Prune belly syndrome associated with Hirschsprung's disease.

Abstract

Prune belly syndrome is a clinical triad of hypoplasia of the abdominal muscles, urinary tract abnormalities, and cryptorchidism. The first feature gives the abdomens of affected infants a characteristic wrinkled appearance, hence the name of the syndrome. Urinary tract findings include hypoplastic or dysplastic kidneys, hydronephrosis, hydroureter, megacystis, and urethral obstruction. Many other abnormalities have been described, including malrotation of the gut, small bowel atresia, imperforate anus, cardiovascular malformations, pulmonary hypoplasia, and various skeletal anomalies. 1 To our knowledge, the patient described herein is the first reported case of prune belly syndrome associated with Hirschsprung's disease. Report of a Case .—The patient was a term infant delivered to a 16-year-old prima gravida mother via cesarean section because of cephalopelvic disproportion. The pregnancy and delivery were free of any other complications. At birth, the infant was noted to have a prominent forehead, large ears, hypoplastic abdominal muscles, a small penis, an