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 54-year-old Japanese woman presented with bilateral blurring of vision associated with headache that had started 2 weeks earlier. Her general family medical history and her personal medical history, including her history of ocular disease, were unremarkable. The best-corrected visual acuity on presentation was 0.6 in the right eye and 0.1 in the left eye. The intraocular pressure was normal in both eyes. Slit-lamp biomicroscopy showed bilateral anterior chamber cells and posterior synechiae, and fundus examination showed bilateral disc swelling with exudative retinal detachment (fig. ).\nFluorescein angiography detected bilateral multifocal leakage with pooling of dye in the subretinal space in the late phase (fig. ). Indocyanine green angiography showed patches of hyperfluorescence and hypofluorescent spots bilaterally (fig. ). A spectral-domain OCT (Cirrus HD-OCT 4000; Carl Zeiss Meditec Inc.) image of the eyes showed the bilateral presence of subretinal fluid and pigment epithelial detachment. Moreover, only in the left eye was unilateral choroidal excavation detected under the fovea, and the contour of the retinal pigment epithelium appeared to be conforming to the shape of the fibrin (fig. ).\nSystemic workup revealed positive HLA-DR4 as well as cerebrospinal fluid pleocytosis. A diagnosis of VKH was reached soon afterwards. Thus, our patient was treated with intravenous pulse methylprednisolone followed by oral prednisolone (initial dose of 40 mg/day) with tapering over a course of 6 months. Treatment successfully resolved the VKH symptoms, and serial OCT showed a gradual reduction in size and a subsequent disappearance of the subretinal fibrin and fluid (fig. ). However, 1 month after having started treatment, the eye was left with an area of choroidal excavation at the fovea despite a normal contour in the right eye (fig. ). There was a good recovery of visual acuity, with 1.2 and 0.9 in the right and the left eye, respectively. Presently, the patient is being followed-up without complications and medications.