Megacystis–microcolon–intestinal hypoperistalsis syndrome: a rare cause of intestinal obstruction in the newborn

Berdon et al. (1976) first described a syndrome comprising intestinal obstruction and microcolon and a giant urinary bladder with megaureters. The authors coined the term “The megacystis microcolon intestinal hypoperistalsis syndrome”. We report a further case of this syndrome. K.A. was a full-term female infant born by vaginal delivery on February 8, 1978, following a pregnancy complicated by hydramnios. Her birth weight was 3.09 kg. This was the fourth pregnancy. Two siblings are alive and well. The first child, also female, was born alive but died at 4 days of intestinal obstruction. The patient was transferred to Alder Hey on the day of birth because of abdominal distension and deficient abdominal muscles. On examination her abdomen was midlly distended in its upper half, the abdominal wall was very lax but some muscle was present. The skin was redundant and had the appearance of a “prune belly”. No meconium had been passed. Nasogastric aspiration yielded 3.0 ml of dark green fluid. Apart from serum potassium (7.2 mmol/1) and urea (7.9 mmol/1) which were slightly raised, biochemical screening was normal. Supine X-ray showed a relatively airless abdomen with some gas in the stomach and duodenum (Fig. 1). A barium enema revealed a microcolon with complete malrotation of the bowel … the caecum lying in the left hypochondrium (Fig. 2). There was reflux into the small bowel which contained meconium and was also of micro size.