Sickle Cell Maculopathy: Prevalence, Associations and Impact on Visual Acuity

Abstract

Aims: To investigate the prevalence of sickle cell maculopathy (SCM), and associations with age, sex, genotype, proliferative sickle cell retinopathy (PSR) stage, and the impact on visual acuity. Methods: Age, sex, and visual acuity were recorded and spectral domain OCT and ultra-wide-field images of the macula and retina were reviewed in a consecutive series of 74 adults with sickle cell disease. Results: The median age was 37 years (range 19–73 years) and 36 cases (48.6%) were male. SCM was present in at least 1 eye of 40 cases (54.1%) or in 67 of all eyes (42.3%). SCM prevalence was 54.8%, 62.5%, and 25% for the HbSS, HbSC, and HbS/BThal or other genotypes, respectively. SCM was observed in 41 (39.4%) of the eyes with PSR stages 0, 1, and 2, and in 21 (51.2%) of the eyes with PSR stages 3, 4, and 5, respectively. Mild visual impairment or worse was present in 3 eyes (4.8%) with SCM but this was secondary to other pathology. Conclusion: SCM is a frequent finding in the eyes of adults with sickle cell disease. The prevalence is similar for the HbSS and HbSC genotypes and is not related to the PSR stage. High-contrast distance visual acuity is typically preserved.