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. ).
A 55-year-old man (subject 1) presented with bilateral metamorphopsia, with decimal visual acuity being 0.8 in the right eye and 1.2 in the left. The clinical diagnosis of VKH was made based on bilateral panuveitis and multifocal exudative retinal detachments at the posterior poles (). High-dose corticosteroid therapy with gradual tapering was initiated 3 days after presentation. The serous detachments entirely resolved within 9 weeks and visual acuity in the right eye improved to 1.2.\nSerial spectral-domain OCT (SD-OCT) images obtained at the preuveitic, uveitic, and posttreatment phase are shown in . SD-OCT obtained 1 year before the onset of VKH due to symptoms of a floater and visual disturbance demonstrated a dome-shaped posterior protrusion of the RPE and outer retinal layers into the choroidal cavity (ie, conforming FCE) at the fovea in the right eye. SD-OCT at the uveitic phase identified multiple bilateral sensory retinal detachments, with the FCE now involving only the RPE (ie, nonconforming FCE). Nine weeks after the treatment, the nonconforming FCE returned into a conforming pattern.\nThe FCE pattern and its alteration during treatment of the case with VKH were compared to those of four subjects with FCE associated with other chorioretinal conditions (). This comparison group consisted of two patients with age-related macular degeneration (AMD; subjects 2 and 3), one subject with a macular hole (MH; subject 4), and one asymptomatic individual (subject 5). Treatment with intravitreal ranibizumab injections and vitrectomy had been performed in the patients with AMD and MH, respectively. A conforming FCE was observed in three patients (subjects 2, 3, and 5) and pattern alteration from nonconforming to conforming FCE was found after treatment in one subject (subject 4). In the two subjects with AMD, a hyperreflective material around the FCE was absorbed, resulting in a well-demarcated conforming FCE after treatment.