The Surgery of Occult Spinal Dysraphism

  • M. Choux
  • G. Lena
  • L. Genitori
  • M. Foroutan
Part of the Advances and Technical Standards in Neurosurgery book series (NEUROSURGERY, volume 21)


The spinal dysraphism state includes all lesions which are the consequence of incomplete formation of the midline structures of the dorsum. It is therefore related to all forms of spina bifida (spina bifida aperta or cystica) as well as occult spinal dysraphism. The basic differences of both malformations result from the nature of their origin, Spina bifida cystica is an intrinsic failure of development of the spinal cord tissues. with added destructive processes is utero and cellular dysplasia. together with brain anomalies. By contrast, in spina bifida ooculta there may be some dysplasia on the spinal cord but none of the other destructive processes. Chiari malformation is very unusual in spina bifida occulta. The term occulta means toac the malformation is covered by skin, with no cutaneous abnormalities. Occult spinal dysraphism and spina bifida occulta are genenlly synonymous. We prefer the term occult spinal dysraphism to spina bifida occulta. The common pathological finding is a tethered spinal cord even is lesions such as dermal sinus or neurenteric cysts (typically considered as occult spinal dysraphic lesions) are not generally associated with tethering of the spinal cord.


Spina Bifida Thick Filum Tethered Cord Pediatric Neurosurgery Dermal Sinus 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Amacher A, Drake CG, McLachine AD (1968) Anterior sacral meningocele. Surg Gynecol Obstet 126: 986–994.PubMedGoogle Scholar
  2. 2.
    Anderson EM (1975) Occult spinal dysraphism: a series of 73 cases. Pediatrics 55: 826–835.PubMedGoogle Scholar
  3. 3.
    Assaad A, Mansy A, Kotb M, Hafez M (1989) Spinal dysraphism: experience with 250 cases operated upon. Childs Nerv Syst 5(5): 324–329.PubMedCrossRefGoogle Scholar
  4. 4.
    Barnes PD, Lester PD, Yamanashi WS, Prince JR (1986) Magnetic resonance imaging in infants and children with spinal dysraphism. AJNR 7: 465.Google Scholar
  5. 5.
    Bentley JFR, Smith JR (1960) Developmental posterior enteric remnants and spinal malformations. Arch Dis Child 35: 76–86.PubMedCrossRefGoogle Scholar
  6. 6.
    Bollini G (1989) Sacral and lumbar agenesis. In: Raimondi A, Choux M, Di Rocco C, Principles of pediatric neurosurgery. The pediatrie spine, Vol II. Springer, Berlin Heidelberg New York Tokyo, pp 144–166.Google Scholar
  7. 7.
    Bollini G (1989) Dysraphisme spinal fermé. Cahiers d’enseignement de la SOFCOT. Conférences d'enseignement. Masson, Paris, pp 11-34.Google Scholar
  8. 8.
    Boop FA, Chadduck WM (1991) Silastic duraplasty in pediatrie patients. Neurosurgery 29(5): 785–787 (discussion 788).PubMedCrossRefGoogle Scholar
  9. 9.
    Borne G, Cristino G, Bedou G, Schaefer JP, Pinaudea M (1990) Dysembryome pluritissulaire mature medullaire lombaire avec spina bifida occulta sacre. Neurochirurgie 36(3): 180–184.PubMedGoogle Scholar
  10. 10.
    Bremer JL (1952) Dorsal intestinal fistula occupying vertebral canal: diastematomyelia. Arch Pathol Lab Med 54: 132–138.Google Scholar
  11. 11.
    Bret P, Patet JP, Lapras C (1989) Diastematomyelia and diploymelia. In: Raimondi A, Choux M, Di Rocco C (eds) The principles of pediatrie neurosurgery. The pediatrie spine, Vol II.Google Scholar
  12. 12.
    Bruce DA, Shut L (1982) Spinal lipomas in infancy and childhood. Childs Brain 5: 192–203.Google Scholar
  13. 13.
    Bryant T (1837) Case of deficiency of the anterior part of the sacrum with a thecal sac in the pelvis; similar to the tumour of the spina bifida. Lancet: 358-360.Google Scholar
  14. 14.
    Byrd SE, Darling CF, McLone DG (1991) Developmental disorders of the pediatrie spine. Radiol Clin North Am 29(4): 711–752.PubMedGoogle Scholar
  15. 15.
    Carcassonne M, Bergoin M, Choux M, Monfort G, Gregoire A, Morisson-Lacombe G, Bollini G (1981) Les malformations de la colonne lombo-sacrée et leurs implications viscérales. (Myéloméningocele exceptée). Chir Ped 22(2-3): 1–223.Google Scholar
  16. 16.
    Carvajal L, Uraga E, Tama, F, Martinez G (1989) Mal perforans and spinal dysraphia and lumbar stenosis syndromes. Med Cutan Ibero Lat Am 17(6): 360–364.PubMedGoogle Scholar
  17. 17.
    Chapman PH (1982) Congential intraspinal lipomas. Anatomic considerations and surgical treatment. Childs Brain 9: 37–47.PubMedGoogle Scholar
  18. 18.
    Chapman PH, Beyerl B (1986) The tethered spinal cord with particular reference to spinal lipoma and diastematomyelia. In: Hoffman HJ, Epstein F (eds) Disorders of the developing nervous system. Diagnosis and treatment. Blackwell, Boston, pp 109–132.Google Scholar
  19. 19.
    Choux M (1981) Les Malformations de la colonne lombo-sacrée et leurs implications viscérales. (Myéloméningocele exeptée). Chir Ped 22(2-3): 136–159.Google Scholar
  20. 20.
    Cohen AR (1991) The mermaid malformation: cloacal extrophy and occult spinal dysraphism. Neurosurgery 28(6): 834–843.PubMedCrossRefGoogle Scholar
  21. 21.
    Davidoff AM, Thompson CV, Grimm JM, Shorter NA, Filston HC, Oakes WJ (1991) Occult spinal dysraphism in patients with anal agenesis. J Pediatr Surg 26(8): 1001–1005.PubMedCrossRefGoogle Scholar
  22. 22.
    De-Bakker HM, Roos RA, Voormolen JH, Vielvoye GJ (1990) Lumbar disk degeneration in spinal dysraphism. AJNR 11(2): 415.PubMedGoogle Scholar
  23. 23.
    Di Pietro MA, Venes JL (1988) Real time sonography of the pediatrie spinal cord: horizons and limits. In: Concepts in pediatrie neurosurgery, Vol 8. Karger, Basel, pp 120–132.Google Scholar
  24. 24.
    Di Rocco C, Ceddia A, Colosimo C (1989) Spinal meningeal malformations. In: Raimondi A, Choux M, DiRocco C (eds) Principles in pediatrie neurosurgery. The pediatrie spine, Vol II. Springer, Berlin, Heidelberg, New York, Tokyo, pp 1–16.Google Scholar
  25. 25.
    Eisenbrey A (1982) Dermal sinus. In: Pediatrie neurosurgery. Surgery of the developing nervous system. Grune and Stratton, New York, pp 91–94.Google Scholar
  26. 26.
    Erkulvrawatr S (1979) Intrathoracic meningoceles and neurofibromatosis. Arch Neurol 36: 557–559.PubMedCrossRefGoogle Scholar
  27. 27.
    French BN (1983) The embryology of spinal dysraphism. Clin Neurosurg 30: 295–340.PubMedGoogle Scholar
  28. 28.
    Frerebeau Ph, Dimeglio A, Gras M, Harbi H (1983) Diastematomyelia report of 21 cases surgically treated by a neurosurgical and orthopedic team. Childs Brain 10: 328–339.PubMedGoogle Scholar
  29. 29.
    Garceau GJ (1953) The filum terminale syndrome. J Bone Joint Surg 35A: 711–716.Google Scholar
  30. 30.
    Guthkelch AN (1974) Diastematomyelia with median septum. Brain 4: 729–742.CrossRefGoogle Scholar
  31. 31.
    Guthkelch AN (1985) Diastematomyelia. In: Wilkins RH, Rengachary SS (eds) Neurosurgery, Vol III. McGraw-Hill, New York pp 2058–2061.Google Scholar
  32. 32.
    Han JS, Benson JE, Kaufman B, Rekate HL, Alfidi RJ, Bohlman HH (1985) Demonstration of diastematomyelia and associated abnormalities with MR imaging. AJNR 6: 215.PubMedGoogle Scholar
  33. 33.
    Harrison MJ, Mitnich RJ, Rosenblum BR, Rothman AS (1990) Leptomyelolipoma: analysis of 20 cases. J Neurosurg 73(3): 360–367.PubMedCrossRefGoogle Scholar
  34. 34.
    Hawnaur JM, Hughes D, Jenkins JP, Bannister CM, Isherwood I (1991) Investigation of children with suspected spinal dysraphism by magnetic resonance imaging. Eur J Pediatr Surg 1[Suppl 1]: 18–19.PubMedCrossRefGoogle Scholar
  35. 35.
    Hilal SK, Marton D, Pollack E (1974) Diastematomyelia in children. Radiology 112: 609–621.PubMedGoogle Scholar
  36. 36.
    Hirsch JF, Hirsch E (1989) Neurenteric cysts. In: Raimondi A, Choux M, DiRocco C (eds) Principles of pediatric neurosurgery. The pediatric spine, Vol II. Springer, Berlin Heidelberg New York Tokyo, pp 134–143.Google Scholar
  37. 37.
    Hoffman HJ (1987) Spinal dysraphism. Am Fam Physician 36(6): 129–136.PubMedGoogle Scholar
  38. 38.
    Hoffman HJ, Hendrick EB, Humphrey RP (1976) The tethered spinal cord: its manifestations, diagnosis and surgical correction. Childs Brain 2: 145–155.PubMedGoogle Scholar
  39. 39.
    Hoffman HJ, Taecholarn C, Hendrick BE, Humphreys RP (1985) Lipomyelomeningoceles and their management. In: Concepts in pediatrie neurosurgery, Vol 5. Karger, Basel, pp 107–117.Google Scholar
  40. 40.
    Hoffman HJ (1989) The Tethered spinal cord. In: Raimondi A, Choux M, Di Rocco C (eds) Principles of pediatrie neurosurgery. The pediatrie spine, Vol II. Springer, Berlin Heidelberg New York Tokyo, pp 177–188.Google Scholar
  41. 41.
    Hohl AF (1852) Zur Pathologie des Beckens. I. Das schräg-ovale-Becken. Engelmann, Leipzig, p 61.Google Scholar
  42. 42.
    Hood RW, Risenborough EJ, Nehme AM, Micheli LJ, Strand RD, Neuhauser EB (1980) Diastematomyelia and structural spinal deformities. J Bone Joint Surg 62A: 520–528.Google Scholar
  43. 43.
    Humphreys RP (1991) Current trends in spinal dysraphism. Paraplegia 29(2): 79–83.PubMedCrossRefGoogle Scholar
  44. 44.
    Humphreys RP, Manwaring KH, Carroll NC (1991) Accessory arm, dysraphism or disparity? Case report. J Neurosurg 74(2): 297–300.PubMedCrossRefGoogle Scholar
  45. 45.
    James CCM, Lassman LP (1960) Spinal dysraphism. Arch Dis Child 35: 315–327.PubMedCrossRefGoogle Scholar
  46. 46.
    James CCM, Lassman LP (1962) Spinal dysraphism. J Bone Joint Surg (Br) 44: 828–840.Google Scholar
  47. 47.
    James CCM, Lassman LP (1972) Spinal dysraphism. Spina bifida occulta. Butterworths, London.Google Scholar
  48. 48.
    James HE, Williams J, Brock W, Kaplan GW, U HS (1984) Radical removal of lipomas of the conus and the cauda equina with laser microneurosurgery. Neurosurgery 15: 340–343.PubMedCrossRefGoogle Scholar
  49. 49.
    Kapsalakis Z (1964) Diastematomyelia in two sisters. J Neurosurgery 21: 66–67.CrossRefGoogle Scholar
  50. 50.
    Khoury AE, Hendrick EB, McLorie GA, Kulkarni A, Churchill BM (1990) Occult spinal dysraphism: clinical and urodynamic outcome after division of the filum terminale. J Urol 144: 426-8; Discussion 428-429 and 443–444.Google Scholar
  51. 51.
    Kosnic EJ (1990) A review of the treatment of children with a lipomyelomeningocele at the Columbus Children’s Hospital. In: Concepts pediatrie neurosurg, Vol 10. Karger, Basel, pp 15–21.Google Scholar
  52. 52.
    Lapras C, Patet JD, Huppert J, Bret Ph (1985) Syndrome de traction du cone terminal ou syndrome de la moelle attachée; Lipomes lombo-sacrée. Rev Neurol 141: 207–215.PubMedGoogle Scholar
  53. 53.
    Lapras C, Patet JD, Hupert J, Bret Ph, Mottolese C (1985) Tethered cord syndrome, experience of 58 cases. J Neuroped Sci 1(1): 39–50.Google Scholar
  54. 54.
    Lapras C (1988) Spina bifida. Neurochirurgie 34 [Suppl 1]: 1–103.PubMedGoogle Scholar
  55. 55.
    Lassman LP, James CCM (1977) Meningocele manquée. Childs Brain 3: 1–11.PubMedGoogle Scholar
  56. 56.
    Leibowitz E, Barton W, Sadighi P, Ross JS (1984) Anterior sacral meningocele contiguous with a pelvic hamartoma, case report. J Neurosurgery 61: 188–190.CrossRefGoogle Scholar
  57. 57.
    MacMaster MJ (1984) Occult intraspinal anomalies and congenital scoliosis. J Bone Joint Surg 66A: 588–601.Google Scholar
  58. 58.
    Matson DD, Ingraham FA (1969) Neurosurgery of infancy and childhood. Thomas, Springfield.Google Scholar
  59. 59.
    Matson DD, Woods RP, Campbell JB, Ingraham FD (1950) Diastematomyelia (congenital clefts of the spinal cord). Diagnosis and surgical treatment. Pediatrics 6: 98–112.PubMedGoogle Scholar
  60. 60.
    McComb JG (1985) Congenital dermal sinus. In: Wilkins RH, Rengachary SS (eds) Neurosurgery, Vol III. McGraw-Hill, New York, pp 2081–2083.Google Scholar
  61. 61.
    McCullough DC, Levy LM, DiChiro G, Johnson DL (1990-91) Toward the prediction of neurological injury from tethered spinal cord: investigation of cord motion with magnetic resonance. Pediatr Neurosurg 16(1): 3–7, Discussion 7.PubMedCrossRefGoogle Scholar
  62. 62.
    McEwen GD, Bunnel WP, Sriram K (1975) Acute neurological complication in the treatment of scoliosis. A report of the Scoliosis Research Society. J Bone Joint Surg 57A: 404.Google Scholar
  63. 63.
    McLone DG, Mutlur S, Naidich TP (1983) Lipomyelomeningoceles of the conus medullaris. In: Concepts in pediatrie neurosurgery, Vol 3. Karger, Basel, pp 170–177.Google Scholar
  64. 64.
    McLone DG, Naidich TP (1985) The tethered cord syndrome. In: Holtzman RN, Stein BM (eds) Spinal dysraphism experimental and clinical. Thieme Stratton, New York, pp 14–28.Google Scholar
  65. 65.
    McLone DG, Naidich TP (1986) Laser resection of fifty spinal lipomas. Neurosurgery 18: 611–615.PubMedCrossRefGoogle Scholar
  66. 66.
    Monfort G (1989) Diagnostic and management of urinary problems in neurogenic vesico-sphincteric dysfunction. In: Raimondi A, Choux M, DiRocco C (eds) Principles of pediatrie neurosurgery. The pediatrie spine, Vol III. Springer, Berlin Heidelberg New York Tokyo, pp 187–208.Google Scholar
  67. 67.
    Neuwirth MG, Nainzadeh NK, Bernstein RL (1988) The use of pudendal nerve in monitoring lower sacral roots (S2-4) during anterior and/or posterior spinal stabilization. A report of the Scoliosis Research Society, September/October 1988, Baltimore.Google Scholar
  68. 68.
    Ollivier d’Angers (1937) Traité des maladies de la moëlle épinière, 3rd Ed, Vol 1. Mequignon-Marvis, Paris, pp 189–199.Google Scholar
  69. 69.
    O’Neill P, Stack JP (1990) Magnetic resonance imaging in the preoperative assessment of closed spinal dysraphism in children. Pediatr Neurosurg 16: 240–246.PubMedCrossRefGoogle Scholar
  70. 70.
    O’Neill P, Singh J (1991) Occult spinal dysraphism in children: need for early neurosurgical referral. Childs Nerv Syst 7(6): 309–311.PubMedCrossRefGoogle Scholar
  71. 71.
    Page LK (1985) Occult spinal dysraphisms and related disorders. In: Wilkins RH, Rengachery SS (eds) Neurosurgery, Vol III. McGraw-Hill, New York, pp 2053–2058.Google Scholar
  72. 72.
    Pang D, Wilberger JE (1982) Tethered cord syndrome in adults. J Neurosurg 57: 32–47.PubMedCrossRefGoogle Scholar
  73. 73.
    Pang D (1985) Sacral agenesis. In: Wilkins RH, Rengachery SS (eds) Neurosurgery, Vol III. McGraw-Hill, New York, pp 2075–2077.Google Scholar
  74. 74.
    Pang D (1992) Split cord malformation (Part I): a unified theory of embryogenesis for double spinal cord malformation. Neurosurgery 3: 451–480.CrossRefGoogle Scholar
  75. 75.
    Pang D (1992) Split cord malformation (Part II): clinical syndrome. Neurosurgery 3: 481–500.CrossRefGoogle Scholar
  76. 76.
    Parkinson D (1991) Accessory limbs and spinal dysraphism. Letter. J Neurosurg 75(3): 498–499.PubMedGoogle Scholar
  77. 77.
    Peter JC (1992) Occult dysraphism of the spine. A retrospective analysis of 88 operative cases, 1979-1989. S Afr Med J 81(7): 351–354.PubMedGoogle Scholar
  78. 78.
    Pierre-Kahn A, Renier D, Sainte-Rose C, Hirsch JF (1983) Les lipomes lombo-sacrées avec spina bifida. Corrélations anatomo-cliniques. Résultats thérapeutiques. Neurochirurgie 29: 359–363.PubMedGoogle Scholar
  79. 79.
    Pierre-Kahn A (1986) Intraspinal lipoma with spina bifida. Prognosis and treatment. J Neurosurg 65: 756–761.PubMedCrossRefGoogle Scholar
  80. 80.
    Puussepp M (1934) Variété rare de tératome sous-dural de la région cervicale (intestinome). Quadriplégie, extirpation. Guérison complète. Rev Neurol (Paris) 41: 876–886.Google Scholar
  81. 81.
    Raimondi AJ (1987) Vertebrospinal congenital anomalies. In: Raimondi AJ (ed) Pediatrie neurosurgery. Theoretical principles, art of surgical techniques. Springer, Berlin Heidelberg New York Tokyo, pp 423–452.Google Scholar
  82. 82.
    Rasool MN, Govender S, Naidoo KS, Moodley M (1992) Foot deformities and occult spinal abnormalities in children: a review of 16 cases. J Pediatr Orthop 12(1): 94–9.PubMedGoogle Scholar
  83. 83.
    Raybaud CA, Naidich TP, McLone DG (1992) Development of the spine and spinal cord. In: Manelfe C (ed) Imagerie of the spine and spinal cord. Raven, New York.Google Scholar
  84. 84.
    Reigel DH (1983) Tethered spinal cord. Concepts in pediatric neurosurgery, Vol 4. Karger, Basel, pp 142–164.Google Scholar
  85. 85.
    Roy MW, Gilmore R, Walsh JW (1986) Evaluation of children and young adults with tethered spinal cord syndrome. Utility of spinal and scalp recorded somatosensory-evoked potentials. Surg. Neurol 26: 241–248.Google Scholar
  86. 86.
    Sato K, Shimoji T, Sumie H, Yaguachi K, Yagishita A, Kuru Y, Shozo S (1985) Surgically confirmed myelografic classification of the intraspinal lipoma in the lumbosacral region. Childs Nerv Syst 1: 3–11.PubMedCrossRefGoogle Scholar
  87. 87.
    Sauter R, Klemm T (1988) Spinal sonography of a newborn infant with postpartal paraplegia. Klin Padiatr 200(1): 70–73.PubMedCrossRefGoogle Scholar
  88. 88.
    Scatliff JH, Till K, Hoare RD (1975) Incomplete, false and true diastematomyelia. Radiological revaluation by air myelography and tomography. Neuroradiology 166: 349–354.Google Scholar
  89. 89.
    Scatliff JH, Kendall BE, Kingsley DP, Britton J, Grant DN, Hayward RD (1989) Closed spinal dysraphism: analysis of clinical, radiological, and surgical findings in 104 consecutive patients. AJR 152(5): 1049–1057.PubMedGoogle Scholar
  90. 90.
    Steinbok P, Cochrane DD (1991) The nature of congenital posterior cervical or cervicothoracic midline cutaneous mass lesions. Report of eight cases. J Neurosurg 75(2): 206–212.PubMedCrossRefGoogle Scholar
  91. 91.
    Till K (1969) Spinal dysraphism: a study of congenital malformations of lower back. J Bone Joint Surg 51: 415–422.Google Scholar
  92. 92.
    Villarejo FJ, Blazquez MG, Gutierrez-Diaz JA (1976) Intraspinal lipomas in children. Childs Brain 2: 361–370.PubMedGoogle Scholar
  93. 93.
    Villarejo F, Slavone C, Blazquez ME, Pascual-Castro-Viejo I, Perez-Higueras A, Fernandez-Sanchez A, Garcia Bertrand C (1983) Anterior sacral meningocele: review of the literature. Surg Neurol 19: 57–71.PubMedCrossRefGoogle Scholar
  94. 94.
    Walsh J, Markesbery W (1980) Histological features of congenital lipoma of the lower spinal canal. J Neurosurg 52: 564–569.PubMedCrossRefGoogle Scholar
  95. 95.
    Warf BC, Scott RM, Barnes PD, Hendren WH (1993) Tethered spinal cord in patients with anorectal and urogenital malformations. Pediatr Neurosurg 19: 25–30.PubMedCrossRefGoogle Scholar
  96. 96.
    Wilkins RH (1985) Intraspinal cysts. In: Wilkins RH, Rengachary SS (eds) Neurosurgery, Vol III. McGraw-Hill, New York, pp 2061–2070.Google Scholar
  97. 97.
    Wilkins RH (1985) Lateral and anterior spinal meningoceles. In: Wilkins RH, Rengachary SS (eds) Neurosurgery, Vol III. McGraw-Hill, New York, pp 2070–2075.Google Scholar
  98. 98.
    Wyspold F, Citrin C, Bakovich A, Sherman J (1987) Evaluation of MR in spinal dysraphism with lipoma, comparison with metrizamide computed tomography. Pediatr Radiol 17: 184–188.CrossRefGoogle Scholar
  99. 99.
    Yamane T, Shinoto A, Kamegaya M, Shinada Y (1991) Spinal dysraphism. A study of patients over the age of 10 years. Spine 16(11): 1295–1297.PubMedCrossRefGoogle Scholar
  100. 100.
    Yamada S, Zinke DE, Sandres D (1981) Pathophysiology of “tethered cord syndrome”. J Neurosurg 54: 494–503.PubMedCrossRefGoogle Scholar
  101. 101.
    Zumkeller M, Seifert V, Stolke D (1989) Spinal dysraphia and disordered ascension of the spinal cord in adults. Z Orthop 127(3): 336–342.PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag/Wien 1994

Authors and Affiliations

  • M. Choux
    • 1
  • G. Lena
    • 1
  • L. Genitori
    • 1
  • M. Foroutan
    • 1
  1. 1.Department of Pediatric NeurosurgeryHopital des Enfants, La TimoneMarseilleFrance

Personalised recommendations