Abstract：
OBJECTIVE: The study aimed to evaluate the macular microvasculature of X-linked retinoschisis (XLRS) and identify correlations between vascular changes, structural changes, and functional outcome.
METHODS: Genetically confirmed XLRS patients and heathy control subjects underwent complete ophthalmic examination, dilated funduscopic examination, optical coherence tomography, and optical coherence tomography angiography. Schisis distribution, outer plexiform layer discontinuation, photoreceptor layer thickness, and photoreceptor outer segment length were reviewed using optical coherence tomography. Vascular flow density and foveal thickness at foveal and parafoveal area were measured using optical coherence tomography angiography.
RESULTS: A total of 17 eyes of 9 XLRS patients and 22 eyes of 11 control subjects were examined from July 2018 to August 2020. Flow density in the deep capillary plexus at foveal and parafoveal area decreased in XLRS patients compared with control subjects (P = 0.014 and 0.001, respectively), whereas foveal avascular zone area and perimeter remarkably increased (P = 0.015 and 0.001, respectively). Although outer and total retinal layers were significantly thicker in XLRS, inner retinal layer was thinner with reduced photoreceptor layer thickness and shortened photoreceptor outer segment length (P < 0.001 and P < 0.001, respectively). Foveal flow loss in deep capillary plexus, foveal avascular zone enlargement, thinner inner retina and photoreceptor layer thickness, and shortened photoreceptor outer segment length correlated with best-corrected visual acuity.
CONCLUSIONS: X-linked retinoschisis eyes exhibit decreased flow density in the deep capillary plexus and variable foveal avascular zone with enlarged perimeter. Structural deterioration of the photoreceptor best reflects the degenerative changes, whereas microvascular alteration shows considerable correlation with functional outcome in XLRS.
METHODS: Genetically confirmed XLRS patients and heathy control subjects underwent complete ophthalmic examination, dilated funduscopic examination, optical coherence tomography, and optical coherence tomography angiography. Schisis distribution, outer plexiform layer discontinuation, photoreceptor layer thickness, and photoreceptor outer segment length were reviewed using optical coherence tomography. Vascular flow density and foveal thickness at foveal and parafoveal area were measured using optical coherence tomography angiography.
RESULTS: A total of 17 eyes of 9 XLRS patients and 22 eyes of 11 control subjects were examined from July 2018 to August 2020. Flow density in the deep capillary plexus at foveal and parafoveal area decreased in XLRS patients compared with control subjects (P = 0.014 and 0.001, respectively), whereas foveal avascular zone area and perimeter remarkably increased (P = 0.015 and 0.001, respectively). Although outer and total retinal layers were significantly thicker in XLRS, inner retinal layer was thinner with reduced photoreceptor layer thickness and shortened photoreceptor outer segment length (P < 0.001 and P < 0.001, respectively). Foveal flow loss in deep capillary plexus, foveal avascular zone enlargement, thinner inner retina and photoreceptor layer thickness, and shortened photoreceptor outer segment length correlated with best-corrected visual acuity.
CONCLUSIONS: X-linked retinoschisis eyes exhibit decreased flow density in the deep capillary plexus and variable foveal avascular zone with enlarged perimeter. Structural deterioration of the photoreceptor best reflects the degenerative changes, whereas microvascular alteration shows considerable correlation with functional outcome in XLRS.
摘要：
目的：该研究旨在评估X连锁视网膜裂孔（XLRS）的黄斑微脉管系统，并确定血管变化之间的相关性，结构变化,和功能结果。
方法：经遗传证实的XLRS患者和健康对照受试者接受了完整的眼科检查，扩张眼底镜检查，光学相干层析成像，和光学相干断层扫描血管造影。Schisis分布，外丛状层中断，感光层厚度，使用光学相干断层扫描对感光体外段长度进行了回顾。使用光学相干断层扫描血管造影测量中央凹和旁凹区域的血管流量密度和中央凹厚度。
结果：从2018年7月至2020年8月，共检查了9例XLRS患者的17只眼和11例对照受试者的22只眼。与对照组相比，XLRS患者的中央凹和副凹区域深毛细血管丛的血流密度降低（分别为P=0.014和0.001），而中央凹无血管区面积和周长显着增加（分别为P=0.015和0.001）。尽管XLRS的视网膜外层和总视网膜层明显较厚，视网膜内层较薄，光感受器层厚度减少，光感受器外段长度缩短（分别为P<0.001和P<0.001）。深毛细血管丛中的中心凹流量损失，中央凹无血管区扩大，较薄的内部视网膜和感光层厚度，缩短的光感受器外段长度与最佳矫正视力相关。
结论：X连锁视网膜裂孔眼表现出深部毛细血管丛和可变的中央凹无血管区的血流密度降低，周长增大。感光体的结构退化最好地反映了退行性变化，而微血管改变与XLRS的功能结局有相当大的相关性。
方法：经遗传证实的XLRS患者和健康对照受试者接受了完整的眼科检查，扩张眼底镜检查，光学相干层析成像，和光学相干断层扫描血管造影。Schisis分布，外丛状层中断，感光层厚度，使用光学相干断层扫描对感光体外段长度进行了回顾。使用光学相干断层扫描血管造影测量中央凹和旁凹区域的血管流量密度和中央凹厚度。
结果：从2018年7月至2020年8月，共检查了9例XLRS患者的17只眼和11例对照受试者的22只眼。与对照组相比，XLRS患者的中央凹和副凹区域深毛细血管丛的血流密度降低（分别为P=0.014和0.001），而中央凹无血管区面积和周长显着增加（分别为P=0.015和0.001）。尽管XLRS的视网膜外层和总视网膜层明显较厚，视网膜内层较薄，光感受器层厚度减少，光感受器外段长度缩短（分别为P<0.001和P<0.001）。深毛细血管丛中的中心凹流量损失，中央凹无血管区扩大，较薄的内部视网膜和感光层厚度，缩短的光感受器外段长度与最佳矫正视力相关。
结论：X连锁视网膜裂孔眼表现出深部毛细血管丛和可变的中央凹无血管区的血流密度降低，周长增大。感光体的结构退化最好地反映了退行性变化，而微血管改变与XLRS的功能结局有相当大的相关性。
