 Thank you so much, Carl. Great job. Our next presentation while she gets it pulled up is by Catherine Lewis. She was born initially in Claremont, California. Currently studying as a fourth year at UC Riverside. Interesting to know about Catherine. She studied American Sign Language in college and is relatively proficient at it at this point. I needed her help last week with a patient. And she's going to be speaking to us today on Aniridia more than meets the iris. Hi everyone, good morning. Today I will be talking about Aniridia and about the management of the condition as well. So I have no disclosures as a med student. Starting with a clinical case, we had a 35-year-old male with a past history of congenital Aniridia and Aniridic glaucoma who came to clinic for an IOP check. You can see a surgical history here. He's had multiple diode lasers to both eyes as well as goniotomy for the treatment of his glaucoma. He also has had iris diaphragm insertion bilaterally and cataract surgery as well. You can see on the left his current medications. He's on a host of different things for the control of his glaucoma. On physical exam, his visual acuity was 2400 in the right eye, 2300 in the left. His IOP was 12 in the right and then 30 in the left. Now, typically for this patient, a goal IOP would be mid to low teens. Furthermore, on exam, you can see on slit lamp, he had faint keratopathy, trace cell in the anterior chamber, as well as his iris stump had been rotated anteriorly, which we'll talk about later. He also had cupping on his fundoscopic exam and noticeable cup to disc ratio of 8 and 8.5. So I'm going to take a break for the patient here. And basically, we want to review today about Aniridia and how complex of a condition it truly is. Now, as you saw, our patient had congenital Aniridia and a very difficult glaucoma to treat. And unfortunately, for most patients with Aniridia, their case is no different in terms of the complexity. However, their clinical features may differ. So today, we'll step forward and talk about the clinical features of Aniridia as well as management. So first as an overview, Aniridia has a global prevalence of 1 in 40,000 to 1 in 100,000. And most cases are autosomal dominant. However, there are sporadic cases as well. When you have sporadic cases, you want to think about screening your patients for waggery syndrome as well. And now it's believed to be associated, and actually a lot of research has clinically associated, Aniridia with PAC-6 mutation on chromosome 11. And so on the left here, you can see a patient with Aniridia as well as a significant cataract. And then on the right, we also have another patient with unilateral Aniridia. Now, typically unilateral cases are acquired while congenital are bilateral. If we move forward, I really like this slide because it demonstrates the importance of PAC-6 in the development of not only the human eye, but other species as well. So if you see the wild type on the top, this is normal eye development. However, when you have a PAC-6 mutation, you can see that not only is the iris affected, but many parts of the eye itself. In particular, these are the different findings you can see in congenital Aniridia due to that mutation. So briefly talking about the posterior segment, you can see patients can have foveal hypoplasia, optic nerve hypoplasia as well. If we move forward, many patients develop cataracts. They also have photosensitivity and glare, which is related to the lack of an effective iris. Interestingly enough, these patients also have a limbo stem cell deficiency. And this can vary again amongst any patient that has Aniridia with the PAC-6 mutation. However, as you can imagine with that stem cell deficiency, you get a progressive chronic ulceration of the cornea, with vascularization and opacification as well. Next, we'll briefly talk about the glaucoma as well. There are two main schools of thought as to how glaucoma develops in Aniridia. The first one being that during changes during adolescence in the eye, any iris stump that is present can rotate anteriorly towards the trabecular meshwork. And when this happens, you can imagine that would cause issues with the drainage of the aqueous fluid. The other idea is that even just at birth with the congenital Aniridia, you can see changes in the anterior chamber, which could result in the glaucoma as well. And so today, we're mostly going to be talking about the anterior segment. As you can see, Aniridia is a very extensive conversation that will require much more than 10 minutes today. But I would like to touch on the keratopathy, cataracts, glaucoma. And then there are interestingly some prostheses available to patients with Aniridia, which we'll touch on, and dilemmas of care. And I included this photo because Aniridia, it truly is a balancing act when you're treating patients because treating one entity can worsen the other, and there's just a lot of complexity with it as well. So first, touching on the keratopathy. There are three main phases of the keratopathy seen in Aniridia, phase one through phase three, phase one being the least severe, phase three being the most severe. And so as you could imagine, treatment will change depending on the severity of the keratopathy present. And so even in more severe stages, patients may need amniotic membrane transplantation, corneal graft, or even in some cases, a keratoprostesis, which I'll talk about a little bit later. You can see the image on the left of the significant keratopathy, but it's not too clear there is vascularization of the cornea as well. In terms of cataract surgery, it is and can be done for patients with Aniridia. However, it is a technically complex surgery. These patients often have thin capsules and weak zonules, which makes surgery a little more difficult. And then in talking about glaucoma, as you saw our patient had a very difficult to treat glaucoma. He had diode treatments multiple times, his IOP was still high on the left. However, you can try a variety of normal treatments for patients with glaucoma. So some patients will respond to topical agents. However, it's been shown that other patients do not. And the same goes for surgery. However, in my research, I did see that some are saying there's a role of prophylactic goineatomy in these patients. And I believe the idea behind this is that if you're able to open up the trabecular mesh work before any anatomical changes or the iris stump could rotate, you may be able to lower the IOP and prevent a severe glaucoma. And next we'll briefly talk about the carotoprosthesis. This is really interesting, and I hadn't heard of it before, but in my research it's not performed everywhere and very few centers do this surgery. But as you can see, there's two plates that surround the corneal graft. And the bottom plate, or the back plate, has holes in it, which are then punctured through the cornea to allow for exchange of nutrients. And you can see the image on the left demonstrating what the implant looks like after it's been done. Now, in my research, I saw patients can do quite well with these implants. And some patients even had improved visual acuity of up to 2200, which is pretty good for aneridic patients. However, there is, of course, risk of rejection, infection, and either membrane formation in the back of the eye. And next we'll touch on aneridia rings and implants. There are many companies that make iris diaphragms that can either be cosmetic or purely functional, and Mortier is one of the companies that designs these implants for patients. They can be implanted either in the sulcus or in the capsule as well. And these are two patients from the Moran on the left. And given how their implant looks, I would suspect that it's in the 50 series, which you can... And when these are implanted, the main goal is to decrease photosensitivity and glare for patients. And they're quite effective, although difficult to capture on image. And lastly, I would like to touch on dilemmas and care of aneridia. Whenever you have a patient with aneridia, they don't simply just have cataracts, or they don't just simply have glaucoma. They often have multiple features, which can make treatment difficult. So specifically the relationship of treating glaucoma and keratopathy should be considered. Some of the drops used in glaucoma treatment can worsen keratopathy. And as well, repeated limbo manipulation and surgery could also cause issues for the keratopathy, as you would imagine, since patients have lower stem cell counts in these areas. And so whenever you're treating the glaucoma and patients have a severe keratopathy, you do of course want to weigh the risks and benefits for these patients and the best treatments available for them. And then with cataract surgery, there's always the conversation of picking right candidates for surgery. If patients have very severe foveal hypoplasia, they may not benefit too much from cataract surgery. And so it's always important to consider this panocular disease when you're treating patients, whether or not they're good candidates for surgery. Then again, if they have glaucoma, you may want to do the cataract surgery anyways to help with the IOP. And then lastly, a very rare but serious complication is known as aneridic fibrosis syndrome. And this is believed to happen that when patients have repeated ocular surgeries in aniridia, they can develop fibrosis in the anterior chamber, and that fibrosis can extend to the cornea, to the ciliary body, to the lens, and even to the retina. So as you could imagine, this could cause a lot of havoc in the eye. However, these patients may require extraction of their IOL lens if there was a dislocation. Vitrectomy may be indicated. Or even if their corneal disease has progressed so far, capro insertion. So with that, I would like to say thank you. I think aniridia is very... When I chose the topic, I didn't realize how extensive it would be. But I think today was a good reminder of the complexity and how important it is to really consider your patient as a whole when you're treating their aniridia and that, you know, weighing the risks and benefits is important and they probably will be your patient for life given the complexity of their disease. So thank you. I'm open to questions.