Repeat pull-through surgery for complicated Hirschsprung's disease: indications, techniques, and results.

BACKGROUND: Most children with Hirschsprung's disease (HD) do well after a pull-through procedure. In the occasional child in which the operation fails, a repeat procedure may be necessary.

METHODS: Nine children with HD aged 20 months to 9 years underwent repeat pull-through over a 4-year period. Original pull-throughs (six Soave, two Swenson, one Duhamel) were performed elsewhere 12 to 95 months earlier (median, 36 months). Indications for revision were stricture unresponsive to dilatation (n = 3) and acquired aganglionosis (n = 6). One of the latter had associated segmental intestinal neuronal dysplasia. One child with a stricture after a Swenson procedure underwent a repeat Swenson. The other eight underwent reconstruction using a Duhamel technique. Five had a defunctioning stoma before or at the time of repeat surgery.

RESULTS: Median follow-up was 15 months (range, 4 to 40 months). Complications included wound infection (n = 2), anastomotic bleeding (n = 2), stoma leak (n = 1) or stenosis (n = 1), "kinking" at the top of the Duhamel (n = 1), and persistent septum (n = 1). Three patients have had a good outcome with normal stool patterns. One has intermittent soiling, and one has what is believed to be stool-holding behavior. Four have persistent obstructive symptoms caused by sphincter hypertonicity, which are being successfully managed nonoperatively.

CONCLUSIONS: Repeat pull-through surgery can be performed safely in appropriately selected patients. Duhamel reconstruction usually is preferred for technical reasons, and a stoma is not always necessary. Outcome is generally favorable, but anal sphincter hypertonicity may cause persistent symptoms in some patients.