Management of Patient with Vein of Galen Malformation

  • Kiran Jangra
  • Sanket Agrawal
  • Shiv Lal Soni


A 5-month-old female child brought to the emergency room with the history of failure to thrive, excessive sweating, and unable to take the breastfeed in single sitting. The patient is a preterm neonate delivered by cesarean section for fetal distress, with a birth weight of 1.5 kg.


  1. 1.
    Drake CG. Cerebral arteriovenous malformations: considerations for and experience with surgical treatment in 166 cases. Clin Neurosurg. 1979;26:145–208.PubMedCrossRefPubMedCentralGoogle Scholar
  2. 2.
    Casasco A, Lylyk P, Hodes JE, Kohan G, Aymard A, Merland JJ. Percutaneous transvenous catheterization and embolization of vein of Galen aneurysms. Neurosurgery. 1991;28:260–6.PubMedCrossRefPubMedCentralGoogle Scholar
  3. 3.
    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;27:22–8.PubMedCrossRefPubMedCentralGoogle Scholar
  4. 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;31:109–28.PubMedCrossRefPubMedCentralGoogle Scholar
  5. 5.
    Berenstein A, Lasjaunias P. Arteriovenous fistulas of the brain. In: Surgical Neuroangiography 4. Endovascular treatment of cerebral lesions. Berlin: Springer; 1992. p. 267–317.CrossRefGoogle Scholar
  6. 6.
    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;33:315–33.PubMedPubMedCentralGoogle Scholar
  7. 7.
    Alvarez H, Garcia Monaco R, Rodesch G, Sachet M, Krings T, Lasjaunias P. Vein of galen aneurysmal malformations. Neuroimaging Clin N Am. 2007;17:189–206.PubMedCrossRefPubMedCentralGoogle Scholar
  8. 8.
    Lasjaunias PL, Chng SM, Sachet M, Alvarez H, Rodesch G, Garcia-Monaco R. The management of vein of Galen aneurysmal malformations. Neurosurgery. 2006;59:S184–94.PubMedCrossRefPubMedCentralGoogle Scholar
  9. 9.
    Litvak J, Yahr MD, Ransohoff J. Aneurysms of the great vein of Galen and midline cerebral arteriovenous anomalies. J Neurosurg. 1960;17:945–54.PubMedCrossRefPubMedCentralGoogle Scholar
  10. 10.
    Mortazavi MM, Griessenauer CJ, Foreman P, Bavarsad Shahripour R, Shoja MM, Rozzelle CJ, Tubbs RS, Fisher WS 3rd, Fukushima T. 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.PubMedCrossRefPubMedCentralGoogle Scholar
  11. 11.
    Lasjaunias P, Ter Brugge K, Berenstein A. Vein of Galen aneurysmal malformation, in: surgical Neuroangiography 3. Clinical and interventional aspects in children, ed 2. Berlin: Springer, 2006, Vol 3, pp 105–226.Google Scholar
  12. 12.
    Yasargil MG. Microneurosurgery IIIB. New York: Thieme Medical Publishers; 1988. p. 323–57.Google Scholar
  13. 13.
    Hoffman HJ. Malformations of the vein of Galen. In: Edwards MSB, Hoffmann HJ, editors. Current neurosurgical practice: cerebral vascular disease in children and adolescents. Baltimore: Lippincott, Williams and Wilkins; 1989. p. 239–46.Google Scholar
  14. 14.
    Crawford JM, Rossitch E Jr, Oakes WJ, Alexander E 3rd. Arteriovenous malformation of the great vein of Galen associated with patent ductus arteriosus. Childs Nerv Syst 1990;6:18–22.PubMedCrossRefPubMedCentralGoogle Scholar
  15. 15.
    Pellegrino PA, Milanesi O, Saia OS, Carollo C. Congestive heart failure secondary to cerebral arterio-venous fistula. Childs Nerv Syst. 1987;3:141–4.PubMedCrossRefPubMedCentralGoogle Scholar
  16. 16.
    Bhattacharya JJ, Thammaroj J. Vein of Galen malformations. J Neurol Neurosurg Psychiatry. 2003;74:142–4.CrossRefGoogle Scholar
  17. 17.
    Zerah M, Garcia-Monaco RG, Terbrugge K, Tardieu M, de Victor D, et al. Hydrodynamics in vein of Galen malformations. Childs Nerv Syst. 1992;8:111–7.PubMedCrossRefPubMedCentralGoogle Scholar
  18. 18.
    Sainte-Rose C, La Combe J, Pierre-Kahn A, Renier D, Hirsch JF. Intracranial venous sinus hypertension: cause or consequence of hydrocephalus in infants? J Neurosurg. 1984;60:727–36.PubMedCrossRefPubMedCentralGoogle Scholar
  19. 19.
    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. 1991;7:360–7.PubMedCrossRefPubMedCentralGoogle Scholar
  20. 20.
    Gulati S, Kalra V. An uncommon variety of vein of Galen malformation. Indian Pediatr. 2002;39:307–8.PubMedPubMedCentralGoogle Scholar
  21. 21.
    Gold AP, Ransohoff JR, Carter S. Vein of Galen malformation. Acta Neurol Scand. 1964;40:5–7.CrossRefGoogle Scholar
  22. 22.
    Kothari SS, Naik N, Juneja R, Saxena A. Aneurysm of vein of Galen in the neonates: report of four cases. Indian Heart J. 2001;53:499–502.PubMedPubMedCentralGoogle Scholar
  23. 23.
    Cumming GR. Circulation in neonates with intracranial arteriovenous fistula and cardiac failure. Am J Cardiol. 1980;45:1019–24.PubMedCrossRefPubMedCentralGoogle Scholar
  24. 24.
    Garcia-Monaco R, de Victor D, Mann C, Hannedouche A, Terbrugge K, Lasjaunias P. Congestive cardiac manifestations from cerebrocranial arteriov- enous shunts: endovascular management in 30 children. Childs Nerv Syst. 1991;7:48–52.PubMedCrossRefPubMedCentralGoogle Scholar
  25. 25.
    Lasjaunias P, Terbrugge K, Lopez Ibor L, Chiu M, Flodmark O, Chuang S, et al. The role of dural venous anomalies in vein of Galen aneurysms: report of six cases and review of the literature. AJNR Am J Neuroradiol. 1987;8:185–92.PubMedPubMedCentralGoogle Scholar
  26. 26.
    Vintzileos AM, Eisenfeld LI, Campbell WA, Herson VC, DiLeo PE, Chameides L. Prenatal ultrasonic diagnosis of arteriovenous malformation of the vein of Galen. Am J Perinatol. 1986;3:209–11.PubMedCrossRefPubMedCentralGoogle Scholar
  27. 27.
    Mendelson DB, Hertzanu Y, Butterworth A. In utero diagnosis of a vein of Galen aneurysm by ultrasound. Neuroradiology. 1984;26:417–8.CrossRefGoogle Scholar
  28. 28.
    Reiter AA, Huhta JC, Carpenter RJ Jr, Segall GK, Hawkins EP. Prenatal diagnosis of arteriovenous malformation of the vein of Galen. JCU. 1986;14:623–8.PubMedPubMedCentralGoogle Scholar
  29. 29.
    Hirsch JH, Cyr D, Eberhardt H, Zunk el D. Ultrasonographic diagnosis of an aneurysm of the vein of Galen in utero by duplex scanning. J Ultrasound Med. 1983;2:231–3.PubMedCrossRefPubMedCentralGoogle Scholar
  30. 30.
    Jeanty P, Kepple D, Roussis P, Shah D. In utero detection of cardiac failure from an aneurysm of the vein of Galen. Am J Obstet Gynecol. 1990;163:50–1.PubMedCrossRefPubMedCentralGoogle Scholar
  31. 31.
    Seidenwurm D, Berenstein A, Hyman A. Vein of Galen malformation: correlation of clinical presentation, arteriography and MR imaging. AJNR Am J Neuroradiol. 1991;12:347–54.PubMedPubMedCentralGoogle Scholar
  32. 32.
    Nikas DC, Proctor MR, Scott RM. Spontaneous thrombosis of vein of Galen aneurysmal malformation. Paediatr Neurosur g. 1999;31:33–9.CrossRefGoogle Scholar
  33. 33.
    Surana UM, Patel BN, Patel SB, Dhebar M. Images: vein of Galen malformation. Ind J R adiol Imag. 1999;9:21–2.Google Scholar
  34. 34.
    Johnston IH, Whittle IR, Besser M, Morgan MK. Vein of Galen malformation: diagnosis and management. Neurosurgery. 1987;20:747–58.PubMedCrossRefPubMedCentralGoogle Scholar
  35. 35.
    Hoffmann HJ, Chuang S, Hendrick EB, Humphreys RP. Aneurysms of the vein of Galen: Experience at the Hospital for Sick Children, Toronto. J Neurosurg. 1982;57:316–22.CrossRefGoogle Scholar
  36. 36.
    Diebler C, Dulac O, Renier D, Ernest C, Lalande G. Aneurysms of the vein of Galen in infants aged 2 to 15 months. Diagnosis and natural evolution. Neuroradiology. 1981;21:185–97.PubMedCrossRefPubMedCentralGoogle Scholar
  37. 37.
    Amachar AL, Shillito J Jr. The syndromes and surgical treatment of aneurysms of the great vein of Galen. J Neurosurg. 1973;39:89–98.CrossRefGoogle Scholar
  38. 38.
    Rao V, Mathuriya SN. Pediatric aneurysms and vein of Galen malformations. J Pediatr Neurosci. 2011;6:S109–17.PubMedPubMedCentralCrossRefGoogle Scholar
  39. 39.
    ter Brugge KG. Vein of Galen management in neonatal period. Am J Neuroradiol. 2001;22:1403–9.Google Scholar
  40. 40.
    Khullar D, Andeejani AM, Bulsara KR. Evolution of treatment options for vein of Galen malformations. A review. J Neurosurg Pediatr. 2010;6:444–51.PubMedCrossRefPubMedCentralGoogle Scholar
  41. 41.
    Lasjaunias P, Rodesch G, Terbrugge K, Pruvost P, Devictor D, Comoy J, et al. Vein of Galen aneurysmal malformations. Report of 36 cases managed between 1982 and 1988. Acta Neurochir (Wein). 1989;99:26–37.CrossRefGoogle Scholar
  42. 42.
    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;27:317–20.PubMedPubMedCentralGoogle Scholar
  43. 43.
    Lasjaunias P, Garcia-Monaco R, Rodesch G, Terbrugge K. Deep venous drainage in great cerebral vein (vein of Galen) absence and malformations. Neuroradiology. 1991;33:234–8.PubMedCrossRefPubMedCentralGoogle Scholar
  44. 44.
    Levrier O, Gailloud PH, Souei M, Manera L, Brunel H, Raybaud C. Normal galenic drainage of the deep cerebral venous system in two cases of vein of Galen aneurysmal malformation. Childs Nerv Syst. 2004;20:91–9.PubMedCrossRefPubMedCentralGoogle Scholar
  45. 45.
    Spezler RF, Wilson CB, Weinstein P. Normal perfusion pressure breakthrough theory. Clin Neurosurg. 1978;25:651–72.CrossRefGoogle Scholar
  46. 46.
    Young WL, Dowd CF. Interventional Neuroradiology: Anesthetic management. In: Cottrell JE, Young WL, editors. Cottrell and Young’s Neuroanesthesia. 5th ed. Philadelphia, PA: Mosby Elsevier; 2010. p. p247–63.CrossRefGoogle Scholar
  47. 47.
    Payne BR, Prasad D, Steiner M, Bunge H, Steiner L. Gamma surgery for vein of Galen malformations. J Neurosurg. 2000 Aug;93(2):229.PubMedCrossRefPubMedCentralGoogle Scholar
  48. 48.
    Hansen D, Kan PT, Reddy GD, Mohan AC, Jea A, Lam S. Pediatric knowledge update: approach to the management of vein of Galen aneurysmal malformations in neonates. Surg Neurol Int. 2016;7:S317–21.PubMedPubMedCentralCrossRefGoogle Scholar
  49. 49.
    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 Jan;120(1):120–5.PubMedCrossRefPubMedCentralGoogle Scholar
  50. 50.
    Shin M, Kurita H, Tago M, Kirino T. Stereotactic radiosurgery for tentorial dural arteriovenous fistulae draining into the vein of Galen: report of two cases. Neurosurgery. 2000;46(3):730–3.PubMedCrossRefPubMedCentralGoogle Scholar
  51. 51.
    Gailloud P, O’Riordan DP, Burger I, Levrier O, Jallo G, Tamargo RJ, Murphy KJ, Lehmann CU. Diagnosis and management of vein of Galen aneurysmal malformations. J Perinatol. 2005;25(8):542–51.PubMedCrossRefPubMedCentralGoogle Scholar
  52. 52.
    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. Ch Dis Child Fetal Neonatal Ed. 2002;87(2):F144–9.CrossRefGoogle Scholar
  53. 53.
    Thiex R, Williams A, Smith E, Scott RM, Orbach DB. The use of Onyx for embolization of central nervous system arteriovenous lesions in pediatric patients. Am J Neuroradiol. 2010;31(1):112–20.PubMedCrossRefPubMedCentralGoogle Scholar
  54. 54.
    Ashida Y, Miyahara H, Sawada H, Mitani Y, Maruyama K. Anesthetic management of a neonate with vein of Galen aneurysmal malformations and severe pulmonary hypertension. Paediatr Anaesth. 2005;15(6):525–8.PubMedCrossRefPubMedCentralGoogle Scholar
  55. 55.
    Landringan-Ossar M, McClain CD. Anesthesia for intervention radiology. Pediatr Anaesth. 2014;24(7):698–702.CrossRefGoogle Scholar
  56. 56.
    McClain CD, Landrigan-Ossar M. Challenges in pediatric neuroanaesthesia: Awake craniotomy, intraoperative magnetic resonance imaging, and interventional neuroradiology. Anesthesiol Clin. 2014;32(1):83–100.PubMedCrossRefPubMedCentralGoogle Scholar
  57. 57.
    Davidson AJ, Morton NS, Arnup SJ, de Graaff JC, Disma N, Withington DE, Frawley G, Hunt RW, Hardy P, Khotcholava M, von Ungern Sternberg BS, Wilton N, Tuo P, Salvo I, Ormond G, Stargatt R, Locatelli BG, ME MC. Postoperative apnea after awake regional and general anaesthesia in infants: the general anaesthesia compared to spinal anaesthesia study-comparing apnea and neurodevelopmental outcomes, a randomized controlled trial. Anesthesiology. 2015;123(1):38–54.PubMedPubMedCentralCrossRefGoogle Scholar
  58. 58.
    Ellis JA, Orr L, Ii PC, Anderson RC, Feldstein NA, Meyers PM. Cognitive and functional status after vein of Galen aneurysmal malformation endovascular occlusion. World J Radiol. 2012;4(3):83–9.PubMedPubMedCentralCrossRefGoogle Scholar
  59. 59.
    Deloison B, Chalouhi GE, Sonigo P, Zerah M, Millischer AE, Dumez Y, Brunelle F, Ville Y, Salomon LJ. Hidden mortality of prenatally diagnosed veon of Galen aneurysmal malformation: retrospective study and review of literature. Ultrasound Obstet Gynecol. 2012;40(6):652–8.PubMedCrossRefPubMedCentralGoogle Scholar

Copyright information

© Springer Nature Singapore Pte Ltd. 2020

Authors and Affiliations

  • Kiran Jangra
    • 1
  • Sanket Agrawal
    • 1
  • Shiv Lal Soni
    • 1
  1. 1.Department of Anaesthesia and Intensive CarePostgraduate Institute of Medical Education and ResearchChandigarhIndia

Personalised recommendations