 We're going to get started, since it's now 802, my apologies. I'm Chris Connery, one of the P2I3s. I'm going to be presenting today a case that I saw on a call, and that has kind of circulated through the Marilal Ophthalmology Clinic and also one of the P2I3 clinics. I'm going to entitle with it complicated optic disc edema, and you'll see why. I'm trying to change the title from what's on the flyer, just to make it a little less obvious, but I'm going to change the name, just to check the history. That's right, exactly. And so this was a 13-year-old girl that I saw on a Thursday night that presented to the primary children's hospital with the 10-day history of vision loss, and what she initially described as right and much greater than left. Her pacemote history was significant for Hashimoto's thyroiditis, and then a remote history of headache has kind of had a headache in what she describes as years, and also a BMI of 37, but was otherwise healthy, not taking any medications. She said that this vision loss developed while she was reading a book at school that literally within a minute went from clear vision in her right eye to extremely blurry vision to the point where she could not read the book any longer in really seeing the clock at school. She also endorsed metamorphopsia, but otherwise didn't really endorse any other kind of symptoms. Denied headaches, I asked her probably a half dozen times in ER that night. The neuro-opemology staff, as you can guess, asked her the same thing the next day. She also denied pulse title prejudice at any sort of recent weight gain, at least within the last six months. But prior to that, had endorsed, I think, a little 50 pound weight gain. And denied any medications, any changes in medications was not currently taking the medications at the time. Her story to expand on a little bit further, so 10 days prior to when I got to see her as the resident on call, I guess seven days prior to that, was seen by optometrists in the community who noted bilateral discodema and referred her to a retina specialist. The exact story there's a little, I'm not quite for sure the exact story, this is all outside of our system. The retina specialist then saw her one day prior to me seeing her in the ER and agreed there was bilateral discodema and then noted some sub-retinal blood. So referred her to primary children's hospital for further evaluations, bilateral discodema, mainly for an MRI and a lumbar puncture because he couldn't get her into a neurologist to be seen in the mall, is what they were saying was their own disappointment. So prior to me even seeing her, or I guess in the process to me seeing her, she underwent an LP with an opening pressure of 41, CSF labs would basically become unremarkable over the next 24 hours. MRI brain was unremarkable and then of course we were consulted for discodema and kind of further evaluation. And Chris, just for those in the room, what is the opening pressure of the limit? Yeah, so normal opening pressure 20 to 21 would be normal, extremely elevated. So then on that side exam, she was 2,400 in the right eye, 20 to 20 in the left eye. She had an APB in the right, X-chocomotility was full. She had a central scatoma in the right eye that was large enough that I had to pick up on top of the visual fields. She had an IOP, about the 20 in both eyes and then anterior segment was unremarkable. So on dilated exam, and I'll show you some photos of this here in just a second, but she had a bilateral optic discodema right, forced to the left. And then she had scattered some rep of blood and fluid that basically emanated from the disc and tracked into the macula and almost underneath the philtia of the right eye. So we can see here, those images, so a bilateral discodema, then subredal blood. And then, I'm not gonna mention it now, but I think looking back on these photos, this wasn't as readily apparent. But it looks like there may be a neovascular membrane that was not easily discernible in the ER, or even the base of the X-ray of the slow vantage. And otherwise fairly unremarkable, as that's kind of the findings that I've already mentioned. So at least at this point, we've kind of ruled out several things on the differential diagnosis of bilateral discodema. We had tossed around at least Thursday night that maybe this was pseudo edema, but we had an opening pressure of 41, so that was elevated pseudo edema-related optic disc drusen, IIH. This would be a very, very scary IIH presentation, possibly in fenestration that night if it was all related to IIH. We've already kind of ruled out an intranial mass lesion, CNS infection, we've done the same. The imaging staff and the neuro radiologist felt like they had adequately ruled out these sorts of bronchosis. So then I kind of add this last point because we were kind of trying to explain that all of the subredal blood and the significant vision loss in a patient that looked like a straightforward IIH patient. And so we basically thought, well maybe that's a problem you've asked to remember, maybe it's related to some pseudo edema, but we had an opening pressure of 41. So we weren't quite for sure exactly what was going on with the limited imaging at that point. So we brought her back the next day to the neuro ophthalmology clinic of the Dr. DeGruy and Dr. Neufelds. We did a visual field that had a large blind spot on the left eye and then in the right eye we did a golden visual field in her poor visual acuity denoted this large, simple scatoma. We then did a B scan to look for calcified drusen or I should say Dr. Harry did a B scan looking for calcified drusen. Has an elevated disc but no obvious calcified drusen and supports kind of optic nerve prusen. Then we did an F A that kind of sealed the deal at that point and I'm only showing images in the right eye because the left eye was unremarkable. But kind of that green discoloration we saw in the photos like I said it wasn't nears apparent on exam. You can see this area of leakage, this kind of peritapillary lesion here that progressively leaks as we go farther into the F A suggesting a needle vascular memory. Then we did perform an OCT that was kind of consistent with everything we've already shown and mentioned. So swollen nerve here and fluid that basically tracks all day in the macula and almost anything. So then we'll talk more about this but we started around diamox for IAH advocated weight loss and referred her to retina for this peritapillary neovascular membrane presumably for a vast treatment. So she was seen that afternoon by Dr. Hartman and Dr. Feitz underwent a single avastome injection and we'll talk about that here in a second with resolution or at least near resolution of the subrout of fluid a month later and visual appealing from 2,400 count fingers range to 2,040 with a month of a single avastome injection. And that's not uncommon for these cases and Dr. Feitz wanted me to point this out that she was faced by Feitz and so. So then on visual field and it's kind of hard to compare the golden visual field in the right eye of the initial presentation but she has this kind of enlarged blind spot with kind of tracking into the central visual field and then resolution at this point and a month later of the enlarged blind spot on the left eye. So what do we know about optic discidema and peritapillary neovascular membranes? I can tell you that when I was on call I didn't know anything prior to this but it's a fairly infrequent occurrence but I think it's under reported in the literature actually after talking to some of the retina staff and all mentioning that they've at least seen cases usually quarrel in their clinics. So it's an infrequent occurrence like I said in the literature but I think that may be under reported. A retrospective study basically looked at from a tertiary referral center looked at the incidents of IIH patients referred to them that had peritapillary neovascular membranes and they found incidents of 0.53%. So fairly rare even in tertiary referral centers in the literature itself from 1970 to the 2010 there's been 23 cases of adults presented in the literature and they've attempted about everything in a match. So a lot of this was prior to the advent of a vastance so we've attempted just lowering ICP which has shown that it will actually cause peritapillary neovascular membrane to needle-loop by just lowering ICP. We've done PET and ART on the laser I say we is an ophthalmology community not me, myself, I wasn't born yet. Then we've tried PET and ART on the laser those have kind of fallen out of favor. An optic nerve sheath illustration has also been attempted but seemed to work as well. The exact mechanism of these treatments is not clear but do seem to work. Then with the advent of anti-vetchup in 2009 the first peritapillary neovascular membrane related to IAH was performed and with the single injection of anti-vetchup so a vastin complete regression of the evascular membrane was resolution of vision from basically count fingers to 2020 within six weeks of injection. Unfortunately there are some cases that are refractory and that's not the majority and the majority actually respond to a single evastin but there is a reported case out there that required four injections of anti-vetchup therapy with basically no effect on resolution of the sub-reval fluid and also return the baseline visual outcomes or visual acuity. So there are refractory cases but for the most part they seem to be exquisitely sensitive to being a single anti-vetchup therapy. In the pediatric population it's even more rare that there have been two prior reported cases in the pediatric population. The first was actually described in 2010 and for unclear reasons but kind of similar to lowering ICP this patient only underwent a diagnostic lumbar puncture but I guess it was also therapeutic because they had complete involution of the peritapillary neovascular membrane with just the lumbar puncture alone. Initial diagnosis. Then the second case was in 2013 due to so the patient started on dimox to treat ICP but due to worsening sub-rutin on blood they elected after kind of an extensive conversation with the family about anti-vetchup therapy and the risks the kids elected to perform a evastin injection and noted the resolution of the evastin membrane with single evastin injection. Then if we look at just kind of some simple studies looking at neovascular membranes in the pediatric population so there was a two year retrospective study looking at all the reported neovascular membranes at this specific center and the underlying etiology of those neovascular membranes they found 36 sites so fairly low numbers in the pediatric population at this institute but none were related to optic disc. 47% were related to inflammatory conditions to some sort of mediated phenomenon. And then just to kind of put this in there this is also important in a differential especially these presentations. 8% were from optic nerve head bruising. So fairly rare entity and maybe even more rare in the pediatric population. I still think at least from the discussions that have some of the retina and even neuro-ophthalmology stats maybe under-reported. Not quite as sure in the pediatric population. The mechanism of why this occurs and elevated intracranial pressure discidema is unclear but there's kind of two hypotheses and most individuals think it's a combination of the two. But the first is that there's a disruption of Brux membrane that is basically resultant from discidema that then allows in growth of vessels from the cordic capillaries. Then that's kind of the other side of that hypothesis is that due to discidema there's this chronic kind of axonal swelling along the optic nerve to at least a hypoxic environment, VEGF production, and then subsequently neovascular mutation, possibly being peripatillary neovascular membrane. Most people that have published on this would say that this only seems to occur in chronic optic nerve edema and their rationale for that is kind of following. So if they look at patients that present with peripatillary neovascular membranes in the setting of IIH, 50% of them will deny any sort of headache history similar to our patient. So they're at least hypothesizing that these patients aren't undergoing the normal IIH surveillance, aren't having their optic nerves evaluated and therefore have chronic disc swelling allowing the peripatillary neovascular membrane to grow from this kind of chronic optic nerve swelling. The other 50% of patients that's just their thoughts, I don't know that necessarily explains it all, but then that's kind of further supported by a study that looks at all of their peripatillary neovascular membrane patients in long-celled IIH, and it seemed to be that patients that they had followed for two to nine years, so chronic symptoms that followed for IIH, they never really saw peripatillary neovascular membranes occur until kind of late with chronic disc swelling. So that's kind of their rationale for kind of this chronic disc swelling being a growing etiology. And so that's at least peripatillary neovascular membranes related to IIH. There's a lot of other things that cause peripatillary neovascular membranes. I of course didn't talk about those, but that's at least something we need to be aware of because like I've already mentioned, I think it's much more common than maybe even literature supports. And it's something that we can do, something of talent in most cases. So any thoughts or questions? And I guess before we get to that, Cooper has already begun surveillance or I've already done surveillance for the peripatillary neovascular membranes. Don't worry. Any questions, thoughts, concerns, yes, Dr. Olsen? So if you think about the fact that it was exquisitely sensitive to VEGF inhibitors, it's hard to imagine that the theory that this is largely mechanical makes sense. And there's obviously got to be up-regulation of VEGF. And if you think about the anatomy, you could imagine the swelling and the rest of it in some area of the localized VEGF increase is certainly got to be part of this order. VEGF has to be a key molecule in this process. Probably get sensitive. So the other thing is that we're talking about how common is this. There can be small localized areas and if they never break complete, and then they get their sort of tumor treated. And it's resolved, obviously, in an endemic sense, if you're getting some of that swelling and whatever localized mechanical issues causing localized ischemia, there may be a lot of these. We just never looked. And it could be the issue that Bob Hopman loves to say always about W and N often beats on a gentleman's hand. We never looked. That's right. And this may be an example. So I suggest this could be an interesting study. I don't think, but let's just take everyone who comes in and maybe has had an intrependent open open. This had hypertension, a pseudo tumor for a period of time and just dual CTs of what they did. There may be small cell numbering way more often than the funny. Yeah. And what I had, didn't mention it. Yeah, they did just a very interesting study because like I said, there's numbers there, but wouldn't they find that there are ACEs in the medics, small ones that are there on a much more personal basis? Right. And what most groups will also mention is that a lot of these chronic disc edema patients don't present until they have significant vision decline. And that's usually due to an approach that a subrennial was into the macular and the phobia fast. So, yeah, it's, I think it's, like I said, and Dr. Olson also, yeah, Dr. Kerri. Very nice, Chris. Just a question, your view of the literature about optic dysrhusin and subrennial evascularization, peripapillary, did you get a number from that at all? Yeah, so at least in the pediatric population, I didn't specifically look at adults. But this very, very small study mentions that 8% of peripapillary neo-vascular membranes would be associated with optic nerve tumors. That was pediatric. As a pediatric pediatrics. And like I said, I mean, this study, there are 36 patients. So, I mean, this could be much higher, much lower, but that's literally the literature on peripapillary neo-vascular membranes in the pediatric population. I didn't specifically look at adult population, but at least from a quick kind of review, it looks fairly similar to that. But, so I don't know the exact numbers. I guess around 10, just seeing a lot of derudia just makes kind of a number. It's somewhere in that range of 5% to 15%. It's not large, but it's not something to ignore. Is that you, Eileen? I'd like to ask the neuro-optimologists about the role of FAA in distinguishing papillodema from pseudo-papillodema. Does lack of hyperflorescence at the disc eliminate papillodema or papillitis as a possibility? We absolutely, the axoplasmic kind of congestion really makes them a plot. And so when we're looking at pseudo-papillodema, that's one of the things that we look for. It's just really hard to do FAs on everyone when we have such clinical exam findings that are obvious. But we could go back and do, if we're going to do a study with OCTs, we could add in FAs and also work. So Dr. Hollis, this is great. One of the things that we have trouble sorting out at times are the kids who have non-calcified optic nerve head drusen from chronic dyscadema. And I'm wondering with these, the asymptomatic, I don't have a headache kids who look like they have chronic dyscadema, how many of those actually would treatment, I mean, the nerves change, whether they actually had buried drusen and we know that's a cause of subrenominal nevascular membranes around the disc. I mean, the ones I've personally seen over the years, one that strikes out about 20 years ago was a little girl with chronic, chronic inflammatory dyscadema who had fluorid subrenominal nevascular membranes unfortunately ultimately expired from her anti-inflammatory treatment to try to control things was just a unmitigated disaster. But these could be very difficult. Yeah, very difficult to dissect. I mean, the one thing that kind of forced our was the opening pressure of 40 to one. So at that point, we had to assume it was real great even without identifying optic nerve. You got a elevated opening pressure, I think that's different. But some of these, I wonder how rigorously things were sorted out in some of these reports. So that report of 36 patients, I would not say that's the most rigorous study that I've ever seen. There you go. I mean, they have something out there, right? So that's better than nothing, but it wasn't the most rigorous. Looks like we need them. Okay, well, so any other questions?