Isolated Posterior Atypical Presentation of Vogt-Koyanagi-Harada Syndrome in a Young Girl: A Rare Case Report

Abstract

Background: Vogt-Koyanagi-Harada (VKH) disease is a rare autoimmune disorder affecting melanocyte-rich tissues, including the uvea, meninges, skin, and inner ear. Posterior involvement is typical, yet isolated posterior forms are exceptionally rare and diagnostically challenging. We present a pediatric case of atypical isolated posterior VKH successfully managed with corticosteroid therapy. Case Presentation: A 15-year-old girl presented with sudden bilateral visual loss following diffuse headaches. Fundus examination revealed serous retinal detachments in both eyes. Optical coherence tomography (OCT) showed multilobulated subretinal fluid and disruption of outer retinal layers. Fluorescein angiography demonstrated multiple pinpoint hyperfluorescent leaks and dye pooling, while indocyanine green angiography revealed delayed choroidal perfusion. Laboratory and imaging workup excluded other causes such as posterior scleritis, infectious choroiditis, and central serous chorioretinopathy. She was treated with intravenous methylprednisolone followed by oral tapering, leading to marked improvement in vision and anatomical resolution. Conclusion: Isolated posterior atypical VKH is an uncommon presentation that may mimic several inflammatory or infectious choroidopathies. Early recognition and aggressive corticosteroid therapy are key to achieving favorable visual outcomes and preventing chronic evolution. Follow-up of patients with ocular VKH is long, even lifelong, because relapses and late complications can occur even several years after the onset of the disease.