Congenital aganglionosis causes pseudoobstruction
•Hirschspung’s disease results from aganglionosis of
the terminal colon.
•Associated with the development of megacolon.
•Relatively common disease
–1/5000 births in general
population
–1/500 births in Mennonites (due to inbreeding
•Most commonly due to defect in RET > EDNRB.
