Outcomes of surgical management of total colonic aganglionosis.

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.