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Merck Manual
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Home /
Quick Facts: Children's Health Issues /
Birth Defects of the Digestive Tract /
... /
Hirschsprung Disease /
IN THIS TOPIC

What is Hirschsprung disease?

What are the symptoms of Hirschsprung disease?

How can doctors tell if my baby has Hirschsprung disease?

How do doctors treat Hirschsprung disease?

OTHER TOPICS IN THIS CHAPTER

Birth Defects of the Digestive Tract
Abdominal Wall Defects
Anorectal Malformations
Biliary Atresia
Diaphragmatic Hernia
Esophageal Atresia and Tracheoesophageal Fistula
Hirschsprung Disease
Intestinal Malrotation

Test your knowledge

Vitamin Deficiency Anemia
Deficiencies of vitamin B12 and folate (folic acid) cause a condition called megaloblastic anemia. In patients with megaloblastic anemia the bone marrow produces red blood cells that are large and abnormal (megaloblasts). Which of the following is NOT a cause of Vitamin B12 or folate deficiency? 

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Quick Facts

Hirschsprung Disease

By

The Manual's Editorial Staff

Last full review/revision Apr 2020| Content last modified Apr 2020
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NOTE: This is the Consumer Version. DOCTORS: Click here for the Professional Version
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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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© 2020 Merck Sharp & Dohme Corp., a subsidiary of Merck & Co., Inc., Kenilworth, NJ, USA)
Esophageal Atresia and Tracheoesophageal Fistula
Intestinal Malrotation
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