====== Vein of Galen Malformation Clinical Features ======

[[Vein of Galen malformation]]s may carry life-threatening and catastrophic sequelae in [[child]]ren. Their clinical presentations are different variables between cardiac and cerebral [[syndrome]]s.

[[Newborn]]s tend to present with [[congestive heart failure]] in the first few weeks of life (due to high blood flow)
((Cummings GR. Circulation in neonates with intracranial arteriovenous fistula and cardiac failure. Am J Cardiol. 1980; 45:1019–1024))
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
((Strassburg HM. Macrocephaly is Not Always Due to Hydrocephalus. J Child Neurol. 1989; 4:S32–S40))).
Parenchymatous AVMs are usually diagnosed later in life due to neurological manifestations,
((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))
including focal neurologic deficit and hemorrhage.
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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]]
((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.)).

===== References =====