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The Tethered Cord Syndrome and Its Occult Form

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Book cover Occult Spinal Dysraphism

Abstract

Congenital spinal defects may cause caudal stretching of the spinal cord and associated symptoms and signs known as the tethered cord syndrome (TCS). Natural history studies and experimental evidence that have emerged over nearly five decades have contributed to establishing TCS as a valid diagnostic entity. More recently, the existence of an occult form of TCS (OTCS) has been proposed, distinguished by neuro-urologic symptoms suggestive of distal spinal cord dysfunction, but in the absence of both external stigmata of spinal dysraphism and anatomic abnormalities on MR imaging. In the present chapter, we explore the historic and contemporary evidence surrounding the diagnosis and management of OTCS against a backdrop of comparable criteria for the standard of care of TCS. Through these discussions, we highlight important questions that have been raised regarding the level of evidence available to support these diagnoses, in light of contemporary evidence-based medicine standards.

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References

  1. Johnston A. Fatty tumor from the sacrum of a child connected with the spinal membranes. Trans Pathol Soc Lond. 1857;8:16–8.

    Google Scholar 

  2. Jones W. Spina bifida occulta: no paralytic symptoms until seventeen years of age: spine trephined to relive pressure on the cauda equina: recovery. Brit Med J. 1891;1:173–4.

    Google Scholar 

  3. Fuchs A. Ueber Beziehungen der Enuresis nocturna zu Rudimentärformen der Spina bifida occulta (Myelodysplasie). Wien Med Wochenschr. 1910;80:1569–73.

    Google Scholar 

  4. Lichtenstein BW. Spinal dysraphism: spina bifida and myelodysplasia. Arch Neurol Psychiatry. 1940;44(4):792–809.

    Google Scholar 

  5. Barry A, Patten BM, Stewart BH. Possible factors in the development of the Arnold-Chiari malformation. J Neurosurg. 1957;14(3):285–301.

    CAS  PubMed  Google Scholar 

  6. Bassett RC. The neurologic deficit associated with lipomas of the cauda equina. Ann Surg. 1950;131(1):109–16.

    CAS  PubMed  PubMed Central  Google Scholar 

  7. Rogers HM, Long DM, Chou SN, French LA. Lipomas of the spinal cord and cauda equina. J Neurosurg. 1971;34(3):349–54.

    CAS  PubMed  Google Scholar 

  8. Garceau GJ. The filum terminale syndrome (the cord-traction syndrome). J Bone Joint Surg Am. 1953;35-A(3):711–6.

    CAS  PubMed  Google Scholar 

  9. Jones PH, Love JG. Tight filum terminale. AMA Arch Surg. 1956;73(4):556–66.

    CAS  PubMed  Google Scholar 

  10. James CCM, Lassman LP. Spina bifida occulta. In:Orthopedic, radiological and neurosurgical aspects. London: Academic Press Inc; 1981.

    Google Scholar 

  11. Fitz CR, Harwood Nash DC. The tethered conus. Am J Roentgenol Rad Ther Nuc Med. 1975;125(3):515–23.

    CAS  Google Scholar 

  12. Barson AJ. The vertebral level of termination of the spinal cord during normal and abnormal development. J Anat. 1970;106(Pt 3):489–97.

    CAS  PubMed  PubMed Central  Google Scholar 

  13. Hoffman HJ, Hendrick EB, Humphreys RP. The tethered spinal cord: its protean manifestations, diagnosis and surgical correction. Childs Brain. 1976;2(3):145–55.

    CAS  PubMed  Google Scholar 

  14. Raghavan N, Barkovich AJ, Edwards M, Norman D. MR imaging in the tethered spinal cord syndrome. AJR Am J Roentgenol. 1989;152(4):843–52.

    CAS  PubMed  Google Scholar 

  15. Naidich TP, Harwood-Nash DC, McLone DG. Radiology of spinal dysraphism. Clin Neurosurg. 1983;30:341–65.

    CAS  PubMed  Google Scholar 

  16. Hall WA, Albright AL, Brunberg JA. Diagnosis of tethered cords by magnetic resonance imaging. Surg Neurol. 1988;30(1):60–4.

    CAS  PubMed  Google Scholar 

  17. Yamada S, Zinke DE, Sanders D. Pathophysiology of “tethered cord syndrome”. J Neurosurg. 1981;54(4):494–503.

    CAS  PubMed  Google Scholar 

  18. Tani S, Yamada S, Knighton RS. Extensibility of the lumbar and sacral cord. Pathophysiology of the tethered spinal cord in cats. J Neurosurg. 1987;66(1):116–23.

    CAS  PubMed  Google Scholar 

  19. Yamada S, Lonser RR. Adult tethered cord syndrome. J Spinal Disord. 2000;13(4):319–23.

    CAS  PubMed  Google Scholar 

  20. Lew SM, Kothbauer KF. Tethered cord syndrome: an updated review. Pediatr Neurosurg. 2007;43(3):236–48.

    PubMed  Google Scholar 

  21. Kocak A, Kilic A, Nurlu G, et al. A new model for tethered cord syndrome: a biochemical, electrophysiological, and electron microscopic study. Pediatr Neurosurg. 1997;26(3):120–6.

    CAS  PubMed  Google Scholar 

  22. Pang D, Wilberger JE Jr. Tethered cord syndrome in adults. J Neurosurg. 1982;57(1):32–47.

    CAS  PubMed  Google Scholar 

  23. Huttmann S, Krauss J, Collmann H, Sorensen N, Roosen K. Surgical management of tethered spinal cord in adults: report of 54 cases. J Neurosurg. 2001;95(2 Suppl):173–8.

    CAS  PubMed  Google Scholar 

  24. Iskandar BJ, Fulmer BB, Hadley MN, Oakes WJ. Congenital tethered spinal cord syndrome in adults. J Neurosurg. 1998;88(6):958–61.

    CAS  PubMed  Google Scholar 

  25. Lee GY, Paradiso G, Tator CH, Gentili F, Massicotte EM, Fehlings MG. Surgical management of tethered cord syndrome in adults: indications, techniques, and long-term outcomes in 60 patients. J Neurosurg Spine. 2006;4(2):123–31.

    CAS  PubMed  Google Scholar 

  26. Gupta SK, Khosla VK, Sharma BS, Mathuriya SN, Pathak A, Tewari MK. Tethered cord syndrome in adults. Surg Neurol. 1999;52(4):362–9; discussion 370

    CAS  PubMed  Google Scholar 

  27. Guerra LA, Pike J, Milks J, Barrowman N, Leonard M. Outcome in patients who underwent tethered cord release for occult spinal dysraphism. J Urol. 2006;176(4 Pt 2):1729–32.

    PubMed  Google Scholar 

  28. Selden NR. Occult tethered cord syndrome: the case for surgery. J Neurosurg. 2006;104(5 Suppl):302–4.

    PubMed  Google Scholar 

  29. Fone PD, Vapnek JM, Litwiller SE, et al. Urodynamic findings in the tethered spinal cord syndrome: does surgical release improve bladder function? J Urol. 1997;157(2):604–9.

    CAS  PubMed  Google Scholar 

  30. Giddens JL, Radomski SB, Hirshberg ED, Hassouna M, Fehlings M. Urodynamic findings in adults with the tethered cord syndrome. J Urol. 1999;161(4):1249–54.

    CAS  PubMed  Google Scholar 

  31. Kondo A, Gotoh M, Kato K, Saito M, Sasakibara T, Yamada H. Treatment of persistent enuresis. Results of severing a tight filum terminale. Brit J Urol. 1988;62(1):42–5.

    CAS  PubMed  Google Scholar 

  32. Yamada S, Won DJ. What is the true tethered cord syndrome? Childs Nerv Syst. 2007;23(4):371–5.

    PubMed  Google Scholar 

  33. Yamada S, Won DJ, Pezeshkpour G, et al. Pathophysiology of tethered cord syndrome and similar complex disorders. Neurosurg Focus. 2007;23(2):E6.

    PubMed  Google Scholar 

  34. Hendrick EB, Hoffman HJ, Humphreys RP. The tethered spinal cord. Clin Neurosurg. 1983;30:457–63.

    CAS  PubMed  Google Scholar 

  35. Gregerson DM. Clinical consequences of spina bifida occulta. J Manip Physiol Ther. 1997;20(8):546–50.

    CAS  Google Scholar 

  36. Warder DE, Oakes WJ. Tethered cord syndrome: the low-lying and normally positioned conus. Neurosurgery. 1994;34(4):597–600; discussion 600

    CAS  PubMed  Google Scholar 

  37. Warder DE, Oakes WJ. Tethered cord syndrome and the conus in a normal position. Neurosurgery. 1993;33(3):374–8.

    CAS  PubMed  Google Scholar 

  38. McLendon RE, Oakes WJ, Heinz ER, Yeates AE, Burger PC. Adipose tissue in the filum terminale: a computed tomographic finding that may indicate tethering of the spinal cord. Neurosurgery. 1988;22(5):873–6.

    CAS  PubMed  Google Scholar 

  39. Nazar GB, Casale AJ, Roberts JG, Linden RD. Occult filum terminale syndrome. Pediatr Neurosurg. 1995;23(5):228–35.

    CAS  PubMed  Google Scholar 

  40. Balague F, Dudler J, Nordin M. Low-back pain in children. Lancet. 2003;361(9367):1403–4.

    PubMed  Google Scholar 

  41. Altaf F, Heran MK, Wilson LF. Back pain in children and adolescents. Bone Joint J. 2014;96-B(6):717–23.

    CAS  PubMed  Google Scholar 

  42. Watson KD, Papageorgiou AC, Jones GT, et al. Low back pain in schoolchildren: occurrence and characteristics. Pain. 2002;97(1–2):87–92.

    PubMed  Google Scholar 

  43. Snodgrass W. Relationship of voiding dysfunction to urinary tract infection and vesicoureteral reflux in children. Urology. 1991;38(4):341–4.

    CAS  PubMed  Google Scholar 

  44. Sureshkumar P, Jones M, Cumming R, Craig J. A population based study of 2,856 school-age children with urinary incontinence. J Urol. 2009;181(2):808–15; discussion 815–806

    PubMed  Google Scholar 

  45. Sureshkumar P, Craig JC, Roy LP, Knight JF. Daytime urinary incontinence in primary school children: a population-based survey. J Pediatr. 2000;137(6):814–8.

    CAS  PubMed  Google Scholar 

  46. Saedi NA, Schulman SL. Natural history of voiding dysfunction. Pediatr Nephrol. 2003;18(9):894–7.

    PubMed  Google Scholar 

  47. Steinbok P, MacNeily AE, Hengel AR, et al. Filum section for urinary incontinence in children with occult tethered cord syndrome: a randomized, controlled pilot study. J Urol. 2016;195(4 Pt 2):1183–8.

    PubMed  Google Scholar 

  48. McLone DG, La Marca F. The tethered spinal cord: diagnosis, significance, and management. Semin Pediatr Neurol. 1997;4(3):192–208.

    CAS  PubMed  Google Scholar 

  49. Warder DE. Tethered cord syndrome and occult spinal dysraphism. Neurosurg Focus. 2001;10(1):e1.

    CAS  PubMed  Google Scholar 

  50. Steinbok P, Kariyattil R, MacNeily AE. Comparison of section of filum terminale and non-neurosurgical management for urinary incontinence in patients with normal conus position and possible occult tethered cord syndrome. Neurosurgery. 2007;61(3):550–5; discussion 555–556

    PubMed  Google Scholar 

  51. Steinbok P, Garton HJ, Gupta N. Occult tethered cord syndrome: a survey of practice patterns. J Neurosurg. 2006;104(5 Suppl):309–13.

    PubMed  Google Scholar 

  52. McCullough DC, Levy LM, DiChiro G, Johnson DL. Toward the prediction of neurological injury from tethered spinal cord: investigation of cord motion with magnetic resonance. Pediatr Neurosurg. 1990;16(1):3–7.

    PubMed  Google Scholar 

  53. Levy LM, Di Chiro G, McCullough DC, Dwyer AJ, Johnson DL, Yang SS. Fixed spinal cord: diagnosis with MR imaging. Radiology. 1988;169(3):773–8.

    CAS  PubMed  Google Scholar 

  54. Yamada S, Won DJ. Occult tethered cord. J Neurosurg. 2007;106(5 Suppl):411–3; author reply 413–414

    PubMed  Google Scholar 

  55. Biesheuvel C, Barratt A, Howard K, Houssami N, Irwig L. Effects of study methods and biases on estimates of invasive breast cancer overdetection with mammography screening: a systematic review. Lancet Oncol. 2007;8(12):1129–38.

    PubMed  Google Scholar 

  56. Moynihan R, Doust J, Henry D. Preventing overdiagnosis: how to stop harming the healthy. Brit Med J. 2012;e3502:344.

    Google Scholar 

  57. Davies L, Welch HG. Increasing incidence of thyroid cancer in the United States, 1973–2002. JAMA. 2006;295(18):2164–7.

    CAS  PubMed  Google Scholar 

  58. Guthkelch AN. Diastematomyelia with median septum. Brain. 1974;97(4):729–42.

    CAS  PubMed  Google Scholar 

  59. Hoffman HJ, Taecholarn C, Hendrick EB, Humphreys RP. Management of lipomyelomeningoceles. Experience at the Hospital for Sick Children, Toronto. J Neurosurg. 1985;62(1):1–8.

    CAS  PubMed  Google Scholar 

  60. Lunardi P, Missori P, Gagliardi FM, Fortuna A. Long-term results of the surgical treatment of spinal dermoid and epidermoid tumors. Neurosurgery. 1989;25(6):860–4.

    CAS  PubMed  Google Scholar 

  61. Anderson FM. Occult spinal dysraphism: a series of 73 cases. Pediatrics. 1975;55(6):826–35.

    CAS  PubMed  Google Scholar 

  62. Kanev PM, Lemire RJ, Loeser JD, Berger MS. Management and long-term follow-up review of children with lipomyelomeningocele, 1952–1987. J Neurosurg. 1990;73(1):48–52.

    CAS  PubMed  Google Scholar 

  63. Keating MA, Rink RC, Bauer SB, et al. Neurourological implications of the changing approach in management of occult spinal lesions. J Urol. 1988;140(5 Pt 2):1299–301.

    CAS  PubMed  Google Scholar 

  64. Dias MS, Pang D. Split cord malformations. Neurosurg Clin North Am. 1995;6(2):339–58.

    CAS  Google Scholar 

  65. Tuuha SE, Aziz D, Drake J, Wales P, Kim PC. Is surgery necessary for asymptomatic tethered cord in anorectal malformation patients? J Pediatr Surg. 2004;39(5):773–7.

    PubMed  Google Scholar 

  66. Kulkarni AV, Pierre-Kahn A, Zerah M. Conservative management of asymptomatic spinal lipomas of the conus. Neurosurgery. 2004;54(4):868–73; discussion 873–865

    PubMed  Google Scholar 

  67. Wykes V, Desai D, Thompson DN. Asymptomatic lumbosacral lipomas – a natural history study. Childs Nerv Syst. 2012;28(10):1731–9.

    PubMed  Google Scholar 

  68. Fournier JC, DeRubeis RJ, Hollon SD, et al. Antidepressant drug effects and depression severity: a patient-level meta-analysis. JAMA. 2010;303(1):47–53.

    CAS  PubMed  PubMed Central  Google Scholar 

  69. Cundy T. Proposed new diagnostic criteria for gestational diabetes – a pause for thought? Diabet Med. 2012;29(2):176–80.

    CAS  PubMed  Google Scholar 

  70. Prasad V, Rho J, Cifu A. The diagnosis and treatment of pulmonary embolism: a metaphor for medicine in the evidence-based medicine era. Arch Intern Med. 2012;172(12):955–8.

    PubMed  Google Scholar 

  71. Sackett DL, Rosenberg WM, Gray JA, Haynes RB, Richardson WS. Evidence based medicine: what it is and what it isn’t. BMJ. 1996;312(7023):71–2.

    CAS  PubMed  PubMed Central  Google Scholar 

  72. Every-Palmer S, Howick J. How evidence-based medicine is failing due to biased trials and selective publication. J Eval Clin Pract. 2014;20(6):908–14.

    PubMed  Google Scholar 

  73. Evidence-Based Medicine Working Group. Evidence-based medicine. A new approach to teaching the practice of medicine. JAMA. 1992;268(17):2420–5.

    Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Neurological Surgery, University of Wisconsin Hospitals and Clinics, Madison, WI, USA

    Bermans J. Iskandar

  2. Department of Neurological Surgery, University of Wisconsin School of Medicine and Public Health, Madison, WI, USA

    Steve C. Amaefuna

Authors
  1. Bermans J. Iskandar
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  2. Steve C. Amaefuna
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Correspondence to Bermans J. Iskandar .

Editor information

Editors and Affiliations

  1. Seattle Science Foundation, Seattle, WA, USA

    R. Shane Tubbs

  2. Swedish Neuroscience Institute, Seattle, WA, USA

    Rod J. Oskouian

  3. Pediatric Neurosurgery, Children’s of Alabama, Birmingham, AL, USA

    Jeffrey P. Blount

  4. Division of Neurosurgery, Children’s of Alabama, Birmingham, AL, USA

    W. Jerry Oakes

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Iskandar, B.J., Amaefuna, S.C. (2019). The Tethered Cord Syndrome and Its Occult Form. In: Tubbs, R., Oskouian, R., Blount, J., Oakes, W. (eds) Occult Spinal Dysraphism. Springer, Cham. https://doi.org/10.1007/978-3-030-10994-3_8

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  • DOI: https://doi.org/10.1007/978-3-030-10994-3_8

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-10993-6

  • Online ISBN: 978-3-030-10994-3

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