Semionov Imaging of Thoracic Wall
Semionov Imaging of Thoracic Wall
Semionov Imaging of Thoracic Wall
eISSN 2005-8330
https://doi.org/10.3348/kjr.2019.0181
Korean J Radiol 2019
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
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
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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
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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
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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).
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
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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
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).
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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,
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).
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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.
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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.
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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).
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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).
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.
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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).
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