In 1974 total colectomy and ileoanal straight endorectal pull-through (ERPT) were first used at our institution for the definitive management of total colonic Hirschsprung's disease in infants and children. Early success with this operation encouraged us to use this procedure in children and adults with ulcerative colitis and familial polyposis in 1977. Since 1974 we have performed total colectomy and straight ileoanal ERPT on 100 consecutive patients with ulcerative colitis (79), familial polyposis (19), and total colonic Hirschsprung's disease (10). Patients who have undergone a colectomy and ERPT but have not had their temporary ileostomy closed have been excluded from this report. This group of patients represents the only large series of straight ERPTs available for comparison with the various reservoir modifications that have been reported. All operations were performed under the direction of the author. The mean age at surgery was 20.6 +/- 9.8 years, with a range of 1 to 48 years. Forty-six patients were younger than 18 years at the time of operation. All patients with ulcerative colitis and familial polyposis underwent a temporary loop ileostomy with total abdominal colectomy with ERPT; the 10 infants and children with Hirschsprung's disease underwent the total colectomy and ERPT without a back-up ileostomy. There were two deaths in this series, one from fulminate hepatic failure in the late postoperative period and the other from multiple bowel fistulas and sepsis in a teenager with Crohn's disease, in whom the initial diagnosis was ulcerative colitis. Follow-up has ranged from 3 months to 15 years. There were 13 cases of adhesive bowel obstruction, seven of which required an enterolysis. Pelvic sepsis occurred in three patients, two of whom required operative drainage. Two women developed rectovaginal fistulas, which healed with temporary diversion. Minor wound infections occurred in five patients. There were no anastomotic leaks, nor were any cases of pouchitis encountered. In five patients permanent conversion to a Brooke ileostomy was required. Mean stool frequency 3 years after surgery was 7.7 per 24 hours. Daytime continence was achieved in all patients. Occasional nocturnal soiling occurred in 11.1% of patients at 1 year and was absent by 3 years. Neither age nor diagnosis (ulcerative colitis versus familial polyposis) affected stool frequency.(ABSTRACT TRUNCATED AT 400 WORDS)