新生児ヒルシュスプルング病の診断と治療

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  • Management of Hirschsprung's Disease in the Neonate

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During the past 18 years surgical treatment for the neonate with Hirschsprung's disease has been performed to 112 cases at the National Children's Hospital, Tokyo. Intestinal perforation was complicated in 4 cases at the time admission, and all of these cases were admitted within 3 days of life. Seven cases were died from enterocolitis and associated anomalies. From our experiences points of diagnosis and treatment of the neonatal Hirschsprung's disease are as follows. 1) Most of cases could be diagnosed by Barium enema only. 2) Barium enema should be repeated when the initial study failed to demonstrate the typical findings of aganglionosis and the surgical intervention could be wait by conservative managements. 3) Anorectal manometry and histochemical study should be performed actively in the period of waiting for the surgical treatment. 4) Colostomy is best made on the most distal portion of ganglionic colon. There is no necessity for remaining colostomy after the definitive operation. 5) Early primary operation could be indicated for some selected cases. Indications for early primay operation are full termed, well nutrished patients without enterocolitis. 6) Soave-Denda's procedure is considered feasible as a definitive operation for young patients.

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