Health Library

Hirschsprung's Revision Surgery

What is Hirschsprung's Revision Surgery?

Almost all children with Hirschsprung's disease need surgery. This is called a pull-through procedure. A surgeon removes the diseased part of the colon. Then they pull the healthy portion of the colon down to the anus. They connect it to the anus so the child can poop normally.

Complications Related to the First Hirschsprung's Surgery

Most children who have treatment and a successful surgery for Hirschsprung's disease function normally. But sometimes children can develop significant challenges.

These can include:

• Constipation
• Diarrhea
• Severe bloating
• Bowel obstruction (blockage in the colon)
• Soiling (poop accidents)
• Leaking poop (incontinence)
• Enterocolitis, a potentially life-threatening infection of the colon

Surgeons develop a treatment plan based on your child’s specific complications. This may include laxatives, dietary changes, pelvic floor physical therapy or enemas. On occasion, a surgery may be needed to fix an issue with the original pull-through.

Why a Child May Need a Hirschsprung's Revision Surgery

A child who has complications after their first Hirschsprung's surgery will need to see their surgeon, or a surgeon specialized in complex colorectal conditions. The surgeon will do tests to better understand the problem.

These tests typically include:

• Abdominal X-ray to look for a bowel obstruction
• Contrast enema to examine the colon
• Exam under anesthesia to:
• Check the connection between the colon and the anal canal
• Look for a narrowing in or damage to the anal canal
• Identify a twist or kink in the pull-through
• Take a sample of the pull-through tissue for a repeat biopsy
• Anorectal manometry (using a small, thin tube inserted into the rectum) to measure pressures and check reflexes
• Total colonic manometry (using a small, thin tube inserted into the colon) to measure pressures and check reflexes

Sometimes, the biopsy of the pull-through tissue shows that it doesn’t have a normal amount of ganglion cells. This is called transition-zone pull-through. It may explain why things are not functioning normally. In many cases of transition-zone pull-through, a revision surgery may be needed.

Some children may develop a narrowing in the anal canal over time that makes it hard to poop. This is called a stricture. Depending on the surgical technique, this happens in about 4% to 22% of children. Strictures often need to be repaired through surgery. There are a variety of other issues that can happen that are specific to the various techniques used to perform the pull-through surgery, such as a cuff issue in Yancey-Soave pull-throughs or a spur issue in Duhamel pull-throughs. Some of these will need a revision surgery.

Treatment Options Before Hirschsprung’s Revision Surgery

Botox Treatment

Sometimes surgeons can’t identify a structural or physical problem with the surgery or the anatomy, but a child is still having functional problems, such as soiling, incontinence or severe bloating. In some cases, Botox, or botulinum toxin, may be used to relax the sphincter. This is done in a procedure under general anesthesia or sedation.

Bowel Management Program

Sometimes the child is placed on an enema program, which can be given through the bottom, or in some cases by a surgery called a Malone appendicostomy (or MACE, which stands for Malone Antegrade Continent Enema), or a cecostomy (a tube placed directly into the colon used to give daily flushes). This may be referred to as a bowel management program, which can help children with even the most challenging of problems become clean.

Sacral Nerve Stimulation

In other cases, sacral nerve stimulation may help control incontinence or improve the management of severe constipation. The sacral nerve stimulator is an implantable device that gives tiny electrical signals to the nerves that control the rectum. In some cases, this can help significantly improve the child’s ability to poop.

Ostomy

In extreme cases, a surgeon may create an ostomy. The surgeon moves the end of the bowel to stick out through the abdominal wall. This area is called a stoma. The child will wear a bag over the opening to collect poop. This is usually a temporary measure as part of a long-term plan to get to the best functional result possible. Your child’s surgeon will try to determine the best plan that maximizes safety, minimizes pain, and targets the best outcome for your child.

Treatment Options for Hirschsprung’s Revision Surgery

When Hirschsprung's revision surgery is required, there's no clear formula on how to best proceed. The plan is customized based on issues identified after the complete evaluation. There are small issues that can be handled with a small surgery, either performed laparoscopically or through the bottom (trans-anal). Then there are major surgeries where the entire procedure must be re-done. In some cases, the child has been having severe constipation leading to the bowel becoming extremely dilated (wide). In these cases, the surgeon may suggest starting with a diverting ostomy to allow the bowel to relax and shrink down before eventually performing a revision of the pull-through. Whatever the issue, your child’s surgeon will tailor the plan to your child and your family, and ensure that every issue has been explained fully.

Long-Term Outlook for Children with a Hirschsprung’s Revision Surgery

The outlook for children after Hirschsprung’s revision surgery remains very positive. Hirschsprung’s revision surgery has evolved significantly over the past 20 years. Surgeons today have the best diagnostic tests and surgical techniques to fix these often-challenging problems. The biggest concern for patients undergoing revision surgery—continence—seems to be similar to children who have had only one surgery for their Hirschsprung’s disease. There are some children who end up having more stooling issues after revision surgery, which is why most surgeons reserve surgery for only those problems that cannot be handled another way.