Vogt-Koyanagi-Harada syndrome

Vogt-Koyanagi-Harada syndrome is a multisystem disorder characterized by granulomatous panuveitis with exudative retinal detachments that is often associated with neurologic and cutaneous manifestations.

Epidemiology

Vogt-Koyanagi-Harada usually affected those of Asian, Middle Eastern, Asian Indian, Native American or Hispanic ethnicity . Patients are often typically middle-aged (20 to 50 years old) at presentation .

Clinical presentation

Patients with Vogt-Koyanagi-Harada disease usually initially present to an ophthalmologist for ocular problems, including sudden loss of vision, ocular pain and photophobia. Hearing disturbances and dizziness may be present. After weeks or months, most patients notice cutaneous signs (e.g. hair loss, poliosis, vitiligo).

Pathology

It is considered an inflammatory syndrome occurring probably as a result of an autoimmune mechanism, influenced by genetic factors, and appears to be directed against melanocytes.

Radiographic features

Ocular ultrasound

B-mode ocular ultrasound features of Vogt-Koyanagi-Harada syndrome are:

• low to medium reflective thickening of the posterior choroid
• serous retinal detachments
• mild thickening of the sclera and/or episclera adjacent to areas of choroidal thickening
• vitreous opacities
• subretinal septations may be seen

MRI

MR imaging may detect early CNS involvement by Vogt-Koyanagi-Harada disease before the onset of neurologic symptoms which include:

• typical bilaterality of ocular findings
• scattered periventricular white matter lesions on T2-weighted imaging/FLAIR, and pachymeningeal enhancement.
• bilateral contrast enhancement of the choroid is seen along diffuse choroidal thickening with scleral sparing and retinal detachment