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A Case of Megacystis Microcolon Intestinal Hypoperistalsis Syndrome

Journal of the Korean Pediatric Society 2000;43(2):278-282.
Published online February 15, 2000.
A Case of Megacystis Microcolon Intestinal Hypoperistalsis Syndrome
Jae Woo Lim1, Jong Su Shin1, Kyuchul Choeh1, Chang Su Na1, Tae Il Han2
1Departments of Pediatrics, Eulji Medical College, Taejon, Korea
2Departments of Radiology, Eulji Medical College, Taejon, Korea
Megacystis Microcolon Intestinal Hypoperistalsis Syndrome 1례
임재우1, 신종수1, 최규철1, 나창수1, 한태일2
1을지의과대학 소아과학교실
2을지의과대학 방사선과학교실
Abstract
Megacystis Microcolon Intestinal Hypoperistalsis(MMIH) Syndrome is a rare cause of functional neonatal bowel obstruction, characterized by hypoperistalsis, narrow distal ileum and colon, and bladder distension. We report a case of MMIH syndrome and review the literature. The patient was born after 34 weeks of gestation with marked abdominal distension. Her birth weight was 2,830g with 276ml of urine drained by catheter. Prenatal ultrasonic findings were bladder distension, hydronephrosis and possible intraabdominal mass. Supine view of abdomen on third day of life showed dilated loops of proximal small bowel and stomach without colonic gas shadow. Preoperative diagnosis was intestinal obstruction. When the abdomen was opened, the distal ileum was filled with meconium and postoperative diagnosis was meconium plug syndrome. Ganglion cells were present in the ileal biopsy. All postoperative attempts to feed her resulted in bilous vomiting. Voiding cystourethrography done on the 4th day after colon study showed markedly distended bladder, contrast enhanced microcolon and hypoperistalsis. She was dischaged against medical advice by her parents at the age of 23 days and died at home at the age of 33 days.
Key Words: Megacystis Microcolon Intestinal Hypoperistalsis Syndrome, MMIH, Functional intestinal obstruction


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