Abstract
More than three decades ago, a peculiar hemorrhagic disorder of the macula, polypoidal choroidal vasculopathy (PCV), was first described (see Yannuzzi LA’s “Idiopathic polypoidal choroidal vasculopathy” presented at the annual meeting of the Macula Society, Miami, FL, 1982). This entity has also been designated by Kleiner and associates as “posterior uveal bleeding syndrome” [1, 2] and by Stern and co-workers as “multiple recurrent retinal pigment epithelium (RPE) detachments in black women.” [3] In 1990, Yannuzzi and colleagues suggested the term idiopathic polypoidal choroidal vasculopathy (IPCV) because the pathogenesis was unknown, the primary abnormality involved the choroidal circulation, and the characteristic lesion was an inner choroidal vascular network of vessels ending in an aneurysmal bulge or outward projection, visible clinically as a reddish-orange, spheroid, polyp-like structure [4]. Given its appearance and location between RPE and inner collagenous layer of Bruch’s membrane, the alternative classification “type 1 aneurysmal neovascularization” has also been proposed [5]. However, emerging evidence suggests that many lesions thought to be aneurysmal may instead appear as tangles of vessels upon use of advanced imaging techniques [6]. Regardless, this entity has characteristic morphological features that distinguish it from other exudative maculopathies. Clinically, it was associated with multiple, recurrent, serosanguineous detachments of the RPE and neurosensory retina secondary to leakage and bleeding from the peculiar choroidal vascular lesion (Fig. 1).
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Kleiner RC, Brucker AJ, Johnson RL. Posterior uveal bleeding syndrome. Ophthalmology. 1984;91(Suppl 9):110.
Kleiner RC, Brucker AJ, Johnson RL. Posterior uveal bleeding syndrome. Retina. 1990;10:9–17.
Stern RM, Zakov N, Zegarra H, et al. Multiple recurrent serous sanguineous retinal pigment epithelial detachments in black women. Am J Ophthalmol. 1985;100:560–9.
Yannuzzi LA, Sorenson J, Spaide RF, Lipson B. Idiopathic polypoidal choroidal vasculopathy. Retina. 1990;10:1–8.
Dansingani KK, Gal-Or O, Sadda SR, Yannuzzi LA, Freund KB. Understanding aneurysmal type 1 neovascularization (polypoidal choroidal vasculopathy): a lesson in the taxonomy of ‘expanded spectra’ – a review. Clin Exp Ophthalmol. 2018;46(2):189–200.
Bo Q, Yan Q, Shen M, et al. Appearance of polypoidal lesions in patients with polypoidal choroidal vasculopathy using swept-source optical coherence tomographic angiography. JAMA Ophthalmol. 2019;137(6):642–50.
Spaide RF, Yannuzzi LA, Slakter JS, et al. Indocyanine green videoangiography of idiopathic polypoidal choroidal vasculopathy. Retina. 1995;15:100–10.
Ross RD, Gitter GA, Cohen C, Shoemaker KS. Idiopathic polypoidal choroidal vasculopathy associated with retinal arterial macroaneurysm and hypertensive retinopathy. Retina. 1996;16:105–11.
De Salvo G, Vaz-Pereira S, Keane PA, Tufail A, Liew G. Sensitivity and specificity of spectral-domain optical coherence tomography in detecting idiopathic polypoidal choroidal vasculopathy. Am J Ophthalmol. 2014;158(6):1228–38.
Huang CH, Yeh PT, Hsieh YT, et al. Characterizing branching vascular network morphology in polypoidal choroidal vasculopathy by optical coherence tomography angiography. Sci Rep. 2019;9(1):595.
Chi YT, Yang CH, Cheng CK. Optical coherence tomography angiography for assessment of the 3-dimensional structures of polypoidal choroidal vasculopathy. JAMA Ophthalmol. 2017;135(12):1310–6.
Moussa K, Bloomer MM, Schwartz DM, et al. Polypoidal choroidal vasculopathy: a clinicopathologic study. Retin Cases Brief Rep. 2017;11(Suppl 1):S128–31.
Tso MOM, Suarez MJ, Eberhart CG. Pathologic study of early manifestations of polypoidal choroidal vasculopathy and pathogenesis of choroidal neo-vascularization. Am J Ophthalmol Case Rep. 2017;11:176–80.
Yannuzzi LA, Ciardella AP, Spaide RF, et al. The expanding clinical spectrum of idiopathic polypoidal vasculopathy. Arch Ophthalmol. 1997;115:478–85.
Ciardella AP, Donsoff IM, Huang SJ, et al. Polypoidal choroidal vasculopathy. Surv Ophthalmol. 2004;49:25–37.
Perkovich BT, Zakov ZN, Berlin LA, et al. An update on multiple recurrent serosanguineous retinal pigment epithelial detachments in black women. Retina. 1990;10:18–26.
Lafaut BA, Leyes AM, Snyders B, et al. Polypoidal choroidal vasculopathy in Caucasians. Graefe’s Arcg Clin Exp Ophthalmol. 2000;238:752–9.
Scassellati-Sforzolini B, Mariotti C, Bryan R, et al. Polypoidal vasculopathy in Italy. Retina. 2001;21:121–5.
Yannuzzi LA, Slakter JS, Sorenson JA, et al. Digital indocyanine videoangiography and choroidal neovascularization. Retina. 1992;12:191–223.
Ferris FL III. Senile macular degeneration: a review of epidemiological features. Am J Epidemiol. 1983;118:213–21.
Capone A Jr, Wallace RT, Meredith TA. Symptomatic choroidal neovascularization in blacks. Arch Ophthalmol. 1994;112:1091–7.
Lafaut BA, Aisenbrey S, van den Broecke C, et al. Polypoidal choroidal vasculopathy pattern in age-related macular degeneration. A clinicopathologic correlation. Retina. 2000;20:650–4.
Kuroiwa S, Tateiwa H, Hisatomi T, Ishibashi T, Yoshimura N. Pathological features of surgically excised polypoidal choroidal vasculopathy membranes. Clin Exp Ophthalmol. 2004;32(3):297–302.
Nakashizuka H, Mitsumata M, Okisaka S, et al. Clinicopathologic findings in polypoidal choroidal vasculopathy. Invest Ophthalmol Vis Sci. 2008;49(11):4729–37.
Yannuzzi LA, Wong DW, Sforzolino MS, et al. Polypoidal choroidal vasculopathy and neovascularized age-related macular degeneration. Arch Ophthalmol. 1999;117:1503–10.
Uyama M, Wada M, Nagai Y, et al. Polypoidal choroidal vasculopathy: natural history. Am J Ophthalmol. 2002;133:639–48.
Gomez-Ulla F, Gonzalez F, Torreiro MG. Diode laser photocoagulation in idiopathic polypoidal choroidal vasculopathy. Retina. 1998;18:481–3.
Green WR, McDonnel PJ, Yeo JH. Pathological features of senile macular degeneration. Ophthalmology. 1985;92:615–27.
Okubo A, Sameshima M, Uemara A. Clinicopathological correlation of polypoidal choroidal vasculopathy revealed by ultrastructural study. Br J Ophthalmol. 2002;86:1093–8.
Otsuji T, Takahasi K, Fukushima I, Uyama M. Optical coherence tomographic findings of idiopathic polypoidal choroidal vasculopathy. Ophthalmic Surg Lasers. 2000;31:210–4.
Pauleikhoff D, Löffert D, Spital G, et al. Pigment epithelial detachment in the elderly. Clinical differentiation, natural course and pathogenic implications. Graefes Arch Clin Exp Ophthalmol. 2002;240:533–8.
Ahuja RM, Stanga PE, Vingerling JR, et al. Polypoidal choroidal vasculopathy in exudative and hemorrhagic pigment epithelium detachments. Br J Ophthalmol. 2000;84:479–84.
Lip PL, Hope-Ross MW, Gibson JM. Idiopathic polypoidal choroidal vasculopathy: a disease with diverse clinical spectrum and systemic associations. Eye. 2000;5:695–700.
Mohand-Said M, Nodarian M, Salvanet-Bouccara A. Idiopathic polypoidal choroidal vasculopathy: 2 case report. J Fr Ophthalmol. 2002;25:517–21.
Schneider U, Gelisken F, Inhoffen W. Clinical characteristics of idiopathic polypoidal choroidal vasculopathy. Ophthalmologe. 2001;98:1186–91.
Kwok AKH, Lai TYY, Chan CWN, et al. Polypoidal choroidal vasculopathy in Chinese patients. Br J Ophthalmol. 2002;86:892–7.
Imaizumi H, Takeda M. Knobby-like choroidal neovascularization accompanied with retinal pigment epithelial detachment. Nippon Ganka Gakkai Zasshi. 1999;103:527–37.
Koh AH, Expert PCV Panel, Chen LJ, et al. Polypoidal choroidal vasculopathy: evidence-based guidelines for clinical diagnosis and treatment. Retina. 2013;33(4):686–716.
Iida T, Yannuzzi LA, Freund KB, et al. Retinal angiopathy and polypoidal choroidal vasculopathy. Retina. 2002;22:455–63.
Padhy SK, Kumar A, Dhiman R, Sharma K. Polypoidal choroidal vasculopathy-associated vitreous haemorrhage presenting as hyphema. BMJ Case Rep. 2018:11(1).
Inoue M, Balaratnasingam C, Freund KB. Optical coherence tomography angiography of polypoidal choroidal vasculopathy and polypoidal choroidal neovascularization. Retina. 2015;35(11):2265–74.
Tateiwa H, Kuroiwa S, Gaun S, et al. Polypoidal choroidal vasculopathy with large vascular network. Graefes Arch Clin Exp Ophthalmol. 2002;240:354–61.
Yannuzzi LA, Freund KB, Goldbaum M, et al. Polypoidal choroidal vasculopathy masquerading as central serous chorioretinopathy. Ophthalmology. 2000;107:767–77.
Peiretti E, Iovino C, Sacconi R, Caminiti G, Querques G. Optical coherence tomography angiography characteristics of polypoidal choroidal vasculopathy secondary to chronic central serous chorioretinopathy. Retina. 2018;
Moorthy RS, Lyon AT, Rabb MF, et al. Idiopathic polypoidal choroidal vasculopathy of the macula. Ophthalmology. 1998;105:1380–5.
Yannuzzi LA, Nogueira FB, Spaide RF, et al. Idiopathic polypoidal choroidal vasculopathy: a peripheral lesion. Arch Ophthalmol. 1998;116:382–3.
Iijima H, Iida T, Imai M, et al. Optical coherence tomography of orange-red subretinal lesions in eyes with idiopathic polypoidal vasculopathy. Am J Ophthalmol. 2000;129:21–6.
Spaide RF, Guyer DR, McCormick B, et al. External beam radiation therapy for choroidal neovascularization. Ophthalmology. 1998;105:24–30.
Yang SS, Fu AD, McDonald HR, et al. Massive spontaneous choroidal hemorrhage. Retina. 2003;23:139–44.
Smith RE, Wise K, Kingsley RM. Idiopathic polypoidal choroidal vasculopathy and sickle cell retinopathy. Am J Ophthalmol. 2000;129:105–11.
Bartlett HM, Willoughby B, Mandava N. Polypoidal choroidal vasculopathy in a patients with melanocytoma of the optic nerve. Retina. 2001;21:396–9.
Katsimpris JM, Petropoulas IK, Pharmakakis NM, Pournaras CJ. Idiopathic polypoidal choroidal vasculopathy associated with central retinal vein occlusion. J Fr Ophtalmol. 2003;26:489–92.
Yuzawa M, Mori R, Kawamura A. The origins of polypoidal choroidal vasculopathy. Br J Ophthalmol. 2005;89(5):602–7.
Kawamura A, Yuzawa M, Mori R, Haruvama M, Tanaka K. Indocyanine green angiographic and optical coherence tomographic findings support classification of polypoidal choroidal vasculopathy into two types. Acta Ophthalmol. 2013;91(6):e474–81.
Tanaka K, Mori R, Kawamura A, et al. Comparison of OCT angiography and indocyanine green angiographic findings with subtypes of polypoidal choroidal vasculopathy. Br J Ophthalmol. 2017;101(1):51–5.
Iijima H, Imai M, Gohodo T, et al. Optical coherence tomography of idiopathic polypoidal choroidal vasculopathy. Am J Ophthalmol. 1999;127:301–5.
Giovannini A, Amato GP, D’Altobrando E, Giuliani M. Optical coherence tomography (OCT) in idiopathic polypoidal choroidal vasculopathy. Doc Ophthalmol. 1999;97:367–71.
Honda S, Matsumiya W, Negi A. Polypoidal choroidal vasculopathy: clinical features and genetic predisposition. Ophthalmologica. 2014;231(2):59–74.
Breazzano MP, Fernández-Avellaneda P, Freund KB. Swept-source optical coherence tomography angiography of retinal arterial macroaneurysm with overlying hemorrhage. JAMA Ophthalmol. 2019;137(4):e190247.
De Carlo TE, Kokame GT, Kaneko KN, et al. Sensitivity and specificity of detecting polypoidal choroidal vasculopathy with en face optical coherence tomography and optical coherence tomography angiography. Retina. 2019;39(7):1343–52.
Shields CL, Welch RJ, Malik K, et al. Uveal metastasis: clinical features and survival outcome of 2214 tumors in 1111 patients based on primary tumor origin. Middle East Afr J Ophthalmol. 2018;25(2):81–90.
Pauleikhoff D, Radermacher M, Spital G, et al. Visual prognosis of second eyes in patients with unilateral late exudative age-related macular degeneration. Graefes Arch Clin Exp Ophthalmol. 2002;240:539–42.
Eandi CM, Klais CM, Freund KB, Yannuzzi LA. Chapter 151: Idiopathic Polypoidal Choroidal Vasculopathy. In: Albert & Jakobiec’s Principles & practice of ophthalmology. 3rd ed. Philadelphia: Saunders; 2008. p. 1989–96.
Kokame GT, Lai JC, Wee R. Prospective clinical trial of intravitreal aflibercept treatment for polypoidal choroidal vasculopathy with hemorrhage or exudation (EPIC study): 6 month results. BMC Ophthalmol. 2016;16:127.
Takahashi K, Ohji M, Terasaki H, 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.
Lee WK, Iida T, Ogura Y, et al. Efficacy and safety of intravitreal aflibercept for polypoidal choroidal vasculopathy in the PLANET study: a randomized clinical trial. JAMA Ophthalmol. 2018;136(7):786–93.
Nayak S, Padhi TR, Basu S, Das T. Pneumatic displacement and intra-vitreal bevacizumab in management of sub-retinal and sub-retinal pigment epithelial hemorrhage at macula in polypoidal choroidal vasculopathy (PCV): rationale and outcome. Semin Ophthalmol. 2015;30(1):53–5.
Park UC, Shin JY, Chung H, Yu HG. Association of ARMS2 genotype with response to anti-vascular endothelial growth factor treatment in polypoidal choroidal vasculopathy. BMC Ophthalmol. 2017;17(1):241.
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Breazzano, M.P., Fernández-Avellaneda, P., Ledesma-Gil, G., Wang, F., Tso, M.O.M., Yannuzzi, L.A. (2020). Idiopathic Polypoidal Choroidal Vasculopathy. In: Albert, D., Miller, J., Azar, D., Young, L.H. (eds) Albert and Jakobiec's Principles and Practice of Ophthalmology. Springer, Cham. https://doi.org/10.1007/978-3-319-90495-5_113-1
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