Skip to main content

Radiosurgery of Brain Arteriovenous and Cavernous Malformations

  • Living reference work entry
  • First Online:
Radiation Oncology

Abstract

Brain arteriovenous malformations (bAVMs) consist of an abnormal tangle of vessels that shunts blood directly from an artery to a vein, without intervention of a capillary bed. The absence of a capillary bed results into an abnormal high pressure flow from the feeding artery or arteries into draining vein(s) and thus higher pressure on the venous side. bAVMs are rare vascular entities, with an incidence of approximately 1.3 per 100,000 person-years and a prevalence of 10–18 per 100,000 person-years. bAVMs are the most frequent cause of nontraumatic intracerebral hemorrhage (ICH) in people younger than 35 years. Other clinical manifestations are seizures, headaches and progressive neurological symptoms (8%) related to ischemia of the surrounding 122w2ssssewdsafgewrhytehtbrain tissue. The main treatment goal of bAVMs is the complete elimination or obliteration of the nidus and of the arteriovenous shunts. The first method is microsurgical resection, which can be performed primarily or following bAVM embolization to reduce the risk of bleeding during surgery and make the resection less challenging. The second method is endovascular embolization, which can be used as a complementary method to the previous techniques, however, in some specific circumstances could serve as definitive treatment and achieve complete obliteration of the bAVM. The third option is stereotactic radiosurgery (SRS), which can in line with microsurgery be applied primarily or after embolization. This chapter discuss the current data on classifications and management of bAVM or cavernous malformation patients.

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

Access this chapter

Institutional subscriptions

References

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

    Article  Google Scholar 

  • Abecassis IJ, Nerva JD, Feroze A, Barber J, Ghodke BV, Kim LJ, et al. Multimodality management of Spetzler-Martin Grade 3 brain arteriovenous malformations with subgroup analysis. World Neurosurg. 2017;102: 263–74.

    Article  Google Scholar 

  • Abla AA, Lekovic GP, Turner JD, de Oliveira JG, Porter R, Spetzler RF. Advances in the treatment and outcome of brainstem cavernous malformation surgery: a single-center case series of 300 surgically treated patients. Neurosurgery. 2011;68:403–14;discussion 414–405.

    Article  Google Scholar 

  • Almefty KK, Spetzler RF. Management of brainstem cavernous malformations. World Neurosurg. 2015;83: 317–9.

    Article  Google Scholar 

  • Al-Shahi Salman R, White PM, Counsell CE, du Plessis J, van Beijnum J, Josephson CB, et al. Outcome after conservative management or intervention for unruptured brain arteriovenous malformations. JAMA. 2014;311:1661–9.

    Article  Google Scholar 

  • Al-Shahi R, Fang JS, Lewis SC, Warlow CP. 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  Google Scholar 

  • Amin-Hanjani S, Ogilvy CS, Ojemann RG, Crowell RM. Risks of surgical management for cavernous malformations of the nervous system. Neurosurgery. 1998;42:1220–7;discussion 1227–1228.

    Article  CAS  Google Scholar 

  • Awad I, Jabbour P. Cerebral cavernous malformations and epilepsy. Neurosurg Focus. 2006;21:e7.

    PubMed  Google Scholar 

  • Backes D, Rinkel GJ, Kemperman H, Linn FH, Vergouwen MD. Time-dependent test characteristics of head computed tomography in patients suspected of nontraumatic subarachnoid hemorrhage. Stroke. 2012;43:2115–9.

    Article  Google Scholar 

  • Baranoski JF, Kalani MYS, Przybylowski CJ, Zabramski JM. Corrigendum: cerebral cavernous malformations: review of the genetic and protein-protein interactions resulting in disease pathogenesis. Front Surg. 2017;4:31.

    Article  Google Scholar 

  • Baumann CR, Acciarri N, Bertalanffy H, Devinsky O, Elger CE, Lo Russo G, et al. Seizure outcome after resection of supratentorial cavernous malformations: a study of 168 patients. Epilepsia. 2007;48:559–63.

    Article  Google Scholar 

  • 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;121:878–90.

    Article  Google Scholar 

  • Braksick SA, Fugate JE. Management of brain arteriovenous malformations. Curr Treat Options Neurol. 2015;17:358.

    Article  Google Scholar 

  • Chang SD, Marcellus ML, Marks MP, Levy RP, Do HM, Steinberg GK. Multimodality treatment of giant intracranial arteriovenous malformations. Neurosurgery. 2003;53:1–11;discussion 11–13.

    Article  Google Scholar 

  • Chen CJ, Norat P, Ding D, Mendes GAC, Tvrdik P, Park MS, et al. Transvenous embolization of brain arteriovenous malformations: a review of techniques, indications, and outcomes. Neurosurg Focus. 2018;45:E13.

    Article  Google Scholar 

  • Cordonnier C, Klijn CJ, van Beijnum J, Al-Shahi SR. Radiological investigation of spontaneous intracerebral hemorrhage: systematic review and trinational survey. Stroke. 2010;41:685–90.

    Article  Google Scholar 

  • da Costa L, Wallace MC, Ter Brugge KG, O’Kelly C, Willinsky RA, Tymianski M. The natural history and predictive features of hemorrhage from brain arteriovenous malformations. Stroke. 2009;40:100–5.

    Article  Google Scholar 

  • Dalyai RT, Ghobrial G, Awad I, Tjoumakaris S, Gonzalez LF, Dumont AS, et al. Management of incidental cavernous malformations: a review. Neurosurg Focus. 2011;31:E5.

    Article  Google Scholar 

  • Deibert CP, Ahluwalia MS, Sheehan JP, Link MJ, Hasegawa T, Yomo S, et al. Bevacizumab for refractory adverse radiation effects after stereotactic radiosurgery. J Neuro-Oncol. 2013;115:217–23.

    Article  CAS  Google Scholar 

  • Del Curling O Jr, Kelly DL Jr, Elster AD, Craven TE. An analysis of the natural history of cavernous angiomas. J Neurosurg. 1991;75:702–8.

    Article  Google Scholar 

  • Delev D, Pavlova A, Grote A, Bostrom A, Hollig A, Schramm J, et al. Notch4 gene polymorphisms as potential risk factors for brain arteriovenous malformation development and hemorrhagic presentation. J Neurosurg. 2017;126:1552–9.

    Article  Google Scholar 

  • Derdeyn CP, Zipfel GJ, Albuquerque FC, Cooke DL, Feldmann E, Sheehan JP, et al. Management of brain arteriovenous malformations: a scientific statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. 2017;48:e200–24.

    PubMed  Google Scholar 

  • Englot DJ, Han SJ, Lawton MT, Chang EF. Predictors of seizure freedom in the surgical treatment of supratentorial cavernous malformations. J Neurosurg. 2011;115:1169–74.

    Article  Google Scholar 

  • Fierstra J, Conklin J, Krings T, Slessarev M, Han JS, Fisher JA, et al. Impaired peri-nidal cerebrovascular reserve in seizure patients with brain arteriovenous malformations. Brain. 2011;134:100–9.

    Article  Google Scholar 

  • Galletti F, Costa C, Cupini LM, Eusebi P, Hamam M, Caputo N, et al. Brain arteriovenous malformations and seizures: an Italian study. J Neurol Neurosurg Psychiatry. 2014;85:284–8.

    Article  CAS  Google Scholar 

  • Hernesniemi JA, Dashti R, Juvela S, Vaart K, Niemela 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: 823–9;discussion 829–831.

    Article  Google Scholar 

  • Hofmeister C, Stapf C, Hartmann A, Sciacca RR, Mansmann U, terBrugge K, et al. Demographic, morphological, and clinical characteristics of 1289 patients with brain arteriovenous malformation. Stroke. 2000;31:1307–10.

    Article  CAS  Google Scholar 

  • Horne MA, Flemming KD, Su IC, Stapf C, Jeon JP, Li D, et al. Clinical course of untreated cerebral cavernous malformations: a meta-analysis of individual patient data. Lancet Neurol. 2016;15:166–73.

    Article  Google Scholar 

  • Joint Writing Group of the Technology Assessment Committee American Society of Interventional and Therapeutic Neuroradiology, Joint Section on Cerebrovascular Neurosurgery a Section of the American Association of Neurological Surgeons and Congress of Neurological Surgeons, Section of Stroke and the Section of Interventional Neurology of the American Academy of Neurology, et al. Reporting terminology for brain arteriovenous malformation clinical and radiographic features for use in clinical trials. Stroke. 2001;32:1430–42.

    Article  Google Scholar 

  • Josephson CB, Leach JP, Duncan R, Roberts RC, Counsell CE, Al-Shahi Salman R, et al. Seizure risk from cavernous or arteriovenous malformations: prospective population-based study. Neurology. 2011;76: 1548–54.

    Article  CAS  Google Scholar 

  • Josephson CB, White PM, Krishan A, Al-Shahi SR. Computed tomography angiography or magnetic resonance angiography for detection of intracranial vascular malformations in patients with intracerebral haemorrhage. Cochrane Database Syst Rev. 2014;(9):CD009372.

    Google Scholar 

  • Kim H, Al-Shahi Salman R, McCulloch CE, Stapf C, Young WL, MARS Coinvestigators. Untreated brain arteriovenous malformation: patient-level meta-analysis of hemorrhage predictors. Neurology. 2014a;83: 590–7.

    Article  Google Scholar 

  • Kim MH, Park KM, Hwang JK, Park SC, Moon IS, Kim JI. The natural history of arteriovenous access and risk factors associated with access thrombosis after successful kidney transplantation. Transplant Proc. 2014b;46:602–6.

    Article  CAS  Google Scholar 

  • Kim H, Nelson J, Krings T, terBrugge KG, McCulloch CE, Lawton MT, et al. Hemorrhage rates from brain arteriovenous malformation in patients with hereditary hemorrhagic telangiectasia. Stroke. 2015;46:1362–4.

    Article  CAS  Google Scholar 

  • Koltz MT, Polifka AJ, Saltos A, Slawson RG, Kwok Y, Aldrich EF, et al. Long-term outcome of Gamma Knife stereotactic radiosurgery for arteriovenous malformations graded by the Spetzler-Martin classification. J Neurosurg. 2013;118:74–83.

    Article  Google Scholar 

  • Kondo R, Matsumoto Y, Endo H, Miyachi S, Ezura M, Sakai N. Endovascular embolization of cerebral arteriovenous malformations: results of the Japanese Registry of Neuroendovascular Therapy (JR-NET) 1 and 2. Neurol Med Chir (Tokyo). 2014;54(Suppl 2):54–62.

    Article  Google Scholar 

  • Korja M, Bervini D, Assaad N, Morgan MK. Role of surgery in the management of brain arteriovenous malformations: prospective cohort study. Stroke. 2014;45:3549–55.

    Article  Google Scholar 

  • Labauge P, Denier C, Bergametti F, Tournier-Lasserve E. Genetics of cavernous angiomas. Lancet Neurol. 2007;6:237–44.

    Article  CAS  Google Scholar 

  • Lai LF, Chen M, Chen JX, Zheng K, He XY, Li XF, et al. Multidisciplinary care of unruptured brain arteriovenous malformations to improve symptomatic headache and the onset, progression, and outcomes of unruptured brain arteriovenous malformations. Pain Physician. 2017;20:E127–36.

    PubMed  Google Scholar 

  • Lawton MT, UCSF Brain Arteriovenous Malformation Study Project. Spetzler-Martin Grade III arteriovenous malformations: surgical results and a modification of the grading scale. Neurosurgery. 2003;52:740–8;discussion 748–749.

    Article  Google Scholar 

  • Lawton MT, Kim H, McCulloch CE, Mikhak B, Young WL. A supplementary grading scale for selecting patients with brain arteriovenous malformations for surgery. Neurosurgery. 2010;66: 702–13;discussion 713.

    Article  Google Scholar 

  • Lu XY, Sun H, Xu JG, Li QY. Stereotactic radiosurgery of brainstem cavernous malformations: a systematic review and meta-analysis. J Neurosurg. 2014;120: 982–7.

    Article  Google Scholar 

  • Lunsford LD, Kondziolka D, Flickinger JC, Bissonette DJ, Jungreis CA, Maitz AH, et al. Stereotactic radiosurgery for arteriovenous malformations of the brain. J Neurosurg. 1991;75:512–24.

    Article  CAS  Google Scholar 

  • Lunsford LD, Khan AA, Niranjan A, Kano H, Flickinger JC, Kondziolka D. Stereotactic radiosurgery for symptomatic solitary cerebral cavernous malformations considered high risk for resection. J Neurosurg. 2010;113:23–9.

    Article  Google Scholar 

  • Macmurdo CF, Wooderchak-Donahue W, Bayrak-Toydemir P, Le J, Wallenstein MB, Milla C, et al. Rasa1 somatic mutation and variable expressivity in capillary malformation/arteriovenous malformation (CM/AVM) syndrome. Am J Med Genet A. 2016;170:1450–4.

    Article  CAS  Google Scholar 

  • Mohr JP, Parides MK, Stapf C, Moquete E, Moy CS, Overbey JR, et al. Medical management with or without interventional therapy for unruptured brain arteriovenous malformations (ARUBA): a multicentre, non-blinded, randomised trial. Lancet. 2014;383: 614–21.

    Article  CAS  Google Scholar 

  • Morgan MK, Rochford AM, Tsahtsarlis A, Little N, Faulder KC. Surgical risks associated with the management of Grade I and II brain arteriovenous malformations. Neurosurgery. 2004;54:832–7;discussion 837–839.

    Article  Google Scholar 

  • Morris Z, Whiteley WN, Longstreth WT Jr, Weber F, Lee YC, Tsushima Y, et al. Incidental findings on brain magnetic resonance imaging: systematic review and meta-analysis. BMJ. 2009;339:b3016.

    Article  Google Scholar 

  • Moultrie F, Horne MA, Josephson CB, Hall JM, Counsell CE, Bhattacharya JJ, et al. Outcome after surgical or conservative management of cerebral cavernous malformations. Neurology. 2014;83:582–9.

    Article  Google Scholar 

  • Nakamura M, Samii A, Lang JM, Gotz F, Samii M, Krauss JK. De novo arteriovenous malformation growth secondary to implantation of genetically modified allogeneic mesenchymal stem cells in the brain. Neurosurgery. 2016;78:E596–600.

    Article  Google Scholar 

  • Nikolaev SI, Vetiska S, Bonilla X, Boudreau E, Jauhiainen S, Rezai Jahromi B, et al. Somatic activating KRAS mutations in arteriovenous malformations of the brain. N Engl J Med. 2018;378:250–61.

    Article  Google Scholar 

  • Ogilvy CS, Stieg PE, Awad I, Brown RD Jr, Kondziolka D, Rosenwasser R, et al. 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. Circulation. 2001;103:2644–57.

    Article  CAS  Google Scholar 

  • Ojemann RG, Ogilvy CS. Microsurgical treatment of supratentorial cavernous malformations. Neurosurg Clin N Am. 1999;10:433–40.

    Article  CAS  Google Scholar 

  • 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  Google Scholar 

  • Pham M, Gross BA, Bendok BR, Awad IA, Batjer HH. Radiosurgery for angiographically occult vascular malformations. Neurosurg Focus. 2009;26:E16.

    Article  Google Scholar 

  • Pollock BE, Flickinger JC. A proposed radiosurgery-based grading system for arteriovenous malformations. J Neurosurg. 2002;96:79–85.

    Article  Google Scholar 

  • Pollock BE, Garces YI, Stafford SL, Foote RL, Schomberg PJ, Link MJ. Stereotactic radiosurgery for cavernous malformations. J Neurosurg. 2000;93: 987–91.

    Article  CAS  Google Scholar 

  • Pollock BE, Link MJ, Stafford SL, Lanzino G, Garces YI, Foote RL. Volume-staged stereotactic radiosurgery for intracranial arteriovenous malformations: outcomes based on an 18-year experience. Neurosurgery. 2017a;80:543–50.

    Article  Google Scholar 

  • Pollock BE, Link MJ, Branda ME, Storlie CB. Incidence and management of late adverse radiation effects after arteriovenous malformation radiosurgery. Neurosurgery. 2017b;81:928.

    Article  Google Scholar 

  • Potts MB, Zumofen DW, Raz E, Nelson PK, Riina HA. Curing arteriovenous malformations using embolization. Neurosurg Focus. 2014;37:E19.

    Article  Google Scholar 

  • Rigamonti D, Hadley MN, Drayer BP, Johnson PC, Hoenig-Rigamonti K, Knight JT, et al. Cerebral cavernous malformations. Incidence and familial occurrence. N Engl J Med. 1988;319:343–7.

    Article  CAS  Google Scholar 

  • Ruiz-Sandoval JL, Cantu C, Barinagarrementeria F. Intracerebral hemorrhage in young people: analysis of risk factors, location, causes, and prognosis. Stroke. 1999;30:537–41.

    Article  CAS  Google Scholar 

  • Saeed Kilani M, Lepennec V, Petit P, Magalon G, Casanova D, Bartoli JM, et al. Embolization of peripheral high-flow arteriovenous malformations with onyx. Diagn Interv Imaging. 2017;98:217–26.

    Article  CAS  Google Scholar 

  • Schneider BF, Eberhard DA, Steiner LE. Histopathology of arteriovenous malformations after gamma knife radiosurgery. J Neurosurg. 1997;87:352–7.

    Article  CAS  Google Scholar 

  • Seymour ZA, Sneed PK, Gupta N, Lawton MT, Molinaro AM, Young W, et al. Volume-staged radiosurgery for large arteriovenous malformations: an evolving paradigm. J Neurosurg. 2016;124:163–74.

    Article  Google Scholar 

  • Shin SS, Murdoch G, Hamilton RL, Faraji AH, Kano H, Zwagerman NT, et al. Pathological response of cavernous malformations following radiosurgery. J Neurosurg. 2015;123:938–44.

    Article  CAS  Google Scholar 

  • Spetzler RF, Martin NA. A proposed grading system for arteriovenous malformations. J Neurosurg. 1986;65: 476–83.

    Article  CAS  Google Scholar 

  • Spetzler RF, Ponce FA. A 3-tier classification of cerebral arteriovenous malformations. Clinical article. J Neurosurg. 2011;114:842–9.

    Article  Google Scholar 

  • 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.

    CAS  PubMed  Google Scholar 

  • Tranvinh E, Heit JJ, Hacein-Bey L, Provenzale J, Wintermark M. Contemporary imaging of cerebral arteriovenous malformations. AJR Am J Roentgenol. 2017;208:1320–30.

    Article  Google Scholar 

  • Voigt K, Yasargil MG. Cerebral cavernous haemangiomas or cavernomas. Incidence, pathology, localization, diagnosis, clinical features and treatment. Review of the literature and report of an unusual case. Neurochirurgia (Stuttg). 1976;19:59–68.

    CAS  Google Scholar 

  • Wang JY, Yang W, Ye X, Rigamonti D, Coon AL, Tamargo RJ, et al. Impact on seizure control of surgical resection or radiosurgery for cerebral arteriovenous malformations. Neurosurgery. 2013;73:648–55;discussion 655–646.

    Article  Google Scholar 

  • Wang KY, Idowu OR, Lin DDM. Radiology and imaging for cavernous malformations. Handb Clin Neurol. 2017;143:249–66.

    Article  Google Scholar 

  • Willems PW, Taeshineetanakul P, Schenk B, Brouwer PA, Terbrugge KG, Krings T. The use of 4d-CTA in the diagnostic work-up of brain arteriovenous malformations. Neuroradiology. 2012;54:123–31.

    Article  Google Scholar 

  • Zabramski JM, Wascher TM, Spetzler RF, Johnson B, Golfinos J, Drayer BP, et al. The natural history of familial cavernous malformations: results of an ongoing study. J Neurosurg. 1994;80:422–32.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Nima Etminan .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer Nature Switzerland AG

About this entry

Check for updates. Verify currency and authenticity via CrossMark

Cite this entry

Bazarde, H.A., Wenz, F., Hänggi, D., Etminan, N. (2019). Radiosurgery of Brain Arteriovenous and Cavernous Malformations. In: Wenz, F. (eds) Radiation Oncology. Springer, Cham. https://doi.org/10.1007/978-3-319-52619-5_10-1

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-52619-5_10-1

  • Received:

  • Accepted:

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-52619-5

  • Online ISBN: 978-3-319-52619-5

  • eBook Packages: Springer Reference MedicineReference Module Medicine

Publish with us

Policies and ethics