Reoperation for Hirschsprung's disease.

BACKGROUND/PURPOSE: Reoperation for Hirschsprung's disease traditionally has 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 other complications and the 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 operation was newborn to 6 years (mean, 10 months). Eighty percent had aganglionosis limited to the rectosigmoid colon. Follow-up was by office visit or telephone (mean, 8.5 years).

RESULTS

Twenty-three of the 68 patients with Soave pull-through (34%) underwent 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 segment (three patients, three cured); one necrosis of pull-through converted to Duhamel and cured; and one rectal prolapse that was diverted. Fifteen of the 39 patients with Duhamel procedure (38%) underwent reoperation for severe constipation (seven patients, six cured, one diverted); persistent rectal septum (four patients, 4 cured); and intractable enterocolitis (four patients, three cured, one diverted). Overall, 21 of 23 patients (91%) with reoperation after Soave procedures were cured, whereas 13 of 15 patients (87%) who underwent reoperation after Duhamel procedure were cured, and four patients remain diverted.

CONCLUSIONS

These data show that aggressive reoperation can result in a high cure rate in Hirschsprung's disease. Although there is no significant difference 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 reoperation in patients with Hirschsprung's disease clearly is justified.