Medical and surgical precautions

I. Risk of bleeding.

) Warn the surgeon, dentist, obstetrician and ENT specialist (tonsillectomy, cervico-facial surgery) of the bleeding risk linked to Ehlers-Danlos syndrome, because of the fragility of the connective tissues, blood vessels and the disturbance of the adhesion/aggregation of blood platelets.

2) Avoid digestive endoscopies (especially colonoscopies, to be replaced if possible by colonoscan or virtual colonoscopy) and bronchial endoscopies, unless absolutely necessary. In this case, they must be carried out with great caution and possible bleeding must be prevented by tranexamic acid (Exacyl, 3x1000mg/day: from D-1 in the evening until D+1 in the evening) and vitamin C (3×1000 mg, from D-7 until D+2).

3) The use of non-steroidal anti-inflammatory drugs must be restricted and requires gastric protection (proton pump inhibitors).

4) Anticoagulants and platelet aggregation inhibitors should only be used when absolutely necessary, in the lowest possible doses and with very careful monitoring (ecchymosis, melena, etc.), constantly evaluating the benefit/risk ratio.

5) Rigid immobilisation after a sprain is useless and harmful (worsening of proprioceptive disorder, muscle wasting, risk of algoneurodystrophy, etc.). Resumption of activity in flexible restraints (adhesive restraints placed directly on the skin are forbidden because of the extreme fragility of the skin) and analgesics (local or even general) are treatments that can avoid anticoagulation.

II. Tissue fragility.

1) In case of soft tissue wounds or surgery, appropriate precautions should be taken: gentle gestures, non-absorbable sutures to be removed late and gradually (at least twice the normal time and remove every second or third suture first) to prevent suture loosening. Hemostasis should be checked several times at the end of surgery.

These precautions also apply to abdomino-pelvic surgery (anti-reflux surgery, bariatric surgery, abdominal hernia and ventricular surgery, incontinence and prolapse surgery, cystoceles and rectoceles, cholecystectomy, gynecologic surgery, etc.).).

2) In orthopedic surgery (knees, shoulders, hips, ankles, elbows, spine, etc.): avoid full stabilization procedures (e.g. arthrodesis).

The risk of algoneurodystrophy (CRPS-1) is very high in Ehlers-Danlos Syndrome, and prevention must be instituted in case of Mast Cell Activation Disorder (MCAD, MCAS, MCDs) or in case of a personal history of CRPS-1: anti-histamines, anti-leukotrienes, vitamin C, N-Acetylcysteine, PEA, possibly bisphosphonates, etc.

2020% of EDS patients develop CRPS-1 in their lifetime (S. Daens; P. Chopra).
85% of patients with CRPS-1 are EDS (P. Chopra).

3) In bone surgery, it is important to know that consolidation times (fractures, osteotomy) are prolonged. Bone grafts tend to "melt", especially when an anterior shoulder block is placed, which is contraindicated in multi-directional shoulder dislocations, which are frequent in this pathology.

4) Corticosteroids are contraindicated, except in cases of absolute necessity or in case of short treatment (2 to 3 days).

5) Never manipulate the cervical spine beyond 30°, because of the risk of lesion of the arteries irrigating the brain or of serious dislocation of the cervical vertebrae. Avoid violent or repeated manipulations (even active), which can cause intense and long-lasting pain.

6) Lumbar puncture should be avoided, unless absolutely necessary (frequent risk of meningeal breach). The same applies to epidurals and spinal anaesthesia, which, if necessary, must be carried out with care and followed rapidly by a blood patch treatment in case of accident.

7) Avoid arterial punctures (arterial gas measurements, arteriographies) unless absolutely necessary.

8) During an intravenous injection (injection, blood sampling, perfusion), the veins being fragile, they frequently rupture. The puncture point must be compressed well after the technical act, with the arm stretched out.

9) Care must be taken with electricity (TENS, neurophysiological examination, etc.): the thickness of the skin is reduced and its properties are modified, which increases its conduction capacity, the risk of accident and the manifestations of electrostatism.

III. In case of local or general anaesthesia.

is important to know that local anaesthesia (dental, skin) is often not very or not at all effective: it is advisable to double or triple the dose of anaesthetic, or even to resort to general anaesthesia in case of failure. The same applies to all surgery under local anaesthesia or spinal anaesthesia and epidurals in obstetrics.

In the case of general anaesthesia: be very careful with endotracheal intubation because of potential damage to the cervical spine (dislocation) and airways (tears).

Waking up may be premature in general surgery; sometimes it is even delayed, which may cause concern for the carers.

Pregnancy and childbirth precautions.

Plan on no contraindications: 

Exacyl 1000mg drinkable ampoules (adult), 3x/day from the morning before until 3 to 7 days after the operation (depending on the type of operation) 

Increase vitamin C to 3 x 1g/day from two weeks before (elective) surgery to one week after surgery and then back to 1g/day (increases platelet adhesion, among other things) 

Use non-absorbable sutures if possible (slower healing in EDS) 

Leave the external wires or staples in for two to three times the normal time and check for healing. Remove every second or third wire or staple first and apply steristrips if necessary. 

Provide two to three units of packed red blood cells in the operating room in case of childbirth or potentially bloody procedures. 

Anaesthesia is inconsistent and variable in duration (altered drug metabolism?), epidurals are most often partial or ineffective. 

Consider all pregnancies as high risk (HRG) with increased surveillance.

Check for a gap in the cervix and cerclage (if necessary) around 12-16 weeks gestation.

The incidence of miscarriage is increased (about 27-29%) but the average number of children per woman is broadly within the norm.

Premature rupture of the membranes is possible.

Early and ineffective uterine contractions are common and require the patient to rest.