PEDIATRICS Vol. 63 No. 5 May 1979, pp. 805-808
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Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome

Thomas E. Wiswell M.D.1, James S. Rawlings M.D.1, James L. Wilson M.D.1, and Gary Pettett M.D.1

1 Newborn Medicine Service, Department of Pediatrics, Tripler Army Medical Center Honolulu

The syndrome of megacystis-microcolon-intestinal hypoperistalsis has been reported in a total of seven female infants. Massive abdominal distention secondary to a distended urinary Fig 1. Plain film of markedly distended abdomen with no air beyond stomach. Stomach is displaced upward toward left. der was the major presenting characteristic. Iutestinal hypoperistalsis, apparent in the early neonatal period, persists without improvement. Exploratory laparotomy reveals malrotation and malfixation of a small microcolon. No anatomic cause of intestinal or bladder obstruction can be found. Intestinal and/or rectal biopsy specimens contain abundant ganglion cells and nerve fibers. The outcome has been uniformly fatal, with survival in the reported cases ranging from two days to 34 months.




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