This 45YO male with a known history of sickle cell SC disease presented with new floaters and blurred vision in his right eye. Vision was 20/70 OD and 20/30 OS.
Optos color RG imaging shows a fairly dense vitreous hemorrhage OD, with variably fibrosed peripheral retinal neovascularization superotemporally. The left fundus shows temporal retinal ischemia with white vessels and mostly active retinal neovascularization along its posterior extent. Scattered black sunburst lesions are noted temporally as well. Fluorescein angiography OS shows an irregular, abrupt margin of peripheral complete capillary nonperfusion, especially temporally. The neovascularization in his OD shows late leakage.
Avastin was injected OD. Given the active vessels in his OS, along with the recent vitreous hemorrhage OD, we decided to prophylactically treat the left ischemic retina with scatter laser.
Learning Points:
Patients with sickle cell disease develop symptoms throughout their bodies as stiffened, sickled red blood cells block capillary beds. In the eye, this can cause proliferative sickle cell retinopathy (PSR) with sea-fan-shaped areas of peripheral retinal neovascularization developing at the junction of perfused and ischemic retina. The black sunburst lesions are thought to occur from intraretinal RPE migration in response to photoreceptor damage from intraretinal and subretinal hemorrhage (Asdourian et al, BJO 1975;59:710-716).
Complications include vitreous hemorrhage and traction retinal detachment. Although patients with SS disease tend to have more systemic complications, SC disease is usually associated with more severe ocular findings. Treatment with peripheral scatter laser photocoagulation is somewhat controversial since, unlike more typical proliferative retinopathies (ie from diabetes and branch retinal vein occlusions), these new vessels tend to auto-infarct.
For a great review article on sickle cell disease and the eye, see Elagouz et al, Surv Ophthalmology 2010;55;359-377.
