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Idiopathic Polypoidal Choroidal Vasculopathy (IPCV) in Thai Population Presenting with Choroidal Neovascularization (CNV) A multicenter study Authors Yonrawee Piyacomn 1, Chavakij Bhoomibunchoo 1, Yosanan Yospaiboon 1, Somanus Thoongsuwan 2, Nattapon Wongcumchang 3, Duangnate Rojanaporn 4, Adisai Varadisai 5, Pichai Jirarattanasopa 6, Eakkachai Arayangkoon 7, Atchara Amphornphruet 8, Nawat Watanachai 9, Sritatath Vongkulsiri 10 1 Department of Ophthalmology, Faculty of Medicine, Khon Kaen University 2 Department of Ophthalmology, Faculty of Medicine Siriraj Hospital, Mahidol University 3 Department of Ophthalmology, Faculty of Medicine, Thammasat University 4 Department of Ophthalmology, Faculty of Medicine Ramathibodi Hospital, Mahidol University 5 Department of Ophthalmology, Faculty of Medicine, Chulalongkorn University 6 Department of Ophthalmology, Faculty of Medicine, Prince of Songkla University 7 Department of Ophthalmology, Mettapracharak Watraikhing Hospital 8 Department of Ophthalmology, Rajavithi Hospital 9 Department of Ophthalmology, Faculty of Medicine, Chiang Mai University 10 Deparment of Ophthalmology, Phramongkutklao College of Medicine Introduction The characteristics of IPCV include 1. Presence of dilated reddish-orange lesion 2. Bulging polypoidal lesions in the macular and peripapillary area 1 Mimic age-related macular degenerations (AMDs) and other diseases characterized by the presence of choroidal neovascularizations (CNVs) 2-5 IPCV are distinctive with the use of indocyanine green angiography (ICGA) and optical coherence tomography (OCT) Introduction To the best knowledge of authors, by far there is no data about prevalence of IPCV and its characteristics in Thailand 1 Idiopathic Polypoidal Choroidal Vasculopathy (Ipcv). Retina 1990. 2 Polypoidal choroidal vasculopathy in exudative and haemorrhagic pigment epithelial detachments. Br J Ophthalmol. 2000. 3 Pigment epithelial detachment in the elderly. Graefe's Arch Clin Exp Ophthalmol 2002. 4 Idiopathic polypoidal choroidal vasculopathy as a cause of a disciform macular scar. Clin Exp Ophthalmol 2003. 5 Polypoidal choroidal vasculopathy masquerading as central serous chorioretinopathy. Ophthalmol 2000. Objective To study Approved by Khon Kaen University Ethics Committee in Human Research University, Thailand (Reference No. HE551279) Prevalence of IPCV Characteristics of IPCV 1

In this multicenter descriptive study, we reviewed retrospectively 140 Medical Records Fundus Photos SD-OCT FFA ICGA 10 referral centers March 2013 December 2013 Ten referral centers all over Thailand 1. Siriraj hospital 2. King Chulalongkorn Memorial hospital 3. Ramathibodi hospital 4. Rajavithi hospital 5. Thammasat university hospital 6. Pramongkut hospital 7. Mettapracharak hospital 8. Songklanagarind hospital 9. Maharaj Nakorn Chiangmai hospital 10. Srinagarind hospital Inclusion criteria 1. Patients with any of these findings :- subretinal or sub-pigment epithelial blood subretinal fluid subretinal lipid subretinal pigment ring irregular elevation of pigment epithelium subretinal gray-white lesion cystoid macular edema sea fan pattern of subretinal small vessels Inclusion criteria 2. Complete ocular history and examination slit-lamp microscopy posterior fundus examination 3. Fundus fluorescein angiography (FFA) 4. Indocyanine green angiography (ICGA) 5. Optical coherence tomography (OCT) Exclusion criteria 1. No history of FFA or ICGA 2. Previously performed with any kinds of ophthalmic laser including focal retinal laser, PDT and transpupillary thermotherapy 3. History of intraocular surgery within 60 days prior to study 2

Exclusion criteria 4. Patients with any of these conditions :- Angioid streak Pathologic myopia Ocular histoplasmosis Rhegmatogenous retinal detachment (RRD) Tractional retinal detachment (TRD) Uncontrolled glaucoma Retinal pigment epithelial tear Macular hole The were diagnosed with IPCV by angiographic findings as follows : 1. hypofluorescent halos around lesion (in the first 6 minutes) 2. polyp-like swelling 3. pulsation (can be observed by using video ICGA) 4. branching vascular networks of inner choroidal vessels 5. submacular hemorrhage 6. late phase staining in the area of earlier hypofluorescent lesion b Submacular hemorrhage 58-year-old man Blurred vision,os 1 month PTA Subretinal fluid Subretinal hemorrhage SD-OCT Subretinal lipid The prevalence = A/B A = The number of diagnosed with IPCV B = The number of that fit in inclusion criteria FFA & ICGA at 2.47 minutes Branching venous network and polyps The 95% confidence interval was calculated 3

Statistics Results Total 140 Gender Odds ratio & p-value Pearson s chi square test Age (IPCV VS AMD) Mean & SD Two-sample T-test Male 73 Female 67 The percentage of in each disease (%) IPCV Total 100 pateints of IPCV IPCV 71.43% Idiopathic CNV 11.43% AMD 8.57% Chronic CSR 4.29% Serous PED 1.43% Macroaneurysm 1.43% RAP 0.71% RPE rip 0.71% Right eye 44 Left eye 49 Bilateral 7 107 eyes of IPCV Gender Age Group Total 100 of IPCV 75 70 Mean age The difference of these two group was 10.68 years Male 56% Female 44% 65 60 55 IPCV 61.40 AMD 72.08 Mean age 95% CI 5.43-15.94 years (p = 0.0001) Odds ratio = 1.72 with a 95% CI of 0.77 to 3.88 The difference between sex in IPCV group was not statistically significant (p = 0.149) Age Mean Min Max SD AMD 61.40 42 81 8.82 IPCV 72.08 52 78 7.23 4

Area of involvement (%) The clinical manifestations of IPCV at presentation (%) 80.00% 70.00% 60.00% 50.00% 40.00% 73.83% Exudative pattern : 12 eyes (11.21%) Serous retinal detachment Serous PED Disciform scar Subretinal fibrin 30.00% 20.00% 10.00% 0.00% Macular area 13.08% Temporal to vascular arcades 9.35% Peripapillary area 3.74% Both in macular and peripapillary area Hemorrhagic pattern : 95 eyes (88.79%) Hemorrhagic PED Subretinal hemorrhage Vitreous hemorrhage Serous RD / PED with adjacent subretinal hemorrhage Angiographic findings of IPCV (%) Race IPCV are common in Asian and African-American population 1-4 Less common in Caucasian 5-7 Branching venous networks 7.48% Polypoidal lesions 8.41% Both Branching venous networks and polypoidal lesions 84.11% 1 Incidence and clinical patterns of polypoidal choroidal vasculopathy in Korean. Japanese Journal of Ophthalmology. 2008. 2 Polypoidal choroidal vasculopathy: incidence, demographic features, and clinical characteristics. Arch Ophthalmol. 2003. 3 Clinical characteristics of polypoidal choroidal vasculopathy in Chinese. Graefes Arch Clin Exp Ophthalmol. 2010. 4 Polypoidal choroidal vasculopathy. Survey of Ophthalmology. 2004. 5 Polypoidal Choroidal Vasculopathy and Neovascularized Age-related Macular Degeneration. Arch Ophthalmol 1999. 6 Polypoidal choroidal vasculopathy in Caucasians. Graefe's Archive for Clinical and Experimental Ophthalmology. 2000. 7 Klinische Charakteristika der idiopathischen polipoiden choroidalen Vaskulopathie. Der Ophthalmologe. 2001. Author (s), Year Number of Ethinic composition Yannuzzi et al., 1999 167 Caucasian (20%), Black (50%) Lafaut et al., 2000 374 Caucasian (100%) 4% Scassellati-Sforzolini et al., 2003 Incidence / prevalence 7.8% 194 Italian (100%) 9.8% Ladas et al., 2004 268 Greek (100%) 8.2% Our study IPCV is the most common disease (71.43%) in who presented with CNV pattern Sho et al., 2003 418 Japanese (100%) 24% Wen et al., 2004 166 Chinese (100%) 22.3% Maruko et al., 2007 289 Japanese (100%) 54.7% Liu et al., 2007 155 Chinese (100%) 24.5% Byeon et al., 2008 321 Korean (100%) 24.6% 5

Age group Previous studies : common in age of 50-65 years 1-4 Our study : same age group (mean age of 61.40 +/-7.23) Gender Previous studies, African-American and Caucasian : more common in women 1-2 Asian : more common in men 3-6 Our study, no statistical difference between sex 1 Polypoidal Choroidal Vasculopathy and Neovascularized Age-related Macular Degeneration. Arch Ophthalmol 1999. 2 Polypoidal choroidal vasculopathy. Ophthalmology Clinics of North America. 2002. 3 Polypoidal choroidal vasculopathy in Chinese. Br J Ophthalmol. 2002. 4 Polypoidal choroidal vasculopathy: incidence, demographic features, and clinical characteristics. Arch Ophthalmol. 2003. 1 Polypoidal Choroidal Vasculopathy: A Review. Surv Ophthalmol 2010. 2 Polypoidal choroidal vasculopathy. Ophthalmology Clinics of North America. 2002. 3 Polypoidal choroidal vasculopathy in Chinese. Br J Ophthalmol. 2002. 4 Incidence and clinical patterns of polypoidal choroidal vasculopathy in Korean. Japanese Journal of Ophthalmology. 2008. 5 Subtype lesions of neovascular age-related macular degeneration in Chinese. Graefes Arch Clin Exp Ophthalmol. 2007. 6 Clinical Characteristics of Exudative Age-related Macular Degeneration in Japanese Patients. American Journal of Ophthalmology. 2007. Area of involvement Race / Location Macular Peripapillary Both macular and peripapillary Italy (Scassellati-Sforzolini et al., 2001) Temporal to vascular arcades Periphery 52.63% 36.84% N/A N/A 10.53% Clinical manifestation Exudative pattern Hemorrhagic pattern RPE degeneration and atrophy Belgium (Lafaut et al., 2000) 48.89% 35.56% N/A 13.33% 13.33% Chinese (Cackett et al., 92% 8% N/A N/A N/A 2009b) Korea (Byeon et al., 2008) 87.8% 5% 7.2% - - Japan (Sho et al., 2003) 85% 7% 3% - - Sho et al., 2003 59% 30% 10% Byeon et al., 2008 52% 34.7%+13.3% = 48% Our study 11.21% 88.79% 0% 0% Our study 73.83% 9.35% 3.74% 13.08% - Limitations Hospital-based data prevalence might be over-estimated number of populations is probably small Conclusion In conclusion, from our multicenter retrospective study, the IPCV was the most common macular disease in Thai population with macular CNV 6

Conclusion Clinical presentation of IPCV in Thai population Age : same as previous Asian studies Gender : no sex predilection Location : same as previous Asian studies Clinical manifestation : Hemorrhagic pattern Thank you 7