Hirschsprung’s Disease

Synonyms

Aganglionic megacolon; Congenital megacolon

Definition

Hirschsprung’s disease is a common cause of neonatal intestinal obstruction due to improper muscle movement in the bowel. Patients are unable to defecate because of the lack of ganglion cells. It is a congenital condition; therefore, it is present from birth. Newborns will often have abdominal distension, while older children may suffer from chronic constipation. Hirschsprung’s disease results from the absence of ganglion cells within the myenteric and submucosal plexus of the rectum and/or colon. Colonic ganglion cells are derived from the neural crest and migrate caudally with the vagal nerve fibers along the intestine. These ganglion cells arrive in the proximal colon by 8 weeks of gestation and in the rectum by 12 weeks of gestation. The arrest in ganglionic migration leads to an aganglionic segment. This results in clinical Hirschsprung’s disease.

Clinical Features

• Incidence

  Its incidence is estimated to be 1 in 5,000...