Skip to main content

Advertisement

SpringerLink
Log in

Predicting factors for the follow-up outcome and management decisions in vein of Galen aneurysmal malformations

  • Original Paper
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Purpose

Vein of Galen aneurysmal malformations (VGAMs) are choroidal arteriovenous malformations that develop during an early embryonic stage. Although recent reports have shown improved outcome for these patients, the overall outcome still is poor. In this study, we evaluated the clinical, imaging, and angiographic features that may predict the outcome in VGAM patients.

Methods

Twenty-five patients diagnosed with VGAM were reviewed for clinical symptoms, including neonatal scoring systems, imaging findings, angioarchitecture, treatment decision, initial treatment age, follow-up timing, and follow-up outcome.

Results

Factors that were significantly associated with a poor outcome (p < 0.05) included neurological symptoms at presentation, a medium-to-low overall neonatal score (<12/21), a very poor score (<2/5) in one (or more) categories, focal parenchymal changes, calcifications, tonsillar herniation, arterial steal, or more than two groups of multiple arterial feeders. The venous drainage pattern and treatment age were not significantly associated with the overall outcome.

Conclusions

The presence of multiple factors that are related with poor outcome may warrant withholding aggressive treatment, while a small subgroup of carefully selected patients without any of these factors who are clinically asymptomatic may have a good outcome even with conservative management and close follow-up. For all other patients in which treatment is considered, the optimal treatment time is at 4–5 months of age; however, urgent treatment, regardless of age, should be indicated in those that do not have permanent brain damage on imaging with deteriorating congestive heart failure, evidence of arterial steal, or progressive occlusion of the venous outflow.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6

Similar content being viewed by others

References

  1. Abend NS, Ichord R, Aijun Z, Hurst R (2008) Vein of Galen aneurysmal malformation with deep venous communication and subarachnoid hemorrhage. J Child Neurol 23:441–446

    Article  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  3. Andeweg J (1996) The anatomy of collateral venous flow from the brain and its value in aetiological interpretation of intracranial pathology. Neuroradiology 38:621–628

    Article  CAS  PubMed  Google Scholar 

  4. Aoyama Y, Kinoshita Y, Yokota A, Hamada T (2006) Neuronal damage in hydrocephalus and its restoration by shunt insertion in experimental hydrocephalus: a study involving the neurofilament-immunostaining method. J Neurosurg 104:332–339

    Article  PubMed  Google Scholar 

  5. Baldoli C, Righini A, Parazzini C, Scotti G, Triulzi F (2002) Demonstration of acute ischemic lesions in the fetal brain by diffusion magnetic resonance imaging. Ann Neurol 52:243–246

    Article  PubMed  Google Scholar 

  6. Beucher G, Fossey C, Belloy F, Richter B, Herlicoviez M, Dreyfus M (2005) Antenatal diagnosis and management of vein of Galen aneurysm: review illustrated by a case report. J Gynecol Obstet Biol Reprod (Paris) 34:613–619

    CAS  Google Scholar 

  7. Borthne A, Carteret M, Baraton J, Courtel J, Brunelle F (1997) Vein of Galen vascular malformations in infants: clinical, radiological and therapeutic aspect. Eur Radiol 7:1252–1258

    Article  CAS  PubMed  Google Scholar 

  8. Cherif A, Neji K, Sebai L, Ben Jemaa W, Bellagua I, Jebnoun S, Khrouf N (2007) Vein of Galen aneurysmal malformation: a neonatal case with unusual evolution. Arch Pediatr 14:893–896

    Article  CAS  PubMed  Google Scholar 

  9. 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 28:1126–1130

    Article  CAS  PubMed  Google Scholar 

  10. Del Bigio MR, da Silva MC, Drake JM, Tuor UI (1994) Acute and chronic cerebral white matter damage in neonatal hydrocephalus. Can J Neurol Sci 21:299–305

    PubMed  Google Scholar 

  11. Fayyaz A, Qureshi IA (2005) Vein of Galen aneurysm: antenatal diagnosis: a case report. J Pak Med Assoc 55:455–456

    PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  13. Gailloud P, O'Riordan DP, Burger I, Lehmann CU (2006) 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 27:317–320

    CAS  PubMed  Google Scholar 

  14. Grossman RI, Bruce DA, Zimmerman RA, Goldberg HI, Bilaniuk LT (1984) Vascular steal associated with vein of Galen aneurysm. Neuroradiology 26:381–386

    Article  CAS  PubMed  Google Scholar 

  15. Guimiot F, Garel C, Fallet-Bianco C, Menez F, Khung-Savatovsky S, Oury JF, Sebag G, Delezoide AL (2008) Contribution of diffusion-weighted imaging in the evaluation of diffuse white matter ischemic lesions in fetuses: correlations with fetopathologic findings. AJNR Am J Neuroradiol 29:110–115

    Article  CAS  PubMed  Google Scholar 

  16. Gupta AK, Rao VR, Varma DR, Kapilamoorthy TR, Kesavadas C, Krishnamoorthy T, Thomas B, Bodhey NK, Purkayastha S (2006) Evaluation, management, and long-term follow up of vein of Galen malformations. J Neurosurg 105:26–33

    Article  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  18. Hartung J, Heling KS, Rake A, Zimmer C, Chaoui R (2003) Detection of an aneurysm of the vein of Galen following signs of cardiac overload in a 22-week old fetus. Prenat Diagn 23:901–903

    Article  CAS  PubMed  Google Scholar 

  19. Iizuka Y, Kakihara T, Suzuki M, Komura S, Azusawa H (2008) Endovascular remodeling technique for vein of Galen aneurysmal malformations—angiographic confirmation of a connection between the median prosencephalic vein and the deep venous system. J Neurosurg Pediatr 1:75–78

    Article  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  21. 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 23:1717–1724

    PubMed  Google Scholar 

  22. Karam O, da Cruz E, Rimensberger PC (2009) VGAM induced high-flow congestive heart failure responsive to PGE1 infusion. Int J Cardiol 132:e60–e62

    Article  PubMed  Google Scholar 

  23. Lasjaunias P, Berenstein A, terBrugge K (2006) Surgical neuroangiography; clinical and interventional aspects in children. Springer, Berlin

    Google Scholar 

  24. Lasjaunias PL, Chng SM, Sachet M, Alvarez H, Rodesch G, Garcia-Monaco R (2006) The management of vein of Galen aneurysmal malformations. Neurosurgery 59:S184–S194 discussion S183–113

    Article  PubMed  Google Scholar 

  25. Levrier O, Gailloud PH, Souei M, Manera L, Brunel H, Raybaud C (2004) Normal galenic drainage of the deep cerebral venous system in two cases of vein of Galen aneurysmal malformation. Childs Nerv Syst 20:91–97 discussion 98–99

    Article  CAS  PubMed  Google Scholar 

  26. Misra S, Fu AA, Puggioni A, Karimi KM, Mandrekar JN, Glockner JF, Juncos LA, Anwer B, McGuire AM, Mukhopadhyay D (2008) Increased shear stress with upregulation of VEGF-A and its receptors and MMP-2, MMP-9, and TIMP-1 in venous stenosis of hemodialysis grafts. Am J Physiol Heart Circ Physiol 294:H2219–H2230

    Article  CAS  PubMed  Google Scholar 

  27. Mitchell PJ, Rosenfeld JV, Dargaville P, Loughnan P, Ditchfield MR, Frawley G, Tress BM (2001) Endovascular management of vein of Galen aneurysmal malformations presenting in the neonatal period. AJNR Am J Neuroradiol 22:1403–1409

    CAS  PubMed  Google Scholar 

  28. Nuutila M, Saisto T (2008) Prenatal diagnosis of vein of Galen malformation: a multidisciplinary challenge. Am J Perinatol 25:225–227

    Article  PubMed  Google Scholar 

  29. Quisling RG, Mickle JP (1989) Venous pressure measurements in vein of Galen aneurysms. AJNR Am J Neuroradiol 10:411–417

    CAS  PubMed  Google Scholar 

  30. 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 

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

    Article  CAS  PubMed  Google Scholar 

  32. Rollins N, Booth T, Shapiro K (2000) MR venography in children with complex craniosynostosis. Pediatr Neurosurg 32:308–315

    Article  CAS  PubMed  Google Scholar 

  33. Ruano R, Benachi A, Aubry MC, Brunelle F, Dumez Y, Dommergues M (2003) Perinatal three-dimensional color power Doppler ultrasonography of vein of Galen aneurysms. J Ultrasound Med 22:1357–1362

    PubMed  Google Scholar 

  34. Santo S, Pinto L, Clode N, Cardoso E, Marques JP, Melo A, Cardoso C, Graca LM (2008) Prenatal ultrasonographic diagnosis of vein of Galen aneurysms—report of two cases. J Matern Fetal Neonatal Med 21:209–211

    Article  PubMed  Google Scholar 

  35. Staberg M, Jonsbo F (2007) Vein of Galen aneurysm diagnosed in the perinatal period. A retrospective assessment. Ugeskr Laeger 169:3190–3193

    PubMed  Google Scholar 

  36. Watban JA, Rodesch G, Alvarez H, Lasjaunias P (1995) Transarterial embolization of vein of Galen aneurysmal malformation after unsuccessful stereotactic radiosurgery. Report of three cases. Childs Nerv Syst 11:406–408

    Article  CAS  PubMed  Google Scholar 

  37. Zerah M, Garcia-Monaco R, Rodesch G, Terbrugge K, Tardieu M, de Victor D, Lasjaunias P (1992) Hydrodynamics in vein of Galen malformations. Childs Nerv Syst 8:111–117 discussion 117

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Neuroradiology Division, Department of Diagnostic Imaging, Hospital for Sick Children, 555 University Avenue, Toronto, ON, Canada, M5G 1X8

    Sasikhan Geibprasert, Derek Armstrong & Charles A. Raybaud

  2. Division of Neuroradiology, Department of Medical Imaging, Toronto Western Hospital, University of Toronto, Toronto, ON, Canada

    Timo Krings & Karel G. terBrugge

Authors
  1. Sasikhan Geibprasert
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Timo Krings
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Derek Armstrong
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Karel G. terBrugge
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Charles A. Raybaud
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Sasikhan Geibprasert.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Geibprasert, S., Krings, T., Armstrong, D. et al. Predicting factors for the follow-up outcome and management decisions in vein of Galen aneurysmal malformations. Childs Nerv Syst 26, 35–46 (2010). https://doi.org/10.1007/s00381-009-0959-7

Download citation

  • Received:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-009-0959-7

Keywords

Search

Navigation