Treatment

Currently, the formation of telangiectases or AVMs cannot be prevented, but most can be treated once they are detected. Treatment is administered if they are causing a significant problem, such as frequent nosebleeds from nostril telangiectases or risk of stroke from a lung AVM. The recommended treatment for a telangiectasia or AVM depends on both its size and location in the body.

Nose Bleeds

Bleeding from nostril telangiectases can be controlled or treated at home. For example, humidification of the air and use of an ointment on the lining of the nose can help keep the mucous membrane of the nose moist to reduce nosebleeds. There are products sold over-the-counter in pharmacies or pharmacy sections of big stores that can be used to help control nosebleeds when simple pressure applied to the outside of the nose isn’t enough.

If these home management techniques do not result in a satisfactory control or reduction in nosebleeds, the next treatment that is usually considered is laser therapy. Laser coagulation therapy is preferable to electric and chemical cautery primarily because- if done carefully by an Ears, Nose, and Throat (ENT) physician with specific expertise in both laser therapy and HHT- it has less risk to damage the inside of the nose. A small beam is directed around the margins of each telangiectasia and photocoagulation occurs. Most patients who undergo laser therapy see significant improvement for a period of time, but it usually needs to be repeated periodically. Because the procedure has little risk of harming the nose if done by an experienced physician, it can be repeated as needed.

Some studies have shown hormonal therapy to be helpful in some patients for whom the local therapies (i.e. home moisturizing care and laser therapy) have not been successful. There are some other medical therapies that affect blood clotting and blood vessel function, which are considered in severe cases. The decisions to use these medications are complex and case-by-case, usually with the advice of an expert HHT physician and center.

Septal dermoplasty is another treatment option for severe nosebleeds, and is usually considered when laser therapy and medical therapies have repeatedly failed to help, and nosebleeds are severe enough to lead to chronic anemia. Septal dermoplasty replaces the thin lining of the nose (called the mucous membrane) with a thicker graft of skin. When performed by an ENT physician knowledgeable and experienced with the Saunder’s method, it can significantly reduce the frequency and severity of nosebleeds. It is a more drastic treatment than laser in that it permanently removes the natural lining of the nose and replaces it with skin. Daily care and attention to the nose is required after septal dermoplasty to keep the nose moist and clean.

Embolization (blocking of an artery) can be used to halt severe nose bleeds that have been unresponsive to other treatments, but is usually only effective for 6-8 weeks. Other arteries enlarge and cause recurrence of the bleeding. This therapy for the nose is generally used only on an emergency basis, by expert interventional radiologists, and is generally only a