Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • State of the Art
  • Published:

Diagnosis and Management of Vein of Galen Aneurysmal Malformations

Abstract

Vein of Galen aneurysmal malformations (VGAM) are rare intracranial vascular anomalies typically found in children. The anatomic landmark of a VGAM is the presence of multiple arteriovenous shunts draining into a dilated median prosencephalic vein, an embryonic vessel normally absent at the adult stage. This article reviews the developmental anatomy, the clinical presentation, and the current management of VGAM.

This is a preview of subscription content, access via your institution

Access options

Buy this article

Prices may be subject to local taxes which are calculated during checkout

Figure 1
Figure 2
Figure 3
Figure 4
Figure 5
Figure 6

References

  1. Raybaud CA, Strother CM . Persisting abnormal embryonic vessels in intracranial arteriovenous malformations. Acta Radiol Suppl 1986;369:136–138.

    CAS  PubMed  Google Scholar 

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

    Google Scholar 

  3. Long DM, Seljeskog EL, Chou SN, French LA . Giant arteriovenous malformations of infancy and childhood. J Neurosurg 1974;40:304–312.

    Article  CAS  Google Scholar 

  4. 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;31:109–128.

    Article  CAS  Google Scholar 

  5. Raybaud CA . Development of the arterial supply to the brain tissue. In: Lasjaunias P, Berenstein A, editors. Surgical Neuroangiography, Vol. 3. New York: Springer Verlag; 1990. p. 1–13.

    Google Scholar 

  6. Markowski J . Entwicklung der Sinus durae matris und der Hirnvenen des Menschen. Bulletin international de l’Académie des Sciences et des Lettres. Classe des sciences mathematiques et naturelles. Serie B: sciences naturelles 1921; (Numéro supplémentaire):1–269.

  7. Wolfram-Gabel R, Maillot C, Koritke JG . The vascular pattern in the tela choroidea of the prosencephalon in man. J Neuroradiol 1987;14:10–26.

    CAS  PubMed  Google Scholar 

  8. Levrier O, Gailloud P, Souei M, Manera L, Brunel H, Raybaud CA . Normal galenic drainage of the deep cerebral venous system in 2 cases of vein of Galen aneurysmal malformation (VGAM). Childs Nerv Syst 2004;20:91–97.

    Article  CAS  Google Scholar 

  9. 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 (in press).

  10. Lasjaunias P, Terbrugge K, Piske R, Lopez Ibor L, Manelfe C . Dilatation of the vein of Galen. Anatomoclinical forms and endovascular treatment apropos of 14 cases explored and/or treated between 1983 and 1986. Neurochirurgie 1987;33:315–333.

    CAS  PubMed  Google Scholar 

  11. Taylor GA . Intracranial venous system in the newborn: evaluation of normal anatomy and flow characteristics with color Doppler US. Radiology 1992;183:449–452.

    Article  CAS  Google Scholar 

  12. Alberico RA, Barnes P, Robertson RL, Burrows PE . Helical CT angiography: dynamic cerebrovascular imaging in children. AJNR Am J Neuroradiol 1999;20:328–334.

    CAS  PubMed  Google Scholar 

  13. Jordan L, Raymond G, Lin D, Gailloud P . CT angiography in a newborn child with hydranencephaly. J Perinatol 2004;24:565–567.

    Article  Google Scholar 

  14. Brunelle F . Brain vascular malformations in the fetus: diagnosis and prognosis. Childs Nerv Syst 2003;19:524–528.

    Article  Google Scholar 

  15. Lasjaunias P . Vein of Galen aneurysmal malformation. In: Vascular Diseases in Neonates, Infants and Children. Berlin-Heidelberg: Springer Verlag; 1997. p. 67–202.

    Chapter  Google Scholar 

  16. Gold AP, Ransohoff J, Carter S . Vein of Galen malformation. Acta Neurol Scand 1964;40 (Suppl. 11):1–31.

    Google Scholar 

  17. Rodesch G, Hui F, Alvarez H, Tanaka A, Lasjaunias P . Prognosis of antenatally diagnosed vein of Galen aneurysmal malformations. Childs Nerv Syst 1994;10:79–83.

    Article  CAS  Google Scholar 

  18. Zerah M, Garcia-Monaco R, Rodesch G, et al. Hydrodynamics in vein of Galen malformations. Childs Nerv Syst 1992;8:111–117; discussion 117.

    Article  CAS  Google Scholar 

  19. Halbach VV, Dowd CF, Higashida RT, Balousek PA, Ciricillo SF, Edwards MS . Endovascular treatment of mural-type vein of Galen malformations. J Neurosurg 1998;89:74–80.

    Article  CAS  Google Scholar 

  20. Mitchell PJ, Rosenfeld JV, Dargaville P, et al. Endovascular management of vein of Galen aneurysmal malformations presenting in the neonatal period. AJNR Am J Neuroradiol 2001;22:1403–1409.

    CAS  PubMed  Google Scholar 

  21. Lasjaunias P, Garcia-Monaco R, Rodesch G, et al. Vein of Galen malformation. Endovascular management of 43 cases. Childs Nerv Syst 1991;7:360–367.

    Article  CAS  Google Scholar 

  22. Casasco A, Lylyk P, Hodes JE, Kohan G, Aymard A, Merland JJ . Percutaneous transvenous catheterization and embolization of vein of galen aneurysms. Neurosurgery 1991;28:260–266.

    Article  CAS  Google Scholar 

  23. 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;11:643–648.

    CAS  PubMed  Google Scholar 

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

    Article  CAS  Google Scholar 

  25. Chevret L, Durand P, Alvarez H, 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;28:1126–1130.

    Article  CAS  Google Scholar 

  26. Hendson L, Emery DJ, Phillipos EZ, Bhargava R, Olley PM, Lemke RP . Persistent pulmonary hypertension of the newborn presenting as the primary manifestation of intracranial arteriovenous malformation of the Vein of Galen. Am J Perinatol 2000;17:405–410.

    Article  CAS  Google Scholar 

  27. Dahdah NS, Alesseh H, Dahms B, Saker F . Severe pulmonary hypertensive vascular disease in two newborns with aneurysmal vein of galen. Pediatr Cardiol 2001;22:538–541.

    Article  CAS  Google Scholar 

  28. McElhinney DB, Halbach VV, Silverman NH, Dowd CF, Hanley FL . Congenital cardiac anomalies with vein of Galen malformations in infants. Arch Dis Child 1998;78:548–551.

    Article  CAS  Google Scholar 

  29. Huh J, Noh CI, Choi JY, Yun YS . Pulmonary hypertensive crisis as an initial manifestation of intracranial arteriovenous malformation with aneurysm of the vein of Galen. Int J Cardiol 1998;66:107–109.

    Article  CAS  Google Scholar 

  30. Garcia-Monaco R, De Victor D, Mann C, Hannedouche A, Terbrugge K, Lasjaunias P . Congestive cardiac manifestations from cerebrocranial arteriovenous shunts. Endovascular management in 30 children. Childs Nerv Syst 1991;7:48–52.

    Article  CAS  Google Scholar 

  31. 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;91:583–586.

    CAS  PubMed  Google Scholar 

  32. Friedman DM, Madrid M, Berenstein A, Choi IS, Wisoff JH . Neonatal vein of Galen malformations: experience in developing a multidisciplinary approach using an embolization treatment protocol. Clin Pediatr (Phila) 1991;30:621–629.

    Article  CAS  Google Scholar 

  33. Lasjaunias P, Alvarez H, Rodesch G, et al. Aneurysmal malformations of the vein of Galen: follow-up of 120 children treated between 1984 and 1994. Int Neuroradiol 1996;2:15–26.

    Article  CAS  Google Scholar 

  34. Fullerton HJ, Aminoff AR, Ferriero DM, Gupta N, Dowd CF . Neurodevelopmental outcome after endovascular treatment of vein of Galen malformations. Neurology 2003;61:1386–1390.

    Article  CAS  Google Scholar 

  35. Deciding to forego life extending treatment: ethical, medical, and legal issues in treatment of decisions. In: President's Commission for the Study of Ethical Problems in Medicine and Biomedical and Behavioral Research. Washington: Government Printing Office; 1983. p. 121–146.

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Gailloud, P., O'Riordan, D., Burger, I. et al. Diagnosis and Management of Vein of Galen Aneurysmal Malformations. J Perinatol 25, 542–551 (2005). https://doi.org/10.1038/sj.jp.7211349

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/sj.jp.7211349

This article is cited by

Search

Quick links