Ocular Manifestations in Sickle Cell Disease

Ocular findings in sickle cell disease are manifestations of sluggish blood flow, intravascular sickling, and occlusion of vessels. The effects on ocular tissues include sludged flow, dilatation, and segmentalization of conjunctival vessels; segmental ischemic iris atrophy with occluded vessels and neovascularization; vitreous hemorrhages that become organized by fibrovascular proliferative membranes arising from retinal vessels; marked tortuosity of retinal vessels, occlusion of retinal vessels leading to ischemic infarcts, retinal hemorrhages, chalk-white vessels, retinal atrophy, splitting of the retina, and retinal holes; neovascularization with the abnormal arteriovenous connections causing proliferation of new vessels that form sea-fan lesions; iridescent intraretinal deposits and rhegmatogenous and traction retinal detachment; and chorioretinal infarcts and heavily pigmented scars.