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Hirschsprung’s Disease, What is it?

Hirschsprung’s (pronounced HURSH-sprungz) Disease (HD or HSCR) is a condition affecting the large intestine. It is also known as congenital megacolon & congenital intestinal aganglionosis.

When the baby starts growing in the womb, nerves called Ganglion cells grow in the intestine, starting at the top, working their way down the intestine to the anus. In HD, these cells do not grow the whole way down the bowel but stop before reaching the anus. This disease affects 1 in 5,000 babies and is more common in boys than in girls.

You may hear various terms for HD, Short Segment, Long Segment, Total Colon, Total Intestinal & Ultra-Short Hirschsprung’s. These terms describe how much of the bowel is aganglionic or lacking these cells. Do not be too confused by these terms as there is no strict definition and some specialists do not recognised the difference between Long & Short Segment HD.

Ganglion Cells. What’s their function?

The intestine pushes the stool down the bowel to the anus by wave movements, properly called peristaltic movements, of contracting and then relaxing. The ganglion cells job is to allow the bowel to relax. Without being able to relax, the bowel remains constricted and narrow. No stool can pass this point in the bowel and accumulates back up the bowel. The bowel then swells giving the appearance of the tummy looking bloated. When a contrast or barium x-ray is taken, the bowel looks like a ‘Y’ or ice cream cone, narrow at the bottom getting wider at the top where the stool is accumulating.

What are the symptoms?

The most common sign of Hirschsprung’s is that the baby does not pass the meconium within the usual 24 to 48 hours. Mind you this may have been passed by the baby just before birth but this is unusual. The meconium is the substance that lines the baby’s intestines during pregnancy.

Other Signs are

If your baby has some of these symptoms, don’t worry, the odds are it is not Hirschsprung’s but what you must do is to get your baby examined by a gastrointestinal doctor.

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