General Anaesthesia in the Management of Myotonic Dystrophy Patient Posted for Tympanoplasty

Author(s): Savitri D. Kabade1 , Priti Ravindra Sherikar2

Myotonic dystrophy is a genetic disorder in which there is progressive weakness and loss of muscle mass which often contract but are unable to relax. Anaesthetic management of myotonic dystrophy (DM) can be challenging with complications like loss of airway secondary to medication-induced respiratory depression, aspiration, hypoventilation and sudden death due to cardiac conduction abnormalities. In the light of these complications, one should consider “regional anaesthesia as a viable alternative if the surgical procedure is really necessary”. However, in surgeries which require general anaesthesia one should avoid triggering factors like hypothermia, opioids, inhalational agents and succinylcholine. Severity of the patient disease must be elucidated on preanesthetic evaluation. PONV prophylaxis, rapid sequence intubation, short acting muscle relaxant, ECG and temperature monitoring are the essence of successful anaesthetic management.