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Retinal Physician

Article

Diagnosing and Managing Polypoidal Choroidal Vasculopathy

Evidence-based strategies for diagnosis and therapeutic decision-making.

By: Giulia Gregori, MD, Jay Chhablani, MD
Retinal Physician March 1, 2026 Vol 23, Issue March-April 2026 Page(s): 6-8

Polypoidal choroidal vasculopathy (PCV) is a distinct choroidal vascular disorder initially described in middle-aged Black women who presented with recurrent subretinal and subretinal pigment epithelium (RPE) hemorrhages.1 Although historically reported predominantly in Afro-Caribbean and Asian populations, recent studies have documented PCV in White patients, broadening recognition of the disease globally.2 Established risk factors include smoking, male sex, higher body mass index, hyperlipidemia, hypertension, and elevated serum inflammatory markers, such as C-reactive protein.3 Genetic studies suggest that PCV-associated loci involve the complement cascade, inflammatory pathways, extracellular matrix and basement membrane regulation, and lipid metabolism.4

Previous terminology for PCV includes idiopathic polypoidal vasculopathy, numerous recurrent serosanguinous RPE detachments, posterior uveal hemorrhage syndrome,5 and peripheral exudative hemorrhagic chorioretinopathy.6 Despite this varied nomenclature, these entities represent overlapping phenotypes of a single disease spectrum, providing historical context for current understanding.

Since its initial description, the clinical nature and pathogenesis of PCV have remained subjects of debate. Early reports suggested that the vascular lesions were situated in the inner choroid beneath Bruch’s membrane, based on angiographic findings and clinical observations.7 More recently, PCV is often considered a variant of age-related macular degeneration (AMD), although features such as drusen, pigmentary changes, and geographic atrophy are uncommon. Histologic and imaging studies support the theory that PCV represents a form of type 1 neovascularization rather than a primary choroidopathy, with neovascular complexes situated between the RPE and Bruch’s membrane.8

Figure 1. Multimodal imaging in a 64-year-old male with polypoidal choroidal vasculopathy. Fundus photography (A) shows subretinal hemorrhage involving the macula. Indocyanine green angiography (B) demonstrates 2 focal nodular areas of hypercyanescence, consistent with polypoidal lesions. Optical coherence tomography (C) reveals multiple pigment epithelial detachments with associated subretinal fluid and hemorrhage.

When to Suspect PCV in the Clinic

PCV should be considered in patients presenting with characteristic choroidal vascular lesions, particularly when conventional treatments yield suboptimal results. The dilated choroidal vascular channels that terminate in polyps appear clinically as orange, nodular lesions, most commonly located in the macula and peripapillary region. These lesions are frequently associated with serous or serosanguineous pigment epithelial detachments (PEDs).9 Micro-rips at the margins of PEDs10 and the presence of drusen in 23% to 55% of cases11 have also been observed. Despite these findings, presenting visual acuity is often relatively preserved due to minimal damage to intraretinal structures or extrafoveal lesion location. Bilateral involvement is common.12

Peripheral polyps may cause peripheral exudative hemorrhagic chorioretinopathy, most frequently in the temporal periphery. Although many peripheral polyps are benign and self-resolving, some can cause subretinal hemorrhage that threatens the macula and requires urgent intervention.13 A poor or incomplete response to anti-VEGF therapy is an important clinical clue that may help differentiate PCV from other chorioretinal disorders.14 Race, lesion location, response to therapy, and multimodal imaging can all contribute to an accurate diagnosis.

Figure 2. Multimodal imaging in a 65-year-old man with polypoidal choroidal vasculopathy. Fundus photography (A) shows a scar temporal to the optic nerve and along the inferior arcade, with surrounding suprachoroidal hemorrhage. Indocyanine green angiography (B) demonstrates focal hypercyanescence nasal to the optic disc, suggestive of a polyp. Optical coherence tomography (C) shows extensive pigment epithelial detachments, most prominent nasally.

Diagnostic Criteria

PCV diagnosis requires focal hyperfluorescence associated with at least 1 of the following: abnormal branching vascular network (BVN), nodular appearance on stereoscopic examination, orange retinal nodule, hypofluorescent halo, pulsatile polyp, or large submacular hemorrhage.15 Indocyanine green angiography (ICGA) is the gold standard for PCV diagnosis, identifying the BVN within the choroidal circulation and polypoidal dilations at vessel borders.16,17 Choroidal vascular hyperpermeability is more frequently observed in PCV than in other forms of choroidal neovascularization (CNV).18 Late geographic hyperfluorescence—a well-demarcated hyperfluorescent lesion that appears approximately 10 minutes after ICGA dye injection—is another characteristic finding.19 Nearly all eyes with PCV demonstrate late geographic hyperfluorescence, compared with only 7.5% of eyes with exudative AMD.20 Its presence in the fellow eye, even without a BVN, may indicate preclinical PCV.9

Advances in imaging techniques have led to a paradigm shift from ICGA-based diagnosis to a less invasive approach using optical coherence tomography (OCT). The Asia-Pacific Ocular Imaging Society validated non-ICGA diagnostic criteria, identifying 3 major OCT features with high specificity (91%) and positive predictive value (93%):

  • ring-like lesions beneath the RPE;
  • en face OCT complex RPE elevations corresponding to a BVN; and
  • sharp-peaked PEDs.21

Additional minor criteria include thick choroid with dilated Haller vessels, complex or multilobular PED, and double-layer sign (separation of Bruch’s membrane and RPE by the BVN).22 Enhanced-depth imaging OCT often shows choroidal thickness >300 μm.23,24 This multimodal, noninvasive approach achieves an overall diagnostic accuracy of 82%, effectively distinguishing PCV from typical neovascular AMD when ICGA is unavailable (Figures 1 and 2).13

OCT angiography (OCTA) further improves BVN visualization, revealing hyperflow structures with a detection rate of 55% to 100%.25 BVN flow is typically 28.6 μm below the RPE reference plane.26 Morphologies described include sea-fan, tangled, and Medusa-head patterns.27 Fundus fluorescein angiography (FFA) and ICGA together aid in mapping lesions and determining the greatest linear dimension (GLD), because ICGA alone may not fully visualize PEDs or neurosensory detachments.28-30 Tan and colleagues proposed categorizing PCV based on FFA and ICGA traits: PCV with leaky BVN, PCV with interconnecting channels, and PCV with nonleaking BVN (Table 1).30

Expand

Treatment Approaches

Historically, standard laser photocoagulation was an effective option for extrafoveal polypoidal lesions, achieving lesion regression, reduced exudation, and long-term visual acuity improvement before the advent of current multimodal therapies.31 Today, treatment options include photodynamic therapy (PDT), intravitreal anti-VEGF therapy, a combination of PDT and anti-VEGF, and laser photocoagulation. PDT involves intravenous infusion of verteporfin (Visudyne; Bausch + Lomb), which is activated with a low-intensity laser to induce localized closure of abnormal choroidal blood vessels.

OCT has been used to plan adjunctive rescue PDT without requiring ICGA. Teo et al reported marking near-infrared reflectance images on a 6×6 mm OCT B-scan to cover all polypoidal lesions (PLs) and branching vascular networks (BVNs), achieving coverage of 100% of the PL area and 90% of the BVN area in a single circular location.32

The EVEREST clinical trial demonstrated that PDT, alone or combined with ranibizumab (Lucentis; Genentech), improved polyp closure rates.16 Ranibizumab monotherapy achieved 28.6% closure, compared with 71.4% for PDT alone and 77.8% for PDT plus ranibizumab.33 Anti-VEGF monotherapy and combination therapy were further assessed in EVEREST II and PLANET.34 EVEREST II compared ranibizumab with combination ranibizumab plus PDT at baseline, while PLANET evaluated aflibercept (Eylea; Regeneron) with rescue PDT available after 3 months. At a year, both trials showed significant visual acuity improvement with anti-VEGF monotherapy; polyp closure rates were 34.7% (EVEREST II) and 38.9% (PLANET). The mean number of injections was 7.3 in EVEREST II and 8.1 in PLANET. The evidence suggests that combination therapy resulted in improved vision and achieved a higher rate of complete polyp regression with fewer anti-VEGF injections than monotherapy.35

The LAPTOP study demonstrated that intravitreal ranibizumab provided superior visual acuity outcomes compared with PDT, with benefits maintained up to 5 years.36,37 On the other hand, the EPIC study evaluated intravitreal aflibercept in eyes previously treated with ranibizumab or bevacizumab (Avastin; Genentech), showing stabilization or improvement of vision in 91% of cases over 6 months, along with substantial resolution of subretinal fluid, hemorrhage, polyps, and PEDs in most cases.38

The phase 3 HAWK and HARRIER trials showed that brolucizumab (Beovu; Novartis) was noninferior to aflibercept for visual acuity gains at 48 weeks in patients with neovascular AMD, while allowing >50% of eyes to maintain a 12-week dosing interval.39,40 In PCV subgroups, brolucizumab demonstrated superior anatomic outcomes, including greater reductions in retinal thickness and fluid, suggesting a potential to reduce treatment burden.41

Triple therapy combining PDT, intravitreal anti-VEGF, and corticosteroids has been reported to improve visual outcomes compared to PDT monotherapy. It also allows longer retreatment-free intervals by targeting both vascular and inflammatory components of the disease.42

Recently, Arnold-Vangsted et al reported that faricimab (Vabysmo; Genentech) achieves a polyp regression rate of 48.7% (95% CI, 32.5-65.0%), comparable to rates reported with aflibercept monotherapy.43 In the Japanese subgroup of the phase 3 TENAYA trial, faricimab demonstrated similar visual and anatomical efficacy to aflibercept every 8 weeks at 1 year in treatment-naïve patients with nAMD, with comparable safety profiles. Mean best-corrected visual acuity (BCVA) gains and reductions in central subfield thickness were similar between groups. Notably, faricimab demonstrated greater durability, with approximately 66% of patients maintained on 16-week dosing intervals and nearly 90% on ≥12-week intervals at week 48. These findings, consistent with global TENAYA and LUCERNE results, support faricimab monotherapy as an effective and durable option for PCV.44

Conclusion

For patients with symptomatic PCV, anti-VEGF monotherapy or a combination of anti-VEGF therapy and PDT may serve as the initial treatment approach. Level I evidence from the EVEREST II and PLANET studies demonstrated that anti-VEGF therapy, alone or combined with PDT, can achieve substantial visual improvements after a year. Newer anti-VEGF agents have also shown meaningful efficacy in PCV and PED-associated disease, supporting their potential use as monotherapy.

However, because of the favorable response of the polypoidal complex to PDT, the reduced number of total injections, and the known phenomenon of PCV that is resistant to anti-VEGF treatment, adjunctive PDT may provide superior anatomical and visual outcomes in some cases. Additionally, when patient monitoring or retreatment adherence is a problem, combination therapy may represent the best option.45 RP

 

Giulia Gregori, MD

Giulia Gregori, MD, is an ophthalmologist and researcher in the Eye Clinic, Department of Experimental and Clinical Medicine at the Polytechnic University of Marche in Ancona, Italy. She reports no disclosures.

Jay Chhablani, MD

Jay Chhablani, MD, is a professor of ophthalmology at the University of Pittsburgh School of Medicine in Pittsburgh, Pennsylvania. He reports consulting fees from AbbVie, Allergan, Astellas, Bausch+Lomb, Emmes, Erasca, EyePoint Pharmaceuticals, Iveric Bio, Neurotech, Novartis; OD-OS, and Salutaris. Reach him at chhablanijk2@upmc.edu.

 

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