Hirschsprung Disease

Hirschsprung disease occurs when a portion of a baby’s intestinal nerve cells don’t develop properly, leading to an obstruction of the intestine. This makes the child prone to infection which causes pain, fever and diarrhea.  Hirschsprung disease causes up to 20 percent of intestinal obstructions that occur in newborns. At the Colorectal Surgery Program, part of C.S. Mott Children’s Hospital at the University of Michigan, we provide comprehensive, multidisciplinary care for children with Hirschsprung disease. Our dedicated team of specialists evaluates and will create a customized treatment plan to begin your child’s individualized treatment plan immediately. We offer a truly multidisciplinary approach to caring for children with colorectal disorders, going beyond treatment and management of symptoms to support your child throughout childhood and even into adulthood.

Hirschsprung disease symptoms

In children with Hirschsprung disease, digested food and stool cannot move forward through the affected portion of the digestive tract.  The intestine becomes blocked with stool, causing constipation and pressure on the inside of the intestine, causing part of the intestinal wall to wear thin. Eventually, a bacterial infection can develop in the digestive tract, causing serious problems. Most children with Hirschsprung disease show symptoms in the first six weeks of life.  Symptoms vary, but the most common are:

  • failure to have bowel movement in the first 48 hours of life
  • gradual bloating of the abdomen
  • gradual onset of vomiting
  • fever

Children with only a short segment of intestine that lacks normal nerve cells may not show symptoms immediately.  In general, however, even children who don’t have early symptoms may experience:

  • constipation that becomes worse with time
  • loss of appetite
  • delayed growth
  • small, watery stools

Diagnosing Hirschsprung disease

Diagnosis begins with a thorough health history and a comprehensive physical examination. Some testing will be required and may include:

  • Abdominal x-ray to identify a lack of stool in your child’s large intestine as well swollen segments of the large and small intestine.
  • Barium enema to examine the large intestine for abnormalities
  • Anorectal manometry to measure nerve reflexes in the intestine
  • Biopsy of the rectum or large intestine

Treatment for Hirschsprung disease

Every child’s treatment plan is individualized, based on the patient’s needs and severity of disorder, utilizing cutting-edge technology and the latest research. Most children require surgery to fix the obstruction.  Usually, the surgery can be performed in a single stage. University of Michigan surgeons specialize in minimally invasive techniques whenever possible.  Learn more about pediatric surgery at Mott Children’s Hospital. Depending on how much intestine is needed to be removed, your child may have varying levels of long-term care needs. Temporary or intermittent problems may include:

  • frequent and loose stools
  • difficulty sensing the need to have a bowel movement.
  • problems with bowel movements because the anal opening is tight

From a long-term perspective, removing a large segment of the intestine can prevent a child from getting adequate nutrients and fluids.  Children can have problems with improper digestion, slow growth and infection.  Our nutritionists will work with you to set nutrition goals and offer suggestions on how to achieve your nutrition requirements to ensure your child grows and develops normally.

Discover the Michigan Difference in pediatric colorectal surgical care.

For more information or to make an appointment, call 1-877-475-MOTT (1-877-475-6688).