Vein of Galen aneurysmal malformation (VGAM)

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Vein of Galen aneurysmal malformation (VGAM). This is a congenital vascular malformation. Fully referenced neurology checklist.

Anatomy

  • This is a congenital vascular malformation
  • It results from the persistence of the vein of Markowski
  • This is the embryonic median prosencephalic vein
  • It may be caused by mutations in the EPHB4 gene

Epidemiological features

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Lasjaunias classification

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Yasargil angiographic classification

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Clinical presentation

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Complications

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Magnetic resonance imaging (MRI) features

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Investigational tests

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Treatment

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Poor prognostic features in foetal cases

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Other pineal vascular malformations

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References

  1. Smirniotopoulos JG, Rushing EJ, Mena H. Pineal region masses: differential diagnosis. Radiographics 1992; 12:577-596.
  2. Moon JH, Cho WS, Kang HS, Kim JE, Lee SJ, Han MH. Vein of galen aneurysmal malformation: endovascular management of 6 cases in a single institute. J Korean Neurosurg Soc 2011; 50:191-194.
  3. Vivanti A, Ozanne A, Grondin C, et al. Loss of function mutations in EPHB4 are responsible for vein of Galen aneurysmal malformation. Brain 2018; 14:979-988.
  4. Jones BV, Ball WS, Tomsick TA, Millard J, Crone KR. Vein of Galen aneurysmal malformation: diagnosis and treatment of 13 children with extended clinical follow-up. Am J Neuroradiol 2002; 23:1717-1724.
  5. Chow ML, Cooke DL, Fullerton HJ, et al. Radiological and clinical features of vein of Galen malformations. J Neurointerv Surg 2015; 7:443-448.
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