Skip to main navigation Skip to main content Skip to footer For Medicare For Providers For Brokers For Employers Español For Individuals & Families: For Individuals & Families Medical Dental Other Supplemental Explore coverage through work How to Buy Health Insurance Types of Dental Insurance Open Enrollment vs. Special Enrollment See all topics Shop for Medicare plans Member Guide Find a Doctor Log in to myCigna
Home Knowledge Center Wellness Library Hirschsprung Disease

Hirschsprung Disease

Condition Basics

What is Hirschsprung disease?

Hirschsprung disease is a birth defect that affects the nerve cells in the large intestine (colon). These nerve cells control the muscles that normally push food and waste through the colon.

In children who have Hirschsprung disease, nerve cells are missing from part or all of the colon. Without nerve cells, the colon can't move stool correctly. This can cause trapped stool, serious inflammation of the colon, and narrowing of the rectum. In most cases, the disease affects the end part of the colon. But it can also affect other parts.

Most of the time, the disease is found soon after birth. It is most common in male babies. And it can be linked to other medical problems that your doctor may want to check for.

In rare cases, it can be life-threatening.

What causes it?

Doctors don't know what causes Hirschsprung disease, but it tends to run in families. It may also be linked to other medical problems, such as Down syndrome and congenital heart disease.

What are the symptoms?

Symptoms of Hirschsprung disease can depend on how severe the problem is and how old the child is.

Most newborns pass stool within the first one or two days of their life. Problems passing stool can be a symptom of Hirschsprung disease.

Children who are diagnosed when they're older may have symptoms. Symptoms may include:

  • A swollen belly.
  • Trouble passing stool.
  • Vomiting.
  • Not wanting to eat.
  • Constipation.
  • Not gaining weight or growing.

How is it diagnosed?

Most children who have Hirschsprung disease are diagnosed during their first year. A doctor may think that a child has the disease based on the child's symptoms and the results of a physical exam.

If the doctor suspects that a child has the disease, he or she will do some tests to find out more. These tests may include X-rays and a test to measure how well the muscles in the anus are working.

Sometimes these tests can provide enough information to make a diagnosis. But the doctor may confirm the diagnosis by taking a tissue sample (biopsy) from the rectum. The cells in the sample are then looked at under a microscope.

How is Hirschsprung disease treated?

Children with Hirschsprung disease need surgery. It's often done within the first days or month of life, soon after the disease is found.

During surgery, the doctor will remove the part of the colon that doesn't work right. Then, if possible, he or she will connect the healthy part of the colon to the anus. This is called a "pull through" procedure. Most of the time, surgery can be done without making a large incision. Doctors can often do the surgery using tools put through small incisions in the child's stomach (laparoscopy). The doctor may also operate through the child's anus.

Rarely, some children may need an ostomy surgery. In this case, the doctor brings the healthy part of the colon through the wall of the belly. Then stool is collected in a bag attached to the outside of the child's body.

Some children have long-term (chronic) problems with stomachaches and bowel problems after surgery. But most of the time, these problems aren't severe.

In a few cases, emergency surgery may be needed if a dangerous problem such as serious swelling of the small and large intestines occurs.

This information does not replace the advice of a doctor. Healthwise, Incorporated, disclaims any warranty or liability for your use of this information. Your use of this information means that you agree to the Terms of Use. Learn how we develop our content.

© 1995-2022 Healthwise, Incorporated. Healthwise, Healthwise for every health decision, and the Healthwise logo are trademarks of Healthwise, Incorporated.

Related Links

Abdominal Pain, Age 11 and Younger Constipation, Age 11 and Younger

<cipublic-spinner variant="large"><span>Loading…</span></cipublic-spinner>

Page Footer

I want to...

Get an ID card File a claim View my claims and EOBs Check coverage under my plan See prescription drug list Find an in-network doctor, dentist, or facility Find a form Find 1095-B tax form information View the Cigna Glossary Contact Cigna

Audiences

Individuals and Families Medicare Employers Brokers Providers

Secure Member Sites

myCigna member portal Health Care Provider portal Cigna for Employers Client Resource Portal Cigna for Brokers

Cigna Company Information

About Cigna Company Profile Careers Newsroom Investors Suppliers Third Party Administrators International Evernorth

 Cigna. All rights reserved.

Privacy Legal Product Disclosures Cigna Company Names Customer Rights Accessibility Non-Discrimination Notice [PDF] Language Assistance [PDF] Report Fraud Sitemap

Disclaimer

Individual and family medical and dental insurance plans are insured by Cigna Health and Life Insurance Company (CHLIC), Cigna HealthCare of Arizona, Inc., Cigna HealthCare of Illinois, Inc., and Cigna HealthCare of North Carolina, Inc. Group health insurance and health benefit plans are insured or administered by CHLIC, Connecticut General Life Insurance Company (CGLIC), or their affiliates (see a listing of the legal entities  that insure or administer group HMO, dental HMO, and other products or services in your state). Accidental Injury, Critical Illness, and Hospital Care plans or insurance policies are distributed exclusively by or through operating subsidiaries of Cigna Corporation, are administered by Cigna Health and Life Insurance Company, and are insured by either (i) Cigna Health and Life Insurance Company (Bloomfield, CT); (ii) Life Insurance Company of North America (“LINA”) (Philadelphia, PA); or (iii) New York Life Group Insurance Company of NY (“NYLGICNY”) (New York, NY), formerly known as Cigna Life Insurance Company of New York. The Cigna name, logo, and other Cigna marks are owned by Cigna Intellectual Property, Inc. LINA and NYLGICNY are not affiliates of Cigna.

All insurance policies and group benefit plans contain exclusions and limitations. For availability, costs and complete details of coverage, contact a licensed agent or Cigna sales representative. This website is not intended for residents of New Mexico.

Selecting these links will take you away from Cigna.com to another website, which may be a non-Cigna website. Cigna may not control the content or links of non-Cigna websites. Details