Chronic idiopathic intestinal pseudo-obstruction syndrome in childhood

Sim, Jae Geon; Seo, Jeong Kee; Park, Kui Won; Chi, Je Geun

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 diagnoses were congenital megacolons. But all of the patients showed the presence of ganglion cells 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 nutrition, and four patients improved clinically. Early diagnosis and proper management will reduce the mortality and morbidity.