von Recklinghausen病の特異型の1例

Pulsating Exophthalmos with Defect of the Orbital Wall in von Recklinghausen's Disease; Report of a Case

Ⅰ．はじめに

　von Recklinghausen病（以下R病と略す）は，café-aulait spotsと称される皮膚の色素沈着，皮下軟結節などを主な徴候としながら，しばしば神経系やその付属支持組織，骨系統などの病変を伴い，多彩な病像を呈してくることはよく知られている．そのうちでも特異型というべき一側の眼窩壁の欠損に伴って出現する搏動性眼球突出については，欧米では従来かなりの報告が見られるが，本邦での報告は極めて稀である．今回私共はその1例を経験したので，その特徴的なレ線像，手術所見と共に，文献的考察を加えて報告する．

Pulsating exophthalmos with defect of the orbital wall in von Recklinghausen's disease is a relatively rare con-dition. The following case is illustrative.

A 21-year-old female, who had been noticed by her parents to have dislocation of the left eye and bone defect of the left fronto-temporal region of the skull since birth. was admitted to our clinic on 20 March, 1972 for slowlyprogressive loss of visual acuity and retroocular pain on the left. On admission, the neurological abnormality was limited to the left eye and cafe-au-lait spots were seen all over the body skin.