Morquio A: Anesthetic considerations

1. Morquio A: Anesthetic considerations

2. Airway and anesthetic management of Morquio A patients presenting for surgery is challenging • 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 Theroux et al, PaediatrAnaesth, 2012; Solanki et al, J Inherit MetabDis, 2013; Walker et al, J Inherit MetabDis, 2013; McLaughlin et al, BMC Anesthesiol, 2010; Morgan et al, PaediatrAnaesth, 2002; Shinhar et al, Arch Otolaryngol Head Neck Surg, 2004; Belani et al, J PedSurg, 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, PaediatrAnaesth, 2012; Solanki et al, J Inherit MetabDis, 2013; Walker et al, J Inherit MetabDis, 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 methylprednisoloneprophylactically to prevent intraoperativebronchospasm and mucosal swelling of airway may be considered • Ketamine may be used to maintain bronchodilation at light levels of anesthesia Theroux et al, PaediatrAnaesth, 2012; Solanki et al, J Inherit MetabDis, 2013; Walker et al, J Inherit MetabDis, 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, PaediatrAnaesth, 2012; Solanki et al, J Inherit MetabDis, 2013; Walker et al, J Inherit MetabDis, 2013