 I had distributed a chapter for you guys to read. It contains a tremendous amount of information about pediatric UVIs. I think it's probably the most comprehensive thing on pediatric UVIs that's out there. And it's, you know, recently published in Dr. Hartman's book. Okay? So pediatric UVIs is not UVI's light, okay? It has inherent challenges to this population, both diagnostic management and therapeutic. So from your reading, what are some of these diagnostic challenges, you know, that we face in patients with UVIs? Kind of with pediatric UVIs and kids? That's not hard to do that. Okay? Anything else? They won't tell you there are symptoms. Right. They really don't have symptoms. They may not have symptoms. Exactly. Anything else? The classic treatment I know of these like steroids have a lot of side effects. Right. Okay. What about their diagnosis? What about the representation of diseases? So here's the first one, okay? Kids review systems complete exams hard to obtain. They may not tell you. It's difficult to get that from the kid, from the parents. The differential diagnosis in children varies with age, right? So you have an overrepresentation of infectious diseases. There are pediatric-specific masquerades. Can you name one? Last one. Okay. Unique endogenous syndromes. Can you name one? It has juvenile A. G.I.A. G.I.A. Yeah, okay. And how about atypical presentations of familiar disease editing adults? Soracoid. Exactly. Right. Okay. Then the choice and interpretation of a larger test may vary, right? So what test is classally elevated? Ace. Ace, right. So management challenges. So the disease itself, right, is hard to manage. And why is that? I think like what Abigail said. Exactly. It's insidious, bilateral, it's asymptomatic. Frequent development of complications. Okay. Presentations with established pathology we know is a risk factor for development of more pathology. And probably is, you know, graphic testimony to screening failure. And then of course there's the unique risk of amputation. Right. So cataract is a big deal in children. Okay. What about the therapeutic challenges? You know, what do we use? What's the first line of treatment in UBI? Steroids. Steroids. What about systemic steroids in children? Yeah. Right. So you want to limit systemic steroids in children. Why is that? Ex-growth retardation. Occur complications. What else? What about systemic steroids? Why would you want to limit systemic steroids? Growth retardation. Growth retardation. Yeah. Sorry, I have hearing retardation. Okay. So we have the, you know, corticosteroid systemic and ocular side effects. And then there's therapeutic timidity with respect to using, you know, non-steroid immunomodulation in kids by many practitioners. And what else about kids if you take them to the operating room? What's the, is there something special about surgery in children? What's that? Anesthesia risk. Anesthesia risk? They're capsules hard to tear. What about inflammatory? They're more inflammatory. Yeah. So they have, you know, exuberant inflammatory responses, right? And then there are inherent complications associated with certain diseases like JIA. Okay. Epidemiology. I don't expect you to know numbers. You know, just put this up there. It's in the chapter and all that kind of stuff. But generally speaking, pediatric UBI's is more or less common than adult UBI's. Less common. Less common. How about fourfold? Less common in terms of the incidence and the prevalence. But the thing is that it occurs in young kids. It's chronic. It's insidious. It's a long time. And it may actually, you know, represent significant morbidity for children, right? So it represents about, you know, between, I mean, that's a large range, right? It depends on where you're sampling. Okay. What about the anatomic distribution of UBI's? Okay. So in a tertiary care setting, what's the, what do you think are the two most common anatomic distributions? What's that? So anterior and intermediate. Okay. Not that it's anterior and intermediate. And then posterior and pan-UBI's. So we, you know, there is range associated with that. How about in, you know, if you're in general practice, what are you going to see mostly, right? Anterior. Anterior UBI's, right? Obviously, it's influenced by referral bias and geography. What are the most common, what's the most common diagnosis in UBI's? Hyroidis. What's the most common diagnosis? That's an anatomic location, right? Idiopathic. Idiopathic. Right. Hyroidism is already, that's right. And it, or as, as a nomenclature says now, undifferentiated. Okay. So it doesn't sound so bad. And then JAA, we'll be second. Parasplanitis. And then the most common infectious toxoplasmosis. Okay. Structural complications. Name, name some structural complications that you might see in, in a kid that presents with UBI's. PIS. What? PIS. PIS. Pateract. Pateract. CME. How about with chronic, how about on the corny with chronic disease? Bancaratopathy. What can blind children? Pateract. Pateract. I mean blind. You can fix a pateract, right? But you can't fix it up in urine, right? Okay. So glaucoma is a big problem, right? Hypotenine, macular scar. So how do you think that these structural complications are related to visual outcome? So directly related to the duration of UBI's. Infectious etiologies are poor prognostic indicators and posterior pan-UBI's. So they are associated with a worse, with more complications and worse visual outcome. Visual impairment is not trivial in children, you know, 15% in five years. You know, visual impairment and severe visual loss varies, but it's significant. The differential diagnosis of UBI's in children. I encourage you to just think in anatomic categories, right? So let's just do that. So non-infectious UBI's, anterior UBI's, what is the most common editing do you think will come to your mind if a kid walks into your planet? J-I-A. J-I-A, right. Okay. Then there's also others, you know, that we'll discuss. Okay. They're all listed there. Okay. Intermediate pan-UBI's, as we discussed earlier, posterior UBI's, including these entities, sarcoid and C-P, they're less common, but they do happen. So infectious UBI's anterior, what is the most common infectious anterior UBI's in adults and in children? J-It's right. Exactly. Okay. But, you know, you can also see, you know, fuchs and post-infectious autoimmune syndrome in children, particularly, and post-vaccination syndrome. Post-UBI's, right, the congenital torches, which we'll discuss in a little bit more detail. And then, of course, other infectious diseases that we will also discuss like partenella and hematodes, those disease, depending upon where you live, Lyme, endothelitis, and TB. And, of course, masquerade syndromes are critically important. Think about and recognize in children, and we can, we'll discuss some of those and just list them, but I think the call you hit on most of them, the most important being RB, what else do kids get? What systemic disease do kids get that sometimes presents in their eye? leukemia. Okay. I think it is useful, actually, also to think about the differential diagnosis at the age of presentation, okay? Because, you know, certain things would be more common at the age. So, infants, what do you think about in infants? What group of diseases would you think about in infants? Torches. Torches. Exactly. What do you think? What do you think? Describe what you see, Marshall. So, you see some hyperprinted changes more in the mid-periphery. They have somewhat of a bone-spick-like pattern. What could this be? It could be like a post-infectious thing like rubella. Right, exactly. See, the child is dead. This is rubella, okay? So, the most common, you know, torches, plexoplasmosis, others. We'll talk about some of the others, okay? Which you probably wouldn't think of a little later. Rubella, CNV, HSV, and congenital syphilis. What about children between 2 and 10 years of age? What would be probably the most common presenting diagnosis here? Yeah, yeah, yeah. White, quiet eye, right? Okay, then, of course, you have all these other infectious and neoplasmic things to think about. Just, you know, you're thinking of, like, the top hits, right? So, I know they're a list, but they're useful, right, to have in your mind, okay? Then, adolescents, is it going to be a lot different than adults? Probably not a lot different than adults. So, talk so, you know, with the usual presentation, Intermediate Panne, you guys. You can see HLAB27 associated disease. You can also see that as a subtype of JIA, which we can talk about. And then, these other infectious and neoplasmic diseases. Okay, so let's start out with a case, okay? This is a long, complicated case of a kid that I met when I first got here, that I saw about six months ago, unfortunately. Anyhow, he's lost a follow-up with many social problems, but an interesting case nonetheless. Remember that we saw this kid in October or August of 2003, but he presented to pediatric rheumatology in 2002 for initial evaluation and was diagnosed with arthritis first, okay, and placed on 10 milligrams of oral methotrexate. Thank you, thanks very much. Okay, so Damien comes in August 25th. He's got an ongoing leg pain. He's placed on an onsteroidal and methotrexate. Visual Q is 2020. He's got some inflammation in both eyes. Okay? A lot uncommon in patients with good vision, right? So, what do we do for this guy? Prep for today, every two hours, encyclopedia. Anything else you might consider doing? So he's on methotrexate, right? 10 milligrams. There's, you know, don't be shy. I don't know right and wrong answer. What might you think about doing systemically? You could go up on methotrexate, right? Absolutely. So remember, he's three years old, right? So there's not a lot of room, too, maybe because it's based on weight, right? So we increased his methotrexate to 15 milligrams, and he's on oral, okay? Because he does want to take shots at the age of three. So his mother is incapable, actually, of giving him shots, which is really the problem. Okay, so you're familiar with the treatment paradigm. We'll kind of review it again. But basically, Syrah had spit out the fire, low threshold for immunomodulation in patients that present with chronic disease, established complications with anti-metabolites, and then moving on to biological therapy, sometimes we might use, in some children, an oral steroid bridge in the short term. Okay? So we increased his methotrexate to 15 milligrams, I'm sorry, and for a year, he does okay, and he has these kind of like minor little flare-ups, you know, that are about 0.5 cells, 0.25 cells on 15 milligrams. Not enough in our judgment to increase his modulation given the fact that he had 20-20 vision. I just want to call your attention to a little bit of a controversial issue, and that is there is evidence out of Hawkins that the risk of catarct development is lower, is pretty much close to zero on chronic topical corticosteroids if it's less than or equal to three times a day. This is a little bit of a dangerous recommendation, okay, in that it is, you, it is intended to be a suppressive dose to keep your child quiet, not to initially treat people with steroids and keep on steroids forever, because we know that those children can develop cataract and certainly ocular hypertension. Okay, so this has nothing to do with ocular hypertension, but for kids, there are some kids that have, you know, less than a 1-plus cell, okay, that really, that require maybe DID steroids in order to keep them quiet, that are on immunomodulation, and that 16% of these patients will require additional medication. Okay, so then about a year later he presents, okay, with another flare-up in his left eye. So this is 1-plus cell NKP, so it's a significant inflammation. He's also got systemic arthritis flare with synovial thickening in his knees. So what are we going to do for him now? Is he still on methotrexate? He's still on methotrexate. 15? 15. Then he's taking it orally. It's just unilateral flare, and I would consider it topical. He's not taking any topical now. He's got unilateral flare, but he's got arthritis too, right? So we're going to treat his topical, his topical inflammation, right? We're going to switch him to subcutaneous methotrexate. Why would we do that? Well, he's a little older now. He can actually give it to him himself. But why is subcutaneous methotrexate better than oral methotrexate? More bioavailable, number one. So number two, it may be a set with fewer side effects, but that may or may not be true because there's a tremendous kind of psychological thing that goes on when injecting things. Just like when people use their blood drawn, the big, burly guy that comes in is a weight lifter that chews up steroids all day long as the guy that faints when he gets his blood drawn. What else do we want to do with him? Anti-TNF. What's that? Anti-TNF. Would you want to advance his therapy at this point in time? And what would you advance his therapy to? Anti-TNF, an individual, maybe? Okay. What would you choose? Okay, so remember, this is in 2007. Okay? The speaker. Oh, it's not... And he's not... So what would you choose? Let's hop on. So we chose... we chose infliximab, okay? And we chose infliximab because he has to go get an infusion, okay? We know that he's going to get it if he goes to the infusion center. I think it's adherence, okay? And plus you have a little bit more flexibility in terms of the dosing, okay? And in terms of... You know, we start at five or 10 milligrams per kilogram and go up to 20. And then we can vary the interval. And then we also pull some of the little oral steroids, okay? To help with his knee and hip pain. I didn't prescribe that for him, but it doesn't matter, okay? To help with his knee pain. So he does okay. Then about a year and a half later, he comes in having been quiet for about a year and a half. The oncology has extended his infliximab interval. He's on methotrexate at a fairly low dose. And no ocular inflammation. So why do we keep patients on infliximab on low doses of methotrexate? Because it can cause lupus. Okay, it's really, really uncommon that lupus is... That lupus is... But that is true that lupus can occur, but it's not so much to prevent the formation of lupus, but what kind of a molecule is an infliximab? It's a chimeric molecule, right? So what other purpose would methotrexate serve? To prevent the formation of lupus? Exactly, which might diminish the activity of lupus. Unless it also serves, as we will see in the sycamore trial, as synergy in terms of, you know, efficacy for the drug. So he was switched to Adelumina for convenience, okay? He thought he was doing pretty well. You know, we can give it to him subcutaneously at home. And he was on a short course or put in his own, and he was kept on methotrexate. So Adelumina methotrexate. In November, he... About a year later, he comes in with continued joint involvement, methotrexate and Adelumina met, and he's got a significant flower in his left eye. So what are we going to do now? Well, there were a couple of other, you know, third-line options, not many, available to us at that point in time. One was very hot. One was a kind of a... There were some publications that seemed to show the efficacy. We started Durazol, which I rarely do actually in kids because of the hypertensive effects of Durazol. But he needed, you know, some immediate calling of inflammation to enter your chamber. And we just continued to humora about a sept, okay? Well, now go back to what had been working. Good question. Well, you'll see. But we tried something new because we thought that maybe about a sept might be good for his joints because he had continued joint involvement here and there were reports that show that this was good for both the eye and the joints, okay? So we started on Arensia, which is a, you know, fusion protein, okay? Which has a very interesting mechanism of action, which you should look up. It's kind of like works on the checkpoint of their organs. Okay? So we started him on that. Then he came in in March, low-grade inflammation, persistent arthritis. He was declared about a sept failure. So the literature, subsequent literature on about a sept was not so compelling, you know? I mean, no randomized controlled clinical trials. So we was declared an about a sept failure plan. Go back to what was right. That's exactly what we did, okay? So we switched it back to an influx map every four weeks, loaded them up with higher dose of methotrexate, and topical prednisone with a taper, okay? This seemed to do the trick, okay? So he was quiescent, UVI is an arthritis. He slowly extended his influx map to every six weeks and reduces methotrexate dose. So the idea is if he's on influx map and he's quiet, his joints are good, his eyes are fine, to lower the dose of methotrexate, you can go down to 7.5 milligrams of methotrexate to maintain protection against anticonormy antibody, okay? So at his last exam, this is not quite his last exam, but this is in 2016, he actually had been doing pretty well. This... There are many social issues with this kidney and he showed up on fire again about six months ago. All right. A little didactic stuff about JIA, okay? All right. JIA, okay. JRA, JCA, what is all this, right? It depends on the classification system that you use to classify it. We use the ILAR, the International Leading Against Rheumatism for the JIA classification rather than the ACR. It is the most common form of childhood arthritis, okay? And it's the most common systemic association in the U.S., you all know that, right? Okay. What are the risks for the development of uveitis in JIA? This is a board question. Okay. What are the risks? Okay, let's just talk about JIA. What type of JIA represents the greatest risk? Very young, anti-positive and positive. I just heard it's a mumbly mental. I'm sorry. What type of... What's some type of JIA? The oligoarticular, okay? What is oligoarticular JIA? Anybody? Four fewer joints. Exactly. What's extended anyway for the rheumatologists in the room? Okay. They start out at four or fewer, right? And then they have more than four. Yes. So the JIA subtype is very important. Oligoarticular is associated with, you know, 10 to 30% risk. These are the other subtypes of JIA within the ILAR. Okay? There's rheumatoid factor. Negative polyarthritis, positive polyarthritis, psoriatic arthritis, the encephalitis associated with related disease, which behaves like HLAB-27 associated disease with acute identifying type. So oligoarticular is number one, but these others are also entities to consider and can be very difficult to treat, particularly psoriatic arthritis. What other factors, okay? JIA subtype, so what else? What tests do we always get? A and A. Okay, positivity is a risk factor, right? All right. What else? Young age. Young age at onset. What else? Duration. Duration. Okay. It depends on what you mean by duration. Like the duration of the disease. Or I guess that's a follow-up, isn't that? Yeah, what about sex? Okay, female sex. Younger age of onset. I think what maybe you mean by duration, but younger age of onset. So average age of onset is about four years of age. And so younger age of onset, female sex. Okay, so those are the risk factors. Those are boring questions. And there is HLA as a genetic association, just so for born type purposes, okay? Clinical features, I think you all probably know these. I'm going to blow through this. At presentation, they're not going to tell you that they have arthritis, iridis. That's why we screen them, okay? So asymptomatic, bilateral, non-grinding, it's actually uveitis. Sometimes presenting with complications. Peak onset is between six months and four years of age. So most of them follow the onset of arthritis. So most of them come in. They're referred by rheumatology. Can you please screen this patient for iridis? But about 10% of them, you may perceive the onset of arthritis. These are the kids that are really in trouble, okay? So they enter the healthcare system late. These are the kids that come in, you know, with complications. And so you're chasing, you're behind the eight ball already. Okay? The thing that is important to note is that the systemic and augurant inflammatory components are not synchronous with one another. And that's important to tell parents as well. You know, I'm treating your kid with ques and joint disease with, we're increasing their immunomodulation because their iridis is bad. But there's a sense, okay? So they can be independent of one another. Okay? Predictors of a poor visual prognosis. This seems like common sense, okay? But in the site study, which was a large retrospective study of JIA, you know, predictors of poor visual outcome were, what do you think would be number one? I mean, predictor of poor visual outcome. Okay? Inflammation. Active inflammation, right? So they come in with one plus cell for a year. All right? The presence of posterior snigia, I think was mentioned. And then in the site study, at least prior intraocular surgery, all of which I believe that prior intraocular surgery would represent, you know, would be a proxy marker for disease severity, right? Okay? On-site of UVS before we mentioned this, before the diagnosis of arthritis, 10%, right? A shorter interval from the onset of arthritis to UVS, and at least once they met males did worse than females. There are screening recommendations that, you know, you should be familiar with, and it's based on ANA positivity and the type of arthritis that you have. So oligoarticular disease that's ANA positive is screened every three months, up until the age of, you know, six years, and then it's extended. But we see these patients frequently anyway because they're on immunomodulation, okay? And maybe these screening guidelines are not stringent, you know? Ocular complications. We've kind of met through this band, posterior synchia, cataract, ocular hypertension, ocular edema, ERM, and hypotamine. So you don't want to see an eye like this, you know, first day. This is not good. So 16% of eyes present with an ocular, one ocular complication, and there's a, you know, an incidence of new ocular complication is about 15% per year. It's pretty high. Okay? I think that you guys are quite familiar with this, and I'm going to kind of, for the sake of time, and interested in terms of doing more kind of case stuff. But a therapeutic approach, we want to eliminate active inflammation, right? And we do this by whatever means possible, as Malcolm X would say. And we use a step-ladder approach using topical steroids. Oral non-seronauts are used, not infrequently, in kids with JIA, okay, for their arthritis. And there are some people, at least my mentor seemed to believe that oral non-steroidal inflammation was actually useful as a steroid-spirited strategy. So I think research had shown it's kind of a wash, a third, a third, a third, you know, something better, something the same, something worse. Periocular corticulitis, I mentioned using diaphragm-printed, I use it, but I use it with caution, because it will hasten development of cataract and is associated with elevation and drug and pressure. Similarly, periocular and intravitural steroids, I use a little bit, I'm a little bit more cautious in children, because they seem to have a more robust, hypertensive response, okay? And it is a bit of a problem, you know, when you take into the OR, and I give them an injection, and if they have persistently elevated pressure, you know, then you may or may not be buying that surgery, which you would want to avoid doing it in a kit. Brief steroids for bridging therapy and that have a low threshold for steroid-spirited immunomodulation, okay? So chronic inflammation, right? Presentation of established pathology would be an indication for that. We've gone through this in our first lecture, you know, broad categories, the anti-metabolites, T-cell transduction inhibitors, calcineurin inhibitors and outglading agents, which are used very infrequently in children. And then methotrexate is the first-line agent, okay, in kids. I just want to just call your attention to the fact that there was some very interesting work out of the Netherlands that had to do with the chance of remission in patients with on-methotrexate. So a not unreasonable question for parents is how long is my kidney going to be on this medicine, right? Okay, so it's hard to tell some of them are on this medicine for a long period of time, but a group from Netherlands showed that longer periods of disease quiescence, okay, longer time on-methotrexate minimum of three years and older age at the time of withdrawal were associated with greater remission and less recurrence of disease. That's all I want to show about that. So with non-responders, we have alternative anti-metabolites, you know, including mycophenylate and aziphybrin. Sometimes a child may respond to mycophenylate, but not aziphybrin. Cyclosporin actually can be useful in children and kids do tolerate greater doses of cyclosporin than adults and develop less hypertensive effects. And then, of course, there are the biological response modifiers, all of which are listed here. The most common ones that we use are fliximab and adelumab, but there are others that have been shown to be good third-line agents, such as ptocellizumab and rituximab, and in certain cases, you know, for really weird diseases, you know, like muckel-wells, IL-1 inhibitors like datacentron. Okay. I'm just going to skip through this. You know that adelumab is approved for uveitis in adults, right, for intermediate-pushed and pan-uveitis, but not anterior uveitis in adults. Okay. It's not approved for many classes of arthritis in children, but not for anterior uveitis, believe it or not. Okay. But we use it as such. Okay. There was an important trial called the Sycamore trial in a randomized controlled clinical trial, a multi-syndrome study, which looked at adelumab plus methotrexate versus methotrexate alone. Okay. And this showed that there was a significant delayed time treatment failure with a combination rather than methotrexate alone. Okay. And that there were significant differences in the time you treat response with a combination, which supports the common practice of using both of these drugs together. Okay. There were more side effects associated with the combination, but they seemed to be tolerated. The trial was stopped early because of efficacy. Okay. So we've talked about some of the third-line agents that are available to us about a set, not so much, Rituximab, you know, has been used actually successfully as a third-line agent. You can also change the, you can change the anti-TNF medication. So some children have responded to goalimab and simsia when they have not responded to hemera and influximab. What about the outcomes? Well, I think there is really good evidence in the literature, not prospective evidence, but site evidence from large retrospective cohorts and single-center studies of JAA from Hopkins that show that there is a significant improvement in visual acuity and reduction in structural complications with the use of immunomodulation. So I think that it is a treatment paradigm. I think there's literature to support its use. Okay. JAA, probably the most important topic that we will discuss in this lecture, but really cool stuff is coming in. Okay. So I mean, interesting cases. So here's a case of a four-year-old boy with a red eye for two months. Parents didn't observe any changes in his vision. Okay. Unremarkable review of systems, chosen with porous planitis, normal development, 2030 vision, 2020 in the other eye, otherwise unremarkable. He did have inflammation in front of his eye and he had a massive inferior angle. And that's what it looked like. Okay. So pretty much a unilateral presentation anteriorly. Do you appreciate that mass in the angle down there? And what else do we see? What's your theory? And on B-scan, this is UBM. If you want to describe the angle there. Okay. This is what the back of the eye looked like. Someone might want to quickly describe that. The case of you, there are multiple round type of pigments of lesions that are looked at each other around them, maybe a chloride. The nerve looks a bit pale, but it might just be from exposure and the haze and vitreous. Left eye, similar findings, right? So the left eye didn't look so involved because you're always looking at the other eye, right? So this is a bilateral process. Fluorescene, angiogram, early and late. No big mysteries, right? Okay, a little blockage of some of those lesions and staining. Your observation of the nerve, I think is correct. Left eye, late. Similar findings of staining of this kind of coral lesion, right? Ultrasound, crudal thickening. What else in the corae? Or the super crudal space? A little lucent line there. Hypoechoic. It has a little fluid in the super crudal space. OCT, right eye. See any problem here? Or any abnormality? Subrational fluid. Left eye looks pretty good. Okay, so, you know, pull out the stops. Kids make you nervous and put pretty much kids to sink on him, okay? The interesting, I think, in his exam was his white count, which was a little bit low. Path report, what? Well, Dr. Ramaswami Baurian, actually who was here, in pediatrics at the time, biopsied that. And that takes some guts to do that. Exactly. Because, of course, you are worried about rhinoblastoma, right? You don't want to biopsied that, okay? But the path report really showed an inflammatory process, right? Inflammation. Not granulomas, but... And the stains were negative for bacterioma. Benign inflammatory process. That's kind of like the go-to for pathology, you know? Benign inflammatory process. It doesn't really... It helps us know that it's not an infectious process, okay? So, we've got panubiatis, multifocal cord lesions, right eye grade and left eye. What are we thinking about in this type of this kid? And a path report that shows inflammation, probably a recent onset, just, okay? Malignal disease on the lips, for sure. Okay? Okay, multifocal cord lesions, for sure. All right, so you're thinking along the right lines. So, differential, early onset, sort of like, okay? Familiar with systemic juvenile granulomatosis, we'll talk about that a little bit. Ultrally known as Blaugab's syndrome. People want to name it. Epstein bar virus is weird. I've seen, in case of multifocal cord lesions, I've seen bar virus, but it was negative. And common variable, immunodeficiency syndrome can look very similar to multifocal cord lesions and sarcoidosis. So, plan. What are we going to do for this guy? So, I think that the chances of an neoplastic process are low. His herpes, human herpes, simple as six, but herpes is positive. That usually produces a necrotizing type of retinitis in immunocompromised patients. But nevertheless, it was positive and you can see this in children. I don't think this is necrotizing. Everything is negative on the prior work if you could do oral steroids. So, that's how we felt. But we also, since we're paranoid, covered it. Why not? We started him on prednisone. Endurozole, because the inflammation, we were going to see him back quickly in atropine. Okay, over two months, he did beautifully. Resolution of the iris mass, flattening the cord lesions, tapering and valve tracts, that's what his fund is looking like. And for about two weeks, or three weeks. Okay, so we're on fluid resolve, vision 2020. All prednisone for a month, he had recurrence of AC cell in his right eye. And a new red popular rash on his cheeks, his arms and his legs. Unfortunately, this was not biopsied. And the thought was that the recurrence of the rash was supportive diagnosis for childhood circuitosis. Okay, so, plan here. What are we going to do now? Recurrence inflammation. He's got badness in the front and the back of his eye. So we did. We started with an IMT with a steroid bridge. Okay, he was completely quiet with 2020, two months later, his rash resolved. Plant that time. So, continued and tapered. So, we did do some genetic testing on this child. We'll talk about this a little bit later, but he was negative for a nod mutation. He did not hide any family history of uveitis. Kind of working diagnosis was juvenile circuitosis. So, is it odd that the biopsy wasn't gradient limitous? So that's a very good question, I think. And I gave a lot of thought to that. I don't think it was odd that it wasn't, because it, you know, is in the iris, okay? It was probably very, very new, probably fresh. And probably not enough time to form it. That's my thinking. Also, could it just be cells that had been like, mobile in the vitreous or in the into chamber that just kind of settled and collected but weren't organizing? I guess it could be, but it's a little unusual for it to be so focal. You know what I'm saying? You would expect more of a hypopion type of more diffuse cellular So, circuitosis, you know, is always cited, but it's really uncommon in kids. There are two types in kids. There's early onset and there's late onset. And there are differences between them. Early onset usually is without pulmonary involvement. Late onset is more like that with adults. And the current thinking is that there are probably two types of early onset. It's a sporadic variety, which I think we're sure our kid had. And the other one is associated with a card-15 or non-2 mutation, which is something that is seen in a familial juvenile rhinolabotosis syndrome. Yes? As well as sarcoidosis? Yeah, so there was a thinking that maybe juvenile sarcoidosis is a type of blouse and rums, but it's sporadic. You know, it doesn't occur autonomously in families as blouse does. But there are kids with juvenile sarcoid that have a non-mutation. Okay? I can't remember exactly the statistic, but I think it's over 50% of them. So, we know that it's a granulomatous inflammation. It can cause, you know, most common presentations really anterior umiatis, but it can also cause an intramural, it can do whatever a demo wants to as we know before, right? It can occur in any segment of the eye. Lack of a gland involvement in children is less confident in adults. Uh, chest x-ray may not be so helpful, you know, in a young child, but it would be in an older child, you know, for pulmonary involvement. And you'd want to try to biopsy, you know, not pulmonary sites, skin. So, it was unfortunate that we did biopsy that were unable to diagnose it until it's skin. The differential diagnosis, JIA, obviously, right? Because sargotosis can produce arthritis, okay? And it can be differentiated because it's non-granulomous. It's a ligar-articular and ANA-positive, right? And um, then there are others. Okay? Differences between JIA and sarcoid, non-granulomous versus granulomous, anterior, chiefly involvement in JIA versus anywhere in the eye in sarcoidosis. A ligar-articular involvement as opposed to polyarticular involvement. And no pulmonary involvement in JIA, whereas you can have pulmonary involvement in older children and in some young children with sarcoids. So, when you hear Hopi to think of, right, zebras, of course, and I think it's already been mentioned. Uh, someone wanted to tell me what they're looking at here. It's a triad that comprises a syndrome that's been mentioned here. Okay. So, you got it. So, triad granulomous dermatitis, um, polyarthritis, there's a specific deformity of the hands. Do you know what that's called? Okay, wait for a million dollars. It's called campyloidactyly. It's a deflection deformity of the PIP joints that is characteristic of blouse syndrome. So, anterior uveitis and posterior uveitis as well. Okay. So, java, blouse, femo, granulomotosis, triad that we mentioned. Uh, it is linked to chromosome 16 with 100 phenylsatific, you know, correspondence with a non-tune mutation. It can produce a pan-uveitis multifocal corditis to usually children about four years of age from the development of complications. It's not uncommon. It can also produce some unusual complications such as vasculopathy and it needs to be distinguished from early onset in sarcoen and GIA. Okay. Here's another case. Moving, switching gears. Ten-year-old kid referred with non-granulomous bilateral simultaneous non-granulomous uveitis with papillitis that began after illness with fever, vomiting, sore throat, upper nerve swelling, and AC information. No past medical history. They were actually living in China. Their parents were ambassadors living all over the world. They've been worked up in Singapore and the workup was negative. Look at that workup. There's something missing from that workup. It's actually important. He had elevated inflammatory markers and he was a little bit anemic. He had recurrent episodes of fever diarrhea and lost seven pounds. We worked him up. His vision was 2040 and 2025. He had inflammation in both of his eyes and some cellulose vitreous and some papillitis. You can see here. But no macular edema. Plant. What lab would you order on this kid? Syphilis labs. So we did order syphilis. We also ordered all of these labs again. We didn't want to bring him back from... Initial treatment. We started him on durazol and atropine. And we ordered beta 2 microglock which was extremely all of it. Okay? So, TINU is the diagnosis of this child. And how do we know this was the diagnosis? Well, it's cranny was okay. But we sent him to Raul Nelson who was a little bit alarmed by what he saw and biopsied him. Okay? And so his renal biopsy actually proved this diagnosis with predominance of sites and plasma cells and focal necrosis of his renal tubules. So TINU, all right? Bilateral non-granulomus recurrent anti-univitis associated with interstitial nephritis. It could happen before, during, or after an episode of nephritis. So it's not again not concurrent. The age of onset is about 15. But think about it in like a 30 year old woman particularly there's a female predominance that presents with it. So there's a bimodal distribution to this. And there is a genetic position to developing and we think, you know, it's not it may be by some drug or micro. The definitive diagnosis is renal biops, but most often the diagnosis and we, children are not subjected to renal biops because not infrequently the nephritis will have subsided and the patient will have nephritis. So there are diagnostic criteria that have been set up by a guy named Mandeville and 32 microglog, which is excreted in the parts of the tunule is a very good screening test for this disease. Okay? Differential listed here you know, Lucas-Sorquo, GPA, Sjogren's, Puszt, Dr. Cackel syndrome. Treatment. Well, you know, when this was first described, one who got the impression that it was kind of a benign disease that, you know, oh it goes where the top will seroids, but the top is about 9% and in our clinic more than that require, you know, more significant inflammation. Okay? Anti-inflammatory medication with IMT. Okay, so with our patient he was treated with oral prednisone and cell sept. The beta-2 microglobulin decreased significantly with treatment and he did better. Okay, so you're called for a consult over primary children's hospital and this is what you see. So I'm going to describe these findings. There's a discriminating rash on the fingers and groin and the piquest membranes in the mouth as well. And if you ask them to stick out his tongue what might you see? And so what are you thinking? Okay, otherwise known as eucutaneous lymph nodes. Exactly. Okay? So more common in Asian and South Pacific Islanders uncommon in his Asian systemic vasculitis. Okay? So it's a real disease, you know. Medium-sized vessels. There are systemic manifestations. What is the systemic manifestation that you really need to worry about with this disease? Exactly. Coronary arteritis, vasculitis, exactly. Also meningioencephalitis. Okay? What are the odds? Oh, I guess you're right. Like the conjunctivitis of the perilibal sparing? Yes, that's exactly right. So there you go. Okay? You anticipated my though, ocular manifestations. What's the most common ocular manifestation, Marshall? Conjunctivized with? Perilibal. 100%. What else can you see? Can't hear you, the audience. Objective swallowing, darlin', beans, amurosis. What's the treatment? I really expect you to know this, but you wouldn't be initiating treatment and neither would I, really. So IVIG, really? The first 10 days. Have you been seeing similar ocular findings with this multi-system? The ocular findings to be treated with topical. My question about it was that was the perilibal sparing really specific? It is pretty specific for it. So just a word about bilateral, simultaneous, non-granulose anterior ugeitis. It's pretty uncommon, but it should make you think of a couple of things. Okay? So it usually has limited duration and usually there are recurrences. The etiology can, we already have, we already solved one of them. What other things might cause this? Drugs. And post-infectious phenomena. Particularly streptococ, you know, strep. Okay? So it's really a new Kawasaki disease. HLAB-27 rarely causes bilateral disease, but can, so, you know, test for it. Valuation. We want to get serum ASO-tiders, B-27 and urinary, and why is post-streptococcal ugeitis syndrome important to diagnose or to even think about? Okay, so it can cause bilateral anterior ugeitis, right? It can cause renal disease, cardiac disease. So it's really a serious problem, right? It requires a multidisciplinary approach. It occurs, you know, 50% of them under the age of 15 and post-revolvency and over a third of patients. Okay, another case. Are we doing okay on time? Are we good? Still engaged? Okay. Still mowing down? Yes. Here's an eight-year-old, this is actually a bit interesting case. Eight-year-old white boy, okay, referred by an outside retinophysician for one in history of decreased vision in his right eye. Okay? He was diagnosed with panneoviditis, as many patients are, you know, they present, they come in with that diagnosis and macchiordema in his right eye. He did not put 14 on that. Negative review assistance. However, his past medical history was significant for a specific syndrome called periodic fevers at the stomatitis pharyngeitis, cervical adenitis, which is characterized by recurrent fevers or ulcers, and it was controlled according to the series. So this is something in his past medical history. His family history was significant for a brother with ulcerative colitis, a father with MS. He had poor, you know, moderately reduced vision in his right eye, but his left eye looked pretty good. And on his examination, he had vitritiditis, vitreous haze, hypoallergenic optic disc, and macchiordema. I don't know if that projects that well, but it's on there. Referrarily, he had snowbacking, inferior prism plana with overlying collections of inflammatory material in his right eye and in his left eye, so it was really symmetric and no neovascularization that we could see. So labs, we kind of got the usual labs and they were all negative. Diagnosis. Does he have pan-uveitis? So what anatomic category of uveitis is this? Intermediate uveitis. And it is bilateral, right, asymmetric. So intermediate uveitis with macchiordema, possibly associated with PFA, we treated it with predforte with resolution of his macchiordema and his sub-tenants kennelung injection. And then nine months later he presents with vitreous hemorrhage in his right eye. Left eye showed neovascularization. Unfortunately, this is before really great wide view here. So, what are we going to do now for this guy? He had vitreous hemorrhage in his right eye, young kid, and neovascularization is left eye. Well, that's one possibility. Retract to me. Retract to me with what about his other eye? He's got a neon venerate. You could give him a bad job, but while you're in the OR you'd also face the wrong. So that's what we did. And this is an interesting case for a couple of reasons. So we had to retract him with endolacerin in his right eye and peripheral laser in his left eye. He did great for two years off of all medications. Absolutely no inflammation at all. And he represents with a mild flare in his left eye and reduced vision in his left eye. Two-plus cells in AC and vitreous cell in A's. We wanted to treat him with, since he had no disease in his right eye and disease in his left eye with an intravitral therapy, he really didn't want to have anything to do with it. He didn't want, he didn't like his previous SDK. So he treated it with an oral adrosive steroid. The thing that's interesting is that he's got intravenous hemiitis had undergone a protractomy in his right eye. His vitreous was removed and that eye has remained completely quiet since then. His left eye, which did not undergo a protractomy, has had few, but a couple of flares over the years. So there may be something important to therapy to protractomy. So PFAPA is the most common auto inflammatory fever disorder. Childhood usually before the age of five. Episodes of fever with one of the three atlas stompetitis, hemorrhagitis, cervical adenitis, every three weeks. It is thought to be a defect of the adaptive immune system mediated by inflammatory zones that recruit T-cells to the eye. Usually results spontaneously by the second decade. So we reported this as the first case of intravenous hemiitis associated with this syndrome. And there have been other reports since then. So pediatric intermediate EVIs, the second most common non-infectious EVIs that we see epidemiology just a couple of factoids. It's fairly common first to fourth decades. Most of the time it's bilateral, but not infrequently asymmetric. The third presenting unilaterally. There's no inheritance pattern, but there seems to be a little clustering. Clinical features as opposed to adults with anterior segment with intermediate EVIs, there seems to be a more exuberant anterior segment component in children with intermediate EVIs. At least that's been my experience. The other thing is that non-infrequently children will present with lots of inflammation right retrolytic or behind their lens which I think is probably related to their peripheral through inflammation and peripheral retina. So you see these kind of big swaths of inflammatory material behind their lens. In fact, they're referred sometimes to other have a cataract when in fact they have inflammatory material behind them. The tritus as you know is a cynic one on which are many EVIs with collections of inflammatory debris, vitreous opacities. Here you can see that's a good slit line photograph of 2 or 3 plus cell plus snowballs. Retinal vascularitis is pretty common. Vastals are mostly affected. Arteritis remains. Phlebitis or Arteritis. So it's actually an important distinction. It's mostly Phlebitis. Arteritis you'd be thinking about other diseases like ARN. So you can see vascular occlusion and peripheral ischemia or as we'll see in the next slide a ferning pattern. Neovascularization I can occur in the peripheral retina and the optic nerve but it's important to know if that neovascularization is due to ischemia or through inflammation because the treatment would be different. Interestingly about a quarter of children can actually present with vitreous hemorrhage whereas about 3% of adults over where 6% of adults might present with vitreous hemorrhage. So that's an important presenting sign to be thinking about. Immaculodema is a common cause of decreased vision. I just show this montage on the left hand side you see this kind of peripheral leakage that is classic called a ferning pattern that you might see in patients with intravenous vasculitis but you can also have peripheral non-profusion. So I think that the disease is one of the vasculitis and the peripheral retina mostly and the peripheral non-profusion can give rise to ischemia and neovascularization. Patients I have had several patients that have presented for example with neovascularization of the disc and after doing an angiogram in the vision there are no non-profusion defects and those patients seem to respond extremely well to anti-inflammatory therapy without laser. So you can avoid the destructive effects of laser or even an anti-vegetary by doing an angiogram seeing if there's ischemia. So differential diagnosis of pediatric intermediate EVIS is similar to that of adults and that's just a little one more common line cascrunch, toxo, sort of like etc. So you rule out an infectious disease and if it's there is no systemic or infectious association it is called and there is a snowman parisplanitis. So parisplanitis is the idiopathic variant of intermediate EVIS with complications. I think we don't really need to belabor this. The asterisks have to do with a study that we performed here where cataract was actually quite common. Acura edema acura hypertension was common in our group, band carotopathy. Retinal detachment can occur either due to tractional retinal detachment in the periphery or due to retinoschesis which is seen not uncommonly in patients with intermediate EVIS it's reported between 15-20% of patients and it's thought to be either due to ischemia or to peripheral traction and usually it does not progress usually. What can dyscadema and evalcerations we mentioned in vitreous hemorrhage presenting sign 28% versus 6% and of course amblyopia. Treatment and prognosis similar as in JAA you want to treat early and aggressively in patients that present younger who are at risk for amblyopia they already have structural complications extensive vasculitis irrespective of visual acuity and significant vitreous cell and then a modified step level and I put this up there only because it's an open question you use topical and periocular steroids judiciously and then systemic corticosteroids but one underutilized modality that's in the literature is cryopaxine indirect laser it was initially described for patients with peripheral neovascularization there have been some other states at least adults and non-children that show that renal cryotherapy reduces or increases the risk of remission by fourfold in patients undergoing cryotherapy and then whether or not you treat patients with immunological therapy versus vitrectomy is an open question in terms of what's the next approach and there are pros and cons which we could discuss for a long long time and I think that there are certainly situations in which you would want to do vitrectomy for example a patient has a structural complication or vitreous hemorrhage I'm not so sure that vitrectomy but before immunomodulation is better than immunomodulation than vitrectomy that's an open question however we did look at immunomodulation in the treatment of pediatric QVIs in our group of patients and we found that it was pretty well tolerated very well tolerated using combination of both anti-potatoes and biological agents and among the 30, 20 of the 39 patients 19 of the 20 were able to be tapered off with steroids completely so that's I think huge for pediatric QVIs and 75% of these were inactive at their last follow up alternatively Steve Foster in his group I actually looked at vitrectomy for patients with refractory intermediate QVIs again this is a small group of eyes uncontrolled series which showed 96% of inflammatory control unspecified follow up you know in patients that had poor inflammatory control and that 5 6 of these patients had persistent vascularized which we know is difficult to treat so vitrectomy may contribute to the control of recalibration of information in intermediate QVIs in that subgroup just for your information and it may come up in another model vitrectomy has also been shown at least in the site study to increase the rate of a disease free remission among patients with intermediate QVIs by I think 2.5 so it's an open area for study but it's a hard one to control for all right congenital infections we kind of touch on this do we want to list them real quickly someone? Torches, Toxo, others there are some others here Rubella, Sada Magalovirus, herpes Toxo is the most important one as a congenital infection I think for board purposes you need to know that the is directly associated and this is very inversely associated with gestational age clear? and that 40% of primary maternal infections result in congenital disease and that 70% of the manifests with the ocular lesions and a third of them are in the ocular unfortunately so the classic triad of congenital toxoplasmosis is quite a retinitis hydrocephalus in intracranial calcifications unfortunately child this is like 40% primary maternal infections result in congenital disease is that like for moms that have not ever gotten toxoplasmosis before it's the first time they've gotten toxoplasmosis if you're pregnant and you get toxoplasmosis 40% chance of vertical transmission so if you want to have some protection it doesn't like have exposure to it so they have some protection to it active talk to them so that's why they advise women to stay away from litter boxes but there's kind of a paradigm shift thinking of congenital toxoplasmosis and that there's pretty good evidence that about two thirds of them may represent postnatal infection so that prevention strategies should be targeted for women to prevent them from becoming infected but also for children from eating litter the clinical course the problem is that there can be a subclinical infection with posterior involvement in a very high percentage of patients 85% of patients develop quarter of a minus after 3.7 years 7% of the line in one or both lines so the diagnosis is made clinically but serology is important in the diagnosis of congenital toxoplasmosis in the presence of IGN or IGA in congenital infection and then of course in part is the adult IGG as you know crosses the center treatment indications for congenital toxoplasmosis which you recommend for a child that is diagnosed with toxoplasmosis so it's important to know that any child that is diagnosed with congenital toxoplasmosis receives chronic antiparasite therapy usually with triple therapy for a year because of the fact that whether or not they have lesions or not treatment the indications for treatment in older children are similar to that in adults we know what those are lesions in the magda optic nerve and then of course immunocompromise which requires maintenance and prophylaxis the treatment regimens I'm not going to go through we will go through this I think next week there's the classical triple therapy with cortic seroids usually at a reduced dose and then there are alternatives such as the addition of plinthomycin is also used for patients with peripheral lesions and then intervitual therapy with dexamethasone particularly for patients with foveal lesions and pregnant women there is another I don't know board type of thing if another board thing that is totally useless that sometimes comes up on questions is pyromycin is a macrolide antibiotic that has been used effectively and safely in pregnant women who have toxoplasmosis is not available in the United States or others in the torch group that you may or may not be aware of okay can you think of others that we haven't mentioned that might be similar to toxoplasmosis for example you may not know about this this is what it looks like looks like a toxolation and if you know who this guy is anyway anybody know what band that is that's great and that's Bob Weir often called The Other One and anyhow had a song called The Other One which is kind of a classic anyway, so a few others lymphocytic coriominiditis virus is something that you might want to know something about single stranded RNA virus produces a symptomatic maternal illness in certain cases rarely transmitted during episodes of paternal byrenia serology and by western blood the systemic findings are macroscephaly, hydrocephalus intracranial hallucinations and neurological abnormalities but typically looks very very similar to toxoplasmosis and is distinguished from toxoplasmosis not so much by the lesions in the back of the eye but serologically and the pattern of intracranial hallucinations which are more perpendicularly located in LCMB there's another emerging infection that was very important in the last two or three years that looks like this that produces congenital infections but also produces a congenital syndrome which is pretty awful I don't know what that is Zika so I think it's important to know that Zika viruses can produce these kind of colobometis corto-renal atrial changes it's a single stranded RNA phlobivirus it can produce a mild flu like illness but congenital diseases like coloboma and torpedo maculopathy hemorrhagic retinopathy can be diagnosed by reverse transcriptase PCR you know the blood urine which of course is not widely available to where it's most prevalent okay four-year-old child painless urinal decreased vision that's for business that's what you're looking at toxo-cariasis toxo-plot toxo-chara toxo-cariasis so that's our toxo-cariasis occurs in kids with a history of pika and contact puppies there are two types of toxo-cariasis visceral larval migrants which occurs in a younger age and is associated with peripheral eosinophilia larval migrants older with no eosinophilia and is diagnosed on ophthalmoscopy it's eolateral in 90% there are three different presentations that are important to know about so this is a posterior pole granuloma there's another type where there is a peripheral granuloma which is frequently associated with tractional band to the upper nerve this also has that on examination and then in older children there is an endothelitis presentation the diagnosis is clinical with supportive serology it is important to perform an ultrasound and in some cases in which the diagnosis is difficult and why is that can be helpful in descent to the CDC so the differential diagnosis again of toxo-cariasis or what would be your number one differential diagnosis for toxo-cariasis retinoblastoma you also need to think sometimes these eyes can be very, very inflamed and also other than retinoblastoma other infectious processes as toxoplasmosis was mentioned before and retinobascular to general retinobascular processes such as ROP and fever with these kind of tractional bands coming from the periphery there is no uniformly satisfactory treatment for this some people do use anti-helmet treatment for systemic disease like with the albensol but for the ocular complications we usually treat steroids and vitroretinal surgery complications in the disease this is another interesting case that kind of walked in on a fray 15 year old woman that we saw actually recently in our clinic with congenital CNB with a two week history of photophobia and blurred vision that's medical history of a significant congenital CNB hearing loss and developmental delay she had a quote panheviatus in her felloi with subsequent retinal detachment and repair and vision loss so she's unilateral comes in with visual complaint in her felloi okay so her right eye with LP let's see a picture of that and a left eye 2060 normal pressure cells in her AC and cells in her vitreous this is what her right eye looked like that should kind of get your attention right looking at the other eye sometimes helps you think about what you could possibly be dealing with in the other eye so we see here an eye that has significant inflammation has epiretinal and subrennal fibrosis and is semi-attached okay with it looks like reminiscence is still vitreous over the optic nerve the felloi has this kind of whitish lesion superiorly okay and then there's some other stuff going on peripherally as you can see here anybody want to hazard see anything going on in the peripheral retina of this eye it's not an artifact there could be subrennal fluid you can see any peripheral retinal whitening it projects so well but there's a little bit of retinal vasculitis too okay so we have panheuvitis, retinal vasculitis papillitis retinitis in the left eye okay history of retinal detachment in the felloi which may clue you into maybe something that's going on in the octavide previous labs were so what are you thinking about here what can destroy an eye so an necrotizing retinitis exactly anything else so it can aren't occur and get definitely okay so thinking about infectious disease you know infection can be the usual suspects right I thought possibly there could be a metastatic endothelitis or toxolation in one area non-infectious disease for academic purposes I don't think she had this so this is what she kind of looks like next step what is a clinical diagnosis I think you have that necrotizing retinitis she's got cells in her AC cells in her vitreous retinal right whitening what would be the next step so that's we did an e-way and we actually she had undergone some laboratory work but she did have an AC tap an injection against vicar and flascare and she was positive for BCB so confirming the diagnosis of ARN initial treatment she's a young kid she's developmentally delayed what would you do send her home send her home with IV antiretrovirals that's exactly right so we admitted her IV antiretrovir and then transition to her she received several additional injections and we placed her on prednisone shortly after we started she received a laser barricade to the temporal retina given her history of retinal detachment in her other eye as we know in ARN 75% of the patients can develop retinal detachment and in a young eye in which the vitreous is attached you're playing with fire so okay so there's no randomized controlled data that says that laser barricade is indicated so I think it's pretty low risk and potentially protective so six years later she's still 2025 doing great and she's on prophylactic you know develop acyclovir again I'll probably go over this but you know there are criteria for the diagnosis of necrotizing retinitis and it's really a clinical diagnosis okay we get PCR confirmation PCR isn't always confirmative you go with your clinical gut I think so retinal macrosis rapid progression with our treatments or preferential spread inclusive vasculopathy usually with arterial involvement and prominent inflammatory reaction in vitreous and acy so she met these criteria one of the reasons why people with ARN lose vision it's not only from retinal detachment I try this but it's also optic nerve infiltration from so they can develop an optic nerve can you do anterior chamber instead of vitreous top yeah I mean that's a good question because I was pretty convinced that my differential diagnosis is pretty narrow it was what it was a narrow differential diagnosis it was from the ac tab was pretty good and it was less important for that particular trial we would have done and we wanted to see what she looked like under anesthesia so it was pretty convincing you were pretty sure that you'll get like a positive something herpetic from the ac plus the kids vitreous is pretty solid it's more like traumatic and you might not get what you need is that what you mean I'm not sure I completely heard your question so like and you say that your differential is narrow plus ac tab is less traumatic you mean you're looking for something like herpetic or vzb so we're pretty sure you can get your positive result plus the kids vitreous is pretty solid so it's more traumatic and you're not going to get a positive result so I think that the yield is pretty good for an ac tab for ARN and like in the clinic if a patient comes in with a clinical diagnosis of ARN they have no other you know, contravening factors they don't have any other risk factors not immunosuppressed thinking of you know endogenous endothelminus or other infectious disease I would perform an ac tab and inject them and get them treated because my clinical impression because I'm making a clinical diagnosis of ARN if I'm thinking that it's more it could be ARN but it could also be syphilis or it could also be toxoplasmosis I mean that area could have been I guess it was an immunosuppressed toxoplasmosis where you couldn't really make a differential so it was a clinical call and I mean vitreous pox is not that big a deal okay but in a she already lost her other eye so we're going to minimize complication and at EUA we were pretty convinced that she had a good question okay 12 year old from Idaho unilateral recurrent multifocal cortiretinal inflammatory episodes get scanned to try this we were talking about this yesterday you see anything in that area of this so in the area that's not highlighted what do you see looks like a DMX staff so let's just this is kind of like you know patients don't present like that but what do you see other than the area that's highlighted normal do you see anything in the or PE or periphery it looks like there's hypopigmented lesions it's a multifocal kind of chorus and what do you see in the highlighted area F S shaped hypopigmented track lesion it's like a I don't know something an S shaped something there's a worm there there's a worm there there's a worm there turn up the light turn up the light turn up the light turn up the light so dozen is our nematode infection it could be caused they were initially described by the taxotermic but it's caused by these nematode infections associated with Baleascus which is seen in raccoons and skunks north and midwest and Canada and then eclosicinium dog hookworm this is what it looks like on EM usually it presents with unilateral recurrent multifocal and diffuse inflammation of red and pigment epithelium so differential diagnosis early in the disease you get faked out that these are white dots and drops this is another case of dozen that looked like a white dots but the arrow is pointing to a curled up later so yesterday we were thinking about differential diagnosis of RP so that's why I think aquar and I were thinking throwing out dozen as a differential because the end stages of this can look like that so treatment what's the treatment for dozen? laser it laser the worm and sometimes it can be difficult to laser the worm because they move and they are phototropic you have to shine a light on them and they run into the shadows there are some thought that maybe treating patients with albendazol may get the worm stoned or something and they'll get slowed down and make it easier to burn the worm and there's no danger in terms of inducing lots of information in the eye after treating the patient with laser so you guys are not infrequently called to see pediatric patients for endogenous bacterial infections you guys see this does that look familiar to any of you do you ever see this when you're calling on consults so what is that usually an immunologically immunologically immature or neonates in most places kids or those on immunosuppression here's the same thing a little higher magnification it's a good thing to have a retinal infiltrate with hemorrhage what would be your clinical diagnosis of this what group of organisms what could it be fungal it's in the ICU got lines in fibro because of bacterial infection so important pathogens Candida, Pseudomonas Staph Strep, Mophilus most of the time you will be when you are called to see these patients correct me if I'm wrong but they will tell you the world has an underlying infection we want you to rule out augurum all and I think that a lot of the time it's not always that easy a call and then what do you do when do you treat them what do you treat them for their ocular disease and for Candida the evidence to suggest that the lesions themselves unless they have broken through into the vitreous and producing a contractus can be managed with systemic therapy without intravitual injection or with contractinine but what if you have you don't have an extra ocular focus or an underlying systemic condition and no signs of inflammation again retinoblastoma retinoblastoma so in a patient with retinoblastoma there would be no apparent signs of infection maybe 8 year old female presents with unilateral painless decreased vision in the right eye history of cervical adenopathy mild fever and she has a new pet that's that's the what is this describe what you see what's that I would say this could be Bartonella you would say it's Bartonella I mean that's a possible etiology but what are you actually describing right neuroretinitis exactly and what makes it neuroretinitis so there's a partial macular star on this side so neuroretinitis neuroretinitis doesn't necessarily have to be Bartonella right it can be many things okay infectious disease Bartonella is the most common cause of neuroretinitis no question about it however all of these other pathogens can produce potential neuroretinitis right sylphus, Lyme, Dueson, Rocky Mountain spotted fever okay then you can have what was initially described as labor's idiopathic still ate maculopathy back in 1916 so there's an idiopathic variety of this many people think that maybe labor was describing Bartonella but there's no way to know that there is idiopathic with the recurrent attacks and there are other other retinal diseases that can look like neuroretinitis like Irvan can also produce macular star and then infectious causes such as sarcoid and polyurethrosin so cast-crutch disease most commonly three species cause ocular disease there are seven of them Bartonella, Hensley, Aquatonic and Bacilliformis the highest we see this mostly in kids and southern United States in late fall asking for macular manifestations is helpful in establishing the diagnosis most have proceeding fluid like illness and regional adenopathy and an arithmetis popular at the inoculations site of the cat scratch a disseminated infection can have fever, myiosis melanomegaly and cephalitis ocular disease we know there's parnods ocular glandular syndrome and retinitis and importantly the macular star can also produce a multifocal retinitis so Bartonella can actually have multiple manifestations it can also produce vascular occlusive disease it is clinical diagnosis with confirmatory serology broad differential as we mentioned and then there are really no definitive guidelines for the cat scratch disease because the natural history of this disease is actually very favorable with a very high percentage of patients who are recovering excellent visual acuity but if it does if it does require treatment doxycycline in children that are older than 7 or 8 years old Cipro, Isithro and Ratham can be used I think maybe one or two 16 year old with bilateral blurred vision floaters, vitrides and snowballs we don't see a whole lot of this hikers with she's a hiker with a photograph of her rash you should be able to make this diagnosis by looking at this what are you looking at someone describe this rash with dermatologists in the room targetoid targetoid what is it called arithema migraines it's a targetoid rash that's associated with Lyme disease, she recently relocated from the east coast Lyme disease as you know is tick borne disease from the deartic it is endemic to the northeast of the Atlantic states my colleagues in New York they see actually a lot of this we participated in studying in terms of Lyme testing here and really found that it was positive predictive value of Lyme disease very very very poor without history only 25% of patients recall a tick bite systemic stages of disease are similar to that of syphilis with an early disseminated persistent phase and the systemic manifestations of this disease are dependent upon the stages of the disease early in the disease you have arithema migraines that's diagnostic disseminated even meningitis, Bell's policy I got a call from my mother-in-law a couple of years ago saying that she decided her face was drooping she was going to see her general practice and she lives in New Jersey and I said really and that's exactly what she had she had Lyme disease so you know facial policy is not uncommon and persistent it can be really problematic with arthritis and neurological syndromes the ocular manifestation depends upon the stages of disease with follicular conjunctivitis being most common early and intermediate amnioticis which is the more common presentation disseminated with persistent disease so C, posterior amnioticis although I had never seen that personally and then keratitis and the persistent stage of the disease the diagnosis is with ELISA and with Western block conformation and the differential diagnosis of course in children and older people it is JIA and intermediate amnioticis it's again treated depending upon the stage of the disease with oral antibiotics or with syndromes IV antibiotics and neurological doses finally masquerade syndromes we've touched on this so I'm going to describe this unusual looking hypopia it's mixed with him so called pink hypopia mixed with hemorrhage so what would that make you think about I mean we have masquerade syndromes on there so it's certainly what masquerade syndromes would you think of what's that herpedic herpedic is definitely in the differential for sure but what other masquerade syndromes would you think of excellent usually that doesn't present with hypopia but what does that present with hyphema and nodule right they didn't track your pressure so hyphema, nodule I was trying to get at acute leukemia as usually rather than chronic leukemia we'll produce a pink hypopia in these eyes Cole what's the other diagnosis retinoblastoma okay retinoblastoma ariana JXG for sure endoplamitis post transplant proliferative disorder intracur farmed body you know so modifying the prognosis early diagnosis in kids critical suppression of intracur inflammation or the antibiotic cover if you think you're dealing with an infection the early use of stereospring immunomodulation particularly in children with chronic diseases like GIA identifying eyes at risk for complications right so patients with JIA that present with posterior stegia are at risk for further complications and our historic markers for poorer prognoses we didn't discuss other things like laser photometry there is suggestions to reassess current screening guidelines and then mutual and post-marketing surveillance of randomized controlled trials for the safety and efficacy of new biological therapies that's the end those are my kids awesome you have to edit that out I should have saved that thanks for my telling thank you very vows