An 18-year experience in total colonic aganglionosis: from staged operations to primary laparoscopic endorectal pull-through

Abstract

Background: Traditionally, total colonic aganglionosis (TCA) was managed with enterostomy +/- pull-through. Since 1998, primary laparoscopic endorectal pull-through (PLEP) was offered to patients with TCA in our unit. A retrospective study was conducted and early results reviewed.

Method: Patient data were collected retrospectively. Before 1998, enterostomy followed by open Duhamel pull-through was performed. From 1998, patients were assessed to receive staged operation or PLEP.

Results: From June 1990 to May 2007, 10 patients (6 males) were treated. All presented in the neonatal period. Seven patients, with transition zone within proximal 15 cm of terminal ileum, had pull-through performed. Three had staged pull-through, whereas 4 had PLEP. Two had extensive small bowel involvement with high output stoma pending reconstruction. One had total intestinal aganglionosis and died. The 3 patients with staged pull-through had normal bowel habit without soiling (mean follow-up, 13 years). For PLEP, all procedures were accomplished laparoscopically without intraoperative complication (mean operative time, 6 hours 50 minutes). The mean bowel motion is 5x per day, but most were too young to evaluate continence (mean follow-up, 3.5 years).

Conclusion: Primary laparoscopic endorectal pull-through is feasible in TCA with limited small bowel involvement and has the advantage of a single-stage operation, avoiding a stoma and its related complications.