(Congenital Megacolon; Colonic Aganglionosis)
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- Family members with the disease
- Sex: male
- Presence of Down Syndrome
- Presence of other congenital defects
- Failure to have a bowel movement within the first 48 hours of life
- Vomiting after eating
- Swelling of abdomen
- Severe constipation for most of their lives
- Barium enema with x-ray
- Pull-through operation—The affected area of the colon is removed. The remaining healthy colon is then brought down and joined to the rectum.
- Colostomy—This may be done to allow your bowel time to rest and heal. After the affected area of colon is removed, the healthy colon is not immediately connected to the rectum. Instead, the colon is attached to an opening in the abdominal wall. Waste will then pass through this opening and into a bag outside the body. This may be done in children who are very sick or have a large portion of the colon affected.
- Closure of the colostomy—After the area has healed, the colon will be connected to the rectum. The colostomy opening will be closed. Bowel function will gradually return to normal.
International Foundation for Functional Gastrointestinal Disorders http://www.aboutkidsgi.org
National Digestive Diseases Information Clearinghouse http://digestive.niddk.nih.gov
Health Canada http://www.hc-sc.gc.ca
Hirschsprung disease. EBSCO DynaMed website. Available at: https://dynamed.ebscohost.com/about/about-us . Updated January 24, 2013. Accessed June 27, 2013.
Hirschsprung's disease. American Academy of Family Physicians Family Doctor website. Available at: http://familydoctor.org/familydoctor/en/diseases-conditions/hirschsprungs-disease.html . Updated July 2010. Accessed June 27, 2013.
Hirschsprung's disease. National Digestive Diseases Information Clearinghouse website. Available at: http://digestive.niddk.nih.gov/ddiseases/pubs/hirschsprungs%5Fez/ . Updated May 10, 2012. Accessed June 27, 2013.
Hirschsprung’s disease treatment. University of California San Francisco Benioff Children's Hospital website. Available at: http://www.ucsfhealth.org/childrens/edu/hirschsprung.pdf . Accessed June 27, 2013.
- Reviewer: Michael Woods, MD
- Review Date: 06/2013
- Update Date: 05/11/2013