Reoperation for Hirschsprung's disease

T R Weber1, R S Fortuna, M L Silen

  • 1Department of Surgery, St. Louis University School of Medicine and Cardinal Glennon Children's Hospital, MO 63104, USA.

Insights

Aggressive reoperation for Hirschsprung's disease complications leads to high cure rates. This study supports an assertive surgical approach for improved long-term outcomes in pediatric patients.

Area of Science:

  • Pediatric Surgery
  • Gastrointestinal Surgery
  • Congenital Disorders

Background:

  • Hirschsprung's disease (HSCR) is a congenital condition characterized by the absence of ganglion cells in the distal bowel.
  • Reoperation for HSCR is typically reserved for anastomotic leaks, strictures, or severe constipation due to residual aganglionic segments.
  • Limited data exists on reoperation for other HSCR complications and their long-term results.

Purpose of the Study:

  • To evaluate the efficacy and long-term outcomes of reoperations for various complications of Hirschsprung's disease.
  • To compare reoperation rates and outcomes between the Soave and Duhamel pull-through procedures.
  • To determine if an aggressive reoperative strategy is justified for HSCR.

Main Methods:

  • A retrospective review of 107 infants and children who underwent Soave (68) or Duhamel (39) pull-through procedures for HSCR over 23 years.
  • Patients' age ranged from newborn to 6 years (mean 10 months).
  • Follow-up averaged 8.5 years via office visits or telephone.

Main Results:

  • 34% (23/68) of Soave patients and 38% (15/39) of Duhamel patients required reoperation for complications like enterocolitis, stenosis, leaks, or retained aganglionic segments.
  • Reoperation success rates were high: 91% (21/23) after Soave and 87% (13/15) after Duhamel procedures.
  • Four patients in total remained diverted; Soave pull-throughs necessitated more complex reoperations, sometimes multiple procedures.

Conclusions:

  • Aggressive reoperation for Hirschsprung's disease complications yields high cure rates.
  • While reoperation rates are similar between Soave and Duhamel procedures, Soave cases may require more complex interventions.
  • An assertive approach to reoperation is strongly recommended for managing Hirschsprung's disease complications.
Abstract