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Osler-Weber-Rendu syndrome

Definition

Osler-Weber-Rendu syndrome is a disorder of the blood vessels that can cause excessive bleeding.

Alternative Names

Hereditary hemorrhagic telangiectasia; HHT

Causes

Osler-Weber-Rendu syndrome is inherited, which means it is passed down through families. Scientists have identified 4 genes involved in this condition. All of these genes appear to be important for blood vessels to develop properly.

Symptoms

People with Osler-Weber-Rendu syndrome can develop abnormal blood vessels in several areas of the body. These vessels are called arteriovenous malformations (AVMs).

If they are on the skin, they are called telangiectasias. The abnormal blood vessels can also develop in the brain, lungs, liver, intestines, or other areas.

Symptoms of this syndrome include:

Exams and Tests

An experienced health care provider can detect telangiectases during a physical examination. There is often a family history of this condition.

Signs include:

Tests include:

Genetic testing is available to look for changes in genes associated with this syndrome.

Treatment

Treatments may include:

  • Surgery to treat bleeding in some areas
  • Electrocautery (heating tissue with electricity) or laser surgery to treat frequent or heavy nosebleeds
  • Endovascular embolization (injecting a substance through a thin tube) to treat abnormal blood vessels in the brain and other parts of the body

Some people respond to estrogen therapy, which can reduce bleeding episodes. Iron may also be given if there is a lot of blood loss leading to anemia. Avoid taking blood-thinning medicines. Some drugs that affect blood vessel development are being studied as possible future treatments.

Some people may need to take antibiotics before having dental work or surgery. Ask your provider what precautions you should take.

Support Groups

Cure HHT -- www.curehht.org

Outlook (Prognosis)

People with this syndrome can live a completely normal lifespan, depending on where in the body the AVMs are located.

Possible Complications

These complications can occur:

  • Heart failure
  • High blood pressure in the lungs (pulmonary hypertension)
  • Internal bleeding
  • Shortness of breath

When to Contact a Medical Professional

Call your provider if you or your child has frequent nose bleeds or other signs of this disease.

Prevention

Genetic counseling is recommended for couples who want to have children and who have a family history of hereditary hemorrhagic telangiectasia. If you have this condition, medical treatments can prevent certain types of strokes and heart failure.

References

Ferri FF, Ferri H. Osler-Rendu-Weber syndrome. In: Ferri FF, ed. Ferri's Clinical Advisor 2015. 1st ed. Philadelphia, PA: Elsevier Mosby; 2014:section I.

McDonald J, Wooderchak-Donahue W, VanSant Webb C, Whitehead K, Stevenson DA, Bayrak-Toydemir P. Hereditary hemorrhagic telangiectasia: genetics and molecular diagnostics in a new era. Front Genet. 2015;6(1):1-8.

Review Date:4/20/2015
Reviewed By:Chad Haldeman-Englert, MD, FACMG, Wake Forest School of Medicine, Department of Pediatrics, Section on Medical Genetics, Winston-Salem, NC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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Health
Outcome Data

No data available for this condition/procedure.

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