Skip to main content
SpringerLink
Log in

Vein of Galen Aneurysmal Malformation

  • Chapter
  • First Online:
Pineal Region Lesions

Abstract

In this chapter, the disease pattern “vein of Galen aneurysmal malformation” (VGAM) is outlined. In Part A, the body of the literature is summarized with sections, namely historical aspects and definitions, epidemiology and clinical presentation, pathophysiology and pathoanatomy, classification of VGAM subtypes, and treatment and outcome. Part B covers controversial issues and dedicated management strategies, whereby the perspective and proceedings in a VGAM high-volume center are presented: indications for treatment, the angioarchitecture of VGAMs and strategy of endovascular therapy, and modern techniques in diagnostic and interventional neuroradiology. The authors’ intention is to provide comprehensive clear explanations of VGAMs and its interdisciplinary management. A special focus constitutes the recent advances in the field of diagnostic and interventional neuroradiology, which is key for safe and effective embolization procedures in VGAM patients. In the emergency setting, endovascular therapy must be performed for hemodynamic stabilization with prevention of multi-organ failure and ultimately death. In the staged elective setting, endovascular therapy is applied for the neurological stabilization, with the goal of normal or near-normal neurological development as well as the prevention of intracranial hemorrhage. New images and illustrations help to understand the highly complex embolization techniques and associated hemodynamic changes of cerebral blood flow.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 89.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
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. Mortazavi MM, Griessenauer CJ, Foreman P, Bavarsad Shahripour R, Shoja MM, Rozzelle CJ, et al. Vein of Galen aneurysmal malformations: critical analysis of the literature with proposal of a new classification system. J Neurosurg Pediatr. 2013;12(3):293–306.

    Article  PubMed  Google Scholar 

  2. Gailloud P, O’riordan DP, Burger I, Lehmann CU. Confirmation of communication between deep venous drainage and the vein of galen after treatment of a vein of Galen aneurysmal malformation in an infant presenting with severe pulmonary hypertension. AJNR Am J Neuroradiol. 2006;27(2):317–20.

    CAS  PubMed  PubMed Central  Google Scholar 

  3. Recinos PF, Rahmathulla G, Pearl M, Recinos VR, Jallo GI, Gailloud P, et al. Vein of Galen malformations: epidemiology, clinical presentations, management. Neurosurg Clin N Am. 2012;23(1):165–77.

    Article  PubMed  Google Scholar 

  4. Cao L-R, Cai C-Q. Vein of Galen aneurysmal malformation: an updated review. J Pediatr Neurol. 2019;17(02):045–56.

    Article  Google Scholar 

  5. Khullar D, Andeejani AMI, Bulsara KR. Evolution of treatment options for vein of Galen malformations. J Neurosurg Pediatr. 2010;6(5):444–51.

    Article  PubMed  Google Scholar 

  6. Sivasankar R, Limaye US, Wuppalapati S, Shrivastava M. Endovascular Management of Vein of Galen aneurysmal malformations: a retrospective analysis over a 15-year period. J Vasc Interv Neurol. 2019;10(3):23–9.

    PubMed  PubMed Central  Google Scholar 

  7. Yaşargil MG, Yaşargil MG. AVM of the brain, clinical considerations, general and special operative techniques, surgical results, nonoperated cases, cavernous and venous angiomas, neuroanesthesia; 191 tables. Stuttgart: Thieme [u.a.]; 1988. p. 479. (Microneurosurgery)

    Google Scholar 

  8. Niimi Y. Endovascular treatment of pediatric intracranial arteriovenous shunt. Pediatr Int Off J Jpn Pediatr Soc. 2017;59(3):247–57.

    Article  Google Scholar 

  9. Jea A, Bradshaw TJ, Whitehead WE, Curry DJ, Dauser RC, Luerssen TG. The high risks of ventriculoperitoneal shunt procedures for hydrocephalus associated with vein of Galen malformations in childhood: case report and literature review. Pediatr Neurosurg. 2010;46(2):141–5.

    Article  PubMed  Google Scholar 

  10. Hassan T, Nassar M, Elghandour M. Vein of Galen aneurysms: presentation and endovascular management. Pediatr Neurosurg. 2010;46(6):427–34.

    Article  PubMed  Google Scholar 

  11. Berenstein A, Paramasivam S, Sorscher M, Molofsky W, Meila D, Ghatan S. Vein of Galen Aneurysmal Malformation: Advances in Management and Endovascular treatment. Neurosurgery. 2019;84(2):469–78.

    Article  PubMed  Google Scholar 

  12. Howarth RA, Reisner A, Chern JJ, Hayes LL, Burns TG, Berenstein A. Neurocognitive improvements following endovascular repair of vein of Galen malformation in a child. J Neurosurg Pediatr. 2015;15(2):197–202.

    Article  PubMed  Google Scholar 

  13. Hoang S, Choudhri O, Edwards M, Guzman R. Vein of Galen malformation. Neurosurg Focus. 2009;27(5):E8.

    Article  PubMed  Google Scholar 

  14. Raybaud CA, Strother CM, Hald JK. Aneurysms of the vein of Galen: embryonic considerations and anatomical features relating to the pathogenesis of the malformation. Neuroradiology. 1989;31(2):109–28.

    Article  CAS  PubMed  Google Scholar 

  15. Harrigan MR, Deveikis JP. Handbook of cerebrovascular disease and neurointerventional technique. 2nd ed. Dordecht: Humana Press; 2013. p. 850. (Contemporary medical imaging)

    Book  Google Scholar 

  16. Millar C, Bissonnette B, Humphreys RP. Cerebral arteriovenous malformations in children. Can J Anaesth J Can Anesth. 1994;41(4):321–31.

    Article  CAS  Google Scholar 

  17. Lasjaunias PL, Chng SM, Sachet M, Alvarez H, Rodesch G, Garcia-Monaco R. The management of vein of Galen aneurysmal malformations. Neurosurgery. 2006;59(5 Suppl 3):S184–94. discussion S3–13

    PubMed  Google Scholar 

  18. Lasjaunias PL. Vascular diseases in neonates, infants and children: interventional neuroradiology management. Berlin. New York: Springer; 1997. p. 707.

    Book  Google Scholar 

  19. Albright AL, Pollack IF, Adelson PD, editors. Principles and practice of pediatric neurosurgery. 2nd ed. New York: Thieme; 2008. p. 1286.

    Google Scholar 

  20. Alvarez H, Garcia Monaco R, Rodesch G, Sachet M, Krings T, Lasjaunias P. Vein of galen aneurysmal malformations. Neuroimaging Clin N Am. 2007;17(2):189–206.

    Article  CAS  PubMed  Google Scholar 

  21. Winn HR, Youmans JR. Editors. Youmans neurological surgery. 5. Ed. ff. Philadelphia: Saunders; 2004.

    Google Scholar 

  22. Chevret L, Durand P, Alvarez H, Lambert V, Caeymax L, Rodesch G, et al. Severe cardiac failure in newborns with VGAM. Prognosis significance of hemodynamic parameters in neonates presenting with severe heart failure owing to vein of Galen arteriovenous malformation. Intensive Care Med. 2002;28(8):1126–30.

    Article  CAS  PubMed  Google Scholar 

  23. Geibprasert S, Krings T, Armstrong D, Terbrugge KG, Raybaud CA. Predicting factors for the follow-up outcome and management decisions in vein of Galen aneurysmal malformations. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg. 2010;26(1):35–46.

    Article  Google Scholar 

  24. Heuer GG, Gabel B, Beslow LA, Stiefel MF, Schwartz ES, Storm PB, et al. Diagnosis and treatment of vein of Galen aneurysmal malformations. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg. 2010;26(7):879–87.

    Article  Google Scholar 

  25. Berenstein A, Fifi JT, Niimi Y, Presti S, Ortiz R, Ghatan S, et al. Vein of Galen malformations in neonates: new management paradigms for improving outcomes. Neurosurgery. 2012;70(5):1207–13. discussion 1213-1214

    Article  PubMed  Google Scholar 

  26. Wagner KM, Ghali MGZ, Srinivasan VM, Lam S, Johnson J, Chen S, et al. Vein of Galen malformations: the Texas Children’s hospital experience in the modern endovascular era. Oper Neurosurg Hagerstown Md. 2019;17(3):286–92.

    Article  Google Scholar 

  27. Brinjikji W, Krings T, Murad MH, Rouchaud A, Meila D. Endovascular treatment of vein of Galen malformations: a systematic review and meta-analysis. AJNR Am J Neuroradiol. 2017;38(12):2308–14.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Narayanan M, Atwal GS, Nakaji P. Multimodality management of cerebral arteriovenous malformations. Handb Clin Neurol. 2017;143:85–96.

    Article  PubMed  Google Scholar 

  29. Magro E, Gentric J-C, Batista AL, Kotowski M, Chaalala C, Roberge D, et al. The treatment of brain AVMs study (TOBAS): an all-inclusive framework to integrate clinical care and research. J Neurosurg. 2018;128(6):1823–9.

    Article  PubMed  Google Scholar 

  30. Zaidi HA, Kalani MYS, Spetzler RF, McDougall CG, Albuquerque FC. Multimodal treatment strategies for complex pediatric cerebral arteriovenous fistulas: contemporary case series at Barrow neurological institute. J Neurosurg Pediatr. 2015;15(6):615–24.

    Article  PubMed  Google Scholar 

  31. Meling TR, Patet G. What is the best therapeutic approach to a pediatric patient with a deep-seated brain AVM? Neurosurg Rev. 2019;42(2):409–16.

    Article  PubMed  Google Scholar 

  32. Chow ML, Cooke DL, Fullerton HJ, Amans MR, Narvid J, Dowd CF, et al. Radiological and clinical features of vein of Galen malformations. J Neurointerventional Surg. 2015;7(6):443–8.

    Article  Google Scholar 

  33. Lylyk P, Viñuela F, Dion JE, Duckwiler G, Guglielmi G, Peacock W, et al. Therapeutic alternatives for vein of Galen vascular malformations. J Neurosurg. 1993;78(3):438–45.

    Article  CAS  PubMed  Google Scholar 

  34. Friedman DM, Verma R, Madrid M, Wisoff JH, Berenstein A. Recent improvement in outcome using transcatheter embolization techniques for neonatal aneurysmal malformations of the vein of Galen. Pediatrics. 1993;91(3):583–6.

    CAS  PubMed  Google Scholar 

  35. Lasjaunias P, Garcia-Monaco R, Rodesch G, Ter Brugge K, Zerah M, Tardieu M, et al. Vein of Galen malformation. Endovascular management of 43 cases. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg. 1991;7(7):360–7.

    Article  CAS  Google Scholar 

  36. Ciricillo SF, Edwards MS, Schmidt KG, Hieshima GB, Silverman NH, Higashida RT, et al. Interventional neuroradiological management of vein of Galen malformations in the neonate. Neurosurgery. 1990;27(1):22–7. discussion 27-28

    Article  CAS  PubMed  Google Scholar 

  37. Meila D, Hannak R, Feldkamp A, Schlunz-Hendann M, Mangold A, Jacobs C, et al. Vein of Galen aneurysmal malformation: combined transvenous and transarterial method using a “kissing microcatheter technique”. Neuroradiology. 2012;54(1):51–9.

    Article  PubMed  Google Scholar 

  38. Payne BR, Prasad D, Steiner M, Bunge H, Steiner L. Gamma surgery for vein of Galen malformations. J Neurosurg. 2000;93(2):229–36.

    Article  CAS  PubMed  Google Scholar 

  39. Triffo WJ, Bourland JD, Couture DE, McMullen KP, Tatter SB, Morris PP. Definitive treatment of vein of Galen aneurysmal malformation with stereotactic radiosurgery. J Neurosurg. 2014;120(1):120–5.

    Article  PubMed  Google Scholar 

  40. Zeng X, Hunt A, Jin SC, Duran D, Gaillard J, Kahle KT. EphrinB2-EphB4-RASA1 signaling in human cerebrovascular development and disease. Trends Mol Med. 2019;25(4):265–86.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  41. Burch EA, Orbach DB. Pediatric central nervous system vascular malformations. Pediatr Radiol. 2015;45(Suppl 3):S463–72.

    Article  PubMed  Google Scholar 

  42. Duran D, Karschnia P, Gaillard JR, Karimy JK, Youngblood MW, DiLuna ML, et al. Human genetics and molecular mechanisms of vein of Galen malformation. J Neurosurg Pediatr. 2018;21(4):367–74.

    Article  PubMed  Google Scholar 

  43. Meila D, Schmidt C, Melber K, Grieb D, Jacobs C, Jacobs C, et al. Delayed and incomplete treatment may result in dural fistula development in children with vein of Galen malformation. Interv Neuroradiol J Peritherapeutic Neuroradiol Surg Proced Relat Neurosci. 2018;24(1):82–7.

    Google Scholar 

  44. Brassel F, Meila D, Papke K. Vascular interventions in the head and neck region. Part 2: procedures for vessel occlusion. Radiol. 2011;51(6):519–33.

    CAS  Google Scholar 

  45. Dürr NR, Brinjikji W, Pohrt A, Lanfermann H, Brassel F, Meila D. Non-enhanced MR imaging for preinterventional assessment of the angioarchitecture in vein of Galen malformations. J Neurointerventional Surg. 2018;10(10):999–1004.

    Article  Google Scholar 

  46. Emmel M, Bennink G, Meila D, Brassel F. Coarctation of the aorta and vein of Galen malformation - treatment considerations in a severely compromised patient. Cardiol Young. 2012;22(5):596–9.

    Article  PubMed  Google Scholar 

  47. Meila D, Paramasivam S, Niimi Y, Brassel F, Berenstein A. The persistent primitive marginal sinus-observations in children with vein of Galen malformation. Neuroradiology. 2012;54(12):1375–9.

    Article  PubMed  Google Scholar 

  48. Meila D, Lisseck K, Jacobs C, Lanfermann H, Brassel F, Feldkamp A. Cranial Doppler ultrasound in vein of Galen malformation. Neuroradiology. 2015;57(2):211–9.

    Article  PubMed  Google Scholar 

  49. Meila D, Grieb D, Melber K, Jacobs C, Maslehaty H, Petridis A, et al. Hydrocephalus in vein of Galen malformation: etiologies and therapeutic management implications. Acta Neurochir. 2016;158(7):1279–84.

    Article  PubMed  Google Scholar 

  50. Meila D, Melber K, Grieb D, Jacobs C, Lanfermann H, Brassel F. Fistulous-type vein of Galen malformation phantom model for endovascular training and research. J Neurointerventional Surg. 2017;9(9):880–6.

    Article  Google Scholar 

  51. Winkler O, Brinjikji W, Lanfermann H, Brassel F, Meila D. Anatomy of the deep venous system in vein of Galen malformation and its changes after endovascular treatment depicted by magnetic resonance venography. J Neurointerventional Surg. 2019;11(1):84–9.

    Article  Google Scholar 

  52. Papke K, Meila D, Brassel F. Vascular interventions in the head and neck region. Part 1: recanalization procedures. Radiol. 2011;51(3):223–33. quiz 234–6

    CAS  Google Scholar 

  53. Lecce F, Robertson F, Rennie A, Heuchan A-M, Lister P, Bhate S, et al. Cross-sectional study of a United Kingdom cohort of neonatal vein of galen malformation. Ann Neurol. 2018;84(4):547–55.

    Article  PubMed  PubMed Central  Google Scholar 

  54. Taffin H, Maurey H, Ozanne A, Durand P, Husson B, Knebel J-F, et al. Long-term outcome of vein of Galen malformation. Dev Med Child Neurol. 2019.

    Google Scholar 

  55. Puvabanditsin S, Mehta R, Palomares K, Gengel N, Da Silva CF, Roychowdhury S, et al. Vein of Galen malformation in a neonate: a case report and review of endovascular management. World J Clin Pediatr. 2017;6(1):103–9.

    Article  PubMed  PubMed Central  Google Scholar 

  56. Giorgi L, Durand P, Morin L, Miatello J, Merchaoui Z, Lambert V, et al. Management and outcomes of neonatal Arteriovenous brain malformations with cardiac failure: a 17 years’ experience in a tertiary referral center. J Pediatr. 2019;20

    Google Scholar 

  57. Moersdorf M, Lasjaunias P. Quantified partial embolisation in a vein of galen malformation with congestive cardiac failure. Interv Neuroradiol J Peritherapeutic Neuroradiol Surg Proced Relat Neurosci. 1996;2(2):149–54.

    CAS  Google Scholar 

  58. Casasco A, Lylyk P, Hodes JE, Kohan G, Aymard A, Merland JJ. Percutaneous transvenous catheterization and embolization of vein of galen aneurysms. Neurosurgery. 1991;28(2):260–6.

    Article  CAS  PubMed  Google Scholar 

  59. Sekhon LH, Morgan MK, Spence I. Normal perfusion pressure breakthrough: the role of capillaries. J Neurosurg. 1997;86(3):519–24.

    Article  CAS  PubMed  Google Scholar 

  60. Morgan MK, Johnston IH, Sundt TM. Normal perfusion pressure breakthrough complicating surgery for the vein of Galen malformation: report of three cases. Neurosurgery. 1989;24(3):406–10.

    Article  CAS  PubMed  Google Scholar 

  61. Alexander MD, Connolly ES, Meyers PM. Revisiting normal perfusion pressure breakthrough in light of hemorrhage-induced vasospasm. World J Radiol. 2010;2(6):230–2.

    Article  PubMed  PubMed Central  Google Scholar 

  62. Dowd CF, Halbach VV, Barnwell SL, Higashida RT, Edwards MS, Hieshima GB. Transfemoral venous embolization of vein of Galen malformations. AJNR Am J Neuroradiol. 1990;11(4):643–8.

    CAS  PubMed  PubMed Central  Google Scholar 

  63. Mickle JP, Quisling RG. The transtorcular embolization of vein of Galen aneurysms. J Neurosurg. 1986;64(5):731–5.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Clinic of Radiology and Neuroradiology, Sana Klinikum, Duisburg, Germany

    Fiedhelm Brassel

  2. Diagnostic and Interventional Radiology, Klinikum Stuttgart, Stuttgart, Germany

    Samuel Kobba

  3. Radiology, Heidelberg University Hospital, Heidelberg, Germany

    Christof M. Sommer

Authors
  1. Fiedhelm Brassel
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Samuel Kobba
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Christof M. Sommer
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Department of Neurosurgery, Iuliu Hațieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania

    Ioan Stefan Florian

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Brassel, F., Kobba, S., Sommer, C.M. (2020). Vein of Galen Aneurysmal Malformation. In: Florian, I.S. (eds) Pineal Region Lesions. Springer, Cham. https://doi.org/10.1007/978-3-030-50913-2_23

Download citation

Publish with us

Policies and ethics

Search

Navigation