title: Use of the laryngeal mask airway in patients with severe muscular dystrophy who require sedation or anesthesia.
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.
authors.affiliation: Department of Pediatrics, MetroHealth Medical Center and Case Western Reserve University School of Medicine, Cleveland, Ohio, USA. dbirnkrant@metrohealth.org
authors.forename: David J
authors.initials: DJ
authors.lastname: Birnkrant
authors.forename: Kasia M
authors.initials: KM
authors.lastname: Petelenz
authors.forename: Roy D
authors.initials: RD
authors.lastname: Ferguson
authors.forename: James E
authors.initials: JE
authors.lastname: Martin
authors.forename: Gregory J
authors.initials: GJ
authors.lastname: Gordon
mesh_terms: D000328:Adult; D017147:Defibrillators, Implantable; D005045:Etomidate / Q000627:therapeutic use; D005774:Gastrostomy; D006801:Humans; D006993:Hypnotics and Sedatives / Q000627:therapeutic use*; D017214:Laryngeal Masks; D008096:Lithotripsy; D009136:Muscular Dystrophies / Q000150:complications*; D011175:Positive-Pressure Respiration; D015742:Propofol / Q000627:therapeutic use; D012189:Retrospective Studies; D012720:Severity of Illness Index
chemical_list: D006993:Hypnotics and Sedatives; D015742:Propofol; D005045:Etomidate