First Author: A.Quintero SPAIN
Co Author(s): I. Baquero Aranda J. Yanguas Lucena
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Vogt–Koyanagi-Harada (VKH) disease is an inflammatory autoimmune systemic disorder that presents with panuveitis and iridocyclitis, serous retinal detachments, difusse choroidal thickening and optic disc hyperemia. Neurologic and cutaneous manifestations may be associated. The ocular manifestations are typically bilateral, but an asymmetric involvement is usually present. There are few reports of unilateral disease and some of them represent delayed forms of bilateral VKH. We hereby report a probable case of unilateral VKH disease.
This patient is followed up in our medical retina clinic at Hospital Universitario Virgen de la Victoria, Málaga, Spain.
Extensive review of clinical and photographical documentation of the patient, as well as a comprehensive review of the available literature.
A 35 year-old female presented to our emergency department complaining of decreased visual acuity (VA) in the right eye(OD) for 2 days with associated frontal headache lasting 2 weeks. No systemic or ophthalmologic background were present. VA was 20/60 in OD and 20/20 in the left eye (OS). Neurological examination was normal. Pupillary function, intraocular pressure and biomicroscopic examination were unremarkable. Funduscopy was normal in OS, but showed papillitis, serous retinal detachment superior and inferior peripapillar and maculopapillar bundle in OD. Optical coherence tomography (OCT) confirmed a macular multilobular serous retinal detachment (retinal thickness 370 µm) with thickening of the posterior choroid in OD. Fluorescein angiography (FAG) revealed angiographic features typical of VKH disease. Markers of inflammation, autoimmunity and infectious screening were negative. The patient was initiated on a tapering dose of oral prednisone starting with 1mg/kg/day. Two months later, VA in OD was 20/20, funduscopy revealed no signs of activity and OCT showed resolution of retinal detachment. AFG revealed hyperfluorescent areas at the retinal pigment epithelium in maculopapillar bundle due to fluorescein window effect.
Current diagnostic criteria for VKH disease require the presence of bilateral ocular involvement; however, unilateral or delayed involvement of the fellow eye can occur sporadically. The diagnosis of VKH disease is essentially clinical and angiographic. Tomographic images played an important role in our case, allowing us to clarify the morphologic background of serous retinal detachment typical of VKH and avoiding the systemic risks associated with the performance of AFG. Furthermore, it can help us in the differential diagnosis process of other causes of neuroepithelium detachment, such as serous choroidopathy or exudative macular degeneration. Our patient continues with maintenance oral prednisone and is currently followed up closely in our clinic as a possible delayed contralateral involvement, if it was to occur, would need to be detected and treated as early as possible.