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 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.