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Last Month’s Blink
Idiopathic Acute Exudative Polymorphous Vitelliform Maculopathy
Written by Matthias Koch, MD, and Marion R. Munk, MD, PhD. Photos by Nicole Mantel. All are at Inselspital, University Hospital Bern, Switzerland. Dr. Munk is also at Gutblick, Pfäffikon, Switzerland.
A 23-year-old man presented to our clinic in Switzerland with blurred vision for six days. Upon further investigation, he reported a cold and subfebrile temperature a few days earlier. He reported no headaches, use of medications or illegal drugs, or travel outside of Western Europe. Personal and family ophthalmic history was unremarkable.
VA was 20/16 in the right eye and 20/20 in the left eye, and the anterior segment evaluation was unremarkable. However, in both eyes, the fundus examination revealed a blunted foveal reflex with multiple yellowish-to-white lesions in the posterior pole (Fig. 1), which were even more visible in the multicolor image (Fig. 2). In both eyes, spectral-domain OCT of the posterior pole revealed a few intraretinal cysts and retinal detachment extending to the vascular arcades (Fig. 3A, 3B). Fundus autofluorescence (FAF) showed vermicular hyperautofluorescent lesions (Fig. 4).
Systemic infectious diseases and paraneoplastic origins were ruled out. Within one week, the intraretinal fluid completely resolved, and the subretinal fluid (SRF) decreased. Three weeks after the initial onset, SRF increased again, and VA dropped to 20/32. Systemic steroids were initiated; three months later, SRF had completely resolved, and best-corrected VA had improved to 20/16 in both eyes. However, residual deposits, nicely visible on FAF, remained.
The clinical findings and course are characteristic of acute exudative polymorphous vitelliform maculopathy. Although most patients regain full VA within a few months, subretinal deposits may persist for years. Recurrent or chronic causes are described in the literature. Genetic testing for bestrophinopathy should be considered, and a systemic workup to rule out masquerading diseases is highly recommended.
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