Hayreh S S
Dev Ophthalmol. 1985;10:40-77.
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.
自1971年以来,一种以全葡萄膜炎、视网膜血管炎、视网膜坏死(常继发视网膜脱离)和频繁视神经萎缩为特征的急性眼部疾病已被报道,曾有74例患者(103只眼)被赋予不同的病名。我又报告了4例。本文基于对78例患者107只眼的严格审查,详细描述了这种独特眼部疾病实体的临床特征、发病机制和术语。现有的临床和组织病理学证据有力地表明,其基本病理是虹膜、脉络膜、视网膜和视神经乳头的急性血管炎,导致脉络膜、视网膜和视神经乳头的血管闭塞;因此,临床所见除全葡萄膜炎外,还代表这些组织的急性缺血性病变。从现有证据来看,将这种病症称为“急性眼部全血管炎综合征”似乎比“急性视网膜坏死综合征”更合适,因为视网膜坏死只是该疾病众多病变之一。全血管炎的病因仍然是个谜。