Department of Ophthalmology, Bellvitge University Hospital, Barcelona, Spain.
Department of Ophthalmology, Sant Joan de Deu Hospital, Barcelona, Spain.
Eye (Lond). 2018 Apr;32(4):707-715. doi: 10.1038/eye.2017.281. Epub 2018 Jan 5.
PurposeTo explore the structural features of juvenile X-linked retinoschisis (XLRS) using swept-source-optical coherence tomography (SS-OCT) and optical coherence tomography angiography (OCT-A).DesignRetrospective, observational cross-sectional study.Patients and methodsNine patients (18 eyes) diagnosed with juvenile XLRS were included. SS-OCT and OCT-A were used to evaluate the characteristics of the inner/outer retina and the choroid.ResultsSS-OCT showed that the inner nuclear layer (INL) was the most commonly affected area (16/18 eyes; 89%). No significant differences in central macular thickness (CMT) or subfield choroidal thickness (SFCT) were evidenced between eyes (CMT: 364 μm in the right eye vs 320 μm in the left eye; SFCT: 305 vs 307 μm; P=0.895). Best-corrected visual acuity (BCVA) did not correlate with CMT (rs= -0.19; P=0.445) or SFCT (rs=0.06; P=0.795). BCVA was significantly correlated with the following defects: outer plexiform layer (OPL; rs=0.50; P=0.036); external limiting membrane (ELM; rs=0.65; P=0.003); ellipsoid portion of inner segment (EPIS; rs=0.67; P=0.002); and the cone outer segment tips (COST; rs=0.69; P=0.001). Schisis at the INL revealed a spoke-like pattern in the foveal region and a reticular pattern in the parafoveal region on en-face imaging. In cases in which the schisis affected the OPL, multiple polygonal hyporeflective cavities were observed in the foveal region.ConclusionsThe hyporeflective spaces on SS-OCT were primarily located at the INL and OPL. BCVA did not correlate with CMT or SFCT; however, ELM, EPIS, and COST defects were significantly correlated with worse BCVA. There was a positive correlation between age and SFCT.
目的 利用扫频源光学相干断层扫描（SS-OCT）和光相干断层扫描血管造影（OCT-A）探讨青少年性连锁性视网膜劈裂症（XLRS）的结构特征。
设计 回顾性、观察性横断面研究。
患者和方法 纳入 9 名（18 只眼）诊断为青少年 XLRS 的患者。使用 SS-OCT 和 OCT-A 评估内/外视网膜和脉络膜的特征。
结果 SS-OCT 显示内核层（INL）是最常受累的区域（16/18 只眼；89%）。右眼和左眼的黄斑中心厚度（CMT）或脉络膜下区厚度（SFCT）无显著差异（CMT：右眼 364μm，左眼 320μm；SFCT：右眼 305μm，左眼 307μm；P=0.895）。最佳矫正视力（BCVA）与 CMT （rs=-0.19；P=0.445）或 SFCT （rs=0.06；P=0.795）均无相关性。BCVA 与以下缺陷显著相关：外丛状层（OPL；rs=0.50；P=0.036）；外界膜（ELM；rs=0.65；P=0.003）；内节椭圆体段（EPIS；rs=0.67；P=0.002）；和圆锥体外节尖端（COST；rs=0.69；P=0.001）。INL 劈裂在 ff-OCT 上呈现出在中心凹区呈现辐条状，在旁中心凹区呈现网状的表现。当劈裂影响到 OPL 时，在中心凹区可以观察到多个多边形低反射性腔。
结论 SS-OCT 上的低反射性空间主要位于 INL 和 OPL。BCVA 与 CMT 或 SFCT 无相关性；然而，ELM、EPIS 和 COST 缺陷与更差的 BCVA 显著相关。年龄与 SFCT 呈正相关。
