Abstract
Vein of Galen aneurysmal malformation (VGAM) represents up to 30% of vascular malformations in the pediatric age group. Described in the late 1980s, this entity was associated with a high rate of hydrocephalus and poor prognosis. This work presents the hydrocephalus physiopathology in patients with a VGAM and the modern care of this frequent complication.
If aqueduct stenosis may explain the abnormal accumulation of cerebrospinal fluid in some patients harboring a VGAM, high venous pressure is the most likely predominant physiopathologic mechanism: immature Pacchioni granulations cannot drain the brain during infancy, and the elevated venous pressure leads to a congestive status of the brain.
Thus, the first-line treatment is nowadays embolization, ideally at 5 months. Embolization normalizes venous pressure and treats the hydrocephalus. If emergency treatment is required, endoscopic third ventriculostomy can resolve mechanical compression of the aqueduct. Ventriculoperitoneal shunting, associated with poor outcomes, is not a first-line therapy.
References
Agee OF, Musella R, Tweed CG (1969) Aneurysm of the great vein of Galen. Report of two cases. J Neurosurg 31(3):346–351
Askenasy HM, Herzberger EE, Wijsenbeek HS (1953) Hydrocephalus with vascular malformations of the brain; a preliminary report. Neurology 3(3):213–220
Bateman GA, Alber M, Schuhmann MU (2014) An association between external hydrocephalus in infants and reversible collapse of the venous sinuses. Neuropediatrics 45(3):183–187
Bederson JB, Wiestler OD, Brüstle O, Roth P, Frick R, Yaşargil MG (1991) Intracranial venous hypertension and the effects of venous outflow obstruction in a rat model of arteriovenous fistula. Neurosurgery 29(3):341–350
Beggs CB (2013) Venous hemodynamics in neurological disorders: an analytical review with hydrodynamic analysis. BMC Med 11:142
Chen Q, Feng Z, Tan Q, Guo J, Tang J, Tan L et al (2017) Post-hemorrhagic hydrocephalus: recent advances and new therapeutic insights. J Neurol Sci 375:220–230
Chow ML, Cooke DL, Fullerton HJ, Amans MR, Narvid J, Dowd CF et al (2015) Radiological and clinical features of vein of Galen malformations. J NeuroInterv Surg 7(6):443–448
d’Avella D, Causin F (2016) Hydrocephalus in vein of Galen malformation. Another paradigm shift in neurosurgery. Acta Neurochir (Wien) 158(7):1285–1288
Dandy WE (1919) Experimental hydrocephalus. Ann Surg 70(2):129–142
de Lange S, de Vlieger M (1970) Hydrocephalus associated with raised venous pressure. Dev Med Child Neurol Suppl 22(Suppl 22):28
Deloison B, Chalouhi GE, Sonigo P, Zerah M, Millischer AE, Dumez Y et al (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
Di Rocco C (1991) Vein of Galen aneurysm and hydrocephalus. Childs Nerv Syst 7(7):359
Diebler C, Dulac O, Renier D, Ernest C, Lalande G (1981) Aneurysms of the vein of Galen in infants aged 2 to 15 months. Diagnosis and natural evolution. Neuroradiology 21(4):185–197
Feletti A, Denaro L, Marton E, d’Avella D, Longatti P (2007) Endoscopic treatment of hydrocephalus due to aneurysm of the vein of Galen: case report and literature review. Minim Invasive Neurosurg 50(5):285–291
Girard N, Lasjaunias P, Taylor W (1994) Reversible tonsillar prolapse in vein of Galen aneurysmal malformations: report of eight cases and pathophysiological hypothesis. Childs Nerv Syst 10(3):141–147
Grossman RI, Bruce DA, Zimmerman RA, Goldberg HI, Bilaniuk LT (1984) Vascular steal associated with vein of Galen aneurysm. Neuroradiology 26(5):381–386
Haacke EM, Beggs CB, Habib C (2012) The role of venous abnormalities in neurological disease. Rev Recent Clin Trials 7(2):100–116
Hammock MK, Milhorat TH, Earle K, Di Chiro G (1971) Vein of Galen ligation in the primate. Angiographic, gross, and light microscopic evaluation. J Neurosurg 34(1):77–83
Hooper R (1961) Hydrocephalus and obstruction of the superior vena cava in infancy. Clinical study of the relationship between cerebrospinal fluid pressure and venous pressure. Pediatrics 28:792–799
Jea A, Bradshaw TJ, Whitehead WE, Curry DJ, Dauser RC, Luerssen TG (2010) The high risks of ventriculoperitoneal shunt procedures for hydrocephalus associated with vein of Galen malformations in childhood: case report and literature review. Pediatr Neurosurg 46(2):141–145
Johnston IH, Whittle IR, Besser M, Morgan MK (1987) Vein of Galen malformation: diagnosis and management. Neurosurgery 20(5):747–758
Khullar D, Andeejani AMI, Bulsara KR (2010) Evolution of treatment options for vein of Galen malformations. J Neurosurg Pediatr 6(5):444–451
Lasjaunias P (1997) Vascular diseases in neonates, infants and children. Interventional neuroradiology management. Springer, Berlin
Lasjaunias PL, 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 S3–S13
Li A-H, 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
Meila D, Grieb D, Melber K, Jacobs C, Maslehaty H, Petridis A et al (2016) Hydrocephalus in vein of Galen malformation: etiologies and therapeutic management implications. Acta Neurochir (Wien) 158(7):1279–1284
Mickle JP, Quisling RG (1986) The transtorcular embolization of vein of Galen aneurysms. J Neurosurg 64(5):731–735
Payne BR, Prasad D, Steiner M, Bunge H, Steiner L (2000) Gamma surgery for vein of Galen malformations. J Neurosurg 93(2):229–236
Pearl M, Gregg L, Gandhi D (2011) Cerebral venous development in relation to developmental venous anomalies and vein of Galen aneurysmal malformations. Semin Ultrasound CT MR 32(3):252–263
Portnoy HD, Branch C, Castro ME (1994) The relationship of intracranial venous pressure to hydrocephalus. Childs Nerv Syst 10(1):29–35
Quisling RG, Mickle JP (1989) Venous pressure measurements in vein of Galen aneurysms. AJNR Am J Neuroradiol 10(2):411–417
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(2):109–128
Recinos PF, Rahmathulla G, Pearl M, Recinos VR, Jallo GI, Gailloud P et al (2012) Vein of Galen malformations: epidemiology, clinical presentations, management. Neurosurg Clin N Am 23(1):165–177
Rhoton AL (2002) The cerebral veins. Neurosurgery 51(4 Suppl):S159–S205
Ross DA, Walker J, Edwards MS (1986) Unusual posterior fossa dural arteriovenous malformation in a neonate: case report. Neurosurgery 19(6):1021–1024
Sainte-Rose C, LaCombe J, Pierre-Kahn A, Renier D, Hirsch JF (1984) Intracranial venous sinus hypertension: cause or consequence of hydrocephalus in infants? J Neurosurg 60(4):727–736
Schneider SJ, Wisoff JS, Epstein FJ (1992) Complications of ventriculoperitoneal shunt procedures or hydrocephalus associated with vein of Galen malformations in childhood. Neurosurgery 30(5):706–708
Ustun C (2004) Galen and his anatomic eponym: vein of Galen. Clin Anat 17(6):454–457
Williams H (2008) The venous hypothesis of hydrocephalus. Med Hypotheses 70(4):743–747
Yan J, Wen J, Gopaul R, Zhang C-Y, Xiao S (2015) Outcome and complications of endovascular embolization for vein of Galen malformations: a systematic review and meta-analysis. J Neurosurg 123(4):872–890
Zerah M, Garcia-Monaco R, Rodesch G, Terbrugge K, Tardieu M, de Victor D et al (1992) Hydrodynamics in vein of Galen malformations. Childs Nerv Syst 8(3):111–117; discussion 117
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Zanello, M., Zerah, M., Di Rocco, F. (2018). Hydrocephalus in Vein of Galen Malformation. In: Cinalli, G., Ozek, M., Sainte-Rose, C. (eds) Pediatric Hydrocephalus. Springer, Cham. https://doi.org/10.1007/978-3-319-31889-9_67-1
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DOI: https://doi.org/10.1007/978-3-319-31889-9_67-1
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