What's up Ninja Nerds? In this video today we're going to be talking about esophageal motility disorders. Before we get started I really urge you guys to take a second go down the description box below check out the link to our website where we got some great notes and illustrations that I think are crucial to understanding this topic as we go through it together.
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Hopefully we can continue to help out other students as well. But without, you know, talking anymore, let's get to talking about esophageal motility disorders. So with esophageal motility disorders, it's super, super basic.
The esophagus is having a problem being able to peristaltically move things like food and fluids down the esophagus into the stomach. Now, if we really were to take a chunk of this esophagus and zoom in on it, really when we talk about motility disorders, it's a disorder of two things. It's a disorder really of the nerves that are supplying the esophagus, or it's a disorder of the muscle that is within the esophagus.
So it's either a disorder of the two things, the nerves, or the muscles of the esophagus. Now, if you cause muscular or neural dysfunction of the esophagus, what's the predominant feature that you'll see in these patients? The predominant feature from, again, neuromuscular dysfunction The primary feature that I want you to associate this with is going to be dysphagia.
So it's going to be difficulty with swallowing. This is a super, super common problem with neuromuscular dysfunction of the esophagus. Probably the...
most common chief complaint. Now the dysphagia is actually even a little bit more particular because it's for things like solids and liquids. Now, to take a quick second here, I need to talk about this because this will come up with one of the actual types of esophageal motility disorders. These nerves, so here we're actually zooming in at the bottom part here, this red is the lower esophageal sphincter.
It's a muscle, so if you have a dysfunction of that, obviously you're gonna have problems with this disorders. But nerves innervate this. And these nerves, I'm just zooming in on these here, they release something called nitric oxide and vasoactive intestinal peptide. You know what these chemicals are supposed to do?
These chemicals are naturally supposed to inhibit. this muscle. It's supposed to relax it.
So if you damage the nerves, what do you do to the nitric oxide in the vasoactive intestinal peptide? If you damage these nerves, you'll decrease the VIP, you'll decrease the nitric oxide. And so subsequently, what will you do to that actual muscle? You'll stimulate the life out of it and it'll be super contracted in high tone. There's one disease that can do that.
So the disorders of motility disorders of the esophagus is either neural dysfunction. such as this or muscle dysfunction such as the smooth muscle of the actual esophagus is jacked up. We'll talk about that in more detail. But the things that I want to go into is what are some of the scary complications of esophageal motility disorders.
Well we know dysphagia is one big thing but this may seem weird but you have a very high risk of esophageal cancer with these motility disorders. So if esophageal cancer Now, with the esophageal cancer risk, this is usually particularly a two type of disorder cause. One is, we'll talk about these later, but one I want you to think about and I know it's called achalasia.
Achalasia can definitely increase the risk of esophageal cancer. And the other one is esophageal scleroderma. You'll get their mechanisms a little bit later, but I'm just kind of cuing you in that the risk of esophageal cancer goes up in achalasia. and in esophageal scleroderma.
The next one here is probably pretty obvious. If you can't move food or fluids down, right? This is inhibited because the muscles or the nerves are all jacked up. Where is the food and the fluids gonna go?
It is going to pop right into the airway and you definitely increase the risk of aspiration. So aspiration risk goes up. And so these are the other things that you really wanna.
to be careful of in patients with esophageal motility disorders. Alright, now what I want to do is, I want to go through the patients who come in who complain of, man I'm having really difficulty with swallowing, and because of that I can't swallow foods, I can't swallow liquids, you want to figure out what is the cause of that. Let's talk about those. Alright, so esophageal motility disorders, it's a problem with nerves and muscles. Now, one of those I mentioned is a real specific problem with the nerves, and that's achillesia.
Achalasia is a very interesting motility disorder. Out of all of these, it's probably the most important one and the one that you'll get tested on. The mechanism behind this is actually kind of important. So you see these nerves here that I'm representing in purple? These are the ones that are damaged, but I want you to focus really hard in on the area of the lower esophageal sphincter.
So the problem with these is you have damage to the myenteric plexus. That is what happens here. So there is a decrease, if you will, will.
In which particular structure? The myenteric plexus. Now, if you decrease the function of the myenteric plexus, you have less what chemicals?
Nitric oxide and vasoactive intestinal peptide. If you have less of these, can you have a actual, what do you do to the actual lower esophageal sphincter now? Do you actually increase the tone or decrease the tone?
Well, naturally, these are supposed to do what? Inhibit them. But now, you're having less of these, so you're actually going to lead to an excessive stimulation.
So you're going to increase the lower esophageal sphincter tone. Now, what's the problem with that? If you increase the lower esophageal sphincter tone, you can't get a dang thing through this esophageal sphincter. Everything upwards, the pressure rises. And so you're going to have a super high pressure in the esophagus.
And what do you think it does to this poor wall? It dilates this wall. And then as you dilate the wall of the esophagus, guess what else you do to the actual mid and distal esophageal motility? Over time, that high pressure will decrease the esophageal motility of mid and distal portion.
So another thing that you have to watch out for for this one is that not only will it lead to this, but you'll also have, these are the two things that you will have. Again, you'll have a high lower esophageal sphincter tone. and you'll have decreased motility of the mid distal esophagus and that's because of this crazy high pressure now achalasia one of the ways that they try to mess with you on the exam is the causes oftentimes it's idiopathic we don't know but anytime they can add in a crazy crazy thing they will and so one of the secondary causes of achalasia is a nasty bug it's called trypanosoma cruzi remember that one Now, Trypanosoma cruzi can lead to a very classic triad.
The triad is megaesophagus via achalasia, megacolon, and dilated cardiomyopathy. That is the triad. Remember that. All right, the next one here is diffuse esophageal spasm.
This one's very interesting. All right, so I want us to kind of go with this kind of constant flow here. What is it doing to the tone of the lower esophageal sphincter?
What's it doing to the motility of the mid-distal esophagus? Thankfully, there's no effect on the lower esophageal sphincter tone. It's completely normal, so remember that.
But what is wrong with this disease is, look at this, and this one is super dilated and super tight, and this one you're contracting all over the dang esophagus. The motility is super hyper contractile. You have an insane high motility of the actual mid-distal esophagus.
But the problem is, is the motility is super, super disorganized. And that's why you get dysphagia and an intense chest pain. So again, very, very high motility of the mid-distal esophagus, but it's unorganized. Okay, my friends.
So with a diffuse esophageal spasm, intense contraction of multiple areas that's just organized within the esophagus, super highly motile. But the problem is, is that it's not organized. And then again, lower esophageal sphincter tone is completely normal. What's the mechanism behind this?
We don't really know why the esophagus just randomly contracts in particular areas. No idea. But this is the particular things that you want to take away from the mechanism.
Let's come away with the last one here. And this is esophageal scleroderma. In this mechanism, it's actually thankfully very, very easy. You have atrophy and fibrosis. of the entire esophagus.
That includes the lower esophageal sphincter and the mid-distal esophagus. So if you atrophy and you fibroze the actual tissue, what's going to happen to the tone and what's going to happen to the motility? They're both going to drop.
So in this one, you'll have decreased lower esophageal sphincter tone and decreased motility of the mid-distal esophagus, all due to atrophy and fibrosis. So again with this patient who has esophageal scleroderma look for atrophy fibrosis of the entire esophagus. Low tone, low motility of that mid-distal esophagus.
Now with this we actually do have a particular cause. It's usually associated with scleroderma and this is usually can be systemic but there's what's called a limited cutaneous form that they love to ask on the exam so you want to really associate this one with what's called sclerosis the limited cutaneous form of scleroderma You know what we call this form? We call it Crest Syndrome.
We call this Crest Syndrome. So you actually really want to remember this and associate this with what's called Crest Syndrome. Now, what does Crest stand for? I'm glad you asked.
Crest stands for calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia. If a patient presents with any of these Crest features, right, you really want to be thinking about the limited cutaneous form of scleroderma. Again, think about Kress syndrome. Calcinosis, Raynaud's phenomenon, esophageal dysmotility, that's where you're getting this problem here, and you also have sclerodactyly and telangiectasias.
All right, my friends, that covers the causes and the mechanisms of esophageal motility disorders. Now what I want to do is I want to take a diagnostic approach into these actual disorders. Let's talk about it. All right, my friends, we come into the next part here.
So we talked about three esophageal motility disorders, achalasia, We talked about diffuse esophageal spasm and scleroderma. We know the basic pathophysiology with respect to the motility and the lower esophageal sphincter tone. We even know a little bit about the issues and complications and their classic presentation. How do we diagnose a patient who comes in with esophageal dysphagia that we think is due to an esophageal motility disorder? Well, first thing, it's always good to get a barium swallow.
A barium swallow will give a little bit of a kind of an overview look. It's a nonspecific test, but it may help us. So what are some things that we may see with respect to these three disorders?
Well, the first one that you may see in your exam as a buzzword term is what's called a bird's beak appearance. So because in patients who have what's called achalasia, their mid-distal esophagus doesn't contract as well. So it's a little bit here, you're not really seeing the indentations from it contracting as properly. But on top of that, here at the end, distal part here, the lower esophageal sphincter, that tone is insanely high.
And so it's a really tight sphincter. And because of that, all of the esophagus proximal to that dilates. And so it kind of gives this beak appearance on the actual barium swallow study.
All right. So you look for a dilated esophagus with a very narrow tapering that represents the beak, such as we see in patients with achalasia. The next one is what we call diffuse esophageal spasm.
Whenever you hear the word corkscrew appearance, I really want you to think about diffuse esophageal spasm. You can see here how there's this uncoordinated high amplitude contraction of the mid and distal esophagus. It's completely uncoordinated, very intense contractions, as you can see with the contrast filling here. And then the lower esophageal sphincter, the tone's relatively normal, so we're not getting this massive proximal dilation from that. That really makes me think about this one.
Usually for scleroderma, it's actually not very helpful. So it's relatively a normal kind of barium swallow, and we don't go too crazy on that one. Now, sometimes when patients have a barium swallow and it appears relatively abnormal or even normal, we really want to go to an EGD with biopsy.
Just because we want to exclude that there's not a structural cause, we also want to exclude that there's not some type of cancer, especially since achalasia has a relationship with that. So I think it's important to do this to rule out malignancy in achalasia. But oftentimes it's relatively normal. And what we see in a diffuse esophageal spasm, if you did obtain a biopsy of the esophagus, you'll see a lot of muscle atrophy and fibrosis that could be suggestive of scleroderma, but I wouldn't go to the bank with that. All right.
So EGD with biopsy in a patient with esophageal motility disorders, I would say the only true indication for this is trying to rule out malignancy and that's it. What's the main bang for your... buck to determine the true diagnosis, the gold standard diagnosis for esophageal motility disorders is manometry. So esophageal manometry is where they take this probe, they move it down the esophagus, and they measure the actual kind of the amplitude of contraction via pressures at the different points of the esophagus, the upper, the mid, the distal, and even at the lower esophageal sphincter.
So what we can do here is we can say, oh, that pathophysiology that Zach was talking about and harping on. I can actually understand this now. Let's go through this. First one's achalasia. What do we know?
Well, we know mid distal is the main part here. I said mid and distal esophagus in comparison to normal pressure, it should be low, right? Because of the missing auerbach plexus. And then I said that the lower esophageal sphincter tone should be high in comparison to this normal lower esophageal sphincter tone here. All right.
Well, that would definitely help me to say that this is likely achalasia if they even have a combination of the bird's beak appearance and dilated esophagus on the barium swallow. Boom. Done. Diffuse esophageal spasm.
Oh, my gosh. Look at this. This is ridiculous. It's so obvious.
It's glaring at you. you can see high amplitude, very asymmetric, very uncoordinated, intense high amplitude pressures here in the mid distal esophagus. And then if you compare for the most part, the level here for the lower esophageal sphincter tone, it may have a more intense kind of contraction like here, but you'll see that it's at the same level relatively with the normal lower esophageal sphincter tone.
All right. And if you combine this with the high amplitude mid distal motility and normal LES tone with the corkscrew appearance on the barium swallow, you got yourself a pretty good diagnosis of diffuse esophageal spasm. The last one is scleroderma. So I'm looking here, I know atrophy fibrosis of the entire esophagus.
So mid distal should have very, very poor contractility in comparison to normal. They do. And then if I look at the actual level of this with respect to the normal lower esophageal sphincter tone, it's below that actual pressure.
So I know that this is going to be a decreased lower esophageal sphincter tone. That's suggestive that it's scleroderma. If you add in the fact that there's a normal barium swallow, you add in the fact that relatively the EGD is not super helpful, then I would say that this patient has scleroderma. Add in the fact that they have the Kress syndrome findings. And then oftentimes you'll go down the lane of testing them for antibodies, which we're not going to talk about here.
We'll talk about more in the rheumatology section. All right, my friends, that's how we would use manometry. And that's how we would go about diagnosing these esophageal motility disorders.
So we've diagnosed each one. We figured out the pathophysiology, the causes for the most part, the issues that we could see or the complications that could arise. How do we treat these? It really depends.
It depends upon the type of esophageal motility disorder they have. So with achalasia, the problem is the tone of the lower esophageal sphincter is where it's really the issue. That's the problem. So we want to try to be able to relax the lower esophageal sphincter.
And ways that we can do that is we can try to give them medications. So the first one is calcium channel blockers. One of the most common ones is usually amlodipine.
And amlodipine will block calcium entry into the smooth muscle. Calcium is needed for smooth muscle contraction and contraction in general. So because of that, I'm not going to get a good contraction of the smooth muscle.
And as a result, the tone of the lower esophageal sphincter will go down. It'll open it up and allow for food and fluids to move into the stomach. That'll definitely be a very big help in patients with achalasia.
So calcium channel blockers are going to be one of your first go-tos. Another alternative is nitrates. This is usually more long-acting, not short-acting like sublingual nitrate.
We do long-acting like isosorbide dinitrate would be a preferred drug here. So these would be preferred medications that we would use more in mild disease, so mild achalasia. or patients who really aren't a candidate at this moment for having a surgical type of intervention.
All right. Now, the next thing I think that's important to remember is that in patients with severe achalasia, so they have very profound dysphagia, maybe they even have some associated weight loss from it. Maybe they've had some complications from it, such as aspiration.
or they have not responded to medical management such as calcium channel blockers, nitrates, and even Botox, which we'll talk about next, they may be a candidate for what's called pneumatic dilation. And this will give you about 10 years of control. What they do is they take this probe, they move it down through the esophagus, they go over here during this tight lower esophageal sphincter, they inflate a balloon. When they keep that balloon there for a certain amount of time, maybe 30 seconds to a minute, and what it's going to hopefully do over that time is open up that very, very tight area of the lower esophageal sphincter and allow for food and fluid to move easily from the esophagus into the stomach so it can restore flow.
And this may give you about 10 years. We usually prefer to do this in patients who are oftentimes greater than 60 years of age and they don't really want to undergo a very invasive surgery that we'll talk about next. If the patient's less than 60 years of age, they're a good surgical candidate and they have severe achalasia that's maybe not responding to medical management, such as calcium channel blockers, nitrates, or maybe they've had pneumatic dilation and they haven't had success, we can do something called a Heller's myotomy. So a Heller's myotomy is basically where we'll take and go and cut over the lower esophageal sphincter. And if you cut it, you're kind of taking away the contractile portion of it.
And so essentially, you reduce the tone of the lower esophagus and open up. up that sphincter area and allow for food and fluid to easily move down and restore flow. Again, usually this is for patients who are less than 60 years of age.
A good surgical candidate Maybe they failed medical management and they want to go the route of being a little bit more invasive because they're a little bit younger. The last one is botulinum toxin. Botulinum toxin is really for patients who don't want to undergo any type of surgical therapy. They don't want to do pneumatic dilation.
They don't want to do a Heller's myotomy. They've tried medical therapy and they're just not getting a lot of benefit. A botulinum toxin is really for patients who don't toxin injection may give them about a year or two of kind of like success.
And so they take a probe, they move it down the esophagus and they inject botulinum toxin into the actual lower esophageal sphincter. And the way that this works is actually relatively cool. It inhibits some of those neurons, some of the of those neurons, particularly in the area of the lower esophageal sphincter.
And what happens is, is these patients will start to kind of decrease acetylcholine release, which will decrease calcium entry into the smooth muscle, decrease smooth muscle contraction, and lower that tone, open up the area and restore flow to allow for food and fluid to easily flow into the stomach. So that's the concept behind these therapies here is that achalasia, we can do medical therapy by relaxing the smooth muscle three times. types if you really want to think about them as medical therapy, calcium channel blockers, nitrates, and botulinum toxin, even though this is a little bit more invasive.
So we should consider it more of a surgical intervention. Pneumatic dilation, usually pretty severe disease, moderate to severe, but they're a little bit older and they're not a candidate for a heller myotomy. Heller myotomy, less than 60, good surgical candidate, maybe not benefiting from the medical therapies. And so we're going to pursue this.
Botulinum injection, usually not a good surgical candidate. They're not responding to the action. actual medical therapy, but they don't want to undergo pneumatic dilation or Heller's myotomy. So we'll do that and buy them a year or two. All right.
Diffuse esophageal spasm. Really the goal here is to try to relax that mid and distal esophagus. That's really the goal. And if you think about it, this is a great drug to help with these patients. We really don't want to do surgical therapy in these patients.
We can, but I say that in this patient population, they oftentimes respond very, very well to these calcium channel blockers. and nitrates. It's the same mechanism. It's in the mid-distal esophagus. We're going to give them these.
We're going to reduce the smooth muscle contraction there. Nitrates reduce smooth muscle contraction, and you're going to reduce the peristaltic activity in the mid-distal esophagus. And that's really good in diffuse esophageal spasm.
There is the ability to do the same thing. If they want to get pneumatic dilation, we oftentimes don't really do this a lot in patients with diffuse esophageal spasm. And a botulinum injection can also be considered sometimes in diffuse esophageal spasm as well. Again, the concept behind this is that you're reducing the smooth muscle contraction.
You would just inject it in a different portion of the actual esophagus, more the mid-distal portion. Scleroderma is the next one. Scleroderma is interesting because with medical therapy, that's really the only thing that we're going to do. This is an autoimmune disease. It's progressive atrophy, muscle fibrosis.
There's really no type of specific medical therapy that we'll do other than just preventing them from developing further esophagitis. And so oftentimes we'll just put them on PPIs. We don't really do surgical therapy for these patients because again, there's nothing that you're really going to be able to have benefit from this one. You're not having a tight... lower esophageal sphincter, and you're not really going to be doing anything to help the mid-distal esophagus.
One big tip that I would give you guys is that achalasia will benefit more so from aggressive surgical therapy than medical therapy. So it is better for these patients to undergo pneumatic dilation, Ehlers-Myotomy, than it would be to put them on things like calcium channel blockers and nitrates to have a more long-lasting effect and benefit from those. To diffuse esophageal spasm, it's actually better to be more or like non-invasive, they'll respond much more effectively to medical therapy than they will to any type of surgical therapy.
So it's really important to remember that. All right, my friends, this is esophageal motility disorders. I hope it made sense.
I hope that you guys enjoyed it. And as always, until next time.