The most notable effect of sickle cell disease on the ocular system is:

• A. Neovascularization
• B. Retinal detachment
• C. Cataracts
• D. Glaucoma

Answer: (A)

Proliferative retinopathy driven by retinal ischemia is the hallmark of sickle cell disease in the eye. When peripheral retinal vessels become occluded from the abnormal red blood cells, the retina becomes ischemic and releases VEGF, prompting growth of abnormal new vessels on the retinal surface and optic disc. These neovascularizations are fragile, prone to hemorrhage, and can lead to vitreous bleeding and tractional retinal detachment. Among the options, neovascularization best represents the most notable and characteristic ocular effect. Retinal detachment, cataracts, and glaucoma can occur, but they are not as specifically tied to sickle cell retinopathy as the proliferative neovascular response.