视网膜血管瘤样增生的诊断与治疗

视网膜血管瘤样增生(retinal angiomatous proliferation,RAP)是湿性老年性黄斑变性(age-related macular degeneration,AMD)隐匿性新生血管的另一种病变形式,起源于黄斑旁视网膜深层毛细血管层,以多发性小灶状视网膜内出血、视网膜色素上皮脱离(pigment epithelial detachment,PED)、视网膜-脉络膜血管吻合(retinal-choroidal anastomosis,RCA)为特点,对视力损害严重。本文对近年来的有关文献进行复习,并就RAP的发病机制、临床分期、诊断特点、治疗及预后进行综述。     

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。     

急性中心性浆液性脉络膜视网膜病变三维光相干断层扫描图像特征

中心性浆液性脉络膜视网膜病变(CSC)是常见的眼底病变,荧光素眼底血管造影(FFA)可观察到CSC视网膜色素上皮(RPE)水平的"墨渍"状,"炊烟"状等不同状态的渗漏[1].二维光相干断层扫描(OCT)可显示CSC视网膜神经上皮层和RPE脱离反光带等视网膜横截面图像特征[2],但二维OCT只能反映单一视网膜切面,无法提供整个视网膜神经上皮层脱离区域的RPE脱离状况.     

急性中心性浆液性脉络膜视网膜病变三维光相干断层扫描图像特征

中心性浆液性脉络膜视网膜病变(CSC)是常见的眼底病变,荧光素眼底血管造影(FFA)可观察到CSC视网膜色素上皮(RPE)水平的"墨渍"状,"炊烟"状等不同状态的渗漏[1].二维光相干断层扫描(OCT)可显示CSC视网膜神经上皮层和RPE脱离反光带等视网膜横截面图像特征[2],但二维OCT只能反映单一视网膜切面,无法提供整个视网膜神经上皮层脱离区域的RPE脱离状况.     

急性中心性浆液性脉络膜视网膜病变三维光相干断层扫描图像特征

中心性浆液性脉络膜视网膜病变(CSC)是常见的眼底病变,荧光素眼底血管造影(FFA)可观察到CSC视网膜色素上皮(RPE)水平的"墨渍"状,"炊烟"状等不同状态的渗漏[1].二维光相干断层扫描(OCT)可显示CSC视网膜神经上皮层和RPE脱离反光带等视网膜横截面图像特征[2],但二维OCT只能反映单一视网膜切面,无法提供整个视网膜神经上皮层脱离区域的RPE脱离状况.     

急性中心性浆液性脉络膜视网膜病变三维光相干断层扫描图像特征

中心性浆液性脉络膜视网膜病变(CSC)是常见的眼底病变,荧光素眼底血管造影(FFA)可观察到CSC视网膜色素上皮(RPE)水平的"墨渍"状,"炊烟"状等不同状态的渗漏[1].二维光相干断层扫描(OCT)可显示CSC视网膜神经上皮层和RPE脱离反光带等视网膜横截面图像特征[2],但二维OCT只能反映单一视网膜切面,无法提供整个视网膜神经上皮层脱离区域的RPE脱离状况.     

急性中心性浆液性脉络膜视网膜病变三维光相干断层扫描图像特征

中心性浆液性脉络膜视网膜病变(CSC)是常见的眼底病变,荧光素眼底血管造影(FFA)可观察到CSC视网膜色素上皮(RPE)水平的"墨渍"状,"炊烟"状等不同状态的渗漏[1].二维光相干断层扫描(OCT)可显示CSC视网膜神经上皮层和RPE脱离反光带等视网膜横截面图像特征[2],但二维OCT只能反映单一视网膜切面,无法提供整个视网膜神经上皮层脱离区域的RPE脱离状况.     

急性中心性浆液性脉络膜视网膜病变三维光相干断层扫描图像特征

中心性浆液性脉络膜视网膜病变(CSC)是常见的眼底病变,荧光素眼底血管造影(FFA)可观察到CSC视网膜色素上皮(RPE)水平的"墨渍"状,"炊烟"状等不同状态的渗漏[1].二维光相干断层扫描(OCT)可显示CSC视网膜神经上皮层和RPE脱离反光带等视网膜横截面图像特征[2],但二维OCT只能反映单一视网膜切面,无法提供整个视网膜神经上皮层脱离区域的RPE脱离状况.     

急性中心性浆液性脉络膜视网膜病变三维光相干断层扫描图像特征

中心性浆液性脉络膜视网膜病变(CSC)是常见的眼底病变,荧光素眼底血管造影(FFA)可观察到CSC视网膜色素上皮(RPE)水平的"墨渍"状,"炊烟"状等不同状态的渗漏[1].二维光相干断层扫描(OCT)可显示CSC视网膜神经上皮层和RPE脱离反光带等视网膜横截面图像特征[2],但二维OCT只能反映单一视网膜切面,无法提供整个视网膜神经上皮层脱离区域的RPE脱离状况.     

急性中心性浆液性脉络膜视网膜病变三维光相干断层扫描图像特征

中心性浆液性脉络膜视网膜病变(CSC)是常见的眼底病变,荧光素眼底血管造影(FFA)可观察到CSC视网膜色素上皮(RPE)水平的"墨渍"状,"炊烟"状等不同状态的渗漏[1].二维光相干断层扫描(OCT)可显示CSC视网膜神经上皮层和RPE脱离反光带等视网膜横截面图像特征[2],但二维OCT只能反映单一视网膜切面,无法提供整个视网膜神经上皮层脱离区域的RPE脱离状况.     

Retinal angiomatous proliferation: clinical characteristics and treatment options.

BACKGROUND: A new form of exudative age-related macular degeneration (ARMD), retinal angiomatous proliferation (RAP), has been described in which neovascularization begins in the deep retina, extends through the subretinal space, and eventually communicates with choroidal neovascularization. METHODS: Case series. RESULTS: Common clinical features of RAP include small multiple intra-retinal hemorrhages, intra-retinal edema, vascularized pigment epithelial detachments (PEDs), and retinal choroidal anastomosis (RCA). Fluorescein angiography (FA) reveals ill-defined, occult choroidal neovascularization. Indocyanine green (ICG) angiography is useful in early stages because 'hot spots' can be detected before clinical or FA characteristics are present. Optical coherence tomography (OCT) is useful in illustrating some of the clinical and FA characteristics. The use of photodynamic therapy (POT), combined with intravitreal triamcinolone injection, was successful in stabilizing the RAP lesion in one case discussed in this report. CONCLUSIONS: Retinal angiomatous proliferation is a newly recognized entity of exudative age-related macular degeneration with its own set of clinical, FA, ICG angiography, and OCT features. Experimental treatments such as the use of PDT combined with intravitreal triamcinolone injection demonstrate potential success with this entity. The biggest hope appears to be anti-angiogenic factors currently in clinical trials for the treatment of exudative ARMD.     

中心性渗出性脉络膜视网膜病变的相干光断层扫描结果分析

目的 探讨中心性渗出性脉络膜视网膜病变(CEC)黄斑部视网膜下脉络膜新生血管(CNV)患者的相干光断层扫描(OCT)图像特征。方法 对20例(21只眼)CEC连续治疗患者进行OCT检查,并与荧光素眼底血管造影(FFA)和吲哚青绿眼底血管造影(ICGA)结果进行对比;同时对光动力治疗后患者的OCT图像形态变化进行分析,以评价OCT图像特征对CEC患者的临床治疗价值。结果 21只眼中,有16只眼CNV呈类圆形团块状,自视网膜色素上皮层向上突出,位于视网膜神经上皮下间隙,呈强或中等强度反射;5只眼的CNV呈纺锤形或不规则形,亦呈强或中等强度反射,位于色素上皮层平面。21只眼中有9只眼伴有浆液性神经上皮脱离,6只跟伴有出血性色素上皮脱离,14只眼伴有不同程度的视网膜水肿和增厚。17只眼经光动力治疗后,随访3～12个月,平均6个月,FFA检查显示荧光素渗漏消退或减弱的患者,OCT检查均显示CNV团块不同程度退缩,其形态亦发生变化。OCT图像特征与FFA和ICG检查结果有互补性。结论 OCT检查可以确定CEC患者病变中CNV团块的形态、大小及位置。CEC病变中CNV的OCT图像以突出于色素上皮层的类圆形团块为特征,其形态和大小可因治疗和观察时间而变化。     

Peripheral retinal detachments and retinal pigment epithelial atrophic tracts secondary to central serous pigment epitheliopathy

Twenty-five patients with central serous pigment epitheliopathy (CSP), also known as central serous chorioretinopathy, have been observed to have inferior hemispheric retinal pigment epithelial atrophic tracts, presumptive of antecedent retinal detachments. Five of these patients were noted to have clinically discernible, dependent peripheral retinal detachments. The clinical and fluorescein angiographic features of these patients are reviewed. Alterations in the retina, the retinal pigment epithelium (RPE) and the choroid are also described. They include the commonly associated manifestations of CSP such as RPE leaks and macular detachment as well as some newly recognized disturbances such as retinal capillary dilatation (telangiectasia), retinal capillary leakage, retinal lipid deposition, cystoid macular edema, choriocapillaris atrophy, choroidal neovascularization and disciform scarring.     

Peripheral retinal detachments and retinal pigment epithelial atrophic tracts secondary to central serous pigment epitheliopathy. 1984

Twenty-five patients with central serous pigment epitheliopathy (CSP), also known as central serous chorioretinopathy, have been observed to have inferior hemispheric retinal pigment epithelial atrophic tracts, presumptive of antecedent retinal detachments. Five of these patients were noted to have clinically discernible, dependent peripheral retinal detachments. The clinical and fluorescein angiographic features of these patients are reviewed. Alterations in the retina, the retinal pigment epithelium (RPE) and the choroid are also described. They include the commonly associated manifestations of CSP such as RPE leaks and macular detachment as well as some newly recognized disturbances such as retinal capillary dilatation (telangiectasia), retinal capillary leakage, retinal lipid deposition, cystoid macular edema, choriocapillaris atrophy, choroidal neovascularization and disciform scarring.     

Subretinal choroidal neovascularization as a response to penetrating retinal injury in the pigmented rabbit

Subretinal choroidal neovascularization has been identified in the pigmented rabbit in association with penetrating retinal injury. In this study, the subretinal new vessels were not contained within the fibrous scar, but were located posterior to it and impinged on the visual streak. Histology revealed subretinal neovascularization in the region of transition from normal to atrophic retina. These new vessels originated from the choroidal vessels and extended through a spontaneous break in Bruch's membrane into the retinal pigment epithelium. There was degeneration of the photoreceptors and hyperplasia of the retinal pigment epithelium in the region of neovascularization. The subretinal new vessels identified in the pigmented rabbit have many of the morphological features seen in the human clinical condition, and therefore this animal model may be useful in studying human choroidal neovascularization.     

Optical coherence tomographic features of idiopathic submacular choroidal neovascularization

PURPOSE: To clarify the morphologic features of idiopathic submacular choroidal neovascularization using optical coherence tomography. METHODS: Using optical coherence tomography, we prospectively examined 17 consecutive eyes (17 patients; eight men, nine women; mean age +/- SD, 36.0 +/- 9.3 years) with idiopathic submacular choroidal neovascularization. During the follow-up period (mean +/- SD, 7.5 +/- 3.8 months), we repeated optical coherence tomography in 15 eyes; optical coherence tomography was performed only at the initial examination in two eyes that underwent submacular surgery. RESULTS: Choroidal neovascularization was observed in the subsensory retinal space anterior to the retinal pigment epithelium as a highly or moderately reflective mass, which protruded from the retinal pigment epithelium in 13 of 17 eyes (protruding type). In four eyes, choroidal neovascularization was a highly reflective fusiform mass at the level of the retinal pigment epithelium and choriocapillaris (fusiform type). During the follow-up period, choroidal neovascularization regressed and the protruding mass became a highly reflective fusiform mass in 10 of 11 eyes with the protruding type that underwent repeated examination. In three of the 10 eyes, during 5 to 10 months from initial examination, the choroidal neovascularization was exacerbated and it again protruded into the subsensory retinal space anterior to the retinal pigment epithelium. Four eyes with the fusiform type remained the same throughout the follow-up period. The overlying retina was thickened and had low reflectivity in all 17 eyes at the initial examination. Optical coherence tomography identified subretinal fluid in only 11 of the 13 eyes with protruding type choroidal neovascularization. CONCLUSIONS: Optical coherence tomography confirmed subsensory retinal location of idiopathic submacular choroidal neovascularization anterior to the retinal pigment epithelium. Protruding choroidal neovascularization transforms to fusiform tissue at the level of the retinal pigment epithelium during the process of regression. Sensory retinal edema was always associated with choroidal neovascularization.     

Retinal angiomatous proliferation in age-related macular degeneration

BACKGROUND: It is known that choroidal neovascularization (CNV) in age-related macular degeneration (ARMD) may erode through the retinal pigment epithelium, infiltrate the neurosensory retina, and communicate with the retinal circulation in what has been referred to as a retinal-choroidal anastomosis (RCA). This is extremely common in the end stage of disciform disease. In recent years, the reverse also seems to be possible, as angiomatous proliferation originates from the retina and extends posteriorly into the subretinal space, eventually communicating in some cases with choroidal new vessels. This form of neovascular ARMD, termed retinal angiomatous proliferation (RAP) in this article, can be confused with CNV. PURPOSE: The purpose of this article is 1) to review the clinical and angiographic characteristics of a series of patients with RAP and 2) to propose a theoretical sequence of events that accounts for the neovascularized process. METHODS: In this retrospective clinical and angiographic analysis, 143 eyes with RAP (108 patients) were reviewed and classified based on their vasogenic nature and course. Clinical biomicroscopic examination, fluorescein angiography, and indocyanine green angiography were used to evaluate patients. RESULTS: The results of this series suggest that angiomatous proliferation within the retina is the first manifestation of the vasogenic process in this form of neovascular ARMD. Dilated retinal vessels and pre-, intra-, and subretinal hemorrhages and exudate evolve, surrounding the angiomatous proliferation as the process extends into the deep retina and subretinal space. One or more dilated compensatory retinal vessels perfuse and drain the neovascularization, sometimes forming a retinal-retinal anastomosis. Fluorescein angiography in these patients usually revealed indistinct staining simulating occult CNV. Indocyanine green angiography was useful to make an accurate diagnosis in most cases. It revealed a focal area of intense hyperfluorescence corresponding to the neovascularization ("hot spot") and other characteristic findings. Based on understanding of the nature and progression of the neovascularized process, patients with RAP were classified into three vasogenic stages. Stage I involved proliferation of intraretinal capillaries originating from the deep retinal complex (intraretinal neovascularization [IRN]). Stage II was determined by growth of the retinal vessels into the subretinal space (subretinal neovascularization [SRN]). Stage III occurred when CNV could clearly be determined clinically or angiographically. A vascularized pigment epithelial detachment and RCA were inconsistent features of this stage. CONCLUSIONS: Retinal angiomatous proliferation appears to be a distinct subgroup of neovascular ARMD. It may present in one of three vasogenic stages: IRN, SRN, or CNV. Whereas ICG angiography is helpful in diagnosing RAP and in documenting the stage of the neovascularized process, it is frequently difficult to determine the precise nature and location of the new vessel formation. It is important for clinicians to recognize the vasogenic potential and the associated manifestations of this peculiar form of neovascular ARMD so that a proper diagnosis can be made, and when possible, an appropriate management administered.     

Angiographic aspects of senile macular degeneration: spontaneous course

Between 1971 and 1981, 45 patients (50 eyes) with a senile macular degeneration were seen at an early stage of the disease. These patients were followed for 6 to 72 months (mean follow-up 32 months). The early morphological features and evolution of the lesions were analysed by fluorescein angiography. Three different angiographic types of subretinal new vessels were identified in this study. These were: angiographically visible vascular networks (27 cases) subretinal new vessels hidden behind retinal pigment epithelium modifications (9 cases); and subretinal new vessels associated with pigment epithelial detachment (14 cases). In the first group subretinal new vessels were clearly visible on the angiogram, as a distinct network of cart wheel-like capillary plexuses which fill very early in the arterial phase. Progressive leakage of dye hides this capillary plexus during the late phase. In the second group a pigment epithelial detachment, usually identified on biomicroscopic examination, was associated with neovascularization visible as a hyperfluorescent hot spot or as an uneven hyperfluorescence on the border of the RPE detachment. Other angiographic features are a non-circular shape, irregular filling of the pigment epithelial detachment, and uneven late hyperfluorescence. In the third group, occult neovascularization was usually located behind the retinal pigment epithelium. When subretinal neovascularization was suspected by the presence of a disciform lesion, angiography, showed an irregular pattern of delayed hyperfluorescence with late leakage. These features permit putative diagnosis without precise localization. This clinical study attempted to establish the evolution and prognosis of three clinical forms of subretinal new vessels, in order to more accurately establish the indications of photocoagulation treatment.     

Retinal pigment epithelial detachment

Detachment of the retinal pigment epithelium is a prominent feature of many chorio-retinal disease processes, the most prevalent of which is age-related macular degeneration (AMD). Detachment of the retinal pigment epithelium may or may not be associated with choroidal neovascularization and may be caused by different types of pathogenesis, each associated with distinct angiographic features, natural course, visual prognosis, and response to treatment. The phrase "detachment of the retinal pigment epithelium" is used quite often, not always in the correct association and with no clear differentiation between its various types. It is important to identify the specific nature of detachment of the retinal pigment epithelium, and to establish an accurate diagnosis and treatment plan. Therefore, we present a review of the existing types of detachment of the retinal pigment epithelium with what we propose as being appropriate nomenclature and classification, and potential treatment recommendations.     

High resolution Fourier-domain optical coherence tomography of retinal angiomatous proliferation

PURPOSE: To study the anatomic details of retinal angiomatous proliferation (RAP) in patients with age-related macular degeneration (AMD) using high-resolution Fourier-domain optical coherence tomography (Fd-OCT) and its three-dimensional reconstructions. METHODS: A Fd-OCT instrument was used to image five patients clinically diagnosed with RAP. A series of 100 raster-scanned B-scans centered over the macula was registered and rendered as a three-dimensional volume. These retinal structures were analyzed for anatomic details of the RAP lesions. RESULTS: The RAP lesion could be identified within the retina on Fd-OCT in all five cases. Fd-OCT images of the first four cases revealed areas of intraretinal neovascularization (IRN) in the deep retina adjacent to a pigment epithelial detachment (PED). There was neovascular proliferation anteriorly and posteriorly through a break in the retinal pigment epithelium (RPE). In three of the four cases, Bruch membrane remained intact. There was no identifiable choroidal neovascularization (CNV). The fifth case had both subretinal and sub-RPE neovascular membranes without a PED. CONCLUSION: Fd-OCT provides unprecedented in vivo detail of the anatomy of RAP lesions that nearly resembles histologic specimens. This study suggests that the initial neovascular process in RAP can originate either within the retina or in the sub-RPE space.