The Authors report their experience in the treatment of 86 patients, in pediatric age, with Hirschsprung's disease: 40 with congenital rectosigmoid or subtotal megacolon, 8 with total colic or ileocolic aganglionosis and 38 with ultrashort aganglionic segment. Out of the patients 29% had necrotizing enterocolitis with 40% mortality rate in rectosigmoid or subtotal forms and with 75% mortality rate in total colic or ileocolic forms. In the last group the mortality was higher related also to difficulties and complications of surgical treatment. Necrotizing enterocolitis is the major cause of death in infants with Hirschsprung's disease. Early diagnosis and treatment can reduce the mortality rate.

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