Vitreous, Retina, Macula Consultants of New York, New York, New York.
LuEster T. Mertz Retina Research Laboratory, Manhattan Eye, Ear and Throat Hospital, New York, New York.
Retina. 2020 Jan;40(1):92-98. doi: 10.1097/IAE.0000000000002619.
To characterize structural and angiographic findings in macular telangiectasia Type 2 (MacTel 2) and examine associations with visual acuity.
MacTel 2 patients with complete ophthalmologic examination, including fundus photography, autofluorescence, spectral-domain optical coherence tomography, and projection-resolved optical coherence tomography angiography, were retrospectively evaluated.
There were 43 eyes of 22 patients with a mean age 63.9 (±10.3) years. Six patients had diabetes. Twenty-one eyes (48.8%) had retinal-choroidal anastomoses (RCAs) without any evidence of neovascularization extending laterally in a plane above or below the retinal pigment epithelium. None of the eyes had hemorrhage, lipid, or signs of subretinal exudation. When present, an average of 55 (±33.7) individual RCAs were clustered primarily in temporal juxtafoveal region of involved eyes. Right-angle veins were seen in all 21 eyes with RCAs, and hyperpigmentation was present in 18 (P < 0.001 for both). A conical collection of hyperreflective material spanning from Bruch membrane past external limiting membrane of ≥200-μm basal diameter was found in 21 eyes and labeled outer retinal hyperreflective lesion. Retinal-choroidal anastomoses occurred in clusters, often within the outer retinal hyperreflective lesion. This lesion colocalized with focal thinning of the outer nuclear layer and was surrounded by a larger defect in the ellipsoid zone. The presence of diabetes (P = 0.015), outer retinal hyperreflective lesion (P = 0.006), RCA (P = 0.005), and ellipsoid zone defect extent (P < 0.001) were associated with decreased visual acuity.
Retinal-choroidal anastomoses occur in eyes with MacTel 2 without signs of exudation. Retinal-choroidal anastomoses occur in numerous clusters particularly in the temporal juxtafoveal macula. Diabetes, ellipsoid zone defect extent, RCAs, and the outer retinal hyperreflective lesion predict poorer vision in MacTel 2.
描述 2 型黄斑毛细血管扩张症（MacTel 2）的结构和血管造影特征，并探讨其与视力的关系。
回顾性分析经全面眼科检查（包括眼底照相、自发荧光、谱域光相干断层扫描和投影分辨光相干断层扫描血管造影）的 MacTel 2 患者。
共纳入 22 例患者的 43 只眼，平均年龄为 63.9（±10.3）岁。6 例患者患有糖尿病。21 只眼（48.8%）存在视网膜脉络膜吻合（RCAs），但无任何侧向延伸至视网膜色素上皮上方或下方的新生血管证据。无眼内出血、脂质或视网膜下渗出的迹象。当存在时，平均有 55（±33.7）个 RCA 主要聚集在受累眼的颞侧近黄斑区。所有 21 只存在 RCA 的眼中均可见直角静脉，且 18 只眼中存在色素沉着（两者均 P < 0.001）。在 21 只眼中发现了一个跨越 Bruch 膜至外节膜基底直径≥200μm的圆锥形高反射物质集合，标记为外视网膜高反射病变。RCA 常成群出现，且多位于外视网膜高反射病变内。该病变与外核层局灶性变薄共定位，并被椭圆体带较大的缺损所环绕。糖尿病（P = 0.015）、外视网膜高反射病变（P = 0.006）、RCA（P = 0.005）和椭圆体带缺损程度（P < 0.001）与视力下降有关。
MacTel 2 患者存在无渗出表现的视网膜脉络膜吻合。RCA 成群出现在颞侧近黄斑区。糖尿病、椭圆体带缺损程度、RCA 和外视网膜高反射病变预测 MacTel 2 视力较差。
