J Pediatr Neurol 2019; 17(02): 045-056
DOI: 10.1055/s-0038-1635108
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Vein of Galen Aneurysmal Malformation: An Updated Review

Li-Rong Cao
1  Graduate College of Tianjin Medical University, Tianjin, China
Chun-Quan Cai
2  Department of Neurosurgery, Tianjin Children's Hospital, Tianjin, China
› Author Affiliations
Further Information

Publication History

16 October 2017

23 January 2018

Publication Date:
08 April 2018 (eFirst)


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.