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What Is Hirschsprung Disease?

Hirschsprung disease is a problem in the intestines. Children are born with Hirschsprung disease (it is congenital).

Healthy intestines squeeze with a wave-like motion to move food and stool (feces) along the digestive tract. This motion is called peristalsis (pronounced pair-ih-STALL-sis). Special nerve cells called ganglion help make the intestines push. In children with Hirschsprung disease, the ganglion cells are missing. Most often, they are missing from the end of the large intestine (colon) or the rectum, where waste collects before passing out through the anus. In very rare cases, the ganglion cells are missing from part of the small intestine.

If the ganglion cells are missing, the intestine squeezes shut and stays that way. Because there's no wave-like motion, stool stops moving forward. The intestines can get blocked completely (obstructed) or your child can have ongoing constipation. Constipation means that bowel movements are difficult.

Hirschsprung disease increases your child's risk for having an infection called enterocolitis in the colon. The infection can be treated, but it can threaten a child's life if it is not treated appropriately.

Hirschsprung Disease in Children

Hirschsprung disease is congenital, which means a child is born with it. Certain forms of the disease can be passed from parent to child in the genes. But it's not common for more than one family member to have it.

The condition affects about 1 in 5,000 children. In about half of these children, doctors diagnose the condition soon after birth. Most of the rest are diagnosed by the time they are 2 years old.

Hirschsprung Disease at Seattle Children’s

Over the last decade, we have treated more than 100 children with Hirschsprung disease. We generally see 10 to 15 children each year with this disease. Our surgeons are experienced in performing the operation these children need to repair the intestines so stool can pass. We also have a one-week Bowel Management Treatment Program for children who have trouble with bowel control after surgery.

When you come to Seattle Children's, you have a team of people to care for your child before, during and after surgery. Along with your child's surgeon, you are connected with doctors trained to care for new babies with complex problems (neonatologists), nurses, dietitians, child life specialists, social workers and others. We work together to meet all of your child's health needs and help your family through this experience.

Since 1907, Seattle Children's has been treating children only. Our team members are trained in their fields and also in meeting the unique needs of children. For example, the doctors who give your child anesthesia are board certified in pediatric anesthesiology. This means they have extra years of training in how to take care of kids. Our child life specialists know how to help children understand their illnesses and treatments in ways that make sense for their age. Our expertise in pediatrics truly makes a difference for our patients and families.

Who Treats This at Seattle Children's?

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Summer 2014: Good Growing Newsletter

In This Issue

  • Understanding the Power and Influence of Role Models
  • Legal Marijuana Means Greater Poisoning Risks for Children
  • Why Choose Pediatric Emergency Care?

Download Summer 2014 (PDF)