Wormian bone-multiple fractures-dentinogenesis imperfecta-skeletal dysplasia

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ORPHA:166277OMIM:604922Q78.8
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Overview

Wormian bone-multiple fractures-dentinogenesis imperfecta-skeletal dysplasia is an extremely rare genetic bone disorder. This condition is sometimes grouped within the broader family of osteogenesis imperfecta-like disorders, though it has distinct features that set it apart. The disease affects the way bones and teeth develop, leading to a combination of characteristic problems. People with this condition typically have Wormian bones, which are extra small bone pieces found within the skull's natural joints (sutures). They also experience multiple bone fractures, often from minor injuries or even normal daily activities, because their bones are unusually fragile. Another hallmark feature is dentinogenesis imperfecta, a condition where the teeth appear discolored (often bluish-gray or amber-brown), are weaker than normal, and may wear down or break easily. Additionally, affected individuals show signs of skeletal dysplasia, meaning their bones do not grow or form properly, which can lead to short stature, bowing of the limbs, and other skeletal abnormalities. Because this condition is so rare, treatment is largely supportive and symptom-based. Management typically involves a team of specialists who work together to prevent fractures, protect the teeth, and address skeletal problems. There is currently no cure, but careful medical management can improve quality of life significantly.

Also known as:

Key symptoms:

Bones that break easily from minor injuriesMultiple fractures throughout lifeExtra bone pieces in the skull (Wormian bones)Discolored and fragile teeth (dentinogenesis imperfecta)Short statureBowed or curved limbsAbnormal bone shape and structureJoint looseness or hypermobilityReduced bone density (osteoporosis)Blue or gray tint to the whites of the eyesDelayed motor developmentBone deformities that worsen over timeDifficulty with mobility and walking

Clinical phenotype terms (13)— hover any for plain English
Periorbital fullnessHP:0000629Dentinogenesis imperfectaHP:0000703Toe clinodactylyHP:0001863Upper limb undergrowthHP:0009824Concave nasal ridgeHP:0011120
Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Wormian bone-multiple fractures-dentinogenesis imperfecta-skeletal dysplasia.

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No actively recruiting trials found for Wormian bone-multiple fractures-dentinogenesis imperfecta-skeletal dysplasia at this time.

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No specialists are currently listed for Wormian bone-multiple fractures-dentinogenesis imperfecta-skeletal 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 Wormian bone-multiple fractures-dentinogenesis imperfecta-skeletal dysplasia.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Wormian bone-multiple fractures-dentinogenesis imperfecta-skeletal 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.What is the specific genetic cause of my child's condition, and should we pursue genetic testing?,Would bisphosphonate therapy be appropriate, and what are the potential benefits and risks?,How can we best protect against fractures during daily activities?,What dental treatments are recommended to manage dentinogenesis imperfecta?,Are there any clinical trials or emerging therapies we should know about?,What physical activities are safe, and should we work with a physical therapist?,Should other family members be tested for this condition?

Common questions about Wormian bone-multiple fractures-dentinogenesis imperfecta-skeletal dysplasia

What is Wormian bone-multiple fractures-dentinogenesis imperfecta-skeletal dysplasia?

Wormian bone-multiple fractures-dentinogenesis imperfecta-skeletal dysplasia is an extremely rare genetic bone disorder. This condition is sometimes grouped within the broader family of osteogenesis imperfecta-like disorders, though it has distinct features that set it apart. The disease affects the way bones and teeth develop, leading to a combination of characteristic problems. People with this condition typically have Wormian bones, which are extra small bone pieces found within the skull's natural joints (sutures). They also experience multiple bone fractures, often from minor injuries or

How is Wormian bone-multiple fractures-dentinogenesis imperfecta-skeletal dysplasia inherited?

Wormian bone-multiple fractures-dentinogenesis imperfecta-skeletal dysplasia follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Wormian bone-multiple fractures-dentinogenesis imperfecta-skeletal dysplasia typically begin?

Typical onset of Wormian bone-multiple fractures-dentinogenesis imperfecta-skeletal dysplasia is neonatal. Age of onset can vary across affected individuals.