Hemoglobin S/OArab (Hgb S/OArab) disease is a rare hemoglobinopathy which presents similarly to sickle cell retinopathy, with only three prior reports that describe associated retinal findings. In this report, we present ophthalmic examination findings in 2 patients with Hgb S/OArab. One patient exhibited peripheral ischemia and sunburst lesions without neovascular disease, and the other patient developed proliferative retinopathy of both eyes and multiple posterior-pole branch retinal artery occlusions in one eye. To our knowledge, this is the first case of retinal arterial occlusive disease in Hgb S/OArab, and the first report of fundus autofluorescence and OCT angiography in Hgb/OArab retinopathy.
Keywords: Hemoglobin S/OArab; Hemoglobinopathy; Retinal ischemia; Sickle cell retinopathy.
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