Megacystis-microcolon-intestinal hypoperistalsis syndrome: case report and review of the literature

We report a case of the megacystis-microcolon-intestinal hypoperistalsis syndrome and review the literature. Generally, this disease affects female neonates and is usually fatal within the first year of life. All patients have microcolon megacystis, intestinal hypoperistalsis, abdominal distention and dilated small bowel. Although the dilated bowel and urinary tract suggest an obstruction, such is not usually the case and surgical intervention is not always necessary. The abdominal distension results primarily from distension of the urinary tract.