Benign concentric annular macular dystrophy

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ORPHA:251287OMIM:153870H35.5
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Overview

Benign concentric annular macular dystrophy (sometimes called BCAMD) is a rare inherited eye condition that affects the macula — the small central part of the retina at the back of your eye that is responsible for sharp, detailed vision. In this condition, the cells in the macula slowly break down over time, forming a distinctive ring-shaped (annular) pattern of damage that can be seen during an eye exam. The word 'benign' in the name reflects that the disease tends to progress slowly and does not usually lead to complete blindness, though it does cause meaningful vision changes. The main symptom is a gradual loss of central vision, which is the vision you use for reading, recognizing faces, and seeing fine details. Side (peripheral) vision is usually preserved. Some people also notice that colors look less vivid or that they have difficulty seeing in low light. Symptoms often begin in adulthood, though the age of onset can vary. There is currently no cure or approved treatment that can stop or reverse the damage to the macula in this condition. Care focuses on monitoring vision changes, using low-vision aids such as magnifiers, and adapting daily activities to make the most of remaining peripheral vision. Regular follow-up with a retinal specialist is important to track how the condition is progressing.

Key symptoms:

Gradual loss of central (straight-ahead) visionDifficulty reading small printTrouble recognizing facesColors appearing less bright or washed outDifficulty seeing in dim lighting or at nightA blurry or dark spot in the center of visionReduced ability to see fine detailsPeripheral (side) vision remains largely intact

Inheritance

Autosomal dominant

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

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Benign concentric annular macular dystrophy.

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No actively recruiting trials found for Benign concentric annular macular dystrophy at this time.

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No specialists are currently listed for Benign concentric annular macular dystrophy.

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 Benign concentric annular macular dystrophy.

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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

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.How quickly do you expect my vision to change, and what signs should I watch for?,Should my children or siblings be tested for this condition?,What genetic testing do you recommend, and what will the results tell us?,Are there any clinical trials or research studies I could join?,What low-vision aids or rehabilitation programs do you recommend for me?,Are there any lifestyle changes or supplements that might help protect my remaining vision?,How often should I have my eyes checked, and what tests will you use to monitor my condition?

Common questions about Benign concentric annular macular dystrophy

What is Benign concentric annular macular dystrophy?

Benign concentric annular macular dystrophy (sometimes called BCAMD) is a rare inherited eye condition that affects the macula — the small central part of the retina at the back of your eye that is responsible for sharp, detailed vision. In this condition, the cells in the macula slowly break down over time, forming a distinctive ring-shaped (annular) pattern of damage that can be seen during an eye exam. The word 'benign' in the name reflects that the disease tends to progress slowly and does not usually lead to complete blindness, though it does cause meaningful vision changes. The main sym

How is Benign concentric annular macular dystrophy inherited?

Benign concentric annular macular dystrophy follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Benign concentric annular macular dystrophy typically begin?

Typical onset of Benign concentric annular macular dystrophy is adult. Age of onset can vary across affected individuals.