Vogt-Koyanagi-Harada Syndrome

At a glance

A systemic noninfectious panuveitis in populations with pigmented skin 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.

Synonyms

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.

History

First described in 1906 by Alfred Vogt, Swiss ophthalmologist.

Incidence

Unknown. More common in darker-pigmented populations.

Genetic inheritance

Unknown but strong association with HLADR histocompatibility antigens.

Pathophysiology

Systemic autoimmune disease involving melanocyte rich tissues (eye, inner ear, meninges, hair, and skin). The autoimmune process is directed against antigens associated with melanocytes and is triggered by a viral infection, particularly in patients carrying the allele HLA-DRB1*0405.

Diagnosis

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.

Clinical aspects

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.

Precautions before anesthesia

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).

Anesthetic considerations

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

Pharmacological implications

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.

References