Vein of Galen Aneurysmal Malformation: An Updated Review

Abstract

Vein of Galen aneurysmal malformation (VGAM) is a rare intracranial vascular anomaly in the choroidal arteries characterized by the persistence of the embryonic median prosencephalic vein of Markowski (MProsV). It is common in children with high mortality and disability rates. VGAM-associated clinical presentations include heart failure, hydrocephalus, and neurological symptoms, which make it difficult for diagnosis. Simultaneously, the complex VGAM vascular architecture, characterized by a wide interindividual variability, makes the management of this condition challenging to clinicians. Thus, timely diagnosis and treatment of VGAM are crucial. In this review article, we elucidated the latest research progresses of VGAM in the aspects of the pathogenesis, classification, clinical features, imaging, management, and prognosis based on recent relevant literatures. It is beneficial to raise the awareness of VGAM and improve the level of management and treatment.

Temporal bone hemangioendothelioma as a rare vascular tumor in childhood: case report and review of the literature

“…The main manifestations include congestive heart failure, hydrocephalus, and neurological symptoms. 22 Our patient was asymptomatic and galen vein aneursym was found incidentally on cranial MRI. According to our knowledge, galen vein aneursym and temporal bone hemagioendothelioma have not been reported in the same patient before.…”

“…Its incidence is 1/25000 and constitutes 1% of all cerebral vascular malformations and 30% of vascular malformations in children. 22 Clinical presentations vary with the age of onset and vascular architecture. The main manifestations include congestive heart failure, hydrocephalus, and neurological symptoms.…”

Perioperative Considerations in Patients with Vein of Galen Malformations Undergoing Embolization–A Single-Institution Case Series

“…[2][3][4] Without treatment, mortality is over 90% by infancy, though with recent advances in endovascular technique this has been reduced to 11%. 2,4 These children are at high risk for perioperative complications, with only a few specialized centers encountering them on an annual basis. We describe our perioperative experience with these patients who present for neuroembolization.…”

“…1,2 The clinical presentation varies depending on the age, with high-output heart failure (HF), pulmonary hypertension (PH), and/or multiorgan failure seen mainly in neonates. [2][3][4] Without treatment, mortality is over 90% by infancy, though with recent advances in endovascular technique this has been reduced to 11%. 2,4 These children are at high risk for perioperative complications, with only a few specialized centers encountering them on an annual basis.…”