Sickel Cell Retinopathy - A Young Patient

Sickle cell disease (SCD) is a hereditary condition most often affecting patients of African ancestry. It causes an abnormality in hemoglobin, an oxygen carrying protein in the blood. This leads to irregular crescent-shaped red blood cells that can cause vascular occlusion in the eyes. Some effects of an occlusion include hemorrhages, neovascularization and retinal fibrosis. This 11-year-old patient presented with a history of sickle cell disease and complained of blurred vision in both eyes. His visual acuity was 20/125 in the right eye and 20/32 in the left. Fundus examination confirmed he had retinal pigment epithelium atrophy in his right eye. Optical coherence tomography (OCT) scans show macular atrophy nasally (yellow arrow) and an outer retinal hyperreflective scar in the fovea (red arrow). In the left eye, there was thinning in the retinal layers (blue arrow) but no scar. Fundus photos were taken (as shown), but no lesions were visible by this modality. No ocular treatment was needed. He was asked to return within 1-2 years or as needed if new symptoms were to arise.