Hirschsprung Disease :: Nationwide Children's Hospital

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Hirschsprung Disease

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.

When the nerves are missing, stool gets “backed up” in the intestines. The bacteria that are normally present there begin to overgrow, causing an infection called enterocolitis, which leads to severe diarrhea and dehydration.  In some cases, the blockage can lead to a bowel perforation (a hole in the intestine) and a potentially deadly infection.

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.

Hirschsprung disease occurs in 1 out of every 5,000 live births and develops while a baby is still growing inside the womb. More boys than girls are affected, and children with Down syndrome have a higher incidence. The condition may also be hereditary. There is a slightly increased chance that a couple will have a child with Hirschsprung disease if one of the parents has the condition, or if they have already had one child with the condition.

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