Update and review of the current medical and surgical management of sickle cell retinopathy

BACKGROUND: Sickle cell retinopathy (SCR) is a well-documented and potentially vision-threatening presentation of sickle cell disease (SCD). In this article, we provide a comprehensive review of the management options for non-proliferative sickle cell retinopathy (NPSR) and proliferative sickle cell retinopathy (PSR) based on the existing ophthalmic literature. MAIN TEXT: The mainstay of NPSR treatment focuses on preventing progression to PSR by identifying and altering modifiable risk factors. Once NPSR progresses to PSR, suppression of vascular endothelial growth factor (VEGF) expression with laser photocoagulation or intravitreal anti-VEGF injections can be considered. While no standard criteria exist for timing and type of intervention, both treatment modalities have been utilized for advanced PSR. In contemporary practice, scatter laser photocoagulation is performed far more commonly than the historically described feeder-vessel photocoagulation. Surgical management typically includes pars plana vitrectomy (PPV), scleral buckle (SB), or combined PPV-SB and are generally indicated in PSR for non-clearing vitreous hemorrhage, tractional or rhegmatogenous retinal detachment (RD), and epiretinal membrane formation. CONCLUSION: There is currently no consensus on standard guidelines for the management of SCR. Evidence suggests that surgical intervention can improve vision in advanced stages of PSR and that anti-VEGF therapy may have a role in treatment. However, studies in the ophthalmic literature are limited by relatively small sample sizes and difficulty accounting for a patient's prior medical or surgical interventions. Additionally, more robust studies are required to determine the long-term efficacy and safety of anti-VEGF in SCR. A multidisciplinary team approach to SCR and SCD remains the cornerstone of management for this systemic disease.