CAJ | 학술논문

[目的]观察不同病变时期Best卵黄样黄斑营养不良(BVMD)的光相干断层扫描(OCT)图像特征.[方法]临床确诊为BVMD的28例患者56只眼纳入研究.所有患者常规行最佳矫正视力、裂隙灯显微镜、直接检眼镜、前置镜、眼底照相、眼电图、荧光素眼底血管造影检查.56只眼中,0期8只眼,Ⅰ期2只眼,Ⅱ期10只眼,Ⅱa期12只眼,Ⅲ期6只眼,Ⅳa期6只眼,Ⅳb期5只眼,Ⅳc期7只眼.患者同时行OCT检查,观察不同病变时期的OCT图像特征.[结果]OCT检查发现,0期患眼黄斑区未见明显异常.Ⅰ期患眼黄斑区结构正常,视网膜色素上皮(RPE)下小团块强反射病灶,可见浆液性视网膜脱离.Ⅱ、Ⅱa期患眼RPE-脉络膜毛细血管复合体(ORCC)呈弥漫性增厚隆起信号增强,黄色物质位于光感受器细胞内外节连接( IS/OS)与RPE层之间；Ⅱa期患眼RPE-ORCC增厚区域小,呈圆锥样隆起有弱反射区围绕.Ⅲ期患眼病灶上半部分表现为弱反射区,下半部分黄色物质表现为强反射并堆积于IS/OS与RPE层之间.Ⅳa期患眼病灶处神经感觉层下为无反射区,边界清晰,其下RPE-ORCC变薄,IS/OS光带及外界膜缺失.Ⅳb期患眼纤维化病灶RPE层增厚、隆起,呈强反射,视网膜神经上皮变薄和其上方广泛的IS/OS光带缺失.Ⅳc期患眼RPE连续性中断,可伴有黄斑部水肿.[结论]BVMD病灶中的黄色物质位于IS/OS与RPE层间,表现为强反射.Ⅱa期～Ⅳ期BVMD患眼的OCT典型图像特征为外核层与RPE层之间大小不一的腔隙样弱反射区伴或不伴有同一区间锥形强反射灶.
[목적]관찰불동병변시기Best란황양황반영양불량(BVMD)적광상간단층소묘(OCT)도상특정.[방법]림상학진위BVMD적28례환자56지안납입연구.소유환자상규행최가교정시력、렬극등현미경、직접검안경、전치경、안저조상、안전도、형광소안저혈관조영검사.56지안중,0기8지안,Ⅰ기2지안,Ⅱ기10지안,Ⅱa기12지안,Ⅲ기6지안,Ⅳa기6지안,Ⅳb기5지안,Ⅳc기7지안.환자동시행OCT검사,관찰불동병변시기적OCT도상특정.[결과]OCT검사발현,0기환안황반구미견명현이상.Ⅰ기환안황반구결구정상,시망막색소상피(RPE)하소단괴강반사병조,가견장액성시망막탈리.Ⅱ、Ⅱa기환안RPE-맥락막모세혈관복합체(ORCC)정미만성증후륭기신호증강,황색물질위우광감수기세포내외절련접( IS/OS)여RPE층지간；Ⅱa기환안RPE-ORCC증후구역소,정원추양륭기유약반사구위요.Ⅲ기환안병조상반부분표현위약반사구,하반부분황색물질표현위강반사병퇴적우IS/OS여RPE층지간.Ⅳa기환안병조처신경감각층하위무반사구,변계청석,기하RPE-ORCC변박,IS/OS광대급외계막결실.Ⅳb기환안섬유화병조RPE층증후、륭기,정강반사,시망막신경상피변박화기상방엄범적IS/OS광대결실.Ⅳc기환안RPE련속성중단,가반유황반부수종.[결론]BVMD병조중적황색물질위우IS/OS여RPE층간,표현위강반사.Ⅱa기～Ⅳ기BVMD환안적OCT전형도상특정위외핵층여RPE층지간대소불일적강극양약반사구반혹불반유동일구간추형강반사조.
[Objective] To observe the optical coherence tomography (OCT) features of Best vitellilorm macular dystrophy (BVMD) at different stages.[Methods] Twenty-eight BVMD patients (56eyes) were enrolled in this study.All the patients were examined for visual acuity,slit-lamp microscopy,direct ophthalmoscope,fundus photography,electrooculogram,fundus fluorescein angiography (FFA) and OCT.Fifty-six eyes were classified into stage 0 (eight eyes) 、Ⅰ (two eyes)、Ⅱ (10 eyes)、Ⅱ a (12 eyes)、Ⅲ(six eyes) 、Ⅳa (six eyes)、Ⅳb (five eyes) and Ⅳc (seven eyes) accordingly.The OCT features of BVMD at different stages were observed.[Results] The OCT results showed that the macular area was normal in eyes of stage 0; disturbance of retinal pigment epithelium (RPE) and subretinal hyporeflective area were found in eyes of stage Ⅰ; the location of the yellowish material between RPE and the inner segment and outer segment (IS/OS) with normal appearance in RPE and IS/OS interface were found in eyes of stage Ⅱ.In all the other progressing stages from Ⅱ a、Ⅲ and Ⅳ,the vitelliform material appeared as a thicker highly reflective lesion located between the outer nuclear layer and RPE layer,usually accompanied by optical hyporeflective lesion.Images of stage Ⅳ c were in similar appearance besides edema of retina.OCT images of Ⅳ b stage were demonstrated atrophy of retinal layer and IS/OS loss with fibrosis.[Conclusion]s OCT demonstrated the location of the yellowish material between RPE and IS/OS.Optical hyporeflective lesion between the outer nuclear layer and RPE layer accompanied thicker highly reflected lesion might be the characteristic image in stages Ⅱ a to Ⅳ of BVMD.