Pierre Robin syndrome associated with branchial archs anomalies

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ORPHA:138050
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Overview

Pierre Robin syndrome associated with branchial arch anomalies is a rare condition that affects how certain structures of the face, jaw, and neck develop before birth. The branchial arches are tissue structures in the developing embryo that give rise to the jaw, ears, neck muscles, and other head and neck features. When these arches do not develop properly, it can lead to a combination of problems. The hallmark features of Pierre Robin sequence include 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). When this occurs alongside branchial arch anomalies, patients may also have ear abnormalities, hearing loss, facial asymmetry, and neck or throat structural differences. These combined problems can cause serious breathing and feeding difficulties, especially in newborns. Treatment depends on the severity of symptoms. Breathing problems may require positioning techniques, special airways, or sometimes surgery. Feeding support, cleft palate repair, jaw surgery, hearing aids, and speech therapy are common parts of the treatment plan. A team of specialists typically works together to manage the various aspects of this condition throughout childhood and beyond.

Also known as:

Key symptoms:

Very small or receding lower jawTongue falling back into the throat causing airway blockageOpening or cleft in the roof of the mouthDifficulty breathing, especially when lying on the backFeeding difficulties and poor weight gainEar malformations or abnormally shaped earsHearing lossFacial asymmetry or uneven facial featuresNeck abnormalities such as cysts or fistulasSpeech and language delaysDental problems or misaligned teethRecurrent ear infectionsSwallowing difficulties

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 Pierre Robin syndrome associated with branchial archs anomalies.

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No actively recruiting trials found for Pierre Robin syndrome associated with branchial archs anomalies at this time.

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No specialists are currently listed for Pierre Robin syndrome associated with branchial archs anomalies.

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 associated with branchial archs anomalies.

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Community

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Caregiver Resources

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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 is my child's airway obstruction, and what is the plan to manage it?,Will my child need surgery, and if so, what type and when?,What feeding method is best for my child right now?,Should we pursue genetic testing to find the underlying cause?,What is the long-term outlook for my child's jaw growth and facial development?,How often should my child's hearing be tested?,Are there other family members who should be evaluated or tested?

Common questions about Pierre Robin syndrome associated with branchial archs anomalies

What is Pierre Robin syndrome associated with branchial archs anomalies?

Pierre Robin syndrome associated with branchial arch anomalies is a rare condition that affects how certain structures of the face, jaw, and neck develop before birth. The branchial arches are tissue structures in the developing embryo that give rise to the jaw, ears, neck muscles, and other head and neck features. When these arches do not develop properly, it can lead to a combination of problems. The hallmark features of Pierre Robin sequence include 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 (clef

At what age does Pierre Robin syndrome associated with branchial archs anomalies typically begin?

Typical onset of Pierre Robin syndrome associated with branchial archs anomalies is neonatal. Age of onset can vary across affected individuals.