Surgical outcomes of total colonic aganglionosis in children: A 26-year experience in a single institute

Background There is a lack of consensus regarding the treatment of total colonic aganglionosis (TCA) with respect to perioperative morbidity, mortality, complications, and functional outcomes. The aim of this study was to review the results of surgical TCA treatment over a 26-year period and characterize the outcomes. Methods We retrospectively reviewed the clinical characteristics, surgical courses, and outcomes of TCA patients who underwent definitive pull-through operations from 1986 to 2012. Follow-up data were collected by chart reviews and telephone interviews using a standardized questionnaire. Results We identified nine infants with TCA (8.6%) from among 105 infants with Hirschsprung's disease treated during the 26-year period. Neither sex predominated (male/female ratio = 4:5). All infants underwent laparotomies and simultaneous enterostomies. All patients eventually underwent modified Duhamel pull-through procedures at a mean age of 179 days (range, 47–352 days). Two infants died of complications after surgery including heart failure and sepsis. The remaining infants recovered smoothly with antilaxative medications, and all but one was weaned off these medications. Although the surviving patients did not catch up on growth, they and their families were satisfied with the surgical results. Conclusion Infants with TCA had satisfactory outcomes after the modified Duhamel pull-through operation. Based on our experience, we suggest that the pull-through operation could be performed earlier, even when there are loose stools from the enterostomy.