A 37-year-old woman with moderate myopia presented with complaints of blurred vision and photopsia in her right eye for 1 week. She had no antecedent flu-like illness. The patient’s medical and family histories were unremarkable. Best-corrected visual acuity (BCVA) with a Japanese standard Landolt visual acuity chart was 0.09 OD and 1.2 OS. Left eye showed normal appearance. Slit lamp examination showed 2+ cells in the anterior vitreous OD. Funduscopic examination showed foveal granularity and multiple white dots (Figure A, white arrows) extending from the posterior pole to the retinal midperiphery. Some white dots in the temporal fovea were relatively large (Figure A, yellow arrows). Fluorescein angiography (FA) showed hyperfluorescent spots (Figure B, arrows) from the initial phase, corresponding to the white dots, and the staining of retinal veins and the optic disc in the late phase. ICGA was normal in the early phase, but in the late phase numerous hypofluorescent spots were scattered over a wider area with and without white dots (Figure C). Goldmann perimetry showed a blind spot enlargement and a small central scotoma OD. Additionally, EDI-OCT showed hyper-reflective lesions in the ganglion cell layer (GCL) (Figure A, black arrowheads), the loss of photoreceptor inner segment/outer segment junction (IS/OS) line, detachment between the RPE and Bruch’s membrane, moderately reflective, dome-shaped focal lesions within the photoreceptor layer (Figures A and A; white arrowheads), and locally thickened choroid compared with neighboring areas, corresponding to the large white dots in the temporal fovea and inferotemporal perifovea with pigment epithelium detachment. Multiple ruptures of the RPE and Bruch’s membrane were apparently observed in the temporal lesion (Figure A, red and yellow arrowheads), whereas Bruch’s membrane underlying the damaged RPE was found to be continuous in the inferotemporal lesion (Figure A, red and yellow arrowheads). Choroidal thickness at the fovea and the adjacent temporal lesion was 243 and 330 μm OD, respectively (Figure A). The patient received a diagnosis of MEWDS OD.\nThe white dots spontaneously resolved 1 month after the initial visit, with visual acuity improving to 0.2 OD. The pigment epithelium detachment and abnormal GCL hyper-reflective lesions completely resolved and the photoreceptor-layer focal lesions markedly decreased in the temporal fovea (Figure B). Interestingly, a conforming FCE, without an intervening hyporeflective space between the photoreceptor tips and the RPE [], developed in the same region (Figure B). Choroidal thickness at the fovea and the FCE lesion decreased to 210 and 140 μm, respectively (Figure B). An abnormal hyper-reflective lesion also appeared in the choroid below the FCE (Figure B, arrow) and increased in size 2 months after the initial visit.\nThree months after the initial visit, BCVA improved to 0.4 and scars formed in the temporal and inferotemporal lesions (Figure D, yellow arrows). On ICGA, almost all hypofluorescent lesions disappeared, but the scarred sites exhibited hypofluorescent spots from the initial phase. On EDI-OCT images, the focal lesions in the photoreceptor layer almost resolved (Figures C and B), but cystoid changes emerged in inner retinal layers together with marked thinning of outer retinal layers (Figure C). The choroid below the temporal and inferotemporal scars developed massive hyper-reflective lesions (Figures C and B; yellow arrows), and the subfoveal choroidal thickness further decreased to 181 μm (Figure C). The inferotemporal perifoveal lesion did not develop any apparent sign of FCE (Figure B), in contrast to the temporal foveal lesion (Figure B,C). Twelve months after the initial visit, BCVA was 0.7 OD and the disrupted IS/OS line at the fovea partially improved. No recurrence of white dots or choroidal neovascularization had ever been observed.
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.