A 24-year-old Asian man presented to our emergency department with a two-day history of blurred vision in both eyes, malaise, photophobia, vertigo, headache, and mild hearing loss. Best-corrected visual acuity (BCVA) was 20/200 in the right eye (RE) and 20/32 in the left eye (LE). Extraocular motility, intraocular pressure and pupillary light reflexes were within normal limits. The patient's past medical history was unremarkable; he was afebrile, and systemic blood pressure was 115/65 mm Hg. No signs of meningism or neck stiffness were noted.
Ocular examination revealed 2+ cells in the anterior chamber of the RE, 1+ cells in the anterior chamber of the LE, 1+ (bio-score) of inflammation in the vitreous cavity of both eyes (BE), bilateral optic nerve head hyperemia and multiple serous retinal detachments (SRD) in BE. Multimodal retinal imaging was performed (Figure 1 (Fig. 1)); FA showed multiple hyperfluorescent retinal pigment epithelium leaks in BE with dye pooling beneath SRD in the RE and normal retinal perfusion with no signs of vasculitis in either eye; ICGA showed persistent multiple hypofluorescent lesions in BE; OCT of the RE showed subfoveal serous macular detachment splitting of the photoreceptors' layers (also referred to as bacillary detachment). OCTA (Topcon DRI OCT Triton Plus, Tokyo, Japan) of the choriocapillaris of BE showed multiple areas of decreased flow that correlated with the ICGA hypofluorescent lesions. These imaging findings along with the clinical history were suggestive of VKH in the acute phase,.
The patient was started on immunosuppressive therapy consisting of a tapering course of oral prednisolone starting from 60 mg per day. The patient was closely observed, and during follow-up the presenting symptoms and BCVA steadily improved. Common infectious causes of posterior uveitis were excluded by performing extensive work-up including tuberculosis quantiFERON assay and Treponema serology that resulted negative. Therefore a steroid-sparing agent (cyclosporine A 100 mg twice a day) was given to prevent relapses of the disease. Three months after presentation, BCVA improved to 20/20 in both eyes. OCT revealed complete resolution of the retinal serous detachments and regression of the increased choroidal thickness. ICGA showed marked reduction in size and number of the hypofluorescent lesions with correspondent decrease of the OCTA flow void spots detected at presentation (Figure 2 (Fig. 2)). No signs of relapses were observed during 12 months of follow-up.