Tethered cord syndrome
Updates to Article Attributes
Tethered 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 canal.
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 (e.g. hairy patch, dimple, subcutaneous lipoma)
The condition typically presents in children but occasionally goes undiagnosed until or is acquired in adulthood.
Pathology
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
The 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 syndrome can result from a variety of spinal dysraphisms, 7 such as myelomeningocele, tumors, and other spinal dysraphisms7.
Classification
Classifications of etiologies as "primary" or "secondary" tethered cord syndrome are heterogeneous and conflicting in the literature. For instance, primary tethered cord syndrome may refer to all congenital causes in general, closed spinal dysraphisms as a group, or tight filum terminale in particular 3,8,9. Without a consensus nomenclature, this classification is best avoided.
Radiographic features
Most 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 normal conus position 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.
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 6. Prone MRI imaging is however of little value when supine MRI has demonstrated a causative abnormality.
Treatment and prognosis
Untreated, tethered cord syndrome has a progressive course. Surgical release, in selected patients, can stabilize or improve function 3.
-</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>- +</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>Tethered cord syndrome can result from a variety of spinal dysraphisms, <sup>7</sup> such as <a href="/articles/myelomeningocele-1">myelomeningocele</a>, tumors, and other <a href="/articles/spinal-dysraphism-2">spinal dysraphisms</a> <sup>7</sup>.</p><h5>Classification</h5><p>Classifications of etiologies as "primary" or "secondary" tethered cord syndrome are heterogeneous and conflicting in the literature. For instance, primary tethered cord syndrome may refer to all congenital causes in general, closed <a href="/articles/spinal-dysraphism-2">spinal dysraphisms</a> as a group, or <a href="/articles/tight-filum-terminale-syndrome">tight filum terminale</a> in particular <sup>3,8,9</sup>. Without a consensus nomenclature, this classification is best avoided.</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:
- 9. Cartwright C. Primary Tethered Cord Syndrome: Diagnosis and Treatment of an Insidious Defect. J Neurosci Nurs. 2000;32(4):210-5. <a href="https://doi.org/10.1097/01376517-200008000-00004">doi:10.1097/01376517-200008000-00004</a>
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