Treatment

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 The initial treatment requires performing a "leveling" colostomy in the most distal colon with ganglion cells present. This requires exploration laparatomy with multiple seromuscular biopsies of the colon wall to determine the exact extend of the aganglionosis. The colostomy is placed above the transition zone. Placement of the colostomy in an area of aganglionosis will lead to persistent obstruction
 Once the child has reached an adequate size and age (6-12 months; 20 pounds or more), a formal pull-through procedure is done. Three pull-through procedures are currently in use for treating Hirschsprung's disease. The first of these is the original Swenson procedure, in which the aganglionic rectum is carefully dissected in the pelvis and removed down to the anus. The ganglionic colon is then anastomosed to the anus via a perineal approach. In the Duhamel procedure, dissection outside the rectum is confined to the retrorectal space, and the ganglionic colon is anastomosed posteriorly just
 above the anus. The anterior wall of the ganglionic colon and the posterior wall of the aganglionic rectum are anastomosed, using a stapling device. In Soave's operation, dissection is entirely within the rectum. The rectal mucosa is stripped from the muscular sleeve, and the ganglionic colon is brought through this sleeve and anastomosed to the anus. Complications with all procedures include enterocolitis, constipation and anastomotic stricture, but long-term results with the three procedures are comparable and generally excellent in experienced hands. These three procedures also can be
 adapted for total colonic aganglionosis; the ileum is used for the pull-through or anastomosed to the aganglionic segment of distal colon and rectum to improve absorption.