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 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.