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¹Yeditepe Üniversitesi Tıp Fakültesi Hastanesi, Göz Hast. A.D., İstanbul, Yard. Doç. Dr.
²Yeditepe Üniversitesi Tıp Fakültesi Hastanesi, Göz Hast. A.D., İstanbul, Doç. Dr.
³Yeditepe Üniversitesi Tıp Fakültesi Hastanesi, Göz Hast. A.D., İstanbul, Prof. Dr. Evaluation of central macular function by fundus autofluorescence (FAF), microperimetry and multifocal electroretinography (MfERG) in a case with Bietti crystalline dystrophy (BCD) is described. A 43-year-old woman presented with progressive visual loss in both eyes for the last 4 years. Best corrected visual acuity was 20/125 in the right eye and 20/32 in the left eye. Fundus examination revealed diffuse and profound chorioretinal atrophy at posterior pole along with glistening intraretinal crystals. FAF imaging showed central hypoautofluorescent areas encircled by hyperautofluorescent speckles. Microperimetry revealed an absolute scotoma in the right eye in central 20° . The relative scotoma in the left eye exactly demonstrated the remaining intact foveal zone as an area of reduced retinal sensitivity corresponding to angiography. MfERG in central 20° revealed significant suppression of amplitudes, particularly more reduced in the right eye. Although BCD is a progressive and untreatable chorioretinal disorder, retinal function evaluation offers a better understanding of the severity and the stage of the dystrophy. Keywords : Bietti crystalline dystrophy, microperimetry, fundus autofluorescence, multifocal electroretinography