レット症候群患者に対する側彎症手術2症例の麻酔経験

Anesthesia Management in Two Patients with Rett Syndrome Undergoing Spinal Fusion Surgery

要旨

　レット症候群（Rett syndrome：RTT）は主にMECP2遺伝子の突然変異により発症する進行性の中枢神経疾患である。成長に伴い側彎を発生することが多く，時には手術が必要になる。麻酔管理上の問題点としては，開口障害，呼吸異常，不整脈，覚醒遅延などが挙げられる。今回われわれは，RTT患者の側彎症手術の麻酔を2症例経験したので報告する。

　Rett syndrome（RTT）is an X-linked neurodevelopmental disorder that affects almost exclusively women. It is known that the anesthetic management of patients with RTT must address various difficulties such as dysphagia, respiratory abnormalities, arrhythmias, and delayed arousal. We describe the anesthesia administered for spinal fusion in two patients with RTT. One patient（a 14-year-old Japanese woman）had trismus and thin vessels, and the other patient（a 17-year-old Japanese woman）had QT prolongation；both of these conditions required careful management. Clinicians should be aware that as in the present cases, RTT presents a wide variety of manifestations and requires individualized procedures.