Aims: To investigate the prevalence of sickle cell maculopathy (SCM), associations with age, sex, genotype and 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-widefield images of the macula and retina were reviewed for 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 one eye of 40 cases (54.1%) or in 67 of all eyes (42.3%). SCM prevalence was 54.8%, 62.5% and 25% for HbSS, HbSC and HbS/BThal or other genotypes respectively. SCM was observed in 41 (39.4%) of the eyes with PSR stages 0,1,2 and 21 (51.2%) of the eyes with PSR stages 3,4,5. 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 eyes of adults with sickle cell disease. The prevalence is similar for both HbSS and HbSC genotypes and is not related to the PSR stage. High-contrast distance visual acuity is typically preserved.
Authors Haroen Sahak, Mohammed Saqalain, Pooi Lott, Martin Mckibbin