Hirschsprung’s Disease

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What is Hirschsprung’s disease?

Hirschsprung's disease occurs when some of your baby’s intestinal nerve cells (ganglion cells) don’t develop properly, delaying the progression of stool through 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 called enterocolitis can occur, which causes fever, pain and diarrhea.

How we care for Hirschsprung’s disease

Hirschsprung's disease causes 15 to 20 percent of intestinal obstructions that occur in newborns. These obstructions require surgery. The Colorectal and Pelvic Malformation Center and Motility and Functional Gastrointestinal Disorders Center at Boston Children’s Hospital specialize in the primary treatment and follow-up care of children with Hirschsprung's disease.

Specialists from the Department of Surgery treat children who need their first pull-through procedure. After surgery, your child will be followed by a gastroenterologist to help address chronic constipation and other possible consequences of Hirschsprung's disease.

What are the symptoms of Hirschsprung's disease?

Symptoms vary with age. Eighty percent of children with Hirschsprung's disease have 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 exhibit symptoms for several months or years. Their primary symptom is constipation.

Each child may experience symptoms differently, but common symptoms in infants include:

  • failure to have a bowel movement in the first 48 hours of life
  • sbdominal distention (stomach bloating)
  • gradual onset of vomiting
  • fever
  • constipation or failure to pass regular bowel movements

Children who don’t have early symptoms may experience the following signs of Hirschsprung’s disease as they get older:

  • constipation that becomes worse with time
  • loss of appetite
  • delayed growth
  • passing small, watery stools
  • abdominal distention

What causes Hirschsprung's disease?

Scientists aren’t sure why the ganglion cells don’t migrate down to the end of the rectum completely. However, genetic factors may be involved, especially when longer lengths of intestine are involved or when someone else in the family also has the condition.

For instance, there is an increased chance that a couple will have a child with Hirschsprung's disease if one of the parents has the disease. (The chances are higher if the mother is the one with Hirschsprung's disease). If a family has a child with Hirschsprung's disease, there is a 3 to 12 percent chance that another baby from the same parents will also have the disease.

Hirschsprung's disease occurs five times more frequently in boys than in girls. Children with Down syndrome have a higher risk as well.

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- Sandra L. Fenwick, President and CEO

Boston Children's Hospital
300 Longwood Avenue, Boston, MA 02115
For Patients: 617-355-6000
For Referring Providers: 844-BCH-PEDS | 844-224-7337

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