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Global Cast MD, along with Cincinnati Children's Hospital,

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sharing knowledge to improve child health around the globe.

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Hello, Pediatric Surgery family.

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I'm Emgody, a research fellow from Cincinnati Children's Hospital Medical Center.

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Our 11th annual update course in pediatric surgery was held past August.

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In this video series, we'll recap the sessions and share the main highlights with you.

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Today, our topic is Botox in Hirschsprung Disease.

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Joining the discussions are Dr. Stephen Lee,

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Kaitlyn Smith,

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and Julia Grabowski.

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Here's our clinical scenario.

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Five-month-old male with diagnosis of Hirschsprung Disease status post,

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Swenson, pull through an infancy at your institution.

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Patient now has had two episodes of Hirschsprung-associated enterocolitis responding to home irrigations.

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There's been no evidence of a strictural and rectal examination and contrast enema was done without any abnormality.

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Pathology review, then no evidence of a transition zone on pull through.

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What is your next step?

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You have to always do your due diligence with a patient who has had their pull through.

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Why are they getting recurrent enterocolitis?

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And one of the answers is you did a great operation and they have enterocolitis because they have a poorly functioning stincture,

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which every baby with Hirschsprung Disease has by nature of the disease.

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So first, you want to make sure that it's not a mechanical obstruction.

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And here, Dr. Smith is opening a discussion for using Botox in this patient.

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How do you do your Botox?

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In reading every paper that was ever written on Botox and the anus in the last couple of months,

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people are doing any amount of Botox in any aliquots in any number of locations, yes.

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So I am 100 units in one milliliter.

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I'm usually more like three, four in the dentate line.

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What about you?

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Yeah, I do 100 units, same one cc of saline, what's been commonly practiced,

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and then ultrasound plus or minus per your preference.

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What do you think caused it?

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I know, you know, no matter what operation we do for Hirschsprung, we always leave a ganglionic bowel.

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That bowel is at least the internal sphincter because we can't take that out.

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So I was wondering, do you think that that is great in the fertile grounds for enterocolitis?

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Or is it a mucosal disease? Is it immunologic?

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What are your thoughts?

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In order to maintain continence, you want to preserve the dentate line,

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and you have to leave a tiny bit of a ganglionic internal sphincter.

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And I think that babies outgrow it because their external sphincter is able to overcome their internal sphincter.

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And I think that takes time for their body to mature.

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But I don't know that we have pinpointed the actual path of physiology.

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The Hirschsprung's physiology sorts of sets up the colon to act like a pond, right?

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There's poor emptying and motility issues.

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And if you're not very diligent about clearing the colon,

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it just has the ability for the bacteria to overgrow and create illness in that setting.

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Basically, all of the things that prevent enterocolitis are working towards the opposite in these patients.

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And what we should do is to improve clears of stool from the colon and not allow for it to sit there stagnant.

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Something that's very interesting is we know that there's a significantly higher rate of enterocolitis in Hirschsprung disease after pull-through.

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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.

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So that has always been a little counterintuitive to me.

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And also, much higher rate of enterocolitis in children with TRIsone 21.

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We don't really have a good reason to explain that.

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And you can still get enterocolitis even if you're doing rectal irrigations at home.

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There are times when it's just not enough to empty that colon.

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There is discussion of whether or not when you make the diagnosis of total colon Hirschsprung at that time,

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should you do the colectomy because of the risk of enterocolitis.

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And I don't have a strong opinion on that.

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There are numerous studies and most of them are single institution, retrospective ones.

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The thing about Hirschsprung disease I think we all realize is that there's so much phenotypic variance.

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So in studying the use of Botox in post-pull-through enterocolitis, the data is not perfect.

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According to Dr. Smith, even prospective data is not going to be perfect because people are very different.

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In a surgeon's, you have different types of operations that you do, you have different levels of disease,

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you have different other comorbidities, syndromes, etc.

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Some of the data suggests that prophylactic Botox has not been shown to decrease the risk of enterocolitis.

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But has been shown to decrease the length of stay in patients who have been admitted for enterocolitis.

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Also has been shown to potentially decrease hospitalizations in patients who present with recurrent episodes of obstruction or enterocolitis.

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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

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and using Botox as part of that treatment strategy would be beneficial.

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To sum it up, Hirschsprung disease is characterized by a lack of nerve cells in the colon, which causes problems with bowel movements.

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Even after surgical procedures like the Swanson pull-through, patients might still experience recurrent enterocolitis,

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possibly because of a naturally defective sphincter.

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Botox has been considered as a potential treatment, often administered as 100 units in 1 ml of saline.

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The underlying condition of the disease can lead to excessive bacterial growth in the colon, emphasizing the need for regular clearing.

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While there is some evidence indicating Botox might not consistently prevent enterocolitis,

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it has shown potential in reducing both the length of hospital stays and recurrent episodes.

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Further research is needed to determine its precise utility.

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Thank you for watching this video.

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GlobalCastMD, along with Cincinnati Children's Hospital, sharing knowledge to improve child health around the globe.