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首页> 外文期刊>Journal of Clinical Medicine >Macular Perfusion Impairment in Von Hippel-Lindau Disease Suggests a Generalized Retinal Vessel Alteration
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Macular Perfusion Impairment in Von Hippel-Lindau Disease Suggests a Generalized Retinal Vessel Alteration

机译：Von Hippel-Lindau疾病中的黄斑灌注障碍表明广泛的视网膜血管变化

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Background: To evaluate macular perfusion in patients with Von Hippel–Lindau (VHL) disease. Methods: VHL patients with or without peripheral retinal hemangioblastomas (RHs) were consecutively enrolled. A group of healthy subjects served as controls. Macular perfusion was analyzed by means of OCT angiography (OCTA) in the superficial vascular plexus (SVP), and in the intermediate (ICP) and deep retinal capillary (DCP) plexuses. The following OCTA parameters were measured: Vessel Area Density (VAD), Vessel Length Fraction (VLF), Vessel Diameter Index (VDI) and Fractal Dimension (FD). Results: Sixty-three VHL patients (113 eyes) and 28 healthy controls (56 eyes) were enrolled. All OCTA quantitative parameters were reduced in VHL patients vs. controls, reaching statistical significance for VAD of the SVP (0.348 ± 0.07 vs. 0.369 ± 0.06, p = 0.0368) and VDI of all plexuses ( p 0.03 for all). No significant differences were detected between eyes without or with peripheral RHs. Conclusions: Macular perfusion is reduced in VHL patients demonstrating retinal vessel changes that are independent of the presence of peripheral RHs. VHL gene mutations disrupt the hypoxia-induced (HIF)/vascular endothelium growth factors (VEGF) pathway and the Notch signaling, both essential for the normal retinal vasculogenesis and angiogenesis. Therefore, an anomalous generalized retinal vascular development may be hypothesized in VHL disease.
机译：背景：评估von Hippel-Lindau（VHL）疾病患者的黄斑灌注。方法：连续注册VHL患者或不含外周视网膜血管母细胞瘤（RHS）。一群健康受试者作为对照。通过浅屏血管丛（SVP）中的OCT血管造影（OctA）和中间体（ICP）和深视网膜毛细管（DCP）丛，分析黄斑灌注。测量以下OCTA参数：血管面积密度（VAD），容器长度级分（VLF），容器直径指数（VDI）和分形尺寸（FD）。结果：六十三名VHL患者（113只眼）和28例健康对照（56只眼）。 VHL患者的所有Octa定量参数降低了对照，达到了SVP的VAD统计学意义（0.348±0.07与0.369±0.06，P = 0.0368）和VDI的所有丛（全部P <0.03）。在没有或外围RHS之间没有检测到眼睛之间没有显着差异。结论：在VHL患者中降低了黄斑灌注，证明与周围RHS的存在无关的视网膜血管变化。 VHL基因突变破坏缺氧诱导的（HIF）/血管内皮生长因子（VEGF）途径和凹口信号传导，对正常的视网膜血管发生和血管生成。因此，可以在VHL疾病中假设异常的广泛视网膜血管发育。

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