Hemoglobin S/O(Arab): Retinal Manifestations of a Rare Hemoglobinopathy

Hemoglobin S/O_Arab (Hgb S/O_Arab) 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/O_Arab. 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/O_Arab, and the first report of fundus autofluorescence and OCT angiography in Hgb/O_Arab retinopathy.