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Background and Aims Pure Autonomic Failure (PAF) is a rare neurodegenerative disease of the autonomic nervous system. The etiology is unknown but its pathophysiology involves the accumulation of a protein, called Lewy bodies, in the cells of autonomic nerves, leading to reduced norepinephrine production and release. Therefore, the main symptom of PAF is orthostatic hypotension, but it can also present bladder dysfunction, constipation, anhidrosis and sleep disorders. We describe the successful anesthetic management of a patient with PAF.
Methods A 68 year old man, ASA physical status III, was scheduled for unicompartmental knee prosthesis surgery. He was diagnosed with PAF 5 years before due to orthostatic hypotension, neurogenic bladder, erectile dysfunction, hyposmia and REM sleep behavior disorder. An arterial line and central venous catheter were placed. We performed regional anesthesia with femoral, sciatic, obturator and lateral cutaneous nerve blocks guided by ultrasound and neurostimulation.
Results The surgery took about 1 hour and went out uneventfully with no need to administer vasoactive drugs. The patient was transferred to the intermediate care unit and was discharged home on post-operative day 4.
Conclusions PAF is a rare disease that can present challenges to the Anaesthesiologist. General management must focus on ensuring hemodynamic stability perioperatively. In this clinical case, we demonstrate that regional anesthesia with peripheral nerve blocks can be an effective and safe anesthetic option. Further considerations include: exaggerated or unpredictable response to vasopressors, decreased clearance of drugs with liver metabolism (such as amino amide local anesthetics) and avoidance of prolonged postoperative inactivity.