EALES DISEASE: Clinical Features, Pathophysiology, Etiopathogenesis

Abstract

Eales disease is a clinical entity, classically presenting with repeated vitreous hemorrhage in young adult men. The underlying pathology is retinal phlebitis, predominantly in the peripheral fundus. The clinical manifestations are those of inflammatory retinal vein occlusion, usually multiple branch retinal vein occlusion (BRVO), but sometimes central retinal vein occlusion (CRVO). Phlebitis may or may not be associated with choroiditis, pars planitis, iridocyclitis, arteritis, or papillitis. There seem to be multiple causes with several possible pathologic and immunopathologic processes, which could explain the variability in the acuteness of onset and course of disease. Although in some cases the possible cause can be identified by association, in most cases the cause remains obscure. The management of Eales disease can be highly satisfactory when appropriate treatment is used at the appropriate stage of disease development. Proper management requires a proper understanding of the pathophysiology of Eales disease. Henry Eales (1880–1882) 23,24 was the first to describe a clinical syndrome of recurrent retinal and vitreous hemorrhages in apparently healthy young men. He had not, however, noticed any association with vasculitis or phlebitis. Wadsworth (1887) 73 associated perivasculitis with vitreous hemorrhage. Axenfeld and Stock (1909–1911) 5 were the ones who associated periphlebitis with Eales disease. Duke-Elder et al 22 consider Eales disease to be a clinical entity that is a manifestation of retinal perivasculitis of different causes. There is considerable confusion in terminology and nomenclature involving the pathology of Eales disease. It has been variously described as retinal perivasculitis, 16,43,44,73 obliterative vasculopathy, 34 and periphlebitis. 28 It is clear, however, that Eales disease is a clinical manifestation of inflammatory retinal vein occlusion or phlebitis. The associated arteritis in some cases is not relevant to the development of typical Eales disease. Because retinal vein inflammation can be in the form of massive exudation, obliterating the lumen completely or mildly cuffing vein segments with minimal changes in the venous lumen, it would be inappropriate to describe all cases as periphlebitis. It may be adequate to describe the basic pathology of Eales disease as simply retinal phlebitis, rather than blundering into the realm of pathology.