Journal of Pediatric Neuroradiology 2013; 02(03): 237-261
DOI: 10.3233/PNR-13063
Review Article
Georg Thieme Verlag KG Stuttgart – New York

Advances in the diagnosis and management of vein of Galen malformations

Todd A. Abruzzo
a   Department of Neurosurgery, University of Cincinnati College of Medicine, Cincinnati, OH, USA
b   Department of Radiology, University of Cincinnati College of Medicine, Cincinnati, OH, USA
c   Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA
d   Mayfield Clinic, Cincinnati, OH, USA
,
Lincoln Jimenez
a   Department of Neurosurgery, University of Cincinnati College of Medicine, Cincinnati, OH, USA
b   Department of Radiology, University of Cincinnati College of Medicine, Cincinnati, OH, USA
,
Lauren Ostling
a   Department of Neurosurgery, University of Cincinnati College of Medicine, Cincinnati, OH, USA
,
Charles Stevenson
a   Department of Neurosurgery, University of Cincinnati College of Medicine, Cincinnati, OH, USA
c   Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA
,
Ravishankar Shivashankar
a   Department of Neurosurgery, University of Cincinnati College of Medicine, Cincinnati, OH, USA
b   Department of Radiology, University of Cincinnati College of Medicine, Cincinnati, OH, USA
,
James L. Leach
b   Department of Radiology, University of Cincinnati College of Medicine, Cincinnati, OH, USA
c   Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA
,
Blaise Jones
b   Department of Radiology, University of Cincinnati College of Medicine, Cincinnati, OH, USA
c   Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA
› Author Affiliations

Subject Editor:
Further Information

Publication History

10 October 2012

06 April 2013

Publication Date:
29 July 2015 (online)

Abstract

The vein of Galen malformations are a heterogeneous group of developmental arteriovenous shunt lesions characterized by arterialization of the early embryonic choroidal venous circulation. The arteriovenous shunt lesion manifests a spectrum of age dependent clinical phenotypes each of which expresses a wide range of disease severity. Clinical presentation is determined by lesion specific, host specific and environmental factors. In recent years, patient oriented treatment strategies directed at optimizing cardiovascular and neurodevelopmental outcomes have evolved. Treatment is tailored to address specific clinical phenotypes that are differentiated according to their pathophysiology. This paper will review current approaches to the diagnosis and treatment of vein of Galen malformations patients emphasizing clinical phenotypes of the fetus, neonate and infant.