A 27-year-old woman with mild myopia presented with blurred vision and photopsia of the right eye for 2 weeks. She had no particular medical or ocular histories.\nThe patient’s best-corrected visual acuity (BCVA) was 1.0 OD (oculus dexter) and 1.5 OS (oculus sinister). Relative afferent pupillary defect was negative. There were no abnormal ocular findings OS. Slit-lamp examination revealed no inflammation in the anterior chamber or vitreous. Funduscopic examination showed multiple, faint, subretinal white dots at the posterior pole to the equator and macular granular change (, arrowheads). In addition, retinal vascular sheathing was observed at the midperiphery (, arrows). Fluorescein angiography (FA) showed hyperfluorescence continuing from the initial phase corresponding to the white dots (, arrowheads) and the staining of the optic disk. The retinal sheathing sites showed vascular staining with leakages in the late-phase OD (, arrows). Indocyanine green angiography showed no abnormal appearance in the initial phase and multiple hypofluorescence at the wider area than the white dots (, arrowheads). Enhanced depth imaging optical coherence tomography (EDI-OCT) revealed the discontinuity of the ellipsoid zone at macular area OD (, arrows) and that the subfoveal choroidal thickness (SCT) was 295 µm. Humphrey perimetry (30-2 Swedish threshold interactive algorithm standard test) showed a mild blind spot enlargement OU (oculus uterque) (mean deviation [MD] value: −1.83 dB OD and −1.08 dB OS). Multifocal electroretinography (mfERG) showed entirely decreased amplitude, except normal central amplitude OD, and mildly decreased amplitude at the retinal site that corresponded to the peripapillary area OS. Infection screening, including tuberculosis, syphilis, human T-cell leukemia virus type 1, and toxoplasmosis, showed negative results on QuantiFERON and serological testing or with antibodies for these agents. The chest X-ray was normal and serum angiotensin-converting enzyme was within normal limit. The patient received a diagnosis of MEWDS OD and was followed up with no treatment. One month after the first visit, the white dots and retinal vascular sheathing spontaneously resolved. After 3 months, the ellipsoid zone at the macula restored and the SCT decreased to 237 µm, together with increased BCVA of 1.5 OD (). On Humphrey perimetry, the MD value improved to −0.28 dB OD and −0.69 dB OS.
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.