Vogt-Koyanagi-Harada syndrome in children

Purpose: To describe the clinical presentation and natural history of Vogt-Koyanagi-Harada (VKH) syndrome in children, an often unrecognized cause of pediatric uveitis. Methods: We performed a clinic-based cross-sectional survey and retrospective review of pediatric patients with VHK seen in the uveitis clinic at the Aravind Eye Hospital, Madurai, India, between January 1993 and December 1995. Results: A total of 98 patients with VKH syndrome were seen in the uveitis clinic at the Aravind Eye Hospital during the three years covered by the survey, of whom three (about 3%, with an approximate incidence of I case/year) were children 16 years of age or younger. Initial symptoms included eye pain, redness, and blurred vision in all three patients. Headache and dysacusis accompanied the onset of visual symptoms in one patient. Each patient eventually developed areas of vitiligo and poliosis, although these signs were not present at the time of presentation in any of our patients. Signs of active uveitis included anterior chamber and vitreous inflammation, optic disc edema, exudative retinal detachments, and Dalen-Fuchs'-like nodules. All three patients developed cataracts, two developed retinal pigment epithelium atrophy, and one developed glaucoma. Each patient responded well, with good visual recovery, to topical and/or systemic corticosteriods. Conclusions: Although frequently unrecognized, VKH syndrome may affect children. As with adults, visual loss occurs most typically as the result of cataract, glaucoma, or retinal pigment epithelium atrophy.