Curated Content
Sourced from St. Louis Children’s Hospital
What is Hirschsprung disease?
Hirschsprung disease is a serious birth defect where nerve cells are completely missing from the end of the bowel. Normally, the esophagus, stomach, small intestine and colon have about 500 million nerve cells (i.e. neurons) that control almost everything the gut does. These nerves and their supporting glia are called the enteric nervous system (ENS), a system complex enough that it is sometimes referred to as “the second brain”.
Important functions of the enteric nervous system include controlled movement of food from the beginning to the end of the bowel. The ENS also controls intestinal blood flow, regulates the cells that line the intestine, and influences intestinal inflammation.
Most children with Hirschsprung disease (i.e., about 80 percent) are missing enteric neurons from just a few inches at the end of the colon. Some children with Hirschsprung disease, however, are missing enteric neurons from the entire colon, or the entire colon and part of the small bowel.
Hirschsprung disease symptoms vary dramatically from person to person. This variability is not dependent on the length of bowel that is missing enteric neurons, and is a subject for ongoing research.
There are several “classic” symptoms that may suggest a diagnosis of Hirschsprung disease.
Read the full article at St. Louis Childrens’s Hospital.