Hereditary Hemorrhagic Telangiectasia (HHT)

What is Hereditary Hemorrhagic Telangiectasia (HHT)?

The Yale Sinus and Allergy Program specializes in treating patients with inherited disorders, such as HHT, that affect the nasal and sinus region. 

HHT, or hemorrhagic telangiectasia, is an inherited disorder that causes certain abnormalities of a small percentage of blood vessels. Blood vessels, which include arteries, veins, and capillaries, are the tubes that carry blood throughout our bodies. Capillaries are very small blood vessels that connect arteries to veins. A person with HHT may form blood vessels that do not have capillaries, leaving the blood to go directly from an artery to a vein, making that place where the vein receives the blood from the artery very fragile and prone to rupture. 

Very frequently, this abnormality tends to happen in the lining of the nose, as well as other places. 

At the Yale Sinus and Allergy Program, we can help you find relief from HHT symptoms by using the latest in medical and surgical care. 

Symptoms of Hereditary Hemorrhagic Telangiectasia

HHT symptoms vary depending on the location affected by the disorder. Areas affected may include: 
  • nose 
  • skin of the face, hands, and mouth 
  • lungs 
  • brain 
  • lining of the stomach and intestines (GI tract) 
Recurrent nosebleeds are the most common symptom. Also quite common is the look of small red to purple spots, lacy red vessels, and lesions on different areas on the body, including the face, inside the mouth and nostrils, lips, ears, conjunctiva of the eyes, forearms, hands, and fingers. 

This list should be used as a guideline. Not every symptom is included. If you have one or more of these symptoms, it does not mean that you have HHT. Please contact your doctor if you think you may have this disorder.

Causes of Hereditary Hemorrhagic Telangiectasia

HHT is typically genetic, which means it is passed down from at least one parent. In HHT, an abnormal gene creates less of a substance that normally helps in the formation of blood vessels. Sometimes, HHT occurs as a spontaneous mutation and the child will be the first in a family to have the disease.

Risk Factors of Hereditary Hemorrhagic Telangiectasia

Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get HHT; not having risk factors does not mean that you will not get HHT. If you think you may be at risk, you should talk to your doctor. 

HHT is generally an inherited condition, except in cases where a new mutation occurs. Therefore, the greatest risk factor is having a parent with HHT. 

Treatment for Hereditary Hemorrhagic Telangiectasia

HHT nasal problems can be managed effectively. At the Yale Sinus and Allergy Program, we offer the latest surgical procedures to treat patients with HHT. 

Surgical treatments for HHT for nasal problems include laser coagulation therapy and septodermoplasty. With laser coagulation therapy, a small beam of laser light is used to correct the nosebleeds. With septodermoplasty, surgeons replace the thin lining of the nose with a thicker graft of skin. 

We understand that the surgical process can feel intimidating, and you probably have many questions and concerns. Our surgeons take the time to explain your treatment options and answer all your questions. We want you to feel comfortable with knowing what to expect, every step of the way.