Vogt-Koyanagi-Harada syndrome (uveomeningoencephalitic syndrome)

Abstract

Purpose: To report a case of a 29-year-old man who was examined at the Eye Clinic of Central Military University Hospital Prague for a severe headache and acute blurring of vision in both eyes diagnosed as incomplete Vogt-Koyanagi-Harada syndrome (VKH).

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

Results: A 29-year-old man presented with headache and decreased vision in his left eye (LE) for 2 days. Best-corrected visual acuity was 20/20 in both his eyes. Pupillary function, intraocular pressure, results of external segment examinations, and slit-lamp biomicroscopy were normal bilaterally. Right eye funduscopy was normal; in the LE, funduscopy revealed posterior pole exudative retinal detachments. Optical coherence tomography confirmed multiple serous (bullous) retinal detachments and showed thickening of the posterior choroid also revealed by orbital ultrasound. Fluorescein angiography showed angiographic features typical of VKH disease: disseminated spotted choroidal hyperfluorescence and choroidal multifocal hypofluorescence, multifocal profuse leakage in the retina with late phase pooling, serous retinal detachment, and optic disc hyperfluorescence. All serologic tests for the diagnosis of infectious pathologies were negative. Except for severe headache, the patient did not have any neurologic, integumentary, or auditory manifestations. Immediate high-dose systemic corticosteroid treatment was given.

Conclusions: Despite prompt diagnosis, prompt aggressive systemic therapy, and a rapid response in this case, progressive pigmentary changes of retinal pigment epithelium developed shortly thereafter.