Familial retinal arterial macroaneurysm

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ORPHA:284247OMIM:614224H35.0
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Overview

Familial retinal arterial macroaneurysm (also known as familial retinal arteriolar tortuosity and macroaneurysm or FRAM) is a rare inherited eye condition that affects the blood vessels in the retina — the light-sensitive tissue at the back of the eye. In this disease, small arteries in the retina develop abnormal balloon-like swellings called macroaneurysms. These weakened spots in the blood vessel walls can leak fluid or blood into the surrounding retinal tissue, which can lead to vision problems. The condition runs in families and typically affects multiple members across generations. Patients may experience sudden or gradual vision loss, often due to bleeding (hemorrhage) from the macroaneurysms or fluid leaking into the central part of the retina (macular edema). Some people may have no symptoms at all until a macroaneurysm ruptures. The retinal blood vessels may also appear unusually twisted or tortuous on examination. There is currently no cure for familial retinal arterial macroaneurysm. Treatment focuses on managing complications when they arise. Laser photocoagulation can be used to seal leaking or bleeding macroaneurysms. Anti-VEGF injections (medications injected into the eye) may help reduce swelling and fluid leakage. Regular monitoring by a retinal specialist is essential to detect new macroaneurysms early and preserve vision. The condition differs from the more common non-familial (sporadic) retinal macroaneurysms, which are typically associated with high blood pressure and older age.

Also known as:

Key symptoms:

Sudden painless vision loss in one eyeBlurred or distorted central visionBleeding inside the eye (vitreous hemorrhage)Fluid buildup in the central retina (macular edema)Balloon-like swellings on retinal blood vesselsTwisted or tortuous retinal arteriesFloaters or dark spots in visionReduced sharpness of visionRecurrent episodes of retinal bleeding

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 retinal arterial macroaneurysm.

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No actively recruiting trials found for Familial retinal arterial macroaneurysm at this time.

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No specialists are currently listed for Familial retinal arterial macroaneurysm.

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 retinal arterial macroaneurysm.

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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.How often should I have my eyes examined to check for new macroaneurysms?,What symptoms should prompt me to seek emergency eye care?,Should my family members be screened for this condition?,Is genetic testing available for my family, and would it be helpful?,What treatment options are available if a macroaneurysm starts to bleed or leak?,Are there lifestyle changes I can make to reduce the risk of complications?,How likely is it that my children will inherit this condition?

Common questions about Familial retinal arterial macroaneurysm

What is Familial retinal arterial macroaneurysm?

Familial retinal arterial macroaneurysm (also known as familial retinal arteriolar tortuosity and macroaneurysm or FRAM) is a rare inherited eye condition that affects the blood vessels in the retina — the light-sensitive tissue at the back of the eye. In this disease, small arteries in the retina develop abnormal balloon-like swellings called macroaneurysms. These weakened spots in the blood vessel walls can leak fluid or blood into the surrounding retinal tissue, which can lead to vision problems. The condition runs in families and typically affects multiple members across generations. Pati

How is Familial retinal arterial macroaneurysm inherited?

Familial retinal arterial macroaneurysm follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.