Anaesthesia in myotubular (centronuclear) myopathy

A patient with a known history of myotubular myopathy presented for surgery for insertion of a tibial nail. Anaesthesia was induced and maintained usi ng an intravenous anaesthetic technique. Neuromuscular function was assesse d using mechanomyography, which showed a profound reduction in muscle contr actility. In view of this, the use of muscle relax-ants was avoided altoget her. Nerve conduction was normal but electromyography showed small motor un its, with generalised distribution, suggesting mild to moderately severe my opathy The patient made a slow but uneventful recovery.