Abstract

Sickle cell retinopathy (SCR) is classified by Goldberg based on peripheral vascular changes. Ultra-wide field (UWF) imaging has enhanced visualization of the peripheral retina. However, there is no consensus on the optimal imaging technique for the screening of SCR. We performed a monocentric observational cross-sectional study to compare UWF fundus photography (UWF-FP) with UWF angiography (UWF-FA). All patients who underwent UWF-imaging (Optos, PLC, Scotland, UK) for screening of sickle cell retinopathy between January 2016 and December 2019 were retrospectively included. Eyes with previous laser treatment or concomitant retinal disease were excluded. UWF-FP images were graded based on the Goldberg classification by four graders with various degrees of experience. UWF-FA pictures were reviewed by an independent retina specialist. Differences in Goldberg staging across UWF-FP and UWF-FA were assessed. A total of 84 eyes of 44 patients were included. Based on UWF-FA, most eyes were stage 2 (77.4%) and 19 were stage 3 (22.6%). The pre-retinal neovascularization detection sensitivity on UWF-FP was 52.6 to 78.9%, depending on the graders. UWF-FA led to a later Goldberg stage of retinopathy, in most cases from stage 1 to stage 2. Neovascularization (stage 3) was not detected by our graders on UWF-FP in 21.1 to 57.9% of eyes. UWP-FP tends to underestimate Goldberg stages of retinopathy compared with UWF-FA and is less accurate when detecting neovascularization in sickle cell retinopathy, which has a direct impact on therapeutic management and prognosis.

Highlights

  • Published: 11 February 2022Sickle cell disease (SCD) is one of the most common, worldwide monogenetic disorders caused by mutations in the β-globin gene

  • Sickle cell retinopathy is classified based on peripheral vascular changes

  • Since the development of the Goldberg classification system, ultra-wide field imaging has increased our understanding of sickle cell retinopathy (SCR) through greater visualization of the peripheral retina

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Summary

Introduction

Sickle cell disease (SCD) is one of the most common, worldwide monogenetic disorders caused by mutations in the β-globin gene. Vaso-occlusive complications affect almost all ocular structures, but sickle cell retinopathy (SCR) remains the most important in terms of frequency and visual impairment. Chronic retinal vascular ischemia secondary to rigid sickle-shaped red blood cells stimulate the production of proangiogenic factors such as vascular endothelial growth factor (VEGF) which can lead to pathologic retinal neovascularization [1,2]. The clinical course of sickle cell retinopathy is variable and dependent on different genotypes. It is known that hemoglobin SS patients are less likely to develop advanced stage retinopathy than patients with hemoglobin SC and other variant genotypes. Proliferative sickle cell retinopathy is reported to affect 3.5–30.2% of hemoglobin

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