Sickle Cell Retinopathy: a Review

Sickle cell disease is one of the most prevalent hemoglobinopathies in the world. In Iran, sickle cell disease is more common in southern parts of the country such as Khuzestan province. Retinopathy is the most representative ocular complication of sickle cell disease. Sickle cell retinopathy is characterized by the vaso-occlusion of capillary beds and is classified in two types of proliferative and non-proliferative according to presence or absence of vascular proliferation in fundus. In non-proliferative sickle cell retinopathy, the retinal changes do not involve neovascularization as they do in proliferative sickle cell retinopathy. The two most severe complication of proliferative sickle cell retinopathy are vitreous hemorrhage and retinal detachment, which may lead to visual loss. Identification and prompt referral of these patients has a critical role in prevention of irreversible visual loss. Newer imaging modalities such as ultra–wide field fluorescein angiography, spectral domain optical coherence tomography and optical coherence tomography angiography are now available. These techniques can detect the sickle cell retinopathy in its early stages. In this review, we briefly discuss the manifestations, diagnosis and management of sickle cell retinopathy.