Charbel Issa Peter, Kupitz Elke H, Heeren Tjebo F C, Holz Frank G
Dev Ophthalmol. 2016;55:189-95. doi: 10.1159/000431263. Epub 2015 Oct 26.
Macular telangiectasia (MacTel) type 2 is a bilateral disease of unknown cause with localized retinal degeneration and characteristic changes of the retinal vasculature. Funduscopic findings include reduced retinal transparency, crystalline deposits, ectatic capillaries, blunted venules, retinal pigment plaques, foveal atrophy and neovascular complexes. Leakage of telangiectatic macular capillaries is a characteristic finding on fluorescein angiography, and neurosensory atrophy may be present on optical coherence tomography images. Furthermore, there is a specific depletion of macular pigment in the central retina. Depending on the development of neovascular membranes, a nonproliferative and a proliferative (neovascular) disease stage may be distinguished. To date, there is no evidence for an effective treatment of nonproliferative MacTel type 2. Patients with proliferative MacTel type 2 and hence decreasing visual function may benefit from intravitreal application of vascular endothelial growth factor inhibitors. Early treatment and a small size of the neovascular membrane might be predictive factors for visual function outcome.
2型黄斑毛细血管扩张症(MacTel)是一种病因不明的双侧性疾病,伴有局限性视网膜变性和视网膜血管的特征性改变。眼底检查结果包括视网膜透明度降低、结晶样沉积物、扩张的毛细血管、变钝的小静脉、视网膜色素斑、黄斑萎缩和新生血管复合体。黄斑毛细血管扩张的渗漏是荧光素血管造影的特征性表现,光学相干断层扫描图像上可能出现神经感觉萎缩。此外,中央视网膜的黄斑色素有特定的缺失。根据新生血管膜的发展情况,可分为非增殖性和增殖性(新生血管性)疾病阶段。迄今为止,尚无证据表明对非增殖性2型MacTel有有效的治疗方法。增殖性2型MacTel患者以及因此视力下降的患者可能从玻璃体内应用血管内皮生长因子抑制剂中获益。早期治疗和新生血管膜较小可能是视力功能预后的预测因素。