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

T L Marty1, T Seo, M E Matlak

  • 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.

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