Hirschsprung’s (HIRSH-sproongz) disease is a condition that affects the large intestine (colon) and causes problems with passing stool. Hirschsprung’s disease is present when a baby is born (congenital) and results from missing nerve cells in the muscles of part or all of the baby’s colon.
A newborn who has Hirschsprung’s disease is usually unable to have a bowel movement in the first days after birth. In mild cases, the condition might not be detected until later in childhood.
Hirschsprung’s disease is treated with surgery to bypass or remove the diseased part of the colon.
Signs and symptoms of Hirschsprung’s disease vary with the severity of the condition. Usually signs and symptoms appear shortly after birth, but sometimes they’re not apparent until later in life.
Typically, the most obvious sign of Hirschsprung’s disease is a newborn’s failure to have a bowel movement within 48 hours after birth.
Other signs and symptoms in newborns may include:
- Swollen belly
- Vomiting, including vomiting a green or brown substance
- Constipation or gas, which might make a newborn fussy
In older children, signs and symptoms can include:
- Swollen belly
- Chronic constipation
- Failure to gain weight
It’s not clear what causes Hirschsprung’s disease. It sometimes occurs in families and may in some cases be associated with a genetic mutation.
Hirschsprung’s disease occurs when nerve cells in the colon don’t form completely. Nerve cells are critical to the functioning of the colon. They control the regular muscle contractions that keep food moving through the bowels.
As a baby develops before birth, bundles of nerve cells (ganglia) normally begin to form between the muscle layers along the length of the colon. This process begins at the top of the colon and ends at the bottom (rectum). In children who have Hirschsprung’s disease, the nerve-growing process fails to finish. Most commonly, ganglia fail to form (aganglia) in the last segment of the colon — the rectum and the sigmoid colon. Sometimes aganglia affects the entire colon and even part of the small intestine.
Children who have Hirschsprung’s disease are prone to a serious infection of the intestines called enterocolitis.
Enterocolitis is caused by stool backing up behind the immobile section of colon. The stagnant mass of stool provides a fertile environment for bacteria to grow. As the stool mass expands, it presses on the blood vessels in the walls of the colon. Decreased blood flow can lead to a breakdown of the lining of the colon (mucosa), making it susceptible to infection.
Enterocolitis can be a life-threatening complication. It’s treated in the hospital with colon cleaning and antibiotics.
To help manage constipation:
- Serve high-fiber foods. If your child eats solid foods, include high-fiber foods as part of your child’s diet. For instance, offer whole grains, such as whole-wheat bread. Reduce servings of refined grains, such as white bread. Encourage your child to eat fruits and vegetables. Cut down on fatty animal-source foods, such as meat and butter. Be aware, though, that a sudden increase in high-fiber foods can make constipation worse — so add high-fiber foods to your child’s diet slowly. If your child isn’t eating solid foods yet, ask the doctor about formulas that might help relieve constipation.
- Increase fluids. Encourage your child to drink more water. One of the colon’s jobs is to absorb water from food in the last stages of digestion. If a portion of your child’s colon was removed, your child may have trouble absorbing enough water. Drinking more water can help your child stay hydrated, which may help ease constipation.
- Encourage physical activity. Daily aerobic activity helps promote regular bowel movements.
- Ask your child’s doctor about laxatives. Certain laxatives — medications to encourage bowel movements — might help relieve constipation. Ask the doctor about the risks and benefits of laxatives for your child.
Treatment and Diet
While treatment for this disease usually involves surgery, there are a number of home treatments that may complement medical treatment. Diet should typically be adjusted to include colon friendly foods such as fruits and vegetables. One has to cut back on various processed foods, unhealthy artificial drinks, alcohol as well as refined sugars. You will need excess amounts of roughage in the form of healthy foods such as legumes and vegetables, since constipation could be an issue even after treatment. Thus, water and a fiber rich diet should be your priorities. If your child shows any of the symptoms, your doctor should be contacted as there are a number of tests to identify Hirschsprung’s disease. He or she will help out with diet and home treatments in addition to medical treatment alternatives.