Spondylocamptodactyly syndrome

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ORPHA:3180OMIM:600000Q87.5
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Overview

Spondylocamptodactyly syndrome is an extremely rare genetic condition that affects both the spine and the fingers. The name comes from combining 'spondylo' (meaning spine), 'campto' (meaning bent), and 'dactyly' (meaning fingers or toes). People with this condition typically have abnormalities of the vertebrae (the bones that make up the spine) along with camptodactyly, which is a permanent bending or curling of one or more fingers that cannot be fully straightened. The spinal problems may include tall or flattened vertebral bodies, irregular shapes of the vertebrae, and sometimes mild scoliosis or other spinal curvature issues. These skeletal changes are usually present from birth or early childhood. Because this syndrome is so rare, with only a handful of cases described in the medical literature, our understanding of the full range of symptoms and the best treatments is still limited. There is currently no cure for spondylocamptodactyly syndrome. Treatment focuses on managing symptoms and may include physical therapy to improve hand function, orthopedic care for spinal issues, and regular monitoring of growth and skeletal development. Some patients may benefit from occupational therapy to help with daily tasks affected by finger contractures. The long-term outlook varies depending on the severity of the skeletal involvement, but many individuals can lead relatively functional lives with appropriate supportive care.

Key symptoms:

Permanently bent fingers that cannot be straightenedAbnormally shaped spinal bonesShort stature or growth delaysFlattened vertebrae in the spineStiff or limited movement in finger jointsMild curvature of the spineTall vertebral bodies on X-rayDifficulty gripping or holding objectsJoint stiffness in the hands

Clinical phenotype terms (3)— hover any for plain English
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 Spondylocamptodactyly syndrome.

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

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No specialists are currently listed for Spondylocamptodactyly 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 Spondylocamptodactyly syndrome.

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Community

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Caregiver Resources

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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 are my child's spinal and finger abnormalities, and are they likely to get worse?,Would genetic testing help us understand the cause and recurrence risk for future children?,What types of therapy would be most helpful for improving hand function?,Is surgery an option for the finger contractures, and when would it be considered?,How often should we have follow-up X-rays of the spine?,Are there any adaptive devices or tools that could help with daily activities?,Should we see a genetic counselor to discuss family planning?

Common questions about Spondylocamptodactyly syndrome

What is Spondylocamptodactyly syndrome?

Spondylocamptodactyly syndrome is an extremely rare genetic condition that affects both the spine and the fingers. The name comes from combining 'spondylo' (meaning spine), 'campto' (meaning bent), and 'dactyly' (meaning fingers or toes). People with this condition typically have abnormalities of the vertebrae (the bones that make up the spine) along with camptodactyly, which is a permanent bending or curling of one or more fingers that cannot be fully straightened. The spinal problems may include tall or flattened vertebral bodies, irregular shapes of the vertebrae, and sometimes mild scolios

How is Spondylocamptodactyly syndrome inherited?

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

At what age does Spondylocamptodactyly syndrome typically begin?

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