OCTFFA超声及ERG在先天性视网膜劈裂症诊断中价值

The diagnostic value of OCT, FFA, ultrasonic imaging and ERG detection to XLRS

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 retrospec- tively. Results Twelve eyes of XLRS patients were foveal splitting, B-mode ultrasound showed op- tic 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 neg- ative waveform ERG and b/a smaller; OCT showed foveal morphology disappeared, retinal thicken- ing, 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 slight- ly 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 re- duce misdiagnosis and missed diagnosis, the patients, who have poor eyesight, especially vision cor-rection 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 electro- physiology of vision.