Distal limb deficiencies-micrognathia syndrome

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ORPHA:1307OMIM:246560Q92.3
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8Treatment centers

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Overview

Distal limb deficiencies-micrognathia syndrome is an extremely rare genetic condition that is present at birth. It is also sometimes referred to as a chromosomal duplication syndrome because it is associated with a partial trisomy, meaning there is extra chromosomal material (classified under ICD-10 as Q92.3, which covers duplications seen only at prometaphase or involving partial trisomies). The condition primarily affects the development of the limbs and the jaw. Children born with this syndrome typically have underdeveloped or missing parts of the hands and feet (called distal limb deficiencies), along with an abnormally small lower jaw (micrognathia). The small jaw can cause difficulties with feeding and breathing, especially in newborns. Additional features may include other skeletal abnormalities and growth problems. Because this syndrome is so rare, there are very few reported cases in the medical literature, and the full range of symptoms may not yet be completely understood. There is currently no cure for this condition. Treatment focuses on managing symptoms and may involve surgery to improve limb function or correct jaw abnormalities, as well as supportive therapies such as feeding assistance, physical therapy, and occupational therapy. A team of specialists is usually needed to provide comprehensive care.

Also known as:

Key symptoms:

Missing or underdeveloped fingers or toesShortened hands or feetVery small lower jaw (micrognathia)Feeding difficulties in newbornsBreathing problems due to small jawSkeletal abnormalitiesGrowth delaysAbnormal bone development in the arms or legsPossible cleft palateLow birth weight

Clinical phenotype terms (30)— hover any for plain English
Abnormality of the ankleHP:0003028OligodactylyHP:0012165MicroglossiaHP:0000171
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 ↗OMIM ↗NORD ↗

FDA & Trial Timeline

1 event
Sep 2021Single-stage Surgery With Antibiotic-loaded Hydrogel Coated Implants Versus Two Stage Surgery for Secondary Prevention of Complex Chronic Periprosthetic Hip Joint Infection

Centre Hospitalier Universitaire de Saint Etienne — NA

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Distal limb deficiencies-micrognathia syndrome.

View clinical trials →

No actively recruiting trials found for Distal limb deficiencies-micrognathia syndrome at this time.

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

Search ClinicalTrials.gov ↗Join the Distal limb deficiencies-micrognathia syndrome community →

No specialists are currently listed for Distal limb deficiencies-micrognathia 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 Distal limb deficiencies-micrognathia syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Distal limb deficiencies-micrognathia syndrome

Disease timeline:

New recruiting trial: Single-stage Surgery With Antibiotic-loaded Hydrogel Coated Implants Versus Two Stage Surgery for Secondary Prevention of Complex Chronic Periprosthetic Hip Joint Infection

A new clinical trial is recruiting patients for Distal limb deficiencies-micrognathia syndrome

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 is the exact chromosomal change in my child, and what does it mean?,How severe are the limb and jaw abnormalities, and what treatments are recommended?,Will my child need surgery, and if so, when is the best time?,What therapies should we start right away to support development?,Are there any breathing or feeding concerns we need to monitor closely?,What is the chance of this happening again in a future pregnancy?,Are there any specialists or centers with experience in this specific condition?

Common questions about Distal limb deficiencies-micrognathia syndrome

What is Distal limb deficiencies-micrognathia syndrome?

Distal limb deficiencies-micrognathia syndrome is an extremely rare genetic condition that is present at birth. It is also sometimes referred to as a chromosomal duplication syndrome because it is associated with a partial trisomy, meaning there is extra chromosomal material (classified under ICD-10 as Q92.3, which covers duplications seen only at prometaphase or involving partial trisomies). The condition primarily affects the development of the limbs and the jaw. Children born with this syndrome typically have underdeveloped or missing parts of the hands and feet (called distal limb deficien

At what age does Distal limb deficiencies-micrognathia syndrome typically begin?

Typical onset of Distal limb deficiencies-micrognathia syndrome is neonatal. Age of onset can vary across affected individuals.