The ocular fundi of 24 selected patients with sickle cell-hemoglobin C disease were studied by indirect ophthalmoscopy and fluorescein angiography. Based on these studies, a new, quantitative classification of proliferative sickle retinopathy (PSR) was devised, which reflects both increasing severity of the retinopathy as well as its natural progression. The first sign of PSR is peripheral arteriolar occlusions (Stage I); 91% of eyes were affected. Peripheral arteriolar-venular anastomoses (Stage II) were observed in 85% of eyes. Neovascular and fibrous proliferations (Stage III), which develop from the A-V anastomoses, occurred in 81% of eyes. Seafan lesions are part of this stage. Vitreous hemorrhages (Stage IV) from the neovascular proliferations occurred in approximately 30% of eyes, and retinal detachment (Stage V) was observed in 8% of eyes. Pathogenetic parallels appear to exist between PSR and other vasoproliferative disorders, such as diabetes mellitus, hypotensive vascular diseases, and retrolental fibroplasia.
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