先天性视网膜劈裂症患眼视网膜形态特征及其对视力的影响

The relationship of retinal morphological features and visual acuity of X-linked juvenile retinoschisis

目的 观察先天性视网膜劈裂症（XLRS）患眼视网膜形态特征及其对视力的影响.方法 临床确诊为XLRS且尚未发生并发症的23例38只眼纳入研究.所有患眼均行最佳矫正视力（BCVA）及频域光相干断层扫描（SD-OCT）检查.BCVA采用国际标准视力表进行,将结果换算为最小分辨角对数视力（logMAR）记录.患眼平均logMAR BCVA为0.41±0.28.SD-OCT采用Topcon 3D-OCT 1000和Topcon DRI-OCT检查仪进行,观察患眼劈裂发生的层次.将劈裂形态分为外核层（ONL）与外丛状层（OPI）劈裂、内核层（INL）劈裂、神经节细胞层（GCL）与神经纤维层（NFL）劈裂3种.根据中心凹下扫描区域内有无光感受器内外节连接（IS/OS）层缺失将患眼分为IS/OS层缺失组、IS/OS层未缺失组.对比分析两组患眼的logMAR BCVA及中心凹厚度（FT）的差异.对比分析患眼logMAR BCVA与FT之间的相关性.结果 38只眼中,劈裂形态为ONL与OPL劈裂17只眼,占44.7％;INL劈裂38只眼,占100.0％;GCL与NFL劈裂13只眼,占34.2％.INL劈裂可以单独存在,也可与外层劈裂共存.劈裂累及2层或以上25只眼,占65.8％.IS/OS层缺失组、IS/OS层未缺失组平均logMAR BCVA为0.32±0.17、0.44士0.22.两组间平均logMAR BCVA比较,差异有统计学意义（t=6.531,P=0.008）.IS/OS层缺失组,IS/OS层未缺失组平均FT分别为（517.10±96.92）、（523.08士72.84）μm.两组间平均FT比较,差异无统计学意义（t=2.282,P=0.061）.相关性分析结果显示,患眼logMAR BCVA与FT无相关性（r=0.200,P=0.929）.结论 XLRS患眼的劈裂存在于视网膜多个层次,以1NL最为常见.IS/OS层缺失可能是引起患眼视力下降的主要原因.

Objective To observe the optical coherence tomography （OCT） features of X-linked juvenile retinoschisis （XLRS） and its relationship and visual acuity.Methods Twenty-three XLRS patients （38 eyes） were enrolled in this study.All patients underwent best corrected visual acuity （BCVA） and frequency-domain optical coherence tomography （SD-OCT） examination.The international standard visual acuity chart was used for BCVA test and the results were then converted into logarithm of the minimum angle of resolution （logMAR） visual acuity.The mean logMAR BCVA of all patients was 0.41 ± 0.28.Topcon 3D-OCT 1000 and Topcon DRLOCT were applied to detect the retinal layers of retinoschisis.Based on the OCT features,retinoschisis was classified into 3 types,including splitting between outer nuclear layer （ONL） and outer plexiform layer （OPL）,inner nuclear layer morphology （INL） splitting,splitting between ganglion cell layer （GCL） and nerve fiber layer （NFL）.Based on the OCT features of fovea,there were photoreceptor inner segment （IS）/outer segment （OS） presence group and IS/OS absence group.Results There were 17 eyes （44.7％） with splitting between ONL/OPL,38 eyes （100％） with 1NL splitting,13 eyes （34.2％） with splitting between GCL/NFL.INL splitting can coexist with splitting in other retinal layers.There were 25 eyes （65.8％） with splitting in 2 or more retinal layers.The mean logMAR BCVA were 0.32±0.17 and 0.44±0.22 respectively in the IS/OS absence group and presence group,the difference was statistically significant （t=6.531,P =0.008）.The mean fovea thickness （FT） were（517.10 ± 96.92） and （523.08±72.84） μm respectively in the IS/OS absence group and presence group,the difference was not statistically significant （t=2.282,P=0.061）.There was no correlation between logMAR BCVA and FT （r=0.200,P=0.929）.Conclusions In patients with XLRS,the foveomacular schisis involved in multiple retinal layers and most frequently in the INL.The defect of IS/OS is the important reason of the low visual acuity.