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     Hirschsprung's (HIRSH-sproongz) disease (HD) is a condition that affects the portions of the large intestine (colon) and causes problems with passing stool. The condition is present at birth (congenital) as a result of missing nerve cells in the muscles of the baby's colon. Nerves in the colon control the muscle contractions that move food through the bowels. Without the contractions, stool stays in the large intestine. A newborn who has Hirschsprung's disease usually can't have a bowel movement in the days after birth. There are two main types of Hirschsprung's disease, known as short-segment disease and long-segment disease, defined by the region of the intestine lacking nerve cells (aganglionic segments).

     

     Total colonic Hirschsprungs disease (or TCHD) specifically consists of aganglionosis (affected area) from the anus to at least the ileocecal valve in the small intestine. Total colonic aganglionosis is a relatively uncommon form of Hirschsprung's disease. It occurs in approximately 2-13 % of cases and involves the entire colon which is aganglionic but may extend proximally into varying lengths of small bowel. As a result, it should be separated into Total colonic aganglionosis (TCA) [defined as aganglionosis extending from the anus to at least the ileocaecal valve but no more than 50 cm small bowel proximal to the ileocaecal valve] and total colonic and small bowel aganglionosis (TCSA) which may involve very long segments of small bowel aganglionosis.

     

It's not clear what causes Hirschsprung's disease. It sometimes occurs in families and might, in some cases, be associated with a genetic mutation.

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More about Hirschsprungs disease from NORD

 

 Copyright © 2012. Published by Elsevier Inc.