Vascular-type Ehlers-Danlos syndrome is a severe subtype of a genetic connective tissue disorder. People with this condition have very fragile tissues and are at high risk for severe bleeding and internal injuries. While it isn’t curable, this condition is often manageable, and the complications are often treatable.
Ehlers-Danlos syndrome (EDS) refers to a group of genetic disorders that mainly affect your body's connective tissues, such as ligaments, tendons, and cartilage. There are 13 types of this condition, with vascular EDS being type IV. While extremely rare, it’s also especially severe. People with this subtype of EDS have very fragile arteries and internal organs.
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Vascular Ehlers-Danlos syndrome is genetic, meaning people often inherit it from one or both parents. When it happens to people with no family history of this condition, it’s because this mutation happened spontaneously.
Ehlers-Danlos syndrome overall is a rare condition, only affecting 1 in 5,000 people. The vascular type of this condition is even rarer, with only 1 case per 200,000 to 250,000 people.
Vascular Ehlers-Danlos syndrome affects how strong and durable certain tissues are in a person’s body, especially the arteries and organs. That means people with this condition bruise easily and have a high risk of dangerous bleeding from internal injuries.
The most common symptoms of vascular Ehlers-Danlos syndrome include:
This condition is genetic, meaning it happens because of a genetic mutation that affects certain parts of your DNA. Your body uses DNA like an instruction manual, following the instructions exactly to determine how your cells and organs do their job. Mutations are like typos in the instruction manual, but your body still follows the faulty instructions.
Vascular Ehlers-Danlos syndrome happens because of a mutation in a gene that affects a protein called collagen III. Your body normally uses collagen III to make certain tissues and structures stronger and more durable. That mutation means your body either doesn't make enough collagen III or makes flawed collagen III that doesn't strengthen various tissues as it should.
Because this condition is genetic, people who have it can pass it to their children. A person with this condition has a 50% chance of passing it to their child with each pregnancy (if both parents have this condition, the chance is 100%).
No, this condition can’t pass from person to person.
A doctor may begin to suspect vascular Ehlers-Danlos syndrome based on your medical history, physical examination, symptoms or a family history of this condition. Once they suspect this condition, they can use genetic testing to confirm or rule out that suspicion. Genetic testing is the only way to be certain of this diagnosis because there are only two genetic mutations known (for now) that cause this condition.
Because this condition is very rare, healthcare providers may perform several other tests, such as an echocardiogram or CT scan, before running genetic tests. The early tests may focus on ruling out blood disorders that increase the risk of severe bleeding or easy bruising. However, genetic testing is the only way to be certain that you have vascular Ehlers-Danlos syndrome.
Vascular EDS is not curable. This condition is genetic, meaning that it’s a lifelong condition. Because it isn’t curable, healthcare providers will focus on treating and minimizing the impact of any symptoms.
People with vascular Ehlers-Danlos syndrome have a high risk of developing dangerous internal bleeding because of aneurysms and torn arteries. Because of this, you may need regular medical screenings to look for aneurysms if you have this condition. In some cases, you may need surgery to repair internal injuries or aneurysms. Pregnancy can also lead to a high risk of severe or life-threatening complications from this condition, such as rupture of the uterus or severe bleeding.
A healthcare provider is the best person to explain the potential medications, treatments and procedures that you may need. They can best explain what's happening in your situation, taking into account your circumstances and the details of the care you need or receive.
Because this condition causes you to have very fragile tissues, you will also have a high risk of bleeding. You may also have trouble recovering after surgery for this same reason. A healthcare provider is the best person to explain the potential complications and risks that you may face.
Vascular EDS is a complicated condition that needs close medical monitoring and regular visits to one or more healthcare providers. Because of how complex this condition is, it’s not something you can take care or manage without help from a healthcare provider.
A healthcare provider is the best person to explain what you can expect from this condition, especially if you need treatment to repair an injury or other problem.
Because vascular EDS is a genetic condition, there’s no way to prevent it or reduce your risk of developing it. People with this condition should consider talking to a healthcare provider if they become pregnant or want to have children, so they can know what to expect if their child inherits this condition.
People with vascular EDS have a high risk of complications and problems related to bleeding or fragile internal tissues and organs. Most people with this condition will have at least one instance of severe complications or related conditions by age 20. The risk of having life-threatening complications is 80% by age 40. Half of the people with this condition will live to at least age 48.
Vascular EDS is a condition that you have when you’re born and lasts for your entire life.
People with vascular EDS should see a healthcare provider regularly who can monitor their condition and look for any evidence of new problems. They should also avoid dangerous sports or activities, heavy lifting or other activities that bring a higher risk of injury. They may also want to avoid elective surgery because of the high risk of dangerous bleeding and internal injuries.
You should see your healthcare provider regularly as recommended. They can also tell you any signs or symptoms that mean you need to call or see them.
If you have this condition, you should seek emergency medical attention if you have any unexplained severe pain, especially in your chest or abdomen. You should also get emergency medical attention if you have an external wound or injury that is or was bleeding heavily.
A note from Cleveland Clinic
Vascular Ehlers-Danlos syndrome is a complicated genetic condition that needs close medical monitoring and care. While it's a condition with high risks, advances in medical care and understanding of this condition mean that people with this disorder live longer and have better outcomes than in years past.
Last reviewed by a Cleveland Clinic medical professional on 04/10/2022.
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