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, we'll talk about "Botox in Hirschsprung Disease". Joining the discussion are Drs. Steven Lee, Caitlin Smith and Julia Grabowski.
Host: Em Gootee, MD
References:
https://pubmed.ncbi.nlm.nih.gov/21238663/
Patrus B, Nasr A, Langer JC, Gerstle JT. Intrasphincteric botulinum toxin decreases the rate of hospitalization for postoperative obstructive symptoms in children with Hirschsprung disease. J Pediatr Surg. 2011 Jan;46(1):184-7. doi: 10.1016/j.jpedsurg.2010.09.089. PMID: 21238663.
https://pubmed.ncbi.nlm.nih.gov/35690464/
Svetanoff WJ, Lim-Beutal IIP, Wood RJ, Levitt MA, Rentea RM. The utilization of botulinum toxin for Hirschsprung disease. Semin Pediatr Surg. 2022 Apr;31(2):151161. doi: 10.1016/j.sempedsurg.2022.151161. Epub 2022 Apr 8. PMID: 35690464.
https://pubmed.ncbi.nlm.nih.gov/33422904/
Rentea RM, Noel-MacDonnell JR, Bucher BT, Dorman MR, Lautz TB, Pruitt LCC, Oyetunji TA. Impact of Botulinum Toxin on Hirschsprung-Associated Enterocolitis After Primary Pull-Through. J Surg Res. 2021 May;261:95-104. doi: 10.1016/j.jss.2020.12.018. Epub 2021 Jan 7. PMID: 33422904.
https://pubmed.ncbi.nlm.nih.gov/37221126/
Encisco EM, Lim IIP, Velazco CS, Rosen NG, Garrison AP, Rymeski B, Frischer JS. Hirschsprung-Associated Enterocolitis at a Referral Institution: A Retrospective Review. J Pediatr Surg. 2023 Aug;58(8):1578-1581. doi: 10.1016/j.jpedsurg.2023.04.008. Epub 2023 Apr 20. PMID: 37221126.
https://pubmed.ncbi.nlm.nih.gov/33001257/
Svetanoff WJ, Dekonenko C, Osuchukwu O, Oyetunji TA, Aguayo P, Fraser JD, Juang D, Snyder CL, Hendrickson R, Peter SS, Rentea RM. Inpatient management of Hirschsprung's associated enterocolitis treatment: the benefits of standardized care. Pediatr Surg Int. 2020 Dec;36(12):1413-1421. doi: 10.1007/s00383-020-04747-4. Epub 2020 Oct 1. PMID: 33001257.
Type: video · 5 min · posted 2024-02-01 Canonical: https://library.globalcastmd.com/watch/update-course-rewind-botox-in-hirschsprung-disease-2023-7941 ## Chapters - [0:00](https://library.globalcastmd.com/watch/update-course-rewind-botox-in-hirschsprung-disease-2023-7941?t=0) Introduction and Case Presentation - [1:08](https://library.globalcastmd.com/watch/update-course-rewind-botox-in-hirschsprung-disease-2023-7941?t=68) Botox Technique and Indications - [2:00](https://library.globalcastmd.com/watch/update-course-rewind-botox-in-hirschsprung-disease-2023-7941?t=120) Pathophysiology of Post-Operative Enterocolitis - [3:36](https://library.globalcastmd.com/watch/update-course-rewind-botox-in-hirschsprung-disease-2023-7941?t=216) Evidence for Botox in HAEC - [4:47](https://library.globalcastmd.com/watch/update-course-rewind-botox-in-hirschsprung-disease-2023-7941?t=287) Summary and Conclusion ## Statements - "Every baby with Hirschsprung disease has a poorly functioning sphincter by nature of the disease" — Caitlin Smith (clinical) [1:14](https://library.globalcastmd.com/watch/update-course-rewind-botox-in-hirschsprung-disease-2023-7941?t=74) - "In order to maintain continence, you want to preserve the dentate line, and you have to leave a tiny bit of aganglionic internal sphincter" — Caitlin Smith (clinical) [2:17](https://library.globalcastmd.com/watch/update-course-rewind-botox-in-hirschsprung-disease-2023-7941?t=137) - "Babies outgrow enterocolitis because their external sphincter is able to overcome their internal sphincter, and that takes time for their body to mature" — Caitlin Smith (opinion) [2:23](https://library.globalcastmd.com/watch/update-course-rewind-botox-in-hirschsprung-disease-2023-7941?t=143) - "The Hirschsprung physiology sets up the colon to act like a pond with poor emptying and motility issues, allowing bacteria to overgrow if the colon is not cleared diligently" — Steven Lee or Doctor Julia Grabowski (clinical) [2:37](https://library.globalcastmd.com/watch/update-course-rewind-botox-in-hirschsprung-disease-2023-7941?t=157) - "There is a significantly higher rate of enterocolitis in Hirschsprung disease after pull-through when you take out the entire colon compared to babies with shorter segment disease" — Caitlin Smith (epidemiological) [3:06](https://library.globalcastmd.com/watch/update-course-rewind-botox-in-hirschsprung-disease-2023-7941?t=186) - "There is a much higher rate of enterocolitis in children with trisomy 21" — Caitlin Smith (epidemiological) [3:21](https://library.globalcastmd.com/watch/update-course-rewind-botox-in-hirschsprung-disease-2023-7941?t=201) - "You can still get enterocolitis even if you're doing rectal irrigations at home; there are times when it's just not enough to empty that colon" — I'm Goddy (clinical) [3:25](https://library.globalcastmd.com/watch/update-course-rewind-botox-in-hirschsprung-disease-2023-7941?t=205) - "There is discussion of whether when you make the diagnosis of total colon Hirschsprung, you should do the colectomy at that time because of the risk of enterocolitis" — Caitlin Smith (opinion) [3:35](https://library.globalcastmd.com/watch/update-course-rewind-botox-in-hirschsprung-disease-2023-7941?t=215) - "There is so much phenotypic variance in Hirschsprung disease that studying the use of Botox in post-pull-through enterocolitis yields imperfect data" — Caitlin Smith (opinion) [3:49](https://library.globalcastmd.com/watch/update-course-rewind-botox-in-hirschsprung-disease-2023-7941?t=229) - "Prophylactic Botox has not been shown to decrease the risk of enterocolitis" — Caitlin Smith (clinical) [4:14](https://library.globalcastmd.com/watch/update-course-rewind-botox-in-hirschsprung-disease-2023-7941?t=254) - "Botox has been shown to decrease the length of stay in patients who have been admitted for enterocolitis" — Caitlin Smith (clinical) [4:14](https://library.globalcastmd.com/watch/update-course-rewind-botox-in-hirschsprung-disease-2023-7941?t=254) - "Botox has been shown to potentially decrease hospitalizations in patients who present with recurrent episodes of obstruction or enterocolitis" — Caitlin Smith (clinical) [4:26](https://library.globalcastmd.com/watch/update-course-rewind-botox-in-hirschsprung-disease-2023-7941?t=266) - "Maybe not everyone needs Botox at the time of pull-through, but there is a subset of patients who have predisposition to enterocolitis for whom Botox as part of treatment strategy would be beneficial" — Caitlin Smith (opinion) [4:34](https://library.globalcastmd.com/watch/update-course-rewind-botox-in-hirschsprung-disease-2023-7941?t=274) - "Botox is often administered as 100 units in 1 mL of saline" — Caitlin Smith (clinical) [1:45](https://library.globalcastmd.com/watch/update-course-rewind-botox-in-hirschsprung-disease-2023-7941?t=105) - "Botox injection technique typically involves 3-4 injections in the dentate line" — Caitlin Smith (clinical) [1:48](https://library.globalcastmd.com/watch/update-course-rewind-botox-in-hirschsprung-disease-2023-7941?t=108) - "People are doing any amount of Botox in any aliquots in any number of locations (technique varies widely in the literature)" — Caitlin Smith (clinical) [1:33](https://library.globalcastmd.com/watch/update-course-rewind-botox-in-hirschsprung-disease-2023-7941?t=93) ## Transcript Global Cat MD along with Cincinnati Children's Hospital, sharing knowledge to improve child health around the globe. Hello pediatric surgery family. I'm I'm Goddy, 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 and Hirschsprung disease. Joining the discussions are Doctor Steven Lee. Caitlin Smith And Julia Grabowski. Here's our clinical scenario. 5 month old male with diagnosis of Hirschprung disease status post Swenson pull through an infancy at your institution. Patient now has had 2 episodes of Hirschprung associated enterocolitis responding to home irrigations. There's been no evidence of a stricture on rectal examination and contrast enema but was done without any abnormality. Pathology reviewed and 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 aloquats in any number of locations. Yes, so I am 100 units in 1 mL. I'm usually more like 34 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. What do you think causes it? I know, you know, no matter what operation we do for Hirschprung's, 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 intercolitis, 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 ganglion. 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 Hirschberg's 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 clears 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 Hirschprung 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 back. Bacteria, so that has always been a little counterintuitive to me. And also much higher rate of enterocolitis in children with trisomy 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's just not enough to empty that colon. There is discussion of whether or not when you're, when you make the diagnosis of total colon Hirschprung 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 Hirschhorn disease, I think we all realize is that there's so much phenotypic variance. So in studying the use of Botox in postpo through enterocolitis, the data is not perfect. According to Doctor 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. Suggest that prophylactic Botox has not been shown to decrease the risk of enterocolitis, but 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, often administered as 100 units and 1 mL 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 stays 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 Stay Current MD YouTube channel. Follow our social media channels and download the Stay Current MD app for tons of content in pediatric surgery. Global Cat MD along with Cincinnati Children's Hospital, sharing knowledge to improve child health around the globe. --- Not medical advice · citation policy: https://library.globalcastmd.com/ai