UpToDate
Official reprint from UpToDate®
www.uptodate.com ©2016 UpToDate®

Brain arteriovenous malformations

Authors
Robert J Singer, MD
Christopher S Ogilvy, MD
Guy Rordorf, MD
Section Editor
Jose Biller, MD, FACP, FAAN, FAHA
Deputy Editor
Janet L Wilterdink, MD

INTRODUCTION

Arteriovenous malformations (AVMs) are the most dangerous of the congenital vascular malformations with the potential to cause intracranial hemorrhage and epilepsy in many cases. They have become the focus of scientific study leading to technological advances that have permitted these high-flow lesions to be treated, often with a multidisciplinary approach utilizing surgical, endovascular, and radiosurgical techniques.

This topic review will discuss brain AVMs. Three other general subtypes of congenital vascular malformations have been described: developmental venous anomalies, capillary telangiectasias, and cavernous malformations. These are discussed separately. (See "Vascular malformations of the central nervous system".)

EPIDEMIOLOGY

Brain AVMs occur in about 0.1 percent of the population, one-tenth the incidence of intracranial aneurysms [1]. Supratentorial lesions account for 90 percent of brain AVMs; the remainder are in the posterior fossa. They usually occur as single lesions, but as many as 9 percent are multiple [2].

Brain AVMs underlie 1 to 2 percent of all strokes, 3 percent of strokes in young adults, and 9 percent of subarachnoid hemorrhages [3,4].

PATHOGENESIS AND PATHOLOGY

Brain AVMs are considered sporadic congenital developmental vascular lesions, but their pathogenesis is not well understood. Rare cases of familial brain AVMs have been reported but it is unclear if these are coincidental or indicate true familial occurrence [5]. However, genetic variation may influence brain AVM development and clinical course [6,7].

                     

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Nov 2016. | This topic last updated: Mon Nov 07 00:00:00 GMT+00:00 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2016 UpToDate, Inc.
References
Top
  1. Mohr JP, Kejda-Scharler J, Pile-Spellman J. Diagnosis and treatment of arteriovenous malformations. Curr Neurol Neurosci Rep 2013; 13:324.
  2. Willinsky RA, Lasjaunias P, Terbrugge K, Burrows P. Multiple cerebral arteriovenous malformations (AVMs). Review of our experience from 203 patients with cerebral vascular lesions. Neuroradiology 1990; 32:207.
  3. Al-Shahi R, Warlow C. A systematic review of the frequency and prognosis of arteriovenous malformations of the brain in adults. Brain 2001; 124:1900.
  4. Friedlander RM. Clinical practice. Arteriovenous malformations of the brain. N Engl J Med 2007; 356:2704.
  5. van Beijnum J, van der Worp HB, Schippers HM, et al. Familial occurrence of brain arteriovenous malformations: a systematic review. J Neurol Neurosurg Psychiatry 2007; 78:1213.
  6. Hashimoto T, Lawton MT, Wen G, et al. Gene microarray analysis of human brain arteriovenous malformations. Neurosurgery 2004; 54:410.
  7. Pawlikowska L, Tran MN, Achrol AS, et al. Polymorphisms in genes involved in inflammatory and angiogenic pathways and the risk of hemorrhagic presentation of brain arteriovenous malformations. Stroke 2004; 35:2294.
  8. Bharatha A, Faughnan ME, Kim H, et al. Brain arteriovenous malformation multiplicity predicts the diagnosis of hereditary hemorrhagic telangiectasia: quantitative assessment. Stroke 2012; 43:72.
  9. Perata HJ, Tomsick TA, Tew JM Jr. Feeding artery pedicle aneurysms: association with parenchymal hemorrhage and arteriovenous malformation in the brain. J Neurosurg 1994; 80:631.
  10. Moftakhar P, Hauptman JS, Malkasian D, Martin NA. Cerebral arteriovenous malformations. Part 2: physiology. Neurosurg Focus 2009; 26:E11.
  11. Moftakhar P, Hauptman JS, Malkasian D, Martin NA. Cerebral arteriovenous malformations. Part 1: cellular and molecular biology. Neurosurg Focus 2009; 26:E10.
  12. Garcin B, Houdart E, Porcher R, et al. Epileptic seizures at initial presentation in patients with brain arteriovenous malformation. Neurology 2012; 78:626.
  13. Fullerton HJ, Achrol AS, Johnston SC, et al. Long-term hemorrhage risk in children versus adults with brain arteriovenous malformations. Stroke 2005; 36:2099.
  14. Al-Shahi Salman R. The outlook for adults with epileptic seizure(s) associated with cerebral cavernous malformations or arteriovenous malformations. Epilepsia 2012; 53 Suppl 4:34.
  15. Josephson CB, Leach JP, Duncan R, et al. Seizure risk from cavernous or arteriovenous malformations: prospective population-based study. Neurology 2011; 76:1548.
  16. Evans RW. Diagnostic testing for the evaluation of headaches. Neurol Clin 1996; 14:1.
  17. Gross BA, Du R. Natural history of cerebral arteriovenous malformations: a meta-analysis. J Neurosurg 2013; 118:437.
  18. Brown RD Jr, Wiebers DO, Forbes G, et al. The natural history of unruptured intracranial arteriovenous malformations. J Neurosurg 1988; 68:352.
  19. Ondra SL, Troupp H, George ED, Schwab K. The natural history of symptomatic arteriovenous malformations of the brain: a 24-year follow-up assessment. J Neurosurg 1990; 73:387.
  20. Stapf C, Mast H, Sciacca RR, et al. Predictors of hemorrhage in patients with untreated brain arteriovenous malformation. Neurology 2006; 66:1350.
  21. da Costa L, Wallace MC, Ter Brugge KG, et al. The natural history and predictive features of hemorrhage from brain arteriovenous malformations. Stroke 2009; 40:100.
  22. Guo Y, Saunders T, Su H, et al. Silent intralesional microhemorrhage as a risk factor for brain arteriovenous malformation rupture. Stroke 2012; 43:1240.
  23. Khaw AV, Mohr JP, Sciacca RR, et al. Association of infratentorial brain arteriovenous malformations with hemorrhage at initial presentation. Stroke 2004; 35:660.
  24. Choi JH, Mast H, Sciacca RR, et al. Clinical outcome after first and recurrent hemorrhage in patients with untreated brain arteriovenous malformation. Stroke 2006; 37:1243.
  25. Brown RD Jr, Flemming KD, Meyer FB, et al. Natural history, evaluation, and management of intracranial vascular malformations. Mayo Clin Proc 2005; 80:269.
  26. Horton JC, Chambers WA, Lyons SL, et al. Pregnancy and the risk of hemorrhage from cerebral arteriovenous malformations. Neurosurgery 1990; 27:867.
  27. Saleh RS, Singhal A, Lohan D, et al. Assessment of cerebral arteriovenous malformations with high temporal and spatial resolution contrast-enhanced magnetic resonance angiography: a review from protocol to clinical application. Top Magn Reson Imaging 2008; 19:251.
  28. Barr JC, Ogilvy CS. Selection of treatment modalities or observation of arteriovenous malformations. Neurosurg Clin N Am 2012; 23:63.
  29. Spetzler RF, Martin NA. A proposed grading system for arteriovenous malformations. J Neurosurg 1986; 65:476.
  30. Hamilton MG, Spetzler RF. The prospective application of a grading system for arteriovenous malformations. Neurosurgery 1994; 34:2.
  31. Schaller C, Schramm J, Haun D. Significance of factors contributing to surgical complications and to late outcome after elective surgery of cerebral arteriovenous malformations. J Neurol Neurosurg Psychiatry 1998; 65:547.
  32. Hartmann A, Stapf C, Hofmeister C, et al. Determinants of neurological outcome after surgery for brain arteriovenous malformation. Stroke 2000; 31:2361.
  33. Kim H, Pourmohamad T, Westbroek EM, et al. Evaluating performance of the spetzler-martin supplemented model in selecting patients with brain arteriovenous malformation for surgery. Stroke 2012; 43:2497.
  34. Starke RM, Yen CP, Ding D, Sheehan JP. A practical grading scale for predicting outcome after radiosurgery for arteriovenous malformations: analysis of 1012 treated patients. J Neurosurg 2013; 119:981.
  35. Karlsson B, Lax I, Söderman M. Risk for hemorrhage during the 2-year latency period following gamma knife radiosurgery for arteriovenous malformations. Int J Radiat Oncol Biol Phys 2001; 49:1045.
  36. Steinberg GK, Fabrikant JI, Marks MP, et al. Stereotactic heavy-charged-particle Bragg-peak radiation for intracranial arteriovenous malformations. N Engl J Med 1990; 323:96.
  37. Fabrikant JI, Levy RP, Steinberg GK, et al. Charged-particle radiosurgery for intracranial vascular malformations. Neurosurg Clin N Am 1992; 3:99.
  38. Kurita H, Kawamoto S, Sasaki T, et al. Results of radiosurgery for brain stem arteriovenous malformations. J Neurol Neurosurg Psychiatry 2000; 68:563.
  39. Levy RP, Fabrikant JI, Frankel KA, et al. Stereotactic heavy-charged-particle Bragg peak radiosurgery for the treatment of intracranial arteriovenous malformations in childhood and adolescence. Neurosurgery 1989; 24:841.
  40. Karlsson B, Lindquist C, Steiner L. Effect of Gamma Knife surgery on the risk of rupture prior to AVM obliteration. Minim Invasive Neurosurg 1996; 39:21.
  41. Pollock BE, Flickinger JC, Lunsford LD, et al. Hemorrhage risk after stereotactic radiosurgery of cerebral arteriovenous malformations. Neurosurgery 1996; 38:652.
  42. Friedman WA, Blatt DL, Bova FJ, et al. The risk of hemorrhage after radiosurgery for arteriovenous malformations. J Neurosurg 1996; 84:912.
  43. Yen CP, Sheehan JP, Schwyzer L, Schlesinger D. Hemorrhage risk of cerebral arteriovenous malformations before and during the latency period after GAMMA knife radiosurgery. Stroke 2011; 42:1691.
  44. Kano H, Kondziolka D, Flickinger JC, et al. Aneurysms increase the risk of rebleeding after stereotactic radiosurgery for hemorrhagic arteriovenous malformations. Stroke 2012; 43:2586.
  45. Maruyama K, Kondziolka D, Niranjan A, et al. Stereotactic radiosurgery for brainstem arteriovenous malformations: factors affecting outcome. J Neurosurg 2004; 100:407.
  46. Ogilvy CS, Stieg PE, Awad I, et al. AHA Scientific Statement: Recommendations for the management of intracranial arteriovenous malformations: a statement for healthcare professionals from a special writing group of the Stroke Council, American Stroke Association. Stroke 2001; 32:1458.
  47. Pollock BE, Link MJ, Brown RD. The risk of stroke or clinical impairment after stereotactic radiosurgery for ARUBA-eligible patients. Stroke 2013; 44:437.
  48. Yen CP, Schlesinger D, Sheehan JP. Natural history of cerebral arteriovenous malformations and the risk of hemorrhage after radiosurgery. Prog Neurol Surg 2013; 27:5.
  49. Maruyama K, Kawahara N, Shin M, et al. The risk of hemorrhage after radiosurgery for cerebral arteriovenous malformations. N Engl J Med 2005; 352:146.
  50. Sun DQ, Carson KA, Raza SM, et al. The radiosurgical treatment of arteriovenous malformations: obliteration, morbidities, and performance status. Int J Radiat Oncol Biol Phys 2011; 80:354.
  51. Parkhutik V, Lago A, Tembl JI, et al. Postradiosurgery hemorrhage rates of arteriovenous malformations of the brain: influencing factors and evolution with time. Stroke 2012; 43:1247.
  52. Lunsford LD, Kondziolka D, Flickinger JC, et al. Stereotactic radiosurgery for arteriovenous malformations of the brain. J Neurosurg 1991; 75:512.
  53. Ogilvy CS. Radiation therapy for arteriovenous malformations: a review. Neurosurgery 1990; 26:725.
  54. Fabrikant JI, Levy RP, Steinberg GK, et al. Stereotactic charged-particle radiosurgery: clinical results of treatment of 1200 patients with intracranial arteriovenous malformations and pituitary disorders. Clin Neurosurg 1992; 38:472.
  55. Skjøth-Rasmussen J, Roed H, Ohlhues L, et al. Complications following linear accelerator based stereotactic radiation for cerebral arteriovenous malformations. Int J Radiat Oncol Biol Phys 2010; 77:542.
  56. Friedman WA, Bova FJ, Bollampally S, Bradshaw P. Analysis of factors predictive of success or complications in arteriovenous malformation radiosurgery. Neurosurgery 2003; 52:296.
  57. Zipfel GJ, Bradshaw P, Bova FJ, Friedman WA. Do the morphological characteristics of arteriovenous malformations affect the results of radiosurgery? J Neurosurg 2004; 101:393.
  58. Foote KD, Friedman WA, Ellis TL, et al. Salvage retreatment after failure of radiosurgery in patients with arteriovenous malformations. J Neurosurg 2003; 98:337.
  59. Pollock BE, Meyer FB. Radiosurgery for arteriovenous malformations. J Neurosurg 2004; 101:390.
  60. Flickinger JC, Pollock BE, Kondziolka D, Lunsford LD. A dose-response analysis of arteriovenous malformation obliteration after radiosurgery. Int J Radiat Oncol Biol Phys 1996; 36:873.
  61. Karlsson B, Lindquist C, Steiner L. Prediction of obliteration after gamma knife surgery for cerebral arteriovenous malformations. Neurosurgery 1997; 40:425.
  62. Maruyama K, Koga T, Niranjan A, et al. Radiosurgery for brainstem arteriovenous malformation. Prog Neurol Surg 2013; 27:67.
  63. Hauswald H, Milker-Zabel S, Sterzing F, et al. Repeated linac-based radiosurgery in high-grade cerebral arteriovenous-malformations (AVM) Spetzler-Martin grade III to IV previously treated with radiosurgery. Radiother Oncol 2011; 98:217.
  64. Flickinger JC, Kondziolka D, Lunsford LD, et al. A multi-institutional analysis of complication outcomes after arteriovenous malformation radiosurgery. Int J Radiat Oncol Biol Phys 1999; 44:67.
  65. Ding D, Yen CP, Xu Z, et al. Radiosurgery for patients with unruptured intracranial arteriovenous malformations. J Neurosurg 2013; 118:958.
  66. Yang SY, Kim DG, Chung HT, Paek SH. Radiosurgery for unruptured cerebral arteriovenous malformations: long-term seizure outcome. Neurology 2012; 78:1292.
  67. Flickinger JC, Kondziolka D, Lunsford LD, et al. Development of a model to predict permanent symptomatic postradiosurgery injury for arteriovenous malformation patients. Arteriovenous Malformation Radiosurgery Study Group. Int J Radiat Oncol Biol Phys 2000; 46:1143.
  68. Pollock BE, Gorman DA, Brown PD. Radiosurgery for arteriovenous malformations of the basal ganglia, thalamus, and brainstem. J Neurosurg 2004; 100:210.
  69. Miyawaki L, Dowd C, Wara W, et al. Five year results of LINAC radiosurgery for arteriovenous malformations: outcome for large AVMS. Int J Radiat Oncol Biol Phys 1999; 44:1089.
  70. Buis DR, Meijer OW, van den Berg R, et al. Clinical outcome after repeated radiosurgery for brain arteriovenous malformations. Radiother Oncol 2010; 95:250.
  71. Blonder LX, Hodes JE, Ranseen JD, Schmitt FA. Short-term neuropsychological outcome following Gamma Knife radiosurgery for arteriovenous malformations: a preliminary report. Appl Neuropsychol 1999; 6:181.
  72. Spetzler RF, Martin NA, Carter LP, et al. Surgical management of large AVM's by staged embolization and operative excision. J Neurosurg 1987; 67:17.
  73. Krings T, Hans FJ, Geibprasert S, Terbrugge K. Partial "targeted" embolisation of brain arteriovenous malformations. Eur Radiol 2010; 20:2723.
  74. Hartmann A, Pile-Spellman J, Stapf C, et al. Risk of endovascular treatment of brain arteriovenous malformations. Stroke 2002; 33:1816.
  75. Starke RM, Komotar RJ, Otten ML, et al. Adjuvant embolization with N-butyl cyanoacrylate in the treatment of cerebral arteriovenous malformations: outcomes, complications, and predictors of neurologic deficits. Stroke 2009; 40:2783.
  76. Al-Shahi R, Warlow C. Arteriovenous malformations of the brain: ready to randomise? J Neurol Neurosurg Psychiatry 2005; 76:1327.
  77. Ross J, Al-Shahi Salman R. Interventions for treating brain arteriovenous malformations in adults. Cochrane Database Syst Rev 2010; :CD003436.
  78. Starke RM, Komotar RJ, Hwang BY, et al. Treatment guidelines for cerebral arteriovenous malformation microsurgery. Br J Neurosurg 2009; 23:376.
  79. van Beijnum J, van der Worp HB, Buis DR, et al. Treatment of brain arteriovenous malformations: a systematic review and meta-analysis. JAMA 2011; 306:2011.
  80. Mohr JP, Parides MK, Stapf C, et al. Medical management with or without interventional therapy for unruptured brain arteriovenous malformations (ARUBA): a multicentre, non-blinded, randomised trial. Lancet 2014; 383:614.
  81. Knopman J, Stieg PE. Management of unruptured brain arteriovenous malformations. Lancet 2014; 383:581.
  82. Mohr JP, Stapf C, Sciacca RR, et al. Treatment outcome versus natural history risk in patients with unruptured brain arteriovenous malformation [abstract]. Neurology 2004; 62 (Suppl 5):A101.
  83. Wedderburn CJ, van Beijnum J, Bhattacharya JJ, et al. Outcome after interventional or conservative management of unruptured brain arteriovenous malformations: a prospective, population-based cohort study. Lancet Neurol 2008; 7:223.
  84. Tamaki N, Ehara K, Lin TK, et al. Cerebral arteriovenous malformations: factors influencing the surgical difficulty and outcome. Neurosurgery 1991; 29:856.
  85. Kondziolka D, McLaughlin MR, Kestle JR. Simple risk predictions for arteriovenous malformation hemorrhage. Neurosurgery 1995; 37:851.
  86. Brown RD Jr. Simple risk predictions for arteriovenous malformation hemorrhage. Neurosurgery 2000; 46:1024.
  87. Englot DJ, Young WL, Han SJ, et al. Seizure predictors and control after microsurgical resection of supratentorial arteriovenous malformations in 440 patients. Neurosurgery 2012; 71:572.
  88. Josephson CB, Bhattacharya JJ, Counsell CE, et al. Seizure risk with AVM treatment or conservative management: prospective, population-based study. Neurology 2012; 79:500.
  89. Piepgras DG, Sundt TM Jr, Ragoowansi AT, Stevens L. Seizure outcome in patients with surgically treated cerebral arteriovenous malformations. J Neurosurg 1993; 78:5.
  90. Schäuble B, Cascino GD, Pollock BE, et al. Seizure outcomes after stereotactic radiosurgery for cerebral arteriovenous malformations. Neurology 2004; 63:683.