The Problematic Duhamel Pouch in Hirschsprung’s Disease: Manifestations and Treatment

Abstract

Purpose:

The Duhamel operation is commonly employed to treat Hirschsprung’s disease (HD). We have seen a number of patients referred to our center with problems following a Duhamel procedure performed elsewhere, and have analyzed our experience with these children.

Methods:

We reviewed 17 patients with Hirschsprung’s disease who underwent a Duhamel procedure elsewhere, in whom we performed a redo pull-through for persistent symptoms of constipation, impaction, and enterocolits.

Results:

All patients (n=17) had constipation/impaction or enterocolits, 9 of whom were soiling due to overflow incontinence. Biopsies of the pulled-through bowel found 6 patients with persistent aganglionic bowel and 2 patients with ganglion cells present but hypertrophic nerves, a finding we interpreted as “transition zone bowel”. The remaining 9 patients without a pathological indication for reoperation had a mega Duhamel pouch. All patients underwent a redo operation: 8 via a posterior sagittal approach (7 with a laparotomy, 1 without) and 9 by a transanal, Swenson-type resection with a laparotomy. The posterior sagittal approach was used in cases with severe pelvic fibrosis considered unsuitable for a trans­anal operation. 15 patients were followed up postoperatively for longer than 2 months, 13 of whom now have voluntary bowel movements, including 8 who need a small dose of laxatives. 2 patients are still diverted.

Conclusion:

Although perhaps successful for many patients around the world, the Duhamel pull-through can leave patients with significant symptoms, including impaction and overflow incontinence. It is unclear why some patients with a Duhamel pouch do not empty well. Clearly, those patients with a mega Duhamel pouch suffer from impaction. These patients need to be detected, because reoperation with resection of the Duhamel pouch can dramatically improve their quality of life.

Key words

Hirschsprung’s disease - Duhamel procedure - reoperation - constipation - megarectum

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