Pediatric Radiology > Abdominal > Neonatal > Hirschsprung Disease

Hirschsprung Disease

Contrast shows reduced size of rectum

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.

The transition zone is near the splenic flexure.  

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