White Dot Syndrome Research Articles

Is it Time to Adopt a New Nomenclature and Classification for White Dot Syndromes Using Multimodal Imaging Techniques? Report 1 from Multimodal Imaging in Uveitis (MUV) Task Force

Is it Time to Adopt a New Nomenclature and Classification for White Dot Syndromes Using Multimodal Imaging Techniques? Report 1 from Multimodal Imaging in Uveitis (MUV) Task Force

Clinical and Genetic characteristics of patients with Peripheral Retinal Fleck in Koreans.

To describe the clinical and genetic features of Korean patients with peripheral retinal flecks unrelated to aging. A retrospective analysis was conducted on the clinical characteristics of patients with symmetric peripheral retinal flecks. Age-related deposits such as reticular pseudodrusen were excluded, as well as secondary deposits related to intraocular inflammation, tumor, and drug toxicity. Multimodal imaging, electrophysiological examinations, and genetic testing were analyzed. A total of 10 patients (2 males and 8 females) with bilateral peripheral flecks were enrolled in this study. A mean age at diagnosis was 30.5±19.6 years (4-59 years). Within the 10 patients, 6 were genetically confirmed with monogenic retinal disorders. Biallelic pathogenic variants in RDH5 were found in 5 patients, and 1 patient was diagnosed with retinopathy related to Alport syndrome due to a pathogenic variant in COL4A5. Although not genetically confirmed, one case associated with nanophthalmos and another case showing chorioretinal mottling in a carrier of ocular albinism have been identified. In one patient, genetic testing also revealed unknown causes. The mean logMAR initial visual acuity was 0.12±0.18 and 0.07±0.18 in right and left eyes, respectively. Night blindness was reported by 4 patients (40%), with 3 showing decreased or delayed rod response in electroretinogram, particularly those with RDH5 mutations. Differences in the deposit layers and the patterns of flecks were observed on multimodal imaging. In the study population, we observed various causes and clinical differences in the retinal fleck patterns among Koreans, including RDH5-related fundus albipunctatus and Alport syndrome. Despite reports of night blindness symptoms in some cases, all patients demonstrated satisfactory corrected visual acuity.

Secondary Multiple Evanescent White Dot Syndrome in a patient with North Carolina Macular Dystrophy.

To describe a novel case of secondary multiple evanescent white dot syndrome in a patient with North Carolina Macular Dystrophy (NCMD). The patient was evaluated with ultra-widefield color and autofluorescence imaging, fluorescein angiography, and spectral-domain optical coherence tomography. A 43-year-old man with longstanding blurred vision in both eyes acutely developed glare in the right eye after an upper respiratory illness. He had chorioretinal atrophy in both eyes consistent with North Carolina Macular Dystrophy, which was later confirmed by genetic testing. He alsyo had white spots in the macula of the right eye consistent with secondary MEWDS. The diagnosis of secondary MEWDS was confirmed with fundus autofluorescence and fluorescein angiography, as well as a negative infectious workup. His symptoms and the retinal lesions had resolved by 11 weeks and after a short course of oral corticosteroids. Prior studies have suggested a link between disruption of the RPE/Bruch's membrane complex and secondary MEWDS. This report describes the first case of secondary MEWDS after NCMD and provides further support for this association.

Acute Idiopathic Blind Spot Enlargement and Multiple Evanescent White Dot Syndrome

Acute idiopathic blind spot enlargement syndrome (AIBSES) and Multiple evanescent white dot syndrome (MEWDS) are diseases that fall within the same spectrum. They share common characteristics such as affecting young and middle-aged individuals, being mostly unilateral, causing damage to the outer layers of the retina, and leading to blind spot enlargement in the visual field. In AIBSES, lesions are more frequently located in areas adjacent to the optic disc, whereas in MEWDS, they are observed around the central and peripheral vascular arcades. Unlike MEWDS, in AIBSES, visual field improvement is not expected over time. Multimodal imaging methods, including optical coherence tomography, fundus autofluorescence, fundus fluorescein angiography, and indocyanine green angiography, play an important role in diagnosis and follow-up. In both conditions, the optic nerve can appear normal, but it can also be seen with slightly blurred boundaries and edema, and it should be considered in the differential diagnosis of optic neuropathy.

An update of multimodal imaging in white dot syndrome

Abstract: The white dot syndromes are a group of phenotypically similar disorders characterized by multiple lesions at the level of the outer retina, retinal pigment epithelium, and choroid. Common white dot syndromes whose imaging modalities have been described in this article are multiple evanescent white dot syndrome, acute posterior multifocal placoid pigment epitheliopathy, acute zonal occult outer retinopathy, multifocal choroiditis and panuveitis, punctate inner choroidopathy, serpiginous choroiditis, and birdshot chorioretinopathy. The various imaging modalities help us to better understand the pathophysiology of the various entities and help in diagnosing, monitoring, and prognosticating them. Optical coherence tomography angiography (OCTA) is a comparatively newer tool that helps us to visualize lesions in the choroid that correlate with indocyanine green angiography (ICGA) findings. Even though it is of limited value and cannot replace ICGA, it had gained considerable interest among ophthalmologists. Similarly, the noninvasive nature of modalities such as fundus autofluorescence and OCT makes them appealing and preferable over invasive techniques such as fundus fluorescein angiography and ICGA.

OCTA: Essential or Gimmick?

This commentary article delves into the transformative role of optical coherence tomography angiography (OCTA) in diagnosing and managing a wide array of eye conditions, including diabetic retinopathy, age-related macular degeneration, retinal vein occlusions, and white dot syndromes. Developed in 2005, OCTA has emerged as a non-invasive, high-resolution imaging technique that offers advantages over traditional fluorescein angiography (FA), providing quicker and safer monitoring of ocular conditions with similar diagnostic accuracy. In diabetic retinopathy, OCTA has been instrumental in early identification of retinal changes, offering quantifiable metrics including perfused capillary density (PCD) for assessing vascular alterations. For age-related macular degeneration (AMD), OCTA has deepened our understanding of non-exudative neovascular AMD, allowing for more effective monitoring and potential earlier initiation of treatment. In cases of retinal vein occlusions, OCTA can reveal specific microvascular features and allow for depth-resolved measurements of the foveal avascular zone, providing significant prognostic implications. OCTA has also been invaluable in studying rare white dot syndromes, enabling nuanced differentiation between conditions that often present similarly. Emerging research also suggests that OCTA can have potential utility in neurodegenerative diseases like Alzheimer's, where retinal vascular patterns could offer diagnostic insights. While OCTA is revolutionizing ophthalmic care, further clinical trials and standardization are needed for its broader adoption into clinical practice.

Secondary Multiple Evanescent White Dot Syndrome Following Blunt Trauma.

To report a case of secondary unilateral MEWDS following blunt trauma to the eye. Observational case report of one patient. A 25-year-old male initially presented after being hit in the left eye with a football, with visual acuity of 20/50, traumatic iritis, commotio retinae, vitreous hemorrhage, and a large pigment epithelial detachment in the superior macula. He was lost to follow-up for two years before presenting with a sudden decrease in vision. On exam, best corrected VA (BCVA) was counting fingers and clinical exam demonstrated fibrosis in the superior macula, small white lesions around the optic disc and throughout the posterior pole, and ellipsoid zone disruption on OCT. Given the clinical appearance, a diagnosis of Multiple Evanescent White Dot Syndrome was made and the decision was made to observe. Two months later, without treatment, BCVA improved to 20/20, and there was resolution of the white lesions in the posterior pole as well as improved continuity of the ellipsoid zone on OCT. We describe a case of Multiple Evanescent White Dot Syndrome two years after blunt trauma to the eye; a far longer latency than previously reported cases of MEWDS secondary to blunt trauma.

PUNCTATE INNER CHOROIDOPATHY FOLLOWING PARS PLANA VITRECTOMY FOR HIGH MYOPIC FULL THICKNESS MACULAR HOLE.

To describe a case of punctate inner choroidopathy (PIC) and secondary or epiphenomenon multiple evanescent white dot syndrome (EpiMEWDS) following surgery for high myopic full-thickness macular hole (FTMH). Case report. A 57-year-old high myopic female was diagnosed with cataract and FTMH in the left eye. Her initial best-corrected visual acuity (BCVA) was 20/20 in her right eye and 20/80 in the left eye. She underwent routine combined phacoemulsification and 25-gauge pars plana vitrectomy (PPV) with the inverted internal limiting membrane (ILM) technique and twice-repeated epiretinal membrane (ERM) and ILM staining in the left eye. Two weeks postoperatively, the patient reported significant visual decline and photopsia in her left eye. BCVA decreased to counting fingers. Anterior segment examination was unremarkable. Ophthalmoscopic examination showed multiple whitish-yellow lesions in the macular region compatible with PIC lesions in the left eye. Optical coherence tomography (OCT), blue-light fundus autofluorescence (BAF), fluorescein angiography, and indocyanine green angiography were performed and confirmed the diagnosis. The patient underwent oral steroid therapy for PIC treatment. One week after treatment initiation, BAF showed the occurrence of EpiMEWDS. After one month, all lesions resolved and BCVA improved to 20/100. We report a rare case of PIC and EpiMEWDS development following surgery for FTMH. We hypothesize that several causes, including individual susceptibility (high myopia and female gender), post-surgical inflammation, and/or dye toxicity due to repeated staining could have amplified this inflammatory chorioretinal response. Larger studies are needed to better understand the potential triggers of PIC development after surgery.

Multiple Evanescent White Dot Syndrome following COVID-19 Vaccination: Two Case Reports

Purpose: We present two cases of multiple evanescent white dot syndrome (MEWDS) following COVID-19 vaccination.Case summary: We present two cases of visual field defects following BNT162b2 (Pfizer-BioNTech, Kronach, Germany) COVID-19 vaccination. A 14-year-old male experienced reduced visual acuity in the temporal field of his left eye after his second dose. Similarly, a 38-year-old woman experienced decreased visual acuity in her right eye after her first dose. Notably, neither patient had pre-existing medical conditions or exhibited COVID-19 symptoms following vaccination. Funduscopic examination revealed multifocal peripapillary hyperreflective lesions in both cases. Visual field defects and hyperreflective lesions spontaneously resolved within 6 weeks, with normal visual acuity and fundus findings at the 7-month follow-up.Conclusions: The recent COVID-19 pandemic has led to a significant increase in vaccination rates. Notably, MEWDS demonstrated a significant association with previous vaccines, including influenza and hepatitis, and the cases describe herein also suggest a potential association between BNT162b2 vaccination and MEWDS.

Optic Disc Coloboma May Trigger Multiple Evanescent White Dot Syndrome: Expanding the Clinical Spectrum

Optic Disc Coloboma May Trigger Multiple Evanescent White Dot Syndrome: Expanding the Clinical Spectrum

Application of Ophthalmic Electrophysiology in Inflammatory Disorders of Retina and Optic Nerve.

This review covers the utility of electrophysiological studies relevant to inflammatory diseases of the retina in conditions such as acute posterior multifocal placoid pigment epitheliopathy, acute zonal occult outer retinopathy, Adamantiades-Behçet disease, autoimmune retinopathy and neuro-retinopathy, birdshot chorioretinopathy, multiple evanescent white dot syndrome, and Vogt-Koyanagi-Harada disease. Electrophysiological studies can help with the diagnosis, prognostication, evaluation of treatment effects, and follow-up for these conditions.

Multicenter and multimodal imaging study reveals rare fundus lesions in patients after SARS-CoV-2 infection

To define the characteristics of fundus manifestations in patients after SARS-CoV-2 infection with multimodal imaging techniques. This is a retrospective multicenter and multimodal imaging study including 90 patients. All patients with a visual complaint occurring immediately after SARS-CoV-2 infection were referred to six clinics between December 2022 and February 2023. Demographic information and the temporal relationship between SARS-CoV-2 infection and visual symptoms were documented. The characteristics of the fundus lesions were evaluated using multimodal imaging. Ninety patients from six hospitals were included in this study, including 24 males (26.67%) and 66 (73.33%) females. Seventy-eight patients (86.66%) (146 eyes) were diagnosed with Acute Macular Neuroretinopathy (AMN). The AMN patients were primarily young women (67.95%). Sixty-eight patients (87.18%) had AMN in both eyes. Thirty-eight eyes (24.36%) included Purtscher or Purtscher-like lesions. optical coherence tomography and infrared retinal photographs can show AMN lesions well. Eleven cases were diagnosed with simple Purtscher or Purtscher-like retinopathy (2 cases, 2.22%), Vogt‒Koyanagi‒Harada (VKH) syndrome or VKH-like uveitis (3 cases, 3.33%), multiple evanescent white-dot syndrome (MEWDS) (2 cases, 2.22%), and rhino-orbital-cerebral mucormycosis (ROCM) (5 cases, 5.56%). After SARS-CoV-2 infection, diversified fundus lesions were evident in patients with visual complaints. In this report, AMN was the dominant manifestation, followed by Purtscher or Purtscher-like retinopathy, MEWDS, VKH-like uveitis, and ROCM.

Multiple evanescent white dot syndrome associated with COVID-19 infection: a case report

BackgroundTo report a case of Multiple Evanescent White Dot Syndrome (MEWDS) one month after a COVID-19 infection in a female patient at an age unusual for the occurrence of this disease.Case presentationA 69-year-old Caucasian female reported the presence of floaters, photopsia, and enlarging vision loss in her left eye following the COVID-19 infection. Clinical and multimodal imaging was consistent with the MEWDS diagnosis. Fluorescein angiography examination revealed characteristic hyperfluorescent spots around the fovea in a wreath-like pattern. An extensive lab workup to rule out other autoimmune and infectious etiologies was inconclusive. Visual acuity and white dots resolved after a course of corticosteroids, which was confirmed on follow-up dilated fundus exam and multimodal imaging.ConclusionsMEWDS is a rare white dot syndrome that may occur following COVID-19 infection in addition to other reported ophthalmic disorders following this infection.

Long-Term Clinical Course and Treatment Outcomes in Occult and Overt Acute Zonal Outer Retinopathy

ABSTRACT Purpose To report the longitudinal outcomes for AZOOR patients including treatment response, imaging evolution, and overlap with multiple evanescent white dot syndrome (MEWDS). Methods Visual acuity (VA) and visual field (VF) outcomes of occult and overt AZOOR patients were retrospectively compared between the first and final visits as well as between the two AZOOR subtypes. For treated patients, rates of VA change and fundus lesion area were compared before and after treatment. Analyses were performed using STATA 17. Results Seventeen eyes from 11 occult AZOOR patients and 45 eyes from 29 overt AZOOR patients were included. In a composite VA/VF primary outcome, clinical improvement was noted in five occult AZOOR and three overt AZOOR patients. The decline of logarithm of the Minimal Angle of Resolution (logMAR) VA was minimal in both groups: 0.00016 units/month in occult AZOOR patients and 0.009 units/month in overt AZOOR patients (p = 0.94). Occult AZOOR patients were more likely to have improved or stable VF than overt AZOOR patients (p = 0.04). One occult AZOOR and two overt AZOOR patients developed MEWDS at subsequent visits. Treatment with steroids or immunomodulatory therapy (IMT) was initiated in one occult AZOOR patient and nine overt AZOOR patients. Treated patients had overall VA stability. Fundus lesion area in treated patients changed by a mean of 0.2831 mm2/month, with 40% of patients showing decreased lesion area. Conclusion AZOOR patients generally maintained their VA. Overt AZOOR patients were more likely to receive steroids or IMT; treatment was associated with stabilization of VA.

Increased incidence and diverse manifestations of multiple evanescent white dot syndrome during the COVID-19 pandemic

The advent of coronavirus disease 2019 (COVID-19) pandemic has affected the incidence and course of various diseases and numerous studies have investigated ocular involvement associated with COVID-19 and corresponding vaccines. In this study, we compared the incidence of multiple evanescent white dot syndrome (MEWDS) before and during the COVID-19 pandemic at a single center in Korea and analyzed the demographic and clinical features of patients with MEWDS presenting during the COVID-19 pandemic. We categorized patients with MEWDS into two groups according to date of diagnosis. Pre-COVID19 group included patients diagnosed during the pre-pandemic period (between March 11, 2017, and March 10, 2020), whereas post-COVID19 group included patients diagnosed during the pandemic period (between March 11, 2020, and March 10, 2023). 6 and 12 patients were included in pre-COVID19 group and post-COVID19 group, respectively. Among all hospital visits during the pre-pandemic and pandemic periods, 0.011% and 0.030% were due to MEWDS, indicating a significant increase during the pandemic (p = 0.029, B = 2.756). The annual incidence of patients with MEWDS in 2017–2022 were 0.73, 0.75, 0.78, 1.32, 2.49, and 2.07 per 10,000 population, respectively, corresponding to a significant increase (p = 0.039, B = 1.316). Our results imply that the incidence and manifestation of MEWDS are likely to become more diverse in the COVID-19 pandemic era.

Comprehensive insights into COVID-19 vaccine-associated multiple evanescent white dot syndrome (MEWDS): A systematic analysis of reported cases

ABSTRACT Considering the widespread use of COVID-19 vaccines as a preventive measure against the spread of the virus, it’s necessary to direct attention to the adverse effects associated with vaccines in a limited group of populations. Multiple evanescent white dot syndrome (MEWDS) following COVID-19 vaccination is a rare adverse reaction associated with COVID-19 vaccines. In this systematic review, we collected 19 articles with 27 patients up to November 1, 2023, summarizing the basic information, clinical manifestations, examinations, treatments, and recoveries of the 27 patients. The 27 enrolled patients (6 males, 21 females) had a median age of 34.1 years (15–71 years old) and were mainly from 5 regions: Asia (8), the Mediterranean region (8), North America (7), Oceania (3) and Brazil (1). Symptoms occurred post-first dose in 9 patients, post-second dose in 14 (1 with symptoms after both), post-third dose in 1, and both post-second and booster doses in 1, while details on 2 cases were not disclosed. Treatments included tapered oral steroids (6), topical steroids (3), tapered prednisone with antiviral drugs and vitamins (1), and valacyclovir and acetazolamide (1), while 16 received no treatment. All patients experienced symptom improvement, and nearly all patients ultimately recovered. Moreover, we summarized possible hypotheses concerning the mechanism of COVID-19 vaccine-associated MEWDS. The findings provide insights into the clinical aspects of COVID-19 vaccine-associated MEWDS. More attention should be given to patients with vaccine-associated MEWDS, and necessary treatment should be provided to patients experiencing a substantial decline in visual acuity to improve their quality of life.

The Characteristics of White Dot Syndromes Following SARS-COV-2 Infection: A Systematic Review.

To review all studies reporting the occurrence of white dot syndromes (WDSs) following SARS-COV-2 infection. On May 12, 2023, we registered our protocol on PROSPERO [registration number: CRD42023426012]. Five different databases including PubMed, Scopus, Web of Science, Google Scholar, and Science Direct were searched up to May 2023. We included all studies that reported the symptoms of WDSs following SARS-COV-2 infection. The data was extracted using a uniform Excel extraction sheet. All statistical tests were conducted with a 95% confidence interval and a 5% error margin. A p-value of less than 0.05 was considered statistically significant. The publication bias of included studies was assessed using JBI Critical Appraisal Checklist for Case Reports and IHE Quality Appraisal Checklist for Case Series studies. This review included thirty-two studies involving forty-eight patients. Acute macular neuroretinopathy was the most common disease (70.8%) followed by multiple evanescent white dot syndrome (14.6%) with 58.3% of WDS after their first SARS-COV-2 infection, and paracentral acute middle maculopathy (4.1%). They were mostly unilateral (56.2%). The presenting symptoms were blurred vision (70.8%), visual field disturbance (68.7%), and photopsia (20.8%). About 35.4% of the patients improved by their treatment and future complications were persistent scotoma (4.2%) and macular edema (2.1%). White dot syndromes are very rare entities. Our findings suggest a possible association between white dot syndrome onset and SARS-COV-2 infection. We recommend ophthalmologists should be aware of this suggested association to deliver better management and patients' care.

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.

Multiple evanescent white dot syndrome: case presentation and literature review

The multiple evanescent white dot syndrome is a multifocal ocular condition involving the choroid and the retina that typically affects young myopic females. It is typically unilateral and has a favorable evolution without treatment, recovering the visual function and normal structure upon several weeks since the onset of the condition. In some cases, treatment via systemic corticosteroids has been chosen, as in the case of the patient selected for the present work, in which an excellent visual and structural result was obtained much faster by using the treatment with corticosteriods. The present article presents a literature review on the current issues about this pathology and the possibilities with regard to its management.

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.