Hirschsprung disease
ORPHA:388· ICD-10 Q43.1
Definition
A rare congenital intestinal motility disorder that is characterized by signs of intestinal obstruction due to the presence of an aganglionic segment of variable extent in the terminal part of the colon.
- Prevalence
- 1-5 / 10 000
- Inheritance
- Autosomal dominant, Autosomal recessive, Multigenic/multifactorial, Not applicable
- Age of onset
- Childhood, Infancy, Neonatal