Polypoidal Choroidal Vasculopathy
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Purpose of Review
The aim of this review is to summarize developments in the treatment of active polypoidal choroidal vasculopathy (PCV). PCV is associated with a poor visual prognosis as a consequence of the condition’s hallmark polypoidal dilatation and a branching network resulting in recurrent hemorrhages and serous leakage.
Recent research has provided new insights into the pathogenesis of PCV. While still considered a subtype of age-related macular degeneration, suggestions that PCV belongs to a spectrum of conditions that present with a pachychoroid are increasingly well accepted. Treatment remains challenging. Combination therapy (photodynamic therapy (PDT) and intravitreal anti-vascular endothelial growth factor (VEGF)) is associated with higher polyp closure rate, but polyp closure rate has not been correlated with superior visual outcomes. Current data points to non-inferiority of anti-VEGF alone versus combined with PDT when final vision acuity is the study outcome.
PCV remains a clinical challenge. Classification and treatment of the condition continue to evolve. Combination therapy may not be superior to anti-VEGF treatment alone in terms of visual acuity outcome; however, data on long-term recurrence should be compared in formulating preferred treatment plans.
KeywordsPolypoidal choroidal vasculopathy Branching vascular network Serosanguinous retinal pigment epithelium detachment Retinal detachment Vitreous hemorrhage
The editors thank Dr. Luiz Roisman for lending his expertise and reviewing this manuscript.
Compliance with Ethical Standards
Conflict of Interest
Raquel Goldhardt and Bradley Simon Rosen declare that they have no conflict of interest.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
Supported by the Department of Veterans Affairs, Veterans Health Administration, Office of Research and Development, Clinical Sciences Research EPID-006-15S, NIH Center Core Grant P30EY014801 and Research to Prevent Blindness Unrestricted Grant.
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
- 2.Yannuzzi LA, Ciardella A, Spaide RF, Rabb M, Freund KB, Orlock DA. The expanding clinical spectrum of idiopathic polypoidal choroidal vasculopathy. Arch Ophthalmol. 1997;115:478–85. https://doi.org/10.1001/archopht.1997.01100150480005.CrossRefGoogle Scholar
- 7.Tan CS, Ngo WK, Chen JP, Tan NW, Lim TH, Group ES. EVEREST study report 2: imaging and grading protocol, and baseline characteristics of a randomised controlled trial of polypoidal choroidal vasculopathy. Br J Ophthalmol. 2015;99:624–8. https://doi.org/10.1136/bjophthalmol-2014-305674.CrossRefGoogle Scholar
- 11.Cackett P, Yeo I, Cheung CMG, Vithana EN, Wong D, Tay WT, et al. Relationship of smoking and cardiovascular risk factors with polypoidal choroidal vasculopathy and age-related macular degeneration in Chinese persons. Ophthalmology. 2011;118:846–52. https://doi.org/10.1016/j.ophtha.2010.09.026.CrossRefGoogle Scholar
- 16.Lim FP, et al. Prevalence and clinical correlates of focal choroidal excavation in eyes with age-related macular degeneration, polypoidal choroidal vasculopathy and central serous chorioretinopathy. The British journal of ophthalmology.2015, https://doi.org/10.1109/TBME.2019.2891240.
- 17.Kim JH, Chang YS, Kim JW, Lee TG, Kim CG. Prevalence of subtypes of reticular Pseudodrusen in newly diagnosed exudative age-related macular degeneration and Polypoidal choroidal vasculopathy in Korean patients. Retina. 2015;35:2604–12. https://doi.org/10.1097/IAE.0000000000000633.CrossRefGoogle Scholar
- 19.Wen F, Chen C, Wu D, Li H. Polypoidal choroidal vasculopathy in elderly Chinese patients. Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle. Ophthalmologie. 2004;242:625–9.Google Scholar
- 24.Kikuchi M, Nakamura M, Ishikawa K, Suzuki T, Nishihara H, Yamakoshi T, et al. Elevated C-reactive protein levels in patients with polypoidal choroidal vasculopathy and patients with neovascular age-related macular degeneration. Ophthalmology. 2007;114:1722–7. https://doi.org/10.1016/j.ophtha.2006.12.021.CrossRefGoogle Scholar
- 30.Koh A, Lee WK, Chen LJ, Chen SJ, Hashad Y, Kim H, et al. EVEREST study: efficacy and safety of verteporfin photodynamic therapy in combination with ranibizumab or alone versus ranibizumab monotherapy in patients with symptomatic macular polypoidal choroidal vasculopathy. Retina. 2012;32:1453–64. https://doi.org/10.1097/IAE.0b013e31824f91e8.CrossRefGoogle Scholar
- 32.Liu R, Li J, Li Z, Yu S, Yang Y, Yan H, et al. Distinguishing Polypoidal choroidal vasculopathy from typical Neovascular age-related macular degeneration based on spectral domain optical coherence tomography. Retina. 2016;36:778–86. https://doi.org/10.1097/IAE.0000000000000794.CrossRefGoogle Scholar
- 33.Sato T, Kishi S, Watanabe G, Matsumoto H, Mukai R. Tomographic features of branching vascular networks in polypoidal choroidal vasculopathy. Retina. 2007;27:589–94. https://doi.org/10.1097/01.iae.0000249386.63482.05.CrossRefGoogle Scholar
- 34.Koizumi H, Yamagishi T, Yamazaki T, Kawasaki R, Kinoshita S. Subfoveal choroidal thickness in typical age-related macular degeneration and polypoidal choroidal vasculopathy. Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle. Ophthalmologie. 2011;249:1123–8.Google Scholar
- 42.• Koh A, et al. Efficacy and Safety of Ranibizumab With or Without Verteporfin Photodynamic Therapy for Polypoidal Choroidal Vasculopathy: A Randomized Clinical Trial. JAMA Ophthalmol. 2017;135:1206–13. Evaluated anti-VEGF and combination therapy showing same visual acuity between groups at one year. If following a PRN regimen, 3 monthly ranibizumab injections followed by monthly monitoring with retreatment offered on a PRN basis is indicated. https://doi.org/10.1001/jamaophthalmol.2017.4030.CrossRefGoogle Scholar
- 43.•• Lee WK, et al. Efficacy and Safety of Intravitreal Aflibercept for Polypoidal Choroidal Vasculopathy in the PLANET Study: A Randomized Clinical Trial. JAMA Ophthalmol. 2018;136:786–93. 96-week multicenter randomized clincal trial that compared intravitreal aflibercept injection only versus injection and rescue with PDT showing that at 1 year, vision was the same between both groups with a polyp closure rate of 38.9 in the injection only group. If anti-VEGF monotherapy is chosen, the fixed dose with 3 monthly aflibercept injections followed by 8 weekly injections should be adopted. https://doi.org/10.1001/jamaophthalmol.2018.1804.CrossRefGoogle Scholar
- 44.Takahashi K, Ohji M, Terasaki H, Honda S, Margaron P, Guerin T, et al. Efficacy and safety of ranibizumab monotherapy versus ranibizumab in combination with verteporfin photodynamic therapy in patients with polypoidal choroidal vasculopathy: 12-month outcomes in the Japanese cohort of EVEREST II study. Clin Ophthalmol. 2018;12:1789–99.CrossRefGoogle Scholar
- 50.Kokame GT. Prospective evaluation of subretinal vessel location in polypoidal choroidal vasculopathy (PCV) and response of hemorrhagic and exudative PCV to high-dose antiangiogenic therapy (an American ophthalmological society thesis). Trans Am Ophthalmol Soc. 2014;112:74–93.Google Scholar
- 52.Miyamoto N, et al. Long-term results of photodynamic therapy or ranibizumab for polypoidal choroidal vasculopathy in LAPTOP study. The British journal of ophthalmology.2018, https://doi.org/10.1161/CIRCEP.118.006989.
- 55.Nakata I, et al. Two-year outcome of photodynamic therapy combined with intravitreal injection of bevacizumab and triamcinolone acetonide for polypoidal choroidal vasculopathy. Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle. Ophthalmologie. 2013;251:1073–80.Google Scholar