Retina Division, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Wilmer Biostats Center, Department of Biostatistics, Johns Hopkins University, Baltimore, Maryland, USA.
Am J Ophthalmol. 2020 Jan;209:88-98. doi: 10.1016/j.ajo.2019.08.019. Epub 2019 Aug 29.
To compare the severity of macular vascular changes in children with sickle cell disease (SCD) vs age- and race-matched controls.
Cross-sectional study.
Children (<18 years old) with HbSS and HbS variant (HbSC and HbS thalassemia) genotypes, and their age- and race-matched controls, were recruited between January 2017 and December 2018. All subjects underwent optical coherence tomography angiography (OCTA) scans centered on the fovea and temporal macula. Retinal thickness, superficial capillary plexus (SCP) and deep capillary plexus (DCP) vessel density (VD), and foveal avascular zone (FAZ) size were measured and compared between HbSS and HbS variant vs controls.
Thirty-four HbSS, 34 HbS variant (Goldberg staging 0-3 for SCD eyes), and 24 control eyes (total 48 children, aged 5-17 years) were included. Total VD (3-mm ETDRS circle) was lower in HbS variant eyes than in controls for both the SCP (42.9% vs 47.7%, P = .02) and DCP (47.4% vs 52.6%, P = .01). In HbSS eyes, VD was lower in the DCP (47.7%, P = .008) but not in the SCP (45.5%, P = .5), compared to controls. A higher proportion of HbSS (n = 18, 55%) than HbS variant eyes (n = 9, 26%) had pathologic areas of retinal thinning associated with SCP and DCP flow loss (P = .03). However, retinal thickness measurements and FAZ size did not differ between either HbSS or HbS variant group vs controls.
Children with SCD have similar retinal thickness but less dense vasculature on OCTA compared to age and race-matched controls, suggesting that microvascular insult may precede structural thinning.
比较镰状细胞病（SCD）患儿与年龄和种族匹配的对照组之间黄斑血管变化的严重程度。
横断面研究。
2017 年 1 月至 2018 年 12 月期间，招募了 HbSS 和 HbS 变异体（HbSC 和 HbS 地中海贫血）基因型的儿童（<18 岁）及其年龄和种族匹配的对照组。所有受试者均接受了黄斑中心凹和颞侧黄斑的光学相干断层扫描血管造影（OCTA）扫描。测量并比较了 HbSS 和 HbS 变异体与对照组之间视网膜厚度、浅层毛细血管丛（SCP）和深层毛细血管丛（DCP）血管密度（VD）以及中心凹无血管区（FAZ）大小。
共纳入 34 例 HbSS、34 例 HbS 变异体（SCD 眼 Goldberg 分期 0-3）和 24 例对照组（共 48 例儿童，年龄 5-17 岁）。与对照组相比，HbS 变异体眼的 SCP（42.9%比 47.7%，P=.02）和 DCP（47.4%比 52.6%，P=.01）的总 VD（3mm ETDRS 环）均较低。与对照组相比，HbSS 眼的 DCP（47.7%，P=.008）而非 SCP（45.5%，P=.5）的 VD 较低。与 HbS 变异体眼（n=9，26%）相比，更多的 HbSS 眼（n=18，55%）存在与 SCP 和 DCP 血流丧失相关的视网膜变薄的病理性区域（P=.03）。然而，HbSS 或 HbS 变异体组与对照组之间的视网膜厚度测量和 FAZ 大小均无差异。
与年龄和种族匹配的对照组相比，SCD 患儿的视网膜厚度相似，但 OCTA 上的血管密度较低，提示微血管损伤可能先于结构变薄。
