Robin sequence-oligodactyly syndrome

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ORPHA:3104OMIM:172880Q87.0
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Overview

Robin sequence-oligodactyly syndrome is an extremely rare genetic condition that combines two main features: Pierre Robin sequence and oligodactyly (having fewer fingers or toes than normal). Pierre Robin sequence refers to a group of problems present at birth that includes a very small lower jaw (micrognathia), a tongue that falls back into the throat (glossoptosis), and often a cleft palate (an opening in the roof of the mouth). Oligodactyly means that one or more fingers or toes are missing or underdeveloped. Because of the small jaw and tongue positioning, newborns with this condition may have serious breathing difficulties and feeding problems right from birth. The cleft palate, when present, adds to feeding challenges and may affect speech development later in life. The hand and foot differences can range from mild to more significant, potentially affecting grip and fine motor skills. Treatment focuses on managing each symptom individually. Breathing problems may require positioning techniques, special airways, or sometimes surgery. Cleft palate is typically repaired surgically. Feeding support, speech therapy, and hand therapy or reconstructive surgery for the fingers and toes may also be needed. A team of specialists working together provides the best outcomes for children with this syndrome.

Also known as:

Key symptoms:

Very small lower jawTongue falling back into the throatCleft palate (opening in the roof of the mouth)Missing or underdeveloped fingersMissing or underdeveloped toesBreathing difficulties at birthFeeding problems in infancyFailure to thrive or poor weight gainSpeech difficultiesEar infectionsHearing problems

Clinical phenotype terms (10)— hover any for plain English
Finger aplasiaHP:0009380
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 Robin sequence-oligodactyly syndrome.

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No actively recruiting trials found for Robin sequence-oligodactyly syndrome at this time.

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No specialists are currently listed for Robin sequence-oligodactyly 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 Robin sequence-oligodactyly syndrome.

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Community

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Caregiver Resources

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Mental Health Support

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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 is my child's airway obstruction, and what is the plan to manage it?,Will my child need surgery for the cleft palate, and when is the best timing?,What feeding strategies or equipment do you recommend?,Are there surgical options to improve hand or foot function?,Should we pursue genetic testing, and what might it tell us?,What is the chance of this condition occurring again in future pregnancies?,What therapies (speech, occupational) should we start, and when?

Common questions about Robin sequence-oligodactyly syndrome

What is Robin sequence-oligodactyly syndrome?

Robin sequence-oligodactyly syndrome is an extremely rare genetic condition that combines two main features: Pierre Robin sequence and oligodactyly (having fewer fingers or toes than normal). Pierre Robin sequence refers to a group of problems present at birth that includes a very small lower jaw (micrognathia), a tongue that falls back into the throat (glossoptosis), and often a cleft palate (an opening in the roof of the mouth). Oligodactyly means that one or more fingers or toes are missing or underdeveloped. Because of the small jaw and tongue positioning, newborns with this condition may

How is Robin sequence-oligodactyly syndrome inherited?

Robin sequence-oligodactyly syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Robin sequence-oligodactyly syndrome typically begin?

Typical onset of Robin sequence-oligodactyly syndrome is neonatal. Age of onset can vary across affected individuals.