Orofaciodigital syndrome type 9

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ORPHA:141007OMIM:258865Q87.0
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Overview

Orofaciodigital syndrome type 9 (OFD9), also known as orofaciodigital syndrome with retinal abnormalities or OFD syndrome type IX, is an extremely rare genetic condition that affects the mouth (oral), face (facial), and fingers and toes (digital). It belongs to a larger group of conditions called orofaciodigital syndromes, of which there are multiple types. People with OFD9 may have cleft lip or cleft palate, a tongue with small bumps or growths (hamartomas), extra fingers or toes (polydactyly), and abnormalities of the eyes including retinal problems that can affect vision. Facial features may include a broad or flat nasal bridge and widely spaced eyes. Some individuals may also have intellectual disability or developmental delays. The condition is typically present at birth or recognized in early infancy. Because OFD9 is so rare, with only a handful of cases described in the medical literature, there is no specific cure. Treatment focuses on managing individual symptoms. This may include surgery for cleft lip or palate, removal of oral growths, correction of extra digits, and monitoring of vision. A team of specialists is usually needed to provide comprehensive care. Early intervention services can help children who have developmental delays reach their full potential.

Also known as:

Key symptoms:

Cleft lip or cleft palateSmall growths or bumps on the tongueExtra fingers or toesAbnormalities of the retina in the eyesVision problemsWidely spaced eyesBroad or flat nasal bridgeShort statureIntellectual disability or learning difficultiesDevelopmental delaysAbnormally shaped teeth or missing teethFused or webbed fingers or toesSmall jaw

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 9.

View clinical trials →

No actively recruiting trials found for Orofaciodigital syndrome type 9 at this time.

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

Search ClinicalTrials.gov ↗Join the Orofaciodigital syndrome type 9 community →

No specialists are currently listed for Orofaciodigital syndrome type 9.

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 9.

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Community

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Latest news about Orofaciodigital syndrome type 9

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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 OFD9 does my child have, and how severe are they?,Should we pursue genetic testing, and what type of testing do you recommend?,What surgeries or procedures will my child need, and when should they be done?,How often should my child's eyes be checked for retinal problems?,What developmental support services should we start, and how soon?,Are there other family members who should be tested or evaluated?,Are there any research studies or registries for orofaciodigital syndromes that we could participate in?

Common questions about Orofaciodigital syndrome type 9

What is Orofaciodigital syndrome type 9?

Orofaciodigital syndrome type 9 (OFD9), also known as orofaciodigital syndrome with retinal abnormalities or OFD syndrome type IX, is an extremely rare genetic condition that affects the mouth (oral), face (facial), and fingers and toes (digital). It belongs to a larger group of conditions called orofaciodigital syndromes, of which there are multiple types. People with OFD9 may have cleft lip or cleft palate, a tongue with small bumps or growths (hamartomas), extra fingers or toes (polydactyly), and abnormalities of the eyes including retinal problems that can affect vision. Facial features m

How is Orofaciodigital syndrome type 9 inherited?

Orofaciodigital syndrome type 9 follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Orofaciodigital syndrome type 9 typically begin?

Typical onset of Orofaciodigital syndrome type 9 is neonatal. Age of onset can vary across affected individuals.