 Good morning. I think we'll get started with our grand rounds. We have two speakers this morning. The first will be dr. Kelsey just her sweat lick from our neurology department She'll be presenting about optic nerve drusen and then the second presenter will be dr. Zog Who present about intracameral antibiotics for endophthalmitis prophylaxis? Hey guys, I want to thank you for having me and thank you for a great month here at the Moran My presentation is on optic nerve head drusen and something that came up a lot actually during my month here at the Moran So I have two cases very different to present the first case is a 15 year old female She came for the valuation of transient blurry vision. It involved both eyes Lasted seconds occurred about once a week didn't seem to have any triggers or day or diurnal variation and She was evaluated initially by a outside ophthalmologist who noted inflammation of the left optic nerve And hence their furl to neuroophthalmology. She had a CT head and that was read as normal No signs of increased intracranial pressure She does have a history of headaches. They said sound more of vertiginous in nature And then she had some nonspecific neurologic symptoms including lightheadedness and anxiety Her history was Notable for seizures. She had a febrile seizure when she's a kid and again when she was 12 years old after watching actually a gross Movie in in school. She had some mild headaches. No ocular history that we could ascertain Past medical history. She had recently diagnosed hypothyroidism and just started treatment for that Depression as well. She had syndactyly requiring orthopedic surgery in the past. She was obese Family history. No one had any ocular history. Her mother did have multiple sclerosis as well as migraines She's in the ninth grade having trouble in school because of her anxiety So her exam was Notable for Excuse me. I'll make this quick. It was notable for normal visual acuity. She had normal pressure She did have a APD In the left eye, but not in the right eye her visual fields were full her eyes were Orthotropic and on her Humphrey Humphrey visual field on the right. It was normal on the left she did have superior arcuate defect and If I was really good, this is what I would see on her fundoscopic exam So we can see the right looks pretty unremarkable on the left. There is raised optic nerve 360 degrees And you don't know if anybody can see anything else there was the question of maybe some Optic nerve drusen in the left pretty unclear from from these pictures If we look at sprectralis, they really jump out at you though. So here you see Inferior really on the left optic nerve drusen showing up is a hyper acute fluorescence And her CT head here, which is red is normal. I don't know if anybody can find anything abnormal We're looking at the left eye. It's really hard to make out But on review we thought that there is a little Hyperdensity right there in the posterior globe of the left eye and that would correspond nicely with the buried Druze So this was a really nice example. It's not too exciting. It's something. I'm sure you've all seen before but pretty classic And so a good exemplary case of optic nerve head drusen It's autozomal dominant trait about 1% of the population has it if you actually look for it And the prevalence increases significantly if you have a family history about 10 times It's more common in Caucasians And they do note that it's rare in African-Americans because they tend to have a wider scleral canal Which is interesting the cause is sort of unclear we know it's predisposed by a Scleral canal and optic disc that's large smaller than average and this causes some stasis in the axoplasmic flow and abnormal axonal metabolism Allowing for a calcium like low globular deposit in the papilla the natural history Often early on they're buried and they can be uncalcified And not clinically significant as you age so in the second and third decades They can be come more visible at the op at the disc surface and Symptoms generally asymptomatic you can have visual field defects most commonly You'll have an enlarged blind spot or you'll have a Arquea defect or scatoma Rarely people will note decreased visual acuity Visual field defects are much more common if the optic nerve drusen is visible at the surface if they're buried you only have about a third percent chance of having visual field defects and Interestingly there is an association with Retinal hemorrhages and a lot of times this is thought to be due to the interference of the nerves blood supply This can happen in two to ten percent of the population So identifying optic nerve head drusen on their own they're not Very concerning, but they can lead to Misdiagnosis and mistreatment so we have we had a few cases that received lumbar puncture that were on diamax Which they did not tolerate whose moms were scared. They had a mass lesion in their brain So they can cause some morbidity if they're misdiagnosed and lead to unnecessary testing until recently we relied on direct visualization and in the 90s and early 2000s B scan ultrasonography was thought to be one of the best modalities to pick these up Cat scans as you saw sometimes can see them and then the thought of fundus autofluorescence as well So our second case is a two-year-old boy He was referred from an optometrist After the optometrist noted that he had bilateral disc swelling on exam about a year ago And he was evaluated initially not because he had symptoms but because his sister had IIH and his mom wanted him to be evaluated for that as well. He was started on diamox 500 a day When he was diagnosed with a bilateral disc swelling didn't notice any improvement Especially because he didn't have any symptoms So he denies any of the classic symptoms of IH and no dimouts pulsatile whooshing Diplopia Apostral headaches no risk factors. No weight change or new medications. They did not LP him He has not had a recent MRI His only ocular history. He is colorblind In his past neurologic history is this interesting diagnosis of a pervasive developmental disorder Not otherwise specified with sensory processing disorder is an infant. He's worked up at primary children's mom thinks He's now meeting milestones. He just required some extra tutoring His sister who's now 14 was diagnosed with IH when she was nine mom has quote-unquote poor vision And on his exam his Visual fields were visual function was normal except for color vision. He did have Cometent right isotropia. His anterior chamber exam was normal and on fundoscopic exam You could see what I would describe as funny-looking optic nerves. So very anomalous he has Normal normal vessels around the outside, but definitely raised especially superior and basically no cup-to-disc ratio his mother had Small-coupless optic nerves as well. She did have venous pulsations present and she did not have drusen So this is his spectralis Scans, and we do not see any drusen In comparison to our previous case just the anomalous looking optic nerves And then we went on to get more information because we couldn't quite tell why this guy's optic nerves looked so funny Whether it was just his nerves or there's something else going on So we did get an OCT of the nerve retinal fiber layer, and it just showed some Some superior Fulness on both sides We only did a Humphrey of his left. He had had a Humphrey visual field of his right And he really wasn't very good at doing his Humphrey. So the one on the left really didn't show anything notable And then we went on to get enhanced depth imaging OCT We see here that he had very full optic nerves on both sides And then there's possible very drusen Notably there's a hypo reflective kind of foci here surrounded by some hyper reflective foci a little bit unclear But we thought that there was bilateral optic nerve Drusen and there's some scatter which you can see with those as well So OCT was first used on the human eye in 1991. It's been in clinical practice since early 2000s And it's really revolutionized the way we look for certain pathology. It has a very high resolution So four to five microns And it allows cross-sectional imaging So Randy Cardin who studies this a lot presented multiple cases And lectures at the last nanos Likens this actually to in vivo biopsy Because of the high resolution. There's been many studies Looking at whether you can use OCT to compare optic head nerve drusen to optic dyscadema And they're very favorable. So I noted kind of the three main ones. They're all very similar as far as How many patients they looked at and they're finding so the first Johnson studied 60 patients And found that with 80 percent sensitivity 90 percent specificity You could use a retinal nerve fiber labor and the sub retinal hyper reflective space thickness to differentiate these two entities Lee likewise found that the retinal nerve layer fiber layer was very helpful, especially the nasal section Which was significantly thick thicker in the dyscadema and they gave some standardized levels And then the last one Sarac looked at three it looked at 75 patients and Pretty much had the same findings all statistically significant So what are we looking at here when we talk about the enhanced depth or the with the OCT? on the left we see at the top just a nerve normal optic nerve and then Middle and bottom left. This is mild and this is pretty marked Elevation due to optic nerve drusen and then on the right in comparison is dyscadema and When you compare the two, you notice that drusen tends to have what they call a lumpy bumpy pattern and Over here at the dyscadema. It's more. They call it kind of the lazy V pattern here So enhanced depth imaging is something we started using Recently and it's just a recent modification to this standard spectral domain OCT The first study which really showed The importance of this was say though in 2013 and this was retrospective and they basically Showed that This EDI allows a complete depth of the pre-laminar optic nerve Thereby showing the optic nerve head drusen in its entirety before that you can just kind of glimpse the top part If they're deeply buried they're hard to appreciate Additionally noted an inverse correlation between the size of the drusen and the nerve The retinal nerve fiber layer thickness and then a prospective cross-sectional study was done the same year Showing that the EDI has a statistically significant detection increased detection rate Compared to B scan ultrasound So 52 versus 40 of the 68 patients that they were looking at And the interesting thing here is they divided these cases into definite suspected and then normal optic discs and Enhanced depth imaging compared to just the normal spectrodomain imaging did not have a Statistical significant difference when they were definite cases But it was very helpful when they were just suspected the EDI did have definitely improved improved findings so Kind of in a nutshell the benefits of the enhanced depth imaging it shows the deep borders that are often missed by conventional OCT and It allows us to assess the shape structure and topographic location of the drusen within the optic disc so Here in the middle we have just the Standard OCT and in the bottom we have the EDI OCT and I don't know if it comes up well, but Both of them have this hypo reflective area, but here you can see the kind of inferior margins more clearly you can see hyper reflectivity more clearly So you can imagine if there's a smaller drus very deeply it might not show up kind of in this Gray zone here, but would Here there's a little more contrast So with our two with our last patient. We know he has Anomalous looking nerves. We think he might have drusen is this explaining His presentation are we missing something and we always worry that we could be missing something that could harm him Such as papillodema again. He hasn't had an MRI or a lumbar puncture yet So can we really rule out papillodema at this point even given our new imaging techniques of EDI OCT? so There are many people who do think that EDI OCT can be very helpful in identifying papillodema and this is From a talk Randy Cardin gave he's from the University of Iowa He covered this topic extensively at the last NANOS meeting But he finds that OCT can help quantify papillodema severity Objectively based on the retinal nerve fiber layer thickness in the disc volume. So this is in comparison to the subjectivity of just directly looking at the the discs and He notes that there tends to be in the past we have our grading system, but a lot of intra and Observer variability in the grading and this chart here nicely illustrates a strong correlation between the retinal nerve layer and the grade the freezing grade of the papillodema so Additionally when you look at the enhanced depth imaging OCT Dr. Cardin Asserts that there's a deformation of Bruch's membrane which can allow you to Determine the pressure differential between the kind of retro bulb or compartment in the vitreous cavity So a very positive angle towards the vitreous indicates increased intracranial pressure These are two pictures EDI OCT of the same patient with Increased intracranial pressure and you notice on the top Bruch's membrane here is Angulated sharply up so you can imagine pressure kind of pushing out When this was treated with high volume tap Bruch's membrane has normalized it's almost flat actually almost a negative angle there So that's the treated scan If we look at our patients again So this is from that second patient. We notice here that Bruch's membrane does appear normal So it doesn't look like it's outcouching away from the optic or from the globe and I Recently found out that Drew's is actually a German word for geo because of its glittering appearance And I want to thank dr. Warner and dr. Katz and dr. Crumb and dr. Canard for putting up with all my questions and helping me this very Interesting and challenging month and if you have any easy questions for me, I'll be happy to take those If not, I know someone I can talk to Here are my resources Sorry about the formatting I fixed it last yesterday last night and it didn't stick. Yeah Oh