網膜血管異常と裂孔原性網膜剝離を伴ったSturge-Weber症候群の1例

A case of Sturge-Weber syndrome with retinal vascular abnormality and rhegmatogenous retinal detachment

要約　25歳女性が左眼結膜充血で受診した。生下時から左三叉神経第1と第2枝に単純性血管腫があり，Sturge-Weber症候群と診断されていた。形成外科で眼瞼を含む顔面血管腫に対して全層植皮術を受けていた。矯正視力は右0.7，左0.8であり，右に約-11D，左に-6.5Dの近視があった。左眼に網膜の血管拡張と蛇行があり，上鼻側に裂孔原性網膜剝離があった。網膜血管は赤道より周辺で狭細化または白線化し，この部位に萎縮性円孔が生じていた。網膜復位術を行い，虚血網膜に対して光凝固を行った。網膜血管異常と裂孔原性網膜剝離が本症候群で起こりうることを示した症例である。

Abstract.　A 25-year-old female presented with conjunctival hyperemia in her left eye. She had had naevus flammeus in her face served by the first and second branch of the left trigeminal nerve. She had received whole-layer skin transplantation under the diagnosis of Sturge-Weber syndrome. Her corrected visual acuity was 0.7 right and 0.8 left. She had myopia of about-11 diopters（D）right and-6.5D left. Her left eye showed dilatation and tortuosity of retinal vessels. Rhegmatogenous retinal detachment was present in the superior nasal periphery. Retinal vessels peripheral to the equator,where the retinal break was located,had either narrowed or occluded. We treated the left eye by retinal detachment surgery and by photocoagulation to the atrophic retina. This case illustrates that Sturge-Weber syndrome may be associated with retinal vascular abanormality and rhegmatogenous retinal detachment.