 Good morning. Welcome to our Medical Student Grand Rounds. Our first presenter is here from the University of Utah School of Medicine, Matt Conway. Matt has been to one-third of all Spanish-speaking countries in the last 10 years and plans to get the rest of them, get up to 100% over the next 10 years. And today he will be presenting a case on acute retinal necrosis. Jordan, thanks for that awesome introduction. I'm excited to kick off Grand Rounds today with an ocular emergency, something pretty interesting, acute retinal necrosis. I got to thank Dr. Shakur. This is one of his patients and he has been pivotal in working through this case. So let's dive in. Day one, case starts out with a 47-year-old male waking up in the morning with periocular pain in his left eye. He also has a vision decrease and a headache. He goes to see his local optometrist and he started on prednisolone for anterior uveitis. He was seen seven days later with no improvement. Day 13, the symptoms are still persisting regardless of treatment and he's referred to the VAER and then subsequently the Moran. His past ocular history is notable for LASIK in 2006, but no other recent eye surgeries or trauma. He has had VZV infection before with chickenpox and shingles. His family history is only pertinent for glaucoma and his social history is pertinent for tattoos without swelling. He has multiple cats and has subsequently suffered several scratches from those cats. He's married with no recent international travel. His ocular vital signs show something very obvious, that decrease in visual acuity in the left eye. He went from 2020 to 2100. He also had a possible APD, but his pupils were fixed after post dilation at the VA. On slip lamp exam, big things that stood out on this patient were the cell and flare in the anterior chamber and the edematous and slightly hyperemic disc in the cell and haze in the vitreous. Let's look at this retina right here. You can see that 360 degree retinal whitening. This picture is pretty impressive in a classic presentation of acute retinal macrosis or ARN. You'll see that intra retinal hemorrhages here and you'll also see the posterior pole is spared. There's also some folding coming off of the disc as well. In OCT shows a little bit better, there was some optic disc edema and some macular edema as well. After seeing this kind of presentation, the differential diagnosis that comes to mind is most likely for a viral retinitis. You're looking at ARN, progressive outer retinal macrosis as in porn, CMV retinitis, ocular syphilis and other infectious etiologies. Because this presentation is pretty classic for ARN, I also wanted to go over real quickly what the other viral retinitis look like. Progressive outer retinal macrosis, unlike ARN, you'll see this in patients that are immunocompromised. ARN progresses really rapidly, but porn goes even faster, which is impressive. Also, big difference between the two, on-slip lamp exam in porn, you won't see the tritist as you'll see in ARN. Then the posterior pole is involved a lot faster because the disease progresses a lot faster. Here's a classic presentation of another one of Dr. Shakur's patients. CMV retinitis, these patients are also immunocompromised. You will see a prominence of hemorrhage as compared to with ARN, a minimal vitritus, and also you will see posterior pole involvement a lot earlier on, and the periphery may be involved early as well. Acute retinal macrosis, epidemiology and etiology, the basics. Let's start it out with this. This usually presents in patients that are 50 and 60 years old. This patient was about to hit his 50s. Most cases are caused by herpes viruses. The most common is VZV, followed by HSV. There are some recent cases. There are case studies that show that CMV and EBV can cause us pathology. The patients are usually immunocompetent, but can be immunocompromised. As you saw, this is what makes it stand out compared to the other viral retinitis. Women and men are also affected equally. Acute retinal macrosis presents as you saw with this rapid onset of pain, vision loss, floaters, and photophobia. It usually presents in one eye, but will progress to binocular to involve both eyes in most cases if left untreated. The vitritus is really common. As you saw in that photo, there's those small foci that are yellow and white. They have those very discreet borders, but they will progress together rapidly if left untreated and coalesce together. The arthritis you'll see is pretty profound. Unlike CMV retinitis, there will be few retinal hemorrhages. People can see discodema, optic neuropathy, and retinal detachment. Diagnosis, with the presentation, most of the time it's just a clinical diagnosis, but further testing is required with an anterior chamber tap. You'll send that fluid off for VZV, HSV, and CMV and toxoplasma, toxoplasmosis PCR testing. It's interesting, atypical toxoplasmosis actually presents a very similar to ARN and can be a mimicker. It's something that you got to test for. Other testing considerations, if the history indicates, you can also get a CBC, CMP, and test for cephalus, tuberculosis, and other etiologies that would cause a patient to be immunocompromised. The treatment for acute red and macrosus is changing a little bit, but the conventional treatment is to use IV acyclovir for five to ten days and then to switch over to oral maintenance therapy for six weeks to three months, depending on how the patient progresses. In the last five years, several studies have shown that if you use oral Valtrex, you can get similar concentrations in the body because of the great bioavailability of Valtrex. This option is a lot cheaper and a lot more convenient for the patient and the health care system. It's becoming more commonplace now. In patients that have severe disease, you will want really rapid treatments to stop the progression of the infection. Intravitral antivirals are key. Key signs that we'll clue you in to use those are optic dyscadema and macular involvement. You can also use oral steroids after the first two days of antiviral treatment. Other really important things that you can decide to add on to patient therapy is antiplatelet therapy to prevent ischemic damage to the retina and optic nerve. Additionally, you can use topical steroids if you have an interior chamber involvement in panutipitis. The major goal of treatment is to spare the unaffected eye considering how many complications you will have if infected eye. This case, protocol was followed, AC TAP was sent for the following and it came back positive for the most classic culprit, VZV. The rest were negative. The assessment for this patient was ARN with panuvietis secondary to VZV. Important distinctions was there was no neurological involvement. This patient was immunocompetent and there was 360 degrees of peripheral retina involvement and dyscadema. For that particular presentation, you want to hit it with the full treatment and that's what we did. As you can see, these are the medications we started or the team started and they added in the introvitial injection to stop the progression of the disease. As I said, acute retinal necrosis is not your emergency and can have many complications as outlined here. The most common is retinal detachment. This will happen in 70% of cases. It happens because of the pathophysiology of the disease. The necrosis retina creates these holes in the retina which allow fluid to enter in and separate the retina and causes detachment. This is exactly what happened with this patient. Two months after the initial presentation, they showed up with new flashes, floaters and vertical and horizontal photopsias. Their visual acuity decreased from 2100 to hand motion only in the left eye. On their exam, they showed inferior detachment, extending to the macula, which I'll show you here. You can see that in this image, there's this nice border here of this retinal detachment, classic presentation. Hard to treat with these patients because of the inflammation that's already happening in the eye. Here's the OCT demonstrating the severity of the detachment as well. Management in this case was classic. Patient underwent retinal detachment repair with vitrectomy, scleral buckle, and silicone oil placement as well as endolaser. Three months later, patient returned to the OR for follow-up and to remove the oil and also had developed a cataract considering inflammation in the steroids that the steroid treatment they've been on. They had a cataract niolail placement. The current status of this case, on Friday, we saw this patient together. A year after the original insult, the patient's left eye has decreased the hand motion and only visual acuity. OCT has showed that the macular dema has been resolved, although there's some temporal atrophy. In the right eye, it has been spared thus far, which is a great relief, and its visual acuity is still 2020. We've continued vialcyclovir treatment so that to prevent further progression of the disease, this is the OCT photos of the retina. As you can tell, this is very significant for fibrosis in the periphery with laser scarring, and it's pretty impressive the amount. It's pretty impressive what ARN can do to a patient. The key takeaways from this case is always dilate the pupil when you see anterior uveitis. Earlier treatment can save vision and also prevent progression to the other eye. Huge thank you to Dr. Shakur, Dr. Orozco Patel, Altman and Bear for your help taking care of this patient and also with your guidance in this presentation. Thank you to the marine photographers for providing these awesome photos. These are my references. I'll leave it open for a couple of questions in the last couple of minutes. Delays in treatment cause progression to the other eye that is not involved, so binocular involvement, and then also if you treat it earlier, you can prevent retinal detachment just because there'll be less retinal holes. With that being said though, barricade laser is really controversial and so that is not necessarily a preventative treatment because it doesn't necessarily prevent retinal detachments. It's kind of a follow the treatment guidelines in hope that there's no detachment and hopefully caught it on the map really enough for that there's no holes and no detachment. Thank you Matt. So our next presenter, Madison Perchick, when she was a kid, was on a trip to Costa Rica and mistook a saltwater crocodile for a log classic, classic mix-up and was almost eaten but was not and with her newfound life went to the University of Tennessee College of Medicine and now she's joining us here at the Moran to discuss a case of a complex patient presentation. Thanks. Good morning. I might or might not need help, maybe not. Good morning everyone. My name is Madison Perchick. Thank you so much Jordan for the introduction. Is the true story about the crocodile? I'm so excited to be with you all this morning and my talk is called A Complex Patient Presentation. So let's get started. Alrighty so our patient is a 49 year old female currently and she has a past medical history relevant for Marfan syndrome. So the first encounter that I could find with this patient in Epic was in 2013. At that visit she was said to be a fake kick on the right and she was symptomatic from a dislocated capsular bag with Phymosis. That same year in 2013 she was also diagnosed with myopic degeneration OU and then in 2014 she underwent an anterior PPV, a lenzectomy and then a sutralis three piece scleral fixated IOL and this top picture on the left is a picture of her retina which shows IOL tilt and also scleral show which is common in high myopes and then the bottom picture is an OCT that is consistent with her myopic degeneration. So a little more about her. After that procedure that she had in her surgery that she had in 2014 she did have an episode of post-operative micro high FEMA and after that episode she really was pretty quiet until around 2019. That's when she started having more issues. So in 2019 she started to have some recurrent micro high femas and in that same year she had her first episode of inflammation. She came to Moran and said that her right eye felt like when she woke up that she was waking up from a steam room. So she was found to have uveitis and she had reactivated uveitis about once years since then but subjectively she said in the last year or so that it's been worse, they've lasted longer and they've been more difficult to treat. Additionally with these episodes of uveitis she's also had intraocular pressure spikes that are semi concurrent with these uveitis flares so it seemed to be responsive to the steroids that were being used to treat uveitis and since then she's been on both steroids and intraocular pressure lowering drops and the optimization of which has been ongoing because as they have tried to decrease her inflammation with steroids that increases her intraocular pressure many times so that optimization has been difficult and notably she also had an episode of her pedicarotitis in 2020 that was treated with oral valtrics and then this picture on the left depicts data from Epic that this line graph was pretty cool I thought that Epic gave it to us and the top line the blue line is her intraocular pressure from 2019 to now and then the bottom line is her right eye's distance vision without correction so I just show this to say that you can see that she had a few spikes of intraocular pressure in 2019 on that way left side but you can see in the last year she's had a lot more problems with it and that has correlated with a decrease in her distance vision as well in that right eye so the differential diagnosis for her at this point was Ugg syndrome versus chronic herpedic uveitis and the uveitis team was consulted for help in the rest of this workup so her labs were notable for a positive ANA but the uveitis team was not super worried about RA or lupus as she didn't show any more systemic signs of these conditions and they did note that her inflammation recurred even on valtrics 1 gram TID which should have been plenty of valtrics to treat chronic herpedic uveitis so that kind of leaned them away from that picture and lastly they did an AC tap which was negative for the viral PCR which was kind of the final step to lean away from that chronic herpedic uveitis and then in 2019 a UBM or ultrasound bio microscopy scan showed haptic ciliary body touch and then this picture on the left is from a study that I found that I thought did a nice job of pointing out these little arrows are representations of where the haptic is and it touching different parts of the uveal tissue and then this is a picture from Moran of our patient with her haptic against her ciliary body so because of this whole picture looking at everything the recurrent uveitis the entry increase in trochlear pressure the micro high femas the ubm results in that negative viral PCR the most likely diagnosis for her at this point was ugg syndrome so in 2022 she had an IOL explanation and she's still faking right now and she had an abinterno viscocanolostomy and agonioscopy assisted transliminal traveculotomy all on the right side and her pre-op distance vision without correction was 2060 and her intracular pressure the day before surgery was 41 and at post-op week when she was hand motion on that red eye and then at post-op week two her distance without correction was 2100 she did pinhole to 2060 and her IOP has been much improved her last visit was 16 but she does still have 3 plus cell so she's still on oral and topical steroids at this point she is on PO valchex and she is on cosop BID still her glaucoma or intracular pressure lowering drop regimen has decreased a lot she was on a combination of I think four oral and drop medications for her intracular pressure so that's improved a lot so this patient has just abundant fascinating features of her ocular history I think I could do 10 rounds or 10 grand rounds of of just this patient but I did want to focus the rest of my time on this most likely differential that is uveitis glaucoma hyphema syndrome so this syndrome was first coined by Ellingston in 1978 he described it as a complication of intraocular lens implants causing a mechanical chafing and the pathophysiology of this is that you have this contact of this intraocular lens with uveal tissue that causes inflammation or uveitis it also causes erosion of that tissue so the blood aqueous barrier is broken down which causes release of pigmented cells red blood cells and white blood cells into the ac and so if you have red blood cells in the ac that would be your micro hyphema and then all of these cells can work together kind of to clog up the travecular mesh work and cause that entries increase intracular pressure that we see with this syndrome and ux syndrome is most commonly caused by ac iols or three-piece sulcus iols but they actually can occur with any type of sort of thick lens even in the back so how do we diagnose ux syndrome as you can tell from this patient it's not always super straightforward it can take years it's a chronic syndrome these signs and symptoms that we see do not always happen at the same time and they these patients can have a lot of other things going on as well so a high index of suspicion is really important but on exam we look for things such as of course the micro increased intracular pressure signs of inflammation like cell and flare or hypopions we can look for iris neo vascularization or iris trans elimination defects which is shown in the picture on the left and we also look for malpositioned lenses or haptics it can even cause vitreous hemorrhage or CME as well if you are thinking about ux syndrome in your differential it's also important to go near the patient in order to look for blood within the angle and hyper pigmentation of the trapecular mesh work and signs of mechanical erosion and then lastly like we've we've been talking about ubm is also a mainstay of diagnosis and it's used to confirm the position of the lens and the haptics and its contact or lack of contact with the uveal tissue so lastly the treatment of ux syndrome it if you can imagine a mechanical kind of chafing of the haptic or the lens on any kind of uveal tissue it's probably not going to fix itself so additional surgical intervention is often the most effective treatment so whether that is in the form of repositioning or explaining or exchanging the lens those are the most definitive treatments but in the meantime again because this is a lot of times not kind of a home run diagnosis and it can take years if it's even on your differential there's a lot of there are a lot of medical treatments that can be done in the meantime to treat the patient so for the uveitis we recommend or it's recommended to give them topical or oral corticosteroids to help decrease their inflammation for the intraocular hypertension or glaucoma of course we have oral and drop medications that can decrease the intraocular pressure it is interesting to that we don't usually use prostaglandin analogs in this case because they have been linked to inflammation and then lastly her for hyphema you can recommend that the patient decreases their activity does head elevation uses cycloplegics and then also topical steroids which if they have a uveitis flare at the same time they might already be on and that is all I have I hope that this case was interesting and that it was a good little concise review of ux syndrome for all of you and I just wanted to take a moment and say thank you to everyone for your time today and I've had such an incredible experience at Moran everyone has been so welcoming to me and I've learned so much and thank you to Moran for the photographers as well those pictures are awesome so thank you so much and I'm happy to try to answer any questions you may have and Dr. Manlis if you're unmuted then you can go right ahead thank you it was interesting when UGG was first described by Ellingson it was very poorly finished anterior chamber IOLs causing UGG syndrome and we see this often in in three piece lenses put in the sulcus but also one piece lenses put in the sulcus which should never be used what would be interesting now is to see if UGG will recur following sclerophyxated IOLs using the Yamani technique and I've not seen a case yet but but it would be very interesting because you know the haptics are not rubbing on the ciliary sulcus they're actually going through the ciliary sulcus but it would be interesting to see with the Yamani technique if we start to get any issues of UGG syndrome with the peripheral optic of the IOL rubbing on either the ciliary sulcus or the posterior iris I'm not aware of cases that have been reported I don't know if anybody else has heard cases of UGG syndrome following Yamani surgery the audience says yes that they have seen it before um for the people on zoom that they have seen it with um specifically with when the IOL is tilted yes please I'm sure you can explain it so so what are the great resources in today's medicine is these blogs that people are on and and you can kind of get the A S C R S listserv and for cornea specialists you get the get care in it and so there's a lot of people they're doing complex anterior segment surgery in that forum and Yamani is not 100% full-proof at preventing sort of recurrence of UGG there are cases where they've used I don't know what we call them they're not endoscopes but what do we call them where you actually can look back there with a scope at the ciliary body and kind of see where these haptics are kind of going through there and there are cases where the haptics are actually still rubbing within that ciliary body and then in a lot of cases it is lens tilt issues and iris kind of ploppiness so the recommendation is to have a PI to try to keep the iris a little bit away from the lens if you can and trying to avoid tilt so that your entry points on Yamani are equal and equidistance from the iris as well but yeah definitely not full proof as go to to say we're going to get rid of UGG by taking the ones out and doing Yamani but yeah so Nick this question is kind of directed to you a little bit Rachel is asking Jacoby Dr. Jacoby if anybody's used the apple Miyake view technique to look at a Yamani surgery to kind of see where these haptics are going and what's happening during that surgery you know when when our dear colleague Alan Crandall was alive he and Bob Sione did some Miyake eyes with us when the Yamani technique was first coming out to actually look at the placement and what we found is that it was a small series it wasn't a large series of eyes but we found that the placement of the haptics was variable and I think as as our techniques have improved and people are getting better at marking exactly where you're you know putting these haptics now that has gotten better but still there's a lot of variability in where those haptics actually end up and if they do go too far anterior or tilt as you mentioned then you can get issues with UGG syndrome still and so I think the key thing to prevent that right now is making sure that you have even placement of those haptics and that those haptics are placed far enough back from the iris that they're not going to cause any significant chafing with that optic that could cause UGG syndrome. Okay our next presenter Parker Cox is also joining us from the University of Utah School Medicine by way of Colorado speaking of blogs he runs a food blog with his wife called your cup of cake I was on it this morning it's extremely impressive lots of one pot meals for the busy people out there and more recently he's been cooking up a case review of acute corneal hydrops. Okay perfect no worries all right well thank you Jordan today I'll be talking about acute corneal hydrops and I've broken up my presentation into two primary categories first just going over briefcase review and then second a general overview of acute corneal hydrops I don't have any disclosures to make today so we'll just jump right in. So the patient that I'll be discussing today is a 46 year old male who has a history of keratoconus bilaterally he did have a penetrating keratoplasty on the left side due to the keratoconus and he had multiple cases of rejection of that graft treated primarily with steroids. He initially presented in March of 2021 a complaining of a week of what he described of plouting in his eye he also had some photophobia significant tearing foreign body sensation stabbing pain and dryness just in that right eye he was using artificial tears but said that he was not experiencing any relief it's also important to note that he was having no symptoms in his left eye. I did include some keratometry of his right eye this is however from 2019 so two years prior to his presentation we see some pretty significant steepening of the inferior cornea he has some pretty high k values and some pretty high stigmatism all consistent with keratoconus. Other history worth noting he does have a medical history of migraine hyperlipidemia gout and hypertension. Family history is significant in his father for keratones as well who also has glaucoma and had a stroke mother with history of heart disease. His social history is rather insignificant with the exception of being a former smoker. That corneal transplant took place in 2006 and he had been using a scleral lens since then he had previously tried rigid gas permeable lenses with minimal success. So he at this presentation in 2021 his visual acuity was pretty poor he was only able to visualize hand motions only. He did have pinhole vision at 2200. Left eye was seeing completely normal no afferent people are defect. IOPs were normal bilaterally and movement of eyes were normal bilaterally. On the slit lamp exam his right contactiva was pretty injected left was normal however he had about a six and a half millimeter area of edema centrally in the right cornea as well as some infracentral microcystic edema on that side. On the left as I had mentioned he had had a corneal graft there were no sutures in place there was a little bit inferior scarring and he had 360 degrees of panace with vasculature limited just to the graft host junction. On that right eye the fundus was unable to be visualized due to the clouding of the cornea mededema and the remainder of the slit lamp exam was normal. So he was diagnosed with acute corneal high drops these pictures on the right side of the screen are not from our patient but just shown for visual representation. He was managed with prednisolone, cyclopenthalate, hypertonic saline drops as well as serial follow-up every four to six weeks for several months. Ultimately prednisolone was decreased and the cyclopenthalate was discontinued as well as the mural. His visual acuity was improving with the use of a contact lens and he was recommended that he has plans for a future keratoplasty on that right side. So his clinical course after all this follow-up over the course of about a year and a half he presented to clinic about two weeks ago reporting that his symptoms have been greatly improved. He does continue to experience a little bit of photophobia and some license to be likely due to the scattering of light through that scar. This picture on the right side of the screen above is a picture of his cornea which you can tell there is some notable scarring as well as a vertical tear and decimates membrane here indicated by the arrows. However as I mentioned his visual acuity was improving he sees roughly 2020 to 2040 with the use of a scleral lens. The slump exam showed some apical scarring however there's no edema. And then I also included keratometry from this most recent visit just to note that he still continues to have some steepening of that inferior cornea. No changes in his astigmatism. So for him long-term management includes just continual follow-up every six months continued use of that scleral lens and then future plans for a possible keratoplasty of the right side. So now I'll transition and just talk about a general overview of ACH. It's important to note that it falls into a category of corneal ectatic diseases and is primarily seen in patients with keratoconus and up to 3% of patients or 2.8% of patients with keratoconus can be seen. However also can occur in patients with keratoclubus and polluted marginal degeneration. It's most commonly seen in male patients more than females and typically between the ages of 20 and 40. Risk factors include atopic diseases, eye rubbing and steep keratometry which we saw in our patient. There has been a few studies indicating correlation with learning disorders as well as Down syndrome. And then as far as the pathophysiology the primary cause here is a tear in the decimates membrane and endothelium which causes an influx of the aqueous humor into the stroma and occasionally the posterior or that stroma causing edema. This picture on the right side of the screen shows just that a pretty significant tear here in the central area of the decimates membrane vertically as well as edema due to that flow of aqueous humor through that defect. I've included a couple of histological pictures just to show what that looks like. Here we see some pretty significant apical thinning of the stroma of the cornea. On this side we have some an image of a patient with a cornea stained with trichrome strain showing a defect in the Bowman's layer indicated by the arrow here. And then this picture at the bottom here demonstrates a patient who had a previous ACH and you see the decimates membrane kind of rolled up here at the edges which can be kind of seen in the chronic patient who is not healed well. As far as diagnosis of ACH typically we rely on the physical exam in which decreased visual beauty is common in almost every patient. You may notice an artificially low IOP due to the corneal edema in the patient and then siddle testing can be beneficial primarily just to differentiate whether or not the aqueous humor is flowing through that break of the decimates membrane versus a corneal penetration. And then as far as diagnosing with imaging modalities I've included a picture of anterior segment OCT. This can be helpful just to visualize a break in the DM as well as here we see into the posterior stroma. It can also be helpful for chronic follow-up of patients just to visualize how well they are healing whether or not the DM has been attached and the presence of scarring as well. In vivo conifocal microscopy can be particular beneficial to visualize at the cellular level some of the damage caused and primarily whether or not there are inflammatory cells. Patients who have inflammatory cells present for greater than four weeks have an association or increased likelihood of having neovascularization which can complicate long-term management. And then of course tomography which I've included just to show how the tear in the decimates membrane can affect changes in corneal thicknesses both on the inside and outside. And so as far as management medical management is our mainstay treatments because most cases resolve on their own in about two to four months. As far as pharmacological measures typically we will rely on ocular anti-hypertensives, cyclopegics, steroids and antibiotics as you'll know from our patient we use steroids, cyclopegics and the hypertonic saline. Surgery is needed usually in patients with pretty significant terrapodestimates membrane in which we can use air and gas for a I don't know if I'll say this right but pneumatic disseminopexy to kind of help alleviate some of that greater tearing. The downside to this is patients are required to lay in a supine position for a prolonged period and there is an increased risk for complications. However compression sutures can be used as well for patients who are unable to tolerate the supine position. And then of course as I mentioned in our patient relying on a future plan for a penetrating keratoplasty in that one side this is the mainstay in treatment for patients with keratoconus seem to have pretty positive outcomes such as the patient that I presented today. I just want to give a special thanks to Dr. Amy Lynn and Dr. Gina Kirchenbaum for helping me find this interesting case and give me some direction on some resources to learn from. I'd be happy to answer any questions today or here's the email if there's questions in the comments afterwards. Thanks so much.