68yo lady with progressive mitochondrial disease with neuropathic bladder, for botox injections, cystoscopy and SPC.

Background

• Progressive mitochondrial disease
  • Looked after through mitochondrial dx clinic at RNSH
  • Mostly wheelchair bound
  • Nil known CVS/RS complications
  • Spirometry in clinic somewhat reassuring, values >50%
  • Not a variant strongly associated with refractory seizures
  • Nil reported dysphagia
  • Known to palliative care
• HTN
• Depression

Issues

• Anaesthetic technique?
  • Excellent perioperative management guidelines from Newcastle-Upon-Tyne https://www.newcastle-mitochondria.com/wp-content/uploads/2016/03/Anaesthesia-Peri-Operative-Care-Guidelines.pdf
  • Above guidelines suggest:
    • Greatest perioperative risks derive from the severity of the patient’s pre-existing CVS/RS/CNS dx components
    • No specific anaesthetic technique or drug contraindicated
    • Not associated with MH
    • Chronic bowel dilatation and GI dysmotility puts these patients at risk of severe complications post-op due to opioids. Techniques to minimize opioids are ideal and prescribed bowel care necessary.
    • Baseline CK and lactate levels help to stratify severity of disease and to identify dynamic changes postop.
    • Increased VTE risk due to immobility

Plan

• Proceed with surgery
• Preop TTE as arranged by clinic
• Group consensus was that a short acting SAB would be ideal (? Prilocaine) 
• Procedural anaesthetist notified.