Andrius Macas
Abstract
Summary
Myotonia is an abnormal delay in the relaxation of muscles after contraction. It is a key symptom in a number of muscle diseases called myotonic disorders [1, 19, 20, 24, 26].
Myotoniacongenita is an inherited disorder of skeletal muscle excitability. It is caused by mutations in the muscle chloride channel gene. The disorder is characterized by myotonia and muscular hypertrophy due to prolonged involuntary activation [20, 23].
This case report describes the anaesthetic management in a 39 year-old patient with myotoniacongenita scheduled for femur osteosynthesis performed under spinal anaesthesia. The international literature does not elucidate clear preference toward a particular anaesthesia technique, but we believe that in this setting, central regional anaesthesia and complete monitoring are desirable. In any case, the anaesthesia plan should be prudent and avoid exposure to known dangerous anaesthetics.
Keyword(s): Anaesthetic complications, malignant hyperthermia, myotonia congenita, Thomsen‘s myotonia.
DOI: 10.5200/152
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