Management of a patient with familial amyloid polyneuropathy type I with lumbar epidural anaesthesia

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.