X连锁视网膜劈裂症眼底蜂巢样改变的临床特征分析

Clinical characteristics of retinal honeycomb appearance in patients with X-linked retinoschisis

目的探讨X连锁视网膜劈裂症眼底视网膜蜂巢样改变的临床特点。设计回顾性病例系列。研究对象2017年12月至2020年1月在北京同仁医院确诊的43例(85眼)X连锁视网膜劈裂患者。方法回顾患者的临床病例资料。根据超广角眼底照相和病历记录对视网膜蜂巢样改变的特点进行总结分析。主要指标视网膜蜂巢样改变的形态、位置、发生率及其与视力、其他眼底表现和并发症的关系。结果20例(46.5%)、31眼(36.5%)存在蜂巢样改变,9例(45%)为单眼,11例(55%)为双眼。有、无蜂巢样改变的诊断年龄无统计学差异。蜂巢样改变的出现位置为颞上象限(28眼,90.3%),颞下(16眼,51.6%)、鼻下(5眼,16.1%)、鼻上(4眼、12.9%)。有蜂巢样改变眼最佳矫正视力(logMAR)显著低于无蜂巢样改变眼(0.82±0.14、0.55±0.09,t=2.153,P=0.035)。蜂巢样改变与周边视网膜劈裂和外层孔之间存在正相关性(r=0.377,P=0.000;r=0.278,P=0.08)。有蜂巢样改变眼并发内层孔、玻璃体积血、视网膜脱离的比例较无蜂巢样改变的眼更高(74.2%、59.3%;32.3%、20.4%;32.3%、22.2%),但无统计学意义。结论至少1/3的X连锁视网膜劈裂患者眼底存在蜂巢样改变。存在此改变者视力更差,内层孔、玻璃体积血、视网膜脱离等并发症比例更高。

Objective To investigate the clinical characteristics of retinal honeycomb appearance in patients with X-linked retinoschisis.Design Retrospective case series.Participants 43 patients(85 eyes)diagnosed as X-linked retinoschisis in Beijing Tongren Hospital from December,2017 to January,2020 were included.Methods Ultra-wide-field fundus imaging and examination note were used to review and analyze the clinical characteristics of retinal honeycomb appearance.Main Outcome Measures Morphology,location,incidence of the retinal honeycomb appearance and its association with visual acuity,other peripheral fundus changes and complications.Results The retinal honeycomb appearance was found in 20 cases(46.5%),31 eyes(36.5%).Of which,9 cases(45%)were monocular and 11 cases(55%)were binocular.There was no statistical difference of the age at diagnosis between groups with or without honeycomb appearance.There was a definite predilection for the superior temporal quadrant(28 eyes,90.3%),followed by the inferior temporal(16 eyes,51.6%),the inferior nasal(5 eyes,16.1%)and the superior nasal quadrant(4 eyes,12.9%).The best corrected visual acuity(logMAR)of eyes with honeycomb appearance were significantly lower than eyes without honeycomb appearance(0.82±0.14 VS 0.55±0.09,t=2.153,P=0.035).The honeycomb appearance was significantly associated with both peripheral retinoschisis and outer retinal hole(r=0.377,P=0.000;r=0.278,P=0.08).Eyes with honeycomb appearance had a higher rate of inner retinal hole,vitreous hemorrhage and retinal detachment than eyes without the honeycomb appearance(74.2%VS 59.3%;32.3%VS 20.4%;32.3%VS22.2%),but did not reach statistical significance.Conclusions Retinal honeycomb appearance could be found in at least 1/3 of the patients with X-linked retinoschisis.Eyes with honeycomb appearance had worse BCVA and higher rate of inner retinal holes and complications like vitreous hemorrhage and retinal detachment.