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Idiopathic polypoidal choroidal vasculopathy: a review of literature with clinical update on current management practices

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Abstract

Purpose

Polypoidal choroidal vasculopathy is a major cause of visual disability in a vast majority of Asian population due to exudative maculopathy. Although it is a distinctive disease entity with characteristic pathophysiology, genetics, immunology and clinical features, but it is still misdiagnosed as neovascular age related macular degeneration as both the diseases are a part of pachychoroid spectrum and have some similar features. Also, there are varied options for the management of this disease, but there are no clear recommendations. So, a detailed review of the literature has been done along with special attention to the recent therapeutic advances to help the readers get a better understanding of the disease and its current management practices.

Method

Detailed review of literature regarding polypoidal choroidal vasculopathy was done. The disease pathophysiology, genetics, risk factors, diagnostic modalities along with current treatment guidelines were extensively studied and compiled.

Result

A comprehensive clinical update on polypoidal choroidal vasculopathy was compiled with special emphasis on the recent diagnostic modalities and treatment guidelines.

Conclusion

Polypoidal choroidal vasculopathy is a distinct clinical entity which can be diagnosed based on indocyanine green angiography and optical coherence tomography. Treatment includes various options like photodynamic therapy, anti VEGF agents and thermal laser ablation. A review of literature has been done and recent diagnostic modalities with management practices have been compiled for the better understanding of the disease.

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References

  1. Yannuzzi LA, Sorenson J, Spaide RF et al (1990) Idiopathic polypoidal choroidal vasculopathy (IPCV). Retina 10:1–8

    CAS  PubMed  Google Scholar 

  2. Kleiner RC, Brucker AJ, Johnson RL (1984) Posterior uveal bleeding syndrome. Ophthalmology 94:110

    Google Scholar 

  3. Stern RM, Zakov ZN, Zegarra H et al (1985) Multiple recurrent serosanguineous retinal pigment epithelial detachments in black women. Am J Ophthalmol 100:560–569

    CAS  PubMed  Google Scholar 

  4. Yannuzzi LA, Ciardella A, Spaide RF et al (1997) The expanding clinical spectrum of idiopathic polypoidal choroidal vasculopathy. Arch Ophthalmol 115:478–485

    CAS  PubMed  Google Scholar 

  5. Yannuzzi LA, Wong DW, Sforzolini BS et al (1999) Polypoidal choroidal vasculopathy and neovascularized age-related macular degeneration. Arch Ophthalmol 117:1503–1510

    CAS  PubMed  Google Scholar 

  6. Coscas G, Lupidi M, Coscas F et al (2015) Toward a specific classification of polypoidal choroidal vasculopathy: idiopathic disease or subtype of age-related macular degeneration. Invest Ophthalmol Vis Sci 56:3187–3195

    CAS  PubMed  Google Scholar 

  7. Lafaut BA, Aisenbrey S, VandenBroecke C et al (2000) Polypoidal choroidal vasculopathy pattern in age-related macular degeneration: a clinicopathologic correlation. Retina 20:650–654

    CAS  PubMed  Google Scholar 

  8. MacCumber MW, Dastgheib K, Bressler NM et al (1994) Clinicopathologic correlation of the multiple recurrent serosanguineous retinal pigment epithelial detachments syndrome. Retina 14:143–152

    CAS  PubMed  Google Scholar 

  9. Okubo A, Sameshima M, Uemura A et al (2002) Clinicopathological correlation of polypoidal choroidal vasculopathy revealed by ultrastructural study. Br J Ophthalmol 86:1093–1098

    CAS  PubMed  PubMed Central  Google Scholar 

  10. Nakashizuka H, Mitsumata M, Okisaka S et al (2008) Clinicopathologic findings in polypoidal choroidal vasculopathy. Invest Ophthalmol Vis Sci 49:4729–4737

    PubMed  Google Scholar 

  11. Rosa RH Jr, Davis JL, Eifrig CW (2002) Clinicopathologic reports, case reports and small case series:clinicopathologic correlation of idiopathic polypoidal choroidal vasculopathy. Arch Ophthalmol 120(4):502–508

    PubMed  Google Scholar 

  12. Nakashizuka H, Mitsumata M, Okisaka S et al (2008) Clinicopathologic findings in polypoidal choroidal vasculopathy. Invest Ophthalmol Vis Sci 49(11):4729–4737

    PubMed  Google Scholar 

  13. Lee MY, Lee WK, Back J et al (2013) Photodynamic theory versus combination theory in polypoidal choroidal vasculopathy:changes of aqueous vascular endothelial growth factor. Am J Ophthalmol 156(2):343–348

    CAS  PubMed  Google Scholar 

  14. Subhi Y, Nielsen MK, Molbech CR et al (2017) T-cell differentiation and CD56 + levels in polypoidal choroidal vasculopathy and neovascular age-related macular degeneration. Aging 9:2436–2452

    CAS  PubMed  PubMed Central  Google Scholar 

  15. Lee WK, Back J, Dansingani KK et al (2016) Choroidal morphology in eyes with polypoidal choroidal vasculopathy and normal or subnormal choroidal thickness. Retina 36:S73–S82

    PubMed  Google Scholar 

  16. Kumar S, Nakashizuka H, Jones A et al (2017) Proteolytic degradation and inflammation play critical roles in polypoidal choroidal vasculopathy. Am J Pathol 187:2841–2857

    CAS  PubMed  PubMed Central  Google Scholar 

  17. Subhi Y, Krogh Nielsen M, Molbech CR et al (2018) Altered proportion of CCR2+ and CX3CR1+ circulating monocytes in neovascular age-related macular degeneration and polypoidal choroidal vasculopathy. Clin Exp Ophthalmol 46:661–669

    PubMed  Google Scholar 

  18. Kubisz P, Chudy P, Stasko J et al (2010) Circulating vascular endothelial growth factor in the normo- and/or microalbuminuric patients with type 2 diabetes mellitus. Acta Diabetol 47:119–124

    CAS  PubMed  Google Scholar 

  19. Sakurada Y, Yoneyama S, Imasawa M et al (2013) Systemic risk factors associated with polypoidal choroidal vasculopathy and neovascular agerelated macular degeneration. Retina 33:841–845

    PubMed  Google Scholar 

  20. Lip PL, Hope-Ross MW, Gibson JM (2000) Idiopathic polypoidal choroidal vasculopathy: a disease with diverse clinical spectrum and systemic associations. Eye 14:695–700

    PubMed  Google Scholar 

  21. Cackett P, Yeo I, Cheung CM et al (2011) Relationship of smoking and cardiovascular risk factors with polypoidal choroidal vasculopathy and age-related macular degeneration in Chinese persons. Ophthalmology 118:846–852

    PubMed  Google Scholar 

  22. Ueta T, Obata R, Inoue Y et al (2009) Background comparison of typical age-related macular degeneration and polypoidal choroidal vasculopathy in Japanese patients. Ophthalmology 116:2400–2406

    PubMed  Google Scholar 

  23. Mahdy RA, Nada WM, Hadhoud KM et al (2010) The role of vascular endothelial growth factor in the progression of diabetic vascular complications. Eye 24:1576–1584

    CAS  PubMed  Google Scholar 

  24. Park HS, Kim IT (2012) Clinical characteristics of polypoidal choroidal vasculopathy associated with chronic central serous chorioretinopathy. Korean J Ophthalmol 26:15–20

    PubMed  PubMed Central  Google Scholar 

  25. Ahuja RM, Downes SM, Stanga PE et al (2001) Polypoidal choroidal vasculopathy and central serous chorioretinopathy. Ophthalmology 108:1009–1010

    CAS  PubMed  Google Scholar 

  26. Kikuchi M, Nakamura M, Ishikawa K et al (2007) Elevated C-reactive protein levels in patients with polypoidal choroidal vasculopathy and patients with neovascular age-related macular degeneration. Ophthalmology 114:1722–1727

    PubMed  Google Scholar 

  27. Subhi Y, Krogh Nielsen M, Molbech CR et al (2019) Plasma markers of chronic lowgrade inflammation in polypoidal choroidal vasculopathy and neovascular age-related macular degeneration. Acta Ophthalmol 97:99–106

    CAS  PubMed  Google Scholar 

  28. Cheng H-C, Liu J-H, Lee S-M et al (2014) Hyperhomocysteinemia in patients with polypoidal choroidal vasculopathy: a case control study. PLoS ONE 9:e110818

    PubMed  PubMed Central  Google Scholar 

  29. McCully KS (2009) Chemical pathology of homocysteine. IV. Excitotoxicity, oxidative stress, endothelial dysfunction, and inflammation. Ann Clin Lab Sci 39:219–232

    CAS  PubMed  Google Scholar 

  30. Zeng R, Wen F, Zhang X et al (2013) Serum levels of matrix metalloproteinase 2 and matrix metalloproteinase 9 elevated in polypoidal choroidal vasculopathy but not in age-related macular degeneration. Mol Vis 19:729–736

    CAS  PubMed  PubMed Central  Google Scholar 

  31. Tao Y, Hu J, Li XX (2012) Clinical characteristics of 254 cases of polypoidal choroidal vasculopathy. Chin J Ocular Fundus Dis 28:4

    Google Scholar 

  32. Sho K, Takahashi K, Yamada H et al (2003) Polypoidal choroidal vasculopathy: incidence, demographic features, and clinical characteristics. Arch Ophthalmol 121:1392–1396

    PubMed  Google Scholar 

  33. Ladas ID, Rouvas AA, Moschos MM et al (2004) Polypoidal choroidal vasculopathy and exudative age-related macular degeneration in Greek population. Eye 18:455–459

    CAS  PubMed  Google Scholar 

  34. Yadav S, Parry DG, Beare NAV et al (2017) Polypoidal choroidal vasculopathy: a common type of neovascular age-related macular degeneration in Caucasians. Br J Ophthalmol 101:1377–1380

    PubMed  Google Scholar 

  35. Byeon SH, Lee SC, Oh H-S et al (2008) Incidence and clinical patterns of polypoidal choroidal vasculopathy in Korean patients. Jpn J Ophthalmol 52:57–62

    PubMed  Google Scholar 

  36. Anantharaman G, Ramkumar G, Gopalakrishnan M, Rajput A (2010) Clinical features, management and visual outcome of polypoidal choroidal vasculopathy in Indian patients. Indian J Ophthalmol 58:399–405

    PubMed  PubMed Central  Google Scholar 

  37. Imamura Y, Engelbert M, Iida T, Freund KB, Yannuzzi LA (2010) Polypoidal choroidal vasculopathy: a review. Surv Ophthalmol 55:501–515

    PubMed  Google Scholar 

  38. Lorentzen TD, Subhi Y, Sorensen TL (2018) Prevalence of polypoidal choroidal vasculopathy in white patients with exudative age-related macular degeneration: systematic review and meta-analysis. Retina 38:2363–2371

    PubMed  Google Scholar 

  39. Ciardella AP, Donsoff IM, Huang SJ et al (2004) Polypoidal choroidal vasculopathy. Surv Ophthalmol 49:25–37

    PubMed  Google Scholar 

  40. Honda S, Matsumiya W, Negi A (2014) Polypoidal choroidal vasculopathy: clinical features and genetic predisposition. Ophthalmologica 231:59–74

    PubMed  Google Scholar 

  41. Uyama M, Wada M, Nagai Y et al (2002) Polypoidal choroidal vasculopathy: natural history. Am J Ophthalmol 133:639–648

    PubMed  Google Scholar 

  42. Tan CS, Lim LW, Ngo WK (2016) Treatment of massive subretinal hemorrhage from polypoidal choroidal vasculopathy and age-related macular degeneration. Int Ophthalmol 37(4):779–780

    PubMed  Google Scholar 

  43. Moorthy RS, Lyon AT, Rabb MF et al (1998) Idiopathic polypoidal choroidal vasculopathy of the macula. Ophthalmology 105:1380–1385

    CAS  PubMed  Google Scholar 

  44. Yannuzzi LA, Nogueira FB, Spaide RF et al (1998) Idiopathic polypoidal choroidal vasculopathy: a peripheral lesion. Arch Ophthalmol 116:382–383

    CAS  PubMed  Google Scholar 

  45. Smith RE, Wise K, Kingsley RM (2000) Idiopathic polypoidal choroidal vasculopathy and sickle cell retinopathy. Am J Ophthalmol 129(4):544–546

    CAS  PubMed  Google Scholar 

  46. Bartlett HM, Willoughby B, Mandava N (2001) Idiopathic polypoidal choroidal vasculopathy in a patient with melanocytoma of the optic nerve. Retina 21(4):396–399

    CAS  PubMed  Google Scholar 

  47. Mauget-Faysse M, Cornut PL, Quaranta El-Maftouhi M et al (2006) Polypoidal choroidal vasculopathy in tilted disk syndrome and high myopia with staphyloma. Am J Ophthalmol 142(6):970–975

    PubMed  Google Scholar 

  48. Li H, Wen F, Wu D (2004) Polypoidal choroidal vasculopathy in a patient with circumscribed choroidal hemangioma. Retina. 24(4):629–631

    PubMed  Google Scholar 

  49. Lafaut BA, Leys AM, Snyers B et al (2000) Polypoidal choroidal vasculopathy in Caucasians. Graefes Arch Clin Exp Ophthalmol 238:752–759

    CAS  PubMed  Google Scholar 

  50. Bhoomibunchoo C, Yospaiboon Y, Thoongsuwan S et al (2017) Idiopathic polypoidal choroidal vasculopathy in Thai patients with clinical and angiographic choroidal neovascularization. Clin Ophthalmol 11:317–322

    PubMed  PubMed Central  Google Scholar 

  51. Gomi F, Sawa M, Mitarai K et al (2007) Angiographic lesion of polypoidal choroidal vasculopathy on indocyanine green and fluorescein angiography. Graefes Arch Clin Exp Ophthalmol 245:1421–1427

    PubMed  Google Scholar 

  52. Tan CSH, Ngo WK, Lim LW et al (2014) A novel classification of the vascular patterns of polypoidal choroidal vasculopathy and its relation to clinical outcomes. Br J Ophthalmol 98:1528–1533

    PubMed  Google Scholar 

  53. Anantharaman G, Sheth J, Bhende M, Narayanan R, Natarajan S, Rajendran A et al (2018) Polypoidal choroidal vasculopathy: pearls in diagnosis and management. Indian J Ophthalmol 66:896–908

    PubMed  PubMed Central  Google Scholar 

  54. Koh AH, Expert PCVP, Chen LJ, Chen SJ, Chen Y, Giridhar A, Iida T et al (2013) Polypoidal choroidal vasculopathy: evidence–based guidelines for clinical diagnosis and treatment. Retina 33:686–716

    PubMed  Google Scholar 

  55. Gomi F, Sawa M, Sakaguchi H et al (2008) Efficacy of intravitreal bevacizumab for choroidal vasculopathy. Br J Ophthalmol 92(1):70–73

    CAS  PubMed  Google Scholar 

  56. Spaide RF, Donsoff I, Lam DL et al (2012) Treatment of polypoidal choroidal vasculopathy with photodynamic therapy. Retina 32(Suppl. 1):529–535

    PubMed  Google Scholar 

  57. Chan W-M, Lam DSC, Lai TYY et al (2004) Photodynamic therapy with verteporfin for symptomatic polypoidal choroidal vasculopathy: one-year results of a prospective case series. Ophthalmology 111:1576–1584

    PubMed  Google Scholar 

  58. Silva RM, Figueira J, Cachulo ML et al (2005) Polypoidal choroidal vasculopathy and photodynamic therapy with verteporfin. Graefes Arch Clin Exp Ophthalmol 243:973–979

    CAS  PubMed  Google Scholar 

  59. Akaza E, Yuzawa M, Matsumoto Y et al (2007) Role of photodynamic therapy in polypoidal choroidal vasculopathy. Jpn J Ophthalmol 51:270–277

    PubMed  Google Scholar 

  60. Otani A, Sasahara M, Yodoi Y et al (2007) Indocyanine green angiography: guided photodynamic therapy for polypoidal choroidal vasculopathy. Am J Ophthalmol 144:7–14

    CAS  PubMed  Google Scholar 

  61. Lee SC, Seong YS, Kim SS et al (2004) Photodynamic therapy with verteporfin for polypoidal choroidal vasculopathy of the macula. Ophthalmologica 218:193–201

    CAS  PubMed  Google Scholar 

  62. Kurashige Y, Otani A, Sasahara M et al (2008) Two-year results of photodynamic therapy for polypoidal choroidal vasculopathy. Am J Ophthalmol 146:513–519

    PubMed  Google Scholar 

  63. Akaza E, Yuzawa M, Mori R (2011) Three-year follow-up results of photodynamic therapy for polypoidal choroidal vasculopathy. Jpn J Ophthalmol 55:39–44

    CAS  PubMed  Google Scholar 

  64. Hirami Y, Tsujikawa A, Otani A et al (2007) Hemorrhagic complications after photodynamic therapy for polypoidal choroidal vasculopathy. Retina 27:335–341

    PubMed  Google Scholar 

  65. Oishi A, Kojima H, Mandai M et al (2013) Comparison of the effect of ranibizumab and verteporfin for polypoidal choroidal vasculopathy: 12-month LAPTOP study results. Am J Ophthalmol 156:644–651

    CAS  PubMed  Google Scholar 

  66. Ojima Y, Tsujikawa A, Otani A et al (2006) Recurrent bleeding after photodynamic therapy in polypoidal choroidal vasculopathy. Am J Ophthalmol 141:958–960

    PubMed  Google Scholar 

  67. Imai H, Honda S, Nakanishi Y et al (2006) Different transitions of multifocal electroretinogram recording s between patients with age-related macular degeneration and polypoidal choroidal vasculopathy after photodynamic therapy. Br J Ophthalmol 90(12):1524–1530

    CAS  PubMed  PubMed Central  Google Scholar 

  68. Musashi K, Tsujikawa A, Hirami Y et al (2007) Microrips of the retinal pigment epithelium in polypoidal choroidal vasculopathy. Am J Ophthalmol 143:883–885

    PubMed  Google Scholar 

  69. Lee WK, Kim KS, Kim W et al (2012) Responses to photodynamic therapy in patients with polypoidal choroidal vasculopathy consisting of polyps resembling grape clusters. Am J Ophthalmol 154:355

    PubMed  Google Scholar 

  70. Fan NW, Lau LI, Chen SJ, Yang CS, Lee FL (2014) Comparison of the effect of reduced–fluence photodynamic therapy with intravitreal bevacizumab and standard–fluence alone for polypoidal choroidal vasculopathy. J Chin Med Assoc 77:101–107

    CAS  PubMed  Google Scholar 

  71. Yamashita A, Shiraga F, Shiragami C et al (2010) One-year results of reduced-fluence photodynamic therapy for polypoidal choroidal vasculopathy. Am J Ophthalmol 149:465

    CAS  PubMed  Google Scholar 

  72. Michels S, Hansmann F, Geitzenauer W, Schmidt-Erfurth U (2006) Influence of treatment parameters on selectivity of verteporfin therapy. Invest Ophthalmol Vis Sci 47:371–376

    PubMed  Google Scholar 

  73. Ricci F, Calabrese A, Regine F et al (2012) Combined reduced fluence photodynamic therapy and intravitreal ranibizumab for polypoidal choroidal vasculopathy. Retina 32:1280–1288

    CAS  PubMed  Google Scholar 

  74. Matsuoka M, Ogata N, Otsuji T et al (2004) Expression of pigment epithelium derived factor and vascular endothelial growth factor in choroidal neovascular membranes and polypoidal choroidal vasculopathy. Br J Ophthalmol 88:809–815

    CAS  PubMed  PubMed Central  Google Scholar 

  75. Tong J-P, Chan W-M, Liu DTL et al (2006) Aqueous humor levels of vascular endothelial growth factor and pigment epithelium-derived factor in polypoidal choroidal vasculopathy and choroidal neovascularization. Am J Ophthalmol 141:456–462

    CAS  PubMed  Google Scholar 

  76. Kokame GT, Yeung L, Lai JC (2010) Continuous anti-VEGF treatment with ranibizumab for polypoidal choroidal vasculopathy: 6-month results. Br J Ophthalmol 94:297–301

    PubMed  Google Scholar 

  77. Chhablani JK, Narula R, Narayanan R (2013) Intravitreal bevacizumab monotherapy for treatment-naïve polypoidal choroidal vasculopathy. Indian J Ophthalmol 61:136–138

    PubMed  PubMed Central  Google Scholar 

  78. Yamamoto A, Okada AA, Kano M et al (2015) One-year results of intravitreal aflibercept for polypoidal choroidal vasculopathy. Ophthalmology 122:1866–1872

    PubMed  Google Scholar 

  79. Hara C, Sawa M, Sayanagi K et al (2016) One-year results of intravitreal aflibercept for polypoidal choroidal vasculopathy. Retina 36:37–45

    CAS  PubMed  Google Scholar 

  80. Cho HJ, Kim JW, Lee DW et al (2012) Intravitreal bevacizumab and ranibizumab injections for patients with polypoidal choroidal vasculopathy. Eye 26:426–433

    CAS  PubMed  Google Scholar 

  81. Qu J, Cheng Y, Li X et al (2016) Efficacy of intravitreal injection of conbercept in polypoidal choroidal vasculopathy: subgroup analysis of the Aurora study. Retina 36:926–937

    CAS  PubMed  Google Scholar 

  82. Koh A, Lee WK, Chen L-J et al (2012) 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 32:1453–1464

    CAS  PubMed  Google Scholar 

  83. Koh A, Lai TYY, Takahashi K et al (2017) Efficacy and safety of ranibizumab with or without verteporfin photodynamic therapy for polypoidal choroidal vasculopathy: a randomized clinical trial. JAMA Ophthalmol 135:1206–1213

    PubMed  PubMed Central  Google Scholar 

  84. Lee WK, Iida T, Ogura Y et al (2018) Efficacy and safety of intravitreal aflibercept for polypoidal choroidal vasculopathy in the PLANET study: a randomized clinical trial. JAMA Ophthalmol 136:786–793

    PubMed  PubMed Central  Google Scholar 

  85. Gemmy Cheung CM, Yeo I, Li X, Mathur R, Lee SY, Chan CM et al (2013) Argon laser with and without anti–vascular endothelial growth factor therapy for extrafoveal polypoidal choroidal vasculopathy. Am J Ophthalmol 155:295–3040

    PubMed  Google Scholar 

  86. Zuo C, Zhang X, Li M, Peng Y, Mi L, Liu B et al (2019) Case series of coexistence of polypoidal choroidal vasculopathy with other rare fundus diseases. Int Ophthalmol 39:987–990

    PubMed  Google Scholar 

  87. Asano Shimizu K, Asano S, Murata H et al (2020) Early changes of vascular lesions and responses to combined photodynamic therapy in patients with polypoidal choroidal vasculopathy. Int Ophthalmol 40:1335–1345

    PubMed  Google Scholar 

  88. Calvo GC, Reche Frutos J, Fernández Vigo JI et al (2019) Indocyanine green angiography findings in patients with neovascular age-related macular degeneration refractory to ranibizumab switched to aflibercept. Int Ophthalmol 3:2441–2448

    Google Scholar 

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Sahu, Y., Chaudhary, N., Joshi, M. et al. Idiopathic polypoidal choroidal vasculopathy: a review of literature with clinical update on current management practices. Int Ophthalmol 41, 753–765 (2021). https://doi.org/10.1007/s10792-020-01620-0

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