Contractures-developmental delay-Pierre Robin syndrome

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ORPHA:436003Q87.0
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Overview

Contractures-developmental delay-Pierre Robin syndrome is an extremely rare genetic condition that affects multiple body systems. It is sometimes referred to by its Orphanet designation (ORPHA:436003). The syndrome combines three main features: joint contractures (stiffness and limited movement in the joints), developmental delay (slower than expected progress in learning, thinking, and motor skills), and Pierre Robin sequence (a combination of a very small lower jaw, a tongue that falls back into the throat, and often a cleft palate — an opening in the roof of the mouth). Babies born with this condition may have breathing and feeding difficulties from birth due to the small jaw and airway obstruction. The joint contractures can affect the fingers, elbows, knees, or other joints, limiting movement and physical development. Developmental delay can range from mild to significant and may affect speech, motor skills, and cognitive abilities. Because this is such a rare condition, the treatment approach is largely supportive and symptom-based. Management typically involves a team of specialists who address breathing problems, feeding challenges, orthopedic issues, and developmental support. Surgical interventions may be needed for the cleft palate or to improve airway function. Physical and occupational therapy can help with joint mobility and developmental progress. Early intervention programs are important to maximize each child's potential.

Also known as:

Key symptoms:

Stiff or tight joints that limit movement (contractures)Small lower jaw (micrognathia)Tongue falling back into the throat (glossoptosis)Cleft palate (opening in the roof of the mouth)Breathing difficulties, especially in newbornsFeeding difficulties in infancyDelayed motor milestones like sitting and walkingDelayed speech and language developmentIntellectual disability or learning difficultiesShort stature or growth delaysLimited range of motion in fingers, elbows, or kneesLow muscle tone (floppiness)

Clinical phenotype terms (36)— hover any for plain English
Obstructive sleep apneaHP:0002870SyringomyeliaHP:0003396Cerebral white matter hypoplasiaHP:0012430Lop earHP:0000394
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 Contractures-developmental delay-Pierre Robin syndrome.

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No actively recruiting trials found for Contractures-developmental delay-Pierre Robin syndrome at this time.

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No specialists are currently listed for Contractures-developmental delay-Pierre Robin 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 Contractures-developmental delay-Pierre Robin syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Caregiver Resources

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Social Security Disability

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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 or jaw, and when is the best timing?,What therapies should we start right away to support my child's development?,Is genetic testing recommended for our family, and what would the results mean for future pregnancies?,What feeding strategies or equipment can help my child gain weight safely?,How often should my child be seen by each specialist on the care team?,Are there any clinical trials or research studies we should know about?

Common questions about Contractures-developmental delay-Pierre Robin syndrome

What is Contractures-developmental delay-Pierre Robin syndrome?

Contractures-developmental delay-Pierre Robin syndrome is an extremely rare genetic condition that affects multiple body systems. It is sometimes referred to by its Orphanet designation (ORPHA:436003). The syndrome combines three main features: joint contractures (stiffness and limited movement in the joints), developmental delay (slower than expected progress in learning, thinking, and motor skills), and Pierre Robin sequence (a combination of a very small lower jaw, a tongue that falls back into the throat, and often a cleft palate — an opening in the roof of the mouth). Babies born with thi

How is Contractures-developmental delay-Pierre Robin syndrome inherited?

Contractures-developmental delay-Pierre Robin syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Contractures-developmental delay-Pierre Robin syndrome typically begin?

Typical onset of Contractures-developmental delay-Pierre Robin syndrome is neonatal. Age of onset can vary across affected individuals.