What is the diagnosis and treatment for Tethered Cord Syndrome (TCS)?

From the Guidelines

Tethered cord syndrome should be treated with surgical detethering as soon as progressive neurological symptoms are identified, as this can prevent further neurological deterioration and improve quality of life. The condition is characterized by the abnormal attachment of the spinal cord to surrounding tissues, which can lead to lower extremity neurologic and orthopedic abnormalities, scoliosis, and other manifestations of tethering 1. Patients with spinal dysraphism are at high risk of developing tethered cord syndrome, with up to 75% presenting with lower extremity neurologic and orthopedic abnormalities from tethering.

Key Considerations

• The exact presentation of tethered cord syndrome depends on the age of the patient and the degree and type of lower extremity dysfunction.
• Progression of symptoms is an important feature that suggests an underlying dysraphic malformation with spinal cord tethering.
• While tethered cord release can arrest or improve neurologic symptoms, sensorimotor dysfunction, or urologic deterioration, long-standing or severe orthopedic deformities are unlikely to improve and may require subsequent orthopedic intervention.

Treatment Approach

• Surgical detethering is the primary treatment for tethered cord syndrome, involving a laminectomy and careful dissection to release the tethered elements while preserving nerve function.
• Post-surgery, patients require close monitoring for potential complications like CSF leakage, infection, or retethering.
• Physical therapy is often recommended during recovery to improve strength and mobility.

Importance of Early Intervention

• Early intervention is crucial as nerve damage from prolonged tethering may be irreversible.
• Regular follow-up with neurosurgery is essential to monitor for symptom recurrence, as retethering can occur in approximately 10-20% of cases.
• The condition can be congenital, associated with spina bifida or other spinal malformations, or acquired from spinal trauma, inflammation, or previous spinal surgeries.

From the Research

Definition and Characteristics of Tethered Cord Syndrome

• Tethered cord syndrome (TCS) is a diverse clinical entity characterized by symptoms and signs caused by excessive tension on the spinal cord 2.
• The majority of cases are related to spinal dysraphism, and TCS can present in any age group, with varying presentations according to the underlying pathologic condition and age 2.
• Common symptoms include pain, cutaneous signs, orthopedic deformities, and neurological deficits 2, 3.

Surgical Management and Outcomes

• Surgical untethering is indicated in patients with progressive or new onset symptomatology attributable to TCS, aiming to release the tethering structure and thus the chronic tension on the cord 2, 3.
• Early operative intervention is associated with improved outcomes, with pain relief accomplished in almost all cases, and realistic surgical goals including relief of pain and stabilization of neurological function 2, 4.
• Improvement in function is often seen, and cord untethering can also halt the progression of scoliosis 2, 4.
• The benefits of surgery are debated in asymptomatic patients and patients with normal imaging 2.

Adult Onset Tethered Cord Syndrome

• Adult onset tethered cord syndrome (ATCS) seems not as rare as once thought, but low susceptibility in adulthood commonly leads to a delay in diagnosis and therapy 3.
• Pain is the predominant symptom in adults, and eliciting mechanisms like trauma, excessive physical training, or degenerative spinal canal stenosis are reported more often 3.
• Surgical untethering aims to restore craniocaudal mobility of the spinal cord, prevent further progression of symptoms, restore neurological function, and improve pain 3, 4.
• Factors associated with postoperative deterioration and/or bad outcome include split cord malformation, lipomyelomeningocele, previous surgery, rapid motor function worsening, and long delay in diagnosis 3.

Symptom Improvement and Prognosis

• Pain is the most responsive symptom after surgical untethering, with improvement in 81% of patients 5.
• Sensory and motor symptoms also benefit from surgery, especially if less than 6 months standing and mild, with improvement in 43% and 58.6% of patients, respectively 3.
• Sphincter troubles are less responsive, with improvement in 45.6% of patients 3.
• Patients who experience improvement in any symptom typically do so by 6 months after tethered cord release, with the rate of symptomatic improvement greatest for pain resolution, followed by motor, and then urinary improvement 6.