後極部漿液性網膜剥離と脈絡膜剥離を伴ったUsher症候群の1例

A case of Usher syndrome manifested posterior retinal detachment and choroidal detachment

　網膜色素変性と先天聾があり，Usher症候群と診断され，5年前に白内障手術を受けた40歳男子に，両眼の網膜浮腫，漿液性網膜剥離，ならびに左眼の脈絡膜剥離が発症した。両眼の前房と前部硝子体中に炎症細胞が多数浮遊し，網膜血管の透過性亢進が螢光眼底造影により確認された。全身ステロイド投与で，これら眼底病変は消褪し，視力が回復した。網膜浮腫，漿液性網膜剥離，脈絡膜剥離の原因として，網膜変性に基因する炎症反応が疑われた。

A 40-year-old male presented with decreasedvision in his left eye. He was congenitally deaf andhad been diagnosed as retinitis pigmentosa 20 yearsbefore. He had underwent cataract surgery 5 yearsbefore. He manifested serous retinal detachment inthe posterior fundus bilaterally and severe chor-oidal detachment in the left eye. Prominent signs ofiritis were present in both eyes. The retinal vesselsshowed generalized extravasation of dye by fluor-escein angiography. The observed retinal detach-ment was thought to be due to hyperpermeability ofretinal vessels. Systemic treatments with hyper-osmotic agent and corticosteroid resulted indisappearence of retinal and choroidal detachmentswith final fair visual acuity.