Reoperation for Hirschsprung's disease

Background/Purpose: Reoperation for Hirschsprung's disease traditionally ha s been used for patients with anastomotic leaks or stricture or with severe constipation from retained aganglionic segment or neuronal dysplasia, but there is little information regarding its use for ether complications and t he long-term outcome in these patients. Methods: In a 23-year period, 107 infants and children underwent Soave (68 patients) or Duhamel (39 patients) pull-through procedures. The age at oper ation was newborn to 6 years (mean, 10 months). Eighty percent had aganglio nosis limited to the rectosigmoid colon. Follow-up was by office visit or t elephone (mean, 8.5 years). Results: Twenty-three of the 68 patients with Soave pull-through (34%) unde rwent reoperation for intractable enterocolitis (10 patients, all 10 cured) ; anastomotic stenosis (four patients, three cured, one continued diversion ); anastomotic leak (four patients, four cured); retained aganglionic segme nt (three patients, three cured); one necrosis of pull-through converted to Duhamel and cured; and one rectal prolapse that was diverted. Fifteen of t he 39 patients with Duhamel procedure (38%) underwent reoperation for sever e constipation (seven patients, six cured, one diverted); persistent rectal septum (four patients, 4 cured); and intractable enterocolitis (four patie nts, three cured, one diverted). Overall, 21 of 23 patients (91%) with reop eration after Soave procedures were cured, whereas 13 of 15 patients (87%) who underwent reoperation after Duhamel procedure were cured, and four pati ents remain diverted. Conclusions: These data show that aggressive reoperation can result in a hi gh cure rate in Hirschsprung's disease. Although there is no significant di fference in the rate of reoperation after Duhamel and Soave procedures, the patients with Soave pull-through required more complex reoperations, with several requiring more than one procedure. An aggressive approach to reoper ation in patients with Hirschsprung's disease clearly is justified. J Pedia tr Surg 34:153-157. Copyright (C) 1999 by W. B. Saunders Company.