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1 Financial Disclosure: JP Choroid Chronicles Joseph J. Pizzimenti, OD, FAAO Honoraria " Kemin " Nicox " Review of Optometry " Optometric Management " VSP! Scientific Advisory Boards " Zeavision " Carl Zeiss Meditec " Thrombogenics! CE Companies " CEinItaly.com Goals for This Course! Functional anatomy review! Choroid examination and evaluation! Case examples! Interactive The Choroid: Structure, Function, and Evaluation Melanoma The Choroid! Located between the sclera and the RPE " Extends from ora serrata to optic nerve! Pigmented/vascular tissue.75mm thick! Nourishes the RPE " Choriocapillaris designed to leak! Absorbs light that passes through retina 1
2 The Choroid Photoreceptors RPE Mel. CC BM Sclera Choroidal thickness! Loose connective tissue! Melanocytes! Choriocapillaris " Fenestrated endothelium allows diffusion of proteins " S regulation " High blood flow " Very little O-2 extracted, so high venous O-2 Bruch s Membrane! Basal lamina of RPE! Anterior collagenous layer! Elastic layer! Posterior collagenous layer! Basal lamina of CC endothelium! Contamination of Bruch s can result in d, CNVM Common Causes of CNV! Exudative AMD! Ocular Histoplasmosis! High Myopia! Angioid Streaks 48 y/o WM D Concave fundus, CNV, schisis CNV Variants Polypoidal Choroidal Vasculopathy (PCV) Retinal Angiomatous Proliferation (RAP) Choroidal Vasculature! SPCAs provide blood flow to choroid posterior to equator! ACA and LPCA supply anterior choroid! Vortex veins drain the choroidal veins! V.V. drain into sup, inf ophthalmic vein 2
3 Choroid Anatomy Retinal Vasculature! 2 sources of blood supply:! Choroidal BV " Supply outer retinal layers, including PRs! CRA " 4 branches nourish inner retina " Run radially toward fovea Choroid Microstructure Vascular Layers of the Choroid Imaging the Choroid Fluorescein Angiography (FA)! FA answers the question: is the blood-retinal barrier intact? 3
4 The Fluorescein Angiogram CSC! Stages " Choroidal phase " Arterial phase " Laminar venous phase " Venous phase " Recirculatory phase " Late phase Indocyanine Green Angiography (ICGA)! Uses digital imaging! Dye properties! Sees through blood! Delineates choroidal circulation better than fluorescein angiography! Boundaries of occult membranes imaged Echography of Choroidal Melanoma B-Scan Echogram Assess topographic features, including tumor shape, surface contour and boundaries A-Scan Echogram Internal structure, reflectivity, tumor height (elevation) Hypertensive Choroidopathy Elschnig s Spots + NAION Common Etiologies of Panuveitis! Infections, such as " Infantile toxocariasis " Post-op bacterial endophthalmitis " Toxoplasmosis! Granulomatous Dx.! Multifocal Choroiditis and Panuveitis (right) " MCP in a 27 y/o Asian Female 4
5 Multifocal Choroiditis and Panuveitis! Bilateral, chronic uveitis " Punched-out chorioretinal lesions similar to POHS.! CNV is the most worrisome potential complication.! Conservative monitoring and timely steroid treatment essential to care.! Immunosuppressive drug therapy appears to limit the number of recurrences. Toxocariasis QUESTIONS AND COMMENTS? Case History! 61 y/o BF! CC: Referred for evaluation of retinal cysts! POHx: Unremarkable! PMHx: + HTN/Hypercholesterolemia! Medication: Atenolol, Maxzide Acknowledgement: Sherrol Reynolds, OD, FAAO Ocular Examination! Best-corrected VA: 20/20 OD, 20/20 OS! Pupils: Equal & round, APD New lesion! EOM/CVF: Unremarkable! SLE: Unremarkable DFE OD: Multiple lesions 5
6 OCT-Evaluation DFE: OS RETINAL PIGMENT EPITHELIUM DETACHMENT DFE: OS Another case 60 y/o BF OD 60 y/o BF OD 60 y/o BF OS 6
7 Final Diagnosis! Polypoidal Choriodal Vasculopathy (PCV) serosanguineous! Multiple recurrent serosanguineous detachments of the RPE, sensory retina Serous Fluid (serum) / Hemorrhagic (blood) disorder Polypoidal Choroidal Vasculopathy PCV! First described in 1982 Posterior Uveal Bleed Syndrome -or- Multiple, Recurrent Retinal Pigment Epithelial Detachments in Black Women! Idiopathic Polypoidal Choroidal Vasculopathy (IPCV) Demographics! Higher incidence in darker pigmented people " African American, Hispanic, and Asian " Predilection for African American women Clinical Features! Aneurysmal structures at the termination of a network of dilated choroidal veins! Recurrent hemorrhagic/exudative detachment of the RPE Combined 7
8 Pathogenesis of PCV! Inner choroid! Network of branching vessels with terminal aneurysmal red spheroidal dilitations (polyps) " Dilated, thin-walled vessels of choriocapillaris! Recurrent hemorrhage and leakage! Variant of inner choroidal neovascularization (CNV) Note peripapillary red-orange nodular lesions contiguous with elevated, sinuous, tubular lesions extending through posterior pole Rosa, R. H. et al. Arch Ophthalmol 2002;120: Key Findings in PCV! Usually bilateral! Lack of drusen! Chronic, recurrent RPED and neurosensory detachment! Serosanguineous (serum and/or blood) 8
9 Key Findings in PCV PCV vs. AMD! Chronic, remitting-relapsing course! Peripapillary region most often affected! Good visual prognosis, unless " Fovea involved! Average age of diagnosis is 60 POLYPOIDAL RPED AMD -SUBRETINAAL HEMORRHAGE AMD! Features c/w Wet AMD " race " foveal location " elderly patients (> 70 y/o) " presence of drusen " rapid rate of CNV progression " disciform scarring " poorer visual prognosis ICGA is diagnostic for PCV Polypoidal lesion OD ICG- early phase Late staining of the vascular network and filling of the RPED SCHNEIDER, U. et al. Br J Ophthalmol 1998;82:98-99 Br J Ophthalmol 2008;92: doi: /bjo
10 PCV and Systemic Disease! Concomitant systemic microvascular disease Diabetes Dyslipidemia Hypertension Idiopathic Treatment! Conservative management " Observation! Spontaneous regression of most RPEDs! Longstanding non-progressive exudation! Control underlying systemic conditions Treatment! Thermal (Argon) Laser Photocoagulation! Photodynamic Therapy " ICGA-guided PDT! Anti-VEGF therapy " Intravitreal injections of bevacizumab! Principle: reduce abnormal vasculature of PCV QUESTIONS AND COMMENTS? Matsuoka et al and Tong et al Neoplastic Disease 10
11 To Find Small Ocular Melanoma NEVOMA Most patients with choroidal melanoma have no symptoms. Their tumors are found during a "routine" eye examination. To Find Small Ocular Melanoma T= thickness F= subretinal fluid S= symptoms O= orange pigment M= margin touches disc No risk factors (<4%) 1 risk factor (36%) 3 risk factors (50%) 5 risk factors (70%) DOCUMENTED GROWTH - MEANS EVERYTHING Using Helpful Hints = Ultrasound hollow, halo Orange Pigment = Lipofuscin Fundus Autofluorescence (FAF) on Choroidal Mass 11
12 FAF in Choroidal Melanoma Echography Acoustic hollowness on B-scan of small melanoma. Echography of large melanoma Amelanotic Choroidal Melanoma Variable Presentation of Choroidal Melanoma 12
13 Primary choroidal melanomapre-radiotherapy Melanoma pre/post-radiotherapy Treatment side effects! Main side effect of focal ocular treatment is! Radiation retinopathy!! NVD / NVE! Exudative changes! Macular edema Radiation Retinopathy: exudate, NVD! Occurs several weeks to months after therapy Acknowledgement: Sherrol Reynolds, OD, FAAO Choroidal Melanoma: pre-tx S/P Radiotherapy 13
14 Management of RR Choroidal Metastasis! Avastin/Lucentis/Eylea! Laser!? Silicone oil at time of Brachytherapy Choroidal Metastasis METS Multiple cream colored lesions in posterior pole Large lesion with neurosensory RD Early IVFA OS! Note blocking of the background hyperflouresence in multiple areas, including large central lesion! Note late staining of large central lesion Late IVFA 14
15 Pearl! Rapid shrinkage of a choroidal tumor with treatment may be bad news indicates substantial malignant (and metastatic) potential! QUESTIONS AND COMMENTS? I wish to acknowledge and thank: Brad Sutton, OD, FAAO Carlo Pelino, OD, FAAO Sherrol Reynolds, OD, FAAO Thank you! Joe pizzimen@nova.edu 15
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