Emergency Information for Medical Professionals

Vascular Ehlers Danlos Syndrome

Vascular EDS is a life threatening connective tissue disorder that affects all tissues, arteries and internal organs making them extremely fragile. Patients are at risk of sudden arterial or organ rupture. This can occur at any age. Mid-size arteries are commonly involved.

Patient concerns should be taken seriously and any reports of pain need full and immediate investigation.

Presenting symptoms

• Arterial or intestinal rupture commonly presents as sudden acute abdominal, chest or pelvic pain that can be diffuse or localised.

• Cerebral arterial rupture may present with altered mental status and be mistaken for drug or alcohol use.

• Redness, pain and prominence of one or both eyes and the sound of pulsations in the head can be manifestations of a carotid-cavernous fistula.

• Coronary dissection may present with acute myocardial infarction. Carotid dissection may present with stroke. Limb arterial dissection can present with acute limb ischaemia or claudication.

• Colonic perforation can present with acute abdominal pain and/or signs of infection.

• Pneumothorax can present with pain and shortness of breath.

Management guidance

The fragility of all tissues means that invasive procedures should be avoided where possible. All members of the medical team should be aware of the potential risk for greater than usual harm.

• Vascular dissection and rupture or bleeding can be subtle in presentation, therefore a lower threshold for investigations and imaging is indicated.

• Immediate investigation by MRI or CT scan should be performed.

• Use non-invasive techniques only, avoiding stress and tension on skin, organs or vessels during physical examination.

• Avoid angiography, enemas and endoscopies.

• Avoid intramuscular or subcutaneous injections of heparin or heparin substitutes, as these can cause massive subcutaneous haematoma and bruising.

• Central lines should be placed only with ultra sound guidance to avoid inadvertent arterial injuries.

• Fluoroquinolones/quinolones are a group of antibiotics that should be avoided if possible.

Emergency surgery

Surgical risks are higher for Vascular EDS patients. The threshold for intervention should be higher. All conservative management options should be carefully considered before surgery.

• The primary indication for surgical intervention is life threatening complications of arterial or organ rupture.

• A vascular surgeon should be present during surgery.

• The anaesthetist should be aware of fragile mucus membranes when intubating.

• Self retaining retractors should be used carefully, excessive retraction leads to multiple tissue tears and haematomas.

• Tissues are fragile and do not hold sutures well.

Signs and symptoms that require medical attention

• Severe pain in the head, neck or abdomen which may be diffuse or localised.

• Sudden onset of bleeding.

• Unexplained swelling of a limb.

• Symptoms of a stroke (drooping of the face, arm weakness, slurred or unclear speech).

• Weakness of the limbs.

• Dizziness or loss of consciousness.

• Shortness of breath and/or difficulty breathing or speaking.

• Visual disturbance.

• Redness, pain and prominence of one or both eyes.

• The sound of pulsations in the head.

• A large bruise increasing in size.

Vascular EDS is a serious condition that is associated with a risk of sudden arterial or organ rupture at any age.

Version 4. Review date: 01/07/2026

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