Craniofaciofrontodigital syndrome

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ORPHA:363705OMIM:114620Q87.0
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8Treatment centers

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Overview

Craniofaciofrontodigital syndrome is an extremely rare genetic condition that affects multiple parts of the body, particularly the skull (cranio), face (facio), forehead (fronto), and fingers or toes (digital). This syndrome belongs to a group of conditions where bones and other structures do not develop as expected during growth in the womb and early childhood. People with this condition may have unusual facial features, abnormalities of the skull shape, a prominent or broad forehead, and differences in the fingers or toes such as short digits, curved fingers, or broad thumbs. Some individuals may also experience intellectual disability or developmental delays. Because this syndrome is so rare, the medical understanding of it is still limited. It falls under the broader ICD-10 category Q87.0, which covers congenital malformation syndromes that mainly affect facial appearance. The condition is typically recognized at birth or in early infancy due to the visible physical features. Diagnosis usually involves a careful clinical examination by a geneticist, along with imaging studies and genetic testing. There is currently no cure for craniofaciofrontodigital syndrome. Treatment focuses on managing individual symptoms and may include surgery for skull or facial abnormalities, hand therapy or surgery for digit differences, and developmental support services such as speech therapy, occupational therapy, and special education. A team of specialists working together provides the best care for affected individuals.

Also known as:

Key symptoms:

Unusual skull shapeBroad or prominent foreheadWidely spaced eyesFlat or broad nasal bridgeShort fingers or toesBroad thumbs or big toesCurved fingersAbnormal facial featuresDevelopmental delaysIntellectual disabilityLow-set earsCraniosynostosis (early fusion of skull bones)Short stature

Clinical phenotype terms (50)— hover any for plain English
Congenital, generalized hypertrichosisHP:0004540Thick hairHP:0100874Prominent superficial veinsHP:0001015
Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

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 Craniofaciofrontodigital syndrome.

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No actively recruiting trials found for Craniofaciofrontodigital syndrome at this time.

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No specialists are currently listed for Craniofaciofrontodigital 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 Craniofaciofrontodigital syndrome.

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Community

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Latest news about Craniofaciofrontodigital syndrome

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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 specific features does my child have, and how severe are they?,Is genetic testing recommended, and what might it tell us?,Will my child need surgery for skull or hand abnormalities, and when?,What developmental therapies should we start, and how often?,What is the chance this condition could occur again in future pregnancies?,Are there any signs I should watch for that would need emergency care?,Can you connect us with other families or support groups for rare craniofacial conditions?

Common questions about Craniofaciofrontodigital syndrome

What is Craniofaciofrontodigital syndrome?

Craniofaciofrontodigital syndrome is an extremely rare genetic condition that affects multiple parts of the body, particularly the skull (cranio), face (facio), forehead (fronto), and fingers or toes (digital). This syndrome belongs to a group of conditions where bones and other structures do not develop as expected during growth in the womb and early childhood. People with this condition may have unusual facial features, abnormalities of the skull shape, a prominent or broad forehead, and differences in the fingers or toes such as short digits, curved fingers, or broad thumbs. Some individual

At what age does Craniofaciofrontodigital syndrome typically begin?

Typical onset of Craniofaciofrontodigital syndrome is neonatal. Age of onset can vary across affected individuals.