Surgical Treatment and the Pull-Through Procedure
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The primary treatment for Hirschsprung disease is a pull-through surgery, which removes the intestine lacking nerve cells and connects the healthy bowel to the anus. Surgeons typically use the Soave, Duhamel, or Swenson techniques to perform the procedure and restore bowel function.
Key Takeaways
- • The primary treatment for Hirschsprung disease is a pull-through procedure, which connects healthy bowel to the anus.
- • Rectal irrigations (washouts) are critical before surgery to keep the bowel clear and prevent stretching or infection.
- • The Soave, Duhamel, and Swenson procedures are the three main surgical techniques used, often performed transanally.
- • A severe, blistering diaper rash is very common immediately after surgery due to frequent, highly acidic stools.
- • Surgery may be performed in a single stage, or in multiple stages utilizing a temporary ostomy if the baby is very sick.
The goal of surgery for Hirschsprung disease is to remove the part of the intestine that lacks nerve cells and connect the healthy, working bowel to the anus [1][2]. This procedure is called a pull-through. While it is a major operation, most children go on to have good bowel function and lead active lives [3][4].
Preparing for Surgery: The “Washout”
Before surgery, the bowel must be kept clear to prevent infection and stretching. This is often done through rectal irrigations (also called washouts) [5]. Using a small tube and a salt-water solution, you or a nurse will gently flush the stool out of the baby’s colon.
- Safety Warning: Never force the catheter if you feel resistance, as this could puncture the baby’s bowel. If the water goes in but does not come back out, gently massage the belly or carefully pull the tube back slightly. If it still doesn’t return, contact your medical team.
- Effective Decompression: If washouts work well to keep the belly flat, the surgeon may wait until the baby is about 3 months old to perform the pull-through [5].
The Three Main Techniques
There are three classic ways to perform a pull-through. Your surgeon will choose the one they are most experienced with and that best fits your child’s anatomy.
- Soave Procedure: The surgeon slides the healthy bowel through the “sleeve” of the abnormal rectum, leaving the outer layer of the rectum in place [6][7]. Why choose it? It protects the nerves around the rectum, minimizing the risk of injuring structures that control urine or sexual function.
- Duhamel Procedure: The surgeon pulls the healthy bowel down behind the abnormal rectum and joins them together, creating a new, larger pouch for stool [8][9]. Why choose it? It is often preferred for Total Colonic Aganglionosis (TCA) because the larger pouch can help absorb more water and slow down bowel movements.
- Swenson Procedure: The surgeon removes the entire abnormal segment of the rectum and colon and connects the healthy bowel directly to the anal canal [10][11]. Why choose it? It is the original procedure and completely removes all the aganglionic tissue, which some surgeons believe reduces long-term constipation.
Many of these surgeries are now done using Transanal Endorectal Pull-Through (TEPT), meaning the surgery is done through the anus without large abdominal incisions [2][3].
What to Expect in the Hospital
A typical pull-through surgery requires a hospital stay of a few days to a week. The baby will likely receive IV pain medication initially before transitioning to oral pain relievers. Oral feedings (breastmilk or formula) usually resume once the bowel “wakes up,” signaled by the baby passing gas or having their first stool.
The Immediate Aftermath: Severe Diaper Rash
Warning: Immediately after a pull-through, your child will begin passing stool constantly—sometimes 10 to 20 times a day. Because this stool has not sat in the colon to absorb water, it is highly acidic and will cause a severe, blistering diaper rash within hours if untreated.
- Proactive Care: Before surgery, stock up on heavy barrier creams (like Ilex paste or thick zinc oxide). Apply a thick layer to the baby’s bottom immediately after surgery and reapply diligently with every diaper change to protect their skin.
One Stage or Two?
- Primary Pull-Through (One Stage): The abnormal bowel is removed and the healthy bowel is connected all in one surgery [2][3].
- Staged Procedure (Two or Three Stages): If the baby is very sick, the surgeon may first perform an ostomy (stoma) [12][13]. A small opening is made on the belly for stool to pass into a bag. The pull-through happens in a later surgery.
For what to watch out for after returning home, see Managing Complications and HAEC.
Frequently Asked Questions
What is a pull-through surgery for Hirschsprung disease?
What are rectal washouts and why are they needed?
What are the different types of pull-through procedures?
Will my baby need a stoma bag for Hirschsprung disease?
How can I prevent severe diaper rash after a pull-through surgery?
Questions for Your Doctor
- • Which pull-through technique (Soave, Duhamel, or Swenson) do you use most often, and why is it the best fit for my child's specific subtype?
- • How many pull-through surgeries for Hirschsprung disease do you perform each year, and what are your typical success rates for single-stage procedures?
- • What are the specific criteria you use to decide if my child needs a temporary stoma (staged surgery) versus a primary pull-through?
- • If my child has Total Colonic Aganglionosis (TCA), how will their surgery and long-term recovery differ from a child with a shorter affected segment?
- • What is your team's protocol for managing and preventing Hirschsprung-associated enterocolitis (HAEC) after surgery?
- • What should we expect during the hospital stay in terms of days admitted and pain management protocols?
Questions for You
- • Am I comfortable performing the daily rectal irrigations (washouts) at home while we wait for surgery?
- • Have I discussed the pros and cons of a one-stage versus a two-stage surgery with the surgical team?
- • What is our family's plan for managing a temporary stoma if one is necessary (e.g., supplies, skin care, and support)?
- • Are we prepared for the 'diaper rash' and frequent stools that often occur immediately after the pull-through surgery?
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References
- 1
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Granström AL, Irvine W, Hoel AT, et al.
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PMID: 29703156 - 10
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PMID: 41454984 - 11
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This page explains surgical treatments for Hirschsprung disease for educational purposes. Your child's pediatric surgeon is the best source for discussing specific procedures and surgical care plans.
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