 Okay, good morning. I think we're going to try and get started right at eight just because we have three presentations and we don't want to keep you guys late today. I'm going to give the first presentation, which is an interesting patient I saw in conjunction with the neuro ophthalmology department here who presented eventually with the central retinal artery occlusion as well as a branch vein occlusion. And this was all thought to be related to a CNS vasculitis, so something we don't see very often. To begin, the patient presented to ophthalmology through the triage clinic at the end of April and she had a 24 to 48 hour history of amorous fugix essentially. She had painless blackouts of her vision in the right eye. These lasted for seconds to minutes, resolved completely, never affected her left eye and this was a new event for her entirely. She otherwise is a 76-year-old woman. She has an interesting history of rheumatoid arthritis as well as a dysquoid lupus. So she has a lot of autoimmune conditions and she's on a chronic load of steroid per her rheumatologists for those conditions. You can see on her examination when we saw her at presentation the right eye vision was actually quite good at that time and this is with correction. She didn't demonstrate any afferent pupillary defect or visual field defect by count fingers. Her examination though did demonstrate some interesting nerve and retinal findings. There was a possibility of some optic and some hemorrhaging along the superior arcade that was fairly minor really. So there was nothing obvious, no hollen horse plaque or anything like that that you might look for in conjunction with Amorosis Fugex. So her her ocular exam was not acute but because she was having these blackouts of her vision she was, there were concerns for her basculopathic risk and so blood pressure was noted in the triage clinic to have a systolic greater than 190 and the patient even noted that this was unusual for her so she was in the emergency room and it was communicated to the emergency room that we were worried about cardiovascular and basculopathic risk factors as well as possible giant cell arthritis in the setting of transient monocular vision loss. In the emergency room though they just did a CRP which notably was fairly low and actually decreased from prior findings. Her blood pressure was a little bit better than what we had measured at 171 over 80 and so essentially the patient was sent home without further evaluation or consultation with ophthalmology. So the patient then represented to ophthalmology a little less than one week later and unfortunately at that time she told us that on the morning of April 26th which is the day after she originally saw a centria had sudden painless vision loss again in her right eye but this time unlike the previous times it didn't return to baseline and in fact she noted no improvement or changed the time of that vision loss. Had no trauma precipitating either the transient monocular vision loss or this permanent vision loss and she denied any other neurologic or health changes. So a little bit more about her past medical history in addition to the discoy lupus in the RA that we mentioned she's been diagnosed with manures she has anemia of chronic disease hypertension she has a history of TIA by report and a possible leaky heart valve again by patient report she's hypothyroid she has a high hyperlipidemia and she's osteopenic so her past ocular history she's pseudophagic but no history of any sequelae of autoimmune conditions in the eyes by patient report and she's been followed here by Dr. Mamelis for general eye conditions and has never been noted to have any intracula inflammation etc. So she's on a chronic lotosteritis we mentioned prednisone 5 milligrams daily per her rheumatologist and that's four presumably RA she's also on other medications per her medical history but nothing that would be pertinent necessarily to her findings she has no medical and no so at her follow-up visit unfortunately we noted now from 2020 she has light perception vision in her right affected eye and you can see she has quite a big relative afferent pupillary defect now in this eye on her slit lamp and dilated findings you can see that her eyes fairly quiet there's no inflammation to suggest any uveitic component and then her dilated examination was different than previous exam in that while they're still cate in her right eye she now has a 360 degree swollen optic nerve it's not as well documented in this photo but she also demonstrated a cherry red spot on this exam as well as some box curing the other eye as you could see looked fairly normal and had baseline vision so some additional pertinent history for her and some of the initial plan she was scheduled for a temporal artery biopsy due to concern for possible giant cell arthritis and her steroids were increased to 60 milligrams daily but in terms of her review of systems for temporal arthritis it was fairly negative outside of what she would already be expected to have chronically from the r.a. and there's disquad lupus and then in thinking of embolic factors or vasculopathic factors she has a stated history of possible aortic stenosis or leaky valve but no stated history of atrial fibrillation or plaque when I'll use they I put that in there because I was so surprised by that yeah this was confirmed by her family all right um so 76 year old Caucasian woman she has r.a. and disquad lupus and she's presented now with painless sudden vision loss in the right eye after multiple episodes of the hemorrhacist fugex and her exam demonstrates significant optic nerve edema pallor of the right eye focal intratenal hemorrhages in the cherry red spot so our differential diagnosis here it seems like she has quite a bit going on and this is kind of what we were thinking of initially and then I'll go through some studies we did to narrow this down but she could have ischemic injury to her optic nerve she could have any number of vascular black gauges either venous or arterial she could have a primary optic nerve problem optic neuratus this could be sequelae of malignancy or um uh infection uh and it is fairly unilateral so even though it doesn't fit completely you could think about uh intracular mass so to narrow this down we did a fluorescein angiogram and this was at her second presentation when she had poor vision and you can see here after consultation with retina we determined that we were correct in in our assumption that this is a quite delayed arterial filling here you can see the times noted and this is the laminar phase you're just getting to it about 48 seconds and so there's quite a delay but you can see as you go to the later time points that eventually there is filling and importantly you don't see any leakage or evidence of any vasculitis around the vessels that was one thing we were looking carefully for but even at the later time points that wasn't present and you can see that was her right affected eye and you can see compared to her left eye that there is no evidence of uh any abnormality this uh area here has been chronically noted as kind of an atrophic spot likely related to semacular degeneration and so we also performed a macular OCT and which demonstrates essentially in the right eye that there's some edema um there nasally to the fovea compared to otherwise fairly normal possibly a little thin left macula and we also obtained some imaging so the first thing we did was get an MRI and I it's a little dense here but to highlight um some of the pertinent findings they thought perhaps there was a recent infarct perhaps the distribution suggested a central embolic event um uh component of a microvascular schemia was also possible and then they also again chronic uh comment on chronic microvascular changes but to clarify this and this was really the most important um imaging modality the patient underwent a cerebral angiogram with Dr. Wilde in the neurology department and instead of suggesting an embolic phenomenon as you might um think of with for example a cherry red spot and possible central retinal artery occlusion in fact um he felt that there were many vessel uh luminal irregularities and that this was compatible with a vasculopathy like a vasculitis but we had searched and thought about you know um a retinal vasculitis and didn't feel like we found any evidence of that on her exam or I was completely quiet even on floor scene there wasn't any evidence of a retinal vasculitis so this was uh surprising to us but it still did explain her presentation to some extent so um to kind of go back to what we were thinking then um based on the uh additional data that we have we concluded in conjunction that this patient likely had both retinal retinal artery occlusion and a branch vein occlusion in the setting of a cerebral or CNF vasculitis that didn't involve the retinal and the retinal vasculature so just a brief word about um some of the things that this patient presented with and and um when you see a patient presenting with this just kind of the um take home points of what to do so amoroses for you fugex as we all know sudden painless temporary vision loss lasting two to ten minutes and followed by complete recovery um there's an actual amoroses fugex study group that came out with this um table here of causes inocular blindness that's transient and you can see we think commonly about embolic phenomena this patient would fall more into a hemodynamic phenomenon and then idiopathic so it's really um a broad differential and there was an interesting study that just came out in retina this month that they actually it's the only one I found where they actually looked at the outcomes of for people who have presented with amoroses fugex and what they ended up having and it was interesting to me that it seems like the most common thing was either the CR was the um CRVO with the cilia retinal artery occlusion which was that's just not something I would have guessed I would have thought maybe CRVO or CRAO um but relatively these were um less common in their study group at least but there there isn't another study group to compare this to this is the first of its kind but I think that this is good and just thinking of what could it be and then how do we work up the patient and so we'll talk a little bit more about that so for a central retinal artery occlusion as in this woman um we all are aware that the central retinal artery is off the ophthalmic which is off the internal and that clinically when we see these patients we classically think about a cherry red spot um given the infected and pale retina but it this isn't always present at the cilia retinal artery circulation is intact and the patients we think about presenting with this they're usually a little older they usually have vasculopathic risk factors and cardiovascular risk factors like hypertension hyperchryolesterolemia, diabetes, vascular disease in younger patients um we think more about um coagulopathies and um it would be more important to work these up uh in a patient that who didn't have other underlying risk factors and then um this is an important point for amorous esfugex as well as central retinal artery occlusion that there is an association with giant cell arteritis and that is uh potentially blinding in the patient's other eye and so it's important to really make sure that that's not the cause of their symptoms so in thinking about the workup um exclude arteritic cause and that classic done with uh ESR, CRP and um potentially that's been sources um you saw that this path to see uh what the potential cause of their artery occlusion was uh and potentially hyper hypercoagulable factors and then in managing these patients there's an acute a subacute and a long term um thought process acutely there have been a number of modalities tried to increase circulation through the central retinal artery um that we've talked about previously in other conferences digital massage um carbon dioxide therapy but these are not very efficacious I know Trent had a case of a patient who they actually did um coail and remove the embolus and she got a little bit of vision back recently so but she was presenting very acutely within the first six hours of her onset and so acutely you can think about some management strategies but the efficacy is low subacutely though you have to follow the patients and ensure that they don't develop sequelae like neovascular complications to the eye and um long term you think about modulating their overall risk factors as you've identified in their work up to ensure that they don't have a similar event in their other eye either from an arteritic cause or um embolic or non-arteritic so briefly branch vein occlusion as also we noted in our patient these are the noted risk factors for branch vein occlusion diabetes was not an independent risk factor um initial management is directed at modification of the underlying risk factor subsequent management is directed towards the sequelae such as ocular neovascularization immacular edema the gold standard has been laser photo coagulation for macular edema as well as PRP for neovascularization but um this is a nice review that was out somewhat recently summarizing some of the more recent trials um as we have all seen in our retina clinics we're starting to inject these patients um what rather than waiting three months to treat their macular edema and you can see that um many of the these strategies of injecting steroid versus anti-vegeta agents do show efficacy with people gaining more than 15 letters with some of these trials for example this trial here so um this is kind of shifting the paradigm of how we treat these but I think from a um perspective of someone who this patient is presenting to and you're not always a retina specialist it's important to kind of identify the risk factors understand what the treatment is um and what the sequelae could be and refer as well as help the patient to modulate their risk factors so in terms of our patient one month after the initial vision loss in her right eye um we saw her uh in follow-up and her course had been that she was hospitalized with the neural service for three days uh undergoing the workup and uh dosing of high dose uh steroids through IV she um tolerated this well she's continued now on oral steroids awaiting assistance from rheumatology for further immunomodulatory therapy but her vision is relatively poor and stable her APD is stable um and she's now demonstrating pallor of her optic nerve the swelling has subsided and I think this is frozen oh okay so her hypercoagulable workup um that we did in this uh interim of one month demonstrated um no other um risk factors that we were unaware of that that would change our management strategy and you can see all of them here um and so you can see that she did have a negative temporal artery biopsy we didn't feel like she had giant cell arthritis uh and then other autoimmune risk factors and as well as infectious risk factors were worked up and nothing was found so in consultation with rheumatology and neurology and after all of these studies were back we determined that in fact it was likely an autoimmune mediated cerebral vasculitis that was the cause of this and this is very uncommon I thought it would actually be more common but when I looked in it seems like it would be but um when I looked into the literature um just cerebral vasculitis and the retinal vasculitis causing retinal uh vascular blockages is not very common and this is a slide borrowed from this publication but essentially they're just summarizing what you can see um in these different uh inflammatory autoimmune conditions uh in the setting of cns vasculitis and I mean this one notes possible central retinal artery occlusion and um possible branch retinal artery occlusion but even what's postulated really um doesn't cover what we saw I did find a couple of case reports so the the first one is really most like what we saw branch vein occlusion followed by central retinal artery occlusion this person did have an inflammatory autoimmune condition but not r.a or lupus as with our patient but they do describe the same findings and secondly this publication this was more of a venous blockage actually rather than an arterial blockage but it was interesting because this patient did regain some vision after plasma exchange and significant immunosuppression and modulation so this is fairly rare the treatment is not at all uniform um and I think that kind of brings us to how we manage our patient and how we should be thinking about patients with amorous as fugex so again we'll you think you know if someone presents with this you think about what it could be and this is kind of a nice summation of what to think about and then where do you go from there well you can think about always an arteritic cause I think that's the biggest thing to rule out just because it could affect potentially vision in both eyes but then also importantly thinking about um symbolic factors um as this is the um often the most common cause um thinking about looking at the heart or the carotid arteries for a source of embolus those are very important things to be done in conjunction with their internist and then um the thing that I took away from this really was that in somebody with an unusual history multiple autoimmune conditions and in a quiet eye we thought with a quiet eye that um we were thinking of other things more common things for amorous as fugex because the eye was quiet so what I took away is that even in a quiet eye um it's good to consult with rheumatology and neurology to make sure that there's no um other inflammation in the patient that could be causing this in the cns for example so in a patient with uncommon respectors uncommon things can happen I guess and that's what I took away from it does anyone have any comments about how they would have handled it differently with this patient I think the thing that I most disliked about this case is that she presented with 2020 vision and the next time we saw her she had lp vision and I just I didn't think that there was anything that could have been done differently in her given her ocular findings that would have changed the outcome but I guess does anyone have any comments on that amorous as fugex okay thank you Dr. Warner it comes in the transfer on the blindness you said you were considering for an expedited evaluation for stroke because of the very high rate of upstream stroke or how many people lost a crink in the immediate time and you know obviously somebody's having a kind of blindness for the last five years you know it's just a headache I think that's a different matter but somebody who's never had work and somebody that came in with a headache worse the things that would have made a difference if this person had been sent to your as a tia and they had done say for instance as for what she suggested is in her eye that would change the whole tenor of what's going on because she obviously had evidence of surrealism in it as well and you know no offense about policy but the university don't kind of have that same mental process towards vision loss as they do towards stroke it they've all been brought into this brain attack concept so that's her suggestion and that was really a very important parameter for a long period of what you hear about so this patient it was but it was by him on Friday and what pressure the guy saw and she had to see what was going on but um it was I think by episodes and I spoke to the doctor I sent people were Diane or walked over and made it you know I was I talked about you know because I was concerned with the reaction that was coming back Wednesday to the fourth time yeah and I think that I think that that is it's good it just like the tia thing has to be a very shift I think that it's going to take a while before that really evolves into a process but I guess the only way you can get around that is you know by essentially calling the brain attack by which I don't mean actually on brain attack it by you know commonology yeah because that's the only way to get around the emergency room sort of I'm trying to approach people who are going to be a little bit more involved on the most and I just I I actually call the knowledge directly I just felt I spoke to the art and I'm like really concerned