This 45YO female with a known history of sickle SC disease presented with intermittent bilateral floaters following a COVID infection four months earlier. Vision was 20/40 OD and 20/16 OS.
Optos color imaging shows bilateral temporal fibrosed retinal neovascularization (sea fans) associated with some localized vitreous blood. The right eye had a significant epimacular membrane with traction, with a milder adherent membrane in her left eye.
Fluorescein angiography shows bilateral far temporal ischemia with leaking neovascularization. Some nasal neovascularization is also noted in her left eye. Ultrawidefield OCT shows vitreous traction adherent to the neovascularization.
We elected to closely follow her for now with observation.
Learning Points:
Patients with sickle cell disease develop symptoms throughout their bodies as stiff, 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.
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 sectoral (Farber et al Arch Ophthalmology 1991;109:363-367) or circumferential (Kimmel et al Ophthalmology 1986;93:1429-1434) scatter laser photocoagulation is somewhat controversial since, unlike more typical proliferative retinopathies (i.e., from diabetes and branch retinal vein occlusions), these new vessels tend to auto-infarct with a good natural history for untreated disease (Downes et al Ophthalmology 2005;112:1869-1875).
