Best卵黄样黄斑营养不良不同临床分期眼底影像学分析

Clinical analysis of vitelliform macular dystrophy in different Stage

目的探讨卵黄样黄斑营养不良不同临床分期的眼底表现及眼底荧光血管造影特征，了解卵黄样黄斑营养不良的病变发展情况。方法对2006年1月至2013年1月在云南省第二人民医院眼科就诊的24例（48只眼）卵黄样黄斑营养不良患者的临床资料，按病变进展进行分期，分析各期病变眼底彩照、眼底荧光血管造影、光相干断层扫描结果，并结合相关文献报道，总结该病发展转归及相应的病理生理变化。结果2只眼为0期，眼底影像学检查均正常。5只眼为I期：黄斑区色素紊乱，OCT显示RPE层间见低反射的小裂隙出现。25只眼为Ⅱ期：黄斑区出现卵圆形黄色隆起，眼底荧光血管造影表现为病变区域无荧光或弱荧光区，OCT显示RPE和光感受器层之间有一中等密度反射区域。12只眼为Ⅲ期，眼底卵黄样病变完整性破坏，眼底荧光血管造影显示病变区域荧光形态不规则，OCT显示RPE层增厚、断裂，高反射物质存留。4只眼为Ⅳ期：眼底表现黄斑病变RPE萎缩灶，眼底荧光血管造影显示为边界清晰的瘢痕染色所致的高荧光、合并脉络膜新生血管形成者，可见新生血管荧光渗漏呈强荧光；OCT显示为神经上皮层变薄，RPE层光反射不连续。结论多重眼底影像学技术的联合运用为认识和了解Best卵黄样黄斑营养不良的发病原因、发病部位、病变特征、发展及转归提供了有利的工具。

Objective To analyze the multimodal fundus imaging of vitelliform macular dystrophy. Methods Optical coherence tomography （OCT）, fluorescein fundus angiography （FFA） and flmdus photo- graph were carried out to the vitelliform macular dystrophy in 26 patients. Results Two eyes belonged to stage 0, ftmdus imaging were normal; 5 eyes belong to stage I, pigment disorders can be observed in macular, OCT showed small cracks in RPE; 25 eyes belonged to stage Ⅱ： oval yellow lesions could be observed in macular area, FFA showed no fluorescence or weak fluorescence, OCT showed a medi- um-density reflective region between RPE and photoreceptor layer; 12 eyes belonged to stage Ⅲ, the in- tegrity of the yolk-like lesions was damaged, FFA revealed irregular fluorescence, OCT showed RPE lay- er thickened, highly reflective substance remained; 4 eyes belonged to stage Ⅳ： fundus showed macular degeneration and RPE atrophy, FFA revealed a clear boundary high fluorescence due to scar staining, flu- orescein leakage could be found in choroidal neovascularization, OCT showed thinning neurosensory lay- ers, RPE layer was not continuous. Condusions Combined use of multiple fundus imaging techniques provide a favorable tool to understand the characteristics of Best macular dystrophy.