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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.

We aimed to investigate whether a treat-and-extend regimen of intravitreal brolucizumab (6.0 mg/0.05 mL) is effective for eyes with exudative age-related macular degeneration (AMD) refractory to aflibercept for 12 months. Sixty eyes from 56 patients receiving brolucizumab for exudative AMD refractory to aflibercept were included. Patients received a mean of 30.1 aflibercept administrations for a mean 67.9-month follow-up. All patients exhibited exudation on optical coherence tomography (OCT) despite regular 4–8 weeks of aflibercept administration. Visit 1 was scheduled at the same interval from the last aflibercept injection to the baseline. The treatment interval was extended or shortened by 1–2 weeks depending on the presence or absence of exudation on OCT. After switching to brolucizumab, the follow-up interval significantly extended at 12 months (before switching: 7.6 ± 3.8 weeks vs. at 12 months: 12.1 ± 6.2 weeks, p = 1.3 × 10−7). Forty-three percent of the eyes achieved a dry macula at 12 months after switching. However, the best-corrected visual acuity did not improve at any visit. Morphologically, the central retinal thickness and subfoveal choroidal thickness significantly decreased from baseline at 12 months (p = 3.6 × 10−3 and 1.0 × 10−3, respectively). Switching to brolucizumab can be considered to extend the treatment interval in eyes with exudative AMD refractory to aflibercept.

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.

AIM: To determine the prevalence of focal lamina cribrosa (LC) defect among patients with pachychoroid disease spectrum (PDS) in the absence of peripapillary retinoschisis. METHODS: This retrospective, cross-sectional study comprised of 180 patients with PDS, including polypoidal choroidal vasculopathy (PCV), central serous chorioretinopathy, and pachychoroidal neovasculopathy. Medical records and optic nerve head evaluations conducted using spectral-domain optical coherence tomography with enhanced depth imaging were reviewed. As a control group, 236 patients who underwent ophthalmologic evaluation for vitreous floaters, without obvious ocular disease, were also included. RESULTS: The mean age of the PDS group, which included 118 male patients (65.6%), was 57.4±11.1y. There was no significant difference between the two groups in age (P=0.710) or sex (P=0.248). Six patients (3.3%) in the PDS group and none in the control group showed focal LC defect (P=0.318). Among the six patients with focal LC defect in the PDS group, four eyes had PCV, one eye was the fellow eye of a PCV eye, and one eye had pachychoroidal neovasculopathy. CONCLUSION: Focal LC defect can be defected in patients with PDS in the absence of peripapillary retinoschisis. However, the prevalence of focal LC defect was not different significantly between PDS patients and those who did not have PDS.

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).

A specific form of drusen, known as pachydrusen, has been demonstrated to be associated with pachychoroid eye diseases, such as central serous chorioretinopathy (CSC) and polypoidal choroidal vasculopathy (PCV). These pachydrusen have been found in up to 50% of eyes with CSC and PCV and may affect the disease progression and treatment response. This study aims to investigate the association between pachydrusen and changes in fundus autofluorescence (FAF) in eyes with CSC and PCV. A total of 65 CSC patients and 32 PCV patients were evaluated. Pachydrusen were detected using both color fundus photography and spectral-domain optical coherence tomography. The relationships between pachydrusen and FAF changes were then investigated. The prevalence of pachydrusen in CSC and PCV eyes was 16.7% and 61.8%, respectively. The mean age of patients with pachydrusen was significantly older than those without pachydrusen (CSC: 56.3 vs. 45.0 years, p < 0.001; PCV: 68.8 vs. 59.5 years, p < 0.001). No significant difference was found in the mean subfoveal choroidal thickness between eyes with or without pachydrusen. Eyes with pachydrusen were significantly associated with more extensive FAF changes in both CSC and PCV (p < 0.001 and p = 0.037, respectively). The study demonstrated that pachydrusen are more prevalent in PCV than CSC. Increasing age and more extensive abnormalities in FAF are associated with the presence of pachydrusen, suggesting that dysfunction of retinal pigment epithelial cells is associated with pachydrusen.

Gahyung Ryu,1,2 Donghyoun Noh,1,2 Guihyun Moon,3 Min Sagong1,2 1Department of Ophthalmology, Yeungnam University College of Medicine, Daegu, Republic of Korea; 2Yeungnam Eye Center, Yeungnam University Hospital, Daegu, Republic of Korea; 3Bayer Korea Ltd, Seoul, Republic of KoreaCorrespondence: Min SagongDepartment of Ophthalmology, Yeungnam University College of Medicine, 170 Hyunchungro, Nam-gu, Daegu, 42415, Republic of KoreaTel +82-53-620-3443Fax +82-53-626-5936Email msagong@yu.ac.krPurpose: To investigate the efficacy and safety of intravitreal aflibercept (IVT-AFL) in Asian patients with neovascular age-related macular degeneration (nAMD) in a real-world clinical setting.Patients and Methods: In this analysis of a prospective, regulatory, postmarketing surveillance study for IVT-AFL, 3115 patients with nAMD were included and followed for 8 months. The mean changes in best-corrected visual acuity (BCVA) and central retinal thickness (CRT) were analyzed using last observation carried forward method. A post hoc subgroup analysis and a multivariate logistic regression analysis were also performed to assess factors related to treatment outcomes.Results: Mean BCVA improved from 0.62 to 0.51 logarithm of minimum angle resolution and mean CRT decreased from 383.3 to 289.7 μm, with a mean of 3.4 injections during the 8-month follow-up. In the subgroup analysis, patients who had received 3 initial monthly doses had significantly better anatomical improvements than those treated as needed. Patients with confirmed polypoidal choroidal vasculopathy (PCV) had significantly better anatomical improvements and better visual recovery than those with other types of nAMD. The multivariate regression analysis demonstrated that age, injection number, PCV, and baseline BCVA were significantly associated with higher odds of gaining 3 lines at 8 months, and sex, injection number, PCV, and baseline CRT were associated with CRT ≤ 250 μm at 8 months. No new safety findings were identified.Conclusion: IVT-AFL was effective and well tolerated in a real-world setting with a large number of Asian patients with nAMD. Number of injections and PCV were important determinants for improved treatment outcomes.Keywords: aflibercept, Asian, neovascular age-related macular degeneration, polypoidal choroidal vasculopathy, postmarketing surveillance, real-world

The purpose of this study was to provide an estimate of the number of current and future patients with polypoidal choroidal vasculopathy (PCV) in Europe. We systematically searched 11 literature databases on 18 May 2022 for studies on the prevalence of PCV among a consecutive and representative group of patients with suspected neovascular age-related macular degeneration (AMD). Prevalence of PCV in patients with suspected neovascular AMD was summarized and included in a prevalence meta-analysis. We then used current population data and population forecasts by Eurostat and the Office for National Statistics to determine current and future number of patients with neovascular AMD in Europe. Then, we calculated the number of patients with PCV with our calculated estimate of the prevalence of PCV among Europeans suspected with neovascular AMD. A total of five eligible studies were identified which included a total of 1359 patients. All these studies used the gold standard of indocyanine green angiography as a routine part of their diagnostic approach. Among patients undergoing detailed retinal examination for suspected neovascular AMD, our meta-analysis calculated the prevalence of PCV to be 8.3% (95% confidence interval: 6.8–9.8%). Our population estimates find that a total of 217,404 patients with PCV exist in Europe in the year 2022, which constitutes 0.04% of the entire population of Europe. This number is estimated to increase to 287,517 patients in the year 2040. Our estimates are important for different healthcare stakeholders, especially when planning and allocating expensive resources.

Cushing’s syndrome is a rare disease with an endogenous cause of excess cortisol secretion. More evidence substantially links cortisol levels to the pachychoroid spectrum diseases. In this systematic review and meta-analysis, we summarize available evidence on pachychoroid spectrum diseases in patients with Cushing’s syndrome. We performed a systematic literature search in 11 databases on 21 May 2022. Studies were considered eligible if they performed retinal examination of a consecutive group of patients with Cushing’s syndrome using optical coherence tomography (OCT) scans. We extracted data on subfoveal choroidal thickness in patients with Cushing’s syndrome compared to matched controls. We also extracted data on the prevalence of pachychoroid pigment epitheliopathy (PPE), central serous chorioretinopathy (CSC), and polypoidal choroidal vasculopathy (PCV). We identified six eligible studies with a total of 159 patients with Cushing’s syndrome. On average, patients with Cushing’s syndrome have 49.5 µm thicker subfoveal choroidal thickness compared to matched healthy individuals. Pachychoroid spectrum diseases were relatively common in these patients: PPE in 20.8%, CSC in 7.7%, and PCV in 2.8%. We conclude that there should be low threshold to recommend ophthalmic examination to patients with Cushing’s syndrome, and that a macular OCT is recommended during this examination.

We investigated the relationship between pachydrusen and choroidal thickness and age in eyes with polypoidal choroidal vasculopathy (PCV) and fellow eyes, compared to eyes with central serous chorioretinopathy (CSC). This retrospective study included 89 eyes with PCV and 146 eyes with CSC. The number, location, and shape of the pachydrusen and their association with choroidal thickness and age were analyzed. PCV eyes showed pachydrusen more frequently than eyes with CSC (52% vs. 20%, p < 0.001). Large solitary type and clustered type were more frequent in PCV eyes compared to CSC eyes (p = 0.003 and p = 0.001, respectively). Subfoveal choroidal thickness was associated with pachydrusen in eyes with PCV (odds ratio [OR] 1.006, 95% confidence interval [CI] 1.001–1.011, p = 0.027), while age was associated with pachydrusen in CSC eyes (OR 1.137, 95% CI, 1.073–1.205; p < 0.001). Pachydrusen were localized directly over the pachyvessel on optical coherence tomographic findings in approximately two thirds of PCV eyes and fellow eyes (62% and 67%, respectively). Risk factors for pachydrusen differ according to diseases. The presence of pachydrusen was associated with choroidal thickness in PCV, while the association with age was more prominent in CSC.

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.

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

Several adjunct therapies to the gold standard anti-vascular endothelial growth factor (anti-VEGF) intravitreal injections have been discussed for the treatment of neovascular age-related macular degeneration (nAMD). Low-dose stereotactic radiotherapy (SRT) showed the potential to lower the treatment burden by reducing the anti-VEGF treatment frequency at least over 2–3 years but was associated with retinal microvascular abnormalities in a few cases. We report a 6-year follow-up of a case with bilateral nAMD under anti-VEGF treatment which developed multiple polypoid choroidal vasculopathy (PCV) lesions in the eye adjunct treated with low-dose SRT. The fellow eye suffering from nAMD for the same period of time but never been treated with SRT did not show PCV during the long-term follow-up. We hope to increase the awareness of possible choroidal changes such as PCV in similar patients by sharing this report.

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.

We report the two cases who presented with sudden decreased vision and sub-internal limiting membrane (ILM) hemorrhage without a history of Valsalva maneuver, trauma, or hematological disorders. Multimodal imaging revealed the features suggestive of polypoidal choroidal vasculopathy (PCV) in addition to the sub-ILM hemorrhage. A provisional diagnosis of sub-ILM hemorrhage secondary to PCV was made and was treated with intravitreal Bevacizumab injection. Treatment resulted in the improved visual acuity along with the resolution of the sub-ILM hemorrhage. These cases highlight the possibility of isolated sub-ILM hemorrhage as a presenting fundus finding in PCV, which is previously unreported. This report also highlights the importance of multimodal imaging in diagnosing chorioretinal disorders with unusual presentation.

This study investigated one-year outcomes of treatment with one session of intravitreal recombinant tissue plasminogen activator, ranibizumab, and gas injections for submacular hemorrhage secondary to polypoidal choroidal vasculopathy (PCV). An extended study of a previous prospective trial of this treatment modality in PCV patients was conducted in 64 patients (64 eyes). Early Treatment Diabetic Retinopathy Study (ETDRS) score, central retinal thickness (CRT), and central pigment epithelial detachment thickness (CPEDT) before and 1, 3, and 12 months after treatment were analyzed. Mean ETDRS score increased from 58 at baseline to 64 letters (p = 0.0122), CRT decreased from 543 to 192 μm (p < 0.0001), and CPEDT decreased from 161 to 103 μm (p = 0.0668) at 3 months and were maintained until 12 months. Complications requiring reoperation occurred within one month in four eyes. Recurrence was observed in 46 eyes (72%), and 1.6 ± 1.5 (0–7) intravitreal aflibercept injections were given pro re nata. Univariate and multivariate analyses identified CPEDT as the pre- and post-treatment factor affecting 12-month ETDRS score (p < 0.0001). Improved visual acuity stabilized 3 months after treatment. Although 72% of patients experienced recurrence, an average of 1.6 aflibercept injections/patient maintained visual acuity up to 12 months. CPEDT was the most important factor associated with visual outcome.

Introduction: Since July 2021, a worldwide shortage of verteporfin (Visudyne (R)) occurred: an essential medicine required for photodynamic therapy (PDT). PDT with verteporfin has a broad range of indications in ophthalmology, including chronic central serous chorioretinopathy, polypoidal choroidal vasculopathy and choroidal haemangioma. For these disorders, PDT is either the first-choice treatment or regarded as a major treatment option. Materials and methods: A questionnaire was sent to key opinion leaders in the field of medical retina throughout the world, to assess the role of PDT in their country and the effects of the shortage of verteporfin. In addition, information on the application of alternative treatments during shortage of verteporfin was obtained, to further assess the impact of the shortage. Results: Our questionnaire indicated that the shortage of verteporfin had a major impact on ophthalmic care worldwide and was regarded to be a serious problem by most of our respondents. However, even though there is ample evidence to support the use of PDT in several chorioretinal diseases, we found notable differences in its use in normal patient care throughout the world. Various alternative management strategies were noted during the verteporfin shortage, including lowering the dose of verteporfin per patient, the use of alternative treatment strategies and the use of a centralized system for allocating the remaining ampoules of verteporfin in some countries. Conclusion: The shortage of verteporfin has had a large effect on the care of ophthalmic patients across the world and may have resulted in significant and irreversible vision loss. Mitigation strategies should be developed in consultation with all stakeholders to avoid future medication shortages of verteporfin and other unique ophthalmic medications. These strategies may include mandatory stock keeping, compulsory licensing to an alternative manufacturer or incentivizing the development of competition, for example through novel public-private partnerships.

Purpose: We report a case of old submacular hemorrhage (SMH) due to polypoidal choroidal vasculopathy (PCV). Subretinal endoscopic surgery (SES) was performed, which improved visual function. In addition, we show the intraoperative findings of subretinal aberrant PCV vessels as seen under endoscopic observation, which cannot be observed by microscopic surgery. Observations: A 71-year-old Japanese man presented with an old dehemoglobinized SMH due to PCV in his left eye. At the time of presentation, three weeks had already passed after the onset of the patient's symptoms, and the best-corrected visual acuity (BCVA) was 20/200. SES was performed to remove the SMH and treat the subretinal PCV lesions. After creating retinal detachment using a 38-gauge cannula, three subretinal 25-gauge trocars were inserted from the sclera to the subretinal space. Then, SES was performed under ophthalmic endoscopic observation with continued subretinal irrigation for maintaining the retinal detachment. After removal of the SMH, subretinal polyp-shaped nodular vascular lesions (polyps) and a branching vascular network, which is located inside the retinal pigmented epithelium, were identified. The sites that presumably originated from the aberrant vessels of the PCV and the associated polyps were coagulated using endodiathermy. After the subretinal procedure, the retina was flattened with fluid/air exchange, and silicone oil (SO) was injected into the vitreous cavity. The SMH completely disappeared after surgery. Although at one-month follow-up BCVA (20/250) was slightly worse than that before surgery, there was an improvement in postoperative retinal sensitivity in the macula compared to that before surgery. At the three-month follow-up, the SO was removed. The BCVA was 20/200 one month after SO removal. No postoperative complications occurred. Additional treatment was not required, including anti-vascular endothelial growth factor therapy, for PCV progression or SMH recurrence in the left eye till the final visit two years after surgery. Conclusion and importance: SES could effectively remove the old SMH, and the activity of PCV was suppressed by intraoperative subretinal coagulation. The retinal sensitivity of the macula improved after the SES. In addition, we observed subretinal polyps and a branching vascular network located internal to the retinal pigmented epithelium under intraoperative subretinal endoscopic observation. SES is a good surgical option for the removal of old SMH or treatment of subretinal lesions.

Introduction To evaluate 1-year outcomes of intravitreal aflibercept (IVA) using a treat-and-extend (TAE) regimen for polypoidal choroidal vasculopathy (PCV) and identify the factors for patients whose treatment intervals could be extended. Methods Fifty-one eyes of treatment-naïve PCV patients treated with IVA using a TAE regimen for at least 1 year were examined retrospectively. All patients received at least three IVA injections every 5 weeks, and the intervals were then extended by 2-week adjustments up to 13 weeks. When retinal exudation recurred, the patient was treated with the same regimen, but with a shortened interval of 5 weeks. The main outcome measures were changes in best-corrected visual acuity (BCVA) and central retinal thickness (CRT) as well as the treatment interval at 1 year. Results The mean logarithm of the minimum angle of resolution BCVA improved from 0.24 ± 0.32 at baseline to 0.18 ± 0.31 at 12 months (p = 0.048). The mean CRT decreased from 350.3 ± 147.7 µm at baseline to 215.3 ± 75.0 µm at 4 months (p

Background To determine the frequency of persistent disease activity following 3 loading doses of anti- vascular endothelial growth factor (VEGF) agents, and the anatomic and demographic predictors of early persistent disease activity among patients with neovascular age-related macular degeneration (nAMD). Methods In a retrospective real-world cohort study, 281 consecutive patients with nAMD were reviewed at baseline and after 3 anti-VEGF injections for pre-defined indicators of disease activity. Optical coherence tomography (OCT) features such as subretinal fluid, intraretinal cysts and intraretinal fluid were assessed by reading-center certified graders. Multiple logistic regression was performed on demographic and anatomic factors. Results At month 3, 66.1% of patients had persistent disease activity. The best-corrected visual acuity (BCVA) improvement was 0.16 LogMAR for those with no disease activity compared to 0 for patients with persistent activity (p p = 0.025), intraretinal cysts at baseline (OR 2.95, 95% CI 1.67–5.20, p p = 0.002). At 3 months, 58% of patients had features of activity on OCT. Patients with intraretinal cysts and intraretinal fluid at baseline had worse BCVA at month 3 compared to patients without these OCT features (0.69 vs. 0.43, p p < 0.001, respectively). Conclusions In a real-world study, 66.1% of nAMD patients have persistent disease activity after the initial loading dose, with poorer BCVA compared to those without. Baseline OCT features (intraretinal cysts and subretinal fluid) are useful predictors of persistent disease activity at month 3.

Photodynamic therapy (PDT) using verteporfin (Visudyne®; Bausch + Lomb) is a treatment that is widely used to elicit cell and tissue death. In ophthalmology, PDT targets choroidal vascular abnormalities and induces selective occlusion of vessels. PDT was originally used in combination with full-dose verteporfin to treat neovascular age-related macular degeneration. Since the introduction of treatment with vascular endothelial growth factor receptor inhibitors, the clinical targets of PDT have shifted to other chorioretinal conditions, such as central serous chorioretinopathy, polypoidal choroidal vasculopathy, and choroidal hemangioma. In recent years, clinical studies have facilitated the optimization of treatment outcomes through changes in protocols, including the introduction of reduced treatment settings, such as PDT with half-dose verteporfin and half-fluence PDT. Here, we review PDT and its use for chorioretinal diseases from a practical perspective.

Aim To classify polypoidal choroidal vasculopathy (PCV) into 2 subtypes based on the subfoveal choroidal thickness (SFCT) and to further evaluate their multimodal image features. Methods A retrospective observational case series study. Sixty-four eyes of 64 patients with PCV were enrolled and classified into 2 groups based on SFCT (thick-choroid group/thin-choroid group). Then further analyze the spectrum domain optical coherence tomography (SD-OCT) and indocyanine green angiography (ICGA) differences of the two subtypes. Imaging analysis included measurement of SFCT, maximum vascular diameter ratio (MVDR), choroidal vascularity index (CVI), central macular thickness (CMT), and the presence of pigment epithelial detachment (PED) on SD-OCT. Polypoidal lesions (polyps) number, branching vascular network (BVN) area, greatest linear dimension (GLD), and the choroidal vascular hyperpermeability (CVH) were analyzed by ICGA. Results The distribution of SFCT was bimodal with two peaks at 195 and 285 µm, and a trough at 225 µm. The 225 µm was taken as the cutoff point for the following classification of thick/thin choroid groups. The PCV eyes in the thick-choroid group presented with greater MVDR, CVI within 3 and 6 mm of the fovea, but lower CMT, less PED, small PED diameters on SD-OCT scans, and fewer polyps, smaller BVN and GLD, but more frequency of CVH on ICGA. Conclusion The SFCT at 225 µm can be used as a readily available indicator for the classification of PCV subtypes. The thick-choroid group presents much apparent enlargement of the choroidal layer and vasculature expansion, which indicates different pathogenesis of the two subtypes.

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 evaluate the efficacy of vitreous hemorrhage(VH)in patients with polypoidal choroidal vasculopathy(PCV), and to summarize the clinical characteristics of the cases.METHODS: Retrospective case series. From Jan. 2014 to Dec. 2017, 14 patients(15 eyes)with PCV combined with VH were treated by microincision 25G vitrectomy in our hospital and were followed up for at least 6mo. Data of medical history and follow up observation were collected. The main outcome measures included visual acuity, intraocular pressure, ultrasonography, color fundus photography, fundus angiography, optical coherence tomography, and surgical complications. To analyze the difference of the best corrected visual acuity(BCVA, LogMAR)between pre-operation and postoperation, and to summarize the clinical characteristics and therapeutic effect of the cases.RESULTS: Totally 11 cases had history of hypertension. 9 eyes were diagnosed with PCV before vitrectomy. The follow-up period was from 6-36mo. All 15 eyes were treated with microincision 25G vitrectomy. During the operation, 3 eyes were silicone oil tamponade in the vitreous cavity, 2 eyes were gas tamponade, and the remaining eyes were balanced saline solution tamponade. Postoperative complications included progressed cataract in 2 eyes, recurrent VH in 1 eye, high intraocular pressure in 1 eye, and recurrent retinal detachment in 0 eye. The BCVA before surgery was 2.78±0.46, and the last follow up BCVA after surgery was 1.15±0.50. Postoperative BCVA was significantly improved compared with the preoperation(t=11.14, PCONCLUSION: Microincision vitrectomy is a safe and effective way to treat PCV with VH, which can improve the visual acuity of patients with PCV companied with VH. The history of hypertension may be a risk factor for PCV with VH.

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

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.

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.

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.

Background: To evaluate the short-term outcomes of switching to ranibizumab in aflibercept-resistant polypoidal choroidal vasculopathy (PCV). Methods: This retrospective study included 18 eyes diagnosed with aflibercept-resistant PCV. All patients were treated with two to four consecutive ranibizumab injections at 4–5-week intervals. The best-corrected visual acuity (BCVA), and central retinal thickness (CRT) values before and after switching to ranibizumab were compared. The proportion of eyes showing ≥100 µm decrease in retinal thickness and/or complete resolution of fluid after switching was identified. Results: The mean number of aflibercept injections before switching was 5.7 ± 3.3. After switching, a mean of 2.8 ± 0.6 consecutive ranibizumab injections was performed. The mean logarithm of minimal angle of resolution (logMAR) BCVA was 0.41 ± 0.26 (Snellen equivalents = 20/51) before switching, and 0.40 ± 0.30 (20/50) after switching (p = 0.574). The mean CRT was 422.2 ± 152.4 µm before switching, and 400.7 ± 182.0 µm after switching (p = 0.236). A decrease in CRT of ≥100 µm, and/or complete resolution of fluid was noted in three eyes (16.7%). Conclusions: Switching to ranibizumab in aflibercept-resistant polypoidal choroidal vasculopathy was not effective in most patients, suggesting the need for further investigation to seek more effective treatment options for this condition.

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.

We compared the short-term outcomes between 3-monthly aflibercept and brolucizumab injections for treatment-naïve polypoidal choroidal vasculopathy (PCV). A total of 52 eyes were included. Patients received 3 monthly intravitreal aflibercept (n = 38) or intravitreal brolucizumab (n = 14). Indocyanine green angiography (ICGA) was performed at baseline and at the 3-month visit. Selection of anti-VEGF agents depended on time. In the brolucizumab-treated group, best-corrected visual acuity (BCVA) improved from 0.27 ± 0.34 (log MAR unit) at baseline to 0.20 ± 0.24 at 3-month visit, which is comparable with the aflibercept-treated group (p = 0.87), after adjustment of confounding factors. Central retinal thickness significantly decreased by 43%−44% in both groups. Subfoveal choroidal thickness also significantly decreased by 20.5% during this interval in the brolucizumab-treated group, which was greater than the aflibercept-treated group. The complete resolution rate of polypoidal lesions on ICGA was significantly higher (p = 0.043) in the brolucizumab-treated group (78.6%) than in the aflibercept-treated group (42.1%). Intraocular inflammation was observed in 14.3% (2/14) in the brolucizumab-treated group only. In short-term follow-up, intravitreal injection of 3-monthly brolucizumab was comparable with aflibercept in terms of BCVA and morphological improvement along with higher resolution of polypoidal lesion(s) on ICGA.

BACKGROUND: This study aimed to investigate the trend of selecting ranibizumab and aflibercept for the initial treatment of neovascular age-related macular degeneration (AMD) and polypoidal choroidal vasculopathy (PCV). METHODS: This was a retrospective study that included 460 patients who were diagnosed with treatment-naïve neovascular AMD and PCV and were initially treated with either ranibizumab or aflibercept. The patients were divided into two groups: the ranibizumab group (n = 96) and the aflibercept group (n = 324). The patients’ characteristics and the proportion of the subtypes of macular neovascularization (MNV) were compared between the two groups. RESULTS: Patients in the ranibizumab group were significantly older (mean 74.3 ± 8.4 years) than those in the aflibercept group (mean 70.4 ± 8.8 years, p &lt, 0.001). In the ranibizumab group, the proportions of type 1 or 2 MNV, type 3 MNV, and PCV were 50.0%, 27.1%, and 22.9%, respectively. In the aflibercept group, the proportions were 35.2%, 6.8%, and 58.0%, respectively. There was a significant difference in the proportion of MNV subtypes between the ranibizumab and aflibercept groups (p &lt, 0.001). Ranibizumab was used in 54.2% of patients with type 3 MNVs. However, in patients with PCV, aflibercept was used in 89.5% of patients. CONCLUSIONS: Ranibizumab was preferred as an initial treatment agent in older patients and those with type 3 MNV, whereas aflibercept was highly preferred in patients with PCV. The different characteristics and efficacy of the two agents may have partially contributed to this trend.

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.

Background To evaluate 5-year outcomes of anti-vascular endothelial growth factor (VEGF) monotherapy and combination therapy of anti-VEGF agents and photodynamic therapy (PDT) for polypoidal choroidal vasculopathy (PCV) in a real-world Chinese population. Methods Retrospective study. Fifty-three eyes of 46 patients with subtype 1 and 2 PCV followed up for at least 60 months were grouped into three regimens: anti-VEGF monotherapy, PDT combining with anti-VEGF therapy initially, and PDT combining with deferred anti-VEGF therapy. Main outcome measure was best-corrected visual acuity (BCVA) using logarithm of minimal angle of resolution (logMAR). Results The mean BCVA of eyes with subtype 1 PCV (n = 28) deteriorated from 0.69 logMAR at baseline to 1.25 logMAR at months 60 (P = 0.001), while the mean BCVA of eyes with subtype 2 PCV (n = 25) sustained stable from 0.62 logMAR at baseline to 0.57 at months 60 (P = 0.654). No significant differences of visual outcomes were found between the 3 treatment regimens for subtype 1 PCV. Anti-VEGF monotherapy and initial combination treatment had better visual outcomes in eyes with subtype 2 PCV than deferred combination group during part of follow-up significantly. Initial combination group needed a less number of PDT than deferred combination group (P Conclusions Compared with subtype 1 PCV, subtype 2 PCV has a more favorable visual outcome in real world. All the regimens presented unfavorable visual outcomes for subtype 1 PCV. Anti-VEGF monotherapy and initial combination therapy should be superior to deferred combination therapy in the long-term management of subtype 2 PCV. Prospective randomized studies of larger size are needed to determine the long-term efficacy and safety of various treatment for PCV in real world.

Background Due to some similarities in the manifestations between central serous chorioretinopathy (CSC) and polypoidal choroidal vasculopathy (PCV), PCV may be misdiagnosed as CSC. More attention should be paid to distinguishing these two disorders. Case summary A 52-year-old woman presented to our hospital with blurred vision in her left eye for approximately 1 wk. Anterior segment and intraocular pressure findings were normal in both eyes. Fundus photography of the left eye showed a seemingly normal adult oculus fundus without any obvious hard exudate or hemorrhage. Optical coherence tomography exhibited a hypo-reflective space beneath both the neurosensory retina and the pigment epithelium layer. The late phase of fluorescein angiography revealed increased leakage. The patient was initially diagnosed with CSC. At follow-up, however, the final diagnosis turned out to be PCV. Conclusion CSC and PCV are two different retinal entities. Lipid deposition and hemorrhage are the most important elements that lead to confusion between these two entities. Indocyanine green angiography should be performed to make a definitive diagnosis, especially in cases with suspected PCV.

Polypoidal choroidal vasculopathy(PCV)in the past referred to as “Idiopathic polypoidal choroidal vasculopathy(IPCV)”. It is a chronic vascular abnormality resulting in multiple, recurrent, serosanguinous retinal pigment epithelial detachments(PEDs)secondary to leakage and bleeding from choroidal vascular lesions. PCV have two types, including hemorrhagic type and exudative type, in which hemorrhagic type is prone to submacular hemorrhage. In recent years, with the widely used of indocyanine green angiography and optical coherence tomography angiography in ophthalmology, the research on this disease has gradually deepened. At present, the diagnosis of this disease is mainly confirmed by dilated pupil fundus examination, optical coherence tomography(OCT), OCTA, fluorescence fundus angiography(FFA)+ICGA and other auxiliary examinations. When complicated with a small amount of SMH, it can be absorbed by itself. When SMH is large, it is difficult to be absorbed by itself and has a great influence on vision. The main treatment included anti-VEGF drugs, PDT, gas, t-PA and combined vitrectomy. This article combining the latest progress in the study of domestic and foreign, the SMH induced by PCV epidemiology, pathogenesis, diagnosis, treatment were summarized, and the treatment methods were discussed and prospected.

Importance It is important to identify features of polypoidal choroidal vasculopathy (PCV) that differentiate it from typical neovascular age‐related macular degeneration (nAMD) on various imaging modalities, including fluorescein angiography (FA). Background PCV was thought to be indistinguishable from nAMD using FA alone. In real‐world practice, indocyanine‐green angiography may often be unavailable or contraindicated. Design Analysis of FA images from a prospective, multicentre study. Participants Study images of both PCV and nAMD patients from the EVEREST study. Methods FA features at baseline were independently graded by masked graders (fellowship‐trained ophthalmologists) using standardized diagnostic algorithms. Main Outcome Measures Predictive indicators (sensitivity, specificity, positive and negative predictive values) for PCV. Results Of the 95 patients screened, 61 had PCV. Of the 34 screening failures, 15 were diagnosed as nAMD. Hyperfluorescent nodules on FA were observed in 80% of patients with PCV vs 20% with nAMD (P < 0.001). Blocked fluorescence on FA, which corresponded to the presence of subretinal haemorrhage, occurred more frequently among patients with PCV vs nAMD (61.7% vs 13.3%, P = 0.001). Similarly, the leakage characteristic of occult choroidal neovascularization occurred more frequently among patients with PCV vs nAMD (95.0% vs 73.3%, P = 0.026). The positive predictive value for PCV was 94.1% for hyperfluorescent nodules, 94.9% for blocked fluorescence, 83.8% for occult choroidal neovascularization and 82.0% for pigment epithelial detachment. Conclusions and Relevance This study demonstrated that certain FA features can be predictive of PCV and may be considered as an indication for retina specialists to perform indocyanine green angiography as confirmatory test.

Purpose: To report the visual and anatomical outcomes of intravitreal ziv-aflibercept (IVZ) and bevacizumab (BVZ) monotherapy in treatment-naive polypoidal choroidal vasculopathy (PCV). Methods: This was a retrospective case series of 16 eyes (8 eyes each in IVZ and BVZ groups). The study period was from January 2016 to March 2018. The inclusion criteria were treatment-naive PCV patients who were treated with either IVZ or BVZ monotherapy on pro re nata protocol and followed up monthly for 6 months. The change in best-corrected visual acuity (BCVA), central macular thickness (CMT), and pigment epithelial detachment (PED) height was measured at baseline and 6 months. Results: A total of 16 eyes were studied. IVZ group had an improvement in BCVA by 0.15 logarithm of minimum angle of resolution (logMAR; approximately 1.5 lines) at 6 months, whereas BVZ group had a reduction in BCVA by 0.21 logMAR (approximately 2 lines) (P = 0.027). Five patients and one patient in IVZ and BVZ groups, respectively, had ≥5 letters gain of BCVA. IVZ group had significant reduction in PED height (P = 0.048), whereas the change in CMT was not significant at 6 months (P = 0.681). The mean number of injections (2.87 ± 0.83 in IVZ and 2.25 ± 0.89 BVZ group; P = 0.168) and longest treatment-free interval (3.00 ± 2.20 months in IVZ and 2.12 ± 1.96 months in BVZ group; P = 0.41) were not significantly different. Conclusion: The visual and anatomical outcomes in terms of PED reduction in treatment-naive PCV patients were better in IVZ group compared with BVZ. IVZ monotherapy is a viable, cost-effective alternative in these patients with good safety profile.

AIM: To evaluate the real-life clinical outcomes of intravitreal injection of conbercept combined rescue therapy for polypoidal choroidal vasculopathy (PCV). METHODS: This was an open label, single center, and interventional study. All enrolled patients were treated initially with three consecutive monthly intravitreal conbercept injections (0.5 mg). Additional conbercept injections were administered upon substantial polyp regression with improved visual acuity (VA). Eyes with partial or no polyp regression and poor VA were rescue treated with photodynamic therapy (PDT) for subfoveal polyps or thermal laser photocoagulation for extrafoveal polyps. Best-corrected visual acuity (BCVA), central foveal thickness (CFT) and polyp regression were observed as primary outcomes. Side effects were also collected during the follow-up period. RESULTS: A total of 56 eyes (56 patients) with PCV were included. BCVA increased significantly from the baseline of 43.52±24.21 letters to 55.88±21.94 letters (P

AIM:To explore the photodynamic therapy(PDT)combined with intravitreal injection of Ranibizumab(IVR)on visual acuity, central macular thickness(CMT)and postoperative complications in the treatment of polypoidal choroidal vasculopathy(PCV)complicated with vitreous hemorrhage(VH). METHODS: Totally 71 cases of patients(71 eyes)with PCV complicated with VH were divided into two groups according to the treatment methods. And observation group(n=43)was given PDT with IVR after vitrectomy, and control group(n=28)was only given IVR adjuvant treatment. The best corrected visual acuity(BCVA), CMT and postoperative complications were followed up at the 1st, 3rd and 6th month after treatment. RESULTS: After treatment, the BCVA in the two groups was gradually improved while the CMT was gradually decreased with time, and the difference within-groups at different times was statistically significant(PPPχ2=4.508, PP>0.05). CONCLUSION: PDT combined with IVR for patients with PCV and VH after vitrectomy is beneficial to promote the absorption of vascular exudate and the elimination of polypoid lesions, thus reducing macular edema, improving the visual acuity and reducing postoperative complications. And its clinical efficacy and safety are worthy of recognition.

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.