Hirschsprung's disease in children

Hirschsprung's disease in children

Hirschsprung's disease in children

This is a vital cycle: we eat food, digest it, and excrete what we cannot use.

Moulting, although often disgusting or humorous, is very important. It usually starts on the first day of a baby's life when the newborn passes its first stool, called meconium.

But some kids have problems. If a child cannot have a bowel movement, they may have Hirschsprung's disease, a condition in which there are no nerve cells in the colon.

Hirschsprung disease is congenital, meaning it develops during pregnancy and is present at birth. Some congenital conditions are due to the mother's diet or due to an illness she had during pregnancy. Others are related to genes that parents pass on to the newborn.

Researchers don't know why some people get Hirschsprung's disease, although they believe the disease is due to defects in DNA instructions. Although the disease can be fatal, modern medicine is able to solve the problem surgically, and treated children can live normal, healthy lives.

What causes Hirschsprung's disease?

He begins to settle in the baby in the first months of pregnancy.

Normally, as a child develops, nerve cells form throughout the digestive system, from the esophagus to the rectum. Normally, a person can have up to 500 million of these types of nerve cells. Among other things, they move food through your digestive system from one end to the other.

In a child with Hirschsprung's disease, nerve cells stop growing at the end of the colon, just in front of the rectum and anus. Some children also lack cells elsewhere in the digestive system.

This means that the body cannot sense when the waste reaches a certain point. Thus, the waste gets stuck and forms a block in the system.

Hirschsprung's disease affects about one in 5,000 newborns. Babies with other congenital anomalies, such as Down's syndrome and heart defects, are more likely to get the disease. Parents who carry the Hirschsprung disease code in their genes, especially mothers, can pass it on to their children. Boys have more than girls.

The disease is named after the 19th century Danish physician Harald Hirschsprung, who described the disease in 1888.

Symptoms

The vast majority of people with Hirschsprung disease usually develop symptoms within the first 6 weeks of life. In many cases, symptoms appear within the first 48 hours.

You may see swelling in your child's abdomen. Other symptoms you may see include:

  • No bowel movements: You should be concerned if the newborn does not produce stool or meconium in the first two days. Older children with Hirschsprung may suffer from chronic constipation.
  • Bloody diarrhea: Children with Hirschsprung's disease can also develop enterocolitis, a life-threatening infection of the colon, as well as severe cases of diarrhea and gas.
  • Vomiting: The vomit may be green or brown.
  • In older children, symptoms may include growth problems, fatigue, and severe constipation.

Diagnostics and tests

Your doctor should tell you right away if your child has these symptoms. They can confirm Hirschsprung with specific tests:

  • Contrast Enema: This is also called a barium enema, as the tincture of the element is often used to coat the internal organs of the body and enhance them. The child is placed face down on a table, and the dye is injected through a tube from the anus into the intestines. There is no need for anesthesia. The dye allows the doctor to see problem areas on x-rays.
  • Abdominal x-ray: This is a standard x-ray that the technician can take from different angles. Your doctor will be able to see if anything is blocking the bowel.
  • Biopsy: Your doctor will take a small sample of tissue from your child's rectum. The tissue is examined for Hirschsprung signs. Depending on the age and size of your child, the doctor may use anesthesia.
  • Anorectal manometry: This test inflates a small balloon inside the rectum to see if the muscles in the area are responding. This test is only done on older children.

Treatment

Hirschsprung's disease is a very serious disease. But if it is detected at an early stage, it can almost always be cured with surgery.

Doctors usually perform one of two types of surgery:

  • Pull-through procedure: This operation simply cuts out the part of the colon with missing nerve cells. The rest of the intestine then connects directly to the anus.
  • Ostomy surgery: This operation brings the intestine to an opening made in the body. The doctor then attaches an ostomy bag to the outside of the opening to keep waste from the bowel. An ostomy is usually a temporary measure until the child is ready for the extraction procedure.

After surgery, some children may have problems with constipation, diarrhea, or urinary incontinence (lack of control over bowel movements or urination).

A handful may have enterocolitis. Signs include rectal bleeding, fever, vomiting, and bloating. If this happens, take the child to the hospital immediately.

But with proper care, these conditions usually go away on their own. Within a year of treatment, 95% of children should be cured of Hirschsprung's disease. 


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