Semionov Imaging of Thoracic Wall

Download as pdf or txt
Download as pdf or txt
You are on page 1of 13

Pictorial Essay | Thoracic Imaging

eISSN 2005-8330
https://doi.org/10.3348/kjr.2019.0181
Korean J Radiol 2019

Imaging of Thoracic Wall Abnormalities


Alexandre Semionov, MD1, John Kosiuk, MD2, Amr Ajlan, MD3, Federico Discepola, MD4
1
Department of Diagnostic Radiology, McGill University Health Centre, Montreal General Hospital, Montreal, Canada; 2Department of Diagnostic
Radiology, McGill University Health Centre, Montreal, Canada; 3Department of Radiology, King Abdulaziz University, Faculty of Medicine, Jeddah,
Saudi Arabia; 4Department of Radiology, Jewish General Hospital, Montreal, Canada

Identification of certain abnormalities of the chest wall can be extremely helpful in correctly diagnosing a number of syndromic
conditions and systemic diseases. Additionally, chest wall abnormalities may sometimes constitute diagnoses by themselves.
In the present pictorial essay, we review a number of such conditions and provide illustrative cases that were retrospectively
identified from our clinical imaging database. These include pentalogy of Cantrell, Klippel-Feil syndrome, cleidocranial dysplasia,
Poland syndrome, osteopetrosis, neurofibromatosis type 1, Marfan syndrome, Gardner syndrome, systemic sclerosis, relapsing
polychondritis, polymyositis/dermatomyositis, ankylosing spondylitis, hyperparathyroidism, rickets, sickle cell anemia,
thalassemia, tuberculosis, septic arthritis of the sternoclavicular joint, elastofibroma dorsi, and sternal dehiscence.
Keyword: Chest wall abnormalities

INTRODUCTION abnormalities can provide clues to the underlying disease


and thus aid in establishing the correct diagnosis. Tables 1
The chest wall is commonly defined as a protective and 2 provide a summary of several such conditions, which
structure around vital organs between the neck and the are discussed and illustrated below.
abdomen and is composed of the skin, subcutaneous
fat, muscles, and bones. The latter include ribs and the Congenital Diseases
sternum, spine, and shoulder girdles. The chest wall can
be readily assessed by conventional radiography, computed Pentalogy of Cantrell
tomography (CT), magnetic resonance imaging (MRI), and The hallmark of this syndrome is an omphalocele
nuclear medicine studies. Each of these modalities has its associated with ectopia cordis. The complete spectrum
own advantages and specific indications—radiography is of the pentalogy consists of deficiency of the anterior
readily available and cheap, CT provides superior resolution diaphragm, midline supraumbilical abdominal wall defect,
of bone structures, MRI offers more detailed assessment of defect in the diaphragmatic pericardium, congenital
the soft tissues, and nuclear medicine allows for detection intracardiac abnormalities, and defects of the sternum (1).
of metabolically active lesions. A number of syndromic However, only a few patients with the full spectrum of the
conditions and systemic diseases can affect the chest wall. pentalogy have been described. Anterior body wall defects
In certain cases, recognition of characteristic chest wall usually include complete or partial absence of the sternum
(Fig. 1). Extrathoracic anomalies may include cleft lip or
Received March 11, 2019; accepted after revision June 10, 2019.
Corresponding author: Alexandre Semionov, MD, Department of palate, encephalocele, hydrocephalus, gallbladder agenesis,
Diagnostic Radiology, McGill University Health Centre, Montreal and polysplenia (1).
General Hospital, 1650 Cedar Avenue Montreal, Quebec H3G 1A4,
Canada.
• Tel: (1514) 934-1934 • Fax: (1514) 934-8263 Klippel-Feil Syndrome
• E-mail: alexandre.semionov@mail.mcgill.ca Klippel-Feil syndrome (KFS) is characterized by
This is an Open Access article distributed under the terms of congenital fusion of two or more cervical vertebrae. The
the Creative Commons Attribution Non-Commercial License
(https://creativecommons.org/licenses/by-nc/4.0) which permits
classical clinical findings of KFS are a short neck, low
unrestricted non-commercial use, distribution, and reproduction in posterior hairline, and reduced range of cervical motion
any medium, provided the original work is properly cited. (2). However, less than half of all individuals with KFS

Copyright © 2019 The Korean Society of Radiology 1


Semionov et al.

Table 1. Most Common Imaging Findings of Selected Congenital Pathologies with Chest Wall Involvement
Chest Wall Findings Additional Imaging Findings
Deficiency of anterior diaphragm. Defect in
Pentalogy of Cantrell Defect in lower sternum or absent sternum diaphragmatic pericardium. Ectopia cordis. VSD. ASD.
TOF
Absent or deformed ribs. Fusion of 2 or more
Klippel-Feil syndrome vertebrae. Scoliosis. Vertebral instability, Sprengel Spina bifida occulta. Congenital heart defects
deformity–elevated scapula. Omovertebral bone
Multiple wormian bones. Brachycephaly.
Hypoplastic or absent clavicles. Bell-shaped ribcage. Supernumerary teeth. Basilar invagination. Multiple
Cleidocranial dysplasia
Supernumerary ribs hemivertebrae. Hypoplastic iliac bones. Hypoplastic or
absent fibula and radius
Unilateral absence of pectoralis muscles. Absent or
Poland syndrome Ipsilateral syndactyly
hypoplastic ipsilateral ribs, breast and nipple
Abnormally dense ribs, shoulder girdles and spine. Generalized abnormally increased bone density,
Osteopetrosis
“Sandwich” vertebrae resulting in “bone in bone” appearance
Cutaneous neurofibromas. Scoliosis, posterior
vertebral scalloping, enlarged neural foramina. Plexiform neurofibromas–mediastinal or pleural
Neurofibromatosis type 1
“Ribbon” ribs. Rib notching. Lateral and anterior masses. Bowing and pseudoarthrosis of long bones
meningoceles
Annuloaortic ectasia. Aortic valve insufficiency. Aortic
Scoliosis. Pectus excavatum or pectus carinatum.
Marfan syndrome aneurysm. Aortic dissection. Mitral valve prolapse.
Dural ectasia
Pulmonary artery dilatation
Colonic adenomatous polyps or adenocarcinoma.
Gastric and small bowel polyps. Duodenal carcinoma.
Gardner syndrome Multiple or recurrent desmoid tumours of chest wall
Thyroid cancer. Osteomas. Dental abnormalities.
Epidermoid cysts
ASD = atrial septal defect, TOF = tetralogy of Fallot, VSD = ventricular septal defect

have all three features. One of the commonly encountered Poland Syndrome
features of KFS is the Sprengel deformity, which appears Poland syndrome is a rare sporadic condition characterized
as an elevated scapula on chest radiographs (Fig. 2). The by congenital unilateral absence of the pectoralis muscles.
Sprengel deformity is occasionally concomitant with an It may also be associated with absent or hypoplastic
abnormal omovertebral bone that runs from the cervical or ipsilateral ribs, breast and nipple, and ipsilateral syndactyly
dorsal spine to the scapula. Other anomalies reported in (4). On chest radiography, Poland syndrome manifests as a
association with KFS include scoliosis, spina bifida occulta, hyperlucent hemithorax caused by asymmetry of the chest
absent or deformed ribs, and congenital heart defects (2). wall (Fig. 4A). Cross-sectional imaging can demonstrate
hypoplasia or aplasia of the pectoral muscles (Fig. 4B) and/
Cleidocranial Dysplasia or of the breast and ribs.
Cleidocranial dysplasia (CCD) is an autosomal
dominant skeletal dysplasia characterized by incomplete Osteopetrosis
intramembranous ossification of the bones (3). The Osteopetrosis (OP) refers to a group of hereditary
thoracic manifestations of CCD include hypoplastic or diseases characterized by the failure of osteoclasts to resorb
absent clavicles, a narrow, bell-shaped ribcage, multiple bone. As a consequence, bone remodeling is impaired. This
hemivertebrae, and, occasionally, supernumerary ribs (Fig. results in abnormally dense but fragile bones. In addition
3). The extrathoracic findings can include multiple Wormian to pathological fractures, OP can result in hematopoietic
bones, brachycephaly, supernumerary teeth, basilar insufficiency, hypocalcemia, disturbed tooth eruption,
invagination, hypoplastic iliac bones, and hypoplastic or nerve entrapment syndromes, and growth impairment (5).
absent fibula and radius (3). Radiographs in OP patients that include the spine will

2 https://doi.org/10.3348/kjr.2019.0181 kjronline.org
Thoracic wall Abnormalities

Table 2. Most Common Imaging Findings of Selected Acquired Pathologies with Chest Wall Involvement
Chest wall Findings Additional Imaging Findings
Collagen vascular diseases
Esophageal dilatation. NSIP or UIP. Flexion
Subcutaneous dystrophic calcifications, commonly
Systemic sclerosis deformities and soft tissue atrophy of hands.
around SCJs and shoulders
Acroosteolysis
Tracheal stenosis. Tracheal wall thickening and
Thickening of costal cartilages. Non-erosive
Relapsing polychondritis calcification with sparing of posterior membrane.
arthropathy of spine
Non-erosive arthropathy of appendicular skeleton
Fatty atrophy of involved muscles. Dystrophic Diaphragmatic elevation/basilar atelectasis.
Polymyositis/dermatomyositis
calcifications in muscles and soft tissues Aspiration pneumonia. NSIP. COP
Spondyloarthropathies
Syndesmophytes. Enthesitis of interspinal
ligaments. Ankylosis of facet, costovertebral and Upper lobes fibro-bullous disease. Apical fibrosis.
Ankylosing spondylitis
costotransverse joints. Chance-type insufficiency Ankylosis of SIJs
fractures
Metabolic, storage, and deposition diseases
Resorption of distal clavicles. “Rugger-jersey” Bone resorption. Osteopenia. Brown tumors. Soft
Hyperparathyroidism
spine tissue calcifications
Widening of growth plates. Metaphyseal flaring,
“Rachitic rosary”–nodular enlargement of
Rickets cupping and fraying. Osteopenia. Bone
costochondral junctions
deformities. Insufficiency fractures
Hematogenous disorders
Cardiomegaly. Cephalization of pulmonary vessels.
H-shaped vertebrae. Avascular necrosis of humeral Acute chest syndrome–waxing and waning
Sickle cell anemia
heads airspace opacities and pleural effusions.
Extramedullary hematopoiesis
Expansion and coarsened trabeculations of ribs
Thalassemia and vertebrae. Low T1 signal intensity of bone Extramedullary hematopoiesis
marrow
Infectious
Pott’s disease–TB spondylodiscitis, paravertebral Lymphadenopathy. Calcified granulomata. Cavitary
TB abscess; may progress to vertebral collapse, lesions, nodules, consolidations. Pleural
kyphosis, gibbus formation. Costal osteomyelitis effusion/empyema. Scarring/chronic atelectasis
SCJ effusion, widening of joint space, cortical irregularities. May progress to osteomyelitis of
Septic arthritis of
proximal clavicle and manubrium with bone erosions or sclerosis. Chest wall abscess/phlegmon and
SCJ
mediastinitis
Miscellaneous
Poorly defined, inhomogeneous soft-tissue lesions between chest wall and inferior scapular tip, with
Elastofibroma dorsi
density on CT and MRI signal similar to that of skeletal muscle. Often bilateral
Progressive post-operative displacement, rotation or fracture of sternal wires. Occasionally lucent
Sternal dehiscence strip of more than 3 mm wide along sternotomy. CT may show signs of sternal osteomyelitis and/or
mediastinitis
COP = cryptogenic organizing pneumonia, NSIP = non-specific interstitial pneumonia, SCJ = sternoclavicular joint, SIJ = sacroiliac joint,
TB = tuberculosis, UIP = usual interstitial pneumonia

feature characteristic “sandwich vertebrae,” resulting from outline.


dense bands of sclerosis parallel to the endplates (Fig. 5).
Another characteristic radiographic abnormality in OP is Neurofibromatosis Type 1
the “bone-in-bone” sign, in which abnormal bones appear Neurofibromatosis type 1 (NF1) is the most common of
to have small replicas of themselves inside their normal the phakomatoses and results in a variety of manifestations

kjronline.org https://doi.org/10.3348/kjr.2019.0181 3
Semionov et al.

Fig. 3. Cleidocranial dysplasia. Chest radiograph of 3-year-old male


with cleidocranial dysplasia shows absence of both clavicles and slanting
Fig. 1. Pentalogy of Cantrell. Chest CT scan of 25-year-old male of ribs. Expected location of clavicles is outlined by dashed line.
with Pentalogy of Cantrell, status post remote Fontan procedure for
complex congenital heart abnormality, shows absence of sternum
resulting in partial herniation of right ventricle (arrowheads). Note and extrapleural space extensively and appear as well-
extracardiac Fontan conduit (arrow), multiple mediastinal collateral marginated, smooth masses in the paravertebral regions or
vessels, and bilateral pleural effusions.
along the course of the vagus, phrenic, or intercostal nerves
(6). Classic CT findings in NF1 with thoracic involvement
include small subcutaneous nodules (neurofibromas),
thoracic scoliosis, posterior vertebral scalloping, enlarged
neural foramina, abnormally thinned ribs–”ribbon-ribs,” and
rib notching (Figs. 6, 7).

Marfan Syndrome
Marfan syndrome is an autosomal dominant inherited
disorder resulting from various mutations of the fibrillin-1
gene, which causes multisystemic connective tissue
abnormalities. Up to a third of the cases are sporadic. The
cardiovascular and musculoskeletal (MSK) systems are most
commonly involved. Potential cardiovascular manifestations
include annuloaortic ectasia, aortic aneurysm, aortic
dissection, mitral valve prolapse, and pulmonary artery
dilatation (7). Thoracic MSK manifestations include
Fig. 2. Klippel-Feil syndrome. Chest radiograph of 39-year-old scoliosis, pectus excavatum or pectus carinatum, and dural
female with Klippel-Feil syndrome shows hypoplastic right humeral ectasia (Fig. 8).
head (arrow), dysmorphic right scapula and glenoid, high-riding left
scapula–Sprengel deformity (arrowheads), and multiple upper rib
deformities. There is right ventriculo-peritoneal shunt. Gardner Syndrome
Gardner syndrome (GS) is an autosomal dominant
throughout the body. NF1 is inherited as an autosomal colonic polyposis associated with mutations arising in
dominant disorder, but up to half of the cases are caused by the adenomatous polyposis coli gene, with approximately
spontaneous mutations (6). The classic neurogenic tumors 20% of patients demonstrating de novo mutations (8).
of NF1 are plexiform neurofibromas, which arise from GS demonstrates invariable malignant transformation of
Schwann cells and fibroblasts and may affect any peripheral colonic adenomatous polyps into adenocarcinoma, along
nerve. Neurofibromas may involve the mediastinum with high rates of desmoid tumors and thyroid cancer. Other

4 https://doi.org/10.3348/kjr.2019.0181 kjronline.org
Thoracic wall Abnormalities

A B
Fig. 4. Poland syndrome. 10-year-old female with Poland syndrome.
A. Chest radiograph demonstrates hyperlucent left hemithorax. B. Chest CT with coronal reformation shows absence of left pectoral muscles. Note
normal right pectoral muscles (arrows).

Fig. 6. Neurofibromatosis type 1. Chest CT scan of 50-year-old


female with neurofibromatosis type 1 demonstrates multiple cutaneous
neurofibromas (arrowheads).

Acquired Conditions

Systemic Sclerosis
Systemic sclerosis (SS), also known as scleroderma, is
a chronic multisystem autoimmune disease of connective
Fig. 5. Osteopetrosis. Lateral chest radiograph of 14-year-old male tissue associated with vasculopathy. It is characterized
with osteopetrosis shows abnormally increased density of all bones
and characteristic “sandwich vertebrae” (arrowheads). by diffuse fibrosis of skin and various internal organs and
tissues. SS can manifest with subcutaneous calcinosis,
manifestations of GS include gastric and small bowel polyps, Raynaud’s phenomenon, esophageal dysmotility,
duodenal carcinoma, osteomas, dental abnormalities, retinal sclerodactyly, and telangiectasis (9). Interstitial lung
pigmentation anomalies, and epidermoid cysts. Multiple disease of non-specific interstitial pneumonia (NSIP)
or recurrent desmoid tumors of the body wall should alert type and pulmonary arterial hypertension are common in
clinicians to the possibility of GS (Fig. 9). patients with SS. Aspiration pneumonia occurs frequently
due to esophageal dysfunction. Subcutaneous dystrophic

kjronline.org https://doi.org/10.3348/kjr.2019.0181 5
Semionov et al.

calcifications are frequent and are most commonly seen in nasal, and laryngeal cartilages, tracheobronchial tree, heart
the fingers, but may occur in any location (Fig. 10). valves, rib cage, and joints of the appendicular skeleton.
Airway involvement is seen in half of the patients with
Relapsing Polychondritis RP, and manifests radiologically as subglottic and tracheal
Relapsing polychondritis (RP) is a rare disease stenosis, tracheal wall thickening, and calcification (10),
characterized by recurrent inflammation of cartilaginous with characteristic sparing of the posterior tracheal
structures throughout the body, including the auricular, membrane (Fig. 11). MSK manifestations of RP include non-
erosive arthropathy of the appendicular skeleton, spine, and
sacroiliac joints (10). Thickening of the costal cartilages
can be seen (Fig. 11).

Ankylosing Spondylitis
Ankylosing spondylitis (AS) is a multisystem disorder

Fig. 7. Neurofibromatosis type 1. Chest CT with coronal


reformation in 16-year-old female with neurofibromatosis type Fig. 9. Gardner syndrome. Chest CT scan of 35-year-old female
1 demonstrates multiple rib deformities and bilateral intercostal patient with Gardner syndrome demonstrates bilateral anterior chest
plexiform neurofibromas (arrows). wall lesions (arrows), which are biopsy-proven desmoid tumors.

A B
Fig. 8. Marfan syndrome.
Axial CT angiogram (A) and CT with coronal reformation (B) of 25-year-old male with Marfan syndrome show pectus excavatum (arrowheads),
scoliosis, upper lumbar dural ectasia (arrows), and aneurysm of right subclavian artery (asterisk).

6 https://doi.org/10.3348/kjr.2019.0181 kjronline.org
Thoracic wall Abnormalities

of unknown pathogenesis that primarily affects the joints human leukocyte antigen B27 (HLA-B27)-positive (9).
of the axial skeleton. It is classified as a seronegative AS most commonly manifests as bilateral sacroiliitis and
spondyloarthritis, and the majority of AS patients are spondylitis, which usually progresses from the lumbosacral
to the cervical spine. The characteristic imaging findings of
AS include anterior spondylitis, diskitis, syndesmophytes,
enthesitis of the interspinal ligaments, marked ankylosis
of the sacro-iliac joints, dorsal spine facet joints,

Fig. 11. Relapsing polychondritis. Chest CT scan of 77-year-old


Fig. 10. Systemic sclerosis. Chest CT scan of 54-year-old female male with relapsing polychondritis demonstrates marked thickening
with systemic sclerosis shows exuberant amorphous calcifications of costal cartilages (arrows), as well as thickening of tracheal wall
around left sternoclavicular joint (arrowheads) and patulous esophagus (arrowheads) with sparing of posterior membrane and tracheal luminal
(arrow). narrowing.

A B
Fig. 12. Ankylosing spondylitis.
A. CT with sagittal reformation in 76-year-old male with ankylosing spondylitis shows diffuse ankylosis of thoracolumbar spine. B. Chest CT with
sagittal reformation in different patient with ankylosing spondylitis shows acute Chance fracture in upper lumbar spine (arrow).

kjronline.org https://doi.org/10.3348/kjr.2019.0181 7
Semionov et al.

costovertebral and costotransverse joints, and insufficiency organizing pneumonia being the most frequently
fractures (Fig. 12). Insufficiency fractures of AS are typically encountered patterns. Longstanding disease might result
of the Chance-type, involving the three columns and in fatty atrophy of the involved muscles (Fig. 13). Another
running either through the disc space or the juxta-articular typical MSK finding on imaging of patients with PM/DM
endplate (Fig. 12B) (11). is dystrophic calcification in the muscles and soft tissues,
which is classically sheet-like, the so-called “calcinosis
Polymyositis/Dermatomyositis universalis” (Fig. 13).
Polymyositis/dermatomyositis (PM/DM) are idiopathic
inflammatory myopathies that manifest as muscle Hyperparathyroidism
weakness and inflammation. DM is distinguished from Hyperparathyroidism (HPT) can be primary, secondary,
PM by the presence of cutaneous manifestations (9). The or tertiary. Primary HPT is caused by parathyroid adenoma,
most common chest findings in PM/DM are diaphragmatic hyperplasia, or carcinoma. Secondary HPT is a compensatory
elevation and basilar atelectasis due to diaphragmatic mechanism for hypocalcemia that may result from vitamin
weakness, and aspiration pneumonia due to pharyngeal D deficiency, renal insufficiency, or calcium deprivation.
muscle weakness. Interstitial lung disease is seen in up to Tertiary HPT occurs secondary to the development of
one third of patients with PM/DM, NSIP and cryptogenic autonomous parathyroid hyperplasia after longstanding
secondary HPT. The skeletal changes in primary and
secondary HPT are identical. The classical radiographic
features of HPT are bone resorption, generalized osteopenia,
brown tumors, and soft-tissue calcifications (12). Classical
findings of HPT on chest radiography include a “rugger-
jersey” spine and bilateral distal clavicular subperiosteal
bone resorption (Fig. 14).

Rickets
Rickets refers to osteomalacia caused by failure of
osteoid calcification in a growing child and usually
Fig. 13. Dermatomyositis. Chest CT scan of 51-year-old female with
severe dermatomyositis shows diffuse atrophy of chest wall muscles occurs as a result of vitamin D deficiency. The classical
and extensive subcutaneous calcifications in upper arms (arrows). thoracic manifestation of the disease is the so-called

A B
Fig. 14. Hyperparathyroidism.
Frontal (A) and lateral (B) chest radiographs of 31-year-old male with secondary hyperparathyroidism due to chronic renal failure show
resorption of distal clavicles (arrowheads) and “rugger-jersey” spine.

8 https://doi.org/10.3348/kjr.2019.0181 kjronline.org
Thoracic wall Abnormalities

“rachitic rosary,” referring to nodular enlargement of the Sickle Cell Anemia


costochondral junctions that can be apparent both clinically Sickle cell anemia is an autosomal recessive genetic
and radiologically (Fig. 15). Other radiological features of disorder characterized by abnormally shaped (sickled)
rickets include widening of the growth plates, metaphyseal red blood cells (RBCs). The underlying abnormality is
flaring, cupping and fraying (Fig. 15), generalized the presence of abnormal hemoglobin, which, when
osteopenia, bone deformities, especially bowing of legs, deoxygenated, becomes relatively insoluble and forms long
and insufficiency fractures (13). aggregates, which distort the RBC. The sickled RBCs cause
vascular occlusion, which leads to tissue ischemia and
infarction (14). Chest imaging usually reveals cardiomegaly
and cephalization of pulmonary vessels due to a chronic
high cardiac output state. H-shaped vertebrae and
occasional avascular necrosis of humeral heads are almost
pathognomonic for this condition (Fig. 16).

Thalassemia
RBCs are normally produced in the bone marrow. In cases
of chronic anemia, where RBC production by bone marrow
is not sufficient, hematopoiesis may extend beyond the
confinement of the cortical bone that commonly occurs in
the chest (15). Most cases of extramedullary hematopoiesis
(EMH) are observed in patients with thalassemia. EMH
typically appears as a posterior mediastinal abnormality,
Fig. 15. Rickets. Frontal chest radiograph of 3-month-old premature- in the form of well-defined smooth or lobulated bilateral
born female with rickets demonstrates healing fractures of left oblong paravertebral masses (Fig. 17). CT may also reveal
posterior 6th and 7th ribs (arrows), nodular widening of bilateral
costochondral junctions–rachitic rosary (arrowheads) and fraying of osseous changes of chronic anemia in the form of bone
proximal humeral metaphyses (dashed outlines). expansion and coarsened trabeculations (Fig. 17). MRI may

A B
Fig. 16. Sickle cell anemia.
Frontal (A) and lateral (B) chest radiographs of 39-year-old female with sickle cell anemia demonstrate H-shaped vertebrae (arrowheads) and
left humeral head subchondral sclerosis (arrow), consistent with avascular necrosis.

kjronline.org https://doi.org/10.3348/kjr.2019.0181 9
Semionov et al.

show low signal intensity of the bone marrow, denoting the distal clavicle, the manubrium, or both, seen as bony
active red marrow and iron deposition (Fig. 18). erosions or sclerosis on CT (Fig. 20), and bone marrow
edema on MRI, may be present in up to 55% of cases. Chest
Tuberculosis wall abscess or phlegmon (Fig. 20) and mediastinitis are
Tuberculosis (TB) results from infection by mycobacterium common.
tuberculosis and several other mycobacterial species. MSK
involvement with TB is relatively uncommon and is reported Elastofibroma Dorsi
in 1–3% of cases (16). The thoracic and lumbar spines are Elastofibroma dorsi is fibroelastic pseudotumor thought to
most frequently involved, referred to as the so-called Pott’s result from repeated mechanical friction between the chest
disease. Infection usually begins in the anterior part of the wall and the tip of the scapula (18). Patients often have an
vertebral body adjacent to the end plate and then spreads occupational history of manual labor, such as farming. Most
to the intervertebral disk, with subsequent dissemination patients are older adults. Most elastofibromas are clinically
into additional spinal segments and paraspinal tissues, occult. The most common symptom is stiffness, which is
resulting in the formation of a paravertebral abscess (Fig. observed in approximately one-fourth of the patients (18).
19). The untreated infection may eventually result in The location between the chest wall and inferior scapular
vertebral collapse and anterior wedging, leading to kyphosis tip is most characteristic of elastofibroma. Bilateral lesions
and gibbus formation. TB spondylitis is characteristically are seen in up to 66% of cases. On CT, elastofibroma
associated with little or no reactive sclerosis or local presents as a poorly defined, inhomogeneous soft-tissue
periosteal reaction, a feature that helps distinguish it from density with attenuation similar to that of skeletal muscle
pyogenic spondylitis (16).

Septic Arthritis of the Sternoclavicular Joint


Staphylococcus aureus is responsible for almost half of the
cases of septic arthritis of sternoclavicular joint. Common
risk factors include intravenous drug use, distant site of
infection, diabetes mellitus, trauma, and infected central
venous line (17). Plain radiography may show sclerosis of
the medial clavicle and manubrium. The earliest findings
seen on CT or MRI are joint effusion, widening of the
joint space, or mild cortical irregularity. Osteomyelitis of

Fig. 18. Beta thalassemia. Sagittal T1-weighted MRI of spine in


9-year-old male with beta thalassemia major shows diffuse abnormally
Fig. 17. Beta thalassemia. Chest CT scan of 44-year-old male with low signal intensity of bone marrow related to active red marrow and
beta thalassemia intermedia shows diffuse expansion of osseous iron deposition from multiple blood transfusions. Note that bone
medullary spaces, coarsened trabeculations of ribs and vertebrae, and marrow (asterisk) is of significantly lower intensity than intervertebral
paravertebral extramedullary hematopoiesis (arrows). disks (arrowhead).

10 https://doi.org/10.3348/kjr.2019.0181 kjronline.org
Thoracic wall Abnormalities

A B
Fig. 19. Tuberculosis.
Chest CT in bone (A) and mediastinal (B) windows of 23-year-old male with active post-primary pulmonary tuberculosis and spondylitis
demonstrates irregular lytic lesion involving superior endplate of dorsal vertebra (arrow) and associated perivertebral abscess (arrowhead).

A B
Fig. 20. Septic arthritis of sternoclavicular joint.
Chest CT in bone (A) and mediastinal (B) windows of 41-year-old male, who developed septic arthritis of right sternoclavicular joint several
weeks following penetrating injury to chest, demonstrates erosive lesions in right clavicular head and manubrium (arrowheads) consistent with
osteomyelitis, and adjacent phlegmonous collection (arrows).

and containing linear low-density streaks (Fig. 21). On both


T1- and T2-weighted images, the lesion appears as a well-
defined lenticular mass with intermediate signal intensity
approximately equal to that of skeletal muscle, with
interlaced areas of high signal intensity similar to that of
fat.

Sternal Dehiscence
Sternal dehiscence (SD) refers to disruption of the
sternotomy fixation. When SD occurs in the first post-
operative weeks, it is usually secondary to an off-center
Fig. 21. Elastofibroma dorsi. Chest CT scan of 81-year-old female
sternotomy or faulty sternal wires (19). SD two weeks after shows bilateral soft-tissue masses between scapulae and rib cage
surgery is typically associated with sternal osteomyelitis (arrows), consistent with bilateral elastofibroma dorsi.

kjronline.org https://doi.org/10.3348/kjr.2019.0181 11
Semionov et al.

A B
Fig. 22. Sternal dehiscence. 72-year-old male with chronic sternal dehiscence following remote median sternotomy for aorto-coronary bypass
surgery.
A. Chest radiograph shows fractures and misalignment of many sternal wires (arrows). B. Chest CT with coronal reformation shows separation of
sternotomy edges (arrowheads).

and mediastinitis. Unrecognized SD may lead to osseous REFERENCES


nonunion, which can cause severe pain. Progressive post-
operative displacement, rotation, or fracture of the sternal 1. van Hoorn JH, Moonen RM, Huysentruyt CJ, van Heurn LW,
wires on serial chest radiographs are all reliable signs of Offermans JP, Mulder AL. Pentalogy of Cantrell: two patients
and a review to determine prognostic factors for optimal
SD (Fig. 22). A more specific radiographic sign of SD is the
approach. Eur J Pediatr 2008;167:29-35
presence of a lucent strip of more than 3 mm in width along
2. Samartzis D, Herman J, Lubicky JP, Shen FH. Sprengel’s
the sternotomy. CT may show signs of underlying sternal deformity in Klippel-Feil syndrome. Spine (Phila Pa 1976)
osteomyelitis and/or mediastinitis. 2007;32:E512-E516
3. Paul SA, Simon SS, Karthik AK, Chacko RK, Savitha S. A review
SUMMARY of clinical and radiological features of cleidocranial dysplasia
with a report of two cases and a dental treatment protocol. J
Pharm Bioallied Sci 2015;7(Suppl 2):S428-S432
This pictorial essay illustrates the importance of
4. Urschel HC Jr. Poland syndrome. Semin Thorac Cardiovasc Surg
recognition of certain chest wall abnormalities on imaging, 2009;21:89-94
as it can facilitate accurate diagnosis of several associated 5. Stark Z, Savarirayan R. Osteopetrosis. Orphanet J Rare Dis
syndromes and other pathological conditions. 2009;4:5
6. Fortman BJ, Kuszyk BS, Urban BA, Fishman EK.
Neurofibromatosis type 1: a diagnostic mimicker at CT.
Conflicts of Interest
Radiographics 2001;21:601-612
The authors have no potential conflicts of interest to
7. Ha HI, Seo JB, Lee SH, Kang JW, Goo HW, Lim TH, et al.
disclose. Imaging of Marfan syndrome: multisystemic manifestations.
Radiographics 2007;27:989-1004
ORCID iDs 8. Newman CA, Reuther WL 3rd, Wakabayashi MN, Payette
Alexandre Semionov MM, Plavsic BM. Gastrointestinal case of the day. Gardner
syndrome. Radiographics 1999;19:546-548
https://orcid.org/0000-0002-8255-3324
9. Capobianco J, Grimberg A, Thompson BM, Antunes VB,
John Kosiuk
Jasinowodolinski D, Meirelles GS. Thoracic manifestations of
https://orcid.org/0000-0003-2643-8484 collagen vascular diseases. Radiographics 2012;32:33-50
Amr Ajlan 10. Johnson TH, Mital N, Rodnan GP, Wilson RJ. Relapsing
https://orcid.org/0000-0001-8199-1915 polychondritis. Radiology 1973;106:313-315
Federico Discepola 11. Wang YF, Teng MM, Chang CY, Wu HT, Wang ST. Imaging
manifestations of spinal fractures in ankylosing spondylitis.
https://orcid.org/0000-0002-8556-9153
AJNR Am J Neuroradiol 2005;26:2067-2076

12 https://doi.org/10.3348/kjr.2019.0181 kjronline.org
Thoracic wall Abnormalities

12. Duncan JG. Radiological manifestations of Tuberculosis: a radiologic review. Radiographics 2007;27:1255-
hyperparathyroidism. Proc R Soc Med 1956;49:283-286 1273
13. Calder AD. Radiology of osteogenesis imperfecta, rickets and 17. Ross JJ, Shamsuddin H. Sternoclavicular septic arthritis:
other bony fragility states. Endocr Dev 2015;28:56-71 review of 180 cases. Medicine (Baltimore) 2004;83:139-148
14. Lonergan GJ, Cline DB, Abbondanzo SL. Sickle cell anemia. 18. Kransdorf MJ, Meis JM, Montgomery E. Elastofibroma: MR and
Radiographics 2001;21:971-994 CT appearance with radiologic-pathologic correlation. AJR Am
15. Haidar R, Mhaidli H, Taher AT. Paraspinal extramedullary J Roentgenol 1992;159:575-579
hematopoiesis in patients with thalassemia intermedia. Eur 19. Restrepo CS, Martinez S, Lemos DF, Washington L, McAdams
Spine J 2010;19:871-878 HP, Vargas D, et al. Imaging appearances of the sternum and
16. Burrill J, Williams CJ, Bain G, Conder G, Hine AL, Misra RR. sternoclavicular joints. Radiographics 2009;29:839-859

kjronline.org https://doi.org/10.3348/kjr.2019.0181 13

You might also like

pFad - Phonifier reborn

Pfad - The Proxy pFad of © 2024 Garber Painting. All rights reserved.

Note: This service is not intended for secure transactions such as banking, social media, email, or purchasing. Use at your own risk. We assume no liability whatsoever for broken pages.


Alternative Proxies:

Alternative Proxy

pFad Proxy

pFad v3 Proxy

pFad v4 Proxy