Hirschsprung's Disease

Also known as: Hirschsprung disease, megacolon

What is Hirschsprung's disease?

Hirschsprung's disease describes a congenital condition (happens before birth) where nerve cells in the wall of the large bowel (colon) that normally develop during intrauterine development are missing. These nerve cells are called ganglion cells and when missing from the intestinal wall the breakdown products of food are blocked from moving forward through the colon to the anus, and cannot be passed as stool. It occurs in boys more often than girls and may be associated with some inherited conditions. The length of bowel that may be affected may be short or long and is frequently found in the end parts of the large bowel (upper rectum or sigmoid colon).

What causes Hirschsprung's disease?

The cause of Hirschsprung's disease isn’t entirely clear. It appears to be hereditary in some cases.

What are the symptoms of Hirschsprung's disease?

The main symptom of newborn babies with Hirschsprung's disease is constipation (not passing a stool for 48 hours) from bowel obstruction. Other symptoms include vomiting green or brown vomit, swelling of the belly, the passing of a lot of gas, and bloody diarrhea.

What are Hirschsprung's disease care options?

The primary treatment for Hirschsprung's disease is surgical removal of the portion of the colon without any nerve cells.

Reviewed by: Juan L Calisto, MD

This page was last updated on: 3/23/2018 2:08:56 PM


Dr. Lina Felipez is Director of Inflammatory Bowel Disease (IBD) Research and a pediatric gastroenterologist within the Division of Gastroenterology at Nicklaus Children's Hospital in Miami.

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