(Congenital Megacolon; Colonic Aganglionosis)
by Cynthia M. Johnson, MA
Hirschsprung disease (HD) is an area of the colon (large intestine) that has stopped working the right way. It isn’t able to push stool through as it should, which can lead to a partial or full blockage. This can lead to serious infections and harm to the colon.
HD is present at birth.
The colon is a tube made of muscles. They squeeze then relax to help move waste out of the body. The work of the colon is controlled by a number of nerves. In HD, the nerves that tell the colon to relax are missing in one area. This means that part of the colon can never relax and fully open. Waste movement can get slowed or stopped in the area. It often happens in the lower part of the colon but can sometimes include the whole colon.
The missing nerves do not develop as the fetus grows. It is not clear why this happens. Some families may have a strong history of HD. In this case, it is probably due to a problem with genes.
HD is more common in boys. Other things that may raise your child's chance of HD are:
Symptoms often show soon after birth. HD that is less serious may not be noticed until later in life.
Symptoms found in newborns include:
Symptoms found in older infants and children are:
HD is often diagnosed when an infant fails to have a bowel movement within 24 hours of birth. In older children, the doctor will ask about your child's symptoms and health history. A physical exam will be done.
Your child may have:
HD needs to be treated with surgery. The earlier it is done, the better the results may be.
The goal of surgery is to remove the part of the colon that does not work the right way. Here are the surgery types:
HD can’t be prevented.
If you have one child with the disease, you could have more children with the disease. Genetic counseling can help you learn more about the risk.
International Foundation for Functional Gastrointestinal Disorders
National Institute of Diabetes and Digestive and Kidney Diseases
About Kids Health—The Hospital for Sick Children
Hirschsprung disease. Family Doctor—American Academy of Family Physicians website. Available at: https://familydoctor.org/condition/hirschsprungs-disease/. Updated March 1, 2014. Accessed June 26, 2018.
Hirschsprung disease. EBSCO DynaMed Plus website. Available at: http://www.dynamed... . Updated July 24, 2015. Accessed June 26, 2018.
Hirschsprung’s disease treatment. UCSF Benioff Children's Hospital website. Available at: https://www.ucsfbenioffchildrens.org/conditions/hirschsprungs_disease/treatment.html. Accessed June 26, 2018.
What I need to know about Hirschsprung disease. National Institute of Diabetes and Digestive and Kidney Diseases website. Available at:
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Updated September 2015. Accessed June 26, 2018.
Last reviewed May 2018 by Karri Kassir, MD
Last Updated: 6/26/2018
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