 So I was interested in this case because there's this fairly recent article that talked about this new vascular phenomenon in the retina and there was this case that I was dealing with at the VA and I thought that this would be an interesting way to kind of highlight this new disease phenomenon and also talk about the possible ways that we can alter our management in terms of dealing with hard to manage leaky vessels. Thus the title of this presentation. So it always starts with a case, right? So we have a 75-year-old male who came to our clinic complaining of zig-zag curvy visual disturbance. He said it's been ongoing for about three days, occasionally noticing a dark spot in his visual field from the center of his visual field, but denied any flashes, floaters or headaches. His past ocular history was just significant for cataracts and some mild diabetic retinopathy. On examination he had some mild visual acuity change as well as trace ocular surface disease as well as just mild cataracts. On dilated fundus exam, as you can see with his right fundus, it's pretty much unremarkable, no issues to speak of. With his left eye, left fundus, it's probably hard to appreciate but if you look in the center, you might be able to tell that there's his blunted foveal reflex as well as this kind of this rayish, indistinct area to the fovea. On OCT of his right eye, as you can see it's pretty much unremarkable except for an ERM. With his right eye or with his left eye, you can see that he has this, based on the infrared on FOSS view, you can see that there's this central foveal hyporeflectance and then when you go through the OCT, what you notice is that he has this Cirrus PED and in terms of the central macular thickness, it was about 403 micrometers. IVFA, as you go through time from the early phase down to the bottom with the late phase, you can see that as it starts to evolve, you can see this hyperfluorescence significant of a pooling effect in the foveal area with an appreciable notch to the pooled area as you can see in this image here. So again, we have this 75-year-old male who had this three-day onset of left eye metamorphopsia with the central negative scatoma and then on examination had this mild visual acuity change subretinal fluid on OCT as well as the Cirrus Notch PED. So when you think about a differential for a Notch PED with subretinal fluid, all of these vasculopathies can potentially cause a Cirrus Exudative Process. However, what we need to do is further delineate what could be the cause of this particular patient's issue. So this will need, this requires further testing. And so with ICG and geography, we can actually highlight what may be going on in the Coroidal area. So as you can see in this video, what you first notice here is the central hypofluorescence to the ICG. But for the most part, pretty much unremarkable ICG angiography. And then even later on in the angiography, you can see, again, the underlying Coroidal vessels are unremarkable except for this overlying hypofluorescent area. And again, highlighting that Notched region to the area of the hypofluorescent region. And then OCT angiography is another means that we can actually identify and even look at the vasculature. This is a representation of a normal individual with an OCT angiography. And what I wanted to highlight is just from looking at these top four panels here, you can see this is the inner retina with the superficial plexus, capillary plexus, this is the deep capillary plexus. And then we get into the outer retinal area. And then we go into the coriocapillaris. And this is pretty much what you would expect to see on a normal OCT angiography. In our patient, what you see here is, again, normal inner retina layer, capillary plexus, outer retina layer seems unremarkable. But then when you look at the coriocapillaris area, there's this large hypo, or just large dropout of vascular tissue. And then, of course, you can see where that corresponds to in the OCT itself. So at this point, our plan for this individual who has this Notched PD, we wanted to start with anti-vegfs. So Avastin was a common first choice of what we used in the clinic. After about a three-shot series, we would then re-evaluate to see what changes might have occurred as a result of therapy. So over a segment of three to four months, you can see that as we were injecting the patient with the anti-vegf, you can see that initially he had a decrease in his central macular thickness, but then eventually plateaued at some point. And so we were kind of at this stage scratching our heads as to what's going on here. Essentially, you have this process that's not amenable to anti-vegf therapy. Could there be something else going on that might explain why this is occurring? And should we alter our treatment as a result? So this gets into this recent discovery of this perifovial exudative vascular anomalous complex, or PVAC. I was first discovered by some French authors in 2011. And so this case report, they looked at two individuals and noticed these large perifovial aneurysms that weren't associated with any type of retinal inflammation or vascular disease. In their paper, their initial paper, what they talked about was these aneurysms that had these intra-retinal pathologies, but had no abnormal coroidal findings. And in terms of their OCT, they were able to show that there was this hyper-reflective lesion around what they call this PVAC disease, as well as this surrounding intra-retinal cystic edema. And then they obviously tried and attempted to treat with anti-VGF, in this case, locentis. But again, they had no actual improvement to these patients' condition. And so their hypothesis at this point was, could this be some type of retinal vascular endothelial cell degeneration? Thus explaining why an anti-VGF therapy may not be effective. Later on, this paper came out which talked about expanding the spectrum of PVAC and isolating or at least trying to figure out what are the multimodal ophthalmic imaging features associated with this disease. And in order to help us understand better how to classify this disease. So this is a retrospective cohort study, a multi-center approach looking and they were able to isolate 15 eyes with a mean age of 73 for their patient population ranging again between 46 to 90 years of age. Their inclusion criteria, they were looking at individuals older than 18 who had this unilateral lesion associated with this perifogial large retinal aneurysm. In terms of exclusion, they wanted to just exclude any other type of retinal or systemic issues that could confound the findings that they were looking at. So this is a very busy slide. What I wanted to basically highlight in this graph or this table is just to show that the, again the mean age was somewhere around 73. It was a unilateral process and some of these individuals actually had other conditions associated with it like ARMD as well as myopia. But in terms of the best visual, best corrective visual acuity, the worst visual acuity note it was about 2040. So not much in terms of visual acuity deterioration. And so the authors in this paper basically concluded that this disease phenomenon has pretty much a stable course. Although it's not successfully treated with an anti-vegeta therapy, functionally and anatomically it seems to just maintain a stable condition. And so what they were able to find out with OCT was that, you know, when you're looking at individuals with this PVAC phenomena, they typically have this round hyperreflective lesion. They have these intraretinal cystic spaces. And again, the most important part is there is no curidoneobascularization associated with this disease. And this is a representative sample of a patient from their study. And as you can see, there's just intraretinal vascular lesion here with no signs of any curoidal change whatsoever. And in terms of their ICG and IVFA, they were able to show that there is these well-defined retinal hypoforacin lesions with variable leakage in late frames. And that shows here, again, with this representative of ICG and IVFA, again, just kind of this late leakage with no other findings on the imaging. And then, of course, with the OCT angiography, what they were able to find was that there's this rarefaction of retinal capillaries around the lesion with no connectivity between the retinal capillary plexus as well as the coriocapillaris. And again, this is a representative image showing the capillary system in the retina versus the coriocapillaris. And there not being any type of connection between the lesion here with the coriocapillaris and, of course, this rarefaction of the surrounding capillary bed. So there are many hypotheses regarding the pathogenesis associated with PVEC. But for the authors, they thought that this is probably associated with an idiopathic retinal vascular abnormality. One thought is that this may be a variant of type 1 mactel. But this is most likely not the case because with mactel, you can see that there are differences in the anatomical changes as well as the demographics of who are affected, as well as their response to anti-vegeta therapy. The other thought is that could this be a variant of, say, type 3 neovascularity, a rap lesion. Again, this is probably not the case because when you look at the actual anatomical changes associated with the type 3 corioline vascularity or neovascularization as well as PVEC, they're different. And then, of course, the response to vegeta therapy is totally different between the two. So this then goes back to our patient. Does our patient have this PVEC phenomenon? And now, what I want to do is compare their representative sample or patient versus our patient. And, again, showing the differences in the OCT with his intraretinal pathology versus more of a sub-RPE, sub-SIRIS-PED, as well as some sub-retinal fluid, but not much in terms of intraretinal changes. And then, looking at the IVFA, again, their patient shows this anomalous aneurysm with this late leakage. In our case, we had this patient who had pooling along with this notched PED. And then, finally, when you look at the OCT angiography, you see that there's this lesion here that has no connectivity to the coriocapillaris and this peri-lesional dropout of capillaries. In our patient, the inner as well as the outer plexus was unremarkable. And then when you look at the coriocapillaris, there is this large dropout here. So, again, going back to the question, does our patient have this PVEC phenomenon? Most likely not. This is probably more likely a polypoidal-coortovascularopathy. And our plan in terms of treatment is to try a different approach. Instead of doing a vasten, we wanted to try ILEA and see if there's a possible response with ILEA. Otherwise, if there is no response to three trials of ILEA, then we could go into either photodynamic therapy or even a sub-threshold micro-pulse laser as a potential way of treating it. And that's it.