1. Morquio A: Anesthetic considerations

2. Morquio A patients are at high risk of anesthesia-related morbidity and mortality due to: –Cervical instability and myelopathy –Compromised respiratory function  Upper and lower airway obstruction  Restrictive lung disease –Cardiac abnormalities Any elective surgery requires: –Thorough pre-operative ENT, pulmonary and cardiac evaluations –Pre-operative radiological assessment of the cervical spine –Skilled personnel in airway management –Spectrum of airway management equipment Morquio A patients should be managed by experienced anesthesiologists at centers familiar with MPS disorders Airway and anesthetic management of Morquio A patients presenting for surgery is challenging Theroux et al, Paediatr Anaesth, 2012; Solanki et al, J Inherit Metab Dis, 2013; Walker et al, J Inherit Metab Dis, 2013; McLaughlin et al, BMC Anesthesiol, 2010; Morgan et al, Paediatr Anaesth, 2002; Shinhar et al, Arch Otolaryngol Head Neck Surg, 2004; Belani et al, J Ped Surg, 1993; Walker et al, Anaesthesia, 1994

3. Intubation Anticipate potential problems –Difficulties with intubation and ventilation due to:  upper airway obstruction, restricted mouth opening, enlarged tongue, chest wall deformities and short neck with limited range of motion –Skilled personnel in airway management and difficult airway equipment should be readily available Correct positioning of the Morquio A patient is critical: –ensure neutral position of neck by aligning mastoid process with the clavicle –minimize flexion and extension movements Anterior displacement of tongue by manual retraction facilitates access to the larynx Monitor SSEPs if spinal cord compromise is a concern Theroux et al, Paediatr Anaesth, 2012; Solanki et al, J Inherit Metab Dis, 2013; Walker et al, J Inherit Metab Dis, 2013

4. Ventilation Spontaneous ventilation techniques using oxygen and a high- concentration volatile anesthetic are commonly employed Use of a laryngeal mask airway (LMA) or nasal airway can improve ventilation Use of a single dose of methylprednisolone prophylactically to prevent intraoperative bronchospasm and mucosal swelling of airway may be considered Ketamine may be used to maintain bronchodilation at light levels of anesthesia Theroux et al, Paediatr Anaesth, 2012; Solanki et al, J Inherit Metab Dis, 2013; Walker et al, J Inherit Metab Dis, 2013;

5. Extubation Operating room or intensive care unit? –Consider:  difficulties associated with initial intubation and intraoperative course  pre-existing respiratory illness  pre-existing myelopathy  halo placement Reverse neuromuscular blockers Monitor for early signs of upper airway obstruction and oxygen desaturation Anticipate need for re-intubation Postoperative swelling of buccal mucosa and lips may be reduced by application of steroid cream Theroux et al, Paediatr Anaesth, 2012; Solanki et al, J Inherit Metab Dis, 2013; Walker et al, J Inherit Metab Dis, 2013