 Our next presenter is Michael Burrow and he's the first in our class of Chief Residents to get a real job. He's going to be staying in town as a comprehensive ophthalmologist at Rocky Mountain Eye Associates, so it's really exciting. He's going to be presenting on Rainier Town. Alright, thanks Shrav and thanks Tina for that great presentation. I'm just going to jump right in to keep us moving and get on to Donnie's presentation, which we're all excited about. So this is a presentation about a 26 year old man who presented this last summer to the ER for a week of chest pain and headache. I had no known medical history mostly because he really hadn't seen physicians at all. His mother had hypertension and so he kind of been checking his blood pressure with a home blood pressure cuff and notes that usually during these symptoms he has high blood pressure. On and off headache chest pain for that week and notice that when he checks his blood pressure that it had been in the quote 200 plus range. So naturally he takes some of his mother's blood pressure medications and that'll make him feel a little bit better so then that fixes the problem. So that's how they've been managing it for who knows how long. But he checked it today it was 230 over 150 and since it kind of been going on for a week they decided to present to the ER. His other history really we didn't know because as I said he really hadn't seen a physician at all. I didn't really get this cartoon but Dr. Petty was able to explain it to me so thanks. In the ER just kind of pertinent positives and negatives he obviously right there in red had a blood pressure of 230 over 150 in both arms but his neuro exam was normal. He had no vision changes although he didn't really have his vision checked and then a few other things slightly diaphoretic but normal heart and lung sounds and normal neuro exam. So this I put this in I kind of found this funny but at the same time the next slide kind of does you know give us pause and think this really can affect a young person's life for having this high of a blood pressure for any considerable amount of time. So included in his ER workup he had at least acute kidney injury if not chronic kidney disease with a BUN of 26 and a cranny of 1.4. His troponin when he was even mildly elevated at 20 I mean he was 26 years old and he had this elevated troponin. His BNP used in monitoring for heart failure was very elevated and then he had other signs of left ventricular enlargement which suggests that this was a more chronic process. So he was kind of given the diagnosis of hypertensive emergency which as just a reminder that is reproducible blood pressure measurements of 180 over 120 with target and the organ damage. So in his case he had heart damage with acute heart failure and then also an elevated troponin and then he also had kidney injury. The other ones we kind of said no at least not symptom wise and then he didn't get an eye exam so we really didn't get to look at that. He was admitted to the CVICU for IV blood pressure management and then a workup. And then just as a reminder they're actually a very clear and frequently updated guidelines on the management of blood pressure in hypertensive emergency because there is some permissive hypertension that's allowed so you don't precipitously drop the blood pressure and then actually cause ischemic and organ damage. So this was kind of his goal 180, systolic of 180 for the first one to two hours and then 160 over the next six to twelve hours. During his inpatient stay up to when ophthalmology was involved this was kind of his blood pressure range so the first couple of days he was basically right on target. The third day he maybe dropped a little lower than they would have liked and his lowest was 129 over 89 which is funny because that's I mean perfectly normal but in his case he did note that he had kind of an episode of blurred vision but it went away supposedly. Hospital day four was fine and then hospital day five is when ophthalmology was called kind of late that day so during morning rounds there were no complaints on his party said everything was fine but overnight that night he had a blood pressure of 108 over 75 which when you're coming from a blood pressure of 230 over 150 I mean you can imagine that's just that's quite the difference. So later that evening around five or you know the witching hour five or six o'clock ophthalmology was called and they basically said you know our patient says he can't see anything anymore. So in discussion with the patient it was actually a difficult discussion he just wasn't giving very clear answers. The inpatient team even actually when they called me they said you know this is really weird you know we don't think that he's lying but he's just not being very forthcoming about his answers that's kind of the story that I got so and I agreed when I talked to him he you know he just basically said that you know I can't see anything and when you tried to ask him when did this happen he wasn't sure and part of it was that he had this headache so he's trying to sleep most of the day and feels like maybe he woke up and it was like that but otherwise no pain no other neurologic deficits that we could kind of pull out of him with his difficult answers it was just the vision loss. So we decided after after talking to the team they did an emergent CT head without contrast just to make sure that there wasn't any hemorrhage cerebral hemorrhage and there was not it was read as overall normal. The exam at his inpatient bed he was no light perception in both eyes although with like really intense light from the indirect he did feel like he noted some sense of light detection. We're unable to really do any visual field color red d-sab because of that his pupils were equal and round and they were actually reactive like what I would say is normal and he did not have a relatively fair pupil or defect. Pressures were normal his extracurricular muscles were full and then his bedside anterior exam was normal. So I cheated a little bit obviously I didn't have pictures with me at bedside these are pictures taken kind of a day later when we were able to get into clinic but I bring this up I feel like this was a tricky exam at least for me so if you'll kind of indulge me and think you're at the bedside inpatient you don't have all the tools you know kind of readily available you have this picture in your mind of maybe what might be going on and I thought I had a pretty good idea and we'll talk about the differential in a minute but then I do this exam and his right eye was was okay you know there were kind of some funny kind of pigmentary changes just not kind of the typical fundus in a 26 year old but then I got to the left eye and it it looked like you know he kind of had this really stark well-defined line of retinal whitening and it it kind of tricked me into thinking that wow it is easy that it even feels like it's following this vasculature so then everything was just thrown off for me I said well this doesn't make any sense how can you have someone who's no light perception vision in both eyes he does not have any pupillary deficits no relative a fair pupillary defect but it looks like he's had some sort of vascular occlusion at least that's what I say at the time so I kind of double-checked an exam tried to really get on the periphery and you know this this wasn't exactly following a brand trend already occlusion or anything like that but it still it just wasn't quite normal it was giving you pause so just so I'm going to keep moving I won't kind of have the residents go out but maybe the thing to think about or something that's always helpful for me is to think about the differential diagnosis in a structured way so there's a lot of different ways of indicators one way to kind of go through all the categories so this these are some of the things that I thought of going through a differential diagnosis and kind of what were the things that were highest on my list now rental artery occlusion was not high on my list until I saw that part of the exam and still it didn't make a lot of sense and it was going to have to be combined with something some other process going on so artery occlusion with a PIO and repressor something like that and then stroke was on there and then encephalitis as well so the immediate work of decision you know the inpatient team is basically surrounding me going what do we do next you know breathing down my neck and he needed an MRI brain and orbits with and without contrast but really if he had a CRAO then we actually talked about this just a couple weeks ago with Dr. Jacobson's presentation there's a there's a protocol that you know we call a brain attack especially if it's in this acute setting and that can trigger all sorts of things and most importantly probably is whether or not a patient gets TPA you know the administrative TPA now luck I know if I can say luckily but luckily for me that really wasn't a consideration at this point because he was outside of the TPA window mostly because of his unclear timeline of when this happened so really only decision was well we call it brain attack he's going to get the imaging really quickly the neurology team has to kind of rush over and that's maybe an inconvenience for them but patient safety wise that was probably the best thing to happen for the patient so I did actually go ahead and call a brain attack and the reasoning was hey this looks like it could be a retinal artery occlusion so he had an emergent MRI done and then we said okay permissive hypertension to kind of the 140 systolic range and then plan for a macocet and probably a fluorescent angiography when the time allowed so these are images from his MRI the time so this is a T1 post-contrast scan and you can just see in his occipital lobes here on several slices you have sulcus enhancement involving bilateral posterior parietal and occipital lobes this is a the windows flare with fat suppression and again you can see in these posterior occipital lobes flare abnormality signal abnormality or hyperintensities involving those posts or bilateral occipital lobes and then a couple of kind of specialty windows neuro radiology is always really great to go and talk with so I talked with them about this and they were very illuminating on some of these things so on a GRE hypointensity on the GRE indicates hemorrhage which he has just a little bit of right here and we'll talk about why this is important in a little bit and then on a DWI and an ADC scan especially using those two things together restriction or hyperintensity on DWI that correlates with hypointensity on ADC indicates ischemia so he kind of has all he has everything in the posterior occipital lobes the additional imaging that gets down with the brain attack was normal and specifically the MRI with orbits with and without there was no optic nerve enhancement oh sorry so this is most consistent with diagnosis of press or posterior reversible encephalopathy syndrome he was able the next day to transport over to the retina clinic and get an FA which was normal we talked about this it kind of looks a little blurry out here it's just mostly because of the it just wasn't it the clear picture but it was normal normal arm to retina time and it was otherwise unremarkable on both eyes his Mac OCT he had a longer myopic eye but otherwise the OCT was normal and then here's kind of the fuller picture like I said I cheated a little I kind of shrunk things down this is obviously an optos picture but this is the full picture and I think this does a better job of showing that you know this this really fits more with just some kind of pigmentary abnormalities what we see in things like white without pressure do you think you typically think of in the periphery of the retina but in this case it just happened to be in the posterior pole okay so a little bit about posterior reversible encephalopathy syndrome I don't think this is something that we encounter a lot in fact I know it's not because there's it's just not encountered very a lot very much in the literature so it's a clinical radiological syndrome the symptoms that you can get are headache seizures altered mental status and vision loss and then you have to correlate it with radiologic findings of white matter vasogenic edema which predominantly affects the posterior parietal or occipital lobes it was first described in 96 but anything in the literature are very short case series or case reports so it really isn't all that common age ranges from two to 90 years of age in the literature it's been known by other names but this is kind of the widely accepted name at this point is press and then it's being increasingly diagnosed but the thoughts are at least in most of the literature that I found that this is probably due just to the availability of imaging and it's a relatively newly described disease as well her condition as well not all symptoms are present and then they can vary in their presentation widely so vision can be mildly blurry to in this case bilateral no light perception vision it's most often so it's related to several conditions and that list is growing but it's most often by far most often related to hypertension but here's kind of a list of other things that maybe you can think of as if this is a condition that you're trying to decide is this gonna be on my differential or not importantly these other conditions it can be with or without hypertension so there's actually recently there's a lot of well not a lot but there are several case reports of people who have recently started cancer chemotherapy or bone marrow transplantation who who don't present with hypertension but they actually end up developing press and the mechanisms are unclear why I and then I just added a little tidbit I think this came up a lot this is a really important one to think of in pregnant women who present with vision loss so maybe a little note for us as residents the path of physiology really is unknown there's kind of three main hypotheses one is cerebral vasoconstriction which causes infarcts in the brain leading to cytotoxic edema or cellular damage the second one is failure of cerebral vasculature auto regulation which leads to vasogenic edema and then the third one is endothelial damage with blood brain barrier disruption which then also leads to vasogenic edema kind of a I thought this was a good cartoon rendition of kind of the difference between cytotoxic and vasogenic edema so vasogenic edema is actually what it sounds like to me so there's disruption of the tight junctions of endothelial cells which just leads to leakage of plasma and fluid into the brain causing edema into the perencoma of the brain whereas cytotoxic edema is an initial insult or injury to the cells which then have that they're sodium potassium pumps stop working for example then sodium builds up inside the cells which drives a water gradient into the cells the cells swell but then that leaves the brain perencoma with also a high ionic gradient which then draws fluid from out of the vasculature into the perencoma so you get you kind of get the same endpoint but you have this extra bit of cytotoxic edema in the middle now what why is this necessarily important so I actually found this really interesting with neuro radiology they said you can see this depending on the windows you use in the scans so vasogenic edema is easily seen with a t2 flare with flare abnormalities or hyper intensities and that's that indicates vasogenic edema whereas this kind of DWI diffusion restriction it makes sense because it's water restriction so if you have edema that's caught inside of a cell it's not being able to move that's why you get this flare or the signal abnormality so that's indicative of cytotoxic edema so the diagnosis we we kind of already talked about I but in this slide I just wanted to point out that really these symptoms are pretty variable so headache was present in maybe about half of patients in cephalopathy half to all of the patients again depending on the case series and then seizure was was quite prevalent so our patient never did show any signs of seizure he never to get an EEG but he never had any clinical signs of seizure and then vision loss anywhere from about 40 to you know three four of the time yeah and then imaging just to remind everyone it's the vasogenic edema oh sorry that so in the radiologist report on this they kept on referring to it as atypical or complicated press which I didn't come across much in the literature but they said that there's some radiological literature that says if there's any hemorrhage involved or any ischemia involved in this kind of bumps up the diagnosis from just run-of-the-mill press to complicated press which kind of has prognostic implications yeah yes actually it's a really good point is one of my discussion questions is this is a little bit weird that you know our patient has been in the hospital for five days and then I mean his blood pressure had been training out nicely there were maybe a couple of hypotensive episodes but if this was press all long press typically doesn't happen except for in the acute hypotensive phase so the radiologist absolutely did say hey this this does look like it could be some watershed sewn infarcts and maybe the press was there all along although they they said especially the attending that I talked to she said that it was really difficult for her to believe that the small amount of infarct that they saw was able to account for his no light perception in both eyes so I don't know that we're gonna be able to tell for sure but probably a mixture so the treatment is just treating the underlying condition most often that's hypertension but like I said sometimes it can be withdrawal of the offending agent in the case of chemotherapy and this can be quite significant in someone who's undergoing treatment for cancer and then has a press reaction I mean that's a difficult decision to make because even though in the name it says this is reversible it's it's not always especially if it's complicated or atypical press in fact studies have shown that up to 15% mortality range or permanent neurologic deficit especially in condition in cases of atypical or complicated press so back to our patient so he continued to have his blood pressure control pretty aggressively especially once we kind of ruled out now this was not a rental artery occlusion you know we kind of lacks the the restrictions on his permissive hypertension his inpatient workup for the cause was really unrevealing and he kind of went through the ringer so we still really don't know what's causing his hypertension at time of discharge his vision was still only count fingers at six feet but I mean he was more most recently seen in September he missed a couple of appointments unfortunately with neuro ophthalmology but he was seen by Dr. Christensen and with a excellent refraction he was 2020 in bullfies his visual field by confrontation were full but he has not had an automated visual field done that's it so we well I'll just kind of go ahead and end I'd love to hear thoughts on kind of this misdiagnosis of an RAO especially if anybody had thoughts on anything they do differently or you know was this brain attack warranted or not but we'll maybe save that till after just so we can go through and then we already talked about this first one that's a great question by Dr. Petty so that's it