A Case of Concurrent Acute Macular Neuroretinopathy and Paracentral Acute Middle Maculopathy Following Pfizer-BioNTech COVID-19 Vaccination.

We present a 65-year-old woman who developed sudden and severe vision loss in her left eye one day after the administration of the second dose of COVID vaccine. The best corrected visual acuity in this eye was 1/10. Diffuse paracentral acute middle maculopathy was detected on spectral domain optical coherence tomography (OCT). OCT angiography images revealed concurrent vascular flow defects consistent with acute macular neuroretinopathy in the deep retinal capillary plexus and choriocapillaris layers. At the end of the six-month follow-up, there was no improvement in visual acuity, and atrophy and thinning developed in all layers of the retina.

Bilateral Acute Macular Neuroretinopathy after COVID-19 Vaccination and Infection.

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.

Presumed Recurrent MEWDS following Covid-19 Vaccination.

PURPOSE: To present an unusual case of recurrent multiple evanescent white dot syndrome (MEWDS) following the coronavirus 2019 (Covid-19) vaccination. METHODS: Review of the clinical, laboratory, photographic, and angiographic records of a patient with MEWDS. RESULTS: A 49-year-old female who had MEWDS nine years ago presented to our clinic with blurred vision in her left eye. These symptoms occurred 2 days following Covid-19 vaccination. Fundus examinations and diagnostic testing were consistent with recurrent MEWDS. CONCLUSION: While rare, MEWDS can reoccur following Covid-19 vaccination.

Acute Macular Neuroretinopathy following Coronavirus Disease 2019 Vaccination.

Purpose: To report acute macular neuroretinopathy (AMN) in two young women two days after receiving Vaxzevria Coronavirus disease (COVID-19) vaccination.Methods: Observational case reports.Observation: The first patient was an Italian 22-year-old female with acute onset of paracentral scotoma two days post vaccination. The second patient was an Austrian 28-year-old female who presented with sudden onset paracentral scotoma two days after vaccination. Multimodal retinal imaging was consistent with AMN in both cases. Both patients were on long-term oral contraceptives, had no history of COVID-19 and experienced one-day duration fever the day after the vaccination.Conclusions: Vaccination may represent a possible risk factor for AMN onset in women on oral contraceptives. We encourage ophthalmologists to investigate recent vaccination status when dealing with new onset AMN. Further studies are needed to assess the link between vaccinations and AMN.

Paracentral acute middle maculopathy in patient with atherosclerosis and angioplasty with stents in the coronary artery.

Sophisticated imaging systems have helped to redefine the clinical presentation of acute macular neuroretinopathy and have markedly enhanced diagnostic sensitivity. The proposed mechanism of paracentral acute middle maculopathy is related to ischemia at the level of the superficial and deep retinal capillary plexi. This is a case report of a patient who developed an acute macular neuroretinopathy after an uneventful angioplasty with stents in the coronary artery.

Association of Acute Macular Neuroretinopathy or Paracentral Acute Middle Maculopathy with Sickle Cell Disease.

PURPOSE: To investigate the structural and microvascular changes in the retina and choriocapillaris in patients with sickle cell disease (SCD) demonstrating acute vision loss and acute macular neuroretinopathy (AMN) or paracentral acute middle maculopathy (PAMM) using multimodal imaging including OCT and OCT angiography (OCTA). DESIGN: Retrospective case series. PARTICIPANTS: Four hemoglobin SS (HbSS) or hemoglobin SC (HbSC) patients who demonstrated vision loss attributed to AMN (n = 2) or PAMM (n = 2). METHODS: Clinical characteristics including best-corrected visual acuity and multimodal imaging features from fundus photography, fluorescein angiography, OCT, and OCTA were analyzed. MAIN OUTCOME MEASURES: Longitudinal changes in the structure and microvasculature of the retina and choriocapillaris were examined. RESULTS: In 2 patients with AMN, characteristic hyperreflective changes were observed in the outer nuclear layer with involvement of the ellipsoid and interdigitation zones. In 1 patient, the lesion was located in the foveal avascular zone, and only flow deficits in the choriocapillaris were noted. In the second patient, no flow loss was observed in the superficial capillary plexus (SCP) corresponding to the lesion, but flow loss was seen in the deep capillary plexus (DCP) and choriocapillaris. At the respective 1-year and 4-month follow-up examinations, the hyperreflectivities improved with no ensuing retinal atrophy. Deep capillary plexus and choriocapillaris flow also improved. Of the 2 patients with PAMM, 1 had PAMM in association with branch retinal arteriolar occlusions. In both patients, a hyperreflective band involving the inner plexiform, inner nuclear, and outer plexiform layers was seen in or adjacent to areas of prior macular thinning. These areas corresponded to flow deficits in both the SCP and DCP. At the respective 1-month and 1-year follow-up examinations, the hyperreflective band improved with ensuing atrophy of the middle retinal layers. OCT angiography demonstrated worsening of flow deficits in the corresponding SCP and DCP. CONCLUSIONS: To the best of our knowledge, this is the first report of AMN and the largest case series of AMN or PAMM in SCD to date. Paracentral acute middle maculopathy may precede macular thinning commonly observed in SCD. OCT angiography is a useful tool to demonstrate associated microvascular changes in AMN and PAMM in SCD.

Multimodal Imaging Characteristics and Functional Test Findings in a Case of Acute Macular Neuroretinopathy Accompanied by Behçet Disease.

Purpose: To report a case of acute macular neuroretinopathy (AMN) in Behçet Disease.Case: A 23-year-old male presented with a complaint for central scotoma in his right eye. He had been diagnosed with Behçet Disease 3 years ago. Best-corrected visual acuity (BCVA) was 20/20. Anterior chamber and fundus examinations were unremarkable. Optical coherence tomography revealed a paracentral area of outer nuclear layer thinning. Infrared reflectance showed a well-defined, circular, hyporeflective area. Optical coherence tomography angiography revealed an area of capillary dropout in deep retinal capillary plexus corresponding to that hyporeflective lesion. Microperimetry test showed decreased macular sensitivity on the lesion area and the loss of the macular integrity. In multifocal electroretinogram, diminished amplitudes of the central cone responses were detected nasal to fixation.Conclusion: Behçet disease is a cause of occlusive retinal vasculitis. Accompanied retinal microvascular disease may be a possible risk factor of AMN suggesting ischemic etiopathogenesis for AMN.

Acute Macular Neuroretinopathy Associated with Acute Influenza Virus Infection.

Purpose: To describe a prospective case series of patients with acute macular neuroretinopathy (AMN) associated with acute influenza virus infectionMethods: Patients who presented with acute macular neuroretinopathy associated with confirmed influenza virus infection were subject to a detailed clinical history, HLA typing and longitudinal ophthalmological and imaging examinations.Results: Four female patients aged 18 to 32 years were studied. They reported the onset of ocular symptoms between 2 and 5 days after the development of flu like symptoms. Three patients had confirmed acute influenza B infection, while the fourth had influenza A. OCT angiography only demonstrated abnormal choriocapillaris perfusion in 1 patient and early oral Oseltamivir treatment appeared not to affect the ophthalmic outcome in one patient.Conclusion: This is the first report of AMN associated with virologically confirmed acute influenza virus infection. Variation in HLA alleles do not appear to predispose patients to influenza associated AMN.

Paracentral acute middle maculopathy in patient with atherosclerosis and angioplasty with stents in the coronary artery / Maculopatia média aguda paracentral em paciente com arterioesclerose e angioplastia com stent na artéria coronária

ABSTRACT Sophisticated imaging systems have helped to redefine the clinical presentation of acute macular neuroretinopathy and have markedly enhanced diagnostic sensitivity. The proposed mechanism of paracentral acute middle maculopathy is related to ischemia at the level of the superficial and deep retinal capillary plexi. This is a case report of a patient who developed an acute macular neuroretinopathy after an uneventful angioplasty with stents in the coronary artery.

RESUMO Sistemas de imagem sofisticados ajudaram a redefinir a apresentação clínica da neurorretinopatia macular aguda e têm sensibilidade diagnóstica marcadamente aumentada. A maculopatia média aguda paracentral tem sido relacionada à isquemia ao nível dos plexos capilares superficial e profundo da retina. Este é um relato de caso de paciente que desenvolveu uma neurorretinopatia macular aguda após uma cirurgia de angioplastia com stents da artéria coronária sem complicações.

Acute macular neuroretinopathy in a patient with acute coronary syndrome.

A young man presented with bilateral diminution of vision after acute coronary syndrome. Fundus showed dark wedge-shaped perifoveal lesions in both eyes. Fundus fluorescein angiography and indocyanine angiography were normal. Optical coherence tomography showed characteristic findings of outer nuclear layer thinning and disruption of ellipsoid zone. Optical coherence tomography angiography showed flow voids in deep capillary plexus. A diagnosis of acute macular neuroretinopathy was made. The multimodal imaging findings and pathophysiology of such a scenario are presented.

Photopsia.

'Photopsia' describes the symptom of visual disturbances that are typically flash-like, sudden in onset and brief, and occurring without light entering the eye. Patients reporting photopsia often pose a diagnostic challenge, given the wide range of possible neurological and ophthalmological causes. We review the common causes of photopsia, discuss the assessment and workup of this symptom, and stress the importance of close interdisciplinary liaison to help with its diagnosis and management. We discuss a patient with acute zonal occult outer retinopathy to illustrate these points.

Acute Macular Neuroretinopathy in Purtscher Retinopathy.

Purtscher retinopathy and acute macular neuroretinopathy are two rare clinical disorders that are both probably associated with ischemic pathogenesis. In this report, we describe for the first time the coexistence of Purtscher retinopathy and acute macular neuroretinopathy in a patient with visual complaints after chest trauma. Optical coherence tomography (OCT) scans demonstrated outer retinal defects, while OCT angiography illustrated areas of hypoperfusion in the superficial and deep capillary plexuses as well as the choriocapillaris. In this report, it is emphasized that acute macular neuroretinopathy is a clinical condition that should be kept in mind in patients presenting with post-traumatic vision loss. Although its clinical diagnosis is difficult, characteristic OCT and OCT angiography findings facilitate diagnosis.

A CARE-compliant article: a case report of possible association between recurrence of multiple evanescent white dot syndrome and the Herpesviridae family.

RATIONALE: Multiple evanescent white dot syndrome (MEWDS) is a self-limited multifocal chorioretinopathy that typically affects otherwise healthy young females in the second to fourth decades of life. Current understanding of the pathophysiology of MEWDS is still limited. One of the possible underlying causes is an infectious etiology. PATIENT CONCERNS: A 24-year-old female with recurrent episodes of typical MEWDS ocular manifestation was observed over 2 years. Viral-specific antibody serologic tests showed evidence of exposure to the Herpesviridae family during the acute stage of MEWDS in the first and recurrent episodes. DIAGNOSES: MEWDS was diagnosed by the clinical findings and ancillary testing results of fundus photography, optical coherence tomography, fluorescein angiography, indocyanine green angiography and electroretinogram. The laboratory serology data was positive for varicella-zoster virus (VZV) immunoglobulin M (IgM) in the first episode and exhibited high Epstein-Barr virus (EBV) elevated immunoglobulin G (IgG) titer in the recurrent episode. INTERVENTIONS: Due to the self-limited nature of MEWDS, we observed the clinical course without intervention. OUTCOMES: During acute onset of MEWDS, serologic data for VZV IgM antibody was positive in the first episode. Two years later, the patient had recurrent episodes of MEWDS in the contralateral eye. Serologic study showed highly elevated IgG titer (1:160) of Epstein-Barr virus capsid antigen (EB-VCA) in the acute stage. The follow-up paired serum virus serology test showed that the prior EB-VCA IgG titer decreased fourfold to 1:40 in the recovery stage. LESSONS: Recurrence of MEWDS may be associated with acute systemic infection of the Herpesviridae family or virus-induced autoimmune inflammatory reaction.

ACUTE MACULAR NEURORETINOPATHY IN SUSAC SYNDROME: A NEW ASSOCIATION.

PURPOSE: To report a case of acute macular neuroretinopathy in a patient with Susac syndrome. METHODS: Case report. RESULTS: A 39-year-old male patient presented with severe headache, photopsias, and a sudden onset of hearing loss in the right ear. Fluorescein angiography of the right eye revealed multiple branch retinal artery occlusions. Clinical presentation of encephalopathy, hearing loss, and branch retinal artery occlusions, along with characteristic magnetic resonance imaging findings, led to a diagnosis of Susac syndrome. Despite aggressive immunosuppression for four months, the patient later presented with acute macular neuroretinopathy in the left eye. CONCLUSION: Acute macular neuroretinopathy and Susac is a new association of two well-defined disorders. The concurrence of both disorders supports retinal ischemia as the proximate cause of acute macular neuroretinopathy and inflammation as a potential etiology.

Multiple Evanescent White Dot Syndrome Following Acute Epstein-Barr Virus Infection.

PURPOSE: To investigate the association between multiple evanescent white dot syndrome (MEWDS) and Epstein-Barr (EB) virus infection. METHODS: A prospective, consecutive case series study was performed in patients with the characteristic findings of MEWDS. Patients received EB viral-specific antibody serologic tests. RESULTS: Five cases of MEWDS who had prodromal flu-like symptoms were enrolled, comprising 2 women and 3 men with a mean age of 34. Mean diopter of myopia was -7.5. During acute onset of MEWDS, EB virus infection was confirmed by positive EB virus serology test. One showed positive EB viral capsid antigen (EB-VCA) IgM, and the other four showed highly elevated titer of EB-VCA IgG more than 1:160. Two months later, paired serum virus serology data showed negative EB-VCA IgM, or prior EB-VCA IgG titer decreased four-fold in the recovery stage. CONCLUSION: MEWDS may be associated with acute systemic EB virus infection. Ocular symptoms might develop due to this infection or represent virus-induced autoimmune inflammatory retinitis.

Multiple Evanescent White Dot Syndrome Following Intradermal Influenza Vaccination.

Purpose: To present a case report of MEWDS following an intradermal influenza vaccination. Methods: Review of the clinical, laboratory, photographic, and angiographic records of a patient with MEWDS. Results: 27-year-old female that presented to our clinic with photopsias OD. These symptoms began 2 weeks following an influenza intradermal vaccination. Fundus examination and diagnostic testing were consistent with MEWDS. Conclusion: While rare, MEWDS can occur following influenza vaccination.