Target-controlled infusion of propofol for a patient with myotonic dystrophy

Abstract

We present a patient with myotonic dystrophy (MD) who was anesthetized with propofol using a target-controlled technique for electrophysiologic examination and cardiac catheter ablation. The patient became apneic unexpectedly at the same time when he fell asleep, with effect-site propofol concentration of 1.6 microg ml(-1). We had to insert a laryngeal mask airway (LMA), and mechanical ventilation was performed. The patient opened his eyes on verbal command at an effect-site concentration of 1.2 microg ml(-1) after the procedure. This concentration (1.2 microg ml(-1)) was slightly lower than our institutional average for adult male patients (1.5 +/- 0.2 microg ml(-1)). However, the time from the end of anesthesia to the patient's awakening was about 10 min. We considered that emergence from anesthesia was not delayed in this case. Careful titration of propofol by target-controlled infusion (TCI) enabled to evaluate the patient's sensitivity to propofol. We conclude that TCI of propofol was a useful anesthetic technique in the MD patient. Respiratory depression might occur in MD patients at low propofol concentrations. Precise control and titration over target propofol concentration is important in anesthetic management for MD patients.

Sensitivity to Rocuronium-Induced Neuromuscular Block and Reversibility with Sugammadex in a Patient with Myotonic Dystrophy

“…Given the importance of the marked susceptibility of MD patients to anesthetics, which may cause apnea and respiratory depression [18], careful titration of propofol and remifentanil by a target-controlled infusion is thought to be appropriate when anaesthetizing MD patients.…”

Total intravenous anesthesia in a 10-month-old patient with congenital myotonic dystrophy undergoing endoscopic third ventriculostomy -A case report-

“…However, variations in drug sensitivity in such patients have also been reported, making dose calculations problematic [2,3]. Propofol has also been shown to provoke a myotonic episode [11] and to cause delayed recovery from anesthesia [12].…”

“…Patients with myotonic dystrophy have chronic restrictive respiratory insufficiency, and expiratory muscle weakness may lead to atelectasis while involvement of the inspiratory muscles increases the risk of alveolar hypoventilation [1]. Unpredictable sensitivity to sedatives, anesthetics, and neuromuscular blocking agents may cause recovery from anesthesia to be prolonged, as decribed in previous case reports [2,3]. Volatile anesthetics are best avoided in patients with muscular dystrophy, due to the potential of triggering malignant hyperthermia or rhabdomyolysis and possible cardiac arrest [4].…”

Desflurane anaesthesia in myotonic dystrophy

“…Others have used sevoflurane without relaxants, PFK (Propofol, fentanyl and ketamine combination), dexmedetomedine, total intravenous anaesthesia with propofol, cisatracurium and remifentanil and target controlled infusion of propofol in these patients. [610–12]…”