Children with Hirschsprung disease need surgery, regular follow-up visits and sometimes other treatment. At Seattle Children’s, our surgeons partner closely with other experts from Reconstructive Pelvic Medicine to provide all the care your child needs.
Surgery for Hirschsprung Disease
Surgeons remove the segment of intestine that does not have ganglion cells. This always includes the rectum, and it may include part of the colon. Then, the surgeon connects the remaining intestine to your child’s anus. This surgery is called a pull-through.
There are several ways to do a pull-through. The main ways are called the Swenson, Soave and Duhamel procedures. They differ in how much of the intestine surgeons remove and how surgeons connect the remaining intestine to the anus. There is no evidence that one procedure is better than the others.
Also, there are different ways for surgeons to reach the intestines:
- Through small “keyhole” incisions in the belly (laparoscopic surgery)
- Through a single larger incision (open surgery)
- Through the child’s anus (transanal pull-through)
Your child’s team will talk with you about what they recommend. You and your child’s surgeon will decide which surgery is right for your child. Your child will get medicine to make them sleep without pain during surgery (general anesthesia).
Many babies have surgery for Hirschsprung disease soon after birth. But some children need another operation first if they are very ill with an infection in the intestines (enterocolitis) or if the intestines are very stretched out from being blocked. This operation is called an ostomy.
Surgeons make an opening, or stoma, in the belly. Then they cut through the intestines and attach the end of the intestines to the opening. This lets stool (feces) pass to a pouch attached on the outside of the body, which helps the intestines heal.
If your child needs this, you will get step-by-step details about how to care for the stoma and change the pouch. The ostomy is temporary. In a few months to a year, the intestines will recover. Then, your child’s surgeon will do one of the operations to connect the intestines to the anus.
Care Right After Surgery
After surgery for Hirschsprung disease, we will give your child pain medicine to make them comfortable. It’s likely they will also have:
- An IV (intravenous) line that goes into a vein to provide fluids.
- A tube that passes through the nose into the stomach (nasogastric tube, or NG tube). This keeps their stomach empty until gas and stool can pass through the intestines.
You can expect your child to be in the hospital for three to five days after surgery. Your child will stay in the hospital until they can eat well, they have a bowel movement and they have no fever or other signs of problems. Some children need to stay longer, based on their health and the extent of their surgery.
The surgery team will teach you what to do when your child goes home, such as how to care for incisions, how to feed and bathe your child, whether to limit your child’s activity for a while and what to watch for.
After healing from surgery, your child’s bowel movements may become normal. But surgery doesn’t cure Hirschsprung disease. Some children will have bowel problems — like constipation or fecal incontinence ( “accidents”) — off and on throughout their lives. Ongoing care from an expert team can help with these problems.
Sometimes, these issues occur when babies start eating solid food, when older children add foods that are harder to digest or when children resist going to the bathroom because they are distracted by other activities. There’s also some risk of enterocolitis, even after surgery.
The team at Seattle Children’s works to monitor and treat problems related to Hirschsprung disease in babies, children, teens and young adults even after surgery. Our goal is to follow your child until they reach college age to help ensure they are healthy and living to their full potential.
Your child will need to come back for follow-up visits on a regular basis to check their health. If your child is doing well, these visits may happen only once a year. If your child is having any bowel problems, we may see them more often for a while to provide treatment.
For bowel problems that might happen after surgery, we offer a range of treatment options, including:
- Using medicines and changing your child’s diet, activity level and toilet habits to reduce constipation and incontinence
- Having an internal anal sphincter Botox injection
- Taking part in our one-week Bowel Management Treatment Program
- Having another surgery to remove intestine that isn’t moving stool
Any treatment will be tailored to your child’s and family’s needs.