A 37-year-old woman with moderate myopia presented with complaints of blurred vision and photopsia in her right eye for 1 week. She had no antecedent flu-like illness. The patient’s medical and family histories were unremarkable. Best-corrected visual acuity (BCVA) with a Japanese standard Landolt visual acuity chart was 0.09 OD and 1.2 OS. Left eye showed normal appearance. Slit lamp examination showed 2+ cells in the anterior vitreous OD. Funduscopic examination showed foveal granularity and multiple white dots (Figure A, white arrows) extending from the posterior pole to the retinal midperiphery. Some white dots in the temporal fovea were relatively large (Figure A, yellow arrows). Fluorescein angiography (FA) showed hyperfluorescent spots (Figure B, arrows) from the initial phase, corresponding to the white dots, and the staining of retinal veins and the optic disc in the late phase. ICGA was normal in the early phase, but in the late phase numerous hypofluorescent spots were scattered over a wider area with and without white dots (Figure C). Goldmann perimetry showed a blind spot enlargement and a small central scotoma OD. Additionally, EDI-OCT showed hyper-reflective lesions in the ganglion cell layer (GCL) (Figure A, black arrowheads), the loss of photoreceptor inner segment/outer segment junction (IS/OS) line, detachment between the RPE and Bruch’s membrane, moderately reflective, dome-shaped focal lesions within the photoreceptor layer (Figures A and A; white arrowheads), and locally thickened choroid compared with neighboring areas, corresponding to the large white dots in the temporal fovea and inferotemporal perifovea with pigment epithelium detachment. Multiple ruptures of the RPE and Bruch’s membrane were apparently observed in the temporal lesion (Figure A, red and yellow arrowheads), whereas Bruch’s membrane underlying the damaged RPE was found to be continuous in the inferotemporal lesion (Figure A, red and yellow arrowheads). Choroidal thickness at the fovea and the adjacent temporal lesion was 243 and 330 μm OD, respectively (Figure A). The patient received a diagnosis of MEWDS OD.\nThe white dots spontaneously resolved 1 month after the initial visit, with visual acuity improving to 0.2 OD. The pigment epithelium detachment and abnormal GCL hyper-reflective lesions completely resolved and the photoreceptor-layer focal lesions markedly decreased in the temporal fovea (Figure B). Interestingly, a conforming FCE, without an intervening hyporeflective space between the photoreceptor tips and the RPE [], developed in the same region (Figure B). Choroidal thickness at the fovea and the FCE lesion decreased to 210 and 140 μm, respectively (Figure B). An abnormal hyper-reflective lesion also appeared in the choroid below the FCE (Figure B, arrow) and increased in size 2 months after the initial visit.\nThree months after the initial visit, BCVA improved to 0.4 and scars formed in the temporal and inferotemporal lesions (Figure D, yellow arrows). On ICGA, almost all hypofluorescent lesions disappeared, but the scarred sites exhibited hypofluorescent spots from the initial phase. On EDI-OCT images, the focal lesions in the photoreceptor layer almost resolved (Figures C and B), but cystoid changes emerged in inner retinal layers together with marked thinning of outer retinal layers (Figure C). The choroid below the temporal and inferotemporal scars developed massive hyper-reflective lesions (Figures C and B; yellow arrows), and the subfoveal choroidal thickness further decreased to 181 μm (Figure C). The inferotemporal perifoveal lesion did not develop any apparent sign of FCE (Figure B), in contrast to the temporal foveal lesion (Figure B,C). Twelve months after the initial visit, BCVA was 0.7 OD and the disrupted IS/OS line at the fovea partially improved. No recurrence of white dots or choroidal neovascularization had ever been observed.
The chief complaint for this 41-year-old woman was impaired vision in her right eye. A photograph of her right fundus showed the presence of yellow dots (Figure A). A visual field test during the initial visit indicated there was an enlarged blind spot in her right eye (Figure C). In addition, OCT also revealed a disrupted or irregular photoreceptor inner outer segment (IS/OS) junction in right eye (Figure A) indicated by the arrows, although the IS/OS junction in left eye was normal (Figure B). After 4 months, the IS/OS junction in her affected eye was restored (Figure D). At her initial visit, the deviation map of the ganglion cell analysis showed there was a decrease of the GCL + IPL thickness in both eyes (Figure A and D), even though her left eye was not affected. During the 15 months of follow-up, the deviation map did not indicate any recovery of the decreased GCL + IPL thickness. Signal strengths of all the Cirrus OCT measurements were 10/10.