 So we're going to turn over to Samaya, the national star who comes from Texas Tech. We've had a handful of students rotate with us for that University subcontractor too. Thank you Okay Thank you. Dr. Jardine. Yes. My name is Sam Air. I'm from Texas Tech as he said and I'm very excited to be here today Thank you all so much for this opportunity and coming to listen to my presentation I'm going to give a case presentation and a brief discussion on atropian uvia and secondary glaucoma So this is a patient of Dr. Young seen at the Riverton clinic She was a 10 year old female referred by her PCP for ptosis in her right eye in January of 2017 Mom had some additional concerns that her right pupil looks slightly larger than the left and was a little off-centered on Examination her visual acuity was appropriate for her age eyes were well aligned she did have mild ptosis in the right eye and Some anisocorrhea with the right pupil being slightly larger, but they both responded equally and appropriately to light and Of note at this initial visit was that her pressures were great and her cup to disc ratio is normal so this is some photos of her eyes you can see in her The mild ptosis in the right eye and that the right pupil is slightly larger than the left on this close-up image of her Right pupil you can see that there's some irregularity in the shape of the margin And so she was subsequently diagnosed at that time with atropian uvia and was closely followed up her next two follow-up visits Were exactly the same nothing had changed However in August of 2018 She did have elevated IOP in the right eye and her cup to disc ratio is now 0.6 Six months prior it had been 0.2 So it had changed quite significantly in a short amount of time and she had no symptoms No visual changes that she was able to note and this was when she was about two and a half years old Sorry, and so she was started on dorsolomide and simulol and all imaging done at that time was normal And she came back to the clinic one week later and her pressure was back down to 10 Her next follow-up no changes However in January of 2019 when she was about three years old The pressure had gone back up into the 30s in that right eye And so the tonic rose was added to her medical management and dr. Chio was consulted for an EUA on EUA confirmed that there was an elevated IOP and a cup to disc ratio But importantly her anterior segment was open and there was no signs of any obstruction on gonioscopy a week later She underwent a nasal goniotomy in that right eye, which was about six months ago from today and her pressures have been great ever since So what is a tropion uvia? This is a condition where the posterior iris pigment epithelium is pushed anteriorly onto the surface of the iris And these are some pictures here on the right that you could see and that's what causes this irregular shape To the margin so this can be congenital or acquired There's a couple different theories about what causes the congenital variant The most common theory that I've read in the literature was some type of Arrestment in the neural crest cell migration or development that Causes this hyperplasia another theory that's been postulated is that there's some type of primordial Endothelium in the anterior segment that is not fully regressed as it should and that induces the hyperplasia of the epithelium And causes it to move anteriorly This is rare. I tried to find some epidemiological data on exact numbers I unfortunately could not but every article said that it was rare and It's typically a unilateral finding. However, this can be bilateral as well. Most importantly, it's non-progressive It shouldn't change over time The acquired version is going to be as a result any ischemic or inflammatory process in the area that causes a membrane that then Has traction on the pigment epithelium and pulls it anteriorly And that's most commonly seen in neovascular glaucoma and neovascularization of the iris The congenital ectropion uvia can be an isolated finding. However, it's greatly associated with glaucoma Which I'll talk about in a minute and also in the literature I saw it's associated with ptosis with good levator function, which I thought was very interesting because that's what our patient had as well And the theory about that potentially is that mules muscles of neural crest cell Origin and so it might nicely play into that theory It is seen in a few systemic disorders the most common being neurofibromatosis type one and so Talking about glaucoma specifically secondary to ectropion uvia an article that was published in July of 2018 Did a literature review on the topic and found about 50 cases in the literature about glaucoma specifically secondary to ectropion uvia And through their review found that patients with this condition have 80 to 90 percent Chance of developing glaucoma at some point in their life. It's typically in childhood But it was reported as early as seven months or well into the 40s So it's something to be very Aware of patients with neurofibromatosis type one would tend to have an angle closure glaucoma while patients without that Disorder tend to have an open-angle glaucoma, but I did find one article that Had angle closure glaucoma that did not have any signs of neurofibromatosis The variation in the age presentation likely has to do with the degree of arrest of those neural crest cells or Some type of anatomical change in the trabecular mesh work or the segment that predisposes these patients I included these pictures because I'm a very visual learner and just wanted to understand the anatomy as Well as I can I like to hear yet so Patients with ectropion uvia technically typically have an anterior insertion of their iris It's usually at level B when you're looking at the Schaefer lines And that's at the level of the trabecular mesh work Some port catered. I'm sorry reported as anterior as a which is Schwabby's line So looking at this you can see how if this is more anterior how it would predispose these patients to Developing glaucoma in their life. So there is no consensus as to what is the best treatment for this condition Surgery is ultimately needed in every case to control their pressures Interestingly a few articles I read said that goniotomy and trabeculotomy are not may not be as effective for long-term control Which is interesting because of how effective it is in primary congenital glaucoma Our patient did receive a goniotomy and she's been doing fantastically. So I think this brings up two important cases There are two important points that number one We should monitor her closely for the rest of her life just in case her pressures do go back up And we need to appropriately treat her at that time and number two if her pressures do remain stable after the goniotomy Why is that what's different about her case? What's different about her anatomy and what can we learn more about this rare condition and how to treat it? So it's very exciting for the future According to the literature as of right now It seems that trabeculotomy with mitomycin C is the most successful procedure at controlling these pressures long term So in conclusion Congen or ectropionuria can be congenital or acquired so you can see it typically any age presentation and it's something to be aware of with due to the high association with glaucoma and These patients should be regularly followed as often as they can And I think our patient was very blessed to have Dr. Young as her Physician treating her because we were able to catch her elevated pressure And treat her when and provide intervention before she had any visual symptoms As of right now goniotomy may not be read the best long-term control, but we still have so much to learn about this topic and Could potentially contribute to the literature in the future and I wanted to date Thank Dr. Young and Dr. Chia for allowing me to talk about this patient