Tethered Cord Syndrome

1. Tethered Cord Syndrome
AdeWijaya, MD

2. Introduction
• A constellation of symptoms and signs of motor and sensory
neuron dysfunction attributable to abnormally increased
tension on the spinal cord.
• a spectrum of congenital anomalies resulting in an abnormally
low position of the conus medullaris that may lead to
neurological, musculoskeletal, urological, or gastrointestinal
abnormalities
• Low-lying conus medullaris
• 1910 – 1st case
Lew SM, Kothbauer KF. Tethered cord syndrome: an updated review. Pediatric neurosurgery. 2007;43(3):236-48.
Fuchs A: Ueber Beziehungen der Enuresis nocturna zu Rudimentärformen der Spina bifida occulta (Myelodysplasie). Wien Med Wochenschr 1910; 80: 1569–1573
Hertzler DA, DePowell JJ, Stevenson CB, Mangano FT. Tethered cord syndrome: a review of the literature from embryology to adult presentation. Neurosurgical focus. 2010 Jul 1;29(1):E1.

3. Embryology

4. Pathophysiology
• Traction increases  blood flow and oxidative metabolism
become impaired
• The degree and reversibility of cord dysfunction correlates
with both the magnitude and duration of the traction
• Chronic tension can serve to preload the cord allowing minor
additional stretching to cause severe and permanent damage
• The spinal dysraphysm
Lew SM, Kothbauer KF. Tethered cord syndrome: an updated review. Pediatric neurosurgery. 2007;43(3):236-48.

5. Etiology
• The spinal dysraphysm
Lew SM, Kothbauer KF. Tethered cord syndrome: an updated review. Pediatric neurosurgery. 2007;43(3):236-48.

6. Clinical Presentation
• The clinical presentation ofTCS varies by age group and etiology
• In neonates and infants, cutaneous manifestations of spina bifida
occulta take on particular importance as they may be the only
evidence of a tethering lesion. Cutaneous stigmata include: nevi,
lipomas, tufts of hair, hemangiomas, and dermal sinuses.
• Urinary dribbling as a sign of urodynamic disturbance
• An orthopedic lower extremity deformity or scoliosis
• Anorectal malformation
Muthukumar N, Subramaniam B, Gnanaseelan T, Rathinam R, Thiruthavadoss A: Tethered cord syndrome in children with anorectal malformations. J Neurosurg 2000; 92: 626–630.
Morimoto K, Takemoto O, Wakayama A: Tethered cord associated with anorectal malformation. Pediatr Neurosurg 2003; 38: 79–82.
Levitt MA, Patel M, Rodriguez G, Gaylin DS, Pena A: The tethered spinal cord in patients with anorectal malformations. J Pediatr Surg 1997; 32: 462–468.

7. Clinical Presentation
Toddlers and adolescents
• Difficulties with gait or running may be due to progressive motor dysfunction
• Sensory deficits (nonsegmental distribution)
• Progression of scoliosis
• Development of orthopedic foot deformities
• There may be a regression in bladder or less frequently bowel control
• Pain will be a complaint, either in the lower extremities or back.
• Rarely results in trophic scarring or ulcerations
Lew SM, Kothbauer KF. Tethered cord syndrome: an updated review. Pediatric neurosurgery. 2007;43(3):236-48.

8. Clinical Presentation
Teenage years / adults
• Scoliosis, pain, and sphincter dysfunction predominate
• Pain tends to be localized to the low back, perineum, and/or
legs and is often nondermatomal.
Lew SM, Kothbauer KF. Tethered cord syndrome: an updated review. Pediatric neurosurgery. 2007;43(3):236-48.

9. Clinical Presentation
Physical examination
• Cutaneous stigmata
• Scoliosis
• Leg length discrepancies
• Muscular asymmetry
• Motor and sensory function as well as gait
• Spasticity, clonus, babinski
Lew SM, Kothbauer KF. Tethered cord syndrome: an updated review. Pediatric neurosurgery. 2007;43(3):236-48.

10. Lew SM, Kothbauer KF. Tethered cord syndrome: an updated review. Pediatric neurosurgery. 2007;43(3):236-48.

11. Urodynamics
• The most common urodynamic finding is detrusor
hyperreflexia
• Other common findings include: diminished bladder
compliance, external detrusor-sphincter dyssynergia,
decreased sensation, and hypocontractile detrusor function
Fone PD, Vapnek JM, Litwiller SE, Couillard DR, McDonald CM, Boggan JE, Stone AR: Urodynamic findings in the tethered spinal cord syndrome: does surgical release improve bladder function? J Urol 1997; 157: 604–609.
Giddens JL, Radomski SB, Hirshberg ED, Hassouna M, Fehlings M: Urodynamic findings in adults with the tethered cord syndrome. J Urol 1999; 161: 1249–1254.

12. Lew SM, Kothbauer KF. Tethered cord syndrome: an updated review. Pediatric neurosurgery. 2007;43(3):236-48.

13. Natural History
• Not all patients with anatomic cord tethering develop
symptoms
• Those that become symptomatic have a significant likelihood
of worsening (and little or no likelihood of improving) in the
absence of surgical untethering.
Lew SM, Kothbauer KF. Tethered cord syndrome: an updated review. Pediatric neurosurgery. 2007;43(3):236-48.

14. Management
• Conservative
• Surgical
Surgical outcomes:
- Pain relief
- Neurological function
- Scoliosis
- Spasticity
- Retethering
Lew SM, Kothbauer KF. Tethered cord syndrome: an updated review. Pediatric neurosurgery. 2007;43(3):236-48.

15. Tethered Cervical Spinal Cord
• Less common
• Associated with: lipomas, myelomeningoceles, dermal sinus
tracts, and split cord malformations
• Upper extremity signs and symptoms
Lew SM, Kothbauer KF. Tethered cord syndrome: an updated review. Pediatric neurosurgery. 2007;43(3):236-48.

16. OccultTCS
• Patients exist with symptomatic cord tethering with a conus
medullaris that is considered to be at a normal level
• ‘occult filum terminale syndrome’
Lew SM, Kothbauer KF. Tethered cord syndrome: an updated review. Pediatric neurosurgery. 2007;43(3):236-48.

17. Summary
• Diverse clinical entity which presents with symptoms and
signs resulting from abnormal spinal cord tension
• Spinal dysraphysm
• Tethered cord syndrome can present in any age group, and
presentation differs according to underlying pathologic
condition and age
• Surgical untethering is indicated in patients with progressive
or new-onset symptomatology attributable toTCS