Long-Term Outcome of Laparoscopic Duhamel Procedure for Extended Hirschsprung's Disease

The aim of our study was to report our experience in extended Hirschsprung's disease (HD) in children operated on by laparoscopy.

Retrospective data collection from a single center from 1991 to 2013 concerned extended forms of HD operated on by laparoscopic Duhamel procedure and included extension of aganglionosis, comorbidities, short and late postoperative outcome, and results of endoscopy when performed.

Thirty patients presented an extended form of aganglionosis: 5 involving the transverse colon, 10 the right colon, and 15 the ileum (median length = 15 cm, range 1-60). Short-term outcome showed 13% postoperative complications requiring redo-surgery: occlusion (n = 2), wall abscess (n = 1), and anastomotic leak (n = 1). Median follow-up was of 5 years (range: 1-19 years). Satisfying bowel control was reached in 53%, and 46% had a weight-for-age reference curve up to -1 SD. They had four stools per day on average, 13% of soiling, 6% of constipation, and 26% of recurrent abdominal distention. Late enterocolitis occurred in 22% (n = 7): 6 with ileum involvement and 1 extended to the transverse colon. Endoscopy showed Crohn-like ulcerations in 100% of these cases.

Laparoscopic Duhamel procedure is a safe and effective surgical technique in the management of extended forms of HD, with a low postoperative morbidity, but the frequent occurrence of late enterocolitis associated with Crohn-like ulcerations impairs the late outcome. Link between HD and Crohn disease still requires to be investigated.