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.
A 40-year-old Japanese woman presented with a complaint of blurred vision of her left eye for 1 week after catching a cold. Her past medical history was unremarkable. At the initial examination in our hospital, best-corrected visual acuity (BCVA) using a decimal visual test chart was 1.2 and 0.6 in her right and left eyes, while the intraocular pressure (IOP) was 16 and 13 mm Hg in her right and left eyes, respectively. Slit-lamp examination showed there were no inflammatory cells in the anterior chamber and 1+ cells in the vitreous of her left eye. Ophthalmoscopy revealed multiple white dots ranging from the posterior pole to the midperiphery, with yellow granularity observed in the fovea (Fig. ). Fluorescein angiography (FA) revealed the presence of multiple hyperfluorescent spots from the early through the late phase, with staining of the optic disc observed in the late phase (Fig. ). ICGA demonstrated the presence of hypofluorescence in the fovea with multiple hypofluorescent spots ranging from the posterior pole to the midperiphery in the late phase (Fig. ). Hyperfluorescent lesions in FA and hypofluorescent lesions in ICGA demonstrated the presence of multiple white dots in the fundus. A Humphrey field analyzer revealed a slight enlargement of the blind spot of Mariotte in the left eye. SD-OCT demonstrated the presence of a separation between the RPE and Bruch's membrane (BM) (pigment epithelium detachment), discontinuations in the RPE and BM (Fig. ), and a hyper-reflective lesion in the outer retina under the fovea (Fig. ). A loss of the EZ (Fig. ) and a hyper-reflective lesion in the ganglion cell layer (Fig. ) were observed at the parafovea. Choroidal thicknesses at the center of the fovea and the edge of the pigment epithelium detachment were 235 and 250 μm, respectively (Fig. ). Three weeks after the onset, the hyper-reflection spread to the area between the inner lesion of the outer nuclear layer (Fig. ), and an abnormal hyper-reflective lesion appeared within the choroid (Fig. ). In addition, the separation between the RPE and BM was found to have almost completely disappeared (Fig. ). A hyper-reflective band was clearly observed from the RPE to the sclera under the fovea (Fig. ). The patient was diagnosed with MEWDS and treated with prednisolone, with the 15 mg dose gradually tapered after the initial presentation. Four weeks after the onset, several of the white dots disappeared, the yellow granularity in the fovea became slightly smaller, and the BCVA in the left eye improved to 1.2, which was subsequently maintained. OCT showed there was a slight but clear choroidal excavation with a slight separation between the RPE and neural retina detected under the central fovea (Fig. ). Choroidal thicknesses at the center of the fovea and at the edge of the FCE were 183 and 228 μm, respectively (Fig. ). Three months after the onset, the subretinal fluid had been completely absorbed, with the nonconforming FCE then changing to the conforming FCE (Fig. ). BCVA in the left eye was 1.2, a value that was subsequently maintained. The hyper-reflective lesion in the ganglion cell layer completely resolved. Choroidal thicknesses at the center of the fovea and at the edge of the FCE decreased and became 147 and 200 μm, respectively (Fig. ). Six months after the onset, BCVA of the left eye remained at 1.2 and the EZ at the fovea was clearly detected. There has been no subsequent recurrence of MEWDS or choroidal neovascularization observed. The choroidal excavation has gradually deepened (Fig. ), with the choroidal thicknesses at the center of the fovea and at the edge of the FCE determined to be 132 and 191 μm, respectively (Fig. ).