Balaratnasingam Chandrakumar, Yannuzzi Lawrence A, Spaide Richard F
*Vitreous Retina Macula Consultants of New York, NY; and †Luesther T Mertz Retinal Research Center, Manhattan, Eye, Ear and Throat Hospital, New York, NY.
Retina. 2015 Nov;35(11):2317-22. doi: 10.1097/IAE.0000000000000887.
To use volume-rendered optical coherence tomography angiography to investigate vascular proliferation in macular telangiectasia type 2 (MacTel2), extending beyond the retinal pigment epithelium (RPE).
Six eyes of four patients with MacTel2 with neovascularization proliferating external to the RPE confines were studied. Eyes were scanned using optical coherence tomography using split-spectrum amplitude-decorrelation techniques to derive flow information (RTVue XR; Optovue). These data were extracted and used to create volume rendered images of the area of vascular proliferation.
Mean age was 66.2 years. There was demonstrable vascular proliferation in the sub-RPE space observable by both optical coherence tomography and optical coherence tomography angiography. Fibrovascular RPE detachments were identified in all eyes. The topographic distribution of abnormal vessels located below the plane of the deep retinal vascular plexus and above the RPE closely matched the pattern of hyperfluorescence and leakage on fluorescein angiography. Vessels under the RPE demonstrated different branching patterns and larger diameter lumens than those above the RPE, but anastomosis with the choroidal circulation was difficult to demonstrate.
This study provides evidence that sub-RPE vascular proliferation may be a complication of MacTel2. Retinal pigment epithelium abnormalities are known to occur in MacTel2 and may provide a conduit for abnormal vessels in the subretinal space to proliferate into the sub-RPE compartment. The authors have no reason to exclude the possibility that the choroid contributes to the deep proliferation.
使用容积渲染光学相干断层扫描血管造影术研究2型黄斑毛细血管扩张症（MacTel2）中超出视网膜色素上皮（RPE）的血管增生情况。
对4例MacTel2患者的6只眼睛进行研究，这些眼睛的新生血管在RPE边界外增生。使用光学相干断层扫描技术和分裂光谱幅度去相关技术对眼睛进行扫描以获取血流信息（RTVue XR；Optovue公司）。提取这些数据并用于创建血管增生区域的容积渲染图像。
平均年龄为66.2岁。光学相干断层扫描和光学相干断层扫描血管造影均显示RPE下间隙存在明显的血管增生。所有眼睛均发现纤维血管性RPE脱离。位于视网膜深层血管丛平面下方和RPE上方的异常血管的地形分布与荧光素血管造影上的高荧光和渗漏模式密切匹配。RPE下方的血管显示出与RPE上方的血管不同的分支模式和更大直径的管腔，但与脉络膜循环的吻合难以显示。
本研究提供了证据表明RPE下血管增生可能是MacTel2的一种并发症。已知MacTel2中会出现视网膜色素上皮异常，这可能为视网膜下间隙中的异常血管增生进入RPE下腔室提供了一条途径。作者没有理由排除脉络膜促成深层增生的可能性。
