 Hi, we'll get started here. So I've changed this lecture from previous years based on resident feedback on making things more interactive. So I'll be covering less content, but it'll be more fun. That's all I'll do. OK, so these are cases that I've encountered over the past probably a few years here. So this is the first one. This is a five-year-old referred from Dr. Hoffman for a Salzman nodule on the left eye. And the mom has been noticing it growing over the past year. And it's got a pretty extensive past ocular history. He is aphagic in both eyes at congenital cataracts. He's also has glaucoma. He's on latinoprost and cosopt. He has esotropia, and he has nystagmus. So there's his prescription. So he's aphagic, so he's very hyperopic. He is developmentally delayed, so we can only get a fix and follow on vision. He does have nystagmus. We're not able to measure IOP in the clinic. So there's his exams, right eye. Pretty normal in the anterior segment, except he is aphagic left eye. There is a kind of a paracentral Salzman nodule inferiorly, measuring about 2 by 1 millimeter. Also aphagic there. So what is a Salzman nodule? So Salzman nodules are quite common. They are slowly progressive gray-white nodules located anterior to the epithelial basement membrane. And they're commonly bilateral. And here is an example of some rather large ones. Usually you don't get them quite so large. It's pretty common to see much smaller ones. And they can occur with chronic inflammation, including ocular surface disease. Sometimes we'll actually see them around lasik flaps. And they can also be idiopathic. So the plan was, because these five not very cooperative, we're going to plan a UA and a superficial caretectomy of this left eye. However, during the superficial caretectomy, the nodule did not peel off the epithelial basement membrane easily. So normally with Salzman nodules, you just get underneath the plane, or you find a plane underneath the Salzman nodule. And you can actually just take a 0.12 and just peel it off epithelial basement membrane. However, in this case, this didn't peel because the nodule actually penetrated into the struma. So what we decided to do was shave off and to your superficial portion. There was a deeper section left behind. So it did look better afterwards. But there still was a deeper posterior component that was left behind, a place to bandage contact lens on. What's the diagnosis? And Austin can't say anything, because I think I've mentioned this case to you. Or have you forgotten you can say something. But what do you think the diagnosis is now? So I've already kind of said it's not a Salzman nodule because it didn't peel off. So any suggestions? Dermoid? Oh, what? Dermoid? Dermoid? OK, that's a good thought. But no, it's not Dermoid. Any other thoughts? Let's kind of maybe way, way more common thing that you'll see in the cornea. I mean, you can see someone like this with chronic inflammation. You can see like band carotopathy, but that's pretty. Yeah, that's a thought. But band carotopathy will be, I didn't show a picture of this kid, but it'll be super, super, super white. It'll be calcific. And that also usually peels off of the epithelial basement membrane, or at least will come off quite easily. So good thought, but it's not that. Could be a corneal scar, right? Maybe there was some kind of injury that we didn't know about, or some kind of infection that we didn't know about, and it healed into a scar. So that's a thought. But actually, this was a corneal keloid. So corneal keloids are much rarer than cutaneous keloids. And it can look a little bit like a Salzman nodule. So it's also gray white. It's an epicorneal lesion that results from abnormal proliferation of fibrous tissue. And there's hyperplasia of corneal epithelium, and there's disruption of Bowman's layer, so it does go deeper. Can develop after ocular trauma, but it can be idiopathic. And if it occurs after a trauma, the keloid actually extends beyond the initial wound and develops months to years after the trauma. So it's different than a normal scar. So normally, when we see any sort of scar that happens after any sort of injury, it just scars right in the area of injury. You don't really see it extending beyond. And you don't really see it growing typically far after the initial insult. So keloid is a bit different. They grow months to years after the initial injury and beyond the borders of the initial wound and can penetrate deeper. And bilateral cases are typically associated with congenital disorders. Now, in this patient, he's five. He's got some other issues going on. And we only saw this nodule in one eye. However, oh, I'll go into some more history later, but management of corneal keloids. So topical steroids don't work. Typically, we'll reserve surgical management for visually significant or symptomatic lesions. And there are variable results with superficial care tectomy, PTK, dog, and PK. And recurrences have been reported after partial excision, even after, even when it's paired with an amniotic membrane or with a mitomycin C. So we've got some more family history, or I got some more family history afterwards. So the patient's older brothers have all gone blind from corneal keloids bilaterally. So now, what are you thinking? Is it diagnosis? So we've got corneal keloids. We've got a young kid. We've got glaucoma, congenital cataracts. Think of anything that might cause this. It's familial. Any suggestions? This is low syndrome. So low syndrome is also known as oculocerebrorenal dystrophy. It is X-linked recessive. Pervalence is quite rare, one in 500,000. Systemic manifestations, mental retardation, hypotonia, kidney dysfunction, ocular manifestations, heart congenital cataracts, corneal keloids, and infantile glaucoma. And the patient does have, I don't know about the hypotonia and kidney dysfunction, but he has pretty much everything else. And that, I think, is it for that case. He had never been diagnosed with that before. No, no, I just didn't do the deep dive of the chart, because I looked at the last note from Dr. Hoffman, and it said, these things didn't have the low syndrome, didn't have family history. It was just like, salt and nodules. I was like, okay. This is another reason maybe not to go off of what page, just kind of, you kind of have to take a deep dive of the chart yourself. So I just, once I went back a few notes, then the low syndrome diagnosis did come out. You would do the same thing. I don't think I would have done the same thing, because with this case, I don't know what's gonna turn out later, but sometimes when you take off a keloid partially, it can grow back, it can kind of grow more. And since this patient was fairly asymptomatic, this was not in his line of visual access, he wasn't having pain or irritation with it, it probably would have left it alone. Although it's kind of one of those things where maybe it doesn't matter what you do, because if it's gonna grow anyway, do you wait until it's big, and then try something versus doing something early and have it grow big later? I don't know. So I just did the surgery in the spring, so I haven't seen him back since Dr. Hoffman's been following him. I think last I heard like a month after surgery, he was fine, so I don't know. Time will tell. Okay, so that was that. Any other questions in that first case? Okay, next we have a pop quiz of one question. So which of the following medications can, this is a totally different topic, which of the following medications can cause corneal edema? A, amantadine, B, amiodarone, C, fuconide, and that's supposed to be D, phenothiazine. We can take a poll, show of hands. Once you guys have in your mind maybe thought of the answer you have put down. This is O-caps, or boards. You have to pick an answer. Pick an answer. You can guess. How many people say A? Anyone for B? I think you guys got it right. Okay, so amantadine is typically used to, and I'll have a case after this, but amantadine is typically used to treat Parkinson's. Diskinesia has also been used in Alzheimer's and other conditions can lead to corneal edema because of a loss of endothelial cells. And the edema can actually begin a few months or even several years after the initiation of therapy. Cessation of the drug can lead to a resolution of the edema, but the edema can be irreversible. Amiodarone is one that we see a lot of causing corneal versus a lot of. So it doesn't cause corneal edema. Flecanide was just a random drug that I threw in there. So it's a sodium channel blocker for treating orythmias and does not cause corneal changes. I kind of put it in because it was maybe similar to amiodarone as far as its cardiac effect. Phenothiazine kind of pops up on O-caps and that's an antipsychotic that can cause stellate anterior subcapsular cataracts. It does not cause corneal edema. But this is a case, the inspiration for that question was this case I had several years or maybe four years ago. So this is a 16-year-old kid who was brought in by his care facility for blurred vision in both eyes for one month. And he's in the care facility because he has very severe seizure disorder. His medications did include amantadine which just started maybe a month ago. It has no oculate history. His visual acuity was about 2,400 in both eyes. He didn't wear glasses. He said he had good vision prior to the starting. And on exam, I don't have a picture, but he had bilateral bad 3 plus folds in corneal edema. So I recommended immediately stopping the amantadine. He came back a month later. Edema was almost gone a month after that. It was completely gone. His visual was back to 2020. So important to look at the medication list. And I guess it could be confusing if you have a much older patient. So this one was a little easier in that you don't typically see corneal edema in young kids. It's gotta be from something else. But if you've got someone who's a lot older who may be pseudophagic, you could be thrown off and you could easily call this pseudophagic post-scarotopathy or maybe there's some underlying food dystrophy. But if you look at the meds for amantadine. Okay, next couple cases are a little bit different. So I'm medical director of our HL Lions EyeBank. And so I'll get consults from our EyeBank staff regarding eyes that they have procured, that they have questions on wondering, hey, is this eye of this cornea? Does this meet criteria for transplantation? Or does it not? And they sometimes see things that they're not sure what they're seeing because they're not physicians or ophthalmologists. So here are a couple interesting cases here. So this is called use it or lose it. So you got a cornea, do you use it or do you lose it? This case is a 69 year old male cause of death was a head injury from a intracranial hemorrhage, has a medical history of hypertension arrhythmia and basal cell carcinoma. He's had cataract surgery in both eyes. His cell counts are decent 2200, 2300. And on the epithelium there was some mild, they noted mild to moderate haze and the EyeBank technicians were wondering, hey, what is this haze? Don't know what it is. I don't have a picture, but this is what it looked like if it was a real patient. So what is this? What is it? Is this stenosis? No. Is it just a lot of? Yeah, cornea is just a lot of. So in the epithelium, so you see this in patients who are pretty much on amiodarone. So then I asked for some more history, is this patient on amiodarone? They kind of took back and yes, medications did include amiodarone. So do you use this or do you lose it? So knowing that it's corneal reticillata, and knowing that it is from amiodarone, do you think you would use it or lose it? Reason? Is it visually significant? Okay. What if it was visually significant? Would you still use it? Not that we would know that because patients don't disease. If you're not on amiodarone. Yeah, so it does go away. So corneal reticillata are deposits at the level of the basal epithelium in the interpalpebral space. It's seen with prolonged use of amiodarone that's by far the most common reason. You can also see this with Fabry's disease which is an excellent isosomal storage disorder which is a deficiency of alpha-galactosidase. Another fun fact for OCAPS. And corneal reticillata, they pretty much always resolve with discontinuation of the amiodarone. So yes, we would use it. Next case, this is a 32 year old male, found unresponsive. He also had a seizure disorder. There's no past ocular history. He has Wilson's disease. Depression, GERD, really great cell count. They're almost 3,500. There's a clear zone of eight millimeters. And again, the iBank technicians were saying, hey, we see something on the cornea. We don't know what it is. What is this? And I don't have a picture, but it looks like this. So what is this? Okay, so let's give me. So Wilson's disease is autosomal recessive from a decreased production of ceruloplasmin or a copper transport protein. This leads to an accumulation of copper in the liver and other organs and leads to systemic toxicity. Usually comes out in the first two decades of life and 95% of patients have a Kaiser Fleischer ring. Due to copper deposition at the level of decimase membrane in the peripheral cornea. You can also have sunflower cataracts. I'll show you a picture and treat this with copper chelating agents such as penicillamine. So there's a sunflower cataract here. This is a picture of a Kaiser Fleischer ring that is, let me get the mouse going here. So this is a more subtle Kaiser Fleischer ring. So you see a little brown deposition at decimase, but the overlying stroma is pretty clear and the similar thing is seen up here. So it may not be as obvious as this. So for all those consults on, you get as inpatients for rolling out Wilson's disease, do an eye exam, it can be pretty subtle. And I have read that ideally you wanna do agonio to really take a look at decimase membrane. I know it's hard to do agonio at the bedside, but this is why I kind of like if we could have a portable slip lamp for inpatient use that works. I mean, looking at something like this is gonna be pretty impossible with just, well, it'll be pretty tough with using a 20 diopter. Okay, so use it or lose it? I said use it, but there is a small clear zone. I mean, I think this does clear or it can clear with when there's no longer Wilson's disease present, but you're starting off with a small clear zone of eight millimeters and normally with a PK, I'll go a little bit beyond that. So it's not in the visual axis. No, but you know, surgeons might be put off by having a brown ring on the edge of their transplant, but technically it's usable. Okay, next we're switching back to actual patients who are living. This is a nine-year-old who presented with a growth on her left eye since birth. Mom has not noticed much growth over the past several years. The left eye has had poor vision since birth. Past medical history of golden heart syndrome. Oculohistory, v-pattern esotropia, had muscle surgery at age four, amblyopia in the left eye. There's also something else going on in the left eye, which is this growth. What do you think the growth is? Yes, this is the dermoid. There's her vision. So her right eye's good. 2015, left eye is 2,300. There's her refraction. Right eye is totally normal. And her left eye has about a four-by-four millimeter limbo-dermoid and growth temporally that contains hair follicles. So golden heart syndrome is also known as oculo-ariculo-vertibural syndrome. There's an anomalous development of the first and second brachial arches. There is an unknown cause. It can be genetic in some cases. And it's associated with limbo-dermoids. Also, pre-aricular skin tags, strabismus, scoliosis, and hearing loss. The incidence is about 1 in 3,500 to 1 in 26,000. The male to female ratio is three to two. Here's some pictures of the characteristic pre-aricular skin tags you'll see with golden heart syndrome. And a pretty characteristic limbo-dermoid. This one's a lot bigger than the one I was looking at, but I do have a topography which kind of shows this patient's limbo-dermoid right here. So looking at this topography, what do you think the topographic, I guess, sequelae of this limbo-dermoid is? Like what is it doing to the cornea topographically speaking? Yeah, so this is kind of artifact that corresponds to the location of this limbo-dermoid. And you see all this blue all around it, and 90 degrees away you see very regular, highest dignitism of six diopters. So it's actually causing, it's kind of pushing down right here, flattening it, and that results in steepening 90 degrees away. So it's kind of cool. Nice topography there. Okay, so I had a video of the removal of this and hopefully my mouse, the mouse was working earlier. Oh, I see, oh, this is a sped up video. But I just used a press of play to remove it, really sped up now, get underneath. And it actually comes off pretty nicely underneath. There's a little bit of a crater left over, which you'll see, sorry. And you could leave it there, but I decided to do a corneal patch graph on top. So I measured it off, I think it was about four millimeters, punched out four millimeter punch of cornea. But cornea's pretty thick, I wanted to thin it down. So I took a press of play to thin out the thickness of it. Yeah, you could just close conned up the limbas, and that wouldn't be wrong either. If I could just, yeah, I kind of just would epithelialize over, there'll be kind of a divot there. Some people will put amniotic membrane on top, you could do that too. I just wanted to keep the curvature kind of very similar, so that's why I put in a patch graph. And she, yeah, she did well. She, I see, it's been maybe three years out from surgery and she's pretty good. Her vision didn't really get better, but cosmetically she's very happy with it. Okay, this is my last case, and this is actually a case that I saw a few weeks ago, so Theresa and Austin might know this one. So anyway, don't participate in any discussion. Good participation. So this is a 16-year-old female referred from triage with bilateral corneal ulcers and panacea. She's had light sensitivity for two months and it improved in the past weeks since she was in a triage clinic and was put on maxitrol. Her vision was good, never effective. She's never worn contact lenses ever. Has mental history of ulcerative colitis and her meds include self-isolation supplements and sees a naturopathic doctor. Her vision uncorrected is 2020. Her exam lashes fine. Conjure's quiet at this point on the cornea. I have pictures from later on. I don't have pictures from this day, but her cornea has, in her right eye, super-temporally this three millimeter area of scarring and also some thinning with some associated envy, no epidephic left eye, kind of similar super-temporally there. There is more thinning, maybe 30 to 40% thinning. With some envy and an over-lying epidephect. So then I got some more history. So she said that she had some kind of raised red nodule on her congenitiva a couple months ago. She showed us a picture on her phone. And then she's also had these raised erythematous nodules on her shins for the past four months. And she was recommended to start systemic immunosuppression but her mother has refused. The patient is currently not seeing her rheumatologist. So what's the diagnosis of what's going on in her eyes? So again, there is her history, bilateral ulcer. She's got ulcerative colitis. She is better on maxi-trol and she's got these bilateral areas of thinning. There's an epidephect in one eye. So I'm not seeing her quite super acutely but there's something that was acute there. What's that? Are you a differential? So Puk also known as peripheral ulcerative keratitis. It's an inflammatory condition that's associated with autoimmune conditions. We usually see it with rheumatoid arthritis but you can get it with a lot of other autoimmune conditions. So you get a loss of stromal tissue and there may or may not be an epidephect overlying. It can be unilateral or bilateral. And progressive thinning may lead to dysmedicine and perforation. And if you get to that point, corneal glue may be necessary. Systemic immunosuppression is very much necessary. Surgical treatments such as a transplant is deferred kind of as long as possible until the patient is immunosuppressed. Just because if you just do a transplant acutely, there can be some thinning of the transplant that's put on. So you need some systemic immunosuppression there. Here are a couple of pictures. This is one very obvious one, classic one that you'll see thinning kind of along the limbus, tracking along like that. Sometimes you'll just see one little spot paracentrally where there's kind of a big divot, but it's a small area. And we still call this Puk, even though it's not peripheral, but we'll see this more often in rheumatoid melts. So you can get a big rheumatoid melt like this but this could also be a rheumatoid melt when we see it paracentrally. This patient mentioned some bumps on her legs and her shins. So this is erythema nodosum, which for those of you who are interns are probably kind of no more about this than we do or than certainly my eye. So erythema nodosum is skin inflammation with reddish raised painful bumps below the knees, causes include inflammatory bowel disease such as Crohn's and ulcerative colitis. It can occur secondary to medications, sarcoid, bishettes, also other inflammatory conditions. So clinical course for this patient, so we tapered off her maxitral drop. She came back a couple weeks later and she was doing way better. There's no more light sensitivity. Vision was still good. Her exam was better and I have pictures of this. Though the right eye, there's a very minimal thinning present. Left eye, also the thinning was very much improved. The scar area had improved and no epideffect. And this is what she looks like. It's actually pretty subtle right now. So this is her right eye. You can see this area of kind of scarring and envy and a little bit of thinning here. And the left eye got way better. It used to be kind of the same size as this. And it's just this little one millimeter area of scarring and envy there. So she looks really, really good, very much inactive. But what else should you do? So I have this patient who's 16. She's got all this going on. What do you think? She needs to be put on the systemic. So I had a long talk with the mom about that. Mom really doesn't want to be on anything. She's worried about the side effects. So I kind of have to scare them a little bit and say, look, now she's better. But if this happens again, if her, you know, Crohn's disease or her ulcerative colitis is really revved up, this could lead to, that does not see a perforation vision loss. So patient was actually more scared about that than the mom. But they gave her a name for it. She didn't want to see any rheumatologists here at the U. And I was like, okay, well, what about pediatric rheumatologists? So I gave her a name of a pediatric rheumatologist. We'll see what happens. Yeah, I documented everything. I mean, this is different. I mean, if she were actually perforating, I guess I'd have to call, I don't know, do I call DCSF? DCSF? Child Services. Child Services, yeah. Probably would have to call them, get her admitted. But because she was doing better, I don't think I can really do much more than that. Ethical issues with corneal and systemic. Of course. But that's it. Awesome.