IMPORTANCE Patients with sickle cell disease may develop various macular vascular abnormalities that have not been described previously and can be seen using optical coherence tomographic angiography. OBSERVATIONS Ten eyes from 5 consecutive patients (3 men and 2 women) with sickle cell disease (4 patients with hemoglobin SS disease and 1 patient with hemoglobin SC disease) were included. The mean age was 37.6 years. Five of 10 eyes (50%) had retinal thinning that was identified using spectral-domain optical coherence tomography. Each of these eyes had corresponding loss of vascular density in the superficial or deep retinal plexus (or both). CONCLUSIONS AND RELEVANCE Optical coherence tomographic angiography provides a novel way to view the retinal vasculature, including the superficial and deep capillary plexus, and may provide a sensitivemethod for identifying macular vascular abnormalities in patients with sickle cell disease.
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