Long-term results of surgical treatment in infants with total colonic aganglionosis

The authors describe the long-term results of the surgical treatment of total colonic aganglionosis (TCA) in nine boys who have been treated since 1975. The principal operative procedure was endorectal pull-through of the ganglionic ileum, with the aganglionic colon as an onlay patch. The procedure was employed in five infants, using the right or left colon. Ileorectostomy with an onlay patch was used in two patients; ileoanostomy without the patch was used in two. The distal end of the pulled-through ileum was, on average, 39 cm from the ileocecal junction. The average follow-up period was 11.0 years. Diarrhea and distension were temporary after the pull-through, and all patients ultimately tolerated normal feeding. Considering the variation in operative techniques, we were unable to demonstrate any advantage of one procedure over another. Z scores for height and weight at the last follow-up examination correlated with the weight gain before the definitive surgery and inversely correlated with the length of resected distal ileum at the time of operation.