Vogt-Koyanagi-Harada Syndrome

A systemic inflammatory disease with probable immunogenetic predisposition that affects the eye (panuveitis) leading to rapid loss of vision. It is also associated to a characteristic spectrum of nonophthalmic features involving particularly the skin, hair (alopecia), and ear. Meningitis and raised intracranial pressure have also been reported.

Alopecia-Poliosis-Uveitis-Vitiligo-Deafness-Cutaneous-Uveo-Oto Syndrome; Harada Syndrome; Harada Disease; Vogt-Koyanagi Syndrome; Yugé Syndrome; Oculocutaneous Syndrome; Uveocutaneous Syndrome; Uveitis-Vitiligo-Alopecia-Poliosis Syndrome; Uveomeningeal Syndrome; Uveomeningitis Syndrome; Uveomeningoencephalitis; Uveoencephalitis.

First described in 1906 by Alfred Vogt, Swiss ophthalmologist.

Unknown but more common in darker-pigmented races, particularly Orientals and American Indians.

Unknown but strong association with HLA-DR histocompatibility antigens.

Immunocytologic findings are compatible with a T-cell-mediated autoimmune reaction to uveal and dermal melanocytes.

Requires three of the following features: bilateral iridocyclitis, posterior uveitis (including serous retinal detachment or sunset glow fundus), central nervous system manifestations, and cutaneous manifestations.

Patients are often of short stature. In addition to uveitis, ophthalmic features can include visual loss, retinal detachment, cataract, and glaucoma. Other features include the central nervous system and cutaneous origin, including deafness (sensorineural), headache, tinnitus, dysacousis, and meningism associated with cerebrospinal fluid pleocytosis. Alopecia, premature graying of hair, and vitiligo are also observed. Onset is generally in postadolescent life, but two case reports exist in children, the youngest being 4 years old. The condition is treated with high-dose steroids.

Complete medical history and physical examination pertaining to central nervous system involvement and the use of steroids are important. Evaluate adrenal function (clinical, electrolytes, glycemia).

Strict asepsis is needed because of immunodeficiency induced by repeated steroid treatment.

Preoperative stress dose of steroids might be required. Atropine and all drugs that can induce glaucoma should be avoided. Succinylcholine should probably be avoided in case of retinal detachment and because of its action on intraocular pressure. Consider benefit of using aminoglycosides in cases of incomplete deafness.