Table 3.

Challenges and resolutions associated with surgery

Challenges	Resolutions
Cardiac valve replacement
• Initial intubation resulted in high CO2 pressure • Nasal intubation via right nostril also resulted in high CO2 pressure	• Paediatric and adult anaesthetists with experience in MPS disorders present • Intubation via left nostril successful
• Patient’s short neck made central line insertion difficult	• Ultrasound-guided central line insertion by paediatric anaesthetist
• Pericardial adhesions from previous mitral valve replacement surgery at the age of 24 years • MPS-associated valvular pathology	• Adhesions removed • Fibrous tissue, calcification and GAGs removed from mitral valve
• Physically small patient	• Paediatric catheters used to remove excess blood from ventricles • Smallest adult replacement valve used (size 19 mm CarboMedics Top Hat® mechanical prosthesis)
• Tracheostomy required because of narrow trachea, but difficult for paediatric and adult anaesthetists to perform	• ENT surgeon assisted • Pre-surgery 3D CT of chest and trachea, and fluoroscopy results were used to identify optimal site
Spinal decompression
• No cardiology expertise in hospital performing surgerya	• Medical files provided by the treating doctor • Surgeons discussed surgery with treating doctor to understand MPS-specific requirements
• Patient and family did not wish ERT to be interrupted by surgerya	• ERT infusions arranged to occur during recovery at hospital performing surgery
• Patient had a short stature and restricted respiratory functionb	• Neurosurgeon had extensive experience in paediatric patients
Corneal transplant
• High cardiovascular risk	• Pre-surgery cardiac and respiratory function tests
• Risk that patient may not tolerate procedure or epithelium may be pierced	• Make preparations in case general anaesthesia is required
• Risk of graft rejection	• Endothelium preserved, resulting in reduced risk

^aCase 2. ^bCase 3