Association of Lesion Size and Visual Prognosis to Polypoidal Choroidal Vasculopathy

doi:10.1016/j.ajo.2011.01.002

American Journal of Ophthalmology

Volume 151, Issue 6, June 2011, Pages 961-972.e1

https://doi.org/10.1016/j.ajo.2011.01.002 Get rights and content

Purpose

To investigate the progression of vascular lesions of polypoidal choroidal vasculopathy (PCV) as viewed with indocyanine green angiography and the visual prognosis of these eyes.

Design

Retrospective case study.

Methods

We reviewed retrospectively the medical records of 88 consecutive patients (88 eyes) with PCV who had been examined with indocyanine green angiography for more than 2 years.

Results

Depending on the initial area of the vascular lesion, eyes were divided into smaller PCV (baseline area of lesion being < 1 disc area [DA], n = 22) and larger PCV (baseline area of lesion being ≥ 1 DA, n = 66). In larger PCV, the mean area of the lesion progressed significantly from 6.49 ± 8.96 mm² to 16.27 ± 14.19 mm² (P < .0001) with marked deterioration of visual acuity (P < .0001) during follow-up. In contrast, smaller PCV often showed minimal progression of the lesion, only limited exudative change, and the eyes maintained their initially good vision to the final visit. Smaller PCV lesions rarely progressed to extensive PCV lesions. Severe vision-threatening complications (ie, suprachoroidal hemorrhage, vitreous hemorrhage, and tears of the retinal pigment epithelium) were seen only in eyes with larger PCV, and in studying single nucleotide polymorphisms A69S of ARMS2 genes, there was a significant difference in T allele frequency between individuals with smaller PCV and those with larger PCV (20.2% vs 79.8%; P = .0235).

Conclusions

PCV with small vascular lesions shows minimal progression and no vision-threatening complications, and these eyes often maintain good visual acuity for a long time.

Section snippets

Methods

For this observational case study, we reviewed retrospectively the medical records of 88 consecutive patients (88 eyes) with symptomatic PCV who initially visited the Macula Service of the Department of Ophthalmology at Kyoto University Hospital between January 2004 and October 2007 and who had been examined with both fluorescein and indocyanine green angiography for more than 2 years after their initial visit. When both eyes were diagnosed as having PCV, 1 eye was selected randomly for

Results

In the current study, 88 eyes of 88 patients (60 men and 28 women) with PCV, ranging in age from 50 to 86 years (mean ± standard deviation, 70.4 ± 7.5 years), were examined. The follow-up period ranged from 29 to 61 months (mean ± standard deviation, 46.4 ± 8.6 months), and duration from the initial angiogram to the last ranged from 24 to 60 months (mean ± standard deviation, 39.3 ± 9.4 months). All patients were examined with fluorescein and indocyanine green angiography repeatedly during

Discussion

Based on the initial area of the vascular lesion, we defined smaller PCV as those with a baseline area of < 1 DA. The remaining PCV, in which the baseline area was ≥ 1 DA, were defined as larger PCV. There were no significant differences in age or duration of symptoms between these 2 groups. The larger PCV, however, did often show progression of the vascular lesions, which in many instances showed an exudative change. In larger PCV, poor initial VA was even further lessened despite the

Akitaka Tsujikawa, MD, is a graduate of the Kyoto University Graduate School of Medicine, Kyoto, Japan. He completed his ophthalmology residency at Kyoto University Hospital and a fellowship at the Kurashiki Central Hospital in Japan. Following fellowship, he worked on the retinal microcirculation at the Kyoto University Graduate School of Medicine and at the Children's Hospital in Boston. He currently specializes in macular diseases and retinal vascular diseases at the Kyoto University

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Proteolytic Degradation and Inflammation Play Critical Roles in Polypoidal Choroidal Vasculopathy
2017, American Journal of Pathology
Citation Excerpt :
There were no obvious signs of photoreceptor degeneration (Figure 4E). This observation is consistent with human studies showing that patients with larger lesions often have higher rates of lesion progression and severe complications.6–8 We frequently observed large numbers of inflammatory cells in large PCV lesions.
Polypoidal choroidal vasculopathy (PCV) is a common subtype of wet age-related macular degeneration in Asian populations, whereas choroidal neovascularization is the typical subtype in Western populations. The cause of PCV is unknown. By comparing the phenotype of a PCV mouse model expressing protease high temperature requirement factor A1 (HTRA1) in retinal pigment epithelium with transgenic mice expressing the inactive HTRA1^S328A, we showed that HTRA1-mediated degradation of elastin in choroidal vessels is critical for the development of PCV, which exhibited destructive extracellular matrix remodeling and vascular smooth muscle cell loss. Compared with weak PCV, severe PCV exhibited prominent immune complex deposition, complement activation, and infiltration of inflammatory cells, suggesting inflammation plays a key role in PCV progression. More important, we validated these findings in human PCV specimens. Intravitreal delivery of an HTRA1 inhibitor (DPMFKLboroV) was effective (36% lesion reduction; P = 0.009) in preventing PCV initiation but ineffective in treating existing lesions. Anti-inflammatory glucocorticoid was effective in preventing PCV progression but ineffective in preventing PCV initiation. These results suggest that PCV pathogenesis occurs through two stages. The initiation stage is mediated by proteolytic degradation of extracellular matrix proteins attributable to increased HTRA1 activity, whereas the progression stage is driven by inflammatory cascades. This study provides a basis for understanding the differences between PCV and choroidal neovascularization, and helps guide the design of effective therapies for PCV.
Quantitative Changes in Pigment Epithelial Detachment Area and Volume Predict Retreatment in Polypoidal Choroidal Vasculopathy
2017, American Journal of Ophthalmology
Citation Excerpt :
The independent associations between increase in PED area and volume in the presence of a dry macula and subsequent retreatment were independent of baseline PED size and GLD, reinforcing application of these results regardless of PED and PCV lesion sizes. Interestingly, Tsujikawa and associates had previously reported that larger PCVs with GLD of more than 1 disc area had a greater increase in size during anti-VEGF treatment, and were also associated with poorer VA outcomes, compared with smaller PCV complexes.26 This finding was attributed to possible higher treatment resistance in larger lesions.
To determine if changes in pigment epithelial detachment (PED) area and volume predict retreatment in polypoidal choroidal vasculopathy (PCV).
Retrospective case-control study.
PCV patients on pro re nata (PRN) anti–vascular endothelial growth factor (VEGF) therapy with >1 year follow-up at an academic retina service were included. Monthly anti-VEGF injections were given until a dry macula was achieved, and treatment deferred. Retreatment indication was recurrence of intraretinal or subretinal fluid or new hemorrhage. PED area and volume changes between visits with a dry macula (“D”) and immediate preceding visits (“D−1”) were analyzed with an automated optical coherence tomography–based software. Univariate and multivariate analyses were conducted to determine associations between changes in PED parameters and retreatment need at immediate subsequent visits (“D+1”).
Twenty-two PCV patients (mean age 69.6 years) were included. Of 46 visits D, 11 (23.9%) were followed by retreatment at D+1. An increase in PED area (>0.43 mm²) and volume (>0.0245 mm³) from D−1 to D was associated with 18.2 (95% CI, 3.7–125.6; P < .001) and 101.9 (95% CI, 9.5–14 308.0; P < .001) higher retreatment odds at D+1, respectively. These associations remained significant after multivariate analyses adjusting for baseline PED area or volume, greatest linear dimension, and type of anti-VEGF agent.
In PCV on PRN anti-VEGF therapy, increases in PED area and volume at one visit, despite achievement of a dry macula, are associated with retreatment at the next visit. Retreatment criteria relying on intraretinal or subretinal fluid or new hemorrhages may be expanded to include PED changes. Studies are needed to determine if using PED parameters in treatment decisions reduces recurrences.
Age-related macular degeneration and polypoidal choroidal vasculopathy in Asians
2016, Progress in Retinal and Eye Research
Age-related macular degeneration (AMD) is the leading cause of irreversible blindness in elderly people globally. It is estimated that there will be more Asians with AMD than the rest of the world combined by 2050. In Asian populations, polypoidal choroidal vasculopathy (PCV) is a common subtype of exudative AMD, while choroidal neovascularization secondary to AMD (CNV-AMD) is the typical subtype in Western populations. The two subtypes share many common clinical features and risk factors, but also have different epidemiological and clinical characteristics, natural history and treatment outcomes that point to distinct pathophysiological processes. Recent research in the fields of genetics, proteomics and imaging has provided further clarification of differences between PCV and CNV-AMD. Importantly, these differences have manifested as disparity in response to intravitreal injections of anti-vascular endothelial growth factor (anti-VEGF) treatment between PCV and CNV-AMD, emphasizing the need for accurate diagnosis of PCV and in distinguishing PCV from CNV-AMD, particularly in Asian patients. Current clinical trials of intravitreal anti-VEGF therapy and photodynamic therapy will provide clearer perspectives of evidence-based management of PCV and may lead to paradigm shifts in therapeutic strategies away from those currently employed in the treatment of CNV-AMD. Further research is needed to clarify the relative contribution of specific pathways in inflammation, complement activation, extracellular matrix dysregulation, lipid metabolism and angiogenesis to the pathogenesis of PCV. Findings from this research, together with improved diagnostic technology and new therapeutics, will facilitate more optimal management of Asian AMD.
Etiological Factors and Visual Outcomes of Dense Vitreous Hemorrhage in Patients Aged 80 years and above over the Past Decade in a Tertiary General Hospital
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Relationship between Pachychoroid and Polypoidal Choroidal Vasculopathy
2022, Journal of Clinical Medicine
Recurrence and visual prognostic factors of polypoidal choroidal vasculopathy: 5-year results
2021, Scientific Reports

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Original articleAssociation of Lesion Size and Visual Prognosis to Polypoidal Choroidal Vasculopathy

Purpose

Design

Methods

Results

Conclusions

Section snippets

Methods

Results

Discussion

Am J Ophthalmol

Ophthalmology

Am J Ophthalmol

Surv Ophthalmol

Am J Ophthalmol

Jpn J Ophthalmol

Ophthalmology

Ophthalmology

Ophthalmology

The posterior uveal bleeding syndrome

Retina

Idiopathic polypoidal choroidal vasculopathy (IPCV)

Retina

Indocyanine green videoangiography of idiopathic polypoidal choroidal vasculopathy

Retina

Idiopathic polypoidal choroidal vasculopathy: a peripheral lesion

Arch Ophthalmol

Idiopathic polypoidal choroidal vasculopathy in Japanese patients

Arch Ophthalmol

Peripheral idiopathic polypoidal choroidal vasculopathy

Eye

The expanding clinical spectrum of idiopathic polypoidal choroidal vasculopathy

Arch Ophthalmol

Polypoidal choroidal vasculopathy: incidence, demographic features, and clinical characteristics

Arch Ophthalmol

Polypoidal choroidal vasculopathy in exudative and haemorrhagic pigment epithelial detachments

Br J Ophthalmol

Pigment epithelial detachment in polypoidal choroidal vasculopathy

Am J Ophthalmol

Angiographic lesion of polypoidal choroidal vasculopathy on indocyanine green and fluorescein angiography

Graefes Arch Clin Exp Ophthalmol

Polypoidal choroidal vasculopathy with large vascular network

Graefes Arch Clin Exp Ophthalmol

Clinical features of early and late stage polypoidal choroidal vasculopathy characterized by lesion size and disease duration

Graefes Arch Clin Exp Ophthalmol

Predictable signs of benign course of polypoidal choroidal vasculopathy: based upon the long-term observation of non-treated eyes

Acta Ophthalmol

Visual improvement following trans-Tenon's retrobulbar triamcinolone acetonide infusion for polypoidal choroidal vasculopathy

Graefes Arch Clin Exp Ophthalmol

Original article
Association of Lesion Size and Visual Prognosis to Polypoidal Choroidal Vasculopathy