Congenital Defects Involving the Abdominal Wall, Sternum, Diaphragm and Pericardium

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Abstract
A rare case of congenital anomalies arising from the involvement of the somatic mesodermal layer of the embryo is described. The lesion consisted of congenital anomalies of the sternum, ventral diaphragm, pericardium and abdominal wall but without intracardiac anomalies. Previously reported cases emphasized intracardiac defects as being intimately associated with the syndrome. Embryologic involvement of both somatic and splanchnic layers of the primitive mesoderm prior to the 16th day of development probably accounts for inclusion of cardiac defects, as is most common. Later involvement of the somatic mesoderm spares the cardiac anomaly. Problems arising in the management of this unusual syndrome are discussed.