PIG Meeting: 25th February 2021
34yo patient for elective CS, complex chronic and acute health problems.
- Ehlers Danlos Syndrome
- Hypermobility type
- ‘Marfans features’ (but not Marfans) with lens dislocation, high arched palate, spontaneous L PTx and known small bullae on R.
- Bowel and gastric stasis requiring caecostomy.
- Central and obstructive OSA – adherent to CPAP
- BMI 38
- Uveitis HLAB27 +
- Raynaud’s phenomenon
- Pregnancy – G1P0, nil foetal complications.
- PE – clinical symptoms + V/Q mismatch. On therapeutic clexane since ~10/7
- ? Risk of aortic root aneurysm
- Somewhat unusual picture. Risk difficult to quantify
- Minimal harm from TTE (n.b. normal aortic root on subsequent TTE).
- Risk of local anaesthetic failure
- Patient has previously had some failed peripheral nerve blocks but successful rescue blocks
- What is Ehlers Danlos Syndrome?
- The most common group of disorders in the family of genetically determined heritable connective tissue disorders.
- Ligament laxity, joint hypermobility, fragility of connective tissues, poor wound healing.
- Multiple subtypes with cardiac, GIT, autonomic and chronic pain issues.
- Anaesthetic technique
- Morphine adverse drug reaction (rash).
- Neuraxial ideal. Consider CSE as colorectal team on standby given previous bowel surgery.
- TAP catheters useful to avoid opioid exacerbation of gut stasis postop + risks of resp depression with opioids. Some reports of ineffective or partially effective LAs however not contraindicated and may provide substantial benefit.
- If GA required, lung protective ventilation to minimise risk of bullae rupture.
- Note that Ehlers Danlos patients also have increased risk of bleeding post-operatively
- Postoperative location (? Delivery suite, ? K2)
- High VTE risk postop however this risk continues for weeks so no specific observation needed.
- OSA well optimised
- Normal care on K2 reasonable
- Anticoagulation management
- Discussed ? Need for conversion to heparin given known PE, to minimise time off anticoagulation
- Overall thought that 24h off clexane reasonable and the logistics in starting/stopping heparin make it of limited value for this short period of time.