Sickle cell hemoglobinopathies all share the common feature of an abnormal globin chain, which leads to sickling of erythrocytes and obstruction of the microcirculation. Sickle vaso-occlusive events are insidious and affect virtually every vascular bed in the eye, often with visually devastating consequences. Vaso-occlusion most profoundly affects the retina, the light-sensitive tissue that lines the inside wall of the posterior aspect of the eye, because it is exquisitely sensitive to deprivation of oxygen. Even temporary vaso-occlusion, if longer than about 1.5-2 h, can result in permanent infarction of the retina. Most, if not all, of the complications of sickle cell disease in retina originate from the vaso-occlusive processes. The pathological changes can be divided into nonproliferative and proliferative events.
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