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Outcomes in Occult Spinal Dysraphism

  • Jeffrey P. BlountEmail author
Chapter

Abstract

Occult dysraphic lesions vary significantly. Some give rise to focal tether, and others can induce harm via more than one pathophysiological mechanism. Dermal sinus tracts threaten both by focal tether and as potential portals for infection. The tethering tract is distinct from the neural elements, so risks of neurological injury from surgical untethering are very low though the natural history is threatening. Similarly, split cord malformations (types I and II) are well-established sources of focal tether that demonstrate adverse natural histories when untreated. They can be complex and often accompany open forms of dysraphism but almost uniformly respond favorably to surgical untethering.

Lipomas are the most controversial of the dysraphic lesions. Filum lipomas are common and are often asymptomatic and of dubious pathological significance. There is virtually no controversy regarding surgical clipping of a fatty filum when the filum is thick and fat infiltrated, the conus is low, and there are symptoms consistent with tether. Surgical clipping of filum lipomas has few risks and low morbidity. However, the operative indications for filum clipping for cases that are asymptomatic or where the conus is in the normal position remain controversial. The evidence supporting these procedures is of modest power and quality.

Lipomas of the conus medullaris are the most difficult lesions to manage. Many but not all patients harboring these challenging lesions show progression of neurological decline over time. Many series attest to stabilization or improvement after operative intervention. However, tethering symptoms recur at a high rate (at least 50%) when follow-up is extended. Tether is one of several pathophysiological mechanisms in conus lipomas. There is general consensus that symptomatic patients should be offered surgery, but the role for prophylactic surgery is more variable, complex, and controversial. There are three conceptual approaches. The conservative approach involves reserving any operative intervention for clearly documented clinical or urodynamic decline. The optimum safe resection approach is widely embraced and strives to untether and reduce the volume of fat to the maximum safe extent to enable placode closure but not gross total removal of all fat. The radical approach embraces complete removal of the fatty mass, re-establishment of external pial surfaces, and optimization of the sac-cord ratio to prevent re-tether.

Keywords

Outcomes Surgical outcomes Natural history Lipoma Dermal sinus tract Split cord malformation 

References

  1. 1.
    O’Neill P, Singh J. Occult spinal dysraphism in children: need for early neurosurgical referral. Childs Nerv Syst. 1991;7(6):309–11.PubMedCrossRefGoogle Scholar
  2. 2.
    Cornette L, Verpoorten C, Lagae L, Plets C, VanCalenbergh F, Casaer P. Closed spinal dysraphism: a review on diagnosis and treatment in infancy. Eur J Paediatr Neurosurg. 1998;2(4):179–85.CrossRefGoogle Scholar
  3. 3.
    Koyanagi I, Iwasaki Y, Hida K, Abe H, Isu T, Akino M. Surgical treatment supposed natural history of the tethered cord with occult spinal dysraphism. Childs Nerv Syst. 1997;13(5):268–74.PubMedCrossRefGoogle Scholar
  4. 4.
    Valentini LG, Selvaggio G, Erbetta A, Cordella R, Pecoraro MG, Bova S, Boni E, Beretta E, Furlanetto M. Occult spinal dysraphism: lessons learned by retrospective analysis of 149 surgical cases about natural history, surgical indications, urodynamic testing and intraoperative physiological monitoring. Childs Nerv Syst. 2013;29(9):1657–69.PubMedCrossRefGoogle Scholar
  5. 5.
    Lapsiwala SB, Iskander BJ. The tethered cord syndrome in adults with spina bifida occulta. Neurol Res. 2004;26(7):735–40.PubMedCrossRefGoogle Scholar
  6. 6.
    Cochrane DD. Cord untethering for lipomyelomeningocele: expectation after surgery. Neurosurg Focus. 2007;23:E9.PubMedCrossRefGoogle Scholar
  7. 7.
    Kulkarni AV, Pierre-Kahn A, Zerah M. Conservative management of asymptomatic spinal lipomas of the conus. Neurosurgery. 2004;54:868–75.PubMedCrossRefGoogle Scholar
  8. 8.
    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;176:1729–32.PubMedCrossRefGoogle Scholar
  9. 9.
    Lee GY, Paradiso G, Tator CH, Gentile 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;4:123–31.PubMedCrossRefGoogle Scholar
  10. 10.
    Iqbal N, Qadeer M, Sharif SY. Variation in outcome in tethered cord syndrome. Asian Spine J. 2016;10(4):711–8.PubMedPubMedCentralCrossRefGoogle Scholar
  11. 11.
    Geyik M, Alptekin M, Erkutlu I, Erbas C, Pusat S, Kural C. Tethered cord syndrome in children: a single center experience with 162 children. Childs Nerv Syst. 2015;31(9):1559–63.PubMedCrossRefGoogle Scholar
  12. 12.
    Huttman S, Krauss J, Collmann H, Sorensen N, Roosen K. Surgical management of tethered spinal cord in adults: report of 54 cases. J Neurosurg Spine. 2001;4:173–8.CrossRefGoogle Scholar
  13. 13.
    Blount JP, Elton S. Spinal lipomas. Neurosurg Focus. 2001;10(1):E3.PubMedCrossRefGoogle Scholar
  14. 14.
    Cochrane DD, Finley C, Kestle J, Steinbok P. The patterns of late deterioration in patients with transitional lipomyelomeningocele. Eur J Pediatr Surg. 2000;10(Suppl 1):13–7.PubMedCrossRefGoogle Scholar
  15. 15.
    Tuite GF, Thompson DNP, Austin PF, Bauer SB. Evaluation and management of tethered cord syndrome in occult spinal dysraphism: recommendations from the children’s continence society. Neurourol Urodyn. 2018;37(3):890–903.PubMedCrossRefGoogle Scholar
  16. 16.
    Pierre-Kahn A, Zerah M, Renier D, Cinalli G, Sainte-Rose C, Lellouch-Tubiana A. et.al. Congenital lumbosacral lipomas. Childs Nerv Syst. 1997;13:298–335.PubMedCrossRefGoogle Scholar
  17. 17.
    Colak A, Pollack IF, Albright AL. Recurrent tethering: a common long-term problem after lipomyelomeningocele repair. Pediatr Neurosurg. 1998;29(4):184–90.PubMedCrossRefGoogle Scholar
  18. 18.
    VanCalenbergh F, Vanvolsem S, Verpoorten C, Lagae L, Casaer P, Plets C. Results after surgery for lumbosacral lipoma: the significance of early and late worsening. Childs Nerv Syst. 1999;15(9):439–42.CrossRefGoogle Scholar
  19. 19.
    Patil PS, Gupta A, Kothari PL, Kekre G, Gupta R, Dikshit V, et al. Immediate and long term outcome analysis of lipomyelomeningocele repair in asymptomatic infants in a tertiary care center. J Pediatr Neurosci. 2016;11(2):99–104.PubMedPubMedCentralCrossRefGoogle Scholar
  20. 20.
    La Marca F, Grant JA, Tomita T, McLone DG. Spinal lipomas in children: outcomes of 270 procedures. Pediatr Neurosurg. 1997;26(1):8–16.PubMedCrossRefGoogle Scholar
  21. 21.
    Shut L, Bruce DA, Sutton LN. The management of the child with a lipomyelomeningocele. Clin Neurosurg. 1983;30:464–76.CrossRefGoogle Scholar
  22. 22.
    Goodrich DJ, Patel D, Loukas M, Tubbs RS, Oakes WJ. Symptomatic re-tethering of the spinal cord in postoperative lipomyelomeningocele patients: a meta-analysis. Childs Nerv Syst. 2016;32(1):121–6.PubMedCrossRefGoogle Scholar
  23. 23.
    Heieh MH, Perry V, Gupta N, Pearson C, Nguyen HT. The effects of de-tethering on the urodynamics profile in children with a tethered cord. J Neurosurg. 2006;105(5 Suppl):391–5.Google Scholar
  24. 24.
    Finn MA, Walker ML. Spinal lipomas: clinical spectrum, embryology and treatment. Neurosurg Focus. 2007;23(2):E10.PubMedCrossRefGoogle Scholar
  25. 25.
    Pang D, Zovickian J, Oviedo A. Long-term outcome of total and near-total resection of spinal cord lipomas and radical reconstruction of the neural placode- part I- surgical technique. Neurosurgery. 2009;65(3):511–28.PubMedCrossRefGoogle Scholar
  26. 26.
    Pang D, Zovickian J, Wong ST, Hou YJ, Moes GS. Surgical treatment of complex spinal cord lipomas. Childs Nerv Syst. 2013;29(9):1485–513.PubMedCrossRefGoogle Scholar
  27. 27.
    Chapman PH, Stieg PE, Magge S, Barnes P, Feany M. Spinal lipoma controversy. Neurosurgery. 1999;44:186–93.PubMedCrossRefGoogle Scholar
  28. 28.
    Geyik M, Alptekin M, Erkutlu I, Geyik S, Erbas C, Pusat S, Kural C. Tethered cord syndrome in children: a single center experience with 162 patients. Childs Nerv Syst. 2015;31(9):1559–63.PubMedCrossRefGoogle Scholar
  29. 29.
    Tseng JH, Kuo MF, Kwang TU, Tseng MY. Outcome of untethering for symptomatic spina bifida occulta with lumbosacral cord tethering in 31 patients: analysis of pre-operative predictive factors. Spine J. 2008;8(4):630–8.CrossRefPubMedGoogle Scholar
  30. 30.
    Muthukumar N. Congenital spinal lipomatous malformations: part I-classification. Acta Neurochir. 2009;151(3):179–88.PubMedCrossRefGoogle Scholar
  31. 31.
    Muthukumar N. Congenital spinal lipomatous malformations: part II-clinical presentation, operative findings and outcome. Acta Neurochir. 2009;151(3):189–97.PubMedCrossRefGoogle Scholar
  32. 32.
    Hoffman HJ, Taecholarn C, Hendrick EB, Humphreys RP. Management of lipomyelomeningocele- experience at the Hospital for Sick Children Toronto. J Neurosurg. 1985;62:1–8.PubMedCrossRefGoogle Scholar
  33. 33.
    Kanev P, Bierbrauer K. Reflections on the natural history of lipomyelomeningocele. Pediatr Neurosurg. 1995;22:137–40.PubMedCrossRefGoogle Scholar
  34. 34.
    Drake JM. Occult tethered cord syndrome: not an indication for surgery. J Neurosurg. 2006;104(5 Suppl):305–8.PubMedGoogle Scholar
  35. 35.
    Drake JM. Surgical management of the tethered spinal cord- walking the fine line. Neurosurg Focus. 2007;23(2):E10.CrossRefGoogle Scholar
  36. 36.
    Herman JM, McLone DG, Storrs BB, Dauser RC. Analysis of 153 patients with myelomeningocele or spinal lipoma reoperated upon for a tethered cord. Presentation, management and outcome. Pediatr Neurosurg. 1993;19:243–9.PubMedCrossRefGoogle Scholar
  37. 37.
    Hendrick EB, Hoffman HJ, Humphreys RP. The tethered spinal cord. Clin Neurosurg. 1983;30:457–63.PubMedCrossRefGoogle Scholar
  38. 38.
    Hoffman HJ, Hendrick EB, Humphreys RP. The tethered spinal cord: its protean manifestations, diagnosis and surgical correction. Childs Brain. 1976;2(3):145–55.PubMedGoogle Scholar
  39. 39.
    Bui CJ, Tubbs RS, Oakes WJ. Tethered cord syndrome in children: a review. Neurosurg Focus. 2007;23(2):E2.PubMedCrossRefGoogle Scholar
  40. 40.
    Anderson FM. Occult spinal dysraphism: a series of 73 cases. Pediatrics. 1975;55:826–35.PubMedGoogle Scholar
  41. 41.
    Pang D, Wilberger JE. Tethered cord syndrome in adults. J Neurosurg. 1982;57:32–47.PubMedCrossRefGoogle Scholar
  42. 42.
    Johnson DL, Levy LM. Predicting outcome in the tethered cord syndrome: a study of cord motion. Pediatr Neurosurg. 1995;22:115–9.PubMedCrossRefGoogle Scholar
  43. 43.
    Sathi S, Madsden JR, Bauer S, Scott RM. Effect of surgical repair on the neurologic function in infants with lipomyelomeningocele. Pediatr Neurosurg. 1993;19:256–9.PubMedCrossRefGoogle Scholar
  44. 44.
    Geyik M, Geyik S, Sen H, Pusat S, Alptekin M, Yilmaz AE, et al. Urodynamic outcomes of de-tethering in children: experience with 46 patients. Childs Nerv Syst. 2016;32(6):1079–84.PubMedCrossRefGoogle Scholar
  45. 45.
    Yerkes EB, Halline C, Yoshiba G, Meyer TA, Rosoklija I, Bowman R, et al. Lipomyelomeningoele for the urologist: should we view it the same as myelomeningocele? J Pediatr Urol. 2017;13(4):371.PubMedCrossRefGoogle Scholar
  46. 46.
    Boemers TM, vanGool JD, deJong TP. Tethered spinal cord: the effect of neurosurgery on the lower urinary tract and male sexual function. Br J Urol. 1995;76(6):747–51.PubMedCrossRefGoogle Scholar
  47. 47.
    Klekamp J. Tethered cord syndrome in adults; clinical article. J Neurosurg Spine. 2011;15:258–70.PubMedCrossRefGoogle Scholar
  48. 48.
    Dias MS, Pang D. Split cord malformations. Neurosurg Clin N Am. 1995;6:339–58.PubMedCrossRefGoogle Scholar
  49. 49.
    Goldberg C, Fenelon G, Bllake NS, Dowling F, Regan BF. Diastmatomyelia: a critical review of the natural history and treatment. Spine. 1984;9:367–72.PubMedCrossRefGoogle Scholar
  50. 50.
    Humphreys RP, Hendrick EB, Hoffman HJ. Diastematomyelia. Clin Neurosurg. 1983;30:436–56.PubMedCrossRefGoogle Scholar
  51. 51.
    Talamonti G, D’Alberti G, Nichelatti M, Debernardi A, Picano M, REdaelli T. Asymptomatic lipomas of the medullary conus: surgical treatment versus conservative management. J Neurosurg Pediatr. 2014;14:245–54.PubMedCrossRefGoogle Scholar
  52. 52.
    daRosa SP, Scavarda D, Choux M. Results of the prophylactic surgery of lumbosacral lipomas- 20 years of experience in the Paediatric Neurosurgery Department La Timone Enfants Hospital, Marseille, France. Childs Nerv Syst. 2016;32(11):2205–9.CrossRefGoogle Scholar
  53. 53.
    Thompson EM, Strong MJ, Warren G, Woltjer RL, Selden NR. Clinical significance of imaging and histological characteristics of filum terminale in tethered cord syndrome, clinical article. J Neurosurg Pediatr. 2014;13:255–9.PubMedCrossRefGoogle Scholar
  54. 54.
    Peter JC. Occult dysraphism of the spine. A retrospective analysis of 88 operative cases 1979–1989. S Afr Med J. 1992;81:351–4.PubMedGoogle Scholar
  55. 55.
    Ostling LR, Bierbrauer KS, Kuntz CIV. Outcome, re-operation and complications in 99 consecutive children operated for tight or fatty filum. World Neurosurg. 2012;77:187–91.PubMedCrossRefGoogle Scholar
  56. 56.
    Andar UB, Harkness WFJ, Hayward RD. Split cord malformations of the lumbar region. Pediatr Neurosurg. 1997;26:17–24.PubMedCrossRefGoogle Scholar
  57. 57.
    Mahapatra AK, Gupta NK. Split cord malformations – a clinical study of 254 cases and a proposal of a new clinical-imaging classification. J Neurosurg. 2005;103(6 Suppl Pediatrics):531–6.PubMedGoogle Scholar
  58. 58.
    Nazar GB, Casale AJ, Roberts JG, Linden RD. Occult filum terminale syndrome. Pediatr Neurosurg. 1995;23(5):228–35.PubMedCrossRefGoogle Scholar
  59. 59.
    Arai H, Sato K, Okuda O, Miyajima M, Hishii M, Nakanishi H, et al. Surgical experience of 120 patients with lumbosacral lipomas. Acta Neurochir. 2001;143:857–64.PubMedCrossRefGoogle Scholar
  60. 60.
    Khoury AE, Hendrick EB, McLorie GA, Kulkarni A, Churchill BM. Occult spinal dysraphism: clinical and urodynamic outcome after division of the filum terminale. J Urol. 1990;144(2 Pt2):426–8.PubMedCrossRefGoogle Scholar
  61. 61.
    Usami K, Lallemant P, Roujeau T, James S, Beccaria K, Levy R, DiRocco F, Sainte-Rose C, Zerah M. Spinal lipoma of the filum terminale: review of 174 consecutive patients. Childs Nerv Syst. 2016;32(7):1265–72.PubMedCrossRefGoogle Scholar
  62. 62.
    Metcalfe PD, Luerseen TG, King SJ, Kaefer M, Meldrum KK, Cain MP. Treatment of the occult tethered spinal cord for neuropathic bladder: results of sectioning the filum terminale. J Urol. 2006;176(4):1826–30.PubMedCrossRefGoogle Scholar
  63. 63.
    Proctor MR, Bauer SB, Scott RM. The effect of surgery for split spinal cord malformation on neurologic and urologic function. Pediatr Neurosurg. 2000;32:13–9.PubMedCrossRefGoogle Scholar
  64. 64.
    Moise TS. Staphylococcus meningitis secondary to congenital sacral sinus. Surg Gynecol Obstet. 1926;42:394–7.Google Scholar
  65. 65.
    Wright RO. Congenital dermal sinuses. Prog Neurol Surg. 1971;4:175–91.CrossRefGoogle Scholar
  66. 66.
    Matson DD, Jerva MS. Recurrent meningitis associated with congenital lumbo-sacral dermal sinus tract. J Neurosurg. 1966;25(3):288–97.PubMedCrossRefGoogle Scholar
  67. 67.
    Radmanesh R, Nejat F, El Khashab M. Dermal sinus tract of the spine. Childs Nerv Syst. 2010;26(3):349–57.PubMedCrossRefGoogle Scholar
  68. 68.
    DeVloo P, Lagae L, Sciot R, Demaerel P, van Loon J, van Calenbergh F. Spinal dermal sinuses and sinus-like stalks- analysis of 14 cases with suggestions for embryonic mechanism resulting in dermal sinus like stalks. Eur J Paediatr Neurol. 2013;17(6):575–84.CrossRefGoogle Scholar
  69. 69.
    Pang D, Dias M, Ahab-Barmada M. Split cord malformation: part I: a unified theory of embryognesis for double spinal cord malformations. Neurosurgery. 1992;31:451–80.PubMedCrossRefGoogle Scholar
  70. 70.
    Mount LA. Congenital dermal sinuses as a cause of meningitis, intraspinal abscess and intracranial abscess. JAMA. 1949;139:1263–8.CrossRefGoogle Scholar
  71. 71.
    Ackerman LL, Menezes AH. Spinal congenital dermal sinuses: a 30 year experience. Pediatrics. 2004;112(3):641–7.CrossRefGoogle Scholar
  72. 72.
    Pang D. Split cord malformation: part II: clinical syndrome. Neurosurgery. 1992;31:451–80.PubMedCrossRefGoogle Scholar
  73. 73.
    Ersahain Y, Mutluer S, Kocamar S, Demitras E. Split spinal cord malformations in children. J Neurosurg. 1998;88:57–65.CrossRefGoogle Scholar
  74. 74.
    Jindal A, Mahapatra AK. Split cord malformations- a clinical study of 48 cases. Indian Pediatr. 2000;37:603–7.PubMedGoogle Scholar
  75. 75.
    Borkar SA, Mahapatra AK. Split cord malformations: a two years experience at AIIMS. Asian J Neurosurg. 2012;7(2):56–60.PubMedPubMedCentralCrossRefGoogle Scholar
  76. 76.
    Gower DJ, DelCurling O, Kelly DL Jr, Alexander E. Diastematomyelia-a 40-year experience. Pediatr Neurosci. 1988;14(2):90–6.PubMedCrossRefGoogle Scholar
  77. 77.
    Chapman PH. Congenital intraspinal lipomas: anatomic considerations and surgical treatment. Childs Brain. 1982;9:37–42.PubMedGoogle Scholar
  78. 78.
    Pang D. Occult filum terminale. Pediatr Neurosurg. 1995;23(5):227.PubMedCrossRefGoogle Scholar
  79. 79.
    Jones PH, Love JG. Tight filum terminale. AMA Arch Surg. 1956;73:556–66.PubMedCrossRefGoogle Scholar
  80. 80.
    Brown E, Matthes JC, Bazan C, Jinkins JR. Prevalence of incidental intraspinal lipoma of the lumbosacral spine as determined by MRI. Spine. 1994;19:833–6.PubMedCrossRefGoogle Scholar
  81. 81.
    Garceau GJ. The filum terminale syndrome (the cord-traction syndrome). J Bone Join Surg Am. 1953;35-A(3):711–6.CrossRefGoogle Scholar
  82. 82.
    Steinbok P, Garton HJ, Gupta N. Occult tethered cord syndrome: a survey of practice patterns. J Neurosurg. 2006;104(5 Suppl):309–13.PubMedGoogle Scholar
  83. 83.
    Steinbok P, MacNeily AE. Section of the terminal filum for occult tethered cord syndrome: toward a scientific answer. Neurosurg Focus. 2007;23(2):E5.PubMedCrossRefGoogle Scholar
  84. 84.
    Selcuk M, Unlu A, Ugur HC, Soygur T, Arikan N, Selcuki D. Patients with urinary incontinence often benefit from surgical detethering of tight filum terminale. Childs Nerv Syst. 2000;16(3):150–4.CrossRefGoogle Scholar
  85. 85.
    Cornips EM, Vereijken IM, Beuls EA, Weber JW, Soudant DL, van Rhijn LW, et al. Clinical characteristics and surgical outcome in 25 cases of childhood tight filum syndrome. Eur J Paediatr Neurol. 2012;16:103–17.PubMedCrossRefGoogle Scholar
  86. 86.
    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;61:550–6.PubMedCrossRefGoogle Scholar
  87. 87.
    Warder DE, Oakes WJ. Tethered cord syndrome and the conus in a normal position. Neurosurgery. 1993;33:374–8.PubMedGoogle Scholar
  88. 88.
    Kim AH, Kasliwal MK, McNeish B, Silvera VM, Proctor MR, Smith ER. Features of the lumbar spine on magnetic resonance images following sectioning of filum terminale, clinical article. J Neurosurg Pediatr. 2011;8:384–9.PubMedCrossRefGoogle Scholar
  89. 89.
    Warder DE, Oakes WJ. Tethered cord syndrome: the low-lying and normally positioned conus. Neurosurgery. 1994;34:597–600.PubMedGoogle Scholar
  90. 90.
    Wehby MC, O’Halleren PS, Abtin K, Hume JL, Richards BJ. Occult tight filum syndrome: results of surgical untethering. Pediatr Neurosurg. 2004;40(2):51–7.PubMedCrossRefGoogle Scholar
  91. 91.
    Selcuki M, Coskun K. Managemtn of tight filum terminale syndrome with special emphasis on normal position conus medullaris (NLCM). Surg Neurol. 1998;50(4):318–32.PubMedCrossRefGoogle Scholar
  92. 92.
    Bruce DA, Schut L. Spinal lipomas in infancy and childhood. Childs Brain. 1979;5:192–203.PubMedGoogle Scholar

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Authors and Affiliations

  1. 1.Pediatric Neurosurgery, Children’s of AlabamaBirminghamUSA

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