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Atypical presentation of Vogt-Koyanagi-Harada syndrome (VKH) in infancy

Poster Details

First Author: I.Yago SPAIN

Co Author(s):    J.A. Caballero Agudo   A. PiƱero   J.M. Marin            0   0 0   0 0   0 0   0 0

Abstract Details


To describe the atypical presentation and course of a Vogt-Koyanagi-Harada syndrome (VKH) in an 8-yer-old boy.


VKH is a multisystem disorder, characterized by the T-cell-mediated autoimmune process directed against melanocytic antigens in different organs. The ocular involvement presents in three different stages (acute, subacute and chronic) starting with panuveitis associated with exudative retinal detachment. Isolated anterior uveitis typically appears in the delayed phase of the disease.


An 8-year-old boy was referred to our clinic for bilateral redness, pain and photophobia in both eyes (OU).


His ocular examination revealed best-corrected visual acuities (BCVA) of 20/30 in OU. Anterior segment examination revealed corneal mutton-fat keratic precipitates and moderate anterior chamber cells in OU. The funduscopy revealed a mild hyperaemic disc, with no other relevant finding. Two weeks after starting topical treatment his BCVA was found to be 20/70 OU. Dilated fundus examination disclosed retinal serous detachment, severe vitritis and optic disc oedema in OU. In this case, according to the history and eye examinations, the diagnostic entity considered was VKH. The patient was started on oral and topical steroids. The patient did not improved due to non-compliance to treatment and he was hospitalized for intravenous steroid treatment. The patient was maintained on oral steroids and methotrexate, with vision improvement to 20/25 OU. Six months after the initial symptoms the patient showed a BCVA of 15/25 and 20/25 in his right (OD) and left eye (OS), respectively. Anterior segment exam showed 2+ tyndall bilaterally. Intraocular pressures were 26 OD and 23 OS. Fundus examination showed a sunset-glow fundus with macular RPE clumping and Dallen-Fuch nodules. Treatment was switched to adalimumab, topical corticosteroids and timolol that controlled the inflammation.


VKH has been rarely reported in children. Atypical presentation in children has been described with anterior granulomatous uveitis with no chorioretinal involvement at presentation. Previous reports also confirm de more aggressive course of the disease in children and the importance of an early treatment to avoid RPE changes in the chronic phase.

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