Genetic syndromic Pierre Robin syndrome

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ORPHA:363294
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Overview

Genetic syndromic Pierre Robin syndrome is a condition present at birth where a baby is born with a very small lower jaw (micrognathia), a tongue that falls back into the throat (glossoptosis), and often a gap in the roof of the mouth (cleft palate). What makes this form 'syndromic' is that these jaw and mouth problems occur as part of a broader genetic syndrome, meaning the child also has other health issues affecting different parts of the body. These additional features can include problems with the eyes, ears, heart, skeleton, brain development, or other organ systems, depending on the specific underlying genetic cause. The combination of a small jaw and a tongue that falls backward can cause serious breathing and feeding difficulties in newborns. Babies may struggle to get enough air, especially when lying on their back, and may have trouble latching or swallowing during feeding. This can lead to poor weight gain and growth problems if not managed early. Treatment depends on the severity of symptoms and the specific syndrome involved. Mild breathing problems may be managed by positioning the baby on their stomach (prone positioning), while more severe cases may require a special airway device, tongue-lip adhesion surgery, or jaw distraction surgery to bring the lower jaw forward. Cleft palate repair is typically performed in the first one to two years of life. Because this is a syndromic condition, children often need care from multiple specialists to address the various body systems that may be affected. Early intervention and coordinated care can significantly improve outcomes.

Key symptoms:

Very small or receding lower jawTongue falling back into the throat causing airway blockageOpening or cleft in the roof of the mouthBreathing difficulties, especially when lying on the backFeeding difficulties and poor weight gainHearing problems or frequent ear infectionsEye abnormalities or vision problemsHeart defectsSkeletal or bone abnormalitiesIntellectual disability or developmental delaysShort stature or growth problemsJoint problems or limited joint movementSpeech and language delaysDistinctive facial features

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 ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Genetic syndromic Pierre Robin syndrome.

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No actively recruiting trials found for Genetic syndromic Pierre Robin syndrome at this time.

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No specialists are currently listed for Genetic syndromic 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 Genetic syndromic Pierre Robin syndrome.

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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.What specific genetic syndrome is causing my child's Pierre Robin sequence, and what does that mean for their overall health?,How severe is my child's airway obstruction, and what treatment approach do you recommend?,What feeding method is safest and most effective for my baby right now?,When will cleft palate repair surgery be recommended, and what should we expect?,What other body systems should be checked, and how often do we need follow-up appointments?,Are there genetic implications for future pregnancies in our family?,What developmental milestones should we watch for, and when should we seek early intervention services?

Common questions about Genetic syndromic Pierre Robin syndrome

What is Genetic syndromic Pierre Robin syndrome?

Genetic syndromic Pierre Robin syndrome is a condition present at birth where a baby is born with a very small lower jaw (micrognathia), a tongue that falls back into the throat (glossoptosis), and often a gap in the roof of the mouth (cleft palate). What makes this form 'syndromic' is that these jaw and mouth problems occur as part of a broader genetic syndrome, meaning the child also has other health issues affecting different parts of the body. These additional features can include problems with the eyes, ears, heart, skeleton, brain development, or other organ systems, depending on the spe

At what age does Genetic syndromic Pierre Robin syndrome typically begin?

Typical onset of Genetic syndromic Pierre Robin syndrome is neonatal. Age of onset can vary across affected individuals.