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Hirschsprung's Disease

Definition


Disease: Hirschsprung's Disease Hirschsprung's Disease
Category: Surgical diseases
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Disease Definition:

Hirschsprung's disease is a congenital one, which occurs as the result of missing nerve cells in the muscles of a portion of the baby's colon. This disease affects the large intestine ــ also called the colon ــ and causes problems with passing stool.

Children with this disease may have problems absorbing nutrients from food, or they could be constipated. Mild cases of Hirschsprung's disease may not be detected until later in a child's life. However, in severe cases, a newborn will probably experience an obstructed colon and won't be able to have a bowel movement.

The only treatment for Hirschsprung's disease is the surgical removal of the diseased portion of the child's colon. Usually, nine out of ten children pass stool normally after this surgery.

Work Group:


Prepared by: Scientific Section

Symptoms, Causes

Symptoms:

Depending on the severity of the condition, the signs and symptoms of Hirschsprung's disease may vary. In some cases, the signs and symptoms may not be apparent until the baby becomes a teenager or adult, while in other cases, they may appear right after the baby is born. The signs and symptoms in newborns may be:

 

  • Constipation or gas, which could make a newborn fussy
  • Failure to pass stool within the first or second day of life
  • Diarrhea
  • Vomiting, including vomiting bile, which is a green digestive fluid that is produced in the liver.


Some of the signs and symptoms in older children may be:

 

  • Lack of weight gain.
  • Weight loss, diarrhea or both, as well as delayed or slowed growth due to problems absorbing nutrients.
  • Swollen abdomen.


In newborns or very young children, infections in the colon may include enterocolitis, which is a serious infection with vomiting, fever and diarrhea and in some cases, a dangerous dilation of the colon.

Signs and symptoms may include constipation in older children and adults, in addition to anemia because blood is lost in the stool. Anemia causes the affected person to look pale and to tire easily. If someone has anemia, it means that they have a low number of red blood cells.

Causes:

In a normal baby, bundles of nerve cells called ganglia will begin to form between the muscle layers along the length of the colon, as the baby grows in the womb. This process will begin at the top of the colon and end at the bottom (rectum). This process won't finish in children with Hirschsprung's disease, and the ganglia won't form along the entire length of the colon. In some cases, cells may be missing from only a few centimeters of the colon, while in other cases, a longer portion of the colon could be affected.

The exact cause of this is still not known. It could be associated with multiple endocrine neoplasia, type IIB, which is a syndrome that causes cancer of the thyroid gland (located at the base of the neck) and noncancerous tumors in the mucous membranes and adrenal glands (located above the kidneys). This disease could also be associated with mutations in several genes.

Hirschsprung's disease is ten times more likely to occur in children with Down syndrome. However, this disease isn't caused by anything the mother does during pregnancy. This disease could sometimes be inherited, even if neither of the parents has it.

Complications

Complications:

None

Treatments:

The only proven and effective treatment for Hirschsprung's disease is surgery, which is called pull-through surgery. In this surgery, the section of the colon that has no ganglia cells will be removed and then the remaining healthy end of the colon will be connected to the rectum.

In some cases, pull-through surgery is done in one step immediately after diagnosis, while in other cases the process could be done in two steps. First, the abnormal portion of the colon without ganglia cells will be removed and an ostomy will be performed. To do this, a small hole called a stoma is created in the child's abdomen, and the top, healthy portion of the colon is connected to the stoma. After this, stool will leave the body through the stoma into a bag that is attached to it. This will allow the lower part of the colon to heal. A stoma bag must be emptied several times a day. Ostomy could include:

Ileostomy: In ileostomy, the entire colon is removed and the small intestine is connected to the stoma.
Colostomy: In colostomy, part of the large intestine is left and attached to the stoma.

After this surgery and after the child has had time to recover, the stoma will be closed up and a second surgery will be performed in order to connect the healthy portion of the colon to the rectum.

COMPLICATIONS:
As mentioned before, most children pass stool normally after the surgery. Although initially some children may experience diarrhea, however, stool will become more solid after some time. Because some children may have difficulty coordinating the muscles used to pass stool, toilet training may take a longer time. In most children, this coordination will improve with time. Laxatives could help in case some children continue to have constipation. Diarrhea and constipation could also be avoided by eating high-fiber foods.

After surgery, a child could be at risk of developing enterocolitis in his/her colon or small intestine. Parents should look out for signs and symptoms of enterocolitis which may include:

 

  • Diarrhea
  • Fever
  • Bleeding from the rectum
  • Swollen abdomen
  • Vomiting

 

In case these signs and symptoms appear, parents should immediately call a doctor.

Prognosis:

Not Available

Expert's opinion

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