A 50-year-old female patient presented to the Ophthalmology Department of the İstanbul University İstanbul Faculty of Medicine with an approximately 2-year history of gradual vision loss in her left eye. Despite progressively decreasing vision in her left eye over the course of 2 years, she had not previously consulted any doctor about the problem. There was nothing extraordinary in the patient’s medical or family history. Her vision was 1.0 (decimal) in the right eye and 0.05 in the left eye. Anterior segment examination was normal and intraocular pressure was 15 mmHg in the right eye and 16 mmHg in the left eye. Pigment epithelium changes were observed in both the right and left macula on fundoscopy (). OCT examination revealed extrafoveal inferonasal choroidal excavation in the right eye (), while in the left eye subfoveal focal choroidal excavation was observed, as well as separation of the retinal pigment epithelium (RPE) photoreceptor layer and subretinal fluid in the same area (). Central foveal thickness was 245 µm in the left eye. Fundus fluorescein angiography (FFA) and indocyanine green angiography (ICGA) revealed hyperfluorescence consistent with pigment epithelium window defect in the macula and temporal quadrant of the right eye, and hyperfluorescence starting in the early phase and increasing in the late phases in the left macula (). The patient was diagnosed with chronic CSCR and her left eye was treated with low-fluence photodynamic therapy (PDT) (25 j/cm2, 300 mW/cm2). The spot size was adjusted targeting the area of choroidal vascular hyperpermeability observed in the ICGA mid-phase from which the subretinal fluid originated.\nAt 1 month after PDT, the subretinal fluid had resolved and visual acuity was 0.3. After 18 months of follow-up, no changes were observed in the lesions in the right eye, visual acuity in the left eye as maintained and there was no recurrence of subretinal fluid. OCT at the final examination showed continuity of the RPE and photoreceptor layer in the area of focal choroidal excavation, and no subretinal fluid was observed (). On FFA, the hyperfluorescence due to RPE window defect was not evident () and there was no active leakage apparent on ICGA ().
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.