All issues > Volume 34(7); 1991

Original Article

J Korean Pediatr Soc. 1991;34(7):1015-1021. Published online July 31, 1991.

A case of Prune Belly syndrome associated with holoprosencephaly.

Myung Hee Youn¹, Mi Sung Jeong¹, Hyung Ja Park¹, Shinna Kim¹, Keum Min Park², Song Ja Chin²

¹Department of Pediatrics, Dong Boo City Hospital, Seoul, Korea
²Department of Pathology, Dong Boo City Hospital, Seoul, Korea

Received: February 12, 1991;  Accepted: April 15, 1991.

Abstract

This is brief report of a case of prune belly syndrome associated with holoprosencephaly in one-day old neonate. At birth, physical examination revealed skull bone defect, anophthalmia, severe hypotelorism, arhinia with proboscis, micrognathia, webbed neck, severely dilated abdomen with thin wall, agenesis of external urogenital organs and anus, equinovalgus and genu valgus. Autopsy findings were alobar holoprosencephaly with hydrocephalus, hypoplasia of lung and ribs, markedly dilated cystis (14x11.5x6cm) with hypertrophied muscular layer of 1.2cm in thickness, mild hydroureter, polycystic kidney (Potter type II and IV), colonic atresia and agenesis of gallblad- der, pancreas and internal genital organs.

Keywords :Prune belly syndrome;Alobar holoprosencephaly