Department of Ophthalmology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea.
Retina. 2022 Oct 1;42(10):1939-1949. doi: 10.1097/IAE.0000000000003539.
The study aimed to evaluate the macular microvasculature of X-linked retinoschisis (XLRS) and identify correlations between vascular changes, structural changes, and functional outcome.
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.
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.
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.
本研究旨在评估 X 连锁性视网膜劈裂症（XLRS）的黄斑微血管，并确定血管变化、结构变化与功能结果之间的相关性。
对经基因证实的 XLRS 患者和健康对照者进行全面眼科检查、散瞳眼底检查、光学相干断层扫描和光学相干断层扫描血管造影。使用光学相干断层扫描评估劈裂分布、外丛状层中断、光感受器层厚度和光感受器外节长度。使用光学相干断层扫描血管造影测量黄斑中心凹及旁中心凹区的血管血流密度和黄斑中心凹厚度。
2018 年 7 月至 2020 年 8 月共检查了 9 例 XLRS 患者的 17 只眼和 11 例对照者的 22 只眼。与对照组相比，XLRS 患者黄斑中心凹及旁中心凹区深层毛细血管丛的血流密度降低（P=0.014 和 0.001），而黄斑中心凹无血管区面积和周长显著增加（P=0.015 和 0.001）。尽管 XLRS 患者的外、全视网膜层明显增厚，但内层视网膜变薄，光感受器层厚度变薄，光感受器外节长度缩短（P<0.001 和 P<0.001）。深层毛细血管丛黄斑中心凹血流丢失、黄斑中心凹无血管区扩大、内层视网膜和光感受器层变薄以及光感受器外节长度缩短与最佳矫正视力相关。
X 连锁性视网膜劈裂症患者的深层毛细血管丛血流密度降低，黄斑中心凹无血管区形态可变，周长增大。光感受器的结构恶化最好地反映了退行性变化，而微血管改变与 XLRS 的功能结果有很大的相关性。
