The colon patch graft procedure for extensive aganglionosis: long-term follow-up.

PURPOSE: During the last 17 years, the authors have used the colon patch graft (CPG) procedure to treat 11 patients with extensive aganglionosis. This study evaluates the effectiveness of the CPG procedure in treating patients with this disorder.

METHODS

An initial ileostomy was created during infancy in all 11 patients. At 1 to 21 months (mean, 5.5 months) of age, an 11- to 40-cm (mean, 18 cm) side-to-side ileocolostomy (CPG) was created between the ileum and aganglionic ascending colon, (including aganglionic ileum in three patients). Ten patients later underwent a Swenson-type definitive operation, the CPG segment being brought down to the anus at 9 to 30 months (mean, 19 months) of age. These 10 patients have been followed up for growth, development, and bowel habit patterns for the subsequent 5 to 17 years (mean, 12 years).

RESULTS

Within 1 month after the CPG was created, intravenous nutrition could be discontinued and the patients treated at home until time for the definitive procedure. During this period, one patient died of aspiration. After the definitive operation, body weight for age returned to a normal range in 2 to 4 years. Currently, seven older patients have one to three bowel movements per day, whereas three younger patients have explosive diarrheal defecations. None are incontinent of stool. Iron deficiency anemia developed in four patients. Two are mentally retarded, but the others are doing well in school.

CONCLUSIONS

(1) The colon patch graft procedure effectively reduces "ileostomy diarrhea," shortening the period of intravenous nutrition to 1 month, (2) bowel habit patterns improve with the advance of time, (3) body weight for age returns to normal 2 to 4 years after the definitive procedure, and (4) during long term follow-up, patients must be evaluated for iron deficiency anemia.