Hirschsprung disease

Abstract

Hirschsprung disease refers to congenital aganglionosis of some or all of the colon. Patients typically present with constipation early in life. The diagnosis and treatment of Hirschsprung disease has evolved over time but nearly always includes an operation to remove and replace the aganglionic bowel. These operations demand technical precision and a thoughtful approach. Patients with Hirschsprung disease also often require intensive and sometimes life-long follow-up care to achieve a successful outcome.