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Akosua Nti, Adrienne Scott; Natural history of Proliferative Sickle Cell Retinopathy. Invest. Ophthalmol. Vis. Sci. 2019;60(9):4048.
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© ARVO (1962-2015); The Authors (2016-present)
Proliferative sickle cell retinopathy (PSR) follows a well-characterized sequence of vascular remodeling, beginning with peripheral retinal arteriolar occlusions and arteriovenous anastomoses, followed by development of pathologic neovascularization. Advanced stages of PSR may then progress to vision loss from vitreous hemorrhage and retinal detachment. Published literature regarding the natural history of PSR, specifically, over what time frame vascular remodeling may progress through the various stages, is limited, and has not been described with contemporary imaging techniques. The aim of this study was to use ultra-widefield fluorescein angiography (UWF-FA) to determine progression of sickle cell retinopathy by stage, with or without treatment, and to characterize the types of vascular borders that may predispose to progression.
Retrospective natural history study of sixty one patients with sickle cell disease who were followed with complete dilated examinations and UWF-FA. Sequential UWF-FAs were graded by a masked retina-trained physician, and evaluated for stage of proliferative sickle cell retinopathy, type of proliferative sickle cell retinopathy progression (linear vs nonlinear), vascular border characteristics using Penman border classification system, visual acuity changes, and systemic associations (Hb SC vs Hb SS).
One hundred and twenty eyes of 61 patients followed with serial dilated examinations and UWF-FA were evaluated. Mean age was 31.4 years (range 19-56 years). Sixty percent were male. Sixty percent of patients were HbSS with 40% HbSC and HbS-thalassemia. Mean follow up was 32 months (range 2-84 months). Staging of UWF-FA images showed 16% without sickle cell retinopathy, 13% stage 1, 29% stage 2, 34% stage 3, 9% stage 4, and 5% stage 5. Forty percent of patients received scatter laser photocoagulation, 10% received vitrectomy for non clearing vitreous hemorrhage with or without anti-VEGF concurrent therapy, 10% underwent retinal detachment repair, and 50% were monitored with observation only. Twenty percent of patients lost central vision during follow up duration secondary or vitreous hemorrhage.
Understanding the natural history of proliferative sickle cell retinopathy will allow for more accurate disease staging, prognosis, and management. Longitudinal serial UWF-FA imaging demonstrates utility in imaging peripheral retinal vascular changes over time in patients with PSR.
This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.
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