Hirschsprung's disease: a cause of chronic constipation in children.

Abstract

Constipation is common in neonates and children. Most constipation is benign and easily treated. Hirschsprung's disease is an unusual, but well-recognized cause of chronic constipation. An example of the group of disorders known as dysganglionoses, Hirschsprung's disease is characterized by an absence of the intramural ganglionic cells at the submucosal and myenteric levels of the intestine. The course of the disease is variable, with five distinct patterns of varying severity. Without treatment, mortality approaches 50 percent by one year of age, but early diagnosis and intervention are associated with a favorable outcome. The treatment is surgical, and several approaches are currently used.