2024 FSA Podium and Poster Abstracts

P080: UNCOMPLICATED GENERAL ANESTHESIA IN A PATIENT WITH CHARCOT-MARIE-TOOTH (CMT) DISEASE
Robert Bruce, MD; University of Tennessee HSC

Charcot-Marie-Tooth (CMT) disease is an inherited neuropathy presenting with sensory and/or motor deficits. With an approximate prevalence of 19:100000, anesthesia providers are likely to encounter CMT patients in practice. Anesthesia in patients with CMT disease poses challenges, variable onset of action of neuromuscular blockade, potential link with malignant hyperthermia, and insufficient postoperative pulmonary function. There is a paucity of literature regarding the management of anesthesia for CMT disease.

Herein, we discuss a successful case of general anesthesia in a 45-year-old CMT female patient. She was presented at a level 1 trauma center following a high-speed motor vehicle collision where she was the restrained passenger.  On initial triage, she had patent airway with spontaneous breathing, and was hypotensive.  Gross deformity to the left femur was noted as well as abdominal bruising. FAST exam was negative and following administration of 1L of LR, she became hemodynamically stable and was sent to CT scan.  Imaging indicated a displaced comminuted fracture along the proximal shaft of femur with adjacent soft tissue swelling. Orthopedic surgery was consulted who noted her bilateral clawing of the feet consistent with CMT and her right closed proximal femoral shaft fracture of which she was placed in Buck's traction and planned operative fixation with a CRIM Femur Antegrade.

Preoperative assessment was completed by the anesthesia team which was positive for history of postoperative nausea and vomiting, GERD, and functional limitation at baseline secondary to CMT disorder. Premedication with midazolam followed by a Modified Rapid Sequence Induction with fentanyl, lidocaine, propofol, and rocuronium.  Intubation with a 7.0 ETT was performed easily on first attempt with a McGrath 3 blade.  General anesthesia was maintained with sevoflurane.  Train of four monitoring was measured throughout and midway through procedure required redosing of rocuronium.  Prophylaxis for postoperative nausea and vomiting was achieved with ondansetron, dexamethasone, and a scopolamine patch.  Analgesia was provided with fentanyl and intravenous acetaminophen. Reversal of neuromuscular blockade was achieved with sugammadex.  The patient emerged from anesthesia and extubated uneventfully.  PACU course was similarly uneventful without nausea or vomiting nor significant pain.  Regional anesthesia was planned should significant plain be encountered as while somewhat limited evidence appears to support a favorable safety profile in patients with CMT.  During relief of the case handoff included instruction to the oncoming provider on anesthetic management in CMT and a summary in table form was provided.

This case demonstrates that well established anesthetic practices may be safely employed in a patient with CMT disorder. While case reports seem to indicate succinylcholine may be safe to use in patients with CMT without concern for an exaggerated hyperkalemic response from activation of extrajunctional acetylcholine receptors, we elected for use of rocuronium to facilitate RSI and noted favorable intubating conditions within 30 seconds of administration. Additionally, despite the larger dose of rocuronium at induction, its clinical effect was not noticeably prolonged and easily reversed with sugammadex. 

Our case will offer valuable insights into the realm of anesthesia in CMT patients, providing clinicians with practical knowledge for their decision-making.