目的 探讨光学相干断层扫描(OCT)、荧光素眼底血管造影(FFA)、超声及及视网膜电流图(ERG)在先天性视网膜劈裂症(XLRS)中的诊断价值.方法 对6例(12只眼)的OCT、FFA、超声及ERG的检查结果进行回顾性分析,其中5例(10只眼)行FFA及ERG检查,6例(12只眼)行超声及黄斑区OCT检查.结果 XLRS患者12只眼均发生黄斑区视网膜劈裂,B型超声表现为视盘颞侧即黄斑区球壁未见明显异常或稍增厚,A型超声表现为与基波基本垂直的窄高波峰;ERG表现为a波振幅正常或轻度下降,b波振幅明显下降或消失,呈负波形ERG,b/a变小;OCT表现为黄斑中心凹形态消失,视网膜神经上皮层增厚,层间可见多条柱状组织将其分成多个囊样暗区;FFA表现为黄斑区斑驳状高荧光;2例3只眼(25％)合并周边部视网膜劈裂,B型超声表现为玻璃体内细且较光滑的强回声光带与球壁相连,与球后壁间为无回声暗区;FFA表现为伴有颞侧、颞下方及下方视网膜稍隆起,其上血管稍迂曲,视网膜下斑驳状高荧光.结论 超声、ERG、OCT及FFA等检查在XLRS诊断中具有重要的临床应用价值,对于视力低下尤其是矫正视力未见提高的患者,应散瞳进行全面、详细的眼底检查,结合其影像学及电生理的特点明确诊断,以减少漏诊、误诊.%Objective To investigate the clinical value of optical coherence tomography (OCT),fundus fluorescence angiography (FFA),ultrasonic imaging and electroretinogram (ERG) detection to X-linked juvenile retinoschisis (XLRS).Methods FFA and ERG detection results of 5 cases (10 eyes),OCT and ultrasonic imaging results of 6 cases (12 eyes) of XLRS were reviewed retrospectively.Results Twelve eyes of XLRS patients were foveal splitting,B-mode ultrasound showed optic disc temporal side of the macula ball wall showed no abnormalities or slightly thickened,A-mode ultrasound showed the fundamental basic vertical narrow high peaks; ERG manifested as a wave amplitude of normal or slightly decreased b-wave amplitude decreased or disappeared,was negative waveform ERG and b/a smaller; OCT showed foveal morphology disappeared,retinal thickening,interlaminar seen multiple columnar organization into multiple cystic dark areas; FFA showed the macular mottled high fluorescence; two cases,three eyes (25％) with peripheral retinal splitting,B-mode ultrasound showed smooth glare with ball wall echo connected with the ball after intramural anechoic dark area,FFA associated with the temporal side,a little bulge beneath the retina,on slightly tortuous blood vessels under the retina mottled fluorescence.Conclusions Ultrasonic imaging,ERG,OCT and FFA detection play the important roles in the diagnosis of XLRS.In order to reduce misdiagnosis and missed diagnosis,the patients,who have poor eyesight,especially vision correction have not seen an increase,we should be dilated to conduct a comprehensive,detailed fundus examination,and pay attention to the appearance of fundus and character of the imaging and electrophysiology of vision.
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