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: September 06, 2019 09:21 AM

Related Videos

How to Perform Rectal Irrigations in the Hirschsprung-Associated Enterocolitis Patient

Nicklaus Children's Hospital Nurse Practioner, Raquel Pasarón, DNP, APRN, FNP-BC, walks you through performing rectal irrigations in the Hirschsprung-associated entercolitis patient. Enterocolitis in the Hirschsprung patient may occur before or after surgery is performed. 

What is Hirschsprung's disease?

Dr. Juan Calisto, Director of the Colorectal Center, discusses Hirschsprung's disease.

De La Torre Technique for treating Hirschsprung's Disease

Dr. Juan Calisto discussed the De la Torre Technique used in the treatment of patients with Hirschsprung's Disease in this edition of Children's Health Chats.

Learn more about

Pull-Through Procedures for Hirschsprung's

Hirschsprung's disease is genetic disorder in which newborns lack nerve cells in their intestines. As a result, they have trouble passing stools and become constipated. Pull-through procedures for Hirschsprung's are procedures used to treat Hirschsprung’s disease and remedy the problem. Learn more

Posterior Sagittal Anorectoplasty

Posterior sagittal anorectoplasty is a surgical procedure used to repair birth defects related to the passing of stool through the rectum and anus. Anorectal malformations, cloacal malformation and Hirschsprung’s disease are just a few of the conditions treated by the procedure. Learn more