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Polypoidal Choroidal Vasculopathy
Incidence, Demographic Features, and Clinical Characteristics
Kenichiro Sho, MD;
Kanji Takahashi, MD;
Haruhiko Yamada, MD;
Mitsumasa Wada, MD;
Yoshimi Nagai, MD;
Tsuyoshi Otsuji, MD;
Maki Nishikawa, MD;
Yumiko Mitsuma, MD;
Yukari Yamazaki, MD;
Miyo Matsumura, MD;
Masanobu Uyama, MD
Arch Ophthalmol. 2003;121:1392-1396.
ABSTRACT
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Objective To clarify the incidence, demographic features, and clinical characteristics of polypoidal choroidal vasculopathy (PCV) in Japanese patients.
Methods Consecutive patients with presumed neovascular age-related macular degeneration (AMD) who met the eligibility criteria were examined between January 1, 1999, and October 31, 2001. All patients underwent complete ophthalmologic examination and fluorescein and indocyanine green angiography.
Results Among 471 eyes of 418 patients who met the criteria, 110 eyes (23%) of 100 patients were diagnosed as having PCV and 361 eyes (77%) of 318 patients as having neovascular AMD. Mean age of patients with PCV was 68.4 years, with a male preponderance (63% of patients); involvement was mostly unilateral (90% of patients), and polypoidal vascular lesions were located mainly in the macula (85% of eyes). Retinal manifestations of PCV were characterized by serous macular detachment (52% of eyes), submacular hemorrhage (30% of eyes), and retinal pigment epithelium degeneration (10% of eyes). There were few subretinal fibrovascular proliferations (7% of eyes). Mean visual acuity was 0.31 in eyes with PCV and 0.18 in eyes with AMD. The incidence of severe visual loss (0.2 or worse) was 35% in PCV and 53% in AMD.
Conclusions The incidence of PCV in Japanese patients is high, and the incidence and demographic features vary in different ethnic groups. The clinical manifestations of PCV and AMD resemble each other; however, PCV is characterized by low incidence of subretinal fibrovascular proliferation, slow progression of vascular abnormality, and minimal association with conventional choroidal neovascularization. These factors seem to lead to a more favorable visual outcome in PCV compared with neovascular AMD.
INTRODUCTION
POLYPOIDAL CHOROIDAL vasculopathy (PCV)1-10 is a distinct clinical entity characterized by persistent, recurrent serous leakage and hemorrhage in the macula in the elderly population, caused by a peculiar form of choroidal neovascularization (CNV) in the inner choroid. Indocyanine green (ICG) angiography clearly demonstrates polypoidal and aneurysmal dilations of the terminals of the branching vascular network from the choroidal circulation in PCV.2, 10-12
The retinal manifestations of PCV resemble neovascular age-related macular degeneration (AMD)3; however, the clinical course and visual outcome of PCV are more stable, more favorable, and different from those of AMD.1-4,8-10,12-14 Neovascular AMD usually becomes progressively worse. To establish the optimal management for PCV, it is important to clarify its clinical characteristics.
The histopathologic characteristics of this disease were recently elucidated,15-21 and most reports describe a collection of dilated thin-walled vessels derived from the choroidal vessels beneath the retinal pigment epithelium (RPE) in Bruch membrane, although the exact pathogenesis remains unknown.
This disorder was initially described as recurrent subretinal and vitreous hemorrhage in middle-aged to elderly black women.1, 5-8 Further expansion of the clinical entity, however, indicates that the disease is prevalent in all races and both sexes.2-4,10, 12-14,22-25 There are differences, however, in the incidence and demographic features of PCV in different ethnic groups.3, 12-14 Clinical experience suggests that the incidence of PCV is remarkably high in blacks, considerably high in Asians, and low in whites.2-8,12-14,22-26 In contrast, the incidence of AMD is high in whites, moderate in Asians, and very low in blacks.24-26
There is a preponderance of men, unilateral involvement, and macular location of abnormal vessels in PCV in Japanese patients.12, 24 Kwok et al23 reported similar findings in Chinese patients. Yannuzzi et al,2-3 however, described a preponderance of women, bilateral involvement, and peripapillary location of PCV in white patients. Yannuzzi et al2 emphasized there was little subretinal fibrous proliferation and disciform scarring in PCV, probably because of the low level of subretinal fibrovascular proliferation and hyperplasia of the RPE in PCV.
The unusual manifestations of PCV highlight the significance of this new clinical entity. Although there are many recent reports of PCV,1-25 most of the studies were performed in only a small series. Thus, the exact characteristics remain unclear. We performed this study in a large number of patients to determine the incidence and demographic features of PCV in Japanese patients and to clarify the clinical characteristics of PCV compared with those of neovascular AMD.
METHODS
We prospectively examined consecutive first-visit patients with presumed neovascular AMD in our clinic who met the criteria during a 34-month period between January 1, 1999, and October 31, 2001.
All patients underwent a complete ophthalmologic examination, binocular ophthalmoscopy, slit-lamp biomicroscopy with a contact lens, color fundus photography, fluorescein angiography, and ICG angiography. The ICG angiography was performed by means of scanning laser ophthalmoscopy12 (Rodenstock Co, Ottobrumn-Riemerling, Germany). One of us (K.T.) examined all patients.
Inclusion criteria for the study were as follows: (1) The fundus had exudative maculopathy with serous and/or hemorrhagic detachment of the sensory retina and/or RPE in the posterior pole. (2) The ICG angiography showed a branching vascular network from the choroidal circulation and polypoidal and aneurysmal dilatations at the terminals of the vascular network. The medical history was reviewed for systemic hypertension and diabetes mellitus.
Exclusion criteria were as follows: (1) inactive, presumed late neovascular AMD without any leakage or hemorrhage, (2) other neovascular maculopathies such as high myopia, angioid streaks, and presumed ocular histoplasmosis syndrome, and (3) central serous chorioretinopathy.
Diagnosis of either neovascular AMD or PCV was made by clinical manifestations and ICG angiography. Patients in whom differential diagnosis was unsuccessful were excluded from the study.
RESULTS
Four hundred seventy-one eyes of 418 patients were eligible for the study. All patients were Japanese. Among them, 110 eyes (23%) of 100 patients were diagnosed as having PCV, and 361 eyes (77%) of 318 patients were diagnosed as having neovascular AMD. Patients with PCV ranged in age from 50 to 93 years, with a mean of 68.4 years. Of the 100 patients with PCV, 63 were male, 37 were female, 90 were unilaterally affected, and 10 had bilateral involvement.
Polypoidal vascular abnormalities were located in the macula in 93 eyes (85%), in peripapillary area in 8 eyes (7%), and in both macula and peripapillary areas in 3 eyes (3%). A cluster of grapelike polypoidal vascular dilations24 were observed in 10 eyes (9%), and there was a clear association with conventional CNV (Figure 1, Figure 2, and Figure 3) in 10 eyes (9%), all with classic CNV.
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Figure 1. Fundus photograph of a 68-year-old man with a central scotoma and visual loss of the left eye for 2 months, showing subfoveal fibrovascular proliferation (small arrow) and several polypoidal vascular configurations beneath the retinal pigment epithelium (large arrows). Visual acuity was 0.2 (20/100).
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Figure 2. Fluorescein angiogram (early phase) of the same fundus as in Figure 1, showing polypoidal choroidal neovascularization (large arrows) associated with classic choroidal neovascularization (small arrow).
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Figure 3. Indocyanine green angiogram (early phase) of the same fundus as in Figure 1 and Figure 2, showing polypoidal choroidal neovascularization (black arrows) associated with classic choroidal neovascularization (white arrow).
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Retinal manifestations at the first visit in 110 eyes with PCV were characterized by serous macular detachment (52%), submacular hemorrhage (30%), and RPE degeneration (10%). There were few subretinal fibrovascular proliferations (7%) (Table 1). Best-corrected visual acuities (VAs) of the eyes with PCV and AMD at the first visit are shown in Figure 4. In the eyes with PCV, VA was almost evenly distributed across 3 gradients (Figure 4A), and mean VA was 0.31 (20/60) (decimal VA was calculated in logMAR). In the eyes with neovascular AMD, the eyes with severe visual loss (0.2 or worse) had the greatest distribution (53%), and those with mild visual loss (0.8 or better) had the lowest distribution (14%) (Figure 4B). Mean VA was 0.18 (20/110) (Mann-Whitney U test, P<.001).
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Table 1. Predominant Findings in the Macula in Polypoidal Choroidal Vasculopathy at First Visit*
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Figure 4. Distribution of visual acuity at the first visit in polypoidal choroidal vasculopathy (110 eyes) (A) and neovascular age-related macular degeneration (361 eyes) (B). In polypoidal choroidal vasculopathy (A), visual acuity was evenly distributed across 3 gradients. In neovascular age-related macular degeneration (B), those with severe visual loss (0.2 or worse) had the highest distribution (53%) and those with mild visual loss (0.8 or better) had the lowest (14%).
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Mean duration of the current macular disorder from the onset of symptoms to the time of the first examination was 21.9 months in PCV and 14.6 months in AMD (Mann-Whitney U test, P = .18). Table 2 gives the macular manifestations of the 38 eyes with severe visual loss in PCV. In the patients with PCV, 36 patients had systemic hypertension and 11 had diabetes mellitus.
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Table 2. Macular Manifestations in the Eyes With Severe Visual Loss (0.2 or Worse) in Polypoidal Choroidal Vasculopathy*
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COMMENT
The present study confirms that the incidence of PCV in Japanese patients is remarkably high, and that the incidence and demographic features of PCV vary in different ethnic groups, based on a large number of observations (Table 3). Polypoidal choroidal vasculopathy was diagnosed in 23% of 471 patients with presumed AMD. Many studies2-3,10, 12-13 report that PCV is more prevalent in blacks, Japanese, and other Asians than in whites. On the other hand, the incidence of AMD is very high in whites, but remarkably low in blacks.26-28 The incidence of both diseases is high in Asians.
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Table 3. Incidence and Demographic Features in Polypoidal Choroidal Vasculopathy (PCV)*
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The demographic features of PCV in the present study were consistent with previous reports from our group12, 24; however, they were different from those reported for whites.2, 13-14 The mean age of the patients was 68 years, and there was male preponderance (63%), unilateral involvement (90%), and macular location (85%) (Table 3). In whites,1-3,10, 13-14,22 there is a female preponderance, bilateral involvement, and peripapillary location. The reason for these epidemiologic differences in sex, unilaterality, and location in the different ethnic groups is not known.
In the present study, as well as in previous reports,12, 24 there was a very high percentage of unilateral involvement of the eye in PCV in Japanese patients. The examination was performed in both eyes at the initial visit at our clinic. A similar finding was reported in Chinese patients.23 The same finding was observed for AMD in Japanese patients. These findings may be accounted for by environmental and/or hereditary factors.
Retinal manifestations of PCV resemble those of neovascular AMD (Table 1). Serous detachment of the retina and RPE were most common (52%), followed by retinal hemorrhage (30%). There were few subretinal fibrovascular proliferations (7%) in PCV compared with AMD.2, 12, 24 Subretinal fibrous proliferation in the macula remarkably damaged the sensory retina and RPE, leading to severe visual loss. Disciform scarring is also uncommon in PCV.2 In the present study, however, inactive lesions without active exudation were excluded from examination; thus, disciform scars could not be evaluated.
Characteristically, PCV was rarely associated with conventional CNV (10 eyes [9%]) on fluorescein and ICG angiography (Figure 1, Figure 2, and Figure 3), suggesting that the pathogenesis of polypoidal CNV differs from CNV in AMD.
As mentioned herein, PCV was associated with classic CNV but not with occult CNV. On ICG angiography, it is difficult to differentiate clearly between occult CNV and polypoidal CNV, without polypoidal configuration of the terminals of the vascular networks. In PCV, polypoidal dilations of terminal vessels show a specific appearance on ICG angiography and an intense hyperfluorescence with either the Topcon Corp (Tokyo, Japan) or Rodenstock instrument. Thus, ICG angiography is very valuable for making the correct diagnosis of PCV.
Evaluation of the duration from onset of symptoms of the disorder to the first examination in both PCV and AMD demonstrated a difference in the progression of the 2 diseases. The PCV progressed more slowly (21.2 months) than did AMD (14.8 months).
The mean VA at the first visit in patients with PCV (0.31) was significantly better than in neovascular AMD (0.18). Figure 4 shows the distribution of VA in both diseases. Severe visual loss (0.2 or worse) was common in AMD (53%), but less common in PCV (35%). We previously reported that half of the patients with PCV had an unfavorable outcome. The present study confirmed deterioration to severe visual loss in 35% of the eyes.
In PCV, reactive fibrous proliferation is poor, progression of the disorder is slow, and there is little association with conventional CNV. Thus, visual outcome is markedly more favorable than in neovascular AMD.26
Macular manifestations of the eyes with severe visual loss (Table 2) demonstrated the main causes of visual loss in PCV. First, persistent serous retinal detachment of the macula leads to degeneration and atrophy of the RPE and sensory retina in the macula, which results in severe visual loss (21%) (Figure 5 and Figure 6). Second, subretinal fibrovascular proliferation markedly damages macular function. All 8 eyes in which subretinal fibrovascular proliferation developed in the macula suffered severe visual loss. It is beneficial, therefore, that in PCV there were relatively few subretinal proliferations.2 Third, massive submacular hemorrhage persists and damages the RPE and photoreceptors, then leads to degeneration of RPE, which results in severe visual loss.
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Figure 5. Fundus photograph of a 72-year-old patient with a 30-year history of chronic central serous chorioretinopathy, showing marked degeneration and atrophy of the retinal pigment epithelium at the macula. Visual acuity was 0.2 (20/100). Persistent serous macular detachment caused by polypoidal choroidal vasculopathy resulted in degeneration and atrophy of retinal pigment epithelium.
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Figure 6. Indocyanine green angiogram (early phase) of the same fundus as in Figure 5, showing polypoidal dilations of the terminals (black arrows) of the branching vascular network (V). Polypoidal dilations are observed at the upper margin of the macula. In this patient, polypoidal choroidal vasculopathy mimics chronic central serous chorioretinopathy. P indicates papilla.
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A relationship of systemic hypertension and diabetes mellitus to the pathogenesis of PCV was not found in this study.2, 14
This study was a cross-sectional survey at the first examination of the patients at our clinic. The study was so large that it was separated into 2 parts; a follow-up study, including the clinical course and the results of laser photocoagulation treatment of this series, will be reported in a later article.
In conclusion, the present study confirms that the incidence of PCV in Japanese patients is high (23% in exudative maculopathy) and that the incidence and demographic features of PCV vary in different ethnic groups. Eyes with severe visual loss suggest principal clinical findings in which VA usually deteriorated to severe visual loss: subretinal fibrous proliferation, RPE degeneration and atrophy, and submacular hemorrhage. The clinical manifestations of PCV and AMD are similar; however, PCV is characterized by low incidence of subretinal fibrovascular proliferation, slow progression of vascular abnormality, and minimal association with conventional CNV. These factors seem to lead to a more favorable visual outcome in PCV compared with neovascular AMD. Knowledge of the incidence, demographic features, and clinical characteristics of PCV is important for correct diagnosis and precise management of the disease and a better understanding of its pathogenesis.
AUTHOR INFORMATION
Corresponding author and reprints: Masanobu Uyama, MD, 13-8, Ohkamedani-Naizencho, Fushimi, Kyoto 612-0047, Japan (e-mail: uyamam{at}ceres.ocn.ne.jp).
Submitted for publication November 27, 2002; final revision received March 21, 2003; accepted May 1, 2003.
From the Department of Ophthalmology, Kansai Medical University, Fumizono-cho, Moriguchi, Osaka, Japan. The authors have no relevant financial interest in this article.
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