 Let's go ahead and have a seat. We're going to get started. So I want to thank everyone for coming to our Residence Day. Dr. Olson usually is here to give a very eloquent welcome. And instead, you're stuck with me. I'm Bob Hoffman for those that you don't know. I'm one of the pediatric ophthalmologists here. Dr. Crandall and I are the co-chairs of this meeting. Actually, Elaine does all the work. We just get credit for it. Dr. Olson did ask me to extend a welcome to everyone. We're grateful that you've come to support this day. The major focus of the day is for the Residence to present their research. And the other thing that we have here is we have our distinguished alumni, Dr. Dollywall. And she will be both providing insight, hopefully, into the Grand Rounds patients, which we're going to start with. And then she'll be more formally introduced later before her address. I understand she gave a wonderful talk last night. My apologies for not being there. And I need somebody responsible for each of the Grand Round patients to come up and present them very briefly. Then we're going to give her a chance to comment. And then we can get other comments as time allows. And before I forget, I also want to thank all of the vendors who came to help support this meeting in terms of providing breakfast breaks. And there is CME available. Make sure you sign in at the desk. So who's? All right. Dr. Jorgensen. All right. I'm Adam Jorgensen, one of the chief residents. I'm going to present our first case from this morning. So our patient this morning was a 56-year-old female. Works as a realtor. And overall, pretty healthy. Has asthma and seasonal allergies, some allergic conjunctivitis occasionally, but other than that, no prior eye surgeries. Has a mom with macular degeneration. And her story started back in 2006 when she presented for a refractive surgery screening. And at that screening, she had great, corrected visual acuity and normal intraocular pressure. But she was noted to have an iris lesion and a little bit abnormal pupil shape. And so she was referred for an ocular ultrasound, which showed an iris cyst with the dimensions there. And then just kind of a follow-up ultrasound was done a year and a half later that showed it was basically unchanged. And also, there were multiple smaller adjacent iris cysts. She, over the course of several years, had multiple serial ultrasounds every six months or so that really showed no change in the size of that large iris cyst. In the meantime, though, she was seen in September 2008 for worsening vision. And she was found to have cystoid macular edema in that right eye. And so she was referred to the UVIDA service where she got a pretty extensive lab work up and chest x-ray, which was all normal. And she was thought to likely have an old branch retinal vein occlusion. Actually, by the time she had been seen in the UVIDA's clinic, she had been treated with NSAIDs. And her edema had resolved. Her vision was back to 2020. So she was followed in the UVIDA's clinic every now and then to make sure the edema wasn't coming back. And then in 2013, she came back saying that her vision was worse in the right eye. And there was this abnormal corneal appearance. And that had been noted before that she kind of had this beaten, metal corneal appearance. So she was worked up at that point with a pentacam. It showed some asymmetric pachymetry, specular microscopy, which showed decreased endothelial cell density in the right eye. And she was evaluated gonioscopically which showed pretty significant peripheral anterior synechia in the right eye, but the left eye was wide open. This picture, I apologize, didn't come across very well, but you can kind of see the general morphology of the cornea. And some slit lamp photos. This one you can see this demonstrates the corectopia. And inferiorly, you can appreciate that the angle is more shallow. And that's where that iris cyst is. There's another picture if you look temporally there. There's my pointer here. So there's kind of the larger iris cyst, which is a little bit difficult to appreciate. It was deeper and really seen just on the ultrasound. But then there were these other smaller iris cysts, corectopia and ectropion UVA. Kind of looking at the cornea, it's hard to appreciate blown up like this, but you could see this kind of distinct endothelial appearance, the beaten metal look. And then there's a slit lamp photo through the cornea where you can see some kind of speckles of some stromal haze and scarring. So at this point, residents, any ideas of what we're looking at in this unilateral process, at least maybe some differential considerations? Good, good. That's a great assessment. And how about specific types of eye syndrome? Is there one that you might think about thinking about the three classic types of eye syndrome? Which one would you classify this as? Cogan Reese, also known as diffuse iris nevus syndrome. Good, so that was the presumptive diagnosis given to her at the time. She was followed by the glaucoma service at that point where her visual acuity initially and intraocular pressure were normal and stable. And for a couple of years, up until recently, there was no evidence of glaucoma. But then, September of last year, she was seen again, and her pressure in the right eye was up to 26. She had recently completed some steroids for asthma. So it was thought maybe this was a pressure response, even though it was kind of asymmetric. She will start on Latana Prost at that point. And every visit after that, her pressure was right around the 22 to 23 range. But she still had full visual fields and normal retinal nerve fiber layer OCT. Then here, two weeks ago, she was seen again, and her pressure was up again to 28 in that right eye. Gonioscopically, she was noted to have new areas of PAS superiorly. And the inferior PAS was noted to be spreading temporally. So she started on another pressure-lowering drop. There was olimide. When she was seen today this morning, her pressure was rechecked, and it was 22. Very quick review. We do think this is ice syndrome. This is where endothelial cells get replaced by abnormal migratory, more epithelial type cells. These migrate over the trabecular mesh work and contract and lead to progressive angle closure. And there's these three subtypes, of which we think this one's probably Kogan-Rezer-Iris-Nevis syndrome, where you get multiple small pedunculated nodules on the iris surface. So the points we wanted to discuss in her case. First of all, it was kind of interesting she had the cystoid macular edema. I was able to find one case report of this of CME associated just with ice syndrome. But I was wondering if our retina or UVIDA specialists have seen this before. And also, when's the right time to intervene on this patient who has contraindications to beta blockers and is essentially maxed out on her drops at this point, but doesn't have clinical glaucoma yet? When is the right time to intervene, and what's the correct intervention, what's most likely to work in a progressive case like this? So we'll turn that over for discussion. Good morning, everyone. It's a super awesome pleasure to be here. I'm thrilled, thrilled, thrilled to be back. And I'm a cornea specialist. So I'm not going to comment on the glaucoma with Alan Crandall in the room. But I do want to say that the corneal edema in this patient was not clinically significant yet. And I talked about this yesterday. But how do you know when a patient's endothelium is really pooping out? When is it time to do some intervention like a DMEC? The question that I ask, or in a patient that has fuchs and cataract, how do you know when you have to do a corneal transplant with the cataract surgery, or when can you just get away with the cataract surgery alone? I ask one very simple question. And that is, when you first wake up in the morning, is your vision more cloudy or hazy? First thing in the morning, and then gradually, does it clear with time? So if they say yes, they wake up and they have 45 minutes of cloudy hazy vision that then clears, I know their endothelium is decompensating. Then I will do a combined surgery on that patient. Or if it was a patient like this, I would start seriously thinking about at least using muroointment at night to help the endothelial cells function. So in patients that have this type of corneal decompensation, we first start with conservative therapy. We'll have them use muroointment at night. And that doesn't sting. So they put it in at night. They wake up. In the morning, they can do various things. If they use a muro drops, it should be one drop of the muro, q5 minutes times 3. That's the dosing that we use to really get the tears more hypertonic to draw out the water. Because it's salt and you want to make the tears hypertonic. So that's important in terms of just managing the patient. So they can do the muro drops. And a lot of patients, they'll do the ointment at night. They wake up. Then they'll do the drops. They might do even a series of those drops maybe every hour, just in the beginning in the morning, and get through their day. We talked about a hairdryer. And I do have a patient that loves to blow dryer hair and then her eyeball. And it's very efficient. But that can work as well. Anything to kind of deter just the corneal so they can function later in the day. So for her, if she needed then after medical therapy, then you would have to do surgical therapy. And in this case, she's fake it. She would be a really good candidate for DMACC. However, in ice syndromes patients, these graphs don't do well. I'll be honest with you. This is a really, really challenging condition. And it's generally a progressive situation. So these endothelial cells have epithelial characteristics. They're migrating. They're causing these problems, all this stuff. So it doesn't halt the progression of the disease. So these eyes generally do not do very well with transplantation. But we do our best. What are you going to do? So the procedure that I would recommend for her, when her cornea is really decompensated, would be a DMACC. Now, in terms of medical management of her glaucoma, the ideal thing would be to stay away from carbonic and hydrase inhibitors because those, that class of medication, also affects endothelial pump function. So ideally, in her, she's a little challenging because the beta blockers exacerbated her asthma. And even the Latana Pras, she has a history of CME. And I know Barbara was on that. She said, well, it's a risk-benefit ratio. You have to think about every drop you're giving this patient and how it's going to affect her either systemically or the rest of her eye. But ideally, we like to stay away from the carbonic and hydrase inhibitors for pressure control in this class of patients. So those are my two cents. Anybody else want to jump in for the glaucoma management? Sure. Thank you. Thank you so much. So obviously, this is a patient that I've been sharing in management with some of my other glaucoma colleagues. And I completely agree. It's a risk-benefit. And I hesitated on the true soft. She has a history of allergies. I was more worried about the alfagant. I could switch her over. She's been stable, visual, acuity-wise, even on the prostaglandins now for several years. I agree with you. I did not like the idea of putting her on a CAI. But I thought, OK, she's sitting at 28. At one point, I think she even creeped up into the low 30s. Any role for doing serial endothelial cell counts in her? Or would you really just use the, OK, vision blurry? And that was a conversation we had with her this morning. Since she started the carbonic and hydrase inhibitors, are you noticing any blurred vision? And she hasn't. You're all going to have to pardon my voice today. It's still a little low for my surgery the other day. So the eye syndrome is really interesting. We know the histopathology. We really don't understand the mechanism. So one of the things that's most interesting is why is it almost always women? And why is it almost always only one side? There are case reports of two side, but that's very unusual. The presumptive original reason was abnormal endothelial cells. That was the theory behind certainly the corneal aspect. But it doesn't make sense with the irisnevas. So they've been now clumped into one syndrome. And most people think that there's some one immunohistopathology that has something to do with kicking it off. And others think that these are burrowed nest cells that were present at birth and have somehow been activated by viruses or by some other mechanism. But it's an unusual one. And like you talked yesterday about RKB and the gift that keeps on given for corneal specialists, this is a gift that keeps on given for ophthalmologist or glaucoma guys. Because you rarely can get away with one. And you're basically buying time with each of the procedures until something better comes up. Certainly at this point, by the other way, the other thing is they're almost always missed for the first five or six times they get seen. Almost always not an easy diagnosis unless they show up with super classical presentations. The youngest I've ever seen was a 12-year-old. And the oldest in our original series was like 80 before the diagnosis. It was a pretty big range. So I'd like to hear is, I think, I hope somebody from retina, one of the uveitis could talk about that possible, you know, histochemical aspect of it. And I know Craig and I slightly disagree with this on management at this point. If it comes to surgical, I would do an express shunt because it keeps the, the tube and shunt have two advantages over traps. It's hard for the cells to climb into them. But the problem with the tube from my standpoint, although it's an advantage lifestyle-wise, but they almost always are never off-drops. And the corneas in these people take a beating over time. And that becomes a true problem in this area. So I, I think, and that takes up a lot of space if you do a tube. That's my only, I just, so Craig, you wanna give me your, President? Just regarding her medical management, we talked about CAIs causing corneal edema, but I've actually had maybe half a dozen cases of corneal edema from prostaglanins as well. So I think just about everything that we're gonna use on her has some sort of risk. I didn't get the lens status for her. Is she totally clear lens? She's clear. She's totally clear. So in terms of, I personally would probably put it in a tube. And I think we actually have some real estate that we forget about. And with some of our new tube shunts, we can put those inferiorly. So for cosmesis or for just more real estate, we can either go in for a nasal or super temporal. This would be kind of my first two choices for a tube shunt. And they can hide pretty well. And the tube can also be placed in the sulcus to keep it away from the cornea, although that's a little bit trickier when a patient's faking. But it can be done. So if there was risk for corneal decompensation, or if she ended up having a graft or something and we wanted to keep the tube away from the graft, we could reroute that tube. So I think that's one of the advantages if you have a tube, is that it's less likely to get clogged, but you can also reroute it if necessary. But this is a difficult case. I would probably wait until the absolute last minute. I would not put a tube initially just because she can't tolerate the medications yet. And some considerations for other things later, if she needed surgery would be ECP. That doesn't require any devices. It's an inflow procedure. And that may also help her. But I think we have to treat this as presumptive. Although it looks open right now, essentially it's gonna be angle closure. And so that would be my philosophy. Thanks. Just one thought. I don't tend to be much of a fatalist, but with this disease I completely am. I've got these eyes just over time, just do not do well. So as far as the surgical approach, I totally agree. I would wait until you absolutely felt like you had to do something. And then anything you do, you know it's gonna fail. And I think it's important to tell the patient that right up front, I mean this is gonna be the first of multiple surgeries. And so for me personally in these patients, I start with a Trab, and then I try to revise that Trab, which you and I believe will have to do. And then I do a second Trab and revise that one. And then finally go to tubes. And you know, I mean, Alan's got more years on me, but you know I've got patients like this with eye syndrome that I've been following for 25 years, you know. And I've operated on them five or six times to over that period of 25 years to just try to keep their glaucoma under control. I mean, it really is just this relentless process that you keep trying to fight back for as long as you can. Thank goodness it's almost always just in one eye. And you've got that going for you. And that's another thing that I think you need to reassure your patients about right off is to tell them that in almost all instances, this is just gonna be a one-eyed problem, which in this setting is a real blessing.