Von Voss-Cherstvoy syndrome

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ORPHA:3439OMIM:223340Q87.8
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8Treatment centers

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Overview

Von Voss-Cherstvoy syndrome is an extremely rare genetic condition that affects multiple body systems, particularly during development before and shortly after birth. This syndrome is also sometimes referred to as Von Voss-Cherstvoy-Kemperdick syndrome. It is classified under multiple congenital anomaly syndromes, meaning that affected individuals are born with a combination of birth defects involving several organ systems. The condition is characterized by a combination of features that may include heart defects, abnormalities of the hands and feet (such as extra fingers or toes, or fused digits), skeletal malformations, and sometimes abnormalities of the urinary and genital systems. Some affected individuals may also have facial differences and developmental delays. Because the syndrome is so rare, the full range of symptoms and their severity can vary from person to person. There is currently no cure for Von Voss-Cherstvoy syndrome. Treatment focuses on managing individual symptoms and may involve surgery for heart defects or limb abnormalities, physical therapy, and supportive care. A team of specialists typically works together to address the various needs of affected individuals. Early diagnosis and intervention can help improve quality of life.

Also known as:

Key symptoms:

Heart defects present at birthExtra fingers or toes (polydactyly)Fused fingers or toes (syndactyly)Skeletal abnormalitiesAbnormalities of the urinary tract or kidneysGenital abnormalitiesUnusual facial featuresShort stature or growth delaysDevelopmental delaysLimb malformations

Clinical phenotype terms (23)— hover any for plain English
Occipital encephaloceleHP:0002085Aplasia/hypoplasia involving bones of the upper limbsHP:0006496Upper limb phocomeliaHP:0009813Hydrocele testisHP:0000034Tooth malpositionHP:0000692
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 Von Voss-Cherstvoy syndrome.

View clinical trials →

No actively recruiting trials found for Von Voss-Cherstvoy syndrome at this time.

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

Search ClinicalTrials.gov ↗Join the Von Voss-Cherstvoy syndrome community →

No specialists are currently listed for Von Voss-Cherstvoy 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 Von Voss-Cherstvoy syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Von Voss-Cherstvoy 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 birth defects does my child have, and how severe are they?,Will my child need surgery, and if so, when is the best time?,What kind of genetic testing should we pursue to confirm the diagnosis?,What is the long-term outlook for my child's heart and kidney function?,What therapies or early intervention services should we start?,What are the chances of having another child with this condition?,Are there any specialists or centers with experience in this syndrome?

Common questions about Von Voss-Cherstvoy syndrome

What is Von Voss-Cherstvoy syndrome?

Von Voss-Cherstvoy syndrome is an extremely rare genetic condition that affects multiple body systems, particularly during development before and shortly after birth. This syndrome is also sometimes referred to as Von Voss-Cherstvoy-Kemperdick syndrome. It is classified under multiple congenital anomaly syndromes, meaning that affected individuals are born with a combination of birth defects involving several organ systems. The condition is characterized by a combination of features that may include heart defects, abnormalities of the hands and feet (such as extra fingers or toes, or fused di

How is Von Voss-Cherstvoy syndrome inherited?

Von Voss-Cherstvoy syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Von Voss-Cherstvoy syndrome typically begin?

Typical onset of Von Voss-Cherstvoy syndrome is neonatal. Age of onset can vary across affected individuals.