 To finish things up today, I'm going to talk about a case of optic neuropathy and end stage renal disease and some things that hopefully everyone, at least who's seeing patients with end stage renal disease, which is probably everybody in this room, can think about. So this was a 59 year old man who presented with painless vision loss and dyscadema in both eyes. He had a rapid decline in his vision for about six to eight weeks before he came into Dr. DeGree's clinic. His vision was suddenly very dark and blurry. He couldn't read. He couldn't make out any details and he couldn't do his daily activities. He had an MRI brain which was done about three weeks before coming in to see us and didn't show any new abnormalities. And he was seen by his local optometrist who saw a dyscadema in both eyes and said, I don't know what to do with this. Let's send him to neurophthalmology. So his past medical history is remarkable for a chronic disability, which is mostly accounted for by his mental illness. He's had multiple issues with schizoaffective disorder with bipolar features as well as obsessive compulsive disorder and others. He also has a history of end stage renal disease which arose from chronic lithium use. So the lithium helped to control his psychotic features but unfortunately at the expense of his kidneys and he actually stopped the lithium about five years before this presentation but he unfortunately had suffered long-term permanent consequences. His nephrologist actually had urged him to go on hemodialysis as we typically do with patients who are on end stage renal disease but both the patient and his family declined to continue with hemodialysis for a number of reasons, side effects and other issues. And so actually because he was end stage renal disease and not on hemodialysis he was essentially placed on hospice or given the option to be placed on hospice. He stayed on hospice for about six months and then he really didn't get any worse which was pretty surprising considering all of the things that were going on in his kidneys not working. So they actually took him off hospice just before he came into CS. He also had a history of hypertension, had a prior singulotomy which was done in attempts to cure his OCD which did not work and he also had a long history of chronic knee pain and chronic opiate use which was relevant because he had some changes in his opiate medications prior to coming in for this presentation. On review systems just the chronic knee pain as I mentioned and he had a very poor posture he was extremely kyphotic his head was kind of dropped over to the side and no concerning GCA symptoms and then you know these are his medications pretty standard. On examination his visual acuity was very poor two over 200 in both eyes his pressures were normal his pupils were equal and there was no APD seen and his visual field showed the deficits shown here he had some more in the right than the left eye superior deficits as well as other peripheral deficits really the only quadrant where he could kind of make out some some site was in his lower right quadrant although that was also kind of inconsistent and his extracurricular movements were normal there was no nice stagmus on his slit lamp examination he had dermatoclasis he also had cataracts bilaterally and then the important feature here was the grade three optic discodema which was also accompanied by pallor no-disc heme appeared chronic in nature and obviously with that poor vision he was not able to do much in the way of color vision testing or stereopsis and his neuro exam just showed some decreased strength but otherwise essentially normal neuro exam so i wanted to focus on his optic disc photos here where i'm not sure if it projects very well but you can see there's definitely you know some discodema in both eyes with pallor more pallor in the left eye and then you can also see this uh high watermark line which really tells us that this is chronic and has been there for some time you know probably for at least the six weeks if not you know significant time before that and then his goldmine visual fields were very challenging to uh complete because of his central scatoma and he kept losing fixation but essentially showed altitudinal defects in both eyes and again his oct rnfl just confirms the chronic disc swelling which was observed so our differential diagnosis um i actually put the the thing that we were kind of thinking about the most on top here which is this uremic optic neuropathy which i'll talk a little bit more about it's actually a very rare um cause so we also considered other more common things such as elevated icp which we felt was probably less likely in this case given that his MRI was normal he also had a uh second MRI which included the orbits which again didn't show anything besides a little bit of increased fluid but nothing to suggest markedly increased intracranial pressure we also considered um ai on which again would be more typical to present as a unilateral and then could be sequential bilateral but not typically both eyes at the same time and then an infiltrative cause which again would likely have been detected on MRI and then um uh also because of his kyphosis and he couldn't support his head we were also thinking about things like myasthenia and nodding head syndrome but really all signs tended to point towards this uremic optic neuropathy so i wanted to talk a little bit about ocular complications and end stage renal disease because it's probably not something that we think about all the time but uh something that certainly can come up so uh the anterior segment or surface problems are the most common things that we see and patients come in with red scratchy eyes they've got foreign body sensation they're you know kind of in your typical dry eye patient category and they can also present with band keratopathy and all of these effects are thought to be related to alterations in calcium and phosphate metabolism that happens when you have end stage renal disease and your kidneys are not able to process those uh those materials normally um and then we also have some other effects which are kind of variable so um intraocular pressure can actually increase decrease or might not change at all and so it's kind of variable in the literature as far as what happens with iop um there's also been rare reports of retinal detachments associated with ESRD and this is thought to be maybe due to fluid shift and would probably represent an exudative retinal detachment and then lastly optic neuropathy is um the most likely cause in this case so uremic optic neuropathy was actually first reported in the 1880s uh there was a um a 10 year old girl who came in with a post streptococcal glomerulonephritis who also had non-reactive pupils dyskidema and loss of vision and uh her physician astutely noted that uh when her renal function recovered um her vision recovered as well and so um I don't think at the time he coined this uremic optic neuropathy um and the article was not in English so I couldn't read it but um that was you know kind of what he was getting at um and then uh there's actually really even since the you know since that time not been much in the way of the literature there's been about maybe a handful of case reports and then this case report here this uh by winklemeyer this one actually summarized all of the previous case reports that they could find which were about 14 cases and uh talked about kind of the differences um between those cases and they actually proposed a classification system so in terms of classifying this uremic optic neuropathy um there's the first category which is the uremic optic neuropathy itself meaning there's no other uh other cause or other um etiology that could be inferred and then we also have um uremic we also have optic neuropathy associated with ischemia which could be associated in cases of uremia and then we can also have problems due to adverse drug effects elevated ICP and uh cerebral infarction and this table uh from winklemeyer's article just kind of summarizes those classifications and the types of diagnostic clues that you would look for to lead you in one direction or another and then also the suggested treatment which typically amounts to dialysis and corticosteroids if an infectious etiology can be ruled out again from this article summarizing those 14 cases one of which they had presented in the article the majority of cases did undergo dialysis here and uh about half of them also underwent corticosteroid treatment and about half had some reversibility of their vision loss which brings home the point that this should be recognized early and should be treated appropriately if it is suspected so when do we suspect this any patient that has either known uremia or previously diagnosed uremia who's presenting with vision loss or pupillary changes or both and again the treatment as I mentioned dialysis and then also a corticosteroid oral corticosteroid taper is recommended but we really don't know enough about this condition to say that this is the you know be all end all of how to treat it so if we return back to our patient for just a minute we of course recommended dialysis in our patient but again as he was adverse to dialysis before finding out that his vision was lost we thought he might change his mind but he was not not interested uh we actually referred him back to his nephrologist to talk about some other options but uh couldn't really come up with much there so we said okay next step is to try some corticosteroids but again he's got psychiatric disease that's been well controlled on a number of psychiatric medications we knew that there is a potential to worsen that um so we had a discussion with both his brother who is a psychiatrist here at the university and his um his psychiatrist and came up with a way to increase his psych medications and try to get him the steroid taper so he did undergo steroids for a short period of time but he had no change in his vision unfortunately and he ultimately succumbed to complications of his urenia at two months so that is my case and I'll be happy to take any questions