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Purpose: To describe a case of subretinal hemorrhage due to the Valsalva maneuver in a patient with no underlying chorioretinal disease. Methods: History and clinical examination, optical coherence tomography (OCT), fluorescein, and indocyanine green angiography. Results: We report a case of a 35-year-old man with a 4-day history of central vision loss in the left eye (OS) after a vomiting episode. His best-corrected visual acuity was 20/200 in OS. Fundus examination revealed a subretinal hemorrhage in the posterior pole, associated with a small preretinal hemorrhage in the superotemporal arcade. OCT confirmed the presence of a thick submacular hemorrhage and a focal hemorrhage beneath the inner limiting membrane along the superotemporal arcade. The patient was submitted to pars plana vitrectomy (PPV), subretinal injection of tissue plasminogen activator (tPA), and air tamponade on the following day. Most of the submacular hemorrhage was displaced, resulting in a satisfactory visual outcome (BCVA = 20/30 after 1 month of surgery). Fluorescein and indocyanine green angiography excluded conditions such as retinal arterial macroaneurysm, polypoidal chorioretinopathy, and choroidal neovascularization. Conclusion: Although rare, Valsalva retinopathy may present with submacular hemorrhage in a patient with no underlying chorioretinal disease. PPV and subretinal tPA injection may provide a good visual outcome.

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PURPOSE: To assess the relationship between macular choroidal thickness (CT) measurements and retinal sensitivity (RS) in eyes with myopia and different stages of myopic maculopathy. METHODS: A masked, cross-sectional, and consecutive study involving patients with emmetropia/myopia (control group) and high myopia (HM) eyes. Automated choroidal thickness (CT) and manual outer retinal layer (ORL) thickness were acquired using swept-source optical coherence tomography, while retinal sensitivity (RS) assessed by microperimetry (MP3) in all regions of the macular Early Treatment Diabetic Retinopathy Study (ETDRS) grid. Comparisons were made between groups, and correlations were performed among these measurements, demographic and ocular parameters and myopic maculopathy classification. RESULTS: A total of 37 (74 eyes) patients were included in the study. The mean age was 39 ± 13 years, and 28 patients (76%) were female. HM eyes exhibited inferior best-corrected visual acuity and a more advanced myopic maculopathy classification compared to the control group. The mean macular CT were 255 and 179 µm in the control and HM eyes (P < 0.001), respectively. In the HM eyes, superior ETDRS region presented the greatest values. Mean RS in control and HM groups was 28 and 24 dB (P = 0.001), respectively. Inner temporal followed by superior, were the regions of higher RS. Mean ORL thickness was 83 and 79 µm (P < 0.001), in the control and HM groups, respectively. The inner temporal ETDRS region presented the thickest measure. CT correlated significantly with RS (r = 0.41, P < 0.001) and ORL thickness, (r = 0.58, P < 0.001), which also correlated with RS (r = 0.40, P < 0.001). Spherical equivalent, axial length and myopic maculopathy stage were the parameters that most correlated with CT, RS and ORL thickness. For every 100 µm increase in thickening of CT there was an average increase of 3.4 µm in ORL thickness and 2.7 dB in RS. Myopic maculopathy classification demonstrated influence only with CT. CONCLUSION: Myopia degree is related to ORL and choroidal thinning and deterioration of retinal sensitivity in some ETDRS regions of the macula. Choroidal thinning is associated to with a decline of retinal sensitivity, thinning of ORL, and worsening of myopic maculopathy classification, so new treatments are necessary to prevent myopia progression.

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PURPOSE: To describe two cases of concentric macular rings (CMR) sign in patients with combined hamartoma of retina and retinal pigment epithelium (CHRRPE). METHODS: History and clinical examination, spectral-domain optical coherence tomography (SD-OCT), and optic coherence tomography angiography (OCTA). RESULTS: The first patient was a 26-year-old woman with clinical diagnosis of Neurofibromatosis type 2. The Best-Corrected Visual Acuity (BCVA) was 20/25 in the right eye (OD) and 20/20 in the left eye (OS). She presented a subtle grayish lesion in the macular region of OD. On macular SD-OCT, there was a thickened and disorganized retina, suggestive of CHRRPE. In addition, at the parafoveal region, there was a dentate pattern in the outer plexiform layer (OPL) and Henle fiber layer (HFL) on cross-sectional SD-OCT. OCTA showed no foveal avascular zone (FAZ) in OD and Optos ultra-wide-field revealed CMR sign in this eye. The second patient was a 14-year-old boy, with diabetes mellitus type 1. His BCVA was 20/25 in OD and 20/20 in OS. He had a subtle whitish lesion in the macular region of OD, with SD-OCT findings suggestive of CHRRPE. Similar to the first case, there was a dentate pattern in the OPL and HFL, on SD-OCT. In OD, blue reflectance images exhibited the CMR sign, and OCTA showed absent FAZ. CONCLUSION: CHRRPE may be related to changes in the OPL and HFL interface and may present the CMR sign.

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PURPOSE: To compare the relationship between macular ganglion cell layer (mGCL) thickness and 10-2 visual field (VF) sensitivity using different stimulus sizes in patients with temporal hemianopia from chiasmal compression. METHODS: A cross-sectional study was conducted involving 30 eyes from 25 patients with temporal VF loss on 24-2 SITA standard automated perimetry due to previous chiasmal compression and 30 healthy eyes (23 controls). Optical coherence tomography (OCT) of the macular area and 10-2 VF testing using Goldmann stimulus size I (GI), II (GII), and III (GIII) were performed in the Octopus 900 perimeter. For the sake of analysis, mGCL thickness and VF data were segregated into four quadrants (two temporal and two nasal) and two halves (temporal and nasal) centered on the fovea, in order to evaluate separately both the severely affected nasal hemi-retina corresponding to the temporal VF sectors and the subclinically affected temporal hemi-retina corresponding to the nasal VF sectors. Data from patients and controls were compared using generalized estimated equations. The discrimination ability of GI, GII, and GIII was evaluated, as was the correlation between mGCL and 10-2 VF sensitivity using GI, GII, and GIII. RESULTS: All mGCL parameters in the nasal and temporal halves of the retina were significantly reduced in patients compared to controls. 10-2 VF test sensitivity using GI, GII, and GIII was significantly lower in patients than in controls (p≤0.008) for all parameters, except the three nasal divisions when using GI (p = 0.41, 0.07 and 0.18) Significant correlations were found between temporal VF sectors (all stimulus sizes) and the corresponding nasal mGCL measurements, with similar discrimination ability. Significant correlations were also observed between all three nasal VF divisions and the corresponding temporal mGCL thickness when using stimulus sizes I and II, but not stimulus size III. CONCLUSIONS: On 10-2 VF testing, GII outperformed GI and GIII with regard to discrimination ability and structure-function correlation with mGCL thickness in the subclinically affected nasal part of the VF in patients with chiasmal compression. Our findings suggest that the use of GII can enhance the diagnostic power of 10-2 VF testing in early cases of chiasmal compression, although further studies are necessary to support this conclusion.

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PURPOSE: Tuberous Sclerosis (TS) is a rare, multisystem genetic disease caused by mutations in the TSC1 and TSC2 genes, leading to abnormalities in cell differentiation and proliferation. This study aimed to evaluate the neural integrity of individuals with TS by using Optical Coherence Tomography (OCT) to examine the peripapillary retinal nerve fiber layer (RNFL) thickness and the macular thickness in patients with TS and to compare with healthy controls. METHODS: Peripapillary and macular OCT scans (Optopol Revo NX SD OCT) were performed on 41 eyes from 22 TS patients, divided into two groups based on the presence of retinal hamartomas, and compared to 20 eyes from a control group. The average peripapillary RNFL thickness was measured for each quadrant. The macular total thickness and ganglion cell layer (GCL) + inner plexiform layer (IPL) thickness were measured based on the Early Treatment Diabetic Retinopathy Study (ETDRS) map. All measurements were then compared between the groups and controls. RESULTS: The TS group showed significantly reduced RNFL thickness and macular thickness when compared to the control group. Specifically, patients with retinal hamartomas exhibited an even more pronounced thinning of both RNFL and macular thickness. CONCLUSIONS: These findings suggest that TS patients undergo significant changes in retinal neurodevelopment and experience axonal loss. This finding may have significant prognostic utility regarding central nervous system degeneration in TS, particularly among patients with retinal hamartomas. OCT may serve as a valuable tool for assessing axonal structural abnormalities in TS patients. TRIAL REGISTRATION NUMBER: Not applicable.

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BACKGROUND: To verify the correlation between retinal sensitivity (RS) assessed by the microperimetry (MP) and optical coherence tomography (OCT) parameters measured in eyes submitted to pars-plana vitrectomy (PPV) for idiopathic epiretinal membrane (ERM) treatment. METHODS: 43 patients underwent PPV. Best-corrected visual acuity (BCVA) and OCT imaging were acquired preoperatively and 6 months after surgery. The RS values were recorded 6 months after the surgery. Total macular thickness (TMT) measurements and OCT-evaluated structural findings were also analyzed. The MP examination tested 44 points, with direct topographic correspondence with the OCT-ETDRS map. Correlations between BCVA, RS, and OCT parameters were assessed. RESULTS: TMT measurements in patients were significantly thicker preoperatively and reduced after surgery. All patients demonstrated BCVA improvements after surgery. The RS parameters after surgery were significantly lower in patients. For OCT structural analyses, patients with lower RS at the fovea correlated with the preexisting disorganization of retinal inner layers (DRIL). In addition, lower RS values were associated with DRIL, outer retinal changes (ORC), and intraretinal microcysts after surgery. CONCLUSIONS: The RS values after surgery were significantly lower when compared to controls. The DRIL presence before and after surgery, and microcysts and ORC after surgery were related to worse visual outcomes.

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ABSTRACT This case report presents the details of a 33-year-old female patient who was referred to a specialized retina service because of mild vision loss in her right eye). The patient's visual acuity was 20/25 in right eye and 20/50 in the left eye (; amblyopic); the spherical equivalent was -12.75 diopters (right eye) and -14.75 diopters (left eye). Multimodal retinal imaging showed peripapillary schisis in both the inner and outer retinal layers, grade II posterior vitreous detachment, and a tessellated fundus. Using Humphrey perimetry and MP-3 microperimetry, the functional evaluation indicated macular sensitivity within normal limits and decreased sensitivity in the peripapillary region, especially in right eye. The pattern-re versal visual evoked potential was measured. The N75 and P100 latency and amplitude in right eye were within normal values for checks of 1º. However, the amplitude was low for checks of 15′. Highly myopic patients who have posterior staphyloma that involves the optic nerve are susceptible to posterior hyaloid traction, and the resulting peripapillary vitreous traction may compromise vision.

RESUMO Este relato de caso apresenta um paciente feminino de 33 anos encaminhado para um serviço especializado de retina devido à leve perda de visão em olho direito. A acuidade visual foi de 20/25 no olho direito e 20/50 no olho esquerdo, o equivalente esférico foi de -12,75 dioptrias e -14,75 dioptrias, respectivamente. Avaliações multimodais revelaram isquese peripapilar nas camadas internas e externas da retina, descolamento vítreo posterior grau II e fundo tesselado. Avaliação funcional com perimetria Humphrey e microperimetria MP-3 revelaram sensibilidade macular normais e diminuição da sensibilidade na região peripapilar, especialmente no olho direito. Potencial visual evocado de padrão reverso apresentou no olho direito latência e amplitude N75 e P100 dentro dos valores normais para verificação de 1º. Entretanto, a amplitude foi baixa para a de 15′. Pacientes alto míopes com esfiloma posterior envolvendo o nervo óptico são suscetíveis à tração da hialoide posterior. Portanto a tração vitreopapilar resultante pode causar comprometimento da visão.

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ABSTRACT Purpose: Microperimetry has been used for several years as a form of visual function testing in patients with retinal diseases. Normal microperimetry values obtained with microperimeter MP-3 have not yet been fully published, and baseline values for topographic macular sensitivity and correlations with age and sex are needed to establish degrees of impairment. This study aimed to determine values for light sensitivity thresholds and fixation stability using the MP-3 in healthy individuals. Methods: Thirty-seven healthy volunteers (age, 28-68 years), underwent full-threshold microperimetry using a 4-2 (fast) staircase strategy with the standard Goldmann III stimulus size and 68 test points positioned identically to those in the Humphrey Field Analyzer 10-2 test grid. The fixation stability was simultaneously recorded during the microperimetry test. The relationship between global sensitivity and age was calculated using linear regression analysis. Results: Microperimetry was performed on 37 participants (74 eyes). The global mean sensitivity was 29.01 ± 1.44 (range, 26-31) dB. The mean central sensitivity at 2° measured by the MP-3 was 28.5 ± 1.77 dB in the right eye (OD) and 28.75 ± 1.98 dB in the left eye (OS). The total median fixation stability values within 2° and 4° were 80% and 96%, respectively. The linear regression analysis also revealed an age-related global sensitivity decline per year of -0.051 dB ± 0.018 (OD) and -0.078 dB ± 0.021 (OS). Conclusions: Microperimetry performed with the MP-3 allows for an automatic, accurate, and topography-specific examination of retinal sensitivity thresholds. The results of this study provide a normal and age-matched database of MP-3 microperimetry.

RESUMO Objetivos: A microperimetria tem sido usada há vários anos como uma forma de teste de função visual em pacientes com doenças da retina. Os valores normais de microperimetria obtidos com MP-3 ainda não foram totalmente publicados e os valores basais para sensibilidade macular topográfica e correlações com idade e sexo são necessários para estabelecer graus de comprometimento. O objetivo do trabalho é determinar valores para limiares de sensibilidade à luz e estabilidade de fixação usando o MP-3 em indivíduos normais. Métodos: Trinta e sete voluntários saudáveis (idade: 28-68 anos), submetidos à microperimetria de limiar total usando uma estratégia de escada 4-2 (rápida) com o tamanho de estímulo padrão Goldmann III e 68 pontos de teste posicionados de forma idêntica aos do Humphrey Field Analyzer 10-2 grade de teste. A estabilidade da fixação foi registrada simultaneamente durante o teste de microperimetria. A relação entre a sensibilidade global e a idade foi calculada por meio de análise de regressão linear. Resultados: A microperimetria foi realizada em 37 indivíduos (74 olhos). A sensibilidade média global foi de 29,01 ± 1,44 dB, intervalo: 26-31 dB. A mediana da sensibilidade central a 2° medida pelo MP-3 foi de 28,5 ± 1,77 dB (ER) e 28,75 ± 1,98 dB (OE). Os valores médios totais de estabilidade da fixação em 2° e 4° foram 80% e 96%, respectivamente. A análise de regressão linear também revelou um declínio de sensibilidade global relacionado à idade por ano de -0,051 dB ± 0,018 (ER) e -0,078 dB ± 0,021 (LE). Conclusões: A microperimetria realizada com o MP-3 permite um exame automático, preciso e específico da topografia dos limiares de sensibilidade da retina. Os resultados deste estudo fornecem um banco de dados normal e de idade correspondente da microperimetria MP-3.

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All-trans retinoic acid (ATRA) is a vitamin A derivative which can increase intracranial pressure, causing visual loss and papilledema. Those patients should be treated similarly to others patients with idiopathic intracranial hypertension. We described a case of a 32-year-old woman presenting with severe visual loss and intracranial hypertension induced by ATRA for acute promyelocytic leukemia, which was treated clinically and with optic nerve sheath fenestration. Patients receiving ATRA therapy should be monitored to neurological and ophthalmic signs and symptoms of intracranial hypertension.

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Retinal complications in patients with inflammatory optic neuritis (ON) are generally related to post-infectious neuroretinitis and are considered uncommon in autoimmune/demyelinating ON, whether isolated or caused by multiple sclerosis (MS) or neuromyelitis optica spectrum disorder (NMOSD). More recently, however, cases with retinal complications have been reported in subjects positive for myelin oligodendrocyte glycoprotein (MOG) antibodies. We report a 53-year-old woman presenting with severe bilateral ON associated with a focal area of paracentral acute middle maculopathy (PAMM) in one eye. Visual loss recovered remarkably after high-dose intravenous corticosteroid treatment and plasmapheresis, but the PAMM lesion remained visible on both optical coherence tomography and angiography as an ischaemic lesion affecting the middle layers of the retina. The report emphasises the possible occurrence of retinal vascular complications in MOG-related optic neuritis, an important addition to the diagnosis of, and possible differentiation from, MS-related or NMOSD-related ON.

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The objective is to perform a multimodal ophthalmological evaluation, including optical coherence angiography (OCTA), asymptomatic APS secondary to SLE (APS/SLE), and compare to SLE patients and control group (CG). We performed a complete structural/functional ophthalmological evaluation using OCTA/microperimetry exam in all participants. One hundred fifty eyes/75 asymptomatic subjects [APS/SLE (n = 25), SLE (n = 25), and CG (n = 25)] were included. Ophthalmologic abnormalities occurred in 9 (36%) APS/SLE, 11 (44%) SLE, and none of CG (p < 0.001). The most common retinal finding was Drusen-like deposits (DLDs) exclusively in APS/SLE and SLE (16% vs. 24%, p = 0.75) whereas severe changes occurred solely in APS/SLE [2 paracentral acute middle maculopathy (PAMM) and 1 homonymous quadrantanopsia]. A trend of higher frequency of antiphospholipid antibody (aPL) triple positivity (100% vs. 16%, p = 0.05) and higher mean values of adjusted Global Antiphospholipid Syndrome Score (aGAPSS) (14 ± 0 vs. 9.69 ± 3.44, p = 0.09) was observed in APS/SLE with PAMM vs. those without this complication. We identified that ophthalmologic retinal abnormalities occurred in more than 1/4 of asymptomatic APS/SLE and SLE. DLDs are the most frequent with similar frequencies in both conditions whereas PAMM occurred exclusively in APS/SLE patients. The possible association of the latter condition with aPL triple positivity and high aGAPSS suggests these two conditions may underlie the retinal maculopathy. Our findings in asymptomatic patients reinforce the need for early surveillance in these patients. Key Points • Retinal abnormalities occur in more than 1/4 of asymptomatic APS/SLE and SLE patients. • The occurrence of PAMM is possibly associated with APS and DLDs with SLE. • Presence of aPL triple positivity and high aGAPSS seem to be risk factors for PAMM.

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PURPOSE: Microperimetry has been used for several years as a form of visual function testing in patients with retinal diseases. Normal microperimetry values obtained with microperimeter MP-3 have not yet been fully published, and baseline values for topographic macular sensitivity and correlations with age and sex are needed to establish degrees of impairment. This study aimed to determine values for light sensitivity thresholds and fixation stability using the MP-3 in healthy individuals. METHODS: Thirty-seven healthy volunteers (age, 28-68 years), underwent full-threshold microperimetry using a 4-2 (fast) staircase strategy with the standard Goldmann III stimulus size and 68 test points positioned identically to those in the Humphrey Field Analyzer 10-2 test grid. The fixation stability was simultaneously recorded during the microperimetry test. The relationship between global sensitivity and age was calculated using linear regression analysis. RESULTS: Microperimetry was performed on 37 participants (74 eyes). The global mean sensitivity was 29.01 ± 1.44 (range, 26-31) dB. The mean central sensitivity at 2° measured by the MP-3 was 28.5 ± 1.77 dB in the right eye (OD) and 28.75 ± 1.98 dB in the left eye (OS). The total median fixation stability values within 2° and 4° were 80% and 96%, respectively. The linear regression analysis also revealed an age-related global sensitivity decline per year of -0.051 dB ± 0.018 (OD) and -0.078 dB ± 0.021 (OS). CONCLUSIONS: Microperimetry performed with the MP-3 allows for an automatic, accurate, and topography-specific examination of retinal sensitivity thresholds. The results of this study provide a normal and age-matched database of MP-3 microperimetry.

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ABSTRACT We describe a case of a 33-years-old woman who presents with severe acute bilateral visual loss secondary to massive exudative hypertensive maculopathy as the first sign of immunoglobulin A nephropathy. The patient's ophthalmic examination showed bilateral cotton-wool spots, flame-shaped retinal hemorrhages, diffuse narrow arterioles, optic disk edema, and exudative maculopathy. Systemic workup demonstrated a systolic and diastolic blood pressure of 240 mmHg and 160 mmHg, respectively, proteinuria, and hematuria, suggesting kidney disease as the causative condition. A kidney biopsy confirmed immunoglobulin A nephropathy. She was treated with systemic corticosteroids, antihypertensive drugs, and a single bilateral intravitreal injection of aflibercept. There was a prompt resolution of macular edema and vision improvement. Our case draws attention to the fact that severe bilateral visual loss can be the first sign of severe hypertension. Secondary causes, such as immunoglobulin A nephropathy, should be ruled out.

RESUMO Nosso objetivo é descrever uma paciente de 33 anos de idade, com perda visual bilateral grave por maculopatia hipertensiva exsudativa como o primeiro sinal da nefropatia por imunoglobulina A. A fundoscopia revelou a presença de manchas algodonosas, hemorragias em chama-de-vela, estreitamento arteriolar difuso, edema de disco óptico e maculopatia exsudativa bilateral. A pressão arterial sistólica foi de 240mmHg e a diastólica de 160 mmHg associado a proteinúria e hematúria, sugerindo a presença de doença renal. A biópsia renal confirmou a nefropatia por imunoglobulina A. A paciente foi tratada como corticoide sistêmico, drogas anti-hipertensivas e uma única dose intravítrea de Aflibercept em ambos os olhos. Houve rápida melhora do edema macular e da acuidade visual. Nosso caso chama a atenção para o fato de que a perda visual bilateral grave pode ser a primeira apresentação de uma doença hipertensiva sistêmica. Causas secundárias como a nefropatia por imunoglobulina A devem ser afastadas.

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ABSTRACT This is a case series about retinal arterial macroaneurysms in three patients recovered from COVID-19. None of them had previous past ocular and systemic history. The first patient was a 47-year-old man, with best-corrected visual acuity of 20/20 in both eyes. He presented a cotton wool spot in the right eye and two peripheral exudative retinal arterial macroaneurysms in the left eye. Laser photocoagulation was performed and best-corrected visual acuity remained stable. The second one was a 62-year-old-woman with acute visual loss in the right eye (best-corrected visual acuity of 20/400). There was vitreous hemorrhage and a peripheral retinal arterial macroaneurysm in the right eye. Laser photocoagulation was performed and best-corrected visual acuity improved to 20/20. The third patient was a 54-year-old woman, with best-corrected visual acuity of 20/20 in the right eye and 20/600 in the left eye. Fundus examination showed multiple layer hemorrhage in the macular region and retinal arterial macroaneurysms in the superior temporal branch. The cases underwent fluorescein angiography, which confirmed the diagnosis of retinal arterial macroaneurysms.

RESUMO Esta é uma série de casos sobre macroaneurismas arteriais da retina em três pacientes recuperados da COVID-19. Nenhum tinha história ocular e sistêmica prévias. O primeiro era um homem de 47 anos, com melhor acuidade visual corrigida de 20/20 em ambos os olhos. Apresentava mancha algodonosa em olho direito e dois macroaneurismas arteriais da retina exsudativos periféricos em olho esquerdo. Foi realizada fotocoagulação a laser, e a melhor acuidade visual corrigida permaneceu estável. A segunda era uma mulher de 62 anos com perda visual aguda em olho direito (melhor acuidade visual corrigida de 20/400). Havia hemorragia vítrea e um macroaneurisma periférico em olho direito. O laser foi realizado, e a melhor acuidade visual corrigida melhorou para 20/20. A terceira paciente era uma mulher de 54 anos, com melhor acuidade visual corrigida 20/20 em olho direito e 20/600 olho esquerdo. A fundoscopia mostrou hemorragia em múltiplas camadas na região macular e um macroaneurisma arterial da retina no ramo temporal superior. Os casos foram submetidos à angiofluoresceinografia, que confirmou o diagnóstico de macroaneurismas arteriais da retina.

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BACKGROUND: Primary antiphospholipid syndrome (PAPS) is characterized by the presence of antiphospholipid antibodies (aPL), repetitive fetal loss, and arterial/venous thrombosis and no association with other autoimmune rheumatic disease. Ocular involvement can also occur including retinal vascular thrombosis and neuro-ophthalmological manifestations, such as optic neuropathy and amaurosis fugax. Early detection of ocular changes is crucial to minimize functional loss. PURPOSE: To perform a multimodal evaluation, including the use of Optical Coherence Angiotomography (OCTA), in patients with PAPS without ocular complaints and compare with healthy individuals. METHODS: We performed a complete structural and functional ophthalmological evaluation using OCTA and microperimetry exam in patients with PAPS, followed at a tertiary Rheumatology outpatient clinic. RESULTS: We included 104 eyes of 52 subjects [PAPS without ocular complaints (N = 26) and healthy individuals (N = 26)]. Among PAPS patients, 21 were female (80.8%) and 21 (80.8%) were Caucasians. PAPS manifestations were venous (65.4%), arterial thrombosis (34.6%), and obstetrical (34.6%) and all of them had lupus anticoagulant. Ophthalmologic findings were more frequent in PAPS compared to healthy individuals (19.2% vs. 0%, p = 0.05). The most common retinal change was paracentral acute middle maculopathy (PAMM) (3 patients, 5 eyes), followed by drusen (1 patient, 2 eyes) and pachychoroid pigment epitheliopathy (PPE) (1 patient, 1 eye). Hypertension and hyperlipidemia were present in 100% of the PAPS patients with PAMM, while only six patients (26.1%) with PAPS without PAMM presented these two risk factors together (p = 0.03). CONCLUSIONS: We provide novel evidence that approximately 20% of our asymptomatic PAPS patients without ocular symptoms have ophthalmologic findings that require early identification and careful surveillance focusing on minimizing systemic and vascular risk factors.

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BACKGROUND: Optical coherence tomography angiography (OCTA) is a relatively new non-invasive imaging technique to evaluate retinal vascular complexes. However, there is still a lack of standardization and reproducibility of its quantitative evaluation. Furthermore, manual analysis of a large amount of OCTA images makes the process laborious, with greater data variability, and risk of bias. Therefore, the aim of this study is to describe a fast and reproducible quantitative analysis of the foveal avascular zone (FAZ), macular superficial and deep vascular complexes (mSVC and mDVC, respectively), and peripapillary superficial vascular complex (pSVC) in OCTA images. METHODS: We survey models and methods used for studying retinal microvasculature, and software packages used to quantify microvascular networks. These programs have provided researchers with invaluable tools, but we estimate that they have collectively achieved low adoption rates, possibly due to complexity for unfamiliar researchers and nonstandard sets of quantification metrics. To address these existing limitations, we discuss opportunities to improve effectiveness, affordability, and reproducibility of microvascular network quantification with the development of an automated method to analyze the vessels and better serve the current and future needs of microvascular research. OCTA images of the macula (10°x10°, 15°x15°, or 20°x20° centered on the fovea) and peripapillary area (15 × 15º centered on optic nerve head) were exported from the device and processed using the open-source software Fiji. The mSVC, mDVC, and pSVC were automatically analyzed regarding vascular density in the total area and four sectors (superior, inferior, nasal, and temporal). We also analyzed the FAZ regarding its area, perimeter, and circularity in the SVC and DVC images. RESULTS: We developed an automated model and discussed a step by step method to analyze vessel density and FAZ of the macular SVC and DVC, acquired with OCTA using different fields of view. We also developed an automated analysis of the peripapillary SVC. CONCLUSION: Our developed automated analysis of macular and peripapillary OCTA images will allow a fast, reproducible, and precise quantification of SVC, DVC, and FAZ. It would also allow more accurate comparisons between different studies and streamlines the processing of images from multiple patients with a single command.

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This case report presents the details of a 33-year-old female patient who was referred to a specialized retina service because of mild vision loss in her right eye). The patient's visual acuity was 20/25 in right eye and 20/50 in the left eye (; amblyopic); the spherical equivalent was -12.75 diopters (right eye) and -14.75 diopters (left eye). Multimodal retinal imaging showed peripapillary schisis in both the inner and outer retinal layers, grade II posterior vitreous detachment, and a tessellated fundus. Using Humphrey perimetry and MP-3 microperimetry, the functional evaluation indicated macular sensitivity within normal limits and decreased sensitivity in the peripapillary region, especially in right eye. The pattern-re versal visual evoked potential was measured. The N75 and P100 latency and amplitude in right eye were within normal values for checks of 1º. However, the amplitude was low for checks of 15'. Highly myopic patients who have posterior staphyloma that involves the optic nerve are susceptible to posterior hyaloid traction, and the resulting peripapillary vitreous traction may compromise vision.

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We describe a case of a 33-years-old woman who presents with severe acute bilateral visual loss secondary to massive exudative hypertensive maculopathy as the first sign of immunoglobulin A nephropathy. The patient's ophthalmic examination showed bilateral cotton-wool spots, flame-shaped retinal hemorrhages, diffuse narrow arterioles, optic disk edema, and exudative maculopathy. Systemic workup demonstrated a systolic and diastolic blood pressure of 240 mmHg and 160 mmHg, respectively, proteinuria, and hematuria, suggesting kidney disease as the causative condition. A kidney biopsy confirmed immunoglobulin A nephropathy. She was treated with systemic corticosteroids, antihypertensive drugs, and a single bilateral intravitreal injection of aflibercept. There was a prompt resolution of macular edema and vision improvement. Our case draws attention to the fact that severe bilateral visual loss can be the first sign of severe hypertension. Secondary causes, such as immunoglobulin A nephropathy, should be ruled out.

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BACKGROUND: The present case aims to describe a previously healthy man who presented multiple attacks of transient monocular visual loss after Pfizer-BioNTech COVID-19 vaccination and to discuss the possible mechanisms related to occurrence of this condition. CASE PRESENTATION: We report a case of multiple attacks of transient monocular visual loss in a previously healthy middle-aged man two weeks after Pfizer-BioNTech COVID-19 vaccination. TVL attacks were described as sudden and painless complete visual loss, lasting about one minute, followed by a full recovery. He presented several non-simultaneous attacks in both eyes, 16 in the right eye, and 2 in the left eye on the same day, fifteen days after receiving the second dose of the Pfizer-BioNTech COVID-19 vaccine. The brain's magnetic resonance angiography, echocardiogram, and doppler ultrasound imaging of the carotid and vertebral arteries were non-revealing. The complete blood exam revealed a slightly elevated C-reactive protein test. We assessed fundus examination during the transient visual loss attack and revealed diffuse vascular narrowing for both arterial and venous branches, notably in the emergence of the optic disc in right eye. In addition, the circumpapillary optical coherence tomography angiography (OCTA) vessel density map was reduced. Oral verapamil hydrochloride 60 mg twice daily was initiated, and the attacks of transient visual loss improved after two days. CONCLUSIONS: To date, and the best of our knowledge, this is the first case report of multiple transient monocular visual loss attacks due to retinal vasospasm in a previously healthy middle-aged man documented by fundus retinography and OCTA. We discuss in this article the possible association of retinal vasospasm and Pfizer-BioNTech COVID-19 vaccination, probably related to vaccine-induced inflammation.