Department of Ophthalmology, Renji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China.
Department of Nephrology, Ruijin Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China.
Lasers Med Sci. 2022 Feb;37(1):269-277. doi: 10.1007/s10103-020-03242-5. Epub 2021 Jan 6.
To examine the retinal and choroidal changes in patients with Fabry disease (FD) using optical coherence tomography angiography (OCTA). FD patients and age- and sex-matched healthy subjects were enrolled. A detailed ophthalmological examination was performed for all participants. The retinal thickness, ganglion cell layer with inner plexiform layer (GCIPL) thickness, choroidal thickness (CT), vessel length density (VLD), vessel perfusion density (VPD), and foveal avascular zone (FAZ) were analyzed in a detailed way with OCTA. Moreover, all FD patients underwent several laboratory tests to evaluate systemic conditions. A total of 54 subjects comprising 26 FD patients and 28 normal controls were enrolled. The retinal thickness, GCIPL thickness, and FAZ area showed no significant differences between the two groups (all P > 0.05). Only the superior CT in FD patients was significantly thinner than that in the normal subjects (P = 0.040). The macular VLD and VPD in the FD group were significantly reduced compared with the healthy controls (P = 0.026, P = 0.008). The macular VLD in FD patients had no significant correlations with different laboratory results (all P > 0.05), while the macular VPD were negatively correlated with creatinine (r = - 0.432, P = 0.028) and cystatin C (r = - 0.422, P = 0.032). FD patients may have retinal vascular dropout and choroidal vascular alterations. Analysis of vessel density using OCTA might be useful in the clinical assessment in FD patients.
使用光相干断层扫描血管造影术 (OCTA) 检查法布里病 (FD) 患者的视网膜和脉络膜变化。招募 FD 患者和年龄、性别匹配的健康受试者。对所有参与者进行详细的眼科检查。使用 OCTA 详细分析视网膜厚度、神经节细胞层内丛状层 (GCIPL) 厚度、脉络膜厚度 (CT)、血管长度密度 (VLD)、血管灌注密度 (VPD) 和中心凹无血管区 (FAZ)。此外，所有 FD 患者均进行了多项实验室检查以评估全身状况。共纳入 54 名受试者，包括 26 名 FD 患者和 28 名正常对照者。两组间视网膜厚度、GCIPL 厚度和 FAZ 面积无显著差异（均 P > 0.05）。仅 FD 患者的上方 CT 明显比正常对照组薄（P = 0.040）。FD 组的黄斑 VLD 和 VPD 明显低于健康对照组（P = 0.026，P = 0.008）。FD 患者的黄斑 VLD 与不同的实验室结果无显著相关性（均 P > 0.05），而黄斑 VPD 与肌酐呈负相关（r = - 0.432，P = 0.028）和胱抑素 C（r = - 0.422，P = 0.032）。FD 患者可能存在视网膜血管丢失和脉络膜血管改变。使用 OCTA 分析血管密度可能有助于 FD 患者的临床评估。
