Re-operations for Hirschsprung's disease: long-term complications

Abstract

Introduction: In some patients with Hirschsprung's disease (HD), the initial surgical procedure fails, and the patients suffer from repeated or persistent symptoms. These patients complain of severe inflammation, intestinal obstruction, fecal or urinary incontinence, abdominal pain or dystrophy. However, little data has been published on the long-term follow-up results after re-operations for HD.

Materials and methods: We followed 8 cases between 2004 and 2006, of complicated HD requiring repeated surgery and recorded prior procedures, histological results, indications for re-operation, postoperative follow-up as well as long-term clinical outcomes including stool patterns, nutrition and micturition.

Results: The follow-up period ranged from 3.0 to 5.5 years (mean: 4.4 years). Indications for repeat procedures were as follows: blind rectal pouch after a Duhamel operation (n = 2), persistent aganglionosis (n = 4), long-segment stenosis (n = 1) after a Rehbein operation, and anal stenosis following TERPT (transanal endorectal pull-through) (n = 1). In one patient who had a Duhamel-Martin operation, extirpation of the rectum and a definitive terminal ileostomy was necessary. A Duhamel procedure was performed in five patients with a primary Rehbein and 1 patient with a primary Duhamel operation. Complete stool continence was achieved in 4 patients. Partial fecal incontinence persisted in one patient with associated trisomy 21. 1 patient with total colonic aganglionosis and 1 patient with a pelvic fistula and a previous subtotal colectomy reported soiling 1-2 times per week after a repeat operation. 4 patients in our series experienced postoperative complications following repeated surgery [perianal ulceration (n = 2), repeated botulinum toxin injection for sphincter achalasia (n = 1) and functionally impaired colonic transit without stenosis (n = 1)]. Micturition was normal in 7 patients, 1 patient with associated trisomy 21 was partially continent, and 1 patient reported infrequent urge incontinence.

Conclusions: All patients improved after further surgical intervention. However, resolution of their symptoms was delayed and partial stool incontinence or soiling persisted in 3 patients. Most complications leading to repeat procedures are preventable, especially residual aganglionosis. Therefore, great efforts should be made to minimize complications when planning and performing the primary surgery.