Malignant hyperthermia and muscular dystrophies.
BACKGROUND
Patients with muscular dystrophy have been reported to experience a variety of life-threatening complications during and after general anesthesia. We performed a systematic analysis to define the spectrum of anesthetic-related complications in patients with muscular dystrophy, with an emphasis on malignant hyperthermia susceptibility.


METHODS
A literature search was undertaken using multiple search engines and the appropriate articles were reviewed by the authors to determine anesthetic-associated complications in patients with muscular dystrophy. Of all the types of muscular dystrophy, Duchenne muscular dystrophy (DMD) and Becker dystrophy (BD) represent nearly all the anesthesia-related reports.


RESULTS
Anesthetic complications in patients with DMD and BD include intraoperative heart failure, inhaled anesthetic-related rhabdomyolysis (absence of succinylcholine), and succinylcholine-induced rhabdomyolysis and hyperkalemia.


CONCLUSIONS
We did not find an increased risk of malignant hyperthermia susceptibility in patients with DMD or BD compared with the general population. However, dystrophic patients who are exposed to inhaled anesthetics may develop disease-related cardiac complications, or rarely, a malignant hyperthermia-like syndrome characterized by rhabdomyolysis. This latter complication may also occur postoperatively. Succinylcholine administration is associated with life-threatening hyperkalemia and should be avoided in patients with DMD and BD.
Gurnaney
Harshad
H
Department of Anesthesiology and Critical Care Medicine, The Children's Hospital of Philadelphia, 34th St. and Civic Center Blvd., Philadelphia, PA 19104-4399, USA. gurnaney@email.chop.edu
Brown
Amanda
A
Litman
Ronald S
RS
D000293:Adolescent; D000758:Anesthesia / Q000009:adverse effects*; D018685:Anesthetics, Inhalation / Q000009:adverse effects; D002648:Child; D002675:Child, Preschool; D019317:Evidence-Based Medicine; D005260:Female; D006333:Heart Failure / Q000209:etiology; D006801:Humans; D006947:Hyperkalemia / Q000209:etiology; D008297:Male; D008305:Malignant Hyperthermia / Q000209:etiology* / Q000517:prevention & control; D020388:Muscular Dystrophy, Duchenne / Q000150:complications*; D009467:Neuromuscular Depolarizing Agents / Q000009:adverse effects; D012206:Rhabdomyolysis / Q000209:etiology; D018570:Risk Assessment; D012307:Risk Factors; D013390:Succinylcholine / Q000009:adverse effects
D018685:Anesthetics, Inhalation; D009467:Neuromuscular Depolarizing Agents; D013390:Succinylcholine