PSR is progessive and a major cause of visual morbidity in sickle cell disease. A desirable objective is to treat the neovascular tissue before a vitreous hemorrhage occurs. Glaucoma secondary to rubeosis iridis may occur. Infection may cause a crisis. Preventing infections may avoid a sickle cell crisis.
Treatment is directed toward the underlying infection, hypoxia, dehydration and pain.
Several systemic treatment strategies have evolved, including the inhibition of intracellular HbS polymerization and the polymer effects on the erythrocyte membrane, vasodilation, and modification of the interaction between the erythrocytes and blood vessels. This technique has been successful with the administration of 5-azacytidine, hydroxyurea, and most recently, arginine butyrate. If the intraocular pressure remains elevated after a judicious trial of medical therapy, surgical intervention with an anterior chamber lavage is indicated. Ocular treatment is directed toward preventing vision loss from vitreous hemorrhage, retinal detachment, and epiretinal membranes. Because of potential complications from photocoagulation and the tendency for regression, patients older than 40 years probably do not require treatment.