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S.A. Gollance, R. Radhakrishnan, M.A. Zarbin, N. Bhagat; Aplasia of the Vortex Veins: A New Etiologic Possibility for Idiopathic Uveal Effusion Syndrome . Invest. Ophthalmol. Vis. Sci. 2005;46(13):5576.
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Purpose: Uveal effusion syndrome is an idiopathic condition associated with exudative retinal, choroidal, and ciliary body detachments. We present a case of idiopathic uveal effusion syndrome (IUES) in a patient with aplasia of the vortex veins, normal sclera on histological examination, and a history of recurrent serous retinal detachments in the fellow eye. Methods: Observational case report describing unique ocular, systemic, anatomic, and pathologic findings in a patient with IUES. Results: A 46 year–old Hispanic man with a 10–year history of recurrent serous retinal detachments in one eye presented with a serous retinal detachment in the fellow eye and was diagnosed with IUES. Scleral windows were performed with resolution of the retinal detachment. Exploration of the scleral anatomy at the time of surgery disclosed aplasia of the vortex veins. Histological analysis of the four scleral specimens revealed normal scleral tissue with collagen fibers regularly arranged in parallel with even width and scarce matrix. Alcian blue stain for proteoglycans was negative. Conclusions: We postulate that aplasia of the vortex veins is the etiologic factor contributing to decreased scleral outflow and resultant IUES in this case. Histologically, the sclera was normal without deposition of proteoglycans. To our knowledge, there are no published reports of aplasia of vortex veins associated with IUES. Ophthalmologists should consider idiopathic uveal effusion syndrome in the differential diagnosis of recurrent exudative retinal detachments. A combination of slit–lamp examination, fluorescein angiogram, and echographic findings can aid in the diagnosis of this rare disease.
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