Irrigations can be performed at home by the parents to empty trapped stool from the lower bowels/intestines. Parents are taught how to perform irrigations during a hospital stay.
Irrigations can be done by inserting a small, flexible tube through the anus. A normal saline (salt) solution is then pushed through this tube into the intestines to wash out the trapped stool and gas as the tube is manipulated in and out. This should provide immediate relief for your child. The number of irrigations per day, the volume used, and the length of time that will be required is determined by your surgical team.
The treatment of HD begins with a surgical procedure. To identify the presence or absence of ganglion cells, a series of biopsies are done during surgery to identify the normal intestine, which will have ganglion cells and normal nerves. These biopsies are evaluated under a microscope by a pathologist during the surgery.
Surgery for HD may require a temporary ostomy. The ostomy is created at the site where the biopsy confirms ganglion cells and normal nerves. This allows the enlarged bowel to come down to normal size, which may take weeks to months.
|Large intestine with Hirschsprung disease.
|The healthy intestine is moved to an opening in the abdomen where a stoma is created. Stoma coming from the abdomen.
Once the intestine has had time to return to a more normal size, a second surgery would be done to remove the abnormal bowel. The normal bowel is then “pulled through” to the anus.
If an ostomy is not required, then the abnormal bowel will be removed and the normal bowel will then be “pulled through” to the anus.
|Large intestine with Hirschsprung disease before the pull-through procedure.
|First the diseased segment is removed.
|Then the healthy segment is “pulled through” and attached just above the internal sphincters.
After surgery, your child will remain in the hospital for a few days (length of stay varies depending on the child) to allow time to recover and heal properly. Your child will receive pain management and hydration through an I.V. (special thin, plastic tube that stays in the blood vessel). They may also have a small tube in their nose that ends in their stomach.
If your child had a pull through procedure that did not require an ostomy, then once the child begins to pass gas or stool, the tube from their nose will be removed and they will be allowed to eat. Once they are tolerating their feedings by mouth, passing stool on their own, and have no fevers, the I.V. will be removed and they can go home. In the first few weeks postoperatively, expect that your child will have 8-12 stools per day initially. Over time, the number of stools per day will lessen to 3-4 times per day. You will be given a skin care regimen to assist with their diaper rash.
If your child has an ostomy, one of the APRNs from the wound/ostomy team will meet with you in the hospital to develop a plan for at-home care.
Once your child is at home, they will still be recovering. You will be monitoring the amount of food or formula that they are taking. Keeping your child hydrated will be essential in recovery. Also, they may still need to take some medications. You will need to maintain good skin care in the diaper area or around the ostomy to prevent skin breakdown.
Your child will have a follow-up appointment with their surgeon and APRN one to two weeks after discharge. Once your child has fully recovered from surgery, they will be followed on a routine basis by your surgeon and the APRN group to ensure adequate weight gain and optimal outcomes.
After recovering from the surgery, most children with HD will have good long-term outcomes. They should continue to grow and develop normally. The surgeon and APRN group will continue to monitor their growth at their routine follow-up appointments. Most children will have daily bowel movements that are soft and easily passed. Some children may have loose stools that can be difficult to control or hard stools that can be difficult to pass. A good daily bowel management plan (hyperlink) will help your child manage these issues. Our team is committed to following and monitoring these patients through their childhoods to provide the best care and outcomes.
It is important to note that your child will always have Hirschsprung disease because they lack a normal “recto-anal inhibitory reflex.” Their sphincters still have a small amount of disease that cannot be removed without affecting their ability to hold stool in. A majority of children with HD are able to adapt to this small area of internal anal sphincter achalasia (non-relaxation). HD is a life-long condition that does require monitoring as the child grows and through toilet training years.
Patients with HD may suffer from Hirschsprung-associated enterocolitis (HAEC). This can occur before or after their surgery. Some children with HD have an underlying dysmotility of the intestines, which can lead to stasis (accumulation) of the stool, causing subsequent bacterial overgrowth and then diarrhea.
This overgrowth can cause symptoms such as fevers, abdominal pain, nausea, vomiting, failure to thrive, intermittent cramping, constipation, diarrhea, abdominal distention/bloating, a decreased desire to eat and/or lethargy or increased sleepiness.
If this occurs, you should call your surgeon’s office or bring them to the emergency room for prompt treatment.
Illustrations courtesy of digestive.niddk.nih.gov.