Update Course Rewind: Botox in Hirschsprung Disease 2023
Timestops (9)
Tools Used
Topic Overview
Key Takeaways
- Recurrent enterocolitis post-pull-through requires ruling out mechanical obstruction before attributing to sphincter dysfunction.
- Botox (100 units in 1mL saline, 3-4 injections at dentate line) may reduce hospital stay length and recurrent obstructive episodes.
- Prophylactic Botox has not been proven to prevent enterocolitis but may benefit high-risk subsets with recurrent disease.
- Total colonic Hirschsprung paradoxically has higher enterocolitis rates than short-segment disease despite less residual colon.
- Aganglionic internal sphincter preservation for continence creates stasis, requiring diligent colon clearance to prevent bacterial overgrowth.
Keywords
Hashtags
Transcript
Globalcast MD along with Cincinnati Children's Hospital, sharing knowledge to improve child health around the globe. Hello pediatric surgery family. I'm M. Godi, a research fellow from Cincinnati Children's Hospital Medical Center. Our 11th annual update course in pediatric surgery was held past August. In this video series, we'll recap the sessions and share the main highlights with you. Today, our topic is Botox in Hirschsprung disease. Joining the discussions are Dr. Stephen Lee, Caitlyn Smith, and Julia Grabowski. Here's our clinical scenario. Five-month old male with diagnosis of Hirschsprung disease, status post Swenson pull through an infancy at your institution. Patient now has had two episodes of Hirschsprung associated enterocolitis responding to home irrigations. There's been no evidence of a stricture on rectal examination and contrast enema was done without any abnormality. Pathology review, then no evidence of a transition zone on pull through. What is your next step? You have to always do your due diligence with a patient who has had their pull through, why are they getting recurrent enterocolitis? And one of the answers is you did a great operation and they have enterocolitis because they have a poorly functioning sphincter, which every baby with Hirschsprung disease has by nature of the disease. So, first you want to make sure that it's not a mechanical obstruction. And here Dr. Smith is opening a discussion for using Botox in these patients. How do you do your Botox? In reading every paper that was ever written on Botox in the anus in the last couple of months, people are doing any amount of Botox in any alloquats in any number of locations. Yes. So, I am 100 units in 1 milliliter. I'm usually more like three four in the dentate line. What about you? Yeah, I do 100 units, same 1 cc of saline, what's been commonly practiced. And then ultrasound plus or minus per your preference. Yes. What are you doing? Cause it. I know, you know, no matter what operation we do for Hirschsprung, we always leave a ganglionic bowel. That bowel is at least the internal sphincter because we can't take that out. So, I was wondering, do you think that that is creating the fertile grounds for enterocolitis or is it a mucosal disease? Is it immunologic? What are your thoughts? In order to maintain continence, you want to preserve the dentate line and you have to leave a tiny bit of a ganglionic internal sphincter. And I think that babies outgrow it because their external sphincter is able to overcome their internal sphincter. And I think that takes time for their body to mature, but I don't know that we have pinpointed the actual pathophysiology. The Hirschsprung physiology sorts of sets up the colon to act like a pond, right? There's poor emptying and motility issues and if you're not very diligent and about clearing the colon, it just has the ability for the bacteria to overgrow and create illness in that setting. Basically, all of the things that prevent enterocolitis are working towards the opposite in these patients. And what we should do is to improve clearance of stool from the colon and not allow for it to sit there stagnant. Something that's very interesting is we know that there's a significantly higher rate of enterocolitis in Hirschsprung disease after pull through. So, when you take out the entire colon, they have a higher rate of enterocolitis than babies who have shorter segment and have all that colon to have bacteria. So that is always been a little counterintuitive to me. And also much higher rate of enterocolitis in children with try 21. We don't really have a good reason to explain that. And you can still get enterocolitis even if you're doing rectal irrigations at home. There are times when it just not enough to empty that colon. There is discussion of whether or not when you when you make the diagnosis of total colon Hirschsprung, at that time should you do the colectomy because of the risk of enterocolitis, and I don't have a strong opinion on that. There are numerous studies and most of them are single institution retrospective ones. The thing about Hirschsprung disease, I think we all realize is that there's so much phenotypic variance. So, in studying the use of Botox in post pull through enterocolitis, the data is not perfect. According to Dr. Smith, even prospective data is not going to be perfect because people are very different. And as surgeons, you have different types of operations that you do, you have different levels of disease, you have different other comorbidities, syndromes, etc. Some of the data suggests that prophylactic Botox has not been shown to decrease the risk of enterocolitis, but it has been shown to decrease the length of stay in patients who have been admitted for enterocolitis. Also has been shown to potentially decrease hospitalizations in patients who present with recurrent episodes of obstruction or enterocolitis. Maybe not everyone needs it at the time of pull through, but maybe there is a subset of patients who really do have some predisposition to enterocolitis and using Botox as part of that treatment strategy would be beneficial. To sum it up, Hirschsprung disease is characterized by a lack of nerve cells in the colon, which causes problems with bowel movements. Even after surgical procedures like the Swenson pull through, patients might still experience recurrent enterocolitis, possibly because of a naturally defective sphincter. Botox has been considered as a potential treatment, both when administered as 100 units in 1 milliliter of saline. The underlying condition of the disease can lead to excessive bacterial growth in the colon, emphasizing the need for regular clearing. While there's some evidence indicating Botox might not consistently prevent enterocolitis, it has shown potential in reducing both the length of hospital states and recurrent episodes. Further research is needed to determine its precise utility. Thank you for watching this video. Don't forget to subscribe to the Statcurrent MD YouTube channel. Follow our social media channels and download the Statcurrent MD app for tons of content in pediatric surgery. Globalcast MD along with Cincinnati Children's Hospital, sharing knowledge to improve child health around the globe.