Vein of Galen Malformations in Children Homepage

Authors

Zsombor Gal, BA

Section Editors

Edward Smith, MD
Gianpiero Tamburrini, MD
Alfred Pokmeng See, MD

Editor in Chief

Rick Abbott, MD

Introduction

Vein of Galen malformations are rare congenital AVMs that arise from the failed regression of an embryonic vein. A VOGM is characterized by an AV fistula with multiple feeding arteries and an abnormal venous drainage pattern. High flow through the AV fistula may have severe consequences, including high-output heart failure.

Although these lesions are often detected prenatally, VOGMs may be discovered after the neonatal period. VOGMs usually require both medical and surgical treatment: medical management of its complications and endovascular embolization, the gold standard of definitive treatment. Intervention strategies may be complex and must be informed by the age of presentation, severity of signs and symptoms, and potential need for adjunct therapies.

Key Points

• An error of cerebrovascular development: The pathogenesis of VOGM is poorly understood; these lesions are believed to represent a persistent medial prosencephalic vein, which normally regresses during embryonic development.
• Rare but serious: On a population level, VOGM is rare, but these lesions can lead to severe complications that necessitate treatment. These complications include high-output heart failure, delayed development, venous hypertension, hydrocephalus, and seizures. VOGM presenting in early life are almost always fatal without definitive intervention.
• Variable presentation: Patients with VOGM may present in the neonatal period, during infancy, as an older child, or even as an adult. Symptoms can vary based on the age of presentation and the magnitude of flow across the shunt.
• Evolving treatment strategies: VOGM was once treated with open microsurgery, which had a very high rate of morbidity and mortality. With the development of advanced neurointerventional techniques, endovascular embolization has become the gold standard of treatment and is associated with relatively good clinical and neurodevelopmental outcomes. Moreover, both radiosurgery and fetal embolization are being explored as alternative treatment options.
• Difficult treatment decisions: Decisions regarding the timing and nature of treatment can be challenging, and one must consider the age of presentation, severity of complications, and patient or family preferences.