title: Anesthesia and Duchenne or Becker muscular dystrophy: review of 117 anesthetic exposures.
abstract: BACKGROUND
  Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are associated with life-threatening perioperative complications, including rhabdomyolysis, hyperkalemia, and hyperthermia. Current recommendations contraindicate use of succinylcholine and volatile anesthetics; however, the latter recommendation remains controversial.
  
  
  OBJECTIVE
  To review the perioperative outcomes of patients with DMD and BMD.
  
  
  METHODS
  We reviewed records of patients with DMD or BMD who underwent anesthetic management at our institution from January 1990 through December 2011.
  
  
  RESULTS
  We identified 47 patients (DMD, 37; BMD, 10) who underwent 117 anesthetic exposures (DMD, 101; BMD, 16). Volatile anesthetic agents were used 66 times (DMD, 59; BMD, 7). One patient with undiagnosed BMD received succinylcholine and developed acute rhabdomyolysis and hyperkalemic cardiac arrest. All other major complications were attributed to the procedure (i.e., large bleeding), to preexisting comorbidities (i.e., respiratory failure, cardiac disease), or to both.
  
  
  CONCLUSIONS
  Use of succinylcholine in children with dystrophinopathy is contraindicated. These patients have significant comorbidities and are frequently undergoing extensive operations; complications related to these factors can develop, as evidenced by our series. These complications may occur with use of volatile and nonvolatile anesthetics. However, because most of our patients were older than 8 years at the time of surgery, our observation cannot be generalized to younger dystrophin-deficient children.
authors.affiliation: Department of Anesthesiology, Mayo Clinic, Rochester, MN 55905, USA.
authors.forename: Leal G
authors.initials: LG
authors.lastname: Segura
authors.forename: Jessica D
authors.initials: JD
authors.lastname: Lorenz
authors.forename: Toby N
authors.initials: TN
authors.lastname: Weingarten
authors.forename: Federica
authors.initials: F
authors.lastname: Scavonetto
authors.forename: Katarina
authors.initials: K
authors.lastname: Bojanić
authors.forename: Duygu
authors.initials: D
authors.lastname: Selcen
authors.forename: Juraj
authors.initials: J
authors.lastname: Sprung
mesh_terms: D000293:Adolescent; D000328:Adult; D000758:Anesthesia / Q000009:adverse effects; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D000075202:Contraindications; D016208:Databases, Factual; D016189:Dystrophin / Q000172:deficiency / Q000235:genetics; D005260:Female; D006323:Heart Arrest / Q000209:etiology; D006801:Humans; D006947:Hyperkalemia / Q000150:complications; D007223:Infant; D007431:Intraoperative Complications / Q000503:physiopathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D020388:Muscular Dystrophy, Duchenne / Q000150:complications* / Q000235:genetics / Q000503:physiopathology; D009467:Neuromuscular Depolarizing Agents; D011183:Postoperative Complications / Q000503:physiopathology / Q000628:therapy; D012206:Rhabdomyolysis / Q000150:complications / Q000503:physiopathology; D013390:Succinylcholine; D055815:Young Adult
chemical_list: D016189:Dystrophin; D009467:Neuromuscular Depolarizing Agents; D013390:Succinylcholine
keywords: Becker muscular dystrophy; Duchenne muscular dystrophy; hyperkalemia; hypermetabolism due to defect in mitochondria; rhabdomyolysis; succinylcholine