A ten-year experience with ninety-two cases of Hirschsprung's disease. Including sixty-seven consecutive endorectal pull-through procedures

Abstract

From July 1974 through November 1984, 92 patients with Hirschsprung's disease (congenital aganglionosis) have been treated at the University of Michigan-Mott Children's Hospital. This series includes 67 consecutive modified endorectal pull-through (ERPT) procedures in children. Fifty-one of these 67 patients had standard rectosigmoid disease and underwent a successful ERPT with only two major complications. One of these 51 children underwent a successful ERPT but died in the late postoperative period from severe congenital heart disease. Eighteen of the 92 patients suffered from total aganglionosis or long-segment disease. Sixteen of these have undergone an ERPT with no mortality or operative morbidity. The follow-up ranges from 6 months to 10 years. All of the children who have reached 3 years of age are continent. Of the remaining 23 patients, 12 were referred following an unsuccessful pull-through at another hospital. The 12 operations included five Swenson pull-throughs, five Duhamel procedures, one ERPT, and one subtotal colectomy. It was possible to redo or revise the pull-through procedures successfully in all but one patient, who required a permanent colostomy. Finally, 11 children were referred for management of a variety of complications following pull-through procedures performed at other institutions. None of these 11 patients required a reperformance of their pull-through, and all were successfully treated with lesser surgical procedures or with medical management. The excellent functional results and the low morbidity and zero operative mortality are attributed to the technical ease of performing the modified ERPT.