Vein of Galen Malformation
- Author: Raj D Sheth, MD; Chief Editor: Amy Kao, MD more...
The vein of Galen is located under the cerebral hemispheres and drains the anterior and central regions of the brain into the sinuses of the posterior cerebral fossa. The vein of Galen aneurysmal malformation is a choroidal type of arteriovenous malformation involving the vein of Galen forerunner and is distinct from an arteriovenous malformation with venous drainage into a dilated, but already formed, vein of Galen.
Aneurysmal malformations of the vein of Galen (VGAM) typically result in high-output congestive heart failure or may present with developmental delay, hydrocephalus, and seizures.
Vein of Galen malformation (VGAM) results from an aneurysmal malformation with an arteriovenous shunting of blood. The congenital malformation develops during weeks 6-11 of fetal development as a persistent embryonic prosencephalic vein of Markowski; thus, VGAM is actually a misnomer. The vein of Markowski actually drains into the vein of Galen.
VGAM usually causes high-output heart failure in the newborn resulting from the decreased resistance and high blood flow in the lesion. Associated findings include cerebral ischemic changes such as strokes or steal phenomena that result in progressive hemiparesis. Hemorrhage from the malformation can occur, although this is not a common finding. Finally, the malformation may result in mass effects, causing progressive neurological impairment. Alternatively, the malformation may cause obstruction of the cerebrospinal fluid (CSF) outflow and result in hydrocephalus.
Vein of Galen malformation has been associated with capillary malformation-arteriovenous malformation (CM-AVM), which is a newly recognized autosomal dominant disorder, caused by mutations in the RASA1 gene in 6 families. The authors report severe intracranial AVMs, including vein of Galen aneurysmal malformation, which was symptomatic at birth or during infancy, extracranial AVM of the face and extremities, and Parkes Weber syndrome, previously considered sporadic and nongenetic.
The incidence of the vein of Galen malformation is unknown.
Infants often die if the high-output congestive heart failure is the presenting feature.
Macrocephaly usually improves following shunting for hydrocephalus.
VGAM is a congenital malformation; therefore, it may present at birth or in early childhood. It occurs in all races, and boys and girls are affected equally.
Fetuses with prenatally diagnosed VGAM have unexpectedly poor outcomes in the presence of cardiac or cerebral anomalies, while those with strictly isolated VGAM tend to have more favorable outcomes. Of 21 cases of prenatally diagnosed VGAM, 4 (19.0%) cases were isolated and 17 (81.0%) were associated with other anomalies. There were nine terminations (42.9%) and six neonatal deaths (28.6%).
Golombek SG, Ally S, Woolf PK. A newborn with cardiac failure secondary to a large vein of Galen malformation. South Med J. 2004 May. 97(5):516-8. [Medline].
Incorpora G, Pavone P, Platania N, Trifiletti RR, Parano E. Vein of Galen malformation and infantile spasms. J Child Neurol. 1999 Mar. 14(3):196-8. [Medline].
Revencu N, Boon LM, Mulliken JB, et al. Parkes Weber syndrome, vein of Galen aneurysmal malformation, and other fast-flow vascular anomalies are caused by RASA1 mutations. Hum Mutat. 2008 Jul. 29(7):959-65. [Medline].
Deloison B, Chalouhi GE, Sonigo P, Zerah M, Millischer AE, Dumez Y, et al. Hidden mortality of prenatally diagnosed vein of Galen aneurysmal malformation: retrospective study and review of the literature. Ultrasound Obstet Gynecol. 2012 Dec. 40 (6):652-8. [Medline].
Jagadeesan BD, Cross DT 3rd, Delgado Almandoz JE, et al. Susceptibility-Weighted Imaging: A New Tool in the Diagnosis and Evaluation of Abnormalities of the Vein of Galen in Children. AJNR Am J Neuroradiol. 2012 Apr 19. [Medline].
Gladstone DJ, Silver FL, Willinsky RA, Tyndel FJ, Wennberg R. Deep cerebral venous thrombosis: an illustrative case with reversible diencephalic dysfunction. Can J Neurol Sci. 2001 May. 28(2):159-62. [Medline].
Jee RC, Lin SH. Teaching NeuroImages: Reversible bilateral thalamic lesions in vein of Galen thrombosis. Neurology. 2009 Sep 22. 73(12):e57. [Medline].
Yan J, Wen J, Gopaul R, Zhang CY, Xiao SW. Outcome and complications of endovascular embolization for vein of Galen malformations: a systematic review and meta-analysis. J Neurosurg. 2015 Oct. 123 (4):872-90. [Medline].
Hoang S, Choudhri O, Edwards M, Guzman R. Vein of Galen malformation. Neurosurg Focus. 2009 Nov. 27(5):E8. [Medline].
Lasjaunias PL, Chng SM, Sachet M, Alvarez H, Rodesch G, Garcia-Monaco R. The management of vein of Galen aneurysmal malformations. Neurosurgery. 2006 Nov. 59(5 Suppl 3):S184-94; discussion S3-13. [Medline].
Lylyk P, Vinuela F, Dion JE, et al. Therapeutic alternatives for vein of Galen vascular malformations. J Neurosurg. 1993 Mar. 78(3):438-45. [Medline].
Moriarity JL Jr, Steinberg GK. Surgical obliteration for vein of Galen malformation: a case report. Surg Neurol. 1995 Oct. 44(4):365-9; discussion 369-70. [Medline].
Iizuka Y, Kakihara T, Suzuki M, Komura S, Azusawa H. 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 Pediatrics. 2008 Jan. 1(1):75-8. [Medline].
Feletti A, Denaro L, Marton E, d'Avella D, Longatti P. Endoscopic treatment of hydrocephalus due to aneurysm of the vein of Galen: case report and literature review. Minim Invasive Neurosurg. 2007 Oct. 50(5):285-91. [Medline].
Abend NS, Ichord R, Aijun Zhang, Hurst R. Vein of galen aneurysmal malformation with deep venous communication and subarachnoid hemorrhage. J Child Neurol. 2008 Apr. 23(4):441-6. [Medline].
Geibprasert S, Krings T, Armstrong D, Terbrugge KG, Raybaud CA. Predicting factors for the follow-up outcome and management decisions in vein of Galen aneurysmal malformations. Childs Nerv Syst. 2010 Jan. 26(1):35-46. [Medline].
de Koning TJ, Gooskens R, Veenhoven R, et al. Arteriovenous malformation of the vein of Galen in three neonates: emphasis on associated early ischaemic brain damage. Eur J Pediatr. 1997 Mar. 156(3):228-9. [Medline].
Porzionato A, Macchi V, Parenti A, De Caro R. Vein of Galen aneurysm: anatomical study of an adult autopsy case. Clin Anat. 2004 Sep. 17(6):458-62. [Medline].
Squires LA, Thomas S, Betz BW, Cottingham S. Vein of Galen malformation with diencephalic syndrome: a clinical pathologic report. J Child Neurol. 1998 Nov. 13(11):575-7. [Medline].