Polypoidal Choroidal Vasculopathy

  • Ichiro Maruko
  • Tomohiro Iida


Polypoidal choroidal vasculopathy (PCV) is a disease reported by Yannuzzi et al. (1990) in 1990 and is now considered one of phenotypes of age-related macular degeneration (AMD). While exudative AMD is seen in approximately 5–10 % of western populations, various previous reports have confirmed that its prevalence is high in Asian countries including Japan (Ahuja et al. 2000; Byeon et al. 2008; Kwok et al. 2002; Maruko et al. 2007; Sho et al. 2003; Wen et al. 2004; Yannuzzi et al. 1999). According to our research, in which classification of AMD subtypes using fluorescein and indocyanine green angiography was conducted to 289 cases of patients with exudative AMD newly found in 2 years from 2003 to 2005, PCV, typical AMD, and retinal angiomatous proliferation (RAP) were seen in 158 (54.7 %), 102 (35.3 %), and 13 (4.5 %) cases, respectively (Maruko et al. 2007). While our report is a hospital-based study, when considered in connection with other reports, it shows that approximately half of cases suspected of exudative AMD are most likely to be diagnosed as PCV; therefore, PCV can be considered as one of the most major subtypes rather than a special type of exudative AMD in Japan. Meanwhile, there are some combined cases in which patients have PCV in one eye and typical AMD in the other in our report, so they are figured as disease groups with clinical overlap even though they have different phenotypes (Maruko et al. 2007, 2010). Also, it is shown that there is no large difference of genetic background in development of PCV and exudative AMD (Kondo et al. 2007; Gotoh et al. 2009).


Optical Coherence Tomography Retinal Pigment Epithelium Choroidal Thickness Polypoidal Choroidal Vasculopathy Central Serous Chorioretinopathy 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. Ahuja RM, Stanga PE, Vingerling JR et al (2000) Polypoidal choroidal vasculopathy in exudative and hemorrhagic pigment epithelial detachments. Br J Ophthalmol 84:479–484CrossRefPubMedPubMedCentralGoogle Scholar
  2. Akaza E, Yuzawa M, Mori R (2011) Three-year follow-up results of photodynamic therapy forpolypoidal choroidal vasculopathy. Jpn J Ophthalmol 55:39–44CrossRefPubMedGoogle Scholar
  3. Byeon SH, Lee SC, Oh HS (2008) Incidence and clinical patterns of polypoidal choroidal vasculopathy in Korean patients. Jpn J Ophthalmol 52:57–62CrossRefPubMedGoogle Scholar
  4. Chung SE, Kang SW, Lee JH et al (2011) Choroidal thickness in polypoidal choroidal vasculopathy and exudative age-related macular degeneration. Ophthalmology 118:840–845CrossRefPubMedGoogle Scholar
  5. Gomi F, Sawa M, Sakaguchi H et al (2008) Efficacy of intravitrealbevacizumab for polypoidal choroidal vasculopathy. Br J Ophthalmol 92:70–73CrossRefPubMedGoogle Scholar
  6. Gotoh N, Nakanishi H, Hayashi H et al (2009) ARMS2 (LOC387715) variants in Japanese patients with exudative age-related macular degeneration and polypoidal choroidalvasculopathy. Am J Ophthalmol 147:1037–1041CrossRefPubMedGoogle Scholar
  7. Hikichi T, Ohtsuka H, Higuchi M et al (2010) Improvement of angiographic findings of polypoidal choroidal vasculopathy after intravitreal injection of ranibizumab monthly for 3 months. Am J Ophthalmol 150:674–682CrossRefPubMedGoogle Scholar
  8. Iijima H, Imai M, Gohdo T et al (1999) Optical coherence tomography of idiopathic polypoidal choroidal vasculopathy. Am J Ophthalmol 127:301–305CrossRefPubMedGoogle Scholar
  9. Iijima H, Iida T, Imai M et al (2000) Optical coherence tomography of orange-red subretinallesions in eyes with idiopathic polypoidal choroidal vasculopathy. Am J Ophthalmol 129:21–26CrossRefPubMedGoogle Scholar
  10. Japanese Age-Related Macular Degeneration Tial (JAT) Study Group:Japanese age-relatedmacular degeneration trial (2003) 1-year results of photodynamic therapy with verteporfin in Japanese patients with subfoveal choroidal neovascularization secondary to age-related macular degeneration. Am J Ophthalmol 136:1049–1061CrossRefGoogle Scholar
  11. Japanese study group of polypoidal choroidal vasculopathy (2005) Criteria for diagnosis of polypoidal choroidal vasculopathy. Nihon Ganka Gakkai Zasshi 109:417–427Google Scholar
  12. Khan S, Engelbert M, Imamura Y et al (2012) Polypoidal choroidal vasculopathy: simultaneous indocyanine green angiography and eye-tracked spectral domain optical coherencetomography findings. Retina 32:1057–1068CrossRefPubMedGoogle Scholar
  13. Koh A, Lee WK, Chen LJ et al (2012) EVEREST study: efficacy and safety of verteporfin photodynamic therapy in combination with ranibizumab or alone versus ranibizumabmonotherapy in patients with symptomatic macular polypoidal choroidal vasculopathy. Retina 32:1453–1464CrossRefPubMedGoogle Scholar
  14. Koizumi H, Yamagishi T, Yamazaki T et al (2011) Subfoveal choroidal thickness in typical age-related macular degeneration and polypoidal choroidal vasculopathy. Graefes Arch Clin Exp Ophthalmol 249:1123–1128CrossRefPubMedGoogle Scholar
  15. Koizumi H, Kano M, Yamamoto A et al (2015a) Aflibercept therapy for polypoidal choroidalvasculopathy: short-term results of a multicentre study. Br J Ophthalmol 99:1284–1288. doi: 10.1136/bjophthalmol-2014-306432 CrossRefPubMedGoogle Scholar
  16. Koizumi H, Kano M, Yamamoto A et al (2015b) Short-term changes in choroidal thickness after aflibercept therapy for neovascular age-related macular degeneration. Am J Ophthalmol 159:627–633CrossRefPubMedGoogle Scholar
  17. Kokame GT, Yeung L, Lai JC (2009) Continuous anti-VEGF treatment with ranibizumabforpolypoidal choroidal vasculopathy: 6-month results. Br J Ophthalmol 94:297–301CrossRefPubMedGoogle Scholar
  18. Kondo N, Honda S, Ishibashi K et al (2007) LOC387715/HTRA1 variants in polypoidal choroidal vasculopathy and age-related macular degeneration in a Japanese population. Am J Ophthalmol 144:608–612CrossRefPubMedGoogle Scholar
  19. Kwok AK, Lai TY, Chan CW et al (2002) Polypoidal choroidal vasculopathy in Chinese patients. Br J Ophthalmol 86:892–897CrossRefPubMedPubMedCentralGoogle Scholar
  20. Maruko I, Iida T, Saito M et al (2007) Clinical characteristics of exudative age-related macular degeneration in Japanese patients. Am J Ophthalmol 144:15–22CrossRefPubMedGoogle Scholar
  21. Maruko I, Iida T, Saito M et al (2010) Combined cases of polypoidal choroidal vasculopathy and typical age-related macular degeneration. Graefes Arch Clin Exp Ophthalmol 248:361–368CrossRefPubMedGoogle Scholar
  22. Maruko I, Iida T, Sugano Y et al (2011) Subfoveal retinal and choroidal thickness after verteporfin photodynamic therapy for polypoidal choroidal vasculopathy. Am J Ophthalmol 151:594–603CrossRefPubMedGoogle Scholar
  23. Maruko I, Iida T, Oyamada H et al (2013) Choroidal thickness changes after intravitreal ranibizumab and photodynamic therapy in recurrent polypoidal choroidal vasculopathy. Am J Ophthalmol 156:548–556CrossRefPubMedGoogle Scholar
  24. Ojima Y, Hangai M, Sakamoto A et al (2009) Improved visualization of polypoidal choroidal vasculopathy lesions using spectral-domain optical coherence tomography. Retina 29:52–59CrossRefPubMedGoogle Scholar
  25. Otsuji T, Takahashi K, Fukushima I et al (2000) Optical coherence tomographic findings of idiopathic polypoidal choroidal vasculopathy. Ophthalmic Surg Lasers 31:210–214PubMedGoogle Scholar
  26. Saito M, Iida T, Nagayama D (2008a) Cross-sectional and en face optical coherence tomographic features of polypoidal choroidal vasculopathy. Retina 28:459–469CrossRefPubMedGoogle Scholar
  27. Saito M, Iida T, Nagayama D (2008b) Photodynamic therapy with verteporfin for age-related macular degeneration or polypoidal choroidal vasculopathy: comparison of the presence of serous retinal pigment epithelial detachment. Br J Ophthalmol 92:1642–1647CrossRefPubMedGoogle Scholar
  28. Sasahara M, Tsujikawa A, Musashi K et al (2006) Polypoidal choroidal vasculopathy with choroidal vascular hyperpermeability. Am J Ophthalmol 142:601–607CrossRefPubMedGoogle Scholar
  29. Sato T, Kishi S, Watanabe G et al (2007) Tomographic features of branching vascular networks in polypoidal choroidal vasculopathy. Retina 27:589–594CrossRefPubMedGoogle Scholar
  30. Sho K, Takahashi K, Yamada H et al (2003) Polypoidal choroidal vasculopathy: incidence, demographic features, and clinical characteristics. Arch Ophthalmol 121:1392–1396CrossRefPubMedGoogle Scholar
  31. Spaide RF, Campeas L, Haas A et al (1996) Central serous chorioretinopathy in younger and older adults. Ophthalmology 103:2070–2080CrossRefPubMedGoogle Scholar
  32. Tano Y, Ophthalmic PDT Study Group (2008) Guidelines for PDT in Japan. Ophthalmology 115:585–585CrossRefPubMedGoogle Scholar
  33. Tsujikawa A, Sasahara M, Otani A et al (2007) Pigment epithelial detachment in polypoidal choroidal vasculopathy. Am J Ophthalmol 143:102–111CrossRefPubMedGoogle Scholar
  34. Wen F, Chen C, Wu D et al (2004) Polypoidal choroidal vasculopathy in elderly Chinese patients. Graefes Arch Clin Exp Ophthalmol 242:625–629CrossRefPubMedGoogle Scholar
  35. Yamazaki T, Koizumi H, Yamagishi T et al (2012) Subfoveal choroidal thickness after ranibizumab therapy for neovascular age-related macular degeneration: 12-month results. Ophthalmology 119:1621–1627CrossRefPubMedGoogle Scholar
  36. Yannuzzi LA, Sorenson J, Spaide RF et al (1990) Idiopathic polypoidal choroidal vasculopathy (IPCV). Retina 10:1–8CrossRefPubMedGoogle Scholar
  37. Yannuzzi LA, Ciardella A, Spaide RF et al (1997) The expanding clinical spectrum of idiopathic polypoidal choroidal vasculopathy. Arch Ophthalmol 115:478–485CrossRefPubMedGoogle Scholar
  38. Yannuzzi LA, Wong DW, Sforzolini BS et al (1999) Polypoidal choroidal vasculopathy and neovascularized age-related macular degeneration. Arch Ophthalmol 117:1503–1510CrossRefPubMedGoogle Scholar
  39. Yannuzzi LA, Freund KB, Goldbaum M et al (2000) Polypoidal choroidal vasculopathy masquerading as central serous chorioretinopathy. Ophthalmology 107:767–777CrossRefPubMedGoogle Scholar

Copyright information

© Springer India 2017

Authors and Affiliations

  1. 1.Department of OphthalmologyTokyo Women’s Medical University School of MedicineTokyoJapan

Personalised recommendations