Sternal defects

Sternal defects comprise a broad spectrum of deformities of the sternum, heart, and upper abdominal wall. We have reviewed our experience with sternal defects at the Children's Hospital of Boston and reviewed the world literature to present a current classification of these deformities and summarize frequently associated lesions and their therapeutic implications. Sixteen patients with sternal defects were identified in our series, 5 with thoracic ectopia cordis with a completely naked heart, 8 with thoracoabdominal ectopia cordis with a covered heart, and 3 with a cleft sternum. Thoracic ectopia cordis was uniformly fatal in our series, as it has been in all except 3 cases in the world literature, all with normal hearts. Thoracoabdominal ectopia cordis was fatal in 5 of 8 cases in our series related primarily to associated pulmonary hypoplasia or intrinsic cardiac anomalies. Bifid sternum was successfully repaired in all 3 cases in infancy.