ANESTHESIA FOR CHARCOT-MARIE-TOOTH DISEASE: CASE REPORT

Area of Science:

• Anesthesiology
• Neurology
• Genetics

Background:

• Charcot-Marie-Tooth disease (CMTD) is a group of inherited neurological disorders causing progressive muscle weakness and atrophy.
• Anesthetic management for CMTD patients is complex due to increased risks, including prolonged neuromuscular blockade and potential for malignant hyperthermia.
• Existing literature offers conflicting recommendations for optimal anesthetic strategies in CMTD patients.

Observation:

• A 17-year-old male patient diagnosed with Charcot-Marie-Tooth disease required orthopedic surgery on his lower limb.
• The patient's medical history indicated a heightened risk profile associated with standard anesthetic procedures.
• This case highlights the need for carefully selected anesthetic techniques in this patient population.

Findings:

• Total intravenous anesthesia (TIVA) utilizing dexmedetomidine and propofol was administered.
• A caudal block was performed using bupivacaine combined with dexmedetomidine.
• The combined anesthetic technique resulted in the successful completion of the orthopedic surgery without any reported complications.

Implications:

• This case demonstrates the feasibility and safety of using TIVA with dexmedetomidine and propofol, coupled with a dexmedetomidine-bupivacaine caudal block, for surgical procedures in CMTD patients.
• The findings suggest a potentially advantageous anesthetic protocol for managing patients with Charcot-Marie-Tooth disease, mitigating known risks.
• Further research into anesthetic protocols for rare neuromuscular disorders like CMTD is warranted to establish evidence-based guidelines.