Sickle cell retinopathy: What we now understand using optical coherence tomography angiography. A systematic review.

For over four decades, efforts have been underway for the evaluation of sickle cell retinopathy (SCR) in an attempt to identify peripheral high-risk vascular abnormalities based on Goldberg's classification (gold-standard) (1971). The macula is an area in the center of the retina that is responsible for high-resolution central vision and is also affected in SCR. With the development of new technologies for retinal imaging, the macula became a main focus of interest in the study of sickle cell disease (SCD). Optical coherence tomography angiography is a modern non-invasive method that allows qualitative and quantitative analysis of the retinal vascular network in the macular region. We identified 86 studies on SCD and optical coherence tomography angiography. Of those, 12 met the inclusion criteria for this systematic review. The prevalence of sickle cell maculopathy was 45.6% in the SCD population and it increased with age. We present the main vascular findings and discuss visual impairment and pathophysiology, genotypes, and peripheral high-risk vascular abnormalities. The role of the hematologist, general practitioner, and pediatrician in treatment is also reviewed.