Expanded Clinical Spectrum of Multiple Evanescent White Dot Syndrome With Multimodal Imaging

Abstract

Purpose: To evaluate and characterize multiple evanescent white dot syndrome abnormalities with modern multimodal imaging modalities.Methods: This retrospective cohort study evaluated fundus photography, fluorescein angiography, indocyanine green angiography, optical coherence tomography, enhanced depth imaging optical coherence tomography, short-wavelength autofluorescence, and near-infrared autofluorescence.Results: Thirty-four multiple evanescent white dot syndrome patients with mean age of 28.7 years were studied (range, 14-49 years). Twenty-six patients were women, and eight were men. Initial mean visual acuity was 0.41 logMAR. Final mean visual acuity was 0.03 logMAR. Fluorescein angiography shows a variable number of mid retinal early fluorescent dots distributed in a wreathlike pattern, which correlate to fundus photography, fundus autofluorescence, and indocyanine green angiography. Indocyanine green angiography imaging shows the dots and also hypofluorescent, deeper, and larger spots, which are occasionally confluent, demonstrating a large plaque of deep retinal hypofluorescence. Optical coherence tomography imaging shows multifocal debris centered at and around the ellipsoid layer, corresponding to the location of spots seen with photography, indocyanine green angiography, and fluorescein angiography. Protrusions of the hyperreflectant material from the ellipsoid layer toward the outer nuclear layer correspond to the location of dots seen with photography, indocyanine green angiography, and fluorescein angiography.Conclusion: Multimodal imaging analysis of the retina in patients with multiple evanescent white dot syndrome shows additional features that may help in the diagnosis of the disease and in further understanding its etiology. Multiple evanescent white dot syndrome is predominantly a disease of the outer retina, centered at the ellipsoid zone, but also involving the interdigitation zone and the outer nuclear layer.RETINA 36: [64][65][66][67][68][69][70][71][72][73][74] 2016

Choroidal neovascularisation triggered multiple evanescent white dot syndrome (MEWDS) in predisposed eyes

“…The MEWDS diagnosis was established according to very strict criteria: all six cases met the diagnostic criteria as described by Marsiglia et al 2. Furthermore, as previously described, the hyperautofluorescent spots disappeared after photobleaching 3.…”

“…Recently, Marsiglia et al described and reviewed the characteristic multimodal imaging findings for MEWDS 2. They described hyperfluorescent dots on fluorescein angiography (FA), hypocyanescent spots and dots on indocyanine green angiography (ICGA) and the disruption and alteration of the ellipsoid zone (EZ) on optical coherence tomography (OCT) with subretinal hyper-reflective material.…”

Clinical applications of fundus autofluorescence in retinal disease

“…This disease affects young, healthy women 20–50 years old and manifests as a self-limited, unilateral presentation with multifocal 100–200 µm white spots scattered in the paramacular and mid-peripheral fundus [170]. …”

“…13) [80, 171]. Hypo-autofluorescent foci at the fovea correspond with foveal granularity on exam and accumulation of hyper-reflective material interdigitating between the RPE and photoreceptor layer on OCT [170]. FAF reveals more lesions than on clinical exam or FA, though less than seen on indocyanine green angiography [172, 173], and FAF may be used in lieu of FA and ICG due to its high sensitivity, greater ease of use, and non-invasive nature of administration.

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Multi-modal imaging and anatomic classification of the white dot syndromes

“…Accumulation of hyperreflective material that rests on the RPE and extends anteriorly through the interdigitation zone, ellipsoid zone, and outer nuclear layer (ONL) can also be seen on OCT [61]. FA exhibits a variable number of early hyperfluorescent lesions in a wreathlike configuration in the mid-retina which persists into the late phase of the FA [61]. ICGA shows early and mid-phase hypocyanescent dots and/or plaques that are greater in number than those evident on FA.…”