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Mafalda Macedo, Sara Vaz-Pereira, Gabriella De Salvo, Bishwanath Pal; Natural history of Multiple Evanescent White Dot Syndrome: a multimodality imaging study. Invest. Ophthalmol. Vis. Sci. 2013;54(15):5384.
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To describe the natural history and imaging features of a case series of multiple evanescent white dot syndrome (MEWDS).
Retrospective review of three patients with MEWDS followed at the Medical Retina Department of Moorfields Eye Hospital (MEH). We performed a comprehensive ophthalmic examination, including slitlamp biomicroscopy, funduscopy, evaluation with Amsler grid, visual fields, fundus photographs, red-free images, Spectralis® optical coherence tomography (Spectralis® OCT), fundus autofluorescence (FAF), fluorescein angiography (FA) and indocyanine green angiography (ICGA).
Three previously healthy female patients with an average age of 26 years old presented to MEH with complaints of unilateral acute visual loss, photopsias and central/paracentral scotomas. The average visual acuity (VA) at presentation was 6/11. Fundus examination included multiple yellow-white dots extending from the posterior pole out to the mid-peripheral retina associated with an orange granular appearance of the fovea and a swollen hyperemic optic disc. FAF demonstrated hyperautofluorescence in the distribution corresponding to altered retinal pigment epithelium (RPE) pigmentation. Spectralis® OCT showed pathologic disruption of the outer retina with possible RPE involvement. FA and ICGA were also consistent with the diagnosis. In all patients the prognosis was good with complete spontaneous recovery of vision and resolution of fundus abnormal findings within several weeks. The disrupted outer retina in the acute phase was restored in the convalescent phase in all patients. One patient had a recurrence of MEWDS in the same eye twenty months after the first episode. In this patient, functional and structural abnormalities returned to normal within seven weeks.
The correct diagnosis of MEWDS can be a challenge due to the subtle and evanescent nature of fundus abnormalities. However, a detailed medical history along with several morphological and functional examinations are essential in the diagnosis and follow-up of these patients and may allow a deeper insight in the pathophysiology of this entity. MEWDS appears to involve the outer retina and the RPE/choriocapillaris. It is usually a self-limiting condition with complete visual recovery within a few weeks after the onset, even though it may rarely recur.
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