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 also if you guys like this video it makes sense it helps you please support us by hitting that like button commenting down the coms section and please subscribe and keep telling people about us 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 dis 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 muscular or neural dysfunction of the esophagus what's the predominant feature that you'll see in these patients the dominant feature from again neurom muscular dysfunction the primary feature that I want you to associate this with is going to be dysphasia 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 dysphasia is actually even a little bit more particular because it's for food 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 esophagal 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 dysfunction of that obviously you're going to have problems with this disorders but nerves inate 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 dysphasia 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 uh type of disorder cause one is we'll talk about these later but one I want you to think about now is called acalasia acalasia can definitely increase the risk of esophagal cancer and the other one is esophageal Scleroderma you'll get their mechanisms a little bit later but I'm just kind of queuing you in that the risk of esophagal cancer goes up in acalasia and in esophagal 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 going to 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 want to be careful of in patients with esophageal motility disorders all right 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 all right so it's off 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 acalasia acalasia is a very interesting motility disorder out of all 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 esophagal spincter so the problem with these is you have damage to the mayic plexus that is what happens here so there is a decrease if you will in which particular structure the myant plexus now if you decrease the function of the myeric 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 sagil 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 Sage spincter tone now what's the problem with that if you increase the lower esophagal sphincter tone you can't get a dang thing through this esophagal 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 esophagal motility over time that high pressure will decrease the esophagal 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 acalasia one of the ways that they try to mess with you on the exam is the causes often times 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 acalasia is a nasty bug it's called tripanis cruzi remember that one now tranisa cruzi can lead to a very classic Triad the Triad is mega esophagus via acalasia megacolon and dilated cardiomyopathy that is the Triad remember that all right the next one here is diffuse esophagal 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 Sage spinter 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 hypercontractile you have an insane High motility of the actual mid distal esophagus but the problem is the motility is super super disorganized and that's why you get dysphasia 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 the diffuse esophagal 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 ized and then again Lower sofor tone is completely normal what's the mechanism behind this we don't really know why the esoph 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 a esophageal Scleroderma and 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 fibrose 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 a esophagal 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 L Limited cutaneous form of SC Scleroderma you know 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 ask Crest stands for calcinosis rainout phenomenon esophageal dismotility sclerodactyly and Tasia 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 Crest syndrome calcinosis rainout phenomenon esophageal dismotility that's where you're getting this problem here and you also have sclerodactyly and tangc tasus all right my friends that covers the causes and the mechanisms of esophagal 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 esophagal motility disorders acalasia we talked about diffuse esophagal spasm and Scleroderma we know the basic pathophysiology with respect to the motility and the lower Sagal Spiner 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 dysphasia that we think is due to an asoph motility disorder well first thing it's always good to get a barium swallow a barium swall SW would give a little bit of a kind of a overview look it's a non-specific 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 acalasia their mid distal esophagus doesn't contract as well so it's a little bit here kind of you're not really seeing the indentations from it Contracting as properly but on top of that here at the distal part here the lower Sagal 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 acalasia the next one is what we call diffusus sagos spasm whenever you hear the word cork screw 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 IND distal esophagus it's completely uncoordinated very intense contractions as you can see with the contrast filling here and then the lower esophagal 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 swap swallow and we don't go too crazy on that one now sometimes when patients have a Barum swallow and 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 acalasia has a relationship with that so I think it's important to do this to rule out malignancy in acalasia but often times it's relatively normal and what we see in a duus esophagal 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 esophagal 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 monom 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 esophagal 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 acalasia what do we know well we know mid distal is the main part here I I said mid and dis esophagus in comparison to normal pressure it should be low right because of the missing arbach plexus and then I said that the lower Sage sphincter tone should be high in comparison to this normal lower Sage Spiner tone here all right well that would definitely help me to say that this is likely acalasia if even have a combination of the bird's beak appearance and dilated esophagus on the barium swallow boom done diffuse esophagal spasm oh my God 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 sual sphincter tone it may have a more intense kind of contraction like here but you see that it's at the same level relatively with a normal lower Sagal sphincter tone all right and if you combine this with the high amplitude mid distal motility and normal Lees tone with the cork screw appearance on the barium swallow you got yourself a pretty good diagnosis of diffuses ofal spasm the last one Scleroderma so I'm looking here I know atrophy fibrosis of the entire esophagus so mid distl 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 sulage tone it's below that actual pressure so I know that this is going to be a decrease lower assage Spiner 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 do they have the crest syndrome findings and then often times you'll go down the L 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 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 that 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 esophagal motility disorder they have so with acalasia the problem is the the tone of the lower sulage 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 move into the stomach that'll definitely be a very big help in patients with acalasia 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 acalasia or patients who really AR 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 acalasia so they have very profound dysphasia maybe they even have some Associated like weight loss from it maybe they've had some complications from it such as you know aspiration or they have not responded to medical management such as calcium channel blockers nitrates and even Botox which 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 esophagal 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 Sagel 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 acalasia 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 heler myotomy so a heler 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 that actual that that kind of like sphincter area and allow for food and fluid to easily move down and restore flow again usually this 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's 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 heler myotomy they've tried medical therapy and they're just not getting a lot of benefit a botulin of 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 botulin of toxin into the actual lower Sagal sphincter and the way that this works is actually relatively cool it inhibits some of those neurons some of those neurons particularly in the area of the lower solal 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 acalasia we can do medical therapy by relaxing the smooth muscle three 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 monitor to severe but they're a little bit older and they're not a candidate for hel myotomy hel 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 actual medical therapy but they don't want to undergo pneumatic dilation or hel myotomy so we'll do that and buy them a year or two all right diffuses s Jo spasm really the goal here is to try to relax that 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 often times respond very very well to these calcium channel blockers and nitrates it's the same mechanism it's in the mid disal 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 esophagal spasm there is the ability to do the same thing if they want to get pneumatic dilation we often times don't really do this a lot in patients with diffuse esophagal spasm and a botolinum injection can also be considered sometimes in diffus esophagal 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 ESOP it's 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 will do other than just preventing them from developing further esophagitis and so often times 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 Sage Jo sphincter and you're not really going to be doing anything to help the mid disl esophagus one big big tip that I would give you guys is that acalasia will benefit more so from aggressive surgical therapy than medical therapy so it is better for these patients to undergo pneumatic dilation heler myotomy than it would be to put them on things like calcium channel blockers and nitrates they have a more long lasting effect and benefit from those diffus of sageo spasm it's actually better to be more like non-invasive they'll respond much more effectively to Medical therapy than they will to any any type of surgical therapy so it's really important to remember that all right my friends this is esophagal motility disorders I hope it made sense I hope that you guys enjoyed it and as always until next [Music] time