Although once thought to be a benign condition, retinal vascular occlusive disease and proliferative retinopathy can occur with sickle cell trait (hemoglobin AS) when additional systemic diseases or trauma are present.
The authors discuss the ophthalmologic evaluation and clinical course of a 49-year-old woman with sickle cell trait and rheumatoid arthritis who presented with a cilioretinal artery occlusion.
The patient's laboratory evaluation showed both a high rheumatoid factor titer and a mild hypergammaglobulinemia, causing increased serum viscosity. The high level of sickle hemoglobin-42.3% (range in trait, 22%-46%)-increased serum viscosity, and lower cilioretinal artery perfusion pressure relative to the central retinal artery resulted in cilioretinal artery occlusion.
Isolated cilioretinal artery occlusions carry a good prognosis, and this patient recovered 20/20 visual acuity in the affected eye. The association between sickle cell trait and rheumatoid arthritis resulting in retinal vascular occlusive disease has not been reported previously. The presence of retinal vascular occlusion in sickle cell trait necessitates a medical evaluation for additional systemic diseases.