[Cantrell's pentalogy: a case report]

Abstract

Cantrell's pentalogy is a congenital anomaly resulting from embryologic development defect. The anomalies observed in this disorder were: (1) a midline, supraumbilical abdominal wall defect, (2) a defect of the lower sternum, (3) a deficiency of the anterior diaphragm, (4) a defect in the diaphragmatic pericardium, (5) congenital intracardiac defects. Its occurrence in the newborn is quite uncommon, though it was first described by Cantrell and his colleagues in 1958. A one-day-old male fullterm newborn was presented. Omphalocele and partial ectopia cordis were noted immediately after birth. Echocardiogram revealed tetralogy of Fallot with atrium septal defect. Operative findings were sternal cleft, pericardial defect and ventral diaphragmatic defect. The literature on clinical manifestations, embryogenic defect and management of Cantrell's pentalogy is reviewed briefly.