Comparison of long-term outcome according to involved aganglionic segments of total colonic aganglionosis

Total colonic aganglionosis (TCA) is a rare form of Hirschsprung disease, with more severe symptoms than rectosigmoid Hirschsprung disease. We aimed to evaluate the surgical outcomes according to the involved segments of TCA. Patients with aganglionosis extending from the anus to at least the ileocecal valve were included. The medical records of 33 TCA patients from 1981 to 2014 were reviewed. Three groups were analyzed based on the involved segment (jejunum, jejunoileal junction, and distal ileum). The median age at the pull-through operation was 6.2 (3.3–114) months. The median follow-up duration was 216 (21–411) months. Transition zone in the jejunum, jejunoileal junction, and distal ileum was identified in 3, 5, and 25 patients, respectively. The most common method of operation was Duhamel pull-through. Perianal excoriation and enterocolitis were the most common postoperative complications. The complication rates were 45% to 51% and not different among the groups. The defecation frequency normalized 3 years postoperatively, and body weight started to recover after 2 years irrespective of the involved segment. Therefore, close monitoring with proper management of defecation and body weight for at least 2 to 3 years postoperatively is required.