• Tethered Cord Syndrome

    (Tethered Spinal Cord Syndrome; TCS)

    Definition

    Tethered cord syndrome (TCS) is a group of neurological symptoms caused by an abnormal attachment of the spine. The spinal cord is a bundle of nerves that normally sit freely in the bony spinal column. This allows the spine to move freely without straining the soft tissue of the spinal cord.
    In TCS, the abnormal attachment of the spinal cord to tissue around the spine causes strain on the spinal cord during normal movements. The strain and stretching on the spinal cord can damage the nerves and cause a variety of symptoms.
    TCS is usually diagnosed in childhood, but it may not be found until problems occur in adulthood. Earlier treatment of TCS can help reduce the chance of permanent damage.

    Causes

    Congenital TCS is present at birth. It is caused by abnormal development of the spine during pregnancy. It is often associated with other birth defects, such as spina bifida.
    Acquired TCS develops after birth. Some causes include:
      Growths that press into the spinal cord and restrict movement such as:
      • A benign fatty growth—lipoma
      • Tumors or cysts
    • Excessively tight or thick filum terminale—a band of tissue that connects the spine to the backbone
    • Scar tissue from surgery on the lower back or spinal cord
    Certain spinal injuries, infection, or trauma can also cause damage to spine or spinal cord that restricts spinal cord movement.

    Risk Factors

    Factors that may increase the chance of TCS include:
    • Birth defects, such as spina bifida
    • Structural defects in the lower back or spinal cord
    • Injury or trauma
    • Previous infection
    • Previous surgery
    • History of tumors or cysts

    Symptoms

    TCS symptoms vary with age, but most often appear in young children. Some may never have symptoms, while others may not have symptoms until adulthood.
    Common symptoms in toddlers, adolescents, and adults may include:
    • Difficulty walking or running
    • Foot deformities
    • Abnormal curvature of the spine—scoliosis
    • Colored marks or blemishes on the skin over the lower back
    • Numbness or muscle weakness
    • Lower back, leg, or groin pain
    • Loss of bladder or bowel control, which may include bedwetting
    Other symptoms in infants include:
    • Dribbling during urination
    • Abnormal development of the legs and feet
    Certain skin changes over the lower back in infants may suggest problems with spinal development. The development of the following may occur with spinal development problems:
    • Moles
    • A lesion
    • Hair growth
    • Deep dimple
    • Mass or growth under the skin
    • Areas where skin color is absent
    • Colored marks or blemishes
    Adults may have back pain and other symptoms common to lower back disorders, such as spinal stenosis or a herniated disc.
    Spinal Stenosis
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    Diagnosis

    You will be asked about your symptoms and medical history. A physical exam will be done. If structural problems of the spine are suspected imaging tests will be done. TCS is diagnosed with an MRI scan.
    Other imaging tests may be:
    • Ultrasound—used in infants
    • Myelogram—will show pressure on nerves
    • CT scan—for further tissue assessment
    TCS may affect the nerve going to the bladder. Urodynamic testing may be done to assess problems with the bladder.

    Treatment

    Treatment will be based on a number of factors including, symptoms, age, and overall health.
    If there are no symptoms, watchful waiting may be advised. The doctor will monitor the condition for any changes, or appearance or worsening of symptoms.
    Surgery is the only treatment to release the spinal cord. If surgery isn’t possible or needs to be delayed, medications may help manage symptoms. Medication options to help manage pain include:
    • Over-the-counter nonsteroidal anti-inflammatory drugs (NSAIDs)
    • Prescription opioids
    • Muscle relaxants
    • Antidepressants
    Surgery—The goal of surgery is to release the tethered spinal cord, ease symptoms, and prevent further damage. The exact type and extent of surgery will depend on what is causing the TCS and how much the nearby tissue is affected. Surgical steps may include:
    • Removal or release of the tether
    • Debulking of problematic tissue
    • Repair or release of nearby structures
    More than one surgery may be needed. The spinal cord may also reattach and require a repeat surgery.
    Rehabilitation—TCS can cause nerve damage that cannot be reversed. Some symptoms can persist after surgery. Rehabilitation can help develop a plan to manage any remaining challenges. It may include:
    • Physical therapy to regain as much movement as possible and keep muscles strong
    • Occupational therapy to assist in everyday tasks and self-care
    • Psychological therapy for coping, support, and to decrease the risk of depression

    Prevention

    Folic acid supplements and proper prenatal care may reduce the risk of spinal defects during pregnancy.

    RESOURCES

    American Association of Neurological Surgeons http://www.aans.org

    Healthy Children—American Academy of Pediatrics https://www.healthychildren.org

    CANADIAN RESOURCES

    Canadian Neurological Sciences Federation http://www.cnsfederation.org

    Caring for Kids—Canadian Paediatric Society http://www.caringforkids.cps.ca

    References

    Bui CJ, Tubbs RS, et al. Tethered cord syndrome in children: A review. Neurosurg Focus. 2007;23(2):E2. Available at: http://thejns.org/doi/pdfplus/10.3171/FOC-07/08/E2. Accessed June 2, 2016.

    Tethered cord syndrome. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed. Updated December 12, 2014. Accessed June 2, 2016.

    Tethered spinal cord causes and treatments. Spinal Stenosis website. Available at: http://spinalstenosis.org/blog/tethered-spinal-cord-treatments. Published September 16, 2013. Accessed June 2, 2016.

    Tethered spinal cord syndrome. American Association of Neurological Surgeons website. Available at: http://www.aans.org/Patient%20Information/Conditions%20and%20Treatments/Tethered%20Spinal%20Cord%20Syndrome.aspx. Updated July 2014. Accessed June 2, 2016.

    Treatments for tethered spinal cord in children. Boston Children’s Hospital website. Available at: http://www.childrenshospital.org/conditions-and-treatments/conditions/t/tethered-spinal-cord/treatments. Accessed June 2, 2016.

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