Punctate Inner Choroidopathy (PIC) disease recurrence with inflammatory choroidal neovascular membrane (iCNVM) post-COVID-19 vaccine.

PURPOSE: To report a recurrence of punctate inner choroidopathy (PIC) with an inflammatory choroidal neovascular membrane (iCNVM) after the Pfizer-BioNTech COVID-19 vaccine. METHODS: Case report. RESULTS: A 38-year-old female with a history of myopia and previous episodes of PIC and iCNVM presented with distorted vision in her right eye, seven days after receiving the first dose of the Pfizer-BioNTech COVID-19 vaccine. The patient exhibited active PIC lesions with iCNVM confirmed on multimodal imaging. Treatment with a combination of oral corticosteroids and intravitreal anti-VEGF injection led to disease resolution. Subsequent COVID-19 vaccinations, administered while the patient was immunosuppressed, did not lead to disease relapse. However, relapse occurred following the fourth COVID-19 vaccine, when the patient was not immune suppressed. CONCLUSION: This case highlights the potential risk of PIC disease relapse following COVID-19 vaccination. Further research is needed to investigate the relationship between COVID-19 vaccination and PIC exacerbation, as well as to determine optimal management strategies in this population, including close observation and consideration of prophylactic immune suppression at the time of COVID-19 vaccine for high-risk individuals.

Multiple Evanescent White Dot Syndrome Following Adenovirus Vector-Based COVID-19 Vaccine (Covishield).

PURPOSE: To report a case of multiple evanescent white dot syndrome (MEWDS) following adenovirus vector-based Coronavirus disease 2019 (COVID-19) vaccine, Covishield and to present a summary of previously reported cases of MEWDS following COVID-19 vaccines. METHODS: Retrospective case report and review of literature. RESULTS: A 22-year-old Indian female presented with blurred vision, scotomata, and photopsias in her left eye, a day after administration of second dose of Covishield vaccine. Her clinical findings and imaging features confirmed the diagnosis of MEWDS. Her symptoms resolved spontaneously after 2 weeks. CONCLUSION: This is the first reported case of MEWDS following an adenovirus vector-based COVID-19 vaccine. Comparison with previously reported cases of MEWDS following COVID-19 vaccination showed that patients are generally healthy, young to middle-aged women, who develop symptoms after a median time of one week and recover spontaneously over a median period of 4 weeks.

Multiple Evanescent White Dot Syndrome and Choroidal Neovascularization following SARS-COV-2 Infection in a Patient on Dabrafenib and Trametinib.

PURPOSE: to describe multimodal imaging and electrophysiology of multiple evanescent white dot syndrome (MEWDS) concomitant with COVID-19 infection in a patient on BRAF (B Rapidly Accelerated Fibrosarcoma) and MEK (Mitogen-activated Protein Kinase) inhibitors. METHODS: observational case report and literature review. RESULTS: a 37-year-old woman affected by cutaneous melanoma on BRAF and MEK inhibitors developed visual symptoms in the right eye simultaneously with a SARS-COV-2 infection. The right eye visual acuity was hand movement, and clinical examination disclosed vitreous cells, yellow-white retinal spots, and macular yellowish material. Fundus autofluorescence and angiograms were consistent with MEWDS. Angiograms, optical coherence tomography, and optical coherence tomography angiography revealed a macular choroidal neovascular membrane. The infectious and inflammatory work-up was negative. Electrodiagnostic tests revealed cone dysfunction. MEWDS resolved and anti-VEGF treatment allowed partial vision recovery. CONCLUSION: the case illustrates the association of MEWDS and choroidal neovascularization developing after COVID-19 infection in the setting of immunotherapy.

ACUTE MACULAR NEURORETINOPATHY AFTER SARS-COV-2 VACCINATION.

PURPOSE: To present the rare case of a patient who developed acute macular neuroretinopathy (AMN) after administration of a single-dose adenovector coronavirus vaccine. METHODS: Retrospective chart review. RESULTS: A 26-year-old woman presented with paracentral scotomas in both eyes that acutely developed 2 days after administration of a single-dose adenovector SARS-CoV-2 vaccine (Johnson & Johnson, New Brunswick, NJ). She had previously received the seasonal influenza immunization without any symptoms and denied having any recent history of viral illnesses. On examination, optical coherence tomography showed parafoveal hyperreflective bands in the outer retina of both eyes without retinal thickening and near-infrared reflectance showed wedge-shaped parafoveal lesions pointing to the fovea, both classic findings in acute macular neuroretinopathy. DISCUSSION: This report highlights the development of acute macular neuroretinopathy after a SARS-CoV-2 vaccination in an otherwise healthy female patient. A single case cannot establish cause and effect, and millions of COVID-19 vaccines have been administered safely at the time of writing. However, this may be a rare association, and clinicians can consider inquiring about recent vaccination history in patients presenting with acute macular neuroretinopathy.

ARE THERE TWO FORMS OF MULTIPLE EVANESCENT WHITE DOT SYNDROME?

PURPOSE: To analyze the nature of multiple evanescent white dot syndrome (MEWDS) and differentiate an idiopathic or primary form of MEWDS from a secondary form that is seen in association with other clinical conditions affecting the posterior segment of the eye. METHODS: Clinical and multimodal imaging findings including color fundus photography, fundus autofluorescence, fluorescein angiography, indocyanine green angiography, spectral-domain optical coherence tomography, and optical coherence tomography angiography of patients with secondary MEWDS are presented. RESULTS: Twenty consecutive patients with secondary MEWDS were evaluated. Fifteen patients were female. Most were young adults aged between 20 to 40 years with myopia (less than -6 diopters). Pathologic conditions associated with the secondary MEWDS reaction were high myopia (greater than -6 diopters) in two eyes, previous vitreoretinal surgery for rhegmatogenous retinal detachment in 2 eyes, and manifestations of multifocal choroiditis in 18 eyes. In all eyes, the MEWDS lesions followed a course of progression and resolution independent from the underlying condition. CONCLUSION: Secondary MEWDS seems to be an epiphenomenon ("EpiMEWDS") that may be seen in association with clinical manifestations disruptive to the choriocapillaris-Bruch membrane-retinal pigment epithelium complex.

Idiopathic multifocal choroiditis and punctate inner choroidopathy: an evaluation in pregnancy.

PURPOSE: To evaluate the clinical course of idiopathic multifocal choroiditis (MFC) and punctate inner choroidopathy (PIC) and the efficacy and safety of treatment options during pregnancy. METHODS: Patients with MFC or PIC and a pregnancy in 2011-2019 from two academic centres were enrolled. For the most recent pregnancy, data on best-corrected visual acuity (BCVA) before and after pregnancy, relapse rate in pregnancy and postpartum period and obstetric, maternal and neonatal outcomes were collected. Treatment regimens consisted of a wait-and-see regime and an immunosuppressive treatment regime with systemic corticosteroids and/or azathioprine, both combined with intravitreal antivascular endothelial growth factor injections when indicated. RESULTS: Sixteen women (26 affected eyes) were included. Median Snellen BCVA was 20/19 before pregnancy and 20/18 after delivery. In seven pregnancies a wait-and-see regime and in nine pregnancies an immunosuppressive treatment regime was carried out. Fourteen intravitreal anti-VEGF injections were given in six pregnancies. The relapse rate during pregnancy was 44% and in the postpartum period 31%. Maternal/obstetrical and fetal complications occurred in 31% and 13% of the pregnancies, respectively. Fifteen healthy children were born and one pregnancy ended in a stillbirth in a patient with a complicated obstetrical history. One patient treated with azathioprine developed intrahepatic cholestasis of pregnancy (ICP). CONCLUSIONS: Among women with MFC and PIC BCVA remained stable during pregnancy despite a relapse rate of 44% in pregnancy. No major maternal, obstetric and fetal complications occurred in pregnant patients treated with systemic corticosteroids, azathioprine or intravitreal anti-VEGF injections, though one patient developed ICP while treated with azathioprine.

Multiple Evanescent White Dot Syndrome: Findings from a Large Northern California Cohort.

PURPOSE: To report the clinical and imaging characteristics of multiple evanescent white dot syndrome (MEWDS) from a large single-center cohort. DESIGN: Single-center, retrospective cohort study. PARTICIPANTS: A total of 111 patients previously diagnosed with MEWDS in the Kaiser Permanente Northern California system from 2012 to 2019. METHODS: Two retina specialists reviewed the medical records and all available retinal imaging, including Humphrey visual field testing, fundus photography (FP), OCT, fluorescein angiogram (FA), and fundus autofluorescence (FAF). Patients were excluded from analysis if confirmatory imaging was unavailable. MAIN OUTCOME MEASURES: Patient characteristics, visual acuity, clinical examination and imaging findings, and final diagnosis. RESULTS: Seventy-three patients (65.8%) were confirmed to have the diagnosis of MEWDS. Fifty-eight (79.5%) were female, with a mean (standard deviation [SD]) age at presentation of 35.2 (14.2) years, and mean refractive error of -1.6 diopters. Initial mean (SD) visual acuity was logarithm of the minimum angle of resolution (logMAR) 0.39 (0.31) and improved to mean (SD) logMAR 0.07 (0.15) at final follow-up. Presenting symptoms included blurred vision (82%), scotomas (56%), photopsias (43%), and floaters (23%). Nine patients (12%) had a previously diagnosed autoimmune condition, and 2 patients (3%) had documentation of a recent vaccination. Antecedent upper respiratory infection was documented in 15 of 66 patients (23%). Noted clinical and imaging features include ellipsoid zone disruption (100%), white fundus lesions (92%), FA hyperfluorescence (92%), foveal granularity (74%), vitreous cell (53%), and optic disc edema (52%). Twenty-nine of the 111 patients (26%) were initially misdiagnosed with MEWDS and subsequently given an alternative diagnosis, including other white dot syndromes, syphilis, primary vitreoretinal lymphoma, myopic degeneration, and central serous chorioretinopathy. CONCLUSIONS: Multiple evanescent white dot syndrome is a rare self-limiting condition of the outer retina. Although a distinct set of clinical exam and imaging findings permit recognition of this disease, misdiagnosis is not uncommon.

A Multiple Evanescent White Dot Syndrome-like Reaction to Concurrent Retinal Insults.

PURPOSE: To describe a clinical picture resembling classic multiple evanescent white dot syndrome (MEWDS) potentially triggered by previous or concurrent, apparently unrelated, ocular events and to provide a literature review of similar presentations. DESIGN: Retrospective chart series and literature review. PARTICIPANTS: Consecutive patients diagnosed with MEWDS at the Feinberg School of Medicine, Northwestern University, Chicago, Illinois, and the IRCCS San Raffaele Scientific Institute, Milan, Italy, between July 2019 and June 2020. METHODS: Charts of patients were reviewed. Ophthalmic history, best-corrected visual acuity, spectral-domain OCT results, OCT angiography results, fundus autofluorescence results, ultra-widefield fluorescein angiography results, and indocyanine green angiography results were collected. A PubMed-based search was carried out for similar presentations using the terms MEWDS and white spot syndromes. MAIN OUTCOME MEASURES: An ocular history positive for previous or concurrent ocular events in patients with MEWDS was sought in our cohort and the existing literature. RESULTS: Five eyes of 4 patients (2 females; age range, 16-81 years) were included. The first eye had a history of bilateral Best vitelliform dystrophy and unilateral choroidal neovascularization. The second eye had angioid streaks complicated by choroidal neovascularization and underwent prior thermal laser photocoagulation. The third eye had a history of high myopia and a scleral buckle procedure for retinal detachment. The fourth patient had bilateral idiopathic retinochoroiditis. We identified 16 case reports from 5 previous publications that support a MEWDS-like reaction to previous ocular insults. CONCLUSIONS: We suggest a MEWDS-like reaction may be elicited by ocular events in a subset of susceptible patients. We hypothesize that damage to the outer retina may play a role in triggering the local inflammatory response.

Influenza-Induced Acute Macular Neuroretinopathy With Cerebral Involvement in a Ten-Year-Old Boy.

A 10-year-old male presented with 1-week duration of painless bilateral central vision loss after having been diagnosed with influenza A. Optical coherence tomography revealed superficial retinal nerve fiber layer infarcts, hyperreflectivity of the inner nuclear layer consistent with paracentral acute middle maculopathy (PAMM), and outer nuclear layer hyperreflectivity and disruption of the ellipsoid zone suggesting acute macular neuroretinopathy (AMN). Brain MRI revealed enhancement of the right basal ganglia consistent with focal encephalitis. He was diagnosed with presumed influenza-induced leukocytoclastic vasculitis (LCV) and treated with intravenous steroids. Influenza-induced LCV is a rare phenomenon and can present with AMN, PAMM, and encephalitis. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:293-297.].

Clinical and Morphological Characteristics of Anti-Programmed Death Ligand 1-Associated Retinopathy: Expanding the Spectrum of Acute Macular Neuroretinopathy.

PURPOSE: Immunotherapy with atezolizumab, a checkpoint inhibitor targeting the programmed cell death 1 (PD-1) axis, has shown promising results for the treatment of certain metastatic cancers. Atezolizumab-associated acute macular neuroretinopathy (AMN) with retinal venulitis is a newly reported immune-related adverse event (irAE) that further expands the range of adverse effects associated with checkpoint inhibitor therapy. We describe the clinical course and imaging findings of a similar AMN-like retinopathy after treatment with atezolizumab. DESIGN: Retrospective case series. PARTICIPANTS: Three patients treated with atezolizumab for metastatic breast cancer (n = 1) and non-small-cell lung cancer (n = 2). METHODS: Inclusion criterion was a clinical diagnosis of AMN-like retinopathy with or without retinal vasculitis after atezolizumab administration. MAIN OUTCOME MEASURES: Clinical course and multimodal retinal imaging including color photographs, spectral-domain OCT, near-infrared reflectance, and fluorescein angiography were investigated. RESULTS: Three patients (1 woman and 2 men; mean age, 51 years) experienced the acute onset of reduced visual acuity and paracentral scotomas 2 weeks after their first infusion of atezolizumab. Visual symptoms corresponded to focal areas of pericentral photoreceptor disruption in all cases. In 1 patient imaged with fluorescein angiography, focal segments of retinal venulitis were detected. After treatment cessation, incomplete visual recovery was related to persistent photoreceptor damage. All patients died of their cancer within 6 months after the onset of retinopathy. CONCLUSIONS: To our knowledge, there are 3 previously published cases of atezolizumab-associated AMN with retinal vasculitis. This series of 3 similar cases strengthens the association of programmed death ligand 1 (PD-L1) inhibition with this rare form of retinopathy that was termed "anti-PD-L1-associated retinopathy." This irAE seems to be a consistent occurrence in the second week postadministration with lasting structural and functional deficits seen after treatment cessation. Pathophysiologic mechanisms may include loss of tolerance in an immune-privileged organ and subsequent development of T-cell-driven inflammation. In this emerging field, expanding the spectrum and pathogenesis of irAEs is essential to define strategies for prevention, early detection, and appropriate management.

Masqueraders of multiple evanescent white dot syndrome (MEWDS).

PURPOSE: To describe disorders that can masquerade as multiple evanescent white dot syndrome (MEWDS). DESIGN: Retrospective, multicenter case series. PARTICIPANTS: Patients who presented with clinical findings compatible with a diagnosis of MEWDS but were ultimately diagnosed with an alternative inflammatory, infectious, or neoplastic disorder. METHODS: Clinical records and multimodal imaging findings including fundus photography, fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA), optical coherence tomography (OCT), and OCT angiography (OCTA) were analyzed. MAIN OUTCOME MEASURES: Inclusion criteria to be defined as a masquerade syndrome for MEWDS included the presence of disseminated grayish-white outer retinal spots that were hyperautofluorescent on FAF and associated with ellipsoid zone (EZ) disruption on OCT. RESULTS: Twenty-two eyes of 13 patients were identified. All patients presented with the classic findings of MEWDS listed above. A MEWDS-like presentation was bilateral in nine of 13 patients (69%). Final diagnosis was determined on the basis of additional investigations including serologies and biopsy. These diagnoses included syphilis (three patients), lymphoma (three patients), idiopathic multifocal choroiditis (two patients), idiopathic retinal phlebitis (one patient), idiopathic acute zonal occult outer retinopathy (one patient), sarcoidosis (one patient), tuberculosis (one patient), and cancer-associated retinopathy (one patient). The outer retinal lesions and imaging findings resolved with treatment for the associated systemic disorders. CONCLUSIONS: Widespread grayish-white outer retinal spots associated with hyperautofluorescence on FAF and disruption of the EZ on OCT are not pathognomonic for MEWDS. A high index of suspicion must be maintained for masqueraders of MEWDS, which can include serious inflammatory, infectious, and neoplastic disorders.

Long-Term Outcome of Punctate Inner Choroidopathy or Multifocal Choroiditis with Active Choroidal Neovascularization Managed with Intravitreal Bevacizumab.

Purpose: To evaluate the long-term outcome of active choroidal neovascularization (CNV) in punctate inner choroidopathy (PIC) or multifocal choroiditis (MFC) after intravitreal bevacizumab treatment.Methods: Retrospective study of consecutive patients of PIC/MFC complicated with active CNV. Outcome measures included best-corrected visual acuity (BCVA), total number of intravitreal injections of bevacizumab and recurrence of CNV. Correlation analysis was performed to find the correlation of various clinical factors and final BCVA.Results: There were 23 eyes in 22 patients with a mean age of 33.22 years included in this study. The mean duration of follow-up was 6.48 years. Improvement of BCVA was noted through the first 3 years and at the final follow-up. BCVA at 1, 6, 12 months and recurrence of CNV were correlated with final BCVA.Conclusion: Most patients of PIC/MFC complicated with CNV managed with intravitreal bevacizumab had improved BCVA over 4 years.

Intraretinal Cystoid Spaces in Regression of Punctate Inner Choroidopathy Lesions.

PURPOSE: To describe and evaluate the intraretinal cystoid spaces (ICSs) in the eyes of punctate inner choroidopathy (PIC) patients. METHODS: In this observational study, patients diagnosed with PIC were included and reviewed between December 2016 and November 2018. All patients underwent multimodal retinal imaging examinations. RESULTS: Forty-one eyes of 26 patients diagnosed with PIC were included. ICSs were found in 13 eyes (31.7%) of 11 subjects. Statistical analysis revealed that except for spherical equivalent (P= .020), there were no significant difference between patients with ICSs and those with no ICSs at baseline. There were two types of ICSs according to the multimodal imaging findings. These two types of ICSs appeared with regressive PIC lesion and were stable during the follow-up period. CONCLUSION: ICSs are commonly observed in PIC and they may be a sign of the restoration stage of the disease rather than a need for further clinical intervention.

Choroidal neovascularisation secondary to serpiginous choroiditis: The value of OCT-angiography in diagnosis and response to therapy with aflibercept. / Neovascularización coroidea secundaria a coroiditis serpiginosa: valor de la OCT-angiografía en el diagnóstico y en la respuesta al tratamiento con aflibercept.

A case is presented of a 61 year-old man with a personal history of serpiginous choroiditis (SC), who presented with metamorphopsia and decreased visual acuity (VA) in his right eye (RE). In the examination, using Swept Source OCT-angiography (SS OCT-A), peripapillary haemorrhage secondary to a choroidal neovascularisation (CNV) was observed. The patient was treated with intravitreal aflibercept, having a favourable outcome on his symptomatology and in the SS OCT-A findings. CNV is a complication that can appear in up to 25% of cases after SC. An accurate diagnosis using SS OCT-A, with early treatment with aflibercept, led us to obtain optimal clinical and anatomical results.

Acute Posterior Multifocal Placoid Pigment Epitheliopathy Complicated by Fatal Cerebral Vasculitis.

A 21-year-old man experienced unilateral vision loss associated with multiple atrophic chorioretinal lesions. He was treated for a presumptive diagnosis of acute retinal necrosis, but his vision did not improve with antiviral therapy. Over the course of several weeks, his symptoms progressed to involve both eyes. The fellow eye showed characteristic yellow-white placoid lesions, prompting treatment with oral corticosteroids for acute posterior multifocal placoid pigment epitheliopathy (APMPPE). Despite high-dose therapy with prednisone 80 mg daily, the patient developed the acute onset of mental status changes within the next several days. Neuroimaging revealed multifocal large-vessel strokes associated with cerebral edema; these infarcts led to herniation and death. Postmortem histopathologic examination confirmed granulomatous inflammation in both ocular and cerebral vasculatures. Together with findings from multimodal imaging obtained throughout this patient's clinical course, our findings support the notion that granulomatous choroiditis is the mechanism of the ocular lesions seen in APMPPE. This granulomatous inflammation can also affect cerebral vessels, leading to strokes.

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.

Acute Zonal Occult Outer Retinopathy (AZOOR) in a patient with Hashimoto's thyroiditis / Retinopatia aguda zonal oculta externa em uma paciente com tireoidite de Hashimoto

ABSTRACT Acute zonal occult outer retinopathy (AZOOR) is a rare disease characterized by an acute damage of one or more external retinal zones leading to the visual field or the visual acuity impairment associated with small or no changes in the fundus examination.The main clinical symptoms are scotomas and the sudden onset of photopsias. Abnormal findings on electroretinography and visual field defects are critical for the diagnosis. Central vision is usually preserved and the stabilization occurs in six months in most cases. The objective of this article is to describe a 24-month follow-up of a patient with AZOOR and correlate the findings with the typical features of this disease.

RESUMO A retinopatia aguda zonal oculta externa (AZOOR) é uma doença rara, caracterizada por um dano agudo de uma ou mais zonas da retina externa que levam ao comprometimento do campo visual ou da acuidade visual, estando associada a pequenas ou nenhumas alterações no exame fundoscópico. Os principais sintomas clínicos são escotomas e o aparecimento súbito de fotopsias. Resultados anormais de defeitos de campo visual e eletrorretinografia são críticos para o diagnóstico. A visão central é geralmente preservada e a estabilização do quadro ocorre em seis meses na maioria dos casos. O objetivo deste artigo é descrever o "follow-up" de 24 meses de uma paciente com AZOOR e correlacionar os achados com as características típicas desta doença.