 Okay, so we're going to finish up on retinal vascular diseases, not including diabetes. So most of you guys were here last time, so we covered venous and arterial occlusions last time. And this time, we have a lot of kind of smaller topics to cover and we'll try and get through them as much as we can. We'll start with ocular schema syndrome, hi good morning. So severe carotid occlusions can lead to reduction in the blood flow, and this reduction in the blood flow from the carotid system leads to both anterior and posterior segment ischemia. Most common complaint is vision loss, this is gradually progressive over weeks to even months. A fair number of patients will come in complaining of pain. And this is typically an ischemic type pain, so the pain gets better when they lay down just because they're lowering their perfusion pressure and they're able to perfuse the eye when they're laying down. The pain can also be inflammatory for anterior segment inflammation, or the pain can be from elevated intracular pressure because they can come in with neobascular glaucoma as well. We often see this in older men and it can be bilateral. It's pretty rare, but it is thought that this could be underestimated because it can also mimic mild to moderate NPDR or a mild non ischemic CRVO, so perhaps we're not calling it as often as it really is. So just to refresh the anatomy, so the up-down the garbaderie gives rise to the central retinal artery, and then the posterior ciliary arteries, and those both arise from the internal carotid. If you have an occlusion at the internal carotid, you're going to get a reduction of the blood flow that provides blood to both the anterior and the posterior segments, as well as the retinal and caroidal circulation. So that's how we end up with more widespread ischemia, and they're perfusing from usually collateral circulation that's arising from the circle of willis. So the findings on clinical exam, they often come in with unexplained neobascular glaucoma or rubiosis. They can have spontaneous hyphemos, enter chamber inflammation. They can have more of a cataract on this side of the eye with the occlusion, and they can also have synechia, iris atrophy. On retinal exam, you can find vitreous hemorrhages, peripheral neobascularization. The most common thing we see clinically would be midperforal blot hemorrhages, but they can also have optic nerve changes with pallor and edema or even macular edema. They often will have pretty severe stenosis of that carotid artery on that same side, and this can be the presenting findings for carotid occlusive disease in a pretty high number of patients. I think this is important to keep in mind over at the VA where we have our vascular-pathic population because they often are associated with ischemic heart disease, prior TIAs, peripheral vascular disease, diabetes, hypertension, and they do have a higher stroke rate, 4% stroke rate per year compared to just half a percent in controlled population, which is age-matched. This is the retinal findings that you'll see, which is these midperforal blot hemorrhages. These are deeper blot hemorrhages as opposed to the more flame hemorrhages that you would see in a vein occlusion. In clinic, when you're looking at these patients and you're not sure, is this diabetic, is it a vein occlusion, is it ocular schemic syndrome, one thing you can do pretty easily is ophthalmodinomimometry, and so what you're doing is basically just putting gentle pressure on the globe, so while I'm looking with the indirect, I could just put pressure on the globe and you're looking for cessation of the, well, you'll start causing arterial pulsations, and you shouldn't be able to do that in a normal eye, but with these eyes with poor perfusion, you can overcome that ophthalmic artery perfusion pressure just with gentle, globe digital pressure, does that make sense, how I would do that, so you would just kind of have your indirect, kind of gently pressure on your finger, tell the patient you're going to press, and then you can overcome the central retinal artery and start to see pulsations, then that could be an indication of reduced blood flow and reduced perfusion pressure to the central retinal artery. The other clinical finding is to do a fluorescein angiogram. The most common thing you'll see would be prolongation of the AV transit, that's the most sensitive, but the most specific sign would be delay in the choroidal filling, so normal choroidal filling should happen within five seconds of the dye being pushed, but in ocular schemic syndrome, it can take quite a bit longer, even up to a minute. You can also see retinal vascular staining, and this is typically staining of the arteries, and then you can also have angiographic leakage into the macula as well. So this just shows the delay in the choroidal filling. You can see even at 24 seconds that the choroid isn't completely filled, and then there's pretty significant delay in the AV transit time, up to 49 seconds. We still don't have these veins filled out here. The other thing you can see is staining of the arterial walls. Oh yeah, you can get capillary dropout, you can also see microannurisms as well. I think this is a really good chart, this came from a review article of ocular schemic syndrome, and this just contrasts the different clinical findings between ocular schemic syndrome, vein occlusion, and diabetic retinopathy, and you're really severe a schemic CRVO, you're not going to mix that up with ocular schemic syndrome, but it's more of those kind of mild non-eschemic ones where you'll be like, I don't know, which could it be. One thing to look at would be the retinal veins, so in an ocular schemic syndrome they're dilated but they're not going to be tortuous, whereas in a vein occlusion they should be tortuous. Diabetic retinopathy you'll see more beading rather than tortuosity. And then yeah, this, Lee just answers your question, capillary dropout in the ocular schemic syndrome, which you can also see in vein occlusion and diabetic retinopathy. But you really won't see exudates, hard exudates in an ocular schemic syndrome like you would see in diabetes, and then central retinal perfusion pressure, that would be normal in vein occlusion and diabetic retinopathy. And then this, the angiographic finding of that delayed, caroidal filling. In CRBO you'd see vessels vein staining as opposed to arterial staining that you would see in ocular schemic syndrome, so I think those are the biggest things that can help you kind of figure out the difference. Obviously if you're not sure, you know, doing the test to rule out ocular schemic syndrome is a pretty non-invasive safe test and pretty low risk, so if you're really not sure, I think getting a carotid Doppler and just ruling it out is not going to, no one's going to fault you for that, if you're worried. So the, today I know getting the carotid Doppler, if that's questionable, then go into a CT angiogram. A carotid angiogram is always considered the gold standard, but it's not really done because of the high morbidity associated with it, they have a pretty high risk of strokes and TIAs associated with that carotid angiogram, so it's not really the standard first line test. The prognosis really isn't that great, if they have rubiosis, 90% will be legally blind within one year, so it's pretty high rate of visual loss, you can get rid of the neobascularization about 35% of the time with PRP, you can also use anti-vegeth agents for these patients to help control the pressure, and they do have a pretty high five-year mortality rate due to a cardiovascular disease, it's the most common problem. So as far as the treatments for the eye, controlling inflammation and with steroids and cycloplegics that can help with the pain and the inflammation and managing the intracular pressure, a lot of these patients end up with either shunting procedures from the glaucoma service or even diode, cyclophenyl coagulation, from the retina standpoint we can control the neobascularization with anti-vegeths and PRP, but the main thing is also getting them to the vascular surgeon for a carotid endoterectomy. Any questions about ocular schema syndrome? No, okay, so we'll move on to hypertensive retinopathy. So really most patients with hypertensive retinopathy when they're coming in with kind of mild to moderate hypertension, they might not have any visual complaints at all and the most common thing you'll see would be AV-nicking. There's really not any association between what the blood pressure is and the retinal findings. Patients that come in with vision loss, those are patients that come in with malignant hypertension, really severe accelerated hypertension that you see with like eclampsic, or eclampsic patients, or renal hypertension. So the modified shape classification, grade zero really have no changes. Grade one arterial narrowing, grade two narrowing with irregularities in the arteries, and then in grade three is when you'll see hemorrhages and exudates, and grade four you'll see optic nerve edema. So this is a grade three, you can see that the disc has a nice sharp clear margins, but you do have exudates, cotton wool spots, arterial irregularities, blade hemorrhages, and then this is a grade four with a severe optic nerve edema. Obviously this can be a lot of different things, so you want to keep hypertension on your differential, checking blood pressure in someone that comes in like this, but there's other things you want to consider, Bartonella, mass lesions, anything like that, but if you see someone like this, you want to check the blood pressure in the clinic, and if it's high, obviously send them to the ear. So hypertension can also affect the coroid and the optic nerve. You can see these elishnic spots, these are these little kind of tan, lobular lesions at the level of the coroid, where there's non-perfusion. Initially they're kind of tan, and then later they'll become hyperpigmented. And then the other thing that's kind of classically seen with hypertensive coroid changes are these segrous streaks, and this is just a pigmentation that can follow the coroidal arteries, and this is associated more with acute malignant hypertension in young patients. You can also get serous RDE's and vocal or PE detachments. So check the blood pressure. If it's severely high, I'll send them to the ER. If it's more, if I'm not as worried, I'll send them to their primary care doctor pretty quickly within a week or so. But if it's 1.9,200, they're going to the ER from my clinic. You don't want to send somebody home, and they have a stroke. It's not good. So moving on, sickle cell retinopathy. This is kind of the classic test question. Not that we see it a lot here, but you will be tested on sickle cell retinopathy. The sickle cell hemoglobin S is the valine substitution, and then hemoglobin C is a lysine substitution. You get deformation of these red blood cells that then sickle, and then they thrombose and lead to vascular occlusions. The most common abnormality would be hemoglobin SS disease. That has more systemic complications and not as high a rate of ocular complications. The rate of proliferative retinopathy is highest in hemoglobin SC disease, and that I've seen tested for sure. So in the Iowa sickle cell disease, you get sickling of these abnormal red blood cells, and that leads to thrombosis, and then you get peripheral occlusion and non-perfusion that then leads to retinal neovascularization at the border of the perfused and non-perfused retina. In non-proliferative sickle cell disease, the classic kind of findings would be the salmon patch hemorrhage, and this is an arterial occlusion with downstream intra-retinal hemorrhage, and then that salmon patch hemorrhage can kind of de-hemoglobinize and then lead to these refractile deposits that you can see in the retina. And then the sunburst lesion, this is from sub-retinal hemorrhage, and then you get RPE migration into that. Sorry, I don't have many pictures of sickle cell retinopathy, so I had to take it from books, and they're not the best pictures, but you can kind of get the idea of it. The other thing that you can see in sickle cell patients is angioid streaks, and this is from occlusions at the coriocapolaris that can then lead to breaks in Brooks membranes, and this occurs in about 6% of hemoglobin SS and AS patients. Any other, what do we normally see angioid streaks in here in Utah? Yeah, pseudosanthemalastica. Yeah, that's where I've seen it mostly here. Dr. Kierke, why is it that there's more retinal management stations with the SC and the SS? You know, I was looking into that the other day, and I don't think that anybody really knows the answer to that. At least that I've found, I don't know, I can look into it more, but I didn't see a reason for that, but that's a good question. So the staging, stage one, you get peripheral occlusions and non-perfusion. Stage two, you get these anastomoses peripherally, and then stage three is when you start to see the C-fan, and then stage four is Victorious hemorrhage, and stage five is Attractional Detachment. So this is kind of that classic C-fan appearance, and these are in the peripheral retina, different than diabetic retinopathy, where you see it more in the posterior pole, but they get their proliferation peripherally, and this is kind of a white C-fan, and what's happened here is a C-fan is basically auto-infected, and then it's left this kind of white C-fan. On angiogram, you can see these C-fans just out in the periphery, just at the border of the non-perfused and the perfused retina, and this patient's had some laser treatment. It looks like they need more. And this is just contrasting sickle cell from diabetic retinopathy. In diabetic retinopathy, we see the neovascular membranes along the arcades, along the optic nerve, but in sickle cell, the neovascularizations occurring in the periphery. Another thing you can see are these coma-shaped blood vessels on the conjunctiva. So African-American patients that come in with a hythema, you want to screen for sickle cell disease. It can be much more difficult to control the pressure, and you want to consider an earlier anti-chamber washout. You don't really want to do carbonic and hydrogen inhibitors because that can dehydrate and worsen sickling. I would do a baseline fluorescing angiogram in a patient with sickle cell disease to look for non-perfusion and proliferative disease, and you want to follow them pretty closely. The thing you want to be careful of is that laser treatment in these patients can often precipitate tears and detachments more so than you would see in diabetic retinopathy. So often you start lasering, and then they can end up detaching, so you want to be pretty careful. People often will just do peripheral scatter laser to the non-perfused retina. Some authors advocate for actually lasering the feeder vessel of C-fans, but that's a little controversial because that does lead to tears just right at the base of that C-fans. So most people do laser to the peripheral non-perfused retina. So when considering retina surgery for these patients, you want to be careful with your surgical planning. You would not do an encircling buckle because that buckle can lead to anterior segment ischemia and problems with rubiosis and neovascular glaucoma. You want to be pretty careful with your cryopexy because of the risk of anterior segment ischemia from too much cryopexy. And you want to be really careful with your use of gases and epinephrine because you don't want to make anything worse by causing more sickling. And you have to monitor their pressure really closely in the post-operative period as well. Don't remove extracular bushings. Like you wouldn't detach any muscles when you're in the surgery because of the risk of ischemia from that. Not that we're commonly doing that, but sometimes you end up having to manipulate muscles, but you would not do that in a sickle cell patient. So like I said, the detachment usually starts in that area of the ischemic retina that can be precipitated by laser. And the surgery can be really complicated. They have a high rate of complications. These are tough patients to take care of. This is from your book, just to keep in mind your kind of differential diagnosis of peripheral neovascularization. I will not read that to you, but you can look at it. Yeah, Ali? So for your sickle cell patient, can you do them? So you could either use oil would be a really good option in these patients. I think most people would do oil instead of the expansile gas formation. I mean, to be honest, I don't have any sickle cell patients here in Utah. I think I had a few in Madison that required surgery. I saw more there than here. But here, it's just not a really common thing that we see. So it's not as common to operate on. But I would probably use oil for a lot of these patients. So arterial macroaneurysms. The thing that's always tested on macroaneurysms is you just want to keep this in mind in your differential for something that can cause blood throughout all layers of the retina. It can cause sub-retinal, inter-retinal, pre-retinal, blood, victories, hemorrhage. So that's always kind of like the main OCAPs question on macroaneurysms. Typically, you see it in the second order arterials and it can traverse the entire retinal thickness. These patients can get macular edema, macular fibrosis from the macroaneurysm. They can have vision loss from large pre-retinal, sub-retinal, and vitreous hemorrhages. They typically kind of spontaneously sclerose and thrombose on their own. So often, they don't need treatment once they kind of bleed, they fry bros, and then that's kind of it. But sometimes they can continue to leak and then you might need to consider doing laser treatment. Sometimes with a really large hemorrhage, you would need to do surgery to go in and clear out the blood. We typically see this in kind of older women, hypertension is a really high comorbidity with these patients. And just this is kind of the typical fluorescent angiogram finding where you can see this dense blood. It's kind of hard to tell in the color picture. Maybe there's something right there, but you're not sure. And you do the fluorescent angiogram and you can see that dilation really light up. And then there's another macronurism here that's not quite as big. There are people that advocate using anti-veg F agents for this patient. That's not quite as common. More commonly, you would see photo-quagulation either around the macronurism or even directly to the macronurism to help fibrose it and involute it if it's quite leaky. The risk of kind of treating the macronurism directly is it can lead to downstream vascular occlusion. And then radiation retinopathy. Radiation retinopathy really looks a lot like diabetic retinopathy and it's really managed a lot like diabetic retinopathy and really keeping in mind patients that have had treatments for ocular melanomas. But then there's also patients who've had a fair amount of radiation for other tumors to the head that can have radiation retinopathy. So if you can't quite explain why they look like a diabetic and they're not diabetic, you wanna ask about radiation to the head. It typically comes on a year and a half or so after external beam but with plaque treatment it can come on quite a bit earlier. Their treatments, a lot of anti-ved Jeff agents are used nowadays. Tramsidolone, focal laser for macular demon and PRP. They can require vitrectomy for neobascular complications and vitreous hemorrhages. And then moving on to valsalva retinopathy. Valsalva retinopathy can happen with any kind of strain. You can see it after food poisoning, you can see it after patients have had pneumonia and they've been coughing a lot and they just get these ruptures of these blood vessels. They can be really mild or more severe and they're kind of ruptures of these small capillaries in the macula. Often the blood is under the ILM. Usually it just resolves on its own but sometimes it does require surgery. This is kind of a more mild valsalva retinopathy and this would be one that you would really just watch and that would get better. They would get quite a bit of visual return. This more severe hemorrhage, that would be one that if it's not clear and you'd want to consider surgery for something like that. And then moving on to perchers retinopathy. So perchers retinopathy is due to, usually it's an acute compression injury to the thorax of the chest. It was initially described in someone who fell out of a tree. It's thought that it's kind of activating the complement cascade system and then we get leukocyte aggregation and then occlusion of arterioles that lead to this ischemia. They can also have optic nerve edema that leads to optic nerve atrophy. They can have irreversible vision loss. Sometimes it will get better but they can't have significant visual loss related to this. This arrow is just pointing out perchor fleckin and so this is this polyethanol shape and it's hard to tell here but the whitening doesn't actually reach the vessels. There's a clear area from the whitening to the vessel and it's the occlusion of the precapillary arterioles there from leukocyte. Yeah. The last one is history. Yeah, so with perchers they haven't had a direct blow to the eye, right? That's the main thing. Like perchers retinopathy, you have to have like that compression injury. Perchers-like retinopathy which I'm just gonna cover. That's other causes that can look like this. Perchers-like retinopathy is the fat embolism, pancreatitis is the most common cause. You can also see it with autoimmune disease but comatio, that's that direct trauma to the eye and if patients have severe trauma, they might not know, right? But perchers, it can have some hemorrhages. Usually comatio, you would really severe comatio or dysphilitaria, you're gonna see more hemorrhages associated with it too. And perhaps with comatio you might see like a caroidal rupture that could clue you in that there was a strong blow to the eye as well. You wouldn't see anything like that in perchers obviously because it's all happening at the level of the vessels with that, with the complement activation is the most, the biggest theory on what's happening with perchers. And then Tersan syndrome. This is intracranial hemorrhage or even severe elevation in the intracranial pressure that can lead to these rupture of the retinal vessels. It can happen really quickly after a intracranial hemorrhage, almost within an hour of a large subdural hemorrhage. Can you see intraocular hemorrhages? Often these patients are gonna get better on their own and they have a pretty good prognosis for visual recovery. If they're not clearing, then you wanna do surgery. This is kind of the classic double ring sign that you would see in Tersan. So this inner ring, the darker ring is sub-ILM blood and then this outer ring is sub-hyloid blood. And then Coates disease, kind of moving on to other retinal vascular diseases. I just feel like there's so much to cover in this lecture that you don't get to talk about each as much as you might want. But Coates disease typically presents in young men, young boys actually and infants. This is in your differential of leukocoria. They can have that white, red reflex. You wanna differentiate this from retinoblastoma. Ultrason characteristics to differentiate from retinoblastoma would be calcium. Yeah, calcium, if they have calcium, it's not Coates disease. You wanna be thinking about retinoblastoma. So this is retinal telangiectasia with these ectatic arterioles, microannurisms and exudative RDs. There's a broad differential obviously for leukocoria that you guys should know backwards and forward. And then there's two different staging systems for Coates disease. They're pretty similar in terms of the different stages. I don't actually know why we need two different staging systems for Coates disease, but we have them. Stage four is total RD and then stage five is when you get complications. Usually that's when they have glaucoma issues and they get an enucleation from a blind painful eye. The treatment, kind of the standard treatment is laser photocoagulation to the ectatic arterioles. They can often do cryotherapy as well. These patients, if they get a total RD or even a pretty large RD, you might end up needing a buccal drainage and then enucleation for those blind painful kind of glaucoma to size, which are the really advanced stages that we try to avoid. There is some role for anti-vigil agents in these patients as well. If they present at a younger age, they usually have a worse prognosis than those present later on. So macular, telangiectasia, three types of macular telangiectasia. Type one is unilateral in males and it's really thought to be a variant of Coat's disease. So it's also called Lieber's milliary aneurysms. You might have heard that as well. So some people actually just group Coat's into type one macular telangiectasia, but the BCS separates the two. The real differentiating factor is Coat's disease is gonna be in the young kids, the young boys, and then type one macular telangiectasia. You'll often see that in older patients. Type two is the most common. I feel like type two we talk about all the time. I think you guys probably know type two mac tel pretty well from fluorescence or being in Bernstein's clinic with the studies that he's in. And then type three mac tel is really rare, and I actually don't even know if I've ever seen type three, but they have bilateral disease with retinal capillary obliteration. I can't even think, last night I was trying to think of a case off the top of my head and I don't think I've ever seen it. So I don't know if you guys ever will. So type one, so you can see the similarity with Coat's disease. There's a lot of exudation. And this exudation though is not quite as severe as we were seeing in the pictures of Coat's disease, but it's in the temporal macula and they can have macular edema. Another picture of type one Coat's. Type one B, so one A is the congenital type. Type one B, you often see this in kind of middle-aged males and they'll just kind of have a couple little microangerisms. They have excellent vision, really good visual prognosis. They're usually 20, 25. Often this is something that's not treated at all. And then type two, mactail. This is the one that we talk about a lot. This is often bilateral. There's a genetic component to it. You see RPE hyperplasia with these right-angled venules. They can get crystalline deposits in the macula. Singerman spots are these glistening white dots you can see in the retina and then advanced stages can get caroidal neobascular membranes. So the staging of mactail type two. Stage one, normal exam, but you'll see fluorescein changes with temporal staining. And then stage two, you start to see grain of the phobia and then telangiectetic vessels on the fluorescein. Stage three, you get the dilated blunted venule that's kind of diving at a right angle into the retina. And then stage four is when you start to get pigmentary plaques around those venules. And stage five is when you get neobascular disease. You can see OCT changes on all stages. Here's some autoflorescent changes with mactail that you guys should be pretty familiar with. This is the crystalline-like deposits that you can see with mactail. Gloresins. That's pretty classic. It'll be bilateral temporal leakage late. And then the OCT changes are pretty specific for mactail. And you can see cavitations in the inner and outer retina. And then type three, this is the really rare bilateral capillary occlusions. They can have abrupt loss of vision. And then later, the telangiectetic vessels develop. And like I said, this is just really uncommon. So, type one, the Coates type, often that's just treated with laser. You can also use anti-veg gas. And then type two, there's really no known treatment. Dr. Bernstein's getting involved actually in a study for different treatment options. They don't respond well to laser. People have tried a lot of different things for those retinal cavitations, but there's nothing that really treats those very well. The only treatment really is if they start to develop a caritone vascular membrane, then you would want to start anti-veg treatments for that. All right.