1. Airway involvement in MPS can be multilevel and progressive.
  2. In MPS 4 tracheal narrowing can happen due to disproportionate growth between the trachea and the skeleton.
  3. In MPS 1 & 2 airway compromise can result from the collection of GAG deposits in the lining of the airway.
  4. Airway obstruction in MPS can involve anywhere from ‘lips to lungs‘.
  5. Obstructive Sleep Apnoea (OSA) is more common in MPS.
  6. Patients with MPS can have unstable necks, so don’t over-extend the neck when examining the airway.
  7. Removing the adenoids and tonsils is usually the first operation to improve the airway in MPS.
  8. Airway problems in MPS may be made worse by co-existing heart, lung and skeletal problems.
  9. Poor mouth opening is emerging as a problem in MPS as patients live longer, caused by bony over-growth of the coronoid process of the mandible.
  10. If your hospital isn’t experienced in the management of the airway during anaesthesia and surgery, then send to a hospital that is…don’t take a chance that you will encounter the ‘can’t ventilate, can’t intubate scenario‘.