Unilateral Vogt-Koyanagi-Harada Disease: A Clinical Case Report

Abstract

Purpose: To report a case of a 20-year-old female with decreased visual acuity (VA) in the left eye (LE).

Methods: This is a retrospective and descriptive case report based on data from clinical records, patient observation and analysis of diagnostic tests.

Results: A 20-year-old female presented with decreased VA in the LE for 3 days. Best-corrected visual acuity (BCVA) was 20/20 in the right eye (RE) and 20/40 in the LE. Pupillary function, intraocular pressure, results of external segment examinations and slit-lamp biomicroscopy were normal, bilaterally. RE fundoscopy was normal, and in the LE it revealed papillitis and posterior pole exudative retinal detachment. Optical coherence tomography (OCT) confirmed the macular serous retinal detachment and showed thickening of the posterior choroid also revealed by orbital ultrasound and magnetic resonance imaging (MRI). Fluorescein angiography showed angiographic features typical of Vogt-Koyanagi-Harada (VKH) disease: disseminated spotted choroidal hyperfluorescence and choroidal multifocal hypofluorescence, multifocal profuse leakage in the retina with pooling, serous retinal detachment and optic disc hyperfluorescence. Serological testing for the diagnosis of infectious pathologies was negative, and the review of systems was normal. The patient received systemic steroids and cyclosporine. LE BCVA improved up to 20/20 at 18 months after the diagnosis, with complete reabsorption of subretinal fluid and normal retinal and choroidal thickness by OCT.

Conclusion: Despite the unilateral involvement, the clinical and angiographic features were typical of VKH disease, and ophthalmologists should be aware to recognize this rare clinical variant of the disease.

Keywords: Corticosteroid therapy; Cyclosporine; Fundus fluorescein angiography; Unilateral Vogt-Koyanagi-Harada disease.

Fig. 1

Retinography and OCT of the LE at presentation, revealing papillitis and posterior pole exudative retinal detachment. Retinal thickness was 388 μm, and there was thickening of the posterior choroid.

Fig. 2

FA of the LE at presentation (left) revealing focal areas of delayed choroidal perfusion and multifocal areas of pinpoint leakage in the early arteriovenous phase (top left), with pooling and leakage of dye in the subretinal space in the late stage of the angiogram (bottom left). Brain MRI showing a focal thickening of the uveal tract of 3-4 mm in the LE (right).

Fig. 3

OCT of the LE 1 week after treatment (top left) and 18 months after initial clinical presentation (bottom left), showing a decreased retinal and posterior choroidal thickness, with a ‘sunset glow’ fundus appearance at 18 months (right) in comparison to a normal fundus appearance in the RE (middle).