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Hirschsprung's disease

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.

Symptoms Causes Complications Risk factors

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
  • Diarrhea

In older children, signs and symptoms can include:

  • Swollen belly
  • Chronic constipation
  • Gas
  • Failure to gain weight
  • Fatigue

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.

Factors that may increase the risk of Hirschsprung's disease include:

  • Having a sibling who has Hirschsprung's disease. Hirschsprung's disease can be inherited. If you have one child who has the condition, future biological children also might be at risk.
  • Being male. Hirschsprung's disease is more common in males.
  • Having other inherited conditions. Hirschsprung's disease is associated with certain inherited conditions, such as inherited heart problems and Down syndrome. It may also be associated with multiple endocrine neoplasia, type IIB — a syndrome that causes noncancerous tumors in the mucous membranes and adrenal glands (located above the kidneys) and cancer of the thyroid gland (located at the base of the neck). Roughly a third of children who have Hirschsprung's disease have other abnormalities.
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