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Emergency Situations

If you are having a medical emergency, call 911.

The following are signs of some of the medical emergencies associated with Vascular Ehlers-Danlos Syndrome (VEDS). Other emergencies may occur that may not be listed here.

People with Vascular Ehlers-Danlos Syndrome are at increased risk for emergencies involving the aorta, eyes, arteries, lungs, uterus, and hollow organs (like the intestinal tract).

Emergencies Associated with Vascular Ehlers-Danlos Syndrome

Arterial Dissection

Arterial dissection is a tear between the layers of the artery wall. This can occur in any artery in the body in someone with VEDS. Aortic dissection is a tear between the layers of the aorta, the large blood vessel that carries blood away from the heart. An aortic dissection can be fatal if not treated immediately.  Any arterial dissection can be an emergency and evaluation is needed immediately. Symptoms include:

  • Sudden onset pain; may be “severe,” “sharp,” “tearing,” or “ripping” and may be located anywhere in the body, depending on which artery is affected. Sometimes, the pain is less severe, but a person still has a feeling that “something is very wrong.”
  • Nausea
  • Shortness of breath
  • Fainting
  • Loss of pulse
  • Tingling, numbness, burning, prickling of the skin (parasthesia)
  • Paralysis

The presentation of an artery dissection may be very different depending on where the artery dissection occurs.

  • If the dissection occurs in the arteries in the head or neck, pain may be accompanied by stroke symptoms, including a droopy eyelid with a small pupil on the same side. A room spinning sensation or dizziness may be present, as well as difficulty seeing.
  • If the dissection occurs in the coronary artery, the symptoms may be similar to or cause a heart attack, or cause abnormalities in heart rhythm.
  • If the dissection occurs in the aorta, severe pain usually in the chest (front, back or both) but occasionally in the abdomen if the tear begins there.   

Arterial Rupture

Arterial rupture is a full tear of all the layers of the artery wall. This can occur in any artery in the body and can be fatal if not treated immediately. Emergency care is needed immediately. Symptoms include:

  • Sudden onset pain; may be “severe,” “sharp,” “tearing,” or “ripping” and may be located anywhere in the body, depending on which artery is affected. Sometimes, the pain is less severe, but a person still has a feeling that “something is very wrong.”
  • Rapid swelling of the area may be present
  • Nausea, which may be accompanied with vomiting
  • Clammy skin
  • Shortness of breath
  • Fainting or lightheadedness
  • Loss of pulse
  • Tingling, numbness, burning, prickling of the skin (paresthesia)
  • Paralysis
  • Stroke symptoms may occur if the ruptured artery is in the brain

Collapsed lung (Pneumothorax)

Collapsed lung happens when air or gas collects in the space between the lungs and the chest and prevents the lung from inflating completely. Symptoms include:

  • Chest pain, sudden onset; sharp and may lead to feelings of tightness in the chest
  • Shortness of breath
  • Rapid heart rate
  • Rapid breathing
  • Cough
  • Fatigue
  • Skin may develop bluish color (cyanosis) due to decreased blood oxygen level

Ocular

Carotid Cavernous Sinus Fistula formation (CCSF) is a medical emergency which may occur without injury or trauma in someone with Vascular Ehlers-Danlos Syndrome. CCSF is an abnormal connection between an artery in your neck and the network of veins at the back of your eye. This formation happens as a result of a small tear that sometimes occurs in one of the carotid arteries. Signs of a CCSF are:

  • Sudden onset of a swooshing sound in the head
  • Redness of the eye
  • Pain around the eye
  • Bulging of the eye
  • Slow onset of blurred vision

Bowel Perforation

Bowel perforation occurs when the wall of the bowels (typically a hollow organ, such as the intestines) tears. This allows fecal matter to enter the abdominal cavity, and can result in sepsis and death. In individuals with VEDS, a bowel perforation may occur in the sigmoid colon (lower left quadrant of the abdomen). Esophageal and gastric rupture have rarely been reported.

Symptoms of a bowel perforation include:

  • Abdominal pain that gradually increases and becomes severe or debilitating. This is usually in the lower left quadrant of the abdomen, because usually the perforation is in the sigmoid colon.
  • Nausea
  • Vomiting
  • Chills
  • Rectal bleeding
  • Fever

Uterine Rupture

Uterine rupture is typically limited to the third trimester of pregnancy. A uterine rupture occurs when the wall of the uterus tears. The baby may move into the abdominal cavity outside of the uterus. This is a medical emergency for both the baby and the mother. Signs of a uterine rupture may include:

  • Discomfort in the lower area of the belly or lower back pain that is intermittent (like uterine contractions)
  • An acute, severe pain in the lower abdomen (can feel like ripping pain), sometimes shoulder pain.
  • Blood in the urine.
  • Bloody vaginal discharge with dark brown or bright red bleeding.
  • Change in the contour/shape of the belly- where the baby bump has been located.
  • On fetal heart rate monitor, there are abnormalities in the fetal heart rate pattern.
  • New low blood pressure, dizziness, fainting, maternal pulse is barely palpable and fast heart rate.

Be prepared for emergencies with Vascular Ehlers-Danlos Syndrome!