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Megacystis-microcolon-intestinal hypoperistalsis syndrome: additional clinical, radiologic, surgical, and
Insights
Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) affects newborns, presenting diverse clinical features. This study details four cases, expanding understanding of MMIHS spectrum and histopathology.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Medical Genetics
Background:
- Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a rare congenital disorder.
- MMIHS is characterized by a triad of bladder distension, a small colon, and intestinal hypoperistalsis.
Abstract:
Four newborn infants with megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) were identified at Children's Hospital of Pittsburgh. These cases provide additional insight into the syndrome and broaden its spectrum. This report includes MMIHS in an infant boy, one long-term survivor, an apparently related complication of neonatal obstructive volvulus, evidence of small intestinal hypoperistalsis, and histopathologic findings as follows: (1) apparently increased numbers of ganglion cells in early biopsies and normal or even decreased numbers of ganglion cells in later biopsies probably due to bowel dilatation; and (2) in two of three infants at autopsy, there were many nerve trunks (a neuromalike layer in one), and there was elastosis of the urinary bladder.