Abstract
Purpose :
Sickle cell disease (SCD) is the most common inherited blood disorder with rare but potentially visually devastating ophthalmic manifestations. Growing evidence suggests imaging modalities including spectral-domain optical coherence tomography (SD-OCT), OCT angiography (OCTA) and ultra-wide field fluorescein angiography (UWF-FA) to be effective in detecting retinal microvascular abnormalities in SCD. Additionally, scatter laser photocoagulation is historically the preferred intervention for proliferative sickle cell retinopathy (PSR) while anti-vascular endothelial growth factor (anti-VEGF) intravitreal injections have also shown efficacy in the literature. No evidence based guidelines currently exist for PSR imaging and treatment. We therefore queried retina specialists on evaluation and management preferences to determine contemporary practice patterns for sickle cell retinopathy.
Methods :
An institutional review board-approved survey was distributed to U.S. and international retina specialists. Clinicians were asked which retinal imaging modalities they obtain for new patients with SCD, how SCD patients are referred to their practice, treatment preferences for areas of retinal non-perfusion, neovascularization, and vitreous hemorrhage from PSR, and when they may use anti-VEGF intravitreal injections.
Results :
95 retina specialists completed the survey with 50.53% having greater than 20 years of clinical experience. Non-vitreoretinal ophthalmologists were the most common referral source (75.79%). 36.84% reported ordering imaging on the first visit only if abnormalities exist on fundus exam, while SD-OCT and UWF-FA were the most commonly obtained tests (61.05% and 41.05% respectively). 17.89% reported ordering OCTA on the initial visit. 63.16% would observe and not treat for peripheral non-perfusion. For peripheral retinal neovascularization, 66.32% treat with scatter laser photocoagulation, 5.26% with anti-VEGF, and 13.68% would observe. For vitreous hemorrhage not involving central vision, 70.53% treat with scatter laser, while 16.84% treat with anti-VEGF injections. 48.42% reported not using anti-VEGF injections for SCD.
Conclusions :
Scatter laser photocoagulation still remains the preferred therapy for vision-threatening PSR. Almost 20% of retina specialists report ordering OCTA on the initial visit. Anti-VEGF injections appear to be most utilized when vitreous hemorrhage is present.
This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.