OBSOLETE: Cervical spina bifida aperta

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ORPHA:268392
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Overview

Cervical spina bifida aperta is a rare birth defect that occurs when the bones of the spine in the neck (cervical) region do not close properly during early pregnancy. The term 'aperta' means 'open,' indicating that the spinal cord and its surrounding membranes are exposed through a gap in the vertebrae. This condition is a type of neural tube defect, which happens when the neural tube — the structure that eventually becomes the brain and spinal cord — fails to close completely during the first few weeks of development. Note: This condition has been classified as 'OBSOLETE' in the Orphanet database, meaning it may have been reclassified or merged into a broader category of neural tube defects or spina bifida. Cervical spina bifida aperta is much rarer than spina bifida affecting the lower back (lumbar region). Because the defect occurs in the neck area, it can cause serious neurological problems including weakness or paralysis in the arms and legs, breathing difficulties, and problems with bladder and bowel control. Treatment typically involves surgical closure of the defect shortly after birth to protect the exposed spinal cord and reduce the risk of infection. Long-term management may include physical therapy, occupational therapy, and monitoring for complications such as hydrocephalus (fluid buildup in the brain). The severity of symptoms depends on the size and exact location of the opening and how much the spinal cord is affected. Prevention with folic acid supplementation before and during early pregnancy has been shown to reduce the risk of neural tube defects in general.

Key symptoms:

Visible opening or sac on the back of the neck at birthWeakness or paralysis in the arms and legsLoss of sensation below the level of the defectDifficulty breathingProblems with bladder controlProblems with bowel controlHydrocephalus (fluid buildup in the brain)Difficulty swallowing or feedingAbnormal head positioningMuscle stiffness or spasticityDevelopmental delaysRecurrent urinary tract infectionsSpinal cord tethering

Inheritance

Multifactorial

Caused by a mix of several genes and environmental factors

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for OBSOLETE: Cervical spina bifida aperta.

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No actively recruiting trials found for OBSOLETE: Cervical spina bifida aperta at this time.

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No specialists are currently listed for OBSOLETE: Cervical spina bifida aperta.

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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 OBSOLETE: Cervical spina bifida aperta.

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Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.How severe is my child's spinal defect, and what functions may be affected?,Will my child need surgery, and what are the risks and expected outcomes?,Is there a risk of hydrocephalus, and how will it be monitored?,What kind of rehabilitation and therapy will my child need?,How will bladder and bowel function be managed?,What is the chance of this happening again in a future pregnancy, and can folic acid reduce the risk?,Are there specialized spina bifida clinics or support programs available near us?

Common questions about OBSOLETE: Cervical spina bifida aperta

What is OBSOLETE: Cervical spina bifida aperta?

Cervical spina bifida aperta is a rare birth defect that occurs when the bones of the spine in the neck (cervical) region do not close properly during early pregnancy. The term 'aperta' means 'open,' indicating that the spinal cord and its surrounding membranes are exposed through a gap in the vertebrae. This condition is a type of neural tube defect, which happens when the neural tube — the structure that eventually becomes the brain and spinal cord — fails to close completely during the first few weeks of development. Note: This condition has been classified as 'OBSOLETE' in the Orphanet da

How is OBSOLETE: Cervical spina bifida aperta inherited?

OBSOLETE: Cervical spina bifida aperta follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does OBSOLETE: Cervical spina bifida aperta typically begin?

Typical onset of OBSOLETE: Cervical spina bifida aperta is neonatal. Age of onset can vary across affected individuals.