Osteofibrous dysplasia

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ORPHA:488265OMIM:607278M85.0
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Overview

Osteofibrous dysplasia (OFD) is a rare, non-cancerous bone condition that mainly affects the shin bone (tibia) and, less often, the fibula — the two bones in the lower leg. It is sometimes called Campanacci disease or ossifying fibroma of long bones. In this condition, normal bone tissue is gradually replaced by a mix of fibrous (scar-like) tissue and abnormal bone, making the affected area weaker than healthy bone. This usually happens during childhood and tends to slow down or stop on its own after puberty. The most common signs are a visible bowing or curving of the lower leg, mild pain or swelling, and an increased risk of fractures (broken bones) in the affected area. Many children are diagnosed after a routine X-ray shows a characteristic pattern of lesions in the shin bone. The condition is almost always limited to one leg, though both legs can occasionally be involved. Treatment depends on how severe the condition is. Many children are monitored carefully with regular X-rays and do not need surgery right away. Surgery may be recommended if the bowing is getting worse, if fractures keep happening, or if the bone is at serious risk of breaking. Because the condition often stabilizes after puberty, doctors usually prefer to wait before doing major surgery. There is no medication that treats OFD directly, but pain management and protective bracing can help with daily comfort and safety.

Also known as:

Key symptoms:

Bowing or curving of the lower leg (shin)Swelling or a bump along the shin boneMild to moderate leg painStress fractures or repeated broken bones in the lower legLimping or an unusual walking patternOne leg appearing shorter than the otherVisible deformity of the lower legTenderness when pressing on the shin boneReduced activity tolerance due to leg discomfort

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Osteofibrous dysplasia.

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No actively recruiting trials found for Osteofibrous dysplasia at this time.

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

Search ClinicalTrials.gov ↗Join the Osteofibrous dysplasia community →

No specialists are currently listed for Osteofibrous dysplasia.

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 Osteofibrous dysplasia.

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Community

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Latest news about Osteofibrous dysplasia

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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 severe is my child's condition, and does it need treatment now or can we monitor it?,How often should we come in for check-up X-rays, and what changes should prompt an earlier visit?,What activities are safe, and which ones should my child avoid to protect the bone?,What is the risk that this could be related to or turn into adamantinoma, and how will you monitor for that?,At what point would you recommend surgery, and what type of surgery would be most appropriate?,Should we see a clinical geneticist to better understand the cause of this condition?,Are there any clinical trials or research studies we should know about?

Common questions about Osteofibrous dysplasia

What is Osteofibrous dysplasia?

Osteofibrous dysplasia (OFD) is a rare, non-cancerous bone condition that mainly affects the shin bone (tibia) and, less often, the fibula — the two bones in the lower leg. It is sometimes called Campanacci disease or ossifying fibroma of long bones. In this condition, normal bone tissue is gradually replaced by a mix of fibrous (scar-like) tissue and abnormal bone, making the affected area weaker than healthy bone. This usually happens during childhood and tends to slow down or stop on its own after puberty. The most common signs are a visible bowing or curving of the lower leg, mild pain or

How is Osteofibrous dysplasia inherited?

Osteofibrous dysplasia follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Osteofibrous dysplasia typically begin?

Typical onset of Osteofibrous dysplasia is childhood. Age of onset can vary across affected individuals.