Perioperative Anaesthetic Management in Emery-dreifuss Muscular Dystrophy: A Narrative Review

Emery-Dreifuss Muscular Dystrophy (EDMD) is a very rare but also clinically important laminopathy that is accompanied by early contractures, weakness of the humeroperoneal muscles and progressive cardiac involvement such as conduction abnormalities, atrial arrhythmias, and dilated cardiomyopathy. These multisystem characteristics present special perioperative problems in EDMD, and thus, EDMD is a high-risk condition for anaesthesiologists. This is a narrative review summarising the existing evidence, also highlighting the overall picture of perioperative considerations in EDMD in the light of preoperative optimisation, intraoperative monitoring, anaesthetic pharmacology and postoperative care. Preoperative evaluation of EDMD requires meticulous cardiac assessment with the help of ECG, echocardiography, Holter monitoring, as well as pacemaker/Implantable CardioverterDefibrillator (ICD) interrogation, alongside respiratory evaluation, which proves beneficial for restrictive physiology. Intraoperatively, it is necessary to have rhythm surveillance, invasive haemodynamic surveillance, quantitative neuromuscular surveillance, and the presence of external pacing. Succinylcholine should not be used because of the risks of hyperkalemia and rhabdomyolysis, and the non depolarising agents also require careful titration. Total Intravenous Anaesthesia (TIVA) may be preferred over volatile anaesthetic agents in selected patients, and multimodal analgesia, which is safe. Despite the advantages of the regional and neuraxial practices like opioid sparing and cardiac stability, their implementation needs a thorough selection of patients and cardiac preparedness. In the postoperative period, the EDMD patients should be monitored at the ICU level to identify the arrhythmias, respiratory dysfunction, and delayed neuromuscular recovery. The present review highlights the need for adequately standardised protocols and future research to improve perioperative safety related to the management of EDMD patients.