First Author: A.Saklamaz TURKEY
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To evaluate the clinical, optical coherence and fundus fluorescein angiography findings in a Bietti crystalline dystrophy patient
Atagoz Eye Hospital, Izmir, Turkey
Case report and literature review
Numerous small, yellow or white crystal-like deposits of fatty (lipid) compounds accumulate in the posterior pole of retina. Large atrophy of retinal pigment epithelium and choroidal sclerosis were observed on both funduscopy and angiography. Fluorescein angiography showed geographic areas of RPE and choriocapillaries atrophy. Visual fields were constricted and electrophysiology was greatly impaired. OCT examination revealed modifications of the outer retina and the presence of numerous lesions corresponding to microcrystals deposits in all retinal layers and wide RPE atrophy.
Bietti crystalline dsytrophy is a progressive disease characterized by retinal crystals gradually replaced by atrophy of the retinal pigment epithelium and gradual constriction of visual fields. BCD is a rare, autosomal recessive, progressive, degenerative eye disease caused by mutations in the CYP4V2 gene, for which no treatments are currently available.