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Purpose: To evaluate the incidence, rate, and pattern of progression of myopic maculopathy in eyes operated with macular buckle (MB) for myopic traction maculopathy versus a control group without surgery to find out whether the progression varies due to the MB's indentation and to evaluate possible MB-related pigmentary changes or atrophy., Methods: Eyes operated with MB with two good quality fundus images: one preoperative or early postoperative image and a second image at least 12 months apart; the control group comprised the contralateral eyes. Demographics, axial length, follow-up, stage of myopic traction maculopathy, and myopic maculopathy were reported. Progression results of groups and subgroups (mid- and long-term follow-up) were reported and compared., Results: Overall, 116 eyes of 66 patients were included. Progression was found in 29 eyes (41.4%) and 23 eyes (50%) in the MB group and control group, respectively. The progression rate was 73 per 1,000 eye-years and 88.9 per 1,000 eye-years in the MB group and the control group, respectively. Axial length was found to predict progression (odds ratio [OR], 2.59; P = 0.02)., Conclusion: Progression of myopic maculopathy was similar in both groups and was mildly greater in the control group. No MB-related pigmentary changes or atrophy was detected.

Purpose: To describe the different types of vitreomacular interface abnormalities (VMIA) seen on optical coherence tomography (OCT) in type 2 macular telangiectasia (MacTel) and explain the possible reasons for its development., Methods: In this retrospective cross-sectional study, type 2 MacTel eyes with macular volumetric OCT imaging protocol were included to identify different types of VMIA such as abnormal PVD, vitreomacular traction (VMT), ERM, and lamellar and full-thickness macular hole. The VMIA findings were then correlated with different MacTel disease stages and visual acuity., Results: One thousand forty-three OCTs of 332 type 2 MacTel eyes from 169 patients at different visits were examined. VMIA was detected in 709 (68%) of those OCT scans in 216 (65%) eyes. There were 273 (39%), 31 (4%), 89 (13%), 7 (1%), and 381 (54%) OCT scans with vitreomacular adhesion, VMT, ERM, and inner and outer lamellar macular holes discovered respectively. VMIA eyes had a high frequency of abnormal PVD (p = 0.001) and retinal pigment clumps (RPCs) [p = 0.032]. Eyes with abnormal PVD (p = 0.034) and RPC (p = 0.000) had a higher rate of ERM development. RPC was linked to an increased risk of developing ERM (odd ratio 2.472; 95% CI 1.488-4.052). RPC and ERM contributed significantly to poor visual acuity (0.661 ± 0.416, 20/92)., Conclusion: OCT reveals a high frequency of VMIA in advanced type 2 MacTel eyes. RPC could be responsible for the development of anomalous PVD, as well as subsequent VMIAs and ERM. Additional work is required to examine the long-term changes and surgical outcomes of these eyes., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Competing Interests: Competing interests: None declared.

Purpose: To describe a case of acute macular neuroretinopathy (AMN) in a patient with recent COVID-19 vaccination and infection who demonstrated atypical features on presentation., Observations: A 64-year-old woman presented with central vision loss in both eyes (OU). She had recently received the Moderna COVID-19 vaccine and rapidly developed systemic symptoms. Testing revealed COVID-19 infection. Visual acuities were 20/200 OU and near-infrared reflectance revealed hypo-reflective lesions in the maculae OU, optical coherence tomography (OCT) showed outer nuclear layer thinning and ellipsoid zone disruption OU, and OCT-angiography showed flow voids in the deep capillary plexus and choriocapillaris OU, all consistent with AMN. She was treated with oral prednisone with subsequent mild vision improvement and persistent scotomas., Discussion: COVID-19 associated AMN can present with a more severe clinical presentation than classically seen in AMN. Ischemic and inflammatory changes due to COVID-19 infection may contribute to this more advanced presentation.

Purpose: To analyze the imaging characteristics and the clinical course of patients showing concomitant paracentral acute middle maculopathy (PAMM) and acute macular neuroretinopathy (AMN) post-blunt trauma., Methods: PAMM and AMN lesions post-blunt trauma diagnosed on enhanced depth imaging optical coherence tomography (EDI-OCT) were recruited for the study., Results: : Thirteen eyes of 13 individuals with a history of blunt trauma were included in the study, of whom 11 (85%) were males. Mean age of the patients was 33.62 (range 16-67) years. Mean visual acuity at presentation and the last visit was 1.67 log of minimum angle of resolution (logMAR) and 0.82 logMAR, respectively. Mean interval between trauma and imaging was 5.08 (range 1-15) days. All patients had unilateral involvement, with the right eye being involved in 10 patients (77%). All patients had concomitant PAMM and AMN lesions., Conclusion: : Presence of coincident PAMM and AMN suggests a common pathophysiologic etiology, but the description of concomitant PAMM and AMN in the setting of blunt trauma to eye is hitherto unreported. Identifying AMN in a setting of PAMM requires meticulous examination of the OCT and OCTA images. It can be a cause of suboptimal visual recovery in such eyes., Competing Interests: None

Purpose: To report a case of acute macular neuroretinopathy (AMN) following SARS-CoV-2 vaccination., Methods: Case report., Results: An otherwise healthy 28-year-old white woman presented to an ophthalmology department with complaints of black paracentral vision scotomata on both eyes that appeared 2 days after receiving the first dose of Vaxzevria vaccine. Fundus examination revealed bilateral red brown petaloid lesions around the fovea. On spectral domain optical coherence tomography, a hyperreflective plaque between the outer plexiform and outer nuclear layers along with disruption of ellipsoid zone/interdigitation zone was observed. A diagnosis of AMN was made., Conclusion: AMN may be an extremely rare adverse event of SARS-COV-2 vaccination, especially in patients with other risk factors., Synopsis: An otherwise healthy 28-year-old white woman was diagnosed with AMN 2 days after SARS-CoV-2 vaccination.

Recent evidence has demonstrated that the global public health burden of myopia is rising rapidly. Highly myopic eyes are associated with increased frequency of eye disorders that can lead to irreversible visual impairment. With recent technological advancement in ophthalmic imaging modalities, various macular complications associated with pathologic myopia are being elucidated. The development and progression of myopic chorioretinal atrophy, myopic macular neovascularization, myopic traction maculopathy and dome-shaped macula are vision-threatening myopic macular diseases. In order to overcome the challenges in managing patients with pathologic myopia, it is important to have a complete understanding in the natural course of these myopic macular diseases. Standardising the classification criteria of pathologic myopia is essential for enhancing clinical surveillance. Personalised pharmaceutical therapy and surgical interventions will help to optimise the treatment outcomes in patients suffering from these myopic macular diseases., (© 2023 Royal Australian and New Zealand College of Ophthalmologists.)

Aim: The aim of the present paper is to describe the case study of a female patient with bilateral AMN, suffering from active acute infection with COVID-19., Methods: A 32-year-old female patient with impaired vision bilaterally persisting for 2 days reported to the emergency Department of Ophthalmology at the Central Military Hospital in Ružomberok, Slovakia. The patient manifested symptoms of COVID-19 infection persisting for 3 days before the onset of visual complaints., Results: At the primary examination, initial best corrected central visual acuity was 20/40 bilaterally, paracentral scotomas were present more in the right eye. Upon examination of the ocular fundus, the edges of the optic nerve disc were out of focus more in the left eye; brownish red petaloid lesions were present around the fovea, the periphery was without pathological findings. OCT and OCTA were performed, with a conclusion of bilateral AMN upon a background of COVID-19 infection. Low molecular weight heparin (LMWH) was administered in a preventive dose., Conclusion: The number of documented ocular complications of COVID-19 infection, including microvascular events, is currently increasing.

Aims: To introduce a case report and review the literature on trauma-related acute macular neuroretinopathy as an unusual etiology of acute macular neuroretinopathy., Material and Methods: A 24-year-old man presented with unilateral paracentral scotoma following non-ocular trauma in a car accident. The relative afferent pupillary defect was negative and the best corrected visual acuities of both eyes were 10/10 (by the Snellen chart scale)., Results: Retinoscopy revealed a reduced foveal reflex, along with a small pre-retinal hemorrhage over the mid-pathway of the supranasal arteriole. OCT images showed an obvious ellipsoid zone (EZ) layer disruption in the macula of the left eye. The infrared fundus photograph of the same eye revealed a distinct hyporeflective area involving the macula. On fundus angiography, no macular vascular lesion was detected. The scotoma persisted after 3 months follow-up., Conclusion: Non-ocular trauma including head or chest trauma without direct ocular injury accounts for most cases of trauma-related acute macular neuroretinopathy. It is important to distinguish this entity, given that there are also unremarkable findings in the retinal examination of these patients. Indeed, proper clinical suspicion leads to further suitable investigations and impedes other extraordinary images, which are the basic rules in the management of traumatic patients suffering multiple injuries and incurring medical expenses.

Purpose: To describe the use of hyperbaric oxygen therapy (HBOT) in conjunction with immunosuppression for acute macular neuroretinopathy (AMN) in systemic lupus erythematosus (SLE)., Methods: Two known cases of SLE presented to us with blurred vision and paracentral scotomas due to AMN. Both cases reported worsening of their conditions despite the initiation of high-dose steroid therapy. HBOT was added on as a treatment modality to address vaso-occlusive ischemic injury., Results: Both patients underwent a total of twelve cycles of HBOT. Functional and anatomical improvements were noted immediately after the initiation of therapy and were maintained over more than one year of follow-up. No significant retinal thinning was noted on optical coherence tomography on disease resolution, as has been noted previously. Visual field scotoma showed a complete resolution., Conclusion: Our cases suggest that HBOT may have a role in aiding functional and anatomical recovery in AMN associated with SLE.

Purpose: We present a case of retinal vasculopathy with cerebral leukodystrophy and review the usefulness of optical coherence tomography angiography (OCT-A) in the assessment of long-term outcomes., Case Description: A 31-year-old woman developed sudden-onset scotoma in her right eye. Fundus examination and fluorescein angiography showed a patch of soft exudate and capillary nonperfusion in the posterior pole and outside the vascular arcades. OCT-A revealed that the initial vessel density (VD) of the superficial capillary plexus (SCP) and deep capillary plexus (DCP) of the right eye were 32% and 49.2%, respectively. Interestingly, over time, the VD of the SCP and DCP gradually decreased to 23.1% and 26.2%, respectively. In contrast, the initial VD of the SCP and DCP of the left eye were both stable at 44.3% and 56.2%, respectively, and only decreased slightly to 39.3% and 45.7%, respectively, over time. The average VD loss of the SCP and DCP, assessed over 1 year, was 8% and 13%, respectively, in the right eye, and 3% and 6%, respectively, in the left eye., Conclusion: Based on this case report, in which we demonstrated a long-term decline in VD of the macula in a young woman with mild retinal vasculopathy with cerebral leukodystrophy, we suggest that there is a potential and valuable role for OCT-A in this rare disease.

Significance: Unilateral acute idiopathic maculopathy is a rare retinochoroidal disorder, the cause of which is unknown. Multimodal retinal imaging obtained in this case suggests that the cause of unilateral acute idiopathic maculopathy is impaired choroidal circulation., Purpose: This study aimed to report a patient with unilateral acute idiopathic maculopathy who showed improvement of choroidal circulation over time in optical coherence tomography angiography images., Case Report: A 33-year-old woman with a prodromal flu-like illness noticed a sudden central visual field abnormality in the right eye. Five days after the onset, best-corrected visual acuity was 0.15 in the right eye. Fundus photography showed a yellowish lesion at the macula in the right eye. Optical coherence tomography showed a blurred ellipsoid zone and a thickened retinal pigment epithelium at the fovea and a disrupted/blurred ellipsoid zone and retinal pigment epithelium at the parafovea. Optical coherence tomography angiography segmentation of the choriocapillaris revealed a hypointense region at the fovea with a hyperintense region surrounding it. One week after the onset, best-corrected visual acuity was improved from 0.15 to 2.0 in the right eye. Furthermore, the yellowish macula lesion shrank, and the outer retinal layers showed improvement in optical coherence tomography. Optical coherence tomography angiography showed reduction of the hypointense region and enlargement of the hyperintense region. Approximately 1 year after the onset, the yellowish lesion was faded. Optical coherence tomography revealed an almost normal ellipsoid zone but a thickened interdigitation zone. Optical coherence tomography angiography also revealed an apparent hyperintense instead of the hypointense region and increased choroidal blood flow., Conclusions: The current patient showed a gradual recovery of choroidal circulation and outer retinal layer morphology 1 year after the onset of unilateral acute idiopathic maculopathy. Optical coherence tomography angiography findings indicated that the primary cause of unilateral acute idiopathic maculopathy was impaired choroidal circulation, and choroidal vessels improved morphologically over the disease course., Competing Interests: Conflict of Interest Disclosure : None of the authors have reported a financial conflict of interest., (Copyright © 2022 American Academy of Optometry.)

Abstract: A 26-year-old African American man with sickle cell disease noticed blurry vision in both eyes after a recent complicated hospital admission for sickle cell crisis. Anterior and posterior segment examination of each eye was normal, but visual field testing revealed binasal scotomas. Optical coherence tomography of the macula demonstrated severe thinning of the temporal inner retina, suggesting previous bilateral occlusions of terminal retinal arterioles involving the temporal macula, an uncommon complication of sickle cell disease. This case is a reminder that retinal pathology should always be considered as a potential cause of unexplained visual field defects and highlights the role of macular OCT in evaluation of these patients., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by North American Neuro-Ophthalmology Society.)

Rationale: Peripapillary hyperreflective ovoid mass-like structures (PHOMS) and dome-shaped macula (DSM) are 2 optical coherence tomography findings reported in 2018 and 2008, respectively. To date, there have been no ophthalmic case reports of concomitant PHOMS and DSM., Patient Concerns: A 19-year-old woman presented to our clinic with complaints of decreased vision in both eyes., Diagnosis: The patient was diagnosed with PHOMS and a dome-shaped macula complicated by subretinal fluid in both eyes., Interventions: A micropulse laser under the guidance of Indocyanine green angiography was applied to the hyperfluorescent areas and drugs to improve retinal microcirculation., Outcomes: No response to any intervention over the 41 months of follow-up, her visual acuity remained the same, and the subretinal fluid often recurred., Lessons: PHOMS and DSM are associated with myopia; myopia may be a mediator between PHOMS and DSM. Dome-like structural changes may occur in different parts of the retina (optic disc and macula), caused by asymmetric myopic posterior scleral growth., Competing Interests: The authors have no conflicts of interests to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

Purpose: To investigate whether higher blood pressure and greater arterial stiffness are associated with the presence of macular cysts and whether this association is already present in the absence of micro-aneurysms in individuals with and without type 2 diabetes., Methods: Using spectral domain optical coherence tomography (OCT), we performed a macular volume scan in 2647 individuals (mean age 60 ± 8 years, 50% men, 27% type 2 diabetes). The association between macular cysts and 24-hour systolic and diastolic blood pressure, pulse pressure, mean arterial blood pressure, carotid-femoral pulse wave velocity and carotid distensibility was assessed by use of logistic regression., Results: Twenty-four hours systolic blood pressure was associated with the presence of macular cysts [OR = 1.03 (95% CI 1.00-1.05) per 1 mmHg, p = 0.03]. 24 hr pulse pressure [OR = 1.61 (95% CI 1.11-2.34) per 10 mmHg, p = 0.01] and carotid-femoral pulse wave velocity [OR = 1.16 (95% CI 1.02-1.32) per 1 m/s, p = 0.02] were associated with macular cysts, while carotid distensibility was not [OR = 1.03 (95% CI 0.96-1.11) per 1.0*10 -3 /kPa, p = 0.45]. Associations were similar in individuals with and without type 2 diabetes and were already present in the absence of micro-aneurysms., Conclusion: Twenty-four hours systolic blood pressure, 24 hr pulse pressure and carotid-femoral pulse wave velocity are associated with the presence of OCT-detected macular cysts in individuals with and without type 2 diabetes, even in the absence of micro-aneurysms. Therefore, blood pressure and aortic stiffness are potential factors contributing to macular cysts., (© 2020 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: To evaluate 18 months' results of a strict anti-vascular endothelial growth factor protocol for radiation maculopathy following proton therapy in choroidal melanoma., Methods: Retrospective, comparative, nonrandomized study of 74 radiation maculopathy patients presenting macular lipid deposits, hemorrhages, microaneurysms, cystoid edema, nerve layer infarction, telangiectasia, or capillary nonperfusion. The study group included 52 consecutive patients injected with intravitreal anti-vascular endothelial growth factors (bevacizumab/ranibizumab: 46/6) every two months for the first and every 3 months for the second year, with minimum 12 months' follow-up. The control group consisted of 22 patients having declined this treatment. Best-corrected visual acuity, spectral domain-optical coherence tomography and optical coherence tomography angiography were recorded at baseline, 6, 12, and 18 months. The foveal avascular zone and capillary density were measured at the superficial capillary plexus., Results: Radiation maculopathy was diagnosed at 2 years (1.5-3.5) after proton therapy. Best-corrected visual acuity at baseline, 12 and 18 months improved in the study group from 0.45, 0.3 to 0.2 logarithm of the minimum angle of resolution, but decreased in the control group from 0.5, 0.9 to 1.0 logarithm of the minimum angle of resolution respectively (P < 0.001 at 12 months). Simultaneously, foveal avascular zone enlargement was less in the study (from 0.377, 0.665 to 0.744 mm2) than control group (from 0.436, 1.463 to 2.638 mm2) (P = 0.05 at 12 months). CMT (280 and 276 µm) and capillary density (37% and 38%, at baseline, respectively) did not evolve significantly different., Conclusion: Intravitreal anti-vascular endothelial growth factors, every 2 months for the first and every 3 months for the second year, slow down, over up to 18 months, vision loss and anatomical degradation in radiation maculopathy following proton therapy for choroidal melanoma., (Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the Opthalmic Communications Society, Inc.)

Aim: To describe the ophthalmologic findings on the largest cohort of patients with sialidosis type I due to deficiency of the lysosomal sialidase, neuraminidase 1 (NEU1) and to introduce a quantitative neuroretinal image analysis approach to the associated 'macular cherry-red spot'., Methods: Seven patients with sialidosis type I (mutations in NEU1 ) and one with galactosialidosis (mutations in CTSA ) were included. All patients underwent detailed ophthalmological examinations. The reflectivity of macular optical coherence tomography (OCT) was measured using greyscale analysis (Fiji) and compared with age-matched healthy volunteers. Four patients were evaluated over a time of 1.5+0.5 years., Results: The mean age of the patients at their first visit was 27.5+9.8 years. All patients had a macular cherry-red spot, clear corneas and visually non-significant lenticular opacities. The mean visual acuity was LogMar 0.4 (20/50)+0.4 (20/20 to 20/125). Six patients had good visual function. Optic atrophy was present in two individuals with reduced acuity. A significant increase in macular reflectivity was present in all patients compared to age-matched controls (p<0.0001)., Conclusion: Most of our patients (75%) have preserved visual acuity, even in adulthood. The presence of optic atrophy is associated with poor visual acuity. Increased macular reflectivity by OCT greyscale measurements is noted in all patients, although the underlying biological basis is unknown. These findings complement the current methods for examining and monitoring disease progression, especially in patients for whom visualisation of the cherry-red spot is not entirely clear., Trial Registration Number: NCT00029965., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: To determine the mechanism behind macular bulge height increase in eyes with dome-shaped macula (DSM)., Design: Retrospective, observational case series., Methods: Eyes presenting with DSM followed up for a minimum of 1 year were examined using ocular biometry and spectral-domain optical coherence tomography at baseline and at end of follow-up. Axial length (AL), DSM bulge height, and central and peripheral choroidal thickness (nasal, temporal, superior, and inferior quadrants) were reported. Eyes were categorized into 2 groups for comparison: the "mini-DSM" group (DSM < 100 μm) and the "classic" DSM group (DSM > 100 μm)., Results: Fifty-eight eyes (33 patients) were studied: 32 (55%) were classic DSM and 26 (45%) mini-DSM. During the mean follow-up of 51.76 ± 36.01 months, mean AL increased from 26.99 ± 2.94 mm to 27.12 ± 3.09 mm (P = .010) and mean macular bulge height increased from 235.88 ± 282.47 μm to 262.34 ± 317.15 μm (P < .001). DSM height change was significantly higher than AL change (P < .001). Mean peripheral choroidal thickness significantly decreased nasally (P = .008), temporally (P = .026), and inferiorly (P < .001). Mini-DSM eyes exhibited shorter AL (26.17 vs 27.66 mm; P = .027), greater visual acuity (0.169 vs 0.437 logMAR; P = .002), and fewer macular complications compared to classic DSM eyes., Conclusions: Macular bulge increase in DSM is associated with eye elongation and overall thinning of the peripheral choroid. DSM might result from differential elongation of the eye predominant in the peri-dome region. Mini-DSM (ie, inferior to 100 μm) are characterized by slower evolution, better visual prognosis, and fewer complications compared to "classic" DSM., (Copyright © 2020 The Authors. Published by Elsevier Inc. All rights reserved.)

Purpose: To investigate the relationship between dome-shaped maculas (DSMs) and posterior staphylomas in highly myopic eyes., Methods: Five hundred and eleven eyes of 291 patients with high myopia (refractive error ≤-8.0 diopters or axial length ≥26.5 mm) were examined by ultra-widefield swept-source optical coherence tomography (UWF-OCT). Posterior staphylomas were identified by detecting the staphyloma edges in the UWF-OCT images., Results: Eighty-two eyes of the 511 highly myopic eyes had a DSM, and a posterior staphyloma was observed in 45 of 82 eyes with a DSM (54.9%) and in 301 of 429 eyes (70.2%) without a DSM. The incidence of staphylomas was significantly lower in eyes with a DSM than those without a DSM (P = 0.007). The wide macular type of staphyloma was the predominant type in eyes with a DSM (31/45 eyes; 68.9%), whereas the narrow macular type and wide macular type of staphylomas were present almost equally in the eyes without a DSM., Conclusion: The lack of staphylomas in 45% of eyes with a DSM suggests that DSMs form independently from posterior staphylomas. Dome-shaped maculas tend to occur in eyes with a large expansion of the posterior fundus and should be considered a posterior scleral curvature abnormality.

Importance: Determination of retinal thinning rates may help to identify patients who are at risk of progression of sickle cell retinopathy., Objective: To assess the rates of macular thinning in adults with and without sickle cell retinopathy using spectral-domain optical coherence tomography (OCT) and to identify ocular and systemic risk factors associated with retinal thinning., Design, Setting, and Participants: This longitudinal prospective case-control study enrolled adult participants from a university-based retina subspecialty clinic between February 11, 2009, and July 3, 2019. The study was designed in autumn 2008 and conducted from February 2, 2009, to July 3, 2020. Participants with sickle cell retinopathy (sickle cell group) were matched by age and race with participants without sickle cell retinopathy (control group). Participants received annual spectral-domain OCT and clinical examinations. Those with at least 1 year of follow-up by July 3, 2020, were included in the analysis. Data were analyzed from February 2, 2009, to July 3, 2020., Main Outcomes and Measures: The primary outcome was comparison of spectral-domain OCT measurements from early-treatment diabetic retinopathy study subfield rates of retinal thinning between eyes with and without sickle cell retinopathy and between different sickle cell hemoglobin subtypes. The secondary outcome was identification of ocular and systemic risk factors associated with rates of retinal thinning., Results: Among 370 adults (711 eyes) enrolled in the study, 310 participants (606 eyes) had sickle cell retinopathy, and 60 participants (105 eyes) did not. Of those, 175 of 310 participants (56.5%; 344 of 606 eyes [56.8%]; mean [SD] age, 37.8 [12.8] years; 126 women [72.0%]) in the sickle cell group and 31 of 60 participants (51.7%; 46 of 105 eyes [43.8%]; mean [SD] age, 59 [15.4] years; 22 women [71.0%]) in the control group had at least 1 year of clinical and spectral-domain OCT follow-up data from baseline. The mean (SD) follow-up was 53.7 (32.6) months for the sickle cell group and 54.6 (34.9) months for the control group. Rates of macular thinning in the sickle cell group were significantly higher than those in the control group for the inner nasal (difference, -1.18 μm per year; 95% CI, -1.71 to -0.65 μm per year), inner superior (difference, -1.03 μm per year; 95% CI, -1.78 to -0.29 μm per year), inner temporal (difference, -0.61 μm per year; 95% CI, -1.16 to -0.07 μm per year), and outer nasal (difference, -0.41 μm per year; 95% CI, -0.80 to -0.03 μm per year) quadrants. Patients with sickle cell hemoglobin SC and sickle cell hemoglobin β-thalassemia subtypes had higher rates of retinal thinning than those with the sickle cell hemoglobin SS subtype. Risk factors associated with greater rates of retinal thinning included participant age, stage of retinopathy, previous stroke, and presence of hypertension, acute chest syndrome, or diabetes. Hydroxyurea therapy was associated with decreased rates of retinal thinning and may be a protective factor., Conclusions and Relevance: In this study, rates of retinal thinning were higher among participants with sickle cell retinopathy compared with those without sickle cell retinopathy, and thinning rates increased with participant age and stage of retinopathy. These findings suggest that identifying anatomic worsening of sickle cell maculopathy through spectral-domain OCT may be a useful parameter to evaluate the progression of sickle cell retinopathy.

Purpose: Using optical coherence tomography angiography (OCT-A) to compare the retinal micro vessel alteration between obstructive sleep apnea (OSA) children and normal subjects., Methods: One hundred-thirty-two eyes (132 participants) were recruited in this study. The average age of OSA group (66 eyes) and healthy control group (66 eyes) were 6.23 ± 1.73 and 6.76 ± 1.89 years, respectively. The retinal vascular network density indices and the fovea avascular zone (FAZ) size were analyzed by OCT-A., Results: All of the OCT-A parafoveal vessel indices, including the vascular diameter (VD), vascular area density (VAD), vascular skeleton density (VSD), and vessel perimeter index (VPI) in the macular superficial/deep capillary plexus (SCP/DCP) as well as the FAZ in the DCP, were significantly lower in the OSA group than in the control group ( p < .05). The four parafoveal quadrants indices (temporal, superior, nasal, and inferior) of VD and VAD in both the two layer were significantly lower in the OSA group than in control group ( p < .05). However, the differences of VSD and VPI indices in the SCP/DCP did not show statistical significant changes., Conclusion: The intermittent hypoxia in children with OSA might influence the retinal capillary vascular supply. We speculate that the indices of the quadrants are lower in OSA children compared with those in healthy controls, which may be due to the change of the retinal metabolic demands. OCT-A is an ideal method to evaluate the retinal alterations in pediatric OSA., Abbreviations: OCT-A: optical coherence tomography angiography; OSA: obstructive sleep apnea; FAZ: fovea avascular zone; VD: vascular diameter; VAD: vascular area density; VSD: vascular skeleton density; VPI: vessel perimeter index; SCP/DCP: superficial/deep capillary plexus; BCVA: best corrected visual acuity; SE: spherical equivalent; AL: ocular axial length; T: temporal; N: nasal; (S): superior; (I): inferior; (SD): standard deviation.

A 53-year-old man presented with acute loss of vision, negative scotoma and dyschromatopsia in his left eye. He reported contact with people with severe respiratory syndrome - coronavirus-2 (SARS-CoV-2) 8 days prior symptoms. Funduscopic examination revealed several retinal hemorrhages. Spectral-domain optical coherence tomography showed lesions consistent with acute macular neuroretinopathy and paracentral acute middle maculopathy. Quickly after his presentation, SARSCov-2 was confirmed by chest computed tomography-scan and RT-PCR in this patient. Thrombotic complications associated with Covid-19 infection have high incidence and may involve the retina. We described a case of retinal involvement associated with Covid-19 infection., Précis: Funduscopic examination revealed retinal hemorrhages in a man with loss of vision. Optical coherence tomography showed an acute macular neuroretinopathy and paracentral acute middle maculopathy. Coronavirus disease was confirmed by chest computed tomography-scan and RT-PCR.

Purpose: To investigate the relationship between posterior staphyloma and dome-shaped macula (DSM) in highly myopic eyes., Methods: The clinical data were collected from patients with high myopia: diopter, best-corrected visual acuity, axial length, fundus images, optical coherence tomography, and 3D magnetic resonance imaging. A DSM was defined as a convex curvature of the macula in one or both of the vertical and horizontal optical coherence tomography scans. The relationship between DSM and posterior staphyloma was evaluated., Results: A total of 123 eyes were included. Dome-shaped macula was found in 18 eyes (14.63%). Twelve eyes with DSM had positive 3D magnetic resonance imaging findings. Nine eyes had horizontal oval-shaped dome, and a band-shaped inward convexity that extended horizontally from the optic disc through the fovea could be seen. Three eyes had round dome, and 3D magnetic resonance imaging showed a round inward convexity of the macular area. Five inward convexities were the border of multiple staphylomas, five were the boundary of one staphyloma, and two were within a single staphyloma., Conclusion: The formation of highly myopic DSM is related to the morphological change of the entire posterior segment.

Purpose: To determine the incidence and long-term outcome of macular atrophy (MA) after pars plana vitrectomy (PPV) in pathologic myopia., Methods: Highly myopic patients who underwent PPV for myopic traction maculopathy and macular hole retinal detachment at Tokyo Medical and Dental University between 2012 and 2016 were studied. Fundus photographs and/or optical coherence tomography were examined before and after PPV at every visit., Results: A total of 133 eyes were followed for 39 months with the mean age of 62.8 years and the mean axial length of 30.0 mm. Postoperatively, 14 eyes (10.5%) developed fovea-centered MA, observed initially as a small, isolated, whitish lesion at the center of fovea at 3.5 months after PPV. The appearance of the MA was distinctly different from the choroidal neovascularization-related MA or patchy atrophy-related MA. With time, the lesions enlarged circumferentially. In these 14 eyes, the final best-corrected visual acuity was worse than the baseline, although the difference was not significant. The occurrence of MA was significantly associated with the preoperative foveal status., Conclusion: The development of MA can occur in 11% of highly myopic eyes after PPV for myopic traction maculopathy and macular hole retinal detachment. This postoperative MA might be a new complication of pathologic myopia.

Purpose: This report examines the relationship between glaucomatous macular damage and facial recognition. In addition, it assesses the role of contrast sensitivity (CS) as an intermediary step in the causal pathway between macular damage and impairment of facial recognition., Design: Prospective cross-sectional study., Methods: This study was conducted in a single tertiary care center. The study population included 144 eyes of 72 participants with a diagnosis of open angle glaucoma in one or both eyes and a visual acuity of 20/40 or better in each eye. The presence or absence of macular damage was determined by comparing corresponding regions of the retinal nerve fiber layer and the retinal ganglion cell layer with spectral-domain optical coherence tomography with the 10-2 visual field (VF). Better and worse eye was determined by 10-2 VF mean deviations (MDs). Interventions were 1) macular function as measured by 10-2 VF and 2) CS as measured by the Freiburg Visual Acuity and Contrast Test (FrACT). The primary outcome measure was the Cambridge Face Memory Test (CFMT) score., Results: Regardless of eye, there was a significant correlation between facial recognition and 10-2 VF MD (P < .0001 better, worse eye). The 10-2 VF MD remained a significant predictor of facial recognition after adjusting for potential confounders including glaucoma severity, CS, age, and visual acuity (P = .004 better eye, P = .019 worse eye)., Conclusions: Even with good central visual acuity, patients with glaucomatous macular damage exhibit diminished facial recognition, which is partly mediated through diminished CS., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: To evaluate the detailed structural profile of dome-shaped macula and its association with myopic macular complications., Methods: This retrospective study included 147 eyes of 93 patients who were diagnosed with degenerative myopia. The height of the scleral dome and diameter of the dome base were measured via enhanced depth imaging optical coherence tomography images with 1:1 μm setting. Spherical equivalent and best-corrected visual acuity were compared in eyes with and without dome-shaped macula. In eyes with dome-shaped macula, the height and diameter of the dome were compared in eyes with and without myopic macular complications including choroidal neovascularization, myopic foveoschisis, and macular hole., Results: Dome-shaped macula was noted in 60 eyes (40.8%) of 42 patients. The mean height of the dome in the eyes with dome-shaped macula was 126.5 ± 69.4 μm (53 to 345 μm) and the mean diameter of the dome base was 2862.1 ± 794.9 μm (1567 μm to 4886 μm). In comparing eyes with and without dome-shaped macula, eyes with dome-shaped macula had higher myopia (- 13.7 diopters vs - 12.1 diopters, P = 0.022). There was no difference in visual acuity in eyes with or without dome-shaped macula (P = 0.132). The height and diameter of the dome in eyes with and without myopic foveoschisis were 78.6 ± 20.6 μm and 134.9 ± 71.6 μm, 2499.2 ± 303.1 μm and 2969.3 ± 645.7 μm, respectively (P = 0.009 and P = 0.017). However, the height and diameter of the dome were not related to the incidence of a macular hole (P = 0.324 and P = 0.605) and choroidal neovascularization (P = 0.835 and P = 0.905)., Conclusions: The prevalence of dome-shaped macula was about 40% in the eyes with degenerative myopia. Although dome-shaped macula was associated with higher degrees of myopia, a prominent dome seemed to be protective against myopic foveoschisis.

Purpose: To study changes in retinal microcirculation and the prevalence of chronic paracentral acute middle maculopathy (PAMM) lesions in patients with mild hypertension., Design: Prospective cohort study., Participants: Twenty-seven patients (21 men and 6 women; mean age, 50.3±6.3 years) with mild hypertension and with low cardiovascular risk and 24 healthy participants (15 men and 9 women; mean age, 46.3±13.0 years) were included., Methods: The foveal avascular zone (FAZ) area and the vessel density of the superficial capillary plexus (SCP) and deep capillary plexus (DCP) were assessed using OCT angiography (OCTA). The presence of chronic PAMM lesions was evaluated for both eyes by the revision of cross-sectional OCT images of 6-mm volume scans. A chronic or resolved PAMM lesion was defined as inner nuclear layer thinning with outer plexiform layer (OPL) disruption., Main Outcome Measures: Vessel density of the SCP and DCP and prevalence of chronic PAMM lesions., Results: No difference was found between the hypertensive group and healthy group in demographic and clinical characteristics, vessel density of the SCP (48.9±2.4% and 47.8±2.0%, respectively; P = 0.92), vessel density of the DCP (54.1±3.3% and 54.0±1.8%, respectively; P = 0.93), or FAZ area (0.26±0.13 mm 2 and 0.23±0.09 mm 2 , respectively; P = 0.37). Chronic PAMM lesions were found in 24 of 27 (88.9%) hypertensive patients and in 4 of 24 (16.7%) healthy individuals. The odds ratio for the presence of chronic PAMM lesions in patients with mild hypertension was 40.0 (P < 0.001)., Conclusions: Chronic PAMM lesions are highly prevalent in hypertensive patients and may represent the earliest changes in retinal microcirculation in patients with mild hypertension, before changes in OCTA parameters have become apparent., (Copyright © 2019 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Background/purpose: To present a novel case of paracentral acute middle maculopathy associated with bilateral optic disk swelling., Methods: Retrospective case report., Results: A 67-year-old woman presented with sudden onset of central vision loss and subsequent bilateral optic disk edema, retinal vessel attenuation, and anterior uveitis. Cerebrospinal fluid analysis showed signs of inflammation. Spectral domain optical coherence tomography demonstrated the pathognomonic hyperreflectivity of the middle retinal layers consistent with paracentral acute middle maculopathy. Swept-source optical coherence tomography angiography with custom vessel analysis demonstrated a 18.3% decrease in the deep retinal vascular density and 2.4 times increase in absent flow area in the affected eye compared with the fellow eye., Conclusion: This case demonstrates a novel association between paracentral acute middle maculopathy and bilateral optic disk swelling secondary to central nervous system inflammation and possible infection. Although spectral domain optical coherence tomography is valuable in detecting paracentral acute middle maculopathy, optical coherence tomography angiography with vessel analysis can provide additional insight into the disease mechanism.

Purpose: To describe clinical findings, laboratory values, and treatment response of patients with monoclonal gammopathy of undetermined significance (MGUS) demonstrating neurosensory macular detachment., Design: Retrospective case series., Participants: Seven eyes of 4 patients (3 men and 1 woman; age range, 60-81 years) with neurosensory macular detachment, treatment-resistant submacular fluid, and vitelliform material., Methods: We retrospectively reviewed the medical and ocular histories, ocular examination findings, retinal imaging, ocular disease course, and laboratory findings in 4 patients with submacular fluid associated with MGUS., Main Outcome Measures: Description of the macular findings and treatment courses of 4 patients diagnosed with MGUS maculopathy., Results: Seven eyes of 4 patients demonstrated neurosensory macular detachment with treatment-resistant submacular fluid and vitelliform material. No eyes demonstrated signs of significant hyperviscosity retinopathy. Fluorescein angiography showed no definite leakage in any involved eye. Laboratory evaluation revealed immunoglobulin G MGUS in all 4 patients. All 4 patients were resistant to treatments aimed at resolving the subretinal fluid, including some combination of anti-vascular endothelial growth factor injections, photodynamic therapy, topical dorzolamide, oral dosing of eplerenone or acetazolamide, or some combination thereof. In 3 patients, MGUS underwent malignant transformation 24 to 144 months after diagnosis, in 1 patient to lymphoplasmacytic lymphoma and in 2 patients to multiple myeloma. The fourth patient showed no evidence of malignancy 8 years after diagnosis., Conclusions: Submacular fluid without fluorescein leakage and unresponsive to conventional treatment may suggest an underlying immunoproliferative disorder that we have termed monoclonal gammopathy of macular significance. Given the propensity for monoclonal gammopathy of macular significance to transform into malignant disease in our series, serum protein analysis should be considered in patients with neurosensory macular detachment not attributable to known causes., (Copyright © 2019 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To present the first reported case of paracentral acute middle maculopathy in association with Susac syndrome., Method: Case report., Results: A young female patient presented with unilateral sudden loss of vision of her right eye to count fingers. There were numerous cotton wool spots in a multifocal pattern concentrated around the right macula and optic disk. Clinical examination and spectral domain optical coherence tomography imaging were consistent with paracentral acute middle maculopathy, and fluorescein angiography showed features of retinal arteriole vasculitis. Magnetic resonance imaging demonstrated multiple white matter lesions in a pericallosal distribution. High-dose corticosteroid therapy was commenced, and visual acuity gradually improved to 6/6 corrected. The patient reported hearing impairment and was diagnosed with Susac syndrome for which she has responded extremely well to long-term immunosuppressive therapy., Conclusion: Paracentral acute middle maculopathy can occur in association with Susac syndrome. Susac syndrome may present only with multifocal retinal ischemia, and such patients with paracentral acute middle maculopathy should be checked for characteristic magnetic resonance imaging lesions and hearing loss.

Background: The aim of the present work was to describe and compare multifocal electroretinogram findings (mfERG) between patients with sickle cell disease (SCD) without clinical sign of maculopathy and controls (HbAA)., Methods: Both HbSS (homozygous SCD) and HbSC (compound heterozygous SCD) patients, the two most frequent SCD genotypes, were included. All individuals underwent a full ophthalmologic examination (with a fundoscopy), a spectral domain ocular coherence tomography (SD-OCT) and a mfERG., Results: A total of 86 subjects were included: 54 SCD patients (107 eyes) with 32 HbSS (63 eyes) and 22 HbSC (44 eyes) and 32 controls (64 eyes). None of the eyes showed retinal clinical abnormalities. SD-OCT analysis showed that macular thickness was statistically lower in SCD eyes than in controls. mfERG analysis demonstrated a significant reduction of N1 (initial-negative deflection), and P1 (positive peak) response amplitude densities of HbSS eyes compared to HbAA eyes from the centre (<2°) and to the periphery (>15°). Implicit time response was also reduced in the centre (<2°). N1 and P1 response amplitude densities of HbSC eyes were significantly lower than those of HbAA eyes from the centre (<2°) to the periphery (>15°). N1 implicit time was statistically reduced in HbSS compared to HbSC eyes., Conclusion: Our study is the first one to describe macular electrophysiological dysfunction in SCD patients. Moreover, we confirm that SCD maculopathy is equally frequent in HbSS and HbSC.

Competing Interests: There are no conflicts of interest.

Purpose: To investigate the associations between macular complications and demographic, clinical, and optical coherence tomography characteristics (OCT) of highly myopic eyes with a dome-shaped macula (DSM)., Methods: We retrospectively analyzed the findings of 90 patients (167 eyes) with DSM who had vertical and horizontal OCT scans between January 2011 and June 2018. Demographic data, clinical characteristics, and OCT-derived morphologic parameters were determined, and the associations of these parameters with macular complications were evaluated., Results: Among 167 eyes, the following patterns were observed: horizontally oriented DSM (71%), symmetric DSM (27%), and vertically oriented DSM (2%). Complications or abnormalities involving the macular area were detected by OCT in 63% (106/167) of the eyes. Specific complications were as follows: choroidal neovascularization (CNV) (37.1%), macular retinoschisis (9.0%), serous retinal detachment (RD) (7.8%), epiretinal membrane (4.2%), lamellar macular hole (3.0%), and macular hole (2.4%). The eyes with CNV had thinner choroid (P < 0.001) and lower macular bulge height (P = 0.04). The eyes with serous RD had thicker choroid (P < 0.001) and higher macular bulge height (P < 0.001). Serous RD was significantly more common when the macular height was greater than 250 μm (P = 0.001) and if the DSM pattern was vertical (P < 0.001)., Conclusions: A greater bulge height and thicker choroid in highly myopic eyes with DSM may be protective against the development of myopic CNV. A thicker choroid, a higher macular bulge (> 250 μm), and a vertical DSM pattern are associated with a risk of developing serous RD.

Purpose: To evaluate visual and retinal changes in patients with bipolar disorder. To analyze the correlation between structural changes and visual function parameters., Methods: Thirty patients with bipolar disorder and 80 healthy controls underwent visual function evaluation with Early Treatment Diabetic Retinopathy Study charts at 100%, 2.50%, and 1.25% contrast, Pelli-Robson chart, and color vision Farnsworth and Lanthony tests. Analysis of the different retinal layers was performed using Spectralis optical coherence tomography with automated segmentation software. Correlation analysis between structural and functional parameters was conducted., Results: Patients with bipolar disorder presented worse color vision compared with controls (Lanthony's index, P = 0.002). Full macular thickness, the retinal nerve fiber layer (RNFL), ganglion cell layer, and inner plexiform layer were reduced in patients compared with healthy individuals (P < 0.005). The inner nuclear layer was significantly thickened in patients (P < 0.005). Peripapillary RNFL thickness was reduced in all temporal sectors (P < 0.005). Significant correlations were found between visual acuity and the RNFL thickness, the Pelli-Robson score and the inner plexiform layer, and between the Lanthony's color index and the ganglion cell layer thickness., Conclusion: Patients with bipolar disorder present quantifiable thinning of the macular RNFL, ganglion cell layer, and inner plexiform layer, as well as in the peripapillary RNFL thickness, and increasing thinning in the inner nuclear layer.

Background: Acute optic neuritis (AON) is a common optic nerve disease leading to retrograde degeneration of optic nerve axons, reflected by thinning of the inner retinal layers on optical coherence tomography. On the contrary, acute macular neuroretinopathy (AMN) type 2 is a rare outer retinal disorder that leads to thinning of the outer nuclear layers and is diagnosed by multimodal imaging. The aim of this study was to report a new association between these two diseases., Methods: Patients with a first episode of optic neuritis were invited to participate in a study that involved optical coherence tomography evaluation at baseline and the following 1, 3, 6 and 12 months. All the study patients underwent ophthalmologic evaluation that comprised of visual acuity, visual field and multimodal imaging as well as orbital and brain Magnetic Resonance Imaging. A diagnosis of multiple sclerosis was made according to the 2010 McDonald criteria., Results: Six of the 114 patients with acute optic neuritis also had acute macular neuroretinopathy, of whom three were positive for myelin oligodendrocyte glycoprotein antibodies (MOG-Abs), two had relapsing-remitting multiple sclerosis and one had clinical isolated syndrome., Conclusion: Our study suggests that it is imperative to check for associated AMN in cases of AON, especially those associated with MOG-Abs., (© 2019 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Background/aims: Initially reported in a few patients with homozygous sickle cell disease (SCD), atrophic areas of the retina temporal from the macula are now known to be present in about 48% of eyes of adult patients with SS-SCD and in 35% of eyes of adult patients with SC-SCD. The aim of this study is to describe this paramacular atrophy in children affected with SCD., Methods: In this retrospective series, spectral-domain optical coherence tomography images of 81 children with SCD, acquired with specific patterns including one evaluating the retina temporal to the macula, were reviewed, in order to look for retinal atrophy. Fundus examination status for SCD peripheral retinopathy was also reviewed., Results: Mean age was 12.0 years (SD: 3.56). The genotype distribution was: 64 HbSS (79%), 10 HbSC (12%) and 7 HbS/β 0 thalassaemia (9%). Using a usual fovea-centred programme, retinal atrophy was found in 38% of eyes (52% of children). Using a specific temporal pattern, retinal atrophy was found in 53% of eyes (64% of children), with no significant difference in the prevalence between HbSS and HbSC genotype (p=0.92), and no effect of age (mean 12.3 years (SD=3.61) vs11.9 (3.56), p=0.65). Peripheral retinopathy was found in 11% of children, with a significant relation between the HbSC genotype and the severity of the retinopathy (p=0.003)., Conclusion: Paramacular temporal atrophy occurs early in the course of SCD, which suggests distinct mechanisms from those of peripheral retinopathy., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: To assess the prevalence of macular abnormalities not suspected by the biomicroscopic fundus examination and identified only by macular optical coherence tomography (OCT) in the preoperative evaluation for cataract surgery in a large series of Brazilian patients., Setting: Private practice, Recife, Brazil., Design: Retrospective case series., Methods: All eyes that had cataract surgery by the same physician between August 2014 and July 2016 were eligible. Excluded were eyes with a previous diagnosis of macular abnormalities, with a suspicious biomicroscopic fundus examination, and without OCT results. Based on the preoperative macular OCT, patients were divided into the following 2 groups: those with a normal OCT and those with an abnormal OCT., Results: Nine hundred fifty-two eyes (614 patients) were included in the study. Macular OCT identified abnormalities in 47 eyes (4.9%) of 44 patients (7.2%). Thirty-one eyes (3.3%) had epiretinal membrane, 7 (0.7%) had age-related macular degeneration, 4 (0.4%) had intraretinal cysts, 4 (0.4%) had a lamellar hole, and 1 (0.1%) had a macular hole. Patients with an abnormal OCT had a statistically significant higher mean age (P = .004)., Conclusion: In the preoperative evaluation for cataract surgery in Brazilian patients, 7.2% of those with a normal biomicroscopic fundus examination had macular abnormalities that were identified only by OCT., (Copyright © 2019 ASCRS and ESCRS. Published by Elsevier Inc. All rights reserved.)