Treatment

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Although there is not yet a way to prevent the telangiectases or AVMs from occurring, most can be treated once they occur. They should be treated if they are either causing a significant problem (as in the case of frequent nosebleeds) or if they are at high risk of causing a serious problem (such as a stroke from a lung AVM). The recommended treatment for a telangiectasia or AVM depends on both its size and location in the body.

Bleeding from telangiectases in the nose sometimes responds satisfactorily to some everyday practical treatments implemented at home. Humidification of the air and use of an ointment on the lining of the nose help keep the mucous membrane of the nose moist and can 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 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 an expert HHT physician and centre.

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 (Ears, Nose, and Throat) 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 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 off an artery) can be used to halt severe nose bleeding that has 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 temporary measure.

Telangiectases of the skin can also be treated with laser therapy if they bleed to an extent that is bothersome or if the telangiectases are a cosmetic concern. Lesions of the skin are usually best treated by a dermatologist who has particular expertise in the use of lasers.

Bleeding from the stomach or intestines is generally treated only if it causes anemia (low blood count). Iron replacement therapy is the first line of defense. Iron is usually first given orally (a tablet by mouth), but can be given intravenously (IV) if the oral iron is not tolerated by a patient, or if the oral iron is not getting enough iron into the body. If iron therapy can not control the anemia, transfusion and endoscopic treatments using a heater probe, bicap, argon photocoagulation or laser are options. Some studies have shown hormonal therapy to be helpful. This and other medical therapies that affect blood clotting and blood vessel function are considered in severe cases. The decisions to use these medications are complex and case-by-case, usually with the advice an expert HHT physician and centre.

Lung and brain AVMs should be treated before they cause symptoms or problems in most cases. This is why testing or screening for them is recommended in all individuals with HHT, regardless of their specific symptoms. Lung AVMs can almost always be treated completely and permanently using an outpatient procedure called embolization. An interventional radiologist inserts a small tube (catheter) in a large vein in the groin. The tube is then passed through the blood vessels system to the AVM in the lung. A device (a ‘coil’ or occasionally a ‘plug’) is placed in the artery leading to the AVM to stop blood flow through the AVM. The procedure usually takes 1-3 hours and requires only a few hours of recuperation.

Brain AVMs are treated in different ways depending on the size, structure and location in the brain of the abnormal blood vessels. Surgery, embolization and stereotactic radiosurgery can all be used, separately or in combination, to successfully treat brain AVMs.

Liver AVMs are currently treated only if a patient shows signs of liver or heart failure, as a result of their liver AVM. Embolization, which is so successful for the treatment of pulmonary AVMs, can cause severe complications when performed in the liver. Decisions regarding treatment of liver AVM are made on a case by case basis and should be managed by a physician very familiar with the liver manifestations of HHT.

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