Update Course 2023 - Updates in Pediatric Achalasia Management

Thank you everybody. Um, we're now gonna start our next session and to introduce and moderate that session, we have Doctor Witt Holcomb and Doctor Mack Harmon. So, uh, good morning to those in the, uh, Western Hemisphere and perhaps good evening, uh, to those of our colleagues, um, around the world and the eastern part of the world. We're very, uh, pleased to be able to, um, try to update, uh, everyone on the topic of Aallaia. Uh, my partner, uh, Matt Harmon and, and I are gonna be the moderators. Uh, and, uh, we're gonna let, uh, Tim Kaine and, uh, Mikhail Petrozan, uh, run this show, and then we will, uh, chime in as, uh, needed. Thanks for having us. Can we have the next slide? OK. So we'll talk a little bit about treatment, management, and challenges that we face. Um, it's a very rare disease that kids have. It's, uh, so you may or may not encounter, some of us have seen it. We've become a center, so we do see a lot of kids, and, uh, we'll just go over some questions. Can we have the next slide? OK, we have a nine year old female presented with dysphagia, chest pains, weight loss. Workup revealed type 1 alasia. She was referred for consultation. So Mikael, why don't we stop right there? Why don't you, uh, and we've, I've asked this question before, but why don't you explain the three types of, uh, allaia for our audience? So while it's important to know, I think I was talking to Tim about this when we're coming here, about 67 years ago, we didn't even look at the types of chilasia. Because it really doesn't matter what type you have, because the treatment is essentially is the same. What's important is to understand what types of accolaia exist so you can sort of a set up for parents to understand how the disease progress will go, because the treatment doesn't really respond well to, for example, type 3 alasia. We have a slide, um, let me just show you so it's easier to understand. Um, where's Cecilia? Where's the slide with the type of? It's most of the visual slides, so you can understand the, uh, types of chiasia. Is that in here? Yeah, there you go. So these are the types of allasia that we encounter. They're all essentially the same. What's, what you want to understand is the pressurization and the LES pressure is very high on all three of them. They all act differently. Type 3, if you're gonna have an acolasia, the best one to have is type 2. If you're gonna have one that's worse, it's type 3. Type 3 does not respond well to treatments. So that's why in our stu uh in our uh study, and then team will go over it, you will see that the recurrences happen much more in patients with type 3 alasia. So while it's important to understand types of allasia you're dealing with, it's irrelevant because the treatment is the same. So I, I just put this picture so you can understand most of it is manometry that we get. Uh, so we have to kind of understand how to read the manometry, and those, those of those pediatric surgeons who deal with kids with kalesia or any motility disorder, I encourage you to learn manometry. I encourage you to learn endoscopy because as Tod always pushes us, you, we have to be ahead of the curve, uh, ahead of the ball game because a lot of these diseases goes to the. Gastroenterologist, we're gonna lose the treatment because poems is becoming a main, a mainstay of treatment. I'm gonna go say also that, um, they all respond to, uh, the myotomy, the lower esophageal sphincter, but the outcomes are a little different. So type 2 is the most common but responds also the best of surgery. Type 3 is the most rare in our series, 11%, um, and they have they have, uh, symptoms down the road may or may not be related to your myotomy. We'll try to go back to the question now if we can, uh, you gave us some symptoms. Are those classic? Yes, so it's important to understand the ECAR score. There's also a chart of the ECAR score is, is basically weight loss, dysphagia, chest pains, and the fourth one number regregurgitation. So it's important to know, uh, ECAR scores, and those are the four questions we normally ask. We use the ECAR score to diagnose the kalasia with other things like manometry and also use the ACAR score to follow them clinically. And now it's difficult because a 10 year old won't tell you I have a regurgitation. So you kind of have to understand how you're gonna get that information from parents. And the parents who have kids with kalesia know their kids up and down. They know their symptoms, they can document. They have the diaries of the documentations of their symptoms. So it's easier to understand from parents because a 10 year old won't tell you, yes, I had a regurgitation of a chest pain. So it's important to keep that and learn the ECAR score if you're gonna treat these kids. Um, so the question would be, what would you, what procedure would you recommend? EGD with balloon dilatation, EGD with Botox injection, laparoscopic hermiotomy, or point procedure? Tim can take over this. So yeah, so looks like the lappeller is gonna be the number one, which, which is probably standard of care in, in pediatrics. Um, that's the tried and true, um, approach for calasia, but there is a large, uh, an increasing experience in poem in the world, um, certainly in adults it's become more common than a lap heller, and we'll touch on later about whether you do an anti-reflux procedure or not. I would, I'll just open this up, but who would also do an anti-reflux procedure with, with a heller. About the robotic. 15 year old. Robotic, yeah, you can do that. You can do robotic. Again, if you're comfortable doing the procedure, you should do that procedure, but as of today, I, we believe that Lappeller is probably the gold, gold standard for the procedures. Um, I think there was a, there was a survey done in IPEC in 2015, uh, 2015 somewhere. 95% of pediatric surgeons would do fundoplication with Halermyotomy. I think that was the survey results. Uh, we currently don't do, uh, fundoplication. We do not offer any wrap, whether it's door, um, or toupee. We don't offer wrap if we choose to do hilar myotomies, because in our experience, we've had kids that came back who had heller with door that the wrap twisted the esophagus as they were growing. So when you put the scope in, it's completely twisted. So we've basically went back and taken the wrap down and the symptoms went away. So we had several case reports with with that with so move moved away from doing actual heller uh with the rob. So in the, in the adult literature, if a heller is done, um, there's, there's a fair amount of, uh, good literature showing that you don't need to do a fundallication, correct, uh, and so we can extrapolate that pretty good, uh, uh, in the, uh, pediatric world. So who would do fund application here with the Can I raise your hands? No one. Heller with Fondo without Fondo for you. Yeah. So can you remind us that that don't see it all the time? How detrimental to you at the time of Heller is the EGD dilation, Botox, like if that has happened, so yeah, we have 50% of the kids had some intervention before. Uh, whether it be, uh, not as commonly Botox anymore because people are learning it's, it causes a lot of scar tissue, but many have had dilations and they're just pretty minimal in terms of fibrosis and things. So we don't really recognize it too much. The things that we do see in kids who have had hellers or poems before is there, you got to get into a different plane because it's pretty scarred. Just like if you were doing a redo heller lap heller, you gotta go a little bit off of where your first plane was when you're opening it up. It's doable, but it's definitely a little harder. At least for us, the, the pediatric gastroenterologists are increasingly clever with the, the endo flip and things. It seems, it feels like they're often, they've been there. So, so you're, you're, you're educating them in advance that you don't want this should they find it correct. So that, that's my point was that I think we all as a pediatric surgeons need to be very comfortable doing EGDs. We completely stopped doing rigids all on everything, foreign bodies. We don't do rigid esophagoscopies anymore. It's all EGDs. We've had purchased our scopes. The surgery has their own scopes. And we do our own things. And then I would encourage those who are interested in forgot to register for American Forgot Society. That's a new, relatively new society. It's been done about the last 5 years, and all the guys, Tom De Misters, all those guys who've done oesophageal work are part of that, and they teach flip courses. They teach EGD, they sometimes they have poem courses. That's how we learn in 20132013. So I contacted them. We went to learn it. So learned that it's important to do that because GI will take those from you guys, from all of us. So let me ask you a question back to our poll, uh, question. It seemed like most people, uh, most surgeons would do a, uh, fund application and most would do a door. So the question is, can you tell us, um. What are the disadvantages of doing the fundoplication? I, I think, uh, one is it's a pretty lousy anti-reflex procedure. The most common thing would be that you do that anterior wrap and a kid grows. So if you're doing a kid who's like, you know, 5 to 12 years old, they're gonna grow a lot, and sometimes that'll torque your torque your myotomy. Um, so that's the main thing that we see and what you do see is recurrent dysphagia. So that's kind of the, the. The hallmark of, of Acola is dysphagia. It's not reflux regurgitation. It's a can't eat. And so kids come back, if you do a heller or poem, whatever, they come back because they can't eat. So let's just say you're, you're doing a, um, a laparoscopic, uh, esophagealyotomy and you get a, a little hole in the, uh, anterior esophagus. Would, uh, uh, an anterior fund application help with that, with the healing or preventing complications related to the, uh, perforation? You can. I mean, other people have described doing that to seal the leak, but you can also put a couple of stitches in it and be just fine. OK. And now the door has been modified to a 3 stitch technique, in other words, trying to lessen. The effort you put into it, just 3 simple stitches, right? Just kind of mention about reflux. We have biopsied all the kids that we've done poems a year out, and with our current population, it's the rate of reflux is about 5%. If you compare it to adults at around 50%. So I don't know what's the pediatric physiology is, but the reflux tends to be much, much less common in kids. So hence. Uh, we kind of stayed away from wrapping these kids even if we had to do heller myotomy. So reflux is not really an issue to do the heller, the doorfo obligation, meaning that's what I meant, doorfo obligation after myotomy. So, yeah, the last 10 hellers we've done with no doors. All right, let's go to the next question. All right. So we have a 15-year-old male who presented uh for evaluation. He has a history of type 2 alagia status pool hall myotomy when he was 12 years of age. Continued to have dysphagia, underwent EGD with dilatation and Botox injection. He continues to complain of dysphagia, weight loss, chest pain. He's currently getting feeds by NG tube, so. OK, so, so Does this question, you, you said he had type 2 yasia, for the question, does it matter whether it's type 12, or 3, he's got a recurrence. In a way it doesn't, but it not only it's important, it kind of throws you off because you figure out, well, is type 2 really matter? No, because it's the same treatment. But what, what happens is that you have to realize, is it gonna help them? You have to just tell the parents that type 3, you may have the recurrence much faster than in type 2 or type 1 patient. So what is your diagnosis? Is it recurrent alasia, incomplete myotomy, problem with the Dorfund obligation, or it's end-stage alesia? So let me just ask you a question in this, um, in these possible answers. What is the difference between recurrent alagia and incomplete myotomy? That is, you can actually have a recurrence of the allasia itself as opposed to just doing an incomplete myotomy for the original problem. I think, uh, an incomplete myotomy is basically, you know, not going far enough down. Recurrent calasia, I would more categorize into growth, so someone who grows a lot. So if you have uh symptoms in a child within a year, I think it's an incomplete myotomy. But recurrent alagia could be. So you do a young kid, a 5 year old, they're one and done. You never see them again. You think they're doing OK. That's probably if they end up getting it later, it may be recurrent ylasia based on growth. But so Tim, how far down do you go given that you feel like that's the critical? Yeah, 2 centimeters onto the stomach, 2. Approximately, we, our average length is about 6 centimeters, 6 to 7, so we go about 4 or 5 up. All right, so what does the audience say here, Michel? So, let's see. So, and, again, anything can be right. So we have to be able to, the reason why I put this here because almost except the last D, which is pretty rare to have end-stage alleia in kids, all three could be a possibility. Can he has reco acalegia? Yes, of course. Can he have incomplete myotomy? Of course. Is there a problem with Dorfan lication? Of course. So those are the three things that you have to commonly look if somebody comes in who had the operation before. Uh, with giving the, with the wrap and figure out what, what's wrong with this child. Is there, is any of those three could be the, the answer. So you have to investigate along with the manometry, EGD biopsy, retroflex the camera to look at the, um, at, at the fundus to see if there's any hiatal hernia, maybe. All these stuffs are important to know before you actually come up with a treatment. So, full disclosure, we actually took this kid's door fundal application down, didn't help, and we did a poem. He got better for a little bit, but within a year he had symptoms again, so we redid the poem to get him symptom free. And what's your follow up on this particular patient? So he's a year out now. He's coming back for just routine surveillance next month. Well, it sounds like a perhaps a more complicated patient than we we put it in there because it's important. You'll get these patients and you just have to figure out what's wrong with them. And then surgery is not always the answer. Like Heller, for example, we took the door down to see if that was the problem. It, it wasn't, where in the past we had patients who've had door who've gotten better when we've taken the door down. And then these are the steps we normally proceed. Is it esophagram, manometry, EGD with end of flip, or GI referral, and any of this could be the answer. Uh, if you feel comfortable doing the EGD with endo flip, again, it's not easy to understand the whole mechanism. Uh, go ahead and proceed with that. You can do manometry. Manometry will sometimes always show alesia. It's just never goes away. It's the same manometry. So we don't really send kids for manometry. We'll send them to confirm the kalesi if people come from different institutions and the, or equivocal readings are, and in a sophogram, you can always get. That kind of gives you a roadmap as to what you're gonna encounter when you do the scope. So all the state, all except the GI referral, obviously, if you don't know what to do, you can call GI. But a lot of times pediatric GI, at least in our institution, they're, they're not that comfortable doing the procedures. So they're kind of defer that to us to manage. OK, what's next? I put this there cause we're writing the questions and this is what probably we, what we would do, but again, any of these answers is correct. Um, Endo flip is a, a machine that Um, it's a soft balloon. I don't know if you've seen it. If you haven't seen it, um, there's, uh, like I said, uh, Society offers the courses. It measures the esophageal dissensibility and also the diameter. So it, it guides your way in past when those who, who've done heller myotomy use the scope to guide them into the, the myotomy itself, but now we use this balloon to figure out is the myotomy long enough? Is it good enough to do it, and it's just easy to use once you know the numbers and how to use it. So it measures the diameter of the esophagus. So before and after your myotomy, it also measures, there's accepted numbers for adults in distensibility index, which is the amount of, uh, pressure you need to distend the esophagus a certain amount. And there's accepted standards for normal in adults. And we extrapolate the kids and we, we shoot for those numbers. Uh, numbers are not important, but you can see before and after whether you had an adequate myotomy. So let's, uh, let's just talk a little bit about poem, uh, because I think that for many of those in the audience this is a new concept, certainly a new technique. So just tell us sort of what, what what you're doing, how you got into, to doing the poems and some of your, uh, results. Yeah, so, um, I, I was interested in this in the late, late 2000s. NOA came out with the first, uh, uh, human case in 2010. Um, so I'd always been thinking about it and when Mike was a fellow of mine, finished in 2013, he said we're gonna start doing this. So we went to Lee Swanstrom in Portland, took a course, learned how to do it, and then we're gonna bring this back to Children's. Took us two years to get the equipment, so we really did our first case in 2015. And the first two or three cases, we, we basically struggled and, but we always had the, the component that if we can't do this poem, we're gonna do a heller. So we just, if we couldn't do it, we just turn into a heller. And so eventually we got the hang of it and we haven't really had to convert one since 2018, but it's, it's a steep learning curve. There's a lot of equipment we end up having to learn how to turn everything on and make sure everything's right. You got an Irby knife, you got a, you got a tower, you got injections, um, so there's a lot of stuff that can go wrong, even, you know, a couple of weeks ago someone had hooked the wrong plug up that we had to troubleshoot. So, um. But it's a, it's a, it's hard because it's a volume thing. So if you're not doing a lot of them the first year we did 2 or 3, the next year, 21 year none, and then now we're doing, you know, 15 a year, but it's just doesn't, doesn't build that fast. So it's hard to get your skills up. So doing a course, then what do you think the learning curve is if you were to, so those of us who have not done this, so the learning curve essentially is about 20 and mastery is about 60 is what in most of the adult. So you're an expert now. You two are experts. We're challenging. It's a, it's a tough procedure. Yeah. So, so, tell us why, uh, you feel this is the best way to go or what are the advantages of pulling over the laparoscopic, uh, esophageal myotomy. Yeah, I think you have, you have 360 degrees of options to do a myotomy, whereas with a Heller you're kind of more anterior, so you got maybe 180, um, you don't, you, you got the Vegas there that you gotta worry about. Um, and a re-op for a heller, you, you're, you have all those issues. So you can do a redo poem after a heller or a poem, and you can use it, choose a different side, uh, to do your dissection once you get in a clean spot, so you don't burn any bridges. You don't dissect the hiatus, so you're not worried about reflux, um, so you try to find the sweet spot where you've got enough. You want your endo flip to look not like an hourglass like it looks like pre-myotomy. You want it to look more like a little waste. And so I think yeah, it's a, it's a nice technique. So this is some of our data here. We, we had, we've done 73, you know, through this month. Um, we need for re-intervention, these kids about 15%, which is about equal to what we had in our Heller experience. Although we Uh, in re-intervention means I, I classify that as needing a balloon dilatation within 1 year before 1 year at post-op, um, and we've done 5 redos and interestingly those 5 redos, 3 of them were kids with type 3, and one of them was that kid we just described that that had the heller and then a poem. Um, the, the, and those kids with type 3s presented within a year with trouble, so. Um, it's definitely doable, but those are the kids that are a little harder. And again, this is our, our breakdown of the kids. 11% were type 3, which is kind of high compared to most in the literature. The majority are 62% are type two, which are those are, those are nice because basically you, you, you do the myotomy and they basically don't have any peristalsis type two. So they, you just open that tube and they swallow. The problem with type one is you open the LES and they have spastic uh esophagus, so they sometimes have more symptoms. Um, and type 3 are just like a jackhammer sometimes and. Take a longer time to, you know, become symptom-free if, if they do it all. Uh, poll, the surgeons in the room who has tried this, the poem, anybody other than these two guys? OK, we probably should have had that as an audience question of how many have, well, we're hoping that it's gonna, it's gonna become mainstream once it's trainable for sure, yeah, um, yeah. Any other questions from our audience? Cecilia, I have a question. Um, since we can see that we have like a lot of surgeons here and only you two do the poem, how do you think that you can teach or show other pediatric surgeons how to do this procedure and how to get used to it so that we can spread this among everyone? Exactly how we learn there has to be a course which we're planning to do is a long time in coming, so. It's important to do that and spread the word around because I don't think a course is going to do it. That's what do you suggest? So the answer is because I was thinking about that, the same question, why are, why has this not been adopted, right? There's still two people in the whole country impedes you guys and James is not doing it now, so just you guys speaks poorly for something. Right? If only one center in the entire country, and this has been not new, this has been now a decade that this has been discussed and still only one center. So either there's lack of education, It's not a good procedure. There's distrust among the pediatric surgeons, or they need a team approach with a continued learning thing because I've been to a course. I still don't feel like I am, I can just go start doing it. I think I need a GI who's ready to do it with me, or another partner. I need to have, you know, more than just go do a laparoscopic hernia. It's, it's a much more involved thing. It's like bariatrics. You disagree? No, I agree. I agree. It's, it's rare. So, I mean, just look at the amount of ped surgeons that do thoracoscopic TFs still hitting that 10%, right? It's low. So, I used to think we're going to train everybody, we're all going to do it. And then when in reality, when you got your fellow next to you, some of them are going to do it based on volume in which they've done, and some aren't because they just didn't have the opportunity. And there's so many variables with anesthesia that you're not going to be able to get through a case. So, so here's how it'll happen. Go to a course. And then you guys would have to go to an institution for their first couple, which you're not gonna want to do. You could do telementoring, but they, I, I think it's worthwhile trying to figure out how do we actually, next step is, yes, we've done it. Now, how do we start showing scale up? Well, I also think though that. That there has to be, I guess, a demonstrable improvement over the current technique, which is the laparoscopic esophagoyotomy, which most pediatric surgeons feel comfortable doing and so, uh, I just think that there has to be in your own hands since you're, uh, taking care of that patient, you have to feel that you can do a better or get a better result with the poem tech technique. Now I personally. Uh, I think that the poem, um, that referral to, uh, to Children's National might be a good approach if you have, uh, a failure or incomplete myotomy or a complication related to the laparoscopic hellermyotomy. And I'm not so sure that we should, that this, you know, we've talked about so-called centers of excellence for a long time. That, that, uh, Children's National shouldn't be considered a center of excellence for complicated or recurrent disease or, or, or a small subset of patients as opposed to the having the general, having most pediatric surgeons trying to do it. Just by 2 cents. Um, one other thing I was gonna say is that a lot of times when patients come in, moms and parents want the poem procedure. We don't even talk about Heller. They don't, we don't, we get an email that they tell us we wanna have a poem because they've done the research. They don't wanna hear about Heller, period, period. So a lot of times that's how most of the referrals come to us. They don't say, Well, we're gonna do Heller. They just, they don't wanna hear about it. They just wanna go straight to the poem, even though, uh, the 12 months old wasn't really, we, we did a heller recently. And she just was too small. We didn't have equipment to do, but she wanted to have Heller. I mean, uh, poem, period. Tim wanted to do it. I prevented him doing it. He's 8 months old, 8 months old. Yeah, that didn't happen. But the, the bottom line is I, I do agree with you. However, I think as the surgeons we do have to be comfortable doing these procedures whether it's EGD, and a lot of times we've done PMGs, the, uh, uh, your dad done. And that on 3 patients by just learning this technique. It was, it's exactly the same procedure in a pyloris. For gastroparesis and it worked so well, listen, I think that all of us, uh, congratulate you too on, on what you're doing and we look forward to, you know, continuing to hear about your results and hopefully we'll have a course soon in life and then we can talk about how we're gonna teach the rest of them. Well, that's important. Then they come and spend the day, but that's hard because it's rare they'd watch. Then you have to do telementoring eventually. I mean, we do about 22 to 3 months from now, yeah, so it's, it's been, it's become, becoming my challenge to you guys would be you, you, you always, I was telling you this morning, you guys keep leading us with these things that you do that no one else will do. Next step is, OK, we do it here. Let's figure out how to show next year that the numbers of centers have grown and attribute it to you guys. So congrats. We had a video, but anyway, post it online, people can watch the procedure. Great. Thank you. Thank y'all. Thank you. Mr. All right, um. From allasia to trauma, we're gonna be doing trauma up next. We're gonna take a one-minute break and set up for trauma.