Abstract

Severe muscular dystrophy (MD) has historically led to death in early adulthood, due to mainly cardiopulmonary complications. However, with newer methods of cardiac and respiratory management, survival has improved, and patients with MD are more frequently undergoing procedures requiring deep sedation or anesthesia. Respiratory management of these patients during procedures is challenging; safe and effective options for respiratory support are needed. In this report, we describe our experience using the laryngeal mask airway (LMA) to provide respiratory support during deep sedation or anesthesia for eight patients with severe MD during the following medical procedures: eight percutaneous endoscopic gastrostomy (PEG) placements, three lithotripsies, and placement of an implantable cardioverter-defibrillator. We also review the benefits and risks of the LMA in the context of other respiratory support options for people with MD, and the integral role of non-invasive positive pressure ventilation (NPPV) during induction of and recovery from deep sedation or general anesthesia.