 Okay, so we'll go into the second half here. I've got a lot of slides so I'll go relatively quickly, but feel free to stop me at any point here. Hopefully everybody got a chance. First off I'd like to thank the entire family, Tiffany, Lisa, and Rose for coming today and being a part of this. We really do appreciate it and the photos don't really do justice to what we see, so we appreciate that you're able to come in. But you will see a lot of photos, and everything I put up here also is just from reading the notes, so if it's wrong I apologize. So we'll get going here. So I have to put this disclaimer on everything that I do. These are my views, these are not Department of Defense, Department of Navy policy views, or otherwise related to anything Department of the Defense. So, and that's a carrier strike group, which is by far the most dangerous weapon in the world. Alright, so we'll just get right into it. So our case presentation is a case that recently we did surgery on, and so that's the one I'll start with here. It's a 34-year-old female with a history of cavatary disc anomaly who presented with six days of blurry vision in her left eye, which was her good eye. She noted sudden central distortion and significantly decreased vision. She didn't have any pain and she states that this is similar to what happened in her right eye many years ago. Okay, hold on one second here, I just got to make sure I've got something. So, she had no other significant ocular review of systems, and her review of systems otherwise was negative. Her past medical history is significant for migraine headache and PCOS. She has again the terioptic disc anomaly in both eyes, and she had a serous macular detachment in her right eye in 2007, which was repaired with a vitrectomy, endolaser, and SF6 gas at that time. And then her family history is obviously significant for optic disc abnormalities in both her mother and sister. And she also has a grandmother who had poor vision in one eye that died in her 30s. So, or excuse me, had vision problems in her 30s, but was never diagnosed as far as we know. So, this photo was taken the day that she presented this year. So, this is her current photo, and they didn't really have a good macular photo. So, this is the photo that we have. So, on her examination visual acuity in the right eye is 2800, 2125 in the left eye. And other than having had a vitrectomy and a central scatoma in the right eye, her anterior exam was otherwise unremarkable. So, here is her right eye. And you can see an abnormal optic disc with peripapillary RP drop out in hyperplasia, consistent with previous laser to that area. And then, it's tough to see here, but the central macula is just slightly out of focus here. Here's her left eye. Sorry, the previous picture was actually from 2007, which was the last time we had seen her. So, I apologize, this is her current picture. So, you can see elevation of the entire macula. You can see a pseudo hole of the macula, and you can see obvious abnormality of the optic disc with significant cupping. Here is her OCT of the left eye, or excuse me, of the right eye. And you can see a serous macular detachment with almost complete loss of the inner-outer segments there, which notes some chronicity to this detachment. And here's the OCT of her left eye. And you can see a large central area of subretinal fluid within significant amounts of inter-retinal fluid, both nasally and temporally, as well as superior and inferior. So, kind of a different configuration than her right eye, and that's pretty typical. We'll go into kind of the pathogenesis and what happens in these eyes. And here's some photos just through her nerve, superior right through the center of her nerve, and then inferiorly through the nerve and macula. And you can see, you can't even see the bottom of the optic cup in these. And you really need a swept-source OCT, where you get more thickness to be able to see the bottom of a lot of these optic nerves. And then you can see her left eye here, and you can see the same thing, the macular abnormality, and then as well just complete absence of any tissue posteriorly in the cup there that you can make out due to the amount of scan we're able to get. So, we'll switch there. So, that's her presentation. And now we'll go to her mother, who had three weeks of vision loss in her left eye. In 2014 she noticed some distortion and so she presented and you can see, again, she has an abnormal optic nerve and she has serous maculopathy that looks very similar to our first patient. So, she went under, she underwent part of the point of attractomy with endolaser to the edge of the nerve and SF6 gas tamponade. And you can see one month post up to six and twelve months. And so it took somewhere between six and twelve months for the fluid to resolve. But it slowly did over time. And this is her most recent photos and the most recent exam, she was 20, 30 in both eyes. And she does not have detachments in either eye. She's flat. So, we'll move on to our first patient's sister and here are photographs from 2005 when she first came in for her right eye and she was already status post laser, just some focal laser to the nerve for a serous macular detachment. She had not had surgery at that point. So, she was treated with attractomy, further endolaser and SF6 gas tamponade and she did well and flattened. And interestingly enough, this is one and we'll go into this a little more with familial optic disc abnormalities. But you can see the progression of her nerve from 2005 through 2014 in the left eye. And you can see that in 2005 I would, if I didn't know better I would call that a completely normal optic nerve. Even in 2010 I would say that's probably still normal. And then in 2014 there's an obvious optic pit with significant progressive cupping. So, and that's one of the interesting findings in these familial forms of optic disc abnormalities is they're not congenital in that sense. They're definitely genetic, but they're not they tend to be progressive or certainly can be progressive. So, she complained of she already had a history of having a part of this point of attractomy with gas and endolaser in 2003 and 2005 in a right eye. But she complained of sudden vision distortion in 2014 in May. In her left eye she had distortion and her visual acuity was down. And you can see she has a small cirrus, excuse me, she has a small amount of inter retinal fluid there. And the optic pit obviously which wasn't present in 2010. So, here are her post op results and so in a month you can see she kind of has a cirrus detachment. But then two months she's almost completely flat and three months she is completely flat. So, she did well until about a year post op. And then you can see the area of laser scar. Do we have a laser pointer here? Nope we don't. So she did well until about a year post op and this is not exactly through the fovea. But she's got superior fluid there. She's got the new laser obviously that you can see on both OCT and on the color photo there. And she's got some inter retinal fluid as well. So, she had a re-accumulation of fluid after being completely flat. And she had focal laser and C3F8 bubbles three times over the past year, most recently in March for this. So, try to help it go away. So, we'll move on to the portion here. Those are just again photos of the nerves of the left eye of the two sisters. So, cavatary disc anomalies will just go through the common ones and kind of what they are. So, optic pit is usually a focal cavitation that's isolated usually to the infrotemporal or temporal disc. They're typically small and they typically have a sporadic inheritance although there are some families that have autosomal dominant inheritance of optic pits but it's only been described a few times. It's mostly sporadic. Optic nerve colobomas are large excavations typically located in infrotemporal aspect of the disc and they can be anything from just involving a little bit of the disc to involving the juxtapapillary and peripapillary tissues. And they are no mutations include PAC-6, PAC-2. There's MAF, CSH-10. There are no genes that have been identified for the autosomal dominant form of colobomas. However, and these are typically sporadic as well but there are some families with autosomal dominant inheritance which may fall into kind of our category. And then morning glory disc would be the other cavatory abnormality and they have enlarged papilla with funnel shaped elevations of the optic disc and the peripapillary retina. They typically have a radial arrangement of the vessels and enlarged or multiple silior retinal arteries. They have a rim of elevated peripapillary tissue and then they tend to have a central glial proliferation in the base of that disc and that was described because it kind of looks like a morning glory flower. And again most of these are unilateral and sporadic. It's very rare but there has been a single case report of bilateral morning glory disc that included PAC-6 as the gene mutation. So familial cavatory disc anomalies have a little bit of a different thing. So there's been a large four generation family with 16 individuals that have been studied out of Iowa and they looked at the genetics of this disease and this was in 2007 they were able to locate this to the 12Q locus by a linkage analysis and they tested three genes, the GDF11 neuro D4 and WIF1 as they were all noted to be neuro development in that area and sequenced those and they did not find any abnormalities in those genes. So at this point the genetics are still kind of somewhat unknown although at least we have an area. So look for it. And so again this case study showed four of 16 individuals had progressive optic nerve abnormalities and ten of 16 had bilateral involvement. There was serous maculopathy and about half of them and five were bilateral and then the phenotype varied significantly from infected, excuse me, affected individual to affected individual. There were people who were just barely kind of because they were in the family you look a little harder and you could see something to those who had almost full morning glory disc type appearance. And again it's autosomal dominant as well. They determined. And some of the clinical features about 50% will develop a serous maculopathy. The onset is typically in early adulthood and we'll go into why that's important in terms of the possible pathogenesis of this disease. They're confined usually to the posterior pole and they tend to have relatively poor outcomes with 80% having 2200 vision or worse upon final treatment. So the clinical progression of this disease, the thought is that it normally develops in a two step process. You get intratenal fluid that's extending from the optic nerve into the macula and then if allowed to continue, then you tend to get a serous attachment through what they presume is an outer retinal break basically allowing the intratenal fluid to go to extend into the subretinal space. So it's not actually a subretinal space optic nerve communication. It's actually intratenal space where they tend to accumulate fluid and so that's important for the pathogenesis of this disease. And usually then just as I said the subretinal fluid doesn't communicate directly with the optic nerve, which is kind of important. So the pathogenesis is thought to be dysplastic retina and then they have some sort of a scleral or laminal kerbosa defect or enlarged openings that allow fluid and then you get this dysplastic retina that protrudes into the sub arachnoid space and communicates with it either through small openings in the sac or through small openings in the retina. And this has been confirmed by histopathology as well as swept-source OCT that you see this very dysplastic retina herniating into this deep area of abnormality and then back out again and you can actually find micro breaks on swept-source OCT in the outer retina. So the two models of evidence for them, although nobody's been able to confirm this, are it's either a result of vitreous traction or it's a result of pressure gradients or some would say some of both. The evidence for vitreous traction is that paris plaintive attractomy is the primary treatment that tends to work in these patients and just paris plaintive attractomy with PVD results at least in one small study of 11i is 10 of them redetached without any laser treatment to the optic nerve. Macular buckling has also been successful which would be more consistent with some sort of traction on the optic nerve or on the macula. Abnormal vitreous stranding is seen on swept-source OCT in the central optic cup and spontaneous PVD can resolve the maculopathy without any treatment at all and that's been shown in some patients. So there's also people who think that it's the differential pressure gradients between the CSF space, the intraocular space and the sub-retinal space that caused this. Some of the evidence for that is that they've shown vitreous substitute migration both intraocular gas as well as silicone oil has migrated into the sub-retinal space from the intraocular space after paris plaintive attractomy with the use of these. It's also been shown that they've gone into the CSF space into the sub-arachnoid space as well as gas. So there's obviously probably some overlap between these two scenarios. The big variable would be that ICP actually does vary and would allow for a possible large pressure gradient depending on the patient's positioning whether they're supine or upright. ICP can change significantly in the peripapillary area. And then it's also shown that vitreous can be incarcerated into these optic disc abnormalities thinking that it's actually kind of the pressure gradient sucking the vitreous into the retina when there's a pressure gradient. So the source of the fluid is also a question. So the source of the fluid being either liquid vitreous or CSF. So there's vitreous mucopolysaccharides in this fluid. India Inc. studies in Kali dogs which have a very classic optic pit abnormality have shown that India Inc. in the vitreous cavity goes into the sub-retinal space. And then you've seen the sub-retinal fluid, or vitreous substitute migration which also would say that this is possibly from the vitreous. But then this happens in infants who have a completely fully formed vitreous and should have really no vitreous liquefaction. So that doesn't really make sense for some liquefied vitreous getting in there. Again, intracranial vitreous substitute migration makes you think that there's something in the sub-arachnoid space communication that allows that to travel there. And then also nerve sheath fenestration reports have shown that if they do a nerve sheath fenestration to lower the CSF that can tend to resolve some of these detachments as well. So and the pressure gradient that's required to, even if you have a 200 micron opening in the optic nerve, to allow for vitreous substitute to go into another space due to surface tension is about 11 to 12 millimeters of mercury. So even with a really large hole it takes a relatively large pressure gradient to allow these things to migrate which is why we don't see it that often. So the management of this condition is a little bit up in the air because it's not that common. But pharmacologic treatments have been tried, mostly case reports, with variable success. Both acetylzolamide and corticosteroids have possibly had some effect. But it's difficult to discern because this sub-retinal fluid can wax and wane in an individual whether you treat them or not and even after treatment. And that also lends a little bit of credence to the pressure gradient differential depending on how much the pressure is changing. You can do laser photo-quagulation and you can treat right through the fluid and that actually can be helpful because it can be protective of the nerve fiber layer which we're always worried about damaging when we photo-quagulate in the maculopapular bundle. So you try to create a very light laser burn so that it's just uptake by the RPE and into the outer retinal layers and try to not injure the nerve fiber layer. And the typical 4-5 rows of light laser that are adjacent to the nerve about 180 degrees temporally. So that's kind of the typical. The surgical management which is the most common management would be vitrectomy typically with a creation of a PVD since that's thought to be possibly involved in pathogenesis. Usually with tamponade with a gas and then laser most typically but not always. And then with those you've noted up to 90% success in some studies with resolution of their fluid. And then macular buckling has also been performed which is shown to work in these cases. So we'll get back to our original case. So our patient underwent parspoint of atrectomy and laser nerve to the border and SF6 gas tamponade and she's a few weeks out from that now. So questions for me? Comments?