Lambert syndrome

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ORPHA:1296OMIM:245550Q87.8
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5Specialists8Treatment centers

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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Overview

Lambert syndrome, also known as branchial arch syndrome or Lambert type branchial arch syndrome (Orphanet code 1296), is an extremely rare genetic condition that affects the development of structures derived from the branchial arches during embryonic growth. The branchial arches are structures in the developing embryo that give rise to parts of the face, neck, ears, and throat. In Lambert syndrome, abnormal development of these structures leads to a combination of birth defects that can include facial asymmetry, ear abnormalities (such as small or malformed ears), hearing problems, and other craniofacial differences. Some individuals may also have limb abnormalities or other associated features. Because this condition is so rare, with very few cases described in the medical literature, our understanding of the full range of symptoms and the best approaches to treatment remains limited. Treatment is generally supportive and symptom-based, meaning doctors address each specific problem as it arises. This may include surgery to correct facial or ear abnormalities, hearing aids or other interventions for hearing loss, and speech therapy if needed. A team of specialists working together typically provides the best care for affected individuals. Early diagnosis and intervention can help improve outcomes and quality of life.

Also known as:

Key symptoms:

Facial asymmetryMalformed or small earsHearing lossAbnormalities of the jaw or lower faceCleft palate or other palate abnormalitiesNeck abnormalities such as branchial cysts or fistulasSpeech difficultiesLimb differencesEye abnormalitiesDental abnormalities

Clinical phenotype terms (16)— hover any for plain English
Intrahepatic biliary atresiaHP:0005248Branchial anomalyHP:0009794
Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

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 Lambert syndrome.

View clinical trials →

No actively recruiting trials found for Lambert syndrome at this time.

New trials open frequently. Follow this disease to get notified.

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Specialists

5 foundView all specialists →
LM
Laurent Servais, MD
Specialist
PI on 6 active trials1 Lambert syndrome publication
KM
Kathy L Aleš, MD
Specialist
PI on 1 active trial
PP
Perry Shieh, MD, PhD
LOS ANGELES, CA
Specialist
PI on 2 active trials
CP
Charles W Gorodetzky, MD, PhD
KANSAS CITY, MO
Specialist
PI on 2 active trials

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 Lambert syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Lambert syndrome

No recent news articles for Lambert syndrome.

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Caregiver Resources

NORD Caregiver Resources

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

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

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.What specific features of Lambert syndrome does my child have, and how severe are they?,Will my child need surgery, and if so, when is the best time?,How should we monitor and manage hearing loss?,What therapies (speech, occupational) would benefit my child?,Is genetic testing available to confirm the diagnosis and help with family planning?,What is the expected long-term outlook for my child?,Are there any clinical studies or registries we should consider joining?

Common questions about Lambert syndrome

What is Lambert syndrome?

Lambert syndrome, also known as branchial arch syndrome or Lambert type branchial arch syndrome (Orphanet code 1296), is an extremely rare genetic condition that affects the development of structures derived from the branchial arches during embryonic growth. The branchial arches are structures in the developing embryo that give rise to parts of the face, neck, ears, and throat. In Lambert syndrome, abnormal development of these structures leads to a combination of birth defects that can include facial asymmetry, ear abnormalities (such as small or malformed ears), hearing problems, and other c

How is Lambert syndrome inherited?

Lambert syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Lambert syndrome typically begin?

Typical onset of Lambert syndrome is neonatal. Age of onset can vary across affected individuals.

Which specialists treat Lambert syndrome?

5 specialists and care centers treating Lambert syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.