SECONDARY MULTIPLE EVANESCENT WHITE DOT SYNDROME IN A PATIENT WITH NORTH CAROLINA MACULAR DYSTROPHY

To describe a novel case of secondary multiple evanescent white dot syndrome in a patient with North Carolina Macular Dystrophy.

The patient was evaluated with ultra-widefield color and autofluorescence imaging, fluorescein angiography, and spectral-domain optical coherence tomography.

A 43-year-old man with longstanding blurred vision in both eyes acutely developed glare in the right eye after an upper respiratory illness. He had chorioretinal atrophy in both eyes consistent with North Carolina Macular Dystrophy, which was later confirmed by genetic testing. He also had white spots in the macula of the right eye consistent with secondary multiple evanescent white dot syndrome. The diagnosis of secondary multiple evanescent white dot syndrome was confirmed with fundus autofluorescence and fluorescein angiography, and a negative infectious workup. His symptoms and the retinal lesions had resolved by 11 weeks and after a short course of oral corticosteroids.

Prior studies have suggested a link between disruption of the retinal pigment epithelium/Bruch membrane complex and secondary multiple evanescent white dot syndrome. This report describes the first case of secondary multiple evanescent white dot syndrome after North Carolina Macular Dystrophy and provides further support for this association.