Megacystis-microcolon-intestinal hypoperistalsis syndrome: A cause of intestinal obstruction in the newborn period

Abstract

A 2992 g full term girl was born with marked abdominal distension. Aspiration of thestomach yielded thick bilious fluid. She was transferred to The Children’s Mercy Hospital for further evaluation. The abdomen was distended and nontender with no discrete masses. The remainder of the examination was unremarkable except for slightly webbed neck folds. Serum electrolytes and blood urea nitrogen were normal on admission. Supine roentgenograms showed elevated diaphragms, prominent flanks, a ground glass appearance of the abdomen and a small amount of intestinal air. The findings suggested ascites, but there was no fluid wave and the intestine was displaced laterally rather than centrally. The stomach was displaced upward by a grossly distended urinary bladder (Fig. IA). At 9 hr of age the bladder was easily catheterized with recovery of 150 ml of c/ear dilute urine, under some pressure. Abdominal distension lessened. resulting in a “prune belly” appearance. The baby voided a small volume of urine once but thereafter required a catheter for bladder drainage. The following day intravenous pyelography showed severely hydronephrotic kidneys and dilated intestine in the midabdomen with no air in the rectum (Fig. IB). Cystogram outlined a large bladder with no vesicoureteral reflux. Because of bilious vomiting and failure to pass meconium, intestinal atresia was suspected. Barium enema demonstrated a microcolon with the entire colon appearing “unused” (Fig. IC). At operation (at 39 hr of age) cystoscopy showed a normal female urethra with no bladder outlet obstruction and no ureteroceles. At exploration the abdominal wall musculature was thin but present. There was no ascites. Both kidneys were markedly enlarged with virtually no parenchyma remaining. The ureters were tortuous, moderately dilated and emptied into an enlarged. thick walled bladder. The small intestine was strikingly foreshortened and suspended on a primitive dorsal mesentery. It contained meconium and dark, hemorrhagic fluid suggesting necrotizing enteritis. Intramural hematomas were noted in the ileum after minimal handling. The jejunum and proximal ileum were moderately dilated and the distal ileum was small. The colon was small throughout and nonrotated. There \cas no anatomic site of obstruction. It was felt that the baby’s renal parenchyma was inadequate for long term survival. The serum creatinine was found to be elevated to 1.32/mg/dl. The baby was septic terminally with a blood culture positive for E. coli and Pseudomonas. She died on the third postoperative day.