What Is Hirschsprung Disease?
Hirschsprung disease causes blockage of the intestine. The nerves in the colon (also called the large intestine or bowel) that move poop (stool) forward did not form. During digestion with a normal colon, stool will move all the way through the bowel. With Hirschsprung disease, healthy muscles and nerves of the colon push stool and gas through the colon until it reaches the part with no nerves (ganglion cells). At this point, the stool stops moving and gas builds up in the colon. This causes the healthy area of the colon to become larger. Usually, the part of the colon without ganglion cells is very narrow. Stool building up in the colon can cause serious problems, like infection.
How Is Hirschsprung Disease Treated?
Treatment for Hirschsprung disease may vary per child. With the right treatment (intervention), the long-term outcomes for children with Hirschsprung disease are excellent. Most children will go on to recover normal bowel control and function.
Surgical Treatment for Hirschsprung Disease
Hirschsprung disease is treated with a surgery called posterior sagittal anorectoplasty (PSARP) or a pull-through procedure. Hirschsprung disease pull-through surgery removes the part of the colon without nerve cells and connects the healthy colon to the rectum. It can be done through the opening of the anus. Sometimes, small incisions may need to be made on the belly so that the surgeon can see better with a scope instrument. Pull-through surgery accomplishes two important things:
- Removes the abnormal part of the intestine.
- Reconnects a healthy part of the intestine to the anus so that the body can pass waste normally.
Most pull-through procedures are done when a child is between one and six months old.
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