Update Course Rewind: Botox in Hirschsprung Disease 2023
With Dr. Caitlin Smith & Dr. Steven Lee or Julia Grabowski · hosted by Dr. I'm Goddy · StayCurrentMD
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Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
Every baby with Hirschsprung disease has a poorly functioning sphincter by nature of the disease.
Dr. Smith uses 100 units in 1 mL, usually 3-4 injections in the dentate line.
Common practice is 100 units in 1 cc of saline, with ultrasound guidance optional based on preference.
No matter what operation is done for Hirschsprung's, ganglionic bowel is always left, at least the internal sphincter.
To maintain continence, the dentate line must be preserved, requiring a tiny bit of aganglionic internal sphincter to be left.
Babies outgrow enterocolitis because their external sphincter becomes able to overcome their internal sphincter as their body matures.
The actual pathophysiology of Hirschsprung-associated enterocolitis has not been pinpointed.
Hirschsprung physiology causes the colon to act like a pond with poor emptying and motility issues, allowing bacterial overgrowth if the colon is not cleared diligently.
There is a significantly higher rate of enterocolitis in total colonic Hirschsprung disease after pull-through compared to shorter segment disease.
Children with trisomy 21 have a much higher rate of enterocolitis in Hirschsprung disease.
There is discussion about whether colectomy should be performed at the time of total colon Hirschsprung diagnosis because of enterocolitis risk.
There is significant phenotypic variance in Hirschsprung disease, making study data imperfect.
Not everyone may need Botox at the time of pull-through, but there may be a subset of patients with predisposition to enterocolitis who would benefit from Botox as part of treatment strategy.
Botox dosing varies widely in the literature: any amount of Botox in any aliquots in any number of locations.
Enterocolitis can occur even when patients are doing rectal irrigations at home; sometimes it is not enough to empty the colon.
Most studies on Botox in Hirschsprung disease are single institution retrospective studies.
According to Dr. Smith, even prospective data will not be perfect because of patient variability, different surgical techniques, different disease levels, and different comorbidities and syndromes.
Some data suggest prophylactic Botox has not been shown to decrease the risk of enterocolitis.
Botox has been shown to decrease the length of stay in patients admitted for enterocolitis.
Botox has been shown to potentially decrease hospitalizations in patients with recurrent episodes of obstruction or enterocolitis.