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Croup
Synonyms: acute laryngotracheitis, acute laryngotracheobronchitis Croup is a common childhood illness caused by inflammation of the upper respiratory tract (predominantly the larynx and trachea, but it may affect the bronchi) as a result of viral infection. Some consider that the term croup should not be used to describe illness affecting the bronchi, as there is a higher likelihood of secondary bacterial infection in such cases. Croup tends to be relatively mild and self-limiting, but can cause distressing symptoms such as a barking cough and stridor that may prompt parents to bring their child to their GP or local emergency department. Severe cases may compromise the upper airway and so the condition of the child needs to be assessed carefully and other causes of upper airway obstruction (such as inhaled foreign body and epiglottitis) must be considered and excluded.

Pathophysiology
Viral upper respiratory tract infection (URTI) causes nasopharyngeal inflammation that may spread to the larynx and trachea, causing subglottal inflammation, oedema and compromise of the airway at its narrowest portion. The movement of the vocal cords is impaired leading to the characteristic cough. Occasionally, fibrinous exudation with pseudomembrane formation may occur, causing further airway compromise. It is thought that some children who suffer recurrent bouts of spasmodic croup have a primarily allergic rather than infective aetiology for subglottal oedema. [1]

Causative organisms
Parainfluenza virus types I, II, III and IV (thought to be responsible for about 80% of cases - type I causing 50-70% of severe cases). [1] Respiratory syncytial virus. Adenoviruses. Rhinoviruses. Enteroviruses. Measles. Metapneumovirus. Influenza A and B (type A is associated with severe disease). Mycoplasma pneumoniae (rare cause).

Epidemiology
Croup most often affects children aged 6 months to 3 years, with a peak incidence during the second year of life. However, children as young as 3 months of age, or adolescents and, very rarely, adults can be affected. [2] The annual incidence is variable depending on the cyclical prevalence of viral infection (particularly parainfluenza virus) in children. The incidence is about 60 per 1,000 child years in those aged between one and two years. [3]

Risk factors
Male:female preponderance is about 1.5-2:1. [1] Most common in those aged 6 months to 3 years. Rare in those older than 6 years of age, but can affect some children up to the early teenage years. Most prevalent in autumn and spring.

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Presentation
Croup normally starts with nonspecific symptoms of viral URTI, such as runny nose, sore throat, fever and cough. This progresses over the course of a couple of days to include the characteristic barking cough and hoarseness. These symptoms tend to be worse at night. There is a high degree of variability in clinical findings. There may be a mild-to-moderate fever. Check vital signs (including temperature, pulse and blood pressure). A barking cough and hoarse cry are nearly always present. Stridor (harsh, low-pitched noise heard during inspiration) may be heard at rest or only when the child is agitated or active. Chest sounds are usually normal but can be decreased in volume where there is severe airflow limitation. Respiratory distress with marked tachypnoea and intercostal recession may be noted. A child who appears to be deteriorating but whose stridor appears to be improving and intercostal recession disappears has worsening airways obstruction and is at high risk of complete airway occlusion. [2] Drowsiness, lethargy, cyanosis despite increasing respiratory distress should be considered as red flags for impending respiratory failure. The illness tends to last for about 3-7 days but can persist for up to two weeks.

Differential diagnosis
Epiglottitis. Inhaled foreign body. Inhaled noxious substance. Acute anaphylaxis. Bacterial tracheitis. Diphtheria. Laryngomalacia or another congenital cause of upper airway stenosis (eg aortic arch abnormality causing external airway compression). Peritonsillar abscess (quinsy). Retropharyngeal abscess. Angioneurotic oedema. Laryngeal mucosal lesions such as laryngeal web, papillomata and haemangioma. Vocal cord paralysis.

Assessment of severity
There are many clinical scoring systems for croup. The most commonly cited is the Westley clinical scoring system which classifies cases into mild, moderate or severe. [1] It appears to be useful as a research tool to assess the efficacy of interventions, but there is little proof of its clinical usefulness. [1] The classification of symptoms it uses can be subjective and open to inter- and intraobserver variation. An overall assessment of the patient's condition, taking into account the red flags listed above, SaO2 readings and the degree of respiratory distress, may be an equivalent and simpler guide to deciding what therapy is required.

The modified Westley clinical scoring system for croup

Inspiratory stridor:
Not present - 0 points. When agitated/active - 1 point. At rest - 2 points.

Intercostal recession:
Mild - 1 point. Moderate - 2 points. Severe - 3 points.

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Air entry:
Normal - 0 points. Mildly decreased - 1 point. Severely decreased - 2 points.

Cyanosis:
None - 0 points. With agitation/activity - 4 points. At rest - 5 points.

Level of consciousness:
Normal - 0 points. Altered - 5 points.

Possible score 0-17: <4 = mild croup, 4-6 = moderate croup, >6 =severe croup.

Hospital admission [2]

Most children will have mild croup, which can be managed at home. Immediate admission to hospital is required for: Moderate or severe croup, or impending respiratory failure. Any suspicion of epiglottitis, bacterial tracheitis, peritonsillar abscess, retropharyngeal abscess, or laryngeal diphtheria. Any suspicion of inhaled foreign body, angioneurotic oedema, hypocalcaemic tetany, or ingestion of corrosives. Consider admission to hospital if any of the following are present: History of severe obstruction, previous severe croup, or known structural upper airways abnormalities (eg laryngomalacia, tracheomalacia, vascular ring, Down's syndrome). Age less than 6 months. Immunocompromised. Inadequate fluid intake. Poor response to initial treatment. Uncertain diagnosis. Significant parental anxiety, late evening or night-time presentation, the child's home is a long way from the hospital, or the parents have no transport.

Investigations
The diagnosis is usually made on clinical grounds but the following investigations may be indicated: A low SaO 2 on pulse oximetry (<95%) indicates significant respiratory impairment. FBC may reveal a viral pattern differential white cell count, but is rather nonspecific. Viral titres are used on occasion to decide whether patients should be treated in isolation, or to indicate a need for antiviral therapy in the case of influenza A. X-rays: Plain CXR can exclude other causes such as inhaled foreign body, epiglottitis, bacterial tracheitis or retropharyngeal abscess. Postero-anterior CXR, including the neck area, may reveal the 'steeple sign', which is the presence of a steeple-shaped narrowing of the darker area caused by air in the upper trachea, revealing the subglottal narrowing of the airway. Follow the link to the next reference for an image of this sign. [1] Lateral neck radiographs may also be employed to demonstrate a distended hypopharynx during inspiration. [4] These X-ray findings are not very specific or sensitive, with a significant false-positive rate, and up to 50% of croup sufferers do not have these findings.

Page 4 of 6 Direct or indirect laryngoscopy is not usually required but may be employed where the course of the illness is atypical or there is reason to suspect a congenital or other alternative cause for upper airway obstruction.

Management
Do not give antibiotics unless there are sound clinical reasons to suspect secondary bacterial infection. Keep the child as calm and as comfortable as possible. Allow the child to remain in a parent's arms and avoid any unnecessary painful interventions. Persistent crying increases oxygen demands and respiratory muscle fatigue and worsens the obstruction. [1] Use paracetamol or ibuprofen to control fever. Ensure an adequate fluid intake. Do not advise humidified air (eg steam inhalation). [2] A systematic review of its efficacy in secondarycare emergency settings has not shown any evidence of benefit. [3] Inpatient care includes oxygen therapy if required to maintain SaO2 above 93%.

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Steroids
Systematic reviews of the use of systemic dexamethasone and nebulised budesonide have shown that they are effective in relieving the symptoms of croup as early as six hours after treatment. [5] Treated patients have fewer re-attendances or hospital admissions, and those who are admitted require shorter inpatient stays. [6] Mild croup is largely self-limiting, but treatment with a single dose of a corticosteroid (eg dexamethasone 150 micrograms/kg) by mouth may be of benefit. [7] More severe croup (or mild croup that might cause complications) requires hospital admission. A single dose of a corticosteroid (eg dexamethasone 150 micrograms/kg or prednisolone 1-2 mg/kg by mouth) should be administered before transfer to hospital. [7] In hospital, dexamethasone 150 micrograms/kg (by mouth or by injection) or budesonide 2 mg (by nebuliser) will often reduce symptoms. The dose may need to be repeated after 12 hours if necessary. [7]

Epinephrine (adrenaline) [1]

Nebulised epinephrine (adrenaline) is usually reserved for patients in moderate-to-severe distress. Nebulised epinephrine (adrenaline) solution 1 in 1,000 (1 mg/mL) should be given with close clinical monitoring in a dose of 400 micrograms/kg (maximum 5 mg) repeated after 30 minutes if necessary. [7] The effects of nebulised epinephrine (adrenaline) last 2-3 hours and the child needs to be monitored carefully for any recurrence of the obstruction. [7]

Complications [1]
Bacterial superinfection may result in pneumonia or bacterial tracheitis. The most frequent organism is Staphylococcus aureus, followed by group A streptococcus , Moraxella catarrhalis, Streptococcus pneumoniae, Haemophilus influenzae and anaerobes. Pulmonary oedema, pneumothorax, lymphadenitis and otitis media have also been reported. Inability to maintain adequate fluid intake may lead to dehydration.

Prognosis
Complications are generally rare and case series show that <5% of children require hospitalisation and <2% of those admitted need intubation and ventilatory support. [1] Rarely, secondary bacterial infection can lead to pneumonia or tracheitis. Pulmonary oedema and pneumothorax are seen extremely rarely as sequelae. Mortality rates in intubated children are around 0.5%. [1]

Further reading & references

1. 2. 3. 4. 5. 6. Defendi GL et al, Croup, Medscape, Oct 2011 Croup, Prodigy (September 2008) Moore M, Little P; Humidified air inhalation for treating croup. Cochrane Database Syst Rev. 2006 Jul 19;3:CD002870. Desai A, Imaging in Croup, Medscape, May 2011 Bjornson CL, Johnson DW; Croup. Lancet. 2008 Jan 26;371(9609):329-39. Russell KF, Liang Y , O'Gorman K, et al; Glucocorticoids for croup. Cochrane Database Syst Rev. 2011 Jan 19;(1):CD001955. 7. British National Formulary

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Original Author: Dr Sean Kavanagh Last Checked: 20/02/2012 Current Version: Dr Colin Tidy Document ID: 1584 Version: 23 Peer Reviewer: Dr Helen Huins EMIS

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