Who's speaking
- Speaker 1 — host
- I'm Goddy — host
- Speaker 3 — guest
- Doctor Caitlin Smith — guest
- Speaker 5 — guest
- Speaker 6 — guest
Chapters
- 0:00Introduction and Case Presentation — Introduction to the update course rewind series and presentation of a clinical scenario: 5-month-old with Hirschsprung disease, status post Swenson pull-through, now with recurrent HAEC despite normal anatomy on workup.
- 1:08Botox Technique and Indications — Discussion of the rationale for Botox in post-pull-through patients with recurrent HAEC when mechanical obstruction has been ruled out. Review of Botox injection technique: 100 units in 1 mL saline, 3-4 injections at the dentate line, with or without ultrasound guidance.
- 2:00Pathophysiology of Post-Operative Enterocolitis — Exploration of why enterocolitis persists after pull-through: residual aganglionic internal sphincter, impaired colonic motility creating bacterial overgrowth, and counterintuitive findings that total colonic Hirschsprung has higher HAEC rates than short-segment disease. Higher rates also seen in trisomy 21.
- 3:36Evidence for Botox in HAEC — Review of the literature on Botox for post-pull-through enterocolitis. Prophylactic Botox has not been shown to decrease enterocolitis incidence but may reduce hospital length of stay and decrease recurrent admissions in select high-risk patients.
- 4:47Summary and Conclusion — Recap of key points: Hirschsprung pathophysiology, Botox technique, and evidence suggesting benefit in reducing hospital stays and recurrent episodes rather than preventing initial enterocolitis. Call for further research.
Key claims
- 1:14Every baby with Hirschsprung disease has a poorly functioning sphincter by nature of the disease — Doctor Caitlin Smith
- 2:17In order to maintain continence, you want to preserve the dentate line, and you have to leave a tiny bit of aganglionic internal sphincter — Doctor Caitlin Smith
- 2:23Babies outgrow enterocolitis because their external sphincter is able to overcome their internal sphincter, and that takes time for their body to mature — Doctor Caitlin Smith
- 2:37The 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 — Speaker 5
- 3:06There 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 — Doctor Caitlin Smith
- 3:21There is a much higher rate of enterocolitis in children with trisomy 21 — Doctor Caitlin Smith
- 3:25You 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
- 3:35There 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 — Doctor Caitlin Smith
- 3:49There is so much phenotypic variance in Hirschsprung disease that studying the use of Botox in post-pull-through enterocolitis yields imperfect data — Doctor Caitlin Smith
- 4:14Prophylactic Botox has not been shown to decrease the risk of enterocolitis — Doctor Caitlin Smith
- 4:14Botox has been shown to decrease the length of stay in patients who have been admitted for enterocolitis — Doctor Caitlin Smith
- 4:26Botox has been shown to potentially decrease hospitalizations in patients who present with recurrent episodes of obstruction or enterocolitis — Doctor Caitlin Smith
- 4:34Maybe 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 — Doctor Caitlin Smith
- 1:45Botox is often administered as 100 units in 1 mL of saline — Doctor Caitlin Smith
- 1:48Botox injection technique typically involves 3-4 injections in the dentate line — Doctor Caitlin Smith
- 1:33People are doing any amount of Botox in any aliquots in any number of locations (technique varies widely in the literature) — Doctor Caitlin Smith
Cases discussed
- 0:455-month-old male with Hirschsprung disease, status post Swenson pull-through in infancy, now with 2 episodes of HAEC responding to home irrigations
Open questions
- What is the precise pathophysiology of Hirschsprung-associated enterocolitis after pull-through?
- Why does total colonic Hirschsprung have a higher rate of enterocolitis than short-segment disease?
- Why do children with trisomy 21 have higher rates of enterocolitis?
- Should colectomy be performed at the time of diagnosis in total colonic Hirschsprung to prevent enterocolitis?
- Which subset of patients would benefit most from Botox as part of their treatment strategy?
- What is the optimal Botox dosing, injection technique, and timing (prophylactic vs. therapeutic)?
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