Hirschsprung's disease: three decades' experience at a single institution.

Experience with 250 patients treated for Hirschsprung's disease over the past three decades shows several interesting features. Only 58% had rectosigmoid disease. Twenty-six percent had long-segment disease, and 12% had total colon aganglionosis. This distribution did not change significantly over the decades. The male:female ratio was 3.3 in all groups, but dropped to 2.2 with total colon disease. The mean age at diagnosis decreased from 18.8 months during the 1960s to 2.6 months in the 1980s. The mean age at which the pull-through procedure was performed also dropped from 34 months to 15 months. Twenty-three patients died: 17 in the 1960s and three in each of the succeeding decades. The Swenson procedure was predominant during the 1960s, but was only 2% of the pull-throughs in the 1980s. The endorectal pull-through increased from 22% in the 1960s to 71% in the 1980s. In the 1970s and 1980s the Duhamel accounted for 26% of the pull-throughs. With a mean follow-up of 51 months, the mean number of episodes of post pull-through enterocolitis per patient was 0.53. This increased from 0.44 in the 1960s, to 0.52 in the 1970s, to 0.67 in the 1980s. The incidence of post-pull-through enterocolitis increased somewhat with the length of the bowel involved, and was greatest with the Duhamel (0.95 episodes per patient), less with the Swenson (0.60), and least with the endorectal pull-through (0.38). In contrast, the incidence of complications other than enterocolitis was greatest with the Swenson (20%), less with the endorectal pull-through (17%), and least with the Duhamel (13%).(ABSTRACT TRUNCATED AT 250 WORDS)