Chapters Transcript Endoscopic Management of Achalasia Dr. Arvind Rengarajan, MD shares an overview of how endoscopic techniques are used to manage achalsia. So we have doctor Arvin Ragin, right rag. He earned his medical degree, residency and chief residency in internal medicine, as well as his fellowship in gastroenterology at Washington University School of Medicine in Saint Louis. He then went to Northwestern University in Chicago, Illinois to complete his fellowship and interventional endoscopy. And thankfully returned to Saint Louis to be an assistant Professor of Medicine and interventional endoscopy with the washy physicians at Orange Jewish Hospital. Some of his research interests are echo Asia and esophageal motility disorders, pancre uh pancreatic obi disorders, quality improvement. So I don't know if I pronounced that other one, right? Oh, no, that was perfect. Yes. Thank you. Thank you so much. Thank you. I'll let you take the uh floor. All right, perfect. Yeah, I appreciate that introduction. Uh Good afternoon, everyone. Thanks for attending uh this webinar. Uh Again, my name is Arvin Rra and I just joined Washi faculty as a therapeutic endoscopist. Um And today I'll be presenting on the endoscopic management of Achalasia. Uh I have no financial disclosures. Uh a brief outline of what we'll be discussing today. Uh Again, I hope to kind of provide a brief overview of Achalasia. And then move on to discussing some of the various endoscopic treatment modalities for Achalasia, the benefits and limitations of each. Uh and then we'll sort of wrap up and spend the bulk of the talk discussing some of the uh logistical and procedural specifics uh surrounding her oral endoscopic myotomy or poem as a treatment approach for Achalasia. Uh I don't anticipate taking the entire hour. So I'm happy to field questions. Uh towards the end of the talk, I'll start off with a case presentation. So, uh we have a 45 year old gentleman, no significant past medical history coming in for ongoing issuess with dysphagia and, and retrosternal chest pain. His work up uh is negative for cardiac ideology for his chest pain. He's referred to a gastroenterologist who ends up doing an upper endoscopy, uh which demonstrates no evidence of an esophageal stricture or mass no esophagitis. But they do know that there was um some moderate resistance to passing the endoscope through the gastroesophageal junction. Uh This then leads the gastroenterologist to obtain a high resolution manometry study of the esophagus. And you get uh this sort of weather plot here. Um So this, this looks pretty consistent with something called type two Achalasia, which we'll get into uh a little later on in the talk. Uh And so, you know, he's now in clinic and he asks what treatment options exist for this. So, uh you know, moving on to to achalasia. So this is a, a progressive degeneration of ganglion cells within the myenteric plexi of the esophageal wall, which ultimately leads to failure of relaxation of the lower esophageal sphincter. And it's also accompanied uh by dysmotility uh and a loss of peristalsis within the esophagus itself. Uh This inflammatory degeneration preferentially involve the nitric oxide producing uh inhibitory neurons of the esophagus uh which essentially uh lead to, you know, chronic activation or, or uh ongoing uh sort of activation or tightness of the lower esophageal sphincter. In terms of epidemiology, you know, thankfully, aasia remains a relatively rare disorder uh with an annual incidence of, you know, 1 to 2 cases per 100,000 individuals. Uh men and women are affected equally and it, it's rare to have onset of achalasia before adolescence and patients are typically diagnosed between 25 and 60 years of age. Uh And, and that sort of uh as a result of sort of an insidious onset and gradual progression of symptoms. Uh you know, one uh cross sectional study basically showed that uh you know, patients usually have symptoms for anywhere from 4 to 5 years prior to the, you know, their diagnosis of Elaia. Uh And that's because, you know, again, that they sort of wait until symptoms become severe enough where they, where they then seek out medical attention in terms of, you know, clinical symptomatology of achalasia. Again, the symptoms include but not limited to dysphasia regurgitation or aspiration events, uh retrosternal or substernal chest pain, hiccups, weight loss, um you know, difficulty belching again, that that's in part because that lower esophageus sphincter is, is taught, uh and it doesn't allow for uh normal belching to occur. And this actually has led the A sge to uh release guidelines um that, that, you know, make recommendations that achalasia should be suspected in patients with dysphasia to both solids and liquids. um that are unresponsive to an adequate trial of PP I therapy. Um So, the diagnosis of achalasia generally involves uh a combination of clinical symptoms and presentation as well as endoscopic and radio radiologic imaging and specialized testing including high resolution manometry. Um So, you know, radiographically, the classic birds beak appearance is seen on barium esophagram and that's a result of, you know, a taut lower esophageal sphincter uh and a dilated esophagus uh on endoscopy, 1 may note a pucker or hypertensive lower esophageal sphincter um with retained contents upstream in the esophagus uh as well as you know, resistance to passing the endoscope through that lower esoph esophageal sphincter. High resolution manometry still remains the gold standard uh for the diagnosis of achalasia and can actually help further stratify patients into um specific subtypes of achalasia which will be important for for um discussing treatment uh modalities. So, again, high resolution manometry um you know, involves a catheter system um that has Cial sensors, uh roughly one centimeter apart and uh with digital data assimilation and you know, various software tools. Uh This allows one to basically interrogate important elements of esophageal peristalsis. And as I mentioned before, manometry is important, not only for the diagnosis of achalasia, but then to, to further stratify patients into one of three subtypes of aplasia. Um So, you know, type one is sort of the classic aplasia where you have a chronically taught lower esophageal sphincter that does not relax coupled with complete loss of peristalsis within the esophageal body. Type two is very similar where again, you have a, you know, chronically active lower esophageal sphincter, but there's something called pan esophageal pressurization, which is what um you know, the manometry that I from our case presentation demonstrated and this is actually a favorable sub type uh because this pan eop pressurization um uh gives us sort of uh an indication that the esophageal body peristalsis may recover if that lower esophageal sphincter um is is addressed. Um And then type three is again a very active lower esophageal sphincter coupled with a spastic esophagus. Um So these can be sort of dichotomy into non spastic achalasia, which includes subtypes one and two. And spastic achalasia, which is subtype three in terms of the pathos and, and you know, who develops aplasia. And why again, you know, this is a result of inflammation and, and degeneration of neurons within the esophageal wall. But the exact cause for this inflammatory degeneration uh isn't known in primary achalasia, you know, some of them um that variants of HL A, you know, DDQ may result in circulating antibodies that attack these inter neurons. Uh but nonetheless, you know, there appears to be a complex interplay between environmental factors, autoimmune mechanisms as well as um you know, genetics in terms of, of why 1 may develop aphasia moving on to, you know, management now. So treatment modalities are generally focused on disrupting the lower esophageal sphincter in order to aid with, you know, emptying of esophageal contents into the stomach. And those can include things like pharmacologic agents, um like calcium channel blockers, nitrates, et cetera, um or endoscopic, uh you know, treatment options which may include things like Botox injections, pneumatic dilations, um or endoscopic myotomy or surgical myotomy. And I typically tell patients, you know, the treatment options range on the spectrum of less invasive to more invasive uh with the caveat though that these less invasive options are unfortunately temporizing without any durability or sustained response. Uh and then that these more invasive options typically are the ones that give you a more durable response. Uh And so again, for this talk, I'll be focusing more on just the endoscopic management of equation. So, so sort of moving from the spectrum of less to more invasive. Um we have first up Botox injection. So here, you know, the the mechanism is uh you know, the end endoscopy, basically taking a square therapy needle and injecting botulinum toxin into the lois Opto sphincter. And this basically reduces the pressure by inhibiting release of acetylcholine uh from these nerve endings. Um But again, you know, the main uh shortcoming of this treatment approach is the durability, which if your patient is lucky may get some response for a couple of months. Uh But then ty typically, you know, symptoms recur uh furthermore, the issue with botulinum injection is that one can actually tachyphylaxis, meaning that uh you know, there's sort of a progressive decrease in response um to a given dose after repetitive administrations of Botox. Um transient chest pain tends to be sort of the com the most common adverse event reported in only 4% of injections. So again, it's, it's a, it's a fairly safe, you know, less invasive uh option. But again, the issue is the durability uh then moving along that spectrum is pneumatic dilation. So here, um you know, the the thought is to disrupt the lower esophageal sphincter fibers using um you know, a pressurized balloon under fluoroscopy. Um But you know, your patient that you would recommend this in should be a good surgical candidate because one of the unfortunate sort of complications or adverse events of pneumatic dilations can be an esophageal perforation in which case, you know, they would need to go to surgery for surgical repair. Um Initial success rates are documented anywhere from, you know, 85% clinical response at one month, but again, the issue here is again the durability or sustained response um because the efficacy does over time, uh usually on the order of 6 to 12 months. And as I mentioned, you know, common perioperative adverse events include esophageal Corporation, which can occur up to 3% of the time substantial bleeding and symptomatic. And so that that kind of segues us into, you know, sort of the more invasive endoscopic treatment option, which is poem or her oral endoscopic myotomy. So, uh interestingly, in 2007, you know, this was a paper that was published by doctor uh Patricia and his colleagues. And they basically had a hypothesis that a myotomy could be performed uh by working in the submucosal space with the endoscope. And so they basically experimented on four pigs where they described this poem technique, uh where they perform, performed a circular myotomy, roughly five centimeters above the lower esophageal sphincter. And what they found was that uh lower the lower esophageal sphincter pressures significantly fell after the myotomy. Uh with no, you know, adverse events or significant adverse event, I should say. Uh And so this is sort of a graphical description of, of, you know what poem entails, which is basically, you know, driving the endoscope through this submucosal tunnel. Um And in order to basically create a pot and a safe a buffer to expose the muscle fibers of the esophagus and the lower esophageal sphincter. So that one can then do an endoscopic myotomy. Uh Doctor Ine in Japan was actually the first to perform poem in human patients and published his findings in 2010. Uh This was a case series of roughly six or of 17 patients. Uh And what they found was that poem significantly reduced dysphasia, symptom scores um with a significant reduction in lower esophageal sphincter resting pressures. Uh And additionally, there were no significant adverse events fast forward about a decade. You know, nine years later, this was a pub uh study that was published in Jama, uh which was the first uh randomized control study looking at poem against pneumatic dilation. Um And so this was a study that was performed across six different hospitals uh with patients evenly randomized each treatment arm and clinical success was documented in 92% of patients undergoing poem. Uh and only 54% of patients undergoing pneumatic dilation. Uh One of the criticisms of this paper was that, you know, this was only a one time pneumatic dilation. And that in clinical practice, you know, patients can get repeated pneumatic dilations. But even if you account for um sort of successive pneumatic dilations, clinical response is usually plateau in the 70 to 80% range. So home still, you know, uh is a good initial treatment option for patients with achalasia. Uh And they documented, you know, two serious adverse events in the p nomadic dilation group including one perforation and none in the poem, uh reflux esophagitis was documented in, in 41% of the poem group and 7% of the pneumatic dilation group. And again, you know, again, this is something that I, I try to before, you know, performing a poem discuss with the patient in clinic is that, you know, we, the goal of palm is to really disrupt that lower esophageal sphincter and allow things to move from the esophagus into the stomach. But that can unfortunately predispose an individual to reflux. Um because now, you know, the lower esophageal sphincter is, is open. Um you know, again, so reflux tends to happen in about 40% of, of patients undergoing poem, but it's usually well controlled and in upwards of 95% of patients with, with PP I therapy. Uh Next was a paper that was published in New England Journal of Medicine um which basically was a head to head of poem versus Heller myotomy with uh dwarf on D application. And so here, you know, patients were again, equally randomized each treatment arm and the primary endpoint was clinical success at two years post intervention. And what they found was that there was clinical success and 83% of patients undergoing poem and roughly 82% of patients undergoing Heller myotomy. And so again, this supported the hypothesis that tom is non inferior um to Heller myotomy. And the absolute difference um of clinical response was about 1.4% in support of Tom. Um And when you look at sort of adverse events, you know, g tends to be the one that's sort of overrepresented in, in poem. Again, it, it, it's in part due to the fact that 11 does a heller myotomy, they can then do a fund application to help re reduce reflux. But actually, you know, interestingly, despite the fact that patients underwent fund application, there was still a significant portion of patients who ended up developing e erosive esophagitis. So 29% in the uh Heller myotomy with do fund patients compared to 4% in the pollen group. Um One of the, you know, initial criticisms of poem um again, at least initially was the durability or the lack of long term data. So this was actually a, a paper that was published in 2021 that um basically followed patients over a 10 year period and rolled over 600. Um you know, patients who had undergone poem and what they found was basically procedural time was less than an hour. And clinical sign, you know, clinically significant adverse events only occurred in about 3.4% of patients. And interestingly enough, you know, none of these adverse events led to death surgery or, you know, uh interventional radiology interventions or drains uh and or altered functional status. Um And then when you look at the Kaplan Meyer curve, you can see that the clinical success is maintained over, you know, the 10 year study, uh over 90% of patients. So overall, you know, poem is a minimally invasive and durable therapy for Achalasia. Um And, you know, poem really was derived initially from Heller Myotomy. And so what, what's done sort of classically in poem is to do a standard myotomy, which is, which is basically eight, you know, 6 to 8 centimeters within the esophagus, you know, you're basically cutting muscle uh and then eight centimeter segment, and then you extend that myotomy about 2 to 3 centimeters within the gastric cardia. And again, these numbers come from what's done or, you know what's done classically uh inhaler myotomy. But, but in for patients that have non spastic achalasia, so that type one and type two, you know, again, really, the issue tends to be just at that lower esophageal sphincter. Uh and that, that tends to be sort of this high pressure zone and, you know, pneumatic dilation in the setting of non spastic achalasia is evidence that therapy is really only needed to target this short segment, um which makes the sort of standard myotomy perhaps a little bit too long. You know, again, we're, we're doing an eight centimeter myotomy for really what, what spans about 2 to 3 centimeters in terms of the lower esophageal sphincter. Um And so the downsides that can potentially occur by, by doing a standard myotomy in patients with non spastic aphasia is that there's a risk for developing what's called blown out myotomy, which I'll get into in just a second. Um And this also may also, you know, contribute to the post poem reflux that we see. So, uh you know, a phenomenon that's been sort of um more clearly described as a blown out myotomy. And what that is, is basically a myotomy failure associated with the development of a pseudo diverticulum within the distal esophagus. And, and it likely occurs because again, that myotomy weakens um the wall there. Uh And there's increased strain. Um And then, so this sort of phenomenon is associated with poem and Heller myotomy, but doesn't really be, you know, isn't really seen after doing nematic dilation. And so this is what we see, you know, classically on uh esophagram sort of, you know, this, this distal esophageal pseudo diverticulum. Um And, you know, this was sort of better described uh in a paper out of Northwestern in 2021 where they basically enrolled or, you know, basically collected data on 100 and 29 patients. And, you know, nearly 20% of them had blown out myotomy um with, you know, an overrepresentation in patients who underwent teller myotomy. Um And this was more common in patients that had type three aasia. So this had everyone sort of asking, you know, perhaps doing a standard myotomy in non spastic aphasia may pose more risk than, than, you know, benefit. And So then why don't we do a shorter myotomy to mitigate some of these risks? And so this was a concept that was then further explored. Um This was a paper that was published by uh Nay Reddy's group in India where they were basically randomized patients to either a short myotomy. So a three centimeter instead of the standard 6 to 8 centimeter esophageal myotomy or a standard long esophageal myotomy. And what they found was that procedural times were significantly shorter uh in the short myotomy group. Um you know, that, that makes sense and that short myotomy was non inferior to long myotic and that there was no significant difference in bird rates. And so this is sort of their um you know, a tabular form, a tabular representation of, of their findings. And basically, you can see that clinical success at at one year was, was no different between the two groups. Um And then, you know, this was again, also reiterated and re illustrated by a trial done in China where again, they basically took non spastic achalasia patients and and stratified them to either short versus standard myotomy. And again, they basically found uh you know, again, no significant difference um in terms of clinical response rates at one year. And um so, a recent advancement or development in the world of poem has been the intra procedural use of something called functional Luminal imaging program or flip for short. Um So, prior to this, you know, when one did a conventional poem, uh it would really rely on the endoscopist visual assessment after they did the myotomy where they would basically re examine the esophagus and then make a determination as to whether or not the myotomy they just performed was sufficient enough um without any real real time objective or physiologic data. Um And so then more recently, there's been a push to use this flip um technology interp procedurally. So flip is basically a balloon catheter assay that can basically measure the diss ability across that lower esophageal sphincter. So, again, basically, prior to doing one's mono uh my, um we basically do um you know the flip and what you can see is that that lower esophageal sphincter is very taut, it does not distend, then we do the palm procedure. And then we basically, as soon as we're done with the myotomy, basically repeat the flip. And now you can see that things are, are, are more open. Um So this, this allows for real time assessment and can allow the endoscopist to make sure that the myotomy that they just performed is sufficient and if it isn't sufficient or insufficient to then um you know, basically do more of a myotomy in order to open things up. And so a post therapy distensible index is basically a, a metric that's gleaned from flip uh of less than 2.8 has been associated with worse outcomes. And ation basically, the take home point here is that, you know, with this technology, we now have real time as assessments to make sure that the myotomy that we just performed is sufficient so that we're not having, you know, patients come back with ongoing symptoms due to an insufficient myotic. And so, you know, putting this all together um is kind of a a little video demonstration of, of what all of this entails. So, you know, again, in the setting of pa uh achalasia, you know, we use endoscope, this is a patient that has type two achalasia. We do uh you know, you can use either an anterior or a posterior approach. Um but we basically mark using cautery um and then make a submucosal blood where we take an injection needle and basically make a blood um after injecting. And then this is our initial mucosal incision. And so we make this long enough where we feel like we can tunnel now the scope into the submucosal space. And so, you know, we continue to basically elongate this submucosal tunnel. Um and we do this, you know, to the lower esophageal sphincter and past the lower esophageal sphincter about 1 to 2 centimeters into the gastric cardia. And then as soon as um you know, the submucosal tunnel is complete. Now we focus on the myotomy. So we have the inner circular fibers and the outer longitudinal fibers and attempts are made to really preserve these longitudinal muscle fibers and really focus the myotomy on just the circular muscle fibers. It's thought that these lower, you know, the, the longitudinal fibers don't really play a role in the dispensable of the esophagus. And that perhaps by preserving this layer, we can potentially prevent blown out myotomy in the future. So then the myotomy is then extended through the esophagus into the gastric cardia. And as soon as the anatomy is done, then we pass the flip catheter and we inflate this um to predetermine fill volumes. And we get basically a real time assessment of what's going on. So here you can see that despite having done the myotomy, you know, that lower esophageal sphincter as characterized by this really, you know, red segment is still not really distensible. So it's, it's not really opening as well as we hope, which basically clues us in that the myotomy that we just performed was insufficient. And so then we go back into the tunnel. And what we do is we basically do what's called a rescue lateral myotomy in which we basically uh extend the myotomy laterally uh by again, sort of focusing on those circular muscle fibers and trying to preserve those longitudinal muscle fibers. And we do this for the entire segment that we, you know, just created. And then as soon as the, you know, the this lateral extension of the myotomy is, is complete, then we basically withdraw the endoscope again and then repeat the flip catheter test and, you know, inflate it to predetermined fill volumes. And then now you can see that, you know, a after done, after having done that sort of lateral extension with the lower esophageal sphincter is now much more open. And so we feel ok, the myotomy that we just performed is sufficient and that the patient is going to have a clinical response. And, you know, you get sort of a, a visual clue as to this as well. You know, endoscopically with the lower esophageal sphincter being less taut. And then as soon as we're, you know, basically happy with what we've done, we basically close that initial mucosal incision with a couple of clips and then we're done. And uh that's actually all I have. So I'm happy to field. I don't know how long it was only 30 minutes. I'm happy to field any questions, comments. Thank you so much. That was a great presentation. Thank you. You're welcome. Yeah, if anyone has any comments or questions, um please feel free to type those up in the Q and A or in the chat, either one. I know sometimes we don't have questions. I can't think of questions on the spot. And so if you have any questions later on or whatever, you're more than welcome to email me, you all have my email address um from the registration form. And also uh I will be sending out um some information on how to contact doctor. Right. I'm gonna try it again. It's r gagn. Perfect. Yeah. All right. So um doctor uh Kimberly Buck a nice video at the end. She is one of the nurse practitioners that is with central medical group over at the highlands. So, um but yeah, if I get any other uh comments, questions or anything, I, I will send those on to you and I thank you so much for your time today. Yes, of course. My pleasure. Thank you. Um. Created by Presenters Arvind Rengarajan, MD Assistant Professor of Medicine, Gastroenterology View full profile
