中文摘要：

目的 观察2型黄斑毛细血管扩张症（MacTel 2）的眼底及影像特征。方法 确诊为MacTel 2的8例患者16只眼纳入研究。其中，男性4例，女性4例。年龄44~69岁，平均年龄（59.88±7.85）岁。均行最佳矫正视力（BCVA）、裂隙灯显微镜、眼底彩色照相、眼底自身荧光（AF）、荧光素眼底血管造影（FFA）、频域光相干断层扫描（OCT）、黄斑色素密度（MPOD）检查；2例4只眼同时行OCT血管成像（OCTA）检查。由两名医师对影像检查结果进行独立阅片并对患眼进行分期。所有患眼均随访观察1~19个月，平均随访时间（11.00±8.91）个月。随访观察患眼的眼底及分期进展情况。结果 患眼BCVA为0.07~0.8。16只眼中，1期1只眼，2期1只眼，3期6只眼，4期8只眼。双眼病变程度对称5例，双眼病变不对称3例。眼底彩色照相检查发现，16只眼中，黄斑区中心凹视网膜透明度下降呈灰色，颞侧为重14只眼，占87.50%；可见色素沉着9只眼，占56.25%；中心凹旁小血管直角走形14只眼，占87.50%；类似黄斑裂孔的暗红色病灶5只眼，占31.25%。FFA检查发现，16只眼表现为不同程度的早期黄斑中心凹旁小血管扩张，晚期弥漫性强荧光。频域OCT检查发现，16只眼中，视网膜内外层结构缺失，空腔形成7只眼，占43.75%；外层视网膜萎缩，内层视网膜水肿，外层视网膜与视网膜色素上皮之间不均匀强反射信号9只眼，占56.25%。AF检查发现，16只眼中，黄斑中心正常暗区结构消失12只眼，占75.00%；黄斑中心凹反射信号增强9只眼，占56.25%。MPOD检查发现，16只眼均存在MPOD下降，MPOD模式眼底像可见颞侧局部区域的色素缺失。OCTA检查发现，患眼黄斑中心凹旁浅、深层血管丛破坏，血管间隙增大，中心凹无血管区（FAZ）扩大，血管变形，深层血管为著。随访期间，1例患者1只眼从4期进展到5期，出现视网膜下新生血管?

英文摘要：

Objective To observe the fundus image characteristics of macular telangiectasia type 2 （MacTel type 2） patients. Methods A total of 8 patients （16 eyes） diagnosed of MacTel type 2 were included in this study. There were 4 males and 4 females, age ranged from 44 to 69 years old with a median age of （59.88±7.85） years. All patients received examination of best-corrected visual acuity （BCVA）, slit lamp microscope, indirect ophthalmoscopy, fundus color photography, fundus autofluorescence （AF）, fundus fluorescein angiography （FFA）, spectral domain optical coherence tomography （OCT） and macular pigment optical density （MPOD）. Four eyes of 2 patients received OCT angiography examination at the same time. Classification was made according to the Gass and Blodi′s criteria. The follow-up time was from 1 to 19 months with the average time of （11.00±8.91） months. The clinical characteristics were observed and analyzed. Results The BCVA was 0.07 - 0.8. There were 1 eye in stage 1, 1 eye in stage 2, 6 eyes in stage 3, 8 eyes in stage 4. The disease showed a bilateral appearance with a low progression. Fundus features included loss of retinal transparency （14 eyes, 87.5%）, blunted retinal venule （15 eyes, 93.75%）, pseudo-lamellar hole （5 eyes, 31.25%）, pigment proliferation （9 eyes, 56.25%）. FFA findings were telangiectatic capillaries predominantly temporal to the foveola in the early phase and a diffuse hyperfluorescence in the late phase. Spectral domain OCT features included depletion of the retinal inner, outer structures, cavity （7 eyes, 43.75%）, and atrophy of the neurosensory retina （9 eyes,56.25%）. On AF, reduced foveal masking due to loss of macular pigment can be observed. The loss of macular pigment could also be seen on MPOD. OCTA showed the increased intervascular spaces, broken regular network of foveal avascular zone （FAZ）, right-angled vessel dipping, dilatations, traction of superficial and deep capillary layers in both the superficial and de