Skip to main content

Advertisement

SpringerLink
Log in

Vascular Malformations of the Spine and Spinal Cord*

Anatomy, Classification, Treatment

Spinale vaskuläre Malformationen. Anatomie, Klassifikation, Therapie

  • Review Article
  • Published:
Clinical Neuroradiology Aims and scope Submit manuscript

Abstract

Spinal vascular malformations are rare diseases with a wide variety of neurologic presentations. Their classification depends on the differentiation of shunting versus nonshunting lesions, the latter being the spinal cord cavernomas. In the shunting lesions, the next step in the proposed classification scheme is related to the feeding artery which can subdivide the dural vascular shunts from the pial vascular malformations: while those shunts that are fed by radiculomeningeal arteries (i.e., the counterparts of meningeal arteries in the brain) constitute the dural arteriovenous fistulas, the shunts that are fed by arteries that would normally supply the spinal cord (i.e., the radiculomedullary and radiculopial arteries) are the pial cord arteriovenous malformations (AVMs; whose cranial counterparts are the brain AVMs). Depending on the type of transition between artery and vein, the latter pial AVMs can be further subdivided into glomerular (plexiform or nidus-type) AVMs with a network of intervening vessels in between the artery and vein and the fistulous pial AVMs. The last step in the classification then describes whether the type of fistula has a high or a low shunting volume which will differentiate the “macro-” from the “micro”fistulas. The proposed classification is therefore based on a stepwise analysis of the shunt including its arterial anatomy, its nidus architecture and its flow-volume evaluation. The major advantage of this approach is that it leads to a subclassification with direct implications on the choice of treatment, thereby constituting a simple and practical approach to evaluate these rare diseases.

Zusammenfassung

Spinale Gefäßmalformationen sind seltene und vor der Einführung der Magnetresonanztomographie noch deutlich seltener diagnostizierte Erkrankungen, die unbehandelt zu einer meist progredienten Schädigung des Rückenmarks durch Veränderung der spinalen Zirkulation führen. In Abhängigkeit vom Typ der vaskulären Malformation kann diese Schädigung durch Blutungen (subarachnoidal oder intramedullär), venöse Stauung mit Kongestionsmyelopathie, Durchblutungsstörungen oder Raumforderungseffekte hervorgerufen werden. Auch ein „Steal“-Phänomen wird diskutiert. Während symptomatische vaskuläre Malformationen immer behandlungsbedürftig sind, ist die Behandlung inzidenteller spinaler vaskulärer Malformationen aufgrund ihres unklaren natürlichen Verlaufs weniger gesichert. Die Therapie spinaler vaskulärer Malformationen hängt im Wesentlichen von der Angioarchitektur ab, so dass diese der im vorliegenden Übersichtsartikel benutzten Klassifikation zugrunde gelegt wird. Prinzipiell unterscheidet sich die Einteilung der spinalen Gefäßmalformationen nicht von der Einteilung vaskulärer Malformationen des Gehirns. Während durale arteriovenöse Shunts von Gefäßen gespeist werden, die normalerweise die Dura versorgen (radikulomeningeale Gefäße), werden die pialen (oder intraduralen) arteriovenösen Shunts von Gefäßen gespeist, die normalerweise das Rückenmark versorgen. Die vaskulären Malformationen des Rückenmarks, die nicht mit einem arteriovenösen Kurzschluss einhergehen, sind die spinalen Kavernome.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Thron A. Vascular anatomy of the spinal cord: neuroradiological investigations and clinical syndromes. Berlin-New York: Springer, 1988.

    Book  Google Scholar 

  2. Zawilinski J, Litwin JA, Nowogrodzka-Zagorska M, Gorczyca J, Miodonski AJ. Vascular system of the human spinal cord in the prenatal period: a dye injection and corrosion casting study. Ann Anat 2001;183:331–40.

    Article  CAS  PubMed  Google Scholar 

  3. Lasjaunias PL, Berenstein A, terBrugge K. Surgical neuroangiography, vol 1. Clinical vascular anatomy and variations. Berlin: Springer, 2001.

    Book  Google Scholar 

  4. Krings T, Geibprasert S, Thron A. Spinal vascular anatomy. In: Naidich T, ed. Neuroradiology of the brain and spine. New York: Elsevier, 2010:in press.

    Google Scholar 

  5. Tadie M, Hemet J, Freger P, Clavier E, Creissard P. Morphological and functional anatomy of spinal cord veins. J Neuroradiol 1985;12:3–20.

    CAS  PubMed  Google Scholar 

  6. Krings T, Mull M, Bostroem A, Otto J, Hans FJ, Thron A. Spinal epidural AV fistula with perimedullary drainage. Case report and pathomechanical considerations. J Neurosurg Spine 2006;5:353–8.

    Article  PubMed  Google Scholar 

  7. Tadie M, Hemet J, Aaron C, Bianco C, Creissard P, Huard P. Le dispositif protecteur anti-reflux des veines de la moelle. Neurochirurgie 1979;25:28–30.

    Google Scholar 

  8. Otto J. Morphologie des Sperrmechanismus am Duradurchtritt der Venae radiculares des Menschen. Neuroradiologische und histologische Befunde. Dissertation, Medizinische Fakultät, Universit:at Aachen. 1990.

  9. Thron A, Otto J, Schroeder JM. Functional anatomy of the dural segment of spinal cord drainig veins. A histological and microangiographic study. In: du Boulay G, ed. Symposium Neuroradiologicum. London: Springer, 1990:323.

    Google Scholar 

  10. Krings T, Mull M, Gilsbach JM, Thron A. Spinal vascular malformations. Eur Radiol 2005;15:267–78.

    Article  PubMed  Google Scholar 

  11. Spetzler RF, Detwiler PW, Rinna HA, Porter RW. Modified classification of spinal cord vascular lesions. J Neurosurg 2002;96:145–62.

    PubMed  Google Scholar 

  12. Kendall BE, Logue V. Spinal epidural angiomatous malformations draining into intrathecal veins. Neuroradiology 1977;13:181–9.

    Article  CAS  PubMed  Google Scholar 

  13. Rodesch G, Hurth M, Alvarez H, Tadie M, Lasjaunias PL. Classification of spinal cord arteriovenous shunts: proposal for a reappraisal — the Bicêtre experience with 155 consecutive patients treated between 1981 and 1999. Neurosurgery 2002;51:374–9.

    PubMed  Google Scholar 

  14. Rosenblum B, Oldfield EH, Doppman JL, Di Chiro G. Spinal arteriovenous malformations: a comparison of dural arteriovenous fistulas and intradural AVM’s in 81 patients. J Neurosurg 1987;67:795–802.

    Article  CAS  PubMed  Google Scholar 

  15. Lasjaunias P. Spinal cord vascular lesions. J Neurosurg 2003;98:117–9, author reply 119-20.

    Article  PubMed  Google Scholar 

  16. Rodesch G, Hurth M, Alvarez H, Ducot B, Tadie M, Lasjaunias PL. Angio-architecture of spinal cord arteriovenous shunts at presentation. Clinical correlations in adults and children. The Bicêtre experience on 155 consecutive patients seen between 1981–1999. Acta Neurochir (Wien) 2004;146:217–26.

    Article  CAS  Google Scholar 

  17. Reinges MH, Thron A, Mull M, Huffmann BC, Gilsbach JM. Dural arteriovenous fistulae at the foramen magnum. J Neurol 2001;248:197–203.

    Article  CAS  PubMed  Google Scholar 

  18. Geibprasert S, Pereira V, Krings T, Jiarakongmun P, Toulgoat F, Pongpech S, Lasjaunias P. Dural arteriovenous shunts: a new classification of craniospinal epidural venous anatomical bases and clinical correlations. Stroke 2008;39:2783–94.

    Article  PubMed  Google Scholar 

  19. Hurst RW, Kenyon LC, Lavi E, Raps EC, Marcotte P. Spinal dural arteriovenous fistula: the pathology of venous hypertensive myelopathy. Neurology 1995;45:1309–13.

    Article  CAS  PubMed  Google Scholar 

  20. Krings T, Geibprasert S. Spinal dural arteriovenous fistulas. AJNR Am J Neuroradiol 2009;30:639–48.

    Article  CAS  PubMed  Google Scholar 

  21. Aghakhani N, Parker F, David P, Lasjaunias P, Tadie M. Curable cause of paraplegia: spinal dural arteriovenous fistulae. Stroke 2008;39:2756–9.

    Article  PubMed  Google Scholar 

  22. Jellema K, Canta LR, Tijssen CC, van Rooij WJ, Koudstaal PJ, van Gijn J. Spinal dural arteriovenous fistulas: clinical features in 80 patients. J Neurol Neurosurg Psychiatry 2003;74:1438–40.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  23. Krings T, Lasjaunias PL, Hans FJ, Mull M, Nijenhuis RJ, Alvarez H, Backes WH, Reinges MH, Rodesch G, Gilsbach JM, Thron AK. Imaging in spinal vascular disease. Neuroimaging Clin N Am 2007;17:57–72.

    Article  PubMed  Google Scholar 

  24. Farb RI, Kim JK, Willinsky RA, Montanera WJ, terBrugge K, Derbyshire JA, van Dijk JM, Wright GA. Spinal dural arteriovenous fistula localization with a technique of first-pass gadolinium-enhanced MR angiography: initial experience. Radiology 2002;222:843–50.

    Article  PubMed  Google Scholar 

  25. Mull M, Nijenhuis RJ, Backes WH, Krings T, Wilmink JT, Thron A. Value and limitations of contrast-enhanced MR angiography in spinal arteriovenous malformations and dural arteriovenous fistulas. AJNR Am J Neuroradiol 2007;28:1249–58.

    Article  CAS  PubMed  Google Scholar 

  26. Nijenhuis RJ, Leiner T, Cornips EM, Wilmink JT, Jacobs MJ, van Engelshoven JM, Backes WH. Spinal cord feeding arteries at MR angiography for thoracoscopic spinal surgery: feasibility study and implications for surgical approach. Radiology 2004;233:541–7.

    Article  PubMed  Google Scholar 

  27. Jellema K, Sluzewski M, van Rooij WJ, Tijssen CC, Beute GN. Embolization of spinal dural arteriovenous fistulas: importance of occlusion of the draining vein. J Neurosurg Spine 2005;2:580–3.

    Article  PubMed  Google Scholar 

  28. Van Dijk JM, TerBrugge KG, Willinsky RA, Farb RI, Wallace MC. Multidisciplinary management of spinal dural arteriovenous fistulas: clinical presentation and long-term follow-up in 49 patients. Stroke 2002;33:1578–83.

    Article  PubMed  Google Scholar 

  29. Huffmann BC, Gilsbach JM, Thron A. Spinal dural arteriovenous fistulas: a plea for neurosurgical treatment. Acta Neurochir (Wien) 1995; 135:44–51.

    Article  CAS  Google Scholar 

  30. Nichols DA, Rufenacht DA, Jack CR Jr, Forbes GS. Embolization of spinal dural arteriovenous fistula with polyvinyl alcohol particles: experience in 14 patients. AJNR Am J Neuroradiol 1992;13:933–40.

    CAS  PubMed  Google Scholar 

  31. Niimi Y, Berenstein A, Setton A, Neophytides A. Embolization of spinal dural arteriovenous fistulae: results and follow-up. Neurosurgery 1997;40:675–82, discussion 682-3.

    Article  CAS  PubMed  Google Scholar 

  32. Steinmetz MP, Chow MM, Krishnaney AA, Andrews-Hinders D, Benzel EC, Masaryk TJ, Mayberg MR, Rasmussen PA. Outcome after the treatment of spinal dural arteriovenous fistulae: a contemporary single-institution series and meta-analysis. Neurosurgery 2004;55:77–87, discussion 87-8.

    Article  PubMed  Google Scholar 

  33. Andres RH, Barth A, Guzman R, Remonda L, El-Koussy M, Seiler RW, Widmer HR, Schroth G. Endovascular and surgical treatment of spinal dural arteriovenous fistulas. Neuroradiology 2008;50:869–76.

    Article  PubMed  Google Scholar 

  34. Sherif C, Gruber A, Bavinzski G, Standhardt H, Widhalm G, Gibson D, Richling B, Knosp E. Long-term outcome of a multidisciplinary concept of spinal dural arteriovenous fistulae treatment. Neuroradiology 2008;50:67–74.

    Article  PubMed  Google Scholar 

  35. Ozanne A, Krings T, Facon D, Fillard P, Dumas JL, Alvarez H, Ducreux D, Lasjaunias P. MR diffusion tensor imaging and fiber tracking in spinal cord arteriovenous malformations: a preliminary study. AJNR Am J Neuroradiol 2007;28:1271–9.

    Article  CAS  PubMed  Google Scholar 

  36. Thron A, Caplan LR. Vascular malformations and interventional neuroradiology of the spinal cord. In: Brandt T, Caplan LR, Dichgans J, Diener HC, Kennard C, eds. Neurological disorders course and treatment, 2nd edn. Amsterdam-Boston-London: Academic Press; 2003;517–28.

    Chapter  Google Scholar 

  37. Krings T, Thron AK, Geibprasert S, Agid R, Hans FJ, Lasjaunias PL, Reinges MH. Endovascular management of spinal vascular malformations. Neurosurg Rev:in press (Epub 2009 May 5).

  38. Bostrom A, Krings T, Hans FJ, Schramm J, Thron AK, Gilsbach JM. Spinal glomus-type arteriovenous malformations: microsurgical treatment in 20 cases. J Neurosurg Spine 2009;10:423–9.

    Article  PubMed  Google Scholar 

  39. Reul J. Spinal dural arterio-venous fistulas: diagnosis and treatment from the neuroradiological point of view. Klin Neuroradiol 2005;15:91–8.

    Article  Google Scholar 

  40. Kataoka H, Miyamoto S, Nagata I, Ueba T, Hashimoto N. Venous congestion is a major cause of neurological deterioration in spinal arteriovenous malformations. Neurosurgery 2001;48:1224–9, discussion 1229-30.

    CAS  PubMed  Google Scholar 

  41. Rodesch G, Hurth M, Alvarez H, David P, Tadie M, Lasjaunias PL. Embolization of spinal cord arteriovenous shunts: morphological and clinical follow-up and results — review of 69 consecutive cases. Neurosurgery 2003;53:40–9.

    Article  PubMed  Google Scholar 

  42. Prestigiacomo CJ, Niimi Y, Setton A, Berenstein A. Three-dimensional rotational spinal angiography in the evaluation and treatment of vascular malformations. AJRN Am J Neuroradiol 2003;24:1429–35.

    Google Scholar 

  43. Mourier KL, Gobin YP, George B, Lot G, Merland JJ. Intradural perimedullary arteriovenous fistulae: results of surgical and endovascular treatment in a series of 35 cases. Neurosurgery 1993;32:885–91, discussion 891.

    Article  CAS  PubMed  Google Scholar 

  44. Huffmann BC, Spetzger U, Reinges M, Bertalanffy H, Thron A, Gilsbach JM. Treatment strategies and results in spinal vascular malformations. Neurol Med Chir (Tokyo) 1998;38:Suppl:231–7.

    Article  Google Scholar 

  45. Rodesch G, Hurth M, Alvarez H, Tadie M, Lasjaunias PL. Spinal cord intradural arteriovenous fistulae: anatomic, clinical, and therapeutic considerations in a series of 32 consecutive patients seen between 1981 and 2000 with emphasis on endovascular therapy. Neurosurgery 2005;57:973–83.

    Article  PubMed  Google Scholar 

  46. Niimi Y, Berenstein A. Endovascular treatment of spinal vascular malformations. Neurosurg Clin N Am 1999;10:47–71.

    CAS  PubMed  Google Scholar 

  47. Théron J, Cosgrove R, Melanson D, Ethier R. Spinal arteriovenous malformations: advances in therapeutic embolization. Radiology 1986;158:163–9.

    PubMed  Google Scholar 

  48. Cullen S, Krings T, Ozanne A, Alvarez H, Rodesch G, Lasjaunias P. Diagnosis and endovascular treatment of pediatric spinal arteriovenous shunts. Neuroimaging Clin N Am 2007;17:207–21.

    Article  CAS  PubMed  Google Scholar 

  49. Krings T, Ozanne A, Chng SM, Alvarez H, Rodesch G, Lasjaunias PL. Neurovascular phenotypes in hereditary haemorrhagic telangiectasia patients according to age. Review of 50 consecutive patients aged 1 day-60 years. Neuroradiology 2005;47:711–20.

    Article  CAS  PubMed  Google Scholar 

  50. Krings T, Chng SM, Ozanne A, Alvarez H, Rodesch G, Lasjaunias PL. Hereditary hemorrhagic telangiectasia in children: endovascular treatment of neurovascular malformations: results in 31 patients. Neuroradiology 2005;47:946–54.

    Article  CAS  PubMed  Google Scholar 

  51. Krings T, Geibprasert S, Luo CB, Bhattacharya JJ, Alvarez H, Lasjaunias P. Segmental neurovascular syndromes in children. Neuroimaging Clin N Am 2007;17:245–58.

    Article  CAS  PubMed  Google Scholar 

  52. Spetzler RF, Zabramski JM, Flom RA. Management of juvenile spinal AVM’s by embolization and operative excision. Case report. J Neurosurg 1989;70:628–32.

    Article  CAS  PubMed  Google Scholar 

  53. Weinzierl MR, Krings T, Korinth MC, Reinges MH, Gilsbach JM. MRI and intraoperative findings in cavernous haemangiomas of the spinal cord. Neuroradiology 2004;46:65–71.

    Article  CAS  PubMed  Google Scholar 

  54. Zevgaridis D, Medele RJ, Hamburger C, Steiger HJ, Reulen HJ. Cavernous haemangiomas of the spinal cord. A review of 117 cases. Acta Neurochir (Wien) 1999;141:237–45.

    Article  CAS  Google Scholar 

  55. Zentner J, Hassler W, Gawehn J, Schroth G. Intramedullary cavernous angiomas. Surg Neurol 1989;31:64–8.

    Article  CAS  PubMed  Google Scholar 

  56. Spetzger U, Gilsbach JM, Bertalanffy H. Cavernous angiomas of the spinal cord clinical presentation, surgical strategy, and postoperative results. Acta Neurochir (Wien) 1995;134:200–6.

    Article  CAS  Google Scholar 

  57. Cosgrove GR, Bertrand G, Fontaine S, Robitaille Y, Melanson D. Cavernous angiomas of the spinal cord. J Neurosurg 1988;68:31–6.

    Article  CAS  PubMed  Google Scholar 

  58. Bostroem A, Thron A, Hans FJ, Krings T. Spinal vascular malformations — typical and atypical findings. Zentralbl Neurochir 2007;68:205–13.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Neuroradiology, Department of Medical Imaging, University of Toronto, Toronto Western Hospital, Toronto, ON, Canada

    Timo Krings FRCP(C)

  2. Division of Neuroradiology, Toronto Western Hospital, UHN, 399 Bathurst St., 3MCL – 429, Toronto, ON, M5T 2S8, Canada

    Timo Krings FRCP(C)

Authors
  1. Timo Krings FRCP(C)
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Timo Krings FRCP(C).

Additional information

*Dedicated to Professor Armin Thron, MD, on the occasion of his 65th birthday.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Krings, T. Vascular Malformations of the Spine and Spinal Cord*. Clin Neuroradiol 20, 5–24 (2010). https://doi.org/10.1007/s00062-010-9036-6

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00062-010-9036-6

Key Words

Schlüsselwörter

Search

Navigation