Multiple Evanescent White Dot Syndrome Research Articles

Clinical features and possible pathogenesis of multiple evanescent white dot syndrome with different retinal diseases and events: a narrative review

Multiple evanescent white dot syndrome (MEWDS) is a rare fundus disease, characterized by acute vision loss and visual field defects. Many previous studies have explained the possible pathogenesis and clinical features of primary MEWDS. However, as the number of reported cases increases, secondary MEWDS occurs in other related retinal diseases and injuries, exhibiting some special characteristics. The associated retinal diseases include multifocal choroiditis/punctate inner choroidopathy (MFC/PIC), acute zonal occult outer retinopathy, best vitelliform macular dystrophy, pseudoxanthoma elasticum, and ocular toxoplasmosis. The related retinal injury is laser photocoagulation, surgery, and trauma. Although primary MEWDS often have a self-limiting course, secondary MEWDS may require treatment in some cases, according to the severity of concomitant diseases and complications. Notably, MEWDS secondary to MFC/PIC that is prone to forming choroidal neovascularization and focal choroidal excavation, needs positive treatment with corticosteroids. The possible underlying pathogenesis of secondary MEWDS is the exposure of choroidal antigen after the disruption of Bruch’s membrane. The MEWDS-related features in secondary MEWDS are still evanescent under most circumstances. Its prognosis and treatment depend on the severity of complications. Current studies propose that the etiology is associated with immune factors, including viral infection, inflammation in choroid and Bruch’s membrane, and antigen exposure caused by retinal and/or choroidal insults. More pathogenic studies should be conducted in the future. Accurate diagnosis for secondary MEWDS could benefit patients in aspects of management and prognosis.

A COVID-19 perspective of multiple evanescent white dot syndrome (MEWDS).

Multiple evanescent white dot syndrome (MEWDS) is a rare form of posterior uveitis characterized by involvement in the posterior pole and mid-periphery. A viral etiology that provokes an immune-mediated response has been hypothesized to be the inciting factor of the pathology. Recently, there has been an increase in the literature regarding new-onset uveitis and reactivation of previously diagnosed cases of uveitis following COVID-19 vaccinations. The COVID-19 vaccination has been speculated to trigger an immunomodulatory shift in recipients, resulting in an autoimmune event. MEWDS following COVID-19 vaccination was reported in 31 patients. It was most commonly observed following the first dose, affecting 15 patients, and least commonly after the booster dose, in only one patient. MEWDS-like disease following anti-SARS-CoV-2 vaccinations was reported the most in 16 cases after the Pfizer-BioNTech vaccination (BNT162b2 mRNA). Most of these cases had Primary MEWDS without any previous history of a similar event in the past.

Fundus autofluorescence in uveitis: Meeting an old friend

Fundus autofluorescence (FAF) represents a relatively new imaging modality that has been investigated in a variety of uveitic disease processes. FAF relies on the presence of innate fluorophores within ocular tissues, which can be induced to emit light when stimulated with specific frequencies of light.Autofluorescence in the RPE depends on the outer segment renewal and can be affected by the RPE's ability to clear lipofuscin. Hence, an increased autofluorescence is seen with RPE dysfunction and a decreased autofluorescence is seen with loss of photorecptors or the RPE.Abnormal autofluorescence patterns have been described in a number of infectious and non‐infectious uveitides. Most reports of abnormal FAF in patients with uveitis belong to the spectrum of white‐dot‐syndromes. In the present course we will review the main autofluorescence abnormalities in disease like Serpiginous and Serpiginous‐like choroiditis, multifocal choroiditis, punctate inner choroiditis, multiple evanescent white dot syndrome and AZOOR. The recognition of theses abnormalities (in combination with other imaging techniques like optical coherence tomography and optical coherence tomography‐angiography) is extremely helpful for understanding their pathogenesis and monitoring the inflammatory activity.

Multiple evanescent white dot syndrome following COVID-19 infection: A case report

Multiple evanescent white dot syndrome (MEWDS) is typically a unilateral and transient condition without an exact known underlying cause. Its pathogenesis may involve an immune response to viral antigens that have gained access to the retinal receptor cells. It has been hypothesized that the COVID-19 virus can activate some autoimmune responses. Here, we report a case of MEWDS in a 16-year-old girl with a history of COVID-19 infection two weeks before the visual symptoms. The symptoms and signs improved without any treatment after a three-month follow-up period. In our case, MEWDS occurred shortly after COVID-19 infection and resolved without treatment in three months. Considering ophthalmologic and immunologic associations of COVID-19 can help ophthalmologists in the diagnosis and treatment of similar disorders in the COVID-19 era.

Differential diagnosis of myopic choroidal neovascularization (mCNV): insights from multimodal imaging and treatment implications.

The aim of this article is to conduct a comprehensive systematic review about the current understandings and differential diagnosis of myopic choroidal neovascularization (mCNV) and other several similar diseases, describing their multimodal imaging analysis, prognostic implications, and current types of management. This systematic review was performed based on a search on the PubMed database of relevant papers regarding mCNV and other entities discussed in the paper, according to our current knowledge. Through the integration of a multimodal imaging approach, especially optical coherence tomography (OCT), along with accurate demographic and clinical assessment, it becomes possible to effectively differentiate mCNV from similar yet heterogeneous entities. These conditions include macular hemorrhage due to new lacquer crack (LC) formation, inflammatory diseases such as punctate inner choroidopathy (PIC)/multifocal choroidits (MFC) and epiphenomenon multiple evanescent white dot syndrome (Epi-MEWDS), neovascular age-related macular degeneration (nAMD), idiopathic CNV (ICNV), dome-shaped macula (DSM) with subretinal fluid, retinal pigment epithelium (RPE) humps, angioid streaks (AS), choroidal rupture (CR), and choroidal osteoma (CO). Each one of these entities will be described and discussed in this article. Myopic choroidal neovascularization is a common retinal condition, especially among young individuals. Accurate diagnosis and differentiation from similar conditions are crucial for effective treatment. Multimodal imaging, particularly OCT, plays a crucial role in precise assessment. Future research should focus on defining biomarkers and distinguishing features to facilitate prompt treatment.

Ocular Inflammation Post-Vaccination.

The association between vaccines and ocular disorders has attracted significant attention in scientific research. Numerous mainstream vaccines are associated with a range of uveitis types, including anterior, intermediate, and posterior uveitis. Additionally, they are associated with distinct ocular diseases such as multifocal choroiditis, Vogt-Koyanagi-Harada (VKH) disease, acute posterior multifocal placoid pigment epitheliopathy (APMPPE), and multiple evanescent white dot syndrome (MEWDS). These ocular conditions are often transient, with a vast majority of patients experiencing improvement after steroid intervention. To date, numerous cases of vaccine-induced uveitis have been reported. This study analyzed the correlation between antiviral vaccines, including the hepatitis B virus (HBV), human papillomavirus (HPV), measles-mumps-rubella (MMR), varicella zoster virus (VZV), and influenza vaccines, and different manifestations of uveitis. This is the first comprehensive study to offer a detailed analysis of uveitis types induced by antiviral vaccines. Through an extensive database search, we found a particularly strong link between influenza vaccines, followed by VZV and HPV vaccines. While anterior uveitis is common, conditions such as APMPPE, MEWDS, and VKH are particularly notable and merit careful consideration in clinical practice. Corticosteroid treatment was effective; however, half of the observed patients did not achieve full recovery, indicating potentially prolonged effects of the vaccine.

Multiple evanescent white dot syndrome relapse following BNT162b2 mRNA COVID-19 vaccination.

Multiple evanescent white dot syndrome relapse following BNT162b2 mRNA COVID-19 vaccination.

Presumed Müller Cell Activation in Multiple Evanescent White Dot Syndrome.

The purpose of this study was to investigate the foveal changes occurring in multiple evanescent white dot syndrome (MEWDS) using multimodal imaging techniques with a specific focus on hyper-reflective dots (HRDs). This was a retro-prospective observational study including 35 eyes with active MEWDS. Structural and en face optical coherence tomography (OCT) was performed, with follow-up visits at 2 weeks, 6 weeks, and 2 months from baseline. HRD percentage area (HRD % area) was calculated in a 600 µm fovea centered circle on en face OCT, after background subtraction and image binarization. HRD % area was compared with 23 fellow control eyes. Longitudinal changes in the HRD % areas were assessed using repeated-measure statistics. HRDs were observed as scattered hyper-reflective spots on the vitreoretinal interface on en face OCT images, colocalizing with HRDs or vertical hyper-reflective lines on structural OCT images. The baseline evaluation showed a significantly higher HRD % area in MEWDS eyes compared to fellow eyes (0.10 ± 0.03 vs. 0.08 ± 0.04, P = 0.01). The HRD% area correlated positively with LogMAR visual acuity and inversely with the duration of symptoms. Longitudinal analysis revealed a significant reduction in the HRD % area over time. There was no significant interaction between the rate of HRD disappearance and clinical or demographic factors at baseline. As HRD potentially represents the end-feet projections of activated Müller cells on the retinal surface, this study supports the involvement of Müller cells in the pathogenesis of the disease. The findings highlight the potential of en face OCT imaging for monitoring the progression of MEWDS.

Acute Idiopathic Blind Spot Enlargement Syndrome (AIBSES) in the Era of OCT - a Review.

There are only about 100 case reports on the Acute Idiopathic Blind Spot Enlargement Syndrome (AIBSES). This is characterised by the eponymous visual field loss in the blind spot area, acute onset photopsia, and funduscopically little or no change in the optic disc area, with conspicuous outer retinal bands on optical coherence tomography (OCT). Typical is the unilateral occurrence. Predominantly young women are affected. While previous reviews of AIBSES either predate the introduction of OCT or focus on differentiation from potentially related outer retinal conditions (e.g., multiple evanescent white dot syndrome and acute zonal occult outer retinopathy), the present review will concentrate on the current perspective and treatment strategies that have been developed and will aim to help increase awareness. Since the first description of AIBSES in the late 1980s, the introduction of OCT has simplified the diagnosis and characterisation of AIBSES as a disease of the outer retina. Nevertheless, misdiagnosis remains common in the spectrum of optic neuritis, as AIBSES may be ignored in differential diagnosis.

Unilateral Multiple Evanescent White Dot Syndrome-Like Reaction Following the CyberKnife Stereotactic Radiotherapy for Choroidal Malignant Melanoma.

A 58-year-old otherwise healthy man received a diagnosis of choroidal malignant melanoma (CMM) in June 2021 and underwent a single session of (21 Gy) CyberKnife stereotactic radiotherapy (SRT). Eleven months later, we noticed 3+ anterior chamber cells with occasional vitreous cells in the left eye. Though the tumor looked regressed, there were mild optic disc leakage, early hypofluorescent and late hyperfluorescent punctate lesions scattered 360 degrees, and late staining of the mass on fluorescein angiogram. The findings were compatible with a unilateral multiple evanescent white dot syndrome (MEWDS)-like reaction that was most likely related to CyberKnife SRT-induced tumor necrosis, and a dexamethasone implant was administered intravitreally into the left eye together with topical steroids. A second intravitreal injection of dexamethasone was given three months later due to remittance of the angiographic features. As there are only a few reports on CyberKnife SRT for the treatment of CMM, we wanted to share our interesting observation of a post-treatment MEWDS-like reaction likely related to tumor necrosis syndrome with the ophthalmic community.

Blue-Light Fundus Autofluorescence (BAF), an Essential Modality for the Evaluation of Inflammatory Diseases of the Photoreceptors: An Imaging Narrative.

Our purpose is to describe blue-light fundus autofluorescence (BAF) features of inflammatory diseases of the outer retina characterised by photoreceptor damage. BAF from patients diagnosed with secondary and primary inflammatory photoreceptor damage were retrospectively analyzed and compared to other imaging modalities including fluorescein angiography (FA), indocyanine green angiography (ICGA), and spectral domain optical coherence tomography (SD-OCT). Multiple evanescent white dot syndrome (MEWDS), idiopathic multifocal choroiditis (MFC), acute posterior multifocal placoid pigment epitheliopathy (APMPPE), serpiginous choroiditis (SC), and acute syphilitic posterior placoid chorioretinitis (ASPPC), all cases corresponding to secondary photoreceptor diseases caused by inflammatory choriocapillaris nonperfusion, were included and compared to primary photoreceptor disease entities, including acute zonal occult outer retinopathy (AZOOR) and cancer-associated retinopathy (CAR). Both groups showed increased BAFs of variable intensity. In severe cases of APMPPE and ASPPC, BAF also showed hypoautofluorescent areas. In group 1 (secondary diseases) BAF hyperautofluorescent areas were associated with colocalized ICGA hypofluorescent areas, indicating choriocapillaris nonperfusion; whereas in group 2 (primary diseases), no ICGA signs were detected. The associated colocalized areas of hypofluorescence on ICGA in the first group, which were absent in the second group, were crucial to allow the differentiation between primary (photoreceptoritis) and secondary (choriocapillaritis) photoreceptor diseases. BAF patterns in inflammatory diseases of the outer retina can give relevant information on the photoreceptor and RPE involvement, with ICGA being crucial to detect concurring choriocapillaris damage and differentiating the two pathologies.

PRIMARY MULTIPLE EVANESCENT WHITE DOT SYNDROME AND MULTIPLE EVANESCENT WHITE DOT SYNDROME SECONDARY TO MULTIFOCAL CHOROIDITIS/PUNCTATE INNER CHOROIDOPATHY: A Comparative Study.

To present and compare the clinical features and multimodal imaging (MMI) findings of the primary form of multiple evanescent white dot syndrome (MEWDS) and MEWDS secondary to multifocal choroiditis/punctate inner choroidopathy (MFC/PIC). A prospective case series. Thirty eyes of 30 MEWDS patients were included and divided into the primary MEWDS group and MEWDS secondary to MFC/PIC group. Demographic, epidemiologic, and clinical characteristics and MEWDS-related MMI findings of the two groups were compared. Seventeen eyes from 17 patients with primary MEWDS and 13 eyes from 13 patients with MEWDS secondary to MFC/PIC were evaluated. Patients with MEWDS secondary to MFC/PIC tended to have a higher degree of myopia than those with primary MEWDS. No other significant differences in demographic, epidemiologic, and clinical characteristics and MMI findings were found between the two groups. "MEWDS-like reaction" hypothesis seems to be correct for MEWDS secondary to MFC/PIC, and the authors highlight the importance of MMI examinations in MEWDS. Further research is needed to confirm whether the hypothesis is applicable to other forms of secondary MEWDS.

OPTICAL COHERENCE TOMOGRAPHY IMAGING OF RETINAL MACROPHAGE-LIKE CELLS IN PATIENTS WITH MULTIPLE EVANESCENT WHITE DOT SYNDROME.

To analyze the changes of macrophage-like cells (MLCs) on the inner retinal surface associated with multiple evanescent white dot syndrome using optical coherence tomography. Four eyes of four patients with multiple evanescent white dot syndrome were examined and followed with multimodal imaging, including optical coherence tomography angiography, to visualize MLC on en face structural projections. On en face structural optical coherence tomography angiography projection at presentation, all multiple evanescent white dot syndrome eyes showed a decrease of the nearest neighbor distance of MLC within the region of interest and an increase of MLC density in the perifovea compared with the fellow unaffected eye. Three eyes demonstrated protrusion of MLC into the vitreous. At 1 to 3 months of follow-up, nearest neighbor distance increased, and MLC density decreased to the level of the fellow unaffected eye along with regression of multiple evanescent white dot syndrome symptoms. Multiple evanescent white dot syndrome may be associated with increase of MLC population on the inner retinal surface, which can be tracked with clinical optical coherence tomography imaging.

High Resolution Spectral Domain Optical Coherence Tomography of Multiple Evanescent White Dot Syndrome.

To evaluate a patient with multiple evanescent white dot syndrome (MEWDS) with multimodal imaging including high resolution spectral domain optical coherence tomography (SD-OCT). The patient was evaluated with wide-field color and autofluorescence imaging, microperimetry, and near infrared imaging. SD-OCT was performed using an instrument capable of 3 µm axial resolution, the High-Resolution Heidelberg Spectralis. 28 year-old woman developed photopsias and a scotoma in the field of vision of her left eye. She had multiple whitish spots with granularity in her fovea consistent with the diagnosis of MEWDS. She had supportive fluorescein angiographic and autofluorescence findings. Because of the high resolution and the good layer contrast, it was possible to create en face slab images of the external limiting membrane (ELM), the ellipsoid zone (EZ), interdigitation zone (IZ), and the retinal pigment epithelium (RPE). The ELM showed no abnormalities. There were multiple regions of decreased reflectance in the EZ slab, but even more prominent changes in the IZ. The RPE showed nearly no variation in layer reflectivity. With resolution of symptoms, the color and autofluorescence images returned to normal, the defects in the EZ almost completely resolved and the IZ continued to show abnormalities. Although past studies concluded the EZ was the main region of involvement in MEWDS, high resolution SD-OCT suggests the IZ was more prominently affected in this case.

Topographic patterns of retinal lesions in multiple evanescent white dot syndrome.

To demonstrate different topographic distributions of multiple-evanescent white dot syndrome (MEWDS) and secondaryMEWDS disease and to describe possible associations. Clinical evaluation and multimodal retinal imaging in 27 subjects with MEWDS (29 discrete episodes of MEWDS). Ophthalmic assessment included best-corrected visual acuity testing and multimodal retinal imaging with OCT, blue-light autofluorescence, fluorescein and indocyanine green angiography, fundus photography, and widefield pseudocolor and autofluorescence fundus imaging. The topographic distribution of MEWDS lesions was centered on or around the optic disc (n = 17, 59%), centered on the macula (n = 7, 24%), sectoral (n = 2, 7%), or was indeterminate (n = 3, 10%). The MEWDS episodes either occurred in the absence ('primary MEWDS'; n = 14, 48%) or presence of concurrent chorioretinal pathology ('secondary MEWDS'; n = 15, 52%). In patients with the latter, MEWDS lesions were often centered around a coexisting chorioretinal lesion. The majority of patients in both groups experienced resolution of their symptoms and retinal changes on multimodal imaging by 3months. Distinct distributions of MEWDS lesions were identified. MEWDS may occur in tandem with other chorioretinal pathology, which may impact the topography of MEWDS lesions.

Active Ocular Toxoplasmosis Associated with Simultaneous Multiple Evanescent White Dot Syndrome

ABSTRACT Purpose We describe the association between active ocular toxoplasmosis and Multiple Evanescent White Dot Syndrome (MEWDS). Patients and methods Retrospective observational case report of a patient who presented with concomitant ocular toxoplasmosis and MEWDS (Erasmus University Hospital, Brussels, Belgium). Clinical record and multimodal imaging including fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA), and spectral domain optical coherence tomography (SD-OCT) were analyzed. Results Multimodal imaging of a 25-year-old-woman that presented with concomitant active ocular toxoplasmosis and MEWDS is described. Both clinical entities regressed completely after 8 weeks under steroidal anti-inflammatory drugs and antibiotics. Conclusion Active ocular toxoplasmosis can be associated with simultaneous multiple evanescent white dot syndrome. Further reports are needed to precise and characterize this clinical relationship and its management. Abbreviations: MEWDS: Multiple Evanescent White Dot Syndrome; Fundus Autofluorescence FAF; BCVA: Best-corrected Visual Acuity; FA: Fluorescein Angiography; ICGA: Indocyanine Green Angiography; SD-OCT: Spectral Domain Optical Coherence Tomography; IR: Infrared.

Multiple evanescent white dot syndrome in highly myopic eye in which fundus autofluorescence was diagnostically useful: A case report.

Multiple evanescent white dot syndrome (MEWDS) is characterized by multiple white dots in the posterior pole of the eye, but its diagnosis can be difficult in cases when the white dots are not visible. We report an atypical case of MEWDS that developed in pathologic myopia. A 42-year-old woman presented with floater and reduced and distorted vision in the right eye that she noted 3 days earlier. The right eye was highly myopic at -17.0 diopters, and her decimal best-corrected visual acuity was reduced to 0.2 (20/100). Fundus examinations revealed no abnormalities other than myopic fundus changes and previous laser photocoagulation spots. However, fundus autofluorescence (FAF) showed multiple hyperfluorescent dots, and optical coherence tomography showed a high reflective lesion on the retinal pigmental epithelium at the fovea. Adjustments of the brightness and contrast of the conventional fundus images revealed white dots in the same location as the hyperfluorescent spots seen in the FAF images. We diagnosed her with MEWDS. We treated her with systemic administration of 20 mg prednisolone and the dose of prednisolone was reduced by 5 mg every 4 weeks. The optical coherence tomography and FAF findings gradually normalized, and 5 months later, her decimal visual acuity was restored to 1.0 (20/20). It was suggested that white dots typical to MEWDS may not be evident in pathologic myopia, and FAF images and the brightness and contrast adjustment of fundus images were useful in the diagnosis of atypical MEWDS.

Secondary multiple evanescent white-dot syndrome and multimodal imaging

Secondary multiple evanescent white-dot syndrome and multimodal imaging

Advances and challenges of using multimodal imaging for the diagnosis of Multiple Evanescent White Dot Syndrome

No abstract available

COMPARISON OF PRIMARY AND SECONDARY FORMS OF MULTIPLE EVANESCENT WHITE DOT SYNDROME.

The aim of this study was to compare primary versus secondary forms of multiple evanescent white dot syndrome (MEWDS) at T0 (baseline) and T1 (1-4 months after the onset of symptoms). A total of 101 eyes in 100 patients were included in a multicentric retrospective study. Secondary MEWDS was defined as MEWDS associated with underlying chorioretinal inflammatory pathologies, mainly multifocal choroiditis and punctuate inner choroidopathy. Patients with secondary MEWDS were older (P = 0.011). The proportion of women (P = 0.8), spherical equivalent (P = 0.3), and best-corrected visual acuity at T0 (P = 0.2) were not significantly different between the two groups. The area of MEWDS lesions on late-phase indocyanine green angiography was significantly smaller in secondary MEWDS (P = 0.001) and less symmetrical with respect to both horizontal (P = 0.003) and vertical (P = 0.004) axis. At T0, neither the clinical (P = 0.5) nor the multimodal imaging (P = 0.2) inflammation scores were significantly different between the groups. At T1, the multimodal imaging inflammation score was higher in secondary MEWDS (P = 0.021). In secondary MEWDS, outer retinal lesions are less extensive and located close to preexisting chorioretinal lesions. Mild signs of intraocular inflammation on multimodal imaging are more frequent in secondary MEWDS during recovery. These findings suggest that chorioretinal inflammation may trigger secondary MEWDS.