網膜血管奇形を伴ったSturge-Weber症候群の1例

A case of Sturge-Weber syndrome with retinal vascular malformation

　網膜動静脈奇形を伴ったSturge-Weber症候群の1例を報告した。症例は32歳の男性で，生下時より右側顔面血管腫を認め，1歳頃，癲癇様痙攣発作を起こし，Sturge-Weber症候群と診断された。主訴は右眼霧視で，右眼球結膜血管，上強膜静脈に拡張，蛇行を認めた。眼底には，網膜血管の拡張，蛇行，動静脈吻合がみられ，脈絡膜血管腫によると思われる赤色眼底を呈していた。また，耳側網膜周辺部に無血管領域を認めた。

　左眼には，異常所見はみられなかった。

A 32-year-old male sought medical advice due toblurring of vision in his right eye of recent onset.He had been diagnosed as Sturge-Weber syndromeat the age of one year on account of righthemifacial cavernous angioma and epileptoidattacks. He was mentally retarded. He manifesteddilation and tortuosity of conjunctival and episcler-al vessels in the right eye. Ophthalmoscopically, thesuperior fundus hemisphere appeared diffusely red-dish suggestive of choroidal hemangioma in theright eye. The retinal vessels were dilated andtortuous up to the periphery. A wide avascular zonewas present along the temporal periphery.Fluores-cein angiography showed arteriovenous communi-cations. The left eye was free of pathological find-ings.