Hirschsprung's Disease
Children with Hirschsprung’s disease are born with ganglion cells missing from part of their intestine. Ganglion cells are a type of nerve cell that control the movement of stool down the intestine. With Hirschsprung’s disease, the stool moves slowly or stops at the part with missing nerve cells, and the child isn’t able to have a normal bowel movement.
Most children with this condition have missing nerve cells in the rectum and the sigmoid colon — the last part of the bowel. In some children, the entire colon (large intestine) is missing these cells. In rare cases, the last part of the small intestine is also missing nerve cells.
Hirschsprung’s disease affects about one in 5,000 newborns, though symptoms may not occur until they are older. It is more common in boys and in children who have a close relative with the condition.
Symptoms
In newborns, the first symptoms may be a delay in their first bowel movement after they’re born. Babies also may have:
Green or brown vomit
Diarrhea
Swollen abdomen
Fever
Slowed growth
Toddlers and older children with this condition usually can’t have a bowel movement without first having an enema or glycerin suppository. They also may have:
Swollen abdomen
Diarrhea
Slowed growth
Diagnosis
Children who have symptoms of Hirschsprung’s disease should see a pediatric gastroenterologist. At Orlando Health Children's Arnold Palmer Hospital Center for Digestive Health and Nutrition, your child’s doctor will ask about their symptoms and when they began. The doctor also will do a physical exam and ask whether any close relatives have been diagnosed with Hirschsprung’s disease.
If your child’s symptoms and family health history indicate the possibility of Hirschsprung’s disease, your specialist will recommend one or more of the following tests:
Contrast enema. With this test, the doctor uses a flexible tube to fill your child’s colon with a chalky liquid called barium that shows up on X-rays. This allows the doctor to see any narrowed areas, obstructions or other changes in your child’s colon. Your child won’t need anesthesia for this test, which also is called a lower GI series.
Anorectal manometry. This test evaluates the reflexes in your child’s anus and rectum. The doctor will insert a narrow tube with a balloon at the end into your child’s rectum. When the balloon is inflated, muscles in the anus and rectum normally relax as they would during a bowel movement. If the muscles don’t relax, it could indicate Hirschsprung’s disease.
Rectal biopsy. This procedure is used to get a small piece of tissue from your child’s rectum to examine in the lab. The doctor will insert a small instrument into your child’s rectum to obtain the tissue sample. This test is not painful, though older children may need medicine to relax during the test.
Treatments
Children with Hirschsprung’s disease need surgery to remove the part of the colon that is missing nerve cells. This can be done one of two ways:
Pull-through surgery. With this procedure, the surgeon removes the affected part of the colon and connects the remaining part to the rectum. This can often be done with minimally invasive techniques right after Hirschsprung’s disease is diagnosed.
Ostomy surgery. This is a two-step procedure that allows the lower part of the colon to heal before the second step. The surgeon first removes the affected part of the colon and diverts the healthy part to the outside of the abdomen. The stool is routed into a bag that is emptied several times a day. For the second surgery, the surgeon connects the healthy part of the colon to the rectum and closes the hole where the diversion was located.
Your child’s stools may be loose after surgery, and later on, constipation can be a problem. Your team at Orlando Health Children's Arnold Palmer Hospital Center for Digestive Health and Nutrition may recommend a laxative and a high-fiber diet to help. Surgery for Hirschsprung’s disease usually leads to excellent outcomes, and children have no lasting complications. If their growth was slowed due to their condition, it typically improves afterward.
While most children do well after surgery, Hirschsprung’s disease makes them more likely to develop a serious infection called enterocolitis. It can occur both before and after surgery with symptoms such as severe diarrhea, fever, bleeding from the rectum, vomiting and a swollen abdomen. Enterocolitis is more common among younger children, but if your child ever has any of these symptoms, you should contact your doctor right away.
Next Steps
Your team at Orlando Health Children's Arnold Palmer Hospital Center for Digestive Health and Nutrition will explain each step of your child’s surgery for Hirschsprung’s disease and answer all your questions. Our registered pediatric dietitians will advise you on what foods are best as your child recovers from surgery. And we’re here for you as your child grows up, to treat any problems that may arise and to ensure their continued health.
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