Diagnosis and management of Hirschsprung's disease. A 25 year perspective.

The demographic data and the distribution of the extent of involved bowel in Hirschsprung's disease have remained unchanged over the past 25 years and are similar to those reported by others. The age at which the diagnosis was established decreased initially but increased in the last 10 year period. This increase has been accompanied by an increase in the number of breast-fed infants. Definitive operation for Hirschsprung's disease is now usually performed in children less than 18 months of age. The procedures used were selectively applied in the most recent 10 years. Arbitrary dependence on a single procedure is no longer appropriate and individualized judgment should be used in the application of specific treatment alternatives in the surgical management of Hirschsprung's disease. Mortality after definitive operation is now confined to problems other than Hirschsprung's disease. The patient with undiagnosed Hirschsprung's disease may not survive enterocolitis despite aggressive contemporary resuscitative treatment.