Hirschsprung's Disease | Treatments

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What are the treatment options for Hirschsprung's disease?

Our surgeons frequently perform a single operation to fix intestinal obstruction when Hirschsprung's disease is initially diagnosed. The goal of the surgery is to remove the diseased section of the intestine and to pull the healthy portion of the intestine down to the anus. This is called a pull-through procedure. In most cases, this surgery can be done with minimally invasive techniques. It can sometimes be performed entirely through the anus, leaving no scars at all. Your surgeon can discuss different surgical techniques with you to determine the best option for your child.

What is the outlook for children with Hirschsprung's disease?

It is not uncommon for children with Hirschsprung’s disease to continue to have problems after surgery. At Boston Children’s, our gastroenterologists are dedicated to evaluating and caring for children who continue to have issues related to this disease.

These problems depend on how much unhealthy intestine needed to be removed during surgery and the current functioning of the colon, rectum and anus.

Possible problems include:

  • Intractable constipation
  • Diarrhea
  • Stool accidents
  • Frequent episodes of infections (enterocolitis)
  • Recurrent hospitalizations
  • Severe abdominal distention (a very bloated stomach)
  • Pain
  • Inability to tolerate food
  • Vomiting

Children who have had a large section of intestine removed may also experience long-term digestive problems. Removing a large segment of the intestine can prevent a child from getting adequate nutrients and fluids, leading to problems with improper digestion, slow growth and infection.

Our team will provide a thorough evaluation to locate the problem using one or more advanced diagnostic tests, such as anorectal manometry, a barium enema or a biopsy of the rectum or colon. These tests can provide doctors with a clear picture of how the colon is working after surgery and whether the repair was successful.

Our doctors will then collaborate on a treatment plan that may include:

  • Medications to either slow the transit of the stool in the colon or to make it faster
  • Injections of Botulinum toxin (Botox) into the anal sphincter to relax this muscle
  • Other rectal interventions or surgical procedures that may include redoing previous operations
  • Further removal of abnormal intestine
  • Appendicostomy, a surgical procedure to flush out and empty the colon by creating an opening outside the belly
  • Bowel Management Program
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