Acrocardiofacial syndrome

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ORPHA:2008OMIM:600460Q87.8
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Overview

Acrocardiofacial syndrome is an extremely rare genetic condition that affects multiple parts of the body, particularly the hands and feet (acro-), the heart (cardio-), and the face (facial). This syndrome is sometimes also called acrocardiofacial syndrome or referred to by its Orphanet designation ORPHA:2008. Babies born with this condition typically show a combination of birth defects including heart malformations, distinctive facial features, and limb abnormalities such as split hands or feet (also called ectrodactyly or lobster-claw deformity). Facial features may include a flat or broad nasal bridge, cleft lip or palate, and other craniofacial differences. The heart defects associated with this syndrome can range in severity and may include structural problems such as ventricular septal defects or other congenital heart malformations. Because the condition affects several organ systems at once, affected children often need care from multiple specialists. Treatment is primarily supportive and surgical, focusing on correcting heart defects, improving limb function, and addressing facial differences. Due to the extreme rarity of this condition, much of what is known comes from a very small number of reported cases in the medical literature. Early diagnosis and coordinated care are important for giving affected children the best possible outcomes.

Also known as:

Key symptoms:

Split hands or feet (lobster-claw deformity)Congenital heart defectsUnusual facial featuresCleft lip or cleft palateFlat or broad nasal bridgeShort stature or growth delaysAbnormally shaped or missing fingers or toesWidely spaced eyesLow-set earsPossible intellectual disability or developmental delaysSkeletal abnormalities

Clinical phenotype terms (37)— hover any for plain English
Split footHP:0001839
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 ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Acrocardiofacial syndrome.

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

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No specialists are currently listed for Acrocardiofacial 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 Acrocardiofacial syndrome.

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Community

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Latest news about Acrocardiofacial syndrome

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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.How severe is my child's heart defect, and will surgery be needed?,What surgeries or procedures will be needed for the limb and facial differences?,Should we pursue genetic testing, and what might it tell us?,What is the chance of having another child with this condition?,What therapies should we start early to support my child's development?,Are there any specialists or centers with experience treating this syndrome?,What signs of complications should I watch for at home?

Common questions about Acrocardiofacial syndrome

What is Acrocardiofacial syndrome?

Acrocardiofacial syndrome is an extremely rare genetic condition that affects multiple parts of the body, particularly the hands and feet (acro-), the heart (cardio-), and the face (facial). This syndrome is sometimes also called acrocardiofacial syndrome or referred to by its Orphanet designation ORPHA:2008. Babies born with this condition typically show a combination of birth defects including heart malformations, distinctive facial features, and limb abnormalities such as split hands or feet (also called ectrodactyly or lobster-claw deformity). Facial features may include a flat or broad na

How is Acrocardiofacial syndrome inherited?

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

At what age does Acrocardiofacial syndrome typically begin?

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