Orofaciodigital syndrome type 11

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ORPHA:141000OMIM:612913Q87.0
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Overview

Orofaciodigital syndrome type 11 (OFD11), also known as orofaciodigital syndrome type XI or Gabrielli syndrome, is an extremely rare genetic condition that affects the development of the mouth (oral), face (facial), and fingers and toes (digital). This syndrome belongs to a larger group of orofaciodigital syndromes, which share overlapping features but have distinct characteristics. People with OFD11 may have abnormalities of the mouth such as a cleft palate or tongue abnormalities, unusual facial features, and extra fingers or toes (polydactyly) or other hand and foot differences. Some individuals may also experience intellectual disability and other developmental challenges. The condition is present from birth and is typically recognized in the newborn period or early infancy based on the combination of physical features. Because OFD11 is so rare, with only a handful of cases described in the medical literature, there is no specific cure or targeted treatment. Management focuses on addressing individual symptoms, which may include surgery for cleft palate or extra digits, speech therapy, developmental support, and regular monitoring by a team of specialists. Research into the genetic causes and potential treatments is ongoing but limited by the rarity of the condition.

Also known as:

Key symptoms:

Extra fingers or toes (polydactyly)Cleft palate or cleft lipTongue abnormalities such as lumps or clefts on the tongueUnusual facial featuresIntellectual disabilityDevelopmental delaysHearing lossWidely spaced eyesFlat nasal bridgeShort statureAbnormalities of the teethFused or webbed fingers or 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 ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Orofaciodigital syndrome type 11.

View clinical trials →

No actively recruiting trials found for Orofaciodigital syndrome type 11 at this time.

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

Search ClinicalTrials.gov ↗Join the Orofaciodigital syndrome type 11 community →

No specialists are currently listed for Orofaciodigital syndrome type 11.

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 Orofaciodigital syndrome type 11.

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Community

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Latest news about Orofaciodigital syndrome type 11

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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 of OFD11 does my child have, and how severe are they?,Is genetic testing recommended for our family, and what would the results mean?,What surgeries or procedures will my child need, and when should they happen?,What developmental milestones should I watch for, and when should I be concerned?,What therapies (speech, occupational, physical) should we start, and how soon?,Are there any other organs or systems we should monitor over time?,Can you connect us with other families or support groups for orofaciodigital syndromes?

Common questions about Orofaciodigital syndrome type 11

What is Orofaciodigital syndrome type 11?

Orofaciodigital syndrome type 11 (OFD11), also known as orofaciodigital syndrome type XI or Gabrielli syndrome, is an extremely rare genetic condition that affects the development of the mouth (oral), face (facial), and fingers and toes (digital). This syndrome belongs to a larger group of orofaciodigital syndromes, which share overlapping features but have distinct characteristics. People with OFD11 may have abnormalities of the mouth such as a cleft palate or tongue abnormalities, unusual facial features, and extra fingers or toes (polydactyly) or other hand and foot differences. Some indiv

How is Orofaciodigital syndrome type 11 inherited?

Orofaciodigital syndrome type 11 follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Orofaciodigital syndrome type 11 typically begin?

Typical onset of Orofaciodigital syndrome type 11 is neonatal. Age of onset can vary across affected individuals.