OBSOLETE: Frontonasal arteriovenous malformation

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:141168
Who is this for?
Show terms as
8Treatment centers

Where are you in your journey?

UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
Report missing data

Overview

Frontonasal arteriovenous malformation (AVM) is a very rare vascular condition in which abnormal connections form between arteries and veins in the frontonasal region — the area around the forehead, nose, and midface. Normally, blood flows from arteries through tiny capillaries before reaching veins. In an AVM, blood passes directly from arteries to veins without going through capillaries, creating a tangled cluster of abnormal blood vessels. This can cause swelling, disfigurement, pain, and sometimes bleeding in the affected area. The condition may be present from birth or become noticeable during childhood or later in life. It is important to note that this specific Orphanet entry (141168) has been marked as 'OBSOLETE,' meaning the classification may have been merged into a broader category of craniofacial or extracranial arteriovenous malformations. Patients who were previously diagnosed under this term should work with their medical team to understand the current classification that applies to their condition. Treatment typically involves a combination of approaches including embolization (a procedure to block abnormal blood vessels), surgery, or both. Management is often complex and requires a multidisciplinary team. Because AVMs can grow or change over time, long-term monitoring is essential. The treatment landscape continues to evolve, with newer embolization techniques and surgical approaches improving outcomes for patients with craniofacial vascular malformations.

Key symptoms:

Visible swelling or mass on the forehead or nose areaSkin discoloration or redness over the affected areaA pulsating or throbbing feeling in the affected areaNosebleeds that may be difficult to controlPain or tenderness in the midface regionFacial asymmetry or disfigurementWarm skin over the malformationBleeding from the malformationDifficulty breathing through the noseVision problems if the malformation extends near the eyesHeadaches

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for OBSOLETE: Frontonasal arteriovenous malformation.

View clinical trials →

No actively recruiting trials found for OBSOLETE: Frontonasal arteriovenous malformation at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the OBSOLETE: Frontonasal arteriovenous malformation community →

No specialists are currently listed for OBSOLETE: Frontonasal arteriovenous malformation.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to OBSOLETE: Frontonasal arteriovenous malformation.

Search all travel grants →NORD Financial Assistance ↗

Community

Open OBSOLETE: Frontonasal arteriovenous malformationForum →

No community posts yet. Be the first to share your experience with OBSOLETE: Frontonasal arteriovenous malformation.

Start the conversation →

Latest news about OBSOLETE: Frontonasal arteriovenous malformation

No recent news articles for OBSOLETE: Frontonasal arteriovenous malformation.

Follow this condition to be notified when news becomes available.

Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.What is the exact size and extent of the arteriovenous malformation based on imaging?,What treatment approach do you recommend, and what are the risks and benefits?,How likely is the malformation to grow or recur after treatment?,How often will my child (or I) need follow-up imaging?,Are there any activities or situations I should avoid to prevent complications?,Should I be referred to a specialized vascular anomalies center?,Is genetic testing recommended to rule out an underlying syndrome?

Common questions about OBSOLETE: Frontonasal arteriovenous malformation

What is OBSOLETE: Frontonasal arteriovenous malformation?

Frontonasal arteriovenous malformation (AVM) is a very rare vascular condition in which abnormal connections form between arteries and veins in the frontonasal region — the area around the forehead, nose, and midface. Normally, blood flows from arteries through tiny capillaries before reaching veins. In an AVM, blood passes directly from arteries to veins without going through capillaries, creating a tangled cluster of abnormal blood vessels. This can cause swelling, disfigurement, pain, and sometimes bleeding in the affected area. The condition may be present from birth or become noticeable d

How is OBSOLETE: Frontonasal arteriovenous malformation inherited?

OBSOLETE: Frontonasal arteriovenous malformation follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.