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Death in the Emergency Department: An Unrecognized Awake Malignant Hyperthermia-Like Reaction in a Six-Year-Old

Lavezzi, Wendy A. MD*; Capacchione, John F. MD; Muldoon, Sheila M. MD; Sambuughin, Nyamkhishig PhD; Bina, Saiid PhD; Steele, Deanna MS, CGC; Brandom, Barbara W. MD§‖

doi: 10.1213/ANE.0b013e3182768f99
Pediatric Anesthesiology: Case Report

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.

From the *Office of the District 5 Medical Examiner, Leesburg, Florida; Department of Anesthesiology, Uniformed Services University of the Health Sciences, Bethesda, Maryland; Center for Medical Genetics and Department of Anesthesiology, University of Pittsburgh, Pittsburgh, Pennsylvania; and §North American Malignant Hyperthermia Registry (NAMHR) of the Malignant Hyperthermia Association of the United States.

Accepted for publication September 24, 2012.

Supported by the Defense Medical Research and Development Program, award no. W81XWH-10-2-0117.

This report was previously presented, in part, at the ANESTHESIOLOGY 2011 meeting, which was the subject of an article in ASA Daily News at ANESTHESIOLOGY 2011.

The authors declare no conflicts of interest.

Reprints will not be available from the authors.

Address correspondence to John F. Capacchione, MD, Department of Anesthesiology, Uniformed Services University of the Health Sciences, USUHS4301 Jones Bridge Rd., Bethesda, MD 20814. Address e-mail to jcapacchione@usuhs.mil.

© 2013 International Anesthesia Research Society