Chest Wall: The Ribs


Chest Wall: The Ribs

Short ribs do not extend as far anteriorly as the sternum; they lead to a decreased chest volume that may cause respiratory insufficiency. The more widely know dysplasias and syndromes with short ribs as a feature are discussed in Tables 5.3 through 5.10 .

Table 5.3 Ribs: short ribs





Short and wide ribs with concavity at the rib end.

Narrowing of the intrapedicular distance (as apposed to normal widening) from L1 to L5 on AP projection. Rhizomelic shortening of the humeri, macrocephaly, depression of the nasion, scalloping of the posterior aspects of the vertebral bodies, square iliac bones, and a champagne glass–shaped pelvic inlet. DD: hypochondroplasia.

Thanatophoric dysplasia

Very short ribs that do not extend beyond the anterior axillary line.

Most common form of skeletal dysplasia that is lethal in the neonatal period. “H-” or “U-”shaped vertebral bodies, short and curved humeri, cloverleaf skull deformity, polydactyly, and hypoplastic iliac bones. DD: thanatophoric variants, asphyxiating thoracic dysplasia, homozygous achondroplasia, and achondrogenesis.

Asphyxiating thoracic dysplasia (Jeune)

Fig. 5.2

Short ribs with a horizontal course. The chest diameter is smaller in comparison to the abdomen.

Long bones are shortened, the iliac wings are small, and the acetabula may have spurs. The proximal humeral and femoral epiphyses may be ossified at birth. ± polydactyly. Adults have a high prevalence of medullary cystic renal disease. DD: Ellis-van Creveld dysplasia.

Chondroectodermal dysplasia (Ellis-van Creveld syndrome)

Fig. 5.3

Small chest accentuates the size of the cardiomegaly.

Congenital heart disease (atrial septal defects) with cardiomegaly. Severe respiratory insufficiency results in high neonatal mortality (~50%). High prevalence among the Amish. Appearance of pelvis is similar to Jeune dysplasia. DD: Jeune dysplasia and short rib–polydactyly syndromes.

Other dysplasias and syndromes

For example, atelosteogenesis, Barnes syndrome, campomelic dysplasia, metatropic dysplasia, otopalatodigital syndrome, Schneckenbecken dysplasia.

Fig. 5.2 Asphyxiating thoracic dysplasia. Jeune syndrome. Short ribs and scoliosis.
Fig. 5.3 Chondroectodermal dysplasia. Ellis-van Creveld syndrome with a narrow chest, small iliac bones, and iliac spurs.

Table 5.4 Ribs: slender ribs




Trisomies 8, 13, 18, and 21

Hypoplastic and thin ribs. 11 rib pairs.

In trisomy 18, a short sternum produces a shield deformity of the chest. Acute iliac angle is diagnostic. Ulnar deviation and flexion deformity of the fingers with curled thumb grasped by the fingers.


Slender, twisted, and/or deformed ribs that may be separated by neurofibromas.

Chondrodysplasia punctata

(see Table 5.35 )

Other dysplasias and syndromes

For example, achondrogenesis, campomelic dysplasia, progeria, and Cockayne, Gorlin, Larsen, and Turner syndromes.

Table 5.5 Ribs: wide ribs




Thalassemia major

Undertubulated and broad ribs with heterogeneous ossification.

The rib findings as well as widened dipole, hypoplasia of paranasal sinuses, paraspinal extramedullary foci, and hepatospleno megaly are rarely seen with aggressive marrow replacement and chelation therapy. DD: other chronic anemias, storage disease, and fibrous dysplasia.

Fibrous dysplasia

Affected ribs are widened.

Sickle cell anemia and thalassemia


Ribs are thin proximally near the costovertebral junction and then wider distally.

External flaring of the iliac bones, vertebral body flattening and beaking, atlantoaxial instability, and brachydactyly. DD: Gaucher and Niemann-Pick diseases.

Gorlin (basal cell nevus) syndrome

Bifid, fused, or markedly splayed ribs.

Sprengel and pectus deformities, syndactyly, calcified falx cerebri, flame-shaped lucencies of the hands and feet. Radiologic findings contribute to the list of clinical criteria used to diagnose the syndrome.

Other dysplasias and syndromes

For example, achondroplasia, Erdheim-Chester disease, Niemann-Pick disease, osteopetrosis, pacman dysplasia, tuberous sclerosis.

Table 5.6 Ribs: miscellaneous rib anomalies




Cervical ribs

Unilateral or bilateral, arise from the seventh cervical vertebra, and resemble hypoplastic first thoracic ribs.

Incidental finding (0.2% to 8% prevalence) or are associated with Klippel-Feil anomaly. Can compress adjacent nerves supplying the brachial plexus and vessels (thoracic outlet syndrome or aneurysm formation).

Eleven paired ribs

Occurs in 5%–8% of normal individuals and in one-third of patients with trisomy 21. Associated with several skeletal dysplasias including cleidocranial and campomelic dysplasia.

Pectus excavatum

Posterior ribs are horizontal, whereas the anterior ribs have a more vertical course.

(see Table 5.1 )

Bifid rib

A portion of the rib splits into two separate ribs.

May be incidental and discovered as a palpable mass. DD: Gorlin (or basal cell nevus) syndrome (fourth rib most commonly bifid).

Supernumerary ribs

Trisomy 21 syndrome (although 11 paired ribs is more common) and with VATER (vertebral defects, anal atresia, tracheoesophageal fistula with esophageal atresia, radial or renal dysplasia, and limb anomalies) association. Rarely seen as a variation of normal.


Fig. 5.4a, b, p. 496

Cupping and enlargement at the rib ends of the costochondral junctions (rachitic rosary).

Rib notching

Fig. 5.5, p. 496

Concave notches found on the inferior rib surface.

Most commonly found as a variation of normal anatomy. Pressure erosion (saucerization) from enlarged vascular or neural structures. Notching associated with aortic coarctation usually affects ribs 4–8 (costal arteries are enlarged above the aortic stenosis) and is rare before the age of 8 y. DD: coarctation of the aorta, neurofibromatosis, thalassemia, and postoperative Blalock-Taussig shunt (right-sided notching).

Cerebrocostomandibular syndrome

Ribs have abnormal costovertebral articulations and posterior ossification gaps that resemble fractures.

Gaps will eventually ossify. Usually 11 pairs of ribs. Microcephaly, micrognathia, and congenital heart disease. DD: multiple rib fractures.

Fig. 5.4a, b Rickets with cupping at the rib ends (arrows in a) and metaphyses about the knee (b).
Fig. 5.5 Rib notching. Coarctation of the aorta. Rib notching (arrowheads) and the figure-three sign (arrow).

Table 5.7 Ribs: expansile rib deformity




Fibrous dysplasia

Expansile mixed lucent and sclerotic non-aggressive lesion with a ground-glass matrix, cortical thinning, and modeling deformity.

Most common cause of a benign expansile rib lesion. DD: simple bone cyst, enchondroma, and brown tumor of hyperparathyroidism.

Brown tumor of hyperparathyroidism

Lucent lesion.

(see Table 5.75 )

Other benign tumors

DD: enchondromatosis, osteochondroma, xanthogranuloma.

Malignant tumors

Fig. 5.6

Fig. 5.7

Bone destruction and attempt at remodeling usually with associated soft-tissue mass (see Table 5.74 ).

DD: Ewing sarcoma, primitive neuroectodermal tumor.

Langerhans cell histiocytosis

Fig. 5.8

Expansile and well-defined lucent lesion.

Skeletal survey may be useful to detect multiple lesions.

Lymphangiomatosis (Gorham disease)

Well-defined areas of lucency that may coalesce and replace large portions of bone. Osteolytic with a thin sclerotic rim. Osteolysis may be massive.

Thought to be due to malformation/proliferation of lymphatic vessels in bone.

Fig. 5.6 Malignant tumors. Rib expansion and destruction from metastatic rhabdomyosarcoma (arrow).
Fig. 5.7 Malignant tumors. Chest wall mass with rib destruction from Ewing sarcoma.
Fig. 5.8 Langerhans cell histiocytosis. Lucent and expansile rib lesion produced by Langerhans cell histiocytosis (arrows).

Table 5.8 Ribs: rib absence




Eleven paired ribs

(see Table 5.6 )

Unilateral absent rib

Postsurgical resection, tumor, osteomyelitis.

Table 5.9 Ribs: supernumerary ribs




Turner syndrome

Cervical ribs.

(see Table 5.30 )

Trisomy 8

Eleven or thirteen ribs. Rib abnormalities (wide, sloping, or rib gap).

Vertebral segmentation anomalies. Large and small joint contractures. Congenital heart disease and corneal opacities.

Cornelia de Lange syndrome

Thirteen ribs

(see Table 5.50 )

Table 5.10 Ribs: increased sclerosis




Tuberous sclerosis

Patchy regions of bone expansion and sclerosis.

May be confined to a single rib.

Osteopetrosis (Albers-Schönberg disease)

Generalized increase in bone density.


Fig. 5.9

Intramedullary sclerosis with the appearance of dripping candle wax.

Follows dermatomes.

See Table 5.91

Fig. 5.9 Melorheostosis.

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Jul 12, 2020 | Posted by in PEDIATRIC IMAGING | Comments Off on Chest Wall: The Ribs

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