Hirschsprung Disease (Colonic Aganglionosis)

Hirschsprung's disease is a condition that causes a baby to be born with nerve cells in his or her intestines missing. Sometimes the missing nerve cells affect only a small part of the bowel. In other cases, large sections of the nerve cells are missing. Hirschsprung's disease causes 15 to 20 percent of intestinal obstructions that occur in newborns.

Most babies with Hirschsprung's are diagnosed within the first six weeks after birth. If the disease affects only a small portion of the bowel, it may take long to discover the problem.

Options for Treating Hirschsprung's Disease

Specific treatment for Hirschsprung's disease will be determined by your child's doctor based on:

  • How much of the intestine's are affected by the disease
  • The child's age, overall health and medical history
  • How well the chance can handle certain drugs, procedures or therapies
  • The expected outcomes of the disease

Once the condition has been diagnosed, treatment focuses on removing or bypassing the section of the intestine that lacks nerves and replacing it with normal bowel tissue.

Both minimally invasive and open, traditional approaches are used to surgically treat Hirschsprung's disease. Infants that are stable may be candidates for minimally invasive surgery. When the baby is very ill or when the surgery is likely to be unusually complicated, a more traditional approach may be used. Sometimes both approaches are used at different stages of treatment to do different things.

Minimally Invasive Approach to Treating Hirschsprung's Disease

This approach uses only four to five small incisions. The procedure is done with an endoscope. An endoscope is a tube with a camera on it that allows the surgeon to see what he is doing inside the body. The surgeon to view the procedure on a television monitor.

Sometimes this approach is used only to remove the blockage and tissue that is missing nerves. The healthy sections of bowel are then reconnected. This is called a pull-through procedure.

In other cases, the surgeon will connect the open end of the bowel to the surface of the skin. This is called a colostomy. It makes it possible for wastes to be eliminated through the hole to the outside of the body. This protects the area where the bowel was removed until it has healed. The colostomy may be closed in four to six weeks.

The Open or Traditional Approach

This is done when minimally invasive surgery isn't possible. This procedure uses a large incision to bring a piece of the bowel to the surface of the skin to remove the block. When the baby has recovered, the minimally invasive approach can be used to eliminate the piece of bowel without nerves.

Possible Complications After Hirschsprung's Disease Surgery

Most people who undergo surgery for Hirschsprung's disease recover without problems. Any time a person has a general anesthesia and surgery, however, there is a possibility of complications. These include bleeding, infection or a blockage of the bowel after surgery due to scarring or other factors.

With minimally invasive surgery, there are generally fewer complications. Sometimes the blood supply to the intestines can be disturbed in a pull through operation. If this happens, more surgery may be needed.

It's very important for a person who has had the pull-through surgery to avoid constipation. Constipation can lead to enterocolitis or infections of the intestine that can be life threatening. Whenever a young child who has been treated for Hirschsprung's disease changes his or her diet (such as going to vacation or starting school), it is important to be alert to constipation issues.

A baby or child who becomes constipated, has small watery stools, a swollen belly, lacks energy or runs a fever should be taken to a doctor right away.

A small number of people (about 5 percent) may have severe constipation or incontinence. These may be treated by medical means such as stool softeners, laxatives, enemas or procedures to stretch the muscles of the anus or rectum.

Patients with Down Syndrome and neurologic impairment will have a higher incidence of constipation and incontinence. They can only be treated with medical management and a great deal of patience.

Life After Surgery to Treat Hirschsprungs Disease

The long range outlook for a child who has been treated for Hirschsprung's disease will vary depending on the amount of intestine that lacked nerve cells and how much of the intestine was removed.

Children who are able to have their colostomy closed may have problems that come and go such as:

  • Frequent, loose stools, especially at first. It is important to keep the anal area clean and to apply diaper rash creams to prevent skin irritation.
  • Problems knowing when they need to have a bowel movement. Spending time on the toilet about 10 minutes after eating may help.
  • Difficulties having a bowel movement because the anal opening is tight. This can be helped with a technique to stretch the muscles to prevent tightness. Doing this is called dilation.

If a large section of intestine had to be removed, a child may have longer term problems. Shortening the intestine may make it harder for the child's body to absorb enough nutrition and fluids. This in turn may cause poor digestion or slow growth.

It's best to discuss these issues with your doctor as every child is different.