Skip to main content

AVM Presentation

  • Chapter
  • First Online:
Brain Arteriovenous Malformations
  • 1017 Accesses

Abstract

Cerebral AVMs may go entirely unnoticed, or they may present with neurological symptoms, such as headache, focal neurological deficits, epileptic seizures or intracerebral hemorrhage with decreased levels of consciousness or death. The objective of the subsequent chapter is to outline the various mechanisms of clinical AVM presentation, and their frequency in a given patient population. Due to widespread availability of advanced cranial imaging such as MRI, more and more cerebral AVMs are found incidentally in the western and in many Asian and Australian countries. The frequency of hemorraghic presentation is still in the range of 50% in large reported clinical series, however. Hemorrhage can have dramatic consequences, with up to 30% of mortality and 10–20% of permanent neurological deficits. Some factors, such as diffuse AVM nidus, AVM-associated aneurysms, deep venous drainage and posterior fossa localization predispose to AVM rupture. Epilepsy as a presenting mode of AVMs is reported to be in the range of 10–40%. It is important to distinguish the occasional or sporadic AVM-related seizure from repeated, or chronic seizure activity and from chronic, pharmacoresistent epilepsy due to cerebral AVM. The latter group of patients necessitates elaborate epilepsy surgical evaluation and should surgery be indicated for the AVM and for the epilepsy, the resection may go far beyond the limits of the AVM itself, in order to achieve good epileptological outcomes. Focal neurological deficits are the least likely way cerebral AVMs present, with no more than approximately 10% of patients reported in the respective literature. The so-called “steal-phenomenon” seems not to be responsible for focal neurological deficits in these patients, as has been proved by recent experimental and intraoperative studies. As in symptomatic epilepsy, it seems more likely that local mass-effect and repeated micro-hemorrhage with subsequent tissue scarring may be responsible for the development of focal neurological deficits. Deep localization, i.e. in the brain-stem or in the basal ganglia may also cause presentation with focal neurological deficits.

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

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 139.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 179.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 179.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Abecassis IJ, Xu DS, Batjer HH, Bendok BR. Natural history of brain arteriovenous malformations: a systematic review. Neurosurg Focus. 2014;37(3):E7.

    Article  PubMed  Google Scholar 

  2. Brown RD Jr, Wiebers DO, Forbes G, O’Fallon WM, Piepgras DG, Marsh WR, et al. The natural history of unruptured intracranial arteriovenous malformations. J Neurosurg. 1988;68:352–7.

    Article  PubMed  Google Scholar 

  3. Brown RD Jr, Wiebers DO, Torner JC, O’Fallon WM. 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 

  4. Brown RD Jr, Wiebers DO, Torner JC, O’Fallon WM. Incidence and prevalence of intracranial vascular malformations in Olmsted County, Minnesota, 1965 to 1992. Neurology. 1996;46:949–52.

    Article  PubMed  Google Scholar 

  5. Crawford PM, West CR, Chadwick DW, Shaw MD. 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 

  6. Fullerton HJ, Achrol AS, Johnston SC, McCulloch CE, Higashida RT, Lawton MT, et al. Long-term hemorrhage risk in children versus adults with brain arteriovenous malformations. Stroke. 2005;36:2099–104.

    Article  PubMed  Google Scholar 

  7. 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 

  8. Hernesniemi JA, Dashti R, Juvela S, Väärt K, Niemelä M, Laakso A. Natural history of brain arteriovenous malformations: a long-term follow-up study of risk of hemorrhage in 238 patients. Neurosurgery. 2008;63:823–31.

    Article  PubMed  Google Scholar 

  9. Hillman J. Population-based analysis of arteriovenous malformation treatment. J Neurosurg. 2001;95:633–7.

    Article  CAS  PubMed  Google Scholar 

  10. Mast H, Young WL, Koennecke HC, Sciacca RR, Osipov A, Pile-Spellman J, et al. Risk of spontaneous haemorrhage after diagnosis of cerebral arteriovenous malformation. Lancet. 1997;350:1065–8.

    Article  CAS  PubMed  Google Scholar 

  11. 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;73:387–91.

    Article  CAS  PubMed  Google Scholar 

  12. Pollock BE, Flickinger JC, Lunsford LD, Bissonette DJ, Kondziolka D. Factors that predict the bleeding risk of cerebral arteriovenous malformations. Stroke. 1996;27:1–6.

    Article  CAS  PubMed  Google Scholar 

  13. Stapf C, Mast H, Sciacca RR, Berenstein A, Nelson PK, Gobin YP, et al. The New York Islands AVM Study: design, study progress, and initial results. Stroke. 2003;34:e29–33.

    Article  CAS  PubMed  Google Scholar 

  14. Yang W, Anderson-Keightly H, Westbroek EM, Caplan JM, Rong X, Hung AL, Colby GP, Coon AL, Tamargo RJ, Huang J, Ahn ES. Long-term hemorrhagic risk in pediatric patients with arteriovenous malformations. J Neurosurg Pediatr. 2016;18(3):329–38.

    Article  PubMed  Google Scholar 

  15. Kader A, Young WL, Pile-Spellman J, Mast H, Sciacca RR, Mohr JP, Stein BM. The influence of hemodynamic and anatomic factors on hemorrhage from cerebral arteriovenous malformations. Neurosurgery. 1994;34(5):801–7.

    Article  CAS  PubMed  Google Scholar 

  16. Miyasaka Y, Yada K, Ohwada T, Kitahara T, Kurata A, Irikura K. An analysis of the venous drainage system as a factor in hemorrhage from arteriovenous malformations. J Neurosurg. 1992;76:239–43.

    Article  CAS  PubMed  Google Scholar 

  17. Platz J, Berkefeld J, Singer OC, Wolff R, Seifert V, Konczalla J, Güresir E. Frequency, risk of hemorrhage and treatment considerations for cerebral arteriovenous malformations with associated aneurysms. Acta Neurochir. 2014;156(11):2025–34.

    Article  PubMed  Google Scholar 

  18. Spetzler RF, Hargraves RW, McCormick PW, Zabramski JM, Flom RA, Zimmerman RS. Relationship of perfusion pressure and size to risk of hemorrhage from arteriovenous malformations. J Neurosurg. 1992;76(6):918–23.

    Article  CAS  PubMed  Google Scholar 

  19. Stein KP, Wanke I, Forsting M, Oezkan N, Huetter BO, Sandalcioglu IE, Sure U. Associated aneurysms in infratentorial arteriovenous malformations: role of aneursysm size and comparison with supratentorial lesions. Cerebrovasc Dis. 2016;41:219–25.

    Article  PubMed  Google Scholar 

  20. Turjman F, Massoud TF, Viñuela F, Sayre JW, Guglielmi G, Duckwiler G. Correlation of the angioarchitectural features of cerebral arteriovenous malformations with clinical presentation of hemorrhage. Neurosurgery. 1995;37:856–62.

    Article  CAS  PubMed  Google Scholar 

  21. Vinuela F, Nombela L, Roach MR, Fox AJ, Pelz DM. Stenotic and occlusive disease of the venous drainage system of deep brain AVM’s. J Neurosurg. 1985;63:180–4.

    Article  CAS  PubMed  Google Scholar 

  22. Yamada S, Tamadi Y, Nozaki K, Kikuta K, Hashimoto N. Risk factors for subsequent hemorrhage in patients with cerebral arteriovenous malformations. J Neurosurg. 2007;107:965–72.

    Article  PubMed  Google Scholar 

  23. Da Costa L, Thines L, Dehdashti AR, Wallace MC, Willinsky RA, Tymianski M, Schwartz ML, ter Brugge KG. Management and clinical outcome of posterior fossa arteriovenous malformations: report on a single-centre 15-year experience. J Neurol Neurosurg Psychiatry. 2009;80:376–9.

    Article  PubMed  Google Scholar 

  24. Kouznetsov E, Weill A, Ghostine JS, Gentric JC, Raymond J, Roy D. Association between posterior fossa arteriovenous malformations and prenidal aneurysm rupture: potential impact on management. Neurosurg Focus. 2014;37:E4.

    Article  PubMed  Google Scholar 

  25. Orning J, Amin-Hanjani S, Hamade Y, Du X, Hage ZA, Aletich V, Charbel F, Alaraj A. Increased prevalence and rupture status of feeder vessel aneurysms in posterior fossa arteriovenous malformations. J Neurointerv Surg. 2016;8:1021–4.

    Article  PubMed  Google Scholar 

  26. Torné R, Rodriguez-Hernandez A, Arikan F, Romero-Chala F, Cicuendez M, Vilalta J, Sahuquillo J. Posterior fossa arteriovenous malformations: significance of higher incidence of bleeding and hydrocephalus. Clin Neurol Neurosurg. 2015;134:37–43.

    Article  PubMed  Google Scholar 

  27. Han SJ, Englot DJ, Kim H, Lawton MT. Brainstem arteriovenous malformations: anatomical subtypes, assessment of “occlusion in situ” technique, and microsurgical results. J Neurosurg. 2015;122:107–17.

    Article  PubMed  PubMed Central  Google Scholar 

  28. Kondziolka D, McLaughlin MR, Kestle JR. Simple risk predictions for arteriovenous malformation hemorrhage. Neurosurgery. 1995;37(5):851–5.

    Article  CAS  PubMed  Google Scholar 

  29. Bervini D, Morgan MK, Ritson EA, Heller G. Surgery for unruptured arteriovenous malformations of the brain is better than conservative management for selected cases: a prospective cohort study. J Neurosurg. 2014;121(4):878–90.

    Article  PubMed  Google Scholar 

  30. Schaller K, Steiger HJ. To treat, or not to treat, that is the question: critical review of brain AVM surgery, surgical results and natural history in 2017 by Michael Morgan et al. Acta Neurochir. 2017; doi:10.1007/s00701-017-3221-1.

  31. Schramm J, Schaller K, Esche J, Boström A. Microsurgery for cerebral arteriovenous malformations: subgroup outcomes in a personal series of 288 cases. J Neurosurg. 2017;126:1056–63.

    Article  PubMed  Google Scholar 

  32. Ding D, Quigg M, Starke RM, Yen CP, Przybylowski CJ, Dodson BK, Sheehan JP. Cerebral arteriovenous malformations and epilepsy, Part 2: Predictors of seizure outcomes following radiosurgery. World Neurosurg. 2015;84(3):653–62.

    Article  PubMed  Google Scholar 

  33. Galletti F, Costa C, Cupini LM, Eusebi P, Hamam M, Caputo N, Siliquini S, Conti C, Moschini E, Lunardi P, Carletti S, Calabresi P. Brain arteriovenous malformations and seizures: an Italian study. J Neurol Neurosurg Psychiatry. 2014;85(3):284–8.

    Article  CAS  PubMed  Google Scholar 

  34. Garcin B, Houdart E, Porcher R, Manchon E, Saint-Maurice JP, Bresson D, Stapf C. Epileptic seizures at initial presentation in patients with brain arteriovenous malformation. Neurology. 2012;78:626–31.

    Article  CAS  PubMed  Google Scholar 

  35. Gerszten PC, Adelson PD, Kondziolka D, Flickinger JC, Lunsford LD. Seizure outcome in children treated for arteriovenous malformations using gamma knife radiosurgery. Pediatr Neurosurg. 1996;24(3):139–44.

    Article  CAS  PubMed  Google Scholar 

  36. Hoh BL, Chapman PH, Loeffler JS, Carter BS, Ogilvy CS. Results of multimodality treatment for 141 patients with brain arteriovenous malformations and seizures: factors associated with seizure incidence and seizure outcomes. Neurosurgery. 2002;51:303–309.; discussion 309–11.

    Article  PubMed  Google Scholar 

  37. Josephson CB, Leach JP, Duncan R, Roberts RC, Counsell CE, Al-Shahi Salman R, Scottish Audit of Intracranial Vascular Malformations (SAIVMs) steering committee and collaborators. Seizure risk from cavernous or arteriovenous malformations: prospective population-based study. Neurology. 2011;76(18):1548–54.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  38. Thorpe ML, Cordato DJ, Morgan MK, Herkes GK. Postoperative seizure outcome in a series of 114 patients with supratentorial arteriovenous malformations. J Clin Neurosci. 2000;7(2):107–11.

    Article  CAS  PubMed  Google Scholar 

  39. Turjman F, Massoud TF, Sayre JW, Vinuela F, Guglielmi G, Duckwiler G. Epilepsy associated with cerebral arteriovenous malformations: a multivariate analysis of angioarchitectural characteristics. AJNR Am J Neuroradiol. 1995;16:345–50.

    CAS  PubMed  Google Scholar 

  40. Ruan D, Yu X-B, Shrestha S, Wang L, Chen G. The role of hemosiderin excision in seizure outcome in cerebral cavernous malformation surgery: a systematic review and meta-analysis. PLoS One. 2015;10(8):e0136619.

    Article  PubMed  PubMed Central  Google Scholar 

  41. von der Brelie C, Simon M, Esche J, Schramm J, Boström A. Seizure outcomes in patients with surgically treated cerebral arteriovenous malformations. Neurosurgery. 2015;77(5):762–8.

    Article  PubMed  Google Scholar 

  42. Choi JH, Mast H, Hartmann A, Marshall RS, Pile-Spellman J, Mohr JP, Stapf C. Clinical and morphological determinants of focal neurological deficits in patients with unruptured brain arteriovenous malformation. J Neurol Sci. 2009;287(1–2):126–30.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  43. Mast H, Mohr JP, Osipov A, Pile-Spellman J, Marshall RS, Lazar RM, Stein BM, Young WL. ‘Steal’ is an unestablished mechanism for the clinical presentation of cerebral arteriovenous malformations. Stroke. 1995;26(7):1215–20.

    Article  CAS  PubMed  Google Scholar 

  44. Meyer B, Schaller C, Frenkel C, Ebeling B, Schramm J. Distributions of local oxygen saturation and its response to changes of mean arterial blood pressure in the cerebral cortex adjacent to arteriovenous malformations. Stroke. 1999;30:2623–30.

    Article  CAS  PubMed  Google Scholar 

  45. Schaller C, Schramm J, Haun D, Meyer B. The role of venous drainage in cerebral AVM surgery as related to the development of postoperative hyperperfusion injury. Neurosurgery. 2002;51:921–9.

    PubMed  Google Scholar 

  46. Schaller C, Schramm J, Haun D, Meyer B. Microcirculatory patterns of cerebrovascular reactivity are not predictive for hyperperfusion syndrome after surgery for cerebral arteriovenous malformations. Stroke. 2003;34:938–44.

    Article  PubMed  Google Scholar 

  47. Khaw AV, Mohr JP, Sciacca RR, Schumacher HC, Hartmann A, Pile-Spellman J, Mast H, Stapf C. Association of infratentorial brain arteriovenous malformations with hemorrhage at initial presentation. Stroke. 2004;35:660–3.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Karl Schaller .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2017 Springer International Publishing AG

About this chapter

Cite this chapter

Schaller, K. (2017). AVM Presentation. In: Beneš, V., Bradáč, O. (eds) Brain Arteriovenous Malformations. Springer, Cham. https://doi.org/10.1007/978-3-319-63964-2_6

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-63964-2_6

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-63963-5

  • Online ISBN: 978-3-319-63964-2

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics