Familial intraosseous vascular malformation

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ORPHA:140436OMIM:606893D18.0
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Overview

Familial intraosseous vascular malformation is a very rare inherited condition in which abnormal blood vessel growths (vascular malformations) develop within bones. These malformations are tangles of blood vessels that form inside the bone tissue, most commonly affecting the bones of the skull, spine, or limbs. Because the condition runs in families, multiple members across generations may be affected. The abnormal blood vessels within the bone can weaken the bone structure, leading to pain, swelling, and in some cases fractures. When the skull bones are involved, patients may notice a visible or palpable lump on the head. In more serious cases, the malformations can press on nearby nerves or the spinal cord, causing numbness, weakness, or other neurological symptoms. Some patients may have few or no symptoms and are only diagnosed when imaging is done for another reason. Treatment depends on the location and severity of the malformations. Options may include observation for mild cases, surgical removal of the affected bone area, embolization (a procedure to block blood flow to the malformation), or radiation therapy in select situations. Because this is a familial condition, genetic counseling is recommended for affected families. There is currently no cure, and management focuses on controlling symptoms and preventing complications.

Also known as:

Key symptoms:

Bone painSwelling over affected boneVisible or palpable lump on the skullBone fractures from weakened boneNumbness or tingling if nerves are compressedMuscle weakness near the affected areaHeadaches when skull bones are involvedBack pain if the spine is affectedLimited movement in affected limbsBleeding from the malformation during injury or surgery

Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Familial intraosseous vascular malformation.

View clinical trials →

No actively recruiting trials found for Familial intraosseous vascular malformation at this time.

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

Search ClinicalTrials.gov ↗Join the Familial intraosseous vascular malformation community →

No specialists are currently listed for Familial intraosseous vascular 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 Familial intraosseous vascular malformation.

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Community

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Caregiver Resources

NORD Caregiver Resources

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

Mental Health Support

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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.Where exactly are the vascular malformations located in my bones, and are any near critical structures?,How often should I have imaging to monitor the malformations?,What symptoms should prompt me to seek emergency care?,Are there activities I should avoid to reduce the risk of fractures or complications?,Should my family members be screened for this condition?,What are the treatment options if a malformation starts causing problems?,Is genetic testing available to identify the specific gene change in our family?

Common questions about Familial intraosseous vascular malformation

What is Familial intraosseous vascular malformation?

Familial intraosseous vascular malformation is a very rare inherited condition in which abnormal blood vessel growths (vascular malformations) develop within bones. These malformations are tangles of blood vessels that form inside the bone tissue, most commonly affecting the bones of the skull, spine, or limbs. Because the condition runs in families, multiple members across generations may be affected. The abnormal blood vessels within the bone can weaken the bone structure, leading to pain, swelling, and in some cases fractures. When the skull bones are involved, patients may notice a visibl

How is Familial intraosseous vascular malformation inherited?

Familial intraosseous vascular malformation follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.