A case of Bernard-Soulier syndrome with central retinal vein occlusion

Background: Bernard-Soulier syndrome (BSS) is a coagulopathy that is caused by the lack of glycoprotein Ib/IX/V in the plasma membrane of platelets. Case Report: A 60-year-old male who had been diagnosed with BSS complained of decreased vision in his right eye. Fundus examination revealed central retinal vein occlusion, but fibrinolysis therapy was contraindicated due to the high risk of severe systemic hemorrhage. Alternatively, he underwent corticosteroid therapy, which decreased retinal edema. Corrected visual acuity in the right eye improved from 0.1 to 0.7 by day 7 of treatment, was 1.2 after 8 months, and has remained 1.2 for 1 year after the end of treatment. Conclusions: Central retinal vein occlusion can affect patients with BSS, a platelet function disorder. Corticosteroid therapy was effective in reducing retinal edema in this case.