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Hirschsprung Disease in Children

What is Hirschsprung disease?


Hirschsprung disease is a birth defect in which the end of a child's bowel does not contain all of the typical nerve cells. The condition occurs in approximately one in 5,000 newborns. Children with other medical conditions, such as Down syndrome, are at a higher risk of having Hirschsprung disease. 

The lack of nerve cells along the bowel in a child with Hirschsprung disease causes issues with passing stool. Because of the lack of ability to sense when stool need to be passed, the bowel does not relax and can cause blockages. These blockages can lead to damage within the bowel. Bowel movements can be difficult to pass even after surgery, as can toilet training, so we are available to help with this as well.

Bowel management for Hirschsprung


Bowel management is a clinical experience in which we work to empty the patient's bowels and colon. This experience can be either outpatient through clinic visits, inpatient or a one-week intensive outpatient experience (we call it bowel management bootcamp). These programs are available for children of all ages who are unable to anticipate or control their bowel activity. This experience is tailored to help patients resume normal bowel activity.

Treatment for Hirschsprung disease

 


Children’s Mercy is home to one of a very few Comprehensive Colorectal Centers in the nation. We take a team-based approach to care for children with colorectal malformations such as Hirschsprung disease and other anorectal and pelvic floor disorders, such as imperforate anus/anorectal malformation or cloaca).

Patients with anorectal and pelvic malformations have the potential to have many medical or surgical interventions during their lifetime. A multidisciplinary approach can reduce surgical interventions, anesthetic procedures, endotracheal intubations, and hospital/outpatient visits.

Easing symptoms with rectal irrigation

We also teach parents methods to potentially ease symptoms at home, like rectal irrigation, when appropriate.

How to Perform Rectal Irrigations

Hirschsprung disease can cause your child to have a backup of stool and gas in their colon. This can make them ill and struggle to have a bowel movement. However, there are a few steps you can take that will help keep your child’s colon clean. The Colorectal Center has provided this video as a visual resource for families who need to perform rectal irrigations at home.

What is enterocolitis and should I be worried as a parent?


Hirschsprung's associated enterocolitis (HAEC) or enterocolitis is an inflammation of the colon. Enterocolitis can occur in kids with Hirschsprung disease both before and after surgery and can make your child very sick. Please always call your doctors office to check if you think your child may have enterocolitis.

Symptoms of enterocolitis

  • A swollen tummy
  • Bleeding from the bottom
  • Diarrhea, especially foul-smelling
  • Fever
  • Lack of energy
  • Vomiting