Outcomes of surgical management of total colonic aganglionosis

Abstract

PURPOSE: The purpose of this study was to assess the long-term clinical outcomes and bowel function of patients with total colonic aganglionosis (TCA) after surgery. METHODS: The hospital records of 17 TCA patients treated surgically during 1985 to 2004 were reviewed. Long-term follow-up was done by telephone interviews with the parents. RESULTS: Primary enterostomy was performed in 13 (76%) patients. In three (17%) patients, TCA was not suspected initially. They underwent conservative surgery primarily, which required a second operation soon after. One had transverse colectomy with ileostomy. By pathologic review, nine (53%) patients had small bowel involvement of aganglionosis. Six (35%) patients died before corrective surgery. They all had extensive small bowel involvement. Among 11 patients who had a corrective operation, 10 were treated with Martin's procedure. Long-term (mean 74 months) follow-up was available in seven patients, and the mean weight-for-age percentiles was 27.1% (range 5-50%), the frequency of defecation was three to five times a day in four patients (57%), one or two times a day in two patients (28%), and more than five times a day in one patient (15%). CONCLUSIONS: TCA is difficult to diagnose; but once it is diagnosed correctly and treated by corrective surgery, outcomes seem promising. Martin's operation brought about a good outcome and enabled patients to have acceptable bowel habits. The prognosis is highly dependent on the extent of aganglionosis.