Total colonic aganglionosis: treatment and follow-up

We reviewed the problems during diagnosis and therapy of 11 patients with total colonic aganglionosis. Better knowledge of the clinical picture and greater alertness for the occurrence of this disease has led to an earlier diagnosis. Definitive operative therapy is still controversial. After ileostomy, a crucial point seems to be the allowance of sufficient time for the small bowel to adapt its absorptive ability. Little seems to be gained by performing a difficult procedure, such as Martin's operation, which involves the risk of serious complications. Our experience confirms that a standard Hirschsprung's operation is, in general, satisfactory. At present (mean follow-up 6.7 years), all our patients show normal growth and development, have no serious peri-anal problems, and are (with one exception) continent for faeces.