Hirschsprung disease is a difference in the nerves of the gut. Usually, nerve cells send signals to the intestinal muscles to move food forward. When they’re missing or not working right, the intestines get blocked with feces and expand to a larger-than-normal size.

The blockage puts pressure on the inside of the gut, stretching the intestinal wall. Eventually, this can lead to infection and other serious complications.

In Hirschsprung disease, this motility issue happens because some nerve cells do not form right in the womb. Most kids are missing nerve cells in only the last one to two feet of the large intestine. Researchers don’t know why the nerves stop growing. Because there currently is no known cause, there is no way to prevent it.

Hirschsprung disease may be genetic. If a parent has it, there’s a higher chance their baby will. If one child has it, it’s slightly more likely their sibling will. Boys are more likely to have it than girls. Children with Down syndrome have a 50 times higher risk of it.

Most kids have symptoms within the first 6 weeks of life. If only a short segment lacks normal nerve cells, they may not show symptoms for several months or years.

Symptoms may include:

• No poop in the first 48 hours of life
• Gradual bloating of the belly
• Gradual onset of vomiting
• Fever
• Constipation that gets worse with time
• Loss of appetite
• Poor weight gain or weight loss
• Passing small, watery stools

We tailor your child’s care plan to fit their age, health and needs.

Treatment may include:

• Bowel removal surgery: Most kids with Hirschsprung disease need surgery to take out the part of the bowel that doesn’t work. The surgeon may reattach the healthy bowel to the anus or create a colostomy.
• Colostomy surgery: The surgeon connects the bowel to a small opening in the belly so poop can pass into a bag. This is often temporary. Many kids later have surgery to reconnect the bowel and close the opening.
• Diet care: Dietitians help your child get the nutrition they need to grow and heal.
• Pain care: We help your child feel more comfortable before and after surgery.
• Therapy: Therapists teach kids ways to manage fear, stress or anxiety related to pooping or medical care.

Surgery usually fixes the main problem that is causing symptoms. Removing the affected section of bowel allows poop to pass normally.

However, some kids may still have issues during recovery or as they grow. These might include constipation, accidents, belly pain or infections. These problems usually improve with time, support and follow-up care.

A small number of children may need more care down the line. This might include dietary changes, medicine or therapy to manage symptoms.

At Children’s Wisconsin, our team has special training in rare and complex motility disorders. We are one of the few places in the US able to offer kids full motility testing and a dedicated neurogastroenterology program.

We use the latest tests to guide care. We partner with you, bringing long-term support for every part of your child’s life. We know how stressful digestive health issues can be. That’s why we support your whole family. We give you clear answers, a tailored care plan and a welcoming space where you feel heard.

Our dedicated experts are here to help your family cope with Hirschsprung disease.

Services we offer include

• Diagnostic services
• Second opinions
• Surgical treatment for Hirschsprung disease
• Pain care
• Nutrition care
• Ongoing management for kids who have post-surgical problems

Your First Visit

At your first visit, we take time to hear your child’s full story. We’ll look at their symptoms, health history, test results and treatments you’ve tried. Your child will get a physical exam, and we may have them do more tests to learn how their digestive system is working.

Our team is one of just a few pediatric centers to offer a full range of diagnostic tests for evaluating children with Hirschsprung disease.

Our testing may include:

• Manometry: These tests show how well the muscles and nerves are working in your child’s gut. It helps us pinpoint where that process stops working.
• Colonoscopy: We use a thin, flexible tube to see the lining of the bowel. The doctor can check for any swelling, narrowing or signs of infection. It also helps us plan surgery.
• Endoscopy: We use this test to rule out other digestive problems. It lets us look at your child’s esophagus, stomach and upper small intestine.
• Upper GI study: This test shows how food moves through your child’s stomach and small intestine. We use this test to rule out other digestive problems.

We’ll explain each step of your child’s care plan clearly. We’re here to answer your questions and talk through your goals. Together, we’ll build a care plan that fits your child’s needs and works for your family.

During Treatment

We take a team-based, whole-child approach. We don’t focus on just their physical symptoms. We also work to improve their nutrition, emotional well-being, family routines and long-term quality of life.

When treating your child, our team will work with your family to develop a unique, high-quality care plan.

Your child’s care team may include:

• Your child’s digestive care doctor (pediatric gastroenterologist)
• Nurses with training in motility disorders
• Dietitians who focus on digestive health
• Psychologists who help kids with chronic illness
• Surgeons
• Pain care experts
• Urologists, if your child has urinary issues

We check in regularly, adjust the plan as your child grows, adding experts as needed. Our team is here to help if your child has pain, trouble eating or school and social issues. The families we work with feel supported and empowered.

After Care

Most children do well after surgery. Constipation or stool accidents can happen, but they often get better as your child grows. Many kids with Hirschsprung disease go on to live healthy, active lives.

Some kids need ongoing care after surgery. We’ll track your child’s progress, help you manage any symptoms and work closely with your local doctor.

We’ll check for:

• Loose or frequent stools: This is common at first. Gentle cleaning and diaper cream can help protect the skin.
• Trouble sensing bowel movements: This can improve with time. We may suggest having your child sit on the toilet after meals to help develop a routine.
• Tight anal opening: If needed, your care team may teach you how to do rectal dilations at home to prevent narrowing.
• Nutrition problems: If the surgeon removed a large part of the intestine, your child may need help gaining weight, staying hydrated and absorbing nutrients. Our nutrition team will create a tailored diet plan.

We’re here for as long as you need us. We’ll help you manage any bumps in the road — and support your child’s health every step of the way.