 Good morning everybody. We're going to get started here and We really appreciate all the support faculty and staff coming for our medical students the We have a we have three students one from the University of Utah and then two away students who are Presenting this morning and I'm really grateful to have these medical students with us. I think they really do add a Unique element to just our training environment and the educational experience here at the Moran and we're really grateful to have them They've done a wonderful job so far First we'll hear from Annie Shepherd Child with no red reflex In a case of Coates disease after that you you know all the way the time we were here from Spencer Fuller Who's from UC San Diego? And he has a really interesting research case Of a video recording from the perspective of the patient and counteract surgery and then Brian Kirk from University of Utah is going to be presenting on presenting to us of the patient experience during sequential bilateral counteract surgery So so we really entering topics and I'll turn the time over to Annie. Thanks Annie Hi, I am Annie Shepherd. I am from the University of Tennessee and I will be going over a case of Coates disease this morning I have no financial disclosures So this is a case of a six-year-old There we go So this is a six-year-old male with the past medical history of sickle cell trait He was referred to Ophthalmology from a local optometrist for what mom described as decreasing vision from the right He didn't report any pain or photophobia It was a slow gradual onset and mom also noticed that his eyes look different in pictures much like this image seen here He has no past ocular history and is not currently on any medications On ocular exam. He had an absent by Red reflex on the right and a slightly diminished one on the left Visual acuity on the right was count fingers at two feet and was 20 25 on the left Vitrious showed some cell or pigment bilaterally in the anterior vitreous and dilated exam showed exudative lesions in the foveal region on the right and Squatic vessels with peripheral exudates bilaterally more so on the right than the left and secular dilation of vessels bilaterally Retinal imaging of the right seen here. There is a large exudate in the foveal region It was described on exam as being raised, but you can't really appreciate that on just a photograph there's also peripheral exudates you can see kind of outside of the margin of this photograph and Then on the left, there's also exudates no foveal involvement on this in this eye And you can see the vessels are overlaying overlying the exudate, which means it's deep either intra retinal or sub retinal Imaging was ordered on this patient an MRI was done to rule out retinoblastoma It showed no calcification, which was a good sign to rule out retinoblastoma But the image did show Bands in the orbits bilaterally Which was called either fibrous or inflammatory changes if fluorescent angiogram was also done This showed light bulb dilation of the capillaries and the peripheral retina showed areas of non perfusion and There was exudation in the posterior pole involving the macula on the right which correlated with the fundus exam This is an MRI not of this patient, but of a classic unilateral coats disease You see a smaller orbit on the right that is hyper dense with You can kind of appreciate some Hyper dense banding in the orbit and there's no extra ocular pathology scene This is a fluorescent angiogram. You see a large area of Non-profusion with these dilation of the vessels and on the bottom of the fluorescent angiogram You see a very tortuous dilated vessel and those light bulb dilation in the top So coats disease is also known as exudative retinitis. It was first Described by dr. George coats in 1908. It's most commonly seen in young males ages 6 to 8 But it has been described in patients as young as 6 months old and as old as 71 there's no racial or ethnic predilection and Usually it's unilateral But can be seen bilaterally if there's more going on than just the coats disease in a bilateral In a patient with bilateral disease it would be warranted to do a genetic workup Coats disease has been associated with this is three syndromes, but there has been multiple genetic syndromes that have been associated path all the pathophysiology behind coats disease is idiopathic vascular malformations Which creates fragile vessels with a thin endothelium? this endothelium leads to leakage of intra vascular contents and the build-up of exudates in the retina the The exudates are usually cholesterol and lipids and this leads to the formation of ghost cells They're commonly described as being yellow white in nature and are beneath the post beneath the retinal vessels like we saw on the fundus image the vascular hallmarks seen are telangiectasia aneurysmal vessel dilation and capillary micro aneurysms The normal presentation like I said was in a preschool age child That has trabismus or leukocorea They can also be caught like the patient that we went over having a decreased vision on screening a rare presentation Usually seen late in the disease is a cholesterol bull by Which is seen in this image here just a build-up of cholesterol Deposits in the eye and if a B scan is done of this you'll see a snow globe effect The vitreous becomes much more liquefied and the cholesterol seems to circle inside of the inside the globe older children and adults although rare Can present with decreased vision usually due to macular pathology or retinal detachment? Complications like I said are retinal detachment. These are exudative retinal attachments Glaucoma often neo vascular glaucoma and physicists bull by the differential is pretty broad but can include retinoblastoma persistent fetal vascular chair and Parasplanitis plus these other ones listed management of co-sys is mostly Centered around retinal detachment with 65 percent of patients developing a retinal detachment over five years Current treatment regimens include cryotherapy and photocoagulation or some combination But there have been new studies over the past 10 to 15 years looking at anti-veg F injections as possible additional treatment most patients I Have effective treatment if there is early ablation of these abnormal vascular chairs And that is because there's obviously less exudates if you catch it early and fewer pulls involved So the it knew studies on anti-veg F injections this first study they found that patients with codes disease had increased veg F levels in the vitreous and Their thought behind this was retinal detachment leads to retinal hypoxia which subsequently leads to the release of veg F and Unregulated veg F as we know leads to vascular malformations including telangiectasia microvascular occlusions and microvascular aneurysms and all of these are weak vessels and And are leaky and lead to exudate buildup So this first study looked at a two-year-old boy with code disease that was complicated by retinal detachments that were a fractory to surgery he underwent two rounds of Intervitural injections, which are eight weeks apart and he had complete reattachment and stability six months at six months follow-up and then the second study looked at three patients that had both laser ablative surgery and injections and all three patients had complete reabsorption of sub retinal fluid and They recommend that these anti-veg F injections could be a good additional therapy With the classic laser therapy as well This last study is from May of this past year and it had a larger patient population of 16 patients That were given intervitural injections They made the comment that exudations including the map in the macular area led to less vision improvement than if the macular was not involved And they also noted the patients with the more severe retinal attachment had a more robust reaction to the injections This is just a pathology slide of a total retinal attachment With sub retinal fluid you can see You can appreciate some of the cholesterol clefs and then at a higher magnification These costs become very obvious and also the appearance of ghost cells Which are which are filled with cholesterol and lipids This is a cholesterol crystal that was Obtained from sub retinal fluid in a patient with Coates disease This is it's common to find this in the sub retinal fluid, but it can also be found in the anterior chamber of these patients as well So just to wrap up the we'll go back to the patient presented at the beginning He underwent five rounds of argon laser treatment in both eyes over five months and nine months after his initial presentation his vision in the right had improved to 2,400 but like that study said he had macular involvement So he was unlikely to have dramatic vision improvement and then on the left at state stable at 2025 and you can appreciate the improvement of the exudates in both eyes and but there's still a pretty large macular involvement on that right eye I'd like to thank these individuals in particular and also everyone for listening There any questions