Critical analysis of the operative treatment of Hirschsprung's disease

R. S. Fortuna, T. R. Weber, T. F. Tracy Jr, M. L. Silen and T. V. Cradock
Department of Surgery, St. Louis University School of Medicine, Mo, USA.

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.