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Progressive confluent circumpapillary multiple evanescent white-dot syndrome.

J K Luttrull¹, M F Marmor, M Nanda

¹Department of Ophthalmology, School of Medicine, Stanford University, Stanford, California, USA.

American Journal of Ophthalmology

|October 8, 1999

Summary

Severe multiple evanescent white-dot syndrome (MEWDS) can present with a rare, progressive circumpapillary discoloration mimicking other conditions. This atypical presentation highlights diagnostic challenges in MEWDS.

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Area of Science:

Ophthalmology
Retinal Diseases

Background:

Multiple evanescent white-dot syndrome (MEWDS) is an idiopathic inflammatory condition affecting the outer retina and retinal pigment epithelium.
Diagnosis typically relies on characteristic funduscopic findings and multimodal imaging.

Observation:

This study describes a morphologic variant of MEWDS in three patients with severe, unilateral disease.
The variant presented with atypical progressive circumpapillary discoloration of the fundus.

Findings:

The confluent circumpapillary lesion evolved from pinpoint dots to wreath-like spots, characteristic of MEWDS.
The lesion progressed, resembling geographic retinitis or retinal pigment epitheliitis before resolving spontaneously.

Implications:

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This variant can mimic other retinal disorders, complicating the initial diagnosis of MEWDS.
Accurate identification is crucial to avoid unnecessary investigations and inappropriate treatments for patients with MEWDS.