Tracheotomy is a major step for patients with neuromuscular disease as it requires a higher level of care, which affects quality of life and increases dependence. Intolerance or nonfeasibility of noninvasive ventilation (NIV) is the most common reason for tracheotomy of patients with neuromuscular disease. Other reasons include worsening of respiratory failure with a low vital capacity (VC), persistent hypercarbia, and the need to increase ventilation time. One major condition to realize tracheotomy is to be able to maintain efficient ventilation throughout a procedure while providing efficient analgesia. Due to their severely impaired pulmonary function, respiratory management of patients with neuromuscular difficulties is very challenging. These individuals are at risk of severe complications when they require deep sedation or anesthesia with endotracheal intubation. They present many factors that can lead to difficult intubation: skeletal deformities, tracheal deviation related to kyphoscoliosis, reduced neck mobility due to cervical fusion or myopathy, tongue hypertrophy, and reduced mouth opening. The main risk in case of difficult intubation is a delay in efficient airway protection; adverse events in this setting include desaturation, hypoxemia, aspiration pneumonia, and prolonged stays in the intensive care unit (ICU) or hospital. Muscle relaxants and sedative anesthetic agents may induce severe respiratory depression and inefficient cough; moreover, muscle relaxants may cause rhabdomyolysis with fatal hyperkalemia [1]. A heightened maximal effect of vecuronium has been reported in patients with Duchenne muscular dystrophy (DMD), resulting in a large increase of time to recovery. Sedative agents can induce severe hypotension in patients with cardiac involvement, as seen in DMD; the potential depressive effect of sedatives on cardiac contractility must be considered when used in patients with cardiac dysfunction and at risk of life-threatening cardiac dysrhythmia.
