UVEAL EFFUSION IN HUNTER'S SYNDROMEEvidence That Abnormal Sclera is Responsible For the Uveal Effusion Syndrome

摘要

A patient with Hunter's Syndrome (systemic mucopolysaccharidosis type II) was evaluated for bilateral uveal effusion syndrome. The right eye showed a circular peripheral choroidal detachment for 360deg;. The left eye showed a larger circular peripheral choroidal detachment and an exudative retinal detachment. Evaluation of the sclera during combined sclerectomies and sclerostomies of the left eye revealed markedly thickening sclera and a reduced number of vortex veins. The surgery resulted in complete resolution of the choroidal detachment and exudative retinal detachment of the left eye. The presence of uveal effusion in Hunter's Syndrome, in which the sclera has been histologically demonstrated to be abnormally thickened, supports the recently proposed pathophysiology of the uveal effusion syndrome.