広範なる頭蓋骨缺損と眼球突出を伴えるRecklinghausen氏病症例

A case of Recklinghausen's disease with extensive bone deficiency of the skull and unilateral exophthalmos

I．はじめに

　Recklinghausen氏病（Neurofibromatosis）に関する報告は各分野に於て数多くみられ，その臨床所見も多彩を極めている。著者らは未だ文献上記載がない側後頭骨の広範囲欠損と，眼窩骨変状を伴う同症例を経験したので報告する。

A case of a 14-year-old boy born in incest was reported. The patient showed non-pulsa-ting exophthalmus, high myopia and optic atrophy in his right eye. Roentgenographic examination of the skull revealed a wide bone deficiency of occipito-temporal region, enlarged ventriculus cerebri, abnormal pitui-tary fossa and forward displacement of the temporal fossa. It was of interest that the exophthalmus seemed to be caused by the forward displacement of the bone at the apex of the orbit. Histopathological studies confirmed the diagnosis of neurofibromatosis.