68yo lady with progressive mitochondrial disease with neuropathic bladder, for botox injections, cystoscopy and SPC.
- 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
- 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
- Proceed with surgery
- Preop TTE as arranged by clinic
- Group consensus was that a short acting SAB would be ideal (? Prilocaine)
- Procedural anaesthetist notified.