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Repeat pull-through surgery for complicated Hirschsprung's disease: indications, techniques, and results
1Department of Surgery, Washington University School of Medicine, St Louis, MO, USA.
Insights
Repeat pull-through surgery for Hirschsprung's disease (HD) is safe and effective in selected patients. The Duhamel technique is often preferred, and while outcomes are generally good, anal sphincter hypertonicity can cause persistent issues.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Congenital Disorders
Background:
- Hirschsprung's disease (HD) often requires pull-through surgery.
- While most operations succeed, some children need repeat procedures due to failure.
Observation:
- Nine children with HD underwent repeat pull-through surgery over four years.
- Indications included refractory stricture (3) and acquired aganglionosis (6).
- The Duhamel technique was preferred for reconstruction in most cases.
Findings:
- Complications included wound infection, bleeding, and stoma issues.
- Three patients achieved normal stool patterns; one had intermittent soiling.
- Four patients experienced persistent obstructive symptoms due to sphincter hypertonicity.
Implications:
- Repeat pull-through surgery is a viable option for selected HD patients.
- Duhamel reconstruction is technically favored, and a stoma is not always required.
- Anal sphincter hypertonicity can be a long-term challenge requiring management.
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.