Anaesthesia for Children With Neuromuscular Disease

Highlights
- The only conditions shown to have a definite linkage with malignant hyperthermia (MH) are King–Denborough syndrome, central core disease, and Evans myopathy (View Highlight)
- The muscular dystrophies with an absence of dystrophin in the muscle cell have an unstable and more permeable sarcolemma. Inhalation agents and succinylcholine may increase the underlying instability and permeability of the sarcolemma, resulting in increased intracellular calcium levels and leakage of potassium and CK into the serum. (View Highlight)
- Succinylcholine is contraindicated in DMD. It has been implicated in producing intraoperative cardiac arrests secondary to rhabdomyolysis and hyperkalaemia. (View Highlight)
- In AIR, it is the instability of the sarcolemma that results in ‘leak’ of potassium and CK from necrotic and regenerating muscle cells into the serum. (View Highlight)
- previous uneventful volatile anaesthetic in an individual is not a guarantee of safety (View Highlight)