Color vision defects in sickle cell anemia

The Farnsworth-Munsell 100-hue test was used to compare color vision in patients with sickle cell anemia, 20/20 visual acuity, and mild to severe peripheral retinopathy, but without posterior retinal vessel involvement, with matched normal controls. Patients with sickle cell anemia had significantly more blue-yellow and mixed color vision defects and significantly higher Farnsworth-Munsell 100-hue scores than did controls. There were no significant associations between color vision defects and the severity of peripheral retinal findings or the diameter of the foveal avascular zone. These results suggest that color vision defects may exist in patients with sickle cell anemia in the absence of ophthalmoscopic retinal changes in the posterior fundus.