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Gastrointestinal function after surgical correction of Hirschsprung's disease: long-term follow-up in 135 patients
1University of Utah, Primary Children's Medical Center, Salt Lake City 84113-1100, USA.
Insights
Surgical reconstruction for Hirschsprung
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Clinical Outcomes Research
Background:
- Hirschsprung's disease is a congenital condition affecting the large intestine.
- Surgical intervention is the primary treatment for Hirschsprung's disease.
- Long-term outcomes, particularly regarding fecal soiling, require further investigation.
Purpose of the Study:
- To evaluate the long-term functional outcomes of surgical reconstruction for Hirschsprung's disease in children.
- To identify the incidence and severity of fecal soiling after different surgical techniques.
- To analyze factors influencing residual functional problems.
Main Methods:
- Retrospective review of 177 pediatric patients undergoing definitive surgical reconstruction for Hirschsprung's disease over 22 years.
- Data collection included demographics, surgical procedures, and clinical follow-up information from 135 patients (78%).
- Analysis of functional outcomes, focusing on fecal soiling, stratified by surgical technique and patient characteristics.
Main Results:
- The modified Duhamel procedure was the most common (67%), followed by endorectal pull-through (21%).
- Overall, 12.6% of patients reported severe fecal soiling, with higher rates in those with total colonic aganglionosis undergoing side-to-side anastomosis (40%).
- No statistically significant difference in soiling was found between endorectal pull-through and modified Duhamel procedures.
Conclusions:
- Surgical reconstruction generally provides near-normal gastrointestinal function for most children with Hirschsprung's disease.
- Significant long-term issues with fecal soiling persist in a notable percentage of patients, particularly after specific procedures for extensive disease.
- Outcomes highlight the need for continued monitoring and potential refinement of surgical strategies for Hirschsprung's disease.
Abstract:
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.