The colon (large intestine) holds stool (waste) left in the body after digestion. The colon is lined with nerves. These tell the colon when to contract (squeeze) and relax (open). The colon contracts and relaxes to push stool into the rectum. Stool leaves the rectum through the anus. In a child with Hirschsprung’s disease, nerves are missing in the anus and rectum and in part or all of the colon. The part of the colon without nerves can’t relax. So, stool can’t leave the body.
Hirschsprung’s disease is categorized based on how much colon is affected. Your child’s health care provider can tell you which type of Hirschsprung’s disease your child has. Types of Hirschsprung’s disease include:
Hirschsprung’s disease is most often diagnosed in newborn babies — the most common sign is not passing stool within the first 24 to 48 hours of life. Other signs in newborns can include swelling of the abdomen, vomiting, and fever. In some cases, the problem doesn’t cause signs or symptoms until the child is a little older. When this happens, signs of a problem include constipation (hard, dry stool that can be hard to pass), poor appetite, and not growing as much as normal.
First, the doctor takes a health history and does a physical exam. These help find the cause of the problem. The following tests may then be done:
Hirschsprung’s disease is treated with surgery. The section of colon without nerves is removed. The healthy ends of the colon are then reconnected. This surgery is called resection.
If a large portion of the colon must be removed, the child may have long-term digestive problems. But in general, most children with Hirschsprung’s disease do very well after surgery. Even so, your child’s bowel movements may not be normal. Due to Hirschsprung’s disease, the rectum and anus may be collapsed. If so, stool can’t be pushed out normally. This can result in minor problems such as constipation and leakage. These problems are often treated with medications and a high-fiber diet. Also, children with Hirschsprung’s are more likely to develop an infection of the colon (enterocolitis). Your child’s health care provider can tell you more about your child’s situation.