Polypoidal choroidal vasculopathy: natural history

doi:10.1016/S0002-9394(02)01404-6

American Journal of Ophthalmology

Volume 133, Issue 5, May 2002, Pages 639-648

https://doi.org/10.1016/S0002-9394(02)01404-6 Get rights and content

Abstract

PURPOSE: The present study was performed to clarify the long-term natural history of polypoidal choroidal vasculopathy (PCV).

DESIGN: Prospective, consecutive observational case series.

METHODS: Fourteen eyes of 12 consecutive patients with PCV were prospectively followed in our clinic for at least 2 years without any treatment after a first visit to the clinic between February 1996 and November 1998. All patients underwent complete ophthalmologic examination, color fundus photography, and fluorescein and indocyanine green (ICG) angiography at regular intervals. Inclusion criteria were as follows: eyes had serous and/or hemorrhagic pigment epithelium detachment (PED) and retinal detachment in the posterior pole, and ICG angiography revealed a branching vascular network with polypoidal dilations at the terminals of the network. Exclusion criteria were as follows: other diseases such as exudative age-related macular degeneration, high myopia, angioid streaks, and presumed ocular histoplasmosis syndrome, and patients who previously underwent any ocular surgery.

RESULTS: Patients were followed for mean of 39.9 months (range, 24–54 months). PCV was present in 10 (83%) men and two women and in the elderly (mean age 68.1 years), usually unilateral (83%) with vascular lesions located at the macula (93%). The PCV manifested in two patterns, exudative and hemorrhagic. In the exudative pattern, serous PED and retinal detachment were predominant at the macula. The hemorrhagic pattern was characterized by hemorrhagic PED and subretinal hemorrhage at the macula. ICG angiography revealed polypoidal choroidal neovascularization that was changeable in appearance and repeatedly grew and spontaneously regressed, but the vascular network persisted. In some eyes, a collection of small aneurysmal dilations of vessels resembling a cluster of grapes appeared and all of them had marked bleeding and leakage and worse outcome.

CONCLUSION: Polypoidal choroidal vasculopathy is a long persistent chronic disease and the patients had a variable course. Fifty percent of the patients had a favorable course. In the remaining half of the patients, the disorder persisted for a long time with occasional repeated bleeding and leakage, resulting in macular degeneration and visual loss. Eyes with a cluster of grapes-like polypoidal dilatations of the vessels had a high risk for severe visual loss.

Section snippets

Methods

We prospectively followed consecutive patients with PCV that did not undergo any treatment for at least 2 years in our clinic. Inclusion criteria for this study were as follows: (1) Patients first visited our clinic between February 1996 and November 1998. (2) The fundus had exudative maculopathy with hemorrhagic and/or serous detachment of the RPE and neurosensory retina in the posterior pole. (3) ICG angiography revealed a branching vascular network from the choroidal circulation, and

Case 1

Patient 3, a 76-year-old man, had noticed visual loss in both eyes for 6 months. Ophthalmoscopy revealed shallow serous detachment of the retina and diffuse degeneration of the RPE with lipid deposits in the macula (exudative pattern) in both eyes (Figure 1). Visual acuity was 20/60 in the right eye and 20/40 in the left eye. ICG angiography revealed a branching vascular network and polypoidal dilations superonasal to the fovea in the left eye (Figure 2). In the right eye, ICG angiography

Results

Fourteen eyes of 12 patients met the criteria for the study and were diagnosed with PCV (Table 1). All patients were Japanese; ten (83%) were men and two were women. Their ages ranged from 55 to 79 years (mean 68.1 years). The affected eyes were unilateral in ten patients (83%) and bilateral in two patients for a total of 14 eyes. The polypoidal CNVs were located at the macula in 13 eyes (93%), and peripapillary in one eye. The follow-up period ranged from 24 to 54 months (mean 39.9 months).

Discussion

We prospectively followed 14 eyes of 12 consecutively enrolled Japanese patients with PCV for more than 2 years for a mean duration of 39.9 months. Demographic features of these patients were consistent with those in a previous paper12 and also with other reports on Japanese patients.22, 23, 24 The patients were predominantly elderly, male, and suffered from unilateral macular lesions. The characteristic features were somewhat different in white people. Affected white patients were usually

References (31)

L.A. Yannuzzi et al.
Polypoidal choroidal vasculopathy masquerading as central serous chorioretinopathy
Ophthalmology
(2000)
R.M. Stern et al.
Multiple recurrent sero-sanguineous retinal pigment epithelial detachments in black women
Am J Ophthalmol
(1985)
R.S. Moorthy et al.
Idiopathic polypoidal choroidal vasculopathy of the macula
Ophthalmology
(1998)
F. Shiraga et al.
Surgical treatment of submacular hemorrhage associated with idiopathic polypoidal choroidal vasculopathy
Am J Ophthalmol
(1999)
H. Iijima et al.
Optical coherence tomography of idiopathic polypoidal choroidal vasculopathy
Am J Ophthalmol
(1999)
F. El Baba et al.
Massive hemorrhage complicating age-related macular degeneration, clinicopathologic correlation, and role of anticoagulants
Ophthalmology
(1986)
H. Iijima et al.
Optical coherence tomography of orange–red subretinal lesions in eyes with idiopathic polypoidal choroidal vasculopathy
Am J Ophthalmol
(2000)
W.R. Green et al.
Age-related macular degeneration, histopathologic studies
1992 L E Zimmerman lecture Ophthalmology
(1993)
L.A. Yannuzzi et al.
Idiopathic polypoidal choroidal vasculopathy
Retina
(1990)
R.F. Spaide et al.
Indocyanine green videoangiography of idiopathic choroidal vasculopathy
Retina
(1995)

L.A. Yannuzzi et al.

The expanding clinical spectrum of idiopathic polypoidal choroidal vasculopathy

Arch Ophthalmol

(1997)

L.A. Yannuzzi et al.

Polypoidal choroidal vasculopathy and neovascularized age-related macular degeneration

Arch Ophthalmol

(1999)

B.T. Perkovich et al.

An update of multiple recurrent serosanguineous detachments in black women

Retina

(1990)

R.C. Kleiner et al.

The posterior uveal bleeding syndrome

Retina

(1990)

L.A. Yannuzzi et al.

Idiopathic polypoidal choroidal vasculopathya peripheral lesion

Arch Ophthalmol

(1998)

Cited by (381)

Chinese Guideline on the Management of Polypoidal Choroidal Vasculopathy (2022)
2023, Chinese Medical Sciences Journal
In mainland China, patients with neovascular age-related macular degeneration (nAMD) have approximately an 40% prevalence of polypoidal choroidal vasculopathy (PCV). This disease leads to recurrent retinal pigment epithelium detachment (PED), extensive subretinal or vitreous hemorrhages, and severe vision loss. China has introduced various treatment modalities in the past years and gained comprehensive experience in treating PCV.
A total of 14 retinal specialists nationwide with expertise in PCV were empaneled to prioritize six questions and address their corresponding outcomes, regarding opinions on inactive PCV, choices of anti-vascular endothelial growth factor (anti-VEGF) monotherapy, photodynamic therapy (PDT) monotherapy or combined therapy, patients with persistent subretinal fluid (SRF) or intraretinal fluid (IRF) after loading dose anti-VEGF, and patients with massive subretinal hemorrhage. An evidence synthesis team conducted systematic reviews, which informed the recommendations that address these questions. This guideline used the GRADE (Grading of Recommendations, Assessment, Development, and Evaluation) approach to assess the certainty of evidence and grade the strengths of recommendations.
The panel proposed the following six conditional recommendations regarding treatment choices. (1) For patients with inactive PCV, we suggest observation over treatment. (2) For treatment-naive PCV patients, we suggest either anti-VEGF monotherapy or combined anti-VEGF and PDT rather than PDT monotherapy. (3) For patients with PCV who plan to initiate combined anti-VEGF and PDT treatment, we suggest later/rescue PDT over initiate PDT. (4) For PCV patients who plan to initiate anti-VEGF monotherapy, we suggest the treat and extend (T&E) regimen rather than the pro re nata (PRN) regimen following three monthly loading doses. (5) For patients with persistent SRF or IRF on optical coherence tomography (OCT) after three monthly anti-VEGF treatments, we suggest proceeding with anti-VEGF treatment rather than observation. (6) For PCV patients with massive subretinal hemorrhage (equal to or more than four optic disc areas) involving the central macula, we suggest surgery (vitrectomy in combination with tissue-plasminogen activator (tPA) intraocular injection and gas tamponade) rather than anti-VEGF monotherapy.
Six evidence-based recommendations support optimal care for PCV patients’ management.
Progression of Polypoidal Lesions Associated with Exudative Recurrence in Polypoidal Choroidal Vasculopathy
2023, Ophthalmology
To investigate the characteristics of the branching vascular network (BVN) and polypoidal lesions in polypoidal choroidal vasculopathy (PCV) to determine near-term indicators that may predict exudative recurrence.
Retrospective cohort study.
Patients with PCV receiving anti–vascular endothelial growth factor (VEGF) monotherapy or anti-VEGF plus photodynamic therapy were followed for at least 1 year using swept-source OCT angiography (SS-OCTA) imaging.
Patients were divided into 2 groups based on whether exudative recurrence occurred during follow-up. Multiple parameters were collected and compared between the 2 groups, such as age, gender, visual acuity, number of polypoidal lesions, lesion area at the first SS-OCTA visit, and total lesion area change from the first SS-OCTA visit to the last SS-OCTA visit. To evaluate the association between SS-OCTA imaging-based risk factors and the exudative recurrences, imaging features associated with PCV such as BVN growth and polypoidal lesion progression (enlargement, new appearance, and reappearance) at each follow-up visit were analyzed. The time intervals from the nonexudative visit with lesion progression to the corresponding exudative recurrence visit were documented to explore their association with exudative recurrences. Cox regression and logistic regression analyses were used.
Association between BVN growth and polypoidal lesion progression with exudative recurrence.
Thirty-one eyes of 31 patients (61% men) were included. Sixteen eyes had no recurrence of exudation, and 15 eyes had recurrence during follow-up. The average follow-up duration was 20.55 ± 6.86 months (range, 12–36 months). Overall, the recurrence group had worse best-corrected visual acuity (P = 0.019) and a greater increase in lesion area (P = 0.010). Logistical regression analysis showed that polypoidal lesion progression, including new appearance, enlargement, and reappearance of polypoidal lesions, was associated with exudative recurrences within 3 months (odds ratio, 26.67, 95% confidence interval, 3.77–188.54, P = 0.001).
Growth of nonexudative BVN and progression of polypoidal lesions were found to be lesion characteristics associated with exudative recurrences, and progression of polypoidal lesions might serve as a stand-alone indicator for the near-term onset of exudation. In PCV, more frequent follow-up visits are recommended when polypoidal lesions show progression.
Evolution of Polypoidal Lesions after Treatment of Polypoidal Choroidal Vasculopathy
2022, Ophthalmology Science
To evaluate the status and evolution of polypoidal lesions during the course of treatment of patients with symptomatic macular polypoidal choroidal vasculopathy (PCV).
Comparative cohort study of randomly selected patients from a multicenter, randomized controlled clinical trial.
Thirty randomly selected patients from the EVEREST II study who were treated with combination ranibizumab and verteporfin photodynamic therapy (n = 15) or ranibizumab monotherapy (n = 15).
All patients were randomized at baseline and treated with a standardized treatment protocol. Indocyanine green angiography (ICGA) images were graded at the central reading center at baseline and months 3, 6, 12, and 24. Polypoidal lesions present at baseline were overlaid on ICGA images at subsequent visits to determine if these remained perfused or had regressed completely. New polypoidal lesions occurring at subsequent visits were similarly tracked to detail the evolution of each polypoidal lesion.
Complete polypoidal lesion regression over time.
Complete polypoidal lesion regression was higher in the combination therapy group compared with the monotherapy group at all visits (month 12, 12 of 15 patients [80%] vs. 5 of 14 patients [35.7%]; P = 0.016). Persistence of baseline polypoidal lesions was lower in the combination therapy group: 1 of 15 patients (6.7%) versus 7 of 14 patients (50%) in the monotherapy group at month 12. Recurrences of polypoidal lesions that had regressed completely at an earlier time point were uncommon: 0% in the combination therapy group and 1 patient each at months 6 and 12 in the monotherapy group. Fewer new polypoidal lesions (arising after the baseline visit) were found in the combination therapy group at all visits (combination therapy: 2 of 15 [13.3%] vs. monotherapy: 4 of 14 eyes [28.6%] at month 12). Total polypoidal lesion area was significantly smaller in the combination therapy group compared with the monotherapy group throughout the study (0.013 mm² vs. 0.110 mm²; P < 0.01 at month 12).
Combination therapy was associated with higher rates of complete polypoidal lesion regression and fewer persistent polypoidal lesions compared with monotherapy. Closed polypoidal lesions rarely reopened, regardless of the treatment.
Effect of intravitreal injection of bevacizumab in treated and untreated eyes of Black African patients with polypoidal choroidal vasculopathy
2021, Journal Francais d'Ophtalmologie
To evaluate the response of polypoidal choroidal vasculopathy (PCV) in eyes treated with intravitreal bevacizumab (BVZ) and untreated fellow eyes in black Africans.
We studied 22 eyes (12 patients) divided into 12 treated and 10 untreated eyes from January 2017 to January 2020. Treated eyes received 1 monthly injection of BVZ 2.5 mg for 3 months, with optional additional injections depending on the patient's course. Both groups of eyes were evaluated at presentation and then at 3, 6, 9, and 12 months after treatment. Outcome measures were visual acuity (VA) and ophthalmoscopic and OCT findings.
The mean age of the patients was 66.3 ± 5.6 years. In treated eyes, VA remained stable from 0.10 ± 0.12 at baseline to 0.20 ± 0.30 at month 12, P = 0.84. VA was stable in 83.3% and improved in 16.7% of eyes. On OCT, 41.7% of eyes showed decreased and another 41.7% disappearance of subretinal fluid (SRF) at 12 months. Pigment epithelial detachment (PED) height decreased in 9 eyes (75.0%) but remained unchanged in 3 eyes (25%). In untreated eyes, no difference was observed between the baseline (0.53 ± 0.42) and 12-month VA (0.58 ± 0.40), P = 0.82. VA improved in 2 eyes, decreased in one eye, and remained unchanged in 7 eyes. OCT lesions remained stable in 6 eyes. The PED enlarged in one eye but remained stable in 3 other eyes.
Intravitreal injection of BVZ 2.5 mg led to stabilization of VA, resorption of SRF, and reduction in the size of the PED in the majority of eyes with PCV but was ineffective on the polyps. The one-year prognosis in untreated eyes with PCV was favorable and marked by functional and structural stability.
Évaluer la réponse de la vasculopathie polypoïdale choroïdienne (VPC) dans les yeux traités par le bevacizumab (BVZ) intravitréen et les yeux contralatéraux non traités.
Nous avons étudié 22 yeux (12 patients), dont 12 traités et 10 non traités de janvier 2017 à janvier 2020. Le traitement consistait en une injection intravitréenne mensuelle de 2,5 mg de BVZ pendant 3 mois, avec possibilité d’injections additionnelles selon l’évolution. Les deux groupes d’yeux avaient été évalués avant et à 3, 6, 9 et 12 mois après traitement. Les éléments d’évaluation étaient l’acuité visuelle (AV) et les signes observés en ophtalmoscopie et en tomographie en cohérence optique (TCO).
L’âge moyen des patients était de 66,3 ± 5,6 ans. À 12 mois de suivi, l’AV des yeux traités (0,20 ± 0,30) était demeurée stable en comparison avec l’AV initiale (0.10 ± 0,12), P = 0,84. L’AV était stable dans 83,3 % et améliorée dans 16,7 % d’yeux. En TCO, une proportion similaire d’yeux (41,7 %) avait une réduction et une disparition de l’exsudation sous-rétinienne (ESR). La taille du décollement de l’épithélium pigmentaire de la rétine (DEPR) était réduite dans 9 yeux (75,0 %), mais était restée inchangée dans le reste d’yeux. Il n’y avait pas de différence significative (P = 0,82) entre l’AV initiale (0,53 ± 0,42) et celle à 12 mois (0,58 ± 0,40) dans le group d’yeux non traités. L’AV était améliorée dans 2 yeux, détériorée dans un oeil et stable dans 7 yeux. Les lésions vues en TCO étaient stables dans 6 yeux. Le DEPR s’était aggravé dans un œil, mais était resté sable dans 3 autres.
L’injection intravitréenne de 2,5 mg de BVZ a permis la stabilisation de l’AV, la résorption de l’ESR et la réduction de la taille du DEPR dans la plupart d’yeux avec VPC, mais était inefficace sur les polypes. Le prognostic des yeux non traités était favorable et marqué par la stabilité fonctionnelle et structurelle.
Evaluating photodynamic therapy versus brolucizumab as a second-line treatment for polypoidal choroidal vasculopathy
2024, International Journal of Retina and Vitreous
Macular neovascularization and polypoidal choroidal vasculopathy: phenotypic variations, pathogenic mechanisms and implications in management
2024, Eye (Basingstoke)

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¹: InternetAdvance publication at ajo.com April 15, 2002.

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Polypoidal choroidal vasculopathy: natural history1

Abstract

Section snippets

Methods

Case 1

Results

Discussion

Ophthalmology

Am J Ophthalmol

Ophthalmology

Am J Ophthalmol

Am J Ophthalmol

Ophthalmology

Am J Ophthalmol

1992 L E Zimmerman lecture Ophthalmology

Idiopathic polypoidal choroidal vasculopathy

Retina

Indocyanine green videoangiography of idiopathic choroidal vasculopathy

Retina

The expanding clinical spectrum of idiopathic polypoidal choroidal vasculopathy

Arch Ophthalmol

Polypoidal choroidal vasculopathy and neovascularized age-related macular degeneration

Arch Ophthalmol

An update of multiple recurrent serosanguineous detachments in black women

Retina

The posterior uveal bleeding syndrome

Retina

Idiopathic polypoidal choroidal vasculopathya peripheral lesion

Arch Ophthalmol

Polypoidal choroidal vasculopathy: natural history¹