What are the other Names for this Condition? (Also known as/Synonyms)

• Extracranial AVM
• Head and Neck Arteriovenous Malformation
• Head and Neck AVM

What is Extracranial Arteriovenous Malformation? (Definition/Background Information)

• Arteriovenous malformations (or AVMs) are rare vascular malformations (abnormal tangles of blood vessels where direct connections form between arteries and veins) which disrupt natural blood flow
• AVMs most commonly affect the central nervous system (brain and spinal cord) but can involve any organ. Those affecting the face, head or neck are often called Extracranial Arteriovenous Malformation
• Although present at birth, AVMs may not be clinically evident until childhood or adolescence
• Complications may include disfigurement, destruction of tissue, obstruction of vital structures, pain, bleeding, ulceration and rarely, cardiac overload
• AVMs may be treated with surgery, embolization, or both. The goal of treatment is to control rather than cure the underlying problem

(Source: Extracranial Arteriovenous Malformation; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

Who gets Extracranial Arteriovenous Malformation? (Age and Sex Distribution)

• Extracranial Arteriovenous Malformation is a rare condition. The presentation of symptoms may occur at childhood or adolescence, although the condition may be present at birth
• Both males and females may be affected
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Extracranial Arteriovenous Malformation? (Predisposing Factors)

• Currently, no risk factors have been clearly identified for Extracranial Arteriovenous Malformation

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Extracranial Arteriovenous Malformation? (Etiology)

• Extracranial Arteriovenous Malformation arises owing to malformations in arteries and veins of the head, face and neck
• Most cases of Extracranial AVM are not inherited and a family history is not observed

What are the Signs and Symptoms of Extracranial Arteriovenous Malformation?

The signs and symptoms of Extracranial Arteriovenous Malformation may include:

• Hemorrhage in the affected areas, which may cause discoloration
• Pain in the affected area
• Warmth in the affected area
• Ulceration

How is Extracranial Arteriovenous Malformation Diagnosed?

Extracranial Arteriovenous Malformation is diagnosed on the basis of the following information:

• Complete physical examination
• Thorough medical history evaluation
• Assessment of signs and symptoms
• Laboratory tests
• Imaging studies
• Biopsy studies, if necessary

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Extracranial Arteriovenous Malformation?

The complications of Extracranial Arteriovenous Malformation may include:

• Disfigurement
• Destruction of tissue
• Obstruction of vital structures
• Cardiac overload (rare)

(Source: Extracranial Arteriovenous Malformation; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Extracranial Arteriovenous Malformation Treated?

There is no cure for Extracranial Arteriovenous Malformation, and treatment is usually given to manage the signs and symptoms and any complication that develops. The treatment options may include:

• Surgical excision of the malformed vessels
• Embolization of abnormal blood flow between arteries and veins
• Combination of surgery and embolization

How can Extracranial Arteriovenous Malformation be Prevented?

• At the present time, no guidelines or methods are available for the prevention of Extracranial Arteriovenous Malformation
• Active research is currently being performed to explore the possibilities for treatment and prevention of disorders such as Extracranial Arteriovenous Malformation
• Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Extracranial Arteriovenous Malformation? (Outcomes/Resolutions)

• The prognosis of Extracranial Arteriovenous Malformation is dependent upon the severity of the signs and symptoms and associated complications, if any
• Individuals with mild conditions have better prognosis than those with severe symptoms and complications
• Typically, the prognosis may be assessed on a case-by-case basis

Additional and Relevant Useful Information for Extracranial Arteriovenous Malformation:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/