Patients with nAMD in TENAYA/LUCERNE always on extended faricimab dosing (≥12-weeks) achieved robust disease control throughout 2 years. Additionally, 56% of patients could have potentially extended to every-20-week dosing had the protocol allowed.

Polypoidal choroidal vasculopathy (PCV) is a subtype of neovascular age-related macular degeneration (nAMD), characterised by polypoidal lesions. Prevalence data indicate that PCV is more common in people of Asian ethnicity, although it also occurs in non-Asian individuals. However, because of a lack of globally agreed diagnostic criteria and routine screening programmes, PCV is likely to be under-diagnosed in some populations. Recent consensus suggests that PCV is a form of type 1 macular neovascularisation and should be classified as a subtype of nAMD. Anti–vascular endothelial growth factor (VEGF) monotherapy has demonstrated short-term efficacy in terms of visual acuity in PCV, and outcomes may be further improved by combining anti-VEGF therapy with photodynamic therapy. However, data on the long-term (≥ 5 years) efficacy of these treatments are limited and often conflicting. Although some people with PCV can achieve long-term remission following initial treatment (21.7–51.5

Autoimmune retinopathy (AIR) is a rare, potentially blinding retinal disease that remains a challenging condition to manage when resistant to conventional immune-modulatory approaches. We report clinical and electrophysiological improvement in a 49-year-old patient who underwent an autologous hematopoietic stem cell transplant (aHSCT) for thymoma-associated AIR after experiencing progressive disease despite receiving periocular and systemic steroids, mycophenolate mofetil, baricitinib, tacrolimus, bortezomib, rituximab, plasmapheresis, and intravenous immunoglobulin. The aHSCT had two stages: (i) peripheral blood stem cell harvest following mobilization with cyclophosphamide and granulocyte colony-stimulating factor, and (ii) conditioning regimen with plasmapheresis, rituximab, cyclophosphamide, and anti-thymocyte globulin high-dose therapy, followed by autologous hematopoietic cell infusion of 5.74 million cells. Symptoms of photopsia rapidly abated after undergoing aHSCT, and objective investigations of structure and function similarly demonstrated improvement. At 22 months’ follow-up, she continued to demonstrate the durability of the clinical response. The present report suggests that in judiciously selected patients, HSCT may provide a rescue option for refractory AIR. Further cases are needed to confirm these results.

A panel of 21 international experts are formed by the Asia-Pacific Vitreo-retina Society to work out the consensus and guidelines on polypoidal choroidal vasculopathy (PCV). PCV is a common subtype of neovascular age-related macular degeneration and is more prevalent in Asian populations. Recent advancement in imaging technology allows greater understanding of the disease process of PCV. Furthermore, non-indocyanine green angiography features in optical coherence tomography angiography have been validated for PCV diagnosis and treatment response monitoring. Clinical trials provide new data on the safety and efficacy of various anti-vascular endothelial growth factor (anti-VEGF) agents as well as protocols that aim to improve the sustainability of treatments. In view of the myriads of emerging information, the panel gathered, reviewed, discussed, formulated and voted on the consensus and guidelines of PCV on four areas: 1) disease entity, 2) investigation and diagnosis, 3) treatment options, and 4) management protocol and future development in a five-point Likert scale (strongly agree, agree, neutral, disagree and strongly disagree). We proposed using a consensus score in which the maximum was 100. The summation of the respective "percentages" of experts voting for "agree" or "strongly agree" would be counted as the actual score. Consensus was achieved when the actual score of 75 or more was reached, which meant at least 75 % of the experts had voted for "strongly agree" or "agree" on the consensus statement concerned.

To define the differentiating features of pachychoroid neovasculopathy (PNV) and neovascular age-related macular degeneration (nAMD) without dye angiography. This study included treatment-naive 50 PNV patients and 50 nAMD patients with unilateral Type 1 macular neovascularisation (MNV). The studied optical coherence tomography (OCT) features were subretinal hyperreflective material, intraretinal cysts, choroidal thickness (CT), pigment epithelial detachment (PED), retinal pigment epithelium (RPE) changes, and types of drusen. The choroidal vascularity index (CVI) was calculated. Intervortex venous anastomosis, MNV subtypes, and the main trunk appearance on OCT angiography (OCTA) were evaluated. Features with an area under the receiver operating characteristic curve (AUC) above 0.75 were defined as major, while those between 0.60 and 0.75 were defined as minor differentiating features. Five features met the criteria for major differentiating features: age ≤60 years, SFCT ≥ 254 µm in the diseased eye, CVI ≥ 72

Polypoidal choroidal vasculopathy (PCV) is an ocular condition predominantly affecting elderly individuals of Asian descent, characterized by the presence of polypoidal lesions and branching neovascular networks in the sub-retinal pigment epithelium (RPE) space. It has garnered increased attention for its potential differences from neovascular age-related macular degeneration. Genetic studies have identified specific genetic markers associated with PCV. Advances in imaging techniques, particularly optical coherence tomography (OCT) and OCT angiography, have significantly enhanced the diagnosis of PCV and our insight into its unique pathogenesis. Treatment strategies for PCV have evolved, with anti-vascular endothelial growth factor (VEGF) monotherapy becoming the primary treatment, and combination therapies including photodynamic therapy showing promising results. Consideration of targets beyond VEGF and the incorporation of artificial intelligence (AI) based analysis strategies may open the door to more personalized, precise, and effective treatments for patients. This review comprehensively discusses the current knowledge and recent advancements in PCV, including its epidemiology, genetics, biomarkers, pathogenesis, diagnosis, and management. It also highlights the need to explore mechanism underlying the higher prevalence of PCV in pigmented races, clarify the roles of pachychoroid and pachydrusen in PCV pathogenesis, and develop animal models that can better recapitulate the disease's pathological features.

PURPOSE:There are major gaps in our knowledge of hereditary ocular conditions in the Asia-Pacific population, which comprises approximately 60% of the world's population. Therefore, a concerted regional effort is urgently needed to close this critical knowledge gap and apply precision medicine technology to improve the quality of lives of these patients in the Asia-Pacific region.DESIGN:Multi-national, multi-center collaborative network.METHODS:The Research Standing Committee of the Asia-Pacific Academy of Ophthalmology and the Asia-Pacific Society of Eye Genetics fostered this research collaboration, which brings together renowned institutions and experts for inherited eye diseases in the Asia-Pacific region. The immediate priority of the network will be inherited retinal diseases (IRDs), where there is a lack of detailed characterization of these conditions and in the number of established registries.RESULTS:The network comprises 55 members from 35 centers, spanning 12 countries and regions, including Australia, China, India, Indonesia, Japan, South Korea, Malaysia, Nepal, Philippines, Singapore, Taiwan, and Thailand. The steering committee comprises ophthalmologists with experience in consortia for eye diseases in the Asia-Pacific region, leading ophthalmologists and vision scientists in the field of IRDs internationally, and ophthalmic geneticists.CONCLUSIONS:The Asia Pacific Inherited Eye Disease (APIED) network aims to (1) improve genotyping capabilities and expertise to increase early and accurate genetic diagnosis of IRDs, (2) harmonise deep phenotyping practices and utilization of ontological terms, and (3) establish high-quality, multi-user, federated disease registries that will facilitate patient care, genetic counseling, and research of IRDs regionally and internationally.

INTRODUCTION:Neovascular age-related macular degeneration is a global public-health concern, associated with a considerable burden to individuals, healthcare systems, and society. The objective of this study was to understand different perspectives on the challenges associated with the clinical management of neovascular age-related macular degeneration, which could elucidate measures to comprehensively improve clinical care and outcomes. METHODS:A survey was carried out of patients with neovascular age-related macular degeneration, their providers, and clinic staff in 77 clinics across 24 countries on six continents, from a diverse range of healthcare systems, settings, and reimbursement models. Surveys comprised a series of single/multiple-response questions completed anonymously. Data gathered included patient personal characteristics, appointment attendance challenges, treatment experiences, and opportunities to improve support. Provider and clinic staff surveys asked similar questions about their perspectives; clinic characteristics were also captured. RESULTS:There were 6425 responses; 4558 patients with neovascular age-related macular degeneration, 659 providers, and 1208 clinic staff. Challenges identified included concern about patient burden to family/friends, high frequency of treatment, difficulties in traveling to appointments, long waiting times, and insufficient comprehension of neovascular age-related macular degeneration. Participants identified logistical (improved financial assistance with treatment and out-of-pocket costs, and appointment reminders), operational (addressing clinic set up to reduce waiting times and improving the amount of time providers spend with patients), and educational (improving quality and provision of patient information and expectation-setting) opportunities to improve care. CONCLUSIONS:The wealth of data generated by this global survey highlights the breadth of challenges associated with clinical management of patients with neovascular age-related macular degeneration. Addressing the opportunities raised could improve patient adherence to treatment and potentially outcomes, reduce appointment burden, and increase clinic capacity.

Practice patterns for neovascular age-related macular degeneration (nAMD) have evolved from the landmark registration trials of vascular endothelial growth factor (VEGF) inhibitors. Non-monthly regimens like treat-and-extend (T&E) have become popular due to their effectiveness in clinical practice. T&E regimens attempt to limit the burden of visits and treatments by allowing progressively longer treatment intervals, but in so doing, are potentially associated with the expense of treating quiescent disease. This is acceptable to many patients and their ophthalmologists but can still be problematic in the real-world. Recent studies have further refined the T&E approach by allowing for quicker and longer extension of treatment intervals when less severe disease is detected. With newer drugs offering increased durability, a shift to longer regular intervals may emerge as a new practice pattern for VEGF inhibitor therapy. This review aims to consolidate the current literature on the most effective treatment patterns and update treatment guidelines based on options that are now available. It also summarises new aspects of nAMD management that may help to further refine current practice.

PURPOSE:The objective of this paper is to shed light on the current landscape of genotyping practices, phenotyping practices and availability of essential vision rehabilitation management for inherited retinal diseases (IRD) in the Asia-Pacific (APAC) Region. METHODS:The 62-item questionnaire was distributed electronically via email. The questions covered five domains: (1) structure of the IRD service and registry/database; (2) genotyping practices; (3) genetic counselling; (4) deep phenotyping practices; (5) low-vision rehabilitation services. RESULTS:The survey was completed by 36 of 45 centres in twelve countries and regions in APAC. Among these centres, 42 % reported managing more than 1000 patients. Notably, 39 % of centres lack an IRD database or registry, and 44 % of centres have tested less than one-quarter of their IRD patients. The majority of centres (67 %) do not have genetic counsellors. While there was consistency in the imaging-based investigations, there was marked heterogeneity for functional testing using electrophysiology and formal perimetry. Only 34 % of centres confirmed the availability of access to low-vision assistive devices. CONCLUSIONS:This study reveals several critical gaps in managing IRDs in the APAC region. These include the lack of IRD database/registry in one-third of centres, a substantial proportion of patients remaining genetically undiagnosed, and limited availability of genetic counsellors. The findings also underscore a need to harmonise investigations for evaluating retinal function and identify areas for improvement in the provision of low-vision rehabilitation services.

Ziel dieser Übersichtsarbeit ist es, die gemeinsamen Merkmale und Prognosen verschiedener Subtypen der neovaskulären altersbedingten Makuladegeneration (nAMD) zu identifizieren. Wir schlagen zudem Empfehlungen vor, wie Behandlungen auf den Subtyp der Neogefäße angepasst werden können, um die Ergebnisse bei den Patienten zu optimieren. Die Autoren sind ausgewählte Mitglieder der Vision Academy und trafen sich, um die Behandlungsergebnisse bei nAMD je nach Subtyp der makulären Neovaskularisation (MNV) zu besprechen, wobei Ergebnisse einer Literaturrecherche in der Datenbank PubMed (Abrufdatum: März 2019) herangezogen wurden. Dieser Übersichtsartikel fasst die Empfehlungen der Vision Academy dahingehend zusammen, wie die Charakterisierung von MNV-Subtypen die Behandlungsergebnisse bei nAMD verbessern kann. Die Identifizierung der MNV-Subtypen wurde durch die Einführung multimodaler Bildgebung erleichtert. Zusammengenommen ermöglichen die Ergebnisse der Fluoreszein-Angiografie, der Indocyaningrün-Angiografie und der optischen Kohärenztomografie im Spektralbereich es, die Bestimmung des MNV-Subtyps zu verbessern und zu standardisieren. Bisher wurden in der Literatur 3 Subtypen beschrieben, die bei der Bildgebung spezifische Charakteristika aufweisen. Die Typ-1-MNV weist bessere langfristige Ergebnisse auf, benötigt jedoch in der Regel eine höhere Dosierung des Wirkstoffs gegen den vaskulären endothelialen Wachstumsfaktor (VEGF). Die Typ-2-MNV spricht in der Regel schnell auf die Behandlung an, ist jedoch anfälliger für die Entwicklung fibrotischer Narben, die zu schlechteren Ergebnissen führen können. Die Typ-3-MNV reagiert sehr empfindlich auf die Behandlung mit Anti-VEGF-Wirkstoffen, wird jedoch im Vergleich zu anderen Subtypen mit einer höheren Inzidenz einer äußeren Netzhautatrophie assoziiert. Die genaue Bestimmung des MNV-Subtyps liefert Informationen zur Prognose und hilft, den Umgang mit Patienten mit nAMD zu optimieren.

AIM: To derive a Malaysia guideline and consensus as part of the Malaysia Retina Group’s efforts for diagnosis, treatment, and best practices of diabetic macular edema (DME). The experts’ panel suggests that the treatment algorithm to be divided into groups according to involvement the central macula. The purpose of DME therapy is to improve edema and achieve the best visual results with the least amount of treatment load. METHODS: On two different occasions, a panel of 14 retinal specialists from Malaysia, together with an external expert, responded to a questionnaire on management of DME. A consensus was sought by voting after compiling, analyzing and discussion on first-phase replies on the round table discussion. A recommendation was deemed to have attained consensus when 12 out of the 14 panellists (85%) agreed with it. RESULTS: The terms target response, adequate response, nonresponse, and inadequate response were developed when the DME patients’ treatment responses were first characterized. The panelists reached agreement on a number of DME treatment-related issues, including the need to classify patients prior to treatment, first-line treatment options, the right time to switch between treatment modalities, and side effects associated with steroids. From this agreement, recommendations were derived and a treatment algorithm was created. CONCLUSION: A detail and comprehensive treatment algorithm by Malaysia Retina Group for the Malaysian population provides guidance for treatment allocation of patients with DME.

There have been recent advances in basic research and clinical studies in polypoidal choroidal vasculopathy (PCV). A recent, large-scale, population-based study found systemic factors, such as male gender and smoking, were associated with PCV, and a recent systematic review reported plasma C-reactive protein, a systemic biomarker, was associated with PCV. Growing evidence points to an association between pachydrusen, recently proposed extracellular deposits associated with the thick choroid, and the risk of development of PCV. Many recent studies on diagnosis of PCV have focused on applying criteria from noninvasive multimodal retinal imaging without requirement of indocyanine green angiography. There have been attempts to develop deep learning models, a recent subset of artificial intelligence, for detecting PCV from different types of retinal imaging modality. Some of these deep learning models were found to have high performance when they were trained and tested on color retinal images with corresponding images from optical coherence tomography. The treatment of PCV is either a combination therapy using verteporfin photodynamic therapy and anti-vascular endothelial growth factor (VEGF), or anti-VEGF monotherapy, often used with a treat-and-extend regimen. New anti-VEGF agents may provide more durable treatment with similar efficacy, compared with existing anti-VEGF agents. It is not known if they can induce greater closure of polypoidal lesions, in which case, combination therapy may still be a mainstay. Recent evidence supports long-term follow-up of patients with PCV after treatment for early detection of recurrence, particularly in patients with incomplete closure of polypoidal lesions.

To develop a consensus nomenclature for reporting OCT angiography (OCTA) findings in retinal vascular disease (e.g., diabetic retinopathy, retinal vein occlusion) by international experts.Delphi-based survey.Twenty-five retinal vascular disease and OCTA imaging experts.A Delphi method of consensus development was used, comprising 2 rounds of online questionnaires, followed by a face-to-face meeting conducted virtually. Twenty-five experts in retinal vascular disease and retinal OCTA imaging were selected to constitute the OCTA Nomenclature in Delphi Study Group for retinal vascular disease. The 4 main areas of consensus were: definition of the parameters of "wide-field (WF)" OCTA, measurement of decreased vascular flow on conventional and WF-OCTA, nomenclature of OCTA findings, and OCTA in retinal vascular disease management and staging. The study end point was defined by the degree of consensus for each question: "strong consensus" was defined as ≥85% agreement, "consensus" as 80% to 84%, and "near consensus" as 70% to 79%.Consensus and near consensus on OCTA nomenclature in retinal vascular disease.A consensus was reached that a meaningful change in percentage of flow on WF-OCTA imaging should be an increase or decrease ≥30% of the absolute imaged area of flow signal and that a "large area" of WF-OCTA reduced flow signal should also be defined as ≥30% of the absolute imaged area. The presence of new vessels and intraretinal microvascular abnormalities, the foveal avascular zone parameters, the presence and amount of "no-flow areas," and the assessment of vessel density in various retinal layers should be added for the staging and classification of diabetic retinopathy. Decreased flow ≥30% of the absolute imaged area should define an ischemic central retinal vein occlusion. Several other items did not meet consensus requirements or were rejected in the final discussion round.This study provides international consensus recommendations for reporting OCTA findings in retinal vascular disease, which may help to improve the interpretability and description in clinic and clinical trials. Further validation in these settings is warranted and ongoing. Efforts are continuing to address unresolved questions.

Objective: To compare the outcomes of brolucizumab versus aflibercept in patients with neovascular agerelated macular degeneration with early residual fluid in the HAWK and HARRIER studies. Design: Post hoc analysis using pooled data from the phase 3 studies HAWK (NCT02307682) and HARRIER (NCT02434328). Participants: The early residual fluid cohort for this post hoc analysis consisted of patients treated with either brolucizumab 6 mg (n = 730 patients) or aflibercept 2 mg (n = 729 patients) and who had the presence of intraretinal fluid (IRF), subretinal fluid (SRF), or both verified by spectral-domain OCT at the week 12 clinic visit. Methods: After 3 initial monthly doses, patients treated with brolucizumab received injections every 12 weeks (q12w) or every 8 weeks (q8w), depending on the neovascular age-related macular degeneration disease activity, whereas patients treated with aflibercept received fixed q8w dosing. Main Outcome Measures: The mean change in best-corrected visual acuity and central subfield thickness (CST) from the baseline to that at weeks 48 and 96, the proportion of patients treated with brolucizumab remaining on q12w dosing to week 96, and the change in fluid status at weeks 48 and 96. Results: All analyses were based on 149 of 730 (20.4%) patients treated with brolucizumab and 217 of 729 (29.8%) patients treated with aflibercept with spectral-domain OCTeverified IRF, SRF, or both at their week 12 visit. The best-corrected visual acuity improvements from baseline at weeks 48 and 96 were numerically better for brolucizumab than for aflibercept (least square mean [standard error] 7.9 +/- 1.1 vs. 4.6 +/- 0.9 and 7.4 +/- 1.3 vs. 2.9 +/- 1.1 letters, respectively). The CST reductions from baseline at weeks 48 and 96 were consistently greater with brolucizumab than with aflibercept (least square mean [standard error], mm: -194.9 +/- 13.7 vs. -123.9 +/- 11.3; and -201.1 +/- 14.5 vs. -134.2 +/- 12.0, respectively). At weeks 48 and 96, patients treated with brolucizumab had a 40.4% and 31.3% probability of remaining on q12w dosing intervals, respectively. Fewer patients treated with brolucizumab had remaining IRF, SRF, or both at weeks 48 and 96 than patients treated with aflibercept (59.1% vs. 75.1% and 49.0% vs. 60.4%, respectively). Conclusions: In patients with early residual fluid, defined as spectral-domain OCTeverified IRF, SRF, or both at the week 12 clinic visit, brolucizumab resolved the early residual fluid and reduced CST more effectively than aflibercept, resulting in greater best-corrected visual acuity improvements through week 96 of anti-VEGF treatment. (C) 2022 by the American Academy of Ophthalmology

The aim of this review is to identify the common characteristics and prognoses of different subtypes of neovascular age-related macular degeneration (nAMD). We also propose recommendations on how to tailor treatments to the subtype of neovessels to optimise patient outcomes. The authors, selected members of the Vision Academy, met to discuss treatment outcomes in nAMD according to macular neovascularisation (MNV) subtypes, using evidence from a literature search conducted on the PubMed database (cut-off date: March 2019). This review article summarises the recommendations of the Vision Academy on how the characterisation of MNV subtypes can optimise treatment outcomes in nAMD. The identification of MNV subtypes has been facilitated by the advent of multimodal imaging. Findings from fluorescein angiography, indocyanine green angiography and spectral-domain optical coherence tomography collectively help refine and standardise the determination of the MNV subtype. To date, three subtypes have been described in the literature and have specific characteristics, as identified by imaging. Type 1 MNV is associated with better long-term outcomes but usually requires more intense anti-vascular endothelial growth factor dosing. Type 2 MNV typically responds quickly to treatment but is more prone to the development of fibrotic scars, which may be associated with poorer outcomes. Type 3 MNV tends to be highly sensitive to anti-vascular endothelial growth factor treatment but may be associated with a higher incidence of outer retinal atrophy, compared with other subtypes. Accurately assessing the MNV subtype provides information on prognosis and helps to optimise the management of patients with nAMD.

PURPOSE:To develop a consensus nomenclature for reporting OCT angiography (OCTA) findings in retinal vascular disease (e.g., diabetic retinopathy, retinal vein occlusion) by international experts. DESIGN:Delphi-based survey. SUBJECTS, PARTICIPANTS, AND/OR CONTROLS:Twenty-five retinal vascular disease and OCTA imaging experts. METHODS, INTERVENTION, OR TESTING:A Delphi method of consensus development was used, comprising 2 rounds of online questionnaires, followed by a face-to-face meeting conducted virtually. Twenty-five experts in retinal vascular disease and retinal OCTA imaging were selected to constitute the OCTA Nomenclature in Delphi Study Group for retinal vascular disease. The 4 main areas of consensus were: definition of the parameters of "wide-field (WF)" OCTA, measurement of decreased vascular flow on conventional and WF-OCTA, nomenclature of OCTA findings, and OCTA in retinal vascular disease management and staging. The study end point was defined by the degree of consensus for each question: "strong consensus" was defined as ≥85% agreement, "consensus" as 80% to 84%, and "near consensus" as 70% to 79%. MAIN OUTCOME MEASURES:Consensus and near consensus on OCTA nomenclature in retinal vascular disease. RESULTS:A consensus was reached that a meaningful change in percentage of flow on WF-OCTA imaging should be an increase or decrease ≥30% of the absolute imaged area of flow signal and that a "large area" of WF-OCTA reduced flow signal should also be defined as ≥30% of the absolute imaged area. The presence of new vessels and intraretinal microvascular abnormalities, the foveal avascular zone parameters, the presence and amount of "no-flow areas," and the assessment of vessel density in various retinal layers should be added for the staging and classification of diabetic retinopathy. Decreased flow ≥30% of the absolute imaged area should define an ischemic central retinal vein occlusion. Several other items did not meet consensus requirements or were rejected in the final discussion round. CONCLUSIONS:This study provides international consensus recommendations for reporting OCTA findings in retinal vascular disease, which may help to improve the interpretability and description in clinic and clinical trials. Further validation in these settings is warranted and ongoing. Efforts are continuing to address unresolved questions.