Spinal Vascular Malformations and Treatment

Reference work entry


Spinal vascular malformations are rare, but a diverse group of neurovascular pathologies including arteriovenous malformations (AVMs), cavernous malformations, and dural arteriovenous fistulas (dAVFs) that occur due to developmental derangement of the vascular system. Though classified variously, the commonly followed one is anatomic classification based on the morphology of the malformation to which added are cavernomas and spinal vascular tumors. Pathophysiologic mechanisms leading to clinical symptoms include intramedullary or subarachnoid hemorrhages, arterial ischemia, progressive venous congestion resulting in progressive myelopathy, space-occupying nature of the malformation and its venous drainage, and circulatory steal phenomenon. Most spinal vascular malformations come to clinical attention by nonspecific symptomatology that may be acute, subacute, or chronic. The initial clinical diagnosis is challenging and it is based on MRI; however, for better understanding and planning therapeutic strategy spinal angiogram is necessary. Once diagnosed, most spinal vascular malformations need treatment, as the natural history suggests progressive stepwise neurological deterioration. Early diagnosis and timely management in symptomatic patients can result in improvement or stabilization of clinical condition. Treatment should be performed in specialized centers. Except spinal cord cavernomas and spinal vascular tumors, all other vascular disorders are initially managed by endovascular embolization, as it is the least invasive but technically challenging and demanding procedure. For cases where embolization is technically not possible or when they fail embolization treatment, surgical treatment is an available option. In select cases a combined therapy might be sensible. Early diagnosis and prompt treatment are essential to alter the natural course and result in clinical improvement.


Spinal vascular malformation Arteriovenous malformation Dural arteriovenous fistula Perimedullary arteriovenous fistula Cavernoma Juvenile arteriovenous malformation AVM Myelopathy Paraplegia Endovascular embolization Spinal MRI Spinal angiogram Spinal hemorrhage 


  1. 1.
    Thron A (1988) Vascular anatomy of the spinal cord: neuroradiological investigations and clinical syndromes. Springer, BerlinCrossRefGoogle Scholar
  2. 2.
    Lasjaunias PL, Berenstein A, ter Brugge K (2004) Surgical neuroangiography. Clinical vascular anatomy and variations, vol 1, 2nd edn. Springer, BerlinGoogle Scholar
  3. 3.
    Grunwald I, Thron A, Reith W (2001) Spinal angiography: vascular anatomy, technique and indications. Radiologe 41:961–967 [in German]CrossRefPubMedGoogle Scholar
  4. 4.
    Thron A, Otto J, Schroeder JM (1990) Functional anatomy of the dural segment of spinal cord draining veins: a histological and microangiographic study. In: du Boulay G (ed) Symposium neuroradiologicum. Springer, London, p 323Google Scholar
  5. 5.
    Mull M, Thron A (2006) Spinal infarcts. In: von Kummer R, Back T (eds) Magnetic resonance imaging in ischemic stroke. Springer, Berlin, pp 251–267CrossRefGoogle Scholar
  6. 6.
    Borden JA, Wu JK, Shucart WA (1995) A proposed classification for spinal and cranial dural arteriovenous fistulous malformations and implications for treatment. J Neurosurg 82:166–179. doi:10.3171/jns.1995.82.2.0166CrossRefPubMedGoogle Scholar
  7. 7.
    Spetzler RF, Detwiler PW, Riina HA, Porter RW (2002) Modified classification of spinal cord vascular lesions. J Neurosurg 96:145–156PubMedGoogle Scholar
  8. 8.
    Zozulya YP, Slin’ko EI, Al Q II (2006) Spinal arteriovenous malformations: new classification and surgical treatment. Neurosurg Focus 20:E7CrossRefPubMedGoogle Scholar
  9. 9.
    Krings T et al (2007) Imaging in spinal vascular disease. Neuroimaging Clin N Am 17:57–72. doi:10.1016/j.nic.2007.01.001CrossRefPubMedGoogle Scholar
  10. 10.
    Manelfe C, Lazorthes G, Roulleau J (1972) Arteries of the human spinal dura mater. Acta Radiol Diagn 13:829–841CrossRefGoogle Scholar
  11. 11.
    Gilbertson JR, Miller GM, Goldman MS, Marsh WR (1995) Spinal dural arteriovenous fistulas: MR and myelographic findings. AJNR Am J Neuroradiol 16:2049–2057PubMedGoogle Scholar
  12. 12.
    Finsterer J, Bavinzski G, Ungersbock K (2000) Spinal dural arteriovenous fistula associated with syringomyelia. J Neuroradiol 27:211–214PubMedGoogle Scholar
  13. 13.
    Asakuno K, Kim P, Kawamoto T, Ogino M (2002) Dural arteriovenous fistula and progressive conus medullaris syndrome as complications of lumbar discectomy. Case report. J Neurosurg 97:375–379PubMedGoogle Scholar
  14. 14.
    Vankan Y, Demaerel P, Heye S, Van Calenbergh F, van Loon J, Maleux G, Wilms G (2004) Dural arteriovenous fistula as a late complication of upper cervical spine fracture. Case report. J Neurosurg 100:382–384PubMedGoogle Scholar
  15. 15.
    Rosenblum B, Oldfield EH, Doppman JL, Di Chiro G (1987) Spinal arteriovenous malformations: a comparison of dural arteriovenous fistulas and intradural AVM’s in 81 patients. J Neurosurg 67:795–802. doi:10.3171/jns.1987.67.6.0795CrossRefPubMedGoogle Scholar
  16. 16.
    Atkinson JL et al (2001) Clinical and radiographic features of dural arteriovenous fistula, a treatable cause of myelopathy. Mayo Clin Proc 76:1120–1130. doi:10.4065/76.11.1120CrossRefPubMedGoogle Scholar
  17. 17.
    Koch C, Kucinski T, Eckert B, Rother J, Zeumer H (2003) Spinal dural arteriovenous fistula: clinical and radiological findings in 54 patients. Rofo 175:1071–1078. doi:10.1055/s-2003-40925CrossRefPubMedGoogle Scholar
  18. 18.
    Hurst RW, Grossman RI (2000) Peripheral spinal cord hypointensity on T2-weighted MR images: a reliable imaging sign of venous hypertensive myelopathy. AJNR Am J Neuroradiol 21:781–786PubMedGoogle Scholar
  19. 19.
    De Marco JK, Dillon WP, Halback VV, Tsuruda JS (1990) Dural arteriovenous fistulas: evaluation with MR imaging. Radiology 175:193–199. doi:10.1148/radiology.175.1.2315480CrossRefPubMedGoogle Scholar
  20. 20.
    Huffmann BC, Gilsbach JM, Thron A (1995) Spinal dural arteriovenous fistulas: a plea for neurosurgical treatment. Acta Neurochir 135:44–51CrossRefPubMedGoogle Scholar
  21. 21.
    Lee TT, Gromelski EB, Bowen BC, Green BA (1998) Diagnostic and surgical management of spinal dural arteriovenous fistulas. Neurosurgery 43:242–246, discussion 246–247CrossRefPubMedGoogle Scholar
  22. 22.
    Partington MD, Rufenacht DA, Marsh WR, Piepgras DG (1992) Cranial and sacral dural arteriovenous fistulas as a cause of myelopathy. J Neurosurg 76:615–622. doi:10.3171/jns.1992.76.4.0615CrossRefPubMedGoogle Scholar
  23. 23.
    Britz GW, Lazar D, Eskridge J, Winn HR (2004) Accurate intraoperative localization of spinal dural arteriovenous fistulae with embolization coil: technical note. Neurosurgery 55:252–254, discussion 254–255CrossRefPubMedGoogle Scholar
  24. 24.
    Niimi Y, Berenstein A (1999) Endovascular treatment of spinal vascular malformations. Neurosurg Clin N Am 10:47–71PubMedGoogle Scholar
  25. 25.
    Djindjian M, Djindjian R, Hurth M, Rey A, Houdart R (1977) Spinal cord arteriovenous malformations and the Klippel-Trenaunay-Weber syndrome. Surg Neurol 8:229–237PubMedGoogle Scholar
  26. 26.
    Hurst RW, Bagley LJ, Marcotte P, Schut L, Flamm ES (1999) Spinal cord arteriovenous fistulas involving the conus medullaris: presentation, management, and embryologic considerations. Surg Neurol 52:95–99CrossRefPubMedGoogle Scholar
  27. 27.
    Tobin WD, Layton DD (1976) The diagnosis and natural history of spinal cord arteriovenous malformations. Mayo Clin Proc 51:637–646PubMedGoogle Scholar
  28. 28.
    Berenstein A, Lasjaunias P, TerBrugge KG (2004) Surgical neuroangiography: clinical and endovascular treatment aspects in adults, vol 2.2, 2nd edn. Springer-Verlag, Germany, pp 737–872CrossRefGoogle Scholar
  29. 29.
    Kataoka H, Miyamoto S, Nagata I, Ueba T, Hashimoto N (2001) Venous congestion is a major cause of neurological deterioration in spinal arteriovenous malformations. Neurosurgery 48:1224–1229, discussion 1229–1230PubMedGoogle Scholar
  30. 30.
    Doppman JL, Di Chiro G, Dwyer AJ, Frank JL, Oldfield EH (1987) Magnetic resonance imaging of spinal arteriovenous malformations. J Neurosurg 66:830–834. doi:10.3171/jns.1987.66.6.0830CrossRefPubMedGoogle Scholar
  31. 31.
    Thron A, Mull M, Reith W (2001) Spinal arteriovenous malformations. Radiologe 41:949–954CrossRefPubMedGoogle Scholar
  32. 32.
    Rodesch G, Lasjaunias P, Berenstein A (1993) Embolization of arteriovenous malformations of the spinal cord. In: Valavanis A (ed) Interventional neuroradiology. Springer, Berlin, p 135CrossRefGoogle Scholar
  33. 33.
    Connolly ES Jr, Zubay GP, McCormick PC, Stein BM (1998) The posterior approach to a series of glomus (type II) intramedullary spinal cord arteriovenous malformations. Neurosurgery 42:774–785, discussion 785–776CrossRefPubMedGoogle Scholar
  34. 34.
    Sinclair J, Chang SD, Gibbs IC, Adler JR Jr (2006) Multisession CyberKnife radiosurgery for intramedullary spinal cord arteriovenous malformations. Neurosurgery 58:1081–1089. doi:10.1227/01.NEU.0000215891.25153.BA, discussion 1081–1089CrossRefPubMedGoogle Scholar
  35. 35.
    Menku A, Akdemir H, Durak AC, Oktem IS (2005) Successful surgical excision of juvenile-type spinal arteriovenous malformation in two stages following partial embolization. Minim Invasive Neurosurg 48:57–62. doi:10.1055/s-2004-830184CrossRefPubMedGoogle Scholar
  36. 36.
    Spetzler RF, Zabramski JM, Flom RA (1989) Management of juvenile spinal AVM’s by embolization and operative excision. Case report. J Neurosurg 70:628–632. doi:10.3171/jns.1989.70.4.0628CrossRefPubMedGoogle Scholar
  37. 37.
    Mourier KL, Gobin YP, George B, Lot G, Merland JJ (1993) Intradural perimedullary arteriovenous fistulae: results of surgical and endovascular treatment in a series of 35 cases. Neurosurgery 32:885–891, discussion 891CrossRefPubMedGoogle Scholar
  38. 38.
    Halbach VV, Higashida RT, Dowd CF, Fraser KW, Edwards MS, Barnwell SL (1993) Treatment of giant intradural (perimedullary) arteriovenous fistulas. Neurosurgery 33:972–979, discussion 979–980CrossRefPubMedGoogle Scholar
  39. 39.
    Dillon WP, Norman D, Newton TH, Bolla K, Mark A (1989) Intradural spinal cord lesions: Gd-DTPA-enhanced MR imaging. Radiology 170:229–237. doi:10.1148/radiology.170.1.2909101CrossRefPubMedGoogle Scholar
  40. 40.
    Inoue T, Takahashi T, Shimizu H, Matsumoto Y, Takahashi A, Tominaga T (2006) Congestive myelopathy due to cervical perimedullary arteriovenous fistula evaluated by apparent diffusion coefficient values – case report. Neurol Med Chir 46:559–562CrossRefGoogle Scholar
  41. 41.
    Hida K, Iwasaki Y, Goto K, Miyasaka K, Abe H (1999) Results of the surgical treatment of perimedullary arteriovenous fistulas with special reference to embolization. J Neurosurg 90:198–205PubMedGoogle Scholar
  42. 42.
    Deutsch H, Jallo GI, Faktorovich A, Epstein F (2000) Spinal intramedullary cavernoma: clinical presentation and surgical outcome. J Neurosurg 93:65–70PubMedGoogle Scholar
  43. 43.
    Cohen-Gadol AA, Jacob JT, Edwards DA, Krauss WE (2006) Coexistence of intracranial and spinal cavernous malformations: a study of prevalence and natural history. J Neurosurg 104:376–381. doi:10.3171/jns.2006.104.3.376CrossRefPubMedGoogle Scholar
  44. 44.
    Sandalcioglu IE, Wiedemayer H, Gasser T, Asgari S, Engelhorn T, Stolke D (2003) Intramedullary spinal cord cavernous malformations: clinical features and risk of hemorrhage. Neurosurg Rev 26:253–256. doi:10.1007/s10143-003-0260-2CrossRefPubMedGoogle Scholar
  45. 45.
    Weinzierl MR, Krings T, Korinth MC, Reinges MH, Gilsbach JM (2004) MRI and intraoperative findings in cavernous haemangiomas of the spinal cord. Neuroradiology 46:65–71. doi:10.1007/s00234-003-1072-3CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2016

Authors and Affiliations

  1. 1.Mount Sinai Roosevelt Hospital, Hyman Newman Institute for Neurology and Neurosurgery, Centre for Endovascular SurgeryNew YorkUSA

Personalised recommendations