Skip to main content
SpringerLink
Log in

Vein of Galen Aneurysmal Malformations

  • Living reference work entry
  • First Online:
Textbook of Pediatric Neurosurgery

  • 728 Accesses

Abstract

Vein of Galen aneurysmal malformations are special kind of AVMs, which occur mainly in the pediatric group of patients. VGAMs account for about 30% of vascular malformations in the childhood. This pathology has congenital nature and is characterized by the formation of arteriovenous shunt in the area of one of the main venous collector – a vein of Galen.

The main clinical manifestations are hypertension-hydrocephalic syndrome, delayed psychomotor development, and focal neurological symptoms.

VGAMs often combine with malformations of the heart and vascular system.

Currently, the standard diagnostics of VGAMs should include ultrasound, which allows to make accurate diagnosis already in the third trimester of pregnancy. More detailed information can be obtained by transcranial Doppler ultrasonography, computed tomography, magnetic resonance imaging, magnetic resonance angiography, and cerebral angiography.

At the present stage of development of vascular neurosurgery, the treatment of choice of VGAMs is endovascular surgery. The indications for endovascular treatment are the presence of available afferents of VGAM for the occlusion. The method of the occlusion must be selected individually for each patient after a thorough analysis of the angioarchitectonics of VGAM. For the best results of treatment, patients should undergo surgery within the first months of life.

Microsurgical resection of VGAMs is associated with a high risk of intraoperative complications. Radiosurgical treatment in the presence of dilatation of the vein of Galen and large afferents is ineffective. Shunting procedures can be performed in patients with VGAMs in some cases.

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

Access this chapter

Log in via an institution

Institutional subscriptions

References

  • Ahmad M, Mir S, Wahab S, Rizvi I (2012) Vein of Galen malformation. BMJ Case Rep 2012. pii: bcr2012006822. doi:10.1136/bcr-2012-006822

    Google Scholar 

  • Amacher AL, Shillito J Jr (1973) The syndromes and surgical treatment of aneurysms of the great vein of Galen. J Neurosurg 38:89–98

    Article  Google Scholar 

  • Bekov DB (1965) Atlas of human cerebral venous system. Moscow, p 359

    Google Scholar 

  • Berenstein A, Lasjaunias P (1992) Surgical Neuroangiography: 4 Endovascular treatment of cerebral lesions. Springer, Berlin/Heidelberg, pp 273–307

    Book  Google Scholar 

  • Bhattacharya JJ, Thammaroj J (2003) Vein of Galen malformations. J Neurol Neurosurg Psychiatry 74(Suppl 1):i42–i44

    Article  PubMed  PubMed Central  Google Scholar 

  • Blount JP, Oakes WJ, Tubbs RS, Humphreys RP (2006) History of surgery for cerebrovascular disease in children. Part II. Vein of Galen malformations. Neurosurg Focus 20(6):1–5

    Google Scholar 

  • Boldrey E, Miller ER (1949) Arteriovenous fistula (aneurysm) of the great cerebral vein (of Galen) and the circle of Willis; report on two patients treated by ligation. Arch Neurol Psychiatry 62(6):778

    Article  CAS  PubMed  Google Scholar 

  • Brunelle F (1997) Arteriovenous malformation of the vein of Galen in children. Pediatr Radiol 27(6):501–513

    Article  CAS  PubMed  Google Scholar 

  • Busing K, Kilian K, Schaible T, Neff W (2008) Prenatal magnetic resonance imaging of vein of Galen aneurysm associated with congestive heart failure. Pediatr Int 50(6):818–821

    Article  PubMed  Google Scholar 

  • Byrne JV (2012) Tutorials in endovascular neurosurgery and interventional neuroradiology. Springer, Berlin/Heidelberg, pp 219–228

    Book  Google Scholar 

  • Cai MJ, Ma LT, Yang M, Pan L (2012) Vein of Galen aneurysmal malformation: strategy transarterial embolization using coils alone. Neurol India 60(5):559–560

    Article  PubMed  Google Scholar 

  • Chevret L, Durand P, Alvarez H, Lambert V, Caeymax L, Rodesch G, Devictor D, Lasjaunias P (2002) 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 28(8):1126–1130

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  • Claireaux AE, Newman CGH (1960) Arteriovenous aneurysm of the great vein of Galen with heart failure in the neonatal period. Arch Dis Child 35:605–612

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Corrin R (1959) Three cases of intracranial vascular malformations in infants. J Clin Pathol 12:412–418

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Cumming GR (1980) Circulation in neonates with intracranial arteriovenous fistula and cardiac failure. Am J Cardiol 45(5):1019–1024

    Article  CAS  PubMed  Google Scholar 

  • Dandy WE (1928) Arteriovenous aneurysm of the brain. Arch Surg 17:190–243

    Article  Google Scholar 

  • Deloison B, Chalouhi GE, Sonigo P, Zerah M, Millischer AE, Dumez Y, Brunelle F, Ville Y, Salomon LJ (2012) Hidden mortality of prenatally diagnosed vein of Galen aneurysmal malformation: retrospective study and review of the literature. Ultrasound Obstet Gynecol 40(6):652–658

    Article  CAS  PubMed  Google Scholar 

  • Duvernoy HM, Delon S, Vannson JL (1981) Cortical blood vessels of the human brain. Brain Res Bull 7(5):519–579

    Article  CAS  PubMed  Google Scholar 

  • Ergenoglu MA, Yeniel AO, Akdemir A, Akercan F, Karadadas N (2013) Role of 3D power Doppler sonography in early prenatal diagnosis of Galen vein aneurysm. J Turk Ger Gynecol Assoc 14(3):178–181

    PubMed  PubMed Central  Google Scholar 

  • Frawley GP, Dargaville PA, Mitchell PJ, Tress BM, Loughnan P (2002) Clinical course and medical management of neonates with severe cardiac failure related to vein of Galen malformation. Arch Dis Child Fetal Neonatal Ed 87(2):F144–F149

    Article  CAS  PubMed  PubMed Central  Google Scholar 

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

    Article  PubMed  Google Scholar 

  • Glatt BS, Rowe RD (1960) Cerebral arteriovenous fistula associated with congestive heart failure in the newborn: report of two cases. Arch Dis Child 35:605–612

    Article  Google Scholar 

  • Gupta AK, Varma DR (2004) Vein of Galen malformations: review. Neurol India 52(1):43–53

    CAS  PubMed  Google Scholar 

  • Gupta A, Kanth Rao V, Varma D, Kapilamoorthy T, Kesavadas C, Krishnamoorthy T, Thomas B, Bodhey N, Purkayastha S (2006) Evaluation, management, and long-term follow up of vein of Galen malformations. J Neurosurg 105(1):26–33

    Article  PubMed  Google Scholar 

  • Hamilton MG, Herman JM, Khayata MH, Spetzler FR, (1996) Aneurysms of the vein of Galen. In: Youmans JR (ed) Neurological surgery, vol 2. Philadelphia: Saunders, pp 1491–1510

    Google Scholar 

  • Hassan T, Nassar M, Elghandour M (2010) Vein of Galen aneurysms: presentation and endovascular management. Pediatr Neurosurg 46(6):427–434

    Article  PubMed  Google Scholar 

  • Heuer GG, Gabel B, Beslow LA, Stiefel MF, Schwartz ES, Storm PB, Ichord RN, Hurst RW (2010) Diagnosis and treatment of vein of Galen aneurysmal malformations. Childs Nerv Syst 26(7):879–887

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  • Jaeger JR, Forbes RP, Dandy WE (1937) Bilateral congenital cerebral arteriovenous communication aneurysm. Trans Am Neurol Assoc 63:173–176

    Google Scholar 

  • Johnson W, Berry JM, Einzig S, Bass JL (1988) Doppler findings in nonimmune hydrops fetalis and cerebral arteriovenous malformation. Am Heart J 115(5):1138–1140

    Article  CAS  PubMed  Google Scholar 

  • Johnston IH, Whittle IR, Besser M, Morgan MK (1987) Vein of Galen malformation: diagnosis and management. Neurosurgery 20(5):747–758

    Article  CAS  PubMed  Google Scholar 

  • Jones BV, Ball WS, Tomsick TA, Millard J, Crone KR (2002) Vein of Galen aneurysmal malformation: diagnosis and treatment of 13 children with extended clinical follow-up. AJNR Am J Neuroradiol 23(10):1717–1724

    PubMed  Google Scholar 

  • Karadeniz L, Coban A, Sencer S, Has R, Ince Z, Can G (2011) Vein of Galen aneurysmal malformation: prenatal diagnosis and early endovascular management. J Chin Med Assoc 74(3):134–137

    Article  PubMed  Google Scholar 

  • Khullar D, Andeejani AM, Bulsara KR (2010) Evolution of treatment options for vein of Galen malformations. J Neurosurg Pediatr 6(5):444–451

    Article  PubMed  Google Scholar 

  • Kong JC, Cheng KM, Cheung YL, Chan CM (2012) Transarterial embolisation with Guglielmi detachable coils in an infant with a vein of Galen aneurysmal malformation. Hong Kong Med J 18:435–438

    PubMed  Google Scholar 

  • Konovalov AN, Blinkov SM, Putsillo MV (1990) Atlas of neurosurgical anatomy. Medicine, Moscow, pp 148–161

    Google Scholar 

  • Konovalov AN, Pitskhelauri DI, Arutiounov NV (2002) Surgical treatment of the thrombosed vein of galen aneurysm. Acta Neurochir 144(9):909–915; discussion 915

    Article  CAS  PubMed  Google Scholar 

  • Lasjaunias P (1997) Vascular diseases in neonates, infants and children. Springer, Berlin/Heidelberg

    Book  Google Scholar 

  • Lasjaunias P, TerBrugge K, Lopez Ibor L, Chiu M, Flodmark O, Chuan S, Goasguen J (1987) The role of dural anomalies in vein of Galen aneurysms: report of six cases and review of the literature. AJNR Am J Neuroradiol 8(2):185–192

    CAS  PubMed  Google Scholar 

  • Lasjaunias P, Chng SM, Sachet M, Alvarez H, Rodesch G, Garcia-Monaco R (2006) The management of vein of Galen aneurysmal malformations. Neurosurgery 59(5 Suppl 3):S184–S194; discussion S183–S113

    PubMed  Google Scholar 

  • Li AH, Armstrong D, TerBrugge KG (2011) Endovascular treatment of vein of Galen aneurysmal malformation: management strategy and 21-year experience in Toronto. J Neurosurg Pediatr 7(1):3–10

    Article  PubMed  Google Scholar 

  • Lylyk P, Vinuela F, Dion JE, Duckwiler G, Guglielmi G, Peacock W, Martin N (1993) Therapeutic alternatives for vein of Galen vascular malformations. J Neurosurg 78(3):438–445

    Article  CAS  PubMed  Google Scholar 

  • Meyers PM, Halbach VV, Phatouros CP, Dowd CF, Malek AM, Lempert TE, Lefler JE, Higashida RT (2000) Hemorrhagic complications in vein of Galen malformations. Ann Neurol 47(6):748–755

    Article  CAS  PubMed  Google Scholar 

  • Mortazavi MM, Griessenauer CJ, Foreman P, Bavarsad Shahripour R, Shoja MM, Rozzelle CJ, Tubbs RS, Fisher WS 3rd, Fukushima T (2013) Vein of Galen aneurysmal malformations: critical analysis of the literature with proposal of a new classification system. J Neurosurg Pediatr 12(3):293–306

    Article  PubMed  Google Scholar 

  • Mullan S, Mojtahedi S, Johnson DL, Macdonald RL (1996) Embryological basis of some aspects of cerebral vascular fistulas and malformations. J Neurosurg 85(1):1–8

    Article  CAS  PubMed  Google Scholar 

  • Padget DH (1948) The development of cranial arteries in the human embryo. Contr Embryol 32:205–261

    Google Scholar 

  • Pearl M, Gomez J, Gregg L, Gailloud P (2010) Endovascular management of vein of Galen aneurysmal malformations. Influence of the normal venous drainage on the choice of a treatment strategy. Childs Nerv Syst 26(10):1367–1379

    Article  PubMed  Google Scholar 

  • Pollock AQ, Laslett PA (1958) Cerebral arteriovenous fistula producing cardiac failure in the newborn infant. Case report and review. J Pediatr 53:731–736

    CAS  Google Scholar 

  • Pool JL (1968) Excision of cerebral arteriovenous malformation. J Neurosurg 29:312–321

    Article  CAS  PubMed  Google Scholar 

  • Putsillo MV, Vinokurov AG, Belov AI (2002) Neurosurgical anatomy. Antidor, Moscow

    Google Scholar 

  • Raybaud CA, Strother CM, Hald JK (1989) Aneurysms of the vein of Galen: embryonic considerations and anatomical features relating to the pathogenesis of the malformation. Neuroradiology 31:109–128

    Article  CAS  PubMed  Google Scholar 

  • Reiter AA, Huhta JC, Carpenter RJ, Segall GK, Hawkins EP (1986) Prenatal diagnosis of arteriovenous malformation of the vein of Galen. J Clin Ultrasound 14(8):623–628

    Article  CAS  PubMed  Google Scholar 

  • Sasidharan CK, Anoop P, Vijayakumar M, Jayakrishnan MP, Reetha G, Sindhu TG (2004) Spectrum of clinical presentations of vein of galen aneurysm. Indian J Pediatr 71(5):459–463

    Article  CAS  PubMed  Google Scholar 

  • Sepulveda W, Vanderheyden T, Pather J, Pasquini L (2003) Vein of galen malformation: prenatal evaluation with three-dimensional power Doppler angiography. J Ultrasound Med 22(12):1395–1398

    Article  PubMed  Google Scholar 

  • Vijayaraghavan SB, Vijay S, Kala MR, Neha D (2006) Prenatal diagnosis of thrombosed aneurysm of vein of Galen. Ultrasound Obstet Gynecol 27(1):81–83

    Article  PubMed  Google Scholar 

  • Volpe JJ (2008) Neurology of the newborn, 5th edn. Saunders Elsevier, Philadelphia, pp 996–1002

    Google Scholar 

  • Yan J, Wen J, Gopaul R, Zhang CY, Xiao SW (2015) Outcome and complications of endovascular embolization for vein of Galen malformations: a systematic review and meta-analysis. J Neurosurg 123(4):872–890

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Burdenko Neurosurgical Institute, Moscow, Russia

    Sergey Gorelyshev, Sergey Jakovlev & Adam Adaev

Authors
  1. Sergey Gorelyshev
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Sergey Jakovlev
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Adam Adaev
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Sergey Gorelyshev .

Editor information

Editors and Affiliations

  1. International Neuroscience Institute, Hannover, Germany

    Concezio Di Rocco

  2. Oakland Medical Center, Oakland, California, USA

    Dachling Pang

  3. University of Toronto Hospital for Sick Children, Toronto, Ontario, Canada

    James T. Rutka

Section Editor information

  1. Director of Pediatric Neurosurgery, International Neuroscience Institute, Hannover, Germany

    Concezio Di Rocco

Rights and permissions

Reprints and permissions

Copyright information

© 2017 Springer International Publishing AG

About this entry

Cite this entry

Gorelyshev, S., Jakovlev, S., Adaev, A. (2017). Vein of Galen Aneurysmal Malformations. In: Di Rocco, C., Pang, D., Rutka, J. (eds) Textbook of Pediatric Neurosurgery. Springer, Cham. https://doi.org/10.1007/978-3-319-31512-6_50-1

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-31512-6_50-1

  • Received:

  • Accepted:

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-31512-6

  • Online ISBN: 978-3-319-31512-6

  • eBook Packages: Springer Reference Biomedicine and Life SciencesReference Module Biomedical and Life Sciences

Publish with us

Policies and ethics

Search

Navigation