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Brain Arteriovenous Malformations pp 37–49Cite as

Epidemiology and Natural History of AVMs

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Abstract

Brain AVMs are rare and complex vascular lesions of unknown etiology, with the incidence rate of newly diagnosed cases of approximately 1–1.5 in 100,000 person years. Prevalence of AVMs in the population is unknown, but may be as high as 0.2%, even though the prevalence of confirmed AVM diagnosis is only 0.02%. AVMs seem to be slightly more common in males. Although the incidence of unruptured and asymptomatic AVMs is probably gradually increasing with the availability of noninvasive imaging, the most common and dangerous form of presentation of an AVM is still hemorrhagic stroke. Epileptic seizures, headache and focal neurological deficits are other forms of presenting symptoms. Although intracranial hemorrhage caused by an AVM may be somewhat less harmful than hemorrhagic strokes by other causes, AVM hemorrhage typically affects younger populations, and untreated AVMs are associated with significant long-term excess mortality. Average annual risk of rupture of an all untreated AVMs combined is between 2% and 4%, but the risk is highly variable and depends on the individual characteristics of the lesion. Factors associated with increased risk of hemorrhage include previous rupture, deep and infratentorial location, deep venous drainage, associated aneurysms and possibly large size.

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References

  1. Waltimo O. The change in size of intracranial arteriovenous malformations. J Neurol Sci. 1973;19:21–7.

    Article  CAS  PubMed  Google Scholar 

  2. Pasqualin A, Vivenza C, Rosta L, et al. Spontaneous disappearance of intracranial arterio-venous malformations. Acta Neurochir. 1985;76:50–7.

    Article  CAS  PubMed  Google Scholar 

  3. Kader A, Goodrich JT, Sonstein WJ, et al. Recurrent cerebral arteriovenous malformations after negative postoperative angiograms. J Neurosurg. 1996;85:14–8.

    Article  CAS  PubMed  Google Scholar 

  4. Lasjaunias P. A revised concept of the congenital nature of cerebral arteriovenous malformations. Interv Neuroradiol. 1997;3:275–81.

    Article  CAS  PubMed  Google Scholar 

  5. Lee SK, Vilela P, Willinsky R, TerBrugge KG. Spontaneous regression of cerebral arteriovenous malformations: clinical and angiographic analysis with review of the literature. Neuroradiology. 2002;44:11–6.

    Article  CAS  PubMed  Google Scholar 

  6. Buis DR, van den Berg R, Lycklama G, et al. Spontaneous regression of brain arteriovenous malformations--a clinical study and a systematic review of the literature. J Neurol. 2004;251:1375–82.

    Article  PubMed  Google Scholar 

  7. Morales-Valero SF, Bortolotti C, Sturiale C, et al. Are parenchymal AVMs congenital lesions? Neurosurg Focus. 2014;37:E2.

    Article  PubMed  Google Scholar 

  8. Petridis AK, Fischer I, Cornelius JF, et al. Demographic distribution of hospital admissions for brain arteriovenous malformations in Germany – estimation of the natural course with the big-data approach. Acta Neurochir (Wien). 158:791–6.

    Google Scholar 

  9. 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;78:1213–7.

    Article  PubMed  PubMed Central  Google Scholar 

  10. van Beijnum J, van der Worp HB, Algra A, et al. Prevalence of brain arteriovenous malformations in first-degree relatives of patients with a brain arteriovenous malformation. Stroke. 2014;45:3231–5.

    Article  PubMed  Google Scholar 

  11. Brown RD Jr, Wiebers DO, Torner JC, et al. Incidence and prevalence of intracranial vascular malformations in Olmsted County, Minnesota, 1965 to 1992. Neurology. 1996;46:949–52.

    Article  PubMed  Google Scholar 

  12. Hillman J. Population-based analysis of arteriovenous malformation treatment. J Neurosurg. 95:633–7.

    Google Scholar 

  13. ApSimon HT, Reef H, Phadke RV, et al. A population-based study of brain arteriovenous malformation: long-term treatment outcomes. Stroke. 2002;33:2794–800.

    Article  CAS  PubMed  Google Scholar 

  14. Stapf C, Mast H, Sciacca RR, et al. The New York Islands AVM Study: design, study progress, and initial results. Stroke. 2003;34:e29–33.

    Article  CAS  PubMed  Google Scholar 

  15. Stapf C, Labovitz DL, Sciacca RR, et al. Incidence of adulta brain arteriovenous malformation hemorrhage in a prospective population-based stroke survey. Cerebrovasc Dis. 2002;13:43–6.

    Article  PubMed  Google Scholar 

  16. Al-Shahi R, Warlow C. A systematic review of the frequency and prognosis of arteriovenous malformations of the brain in adults. Brain. 2001;124:1900–26.

    Article  CAS  PubMed  Google Scholar 

  17. Hofmeister C, Stapf C, Hartmann A, et al. Demographic, morphological, and clinical characteristics of 1289 paitents with brain arteriovenous malformation. Stroke. 2000;31:1307–10.

    Article  CAS  PubMed  Google Scholar 

  18. Al-Shahi R, Fang JS, Lewis SC, et al. Prevalence of adults with brain arteriovenous malformations: a community based study in Scotland using capture-recapture analysis. J Neurol Neurosurg Psychiatry. 2002;73:547–51.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  19. Tong X, Wu J, Lin F, et al. The effect of age, sex, and lesion location on initial presentation in patients with brain arteriovenous malformations. World Neurosurg. 2016;87:598–606.

    Article  PubMed  Google Scholar 

  20. Weber F, Knopf H. Incidental findings in magnetic resonance imaging of the brains of healthy young men. J Neurol Sci. 2006;240:81–4.

    Article  PubMed  Google Scholar 

  21. Vlak MHM, Algra A, Brandenburg R, et al. Prevalence of unruptured intracranial aneurysms, with emphasis on sex, age, comorbidity, country, and time period: a systematic review and meta-analysis. Lancet Neurol. 2011;10:626–36.

    Article  PubMed  Google Scholar 

  22. Hartmann A, Mast H, Mohr JP, et al. Morbidity of intracranial hemorrhage in patients with cerebral arteriovenous malformation. Stroke. 1998;29:931–4.

    Article  CAS  PubMed  Google Scholar 

  23. Choi JH, Mast H, Sciacca RR, et al. Clinical outcome after first and recurrent hemorrhage in patients with untreated brain arteriovenous malformation. Stroke. 2006;37:1243–7.

    Article  PubMed  Google Scholar 

  24. Mohr JP, Parides MK, Stapf C, et al. Medical management with or without interventional therapy for unruptured brain arteriovenous malformations (ARUBA): a multicenter, non-blinded, randomised trial. Lancet. 2014;383:614–21.

    Article  CAS  PubMed  Google Scholar 

  25. Forster DM, Steiner L, Håkanson S. Arteriovenous malformations of the brain. A long-term clinical study. J Neurosurg. 1972;37:562–70.

    Article  CAS  PubMed  Google Scholar 

  26. Abad JM, Alvarez F, Manrique M, et al. Cerebral arteriovenous malformations. Comparative results of surgical vs conservative treatment in 112 cases. J Neurosurg Sci. 1983;27:203–10.

    CAS  PubMed  Google Scholar 

  27. Crawford PM, West CR, Chadwick DW, et al. Arteriovenous malformations of the brain: natural history in unoperated patients. J Neurol Neurosurg Psychiatry. 1986;49:1–10.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Itoyama Y, Uemura S, Ushio Y, et al. Natural course of unoperated intracranial arteriovenous malformations: study of 50 cases. J Neurosurg. 1989;71:805–9.

    Article  CAS  PubMed  Google Scholar 

  29. Ondra SL, Troupp H, George ED, et al. The natural history of symptomatic arteriovenous malformations of the brain: a 24-year follow-up assessment. J Neurosurg. 1990;73:387–91.

    Article  CAS  PubMed  Google Scholar 

  30. Mine S, Hirai S, Ono J, et al. Risk factors for poor outcome of untreated arteriovenous malformation. J Clin Neurosci. 2000;7:503–6.

    Article  CAS  PubMed  Google Scholar 

  31. Laakso A, Dashti R, Seppänen J, et al. Long-term excess mortality in 623 patients with brain arteriovenous malformations. Neurosurgery. 2008;63:244–53.

    Article  PubMed  Google Scholar 

  32. Langer DJ, Lasner TM, Hurst RW, et al. Hypertension, small size, and deep venous drainage are associated with risk of hemorrhagic presentation of cerebral arteriovenous malformations. Neurosurgery. 1998;42:481–6.

    Article  CAS  PubMed  Google Scholar 

  33. Stapf C, Mast H, Sciacca RR, et al. Predictors of hemorrhage in patients with untreated brain arteriovenous malformation. Neurology. 2006;66:1350–5.

    Article  CAS  PubMed  Google Scholar 

  34. Fults D, Kelly DL Jr. Natural history of arteriovenous malformations of the brain: a clinical study. Neurosurgery. 1984;15:658–62.

    Article  CAS  PubMed  Google Scholar 

  35. Kader A, Young WL, Pile-Spellman J, et al. The influence of hemodynamic and anatomic factors on hemorrhage from cerebral arteriovenous malformations. Neurosurgery. 1994;34:801–7.

    Article  CAS  PubMed  Google Scholar 

  36. Mast H, Young WL, Koennecke HC, et al. Risk of spontaneous haemorrhage after diagnosis of cerebral arteriovenous malformation. Lancet. 1997;350:1065–8.

    Article  CAS  PubMed  Google Scholar 

  37. Halim AX, Johnston SC, Singh V, et al. Longitudinal risk of intracranial hemorrhage in patients with arteriovenous malformation of the brain within a defined population. Stroke. 2004;35:1697–702.

    Article  PubMed  Google Scholar 

  38. Yamada S, Takagi Y, Nozaki K, et al. Risk factors for subsequent hemorrhage in patients with cerebral arteriovenous malformations. J Neurosurg. 2007;107:965–72.

    Article  PubMed  Google Scholar 

  39. Fullerton HJ, Achrol AS, Johnston SC, et al. Long-term hemorrhage risk in children versus adults with brain arteriovenous malformations. Stroke. 2005;36:2099–104.

    Article  PubMed  Google Scholar 

  40. Hetts SW, Cooke DL, Nelson J, et al. Influence of patient age on angioarchitecture of brain arteriovenous malformations. AJNR Am J Neuroradiol. 2014;35:1376–80.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  41. Ko NU, Johnston SC, Young WL, et al. Distinguishing intracerebral hemorrhages caused by arteriovenous malformations. Cerebrovasc Dis. 2003;15:206–9.

    Article  CAS  PubMed  Google Scholar 

  42. van Beijnum J, Lovelock CE, Cordonnier C, et al. Outcome after spontaneous and arteriovenous malformation-related intracerebral haemorrhage: population-based studies. Brain. 2009;132:537–43.

    Article  PubMed  Google Scholar 

  43. Taylor B, Appelboom G, Yang A, et al. Underlying effect of age on outcome differences in arteriovenous malformation-associated intracerebral hemorrhage. J Clin Neurosci. 2015;22:526–9.

    Article  PubMed  Google Scholar 

  44. Brown RD Jr, Wiebers DO, Torner JC. Frequency of intracranial hemorrhage as a presenting symptom and subtype analysis: a population-based study of intracranial vascular malformations in Olmsted Country, Minnesota. J Neurosurg. 1996;85:29–32.

    Article  PubMed  Google Scholar 

  45. da Costa L, Wallace MC, Ter Brugge KG, et al. The natural history and predictive features of hemorrhage from brain arteriovenous malformations. Stroke. 2009;40:100–5.

    Article  PubMed  Google Scholar 

  46. Laakso A, Dashti R, Juvela S, et al. Risk of hemorrhage in patients with untreated Spetzler-Martin grade IV and V arteriovenous malformations: a long-term follow-up study in 63 patients. Neurosurgery. 2011;68:372–7.

    Article  PubMed  Google Scholar 

  47. Duong DH, Young WL, Vang MC, et al. Feeding artery pressure and venous drainage pattern are primary determinants of hemorrhage from cerebral arteriovenous malformations. Stroke. 1998;29:1167–76.

    Article  CAS  PubMed  Google Scholar 

  48. Stefani MA, Porter PJ, terBrugge KG, et al. Large and deep brain arteriovenous malformations are associated with risk of future hemorrhage. Stroke. 2002;33:1220–4.

    Article  PubMed  Google Scholar 

  49. Khaw AV, Mohr JP, Sciacca RR, et al. Association of infratentorial brain arteriovenous malformations with hemorrhage at initial presentation. Stroke. 2004;35:660–3.

    Article  CAS  PubMed  Google Scholar 

  50. Spetzler RF, Hargraves RW, McCormick PW, et al. Relationship of perfusion pressure and size to risk of hemorrhage from arteriovenous malformations. J Neurosurg. 1992;76:918–23.

    Article  CAS  PubMed  Google Scholar 

  51. Ding D, Starke RM, Quigg M, et al. Cerebral arteriovenous malformations and epilepsy, Part1. 1: Predictors of seizure presentation. World Neurosurg. 2015;84:645–52.

    Article  PubMed  Google Scholar 

  52. Forsgren L. Prospective incidence study and clinical characterization of seizures in newly referred adults. Epilepsia. 1990;31:292–301.

    Article  CAS  PubMed  Google Scholar 

  53. Crawford PM, West CR, Shaw MD, et al. Cerebral arteriovenous malformations and epilepsy: factors in the development of epilepsy. Epilepsia. 1986;27:270–5.

    Article  CAS  PubMed  Google Scholar 

  54. Hoh BL, Chapman PH, Loeffler JS, et al. Results of multimodality treatment for 141 patients with brain arteriovenous malformations and seizures: factors associated with seizure incidence and seizure outcomes. Neurosurgery. 2002;51:303–9.

    Article  PubMed  Google Scholar 

  55. Englot DJ, Young WL, Han SJ, et al. Seizure predictors and control after microsurgical resection of supratentorial arteriovenous malformations in 440 patients. Neurosurgery. 2012;71:572–80.

    Article  PubMed  Google Scholar 

  56. Turjman F, Massoud TF, Sayre JW, et al. Epilepsy associated with cerebral arteriovenous malformations: a multivariate analysis of angioarchitectural characteristics. AJNR Am J Neuroradiol. 1995;16:345–50.

    CAS  PubMed  Google Scholar 

  57. Ellis JA, Mejia Munne JC, Lavine SD, et al. Arteriovenous malformations and headache. J Clin Neurosci. 2016;23:38–43.

    Article  PubMed  Google Scholar 

  58. Choi JH, Mast H, Hartmann A, et al. Clinical and morphological determinants of focal neurological deficits in patients with unruptured brain arteriovenous malformation. J Neurol Sci. 2009;287:126–30.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  59. Kim H, McCulloch CE, Johnston SC, et al. Comparison of 2 approaches for determining the natural history risk of brain arteriovenous malformation rupture. Am J Epidemiol. 2010;171:1317–22.

    Article  PubMed  PubMed Central  Google Scholar 

  60. Hernesniemi JA, Dashti R, Juvela S, et al. Natural history of brain arteriovenous malformations: a long-term follow-up study of risk of hemorrhage in 238 patients. Neurosurgery. 2008;63:823–9.

    Article  PubMed  Google Scholar 

  61. Gross BA, Du R. Natural history of cerebral arteriovenous malformations: a meta-analysis. J Neurosurg. 2013;118:437–43.

    Article  PubMed  Google Scholar 

  62. Kim H, Al-Shahi Salman R, McCulloch CE, et al. Untreated brain arteriovenous malformation. Patient-level meta-analysis of hemorrhage predictors. Neurology. 2014;83:590–7.

    Article  PubMed  PubMed Central  Google Scholar 

  63. Gross BA, Du R. Rate of re-bleeding of arteriovenous malformations in the first year after rupture. J Clin Neurosci. 2012;19:1087–8.

    Article  PubMed  Google Scholar 

  64. Guo Y, Saunders T, Su H, et al. Silent intralesional microhemorrhage as a risk factor for brain arteriovenous malformation rupture. Stroke. 2012;43:1240–6.

    Article  PubMed  PubMed Central  Google Scholar 

  65. Abla AA, Nelson J, Kim H, et al. Silent arteriovenous malformation hemorrhage and the recognition of “unruptured” arteriovenous malformation patients who benefit from surgical intervention. Neurosurgery. 2015;76:592–600.

    Article  PubMed  PubMed Central  Google Scholar 

  66. Guidetti B, Delitala A. Intracranial arteriovenous malformations. Conservative and surgical treatment. J Neurosurg. 1980;53:149–52.

    Article  CAS  PubMed  Google Scholar 

  67. Liu X, Wang S, Zhao YI, et al. Risk of cerebral arteriovenous malformation rupture during pregnancy and puerperium. Neurology. 2014;82:1798–803.

    Article  PubMed  PubMed Central  Google Scholar 

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Authors and Affiliations

  1. Department of Neurosurgery, Helsinki University Central Hospital, P.O. Box 266, Topeliuksenkatu 5, 00260, Helsinki, Finland

    Aki Laakso M.D., Ph.D.

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Correspondence to Aki Laakso M.D., Ph.D. .

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  1. Department of Neurosurgery and Neuro-oncology, Institute of Clinical Neurosciences, Military University Hospital and First Faculty of Medicine, Charles University, Prague, Czech Republic

    Vladimír Beneš

  2. Department of Neurosurgery and Neuro-oncology, Institute of Clinical Neurosciences, Military University Hospital and First Faculty of Medicine, Charles University, Prague, Czech Republic

    Ondřej Bradáč

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Laakso, A. (2017). Epidemiology and Natural History of AVMs. In: Beneš, V., Bradáč, O. (eds) Brain Arteriovenous Malformations. Springer, Cham. https://doi.org/10.1007/978-3-319-63964-2_4

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  • DOI: https://doi.org/10.1007/978-3-319-63964-2_4

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