An evaluation of the modified endorectal procedure for Hirschsprung's disease.

During the past 4 yr, 60 patients with Hirschsprung's disease have been treated at our institution. Twenty-eight patients within this group have undergone a modified endorectal pull-through procedure. There are 18 males and 10 females in the series. There are 5 children with total colonic involvement, 3 patients with aganglionosis to the mid-transverse colon, and one child with Hirschsprung's disease extending to the distal jejunum. The associated anomalies have consisted of four cases of Down's syndrome, one of whom also has transposition of the great vessels. There has been no operative-related mortality or late mortality in our series. Two major complications (anastomotic leak following a colostomy closure and incorrect pathological diagnosis of aganglionic level) and five minor complications have occurred. The average follow-up period is 31.3 mo. The functional results postoperatively have been excellent. The 18 patients over the age of 3 yr are completely toilet-trained. The remaining patients are too young to be toilet-trained, but are continent in the sense that they do not soil between bowel movements. Because of the satisfying functional results in this series, we have now expanded our indications for this procedure to patients requiring total colectomy for ulcerative colitis and familial polyposis. During the past 2 yr, 8 patients with ulcerative colitis have undergone the endorectal pull-through together with a total colectomy.