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Symptoms & Treatments For HHT

People with HHT often have a group of recognizable symptoms that includes bleeding and hemorrhaging.

Nosebleeds

The most common complaint of HHT patients is nosebleeding. Our ear, nose and throat (ENT) physicians treat nosebleeds with a variety of surgical and non-surgical methods.

Treatment

The method used to treat nosebleeds depends on a combination of nosebleed severity, number, and appearance of telangiectasia in the nose, past treatments, and patient preference. Surgical methods include laser therapy and Young's procedure.

Telangiectasia Of The Skin Or Mouth

Telangiectasia

Almost everyone with HHT eventually develops these small red spots on their face, hands, lips or mouth.

Treatment

Most often no treatment is required. However, if one or more become bothersome, either cosmetically or due to bleeding, they are typically treated by laser therapy.

Intestinal Bleeding

Bleeding inside the intestine from telangiectasia is less common than usually assumed and rarely occurs before age 60.

Treatment

We recommend treatment of telangiectasia in the intestinal tract only if anemia results. Initial anemia treatment focuses on iron replacement to correct the anemia, but other treatments, such as cautery/laser ablation and drug therapy, are available when the anemia isn’t corrected by iron replacement therapy alone.

Lung AVMs

Lung AVMs (areteriovenous malformations) are the most common cause of severe and sudden harm to individuals with HHT. Most often lung AVMs do not cause harm by bleeding, but reduce the ability of the lungs to filter blood from clots or clumps of bacteria as it circulates through the body. This filtering of impurities of the blood after each trip around the body is an important function of the lungs. Because blood clots and clumps of bacteria are less likely to be filtered from the blood, individuals with AVMs in their lungs are more likely to have (clot type) strokes and brain abscess. These can be prevented if lung AVMs are detected and treated before they cause symptoms.

Treatment

Lung AVMs are treated by a non-surgical procedure called embolization. This procedure uses catheters to remove lung AVMs by blocking off their blood flow.

Brain AVMS

About one in 10 individuals with HHT have a brain AVM, and about half of these will result in a brain hemorrhage over a lifetime if not detected and treated. These hemorrhages usually occur without prior warning signs, and the consequence is often death or disability. Since the hemorrhages often take place very early in life, we recommend screening during a child's first year.

Treatment

If detected, most brain AVMs can be safely treated by stereotactic radiosurgery, embolization, or surgery.

LIVER AVMS

Although liver arteriovenous malformations are common in HHT, they almost never rupture and bleed as can telangiectasia and AVMs in most other locations. Treatment is rarely needed unless the heart is adversely affected by the resulting extra blood flow.

Treatment

When needed, treatment with an anti-angiogenesis drug is most common, since AVMs in the liver do not respond well to either surgery or embolization.