Isolated tetra-amelia

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Overview

Isolated tetra-amelia is an extremely rare birth defect in which a baby is born without all four limbs (both arms and both legs). The word 'amelia' means the complete absence of a limb, and 'tetra' means four, so tetra-amelia describes the absence of all four limbs. In the 'isolated' form of this condition, the missing limbs are the main feature, and other major organ problems that can sometimes accompany tetra-amelia (such as lung, skull, or genital abnormalities) are not present or are minimal. This condition is apparent at birth or even earlier during prenatal ultrasound imaging. The exact cause of isolated tetra-amelia is not always identified, but in some families it has been linked to changes in the WNT3 gene, which plays a key role in limb development during early pregnancy. The condition is inherited in an autosomal recessive pattern in these families, meaning both parents carry one copy of the altered gene without showing symptoms themselves. There is no cure for isolated tetra-amelia. Treatment focuses on supportive care, adaptive devices, and prosthetics to help the individual achieve as much independence as possible. Physical therapy, occupational therapy, and psychological support are important parts of long-term management. Despite the significant physical challenges, many individuals with this condition can lead meaningful lives with appropriate support and assistive technology.

Also known as:

Key symptoms:

Complete absence of all four limbs from birthShortened or absent shoulder and hip structuresDifficulty with mobility and movementInability to perform tasks requiring hands or armsPossible mild abnormalities of the pelvis or shoulder bones

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 Isolated tetra-amelia.

View clinical trials →

No actively recruiting trials found for Isolated tetra-amelia at this time.

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

Search ClinicalTrials.gov ↗Join the Isolated tetra-amelia community →

No specialists are currently listed for Isolated tetra-amelia.

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 Isolated tetra-amelia.

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Community

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Latest news about Isolated tetra-amelia

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Caregiver Resources

NORD Caregiver Resources

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

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.Is my child's condition truly isolated, or could there be other organ involvement we should check for?,Should we pursue genetic testing for WNT3, and what would the results mean for future pregnancies?,At what age should we begin fitting prosthetic limbs, and what types are recommended?,What physical and occupational therapy programs are best for children with tetra-amelia?,Are there clinical trials or emerging technologies that might benefit my child?,What psychological support services are available for our family?,How can we best prepare for school accommodations and long-term independence?

Common questions about Isolated tetra-amelia

What is Isolated tetra-amelia?

Isolated tetra-amelia is an extremely rare birth defect in which a baby is born without all four limbs (both arms and both legs). The word 'amelia' means the complete absence of a limb, and 'tetra' means four, so tetra-amelia describes the absence of all four limbs. In the 'isolated' form of this condition, the missing limbs are the main feature, and other major organ problems that can sometimes accompany tetra-amelia (such as lung, skull, or genital abnormalities) are not present or are minimal. This condition is apparent at birth or even earlier during prenatal ultrasound imaging. The exact

How is Isolated tetra-amelia inherited?

Isolated tetra-amelia follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Isolated tetra-amelia typically begin?

Typical onset of Isolated tetra-amelia is neonatal. Age of onset can vary across affected individuals.