Treatment for Hirschsprung disease involves surgery. Surgeons remove the segment of the intestines that does not have ganglion cells. This always includes the rectum. Depending on where your child lacks ganglion cells, it may also includes part of the colon. Then, the surgeon connects the part of the intestine that has ganglion cells to your child's anus.
There are several ways to do this surgery. In all types, your child will get medicine to make them sleep without pain during surgery (general anesthesia).
Surgery for Hirschsprung Disease
To treat Hirschsprung disease, you and your child's surgeon will consider several options for surgery. The primary surgeries are called the Swenson, Suave or Duhamal operations. Each of these operations has different versions. Which type is best for your child depends on their condition. You and your child's surgeon may also prefer one type of surgery over another. Some of the different types of surgery are:
- Minimally invasive procedure. The surgeon makes a few small cuts (incisions) in your child's belly. Then, they insert a thin, lighted tube with a camera and their surgical tools. This is sometimes called laparoscopic surgery.
- Open surgery: The surgeon uses a single cut to get to the intestines that need to be removed. The incision is usually larger than those used in minimally invasive surgery.
- Transanal pull-through: Sometimes the surgeon can reach the intestines through your child's anus. Then, they remove the unhealthy section and pull the remaining intestines down.
- Combination: In some cases, surgeons use laparoscopic or open techniques along with a transanal operation.
All of these surgical options are available at Seattle Children's. Your child's surgery team will talk with you about their recommendations.
Many babies have surgery for Hirschsprung disease soon after birth. But in some cases, babies need another operation first. This operation is called an ostomy. Your baby may need it if doctors think there is an infection in the intestines or if the intestines are very stretched out from being blocked.
In 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 waste pass to a pouch attached on the outside of the body. It gives doctors a chance to wash out the intestines, and helps the intestines heal.
This situation is temporary. In a few months to a year, the intestines recover. After they recover, your child's surgeon will do one of the operations to connect the intestines to the anus.
If your child needs an ostomy, you will get step-by-step details about how to care for the stoma and change the pouch.
After Surgery for Hirschsprung Disease
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, pass waste and have no fever or other signs of problems. Some children need to stay longer, depending on their health and the extent of their surgery. Your child's surgical team can give you an idea of how long your child will be in the hospital.
After your child goes home, you'll need to keep incisions clean and dry until they heal. The surgery team will teach you how to care for incisions and how to feed your child. They will tell you if you need to limit your child's activity for a while.
The skin around your child's anus needs special care after surgery. At first, your child will pass stool often. The skin around the anus tends to get irritated. Your child's healthcare team will teach you how to keep skin irritation down.
We have a one-week Bowel Management Treatment Program for children with Hirschsprung disease who have trouble with bowel control after surgery.