Anesthesia in surgery for people with FSHD

This webinar discusses anesthetic considerations for patients with FSHD, emphasizing that each anesthetic plan is tailored to the individual patient and procedure. The speaker is Bruna Castro de Oliveira, a board-certified anesthesiologist and director of the orthopedic anesthesia service at Mass General Hospital. Working with Dr. Bassem Elhassan, she has participated in many scapular surgery procedures for patients with FSHD.

Key points include:

• Neuromuscular Considerations: Patients with FSHD can have increased sensitivity to anesthetics, particularly neuromuscular blocking agents like succinylcholine, which should be avoided. Non-depolarizing agents like rocuronium and vecuronium can be used with careful monitoring to avoid prolonged muscle weakness.
• Preoperative Evaluation: A comprehensive, multidisciplinary assessment involving neurology, pulmonology, and cardiology may be necessary based on the patient’s condition. Pulmonary optimization includes baseline pulmonary function tests and incentive spirometry. Cardiac assessment includes an EKG, and possibly an echocardiogram, depending on the patient’s cardiac condition.
• Medication Considerations: Sedating medications should be used cautiously due to the risk of respiratory depression. Steroid use should be carefully managed, with potential need for a stress dose.
• Respiratory Considerations: Some patients may have restrictive lung disease, increasing the risk of hypoventilation and pulmonary complications, especially with sedation or pain medications. Sleep disorders like sleep apnea are also a concern. Pulmonary function tests may be required for patients with advanced lung disease.
• Cardiac Considerations: Arrhythmias and cardiomyopathy can be a concern, potentially requiring an EKG or echocardiogram.
• Anesthetic Plan: General anesthesia may involve endotracheal intubation or an LMA. Due to potential difficulties with intubation and extubation, extra care is required. Neuromuscular blockade should be fully reversed, using sugammadex if needed. Propofol is a safe induction agent, and opioids can be used cautiously for pain management. Regional anesthesia can decrease the amount of opioids needed.
• Intraoperative Management: Ventilation strategies should be gentle, using low tidal volumes and lung-protective ventilation. Train-of-four monitoring is standard for neuromuscular monitoring. Temperature monitoring is essential, and warming blankets are used to prevent hypothermia. Positioning requires careful attention to avoid nerve compression, with a multidisciplinary approach involving the anesthesiologist, circulating nurse, and surgeon. The patient’s range of motion limitations should be considered.
• Postoperative Considerations: Extubation planning considers the patient’s pulmonary function.