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 55-year-old man (subject 1) presented with bilateral metamorphopsia, with decimal visual acuity being 0.8 in the right eye and 1.2 in the left. The clinical diagnosis of VKH was made based on bilateral panuveitis and multifocal exudative retinal detachments at the posterior poles (). High-dose corticosteroid therapy with gradual tapering was initiated 3 days after presentation. The serous detachments entirely resolved within 9 weeks and visual acuity in the right eye improved to 1.2.\nSerial spectral-domain OCT (SD-OCT) images obtained at the preuveitic, uveitic, and posttreatment phase are shown in . SD-OCT obtained 1 year before the onset of VKH due to symptoms of a floater and visual disturbance demonstrated a dome-shaped posterior protrusion of the RPE and outer retinal layers into the choroidal cavity (ie, conforming FCE) at the fovea in the right eye. SD-OCT at the uveitic phase identified multiple bilateral sensory retinal detachments, with the FCE now involving only the RPE (ie, nonconforming FCE). Nine weeks after the treatment, the nonconforming FCE returned into a conforming pattern.\nThe FCE pattern and its alteration during treatment of the case with VKH were compared to those of four subjects with FCE associated with other chorioretinal conditions (). This comparison group consisted of two patients with age-related macular degeneration (AMD; subjects 2 and 3), one subject with a macular hole (MH; subject 4), and one asymptomatic individual (subject 5). Treatment with intravitreal ranibizumab injections and vitrectomy had been performed in the patients with AMD and MH, respectively. A conforming FCE was observed in three patients (subjects 2, 3, and 5) and pattern alteration from nonconforming to conforming FCE was found after treatment in one subject (subject 4). In the two subjects with AMD, a hyperreflective material around the FCE was absorbed, resulting in a well-demarcated conforming FCE after treatment.