Retinal Physician

JAN-FEB 2017

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28 R E T I N A L P H Y S I C I A N . C O M | J U N E 2 0 1 3 (Figure 5), patients frequently complain of nyctalopia and paracentral scotomas. is paracentral scotoma can be identi- fied and tracked with visual fields, the most sensitive of which is a 10-2 Humphrey visual field. Early in the course of the dis- ease, fundus appearance is normal and in later stages a bull's eye maculopathy develops. Macular OCT reveals a loss of the external limiting membrane, disruption of the ellipsoid zone, parafoveal thinning of the outer nuclear layer, and RPE dam- age. 18 On multifocal ERG, paracentral, central or generalized amplitude reductions develop. 19 FAF findings vary depending on disease severity: early pathology highlights a paracentral ring of increased autofluorescence; moderate severity shows a paracentral mottled hypoautofluorescence with an adjacent hyperautofluorescence; and advanced disease reveals a complete central loss of autofluorescence. 19 Cancer Associated Retinopathy Cancer associated retinopathy (Figure 6) typically presents with subacute vision loss over weeks to months. 20 Symptoms vary depending on the degree of rod and cone involvement; patients frequently present with symptoms of shimmering or flicker- ing lights. 20 On clinical examination, patients show a normal appearing fundus early in the course of the disease, but with progression, they develop retinal arteriolar attenuation, retinal pigment epithelial mottling and optic disc pallor. 20,21 Goldman visual field testing can identify a generalized depression or a central, paracentral, arcuate or ring scotoma. 22 ERG is a sen- sitive diagnostic test early in the course of disease and dem- onstrates a depression of a- and b-waves on either phototopic and/or scotopic conditions depending on the degree of rod or cone involvement. 22 FAF can reveal abnormal hyperautofluo- rescence surrounding a parafoveal region of normal autofluo- rescence .23 Macular OCT shows a loss of outer retinal complex R E T I N A L P H Y S I C I A N | J A N U A R Y / F E B R U A R Y 2 0 1 7 28 AMD MASQUERADE SYNDROMES Figure 5. is 65-year-old female was referred with a 2-month history of bilateral blurring of central vision. VA measured 20/30 RE and 20/20 LE. Fundus examination revealed a bilateral bull's eye paern of RPE changes and FAF demonstrated a bilateral ring of hypoautofluorescence surrounded by a ring of hyperautofluorescence (Figures A and C). Macular OCT identi- fied parafoveal ellipsoid zone loss along with generalized reti- nal thinning (Figure B). Aer additional questioning regarding her current and past medications, she endorsed a 20-year his- tory of hydroxychloroquine use that was stopped several years prior to presentation. Despite no hydroxychloroquine use for a number of years, this patient most likely demonstrated symptomatic progression of hydroxychloroquine toxicity. C Figure 6. is 71-year-old male was referred with nyctalopia and with subacute blurring of central vision bilaterally. VA mea- sured 20/50 RE and 20/40 LE and fundus examination revealed parafoveal RPE moling (Figure A). Macular OCT identified a generalized irregularity of the ellipsoid zone (Figure B). FA revealed staining of the RPE moling only (Figure C). ERG was consistent with rod > cone dysfunction. A Figure 7. is 43-year-woman with a history of myotonic dystrophy was referred with blurring of central vision LE. VA measured 20/30 LE. Fundus examination revealed mild pigmentary changes in the macula (top le), corroborated on red-free fundus photography (top right). FA demon- strated patchy staining of the RPE moling on early and late frames (boom le and right respectively). ese findings were consistent with myotonic dystrophy with paern dys- trophy of the macula. A B C D C A B B

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