Total colonic aganglionosis (with or without ileal involvement): a review of 27 cases.

From 1960 to 1984, 27 cases of total colonic aganglionosis were treated at the Sick Children's Hospital in Paris; in 19 cases there was ileal involvement, 16 of them extending more than 15 cm above the ileocecal valve. Five had a family history of Hirschsprung's disease. Nine infants died without having had definitive surgery, because of delayed diagnosis, or intractable malabsorption in extensive ileal aganglionosis. Two cases were diagnosed only at the ages of 6 and 13 years. Eleven children had Martin's modification of the Duhamel operation, the oldest of these being now 13 years old; and one girl aged 13 underwent a Swenson operation with ileoanal anastomosis, and one child has had a Kimura procedure. Four infants still have a diverting ileostomy. One out of the 14 operated children died 3 years after operation with fulminating enterocolitis. Late surgical nutritional results are analyzed with regard to the length of the side-to-side ileocolorectal anastomosis, and to the size of the ileorectal anastomosis, on which adequate pouch emptying depends. The essential problem in total colonic aganglionosis is not the surgical management of the condition, but rather its prompt diagnosis and the handling of the neonatal intestinal obstruction.