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Chronic Idiopathic Intestinal pseudo-obstrucion Syndrome in Childhood

Journal of the Korean Pediatric Society 1993;36(11):1583-1595.
Published online November 15, 1993.
Chronic Idiopathic Intestinal pseudo-obstrucion Syndrome in Childhood
Jae Geon Sim1, Jeong Kee Seo1, Kui Won Park2, Je Geun Chi3
1Department of Pediatrics, Seoul National University, College of Medicine, Seoul, Korea
2Department of Pediatrics Surgery, Seoul National University, College of Medicine, Seoul, Korea
3Department of Pathology, Seoul National University, College of Medicine, Seoul, Korea
소아 만성 가성 장 폐색 증후군
심재건1, 서정기1, 박귀원2, 지제근3
1서울대학교 의과대학 소아과학교실
2서울대학교 의과대학 소아외과
3서울대학교 의과대학 병리과
Abstract
Chronic idiopathic intestinal pseudo-obstruction syndrome is a clinical condition induced by an impaired function of intestinal motility. Although its clinical symptoms are those of intestinal obstruction, mechanical obstruction of the intestine cannot be found by vigorous studies, even by operative exploration. We have experienced nine cases of chronic idiopathic intestinal pseudo-obstruction syndrome. It will help in diagnosis and treatment of the pseudo-obstruction to present the patients with these syndromes. In all cases severely delayed intestinal transit times were noted in radiological gastrointestinal studies without evidence of mechanical obstruction. The most frequent tentative dianoses were congenital megacolons. But all of the patients showed the presence of ganglion colls in the intestinal wall. Histological abnormalities of the muscle layer were found in three patients. We could not resolve the symptoms by the surgical decompression of the intestine (such as colostomy, ileostomy etc.). But the symptoms were improved after total colectomy in a patient whose main affected site was confined to the colon. The megacystis was the most frequent combined anomaly. All patients had been improved with NPO and total parenteral nutrition therapies were inevitable. The drugs which facilitate the intestinal activities such as cisapride could not resolve the symptoms of obstruction. Three patients were died with the complications of total parenteral nurtition, and four patients improved clinically. Early diagnosis and proper management will reduce the mortality and morbidity.
Key Words: Intestinal pseudo-obstruction, Child


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