A Case of Vortex Vein Aplasia and Recurrent Idiopathic Uveal Effusion Syndrome

“Uveal effusion” was first described in a nanophthalmic patient by Schepens and Brockhurst in 1963 [1]. Gass and Jallow [2] described a similar syndrome in 1982, idiopathic uveal effusion syndrome, in healthy middle-aged men with normal eyes [2]. These patients usually present with visual loss in either one or both eyes with a superior visual field defect derived from an inferior RD [3]. It is widely accepted that scleral abnormality with thickened sclera, disorganized collagen fibers and deposition of GAG and compression of vortex veins by the thickened sclera are the primary causes [4]. We report a case of recurrent IUES. Absence of vortex veins was noted intra-operatively. Histologically, the sclera was of normal thickness, but areas of randomly arranged collagen fibers with fraying of collagen fibrils into 50 nm filaments were present. Granular deposits of GAG were noted in the inter fibrillar space.