The Tulane Hypermobility and Ehlers-Danlos Syndrome (EDS) clinic team is here to help prevent surgical complications in patients with Ehlers-Danlos syndrome. The most common complications that we have noticed involve prolonged wound healing and scar tissue formation around the surgical site or intra-abdominal. Co-morbidities common with EDS include POTS, mast cell activation spectrum disorders (anaphylaxis, allergies to medications) and complex regional pain syndrome. Here are a few tips to help prevent complications when performing a surgical procedure on a patients with Ehlers-Danlos Syndrome.
Potential risks in patients with Ehlers-Danlos Syndrome
- Unstable C spine
- TMJ dislocation/subluxation
- Neurologic injury/compression
- Occipitalatlantoaxial instability, cervical instability (recommend pre-op cervical flexion and extension x-rays)
- Fragile mucosa/skin, (decreased)
- Lung injury/pneumothorax/shunt with PPV
- Increased risk of ocular injury, arterial/intestinal rupture
- Increased risk of poor wound healing
- Increased risk of hypotension/orthostasis secondary to dysautonomia (POTS), may need aggressive IV fluid use
- Opiates may not be effective. Consider high dose Tylenol, tramadol, neuropathic agents.
- Allergic reactions are common due to mast cell dysfunction. Consider pre-treatment with antihistamines/steroids.
- Aggressively manage pain.
- Benzodiazepines are helpful (stabilize mast cells).
- Gastroparesis. Aggressively use laxatives and optimize blood pressure.
Helpful peri-operative consultants: Immunology, cardiology, plastic surgery, local EDS-specialist
Helpful peri-operative labs/tests: echocardiogram, orthostatics/flexion-extension cervical spine x-rays
Check out other resources on our site, or other great information from the Ehlers-Danlos Society.