Update Course Rewind: Botox in Hirschsprung Disease 2023

Every baby with Hirschsprung disease has a poorly functioning sphincter by nature of the disease

People are doing any amount of Botox in any aliquots in any number of locations (technique varies widely in the literature)

Botox is often administered as 100 units in 1 mL of saline

Botox injection technique typically involves 3-4 injections in the dentate line

In order to maintain continence, you want to preserve the dentate line, and you have to leave a tiny bit of aganglionic internal sphincter

Babies outgrow enterocolitis because their external sphincter is able to overcome their internal sphincter, and that takes time for their body to mature

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

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

There is a much higher rate of enterocolitis in children with trisomy 21

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 when you make the diagnosis of total colon Hirschsprung, you should do the colectomy at that time because of the risk of enterocolitis

There is so much phenotypic variance in Hirschsprung disease that studying the use of Botox in post-pull-through enterocolitis yields imperfect data

Prophylactic Botox has not been shown to decrease the risk of enterocolitis

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

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

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