Vein of Galen Malformation

Stanley Hoang, B.S.; Omar Choudhri, M.D.; Michael Edwards, M.D.; Raphael Guzman, M.D.

Neurosurg Focus. 2009;27(5):E8

Abstract and Introduction

Abstract

A vein of Galen malformation is a rare intracranial vascular lesion affecting the pediatric population. Its poor prognosis has been significantly improved with the development of endovascular embolization. In this paper the authors review the developmental mechanisms, clinical pathophysiology, and the available data on the management and outcome of the disease.

Introduction

AVGM consists of multiple arteriovenous shunts draining into a dilated median prosencephalic vein of Markowski.^[1,33,46] This persistent embryonic vein is normally absent in the adult. Although a rare intracranial vascular anomaly, VGM has been estimated to represent 30% of pediatric vascular malformations.^[32]

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Abstract and Introduction
Review of VGMs
Conclusions
References

Table 1. Summary of the Bicêtre neonatal evaluation score*
Score†	Function
Score†	Cardiac	Cerebral	Respiratory	Hepatic	Renal
5	normal	Normal	normal
4	overload & no med Tx	subclinical; isolated EEG abnormalities	tachypnea; finishes bottle
3	failure; stable w/med Tx	nonconvulsive, intermittent neurological signs	tachypnea; does not finish bottle	no hepatomegaly; normal hepatic function	normal
2	failure; not stable w/med Tx	isolated convulsion	assisted ventilation; normal saturation FiO₂ < 25%	hepatomegaly; normal hepatic function	transient anuria
1	ventilation needed	seizures	assisted ventilation; normal saturation FiO₂ > 25%	moderate or transient hepatic insufficiency	unstable diuresis w/Tx
0	resistant to med Tx	permanent neurological signs	assisted ventilation; desaturation	abnormal coagulation, elevated enzyme levels	anuria

* Table adapted from Lasjaunias et al., 2006. Abbreviations: EEG = electroencephalogram; FiO₂ = fractional inspired oxygen; med = medical.
† Maximal score is 21: 5 (cardiac) + 5 (cerebral) + 5 (respiratory) + 3 (hepatic) + 3 (renal).

Table 2. Studies on clinical outcomes following embolization for treatment of VGM
Authors & Year	Outcome
Mickle & Quisling, 1986	authors used transtorcular embolization of the VGM using Gianturco coils in 3 pts; satisfactory outcome in 2 pts
Friedman et al., 1993	improved embolization techniques decreased the mortality rate from 50% (w/22 neonates) in 1991 to 0% (11 neonates) in 1993
Fullerton et al., 2003	embolization in 27 pts resulted in 61% having no or mild developmental delay & a 15% mortality rate during hospitalization
Gupta et al., 2006	embolization in 13 pts w/VGM & 2 pts w/VGAD resulted in complete obliteration in 66%, & a mortality rate of 20% (3 of 15)
Lasjaunias et al., 2006	endovascular embolization in 233 pts resulted in angiographically confirmed 90–100% occlusion in 55% of pts; mortality rate 10.6%; 74% of survivors neurologically normal on follow-up

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Vein of Galen Malformation

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Vein of Galen Malformation

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