眼窩後壁欠損を伴ったレックリングハウゼン病の1例

A case of neurofibromatosis of Recklinghausen with partial absence of orbital wall

（P3-2-43）　78歳の男性で拍動性の眼球陥凹を伴ったRecklinghausen病の1例を報告する。全身に多数のカフェ・オ・レ斑と神経線維腫がみられ，両眼に虹彩結節もみられた。眼球拍動は脈拍に一致し，顎下げによって眼球は突出した。3D-CTなどの放射線科的検査により，蝶形骨大翼・小翼の欠損，前床突起・後床突起の欠損，中頭蓋窩の拡大を認めた。骨欠損のため脳の拍動が眼球に伝わり，加齢により眼窩組織が減少し，骨欠損に比して脳髄膜瘤が小さいため拍動性の眼球陥凹を生じた。本症例に対し球後麻酔を施行すると脳脊髄液中に麻酔薬を誤注入する危険性がある。Recklinghausen病患者の眼科手術時には，術前に放射線科的検査を行い，麻酔法を決定すべきである。

A 78-year-old male presented with pulsating enophthalmos in his left eye. He showed typical features of neurofibromatosis including cafe-au-lait spots, neurofibroma and iris nodules in both eyes. The ocular pulsation was synchronous with the heart beat. The eyeglobe protruded in chin-down position. Radiological studies including 3-dimensional computed tomography showed absence of sphenoidal wings, deformity of clinoid process, enlarged middle cranial fossa, and encephalocele. The ocular pulsation appeared to be transmitted from the cranial cavity through the absent sphenoidal wing. Enophthalmos appeared to be the consequence of decreased orbital content due to aging and the smaller size of encephalocele. This case illustrates that retrobulbar anesthesia is contraindicated in such cases because of the risk of anesthetic agents entering the cerebrospinal fluid. A thorough radiological examination is necessary when planning an eye surgery in patients of neurofibromatosis.