Treatment
Surgery is thought to be the most effective treatment for Hirschsprung disease. This can be done in one step or two, depending on the condition's severity. Children who are very sick at the time of surgery (due to an inflamed colon or poor nutrition) may need to undergo surgery in two steps.
The most common surgery to correct Hirschsprung disease involves removing the section of the colon without nerves and reattaching the remaining portion of the colon to the rectum. Often, this can be done through minimally invasive (laparoscopic) surgery immediately after the condition is diagnosed.
In some cases, the doctor may do the surgery in two steps. In the first step, the doctor will remove the unhealthy portion of the colon and then perform a procedure called an ostomy. In an ostomy, the doctor creates a small hole, or stoma, in the child's abdomen and attaches the upper, healthy portion of the colon to the hole.
The two types of ostomy are:
- Ileostomy: removing the entire large intestine and connecting the small intestine to the stoma
- Colostomy: removing just a portion of the colon
The child's stool passes through the stoma into a bag that is connected to it and needs to be emptied several times a day. This allows the lower part of the colon to heal prior to the second surgery. In the second surgery, the doctor closes up the hole and attaches the normal portion of the colon to the rectum.
After surgery, kids often get constipated. Laxatives can offer some relief, but check with your doctor about which would be best for your child if you decide to use them. For children old enough to eat solid foods, a high-fiber diet can ease and prevent constipation. Drinking plenty of water is also important, and helps prevent dehydration. The large intestine helps absorb water from food, so dehydration can be a concern for children who have had part of their intestine removed.
Kids who continue to have symptoms or develop new ones after surgery (such as explosive and watery diarrhea, fever, a swollen belly, or bleeding from the rectum) should have medical attention right away. These can be signs of enterocolitis, an inflammation of the intestines.
Outlook
The general outlook for children who have been treated surgically for Hirschsprung disease is excellent. After surgery, most can pass stool normally and have no lasting complications. However, a few kids might continue to have symptoms, including constipation and bowel control problems.
Reviewed by: J. Fernando del Rosario, MD
Date reviewed: January 2015