Successful Surgical Repair of Pentalogy of Cantrell at 14 Months of Age

Pentalogy of Cantrell with ectopia cordis is a rare congenital anomaly, first described in 1958 by Cantrell, has a reported incidence of around 5 - 10 cases per one million live births with wide variety of clinical presentations. We are reporting a child with ectopia cordis along with cleft lower sternum, upper abdominal wall defect, ectopic umbilicus and diaphragmatic defect. Echocardiography in first month of life revealed a restrictive perimembranous ventricular septal defect and a small patent Foramen Ovale, both closed spontaneously in infancy. CT angiography at 10 months of age revealed a defect in the thoracic and abdominal walls along with herniation of left ventricular apex into epigastrium. The two ventriculi formed a tail that looked like a crocodile. This patient underwent surgical correction at our institution at 14 months of age and recovered well with no residual issue.