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Catarina Castro,1 João Heitor Marques,1 Nisa Silva,1 Ana Carolina Abreu,1 Maria João Furtado,1,2 Miguel Lume1 1Department of Ophthalmology, Centro Hospitalar Universitário de Santo António, Porto, Portugal; 2Department of Ophthalmology, Instituto de Ciências Biomédicas Abel Salazar – Universidade do Porto, Porto, PortugalCorrespondence: Catarina Castro, Largo Prof. Abel Salazar, Porto, 4099-001, Portugal, Tel +351222077500, Email catarinamscastro@gmail.comPurpose: To evaluate the agreement between conventional fundus photography (CFP) and multicolor fundus imaging (MFI) for the detection of lesions of diabetic retinopathy (DR) and retinal vein occlusion (RVO).Methods: Cross-sectional analysis of eyes with DR or RVO who underwent CFP and MFI. All images were independently analyzed by two observers (O1 and O2), and the evaluated lesions were classified as “present” or “absent”. Then, a paired comparison between both exams of the same eye was performed, to assess which made it easier to detect the lesions.Results: Considering DR, the agreement was substantial for cotton wool spots and photocoagulation scars for both observers (O1: κ=0.75 and κ=0.67; O2: κ=0.71 and κ=0.64, respectively) and for hard exudates for O1 (κ=0.80). These lesions were detected more frequently on MFI. Regarding RVO, the agreement was considered substantial for venous sheathing by O1 (κ=0.64) and moderate for optociliary shunts by O2 (κ=0.60). Optociliary shunts were detected more frequently in CPF by both observers and venous sheathing on MFI by O1. For microaneurysms, retinal hemorrhages, retinal neovascularization, and proliferative membranes, in DR, and retinal hemorrhages, venous engorgement, and retinal neovascularization in RVO, the agreement was almost perfect (κ> 0.82). In the paired analysis, both observers considered that, in DR, microaneurysms and retinal hemorrhages were easier to detect on CFP and that retinal neovascularization, cotton wool spots, and photocoagulation scars were easier to identify on MFI. Regarding RVO, optocilliary shunts were easier to identify on CFP and venous engorgement on MFI.Conclusion: The agreement of MFI and CFP was substantial to almost perfect for most lesions. MFI seems better to detect cotton wool spots and photocoagulations scars in DR and venous sheathing in RVO. Optocilliary shunts seem easier to detect on CFP.Keywords: diabetic retinopathy, retinal vein occlusion, multicolor, color fundus photography

João Leite,1 Ana Carolina Abreu,1 Maria João Furtado,1,2 Miguel Lume1 1Department of Ophthalmology, Centro Hospitalar Universitário de Santo António, Oporto, Portugal; 2ICBAS - Instituto de Ciências Biomédicas Abel Salazar, Oporto, PortugalCorrespondence: João Leite, Ophthalmology, Centro Hospitalar Universitário de Santo António, Largo Do Prof. Abel Salazar, Oporto, 4099-001, Portugal, Tel +351916523942, Email leite.jmo@gmail.com; u13456@chporto.min-saude.ptBackground: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) disease was first reported in 2019 and was initially associated with respiratory pathology. With the improvement of knowledge about this disease, it was noticed that, among other symptoms, some patients presented visual acuity changes associated with retinal vascular changes, mainly associated with thrombotic phenomena. Later, with the development of vaccines against SARS-CoV-2 disease, cases of visual acuity alterations secondary to thrombotic phenomena were also reported.Case Presentation: In this article, a series of clinical cases with retinal vascular alterations after COVID-19 infection and vaccination are described.Conclusion: COVID-19 infection and vaccination increase the risk of retinal vascular events. The purpose of this article is to present a set of clinical cases with various manifestations of vascular changes in the retina associated with COVID-19 infection and COVID-19 vaccination observed in the Department of Ophthalmology of Centro Hospitalar Universitário de Santo António, in Porto, Portugal.Keywords: COVID-19 infection, COVID-19 vaccination, retinal vascular events

Rita Vieira, Maria João Furtado Ophthalmology Department of Centro Hospitalar Universitário do Porto (CHUPorto), Oporto, PortugalCorrespondence: Rita Vieira, Ophthalmology Department of Centro Hospitalar Universitário do Porto (CHUPorto)- Largo do Prof. Abel Salazar, Oporto, 4099-001, Portugal, Tel +351 913748812, Email anarita.vieira1693@gmail.comPurpose: To describe a case of long-term poorly treated bilateral acute syphilitic posterior placoid chorioretinitis (ASPPC) complicated with choroidal neovascularization, with a multimodal imaging approach.Patients and Methods: Retrospective case report. Analysis of patient’s clinical data.Case Report: A healthy 66-year-old man complained of decreased visual acuity in the right eye (RE). He had a past history of bilateral exudative maculopathy of unknown etiology, lasting for more than 10 years and leading to severe and irreversible vision loss on the left eye. The corrected distance visual acuity (CDVA) was 20/63 on the RE and < 20/400 on the left eye (LE). On slit-lamp, no anterior chamber reaction was observed, a vitreous haze was present on the RE as well as large, bilateral yellowish lesions in the macula, with exuberant macular atrophy, particularly on the LE. These well-defined lesions were confined to the posterior pole. The spectral domain optical coherence tomography (SD-OCT) showed a flat retinal pigmented epithelium (RPE) detachment with hyperreflective material beneath the RPE and cystoid macular edema on the RE and an exuberant macular atrophy on the LE, with both eyes showing anomalous enlargement of choroidal vessels. The fluorescein angiography showed general hypofluorescence and indocyanine green angiography revealed a heterogeneous pattern. The OCT angiography (OCTA) of the RE demonstrated an anomalous vascular network related to the presence of choroidal neovascularization (CNV). An etiological study was performed and a positive treponemal test (TPHA) was found. The patient experienced visual acuity improvement to 20/25 after systemic treatment with Penicillin and anti-VEGF injections.Conclusion: Clinical data and multimodal imaging information suggest that this case represents a complication of long-term untreated bilateral acute syphilitic posterior placoid chorioretinitis (ASPPC) with associated choroidal neovascularization. Once the RE presented signs of active disease, besides intravitreal anti-VEGF, treatment of the underlying disease was essential for visual recovery.Keywords: ASPPC, syphilis, neovascularization, ocular syphilis

Purpose: To assess the choroidal status of Systemic Lupus Erythematosus (SLE) patients using Optical Coherence Tomography (OCT) and OCT-Angiography., Methods: SLE patients with disease duration < 10 years, no disease activity and no ocular involvement were recruited and cross-sectionally evaluated. A demographically similar cohort of healthy subjects was used for comparison. The main outcome is choroidal vascularity index (CVI). As secondary outcomes, choriocapillaris parameters and choroidal thickness (CT) were evaluated., Results: Forty eyes of 40 subjects (20 SLE patients and 20 healthy subjects) were studied with a mean ± SD age of 36.7 ± 9.9 years. In the SLE group, the mean ± SD duration of disease was 7.35 ± 2.21 years. Increased CVI was found in the SLE group (p = 0.022). Considering the choriocapillaris, SLE patients presented a lower number (p = 0.037) and a smaller total area (p = 0.041) of signal voids. No differences between groups were found in CT. For SLE patients, CT at subfoveal, temporal and inferior locations presented a negative moderate correlation with disease duration. A strong correlation between choriocapillaris parameters and age was demonstrated for both groups., Conclusions: This study provides evidence of subclinical choroidal changes in adult SLE patients with inactive disease and no overt ocular manifestation. Increased CVI and fewer and smaller flow voids in choriocapillaris with normal CT suggest increased choroidal vascularity in SLE., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Uveitis is a heterogeneous collection of infrequent diseases, which poses significant challenges to cost-effective research in the field. Medical registries are being increasingly recognized as crucial tools to provide high-quality data, thus enabling prospective clinical research. This paper describes the design and technical structure development of an innovative countrywide electronic medical record for uveitis, Uveite.pt, and gives an overview of the cohort registered since its foundation, March 2020.Uveite.pt is an electronic medical record platform developed by the Portuguese Ocular Inflammation Group (POIG), a scientific committee of the Portuguese Ophthalmology Society. This is a nationwide customized web-based platform for uveitis patients useful for both clinical practice and real-world-based research, working as a central repository and reporting tool for uveitis. This paper describes the technical principles, the design and the development of a web-based interoperable registry for uveitis in Portugal and provides an overview of more than 400 patients registered in the first 18 months since inception.In infrequent diseases, the existence of registries enables to gather evidence and increase research possibilities to clinicians. The adoption of this platform enables standardization and improvement of clinical practice in uveitis. It is useful to apprehend the repercussion of medical and surgical treatments in uveitis and scleritis, supporting clinicians in the strict monitoring of drug adverse reactions and surgical outcomes.

Systemic lupus erythematosus (SLE) is a chronic, systemic, autoimmune connective tissue disease that affects several vascular territories. We sought to assess the role of optical coherence tomography angiography in detecting subclinical microvascular alterations in SLE patients. PubMed, Scopus, and Web of Science databases were systematically searched until January 21, 2023. Studies using optical coherence tomography angiography as a primary diagnostic method to evaluate the macular microvasculature of SLE patients versus healthy controls were included. Primary outcomes were macular vessel density and foveal zone parameters. A meta-analysis was performed using a random-effects model. Of 301 screened abstracts, 15 were found eligible, enrolling 1,246 eyes from 1,013 patients. SLE patients presented a reduction of macular vessel density at both plexuses in all zones (whole scan, fovea, parafovea, and perifovea), and of foveal density compared with healthy controls. No differences were found at foveal avascular zone parameters. SLE patients presented a reduction of macular vessel density without signs or symptoms of SLE ocular involvement. Optical coherence tomography angiography application for the assessment of subclinical microvascular changes needs to be elucidated with longitudinal studies., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest to declare., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Purpose: To report the case and multimodal imaging findings of a healthy young woman who developed paracentral acute middle maculopathy 9 weeks after COVID-19., Methods: Case report. Ultra-widefield fundus photography, macular spectral domain optical coherence tomography, fluorescein angiography, and optical coherence tomography angiography were performed., Results: A 36-year-old woman went to the emergency department with sudden, painless, left eye vision loss. The only relevant medical history was COVID-19 9 weeks before. The best-corrected visual acuity was 20/200, a left eye relative afferent pupillary defect was present, and superficial hemorrhages throughout the macular area and peripheral retina were found. Nearly 4 hours after admission, the left eye best-corrected visual acuity recovered to 20/20 without relative afferent pupillary defect. Five days after presentation in the emergency department, the patient returned with recurrent left eye vision loss, with spontaneous recovery within 12 hours. Macular spectral domain optical coherence tomography revealed hyperreflectivity of the inner plexiform and inner nuclear layers, and the diagnosis of paracentral acute middle maculopathy was established. The patient started oral acetylsalicylic acid and oral prednisolone. The patient did not report any new episodes of vision loss, and there was a progressive resolution of abnormal fundus findings., Conclusion: SARS-CoV-2 infection increases the risk of vascular thrombotic events with possible involvement of the retinal circulation, and paracentral acute middle maculopathy may present as a possible complication. Ophthalmologists should be able to recognize it promptly through multimodal imaging findings.

Background: Retinal toxicity with long-term hydroxychloroquine (HCQ) treatment is a major concern. This systematic review aims to assess the application of optical coherence tomography angiography (OCTA) to detect microvascular alterations in patients under HCQ., Methods: PubMed, Scopus, Web of Science, and Cochrane Library databases were systematically searched until January 14, 2023. Studies using OCTA as a primary diagnostic method to evaluate the macular microvasculature of HCQ users were included. Primary outcomes were macular vessel density (VD) and foveal avascular zone (FAZ) at the superficial (SCP) and deep (DCP) capillary plexus. Meta-analysis was performed using a random-effects model., Results: Of 211 screened abstracts, 13 were found eligible, enrolling 989 eyes from 778 patients. High-risk patients due to longer duration of treatment presented lower VD in the retinal microvasculature than those with low-risk in SCP (P = 0.02 in fovea; P = 0.004 in parafovea) and in DCP (P = 0.007 in fovea; P = 0.01 in parafovea). When compared with healthy controls, HCQ users had lower VD in both plexus-no quantitative synthesis was presented., Conclusions: Microvascular changes were found in autoimmune patients under HCQ treatment without any documented retinopathy. However, the evidence produced so far does not allow to draw conclusion concerning the effect of drug as studies were not controlled for disease duration., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: Intravitreal injections (IVIs) are currently the most common intraocular surgical procedure worldwide. Some studies have reported a higher risk of intraoperative complications, namely, posterior capsular rupture (PCR), during cataract surgery. The aim of this retrospective and observational study, conducted at Department of Ophthalmology, Centro Hospitalar Universitário do Porto, was to assess the risk of PCR during cataract surgery in eyes previously treated with IVIs with anti-vascular endothelial growth factor (anti-VEGF) and/or corticosteroids., Methods: Eyes undergoing cataract surgery between June 2019 and May 2021 were included. Combined surgeries, such as glaucoma surgery and pars plana vitrectomy, were excluded. The occurrence of PCR during cataract surgery in treated and previously untreated eyes with IVI was analyzed., Results: A total of 5,813 cataract surgeries were analyzed; 4.1% of the cases had previously undergone IVI. The PCR rate in cataract surgery was 1.8%: 6.7% in eyes previously treated with IVI and 1.6% without previous IVI (OR = 4.5, 95% CI: 2.6-7.7, p < 0.001). The combined therapy (anti-VEGF with corticosteroids) presents a higher risk compared to the two therapies alone as monotherapy (OR = 11.6, 95% CI: 4.7-28.5, p < 0.001), as well as treated eyes treated with ≥10 IVI (OR = 2.1, 95% CI: 0.8-6.1, p = 0.144) and a time interval between the last IVI and cataract surgery was ≤6 months (OR = 1.9, 95% CI: 0.6-6.1, p = 0.296)., Conclusion: These results demonstrate that eyes that require IV treatment prior to cataract surgery are at increased risk of CPA during cataract surgery, and careful assessment of the characteristics of the cataract and posterior capsule is critical., (© 2022 S. Karger AG, Basel.)

Purpose: This study aimed to evaluate the long-term effectiveness of intravitreal anti-vascular endothelial growth factor (VEGF) injections in the treatment of choroidal neovascularization (CNV) associated with angioid streaks., Methods: Multicenter retrospective cohort study, including eyes with CNV secondary to angioid streaks treated with anti-VEGF injections, were performed. Best-corrected visual acuity (BCVA) in ETDRS letters; qualitative and quantitative (foveal thickness) OCT parameters; anti-VEGF type; and number of injections were collected at baseline and at 3, 6, 12, 24, 36, 48, 60, and 72 months., Results: Thirty-nine eyes from 29 patients, 17 (58.6%) females, were included. The mean follow-up time was 69.4 ± 34.5 months. BCVA was 59.3 ± 23.3 letters at baseline and 63.7 ± 21.9 letters at 48 months. At 3 months, BCVA improved 6.9 ± 11.7 letters ( P =0.003). Then, BCVA remained stable. The mean foveal thickness decreased from 343.3 ± 120.2  μ m at baseline to 268.3 ± 65.4 at 48 months ( P =0.021). The mean number of injections was 4.6 ± 2.1 at 12 months, decreasing to 1.7 ± 2.4 injections between 36 and 48 months ( P =0.093)., Conclusion: This real-world study suggests that the functional and morphologic response to anti-VEGF therapy for CNV related to angioid streaks is generally satisfactory and maintained in the long term., Competing Interests: Manuel Falcão has participated in advisory boards for Bayer and has received travel grants from Novartis, Alimera, and Allergan. Ângela Carneiro has participated in advisory boards for Alcon, Bayer, Novartis, Alimera, Allergan, and Roche. The other authors report no conflicts of interest in this work., (Copyright © 2022 Sónia Torres-Costa et al.)

Aim: To develop the first Ophthalmology joint guidelines with Paediatric Rheumatology with recommendations on the screening, monitoring and medical treatment of juvenile idiopathic arthritis-associated uveitis (JIA-U), endorsed by the Portuguese Society of Ophthalmology (SPO)., Methods: A systematic literature review was conducted to include publications up to July 14th 2020, with no language restrictions, in order to include all the international position papers/guidelines concerning the medical management of JIA-U and randomised clinical trials assessing the efficacy and safety of medical treatment in this field. We searched through MEDLINE (PubMed), Scopus, Web of Science and Cochrane Library. The Delphi modified technique to generate consensus was used. Preliminary evidence statements were subject to an anonymous agreement assessment and discussion process using an online survey, followed by further discussion and update at a national meeting. A draft of the manuscript with all recommendations was then circulated among all participants and suggestions were incorporated. The final version was again circulated before publication., Results: Twenty-six recommendations were developed focusing on the following topics: general management (3), screening and follow-up of uveitis (4), treatment (17) and health education in JIA-U among patients and families (2)., Conclusion: These guidelines were designed to support the shared medical management of patients with JIA-U and emphasize the need for a multidisciplinary approach between Ophthalmology and Paediatric Rheumatology regarding the comprehensive care of JIA-U. We acknowledge that updating these recommendations will be warranted in the future, as more evidence becomes available., Key-Words: juvenile idiopathic arthritis, uveitis, biological treatment, conventional immunosuppressive treatment, multidisciplinary management, guidelines, consensus, review, Delphi Technique.