001905.
When the drug is delivered too effective as rectal diazepam in status Dieckmann RA, Brownstein D, Gausche M. Textbook for
paediatric education for prehospital professionals. Sudbury,
proximal in the rectum, absorption may epilepticus. MA: Jones & Bartlett; 2001. p. 2912.
occur into the superior haemorrhoidal Do not perform endotracheal intubation Dieckmann RA, Fiser D, Selbst S. Illustrated textbook of
paediatric emergency and critical care procedures. St Louis,
veins, which drain into the portal system on every child with diminished breathing MO: Mosby; 1997. p. 2748.
instead of the middle and inferior after rectal diazepam. Most will not need Dieckmann RA. Rectal diazepam for prehospital paediatric
status epilepticus. Ann Emerg Med 1994;23(2):21624.
haemorrhoidal veins, which drain into the it if bagvalvemask is effectively Fitzgerald BJ, Okos AJ, Miller JW. Treatment of out-of-hospital
systemic venous system. The drug will performed for a few minutes until the status epilepticus with diazepam rectal gel. Seizure
2003;12(1):525.
then undergo hepatic metabolism, child begins breathing again Mitchell WG, Conry JA, Crumrine PK, et al. An open-label
which will significantly reduce brain spontaneously. study of repeated use of diazepam rectal gel (Diastat) for
episodes of acute breakthrough seizures and clusters:
concentrations. Do not use the benzodiazepine reversal Safety, efficacy, and tolerance. North American Diastat
Rectal tearing. agent flumazenil when respiratory Group. Epilepsia 1999;40(11):16107.

23.13 Umbilical vessel cannulation

Judith Klein

of the umbilical arteries if blood pressure

Background monitoring and arterial blood gas sampling Preparation
During fetal life, two umbilical arteries trans- are important for patient management. Prepare all equipment in advance.
port nutrients and oxygen from the placenta Flush the umbilical catheter with
and one umbilical vein helps dispose of saline or heparin flush and attach
fetal waste. During delivery, these vessels Indications the stopcock.
are cut and clamped and the newborn is Emergent vascular access for  Place the infant supine under a
separated from the placenta. However, the resuscitation. radiant warmer with all extremities
umbilical vessels can be recannulated and Central access for delivery of fluids, restrained.
utilised for emergent vascular access in ill medications, or exchange transfusion.
neonates for up to 7 days after birth. This Frequent blood gas sampling.
is an excellent method of central drug and Haemodynamic monitoring.
fluid delivery, because peripheral venous Table 23.13.1 Equipment for cannulation
of the umbilical artery or vein
access in infants in the first week of life
Sterile gown, gloves and mask
is quite difficult, particularly in the ill or Contraindications Povidone-iodine solution
intravascularly depleted neonate. The other Sterile gauze
alternative for emergency vascular access in Do not place an umbilical vessel line if a Sterile drapes
Umbilical tape
newborns is the insertion of an intraosseous peripheral intravenous line is available and 3.0 Silk suture with needle
needle (Chapter 23.11). adequate for the infants needs. Scalpel
Haemostats
While either of the umbilical arteries and Smooth, curved iris forceps
Iris scissors
the umbilical vein is available for vascular
Equipment Needle holder
access, the umbilical vein is technically Umbilical catheter (3.5 or 5.0F)
Heparinised saline/syringes
easier to cannulate. Therefore, use the vein Table 23.13.1 lists the equipment for cannu- Three-way stopcock
in an emergent situation. Cannulate one lation of either the umbilical artery or vein.

503
23.13 UMBILICAL VESSEL CANNULATION

Utilise cardiorespiratory and pulse Use gentle, but steady, pressure while
oximetry monitors for the duration of inserting the catheter and provide
the procedure. gentle traction on the cord if resistance
Don a mask, sterile gown and sterile is met. Insert the catheter so that the tip
Umbilical
gloves. is in the inferior vena cava below the arteries
diaphragm at a depth of approximately
45 cm or above the diaphragm (via the Umbilical vein
portal system) at a depth of 812 cm
Procedure (Fig. 23.13.3).
Umbilical vein catheterisation The deeper line allows for
Have an assistant to hold the umbilical administration of higher concentration
stump up, scrub the umbilicus with fluids as well as central venous
povidone-iodine solution from xiphoid to pressure monitoring. Confirm that
pubis. blood returns freely through the
Apply a sterile drape. catheter prior to infusion of any fluids Fig. 23.13.4 Stabilising the vessels.
 To provide haemostasis, loosely tie or medications.
umbilical tape at the base of the
umbilical cord at the junction with the
skin and cut the cord approximately Umbilical artery catheterisation Insert the smooth-curved iris forceps into
12 cm from the skin. Prepare and drape the umbilical the artery selected, and gently dilate the
Identify the two thick-walled arteries cord as with umbilical vein artery (Fig. 23.13.5).
and the larger, thin-walled umbilical vein catheterisation. Continue dilating until the forceps can
(Fig. 23.13.1). Loosely tie umbilical tape at the base of be inserted about 1 cm. Gently
Holding the umbilical cord with a the umbilical cord at the junction with introduce and advance the preflushed
haemostat, insert the flushed 3.5F the skin and cut the cord approximately catheter (3.5 preterm; 5.0 term) towards
(preterm) or 5F (term) catheter into the 12 cm from the skin. the patients feet with gentle cephalad
umbilical vein (Fig. 23.13.2).  Identify the two thick-walled arteries traction on the cord (Fig. 23.13.6).
and attach two haemostats to either Overcome any resistance with gentle
side of the umbilical cord to stabilise the steady pressure, but do not force the
vessels (Fig. 23.13.4). catheter. Place the catheter either low
Umbilical vein
Do not place the haemostats on the at the level of the L3 vertebra or high
arteries. between the T6 and T9 vertebrae
Umbilical arteries

Fig. 23.13.1 Identifying the umbilical

arteries and vein.

High (812 cm)

Superior mesenteric artery

Umbilical arteries
Renal artery
Low (45 cm)
Inferior mesenteric artery
Umbilical vein
Level of L3
Umbilical vein (desired catheter placement)

Umbilical arteries

Fig. 23.13.2 Inserting the catheter into the

umbilical vein. Fig. 23.13.3 Location of tip of catheter.

504
23.13 UMBILICAL VESSEL CANNULATION
23

COMMON PROCEDURES
extremities. If blanching or cyanosis of
Ductus the lower extremities occurs, remove the
arteriosus catheter immediately.

Umbilical
arteries T6 6 High
position
Tips
T10 Always advance catheters slowly and use
Umbilical vein Celiac artery only gentle pressure if resistance is
Renal arteries encountered.
Inferior mesenteric
Low Aggressive catheter insertion can result
position
artery in creation of a false lumen or perforation
Aortic
bifurcation of a vessel.
Iliac Always confirm catheter position
artery radiographically.
Fig. 23.13.7 Placing the catheter. If an umbilical venous catheter is needed
Fig. 23.13.5 Inserting the iris forceps into the emergently prior to radiographic
artery.
confirmation of position, place it low to
avoid accidental injection of sclerosing
medications directly into the liver.
However, if time allows for radiographic
confirmation of catheter position, place
the umbilical artery catheter high since
that position appears to be associated
Umbilical arteries with fewer complications.
Umbilical vein

Further reading
Barrington KJ. Umbilical artery catheters in the newborn:
Effects of catheter materials. Cochrane Database Syst Rev
Fig. 23.13.8 Anchoring the catheter to the 2000;2:CD000949.

Fig. 23.13.6 Advancing the catheter. umbilical cord. Barrington KJ. Umbilical artery catheters in the newborn:
Effects of position of the catheter tip. Cochrane Database
Syst Rev 2000;2:CD000505.
Cohen RS, Ramachandran P, Kim EH, Glasscock GF.
Retrospective analysis of risks associated with an
(Fig. 23.13.7). Low catheter insertion umbilical artery catheter system for continuous monitoring
Complications of arterial oxygen tension. J Perinatol 1995;15(3):1958.
distances are based on patient weight Cunningham FJ, Engle WA, Rescorla FJ. Paediatric vascular
and are available on graphs. High Haemorrhage. access and blood sampling techniques. In: Roberts JR,
Hedges JR, editors. Clinical procedures in emergency medicine.
catheter insertion distance is Infection. 3rd ed. Philadelphia, PA: WB Saunders; 1998. p. 281308.
approximately 60% of the distance Air embolism. Green C, Yohannan MD. Umbilical arterial and venous
catheters: Placement, use, and complications. Neonatal
between the infants umbilicus and Vessel dissection. Netw 1998;17(6):238.
either shoulder. Perforation. Kim JH, Lee YS, Km SH, et al. Does umbilical vein
 Anchor the catheter to the umbilical If umbilical venous catheters are left in catheterisation lead to portal venous thrombosis?
Prospective US evaluation in 100 neonates. Radiology
cord with a purse-string suture then the liver, sclerosing substances injected 2001;219(3):64550.
McAneney C. Umbilical vessel catheterisation. In:
secure the catheter to the abdomen with via the catheter can cause hepatic Dieckmann RA, Fiser DH, Selbst SM, editors. Paediatric
tape (Fig. 23.13.8). damage. emergency and critical care procedures. St Louis, MO:
 Verify placement of the radio-opaque Thromboembolic events, particularly to Mosby; 1997.
Weber HS. Transumbilical artery interventions in the neonate.
catheter by thoracoabdominal X-ray. kidneys, intestines and the lower J Invas Cardiol 2001;13(1):3943.

505
 23.14 Electrical countershock
Ronald A. Dieckmann Conor Deasy

Background
VF, such as cardiopulmonary resuscitation  Newer models feature lower power
(CPR), improved gas exchange, correction outputs to deliver lower energy
When a childs heart deteriorates into of acidosis and vasopressor support of perfu- countershocks.
ventricular fibrillation (VF) or pulseless sion may change the type of VF from fine,
ventricular tachycardia (VT), there is usually low amplitude VF to coarse, high amplitude
a severe systemic insult, such as profound VF. Coarse, high amplitude VF is more
responsive to electrical countershock than
Standard preparation
hypoxia, ischaemia, acidosis, electrocution,
or myocarditis. Sometimes the child has a fine, low amplitude VF. After electrical coun- Open airway. Look listen and feel for no
congenital heart defect such as idiopathic tershock for a ventricular dysrhythmia, more than 10 seconds. If the child is not
hypertrophic subaortic stenosis or long QT administer amiodarone to reduce recur- breathing or is not breathing normally
syndrome. Any detectable ventricular rhythm, rence. In addition, in children with torsades then deliver 5 rescue breaths. Take no
even fibrillation, suggests that there is still de pointes VT, give magnesium. more than 10 seconds to assess for signs
some perfusion of the heart therefore VF In certain clinical scenarios, other inter- of life or a pulse. If there are no signs
or pulseless VT is a survivable presenting ventions in addition to countershock may of life, no pulse, or a pulse rate less than
rhythm. This is in distinction to the clinical also improve survival, e.g. fluids for hypovo- 60 beats per minute commence chest
circumstances with asystole or bradyasystole, laemia, potassium-lowering treatment for compressions.
the most common paediatric cardiopulmo- hyperkalaemia, calcium for hypocalcaemia, If child is pulseless, begin closed
nary arrest rhythms. In these rhythm pre- pericardiocentesis for pericardial tampo- chest compressions without delay,
sentations, the degree of hypoxia is usually nade and needle thoracostomy for tension delivering 100 chest compressions per
so severe that treatment of any kind is less pneumothorax. minute. For lone rescuers with victims
to work. Ventricular dysrhythmias represent of all ages the chest compression to
approximately 510% of presenting paedia- ventilation rate is 30:2. For health-
tric cardiopulmonary arrest rhythms. SVT Indications for care providers performing 2-rescuer
represents <1%. Resuscitation from such pul- asynchronous CPR for infants and children: 15:2
seless rhythms also includes ventilation, oxy- countershock (and 3:1 for neonates).
genation, and effective chest compressions. (defibrillation)  Turn on the defibrillator but do not
Electrical countershock for tachydysrhy- activate the synchronised mode.
thmias is a common life-saving procedure in
VF. Select the proper paddles size. Use the
adult emergency cardiac care and is the most
Pulseless VT. 8-cm adult paddles if these will fit on the
effective treatment for sudden cardiac arrest chest wall; otherwise, use the 4.5-cm
from ventricular dysrhythmias in all age Indications for synchronous paediatric paddles. Self-adhesive
groups. The concept behind electrical counter- electrode pads are simpler, safer and
countershock
shock is that if a properly delivered electrical more efficient than the conventional
dose is applied to the heart in a magnitude SVT with shock and pulse but no vascular paddles when defibrillating or
that does not damage the myocardium, all access rapidly available. cardioverting. Paediatric pads are
electrical activity will be momentarily ablated VT with shock and pulse present. usually recommended for any child
through depolarisation, and the intrinsic Atrial fibrillation or atrial flutter with under 1015 kg.
pacemaker of the heart will resume rhythm shock. Prep paddles with electrode jelly,
control (automaticity). When the child is pulse- Stable VT or SVT in collaboration with paste, or saline-soaked gauze pads, or
less and has a ventricular dysrhythmia the specialist. use self-adhesive defibrillator pads.
most effective treatment is immediate electri- Do not let jelly or paste from one site
cal asynchronised countershock (defibrilla- touch the other and form an
tion) performed as quickly as possible with Contraindications electrical bridge between sites, which
the appropriate technique. In contrast, if a Conscious patient with good perfusion. could result in ineffective defibrillation
child has a pulse, and the rhythm is either or skin burns.
SVT or VT, use synchronised countershock. Establish appropriate electrical charge
Electrical countershock is frequently which is 4 J kg 1 asynchronous for VF/
effective in children with ventricular dys-
Equipment pulseless VT (Table 23.14.1).
rhythmias but it will not provide benefit in Standard defibrillator (monophasic or Charge the defibrillator while another
asystole, bradyasystole or pulseless electri- biphasic). rescuer continues chest compressions.
cal activity (PEA). Essential treatments for Automated external defibrillator (AED). Stop CPR, verify rhythm and if shockable

506
23.14 ELECTRICAL COUNTERSHOCK
23

COMMON PROCEDURES
Table 23.14.1 Appropriate electrical charge for countershock If still VF/VT, give a second shock at
4 J kg 1.
Dysrhythmia Mode Charge After 2 minutes, if no response with an
VF Asynchronised (defibrillation) Initial shock 2 J kg1, additional shock, administer
Pulseless tachycardia subsequent shocks 4 J kg1
amiodarone (5 mg kg 1) for
VT with pulse Synchronised 0.51.0 J kg1 defibrillation resistant rhythms.
SVT
Atrial fibrillation and atrial
 Treat reversible causes, remembering
flutter with shock the Hs and the Ts: Hypoxia,
Hypovolaemia, Hypo/hyperkalaemia,
Hypothermia, Tension pneumothorax,
deliver 4 J kg 1 asynchronous and Tamponade (cardiac), Toxins,
immediately resume chest compressions Thromboembolism.
without pulse or rhythm check.  Treat other rhythms that develop such
as PEA or asystole.
 Therapeutic hypothermia is
Standard procedure recommended if the victim fails to
resume consciousness after
Apply the paddles or self-adhesive resuscitation.
electrode pads directly to the skin of the
chest wall with firm pressure, placing
one defibrillator pad or paddle on the
chest wall just below the right clavicle, AED procedure
and one in the left anterior axillary line, A standard AED can be used in children
under the left nipple (Fig. 23.14.1). over 1 year. If it is likely that the AED will
As another option, use the anterior be used for children the purchaser
posterior position (Fig. 23.14.2) however should check that the performance of
it is not then possible to continue CPR the particular model has been tested
easily. against paediatric arrhythmias. Purpose-
 Clear the nearby area of personnel made paediatric pads, or programs
except the person delivering chesty which attenuate the energy output of an
compressions so that no-one is in AED, are recommended for children 18
contact with the patient, gurney or years. However, if this is not available
Fig. 23.14.2 Placement of paddles on the
childs chest and back. an unmodified adult AED may be used in
children over 1 year. In children less than
1 year there is little evidence beyond
equipment. Charge the defibrillator case reports so the decision may be
while chest compressions are ongoing. taken on a risk or benefit ratio which in
Then stop chest compressions, if the this setting will probably be in favour of
patient is in a shockable rhythm (VF/ using whatever is available. Several
pulseless VT) then deliver the AEDs that are currently on the market
asynchronous shocks. If not in a have paediatric attenuating devices to
shockable rhythm press disarm on the reduce the electrical dose delivered to
defibrillator which will dump the charge. children younger than 8 years, including
Another way of dumping the charge is infants; whilst these are the preferred
by turning the defibrillator off. AED, if not available the responder
Immediately resume CPR. should use a standard AED rather
This is followed by immediate than delay the delivery of a potentially
uninterrupted cardiopulmonary life-saving intervention.
resuscitation (15:2) for 2 minutes then Open airway ventilate while preparing
rhythm is assessed. equipment.
If there has been no rhythm response, If child is pulseless, begin chest
immediately recommence chest compression.
compressions and administer adrenaline Turn AED on.
(epinephrine) 10 mcg kg 1. Adrenaline  Attach AED electrodes to chest wall
Fig. 23.14.1 Placement of paddles on the (epinephrine) 10 mcg kg 1 should be (right clavicle, and one in the left anterior
childs chest. given every 35 minutes during CPR. axillary line, under the left nipple.).

507
23.15 PERICARDIOCENTESIS

Allow the device to analyse rhythm. unknown. When shocks are indicated for Use sedation with analgesia for conscious
If countershock indicated, clear the area VF or pulseless VT, use an initial energy children receiving countershocks.
and deliver electricity. dose of 2 to 4 J kg 1 of either waveform; Assure the device is in the synchronised
Do CPR and check airway, breathing and doses higher than 4 J kg 1, especially if mode (SYNC) before every
circulation for 2 minutes, then delivered with a biphasic defibrillator, countershock.
re-analyse rhythm and deliver may also be safe and effective.
additional countershocks as indicated. When performing chest compressions
push hard, push fast, and minimise Further reading
interruptions of chest compression; allow Biarent D, Bingham R, Eich C, et al. European Resuscitation
Complications full chest recoil, and dont provide Council Guidelines for Resuscitation 2010 Section 6.
Paediatric life support. Resuscitation 2010;81(10):136488.
excessive ventilation. Kleinman ME, de Caen AR, Chameides L, et al. Part 10: Pediatric
Ineffective delivery of countershock Take no more than 10 seconds for the basic and advanced life support: International Consensus
because of failure to charge, improper on Cardiopulmonary Resuscitation and Emergency
pulse check. It is often difficult to detect a Cardiovascular Care Science With Treatment
positioning on the chest, incorrect
pulse in an infant or child; the carotid Recommendations. Circulation 2010;122(16 Suppl 2):
paddle size, or improper conduction S466515.
pulse in the neck in the case of a child, American Heart Association Guidelines for Cardiopulmonary
medium (e.g. alcohol swabs).
the brachial pulse on the inner aspect Resuscitation and Emergency Cardiovascular Care.
Burns on the chest wall.
of the upper arm in the case of an infant Circulation 2005;112(24 Suppl):IV1203.
APLS: Student Manual: The Pediatric Emergency Medicine
Failure to clear before voltage discharge,
are the sites where pulse should be Course by The American Academy of Pediatrics. 4th ed.
leading to electrical shock of a team PALS Provider Manual (Paperback) American Heart
checked. Association.
member or bystanders.
For a child with VF or pulseless VT, use the Markenson D, Pyles L, Neish S; American Academy of
Tachydysrhythmias.
asynchronised mode the defibrillator Pediatrics Committee on Pediatric Emergency Medicine;
American Academy of Pediatrics Section on Cardiology
Bradycardia.
will not discharge in the synchronised and Cardiac Surgery. Ventricular fibrillation and the use
Myocardial damage or necrosis.
mode. of automated external defibrillators on children. Pediatrics
2007;120(5):e136879. Epub 2007 Oct 29. Review.
Cardiogenic shock.
Failure to apply paddles firmly to the PubMed PMID: 17967922.
Embolic phenomena.
chest wall, using too small paddles or too Cecchin F, Jorgenson DB, Berul CI, et al. Is arrhythmia detection
by automatic external defibrillator accurate for children?
low energy will decrease effectiveness. Sensitivity and specificity of an automatic external
defibrillator algorithm in 696 paediatric arrhythmias.
TIPS Self-adhesive electrode pads are simpler, Circulation 2001;103(20):24838.
safer and more efficient than the Deboer S, Sicilia MR, Seaver M, et al. Paediatric defibrillation:
Concerns and opportunities. Paediatr Emerg Care 2002;18
Biphasic defibrillators have better speed conventional paddles when defibrillating (6):4668.
and efficiency on the first shock than or cardioverting. Dieckmann RA, Brownstein D, Gausche M. Textbook for
paediatric education for prehospital professionals. Sudbury,
older monophasic defibrillators. Either uncuffed or cuffed tracheal tubes MA: Jones & Bartlett; 2001. p. 2835.
Minimize the interval between stopping may be used in infants and children. Dieckmann RA, Fiser D, Selbst S. Illustrated textbook of
paediatric emergency and critical care procedures. St Louis,
compressions and delivering shocks and Endotracheal tube placement should MO: Mosby; 1997. p. 32336.
always resume CPR immediately after be confirmed with the use of carbon Silka MJ, Kron J, Walance CG, et al. Assessment and follow-up
of paediatric survivors of sudden cardiac death. Circulation
shock delivery. dioxide detection. 1990;82(2):3419.
The correct energy dose for defibrillation If the child has SVT or VT and is in Field JM, Hazinski MF, Sayr MR, et al. American Heart
Association Guidelines for Cardiopulmonary Resuscitation
(with either a monophasic or biphasic shock and unresponsive, give electrical and Emergency Cardiovascular Care Science. Circulation
device) in infants and children is countershock at 0.51.0 J kg1. 2010;122:S64056.

23.15 Pericardiocentesis
Ronald A. Dieckmann

a penetrating injury in the high-risk zone distant heart sounds, neck vein distension
Background
formed by the triangle of the two nipples and hypotension (Becks triad). However,
The pumping heart is extremely sensitive to and the sternal notch. The risk of death from because other blood loss may concurrently
rapid accumulation of pericardial fluid. Small a penetrating injury to the heart is higher reduce central venous pressures, and
amounts of fluid acutely increase intraperi- from the haemopericardium than from direct because audible intensity of heart sounds
cardial pressure and may significantly injury to the myocardium, coronary arteries may be difficult to distinguish in children,
impede venous return and cardiac output. or cardiac valves. suspect tamponade in any child with a
Haemopericardium is the most common The classical presentation of paediatric high-risk chest-wall penetration in the
fluid collection blood collects between cardiac tamponade is a child with a pene- triangle, even tiny, with signs of a possible
the visceral and parietal pericardium after trating anterior chest-wall injury who has perfusion deficiency tachycardia, poor skin

508
23.15 PERICARDIOCENTESIS
23

COMMON PROCEDURES
colour or temperature, delayed capillary In the non-emergent situation, an
refill or diminished pulses. uncorrected bleeding diathesis is a Standard preparation
In contrast to haemopericardium, slow fluid contraindication. Place the child in a semi-reclining
accumulation in the pericardium from other position (3045 degrees).
aetiologies, such as viral, bacterial or myco- Establish secure vascular access and
bacterial infection, or from immunological/ Equipment apply cardiac monitor.
collagen vascular disease or malignancy, has
Emergent procedure
 Secure the airway if necessary.
a minimal effect on cardiac function. The dis-
Povidone-iodine solution.
If the procedure is non-emergent,
tensible pericardium readily accommodates administer sedation.
3050-mL syringe.
slow fluid collections and the presentation
 2.5 or 3.5-inch 1820-gauge spinal
Identify the subxiphoid entry site
of such patients often does not involve per- (Fig. 23.15.1), below and to the left of
fusion abnormalities. In this setting, needle needle.
the xiphoid process.
Ultrasound machine.
pericardiocentesis is best accomplished with Prepare the area widely with povidone-
echocardiographic guidance. iodine solution.
Pericardiocentesis is a needle procedure Non-emergent procedure Infiltrate the entry site with lidocaine in
for either life-saving decompression of acute 1% lidocaine.
conscious patients.
cardiac tamponade, or for diagnostic evalua- 25-gauge needle.
tion of a non-emergent pericardial effusion.  Two 5-mL syringes.
Non-emergent procedure
Rapid removal of acute haemopericardial Two 68-cm (2.5 or 3.5-inch)
In addition to the preparation:
fluid by needle pericardiocentesis is some- 1820-gauge spinal needles.
times life saving. The decompression of Three-way stopcock. Attach the correctly-sized spinal needle
the pericardium will temporarily restore 3050-mL syringe. to a stop cock and 30-mL syringe.
myocardial performance in a perfusing 18 and 20-gauge over-the-needle Attach one clip of the cable to the hub of
patient, until reaccumulation occurs. Hence, catheters. the spinal needle and one clip to the
needle pericardiocentesis is a temporising  Cable with alligator clip at each end. V-lead of the ECG machine (Fig. 23.15.2).
measure that precedes open surgical  Sample containers.  Turn the ECG to the V-lead position.
decompression. Sometimes, it is a heroic  Electrocardiograph (ECG) machine.
intervention in cardiopulmonary arrest. In Echocardiograph (desirable).
non-emergent settings, an indwelling cathe- Ultrasound machine. Procedure
ter may be necessary to prevent fluid reaccu-
Establish location of effusion with
mulation. The procedure can be done with or
Indwelling catheter ultrasound (Fig. 23.15.3).
without electrocardiographic or echocardio-
Flexible guidewire or J-wire. Under constant ultrasound guidance,
graphic guidance, depending on the urgency
Plastic over-the-wire catheter. aim the needle to the left shoulder and
of the clinical situation. Because the major-
ity of needle pericardiocentesis procedures
are performed on children in cardiopul-
monary arrest, who have low probability
of survival, the efficacy and safety of the
procedure are poorly understood.

Indications
Cardiac tamponade.
Pericardial effusion of unknown aetiology.

Contraindications Left circumflex

Right auricle coronary artery
There are no contraindications in the
Right coronary artery Left ventricle
child presenting emergently with shock
or cardiopulmonary arrest and Left anterior
evidence of acute tamponade. If descending
coronary artery
equipment and appropriate
personnel are present, open thoracotomy
Right ventricle
may be preferred over needle
pericardiocentesis. Fig. 23.15.1 Subxiphoid entry site.

509
23.15 PERICARDIOCENTESIS

Fig. 23.15.4 Injury current with needle in

myocardium.

to ECG
Haemothorax.
Diaphragm perforation.
Fig. 23.15.3 Pericardial effusion. Image Bowel or stomach perforation.
courtesy of Dr. Ronald Dieckmann. Infection.
Cardiac arrest.
and place the larger catheter with side
holes over the wire.

Tips
Non-emergent procedure
After the preparation: If the effusion is not causing tamponade,
identify the size and location of the
Do constant ECG monitoring.
effusion with echocardiography,
Fig. 23.15.2 Using the ECG for guidance.
If the needle punctures the ventricular
computerised tomography or magnetic
epicardium, ST segment elevation or a
resonance imaging before doing
dysrhythmia may occur (Fig. 23.15.4).
pericardiocentesis.
 If an injury pattern develops,
enter at a 3045 degree angle Always use ultrasound guidance.
withdraw the needle slightly or
(Fig. 23.15.2) toward the anatomic If there is a large volume of aspirated
reposition more medially.
location of the effusion. blood, the needle may be in the
 Apply continuous negative pressure
Always use the ultrasound to guide the
ventricle. The presence or absence of
needle to the area of largest fluid
and advance the needle until there is clotting does not reliably indicate
accumulation.
a pop as the needle enters the needle location.
pericardium.
When blood flows into the syringe, place
a haemostat at the skin surface to
Complications Further reading
prevent further advancement of the
Dieckmann RA, Fiser D, Selbst S. Illustrated textbook of
needle. Ventricular puncture. paediatric emergency and critical care procedures. St Louis,
Aspirate until blood is evacuated from Atrial puncture. MO: Mosby; 1997. p. 5925.
Lee C, Mason LJ. Paediatric cardiac emergencies. Anesthesiol
the pericardium. Coronary artery laceration. Clin N Am 2001;19(2):287308.
If prolonged drainage is necessary, Cardiac dysrhythmia. Tsang TS, El-Najdawi EK, Seward JB, et al. Percutaneous
echocardiographically guided pericardiocentesis in
advance the guidewire or J-wire through Haemopericardium. paediatric patients: Evaluation of safety and efficacy. J Am
the needle, remove the spinal needle, Pneumothorax. Soc Echocardiogr 1998;11(11):10727.

510
 23

COMMON PROCEDURES
23.16 Transurethral catheterisation and
suprapubic bladder aspiration
Conor Deasy Judith Klein

Background Indications Significant abdominal distension.

Recent abdominal surgery.
Obtaining an uncontaminated specimen Indications for transurethral
In addition, if ultrasound is available, do not
of urine is essential to diagnose urinary catheterisation
perform suprapubic aspiration if the bladder
tract infection (UTI). UTIs are a common Collection of a diagnostic urine specimen. diameter is less than 3.5 cm or if a volu-
aetiology of fever and serious bacterial Intermittent bladder decompression for metric scan demonstrates less than 10 mL
infection in infants. In children who cannot neurogenic bladder.
of urine (Fig. 23.16.1). Under these circum-
provide clean-catch urine specimens, the Urological study. stances, delay the procedure because it is
diagnosis of UTI involves obtaining a urine Measurement of urine output in critically unlikely to be successful.
sample directly from the bladder. Urine ill or injured child.
specimens obtained via bags attached tem- Acute urinary retention.
porarily on to the perineum are frequently
contaminated samples, particularly with Indications for suprapubic Transurethral
infant girls. Waiting for bagged urine speci- bladder aspiration catheterisation
mens is also time-consuming and frustrating Collection of a clean diagnostic urine speci-
in the emergency department (ED) setting. Equipment
men when transurethral catheterisation is The equipment required for transurethral
Hence, urinary bag specimens frequently impossible or unsuccessful.
confuse diagnosis of UTI, and often result catheterisation is often available in prepack-
in false positive specimens and unnecessary aged trays to which only the appropriately
treatment. sized catheter need be added. Determine
Obtaining an appropriate urine specimen Contraindications the appropriate catheter size either by using
requires either transurethral catheterisation a length-based resuscitation tape, by using
Urethral catheterisation
or suprapubic aspiration. Unfortunately, the formula 2 (endotracheal tube size),
Obvious pelvic trauma. or by using a table listing equipment sizes
in very young females and circumcised Blood at the urethral meatus.
males with small foreskins, the urethra by age (Table 23.16.1). Remember to use
Upward displacement of the prostate in lidocaine jelly in a syringe, particularly when
can be difficult to find and even more diffi- males following trauma.
cult to cannulate. In these cases, perform catheterising the male urethra.
Perineal haematoma.
suprapubic aspiration of urine directly from
the bladder, whenever possible under ultra- In these circumstances, perform a retrograde
Preparation
urethrogram prior to cannulation of the ure-
sound guidance. This is an easy procedure Review the male and female anatomy of
that requires minimal equipment. Supra- thra. Placement of a catheter without such a
the perineum.
study could convert a partial urethral tear
pubic aspiration provides a sterile urine Have all equipment readily near the child.
specimen with less trauma to the infant into a complete transection. Urethral inju-
than repeated, failed attempts at urethral ries are uncommon in females because of
catheterisation. the relatively short distance between the
Transurethral catheterisation and place- urethral meatus and the bladder. Bladder
ment of an indwelling bladder catheter injuries, however, are more common in
is also useful in the management of critically females and children in general because of
ill and injured children, to monitor urine the higher abdominal location of the organ
output in the setting of shock or trauma. in children versus adults. If blood appears
Urine output is one of the best measures following urethral catheterisation in trauma,
of core perfusion and can help guide fluid perform a retrograde cystogram to search
resuscitation of a child in shock. The combi- for bladder injury.
nation of heart rate and urinary bladder
output are two excellent measurements Suprapubic aspiration
of intravascular volume and response to Coagulopathy. Fig. 23.16.1 Using ultrasound for suprapubic
treatment. Thrombocytopenia. aspiration.

511
23.16 TRANSURETHRAL CATHETERISATION AND SUPRAPUBIC BLADDER ASPIRATION

catheter is placed in a child with urethral

Table 23.16.1 Catheter sizing for children
trauma.
Male (French) Girl (French) Type Urethral strictures can also occur if
Newborn 3 3-5 Straight catheters are left indwelling for a
prolonged period of time.
12 years 5 5 or 8 Straight

35 years 8 8 Balloon-tipped Tips

610 years 8 or 10 8 or 10 Balloon There are circumstances in which
urine specimens can be obtained
1012 years 10 or 12 12 Balloon
non-invasively using urinary bags.
13 years and older 12 12 or 14 Balloon For example, in the setting of trauma,
microscopic haematuria (urinanalysis
>1025 red blood cells (RBC) per
high-powered field) can help detect
 Place the appropriately sized catheter If the catheter is being left in, inflate the occult intra-abdominal and genitourinary
and the syringe with lidocaine jelly balloon, if there is one, and pull back trauma in children.
(Lidojet) on to the sterile field. until resistance is met. Inflate the In uninjured children, a bag urine
Have an assistant gently restrain the balloon with the amount of fluid listed specimen is acceptable if the intent of
child in a frog-leg position to prevent on the catheter itself. diagnostic specimen collection is not
disruption of the sterile field and  Connect the catheter to a sterile closed the detection of infection.
unnecessary trauma due to patient drainage system. With a balloon-tipped
movement. catheter, secure the catheter or tubing
If the child is older, discuss the to the inner thigh with tape. With
Suprapubic aspiration
procedure, clearly relate the steps of the non-balloon catheters, tape the tube
procedure to the child, and move slowly. to the shaft of the penis in a spiral Equipment
fashion or to the proximal inner thigh Fig. 23.16.2 illustrates the equipment required
next to the labia majora in females. for suprapubic aspiration. Use an ultrasound
Procedure  Remember to return the foreskin to its to improve the likelihood of success.
Use sterile procedure. original position in uncircumcised males
With a male patient, use the to prevent paraphimosis and penile Preparation
non-dominant hand and a gauze to constriction. Review the anatomy of the lower
retract the foreskin and expose the abdomen (Fig. 23.16.3).
meatus. In the circumcised male, the Restrain the child in a frog-leg position
Complications
gauze is still useful to maintain a using an assistant who can help soothe
Table 23.16.2 lists the complications of ure-
firm grasp on the penis during the child.
thral catheterisation.
catheterisation.  Have all equipment near the patients
 With a female patient, use the thumb The most common complication of abdomen including the ultrasound.
and forefinger of the non-dominant urethral catheterisation is infection,
hand to spread the labia majora and particularly if a catheter is left indwelling Procedure
expose the urethra. Clean the urethral for a prolonged period of time. Single Locate a spot approximately 1 cm
meatus three times with povidone- urethral catheterisations for collection of cephalad of the pubic symphysis in the
iodine solution, making sure to swab diagnostic specimens are rarely midline.
from front to back in females to avoid complicated by infections.
contamination. Urethral or bladder trauma can also occur
Insert a few millilitres of lidocaine jelly with urethral catheterisation, particularly
using a syringe device to provide topical with oversized catheters or when a
anaesthesia, particularly in male
patients.
Lubricate the catheter with the same
jelly and insert the catheter into the Table 23.16.2 Complications of urethral
urethra. Do not force the catheter catheterisation
against resistance. Once urine flows Urinary tract infection
Urethral or bladder trauma
through the catheter, stop advancing. Haematuria
Discard the first 1 mL of urine, which Paraphimosis
Vaginal catheterisation
may be contaminated, and collect a Urethral strictures Fig. 23.16.2 Equipment for suprapubic
specimen. aspiration.

512
23.16 TRANSURETHRAL CATHETERISATION AND SUPRAPUBIC BLADDER ASPIRATION
23

COMMON PROCEDURES
Large and small bowel 1020

Pubic Umbilicus
symphysis
Bladder 22-gauge needle

Pubic bone
Bladder

Fig. 23.16.3 Anatomy of the lower abdomen.

Use ultrasound to determine if the Fig. 23.16.4 Inserting the suprapubic needle.
bladder is full enough. If the bladder
diameter is 3.5 cm or if 10 cm3 of urine is
present on a volumetric bladder scanner, Infections around the puncture site can the suprapubic aspiration should
the procedure is likely to be successful. occur, but are unusual with a sterile be successful. If the volume is
 Prepare the skin at this spot with technique. less than this, or the bladder diameter
povidone-iodine solution. Microscopic haematuria (<10 RBC per is less than 3.5 cm then give fluids
In males, provide gentle penile high powered field) can occur transiently and wait for more urinary volume to
compression prior to skin cleansing to following aspiration. be present.
prevent voiding prior to the procedure. It is a good tip to always have a
Using a 3-cm3 syringe attached to either container at the ready to collect a clean
a 23-gauge butterfly or 22-gauge Tips catch of urine as pressure with the probe
3.5-cm (1.5-inch) needle, insert the Suprapubic aspiration is not often in the suprapubic area may stimulate
needle at an angle approximately required in the ED. However, when urination.
1020 degrees cranially (Fig. 23.16.4). urethral catheterisation is unsuccessful,
Remember that the bladder lies higher it is a straightforward and safe
in the abdomen in children than in procedure that provides diagnostic
adults. specimens with a contamination rate Further reading
Provide constant backpressure on the approximately half that of transurethral Bell LM. Transurethral bladder catheterization.
syringe. catheterisation. In: Dieckmann RA, Fiser DH, Selbst SM, editors.
Aspirate the urine specimen, remove the Ultrasound guidance of suprabubic Paediatric emergency and critical care procedures. St Louis,
MO: Mosby; 1997. p. 533.
needle, and place a small bandage on aspiration improves the success rate of Bell LM. Suprapubic bladder aspiration. In: Dieckmann RA,
Fiser DH, Selbst SM, editors. Paediatric emergency and
the site. the procedure considerably, from 36% critical care procedures. St Louis, MO: Mosby; 1997.
to 90%. The ultrasound is performed Garcia-Nieto V, Navarro JF, Sanchez-Almeida E,
Garcia-Garcia M. Standards for ultrasound guidance of
Complications to determine whether there is enough suprapubic bladder aspiration. Paediatr Nephrol 1997;11
Bowel perforation. This is a rare urine present for a suprapubic tap to (5):6079.
Liso JC, Churchill BM. Paediatric urine testing. Paediatr Clin N
complication. The only reports of bowel be successful, not to direct needle Am 2001;48(6):42540.
penetration are in children with markedly placement. The ultrasound is Pollack CV, Pollack ES, Andrew ME. Suprapubic
bladder aspiration versus urethral catheterization in
distended abdomens. Even if needle performed using gel placed on the ill infants: Success, efficiency and complication rates.
penetration of the bowel occurs, there is ultrasound probe head to improve the Ann Emerg Med 1994;23(2):22530.
Schneider RE. Urologic procedures. In: Roberts JR, Hedges JR,
no treatment and infection is extremely quality of the image. If the bladder editors. Clinical procedures in emergency medicine. 3rd ed.
unusual. contains greater than 20 mL of urine Philadelphia, PA: WB Saunders; 1998. pp. 4557.

513
 23.17 Penile zipper injury
Michelle Lin

Background In a child whom you anticipate is going Provide supportive wound care to
to be too agitated or fearful of the the injury site using warm soaks
The most common cause of penile entrap- procedure, consider procedural sedation. and apply a dressing. In severe soft
ment is an unintentional zipper injury. Most tissue injuries, however, suture
commonly seen in boys aged 26 years, this repair and/or debridement of the
injury occurs while the child is zipping up his Procedure wound may be necessary under
pants. In addition to being painful, this con- the guidance of a consulting
dition is a tremendously anxiety-inducing Soak the exposed external genitalia in urologist.
injury for the child. mineral oil for 10 minutes to lubricate
The patient usually presents for medical the contact points. Occasionally, this
attention only after failed attempts at self- manoeuvre alone will allow the zipper to
extrication. There are two patterns of easily retract so that the tissue is freed.
entrapment for the often-macerated and Anaesthetise the entrapped tissue using Complications
oedematous soft tissue. First, the movable 1% lidocaine without adrenaline The primary complication in penile zipper
zipper head may catch onto the external (epinephrine). removal is pain. This occurs most commonly
genitalia tissue. Second, the interlocking  For the external genitalia entrapped in during the positioning and preparation of
teeth along the zipper path may entrap the zipper sliding device, use the bone or the patient. Gentle manipulation and posi-
some soft tissue. In order to disassemble a wire cutter to break the thin median bar tioning are essential for this procedure.
zipper, one must understand its anatomy. that separates the two sliding tracks.
Specifically, a thin median bar is the only The front and back plates will fall apart,
structure separating the two tracks on the releasing the entrapped skin
zipper-sliding device, interlocking them. (Fig. 23.17.1).
For the external genitalia entrapped in Tips
the interlocking teeth, cut the zipper just Cutting off the pant legs (and possibly
Indications inferiorly to the entrapment site. The even the rest of the pants around
two interlocking rows of teeth will, with the zipper) can provide significant pain
Soft-tissue entrapment in a zipper. gentle manipulation, slowly separate relief.
from one another and release the skin. Because pain is a significant fear in
these patients, a compromise between
Contraindications local anaesthesia and procedural
None. sedation is a regional block. A dorsal
penile block will anaesthetise the
entire penis.
Equipment
Bone or wire cutters.
Mineral oil.
 Local anaesthesia (1% lidocaine without Further reading
adrenaline (epinephrine), syringe, Kanegaye JT, Shonfeld N. Penile zipper entrapment: A simple
30-gauge needle). and less threatening approach using mineral oil. Paediatr
Emerg Care 1993;9(2):901.
Lundquist ST, Stack LB. Diseases of the foreskin, penis, and
Cut the median bar urethra. Emerg Med Clin N Am 2001;19(3):52946.
Nolan JF, et al. Acute management of the zipper-entrapped
Preparation and positioning penis. J Emerg Med 1990;8(3):3057.
Woodward GA. Penile zipper injuries. In: Dieckmann RA, et al.,
Carefully position the patient supine or editors. Paediatric emergency and critical care procedures.
St Louis, MO: Mosby; 1997. p. 4256.
semisupine in the caregivers lap, being Fig. 23.17.1 Diagram of zipper separation Wyatt JP, Scobie WG. The management of penile zip
careful not to jostle the pants or zipper. after cutting median bar. entrapment in children. Injury 1994;25(1):5960.

514
 23

COMMON PROCEDURES
23.18 Lumbar puncture
Ronald A. Dieckmann

Focal neurological signs. administer oxygen and begin cardiac

Background monitoring and pulse oximetry.
Significant intracranial pressure elevation
Lumbar puncture (LP) is a time-honoured (hypertension, bradycardia, and apnoea). Secure the airway if necessary.
method for obtaining cerebrospinal fluid  Place the child in a lateral recumbent
In children with possible significant intracra-
(CSF) for diagnostic evaluation of suspected (Fig. 23.18.1) or sitting position
nial pressure elevation from oedema or
central nervous system (CNS) abnormalities. (Fig. 23.18.2) at the edge of the gurney.
LP is an essential procedure in children with
space-occupying brain lesions, brain hernia-
If the child is conscious, consider
tion after LP is an important consideration.
suspected meningitis, and represents the only sedation.
simple method of obtaining fluid for rapid
Obtain a computerised axial tomography
Identify the entry site at the L3L4
(CT) brain scan before the LP.
diagnosis and appropriate pathogen analysis interspace.
for specific treatment. While LP is most com- Don sterile gloves and prepare the
monly performed to diagnose meningitis, it area widely with povidone-iodine
is also a useful procedure to help identify Equipment solution.
encephalitis, CNS haemorrhage, malignancy, If the child is conscious, infiltrate the
Pre-packaged LP trays are available in most
and other rare metabolic and degenerative entry site with 1% lidocaine and a
hospitals.
conditions of childhood. Occasionally, LP is a 25-gauge needle, then the deeper
therapeutic procedure in treatment of such 18-, 20- or 22-gauge styleted spinal tissue to the level of the paraspinous
conditions as pseudotumour cerebri, or for needles: ligaments with a 20-gauge needle.
administration of intrathecal antibiotics or 3.5-cm (1.5-inch) for neonates, infants,
chemotherapeutic agents. young children
LP is a relatively simple procedure in infants 6-cm (2.5-inch) for older children and Lateral decubitus position
and children but has a known complication adolescents Flex the childs knees and torso, but do
rate, with both minor and major sequelae. 9-cm (3.5-inch) for large patients. not over-flex the neck and compress the
Appropriate patient selection, preparation Povidone-iodine solution. airway.
and patient positioning, and aseptic tech-  1% lidocaine. Have an assistant hold the child firmly in
nique will avoid most complications. Do not 25-gauge needle. the decubitus position.
perform LP immediately on haemodynami- 20-gauge needle.
cally unstable patients, who first require 35-mL syringe.
meticulous management of airway, breathing Four capped sterile specimen tubes. Sitting position
and circulation, or on patients with clinical  Manometer (for children aged Have an assistant hold the child upright
signs of focal CNS processes or signifi- 2 years). with the hips flexed. Hold the childs
cantly elevated intracranial pressure. In such  Stopcock. right elbow and knee with the left hand
patients, obtain blood cultures and administer and the left elbow and knee with the
antibiotics first, then perform LP after stabili- right hand.
sation and brain imaging, when indicated.
Preparation and positioning Put the thighs against the abdomen and
flex the trunk.
In most patients, establish secure  Keep the craniospinal axis perpendicular
Indications vascular access, obtain blood cultures to the transverse plane of the line

2 and appropriate blood tests, connecting the iliac crests.
Clinical symptoms and signs of meningitis
or encephalitis in neonate, infant or child.
Evaluation of sepsis in infant <3 months liac crest
of age.
Seizure with fever in child <12 months
of age.
Intrathecal drug administration.

Contraindications
L3,4 interspace
Haemodynamic instability.
Active seizure activity. Fig. 23.18.1 Lateral decubitus position.

515
23.18 LUMBAR PUNCTURE

for a child in the lateral decubitus Temporary paralysis.

position is 520 cm, but a struggling Epidermoid tumours.
child may artificially elevate this Discitis.
number. Do not attempt an opening Epidural haematoma.
pressure in a sitting child, because it is Epidural abscess.
unreliable. Osteomyelitis.
 Limit CSF withdrawal to 2 mL in Traumatic LP.
neonates, or 36 mL in infants and
older children. Put the CSF in 34
CNS
separate tubes, labelled #1, #2, #3,
and #4.
Subarachnoid haemorrhage.
Fig. 23.18.2 Sitting position. Subdural haemorrhage.
 Replace the stylet and remove the
needle.
Brainstem herniation.
Bandage the entry site and encourage
Cardiopulmonary arrest.
Procedure
the child to remain prone for 34 hours
Drape the area. to minimise CSF leak.
Grasp the needle with the thumbs and Send CSF for white blood cell (WBC)
index fingers. count and differential, glucose, protein,
Tips
 Position the needle with the bevel up Gram stain, other pathogen studies (e.g. Positioning and restraining the infant or
(if in the lateral position). viral, AFB or fungal cultures, antigen child properly greatly facilitates the
Aim the needle into the interspace at studies, VDRL). If the CSF is bloody, ask procedure.
90 degrees, just above the L4 spinous for a cell count on both tubes #1 and #4. If no CSF appears in the hub after initial
process and advance slowly until there Clearing of blood suggests a traumatic penetration, rotate the needle 90
is resistance at the paraspinous tap, whereas no clearing suggests degrees and withdraw the stylet.
ligaments. subarachnoid haemorrhage. If there is no success with multiple
Advance the needle until there is a pop Interpret CSF findings (Table 23.18.1). attempts, try a paramedian approach a
as the needle enters through the dura, few millimetres off the midline.
into the subarachnoid space. Use latex agglutination or another
Remove the stylet. bacterial antigen test for partially treated
If CSF does not appear at the hub,
Complications or Gram-stain negative CSF.
replace the stylet and advance further. Local If the child appears ill, administer empiric
 If the child is over 2 years old, attach Puncture-site pain. antibiotics after blood cultures are
a three-way stopcock and manometer Backache. obtained.
to the hub, and measure opening Headache (uncommon <10 years of age). If the LP is traumatic and CSF is bloody,
pressure. Normal opening pressure Vomiting. calculate a WBC:red blood cell (RBC)

Table 23.18.1 Interpretation of CSF

Condition WBC mm3 Protein Glucose Gram stain Opening pressure

(mg dL1) (mg dL1) (cmH2O)

Normal <6 <45 6070% Negative 520

peripheral

Bacterial meningitis 10010 000 (PMN 50100 <50% Positive Elevated

predominance) peripheral (80% of
cases)

Partially treated 10100 (PMNs or 50100 <50% Negative or Elevated

lymphs) peripheral positive

Viral meningitis 20100 (early PMNs, <100 Normal Negative Normal or slightly
late lymphs) elevated

TB meningitis 20500 (usually 50200 <40% Negative Elevated

lymphs)

Crypto meningitis Few100s 100s <40% Negative Elevated

Brain abscess 10100s 50100s Normal Negative Elevated

PMN, polymorphonuclear.

516
23.18 LUMBAR PUNCTURE
23
ratio <0.01 (1:100) and an O:P ratio

COMMON PROCEDURES
ratio and Observed: Predicted (O:P) Barnett ED, Bauchner H, Teele DW, Klein JO. Serious bacterial
infections in febrile infants and children selected for lumbar
CSF WBC to help predict probability <0.01 makes bacterial infection puncture. Paediatr Infect Dis J 1994;13(11):9503.
of bacterial infection. The WBC:RBC unlikely. Dieckmann RA, Fiser D, Selbst S. Illustrated textbook of
paediatric emergency and critical care procedures. St Louis,
ratio is calculated as a simple ratio MO: Mosby; 1997. p. 5337.
on CSF fluid. The Predicted CSF WBC Grant T. Paediatric meningitis. Ann Emerg Med 1994;24

is calculated using the formula Further reading (1):118.

Ward E, Gushurst CA. Uses and technique of pediatric lumbar
Al-Eissa YA. Lumbar puncture in the clinical evaluation of
Predicted CSF WBC CSF RBC
children with seizures associated with fever. Paediatr Emerg
puncture. Am J Dis Child 1992;146(10):11605.

blood WBC/blood RBC. A WBC:RBC Care 1995;11(6):34750.

517
SECTION

24 ORTHOPAEDICS AND
RHEUMATOLOGY
Section editor Gary Browne

24.1 Orthopaedics and rheumatology 518 24.4 Risk management in acute paediatric
orthopaedics 553
24.2 Fractures and dislocations 532
24.3 Spinal injury 544

24.1 Orthopaedics and rheumatology

Robyn Brady Navid Adib

as well as the techniques used in assess-

ESSENTIALS ment. These differences are outlined in
Table 24.1.1.
1 Painful dysfunction of limb, posture, or gait may be caused by trauma, infection, or
less commonly, inflammatory or neoplastic developments.
The limping child
2 Hip, knee, abdominal, pelvic, and spinal pathology must be considered with each
The wide spectrum of causes of limp in chil-
presentation. In particular, isolated knee pain may be the only presenting feature of
dren is illustrated in Table 24.1.2. In terms of
serious hip pathology.
frequency, common causes in different age
3 Careful attention to history and close assessment of posture and response to groups are outlined in Table 24.1.3.
palpation are required to delineate focal pathology. Diagnoses that require specific treatment
to avoid further damage or danger are pri-
marily displaced or unstable fractures
(including slipped upper femoral epiphysis),
abusive injury (as a presenting feature or
incidental finding), bone or joint infection,
and neoplastic processes, principally bone
tumours and leukaemia.
Introduction
THE CHILD WITH ACUTE Assessment
The child who has pathology of the bones
MUSCULOSKELETAL The most important characteristics of
and joints may present in a variety of ways.
Acute paediatric musculoskeletal conditions
PAIN OR DYSFUNCTION a child with an acute limp or painful limb
dysfunction are the following:
range from the many infective, inflamma-
tory and other causes of limp and limb age;
General approach trauma history;
pains, to the isolated acute limb fracture
as a result of moderate trauma, which is The spectrum of musculoskeletal pathology well-being;
among the top ten paediatric emergency occurring in the paediatric population, pain magnitude;
department (ED) presentations in Australia.1 with the exception of some fractures and pain localisation.
An awareness of the range of possible adolescent injury, is very different from that
conditions and their clinical appearance, seen in the adult population. Age
and skilled physical examination and Infant and child development have The neonate or infant with focal pain or
clinical reasoning, are essential for optimal extensive influence on the musculoskeletal dysfunction should not be discharged
outcomes. pathology seen and its manifestations, without a specific diagnosis, (most of

518
24.1 ORTHOPAEDICS AND RHEUMATOLOGY
24

ORTHOPAEDICS AND RHEUMATOLOGY

Table 24.1.1 How children differ: impact of development on musculoskeletal pathology eating and activity level, which are
usually abnormal in sepsis;
Body proportions
minor illnesses are also common and can
Large, heavy head: higher fulcrum for spinal disruption
Low centre of gravity co-exist with injury.
Relatively short legs

Physiology of developing bone Pain magnitude

Zone of calcification: weakest point of muscle/tendon/ligamentous/bony continuum Septic arthritis is painful to the slightest
Increased plasticity means increased susceptibility to plastic deformation, e.g. torus, bowing movement.
fractures
Physeal vulnerability predisposes to specific pathological responses to subacute or chronic Subacute arthritides and Perthes
microtrauma, e.g. avascular necrosis of ossification centres, slipped upper femoral epiphysis disease present fluctuating disability
Physeal damage from fracture or ischaemia produces long-term complications
High blood flow to physeal zones increases risk of vascular dissemination of bacterial disease, and pain.
e.g. osteomyelitis
Disruption of night sleep needs further
Joints and ligaments assessment.
Increased flexibility means less brittleness and fewer fractures in response to arcuate deformation,
e.g. carpal, tarsal, rib, vertebral fractures less common
Ligaments generally stronger than bones: sprains uncommon and complete ligamentous tears rare Pain localisation
This is the foundation of acute orthopaedic
Exposure to mechanisms
diagnosis and efficient investigation use.
Infant-abuse mechanisms especially shearing/shaking forces
Pre-school children fall on average four times a day (same-level falls) A suggested sequence of examination for
Highly active pre- and school-age children have frequent, relatively low-force impactions from falls young children with musculoskeletal pain
(monkeybars, trees)
Pedestrian vs. car causes classical femoral or tibial/fibula injuries from fender, plus head injury from is shown in Table 24.1.4.
secondary impact Other useful features in assessment include:
High-force MVA/MBA/high energy sports injuries increase from adolescence

Immunology History
Increased incidence of bacterial and viral infection
More rapid dissemination of, and destruction from, bacterial infection Details of onset.
Whether symptoms are constant or
Psychology intermittent.
Fear and pain make young children difficult to examine and increase importance of observation and
gentle handling Exacerbations.
Immature intellectual development means poor verbalisation of symptoms, incomplete self-other Daily pattern, particularly the presence or
differentiation, and other blocks to symptom communication
Medical management of traumatic experiences, such as injuries, may influence future psychological
absence of night pain.
responses to trauma History of previous limp or similar
Altered body perception increases susceptibility of adolescents (particularly females) to unconsciously
exaggerated dysfunction in response to minor injuries
pains.
Physical activities, such as elite sport.
Healing Bullying or school/home problems.
Rapid healing of most fractures (e.g. femoral fractures: infants 3 weeks!)
Enormous remodelling potential of deformation within arc of use
Great ability to compensate, physically and psychologically, for loss of function Observation and examination
Minimal stiffness after immobilisation
Gait if ambulant.
MBA, motorbike accident; MVA, motor vehicle accident. Posture and symmetry.
Activity level.
Well-being fever, heart rate.
which, with the specific exception of pulled there is always a possibility of abusive Bones, joints, soft tissues.
elbow, warrant admission), and should injury and inadequate or erroneous Skin integrity.
generally be assessed by someone experi- history. Abdomen and spine.
enced in this area. Adolescents with a limp Lymphadenopathy and lymphadenitis.
However, a clear history of completely
or knee or hip pain must have slipped Focal pathology, e.g. warts, bites, and
normal function prior to a specific event that
upper femoral epiphysis (SUFE) specifically callosities.
precipitated crying and subsequent pain and
ruled out.
dysfunction should prompt a search for a Lower limb joint examination should occur
fracture. with the patient lying supine on the couch.
Trauma history
Take note of the resting position of the joints
The history is less sensitive or specific than
and any asymmetry, redness, swelling,
in adult medicine because: Well-being
or wasting. Joints should be compared
The following key points should be sought:
children are often poor historians; in symmetrical posture as position influences
minor injuries are very frequent; history of fever/malaise; external appearance. Isolate joints (e.g. knee)

519
24.1 ORTHOPAEDICS AND RHEUMATOLOGY

child lifting the buttock off the couch.

Table 24.1.2 Causes of acute limp in children
In the normal hip this adduction should
Trauma allow the ipsilateral knee to be positioned
Fractures accidental and non-accidental over the opposite leg whereas an inability
Infection (point focus) to adduct past the midline is common
Septic arthritis in the irritable hip or other causes of hip
Osteomyelitis joint pathology.
Inguinal lymphadenitis
Muscle abscess, e.g. psoas At the conclusion of the history and
examination the emergency physician
Post-infective
should have established the overall well-
Rheumatic fever/post-streptococcal arthritis
Post-infectious arthritis, e.g. Salmonella, Shigella, or Campylobacter enteritis being of the child, the site and severity
Serum sickness of musculoskeletal pain, practical precipi-
Post-immunisation inflammation
tants, and the likelihood of traumatic, infec-
Inflammatory tive, or other processes. In patients with
Transient synovitis minimal physical findings, or findings out
Vasculitis, e.g. HenochSchonlein purpura or Kawasaki disease
Inflammatory arthritis in lower limbs or axial skeleton, e.g. oligoarthritis of keeping with other aspects of history
Enthesitis in pelvis or lower limbs, e.g. enthesitis related arthritis and examination, the environmental and
Associated with, e.g. systemic lupus erythematosus, dermatomyositis, or inflammatory
bowel disease psychological context of the limp should
be explored further. Clues to possible
Primary bone disorders
neoplastic illness are discussed later in this
Slipped upper femoral epiphysis (SUFE)
Avascular necrosis, e.g. Perthes disease (hip), Freibergs disease (metatarsal heads) chapter.
Osteochondroses, e.g. OsgoodSchlatter disease (patellar tendon insertion), Severs disease The child whose examination findings
(Achilles tendon insertion)
Unicameral bone cyst/aneurysmal bone cyst/fibrocystic disease/eosinophilic granuloma suggest an isolated irritable hip is likely to
Blount disease (asymmetrical tibial physis closure) have one of the pathologies outlined in
Tarsal coalitions
Table 24.1.5. A suggested pathway for
Neoplastic investigation is shown in the algorithm in
Leukaemia Fig. 24.1.1. This algorithm incorporates
Neuroblastoma
Bony tumours, e.g. Ewings sarcoma, osteosarcoma the findings of a number of studies of the
Non-malignant tumours, e.g. osteoid osteoma, enchondroma prognostic significance of various features
Haematological of the limping child with respect to septic
Haemarthrosis, e.g. Haemophilia A arthritis, which are further discussed below.
Sickle cell disease arthropathy

Physical Investigations
Splinter/foreign body A number of recent research findings have
Compensatory, e.g. footwear allowed for more selective and meaningful
Soft-tissue and overuse injury
Joint hypermobility syndrome investigation of the limping child.
Plantar warts/calcaneal spurs
Bites and envenomation
Inflammatory markers
Psychological and idiopathic pain syndromes Traditional blood investigations for inflam-
Pain amplification syndromes, conversion disorders matory markers include full blood count
Complex regional pain syndrome (CRPS, previously known as reflex sympathetic
dystrophy) (FBC), erythrocyte sedimentation rate (ESR)
and C-reactive protein (CRP).
Abdominal
FBC is useful primarily to flag bone marrow
Appendix
Infective and inflammatory bowel disease dysfunction. Each cell line (haemoglobin,
white cells and platelets) should be indivi-
Spine
dually assessed. The sensitivity of a raised
Scoliosis
Discitis white cell count (WCC) (>12
109 L1) for
Transverse myelitis the identification of septic arthritis is variable
Spondylolisthesis
Scheurmans disease (2075%).3,4 The absence of an elevated
GuillainBarre syndrome white cell response should never be used
to rule out septic arthritis. ESR has shown
when assessing range of motion so that with buttocks flat on the bed, flex the higher sensitivity (9095%) in identifying
incidental movement of another joint hip (first the unaffected and then the the subgroup with septic arthritis; however,
(e.g. hip) does not cause misleading results. affected) to 90 degrees while supporting this may miss early infection.3 CRP has shown
When assessing hip range of motion, the lower leg. Then gently attempt to fold superior sensitivity (up to 80%)4 and speci-
the flexion adduction test2 may be helpful: the knee over the contralateral leg without ficity in the early identification of invasive

520
24.1 ORTHOPAEDICS AND RHEUMATOLOGY
24

ORTHOPAEDICS AND RHEUMATOLOGY

for these pathologies. Disadvantages of ultra-
Table 24.1.3 Common causes of acute limp/single-limb dysfunction in children of various
ages presenting to the ED with no specific history of trauma sound include variations in quality from differ-
ent operators, and difficulty in after-hours
Infants Young children Primary school - Adolescents
age children
access. An ultrasound cannot distinguish
between reactive and infective effusions; how-
Occult fracturea Occult fractureb Transient synovitis Septic arthritis/
osteomyelitis ever, the presence of an effusion confirms
organic pathology and its absence in a clini-
Septic arthritis/ Transient synovitis Septic arthritis/ SUFE
osteomyelitis Inflammatory arthritis osteomyelitis Inflammatory arthritis cally abnormal presentation should prompt
Inflammatory arthritis Inflammatory arthritis a search for an extrasynovial focus. There is
Septic arthritis/ Perthes disease Osteochondroses controversy over the place of ultrasound-
osteomyelitis Psychogenic pain Tumour guided hip aspiration in the evaluation of
Perthes disease
the irritable hip with an effusion.8 The major-
a
Includes pulled elbow, accidental and non-accidental injury. ity of Australian paediatric orthapaedic
b
Includes pulled elbow, toddler fracture. units favour orthopaedic evaluation of
irritable hips with selective aspiration and
Table 24.1.4 Tips for assessing young children arthrotomy/washout under general anaes-
The foundation of acute musculoskeletal diagnosis is precise localisation of pain and dysfunction
thesia. However, ultrasound-guided diagnos-
The foundation of a productive paediatric examination is a relaxed, non-fearful child tic hip aspiration has a role in some units.911
Personality and parental factors aside, a gentle, slow-moving, highly observant examiner whom the
child senses has the trust of the parent, will be most successful in gaining meaningful information
Bone scan
1. Be friendly and establish rapport with the parent
2. Ask parents for clues about site of pain, e.g. is it worse during nappy changes or being picked up
Isotope bone scans with three-phase
under arm, crying associated with going over bumps in car ride, etc. If the child is old enough, ask technetium-99m MDP are sensitive early,
them to tell or show you exactly where it hurts
3. Where possible, observe child at free play before attempting palpation
and well tolerated by children, but lack spec-
4. Visually scan child for alterations of posture, symmetry, or function ificity. Confusion may arise particularly in
5. Record any other clues, such as temperature, dysmorphic features, bruising, psychological state
6. Examine the infant or child in parents arms (under 6 months and over 5 years they may often be
relation to physeal sites, where uptake is
assessed on the couch without concern) already above base-line. Hot scans indicate
7. Introduce a washable or disposable toy, such as light, name-badge, or funny face
8. Make your first touch very gentle and at a site distant from the target limb! Keep some touch
increased osteoblastic uptake and generally
continuous so the rhythm is reassuring. Verbal soothing, such as a hum, may settle anxiety relate to a response to infection or injury.
9. Palpate the limb in question from one end to the other, watching the childs face continuously
10. The first sign of discomfort may be an eyebrow flicker (i.e. a pre-frown) or a flinch
A cold scan suggests infarction, which in
11. If discomfort is sensed, move touch to another area immediately and slowly move back in even more severe cases may be a consequence of oste-
gently for confirmation and further information
12. Put all joints through their full range of movement unless discomfort is noted
omyelitis. Bone scan is of particular value in
13. Examine the spine, groin, and abdomen of all lower limb or gait-disturbed children children in whom multifocal disease is sus-
14. Areas of focal tenderness or limited range of motion can be further assessed by radiology or
ultrasound
pected e.g. neonatal osteomyelitis or chronic
recurrent multifocal osteomyelitis (CRMO).6

MRI
bacterial infection in general and of septic radiology should not be encouraged. An MRI is excellent for detailed delineation of
arthritis in particular. CRP rises within effusion is better diagnosed by ultrasound, soft-tissue and bony pathology, particularly
24 hours of acute illness, and also falls rapidly and deep soft tissue infection such as osteo- when surgery is planned.12 It is the investiga-
and can be used to monitor effectiveness of myelitis, by magnetic resonance imaging tive modality of choice to delineate the site of
treatment.5 (MRI). X-rays are required in trauma with possible bacterial infection in a child with
focal tenderness or non-weight-bearing, to localised musculoskeletal pain and a septic
rule out SUFE (see below) in the adolescent picture in whom septic arthritis has been
Radiology child with hip pain/dysfunction with or with- ruled out by clinical assessment, ultrasound
X-ray out a trauma history, and in cases of persis- or arthrocentesis.13 Limitations include cost,
X-rays are usually non-contributory in chil- tent unexplained pain or limb dysfunction. access limitations, and the need to remain
dren under 10 years of age with acute still for a longer time period, necessitating
onset limp (<1 week) without a specific general anaesthetic in most small children.
trauma history. Radiological findings in Ultrasound
osteomyelitis are usually not present for Ultrasound is a sensitive, non-invasive assess- Clinical decision making in a child
up to 10 days from the onset of illness, at ment tool for evaluation of the irritable hip. It with a limp
which time there may be periosteal eleva- will detect even small hip effusions, may Relative weightings of various symptoms and
tion outlined by new bone formation and/ show a subperiosteal pus collection in some signs in the various acute paediatric hip
or lucent areas.6 Due to the higher burden cases of osteomyelitis, and may be diagnostic pathologies are expressed in Table 24.1.5
of pelvic or gonadal irradiation, non- for Perthes disease and SUFE,7 although and Fig. 24.1.1. However, spinal, abdominal,
selective or routine pelvic/lower limb it is not usually the first-line investigation and pelvic pathology can present as a

521
24.1 ORTHOPAEDICS AND RHEUMATOLOGY

Table 24.1.5 Comparative features of paediatric hip-joint pathology

Diagnosis Transient Septic Femoral neck Perthes SUFE Inflammatory Bone

synovitis arthritis osteomyelitis arthritis tumour

Age 36 Any Any 312 915 Any Any

Joint Hip Hip, knee Hip Hip Hip Any Not joint

Pain (03) 1 3 1 01 early 13 02 1

Illness (03) 0 13 13 0 0 02 Variable

ROM >80% <50% <80% 6080% Flexes into external Usually some Normal unless
rotation movement spasm
possible

Inflammatory Normal Elevated Elevated Normal Normal Variable Variable

markers

Ultrasound Effusion Effusion ?Reactive effusion, Diagnostic Diagnostic ?Effusion n/a

?subperiosteal changes changes
collection

Plain X-ray ?HTDD up ?HTDD up Bony changes Diagnostic Diagnostic May be normal May be
delayed changes changes, e.g. diagnostic
eventually widened physis in
preslip;
Trethowans sign

ROM, range of movement; HTDD, head-teardrop distance.

limp, and must be considered prior to this

narrowed focus. The infant or toddler, because
of their increased risk for invasive bacterial
Exclusion: illness, also represents a special circumstance
Infant/toddler
Duration > 3 weeks and the ill child under 2 with an abnormal
Atypical history: multifocal pain or joint dysfunction acute musculoskeletal assessment is best
admitted for combined paediatric and ortho-
Recent significant trauma OR
pain greater than one week OR paedic assessment and investigation.
adolescent?
The child over 2 with acute atraumatic
Yes
localised knee or hip pain and an abnormal
Frog-leg X-ray pelvis/hip with gonadal protection hip examination is most likely to have an
No irritable hip (transient synovitis), and the
Normal Abnormal
main issue is to exclude septic arthritis.
Sepsis risk: any of non-weight- Orthopaedic evaluation An ultrasound assessment should confirm
bearing OR febrile > 38.5 OR the suspicion of a hip effusion as suggested
ROM hip < 90% normal
by examination, but does not differen-
Yes Abnormal tiate between transudate and exudate.
Table 24.1.6 lists conditions at higher risk
Sepsis screen: FBC/CRP/ESR
of septic arthritis which must be flagged;
Abnormal however, the majority of infections occur in
No children without underlying pathology. Vari-
Normal Hip ultrasound
ous attempts have been made to create a
Normal
valid decision rule with a high sensitivity
and specificity for bacterial infection,4,1416
Senior ED clinical assessment however none have demonstrated sustained
Low risk High risk power prospectively. The four most impor-
tant variables to give a predicted probability
Consider X-ray or
Discharge home
ultrasound if has not been of septic arthritis in a given child with
Information sheet;
ibuprofen, paracetamol; done previously. an acutely irritable hip are shown in
review 2 days or worse Refer orthopaedic team
Consider bone scan or MRI Table 24.1.7. Degree of pain and range of
motion of the joint are auxiliary clinically
Fig. 24.1.1 Algorithm for management of children >2 years presenting with acute irritable hip. important variables in the differentiation.

522
24.1 ORTHOPAEDICS AND RHEUMATOLOGY
24

ORTHOPAEDICS AND RHEUMATOLOGY

concern about occult Perthes being an and children under 3 are at particular risk of
Table 24.1.6 Risk factors for septic
arthritis underlying diagnosis, especially in those with septic arthritis, comprising one-third and
delayed bone age at the first imaging.20 one-half, respectively, of a large paediatric
Relative immune deficit
All children discharged with this presump- series.22 As well as focal clinical inflamma-
Neonates
Malnourished tive diagnosis should have orthopaedic tion, children may present with occult infec-
HIV infection follow up if pain or limp persists or recurs. tion, pseudo-paralysis or generalised sepsis.
Immunosuppressive therapy
Corticosteroid therapy Comorbidity or deficient host defences, such
as those shown in Table 24.1.6, predispose
Injury mechanism ESSENTIALS to infection that may be more rapidly pro-
Penetrating trauma
1 Diagnosis of septic arthritis is gressive or occur in the older child.
Joint disease Joints of the lower limb, especially hip and
suspected on clinical features, and
Chronic arthritis knee, account for two-thirds of the infections
Sickle cell disease confirmed by blood and synovial cell
count and fluid culture. Isotope scans in children.
and MRI have an important role in In osteomyelitis, bacteria enter the vascu-
localising osteomyelitis. lar metaphyseal bone initially, then typically
extend to the sub-periosteal space forming
Table 24.1.7 Features suggestive of
deep bacterial infection in a child with an
2 Delayed or inadequate treatment an abscess. New bone deposition results,
acutely irritable hip of septic arthritis can lead to with later necrosis of cortical bone. Classi-
Non-weight-bearing
irreversible joint damage and/or cally, bone fragments or sequestra are
Febrile >38.5 C septicaemia. formed over time, which harbour bacteria.
WCC > 12
109 L1
ESR > 40 mm hr1 3 Staphylococcus aureus and Successful treatment must combine eradica-
CRP > 20 mg L1 tion of the bacteria and complete removal of
Streptococcus species are the most
frequent pathogens, via any necrotic infected bone. However, in the
haematogenous spread or direct developed world, earlier diagnosis and
extension from infected joint to bone. aggressive antibiotic therapy have limited
Specific syndromes Community-acquired methicillin the degree of bone destruction present.
resistant Staph. aureus (CAMRSA) is The disorder of CRMO has been recognised
Transient synovitis in infants and children. This is an inflamma-
Transient synovitis is a self-limited inflamma- increasingly common worldwide and
mandates alternative antibiotic tory process of unknown origin, which
tory disorder of uncertain aetiology, occur- demonstrates culture-negative bony inflam-
ring particularly in boys (70%) in the age treatment.
mation with histological evidence of necrosis
group 310 years. The onset of lower limb 4 Successful treatment requires and chronic inflammatory cell infiltrates.
pain is generally gradual and initially may parenteral antibiotics and complete Biopsy and culture are mandatory to diag-
be localised to hip, knee, groin or thigh. drainage of pus from the joint, or nose the disorder, but antibiotics may be
The child may be mildly unwell or have had surgical clearance of necrotic bone, discontinued if pathology is consistent with
a recent non-specific upper respiratory tract with long-term follow up. CRMO. In mild cases, treatment of symptoms
illness, although this is not uniformly present. may be possible with non-steroidal anti-
Range of movement at the hip joint is usually inflammatory drugs (NSAIDs); however, in
mildly diminished, with discomfort at the more severe cases systemic steroids or treat-
end-points of the range. A hip effusion is pres- Septic arthritis
ment with intravenous bisphosphonates, and
ent. Transient synovitis is a diagnosis of and osteomyelitis long-term follow up will be required.2327
exclusion, the major differential being septic Introduction
arthritis. Children with localised hip pain Septic arthritis is infection of the synovial lining Presentation
should be managed according to the algo- and fluid of a joint. Bacteria are the usual patho- History
rithm in Fig. 24.1.1, and non-weight-bearing gens through haematogenous spread from Cardinal features of septic arthritis are
children with a hip effusion who do not have other, sometimes occult, sites. Direct spread recent onset of a painful, red or swollen
needle aspiration to rule out septic arthritis from adjacent bone infection may also occur, joint or limb, limp or refusal to bear weight.
should be admitted under the close observa- particularly where the metaphysis is intracap- Pseudoparalysis or refusal to move a limb
tion of the orthopaedic team. Weight-bearing sular, as in the proximal femur. Phagocytic may occur in neonates and young infants.
children without unusual or high risk features and neutrophil responses to the bacteria result Infants may present with non-specific symp-
can be managed with parental instruction, in proteolytic enzyme release and cytokine toms such as poor feeding, vomiting, leth-
ibuprofen, and ED follow up in 2 days.17,18 production, with synovial abscess formation argy or fever. Infrequently, a vertebral or
and cartilage necrosis.21 Pus under pressure pelvic infection will be the cause of an
Prognosis may also reduce epiphyseal blood flow. abnormal gait or abdominal pain. Relevant
Taylor et al showed a 15% recurrence rate in Most infections in children are community past history includes trauma, past history
transient synovitis,19 and there is some acquired and occur in normal joints. Infants of recurrent staphylococcal infection such

523
24.1 ORTHOPAEDICS AND RHEUMATOLOGY

as boils, and immunisation status in of paediatric septic- and osteomyelitis

Table 24.1.8 Organisms cultured in
respect to Haemophilus influenzae and paediatric invasive bone and joint worldwide.4,2931 Increasing availability of
Pneumococcus. infection in Australia polymerase chain reaction (PCR) may
Most common (80% of most isolates)
increase specific bacteriological identi-
Examination Staphylococcus aureus fication.32 Despite increasing CAMRSA
Typical findings in septic arthritis include a Streptococcus species and a high negative culture rate, the
warm tender joint with marked limitation Other organisms
Australian experience has also been of a
of passive and active movement due to pain. Pneumococcus low rate in treatment failure, presum-
Children with a painful hip will usually main- Kingella kingae ably due to early case identification
Fusobacterium
tain the joint in slight (20 degrees) flexion Meningococcus and susceptible organisms. However, the
and external rotation. In most distal joints, Pseudomonas increased prevalence of CAMRSA in the
Salmonella
an effusion is usually clinically evident. In US has been associated with an increase
general, fever is low grade and most patients in severity of osteomyelitis.33,34 Tuber-
will not appear toxic or unwell. Osteomyeli- culosis is still a cause of chronic bone and
tis may be suggested by an area of maximal as Kingella.30 Initial Gram stain results and spine infection in South-East Asia, Papua
tenderness next to a joint, with a greater clinical history will guide empiric antibiotic New Guinea, and the Torres Strait Islands
range of movement of that joint than would therapy, with definitive therapy based on and should be considered in atypical
be expected with septic arthritis. Deep or final cultures. cases.35,36
partially treated infections may be clinically Organisms implicated in two recent
subtle, with minimal specific examination series involving Australian children4,31 are
findings. Careful examination of the skin shown in Table 24.1.8. An algorithm for ini- Management
may reveal areas of infection or trauma as tial intravenous therapy of paediatric septic Joint drainage and empiric parenteral anti-
an entry point for haematogenous seeding. arthritis and osteomyelitis is shown in biotic therapy are the mainstays of treat-
Fig. 24.1.2. ment for septic arthritis and should take
Differential diagnosis Negative cultures of blood, synovium, or place without delay. Suggested antibiotic
The differential diagnosis of single focus infected bone occur in 4080% of cases regimes for bone and joint infection are
septic arthritis and osteomyelitis is discussed
in detail in the section on the limping child.
The child presenting with polyarthralgia/
Clinical picture SA/OM
polyarthritis is considered in the section on Blood cultures taken
acute arthritides. Synovial/other samples taken/imminent

Microbiology CAMRSA risk factors

Synovial fluid examination and Pacific Islander
Clindamycin IV
Aboriginal or Torres Strait Islander
culture Aspiration of affected joints Family boils
should be performed promptly to confirm
the diagnosis and obtain culture specimens.
Most young children will require general Allergy to penicillin (not anaphylaxis) Cefalothin IV
anaesthesia or conscious sedation for
these procedures, and in the majority Anaphylaxis with penicillin Vancomycin IV
of units in Australia, arthrotomy with wash-
out performed by the orthopaedic team is
the procedure of choice in probable At risk of Haemophilus influenzae
Cefotaxime +
(unimmunised and under 5)
septic arthritis of the hip. The overriding flucloxacillin IV
principles are early accurate diagnosis, and
minimal delay or exposure of the joint to Extremely unwell Vancomycin +
the chondrolytic enzymes of bacterial joint flucloxacillin IV
infection.28
Sneaker (puncture wound) osteomyelitis Ceftazidime +
A cell count of greater than 50 000 per flucloxacillin
microlitre of synovial fluid aspirate suggests
a bacterial cause, although positive cultures All others Flucloxacillin
can occur with lower counts, and higher monotherapy
counts may occasionally occur in inflamma- Monitor patient and indices for clinical improvement.
tory conditions.29 Synovial fluid should also Refine choices when cultures and sensitivities known.
Clindamycin-resistant CAMRSA may respond to cotrimoxazole.
be inoculated directly into culture medium
to avoid loss of fastidious organisms such Fig. 24.1.2 Antibiotic therapy for septic arthritis (SA)/osteomyelitis (OM) in Australia.

524
24.1 ORTHOPAEDICS AND RHEUMATOLOGY
24

ORTHOPAEDICS AND RHEUMATOLOGY

shown in Fig. 24.1.2, and should be debridement, wound cleansing and anti- and deterioration of the cartilaginous
further guided by Gram stain and culture/ biotics in the management of open bone mechanical properties. Cartilaginous ischae-
sensitivity results. trauma. mia is thought to be the active mechanism.
Community-acquired methicillin resis- It is more prevalent in malnourished chil-
tance is becoming an increasing problem dren,41 those with delayed bone age, and
world-wide. Specific populations at high risk Controversies those exposed to passive smoking.20,42,43
of CAMRSA include Aboriginal and Torres The role of ultrasound-guided hip The end result may be subchondral fractur-
Strait Islanders in Western and Northern aspiration: most orthopaedic surgeons ing, anterolateral deformation and joint
Australia, and Pacific Islanders in Eastern prefer open arthrotomy on selected incongruence, although the majority of
Australia and parts of New Zealand.37 patients. children have spontaneous gradual remis-
Children with a personal or family history sion of their disease process. Four stages
of recurrent treatment for boils are also Extent and timing of surgery are recognised:44
at risk.38 in suspected osteomyelitis.
Initial ischaemia causes cartilage
Neonatal septic arthritis requires special  Duration and route of antimicrobial hypertrophy and synovitis; X-ray smaller
consideration in view of the destructive therapy for bone and joint infections. femoral head, increased joint space.
potential and broader microbiological differ-
Fragmentation X-ray shows
ential.39 These children should be reviewed
fragmented epiphysis and subchondral
by a team of orthopaedic, neonatal, and
radiolucency (Caffeys crescent line).
infectious-disease specialists. Perthes disease
Because of earlier diagnosis and other
 Reossification altered shape of
femoral head with increased radiopacity.
aspects of modern care, in most cases
beyond the neonatal period combined in-
ESSENTIALS Healing resolution or persistence of
deformity.
patient/outpatient treatment can occur 1 Perthes disease is a chronic
with length of treatment guided by clinical disorder of the femoral head in ED presentations
and inflammatory markers.4 children, causing limp and usually When hip pain is present in Perthes disease
low-grade pain. While most it is usually mild, chronic and dull, increasing
Prognosis children outgrow their disease, a with physical activity, often with a history of
This depends upon the organism, patient proportion have permanent pain for weeks to months. There are no sys-
comorbidity, age of patient and the adequacy structural changes requiring temic symptoms. Because of the gradual
and rapidity of treatment. Remarkable remo- orthopaedic correction. onset of Perthes disease, acute presenta-
delling of bony deformity can occur in the tions to the ED usually represent either an
young child providing treatment has been
2 Clinical findings are of
early (stage 1) Perthes and fall within the
decreased range of hip motion,
adequate. Out of a 30-year series of 332 irritable hip differential, as discussed previ-
particularly internal rotation
infants and children with osteomyelitis, with ously, or a flare-up in a child with known
and adduction, with low-grade
documented follow up of 170 cases, compli- Perthes disease in whom coincidental
discomfort.
cations were described in 19%.22 These comorbidity must be excluded. Remember
related largely to joint complications, but 3 X-ray changes of established Perthes can present as a painless limp.
some 8% demonstrated epiphyseal damage. disease include joint effusion, loss of
However, more recent series showed many femoral head height, fragmentation Examination
fewer complication rates.40 Patients at high- of epiphysis. Children with Perthes disease have limited
est risk of complication include neonates, sep- range of hip movement, particularly in adduc-
tic arthritis in which diagnosis has been
4 Early X-ray changes may be subtle tion and internal rotation, from synovial
and children with persisting thickening and adductor muscle spasm. Those
delayed, complicated osteomyelitis (involu-
undiagnosed limp should have MRI with long-standing disease may have muscle
crae, sequestrum or sinus formation) or epiph-
examination of their hip and, if atrophy, leg-length discrepancy, and a positive
yseal involvement, which may cause
necessary, orthopaedic follow up. Trendelenburg test on the affected side.
subsequent limb length discrepancy or defor-
mity. All cases should have close orthopaedic
follow up. Differential diagnosis
Pathophysiology In the early stages of disease, X-ray changes
Prevention LeggCalvePerthes disease is a disorder will mimic those of transient synovitis, and
Primary prevention involves minimising of unknown aetiology principally affecting children should be managed according to
skin or muscular trauma or sepsis (e.g. boils, boys (M:F 5:1), commonly in the 48-year the irritable hip outline suggested earlier.
infected scabies), since damaged tissue age group. It is bilateral in approximately Children with more minor hip discomfort
predisposes to haematogenous bacterial 10%. The pathophysiology affects arti- and non-specific X-ray or ultrasound evalua-
spread of skin micro-organisms. Secondary cular cartilage, where synovitis results in tions should be referred for MRI examina-
prevention can be provided by thorough oedema, hypermetabolism, hypertrophy, tion of hip for detection of early stages of

525
24.1 ORTHOPAEDICS AND RHEUMATOLOGY

avascular necrosis4548 and orthopaedic

evaluation if symptoms persist longer than ESSENTIALS
3 weeks.
1 All adolescents with undiagnosed
Investigation and disposition acute hip or knee pain should have hip
The plain hip X-ray is diagnostic in estab- X-rays or ultrasound performed by
lished Perthes disease, although findings competent operators. A history
Normal AP
may be subtle in early disease. Extensive of trauma is not required.
head involvement, loss of the femoral head 2 Non weight-bearing adolescents
lateral pillars, subluxation of head beyond with acute hip pain must not undergo
the acetabular margin, and late age at pre- passive hip manipulation (even for
sentation are all poor prognostic indicators. X-ray) until diagnosis is established.
MRI examination of the hip joint may be
necessary to provide early diagnosis and 3 Delay in stabilisation of SUFE Trethowans sign

prognosis (references from above). Ortho- increases the risks of avascular

paedic management goals are restoration necrosis, chondrolysis, deformity,
of range of hip movement and containment and long-term poor hip outcomes.
of the hip within the acetabulum. These 4 ED doctors must be able to
goals are sought through a variety of meth- recognise early SUFE (see
ods including traction, surgery, orthotics and Figure 24.1.3) and must refer all
physiotherapy. In general, the younger the patients with SUFE to orthopaedics
child the better the outcome; girls do worse immediately. Frog leg lateral
than boys of the same age as they are skel-
Fig. 24.1.3 Slipped upper femoral epiphysis
etally more mature. Some three-quarters (SUFE). In the normal pelvic AP, (top) a
of patients with Perthes disease are pain- Klein line (a continuation of the superior
free and active in 1020-year follow-up Clinical presentation border of the femoral neck) should INTERSECT
studies.44 The classic child with SUFE will be an obese a portion of the femoral head. Trethowans
sign, (middle) in which the Klein line fails to
adolescent with pain either in the groin or
intersect the head in the AP view, is
referred to knee (femoral) or medial thigh abnormal and represents Grade 1 SUFE. Note
Slipped upper femoral (obturator nerve). They may have some the frog-leg view (shown above as in a
epiphysis (SUFE) leg-length discrepancy and hold the leg in normal hip) is a better view for diagnosing
external rotation. Range of motion depends SUFE in the ambulant child with hip pain,
Epidemiology and since the posterior slip is usually more clearly
on degree of slip and chronicity. A classical
pathophysiology shown. However the hip must not be
feature of the chronic slip is that passive manipulated into frog-leg position in an
SUFE is more common in males, with a peak
flexion is accompanied by external rotation. unstable (non-weight-bearing) child with
age of 1016 years in males and 915 years
Initial presentation of SUFE may take one possible SUFE, as inadvertent reduction
in females, although it has been reported may occur and potentially increase risk of
of four patterns:
in children as young as 8 years. SUFE is avascular necrosis.
usually related to puberty (80% occurring Preslip. This is usually a retrospective
during the adolescent growth spurt), and diagnosis, relating to an episode of
obesity (two-thirds are >90th centile intermittent pain and evidence of
with less than 3 weeks prodrome
weight-for-height), with genetic and endo- synovitis with widening of the growth
and no radiological evidence of
crine factors playing a role.44 Mechanical plate but without radiological evidence
remodelling.
failure occurs at a widened zone of hypertro- of epiphyseal shift.
phy within the physeal plate. It is important Chronic slip. Pain longer than 3 weeks in SUFE is also graded as Stable or Unstable
to note that although traditional Salter an ambulant child suffering mild to depending on the ability to weight-bear
Harris proximal femoral physeal fractures moderate pain, with decreased range of at the time of presentation, and as mild,
can occasionally occur in adolescence from motion. X-ray shows SUFE  evidence of moderate or severe depending on the per-
high-energy injuries, these differ from classi- remodelling. centage of epiphyseal translation. This grad-
cal SUFE both in the amount of force  Acute-on-chronic slip. Increased pain ing is important, as unstable slips have a
involved and in the histological plane of and radiological evidence of slip high incidence (50%) of complications
cleavage, i.e. the SUFE can be thought of following a period of lower-grade such as avascular necrosis.
as a pathological SalterHarris type 1 frac- symptoms. Acute slip and evidence of The radiological findings in SUFE are out-
ture occurring as a result of minor torsional remodelling are present on X-ray. lined in Fig. 24.1.3. It is essential that the
or low-energy injury in a weakened physeal Acute slip (10%). Acute, severe hip emergency physician be aware of the clinical
plate with a high-shear stress load.44 pain and diminished range of movement and radiological features of SUFE so that

526
24.1 ORTHOPAEDICS AND RHEUMATOLOGY
24

ORTHOPAEDICS AND RHEUMATOLOGY

they can recognise at-risk presentations, and Delays in diagnosis and referral are Table 24.1.9 Causes of acute multifocal
actively seek Kleins line (a line extending common and avoidable.49 Causes of delay limb or joint pain in children presenting to
from the superior border of the femoral neck include: the ED
which should intersect the lateral femoral Infection
head in the AP view of normal hip/pelvis). Failure to X-ray (particularly with Acute viral illness{
Although the slip is usually radiologi- low-grade and/or referred pain). Streptococcal disease
Other bacterial illness, e.g. Neisseria
cally more evident in the frog-leg lateral, Failure to recognise knee pain as a
Kawasaki disease
manipulation into this position should be presenting symptom of hip pathology.
Failure to interpret X-ray correctly, Post-infectious (immune-mediated)
avoided in unstable (non-weight-bearing)
particularly in Grade 1 (ED training Serum sickness{
children with possible SUFE, in case the Reactive arthritis{
process of obtaining it may precipitate should include the X-ray appearance of Rheumatic fever
further shear. the normal femoral head).
Inflammatory/vasculitic
Ultrasound by experienced operators has Failure to refer appropriately (immediate
HenochSchonlein purpura{
also been shown to have high sensitivity and telephone contact with orthopaedic Juvenile idiopathic arthritis (JIA)
team). Associated with SLE/IBD/other systemic
specificity for epiphyseal displacement, even inflammatory disorders
in the early stages.7
Neoplastic
Leukaemia{
Differential diagnosis GENERALISED OR Neuroblastoma
The differential diagnosis of SUFE has been MULTIFOCAL BONE/
Haematological
discussed in the section on limp. A normal JOINT PAIN Sickle cell
frog-leg pelvic X-ray in the ambulant child Haemophilia
rules out all but the preslip phase of SUFE.
In high-risk clinical situations (e.g. past or fam- Introduction IBD, inflammatory bowel disease; SLE, systemic lupus
erythematosus.
{
ily history of contralateral SUFE), MRI may be Common causes.
The child presenting with generalised or
helpful.
multifocal acute musculoskeletal distur-
bance represents a different diagnostic spec- Lymph nodes.
Treatment trum. This is outlined in Table 24.1.9. As Liver and spleen.
Once an unstable SUFE has been diagnosed, might be expected, infective and inflamma- Heart and lungs.
patients should be treated as at risk of avas- tory disorders predominate and the risk Kidneys and urine.
cular necrosis. Admission under the ortho- profile relates to the possible consequences
paedic team pending surgical fixation of the generalised disease process, such Investigations
should be expedited. Bed-rest with toilet pri- as rheumatic fever, nephritis in association Urinalysis and microscopy for casts.
vileges is usual, with pain relief as required. with HenochSchonlein purpura (HSP), Inflammatory markers FBC and film,
The acutely slipped femoral head is usually leukaemia, or juvenile arthritis. CRP, ESR.
fixed by percutaneous or minimal-incision In assessing the child with multifocal joint Serology, for recent infection due to e.g.
internal fixation. or bone pain, or with arthralgia or arthritis in streptococcus (antistreptolysin O test
a setting of a febrile illness, the following (ASOT), antiDNAse), EpsteinBarr virus
Complications features should be explored: (EBV), cytomegalovirus (CMV),
Avascular necrosis may occur in up to parvovirus, mycoplasma, Barmah Forest
50% of unstable slips, with an overall History Virus, Ross River fever virus, Yersinia.
incidence in SUFE of 5%. Other complica- Rheumatological investigations
Antigen exposure drugs, infections,
tions include chondrolysis and leg-length insects, animals. antinuclear antibodies, rheumatoid
discrepancy. factor, HLA-B27 antigen.
Previous infection or autoimmune
dysfunction.
Controversies/ Features suggestive of infection fever and
its pattern and duration, other symptoms.
developments Juvenile idiopathic arthritis
Features suggestive of metabolic drain
The necessity or danger of attempted lethargy, weight loss, anorexia, night (JIA)
reduction (which may precipitate sweats. This spectrum of disorders is outlined in
avascular necrosis) prior to fixation. Table 24.1.10.50 Whilst the course of these
The value of pinning the Examination disorders can be chronic, the onset of arthri-
contralateral hip (asymptomatic Skin and mucosae. tis may be sudden, although the affected
bilateral SUFE evolves in up to 40% Eyes (visual acuity mandatory). child may not be as acutely unwell as seen
of children). Bones (including spine), joints, and in septic arthritis. Presentation with pain is
surrounding soft tissues. variable and patients may present to their

527
24.1 ORTHOPAEDICS AND RHEUMATOLOGY

Arthritis or arthralgia may be minimal or

Table 24.1.10 Juvenile idiopathic
arthritis (JIA) absent in the early stages of disease.52 Urticaria and serum
Depending on the duration of fever at sickness
Systemic arthritis: fever 2 weeks plus at least
one of rash, enlarged lymph nodes, the time of ED presentation, and the clinical
hepatosplenomegaly, serositis
The young child presenting with low-grade
features present, differential diagnosis
Oligoarthritis (<5 joints in first 6 months since fever and urticarial rash may also have
onset): for these children may include acute viral
Persistent oligoarthritis: <5 joints involved
arthralgia or arthritis. Toddlers and pre-
or bacterial infection, Kawasaki disease
after 6 months school children may present with dramatic
Extended oligoarthritis >4 joints after 6 and other vasculitides, malignancy (e.g.
months
skin signs of urticaria, with migratory wheals
neuroblastoma), and other autoimmune dis-
Polyarthritis: at least 5 joints in first 6 months with or without target lesions, which are the
since onset orders such as systemic lupus erythemato-
RF negative
hallmark of erythema multiforme. Low-grade
sus. Investigations must include blood
RF positive temperature may be present and there may
Enthesitis-related arthritis: arthritis and/or culture, full blood examination and inflam-
enthesitis, plus two of sacroiliac/spine
be significant soft-tissue swelling and
matory markers, viral serology, and coagula-
inflammation, or HLA B27, or family history arthralgias. Whilst up to 50% of cases may
HLA-B27-associated disease in first- or tion studies. Investigations as appropriate to
second-degree relative, or anterior uveitis, or
be idiopathic, the most common single trig-
the clinical findings and differential
boy with arthritis onset over 6 years age ger identified with this symptom complex in
Psoriatic arthritis: arthritis and psoriasis or diagnoses, e.g. chest X-ray, electrocardio-
two of dactylitis, nail abnormalities (dystrophy
Australia is cephalosporin use.56 Atypical
gram (ECG), echocardiogram, and abdo-
or pitting), family history of psoriasis in a first- features such as mucosal lesions, inability
degree relative minal ultrasound may be warranted.
Undifferentiated arthritis: fits into more than
to weight-bear, high fevers and sick appear-
Anaemia of chronic disease is usual, with
one category, or excluded from any category ance, haematuria or organomegaly, require
by exclusion criteria elevated inflammatory markers and throm-
more detailed investigations and exclusion
bocytosis, which may exceed 106 per cubic
RF, rheumatoid factor. of more serious aetiologies. If symptoms
millimetre.53 Liver enzymes may be ele-
are mild, this condition may be managed
vated, whilst coagulation abnormalities
expectantly with removal of any unneces-
may be present and correlate with disease
sary medication and rest. Antihistamines
primary care services or ED. Associated activity.54
and NSAIDs may be useful for itch and
symptoms may include reduced function In order to optimise the outcomes of
joint pains.
(e.g. unwillingness to play) or gait abnorm- children with JIA, contact with a paediatric
alities. Inflammatory symptoms (e.g. gel- rheumatologist must be made. NSAIDs may
ling) may be more pronounced after a be used to treat the symptoms; however,
period of inactivity e.g. early morning stiff- disease-modifying drugs such as systemic
HenochSchonlein purpura
ness. For appropriate risk management, sep- steroids, cytotoxics, and joint procedures This leucocytoclastic vasculitic disorder of
tic arthritis needs to be excluded. High may be required. uncertain aetiology is among the more fre-
fevers, refusal to allow examination of quent diagnoses presenting as joint pain
the joint, as well as important elevation and rash in the paediatric population. Char-
in inflammatory markers and leucocyte acteristically there are four potentially
count, may suggest septic arthritis. Multiple affected systems:
joint involvement usually suggests Macrophage activation
inflammatory arthropathy; however, the
Skin extensor surface petechiae and
syndrome maculopapules in lower limbs and
most common subtype of JIA is oligoarthri- (haemophagocytic buttocks.
tis, with the knee as the most frequent
presenting joint.51
lymphohistiocytosis) Joints acral arthritis, but more
commonly arthralgia and associated
Presence of systemic features such as This rare disorder may present as a compli-
limb swelling.
persistent fevers (more than 2 weeks), cation of systemic arthritis and include coa-
especially with quotidian pattern (regular gulopathy and disseminated intravascular
 Kidney micro-or macroscopic
haematuria, with or without proteinuria,
spikes of temperature at a predictable time), coagulation, encephalopathy, with liver and
may be present or there may be frank
evanescent rash, lymphadenopathy and multiorgan failure.
nephritis.
organomegaly, with or without arthritis, Laboratory investigations reveal hepatic
should alert the attending clinician to the dysfunction, consumption coagulopathy
Gastrointestinal system abdominal
pain with submucosal vasculitis
possibility of systemic arthritis. Serositis with hypofibrinogenaemia, lowered haema-
presenting as intussusception, per
may manifest as pleural or pericardial tological indices without classical leukaemic
rectum bleed or melaena, or
effusion, requiring urgent treatment as car- features, and a rapidly falling ESR usually
peritoneal irritation.
diovascular emergency. Abdominal pain inappropriate to the level of severity of
may be the result of peritoneal irritation by the patients condition. High-dose steroids, Risk management involves ruling out other
the intra-abdominal inflammatory fluid, or ciclosporin and other cytotoxics, with high- causes of petechial illness, including leukae-
non-specific vasculitis affecting abdominal dependency support in an intensive care mia, idiopathic thrombocytopenic purpura
viscera. unit can be life-saving.55 and partially treated meningococcal illness,

528
24.1 ORTHOPAEDICS AND RHEUMATOLOGY
24

ORTHOPAEDICS AND RHEUMATOLOGY

and watching for renal complications.
Table 24.1.11 Diagnosing acute rheumatic fever in Australia60
Children must be followed-up with urin-
alysis for 6 months for delayed renal High-Risk Groups* All Other Groups
complications.57 Initial episode of ARF 2 major or 1 major and 2 minor manifestations
plus
evidence of a preceding GAS infection{

Recurrent attack of ARF 2 major or 1 major and 2 minor or 3 minor manifestations

in a patient with known plus
past ARF or RHD evidence of a preceding GAS infection{
Rheumatic fever
Major manifestations Carditis (including subclinical Carditis (excluding subclinical
Rheumatic fever has become rare amongst evidence of rheumatic valve disease evidence of rheumatic valve disease
Caucasians in Australia and New Zealand on echocardiogram) on echocardiogram)
Polyarthritis or aseptic monoarthritis Polyarthritis{
but still occurs and causes preventable or polyarthralgia{ Chorea
morbidity and mortality in the Aboriginal Chorea Erythema marginatum}
Erythema marginatum} Subcutaneous nodules
and Pacific Islander populations of these Subcutaneous nodules
countries, particularly in northern and
Minor manifestations FeverH FeverH
central Australia and the Maori and Pacific ESR 30mm/hr or CRP 30mg/L Polyarthralgia or aseptic mono-
Islander population of certain regions arthritis{
Prolonged P-R interval on ECGY ESR 30mm/hr or CRP 30mg/L
of New Zealand.58 The incidence in indige- Prolonged P-R interval on ECGY
nous children aged between 5 and 14 years
All categories assume that other more likely diagnoses have been excluded.
in northern Australia is estimated to be Please see text for details about specific manifestations.
250350/1000 children.58 Infected skin CRP, C-reactive protein; ECG, electrocardiogram; ESR, erythrocyte sedimentation rate; GAS, group A streptococcus.
*High-risk groups are those living in communities with high rates of ARF (incidence >30 per 100 000 per year in 514-
lesions from scabies are more often a source year-olds) or RHD (all-age prevalence >2 per 1000). Aboriginal and Torres Strait Islander Australians living in rural or
of the Streptococcus than throat carriage remote settings are known to be at high risk. Data are not available for other populations, but Aboriginal and Torres
Strait Islander Australians living in urban settings, Maori and Pacific Islander people, and potentially immigrants from
in this group. Because of the disparity of developing countries may also be at high risk.
{
likelihood in different populations, the Elevated or rising anti-streptolysin O or other streptococcal antibody, or a positive throat culture or rapid antigen test
for GAS.
Australian Heart Foundation recommends {
A definite history of arthritis is sufficient to satisfy this manifestation. Other causes of arthritis/arthralgia should be
different diagnostic criteria in high- and carefully excluded, particularly in the case of monoarthritis (e.g. septic arthritis, including disseminated gonococcal
infection), infective or reactive arthritis (e.g. Ross River virus, Barmah Forest virus, influenza, rubella, Mycoplasma,
low-risk Australian sub-populations. These cytomegalovirus, EpsteinBarr virus, parvovirus, hepatitis and Yersinia), and auto-immune arthropathy (e.g. juvenile
revised, bi-level Modified Jones criteria are chronic arthritis, inflammatory bowel disease, systemic lupus erythematosus, systemic vasculitis, sarcoidosis). Note
that if polyarthritis is present as a major manifestation, polyarthralgia or aseptic monoarthritis cannot be considered an
shown in Table 24.1.11. additional minor manifestation in the same person.
Rheumatic fever in indigenous Austra-
Rheumatic (Sydenhams) chorea does not require other manifestations or evidence of preceding GAS infection,
provided other causes of chorea are excluded.
lian children classically presents with an }
Erythema marginatum is a distinctive rash (see text). Care should be taken not to label other rashes, particularly non-
extremely painful polyarthritis, particularly specific viral exanthemas, as erythema marginatum.
H
Oral, tympanic or rectal temperature 38 C on admission or documented during the current illness.
affecting knees or ankles. Pain out of propor- Y
Note that, if carditis is present as a major manifestation, prolonged P-R interval cannot be considered an additional
tion to clinical effusion is the rule. The arthri- minor manifestation in the same person.
tis is slowly migratory but affected joints
often overlap in time. Treatment with
NSAIDs and/or aspirin (to which the joints
in rheumatic arthritis are acutely sensitive), Post-streptococcal and (e.g. erythema nodosum) or uveitis. The reac-
reduces the clinically apparent number of other post-infective tive arthritis pattern of post-streptococcal
joints involved. arthritis is more fixed than the migratory
immune-mediated reactive
Rheumatic fever classically occurs up to arthritis of classical rheumatic fever; teno-
arthritides synovitis is often present; and there are no
a month after a skin infection, whereas
other immune-mediated post-streptococcal Post-streptococcal and other post-infective cardiac manifestations. Post-streptococcal
disorders (reactive arthritis and glomerulone- reactive arthritides (as distinct from the rheu- arthritis also lacks the characteristic aspirin
phritis) tend to occur earlier, e.g. 2 weeks post- matic fever complex) need to be considered sensitivity of rheumatic fever.59 However,
infection. in the differential diagnosis of any young the association mandates investigation of
Although rheumatic fever and rheumatic child presenting with rash and joint swelling. these children with FBC, ASOT and anti-
heart disease are discussed in more detail These conditions may occur after viral or bac- DNAse, ECG, and referral for follow up by
in another section (see Chapter 5.6), terial infections, alone or in immunogenic a paediatrician or rheumatologist. Other
it is critically important to consider rheu- combination with antibiotics. These condi- organisms implicated in immune-mediated
matic fever in any indigenous or Pacific tions have generally a favourable joint out- arthritis include EBV, CMV, parvovirus,
Islander child presenting with acute arth- come, although occasionally other system mycoplasma, and various gastrointestinal
ralgias and fever, in view of the serious manifestations may be observed. pathogens.60
sequelae and preventable nature of this Children may also have other clinical man- The triad of urethritis, arthritis, and
disease. ifestations, including cutaneous vasculitis conjunctivitis associated with Chlamydia

529
24.1 ORTHOPAEDICS AND RHEUMATOLOGY

infection may present as Reiters syndrome Bone tumours apophysis and soft-tissue swelling. However,
in the sexually active adolescent, whilst Osteosarcoma and osteogenic sarcoma are this is primarily a clinical diagnosis (and frag-
other arthritogenic bacteria (e.g. Salmonella uncommon but serious causes of recent- mentation may be a normal radiological vari-
or Shigella) may cause arthritis associated onset limb pain or limp in the paediatric ant in developing apophyses). Treatment
with gastroenteritis. In each case, attempt population. Characteristic features include: includes rest, non-steroidal anti-inflammatory
must be made to identify the causative medication, and rheumatology or orthopaedic
organism and, if indicated, antimicrobial
osteosarcoma bone destruction and referral for persistent disability.
infiltration;
treatment implemented.
osteogenic sarcoma (Ewings tumour)
periosteal new bone formation, sunray Torticollis
and onion-skin appearance.
The infant presenting with torticollis, with-
Neoplastic presentations Differential diagnosis includes neuroblas- out specific trauma or abnormal neurology,
Bone or joint pain may be among the toma and osteomyelitis. These children is most likely to have congenital muscular
presenting symptoms in children with leu- should be referred for urgent orthopaedic torticollis (sternomastoid tumour). Theories
kaemia, neuroblastoma and other bone review and investigation. of pathogenesis include in-utero crowding
marrow-related malignancies. Features char- Osteoid osteoma is an occasional cause of and compartment syndrome. Characteristic
acteristic of these children by comparison leg pain, particularly nocturnal. X-rays show features include:
with other causes of limp or joint pain are an area of periosteal thickening and new bone
formation around a central radiolucency.
onset from birth, often brought to
shown in Table 24.1.12. This pain is gener- parents attention by new observer;
ally more severe and unremitting, and the Simple and aneurysmal bone cysts may
also be seen, either as incidental findings
cock-robin appearance (head tilt on
debility more extreme, than in children with involved side with contralateral chin
juvenile idiopathic arthritis. Blood films must or in the setting of pathological fracture.
rotation) with range of motion limited by
always be carefully analysed for the three All of these can be referred to the orthopae-
the affected sternomastoid tightness;
cell lines (red cells, white cells and platelets). dic unit for further evaluation.
normal neurology, behaviour, and
Minor degrees of anaemia, leucopenia, or occipitofrontal circumference;
thrombocytopenia are common early fea- Tip
firm painless swelling or tightness
tures: these must be flagged and followed Beware the child who is carried into the
palpable within the sternomastoid
closely.61,62 ESR is traditionally elevated examination room.
muscle opposite the chin;
out of proportion to the degree of arthritic
mild facial hemihypertrophy due to increased
manifestations.55 relative blood flow to the dependent side.
Plain radiography has been suggested as OTHER IMPORTANT
first-line radiological investigation to differ- SUBACUTE PAEDIATRIC Hips should always be checked, as a signifi-
entiate between acute lymphocytic leukae- MUSCULOSKELETAL cant proportion may have developmental
mia (ALL) and JIA, to aid establishment of dysplasia.65 Infants with this characteristic
PRESENTATIONS
a correct diagnosis in the child with persis- constellation of features should be referred
tent bone or joint pain.63 Soft tissue swelling for physiotherapy and follow up.
and osteopenia were characteristics of JIA Apophysisitis: Osgood Other causes of torticollis in the paediatric
cases, whilst radiolucent metaphyseal bands period include vertebral anomalies such
Schlatter and Severs
and coarse trabeculation were almost exclu- as KlippelFeil syndrome and neurological
diseases disorders including brain and spinal-cord
sive to ALL patients.
Malignancies presenting in this fashion These disorders are the result of chronic micro- tumours and ocular dysfunction. Wide based
include leukaemia and neuroblastoma.61,62,64 avulsion injury at the apophyseal insertion gait and frequent falls may be accompanied
sites of the major leg-muscle groups. Com- by cervical spine tumours. Children with JIA
monest sites are the tibial tuberosity (quadri- may present with abnormalities in head and
Table 24.1.12 Features suggestive of
ceps via patellar tendon: OsgoodSchlatter neck posture caused by cervical spine arthri-
malignancy in infants/children presenting
with bone/joint pain or limp disease) and the calcaneal apophysis (calf tis as their initial presentation, although
Non-weight-bearing or refusal to walk
muscle via Achilles tendon: Severs disease). symmetrical reduced range of motion is
Night time pain, waking from sleep In OsgoodSchlatter disease, the child or ado- the more common finding.
Non-articular bone pain or tenderness
Back pain
lescent typically presents during the growth Causes of acute torticollis in children with
Abnormal bone swelling spurt with a story of pain getting up from sit- previously normal neck posture and motion
Systemic illness (fever, rash, weight loss,
anorexia, night sweats)
ting or going up stairs, and has tenderness include atlantoaxial rotary subluxation (see
Bruising localised to the tibial tuberosity and some Chapter 24.2), a reaction to other acute
Abdominal mass or organomegaly
Abnormal neurology
localised swelling, with an otherwise normal head or neck pathologies, such as lymphade-
Low Hb, WCC, or platelet count joint examination. In Severs disease there is nopathy or retropharyngeal abscess, and
High ESR or CRP (out of proportion to joint
findings)
pain on walking or running, and heel tender- short-term muscle spasm in association with
ness. X-ray may show fragmentation of the respiratory-tract disorders or minor trauma.

530
24.1 ORTHOPAEDICS AND RHEUMATOLOGY
24

ORTHOPAEDICS AND RHEUMATOLOGY

Features suggestive of significant underly- management plan can be improvised. Early be treated with nonsteroidal anti-inflammatory drugs?
Ann Emerg Med 2002 09;40(3):2949.
ing pathology and need for radiological communication with relevant specialties 19. Taylor GR, Clarke NM. Recurrent irritable hip in
and specialist referral include: will aid the efficient delivery of the above childhood. J Bone Joint Surg Br 1995 09;77(5):
74851.
healthcare path. 20. Keenan WN, Clegg J. Perthes disease after irritable hip:
other congenital or orthopaedic delayed bone age shows the hip is a marked man.
anomaly; J Pediatr Orthop 1996 01;16(1):203.
21. Goldenberg D. Bacterial arthritis. In: Kelley W, Harris E,
abnormality of neurological or
ophthalmological assessment; Acknowledgement Ruddy S, Sledge C, editors. Textbook of rheumatology.
4th ed. Philadelphia, PA: WB Saunders; 1993.
p. 144966.
symptoms suggestive of intracranial The contribution of Trevor Jackson as author 22. Trobs R, Moritz R, Huppertz H. Changing pattern of
pathology, e.g. headache, vomiting, in the first edition is hereby acknowledged. osteomyelitis in infants and children. Leipzig Paediatr
irritability; Surg Int 1999;15:36372.
23. Duffy CM, Lam PY, Ditchfield M, et al. Chronic recurrent
atypical musculoskeletal examination, multifocal osteomyelitis: review of orthopaedic
e.g. limited range of motion, visual complications at maturity. 07J Pediatr Orthop 2002;22
References (4):5015.
disorder, or persistent or fixed 24. Girschick HJ, Raab P, Surbaum S, et al. Chronic non-
1. Top 10 Paediatric Emergency Department EDIS Discharge
torticollis. bacterial osteomyelitis in children. Ann Rheum Dis 2005
Diagnoses. Brisbane, Australia: Mater Childrens Hospital
02;64(2):27985.
South Brisbane; 1999.
25. Huber AM, Lam P-Y, Duffy CM, et al. Chronic recurrent
2. Woods D, Macnicol M. The flexion-adduction test: an
multifocal osteomyelitis: clinical outcomes after more
early sign of hip disease. J Pediatr Orthop B 2001 07;10 than five years of follow-up. J Pediatr 2002 08;141
(3):1805.
Vertebrospinal 3. Kallio M, Unkila-Kallio L. Serum CRP, ESR and WCC in
(2):198203.
26. Simm P, Allen R, Zacharin M. Bisphosphonate
inflammation septic arthritis of children. Pediatr Infect Dis J
treatment in chronic recurrent multifocal osteomyelitis.
1997;16(4):4113.
J Pediatr Apr;:571-5 Epub 2007 Nov 5. 2008;152
4. Jagodzinski NAM, Kanwar RM, Graham KMDFF,
While uncommon, vertebrospinal inflamma- Bache CEF. Prospective evaluation of a shortened
(4):5715.
27. Gleeson H, Wiltshire E, Briody J, et al. Childhood chronic
tory and infectious problems are charac- regimen of treatment for acute osteomyelitis and
recurrent multifocal osteomyelitis: pamidronate therapy
septic arthritis in children. J Pediatr Orthop 2009;29
terised by delay in diagnosis and diagnostic (5):51825.
decreases pain and improves vertebral shape.
J Rheumatol 2008;35(4):70712.
confusion.66 These children may present with 5. Ng T. ESR, plasma viscosity and CRP in clinical practice.
28. Graham H. Acute septic arthritis of the hip in children in
Br J Hosp Med 1997;58(10):5213.
back or abdominal pain, or with altered gait 6. Kothari NA, Pelchovitz DJ, Meyer JS. Imaging of
northern Australia [commentary]. Aust N Z J Surg
2003;73:91.
or neurological dysfunction. In discitis and musculoskeletal infections. Radiol Clin North Am 2001
29. Appleton S, Nourse C. Mater Health Service
07;39(4):65371.
vertebral osteomyelitis there will be point 7. Kallio P, Lequesne G, Paterson D, et al. Ultrasonography
Brisbane: Septic arthritis: cell counts and cultures
2007-2009.
tenderness over a spinous process, particu- in slipped capital femoral epiphysis. Diagnosis and
30. Moumile K, Merckx J, Glorion C, et al. Bacterial aetiology
assessment of severity. J Bone Joint Surg 1991;73
larly to percussion, and localised scoliosis or (6):8849.
of acute osteoarticular infections in children. Acta
Paediatr 2005 04;94(4):41922.
muscle spasm. Fever and constitutional symp- 8. Skinner J, Glancy S, Beattie T, Hendry G. Transient
31. Tilse M. Organisms cultured in paediatric septic arthritis
synovitis: Is there a need to aspirate hip joint effusions?
toms are variable. Inflammatory markers are Eur J Emerg Med 2002;9(1):158.
1998-2007. Unpublished data: South Brisbane, Australia:
characteristically elevated. X-ray may show Mater Childrens Hospital; 2007.
9. Jaramillo D, Treves S, Kasser J. Osteomyelitis and
septic arthritis in children: Appropriate use of imaging 32. Ilharreborde B, Bidet P, Lorrot M, et al. New real-time
muscle spasm or soft-tissue oedema, or an to guide treatment. Aust J Rheumatol 1995;165: PCR-based method for Kingella kingae DNA detection:
abnormal intervertebral disc space. Bone 399403. application to samples collected from 89 children with
10. Fink A, Bermann L, Edwards D, Jacobson S. The irritable acute arthritis. J Clin Microbiol 2009; 06/15/;47
scans are helpful to localise an abnormality hip: Immediate ultrasound guided aspiration and (6):183741.
where examination findings are equivocal. prevention of hospital admission. Arch Dis Child 1995;72 33. Arnold SR, Elias D, Buckingham SC, et al. Changing
(2):1103. patterns of acute hematogenous osteomyelitis and septic
Definitive diagnosis and evaluation are best 11. Beach R. Minimally invasive approach to management of arthritis: emergence of community-associated
made by MRI. Treatment with bed-rest irritable hip in children. Lancet 2000;355:12023. methicillin-resistant Staphylococcus aureus. J Pediatr
12. White P, Boyd J, Beattie T, et al. Magnetic resonance Orthop 2006;26(6):7038.
and intravenous antibiotics is usual, although imaging as the primary imaging modality in children 34. Saavedra-Lozano JM, Mejias A, Ahmad N, et al. Changing
isolated discitis may respond better to steroid presenting with acute non-traumatic hip pain. Emerg trends in acute osteomyelitis in children: impact of
Med J 2001;18(1):259. methicillin-resistant Staphylococcus aureus infections.
treatment. 13. McPhee EMD, Eskander JPAB, Eskander MSMD, et al. J Pediatr Orthop 2008;28(5):56975.
Imaging in pelvic osteomyelitis: support for early 35. Report WHO. Global tuberculosis control: surveillance,
magnetic resonance imaging. J Pediatr Orthopaed planning, finance 2003. Geneva: World Health
2007;27(8):9039. Organization; 2003.
Conclusion 14. Kocher MS, Zurakowski D, Kasser JR. Differentiating
between septic arthritis and transient synovitis of the hip
36. Teo HEL, Peh WCG. Skeletal tuberculosis in children.
Pediatr Radiol 2004 11/24/;34(11):85360.
in children: an evidence-based clinical prediction 37. Turnidge J, Bell J. Methicillin-resistant Staphylococcus
Musculoskeletal presentations of children algorithm. J Bone Joint Surg Am 1999 12;81 aureus evolution in Australia over 35 years. Microb Drug
and adolescents to emergency rooms have (12):166270. Resist 2000;6(3):2239.
15. Luhmann SJ, Jones A, Schootman M, et al. Differentiation 38. Lo W-T, Lin W-J, Tseng M-H, et al. Risk factors and
a wide range from acute illnesses requiring between septic arthritis and transient synovitis of the hip molecular analysis of panton-valentine leukocidin-
immediate treatment to the more indolent in children with clinical prediction algorithms. J Bone positive methicillin-resistant Staphylococcus aureus
Joint Surg Am 2004 05;86-A(5):95662. colonization in healthy children. Pediatr Infect Dis J 2008
conditions with longer time since symptom 16. Kocher MS, Mandiga R, Zurakowski D, et al. Validation of 08;27(8):7138.
onset. Such presentations are age depen- a clinical prediction rule for the differentiation between 39. Aroojis AJ, Johari AN. Epiphyseal separations after
septic arthritis and transient synovitis of the hip in neonatal osteomyelitis and septic arthritis. J Pediatr
dent and their urgency may also be related children. J Bone Joint Surg Am 2004 08;86-A Orthop 2000;20(4):5449.
to patient characteristics and their respec- (8):162935. 40. Rasmont Q, Yombi J-C, Van der Linden D, Docquier P-L.
17. Mattick A, Turner A, Ferguson J, et al. Seven year follow Osteoarticular infections in Belgian children: a survey of
tive environment. Eliciting accurate medical up of children presenting to the accident and emergency clinical, biological, radiological and microbiological data.
history as well as a detailed medical exami- department with irritable hip. J Acc Emerg Med 1999 Acta Orthop Belg 2008 06;74(3):37485.
09;16(5):3457. 41. Kealey W, Moore A, Cook S, Cosgrove A. Deprivation,
nation are essential requirements before 18. Kermond S, Fink M, Graham K, et al. A randomized clinical urbanisation and Perthes disease in Northern Ireland.
investigations and ultimately a rational trial: should the child with transient synovitis of the hip J Bone Joint Surg 2000;82(2):16771.

531
24.2 FRACTURES AND DISLOCATIONS

42. Vila-Verde V, da Silva K. Bone-age delay in Perthes of juvenile idiopathic arthritis: second revision. J 60. Diagnosis and management of acute rheumatic
disease and transient synovitis of the hip. Clin Orthop Rheumatol 2004;31(2):3902. fever and rheumatic heart disease in Australiaan
Relat Res 2001;385:11823. 51. Cassidy J, Petty R. Textbook of pediatric rheumatology. evidence-based review: National Heart Foundation of
43. Mata S, Aicua E, Ovejero A, Grande M. LeggCalve 4th ed. Philadelphia; London: W.B. Saunders; 2001. Australia (RF/RHD guideline development working
Perthes disease and passive smoking. J Pediatr Orthop 52. Wright D. Juvenile idiopathic arthritis. In: Morrissey R, group) and the Cardiac Society of Australia and
2000;20(3):32630. Weinstein S, editors. Lovell and Winters paediatric New Zealand 2006.
44. Morrissey R, Weinstein S. Lovell and Winters paediatric orthopedics. 5th ed. Philadelphia: Lippincott Williams & 61. Gupta D, Singh S, Suri D, et al. Arthritic presentation of
orthopaedics. 5th ed. Philadelphia: Lippincott Williams & Wilkins; 2001. p. 42757. acute leukemia in children: experience from a tertiary
Wilkins; 2001. 53. Schneider R, Laxer R. Systemic onset juvenile rheumatoid care centre in North India. Rheumatol Int [serial on the
45. Dillman JR, Hernandez RJ. MRI of LeggCalvePerthes arthritis. Baillieres Clin Rheumatol 1998;12:245. Internet] 2009.
disease. AJR Am J Roentgenol 2009;193(5):1394407 54. Bloom B, Tucker L, Miller L. Fibrin D dimer as a marker of 62. Jones O, Spencer C, Bowyer S, et al. A multicenter case-
Review. disease activity in systemic onset juvenile rheumatoid control study on predictive factors distinguishing
46. Gent E, Antapur P, Fairhurst J, et al. Perthes disease in arthritis. J Rheumatol 1998;25:1620. childhood leukemia from juvenile rheumatoid arthritis.
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(1):1622. activation syndrome and rheumatic disease in childhood: 63. Tafaghodi F, Aghighi Y, Rokni Yazdi H, et al. Predictive
47. Comte F, De Rosa V, Zekri H, et al. Confirmation of the a report of four new cases. Clin Exp Rheumatol 1993 plain X-ray findings in distinguishing early stage
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League of Associations for Rheumatology classification fever. Lancet 2005;366:15568.

24.2 Fractures and dislocations

Robyn Brady John Walsh

different age subgroups, the distribution

ESSENTIALS varies thus:

1 Fractures are common in childhood, due to high-level motor activity, developing co- infants/toddlers higher proportions of
ordination, and the mechanical properties of the growing skeleton. However, femur and skull fractures;
compared to adult mechanisms, the majority are relatively low-force injuries. primary school higher proportion of
elbow-region fractures, especially
2 The patterns of bony disruption are completely different from adult fracture supracondylar;
patterns and include buckle and greenstick fractures and growth-plate injuries. Bony
adolescents complex ankle fractures;
disruption/deformity is more common than ligamentous disruption: sprains and
higher force sporting injuries; transition
ruptures are uncommon.
to adult pattern with increasing
3 Displaced fractures are a common and highly traumatic event for children and ligamentous injuries, e.g. elbow
rapid attention to physical and psychological distress can minimise the effects of this dislocations.
trauma.
The majority of ED paediatric fracture pre-
4 Certain missed fractures have a high propensity for serious long-term functional sentations occur at the distal radius and
morbidity and must be actively sought. These include elbow injuries, such as lateral ulna. This is one of the top ten ED diagnoses
condylar and Monteggia-type fractures. for children in Australia. Many displaced
forearm fractures can be reduced under
sedation by emergency staff with appropri-
cleavage or deformation from a given ate training and follow up, making this
Fracture patterns a most valuable area of expertise.
injury mechanism, and an extra anatomical
in childhood structure (the physis) to consider when Paediatric limb fractures, depending on
In the previous chapter the impact of devel- analysing the effects of trauma and the the angle of force to which they have been
opment (behavioural and physiological) on future outcome of a given disruption. subjected, can occur to shaft, metaphysis,
musculoskeletal pathology was broadly Fig. 24.2.1 shows the frequency of or physeal region. The different quality of
outlined (see Table 24.1.1). With respect common fractures presenting to a chil- developing bone means that even injuries
to injury, this means different points of drens emergency department (ED). Within to shaft and metaphysis tend to have

532
24.2 FRACTURES AND DISLOCATIONS
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ORTHOPAEDICS AND RHEUMATOLOGY

A

Face and skull 18 B

Spine and pelvis 2
Clavicles and upper humerus 28
Elbow 57
Forearm ML 151
Hand and carpus 55
Femur and patella 5 C
Tibia and fibula 27
Foot and tarsus 24 Fig. 24.2.4 Common distal radial epiphyseal
fractures. (A) Partial slipped distal radial
Fig. 24.2.1 Frequency of fracture types at epiphysis (SalterHarris 1) with 10% translation;
Brisbane Mater Childrens Hospital ED. (B) slipped distal radial epiphysis (SalterHarris 1)
with 50% translation and probable intact dorsal
periosteal sleeve; (C) SalterHarris 2 fracture of
different patterns of deformation, including distal radius with metaphyseal fragment
torus or buckle injuries, bowing, and green- angulated to 45 degrees and 50% translation of
stick fractures. The importance of this epiphyseal plate. Drawing by Terry McGuire.
awareness for the emergency physician is
best illustrated by the Monteggia equivalent
injury in which shortening from proximal
radial dislocation is matched by ulnar bow-
ing. The resultant injury has no radiologi- Fig. 24.2.2 Monteggia fracture-dislocation.
Demonstration of the abnormal radio-capitellar
cally obvious fracture in the traditional
relationship (see Fig. 24.2.8 for contrast).
sense but has serious consequences if not Drawing by Terry McGuire.
recognised and reduced (Fig. 24.2.2).
The SalterHarris classification (Fig. 24.2.3)
remains the most useful way of describing
the pattern of cleavage with respect to the visible fragment to an extensive trian-
physis. In reality, types 1 and 5 represent gle. Injuries through the epiphysis itself,
mechanical force patterns (separation and SalterHarris types 3 and 4, are more worry-
compression) rather than a radiological pat- ing in their prognosis because they are intra- Fig. 24.2.5 Tillaux fracture (SalterHarris 3).
tern as, unless there is lateral translation or articular as well as involving the physis. The Early fusion of the medial distal tibial physis and
adjacent bony or soft-tissue deformation, the classic example of a SalterHarris type 3 relative superior strength of the distal tibiofibular
ligament cause shearing force to separate the
physis may appear radiologically normal in injury is the Tillaux fracture (Fig. 24.2.5), central physeal region and travel along the lateral
these injuries. An example of SalterHarris while lateral condylar fractures at the elbow distal tibial physis. Sometimes a metaphyseal
type 1 injuries with lateral shift is the so- are SalterHarris 4 in type. fragment is also cleaved (SalterHarris 4).
called slipped distal radial epiphysis
(Fig. 24.2.4). The disorder of slipped upper
femoral epiphysis (SUFE) has been discussed
in Chapter 24.1 as, although minor trauma
may precipitate an acute slippage, the
cleavage is due to an abnormal physeal
predisposition and should not be looked
upon as truly traumatic.
SalterHarris type 2 injuries are the most
common physeal injury pattern seen, the
metaphyseal corner (the Thurston-Holland 1 2 3 4 5
fragment) ranging in size from a barely Fig. 24.2.3 SalterHarris classification of epiphyseal fractures. Drawing by Terry McGuire.

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24.2 FRACTURES AND DISLOCATIONS

Table 24.2.1 Examples of paediatric vs. adult outcomes of common fall mechanisms (different paediatric injuries occur at different ages
depending on planes of weakness). The ligaments in children provide greater resistance to shear injury than the growing bone, so avulsion type
injuries occur in place of ligamentous tears or dislocations.

Mechanism Adult injury Paediatric injury

Fall onto point of shoulder AC separation Lateral clavicular fracture

Shoulder extension/compression Shoulder dislocation Proximal humeral fracture

Fall on hand, elbow hyperextension Elbow dislocation Supracondylar/condylar fractures

Wrist hyperextension/compression Scaphoid fracture Distal forearm fracture

Fall onto hand Colles fracture Midshaft, metaphyseal, or epiphyseal fracture

Thumb abduction 1 Bennets fracture Metaphyseal fracture base first metacarpal

Thumb abduction 2 Gamekeepers thumb (UCL) UCL avulsion fracture (SalterHarris type 3
proximal phalanx thumb)

Rotation of knee on lower leg ACL, cartilage tear Tibial spine fracture

Valgus/varus knee stress Ligament, cartilage tear Distal femoral physeal separation

Forceful jump (quadriceps) Ligament tear Patellar tendon avulsion fracture (Tibial tubercle)
fracture

Forceful jump (calf) Achilles tendon tear Calcaneal avulsion fracture

Rotation of tibia on calcaneus Ankle sprains, Potts fractures Tibial spiral fracture, Tillaux fracture, triplane
fracture

Inversion ankle Talofibular ligament tear SalterHarris type 1 or 2 distal fibula

Table 24.2.1 shows some examples of should always be carried out bearing in mind or neurovascular impairment, and organise
the corresponding injury occurring in the described injury mechanism and the pain relief, fasting, radiology, splintage,
adults and children for a given mechanism. childs complaints of pain, so that any asso- and antibiotics if required, within a brief
This table illustrates the maxim that ciated injuries, e.g. to head, abdomen, or period.
children tend to fracture rather than spine, may be recognised and evaluated Fracture descriptions to the orthopaedic
sprain, as the physis is the weakest point early. An efficient early assessment should team should start with the childs age, mech-
of the musculoskeletal continuum, i.e. be able to establish mechanism, possible anism, and clinical findings, and proceed to
a ligament will avulse its bony origin or other sites of injury, probable fracture type, the part of bone, type of fracture, and extent
insertion rather than tearing. In some presence or absence of compound features of angulation and/or displacement and
cases, this is to the childs advantage, as
the cellular architects of bone development
which contribute to its mechanical weak-
Table 24.2.2 Initial assessment and management of traumatic limb deformity
ness contribute to rapid healing and
1. Rapport: establish rapport and explain procedures
extensive remodelling. A midshaft femoral 2. Mechanism and associated dangers: rapidly ascertain mechanism and ensure primary survey
fracture, for example, will heal in 23 weeks stability and allergy potential
3. Pain management: where there is a greater than 90% likelihood of initial IV success, insert a small IV
in an infant, whereas the same disruption cannula into the opposite hand and titrate morphine 0.05 mg kg1 until pain relieved (appropriate
will take 12 weeks to union in a teenager. monitoring should be in place). When no parent is present the risks of medication must be weighed
against the potential benefit and attempts made to contact someone familiar with serious allergies or
other medical problems. Other means of rapid pain relief include inhaled Penthrane or NO2, and or
intranasal fentanyl
4. Assess for site of anatomical disruption: look at and gently palpate the injured limb to estimate
Initial assessment probable anatomical site of disruption, e.g. lateral elbow, mid-shaft forearm, etc. (comparison with
other limb is often helpful)
and management 5. Check for associated neurovascular dysfunction: (see Table 24.2.3), document and notify deficits
immediately
The initial assessment of the paediatric 6. Check for any evidence of an open wound: if this is present, cover with a sterile dressing and
commence appropriate antibiotics IV, e.g. cefalothin 50 mg kg1 and notify orthopaedic team
isolated limb injury (fracture/dislocation) immediately
is shown in Table 24.2.2 and the neuro- 7. Immobilise limb: provide appropriate splintage (e.g. we use a POP slab for distal fractures, leaving
radial artery palpable, and a fibreglass slab for elbow injuries, less radiological artefact-mouldable, re-
vascular assessment in Table 24.2.3. Limb usable, radiolucent slabs)
injury must always be considered in the 8. Keep stomach empty: identify time of last ingestion and inform patient and parent about fasting
9. Organise appropriate radiology
broader context of trauma. Primary and sec- 10. Discuss clinical and radiological findings with orthopaedic team
ondary survey, however brief and targeted,

534
24.2 FRACTURES AND DISLOCATIONS
24

ORTHOPAEDICS AND RHEUMATOLOGY

Table 24.2.3 Presence/absence of Table 24.2.4 Features suggestive of possible non-accidental injury
associated neurovascular injury
Fractures
Radial a:
Proximal humeral or humeral shaft fractures under 3 years
Pulse, cf. other side
Fractures with a shearing or distracting mechanism
Hand perfusion/capillary refill, cf. other side
Corner or bucket-handle metaphyseal injuries
Brachial a:
Femoral fractures in infants
Beware spasm or intimal shear in
Rib fractures
supracondylar injuries
Complex skull fractures
Radial n:
Multiple fractures, especially different ages
Sensation dorsum hand, action dorsiflex
wrist, extend fingers (displaced humeral shaft
injury) Presentation features
Median n: Delayed presentation
Sensation thenar eminence, action Different care-giver
opposition, flexion IP thumb (supracondylar # Unwitnessed injury
or elbow dislocation) Recurrent fractures
Ulnar n: Unexplained soft-tissue markings
Sensation hypothenar eminence, action Unexplained tension/anxiety
abduction, adduction fingers (elbow
dislocation, supracondylar #) Assessment
Posterior interosseous n:
Branch of radial n, action finger extension, Draw diagram of injury history as described by witness
e.g. (Monteggia #/dislocation) Examine child all over and plot weight
Ascertain any previous history of burns or fractures
Is the developmental level compatible with the explanation?
Is the history adequate to explain the injury?

Rule of thumb
associated findings. Clinical findings must Infants within view, toddlers within earshot, unless asleep, i.e. injuries to pre-school children are usually
seen or heard
always be kept paramount. Skin breach must
be actively sought and described, then Refer
photographed and covered with a sterile All fractures in children under 12 months, and all fractures in children under 4 years in which there is an
dressing. Prominently placed photographic inadequate or questionable mechanism, should be discussed with a child-protection specialist. In the
interim, supportive and non-judgemental care for child and care-giver must be maintained
displays of common paediatric fractures
within the emergency department may aid
accurate description. The following sections describe the mech- must be considered. The prognosis for neo-
Doctors share in the community responsi- anism, recognition, and ED treatment of natal clavicular injuries is excellent.
bility for child safety. Within the ED setting individual fractures.
this means getting a clear description of Shoulder dislocation
the setting and mechanism of injury, partic- This is uncommon under 10. The adolescent
ularly with injuries to pre-verbal children.
Upper limb and shoulder anterior dislocation can be reduced by trac-
These data are important:
girdle injuries tion in the prone position or by gentle arm
to more clearly anticipate associated traction to a seated child against counter-
Midshaft clavicular fractures traction with a sheeted thorax.
injuries (e.g. foreign bodies or distant
These may occur at any age from a fall onto
possibility of abusive injury);
the shoulder or outstretched hand, are usu-
to gather cumulative injury prevention
ally greenstick in nature and heal well in a Proximal humerus
data to support legislative change (e.g. These fractures vary from minor buckling at
sling or figure-of-eight bandaging. A fall
road access); and the proximal metaphysis, to proximal humeral
onto the point of the shoulder, such as would
to flag possible abusive injury, which is
cause an acromioclavicular disruption in an epiphyseal SalterHarris type 2 fracture-
thought to have occurred in 12% of separations (Fig. 24.2.6). Because of the uni-
adult, may cause a lateral clavicular physeal
paediatric injury presentations, versal motion at the glenohumeral joint and
fracture-separation. If these are posteriorly
particularly in very young children.1 the remodelling potential of children, a
displaced, operative treatment may be
In general, fractures in pre-verbal children required. Treatment is by sling or shoulder remarkable range of initial traumatic defor-
without a clear, developmentally appropriate immobilisation followed by graduated mity is acceptable in children prior to physeal
mechanism/history or with other concerning exercises. closure (age 1416), including complete dis-
features, will need further assessment. The neonatal shoulder may come to med- placement and up to 60 degrees of angula-
Features suggestive of non-accidental injury ical attention due to asymmetrical arm tion.2 A collar and cuff is the usual treatment.
are shown in Table 24.2.4, and child abuse movement or swelling. Causes include birth
is discussed in more detail in Chapter 18.2. injury with clavicular fracture or brachial Midshaft humeral fractures
As a minimum, all fractures occurring plexus injury, proximal humeral physeal sep- These are less common and sometimes the
in children under 12 months should be aration, and joint or bone infection. Senior result of blunt or non-accidental trauma.
discussed with a paediatrician or child-protec- orthopaedic involvement is essential for Check and document radial nerve function,
tion specialist. the diagnosis, and non-accidental injury and immobilise with a U-slab.

535
24.2 FRACTURES AND DISLOCATIONS

A
A B

Fig. 24.2.6 Proximal humeral fractures. (A) Normal undulating physeal line (not a fracture); (B)
greenstick metaphyseal fracture; (C) severely angulated and displaced SalterHarris 2 fracture at the
proximal humeral epiphysis. Due to the universal motion of the shoulder joint, this fracture will still unite
and remodel completely with conservative treatment. Drawing by Terry McGuire. B

Fig. 24.2.8 The normal capitello-radial head

Injuries to the elbow region The first point in itself will define relationship. Drawing by Terry McGuire.
The elbow region accounts for 10% of all pae- an anatomically intact elbow, while the
diatric fractures. Supracondylar fractures other points help the physician to narrow
make up 75% of these, and lateral condylar down the type of abnormality where the Gartland Type 1 undisplaced supra-
fractures 17%.3 Missed or inadequately first point is abnormal. condylar fracture This is the presump-
treated paediatric elbow injuries figure tive diagnosis with elbow effusions that are
prominently in orthopaedic litigation series.4 Supracondylar fracture tender bilaterally and have no ulnar or radial
Post-traumatic elbow effusion in childhood Supracondylar injuries occur in the young dislocations or significant displacement/
without a radiologically apparent fracture school-age child as a result of a fall on the angulation. The fracture line through the
line most commonly represents a minimally outstretched hand, transmitted through bone between olecranon and coronoid
displaced supracondylar fracture. These must elbow hyperextension to the narrow region fossae may be seen on the AP view or recog-
be immobilised by collar and cuff to avoid between olecranon and coronoid fossae. nised as a disruption to the normal teardrop
any potential extension from further falls, Degrees of rotation of the distal region rela- between the opposing fossae on the lateral
and followed up in a fracture clinic for tive to the main axis of the humerus are humeral view, but its absence should not
a repeat X-ray at 714 days. It is some- common, depending on the degree of prona- prevent the immobilisation of these elbow
times useful to start with the examination tion/supination at the time of fall. effusions until early orthopaedic review.
findings in the normal elbow, as outlined
in Table 24.2.5 (Figs 24.2.7 and 24.2.8).
Appear
Cap 1
Rad 3
Table 24.2.5 Requirements for elbow Int (Med) 5
clearance Ext (Lat) Int (Med) Troc 7
Olec 9
The normal paediatric elbow must have Troc Ext (Lat) 11
Full extension, supination and pronation Cap
No swelling or significant focal bony C. R. I. T. O. E.
tenderness Rad
No abnormal anterior or posterior fat-pad
sign on radiographs
Normally placed and age-appropriate Olec
ossification centres (see Fig. 24.2.7)
An intact radio-capitellar relationship (see
Fig. 24.2.8) Fig. 24.2.7 CRITOE. The approximate order of appearance of the six elbow ossification centres according
to the CRITOE mnemonic. Drawing by Terry McGuire.

536
24.2 FRACTURES AND DISLOCATIONS
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ORTHOPAEDICS AND RHEUMATOLOGY

Gartland Type 2 posterior angulation Lateral condyle
with probable intact periosteal hinge This fracture results from a varus force on
(Fig. 24.2.9A) In this situation it is impor- the supinated forearm, avulsing the condyle
tant to check for associated rotation or varus/ (Figs 24.2.11 and 24.2.12). There is clinical
valgus injury. Use the anterior humeral line swelling and tenderness, which is maximal
as a guide to the degree of posterior angula- over the lateral condyle. It is usually a
tion (Fig. 24.2.10), and consult orthopaedics SalterHarris type 4 fracture, but the late
about all injuries. Simple (2a) fractures with
less than 20 degrees of angulation may be
managed conservatively in a backslab and
collar and cuff, with orthopaedic follow up. 1/3
2/3
If there is varus/valgus angulation or rotation
on the AP view (Gartland Type 2b), then
orthopaedic consultation is required acutely Fig. 24.2.10 The anterior humeral line rule for
as surgery may be indicated. Remember that subtle supracondylar fractures. A line passed
remodelling may correct some loss of flexion along the anterior humeral cortex on a lateral
elbow radiograph should bisect the anterior and
or extension but will not correct rotation or
middle thirds of the capitellum. If it passes
varus/valgus deformity. anterior to the capitellum, there is at least
20 degrees dorsal angulation of the distal
Gartland Type 3 grossly displaced/ humerus. Drawing by Terry McGuire.
rotated (Fig. 24.2.9B) Check for open
injury or neurovascular compromise. Brachial
artery spasm or kinking is common with
this injury, and the gross associated swelling
may predispose to compartment syndrome.
Radial pulse and hand perfusion should Fig. 24.2.11 Subtle lateral condylar fracture in
be continuously reassessed. In cases with a toddler. Note only two ossification centres
extreme swelling, extension may be (capitellum and radial head) and thin rim of
safest. Immediate orthopaedic notification metaphysis shorn away with capitellum. Greater
angulation/displacement of metaphyseal
is required.
fragment may be shown on lateral view. Gross
If orthopaedic help is not available within clinical swelling is the key. Drawing by Terry
1 hour of the onset of poor hand perfusion, McGuire.
A
attempt gentle traction and reduction under
anaesthesia, e.g. ketamine, aiming for the
position with best hand perfusion.
Median or radial nerve injury may also
occur (usually as praxis), and require ortho-
paedic evaluation. Ulnar nerve injury is most
commonly reported as an iatrogenic injury
following internal fixation.
Supracondylar Gartland Types 2b and
3 fractures require admission for MUA
and K-wiring and occasionally open reduc-
tion, and appropriate management of
complications.
B

Intercondylar (T-condylar) fracture

This is a variant of the supracondylar frac-
ture occurring in adolescents. It results from
axial impaction and intra-articular separa-
Fig. 24.2.9 Supracondylar fractures. (A) Grade tion of capitellum and trochlea, as well as
Gartland Type 2, approximately 45 degrees dorsal
proximal disruption of the medial and lateral
angulation but probable intact dorsal periosteal Fig. 24.2.12 Displaced and rotated lateral
hinge; (B) grade Gartland Type 3, complete distal humeral columns. Treatment is by condylar fracture (Milch type I). Long-term
displacement, often co-existent rotation and/or internal fixation, with or without open complications may ensue if not surgically fixed.
neurovascular impairment. Drawing by Terry McGuire. reduction. Drawing by Terry McGuire.

537
24.2 FRACTURES AND DISLOCATIONS

appearance of the trochlear and lateral epi- Pulled elbow (radial head
condylar ossification centres means that the subluxation, RHS)
true structural disruption is not demon- Children from age 6 months presenting with
strated by radiology, and therefore not acute disuse of one arm, which they hold in
appreciated by emergency staff, particularly a semi-flexed and pronated posture, and a
in the younger child. The varus angulating history of traction, can be presumed to
force characteristically causes disruption have pulled elbow or RHS if there is point
commencing above the lateral condyle, tenderness at the radial head and no
passing to a varying extent along the physis, palpable elbow effusion, i.e. no infilling of
and in complete disruptions exiting either the soft tissue space medial and lateral
lateral (in the majority of cases; Milch type 1), to the olecranon in comparison with the
or medial (Milch type 2) to the capitellar- unaffected arm.
A
trochlear groove. If uncorrected, the injury Subluxation occurs because the oval
may result in valgus deformity, and possible shape of the radial head allows the head
delayed ulnar nerve palsy and degenerative to sublux slightly through the annular liga-
elbow disease. ment when the forearm is pulled in prona-
Bony displacement is often best seen on tion. Part of the ligament is caught in the
the lateral X-ray. radiocapitellar space, and in fact partial
The clinical significance of this fracture tears can occur.5,6 In older children the liga-
means that: ment is thicker and more densely attached,
and subluxation in a child over 5 is unusual.
Any lateral condyle fracture with a
X-ray and/or ultrasound, seeking alterna-
greater than 2 mm separation of
tive diagnoses, should be obtained in any
fracture segments is likely to require
child with other points of focal tenderness,
internal fixation, particularly if
an elbow effusion, a mechanism of greater
displacement is proximal.
trauma, an atypical history, e.g. fever, or a
All young children with major elbow
failure of the procedure detailed below.
deformity/swelling as a result of injury
should be assessed early by experienced
orthopaedic personnel. Ultrasound, B
Reduction of RHS
magnetic resonance imaging, and
Fig. 24.2.13 Medial epicondylar avulsion. A recent prospective randomised trial has
arthrography may all have a role to play (A) The opacity below the humerus is the avulsed suggested that hyperpronation is more
in determining the line of injury and medial epicondyle, which should be sitting more likely than supination to reduce the pulled
consequent best means of fixation. proximal and medial. There should not be a
trochlear opacity without a medial epicondylar
elbow on the first occasion, and elicits less
Within the ED, an internal oblique
ossification centre. Developmentally, there discomfort.7
radiograph can be helpful.
should not be an ossification centre in the After a brief parental explanation and
The elbow may be supported in a radiolu- trochlea position without one in the medial oral or intranasal pain relief, these children
cent backslab while awaiting orthopaedic epicondylar position. (see Fig. 24.2.7). (B) Lateral should be held firmly by a parent while
view shows opacity between capitellum and
review, but X-rays in this radiologically the forearm is hyperpronated. It is helpful
olecranon, possibly intra-articular. Drawing by
complex region are best performed prior to Terry McGuire. for the doctor to cradle the elbow in the
plaster application. outer hand with the thumb over the radial
In infants, lateral humeral condylar sepa- six. It will generally reunite readily with the head while their inner arm rotates. Success
ration may occur as a SalterHarris 1 type humerus if it lies within 5 mm, unless there is is usually denoted by a momentary pain, a
fracture and be difficult to diagnose radio- interposing tissue. Occasionally, particularly palpable click, and a return to functional
logically, although the elbow will be grossly when the avulsion has occurred in associa- use. If the procedure is not successful, the
abnormal with maximal swelling laterally. tion with a posterior elbow dislocation, the procedure can be repeated, and if this fails,
History must explain the varus force, epicondyle and its attachments may become the traditional method of full, firm supina-
and abusive injury should be considered. lodged within the elbow joint and may block tion and flexion can be attempted. This com-
Ultrasound may be useful diagnostically. an attempt at closed reduction. Ulnar nerve bination of techniques should elicit success
injury is a common association. This circum- in >90% of cases of radial head subluxa-
Medial epicondylar avulsion stance is one of the main practical uses of tion.7 If the above process is unsuccessful,
This may occur in association with other knowledge of elbow ossification centres the history and examination should be revis-
disruptions, e.g. elbow dislocation, or as a (see Fig. 24.2.7). These must be systemati- ited and imaging sought. Interestingly, while
discrete event (Fig. 24.2.13). The medial epi- cally reviewed on every elbow X-ray so radiographs should be normal (and should
condyle is the origin of the common flexor that missing or misplaced opacities may be not show posterior fat pad elevation, which
tendon, and ossifies at approximately age identified. should suggest alternative diagnoses), the

538
24.2 FRACTURES AND DISLOCATIONS
24

ORTHOPAEDICS AND RHEUMATOLOGY

radiocapitellar distance is significantly about 135 degrees, against countertraction by the presence of localised tenderness
increased in radial head subluxation on to distal humerus. Full extension should be at the radial head. Neurovascular injury, par-
ultrasound due to the presence of the inter- avoided as it may cause further damage to ticularly to posterior inter-osseous nerve
posed ligament; a tear may sometimes be the ulnar nerve. If there is difficulty in resit- (finger extension), should always be sought.
shown. If an alternative diagnosis is not ing the olecranon, there may be soft tissue Undisplaced fractures and those with up to
suggested by imaging and careful re- inter-position and orthopaedic help should 5060% displacement do well with conser-
assessment, RHS remains the most likely be sought, as a computerised tomography vative treatment and may be immobilised in
diagnosis: the child can be allowed home (CT) scan may be indicated. The reduced a collar and cuff with orthopaedic follow up.
with the arm supported in a sling in a neu- elbow should be held in flexion with a poste-
tral position, and with review at 24-48 rior splint, a check X-ray performed, and Monteggia fracture dislocation
hours, at which time many will have sponta- orthopaedic follow up arranged. The peak incidence of this injury complex is
neously reduced. Persistent dysfunction in the 410 age group. Although, classically,
beyond 48 hours requires orthopaedic eval- radio-capitellar disruption accompanies an
Proximal radial and ulnar
uation. Although some children sustain angulated or shortened ulnar fracture, the
fractures
recurrent RHS, it rarely requires operative displacement may occur in association with
Olecranon fractures
intervention.8 any displacement to the radioulnar loop.
These may occur in older children as: (1)
avulsion (flexion) fractures (generally requir- Because of the significant complications of
ing internal fixation); (2) extension fractures a missed radio-capitellar disruption, this
Elbow dislocation
with intra-articular opening (may be stable lesion must be actively clinically and radio-
Appearing first in adolescence, this injury,
in flexion); or (3) comminuted fractures from logically sought in any child with a forearm
the result of a fall on to the hand with par-
a direct blow to the elbow. Clinical correla- fracture and elbow swelling. Most units
tially flexed elbow, is uncommon in young
tion must be sought in diagnosing olecranon adopt a rule of always X-raying the elbow
children (who sustain supracondylar frac-
fractures, as the ossification centre, which of any child with forearm or wrist injury,
tures instead). The majority dislocate poste-
appears around 10 years of age and may unless the elbow has been specifically clini-
riorly, tearing joint capsule, and stretching
be bipartite or fragmentary in appearance, cally cleared (see Table 24.2.5).
soft tissues. The displacement may cause
is easily mistaken for a fracture. The classical (Type 1) Monteggia lesion
fracture to the coronoid process of the ulna
involves an angulated apex-volar ulnar shaft
or radial neck, or the medial epicondyle may
fracture and anterior displacement of the
be avulsed. Neuropraxis of median or ulnar Radial neck fractures
radial head (see Fig. 24.2.2). Variants include
nerves may occur. These are relatively common injuries in the
lateral radial head displacement, often in
The dislocation should be suspected paediatric population. The radial head itself
association with proximal ulnar/olecranon
clinically. After assessing for associated is largely cartilaginous and rarely injured.
fractures (Type 3), Monteggia equivalent
injuries, it should be reduced in the ED, Injuries range from subtle torus type beak-
fractures including proximal radial fracture/
generally under ketamine anaesthesia ing of the neck, which is best seen on the lat-
dislocation, and others. A bowing deformity
(Fig. 24.2.14). Gentle downwards pressure eral side on the lateral projection, to
may be the only evidence of fracture. Ortho-
can be applied to the supinated proximal displaced SalterHarris type 1 or 2 fractures.
paedic manipulation of the radial head into
forearm, with extension of the elbow to Suspicion of an isolated injury may be made
its articulation is the aim of the treatment.

Midshaft radial and ulnar

fractures
Assistant
holds These injuries fall into two broad groups:
(1) common low-energy greenstick fractures
as a common consequence of falls in child-
Downward
pressure hood; and (2) higher-energy complete frac-
tures, which may be difficult to reduce,
requiring internal fixation in 510% of cases.
In the former group, with the exception of
bowing fractures (which generally require
general anaesthetic/ orthopaedic reduction,
as prolonged corrective force is necessary),
ED reduction may be possible thus:
Traction
apex volar pronate forearm and apply
Fig. 24.2.14 Reduction of elbow dislocation. Assistant anchors humerus to avoid distal/anterior
wrist traction and volar pressure;
movement. Bring forearm in full supination to approximately 20 degrees from full extension, and provide
simultaneous downwards traction and downwards pressure over the proximal forearm to lever the apex dorsum supinate forearm and
coronoid process back under the distal humerus. Drawing by Terry McGuire. apply wrist traction and dorsal pressure.

539
24.2 FRACTURES AND DISLOCATIONS

Although these fractures may be simple to incidence of subsequent premature physeal acceptable. Isolated metacarpal fractures
relocate under anaesthesia, e.g. ketamine, closure or other physeal disruption. This risk will not create malrotation. Isolated meta-
their inherent instability makes expert is greatest following multiple or delayed carpal neck fractures should be treated with
three-point moulding essential. Therefore, reduction attempts, or compressive injuries, neighbour strapping and mobilisation.
manipulation should not be attempted or distal physeal separation of the ulna.2 Fol-
unless such expertise and assistance is avail- lowing manipulation, referral to fracture Phalangeal fractures
able, and follow up within 1 week is impera- clinic must be within a week as repeat Common paediatric phalangeal fractures
tive in case of subsequent loss of position. manipulation of the physis is contraindi- include SalterHarris 2 fractures at the base
cated after 7 days. of the first phalanx, which may cause radial/
Distal radial and ulnar fractures ulnar angulation and should be corrected by
These common injuries may be metaphyseal Carpal injuries: the scaphoid traction after a ring block.
or epiphyseal in nature. Dorsal angulation Because of the flexibility of the paediatric These may be radiologically subtle and
occurs in 80% of cases, but radial or volar wrist and the plastic properties of preossified require careful clinical evaluation. All open,
angulation/displacement of the distal frag- bone, carpal injuries are very rare in children intra-articular or oblique (unstable) frac-
ments may also occur. Regarding manage- under 8 years. Scaphoid injuries are gener- tures should be referred for orthopaedic
ment, the following guidelines apply: ally overdiagnosed in children in the ED evaluation.
setting. Of those fractures that do occur,
Simple torus (buckle) fractures with no 65% are distal pole and non-union is rare Thumb fractures
periosteal or cortical breach, which
because of the different mechanical and vas- Forced thumb abduction, e.g. from fall onto
represent plastic deformation only, may
cular properties of immature bone. As chil- the splayed hand, can cause avulsion of part
be placed in a forearm splint or brace for
dren reach adolescence, their risk of adult- of the proximal thumb physis (a Salter
comfort and referred for follow up in 12
type scaphoid fractures increases. Scaphoid Harris type 3 injury instead of the adult
weeks.9
views (AP and oblique with attention to pos- ulnar collateral ligament tear), or a meta-
Undisplaced greenstick fractures, in which
sible obliteration of the navicular fat pad) physeal fracture of the base of the first
a cortex or periosteum has been
are suggested in older children if:1214 metacarpal. The intra-articular SalterHarris
breached, have inherent instability and
type 3 avulsion injury should be internally
the potential for further deformation. adolescent (10 years and over); fixed, so referral is essential. However, in the
These should be managed in a well- high-velocity injury, especially kickback; metaphyseal injury, because of the universal
moulded (three-point fixation) plaster in single-point tenderness and swelling over motion of the first carpometacarpal joint,
neutral position,10 and reviewed within scaphoid both dorsally (in anatomical
significant angulation and displacement will
1 week. snuffbox) and on volar surface under base
remodel if the child is under 10 years, and
Angulated or displaced greenstick of first metacarpal (more specific finding);
the child may have the fracture immobilised
fractures of the distal forearm generally KirkWatson test (pain/clunk in
in a scaphoid type plaster extending to the
require reduction if the angulation is scaphoid/scapholunar ligament on
tip of the thumb, elevated, and be referred
greater than 20 degrees, although passive radial deviation of wrist);
for early orthopaedic consultation.
remodelling potential varies with the age pain to compression along first
of the child and the distance of the metacarpal ray.
Fingertip injuries
fracture from the physis. Closed reduction
Suspected fractures should be managed These injuries to young children are
may be performed in the ED if staffing
with scaphoid plaster and orthopaedic extremely common from inadvertent closure
and expertise permit.
follow up as usual: conservative treatment in doors or gates, especially in cooler cli-
Again, attention must be paid to ensure that usually allows resolution of true injuries mates. Injuries include partial or complete
a well-moulded plaster will maintain reduc- but may take up to 6 months. amputation, nail-plate injuries, and distal
tion, and follow-up orthopaedic review phalangeal fractures.
should be early enough to detect this and Metacarpal fractures The classical crush injury includes a distal
remanipulate if necessary i.e. within 1 week. Crush injuries to metacarpal bones may phalangeal tuft fracture and a partial ampu-
Early orthopaedic referral should occur for occur, and adolescence sees an increase in tation anteriorly through the nail bed, with
angulated isolated radial fractures and for fractures of the head of the fifth metacarpal, intact volar soft tissue attachments and vas-
fractures of radius and ulna with complete as in adults, although the intact distal meta- cular integrity. Tip salvage is usual. However,
displacement and shortening, as a signifi- carpal physis will allow some remodelling to without meticulous repair to the nail-bed
cant proportion of these manipulations will correct flexion loss. Penetrating trauma, ten- laceration nail deformities are common, so
be problematic or require internal fixation.11 don damage, open injuries and neurovascu- referral to orthopaedics or plastic services
lar impairment must be identified and is recommended.
Distal radial epiphyseal fractures referred. Multiple fractures may create an Tip amputations distal to the terminal
These can generally be treated by manipula- unstable hand plate, and finger flexion phalanx may often heal by secondary intent,
tion and closed reduction in the same way as should be observed for possible associated because of the excellent vascular supply in
metaphyseal fractures. There is a very low rotational malalignment, which is not childhood. More proximal injuries require

540
24.2 FRACTURES AND DISLOCATIONS
24

ORTHOPAEDICS AND RHEUMATOLOGY

assessment of nail-bed integrity, the The simultaneous management of all these be managed by cast immobilisation. Ortho-
possibility of soft-tissue (e.g. nail-bed) inter- challenging priorities is a good test of paedic consultation is imperative because of:
position within an angulated/displaced the mature, multidimensional emergency
fracture, or other indications for reconstruc- physician.
the possibility of co-existent intra-
articular ligament disruption;
tive procedures. Simple procedures may be As has been mentioned earlier, all limb
performed in the ED under ketamine anaes- fractures should be initially approached with
the need for perfect articular surface
realignment;
thesia or sedation and ring block. rapid primary and secondary survey while
the integrity of airways, breathing, circula-
the significant possibility of subsequent
asymmetrical physeal arrest.
tion, conscious state and spinal column are
assessed, mechanism ascertained, and areas
Lower limb and pelvis of tenderness identified. An intravenous (IV) Tibial spine injury
injuries cannula should be inserted immediately, Because of the mechanical properties of
Pelvic fractures under nitrous oxide or intra-nasal fentanyl developing bone, twisting injuries to the
These injuries are less common in paediatric if necessary. Early attention to issues of pain paediatric knee result in avulsion of the
than adult trauma. Avulsion injuries in ath- and anxiety has been shown to reduce the tibial attachment of the anterior cruciate lig-
letic adolescents may occur. The implica- stress and pain of later procedures. Femoral ament (Fig. 24.2.15). The child presents
tions for blood loss and urogenital injury nerve block, preferably ultrasound guided, non-weight-bearing, with a large effusion
of unstable pelvic fractures in children are should be inserted early, with Thomas splint and joint-line tenderness. Although AP
similar to those in adulthood. The bladder immobilisation following in a timely fashion. X-ray may be deceptive, lateral projections
is an intra-abdominal organ in infants. Not all femoral fractures are the result of show a characteristic beak-like appear-
major trauma. In the newly ambulant child, ance of the superior surface of the tibial
the torsion resulting from a change of for- plateau, with posterior hinging. All children
Hip dislocation ward momentum with the foot fixed at an with large effusions should be referred
This is uncommon in childhood, occurring angle may produce a spiral midshaft frac- for orthopaedic evaluation. Orthopaedic
either with low force as a result of increased ture. Mechanisms in abusive injury include
ligamentous laxity, or in the high-force forced external rotation or abduction, e.g.
mechanisms more typical of adult hip dislo- from nappy change position, or direct blows.
cation. The sequel of avascular necrosis is Types of femoral fracture in children, and
less common, occurring in only about 5% their orthopaedic implications, are as follows:
of cases.2 Reduction, by gentle closed longi-
tudinal traction against a fixed pelvis, should Proximal femoral fractures include
transepiphyseal, transcervical,
be performed within 6 hours. Particular care
basicervical, and intertrochanteric
must be taken in the adolescent in whom an
fractures. These are serious injuries.
occult physeal injury may be displaced. Post
Avascular necrosis and physeal growth
manipulation X-rays  CT are indicated.
arrest are significant potential
complications of higher fractures, and early
Femoral fractures notification and reduction are urgent.
Although comprising less than 5% of pae- Slipped upper femoral epiphysis
diatric fracture presentations to the ED, (masquerading as trauma) is discussed in
the infant or child presenting with a femoral Chapter 24.1.
fracture presents particular challenges to In femoral shaft fractures, definitive
the emergency physician because of: orthopaedic management depends on the
degree of precipitating force, associated
the frequent association with major injuries, the age of the child, home
trauma and other unstable injuries;
circumstances, and the institution. Low-
the high level of pain and emotional
force injuries in young children may be
distress for the child and the family,
treated by closed reduction and early hip
particularly in the setting of road trauma;
spica casting. Options in older children
the need for procedural skills, such as
usually involve internal or external fixation.
femoral nerve block, and the application
of traction splinting, such as the
Thomas splint; Injuries about the knee Fig. 24.2.15 Lateral and AP views of the
paediatric knee with an avulsed tibial spine.
The possibility of non-accidental Distal femoral physeal separation
Due to the intact anterior cruciate ligament, the
injury (possible incidence suggested These injuries result from high-force trauma tibial spine fracture (shaded) may be hinged
at between 30 and 80% in children to the knee. Any of the SalterHarris pattern posteriorly, opening anteriorly on knee flexion.
under 12 months15,16). injuries may occur. Undisplaced injuries may Drawing by Terry McGuire.

541
24.2 FRACTURES AND DISLOCATIONS

management involves an assessment, gen- degree of angulation or displacement; Ankle fractures

erally under anaesthetic, of the reducibility associated soft-tissue damage or other As with adults, inversion, eversion and twist-
of the avulsed spine and the likelihood injuries; ing mechanisms may cause a variety of
of, e.g. meniscal interposition. Treatment involvement of physes; injury patterns at the ankle depending on
options include immobilisation in extension integrity of fibula. age, degree of force, and mechanism. Ankle
or partial flexion, and internal fixation. Some injuries requiring same-day orthopaedic
Compound injuries and those with physeal
degree of subsequent instability and loss of consultation include:
involvement or significant angulation or dis-
extension may follow.
placement should be referred for inpatient open injuries;
Avulsion of tibial tubercle orthopaedic evaluation. Proximal tibial epiph- unstable injuries or ankles with extensive
This adolescent injury represents avulsion of yseal injury may be complicated by vascular bilateral tenderness and swelling,
the insertion site of the patellar tendon, usu- compromise, as with adult knee dislocation. suggesting possible mortice instability;
ally as a result of forceful quadriceps contrac- Varus or valgus deformity, particularly at the displaced or angulated distal tibial
tion against resistance. Displaced injuries proximal tibia, may progress. Stable, undis- fractures;
require internal fixation and immobilisation. placed or minimally displaced oblique or spiral SalterHarris type 3 and 4 injuries;
shaft fractures of the tibia may be placed in a Triplane and Tillaux fractures.
Patellar dislocation well-moulded above-knee cast with the knee
This injury occurs most commonly in female flexed to 90 degrees and the ankle in 15 Tillaux fracture
adolescents with an excessive Q-angle degrees of plantar flexion.17 Admission is This is a SalterHarris type 3 fracture at the
(the complementary angle to the vastus not required if swelling is minimal, mechanism distal tibial physis, occurring in adolescence
lateralis/patellar tendon vectors), torsional is clear, and parents are sensible. after partial closure of the medial growth
anomalies, or hypermobility. The usual pattern The so-called toddler fracture is an undis- plate (see Fig. 24.2.5). External rotation of
is of lateral dislocation following a twist, e.g. placed tibial shaft fracture that occurs as a the foot and ankle causes avulsion of the
during a fall. Most reduce spontaneously or result of a rotational shearing force in the anterolateral portion of the distal tibial phy-
during transport; extension of the knee may newly ambulant child. Presentation is with sis by its attachment to the fibula (anterior
be facilitated in the prone position to relax non-weight bearing or limp and the differen- tibiofibular ligament). The diagnosis should
hamstring muscles. A sky-line view should be tial includes other pathologies, e.g. irritable be suspected in a non-weight-bearing ado-
obtained for best visualisation of osteochon- or septic hip. Tenderness should be localised lescent with significant anterolateral ankle
dral fragments from the medial patella. The to the tibial shaft but initial radiology may swelling and tenderness. Oblique ankle
knee should be placed in a Richards-type splint be normal. If this diagnosis is suspected views or CT assessment may be required to
and the child referred for physiotherapy and in a well child with normal joint examina- detail alignment.
orthopaedic follow up. Chronic dislocation tion, POP immobilisation and orthopaedic
occurs in approximately 1:6 cases. follow up with repeat X-ray at 10 days or Triplane fracture
nuclear bone scan may be helpful. This also occurs in the adolescent with exter-
Patellar fractures nal rotation injury, but the fracture line also
These are less common in children than in tears off a section of posterior tibial meta-
adults. Osteochondral avulsions may occur physis, which is clearly visible on the lateral
with dislocation. Displaced fractures, with
Controversies
radiograph.
tense haemarthroses and lack of full knee Controversy exists surrounding Both of these transitional fractures may
extension, should be referred to the ortho- boundary issues between emergency be complicated by subsequent joint incon-
paedic team. The patellar sleeve fracture medicine and orthopaedics. Who gruity or growth disturbance. Orthopaedic
may be radiographically occult as it repre- performs which reduction should be advice should always be sought.
sents separation of the cartilaginous distal determined by consideration of safe,
patella from the ossification centre. Findings effective resource use between
Inversion injuries
will include patella alta and excessive ante- emergency and orthopaedic services.
Ligamentous rupture is rare in children.
rior tenderness and swelling. Structured opportunities for
Carefully assess point of maximal tender-
Bipartite patella (secondary ossification cen- interdepartmental teamwork help
ness. A point of maximal tenderness over
tre) is a common radiographic normal variant. reduce angst and improve systems and
the physis may represents a SalterHarris
service quality.
type 1 separation of the distal fibular physis
Lower leg fractures
Lower leg fractures are common in child-
Length (SA vs LA POP) and type and should be cast immobilised for 23
(circumferential vs 3/4 slab) of plaster weeks until stable. Bony injury may also
hood. Factors to be considered in their
for unstable forearm fractures post occur at the base of the 5th metatarsal.
assessment include:
reduction is controversial; however, Radiographically, this is shown by an avul-
age; accuracy of moulding is probably more sion fracture line at right angles to the
mechanism (low- or high-force, type of critical than either other variable. metatarsal bones (the normal apophysis
injury, e.g. valgus/rotation); sits parallel and lateral). Although the

542
24.2 FRACTURES AND DISLOCATIONS
24

ORTHOPAEDICS AND RHEUMATOLOGY

Ottawa ankle rules have not been specifi- 5. Kim MC, Eckhardt BP, Craig C, Kuhns LR. Ultrasonography

cally validated in children, the requirement Future directions of the annular ligament partial tear and recurrent pulled
elbow. Pediatr Radiol 2004;12/27/; 34(12):9991004.
for further assessment of an injury involving The increasing trend to outpatient 6. Kosuwon W, Mahaisavariya B, Saengnipanthkul S, et al.
Ultrasonography of pulled elbow. J Bone Joint Surg Br
extensive swelling, bony tenderness, or management of injuries will mean 1993 05;75(3):4212.
inability to weight bear seems appropriate. increasing numbers of paediatric 7. Bek D, Yildiz C, Kase O, et al. Pronation versus supination
maneuvers for the reduction of pulled elbow: a
fractures having definitive treatment in randomized clinical trial. Eur J Emerg Med 2009 06;16
(3):1358.
the emergency department. A thorough 8. Triantafyllou SJ, Wilson SC, Rychak JS. Irreducible pulled
Controversies familiarity with paediatric fractures and elbow in a child. A case report. Clin Orthop Relat Res
1992;11(284):1535.
their traps is essential, as is attention to 9. Davidson J, Brown D, Barnes S. Simple treatment for torus
Paediatric fracture management
quality assurance as discussed in fractures of the distal radius. J Bone Joint Surg Br 2002;84
interfaces the emergency practitioner (7):1085.
Chapter 24.4. 10. Boyer B, Overton B, Scrader W, Riley P. Position of
with orthopaedic and physiotherapy
immobilisation for paediatric forearm fractures. J Pediatr
teams, amongst whom management of Orthop 2002;22(2):1857.
such issues as growth plate injuries 11. Gibbons C, Woods D, Pailthorpe C, et al. The management
of isolated distal radius fractures in children. J Pediatr
(including SUFE), and early mobilisation, Acknowledgement Orthop 1994;14(2):20710.
may be controversial. 12. Evenski AJ, Adamczyk MJ, Steiner RP, et al. Clinically
The contribution of Terry McGuire as author suspected scaphoid fractures in children. J Pediatr Orthop
2009 06;29(4):3525.
in the first edition is hereby acknowledged. 13. Hernandez JA, Swischuk LE, Bathurst GJ, Hendrick EP.
Scaphoid (navicular) fractures of the wrist in children:
Conclusions attention to the impacted buckle fracture. Emerg Radiol
2002 12/09/;9(6):3058.
14. Weber DM, Fricker R, Ramseier LE. Conservative
Paediatric fracture patterns are completely treatment of scaphoid nonunion in children
different from adult fracture patterns and References and adolescents. J Bone Joint Surg Br 2009 09;91
1. Clark RC, Brady RM, Pitt WR, et al. Flagging possible (9):12136.
include buckle and greenstick fractures abusive injury in young children: The role of the injury 15. Thomas S, Rosenfield N, Leventhal J, Markowitz R. Long-
and growth-plate injuries. proforma. Abstract epublished in Emerg Med Australasia bone fractures in young children: Distinguishing
2010 Feb. accidental injuries from child abuse. Paediatrics 1991;88
Sprains are uncommon as the physis or 2. Morrisey R, Weinstein S. Lovell and Winters pediatric (3):4716.
ligamentous insertions are the weakest orthopedics. 5th ed. Philadelphia: Lippincott Williams & 16. Hui C, Joughin E, Goldstein S, et al. Femoral fractures in
Wilkins; 2001. children younger than three years: the role of
chains 3. Mater Childrens Hospital B. 2002 Fractures in children by nonaccidental injury. J Pediatr Orthop 2008;28
Despite a great propensity for remodelling, EDIS discharge diagnosis SepNov 2002. (3):297302.
4. United Medical Protection pc. Orthopaedic claims under 17. Yang J, Letts R. Isolated fractures of the tibia with intact
unrecognised displacement/angulation may 16 years over the period 1985-2002. In: RM B, ed. fibula in children: A review of 95 patients. J Pediatr
have serious and long-term implications. Brisbane: 2003. Orthop 1997;17:34751.

543
 24.3 Spinal injury
Ed Oakley

The large amount of cartilage present in

ESSENTIALS the paediatric cervical spine can make radio-
graphic evaluation difficult. This is espe-
1 Spine injury is often associated with other severe injury. cially true in the first few years of life, and
2 Upper cervical spine injuries are more common in children. Thoracic and lumbar in the upper cervical vertebrae.
spine injury is strongly associated with spinal cord injury. The atlas ossifies from three ossification
centres: two ossification centres of the
3 Adequate immobilisation of the entire spine is needed. Spinal immobilisation can lateral masses and one ossification centre
cause complications, especially in the patient with spinal cord injury.
for the body. The ossification centre for
4 Clinical evaluation of the cervical spine can only occur in a child who is conscious, the body does not ossify until about
able to communicate and free from any other significant injuries. The cervical spine 1 year of age. The posterior arches fuse by
can be cleared on clinical findings alone in specific circumstances. 3 or 4 years of age, while the synchond-
rosis between the lateral masses and the
5 A good quality three-view series of the cervical spine (lateral, AP and odontoid body fuses at approximately 7 years of
view) is adequate to assess the bony structures. Clinical findings should be considered
age.3,5
when clearing the cervical spine radiographically.
The axis ossifies from seven ossification
6 Patients with spinal injury have a high incidence of fractures at another level. centres. The five primary ossification centres
are two for the lateral masses, two for the
7 Spinal cord injury may cause hypotension and relative bradycardia. odontoid (which are usually fused at birth
8 Spinal cord injury may occur without radiographic abnormality. but occasionally persist as a dens bicornis),
and one for the body. The odontoid is sepa-
rated from the body by a synchondrosis,
which fuses between 3 and 6 years old.
is similar to that in adults, with the majority The two secondary ossification centres are
Introduction in the lower three cervical vertebrae.2,5,6 The the tip of the odontoid process (which
Injuries to the spine and spinal cord are less thoracolumbar junction is the most com- appears at about 3 years of age and is usu-
common in children than adults. The injuries monly injured area outside the cervical ally fused by 12 years of age), and the infe-
have different distributions and frequency spine, with the thoracic and lumbar spines rior ring apophysis (which, like other ring
in different-aged children, dependent upon having roughly equal incidence of about apophyses, generally ossifies after 8 years
the developmental, anatomical and phy- 25%. There is an increased incidence of neu- of age and fuses in the early 20s).3,5
siological differences with age. Children rologic injury in fractures of the thoracolum- The remainder of the cervical vertebrae
account for up to 10% of all spinal injuries, bar junction. The relatively high incidence of each contain three primary ossification cen-
but the mortality among spine-injured injuries in this region is due to the large tres, one for the body and one for the two
children is higher than in adults, with esti- range of motion and the changing orienta- neural arches, and two secondary ossifica-
mates ranging from 2530%, with death tion of the facet joints.1,7 Approximately tion centres, the ring apophyses. The neural
most often due to associated injuries to 30% of patients with spinal cord injury have arches fuse posteriorly by the age of 3,
other organs, especially the brain.1 fractures at more than one spinal level. and anteriorly the three ossification centres
The incidence of spinal cord injury A majority of these are in contiguous fuse between 3 and 6 years of age. Impor-
amongst spine-injured children is probably vertebral segments, but 515% may be in tantly, the vertebral bodies are wedge-
about 1%.1,2 In neurologically impaired different regions.7 shaped until the age of 7 when they begin
survivors the injuries are most commonly to square off.3,4
at the C1C2 level, or in the lower cervical The thoracic and lumbar spines develop in
Developmental anatomy
or thoracic spine.1 The common causes of a similar way, with secondary ossification
spine and spinal cord injuries in children
and physiology centres for the spinous process and the
are motor vehicle crashes, falls, diving acci- There are a number of injury patterns of the transverse processes added. By the time
dents, sports injuries and, occasionally, spine and especially the cervical spine the child is 810 years of age the spine
non-accidental injury.1,3,4 that are unique to children. In order to has reached near-adult size.3,4
The majority of injuries in children occur in understand the differences between adult There are a number of other differences
the cervical spine. In children under 8, most and paediatric spine injuries knowledge of of importance for the spine. The fulcrum of
(about 80%) occur in the C1C3 region, the developmental anatomy of the spine is movement of the neck is located at C2C3
whereas after 8 years of age the incidence essential. in the infant, at C3C4 by the age of 6,

544
24.3 SPINAL INJURY
24

ORTHOPAEDICS AND RHEUMATOLOGY

and by the age of 8 the fulcrum is at C5C6, assessment difficult, and potentially raising
Table 24.3.1 Indications for initial
as it is in the adult. There is a relatively immobilisation of the spine intracranial pressure. Rigid immobilisation
large head and weak neck muscles; laxity in a collar and head immobiliser on a spinal
Level of consciousness
of the ligaments and joint capsule; and Inability to give history of pain board has also been shown to decrease tidal
relatively horizontal positioning of the Neck or back pain volume and respiratory excursion.10 In addi-
Neurological signs or symptoms
facet joints with underdevelopment of the Multiple system trauma tion, because the young childs head is dis-
uncinate processes.1,3,4 All these features History of significant trauma proportionately large, the neck is flexed
Fall from height >3 metres
increase the risk of injury to the childs spine. Pedestrian or cyclist hit by car when immobilised on a standard spinal
Unrestrained passenger in motor vehicle board. This causes flexion of the cervical
Crash diving accident
History of spinal abnormality spine, which may cause movement at the
site of injury. To prevent this, a spinal board
Initial assessment with a recess for the head or padding that
All patients with significant trauma should elevates the torso is needed for children less
be assumed to have a spine or spinal cord than 8 years old (Fig. 24.3.1).1113
injury and appropriate precautions must A number of different cervical collars
Table 24.3.2 Problems associated with
be taken to prevent further exacerbating spinal immobilisation in children are available and satisfactory to use.
any possible injury. The initial assessment Incorrectly fitted cervical-spine collar causing
The clinician must be familiar with the
of patients with potential spine or spinal distraction of the spine or allowing excessive method of sizing and applying the collar
movement
cord injury should be directed at the Flexion of the spine in children under 8 years
available, as an incorrect fit will allow too
airway, breathing and circulation, in line of age much movement of the collar if too small,
Reduction in tidal volume and limitation of
with trauma resuscitation guidelines (see respiratory effort
or will produce a distraction of any existing
Section 2). The patient should be stabilised Airway obstruction injury if too large.
Increased intracranial pressure
and a thorough secondary survey performed. Discomfort
For children who have less major trauma
At this point all possible spine and spinal Distress and anxiety but are at risk of spinal injury, a degree of
Pressure sores
cord injuries should be identified. The lateral judgement is needed by the clinician as to
cervical spine X-ray will be done at this the level of immobilisation that is appropri-
stage in patients with major trauma. Thor- ate. However, in all patients with multiple or
ough radiological assessment of the injuries significant other injuries immobilisation is
should be completed once resuscitation and and alternative immobilisation is needed. still recommended. If the child is not on a
stabilisation and the secondary survey have An ill-fitting collar may cause the chin to spine board, immobilisation solely in a cervi-
all been accomplished. become trapped under the chin support cal collar, maintaining alignment of the neck
A thorough history of mechanism of injury, and may cause airway obstruction. The and the entire spine, is appropriate. Using
previous spinal injury, other illnesses, partic- young child may become distressed from sandbags and tape or other means to immo-
ularly respiratory illness (acute or chronic), being rigidly immobilised, making further bilise the head by fixing it to the bed risks
cardiac illness, or bone disorders, medication
and allergies is needed to determine the
patients premorbid physiological status.

Spinal immobilisation A
Spinal immobilisation is currently a contro-
versial issue, especially in young children.
A balance must be found that will diminish
the risk of further injury to the childs spine,
but not interfere with the assessment, or B
the normal physiological functions, of the
child. Traditionally the spine has been immo-
bilised in a rigid cervical collar, on a spine
board with a head immobiliser and straps,
or with sandbags and tapes, thus provi- C
ding adequate control of the entire spine
Fig. 24.3.1 Effects of spine-board on cervical spine position in children. Child immobilised on a
(Table 24.3.1).3,8,9
standard backboard (A), and on backboards modified with an occipital recess (B) and a mattress
There are a number of potential problems pad (C). Modified from Herzenberg et al 1989 Emergency transport and positioning of young children
with this immobilisation (Table 24.3.2). who may have an injury of the cervical spine: The standard backboard may be hazardous. Journal
Cervical collars are not made to fit infants of Bone & Joint Surgery 71(1): 1522.

545
24.3 SPINAL INJURY

further complications, including airway com-

Table 24.3.3 A classification of spine injuries
promise, and the risks are likely to outweigh
the benefits.14,15 Mechanism of injury Stability
The back is examined by log rolling the Flexion
child. This requires at least three people in Flexion teardrop fracture Very unstable
the smallest of children, and up to five in Bilateral facet joint dislocation Unstable
Atlanto-occipital dislocation Unstable
larger patients. The child is freed from the Displaced odontoid fracture Unstable
head immobiliser and any strapping to the Anterior subluxation Unstable
Anterior wedge fracture Stable
spinal board removed. One person must sta- Clay shovellers fracture Very stable
bilise the cervical spine by supporting the
Extension
head and neck in the neutral position
Atlantoaxial dislocation Very unstable
throughout movement. No traction should Hangmans fracture C2 Unstable
be applied to the neck. The other team Extension teardrop fracture Unstable (in extension)
members control the shoulders, hips and Rotation
legs, and one person must be free to inspect Rotary atlantoaxial dislocation Unstable
and palpate the back. The back is inspected Rotary atlantoaxial subluxation Stable
Unilateral facet dislocation Stable
for bruises, abrasions, wounds or deformity
of the spine. The spinous processes are pal- Vertical compression
pated for tenderness. The back of the neck Jefferson fracture (burst # C1) Very unstable
Burst fracture vertebral body Stable
and head and the buttocks and anus should
be inspected. A rectal examination is not Source: Modified from Rosen P et al 1997. Emergency Medicine, 4th edn. Mosby, St. Louis, MO, USA.
mandated in all children.
To limit discomfort and the likelihood of
pressure sores a child should be kept on
the spinal board for the shortest possible of the vertebra leading to a compression at C1 being most unstable. Spinal cord
time. Once resuscitation and urgent proce- fracture or teardrop fracture. The posterior involvement is from a retropulsed fragment
dures and investigations are completed elements of the spine are distracted with lig- of bone or intervertebral disc.
the board should be removed. This is espe- amentous injury, dislocations, or avulsion In many instances, not one but a combi-
cially important in patients with suspected fractures of the spinous processes. These nation of these mechanisms of injury is
spinal cord injury.16 injuries can be stable (such as the anterior involved, producing more than one type of
wedge compression fracture or the clay sho- injury.
vellers fracture) or unstable (such as a bilat-
Cervical spine injuries eral facet joint dislocation).
Clinical assessment
As the cervical spine is the most common After immobilisation and resuscitation of
region injured and accounts for the majority
Extension
Hyperextension injuries produce distracting the injured child and as part of the second-
of spinal cord injuries, a thorough knowl- ary survey the neck should be examined.
forces anteriorly, while compressing the pos-
edge of injuries in this region and the appro- This is done to look for neck abrasions and
terior vertebral structures. Most extension
priate assessment of the spine both clinically signs of injury to other structures in the neck
and radiographically is mandatory. In chil- injuries are unstable, and buckling of the
ligamentum flavum into the posterior of as well as to examine the cervical spine. The
dren younger than 8 years of age the major- decision to evaluate the cervical spine for
the spinal canal can cause a central or pos-
ity (but not all) injuries occur above the injury should only be made in children who
terior spinal cord syndrome.
fourth cervical vertebra. After 8, the pattern are conscious and alert, who are not drug
of injury is similar to that seen in adults (the or alcohol affected, and who do not have
majority of injuries below C4).2,17 Rotation other injuries that are painful or distracting
In the cervical spine isolated rotary injuries enough to make assessment of neck pain
Mechanisms of injury are uncommon. Subluxation can be sponta- difficult.2,1719
The mechanism of injury is an important his- neous or follow minor or major trauma. It is The cervical collar should be removed
torical factor, as it will determine the type generally a stable injury. However, if there is while another person holds the head inline.
and possible instability of the underlying dislocation of the facet joints of C1/C2 the No traction should be applied to the neck.
injury (Table 24.3.3).15 lesion is unstable. The cervical spine is palpated for tenderness
over the spinous processes. If there is tender-
Flexion Vertical compression ness over a specific region the collar should
The most common mechanism of injury seen These injuries are due to axial compression be reapplied and the spine evaluated with
is hyperflexion. This type of injury produces of the cervical spine. This can produce a X-ray. If there is no tenderness (or only soft
a compressive force on the anterior segment burst fracture of any vertebra, with a lesion tissue tenderness) the child should be

546
24.3 SPINAL INJURY
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ORTHOPAEDICS AND RHEUMATOLOGY

allowed to gently move the head from side Flexion and extension the history and physical examination. Due
to side. If this produces pain posteriorly in Lateral cervical spine radiographs have been to the physiological differences described
the neck the collar should be reapplied used to assess the spine for ligamentous above, a number of normal radiological find-
and the spine X-rayed. If there is no pain injury. The suggested indications have been ings in children are significantly different
on movement the collar and cervical spine symptomatic patients with normal plain X- from those in adults. The common findings
protection can be removed.2,1719 rays, or the unconscious patient with normal that cause concern are: pseudosubluxation
Young children represent a difficult sub- X-rays and/or CT scans. These images have of C2 on C3 (seen in up to 25% of children);
group. They are preverbal and cannot follow become popular in assessing adult patients exaggerated atlantodens distance (seen in
commands or communicate easily. If palpa- but investigations to date have consistently 20% of children under 8 years of age); and
tion of the posterior cervical spine does failed to show a benefit over other imaging, radiolucent synchondrosis between the
not cause distress the neck should be let with a significant number of studies limited odontoid and C2 (seen in all children under
free; if the child spontaneously moves the by inadequate motion in the acute setting.22 4 and in 50% of those under 10 years of
neck without discomfort the neck can be Most paediatric cervical-spine guidelines do age). Other normal findings that can be mis-
cleared. In this authors experience no young not call for the routine use of these views.23 interpreted include a variable anterior soft
child with a neck fracture will spontaneously tissue width altering with head positioning
move its neck without discomfort. Computerised tomography (CT) and crying the anterior ring apophyses of
CT of the cervical spine is common. There the vertebral bodies, and the anterior wed-
have been a number of indications for routine ging of the vertebral bodies (especially
Radiographic images CTsuggested, with the most widely accepted C3).17,28,29 All of these normal findings
Radiological evaluation is required for all being for further evaluation and elucidation can be mistaken for acute traumatic injuries
children who do not meet all the criteria of fractures identified, or to view areas not in children following trauma.
for clinical clearance of the cervical spine. seen adequately on the initial cervical spine Evaluation of the lateral cervical spine
As part of the secondary survey in major series.17 Other suggested indications are for radiograph begins with assessment of the
trauma patients the cross-table lateral cervi- the assessment of the unconscious patient four lines, corresponding to the anterior ver-
cal spine X-ray would have been performed. with normal initial radiographs, and for tebral bodies, the posterior vertebral bodies,
This is a guide to the presence of serious cer- patients having a CTof the brain. Proponents the spinolaminar line, and the tips of
vical spine trauma only and cannot be used of CT suggest scanning the entire spine in the spinous processes. All four of these
to exclude cervical spine injury.2,17 All both of these instances and clearing the lines should follow a smooth, even contour
patients who require radiological evaluation spine if scans are normal19 (or progressing to (Fig. 24.3.2). The articular facets should be
require a full cervical spine series. flexion-extension views).17,24 Another group parallel, the intervertebral disc spaces, at
The cervical spine series consists of a lat- suggests just scanning the upper cervical ver- the posterior margin of the vertebral bodies,
eral film, an anterior-posterior film and an tebrae in children under 8 as most injuries should be similar, and the distances between
odontoid view. Using these three views all occur in this region.25,26 There are no studies spinous processes should show no signifi-
abnormal cervical spines will be detected, that have systematically evaluated the role cant widening (fanning). Review of the soft
allowing further investigation to fully delin- of CT in the evaluation of paediatric cervical tissue shadow should show a retropharyn-
eate the individual injuries.2,5 All seven spine injuries. CT appears to be as good as geal space of not more than one half
vertebrae must be included in the lateral any other modality in identifying injuries the AP diameter of the vertebral body at
view, along with the cervicothoracic junc- but the radiation exposure of young children C2 and no wider than the full width of the
tion. If this is not visible gentle traction needs to be considered.23 vertebral body at C6. As mentioned, this
should be applied to the arms and the film may be difficult to interpret in the crying
repeated, or a swimmers view (transaxillary) child.1,3,17
should be obtained. Oblique views of the Magnetic resonance imaging (MRI) Assessment of these areas of possible
cervical spine may also be of assistance, MRI is the imaging method of choice for abnormality has been made easier by the
especially if the cervicothoracic junction is assessing ligamentous injuries and for spinal formulation of a series of normal measure-
difficult to visualise. Oblique views, however, cord injuries. MRI will visualise most liga- ments. For the atlantoaxial relationship
do not provide any more information regard- mentous injuries and all spinal cord injuries. (C1C2), a measurement of the distance
ing the likelihood of injury than the standard In many instances MRI will alter the specifics (on the lateral film) from the posterior bor-
three views.2,5,17 of surgical management in those who der of the anterior arch of C1 to the anterior
In young children, getting co-operation for require surgical stabilisation. MRI provides margin of the odontoid should be less than
the odontoid (open mouth) view is difficult. It prognostic information in children with spi- 5 mm in children under 8 years of age, and
has been shown that this view can be nal cord injury.17,27 3 mm in older children and adults. To
excluded in children under 5 years of age with assess the relationship of the basion of the
little likelihood of missing a fracture.20,21 Radiographic evaluation skull to the atlas, the most reliable measure-
Other specialised radiological investiga- Once the X-rays have been obtained, care ment in children is Harriss posterior axial
tions have been used to evaluate cervical needs to be taken to interpret the images line (Fig. 24.3.3), which should lie within
spine injuries: accurately and correlate the findings with 12 mm of the basion of the skull.

547
24.3 SPINAL INJURY

Predental space can be removed. If there is still significant

G pain or tenderness, a CT scan of the entire
F
E Posterior spine should be performed. If this is normal
C1 cervical line and the patient alert and co-operative,
immobilisation should be kept in place
C2 and flexion and extension views
performed 37 days later, once the muscle
C3 spasm has resolved. If the patient is
significantly unwell, or has multiple
C4 trauma, CT or MRI scanning should be
considered. Consultation with a paediatric
C5 orthopaedic surgeon or neurosurgeon is
recommended.19
C6

C7 Atlantoaxial rotary subluxation

D Fig. 24.3.4 Posterior cervical (Swischuk) line. Fixed rotary subluxation at the atlantoaxial
joint is more common during childhood than
C
B adulthood. It can present after minor trauma,
A likelihood of the subluxation being physio- in conjunction with an upper respiratory tract
logical (Fig. 24.3.4). The spinolaminar junc- infection, and often no inciting cause is
Fig. 24.3.2 Cervical spine lines. Lateral cervical
spine. (A) Anterior vertebral bodies; (B) posterior tion of C2 should lie within 2 mm of this found. The clinical picture is that of the head
vertebral bodies and anterior spinal canal; line. If the distance is greater than 2 mm a turned to one side and held in the cock-robin
(C) spinolamial line and posterior spinal canal; fracture or pathological subluxation is likely. position. The child is unable to turn its head
(D) spinous process tips C2-C7; (E) odontoid
past the midline, and attempts to move the
process of dens of C2; (F) anterior arch of C1;
(G) predental space between posterior surface Cervical spine clearance head often cause pain. The spasm of the ster-
of anterior arch of C1 and anterior surface of guidelines nocleidomastoid (SCM) muscle is on the side
odontoid process. Modified from Barkin et al to which the head is turned (ipsilateral side),
With the knowledge of mechanism of injury,
1994 Emergency Pediatrics, 4th edn. Mosby, as the muscle is trying to right the head. In
St. Louis, MO, USA. immobilisation needed, clinical examina-
tion, and radiographic interpretation, it is contrast, in a wry neck the SCM spasm is on
possible to devise guidelines (Fig. 24.3.5) the side opposite to which the head is turned
that allow safe and effective management (contralateral side), with the spasm causing
of the potentially injured cervical spine the head turning.1,15,17
while minimising the investigations needed. Plain radiographs may be diagnostic,
There is as yet no guideline that is well revealing the lateral mass of C1 rotated
Clivus established for young children.30,31 The anterior to the odontoid on the lateral view,
guideline should include the following or rotation of the spinous processes to the
points: ipsilateral side on the AP view. If clinical
examination and plain radiographs cannot
All patients with a significant mechanism confirm the diagnosis, CT imaging should
of injury, altered conscious state, or be considered.17
neurological symptoms, or who cannot be Rotary subluxation of short duration will
assessed should be immobilised. often spontaneously reduce; those that do
C3 If on assessment the patient is alert, not not or that have been present for a longer
drug affected, has no other major injuries, duration (days) may need traction or manipu-
has no posterior cervical tenderness, has lation to reduce. Post-reduction immobilisation
no posterior cervical pain on movement, is needed to maintain reduction and the
Fig. 24.3.3 Posterior axial line for and has no neurological signs, the duration of immobilisation should vary accord-
identification of occipito-axial dislocation. immobilisation can safely be removed ing to the duration of subluxation.1,17,32
and no further investigations are needed.
If these criteria are not all satisfied or
The subluxation of C2 on C3 and C3 on assessment is incomplete, immobilisation
Thoracic and lumbar spine
C4 consistently causes difficulty in inter- should be maintained and a three-view
injuries
pretation of paediatric cervical spine films. plain X-ray series performed.18,19
Swischuks line (posterior cervical line), If the X-rays are normal the patient should Fractures of the thoracic spine account for
which joins the spinolaminar line of C1 to be re-examined. If the patient has a 2530% of spine injury in children, while
that of C3, allows assessment of the normal examination, the immobilisation lumbar fractures account for 2025%.

548
24.3 SPINAL INJURY
24

ORTHOPAEDICS AND RHEUMATOLOGY

Unconscious or uncooperative or major distracting injury? A number of bony injuries are caused by
these mechanisms. Compression of the ver-
Yes No tebral body with anterior wedging is the
most common. However, a degree of anterior
Apply one-piece semi rigid collar Assess for wedging is normal in children. Burst frac-
Apply head immobiliser and straps neck pain (posterior) tures occur where there is disruption of the
if on spinal board Any abnormal neurological deficit
Consult neurosurgeon if any Undo collar, maintain head endplates and herniation of the disc into
neurological signs alignment, and assess for the vertebral body. Retropulsed fragments
posterior midline tenderness can cause spinal cord injury. The seat-belt
If above normal assess for fracture, associated with the lap-only seat
limitation in movement
(45 to right and left)
belt, is a hyperflexion and distraction injury
most commonly seen at the thoracolumbar
junction, and is frequently associated with
All normal
visceral or mesenteric injury. The posterior
bony or ligamentous elements are disrupted
Leave collar off and the fracture line extends horizontally
No X-ray
Document assessment in history
into the vertebral body or through the inter-
vertebral disc.1,4,7,33

Patient intubated Cervical spine series Patient cooperative Clinical assessment

No Normal
and having urgent AP, lateral odontoid and no major As with all trauma patients, assessment of
CT brain? view ( 5 yrs) distracting injury? the spine should take place in the secondary
survey after the airway, breathing and circu-
Yes Abnormal No Yes lation have been assessed and stabilised.
A history of mechanism of injury is impor-
tant in identifying the risk of thoracic or
lumbar spine injury. The presence of pain
CT cervical spine CT scan Reassess patient for: in the back makes injury more likely, but
Consult neuro or Area of local abnormality neurological signs
orthopaedic surgeon Area not well visualised Undo collar and assess for: absence of pain does not exclude injury. Clin-
Change to a two-piece Consult neuro or orthopaedic posterior midline tenderness ical examination should focus on tenderness
collar surgeon muscle spasm and signs of bruising or deformity over the
If needing immobilisation limitation in movement spine. This is assessed by log rolling the
for >6 hrs, change to a (45 to right and left)
two-piece collar patient while spinal immobilisation is still
in place. A search for signs of spinal cord
All or cauda equina lesions should also be made.
Any abnormal normal
Any patient who has pain or tenderness
over the spine should have the spine evalu-
Discuss with emergency, neurosurgical, Leave collar off ated by radiography. Patients without pain
or orthopaedic consultant Document assessment or tenderness who have altered conscious
Immobilise in a two-piece collar in history state or other significant injuries are at risk
of having thoracic or lumbar spine injuries
Fig. 24.3.5 Guide to management of the potentially injured cervical spine. missed (as they are for cervical spine inju-
ries).34 As multi-level injuries are common,
any child with a proven cervical spine frac-
Injuries to the thoracic spine and the thora- have associated small bowel and visceral ture or spinal cord injury should have the
columbar junction have a higher incidence injury in up to 50% of cases.1 Road traffic entire spine imaged with plain radiographs
of spinal cord injury, with neurological deficit accidents and falls account for most of the (see Fig. 24.3.5).
seen in up to 40% of cases.1,7 The high inci- injuries, but non-accidental injuries in these
dence of cord injury is related to the rela- regions do occur. Radiographic evaluation
tively large size of the spinal cord in the After clinical evaluation, radiographs should
thoracic region, the inherent stability of Mechanism of injury be taken on any child who is at risk of
the thoracic spine requiring larger forces Injuries to the thoracic and lumbar spine are having a thoracic or lumbar spine injury
to cause bony injury. Multiple level injuries flexion/extension or vertical compression (Table 24.3.4). The standard views for both
are seen in 3040% of children with tho- injuries; often a combination of these is pres- areas are the anterior-posterior radiograph
racic or lumbar spine fractures.7 Fractures ent, and also a degree of distraction is not and the lateral film. In the thoracic region
of the lower thoracic and upper lumbar spine uncommon. the radiographs need to be overexposed

549
24.3 SPINAL INJURY

spine injury exist and can help make the Spinal cord injury syndromes
Table 24.3.4 Indications for
thoracolumbar spine X-rays diagnosis. A paravertebral haematoma can A number of patterns of neurological deficit
usually be seen at the site of injury, blood are seen in patients with spinal cord injury,
Pain in the thoracic or lumbar region
Tenderness of the spine in the thoracic or in the pleural space may be seen as a pleural with the deficit dependant on the portion
lumbar region cap, and widening of the mediastinum of the spinal cord damaged. Neurogenic
Significant bruising or deformity of the spine
Altered conscious state may be present. Unfortunately these signs shock the manifestations of loss of sympa-
Proven fracture in another region of the spine cannot differentiate a thoracic spine injury thetic output to the cardiovascular system
from an aortic arch injury.7 is seen immediately after complete cord injury
at the level of T6 or above. This should not
be confused with spinal shock, which is the
compared to a normal chest X-ray to allow Management reversible dysfunction of the spinal cord asso-
adequate views of the spine. The shoulders Management must start with care of the ciated with injury. It is likened to a concussion
often obscure the upper thoracic spine and airway, breathing and circulation. Only once of the cord without permanent damage. It
a swimmers view may be needed to visualise these areas have been stabilised should may exist alone or in combination with perma-
the first two thoracic vertebrae. management of the spine proceed. However, nent cord injury. Its resolution is responsible
The films should be evaluated by follow- while the patient is being stabilised the spine for the improvement in neurological function
ing the anterior and posterior vertebral body should be maintained in alignment and the seen in the first few days post-injury.15,35
lines and the spinolaminar line on the lateral patient moved by log rolling. A thorough The distinction between a complete injury
view. These three lines should have a paral- assessment and investigation of the abdo- and a partial injury with preservation of
lel course. The height of each vertebral body men and chest is mandatory for all patients some motor or sensory function below the
should be assessed anteriorly and posteri- with significant thoracic and upper lumbar lesion is vital for prognosis. Many patients
orly, and a difference of more than about spine injuries, and injuries to the pelvis must with partial injury will regain much or all
3 mm treated as pathological. On the AP not be forgotten with lumbar spine injuries. of the neurological function. A partial cord
view the paraspinal lines should be closely As many of these injuries are associated with injury may occur at any region and with
inspected to detect evidence of para- intra-abdominal injuries an ileus is common any mechanism of injury.
spinal haematoma. The posterior elements and nasogastric or orogastric tube should Complete cord injury is usually seen in
should be visible through the vertebral body be inserted. Consultation with a paediatric injuries of the thoracic spine and thoraco-
and should be in alignment. Each vertebra orthopaedic surgeon or neurosurgeon should lumbar junction. The spinal cord is large in
should be inspected; an apparently empty be sought for the definitive care of the injury. relation to the size of the spinal canal at this
or invisible vertebral body indicates a frac- Surgical stabilisation is usually required level. Complete cord injuries that remain at
ture dislocation with distraction.1,33 for unstable fractures and those fractures 24 hours rarely regain any significant func-
There are a number of features on the associated with neurological injury.4,7,33 tion. Cauda equina lesions injuries at or
radiographs that indicate an unstable frac- below L2 involve the peripheral nerves
ture: vertebral body collapse with widening rather than the spinal cord and can show sig-
of the pedicles; greater than 33% compro- nificant recovery of lower limb and sphincter
mise of the spinal canal by retropulsed function even weeks after the injury.15,35
fragments of the lamina, pedicles or body;
Spinal cord injury
Central cord syndrome is usually seen in
translocation of more than 2.5 mm between The goal of management of injury to the spi- hyperextension injury resulting in herniation
vertebral bodies in any direction; bilateral nal cord or cauda equina is to minimise the of the intervertebral disc into the spinal cord.
facet joint dislocation; or greater than resulting neurological deficit. This commen- The resulting injury causes a motor deficit that
50% anterior compression of the vertebral ces with adequate immobilisation of the is greater in the arms than legs and most
body associated with widening of the inter- spine and complete and thorough investiga- extensive in the small muscles of the hand.
spinous space.33 tion to detail the anatomy of the injury.35 The sensory deficit is variable. BrownSequard
While most thoracic and lumbar spine Spinal cord injury should be suspected in syndrome hemisection of the cord causes a
fractures are diagnosed on the initial plain any child who has multisystem trauma, contralateral loss of pain and temperature
radiographs, these films often do not provide minor trauma associated with spinal pain, sensation and an ipsilateral motor paralysis
enough information on the extent of the sensory or motor symptoms, and any patient and loss of proprioception below the level of
injury and a CT scan of the region is usually with altered consciousness. The patient injury. Approximately two-thirds of those with
obtained to elucidate the full extent of the must be adequately immobilised, such as central cord syndrome and one-third with
injury. MRI will be needed to visualise all lig- on a spinal board with a cervical collar and BrownSequard syndrome will recover.15,35
amentous and spinal cord involvement. head immobiliser (see section on spinal
Some fractures in the thoracic region can immobilization, p. 545), and any assessment
be difficult to see on plain films, especially if of the back or patient movement accom- Clinical assessment
there were technical difficulties in obtaining plished by log rolling. Up to 50% of patients Initial steps in the management of a patient
the films in multiply-injured patients. with spinal cord injury will have at least with a suspected spinal cord lesion are the
A number of secondary signs of thoracic moderate head injury.35 assessment and resuscitation of the airway,

550
24.3 SPINAL INJURY
24

ORTHOPAEDICS AND RHEUMATOLOGY

breathing and circulation. The most immedi- intraspinal problems in patients with a partial compromise that may occur in the ensuing
ate threats to life and spinal cord function of neurological deficit. The appearance of the weeks, the psychosexual issues that accom-
patients with spinal cord injury remain hyp- spinal cord on MRI also allows prediction of pany spinal cord injury, the urological
oxia and hypotension. Spinal cord lesions in neurological outcome. Cord transection and problems, and the potential for skin
the upper cervical spine may impair respira- major haemorrhage have a poor outcome, breakdown, that are exaggerated in these
tory function and require early intubation minor haemorrhage and oedema have a mod- patients.
and mechanical ventilation. The unstable erate to good outcome, and a normal MRI is
cervical spine must be maintained in align- associated with complete recovery.1
ment without traction during treatment of
SCIWORA
the airway. The loss of sympathetic vasomo-
tor tone after cervical spinal cord injury will Treatment Spinal cord injury without radiographic
result in vasodilatation, venodilatation, and Most of the treatment available for spinal abnormality (SCIWORA) is defined as
reduced venous return to the heart causing cord injuries is supportive. The breathing objective signs of myelopathy as a result
hypotension. There should be an associated and circulation must be supported as of trauma with no evidence of fracture or
relative bradycardia for age and existing needed. As there will be a neurogenic blad- ligamentous instability on plain X-rays or
blood pressure, which will help distinguish der, catheterisation is necessary, and a naso- tomography.37 SCIWORA is most frequently
this response from haemorrhagic shock.35 gastric tube is needed to treat the gastric seen in younger children (especially
Initial fluid resuscitation with 1020 mL and bowel stasis that ensues. For transport, <8 years of age), and in injuries of the
kg1 should adequately replace the relative antiemetic is useful to prevent vomiting and cervical spine. Postulated causes include
hypovolaemia. However, if hypotension per- spine movement or airway compromise. Sub- ligamentous laxity and bony immaturity
sists, measurement of central venous pressure cutaneous low-molecular-weight heparin allowing excessive, transient movement
(CVP) may be needed to guide fluid replace- should be instigated once the patient is sta- during trauma, causing distraction or com-
ment. Excessive fluid replacement that pushes ble to prevent deep venous thrombosis.36 pression of the spinal cord, or cord ischaemia
central venous and pulmonary artery pressures Specific treatment of the spinal cord lesion due to vascular injury or hypoperfusion.
above the normal range will result in pulmo- is controversial. Four substances have been The incidence reported in children is
nary oedema. Treatment with a vasoconstric- studied in prospective, randomised trials 110% of all spinal cord injuries.1,15,38,39
tor, such as metaraminol, may be useful for methylprednisolone, tirilazad, naloxone and Younger children tend to have more pro-
the patient who has adequate CVP and GM-1 ganglioside. All studies to date have found neurological injury, and hence less
remains hypotensive. In patients who are also excluded children under 13 years of age. Tirila- long-term improvement.38,39 A number of
significantly bradycardic, inotropic agents, zad and naloxone have failed to show any children will present with minor neurological
such as dopamine or adrenaline (epinephrine), benefit in trials to date. There is conflicting evi- injury and progress to complete or partial
may be useful. Patients requiring more than dence regarding the benefits of methylpred- spinal cord injury. The incidence of this
40 mL kg1 of fluid replacement and having nisolone and documented evidence that its delayed presentation of the serious symp-
a low CVP must be assumed to have other use increases the risk of bacterial infection. It toms is 550%. The delay to presentation
injuries causing blood loss. is recommended that it be used with knowl- of full symptoms has been as long as 4
Examination of the neurological impair- edge of the risks and possible benefits.15,36 days.1,3 Because of these presentations, all
ment is done as part of the secondary survey. The current regimens for use are seen in children with history of neurological symp-
A thorough examination of the motor func- Table 24.3.5. GM-1 ganglioside has yet to be toms or any neurological deficit should be
tion of the limbs and assessment of reflexes shown to offer significant benefit in spinal cord treated as patients with potential spinal
should be performed and a level of sensory injury, and is not recommended for routine use. cord injuries.
deficit sought. Knowledge of the derma- Once the patient has been stabilised and After the primary survey, resuscitation and
tomes and myotomes will allow determina- investigated, transfer to a spinal cord injury secondary survey and radiographic evalua-
tion of the level of neurological injury. unit should be expedited. These units tion, any patient with any neurological
and associated intensive-care units are deficit should remain immobilised until all
Radiographic evaluation geared to manage the cardiorespiratory bony, ligamentous and spinal cord injury is
In all patients with suspected spinal cord
injury the spine should be X-rayed. The radio-
graphs should include the entire spine, as Table 24.3.5 Methylprednisolone administration in spinal cord injury
multiple levels of injury are common. Once Time after injury
the patient is stabilised, further investigation
of the lesion should follow. The bony injuries 03 hours 38 hours >8 hours

should be investigated as discussed above. Initial IV dose 30 mg kg1 (over 15 30 mg kg1 (over 15 Not recommended
minutes) minutes)
Investigation of the cord itself will require
MRI. MRI should be performed as soon as pos- Maintenance IV dose 5.4 mg kg1 hr1 5.4 mg kg1 hr1
sible after identification of a spinal cord injury, Duration 24 hours 48 hours
as it will allow identification of remedial

551
24.3 SPINAL INJURY

excluded or treated. Further investigation 3. Dormans JP. Evaluation of children with suspected 22. Insko E, Gracias V, Gupta R, et al. Utility of flexion and
cervical spine injury. Instr Course Lect 2002;51:40110. extension radiographs of the cervical spine in the acute
with a CT scan focused at the level of symp- 4. Reynolds R. Pediatric spinal injury. Curr Opin Pediatr evaluation of blunt trauma. J Trauma 2002;53:4269.
toms and MRI to view the cord should be per- 2000;12(1):6771. 23. Rana AR, Drongowski R, Breckner G, Ehrlich PF.
5. Jaffe DM, Binns H, Radkowski MA, et al. Developing a Traumatic cervical spine injuries: characteristics of
formed. MRI provides the same prognostic clinical algorithm for early management of cervical spine missed injuries. J Pediatr Surg 2009;44(1):1515;
information in SCIWORA injuries as in other injury in child trauma victims. Ann Emerg Med 1987;16 discussion 55.
(3):2706. 24. Berne JD, Velmahos GC, El-Tawil Q, et al. Value of
spinal cord injuries.15 6. Platzer P, Jaindl M, Thalhammer G, et al. Cervical spine complete cervical helical computed tomographic
injuries in pediatric patients. J Trauma-Injury Infect Crit scanning in identifying cervical spine injury in the
Care 2007;62(2):38996; discussion 946. unevaluable blunt trauma patient with multiple injuries:
7. Brandser EA, el-Khoury GY. Thoracic and lumbar spine a prospective study. J Trauma-Injury Infect Crit Care
Controversies trauma. Radiol Clin North Am 1997;35(3):53357. 1999;47(5):896902; discussion 02-3.
8. Jaffe DM. Evaluation of children for cervical spine injuries. 25. Browne GJ, Cocks AJ, McCaskill ME. Current trends in the
The clinical assessment of potential In: Strange GR, editor. Paediatric emergency medicine: a management of major paediatric trauma. Emerg Med
comprehensive study guide. 2nd ed. New York: McGraw- (Fremantle) 2001;13(4):41825.
spinal injuries in the preverbal child can Hill; 2002. 26. Hutchings L, Atijosan O, Burgess C, Willett K. Developing
be very difficult. 9. Woodward G. Neck trauma. In: Fleisher G, Ludwig S, a spinal clearance protocol for unconscious pediatric
editors. Textbook of Paediatric Emergency Medicine. trauma patients. J Trauma-Injury Infect Crit Care
There is debate about how to 4th ed. Philadelphia: Lippincott Williams & Wilkins; 2000.
10. Schafermeyer RW, Ribbeck BM, Gaskins J, et al.
2009;67(4):6816.
27. Holmes J, Mirvis S, Panacek E, et al. Variability in
adequately immobilise the cervical spine Respiratory effects of spinal immobilisation in children. computed tomography and magnetic resonance imaging
of a child less than 2 years of age. Ann Emerg Med 1991;20:10179. in patients with cervical spine injuries. J Trauma
11. Herzenberg JE, Hensinger RN, Dedrick DK, Phillips WA. 2002;53:52430.
 The use of flexion and extension Emergency transport and positioning of young children
who have an injury of the cervical spine. The standard
28. Swischuk L. The cervical spine in childhood. Curr Probl
Diagn Radiol 1984;13:126.
films in the acute situation is backboard may be hazardous. J Bone Joint Surg Am 29. Kriss VM, Kriss TC. imaging of the cervical spine in infants.
1989;71(1):1522. Pediatr Emerg Care 1996;13(1):449.
controversial. 12. Treloar DJ, Nypaver M. Angulation of the pediatric 30. Ehrlich PF, Wee C, Drongowski R, Rana AR. Canadian

cervical spine with and without cervical collar. Pediatr C-spine Rule and the National Emergency X-Radiography
The role of routine CT of the cervical Emerg Care 1997;13(1):58. Utilization Low-Risk Criteria for C-spine radiography
spine in major trauma has been 13. Curran C, Dietrich AM, Bowman MJ, et al. Pediatric in young trauma patients. J Pediatr Surg 2009;
cervical-spine immobilization: achieving neutral 44(5):98791.
suggested, but needs to balanced position? J Trauma 1995;39(4):72932. 31. Garton HJ, Hammer MR. Detection of pediatric cervical
against the radiation exposure to 14. Anonymous. Cervical spine immobilization before spine injury. Neurosurgery 2008;62(3):7008; discussion
admission to the hospital. Neurosurgery 2002;50(Suppl. 00-8.
children. 5):S717. 32. Subach BR, McLaughlin MR, Albright AL, Pollack IF.
15. Mathison DJ, Kadom N, Krug SE. Spinal cord injury in Current management of pediatric atlantoaxial rotatory
The timing of removal of spinal the pediatric patient. Clin Ped Emerg Med subluxation. Spine 1998;23(20):21749.
2008;9:10623. 33. Clark P, Letts M. Trauma to the thoracic and
immobilisation in the unconscious child 16. Quigley S, Curley M. Skin integrity in the pediatric lumbar spine in the adolescent. Can J Surg 2001;
remains controversial. population: preventing and managing pressure ulcers. 44(5):33745.
JSPN 1996;1(1):718. 34. Meek S. Lesson of the week: fractures of the
The role of methylprednisolone in 17. Anonymous. Management of pediatric cervical spine and
spinal cord injuries. Neurosurgery 2002;50(Suppl. 3):
thoracolumbar spine in major trauma patients.
[comment]. Br Med J 1998;317(7170):14423.
spinal cord injury remains unproven S8599. 35. Chiles BW, Cooper PR. Acute spinal injury. N Engl J Med
despite one large study suggesting 18. Hoffman JR, Mower JR, Wolfson AB, et al. Validity of a set 1996;334(8):51420.
of clinical criteria to rule out injury to the cervical spine in 36. Anonymous. Pharmacological therapy after acute
benefit. patients with blunt trauma. N Engl J Med cervical spinal cord injury. Neurosurgery 2002;
2000;343:949. 50(Suppl. 3):S6372.
19. Hutchings L, Willett K. Cervical spine clearance in 37. Pang D, Pollack I. Spinal cord injury without radiographic
pediatric trauma: a review of current literature. J Trauma- abnormality in children - The SCIWORA syndrome.
Injury Infect Crit Care 2009;67(4):68791. J Trauma 1989;29(5):65464.
References 20. Buhs C, Cullen M, Klein M, Farmer D. The pediatric 38. Pang D, Wilberger J. Spinal cord injury without
1. Roche C, Carty H. Spinal trauma in children. Pediatr trauma C-spine: is the odontoid view necessary? J Pediatr radiographic abnormalities in children. J. Neurosurg.
Radiol 2001;31(10):677700. Surg 2000;35(6):9947. 1982;57:11429.
2. Viccellio P, Simon H, Pressman BD, et al. A prospective 21. Swischuk LE, John SD, Hendrick EP. Is the open-mouth 39. Anonymous. Spinal cord injury without radiographic
multicentre study of cervical spine injury in children. odontoid view necesary in children under 5 years? Pediatr abnormality. Neurosurgery 2002;50(Suppl. 3):
Paediatrics 2001;108(2):e20. Radiol 2000;30:1869. S1004.

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 24

ORTHOPAEDICS AND RHEUMATOLOGY

24.4 Risk management in acute paediatric
orthopaedics
Robyn Brady

comments about pain, swelling or loss

ESSENTIALS of function documented in more than
60% of cases, which had not been
1 Inadequate identification and management of unstable or displaced injuries such acted upon. In 20% of cases the doctor
as lateral condylar elbow fractures, Monteggia fracture dislocations, and slipped
was alerted to the possible problem by
upper femoral epiphysis (SUFE), can cause serious long-term deformity and
the patient or his/her relatives.
malfunction.
Fractures about the joint are at
2 Immobilisation complications, from loss of position to skin ulceration or disproportionately high risk both for
compartment syndromes, demand scrupulous departmental risk management being missed and for adverse events.
protocols, as they often relate to weakest link situations. Although most series of fractures missed
in the ED include a large array of
3 The importance of adequate initial clinical evaluation and follow up of patient or different sites (45 fractures at 15 sites
clinician concerns cannot be over-stressed.
and 69 fractures at 27 sites5 in two
4 A variety of departmental risk-management strategies provide a safety net for series not exclusively paediatric), knee
doctor and patient. injuries (tibial spine and plateau) were
disproportionately represented in the
former (nine fractures compared with
that 12% of these reports have a discrepant
Introduction
concordance significant enough to alter man-
Table 24.4.1 Adverse events in acute
Emergency medicine is an area of medical agement26 although in many series there paediatric orthopaedics
practice with a high rate of litigation, and were no long-term adverse events detailed.
orthopaedic conditions (especially missed Missed displaced fracture/dislocations
Fractures can also be missed because of pri-
Elbow
fractures) make up a high proportion of mary assessment failure (failure of thorough Supra-condylar fractures
successful claims. Many factors intrinsic to history or examination, therefore failure to Lateral condyle fractures
Monteggia fracture dislocations
emergency medicine contribute to this X-ray). X-rays may be inadequate in coverage Medial epicondylar joint entrapment
danger, including high staff turnover, or resolution, the abnormality may be missed Knee
Tibial spine
24-hour practice with multiple hand-overs, by the ED physician(s), or by radiologists; this Phalangeal fractures with angulation
wide-ranging procedural demands, and too tends not to show in reported series, Hip
Slipped upper femoral epiphysis (SUFE)
workload flux. which presume radiology reports to be the Open injuries
Just as many of the problems are systemic, gold standard, and there may be breakdown Fracture complications, e.g. compartment
syndrome, neurovascular compromise
so too are many of the solutions. Adverse out- between radiologist reporting and ED High-risk patients
comes can occur when the clinical problems physician notification and recall of patient. Multitrauma, multiple disability
discussed in this section are not recognised All such oversights have been specifically
Missed undisplaced fractures
or dealt with adequately, and there are clear associated with adverse outcomes7 and the Buckle fractures
emergency department (ED) systemic inter- physician responsibility for follow up and Proximal humerus
ventions that can minimise their occurrence. Radial neck
communication, which extends to ensuring Distal radius and ulna
that a booked follow up does actually occur, Distal femur
Toddlers fracture (tibial stress fracture)
must not be overlooked.
Adverse events in paediatric Common or serious acute paediatric Delayed identification of
acute orthopaedics orthopaedic adverse events are shown in Septic arthritis
Table 24.4.1. Osteomyelitis
Non-identification or delayed Leukaemia/other neoplastic infiltration
Regarding missed displaced fractures and Congenital serious orthopaedic disorder, e.g.
identification CDH, spinal problems
dislocations, the following points should be
Missed fractures are commonly associated
considered: Procedural problems
with ED litigation, accounting for approxi-
mately half of ED negligence claims in the Anatomical distortions should be

POP-related injuries
Loss of position post-manipulation
UK and Australia.1 Articles auditing the clinically apparent to patient and Mal-union
Adverse events in association with
correlation of ED doctor X-ray analysis with physician. In fact, in a series of 80 injuries procedural sedation
radiologist reporting in recent years show with delayed identification,5 there were

553
24.4 RISK MANAGEMENT IN ACUTE PAEDIATRIC ORTHOPAEDICS

only one missed scaphoid fracture), and Procedure-related problems Plaster of Paris (POP) immobilisation
elbow injuries were disproportionately Problems relating to ED interventions fall results in many complications: too soft; too
represented in paediatric adverse into a separate category, and increase pro- tight; too cylindrical; inadequately immobi-
events. However, in all the significant portionally with the number and types of pro- lising; excessively immobilised; etc. At the
paediatric elbow injuries listed as cedural activity undertaken in a department, serious end of the spectrum is the possibility
potentially missed (unstable which varies widely in Australia, particularly of compartment syndrome. More frequent
supracondylar fracture, lateral condyle for the paediatric population. Strategies to adverse outcomes include skin loss, due to
fracture, Monteggia fracture-dislocation minimise these problems are now so well internally protruding plaster shelves, or
and a trapped medial epicondyle, e.g. described in the risk-management literature incompetent plaster saw use. Departments
after elbow dislocation), elbow that any department planning to change performing manipulations must be
examination was grossly abnormal, procedural practice would do well to follow equipped and staffed to monitor their
including an effusion and a limitation of these guidelines (Table 24.4.2). patients for anaesthetic adverse events,
range of motion. Although the and must balance the need to avoid a tight
radiological Monteggia fracture- POP with the need to maintain reduction
dislocation has often been missed by an Table 24.4.2 Risk management through appropriately positioned moulding.
strategies: acute paediatric orthopaedics
untrained X-ray viewer, the clinical The restriction of paediatric ED orthopaedic
1. Training
elbow abnormality should not be. A lack Credentialing of staff for individual
manipulations to experienced medical per-
of clinical joint integrity (effusion, procedures, e.g. plastering, sedation sonnel, and next day follow up, may mini-
Independent learning multimedia
asymmetry cf. the other side, reduced programs, e.g. for plastering, fracture
mise problems.
range of movement) is an indication for identification
Orthopaedic/fracture management
early orthopaedic review even if the tutorials as core resident/registrar training
X-ray appears normal. 2. Departmental information milieu
 Two special categories of high-risk Textbooks, e.g. Keats, McRae, Gill,
Swischuck, Rockwood
References
patients deserve mention. The first On-line point-of-care clinical 1. Gwynne A, Barber P, Taverner F. A review of 105
management guides, e.g. PEMsoft, MD negligence claims against accident and emergency
is multitrauma patients,7,8 due to consult, etc. departments. J Accid Emerg Med 1997;14:2435.
several factors including urgency of POP how-to wall charts and templates 2. Klein EJ, Koenig M, et al. Discordant radiograph
Paediatric fracture identification photo- interpretation between emergency physicians and
other clinical problems, the possibility of chart radiologists in a paediatric emergency department. Pediatr
an altered or distracted conscious state, 3. Computer-based CQI strategies Emerg Care 1999;4:2458.
Computer-generated links to discharge 3. Walsh-Kelly CM, Hennes HM, Melzer-Lange MD. False-
and hand-over problems. The second advice, e.g. wound care, POP positive preliminary radiograph interpretations in a
group of note is children with complex management, post-sedation advice pediatric emergency department: Clinical and economic
Automated prompt-sheets for particular impact. Am J Emerg Med 1997;15(4):3546.
medical conditions including autistic presentations, e.g. injury under 2, 4. Simon HK, Khan NS, Nordenberg DF, Wright JA. Paediatric
spectrum disorders, due to difficulties multitrauma emergency physician interpretation of plain radiographs:
Automated communications, e.g. faxed Is routine review by a radiologist necessary and cost-
in patient communication, occasional feedback to registered LMO effective? Ann Emerg Med 1996;27(3):2958.
pre-existing anatomical abnormalities, 4. Procedural policies, flow-sheets, and 5. Kremli MK. Missed musculoskeletal injuries in a university
credentialing for hospital in Riyadh: Types of missed injuries and responsible
varying pain thresholds, and a need Procedural sedation factors. Injury 1996;27(7):5036.
to establish base-line normal. Both Fracture/dislocation reduction 6. Cameron MG. Missed fractures in the emergency
5. Departmental audits department. Emerg Med 1994;6(1):379.
of these categories of patient require X-ray results 7. Alpers A. Key legal principles for hospitalists. Disease
senior medical involvement, careful Procedural sedation audit Monitor 2002;48(4):197206.
Fracture reduction feedback system 8. Connors JM, Ruddy RM, McCall J, et al. Delayed diagnosis
and often repeated systematic POP quality audit/feedback system in paediatric blunt trauma. Pediatr Emerg Care 2001;
assessment and documentation. 17(1):14.

554
 25

SECTION
MALE GENITALIA
Section editor Gary Browne

25.1 Male genitalia 555

25.1 Male genitalia

Colin S. Kikiros

may increase to an alarming degree in the

ESSENTIALS following days and appearances can then
be similar to those of testicular torsion.
1 A paediatric surgeon or urologist should be contacted to assess an acute scrotum The scrotal swelling is often due to a small
so as not to miss a testicular torsion.
secondary hydrocele. The testis is usually
2 The testicles must always be examined when boys present with abdominal normally aligned and in normal position in
pain. the scrotum. Tenderness is maximal at the
upper pole of the testis (where the append-
3 Testicular torsion is a condition requiring urgent surgery. age is located) and a blue dot may be seen
4 Colour Doppler ultrasound examination and nuclear scans of the testicle may be through the skin at the upper pole consis-
helpful, but should not be relied upon, in the diagnosis of testicular torsion. When in tent with an infarcted appendage.
doubt, exploration of the testicle is by far the safest treatment.

haematuria are present. A radionuclide scan Torsion of the testis

THE ACUTE SCROTUM may assist in differentiating this condition This is an acute emergency and is due, in
from torsion of the testicle. most situations, to medial rotation of the
spermatic cord. However, in one-third of
Introduction
cases the testis rotates in a lateral direc-
The acute scrotum is defined as a painful tion.2 The torsion usually occurs spontane-
Torsion of a testicular or
and/or enlarged scrotum and may be acute ously. However, sometimes it can follow
or subacute in onset. The origin of the
epididymal appendage direct trauma to the testis. The pain is likely
pathology may be from the testis, the groin Appendages of the testis and epididymis to be acute and severe and may be asso-
or the scrotal skin. In a younger child, the occur in 90% of testicles. Torsion of these ciated with nausea or vomiting. The older
pain will cause the child to be unsettled with appendages is the most common cause of child is often reluctant to ambulate. Often
crying, and it may be intractable. In an older testicular pain. The most common age of there is a history of previous short-lived pain
child, the reluctance to ambulate may be a presentation is at the onset of puberty and in a testis consistent with intermittent epi-
predominant feature. In addition, referred this is thought to be due to the release of sodes of spontaneously resolving torsion.
pain from a sore testis may present as oestrogens and androgens from the male The testis is usually enlarged and in a high
abdominal pain. Therefore, in patients pre- adrenal gland stimulating the appendages position in the scrotum or even in the groin.
senting with abdominal (especially lower- and causing them to enlarge. As the appen- It is not usually in its normal lie and there is
abdominal) pain one must examine the dages are on a narrow stalk they have a ten- much redness and swelling of the scrotal
inguinal scrotal region so as not to overlook dency to twist.1 Oedema of the appendages skin. Usually a secondary hydrocele is pres-
torsion of a testicle. may also occur following trauma to the tes- ent. The contralateral testicle may lie in a
The diffuse vasculitis of HenochSchon- tis. Onset of the pain is usually gradual and bell-clapper fashion owing to the insertion
lein purpura may affect the testis and/or the child is often able to ambulate without of the epididymis in the central part of the
scrotum. Usually other manifestations of difficulty. Redness and swelling of the scro- testicle and this in turn predisposes the
the condition such as a skin rash or tum are also mild in the first 24 hours but testicle to undergo torsion. The child should

555
25.1 MALE GENITALIA

be taken to the operating theatre urgently, perineal regions. The testis is not swollen Occasionally, however, intercurrent illnesses,
even if the child is not adequately fasted, and is normally aligned and the tenderness such as gastroenteritis and upper respiratory
as prolonged obstruction of the testicular arises from the palpably thickened scrotal tract infections, may result in an increase in
vessels may lead to partial or complete atro- wall. Occasionally, eosinophilia may occur volume of the peritoneal fluid. This leads to
phy of the testicle. Therefore nuclear scans and, characteristically, ultrasound examina- an increase in the amount of fluid around
and colour Doppler ultrasound should be tion shows marked thickening of the scrotal the testis and the patient may present to
avoided if the diagnosis is clear, as these wall, increased peri-testicular blood flow and the ED with a large scrotal swelling. The tes-
investigations may result in unnecessary a mild reactive hydrocele.10 The condition tis in this situation is not painful and one can
delay and they are not reliable. False nega- resolves in 14 days and no treatment, determine that the swelling does not extend
tives and false positives have been reported apart from pain relief, is required. into the groin. Ultrasound examination is
with both modalities. Colour Doppler ultraso- useful in confirming the diagnosis and
nography may be misleading as intratesticu- unnecessary surgery can be avoided.
lar flow may be seen even in testicles that Testicular tumours
have undergone torsion.3 Visualisation of a
twist in the cord is more reliable.4 Survival Primary or secondary (leukaemia, for exam- ACUTE PROBLEMS OF
of the testicle will depend on the number ple) tumours may cause the testicle to
THE PENIS AND
of twists that the spermatic cord has under- enlarge and become painful. The onset is
usually gradual and the pain more chronic.
FORESKIN
gone, along with the length of time that
the cord has been twisted, with the prognosis However, tumours may present acutely
being excellent for those undergoing surgery when they have been subjected to trauma.
The testicle may become extremely large,
ESSENTIALS
within 6 hours of the onset of symptoms.5,6
although the scrotal skin is not usually ery- 1 Urinary retention may arise from
thematous. Ultrasound and full blood pic- phimosis or balanitis and requires
ture may aid in the diagnosis. Paediatric urgent urinary diversion or
Epididymo-orchitis surgeons and oncologists are predominantly circumcision.
required in managing these patients.11
Infection or inflammation may affect the 2 Priapism is an acute emergency
epididymis or the testis. Infections in the epi- and requires urgent treatment.
didymis may arise from retrograde flow
along the vas deferens or lymphatics from Irreducible inguinal hernia
urinary tract infections, or from the blood-
Segments of intestine may on occasions Introduction
stream. Inflammation of the testis may arise
descend into a hernia sac in the scrotum
from conditions such as mumps. The epidid- Currently, approximately 90% of young
and become irreducible. Acute pain may
ymis is tender and swollen and the testis also males in Australia are not circumcised. This
be felt in the scrotum and also in the groin
may be tender. The testis is of normal lie and compares to a generation ago, when the
(see Chapter 7.11 on herniae).
in a normal position in the scrotum. A raised majority of males were circumcised, often
interleukin-6 level may be clinically helpful in the neonatal period. As a result, problems
in assisting the diagnosis of epididymitis.7 with the foreskin are increasing and patients
Once the diagnosis has been confirmed Rupture of the testis
are often referred to the emergency depart-
the childs urine should be sent for analysis This is usually the result of trauma. ment for treatment.
and he should then be commenced on anti- The testis becomes enlarged and painful Parents are often unsure of the correct
biotics. Enteric organisms are the usual and there is associated bruising and fre- management of the foreskin. In most cases
cause of the urinary tract infection.8 Once quently an associated hydrocele. Ultrasound the foreskin should be left alone until the
the condition has resolved renal ultrasound examination often reveals irregularity of the age of 5 or 6 and then should be retracted
and micturating cystourethrogram should testicular outline and intratesticular haema- gently to clean the under surface of the fore-
be performed as the urine infection may toma.12 The patient should be referred to skin and the glans. If the foreskin cannot be
have resulted from an abnormality in the uri- a surgeon for exploration and repair of the retracted easily, the use of a mild steroid
nary tract, such as posterior urethral valves.9 testicle. ointment for a short period may correct
the phimosis. If this is not successful, circum-
cision may be required.
Idiopathic scrotal oedema Acute hydrocele
The cause of this condition is unknown. The A patent processus vaginalus may allow
Phimosis of the foreskin
child presents with scrotal discomfort, intraperitoneal fluid to flow into the space
oedema and erythema of one side of the around the testicle in the scrotum. Often In this condition the foreskin cannot be
scrotum, which may spread to affect the the amount of fluid is minimal and the easily retracted and in the more severe
entire scrotum, the penis and inguinal and patient does not present acutely. cases the outflow of urine is significantly

556
25.1 MALE GENITALIA
25

MALE GENITALIA
obstructed. Ballooning of the foreskin may position. Oedema of the foreskin distal to permanent inability to have erections
occur with micturition when the urine flow the tight ring can develop and the foreskin and/or penile fibrosis.26
into the foreskin space is greater than the can become painfully swollen and difficult
flow exiting out of the foreskin. Urinary tract to reduce.
infections,13 dysuria and possibly urinary The foreskin is more easily reduced References
retention may develop.14 the sooner that the patient is treated.19 1. Samnakay N, Cohen RJ, Orford J, et al. Androgen and
A sample of urine and swabs from the The patient should be given appropriate oestrogen receptor status of the human appendix testis.
Pediatr Surg Int 2003;19:5204.
foreskin should be obtained and the patient analgesia and gentle digital pressure should 2. Sessions AE, Rabinowitz R, Hulbert WC, et al. Testicular
should be commenced on intravenous or be used on the foreskin through saline- torsion: Direction, degree, duration and disinformation.
J Urol 2003;169:6635.
oral antibiotics. In cases of urinary retention, soaked gauze. Once some of the oedema 3. Nussbaum Blask AR, Bulas D, Shalaby-Rana E, et al. Color
urinary diversion may be required. For exam- has been dispersed the foreskin should Doppler sonography and scintigraphy of the testis: A
prospective, comparative analysis in children with acute
ple, a suprapubic catheter may need to be be gently replaced in its normal position. scrotal pain. Pediatr Emerg Care 2002;18:6771.
inserted under general anaesthesia. Alterna- If this is not possible the child will need to 4. Arce JD, Cortes M, Vargas JC. Sonographic diagnosis
of acute spermatic cord torsion. Rotation of the cord:
tively, urgent circumcision can treat the be seen by a paediatric surgeon, who may A key to the diagnosis. Pediatr Radiol 2002;32:48591.
condition.14 attempt the same procedure. If this again 5. Dunne PJ, OLoughlin BS. Testicular torsion: Time is the
enemy. Aust N Z J Surg 2000;70:4412 [comment].
In mild cases of phimosis, the application fails, the foreskin should be reduced under 6. Rampaul MS, Hosking SW. Testicular torsion: Most delay
of half-strength BetnovateW ointment to the a general anaesthetic. Some surgeons occurs outside the hospital. Ann R Coll Surg Engl
1998;80:16972.
tip of the foreskin for 6 weeks has a reported favour circumcision whilst the child is anaes- 7. Rivers KK, Rivers EP, Stricker HJ, et al. The clinical utility of
success of 7588%.14,15 thetised so as to avoid another anaesthetic serologic markers in the evaluation of the acute scrotum.
Acad Emerg Med 2000;7:106972.
in the future. 8. McAndrew HF, Pemberton R, Kikiros CS, Gollow I. The
Alternative methods described to incidence and investigation of acute scrotal problems in
children. Pediatr Surg Int 2002;18:4357.
reduce the paraphimosis are the use of
Balanitis hyaluronidase20 and puncture technique21
9. Ng JW, Chan AY, Kong CK, Wong MK. Posterior urethral
valves presenting as acute epididymo-orchitis: A case
report and follow-up study. Aust N Z J of Surg 1996;66:
This condition usually arises when phimosis whereby the oedematous foreskin is punc- 12930.
of the foreskin is present and infection tured in several places with an 18-gauge 10. Klin B, Lotan G, Efrati Y, et al. Acute idiopathic scrotal
edema in children revisited. J Pediatr Surg 2002;37:
has occurred in the space under the fore- hypodermic needle to evacuate the oedema 12002.
skin. Bacteria, often enteral or cutaneous so as to allow for easy reduction of the 11. Nichols CR. Testicular cancer. Curr Probl Cancer
1998;22:187274.
in origin, migrate into this space and foreskin. 12. Micallef M, Ahmad I, Ramesh N, et al. Ultrasound
as they cannot be washed away, infection features of blunt testicular injury. Injury 2001;32:236.
13. Hiraoka M, Tsukahara H, Ohshima Y, Mayumi M. Meatus
results. This is frequently a subacute or tightly covered by the prepuce is associated with
chronic condition, but on occasions can urinary tract infection. Pediatr Int 2002;44:65862.
14. Ashfield JE, Nickel KR, Siemens DR, et al. Treatment of
be acute.
Priapism phimosis with topical steroids in 194 children. J Urol
Again a sample of urine and swabs of the 2003;169:11068.
15. Kikiros CS, Beasley SW, Woodward AA. The response of
foreskin should be obtained and the patient This is an acute emergency. Low-flow pria- phimosis to local steroid application. Pediatr Surg Int
should be commenced on IV or oral antibio- pism is due to obstruction of venous outflow 1993;8:32933.
16. Escala JM, Rickwood AM. Balanitis. Br J Urol 1989;63:
tics. The condition usually subsides within a from the penis. It may occur spontaneously 1967.
few days. However, in the long term, circum- or it can be secondary to medication, sickle 17. Webster TM, Leonard MP. Topical steroid therapy for
phimosis. Can J Urol 2002;9:14925.
cision may be required.16 cell disease or leukaemia. Uncontrolled arte- 18. Meuli M, Briner J, Hanimann B, Sacher P. Lichen sclerosus
Balanitis xerotica obliterans, also called rial inflow, usually caused by direct trauma et atrophicus causing phimosis in boys: A prospective
study with 5 year followup after complete circumcision.
lichen sclerosus et atrophicus, is thought to the penis or perineum, results in high-flow J Urol 1994;152:9879.
to be an autoimmune condition affecting priapism. Both types of priapism result in a 19. Choe JM. Paraphimosis: Current treatment options.
Am Fam Physician 2000;62:26238.
the foreskin and glans. It leads to irreversible painful, sustained erection. Urgent referral 20. Devries CR, Miller AK, Packer MG. Reduction of
phimosis of the foreskin and presents with to a paediatric surgeon or urologist is paraphimosis with hyaluronidase. Urology 1997;48:
4645.
inability to retract the foreskin and varying required for immediate treatment or surgery. 21. Fuenfer MM, Najmaldin A. Emergency reduction of
degrees of urinary obstruction. It is best trea- In the meantime, perineal compression paraphimosis. Eur J Pediatr Surg 1994;4:3701.
22. Hatzichristou D, Salpiggidis G, Hatzimouratidis K, et al.
ted with circumcision, as studies using topi- should be attempted as it may successfully Management strategy for arterial priapism: Therapeutic
cal steroid treatment have failed to show reverse high-flow priapism.22 If this fails, col- dilemmas. J Urol 2002;168:20747.
23. Volkmer BG, Nesslauer T, Kraemer SC, et al. Prepubertal
any permanent improvement.17,18 our Doppler ultrasonography of the corpora high flow priapism: Incidence, diagnosis and treatment.
cavernosa can reveal a blood leak. Bilateral J Urol 2001;166:101822.
24. Shankar KR, Babar S, Rowlands P, Jones MO.
internal pudendal arteriography and embo- Posttraumatic high-flow priapism: Treatment with
lisation can then follow.23,24 Intracavernous selective embolisation. Pediatr Surg Int 2000;16:4546.
Paraphimosis 25. Gbadoe AD, Atakouma Y, Kusiaku K, Assimadi JK.
injections of etilefrine can be effective in Management of sickle cell priapism with etilefrine. Arch
On occasions the foreskin may be tight but reducing priapism in children with acute Dis Child 2001;85:523.
26. El-Bahnasawy MS, Dawood A, Farouk A. Low-flow
the child, or parent, may have attempted sickle-cell crisis.25 Failure to resolve the priapism: Risk factors for erectile dysfunction. BJU Int
to retract it and not returned it to its normal condition in a timely manner may result in 2002;89:28590.

557
SECTION

26 ACUTE NEONATAL
PROBLEMS
Section editor Gary Browne

26.1 Acute neonatal emergencies 558

26.1 Acute neonatal emergencies

Paul Craven Elly Marillier

ESSENTIALS NEONATAL
1 Early recognition, appropriate treatment and referral for definitive care are key to EMERGENCIES
a good outcome in the sick neonate. Recognising the signs of respiratory distress in
Studies have indicated that shorter post-
newborns and infants allows for appropriate acute management in the ED.
partum hospital stays have resulted in an
2 Ensuring appropriate resuscitation equipment is available for the newborn infant, increased attendance at the emergency
with a knowledge of drug doses, is essential to appropriately manage acute department (ED) of newborn infants and
cardiorespiratory arrest. those in the first month of life. The common
presenting symptoms and signs were jaun-
3 In the crying neonate, determine whether this presentation is part of a recurrent
dice, poor feeding, breathing difficulties
stereotypical pattern in an otherwise well infant, or a single acute episode. A careful
and irritability.
history and examination will often lead to an appropriate diagnosis. Screening tests,
The underlying common pathologies
with the exception of urine culture, have little utility. Review carefully the carers
were found to be physiological jaundice,
coping skills and supports and organise appropriate follow up.
feeding problems and suspected sepsis.
4 Recognition of peripheral versus central cyanosis in a newborn infant is essential as Maternal experience, social support, early
central cyanosis requires urgent evaluation. Respiratory and cardiac causes of cyanosis postnatal discharge and perinatal instruc-
must be differentiated. Despite the majority of causes being cardiorespiratory it is tion influenced presentation to the ED.
important to recognise the other subtle causes of cyanosis that can affect this age group.

5 Neonatal seizures are relatively common and generally reactive in nature, and thus
should not be labelled as neonatal epilepsy. Their presence is often a sign of
neurological dysfunction and this should be fully investigated. The majority of The infant with breathing
neonatal seizures occur in the early neonatal period (day 17 of life). Neonatal difficulty
seizures are often subtle; they may be missed, and are sometimes hard to differentiate
Respiratory emergencies are some of the
from more benign movement disorders.
commonest conditions presenting in the
6 Persistent vomiting, if found in the neonate, requires full investigation. Bilious neonatal period. The increased work of
vomiting in the neonate is a surgical emergency until proven otherwise. breathing is manifested as:
7 Sepsis remains the most common cause for the collapsed neonate in the tachypnoea (RR >60 breaths per
emergency department. minute);
8 In the collapsed newborn consider sepsis first then look for clues of other rarer intercostal and subcostal recession
(excessive use of accessory muscles of
underlying disorders if there is a poor clinical response to treatment.
respiration);
9 The key to resuscitation of the newborn is adequate ventilation. flaring of the alae nares (accessory
muscles of respiration);

558
26.1 ACUTE NEONATAL EMERGENCIES
26

ACUTE NEONATAL PROBLEMS

grunting (forced glottic closure to create laryngomalacia, vocal cord anomalies and (CT) and magnetic resonance imaging (MRI),
positive end expiratory pressure); tracheomalacia. There are many rare causes. are useful in specific clinical situations, based
cyanosis (>5 g dL1 of desaturated on the possible differential diagnosis. Other
haemoglobin); Clinical features diagnostic techniques employed include
apnoea (a pause in breathing of Stridor is the classic presenting sign of upper barium swallow and laryngobroncho-
>20 seconds or a pause of less than airway obstruction and is a rare phenome- oesophagoscopy (LBO).
20 seconds associated with bradycardia); non in the neonatal period. Stridor is an indi-
tracheal tug. cation of partial obstruction of the large Management
diameter airways, from either an intrinsic The management of upper airway obstruc-
The causes of respiratory distress are varied
developmental defect or from secondary tion requires the expertise of an ear, nose
and are summarised in Table 26.1.1. They
external compression and distortion. Stridor and throat specialist, and can range from
can be broadly divided into primary respira-
is the inspiratory noise that indicates this conservative management, in laryngomala-
tory and non-respiratory causes. Primary
partial obstruction and early referral to an cia, ensuring adequate growth and develop-
respiratory pathology is a direct result of
ear, nose and throat specialist should be con- ment, to the requirement for a tracheotomy
upper, lower or mixed airway pathology.
sidered. In addition to the stridor, infants in bilateral vocal cord paralysis.
This section will discuss:
often have an associated degree of respira-
emergencies of the upper airway; tory distress, but may have a normal or
emergencies of the lung parenchyma; hyper-expanded chest radiograph.
non-pulmonary causes affecting the lung Respiratory distress
parenchyma; Investigations attributed to lung
the at-risk neonate. These will be guided by the history and exam- parenchyma pathology
ination findings. Infants with severe stridor
Causes
and respiratory distress should be urgently
Respiratory distress secondary to lower
transported to a tertiary care centre, where
Upper airway obstruction respiratory tract involvement may be a sig-
intensive-care support can be continued by
nificant feature of either congenital or
Causes qualified personnel. Airway support may
acquired parenchymal lung disease.
Of all cases of upper airway obstruction involve precise positioning, continuous posi-
Acquired conditions likely to present in the
presenting in the neonatal period, 60% tive airways pressure or intubation, which
ED include pneumonia, either viral or bacte-
are congenital and 40% are acquired. can prove an extremely difficult procedure
rial. The commonest viral presentation is
Causes of upper airway obstruction include in this setting. Imaging studies, including
bronchiolitis secondary to respiratory syncy-
choanal atresia, micrognathia, macroglossia, plain radiographs, computerised tomography
tial virus. Other parenchymal diseases
include pulmonary oedema, due to either
an underlying pulmonary or cardiac anomaly.
Table 26.1.1 General causes of respiratory distress Associated with underlying parenchymal
anomalies the risks of pneumothorax are
General causes of respiratory distress Specific conditions
increased, once again exacerbating the
Respiratory disorders Hyaline membrane disease respiratory distress.
Congenital pneumonia
Meconium aspiration syndrome Congenital anomalies that may present
Transient tachypnoea of the newborn include cystic malformations of the lung, lobar
Pneumothorax
Hydro/haemothorax emphysema or pleural effusions secondary
to an underlying lymphatic pathology.
Upper airway abnormalities Laryngomalacia
Micrognathia The causes of respiratory failure may
Vocal cord anomalies be divided into two forms, common and
Cardiac anomalies Heart failure uncommon:
Myocarditis
Pericardial effusion
Cyanotic congenital heart conditions
Common
Structural abnormalities Diaphragmatic hernia Respiratory distress syndrome (RDS),
Congenital cystic lesions
Diaphragmatic paralysis
transient tachypnoea of the newborn
(TTN).
Chest deformities Arthrogryposis
Thoracic dystrophy Meconium aspiration (MAS).
Pneumonia/sepsis.
Haematological causes Anaemia
Drug-induced respiratory depression.
CNS lesions Infection Bronchiolitis.
Metabolic conditions Metabolic acidosis Air-leak syndrome (pneumothorax).
Congestive heart failure.

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26.1 ACUTE NEONATAL EMERGENCIES

Uncommon commonly viral, with respiratory syncytial Examination

Diaphragmatic hernia. virus, adenovirus and parainfluenza virus Infants with disease affecting the lung
Congenital airway abnormalities, such all commonly identified. A history of parenchyma, either primary respiratory or
as choanal atresia. affected family members gives some indica- cardiac, generally present with the classical
Pulmonary hypoplasia. tion of this potential. examination findings of respiratory distress,
Cystic adenomatoid malformation. Any infant with underlying lung pathol- notably: recession of the intercostal and sub-
Pulmonary haemorrhage. ogy is at risk of air leak, and any sudden costal spaces; nasal flaring; tachypnoea
decompensation in an infant with respira- (>60 bpm); and expiratory grunting.
tory distress should lead one to consider Nasal flaring is a result of the alae nasi
Clinical features this diagnosis. In addition, air leak can be being the first muscles to be activated dur-
History a spontaneous phenomenon with no identi- ing inspiration and they aim to decrease air-
In respiratory distress attributed to paren- fied cause. way resistance. The recession of the inter-
chymal involvement, a clinical history is Non-infectious causes of acquired respira- and subcostal spaces is a result of the com-
essential to elucidate potential predisposing tory distress include any conditions in which pliance of the chest wall being reduced in
factors. Of the causes of parenchymal there is an abnormally high or low blood flow neonates. During inspiration the pleural
disease presenting to the ED, pneumonia to the lungs, an increased demand for oxygen, pressure is reduced, but in neonates with
and extra-pulmonary cardiac failure are or a decreased number of red blood cells. parenchymal lung disease this needs to be
the two most common causes. These can The commonest non-pulmonary cause reduced more than normal and thus the con-
often be differentiated by clinical history. of respiratory distress seen in the ED is that sequences are that the compliant chest wall
Bacterial pneumonia in the first few hours of pulmonary oedema, secondary to con- may cave in as a result of these more nega-
of life may be impossible to distinguish from genital heart disease. tive pressures. The recession in conjunction
respiratory distress syndrome or transient Congenital heart disease is one of the com- with the abdominal protuberance asso-
tachypnoea of the newborn. Therefore, respi- monest malformations, with an incidence of ciated with diaphragmatic descent give
ratory distress in newborns generally should 0.6%. Although 3060% of congenital the characteristic seesaw pattern of neona-
be treated as bacterial pneumonia until heart disease is identified antenatally, this tal respiratory distress.
proven otherwise. When associated with still leaves a large percentage presenting in Infants with respiratory infections may
chorioamnionitis, it is caused most com- the postnatal period. Predischarge satura- present in a very similar manner to those
monly by Group B streptococci (GBS) or tion monitoring of all newborn infants has with cardiac anomalies. Specific findings
by Escherichia coli. However, Haemophilus the possibility of increasing the early identi- for infants with pneumonia may be the pres-
influenzae, Streptococcus pneumoniae (pneu- fication of congenital heart disease, prior to ence of fever or temperature instability, feed
mococcus), Group D streptococci, Listeria the onset of respiratory distress, cyanosis or intolerance and rhinorrhoea.
and anaerobes have also been described as collapse. Cyanotic lesions generally present If there is a suggestion of pulmonary air
pathogens in this setting. Infants infected early but neonates with ductal-dependent leak, the clinical signs are specific. There will
with these organisms are often preterm systemic circulations are often well in the be reduced air entry on the side of the leak,
and have very early onset of respiratory early neonatal period and collapse around with a reduction in chest movement on that
distress. Of note, infants may also develop day 4 of life. Closure of the ductus, with asso- side. Percussion, although seldom used in
bacterial pneumonia transnatally in the ciated systemic collapse, is one of the com- newborn infants, should be hyper-resonant
absence of maternal chorioamnionitis. Here, monest presentations to the ED with severe and if the air leak is under tension there
the causative organism is likely to be GBS, respiratory distress, secondary to associated may be associated displacement of the tra-
and the onset of symptoms tends to occur pulmonary oedema. chea and apex beat to the contralateral side.
1224 hours after birth. Factors in the history pointing towards If a primary respiratory cause cannot be
Neonatal pneumonia can be either con- congenital heart disease being the cause of identified, non-respiratory causes should
genital or acquired. Congenital pneumonia collapse include a family history, syndromic be sought. Signs indicative of congenital
commences before birth and the most com- malformations and associated abnormalities. heart disease may include weak femoral
mon infecting organisms include Group B In addition to predisposing factors, a his- pulses, an active praecordial impulse, hepa-
Streptococcus and E. coli. Despite the major- tory of poor feeding often predates the tomegaly and a cardiac murmur.
ity of infants being unwell at birth, some collapse. The classic cardiac lesions presenting with
infants do acquire these infections after Other causes of respiratory distress pre- respiratory distress in the neonatal period
birth and present with similar signs and senting in the ED are: include:
symptoms of respiratory distress, poor feed-
ing, fever and apnoea. The clinical history inborn errors of metabolism, with Left to right shunting lesions
should focus on the maternal Group B associated acidosis, a family history, (atrioventriculoseptal defects (AVSD)
Streptococcus carriage in pregnancy, length consanguinity or an abnormal smell and ventriculoseptal defects (VSD)).
of rupture of membranes, maternal antibi- noted from the infant; Typically, large VSDs present between
otic therapy during labour and maternal central nervous system anomalies; weeks 2 and 4 of life after the
fever. Acquired neonatal pneumonias are non-accidental injury. pulmonary pressures have reduced and

560
26.1 ACUTE NEONATAL EMERGENCIES
26

ACUTE NEONATAL PROBLEMS

this allows increased left to right flow interstitial changes. Chest radiographs of outlined in the resuscitation guidelines
with resultant pulmonary oedema. The infants who have bacterial pneumonia may devised by the International Liaison Com-
infant will present with increasing exhibit a diffuse reticular nodular appear- mittee on Resuscitation (ILCOR). Maintain-
tachypnoea, recession and poor feeding, ance but, in contrast to respiratory distress ing a neutral airway position and ensuring
in association with a loud cardiac syndrome, they tend to show normal or the airway is free of obstruction allows opti-
murmur and crepitations audible in increased lung volumes with possible focal mal oxygen to be delivered. Saturations
the chest. or coarse densities. There may also be pleural should be maintained with either nasal can-
Duct-dependent obstructive left ventricle effusions, particularly with GBS pneumonia. In nulae oxygen, head box oxygen, continuous
conditions (hypoplastic left heart, critical the newborn who has bacterial pneumonia, positive airways pressure or endotracheal
aortic stenosis and coarctation of the blood cultures obtained before the initiation intubation and ventilation. If working in a
aorta). Once the ductus arteriosus closes of antibiotics commonly grow the offending hospital with a Neonatal Intensive Care Unit
around week 1 of life, the systemic organism. Cultures of urine and cerebrospinal or Paediatric Intensive Care Unit, support
circulation is no longer maintained. fluid should be obtained at the time of the from personnel working in these areas can
Infants present shocked, pale and with blood culture if a newborn infant is systemi- be invaluable in maintaining a patent airway.
severe respiratory distress. Specifically cally unwell. If the diagnosis is viral, mucus If sepsis is suspected then intravenous
they have weak femoral pulses and plugging, with over-aeration and hyper- antibiotics should be commenced, to cover
invariably a large liver. expansion may be characteristic. Although both Gram-positive and Gram-negative
tension pneumothorax should be a clinical bacteria and an evaluation made of the need
With other causes of respiratory distress
diagnosis, the plain radiograph is good at for both fluid and inotropic support. Empiric
there may be features consistent with spe-
demonstrating small pneumothoraces. An treatment should be initiated as soon as
cific diagnoses. Metabolic conditions are
anterior pneumothorax may be subtle and possible with ampicillin 100 mg kg1 day1
often associated with hepatosplenomegaly,
easily missed by the unwary as appearing divided every 12 hours (infants <1.2 kg) or
coma, hypoglycaemia and jaundice. Central
more lucent on the side of the pneumothorax every 8 hours (infants >1.2 kg) and cefo-
nervous system (CNS) lesions may have sei-
in the absence of a lateral air meniscus. taxime 100 mg kg1 day1 divided every
zures associated.
Laboratory investigations, including full 12 hours or 150 mg kg1 day1 divided every
blood count, C-reactive protein (CRP) and 8 hours (infants >1.2 kg and >7 days old).
Investigations blood cultures, are useful adjuncts to the Gentamicin is an alternative treatment,
Any infant in the first month of life with respi- diagnosis. The white cell count may show particularly when there is no evidence of
ratory distress should be observed and moni- a leucocytosis with associated left shift or, meningitis. Treatment should be continued
tored closely. This includes pulse rate, oxygen more commonly, may show a consumptive for at least 10 days if sepsis is present,
saturation, respiratory rate, temperature, picture with neutropenia and associated although 1421 days may be required,
blood pressure and capillary refill time. thrombocytopenia in the septic infant. The particularly for Gram-negative infections.
The normal heart rate for a neonate in the CRP is another non-specific marker of infec- Viral infections are treated conservatively
first month of life is 120160 bpm. Some tion, but appears more useful in monitoring by respiratory and circulatory support as
newborn infants, however, have a resting response to treatment of infection rather required. An unusual or unresponsive neo-
heart rate below 90 bpm. Respiratory dis- than in its diagnosis. natal presentation of pneumonia warrants
tress is generally associated with respiratory If a viral respiratory tract infection is sus- further evaluation. The maternal history
rate greater than 60 breaths per minute. pected then a nasopharyngeal sample may offer important clues. Neonatal pneu-
Fever as a sign of sepsis is variable. viewed with electron microscopy for respira- monia involving cytomegalovirus (CMV) or
Plain radiographs of the chest are useful, tory viruses may reveal the common causes other viruses may be transmitted transpla-
but do not generally differentiate the various of bronchiolitis. centally. CMV pneumonia may not require
causes of respiratory distress. Sepsis and car- Arterial blood gas analysis or indirect treatment in the otherwise healthy infant.
diac failure both demonstrate increased transcutaneous monitoring may reveal arte- However, neonatal respiratory distress in
interstitial markings. In cardiac failure, fluid rial hypoxaemia and hypercarbia. The degree the setting of perinatal exposure to herpes
more specifically radiates into the intersti- of hypoxaemia and acidosis will be a guide to simplex virus, particularly if there is primary
tium from the hilum and in severe cases the need for respiratory positive pressure sup- maternal genital infection, warrants treat-
may be associated with an effusion. Again, port. In addition to diagnosing the severity of ment with aciclovir 30 mg kg1 day1
in cardiac disease the size of the cardiac the respiratory acidosis, an arterial blood gas divided every 8 hours for 1421 days until
shadow may be increased. A cardiac silhou- may also reveal a metabolic acidosis, making all cultures are negative. Ureaplasma urealy-
ette greater than 60% of the transthoracic inborn errors of metabolism a potential differ- ticum is another important organism and
diameter is indicative of cardiac disease ential diagnosis of the respiratory distress. treatment of Ureaplasma infections in the
and needs to be investigated further, by newborn should include erythromycin
means of an electrocardiogram and echocar- Management 50 mg kg1 day1 divided every 6 hours.
diogram, and referral to a cardiologist. A neonate with respiratory distress needs to If the cause of the respiratory distress
The chest radiograph of an infant with be observed closely. Evaluation of airway, is believed to be cardiac then once again
pneumonia may show lobar or diffuse breathing and circulation are imperative, as supporting the airway, breathing and

561
26.1 ACUTE NEONATAL EMERGENCIES

circulation is imperative. Added caution Primary pulmonary hypertension of the neurological depression may be hypotonic,
with fluid resuscitation should be conside- newborn. have abnormal autonomic responses and
red so as not to exacerbate the cardiac Central nervous system disease: have poor feeding with associated failure
failure. Use of prostaglandin E1 allows  Intracranial haemorrhage to thrive and may indeed have seizure
reopening of the ductus and increased  Maternal sedative administration activity.
systemic circulation. This is a temporising  Meningitis. Once again, if seizures are suspected then
measure prior to the definitive surgery the Methaemoglobinaemia. a detailed family history may indicate a
infant may require. Hypoglycaemia. genetic syndrome as the cause of the sei-
Management of a pneumothorax requires Sepsis. zures. A thorough history of the pregnancy
either acute drainage with needle thoraco- Cold. and delivery is also important to rule out
centesis, in the presence of a tension pneu- other causes of seizures, and early checking
mothorax, or intercostal catheter insertion for hypoglycaemia is essential. Considering
followed by appropriate underwater drain- Clinical features neonatal sepsis is always imperative and a
age. A repeat chest X-ray (CXR) to ensure History detailed history of maternal substance
adequate lung expansion is required prior Once cyanosis has been diagnosed in an abuse may indicate drug withdrawal as the
to the removal of the chest drain. infant presenting to the ED, the most impor- cause of the seizures.
tant step is to differentiate between pulmo-
nary and cardiac causes of cyanosis.
Pulmonary causes of cyanosis include Examination
The blue infant pneumonia, both bacterial and viral, pneu- Once a detailed history has been taken to
Neonatal cyanosis is a result of deoxygen- mothorax, pleural effusions and airway elicit possible causes of cyanosis then the
ated blood in the systemic circulation. It is anomalies. If the cause of the cyanosis is felt infant should be examined carefully.
defined as an arterial saturation less than to be pulmonary, the infant will generally Infants with respiratory causes for cyano-
90%. have a history of worsening respiratory dis- sis will generally have signs of distress,
History, examination and simple investi- tress that interferes with the infants ability namely tachypnoea, recession of the inter-
gations available in the ED should be able to feed successfully. The history may be costal and subcostal spaces, tracheal tug,
to distinguish the cause of the cyanosis indicative of an infective cause, with rhinor- nasal flaring and expiratory grunting. As
and this will predict the management of this rhoea, fever, cough, poor feeding and wors- to the precise respiratory cause of this dis-
condition. ening recession of the inter- and subcostal tress, septic infants often have an associated
spaces. In addition, there may be a history tachycardia and may have temperature
of other affected family members and respi- instability. They may have poor capillary
Causes
ratory infections are generally more promi- return and may also have associated
Babies can be peripherally blue or centrally
nent in the winter months especially apnoeas, a pause in breathing of greater
blue and this is important in differentiating
associated with epidemics of bronchiolitis. than 20 seconds or a pause in breathing less
the cause of the cyanosis. Peripheral cyanosis,
Infants with cardiac causes of cyanosis than 20 seconds but associated with brady-
affecting the hands and feet, known as acro-
may have no preceding history and gener- cardia. Infants who are septic and cyanosed
cyanosis is a normal phenomenon that gen-
ally breathe normally. A detailed antenatal may have localised respiratory infections,
erally clears within 12 days and needs no
history, including family history, genetic such as viral bronchiolitis, or may be
treatment. Central cyanosis, evidenced by
abnormalities and the results of antenatal septicaemic.
cyanosis of the gums and mucous membranes
ultrasound scans, will be important. In addi- A cyanosed infant with little or no respira-
is generally pathological and needs urgent
tion, the timing of the cyanosis may also tory distress is more likely to have congenital
evaluation if presenting in the ED. Babies
give a clue to the diagnosis, with duct- heart disease. The commonest lesions pre-
can be centrally cyanosed for many reasons
dependent cardiac lesions generally worsen- senting with cyanosis in the neonatal period
including heart disease, lung or other breath-
ing when the ductus arteriosus shuts around are: transposition of the great arteries; total
ing problems, being cold, or having seizures.
day 3 or 4 of life. Although congenital heart anomalous pulmonary venous return; pul-
Not all children who turn blue have a heart
disease is generally divided into cyanotic monary atresia with an intact ventricular
problem. A thorough history, examination
and acyanotic, those lesions generally classi- septum; severe pulmonary stenosis; and
and simple investigations will be able to dif-
fied as acyanotic but duct dependent can severe tetralogy of Fallot.
ferentiate these causes of central cyanosis.
present with severe respiratory distress Examination of a cyanosed infant with
and a degree of cyanosis. suspected cardiac disease might reveal dys-
Non-respiratory causes of cyanosis In addition to congenital heart disease morphic features suggesting a syndromic
Cardiac defects: and pulmonary causes, an infant may pres- association with congenital heart disease.
 Decreased pulmonary blood flow ent cyanosed because they have neurologi- Auscultation of the lung fields and praecor-
 Admixture lesions cal depression or seizures. A history of the dium may reveal evidence of pulmonary
 Congestive heart failure (pulmonary infants general activity, tone and feeding oedema and murmurs, and an abdominal
congestion). patterns will be helpful. Infants with examination may reveal hepatomegaly.

562
26.1 ACUTE NEONATAL EMERGENCIES
26

ACUTE NEONATAL PROBLEMS

To differentiate an infant with non- in the diagnosis of neonatal cyanosis include for 510 minutes. If the oxygen tension
cardiopulmonary causes for the cyanosis, a full blood count to reveal polycythaemia rises to exceed 150 mmHg, cyanotic con-
the examination would need to concentrate and a raised or depressed white blood cell genital heart disease is unlikely, although
on the neurological system of the infant. count, blood cultures and a nasopharyngeal little or no change in oxygen tension
Tone, movements and reflexes will all give aspirate. strongly suggests that such a cardiac defect
important information as to the neurological In addition to blood gas analysis to indi- is the cause of the cyanosis. The gold stan-
status. Examination for dysmorphic features cate fixed or variable hypoxia, the carbon dard for immediately ruling out non-cardiac
and examination of the fundi and skin may dioxide will generally be raised in respiratory causes of cyanosis and establishing the
add important information as to the cause pathologies contributing to the cyanosis but diagnosis of cyanotic congenital heart dis-
of the seizures. In the neonatal period, may be normal in cardiac disease. ease remains echocardiography. The cyano-
important causes of seizures would include An electrocardiogram (ECG) may help dif- sis that results from pulmonary disease
hypoxia, hypoglycaemia and biochemical ferentiate cardiac disease, but the gold stan- usually resolves partially, if not completely,
anomalies, narcotic withdrawal and struc- dard investigation to help differentiate following the administration of oxygen.
tural brain abnormalities. cardiac causes of cyanosis from non-cardiac Such treatment should not produce similar
causes will be an echocardiogram combined results in patients who have non-respiratory
Investigations with colour Doppler flow mapping and cyanosis. Here the cause must be deter-
To differentiate the various causes of cyano- pulsed-wave Doppler studies. The echocar- mined to achieve a successful outcome.
sis in the neonatal period a full set of obser- diogram should be performed as a matter All neonates should initially receive
vations should be performed. Oxygen of urgency in such situations to allow oxygen until a definitive diagnosis has been
saturations should be performed initially in appropriate management of congenital made. If the diagnosis is pulmonary then
room air to serve as a baseline. Subsequent heart disease. Echocardiography can be the addition of oxygen should resolve the
oxygen measures should be performed easily performed by a paediatric cardiologist cyanosis. If the respiratory distress is severe,
in 100% oxygen, achieved by means of a in the ED or the infant may need to be trans- additional support by means of positive end
headbox sealed over the babys head and ferred to a specialist centre to achieve such expiratory pressure or positive pressure ven-
neck. The so-called hyperoxia test may help an investigation. tilation may be required. If an infant requires
to differentiate cyanotic heart disease from If a neurological cause is suspected for the intubation then a person skilled in this pro-
other causes of neonatal cyanosis. Infants cyanosis, urgent investigation including cedure should be asked to attend the ED.
with neurological or pulmonary causes of blood sugar level monitoring is imperative. Following institution of airway support the
the cyanosis will demonstrate substantial Additional biochemical investigations may infant requires intensive-care admission.
increases in the arterial blood saturation, include calcium, magnesium and sodium as Once the airway and breathing have been
while infants with cyanotic congenital well as specific metabolic investigations, supported then appropriate assessment
heart disease will show minimal elevation. including a urine metabolic screen and and management of the circulation is essen-
As well as oxygen saturation changes, the newborn screening test if inborn errors of tial. In any infant with a pulmonary cause for
difference should be confirmed with arterial metabolism are suspected to be causing the cyanosis, the potential for sepsis should
blood gas analysis of arterial oxygen partial neurological depression. If structural lesions be evaluated and treated with intravenous
pressures. A partial pressure <100 mmHg in or intra-cerebral haemorrhage is suspected, antibiotics.
100% inspired oxygen is more indicative of ultrasound is an easy bedside test to per- The laboratory evaluation of most neo-
cardiac disease. form, but magnetic resonance imaging is nates who are cyanosed includes a haema-
Once arterial oxygen saturations and the gold standard for CNS investigation. tocrit and haemoglobin determination,
blood gas analysis have been performed Additional investigations may include elec- white blood cell count, differential count,
the infant should have a chest radiograph. troencephalogram (EEG) and urine/meco- blood glucose determination, and a chest
Abnormalities of the lung fields may suggest nium drug screening. radiograph. If a cardiac aetiology is likely,
a primary pulmonary cause for the cyanosis echocardiography is essential. In selected
or a cardiac cause if the changes are sugges- Management cases, cardiac catheterisation and angio-
tive of pulmonary oedema with increased Any neonate that presents with cyanosis cardiography may be necessary to define
vascular markings. should be appropriately resuscitated as out- the cardiac anatomy more precisely. Ele-
Assessing the cardiothoracic diameter lined in the guidelines devised by ILCOR. ctrocardiography should be performed if
and shape of the cardiac shadow may give Once the presence of cyanosis is deter- clinical findings suggest a tachyarrhythmia.
some clues to a cardiac cause of the cyano- mined from the physical examination of If a CNS aetiology is suspected, appro-
sis. A typical boot-shaped heart is classically the infant, its degree of severity should be priate scans and drug levels should be
described in tetralogy of Fallot and an egg documented immediately by oximetry and considered. The presence of methae-
on a string appearance is described in trans- confirmed by arterial blood gas and pH moglobin may be detected by placing a
position of the great arteries. Generally dif- determinations. Arterial blood gases and few drops of the patients blood on filter
ferentiating the cardiac causes of cyanosis pH should be determined with the patient paper and comparing it with normal blood.
is difficult and requires an echocardiogram. breathing room air (if clinically stable) and Methaemoglobin will produce a chocolate-
Additional investigations that may be useful following the breathing of 100% oxygen brown colour.

563
26.1 ACUTE NEONATAL EMERGENCIES

Seizures that result in cyanosis should delivery should be established as well as a

be managed according to the results of ini- The infant with possible detailed family history of seizures.
tial investigations. Hypoglycaemia should seizures A perinatal history of maternal fever,
be managed with a 5 mL kg1 bolus of prolonged rupture of membranes, low vagi-
Although the incidence of seizures is higher
10% dextrose, followed by an infusion of nal swab positive for Group B Streptococcus
in the first 4 weeks of life than in any other
dextrose. Hypocalcaemia should be man- and associated fetal distress may be clues
age group, the actual incidence cannot be
aged with intravenous calcium and other to an infective cause of the seizures. In addi-
delineated because of the large number of
electrolyte imbalances should be corrected tion, a history of poor Apgar scores and poor
subtle presentations. The seizures that do
before trying to control the seizure. Once cord blood gas results, and the need for
present to the ED generally do so in the early
treatable causes of neonatal seizures resuscitation at birth may indicate a perina-
neonatal period (days 17) and it is essen-
have been identified and treated then tal asphyxial event.
tial to recognise these for two reasons:
appropriate anticonvulsant therapy should If a perinatal cause for the seizures is not
be administered. Phenobarbital or phenyt- They may represent the manifestation identified in the history then a postpartum
oin are first-line anticonvulsants used in of serious underlying disease that needs cause needs to be considered. A history of
the neonatal period. treating. maternal substance abuse may indicate
In any infant with seizures in the first If unrecognised, and thus untreated, seizures secondary to neonatal abstinence
month of life, sepsis should be thoroughly seizures may be prolonged and possibly syndrome and a family history of seizures
investigated with both blood culture and cause brain damage. is suggestive of a genetic cause for the
cerebrospinal fluid (CSF) cultures for both seizures.
Seizures in the neonatal period are a result
bacteria and viruses. Treatment with anti- Poor understanding of the nutritional
of an excessive depolarisation of neurones
biotics and antiviral medications should be requirements of the newborn and excessive
from many different causes. Disturbances
considered if there are any risk factors, symp- weight loss may indicate an electrolyte imbal-
of electrolytes (sodium, calcium, and magne-
toms or signs of sepsis. ance as the cause of the seizures. Hypoglycae-
sium) and an imbalance of excitatory and
Of all of the above aetiologies, the most mia and hypocalcaemia may both present
inhibitory amino acids have been identified
common cause of cyanosis in the neonate with a preceding history of jitteriness.
as predisposing to neonatal seizure activity.
is a cyanotic congenital heart defect. In In addition to the history of the actual
It is important to identify the cause of the
many such infants, pulmonary blood flow cause of the seizures it is also important to
seizures, as many of them are easily treat-
depends primarily or entirely upon the get a realistic picture as to the nature of
able, but if missed there may be major
patency of the ductus arteriosus. If congeni- the seizures. Although most neonatal sei-
long-term consequences.
tal heart disease is suspected from the zures are partial, they can be generalised,
hyperoxia test, chest radiograph, ECG or and there are many other presenting signs
echocardiogram then referral should be Causes of neonatal seizures of neonatal seizure activity including:
made to a paediatric cardiologist. A discus- Hypoxia: hypoxic ischaemic jitteriness; cyanosis; apnoea; tachycardia;
sion should take place as to whether prosta- encephalopathy. lethargy; and collapse.
glandin E1 should be commenced to either Intracranial haemorrhage: primary
open or maintain the patency of the ductus subarachnoid, periventricular/ Examination
arteriosus and the appropriate dosage intraventricular, subdural. Any infant that has presented with seizures
should be discussed with the accepting pae- Electrolyte imbalance: hypoglycaemia, or potential seizures needs a thorough
diatric cardiologist. This will allow mixing of hypocalcaemia, hypomagnesaemia, examination. The anterior fontanelle should
deoxygenated and oxygenated blood, hyponatraemia. be soft and non-bulging in the neonatal
increasing the oxygen saturations of the Metabolic: amino acidopathies, organic period. A tense fontanelle is suggestive of
infant. A dose of 0.050.1 mcg kg1 min1 acidopathies, hyperammonaemia, raised intracranial pressure. This may result
IV is generally recommended, remembering pyridoxine deficiency. from an intracranial bleed or excessive
that the most serious side effects of this drug Intracranial infections: bacterial and viral. swelling secondary to neonatal meningitis.
are hypoventilation and apnoea. Developmental defects: If a bleed is suspected, this may be idio-
Once a diagnosis of congenital heart neuromigrational disorders. pathic, secondary to a bleeding tendency
disease has been made then ongoing man- Drug withdrawal: opiates or maternal or a result of non-accidental injury. If a
agement by a paediatric cardiologist is benzodiazepine abuse. bleeding tendency is suspected other
essential. Transfer to a paediatric cardiology Familial. bruising or other bleeding diatheses may
centre should be organised and the infant be identified and the stool and urine should
should be fully monitored prior to transfer, both be examined for the presence of blood.
including pre- and post-ductal saturations, Clinical features The head should be measured and the
respiratory rate, temperature, pulse, ECG History head circumference should be plotted on a
monitoring and blood pressure. Transport The clinical history will provide essential centile chart to compare with the centile
should be provided by a team expert in clues to the cause of the seizures. A thor- that the head circumference lay on initially.
neonatal resuscitation. ough history of the pregnancy, labour and This will indicate if there is progressive

564
26.1 ACUTE NEONATAL EMERGENCIES
26

ACUTE NEONATAL PROBLEMS

hydrocephalus. Once the fontanelle has differential diagnosis. If a congenital infec- respiratory reserves of the neonate. This
been examined the rest of the nervous sys- tion is possible, either the mother is non- should be considered early as the rapid
tem should be assessed. Tone and reflexes immune or has risk factors for the possible correction of hypoxia aids in limiting
should be elicited to indicate any CNS development of rubella, cytomegalovirus or seizure time.
involvement. The eyes should be examined toxoplasmosis in pregnancy, then a TORCH Ensure cardiac support. Fluid therapy is
for retinal haemorrhages and signs that screen should be performed. often required and may involve bolus
the intracerebral injury could be deliberate. In addition to biochemical and infective replacement therapy in a shocked and
Severe hydrocephalus associated with sei- profiles, a metabolic screen should be per- acidotic infant.
zures may be associated with a downward formed on fresh urine collected from the If hypoglycaemic: glucose 10% solution:
deviation of the eyes. infant, and certain inborn errors of metabolism 5 mL kg1 IV followed by continuous
Considering the multitude of causes of can be eliminated by chasing the new- infusion at 57 mg kg1 min1.
neonatal seizures there will be many poten- born screening test performed on all newborn Other specific treatments (as indicated):
tial physical signs that can be elicited. Signs infants between days 3 and 5 of life. calcium gluconate 5% solution 4 mL kg1
of infection, either congenital or acquired, Once basic blood, urine and CSF tests IV; magnesium sulfate 50% solution
may include a petechial rash, respiratory have been collected, a cerebral ultrasound 0.2 mL kg1 intramuscular; pyridoxine
distress and hepatosplenomegaly, or in scan should be organised. This allows both 50100 mg IV.
neonatal meningitis they may be associated sagittal and coronal views of the brain Symptomatic treatment: phenobarbital
with severe septic shock. through the anterior and posterior fonta- loading dose 20 mg kg1 IV; additional
It is important to recognise that a lethar- nelles. This simple bedside test will demon- doses 5 mg kg1 IV (1015 minutes) to
gic infant may be dehydrated and suffering strate intracerebral haemorrhage and may maximum of 20 mg kg1; phenytoin
from the effects of an electrolyte imbalance. demonstrate certain changes typical of con- 20 mg kg1 (1 mg kg1 min1).
In addition, an infant with metabolic genital infections. If there is ongoing suspi- Brain cooling. Newborn infants born
derangements may also present shocked, cion of intracerebral pathology an MRI or greater than 35 weeks gestation,
acidotic and with seizures. The metabolic CT scan may be required. At this stage the who require extensive resuscitation
derangements may have a characteristic infant would require admission and ongoing and have poor Apgar scores and have
odour that, if identified, may lead to a rapid investigation in hospital. abnormal neurological signs including
diagnosis of the seizures. Once a neonate is admitted to hospital with seizures, may be eligible for whole
Although there are many causes of seizures seizures, further investigations may involve an body cooling. If criteria are met
in the neonatal period, the diagnosis is only EEG, with video telemetry to differentiate and temperatures are reduced to
made when adequate investigations are a true seizure from other common diagnoses. 3334 C within 6 hours of birth, the
performed. A paediatric neurologist may be long-term survival and survival free
involved in more intractable forms of seizure. Differential diagnoses of significant morbidity can be
As neonatal seizures can present with such a significantly reduced. This MUST always
Investigations complex array of symptoms and signs, the list be discussed with a neonatal or
Any neonate presenting to the ED with pos- of differential diagnoses is great. Some of com- paediatric intensive care unit prior to
sible seizures needs prompt investigation. moner differential diagnoses include benign commencement.
A first line would be to perform a full set neonatal sleep myoclonus, gastro-oesophageal
The most important determinant of prognosis
of clinical observations, including oxygen reflux, anoxia and neonatal jitters.
is the underlying aetiology. Thus, infants who
saturations, respiratory rate, pulse rate,
have cerebral dysgenesis have uniformly poor
blood pressure temperature and ECG. Once Management
outcomes, and approximately 50% of those
the airway and breathing have been Any neonate presenting with a potential sei-
who have moderate or severe hypoxic
assessed, then a blood sugar should be zure needs urgent management. Airway,
ischaemic encephalopathy develop sequelae.
performed, and serum taken for calcium, breathing and circulation should all be
In contrast, infants who have transient meta-
magnesium and sodium levels. assessed and secured as a priority. Pheno-
bolic derangements and are treated promptly
In any infant, sepsis should be high on the barbitone remains the standard first-line
or who have only subarachnoid haemorrhage
list of differential diagnoses and thorough treatment for neonatal seizures worldwide
usually have a good outcome. Intracranial
investigation should be performed. This but is ineffective in many neonates.
infection and inborn errors of metabolism
should include full blood count, blood cul- Second-line anticonvulsant regimens vary
are associated with a variable prognosis.
tures and, importantly, a lumbar puncture. widely but usually involve the benzodiaze-
The cerebrospinal fluid should be examined pines (diazepam, clonazepam, lorazepam,
for evidence of both bacterial and viral infec- midazolam), phenytoin, or paraldehyde.
tions. If viral infection is suspected then THE VOMITING INFANT
polymerase chain reaction should be per- Acute treatment
formed for both enteroviruses and herpes Ensure respiratory support. Endotracheal True vomiting in babies is best divided into
simplex virus. Glucose and protein per- intubation and respiratory support are two broad categories: non-bilious and bil-
formed on the CSF also help guide the often required due to the limited ious. Bilious vomiting occurs when bile is

565
26.1 ACUTE NEONATAL EMERGENCIES

purged along with the gastric contents. or ketosis and a family history that includes diagnosed relatively easily by gastrointesti-
Although some small intestinal reflux into possible consanguinity can help to determine nal contrast study. It is more common in
the stomach is common with all vomiting, the diagnosis. older children.
in non-bilious vomiting, antegrade intestinal Vomiting occurs in any neurological condi-
flow is preserved, and the majority of the tion that involves increased intracranial pres-
bile drains into the more distal portions of sure (ICP), such as hydrocephalus, congenital Management
the intestine. If an obstruction is present, malformation, intracranial haemorrhage or Any infant who is seriously ill with vomiting
non-bilious vomiting implies that the mass lesion and infection. Additionally, requires immediate resuscitation and admis-
obstruction is proximal to the ampulla of babies who have seizures, autonomic disor- sion to hospital. In many cases, aggressive
Vater. Conditions leading to bilious vomiting ders (RileyDay syndrome), and conditions fluid management in addition to resuscita-
involve either a disorder of motility or affecting the floor of the fourth ventricle tion following ILCOR recommendations will
physical blockage to this antegrade flow of without increased ICP frequently have their need to be implemented without delay.
proximal intestinal contents distal to the condition worsened by vomiting. In the case of the infant with persistent
ligament of Treitz. The anatomic and, thus, the generally sur- non-bilious vomiting, early consultation with
gical causes of non-bilious vomiting are a paediatrician or paediatric surgeon should
those that affect the intestinal tract proxi- occur, as in many cases further evaluation
mal to the point of bilious drainage (ampulla may be necessary. Where an underlying met-
Non-bilious vomiting of Vater), which is proximal to the ligament abolic or endocrine disorder was considered
Gastro-oesophageal reflux (GOR), although of Treitz. These include oesophageal/gastric in the differential diagnosis, stabilisation
not true vomiting, is frequently included in atresia, duplication/diverticulum/choledo- should occur as outlined above, with partic-
discussions of vomiting but really only chal cyst, pyloric stenosis and web. Any ular attention to any underlying metabolic
occurs as a result of failed normal oesopha- infant who exhibits persistent non-bilious and electrolyte derangement. Early consul-
geal function. Normally, the lower oesopha- vomiting, with or without feeding, in the tation with a specialist with experience in
geal sphincter (LES) relaxes with swallowing immediate newborn period must be sus- the care of these rare metabolic conditions
and propagation of oesophageal peristalsis, pected of having an intestinal atresia or a should occur as soon as the diagnosis is
allowing a food bolus to enter the stomach. luminally obstructing lesion (pyloric steno- entertained. Stable infants with vomiting
Its basal contraction prevents food from re- sis, luminal band, web) proximal to the point that is not serious and who are otherwise
entering the oesophagus from the stomach. of bile drainage An easy and rapid test to well, such as those with GOR, can be dis-
Transient relaxation of the LES predisposes evaluate possible oesophageal atresia is charged from the ED, provided that suitable
to GOR and is the major mechanism in the ability to pass a nasogastric tube easily follow up and family support have been
infants who have GOR. The LES is aided by into the stomach. After the tube has been organised.
surrounding structures, especially the crural passed, it is important to obtain a radio- For those infants with bilious vomiting, an
diaphragm, and disruption of these struc- graph to ensure that the tube is in the stom- underlying surgical disorder must be consid-
tures, as with a hiatal hernia, contributes ach and not coiled in an atretic oesophagus. ered until proven otherwise. Early consulta-
to the GOR in some patients. GOR also is Any resistance to passage of the tube is an tion with a paediatric surgeon is mandatory
distinguished from true vomiting by its indication for evaluation by contrast radio- in every case. If the infant is seriously ill, then
symptoms the emesis of GOR is effortless graph for an obstruction. If an obstruction resuscitation should commence immediately
and generally not associated with retching is present, naso-oesophageal tube drainage following ILCOR recommendations, with a
or autonomic symptoms. is important to prevent aspiration of pooled particular focus on maintaining circulation
Both inborn errors of metabolism and oesophageal secretions. Contrast studies and fluid status in addition to correcting
endocrine disorders can cause vomiting in are the standard for the diagnosis of these underlying metabolic and electrolyte
neonates. The physician should consider gly- conditions. derangements. After the diagnosis has been
cogen storage disease II (Pompes disease), established radiographically, the gastroin-
galactosaemia, urea cycle defects, phenyl- testinal tract should be decompressed with
ketonuria, Zellwegers disease, adrenal a nasogastric tube, the infant kept nil by
leukodystrophy and carnitine deficiency syn-
Bilious vomiting mouth and supported with intravenous fluids
dromes in the sick vomiting neonate. The Although not absolute, anatomic conditions until definitive surgical intervention can be
inborn errors of metabolism generally pres- causing luminal obstruction distal to the lig- undertaken.
ent in early infancy, and the vomiting is asso- ament of Treitz usually cause bilious vomit-
ciated with symptoms of lethargy, hypo- or ing. Bilious vomiting is an ominous sign that
hypertonia, seizures, or coma. The constella- mandates immediate evaluation. Conditions THE COLLAPSED INFANT
tion of symptoms is similar to that seen to be considered in the vomiting baby
in sepsis, necessitating a high index of suspi- include intestinal atresia and stenosis, mal- Occasionally, a young infant will be brought
cion in the evaluation of these patients. rotation with or without volvulus and intes- to the ED because they just dont look right
The presence or absence of metabolic tinal duplication. Also, malrotation with to the parents. Even inexperienced parents
acidosis, hypoglycaemia, hyperammonaemia, volvulus is a surgical emergency that is whose first baby is just a few weeks old

566
26.1 ACUTE NEONATAL EMERGENCIES
26

ACUTE NEONATAL PROBLEMS

may notice when their child is unusually obtained and supportive care commenced. diarrhoea, vomiting, anorexia, or fever may
sleepy, fussy, or not eating as well as usual. The approach to the collapsed infant be a manifestation of sepsis. Fever is a very
To the physician in the ED, such an infant is presented in Figure 26.1.1. unreliable finding in the septic infant as
may appear quite ill, with pallor, cyanosis, most septic infants will be hypothermic.
or ashen in colour. The infant may be irrita- The septic infant is often pale, ashen, or
ble or lethargic, with or without fever. In even cyanotic, with the skin often cool
addition there may be tachypnoea or tachy-
Sepsis and mottled owing to poor perfusion. The
cardia and/or hypotension, and other signs Sepsis should always be considered when infant may seem lethargic, obtunded, or
of poor perfusion may also be apparent. confronted with an ill-appearing infant. quite irritable. If there is marked tachy-
The most common entities to be consid- The signs and symptoms of sepsis may be cardia (heart rate approaching 200 bpm)
ered include bacterial infection and viral quite subtle. The history may vary, and some together with tachypnoea (respiratory rate
syndromes. There are a number of other dis- infants may seem to be ill for several days, >60 breaths min1), this may herald a rapid
orders that are uncommon, but demand while others deteriorate rapidly. Any one collapse. Disseminated intravascular coagu-
diagnostic consideration because they are or a combination of symptoms, such as lopathy may develop, manifest as scattered
potentially life threatening, yet treatable lethargy, irritability, respiratory distress, petechiae or purpura. If meningitis is present
(Table 26.1.2).
An infant who is critically ill in the first
month of life should initially be presumed Collapsing infant
to have sepsis and empiric antibiotics com-
menced. As Escherichia coli, GBS, Listeria, Supportive care
and other anaerobes are the most likely Airway/breathing/circulation
Intravenous access and fluid bolus 1020 mL kg-1
causative organisms, a combination of ampi- Consider empiric antibiotics (ampicillin 200 mL kg-1 day-1
cillin 200 mg kg1 day1 and gentamicin gentamicin 7 mL kg-1 day-1
7 mg kg1 day1 in divided doses is a reason-
History, examination and initial laboratory tests
able starting point. In the case of suspected
meningitis the addition of cefotaxime
Suggestive physical findings
200 mg kg1 day1 in divided doses may also
be considered. This is a life-threatening Yes No
situation; the airway, breathing, and circula-
tion should be restored, vascular access
GIT, pulmonary, etc
Manage accordingly

Table 26.1.2 Differential diagnosis

of the collapsed infant
1. Infectious diseases Infiltrate on chest radiograph
Bacterial sepsis
Meningitis
Urinary tract infection
Virus infection Pneumonia Yes No
Congenital syphilis
2. Cardiac disease
Congenital heart disease
Paroxysmal supraventricular tachycardia Abnormal blood studies
Myocardial infarction due to anomalous
coronary vessels
Pericarditis
Myocarditis Yes No Sepsis
3. Endocrine disorders UTI
Congenital adrenal hyperplasia Viraemia
4. Metabolic disorders
Hyponatraemia
Hypernatraemia
Inborn errors of metabolism Acidosis Alkalosis Hyponatraemia Hyperkalaemia Hypoglycaemia Leucocytosis
Hypoglycaemia CAH Pyloric stenosis Metabolic disease CAH Shock Sepsis
Drug toxicity Shock Gastroenteritis CAH CAH Viraemia
5. Haematological disorders Dehydration Drug toxicity Water intoxication Metabolic error Myocarditis
Severe anaemia Methaemoglobinaemia Drug toxicity Drug toxicity Pericarditis
Methaemoglobinaemia Appendicitis Reyes syndrome CNS bleed
6. Gastrointestinal disorders
Drug toxicity Methaemoglobinaemia NEC
Gastroenteritis with dehydration
Pyloric stenosis
Appendicitis
Intussusception Intussusception
7. Neurological disease Methaemoglobinaemia
Infant botulism
Child-abuse intracranial bleed Fig. 26.1.1 Approach to the collapsing infant. Source: Adapted from Selbst SM 1985 The septic-
appearing infant. Paediatr Emerg Care 3: 160167.

567
26.1 ACUTE NEONATAL EMERGENCIES

there may be a bulging or tense fontanelle An arrhythmia may cause an infant to

with a high-pitched cry. If the infection has Cardiac disease appear quite ill. An infant with supraventric-
localised elsewhere, there may be otitis When confronted with an unwell infant, car- ular tachycardia often presents with findings
media, abdominal rigidity, joint swelling or diac disease should be considered. An infant quite similar to those of a septic infant. This
tenderness in one extremity, or possibly with a large septal defect, valvular insuffi- arrhythmia is most commonly idiopathic or
chest findings, such as crackles. The disease ciency or stenosis, hypoplastic left heart may be associated with underlying heart dis-
may progress rapidly, with the infant devel- syndrome, or coarctation of the aorta may ease drugs, fever, or infection. Infants with
oping hypotension and/or frank shock. present with congestive heart failure. The supraventricular tachycardia often go unrec-
A high index of suspicion is needed, infant may arrive collapsed with clinical find- ognised at home for days, initially only exhi-
as although the laboratory may be helpful ings that are quite similar to those of sepsis. biting poor feeding, fussiness, and rapid
in suggesting a diagnosis of sepsis, defini- A chronic history of poor growth and poor breathing. If untreated, the infants will
tive cultures require time for processing. A feeding may suggest heart disease. The develop congestive heart failure, often pre-
complete blood count may reveal a leuko- presence of a cardiac murmur is very sugges- senting in a collapsed state, with a heart rate
cytosis or left shift, although this is often unre- tive that a structural lesion needs to be con- in excess of 300 bpm. The ECG will show reg-
liable in infants. A coagulation profile may sidered. The presence of a gallop rhythm, ular atrial and ventricular beats with 1:1
show evidence of disseminated intravascular hepatomegaly, and peripheral oedema conduction and the CXR may show cardio-
coagulopathy, and blood chemistries may should lead to the early consideration of pri- megaly and pulmonary congestion. Man-
reveal hypoglycaemia or metabolic acidosis. mary cardiac pathology. Crackles, wheezing agement should begin with simple
Aspiration and Gram stain of urine, joint fluid, and intercostal retractions are non-specific manoeuvres such as dunking the infants
and spinal fluid, or pus from the middle ear findings that commonly present in either face in a cold water/ice bath; if this is inef-
may reveal the offending organism. Similarly, heart failure or pneumonia. A CXR is the fective, adenosine intravenously in incre-
a chest X-ray may show a lobar infiltrate if most useful investigation and will often ments of 50 mcg kg1 every 2 minutes
pneumonia is present. A Gram stain of a pete- show cardiac enlargement, pulmonary vas- until tachycardia resolves (maximum
chial scraping should be considered as this cular engorgement or interstitial pulmonary 4 mg). In those rare situations where there
will often reveal the responsible organism. oedema. This must be distinguished from is no response to therapy, and the infant
the lobar infiltrates seen in pneumonia. An remains in heart failure, cardioversion
ECG may be helpful in revealing certain con- should be considered.
Viral infections genital heart lesions, in particular in hypo- Additional cardiac pathologies to be con-
Overwhelming viral infections must be con- plastic left heart syndrome where the ECG sidered include myocarditis and pericarditis.
sidered in the unwell-looking infant. Entero- invariably shows right axis deviation, with Such infections are now most commonly due
viral infections in neonates commonly right atrial and ventricular enlargement. to Staphylococcus aureus and coxsackie B
present as a sepsis-like illness. These infants If primary cardiac pathology is considered, virus. These are often fulminant infections,
should be investigated with both polymer- an early consultation with a paediatric and the baby with such a condition will
ase chain reaction and appropriate swabs cardiologist should occur, as an urgent echo- appear critically ill. A complete physical
as directed by an infectious disease cardiogram will be essential in making examination may help to distinguish these
specialist. Respiratory distress will be pres- the definitive diagnosis. Another important conditions from other diagnoses in that
ent in all of these infants, and haemorrhagic cause of the collapsed infant to consider is signs of heart failure may be seen. Pericardi-
manifestations, including gastrointestinal a tight coarctation of the aorta. In this situ- tis may produce distant heart sounds
bleeding or bleeding into the skin, may be ation the commencement of prostaglandin together with a friction rub. Laboratory tests
seen. Seizures often occur as well as icterus, E1 may be life saving, stabilising the infant may be helpful, since a chest X-ray will show
splenomegaly, congestive heart failure and so that urgent transfer to a tertiary centre cardiomegaly and a suggestion of effusion if
abdominal distension. Mortality rates for can take place for definitive diagnosis and pericarditis is present. The ECG will show
enteroviral infections in neonates are quite operative care. generalised T-wave inversion and low volt-
high. Epidemics of respiratory syncytial virus Rarely, an infant with anomalous or age QRS complexes, especially if pericardial
(RSV) occur in the winter, and infants may obstructed coronary arteries will develop a fluid is present. Also, ST-T-wave abnor-
present with apnoea or respiratory distress myocardial infarction and appear initially malities may be seen. The echocardiogram
with cyanosis. Those born prematurely, or as a collapsing septic infant. Such infants will confirm the presence or absence of a
with previous respiratory disorders, are espe- present with dyspnoea, cyanosis, vomiting, pericardial effusion.
cially susceptible to apnoea. These infants pallor, and general signs of cardiac heart fail-
often appear septic. However, a knowledge ure. These infants may also have cardiome-
of illness in the community and a predomi- galy on CXR, and ECG changes of T-wave
inversion and deep Q waves in leads I and
Endocrine disorders
nance of respiratory signs may lead one to
suspect RSV bronchiolitis. A rapid slide test AVL. There is a high level of urgency in trans- Infants with congenital adrenal hyperplasia
for RSV will quickly be diagnostic. The CXR ferring these infants to a tertiary centre may present with a history of vomiting,
shows diffuse patchy infiltrates and possibly where definitive investigation and specialist lethargy, or irritability. On arrival, signs of
lobar atelectasis. intensive care can be commenced. marked dehydration may be present, with

568
26.1 ACUTE NEONATAL EMERGENCIES
26

ACUTE NEONATAL PROBLEMS

tachycardia and possibly hypothermia. A his- methaemoglobinaemia can produce a abdomen will show evidence of small bowel
tory of poor feeding from birth and symptoms toxic-appearing infant. This condition was obstruction.
that have progressed over a few days may reported when the local anaesthetic agent Several other unusual but important gas-
distinguish this condition from sepsis. The prilocaine was used in penile blocks for trointestinal disorders have to be considered
physical examination can establish the diag- circumcision, (methaemoglobinaemia is a in infants. Necrotising enterocolitis occurs in
nosis in females if ambiguous genitalia are well-known adverse effect of excessive doses premature infants in the first few weeks of
noted. Metabolic disturbance is common, of this local anaesthetic). These infants may life and can also occur in term infants, usu-
with marked hyponatraemia with severe present with cyanosis, poor feeding, vomit- ally within the first 10 days of life. A history
hyperkalaemia. Other non-specific labora- ing, diarrhoea, lethargy, hypothermia, tachy- of an anoxic episode at birth, or other neo-
tory findings include hypoglycaemia, acido- cardia, tachypnoea, hypotension and severe natal stresses, may be risk factors for necro-
sis, and peaked T waves or arrhythmias on acidosis. They appear mottled, cyanotic, tising enterocolitis. These infants are quite
the ECG. The finding of elevated urinary or ashen, and oxygen administration does ill, with lethargy, irritability, and anorexia,
excretion of 17 ketosteroids confirms the not affect the cyanosis. The diagnosis will as well as distended abdomen, bilious vomit-
diagnosis. The infant should be resuscitated be confirmed by a methaemoglobin level ing and bloody stools. Abdominal radio-
as per ILCOR guidelines and initial emer- and if elevated to 65% (normal 02%), graphs may be very helpful and usually
gency management should be discussed with the infant should be transferred to intensive show pneumatosis cystoides intestinalis
a specialist so steroid replacement can be care and treatment with methylene blue because of gas in the intestinal wall. Neona-
commenced as a matter of urgency. They considered. tal appendicitis is a rare event, but several
should then be referred and transferred to cases have been reported to closely mimic
a tertiary centre where management will sepsis. The mortality for this disorder is close
continue with steroid replacement tailored to 80%, and perforation obviously worsens
to the infants underlying needs. Gastrointestinal disorders the prognosis. Thus, rapid diagnosis is essen-
Gastroenteritis, even without electrolyte dis- tial. The most common presenting signs
turbances, can lead to severe dehydration in include irritability, vomiting, and abdominal
Metabolic disorders the infant with little reserve. Bacterial infec- distension on examination. There may also
tions like Salmonella and Campylobacter be hypothermia, ashen colour, and shock
Metabolic disorders may result from pro- as the condition progresses. There may also
and even viral agents need to be considered.
longed diarrhoea or vomiting producing be oedema of the abdominal wall, localised
A stool smear that shows polymorphonu-
dehydration, electrolyte disturbances, and to the right flank, and possibly erythema of
clear leucocytes is suggestive of bacterial
acidbase abnormalities, so that an infant the skin in that area as well. The white blood
infection.
will appear quite ill. These infants may have cell count may be quite elevated with a left
Pyloric stenosis is most commonly seen in
marked hyponatraemia, appear extremely shift, and there may be a metabolic acidosis
male infants 46 weeks of age and may
lethargic, with slow respirations, hypother- present, as well as disseminated intravascu-
cause severe vomiting with significant dehy-
mia, and possibly seizures. Likewise, dehy- lar coagulation. Abdominal radiographs
dration and lethargy without fever. A careful
drated infants with hypernatraemia may may show a paucity of gas in the right lower
history reveals vomiting to be the predomi-
be lethargic or irritable, with muscle weak- quadrant, evidence of free peritoneal fluid,
nant feature of the illness, and there may
ness, seizures, or coma. Those infants with or an abnormally thickened right abdominal
be a positive family history for pyloric steno-
persistent vomiting (classically in pyloric ste- wall owing to oedema. Other unusual
sis. The physical examination may reveal an
nosis) may have hypochloraemic alkalosis gastrointestinal emergencies to consider
abdominal mass, or olive, in up to 50% of
with hypokalaemia, and they may appear include volvulus, perforation due to trauma
cases; this strongly suggests the diagnosis
weak or have cardiac dysfunction. Rare from enemas or thermometers, and Hirsch-
of pyloric stenosis. Electrolytes typically
inborn errors of metabolism may produce sprungs enterocolitis.
show hypochloraemia and hypokalaemia,
vomiting in young infants who will then
and alkalosis is prominent. An abdominal
present with lethargy, seizures, or coma
ultrasound gives a definitive diagnosis in
caused by metabolic acidosis, hyperammo-
the majority of cases.
naemia, or hypoglycaemia. It is thus essen-
While intussusception is rare in infants
Neurological disease
tial to evaluate the electrolytes, acidbase
less than 5 months old, some young infants The infant with botulism (Clostridium botu-
status and blood sugar in young infants with
may present with vomiting, fever, or signs of linum) may present with similar symptoms
significant symptoms of gastroenteritis, leth-
abdominal pain (legs drawn up, irritability). to an infant with collapse or sepsis. An infant
argy, or irritability and coma.
The infant may appear to have spasms of with botulism will often be quite lethargic
pain during which he/she is quite fretful. upon presentation to the ED, with a weak
This can be followed by apathy and listless- cry and possibly signs of dehydration. These
Haematological disorders ness. Diarrhoea is a late sign, as is the typical infants are usually afebrile. If constipation
Any infant with severe anaemia or blood redcurrant jelly stool. An abdominal mass has preceded the acute illness, botulism
loss can look ill. In addition to anaemia, may be palpated, but the diagnosis can be should be seriously considered. In Australia,
disorders of haemoglobin, such as made using ultrasound. A plain film of the the disease is most commonly associated

569
26.1 ACUTE NEONATAL EMERGENCIES

with the ingestion of honey. The parents intensive care. With adequate anticipation, steps. The appropriate response to abnor-
may note a more gradual progression of this it is possible to optimise the delivery setting mal findings also depends on the time
illness. Infants with botulism are notably with appropriately prepared equipment elapsed since birth and how the infant
hypotonic, hyporeflexic, and may have and trained personnel who are capable of has responded to previous resuscitative
increased secretions due to bulbar muscle functioning as a team during neonatal interventions.
weakness. Also, the presence of a facial resuscitation. Most newborn infants will respond to the
droop, ophthalmoplegia, and decreased Neonatal resuscitation can be divided stimulation of the extrauterine environment
gag reflex are consistent with botulism, into four actions: (1) basic steps, which with strong inspiratory efforts, a vigorous
while they remain unusual findings for a sep- include rapid assessment and stabilisation; cry, and movement of all extremities. If
tic infant. A stool specimen to identify toxins (2) ventilation; (3) chest compressions; and these responses are intact, colour improves
of C. botulinum may be diagnostic but (4) administration of medications or fluids. steadily from cyanotic or dusky to pink,
requires considerable time for identification. Tracheal intubation may be required during and heart rate increases. The infant who
Management is with good supportive care; any of these steps. All newborn infants responds vigorously to the extrauterine envi-
an antitoxin exists but it is not readily require rapid assessment, including exami- ronment and who is term can remain with
available. nation for the presence of meconium in the mother to receive routine care (warmth,
Intracranial haemorrhage secondary to the amniotic fluid or on the skin; evaluation maintenance of a patent airway and drying).
non-accidental injury must be considered of breathing, muscle tone, and colour, and Indications for further assessment under a
in the evaluation of the very ill infant. The classification of gestational age as term or radiant warmer and possible intervention
history may or may not be helpful in estab- preterm. Newborn infants with a normal include: meconium in the amniotic fluid
lishing a diagnosis. The infant may appear rapid assessment require only routine or on the skin, absent or weak responses,
gravely ill with apnoea, bradycardia, hypo- care (warmth, maintaining a patent airway persistent cyanosis and preterm birth.
thermia, and bradypnoea. Careful physical and drying). All others receive the initial Further assessment of the newly born
examination may suggest abuse rather steps, including warmth, maintaining a pat- infant is based on (1) heart rate; (2) respira-
than any other diagnosis. The head circum- ent airway, drying, stimulation to initiate tory effort; and (3) tone. Once resuscitation
ference is often above the 90th percentile, or improve respirations, and oxygen as nec- has commenced oxygen saturations should
the fontanelle may be full or bulging and reti- essary. Subsequent evaluation and inter- also be measured continuously. After initial
nal haemorrhages are found. A computer- ventions are based on (1) respirations; (2) respiratory efforts, the newly born infant
assisted tomography scan will usually demon- heart rate; and (3) tone. Most newborn should be able to establish regular respira-
strate a small posterior, interhemispheric infants require only the basic steps, but for tions sufficient to improve colour and main-
subdural haematoma. Referral to the appro- those who require further intervention, the tain a heart rate >100 bpm. Gasping and
priate authority for further investigation and most crucial action is establishment of ade- apnoea are signs that indicate the need
ongoing management is now legally manda- quate ventilation. Only a very small percent- for assisted ventilation. Heart rate should
tory and a key component of the infants age will need chest compressions and be consistently >100 bpm in an uncompro-
acute care. medications (<1%). mised newly born infant. An increasing or
decreasing heart rate also can provide evi-
dence of improvement or deterioration. An
uncompromised newly born infant will be
RESUSCITATION OF THE Evaluation of the newborn able to maintain a pink colour of the mucous
NEWBORN INFANT Determination of the need for resuscitative membranes without supplemental oxygen.
efforts should begin immediately after birth Pallor may be a sign of decreased cardiac
The following is based on the ILCOR recom- and proceed throughout the resuscitation output, severe anaemia, hypovolaemia,
mendations for newborn infants. Resuscita- process. An initial complex of signs (meco- hypothermia, or acidosis.
tion of the newborn infant presents its nium in the amniotic fluid or on the skin,
own set of challenges. The transition from cry or respirations, muscle tone, colour, term
placental gas exchange in a liquid-filled or preterm gestation) should be evaluated
intrauterine environment to spontaneous rapidly and simultaneously by visual inspec-
Basic steps
breathing of air requires dramatic physiolog- tion. Actions are dictated by integrated eval- Preventing heat loss in the newborn is vital
ical changes in the infant within the first uation rather than by evaluation of a single because cold stress can increase oxygen con-
minutes to hours after birth. Up to 10% of vital sign, followed by action on the result, sumption and impede effective resuscita-
all newborn infants may require some and then evaluation of the next sign tion. Placing the infant under a radiant
degree of active resuscitation at birth. (sequential action). Evaluation and interven- warmer away from draughts, rapidly drying
In 50% of cases the need for resuscitation tion for the newly born are often simulta- the skin, removing wet linen immediately,
of the newborn infant can be predicted. neous processes, especially when more and wrapping the infant in warm blankets
However, in the ED such circumstances than one trained provider is present. To will reduce heat loss. The infants airway is
may arise suddenly and may occur in facil- enhance educational retention, this process cleared by positioning of the infant in a
ities that do not routinely provide neonatal is often taught as a sequence of distinct neutral position and removal of secretions,

570
26.1 ACUTE NEONATAL EMERGENCIES
26
or gasping respirations, heart rate <100

ACUTE NEONATAL PROBLEMS

blood, meconium or pus by suctioning if apnoea. If these efforts do not result in
needed. If respiratory efforts are present prompt onset of effective ventilation, discon- bpm, and persistent central cyanosis despite
but not producing effective tidal ventilation, tinue them because the infant is in second- 100% oxygen. Resuscitation bags used for
often the airway is obstructed. Immediate ary apnoea and positive-pressure ventilation neonates should be no larger than 500 mL
efforts must be made to correct over- will be required. If an infant remains brady- and preferably self-inflating. Newer flow
extension or flexion or to remove secre- cardic with a HR <100 bpm or apnoeic, after driven pressure limited devices, reliant on
tions. Aggressive pharyngeal suction can drying and airway manoeuvres, positive a flow of gas to create a pressure, are also
cause laryngeal spasm and vagal bradycar- pressure ventilation should be commenced. recommended and can be used in the new-
dia and delay the onset of spontaneous This can be commenced with air but 100% born and early infant period.
breathing. When providing oropharyngeal oxygen should be available if CPR is required Although the pressure required for estab-
suction, limit depth of suction to approxi- or the newborn is ever asystolic. If positive lishment of air breathing is variable and
mately 5cm from the lips. Negative pressure pressure ventilation is commenced with air, unpredictable, higher inflation pressures
of the suction apparatus should not ex- an oxygen saturation probe should be at- (3040 cmH2O or higher) and longer infla-
ceed 100 mmHg (13.3 kPa or 136 cmH2O). tached to the newborn infants right hand. tion times may be required for the first sev-
If copious secretions are present, the infants The aim is to use blended oxygen and air to eral breaths than for subsequent breaths.
head may be turned to the side, and suction- achieve saturations of 8085% by 5 minutes Visible chest expansion is a more reliable
ing may help clear the airway. Maintaining and 90% by 10 minutes in a newborn sign of appropriate inflation pressures than
proper head position may be helpful in term infant. If air is not available for use in any specific manometer reading. The
up to 12% of deliveries. a positive pressure system, resuscitation of assisted ventilation rate should be 4060
term infants should continue in 100% breaths per minute (30 breaths per minute
oxygen. For cyanosed infants with regular when chest compressions are also being
respirations and a HR >100 bpm, free-flow delivered). Signs of adequate ventilation
Clearing the airway
oxygen or blended oxygen can be delivered include bilateral expansion of the lungs, as
of meconium through a face mask and flow-inflating bag, assessed by chest wall movement and
Approximately 12% of deliveries are compli- an oxygen mask, or a hand cupped around breath sounds, and improvement in heart
cated by the presence of meconium in the oxygen tubing. These babies should have rate and colour. If ventilation is inadequate,
amniotic fluid. When meconium is present oxygen saturation monitors attached to check the seal between mask and face, clear
a significant number (2030%) of infants achieve saturations of 8085% by 5 minutes any airway obstruction (adjust head posi-
will have meconium in the trachea and the and 90% by 10 minutes of age. The oxygen tion, clear secretions, open the infants
need for tracheal suctioning after delivery source should deliver at least 5 L min1, mouth), and finally increase inflation pres-
is indicated in depressed infants or those and the oxygen should be held close to sure and check the equipment being used
apnoeic at birth. If the fluid contains meco- the face (nose) to maximise the inhaled con- is not malfunctioning. Prolonged bagmask
nium and the infant has depressed or absent centration. Many self-inflating bags will not ventilation may produce gastric inflation
respirations, perform direct laryngoscopy passively deliver sufficient oxygen flow (i.e. and this should be relieved by insertion of
immediately after birth for suctioning of when not being squeezed). The goal of sup- an orogastric tube. If such manoeuvres do
residual meconium from the hypopharynx plemental oxygen use should be normoxia. not result in adequate ventilation, endotra-
(under direct vision) and intubation/suction Sufficient oxygen should be administered to cheal intubation should follow.
of the trachea. Tracheal suctioning of the achieve pink colour in the mucous mem- After 30 seconds of adequate ventilation,
vigorous infant with meconium-stained fluid branes. If cyanosis returns when supplemen- spontaneous breathing and heart rate
does not improve outcome. Accomplish tra- tal oxygen is withdrawn, post-resuscitation should be checked. If spontaneous respira-
cheal suctioning by applying suction via a care should include monitoring of adminis- tions are present and the heart rate is
meconium aspirator to a tracheal tube as tered oxygen concentration and arterial 100 bpm, positive-pressure ventilation
it is withdrawn from the airway. If the oxygen saturation. may be gradually reduced and discontinued.
infants heart rate or respiration is severely Gentle tactile stimulation may help main-
depressed, it may be necessary to institute tain and improve spontaneous respirations
positive-pressure ventilation despite the while free-flow oxygen is administered. If
presence of some meconium in the airway.
Ventilation spontaneous respirations are inadequate,
Babies born with meconium stained liquor The key to successful neonatal resuscitation or if heart rate remains below 100 bpm,
who are active at birth do not require rou- is establishment of adequate ventilation. assisted ventilation must continue with
tine endotracheal suction. Reversal of hypoxia, acidosis, and brady- bag and mask or tracheal tube. If the heart
Drying and maintaining a patent airway cardia depends on adequate inflation of rate is <60 bpm, continue assisted ventila-
produce enough stimulation to initiate fluid-filled lungs with air or oxygen. Most tion, begin chest compressions, and consider
effective respirations in most newborn newborn infants who require positive- endotracheal intubation. If chest compres-
infants. Tactile stimulation may initiate pressure ventilation can be adequately ven- sions are commenced for bradycardia or
spontaneous respirations in newly born tilated with a bag and mask. Indications for asystole, 100% oxygen should also be used
infants who are experiencing primary positive-pressure ventilation include apnoea for the positive-pressure ventilation.

571
26.1 ACUTE NEONATAL EMERGENCIES

signs in the vast majority of newly born sequence of hypotension followed by

Endotracheal intubation infants. Initiate chest compressions if hypertension possibly increases the risk of
Endotracheal intubation may be indicated there is a heart rate <60 bpm, even if venti- intracranial haemorrhage, especially in pre-
at several points during neonatal resuscita- lation is adequate on 100% oxygen for term infants.
tion: when tracheal suctioning for meconium 30 seconds. Because chest compressions Volume expanders may be necessary to
is required; if bagmask ventilation is inef- may diminish the effectiveness of ventilation, resuscitate a newly born infant who is hypo-
fective or prolonged; when chest compres- do not initiate them until lung inflation and volaemic. Suspect hypovolaemia in any
sions are performed; when tracheal ventilation have been established. Provision infant who fails to respond to resuscitation.
administration of medications is desired; or of chest compressions is likely to compete Consider volume expansion when there has
during special resuscitation circumstances, with provision of effective ventilation. Coor- been suspected blood loss or the infant
such as congenital diaphragmatic hernia dinate compressions and ventilations to appears to be in shock (pale, poor perfusion,
or extremely low birth weight. The timing avoid simultaneous delivery. There should weak pulse) and has not responded ade-
of endotracheal intubation may also depend be a 3:1 ratio of compressions to ventilations, quately to other resuscitative measures.
on the skill and experience of the resuscita- with 90 compressions and 30 breaths to The fluid of choice for volume expansion is
tor. Perform endotracheal intubation orally, achieve approximately 120 events per min- an isotonic crystalloid solution such as nor-
using a laryngoscope with a straight blade ute. Reassess the heart rate approximately mal saline or Ringers lactate. Administra-
(size 0 for premature infants, size 1 for term every 30 seconds. Continue chest com- tion of O-negative red blood cells may be
infants). Insert the tip of the laryngoscope pressions until the spontaneous heart rate indicated for replacement of large-volume
into the vallecula or under the epiglottis is 60 bpm. blood loss. The initial dose of volume
and elevate gently to reveal the vocal cords. expander is 10 mL kg1 given by slow intra-
Cricoid pressure may be helpful. Insert the venous push over 510 minutes. The dose
tube to an appropriate depth through the may be repeated after further clinical assess-
Drugs ment and observation of response. Higher
vocal cords as indicated by the vocal cord
guide line and check its position by the cen- Drugs are rarely indicated in resuscitation of bolus volumes have been recommended
timetre marking on the tube at the upper lip. the newborn infant. Bradycardia in the for resuscitation of older infants. However,
Record and maintain this depth of insertion. newly born infant is usually the result of volume overload or complications such as
Variation in head position will alter the inadequate lung inflation or profound hyp- intracranial haemorrhage may result from
depth of insertion and may predispose to oxia, and adequate ventilation is the most inappropriate intravascular volume expan-
unintentional extubation or endobronchial important step in correcting bradycardia. sion in asphyxiated newly born infants as
intubation. Administer medications if, despite adequate well as in preterm infants.
After endotracheal intubation, confirm ventilation with 100% oxygen and chest Use of sodium bicarbonate is discouraged
the position of the tube by the following: compressions, the heart rate remains during brief cardiopulmonary resuscitation.
observing symmetrical chest-wall motion; <60 bpm. Medications and fluids are easily If it is used during prolonged arrests unre-
listening for equal breath sounds, especially administered via an umbilical venous cathe- sponsive to other therapy, it should be given
in the axillae, and for absence of breath ter or a peripherally placed intravenous only after establishment of adequate ventila-
sounds over the stomach; confirming the catheter. The intraosseous route is less com- tion and circulation. Later use of bicarbonate
absence of gastric inflation; watching for a monly needed in newborns but is useful for treatment of persistent metabolic acidosis
fog of moisture in the tube during exhala- in the emergency department in the resusci- or hyperkalaemia should be directed by
tion and noting improvement in heart rate, tation of infants. arterial blood gas levels or serum chemis-
colour, and activity of the infant. If available, Administration of adrenaline (epinephrine) tries, among other evaluations. A dose of
the use of a carbon dioxide detecting device is indicated when the heart rate remains 12 mEq kg1 of a 0.5 mEq mL1 solution
is extremely useful in neonatal intubation. If <60 bpm after a minimum of 30 seconds may be given by slow intravenous push (over
endotracheal intubation is unsuccessful it is of adequate ventilation and chest compres- at least 2 minutes) after adequate ventila-
possible to use a size 1 laryngeal mask air- sions. Adrenaline is particularly indicated in tion and perfusion have been established.
way to support breathing in infants >34 the presence of asystole. The recommen- Naloxone hydrochloride is a narcotic antag-
weeks gestation or more than 2 kg in weight. ded intravenous dose is 0.10.3 mL kg1 of onist without respiratory-depressant activity.
a 1:10 000 solution (0.010.03 mg kg1), It is specifically indicated for reversal of respi-
repeated every 35 minutes as indicated. ratory depression in a newly born infant whose
If endotracheal adrenaline is to be admin- mother received narcotics within 4 hours of
Chest compressions
istered a higher dose is recommended of delivery. Always establish and maintain ade-
Asphyxia causes peripheral vasoconstriction, 0.51 mL kg1 of a 1:10 000 solution quate ventilation before administration of
tissue hypoxia, acidosis, poor myocardial (0.050.1 mg kg1) placed down the endo- naloxone and the infant should always be
contractility, bradycardia, and eventually tracheal tube. Higher doses have been transferred to a neonatal or paediatric inten-
cardiac arrest. Establishment of adequate associated with exaggerated hypertension sive care unit for observation if naloxone
ventilation and oxygenation will restore vital but lower cardiac output in animals. The has been administered. Do not administer

572
26.1 ACUTE NEONATAL EMERGENCIES
26

ACUTE NEONATAL PROBLEMS

naloxone to newly born infants whose Illingworth RS. Three months colic. Arch Dis Child

mothers are suspected of having recently Further reading 1954;145:16574.

Lucassen PLBJ, Assendelft WJJ, Gubbels JW, et al.
abused narcotic drugs because it may precipi- Alexander R, Crabbe L, Sato Y, et al. Serial abuse in children Effectiveness of treatments for infantile colic:
who are shaken. Am J Dis Child 1990;144:5860. Systemic review. Bri Med J 1998;316:
tate abrupt withdrawal signs in such infants. Brazelton TB. Crying in infancy. Paediatrics 1962;29:57988. 15639.
The recommended dose of naloxone is Carey WB. The effectiveness of parent counseling in McKenzie S. Troublesome crying in infants: Effect of
managing colic. Paediatrics 1994;94(3):3334. advice to reduce stimulation. Arch Dis Child 1991;66:
0.1 mg kg1 of a 0.4 mg mL1 or 1.0 mg mL1 Forsyth BWC. Colic and the effect of changing formulas: A 141620.
solution given intravenously, endotracheally, double blind, multiple-crossover study. J Paediatr Millar KR, Gloor JE, Wellington N, Joubert G. Early neonatal
1989;115:5216. presentations to the paediatric ED. Paediatr Emerg Care
or if perfusion is adequate intramuscularly Holzki J, Laschat M, Stratmann C. Stridor in the neonate and 2000;16(3):14550.
or subcutaneously Because the duration of infant. Implications for the paediatric anaesthetist. Poole SR. The infant with acute, unexplained, excessive
Prospective description of 155 patients with congenital crying. Paediatrics 1991;88(3):4505.
action of narcotics may exceed that of nalox- and acquired stridor in early infancy. Paediatr Anaestha Selbst SM. The septic-appearing infant. Paediatr Emerg Care
one, continued monitoring of respiratory func- 1998;8(3):2217. 1985;3:1607.
ILCOR. International Liaison Committee on Resuscitation Singer JI, Rosenberg NM. A fatal case of colic. Paediatr Emerg
tion is essential, and repeated naloxone doses (ILCOR). Advisory statement: Resuscitation of the newly Care 1992;8(3):1712.
may be necessary to prevent recurrent apnoea. born infant. Paediatrics 1999;103:56.

573
SECTION

27 TRANSPORT AND
RETRIEVAL
Section editor Ian Everitt

27.1 Emergency medical transport and retrieval 574 27.2 Sick child in a rural hospital 583

27.1 Emergency medical transport

and retrieval
Andrew Berry

of children require an emergency response

ESSENTIALS because the problem is time-critical for the
team expertise to reach the patient and/
1 Children are different from adults and therefore can have different needs in terms or the patient to reach the ICU.
of medical retrieval. Early consultation with clinicians skilled in paediatric critical
It is often said that children are not
care is encouraged. Their advice can assist in making a diagnosis, determining
small adults and in several important
treatment and developing a transfer plan. Every hospital likely to use such a service
respects their physiology, pathophysiology
should have a defined process for accessing it.
and behaviour are different from adults.
2 Careful stabilisation of the child pre-transfer achieves the best clinical outcome. In patient transport, not only are these
This is a combined activity of treating staff at the referring hospital and a transport differences relevant, but the organisation
team. That continuum of care is often under the supervision of remote medical of critical care services and hospital net-
experts using telephone support, conference call techniques and remote vision works is also different. There is a more
technology. centralised organisation of specialist and
intensive medical care for children than
3 The transport network has the responsibility for the organisation of a suitable adults and this has implications for their
clinical escort, urgency of response and type of vehicle. Centralisation of that retrieval
transport and how transport systems are
network ensures this occurs in a rapid and seamless manner.
delivered.
4 The medical retrieval team is especially equipped and trained to handle all
situations that may be encountered during patient transfer and hence are the
preferred clinical escort.
5 The key to good patient outcome throughout this process remains effective Regionalisation
communication.
Different hospitals within a region offer dif-
6 Performance of the system should be monitored for quality purposes and fering levels of care for children. There are
system improvement. Reports should include feedback to all participants in broadly three levels of care for children:
the process. (1) basic; (2) specialist; and (3) tertiary. In
Australia and New Zealand, intensive or crit-
ical care tends to be centralised to a few
tertiary hospitals. (There are 22 neonatal
retrieval transfer is discussed. That is, where ICUs in Australia and six in New Zealand.
Introduction expertise normally associated with an inten- There are seven full-time paediatric ICUs
This chapter addresses the secondary trans- sive care unit (ICU) is sent from a central in Australia and one in New Zealand. Some
portation of patients; that is, inter-facility location to the patient and then transports adult units have a specialist interest in pae-
transfer. Primary transport (pre-hospital or the patient back to an ICU or tertiary pae- diatric ICU as well.) This principle is based on
scene transfer) is beyond the scope of the diatric emergency department (ED). Addi- evidence that a centralised approach pro-
chapter. In particular, the role of medical tionally, most medical retrieval transports duces better outcomes than having a larger

574
27.1 EMERGENCY MEDICAL TRANSPORT AND RETRIEVAL
27

TRANSPORT AND RETRIEVAL

number of hospitals in a region each doing a heart lesion, such as left heart obstruction, This clinical conversation is complicated
small amount of critical care.1,2 As a result: than more traditional causes of shock. by the need to decide what level of care
These and other examples require patient the patient requires and whether a rural
Most newborns likely to need critical care transfer decisions to be made by clinicians treating clinician should call a regional or
are delivered in tertiary perinatal centres
aware of these differences and skilled in a tertiary hospital. Some regional hospitals
where neonatal intensive care is available
eliciting information that will clarify the have their own retrieval capacity, allowing
on site and from birth. Progressive
situation. a child to be transported to that hospital
improvements are being achieved in
by a non-specialist team. In some cases, a
better selecting high-risk pregnancies for
paediatric specialist may be able to travel
a more appropriate place of birth.
to the referring hospital to offer on-site
Newborns with major surgical conditions Referring a patient
resuscitation, assistance and assessment in
or complex medical problems requiring to another hospital parallel with a specialist paediatric medical
intensive care are treated in childrens
Referring doctor/hospital ! retrieval team responding from a distance.
hospitals.
referral doctor/hospital In some regions, highly trained paramedics
Infants and children requiring major
Referring a patient for a higher level of care or flight nurses offer a level of transport clin-
surgery or intensive care are treated in
involves a dialogue between clinicians. For ical escort higher than regular ambulance
childrens hospitals.
emergency transfers this generally involves transport. Decisions about how these chil-
Basic hospitals admit children with non-life- a telephone call to a selected referral hospi- dren move and whether transport should
threatening illnesses. Regional and urban tal or physician. The referrer usually makes be to a regional hospital or a tertiary hos-
hospitals with specialist paediatric services this selection on the basis that the referral pital can be difficult. Sometimes a discus-
take care of children with significant illness clinician has the skills and resources to deal sion with both will help determine the
but not those needing intensive care. Chil- with the problem and is geographically prox- best plan.
dren who need intensive care will usually imate. The ISBAR (Introduction, Situation, The relationships between the three
require a medical retrieval team to move Background, Assessment and Recommenda- levels of care are shown diagrammatically
them safely to a tertiary hospital. Other chil- tion)3,4 communication technique is a useful in Fig. 27.1.1.
dren admitted to rural or basic hospitals with tool to assist in this clinical conversation A dialogue with one referral hospital
less-critical conditions may be appropriate (Table 27.1.1). option may recommend that the other is
for transfer using local clinical escorts and The process of telephone triage (tele- a more appropriate destination. Unfortu-
admission to a regional specialist hospital. triage) requires skills of careful listening nately, a series of discussions with different
and intelligent questioning by the person referral hospitals can be time-consuming
taking the call. It is not learnt overnight. and may interfere with direct patient care.
Clinical discussion should precede and deter- Various means have been introduced to
Whats different about
mine the logistics of transport, not the other streamline this process. In some referral hos-
children?
way around. Referral of a critical-care pitals, there is often a designated person
This general question is addressed in other patient can be confounded by resource available to take such calls. Occasionally, a
chapters. However, from a transport point and logistic issues with the potential to pre- specific telephone number in the referral
of view, the type of illness or injury and vent or impair an appropriate and construc- hospital is advertised for that purpose. How-
the process by which a clinical diagnosis is tive clinical discussion. Keep the focus of the ever, if that number takes the referrer down
reached are often different in children. For discussion on the patient. a one-way path (e.g. to a personal cellular
instance, some conditions, such as bronchiol- phone), he or she may need to make other
itis, are not only largely unique to children, calls or await a call-back. Now that hospi-
Table 27.1.1 ISBAR
but manifest in quite different ways from tal voice-response systems with menus
I Introduction. I am (name and role) calling
adults. In the first few months of life, presen- from . . .... . . on behalf of (clinician in charge)
designed for the public and long queues
tation with apnoea along with a history of S Situation. I have a patient (age and weight)
who is a) seriously ill, critically injured, unstable
close-family upper respiratory infection sug- with slow/rapid deterioration, stable but I have
gests this diagnosis even though there may concerns
B Background. The story is (give information Tertiary hospital
be no signs of respiratory distress or problem pertinent to clinical problem/age-group. May (intensive care)
with gas exchange. Making this diagnosis is include date/time of injury/presentation, R
presenting signs and symptoms/medications/ T
often possible over the phone by clinicians recent vital signs/test results/trends in status. R
who are experienced in paediatric critical Ive already spoken to. . .. . .. (where appropriate) T Regional hospital
A Assessment. On the basis of the above, the (specialist care)
care and offering advice from a distance. patients condition is. . ... They are at risk of. . ..
Assessing conscious state in a young child There is a need for . . .. . . T
R Recommendation. Be clear about what you Rural hospital R
with a head injury requires knowledge of are requesting, e.g. the patient needs to be (basic care)
developmental milestones. A baby present- intubated/in an ICU/assessed by a surgeon/
sent for urgent imaging/etc. I need your advice
ing unexpectedly at 1 week of age with but at this stage do not require transfer. Fig. 27.1.1 The relationship between the three
shock is more likely to have a congenital levels of care.

575
27.1 EMERGENCY MEDICAL TRANSPORT AND RETRIEVAL

awaiting an operator have become common, a clinical conduit into the referral hospital Other causes of neurological
simply calling the switchboard of a hospital system but also to activate the logistics depression:
no longer provides direct access for the of transport.5 central nervous system (CNS)
referring doctor. infection
In an ideal world the referring doctor acute life-threatening episode.
would make just one call. After all, the clini- Any condition with the potential for
cal problem, which by definition exceeds Which patients? sudden cardiovascular or neurological
them and/or their hospital, often requires deterioration.
Selecting patients for retrieval transfer
their full attention. Only the treating doctor
depends on the threshold for referral in each Adapting such a list may be helpful in deter-
can place the cannula, intubate, etc. Sub-
hospital; based on the role that hospital has mining the local policy. If in doubt, consult.
verting these clinical activities to make sev-
for treating patients with problems of vary-
eral (or many) phone calls can significantly
ing and different severity. The important
compromise patient care and outcome. As
point is that discussing the possibility of
referral hospitals become busier and/or
transfer should be part of a clinical consulta- Who will move the patient
more efficient, the availability of resources
to accept the referred patient can
tion about the patient. The following list and how?
suggests conditions that should prompt that
dominate the referral process. Too often, After an appropriate clinical discussion, the
discussion with a paediatric ICU or retrieval
the question of whether a Bed (defined as: question of how the patient should be
service:
Space Equipment Nurses Doctors moved can be addressed. This can be
Other Resources) is available intrudes on the Head injury (symptomatic). straightforward or complex, depending on
referral call and can interrupt the conversa- Altered level of consciousness circumstances. The transfer of a patient from
tion with an invitation to try elsewhere. (for any reason). referring hospital to referral hospital can be
Whenever the clinical referral process  Hypoxia despite oxygen therapy. accomplished in several ways. In a patient
becomes a question of bed-finding, many High oxygen requirement. transfer, an escort transports the patient
referring doctors respond by delegating Respiratory failure (e.g. bronchiolitis, and then returns (in Fig. 27.1.1, T followed
the task to others. Such staff wont necessar- severe asthma, apnoea). by R). In a retrieval transfer, the dispatch
ily have any clinical knowledge of the Upper airway obstruction. of a team (response) is followed by patient
patient or the problem requiring transfer. Near drowning (especially with transport (R followed by T).
Some referral hospitals have similarly dele- neurological depression or respiratory Decisions need to be made about three
gated the process of bed-locating to man- symptoms). main topics:
agers whose focus is on managing the  Ingestion with risk of circulatory,
bed resource rather than entering into a airway or neurological compromise.
Clinical escort.
a. Patient transfer:
clinical discussion about the patient. This  Envenomation.
combination results in lost opportunities  Burns:
ambulance officer (various levels
of skill may be available)
for clinical decisions that can change the >10%
nurse
course of events independently of the encircling the neck or involving the
transport process. For instance, an incorrect airway, face, hands, feet, perineum,
doctor.
b. Retrieval transfer:
diagnosis or treatment may be recognised or inner joint surfaces
flight nurse or flight paramedic
and corrected as the result of a clinical dis- associated other significant
cussion. Having discussed the patient, an injury
doctor (medical retrieval)
other.
alternative referral hospital may be recom- electrical or chemical burns. Degree of urgency.
mended on clinical grounds. A conversation Seizures (with persisting neurological
a. Urgent (immediate response).
along these lines is desirable and possible, depression).
b. Emergency (specify expectation
regardless of bed availability. Major trauma (including spinal
of response).
In regions where there are several referral injury)
c. Time window (before a particular
hospitals serving the same referring hospi- Metabolic disturbance, e.g.
time).
tals, a single point of contact is provided diabetic ketoacidosis d. Elective.
for transport of a critical-care patient. In acidaemia  Type of vehicle.
most cases, however, such single number severe biochemical abnormality. a. Surface (road, boat, train, etc.).
systems are designed to facilitate the logis- Heart failure or arrhythmia
b. Air:
tics of transport and the clinical referral pro- (symptomatic).
cess requires a separate, additional phone Shock (requiring treatment with volume
rotary wing
call. Translated simply, this amounts to: replacement or inotropes), e.g.
fixed wing.
find the bed and well arrange transport. blood or fluid loss A patient transfer is one in which the skill
An integrated approach is possible, where dehydration level of the escort is at or below that of
the same number is used not only as septicaemia. the referring hospital. That is, the patient

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TRANSPORT AND RETRIEVAL

can be escorted by ambulance personnel stabilised prior to transport using skills and latest time by which the transfer should
(ambulance transfer). Alternatively, staff equipment not normally available in the be concluded.
from the referring hospital may accompany referring hospital. This approach is prefera- Elective The timing of transfer is
the patient (medical or nursing transfer). ble to hurriedly rushing from A to B with discretionary hours or days and by
In theory these options offer a more rapid the patient in an unstable condition (the mutual consent.
process, although review of actual experi- mercy dash) and risking en-route deteriora-
ence often shows that time benefits can tion. To the extent that the arrival of the The factors determining how quickly a team
be overrated. Using local resources on an retrieval team in the referring hospital can be at the bedside include:
ad hoc basis is often slower to mobilise than represents the patients arrival in intensive Geographical distance.
anticipated. A doctor or nurse from the refer- care, the patient should not suffer any time Modes of transport available.
ring hospital may well offer an adequate penalty from using the retrieval rather than  Functional distance:
level of care but the implications for that the transfer strategy.
team availability
hospital during their absence may include The exceptions are conditions requiring a
vehicle availability
a significant staffing reduction for that hos- procedure or an investigation that a
accessibility
pital. The smaller the hospital, the greater retrieval team cannot supply. In children,
number of vehicle changes.
the impact that this will have on local cover the situations where this is the case are
and, since distances tend to be greater for fewer than in adults. Examples include head Looking at a map to judge the time to reach
smaller hospitals, the longer this impact will injury requiring urgent neurosurgery, some the patient may be misleading. When alter-
last. A hospital clinician who, having cared cases of penetrating trauma, volvulus with native modes of transport are available,
for the patient for some time already, then ischaemia and transposition of the great fixed wing is roughly twice the speed of heli-
leaves town escorting a patient may experi- arteries with intact septum. Some proce- copter and helicopter twice the speed of sur-
ence significant fatigue. There may be pro- dures may be possible in the referring hospi- face transport by road. However, the process
blems finding transport back. tal if those skills are part of the retrieval of activating a helicopter or plane with a
On the other hand, the level of care avail- team. Examples include taking a surgeon team on board can mean the overall
able to the patient should not regress during (neurosurgery, ENT) or obstetrician to response time is double or triple the flight
the transport process. Indeed, care should selected cases to perform an emergency pro- time. The response time also includes com-
increase at each stage of the patients prog- cedure such as decompressing raised intra- ponents of travel after the flight, which
ress through the system. If the patients con- cranial pressure or fibre-optic endoscopy may involve ground transport. The more
dition is unstable, it may be better to for foreign body. Such options need to be vehicle changes that are required, the
continue treatment in the referring hospital planned rather than an ad hoc response. greater the overall time. Each vehicle
and await the arrival of a retrieval team. On the other hand, there is the additional change adds significantly to the total time.
In the retrieval transfer, options for clini- time awaiting the arrival of the retrieval Traditionally, arbitrary distance arcs are spe-
cal escort include a flight nurse or paramedic team. It is often tempting to say, we could cified to help in selecting between road, heli-
attached to an air ambulance service. Exact have been there by now! However, young copter and fixed wing options. However, the
team composition will vary according to children and newborns travel poorly if inade- cut-over from one mode of transport to
local practice and the regulations governing quately stabilised and the lost ground may another in any particular system of retrieval
the skills and certification required for never be regained. needs to be determined around functional
administration of specific treatments and Assessing the level of urgency requires distance rather than geographical distance.
drugs. assessment of how quickly skills or interven- No matter how fast a vehicle travels, the
Doctors participate in medical retrieval tion are required in the referring hospital availability of a suitable vehicle and/or
teams in Europe, South Africa and Australa- and also how quickly the patient needs defi- team may delay departure. Therefore
sia but less so in North America. Most doc- nite care in a referral hospital. In most cases, deployment by road may sometimes result
tors are tasked from the referral hospital the operational response in reaching the in a more rapid response time than by air.
but others are attached as a regional or patient is more relevant. For a particular referring hospital, the choice
state-wide retrieval service. of appropriate vehicle (road, helicopter or
The principle of medical retrieval is to fixed wing) may differ from time to time,
bring the required critical care skills to the according to time of day and day or week.
patient from an ICU setting and initiate that
Degrees of urgency Good data about actual response times
care prior to transport. In a randomised trial, Urgent The patient is in extremis, or is involved in previous cases can inform the
medical retrieval has been shown to offer deteriorating, or needs immediate, time- decision-making process. Such information
significant benefits to preterm infants in critical therapy. is much more useful than simple speed ver-
terms of mortality and morbidity.6 However, Emergency The patient has a serious sus distance graphs.
evidence is not so clear-cut in older age condition and the treatment required is In some cases, the choice of vehicle for the
groups. Medical retrieval offers a higher not possible in the referring hospital. patient transport phase is different from
level of care both during transport and, Time window The patient needs to be at the that selected for team mobilisation. If, for
importantly, prior to travel. Patients can be destination by a certain time there is a instance, a helicopter could not respond

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27.1 EMERGENCY MEDICAL TRANSPORT AND RETRIEVAL

because of availability or weather and the the patient so that basic physiology is
retrieval was launched by road, it may be controlled, with airway, breathing and circu- Comparing adult practice
possible, and clinically appropriate, as the lation supported. The threshold for interven- In recent years medical retrieval services for
weather improves, to use a helicopter for ing in each of these areas is reduced for adults have developed and are now found in
the next leg. When stabilisation times are patients being moved. A successful stabilisa- both metropolitan and regional centres. It is
significant, the use of different vehicles for tion is one in which the risk of an unplanned important that paediatric clinicians have
the outbound and inbound legs of a retrieval intervention being necessary en route is input into decisions made about any older
may make better use of those vehicles with- reduced to a negligible level. Rigorous atten- children whom these services might plan
out impairing the clinical process. tion is required to the patency and security to transport. Many older children may be
The busier a service, the more likely one of the artificial airway and vascular lines. appropriately moved by an adult team.
mission impinges on the next. Delays await- These lines and other connections to the Good communication between the clinical
ing a teams return from one mission should patient, such as non-invasive monitoring players is essential, particularly if the refer-
be analysed to see whether more teams probes, electrocardiogram (ECG) leads, ring hospital calls the regional hospital that
should be available. transducers and drains, should be positioned has an adult retrieval capability and it seems
in such a way that there is no physical force clear that the child is likely to need a higher
causing either traction or torsion. Despite level of care. In some rural emergencies, a
While waiting firm fixation, these forces will cause dis- joint response to the primary hospital by
lodgement in transit. both a regional service and a specialist pae-
The clinical discussion with the referral hos-
pital should include specifics of the actual diatric retrieval team may be appropriate.
condition of the patient, their current treat- After such a joint response, the optimum
ment and a clearly communicated plan of Principles of medical destination for the patient can be deter-
management. Depending on the confidence retrieval mined (regional hospital or specialist chil-
of the referring doctor about these issues, drens hospital).
The stay and play approach is the default Medical retrieval teams that mostly deal
this discussion may require more or less
for children and wrap and run the excep- with adults are often inclined to be fairly
input from the referral doctor. Telephone
tion. The golden hour philosophy can be aggressive in controlling the airway. Unnec-
advice is always central but other modalities
mistakenly applied to paediatric patients. essary intubation and mechanical ventila-
offer additional benefits; including image
It relies on battlefield experience and the tion only complicates the post-transport
(X-ray, clinical photography), remote CCTV
principle that the outcome depends on the management in the ICU and a little more
and customised weight-specific work-plans
patient being on the operating table in less time spent in assessment and stabilisation
faxed to the referring hospital. Such consul-
than an hour from injury. This principle of, for instance, a seizure disorder may
tation is even more relevant when distance
evolved from management of penetrating obviate this intervention.
and the time taken to reach definitive care
trauma in military personnel during the
increases. The expertise of specialists in pae-
Vietnam War and is rarely relevant to criti-
diatric ICU, ED, surgery, burns, neonatology,
cally ill or injured children particularly
and other paediatric disciplines, as well as Accompanying parents?
when they have already presented to a
toxicology and envenomation experts, is
health facility and some or all of that hour Parents will often wish to remain with their
available by telephone through regional,
has already gone. It is tempting for rescue child. Sometimes the presence of a parent
state and national networks.
retrieval services, who normally deal with may be therapeutic if it has a calming effect
adults, to take this approach with children on an otherwise distressed child. The child
and arrive with a patient who then needs who is aware but has significant upper air-
Stabilisation
post-transport resuscitation. way obstruction or respiratory failure is a
Stabilisation is intended to reduce the risk of Improvements in the responsiveness of good example. In other cases the parent
en route deterioration, improve tolerance retrieval teams have shortened the R phase. may not be required to make the childs
of transport and increase the safety of the This is gradually eroding the reflex tendency transport safe but it seems unreasonable
process. to wrap and run with the patient in the not to make provision for a parent to travel
All patients should receive a certain hope that their condition will survive a in the ambulance or aircraft, where possible.
amount of stabilisation prior to transport, mercy dash. Such improvements are occur- Vehicle specifications should allow for a par-
whether their departure is imminent (as is ring through use of dedicated retrieval ent to be accommodated in paediatric medi-
generally the goal if a local escort is teams, on-site staffing and more readily cal retrievals. Should a parent be unwell
planned) or delayed until a retrieval team available and/or faster forms of transport. themselves (e.g. a postnatal mother after
has arrived to transport them. Additional strategies include pre-alerting an operative delivery or a parent injured in
Transport medicine is not so much about of the system during the pre-hospital phase, a multiple trauma case), their medical needs
how a patient is managed in a mobile encouraging earlier referral by the referring cannot reasonably be catered for by a medi-
setting but how to prepare for the transport hospital and more streamlined processes cal retrieval team whose focus is on the
process. Stabilisation involves packaging occurring in parallel rather than in series. child. Clinicians making decisions about

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TRANSPORT AND RETRIEVAL

moving these parents should judge the risks Amelioration: Is available to advise the referrer and/or
and safety of their transfer independently a. Telemedicine improve the referral transport team about further
of the child and, if it is appropriate to process by adding images to management.
move them, a suitable escort should be enhance simple telephone Reviews professional and service practice
provided. conversations. in quality activities.
b. Improved data interfaces between
hospital systems to increase the The transport service
information flow between referring Assesses the level of clinical care required
Prevention hospital, retrieval team and referral to transport the patient.
The retrieval process is not an end in itself. If hospital. Selects the most appropriate (based on
possible, patients should not be allowed to clinical skills) and best available (most
reach a level of clinical severity needing timely) team to treat and escort the
intensive care. Some of this involves general Expectations and roles patient.
preventative strategies in health care and What are the expectations of those partici-
Chooses the most appropriate transport
injury prevention. Those patients that do vehicle (road or air; rotary or fixed wing)
pating in the retrieval process? There are
come to intensive care should, where possi- through liaison with ambulance and
four key players:
ble, present directly to the appropriate facil- other vehicle providers.
ity. Of course, geography makes this goal The referring clinician. Offers a link between the referring
unachievable in many cases. However, pro- The referral (receiving) clinician. clinician and receiving physician. This is
cesses that seek to have the pre-hospital  The transport service. particularly relevant when there are
response deliver the patient to the right hos- The transport clinician. several potential destination units.
pital, bypassing less-well-equipped hospi- An organised and streamlined interaction
tals, are becoming more sophisticated. between these components is required for The transport clinician
Trends towards greater centralisation in the best results. What follows is a summary Participates in, or is informed about, prior
healthcare organisation make this goal of the expectations, roles and responsibil- clinical discussions about the patient.
harder to achieve and the retrieval process ities of each of them. Prepares for the obvious and considers
is likely to be more frequently applied in the not-so-obvious possibilities while
the future. A summary of preventative stra- outbound to the patient.
The referring clinician
tegies includes: Assesses the patient at first look and acts
Expects to make just one phone call to
Reduce the need. initiate the process. according to clinical need.
a. Injury prevention and immunisation Focuses on the basic principles: May re-triage the patient to a different
programs, better interval care of airway, breathing, circulation, for a level of care and/or initiate an
recurrent severe conditions; such as child; warm, pink and sweet, for alternative transport plan.
asthma. a newborn. Chooses between rapid-sequence and full
b. Recognise the fetus at-risk of needing Provides basic details of history, stabilisation retrieval.
ICU or urgent surgery or other assessment, examination and Stabilises the patient using service-
intervention. interventions. specific clinical guidelines.
c. Plan for high-risk births to occur in a Uses the ISBAR technique. Communicates with receiving clinician(s),
tertiary perinatal centre (major Nominates a preferred destination, as appropriate.
surgical and cardiac conditions). based on clinical need, proximity,
d. Facilitate emergency transfer of family, etc.
women when that will result in the
What equipment?
birth occurring in the appropriate The referral (receiving) clinician
hospital in particular, in preterm Offers telephone advice about Medical equipment used for patients in
labour. management. transport needs to be adequate to the task,
Assist and support all participants and Elicits appropriate additional information robust and capable of operating at all stages
players (including remote, rural, regional from the referrer. of the process. It should be secured in the
and metropolitan hospitals) with Assumes some responsibility for vehicle according to the applicable safety
resources, education and support to the patient and the solution of their standards for that vehicle. A monitor or
optimise the care of seriously ill or problem. ventilator attached to an intravenous pole
injured patients at every stage of the Accepts the transfer (receiving) or by friction nut or lying in the bed near the
process. ensures that an appropriate alternative is patient is not safely restrained in a moving
 Conduct quality activities including arranged. vehicle.
audit, incident monitoring, case Ensures that others within the tertiary Power requirements of the equipment
review. hospital are informed and involved. and battery capacities should be thoroughly

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27.1 EMERGENCY MEDICAL TRANSPORT AND RETRIEVAL

understood. Electrically powered devices All equipment should be tested for elec- Most road ambulance vehicles are con-
should be able to utilise external power, tromagnetic interference and radiation. structed on a rigid chassis and have rela-
where possible. That is, susceptibility to external radiofre- tively stiff suspension. A fully configured
Medical oxygen and medical air should be quency interference as well as their ability ambulance is quite heavy, with handling
available to supply constant flows or drive to adversely affect aircraft navigation sys- and ride quality akin to a truck. For a patient
mechanical ventilators. Oxygen can be car- tems. Specific items of transport equipment with a painful injury or disease process, a
ried in aircraft or ground ambulances in com- have been certified for use in aircraft. road journey on anything worse than ideal
pressed or liquid form. Each has its benefits The pack carried by teams should include road surfaces can be a very painful one.
but the choice depends largely on availabil- basic and advanced life-support equipment. Patients with unstable cardiorespiratory sta-
ity. Some ventilators do not require a supply For inter-facility transports, some reliance on tus often tolerate poor road surfaces badly
of medical air. referring hospital equipment is reasonable. and need more aggressive support and/or
Most modern monitors designed for trans- However, for any foreseeable need during increased sedation, analgesia or muscle
port are appropriate for children if they have transport itself, self-reliance is essential. relaxation to improve the situation.
suitable transducers for age for oxygen sat- On the other hand, carrying too many items Road transfers generally involve less
uration monitoring, appropriate algorithms or too many of a particular item can be a dis- handling, since the patient is loaded and un-
for non-invasive blood pressure measure- advantage. It is not possible to cater for loaded only once. The exception is that some
ment and selectable respiratory waveforms. absolutely every contingency and a reason- ambulance services conduct long-distance
Monitoring of temperature, blood pressure able inventory is the product of common road trips using multiple road crews in relay.
(nIBP and IBP), oxygen saturation, ECG sense and experience.
and end-tidal CO2 should be available. For Helicopters
the newborn, skin surface oxygen and CO2 These are fast and travel point to point. They
tension is often added. generally offer a smooth trip although turbu-
Infusion pumps are no longer age-specific Which vehicle? lence can have an impact on the patient as
and most are capable of driving fluids at The most frequently available transport well as the team. The efficiency of a helicopter
accurate low rates suitable for young vehicles include road ambulances, fixed- is highly dependent on whether they can offer
children. wing aircraft and helicopters. The choice door to door service. If a road ambulance is
There are many mechanical ventilators of vehicle depends on the nature of the required to provide transport between
designed for adults that are also suitable distance and the total time involved in landing site and hospital, their efficiency is
for children over the weight of about the outward and return journeys and the compromised. Even short trips by road to
20 kg. There are also ventilators suitable requirements of the patients illness or injury. the helipad add substantially to the time fac-
for newborns and infants up to about Other considerations may include availabil- tor. The handling of the stretcher and patient
5 kg. However, the patient weighing ity of airports or helipads for aircraft and from one vehicle to another is more important
520 kg presents some difficulties and a weather conditions. that the actual road distance. Flight in poor
suitable transport ventilator may be hard Remember it is not the vehicle per se that weather may not be possible unless the air-
to find. is important to the patient it is the maxi- craft and pilots are instrument flight rated.
Defibrillation equipment suitable for chil- misation of available treatment, in the least Even then, helicopters are restricted by opera-
dren should be available in the referring hos- time reasonably possible and movement to tional limits to fuel carrying capacity, range
pital and, in selected cases, to the team in the tertiary centre in a careful, safe and con- and until recently, inability to operate in
transit. The requirement for cardioversion trolled manner. known icing conditions.
is quite rare in childhood and the need can Each vehicle type has advantages and Helicopters expose the patient to the
usually be anticipated. disadvantages. effects of altitude. Cabin pressurisation is not
A portable point of care testing unit for available. The amplitude and frequency of air-
blood gas analysis and basic chemistry is frame vibration in helicopters vary according
particularly useful. Even in referring hospi- Road vehicles to type of blade system and the number of
tals with such facilities, the improved turn- These are relatively slow, and are even blades in the main rotor. Most patients seem
around time to obtain a result may utilise slower if the patient cannot tolerate much to tolerate higher frequencies associated with
the teams time far more efficiently. movement and the driver travels cautiously multiblade aircraft better than two-bladed
Stretchers are not normally designed for to compensate. The driver can stop if machines. Anecdotal experience is that new-
young children and it is necessary to rely required by the team, allowing the team borns with persistent pulmonary hypertension
on paediatric immobilisation systems or to re-evaluate the situation and possibly actually improve as a result of these vibrations.
restraint systems that interface between perform a procedure, such as suction, intuba- These observations have not been tested.
the patient and an adult stretcher. Spinal tion or chest-tube insertion.
immobilisation requires an age-appropriate Road vehicles are at the mercy of road Fixed-wing aircraft
spinal board and careful selection of cervical congestion even if use is made of warning These offer speed and, if pressurised, can fly
collars particularly in smaller children and devices such as lights and sirens to expedite higher at levels less exposed to weather and
infants. progress. turbulence. Pressurisation systems are not

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TRANSPORT AND RETRIEVAL

designed to make patient transport easier required. For most of the flight the pilot can- simultaneous communications akin to a
for medical retrieval teams. They are not assume that anything outside is visible telephone call or face-to-face communica-
intended to provide physiologically tolerable and all navigation and descent to land at tion) for conversational dialogue between
conditions for healthy passengers at alti- the destination relies on the instruments clinicians. Where conventional radios are
tudes that would not normally be comfort- in front of him. The weather conditions at used, a lack of familiarity with their simplex
able. Think of it as being at the elevation the departure point, en route and at the des- mode of operation, where a press-to-talk
of Mount Everest (8850 m, 29 035 ft) but tination must all be assessed. Not only must switch is required, can lead to information
in a cabin pressure altitude (CPA) effectively the pilot be able to take off visually, but the being missed because both parties talk
no higher than Mount Kosciusko (2228 m, in-flight conditions must be compatible with simultaneously.
7310 ft). Healthy passengers travel reason- the aircrafts capabilities. If the expected Wireless technology now makes commu-
ably comfortably at altitudes up to about weather conditions at the destination fall nication more ubiquitous than ever. Cellular
3000 m (10 000 ft) but patients should below specified minima, an alternative land- phones and/or satellite phones can be
not normally be exposed to above 1200 m ing site with better weather conditions must installed in road vehicles and aircraft used
(4000 ft). be within range. The logical algorithms by for patient transport. Performance depends
Patients at increased altitude can find which such assessments are made are unam- on the type of technology employed and
their problems with oxygen delivery exacer- biguous, quantifiable, auditable and gener- the coverage offered by the network. GSM
bated and trapped internal fluids (gases and ally not open to subjectivity. The process digital phones are not designed to be
liquids) subject to expansion according to of IFR planning leads the pilot to give either used in the air and are almost useless for
Boyles law. A patient with marginal oxyge- a go or no go decision. The planning pro- aeromedical work. Satellite phones require
nation may not tolerate a substantial alti- cess might be quite complex and at least careful installation, especially in helicopters,
tude and trapped gases in the gut, pleural 30 minutes may be required to reach a con- where the aerial should not be mounted
space or pericardium should be vented clusion about the flights feasibility. A deci- beneath the rotor hub. When installed they
to atmosphere prior to flight. Otherwise, sion to go may not guarantee arrival at can be quite effective although call rates
ground transport or transport by fixed-wing the intended destination. It simply says and voice delay make them less desirable
aircraft pressurised at or close to sea level that the flight to reach that location can than terrestrial systems. These systems are
should be used. Not all fixed-wing aircraft be attempted safely in the knowledge now ready to carry data as well as voice.
can pressurise the cabin to sea level equiva- that landing at an alternative location Some services are working at creating a
lence at least not without operating at a (including returning to the departure point) paperless environment for the retrieval
significantly lower actual altitude. Aircraft is factored in. team, using electronic devices to enter
pressurisation cannot be activated on the As an oversimplification, modern fixed- patient-care information.
ground, even if the airport is quite high wing aircraft are able to operate in most
and the patient might benefit from descend- weather conditions. Helicopters cannot fly
ing to denser air. In a flight originating from in cloud that is close to 0 C. (freezing point
Medical control
an airport at a significant elevation and of water) or in severe turbulence. Neither
intending to land at sea level or at a signifi- type of aircraft can operate through contig- Most medical retrieval teams operate under
cantly lower elevation, the pilot can dial up uous lines of thunderstorm activity or when clinical policies and procedures determined
the destination altitude quite soon after fog is present for take off or landing. in advance. Such off-line control of the
take off. This strategy can be quite helpful teams practice should be supplemented
in a marginal patient. by on-line control as required. That is,
the team should be able to seek advice
Weather factors
Communications and guidance at any stage of the process
Both helicopters and fixed-wing aircraft are The process of discussing a referral and tria- by a telephone conversation in real time
subject to weather limitations. Operated ging the optimal transport solution relies on with a senior clinician at consultant level.
under visual flight rules (VFR), either type good communication. Earlier, reference has It is helpful if the advice of several senior
of aircraft can be mobilised quite quickly been made to easy access to the key person- clinicians is available in a conference call
by day. A brief assessment of the weather nel in the referral hospital. Subsequently, the whenever there are multidisciplinary
is adequate and, if suitable for flight, the air- lines of communication need to be main- management issues.
craft can be airborne within 5 to 10 minutes. tained so that any change in the patients It seems ironic that once a patient is in a
The pilot navigates by visual reference to condition or further advice sought about major institution, many levels of medical
the ground and recognisable landmarks. management can be conveyed effectively. control are applied to their care. Prior to that
Short-range helicopter operations are partic- During transport, transfer and retrieval and at the phase of their disease process
ularly suitable for this kind of operation. teams should have the ability to call and when the information was leanest and
At night and in poor weather, instrument be called at any phase of the process. Relay- the greatest uncertainty applied, the res-
flight rules (IFR) operations are generally ing messages through non-clinical radio ponsibility for medical care is traditionally
used. A totally different approach to the operators is not sufficient. The communica- delegated to a quite junior medical or para-
planning and conduct of the flight is tion devices should allow duplex (two-way medical level.

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paper, either during pre-hospital care or in to consider holding these activities both in
Some definitions the referring hospital. Furthermore, elec- referring hospitals and referral hospitals.
Common expressions tronic data systems developed to support The informal contact accompanying these
CPA: cabin pressure altitude. pre-hospital care, EDs and neonatal nur- quality activities breaks down barriers,
IFR: instrument flight rules. series are generally not designed to talk increases mutual respect and facilitates tele-
VFR: visual flight rules. with the next party in the chain. As a result, phone communication about real patients in
Referring: doctor or hospital with the each party collects the same information the future.
patient needing transfer. and records it again. This process is ineffi- It is also recommended that retrieval
Referral: doctor or hospital receiving the cient and also liable to error, duplication teams contribute to anonymous incident
call and possibly accepting transfer. and doubt. A challenge yet to be met is to monitoring systems (AIMS). Such tools help
develop a daisy-chain between information to quantify adverse events.
Process systems, which allows previous information
Launch time: time from decision to to be automatically accessible to the next
respond to team departure player in the process. From pre-hospital to Summary
Response time: launch time outbound referring hospital to medical retrieval team
to referral hospital, each field of information Be available.
leg circulation (time spent moving from
should only be collected once. It should then Teletriage intelligently.
one vehicle to the next or from the last
be accessible to and shared with subsequent Respond rapidly as appropriate.
vehicle to the bedside).
carers. Ultimately, this chain should link back Stabilise fully (stay and play rather than
Scene time: stabilisation time
to the referring hospital, completing a circu- wrap and run).
circulation.
lar pathway that ensures outcome and feed- Transport carefully.
Inbound leg: time travelling toward the
Provide feedback to clients.
destination hospital circulation. back is provided to the original carers.
Monitor and review practice.
Re-positioning leg: time taken to return
from the destination to base.
Mission time: response scene
inbound outbound re-positioning.
Quality
References
Restoration time: time reconfiguring The search for improvement applies to 1. Pearson GA, Shann F, Barry P, et al. Should paediatric
equipment and restocking the kit. intensive care be centralised? Trent versus Victoria. Lancet
patient transport as much as any other area 1997;349:12137.
of care. Given the multiple participants and 2. Lewis FR. Improved outcomes from tertiary center pediatric
intensive care: A statewide comparison of tertiary and non-
complex and variable scenarios, a robust and tertiary care facilities. Crit Care Med 1991;19(2):1509.
thorough process of quality assurance is 3. Velji K, Ross Baker G, Fancott C, et al. Effectiveness of an
Information systems essential. adapted SBAR communication tool for a rehabilitation
setting. Healthc Q 2008;11(Sp):729.
Data about patients being transported are The medical retrieval process cannot 4. Marshall S, Harrison J, Flanagan B. The teaching of a
structured tool improves the clarity and content of
supplied from telephone conversations in stand alone from referring and referral hos- interprofessional clinical communication. Qual Saf Health
the referral process and paper records pital practice. In practice, conducting routine Care 2009;18:13740.
5. NSW Emergency Transport Service, Australia. www.nets
travelling with the patient or transmitted case debriefs, morbidity and mortality meet- .org.au.
by fax. In an acute-care setting, many of ings and open-case presentations should 6. Chance GW, Matthew JD, Gash J, et al. Neonatal transport:
A controlled study of skilled assistance. Mortality and
the details required to assist the consulta- include as many players as possible. To per- morbidity of neonates <1.5 kg birth weight. J Pediatr
tive process have yet to be committed to mit full participation, retrieval teams need 1978;93(4):6626.

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 27

TRANSPORT AND RETRIEVAL

27.2 Sick child in a rural hospital
Murali Narayanan Robert Henning

or of European society in general and vary-

ESSENTIALS ing issues with language difficulties may
lead to reluctance to report illness and to
1 There are significant challenges in translating experience from the urban to rural delay presentation to seek medical atten-
healthcare settings including differences in the patients, illnesses and health services
tion. Certain cultural issues, for example
available.
not speaking the name of a deceased
2 The scoop and run model of pre-hospital care is not appropriate in rural/remote Aboriginal person, or the tendency of
medicine patients must be packaged as much as possible before transport. Aboriginal women to avoid eye contact with
a strange man, become important consid-
3 Coordination between rural, central and retrieval services must be optimal. erations in the setting of paediatric emer-
4 Rural services should be supported through targeted training of personnel tailored gency medicine.
to the needs of rural clinicians, easily accessible guidelines, accessible consultation
services, rural clinical facilities with necessary resources and a supportive
relationship with a major urban centre.
The illnesses
Birth history
Low birth weight and difficult access to ante-
natal care in rural areas (especially in indig-
is 25% lower than that in major cities, and
Introduction enous communities) can lead to severe
rural families are 50% more likely to be
illness in the newborn period, with conse-
Most emergency physicians are trained in dependent on government pensions and
quent disability and need for hospital care.
urban centres. Their skills and knowledge allowances than urban families.
are most readily applied when trained Low income can lead to reluctance to con-
staff, equipment and hospital facilities are sult a doctor and to reluctance of the doctor Trauma
immediately available, and when there is to prescribe expensive though appropriate Age-standardised rates of serious injury in
ready access to specialists. This becomes treatments or to commit the family to children from road trauma, interpersonal
an issue when the sick child presents to expensive travel to consult specialists in violence and self-harm increase with dis-
the emergency department in a rural hospi- urban hospitals. tance from major cities in Australia: from
tal. Protocols and treatment plans that are 50% greater in inner regional areas to
appropriate to urban hospitals may be Housing 100% greater in very remote areas. Farm
difficult to apply directly. The quality of housing and of home mainte- accidents occur frequently: 2/3 of acciden-
nance is lower in rural areas: maintenance tal child deaths on farms occur in boys.
costs are higher, and dwellings deteriorate Farm vehicles, and especially all-terrain
Challenges in the rural faster in harsh environments and scarce vehicles and four-wheel motor bikes, account
setting accommodation leads to overcrowding. for many deaths and injuries, especially to
farm visitors. 3540% of child deaths on
One-third of Australians live in areas defined
farms are due to drowning in dams or drains,
as rural or remote (areas outside major cit- Education
especially in children aged less than 5 years.
ies). While Aboriginal and Torres Strait Islan- Lower rates of secondary school completion
ders (ATSI) constitute 2.5% of the total in rural areas than in the city affect both the
Australian population, they make up 24% child and her parents. Infant mortality is Infection and diet
of the population in remote areas and closely related to the level of education com- Infective conditions, especially of the skin,
45% of the population in very remote areas. pleted by the mother. respiratory and gastrointestinal systems,
Chronic or recurrent infection, malnutrition, These issues apply also to children in occur commonly in children from remote
lack of transport, lack of access to health isolated indigenous communities, where communities, where rheumatic heart disease
care and educational deficits are all factors the additional problems of distance, access and post-streptococcal glomerulonephritis
which contribute to poorer health status of to health care and a relative scarcity of may result.
the people living in these areas. indigenous health workers lead to relatively There is lack of easy access to fresh
late hospital presentation of severe illness. food, fruits and vegetables in remote areas.
Income Dietary deficiencies and substance abuse
Although the overall income differences Culture may also contribute to patterns of illness
between rich and poor may be smaller, the A tradition of self-reliance and stoicism, different from those found in children who
median household income in rural Australia combined with suspicion of medical services present to urban hospitals.

583
27.2 SICK CHILD IN A RURAL HOSPITAL

The health services secured. Plan for adequate stocks of

These include community clinics staffed by Caring for the critically medications, compatible fluids and
nurses and/or general medical practi- ill child disposables.
tioners (GPs); local hospitals (staffed by Decisions to consider when child Local temperature issues: for an airplane
nurses, remote emergency physicians and transport on a 45 C day, the
presents with an emergency
GPs) and ambulance services; regional temperature on the tarmac is easily
The scoop and run model which may be
(base) hospitals which often have paediatri- 5560 C. This may have critical
appropriate in some metropolitan environ-
cians, emergency physicians, surgeons, implications for a neonate in a Perspex
ments is not applicable in the remote
anaesthetists and advanced radiology transport cot which is directly exposed to
setting. In fact, the vast distances over which
and pathology facilities. Finally, there the sunlight, especially if there is a
these patients may need to be transported
are city-based patient retrieval services delay in boarding the craft. Equally, cold,
(usually by the RFDS or other state-based
and tertiary hospitals with access to wet and windy conditions may
retrieval service) mandates that they need
paediatric and neonatal intensive care dramatically impact on the stability of
to be very well stabilised prior to transfer.
facilities. a critically ill child.
It needs to be appreciated that the time
Although inner and outer regional and taken to adequately prepare a patient for Flight weather problems: a common
remote/very remote areas in Australia all problem during summer in Northern
transfer from a remote setting is far in excess
have more primary care medical practi- Australia, air turbulence, severe electrical
of the time taken for the same patient in an
tioners per 100 000 population than urban storms and even cyclones can have major
urban setting. Effective and ongoing com-
areas, this is far outweighed by the ready logistical implications for the transfer of
munication between the treating doctor,
access of city dwellers to urban hospitals the patient. Flight plans may need to be
their specialist colleagues in the tertiary
and to specialist care. changed, sometimes once the patient is
emergency department or paediatric inten-
Rural general medical practitioners deal already en route, and back-up plans need
sive care unit, and the RFDS/Retrieval Ser-
with a wide range of illness in a wide range to be in place. Liaising with the highly
vice is critical to the childs management.
of patients. The nature of general practice experienced doctors and operational staff
means that any single practitioner may of the RFDS/ Retrieval Service is critical in
rarely (or never) encounter any one of the Coordination of a resuscitation such a situation.
critical life-threatening illnesses of child- team prior to the childs arrival Aeromedical problems: consideration
hood. This can potentially contribute to If the staff in a remote location have prior needs to be given to changes in cabin
a delayed diagnosis and may lead to warning of the arrival of a sick or injured pressure, oxygenation and temperature
dilemmas in management. In many rural child, they have the opportunity to mobilise which may affect the patient (e.g. child
and remote areas, GPs may be in solo prac- any local resources available to them, and with a pneumothorax). The highly
tice, so that consultation with a colleague also to liaise with tertiary specialist collea- experienced doctors of the RFDS are an
is difficult, and many regions lack a gues and ensure that lines of communica- invaluable resource in this regard.
regional paediatrician to provide timely tion are open for when more clinical
consultation. information is available. It is important to utilise the expertise of GPs
Regional hospitals frequently offer sub- and nurse practitioners, clerical staff, order-
specialty clinics staffed by visiting specia- Potential problems to the lies, RFDS staff and rural medical personnel
lists (e.g. paediatric surgery or paediatric stabilisation the child to share the load when dealing with the mul-
cardiology) but these are relatively infre- The reality is that should something go titasking required in the management of the
quent and may not coincide with the childs wrong during an air transport, it usually critically ill child. Midwives are very able in
severe illness. The burden of diagnosis goes badly wrong, and is difficult to fix caring for sick neonates. One should be
and treatment then falls on the clinician whilst in the air. Preparation and mindful of the fact that it is very stressful
on the spot. anticipation of the potential problems in for the healthcare practitioner dealing with
The resources available in rural hospitals individual cases is thus critical to a paediatric emergency in the isolated
vary: for example, pathology and radiology minimise avoidable pitfalls, prior to remote setting. From their perspective they
staff may be on-call rather than in-house patient transfer. should utilise all available resources to help
after hours, and the selection of tests, scans Vascular access needs to be bullet proof share the load. Do not underestimate the
and other investigations that are available (i.e. more than one intravenous cannula, value of the telephone as an important
may be limited. well secured, and kept patent), piece of resuscitation equipment to avail
Distance and difficulty of access to some endotracheal tubes must be very well resources in times of need.
rural hospitals means that the delay before secured, and painful unstable injuries Conversely, from the perspective of the
arrival of a city-based retrieval team can be appropriately immobilised and pain receiving tertiary unit, it is important to offer
protracted, sometimes many hours. During relieved. ongoing advice and support, and to help
this time, the rural clinician often has to Preparation of equipment, drugs: ensure plan and facilitate the transfer by liaising
manage a very ill and unstable child within there are adequate lines for potentially supportively with the transferring team
the resources of the local hospital. required infusions inserted and well (Fig. 27.2.1).

584
27.2 SICK CHILD IN A RURAL HOSPITAL
27

TRANSPORT AND RETRIEVAL

Remote setting
As tertiary paediatric hospitals have care at a paediatric hospital will be needed.
Treating health responsibility for medical care of children A centrally located paediatric triage and
care professional in their region, they may organise hands- retrieval service, staffed by nurses and doc-
on training programs (e.g. APLS courses), rel- tors with training and experience in triage
Transport team
evant to rural practice and located in the and paediatric retrieval (see Chapter 27.1)
RFDS paediatric hospital, in which rural doctors can transfer severely ill and unstable
PETS, NETS are invited to participate. patients to an urban tertiary hospital.
When distances to a large city are very
Tertiary specialists
Content of training great, it may be more appropriate to transfer
Paediatric ED Illness recognition and resuscitation courses the child to a smaller base hospital closer to
Paediatric ICU such as APLS, PALS, ATLS/EMST should the childs home, if the severity of the childs
be very freely available and the fees illness permits.
Fig. 27.2.1 The Communication Loop dont government-subsidised for nurses and doc-
stop communicating!
tors in rural hospitals. Follow-up training in Management protocols
these areas and on-line plus face-to-face These should be available for a range of
courses on topics relevant to severe child- severe childhood illnesses and be immedi-
What can be done to assist hood illness should also be provided by the ately available (e.g. on-line) in the emer-
care in remote staff of the regional tertiary hospital. gency department of rural hospitals. These
environments? protocols should be up-dated regularly to
Consultation support keep abreast of current therapies. They
Personnel A centrally organised and funded framework should be modified to be clearly appropriate
The numbers of medical and nursing practi- for provision of 24-hour, 7-day advice on all to rural hospital circumstances, although
tioners available in rural communities may medical sub-specialties (including paediatric they may be adapted from those in use in
be increased by financial and educational subspecialties) would relieve much of the the regional tertiary hospital.
incentives and by imposing conditions on uncertainty of remote rural practice and These protocols should be jointly pro-
professional registration that require a would remove many of the delays currently duced by tertiary hospital staff, regional
period of rural service. These may be seen in diagnosis and management of rela- paediatricians and community general prac-
backed up by enhanced arrangements for tively uncommon conditions. titioners, to ensure suitability for the condi-
in-service education, and facilitation of The use of telemedicine, including telera- tions which prevail in a rural hospital. They
career paths. If these arrangements are to diology, as well as the widespread availabil- should contain advice on when to consult,
succeed, serious consideration must be ity of point-of-care biochemical testing can whom to consult, and how to arrange trans-
given to spouse employment, education narrow the uncertainty in diagnosis and fer to a paediatric hospital or to the paedia-
of children and provision of incentives such allow more accurate assessment and moni- tric ward of a regional base hospital.
as a house and car. toring of the response to treatment in the
remote setting. Hospital facilities
A centrally coordinated telemedicine net- Apart from telemedicine, teleradiology and
Education work requires a terminal at each point of point-of-care biochemistry facilities, men-
Regular education sessions to remote envir- care (rural medical centre or rural hospital tioned above, the emergency department
onments can be arranged through urban ter- emergency department or paediatric ward), of a country hospital which only occasionally
tiary paediatric hospitals. These should be capable of high resolution video trans- treats severely ill children needs to be
on-line when possible, with self-testing. They mission and reception, and one or more equipped adequately and appropriately to
should be relevant to the needs of the local corresponding terminals within the city deal with the child on those infrequent
practitioner and should be followed up by paediatric hospital (and general teaching occasions.
face-to-face teaching sessions which are pri- hospitals for other medical specialties). A collection of appropriate resuscitation
marily hands-on, using the information Facilities for on-line transmission of medical and paediatric care equipment, common to
given in the on-line tutorials. The hands-on imaging to a centrally located radiologist all rural hospitals, maintained by the central
teaching may consist of procedural sessions, are also needed. health authority of the state or country,
as well as paediatric mock scenario teaching A roster of designated subspecialty con- devised and updated by people who are
and group discussions of clinical issues sultants with access to telemedicine facil- using such equipment frequently, should
which have arisen within the hospital. ities 24 hours per day would complete the be supplied to each hospital. This equip-
This form of regular in-service teaching consultation support network. ment should be kept in the emergency
should be centrally coordinated, with input department of the rural hospital and
from the local practitioners organisation. Transport checked daily. When an item is used or
It must be centrally funded, with adequate In some cases, even the above consultation out of date, it should be replaced promptly
time allocation in the local practitioners system will not enable the child to be man- from the regional urban store. Such items
calendar. aged at the rural hospital, and specialist include a paediatric range of cervical collars;

585
27.2 SICK CHILD IN A RURAL HOSPITAL

arm splints; intravenous cannulae; laryngo- Clearly, it is very important that tertiary Australian Institute of Health and Welfare. A picture
of Australias children 2009. Canberra: AIHW; 2009.
scope blades; endotracheal tubes; oropha- hospital staff work co-operatively with prac- Cat. no. PHE 112.
ryngeal airways and laryngeal mask titioners in rural areas, and that members of Australian Institute of Health and Welfare. Medical. Labour
Force 2007. National Health Labour Force Series no. 44.
airways (LMAs). this team are aware of each others needs. Canberra: AIHW; 2009.
This culture of co-operation, support and Emergency Medicine in Rural Australia. Submission to DoHA
by the Rural Doctors Association of Australia. Available
mutual respect should be part of the way from: http://www.rdaa.com.au/Uploads/Documents/
in which the tertiary hospital sees its role Emergency%20Medicine%20in%20Rural%
Relations between rural and in the health system.
20Australia_20101012041618.pdf; 2007 [accessed
29.10.2010].
urban hospitals Regular communication between urban Fragar LJ, Stiller L, Thomas P. Child injury on Australian farms.
Canberra: RIRDC; 2005 Rural Industries Research and
In the interests of better coordination and of and rural practitioners, aided by teaching Development Corporation, and Australian Centre for
uniformly good service provision to children exchanges, training visits and telemedicine Agricultural Health and Safety.
Goh A Y-T, Abdel-Latif M El-A, Lum LC-S, Abu-Bakar MN.
in rural areas, the mechanism by which facil- consultations, can promote awareness by each Outcome of children with different accessibility to
ities are extended to rural hospitals and party of the concerns and ideas of the other. tertiary pediatric intensive care in a developing
country a prospective cohort study. Intens Care Med
their staff should be administered at the 2003;29:97102.
Marcin JP, Nesbitt TS, Kallas HJ, et al. Use of telemedicine to
regional paediatric hospital. This mecha- provide pediatric critical care inpatient consultations to
nism should be sensitive to the needs of staff Further reading underserved rural Northern California. J Pediatr 2004;144:
37580.
of rural hospitals, and responsive to their Australian Institute of Health and Welfare. Australias health Peake SL, Judd N. 2007 Supporting rural community-based
suggestions about changes. 2008. Canberra: AIHW; 2008. Cat. no. AUS 99. critical care. Curr Opin Crit Care 2007;13:7204.

586
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SECTION
TEACHING
PAEDIATRIC
EMERGENCY
MEDICINE
Section editor Ian Everitt

28.1 Availing web-based resources 587 28.2 Teaching paediatric emergency medicine 589

28.1 Availing web-based resources

Colin Parker

increasingly from home, the requirements

ESSENTIALS varying between individuals and their learn-
ing needs. Traditionally these materials
1 Web resources are becoming increasingly available and integrated into every day mostly books have been supplied by
practice.
the hospital or ED library, or purchased by
2 Web-based products would ideally provide clinical decision support platforms, staff themselves for use at home. Interactive
rather than being simple data repositories. forms of learning such as lectures, tutorials
and one-to-one supervision are still relevant,
3 Users should be able to generate web material more easily via new media such as but can now be supplemented by newer
blogs, forums, wikis, social networks, podcasts and online data storage.
technologies, to deliver content at a time
and place convenient to the learner.
Social needs should not be overlooked,
these operational, educational and social
Accessing web-based because a cohesive team environment fosters
resources available from a single portal is
resources good clinical care and flow of information.
finally being utilised.
While books are still a popular medium for Operational materials used in everyday
doctors with regard to their daily work and clinical work include work rosters, memos,
learning needs, the rapid progression of web- notice boards, directories, as well as clinical Solutions currently
based media demands that we have a working resources. Clinical guidelines and protocols available on the web
knowledge of how to utilise this evolving tech- of local, national and international origin,
Web-based resources are rapidly evolving
nology to its maximum effect. This chapter as well as traditional textbook-style content
in terms of the modes of delivery as well as
aims to briefly explore the needs of Paediatric about diseases and conditions, can be used
format and flow of content. As books give
Emergency Medicine (PEM) staff, types of web- in a traditional stand-alone way. More signif-
way to CD-ROMs and software based on desk-
based solutions, and possible future trends. icantly, tools for drug doses and other clini-
top computers, so content has moved to the
cal calculations are paving the way for
internet and can be retrieved from hand-held
interactive clinical decision support plat-
devices such as smart-phones via wireless
Needs of paediatric forms, where disease management algo-
networks or telephone service networks.
emergency medicine staff rithms can be integrated with patient flow,
A similar evolution is happening from static
investigation ordering and interpretation,
In previous times, resources for staff working html to dynamic (database-driven) content,
and the electronic health record.
in emergency departments (EDs) were scat- through Web 2.0 towards the semantic web.
Educational materials are used to a
tered around notice boards, bookshelves, Traditionally, content has been provided
lesser extent during everyday work and
folders and in-trays. The technology to make by a limited number of authoritative sources,

587
28.1 AVAILING WEB-BASED RESOURCES

such as publishers of medical textbooks and for professional and educational matters,
journals, generally as high-quality, peer- has been relatively limited thus far. Future directions
reviewed paper publications. Many of these Wikis are web-pages that can be edited by As medicine catches up with technological
are reproduced on computer screens, as a any user, thus harnessing the power of col- advances, we can look forward to
paid service. While these providers will lective knowledge, accelerating the editorial unrestricted, high-speed wireless internet
always have an important role as trustwor- process, and keeping content current. access in our EDs, using hand-held and
thy sources, the trend is towards sharing Social networks such as Facebook and bedside devices which integrate with
more information for free, and greater use Twitter are increasingly being used by medi- decision-support software and the
of flexible copyright licensing of intellectual cal professionals to broaden their social and Electronic Health Record. Information flow
property such as the Creative Commons. In professional networks and to share knowl- to patients could be facilitated by these
the midst of this trend, content is funded edge. Recognised medical experts may have new systems, including the electronic
either by sponsoring organisations such as tens of thousands of followers who receive provision of summaries of clinical
governments or pharmaceutical companies, the snippets of information which they information, test results, discharge
or by selling advertising on the web pages. choose to share. instructions, and medication lists.
Documents are increasingly stored on the Educational interactive spaces
Web 2.0 web, in the so-called Cloud. Many services incorporating RSS feeds, blogs, vodcasts,
The original internet model of web pages allow editing and sharing of documents editable wikis and reference documents
being published by a webmaster and online, as a smarter alternative to emailing will probably be combined in an
distributed in a one-to-many fashion is rap- multiple versions between collaborators, interactive platform where users can ask
idly being replaced by the web 2.0 paradigm, and enabling easy access from any web- questions and discuss topics as an online
where content is generated by the users accessible device. community, from the comfort of home.
themselves, updated continuously and While many of us utilise the free elec- Hopefully, technical standards will
shared in an interactive way. The advantages tronic table of contents (eTOCs) services of continue to converge, allowing EDs
of this wisdom of crowds approach probably reputable journals, often prompted by our around the country and around the world
outweigh the disadvantages of allowing local hospital libraries, personal experience to combine their resources of hardware
open access for anyone to edit online con- suggests that this method of accessing infor- and data via the concept of grid
tent. Web 2.0 is characterised by user-gener- mation has limited usefulness. RSS is a way computing and the deep web. By sharing
ated content, in the form of metadata tags, for information from multiple sources (feeds) unified, coordinated mega-databases of
blogs, forums, wikis, social networks, online to be pushed (rather than having to be clinical data, investigation results,
document storage, RSS feeds, and podcasts. actively fetched or pulled) to the user, and treatments and outcomes, we could
Metadata, or information about data, is collated in one place, the RSS reader or feed potentially combine thousands of
used to tag articles with keywords, in such aggregator. This allows the user to receive clinician lifetimes of individual
a way that content from disparate sources information in a much more efficient way experience into an intelligent, cohesive
can be browsed by clicking on the keywords than browsing journals or websites, and con- resource. Taking this a step further, we
associated with a particular item. This is tent can be viewed at a time and place of the might eventually see internationally-
sometimes displayed in the form of tag clinicians choice. coordinated administration of
clouds, where commonly occurring keywords Podcasts (portable broadcasts) and vod- epidemiological data, clinical research,
are displayed more prominently. Users can casts (video podcasts or videocasts) are a decision support via neural networks,
usually add metadata tags to an item, for utility which also push content to the and even generating new hypotheses for
the benefit of other users. user, in the form of audio or video content, research via artificial intelligence
Blogs (web logs) started out as personal which in the medical context usually means systems constantly re-examining the
online diaries for sharing with others. The lectures, interviews or small-group discus- body of clinical data.
simple tools which allow non-technical users sions with a panel of experts. The major
to add and update web-pages have allowed advantage for busy doctors and nurses is
blogs to evolve into an easy way for anyone being able to enjoy these offerings while Links
to produce their own website. Many medical travelling, exercising, or relaxing at home. A list of useful web resources for PEM can
blogs have thousands of subscribers who The challenge for healthcare profes- be found at: http://lifeinthefastlane.com/
receive updates via email or Really Simple sionals involves assessing the quality resources/PEM
Syndication (RSS). Users can leave comments, and trustworthiness of each of these sources
effectively supporting online discussions. of information. Patients and their families Further reading
Forums are a dedicated online space for may be less aware of this issue as they http://lifeinthefastlane.com/2009/07/information-
discussion topics, usually highly specific to utilise a similar spread of information overload.
http://en.wikipedia.org/wiki/Health_2.0.
a defined area of interest. These discussion sources. We may need to offer our guidance http://www.bmj.com/cgi/content/full/333/7582/1283.
threads are effectively an online record of on the content they are getting from http://casesblog.blogspot.com/2005/10/web-20-in-
medicine.html.
written conversation between two or more online forums, support groups and personal http://www.slideshare.net/colinparker/emergency-medicine-
users, and their use by medical professionals, blogs. web-resources. [Websites accessed 29.10.10].

588
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TEACHING PAEDIATRIC EMERGENCY MEDICINE

28.2 Teaching paediatric emergency
medicine
Colin Parker

Humility Growth in the acquisition of

ESSENTIALS medical knowledge can be likened to explor-
ing a forest: initially it is hard to see the
1 Effective communication skills and healthy attitudes are prerequisites for good wood for the trees; as one gets to know
paediatric emergency medicine practice, and their teaching should take precedence
the landscape, one can develop an appreci-
over teaching knowledge and skills.
ation for the large valley in which the forest
2 Self preservation skills should also be taught. sits. By the third postgraduate year doctors
are familiar with the whole valley. Most then
3 Clinical experience is essential in gaining perspective on clinical knowledge. travel up to the crest and realise that the val-
4 There are unlimited methods for acquiring new knowledge; part of teaching new ley is but a small part of the countryside, and
trainees is educating them about how to use available media and methods to best there is in fact a vast body of knowledge
effect. which they may never know and never dis-
cover. While knowledge itself is a valuable
asset, a high knowledge-to-confidence ratio
is perhaps more desirable.
communicate, it is important to teach the
Introduction correct attitudes before teaching skills and Caring Many communication difficulties
Despite the massive explosion in the avail- knowledge. Doctors with healthy attitudes can be overcome by having and projecting
ability of information, doctors will never be will be driven to continually expand their a caring attitude. With children especially,
replaced by computers. Paediatric emer- knowledge and skills, and will seek out any parents may not care what the doctor knows,
gency medicine (PEM) has elements of sci- available resources to improve the way until they know that the doctor cares. Doc-
ence, particularly with the steady growth they care for their patients. tors own biases and personality differences
of evidence-based medicine, but also relies can sometimes make this caring seem a dif-
heavily on the practical application of expe- ficult task. Projecting a caring attitude initi-
rience. Perhaps the emergent aspects of ally usually results in a positive feedback
PEM, such as trauma resuscitation, have a Desirable attitudes in PEM which sets the scene for genuine caring to
more formulaic basis; however, clear and supervene.
With the success of immunisation pro-
effective communication from the team
grammes and, to a lesser extent, injury pre-
leader is of paramount importance in a chal- Empathy and compassion Doctors who
vention strategies, there seems to be a
lenging resuscitation. are themselves parents find it easier to
changing spectrum of illness in children pre-
The bulk of PEM does not consist of emer- imagine the emotional strain of having a
senting to emergency departments (EDs),
gent interventions, but rather risk stratifica- sick child. This tension may sometimes be
with fewer critically unwell children, medi-
tion, and trading information with parents of expressed in fiercely protective terms by
calisation of behavioural issues, and chil-
children who may or may not be very unwell. a worried parent, in the same way that
dren with a functional component to their
Parents bring their children for reassurance, patients themselves often forego the social
dis-ease. In addition, the public perception
explanation, and occasionally some form of graces when they are unwell. It is important
of the wonders of modern medicine results
treatment. They want to feel that they have not to take this personally, and try to imagine
in high expectations, which are sometimes
been taken seriously, and that someone how the parents are feeling.
difficult for clinicians to meet. The best
cares about the well-being of their child. In
protection is to approach with healthy
short, they want to feel better after seeing Non-judgemental approach There is a
attitudes:
the doctor. common perception among some ED staff
The practice of PEM requires a set of skills, humility; that some children should not have been
knowledge and attitudes broadly similar to caring; brought to the hospital because its not an
those required for the practice of clinical empathy and compassion; emergency. They may blame the referring
medicine generally, but with the added non-judgemental approach; clinician, the parent or the patient. Everyone
requirement of having to communicate honesty and integrity; who brings their child to an ED has crossed
effectively under the pressures of time advocacy and healthy paternalism; some threshold of anxiety about the per-
and emotional stress. Because doctors self-monitoring and awareness of ceived illness, and every one of them has
attitudes are so enmeshed with ability to cognitive errors in medicine. considered not coming to the ED. Most of

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them do not have significant knowledge of skills, teaching and self-preservation. The Several types of information may need to
health-related matters. There is no place most important of these is communication, be provided to the patient and parents. These
for a judgemental approach, which will not a skill which is intimately entwined with atti- include imparting medical facts, explaining
help the child or prevent a future ED visit. tudes and which improves with practice. the natural history of a condition, describing
or clarifying risk, and providing insight or per-
Honesty and integrity It is important to Effective communication spective to the clinical situation. It may help
teach our colleagues humility. Doctors are The groundwork for an effective consulta- to impart the doctors own feelings about
often humbled by questions they cannot tion starts before meeting the parents. Even the clinical risk in an honest and caring
answer and mistakes they may have made. in busy, seemingly chaotic EDs, parents are way, for example, Im a bit concerned about
Appropriate teaching to junior collea- watching and listening to those around some aspects, but my feeling is that your
gues should suggest that answers to parents them, especially members of staff. Individual child will be completely better in a day or
should be I dont know but I will try to find doctors may be seen in a good light by the two. This often goes a long way towards man-
out and to colleagues thats a good idea, way they interact with staff, patients or par- aging unrealistic expectations or unreason-
I forgot to check that. ents, or subtle clues in the way they are able demands, provided that a therapeutic
introduced by their co-workers. Non-verbal relationship has been established first, such
Advocacy and healthy paternalism
aspects of communication play an important that the parents trust the doctor as some-
Planning a course of action requires a collab-
role in the initial impression; the demeanour one who genuinely cares about their child.
oration between the clinician and the
of the doctor is probably more important This is perhaps the most important aspect to
patient or parent. This collaboration needs
than the way they are dressed, although rea- teach: establishing a trusting relationship built
to walk the fine line between patient/par-
sonable standards in dress code assist in on mutual respect, by exercising the attitudes
ent autonomy and the benefit of the unique
engendering trust. previously outlined: humility, caring, empathy,
knowledge and perspective of the health
The essential communication tasks which compassion, non-judgemental approach, hon-
professional. In this regard, doctors need to
need to be achieved in a PEM consultation esty, advocacy and a healthy awareness of
act as advocates for the child and gently
include establishing rapport, gathering ones own limitations. A useful conclusion to
steer the collaborative decision towards
information (taking a clinical history), any consultation is to invite questions. Giving
one that leads to the best outcome for the
providing information, and demonstrating the parent or patient permission to clarify
patient and family.
the attitudes discussed previously. any areas of concern to them provides the
Self-monitoring and awareness of An important teaching point is that gain- doctor with the opportunity to deal with any
cognitive errors Most clinical presenta- ing the trust of children starts with getting concealed dissatisfaction. It also can result in
tions have a differential diagnosis. Healthy their parents or caregivers on side. The child positive feedback, and reassurance that all
self-doubt and consideration of potentially is assessing the way their parent relates to aspects have been well explained.
bad outcomes are useful safeguards. Doctors this stranger, relying heavily on non-verbal
process and synthesise available information cues to decide whether to trust the doctor.
in various ways, subject to a number of errors When talking to the child, an approach that Skilful clinical examination
of cognition. These include diagnosis momen- illustrates the doctors interest in them, at an A good physical evaluation helps to illus-
tum, where doctors accept the perceptions age-appropriate level, is preferred. Rather trate the special skills and knowledge that
of other clinicians and allow a diagnostic label than trying to playfully examine a child, time have been acquired by the clinician, even
to stick, without making an independent spent exclusively in play or developing rap- if these particular skills are not absolutely
assessment. Or doctors may be affected by port for the first minute or two, is time well- essential to the examination. For example,
anchoring, where they decide on a particular invested. All family members and supporting testing the tendon reflexes in a child with
diagnosis relatively early on in the assessment visitors should be acknowledged individu- a minor head injury seldom changes the clin-
process, and reject subsequent information ally. In particular, siblings of pre-school age ical impression, but adds value to the rela-
that does not fit. When there is some diag- may become disruptive later in the consulta- tionship in demonstrating thoroughness.
nostic uncertainty, the simple act of discussing tion if they do not feel involved, and it may Just as it is important to acknowledge all
the case with a colleague can often provide be helpful to give them a minor task or dis- family members and friends at the bedside,
clarity from this cognitive fog. The main traction: could you please hold my torch one may need to ensure that a parent or
stumbling-block is the clinical maturity until I need it. grandparent in the waiting room is in atten-
required to recognise feelings of uncertainty, History taking may be facilitated by clini- dance for the examination and subsequent
and to act on those feelings. cal pathways, pro formas, or checklists, but explanation of findings. This is more time-
diverting attention to the paperwork is a bar- effective than dealing with queries later.
rier to forming an effective clinical relation- It is important to teach that the examina-
ship. These checklists are best incorporated tion of infants and toddlers should usually
Skill set for PEM into practice by referring to them after the be more opportunistic than systematic,
Doctors in PEM need to develop a set of consultation, and going back to fill in the but also that a confident, gentle and
practical skills, including the art of communi- detail later; this is certainly an incentive to structured examination usually inspires
cation, clinical examination, procedural remember the information in future. confidence and trust.

590
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28

TEACHING PAEDIATRIC EMERGENCY MEDICINE

Mastering procedural skills build mutually supportive working relation- each clinical encounter, doctors can become
A handful of life-saving emergency proce- ships with colleagues. Doing meaningful experienced, mature clinicians. A voluminous
dures and a few time-important urgent prac- work as a valued member of a supportive clinical workload without reflection, and
tical skills need to be addressed in the team is the goal to strive for. learning without patients are both sub-opti-
training of doctors working in a paediatric mal routes to this desired outcome.
emergency setting. These include basic
and advanced airway and ventilation skills,
Putting knowledge into
intravenous and intraosseous access, and Helping others acquire
perspective
screening tests for infection such as blood
knowledge, skills and
cultures, lumbar puncture, and urgent urine There is no real limit to the breadth and
attitudes: modes of learning
sampling by suprapubic aspiration or ure- depth of the knowledge-base of PEM. A con-
thral catheterisation. tinuous thirst for knowledge, inspired by the A customised local solution for teaching
desirable attitudes previously mentioned, PEM in a particular ED or institution
can motivate doctors in this exploration, depends on the spectrum of learners (stu-
Learning to teach
but it is impossible for us to acquire all the dents, nurses, doctors of varying experience
With increasing competence it is expected that
medical facts, theories and controversies. and qualifications), incentives to encourage
most doctors will adopt the roles of supervising
The need to carry around encyclopaedic learning, and relative availability of differ-
and teaching others. Teaching the teacher
volumes of knowledge in doctors heads is ent information resources: people, books,
poses a new set of challenges but also brings
slowly diminishing as the information age and other media.
new rewards because of the variety of learning
starts to live up to its promise of instant avail- The requirements of learners vary
and teaching styles amongst different indivi-
ability of highly specific information. There- depending on whether they are nursing stu-
duals. The advantage to those doctors acquir-
fore, learning how to access information, dents, nurses of various grades, medical stu-
ing new knowledge, skills and attitudes is
knowing where to look for high-quality, trust- dents, prevocational doctors, specialists-in-
that they can pick and choose from a variety
worthy content, and being able to critically training from either a paediatric or an emer-
of teachers, and thereby develop their own
appraise and assess the relative value of gency medicine background, senior trainees
style of clinical practice and teaching. Supervis-
the information are becoming vitally impor- in PEM, or consultants with different back-
ing colleagues involves the constant appraisal
tant skills. grounds and strengths. Learners themselves
and reappraisal of clinical risk, if one considers
The value for the diagnostician in explor- should be asked what they perceive as their
that every clinical interaction puts three
ing a large body of knowledge such as PEM general and specific goals and learning
people at risk: the patient, the doctor, and
is not so much in being able to instantly objectives.
the doctor in charge. Inevitably there is an
recall specific facts, but rather to acquire a Incentives to encourage learning include
element of trust, based on intuition, based
low-resolution background of finer forgotten the unavoidable performance appraisal that
on previous experiences, and based on the
details. A specific part of the clinical picture accompanies employment as a hospital doc-
answers to a few pertinent questions. The saf-
may then trigger a diagnostic thought pro- tor, coupled with the concept of gaining a
est alternative for all is for the reviewing doctor
cess leading to an appropriate search. positive job reference for the next rotation.
to adopt a hands-on approach and personally
PEM is unpredictable in terms of what chal- This incentive is relatively subjective, low-
meet the child-parent unit, until that collegiate
lenges might come through the door at any impact, diffuse, lacks immediate reward for
trust is well established and well founded.
moment, yet there are a few recurring themes good performance, and does not generally
which make up a large volume of the clinical identify the acquired abilities of candidates
Self-preservation work. If one excludes minor injuries and seri- with any degree of detail. Formal testing
Working as a doctor in PEM is emotionally ous trauma, more than 80% of the remaining in the form of tests or assignments adds a
tiring, because it involves caring communi- medical presentations are encompassed by dimension of anxiety and workload which
cation under pressure. Doctors in this envi- six clinical scenarios: fever, breathing diffi- may be perceived as a nuisance for both
ronment need to be taught to be careful culty, vomiting with or without diarrhoea, learners and teachers. For this reason, most
to avoid burnout. They should think about abdominal pain, skin rash, and possible sei- testing is ad hoc and informal, being con-
providing the greatest good to the greatest zure. Therefore there is a relatively well- ducted during clinical supervision, shift
number of children over their professional defined scope of learning for those who need handover and small-group tutorials, usually
lifetimes. A doctor who is impaired by ill- to get comfortable with the majority of child- as an opportunistic, subconscious activity
health or emotional exhaustion cannot pro- hood clinical conditions presenting to an ED, by a range of assessors. The traditional
vide good clinical care. and a much larger range of conditions for old-fashioned incentive of accountability
Strategies for avoiding burnout include those who need to know more. for the well-being of ones patients, and the
learning to say no to extra work (even if it While experience on its own can be rela- awkwardness of not knowing what to do in a
brings extra rewards), learning effective tively uninformative, reflecting on experiences given clinical situation, is perhaps being
time management and prioritising tasks enables doctors to increase knowledge, and overtaken by the trend towards increasing
appropriately, physical exercise and pursuit gain perspective. Thus, by seeing many degrees and seniority of supervision at the
of non-work enjoyments, and learning to patients and learning a small amount from clinical coalface. Staff are additionally

591
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motivated to learn by their caring outlook Books resources rests with combining these media
and pride in their work. The option of a Textbooks, handbooks and journals are still into a single interactive space, managed by
safety net should be considered, whereby a convenient, reliable, low-tech learning an active and interactive community of
there are minimum requirements of docu- medium. The limitations of portability, cost, users. It seems likely that software solutions
mented attendance at learning activities. and infrequent updates are still outweighed such as clinical decision support systems
Unfortunately this does not guarantee the by the longstanding trusting relationship doc- and interactive multiple choice question
acquisition of a minimum standard of atti- tors have with the distributors of high-quality, programs will eventually be replaced by
tudes, skills and knowledge. peer-reviewed content from experts in their internet-based interactive spaces or plat-
respective fields. The challenge for these dis- forms combining traditional text, audio
tributors is to adapt their resources to newer and video content with the interactivity of
Learning resources media and models of delivery, rather than blogs, forums, and wikis so that users can
duplicate their books onto a computer screen. contribute to the body of knowledge while
People experiencing the content in a direct and
The centuries-old tradition of mentorship
meaningful way.
and a clinical apprenticeship is no longer Other media
available as a one-to-one model, but this Exciting new modes of learning are con-
can be approximated by arranging for learn- stantly being developed, and the current
ers and teachers to do clinical work in the generation of learners awaits these new
same physical space, within sight and ear- media with anticipation. While there is an Conclusions
shot of each other. The opportunity exists abundance of free content available, a fair
in many EDs for doctors from a paediatric proportion of high-quality content is by paid Engaging learners in the task of acquiring
background to engage in a two-way subscription or membership. Delivery modes the skills and knowledge for the safe and
exchange of ideas with those from an emer- include audio, video, simulation with high or rewarding clinical practice of high-quality
gency medicine background. We learn a low-fidelity training models, dedicated soft- paediatric emergency medicine is difficult.
great deal from teaching our team-mates. ware solutions, and an ever-expanding suite It starts with the challenge of instilling the
Didactic lectures are a way of sharing of web-based resources. appropriate attitudes required to propagate
information with large numbers of learners, Audio and video reproductions of lectures, these skills and knowledge in ourselves and
but are limited by the relative lack of inter- discussions, opinions and procedures may be in our colleagues.
activity, and are likely to be superseded by delivered via physical media such as com-
technological alternatives which allow the pact discs and digital versatile discs, or
learners to choose the time and the environ- increasingly, via web technology such as
ment most convenient for themselves. Small- streaming, mp3s, podcasts and vodcasts. Further reading
group tutorials offer more interactivity, but While these traditional media of text, Groopman J. How Doctors Think. New York: Mariner Books,
are limited by availability of protected audio and video may be individually repro- Houghton Mifflin Company; 2008.
Henry GL. Human interaction: practical ways to prevent
teaching time away from clinical duties for duced on computers and hand-held mobile malpractice. Emergency Medicine Reviews and Perspectives
both learners and facilitators. devices, the real future of web-based 2003; April 2003 (audio series).

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SECTION
PAEDIATRIC
RESEARCH IN THE
EMERGENCY
DEPARTMENT
Section editor Ian Everitt

29.1 Research in children in the emergency

department 593

29.1 Research in children in the emergency

department
Franz E. Babl Meredith Borland Andrew J. Davidson

Increasing numbers of emergency physi-

ESSENTIALS cians, nurses and allied health personnel
are involved in research, and trainees are
1 Research is essential to establish an evidence base in paediatric emergency now required to complete research projects
medicine and optimise individual treatment.
during training. Yet few emergency clini-
2 At the core of any research project is the development of a viable research cians have formal research training. In addi-
question which drives the study design. tion, over the past years both national and
local regulatory requirements have become
3 A biostatistician should be involved early in the study design. more complex. Research funding is often dif-
4 Agreement on key data elements and outcomes, including definitions, feasibility ficult to obtain in both the emergency and
of collection and validity of observations must happen early in the study design. paediatric setting. Hospitals and depart-
ments often have to focus on clinical care
5 Apart from a lack of subjects, the major problem in clinical research projects is with limited resources for dedicated staff,
poor design.
time for research, research assistants or
6 Good clinical research practice is important in all studies. infrastructure for studies. Hence, there is a
critical and overdue requirement for the
7 Consent and risk are key ethical issues in all studies involving children development of paediatric emergency medi-
8 Multicentre research can address the difficulty of recruiting adequate numbers of cine within university academic settings,
paediatric patients. where research is highly valued and an
intrinsic part of daily business.
In addition, serious outcomes and adverse
events are rare in children and data col-
lected at paediatric tertiary institutions
Introduction question, use of an appropriate study may not be applicable to other settings.
Research is an important part of emergency design, adherence to good clinical research Informed consent is difficult to obtain in
medicine as it provides the scientific basis practice and an understanding of the ethical the emergency setting and the ethics of
for optimal patient care. Key elements for basis for research. Study design, the quality research in children, as a particularly vulner-
conducting high quality, ethical research of the conduct of the study and its ethics are able group, creates an additional degree of
are the development of a good research all intricately linked. complexity.

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29.1 RESEARCH IN CHILDREN IN THE EMERGENCY DEPARTMENT

However, despite these difficulties, a number Literature review and it work in a study?) or the effectiveness
of strategies can be used to overcome the level of evidence (does it work in the everyday ED situation?)
perceived barriers and achieve quality research The literature review should provide the (Table 29.1.2).
in children in the emergency department. background to the study question. Litera-
ture databases like Pubmed, Medline,
Google scholar internet search engines, The ethics of medical
EMBASE and CINAHL (Cumulative Index research
Research science to Nursing and Allied Health Literature)
are useful but may initially be overwhelm- Following atrocities during the Second World
All research should have a sound scientific ing. Helpful starting points can be stan- War, written codes of medical ethics have
basis. Getting the science right is essential dard textbooks, the Cochrane library, a been developed such as the Nuremberg
before starting any project. search of BestBets (www.bestbets.org), Code2, the Declaration of Helsinki3 and
BMJ Clinical Evidence (http://clinical- the Belmont Report.4 The key principles
evidence.bmj.com) and the assistance of developed in these documents still underpin
Research question a medical librarian. most ethical guidelines.
The most crucial component of every project It is important to grade the importance of
is the research question. The question defines medical research evidence. There are many Key principles
all the other elements of the research design, grading systems in use internationally. A The key principles of research ethics include
including the hypothesis and objective of the commonly used example from National respect for persons, beneficence and justice
project. Health and Medical Research Council of as well as research merit and integrity.
A good study question should be: Australia (NHMRC) is shown in Table 29.1.1.1 Respect for persons recognises the value of
Systematic reviews often use such a grading autonomy to an individual. Participants must
original or add significantly to what is system as the basis for clinical management have the power to make their own decisions, if
known;
recommendations. The current standard of possible. Having respect for persons requires
relevant and important; research evidence is the randomised clinical due regard for beliefs, customs and cultural
feasible, based on resources, skills, time, trial (RCT), and the highest level of evidence heritage of individuals as well as respecting
subject availability;
is meta-analysis of RCTs. privacy and confidentiality. Consent is the
ethical; key element of respect. The consent to partici-
plausible, that is there should be a pate in a research project must be free and
scientific basis for the question;
Types of studies informed. Free consent implies a voluntary
clearly defined. Studies can be grouped in a number of ways. choice not influenced by external coercion,
Determining these factors requires clinical Studies can be descriptive, analytic or inter- pressure or inducement. Informed consent
perspective and a detailed literature search. ventional. Intervention studies can either implies that the participant has sufficient
In clinical research the most relevant or assess the efficacy of the intervention (does information and adequate understanding of
important questions are those that actually
change clinical practice.
A question can be defined in terms of the Table 29.1.1 Levels of evidence according to type of research question
acronym PICOT:
Level Study design
population (who should be in the study?); I Evidence obtained from a systematic review of all
indicator (what is the intervention or relevant randomised controlled trials
exposure of interest?); II Evidence obtained from at least one properly
comparator (what is the gold standard? designed randomised controlled trial
comparison vs baseline vs control III-1 Evidence obtained from well-designed pseudo-
group?); randomised controlled trials (alternate allocation or
some other method)
outcome (what is the outcome of
interest?); III-2 Evidence obtained from comparative studies
(including systematic reviews of such studies) with
timeframe (over what time period?). concurrent controls and allocation not randomised,
cohort studies, case-control studies, or interrupted
The outcome measures are often the most time series with a control group
difficult to determine and must be clearly
III-3 Evidence obtained from comparative studies with
defined, usually with a single primary out- historical control, two or more single arm studies, or
come to answer the study question and fur- interrupted time series without a parallel control
group
ther secondary outcomes, which may be
multiple, to expand on this question. These IV Evidence obtained from case series, either post-
test or pretest/post-test
should be determined in advance, not after
study results are in. Adapted from NHMRC 1999.

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29.1 RESEARCH IN CHILDREN IN THE EMERGENCY DEPARTMENT
29

PAEDIATRIC RESEARCH IN THE EMERGENCY

Table 29.1.2 Types of studies

Study groups and question Study type Description Limitations Level of evidence

Descriptive
Describes the distribution of Case series Case reports without control Data often obtained IV
a certain variable such as an of interventions. May develop retrospectively with
exposure/disease/ hypothesis for later inaccurate, incomplete or
symptom, e.g. How many prospective trials measurement bias
children vomit after
intranasal fentanyl? Cohort studies Prospective or retrospective Selection bias III-2
longer term follow up of No specific interventions
patients. Can be performed
observational or case-
controlled

Cross-sectional studies At given time point population Causal relationship cannot be IV

studied to determine established
prevalence (not incidence) of
disease. Quick, relatively
inexpensive

Analytical
Evaluate associations to Randomised controlled trials Definitive method to assess Difficult to conduct and large II
discover cause and effect (RCT) effect of intervention numbers may be required
relations, e.g. What factors Allocates to intervention or
are associated with control group
vomiting after intranasal
fentanyl? Case-controlled studies Recruit disease vs non Prone to selection bias III-3
disease and assess exposure No information on risk of
status between groups. Used disease
for rare disease and
infrequently in ED

Pilot study Identifies problems with study Common euphemism for

protocol, derives mean and poorly planned study with
standard deviation for power inadequate numbers to detect
calculations planned outcome

Crossover studies In stable disease can alternate Can have high drop-out rates III-2
treatments in same patients which significantly reduces
and reduces sample size study power

Literature review
Review Summary of available May be incomplete due to
evidence inadequate search strategies
Variable use of formal
critique of evidence

Meta-analysis Re-analysis of previously Subject to limitations in Depends on studies

reported trials original study design. Only contained. If studies are
Used to attempt to find 50% of published articles level II then will be assigned
relationships not apparent in found in Medline search level I
original trials due to lack of Different studies have
numbers different methodology
Positive studies published
more than negative studies

the proposed research, and, particularly in the burden or risks and the benefits of research. to the key component of informed consent.
paediatric context, is sufficiently mature to The most obvious examples of injustice The age at which a child develops the maturity
understand the consequences of the deci- are where research benefits or risks are to understand, give informed consent or accept
sion to take part in the study. unevenly distributed amongst the wealthy risk for altruistic reasons is not predefined
The principle of beneficence includes the and poor, or conducting research exclusively by chronological age. There is variability in this,
concept of maximising possible benefits in minority populations to benefit non- based on complexity of the proposed inter-
and minimising possible harm while avoid- minority populations. vention. It is important to note that ethical
ing unnecessary burdens. Non-therapeutic standards and criteria for study participation
research or research where the risk of harm Ethics of research involving evolve over time, and what is acceptable now
is possibly greater than the risk of benefit children may be controversial or unacceptable in the
is not beneficent. Due to the exploitation of children prior to the future, just as previous standards may now
The principle of justice implies there Nuremberg Code there is added complexity be considered questionable. This is particularly
should be no inequality in sharing the relating to research in children. This relates so in relation to children.

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29.1 RESEARCH IN CHILDREN IN THE EMERGENCY DEPARTMENT

Assent by the child is a requirement used illness. This makes it more difficult to fully HREC approval is often contingent on
in some countries (e.g. USA) from the age of assess the childs maturity level and under- other processes. The hospital lawyer and
approximately 7 years for studies involving standing and to appreciate the potential hospital insurer may have to sign off on
children, although formal consent is still for researchers to apply covert pressure to high-risk projects; the trials may need regis-
required from the legal guardians. a reluctant child. Enrolment may also be cur- tration and Therapeutic Goods Administra-
There are in general four recruitment sce- tailed by parents/guardians being unwilling tion (TGA) notification may be required in
narios for children presenting to the ED who or unavailable to consent during the stress clinical drug or device studies. A study can only
may be enrolled in research studies:5 of the presentation to the ED. be commenced once HREC approval has been
obtained and only HREC approved study
Infants/toddlers without the capacity to
Ethics review process documents may be used during a study.
understand or take part in discussions
The implementation of national guidelines
regarding a research project and whose
and principles to individual projects is left
parents/guardians are approached for
to the discretion of committees adminis-
consent.
tered locally by research institutions or hos-
The practice and
Children able to understand some
pitals. Research other than low or negligible governance of research
relevant information and take part in
risk research must be reviewed by a local Well-conducted clinical research is far more
limited discussion about the research,
ethics committee. Ethics committees are likely to discover the truth. There are basic
but whose consent is not required. Only
usually composed of representatives from professional standards in the conduct of
parent/guardian consent is required for
clinical (doctors, nurses etc), research and research which must be met during this
these children.
community (lay people and clergy) groups.
 Young people of developing maturity, process.
All research must be approved in writing
able to understand the relevant
by the sponsoring institution before it can
information but whose relative Research documents
commence. Research requiring Human
immaturity means they remain Good documentation is a key to a successful
Research and Ethics Committee (HREC)
vulnerable. The consent of these young study. Documents should be kept together,
approval may not start until there is clear
people is required, but is not sufficient to be complete, dated, and secured. There
written approval from the committee.
authorise research, therefore requiring should be a clear trail of all data from the
The primary objectives of an HREC are to
additional parent/guardian consent. point of data collection, collation into data-
assess the ethical principles by which
Young people mature enough to base through to publication. This trail may
research projects in humans are proposed
understand and consent, and not be scrutinised if there is any question about
and conducted, protecting the welfare and
vulnerable through immaturity in ways the veracity of a research finding. Clear doc-
rights of the research participants and to
that warrant additional consent from a umentation is also essential to reduce the
facilitate research that is or will be of benefit
parent or guardian. chance of error and to keep the project run-
to the researchers community or to human-
ning smoothly.
As children have limited capacity to consent, kind. HRECs may also consider all matters
the degree of risk they can be exposed to relating to project design, technical feasibil-
needs careful consideration. In general, risk ity and any other ethical implications asso- Research protocol
is classified in degrees, and the degree of risk ciated with each project. The ethics review The key document for any study is the
acceptable depends on the importance of process is not perfect and may be frustrating research protocol. It sets out why a trial
the study, and the likelihood of any direct but a wise researcher engages with the should be run, acts as an operations manual
benefit to the child. For example, the risk ethics committee in a positive and helpful and is the scientific design document for the
of complications from placing an intrave- manner. trial. It is submitted to the HREC at the time
nous cannula may be unacceptable in a Many small projects can be undertaken as of approval application along with the case
child when studying a minor condition such clinical audits and would be regarded by the report forms, patient information statement
as otitis media but acceptable in the setting community as an essential part of clinical and consent form. The research protocol out-
of determining the effectiveness of a chemo- practice, such as an analysis of the types lines in detail the research question being
therapy agent. In order that some important of clinical presentations that did not wait asked, background and rationale for the
research involving children may legitimately to be seen. These projects should still be pre- study, the design and methodology by which
be carried out, most jurisdictions deem that sented to the HREC, but in most institutions the question will be addressed, secondary
it is acceptable to have some degree of risk should undergo an expedited review, with- objectives, primary and secondary outcomes,
for the child who is unable to consent where out the need for a full ethics submission statistical considerations including sample
there is no direct benefit accruing to that and the consequent delay. In this circum- size and power calculations. There should
child, provided certain limitations are stance, it is still important to plan the also be a discussion of any ethical implica-
adhered to. research project fully, as the project will tions of the study being undertaken. Tem-
In addition, in the ED setting there is the not be of benefit to anyone if a researcher plates for protocols can be found on the
added complexity relating to recruitment for has not collected and analysed the data in websites of a number of organisations such
a study at the time of an acute injury or a systematic and rigorous manner. as the TGA.6

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PAEDIATRIC RESEARCH IN THE EMERGENCY

Case report forms (CRF) and/or technicians, health economists, trial Key regulatory documents
The CRF is designed to record all of the coordinators, data manager, data entry staff The National Statement on Ethical
required information as defined in the proto- and research nurses or assistants. Conduct in Human Research5
col for accurate analysis. Badly designed Larger studies may require several groups The peak medical research bodies in Australia
CRFs negatively affect the quality of data or committees such as a trial management (NHMRC, Australian Research Council (ARC)
analysis and the ability of the project to group (to manage the trial on a day-to-day and the Australian Vice Chancellors Commit-
answer the research question. CRFs should basis), trial steering committee, data moni- tee (AVCC)) produce this joint statement which
be designed concurrently with the protocol, toring and/or safety committee and data is Australias primary source of guidance pro-
preferably with input from a biostatistician management committee. These committees moting ethically sound review and conduct
and should be piloted prior to study com- should have regular minuted meetings. of human research with national standards
mencement. All data collected for the study to guide institutions, researchers and HRECs.
should be recorded directly, promptly, accu- Databases and analysis
rately and legibly. Good data management should be carefully
planned from before the first data are col- International Conference on
Patient information statement lected though to the analysis of data; other- Harmonisation of Good Clinical
and consent form wise implausible values and inconsistencies Practice guidelines (ICH-GCP)10
In order to gain informed consent from a are carried through to the analysis phase, This is an international document accepted
potential research participant, research pro- where it is very difficult to correct errors. A in Australia. It was developed through a
jects require patient information statements database is a computer software program drive to regulate requirements for a single
in plain language and consent forms that (e.g. EpiData) where data is entered and market for pharmaceuticals. ICH-GCP is
must be approved by the ethics committee stored in a table or file, which is called the also known as GCP or GCRP (good clinical
prior to their use. They are intended to outline data file. Each study patient should have a research practice). It is an overarching
the proposed study in language lay people unique identifier different from the patients ethical and quality standard for the design,
can understand. Good patient information hospital number to ensure confidentiality. conduct, performance, recording, analysis,
statements are difficult to write and poorly Even for simple databases, a coding manual monitoring, auditing and reporting of clini-
written ones may cause delays in obtaining should be created to explain variable names, cal research. It also includes protection of
ethics approval.7 describe the variables and their units, spec- human rights as a subject in clinical trial.
ify the number of decimals, etc. Studies are
often performed over a period of time and
Study document file what seems obvious at the beginning of Australian Code for the Responsible
The study document file should be kept in the process may become cryptic at the anal- Conduct of Research11
the ED administrative area and contain ysis stage. Data must be kept securely with Having been jointly authored by ARC, AVCC,
copies of all documentation relevant to password protection and limited access by and NHMRC, this code is designed to guide
the study in an orderly and systematic nominated and HREC-approved researchers, institutions and researchers in how to achieve
fashion. Good data handling and record and be regularly backed up. and maintain responsible research practice.
keeping ensures a paper and electronic trail All research in Australia must abide by
that testifies to the accuracy of the reported Reporting guidelines the National Statement. The National State-
data, as a study may be audited even years The reporting of clinical trials has a recom- ment requires that research is conducted in
after it has finished. mended, standardised approach, outlined accordance with the Australian Code of Con-
by the CONSORT Statement, an evidence duct. However, this document does not pro-
The research team based format aimed at improving the qua- vide an exhaustive description of how to
Research is a team effort, even for a small lity and integrity of reporting for RCTs. conduct research. The TGA has published
trainee-led project. Good research practice CONSORTconsists of a checklist and flow dia- a more detailed document describing how
involves having an effective team of resear- gram for reporting a RCT.8 The flow diagram research should be performed in Australia
chers and collaborators (such as nurses, provides readers with a clear picture of the which in turn requires that researchers fol-
junior doctors, allied health workers) with a progress of all participants in the trial, from low relevant sections of the ICH GCP.10
clear understanding of who is responsible the time they are randomised until the end The aim of these documents is not only to
for what and who reports to whom. Even in of their involvement. Both documents are increase the scientific quality and hence the
small studies, biostatisticians should be also very helpful at the design stage of the veracity of findings but also to ensure
involved early in the design stage. They will trial and ensure that the research protocol research is conducted in an ethically respon-
help guide researchers to determine study is comprehensive and logical. sible manner and that the findings are verifi-
design and sample size in addition to their There are also a number of reporting able. Being compliant with these documents
role in database creation and data analysis. guidelines for other types of studies. Infor- assures the public that the data and reported
Depending on the size and type of study, mation about reporting guidelines, includ- results are credible and accurate and that the
teams may include, in addition to clinicians, ing key checklists and flow diagrams, is rights, integrity and confidentiality of human
expert researchers, statisticians, pharmacists listed at the EQUATOR website.9 research subjects have been protected.

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Project registration
Funding research Expansion of cross-linkages between
Since July 2005 all clinical trials must be
international research networks is being
registered on a Clinical Trials Register before Finding funding for research is a challenge. developed and should further enhance
the enrolment of the first participant. The In Australia the major large sources are the dissemination of evidence based
guidelines of the International Committee ARC and NHMRC grants, with smaller dis- emergency medicine.
of Medical Journal Editors (ICMJE) state that ease- and specialty-specific competitive
any trial must be registered in order to be funding bodies such as Diabetes Australia
published in any of their comprehensive list Research Trust, National Heart Foundation,
of journals.12 The purpose of trial registration and so on. Obtaining such funding requires
is a greater efficiency by reducing unneces- a robust research question and study design,
sary duplication of research effort, better
References
and a strong track record. Evidence of seed 1. National Health and Medical Research Council of
compliance, and a greater assurance that funding and pilot data is also useful. Seed Australia. Levels of Evidence Guidelines. Available from:
all clinical trials reports are reported, includ- funding may come from professional socie- http://www.nhmrc.gov.au/guidelines/consult/
consultations/add_levels_grades_dev_guidelines2.htm
ing those with negative results. There are ties, research institutions and private prac- [accessed 29.10.10].
several clinical trial registers worldwide tice funds. Infrastructure support may 2. National Institutes of Health. Office of Human Subjects
Research. Regulations and Ethical Guidelines. Available
including the Australian and New Zealand come from the hospitals, universities or from:http://ohsr.od.nih.gov/guidelines/
Clinical Trial Registry (ANZCTR).13 research institutes. Philanthropic funds are nuremberg.html [accessed 29.10.10].
3. World Medical Association Declaration of Helsinki.
also available, though these are increasingly Ethical Principles of Medical Research involving Human
Privacy and confidentiality competitive. Collaboration with other suc- Subjects. Available from:http://www.wma.net/en/
30publications/10policies/b3/index.html [accessed
Clinical research almost invariably involves cessful groups and researchers is another 29.10.10].
the use of personal information. Patients key to funding success. 4. National Institutes of Health. Office of Human Subjects
Research. Regulations and Ethical Guidelines. Available
provide information with the expectation from:http://ohsr.od.nih.gov/guidelines/belmont.html
that it will be treated confidentially and [accessed 29.10.10].
5. National Health and Medical Research Council.
the unauthorised disclosure of such informa- Controversies and future Australian Research Council. Australian Vice Chancellors
tion may be a risk to the subject which directions Committee. National Statement on Ethical Conduct in
Human Research. Available from:http://www.nhmrc
should be considered in any research project.
Similar to medical records in general, patient
Trainees are expected to undertake .gov.au/PUBLICATIONS/ethics/2007_humans/
contents.htm [accessed 29.10.10].
research during their training. Due to 6. Therapeutics Goods Administration. Available from:www.
privacy laws apply to research records. tga.gov.au/docs/pdf/euguide/ich/ich13595.pdf
time constraints this encourages low [accessed 29.10.10].
level studies and biases against RCTs 7. Green JB, Duncan RE, Barnes GL, Oberklaid F. Putting the
informed into consent: a matter of plain language.
or Systematic Reviews. Supervisors
Multicentre research should offer research training for
J Paediatr Child Health 2003;39(9):7003.
8. CONSORT group. The CONSORT Statement. Available
from:http://www.consort-statement.org/consort-
Some of the difficulties in emergency trainees. statement/ [accessed 29.10.10].
research in children, such as the low fre-
Most public hospital departments
9. EQUATOR Network. Introduction to reporting guidelines.
Available from: http://www.equator-network.org/
quency of major outcomes or limited appli- resource-centre/library-of-health-research-reporting/
are poorly resourced both financially and
cability of findings, can be overcome by reporting-guidelines/ [accessed 20.10.10].
with research-specific personnel to 10. International Conference on Harmonisation of Technical
co-operating with other institutions. This Requirements for Registration of Pharmaceuticals for
undertake research in emergencies in
recognition has led to a number of co- Human Use. ICH Guidelines. Available from:http://www.
children. There is a pressing need for the ich.org/cache/compo/276-254-1.html [accessed
operative research networks, initially in North 29.10.10].
development of university departments
America (Pediatric Emergency Research 11. National Health and Medical Research Council.
of paediatric emergency medicine. Australian Research Council. Australian Vice Chancellors
Canada (PERC) and Pediatric Emergency Committee. Australian Code for the Responsible Conduct
Care Applied Research Network (PECARN)),  The development of cross linkages of Research. 2007. Available from: http://www.nhmrc.
gov.au/_files_nhmrc/file/publications/synopses/
in Australia and New Zealand (Paediatric through research networks such as r39.pdf [accessed 29.10.10].
Research in Emergency Departments Inter- PREDICT has helped build capacity in 12. International Committee of Medical Journal Editors.
Uniform Requirements for Manuscripts Submitted to
national Collaborative (PREDICT)) and now emergency departments. It has also Biomedical Journals: Publishing and Editorial Issues
also in Europe (Research in European Paedia- provided increased exposure of Related to Publication in Biomedical Journals: Obligation
to Register Clinical Trials. Available from:http://
tric Emergency Departments (REPEDS)). paediatric emergency research to www.icmje.org/publishing_10register.html [accessed
These networks have increased the profile funding agencies which may be the basis 29.10.10].
13. Australian and New Zealand Clinical Trials Registry.
of paediatric emergency medicine, illustrated for developing high level research Available from:www.anzctr.org.au; [accessed 20.10.10].
the similarities and differences in practice studies with dissemination of both 14. Kuppermann N, Holmes JF, Dayan PS, et al. Identification
of children at very low risk of clinically-important brain
across geographical areas and are increasing research projects and their results into injuries after head trauma: a prospective cohort study.
the evidence base for interventions.14 more emergency departments. Lancet 2009;374(9696):116070.

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SECTION
ADOLESCENT
MEDICINE IN THE
EMERGENCY
DEPARTMENT
Section editor Ian Everitt

30.1 Adolescent medicine in the emergency

department 599

30.1 Adolescent medicine in the emergency

department
Katherine Barton Donald Payne

think of adolescence as a process during

ESSENTIALS which an individual moves from being a
dependent child to an independent adult.4
1 Establishing rapport is essential for a successful adolescent health consultation. The developmental changes that occur
2 Interviewing adolescents in a quiet, more private setting, removed from the main during adolescence include the obvious
ED, is a great advantage. physical changes of growth and puberty as
well as the less well-recognised cognitive
3 It is important to see adolescents on their own. and social changes. A key task of adoles-
4 Using the HEADSS framework as an aide-memoire will help exploration of relevant cence is for individuals to establish their
psychosocial issues. own identity and self-image. This involves
developing independence from parents,
5 Explanation of confidentiality is essential. forming relationships outside the family,
6 Discussion should be professional and in language the young person is able to challenging authority and experimenting
understand. with different behaviours, some of which
can pose a health risk. Health professionals
working with adolescents need to acknowl-
edge this process and be aware of the
adolescents usually reflects a lack of impact of emerging adolescent behaviours
Introduction training in this area.2 However, as with on health outcomes, as well as the
Many health professionals find working any area of medicine, specific training effect of illness on normal adolescent
with adolescents challenging. Communi- can lead to an increase in clinicians com- development.4,5
cation can be difficult, the priorities of ado- petence and confidence in dealing with
lescents are different to those of adults adolescents.3 Keys to working effectively
and issues of consent, confidentiality and with this group include developing confi-
Adolescent health problems
privacy take on particular significance.1 Ado- dence in talking to adolescents and
understanding the concept of adolescent
in the ED
lescents also take time, which is not always
readily available in the emergency depart- development. Whether the setting is an adult, mixed or
ment (ED). The perceived discomfort that Although different age ranges have paediatric ED, all emergency doctors are
some professionals experience working with been proposed, it is more appropriate to likely to see adolescents in their daily

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practice. The leading causes of death among ability to see adolescents in a quiet, more
Table 30.1.2 HEADSS
adolescents and young adults are motor private setting, removed from the main busi-
H Home
vehicle accidents and suicide.6 The most ness of the ED, is a great advantage. Where do you live? Who lives with you?
common causes of morbidity include injuries How do you get on with the people you live
with?
(both intentional and non-intentional), men- Seeing adolescents alone Who would you talk to if you had a problem?
tal health problems, drug and alcohol misuse It is important to see adolescents on their E Education (or employment)
Which school do you go to? Which year are
and sexual health problems (Table 30.1.1). own, separate from parents, for at least part you in?
In addition, the number of adolescents and of the consultation. This helps to establish Which subjects do you enjoy? What are you
good at?
young adults growing up with chronic dis- rapport and trust and optimises the chances Who do you spend time with at school? What
eases of childhood is increasing as a result that the young person will talk openly about are the teachers like?
A Activities
of improved treatment of these conditions. relevant issues. Adolescents often contrib- What do you enjoy doing outside of school?
Many presentations to ED for primary physi- ute little to the history when parents are Are you in any clubs or sports teams?
Who do you meet up with at weekends?
cal problems are linked with psychosocial present. However, on their own, they are D Drugs
issues or with health risk behaviours, such much more likely to open up. Parents may Do your friends smoke cigarettes or drink
alcohol? How about you?
as drug and alcohol use, unsafe sex and find it difficult to separate from the adoles- How much do you smoke/drink? Every day?
physical risk-taking. Clinicians working in cent for the health consultation. This pro- At weekends?
Have you ever tried marijuana or other drugs?
ED must therefore be aware of these under- cess is helped by explaining that it is S Sexuality
lying risk factors and feel confident in being routine practice to see adolescents on their Do any of your friends have girlfriends/
boyfriends? How about you?
able to discuss them and liaise with local own and emphasising the importance of the Have you ever had sex? Do you use condoms/
adolescent resources as needed. young person beginning to take responsibil- the pill?
S Suicide
ity for their own health. Spending time with How would you describe your mood? Do you
parents on their own afterwards (after dis- ever get really down?
Some people who feel really down often feel like
The approach to the cussing with the young person what you will hurting themselves or even killing themselves.
tell them) may alleviate their anxieties. Have you ever felt like that?
adolescent in the ED Have you ever tried to hurt yourself?
Unless the urgency of the situation dic-
Establishing rapport tates otherwise, it is helpful to begin the con-
Establishing rapport is essential for a suc- sultation by asking adolescents one or two
cessful adolescent health consultation. general questions (e.g. what school do you empathic and non-judgemental but to con-
Empathy, trust and respect are important go to? where do you live? who else is at vey concern about risk taking and its poten-
keys in any doctorpatient relationship, home?) rather than immediately focusing tially harmful consequences. It is also
but especially so for adolescents. Establish- on the medical presentation. This helps to important to frame discussions about treat-
ing rapport in ED can be challenging, but put them at ease and shows that you see ment options in language which the young
not impossible. Adolescents may present to them as a person first who happens to have person is likely to understand.4 Tailoring
an adult department, where they could find a medical problem. your approach depending on the develop-
themselves surrounded by much older mental stage of the adolescent allows you
patients, or to a paediatric department, full Using the HEADSS framework to take a thorough history more easily. For
of infants, toddlers and understandably anx- Table 30.1.2 shows the HEADSS framework, example, younger adolescents (with more
ious parents. The setting may therefore have used widely around the world, which acts as concrete thinking) respond best to simple,
a significant impact on the consultation. The a helpful guide for clinicians to use when closed questions. Older adolescents under-
interviewing adolescents.7 HEADSS begins stand more abstract concepts and can more
with relatively unthreatening questions easily answer open-ended questions and
Table 30.1.1 Health issues for about home, school and activities. These contemplate the future. This becomes
adolescents presenting to medical care
have the dual purpose of gathering informa- important when discussing adherence to
Accidents and injuries: motor vehicle accidents, tion and allowing time to develop rapport. treatment and health risk behaviours.
bicycle and pedestrian accidents, work related
injuries, falls, assaults, poisonings However, difficulties in these areas (e.g. pro- Younger adolescents are only able to com-
Mental health issues: anxiety, depression, self longed school absence, no hobbies or inter- prehend short-term consequences, whereas
harm, suicide attempts, psychosis, personality
disorders, eating disorders ests) may be a reflection of problems in older adolescents can foresee the longer-
Drug related: alcohol, illicit drug use other areas. As mentioned earlier, mental term implications of their behaviour. At
Infectious diseases: e.g. influenza,
meningococcal infection, pneumonia health problems, such as anxiety or depres- times, adolescent patients will be difficult
Chronic disease: asthma, cystic fibrosis, sion, and health risk behaviours such as to engage. This is especially so considering
diabetes mellitus, inflammatory bowel disease,
chronic pain/fatigue syndromes, allergies smoking, alcohol and other drug use are that many presentations to ED will be in
Sexual health: pregnancy, sexually transmitted common in adolescents and should always relation to sensitive issues such as psychoso-
infections
be considered. cial problems or substance abuse. It is often
Source: Australian Institute of Health and Welfare Doctors may find it difficult to communi- a situation of high stress for both the young
(AIHW) 2007. Young Australians Their Health and Well
Being 2007. Cat. No. PHE 87. Canberra. www.aihw.
cate with adolescents with regards to health person and their family. Acknowledging this
gov.au. risk behaviours. It is important to remain and demonstrating patience is important.

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30

ADOLESCENT MEDICINE IN THE EMERGENCY

Confidentiality treatment for their patients at the time of Accurate staging of puberty may be required
One of the barriers to adolescents seeking presentation. In addition, for adolescents, in certain cases. At the end of
medical care is a perceived lack of it is particularly important that their experi- the examination, the findings should be
confidentiality. Establishing confidentiality ence of health services is a positive one, explained in language the young person
is thus essential at the beginning of the ado- thereby optimising the chances that they is able to understand. If the examination is
lescent consultation. It is important to will feel confident to access appropriate normal, a simple explanation of this fact
explain that, whilst you may discuss aspects health services in the future. will be very reassuring for a young person.4
of their case with colleagues and write in
patient notes, you will not discuss things Psychosocial screening Linking adolescents with
with their parents without their permission. Adolescence is a critical time when health community follow up
It is also essential to explain the limitations behaviours are established, and doctors Given that mental health problems, health
of confidentiality. The disclosure of any can have an important role in reinforcing risk behaviours and sexual health problems
activity that puts the patient at serious positive health behaviours. Presentations are common among adolescents and young
risk of significant harm (such as suicidal to ED should be seen as an opportunity adults presenting to ED, it is useful for clin-
thoughts or physical/sexual abuse) cannot for health promotion, especially considering icians to have access to information about
remain confidential. Neither can the disclo- many young people do not have a local youth-friendly services available in the local
sure of activities that put others at risk. general practitioner.6 Although more pertain- community. These include sexual health
Clearly, establishing confidentiality at the ing to a physician consultation in an clinics, drug and alcohol centres, mental
beginning of a consultation fosters an hon- outpatient clinic setting, psychosocial health services and drop-in centres. Some
est and open communication between the screening (HEADSS) can be a very useful may require a referral, others may accept
doctor and the young person. Adolescents tool in the ED consultation. Due to the fact self-referrals. Clinicians should be able to
who are assured of some degree of confi- that many young people present with men- provide adolescents with written informa-
dentiality are more likely to speak frankly.8 tal health issues, it is important to assess the tion and website addresses. Education
In practice, this increases the chances of psychosocial factors that may be contribut- should also be considered. If appropriate
being able to address a range of health- ing to the current presentation. This is espe- (e.g. those with prolonged school absence),
related issues, such as treatment adherence, cially important when young people present an attempt should be made to link adoles-
mood and drug and alcohol use, thus open- to the emergency department with mental cents with hospital school services, where
ing up the possibility of providing effective health issues, alcohol and drug issues and they exist.10
health care. chronic unexplained problems such as pain
and fatigue. It is helpful to summarise
The mature minor principle in advance the types of questions you plan Summary
The legal age for consent to treatment var- to ask and to explain their relevance to
Adolescents require clinicians to have a dif-
ies between states within Australia.5 How- health outcomes. Normalising the process
ferent approach compared to paediatric or
ever, when working with adolescents, by explaining that you ask all adolescents
adult patients. Priorities should include pri-
regardless of their age, the mature minor these same questions can help make the
vacy, confidentiality and adequate time
principle can always be employed.9 This young person feel more at ease. Beginning
spent with the young person. The reasons
states that a young person can consent to with an explanation about confidentiality
for presenting to ED are often complex.
treatment without parental knowledge if is essential.5 Sensitive questions can be
Performing a psychosocial screen can be
they are considered to be competent to do more easily asked using a third person
very rewarding. Many young people have
so by their treating clinician. The clinician approach, such as Do any of your friends
no general practitioner and therefore ED vis-
must be confident that the young person use marijuana? before progressing to asking
its should be seen as an opportunity for pre-
understands the proposed treatment, its about the patient themselves.
ventive health screening, in addition to
benefits and risks and is able to make an
acute management. Training in adolescent
informed decision. An adolescents compe- Physical examination
health for all staff working in ED will be of
tence to consent to treatment will clearly The examination of the adolescent patient
benefit to both patients and staff.
depend on the complexity of the treatment is essentially the same as the examination
proposed. In practice it is best to try to of an adult patient, from general inspection
encourage adolescents to involve their par- and observations to a systematic approach Controversies and future
ents, or another adult whom they trust, in to each of the major systems. However, clin- directions
any treatment decision. However, some- icians must be sensitive to the developmen-
Where should adolescents and young
times adolescents will choose not to involve tal stage of the young person. Priority should
adults be seen?
their parents. In these circumstances it is be placed on privacy, and making the young
sensible for clinicians to consult with person feel comfortable. Simply explaining There is no consistent policy between
another colleague rather than taking sole what you are about to do can ease embar- different hospitals regarding the upper
responsibility for difficult decisions. Clini- rassment. It is sensible to try to find a chap- age limit for attendance at a paediatric
cians have a duty of care to provide the best erone to be present during an examination. ED. Some departments will not treat

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30.1 ADOLESCENT MEDICINE IN THE EMERGENCY DEPARTMENT