Megacystis-microcolon-intestinal hypoperistalsis syndrome: a new cause of intestinal obstruction in the newborn. Report of radiologic findings in five newborn girls

Abstract

Five newborn girls presented with small intestinal obstruction and microcolon and a giant bladder (megacystis). Organic causes of obstruction were not found, and the gastrointestinal tract failed to function after appropriate diversion. Two died in the postoperative period, two lived several months on central venous hyperalimentation, and one died at 34 months of age following chronic though intermittent hyperalimentation. Pathologic studies showed an abundance of ganglion cells in both dilated and narrowed areas of intestine; the combined small bowel-colon length was one-third of normal in the absence of an evident obstructive or vascular insult. The five patients represent the most severe manifestation of defective intestinal peristalsis in a larger group of distended newborns in whom organic gastrointestinal obstruction is not found. Treatment with central venous hyperalimentation may sustain life, and some patients eventually recover gastrointestinal function. The hypoperistalsis is largely refractory to pharmacologic treatment; its cause is unknown.