Features
Multiple evanescent white dot syndrome (MEWDS) is unilateral, multifocal retinitis that affects young, myopic women aged 12 to 57 years, with a mean age of 27 years. Females are affected three to five times more often than males. There are few reports of bilateral or asymmetric presentation. There is no apparent ethnic preponderance.
68.1.1 Common Symptoms
Acute, painless, unilateral blurry vision common; photopsias, dyschromatopsia, and an enlarged blind spot or central/paracentral scotoma are often present. About one-third to one-half of patients generally report a flu-like prodrome 1 to 2 weeks prior to presentation.
68.1.2 Exam Findings
Multiple discrete nummular white spots from 100 to 750 µm are seen in the posterior pole and midperipheral retina at the level of the outer retina, retinal pigment epithelium, and inner choroid (▶ Fig. 68.1). Each spot is actually composed of a cluster of smaller lesions. The fovea may appear granular with white, yellow, or orange specks. Other less common findings include a mild vitritis, retinal vasculitis, disc edema and hyperemia, and relative afferent pupillary defect. External and anterior segment examinations are typically normal. Ophthalmoscopy typically confirms the diagnosis, but additional imaging can be supportive.
Fig. 68.1 Multiple white lesions at the level of the outer retina and retinal pigment epithelium are seen in the posterior pole and the midperiphery in multiple evanescent white dot syndrome.
68.2 Key Diagnostic Tests and Findings
68.2.1 Optical Coherence Tomography
A dome-shaped hyperreflective lesion in the subretinal space with increased choroidal reflectivity corresponds to the white dots. Inner segment/outer segment (i.e., ellipsoid zone) disruption can also be seen, often in both eyes.
68.2.2 Fluorescein Angiography or Ultra-Widefield Fluorescein Angiography
White spots exhibit early punctate hyperfluorescence with a wreath-like appearance. Late staining of the spots and late leakage of the disc are also visible (▶ Fig. 68.2). Window defects may correlate with macular granularity. Fluorescein angiography (FA) can continue to show lesions even after clinical resolution of spots.
Fig. 68.2 Fluorescein angiography shows disk leakage, mild vascular staining, and wreath-like staining of the white lesions in the posterior pole in the late phase.
68.2.3 Indocyanine Green Angiography
Multiple hypocyanescent spots are visible which correspond to white dots. More spots are seen with indocyanine green angiography (ICGA) than are detected with ophthalmoscopy or FA. A hypocyanescent annular area encircling the optic nerve is associated with the enlarged blind spot. Spots may persist for many months after resolution of visual symptoms and inflammation. Spots are sometimes present in the other, asymptomatic eye.
68.2.4 Fundus Autofluorescence
Hyperautofluorescence corresponds to the white dots, which may be more easily visualized with fundus autofluorescence (FAF; ▶ Fig. 68.3). Even in the absence of white dots on funduscopic examination, FAF can reveal the characteristic lesions. FAF lesions can persist even after FA and ICGA lesions appear resolved.
Fig. 68.3 Montage fundus autofluorescence demonstrates hyperautofluorescent dots that correlate with the white dots observed on funduscopy.
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