Hirchsprung Disease is aganglionosis of the colon with absence of parasympathetic ganglia in mucosal and submucosal layers of colon. This is a result of the failure of normal cranial-caudal migration of ganglion cells. The most common transition site is the rectosigmoid colon. Total colonic aganglionosis is rare.

The incidence is 1 in 5,000-8,000 live births, with a male to female ratio of 4-9 to 1. Patients may present with failure to pass meconium within the first 24 hours of life or later with constipation and paradoxical diarrhea (25%). The treatment is a surgical pull-through procedure.