Case report: Death in the emergency department: an unrecognized awake malignant hyperthermia-like reaction in a six-year-old

Anesth Analg. 2013 Feb;116(2):420-3. doi: 10.1213/ANE.0b013e3182768f99. Epub 2012 Dec 24.

Abstract

A healthy 6-year-old boy developed lower extremity rigidity, trismus, and fever after playing in a splash pool. On arrival in the emergency department, he appeared to be seizing. An endotracheal tube was emergently placed using succinylcholine. Cardiac arrest followed. He could not be resuscitated. Postmortem genetic analysis found a novel RYR1 variant. Family testing revealed the same variant in his father who also had muscle contracture testing diagnostic for susceptibility to malignant hyperthermia and central core disease diagnosed histologically. Because there was no exposure to volatile anesthetics before the onset of symptoms, this is a case of "awake" malignant hyperthermia worsened by succinylcholine.

Publication types

  • Case Reports
  • Research Support, U.S. Gov't, Non-P.H.S.

MeSH terms

  • Child
  • Diazepam / adverse effects
  • Emergency Medical Services*
  • Emergency Service, Hospital*
  • Fatal Outcome
  • Humans
  • Hypnotics and Sedatives / adverse effects
  • Intubation, Intratracheal
  • Liver / chemistry
  • Lorazepam
  • Male
  • Malignant Hyperthermia / pathology
  • Malignant Hyperthermia / physiopathology*
  • Muscle Relaxants, Central / adverse effects
  • Muscle Rigidity / chemically induced
  • Myopathy, Central Core / genetics
  • Neuromuscular Depolarizing Agents / adverse effects
  • Respiratory Distress Syndrome / chemically induced
  • Ryanodine Receptor Calcium Release Channel / genetics
  • Succinylcholine / adverse effects

Substances

  • Hypnotics and Sedatives
  • Muscle Relaxants, Central
  • Neuromuscular Depolarizing Agents
  • Ryanodine Receptor Calcium Release Channel
  • Succinylcholine
  • Lorazepam
  • Diazepam