Vein of Galen malformation in a neonate: A case report and review of endovascular management

Abstract

Vein of Galen malformation (VOGM) is a rare congenital vascular malformation caused by the maldevelopment of its embryonic precursor, the median prosencephalic vein of Markowski. VOGM results in neonatal morbidity and mortality, and premature delivery does not improve the outcome. We report a term female neonate in whom a vein of Galen malformation was diagnosed prenatally at 37 wk of gestation during a growth ultrasound and confirmed by fetal magnetic resonance imaging. Signs of cardiac decompensation were evident in the fetus. Multiple interventional radiology embolizations of the feeding vessels were performed successfully on days 7, 10, 12, 14 and 19. A review of the literature on the endovascular management of neonates with these malformations is presented herein.

“Melting brain” as complication of a vein of galen aneurysmal malformation diagnosed by fetal MRI

“…If left untreated a VGAM or VGAD has an almost 100% morbidity and mortality. The first attempts of treating these malformations were recorded in an infant with intracranial hypertension in the early 20 th century [1,[2][3][4][5][6][7][8]. It can be diagnosed as early as week 6 to 11 of gestation, but is usually not detected prior to the 24 th week of gestation [9].…”

“…This chronic venous congestion may result in a progressive destruction of the hemispheric white matter and overlying cortex known as the "melting brain syndrome". Furthermore the increased venous pressure impairs the resorption of cerebrospinal fluid at the Pachionic granulations with subsequent hydrocephalus which may be aggrevated by direct compression and obstruction of the Sylvian aqueduct by the dilated vein of Galen [3,6]. Furthermore systemic complications of the AV-shunting include congestive heart failure, pleural and pericardial effusions, ascites as well as generalized subcutaneous edema (fetal hydrops).…”

A review of extraaxial developmental venous anomalies of the brain involving dural venous flow or sinuses: persistent embryonic sinuses, sinus pericranii, venous varices or aneurysmal malformations, and enlarged emissary veins

“…Emissary veins can provide conduits for infection in the intracranial sinuses and brain parenchyma. 13,16,53,63 Enlarged emissary veins have been involved as sources of intraoperative air emboli 42 and have also been described as forming part of an AVF 21 and providing vascular connection to the diploic venous system in the formation of SP. 47 Along with the developmental, pathophysiological, and neurosurgical implications of emissary veins, a detailed understanding of emissary veins is necessary in the context of venous flow patterns and even inheritance patterns.…”

“…The vein of Galen is a venous structure within the quadrigeminal cistern formed by the confluence of the internal cerebral veins and the basal veins of Rosenthal. A vein of Galen malformation (VOGM) is a rare vascular anomaly caused by abnormal development of the median prosencephalic vein of Markowski, 52,53 which is a precursor of the vein of Galen, and VOGMs are thought to develop by the 11th week of gestation. 53 Raybaud et al have made significant contributions to the embryological understanding of the vein of Galen.…”

The Prognosis for Fetuses with Vein of Galen Malformation and Symptoms of Heart Failure

“…He also changed the treatment technique by using arterial rather than venous access to endovascular therapy of VGM 5 . We can find more and more cases of fetuses with VGM and prenatal and postnatal signs of circulatory failure, witch (who) had good treatment effect in PubMed 7,8 .…”