Anterior Ischemic Optic Neuropathy Associated with Vogt-Koyanagi-Harada Disease

Background: Optic disc swelling is a common finding associated with Vogt-Koyanagi-Harada disease (VKH); however, visual field loss from optic disc involvement is uncommon. This reports report presents recent findings regarding unusual patients with visual field defects from optic disc involvement, thus suggesting the presence of anterior ischemic optic neuropathy (AION) in the acute phase of VKH.

Methods: Observational case series. A consecutive series of 52 patients with VKH (6 complete VKH, 46 incomplete VKH) was reviewed. Fifteen patients in this series had optic disc swelling, and six of them developed irreversible visual field defects in the acute phase of VKH. The clinical features of these six patients were analyzed.

Results: The patients with associated visual field loss were four males and two females between 54 to 79 years of age. They had bilateral panuveitis associated with meningismus. All of the patients had bilateral optic disc swelling and fluorescein angiography showed both a filling delay and late leakage of the optic disc. Visual field examinations revealed various degrees of visual field defects in 11 eyes. They were treated with high-dose corticosteroid therapy, and several weeks later, both the uveal inflammation and optic disc swelling disappeared. The visual fields showed some improvement as the retinal detachments and disc swelling resolved, but the visual field defects remained in ten eyes. The small and localized visual field defects were asymptomatic. Subsequently, optic disc pallor developed in nine eyes and retinal nerve fiber layer thickness, measured by optical coherence tomography, was decreased in six eyes. The fundus gradually showed various degrees of hypopigmentation, but did not show any chorioretinal atrophy causing visual field loss. Four patients had risk factors for AION, including diabetes mellitus or a relatively small optic nerve head.

Conclusions: In this consecutive series of patients with VKH, 15 out of 52 patients were found to have disc swelling, and six patients, who were mostly elderly, had associated visual field loss, which is probably due to AION associated with VKH.