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Treatment of HHT Symptoms
HHT management consists of anemia management, as well as prevention and treatment of epistaxis. The management of AVM relies on early detection and occlusion, often by interventional radiology, depending on the location. In cases of liver disease, liver transplantation is required until antiangiogenic drugs, such as thalidomide and anti-VEGF (Vascular Endothelial Growth Factor) antibodies, are proven to be effective and are accepted.
Many patients with HHT have iron deficiency anemia caused by frequent epistaxis and GI bleeding. Treatment options include oral iron supplementation and intravenous iron infusion. Some patients may require blood transfusions. Because of the risk of anemia in patients with HHT, all patients older than 35 years should have annual measurement of hemoglobin or hematocrit levels.
Approximately 50% of patients with HHT have an AVM of the brain, lung, or liver, or a combination of 2 or 3 and will require therapy usually by a team of internists and interventional radiologists with expertise in this disorder. In North America, nine HHT centers have been developed over the past nine years at the University of Oregon, University of Utah, Washington University St. Louis, University of California San Diego, University of Pennsylvania, the Mayo Clinic, Medical College of Georgia, University of Toronto, and Yale University. There are also centers in Europe, Asia and Australia.
Treatment of epistaxis involves local measures, including the use of ointments to decrease drying of the nasal mucosa. Humidification of indoor air and avoidance of dry climates can also be beneficial. Because the nasal mucosa is a hormone-modulated tissue, estrogen may be used (topically or systemically) to help support the nasal mucosa and decrease bleeding. Laser coagulation of nasal telangiectasias may be helpful for patients who do not respond well to medical treatment and should be considered the first-line treatment option for HHT-related epistaxis requiring surgical intervention. Septal dermoplasty is a procedure in which the nasal mucosa is stripped from the nose and replaced with a thin dermis graft. The effects of laser coagulation and septal dermoplasty tend to be temporary and often require repeated treatment. The Youngs procedure involves complete closure of the nasal passages and can be curative; however, patients become obligate mouth breathers and lose their sense of smell. Embolization may be useful as a short-term treatment for marked epistaxis, but results tend to be temporary and it should not be considered a first-line treatment.