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Natural History of Occult Spinal Dysraphism

  • Jeffrey P. BlountEmail author
  • Zachary G. Wright
Chapter

Abstract

The natural history of the occult dysraphic lesions has never been comprehensively studied, and collective understanding remains incomplete. The unifying event that is widely considered central to all OSDs is tethered cord syndrome (TCS). The evidence that this phenomenon directs the natural history of the OSDs is empirical and originates from early observations that young patients harboring OSDs are less impaired than older ones. As longer follow-up and more comprehensive observation have occurred, it has become evident that there is at least some variability in the natural history of some occult dysraphic lesions. DSTs and SCMs appear to have high rates of progressive decline from TSC, but there is greater variability in the lipomas. Up to 50% of conus lipomas may demonstrate minimal progression of signs and symptoms, and filar lipomas are common incidental findings that are likely of variable significance. Patient registries and studies of better design will be required to advance understanding of the natural history of the occult spinal dysraphisms.

Keywords

Occult dysraphism Natural history Dermal sinus tract Split cord malformation Conus lipoma Filum lipoma 

Abbreviations

DST

Dermal sinus tract

OSD

Occult spinal dysraphism

SCM

Split cord malformation

TCS

Tethered cord syndrome

References

  1. 1.
    Ackerman LL, Menezes AH. Spinal congenital dermal sinuses: a 30 year experience. Pediatrics. 2003;112:641–7.PubMedGoogle Scholar
  2. 2.
    Goldberg C, Fenelon G, Blake NS, Dowling F, Regan BF. Diastematomyelia: a critical review of the natural history and treatment. Spine. 1984;9:367–72.PubMedGoogle Scholar
  3. 3.
    Humphreys RP, Hendrick EB, Hoffman HJ. Diastematomyelia. Clin Neurosurg. 1983;30:436–56.PubMedGoogle Scholar
  4. 4.
    LaMarca F, Grant J, Tomita T, McLone DG. Spinal lipomas in children: outcome of 270 procedures. Pediatr Neurosurg. 1997;26(1):8–16.Google Scholar
  5. 5.
    Guthkelch AN. Diastematomyelia with median septum. Brain. 1974;97:729–42.PubMedGoogle Scholar
  6. 6.
    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.PubMedGoogle Scholar
  7. 7.
    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.PubMedPubMedCentralGoogle Scholar
  8. 8.
    Proctor MR, Scott RM. Long term outcome for patients with split cord malformation. Neurosurg Focus. 2001;10(1):1–5.Google Scholar
  9. 9.
    Radmarresh F, Nejat F, El Khashab M. Dermal sinus tract of the spine. Childs Nerv Syst. 2010;26:349–57.Google Scholar
  10. 10.
    English WJ, Maltby GL. Diastematomyelia in adults. J Neurosurg. 1967;27:260–4.PubMedGoogle Scholar
  11. 11.
    Garceau GJ. The filum terminale syndrome (the cord-traction syndrome). J Bone Joint Surg Am. 1953;35-A(3):711–6.PubMedGoogle Scholar
  12. 12.
    Kanev P, Bierbrauer K. Reflections on the natural history of lipomyelomeningocele. Pediatr Neurosurg. 1995;22:137–40.PubMedGoogle Scholar
  13. 13.
    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.PubMedGoogle Scholar
  14. 14.
    Veenboer PW, Bosch JL, van Asbeck FW, de Kort LM. Paucity of evidence for urinary tract outcomes in spinal dysraphism: a systemic review. BJU Int. 2013;112(7):1009–17.PubMedGoogle Scholar
  15. 15.
    Jindal A, Mahapatra AK. Split cord malformations- a clinical study of 48 cases. Indian Pediatr. 2000;37:603–7.PubMedGoogle Scholar
  16. 16.
    Morris KM, Findlay GF. Dysraphic problems in the lumbar spine: dilemmas in management – a review of 49 cases. Eur Spine J. 1992;1(2):105–8.PubMedGoogle Scholar
  17. 17.
    Tu A, Hengel AR, Cochrane DD. The natural history and management of patients with congenital defects associated with lumbosacral lipomas. Childs Nerv Syst. 2016;32(4):667–73.PubMedGoogle Scholar
  18. 18.
    Tseng JH, Kuo MF, Kwang TY, Tseng MY. Outcome of untethering for symptomatic spina bifida occulta with lumbosacral spinal cord tethering in 31 patients: analysis of pre-operative prognostic factors. Spine J. 2008;8(4):630–8.PubMedGoogle Scholar
  19. 19.
    Wykes V, Desai D, Thompson DN. Asymptomatic lumbosacral lipomas- a natural history study. Childs Nerv Syst. 2012;28(10):1731–9.PubMedGoogle Scholar
  20. 20.
    Cools MJ, Al-Holou WN, Stetler WJ, Wilson TJ, Maraszko, et al. Filum terminale lipomas: imaging prevalence, natural history and conus position. J Neurosurg Pediatr. 2014;13:559–67.PubMedGoogle Scholar
  21. 21.
    Rapal S, Salamat MS, Tubbs RS, Kelly DR, Oakes WJ, Iskander BJ. Tethering tracts in spina bifida occulta: revisiting an established nomenclature. J Neurosurg Spine. 2007;7:315–22.Google Scholar
  22. 22.
    Jones PH, Love JG. Tight filum terminale. AMA Arch Surg. 1956;73:556–66.PubMedGoogle Scholar
  23. 23.
    Cochrane DD. The patterns of late deterioration in patients with transitional lipomyelomeningocele. Eur J Pediatr Surg. 2000;10(Suppl 1):13–7.PubMedGoogle Scholar
  24. 24.
    O’Neill P, Singh J. Occult spinal dysraphism in children: need for early neurosurgical referral. Childs Nerv Syst. 1991;7(6):309–11.PubMedGoogle Scholar
  25. 25.
    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.PubMedGoogle Scholar
  26. 26.
    Valentini LG, Selvaggio G, Erbetta A, Cordella A, Pecoraro MG, et al. Occult spinal dysraphism: lessons learned by retrospective analysis of 149 surgical cases about natural history, surgical indications, urodynamic testing and intraoperative neurophysiologic monitoring. Childs Nerv Syst. 2013;29(9):1657–9.PubMedGoogle Scholar
  27. 27.
    Andar UB, Harkness WF, Hayward RD. Split cord malformations of the lumbar region. A model for the neurosurgical management of all types of ‘occult’ spinal dysraphism. Pediatr Neurosurg. 1997;26(1):17–24.PubMedGoogle Scholar
  28. 28.
    Wright RO. Congenital dermal sinuses. Progr Neurol Surg. 1971;4:175–91.Google Scholar
  29. 29.
    Iqbal N, Qadeer M, Sharif SY. Variation in outcome in tethered cord syndrome. Asian Spine J. 2016;10(4):711–8.PubMedPubMedCentralGoogle Scholar
  30. 30.
    Tu A, Hengel AR, Cochrane DD. Radiographic predictors of deterioration in patients with lumbosacral lipomas. J Neurosurg. 2016;18(2):171–6.Google Scholar
  31. 31.
    Averbeck MA, Madersbacher H. Follow up of the neuro-urological patient: a systematic review. BJU Int. 2015;115(Suppl 6):39–46.PubMedGoogle Scholar
  32. 32.
    Ersahain Y, Mutluer S, Kocaman S. Split cord malformations in children. J Neurosurg. 1998;88:57–65.Google Scholar
  33. 33.
    Cornette L, Verpoorten C, Lagae L, Van Calenbergh F, Plets C, Vereecken R, Casaer P. Tethered cord syndrome in occult spinal dysraphism: timing and outcome of surgical release. Neurology. 1998;50(6):1761–5.PubMedGoogle Scholar
  34. 34.
    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.PubMedGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Pediatric NeurosurgeryChildren’s of AlabamaBirminghamUSA

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