Total colon aganglionosis: report of nine cases.

Abstract

Nine infants had aganglionosis of the entire colon. Bile-stained vomiting was the most consistent symptom. The bowel evacuation pattern varied from normal meconium passage with subsequent diarrhea to total obstipation. Plain roentgenograms showed a partial or complete small bowel obstruction, and results of the barium enema (seven patients) were interpreted as normal in four and suggestive of total aganglionosis in three. Intraoperative findings consisted of dilated small bowel, which becomes narrow in its terminal portion, and a small or normal colon. There were two deaths, one with aganglionosis of the entire intestine and one incorrectly diagnosed. Initial therapy in six of the patients was an ileostomy in ganglionic bowel, and final operative treatment was either a Swenson or Duhamel procedure. One patient who had an associated ileal atresia has a permanent ileostomy.