What are the anesthetic considerations for a patient with Charcot‑Marie‑Tooth disease?

Anesthetic Considerations for Charcot-Marie-Tooth Disease

Total intravenous anesthesia (TIVA) with propofol and remifentanil is the preferred anesthetic technique for patients with Charcot-Marie-Tooth disease, avoiding volatile anesthetics due to potential malignant hyperthermia risk, while non-depolarizing neuromuscular blocking agents can be used safely with careful monitoring, and succinylcholine should be avoided due to hyperkalemia risk. 1, 2

Neuromuscular Blocking Agent Considerations

Avoid Depolarizing Agents

• Succinylcholine is contraindicated due to reported cases of hyperkalemia in CMT patients 1
• This risk stems from the underlying denervation and muscle membrane instability characteristic of the disease 1

Non-Depolarizing Agents Require Monitoring

• Rocuronium and other non-depolarizing agents can be used safely but require neuromuscular monitoring throughout anesthesia 3, 1, 2
• CMT patients may demonstrate increased sensitivity to non-depolarizing muscle relaxants with potential for prolonged blockade 1, 4
• The French Society of Anaesthesia and Intensive Care strongly recommends using neuromuscular monitoring devices throughout anesthesia when muscle relaxants are employed 3
• Muscle relaxants facilitate tracheal intubation and should be used when intubation is planned 3

Anesthetic Technique Selection

Total Intravenous Anesthesia (TIVA) is Preferred

• Use propofol and remifentanil infusions as the primary anesthetic technique 1, 2, 5
• Multiple case reports from 2016-2023 demonstrate successful outcomes with TIVA in CMT patients undergoing various procedures including cardiac surgery 1, 2, 4, 5
• Avoid volatile anesthetics due to theoretical risk of malignant hyperthermia, though this association remains controversial 1, 2, 4
• Have a malignant hyperthermia protocol readily available even when using TIVA 1

Regional Anesthesia is Safe

• Regional anesthesia and peripheral nerve blocks are not contraindicated in CMT patients 6
• A 2022 cohort study of 53 CMT patients receiving 132 regional anesthetics found no documented neurologic complications or increased nerve injury risk related to regional techniques 6
• This challenges the traditional teaching that regional anesthesia should be avoided in CMT 6
• Caudal blocks combined with TIVA have been successfully used for lower limb orthopedic procedures 4

Critical Preoperative Assessment

Cardiopulmonary Evaluation

• Thoroughly evaluate for underlying cardiac abnormalities including pre-existing arrhythmias and cardiomyopathy, which can occur in CMT 1
• Assess respiratory muscle weakness preoperatively, as this affects ventilation management and extubation planning 1
• CMT patients may have reduced perioperative respiratory function requiring careful monitoring 5

Autonomic Nervous System Dysfunction

• Screen for autonomic dysfunction which can affect hemodynamic stability during anesthesia 5
• CMT patients may show altered responses to vasoactive medications 5

Intraoperative Management

Positioning Challenges

• Anticipate difficulty with positioning due to skeletal deformities, muscle atrophy, and joint contractures 5
• Many CMT patients have scoliosis and foot deformities requiring special padding and positioning considerations 2, 5

Sedation Sensitivity

• CMT patients may demonstrate increased sensitivity to intravenous anesthetic agents requiring dose adjustments 5
• When using moderate sedation, midazolam and propofol have been used successfully with careful titration 5

Postoperative Considerations

Extubation Planning

• Plan extubation carefully considering baseline respiratory muscle weakness 1
• Ensure complete reversal of neuromuscular blockade before extubation using objective monitoring 3
• Sugammadex can be used for reversal of rocuronium-induced blockade 3

Monitoring Duration

• Extended postoperative monitoring may be warranted given potential for delayed complications from respiratory muscle weakness 1