Total colectomy and ileorectal anastomosis in the treatment of total colonic aganglionosis: a long-term follow-up study of six patients.

Six patients with total colonic aganglionosis (TCA) with ileal involvement were treated with a total colectomy and ileorectal anastomosis according to Rehbein. Follow-up lasted from 1 to 7 years and showed no mortality. Postoperative management consisted of total parenteral nutrition initially, followed by gradual introduction of oral feeding beginning with a low-osmolarity low-residue diet, the use of loperamide, an adequate oral intake of salt, and regular bowel deflation. Major postoperative complications disappeared and all patients demonstrated weight gain by the end of the first postoperative year, with normal feeding and two to three soft stools per day.