Hirschsprung Disease :: Nationwide Children's Hospital

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Hirschsprung Disease

Hirschsprung disease is a condition you are born with, which causes blockage of the intestine. The reason for this blockage is a lack of nerves in the bottom segment of the colon. These nerves normally allow the muscles in the wall of the bowel to contract and thus move digested material downstream toward the anus, so the child can eliminate his or her waste.

Hirschsprung disease occurs in 1 out of every 5,000 live births. The disease occurs more often in males than in females, and sometimes can be associated with inherited conditions, one common example being Down syndrome. Hirschsprung disease, also known as megacolon, is a congenital (happening before birth) condition in which nerve cells in the lining of the intestine do not develop properly. These nerves are responsible for the wave-like motion that helps push food through the intestines so that it can be digested and passed out of the body as stool.

A child’s large intestine is about five feet long – and Hirschprung disease can affect either a small or large portion of the organ. The condition occurs most commonly in the upper rectum or the sigmoid colon, which is the very last part of the large intestine before stool reaches the anus.

Children with Hirschsprung disease will always need surgery to remove the non-functional segment of the intestine, and to restore the ability to push stool through the digestive tract and out of the body. 

With proper surgical intervention, the long-term outcomes for children with Hirschprung disease are excellent, and most will go on to recover normal bowel control and function. 

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