A 32-year-old female presented with decreased central vision in the right eye to our tertiary eye center (Khatam Eye Hospital) affiliated to Mashhad University of Medical Sciences. Institutional review board approval was obtained for this case report. Complete ophthalmic examination was done. BCVA was 20/100 on the right and 20/20 on the left one. Ophthalmoscopy revealed bilateral multifocal yellow-orange elevated choroidal masses with distinct borders at the posterior pole. The left eye was conspicuous in its macular tumor as well as juxtapapillary foci, indicative of multifocal nature of the lesion ().\nFundus autofluorescence (FAF) images revealed the calcified parts of the tumor to be isoautofluorescent (AF) or mild hyper-AF on the left, whereas the lesion was variable (hypo-hyper AF) on the right side, possibly due to RPE changes, which in turn stemmed from decalcification, CNV, and subretinal fluid (SRF) (). Fluorescein angiography (FAG) demonstrated variable hyperfluorescence of the tumor on both sides as well as CNV in the right eye (). Indocyanine green angiography (ICG), in early to mid-phase, showed the well-defined tumor to be hypofluorescent with CNV on the right side ().\nB-scan ultrasonography (NIDEK Echo scan US-4000, Gamagori, Japan) indicated hyperechogenicity of the tumor on both sides. Enhanced depth imaging optical coherence tomography (EDI-OCT) (Spectralis HRA + OCT, Heidelberg Engineering, Heidelberg, Germany) disclosed the thinning of choriocapillaries as well as large choroidal vessels, which were invisible in the calcified parts. Outer retinal abnormalities were attributed to CNV in the right eye. These regions also showed the disturbance of the outer retinal layers whereas the inner retina turned out to be entirely intact. The tumor displayed smooth undulating surface with horizontal layered hyperreflective lines in the tumor (). Orbital computerized tomography (CT) scan showed hyperdense plaques, with bone like homogeneity in the posterior pole. Two plaques were seen in the left eye ().\nThe final diagnosis of bilateral multifocal choroidal osteoma in association with CNV in the right eye was made based on multimodal imaging findings. The decalcified portion of tumor on the right was yellowish-white in color.\nThe safe conclusion pertaining to the above details was suggestive of the trend whereby tumor calcification and CNV had led to visual loss on the right eye. Thus, the patient received injections of intravitreal bevacizumab (Avastin, Genentech Inc., San Francisco, CA) in the right eye, which subsequently improved the BCVA to 20/40. This BCVA sustained during nine months of follow-up.
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. ).