Department of Ophthalmology, Massachusetts Eye and Ear, Harvard Medical School, Boston, Massachusetts.
Department of Ophthalmology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.
Ophthalmol Retina. 2024 Feb;8(2):184-194. doi: 10.1016/j.oret.2023.09.005. Epub 2023 Sep 9.
To longitudinally assess macular thickness and microvascular changes in children with sickle cell disease (SCD).
A retrospective consecutive series.
Children with SCD aged ≤ 18 years who had an ophthalmic examination at Boston Children's Hospital between January 1998 and August 2022.
Qualitative and quantitative analyses of both OCT and OCT angiography (OCTA) images were performed.
Total retinal thickness measured on macular OCT, superficial capillary plexus and deep capillary plexus (DCP) vessel density (VD), and foveal avascular zone (FAZ) area measured on 6- × 6-mm OCTA scans.
International Classification of Diseases, 10th Revision, code search identified 303 pediatric SCD patients who underwent ophthalmic examination during the study period. OCT and OCTA images were acquired on 104 (17.2%) and 60 (9.9%) eyes at presentation and on 159 (26.2%) and 100 (16.5%) eyes at final visit, respectively. Overall, temporal retinal thinning was noted qualitatively in 35.6% of SCD patients at presentation and 39.6% at final visit. Of those patients with macular thinning, 94.6% and 90.5% had peripheral sickle cell retinopathy (SCR) at presentation and final visit. On quantitative OCT analysis, HbSS eyes had a lower retinal thickness in the fovea and temporal parafovea compared with HbSC (P < 0.05). Eyes with peripheral SCR had a larger FAZ at presentation compared with eyes without peripheral SCR (P = 0.004), a lower DCP VD at final visit in the inferior temporal macula (P = 0.03), and a higher DCP VD at final visit in the superior nasal macula (P = 0.01). Eighty eyes of 40 patients had OCT, and 34 eyes of 20 patients had both OCT and OCTA images acquired at both initial and final visits. At final visit, retinal thickness decreased at the fovea, inferior perifovea, and temporal perifovea compared with presentation (P < 0.05). In parallel, VD DCP in the superonasal quadrant increased at final visit (P = 0.03).
Macular retinal thinning was progressive and observed in eyes with and without peripheral SCR. Over time, there was a compensatory increase in DCP VD in the nasal macula on OCTA.
FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
纵向评估镰状细胞病（SCD）患儿的黄斑厚度和微血管变化。
回顾性连续系列。
1998 年 1 月至 2022 年 8 月在波士顿儿童医院接受眼科检查、年龄≤18 岁的 SCD 患儿。
对 OCT 和 OCTA（OCTA）图像进行定性和定量分析。
黄斑 OCT 测量的总视网膜厚度、浅层毛细血管丛和深层毛细血管丛（DCP）血管密度（VD）、6×6mm OCTA 扫描测量的中心凹无血管区（FAZ）面积。
国际疾病分类第 10 版编码检索确定了 303 名在研究期间接受眼科检查的儿科 SCD 患者。在就诊时和最后一次就诊时，分别有 104 只（17.2%）和 60 只（9.9%）眼获得 OCT 和 OCTA 图像，159 只（26.2%）和 100 只（16.5%）眼获得最终就诊时的 OCT 和 OCTA 图像。总体而言，35.6%的 SCD 患者在就诊时和 39.6%的患者在最后一次就诊时出现颞侧视网膜变薄。在出现黄斑变薄的患者中，94.6%和 90.5%的患者在就诊时和最后一次就诊时均存在周边镰状细胞视网膜病变（SCR）。定量 OCT 分析显示，与 HbSC 相比，HbSS 眼的黄斑区和颞侧旁中心凹的视网膜厚度较低（P<0.05）。与无周边 SCR 的眼相比，有周边 SCR 的眼在就诊时 FAZ 较大（P=0.004），在最终就诊时下方颞侧黄斑 DCP VD 较低（P=0.03），上方鼻侧黄斑 DCP VD 较高（P=0.01）。40 名患者中的 80 只眼接受了 OCT 检查，20 名患者中的 34 只眼在初始和最终就诊时均同时接受了 OCT 和 OCTA 检查。在最后一次就诊时，与就诊时相比，黄斑、下旁中心凹和颞旁中心凹的视网膜厚度均减少（P<0.05）。同时，OCTA 中上方鼻侧象限的 DCP VD 增加（P=0.03）。
黄斑视网膜变薄是进行性的，在有和没有周边 SCR 的眼中均可观察到。随着时间的推移，OCTA 上鼻侧黄斑 DCP VD 代偿性增加。
