Pathological Anatomy of Spina Bifida

  • Aydin Sav


Spina bifida occulta results from incomplete closure of the neural tube around the twentieth day of embryonic development [1]. Spina bifida was described in the medieval literature and was recognized even earlier. Indeed, the association of foot deformities with sacral hypertrichosis may be the origin of the mythological figure of the satyr [2]. The term spina bifida encompasses the entire central nervous system, ranging from merely an absent spinous process through to myelomeningocele (MMC), Chiari malformation and hydrocephalus to cortical cytoarchitectural changes [2].


Spina Bifida Spinal Dysraphism Dermal Sinus Tethered Cord Syndrome Conus Medullaris 
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.
    Banta JV, Lin R, Peterson M et al (1990) The team approach in the care of the child with myelomeningocele. JPO 2:263–73Google Scholar
  2. 2.
    von Recklinghausen(1886) In: Friede RL (ed) Develop — mental neuropathology. 2nd and revised edition. Springer-Verlag, Berlin Heidelberg New York, pp 248–262Google Scholar
  3. 3.
    Kumar R, Bansal KK, Chhabra DK (2002) Occurrence of split cord malformation in meningomyelocele: Complex spina bifida. Pediatr Neurosurg 36:119–127PubMedCrossRefGoogle Scholar
  4. 4.
    Sattar MT, Bannister CM, Turnbull IW (1996) Occult spinal dysraphism-The common combination of lesions and the clinical manifestations in 50 patients. Eur J Pediatr Surg 6 Suppl 11:10–14PubMedGoogle Scholar
  5. 5.
    Tortori-Donati P, Rossi A, Cama A (2000) Spinal dysraphism: a review of neuroradiological features with embryological correlations and proposal for a new classification. Neuroradiol 42:471–491CrossRefGoogle Scholar
  6. 6.
    Reigel DH, Rotenstien D (1994) Spina bifida. In: Section of pediatric neurosurgery of the American Association of Neurological Surgeons (ed) Pediatric neurosurgery, 3rd edn. Saunders, Philadelphia, pp 51–76Google Scholar
  7. 7.
    Iskandar BJ, McLaughlin C, Oakes WJ (2000). Split cord malformations in myelomeningocele patients. Br J Neurosurg 14:200–203PubMedCrossRefGoogle Scholar
  8. 8.
    Sutow WW, Pryde AW (1956) Incidence of spina bifida occulta in relation to age. Am J Dis Child 91:211–217Google Scholar
  9. 9.
    James CC, Lassman LP (1972) Spinal dysraphism. Spina bifida occulta. Butterworth, LondonGoogle Scholar
  10. 10.
    Alter M (1962) Anencephalus, hydrocephalus, and spina bifida. Epidemiology, with special reference to a survey in Charleston. Arch Neurol 7:411–422PubMedGoogle Scholar
  11. 11.
    Boris M, Blumbcig R, Feldman DB, Sellers TF Jr (1963) Increased incidence of meningomyeloceles. JAMA 184:768Google Scholar
  12. 12.
    Lucey JF, Mann RW, Simmons GM, Friedman E (1964) An increased incidence of spina bifida in Vermont in 1962. Pediatrics 33:981–984PubMedGoogle Scholar
  13. 13.
    Timson J (1969) The sex ratio in spina bifida. Genetics 40:427–433CrossRefGoogle Scholar
  14. 14.
    Record RG, McKeown T (1949) Congenital malformations of the central nervous system I. A survey of 930 cases. Br I Soc Med 3:183–219Google Scholar
  15. 15.
    Doran PA, Guthkelch AN (1961) Studies in spina bifida cystica. General survey and reassessment of the problem. J Neurol Neurosurg Psychiatry 24:331–345PubMedGoogle Scholar
  16. 16.
    Friede RL (1989) Developmental neuropathology. 2nd and revised edition. Springer-Verlag, Berlin Heidelberg New YorkGoogle Scholar
  17. 17.
    Cama A, Tortori-Donati P, Piatelli GL et al (1995) Chiari complex in children. Neuroradiological diagnosis, neurosurgical treatment and proposal of a new classification (312 cases). Eur J Pediatr Surg 5 (Suppl 1):35–38PubMedGoogle Scholar
  18. 18.
    Tortori-Donati P, Cama A, Fondelli MP et al (1996) Le malformazioni di Chiari. In: Tortori-Donati P, Taccone A, Longo M (eds) Malformazioni cranio — encefaliche. Neuroradiologia. Minerva Medica, Turin, pp 209–236Google Scholar
  19. 19.
    Keiller VH (1922) A contribution to the anatomy of spina bifida. Brain 45:31–103CrossRefGoogle Scholar
  20. 20.
    Barson AJ (1970) Spina bifida: the significance of the level and extent of the defect to the morphogenesis. Dev Med Child Neurol 12:129–144PubMedGoogle Scholar
  21. 21.
    Fisher RG, Uihlein A, Keith HM (1952) Spina bifida and cranium bifidum: study of 530 cases. Proc Staff Meet Mayo Clin 27:33–38PubMedGoogle Scholar
  22. 22.
    Naidich TP, Zimmerman RA, McLone DG et al (1996) Congenital anomalies of the spine and spinal cord. In: Atlas SW (ed) Magnetic resonance imaging of the brain and spine, 2nd edn. Lippincott-Raven, Philadelphia, pp 1265–1337Google Scholar
  23. 23.
    Cameron AH (1957) Arnold-Chiari and neuro — anatomical malformations associated with Spina bifida. J Pathol Bacteriol 73:195–211CrossRefGoogle Scholar
  24. 24.
    Daniel PM, Strich SJ (1958) Some observations on the congenital deformity of the central nervous system known as the Arnold-Chiari malformation. J Neuropathol Exp Neurol 17:255–266PubMedCrossRefGoogle Scholar
  25. 25.
    Cleland J (1883) Contribution to the study of spina bifida, encephalocele and anencaphalus. J Anat Physiol 17:257–292PubMedGoogle Scholar
  26. 26.
    Peach B (1965) Arnold-Chiari malformation: morphogenesis. Arch Neurol 12:527–35PubMedGoogle Scholar
  27. 27.
    MacKenzie NG, Emery JL (1971) Deformities of central nervous cord in children with neurospinal dysraphism. Dev Med Child Neurol 13(Suppl 25):58–61Google Scholar
  28. 28.
    Gibert N, Jones KC, Rorke LB et al (1986) Central nervous system anomalies associated with menin — gomyelocele, hydrocephalus, and the Arnold-Chiari malformations: reappraisal of thesis regarding the pathogenesis of posterior neural tube closure defects. Neurosurgery 18:559–563Google Scholar
  29. 29.
    Pang D, Dias MS, Ahab-Barmada M (1992) Split cord malformation. I. A unified theory of embryogenesis for double spinal cord malformations. Neurosurgery 31:451–480PubMedCrossRefGoogle Scholar
  30. 30.
    Breningstall GN, Marker SM, Tubman DE (1992) Hydrosyringomyelia and diastema tomyelia detected by MRI in myelome ningocele. Pediatr Neurol 8:267–271PubMedCrossRefGoogle Scholar
  31. 31.
    Pang D, Dias MS (1993) Cervical myelo meningoceles. Neurosurgery 33:363–373PubMedCrossRefGoogle Scholar
  32. 32.
    Naidich TP, McLonc DG, Mutluer S (1983) A new understanding of dorsal dysraphism with lipoma (lipomye loschisis): radiological evaluation and sur gical correlation. AJNR 4:103–116Google Scholar
  33. 33.
    Pierre-Kahn A, Zerah M, Renier D et al (1997) Congenital lumbosacral lipomas. Childs Nerv Syst 13:298–334PubMedCrossRefGoogle Scholar
  34. 34.
    Raimondi AJ (1989) Hamartomas and the dysraphic state. In: Raimondi AJ, Choux M, Di Rocco C (eds) The pediatric spine 1. Development and the dysraphic state. Springer, Berlin, pp 179–199Google Scholar
  35. 35.
    Lee KS, Gower DJ, McWhorter JM et al (1988) The role of MR imag ing in the diagnosis and treatment of ante rior sacral meningocele. Report of 2 cases. J Neurosurg 69:628–631PubMedGoogle Scholar
  36. 36.
    Castillo M, Mukherji SK (1996) Imaging of the pediatric head, neck, and spine. Lippincott-Raven, Philadelphia, pp 638–640Google Scholar
  37. 37.
    Okada T, Imae S, Igarashi S et al (1996) Occult intrasacral me ningocele associated with spina bifida: a case report. Surg Neurol 46:147–149PubMedCrossRefGoogle Scholar
  38. 38.
    Byrd SE, Harvey C, Darling CF (1995) MR of terminal myelocystoceles. Eur J Radiol 20:215–220PubMedCrossRefGoogle Scholar
  39. 39.
    Peacock WJ, Murovic JA (1989) Magnetic resonance imaging in myelocystoceles. Report of two cases. J Neurosurg 70:804–807PubMedGoogle Scholar
  40. 40.
    McComb JG (1993) Comment on: Pang D, Dias MS. Cervical myelomeningoceles. Neurosurgery 33:373Google Scholar
  41. 41.
    Parent AD (1993) Comment on: Pang D, Dias MS. Cervical myelomeningoceles. Neurosurgery 33:372–373CrossRefGoogle Scholar
  42. 42.
    Tortori-Donati P, Cama A, Rosa ML et al (1990) Occult spinal dysraphism: neuroradiological study. Neuroradiology 31:512–522PubMedCrossRefGoogle Scholar
  43. 43.
    Raghavan N, Barkovich AJ, Edwards M, Norman D (1989) MR imaging in the tethered spinal cord syndrome. AJNR 10:27–36Google Scholar
  44. 44.
    Altman NR, Altman DH (1987) MR imaging of spinal dysraphism. AJNR 8:533–538PubMedGoogle Scholar
  45. 45.
    Uchino A, Mori T, Ohno M (1991) Thick ened fatty filum terminale: MR imaging. Neuroradiology 33:331–333PubMedCrossRefGoogle Scholar
  46. 46.
    Kernohan JW (1924) The ventriculus ter minalis: its growth and development. J Comp Neurol 38:10–125CrossRefGoogle Scholar
  47. 47.
    Coleman IT, Zimmerman RA, Rorke LB (1995) Ventriculus terminalis of the conus medullaris: MR findings in children. AJNR 16:1421–1426PubMedGoogle Scholar
  48. 48.
    Dias MS, Walker ML (1992) The embry ogenesis of complex dysraphic malforma tions: a disorder of gastrulation? Pediatr Neurosurg 18:229–253PubMedCrossRefGoogle Scholar
  49. 49.
    Prop N, Frensdorf EL (1967) A post vertebral endodermal cyst associated with axial deformities: a case showing the “en dodermal-ectodermal adhesion syndrome ”. Pediatrics 39:555–562PubMedGoogle Scholar
  50. 50.
    Faris JC, Crowe JE (1975) The split no tochord syndrome. J Pediatr Surg 10:467–472PubMedCrossRefGoogle Scholar
  51. 51.
    Hoffman CH, Dietrich RB, Pais MJ et al (1993) The split notochord syndrome with dorsal enteric fistula. AJNR 14:622–627PubMedGoogle Scholar
  52. 52.
    Kincaid PK, Stanley P, Kovanlikaya A et al (1999) Coex istent neurenteric cyst and enterogenous cyst. Further support for a common em bryologic error. Pediatr Radiol 29:539–541PubMedCrossRefGoogle Scholar
  53. 53.
    Burger PC, Scheithauer BW, Vogel FS (2002) Surgical pathology of the nervous system and its coverings. Churchill Livingstone, New YorkGoogle Scholar
  54. 54.
    Brooks BS, Duvall ER, El Gammal T et al (1993) Neuroimaging features of neurenteric cysts: analysis of nine cases and review of the literature. AJNR 14:735–746PubMedGoogle Scholar
  55. 55.
    Ciao P, Osborn AG, Smirniotopoulos JG et al (1995) Neurenteric cysts. Pathology, imaging spectrum, and differential diagnosis. Int J Neuroradiol 1:17–27Google Scholar
  56. 56.
    Schlesinger AE, Naidich TP, Quencer RM (1986) Concurrent hydromyelia and diastematomyelia. AJNR 7:473–477PubMedGoogle Scholar
  57. 57.
    Tortori-Donati P, Fondelli MP, Rossi A (1998) Anomalie congenite del midollo spinale. In: Simonetti G, Del Maschio A, Bartolozzi C, Passariello R (eds) Trattato italiano di risonanza magnetica. Idelson Gnocchi, Naples, pp 517–553Google Scholar
  58. 58.
    Ersahin Y, Mutluer S, Kocaman S et al (1998) Split spinal cord mal formations in children. J Neurosurg 88:57–65PubMedGoogle Scholar
  59. 59.
    Barkovich AJ, Edwards MSB, Cogen PH (1991) MR evaluation of spinal dermal si nus tracts in children. AJNR 12:123–129PubMedGoogle Scholar
  60. 60.
    Duhamel B (1961) From the mermaid to anal imperforation: the syndrome of cau dal regression. Arch Dis Child 36:152–155CrossRefGoogle Scholar
  61. 61.
    Tortori-Donati V, Fondelli MP, Rossi A et al (1999) Segmental spinal dysgenesis. Neuro radiologic findings with clinical and em bryologic correlation. AJNR 20:445–456PubMedGoogle Scholar
  62. 62.
    Valenzano M, Paoletti R, Rossi A et al (1999) Sirenomelia. Pathological features, antenatal ultrasonographic clues, and a review of current embryogenic theories. Hum Reprod Update 5:82–86PubMedCrossRefGoogle Scholar
  63. 63.
    Raffel C, Litofsky S, McComb JG (1991) Central nervous system malformations and the VATER association. Pediatr Neu rosurg 16:174–173Google Scholar
  64. 64.
    Currarino G, Coln D, Votteler T (1981) Triad of anorectal, sacral, and presacral anomalies. AIR 137:395–398Google Scholar
  65. 65.
    Dias MS, Azizkhan RG (1998) A novel embryogenetic mechanism for Currarino’s triad: inadequate dorsoventral separation of the caudal eminence from hindgut en doderm. Pediatr Neurosurg 28:223–229PubMedCrossRefGoogle Scholar
  66. 66.
    Gudinchet F, Maeder I, Laurent T et al (1997) Magnetic resonance detection of myelodysplasia in children with Currarino triad. Pediatr Radiol 27:903–907PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Italia 2008

Authors and Affiliations

  • Aydin Sav
    • 1
  1. 1.Department of Pathology and Division of NeuropathologyMarmara University Medical CenterIstanbulTurkey

Personalised recommendations