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Hirschsprung Disease in Children

What is Hirschsprung disease?


Hirschsprung disease is a birth defect in which nerve cells that naturally occur in the colon are missing. It occurs in approximately one in 5,000 newborns. Children with Down syndrome and other medical problems, such as congenital heart defects, are at greater risk of having Hirschsprung disease. 

Normally, the bowel contains many nerve cells all along its length which control how the bowel works. When the bowel is missing nerve cells, it does not work well. The bowel without nerve cells does not relax and causes blockages in the bowel. When blockage occurs, the stool does not move through the bowel normally. 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 on getting the bowels and colon empty and the child clean for poop. 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.

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