Visual Diagnosis in Pediatrics
1st Edition

28
Abnormal Chest Shape
Amy Renwick
Approach to the Problem
Several elements contribute to chest shape, including muscles, bones (ribs, sternum, clavicles, and spine), and underlying organs. Deficiency, hypertrophy, or malformation of any of these structures may produce abnormalities in the appearance of the chest wall. Abnormalities in lung form or function may cause changes in the chest shape; conversely, alterations in the size or shape of the thorax may significantly affect pulmonary function. Rarely chronic cardiac enlargement may produce a prominence in the precordial chest wall. Pectus excavatum is the most common pediatric chest wall deformity; most of the others are quite rare.
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Figure 28-1 Pectus excavatum. Anterior view of child with pectus excavatum. (Courtesy of George A. Datto, III, MD.)
Figure 28-2 Pectus excavatum. Lateral view of child with pectus excavatum. (Courtesy of George A. Datto, III, MD.)
Figure 28-3 Pectus excavatum. Severe pectus in an adolescent with significant exercise intolerance. (Courtesy of Christopher Derby, MD.)
Figure 28-4 Poland sequence. Absence of pectoralis major muscle. (Used with permission from
Staheli LT. Practice of pediatric orthopedics. Philadelphia: Lippincott Williams & Wilkins; 2001:191
)
Figure 28-5 Cleidocranial dysostosis. Narrow chest with drooping shoulders. (Used with permission from
Staheli LT. Practice of pediatric orthopedics. Philadelphia: Lippincott Williams & Wilkins; 2001:192
)
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DIAGNOSIS ICD-9 DISTINGUISHING CHARACERISTICS ASSOCIATED FINDINGS COMPLICATIONS PREDISPOSING FACTORS
Pectus Excavatum 754.81 Sternal depression
Sternum may rotate to right, making right-sided structures smaller
Deformity worsens during puberty
Increased incidence of scoliosis, congenital heart disease
May be associated with Marfan syndrome, homocystinuria, Noonan syndrome, and other genetic disorders
Rarely interferes with cardiac or respiratory function, though some children have exercise limitations
May be a significant cosmetic problem
Can give false appearance of cardiomegaly on chest x-ray.
Excessive growth of costal cartilage
Male:female(3:1)
Pectus Carinatum 754.82 Sternal protrusion
Generally noted later in childhood than pectus excavatum
As for pectus excavatum Rarely interferes with cardiac or respiratory function, though some children have exercise limitations
May be a significant cosmetic problem
Excessive growth of costal cartilage
More common in males
Barrel Chest 738.3 AP diameter of chest increased relative to transverse diameter
(Normal ratio is 1:1 in infancy, with transverse diameter increasing with age)
Digital clubbing According to underlying cause Severe asthma or other obstructive lung disease (e.g., Meconium aspiration syndrome)
Genetic disorders such as spondyloepiphyseal dysplasia, Smith-McCort dysplasia.
Costello syndrome
Shield Chest 754.89 Wide chest with broadly spaced nipples Turner syndrome
Noonan syndrome
According to underlying cause N/A
Poland Sequence 756.81 Unilateral deficiency of pectoralis muscles and breast structures
Usually right-sided
May also have rib defects
Ipsilateral syndactyly of hand
Respiratory problems when significant rib defects are present
Breast tissue also is typically absent on affected side
Male:female (3:1)
Abnormal blood flow through subclavian artery
Sternal Cleft 756.3 Complete or partial separation of sternum
May manifest as U-shaped or V-shaped depression, or with bulge from underlying structures
May change with respiration
Pulsations of heart may be visible. If there is overlying skin, it is often thin and hyperpigmented.
Usually an isolated anomaly
Ectopia cordis
Pentalogy of Cantrell
Facial hemangiomas
Cleft lip or palate
None (unless associated defects) None
Cleidocranial dysostosis 755.59 Absent or hypoplastic clavicles resulting in abnormal shoulder movement
Narrow thorax
Variety of other skeletal anomalies
Abnormal teeth
Delayed tooth eruption
Deafness
Respiratory problems in infancy Autosomal dominant inheritance
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Other Diagnoses to Consider
  • Vertebral deformities
  • Acute or healed injuries
  • Isolated rib anomalies
  • Isolated clavicle anomalies
  • Other genetic syndromes or congenital malformations
Suggested Readings
Grissom LE, Harcke HT.Thoracic deformities and the growing lung. Semin Roentgenol. 1998;33:199-208.
Jones K. Smith’s recognizable patterns of human malformation. 5th ed. Philadelphia:WB Saunders; 1997.
Myers NA. An approach to the management of chest wall deformities. Prog Pediatr Surg. 1991;27:170-190.
Ravitch M. Congenital deformities of the chest wall and their operative correction. Philadelphia: WB Saunders; 1977.
Staheli LT. Fundamentals of pediatric orthopedics. 3rd ed. Philadelphia; Lippincott Williams & Wilkins; 2003:112.
Welch KJ. Chest wall deformities. In: Holder TM.Ashcraft KW, eds. Pediatric surgery. Philadelphia:WB Saunders; 1980:162-182.