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.