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 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.