GA3-75
Rationale of using sugammadex in a patient with myotonic dystrophy
1Ahmed S, 2Naguib A, 2Tobias J
1Nathingwide children's hospital, colombus, Ohio, USA; 2Nationwide Childrens Hospital, Columbus, Ohio, USA
Introduction: Myotonic dystrophy (DM) is an autosomal dominant inherited disorder of the sarcolemma, resulting in abnormal responses to neuromuscular blocking agents (NMBAs) that may impact perioperative care. Acetylcholinesterase inhibitors such as neostigmine, are contraindicated in these patients due to their myotonic effect. Sugammadex is a novel pharmacologic agent, which encapsulates rocuronium or vecuronium to reverse their effect. We report our experience with using sugammadex to reverse NMBAs in a patient with DM.
Case Report: We present 25-year-old, 51.6 kg male, initially admitted for cardiothoracic surgery. His history included progressive aortic valve insufficiency and DM type 1. Clinical examination revealed symmetrical facial weakness, ptosis with temporal balding, and upper and lower limb muscle weakness. Preoperative EKG demonstrated a heart rate of 54 bpm, intraventricular conduction delay, left ventricular hypertrophy, and early repolarization. Echocardiogram showed an abnormal bicuspid aortic valve and aortic insufficiency. The patient was held nil per os (NPO) for 6 hours and transported to the operating room (OR) where ASA monitors were placed. A peripheral intravenous catheter was placed and a rapid sequence induction (RSI) with endotracheal intubation was performed using midazolam (2 mg), fentanyl (100 µg), etomidate (10 mg), propofol (30 mg), rocuronium (50 mg), and lidocaine (60 mg). Maintenance anesthesia was provided with desflurane, dexmedetomidine, and fentanyl. Cardiopulmonary bypass time was 3:10 hours and the total rocuronium dose was 110 mg. Neuromuscular blockade was reversed with sugammadex (4 mg/kg), and after return of appropriate ventilation, the patient’s trachea was extubated when he was awake in the OR. The patient returned in 5 weeks for transesophageal echocardiography and cardioversion due to atrial flutter with depressed myocardial function. He was held NPO for 6 hours and was transported to the procedure room where ASA monitors were placed. RSI with endotracheal intubation was performed using etomidate (16 mg), fentanyl (25 µg), and rocuronium (50 mg). Maintenance anesthesia was provided with desflurane. Following electrical cardioversion, the patient returned to normal sinus rhythm. Forty-three minutes after the single dose of rocuronium, sugammadex (4 mg/kg) was administered. Within 10 minutes, the patient regained baseline neuromuscular function and when awake, his trachea was extubated. During both procedures, the postoperative course was unremarkable.
Discussion: Perioperative morbidity in patients with DM may be due to respiratory failure, upper airway obstruction, cardiac failure, arrhythmias, or aspiration. Given the potential for exacerbating myotonia and associated hypotonia, the choice of NMBA and reversal agents may significantly impact the perioperative course. Succinylcholine and neostigmine are relatively contraindicated in these cases. By contrast, sugammadex allows rapid and full reversal neuromuscular blockade in patients with DM without exacerbating myotonia. In our patient, sugammadex (4 mg/kg) effectively reversed neuromuscular blockade and allowed for early tracheal extubation.
Reference:
1. Tobias JD. Paediatr Anaesth 1995;5:335-8
Top
