CJA
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
This Article
Right arrow Full Text (PDF)
Right arrow Submit a scholarly reply
Right arrow Alert me when this article is cited
Right arrow Alert me when eLetters are posted
Right arrow Alert me if a correction is posted
Services
Right arrow Similar articles in this journal
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by WANG, J. M.
Right arrow Articles by STANLEY, T. H.
Right arrow Search for Related Content
PubMed
Right arrow Articles by WANG, J. M.
Right arrow Articles by STANLEY, T. H.

Canadian Journal of Anesthesia, Vol 33, 492-497, Copyright © 1986 by Canadian Anesthesiologists' Society

Duchenne Muscular Dystrophy and Malignant Hyperthermia Two Case Reports

JANICE M. WANG MD1 and THEODORE H. STANLEY MD1

1 Department of Anesthesiology, University of Utah School of Medicine, Salt Lake City, Utah

Address correspondence to: Dr. Theodore H. Stanley, Department of Anesthesiology, University of Utah School of Medicine, 50 North Medical Drive, Salt Lake City, Utah 84132.

The case histories are presented including the anaesthetic and postoperative management, of two children, a two-year-old with undiagnosed Duchenne muscular dystrophy (DMD) and a three-year-old with known DMD. The child with undiagnosed DMD had no symptoms of DMD and had received halothane twice before, without succinylcholine, with no apparent difficulty. Following an uneventful induction of anaesthesia with halothane, nitrous oxide and O2, succinylcholine resulted in bilateral masseter muscle spasm and then, in rapid sequence, ventricular tachycardia and cardiac arrest. Resuscitation was difficult, prolonged and associated with hyperkalaemia (K+ = 12.57 mEq·L-1), severe metabolic and respiratory acidosis, high peripheral venous pressure and massive hepatospleenomegaly, but not hyperthermia. The patient was finally resuscitated but died two days later. Skeletal muscle biopsy results were consistent with malignant hyperthermia. The second patient was known to have DMD but did not receive prophylactic or intraoperative dantrolene nor have his anaesthetic machine flushed with oxygen for an extended period prior to induction of anaesthesia. This child was anaesthetized with fentanyl and N2O and, with the exception of a high intraoperative heart rate (155-160 beats·min-1), had an uncomplicated anaesthetic and operation (intraoperative axillary temperatures ranged between 36.8-37.9° C). Postoperatively his temperature rapidly increased to 38.8° C and then 40.3° C and he became metabolically acidotic. Intravenous administration of dantrolene for 48 hours reduced the temperature and allowed normal recovery and discharge. A postoperative muscle biopsy was consistent with DMD. These case reports confirm that children with DMD have the potential for developing malignant hyperthermia during or after anaesthesia and therefore should be prepared preoperatively and managed intraoperatively accordingly.

Key Words: ANAESTHESIA: paediatric • ANAESTHETICS: succinylcholine, halothane • COMPLICATIONS: cardiac arrest, hyperkalaemia, hyperthermia • GENETIC FACTORS: Duchenne's muscular dystrophy






HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
Copyright © 1986 by the Canadian Anesthesiologists' Society.