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Hirschsprung's disease occurs when some of your baby’s intestinal nerve cells don’t develop properly, causing them to interfere with the movement of food and stools in the intestines. The intestine becomes blocked with stool, and your baby or child will be constipated (unable to have normal bowel movements). Often, a serious infection (enterocolitis) can occur, which causes a child to have a fever, pain and diarrhea.
Hirschsprung's disease causes 15 to 20 percent of intestinal obstructions that occur in newborns. The obstructions require surgery. Boston Children’s GI Motility Center and Colorectal Program specialize in the treatment and follow-up care of children with Hirschsprung's that have had surgery previously and continue to have problems.
Symptoms vary with age. Of children with Hirschsprung's disease, 80 percent show symptoms in the first six weeks of life. However, children who only have a short segment of intestine that lacks normal nerve cells may not show symptoms for several months or years. Their primary symptom is constipation.
Each child may experience symptoms differently, but common symptoms in infants include:
Children who don’t have early symptoms may also have the following symptoms:
Scientists are not sure why the intestinal nerve cells do not form completely. However, genetic factors likely play a role in Hirschsprung's disease, especially when longer lengths of intestine are involved.
Hirschsprung's disease occurs five times more frequently in boys than in girls. Children with Down syndrome have a higher risk.
Your child may receive one or more of the following tests in order to properly diagnose their condition and confirm whether or not they have Hirschsprung’s:
There are other tests that, when abnormal, suggest that Hirschsprung’s may be present. Your child may need to have more testing or a biopsy to confirm or to rule out this diagnosis.
Read more about GI motility testing at Boston Children’s.
Due to advances in minimally invasive surgery, our surgeons frequently perform a single operation to fix intestinal obstruction at the time that Hirschsprung's disease is initially diagnosed. The goal of the surgery is to remove the diseased section of the intestine and to pull the healthy portion of the intestine down to the anus.
In most cases, this surgery can be done with minimally invasive surgical techniques and sometimes entirely through the anus with no scars at all.
Learn more our GI motility treatments and approach at Boston Children’s.
It is not uncommon for children that have had Hirschsprung’s disease surgery to continue to have problems after surgery. At Boston Children’s, our specialists are dedicated to evaluating and caring for children who continue to have issues related to this disease.
Problems that may occur after surgery depend on how much unhealthy intestine needed to be removed during surgery and the current functioning of the colon, rectum and anus.
Problems that may occur include:
Children who have a large section of intestine removed may also have long-term digestive problems. Removing a large segment of the intestine can prevent a child from getting adequate nutrients and fluids, leading to problems with improper digestion, slow growth and infection.
Our team will provide a thorough evaluation to locate the problem using one or more advanced diagnostic tests, such as anorectal manometry, a barium enema or a biopsy of the rectum or colon. These tests will provide doctors with a clear picture of how the colon is working after surgery and whether the repair was successful.
Our doctors will then collaborate on a treatment plan that may include:
Read more about our GI motility treatments to understand our comprehensive approach for long-term management of these conditions.
For an appointment, more information or to obtain a second opinion for your child, please contact the Motility and Functional Gastrointestinal Disorders Center at 617-355-6055 or request an appointment online.
We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”