Management of autoimmune retinopathy treated with intravitreal dexamethasone implant.

PURPOSE: The purpose of this study is to evaluate clinical outcomes of autoimmune retinopathy (AIR) in the patients treated with intravitreal dexamethasone implant (IDI). METHOD: Twenty-one eyes of 11 AIR patients treated with at least 1 injection of IDI were retrospectively reviewed. Clinical outcomes before and after treatment, including best corrected visual acuity (BCVA), optic coherence tomography (OCT), fundus autofluorescence (FAF), full-field electroretinography (ff-ERG), and visual field (VF) at last visit within 6 and/or 12 months, were recorded. RESULTS: Among all the patients, 3 had cancer-associated retinopathy (CAR) and 8 had non-paraneoplastic-AIR (npAIR) with mean followed up of 8.52 ± 3.03 months (range 4-12 months). All patients achieved improved or stable BCVA within 6 and/or 12 months after the treatment. Cystoid macular edema (CME) in 2 eyes and significant retinal inflammation in 4 eyes were markedly resolved after single injection. Central retinal thickness (CFT) in all eyes without CME, ellipsoid zone (EZ) on OCT in 71.4% of eyes, ERG response in 55% of eyes, and VF in 50% of eyes were stable or improved within 6 months after treatment. At last visit within 12 months, both BCVA and CFT remained stable in the eyes treated with either single or repeated IDI; however, progression of EZ loss and damage of ERG response occurred in some patients with single IDI. CONCLUSION: Clinical outcomes, including BCVA and parameters of OCT, ERG, and VF, were stable or improved after IDI in a majority of AIR patients. Local treatment of AIR with IDI was a good option to initiate the management or an alternative for the patients' refractory to the systemic therapy but with limited side effect.

Purtscher-like retinopathy following coronary artery bypass grafting in an antiphospholipid syndrome patient: a case report.

BACKGROUND: Purtscher retinopathy is a rare occlusive microangiopathy comprising a constellation of retinal signs including cotton wool spots, retinal hemorrhages and Purtscher flecken. While classical Purtscher must be antedated by a traumatic incident, Purtscher-like retinopathy is used to refer to the same clinical syndrome in the absence of trauma. Various non-traumatic conditions have been associated with Purtscher-like retinopathy e.g. acute pancreatitis, preeclampsia, parturition, renal failure and multiple connective tissue disorders. In this case study, we report the occurrence of Purtscher-like retinopathy following coronary artery bypass grafting in a female patient with primary antiphospholipid syndrome (APS). CASE PRESENTATION: A 48-year-old Caucasian female patient presented with a complaint of acute painless diminution of vision in the left eye (OS) that occurred approximately two months earlier. Clinical history revealed that the patient underwent coronary artery bypass grafting (CABG) two months earlier and that visual symptoms started 4 days thereafter. Furthermore, the patient reported undergoing percutaneous coronary intervention (PCI) one year before for another myocardial ischemic event. Ophthalmological examination revealed multiple yellowish-white superficial retinal lesions i.e. cotton-wool spots, exclusively in the posterior pole and predominantly macular within the temporal vascular arcades only OS. Fundus examination of the right eye (OD) was normal and the anterior segment examination of both eyes (OU) was unremarkable. A diagnosis of Purtscher-like retinopathy was made based on clinical signs, suggestive history and consolidated by fundus fluorescein angiography (FFA), spectral domain optical coherence tomography (SD-OCT) and optical coherence tomography angiography (OCTA) of macula, optic nerve head (ONH) according to the diagnostic guidelines of Miguel. The patient was referred to a rheumatologist to identify the underlying systemic cause and was diagnosed with primary antiphospholipid syndrome (APS). CONCLUSIONS: We report a case of Purtscher-like retinopathy complicating primary antiphospholipid syndrome (APS) following coronary artery bypass grafting. This conveys a message to clinicians that patients presenting with Purtscher-like retinopathy should undergo meticulous systemic work-up in order to identify potentially life-threatening underlying systemic diseases.

Coincident Acute Macular Neuroretinopathy and Paracentral Acute Middle Maculopathy in COVID-19.

An ophthalmology consultation was requested for a 29-year-old woman complaining of visual field defects. The patient had presented to the emergency department with cough and high fever one day earlier. Chest computed tomography demonstrated pneumonia and two severe acute respiratory syndrome coronavirus 2 polymerase chain reaction tests were positive. The patient had undergone renal transplantation 11 years ago due to glomerulonephritis. Best-corrected visual acuity (BCVA) was 20/40 in the right eye and 20/30 in the left eye. Fluorescein angiography showed macular hypoperfusion, and optical coherence tomography (OCT) showed hyperreflectivity in the inner nuclear, outer plexiform, and outer nuclear layers, as well as discontinuity of the ellipsoid zone. Perimetry confirmed bilateral central scotoma. Levels of D-dimer and fibrinogen were 0.86 g/mL and 435.6 g/mL, respectively. The patient was diagnosed as having concurrent acute macular neuroretinopathy and paracentral acute middle maculopathy and was given low-molecular-weight heparin treatment for one month. Her BCVA improved to 20/20 in both eyes, and regression was observed in the retinal findings, hyperreflectivity and ellipsoid zone disruption on OCT, and scotoma in perimetry. Inflammation, thrombosis, and glial involvement may play a role in the pathogenesis of retinal microvascular impairment in COVID-19.

Routine Ophthalmological Examination Rates in Adults with Sickle Cell Disease Are Low and Must Be Improved.

The American Academy of Ophthalmology and the National Heart, Lung and Blood Institute recommend patients with sickle cell disease (SCD) undergo dilated funduscopic exams (DFE) every 1-2 years to screen for sickle retinopathy. There is a paucity of data on the adherence rate to these guidelines; a retrospective study was performed to evaluate our institution's adherence. A chart review of 842 adults with SCD, seen 3/2017-3/2021 in the Montefiore healthcare system (All Patients), was done. Only about half of All Patients (n = 842) had >1 DFE during the study period (Total Examined Patients, n = 415). The Total Examined Patients were categorized as screening, those without retinopathy (Retinopathy-, n = 199), or follow-up, including individuals previously diagnosed with retinopathy (Retinopathy+, n = 216). Only 40.3% of screening patients (n = 87) had DFE at least biennially. As expected, there was a significant decrease in the average DFE rate of the Total Examined Patients after the COVID-19 pandemic started (13.6%) compared to pre-COVID (29.8%, p < 0.001). Similarly, there was a significant decrease in the screening rate of Retinopathy- patients from 18.6% on average pre-COVID to 6.7% during COVID (p < 0.001). This data shows the sickle retinopathy screening rate is low and innovative approaches may need to be employed to remedy this issue.

Bilateral Purtscher-Like Retinopathy Associated With COVID-19 Infection.

This case report describes a 37-year-old female individual who presented with sudden-onset blurred vision in both eyes 4 days after testing positive for COVID-19.

Multiple Evanescent White Dot Syndrome Associated with Coronavirus Infection: A Case Report.

Coronavirus disease 2019 (COVID-19) causes ocular manifestations in approximately 11% of patients. Most patients typically develop ocular symptoms within 30 days of the onset of the first COVID-19 symptoms. The most common ocular manifestation is conjunctivitis, which affects nearly 89% of patients with eye problems. Other much less common anterior segment abnormalities caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) are scleritis, episcleritis, and acute anterior uveitis. Posterior segment abnormalities caused by SARS-CoV-2 are mainly vascular, such as hemorrhages, cotton wool spots, dilated veins, and vasculitis. Herein, we report a rare manifestation of COVID-19 and multiple evanescent white dot syndrome (MEWDS) of the retina. In April 2021, a 40-year-old female patient was admitted to the Eye Clinic of Clinical Center of Montenegro (Podgorica, Montenegro). The patient's main complaint was sudden vision impairment, which occurred 14 days after a positive polymerase chain reaction (PCR) test result for SARS-CoV-2 infection. A complete eye examination was performed, followed by fundoscopy, optical coherence tomography (OCT), and fluorescein angiography (FA) tests. The results showed retinal changes associated with MEWDS. The patient underwent additional examinations to rule out common causes of multifocal retinitis, all of which were unremarkable. Therefore, it was concluded that retinitis was a complication of COVID-19. Given its non-invasive nature, fundus examination should be used as a standard screening method for retinal changes in patients with COVID-19.

Bilateral radiation retinopathy 17 years following radiotherapy for nasopharyngeal carcinoma: A diagnostic and therapeutic challenge during COVID-19 lockdown.

A 40-year-old male presented with reduced vision in the right eye for one week. He had a history of nasopharyngeal carcinoma for which 34-Gy radiation was administered. The best-corrected visual acuity (BCVA) was 20/40 in the right eye and 20/20 in the left eye. Anterior segment examination suggested a bilateral early posterior subcapsular cataract. Fundoscopy revealed bilateral localized telangiectasia and macular edema in the right eye. Diagnosis of bilateral extremely delayed onset radiation retinopathy with right eye macular edema was made. Three doses of intravitreal bevacizumab injection were administered in the right eye. The patient was lost to follow-up due to COVID-19 and presented with recurrence.

Unilateral Purtscher-like retinopathy post-COVID-19.

A 32-year-old male with no known systemic illness presented with unilateral Purtscher-like retinopathy in his left eye 2 weeks after recovering from a severe COVID-19 infection. Fundus examination revealed areas of intraretinal whitening and few cotton wool spots. Multimodal imaging findings were consistent with embolic occlusion of capillaries seen in Purtscher-like retinopathy. The case highlights the effect of virus-directed coagulation cascade activation leading to unilateral microvasculopathy in our patient. The case adds to the spectrum of COVID-19 retinopathy and presses that retina screening strategies should be established for patients suffering from or recovering from severe COVID-19 infection.

Acute macular neuroretinopathy following COVID-19 infection.

COVID-19 is a global pandemic with over 600 million cases worldwide and over 1.7 million cases in New Zealand to date. The most recent spread of Omicron variant saw widespread infection across the country that was unable to be controlled like the initial Alpha or Delta variants. There is limited information on ocular complications of COVID 19. In our case, there was a close relationship between time of COVID-19 infection and acute visual changes including ongoing scotomas (blind spots). This report explores a case of a young female with positive visual phenomena following COVID-19 infection, with the diagnosis of acute macula neuroretinopathy.

Purtscher-like retinopathy in a patient with COVID-19-associated coagulopathy.

The most frequently reported ophthalmic manifestation of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is conjunctivitis. We have described a case of Purtscher-like retinopathy in a patient with severe coronavirus disease 2019 (COVID-19)-associated coagulopathy. A young woman with multiple comorbidities was admitted for COVID-19-related acute respiratory distress syndrome. Her course was complicated by fungemia. Ophthalmic examination revealed bilateral posterior pole, intraretinal lesions and fluconazole was added for presumed fungal retinitis. At 1-week follow-up, widespread peripapillary cotton-wool spots and hemorrhages suggestive of Purtscher-like retinopathy were observed. The levels of D-dimers, fibrinogen, and C-reactive protein were markedly elevated prior to our consultation, indicating preceding prothrombotic and pro-inflammatory states. Subsequent venous duplex revealed deep venous thrombosis in the right subclavian and internal jugular veins. Von Willebrand factor indices were markedly elevated, suggesting severe COVID-19-associated coagulopathy. Purtscher-like retinopathy, a rare occlusive microangiopathy has been described in various pro-inflammatory and prothrombotic conditions. To the best of our knowledge, this is the first report of Purtscher-like retinopathy in COVID-19-associated coagulopathy.

Multiple evanescent white dot syndrome following SARS-CoV-2 infection - A case report.

To report a case of MEWDS post-COVID-19 infection with multimodal imaging. A 17-year-old boy reported blurring of vision in both eyes 2 months and 10 days following the SARS-CoV-2 infection. Fundus examination revealed hypopigmented lesions nasal to the optic disc, inferior periphery, and near macula in both eyes. Multimodal imaging was consistent with MEWDS. A complete systemic workup was performed to exclude infectious etiology. Fundus lesions resolved with a course of oral corticosteroids and was confirmed on multimodal imaging.

BRANCH RETINAL ARTERY OCCLUSION WITH PARACENTRAL ACUTE MIDDLE MACULOPATHY PRESUMABLY RELATED TO HEAVY CANNABIS USE.

PURPOSE: To report a case of branch retinal artery occlusion associated with paracentral acute middle maculopathy on spectral-domain optical coherence tomography presumably related to heavy cannabis consumption. METHODS: Retrospective case report. Spectral-domain optical coherence tomography, fluorescein angiography, and optical coherence tomography angiography were performed. RESULTS: A 21-year-old healthy man described the acute onset of superior visual field loss in his right eye. He admitted smoking approximately 15 g daily of cannabis for several weeks during COVID-19 confinement. Ophthalmoscopic examination of the right eye showed inferotemporal retinal whitening. Spectral-domain optical coherence tomography illustrated evidence of the ischemic cascade with diffuse hyperreflectivity of the inner and middle retinal layers within the central region of the retinal infarct and paracentral acute middle maculopathy at the border of the infarct. Optical coherence tomography angiography demonstrated predominant flow signal loss at the level of the deep retinal capillary plexus. Fluorescein angiography and complete systemic workup were unremarkable. CONCLUSION: Branch retinal artery occlusion and paracentral acute middle maculopathy may be related to heavy cannabis use as the result of transient arterial vasospasm.

Paracentral acute middle maculopathy in Susac syndrome after dual exposure to SARS-CoV-2 antigen.

We report a case of Susac syndrome after SARS-CoV-2 infection and subsequent vaccination that presented with meningitis and retinal microembolisation in the form of paracentral acute middle maculopathy (PAMM). After presenting with headache, fever and myalgia followed by scotomata, a woman in her 50s was hospitalised for meningitis; she had had mild COVID-19 infection 2 months prior to admission, having received the first vaccine dose 1 month prior to the neurological manifestation. Eye fundus examination and optical coherence tomography were suggestive of PAMM. D-dimer levels and erythrocyte sedimentation rate were elevated. Before infectious investigation results were available, she was started on empirical antibiotic and antiviral treatment. Having ruled out infectious causes, she was started on high-dose prednisolone. After 1 month, there was partial resolution of retinal lesions. This case highlights that exposure to SARS-CoV-2 antigen may be related to this rare syndrome; treatment with steroids may improve central and retinal impairment.

Multiple Evanescent White Dot Syndrome Following Medigen Vaccine Biologics Corporation COVID-19 Vaccination.

PURPOSE: To report a case of multiple evanescent white dot syndrome (MEWDS) following severe acute respiratory syndrome coronavirus 2 vaccination. STUDY DESIGN: Case report. RESULTS: A 36-year-old healthy Taiwanese female was presented with flashing lights in the right eye two days after the first dose of Medigen Vaccine Biologics Corporation (MVC) coronavirus disease 19 (COVID-19) vaccine. Examination of the retina revealed multiple white dots in the posterior pole extending to the mid-periphery. Disruption of ellipsoid zone on optical coherence tomography, early hyperfluorescence on fluorescein angiography, late hypo-cyanescence on indocyanine green angiography, and paracentral scotoma on visual field test were consistent with MEWDS. At four-week follow-up, the patient's fundus lesions resolved, and symptoms subsided without treatment. CONCLUSION: Resembling previous post-vaccine MEWDs cases, the symptoms are self-limited, and the visual prognosis is excellent. The presented case demonstrates MEWDS following MVC COVID-19 vaccine and suggests the immune-mediated basis for MEWDS in predisposed patients.

Changes in the Incidence of Retinal Vascular Occlusions After COVID-19 Diagnosis.

Importance: COVID-19 is associated with systemic vascular damage; however, the risk posed to the retinal vasculature remains incompletely understood. Objective: To assess if there is a change in the incidence of retinal vascular occlusions after COVID-19 infection. Design, Setting, and Participants: This cohort study at an integrated health care organization (Kaiser Permanente Southern California) included patients without a history of retinal vascular occlusion who were diagnosed with COVID-19 infection between January 20, 2020, and May 31, 2021. Patients were excluded if they had a history of retinal artery occlusions (RAOs) or retinal vein occlusions (RVOs) more than 6 months before their COVID-19 diagnosis or if they were enrolled in Kaiser Permanente Southern California for less than 6 months before COVID-19 diagnosis. Exposures: COVID-19 infection. Main Outcomes and Measures: The change in the average biweekly incidence of new RAOs and RVOs after COVID-19 diagnosis. Adjusted incidence rate ratios (IRRs) were calculated to compare the incidence of retinal vascular occlusions before and after COVID-19 diagnosis after accounting for baseline demographic characteristics, medical history, and hospitalization. Results: A total of 432 515 patients diagnosed with COVID-19 infection were included in this study. The mean (SD) age was 40.9 (19.2) years, and 231 767 patients (53.6%) were women. Sixteen patients had an RAO (crude incidence rate, 3.00 per 1 000 000 patients), and 65 had an RVO (crude incidence rate, 12.20 per 1 000 000 patients) in the 6 months after COVID-19 diagnosis. The incidence of new RVOs was higher in the 6 months after COVID-19 infection compared with the 6 months before infection after adjusting for age; sex; self-reported race and ethnicity; body mass index; history of diabetes, hypertension, or hyperlipidemia; and hospitalization (adjusted IRR, 1.54; 95% CI, 1.05-2.26; P = .03). There was a smaller increase in the incidence of RAOs after COVID-19 diagnosis (IRR, 1.35; 95% CI, 0.64-2.85; P = .44). The peak incidence of RAOs and RVOs occurred 10 to 12 weeks and 6 to 8 weeks after COVID-19 diagnosis, respectively. Conclusions and Relevance: The findings of this study suggest that there was an increase in the incidence of RVOs after COVID-19 infection; however, these events remain rare, and in the absence of randomized controls, a cause-and-effect relationship cannot be established. Further large, epidemiologic studies are warranted to better define the association between retinal thromboembolic events and COVID-19 infection.

Nonmydriatic Ultra-Widefield Fundus Photography in a Hematology Clinic Shows Utility for Screening of Sickle Cell Retinopathy.

PURPOSE: To determine the feasibility and accuracy of nonmydriatic ultra-widefield (UWF) fundus photographs taken in a hematology clinic setting for screening of sickle cell retinopathy (SCR) DESIGN: Prospective cohort study. METHODS: This single-site study took place at the Johns Hopkins Sickle Cell Center for Adults and the Wilmer Eye Institute. The study population was 90 eyes of 46 consecutive adults with sickle cell disease (SCD). Bilateral nonmydriatic fundus photos taken by clinic personnel during the participants' routine hematology appointment were graded by 2 masked retina specialists at the Wilmer Eye Institute for the presence of nonproliferative SCR (NPSR) and proliferative sickle retinopathy (PSR). A third retina specialist adjudicated in cases of grader disagreement. All participants underwent the standard dilated fundus examination (DFE) within 2 years of acquisition of UWF photographs. The main outcome measure was the sensitivity and specificity of nonmydriatic UWF images for the detection of NPSR and PSR RESULTS: PSR was noted in 19 of 90 eyes that underwent DFE and in 9 of 67 gradable UWF images. Interrater agreement between the 2 graders was moderate, with κ = 0.65 (range 0.43-0.87) for PSR. For gradable UWF photos, the sensitivity and specificity of detecting SCR using the nonmydriatic photos compared with the DFE were 85.2% and 62.5% for NPSR, respectively, and 69.2% and 100% for detection of PSR, respectively. One participant required ophthalmic therapy in both eyes for active sea-fan neovascularization. CONCLUSIONS: UWF imaging shows utility in screening for SCR and may help identify patients with PSR who require a DFE and who may benefit from treatment.