An ileostomy at the aganglionic ileum for total colon aganglionosis

For the initial management of total colon aganglionosis (TCA), an ileostomy is usually created at the distal end of the ganglionic bowel, which is ass ociated with profuse diarrhea resulting in a water and electrolyte imbalanc e as well as malnutrition during the ileostomy period. An ileostomy at the aganglionic ileum several centimeters toward the anal side of the transitio n for TCA is thus expected tb cause bowel retention without any bowel obstr uction and to facilitate water reabsorption in the small bowel. We performe d this procedure in 2 babies with TCA. The length of the aganglionic ileum was 50 cm and 10 cm, respectively. The ileostomies were made 5 cm distal to the transition at 1 month and 1 day after birth, respectively. Oral feedin gs were well tolerated, resulting in adequate weight gain and the stools we re semisolid during the ileostomy periods. Definitive operations using the Duhamel procedure with a right colon patch graft without the creation of a new ileostomy were successfully performed at 7 and 3 months of age, respect ively. The postoperative courses were satisfactory. An ileostomy at the aga nglionic ileum appears to be the method of choice as initial management for patients with TCA.