Each child with Hirschsprung’s disease has unique needs. The multidisciplinary care team will make a treatment plan suited for your child’s condition and overall health.
Based on your child’s needs and stage of treatment, the care team may include:
- Specialized pediatric colorectal surgeon
- Dedicated nurse
- Pediatric gastroenterologist specializing in motility (motion of the digestive system)
- Registered dietician
- Pelvic floor physical therapist
- Social worker
- Child life specialist
- Other experts as needed
In cases of total colonic Hirschsprung's disease children need to have the affected non-functioning intestine removed.
Due to the complexity of the condition, the corrective treatment must be performed in stages.
Children with total colonic Hirschsprung’s disease will require rectal irrigations at the time of initial diagnosis. Performing these irrigations will relieve the obstructed non-functioning bowel by removing the trapped stool and gas. Rectal irrigations are something your healthcare team will teach you how to perform at the time of TCHD diagnosis.
The first surgery is to create an ileostomy. An ileostomy has an opening in the small intestine just above the colon (the ileum) that is brought to the skin. The purpose of the ileostomy is so the stool and gas can come out of this opening before it gets to the poorly functioning colon.
After creation of ileostomy, rectal irrigations may still be needed to prevent enterocolitis symptoms.
Prior to surgery to close the ileostomy, the patient should also be able to accept rectal irrigations. These irrigations help treat and prevent enterocolitis. Patients with TCHD are more likely to develop enterocolitis and therefore must be able to accept rectal irrigations before the surgeon is comfortable with closing the ileostomy.
The next step in the surgical treatment of Hirschsprung’s disease involves removing the part of the intestine that isn’t working properly and connecting the working end of the small intestine (the ileostomy end) to make a connection at the anus. This allows for the child to have bowel movements. For more details about this surgery, discuss this with your surgeon.
Timing of this surgery varies depending on the surgeon. Most surgeons who treat this condition prefer to wait until the child is at least a year of age before considering performing this surgery. Some surgeons prefer to wait until the child is potty-trained for urine. The key to determining if a child should have this step of the surgery involves understanding what will happen after surgery.
Once the surgery happens, the stool that had been coming out the ileostomy will now be coming out of the bottom. If the stool is loose and watery, there may be frequent accidents in a potty trained child. They may have six or more loose bowel movements in a day and may also have stooling at night as well. In a diaper aged child, the frequent watery stool may have a risk of severe diaper rash.
To help prevent this, when a child is over a year of age, we may suggest a trial where we attempt to thicken the stool with constipating foods, fiber and medication, if needed, to slow the down the small intestine. If the stool is a good consistency that would be good for future potty training then this surgery would likely be successful. Success means good long term outcome and quality of life.
Rectal irrigations may be needed EVEN AFTER the colon has been removed and stool is now coming out from the bottom. The irrigations are needed to prevent or treat enterocolitis symptoms. Rectal irrigations are something your healthcare team will teach you how to perform at the time of TCHD diagnosis.