Anorectal function after total colectomy with ileoanal anastomosis for total colonic aganglionosis

Background/purpose Total colonic aganglionosis is a rare form of Hirschsprung’s disease. Many techniques have been described for its management to improve the outcome as regards anastomotic leakage, recurrent constipation, and incontinence. The aim of this study was to assess the function of anorectum as regards the frequency and continence using the standard scoring system. Patients and methods In this retrospective study, 15 patients were evaluated after treatment in Zagazig University Hospitals. All cases underwent transanal endorectal pull-through with ileoanal anastomosis. All patients were evaluated by applying the Wexner score at 1, 3, and 6 months postoperatively. Results Fifteen infants were included in the study. None of them reached a high score level at sixth month postoperatively (i.e. totally incontinence). On applying the scoring system, the mean±SD result of all patients at first postoperative month was 15.0667±2.52039; at the second evaluation in the third month postoperatively the mean±SD score was 11.0667±3.28344, and lastly at the sixth month evaluation the mean±SD was 5.8667±2.82506. All results were significant on comparing the third month results with the first month results and on comparing the sixth month score results with either the first or the third month postoperatively. Conclusion Although total colonic aganglionosis is a rare form of Hirschsprung’s disease, it needs staged surgical intervention. The most important stage is the second definitive one. Despite variable methods described for reconstruction after total colectomy, ileoanal one carries less major surgical complications and acquires accepted anorectal functional results with more improvement with time postoperatively.