HIRSCHSPRUNG'S DISEASE -CLINICAL FINDINGS AND STUDIES IN SURGICAL TREATMENT-

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  • Hirschsprung病の臨床像と治療法に関する検討

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Abstract

From April 1983 to December 1994, 82 infants and children with Hirschsprung's disease were treated at Saitama Children's Medical Center. Of 62 patients with short segment aganglionosis (rectum 33, sigmoid 29), 32 patiens (51.6%) were treated within the first month of life. On the other hand 20 patients with long segment aganglionosis were all neonates, of these five were entire colonic and four were more extensive type. Ten patients were associated with congenital anomalies. Four of them had Down's syndrome, five cardiac anomalies, two congenital deafness, two chromosome disorders other than 21 trisomy, and one imperfrate anus and ectodermal dysplasia, respectively. Three infants with extremely extensive aganglionosis and one with entire colonic aganglionosis associated with 22 q-trisomy were dead. We have been performing Duhamel-Ikeda procedure (Z-shaped anastomosis) using crushing clamps instead of GIA staples. Our new curved clamps, designed to be more effective and safe in crushing, are shown comparing with Ikeda's clamps. Also the type of skin incision in Z-shaped anastomosis are considered in its cosmetic aspects.

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