摘要目的:观察黄斑毛细血管扩张症2型（Mac-Tel 2）患眼光相干断层扫描血管成像（OCTA）影像特征。方法:回顾性病例分析。2017年10月至2021年6月于南京医科大学附属眼科医院经多模式影像检查确诊的Mac-Tel 2患者11例22只眼纳入研究。其中，男性5例10只眼，女性6例12只眼；年龄（41.61±11.32）岁。所有患者均行最佳矫正视力、间接检眼镜、眼底彩色照相、荧光素眼底血管造影（FFA）、光相干断层扫描（OCT）、OCT血管成像（OCTA）检查。采用OCTA仪对患者双眼黄斑区3 mm×3 mm范围进行扫描，设备自带软件自动合成处理图像后提供视网膜浅层毛细血管层、深层毛细血管层、视网膜外层、脉络膜毛细血管层血流图以及对应的B扫描像。观察其影像特征。结果:22只眼中，病变早期14只眼，病变中晚期即继发视网膜下新生血管（SRN）和（或）脉络膜新生血管（CNV） 8只眼。FFA检查，病变早期的14只眼黄斑中心凹旁毛细血管扩张，血管牵拉，造影晚期以荧光着染为主；病变中晚期即继发SRN及CNV的8只眼可见强荧光素渗漏改变。OCTA检查，病变早期，黄斑区颞侧毛细血管扩张牵拉，以深层更为显著，毛细血管间隙增大，可见直角小静脉改变并向深层潜入；病变侵犯至视网膜外层时，可见花团样SRN；病变进一步发展继发CNV时，视网膜外层及脉络膜毛细血管层可见新生血管。对应B扫描像，病变早期，神经上皮层间不规则弱反射腔、板层裂孔改变；继发SRN、CNV时，可见小团状强反射并伴丰富血流信号改变。结论:Mac-Tel 2患眼OCTA影像特征表现为黄斑区颞侧浅层、深层毛细血管扩张、牵拉以及血管呈直角状改变。

abstractsObjective:To observe the imaging characteristics of optical coherence tomography angiography in macular telangiectasia type 2 (Mac-Tel 2).Methods:A retrospective case analysis. From October 2017 to June 2021, 11 patients (22 eyes) diagnosed as Mac-Tel type 2 by multi-modal imaging in Nanjing Medical University Eye Hospital were included in this study. There were 5 males (10 eyes) and 6 females (12 eyes). The age were 41.61±11.32 years old. All patients underwent the examinations of best corrected visual acuity, indirect ophthalmoscope, fundus color photography, fluorescein fundus angiography (FFA), optical coherence tomography (OCT), and OCT angiography (OCTA). The scope of 3 mm × 3 mm in macular area of eyes was scanned by OCTA. After automatic image processing, the system could provide the blood flow image of capillary layer, deep capillary layer, outer retina, choroidal capillary layer, and the B-scan image. The imaging characteristics were observed.Results:Among the 22 eyes, 14 eyes were in the early stage of the disease, and 8 eyes had secondary subretinal neovascularization (SRN) and/or choroidal neovascularization (CNV). FFA examination that in the early stage of the disease, the capillaries near the fovea were dilated, the blood vessels were stretched, and the late fluorescence was mainly stained; high-fluorescence leakage was seen when SRN and CNV were developed. OCTA examination showed that in the early stage of the disease, the temporal capillaries in the macular area were dilated and stretched, especially in the deep layer. The capillary space was enlarged, and the right-angled venules were seen to change and infiltrate into the deep layer; when the lesions invaded the outer retina, flower clusters-like SRN were seen; neovascularization was seen in the outer retina and choroidal capillary layer when CNV was developed. B-scan image showed that in the early stage of the disease, irregular weak reflex cavities and lamellar holes change between the neuroepithelial layers; secondary SRN and CNV showed strong little clumpy reflexes accompanied by abundant blood flow signals.Conclusion:The image characteristics of OCTA in the eyes of Mac-Tel 2 were dilated, stretched superficial and deep temporal capillaries in the macula area and right-angled changes in blood vessels.