肥厚型脉络膜谱系疾病的研究进展

肥厚型脉络膜谱系疾病包括脉络膜增厚性视网膜色素上皮病变(PPE)、中心性浆液性脉络膜视网膜病变(CSC)、肥厚性脉络膜新生血管病变(PNV)和息肉状脉络膜血管病变(PCV)。PPE指脉络膜厚度永久性异常增加,表现为脉络膜大血管层(Haller层)扩张,压迫其周围的中血管层(Sattler层)和毛细血管层(Choriocapillaris层),导致RPE供血不足,引起一系列色素上皮病变。其余3种疾病可由脉络膜增厚性色素上皮病变进展而来,对于肥厚型脉络膜谱系疾病的发病特征、影像学变化的研究有助于探索该谱系疾病发病机制,为该类疾病的早期临床诊断及预防和治疗提供参考。     

肥厚型脉络膜谱系疾病影像特征研究进展

肥厚型脉络膜谱系疾病(PSD)是近年来被定义的一组具有相似脉络膜解剖学特点及相同病理过程的一组疾病,包括中心性浆液性脉络膜视网膜病变(CSC)、息肉样脉络膜血管病变(PCV)等。其主要特征是脉络膜各血管层厚度改变和血管通透性增强。随着以光学相干断层扫描血流成像(OCTA)为代表的现代眼科影像技术不断发展,使这类疾病脉络膜各血管层的变化得以更直观地呈现,从而对该谱系疾病发病机制研究有了新的认识。本文对健康人及PSD的共同临床特点进行归纳,对各种PSD的最新影像学发现进行综述。从遗传背景及解剖学结构等不同角度,讨论各型PSD是同一疾病不同阶段的表型还是类似特征的不同疾病。以期为该类疾病的发病机制、早期临床诊断及防治提供参考。     

息肉状脉络膜血管病变中脉络膜异常改变的研究进展

脉络膜组织结构及功能的完整对视网膜功能正常发挥起重要作用.息肉状脉络膜血管病变(polypoid choroidal vasculopathy,PCV)是一类以异常分支状血管网和血管网末端息肉状膨大为特征的视网膜脉络膜疾病.其属于肥厚型脉络膜疾病谱,谱系疾病之间可以发生转化并且存在特征性脉络膜改变:局灶性或弥漫性脉络膜...     

肥厚型脉络膜谱系疾病与脉络膜、涡静脉、巩膜改变的研究进展

肥厚型脉络膜谱系疾病(pachychoroid disease spectrum,PCD)包括肥厚型脉络膜色素上皮病变(pachychoroid pigment epitheliopathy,PPE)、中心性浆液性脉络膜视网膜病变(central serous chorioretinopathy,CSC)、肥厚型脉络膜新生血管病变(pachychoroid neovasculopathy,PNV)、息肉样脉络膜血管病变(polypoidal choroidal vasculopathy,PCV)、局灶性脉络膜凹陷(focal choroidal excavation,FCE)和盘周肥厚型脉络膜综合征(peripapillary pachychoroid syndrome,PPS)。有学者将PCD看作脉络膜功能障碍引发的一系列连续疾病过程,但关于PCD的发病机制、形态改变尚未明确。该文对PCD的脉络膜、涡静脉及巩膜相关改变做一综述。     

肥厚型脉络膜疾病谱的研究进展

肥厚型脉络膜疾病包括中心性浆液性脉络膜视网膜病变、息肉样脉络膜血管病变、肥厚型色素上皮病变、肥厚型脉络膜新生血管病变、局灶性脉络膜凹陷、视盘旁肥厚型脉络膜综合征及周边部渗出性出血性脉络膜视网膜病变等。这类疾病的共同特征是脉络膜血管扩张、通透性增强伴脉络膜厚度增加及视网膜色素上皮异常, 但不同肥厚型脉络膜疾病的临床表现及预后各不相同。随着多模式影像技术的发展及应用, 对于肥厚型脉络膜疾病的认识仍将不断深入。本文对目前已知的肥厚型脉络膜疾病就病理生理学特点、多模式影像特征及其可能的发病机制等进行综述。     

使用OCT评估中心性浆液性脉络膜视网膜病变患者脉络膜厚度的变化

目的:使用光谱域光学相干断层成像(SD-OCT)技术研究中心性浆液性脉络膜视网膜病变(CSCR)3mo后脉络膜厚度的变化。方法:前瞻性研究。共纳入60眼,20眼(平均年龄:33.65±5.24岁)典型急性单侧中心性浆液性脉络膜视网膜病变以及对侧正常眼,20眼为健康对照组。进行荧光素血管造影和OCT检查。测量中心凹下脉络膜厚度(SFCT),黄斑中心凹视网膜厚度(CMT),到中央凹和视网膜下液1000μm处颞部和鼻部。结果:在三个不同的位置,三组间的SFCT差异有统计学意义。中心性浆液性脉络膜视网膜病变眼中心凹下脉络膜厚度(372.40±34.39μm)在基线和随访3mo后均显著大于对侧正常眼(302.10±8.9μm)和对照组眼(279.80±14.49μm)。CSCR眼平均CMT为317±141.86μm,并且SFCT与CMT呈显著正相关。结论:不同部位脉络膜厚度的增加,以及被称为“厚脉络膜”的过度扩张和高渗透血管,似乎在包括中心性浆液性脉络膜视网膜病变在内的广泛疾病中起着重要作用。     

肥厚型脉络膜疾病研究现状及展望

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...     

肥厚型玻璃膜疣的研究进展

肥厚型玻璃膜疣见于息肉样脉络膜血管病变(PCV)、中心性浆液性脉络膜视网膜病变(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病变(PNV)等肥厚型脉络膜病变患眼内。这类疾病的共同特征是脉络膜血管扩张、通透性增强伴脉络膜厚度增加及视网膜色素上皮(RPE)异常。本文概括了肥厚型玻璃膜疣这一新近命名的玻璃膜疣类型的临床表现、流行病学、遗传学及组织病理学研究,以期为肥厚型玻璃膜疣的诊断和相关疾病的治疗进展提供方向。     

中心性浆液性脉络膜视网膜病变眼底自发荧光影像的特点

目的：研究急性和慢性特发性中心性浆液性脉络膜视网膜病变眼底自发荧光影像模式及眼底荧光血管造影相关性发现。

方法：观察性研究案例。回顾性分析中心性浆液性脉络膜视网膜病变患者临床数据,眼底荧光血管造影及眼底自发荧光影像,并对其调查结果进行比较。

结果：该研究共纳入17例25眼。确诊为急性中心性浆液性脉络膜视网膜病变5眼,慢性疾病或复发性慢性疾病20眼。急性病例眼底自发荧光影像显示低荧光点与荧光血管造影检测出的荧光渗漏点位置相同。慢性特发性中心性浆液性脉络膜视网膜病变眼底荧光血管造影为视网膜色素上皮弥漫性萎缩区域,可透视荧光。眼底自发荧光影像的低荧光区域的形态和位置与眼底荧光血管造影的高荧光区域相对应,然而眼底荧光血管造影的低荧光区域与眼底自发荧光影像的高荧光区域相对应。在急性病例中,低自发荧光点不能准确指出视网膜色素上皮的渗漏点。

结论：中心性浆液性脉络膜视网膜病变眼底自发荧光影像模式能够描述疾病不同阶段的特征,具有无风险和可再生性,可替代荧光素血管造影术治疗中心性浆液性脉络膜视网膜病变。     

外周渗出性出血性脉络膜视网膜病变诊断及治疗研究进展

外周渗出性出血性脉络膜视网膜病变(PEHCR)是以视网膜下出血和(或)视网膜色素上皮下出血或渗出为主要特征的周边视网膜疾病, 常被误诊为老年性黄斑变性、息肉样脉络膜血管病变或脉络膜黑色素瘤等。随着多模式影像学发展, PEHCR在B型超声、荧光素眼底血管造影、光相干断层扫描等检查中表现出不同特征, 据此可与上述疾病鉴别。该病治疗方法包括激光光凝治疗、玻璃体腔注射抗血管内皮生长因子药物和玻璃体切割手术等, 但目前尚无统一治疗标准。未来仍需进一步深入了解PEHCR临床特征、治疗方案及预后, 最大程度避免临床漏诊与误诊, 提高治疗效率。     

视盘周围脉络膜肥厚综合征研究进展

视盘周围脉络膜肥厚综合征是近年来提出的一个相对较新的概念,描述了一类以视盘周围脉络膜增厚及脉络膜大血管层扩张为特点的视网膜脉络膜疾病,其特征为局限性或弥漫性脉络膜增厚、脉络膜最厚区域位于视盘周围、视盘周围视网膜层间积液、黄斑区神经上皮脱离合并视网膜色素上皮脱离。本文对视盘周围脉络膜肥厚综合征的临床特征、发病机制、诊断和鉴别诊断以及治疗等进行综述。     

盘周厚脉络膜综合征:厚脉络膜疾病谱的新病种

盘周厚脉络膜综合征(PPS)是2017年上线报告的、厚脉络膜疾病谱(PDS)中的新病种。PPS眼的鼻侧黄斑脉络膜要比颞侧黄斑脉络膜更厚,不像PDS眼的颞侧黄斑脉络膜较厚。盘周视网膜内和/或视网膜下的液体常覆盖于扩张的Haller层血管(厚血管)之上。多数PPS眼显示出其他PDS眼的表现,包括视网膜色素上皮改变、浆液性色素上皮脱离及外层视网膜萎缩。视盘通常拥挤,一些眼伴有视盘水肿和轻度的晚期荧光渗漏。患者多为老年,短眼轴和远视常见。有些患者视力有下降,但总体上视力结果相对较好。虽然应用了抗VEGF和光动力疗法,但优化的处理尚不明确。识别PPS对鉴别后葡萄膜炎和神经眼科等有重叠特征的疾病是重要的。已提出PDS是涡静脉慢性瘀滞的后果。而脉络膜静脉超负荷的假说为此类疾病的病理生理学提供了统一的概念。     

Ultra-Widefield Indocyanine Green Angiography Reveals Patterns of Choroidal Venous Insufficiency Influencing Pachychoroid Disease

PurposeTo compare patterns of choroidal venous drainage in eyes with pachychoroid disease to those of healthy subjects using ultra-widefield indocyanine green angiography (UWF ICGA).MethodsPatients with pachychoroid disease and healthy controls were recruited at two referral centers. UWF ICGA images were used to evaluate the proportion of the postequatorial fundus drained by major vortex vein systems in each quadrant and to study the incidence and topography of choroidal vascular hyperpermeability (CVH) and intervortex venous anastomoses. Widefield swept-source optical coherence tomography (SS-OCT) was used to evaluate choroidal thickness at the posterior pole in eyes with pachychoroid disease.ResultsFifty-two pachychoroid eyes and 26 healthy eyes were evaluated. Eyes with pachychoroid disease showed a significant within-subject variance in the proportion of the postequatorial fundus drained by each vortex vein system (range, 4.1%–48.1%; P < 0.0001) that was not seen in controls (range, 17.3%–31.7%; P = 0.11). CVH was present in all pachychoroid disease eyes and three of 26 controls. Intervortex venous anastomoses were present in 46 of 52 pachychoroid disease eyes and nine of 26 control eyes. Vortex vein systems with large drainage areas showed greater density of CVH spots. SS-OCT demonstrated asymmetric choroidal drainage in the macula of 59% of pachychoroid eyes. CVH and intervortex venous anastomoses were more prominent in areas showing maximal choroidal thickness.ConclusionsIn eyes with pachychoroid disease, imbalanced choroidal venous drainage with congestion of specific vortex vein systems may contribute to a state of choroidal venous insufficiency characterized by regional choroidal thickening, CVH and remodeling of venous drainage routes.     

值得关注的肥厚型脉络膜病变

近年来,随着眼科学影像技术的不断发展,人们对于脉络膜的特征及其在眼底疾病发生发展中的作用的了解不断加深,提出了"肥厚型脉络膜病变"的概念,拓宽了视野。然而目前仍有很多问题需要明晰和探索,如肥厚脉络膜和肥厚血管的概念、多种影像检查时疾病的表现特征、此类疾病之间的关系和转归以及对治疗的反应等。关注这些问题,将有助于提高对此类疾病的临床诊疗水平。     

Venous overload choroidopathy: A hypothetical framework for central serous chorioretinopathy and allied disorders

In central serous chorioretinopathy (CSC), the macula is detached because of fluid leakage at the level of the retinal pigment epithelium. The fluid appears to originate from choroidal vascular hyperpermeability, but the etiology for the fluid is controversial. The choroidal vascular findings as elucidated by recent optical coherence tomography (OCT) and wide-field indocyanine green (ICG) angiographic evaluation show eyes with CSC have many of the same venous patterns that are found in eyes following occlusion of the vortex veins or carotid cavernous sinus fistulas (CCSF). The eyes show delayed choroidal filling, dilated veins, intervortex venous anastomoses, and choroidal vascular hyperpermeability. While patients with occlusion of the vortex veins or CCSF have extraocular abnormalities accounting for the venous outflow problems, eyes with CSC appear to have venous outflow abnormalities as an intrinsic phenomenon. Control of venous outflow from the eye involves a Starling resistor effect, which appears to be abnormal in CSC. Similar choroidal vascular abnormalities have been found in peripapillary pachychoroid syndrome. However, peripapillary pachychoroid syndrome has intervortex venous anastomoses located in the peripapillary region while in CSC these are seen to be located in the macular region. Spaceflight associated neuro-ocular syndrome appears to share many of the pathophysiologic problems of abnormal venous outflow from the choroid along with a host of associated abnormalities. These diseases vary according to their underlying etiologies but are linked by the venous decompensation in the choroid that leads to significant vision loss. Choroidal venous overload provides a unifying concept and theory for an improved understanding of the pathophysiology and classification of a group of diseases to a greater extent than previous proposals.     

Multimodal imaging in pachychoroid spectrum

Diagnostic investigation on pachychoroid spectrum disease (PSD) has been growing along with the rapid advancement of imaging technology. In optical coherence tomography (OCT)-based studies, choroidal thickness profile, luminal and stromal choroidal ratio, and abnormalities in the neurosensory retina have demonstrated various patterns in different clinical entities related to PSD. The emerging role of OCT angiography ()CTA) has been expanded to involve the quantitative analysis of the OCTA parameters in different clinical entities of PSD and to evaluate the choriocapillaris signal void and vessel density as indicators of choriocapillaris ischemia. OCTA has broadened our knowledge in characterization and assessment of both active and quiescent choroidal neovascularization and its association with treatment response. Recent studies using indocyanine green angiography have focused on the evaluation of choroidal vascular hyperpermeability and its relationship with other pachychoroid related features. Ultra-widefield indocyanine green angiography enables observation and characterization of peripheral choroidal pathologies and their associations with macular abnormalities. Multicolor imaging is an emerging modality with the capability to demonstrate early abnormalities in PSD. We summarize all investigations reflecting the new insights into the application of multimodal imaging for PSD and focus on novel findings observed in different clinical entities with each imaging modality.