Total colonic aganglionosis. Analysis of 16 cases.

Sixteen of 116 infants (14 percent) with Hirschsprung's disease had total colonic aganglionosis. Treatment was delayed in 6 of 16 patients because the condition was not recognized. Diagnosis was eventually achieved by rectal biopsy and the extent of aganglionosis was documented after multiple intestinal biopsies. All patients were initially managed by diverting enterostomy. Ileoanal pull-through operation was performed in nine patients at 12 to 24 months of age with no operative mortality. The overall mortality rate was 25 percent, including the deaths of two infants with diagnostic delay. Survivors had normal growth and satisfactory bowel function, Hirschsprung's disease should be suspected and rectal biopsy performed in infants with persistent or intermittent abdominal distention and constipation. Instances of total colonic aganglionosis can be documented by appropriate biopsies at the time of laparotomy. The modified Duhamel operation is an acceptable procedure for this condition. A ganglionic antimesenteric patch enteroplasty may prove a useful adjunct in infants with extensive aganglionosis involving the proximal small bowel.