Surgical repair of anterior chest wall deformities: The past, the present, the future
Anatomical, Histologic, and Genetic Characteristics of Congenital Chest Wall Deformities

https://doi.org/10.1053/j.semtcvs.2009.03.001Get rights and content

There is a large and diverse group of congenital abnormalities of the thorax that manifest as deformities and/or defects of the anterior chest wall and, depending on the severity and concomitant anomalies, may have cardiopulmonary implications. Pectus excavatum, the most common anterior chest deformity, is characterized by sternal depression with corresponding leftward displacement and rotation of the heart. Pectus carinatum, the second most common, exhibits a variety of chest wall protrusions and very diverse clinical manifestations. The cause of these conditions is thought to be abnormal elongation of the costal cartilages. Collagen, as a major structural component of rib cartilage, is implicated by genetic and histologic analysis. Poland syndrome is a unique unilateral chest/hand deficiency that may include rib defects, pectoral muscle deficit, and syndactyly. Cleft sternum is a rare congenital defect resulting from nonfusion of the sternal halves, which leaves the heart unprotected and requires early surgical intervention.

Section snippets

Pectus Excavatum

The main characteristic of pectus excavatum (PE) is the inward displacement of the sternum and the adjacent costal cartilages (Figure 2, Figure 3). PE, or “funnel chest,” is the most common chest wall deformity and has an estimated occurrence of 1 in 400 to 1 in 1000 live births with males affected 3-5 times more often than females. PE comprises approximately 90% of all chest wall deformities and is usually sporadic, although a definitive group of familial cases has been well-documented.1, 2, 3

Pectus Carinatum

The second most common deformity of the anterior chest wall is pectus carinatum (PC), which is defined by the outward displacement of the sternum and/or abnormal protrusion of the ribs. PC is the most diverse group, which exhibits even more pronounced male predominance. The proportion of occurrence between PC and PE ranges from 1:13 to 1:4 and PC accounts for 5%-20% of all anterior chest wall deformities. Most cases of PC are sporadic; however, familial incidence has been reported in as many as

Pouter Pigeon Breast

Pouter pigeon breast (PPB) is a most intriguing and such a peculiar deformity that it could even be considered as a separate entity; however, it is most often described within the pectus carinatum group. This condition is characterized by a protrusion of the manubriosternal junction and the adjacent ribs with premature ossification of the sternum. PPB is sometimes referred to as combined (PE and PC) deformity of the chest. The distinctive z-shaped and abnormally ossified sternum is best

Poland Syndrome

Poland syndrome (PS) is a unilateral pectoral aplasia/dysdactylia syndrome characterized by unilateral absence of the costosternal portion of the pectoralis major muscle, absence of the pectoralis minor muscle, aplasia or deformity of the costal cartilages of the second through the fifth ribs, hypoplasia or absence of the breast and nipple, hypoplasia of the subcutaneous tissue of the region, alopecia of the axillary and mammary region, and brachysyndactyly, all on the same side (Fig. 14).40

Cleft Sternum

Cleft sternum (CS) is a rare abnormality in which failed fusion of the two sternal halves results in defects of different lengths in the breast bone. It is also known by the term “sternum bifidum.”50 A history of mechanical stress may play an important role in skeletal morphogenesis and in the pathogenesis of congenital abnormalities.51

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