So-called 'acute retinal necrosis syndrome'--an acute ocular panvasculitis syndrome

An acute ocular disorder, characterized by panuveitis, retinal vasculitis, retinal necrosis (with frequent secondary retinal detachment) and frequent optic atrophy, has been reported since 1971 under different eponyms in 74 patients (103 eyes). I report 4 more cases. This paper presents, based on a critical review of 107 eyes in 78 patients, a detailed clinical description, pathogenesis and terminology of this distinct ocular disease entity. The available clinical and histopathological evidence very strongly indicates that the basic pathology is acute vasculitis of the iris, choroid, retina and optic nerve head, producing vascular occlusion in the choroid, retina and optic nerve head; the clinical findings thus represent acute ischemic lesions of those tissues, in addition to panuveitis. From the available evidence, it seems more appropriate to call this condition 'acute ocular panvasculitis syndrome' rather than 'acute retinal necrosis syndrome' since retinal necrosis is only one of a number of lesions seen in this disease. The etiology of the panvasculitis still remains a mystery.