Two cases of the entire colon aganglionosis were presented; one was involved with the aganglionosis bellow the 25 cm from the terminal ileum and an ileostomy was made at 55 cm from the terminal ileum. The other, involved bellow the cecum and an ileostomy was made at 25cm from the terminal ileum. A low residue diet (LRD), clinimeal^<[○!R]> was substituted for the postoperative oral nutrition without using intravenous hyperalimentation. Daily amount of stool excreted from the ileostomy stoma, ranged from 50〜100 g, and their body weight gain was sufficient. They were undergone Duhamel-Ikeda's operation with Martin's procedure at the age of 5 and 6 months respectively. The postoperative courses were uneventful with the low residue diet. The laboratory dada through their clinical courses, revealed relatively low levels of serum total cholesterol and transient high alkaline phosphatase levels. Althogh the analysis of fatty acid in plasma showed normal in one case, the analysis of amino acid in plasma showed mild elevation of valine, proline, and glycin in both cases. In conclusion, clinimeal is considered to be very effective for the postoperative nutritional management in the patient with entire colon aganglionosis.
