Hirschsprung's disease

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Causes

By Mayo Clinic staff

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Illustration showing colon and rectum 
Colon and rectum

It's not clear what causes Hirschsprung's disease. Doctors know that Hirschsprung's disease occurs when nerve cells that surround the colon don't form completely.

Normally, as a baby grows in the womb, bundles of nerve cells (ganglia) begin to form between the muscle layers along the length of the colon. This process begins at the top of the colon and ends at the bottom (rectum). In children with Hirschsprung's disease, this process does not finish, and the ganglia do not form normally along the entire length of the colon. Sometimes the cells are missing from only a few centimeters of the colon. Other times a longer portion may be affected.

References
  1. What I need to know about Hischsprung disease. National Institute for Diabetes and Digestive and Kidney Disease. http://digestive.niddk.nih.gov/ddiseases/pubs/hirschsprungs_ez/. Accessed Oct. 12, 2010.
  2. Wyllie R. Motility disorders and Hirschsprung disease. In: Kliegman RM. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa.: Saunders Elsevier; 2007. http://www.mdconsult.com/das/book/body/208746819-6/0/1608/0.html. Accessed Oct. 12, 2010.
  3. Kahn E, et al. Anatomy, histology, embryology and development anomalies of the small and large intestine. In: Feldman M, et al. Sleisinger and Fordtran's Gastrointestinal and Liver Disease. 9th ed. Philadelphia, Pa.: Saunders; 2010. http://www.mdconsult.com/book/player/linkTo?type=bookHome&isbn=978-1-4160-6189-2&eid=4-u1.0-B978-1-4160-6189-2..X0001-7--TOP&uniq=200844987-3. Accessed Oct. 12, 2010.
  4. Philichi L. When the going gets tough: Pediatric constipation and encopresis. Gastroenterology Nursing. 2008;31:121.
  5. Nutrition therapy for constipation. ADA Nutrition Care Manual. http://nutritioncaremanual.org/index.cfm. Accessed Oct. 12, 2010.
DS00825 Nov. 11, 2010

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