Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS): a case report in Thailand.

[megacystis-microcolon-intestinal hypoperistalsis syndrome]

Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS ) is a rare congenital disease with high mortality rate . The authors report a case of a female term infant with massive abdominal distension at birth . Abdominal ultrasonography revealed a huge cystic mass resolved after urinary catheterization . Exploratory laparotomy was performed and intraoperative findings were an enlarged urinary bladder , microcolon , short bowel and malrotation of the small intestine . Ladd 's procedure , ileostomy and vesicostomy were performed . Pathological findings of rectal biopsy revealed normal ganglion cells . Although prokinetic drugs were given for promoting bowel motility , enteral feedings were not tolerated . She died from septicemia at the age of 50 days .