Shono S, Higa K, Sakimura M, Dan K
Division of Anesthesiology, Arao City Hospital, Japan.
Eur J Anaesthesiol. 1998 Mar;15(2):242-5.
Familial amyloid polyneuropathy (FAP) type I is a rare disease characterized by sensorimotor polyneuropathy, and autonomic nervous system and cardiac conduction system dysfunction. Severe bradyarrhythmia and hypotension may occur during anaesthesia and surgery in patients with the FAP type I. Only one report has been published of a patient with FAP type I who was managed with epidural anaesthesia. The present authors report a 38-year-old Japanese man with a permanent pacemaker because of advanced FAP type I who underwent rotation flap of the gluteus maximus muscle to cover a trophic ulcer in the sacral region under lumbar epidural anaesthesia. Ten millilitres of adrenaline (1:200,000) was injected around the ulcer prior to surgical manipulation. Neither bradycardia nor hypotension developed during the procedures. The haemodynamic changes under lumbar epidural anaesthesia in FAP type I are discussed.
I型家族性淀粉样多神经病（FAP）是一种罕见疾病，其特征为感觉运动性多神经病、自主神经系统和心脏传导系统功能障碍。I型FAP患者在麻醉和手术期间可能会出现严重的缓慢性心律失常和低血压。关于I型FAP患者接受硬膜外麻醉的报道仅有一例。本文作者报告了一名38岁的日本男性，因晚期I型FAP植入了永久性起搏器，该患者在腰段硬膜外麻醉下接受了臀大肌旋转皮瓣手术，以覆盖骶部的营养性溃疡。手术操作前，在溃疡周围注射了10毫升肾上腺素（1:200,000）。手术过程中未出现心动过缓或低血压。本文讨论了I型FAP患者腰段硬膜外麻醉下的血流动力学变化。
