Management of Hirschsprung's disease in adolescents

It is unusual for Hirschsprung's disease to go undiagnosed until late childhood. Adolescents presenting with Hirschsprung's disease offer unique challenges to the surgeon because of the massively dilated and hypertrophied colon that is invariably present. Five adolescents (four males and one female; ages, 11 1/2 to 16 1/4 years) with newly diagnosed Hirschsprung's disease were managed by slightly different methods between January 1981 and March 1987. Three patients had a "leveling" colostomy, followed by a Swenson procedure in two and a Duhamel procedure with a temporary diverting colostomy in one. One patient had a primary Duhamel procedure with a temporary diverting ileostomy. One patient had a primary Swenson procedure without diversion. Long-term results were uniformly excellent. Based on the experience with these patients the following principles of management emerge: 1) Avoid loop "leveling" colostomies because of their grotesque size and their tendency to retract or prolapse. 2) Rectal tube decompression can often adequately prepare a patient for a primary pull-through procedure. 3) When a diverting ostomy is required, it should be an ileostomy rather than a colostomy because its subsequent closure will not endanger the marginal artery, which if divided, could compromise the blood supply to the pulled-through colon. 4) The Swenson procedure, although difficult in adolescents, can be done with excellent long-term results. 5) Several applications of the stapling instrument are required in the Duhamel procedure to fully divide the septum between the aganglionic rectum anteriorly and the normal colon posteriorly.