Tethered cord syndrome
Updates to Article Attributes
Tethered spinal cord syndrome, also known as an occult spinal dysraphism sequence, is a neurological disorder caused by tissue attachments that limit the movement of the spinal cord within the spinal columncanal.
Clinical presentation
Tethered cord syndrome is a clinical diagnosis based on neurologic deterioration 7. Patients may present with any combination of the following 4:
- progressive leg weakness or sensory loss
- disturbance of bowel or bladder function
- low back pain or sciatica
- spinal deformities such as scoliosis
- foot deformities such as pes cavus
- cutaneous stigmata of spinal dysraphism
(hairy(e.g. hairy patch, dimple, subcutaneous lipoma)
The condition typically presents in children but may gooccasionally goes undiagnosed until or is acquired in adulthood.
Pathology
Spinal cord tethering should be thought of as primary and secondary:
primary tethered cord syndrome occurs as an isolated anomaly-
secondary tethered cord syndrome occurs in the setting of other abnormalities, typicallyspinal dysraphismor prior surgery/trauma3:
The spinal column develops at a greater rate than the spinal cord during fetal development, and abnormal attachments lead to abnormal stretching of the spinal cord.
Etiology
Tethering may also developThe term "primary tethered cord syndrome" is defined in different ways in the literature, sometimes referring to tight filum terminale syndrome with or without low conus medullaris position, and at other times indicating all congenital causes such as myelomeningocele, tumors, and other spinal dysraphisms3. In contrast, secondary tethered cord syndrome is acquired, such as after perinatal repair of spinal dysraphisms like myelomeningocele 8.
Tethered cord injury and scar tissuesyndrome can block the flowresult from a variety of fluids around the spinal corddysraphisms 7.
Fluid pressure may cause cysts to form a syringomyelia.
Radiographic features
Imaging features areMost commonly, the conus medullaris terminates in a low position below the level of the L2 vertebral body 1. However, tethered cord syndrome is possible in the setting of a lownormal conus medullaris (below L2) and thickenedposition 5,7.
Additional imaging features depend on the etiology, which are described separately:
Commonly associated
Ultrasound
Ultrasound may be a useful screening exam and may be of use in the paediatric population. The lack of ossification of the posterior arch of the spine in normal infants and the presence of a bony defect in patients with spina bifida permit sonographic examination.
MRI
MRI is useful in visualizing the conus medullaris, assessing the thickness of the filum terminale, identifying traction lesions, and evaluating associated bony dysraphisms.
In one study of 25 patients in 84% the tip of the conus was below the level of the mid-L2 vertebral body 1.
Prone imaging may be useful in patients who have undergone tethered cord surgery or in those in whom clinical suspicion is high while supine MRI imaging demonstrated no abnormalities6. Prone MRI imaging is however of little value when supine MRI has demonstrated the defecta causative abnormality.
Treatment and prognosis
Untreated, tethered cord syndrome has a progressive course. Surgical release, in selected patients, can dramaticallystabilize or improve function 3.
-<p><strong>Tethered spinal cord syndrome</strong>, also known as an <strong>occult spinal dysraphism sequence</strong>, is a neurological disorder caused by tissue attachments that limit the movement of the spinal cord within the spinal column.</p><h4>Clinical presentation</h4><p>Patients present with any combination of the following <sup>4</sup>:</p><ul>- +<p><strong>Tethered cord syndrome</strong>, also known as an <strong>occult spinal dysraphism sequence</strong>, is a neurological disorder caused by tissue attachments that limit the movement of the <a href="/articles/spinal-cord">spinal cord</a> within the <a href="/articles/spinal-canal">spinal canal</a>.</p><h4>Clinical presentation</h4><p>Tethered cord syndrome is a clinical diagnosis based on neurologic deterioration <sup>7</sup>. Patients may present with any combination of the following <sup>4</sup>:</p><ul>
-<li>cutaneous stigmata of <a href="/articles/spinal-dysraphism-2">spinal dysraphism</a> (hairy patch, dimple, subcutaneous lipoma)</li>-</ul><p>The condition typically presents in children but may go undiagnosed until adulthood.</p><h4>Pathology</h4><p>Spinal cord tethering should be thought of as primary and secondary:</p><ul>-<li>primary tethered cord syndrome occurs as an isolated anomaly</li>-<li>secondary tethered cord syndrome occurs in the setting of other abnormalities, typically <a href="/articles/spinal-dysraphism-2">spinal dysraphism</a> or prior surgery/trauma <sup>3</sup>:<ul>-<li><a href="/articles/lipomyelomeningocele-1">lipomyelomeningocele</a></li>-<li><a href="/articles/lipoma-of-the-filum-terminale">filum terminale lipoma</a></li>-<li>-<a href="/articles/myelomeningocele-1">myelomeningocele</a> repair</li>-<li><a href="/articles/diastematomyelia">diastematomyelia</a></li>-</ul>-</li>-</ul><p>The spinal column develops at a greater rate than the spinal cord during fetal development, and abnormal attachments lead to abnormal stretching of the spinal cord.</p><p>Tethering may also develop after spinal cord injury and scar tissue can block the flow of fluids around the spinal cord.</p><p>Fluid pressure may cause cysts to form a <a href="/articles/syringomyelia">syringomyelia</a>. </p><h4>Radiographic features</h4><p>Imaging features are of a low conus medullaris (below L2) and thickened filum terminale (>2 mm).</p><h5>Ultrasound </h5><p>Ultrasound may be a useful screening exam and may be of use in the paediatric population. The lack of ossification of the posterior arch of the spine in normal infants and the presence of a bony defect in patients with spina bifida permit sonographic examination.</p><h5>MRI</h5><p>MRI is useful in visualizing the conus medullaris, assessing the thickness of the filum terminale, identifying traction lesions, and evaluating associated bony dysraphisms.</p><p>In one study of 25 patients in 84% the tip of the conus was below the level of the mid-L2 vertebral body <sup>1</sup>.</p><p>Prone imaging may be useful in patients who have undergone tethered cord surgery or in those in whom clinical suspicion is high while supine MRI imaging demonstrated no abnormalities. Prone MRI imaging is however of little value when supine MRI has demonstrated the defect.</p><h4>Treatment and prognosis</h4><p>Untreated, tethered cord syndrome has a progressive course. Surgical release, in selected patients, can dramatically improve function <sup>3</sup>. </p>- +<li>cutaneous stigmata of <a href="/articles/spinal-dysraphism-2">spinal dysraphism</a> (e.g. hairy patch, dimple, subcutaneous lipoma)</li>
- +</ul><p>The condition typically presents in children but occasionally goes undiagnosed until or is acquired in adulthood.</p><h4>Pathology</h4><p>The spinal column develops at a greater rate than the spinal cord during fetal development, and abnormal attachments lead to abnormal stretching of the spinal cord.</p><h5>Etiology</h5><p>The term "primary tethered cord syndrome" is defined in different ways in the literature, sometimes referring to <a href="/articles/tight-filum-terminale-syndrome">tight filum terminale syndrome</a> with or without low <a href="/articles/conus-medullaris">conus medullaris</a> position, and at other times indicating all congenital causes such as <a href="/articles/myelomeningocele-1">myelomeningocele</a>, tumors, and other <a href="/articles/spinal-dysraphism-2">spinal dysraphisms</a> <sup>3</sup>. In contrast, secondary tethered cord syndrome is acquired, such as after perinatal repair of spinal dysraphisms like myelomeningocele <sup>8</sup>.</p><p>Tethered cord syndrome can result from a variety of spinal dysraphisms <sup>7</sup>.</p><h4>Radiographic features</h4><p>Most commonly, the conus medullaris terminates in a low position below the level of the L2 vertebral body <sup>1</sup>. However, tethered cord syndrome is possible in the setting of a normal conus position <sup>5,7</sup>.</p><p>Additional imaging features depend on the etiology, which are described separately:</p><ul><li><a href="/articles/tight-filum-terminale-syndrome">tight filum terminale</a></li></ul><p>Commonly associated </p><h5>Ultrasound </h5><p>Ultrasound may be a useful screening exam and may be of use in the paediatric population. The lack of ossification of the posterior arch of the spine in normal infants and the presence of a bony defect in patients with <a href="/articles/spina-bifida">spina bifida</a> permit sonographic examination.</p><h5>MRI</h5><p>MRI is useful in visualizing the conus medullaris, assessing the thickness of the filum terminale, identifying traction lesions, and evaluating associated bony dysraphisms.</p><p>Prone imaging may be useful in patients who have undergone tethered cord surgery or in those in whom clinical suspicion is high while supine MRI imaging demonstrated no abnormalities <sup>6</sup>. Prone MRI imaging is however of little value when supine MRI has demonstrated a causative abnormality.</p><h4>Treatment and prognosis</h4><p>Untreated, tethered cord syndrome has a progressive course. Surgical release, in selected patients, can stabilize or improve function <sup>3</sup>. </p>
References changed:
- 5. Warder D & Oakes W. Tethered Cord Syndrome. Neurosurgery. 1994;34(4):597-600. <a href="https://doi.org/10.1227/00006123-199404000-00005">doi:10.1227/00006123-199404000-00005</a>
- 6. Stamates M, Frim D, Yang C, Katzman G, Ali S. Magnetic Resonance Imaging in the Prone Position and the Diagnosis of Tethered Spinal Cord. J Neurosurg Pediatr. 2018;21(1):4-10. <a href="https://doi.org/10.3171/2017.3.peds16596">doi:10.3171/2017.3.peds16596</a>
- 7. Warder D. Tethered Cord Syndrome and Occult Spinal Dysraphism. FOC. 2001;10(1):1-9. <a href="https://doi.org/10.3171/foc.2001.10.1.2">doi:10.3171/foc.2001.10.1.2</a>
- 8. Ohe N, Futamura A, Kawada R et al. Secondary Tethered Cord Syndrome in Spinal Dysraphism. Child's Nerv Syst. 2000;16(7):457-61. <a href="https://doi.org/10.1007/pl00007291">doi:10.1007/pl00007291</a>
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