SICKLE CELL RETINOPATHY

Occlusion of small blood vessels in the eye, retinal neovascularization and its consequences occur in high frequency in patients with sickle cell disease. Neovascular sea fans arise at the interface of perfused and nonperfused portions of the retina. Although some lesions undergo spontaneous autoinfarction, most show progressive growth, resulting in vitreous hemorrhage and retinal detachment. The emergence of neovascolarization is stimulated by the production of vascular growth factors and immunoreactivity for these factors is greater within neovascularization than in adjacent retina and in non perfused peripheral retina. All these lesions can be observed directly with an ophthalmoscope and indirectly with fluorescein angiography. The differential diagnosis of the nevascularization with other ischemic and inflammatory diseases is necessary. The usual goal of management is the early treatment of stage IlI lesions with laser photocoagulation and cryotherapy, pars plana vitrectomy or scleral buckling are avoided unless it is absolutely necessary.