Vein of Galen Malformation Clinical Features

Vein of Galen malformations may carry life-threatening and catastrophic sequelae in children. Their clinical presentations are different variables between cardiac and cerebral syndromes.

Newborns tend to present with congestive heart failure in the first few weeks of life (due to high blood flow) 1) and a cranial bruit. Hydrocephalus may result from obstruction of the Sylvian aqueduct by the enlarged Vein of Galen Malformation, or it may be caused by the increased venous pressure (which can also produce prominence of the scalp veins 2)). Parenchymatous AVMs are usually diagnosed later in life due to neurological manifestations, 3) including focal neurologic deficit and hemorrhage.

There may be a possible relationship between the Galenic angioarchitecture and the clinical presentation.

Significant stenosis (>70%) of the draining sinus is a significant factor for Vein of Galen malformation aneurysmal enlargement and the occurrence of hydrocephalus. Probably, venous outflow impairment decreases the incidence of high-output heart failure and increases the incidence of hydrocephalus 4).

Cummings GR. Circulation in neonates with intracranial arteriovenous fistula and cardiac failure. Am J Cardiol. 1980; 45:1019–1024
Strassburg HM. Macrocephaly is Not Always Due to Hydrocephalus. J Child Neurol. 1989; 4:S32–S40
Clarisse J, Dobbelaere P, Rey C, et al. Aneurysms of the great vein of Galen. Radiological-anatomical study of 22 cases. J Neuroradiol. 1978; 5:91–102
Hassan T, Sultan A, Ibrahim T, El-Saadany WF. The Impact of Angiographic Geometry on the Clinical Presentation of True Vein of Galen Aneurysmal Malformations. Pediatr Neurosurg. 2021 Jan 13:1-7. doi: 10.1159/000512213. Epub ahead of print. PMID: 33440394.
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  • Last modified: 2021/01/14 09:35
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