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Table 7 Guidance statements for anaesthesia

From: Recommendations for the management of MPS VI: systematic evidence- and consensus-based guidance

Statement Percentage consensus
Pre-, intra- and post-operative care (until extubation is complete) for all procedures requiring general anaesthesia, conscious or deep sedation, should be supervised by an anaesthetist with experience in treating patients with MPS and/or complex airway management. In addition, the anaesthetist should have access to Intensive Care support and be surrounded by an experienced team capable of performing emergency tracheotomy if required
Evidence Grade: C (level 3/4 studies)
98%
A full assessment of the risks and benefits should take place with the patient and family prior to any procedure. All pre-operative information should be made available to allow decision making
Evidence Grade: C (level 4 study and extrapolation from level 3 study)
100%
ENT respiratory, cardiac, and radiological assessment should be performed prior to any procedure requiring anaesthesia
Evidence Grade: C (level 3 study and extrapolation from level 3 study)
93%
It is critical to maintain a neutral neck position during all surgeries, and during intubation and extubation to avoid paralysisa. Strongly recommend the use of techniques that allow maintenance of the neutral neck position, including use of laryngeal mask airway (LMA) for shorter procedures, or intubation with a video laryngoscope or fibreoptic intubation
Evidence Grade: C (level 3/4 studies)
87%
Pre-operative and intra-operative measures to avoid hypotension should be adopted during all surgical procedures in patients with MPS VI to maintain spinal cord perfusion and therefore protect spinal cord function
Evidence Grade: D (expert clinical opinion)
98%
Intra-operative neurophysiological monitoring (including somatosensory evoked potentials [SSEP], electromyography [EMG] and motor evoked potentials [MEP]) is strongly recommended during all spinal surgeries and other potentially lengthy or complicated procedures, including those that require manipulation of the head and neck
Evidence Grade: D (limited published evidence)
94%
For other surgeries and procedures, neurophysiologic monitoring should be considered based on pre-existing risk for spinal cord compression and instability, need for spine manipulation, possibility of hemodynamic changes and blood loss, or extended length of time
Evidence Grade: D (limited published evidence)
94%
Intrathecal and epidural techniques should be used with extreme caution in patients with MPS VI, due to the anatomical challenges of very short stature, as well as spinal abnormalities causing insertion problems and unpredictability of spread of local anaesthesia. However, these techniques may be considered to avoid general anaesthesia in a high-risk situation or during pregnancy
Evidence Grade: D (expert clinical opinion)
88%
  1. Post-consensus comments by the SC to be taken into consideration
  2. aIt is critical to maintain a neutral neck position to avoid any spinal cord injury