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 54-year-old Chinese man presented in the clinic for a routine two-year ophthalmic examination. Past medical history is PNH. The patient was diagnosed with PNH at the age of 22. He is on Eculizumab (anti-complement factor 5 antibody) infusion which he started in 2007.\nHe was asymptomatic with best-corrected visual acuity of 20/40 on the right eye (spherical equivalent is +0.5), and 20/20 on the left eye (spherical equivalent is +0.25). Slit-lamp examination and intraocular pressures were normal. Posterior segment examination revealed subtle pigmentary changes temporal to the macula in both eyes (). Enhanced depth imaging-OCT (EDI-OCT) of the macula was obtained and the entire posterior pole was also scanned. The OCT-detected multiple FCEs corresponded with the retinal pigmentary changes by infrared fundus photo in OCT (). There was no FCE involving the foveal region. EDI-OCT showed the retinal pigment epithelium (RPE) layer complied with the contour of the choroidal excavation. And the outer nuclear layers (ONLs) conformed to the contour of the RPE within the excavation (). There was no RPE detachment and no separation between the photoreceptor layers and the RPE. One area of ONL in proximity to the excavation demonstrated hyperreflectivity, similar to the reflectivity of the adjacent outer plexiform layer (OPL) ( OD1 and OS2). The inner retinal layers from the OPL to the retinal nerve fiber layer were essentially undisturbed. Choriocapillaris thinning in the site of excavation was adjacent to large blood vessels as compared with the normal surrounding choroid layers without FCE ( OD2). The sclerochoroidal junction was smooth and undisturbed without staphylomatous changes.\nOn FA, the FCE appeared to have normal fluorescence without leakage. No sign of neovascularization was found in the inner and subretinal space.\nFAF photos of both eyes showed irregular hyper and hypoautofluorescence in the macular area, which suggested some RPE loss or disturbance, which is corresponding to the locations of FCEs ().\nHumphrey Visual field testing in both eyes was performed twice. First was 24-2 Sita fast and the second was 10-2 Sita fast. But both of the visual field results were not reliable because of a high fixation loss.\nThe patient was observed without any intervention. The patient was followed up in 6 months with no changes of the symptoms, visual acuity, and the fundus exam.