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Hirschsprung Disease

Alternate Names

  • congenital aganglionic megacolon
  • Digestive system

Definition

Hirschsprung disease is a blockage in the large intestine. It is caused by an lack of nerve cells in a portion of the intestine. A person is born with this condition.

What is going on in the body?

A person with this disease is missing the nerve cells known as ganglia cells in part or all of the large bowel. These cells are absent at birth. The normal function of these cells is to enable the bowel muscle to relax and contract. This motion pushes the stool through the intestine. Stool cannot pass through the segment of the bowel where the nerves are missing. As a result, stool collects at that point causing an intestinal blockage.

Risks

What are the causes and risks of the disease?

This disease is present at birth. It occurs because some nerve cells fail to develop in portions of the large bowel while the fetus is growing in the uterus. The disease may be inherited, but most of the time the cause is unknown. It is more common in boys than girls. This disease may also be associated with other conditions that are present at birth such as Down syndrome.

Prevention

What can be done to prevent the disease?

This disease cannot be prevented. If a person has this disease, genetic counseling may be useful to explain to the person and his or her family how the disease may be passed on to children.

Diagnosed

How is the disease diagnosed?

The healthcare professional will first do a physical exam. Abdominal swelling may be present. There may not be any fecal material in the anal canal or rectum, the areas in which stool is stored before it leaves the body. Also, there may be a gush of pale, liquid stool and gas as the healthcare professional withdraws a finger after performing the rectal exam.

Long Term Effects

What are the long-term effects of the disease?

Severely affected infants will die without treatment. Other long-term effects include a possible perforation of the intestine, and infection or inflammation of the intestines and colon. These, too, may lead to death. Treatment helps to prevent these complications.

Other Risks

What are the risks to others?

This disease is not contagious. It can be inherited in some cases. Genetic counseling may be useful for family members of children with this disease.

Treatments

What are the treatments for the disease?

Most people with this disease will need surgery. Usually, the affected part of the bowel is removed. Surgery is often a two-part process.

Side Effects

What are the side effects of the treatments?

Surgery can be complicated by bleeding, infection, or an allergic reaction to the anesthetic or to pain medications. If antibiotics are used, they may cause stomach upset or allergic reactions. Some people experience involuntary loss of stool after the surgery.

After Treatment

What happens after treatment for the disease?

After surgery, symptoms usually go away. Most children are able to have normal bowel movements after surgery. In general, the earlier a person receives treatment, the better the outcome.

Monitor

How is the disease monitored?

The child's caregiver should contact a healthcare professional right away if new or worsening symptoms occur after surgery.

Sources

[hyperLink url="http://www.niddk.nih.gov/health/digest/summary/hirsch/hirsch.htm" linkTitle="www.niddk.nih.gov/health/digest/summary/hirsch/hirsch.htm"]www.niddk.nih.gov/health/digest/summary/hirsch/hirsch.htm[/hyperLink]

CURRENT Pediatric Diagnosis&Treatment, Hathaway, Hay, Groothus, Paisley, 1993.

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