Hereditary hemorrhagic telangiectasia (HHT)

Treatment

People who have HHT should seek treatment at a medical center with experience treating this disorder. Because the disorder is uncommon, finding a specialist in HHT can be difficult. In the United States, HHT Centers of Excellence, including Mayo Clinic, are designated by the HHT Foundation International for their ability to diagnose and treat all aspects of the disorder.

Although no cure exists, doctors can treat the symptoms of HHT and help avoid serious complications caused by abnormal blood vessels. HHT treatment often depends on which parts of the body are affected.

Nose

Telangiectases in the nose can cause bleeding that ranges from minor to severe. Minor nosebleeds may respond to home remedies, such as humidifying the air or using moisturizing nose sprays or drops. Treatments for more-serious nosebleeds include:

• Laser treatment. During laser coagulation therapy, a small beam of laser light is directed around the margins of telangiectases. This destroys and seals off the vessels that are bleeding.
• Septal dermoplasty. During this surgical procedure, the mucous lining of the nose is replaced with a graft of thicker skin. This procedure permanently removes the natural lining of the nose.
• Hormonal therapy. In some cases, your doctor may prescribe hormone medications to help control nosebleeds. This may slow down bleeding, but is often not successful.
• Blocking an artery (embolization). This procedure, called embolization, blocks off an artery that carries blood to the malformed vessel. This is usually only effective for six to eight weeks because other arteries will replace the blocked one.

Skin

Often, telangiectases require little more than medical monitoring. Telangiectases that bleed and discolor the skin are usually treated with laser therapy. The laser beam can selectively destroy the discolored tissue.

Stomach and intestines

Bleeding in the stomach or the intestines is often difficult to treat due to the large number of telangiectases that may occur in the gastrointestinal tract. Telangiectases can be treated with laser, a heater probe, hormonal treatment or a combination of therapies. Anemia caused by blood loss is typically treated with iron supplements or blood transfusions.

Lung

AVMs in the lung should be treated before they cause symptoms. To treat lung AVMs, doctors may perform an embolization through a procedure called a pulmonary angiogram. In a pulmonary angiogram, a radiologist will first guide a thin tube (catheter) through a needle insertion in the groin to the AVM. At this point, the radiologist does an embolization by placing a tiny balloon or coil to seal off the AVM and block it from the body's circulation.

Brain

Treatment for AVMs in the brain depends on their size, location and how they are formed. Treatments include surgery, embolization, stereotactic radiosurgery and, in some cases, observation.

Liver

Because treating vessel malformations in the liver can sometimes lead to serious complications, the decision to treat the liver is evaluated for each person. Options may include liver transplantation in rare cases.