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Table of Contents    
Year : 2012  |  Volume : 60  |  Issue : 5  |  Page : 559-560

Vein of Galen aneurysmal malformation: Strategy transarterial embolization using coils alone

Department of Neurosurgery, Wuhan General Hospital of Guangzhou Command, People's Republic of China

Date of Web Publication3-Nov-2012

Correspondence Address:
Ming-Jun Cai
Department of Neurosurgery, Wuhan General Hospital of Guangzhou Command, People's Republic of China

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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.103227

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How to cite this article:
Cai MJ, Ma LT, Yang M, Pan L. Vein of Galen aneurysmal malformation: Strategy transarterial embolization using coils alone. Neurol India 2012;60:559-60

How to cite this URL:
Cai MJ, Ma LT, Yang M, Pan L. Vein of Galen aneurysmal malformation: Strategy transarterial embolization using coils alone. Neurol India [serial online] 2012 [cited 2020 Feb 23];60:559-60. Available from:


Vein of Galen aneurysmal malformations (VGAM) are rare congenital intracranial arteriovenous shunts and accounts for less than 1% of all arteriovenous malformations. They are characterized by multiple arteriovenous shunts draining into a dilated median prosencephalic vein (MProsV) of Markowski, and not the vein of Galen. [1],[2],[3]

A 15-month-old male child presented with seizures disorder, abnormally dilatation of the scalp and facial veins, and a mild increase in head circumference [Figure 1]a. Three-dimensional time-of-flight (3D TOF) magnetic resonance angiography (MRA) done at the age of 9 weeks revealed the VGAM [Figure 1]b. Three-dimensional computed tomographic angiography (3D CTA) at age 15 months demonstrated a mural-type VGAM with tortuous fistulas into the dilated draining MProsV of Markowski, and the embryonic falcine draining sinus was also visualized [Figure 1]c. Subsequent angiography showed two direct arteriovenous fistulas with high-flow communication supplied by posterior choroidal arteries originating from the left posterior cerebral artery [Figure 1]d. The lesion was embolized with multiple coils via transarterial approach two times at 15 and 18 months of age, respectively, which resulted in complete obliteration of the shunt without any complication [Figure 1]e. The child had an uneventful course. After 11 days of second session, there was no evidence of remaining shunt on the follow-up 3D CTA [Figure 1]f. His seizures could be controlled with antiepileptic medications, and at follow-up he did not develop any new symptom related to the malformation.
Figure 1: (a) Photograph of abnormally scalp and facial veins prominence. (b) 3D TOF MRA. (c) 3D CTA. (d) Pre-embolization vertebral artery angiogram of the anteroposterior views. (e) Postembolizations vertebral artery angiogram shows complete obliteration of the fistula with good patency of the posterior cerebral artery. (f) Follow-up 3D CTA at 11 days demonstrates no evidence of remaining shunt

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The first published description of a presumed VGAM was in 1895 by Steinheil as a varix aneurysm. [2] However, rather than a brain arteriovenous malformation of the true vein of Galen, VGAM represent an arteriovenous fistula with drainage to the MProsV of Markowski, the embryonic precursor of the vein of Galen itself, which is normally absent at the adult stage. [3] The current classification of VGAM has been simplified by Berenstein and Lajaunias into two types of angioarchitecture: [4] mural and choroidal. The mural type has a small number of direct arterial connections into the wall of the MProsV of Markowski. The choroidal type has multiple choroidal feeders that form a nidal network that drains into the MProsV of Markowski. Patients with a VGAM most commonly present with cardiac failure and neurologic complications. [4],[5] In neonates and infancy, clinical presentation is related to high-output congestive heart failure, hydrocephalus, and macrocephaly. Older children and adults usually present with symptoms of hydrocephalus, neuropsychomotor decline, seizures, and hemorrhage. [6]

Currently, endovascular therapy is the first-line treatment for VGAM and its associated sequelae. [7],[8] There are two main approaches for percutaneous embolization: transarterial and transvenous. Patients treated with a strategy of staged transarterial and transvenous embolizations can have excellent long-term cognitive and functional outcomes. Several studies have documented the efficacy and safety of endovascular treatment in these patients. [4],[6] The timing of endovascular management is determined by the clinical presentation. The use of the Bicetre scoring system developed by Lasjaunias and colleagues can help to guide the management of neonates with VGAM. [4] However, despite recent advances in endovascular techniques, the disability following treatment of these lesions continues to be high. [4] A multidisciplinary approach with individualized therapeutic goals, tailored to the unique clinical scenario, will ensure the best possible outcome for each patient. Anatomic cure of the VGAM is not the main aim of treatment; the ultimate aim is control of the malformation, to allow the brain to mature and develop normally, and the child to grow healthy. [4]

  Acknowledgment Top

The Author would like to thank the patient for providing consent to use her photograph in this article.

  References Top

1.Recinos PF, Rahmathulla G, Pearl M, Recinos VR, Jallo GI, Gailloud P, et al. Vein of Galen malformations: Epidemiology, clinical presentations, management. Neurosurg Clin N Am 2012;23:165-77.  Back to cited text no. 1
2.Dandy WE. Cerebrospinal fluid: Absorption. Chicago: American Medical Association; 1929. p. 2012.  Back to cited text no. 2
3.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.  Back to cited text no. 3
4.Alvarez H, Garcia Monaco R, Rodesch G, Sachet M, Krings T, Lasjaunias P. Vein of galen aneurysmal alformations. Neuroimaging Clin N Am 2007;17:189-206.  Back to cited text no. 4
5.Gupta AK, Varma DR. Vein of Galen malformations: Review. Neurol India 2004;52:43-53.  Back to cited text no. 5
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6.Berenstein A, Fifi JT, Niimi Y, Presti S, Ortiz R, Ghatan S, et al. Vein of Galen malformations in neonates: New management paradigms for improving outcomes. Neurosurgery 2012;70:1207-13.  Back to cited text no. 6
7.Johnston IH, Whittle IR, Besser M, Morgan MK. Vein of Galen aneurysmal malformation: Diagnosis and management. Neurosurgery 1987;20:747-58.  Back to cited text no. 7
8.Khullar D, Andeejani AM, Bulsara KR. Evolution of treatment options for vein of Galen malformations. J Neurosurg Pediatr 2010;6:444-51.  Back to cited text no. 8


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