Search

To evaluate morphologic alterations in choroidal veins in eyes with typical neovascular age-related macular degeneration (nAMD) and polypoidal choroidal vasculopathy (PCV).A retrospective review of baseline indocyanine green angiography in eyes with typical nAMD and PCV. We evaluated Haller layer veins in the early-phase indocyanine green angiography (before 2 minutes) for 1) macular anastomosis, 2) dilated Haller veins, and 3) focal variation in vessel caliber by at least 50% from the narrowest to largest diameters.We included 70 patients with gradable indocyanine green angiography for the prespecified features in the study eye (36 typical nAMD and 34 PCV) and 59 fellow eyes. The median subfoveal choroidal thickness was 167 µm versus 219 µm, P = 0.08, in the presenting eyes in typical nAMD and PCV, respectively. Macular anastomosis was common in both typical nAMD and PCV (presenting eyes 58.3% vs. 58.8%. P = 0.97; fellow eyes 65.5% vs. 63.3%, P = 0.86). Dilated Haller veins were numerically less common in typical nAMD than PCV (presenting eyes 52.8% vs. 67.6%, P = 0.21; fellow eyes 65.5% vs. 70.0%, P = 0.71), while vascular caliber variation was numerically more common in typical nAMD than PCV (presenting eyes 72.2% vs. 63.8%, P = 0.45; fellow eyes 69.0% vs. 56.7%, P = 0.33). The presence of all three features was more common in the presenting eyes with PCV compared with typical nAMD (35.3% vs. 13.9%, P = 0.03). In a multivariable analysis, every increase of 100 µm of CT conferred a 2.75 risk of having all three features present.Choroidal vascular remodeling is common in both tAMD and PCV but may be driven by different stimuli.

Objectives: To determine the prevalence of polypoidal choroidal vasculopathy (PCV) in intravitreal (IV) anti-vascular endothelial growth factor (anti-VEGF)-resistant neovascular age-related macular degeneration (nvAMD) cases. Materials and Methods: Eyes that were diagnosed as having active and treatment-naive nvAMD in the Ege University Ophthalmology Department, Retina Unit in 2011-2018, were non-responsive to IV anti-VEGF treatment, and for which indocyanine angiography (ICGA) could be obtained were included in the study. Active nvAMD was defined as the presence of fresh hemorrhage on clinical examination or findings of subretinal, intraretinal, or sub-retinal pigment epithelial fluid on spectral domain optical coherence tomography and accompanying fluorescein dye leakage in fluorescein angiography. Eyes that had activation findings despite at least 6 consecutive intravitreal anti-VEGF injections were defined as non-responders and underwent ICGA to assess for PCV. The diagnosis of PCV was based on the Everest II study criterion. Results: A total of 97 eyes of 88 patients were included in the study. Of 88 patients, 44 (50%) were female, 44 (50%) were male, and the mean age was 75.9±8.3 years (range: 59-93). The mean number of anti-VEGF injections until the time of ICGA was 7.3±2.2 (range: 6-15). PCV was detected in 62 eyes (63.9%) on ICGA. Conclusion: The prevalence of PCV is quite high among eyes with IV anti-VEGF treatment-resistant nvAMD in Turkey (63.9%). ICGA evaluation for PCV should be conducted for all nvAMD cases that are non-responsive to IV anti-VEGF treatment, both to shed light on the reason for resistance and to modify treatment as necessary. © 2022 by Turkish Ophthalmological Association Turkish Journal of Ophthalmology, published by Galenos Publishing House.

Introduction To evaluate the effects of combined therapy using intravitreal Aflibercept (IVA) and photodynamic therapy (PDT) on polypoidal choroidal vasculopathy related to pachychoroid disease (PPCV).Methods Patients with PPCV were treated with PDT combined with 3 IVA injections on a monthly basis, followed by pro re nata criteria. The 12-month follow-up consisted of multiple revaluations of visual acuity and SSOCT parameters of clinical activity.Results Nineteen eyes were included in the study; mean age was 65.5 years. Visual acuity improved after 12 months (0.35 ± 0.25 to 0.2 ± 0.20 logMAR, p = 0.005). Percentage of eyes with intraretinal and subretinal fluid reduced from baseline to the 12-month follow-up (from 52.6–10.5%, p = 0.12, and from 89.5–5.3% p = 0.0009, respectively). Central retinal and mean macular thicknesses reduced (258 ± 39.6 to 204.8 ± 38.8 µm p = 0.04 and 293.8 ± 32.1 to 248.1 ± 29.6 µm p = 0.017 respectively). Central choroidal and mean choroidal thicknesses also displayed a reduction (328.6 ± 54.9 to 289.8 ± 44.6 µm p = 0.001 and 314.5 ± 55.3 to 287.9 ± 47.6 µm p = 0.015 respectively). The mean number of injections was 4.6/year.Conclusion The results support the use of a combined therapy with Aflibercept and PDT in PPCV. This treatment would act in synergy, with anti-VEGF controlling exudation and PDT closing the aneurysmal vessel and reducing choroidal congestion.

Introduction To investigate the efficacy and safety of proton-beam irradiation (PBI) combined with intravitreal conbercept (IVC) injection for refractory or recurrent polypoidal choroidal vasculopathy (PCV). Methods A prospective interventional clinical trial included 12 patients with refractory PCV (defined as persistent exudation or fluid after six consecutive injections at monthly intervals and/or photodynamic therapy) or recurrent PCV (defined as new exudative signs after six monthly injections and/or photodynamic therapy) treated between January 2019 and September 2020. Every patient underwent single PBI (14 GyE) with concomitant IVC (0.5 mg) within 1 week and further doses of IVC were administered pro re nata. Results By the 12-month follow-up, the subretinal fluid was completely absorbed in 9 eyes (81.8%). The angiographic regression and closure rates of the polyps were 60% (12/20) and 90% (18/20), respectively. The mean number of IVC injections was 3.1 ± 1.37. The mean BCVA improved by 20 letters (P = 0.006). The mean central macular thickness (CMT) decreased from 476.50 ± 123.63 μm to 317.70 ± 89.34 μm (P = 0.004). The areas of branching vascular networks and polyps decreased by 37.2% and 72.3%, respectively. Radiation retinopathy was observed in five eyes, but no systemic adverse events were observed. Conclusion PBI combined with IVC appears to promote polyp regression and closure, reduce CMT, and improve BCVA, with a favorable safety profile, after 12 months. Therefore, PBI may be a useful adjuvant therapy for patients with refractory or recurrent PCV. Trial registration Proton-Beam Irradiation Combined with Intravitreal Conbercept for Refractory or Recurrent Polypoidal Choroidal Vasculopathy: Prospective Phase II Clinical Study (ChiCTR2000038987).

Purpose To report the efficacy and safety of brolucizumab in the treatment of refractory serous pigment epithelial detachment (PED) secondary to polypoidal choroidal vasculopathy (PCV). Methods Twenty-six eyes of 26 patients were included. Intravitreal brolucizumab 6.0 mg was administered, followed by pro re nata (PRN) retreatment at monthly follow-ups. All patients underwent spectralis domain optical coherence tomography (SD-OCT), fluorescein angiography, and indocyanine green angiography before the first brolucizumab injection. SD-OCT was repeated at follow-up visits. The height and width of the serous PEDs, measured using SD-OCT, were analyzed. Results The number of previous anti-VEGF injections was 12.3 ± 15.0. During brolucizumab treatment, anatomical improvement was achieved and maintained in the height and width of the PEDs (p p > 0.05). A good response was achieved in 69.2% of eyes at 1 month and at the last visit. Relapse and complete resolution were observed in 27.8 and 23.1% of patients, respectively. The number of brolucizumab injections was 2.00 ± 0.85. Intraocular inflammation, vascular obstruction, and retinal pigment epithelial tears were not observed. Conclusion Intravitreal brolucizumab may be an effective and safe treatment option for refractory serous PEDs in patients with PCV.

Objective: To identify retinal abnormalities in the unaffected fellow eyes of patients with unilateral polypoidal choroidal vasculopathy (PCV) and neovascular age-related macular degeneration (n-AMD). Methods: In this cross-sectional, retrospective case series, the medical records of patients with PCV and n-AMD were reviewed and the baseline patient characteristics recorded. Abnormal findings on spectral-domain optical coherence tomography (SD-OCT) (steep/notched pigment epithelial detachment [PED], double-layer sign, hyporeflective lumen within the PED), fundus autofluorescence (FAF) (ring/patch patterns), and indocyanine green angiography (ICGA) (punctate hyperfluorescence spot [PHS]) were studied. Results: Seventy-one fellow eyes of patients with PCV and 64 fellow eyes of patients with n-AMD were included. FAF showed abnormalities in 26 (36.6%) and 33 (51.6%) fellow eyes of those with PCV and n-AMD, respectively (p=0.081). SD-OCT detected abnormalities in 25 (35.2%) and 36 (56.3%) fellow eyes of those with PCV and n-AMD, respectively (p=0.014). ICGA detected PHS in 47 (66.2%) and 34 (53.1%) fellow eyes of PCV and n-AMD, respectively (p=0.122). Conclusion: Multimodal imaging showed abnormalities in most asymptomatic fellow eyes of patients with PCV and n-AMD. Regular and long-term self-monitoring and fundus evaluation are important for these patients. The current findings support the differences in the pathogeneses of PCV and n-AMD.

Purpose To evaluate the one-year efficacy of intravitreal aflibercept injection in polypoidal choroidal vasculopathy (PCV) with best-corrected visual acuity (BCVA) of 20 / 40 or better. Methods This was a multicenter retrospective study. The medical records of patients diagnosed with treatment-naive PCV were retrospectively reviewed. Patients with an initial BCVA of 20 / 40 or better and who had undergone intravitreal aflibercept injection were included. Patients were treated with three consecutive monthly injections, followed by pro re nata regimen according to the clinician's discretion at variable interval visits. The proportions of eyes for which BCVA was maintained (≤ 0.2 logarithm of the minimum angle of resolution change) or improved at 12 months were evaluated. The changes of BCVA, central subfield macular thickness (CSMT), pigment epithelial detachment, and subretinal fluid also were assessed. Results A total of 86 eyes were included. The mean number of injections for 12 months of treatment was 5.4 ± 1.7. BCVA was maintained or improved in 94.2% (81 / 86) of cases. Mean BCVA (logarithm of the minimum angle of resolution) had changed from the baseline (0.23 ± 0.09) at 3 months (0.21 ± 0.14), 6 months (0.24 ± 0.22), and 12 months (0.20 ± 0.18), but with no statistical significance. CSMT had improved significantly from the baseline (336.1 ± 97.3 μm) at 3 months (223.6 ± 47.22 μm), 6 months (239.6 ± 64.2 μm), and 12 months (223.8 ± 47.9 μm). Pigment epithelial detachment was observed in 93% of cases at the baseline, 72.1% at 3 months, and 69.8% at 12 months, showing a significant decrease at all observation points. Subretinal fluid was observed in 91.9% of cases at the baseline, 20.9% at 3 months, and 29.1% at 12 months, showing a significant decrease at all observation points. Conclusions In cases of PCV with good visual acuity, intravitreal aflibercept injections decreased CSMT and were effective in maintaining visual acuity.

Purpose: To evaluate the classification system of branching neovascular network (BNN) morphology in polypoidal choroidal vasculopathy (PCV) patients based on swept-source optical coherence tomography (SS-OCT) and swept-source optical coherence tomography angiography (SS-OCTA), and analyze the morphological features in each group as potential prognostic features. Methods: A total of 32 PCV eyes were included in this retrospective study. SS-OCT and SS-OCTA images of 6 mm × 6 mm centered on the foveal of each eye were analyzed. PCV cases were classified into three types (“trunk”, “glomeruli”, and “stick” type) based on the morphological features of BNN. OCT and OCTA features were compared among the three groups. The correlation of OCT/OCTA features with visual acuity at 12 months after anti-VEGF treatment was also analyzed. Results: Type 1 group had the largest BNN area and the largest numbers of polypoidal lesions. Type 2 group has the largest pigment epithelial detachment (PED) area, PED volume, subretinal fluid (SRF) area, and SRF volume. Type 3 group had better baseline BCVA, the smallest BNN area, the smallest PED size, and the smallest SRF size. Type 1 was also featured by a clear break on Bruch’s membrane which corresponded to the origin of neovascular tissue. BCVA at 12 months was not significantly different among groups. Baseline BCVA and baseline central macular thickness were correlated with the final BCVA. Conclusions: The current classification system based on BNN morphology on SS-OCTA was highly applicable and revealed distinct characteristics in each group. The BNN type was not correlated with BCVA at 12 months after treatment.

The objective of this study was to characterize the choroidal morphology and vasculature in pachychoroid diseases (PCD). A total of 49 eyes with polypoidal choroidal vasculopathy (PCV), 43 eyes with neovascular age-related macular degeneration (nAMD), and 50 eyes with central serous chorioretinopathy (CSC), along with 80 healthy eyes, were enrolled in this nested case-control study. The swept-source optical coherent tomography (OCT), OCT angiography, and En face images were quantitatively analyzed. Multivariate logistic regression models showed that older age and increased vessel density (VD) in the choriocapillaris (CC) layer were independent risk factors for both PCV (page < 0.001, pVD = 0.004), and nAMD (page < 0.001, pVD = 0.005). Decreased VD in the Sattler’s layer was an independent risk factor for PCV (p = 0.014). Increased VD in the Haller’s layer was an independent risk factor for CSC (p = 0.001). The proportion of the diffuse type of collateral circulation in the Sattler’ layer in CSC group was significantly higher than in the other three groups (p < 0.001). We concluded that the involvement of the blood flow in the CC, Haller’s, and Sattler’s layers are differently affected in CSC, nAMD, and PCV eyes, indicating the different pathological mechanism underlying the phenotype of PCD. The age-dependent establishment of collateral circulation in the Sattler’s layer may play a compensatory role regarding ischemic injury in the development of PCD.

Central serous chorioretinopathy (CSCR) can be complicated by different types of choroidal neovascularization (CNV). The purpose of this study was to investigate the incidence and quantitative optical coherence tomography angiography (OCT-A) features of CSCR-related CNVs. Methods: This was a retrospective multicenter study including 102 eyes of 102 Caucasian patients with acute or complex CSCR. All patients underwent a comprehensive ophthalmological examination. Quantitative OCT-A parameters, including vascular perfusion density (VPD), fractal dimension (FD), and lacunarity (LAC), were measured in CNV eyes. Results: Forty eyes (39.2%) had acute CSCR, whereas the remaining sixty-two (60.8%) had complex CSCR. CNV was observed in 37 (36.27%) eyes, all of which had the complex form. CNVs were classified as type 1 CNV in 11/37 (29.73%) cases and as polypoidal choroidal vasculopathy (PCV) in the remaining 26/37 (70.27%). Overall, the mean VPD, FD, and LAC of CSCR-related CNVs were 0.52 ± 0.20%, 1.44 ± 0.12, and 2.40 ± 1.1, respectively. No significant difference between type 1 CNV and PCV was found. Conclusion: Complex CSCR is often complicated by type 1 CNV and PCV with similar neovascular architecture and branching complexity, a finding supporting the idea that they might be different stages of the same neovascular process. Future OCT-A fractal analysis-based studies that also include other relevant parameters, such as demographics, presentation, morphology on multimodal imaging, and response to treatment, are necessary before drawing any definitive conclusions.

Polypoidal choroidal vasculopathy (PCV) is a condition characterized by multiple, recurrent, serosanguineous pigment epithelial detachments, and neurosensory retinal detachments due to abnormal aneurysmal neovascular lesions. It is generally considered as a variant of neovascular age-related macular degeneration, but there are some differences between the clinical presentation, natural history, and treatment response between patients with PCV and typical neovascular age-related macular degeneration patients. Over the past decade, new research and technological advancements have greatly improved our understanding of the PCV disease process and the management of PCV. This review aims to summarize the recent research findings to highlight the epidemiology, pathogenesis, genetics, the application of various diagnostic tools for PCV, and the available treatment options for PCV.

Purpose Genetic studies have identified the association of some single-nucleotide polymorphisms (SNPs) with polypoidal choroidal vasculopathy (PCV), but little is known about whether these SNPs are related to PCV clinical features as well. We performed this study to examine the association of 12 SNPs with PCV clinical phenotypes. Methods Sixty-nine PCV eyes of 69 patients were included. Genomic DNA was extracted from peripheral blood. Agilent SureSelect Human ALL Exon V6 was used to sequence the 12 SNPs previously reported to associate with PCV. Baseline best-corrected visual acuity (BCVA), sub-foveal choroidal thickness (SFCT), choroid maximum vascular diameter (MVD), choroidal vascular hyperpermeability (CVH), and greatest linear dimension (GLD) of entire lesion were measured and compared between patients of different genotypes. Fisher’s exact test and Mann-Whitney U test were mainly used to compare categorical variables and continuous variables respectively. Results HTRA1 rs2293870 was a protective factor of PCV or AMD in the fellow eye (P = 0.040) and was related with greater SFCT in PCV eye after multiple linear regression (P = 0.043). C3 rs17030 was associated with smaller GLD (P = 0.033). CFH rs2274700 was related to lower MVD (P = 0.043) and was a protective factor for CVH (P = 0.034). Conclusion Multiple PCV-associated SNPs are associated with PCV clinical phenotypes. The involvement of several synonymous SNPs calls for further research on the role of transcriptional alterations and trans-regulation of distant signaling pathways in PCV pathogenesis.

AIM: To compare the qualitative and quantitative features among untreated polypoidal choroidal vasculopathy (PCV), neovascular age-related macular degeneration (nv-AMD) and central serous chorioretinopathy (CSC) using optical coherence tomography (OCT) and OCT angiography (OCTA). METHODS: This retrospective study included 16 eyes with thin-choroid PCV, 18 eyes with thick-choroid PCV, 16 eyes with nv-AMD and 17 eyes with CSC, respectively. The indicators were obtained by OCT and OCTA. RESULTS: Sub-foveal choroidal thickness (SFCT) in CSC was thicker compared to other groups (all P

PurposeTo compare the short-term efficacy in patients with polypoidal choroidal vasculopathy (PCV) treated using either aflibercept or conbercept.MethodsThis prospective study included 41 patients with treatment-naive PCV (42 eyes). All the patients were treated with either aflibercept or conbercept using an initial series of 3 monthly loading injections. Changes in the best-corrected logMAR visual acuity (BCVA) and anatomic outcomes were evaluated at 3 months.ResultsBCVA was improved with reduction in central choroidal thickness (CCT), central foveal thickness (CFT), and subretinal fluid (SRF) after 3 monthly loading injections in both aflibercept (IVA) and conbercept (IVC) groups. There was no significant difference in either visual or anatomic outcomes between the two groups after 3 months of treatment. However, compared with the IVC group, significantly higher BCVA improvement was observed in the patients in the IVA group with baseline BCVA better than 1. A visual outcome improved ≥3 lines in 13 patients in the IVA group (59%), and 9 patients in the IVC group (45%). A relatively high proportion of polyp regression was observed in the IVA group (63%) compared with the IVC group (55%) via OCTA.ConclusionsVisual and anatomic outcomes were significantly improved in both IVA and IVC groups, but the results suggest a potentially superior short-term response in the IVA group.

Purpose: Review and provide consensus recommendations on use of treat-and-extend (T&E) regimens for neovascular age-related macular degeneration (nAMD) and polypoidal choroidal vasculopathy (PCV) management with relevance for clinicians in the Asia-Pacific region. Methods: A systematic search of MEDLINE, EMBASE, and Cochrane databases, and abstract databases of the Asia-Pacific Vitreo-retina Society, European Society of Retina Specialists, American Academy of Ophthalmology, and Controversies in Ophthalmology: Asia-Australia congresses, was conducted to assess evidence for T&E regimens in nAMD. Only studies with ≥100 study eyes were included. An expert panel reviewed the results and key factors potentially influencing the use of T&E regimens in nAMD and PCV, and subsequently formed consensus recommendations for their application in the Asia-Pacific region. Results: Twenty-seven studies were included. Studies demonstrated that T&E regimens with aflibercept, ranibizumab, or bevacizumab in nAMD, and with aflibercept in PCV, were efficacious and safe. The recommendation for T&E is, after ≥3 consecutive monthly loading doses, treatment intervals can be extended by 2 to 4 weeks up to 12 to 16 weeks. When disease activity recurs, the recommendation is to reinject and shorten intervals by 2 to 4 weeks until fluid resolution, after which treatment intervals can again be extended. Intraretinal fluid should be treated until resolved; however, persistent minimal subretinal fluid after consecutive treatments may be tolerated with treatment intervals maintained or extended if the clinical condition is stable. Conclusions: T&E regimens are efficacious and safe for nAMD and PCV, can reduce the number of visits, and minimize the overall burden for clinicians and patients.

PurposeTo compare the efficacy of initial vs. delayed photodynamic therapy (PDT) in combination with intravitreal injection of conbercept (IVC) for polypoidal choroidal vasculopathy (PCV).DesignMulticenter, randomized, non-inferiority clinical trial.SubjectsNaïve PCV patients.MethodsPatients were randomized 1:1 into two groups: initial PDT with IVC and delayed PDT with IVC. At baseline, patients in the initial combination group were treated with PDT and IVC within 1 week, while patients in the delayed combination group were treated with IVC alone. PDT and IVC was given PRN during the follow-up in each group.Main Outcome MeasuresNon-inferiority of delayed PDT with IVC to initial PDT with IVC for mean change in best-corrected visual acuity from baseline to month 12 (95% CI of the difference entirely above −5 letters).ResultsEighty-six patients were enrolled, with 43 in each group. At month 12, the change of BCVA in initial combination group was equivalent to that in the delayed combination group, with gains of 6.42 ± 1.89 and 7.49 ± 2.14 (mean ± standard error) letters, respectively [delayed group minus initial group: 1.07 letters; 95% confidence interval (CI): −4.62 to 6.76; Pnon−inferiority = 0.0198]. The rates of complete polyp regression were 66.67 and 45.83% in the initial and delayed combination groups, respectively. The difference was not statistically significant (P = 0.386). The mean reductions of CRT were 204.77 ± 28.79 and 84.14 ± 30.62 μm in each group respectively. The difference was statistically significant (P = 0.005). In addition, the mean injection numbers were 3.47 ± 2.39 and 4.91 ± 2.65 in each group respectively. The differences were statistically significant (P = 0.010).ConclusionsThere was effective in both groups in patients with PCV. The initial combination group showed a more efficient decrease in CRT and polyp regression, along with fewer injections. However, the delayed combination group was non-inferior compared with the initial combination group in terms of the improvement of BCVA.Trial Registrationhttps://ClinicalTrials.gov, Identifier: NCT02821520.

Background Regular membrane peeling vitrectomy for epiretinal membrane (ERM) patients seldom causes large pigment epithelial detachment (PED). We presented an unusual case of the activation of quiescent polypoidal choroidal vasculopathy (PCV) after membrane peeling vitrectomy for ERM, with an uneven therapeutic process. Case presentation A 75-year-old female patient complained of metamorphopsia in her left eye for 2 years. Her best-corrected visual acuity was 20/160 with a moderate nuclear cataract. An irregular ERM and slight PED were shown in optical coherence tomography (OCT). No obvious orange-red lesion was detected. The patient underwent vitrectomy + ERM peeling + cataract surgery. After the operation, large PED emerged, and indocyanine green angiography (ICGA) confirmed PCV. Four monthly injections of intravitreal ranibizumab were administered, but PED persisted. After focal laser therapy targeted to the polyps combined with ranibizumab treatment, PED was absorbed. Conclusions Careful evaluation for PCV before membrane peeling vitrectomy for ERM is important, as indolent PCV may be activated postoperatively. Anti-VEGF therapy accompanied by laser photocoagulation may be more effective for PCV polyps located away from the fovea.

Background We report a case of bilateral pachychoroid disease manifesting polypoidal choroidal vasculopathy (PCV) with punctate hyperfluorescent spot (PHS) in one eye, and peripheral exudative hemorrhagic choroidal retinopathy (PEHCR) with central serous chorioretinopathy (CSC) and PHS in the contralateral eye. Case presentation : A 51-year-old healthy woman presented with complaint of blurred vision in her right eye. Corrected visual acuity was 20/20 in the right and 24/20 in the left eye. Fundus examination was normal in the left eye. In the right eye, fundus finding of an orange-red nodular lesion and optical coherence tomography (OCT) finding of polypoidal lesions led to a diagnosis of PCV. Four aflibercept intravitreal injections were performed in her right eye. After treatment, indocyanine green angiography (ICGA) confirmed residual polypoidal lesions with branching vascular networks and PHS with choroidal vascular hyperpermeability. OCT showed PHS associated with small sharp-peaked retinal pigment epithelium (RPE) elevation in peripheral fundus and small RPE elevation in posterior fundus. Based on the above findings, PCV with PHS was finally diagnosed in the right eye. Posttreatment corrected visual acuity in the right eye was 20/20. She presented again 32 months later, with complaint of vision loss in her left eye. Left corrected visual acuity was 20/20, and fundus examination showed mild vitreous hemorrhage. Vitrectomy was performed. In temporal midperipheral fundus, fluorescein angiography revealed CSC, and OCT showed pachychoroid. ICGA depicted abnormal choroidal networks and PHS in peripheral fundus. Furthermore, polypoidal lesions were confirmed by OCT. Based on the above findings, PEHCR and CSC with PHS was finally diagnosed in the left eye. Postoperative corrected visual acuity in the left eye was 20/20, and aflibercept intravitreal injection was performed for prevention of recurrence of vitreous hemorrhage. Conclusions This is the first case report of PCV with PHS in one eye, and PEHCR with CSC and PHS in the contralateral eye. This case suggests that PCV, PEHCR, and CSC may be linked pathologies of pachychoroid spectrum disease.

Purpose: To evaluate the status and evolution of polypoidal lesions during the course of treatment of patients with symptomatic macular polypoidal choroidal vasculopathy (PCV). Design: Comparative cohort study of randomly selected patients from a multicenter, randomized controlled clinical trial. Participants: Thirty randomly selected patients from the EVEREST II study who were treated with combination ranibizumab and verteporfin photodynamic therapy (n = 15) or ranibizumab monotherapy (n = 15). Methods: All patients were randomized at baseline and treated with a standardized treatment protocol. Indocyanine green angiography (ICGA) images were graded at the central reading center at baseline and months 3, 6, 12, and 24. Polypoidal lesions present at baseline were overlaid on ICGA images at subsequent visits to determine if these remained perfused or had regressed completely. New polypoidal lesions occurring at subsequent visits were similarly tracked to detail the evolution of each polypoidal lesion. Main Outcome Measures: Complete polypoidal lesion regression over time. Results: Complete polypoidal lesion regression was higher in the combination therapy group compared with the monotherapy group at all visits (month 12, 12 of 15 patients [80%] vs. 5 of 14 patients [35.7%]; P = 0.016). Persistence of baseline polypoidal lesions was lower in the combination therapy group: 1 of 15 patients (6.7%) versus 7 of 14 patients (50%) in the monotherapy group at month 12. Recurrences of polypoidal lesions that had regressed completely at an earlier time point were uncommon: 0% in the combination therapy group and 1 patient each at months 6 and 12 in the monotherapy group. Fewer new polypoidal lesions (arising after the baseline visit) were found in the combination therapy group at all visits (combination therapy: 2 of 15 [13.3%] vs. monotherapy: 4 of 14 eyes [28.6%] at month 12). Total polypoidal lesion area was significantly smaller in the combination therapy group compared with the monotherapy group throughout the study (0.013 mm2 vs. 0.110 mm2; P < 0.01 at month 12). Conclusions: Combination therapy was associated with higher rates of complete polypoidal lesion regression and fewer persistent polypoidal lesions compared with monotherapy. Closed polypoidal lesions rarely reopened, regardless of the treatment.

Purpose: The present study aimed to quantify various factors of vessel morphology, including vessel diameter, length, and complexity (fractal dimension and lacunarity) of both choroidal neovascularization (CNV) and en face Haller vessels using optical coherence tomography angiography (OCTA) and en face structural optical coherence tomography in typical neovascular age-related macular degeneration (nAMD) and polypoidal choroidal vasculopathy (PCV) and to identify factors associated with visual acuity (VA) loss and number of injections within a year after the day of OCTA.Methods: We retrospectively analyzed 43 eyes of nAMD patients and 33 eyes of PCV patients whose OCTA was performed at least 12 months after an initial anti-vascular endothelial growth factor treatment. Quantitative parameters, including vessel area, vessel diameter, vessel length, fractal dimension, and lacunarity were analyzed from en face images of CNV and Haller vessels. Clinical information, including logarithm of the minimum angle of resolution visual acuity and injection number of anti-vascular endothelial growth factor were acquired after 12 months from OCTA date. Using logistic regression analyses, parameters associated with logarithm of the minimum angle of resolution VA loss of 0.2 or more (VA loss group) and a number of injections of four or more (unstable group) after 12 months were analyzed.Results: In typical nAMD, the VA loss group was associated with a smaller number of intersections of Haller vessels. The unstable group was associated with an increased lacunarity of CNV in typical nAMD. In PCV, both VA loss and unstable groups were associated with a higher maximal diameter of Haller vessels.Conclusions: VA loss and injection number of nAMD and PCV 12 months after OCTA imaging were associated with different morphological parameters of CNV and Haller vessels. Therefore, quantitative analyses of both CNV and Haller vessels from OCTA and en face optical coherence tomography might provide prognostic information about visual outcome and injection frequency within 12 months after OCTA imaging.

Purpose To evaluate the areas of lesion components of polypoidal choroidal vasculopathy (PCV) measured using multicolor imaging compared to indocyanine green angiography (ICGA). Methods In a prospective study of 50 consecutive treatment-naive PCV patients, multicolor imaging and ICGA were performed. The images were independently graded by reading center-certified retinal specialists to confirm the diagnosis of PCV and identify lesion components. The areas of the respective lesion components were compared. Results The mean age of the participants was 67.8 years. PCV was diagnosed in 96% of eyes using multicolor imaging. The mean numbers of polypoidal lesions identified using ICGA and multicolor were 4.0 and 2.1, respectively (P < 0.001), with mean total polypoidal lesion areas of 0.32 mm2 versus 0.30 mm2 (P = 0.727). The area of the branching vascular network (BVN) on ICGA was 7.8 mm2 compared to 5.7 mm2 on multicolor imaging (P = 0.289). Patients with four or more polypoidal lesions on ICGA had larger differences in total lesion area between ICGA and multicolor imaging (4.07 vs. -0.70 mm2, p = 0.039). Those with total lesion area ≥ 2.0 mm2 on ICGA had larger differences in mean polypoidal lesion number compared to those with smaller areas (2.2 vs. 0.5; P = 0.026). Conclusions Multicolor imaging is a useful, noninvasive adjunct for detecting PCV lesion components, revealing lesion areas similar to but generally smaller than those seen on ICGA. This is important to consider when making treatment decisions with different imaging modalities. Translational Relevance New features seen on multicolor imaging can aid in the diagnosis and treatment of PCV.

We experienced a case of sympathetic ophthalmia in a fellow eye after vitrectomy for subretinal hemorrhage related to polypoidal choroidal vasculopathy. A 60-year-old male consulted us for polypoidal choroidal vasculopathy with subretinal hemorrhage in his left eye. The recurrence of massive subretinal hemorrhage refractory to repeated pars plana vitrectomies leads to phthisis bulbi. Two months later, multiple serous retinal detachments were observed in his right eye. Positive human leukocyte antigen-DR4 and the uveitis were helpful in distinguishing between sympathetic ophthalmia and age-related macular degeneration. High-dose pulse intravenous steroid contributed to recovery of visual acuity after resolution of serous retinal detachment.

Mansing Ratanasukon, Patama Bhurayanontachai, Pichai Jirarattanasopa Department of Ophthalmology, Faculty of Medicine, Prince of Songkla University, Hat Yai, Songkhla Province 90110, ThailandPurpose: The purpose of this article was to study the real-life treatment results of polypoidal choroidal vasculopathy (PCV).Design: This was a retrospective study.Methods: Patients with presumed age-related macular degeneration were reviewed, and PCV diagnosis was made using the EVEREST study criteria. Outcomes were changes in visual acuity (VA) and central retinal thickness, time between treatments, follow-up time, and number of treatments.Results: The prevalence of PCV was 30.8%. At the beginning, 195 eyes received monotherapy of anti-vascular endothelial growth factor (anti-VEGF) injections, either bevacizumab or ranibizumab, and only six eyes received the combination of anti-VEGF injection and photodynamic therapy (PDT) at the time of the first treatment. During the follow-up, some patients received “rescue or add-on” PDT when they had a poor response after anti-VEGF injections. After 4 years, the average number of injections was 8.25 and 9.15 for the anti-VEGF monotherapy and the combination groups, respectively. The average time between the first anti-VEGF injections and the first PDT was 21.4 months. The average VA in the anti-VEGF monotherapy group increased by 1.5 letters, whereas it decreased by 0.95 letters in the combination group (P=0.48).Conclusion: The review demonstrated the same visual outcomes between the combination therapy of anti-VEGF injections and rescue or add-on PDT vs monotherapy anti-VEGF injections in PCV treatment. When compared with EVEREST II and Planet studies, the “initial” or “rescue or add-on PDT” might have different effects on the final visual outcomes.Keywords: polypoidal choroidal vasculopathy, anti-vascular endothelial growth factor, photodynamic therapy

Min Sagong,1 Se Joon Woo,2 Youkyung Lee3 On behalf of the LUMINOUS™ study investigators from South Korea1Department of Ophthalmology, Yeungnam University College of Medicine, Daegu, Korea; 2Department of Ophthalmology, Seoul National University Bundang Hospital, Seongnam, Korea; 3Novartis Korea Ltd., Seoul, KoreaCorrespondence: Se Joon WooDepartment of Ophthalmology, Seoul National University Bundang Hospital, 173– 82 Gumi-ro, Bundang-gu, Seongnam-si, Gyeonggi-do, 13620, South KoreaTel +82-31-787-7377Fax +82-31-787-4057Email sejoon1@snu.ac.krPurpose: To evaluate the real-world effectiveness, treatment patterns, and safety of ranibizumab in Korean patients with neovascular age-related macular degeneration (nAMD).Methods: LUMINOUS™ is a 5-year, global, prospective, observational, open-label study. Adults aged ≥ 18 years who were either treatment-naïve or prior-treated were enrolled and treated with ranibizumab 0.5 mg as per the local label. Outcome measures included mean (± standard deviation [SD]) changes from baseline in visual acuity (VA) and central retinal thickness (CRT), and rate of ocular and non-ocular adverse events (AEs).Results: Overall, 367 Korean patients with nAMD (152 treatment-naïve and 215 prior-treated) were enrolled. The mean (SD) VA changes from baseline at 1-year were +10.1 (± 21.77; P=0.0005) and +1.4 (± 15.17; P=0.2142) Early Treatment Diabetic Retinopathy Study letters, with mean numbers of injections of 5.2 and 3.4 in the treatment-naïve and prior-treated groups, respectively. VA gains were greater in patients with lower baseline VA, who received a loading dose, and with polypoidal choroidal vasculopathy (PCV). Multivariate logistic regression analyses demonstrated younger age, worse baseline VA, and those who received loading dose being associated with higher odds of any gain in VA at 1 year (P< 0.05). Mean (SD) CRT changes from baseline were – 126.7 (± 174.90) μm (P< 0.0001) and +10.8 (± 89.62) μm (P=0.5833) in the treatment-naïve and prior-treated groups, respectively, with greater reductions observed in patients with PCV. Ocular and non-ocular AEs were reported in 8.4% (n=31) and 10.1% (n=37) of patients, respectively.Conclusion: The LUMINOUS study confirms real-world effectiveness and safety of ranibizumab in Korean patients with nAMD; factors including age, baseline VA, and loading-dose were associated with VA gain at one-year post-treatment.Keywords: neovascular age-related macular degeneration, polypoidal choroidal vasculopathy, ranibizumab, real-world

The objective of this study was to compare visual parameters and retinal layers’ morphology pre-treatment (baseline) and 6 months post-treatment in polypoidal choroidal vasculopathy (PCV) eyes. A single centre, longitudinal, prospective study was conducted at a public tertiary hospital of Malaysia. Visual parameters including distance and near visual acuity (DVA and NVA), contrast sensitivity (CS), reading speed (RS), and different qualitative and quantitative optical coherence tomography (OCT) parameters were evaluated pre- and 6 months post-treatment. Thirty-three naïve PCV eyes of 32 patients (mean age of 67.62 years) were evaluated pre- and post-treatment of intravitreal ranibizumab with and without photodynamic therapy. After treatment, sub retinal fluid decreased from 27 eyes (84.35%) at baseline to 7 eyes (21.88%) at 6 months while pigment epithelium detachment decreased from 32 eyes (100%) at base line to 15 eyes (46.87%) at 6 months. Mean pre-treatment quantitative morphological OCT retinal parameters including thickness and volume of central sub field, center thickness, center minimum, and maximum thickness reduced significantly. Similarly, all visual parameters including DVA, NVA, CS, and RS showed statistically significant improvement. While 89% of the eyes showed improvement in CS, 78%, 71%, and 65% of the eyes showed improvement in NVA, RS, and DVA, respectively. Thus, CS was the most treatment responsive visual parameter.

PURPOSE To evaluate the efficacy and safety of monthly and pro re nata (PRN, guided by visual acuity stabilization and disease activity criteria) ranibizumab regimens in Chinese patients with neovascular age-related macular degeneration (nAMD) and polypoidal choroidal vasculopathy (PCV). METHODS This double-masked study randomized nAMD patients (1:1) to ranibizumab monthly from baseline to Month (M) 11 to a PRN regimen from M12 to M23 (monthly group, n = 167) versus ranibizumab three monthly doses followed by a PRN regimen up to M23 (PRN group, n = 166). Subgroups were assessed based on the presence/absence of PCV (indicated by indocyanine green angiography). RESULTS Of 334 randomized patients, 41.7% had PCV at baseline. Mean average best-corrected visual acuity (BCVA) change from M3 to M4 through M12 was 3.3 letters with monthly and 1.7 letters with PRN (mean difference: 1.6; 95% CI: -2.95, -0.20, primary end-point). Mean change in BCVA from baseline (monthly/PRN, 53.8/53.7) to M12 and M24 was 12.3 and 11.3 letters in monthly and 9.6 and 9.3 letters in PRN group. Corresponding values for patients with PCV/without PCV were 12.7/12.1 letters (M12) and 12.3/10.6 letters (M24) in monthly and 9.4/9.4 letters (M12) and 9.7/8.7 letters (M24) in PRN groups. The mean number of injections was 11.4 (monthly) and 8.2 (PRN) from Day 1 to M11 and 4.8 (monthly) and 5.0 (PRN) from M12 to M23. No new safety findings were reported. CONCLUSIONS The study results support the use of either ranibizumab monthly or PRN regimens in Chinese patients with nAMD, regardless of presence of PCV.

Purpose To describe the clinical characteristics and outcome of polypoidal choroidal vasculopathy (PCV), also known as aneurysmal type 1 (sub-retinal pigment epithelium (RPE)) neovascularization, in Caucasian patients. Methods Single-centre study in 66 Caucasian patients with a diagnosis of PCV based on optical coherence tomography scan and indocyanine green angiography. Clinical characteristics and multimodal imaging were collected and assessed by an experienced retina specialist. Results This study involved 74 eyes of 66 patients with PCV, with a mean age at onset of 73 years and a female preponderance of 66%. The mean number of polypoidal lesions per eye was 1 (range: 1-5 lesions), out of which 75% was located in the macula and 19% in the peripapillary region. Of the 74 eyes, 37 eyes (50%) had PCV associated with a drusenoidal neovascular age-related macular degeneration (AMD) phenotype (PCV-AMD) and 18 eyes (24%) had PCV associated with non-polypoidal type 1 choroidal neovascularization/branching vascular network (PCV-BVN) without signs of drusenoidal AMD, while 19 eyes (26%) had idiopathic, isolated PCV (iPCV). The mean subfoveal choroidal thickness measured in 22 patients was 245 mu m (range: 71-420 mu m). In 51% of patients, the initially performed therapy showed good anatomical recovery (resolution of intra- and subretinal fluid). Conclusions A spectrum of PCV (aneurysmal type 1/sub-RPE neovascularization) can be seen in Caucasian patients. PCV associated with a drusenoidal neovascular AMD phenotype in Caucasians is phenotypically and presumably pathophysiologically more associated with neovascular AMD (PCV-AMD: type A PCV). However, this may not be the case for patients with PCV with non-polypoidal type 1 choroidal neovascularization or BVN and no signs of drusenoidal AMD (PCV-BVN: type B PCV), and for patients with idiopathic PCV without associated drusen or BVN (iPCV; type C PCV). Most patients have a thin choroid, even when drusen are absent. For the entire patient group, a moderate anatomical recovery was observed after treatment.

Introduction: Choroidal osteoma (CO) is a rare benign tumor that particularly affects young, healthy women. Its prognosis is influenced by complications, such as choroidal neovascularization (CNV), subretinal hemorrhage, subretinal fluid (SF), decalcification status, and overlying retinal pigment epithelium (RPE) atrophy. In case of CNV as the complication of CO, it is typically present in the classic form; however, reports on polypoidal choroidal vasculopathy (PCV) have been rare. Here, we report a case of an older, male patient with PCV as a complication of CO. Patient concerns: A 70-year-old male patient visited the hospital with vision impairment in the right eye since 2 weeks. Diagnosis: Fundus examination revealed a red-yellow, well-demarcated, scalloped lesion around the optic nerve in each eye; the lesions were highly reflective on ultrasound examination, and thus, CO was diagnosed. Indocyanine green fluorescence angiography and optical coherence tomography (OCT) revealed that the right eye also had PCV accompanied with SF. OCT confirmed the presence of large quiescent type 1 CNV bilaterally in decalcified areas of the lesions adjacent to the optic nerve. Interventions: Intravitreal bevacizumab (IB) injection was performed. Outcomes: Best-corrected visual acuity had improved and OCT showed a decrease in the SF, while OCT angiography showed partial regression of branching vascular network. Conclusion: CO can be accompanied by quiescent type 1 CNV; this should be closely monitored because it can progress to PCV. Optical coherence tomography, alongside indocyanine green fluorescence angiography, is useful for the diagnosis and monitoring of potential CNV as a complication of CO.

Background To evaluate the three-year outcome after intravitreal aflibercept injection (IAI) for neovascular age-related macular degeneration (nAMD). Methods Forty-nine treatment-naïve nAMD patients (50 eyes) were enrolled in this prospective study. The eyes received IAI at two-month intervals in the first year. The treatment regimen was changed to IAI based on a treat-and-extend approach in the second and third years. Results Twenty-nine eyes of 28 patients were successfully followed up over 36 months. The nAMD subtypes included 15 eyes with typical AMD and 14 eyes with polypoidal choroidal vasculopathy. The number of IAIs performed over the 3 years was 17.2 ± 3.1 (mean ± standard deviation). The mean logMAR, which was 0.42 at baseline, improved to 0.19 (P = 0.001) at 12 months, and 0.26 (P = 0.049) at 36 months. The central retinal thickness (CRT) was 329 ± 120 μm at baseline, 151 ± 38 μm (P P P P Conclusion Long-term aflibercept injection can achieve visual improvement and reduce the thickness of the retina and choroid in nAMD. Morphological improvement of these tissues may not be sufficient to sustain earlier visual improvement over the long-term.

Optical coherence tomography angiography (OCT-A) is a non-invasive, non-dye-based imaging modality that has the potential to enhance our understanding of retinal diseases. While this rapidly advancing imaging modality offers great potential, there is a need for community-wide understanding of the range of technologies and methods for interpreting the images, as well as a need to enhance understanding of images from disease-free eyes for reference when screening for retinal diseases. Importantly, clinical trials have been designed without OCT-A-based endpoints; therefore, caution is required when making treatment decisions based on OCT-A imaging alone. With this in mind, a full understanding of the advantages and limitations of OCT-A will be vital for effective development of the technique within the field of ophthalmology. On behalf of the Vision Academy Steering Committee (sponsored by Bayer), this publication summarizes the views of the authors on the current use of OCT-A imaging and explores its potential for future applications in research and clinical practice. © 2018, The Author(s).

Purpose: To describe the histopathologic features of an early case of presumably bilateral polypoidal choroidal vasculopathy (PCV) in two eyes obtained at autopsy from a patient with no prior ocular therapy. Observations: The choroid of both eyes at the macular and peripapillary regions was greatly thickened with dilated, thin walled choroidal venules intertwining with arteriosclerotic arterioles in the Sattler's layer of the choroidal vasculature. At the temporal and nasal equatorial regions of both eyes many of these congested venular channels abruptly disappeared and were replaced by loose connective tissue with loss of the normal choroidal stromal tissue and uveal melanocytes. A few remaining venules showed intraluminal sloughing of endothelial cells and deposition of fibrinous material networks suggesting occlusion of these choroidal venules. At this equatorial location, serous detachment of retinal pigment epithelium (RPE) appeared and a thin neovascular membrane with cords of endothelial cell invaded into the sub-RPE space. Anteriorly, the neovascular membrane expanded and bulged into the sub-retinal space with dilated neovascular capillaries in a “grape like” or polypoidal configuration. Conclusion and importance: Polypoidal Choroidal Vasculopathy is a disease of the dilated and multi-layered choroidal venules. Occlusion of these choroidal vascular channels might give rise to choroidal stasis and ischemia leading to serous RPE detachment and a sub-RPE neovascular membrane. Gross dilatation of the choroidal venules and capillaries in the sub-RPE neovascular membrane leads to the characteristic “grape like” structures, a unique clinical feature in this disease entity. These pathologic features of PCV are different from the pathologic changes of neovascular age-related macular degeneration (nAMD). Consequently, PCV and nAMD are two distinct diseases. However, in the late stage of both entities, choroidal ischemia in both diseases, lead to sub-RPE neovascularization and subsequent sub-RPE and/or sub-retinal hemorrhage. These results in both entities showed comparable clinical and pathologic features that are frequently mistaken PCV as a sub-type of Neovascular AMD. Keywords: Polypoidal choroidal vasculopathy, Neovascular age-related macular degeneration, Choroidal venule, Choroidal neo-vascularization, Venous stasis choroidopathy

Purpose: To explore novel Optical Coherence Tomography (OCT) biomarkers and precursor lesions in Polypoidal Choroidal Vasculopathy (PCV). Methods: This retrospective cohort study included 76 treatment naïve fellow eyes of PCV. Focus was given to analyse the various morphological changes in the clinically unaffected fellow retina during the follow-up period. Results: 11 fellow eyes (14.47%) developed disease activity in the form of Sub Retinal Fluid (SRF) or Intra Retinal Fluid (IRF) within a mean follow-up of 17 months. All 11 eyes (100%) showed the presence of flat irregular pigment epithelial detachment (FIPED) and a peculiar property of lateral elongation of FIPED during disease activity. A positive correlation with the disease progression was found for the same (P < 0.0001). The mean horizontal dimension of the flat irregular PED at the enrolment was 1984 ± 376u and the mean expansion of FIPED at SRF formation was 461 ± 152u. ICG taken at the time of disease activity in the fellow eye revealed branching vascular network (BVN) in 9 (81.8%) eyes, polyps in 7 (63.6%) eyes, a combination of both in 5 (45.4%) eyes. Type one BVN with interconnecting channels showed faster disease progression than type two BVN. Eye tracking ICG illustrated that BVN corresponded to the FIPED in OCT and polypoidal lesions developed at the end of expanding FIPED. Conclusion: Flat irregular pigment epithelial detachment with its characteristic property of lateral elongation may be considered as a precursor lesion for PCV and as a novel OCT biomarker for the disease activity. Fellow eyes with FIPED need close monitoring to identify development of disease activity at the earliest.

Background The concentrations of cytokines in the aqueous humor from neovascular age-related macular degeneration (nAMD) and polypoidal choroidal vasculopathy (PCV) may vary. The study was conducted to compare various cytokine levels in the aqueous humor of eyes with PCV, nAMD and control.Methods The present case control study included 49 treatment-naïve eyes from 49 patients (PCV 24, nAMD 11, and cataract 14 eyes). Totally 34 angiogenic and inflammatory cytokines in the aqueous humor were measured by Luminex bead-based multiplex array.Results The aqueous humor levels of IL-8 and IL-12p70 in the nAMD group were significantly higher than the PCV group (p=0.031, p=0.012, respectively). The levels of IL-8, IL-18, IL-21, IL-31, LIF, SDF1-α, FGF-basic, VEGF-A, and VEGF-D in the aqueous humor were significantly higher in the nAMD group and PCV group than the control (nAMD vs control, p=0.004, 0.002, 0.005

BackgroundNeovascular age-related macular degeneration (AMD) and polypoidal choroidal vasculopathy (PCV) are sight-threatening maculopathies with both environmental and genetic risk factors. We have previously shown relative risks posed by genes of the complement pathways to neovascular AMD and PCV.MethodsIn this study, we investigated the haplotype-tagging single nucleotide polymorphisms (SNPs) in thecomplement component 5(C5) gene in 708 unrelated Chinese individuals: 200 neovascular AMD patients, 233 PCV patients and 275 controls. Six tagging SNPs inC5were genotyped. Univariate single SNP association analysis, haplotype-based association analysis and gene-gene interaction analysis betweenC5and other AMD-associated genes were performed.ResultsThe results revealed none of the six tagging SNPs of theC5gene had a significant association with neovascular AMD or PCV (P > 0.05). We also found insignificant haplotype-based association, and no significant SNP-SNP interaction betweenC5and other genes (includingC2-CFB-RDBP-SKIV2L,SERPING1,CETP,ABCG1,PGF,ANGPT2,CFHandHTRA1) for neovascular AMD and PCV.ConclusionsThis study showed no statistical significance in the genetic association ofC5with neovascular AMD or PCV in a Hong Kong Chinese population. Further studies in large samples from different populations are warranted to elucidate the role ofC5in the genetic susceptibility of AMD and PCV.

Background Polypoidal choroidal vasculopathy (PCV) is one of the disorders within the pachychoroid spectrum diseases. The presence of pachyvessels is one of the characteristics of pachychoroid disorders. However, the relationship between the presence of pachyvessels and the clinical characteristics of PCV eyes has not been determined. The purpose of this study was to determine the relationship between the presence of choroidal pachyvessels and the clinical characteristics of eyes with PCV. Methods The medical records of patients who were diagnosed with PCV and were treatment-naïve were reviewed. Fluorescein and indocyanine green angiography, fundus photography, spectral domain optical coherence tomography (SD-OCT), and enhanced depth imaging OCT (EDI-OCT) were used to obtain images of the choroid. The presence of pathologically dilated outer choroidal vessels, pachyvessels, was determined by ICGA images. These pachyvessels were confirmed to correspond with the large choroidal vessels in the EDI OCT images. The PCV eyes were divided into two groups based on the presence or absence of pachyvessels and clinical features and subfoveal choroidal thickness (SFCT) were evaluated between the two groups. Results Eighty-six eyes of 84 patients with PCV were evaluated. Pachyvessels were detected in 48 eyes (55.8%). The mean SFCT was 203.9 ± 83.9 μm in all 86 eyes, and it was significantly thinner in eyes with pachyvessels (+) than without pachyvessels (−) (183.2 ± 58.4 μm vs 230.2 ± 103.1 μm; P = 0.01). The differences in the incidence of subretinal fluid, pigment epithelial detachments, and hemorrhages between the two groups were not significant. However, the PCV eyes in pachyvessels (+) group with hemorrhage had the thinnest choroid (P = 0.047). The choroidal features of the fellow eyes were similar to those of the PCV affected eyes, that is, the fellow eyes in pachyvessels (+) group had pachyvessels and the fellow eyes in pachyvessels (−) group did not have pachyvessels. Conclusions Pachyvessels were presented 55.8% in eyes with PCV, and these eyes had the thin SFCT. The presence of pachyvessels and attenuation of the inner choroid were probably due to the pathological changes in the eyes with PCV.

Purpose To describe management of residual branch vascular network (BVN) in polypoidal choroidal vasculopathy (PCV) by thermal laser photocoagulation of feeder vessel. Observations Case report of sixty-four year old female with polypoidal choroidal vaculopathy (PCV) with moderate response to seven doses of intravitreal ranibizumab, six doses of intravitreal bevacizumab and one session of photodynamic therapy (PDT). The patient has resolved polyps but persistence of disease activity due to residual BVN and large pigment epithelial detachment (PED). Patient underwent thermal laser photocoagulation of feeder vessel of BVN identified on indocyanine green angiography (ICGA). There was complete resolution of residual BVN and large PED, which was confirmed on ICGA. Conclusions and Importance Recalcitrant cases of PCV without polyps but having BVN with feeder vessel can be managed by ICGA guided thermal laser photocoagulation. The case report illustrates the importance of utilizing multimodal imaging such as video indocyanine green angiography (ICGA) for identification of feeder vessel and its deployment for optimal management of refractory PCV.

To evaluate and compare the possible effects of hard and soft nuclear cataract surgeries in the development of early postoperative retinal complications. In a retrospective study design 1388 uncomplicated phacoemulsification surgery were enrolled in the study. Data for 688 Grade 5-6 and 700 Grade 2-3 nuclear cataracts according to Lens Opacities Classification System III (LOCS III) were used for comparison. All patients were evaluated for visual acuity (VA), intraocular pressure (IOP), central macular pathologies and angiographic fluid leakage pattern at 1 week and 1 month. Biomicroscopic evaluation, optical coherence tomography (OCT), fleuroscein angiography (FA) and indocyanine green angiography (ICG) were used for examination. Follow up time was 1 month. Mean CMT of patients were higher at hard cataracts but not statistically significant (p>0.24). Clinically significant cystoid macular edema after hard nuclear cataract surgery was 1.59 %(11) and 1.28 %(9) for soft cataracts (p>0.05). After 20/20 visual acuity at 1 week, 5 (0.72 %) patients from hard nuclear group had visual loss and hemorrhagic pigment epitelial detachment (PED) at 1 month control. Angiographic evaluations revealed them 4 as insignificant polypoid choroidal vasculopathy (PCV). One of them is still controversial. IOP and VA results were similar at both controls (p> 0.05). Subretinal hemorrhage after cataract surgery may be the first sign of some PCV patients. Patients who were diagnosed with PCV should be carefully evaluated for cataract surgery. Hard nuclear cataract may be a predisposing factor for early hemorhagic PED in patients with PCV. Any retinal pathology should be carefully evaluted before cataract surgeries. © 2018, Yuzuncu Yil Universitesi Tip Fakultesi. All rights reserved.

Purpose: To describe an unusual case of spontaneous closure of a chronic, large, idiopathic, stage 4 macular hole after failed surgery. Observations: A 75-year-old female presented with a history of a chronic, full thickness macular hole after failed surgery in the right eye. Two years after onset, she developed a fibrotic scar, which closed the macular hole and unexpectedly improved her vision. At her 4 year follow up exam, optical coherence tomography demonstrated a stable, closed macular hole with continued improvement in her visual acuity despite lack of surgical and medical intervention. Conclusions and importance: The spontaneous closure of an idiopathic full thickness macular hole is an unusual event. When it occurs, it is typically in an acute setting and is attributed to bridging retinal tissue, vitreofoveal separation, and a small diameter size. In this report, we show that a chronic, large break, that failed prior surgical intervention, can spontaneously close. The formation of an underlying fibrotic scar from type 1 neovascularization bridged the macular hole and improved her visual acuity. Keywords: Idiopathic macular hole, Spontaneous closure, Optical coherence tomography, Polypoidal choroidal vasculopathy, Type 1 neovascularization

Purpose Swept-source optical coherence tomography angiography was used to investigate choroidal changes and their association with pigment epithelial detachments (PEDs) in eyes with polypoidal choroidal vasculopathy (PCV) after treatment with vascular endothelial growth factor (VEGF) inhibitors. Methods Patients with treatment-naïve PCV were included and underwent anti-VEGF therapy. Mean choroidal thickness (MCT), choroidal vascularity index (CVI), and PED volume measurements were obtained before and after treatment. Results Thirty-four treatment-naïve PCV eyes from 33 patients were included. The PED volume decreased after treatment (P < 0.05). The MCT decreased from 223.0 ± 79.6 µm at baseline to 210.9 ± 76.2 µm after treatment (P < 0.001). The CVI at baseline was 0.599 ± 0.024, and the CVI after treatment was 0.602 ± 0.023 (P = 0.16). There was a correlation between the decreased PED volumes and the decreased MCT measurements (r = 0.47; P = 0.006). Also, there was a correlation between the decreased PED volumes and the increased CVI measurements (r = −0.63; P < 0.001). Conclusions In treatment-naïve eyes with PCV, the decreases in PED volumes were correlated with the decrease in MCT and the increase in CVI measurements. We propose that, at baseline, the PCV lesions serve as high-volume arteriovenous shunts between choroidal arterial and venous circulation, causing transudation into the choroidal stroma. We propose that, after treatment, the blood flow through the vascular shunt is reduced, the excess stromal transudation is resorbed, and the exudation from the neovascular lesion is reduced, resulting in thinning of the choroid, resolution of the PEDs, and an increase in the CVI due to the resorption of excess choroidal transudation.

Polypoidal choroidal vasculopathy is characterized by multiple and recurrent serosanguineous detachments of the retinal pigment epithelium and aneurysmal protrusions in the choroidal vessels. Different therapeutic options have been proposed, including anti-vascular endothelial growth factor drugs and photodynamic therapy. Controversy exists as to whether combination therapy is superior to anti-vascular endothelial factor drugs alone.We searched Epistemonikos, the largest database of systematic reviews in health, maintained by screening multiple sources of information, including MEDLINE/PubMed, EMBASE, and Cochrane. We extracted data from the identified reviews, analyzed the data from the primary studies, performed a meta-analysis, and prepared a summary table of the results using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) method.We identified three systematic reviews that together included twelve primary studies. Of these, two were randomized trials, and only one of them was included in the analysis.The addition of photodynamic therapy may result in little or no difference in the incidence of retinal hemorrhage and visual acuity gain at six months (low certainty of evidence). On the other hand, the combination of photodynamic therapy and anti-vascular endothelial growth factor drugs compared to anti-vascular endothelial growth factor alone is likely to increase polyp regression at three and six months and reduce central retinal thickness at six months.La vasculopatía coroidea polipoidea es una enfermedad caracterizada por la aparición de múltiples y recurrentes desprendimientos serosanguíneos del epitelio pigmentario retinal, junto con la presencia de protuberancias aneurismáticas en los vasos coroideos. Se han propuesto diferentes opciones terapéuticas, incluyendo los fármacos anti factor de crecimiento vascular endotelial y la terapia fotodinámica. Existe controversia sobre si la terapia combinada es superior a la monoterapia con anti factor de crecimiento vascular endotelial.Realizamos una búsqueda en Epistemonikos, la mayor base de datos de revisiones sistemáticas en salud, la cual es mantenida mediante el tamizaje de múltiples fuentes de información, incluyendo MEDLINE/PubMed, EMBASE, Cochrane, entre otras. Extrajimos los datos desde las revisiones identificadas, analizamos los datos de los estudios primarios, realizamos un metanálisis y preparamos una tabla de resumen de los resultados utilizando el método Grading of Recommendations Assessment, Development and Evaluation, GRADE.Identificamos tres revisiones sistemáticas que, en conjunto, incluyeron doce estudios primarios. De ellos, dos corresponden a ensayos aleatorizados. Solo se incluyó un ensayo aleatorizado en el análisis del presente trabajo.La adición de terapia fotodinámica podría resultar en poca o nula diferencia sobre la incidencia de hemorragia retinal y ganancia de agudeza visual a los seis meses (certeza de la evidencia baja). Por otro lado, adicionar terapia fotodinámica a anti factor de crecimiento vascular endotelial comparada con la monoterapia con anti factor de crecimiento vascular endotelial, probablemente aumente la regresión de pólipos a los tres y seis meses, y reduzca el grosor central retinal a los seis meses.

Purpose To investigate the morphological changes of polyps in eyes with polypoidal choroidal vasculopathy (PCV) after treatment with vascular endothelial growth factor (VEGF) inhibitors using swept source optical coherence tomography angiography (SS-OCTA). Observations Following anti-VEGF therapy, polyps were found to evolve into typical type 1 macular neovascularization (MNV) in five eyes. In all of these five eyes, a polypoidal lesion was detected adjacent to a serous or hemorrhagic retinal pigment epithelial detachment (PED). Conclusions and importance Polypoidal lesions in PCV can evolve into typical type 1 MNV. This morphological evolution suggests that these polyps are clusters of tangled vessels that can proliferate into a more typical neovascular pattern, and this evolution may be facilitated by being adjacent to a PED. Since this morphological appearance could be associated with a better prognosis, SS-OCTA might be helpful in identifying cases of transformed polyps that may be associated with a decreased risk for vision loss.

Purpose To report a case of a 46-year-old patient affected by polypoidal choroidal vasculopathy (PCV) in large colloid drusen (LCD) and to show how switching to intravitreal injection of aflibercept could be considered as a useful treatment of PCV not responsive to other anti-vascular endothelial growth factor (VEGF) injections. Observations A 46-year-old woman was referred to our department with diagnosis of early-onset retinal drusen. Multimodal imaging confirmed the diagnosis of LCD in both eyes, complicated by suggestive PVC in the left eye. Due to the absence of anatomical improvement after 6 intravitreal injections of ranibizumab, the patient was switched and treated by a single injection of aflibercept, showing a complete anatomical and functional recovery. Conclusions and Importance This case suggests progressive development of PCV as a possible late evolution of degenerating LCD. In case of exudative complication, intravitreal aflibercept injection could be considered as a useful treatment, especially in patients who are not responsive to others anti-VEGF injections.

Akira Arakawa,1,2 Maiko Inoue,2 Shimpei Sato,2 Shin Yamane,2 Kazuaki Kadonosono2 1Department of Ophthalmology, St Marianna University School of Medicine, Yokohama City Seibu Hospital, 2Department of Ophthalmology, Yokohama City University Medical Center, Yokohama, Japan Purpose: To evaluate the efficacy of intravitreal aflibercept therapy in treatment-naïve Japanese patients with polypoidal choroidal vasculopathy (PCV) using Early Treatment Diabetic Retinopathy Study (ETDRS) letter scores.Subjects and methods: This study was a prospective, nonrandomized, interventional exploratory clinical trial performed in an institutional setting. Patients with PCV were treated with intravitreal aflibercept 2 mg/0.05 mL every 2 months after 3 initial monthly doses, for 1 year. Visual acuity test using the ETDRS chart and indocyanine green angiography was performed at baseline and at 6 and 12 months after initiating the treatment, in addition to routine examinations performed at each visit. The main outcome measure was the proportion of patients who achieved

Polypoidal choroidal vasculopathy (PCV) is the rare subtype of agerelated macular degeneration (AMD), which is hard to diagnose and it is resistant to standard anti-VEGF treatment of AMD. The morphological base of PCV is the pathology of choroid vessels with formation of expansions on their ends – polyps. This literature review presented a current conception of the prevalence, risk factors, pathogenesis, diagnostic techniques and the main treatment approaches of this disease.

Tetsuyuki Suetsugu,1,2 Aki Kato,1 Munenori Yoshida,1 Tsutomu Yasukawa,1 Akiko Nishiwaki,1,3 Norio Hasegawa,1 Hideaki Usui,1 Yuichiro Ogura1 1Department of Ophthalmology and Visual Science, Nagoya City University Graduate School of Medical Sciences, 2Department of Ophthalmology, General Kamiiida Daiichi Hospital, 3Nishiwaki Eye Clinic, Nagoya, Aichi, Japan Purpose: We aimed to evaluate the prevalence of abnormal peripheral fundus autofluorescence (FAF) in wet age-related macular degeneration (AMD) using wide-field imaging instrument. Patients and methods: A retrospective, case-controlled study involving 66 eyes of 46 Japanese wet AMD patients and 32 eyes of 20 control patients was performed. Wide-field FAF images were obtained for typical AMD (37 eyes/28 patients), polypoidal choroidal vasculopathy (PCV) (22 eyes/20 patients), and retinal angiomatous proliferation (RAP) (seven eyes/four patients). Two masked ophthalmologists independently graded the images for mottled, granular, and nummular patterns. Main outcome measures were abnormal peripheral FAF frequencies and relative risks by disease subgroups and treatments. Results: Abnormal peripheral FAF patterns were found in 51.5% of wet AMD eyes compared with 18.8% of control eyes (P

Supplemental Digital Content is Available in the Text. Twelve-month results from LUMINOUS support the use of ranibizumab as an effective first-line therapy for polypoidal choroidal vasculopathy. Higher baseline visual acuity and adequate injection frequency were associated with better visual acuity outcomes. No new safety signals were identified., Purpose: To evaluate the real-world effectiveness and safety of intravitreal ranibizumab 0.5 mg in treatment-naive patients with and without polypoidal choroidal vasculopathy (PCV). Methods: Assessment of neovascular age-related macular degeneration patients with or without PCV after 12 months of ranibizumab treatment during the LUMINOUS study. Outcome measures were visual acuity and central retinal thickness changes from baseline and the rate of ocular adverse events. Results: At baseline, 572 and 5,644 patients were diagnosed with and without PCV, respectively. The mean visual acuity gain from baseline at Month 12 in the PCV and non-PCV groups was +5.0 and +3.0 letters, respectively; these gains were achieved with a mean of 4.4 and 5.1 ranibizumab injections. Eighty percent of PCV patients and 72.2% of non-PCV patients who had baseline visual acuity ≥73 letters maintained this level of vision at Month 12; 20.6% and 17.9% of patients with baseline visual acuity

Polypoidal choroidal vasculopathy (PCV) is a subtype of neovascular age-related macular degeneration (nAMD), commonly seen in the Asian population. It is dissimilar in epidemiology, genetic heterogeneity, pathogenesis, natural history, and response to treatment in comparison to nAMD. Confocal scanning laser ophthalmoscopy-based simultaneous fluorescein angiography and indocyanine green angiography, spectral-domain optical coherence tomography (OCT) with enhanced depth imaging, swept-source OCT, and OCT angiography have improved the ability to detect PCV, understand its pathology, and monitor treatment response. A plethora of literature has discussed the efficacy of photodynamic therapy, anti-vascular endothelial growth factor (VEGF) monotherapy, and combination of both, but only a few studies with higher level of evidence and limited follow-up duration are available. This review discusses the understanding of PCV with respect to epidemiology, pathogenesis, clinical features, natural history, imaging techniques, and various treatment options. Recent clinical trials (EVEREST-II and PLANET study) have emphasized that either anti-VEGF monotherapy or combination treatment is equally capable to strike a balance between polyp regression and stabilization of visual acuity. The recurrent nature of the disease, the development of macular atrophy, and the long-term poor visual prognosis despite treatment are concerns that open avenues for further research.

Background Neovascular age-related macular degeneration (AMD) and polypoidal choroidal vasculopathy (PCV) are sight-threatening maculopathies with both environmental and genetic risk factors. We have previously shown relative risks posed by genes of the complement pathways to neovascular AMD and PCV. Methods In this study, we investigated the haplotype-tagging single nucleotide polymorphisms (SNPs) in the complement component 5 (C5) gene in 708 unrelated Chinese individuals: 200 neovascular AMD patients, 233 PCV patients and 275 controls. Six tagging SNPs in C5 were genotyped. Univariate single SNP association analysis, haplotype-based association analysis and gene-gene interaction analysis between C5 and other AMD-associated genes were performed. Results The results revealed none of the six tagging SNPs of the C5 gene had a significant association with neovascular AMD or PCV (P > 0.05). We also found insignificant haplotype-based association, and no significant SNP-SNP interaction between C5 and other genes (including C2-CFB-RDBP-SKIV2L, SERPING1, CETP, ABCG1, PGF, ANGPT2, CFH and HTRA1) for neovascular AMD and PCV. Conclusions This study showed no statistical significance in the genetic association of C5 with neovascular AMD or PCV in a Hong Kong Chinese population. Further studies in large samples from different populations are warranted to elucidate the role of C5 in the genetic susceptibility of AMD and PCV.

Background To investigate the one-year visual and anatomical outcomes of combination therapy with intravitreal aflibercept (IVA) and photodynamic therapy (PDT) for treating polypoidal choroidal vasculopathy (PCV). Methods This was a retrospective case-series study, including 30 eyes from 30 patients with treatment-naïve PCV treated by combination therapy with IVA and PDT. Best-corrected visual acuity (BCVA), central retinal thickness (CRT), complete polyp regression rate, and dry macula rate were recorded every 3 months during 12-month follow-up. Clinical factors associated with final visual outcome and retreatment were investigated. Results The mean LogMAR BCVA was significantly improved from 0.73 ± 0.65 at baseline to 0.51 ± 0.60 (p = 0.01), and the mean CRT was also significantly improved from 339 ± 96 μm at baseline to 244 ± 43 μm at 12-month follow-up (p

AIM: To describe the long-term observation of vitrectomy without subretinal hemorrhage (SRH) management for massive vitreous hemorrhage (VH) secondary to polypoidal choroidal vasculopathy (PCV). METHODS: This is a retrospective, consecutive case series. A total of 86 eyes of 86 patients with >14d of massive VH associated with PCV were included. All patients underwent vitrectomy without SRH management, followed by intravitreal ranibizumab injections and/or photodynamic therapy (PDT) as needed. The main outcome measures were best-corrected visual acuity (BCVA), postoperative adverse events and the recurrence of VH. RESULTS: The average follow-up period was 25.5±9.2mo (range 12-35mo). Mean BCVA at baseline (2.16±0.39 logMAR) had improved significantly, both 3mo after surgery (1.42±0.66 logMAR, P