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Table 3 Challenges and resolutions associated with surgery

From: Critical clinical situations in adult patients with Mucopolysaccharidoses (MPS)

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
  1. aCase 2. bCase 3