The place of surgery in the SED

Some people with Ehlers-Danlos syndrome are multi operated (40 times in one of our cases).

This multiplication of interventions is a clear indication of their uselessness in most cases.

The connective tissues of Ehlers-Danlos Syndrome, do not tolerate tendon transfers and other soft tissue interventions. Knee surgery results in failures.

Shoulder stops are failures.

In addition, osteolysis is observed.

Interventions for pseudoherniated discs, which are actually images produced by the flexibility of the intervertebral discs, usually result in increased pain.

Surgery retains indications in shoulder dislocations, peroneal tendon dislocations, fingers, sometimes.

As regards the abdomen and pelvis, here too, one must be very careful about the risk of failure, suture loosening, bleeding.

A special case: vesicular stones that threaten to perforate a fragile vesicle

At the slightest suspicious sign of complication, intervention is required.

Should it be done preventively?

Pneumothoraxes are very rare, surgery seems to give good results in the few cases we have followed.

Bariatric surgery is beginning to take its place in major obesities that are rebelling against drug (medformin) and dietary treatments.

 

ENT

 

Ear infections are common, especially in early childhood and require local treatment and iterative antibiotics.

Hyperacusis, tinnitus, hyperosmia create situations of handicap for which solutions have to be found.

Hearing loss can be fitted.

 

Dental care

 

Many maxillofacial and dental problems arise:

  • ineffective dental anesthesia,

  • bleeding,

  • gingivitis, fragility,

  • mobility, dental,

  • anarchic dental growth.

Orthotics and orthodontics have a place here adapted to the very particular characteristics of the syndrome.

 

 back to "SED CARE

Precautions during surgery for Ehlers-Danlos Syndrome:

  • Avoid intra-operative and post-operative bleeding :
    • Take Exacyl 500 (children) or 1000 mg (adults), three times a day from the day before surgery in the morning until 3 to 7 days after surgery (depends on the surgery: wisdom teeth 2 days, appendectomy or cholecystectomy (5 days), Aorta or other heavy abdominal surgery (7 days).
    • Increase vitamin C intake (which is taken continuously in Ehlers-Danlos Syndrome): Take 3 x 1 gram per day from 7 days before a scheduled procedure until 7 days after the procedure (then decrease to 1 g/day). Vitamin C increases the adhesion of the platelets and thus reduces the risk of per- and post-operative bleeding.
    • In case of heavy intervention, plan to infuse Desmopressin 0.3 mcg /Kg body weight, to be run in 30 min, one hour before the intervention. It increases factor VIII and von Willebrand factor, it increases the adhesion of blood platelets to the vessel walls, it decreases APTT and bleeding time.
    • The injection of recombinant activated factor VII (Novosen) should be discussed on a case-by-case basis.
  • Surgical sutures to be used preferentially?
    • If possible, NON-resorbable surgical sutures should be used. The healing time of the tissues may be slower than the resorption of resorbable sutures, which may result in dehiscence of the internal (and external) tissues.
  • The position of the patient during surgery?
    • Care must be taken to avoid any position that could lead to tissue, joint or cervical lesions, elongation of the nervous plexus, etc.
  • Endotracheal intubation?
    • It must be done with caution, on the one hand to avoid any perforation or laryngeal or tracheal lesion, and, on the other hand, any lesion of the cervical spine during hyper-extension of the cervical spine during intubation.
  • The anesthesia?
    • Epidurals are most often partial or ineffective (the same applies to dental anaesthesia). You should be wary of meningeal breaches and be prepared for a blood-patch to seal the breach. In the case of general anaesthesia, the patient may wake up too early (during the operation) or later (causing anxiety for the caregivers). This may be due to a different metabolism of anaesthetic substances in the connective tissue that is altered by Ehlers-Danlos Syndrome.
  • When to remove external wires or staples ?
    • It takes twice the normal time to remove external wires or staples. First remove every second or third thread or staple. If the healing is incomplete, use "steristrips" and delay the removal of the threads or staples.