Craniosynostosis-dysmorphism-brachydactyly syndrome

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ORPHA:1535
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Overview

Craniosynostosis-dysmorphism-brachydactyly syndrome is an extremely rare genetic condition that affects the development of the skull, face, and hands. The name describes its three main features: craniosynostosis (early fusion of the skull bones), dysmorphism (unusual facial features), and brachydactyly (short fingers and toes). In this condition, one or more of the joints (called sutures) between the bones of a baby's skull close too early, before the brain has finished growing. This can lead to an abnormal head shape and, in some cases, increased pressure inside the skull. Affected individuals may also have distinctive facial features such as a flat midface, wide-set eyes, or a small jaw. The short fingers and toes can vary in severity from mildly shortened to noticeably stubby digits. Because this syndrome is so rare, with very few cases described in the medical literature, the full range of symptoms and the best approaches to treatment are still being understood. Treatment is generally focused on managing symptoms and may include surgery to correct the skull fusion, as well as supportive therapies for hand function and any developmental concerns. A team of specialists is usually needed to provide comprehensive care.

Also known as:

Key symptoms:

Early fusion of skull bones causing abnormal head shapeShort fingers and toesUnusual facial featuresFlat or underdeveloped midfaceWide-set eyesSmall or recessed jawPossible increased pressure inside the skullPossible developmental delaysShort statureLimited range of motion in fingersBroad thumbs or big toes

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 ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Craniosynostosis-dysmorphism-brachydactyly syndrome.

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No actively recruiting trials found for Craniosynostosis-dysmorphism-brachydactyly syndrome at this time.

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Search ClinicalTrials.gov ↗Join the Craniosynostosis-dysmorphism-brachydactyly syndrome community →

No specialists are currently listed for Craniosynostosis-dysmorphism-brachydactyly syndrome.

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 Craniosynostosis-dysmorphism-brachydactyly syndrome.

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Community

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Latest news about Craniosynostosis-dysmorphism-brachydactyly syndrome

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Caregiver Resources

NORD Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.Does my child need surgery for the skull fusion, and if so, when is the best time?,How will you monitor for increased pressure inside the skull?,Should we pursue genetic testing to identify the specific cause?,What developmental milestones should I watch for, and when should I be concerned?,Will my child need hand therapy or surgery for the short fingers?,What is the chance that future children could have the same condition?,Are there any clinical trials or research studies we should know about?

Common questions about Craniosynostosis-dysmorphism-brachydactyly syndrome

What is Craniosynostosis-dysmorphism-brachydactyly syndrome?

Craniosynostosis-dysmorphism-brachydactyly syndrome is an extremely rare genetic condition that affects the development of the skull, face, and hands. The name describes its three main features: craniosynostosis (early fusion of the skull bones), dysmorphism (unusual facial features), and brachydactyly (short fingers and toes). In this condition, one or more of the joints (called sutures) between the bones of a baby's skull close too early, before the brain has finished growing. This can lead to an abnormal head shape and, in some cases, increased pressure inside the skull. Affected individual

How is Craniosynostosis-dysmorphism-brachydactyly syndrome inherited?

Craniosynostosis-dysmorphism-brachydactyly syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Craniosynostosis-dysmorphism-brachydactyly syndrome typically begin?

Typical onset of Craniosynostosis-dysmorphism-brachydactyly syndrome is neonatal. Age of onset can vary across affected individuals.