Duchenne Muscular Dystrophy: An Old Anesthesia Problem Revisited

Paediatr Anaesth. 2008 Feb;18(2):100-6. doi: 10.1111/j.1460-9592.2007.02302.x.

Abstract

Patients with Duchenne and Becker muscular dystrophy suffer from a progressive deterioration in muscle secondary to a defect in the dystrophin gene. As such, they are susceptible to perioperative respiratory, cardiac and other complications, such as rhabdomyolysis. Inhalational anesthetic agents have been implicated as a cause of acute rhabdomyolysis that can resemble malignant hyperthermia (MH). This article reviews perioperative 'MH-like' reactions reported in muscular dystrophy patients and groups them into three categories according to clinical presentation. The etiology and underlying pathophysiological process responsible for these reactions is discussed and recommendations are proposed for the safe anesthetic management of these patients.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Anesthesia, Inhalation*
  • Anesthetics, Inhalation / adverse effects
  • Child
  • Child, Preschool
  • Diagnosis, Differential
  • Heart Arrest / complications
  • Heart Arrest / etiology
  • Humans
  • Hyperkalemia / complications
  • Intraoperative Complications / diagnosis
  • Intraoperative Complications / etiology
  • Intraoperative Complications / physiopathology*
  • Malignant Hyperthermia / diagnosis
  • Malignant Hyperthermia / metabolism*
  • Malignant Hyperthermia / physiopathology
  • Muscular Dystrophy, Duchenne / complications
  • Muscular Dystrophy, Duchenne / metabolism*
  • Muscular Dystrophy, Duchenne / physiopathology
  • Pediatrics*
  • Perioperative Care
  • Rhabdomyolysis / diagnosis
  • Rhabdomyolysis / etiology
  • Rhabdomyolysis / physiopathology*

Substances

  • Anesthetics, Inhalation