息肉状脉络膜血管病变的分型研究

目的:依据吲哚菁绿血管造影(indocyanine green angiography,ICGA)显示的息肉状脉络膜血管病变(polypoidal choroidal vasculopathy,PCV)的形态进行分型,结合光学相干断层扫描(optical coherence tomography,OCT)及临床特征,探讨各型的特点,为PCV的治疗和预后评价提供依据。方法:回顾性分析在宁夏眼科医院就诊的33例PCV患者。所有病例均经过OCT和荧光素眼底血管造影(fundus fluorescein angiography,FFA)与ICGA等全面检查后确诊。根据ICGA的表现进行分型,同时对各亚型的病变特征进行比较。结果:共收集到33例54眼PCV患者,单眼12例,双眼21例;男20例,女13例;年龄42～86(平均70.63±9.42)岁。45眼病变部位位于黄斑区,占83%。根据ICGA的表现分为:息肉状扩张型(20眼,37%)、异常分支血管网型(16眼,30%)、经典型(18眼,33%)。27眼(50%)出现视网膜色素上皮脱离(pigment epithelial detachment,PED),其中分支血管网型及经典型出现PED的比例相对较高,分别为11眼(68.8%)和12眼(66.7%)。23眼(43%)出现视网膜神经上皮层脱离,其中分支血管网型10眼(62.5%)和经典型9眼(50.0%)易出现神经上皮脱离。31眼(57%)表现为活动型病变,其中分支血管网型和经典型常见,分别为13眼(81.3%)和13眼(72%)。10眼(19%)出现纤维机化膜,其中分支血管网型及经典型多见,分别为7眼(43.8%)和2眼(11.1%)。37眼(69%)有滋养血管的存在,其中分支血管网型和经典型分别为81%和94%。结论:分支血管网型及经典型易出现视网膜色素上皮脱离及视网膜神经上皮层脱离,多表现为活动型病变,存在滋养血管。而息肉型较少出现视网膜色素上皮脱离和神经上皮脱离,多表现为静止型,大多无滋养血管。异常分支血管网型易出现纤维机化膜。     

息肉状脉络膜血管病变的遗传基因研究进展

息肉状脉络膜血管病变（PCV）是一种与湿性年龄相关性黄斑变性（AMD）相似但又具有特殊性的一种渗出性黄斑病变,具有种族发病倾向,在亚洲人群中发生率较高。它的发生是多种遗传因素作用的结果。近年来研究发现与其相关的基因有补体因子H（CFH）基因、LOC387715／HTRA1基因、补体因子B／补体成分2（CFB／C2）基因、ApoE4基因、弹性蛋白（ELN）基因、锰超氧化物歧化酶（SOD2）基因、SERPING1基因、色素上皮细胞衍生因子（PEDF）基因。这些基因及其多态性与PCV及湿性AMD的发生有一定关系,并且部分基因的多态性可能揭示了PCV具有种族发病倾向的原因。     

新生血管性年龄相关性黄斑变性和息肉状脉络膜血管病变的分子生物学研究现状及存在问题

新生血管性年龄相关性黄斑变性（nAMD）和息肉状脉络膜血管病变（PCV）是常见的引起老年人致盲和严重视力损害的眼病,都以黄斑区出血和渗出为特征。近年来,关于PCV是否为nAMD的一种亚型存在着争论。nAMD和PCV具有部分相同的强关联易感基因位点（CFH,ARMS2／HTRAI）,但在更多不同的基因位点（如BF／C2,Elastin,PEDF,SERPlNGl,VEGF,APOE等基因）上存在区别,这可能提示了PCV和nAMD在病因学上存在差异。但就目前nAMD和PCV的研究现状,尚无法严格地将其定义为两种不同的疾病。就nAMD和PCV的分子生物学研究现状和存在问题进行阐述。     

特发性息肉状脉络膜血管病变

特发性息肉状脉络膜血管病变(idiopathic polypoidal choroidal vasculopathy, IPCV)是近年认识的一种与渗出型老年黄斑变性(age-related macular degeneration, AMD)表现较为相似的眼病，但两种疾病在流行病学、病情转归、视力预后及治疗等方面均有较大的不同。因此IPCV诊断及鉴别诊断具有较为重要的临床意义。IPCV的吲哚青绿血管造影(indocyanine green angiography，ICGA)显示有分支状脉络膜血管网及末梢的息肉状血管扩张等特征性表现，为IPCV诊断与鉴别诊断提供了重要的依据。 (中华眼底病杂志, 2002, 18: 310-312)     

息肉状脉络膜血管病变的现状和进展

息肉状脉络膜血管病变(polypoidal choroidal vasculopathy,PCV)是一种发病原因和发病机制不明的疾病,一些研究表明其可能与某些基因有关。PCV眼底镜下表现常与渗出型年龄相关性黄斑变性(age-related macular degeneration,AMD)相似,通过吲哚青绿血管造影(indocyanine green angiography,ICGA)及光学相干断层扫描(optic coherence tomography,OCT)等影像学检查可予以鉴别。目前主要通过眼底镜下特征性的橘红色息肉状损害和ICGA下见血管网边缘息肉状血管损害对PCV进行诊断。主要的治疗方法包括光动力疗法(photodynamic therapy,PDT)、抗血管生成药物及联合疗法等。我们从历史、流行病学、病理学、发病机制、基因、临床表现、影像学、诊断及治疗等方面对其进行综述,并提出了对PCV的研究方向的讨论和展望。     

老年性黄斑变性和息肉状脉络膜血管病变

老年性黄斑变性(age-relatedmaculardegeneration,AMD)根据病理和临床表现分为萎缩型(atrophicsenilemaculardegeneration)和渗出型(exudativesenilemaculardegeneration)。萎缩型AMD主要为脉络膜毛细血管萎缩、玻璃膜增厚和视网膜色素上皮萎缩等所致的黄斑区萎缩变性;渗出型AMD主要为玻璃膜破坏、脉络膜血管侵入视网膜下形成新生血管,导致视网膜和/或色素上皮有浆液和/或出血[1]。以往息肉状脉络膜血管病变(polypoidalchoroidalvasculopathy,PCV)在诊断和治疗方面与渗出型AMD无明确区别,1990年Yannuzzi等[2]对该病进行了描述,因发病…     

特发性息肉状脉络膜血管病变

特发性息肉状脉络膜血管病变 (idiopathicpoly poidalchoroidalvasculopathy ,IPCV) ,以往〔1〕称之为后部葡萄膜出血综合症或黑人女性多发反复出血性浆液性视网膜色素上皮脱离。 1994年 ,Yannuzzi等〔2〕首次把 11例 4 0～ 70岁患者出现的视网膜下血管息肉状改变导致的视网膜色素上皮严重出血性脱离 ,黄斑功能障碍的疾病命名为IPCV。十年来 ,IPCV的报道逐渐增多 ,对IPCV的认识也逐步深入 ,我们在此做一简要介绍。一、流行病学意大利学者Scassallati -Sforzolini等〔3〕回顾分析了 5 0岁以上共 194例曾诊为老年黄斑变性 (age -re la…     

息肉状脉络膜血管病变的研究进展

息肉状脉络膜血管病变(polypoidal choroidal vasculopathy,PCV)与渗出型年龄相关性黄斑变性(age-related macular degeneration,AMD)的临床表现十分相似,但PCV的流行病学特点、自然病程、影像学特征、预后均与渗出型AMD不同.临床上主要通过特异性眼底表现和吲哚青绿血管造影进行诊断及鉴别.目前PCV的病因、发病机制仍不清,治疗方法主要有激光光凝、光动力治疗、抗血管内皮生长因子药物治疗及联合治疗.     

息肉样脉络膜血管病变的诊断及治疗

息肉样脉络膜血管病变(polypoidal choroidal vasculopathy,PCV)特征是后极部视网膜下桔红色息肉样病变,伴有出血性和浆液性视网膜色素上皮及神经上皮脱离.荧光素眼底血管造影无特征性表现,吲哚青绿血管造影能显示分支血管组成的脉络膜血管网和血管末端息肉样或动脉瘤样扩张.OCT表现为视网膜色素上皮及脉络膜毛细血管高反射层呈陡峭的穹窿状隆起,其下见中等反射或结节状改变.本病应与湿性年龄相关性黄斑变性和某些长期不愈的中心性浆液性脉络膜视网膜病变进行鉴别.尽管PCV的治疗仍缺乏通用的方案,光动力疗法结合玻璃体内注射血管内皮生长因子抑制剂可改善或稳定患者视力.     

玻璃体腔注射康柏西普治疗湿性老年性黄斑变性及息肉状脉络膜血管病变

目的：研究玻璃体腔注射康柏西普(Conbercept)治疗湿性老年性黄斑变性(wet age-related macular degeneration, wAMD)和息肉状脉络膜血管病变(polypoidal choroidal vasculopathy, PCV)的疗效。

方法：临床确诊为wAMD患者16例16眼,确诊为PCV患者5例5眼纳入研究。所有患者常规行玻璃体腔注射康柏西普治疗,根据需要补充治疗。定期行最佳矫正视力和中央视网膜厚度、脉络膜厚度测量。

结果：6mo时平均注射次数为2.93±0.27。基线及6mo时平均最佳矫正视力分别为0.87±0.52和0.74±0.43,差异无统计学意义。6mo时视网膜中央厚度从352.24±131.82降至251.73±69.41μm(P<0.01),脉络膜厚度从331.93±115.35降至304.72±104.59μm(P<0.05)。

结论：玻璃体腔注射康柏西普治疗wAMD及PCV可稳定视力,降低患眼视网膜厚度,具有良好的安全性。     

Use of fluorescein and indocyanine green angiography in polypoidal choroidal vasculopathy patients following photodynamic therapy

Background: Exudative age-related macular degeneration (AMD) is a sight-threatening event in many elderly people. Some patients have a much better outcome in visual acuity (VA) than others after treatment with photodynamic therapy (PDT) with verteporfin. The combination of fluorescein angiography (FA) and indocyanine green (ICG) angiography using the Heidelberg Retina Angiograph II (HRA 2) should make a delineation of distinct pattern(s) possible in order to better select and assess therapy.Methods: This is a retrospective, case-control, single-centre study. We identified a total of 168 eyes of 168 patients from July 2003 to June 2006, including 30 eyes of 30 patients with better visual outcome, defined in this study as VA ≤0.48 logMAR (≥20/60 Snellen chart) at the end of the study. Best-corrected VA, maximal central retinal thickness as measured by optical coherence tomography, and results of the FA/ICG angiography using the HRA 2 were analyzed. In this article, we discuss patients with polypoidal choroidal vasculopathy (PCV) and their characteristics.Results: The average follow-up time was 15.3 months (range 4–28 months). Seventeen (57%) of the 30 patients with better visual outcome had PCV. All patients in the group with better visual outcome needed fewer PDT treatments compared with our control group of patients with an exudative AMD.Interpretation: Simultaneous FA/ICG angiography using the HRA 2 allowed delineation of a subgroup of patients with PCV who showed a better visual outcome compared with those with other types of exudative AMD, after treatment with PDT.     

Incidence and clinical patterns of polypoidal choroidal vasculopathy in Korean patients

Purpose To determine the incidence, demographic features, and clinical characteristics of polypoidal choroidal vasculopathy (PCV) in Korean patients. Methods A retrospective review was undertaken of 392 eyes of 321 symptomatic patients suspected of having exudative age-related macular degeneration (AMD) after their first visit to a tertiary hospital between February 2002 and May 2006. All patients underwent a complete ophthalmic examination, including fluorescein and indocyanine green angiography (ICGA). Results Of the 321 patients (392 eyes), 79 (98 eyes, 24.6%) were diagnosed with PCV. The mean PCV patient age was 64.6 ± 7.6 years. PCV was more common in men (78.5%), and was usually unilateral (75.9%). In terms of PCV clinical manifestation, 52% of patients showed an exudative pattern, 34.7%, a hemorrhagic pattern, and 13.3%, an extensive hemorrhagic pattern. The mean visual acuity at presentation was 0.231 ± 0.256. Classification was based on ICGA findings; 52% of patients showed relatively large aneurismal dilations, 25.5% showed atypical vessel deformations, and 22.5% showed dense clusters of numerous small hyperfluorescent dots. Conclusions The incidence of PCV in Korean exudative AMD patients was relatively high compared with that in other ethnic groups. As in other Asian patient populations, PCV occurred more commonly in men and was predominantly unilateral.     

Polypoidal choroidal vasculopathy with drusen

Purpose To study the prevalence and clinical features of drusen in eyes with polypoidal choroidal vasculopathy (PCV). Methods Retrospectively, we reviewed the records of 98 consecutive patients with PCV (102 eyes). Drusen were examined in the inner macula, which was defined as an area within 1500μm of the center of the fovea. Eyes that had at least one intermediate or large (≥63μm) soft druse within the inner macula were defined as having drusen. Results In these 102 eyes with PCV, 23 (23%) showed soft drusen in the inner macula. In such cases, the fellow eyes, those without a PCV lesion, sometimes also showed soft drusen in the inner macula (11 eyes); 34 (33%) of the eyes with PCV had soft drusen in the inner macula of at least one eye. Comparison of the group of eyes with PCV and drusen in one or both eyes with the group with PCV and no drusen in either eye revealed no significant difference in patient characteristics or in ocular manifestations. Conclusions Drusen are not an unusual feature of PCV, at least in Japanese patients, and, when present, they appear to have only a minor effect, if any, on the clinical course.     

Polypoidal choroidal vasculopathy

Polypoidal choroidal vasculopathy was first described as a peculiar hemorrhagic disorder of the macula, characterized by recurrent sub-retinal and sub-retinal pigment epithelium bleeding in middle aged black women. The use of indocyanine green angiography and subsequently of optical coherent tomography has widened our ability to study and understand the pathophysiology of this disorder. The primary abnormality involves the choroidal circulation, and the characteristic lesion is an inner choroidal vascular network of vessels ending in an aneurysmal bulge or outward projection, visible clinically as a reddish orange, spheroid, polyp-like structure. We have also recognized that individuals of African-American and Asian descents are more at risk for developing polypoidal choroidal vasculopathy as the disorder seems to preferentially affect pigmented individuals. However, it has been shown that while that still holds true, patients of other racial backgrounds may be afflicted. Particularly, polypoidal choroidal vasculopathy has been found to be present in about 8-13% of white patients with clinical appearance of exudative age-related macular degeneration. Polypoidal choroidal vasculopathy has also been reported in Irish, French, German, and Italian patients. The natural course of the disease often follows a remitting-relapsing course, and clinically, it is associated with chronic, multiple, recurrent serosanguineous detachments of the retinal pigment epithelium and neurosensory retina with long-term preservation of good vision. Photodynamic treatment appears to be a promising alternative to conventional laser therapy, for the treatment of polypoidal choroidal vasculopathy. In conclusion, polypoidal choroidal vasculopathy seems to be a distinct clinical entity that should be differentiated from other types of choroidal neovascularization associated with age-related macular degeneration and other known choroidal degenerative, inflammatory, and ischemic disorders.     

Twenty-four-month real-life treatment outcomes of polypoidal choroidal vasculopathy versus type 1 macular neovascularization in Caucasians

Background

To compare 24-month real-world outcomes of Vascular Endothelial Growth Factor (VEGF) inhibitors for Polypoidal Choroidal Vasculopathy (PCV) and type 1 Macular Neovascularization (MNV) in a Caucasian population.

Methods

Retrospective analysis from a prospectively designed observational database. Data from Italian centres participating in the Fight Retinal Blindness! (FRB!) project were collected. Treatment-naïve PCV or type 1 MNV commencing treatment after January 2009 were included. The primary outcome was 24-month visual acuity (VA) change; other outcomes included baseline characteristics, number of anti-VEGF injections, time to lesion inactivation and proportion of active visits.

Results

A total of 322 eyes (114 PCVs) from 291 patients were included. Median [Q1, Q3] VA at baseline was comparable (70 [55, 75.8] vs. 70 [58.8, 75] letters, p = 0.95). Adjusted VA change at 2 years was higher in PCV (mean [95% CI], +1.2 [−1.6, 4.1] vs. −3.6 [−6, −1.2] letters, p = 0.005). PCV received fewer anti-VEGF injections over the first 24 months of treatment than type 1 MNV (median [Q1, Q3], 8 [5, 10] vs. 9 [7, 12.2] injections, p = 0.001), inactivated earlier (median [Q1, Q3], 235 [184, 308] vs. 252 [169, 343] days, p = 0.04) and was less frequently graded ‘active’ (62% vs. 68% of visits, p = 0.001).

Conclusions

PCV had slightly better VA outcomes over 24 months of treatment than type 1 MNV after receiving less anti-VEGF injections. These results suggest a possible overlap of the two clinical entities with similar visual prognosis in Caucasians.