视网膜血管瘤样增生

视网膜血管瘤样增生（RAP）是年龄相关性黄斑变性的一种特殊类型,起源于视网膜感觉层毛细血管。患者女性,50岁,右眼0．3。右眼黄斑区视网膜轻度水肿、隆起,中心凹上方约1／2DD黄色病灶与视网膜血管连接（图1A）。荧光素眼底血管造影（FFA）检查显示：     

视网膜血管瘤样增生的研究概况

视网膜血管瘤样增生（retinal angiomatous proliferation，RAP）是年龄相关性黄斑变性。（ARMD）的一种特殊类型。通常意义上的ARMD起源于脉络膜，而RAP起源于视网膜感觉层毛细血管。这种特殊类型的ARMD最近受到临床医师和研究人员的密切关注。本文就RAP的研究历程、临床表现、病理学特点、形成机制、分期及诊断和治疗现状进行综述。     

视网膜血管瘤样增生治疗进展

视网膜血管瘤样增生（RAP）是渗出性年龄相关性黄斑变性（AMD）的一种新形式，占新诊断AMD的12％-15％。近年来，对RAP的治疗已经进行了大量的多中心临床试验，涌现出激光光凝、经瞳孔温热疗法、手术治疗、光动力疗法、玻璃体腔内注药及联合治疗等方法。本文就RAP目前的治疗方法及其研究进展作一综述。     

视网膜血管瘤样增生的研究概况

视网膜血管瘤样增生(RAP)是年龄相关性黄斑变性的一种特殊类型,它起源于黄斑旁视网膜深层毛细血管网,并能不断增殖突破视网膜下间隙最终与脉络膜血管吻合.本文就视网膜血管瘤样增生的研究历程、分期、临床表现、诊断及治疗现状作一综述.     

视网膜血管瘤样增生一例

视网膜血管瘤样增生(retinal angiomatous proliferation,RAP)临床较为罕见,是年龄相关性黄斑变性(age-related macular degeneration,AMD)的一种特殊类型.通常意义上的AMD起源于脉络膜,而RAP起源于视网膜感觉层毛细血管.我们在临床上发现1例典型RAP,现报告如下.     

视网膜血管瘤性的增生 光学相干断层检查证实的一个视网膜内病灶

视网膜血骨瘤增生逐渐被认识到是年龄相关性黄斑变性中新生血管的一种变异。视网膜血管瘤性增生被认为是视网膜内新生血管芽。这些血管芽长入视网膜下腔导致浆液性视网膜色素上皮脱离并且可以与脉络膜新生血管吻合。我们报道1例视网膜视网膜血管瘤性的增生合并浆液性视网膜色素上皮脱离．通过光学相干断层扫描（OCT）证实有视网膜内新生血管。     

视网膜血管瘤样增生的研究概况

视网膜血管瘤样增生(retinalangiomatousproliferation,RAP)是年龄相关性黄斑变性(ARMD)的一种特殊类型。通常意义上的ARMD起源于脉络膜,而RAP起源于视网膜感觉层毛细血管。这种特殊类型的ARMD最近受到临床医师和研究人员的密切关注。本文就RAP的研究历程、临床表现、病理学特点、形成机制、分期及诊断和治疗现状进行综述。     

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。     

视网膜血管瘤样增生的眼底影像特征

自Hartnett等[1]首次描述了发生于老年性黄斑变性(AMD)患者“深层视网膜血管异常复合体”以来,视网膜血管瘤样增生( RAP)逐渐被认可为一种渗出型AMD的特殊类型[2,3].与其他类型的AMD不同,RAP的新生血管来源于视网膜深层毛细血管,并纵行向视网膜下腔发展,形成视网膜脉络膜吻合.Yannuzi等[4 ]研究发现,RAP常发生于白人,亚洲人中并不常见;且国内关于该病的报道也较少.为此,我们观察了一组RAP患者的影像学特征,现将结果报道如下.     

视网膜血管瘤样增生和湿性年龄相关性黄斑病变的鉴别

视网膜血管瘤样增生(retinal angiomatous proliferation,RAP)是湿性年龄相关性黄斑变性(age-related macular degeneration,AMD)的一种特殊类型,临床一般分三期,晚期有新生血管形成,但与起源于脉络膜的新生血管不同,多数学者认为RAP的新生血管起源于视网膜深层毛细血管网。目前对RAP和湿性AMD两者到底是同一种疾病的不同亚型还是独立的两种疾病还存在争议,由于两者的病程、预后及治疗存在差异,因此临床鉴别诊断十分重要。近年来,随着吲哚菁绿血管造影(indocyanine green angiography,ICGA)技术和新一代光学相干断层扫描(optical coherence tomography,OCT)技术的应用,对RAP和湿性AMD的鉴别取得了显著进步。本文就两者的临床表现、自然病程、治疗方法及预后等方面做一综述。     

Retinal Angiomatous Proliferation in an Eye with Cuticular Drusen

Purpose

To report the presence of retinal angiomatous proliferation (RAP) in an eye with cuticular drusen detected by fluorescein and indocyanine green angiography by confocal scanning laser ophthalmoscopy and by spectral-domain optical coherence tomography (SD-OCT).

Methods

Case report of a 65-year-old Japanese woman with cuticular drusen.

Results

At her first ophthalmic examination, her visual acuity was 20/20 in both eyes. An ophthalmoscopy showed many small subretinal pigment epithelial deposits in both eyes. These deposits had a ‘saw-tooth pattern’ in the SD-OCT images. During the follow-up examination, retinal hemorrhages were observed, and fluorescein angiography showed a ‘stars-in-the-sky’ appearance and intraretinal neovascularization. The patient was diagnosed with cuticular drusen associated with RAP.

Conclusion

We suggest that the cuticular drusen were associated with RAP, so periodic follow-up examinations are needed for patients with cuticular drusen for the early detection and treatment of RAP.Key Words: Cuticular drusen, Basal laminar drusen, Retinal angiomatous proliferation, Spectral domain optical coherence tomography     

Characteristic Findings of Optical Coherence Tomography in Retinal Angiomatous Proliferation

Purpose

To identify the unique pathologic findings of retinal angiomatous proliferation (RAP) in optical coherence tomography (OCT).

Methods

Retrospectively, 29 eyes of 25 patients with age-related macular degeneration and complicated RAP were analyzed. All 29 eyes had choroidal neovascularization (CNV) in the area of pigment epithelial detachment (PED) or adjacent to it, which was visible with fluorescein angiography or indocyanine green angiography. Cross-sectional images were obtained by OCT scanning through the CNV lesions.

Results

Six distinctive findings of OCT included drusen (100%), inner retinal cyst (80%), outer retinal cyst (68%), fibrovascular PED (84%), serous retinal detachment (40%), and PED (68%).

Conclusions

Through analysis of OCT findings, we revealed six different types of lesions distinctive of RAP which may provide helpful diagnostic information for subsequent treatment and predicting the prognosis of RAP.     

Retinal Pigment Epithelium Tear after Vitrectomy for Vitreomacular Traction Syndrome in an Eye with Retinal Angiomatous Proliferation

An 87-year-old Japanese man presented with retinal angiomatous proliferation (RAP) and a retinal pigment epithelium (RPE) detachment in his right eye. His decimal best-corrected visual acuity was 0.15 in the right eye, and optical coherence tomography (OCT) showed a vitreomacular adhesion in the right eye as well. After 3 monthly intravitreal injections of ranibizumab, the size and height of the RPE detachment was significantly reduced. The accumulated intra- and subretinal fluid also disappeared, but the vitreomacular traction remained. Pars plana vitrectomy was performed, and the posterior hyaloid was separated from the retina with a vitrectomy cutter without any intraoperative complications. Two months after the surgery, a large RPE tear was observed over the macular area. His visual acuity decreased to 0.06 and remained unchanged thereafter. We suggest that the small tear led to the larger RPE tear because vitreomacular traction was transmitted to the RPE through the fibrovascular tissue of the RAP during the creation of the hyaloid detachment. Because such an RPE tear has not been reported after vitrectomy for vitreomacular traction, surgeons need to pay special attention to this potential complication in eyes with vitreomacular traction and RAP.Key Words: Retinal angiomatous proliferation, Vitreomacular traction syndrome, Vitrectomy, Retinal pigment epithelium tear     

Successful Treatment of Retinal Angiomatous Proliferation with Intravitreal Triamcinolone and Ranibizumab Injections in a 67-Year-Old Male

A 67-year-old male who presented to the eye casualty department with deterioration in his vision was diagnosed with retinal angiomatous proliferation. After initial deterioration with ranibizumab intravitreal injections, we have demonstrated successful treatment and stabilised vision with ranibizumab and a single intravitreal triamcinolone injection. Stringent follow-up and top-up ranibizumab injections have stabilised his vision and have shown foveal improvement on optical coherence tomography imaging.Key words: Retinal angiomatous proliferation, Neovascularisation, Triamcinolone, Ranibizumab, Retina, Age-related macular degeneration     

Early Recurrent Hemorrhage in Submacular Hemorrhage Secondary to Type 3 Neovascularization or Retinal Angiomatous Proliferation: Incidence and Influence on Visual Prognosis

Purpose: To evaluate the incidence of early recurrent hemorrhage in submacular hemorrhage secondary to type 3 neovascularization or retinal angiomatous proliferation (RAP) and its influence on visual prognosis.

Methods: This retrospective study included 32 eyes with submacular hemorrhage secondary to type 3 neovascularization or RAP that underwent anti-vascular endothelial growth factor (VEGF) therapy. The eyes exhibiting an increase in the extent of hemorrhage within 6 months after hemorrhage development were included in the early recurrent hemorrhage group, and the remaining eyes were included in the non-early recurrent hemorrhage group. The best-corrected visual acuities (BCVAs) measured at the time of hemorrhage development and at 12 months were compared between the two groups.

Results: During the follow-up period, 8 eyes underwent vitrectomy to clear vitreous hemorrhage, and the remaining 24 eyes underwent anti-VEGF monotherapy. In the early recurrent hemorrhage group (n = 12), the mean logarithm of the minimal angle of resolution BCVA at the time of hemorrhage development and after 12 months was 1.17 ± 0.40 (Snellen equivalents: 20/295) and 2.35 ± 0.59 (20/4477), respectively. In the non-early recurrent hemorrhage group (n = 20), the corresponding values were 1.07 ± 0.43 (20/234) and 1.44 ± 0.71 (20/550), respectively. The BCVA at 12 months was significantly worse in the early recurrent hemorrhage group (P = 0.003) despite comparable BCVA at diagnosis between the two groups (P = 1.000).

Conclusions: Early recurrent hemorrhage was noted in 37.5% of eyes with submacular hemorrhage secondary to type 3 neovascularization or RAP and was closely associated with a poor prognosis.