Advertisement

Pathology of Syringomyelia Due to Chiari Malformations

Chapter

Abstract

Since the early nineteenth century, some authors have used the term syringomyelia to describe any tubular cavity within the spinal cord. More recently, classification systems have evolved to describe syringomyelia subtypes as well as distinguish other entities such as hydromyelia, syringobulbia, and myelomalacia. Our current understanding of the etiology of syringomyelia, although more advanced, is still incomplete. Syringomyelia occurs in many different clinical settings with variable clinical symptomatology and different cavitary patterns. Anatomic and pathologic criteria are helpful in establishing basic classification of syringes as well as ­understanding syringomyelia in the setting of Chiari malformation. The pathophysiological hypotheses for syringomyelia are numerous, controversial, and variable according to the proximate cause. The gross pathology and histology are similar irrespective of cause, and competing theories continue to evolve on the pathogenesis of syrinx formation.

Keywords

Subarachnoid Space Fourth Ventricle Central Canal Chiari Malformation Chiari Type 
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.

References

  1. 1.
    Bielschowsky M, Unger E. Syringomyelie mit Teratom-und extramedulla¨ rer Blastom-bildung. J Psychol Neurol (Lpz). 1920;25:173–218.Google Scholar
  2. 2.
    Chang HS, Nakagawa H. Hypothesis on the pathophysiology of syringomyelia based on simulation of cerebrospinal fluid dynamics. J Neurol Neurosurg Psychiatry. 2003;74:344–7.PubMedCrossRefGoogle Scholar
  3. 3.
    Chang HS, Nakagawa H. Theoretical analysis of the pathophysiology of syringomyelia associated with adhesive arachnoiditis. J Neurol Neurosurg Psychiatry. 2004;75:758–64.CrossRefGoogle Scholar
  4. 4.
    Chern JJ, Gordon AJ, Mortazavi MM, Tubbs RS, Oakes JW. Pediatric Chiari malformation type 0: a 12-year institutional experience. J Neurosurg Pediatr. 2011;8:1–5.PubMedCrossRefGoogle Scholar
  5. 5.
    Ellertsson AB. Syringomyelia and other cystic spinal cord lesions. Acta Neurol Scand. 1969;45:403–17.PubMedCrossRefGoogle Scholar
  6. 6.
    Ellertsson AB, Greitz T. The distending force in the production of communicating syringomyelia. Lancet. 1970;1:1234.PubMedCrossRefGoogle Scholar
  7. 7.
    Feigin I, Ogata J, Budzilovitch G. Syringomyelia: the role of edema in its pathogenesis. J Neuropathol Exp Neurol. 1971;30:216–32.PubMedCrossRefGoogle Scholar
  8. 8.
    Foster JB, Hudgson P. The pathology of communicating syringomyelia. In: Barnett HJM, Foster JB, Hudgson P, editors. Syringomyelia. Philadelphia: WB Saunders; 1973. p. 79–103.Google Scholar
  9. 9.
    Gardner WJ, Goodall RJ. The surgical treatment of Arnold-Chiari malformation in adults: an explanation of its mechanism and importance of encephalography in diagnosis. J Neurosurg. 1950;7:199–206.PubMedCrossRefGoogle Scholar
  10. 10.
    Gardner WJ, Angel J. The cause of syringomyelia and its surgical treatment. Cleve Clin Q. 1958;25:4–8.PubMedCrossRefGoogle Scholar
  11. 11.
    Gardner WJ, Angel J. The mechanism of syringomyelia and its surgical correction. Clin Neurosurg. 1959;6:131–40.Google Scholar
  12. 12.
    Gardner WJ. Hydrodynamic mechanism of syringomyelia: its relationship to myelocele. J Neurol Neurosurg Psychiatry. 1965;28:247–59.PubMedCrossRefGoogle Scholar
  13. 13.
    Greitz D, Ericson K, Flodmark O. Pathogenesis and mechanics of spinal cord cysts: a new hypothesis based on magnetic resonance studies of cerebrospinal fluid dynamics. Int J Neuroradiol. 1999;5:61–78.Google Scholar
  14. 14.
    Heiss JD, Patronas N, DeVroom HL, Shawker T, Ennis R, Kammerer W, et al. Elucidating the patho­physiology of syringomyelia. J Neurosurg. 1999;91:553–62.PubMedCrossRefGoogle Scholar
  15. 15.
    Hinokuma K, Ohama E, Oyanagi K, Kakita A, Kawai K, Ikuta F. Syringomyelia: a neuropathological study of 18 autopsy cases. Acta Pathol Jpn. 1992;42:25–34.PubMedGoogle Scholar
  16. 16.
    Klekamp J, Samii M. Syringomyelia: diagnosis and treatment. Berlin: Springer; 2002.CrossRefGoogle Scholar
  17. 17.
    Klekamp J. The pathophysiology of syringomyelia – historical overview and current concept. Acta Neurochir. 2002;144:649–64.PubMedCrossRefGoogle Scholar
  18. 18.
    Levine DN. The pathogenesis of normal pressure hydrocephalus: a theoretical analysis. Bull Math Biol. 1999;61:875–916.PubMedCrossRefGoogle Scholar
  19. 19.
    Levine DL. The pathogenesis of syringomyelia associated with lesions at the foramen magnum: a critical review of existing theories and proposal of a new hypothesis. J Neurol Sci. 2004;220:3–21.PubMedCrossRefGoogle Scholar
  20. 20.
    Levy EI, Heiss JD, Kent MS, Riedel CJ, Oldfield EH. Spinal cord swelling preceding syrinx development. J Neurosurg. 2000;92:93–7 (Spine 1).PubMedGoogle Scholar
  21. 21.
    Liber AF, Lisa JR. Rosenthal fibers in non-neoplastic syringomyelia. J Nerv Ment Dis. 1937;86:549–58.CrossRefGoogle Scholar
  22. 22.
    Milhorat TH, Kotzen RM, Anzil AP. Stenosis of central canal of spinal cord in man: incidence and pathological findings in 232 autopsy cases. J Neurosurg. 1994;80:716–22.PubMedCrossRefGoogle Scholar
  23. 23.
    Milhorat TH, Capocelli Jr AL, Anzil AP, Kotzen RM, Milhorat RH. Pathological basis of spinal cord cavitation in syringomyelia: analysis of 105 autopsy cases. J Neurosurg. 1995;82:802–12.PubMedCrossRefGoogle Scholar
  24. 24.
    Milhorat TH, Capocelli AL, Kotzen RM, et al. Intramedullary pressure in syringomyelia: clinical and pathophysiological correlates of syrinx distension. Neurosurgery. 1997;41:1102–10.PubMedCrossRefGoogle Scholar
  25. 25.
    Milhorat TH. Classification of syringomyelia. Neurosurg Focus. 2000;8(3):1–6.CrossRefGoogle Scholar
  26. 26.
    Moriwaka F, Tashiro K, Tachibana S, Yada K. Epidemiology of syringomyelia in Japan—the nationwide survey. Rinsho Shinkei-gaku-Clin Neurol. 1995;35:1395–7.Google Scholar
  27. 27.
    Netsky MG. Syringomyelia: a clinicopathologic study. Arch Neurol Psychiatry. 1953;70:741–77.CrossRefGoogle Scholar
  28. 28.
    Oldfield EH, Muraszko K, Shawker TH, Patronas NJ. Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. J Neurosurg. 1994;80:3–15.PubMedCrossRefGoogle Scholar
  29. 29.
    Rossier AB, Foo D, Shillito J, Dyro FM. Posttraumatic cervical syringomyelia. Incidence, clinical presentation, electrophysiological studies, syrinx protein and results of conservative and operative treatment. Brain. 1985;108:439–61.PubMedCrossRefGoogle Scholar
  30. 30.
    Tubbs RS, Elton S, Grabb P, Dockery SE, Bartolucci AA, Oakes WJ. Analysis of the posterior fossa in children with the Chiari 0 malformation. Neurosurgery. 2001;48:1050–5.PubMedCrossRefGoogle Scholar
  31. 31.
    Williams B. The distending force in the production of “communicating syringomyelia”. Lancet. 1969;2:189–93.PubMedCrossRefGoogle Scholar
  32. 32.
    Williams B. Current concepts of syringomyelia. Br J Hosp Med. 1970;4:331–42.Google Scholar
  33. 33.
    Williams B. On the pathogenesis of the Chiari malformation. Z Kinderchir Grenzgeb. 1977;22:533–53.Google Scholar
  34. 34.
    Williams B. Simultaneous cerebral and spinal fluid pressure recordings: 2. Cerebrospinal dissociation with lesions at the foramen magnum. Acta Neurochir. 1981;59:123–42.PubMedCrossRefGoogle Scholar
  35. 35.
    Williams B. Pathogenesis of syringomyelia. In: Batzdorf U, editor. Syringomyelia: current concepts in diagnosis and treatment. Baltimore: Williams & Wilkins; 1991. p. 59–90.Google Scholar
  36. 36.
    Harding BN, Copp AJ. Pathology of malformations. In: Love S, Louis DN, Ellison DW, editors. Greenfield’s neuropathology. 8th edn. London: Hodder Arnold; 2008. p. 429–33.Google Scholar

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  1. 1.Department of Pathology and Laboratory MedicineChildren’s of AlabamaBirminghamUSA

Personalised recommendations