Idiopathic polypoidal choroidal vasculopathy (IPCV).

Eleven patients, 40 to 71 years old, had a choroidal vasculopathy that led to hemorrhagic and exudative macular degeneration. The patients had peculiar polypoidal, subretinal, vascular lesions associated with serious and hemorrhagic detachments of the retinal pigment epithelium. This macular disorder, which we have named idiopathic polypoidal choroidal vasculopathy (IPCV), appears to represent a distinct entity that differs clinically and demographically from age-related macular degeneration (AMD) and other macular diseases associated with subretinal neovascularization. Recognition of this condition is important because it may have specific risk factors, natural course, and management considerations that differ from those of age-related macular degeneration.     

Idiopathic polypoidal choroidal vasculopathy (IPCV) presenting with simultaneous choroidal neovascular membrane (CNM).

Idiopathic polypoidal choroidal vasculopathy (IPCV), a rare retinal condition initially described in 1982, is characterized by retinal pigment epithelial (RPE) detachments associated with choroidal polypoidals. Although it is recognized as a unique entity, many consider it a peculiar representation of choroidal neovascular membrane (CNM), commonly associated with age-related macular degeneration (ARMD). We report a case of IPCV with simultaneous presentation of CNM. Dilated examination and fluorescein angiography (FA) revealed RPE detachments associated with choroidal polypoidals. FA also revealed a lacy hyperfluorescent vascular lesion. Ocular manifestations, differential diagnoses, and treatment options are discussed, with emphasis on similarities and differences between IPCV and CNM. It is imperative to consider IPCV in the differential diagnosis of RPE detachments, including those associated with CNM. Careful funduscopic evaluation, FA, and/or indocyanine green videoangiography analysis helps confirm the diagnosis.     

Idiopathic polypoidal choroidal vasculopathy leading to blindness

Idiopathic polypoidal choroidal vasculopathy (IPCV) has recently been recognized as a distinct cause of recurrent subretinal hemorrhages and exudates as well as multiple hemorrhagic retinal pigment epithelium detachments in the macula. IPCV is usually considered to have a good visual prognosis. We report the case of a 44-year-old woman with particularly severe bilateral macular and peripheral IPCV. The patient was followed for 13 years and had final visual acuity of counting fingers in the right eye and 20/400 in the left eye. Patients with IPCV with macular choroidal neovascularization tend to have a poor visual prognosis. This case of IPCV should alert the physician to be particularly attentive to the follow-up of these patients.     

Optical coherence tomography (OCT) in idiopathic polypoidal choroidal vasculopathy (IPCV)

Purpose: Idiopathic polypoidal choroidal vasculopathy (IPCV) is a distinct clinical entity characterized by an edematous maculopathy and typical choroidal vascular change. The purpose of this study is to evaluate the use of optical coherence tomography (OCT) for diagnosing of this disease. Methods: 4 patients affected by IPVC (mean age 71–84 years) underwent biomicroscopy with a three mirror conctact lens, fluorescein angiography (FA), indocyanine green angiography (ICGA) and OCT (Humphrey Zeiss) scan. Results: in all 4 cases OCT tomograms acquired at the location of the typical choroidal abnormalities demonstrated by ICGA, revealed a characteristic hyper-reflectivity in the choroidal layers. Conclusion: ICGA is essential to diagnose IPVC, but OCT may be able to identify characteristic reflectivity patterns. This revised version was published online in July 2006 with corrections to the Cover Date.     

Idiopathic polypoidal choroidal vasculopathy and sickle cell retinopathy

PURPOSE: To report a patient who had concomitant idiopathic polypoidal choroidal vasculopathy and sickle cell retinopathy. METHOD: Case report. A 46-year-old black woman presented with sudden onset of "blurring" in the left eye. Her ocular history was unremarkable, and her medical history was relevant for sickle cell disease with yearly episodes of sickle cell crises. RESULTS: Both eyes had evidence of idiopathic polypoidal choroidal vasculopathy and peripheral retinal neovascularization secondary to sickle cell disease. The left eye had hemorrhagic retinal pigment epithelial detachments secondary to idiopathic polypoidal choroidal vasculopathy. CONCLUSION: We present this case as an example of concomitant sickle cell retinopathy and idiopathic polypoidal choroidal vasculopathy. We are unaware of previous reports with these findings.     

Idiopathic polypoidal choroidal vasculopathy associated with central retinal vein occlusion

We present the case of a 54-year-old man with a previous history of central retinal vein occlusion in the right eye, who developed an idiopathic polypoidal choroidal vasculopathy in the right eye and an epiretinal membrane in the left eye. The patient's medical history disclosed arterial hypertension controlled with medical treatment. Although idiopathic polypoidal choroidal vasculopathy has been associated with several conditions, this is the first time that this entity has been described in the literature in association with central retinal vein occlusion.     

Giant non-pulsatile polypoidal choroidal vasculopathy

Letter to the Editor     

Pulsation in polypoidal choroidal vasculopathy

PURPOSE: To evaluate the relationship between pulsation and the fundus lesion in polypoidal choroidal vasculopathy (PCV). OBJECTIVE AND METHODS: We studied 26 eyes with PCV by indocyanine green angiography (IA) with a heidelberg retina angiograph(HRA). The 26 eyes were classified into two groups; pulsatile PCV and non-pulsatile PCV. We evaluated the strength and the amplitude of the pulsation, and the frequency of subretinal hemorrhage within one year after the first IA. Seven eyes in the pulsatile PCV group were tested by HRA twice or more to evaluate the relationship between the fundus lesion and the change in the strength and the amplitude of pulsation. RESULTS: Subretinal hemorrhage occurred in 9 of 14 eyes with pulsatile PCV and in 2 of 12 eyes with non-pulsatile PCV (odds ratio : 12.5). The possibility of subretinal hemorrhage was significantly higher in pulsatile PCV than in non-pulsatile PCV. In 3 of the 7 eyes tested twice or more, the fundus lesion worsened as the pulsation strengthened and the area of pulsation enlarged. In 1 of the 7 eyes, the fundus lesion improved as the pulsation weakened and the area of pulsation decreased. CONCLUSION: There is a good possibility that strengthened and extended pulsation is related to increasing subretinal hemorrhage in PCV patients.     

Plasticity of polypoidal lesions in polypoidal choroidal vasculopathy

Purpose The aim of this study was to describe the clinical course in a patient with polypoidal choroidal vasculopathy (PCV).Methods A 68-year-old man with PCV in the left eye was followed up by means of routine examinations including fluorescein angiography and indocyanine green angiography for over 60 months.Results Throughout the follow-up period, the patient experienced repeated lesions in the macula, such as serosanguineous detachment of the retinal pigment epithelium and neurosensory retina, but retained good visual acuity. Indocyanine green angiography disclosed spontaneous regression of polypoidal vessels followed by significant changes in the choroidal circulation: a group of polypoidal structures disappeared, and after several months a small choroidal vessel became apparent that was distant from the previously observed polypoidal structure rather than representing an extension of the original lesion.Conclusion The clinical observation suggests that in some cases of PCV the choroidal vasculature may be altered with time, in that some vessels in the inner choroid and even the choriocapillaris may close and collateral vessels and/or new vessels may develop to form complex such as that described here.     

Idiopathic polypoidal choroidal vasculopathy: its extreme aspects in one patient--case report

To describe a case of idiopathic polypoidal choroidal vasculopathy and the role of indocyanine green angiography findings in the differential diagnosis of exudative maculopathies, particularly with age-related macular degeneration, and the extreme evolution of idiopathic polypoidal choroidal vasculopathy in one patient. A patient with vitreous hemorrhage was examined and evaluated by fluorescein and indocyanine green angiographies in the right eye and with hemorrhagic detachment of the retinal pigment epithelium in the left eye. The patient was treated by pars plana vitrectomy in the right eye which was followed by retinal detachment and vision loss. In the left eye an involution of the hemorrhagic detachment of the retinal pigment epithelium with preservation of the vision was seen. The idiopathic polypoidal choroidal vasculopathy seems to be a distinct clinical entity that can and should be differentiated from age-related macular degeneration and the fluorescein and indocyanine green angiographies should be performed to evaluate the choroidal vasculature in an attempt to establish a more definitive diagnosis. Particularly in this case the entity had an extreme clinical course in the patient.     

Photodynamic therapy for polypoidal choroidal vasculopathy

Graefe's Archive for Clinical and Experimental Ophthalmology -     

Retinal angiopathy and polypoidal choroidal vasculopathy

PURPOSE: To describe the clinical and angiographic features of patients with polypoidal choroidal vasculopathy, exudative detachment of the macula, and an associated retinal microangiopathy. METHODS: Case series. RESULTS: Four patients with chronic exudative detachment of the macula with a variable degree of lipid deposition are described. The retina in the detached area, but not beyond, was noted to have a microangiopathy. There was capillary telangiectasia, microaneurysm formation, patchy nonperfusion, and intraretinal leakage. In each patient, there were no other retinal vascular changes in the fundus of either eye. The fluorescein angiogram showed subretinal leakage suspicious for occult choroidal neovascularization. The indocyanine green angiogram showed the presence of underlying polypoidal choroidal neovascularization, accounting for the exudative detachment. After photocoagulation, the retinal angiopathy improved, but not completely. CONCLUSION: Retinal microangiopathy may occur in a chronic macular detachment secondary to polypoidal choroidal neovascularization. The development of these secondary retinal changes is not clearly understood; however, hypoxia from the chronic detachment, a neurotoxic effect from the lipid deposition, or a biochemically induced microvascular abnormality from secretion of vasogenic mediators are possible mechanisms. Indocyanine green angiography is helpful in making a definitive diagnosis. Clinicians should be aware that a retinal microangiopathy may occur in such eyes so that the proper diagnosis can be made and appropriate treatment administered.     

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.     

息肉状脉络膜血管病变的随访观察

目的 观察息肉状脉络膜血管病变(PCV)患者的临床特征及其视力预后情况.方法 回顾性病例系列研究.对经眼底彩色照相、荧光素眼底血管造影及吲哚氰绿血管造影等确诊的45例(51只眼)PCV患者的临床资料进行回顾性分析,包括患者年龄、随访前视力、有无脉络膜视网膜病变、病灶面积、病灶是否累及黄斑中心凹、随访期间病灶消退且无新病灶出现等6项因素.对患者随访期间视力变化情况进行多因素相关回归分析.结果 45例(51只眼)PCV患者随访时间6～18个月,平均随访(11.1±2.0)个月.随访期间病变区出血、渗出增多者15只眼(29.4%),出血、渗出减少者25只眼(49.0%),盘状瘢痕与萎缩改变者分别为5只眼(9.8%)和6只眼(11.8%).视力增加、稳定及下降者分别为11(21.6%)、21(41.2%)及19(37.2%)只眼.息肉状病灶累及黄斑中心凹者25只眼(49.0%),未累及者26只眼(51.0%).在病程进展过程中,息肉状病灶部分消退18只眼(35.3%),稳定不变13只眼(25.5%),扩大和新生13只眼(25.5%),消退和新生并存7只眼(13.7%).患者视力变化与年龄、随访前视力、息肉状病灶累及黄斑中心凹诸因素相关(b=-0.005,0.382,-0.430;P=0.034,0.000,0.000),视力提高与息肉状病灶累及黄斑中心凹(b=-2.957,P=0.013,OR=0.052)、息肉状病灶消退且无新病灶出现相关(b=2.259,P=0.019,OR=9.578).结论 PCV患者视力预后情况不同,年龄小者随访前视力较好;息肉状病灶未累及黄斑中心凹者随访视力相对较好,其中无新病灶出现者,随访期间视力提高的可能性较大.脉络膜视网膜病变情况和病灶面积不能作为判断预后的敏感指标.

Abstract：

Objective To observe the natural course and evaluate the prognostic factors influencing the follow-up visual acuity of polypoidal choroidal vasculopathy (PCV). Methods It was a retrospective case series. Forty-five consecutive patients (51 eyes) who were diagnosed with PCV by fundus photography,fundus fluorescein angiography (FFA) and indocyanine green angiography (ICGA) were followed up with (11.1 ± 2. 0 )months (mean ± SD ). Age, baseline visual acuity, the presence of pigment epithelium detachment (PED), lesion size, the polypoidal lesions involving the fovea and the regression of polypoidal lesions were recorded. Multi-factor regression analysis of visual acuity at follow up was applied with SPSS 16. 0 statistics software. Results Among the 45 patients (51 eyes), the hemorrhage or exudation were increased in 15 eyes (29. 4% ), decreased in 25 eyes (49. 0% ), 5 eyes (9. 8% ) developed macula scar and 6 eyes (11.8%) macula atrophy . During the follow-up period, the visual acuity was improved in 11 eyes (21.6%), stable in 21 (41.2%) and regressed in 19 (37. 2% ). Twenty-five eyes (49.0%)demonstrated polypoidal lesions involving the fovea and 26 eyes (51.0%) did not. ICGA revealed that the polypoidal lesions were unchanged in 18 eyes ( 35. 3% ), regressed in 13 ( 25.5% ), grew in 13 (25.5%), and mixed in 7 eyes ( 13.7% ). The changes of visual acuity at follow up were related to the age, baseline visual acuity, and polypoidal lesions involving in the fovea ( b = - 0. 005, 0. 382 and - 0. 430 ;P = 0. 034, 0. 000 and 0. 000). Improvement of visual acuity at follow up was related to the regression of polypoidal lesions ( b = 2. 259, P = 0. 019, OR = 9. 578). Conclusions There is a large variation in the visual prognosis in Chinese patients with PCV. The presence of PED and the lesion size had no effect on the visual prognosis of PCV. Better visual acuity during follow-up period is correlated with younger age, better baseline visual acuity, polypoidal lesions not involving the fovea and a regression of polypoidal lesions.