MEGACYSTIS-MICROCOLON-INTESTINAL HYPOPERISTALSIS SYNDROME - report of a very rare pathology in a neonate

We are reporting a baby who presented with features of neonatal intestinal obstruction, with an x-ray picture suggestive of hold up at the duodenal level. On laparotomy he had malrotation of the gut which was corrected. He also had a dilated urinary bladder, which later on was treated with vasicostomy. The baby did not move his bowel inspite of no mechanical obstruction and even bypass procedure (gastrojejunostomy). She had all the features of Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome (MMIHS). MMIHS (also called hollow viscus myopathy) is a neonatal condition causing a severe form of pseudo (functional) intestinal obstruction. This is an autosomal recessive disorder more common in females. The various components of the syndrome are dilated urinary bladder, decreased motility of the gut, microcolon, intestinal malrotation and lax abdominal musculature. All these components of the syndrome were found in our case.