Hirschsprung Disease

Written by Amanda Delgado | Published on August 15, 2012
Medically Reviewed by George Krucik, MD

What is Hirschsprung Disease?

Hirschsprung disease is a blockage that occurs in the colon. It interferes with your child’s ability to pass stool. According to the Cincinnati Children’s Hospital Medical Center, this condition is present in about one in every 5,000 live births (CCHMC). Hirschsprung disease is usually treated successfully through surgery.

How Your Child May Have Gotten Hirschsprung

The exact cause of Hirschsprung disease is unknown. It develops when the nerve cells around the colon don’t fully form. This can affect a long portion of the colon or a very small part of it. Either way, when this happens, digested food and stool can’t be pushed through these areas. This causes a blockage of stool in your child’s intestine. Your child won’t be able to have regular bowel movements due to this obstruction. It can also cause the intestinal wall in this area to become thin, which can lead to a serious bacterial infection.

Is My Child at Risk for Hirschprung?

According to the National Institutes of Health, Hirschsprung disease is five times more common in boys than girls (NIH). It’s also more likely to occur in children with Down syndrome and other inherited conditions. Lucile Packard Children’s Hospital at Stanford states that if you already have one child with Hirschsprung Disease, any other children you have will have a three to 12 percent chance of inheriting it (Stanford). Your child also has an increased risk of having this condition if one parent has it, especially when the mother is a carrier.

What Are the Symptoms of Hirschprung?

Infants

According to the Cincinnati Children’s Hospital Medical Center, symptoms appear during the first six weeks after birth in 80 percent of children who have this condition (CCHMC). Symptoms in newborns typically start within 48 hours after birth. These include:

  • not having a bowel movement within 48 hours after birth
  • vomit that is green or brown
  • abdominal swelling
  • being very gassy
  • bloody diarrhea
  • infrequent and explosive stools

Toddlers and Older Children

If a shorter section of the intestine is affected, symptoms generally won’t appear until several months or even years later. Common symptoms include:

  • needing to use laxatives or enemas to have bowel movements (laxatives loosen stool and enemas use water or mild soap to stimulate bowel movements)
  • abdominal swelling
  • slow growth
  • anemia: a condition that causes low energy levels due to a shortage of red blood cells
  • being very gassy

What Tests Does My Child Need?

Your child’s doctor will use one or more of the following tests to check for Hirschsprung disease:

  • abdominal X-ray to look for a wider area of colon or a section with less air in it, which results from a buildup of stool
  • manometry test, which involves inflating a balloon in the rectum to see if the muscles respond normally by relaxing; this is usually done in older children
  • X-ray with a contrast dye to show a clear silhouette of the rectum and colon
  • biopsy, which involves taking a tissue sample for lab testing

How Is Hirschsprung Treated?

Surgery

Hirschsprung disease is treated with surgery. The surgery removes the portion of the colon containing the defective nerve cells. Then, the rectum and healthy portion of the colon are rejoined. This makes it possible for stool to pass through and leave your child’s body in a normal manner. In milder cases, this can be done as one operation. However, usually a colostomy is needed as well and is considered a separate, second surgical procedure.

Colostomy

If your child’s condition is serious, a colostomy will be used to give the lower part of the colon time to heal after the abnormal section is removed. This involves making a tiny hole called a stoma in the abdomen and attaching the upper part of the colon to it. Your child’s stool will leave through the stoma and enter a collection bag, which has to be changed several times each day.

Depending on the length of the removed intestine, the stoma may be permanent. However, if the intestine heals properly, as is usually the case in children, the stoma can be surgically closed and the colon surgically joined to the rectum.

Risks of Surgery

Your child could develop a serious condition called enterocolitis after surgery. When this happens, the colon becomes inflamed. Watch for signs of this condition, and call your child’s doctor right away if any occur. These symptoms include:

  • rectal bleeding
  • fever
  • diarrhea
  • abdominal swelling
  • vomiting

Post-Surgery Care

If your child is constipated after surgery, you can help ease this condition. Make sure your child drinks plenty of water to avoid dehydration. Offer your child foods that are high in fiber, such as whole grains and vegetables. You should add these to your child’s diet slowly since eating a lot of fiber suddenly could make constipation worse. Your child can also take laxatives with a doctor’s permission.

Outlook: Will My Child Be OK?

Your child’s symptoms should be gone after surgery, especially if it’s done early or if the abnormal colon segment is shorter. Your child might have constipation, loose stools, or trouble recognizing the urge to pass stools after surgery.

If the removed colon segment was longer, your child might have digestive problems or difficulty getting enough nutrients. These can be long-term effects and can lead to slow growth or infections. Your child’s doctor can help determine dietary and prescription treatments to help minimize these potential long-term consequences.

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