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Critical analysis of the operative treatment of Hirschsprung's disease
R S Fortuna1, T R Weber, T F Tracy
1Department of Surgery, St. Louis University School of Medicine, Mo, USA.
Insights
Operative treatment for Hirschsprung's disease (HS) has a 60-67% uneventful recovery rate. While reoperation rates are similar for Soave and Duhamel procedures, Soave complications often require more extensive interventions.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Clinical Outcomes Research
Background:
- Hirschsprung's disease (HS) is a congenital disorder characterized by the absence of ganglion cells in the distal bowel.
- Surgical correction is the primary treatment for HS, with the Soave and Duhamel pull-through procedures being the most common.
- Long-term outcomes and complication profiles of these procedures require ongoing evaluation.
Purpose of the Study:
- To critically analyze the complications and long-term results of operative treatments for Hirschsprung's disease.
- To compare the outcomes of the Soave (endorectal) and Duhamel (retrorectal) pull-through operations.
Main Methods:
- Retrospective review of medical records for 82 patients treated for Hirschsprung's disease between 1975 and 1994.
- Standardized telephone questionnaires were used for follow-up assessment.
- Data collected included postoperative complications, reoperations, hospitalizations, and current bowel habits.
Main Results:
- Uneventful recovery rates were 67% for the Duhamel operation and 60% for the Soave operation.
- Complications included enterocolitis, rectal achalasia/stenosis, and fistulas, with Soave procedures often requiring more extensive reoperations.
- Long-term follow-up (mean 89.3 months) showed 100% continence by 15 years, though 21% required daily medication.
Conclusions:
- Both Soave and Duhamel pull-through operations for Hirschsprung's disease have significant complication rates.
- While reoperation rates are similar, Soave complications tend to be more complex, necessitating multiple procedures.
- Further refinement of surgical techniques and diligent patient follow-up are essential for optimizing outcomes in Hirschsprung's disease management.
Objective:
To critically analyze complications and long-term results of the operative treatment of Hirschsprung's disease.
Design:
Medical records of patients with Hirschsprung's disease were reviewed retrospectively. Follow-up was obtained using a standardized telephone questionnaire.
Setting:
Major pediatric referral center.
Patients:
Eighty-two infants and children (68 boys, 14 girls) were treated for Hirschsprung's disease during a 20-year period (1975 to 1994). The age at diagnosis was younger than 30 days in 47 neonates (57%), 30 days to 1 year in 22 infants (27%), and older than 1 year in 13 children (16%). Aganglionosis was limited to the rectosigmoid region in 66 patients (81%). Fifty-five Soave (endorectal) and 27 Duhamel (retrorectal) primary pull-through operations were performed.
Main Outcome Measures:
Postoperative complications, reoperations, hospitalization, and current bowel habits.
Results:
Eighteen children (67%) undergoing the Duhamel operation recovered uneventfully compared with 33 children (60%) undergoing the Soave operation. The complications following the Duhamel operation included enterocolitis in five cases (19%), rectal achalasia in four cases (15%), and persistent rectal septum in two cases (7%). Additional operations, which included myomectomy, rectal septum division, diverting enterostomy, and sphincterotomy, were required in seven patients (26%). Only one patient required more than one reoperation. In contrast, complications following the Soave operation included enterocolitis in 15 cases (27%), rectal stenosis in 12 (22%), anastomotic leak in four (7%), late perirectal fistula in three (5%), rectal prolapse in one (2%), and recurrent severe constipation in one (2%). Sixteen patients (29%) required additional operations, including diverting enterostomy, myomectomy, redo pull-through, sphincterotomy, fistulectomy, and revision of rectal prolapse. In this group nearly two reoperative procedures per patient were required. Telephone follow-up (mean, 89.3 months) after pull-through operations in 61 patients (74%) showed a mean of 2.8 stools per day, with 13 patients (21%) requiring daily medications.
Conclusions:
The most common operations (Soave and Duhamel) for Hirschsprung's disease result in an uneventful recovery in only 60% to 67% of patients. Although both Soave and Duhamel pull-through operations have nearly identical reoperation rates (26% vs 29%), complications after Soave pull-through operations often require multiple, more extensive procedures. Short-term total continence rates for both procedures are less than 50%, however, 100% became continent by 15 years after the pull-through procedure. Further refinement in operative technique and close follow-up are warranted.