Gut Check: Ken DeVault, MD, on Diagnosis and Management of Achalasia

Dr Ken DeVault of Mayo Clinic-Florida provides an in-depth look at the diagnosis and treatment of achalasia with Gut Check host Dr Brian Lacy.

Brian Lacy, MD, is a professor of medicine at Mayo Clinic-Florida in Jacksonville, Florida. Kenneth R. DeVault, MD, is Professor and Chair of the Department of Medicine at Mayo Clinic Florida.

TRANSCRIPT:

Any views and opinions expressed are those of the authors and or participants, and do not necessarily reflect the views policy or position of the Gastroenterology Learning Network or HMP Global its employees and affiliates.

Dr Brian Lacy:

Welcome to GutCheck, a podcast from the Gastroenterology Learning Network. My name is Brian Lacy. I'm a professor of medicine at the Mayo Clinic in Jacksonville, Florida, and I am absolutely delighted to be speaking today with Dr. Ken DeVault, past president of the American College of Gastroenterology and Professor of Medicine in the Department of Medicine at the Mayo Clinic in Jacksonville, Florida. Dr. DeVault has focused his career on disorders of the esophagus and is an internationally recognized expert in gastroesophageal reflux disease. One of his many other areas of expertise is that of achalasia, a condition first described in 1674 by Sir Thomas Willis. Not surprisingly, a lot has changed since then. And with that as a background, let's discuss achalasia past, present, and future.

So Dr. DeVault, welcome, what an honor to have you on our podcast today to set the stage for our listeners. Achalasia is considered the classic motility disorder of the esophagus. How common is achalasia and who is most likely to be affected?

Dr Kenneth DeVault:

Thank you for the opportunity. Achalasia remains a very rare disorder. Looking at the most recent reviews in meta-analyses, the incident rate is probably about 1 case per 100,000 person-years. Globally, maybe with the prevalence of around 10 cases per 100,000 persons in the United States, it may be a little bit higher and whether that's just we identify it more commonly or it's truly more common, and if you look at things like the Medicare database, that prevalence may increase up to 25 per 100,000 person-years. If you add that all up in the recent guidelines that would come to about 20 to 40,000 individuals in the US. It affects both sexes equally. It can occur at any age, but we're seeing a gradual increase with age as well. So still a pretty rare disorder.

Dr Lacy:

Thank you. And so thinking about achalasia, this classic motility disorder, what are some of the most common symptoms of achalasia?

Dr DeVault:

Well, the one we always look for is dysphagia or difficulty swallowing. It's an interesting symptom because I've had patients tell me, I swallow fine, it just won't get out of my esophagus. So you have to ask the question correctly because it really is a lower esophageal dysphagia, a regurgitation of undigested food or saliva. It should not be acidic because it's coming from the esophagus. A certain number of patients will have chest pain and some will lose weight. When I really start to worry about achalasia producing long-term damage is frankly if patients can't maintain their nutrition. And the other really kind of scary complication of achalasia is aspiration. If the food builds up in the esophagus and particularly at night gets into the lungs, it can produce difficult lung disease and is a time we really need to intervene.

Dr Lacy:

So thinking about some of these symptoms, a lot of them are fairly nonspecific, right? Symptoms of regurgitation and some reflux and some chest pain and sometimes this vague sense of things not going down. Do these nonspecific symptoms, does it lead to delay in diagnosis in some patients?

Dr DeVault:

Yeah, that's pretty common. I mean, the classic discussion was delay in diagnosis of achalasia from symptom onset of up to 10 years. I think it's less now because more people know about achalasia. The most common misdiagnosis is of gastroesophageal reflux—patients who feel the regurgitation. So it'd be natural for a physician to think that might be reflux and some of them will have come in having been treated for many, many years with acid blockers with no real improvement in their symptoms. Other diagnostic delays used to be lack of availability of esophageal manometry, which is the definitive test that we'll talk about in a little bit. So I think that as long as you think about it and people that aren't responding to acid blockers, that's a possibility and we'll talk about some of the testing later, that delay might be decreased, but I still quite commonly see people that have had symptoms for 5 years or more.

Dr Lacy:

This kind of progressive process and I think as we all teach medical students this progressive dysphagia first to solids and then to soft foods and then to liquids. So that's a perfect segue. Thank you.

So what is the best diagnostic test right now for achalasia? Is it still a time barium swallow where you drink the barium stand up and we measure you at 1, 3, and 5 minutes? Or is it high resolution esophageal manometry or should we be moving on and is it EndoFLIP right now?

Dr DeVault:

Well, I think when you're thinking about achalasia and you're thinking about a intervention that again we'll talk about later, it's probably good to have most, at least 2 of those, if not all of them. But if you're screening for achalasia, if someone has a normal appearing esophagus on barium that empties, well, they probably don't have achalasia. So if you don't have access, I think an esophageal manometry remains the gold standard and probably is required prior to any type of a significant intervention. The EndoFLIP’s an interesting new technology most everyone knows, but the EndoFLIP is a procedure that's done within an endoscopy that screens for disorders of secondary peristalsis. If it's normal and there's normal contractions that move from top to the bottom in the esophagus on flip, you don't have achalasia. So it can exclude the diagnosis. Now the vast majority of people that have an abnormal EndoFLIP don't have achalasia, so it's good to exclude it and I use that If I need to do an endoscopy on someone who I think vaguely might have achalasia and it's normal, then I can just put that aside. So I would still say a barium swallow is cheap in the right hands. A good test, you need to do a manometry before intervention and EndoFLIP. If you're doing an endoscopy and you've not done either of the other two and it's available and in the procedure you think it might be achalasia, it's a good way to exclude it if it's normal.

Dr Lacy:

Great. That's very helpful. And I think our listeners today, both providers and patients, making sure we get that diagnosis and with the right diagnostic test is really critical because interventions have some risks and downstream consequences, so we want to make sure we get it right.

So thinking about high resolution esophageal manometry and the Chicago criteria and all the great stuff coming out of Chicago and Northwestern, it's made us think about achalasia a little bit differently and we now subtype it into 3 different categories of type 1 or type 2 or type 3 achalasia. Why is that important? Isn't just achalasia, achalasia?

Dr DeVault:

Yeah, it's interesting. I think type 1 and 2, we always sort of did that. Personally, I don't think there's much difference in those 2centities in exactly what's happening. Type 1 is classic achalasia where the lower sphincter doesn't relax and then the esophageal body itself doesn't produce any kind of a contraction or pressure wave. I mean that would be the board test definition of achalasia, and we've always seen that. Frankly, the lower esophageal sphincter relaxation was really difficult to do prior to high resolution manometry. Quite frequently I diagnosed achalasia back then in a patient who actually looked like they had normal LES relaxation, but because of the way the catheter was designed, it would slip out of the LES and there were all kinds of additions that were added to catheters over the years to try to get a better look at the LES. But again, if the body of the esophagus was aperistaltic, there was a present LES and a barium test was confirmatory, we didn't concentrate on the LES relaxation as much high resolution manometry because it has multiple sensors across the LES has allowed us to do that.

Now why do I think type 2 is like type 1? I think type two is simply type one with a water column within the esophagus. So if you take a bucket of water and you put a manometry catheter in it and you splash some more water in it, you're going to see simultaneous contractions because pressure is transmitted throughout a water column instantaneously in essence. And in fact, sometimes we see patients who the first couple of swallows look like type 1, and as the esophagus fills up with liquid, they begin to look more like type 2. We used to call those Isobaric contractions, which really just means they're probably not muscle contractions, they're probably just splashing in the esophagus. There were some patients that we described as vigorous achalasia, maybe those were type 3.

Type 3 is the new kit on the block, and I think that might be why there's an increased incidence of achalasia, at least in our minds because it has really expanded the diagnosis and the things I think in the past we probably characterized as distal spasm or perhaps nonspecific disorder. One of the things I would remind the listeners is with the most recent versions of the Chicago classification, you still cannot have any peristalsis present and be declared either type 1, 2, or 3. Some of the earlier classification symptom systems, I think were a bit more permissive and may have resulted in some mischaracterization. Type 3 is different. We'll talk about treatment, but type 3 is more difficult to treat. And just giving a little look ahead, it may do better with POEM treatment, but I do think it's important to classify, although again, most of the data would say 1 and 2 perform very similarly to individual interventions where it's possible type 3 may do better with a longer myotomy.

Dr Lacy:

That's great. And again, a nice transition to talking about treatment. But before we talk about specific treatments, you've done this job for so long, you have 35 years of experience. When you sit down with a patient and you've made that diagnosis of achalasia, lead us down the pathway. How do you explain treatment options to patients? What treatment options do you talk about and what other factors come into play if they're a man or a woman, if they're young versus old, how do you flavor that discussion?

Dr DeVault:

I appreciate that question. I think the first thing I do is I spend a lot of time making sure I have all the studies, and at least the majority of those studies are consistent with achalasia. If they come to us at Mayo Clinic and they've had a manometry somewhere else, I prefer to actually get the computer disc with the tracing on it so I can look at it myself. At least the color photographs can be helpful. Again, barium testing, it's one of those things that if you're at a small hospital that rarely does them, you might not get the data you want. So really the first thing I do is dig through the record, sit with the patient, and make sure there's no significant inconsistencies with the diagnosis.

I also want to make sure that they've had a good endoscopy by a good endoscopist with a careful retroflex view of the lower esophageal sphincter, because I'd say in my career I've probably seen 5 or 6 unexpected esophageal gastric tumors that came to me for achalasia therapy, but it's pretty rare, but it's part of the guidelines. It's something you need to do. So again, if you're a tertiary doctor like me and you trust your outside endoscopist, which if I know them I will, then I'll know that it's probably okay if they've had a good retroflex view.

The other thing I always tell my patients early in our interaction is achalasia is not a curable illness. We palate achalasia by improving the LES opening. It's rare, I used to say never, but maybe close to never that peristalsis returns in the esophagus. So it's not surprising that it's not a one and done situation, and I think that's a key factor. I always tell them, I can help you a lot. I can make your life way better, but will this go away forever? I can't promise.

Then I start looking at which thing are we going to talk about? First question in my mind is, are they fit for surgery? That influences both my endoscopic therapy, which could have a complication and their surgical therapy. If they're not fit for surgery, we talk a little bit about Botox, we're going to talk about the treatments in a minute, and that's really the key things. Do they have the disease? Have we excluded other things? The other things you really have to make sure of are a few things that actually over time have gotten more common— narcotics because if a patient's on chronic narcotics, they can have a manometry that looks just like a collision. You may have to actually treat them like achalasia if they're never going to stop their narcotics, but it is an issue. Bariatric surgery is another area where you can get an achalasia-like syndrome most commonly with gastric lap bands, particularly when they're too far proximal. Also though with gastric sleeves. So that's something I want to make sure. And then I often in my mind is an 85-year-old with achalasia the same thing as a 35-year-old with achalasia? I don't think they are, but I think that's one of the things that I try to look at, try to take the patient as a whole before we start talking about which therapy they might want. And with the internet and Dr. Google, they often have decided what they want before they come to see me.

Dr Lacy:

Our friend, Dr. Google. But this is really important. This is not a cookie-cutter approach. You really need to personalize your treatment based on the patient, the patient's comorbid conditions, and maybe even their expectations. So thinking about therapies, in my introduction I mentioned Sir Thomas Willis who used a whalebone tip with a sponge to dilate patients with achalasia over 300 years ago. What's the role for dilation now and do you have any tips for our listeners on how to make dilation more effective?

Dr DeVault:

Well, I would say that it's dang hard to get whale bones anymore, but anyway, not that I would use that, but it was interesting. He tied a rag on the end of it and I'm not sure it was a real dilation. I think he was just doing plunger work by just pushing the food down.

Dr Lacy:

Yep.

Dr DeVault:

First thing I think all endoscopists need to know is that standard 20 millimeter dilation probably doesn't do much with achalasia. It's worth a try when you're doing an endoscopy, I think we've used savary vs Maloneys or 20 millimeter balloons. They probably don't make a difference. Now in the previously treated patients, I actually use those sometimes to make sure it's not a mucosal stricture that's present rather than a recurrent achalasia, but generally it's not going to get much. I would say if it's a rare enough disorder that if you're not comfortable with pneumatic dilation, I probably wouldn't do it. I would probably refer them. I can tell you there was a study came out a while ago and I've been doing pneumatic dilation the same way for 35 years. Don Castell before me did them the same way. So we're using like a 70-year-old technique maybe or something like that.

So actually for this new study from Europe came out, they did them a little differently. I actually called all my buddies around the country to make sure I hadn't missed something and I hadn't. Dilation has been a long-term thing. So if somebody's going to do that, we had a lot of data about the time I was a fellow in the early nineties on how to do it safely and effectively. First 30 millimeter balloons are the place to start. You don't need to start with anything bigger than that. I've occasionally used a 35 millimeter balloon if they previously had partial response. I don't actually remember ever using a 40 millimeter balloon. I might've, but I can't ever remember doing that. So 30 millimeter balloon.

I do it the traditional way. I localize the lower esophageal sphincter using endoscopy and fluoroscopy. I think fluoroscopy is really important because these balloons move whenever you inflate them and I keep the fluoro on the whole time I'm inflating the balloon, not because of any other reason than that it can move. It is a guidewire technique, so it's not a TTS balloon. So you place a guidewire under endoscopy and fluoroscopic control and place the balloon centered in the LES, which you've identified endoscopically. the inflation. You really just really need to inflate it until the indentation or what we call the waste and the balloon is gone. It originally, I say it looks like a dumbbell and then when it pops open it looks like a sausage. I don't know why I say that, but anyway, that's what I say. The data from the University of Pennsylvania really guided this for us. I usually try not to go above 12 PSI and again, there's no need to hold it for a minute. We used to do that. So 12 PSI for 15 seconds, deflate the balloon when it comes out, if it's got blood on it, that's a good thing.  The first few times you do it with a nurse, it really freaks 'em out and I say, Nope, that's good. There's blood on there.

And current guidelines actually do not require an esophagus after dilation, but I still do it routinely. I actually hate to say this because I've only got a little while longer in this job before I go somewhere else. I haven't had a perforation in a long, long time, so I'll probably have one tomorrow, but I still feel like it's important to know if they have a leak before they go home. I've had patients who had contained mucosal perforations that I keep in the hospital overnight. We used to hospitalize everybody overnight. We let them go home. The other thing is if in our practice where people travel, I ask for them to stay in town one night so they don't go back to a community hospital because if you do have a perforation from pneumatic dilation, you want to have a laparoscopic or chest surgeon available that can fix the perforation and ideally actually do a myotomy at the same time. So usually we've got enough here that it's okay. I used to whenever we only had really one surgeon that would do this, would not do the pneumatic dilation if he was on vacation. So I think I'm a little paranoid compared to everybody, but I still follow the technique that I learned 35 years ago and it's served me well to this point.

Dr Lacy:

Well, that's why your patients are lucky to have you doing this and so many great teaching points that you've just covered. But for our listeners, if you can visualize kind of this waist, so an hourglass figure, and as you inflate the balloon, you see that waist disappear. And as Dr. DeVault nicely pointed out, if you don't have a little bit of blood, it probably means you didn't get the tear you wanted and that's not scary. It's appropriate.

So let's think about Botox. So Botox injection for achalasia was first performed over 30 years ago in 1994, and now we've got 30 years of data. Is there still a role for a Botox injection of the LES for Achalasia?

Dr DeVault:

I mean, it's my preferential treatment for a patient who I don't think is very fit for other treatments as far as more, I would consider them more definitive. And in placebo-controlled trials for documented achalasia, Botox did exceed placebo in response. Dr. Pasricha, who's the chair of medicine in Arizona at Mayo now, was the original person who did that. I started doing them very shortly after that. I actually had to go through our IRB because it was said to be an experimental technique and I had to consent. everybody like that for the first several years.

That having been said, if somebody's fit for other things, I don't really do it. The other thing that I don't like is using Botox as a test to see if they get better before a prior treatment. We don't know if Botox makes it harder to do more definitive therapy, and most people now say it's not the case. But you will occasionally somebody see someone that gets a good bit of fibrosis from Botox injection because we don't have great control. Some of it we squirt walk through the wall, we don't know where it goes for sure, but it's still a reasonable thing for people that either don't desire more definitive treatment I have those patients or that they're unfit for more definitive treatment.

Dr Lacy:

I like all those points and for some people, maybe even if they're just not a risk taker at all, and they don't want to talk about the risks of pneumatic dilation or surgery.

So POEM—P-O-E-M, per oral endoscopic, it was first performed in Japan in 2008, so about 17 years ago, and it's really revolutionized the treatment of achalasia. Which patient is the right patient for this procedure and what's the success rate?

Dr DeVault:

Well, Dr. Lacy always knows the literature better than me, but I know the literature on this one okay. If you look at Heller myotomy, POEM, and pneumatic dilation, those are the 3 more definitive treatments and you really look at all of the randomized controlled trials we have, they're pretty darn close, and a lot of it depends on the study technique and the definition. And I would say that's the case for type 1 and type 2 achalasia. And when I say effective, they're probably have a 90 to 95% initial effectiveness of relieving dysphagia. Again, if it's some of the more atypical symptoms, chest pain and things like that, that's not going to be that good. And that is in type 1 and 2. So when I counsel a patient, I talk to them about all 3. There are some data that would indicate younger patients do better with one of the cut techniques, Heller or POEM as opposed to the pneumatic dilation, particularly for some reason young males— I don't really know.

So younger people, I tend to discuss the Heller myotomy and the POEM. In well-established type 3 achalasia is probable, it's possible and probable that POEM’s a better technique. The reason is that with a Heller myotomy, which is currently done laparoscopically, you can only cut about 3 or 4 centimeters up into the esophagus where type 3 is probably a little bit of a distal spasm mixed with LES dysfunction, you need to get a higher myotomy. So I think that's why the type 3s are more effectively treated with POEM. I'm sending more and more patients for POEM. We'll talk about some of the downsides in a few minutes.

I will say that I'm hesitant recommending any manometry in type 3 unless I'm pretty sure they have it. In that situation, it would include a manometry confirmed with at least an abnormal EndoFLIP or a timed barium esophagram and ideally both. I ask a lot about narcotics, make sure the symptom’s dysphagia, not chest pain because chest pain may not get ready and have the patient understanding that with type 3, regardless of which of these approaches, the outcomes are a little bit worse.

Dr Lacy:

And I like all of what you said, but I also like the point that you have to be able to counsel the patient that maybe not all symptoms will go away because some of these other symptoms may not really accurately reflect drainage of the esophagus that may improve with treatments directed at the LES. So you kind of set the stage again so nicely, POEM, per oral endoscopic myotomy is not without its downsides. What are some of the side effects or complications from poem?

Dr DeVault:

Well, I think poem can occasionally be rolled out in centers with not enough experience. And by that I mean it's probably not something you can go to a hands-on session at a national meeting and come away knowing how to do. I mean, I really would not really…You have to ask yourself, is that the level we need to train for this? So it is a complicated technique, not that Hellers aren't, and the one thing I didn't say about the choice of treatments is I think it matters who's good at it at your center, if you've got somebody with good experience at pneumatic, you may do more of those than POEM and Heller.

But the downsides of POEM, there are technical issues during performing the procedure. Perforation is one of those. Our group does a lot of studies including bariums and things to rule out a perforation. If you identify a perforation during the performance of the POEM and you put an endoclip on it, they do okay. If the patient has a perforation that shows up at 3 in the morning after they've had a morning POEM, they might not do okay because they're going to get mediastinitis and all the complications of that. So I think having a good technique and a good protocol where you're doing appropriate air insufflation, things of that nature, and again, it's probably good to have a surgeon at least somewhere around in case something goes astray.

One of the problems that we occasionally get into, again, I would hate to keep saying this, in less experienced hands, is an incomplete LES myotomy that the lower esophageal sphincter is not just an esophagus thing. It has a significant contribution by the sling fibers and other fibers in the proximal part of the esophagus.

So traditionally, a myotomy should go down 2 to 3 centimeters along the curve of the stomach. So if you don't get all the way down and you cut the body, the esophagus, you really set up patients for what we see in a quaternary practice, like we're in, a blown out myotomy, which basically means you did the myotomy but you didn't relieve the LES, and that is a disastrous outcome. So really it's a matter of making sure they get far. So those are the complications.

Patient selection—they don't have achalasia, they're not likely to do well. I guess that's one of the things. POEM compared to Heller myotomy with partial fundoplication has more reflux, acid reflux, because you don't do an antireflux procedure, I would say if you do pH testing, and we routinely at about 6 months do an endoscopy with a Bravo pH test, about half the polling patients have a significant reflux.

So when I counsel patients, if they're afraid of PPIs, I tell them, this is not a great idea. You at least have a 50% chance of being on lifetime PPIs, which isn't bad. I’d take that chance, I'm not afraid of them, but patients are often afraid of them. So post myotomy reflux, and I guess one of the things I would emphasize to the listeners is be really careful of isolated EGJ junction outflow obstruction because it's not clear that does great with POEM and it's actually not even clear. It does great with Botox injection. So if they have a normal peristalsis and you're worried about their EGJ walk carefully, that's all I can say because they don't necessarily do well with either procedure.

Dr Lacy:

Thinking a little bit about surgery, and you've kind of mentioned this a couple of times, the open Heller Myotomy was first performed in 1913, so we've over a hundred years ago. You've kind of mentioned there's still a role for it. Who is the best patient you think? Where would you recommend at this time a laparoscopic Heller myotomy?

Dr DeVault:

So when I first started as an esophagologist, Heller myotomy—and these were done with a thoracic incision— a lot of my patients had severe pain. Sometimes they had to take a rib out to get there. I mean, it was a difficult surgery and we did a lot of pneumatic dilations back then because POEM didn't exist, Botox didn't exist, and we did everything we could do to keep those patients from having to have a thoracotomy, because those are not a piece of cake for most people.

The advent of laparoscopic fundoplication was—I was already on staff here at Mayo, I would say early nineties was whenever that came over and it made a huge difference. So which patient do I push towards Heller if they're afraid of reflux, it won't take a PPI. They probably should have a Heller with a partial fundoplication because really a Heller and a properly preferred point is the same surgery. You just get the fundoplication with the Heller.

I think that up until recently, I pushed more patients in that direction because until probably when Dr. Kumbhari came 4 or 5 years ago, we really didn't have super expertise in the POEM procedure in our center. And I don't think it goes well unless you have a dedicated group that do several per month hopefully or more than 1 or 2 a year. So if your center doesn't really have an advanced endoscopist, well-trained in POEM, you're better off to have your surgeon do a laparoscopic Heller. But again, if they don't do many laparoscopic Hellers, if they're a cardiac surgeon, that's mainly a coronary artery surgeon and way back in training somewhere they saw a Heller, again, rare disorder. Some of the good outcomes in Europe frankly are because only a few hospitals are allowed to do these things and everybody goes, I think there's one hospital in the Netherlands that does all, and sometimes I know that's not the way we do thing in America, but it does come out to a better outcome.

So this is a rare enough disorder. If you're only doing 1 or 2 a year of any of these things, it's probably better to refer. And if I went to a place and I did not have a good thoracic surgeon, I'd be hesitant to set up an achalasia program just because I need to have my complications dealt with as well. So there's a role, fear of reflux, expertise. It's still a really good procedure, and I would say it's still a gold standard, but again, the advantage of POEM is a quicker recovery. Although our patients do stay in the hospital one night, which is the same they stay for a laparoscopic Heller, maybe less scar tissue in the abdomen and things like that, and you don't burn a bridge if you do a POEM on a patient, they have bad reflux. You can go back and into a partial fundoplication on if you need to.

Dr Lacy:

Wonderful. So Ken, we've discussed the achalasia of the past and achalasia the present where we're now, but where we going in the future with the evaluation and treatment of achalasia? Where do you think we're headed?

Dr DeVault:

That's really frustrating. I think when I was in medical school in the 1980s, I think the lecture on Achalasia said, we don't know why people get achalasia. And somebody asked me, we don't know why people get achalasia. I think it would be nice to know. I mean, there's a disease in South America called Chagas disease. We kind of know that, and we assume they're somewhat similar, that there's some autoimmune thing. Achalasia is a neurodegenerative disease. By that I mean that the nerves that cause the lower esophageal sphincter to relax, preferentially disappear in patients with achalasia. I don't know why, but it definitely happens. A lot of theories over the years, and I won't go into those. So it's a neurodegenerative disease. You would think stem cells would be something you could do to regrow those nerves, and there's not really been tried very much. One of the problems is there's not an animal model for the disease. Animals—one thing people don't know about me is I'm an alpaca farmer. They get megaesophagus. Sometimes we think their megaesophagus comes from repetitive choking when they overeat, and they're a multistomach creature, so they've got a really weird upper GI tract anyway. But I mean, there are animals. Dogs get megaesophagus. It happens, but pathophysiologically, it's not just like achalasia. So it's been hard to come up with research on medical treatments that might make a difference.

That would be my hope is that we come up with some type of a gene therapy or stem cell therapy that might make a difference. That would require us to find the disease early before the esophagus becomes massively dilated. I hope discussions like this and people knowing about the disease will decrease the patients who present the clinic with a massively dilated esophagus that I know I'm not going to be able to help much. I saw one last week who had what we call a sigmoid esophagus where the esophagus actually goes down and then has to come back up to get into the esophagus. Its volume, the volume of esophagus was 3 times the volume of the person's stomach. So I can't really do much with that. Those kind of patients, again, need to be referred. You can consider an esophagectomy. It's an awful test, but I've had a procedure, I've had some patients do very well with it. There is a new technique called the serra doria procedure, which came from South America, and we've done some, which is essentially opening up the lower part of the esophagus and opening up the stomach and connecting the two without taking the esophagus out. And it seems to be helpful in these patients with gigantically dilated esophagus. But again, that's where you got to just find the right person who's interested in doing something innovative to try to help these folks. Again, as you're following them, if they're aspirating or if they're doing fairly well, that's okay, but if they're aspirating or losing weight, you need to really get aggressive.

Dr Lacy:

Great teaching point. So Ken, this has been a wonderful conversation. Any last thoughts for our listeners?

Dr DeVault:

Yeah. I will say that in practice, you may be following more patients that have been treated with achalasia. So some things to think about. Like I said, after POEM, we're routinely doing endoscopy and pH monitoring a few months after the myotomy. The risk of cancer has always been something that's been discussed in achalasia. Originally it was squamous cell cancer. I think most of those were incident cases, and it really probably was not something to screen for, and we really didn't have a screening protocol. Now we've seen patients develop Barretts and even adenocarcinoma after both Heller myotomy and POEM. So I scope my patients every 1 to 3 years after achalasia therapy, especially if they're not being treated for reflux. So I think you need to follow them, pay attention. The other thing that we believe, and I believe it, is that those patients with the dilated esophagus lose some sensation in their esophagus. The same thing--the sensory nerves don't work as well, so bad things can happen and you don't know they're happening. Again, I think that just not letting them disappear and keeping them in your practice is not a bad thing for these patients.

Dr Lacy:

Wonderful. Again, your patients are lucky to have you with all your expertise. And Ken, again, thank you so much for lending your experience and expertise on this important topic to our listeners on Apple, Spotify, and other streaming networks, I'm Brian Lacy, a professor of medicine at the Mayo Clinic in Jacksonville, Florida. You have been listening to Gut Check, a podcast from the Gastroenterology Learning Network. Our guest today was Dr. Ken DeVault, professor of medicine for the Mayo Clinic in Jacksonville, Florida. I hope you found this just as enjoyable as I did, and I look forward to having you join us for future Gut Check podcasts. Stay well.