EDS and surgeries


Ehlers-Danlos syndrome patients can be challenging for a variety of disciplines, especially concerning the operative and post-operative management.

Basically, EDS patients should be only operated on in appropriate centers with sufficient experience related to surgery and aftercare of EDS. Furthermore, the whole pre-, peri- and postoperative team should be familiar with the complexity of this disease.

Complications that could occur during surgery:

Anesthesia:

Many EDS patients report delayed, missing or even massively increased effects of local anesthesia (the latter one often in the presence of MCAS). This should be considered when choosing anesthesia.
Epidural anesthesia could cause spontaneous CSF leaks.
Be careful with muscle relaxants in patients with muscle weakness.
Avoid invasive monitoring (for example CVC), especially in patients with increased risk of vessel rupture.
Use tourniquets cautiously because of hematoma/bleeding.

Positioning:

Avoidance of shear forces.
Place the joints well padded to avoid dislocation of the joints. (Plexus neuropathy, eye damage, hematoma, skin damage can occur due to incorrect positioning).

Airway management:

Avoid temperomandibular dislocations.
Do not damage connective tissue during intubation (use a smaller tube if necessary).
Risk of pneumothorax! Keep pressure low.
Particular caution should be taken in patients with Chiari/cervical spine instability, Dysautonomia and MCAS.

Wound healing and stitching:

Wound healing could be delayed or very bad; Stitches should stay longer than normal in EDS patients.

Bleeding:

Many EDS patients are suffering from vascular fragility or increased and prolonged bleeding. Coagulation medication could be given prophylactically if necessary. However, the vascular type of EDS has the highest risk of bleeding.
Blood clotting studies are recommended!

Skin:

Plaster allergy?
Tearing of the skin?

Additional risk factors may arise from comorbidities of EDS. Those comorbidities might complicate operations even further and must of course be taken into account with. For example mitral valve regurgitation, muscle weakness, or MCAS and POTS.

Postoperative monitoring:

Hematoms? Muscle weakness?
EDS should be particularly noted when it comes to mobilization after surgery. On the one hand it is important to start early with reconditioning because EDS patients lose muscles very quickly but on the other hand subluxations must be avoided.

Anesthesia recommendations for patients suffering from Ehlers-Danlos syndrome
Wiesmann, Malfait, Castori
https://www.orpha.net/data/patho/Pro/en/Ehlers_Danlos_En.pdf

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