Pierre Robin syndrome-faciodigital anomaly syndrome

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ORPHA:2888OMIM:311895Q87.8
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Overview

Pierre Robin syndrome-faciodigital anomaly syndrome (also known as Pierre Robin sequence with digital anomalies or Carey-Fineman-Ziter syndrome-like conditions) is an extremely rare genetic condition that combines features of Pierre Robin sequence with abnormalities of the fingers and toes, and sometimes other facial differences. Pierre Robin sequence itself involves a small lower jaw (micrognathia), a tongue that falls back into the throat (glossoptosis), and often a cleft palate — the opening in the roof of the mouth. In this particular syndrome, patients also have unusual findings in their hands and feet, which may include short fingers, extra fingers, or other digit malformations. Because the jaw is small and the tongue tends to fall backward, newborns with this condition can have serious breathing and feeding difficulties right from birth. The cleft palate adds to feeding challenges and may affect speech development later. The digital (finger and toe) anomalies vary in severity from mild shortening to more complex structural differences. Treatment is primarily supportive and depends on the specific problems present. Breathing difficulties may require positioning techniques, special airways, or sometimes surgery. Cleft palate repair is typically performed in infancy. Hand and foot abnormalities may need orthopedic or surgical evaluation. A team of specialists working together is essential for the best outcomes. Because this syndrome is so rare, each patient's care plan is highly individualized.

Also known as:

Key symptoms:

Small lower jawTongue falling back into the throat causing breathing problemsCleft palate (opening in the roof of the mouth)Feeding difficulties in infancyShort or abnormally shaped fingersShort or abnormally shaped toesExtra fingers or toesBreathing problems at birthUnusual facial featuresSpeech delaysHearing problemsFailure to gain weight properlyPossible developmental delays

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 Pierre Robin syndrome-faciodigital anomaly syndrome.

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No actively recruiting trials found for Pierre Robin syndrome-faciodigital anomaly syndrome at this time.

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No specialists are currently listed for Pierre Robin syndrome-faciodigital anomaly 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 Pierre Robin syndrome-faciodigital anomaly syndrome.

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Community

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Latest news about Pierre Robin syndrome-faciodigital anomaly syndrome

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

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.How severe are my child's breathing problems, and what is the best way to manage them?,Will my child need surgery for the cleft palate, and when is the best time?,What kind of genetic testing should we pursue to understand the cause?,Are the finger and toe differences likely to affect my child's ability to use their hands normally?,What specialists should be part of our care team?,Is there a risk of this condition occurring again in future pregnancies?,What developmental milestones should we watch for, and when should we be concerned?

Common questions about Pierre Robin syndrome-faciodigital anomaly syndrome

What is Pierre Robin syndrome-faciodigital anomaly syndrome?

Pierre Robin syndrome-faciodigital anomaly syndrome (also known as Pierre Robin sequence with digital anomalies or Carey-Fineman-Ziter syndrome-like conditions) is an extremely rare genetic condition that combines features of Pierre Robin sequence with abnormalities of the fingers and toes, and sometimes other facial differences. Pierre Robin sequence itself involves a small lower jaw (micrognathia), a tongue that falls back into the throat (glossoptosis), and often a cleft palate — the opening in the roof of the mouth. In this particular syndrome, patients also have unusual findings in their

At what age does Pierre Robin syndrome-faciodigital anomaly syndrome typically begin?

Typical onset of Pierre Robin syndrome-faciodigital anomaly syndrome is neonatal. Age of onset can vary across affected individuals.