What are the risks of prolonged intubation after muscle relaxation in Charcot-Marie-Tooth (CMT) disease?

From the Guidelines

Prolonged intubation after muscle relaxation in Charcot-Marie-Tooth (CMT) disease poses significant risks, including respiratory complications, and therefore, it is crucial to carefully monitor and manage neuromuscular blockade reversal to minimize these risks. When managing anesthesia for CMT patients, it is essential to consider the potential for increased sensitivity to neuromuscular blocking agents, slower metabolism and clearance of drugs, and underlying respiratory muscle weakness 1. Key considerations for anesthesia management in CMT patients include:

• Using shorter-acting non-depolarizing agents, such as rocuronium or cisatracurium, for muscle relaxation
• Monitoring neuromuscular function closely with train-of-four stimulation
• Reversing muscle relaxation with selective relaxant binding agents (SRBA) like sugammadex, which has been shown to more predictably reverse neuromuscular blockade compared to neostigmine or glycopyrronium 1
• Extubating only when the train-of-four ratio is >0.9 and the patient shows adequate spontaneous breathing To minimize complications, it is also important to:
• Use pressure-controlled ventilation to reduce the risk of barotrauma
• Implement early mobilization and chest physiotherapy
• Monitor closely for respiratory infections The use of SRBA, such as sugammadex, can help reduce the risk of postoperative pulmonary complications, including respiratory failure, by ensuring more reliable reversal of neuromuscular blockade 1. By taking a cautious and individualized approach to anesthesia management and post-operative care, healthcare providers can help minimize the risks associated with prolonged intubation after muscle relaxation in CMT patients and optimize their recovery outcomes.

From the Research

Risks of Prolonged Intubation after Muscle Relaxation in Charcot-Marie-Tooth (CMT) Disease

• The response to non-depolarizing neuromuscular blocking drugs is variable in patients with CMT disease 2.
• Patients with CMT may have increased sensitivity to non-depolarizing neuromuscular blocking agents, and hyperkalemia associated with the administration of succinylcholine has been reported 3.
• The potential risk of malignant hyperthermia and underlying cardiopulmonary abnormalities, such as pre-existing arrhythmias, cardiomyopathy, or respiratory muscle weakness, must also be considered in patients with CMT 3.
• CMT patients undergoing surgery require special consideration of their anesthetic management plan to ensure patient safety and optimize perioperative outcomes 3.
• Total intravenous anesthesia with propofol and remifentanil infusions, and the use of a non-depolarizing neuromuscular blocking agent, may be a suitable anesthetic plan for patients with CMT 3, 4.
• The use of a malignant hyperthermia protocol with avoidance of volatile anesthetics may help decrease the possible risk of malignant hyperthermia in patients with CMT 3.

Anesthetic Management Considerations

• Careful consideration should be given to the anesthetic plan, including thorough pre- and perioperative evaluation of cardiac function 3.
• The use of non-depolarizing neuromuscular blocking agents should be carefully monitored due to the potential for increased sensitivity in patients with CMT 2, 3.
• The risk of delayed muscle relaxation or malignant hyperthermia should be considered when using neuromuscular blocking agents in patients with CMT 3, 4.