Macular edema in chronic Vogt-Koyanagi-Harada syndrome

Background: Macular edema is relatively rare in the Vogt-Koyanagi-Harada (VKH) syndrome, in contrast to many other syndromes of chronic intraocular inflammation, in which macular edema is relatively common. Five eyes of three patients with chronic VKH syndrome and macular edema are described. Methods: The authors retrospectively reviewed the clinical and fluorescein angiographic records of 27 patients with chronic VKH syndrome (duration of disease more than 3 months) and identified three patients (five eyes) who had macular edema. Fluorescein angiography was performed using a scanning laser ophthalmoscope. Results: These patients had either unusually severe and protracted inflammation (3 eyes) or the presence of an epiretinal membrane (2 eyes). Fluorescein angiography demonstrated prominent perifoveal fluorescein leakage with pooling of fluorescein within perifoveal cystoid spaces. Treatment with sub-Tenon's triamcinolone injections resulted in improvement of visual acuity by two or more Snellen lines in four of the five eyes. Conclusion: Macular edema is an uncommon but treatable cause of late loss of vision in patients with the VKH syndrome, and may be the result of leakage from perifoveal retinal capillaries or leakage of fluid at the level of the retinal pigment epithelium.