GASTROINTESTINAL FUNCTION AFTER SURGICAL-CORRECTION OF HIRSCHSPRUNGS-DISEASE - LONG-TERM FOLLOW-UP IN 135 PATIENTS

This study is a retrospective review of all children treated for Hirsc hsprung's disease over the past 22 years at a single pediatric institu tion. During this time 177 patients had definitive surgical reconstruc tion. Five children died of causes unrelated to Hirschsprung's disease , and five children died from enterocolitis after an uneventful postop erative course. Clinical follow up information was obtained from 135 ( 78%). Demographic data includes the following: sex ratio 74% male, 26% female; current mean age 9.9 years; mean length of follow-up 7.9 year s (range, 3 months to 21.5 years). Mean age at surgical reconstruction was 1.6 years. Definitive surgical procedures included endorectal pul l-through (Soave), 21%; modified Duhamel, 67%; extended side-to-side i leocolic anastomosis, 8%; rectal myomectomy, 4%. Transition zone was w ithin rectum or rectosigmoid region in 86%. Overall, 32% (43/135) repo rt difficulty with fecal soiling, and 12.6% (17/135) identify this as a severe problem. These numbers include patients with trisomy 21 and t otal colonic aganglionosis. Severe fecal soiling was reported in 7.1% (2/28) after an endorectal pull-through, and in 12.1% (11/91) after th e modified Duhamel. The difference in incidence of soiling after these two procedures is not statistically significant. However, 40% (4/10) of the patients after the long side-to-side anastomosis for total colo nic aganglionosis report severe problems with fecal soiling (P = .03). Surgical reconstruction for Hirschsprung's disease provides near-norm al gastrointestinal function for the majority of children, but long-te rm follow-up shows significant residual problems with soiling in 12.6% of the patients. This is consistent with reported experience worldwid e. Copyright (C) 1995 by W.B. Saunders Company