ABSTRACT
PURPOSE: In the present study, a case of repeated intravitreal dexamethasone implantation for a suspected idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome associated with recurrent exudative retinal detachment and macular edema is reported. CASE SUMMARY: A 39-year-old female who underwent steroid pulse therapy due to Vogt-Koyanagi-Harada disease in the left eye was referred for exudative retinal detachment and macular edema. Best corrected visual acuity (BCVA) was 1.0 in the right eye and 0.5 in the left eye. Cystoid macular edema combined with serous retinal detachment was observed on spectral-domain optical coherence tomography. Fluorescein angiography revealed neovascularization and multiple macroaneurysms with fluorescein leakage in the left peripapillary area. Severe peripheral capillary non-perfusion and fluorescein leakage were also observed in both eyes. Intravitreal dexamethasone implantation was performed in the left eye and macular edema showed wax-and-wane pattern. No edema was observed after 4 additional dexamethasone implantations, however, preretinal hemorrhage occurred in the peripapillary area during treatment. Seventeen months after initiation of treatment, BCVA was 0.6 in the left eye and dry macula was maintained. CONCLUSIONS: Repeated intravitreal dexamethasone implantation was effective for recurrent macular edema in a patient suspected with IRVAN syndrome.