A 16-year-old woman with mild myopia complained of blurred vision and photopsia of the right eye for 1 month. The patient had no particular medical or ocular histories.\nHer BCVA was 1.2 OU. Relative afferent pupillary defect was negative. Left eye was normal. Slit-lamp examination revealed no cells in the anterior chamber, but mild anterior vitreous cells OD. Funduscopic examination showed multiple, faint, small, subretinal white dots at the posterior pole to the midperiphery (, arrowheads), macular granularity, and retinal vascular sheathing at superotemporal midperiphery (, arrows). On FA, the hyperfluorescence was seen from the initial phase corresponding to the white dots (, arrowhead), with the staining of the optic disk and vascular leakages corresponding to the retinal vascular sheathing on the late phase (, arrow). On late-phase indocyanine green angiography, multiple spots of hypofluorescence were observed at the wider area despite no initial abnormal findings (, arrowheads). EDI-OCT revealed segmental loss of the ellipsoid zone at areas of the white dots (, arrows), and the SCT was 341 µm. Goldmann perimetry showed a mild blind spot enlargement of 5×10 degree OD and no abnormality OS. On Humphrey perimetry, MD value was −5.47 dB OD, and OS was unmeasured. In mfERG, entirely decreased amplitude except central normal amplitude was present OD, and the result was normal OS. Systemic screening, similar to what was done for Case 1, showed negative results. She was diagnosed as having MEWDS OD and was followed up with no treatment. One month later, white dots and retinal vasculitis spontaneously disappeared. Three months after the initial visit, the ellipsoid zone at the macula restored and the SCT decreased to 319 µm (). The BCVA remained unchanged. Twelve months after the initial visit, the MD value improved to −0.24 dB OD. Thereafter, there were no recurrences of MEWDS during follow up.
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.