Tethered Cord Syndrome
Tethered cord syndrome is a condition in which the lower end of the spinal cord is anchored by abnormal tissue to the lower spinal canal and remains under tension. Tension increases with growth and can cause progressive neurological damage. Early diagnosis and surgical release (detethering) aim to halt progression.
Ultimo aggiornamento: 2026-06-07
Definition
In tethered cord syndrome the lower end of the spinal cord (conus medullaris), which normally ends freely around L1-L2, is fixed lower down by abnormal tissue and held under tension. As the spine grows during childhood while the cord remains anchored, the tension increases; this mechanical stress and impaired microcirculation can lead to neurological injury over time.
Causes
The main tethering structures are a thick, shortened filum terminale, a lipoma (lipomyelomeningocele), scar tissue after previous surgery or spina bifida repair, dermoid/epidermoid cysts and diastematomyelia. The condition is therefore classified as congenital (accompanying developmental anomalies) or acquired (scarring after surgery or trauma). Congenital cases often present in childhood, while acquired cases can present at any age.
Symptoms
Symptoms are insidious and progressive. Back and leg pain (especially with activity), progressive leg weakness and muscle wasting, gait disturbance, foot deformities (pes cavus, foot drop) and sensory loss may occur. Bladder dysfunction (incontinence, urinary frequency, recurrent infections) and bowel problems are common. Skin findings over the lower back—hair tufts, hemangioma, lipoma or a sacral dimple—may point to an underlying anomaly. Symptoms can worsen during periods of rapid growth.
Diagnosis
Diagnosis rests on clinical suspicion and imaging. Spinal magnetic resonance imaging (MRI) is the reference method, showing a low-lying conus medullaris, a thickened filum terminale and associated anomalies (lipoma, dermoid cyst, diastematomyelia). Neurophysiological tests (somatosensory and motor evoked potentials, EMG) assess spinal cord and nerve root function. Urodynamic studies evaluate the bladder. In infants with skin findings, screening spinal MRI is advised.
Treatment
Treatment is surgical release (detethering). Under general anesthesia, a laminectomy opens the spinal canal and, beneath the dura, the tethering structure (thick filum terminale, lipoma, fibrous bands or scar) is divided or removed using microsurgical technique. Intraoperative neurophysiological monitoring helps protect the cord and nerve roots. The goal is to preserve existing function and halt progression; pain and bladder symptoms often improve, while established motor deficits usually recover only partially. In asymptomatic, incidentally detected low-conus cases, observation may be preferred.
Course and Re-tethering
Surgery performed early, while symptoms are still mild, tends to give better results. In some patients, scar tissue or adhesions can cause re-tethering after surgery, and recurrence of symptoms may require revision surgery. For this reason, long-term follow-up with regular neurological examination and MRI when needed is important. Outcomes vary between patients and none can be guaranteed.
Riferimenti
- Greenberg MS. Greenberg's Handbook of Neurosurgery. 10th ed. Thieme; 2023:290-291.
- Yamada S, Won DJ, Yamada SM. Pathophysiology of tethered cord syndrome: correlation with symptomatology. Neurosurg Focus. 2004;16(2):E6.
- Hertzler DA, DePowell JJ, Stevenson CB, Mangano FT. Tethered cord syndrome: a review of the literature from embryology to adult presentation. Neurosurg Focus. 2010;29(1):E1.
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