Advances in the diagnosis and management of vein of Galen malformations

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.

Rapid ventricular overdrive pacing and other advanced flow-control techniques for the endovascular embolization of vein of galen malformations

“…Since the first report of successful endovascular embolization of a VOGM in 1989 ( 2 ), endovascular techniques have significantly evolved and endovascular embolization is now universally considered the primary definitive treatment modality for these lesions ( 1 , 3 , 4 ). The technical goal of treatment is effective and durable disconnection of individual high-flow arteriovenous shunts within the VOGM.…”

“…This typically requires multiple stages of treatment, each addressing one or more individual arteriovenous shunts. A variety of different approaches have been described to treat these dangerous lesions ( 1 , 3 , 4 ).…”

“…Despite significant advances in endovascular embolization technologies and techniques – including the development of advanced microcatheter systems and liquid embolysates such as n-butyl cyanoacrylate (n-BCA) glue (Trufill®, Cerenovus, Irvine, CA, USA) and DMSO based ethylene vinyl alcohol co-polymer (Onyx®, Medtronic, Minneapolis, MN, USA), VOGMs remain very technically challenging lesions ( 1 , 3 – 8 ). While this is due to number of factors, the most significant is the difficulty associated with successful on-target deposition of embolic material at the fistulous site in the setting of the very high-flow arteriovenous shunt.…”

“…Both liquid embolysates and detachable coils have poor stability within the high-flow fistulous target. The high-velocity, turbulent flow created by these arteriovenous shunts markedly increases the difficulty of successful, on-target embolization as the torrential background flow washes embolic agent into the venous outflow tract of the fistula, resulting in venous escape of embolic agent into intracranial venous outlets and pulmonary arteries, with potentially catastrophic consequences ( 1 , 3 – 8 ).…”

“…To increase the safety and efficacy of endovascular treatment for VOGM, several adjunctive flow-control techniques have been developed to counter the challenges created by the extreme high background flow environment ( 1 , 3 ). All these techniques aim to increase the accuracy of embolization and mitigate venous escape.…”