Purpose: To report the clinical case of a 64-year-old man who developed several features of multiple evanescent white dot syndrome (MEWDS) but with central visual loss that persisted because of significant structural macular disease.
Methods: A case report was generated by a review of the clinical course and imaging investigations of one patient, along with review of the literature.
Results: A 64-year-old man noted decreased vision in the right eye for 2 weeks, associated with a central scotoma with shimmering, gauze effect of the vision. He had a good vision with normal ophthalmic examinations in the past. Ophthalmic examination revealed acuity of 20/400 with central scotoma and trace pupillary defect in the right eye, normal anterior segment, no evidence of intraocular inflammation, and fundus findings of unilateral MEWDS associated with a central zone of macular pigmentary atrophy. Autofluorescence imaging revealed reflectance changes of MEWDS as well as prominent central hypofluorescence and a zone of hyperfluorescence in inferior macula. Spectral-domain optical coherence tomography identified mild disruptions of outer retina in the regions of the MEWDS lesions and disorganization of the outer retina in the macular region overlying a shallow irregular retinal pigment epithelial detachment. The choroid demonstrated increased thickness compared with the fellow eye with suggestions of dilated outer vessels/pachyvessels. Fluorescein angiography and indocyanine green angiography revealed window defects and staining and hypocyanescence, respectively, of the central macular lesion. The MEWDS lesions resolved without evolution to chorioretinal scars, and the central vision and lesions did not benefit from a systemic steroid course or intravitreal anti-vascular endothelial growth factor therapy.
Conclusion: This case illustrates an unusual presentation of MEWDS characterized by unilateral retinal disease presenting with symptoms and signs of profound central macular dysfunction. The clinical course did not evolve into other inflammatory retinal phenotypes, such as multifocal choroiditis or AZOOR (acute zonal occult outer retinopathy) that can sometimes develop MEWDS-like features or central disease. The central structural disease resembles some features of the pachychoroid clinical spectrum, which may have represented a superimposed diagnosis unrelated to the inflammatory phenotype.