The definitive management of Hirschsprung's disease with the endorectal pull-through procedure

Abstract

From 1 July 1974 to 31 August 1985, 99 patients with Hirschsprung's disease were treated at the University of Michigan Mott Children's Hospital; 75 were initially diagnosed and treated at our institution. Of these 75 patients, 55 had standard rectosigmoid disease and 20 had long-segment disease. The endorectal pull-through (ERPT) procedure was used consecutively in 73 of the 75 patients; the other two children died, one because of total intestinal aganglionosis and the other due to severe congenital anomalies, prior to a definitive procedure. Of the 24 patients referred by other institutions, 12 underwent repeat pull-through procedures and 12 required lesser operations to deal with the complications of prior procedures. There were no postoperative deaths and no permanent enterostomies among the 73 consecutive ERPT patients. The follow-up in this series ranges from 4 months to 10 years, and all patients have fecal continence with an average of 3–4 stools daily. Our treatment plan involves the use of suction rectal biopsy for diagnosis and leveling colostomy in the neonatal period. An endorectal pull-through procedure is then carried out between 8 months and 1 year of age. The excellent functional results, 0% operative mortality, low morbidity, and technical ease of performing the ERPT underscore our enthusiasm for continued use of this procedure in Hirschsprung's disease.