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Hirschsprung Disease


The Manual's Editorial Staff

Last full review/revision Apr 2020| Content last modified Apr 2020
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What is Hirschsprung disease?

The large intestine is lined with muscles that contract to move stool through the intestine. Hirschsprung disease is a birth defect in which part of the colon (large intestine) is missing the nerves that signal the muscles to contract.

  • Stool builds up in the intestine and causes a blockage

  • A child with Hirschsprung disease may throw up, refuse to eat, and have a swollen belly

  • Doctors do surgery to remove the part of the intestine that is missing nerves

  • If not treated, Hirschsprung disease can lead to a fatal infection of the intestines called enterocolitis

What are the symptoms of Hirschsprung disease?

Symptoms include:

  • In newborn babies, not having a bowel movement within the first 24 hours after birth

  • Vomit that is green or brown

  • A swollen belly

  • Refusing to eat

If only a small part of a child’s large intestine is blocked, the symptoms can be mild and include:

  • Thin stool, like a ribbon

  • A swollen belly

  • Not gaining weight

  • Not passing stool

If Hirschsprung disease isn’t treated, a child may get Hirschsprung enterocolitis. This can be life-threatening with symptoms that include:

  • Sudden fever

  • Swollen belly

  • Explosive diarrhea

How can doctors tell if my baby has Hirschsprung disease?

Doctors may suspect Hirschsprung disease is if a baby doesn't poop in the first 24 hours after birth.

Doctors test for Hirschsprung disease with the following:

  • Barium enema x-ray

  • Rectal biopsy (doctors take out a small piece of the child’s rectum to look at under a microscope)

  • Measuring the pressure inside the child’s rectum

How do doctors treat Hirschsprung disease?

Doctors treat Hirschsprung disease by:

  • Doing surgery to take out the abnormal section of intestine and reconnect the 2 ends of working intestine

Sometimes, if the child is very sick, doctors first do a temporary colostomy. They make a hole in the large intestine and connect it to a hole in the belly. Your baby's poop goes out the hole into a bag until your baby is healthy enough to have another operation. Then doctors do a second surgery to remove the part of the intestine that isn’t working, reattach the working intestine, and seal the opening for the colostomy.

Doctors treat infection (enterocolitis) with:

  • Fluids and antibiotics in the vein

  • Washing stool out of the intestines with salt water given through a tube in the rectum

  • Surgery

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