Hereditary Hemorrhagic Telangiectasia (HHT) Center

St. Louis Children’s Hospital is an Hereditary Hemorrhagic Telangiectasia (HHT) Center of Excellence that provides care to children in families with HHT.

What is HHT?
Hemorrhagic Telangiectasia (HHT), also known as Osler-Weber-Rendu disease, is a disorder in which some blood vessels develop abnormally.

People with HHT may have different kinds of abnormal blood vessels, the most common of which are called telangiectases. Others are called arteriovenous malformations (AVMs).

These abnormal blood vessels are more prone to bleeding, especially if they are in the nose, GI tract, lungs or brain. AVMs have the potential to cause life-threatening complications such as stroke or pulmonary hemorrhage.

HHT is a genetic disorder that is passed on through generations in families. If one parent has HHT, there is a 50 percent chance that each child will have the disorder.

Symptoms of HHT:

• Repeated nose bleeds
• Solid organ AVMs
• Skin telangiectase --small dialated blood vessels near the skin

Symptoms may vary greatly within the same family. It is important to evaluate every family member who has the potential to have HHT. It is especially important that children are screened, as they often do not develop obvious signs of HHT until adolescence, or even into adulthood.

A Center of Excellence
Our pediatric center is part of an internationally recognized joint program with the Washington University School of Medicine, providing care to children and adults with HHT.

Founded nearly 20 years ago, the HHT Center has been accredited by the HHT Foundation International as a Center of Excellence. It is only one of 14 centers in North America treating the disorder, and is the only center in the United States with a dedicated pediatric team.

The team includes specialists from pulmonology, pediatrics, otolaryngology, radiology, neurosurgery, genetics, cardiology, gastroenterology, hematology and dermatology.

What to Expect
Your visit begins with a telephone conference with a team member. We will gather a detailed family and patient health history and obtain all necessary medical records. Once this information is carefully reviewed by our multidisciplinary team, we will tailor the specific diagnostic tests and imaging necessary for your child.

On the day of the visit, your child will undergo a thorough physical examination and imaging that may include:

• an MRI of the brain
• CT angiogram of the chest
• Or a bubble echocardiogram

The testing and consultation can often be completed within one to two days. Once a diagnosis of probable or definite HHT is made, our goal is to treat current symptoms and to avoid any future complications. Because HHT can affect many organs, it is important to have a multidisciplinary team of doctors carefully monitoring and managing your child’s ongoing care.