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Meet the Choroid N Optometric Management Joe Pizzimenti, OD, FAAO O Scientific Advisory Boards [email protected] N Zeiss N Zeavision N Thrombogenics N Genentech

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Meet the Choroid N Optometric Management Joe Pizzimenti, OD, FAAO O Scientific Advisory Boards Allthingsoct@Gmail.Com N Zeiss N Zeavision N Thrombogenics N Genentech 10/29/19 Financial Disclosures o Honoraria n Review of Optometry Meet The Choroid n Optometric Management Joe Pizzimenti, OD, FAAO o Scientific Advisory Boards [email protected] n Zeiss n Zeavision n Thrombogenics n Genentech Financial Disclosures Goals for This Course o Consulting Fees n Zeiss o Functional anatomy review n Zeavision n Choroid n Maculogix o Choroid examination and evaluation o Proprietary Interests o Case examples n None o Interactive o Stockholder: Zeavision Questions? 1 10/29/19 The Choroid The Choroid: Structure, o Located between the Function, and Evaluation sclera and the RPE n Extends from ora serrata to optic nerve o Pigmented/vascular tissue .75mm thick o Nourishes the RPE n Choroiocapillaris designed to leak o Absorbs light that passes through retina The Choriod RPE Bruch’s Membrane thickness o Loose connective tissue o Basal lamina of RPE o Melanocytes o Anterior collagenous o Choriocapillaris layer Mel. n Fenestrated endothelium o Elastic layer allows diffusion of o Posterior collagenous proteins CC layer n S__________ regulation o Basal lamina of CC BM n High blood flow endothelium n Very little O-2 extracted, o Contamination of so high venous O-2 Bruch’s can result in sclera d________, CNVM Nourishing the Retina Choroid Microstructure o 2 main sources of blood supply to retina: • Choriocapillaris o Choroidal BVs n Supplies outer retinal • Sattler’s layer layers, including PRs o CRA • Haller’s layer n 4 branches nourish inner retina • Supra - choroid n Run radially toward fovea 2 10/29/19 Imaging the Vascular Layers Imaging the Choroid of the Choroid WHAT IS ENHANCED Imaging the Choroid-EDI DEPTH OCT IMAGING? • EDI-OCT • Enhanced-depth imaging (EDI) OCT modifies the standard technique of image acquisition to better reveal the structural details of the choroid. EDI HOW IS EDI ACHIEVED? • SD-OCT has a coherence gate of about 2 mm. • Coherence gate is the tissue depth at which the interference image can be obtained. • An interference signal can be obtained when the tissue being examined enters the coherence gate. • However, the signal intensity attenuates in the depth direction, from superficial (retinal) to deep layers (choroid). 3 10/29/19 HOW IS EDI ACHIEVED? EDI SHOWS DEEPER INTRAORBITAL ON, LAMINA, C/S JXN • Consequently, to obtain high-quality images in standard SD-OCT, it is important to bring the retinal tissue (B-scan) to the upper aspect of the imaging range. • In contrast, EDI-OCT creates an inverted mirror image. The reference surface of the inverted mirror image is on the choroidal side. EDI Indocyanine Green Angiography (ICGA) o Uses digital imaging systems o Dye properties o “Sees” through blood o Delineates choroidal circulation better than fluorescein angiography o Boundaries of occult membranes imaged Questions? Uveal Melanoma 4 10/29/19 Many patients with uveal melanoma have no symptoms. Their tumors are found during a "routine" eye examination. Uveal melanoma is the second-most common form of melanoma (skin) and the most common primary intraocular malignancy. Up to 50% of patients are at risk for fatal metastatic disease. Choroidal melanoma has an annual incidence of 5-6 cases per million people per year. Other than having blue or green eyes and a light complexion, studies have not identified any definite risk factors or exposures that Uveal Melanoma predispose patients to developing this cancer. Amelanotic Choroidal Melanoma 5 10/29/19 Q: Where does Choroidal melanoma come from? Asymptomatic A: Choroidal Nevus Systemically Flashes well • Ch Nevus is the most common intraocular Pain is rare Scotoma tumor • Proliferation of choroidal melanocytes Vitreous VF hemorrhage defect • Present in ~ 7.9% of Caucasians • Growth is rare after Presentation of Melanoma puberty? Choroidal Nevus? As we age: • Nevi < 2 mm in thickness (A-scan) • Nevi increase in number and • No known relationship thickness to sunlight exposure • Pigment • Indistinct borders changes • May undergo malignant • Metaplasia change into melanoma Choroidal Nevus? •Drusen/lipofuscin Choroidal Nevus Workup and Management • Baseline fundus photography, FAF • OCT if location permits, OCTA? • A/B-Scan • IVFA? • Yearly dilated fundus examination –Or more frequent 6 10/29/19 Risk Factors for Melanoma “Nevoma” qCaucasian qLight colored eyes (blue) qFair skin qPropensity to burn when exposed to UV light qCutaneous nevi or freckles qIris nevi qWelders To Find Small Ocular Melanoma Using Helpful Hints Daily • T= thickness (>2mm) • No risk factors (<4%) Follow or Co-manage? • F= subretinal fluid • 1 risk factor (36%) • S= symptoms • 3 risk factors (50%) • O= orange pigment • 5 risk factors (70%) • M= margin touches disk DOCUMENTED GROWTH - MEANS EVERYTHING Using Helpful Hints Daily= Ultrasound hollow, halo absent, drusen absent 7/29/2016 OcularmelanomaCalculator.com 7 10/29/19 Echography of Small Choroidal Melanoma EDI-OCT B-Scan Echogram Assess topographic features, including tumor shape, surface contour and boundaries A-Scan Echogram Internal structure, o Mean small melanoma thickness was reflectivity, tumor height (elevation) 1025 microns on EDI-OCT compared to 2300 microns on ultrasonography. Enhanced Depth OCT (EDI) of a small melanoma Orange Pigment = Lipofuscin Shields, 2012 Fundus Autofluorescence (FAF) Fundus Autofluorescence (FAF) in of a Small Choroidal Mass Choroidal Melanoma Melanoma 8 10/29/19 Echography of Large Melanoma Nevus w/Drusen = Chronicity No Drusen, No halo Halo Nevus = Chronicity No Drusen, no Halo Questions? 9 10/29/19 • Common, benign lesion • Focal area in which RPE cells are taller and more densely Differential Diagnoses packed with melanosomes Congenital Hypertrophy of the Retinal Pigment Epithelium Familial Adenomatous Metastatic Tumors to the Choroid Polyposis (FAP) & CHRPE • Breast cancer is the most common CA type to – AD inheritance metastasize to the eye - followed by lung CA – Adenomatous polyps throughout rectum & • 85% of patients with breast CA metastases will have colon a known history of breast CA – Starts to develop in • Breast CA metastases tend to be bilateral and adolescence (15-40 yrs) multifocal (multiple) – If untreated, all pts will develop colorectal CA >8 0 % of patients with • 40% of these patients have a brain metastasis FAP have atypical CHRPE lesions Metastasis from lung to choroid Metastatic Prostate Cancer 10 10/29/19 • Benign melanocytic uveal METS tumor Melanocytoma • Composed of large, plump nevus cells that are heavily pigmented • Can present in all age groups and races, though more common in • African-Americans • Females • Patients are usually Melanocytoma Combined Hamartoma of the Retina and RPE asymptomatic • Grey pigmentation with superficial gliosis • Adjacent to or within • the optic nerve Secondary retinal wrinkling and vessel tortuosity • Black in color with feathery margins • Lesions can be juxtapapillary, peripapillary or within • Visual field defect may be present the posterior pole Absence of retinal detachment, • APD may be present hemorrhage, exudation or vitreous inflammation Genetic Testing on Small Melanoma • Enucleation • Transpupillary (Shields, • Radioactive thermotherapy 2015) plaques • Proton beam • Local resection radiotherapy Most widely accepted Less common Treatment & Management 11 10/29/19 Collaborative Ocular Melanoma Study Co-manage Uveal • Organized and funded in 1985 to address issues related to management of choroidal melanoma. Melanoma with: • Main Outcome: overall survival of patient following treatment • > 4000 patients. 65% pts eligible Retina/Ocular Oncology Small melanomas < 2.5 mm in height Medium melanomas 2.5 – 10.0 mm PCP Large melanomas > 10.0 mm General Oncology • Secondary outcomes: metastasis-free survival, years of useful vision Brachytherapy for Uveal Melanoma Treatment Side Effects Plaque left in place for 4 days to provide 8,000 centigray of radiation to entire tumor. The remainder of the body receives a small amount of radiation, about the equivalent of a chest x-ray. • Main side effect of focal ocular treatment is… • Radiation retinopathy! • NVD / NVE • Exudative changes • Macular edema • Occurs several weeks to months after therapy Melanoma pre-Tx echography (left), Choroidal melanoma- post-radiotherapy (right) pre-Radiotherapy 12 10/29/19 Radiation Retinopathy: Choroidal Melanoma Pre-Tx exudate, NVD Acknowledgement: SherrolRey n o ld s, O D , FA A O S/P Radiotherapy Management of RR • Avastin/Lucentis/Eylea • Laser • Silicone oil at time of Brachytherapy –attenuates radiation dose, and may protect against radiation retinopathy Melanoma Metastasis Risk factors for metastasis from the choroid • Thickness > 2 mm • Symptoms – Flashes, floaters, loss of vision • Proximity to the optic nerve • Documented growth Shields Cl Shields JA. Risk factors for metastasis of small choriodal melanocytic Les ions . Ophthalmology 1995 13 10/29/19 Ocular Melanoma Quiz Ocular Melanoma Quiz • What is the 10-year mortality rate for • What is the 10-year mortality rate for patients diagnosed with a large uveal patients diagnosed with a large uveal melanoma? melanoma? a. 1 % a. 1 % b. 7% b. 7% c. 9% c. 9% d. 50% d. 50% CENTRAL SEROUS Questions CHORIORETINOPATHY OCT W/EDI IN CSC CSC MANAGEMENT PACHYCHOROID AND SUBRETINAL FLUID IN CSC • Due to the high likelihood of spontaneous resolution, first line therapy for first time CSC remains risk factor modification (reduce stress, d/c steroids) and observation. 14 10/29/19 CSC MANAGEMENT • For CSC that persists or returns: Common Causes of CNV • In eyes with focal lesions not involving the
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