A 34-year-old healthy woman with bilateral choroidal osteoma complicated by CNV in the right eye for the two years presented with acute deterioration of visual acuity (VA) in the right eye. The findings of multimodal imaging for this case were reported and published in 2015, and there was no FCE in the right eye at that time.[\nBest corrected visual acuity (BCVA) of the right eye had decreased from 20/40 to 20/100, while that of the left eye was 20/20. Fundus examination of both eyes showed a yellow–orange lesion with sharp borders at the level of the choroid (Figure 1). A small retinal hemorrhage accompanied by subretinal fluid (SRF) was seen in the macular area of the right eye. Enhanced depth imaging OCT (EDI-OCT) (Spectralis HRA + OCT, Heidelberg Engineering, Heidelberg, Germany) showed that the inner retina was intact, although the outer retinal layers had outward displacement due to active CNV along with SRF and a wedge-shaped choroidal depression in the right eye. The choroidal excavation involved the retinal pigment epithelium (RPE) and outer retinal layers up to the outer plexiform layer. The integrity of the inner retinal layers was maintained, and an optically clear space was present between the neurosensory retina and RPE. EDI-OCT showed thinning of the choriocapillaris and large choroidal vessels and a sponge-like choroidal lesion (Figure 2). Fluorescein angiography (FAG) demonstrated mild hyperfluorescence in the choroidal osteoma due to window defect, as well as juxtafoveal hyperfluorescence corresponding to active CNV in the right eye (Figure 3), which was better delineated on OCT angiography (Figure 4).\nThus, the diagnosis was reactivation of CNV associated with the development of FCE in the right eye in a known case of choroidal osteoma. The patient received two consecutive intravitreal bevacizumab (Avastin, Genentech Inc., San Francisco, CA) injections, which resulted in improvement of BCVA to 20/40. OCT showed disappearance of SRF and resolution of the disturbances in the outer retinal layers. FCE was more clearly visible and associated with severe choroidal thinning after quiescence of the CNV (Figure 2c). BCVA was maintained in both eyes during a follow-up period of one year.
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.