Strategies for Neuromuscular Blockade in Patients with Myotonic Dystrophy
| Authors | |
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| Year of publication | 2015 |
| Type | Chapter of a book |
| MU Faculty or unit | |
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| Description | Myotonic dystrophy is a chronic, slowly progressing, highly variable, inherited multisystemic disease. It is characterized by wasting of the muscles, cataracts, heart conduction defects, endocrine changes, slow gastric and bowel emptying, and myotonia. Patients with myotonic dystrophy show myotonic responses to succinylcholine and neostigmine, and increased sensitivity to non-depolarising muscle relaxants. Sugammadex, a novel drug developed specifically for the rapid reversal of neuromuscular blockade induced by steroidal non-depolarising muscle relaxants, would be useful in patients with muscular disorders. The use of sugammadex was reported in a patient with myasthenia gravis and Huntington’s disease. During 2012-2014 were reported successful use of sugammadex in some cases for caesarean section, for laparoscopic ovarian cystectomy and for urgent abdominal surgery. In this chapter we would like to present, based on best evidence, strategy of neuromuscular blockade for the patient with myotonic dystrophy. We will report physiology and pathophysiology of neuromuscular transmission and potentially suitable approaches for neuromuscular blocking agent use. We will especially focus on premedication, induction of anaesthesia, response to muscle relaxants, reverse of neuromuscular blockade, the crucial points of safe anaesthesiological care, and other perioperative triggers. Moreover we will discuss use of neuromuscular blockade and its reverse in patients with myotonic dystrophy undergoing some special surgeries – caesarean section, scoliosis surgery and general surgery. Patients with myotonia may have abnormal responses to anaesthetic agents. An increased sensitivity to non-depolarising muscle relaxants is described in patients with myotonic dystrophy. Anti-cholinesterase drugs (neostigmine) can lead to myotonic reaction. The combination of rocuronium and sugammadex have potential to replace previous (traditional) anaesthetic management in patients with muscle disorders. The use of rocuronium is well described and is common alternative to succinylcholine at induction of general anaesthesia especially during Rapid Sequence Induction. Active selective reversal of neuromuscular blockade induced with rocuronium for C.S. is also well described. To the best of known knowledge we suggest that rocuronium and sugammadex use in patients with myotonic dystrophy might be useful in general when muscle relaxation is needed. |