Hirschsprung disease happens when a baby’s large intestine (colon), and sometimes the small intestine, don’t have nerve cells that help move stool. It’s a type of gastrointestinal (GI) motility disorder, which means stool (poop) doesn’t move through the intestines as it should.

The condition causes chronic constipation. Some children develop potentially life-threatening intestinal inflammation (enterocolitis) or intestinal obstructions (bowel blockage).

Also called congenital megacolon and HSCR, Hirschsprung disease is a congenital abnormality, meaning it is present at birth. The condition gets its name from Danish pediatrician, Dr. Harald Hirschsprung, who first described it in 1886.

There are different types of Hirschsprung disease:

• Short-segment (most common): There are no nerve cells in the rectum and possibly the sigmoid colon (the last parts of the large intestine that connect to the anus, where poop comes out).
• Long-segment: There are no nerve cells in the rectum and a long portion of the large intestine.
• Total colonic: There are no nerve cells in the rectum and all of the large intestine.
• Small intestinal: There are no nerve cells in the rectum, large intestine and the end of the small intestine.
• Total intestinal: There are no nerve cells in the rectum, large intestine and most (or all) of the small intestine.

Doctors aren’t sure what causes Hirschsprung disease. Something happens while a baby is in the womb that stops intestinal nerve cells from growing as they should. In some children, a gene change (mutation) may play a role.

Certain factors may increase a child’s risk of Hirschsprung disease, including. 

Constipation is a common problem in children, making Hirschsprung disease easy to overlook. It’s important to see pediatric surgical and GI specialists, such as our experts at UC Davis Children’s Hospital, who are familiar with the condition.

About half of all diagnoses occur during a child’s first year of life, and almost all children (90%) who have Hirschsprung disease receive a diagnosis by age 13. Your child’s doctor will review symptoms, do a physical exam and order diagnostic and medical imaging tests to make a diagnosis.  

A child with Hirschsprung disease needs surgery to help them poop. Our pediatric colorectal surgeons are experts at Hirschsprung disease surgery. They also have the experience and skills to deliver medical care and explanations in a child- and family-friendly way. Your child may still have digestive problems that require long-term care, and our compassionate team guides you and your child throughout the process. We offer comprehensive services for children with Hirschsprung disease.