• Hyun-Yoon Ko


Syringomyelia is not a disease in itself, but a manifestation of other pathological processes, including obstruction of cerebrospinal fluid (CSF) circulation in the spinal canal, tethering of the spinal cord, or an intramedullary tumor. Syringomyelia is a disorder in which a cyst develops and expands in the spinal cord. The expanding syrinx insidiously damages the cord and causes paresthesia, pain, weakness, and stiffness of the trunk, shoulders, arms, or legs. Sweating, sexual function, and bladder and bowel control may also be disturbed. The disease is usually caused by a congenital abnormality of the craniocerebral junction, the Chiari I malformation. Syringomyelia can occur in patients after spinal cord injury.


  1. Battal B, Kocaoglu M, Bulakbasi N, et al. Cerebrospinal fluid flow imaging by using phase-contrast MR technique. Br J Radiol. 2011;84:758–65.CrossRefGoogle Scholar
  2. Barnett HJM, Jousse AT. In: Vinken PJ, Bruyn GW, Braakman R, editors. Injuries of the spine and spinal cord Posttraumatic syringomyelia (cystic myelopathy). Amsterdam: North-Holland Publishing Company; 1976. p. 113–57.Google Scholar
  3. Blegvad C, Grotenhuis JA, Juhler M. Syringomyelia: a practical, clinical concept for classification. Acta Neurochir. 2014;156:2127–38.CrossRefGoogle Scholar
  4. Brodbelt AR, Stoodley MA. Post-traumatic syringomyelia: a review. J Clin Neurosci. 2003;10:401–8.CrossRefGoogle Scholar
  5. de Souza RM, Zador Z, Frim DM. Chiari malformation type I: related conditions. Neurol Res. 2011;33:278–84.CrossRefGoogle Scholar
  6. Dicianno BE, Kurowski BG, Yang JM, et al. Rehabilitation and medical management of the adult with spina bifida. Am J Phys Med Rehabil. 2008;87:1027–50.CrossRefGoogle Scholar
  7. DuBoulay GH, Shah SH, Currie JC, et al. The mechanism of hydromyelia in Chiari type I malformation. Br J Radiol. 1974;47:579–87.CrossRefGoogle Scholar
  8. Eagler GL, Cole J, Merton WL, editors. Spinal cord diseases: diagnosis and treatment. New York: Marcel Dekker Inc.; 1998.Google Scholar
  9. Eisenstat DDR, Bernstein M, Fleming JFR, et al. Chiari malformation in adults: a review of 40 cases. Can J Neurol Sci. 1989;13:221–8.CrossRefGoogle Scholar
  10. Falci SP, Indeck C, Lammertse DP. Posttraumatic spinal cord tethering and syringomyelia: surgical treatment and long-term outcome. J Neurosurg Spine. 2009;11:445–60.CrossRefGoogle Scholar
  11. Fujimoto S, Mizuno R, Saito Y, et al. Clinical application of wave intensity for the treatment of essential hypertension. Heart Vessel. 2004;19:19–22.CrossRefGoogle Scholar
  12. Griffiths ER, McCormick CC. Post-traumatic syringomyelia (cystic myelopathy). Paraplegia. 1981;19:81–9.PubMedGoogle Scholar
  13. Hilton EL, Henderson LJ. Neurosurgical considerations in posttraumatic syringomyelia. AORN J. 2003;77:135–9, 141–4.CrossRefGoogle Scholar
  14. Hirai T, Korogi Y, Shigematsu Y, et al. Evaluation of syringomyelia with three-dimentional constructive interference in a steady state (CISS) sequence. J Magn Reson Imaging. 2000;11:120–6.CrossRefGoogle Scholar
  15. Klekamp J. Surgical treatment of Chiari I malformation-analysis of intraoperative findings, complications, and outcome for 371 foramen magnum decompressions. Neurosurgery. 2012a;71:365–80; discussion 380CrossRefGoogle Scholar
  16. Klekamp J. Treatment of posttraumatic syringomyelia. J Neurosurg Spine. 2012b;17:199–211.CrossRefGoogle Scholar
  17. Klekamp J. Treatment of syringomyelia related to non-traumatic arachnoid pathologies of the spinal canal. Neurosurgery. 2012c;72:376–89.CrossRefGoogle Scholar
  18. Ko HY, Kim W, Kim SY, et al. Factors associated with early onset post-traumatic syringomyelia. Spinal Cord. 2012;50:695–8.CrossRefGoogle Scholar
  19. Kramer KM, Levine AM. Posttraumatic syringomyelia: a review of 21 cases. Clin Orthop Relat Res. 1997;334:190–9.CrossRefGoogle Scholar
  20. Levy WJ, Mason L, Hahn JF. Chiari malformation presenting in adults: a surgical experience in 127 cases. Neurosurgery. 1983;12:377–90.CrossRefGoogle Scholar
  21. Liptak GS, Dosa NP. Myelomeningocele. Pediatr Rev. 2010;31:443–50.CrossRefGoogle Scholar
  22. Madsen PW 3rd, Yezierski RP, Holets VR. Syringomyelia: clinical observations and experimental studies. J Neurotrauma. 1994;11:241–54.CrossRefGoogle Scholar
  23. McVige JW, Leonardo J. Imaging of Chiari type I malformation and syringohydromyelia. Neurol Clin. 2014;32:95–126.CrossRefGoogle Scholar
  24. Milhorat TH, Kotzen RM, Anzil AP. Stenosis of the central canal of spinal cord in main: incidence and pathological findings in 232 autopsy series. J Neurosurg. 1994;80:716–22.CrossRefGoogle Scholar
  25. Rigamonti DD, Wrathall JR, Kao CC. Lysosomal autolysis of contused spinal cords. Prog Soc Neurosci. 1978;4:570.Google Scholar
  26. Roy AK, Slimack NP, Ganju A. Idiopathic syringomyelia: retrospective case series, comprehensive review, and update on management. Neurosurg Focus. 2011;31:E15.CrossRefGoogle Scholar
  27. Rufener S, Ibrahim M, Parmar HA. Imaging of congenital spine and spinal cord malformations. Neuroimaging Clin N Am. 2011;21:659–76.CrossRefGoogle Scholar
  28. Sekula RF Jr, Arnone GD, Crocker C, et al. The pathogenesis of Chiari I malformation and syringomyelia. Neurol Res. 2011;33:232–9.CrossRefGoogle Scholar
  29. Shields CB, Zhang YP, Shields LB. Post-traumatic syringomyelia: CSF hydrodynamic changes following spinal cord injury are the driving force in the development of PTSM. Handb Clin Neurol. 2012;109:355–67.CrossRefGoogle Scholar
  30. Umbach I, Heilporn A. Review article: post-spinal cord injury syringomyelia. Paraplegia. 1991;29:219–121.PubMedGoogle Scholar
  31. Vannemreddy SS, Rowed DW, Bharatwal N. Posttraumatic syringomyelia: predisposing factors. Br J Neurochir (Wien). 2002;55:295–302.Google Scholar
  32. Williams B. On the pathogenesis of syringomyelia: a review. J Royal Soc Med. 1980;73:798–806.CrossRefGoogle Scholar
  33. Williams B, Terry AF, Jones HWF, et al. Syringomyelia as a sequel to traumatic paraplegia. Paraplegia. 1981;19:67–80.PubMedGoogle Scholar
  34. Yoshimura K, Sasaki M, Morris S, et al. Posttraumatic syringomyelia developing soon after spinal cord injury. A case report. No Shinkei Geka. 2008;36:895–900.PubMedGoogle Scholar

Suggested Reading

  1. Altman J, Bayer SA. Development of the human spinal cord: an interpretation based on experimental studies. 1st. New York: Oxford University Press; 2001.Google Scholar
  2. American Spinal Injury Association. International standards for neurological classification of Spinal Cord injury. Revised 2011. Updated 2015th ed. Atlanta: American Spinal Injury Association; 2015.Google Scholar
  3. Byrne TN, Benzel EC, Waxman SG. Diseases of the spine and spinal cord. Oxford: Oxford University Press; 2000.Google Scholar
  4. Cohen-Adad J, Wheeler-Kingshott CAM, editors. Quantitative MRI of the spinal cord. New York: Elsevier; 2014.Google Scholar
  5. Crossman A, Neary D. Neuroanatomy: an illustrated colour test. 5th ed. Philadelphia: Elsevier; 2015.Google Scholar
  6. Durrant DH, True JM. Myelopathy, radiculopathy, and peripheral entrapment syndromes. Boca Raton: CRC Press; 2002.Google Scholar
  7. Eagler GL, Cole J, Merton WL, editors. Spinal cord diseases: diagnosis and treatment. New York: Marcel Dekker Inc.; 1998.Google Scholar
  8. Flint G, Rusbridge C, editors. Syringomyelia, a disorder of CSF circulation. London: Springer; 2014.Google Scholar
  9. Fulton JF, Keller AD. The sign of Babinski: a study of the evolution of cortical dominance in primates. Springfield: Charles C Thomas; 1932.Google Scholar
  10. Guttmann L. Spinal cord injuries. Comprehensive management and research. Oxford: Blackwell Scientific Publications; 1976.Google Scholar
  11. Hancox RJ, Whyte KF. Pocket guide to lung function tests. New York: The McGraw-Hill Companies, Inc; 2001.Google Scholar
  12. Harvey L. Management of spinal cord injuries. A guide for physiotherapists. Philadelphia: Churchill Livingstone; 2008.Google Scholar
  13. Hattingen E, Klein JC, Weidauer S, Vrionis F, Setzer M, editors. Diseases of the spinal cord. Heidelberg: Springer; 2015.Google Scholar
  14. Jallo J, Vaccaro AR, editors. Neurotrauma and critical care of the spine. 2nd ed. New York: Thieme; 2018.Google Scholar
  15. Kirshblum S, Campagnolo DI, editors. Spinal cord medicine. 2nd ed. Philadelphia: Wolters Kluwer, Lippincott, Williams & Wilkins; 2011.Google Scholar
  16. Lin VW, editor. Spinal cord medicine. principles and practice. 2nd ed. New York: Demos Medical; 2010.Google Scholar
  17. Mai JK, Paxinos G, editors. The human nervous system. 3rd ed. London: Elsevier; 2011.Google Scholar
  18. Mancall E. Gray’s clinical neuroanatomy: the anatomic basis for clinical neuroscience. Philadelphia: Elsevier; 2011.Google Scholar
  19. Mtuid E, Gruener G, Dockery P. Fitzgerald’s clinical neuroanatomy and neuroscience. 7th ed. Philadelphia: Elsevier; 2016.Google Scholar
  20. Neuburger M. The historical development of experimental brain and spinal cord physiology before Flourens. Baltimore: The Johns Hopkins University Press; 1981.Google Scholar
  21. Noback CR, Strominger NL, Demarest RJ, Ruggiero DA. The human nervous system: structure and function. 6th ed. Totoma: Humana Press; 2005.Google Scholar
  22. Verhaagen J, McDonald JW III. Spinal cord injury. In: Aminoff MJ, Boller F, Swaab DF, editors. Handbook of clinical neurology, 3rd series, vol. 109. London: Elsevier; 2012.Google Scholar
  23. Vinken PJ, Bruyn GW, editors. Injuries of the spine and spinal cord. Part I. Handbook of clinical neurology, vol. 25. Oxford: North-Holland Publishing Company; 1976.Google Scholar
  24. Vinken PJ, Bruyn GW, editors. Injuries of the spine and spinal cord. Part II. Handbook of clinical neurology, vol. 25. Oxford: North-Holland Publishing Company; 1976.Google Scholar
  25. Vodusek DB, Boller F. Neurology of sexual and bladder disorders. In: Aminoff MJ, Boller F, Swaab DF, editors. Handbook of clinical neurology, 3rd series, vol. 130. London: Elsevier; 2015.Google Scholar
  26. Vogel LC, Zebracki K, Betz RR, Mulcahey MJ, editors. Spinal cord injury in the child and young adult. London: Mac Keith Press; 2014.Google Scholar
  27. Weaver LC, Polosa C, editors. Autonomic dysfunction after spinal cord injury. In: progress in brain research, vol. 152. New York: Elsevier; 2006.Google Scholar
  28. Weidner N, Rupp R, Taney KE, editors. Neurological aspects of spinal cord injury. Cham: Springer; 2017.Google Scholar
  29. Wilberger JE, editor. Spinal cord injuries in children. New York: Futura Publishing Company; 1986.Google Scholar

Copyright information

© Springer Nature Singapore Pte Ltd. 2019

Authors and Affiliations

  • Hyun-Yoon Ko
    • 1
  1. 1.Department of Rehabilitation MedicineRehabilitation Hospital, Pusan National University Yangsan Hospital, Pusan National University School of MedicineYangsanSouth Korea

Personalised recommendations