OBSOLETE: Orofaciodigital syndrome type 10

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ORPHA:2756
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Overview

Orofaciodigital syndrome type 10 (OFD10), also known as Figuera syndrome or orofaciodigital syndrome with fibular aplasia, is an extremely rare genetic condition that affects the development of the mouth (oral), face (facial), and fingers and toes (digital). This specific subtype has been classified as 'obsolete' in some medical databases, meaning it may now be grouped under a broader category of orofaciodigital syndromes or reclassified based on newer genetic understanding. The condition was originally described in a very small number of patients and is characterized by features such as cleft lip or palate, abnormalities of the tongue, facial asymmetry, and malformations of the fingers and toes including shortening or absence of certain bones. Some patients also had absence or underdevelopment of the fibula (the smaller bone in the lower leg). Because so few cases have been reported, our understanding of this condition is limited. Treatment is mainly supportive and focuses on managing the specific birth defects present in each individual, which may include surgery for cleft palate, orthopedic care for limb differences, dental care, and speech therapy. A team of specialists is usually needed to provide comprehensive care.

Also known as:

Key symptoms:

Cleft lip or cleft palateAbnormalities of the tongue such as extra tissue or lobulated tongueShortening or malformation of fingers or toesAbsence or underdevelopment of the fibula (lower leg bone)Facial asymmetryWidely spaced eyesBroad or flat nasal bridgeExtra fingers or toes (polydactyly)Short statureDental abnormalities such as missing or extra teethLimb length differencesDifficulty with feeding in infancy

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 OBSOLETE: Orofaciodigital syndrome type 10.

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No actively recruiting trials found for OBSOLETE: Orofaciodigital syndrome type 10 at this time.

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No specialists are currently listed for OBSOLETE: Orofaciodigital syndrome type 10.

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 OBSOLETE: Orofaciodigital syndrome type 10.

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Community

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Latest news about OBSOLETE: Orofaciodigital syndrome type 10

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Caregiver Resources

NORD Caregiver Resources

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Mental Health Support

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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 of orofaciodigital syndrome does my child have, and how severe are they?,Should we pursue genetic testing, and what might it tell us?,What surgeries will my child likely need, and when should they happen?,What therapies (speech, physical, occupational) should we start, and how soon?,Is there a risk of this condition occurring again in future pregnancies?,Are there any other health problems we should screen for?,Can you connect us with other families or support groups for orofaciodigital syndromes?

Common questions about OBSOLETE: Orofaciodigital syndrome type 10

What is OBSOLETE: Orofaciodigital syndrome type 10?

Orofaciodigital syndrome type 10 (OFD10), also known as Figuera syndrome or orofaciodigital syndrome with fibular aplasia, is an extremely rare genetic condition that affects the development of the mouth (oral), face (facial), and fingers and toes (digital). This specific subtype has been classified as 'obsolete' in some medical databases, meaning it may now be grouped under a broader category of orofaciodigital syndromes or reclassified based on newer genetic understanding. The condition was originally described in a very small number of patients and is characterized by features such as cleft

How is OBSOLETE: Orofaciodigital syndrome type 10 inherited?

OBSOLETE: Orofaciodigital syndrome type 10 follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does OBSOLETE: Orofaciodigital syndrome type 10 typically begin?

Typical onset of OBSOLETE: Orofaciodigital syndrome type 10 is neonatal. Age of onset can vary across affected individuals.