What is Hirschsprung’s Disease?

Hirschsprung’s disease (HD) is a disorder of the abdomen that occurs when part or all of the large intestine or lower parts of the gastrointestinal tract have no nerves and therefore cannot function. During normal fetal development, cells from the neural crest migrate into the large intestine (colon) to form the networks of nerves called Auerbach’s plexus and Meissner’s plexus. In Hirschprung’s disease, the migration is not complete and part of the colon lacks these nerve bodies that regulate the activity of the colon. The affected segment of the colon cannot relax and pass a stool through the colon, creating an obstruction.  In most affected people, the disorder affects the part of the colon that is nearest the anus. In rare cases, the lack of nerve bodies involves more of the colon.

Typically, Hirschsprung’s disease is diagnosed shortly after birth, although it may develop well into adulthood, because of the presence of megacolon, or because the baby fails to pass the first stool meconium within 48 hours of delivery. Normally, 90% of babies pass their first meconium within 24 hours, and 99% within 48 hours. Other symptoms include: green or brown vomit, explosive stools after a doctor inserts a finger into the rectum, swelling of the abdomen, lots of gas and bloody diarrhea.

The relevance of understanding Hirschsprung’s disease for paramedics is relatively limited to the fact that paramedics will treat these patients for associated conditions such as abdominal pain, bowel obstructions, nausea and vomiting. Consequently, it is important to have some background understanding of the disease.