Hirschsprung Disease Audience Q&A with Dr. Marc Levitt

Published: 2017-04-14

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Inside this episode

Kai, the Library's AI content creator, listened to this episode and mapped who's speaking, the chapters, key claims, and cases. Every item links to the exact moment in the recording.

AI-enriched

Who's speaking

Speaker 1 — host
Todd Ponsky — host
Scott Ingham — guest
Marc Levitt — guest
Speaker 5 — host

Chapters

0:00Introduction and Question Setup — Introduction to the Q&A format with Dr. Scott Ingham from Fargo, North Dakota posing questions about Botox use in Hirschsprung disease to Dr. Marc Levitt.
1:34Botox Mechanism and Primary Indications — Dr. Levitt explains the mechanism of Botox in Hirschsprung patients, its role in overcoming high anal sphincter tone in infants, and appropriate timing for use after pull-through surgery.
3:59Patient Selection and Anatomic Workup — Discussion of the borderline age group (6 months to 1.5 years), importance of ruling out anatomic and pathologic problems including retained cuff and transition zone, and detailed injection technique.
7:50Technical Variations in Botox Administration — Comparison of injection volumes and concentrations, with Dr. Levitt describing his approach of 100 units in 10cc versus more concentrated alternatives.
9:09Management of Dilated Pull-Through Segments — Discussion of redo pull-through for dilated segments with normal ganglion cells, decision-making around tapering, and importance of diversion and removing distal obstruction.
12:01Closing — Hosts thank participants and provide podcast subscription information.

Key claims

1:57Botox paralyzes skeletal muscle theoretically but clearly has some impact on smooth muscle — Marc Levitt
2:06Babies have very tight anal sphincter with or without Hirschsprung's disease — Marc Levitt
2:14In normal babies who hold stool in successfully they get constipated, in Hirschsprung babies they get enterocolitis — Marc Levitt
2:26After a perfectly done operation preserving 1 centimeter of anal canal, you can have high tone in a baby that doesn't know how to relax — Marc Levitt
3:15Botox is valuable if a baby is coming back with enterocolitis episodes after ruling out anatomic or pathologic problems — Marc Levitt
3:27In the early period up to one year of age, you can have bad behavior by the patient even with a perfectly done pull-through — Marc Levitt
3:36After a year or so of age, there may be an anatomic or pathologic problem and Botox is not very valuable because you have to figure out what the problem is — Marc Levitt
4:47A retained cuff that is too big, not split completely, or rolled up is particularly offensive to the ability of the pull-through to empty — Marc Levitt
5:09Patients with retained cuff will get better temporarily with Botox but will continue to fall off the wagon every 2 or 3 months — Marc Levitt
5:35Nerves greater than 40 microns indicate a transition zone pull-through — Marc Levitt
5:43Many pathologists are not measuring the nerves, and pediatric surgeons should demand that their pathologists do that — Marc Levitt
5:58If anatomic and pathologic issues are completely ruled out, Botox maybe gets done once, maybe a second time, and then you're done — Marc Levitt
6:14If a patient over 1 year of age keeps misbehaving, there is likely an anatomic or pathologic problem — Marc Levitt
6:24Dr. Levitt has never met a patient with Hirschsprung disease that is anatomically perfect (no stricture, no cuff, no duhamel pouch causing trouble, no twist) with normal ganglion cells and nerves less than 40 microns that does not spontaneously empty, except rare patients under a year of age who have not learned to relax their anal canal — Marc Levitt
7:00If an older child is not emptying spontaneously, there is an anatomic or pathologic problem that has not yet been identified — Marc Levitt
7:07Botox injection technique: 100 units in 10cc saline, 2.5cc submucosal into each quadrant right into the muscle surrounding the anal canal — Marc Levitt
7:30Dr. Levitt will never do an internal sphincterotomy because it is permanent Botox and could cause permanent incontinence — Marc Levitt
7:45Patients will eventually figure out how to relax, so there is no need to make sphincterotomy a permanent anatomic solution — Marc Levitt
8:08Most people use between 60 units and 100 units of Botox for Hirschsprung patients — Scott Ingham
8:47Some practitioners use much less volume (1mL total, 0.25mL per quadrant) for Botox injection — Scott Ingham
9:35A dilated pull-through segment may be secondary to noncompliance with dilations or bowel regimen, or the surgeon may not have taken out enough bowel at the original surgery — Marc Levitt
10:01For patients with a dilated segment and no other anatomic abnormality who continue to misbehave, redo surgery to remove the dilated segment can be offered — Marc Levitt
10:12Almost always there is something causing the dilation, either a cuff or a transition zone segment of bowel — Marc Levitt
11:24If a tapered segment is created during redo, it will be a fairly dysmotile segment of bowel for many, many months — Marc Levitt
11:31If tapering or redo is necessary, the patient should always be diverted with an ileostomy — Marc Levitt
11:40Most likely you can remove the dilated segment and bring the healthy segment down without tapering — Marc Levitt
11:46You must make sure any distal obstruction is removed during redo surgery — Marc Levitt

Points of disagreement

7:50Optimal volume for Botox injection
- Marc Levitt: Uses 100 units in 10cc saline (2.5cc per quadrant) for good spread without losing concentration
- Scott Ingham: Notes that some practitioners use much less volume (1mL total, 0.25mL per quadrant) and questions whether volume affects success rate

Open questions

Does injection volume (10cc vs 1cc total) affect Botox success rate in Hirschsprung patients?
Would empiric Botox at the time of primary pull-through decrease the incidence of enterocolitis? (prospective trial being designed)
What is the optimal interval for repeat Botox injections in the 6-18 month age group showing temporary improvement?

This episode was analyzed and enriched by Kai, the Library's AI content creator. Every item links to the moment it comes from — click a timestamp to listen in context.

Written for:

Botox Injection Volume in Hirschsprung Disease: Does Dilution Matter?

The points where the speakers disagreed, with each position presented side by side. Written by Kai from the episode transcript and reviewed before publishing.

For specialists · Points of disagreement · AI-written, human-reviewed

The Question

When injecting botulinum toxin for high anal tone following Hirschsprung pull-through, does the volume of diluent affect clinical outcomes? The standard dose range is established—most practitioners use 60 to 100 units 8:08—but the concentration varies widely in practice.

The High-Volume Approach

Marc Levitt uses 100 units diluted in 10 mL saline, delivering 2.5 mL submucosally into each quadrant of the anal canal musculature 7:07. This technique aims to distribute the toxin throughout the sphincter complex while maintaining sufficient concentration at each injection site 7:07. The volume allows coverage of the muscle surrounding the anal canal without requiring multiple needle passes per quadrant 7:07.

The Low-Volume Alternative

Scott Ingham notes that some practitioners use substantially less diluent—1 mL total volume, with 0.25 mL injected per quadrant 8:47. This represents a tenfold difference in concentration compared to Levitt's technique 7:07 8:47. The appeal of minimal volume is theoretical: higher local concentration at the injection site, less tissue distension, and potentially more precise targeting of the internal sphincter.

Where They Converge

Both approaches share the same anatomic target—submucosal injection into the anal canal musculature in four quadrants 7:07. Neither advocate for intramuscular injection into the external sphincter, which would risk permanent damage. The total dose range overlaps substantially, with most practitioners staying between 60 and 100 units regardless of dilution 8:08. Both techniques avoid internal sphincterotomy, which Levitt describes as "permanent Botox" with risk of permanent incontinence 7:30.

More fundamentally, both agree that Botox addresses only one specific problem: high anal tone in an anatomically and pathologically normal pull-through 3:15. If a retained cuff, stricture, transition zone segment, or twist is present, Botox provides only temporary relief 3:15 5:09. The patient will experience temporary improvement but symptoms recur 5:09. In children over one year of age who continue to have obstructive symptoms despite Botox, both would pursue anatomic or pathologic explanations rather than repeat injections indefinitely 3:36 6:14.

What Would Resolve It

The discussants acknowledge they do not know whether volume affects success rates 8:47. One of the discussants states that good results have been achieved with higher volumes, but smaller volumes have not been tried 8:47. The other frames it as an open question about whether volume influences success rate 8:47.

A comparative trial would need to control for the variables that actually determine outcome in these patients 3:15 5:09 6:14. Success depends on ruling out anatomic problems—no cuff, no stricture, no Duhamel pouch causing obstruction, no twist 6:24—and confirming normal pathology with ganglion cells present and nerve trunks measuring less than 40 microns 5:35. Levitt emphasizes that pathologists must measure nerve diameter, not simply report ganglion cells as present, to avoid missing a transition zone pull-through 5:43. In a properly selected cohort—infants under one year with anatomically perfect pull-throughs and normal pathology who have not yet learned to relax their anal sphincter 3:36 7:42—the volume question could be tested cleanly.

The trial would also need to distinguish temporary from durable response 5:09. If high-volume injection produces resolution after one or two treatments while low-volume requires repeated injections, that would favour dilution. If both achieve the same endpoint—spontaneous emptying without further intervention—then concentration is irrelevant and practitioners can choose based on technical preference.

Until such data exist, the choice remains empiric 8:47. Both techniques report success in appropriately selected patients 3:15 3:36, and both fail when anatomic or pathologic problems are present but unrecognized 5:09 6:14. The disagreement is real but narrow, and the answer matters less than the diagnostic work that precedes injection 3:15 5:09 5:35 5:43 6:14 6:24.

Takeaways from this story

Botox volume varies tenfold in practice (1 mL vs 10 mL for 100 units), but no data compare outcomes between high and low dilution.
Repeated Botox need beyond 1-2 injections signals missed anatomy (cuff, stricture, twist) or pathology (transition zone, nerves >40 microns).
Demand nerve trunk measurement from pathology—ganglion cells alone don't exclude transition zone pull-through if nerves exceed 40 microns.
Internal sphincterotomy is permanent Botox with permanent incontinence risk; infants eventually learn sphincter relaxation without it.

When to Use Botox After Hirschsprung Pull-Through — and When to Look Harder

The episode's teaching points arranged as a structured lesson, building from the basics up to the finer points. Written by Kai from the episode transcript and reviewed before publishing.

For the care team · Teaching arc · AI-written, human-reviewed

The Mechanism and the Misconception

Botox in Hirschsprung management targets skeletal muscle in theory, but clearly affects smooth muscle as well 1:57. The fundamental insight is that all infants — with or without Hirschsprung disease — have high anal sphincter tone 2:06. In a normal infant who holds stool, the result is constipation; in a Hirschsprung infant, the result is enterocolitis 2:14. Even after a technically perfect pull-through preserving one centimeter of anal canal, you can encounter high tone in an infant who has not yet learned to relax 2:26. This is behavioral, not anatomic, and it resolves with time.

The Age-Based Decision Framework

Up to one year of age, bad behavior can occur despite a perfectly executed pull-through 3:27. In this window, Botox is valuable if the infant returns with enterocolitis episodes — provided you have ruled out anatomic and pathologic problems first 3:15. After one year of age, the calculus changes entirely. Persistent symptoms now suggest an anatomic or pathologic problem, and Botox is not valuable because you must identify what that problem is 3:36. If an older child is not emptying spontaneously, there is an anatomic or pathologic problem that has not yet been identified 7:00.

One of the discussants' position is unequivocal: patients with Hirschsprung disease who are anatomically perfect — with no stricture, no cuff, no Duhamel pouch causing trouble, no twist — with normal ganglion cells and appropriately sized nerves will spontaneously empty, except for the rare patient under a year of age who simply has not learned how to relax their anal canal 6:24.

The Systematic Workup Before Botox

Before attributing symptoms to sphincter dysfunction, complete the following: digital rectal exam to rule out retained cuff or stricture; biopsy to confirm normal ganglion cells; measurement of nerve size to exclude transition zone (nerves greater than 40 microns indicate transition zone pull-through) 5:35; and imaging or examination to rule out twist. A retained cuff that is too large, incompletely divided, or rolled up is particularly detrimental to pull-through emptying 4:47. Patients with retained cuff will improve temporarily with Botox but will relapse every two to three months 5:09. Many pathologists do not measure nerve size, and pediatric surgeons should demand that they do so to avoid performing a technically adequate pull-through in the transition zone 5:43.

The Injection Technique and the Permanent Alternative to Avoid

If anatomic and pathologic issues are completely ruled out, Botox may be administered once, possibly twice, and then you are done 5:58. The technique: 100 units in saline, divided into each quadrant directly into the muscle surrounding the anal canal 7:07. Internal sphincterotomy is never indicated — it is permanent Botox and risks permanent incontinence 7:30. Patients will eventually learn to relax, so there is no justification for a permanent anatomic solution 7:45.

The Dilated Segment Problem

A dilated pull-through segment may result from noncompliance with dilations or bowel regimen, or the surgeon may not have resected enough bowel at the original operation 9:35. If a patient has a dilated segment with no other anatomic abnormality and continues to have symptoms, redo surgery to remove the dilated segment can be offered 10:01. However, almost always there is something causing the dilation — either a retained cuff or a transition zone segment 10:12. If tapering is necessary during redo, the tapered segment will be dysmotile for many months 11:24, and the patient should always be diverted with an ileostomy 11:31. Most likely you can remove the dilated segment and bring healthy bowel down without tapering 11:40, but you must ensure that any distal obstruction — particularly a retained cuff — is removed 11:46.

The central teaching is this: if you are Botoxing a Hirschsprung patient repeatedly after one year of age, you have not yet found the anatomic or pathologic problem. The workup is incomplete, not the patient's sphincter.

Takeaways from this story

After age one, repeated Botox need signals unidentified anatomic or pathologic problem — not persistent sphincter dysfunction.
Demand nerve measurement on biopsies: nerves >40 microns mean transition zone pull-through despite technically adequate surgery.
Retained cuff causes temporary Botox response with relapse every 2-3 months — find it on digital exam before repeated injections.
Never perform internal sphincterotomy — it risks permanent incontinence for a problem that resolves as infants learn to relax.

Topic overview

A Q&A session addressing Botox injection use in Hirschsprung disease management, particularly for patients with persistent symptoms after pull-through surgery. Dr. Marc Levitt discusses patient selection criteria, emphasizing that Botox is most valuable in infants under one year with high anal sphincter tone and no anatomic or pathologic abnormalities. He describes his injection technique (100 units in 10cc saline, 2.5cc per quadrant submucosally) and stresses the importance of ruling out retained cuff, stricture, transition zone pull-through (nerves >40 microns), or twist before attributing symptoms to behavioral sphincter dysfunction. The discussion also covers management of dilated pull-through segments with normal ganglion cells.

Key takeaways

Botox is most valuable in infants <1yr with high sphincter tone after ruling out anatomic/pathologic causes like retained cuff or stricture. (3:15)
Nerves >40 microns indicate transition zone pull-through; demand pathologists measure nerve caliber in all Hirschsprung specimens. (5:35)
Inject 100 units Botox in 10cc saline submucosally (2.5cc per quadrant). Avoid permanent sphincterotomy—patients learn to relax over time. (7:07)
Persistent symptoms in children >1yr almost always indicate unidentified anatomic/pathologic problem, not behavioral sphincter dysfunction. (6:14)
For dilated pull-through with normal ganglion cells, identify cause (cuff/transition zone). If redo needed, always divert with ileostomy. (10:01)

Keywords

Hirschsprung disease enterocolitis constipation high anal sphincter tone stricture transition zone pull-through pull-through surgery Botox injection internal sphincterotomy rectal biopsy examination under anesthesia contrast study redo pull-through tapering ileostomy diversion

Hashtags

#HirschsprungDisease #PediatricSurgery #BotoxTherapy #Enterocolitis

Transcript

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Stay Current is an audio publication designed to keep healthcare professionals up to date with standards of care and new emerging ideas. These podcasts are designed to keep healthcare professionals current while on their commute. Stay Current is created and edited by Todd Ponsky and Nicholas Bruns in partnership with Globalcast MD and is recorded and produced at Akron Children's Hospital in Akron, Ohio. Now it's time for viewer mailbag, and today we have a question from Doctor Scott Ingham for Doctor Mark Levitt regarding his podcast on Hirschprung's disease. Uh, Scott, how are you doing today? Very good. Thanks for, for, uh, calling in. Why don't you tell us a little bit about yourself and then go ahead and, um, ask your, uh, question or give your comments. I'm a pediatric surgeon up here in Fargo, North Dakota with the Stanford Health System, and I appreciate Dr. Levitt's previous couple of GlobalC events with Hirschprung's disease, but there were A couple of areas I think all of us still struggle with that weren't covered in those separate sessions, and I wanted to pose the concern about the algorithm on the use of Botox injections and whether Dr. Levitt, you have certain patients that seem to fit that. Is there a technique difference, a dosage, and some interval concerns that you might want to use. I, I think um that's a great question, and you're right, we didn't um delve deeply into the use of Botox. My, my understanding, frankly, of what actually we're doing with Botox is definitely. Changed in the last couple of years as I've started to collaborate more strongly with GI, and the truth of the matter is I think it has a very valuable role in Hirschberg's disease, but you have to understand what you're trying to do. Botox is paralyzing theoretically the skeletal muscle, but it clearly has some impact on the smooth muscle. And what I believe happens in babies is they have a very tight anal sphincter. No matter what, with or without Hirschprung's disease, and if they hold it in successfully, then in a normal baby they just get constipated, and then the Hirschprung's baby they get enterocolitis. So even after a perfectly done operation, you can have high tone, and by definition, if you do a good job with the surgery and you don't hurt the sphincters and you preserve the 10 or 1 centimeter of the anal canal like you're supposed to. You're going to get some high tone in a baby that doesn't know how to relax. So I think that's the ideal circumstance in the primary situation to do Botox, essentially to overcome their instinct of holding in the stool. Um, so I do use that, and it's for some people talk about actually using it empirically when they've done the primary pull through. I haven't started doing that, but we are designing a prospective trial to test that and see if that would decrease the incidence of enterocolitis. But it's certainly valuable if you have a baby that's coming back with enterocolitis episodes. Um, of course, as we talked about during the podcast, we have to make sure there's no anatomic or pathologic problem with that pull-through. But in the early period, I mean up to one year of age, you can have this bad behavior by the patient, even if it's the perfectly done pull-through. Um, after a year or so, I'm very conscious of the fact that there may be an anatomic or pathologic problem. And those are the patients that I really don't think Botox is very valuable because you have to figure out what the anatomy or pathology problem is. And if you give Botox to those patients, they It might get better temporarily, but you're not giving them a permanent fix. Well, I think, you know, we still struggle with some of those patients that respond to it for that 3 or 4 months that are in that borderline age group that, you know, 6 months to a year and a half, and I guess the question is, is you're seeing an improvement that then goes away, and you've got ganglion cells, or at least what appear to be normal ganglion cells on a rebiopsy if you're concerned about a recurrent Hirschsprung's. And I guess I'm, I'm stuck in that middle group, you know, what is, you know, like you said, it's the anatomical workup, you know, when you see some improvement, you don't have obstruction, you've got ganglion cells, and, um, you know, at what point do you stop doing, you know, the injections, even though they've been helping, and they're over that age group. Well, I think one of the things that I've seen um that has been, is particularly offensive to the ability of the pull through to empty is a retained cuff. And if the cuff is too big or was not split completely or rolled up, You can Botox that. You don't really know that you're actually doing that, but you're actually Botoxing the cuffs, and they will get better temporarily, and they will continue to fall off the wagon every 2 or 3 months. So, if you do a digital rectal exam and do not feel the cuff and do not feel a stricture, And you have biopsied and showed that there are normal ganglion cells, and you have measured the nerves, and that's a really important detail. You can't have nerves that are greater than 40 microns because that is a um a transition zone pull through. And many pathologists are not measuring the nerves, and I want to encourage that all the pediatric surgeons demand that their pathologists do that so that they don't put themselves in a situation where they're doing a beautiful pull-through, but it's the transition zone valve. So if I have a patient that has completely ruled out anatomic and pathologic issues. Including no twist, etc. then I would do Botox. In my experience, that maybe gets done once, maybe a second time, and then you're done. If you're dealing with a patient that's over 1 year of age, And they keep misbehaving. I bet there's an anatomic or pathologic problem. I have yet to meet a patient with Hirschberg's disease, and people think I'm crazy when I say this, but I've yet to meet a patient with Hirschberg's disease that is anatomically perfect. Meaning no stricture, no cuff, no duhamel pouch that's causing trouble, no twist with normal ganglion cells and nerves less than 40 microns. I've never met such a patient that does not spontaneously empty, except the rare patient under a year of age who simply has not learned how to relax their anal canal and allow their poop to exit. So if you're having that happen in an older kid, there is an anatomic or pathologic problem that has just yet not been identified. The actual technique I put in submucosal with a very small gauge needle. I take 100 units of Botox and put it in 10 cc's of saline and put 2.5 cc's submucosal right into the muscle that surrounds the anal canal. And I will never do, ever do. I've never done and will never do an internal sphincterotomy because I'm essentially that's permanent Botox, and my worry is that it could cause permanent incontinence because you shouldn't have to keep Botoxing. They'll eventually figure it out, so why make that a permanent anatomic solution. Now your technique, um, is certainly different than Langer's and, you know, that's been described where he certainly would use a much smaller volume. Is there something that is positive about using that large of a volume? You mean the amount of Botox or the amount of fluid? Well, you're using 100 units, which is not uncommon. Most people do between 60 units and 100 units. And, and are you going 4 different quadrants, and you said splitting up that 1010 mL. Is that correct? Yeah, I put 100 units in 10 cc's and I put 2.5 ccs in each quadrant. This seems like a good amount of fluid to inject. OK. Because that's certainly there, there are people that would go with a lot less volume, and I don't know whether that has a clinical difference. I honestly don't know. You mean that it's more concentrated? I, I don't know. You could, you could certainly use less volume. It doesn't liter of fluid, uh, 1 mL of fluid, and injected 0.25 into each quadrant, and I don't know if the volume has something to do with success rate. I don't know. I don't know. I, I want good spread, but I don't want it to be, to lose its concentration, so. And I've had good results with that, but I've never tried a tiny volume like you're describing. I'm not sure. Uh, that actually leads into my second question that deals with the enlarged pull-through segment that does have normal ganglion cells, and let's say they have normal nerve size. You know, how do we approach those patients because they may have been noncompliant with dilations, they may have been noncompliant with the bowel regimen, and subsequently now they have dilated their pull-through segment. Well, I would say that that is an additional soft indication. Where the bowel segment is dilated. And I'm not sure if that's secondary to all the things you just described, or at the original surgery, the surgeon simply didn't take out enough. They got the good ganglion cells and normal nerves, but they didn't go a little bit higher to get rid of that dilated segment. There are definitely patients that have a dilated segment with no other anatomic abnormality, and they continue to misbehave. And for those patients, I have offered a redo to remove that dilated segment. That's exceedingly rare. Almost always there is something causing the dilation, and that something is either a cuff or a transition zone segment of bowel. Um, but if all things are ruled out, having, and you're still a patient having trouble and there's a dilated segment, then that dilated segment could, could be the explanation. Um, and you're quite right, there are patients that migrate into the systems that have been operated on by others, and you have to make an effort of doing a contrast study, doing examination under anesthesia and a rectal biopsy. And that's a fairly, fairly straightforward workup. And with that, you're going to find a lot of people that are anatomically or pathologically not right, and that's why their poulters are not emptying. Now, have you ever, have you ever tried to save a redo pull through and, and, and benefit from a taper, or do you think you're stuck with the straight pull redo pull through? Well, what I will do is I will redo the pull through. Transanally, saving the anal canal, deliver that up into the abdomen, and then make a decision on whether it needs to be tapered or not. Um, if you do have to taper it, that's going to be a fairly dysmotile segment of bowel for many, many months. In addition, if I ever have to taper or redo, I will always divert that patient with an ileostomy. Um, but these are rare circumstances, what you're describing. Most like, most likely you can remove the dilated segment and bring the healthy segment, uh, down. And most importantly, you have to make sure that you've removed any distal obstruction. I've seen patients that, that have been redone. And the suave cuff was left alone and then I had to redo the redo to remove the cuff, and then the patient got better. They're always tough patients, so. They are, they really are, and so I, I wanna thank both of you, uh, for taking the time to, to hit on these questions because I, I assume that a lot of people are wondering the same thing and, uh, uh, I hope you guys have a good rest of your day. We hope you enjoyed this episode of Stay Current in Pediatric Surgery. You can listen to Stay Current in Pediatric Surgery by subscribing on the iTunes Store or by downloading the Globalcast MD podcast app. Please send questions or comments to us at staycurrent podcast@gmail.com. We'll see you next time.

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