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 54-year-old Chinese man presented in the clinic for a routine two-year ophthalmic examination. Past medical history is PNH. The patient was diagnosed with PNH at the age of 22. He is on Eculizumab (anti-complement factor 5 antibody) infusion which he started in 2007.\nHe was asymptomatic with best-corrected visual acuity of 20/40 on the right eye (spherical equivalent is +0.5), and 20/20 on the left eye (spherical equivalent is +0.25). Slit-lamp examination and intraocular pressures were normal. Posterior segment examination revealed subtle pigmentary changes temporal to the macula in both eyes (). Enhanced depth imaging-OCT (EDI-OCT) of the macula was obtained and the entire posterior pole was also scanned. The OCT-detected multiple FCEs corresponded with the retinal pigmentary changes by infrared fundus photo in OCT (). There was no FCE involving the foveal region. EDI-OCT showed the retinal pigment epithelium (RPE) layer complied with the contour of the choroidal excavation. And the outer nuclear layers (ONLs) conformed to the contour of the RPE within the excavation (). There was no RPE detachment and no separation between the photoreceptor layers and the RPE. One area of ONL in proximity to the excavation demonstrated hyperreflectivity, similar to the reflectivity of the adjacent outer plexiform layer (OPL) ( OD1 and OS2). The inner retinal layers from the OPL to the retinal nerve fiber layer were essentially undisturbed. Choriocapillaris thinning in the site of excavation was adjacent to large blood vessels as compared with the normal surrounding choroid layers without FCE ( OD2). The sclerochoroidal junction was smooth and undisturbed without staphylomatous changes.\nOn FA, the FCE appeared to have normal fluorescence without leakage. No sign of neovascularization was found in the inner and subretinal space.\nFAF photos of both eyes showed irregular hyper and hypoautofluorescence in the macular area, which suggested some RPE loss or disturbance, which is corresponding to the locations of FCEs ().\nHumphrey Visual field testing in both eyes was performed twice. First was 24-2 Sita fast and the second was 10-2 Sita fast. But both of the visual field results were not reliable because of a high fixation loss.\nThe patient was observed without any intervention. The patient was followed up in 6 months with no changes of the symptoms, visual acuity, and the fundus exam.