Background: Macular edema is relatively rare in the Vogt-Koyanagi-Hara
da (VKH) syndrome, in contrast to many other syndromes of chronic intr
aocular inflammation, in which macular edema is relatively common. Fiv
e eyes of three patients with chronic VKH syndrome and macular edema a
re described. Methods: The authors retrospectively reviewed the clinic
al and fluorescein angiographic records of 27 patients with chronic VK
H syndrome (duration of disease more than 3 months) and identified thr
ee patients (five eyes) who had macular edema. Fluorescein angiography
was performed using a scanning laser ophthalmoscope. Results: These p
atients had either unusually severe and protracted inflammation (3 eye
s) or the presence of an epiretinal membrane (2 eyes). Fluorescein ang
iography demonstrated prominent perifoveal fluorescein leakage with po
oling 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. Conclus
ion: 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 lea
kage from perifoveal retinal capillaries or leakage of fluid at the le
vel of the retinal pigment epithelium.