 All right, so our first presentation is by Jason Hooten. He's a fourth year medical student from the University of Washington. He graduated from high school in Kansas City. And he went to BYU, Idaho. And he's going to be presenting Terian's Marginal Degeneration Case Presentation and Discussion. Good morning. Like I said, my name is Jason Hooten. I'm a fourth year medical student at the University of Washington. And today I'll be talking about a case of marginal degeneration that I've been working on under the direction of Dr. Moshe Farr and Dr. Edmonds for about the last week and a half. It's an interesting case, I believe. It's got good pathology, but it's also been fun since the patient is actually from Iran and lives there in Iran. So I've been corresponding with some doctors there. The language has been a bit of a barrier, to say the least. And I appreciate the patience. But it's been pretty fun, and I appreciate the opportunity. So let's start off and look at the patient. I guess this doesn't look forward. We have a 16-year-old female who comes in with a complaint of decreasing vision in her left eye over the last month. She denies any pain, photophobia, itching, or tearing. Her history is known before a case of juvenile idiopathic arthritis. She's had since the age of five years old and has these symptoms that go along with that. We look at her. She comes in the door. So obviously, she has a little bit of a sensory exotropia and then some significant edema in that left eye. Continuing with the history, she has had a couple of episodes of this in the past. Not nearly significant, as this case she says. And they were basically resolved with some sodium chloride, 5% ointment, and some drops. They resolve spontaneously. But again, this is much more significant this time. She has no history of eye infections or contact lens use, and like I said, has the history of arthritis. Social history. She works as a carpet weaver, which is obviously complicated by the arthritis. She lives in a small city quite a ways away from the clinic, which makes follow-up difficult. She has been started on methotrexate weekly and prednisone daily, which has given her some relief of symptoms. But as you can see in this picture, her diseases has already started to cause destruction of her joints. In that third digit of her left hand, she already has a bit of a swan neck deformity. And I've actually seen more recent pictures that show that it's progressed even past that. So the exam, when she presents, her vision is 20 over 40 in her right eye. Counting fingers at 10 centimeters in her left eye. Best corrected visual acuity shows no improvement. The right pupil does seem to respond to light, but the left pupil isn't well-visualized due to the edema. And it's obviously difficult to check for a relative afferent pupil area defect. Extracular motility is full, except for that sensory exotropia. And then everything else is basically unremarkable. So let's look at the eye. This is the right eye. We can see here some injunction of the conjunctiva. The real pathology, though, is here in the cornea. We can see circumferential peripheral thinning of the cornea with some opacification. There's lipid deposition, especially at the leading edge. And it's hard to tell here, but there is a little bit of neovascularization, I think, is also occurring. It's also hard to tell from these photographs, but the physicians describe that there is thinning of the cornea, bestieing on slit lamp. And also some circumferential bulging or ectasia is what they also describe. The other thing here that's kind of hard to see, but if we look closely, is that in this inferior nasal region of the cornea, there is some cloudiness, some edema. And that correlates well when we look at these next couple of pictures. This isn't the greatest photograph, but there is what appears to be, in the posterior aspect of the cornea, a detachment of decimates membrane. And if we look at the OTC, it correlates again well with what we saw earlier. There is what appears to be a cyst or what we're calling a partial detachment of decimates membrane that coincides exactly with where we saw that edema earlier. And if you also look at this picture, there's some thinning occurring here in the periphery and then some other maybe edema or cystic type changes as well. Here's an orb scan looking at the corneal thickness. And again, it's really obvious what we're looking at here with this significant thickness in this region where we saw that cloudiness. And then it's basically thickness throughout the entire cornea. If we look at the keratometry, I wouldn't put a whole lot of weight in this just because the edema is probably giving us some weird results. We see thinning of the vertical axis, which it really isn't typical for terrians. So it's hard to see really how much weight we should put in this image. Moving on to the left eye, very similar to the right in the presentation, except for the fact that we also have the significant edema, which is what's causing her the problems with her vision. Again, the opacification, the lipid deposition circumferentially around the entire eye, it's pretty stereotypical for terrians. And again, it coincides really well with what we see on OTC or OCT. This isn't a superior aspect of the cornea, but if we followed it through, if we had time to look at all the different slices, it pretty much follows through most of the eye. We're calling this a complete detachment. And again, there's a little bit of thinning in the periphery. The corneal scan, again, probably not a whole lot of significance here. It does, the center and the rest of the eye does appear to be thicker than normal. But, and we do see a little bit of thinning there in the periphery, but I also wanted to show you a little bit better picture. This is from a pentacan, and this does a better job of illustrating this thinning that's occurring in the edges. We can see these areas of yellow and red that really shouldn't be there and demonstrate good pathology for this corneal thinning disease. And again, the keratometry, this is actually a lot more typical for what we would expect in a terian's patient. Vertical increase, excuse me, increased deepness in the vertical axis, causing significant amounts of astigmatism, which causes the normal change in vision for these patients. So the diagnosis that we already talked about, this is terian's marginal degeneration. This is a disease that has been around since it was first diagnosed by terian back in the year 1900, so it's been around for a while. I tried to look up some information about this terian individual, but I Googled it and really couldn't find anything. So after wasting probably more time than I should have, I decided to move on. This is a little bit better example of what you see in slit lamp with the thinning. There's a bit of a step off here that is even more pronounced in some other pictures. So the etiology, this is a rare disease. There aren't that many case reports actually about it out there. There's a predilection for males, three to one. It's onset, depending on where you look in the literature is 20s to 30s, but I guess by definition it's over 40. It, by definition as well, does not have any systemic associations, which is what makes this case kind of interesting. Signs, the disease usually starts out with some opacification, some small dots in the anterior portion of the stroma and the superior aspect of the eye. As it progresses, the thin panacea will form and kind of coalesce and start moving circumferentially. There's thinning that's obviously occurring, which is a significant part of the disease. It's usually a pretty slow process taking years, which is again why this patient is kind of an interesting example. As the disease progresses, we see again a stigmatism, a regular stigmatism occurring, causes decrease in vision, and then in extreme cases, we actually see perforation. The patients are usually pretty asymptomatic. They don't usually have any complaints until the diseases progress to the point that there is the astigmatism. At that point, it's usually just a bit of supportive care. Rigid contact lenses usually do a pretty good job of correcting the astigmatism. In more complicated cases, piggyback lenses work well. The thing to consider with these patients is that because of the thinning, they already increased risk of perforation if there is some sort of trauma, so we do encourage eye protection. And then finally, if the disease is again in its most advanced stages and it's looking like it's actually going to have a perforation of the cornea, then there have been examples of people performing lamellar penetrating keratoplasty as a more extreme measure. So this, what I've been talking about is basically what we call the traditional Terrians. There has been an inflammatory variant that was described by Austin and Brown back in the 1980s. And there has been more and more case reports correlating with what they found. These patients are usually younger in age. They have recurrent episodes of episcularitis and scleritis. Remember, technically Terrians is a non-inflammatory disease, but we see that and we see a difference in these examples. These patients also usually have faster progression, which correlates well with what we saw today. And then because it's an inflammatory process, top of the steroids do provide, excuse me, some relief. So what were the significant features of this case? First, it showed bilateral complete circumferential thinning. It's usually not that advanced and usually doesn't manifest itself in such a complete manner. The second thing is that there's actually no reported cases in the literature of a decimais detachment occurring with Terrians. And there is several examples in the literature of breaks in the decimais membrane and maybe a little bit of cystic changes and cysts forming, but nothing to this extent. So what we believe is that maybe this is just demonstrating the spectrum. It maybe starts out as some thinning in the membrane, maybe some breaks, progresses to assist, and then finally at the extreme edge as we see the actual detachment. And then finally the association with juvenile adiopathic arthritis. Again, by definition, the disease is not associated with any systemic conditions. There are two examples in the literature of being associated with rheumatoid arthritis, but none with juvenile adiopathic arthritis. And we think this is important because even though technically it's a non-inflammatory disease, even in these non-inflammatory variants they've shown in the last few years, they've shown that there is signs of inflammation at subclinical level. So we think that this might be what's kind of driving the progression of the disease and maybe the reason that this patient manifested so early and so rapidly is because the juvenile adiopathic arthritis was really exacerbating the condition. To conclude, I just did want to give some few clinical pearls in terms of differentiating this disease from some other similar diseases. A couple, a few buzzwords here. Perforative ulcerative keratitis is usually associated with conditions like rheumatoid arthritis, although it can be found with infectious causes and trauma. The big difference here is the epithelial defect. We don't see an epithelial defect in Terian's Marshall degeneration. We do see it with a preferred ulcerative keratitis. If we look at more, excuse me, Moran's ulcer, this is also, I guess some people would say even a type of puK. This is different first because there's usually significant pain. And then in addition to the epithelial defect, there's also, even though it actually does show some peripheral gutter, on clinical exam, there's actually an overhanging edge with Moran's ulcer, which really, I guess, differentiates it pretty easily from Terian's. Finally, furrow degeneration is really a benign condition compared to Terian's. It's asymptomatic and doesn't have the vascularization or the ectasia. Keraconis and polluted margel degeneration are both similar diseases, but can be easily differentiated because of a lack of a gutter. And the ectasia in these examples is usually in the center or in the inferior margin of the cornea. So I like to thank Dr. Mosafar and Dr. Evans for their help throughout this last week and a half, and then also Dr. Vigidani for his, never-ending patients as we emailed back and forth and tried to get the specifics of the case. Any questions? And there was no systemic disease at all, it was just the, it was interesting, this patient still seemed to progress even being on methotrexate, which I thought would hopefully slow it down a little bit, but yeah, topical stories wouldn't be an ideal long-term solution. It's interesting though. It's pretty rare. I just had this question. Yeah. So any other questions? Okay. Yeah, and I felt the mention, they did do an inter-cameral injection. This is a tricky, this is interesting. And they did do an inter-cameral injection of air. It worked for a little while. And then Dr. Roshnara talked about that maybe because there's a thinning in the periphery, it's then creating some sort of force that's kind of puffing it down. There's got to be some vector force going on. There are. All right, so our next presentation is by Chad Jackson. He's a fourth year student at the University of Kentucky. He grew up here in Salt Lake. Made the unfortunate decision to go to the school down south. And as well as our next presenter, actually, this is like all BYU grand rounds. This is a great week for that. So he's going to be presenting bird shot or not.