PDF(Pubmed)
Abstract：
UNASSIGNED: To describe the multimodal imaging features, including en face optical coherence tomography (OCT), of Bietti\'s crystalline dystrophy (BCD).
UNASSIGNED: Wide field fundus photography, autofluorescence (FAF) imaging, and cross sectional and en face OCT were performed in a case of BCD. The level of the crystals in the retina were analyzed.
UNASSIGNED: A 42-year-old patient was referred for retinal evaluation with nyctalopia, photophobia and metamorphopsia. Retinal examination and wide field color fundus photography were remarkable for bilateral crystalline deposits in the posterior pole and midperipheral retina. Wide field FAF showed extensive nummular atrophy of the retinal pigment epithelium (RPE) in the macula and periphery. Spectral-domain (SD) OCT illustrated bilateral chorioretinal atrophy in the macula. En face SD OCT captured the hyperreflective crystals in various retinal layers, depending on the selected segmentation. The patient was diagnosed with BCD and genetic testing confirmed the diagnosis (CYP4V positive for two variants).
UNASSIGNED: In this case report, we describe the multimodal imaging features of Bietti\'s Crystalline Dystrophy. Wide field FAF illustrated diffuse nummular RPE atrophy in the posterior pole and periphery and en face OCT captured the hyperreflective crystals in different layers of the retina.
UNASSIGNED: Wide field fundus photography, autofluorescence (FAF) imaging, and cross sectional and en face OCT were performed in a case of BCD. The level of the crystals in the retina were analyzed.
UNASSIGNED: A 42-year-old patient was referred for retinal evaluation with nyctalopia, photophobia and metamorphopsia. Retinal examination and wide field color fundus photography were remarkable for bilateral crystalline deposits in the posterior pole and midperipheral retina. Wide field FAF showed extensive nummular atrophy of the retinal pigment epithelium (RPE) in the macula and periphery. Spectral-domain (SD) OCT illustrated bilateral chorioretinal atrophy in the macula. En face SD OCT captured the hyperreflective crystals in various retinal layers, depending on the selected segmentation. The patient was diagnosed with BCD and genetic testing confirmed the diagnosis (CYP4V positive for two variants).
UNASSIGNED: In this case report, we describe the multimodal imaging features of Bietti\'s Crystalline Dystrophy. Wide field FAF illustrated diffuse nummular RPE atrophy in the posterior pole and periphery and en face OCT captured the hyperreflective crystals in different layers of the retina.
摘要：
■为了描述多模态成像特征，包括正面光学相干断层扫描（OCT），Bietti的晶体营养不良（BCD）。
■宽视野眼底摄影，自发荧光（FAF）成像，在BCD病例中进行横截面和正面OCT。分析视网膜中晶体的水平。
■一名42岁的患者因夜视转诊进行视网膜评估，畏光和变形。视网膜检查和宽视野彩色眼底照相对于后极和中周视网膜的双侧晶体沉积是显着的。宽视野FAF显示黄斑和周边的视网膜色素上皮（RPE）广泛的结节状萎缩。谱域（SD）OCT显示黄斑双侧脉络膜视网膜萎缩。在面部SDOCT捕获了各种视网膜层中的超反射晶体，取决于所选择的分割。患者被诊断为BCD，基因检测证实了诊断（CYP4V两种变异呈阳性）。
■在此案例报告中，我们描述了Bietti晶体营养不良的多模态影像学特征。宽场FAF显示了后极和外围的弥漫性块状RPE萎缩，并且面对OCT捕获了视网膜不同层中的超反射晶体。
■宽视野眼底摄影，自发荧光（FAF）成像，在BCD病例中进行横截面和正面OCT。分析视网膜中晶体的水平。
■一名42岁的患者因夜视转诊进行视网膜评估，畏光和变形。视网膜检查和宽视野彩色眼底照相对于后极和中周视网膜的双侧晶体沉积是显着的。宽视野FAF显示黄斑和周边的视网膜色素上皮（RPE）广泛的结节状萎缩。谱域（SD）OCT显示黄斑双侧脉络膜视网膜萎缩。在面部SDOCT捕获了各种视网膜层中的超反射晶体，取决于所选择的分割。患者被诊断为BCD，基因检测证实了诊断（CYP4V两种变异呈阳性）。
■在此案例报告中，我们描述了Bietti晶体营养不良的多模态影像学特征。宽场FAF显示了后极和外围的弥漫性块状RPE萎缩，并且面对OCT捕获了视网膜不同层中的超反射晶体。
