Charcot-Marie-Tooth Disease With Persistent Perioperative Hypotension due to Autonomic Dysfunction: A Case Report.

Abstract

Charcot-Marie-Tooth (CMT) is a hereditary neuromuscular syndrome associated with peripheral neuropathy. We describe a case of long-standing CMT disease who underwent general anesthesia for a total knee replacement and developed significant perioperative hypotension. Clinical assessment revealed vasoplegia due to underlying autonomic dysfunction as the most likely cause. Further review also revealed a similar episode of intraoperative hypotension during a previous anesthetic. After failure of conservative strategies, the patient was started on a vasopressor infusion. However, hypotension persisted in the early postoperative period. This case serves to highlight the significance of autonomic dysfunction in CMT patients which can cause perioperative hypotension, a finding that has been rarely reported in the literature. We also discuss current diagnostic modalities that may be useful in managing cases of autonomic dysfunction that may present with hypotension.