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* From the Department of Anesthesia and Perioperative Medicine, the
Division of Plastic Surgery, Hand and Upper Limb Centre, Department of Surgery, University of Western Ontario, London, Ontario, Canada; and the
University Hospitals Coventry and Warwickshire, Coventry, United Kingdom.
Address correspondence to: Dr. Shalini Dhir, Assistant Professor, Department of Anesthesia and Perioperative Medicine, St Joseph’s Health Care, 268 Grosvenor Street, University of Western Ontario, London, Ontario N6A 4V2, Canada. Phone: 519 646-6000, ext. 64219; E-mail: shalini.dhir{at}sjhc.london.on.ca
Purpose: To describe the clinical presentation of three patients with Charcot-Marie-Tooth disease, who underwent uneventful upper limb surgery following successful peripheral nerve blockade, and to review the anesthetic implications in patients with Charcot-Marie-Tooth disease.
Clinical features: In three patients with Charcot-Marie-Tooth disease presenting for surgery of the upper limb, the motor response, following nerve stimulation, was suboptimal. However, ultrasound guidance was effective in visualizing the needle-nerve interaction, and local anesthetic was injected around the nerves. Good block ensued and surgery proceeded in all patients without complications. No exacerbation of the neurological condition was observed in any patient.
Conclusions: Charcot-Marie-Tooth disease is a demyelinating, hereditary, motor and sensory neuropathy characterized by abnormalities of nerve conduction. Regional anesthesia of the upper limb is feasible in these patients, and these cases show that ultrasound guidance makes peripheral nerve block possible in patients for whom traditional methods of nerve localization fail.
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