頭蓋底陥入症を合併した骨形成不全症患者に対する脊椎側彎症手術の麻酔経験

Anesthetic Management for Spinal Scoliosis Surgery in a Patient with Osteogenesis Imperfecta Associated with Basilar Invagination：A Case Report

要旨

　頭蓋底陥入症を合併した骨形成不全症患者の側彎症手術の麻酔経験を報告する。気管挿管は助手による頸部不動監視下にビデオ喉頭鏡を用いて行った。体位変換や側彎矯正に伴う神経障害を検知するため運動誘発電位検査および体性感覚誘発電位検査を行い，延髄圧迫による循環動態の変動に対処できるよう中心静脈カテーテルを用いた。周術期の呼吸・循環器系合併症が懸念されたが，明らかな有害事象なく管理を行いえた。

　Basilar invagination（BI）is one of the rare complications of osteogenesis imperfecta. BI occurs when the odontoid process of C2 prolapses into the foramen magnum. This causes brainstem compression or syringomyelia, which may lead to severe neurological disability. Surgical intervention may be necessary to slow down the progression of skeletal deformity but may pose problems in airway and respiratory management as well as intraoperative positioning. In this article, we report our experience with anesthesia for scoliosis surgery in a patient with osteogenesis imperfecta and severe basilar invagination.