Are There Two Forms of Multiple Evanescent White Dot Syndrome?

Essilfie, Juliet; Bacci, Tommaso; Abdelhakim, Aliaa H.; Ramtohul, Prithvi; Turchi, Federica; Freund, K. Bailey; Yannuzzi, Lawrence A.

doi:10.1097/iae.0000000000003288

Cited by 30 publications

(43 citation statements)

References 30 publications

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“…A novel hypothesis has been advanced, according to which secondary WDS are triggered by damage to the choriocapillaris, the BrM, and the RPE after unveiling antigens usually hidden to the immune system. 9,12 White dot syndromes with PIC-like features have been well described in eyes with PM. 7 Our cases are examples of WDS with PIC-like features in eyes with extensive outer retinal disruption because of inherited or acquired conditions other than PM.…”

Section: Discussionmentioning

confidence: 99%

“…4,[8][9][10][11] Secondary MEWDS or MEWDSlike reaction has the same benign clinical course as primary MEWDS and does not seem to modify the visual prognosis of the underlying disease. 9,12 The acronym EpiMEWDS for epiphenomenon MEWDS has also been proposed, suggesting that MEWDS may be a secondary phenomenon that occurs alongside or in parallel to a primary retinal condition. 12 Parallelly, PIC is clinically associated with pathologic myopia (PM).…”

mentioning

confidence: 99%

“…9,12 The acronym EpiMEWDS for epiphenomenon MEWDS has also been proposed, suggesting that MEWDS may be a secondary phenomenon that occurs alongside or in parallel to a primary retinal condition. 12 Parallelly, PIC is clinically associated with pathologic myopia (PM). 7,13 As PIC lesions tend to cluster around sites of pre-existent retinal pigment epithelium/Bruch membrane (RPE/BrM) complex damage (e.g., lacquer cracks) in eyes with PM, a causative relationship between local inflammation and mechanical insults to RPE/BrM has been postulated.…”

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confidence: 99%

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Punctate Inner Choroidopathy–like Reactions in Unrelated Retinal Diseases

Cicinelli

Marchese

Ramtohul

et al. 2022

Retina

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Purpose: To report a cohort of patients with a punctate inner choroidopathy (PIC)-like reaction in concurrent, unrelated, chorioretinal disorders.Methods: This was a retrospective observational study of patients seen at two referral centers with lesions consistent with PIC on multimodal imaging; patients with lesions resembling idiopathic multifocal choroiditis were also included. Active PIC-like lesions appeared as focal hyperreflective lesions splitting the retinal pigment epithelium/Bruch membrane (RPE/BrM) complex on optical coherence tomography. Chronic PIC-like lesions included subretinal fibrosis, multifocal punched-out chorioretinal atrophy, and curvilinear streaks. Patients' demographics, additional imaging features, and treatment responses were collected and summarized.Results: Twenty-two eyes of 16 patients with a PIC-like reaction were included (75% females; median age 40 years). Underlying diagnoses included hereditary retinal conditions (10 patients, 63%) and acquired etiologies, all characterized by the RPE/BrM or outer retinal disruption. Fifteen eyes (68%) had active PIC-like lesions; seven eyes (32%) had chronic PIC-like lesions. Active PIC-like lesions regressed with time and responded to systemic steroids. Subretinal fibrosis (3 eyes, 20%), macular atrophy (3 eyes, 20%), and concomitant subretinal fibrosis and macular atrophy (5 eyes, 33%) developed on follow-up. Recurrences occurred in five eyes (23%).Conclusion: RPE/BrM or outer retina disruption may trigger a PIC-like reaction in susceptible patients, presumably because of the loss of immune privilege. A PIC-like reaction may influence the clinical progression and the visual prognosis of the primary chorioretinal disease.

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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confidence: 99%

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Punctate Inner Choroidopathy–like Reactions in Unrelated Retinal Diseases

Cicinelli

Marchese

Ramtohul

et al. 2022

Retina

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“…One hypothesis regarding the pathogenesis of secondary MEWDS is that damage to subretinal tissue may alter the posterior retinal-blood barrier and trigger transient major inflammation in the outer retina. 17,19 Therefore, inflammation of the outer retina may start around the initial chorioretinal lesions and spread secondarily, explaining why secondary MEWDS is less extensive. In cases of MEWDS secondary to optic neuritis, the inflammation may spread through contiguity from the optic nerve to the retina, resulting in MEWDS.…”

Section: Discussionmentioning

confidence: 99%

Comparison of Primary and Secondary Forms of Multiple Evanescent White Dot Syndrome

Serrar

Cahuzac

Gascon

et al. 2022

Retina

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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).Methods: A total of 101 eyes in 100 patients were included in a multicentric retrospective study.Results: 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). Conclusion: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.

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“…The disruption of the ellipsoid zone could be seen and that could make the foveal area granular. Furthermore, optical coherence tomography showed swelling of the outer retinal layers and granules at the level of RPE [ 7 ].…”

Section: Case Reportmentioning

confidence: 99%

White Dot Syndrome Report in a SARS-CoV-2 Patient

Smeller¹,

Tóth-Molnár²,

Sohár³

2022

Case Rep Ophthalmol

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Our purpose was to report clinical features in bilateral white dot syndrome in a 47-year-old female patient who was tested positive for the SARS-CoV-2. A 47-year-old female visited our department with complaints of bilateral photophobia and blurred vision in both her eyes. She visited our department during the pandemic period after her PCR-proven SARS-CoV-2 positivity. Her symptoms were chills and fever with a temperature of 40.0°C, associated with fatigue, sweat, and complete loss of taste. Besides basic ophthalmological examinations, ocular diagnostic testing were made to differentiate between specific white dot syndromes with suggestive features of fluorescein angiography, optical coherence tomography, and fundus autofluorescence. Laboratory tests were ordered, including immunserological and haematological ones. Eye examination revealed mild bilateral vitritis and white dots in the fundus of both eyes, including the macula explaining the blurred vision. Herpes simplex virus reactivation was proved, after the SARS-CoV-2 infection. Local corticosteroids were given according to the European Reference Network’s recommendations for patients with uveitis during the COVID-19 pandemic. Our report demonstrates that white dot syndrome with blurred vision could be associated with SARS-CoV-2 infection, being potentially sight-threatening because of macular involvement. Ophthalmological examinations found posterior uveitis white dot syndrome, and this should call attention to the risk of acute 2019-CoV infection or occurred 2019-CoV infection. Immunodeficiency favours the occurrence of other viral infections, such as herpes virus infections. Everybody should be aware of the risk of 2019-CoV infection, especially professionals, social workers, and those who work or live with elder people and people with immunodeficiency.

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Are There Two Forms of Multiple Evanescent White Dot Syndrome?

Cited by 30 publications

References 30 publications

Punctate Inner Choroidopathy–like Reactions in Unrelated Retinal Diseases

Punctate Inner Choroidopathy–like Reactions in Unrelated Retinal Diseases

Comparison of Primary and Secondary Forms of Multiple Evanescent White Dot Syndrome

White Dot Syndrome Report in a SARS-CoV-2 Patient

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