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In absence of definitive diagnosis, the goal is an anesthetic technique that minimises the risk of rhabdomylosis, malignant hyperthermia, & propofol infusion-syndrome for which these children may be at risk. Volatile anesthetics are ideally avoided in muscular dystrophies, & propofol in high and prolonged doses is ideally avoided in mitochondrial disorders. Short duration propofol infusion is generally a safe option for either. refl.in/fv

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Enquire about family history of muscular dystrophies or related disorders.

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Has the patient had a previous anesthetic? If so, acquire details.

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Minimise the preoperative fasting period to avoid hypovolemia and depletion of glucose stores. refl.in/fv

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The estimated risk of a patient with suspected neuromuscular disorder developing MH as a result of exposure to volatile anesthetic agents during muscle biopsy is 1.09% or less. refl.in/fx This risk can be reduced by choosing a total intravenous-anesthesia technique, keeping in mind that children with mitochondrial disorders may be at higher risk of propofol infusion-syndrome. refl.in/fv

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If there is a suggestion that the condition may be muscular dystrophy (family history, hypertrophied calves despite generalised hypotonia, elevated CK), consider avoiding volatile anesthetic agents to minimise the risk of rhabdomyolisis. refl.in/fw

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In prolonged cases, minimise the risk of propofol infusion syndrome by using remifentanil, nitrous oxide and ketamine. #ref

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Intravenous fluids should contain glucose and avoid additional lactate that might worsen an underlying lactic acidosis. refl.in/fv

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Avoid suxamethonium to minimise the risk of hyperkalemia #ref

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Anaesthesia and the paediatric patient with neuromuscular disease. S Afr J Anaesthesiol Analg
21 2010;16(1)
www.sajaa.co.za/index.php...

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Anaesthesia for children with neuromuscular disease
Contin Educ Anaesth Crit Care Pain (2010) 10 (5): 143-147.
dx.doi.org/10.1093/bjacea...

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