Definitive treatment of extended Hirschsprung's disease or total colonic form

Between December 1990 and March 1999, five laparoscopic Duhamel pull-through procedures for extended or total aganglionosis were performed in our department, one of which had a rectosigmoid form with a short bowel and a colonic resection due to a volvulus.

The aim of this study was to show that even when the extended form of Hirschprung's disease or anatomic difficulties such as a short bowel and anterior colonic resection are encountered, the laparoscopic approach is possible and total colectomy is feasible. The procedure has been described previously. We used one camera port and three working ports. The sigmoid, transverse, and right colon up to the last ileal cove were mobilized laparoscopically. A standard posterior ileo-anal anastomosis was performed, and an endo-GIA stapler was used for the anterior anastomosis.

A total of five patients underwent laparoscopic surgery for Hirschprung's disease. There were three total colonic forms, one rectosigmoid form with a short bowel and colon resection attributed to a volvulus, and one transverse variant that required a Deloyers' maneuver for the pull-through. Three of the infants required total parenteral nutrition (TPN) for an average of 49 days (range, 28-60) from diversion until the time of the definitive procedure. Only one patient did not receive TPN. Postoperatively, there were two complications-one wound infection and one hectic fever. The clinical results were good, with no soiling or stool incontinence and no constipation.

The laparoscopic procedure for total aganglionosis or the extended form of Hirschprung's disease is safe, feasible, and reproducible.