Megacystis-microcolon-intestinal hypoperistalsis syndrome: survival of a male infant.
The combination of megacystis with microcolon and severe intestinal hypoperistalsis constitutes a rarely encountered syndrome in neonates. Of 16 previously reported cases, 14 have occurred in females and all but one have been fatal. Various forms of intestinal diversions have been unsuccessful. Aganglionosis has been excluded in every case. A method of management involving prolonged total parenteral nutrition, followed by graduated jejunostomy and gastrostomy feedings, has resulted in the survival of a male infant with this syndrome. No previous male survivors have been reported.
D000015:Abnormalities, Multiple; D003106:Colon / Q000002:abnormalities*; D005260:Female; D005769:Gastrointestinal Motility; D005774:Gastrostomy; D006801:Humans; D007231:Infant, Newborn; D007415:Intestinal Obstruction / Q000209:etiology* / Q000601:surgery / Q000628:therapy; D007583:Jejunum / Q000601:surgery; D008297:Male; D010289:Parenteral Nutrition, Total; D010528:Peristalsis; D013577:Syndrome; D001743:Urinary Bladder / Q000002:abnormalities*