Modified Endorectal Procedure for Management of Long-segment Aganglionosis

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Abstract
During the past 5 yr, 65 patients with Hirschsprung''s disease were treated. Ten of these patients had long segment aganglionosis, 5 with total colonic involvement, 4 with aganglionosis to the midtransverse colon and 1 child with Hirschsprung''s disease extending to the distal jejunum. There are 6 females and 4 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. This 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 10 of these patients were definitively treated with a modified endorectal pull-through procedure. The mortality rate with this procedure was zero. There was 1 major complication (incorrect pathologic diagnosis of the level of aganglionosis) and 4 minor complications. The average follow-up period is 42.6 mo. The functional results were excellent with an average of 3-4 soft bowel movements/day. All of the children over 3 yr of age are completely toilet trained. The remaining 2 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.