肥厚型脉络膜疾病研究现状及展望

Research progress on pachychoroid disease

肥厚型脉络膜疾病是近年提出的一个相对较新的概念,描述了一类以病理情况下脉络膜慢性增厚及脉络膜血管扩张为特点的视网膜脉络膜疾病谱,其特征性表现为脉络膜毛细血管层容量衰减、脉络膜血管扩张、局灶性或弥漫性的脉络膜增厚、进行性的视网膜色素上皮功能障碍及脉络膜新生血管(CNV)生成。如今对于肥厚型脉络膜疾病的定义,已从既往强调单纯的脉络膜异常增厚逐渐转变为对复杂的病理状态(肥厚型脉络膜疾病)的形态学描述。肥厚型脉络膜疾病谱包括中心性浆液性视网膜脉络膜病变(CSC)、肥厚型脉络膜视网膜色素上皮病变(PPE)、肥厚型脉络膜新生血管性疾病(PNV)、息肉样脉络膜血管病变(PCV)、局灶性脉络膜凹陷(FCE)、视盘周围脉络膜增厚综合征(PPS)。这些疾病都表现出各自的特征性脉络膜病变。本文对肥厚型脉络膜疾病的分类、特征、最新治疗进展进行综述。

Pachychoroid disease is a relatively novel concept proposed in ophthalmology in recent years,which describes a series of pathological conditions including chronic persistent choroidal thickening and choroidal vasodilatation,with choroid blood capillary layer capacity attenuation,choroidal vasodilation,focal or disseminated choroidal thickening,progressive retinal pigment epithelial dysfunction and choroid retinal neovascularization as characteristic symptoms.Nowadays,the definition of pachychoroid disease has gradually changed from the previous emphasis on the simple thickening of choroid to the morphological description of complex pathological conditions.The clinical manifestations of certain pachychoroid diseases have been clearly described including central serous chorioretinopathy(CSC),pachychoroid neovasculcularization(PNV),polypoid choroidal vasculopathy(PCV),focal choroidal excavation(FCE),peripapillary pachychoroid syndrome(PPS).These entities have certain characteristic choroidal vasculopathy,which may represent different clinical manifestations of the same type of pachychoroid diseaseor indicate that they have similarpathogenesis.In this article,the existing literature and recent findings about pachychoroid disease,as well as the classification and treatment of pachychoroid diseases spectrum were summarized and discussed.