Background: Bietti crystalline dystrophy (BCD) is a rare autosomal recessive disorder due to genetic defect in the CYP4V2 gene. BCD is a disease characterized by shiny yellow crystalline deposits in the retina with progressive atrophy of the retinal pigment epithelium and choriocapillaris. Our aim is to present ocular imaging findings of a patient with BCD.Materials and Metods: A 38-year-old female patient with BCD was evaluated and the findings of optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA) were examined.Results: OCT imaging revealed multiple outer retinal tubulations (ORTs) with a few hyperreflective crystalline deposits. OCTA imaging showed that the vessel density of superficial anddeep capillary plexus and choriocapillaris blood flow were significantly decreased. ORTs were composedof multiple microtubules as finger-like protrusions that joined the macrotubules.Conclusion: In BCD, crystallinedeposits, ORTs and retinal vascular morphology can be shown in detail using OCT and OCTA.

背景：Bietti晶体营养不良（BCD）是一种罕见的常染色体隐性遗传疾病，由于CYP4V2基因的遗传缺陷。BCD是一种特征在于视网膜上有发亮的黄色晶体沉积，视网膜色素上皮和绒毛膜毛细血管逐渐萎缩的疾病。我们的目的是介绍BCD患者的眼部影像学表现。材料和方法：对一名38岁的BCD女性患者进行了评估，并检查了光学相干断层扫描（OCT）和光学相干断层造影血管造影（OCTA）的发现。结果：OCT成像显示多处视网膜外肾小管（ORT）以及一些高反射性晶体沉积物。OCTA成像显示浅层和深层毛细血管丛的血管密度和脉络膜毛细血管的血流明显减少。ORT由多个微管组成，这些微管是连接大管的手指状突起。结论：在BCD中，使用OCT和OCTA可以详细显示晶体沉积物，ORT和视网膜血管形态。