Journal d'ophtalmologie clinique et expérimentale
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A Case of Vortex Vein Aplasia and Recurrent Idiopathic Uveal fusion Syndrome

Neelakshi Bhagat, Yufei Tu and Marco A Zarbin

A 46-year-old Hispanic man with a history of retinal detachment (RD) in the left eye presented ten years later with worsening superior field visual defect due to inferior serous RD in the right eye. Choroidal and ciliary body detachments were present. A diagnosis of idiopathic uveal effusion syndrome (IUES) was made after exclusion of other etiologies, and he underwent placement of sclera windows. Absence of vortex veins was noted intra-operatively. Histological analysis of the sclera specimen showed abnormal arrangement of collagen fibrils with the deposition of glycosoaminoglycan (GAG). His vision gradually improved postoperatively, and the retina completely attached within four months. Four years later, however, patient developed recurrence of IUES and subsequently underwent debridement of previouslyconstructed scleral windows that were noted to be covered with ingrowth of fibrous tissue. Resolution of subretinal fluid and recovery of baseline vision were achieved postoperatively within 4 months, and no relapse has occurred during 27-month follow-up.