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SVU On Demand Webinar 071123: Fibromuscular Dyspla ...
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Good evening. Thank you all for joining today's webinar. My name is Dr. Anil Kumar. I'm the chairperson for the SVU eLearning Educational Committee. Now before we begin, here are a couple of notes from the SVU office to share, especially for the first time attendees. Everyone should see that there's a question section along the side menu of your screen. It is very close to the chat button. If you see that, please type in any questions that come to your mind during the presentation. Once the presenter has concluded the presentation, we should have some time for discussion. This webinar will be recorded and available on demand for free to the SVU members in seven to 10 business days. Within seven to 10 business days, you will receive an email containing an evaluation that must be completed in order to obtain CME for tonight's webinar. Tonight we are honored to have Dr. Heather Gornick and Melinda Bender from Cleveland Clinic. Dr. Gornick is the co-director of the Vascular Center in the University Hospital Heart and Vascular Institute. She's the director of FMD arterial dissection program at University Hospital and a member of the medical advisory board of the FMD Society of America. She's passionate about vascular ultrasound and has been an SVU member since her vascular medicine fellowship in 2004. Dr. Melinda Bender has been a vascular technologist for over 20 years. She received her bachelor's degree from Oregon Institute of Technology, where she is now pursuing her master's. She's currently working at University Hospital in Cleveland, Ohio, and is a lead technologist in quality and education. She serves as a technical director for the board of directors for the Society of Vascular Ultrasounds. Now we all know fibromuscular dysplasias are very uncommon, but it is far more common than it was originally thought. As Dr. Gornick says, it can cause multiple issues like hypertension, carotid bruise, migraine headaches, pulsatile tinnitus, different type of arterial dissections. It can lead to MIs and stroke. There's a lot of unknowns about these fibromuscular dysplasias. I will invite Dr. Gornick and Melinda Bender now to please take the stage. Thank you, Dr. Kumar, and thank you to SVU, and as always, thank you to Missy McLean for all she does organizing these various events. These are our disclosures. We have nothing to disclose. And greetings from Cleveland, Ohio, and we are actually at University Hospital's Ahuja Medical Center. We're coming to you live from Beachwood, Ohio, which is just right outside of Cleveland. My partner Melinda and I just finished FMD clinic today, and no better time than today to talk about FMD. So for the next 45 minutes or so, we're going to give you an overview on what is FMD, why it's important to recognize FMD in the vascular lab, what are the sonographic findings of FMD. Melinda's put together some terrific tips and tricks for optimal scanning, and then we've got some interesting and unusual cases to wrap up, and of course, your questions. So first of all, to put FMD in context, it's one of our zebras in the vascular lab, and 90% of what we see in terms of arterial disease in the vascular lab is going to be atherosclerosis and plaque, but then there's an important list of other entities which cause arterial disease, things like vasculitis, dissection, aneurysm, compression syndromes, trauma, congenital anomalies, arterial thrombosis, and FMD is one of these non-atherosclerotic entities that can cause arterial disease. And it is, as mentioned, thought to be rare. It may be more common than we originally thought. Disease of medium-sized arteries. It does not involve the veins, and it can affect arteries throughout the body. The pathogenesis and genetics of FMD remain uncertain, and that's an area of active research, but we think there are hormonal and mechanical factors that likely contribute as well as genetic factors, and greater than 90% of patients with FMD are women, so clearly some hormonal component. And FMD causes a broad spectrum of arterial lesions, including, as shown here, the classic string of beads of multifocal FMD, but also can have focal lesions, occlusions, portuosity. And then recently, through research registries, we've learned that FMD is associated with a really significant prevalence of aneurysm, 20 plus percent of patients with FMD have an aneurysm in the body, and dissection, more than a quarter of patients with FMD have had an arterial dissection. And as mentioned, FMD has variable clinical manifestations, and it's really driven by the severity of the lesions, and also the vascular territory involved. So for renal FMD, of course, hypertension is most common, but also things like abdominal bruit, renal infarction, headaches. Renal FMD is actually associated with headaches. Carotid and vertebral FMD can present with a cervical bruit, migraine-type headaches, TIA or stroke, cervical artery dissection, a very common symptom of pulsatile tinnitus or a swishing noise in the ears at times with the heartbeat. And then FMD at other sites can have variable manifestations, can have ephemeral bruit, arm or leg claudication if it's in the limbs, and artery dissection in the limbs. FMD in the heart presents with spontaneous coronary artery dissection, causing myocardial infarction, and there can be mesenteric artery disease with weight dissection or sometimes weight loss due to occlusive disease. If you're interested in reading more about FMD and for what I think are really the clinical standards for FMD in the current time, I would draw your attention to this document. It's called the International Consensus on FMD. It was a consensus from the Society for Vascular Medicine and the European Society of Hypertension, and it's a free download with the journal Vascular Medicine. It was also co-published in the European Journal of Hypertension. And this is really a summary of the state-of-the-art on diagnosis and treatment of FMD. Just want to mention something about nomenclature. Some of the older attendees may remember years ago we used to refer to FMD by terms such as medial fibroplasia or intimal fibroplasia, more referring to what the lesions looked like under the microscope. But we realized we almost never get pathology for patients with FMD anymore. In my very busy practice, maybe once a year, we have pathology. The diagnosis now is really strictly based on imaging. And in our international consensus, we have two types of FMD. The string of beads type is the multifocal type shown here in the internal carotid and renal artery. And then this is the focal type of FMD shown here in the renal and internal carotid arteries. So please use this nomenclature. The focal type is the more common type in the pediatric population. The multifocal type is by far the most common type of FMD. So again, please use that nomenclature. Briefly want to mention an initiative I've been involved in, which is previously the United States Registry for FMD. I just changed its name to the North American Registry for FMD when we welcomed the Ottawa Hospital in Canada. This is sponsored by the FMD Society of America and coordinated by the University of Michigan Cardiovascular Outcomes Research and Reporting Program. And we've learned a lot about FMD through this registry. And also the Europeans have a parallel registry initiative. But for the North American Registry, we started enrolling in 2009 at seven centers. We now have 18 active centers in the U.S., including one Canadian center. Most centers enroll adult patients. A few also enroll children. And as of a few days ago, there were 3,978 patients in the North American Registry for FMD. Briefly want to mention treatment for FMD. Medical therapy is vital for all patients. We recommend antiplatelet therapy in the international FMD consensus, unless there's a contraindication. That's to prevent thromboembolic events. Blood pressure control with ACE inhibitors and angiotensin receptor blockers as first-line agents, especially for those with renal FMD, and smoking cessation. Patients with FMD who smoke have worse outcomes. We spend a lot of time helping patients with their headaches and also the pulsatile tinnitus. We avoid the triptan medications for migraine because those are vasoconstrictors. And fortunately, we have a lot of good alternatives. And we do put patients in an imaging follow-up program. We recommend one-time brain-to-pelvis CTA or MRA in some centers to look for occult aneurysms and dissections. And then based on what vascular beds are involved, we put together a surveillance plan. For most patients, it's duplex for carotid and renal FMD, but there's certain areas, such as the brain arteries, if there's an aneurysm that will need MRA or CTA. In terms of intervention, really what drives intervention is symptoms, not just having beads. You don't need to treat beads if they're not causing problems. For renal FMD, if there's poorly controlled or recent onset high blood pressure. For cerebrovascular FMD, it's if there's cerebral hypoperfusion or such severe lesions that there's not good blood flow to the brain, certain types of dissections, recurrent TIA or stroke despite antiplatelet therapy. And then, of course, certain aneurysms need treatment, even if they're not symptomatic. And we don't have FMD-specific size thresholds for FMD. Thresholds to repair aneurysms really vary depending on the location. Fortunately, there's been such great advancement in endovascular technologies to treat aneurysm. The most common location where patients with FMD need an intervention is in the renal arteries. And we rarely intervene on carotid and vertebral FMD. In terms of the diagnosis, this disease is diagnosed by imaging. There's no blood test, and we don't get biopsies of tissue for FMD. In 2023, catheter angiography, often with hemodynamic measurements across stenoses, is the diagnostic gold standard for both renal and cerebrovascular FMD. But we have really good noninvasive options. And I think for most cases, duplex ultrasound is really at the first line of diagnosis, followed by CTA and MRA. We have little data on the best modality to diagnose FMD in various locations. So the choice of the modality depends on the location to be imaged, the availability, and institutional expertise. And just to wrap up the introduction, we put together four reasons why it's important to recognize FMD in the vascular lab. First is this condition is woefully underrecognized by the medical community and often misdiagnosed or diagnosed years after patients have been suffering with symptoms. So we need more awareness. And that starts, I think, on this webinar with the vascular technologists. Vascular technologists and interpreting physicians are really at the front lines in diagnosing the disease. Indication, brewy. Your technical findings can make all the difference in terms of heading this patient on the right path. If you put greater than 70% stenosis versus findings consistent with carotid FMD, and it's a case of FMD and not plaque, you have a huge impact on the outcome. Very different management. Same whether you diagnose greater than 60% renal artery stenosis or findings consistent with renal FMD. And it's very important to distinguish FMD from atherosclerosis because the management is very, very different. We don't do prophylactic and arterectomies for carotid FMD, even if velocities are 400 centimeters per second. And then finally, you're probably seeing FMD more often than you realize. So with that, I will turn it over to my colleague here for some pearls on scanning. So when we talk about scanning FMD, we're not going to reinvent the wheel here. You're just going to take what you learned in school, and you're going to optimize your imaging. And that's what they teach you to do in school. So first, think back to your first day of clinicals. You didn't go in and get your first patient and say, oh, wow, you're my very first patient, or I've never done this before, or my very first renal cyst. Instead, you used phrases like, I'm a student. Oh, we spent months learning normal before we went out into the field. And you reassure them of their confidence. Please, when these patients come in with suspected FMD, please don't tell them that you don't know anything about FMD or that you're going to go Google it. Because you do that, and then they come in here, and they have a high level of anxiety that we aren't knowing what we're doing. And you're going to be able to see it, and you're going to be able to scan it. So think about your first aha moment, whether you went through a general program or a vascular program or an echo program. First time you saw a gallstone, you're like, oh, yeah, I wouldn't have missed that, or an acute occlusive DVT, or a myxoma. You're like, oh, yeah, that wouldn't have missed that. FMD isn't that easy to see. But it is more like a regional wall motion abnormality, or a chronic dissection, or a renal mass versus a lobulation. With time, you will be able to recognize it much easier. So don't worry about it. You will get it. The important part is to thoroughly document what you see and discuss with your reading physician so that you can see what you might have done better in the future or what you did great, and just discuss it. And when I first started with Dr. Gornick, I reviewed every case with her every time, and she helped me do things better. So again, you learned about your gain in school. Don't overgain. The reason that that's a problem is if you overgain and your color flow bleeds over the vessel walls, you're not going to see those beads very well. And second, optimize your color scale. If you make that color scale too high, you're going to eliminate all the turbulence that you would see with FMD. And if you make it too low, like the picture down here in the corner, everything's going to look turbulent, and that's not accurate either. So we like to keep our scale around 27, sometimes below 30. So our machine is set, the next one below 30 is 27. So we keep it between a scale of 24 and 27, depending on how good the output is. So now we're going to go to renal FMD. All right, so we're going to give you just an overview of what you might expect to see in each vascular territory when scanning for FMD. So for renal FMD, you're looking for elevated velocities, peak systolic velocity in the mid to distal renal artery in contrast to atherosclerotic lesions, which are typically at the origin. I will say with a caveat, sometimes focal FMD can have a proximal lesion, but generally string of beads type will be mid to distal. In general, you're looking for velocities above 200, you're looking for a doubling of velocities across the renal artery. You could use that classic RAR of 3.5 with the caveat that has not been validated specifically for FMD. The velocity shift will be out of proportion to any plaque visualized. And the hallmark of FMD in the renals in all areas is turbulent color flow and also turbulence on the spectral display. You're going to look for kidney size discrepancies, beading may or may not be appreciable on color power angiography or B flow in the renals. And very importantly, diagnostically, please, if this is FMD or suspected FMD, report findings consistent with or are suspicious for FMD rather than just a straight greater than 60% stenosis. So remember when we were talking about scanning, this is a perfect example. This is one of the girls who works in our lab who this was the very first exam she did with FMD and she did exactly what she learned in school. So when you think something's abnormal, what do you do? You document the location, you describe what it looks like, you assess the blood flow through color and spectral Doppler and you try to give them as much information as you can. So this is the very first time that she had ever scanned FMD and look how beautifully she imaged those beads in the right renal artery. Again, don't over gain. Notice you can see the vessel wall and because I've lowered the gain down a little bit, you can see the beads. And if that were to be an over gained picture, you wouldn't see the beads in that artery as well. It would kind of look more uniform. And then turbulence. If you see this proximal part of the vessel, it's very laminar. And then you get here and it's more turbulent and of course, you kind of think it might have been because it's tortuous, but all FMD is tortuous. Another thing you want to recognize is that you want your sample gate in the center of the vessel. Let's remember our physics and hemodynamics. Anytime you put a sample gate towards the wall, the friction of the flow against the wall will make it appear more turbulent. So you want that sample gate in the center of the vessel where the flow would have been the most laminar. And then you can see here how turbulent that is. And that is because of the FMD beads that are there. Also in addition to the turbulent color flow, you often see a tissue brewery and that. So even if you have the color optimized with the gain, you get this bleeding of the tissue. And then here you can see that tissue brewery as well. Again here we have a tissue brewery. You can obviously see that on this vessel and then on the spectrum, you see this like little blip underneath the actual waveform and that is also a tissue brewery. That will be noted in an FMD lesion as well as in a stenotic lesion. And then rule of thumb with FMD, in order to actually visualize the area of concern, you want to elongate out that vessel. You want to visualize as much of the vessel continuously before you start taking the Dopplers. And then you just move your sample gate slowly through the vessel, making sure to hit all of the areas where you have the color aliasing. A lot of you know this. You probably learned this in school. This is obviously a transabdominal image. But if I were in right lateral decubitus, you would best image this left renal artery with the kidney in long. And then on the right side, you'll usually be able to spread the artery out with the kidney and transverse. So here is an FMD lesion. This is the color image. Notice that we have it spread. We've shown the whole artery. So we know that this section here is where we're probably going to get the highest velocity shift. Now, it does take a little bit of time to be able to get, you want to want to just not start taking your samples of the Doppler. You're going to want to try to get this picture and then move your sample gate. And I've always found that when I'm looking at an artery, in order to elongate it out, I'm usually placing my probe higher in the abdomen than I originally would have thought it would be. And then, of course, you want to show the velocity shift and the postgenetic turbulence. And can I just chime in? I think turbulence is the hallmark of string of beads FMD because you have these little eddies and jets of flow swirling around in those beads and dilated areas. So we really, as the reader, want to see the turbulence. And then here, as you learned, when you're in school, parenchymal flow, when you have the resistive index, a normal resistive index in the literature is 0.5 to 0.7. A lot of labs use 0.8 as a normal before they indicate disease. But if you're on the lower end of this resistive index, you might want to start thinking that there might be some kind of subtle abnormality in the artery. And we often see resistive index in FMD around 50. And that would be because we're just, that's causing that blockage of flow. So pearls of wisdom, gather any information you can from other studies before you start. You never know when you're going to miss a horseshoe kidney or especially two renal arteries. So if you read the CTA report and it says there's two renal arteries, you know to look for two because just because they have FMD doesn't mean both of those arteries are going to have beads or a velocity shift. So you're going to want to be able to find which artery it is. Otherwise, you're going to be negatively reporting that the patient does not have FMD. And I'll say this story, this was a patient who had a renal scan and the technologist was really struggling with anatomy and about, I think about 35, 40 minutes in really figured out the anatomy, but the CT was available all along. So use all the information available. All right, now moving on to carotid FMD. The most common location being the internal carotid arteries. And here, what we're looking for is no plaque, but sometimes you can have plaque at the bifurcation and then FMD more distally. So be aware of that. And you're looking for velocity shifts and turbulence of flow. And again, these for string of beads FMD are in the mid to distal ICA, very far beyond where we see it for atherosclerotic disease. The most important thing I can tell you is that those ICA carotid diagnostic criteria, which I've spent a lot of time thinking about for atherosclerotic disease, do not apply to FMD. So please do not put 50 to 69% stenosis, greater than 70% stenosis on the impression for patients with FMD. It's not been validated in that setting. And also in FMD, you often have these tandem lesions. So it's not uncommon to see patients with velocities of 300s, even 400s, who've been stable and doing fine all along. So please report this elevated velocity, turbulent flow, maybe beating if you see it, consistent with FMD rather than just your diagnostic criteria for atherosclerotic disease. Okay, so the one thing you want to learn about ICA FMD is that you always want to use the curvilinear probe. And I believe there are some programs out there now that are now training their sonographers to always break out the curvilinear probe for the distal vessel. And pay attention to patient positioning. For ergonomics and for your normal routine scan, of course, they tell you to turn the patient's head to the side so that you can hold your hand on the vessel and not strain yourself. And I've discussed it with several of our sonographers and had them experiment. When I get to the distal ICA, and this is my explanation, and maybe I'm wrong, but when you think about the carotid artery, it has to enter the skull, and it enters the skull through the carotid foramen, which is a stationary position in the skull. So if you're turning your head to the side, you're twisting the artery. And as you know, tortuosity makes the vessel harder to image. So when I get to the very distal, I always have them turn their head and look straight up. And I can usually visualize another two, three, four millimeters, maybe more. Sometimes it's a significant amount, depending on the patient. Sometimes it's not very noticeable at all, but I always attempt to do it. So in case their FMD starts very high up, that we're able to see it. So here is a pitfall that has happened. This 67-year-old woman came in with carotid beroeys, and she had this scan done. I mean, hey, if I looked at this, I would say, yes, absolutely. Normal scan, you know, clean window. Who knows why she has that beroey? But then they broke out the curvilinear probe, and then look what they found here. She had velocities of 531 over 199, and very turbulent flow and tortuous. So she actually does have FMD. Yeah, and again, I can't, turbulence is what I want to see, so I appreciate the spectral beroey too, really showing that turbulence. So again, the pearl in this one is you cannot rule out ICA FMD until you scan very high in the neck with a curved, low-frequency transducer. And then you can appreciate the beating. Now, my machine actually has a Bflow, and I love it. You can also get the same type of an image with a ColorPower Angio. Some of the machines have something called Radiant Flow or Brilliant Flow. Basically, the rule of thumb here is use any tool that you have to see what image is better. Everyone's going to, sometimes it's ColorPower Angio, sometimes it's that pink instead. And sometimes you can see it in grayscale, just change your gray color map. And this is beading of the ICA in grayscale and in Bflow. And here are some more images of beads in the ColorPower Angio in the Bflow, showing you the various different types. Sometimes you have tiny little beads that come together, or sometimes you have, they're a little bit bigger. Sometimes they're very pronounced, sometimes they're more subtle. And you can always just try every, use every tool in the toolbox to get it the best way you can. And then again, like with the renal FMD, optimize your color scale. Notice the scale over here, like I said, is at 27. And so if you look, it's very laminar until you get to that beading, and then it becomes very turbulent, and then we have a velocity of 282. And again, here's the turbulence you can see on the spectrum, and even in the color. And notice that the sample gate is in the center of the vessel. So that's not a false turbulence based on friction. All right, for mesenteric FMD, you can sometimes see beading, especially in the superior mesenteric artery with velocity shifts and turbulence. The other things we commonly see in the mesenteric arteries are dissection of the celiac or SMA, most commonly, or visceral aneurysms. So we're looking for all of that in the vascular lab. So this is a very common finding that we find is a dissection. This one is in the SMA. You can clearly see the intimal flap, and you can see the two color channels with the very turbulent flow through the channels. And here is another SMA dissection in B-flow and in grayscale. I do say sometimes I over-game when I see a dissection because it just makes... So it does cause internal echoes, but it does make you see the intimal flap better. One thing I caution you against is trying to make that lumen when you're looking for a dissection nice and clean and altering your TGCs because you might filter out your intimal flap. So I know that it's a pet peeve of a lot of people to make that very, very clean. And for the normal patient, yes, but if you're really thinking they might have a dissection, you don't want to clean that out all the way. And here is another section, and this is a prominent bead in ectasia, and we can see the very turbulent... Now, this one's a little more proximal than what we would normally see it, but the very turbulent flow with the ectatic segment in the mid. And this patient also had renal FMD with the ectasia, and here you see the arrow showing you the ectasia. And she had such severe disease, she actually is forming collaterals in the abdomen. So that would be all of this lovely spaghetti there. Another common area for aneurysm with patients with FMD is the splenic artery. And one thing to remember, a lot of times you will see aneurysms in the arteries more proximally with FMD, but if it reads that the patient has a splenic artery aneurysm, go back to what you learned in school. Where do we normally find aneurysms in the vessel? At the distal segment. So if you're looking for a splenic artery aneurysm and you aren't seeing it, move the patient up into a right lateral decubitus and image at the splenic hilum. I'm going to say probably 75 to 80% of the ones I find are way over there on the left-hand side. And I just... It's much easier to visualize the spleen from right lateral decubitus. All right. So now FMD in the extremities is very interesting. The common locations in the extremities are the brachial arteries and the external iliac arteries. And this is actually an area where we think mechanical factors may be at play because we're obviously bending at the elbow and bending at the hip joint. So there may be mechanical factors that lead to the development of FMD in these locations. But this is generally where we find FMD in limbs, rarely can present with claudication, sometimes a dissection. More commonly, you'll just hear a bruit over the groin area or the antecubital fossa or sometimes a blood pressure discrepancy in the arms. So this is external iliac FMD, and we do have an increased velocity, and you can clearly see the beads on the posterior wall and in the B-flow color field defect. But this looks far more subtle than your normal FMD as we'd shown you in the renals or the carotids. So really on this one, you have to focus on that turbulence because, I mean, you might overlook those beads and think it's really just a normal vessel. Again, here, here's the same vessel. This is a side-by-side image. That vessel looks fairly normal until you turn on the color and then look at the turbulence and the shift and the post-sonotic turbulence in the vessel. And I'll say the turbulence you're seeing in color and spectral display is what we hear when we hear the femoral brewery. So, and then here is brachial FMD in color power and B-flow and then in angiography. And you can clearly see the beads in all of these as well. And then sometimes you have to be, now, a lot of times when I'm doing the brachial artery, I like to use, again, remember what you learned in school. You wanna use the highest frequency possible. So most of the time when I do brachials, I actually break out that high-frequency interoperative probe and I believe it's like 18 to 22 megahertz so that you can get, like right here, this is a color flow defect and I've under gained it. And if I would have turned that gain up, you would be able to see that webbing right there in the vessel. And then here is some on the posterior wall with B-flow. So now we're on to interesting cases. So we're gonna put it all together and share some of our interesting cases from our mutual archives. So this case is a 41-year-old female who presented with pulsatile tinnitus with bilateral ICA multifocal FMD, right vertebral multifocal FMD, a small right renal artery aneurysm and additional findings in the proximal ICA. So this here is actually what we call a carotid bulb diaphragm. And what that is is a non-atherosclerotic lesion that you find at the origin of the ICA. Typically that is not found with beating. So this was a very interesting case because it's both and that's not usually what we report. And here we have the same one with the turbulence past that bulb. And you can see a little bit of the bulb diaphragm right here, but you can tell that it's the turbulence and the delayed upstroke. And then the other key finding, I'll show you some examples with these bulb diaphragms or webs, which we have seen in some patients with string of beads FMD, or sometimes just alone, is you get stagnation of flow posterior to the web. So you can see here, there's not a lot of color filling and that's associated with thrombus formation due to stasis and embolic TIA and stroke. So this is an interesting entity. It's interesting, it was first described among people of Afro-Caribbean origins, such as Haitians, but it's since been observed in multiple demographic populations. This may be some variant of FMD, and we have seen it in patients who also have string of beads FMD, but what it is is a focal web, as Melinda mentioned, at the origin of the ICA. And again, clots form behind these webs because of the stagnant flow, and you can have embolic TIA or stroke. And there's a little bit of controversy in terms of how best to manage these, but in general, it's felt that when these webs are symptomatic, that patients should be treated. So when you think of that, you can always think of what you learned about venous disease in school. Where is a common place for a clot and a vein to form? Behind the valve. So think of a web like a venous valve. Very similar, for sure. And this is another patient of mine with a web. This was interesting. This lady presented to an emergency room with left-sided neck pain, so over here, but actually had this completely incidental finding of a carotid bulb diaphragm web. This has been asymptomatic, and this patient we've managed with antiplatelet therapy, and she's done fine, fortunately. All right, I'm gonna tackle this next case. This was a 40-year-old woman who was a nonsmoker who was diagnosed with renal FMD and hypertension. It was during workup of hypertension. She was also found to have an intracranial aneurysm, and she actually had a family history of abdominal aortic aneurysm, but she was found in the evaluation of her renal FMD for hypertension to have a rather sizable 2.8, depending on modality, up to three centimeter aneurysm of the right renal artery, as you can see here, really nicely seen on grayscale and also on color flow. You can see that Pepsi-Cola yin-yang sign in the aneurysm on duplex. I thought that was beautifully imaged. She went on to catheter angiography. You can see she had multifocal FMD followed by that large aneurysm. Here is her turbulence of flow on color and spectral display, elevated velocities, and post-synodic turbulence, very classic. This was actually inside the aneurysm sac. And this patient actually underwent a very complex repair by one of our vascular surgeons and a transplant surgeon, Dr. Kashub and Dr. Sanchez here at University Hospitals, and they actually resected the aneurysm and then did an auto-transplant where they took a segment of her own hypogastric or internal iliac artery to use as conduit. We don't like to use venous conduit in these patients because they tend to form aneurysms. They dilate over time. And they actually basically grafted the beaded segment, so cured her beads, and then they did an auto-transplant and they moved the kidney and actually anastomosed the renal vasculature to the common iliac artery and vein. So she had what we call an auto-transplant as well as resection of the aneurysm and repair of the artery and this actually cured her hypertension. This was her post-procedural scan where you can see now very laminar flow in that hypogastric conduit renal artery that's been anastomosed to her iliac system, had a good flow pattern in the iliac and all velocities were normal and she's done really well. All right, so here's a case that clearly describes to you why it's good to review all the previous imaging because let's be realistic. How many of us would see a three-millimeter aneurysm on the proximal celiac artery unless they were to look at the CTA and see this image here? So this was a 57-year-old woman with a history of hypertension and a heart attack due to spontaneous coronary artery dissection. So she had her vascular imaging after the scadural at FMD. So we did the head-to-pelvis CTA and what was found on her scan was that three-millimeter saccular aneurysm on the celiac artery. And she's actually, we're just following this, this tiny little blister-like aneurysm and it's been stable, but really unusual morphology. Tiny, tiny little blister. So the fourth case is a 54-year-old woman with a history of visceral artery aneurysms and a recent diagnosis of carotid and renal multifocal FMD. And she presented to the vascular lab with left arm claudication and subclavian BRUI. And as you can see here, we did the PBR tracings. And so you didn't think you were gonna get PBR education today as well, but you can see the marked difference between the left arm and the right arm, whereas the right arm has a very sharp upstroke, the left arm is very delayed and it has a dampened waveform that continues through all the way to the fingers. So we went ahead and did an arterial duplex on her. And this was what we found in her proximal left subclavian artery. She had a very high velocity with a significant tissue BRUI artifact and a monophasic flow distally. So it indicated that she definitely had something going on. So even if you were to take your subclavian, even if you didn't break out that curvilinear probe and go all the way down towards the arch that you could, if you took this far picture on the right here, you would say, that's not a normal subclavian. And you would investigate further and find something like that. So after we did the subclavian, we went out to look at her vertebral and noticed it was bidirectional, which would indicate again, if we were just doing a carotid, that there would be a problem. So we went ahead and exercised her. And after we exercised her, we Doppler the vertebral artery again. And instead of being bidirectional, that flow in the left vertebra went completely retrograde. Melinda, how did you, just for everybody's education, how would you exercise for the arms? So we don't have a set criteria, but I like to use something that can be reproducible. So we have them do shoulder presses holding a full gel bottle for five minutes, if they can do it. Yeah, and this patient, so this was interesting because she has string of beads FMD in her carotid and renal arteries. And then she has a lesion in her subclavian artery, which is very, really looks focal. So it's right there. So it's a focal subclavian lesion. This patient really does not have atherosclerotic disease. So I don't think this is atherosclerosis. I do think this may be a case of a patient who has mixed string of beads and focal lesions in her vessel. So even for someone like me who sees FMD all the time, the disease state continues to surprise me. So case number five is a 74 year old woman with a long history of renal FMD. Progressive asymmetry or loss of right kidney mass. So as you can see here, we have a normal velocity. And then when we get to the mid segment, she does have increased flow and it's 267 meters per second. And then when you take, when you compare the two, the right kidney is 7.9 centimeters, which is obviously out of normal range of nine to 13. And the left side is 10.3. So the size of the right kidney alone would be indicative. But even if you had a kidney that was in the normal range, if it was over nine, but the other side was two centimeters larger, you would want to think that there might be an issue. So the left kidney is normally bigger than the right, simply because the kidneys grow to fill the space and the liver is much larger than the spleen. But to see a greater than two or two and a half centimeter difference should indicate that you should probably take a few extra pictures. Definitely try to find out if you don't find a lesion of stenosis, a atherosclerotic lesion, then you want to go ahead and try to lay out that artery and see if maybe they have FMD. And this patient, this was, as mentioned, someone I had followed for many years and had no renal FMD, but basically her kidney was getting progressively smaller. It was literally shrinking before my eyes over the past couple of years of scans. So really decided to pursue a more aggressive course with her and sent her to my partner here at University Hospitals, Dr. Mehdi Shishabour, who did a protocol we have from our international consensus where we do catheter angiography, actually measure pressure gradients across the area of the string of beads to confirm there's a hemodynamically significant lesion, which there was in this case. And then he also did intravascular ultrasound, and you could see this medial layer thickening on the ivus and a small lumen. And this patient actually went on to balloon angioplasty, mainly because she was losing kidney volume due to presumed progressive renal artery stenosis, and she's done well. So our take-home points is that you will make a diagnosis of FMD at least once in your career, and be aware of the hallmarks of the findings, the velocity shifts in the mid-distal vessel, the turbulence of flow, the beating or webbing. Look for aneurysms and dissection because they are very common. Arterial tortuosity is common with FMD and can make it really tricky to scan it, but I'm sure you've done it in the past and you will do it again. And optimize your equipment and the machine settings to make the correct diagnosis. And don't take traditional criteria of atherosclerotic for reporting FMD lesions. Instead, write findings consistent with FMD. We all learned how to scan in school and we're all good at it. All you need to do is take the time to optimize that image as best as possible. Remember what you learned in school. Highest frequency possible. Keep the color gain appropriate. Keep the scales appropriate. And you will do this just fine. And most important, look at the other imaging. Look at the other imaging, otherwise you might miss it simply because you're missing vessels. So just take a deep breath. You can do this. I did this. You can do it too. So just wanted to end with giving you a few resources to learn more about FMD. Would direct you to this amazing organization, the FMD Society of America. They sponsor the North American Registry. They have a lot of resources on their webpage and also have an annual conference. And then actually some attendees have asked, we here at UH, we actually run a quarterly patient information and support group for patients with FMD and arterial dissection. We have a different topic every quarter and it's open to registrants even if they're not our patient here at UH. This is more patient focused, but we do cover FMD educational topics. Our next topic is a classic, women's health and menopause, which will be next week. And you can register. Here's the registration information. And as we wrap up, Melinda and I just wanna acknowledge and thank our entire team here in the FMD and arterial dissection program at University Hospitals, especially our nurse coordinator, Becky Young, our administrative coordinators, Stephanie Pisoski, and our research coordinator, Dina Dijonovic, and also wanna just acknowledge all.
Video Summary
In this video webinar, Dr. Anil Kumar, the chairperson for the SVU eLearning Educational Committee, introduces Dr. Heather Gornick and Melinda Bender from the Cleveland Clinic. They discuss fibromuscular dysplasia (FMD), a non-atherosclerotic disease that affects arteries throughout the body. FMD can cause multiple issues such as hypertension, carotid bruise, migraine headaches, pulsatile tinnitus, and arterial dissections. Dr. Gornick and Melinda Bender provide tips on scanning for FMD and share interesting cases from their experience. They emphasize the importance of recognizing FMD in the vascular lab and distinguishing it from atherosclerosis as the management differs. They also urge vascular technologists to familiarize themselves with various diagnostic modalities such as duplex ultrasound, CT angiography, and magnetic resonance angiography to accurately diagnose FMD. They mention the International Consensus on FMD as a valuable resource for clinicians. The webinar concludes with Dr. Gornick and Melinda Bender thanking their team and mentioning resources for further learning on FMD, including the FMD Society of America and a quarterly patient support group organized by their team at the Cleveland Clinic. Overall, the webinar provides an overview of FMD, highlights diagnostic techniques, and shares insights from clinical cases. No credits are mentioned in the video.
Keywords
fibromuscular dysplasia
hypertension
carotid bruise
migraine headaches
pulsatile tinnitus
arterial dissections
vascular technologists
Cleveland Clinic
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