Modified endorectal procedure for management of long-segment aganglionosis.

During the past five years, 65 patients with Hirschsprung's disease have been treated at our institution. Ten of these patients had long-segment aganglionosis, five with total colonic involvement, four with aganglionosis to the midtransverse colon, and one child with Hirschsprung's disease extending to the distal jejunum. There are six females and four males in the series, which is a reversal of the normal sex ratio for aganglionosis. An extensive literature review of long-segment Hirschsprung's disease has revealed a total of 489 reported cases. The mean frequency of long-segment aganglionosis in the literature is 12% of all cases of aganglionosis. Our series is comparable, with an incidence of 15%. The overall mortality rate in the literature of 54% indicates the poor therapeutic results obtained with this disease in the past. All ten of our patients have been definitively treated with a modified endorectal pull-through procedure. The mortality rate with this procedure has been zero. There has been one major complication (incorrect pathologic diagnosis of the level of aganglionosis) and four minor complications. The average follow-up period is 42.6 months. The functional results have been excellent with an average of three to four soft bowel movements per day. All of the children over 3 years of age are completely toilet trained. The remaining two patients are too young to be toilet trained, but are continent in the sense that they do not soil between bowel movements. Awareness of this entity of long-segment aganglionosis should lead to earlier diagnosis, appropriate operative therapy, and lower mortality rates.