LONG-TERM RESULTS OF SURGICAL-TREATMENT IN INFANTS WITH TOTAL COLONICAGANGLIONOSIS

The authors describe the long-term results of the surgical treatment o f total colonic aganglionosis (TCA) in nine boys who have been treated since 1975. The principal operative procedure was endorectal pull-thr ough of the ganglionic ileum, with the aganglionic colon as an onlay p atch. The procedure was employed in five infants. using the right or l eft colon. Ileorectostomy with an onlay patch was used in two patients ; ileoanostomy without the patch was used in two. The distal end of th e pulled-through ileum was, on average, 39 cm from the ileocecal junct ion. The average follow-up period was 11.0 years. Diarrhea and distens ion were temporary after the pull-through, and all patients ultimately tolerated normal feeding. Considering the variation in operative tech niques, we were unable to demonstrate any advantage of one procedure o ver another. Z scores for height and weight at the last follow-up exam ination correlated with the weight gain before the definitive surgery and inversely correlated with the length of resected distal ileum at t he time of operation. Copyright (C) 1994 by W.B. Saunders Company