Gastrointestinal function after surgical correction of Hirschsprung's disease: long-term follow-up in 135 patients.

This study is a retrospective review of all children treated for Hirschsprung's disease over the past 22 years at a single pediatric institution. During this time 177 patients had definitive surgical reconstruction. Five children died of causes unrelated to Hirschsprung's disease, and five children died from enterocolitis after an uneventful postoperative 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 years (range, 3 months to 21.5 years). Mean age at surgical reconstruction was 1.6 years. Definitive surgical procedures included endorectal pull-through (Soave), 21%; modified Duhamel, 67%; extended side-to-side ileocolic anastomosis, 8%; rectal myomectomy, 4%. Transition zone was within rectum or rectosigmoid region in 86%. Overall, 32% (43/135) report difficulty with fecal soiling, and 12.6% (17/135) identify this as a severe problem. These numbers include patients with trisomy 21 and total colonic aganglionosis. Severe fecal soiling was reported in 7.1% (2/28) after an endorectal pull-through, and in 12.1% (11/91) after the 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 colonic aganglionosis report severe problems with fecal soiling (P = .03). Surgical reconstruction for Hirschsprung's disease provides near-normal gastrointestinal function for the majority of children, but long-term follow-up shows significant residual problems with soiling in 12.6% of the patients. This is consistent with reported experience worldwide.