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Associations of the Occult Spinal Dysraphisms

  • Mohammad W. Kassem
  • R. Shane TubbsEmail author
Chapter

Abstract

Many diagnoses may also have an associated form of the occult spinal dysraphisms. Many of these are uncommonly related, but some (e.g., anorectal atresia) are frequently seen in conjunction with an underlying spinal malformation. Each of these diagnoses should be investigated fully in order to minimize long-term consequences of an untreated or unrecognized tethered spinal cord.

Keywords

Spine Congenital Tethered spinal cord Cutaneous stigmata Comorbidities 

References

  1. 1.
    Lichtenstein B. Spinal dysraphism. Arch Neurol Psychiatr. 1940;44(4):792.CrossRefGoogle Scholar
  2. 2.
    McCormick P, Kaiser M, Angevine P, Ogden A, Mandigo C, Lo L, Anderson R, Feldstein N. Tethered spinal cord – The Spine Hospital at The Neurological Institute of New York. [online] The Spine Hospital at The Neurological Institute of New York; 2017. Available at: http://columbiaspine.org/condition/tethered-spinal-cord/. Accessed 3 Jul 2017.
  3. 3.
    Fuchs A. Über Beziehungen der Enuresis nocturna zu Rudimentärformen der Spina bifida occulta (myelodysplasie). Ann Surg. 1910;131:109–16.Google Scholar
  4. 4.
    Garceau G. The filum terminale syndrome. J Bone Joint Surg. 1953;35(3):711–6.PubMedCrossRefGoogle Scholar
  5. 5.
    Hoffman H, Hendrick B, Humphreys R. The tethered spinal cord: its protean manifestations, diagnosis and surgical correction. Childs Brain. 1976;2:145–55.PubMedGoogle Scholar
  6. 6.
    Lew S, Kothbauer K. Tethered cord syndrome: an updated review. Pediatr Neurosurg. 2007;43(3):236–48.PubMedCrossRefGoogle Scholar
  7. 7.
    Yamada S, Zinke D, Sanders D. Pathophysiology of “tethered cord syndrome”. J Neurosurg. 1981;54(4):494–503.PubMedCrossRefGoogle Scholar
  8. 8.
    Koçak A, Kılıç A, Nurlu G, Konan A, Kılınç K, Çırak B, Çolak A. A new model for tethered cord syndrome: a biochemical, electrophysiological, and electron microscopic study. Pediatr Neurosurg. 2008;26(3):120–6.CrossRefGoogle Scholar
  9. 9.
    Warder D, Oakes W. Tethered cord syndrome and the conus in a normal position. Neurosurgery. 1993;33(3):374–8.PubMedGoogle Scholar
  10. 10.
    Khoshhal K, Murshid W, Elgamal E, Salih M. Tethered cord syndrome: a study of 35 patients. J Taibah Univ Med Sci. 2012;7(1):23–8.Google Scholar
  11. 11.
    Hertzler D, DePowell J, Stevenson C, Mangano F. Tethered cord syndrome: a review of the literature from embryology to adult presentation. Neurosurg Focus. 2010;29(1):E1.PubMedCrossRefGoogle Scholar
  12. 12.
    Kuo M, Tsai Y, Hsu W, Chen R, Tu Y, Wang H. Tethered spinal cord and VACTERL association. J Neurosurg Pediatr. 2007;106(3):201–4.CrossRefGoogle Scholar
  13. 13.
    Emans P, Kootstra G, Marcelis C, Beuls E, van Heurn L. The Currarino triad: the variable expression. J Pediatr Surg. 2005;40(8):1238–42.PubMedCrossRefGoogle Scholar
  14. 14.
    AbouZeid A, Mohammad S, Abolfotoh M, Radwan A, Ismail M, Hassan T. The Currarino triad: what pediatric surgeons need to know. J Pediatr Surg. 2017;52(8):1260–8.PubMedCrossRefGoogle Scholar
  15. 15.
    Sivrikoz T, Has R, Esmer A, Kalelioglu I, Yuksel A, Taskin O. Prenatal diagnosis of tethered spinal cord associated with sacrococcygeal teratoma. J Clin Ultrasound. 2016;44(8):506–9.PubMedCrossRefGoogle Scholar
  16. 16.
    Chen S, Peng S, Kuo M, Lee W, Liang J. Miller-Dieker syndrome associated with tight filum terminale. Pediatr Neurol. 2006;34(3):228–30.PubMedCrossRefGoogle Scholar
  17. 17.
    Hsieh D, Jennesson M, Thiele E, Caruso P, Masiakos P, Duhaime A. Brain and spinal manifestations of Miller-Dieker syndrome. Neurol Clin Pract. 2013;3(1):82–3.PubMedPubMedCentralCrossRefGoogle Scholar
  18. 18.
    Milhorat T, Bolognese P, Nishikawa M, Francomano C, McDonnell N, Roonprapunt C, Kula R. Association of Chiari malformation type I and tethered cord syndrome: preliminary results of sectioning filum terminale. Surg Neurol. 2009;72(1):20–35.PubMedPubMedCentralCrossRefGoogle Scholar
  19. 19.
    Glenn C, Cheema A, Safavi-Abbasi S, Gross N, Martin M, Mapstone T. Spinal cord detethering in children with tethered cord syndrome and Chiari type 1 malformations. J Clin Neurosci. 2015;22(11):1749–52.PubMedCrossRefGoogle Scholar
  20. 20.
    De Vloo P, Monea A, Sciot R, van Loon J, Van Calenbergh F. The filum terminale: a cadaver study of anatomy, histology, and elastic properties. World Neurosurg. 2016;90:565–573.e1.PubMedCrossRefGoogle Scholar
  21. 21.
    McLendon R, Oakes J, Heinz R, Yeates A, Burger P. Adipose tissue in the filum terminale: a computed tomographic finding that may indicate tethering of the spinal cord. Neurosurgery. 1988;22(5):873–6.PubMedCrossRefGoogle Scholar
  22. 22.
    Blount J, Elton S. Spinal lipomas. Neurosurg Focus. 2001;10(1):1–13.CrossRefGoogle Scholar
  23. 23.
    Finn M, Walker M. Spinal lipomas: clinical spectrum, embryology, and treatment. Neurosurg Focus. 2007;23(2):1–12.CrossRefGoogle Scholar
  24. 24.
    Jallo G, Kothbauer K, Pradilla G. Controversies in pediatric neurosurgery. New York: Thieme; 2010.CrossRefGoogle Scholar
  25. 25.
    Thompson E, Strong M, Warren G, Woltjer R, Selden N. Clinical significance of imaging and histological characteristics of filum terminale in tethered cord syndrome. J Neurosurg Pediatr. 2014;13(3):255–9.PubMedCrossRefGoogle Scholar
  26. 26.
    Barutcuoglu M, Selcuki M, Selcuki D, Umur S, Mete M, Gurgen S, Umur. Cutting filum terminale is very important in split cord malformation cases to achieve total release. Childs Nerv Syst. 2014;31(3):425–32.PubMedCrossRefGoogle Scholar
  27. 27.
    Albright A, Pollack I, Adelson P. Principles and practice of pediatric neurosurgery. 2nd ed. New York: Thieme; 2008. p. 366–93.Google Scholar
  28. 28.
    Tian A, Foster K, Jakacki R, Reyes-Múgica M, Greene S. Neurocutaneous melanosis is associated with tethered spinal cord. Childs Nerv Syst. 2014;31(1):115–21.PubMedCrossRefGoogle Scholar
  29. 29.
    Proctor M, Scott R. Long-term outcome for patients with split cord malformation. Neurosurg Focus. 2001;10(1):1–5.CrossRefGoogle Scholar
  30. 30.
    Pang D. Split cord malformation. Neurosurgery. 1992;31(3):481–500.PubMedCrossRefGoogle Scholar
  31. 31.
    Pérez L, Barnes N, MacDiarmid S, Oakes W, Webster G. Urological dysfunction in patients with diastematomyelia. J Urol. 1993;149(6):1503–5.PubMedCrossRefGoogle Scholar
  32. 32.
    Erşahin Y. Split cord malformation types I and II: a personal series of 131 patients. Childs Nerv Syst. 2013;29(9):1515–26.PubMedCrossRefGoogle Scholar
  33. 33.
    Abu-Bonsrah N, Purvis T, Rory Goodwin C, Petteys R, De la Garza-Ramos R, Sciubba D. Adult cervicothoracic lipomyelomeningocele. J Clin Neurosci. 2016;32:157–9.PubMedCrossRefGoogle Scholar
  34. 34.
    Barry A, Patten B, Stewart B. Possible factors in the development of the Arnold-Chiari malformation. J Neurosurg. 1957;14(3):285–301.PubMedCrossRefGoogle Scholar
  35. 35.
    Warder D. Tethered cord syndrome and occult spinal dysraphism. Neurosurg Focus. 2001;10(1):1–9.CrossRefGoogle Scholar
  36. 36.
    Hoffman H, Taecholarn C, Hendrick E, Humphreys R. Management of lipomyelomeningoceles. J Neurosurg. 1985;62(1):1–8.PubMedCrossRefGoogle Scholar
  37. 37.
    Koyanagi I, Iwasaki Y, Hida K, Abe H, Isu T, Akino M. Surgical treatment of syringomyelia associated with spinal dysraphism. Childs Nerv Syst. 1997;13(4):194–200.PubMedCrossRefGoogle Scholar
  38. 38.
    Kanev P, Lemire R, Loeser J, Berger M. Management and long-term follow-up review of children with lipomyelomeningocele, 1952–1987. J Neurosurg. 1990;73(1):48–52.PubMedCrossRefGoogle Scholar
  39. 39.
    Barkovich A, Raybaud C. Pediatric neuroimaging. 3rd ed. Philadelphia: Wolters Kluwer; 2000. p. 641.Google Scholar
  40. 40.
    Tubbs R, Bui C, Rice W, Loukas M, Naftel R, Holcombe M, Oakes W. Critical analysis of the Chiari malformation type I found in children with lipomyelomeningocele. J Neurosurg Pediatr. 2007;106(3):196–200.CrossRefGoogle Scholar
  41. 41.
    Agopian A, Canfield M, Olney R, Lupo P, Ramadhani T, Mitchell L, Shaw G, Moore C. Spina bifida subtypes and sub-phenotypes by maternal race/ethnicity in the National Birth Defects Prevention Study. Am J Med Gen Part A. 2011;158A(1):109–15.CrossRefGoogle Scholar
  42. 42.
    Sarris C, Tomei K, Carmel P, Gandhi C. Lipomyelomeningocele: pathology, treatment, and outcomes. Neurosurg Focus. 2012;33(4):E3.PubMedCrossRefGoogle Scholar
  43. 43.
    Yiğit H, Özdemir H, Yurduseven E. Duplication of spine with hemi-lipomyelomeningocele. Eur Spine J. 2013;22(S3):487–90.PubMedCentralCrossRefGoogle Scholar
  44. 44.
    Levitt M, Dlugy E, Kravarusic D, Ben-Meir D, Rajz G, Konen O, Freud E, Samuk I. Caudal duplication syndrome: the vital role of a multidisciplinary approach and staged correction. Eur J Pediatr Surg Rep. 2015;04(01):001–5.CrossRefGoogle Scholar
  45. 45.
    Franco A, Jo S, Mehta A, Pandya D, Yang C. A rare triad of giant occipital encephalocele with lipomyelomeningocele, tetralogy of fallot, and situs inversus. J Radiol Case Rep. 2016;10(3):36–46.PubMedPubMedCentralGoogle Scholar
  46. 46.
    Girard C, Bigorre M, Guillot B, Bessis D. PELVIS syndrome. Arch Dermatol. 2006;142(7):884–8.PubMedCrossRefGoogle Scholar
  47. 47.
    Cohen A. The mermaid malformation: cloacal exstrophy and occult spinal dysraphism. Neurosurgery. 1991;28(6):834–43.PubMedCrossRefGoogle Scholar
  48. 48.
    Piatt J. Syringomyelia complicating myelomeningocele: review of the evidence. J Neurosurg Pediatr. 2004;100(2):101–9.CrossRefGoogle Scholar
  49. 49.
    Tubbs R, Kelly D, Mroczek-Musulman E, Oakes W. Occult spinal 91dysraphism and Pacinian hamartomas. Childs Nerv Syst. 2005;21(4):343–4.PubMedCrossRefGoogle Scholar
  50. 50.
    Puusepp M. Variété rare de tératome sous-dural de al region cervicale (intestinome): Quadriplégie, extirpation, guérison complète. Rev Neurol. 1934;2:879–86.Google Scholar
  51. 51.
    Zülch K. Histological typing of tumours of the central nervous system. Geneva: World Health Organization; 1979.Google Scholar
  52. 52.
    Rauzzino M, Tubbs R, Alexander E, Grabb P, Oakes W. Spinal neurenteric cysts and their relation to more common aspects of occult spinal dysraphism. Neurosurg Focus. 2001;10(1):1–10.CrossRefGoogle Scholar
  53. 53.
    Alrabeeah A, Gillis D, Giacomantonio M, Lau H. Neurenteric cysts—a spectrum. J Pediatr Surg. 1988;23(8):752–4.PubMedCrossRefGoogle Scholar
  54. 54.
    Menezes A, Ryken T. Craniocervical intradural neurenteric cysts. Pediatr Neurosurg. 1995;22(2):88–95.PubMedCrossRefGoogle Scholar
  55. 55.
    Mann K, Khosla V, Gulati D, Malik A. Spinal neurenteric cyst. Association with vertebral anomalies, diastematomyelia, dorsal fistula, and lipoma. Surg Neurol. 1984;21(4):358–62.PubMedCrossRefGoogle Scholar
  56. 56.
    Santos de Oliveira R, Cinalli G, Roujeau T, Sainte-Rose C, Pierre-Kahn A, Zerah M. Neurenteric cysts in children: 16 consecutive cases and review of the literature. J Neurosurg Pediatr. 2005;103(6):512–23.CrossRefGoogle Scholar
  57. 57.
    Arai Y, Yamauchi Y, Tsuji T, Fukasaku S, Yokota R, Kudo T. Spinal neurenteric cyst. Spine. 1992;17(11):1421–3.PubMedCrossRefGoogle Scholar
  58. 58.
    Paleologos S, Thom M, Thomas DG. Spinal neurenteric cysts without associated malformations. Are they the same as those presenting in spinal dysraphism? Br J Neurosurg. 2000;14(3):185–94.PubMedCrossRefGoogle Scholar
  59. 59.
    Kumar R, Nayak S. Unusual neuroenteric cysts: diagnosis and management. Pediatr Neurosurg. 2002;37(6):321–30.PubMedCrossRefGoogle Scholar
  60. 60.
    Cai C, Shen C, Yang W, Zhang Q, Hu X. Intraspinal neurenteric cysts in children. Can J Neurol Sci. 2008;35(05):609–15.PubMedCrossRefGoogle Scholar
  61. 61.
    Lippman C, Arginteanu M, Purohit D, Naidich T, Camins M. Intramedullary neurenteric cysts of the spine. J Neurosurg Spine. 2001;94(2):305–9.CrossRefGoogle Scholar
  62. 62.
    Rao M, Rout D, Misra B, Radhakrishnan V. Craniospinal and spinal enterogenous cysts—report of three cases. Clin Neurol Neurosurg. 1996;98(1):32–6.PubMedCrossRefGoogle Scholar
  63. 63.
    Rotondo M, D’Avanzo R, Natale M, Pasqualetto L, Bocchetti A, Agozzino L, Scuotto A. Intramedullary neurenteric cysts of the spine. J Neurosurg Spine. 2005;2(3):372–6.PubMedCrossRefGoogle Scholar
  64. 64.
    Menezes A, Traynelis V. Spinal neurenteric cysts in the magnetic resonance imaging era. Neurosurgery. 2006;58(1):97–105.PubMedCrossRefGoogle Scholar
  65. 65.
    Preece M, Osborn A, Chin S, Smirniotopoulos J. Intracranial neurenteric cysts: imaging and pathology spectrum. Am J Neuroradiol. 2006;27(6):1211–6. Available at: https://www.ncbi.nlm.nih.gov/pubmed/16775266.PubMedGoogle Scholar
  66. 66.
    Tuzun Y, Izci Y, Sengul G, Erdogan F, Suma S. Neurenteric cyst of the upper cervical spine: excision via posterior approach. Pediatr Neurosurg. 2005;42(1):54–6.CrossRefGoogle Scholar
  67. 67.
    Devkota U, Lam J, Ng H, Poon W. An anterior intradural neurenteric cyst of the cervical spine. Neurosurgery. 1994;35(6):1150–3.PubMedCrossRefGoogle Scholar
  68. 68.
    Garg N, Sampath S, Yasha T, Chandramouli B, Indira Devi B, Kovoor J. Is total excision of spinal neurenteric cysts possible? Br J Neurosurg. 2008;22(2):241–51.PubMedCrossRefGoogle Scholar
  69. 69.
    Powell K, Cherry J, Hougen T, Blinderman E, Dunn M. A prospective search for congenital dermal abnormalities of the craniospinal axis. J Pediatr. 1975;87(5):744–50.PubMedCrossRefGoogle Scholar
  70. 70.
    Amacher L, Hovind L. Intracranial tumefactions in children. Pediatr Neurosurg. 1979;5(6):518–29.CrossRefGoogle Scholar
  71. 71.
    Kanev P, Park T. Dermoids and dermal sinus tracts of the spine. Neurosurg Clin N Am. 1995;6:359–66. Available at: https://www.ncbi.nlm.nih.gov/pubmed/7620359.PubMedCrossRefGoogle Scholar
  72. 72.
    French B. The embryology of spinal dysraphism. Clin Neurosurg. 1983;30:295–340. Available at: https://www.ncbi.nlm.nih.gov/pubmed/6365396.PubMedCrossRefGoogle Scholar
  73. 73.
    Gupta D, Shastank R, Mahapatra A. An unusual presentation of lumbosacral dermal sinus with CSF leak and meningitis. Pediatr Neurosurg. 2005;41(2):98–101.PubMedCrossRefGoogle Scholar
  74. 74.
    Naidich T, Hardwood-Nash D, McLone D. Radiology of spinal dysraphism. Clin Neurosurg. 1983;30:341–65.PubMedCrossRefGoogle Scholar
  75. 75.
    Clark P, Davidson L. Case report: pseudotail with dermal sinus tract and tethered cord. J Ultrasound. 2016;19(3):239–41.PubMedPubMedCentralCrossRefGoogle Scholar
  76. 76.
    Shah R, Chaljub G, Swischuk L. Lower cervical dermal sinus tract and associated intraspinal abscess causing meningitis in a child. Emerg Radiol. 2003;10(3):160–2.PubMedCrossRefGoogle Scholar
  77. 77.
    Mete M, Umur A, Duransoy Y, Barutçuoğlu M, Umur N, Gurgen S, Selçuki M. Congenital dermal sinus tract of the spine. J Child Neurol. 2014;29(10):1277–82.PubMedCrossRefGoogle Scholar
  78. 78.
    O’Neill B, Gallegos D, Herron A, Palmer C, Stence N, Hankinson T, Corbett Wilkinson C, Handler M. Use of magnetic resonance imaging to detect occult spinal dysraphism in infants. J Neurosurg Pediatr. 2017;19(2):217–26.PubMedCrossRefGoogle Scholar
  79. 79.
    Ackerman L, Menezes A. Spinal congenital dermal sinuses: a 30-year experience. Pediatrics. 2003;112(3):641–7.PubMedCrossRefGoogle Scholar
  80. 80.
    Radmanesh F, Nejat F, El Khashab M. Dermal sinus tract of the spine. Childs Nerv Syst. 2009;26(3):349–57.PubMedCrossRefGoogle Scholar
  81. 81.
    Singh I, Rohilla S, Kumar P, Sharma S. Spinal dorsal dermal sinus tract: an experience of 21 cases. Surg Neurol Int. 2015;6(18):429.CrossRefGoogle Scholar
  82. 82.
    Ramdurg S, Sukanya M, Maitra J. Pediatric encephaloceles: a series of 20 cases over a period of 3 years. J Pediatr Neurosci. 2015;10(4):317.PubMedPubMedCentralCrossRefGoogle Scholar
  83. 83.
    Thompson D. Spinal inclusion cysts. Childs Nerv Syst. 2013;29(9):1647–55.PubMedCrossRefGoogle Scholar
  84. 84.
    Newton E. Syringomyelia as a manifestation of defective fourth ventricular drainage. Ann R Coll Surg Engl. 1969;44:194–213. Available at: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2387612/.PubMedPubMedCentralGoogle Scholar
  85. 85.
    Iskandar B, Oakes J, McLaughlin C, Osumi A, Tien R. Terminal syringohydromyelia and occult spinal dysraphism. J Neurosurg. 1994;81(4):513–9.PubMedCrossRefGoogle Scholar
  86. 86.
    Gardner W, Goodall R. The surgical treatment of Arnold-Chiari malformation in adults. J Neurosurg. 1950;7(3):199–206.PubMedCrossRefGoogle Scholar
  87. 87.
    Rodriguez A, Kuhn E, Somasundaram A, Couture D. Management of idiopathic pediatric syringohydromyelia. J Neurosurg Pediatr. 2015;16(4):452–7.PubMedCrossRefGoogle Scholar
  88. 88.
    Hankinson T, Klimo P, Feldstein N, Anderson R, Brockmeyer D. Chiari malformations, syringohydromyelia and scoliosis. Neurosurg Clin N Am. 2007;18(3):549–68.PubMedCrossRefGoogle Scholar
  89. 89.
    Timpone V, Patel S. MRI of a syrinx: is contrast material always necessary? Am J Roentgenol. 2015;204(5):1082–5.CrossRefGoogle Scholar
  90. 90.
    Anderson N, Willoughby E, Wrightson P. The natural history and the influence of surgical treatment in syringomyelia. Acta Neurol Scand. 1985;71:472–9.PubMedCrossRefGoogle Scholar
  91. 91.
    Tsitouras V, Sgouros S. Syringomyelia and tethered cord in children. Childs Nerv Syst. 2013;29(9):1625–34.PubMedCrossRefGoogle Scholar
  92. 92.
    Logue V, Edwards M. Syringomyelia and its surgical treatment – an analysis of 75 patients. J Neurol Neurosurg Psychiatry. 1981;44(4):273–84.PubMedPubMedCentralCrossRefGoogle Scholar
  93. 93.
    Goel A, Desai K. Surgery for syringomyelia: an analysis based on 163 surgical cases. Acta Neurochir. 2000;142(3):293–302.PubMedCrossRefGoogle Scholar
  94. 94.
    Elster A, Chen M. Chiari I malformations: clinical and radiologic reappraisal. Radiology. 1992;183(2):347–53.PubMedCrossRefGoogle Scholar
  95. 95.
    Caird J, Flynn P, McConnell R. Significant clinical and radiological resolution of a spinal cord syrinx following the release of a tethered cord in a patient with an anatomically normal conus medullaris. J Neurosurg Pediatr. 2008;1(5):396–8.PubMedCrossRefGoogle Scholar

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Authors and Affiliations

  1. 1.Seattle Science FoundationSeattleUSA

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