Achalasia

Hi, and welcome back to Behind the Knife. I'm Joey, a second year surgical resident at Duke here with the MIS Behind the Knife Team to discuss the treatment of Achalasia. I'm joined by Dr. Zach Weitzner, our MIS fellow, Dr. Jake Greenberg, the head of our division and Vice chair for education, and Dr. Dana Portier, one of our senior faculty and MIS Fellowship Director.

Hi, I am Jake Greenberg, and today we're gonna be talking about achalasia. So let's dive right in. So, understanding that many of our listeners probably know the answers to these next few questions, Joey what do I need to diagnose Achalasia and, and what tests help us diagnose it. So you need to know that their lower esophageal sphincter doesn't relax.

There's an absence of organized peristalsis and there isn't a mass stricture or another structural explanation for your findings. The gold standard for diagnosis is esophageal manometry with an EGD. Okay, so let's say our patient meet those criteria but we want to further classify them. Joey, are there any different types of achalasia.

Yes.

So the Chicago classification breaks down achalasia into three types based on peristalsis, spasm with swallows and integration relaxation pressure, or IRP, which is a measurement taken during manometry that assesses how well the lower esophageal sphincter relaxes. So if a patient's lower esophageal sphincter does not adequately relax, you'll have a higher than normal pressure, greater than 15 millimeters mercury.

If you have an IRP greater than 15 and failed peristalsis, you have type one achalasia. If you have both of those things in pan esophageal pressurization, which is another manometry finding, in 20% of swallows, you'll have type two achalasia. And if you have all three of those things as well as spastic and distal contractions, you'll have type three achalasia.

So type three is also known as spastic sia. Great. Zach, does it matter what kind the patients have? It's crucial to properly recognize the different types of achalasia because treatment recommendations vary based on disease type per oral endoscopic myotomy or poem is the treatment of choice for type three achalasia because it allows for an extended myotomy in the setting of pan esophageal

pressurization and distal spasms types one and two or treated with either Haller, myotomy or poem.

Okay, great. So once we identify the subtype, what else do we need to know about our patient? We need to know about their medical comorbidities, their baseline functional status, and other factors that affect their surgical candidacy. This matters because while some less invasive procedures such as pneumatic dilation or Botox injection may be considered less durable and effective, they're often better tolerated for high risk surgical patients.

This is actually a great opportunity to segue into a head-to-head comparison of a couple of these strategies. Dr. Portier. Awesome. The first paper we have to discuss is a landmark study published in 2023 in gut, a gastroenterology journal that charted the 10 year follow up of the European Achalasia trial.

When this paper came out, I was surprised to find out how durable pneumatic dilation actually was when graded over time. As a surgeon, I typically lean towards more definitive myotomy, but this

showed that with the right protocol, pneumatic dilation can hold its own long term. Yeah, I remember first reading this paper and then, and actually even reading the initial study when it came out with just two year results, and what stood out to me was that.

I personally would rather have a one and done treatment, so I would I, if I were a patient in this study, I would want to have a hell or myotomy as opposed to serial pneumatic dilations because I could go back to work, be done for 10 years and be equivalent to someone who needs two and maybe three sets of different dilations.

However, this study really lets us have informed discussions with patients so that they can make decisions about their thoughts for treatments. Because they do seem quite equivalent. Additionally, this study really emphasizes how difficult it is to study achalasia due to the varying symptom presentation and lack of level one evidence.

All right, so traditionally we've considered pneumatic dilation is not very effective. In fact, prior to this study we were going on estimates of around a 40% success. What's different about how these

folks are measuring it from prior studies? When setting up their groups, they have a different definition of treatment failure compared to previous studies In this study, the pneumatic dilation group is allowed to have three serial dilations in the first two years and are only considered to have treatment failure if red dilation is required outside of that time window, if there's an esophageal perforation.

If the patient refuses subsequent dilations in older studies, requiring even a second series of dilations would have constituted treatment. Failure of note in the initial study, esophageal perforations were much higher risk with a 3.5 centimeter starting balloon size, which was subsequently reduced to three centimeters with insignificant improvement in perforation risk.

Okay, so what was the difference in the outcomes? The 10 year follow-up makes this one of the most robust studies in the treatment of achalasia that we have. The only statistically significant difference in outcomes was in the barium column height at five minutes on an upper GI with pneumatic dilation being higher, but both were well below the threshold of clinical significance with respect to

recurrence, what I think is most important is that the patient reported symptoms were not statistically different at 10 years between the two groups.

Joey, why is the subgroup analysis important here? Type two Achalasia appears equally well served by pneumatic dilation or lap heller with a nons statistically significant trend toward pneumatic dilation while type three is better served by lap Heller. The risk of GERD was essentially the same for both groups.

All right, but now we have to bring in a third option per oral endoscopic myotomy or poem. Yeah. To me, I think poem really represents the, the true progression of treatment of this disease over time. I remember seeing the, the first treatment of achalasia through a, a thoracotomy and a Heller myotomy, and then over time, this progressed to a laparoscopic Heller Myotomy, and then eventually a robotic Heller Myotomy.

And now finally a poem where we have no incisions whatsoever. To me, this really emphasizes the progression of surgery to minimally invasive techniques in order to achieve better patient outcomes. Joey,

why don't you get us started here? How'd they approach comparing poem to pneumatic dilation? Okay, so here we have a landmark study in JAMA from 2019.

133 patients were randomized to either pneumatic dilation with a 30 millimeter and 35 millimeter balloon or poem across six hospitals in six different countries. Treatment success was defined as an ECKHART score less than or equal to three, which is a patient related outcome measure associated with achalasia symptom severity, and the absence of severe complications or re-treatment.

Compared to the above study, patients could have one to two dilations within six to eight weeks of randomization. Patients with quiescent symptoms. After two dilations were offered poem followup period was two years. Notice the difference in definition here. While those in the prior study could receive up to three dilations over a longer time period, these patients could receive only two in a tighter timeframe with the narrower definition of success.

Pneumatic dilations succeeded only 54% of the time while poem had a 92% success rate. Poem also carried a lower risk of serious adverse events with zero perforations compared to one in the

pneumatic dilation Group. Poem did however, carry a higher risk of reflux esophagitis at 41% compared to pneumatic dilation at only 7%.

So how about the long-term follow-up for poem? What do we know about that? Yeah, that's a little tricky. So poem really only came onto the scene in 2009, 2010, and most studies we have go out only two to three years. That said, there's a prospective cohort study in gastrointestinal endoscopy from 2021 that integrates data across a 10 year period with follow up as long as seven years.

Notes some interesting trends. The success rate defined as an Eckhart score less than three, and no need for additional treatment is still 91% at seven years. Though it's worth noting that it's an N of only 29 by that time point. The other interesting finding is in relation to GERD as opposed to LAP PAL or myotomy, which we know has increasing rates of reflux over time.

Presumably, as the wrap loosens poem appears to have lower rates of GERD with increased length of follow up, possibly because the lower esophageal sphincter pressure increases and the compliance decreases over time. Though evidently not to pre poem

levels. So based on their data around five years out, the risk or presence of GERD is about the same between POEM and LAP Pal Myotomy recipients, because the former's gotten better and the latter's gotten worse.

And finally, it's also worth noting that this reflux is responsive to PPI and that in this study progression to Barrett's esophagus or peptic stricture was seen only in a small number of patients non-compliant with their PPI and the strictures were treated successfully with serial dilation. This again, really highlights the balance in the treatment of Achalasia.

And I think we have to expect that some patients will have GERD after we treat their achalasia, but we're doing so to maximize improvement in their swallowing. So in order to effectively treat achalasia, I believe we have to accept that there will be some iatrogenic GERD afterwards. And thankfully, luckily most of it is able to be treated medically in most cases.

Yeah, this is where it gets tricky for me. I'm trained to think in terms of durable mechanical fixes. So laparoscopic hell or myotomy with fundoplication has always felt like the safer bet when it comes to reflux. But the poem data

really makes me pause. Yes, gird rates are higher early on, but they actually may trend down over time, as Joey alluded to, which isn't what I would've expected and what I appreciate.

BA. About poem is that it leaves the door open. You can take a step up approach, start less invasive, see how the patient does, and escalate treatment for GERD only if needed, whether it's just PPI or even a fund application later if symptoms warrant it. So who is doing poems? Is that a skillset I'm gonna have graduating residency?

Is it baked into every MIS fellowship? What exactly is the role of an MIS surgeon versus an inve, interventional gastroenterologist for the management of achalasia? I think ideally it's really a team-based approach. Achalasia is a very, very rare disease. And so if everyone was doing poems, you'd have multiple different providers across very, very different specialties doing maybe one to two a year.

And instead, it's probably better to concentrate that skillset into fewer providers so that they can do more

cases and, and maintain their skillset within them. So while it's part of some MIS fellowships, it's not part of others, it's. Part of some gi practitioner skill sets and not part of others.

And so I think it, it's good to have a multidisciplinary team approach, but really to, to restrict the number of people who are doing it to a few so that they can do a large volume of those procedures and become truly expert at it. Awesome. In the first paper that we discussed, the risk of perforation was higher, starting with a 35 millimeter balloon.

What do you traditionally start with? I usually start with a 30 millimeter balloon, especially after seeing the data on perforation risk with larger balloons. If symptoms persist and the anatomy looks favorable, then I'll escalate, but I don't jump right to 35 millimeter anymore. Okay. Zach, let's go through some quick hits.

Who should get a poem? Certainly. So higher risk surgical candidates are more appropriate for pneumatic dilation or poem rather than a lap. Heller poem is also

ideal for patients requiring an extended esophageal myotomy, such as those with type three achalasia. Both a poem and a Heller Myotomy, however, require general anesthesia.

Is there any reason more patients shouldn't be considered for a poem? Anytime you perform a myotomy of the lower esophageal sphincter, you're putting the patient at risk for reflux. However, patients undergoing hell or myotomy may also have a concomitant fundoplication typically adore to create a reflux barrier.

Dr. Greenberg, when would you consider pneumatic dilation a failure? I. My personal view is that requiring serial pneumatic dilations constitutes a treatment failure compared to a Heller or a poem, which are really more of a one and done type procedure. I also wanna mention that, that we talk about poem being less risky for a patient with significant comorbidities compared to a lap Heller.

I think that's maybe a little bit overstated. Both of the procedures require general anesthesia, which is likely the biggest cardiovascular risk for these patients. And then once they're in the operating room, the risk of those two procedures are, are actually quite similar

For me, if a patient needs more than two dilations in quick succession, especially if they're younger and otherwise healthy, I lean towards offering them a surgical solution.

But if someone is older and has comorbidities, then dilation is reasonable, even if it's not perfect. Are there any new procedures or devices under consideration for future treatment of achalasia? There are a few, but all of them are still pretty investigational. Some endo endoscopists are studying combining poem with the fundoplication, either with a TIFF or an endoscopic suture gastric plication to create an anti-reflux valve.

Surgically, LES stimulation via laparoscopically placed electrodes on the lower esophageal sphincter combined with an implanted pulse electrode, similar to gastric stimulators for gastroparesis and gerd are also under investigation as well. Yeah, I'm really excited about where this field is heading.

Particularly with the rise of Endoluminal robotics, these platforms are gonna dramatically improve our precision and

versatility when working from inside the lumen, it opens up the possibility of combine combining therapies, imagine performing poem, and in the same session, adding an endoscopic anti-reflux procedure without ever making an external incision with current poem techniques.

GERD Prevention is a. Being explored with shorter myopathy links, sling fiber preservation, anterior versus posterior poems, notes, fundoplications and others outside the poem data. We've seen some intriguing early data with techniques like GE Junction, mucus ectomy, also known as arms or. Antireflux mucus ectomy.

One small study showed that nearly two thirds of patients were able to come off PPIs at six months, and it was done safely without major complications. Also, with robotics, adding better control and ergonomics, I think we're moving towards a future where we can offer truly tailored and su incisionless therapy for achalasia, including not

just effective symptom control.

But also built-in reflux prevention. That's a big leap forward, and it's exciting to think that we're not that far off. Awesome. There are some very cool treatments potentially coming in the future. So before we finish up, Dr. Greenberg and Dr. Portier, any final thoughts on the research involving Achalasia that we've talked about today?

No, I mean, I think this is a very hard disease to do meaningful research on, particularly level one. Randomized controlled trials because accrual is a challenge. This is a very rare disease that affects I think one in every a hundred thousand patients or so. And so to accrue patients. For the European ACHALASIA trial, it looked like the period of accrual was five years over multiple countries in multiple different institutions.

So it's a very hard disease to study in a meaningful way. And because it is a low volume procedure overall and there's a great degree of variability amongst techniques that surgeons are doing, it's hard to get an adequate end of any one good technique and make that reproducible across centers in a way that can be

studied meaningfully.

I. So I, I think the one, one of the other important take home points of this 10 year outcome study is, is the recognition that achalasia is really a lifelong disease, right? Even though we try to cure it, we're not actually curing anything. We're palliating the symptoms of the disease process. And it's a lifelong problem that patients may have to deal with down the road no matter what form of treatment we pursue initially.

So being open to. The fact that this is a chronic illness and treating it as such with intermittent dilations if needed, or anti-reflux medications, if they get GERD, is gonna be part and parcel of this patient's long-term care. So having a meaningful discussion about this being a lifelong disease is important to have with your patients.

So keeping that in mind, are there any treatments that you think burn bridges in anticipation of future treatments that these patients might need? Not really. I mean, there are some people who believe that that Botox can make a poem or some other procedure harder afterwards, but they can certainly still be

done.

So I think you should have a meaningful discussion with your patients about what their goals of care are and what procedure might best align with those goals. Let's finish up here with a quick case example. A 45-year-old woman presents to the office with a one year history of progressively worsening difficulty swallowing food.

Initially, this was just with solid food, but over the past few months, this progressed to trouble with liquids as well. She reports feeling the food getting stuck in her chest, and also describes regurgitation of undigested food sometimes even at night while she sleeps as well as coughing fits. She's lost 15 pounds over the past year and has chest pain and heartburn associated with her symptoms.

She presents with workups she had with her gastroenterologist. An esophagus was performed demonstrating a bird beak appearance of the distal esophagus with proximal dilation and no masses. Her gastroenterologist performed an EGD, which showed normal mucosa, no strictures or obstruction, but a tight, lower esophageal sphincter with retained food.

Random biopsies were benign.

Great. What should we do next? We'd get high resolution esophageal manometry, which in her showed absent peristalsis in the esophageal body in complete LES relaxation and increased resting pressure, which makes this, which type of achalasia Type two achalasia. Exactly. What would you offer the patient?

I. We offered the patient either a poem or hell or Myotomy, and given her young age and lack of additional comorbidities, she elected for a lap Heller with a D fundoplication. She did well postoperatively, was maintained on clear liquids for a day, progressed to full liquids the day after, and was maintained on this until her two week postoperative visit, after which she was cleared to advance to a soft diet and eventually gradually advance at home.

She was seen by her PCP six months postoperatively, who noted her symptoms had resolved, and she was quite happy with the improvement in her symptoms as well. Okay. That's all we have for today. Thanks so much for joining us to talk about achalasia. Stay tuned for the next MIS episode so that you too

can dominate the day.