The anesthetic management of a patient with Emery-Dreifuss muscular dystrophy for orthopedic surgery [La prise en charge anesthésique d'un patient de chirurgie orthopódique atteint de dystrophie musculaire de Emery-Dreifuss]

Purpose: To report a patient with Emery-Dreifuss muscular dystrophy (EDMD) coming for an orthopedic procedure, with potential problems of sudden cardiac death, difficult airway, and neuromuscular disorders who was managed successfully by permanent pacemaker insertion, total iv anesthesia (TIVA), laryngeal mask insertion (LMA) insertion and continuous epidural blockade. Clinical features: A 22-yr-old man with known EDMD presented for triple arthrodesis of his right foot and fractional lengthening of his hamstrings bilaterally. Anesthesia was induced with a TIVA technique, and maintained throughout the operative period. A suspected difficult airway was managed by the use of a LMA, and analgesia for the peri-, and postoperative period provided by a continuous epidural infusion. The patient's perioperative course was uneventful. Conclusion: EDMD is a rare disorder. However, anesthesia is often required for orthopedic procedures. This case report illustrates the many potential difficulties that may be encountered. Regional anesthesia combined with light general anesthesia offers a method of avoiding many of these difficulties.