Objectives. Myth 1: Weiss ring = PVD. Myths 1/23/2018. To review misconceptions and clinical pearls regarding common vitreoretinal presentations.

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1 Objectives David RP Almeida MD MBA PhD VitreoRetinal Surgery, PA To review misconceptions and clinical pearls regarding common vitreoretinal presentations. Retina Update Minneapolis MN January 2018 Myths 1. Presence of a Weiss ring indicates a complete posterior vitreous detachment from the retina. 2. All retinal detachments are an emergency. 3. White dots in the posterior segment are due to inflammation. 4. There is no harm from the use of topical corticosteroids for intraocular inflammation. Myth 1: Weiss ring = PVD Usually BUT NOT ALWAYS the presence of a Weiss ring indicates a complete PVD Normally, vitreous detaches out to the retinal break(s) in acute retinal detachment, where it remains adherent and causes traction on the retinal flap 5. Viral vectors for gene therapy are not safe. Not uncommonly, triamcinolone stained residual layer of vitreous over the posterior and peripheral retina in many eyes with retinal detachment Including some with a preexisting Weiss ring 1

2 Not uncommonly, triamcinolone stained residual layer of vitreous over the posterior and peripheral retina in many eyes with retinal detachment (including some with a preexisting Weiss ring) Incomplete PVD? Vitreoschisis? Immature PVR membranes? Eric K Chin, David RP Almeida & James C Folk, Posterior Hyaloid Removal, Ophthalmic Surgery, Lasers & Imaging Retina 2015, 46(4):404 Robin K Kuriakose, Kunyong Xu, Eric K Chin MD & David RP Almeida, Proliferative Vitreoretinopathy (PVR) Update: Current Surgical Techniques and Emerging Medical Management, Journal of VitreoRetinal Diseases 2017, 1(4):261 Kunyong Xu, Eric K Chin, Steven R Bennett, David F Williams, Edwin H Ryan, Sundeep Dev, Robert A Mittra, Polly A Quiram, John B Davies, D. Wilkin Parke III, H Culver Boldt & David RP Almeida, Predictive factors for proliferative vitreoretinopathy formation after uncomplicated primary retinal detachment repair, RETINA 2018, in press Kunyong Xu, Eric K. Chin, D. Wilkin Parke III & David RP Almeida, Epiretinal Membrane and Cystoid Macular Edema as Predictive Factors of Recurrent Proliferative Vitreoretinopathy, Clinical Ophthalmology 2017, 11:1819 Incomplete vitreous separation in some eyes with RRD Partial PVDs in 71 of 786 eyes = 7% (autopsy study) Midperipheral to peripheral vitreous was detached over one large segment of the retina but remained attached in the other segments Incomplete vitreous separation in some eyes with RRD Partial PVDs in 71 of 786 eyes = 7% (autopsy study) Midperipheral to peripheral vitreous was detached over one large segment of the retina but remained attached in the other segments In RRD, posterior hyaloid usually absent from quadrant with retinal breaks In RRD, posterior hyaloid usually absent from quadrant with retinal breaks Dilute intravitreal triamcinolone allows for easy, reliable, and consistent identification of the adherent vitreous layer on the retinal surface Myth 2: All RDs are an emergency Retinal detachments are usually a surgical urgency but this does not apply to all retinal detachments all of the time. 2

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4 Is the macula attached? Macula on or macula off? Acute or chronic? Urgent or emergent? Mac on or mac off? Mac off OCT clearly shows macula is detached in multiple areas Retinal folds present (subretinal fluid) High watermark above fovea Mac 0n: VA = 20/25 OCT foveal thread is attached RD risk factors can help you triage Risk factors Age RD fellow eye (15 20%) High myopia Family history Lattice degeneration Trauma Cataract surgery (0.6% 1.7% annual risk) Nd:YAG posterior capsulotomy (Pseudophakic Retinal Detachment. Surv Ophthalmol 2003;48:467) RD after Nd:YAG Rhegmatogenous retinal detachment: Where is the break? Finding retinal break helps: Rule in rhegmatogenous process Aids triage timing (superior versus inferior) 4

5 Find (historical) breaks Dr. Harvey Lincoff Cryopexy for RD Cryopexy could destroy retinoblastoma Pioneered straight chain perfluorocarbon gases for complicated detachments Lincoff Rules (Lincoff H, Gieser R. Finding the Retinal Hole. Arch Ophthalmol. 1971;85(5):565) RD, but no break found? Chronic RD and retinal break has sealed Myope with small retinal break difficult to find on office dilated depressed exam Retinoschisis Exudative process Case Middle age male with 20/20 OS Superior visual field defect Inferior macula on retinal detachment No break identified 5

6 Case Medium sized malignant melanoma Systemic workup revealed metastatic disease Currently working with oncology team Myth 3: White dots = inflammation White dots in the posterior segment are due to inflammation (white dot syndromes) 6

7 PEARL: Mets have lumpy bumpy surface due to involution in area away from blood supply (vs melanoma with smooth dome due to rich blood supply present). 7

8 1/23/2018 Differential diagnosis Melanoma (amelanotic) Hemangioma Metastasis Osteoma Atypical nevus Posterior scleritis Choroidal detachment Granuloma Old subretinal hemorrhage OD NORMAL FOVEAL DEPRESSION OS NFL GCL IPL INL OPL ONL OLM RPE Choroidal Elevation OS LOSS OF FOVEAL CONTOUR Differential diagnosis Melanoma (amelanotic) Hemangioma Metastasis Osteoma Atypical nevus SRF Posterior scleritis Choroidal detachment Granuloma Old subretinal hemorrhage 8

9 Choroidal Metastases #1 intraocular tumor in adults Autopsy studies show 10% cancer have choroidal mets Breast cancer choroidal mets almost 40% Hematogenous dissemination Choroid > iris/ciliary body > retina/optic nerve Ciliary body 2% Iris 9% Choroid 89% Primary site Mostly carcinomas 1/3 do not have a known cancer history Presenting symptoms Asymptomatic (11%) Male Lung (40%) Unknown (30%) GI (10%) Kidney Prostate Skin Others Breast Female Breast (70%) Lung (10%) Unknown (10%) Others GI Skin Kidney Blurred vision (70%) Flashes & floaters (12%) Pain (7%) Clinical appearance Relatively flat, ill defined, creamy yellow Overlying RPE changes ( leopard spotting in breast ca) Exudative RD Ultrasonography A scan: Moderate high internal reflectivity B scan: Ill defined, ±lobulated choroidal mass Bilateral/multifocal in 25% (breast ca most likely) 9

10 Fluorescein angiography Early hypofluorescence with late hyperfluorescence Rarely useful to differentiate from other lesions Prognosis & Treatment Prognosis Mean life expectancy 9 10 months Treatment Systemic chemotherapy Hormone therapy External beam, brachytherapy, proton beam Surgery Myth 4: No harm from topical steroids for inflammation When considering intraocular inflammation and a possible uveitic diagnosis, the top priority is to rule out infection. Beware of 5 uveitis red flags First episode in elderly patient Intensive topical corticosteroid therapy can significantly confound clinical picture. 5 uveitis red flags First episode in elderly patient 5 uveitis red flags First episode in elderly patient Significant systemic illness Significant systemic illness Immunosuppressed patients 10

11 5 uveitis red flags First episode in elderly patient Significant systemic illness Immunosuppressed patients HIV/AIDS 5 uveitis red flags First episode in elderly patient Significant systemic illness Immunosuppressed patients HIV/AIDS Drug addicts 5 uveitis red flags First episode in elderly patient Significant systemic illness It s an infection if Hospitalized patients indwelling catheters Post bowel surgery Immunosuppressed patients HIV/AIDS Drug addicts It s an infection if Hospitalized patients with indwelling catheters, bowel surgery HIV/AIDS It s an infection if Hospitalized patients with indwelling catheters, bowel surgery HIV/AIDS Cancer history and on immunosuppression 11

12 It s an infection if Myth 5: Viral vectors not safe Hospitalized patients with indwelling catheters, bowel surgery HIV Cancers on immunosuppression Conditions with low white blood cell counts E.g., Leukemia Leber congenital amaurosis (LCA) Clinical features Group of disorders due to mutation of at least 16 different genes Autosomal recessive in majority of cases Severe visual impairment before age 1, nystagmus, poor pupillary reflexes, normal or mildly abnormal fundus appearance, profoundly abnormal or absent ERG (Graefes Arch Clin Exp Ophthalmol 1869;15:1, Confinia Neurol 1954;14:184, Ophthalmologica 1947;114:332) Clinical features Normal fundus appearance RPE granularity Vessel attenuation Macular coloboma Salt and pepper retinopathy Retinitis punctata albescens Nummular pigmentation LCA: no treatment available! But, you have preservation of all retinal layers Of all causal genes, recessive biallelic RPE 65 is prototype Related to how eyes detect light (Clin Genet 1973;4:270, Am J Ophthalmol 1977;83:27, Int Ophthalmol Clin 1968;8:949) 12

13 RPE65 is enzyme that resets mousetraps 11 cis retinal (Vit A) High Energy State RPE 65 gene replacement supplies HIGH Energy Vitamin A 1 to 1000 All trans retinal (Vit A) Low Energy State } Rod Outer Segment (ROS) Retinal Pigment Epithelium (RPE) 1 : 1000 RPE65 gene (replacement) therapy Use nano injectors to insert DNA into cell Nano injector is adeno associated virus (AAV) Virus has limited space for DNA Small gene small virus class, AAV Large gene large virus 1 month 13

14 Untreated eye Treated eye LCA RPE-65 is a prototypical inherited ocular disease No treatment available Rare but blinding disease for those affected Gene replacement therapy Safe Effective Long-lasting Myths 1. Presence of a Weiss ring indicates a complete posterior vitreous detachment from the retina. Thanks! 2. All retinal detachments are an emergency. 3. White dots in the posterior segment are due to inflammation. 4. There is no harm from the use of topical corticosteroids for intraocular inflammation. 5. Viral vectors for gene therapy are not safe. 14