Marques João Heitor, Coelho João, Menéres Maria João, Melo Beirão João
Serviço de Oftalmologia, Centro Hospitalar Universitário do Porto, Porto, Portugal.
Instituto de Ciências Biomédicas Abel Salazar, Universidade do Porto, Porto, Portugal.
Clin Ophthalmol. 2022 Jul 9;16:2227-2233. doi: 10.2147/OPTH.S359312. eCollection 2022.
Retinal angiopathy associated with hereditary transthyretin amyloidosis (ATTRv), if untreated, may lead to irreversible vision loss. Our purpose was to systematically review the clinical and imaging features of retinal angiopathy associated with ATTRv and assemble a monitoring approach for these patients. All types of original research studies reporting clinical and imaging findings on retinal angiopathy associated with ATTRv were included. The most common clinical findings were tortuous retinal vessels, microaneurysms, retinal hemorrhages, sheathing of retinal vessels, whitish amyloid deposits along retinal arteries, obliteration of retinal vessels, vitreous hemorrhage, retinal and iris neovascularization. The most relevant imaging findings were hyperautofluorescence of perivessel amyloid deposits; delayed arterial filling, vascular leakage, and retinal ischemia on fluorescein angiography; late hypercyanescence along the choroidal arteries on indocyanine green angiography; perivascular hyperreflective material, needle-shaped deposits on the retinal surface and macular edema on optical coherence tomography (OCT) and attenuated retinal vascular network on OCT-angiography. ATTRv patients should be strictly followed to detect and treat retinal angiopathy, avoiding complications. Both panretinal photocoagulation and intravitreal anti-vascular endothelial growth factor have been used to treat retinal angiopathy in ATTRv. In an individual that presents with retinal angiopathy of unknown etiology, ATTRv should be considered as in the differential diagnosis, even out of the initial core countries. The prognostic value of subclinical findings, namely in OCT-A, is not yet established.
与遗传性转甲状腺素蛋白淀粉样变性（ATTRv）相关的视网膜血管病变，若不治疗，可能导致不可逆的视力丧失。我们的目的是系统回顾与ATTRv相关的视网膜血管病变的临床和影像学特征，并为这些患者制定一种监测方法。纳入了所有报告与ATTRv相关的视网膜血管病变临床和影像学发现的原始研究类型。最常见的临床发现是视网膜血管迂曲、微动脉瘤、视网膜出血、视网膜血管鞘、沿视网膜动脉的白色淀粉样沉积物、视网膜血管闭塞、玻璃体积血、视网膜和虹膜新生血管。最相关的影像学发现是血管周围淀粉样沉积物的高自发荧光；荧光素血管造影显示动脉充盈延迟、血管渗漏和视网膜缺血；吲哚菁绿血管造影显示脉络膜动脉晚期高氰化；光学相干断层扫描（OCT）显示血管周围高反射物质、视网膜表面针状沉积物和黄斑水肿，以及OCT血管造影显示视网膜血管网络变细。应严格随访ATTRv患者，以检测和治疗视网膜血管病变，避免并发症。全视网膜光凝和玻璃体内抗血管内皮生长因子均已用于治疗ATTRv中的视网膜血管病变。在病因不明的视网膜血管病变患者中，即使不在最初的核心国家，也应在鉴别诊断中考虑ATTRv。亚临床发现（即OCT-A中的发现）的预后价值尚未确定。
