scholarly journals Vein of Galen malformation

2009 ◽  
Vol 27 (5) ◽  
pp. E8 ◽  
Author(s):  
Stanley Hoang ◽  
Omar Choudhri ◽  
Michael Edwards ◽  
Raphael Guzman

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.

Author(s):  
Elizabeth C. Eastburn ◽  
Mary Landrigan-Ossar

Vein of Galen malformation (VGM) is a high-flow intracranial vascular malformation, presenting in utero or after birth with a range of initial symptoms. The natural history of untreated VGM is grave, with progressive permanent neurological dysfunction, developmental delay, seizures, intracranial hemorrhage, and death. Presentation varies with age, with more severe manifestations resulting in earlier presentation and poorer outcome. The mainstay of treatment is medical stabilization followed by endovascular embolization. The goal is to reduce flow through the malformation such that normal development can proceed without neurological deficit. The past 20 years have seen great advances both in the technical treatment of the vascular lesion and in the overall prognosis of patients treated for this condition. Key to a good outcome is appropriate selection of patients and treatment by physicians familiar with this pathophysiology.


2016 ◽  
Vol 9 (9) ◽  
pp. 880-886
Author(s):  
Dan Meila ◽  
Katharina Melber ◽  
Dominik Grieb ◽  
Collin Jacobs ◽  
Heinrich Lanfermann ◽  
...  

IntroductionVein of Galen malformation (VGM), a high-flow intracranial arteriovenous shunt, is among the most severe neurovascular diseases in childhood. In many cases untreated children die or survive only severely disabled. Endovascular embolization is the preferred treatment.ObjectiveTo develop a simple fistulous-type VGM phantom model for teaching and training of different endovascular treatment methods and to investigate new treatment options and devices.MethodsAn experimental in vitro pulsatile phantom model was developed imitating a high-flow fistulous-type VGM, which is typical, especially in the neonatal phase. Pressure measurements at different arterial sites were performed before and after closure of the VGM. Closure of the VGM was achieved by coiling using a combined microcatheter-based transvenous and transarterial approach called ‘kissing microcatheter technique’.ResultsThe behaviour of the phantom model in vitro under fluoroscopy and under angiographic runs was extremely similar to that in in vivo conditions in children. The results showed that intra-arterial pressures changed and increased statistically significantly at all measurement sites after embolization, as in human arteriovenous malformation. We also demonstrated different and complementary visualizations of hemodynamics and angioarchitecture by antegrade and retrograde microcatheter injections.ConclusionsOur phantom model behaves like a typical fistulous-type VGM and can be used in vitro for teaching and training and for further research. It offers a new and better understanding of hemodynamics and angioarchitecture in the endovascular management of VGM.


2019 ◽  
Vol 09 (02) ◽  
pp. e172-e176 ◽  
Author(s):  
R. E. Giesinger ◽  
Y. N. Elsayed ◽  
M. P. Castaldo ◽  
P. J. McNamara

AbstractVein of Galen malformation results in predictable changes in physiology which exist on a continuum. Severe pulmonary hypertension may present as hypoxemia; however, excessive reduction in pulmonary vascular resistance may precipitate progressive pulmonary overcirculation and impaired systemic blood flow. Right ventricular performance and the patency and direction of the ductus arteriosus may play a crucial role in postductal organ perfusion. Physiological stabilization may be complex and variable over time. The utilization of targeted neonatal echocardiography to guide treatment decisions may improve the ability to provide therapy tailored to the specific disease pathophysiology and monitor serially as conditions change. An enhanced approach to physiological stabilization may reduce the risk of unexpected decompensation and allow for thoughtful, controlled endovascular embolization in appropriate candidates.


2015 ◽  
Vol 15 (2) ◽  
pp. 197-202 ◽  
Author(s):  
Robyn A. Howarth ◽  
Andrew Reisner ◽  
Joshua J. Chern ◽  
Laura L. Hayes ◽  
Thomas G. Burns ◽  
...  

Cognitive regression is a well-described presentation of vein of Galen aneurysmal malformations (VGAMs) in childhood. However, it remains unclear whether successful treatment of the malformation can reverse cognitive regression. Here, the authors present the case of a 5-year-old girl with a VGAM that was treated with staged endovascular embolization procedures. Comprehensive neurocognitive assessments were completed before intervention and approximately 6 years after initial presentation. There were significant age-matched improvements in this child's neurocognitive profile over this period. The authors believe that timely and successful treatment of VGAM in children may not only stabilize the associated cognitive deterioration but, in some cases, may ameliorate these deficits. Details of this case and a discussion of neurocognitive deficits related to VGAM are presented.


2020 ◽  
Vol 56 (2) ◽  
pp. 296-297
Author(s):  
H. Bouchghoul ◽  
C. Adamsbaum ◽  
H. Maurey ◽  
A. Ozanne ◽  
M. V. Senat

2006 ◽  
Vol 26 (5) ◽  
pp. 273-278 ◽  
Author(s):  
F Y Wong ◽  
P J Mitchell ◽  
B M Tress ◽  
P A Dargaville ◽  
P M Loughnan

2010 ◽  
Vol 6 (5) ◽  
pp. 444-451 ◽  
Author(s):  
Dhruv Khullar ◽  
Ahmed M. I. Andeejani ◽  
Ketan R. Bulsara

Object Vein of Galen aneurysmal malformations (VGAMs) continue to account for high morbidity and mortality rates in the pediatric population. Whereas in the past, mortality rates were nearly 100%, recent developments in endovascular embolization and improvements in neonatal care have improved prognoses. It is now possible that some patients can achieve normal neurological development following embolization of the VGAM. Access to the lesion can be gained via transarterial or transvenous routes. In this paper the authors review the pathophysiological characteristics of VGAM and discuss the evolution of treatment options. Methods A PubMed literature search was performed for vein of Galen malformation treatment options, beginning in the 1970s. A total of 22 papers were reviewed in full, and outcome data for 615 patients from 1983 to 2010 were compiled. Articles were reviewed if they focused primarily on the treatment of VGAM and reported outcomes for at least 5 treated patients. Results Of the 265 patient outcomes reported between 1983 and 2000, 200 received endovascular therapy. Of these patients 72% had a favorable outcome, and a 15% mortality rate was found. Microsurgery was found to have an 84.6% mortality rate. Furthermore, 76.7% of untreated patients died. More recently, endovascular embolization has become the mainstay of VGAM treatment. Of the 350 patients assessed between 2001 and 2010, 337 were treated endovascularly, mostly via the transarterial approach. Of these patients, 84.3% were found to have good or fair outcomes, and a 15.7% mortality rate was found. Neonates had the worst clinical outcomes following endovascular treatment, with a 35.6% mortality rate, whereas infants and children had significantly better outcomes, with mortality rates of 6.5% and 3.2%, respectively. Conclusions Endovascular embolization has considerably improved outcomes in patients with VGAM. In the past, the prognosis for patients with VGAM was dismal, and successful procedures were considered to be those that partially or completely obliterated the lesion, but did not necessarily improve the patient's symptoms. More recently, with the continued development and improvement of endovascular techniques, many patients are found to be neurologically normal on follow-up, and mortality rates have dropped substantially when compared with microsurgical treatment.


2018 ◽  
Vol 21 (4) ◽  
pp. 380-383 ◽  
Author(s):  
Nam K. Yoon ◽  
Jonathan P. Scoville ◽  
Philipp Taussky

Vein of Galen malformations are congenital high-flow vascular malformations that often present with heart failure, hydrocephalus, developmental delay, and intracranial hemorrhage. Because open surgical treatment is associated with high morbidity and mortality, endovascular embolization is increasingly becoming the preferred method of intervention. However, embolization of these lesions can be difficult because of their high-flow nature. The use of adenosine-induced cardiac standstill for treatment of vein of Galen malformations has not been previously described in neonates. The authors describe 3 treatments in 2 patients that demonstrate that the use of adenosine is well tolerated and allows safe transarterial embolization of high-flow vein of Galen malformations in the pediatric population.


2021 ◽  
pp. 159101992199336
Author(s):  
Timothy G White ◽  
Amir R Dehdashti ◽  
Henry H Woo

Background Even in the modern endovascular era, the treatment of Vein of Galen Malformations (VOGM) is extremely challenging. While their natural history is very poor, endovascular embolization has emerged as the standard of care. These lesions often require multiple treatment sessions to decrease shunting, with each treatment including multiple pedicles. Here we present the first reported use of the Scepter Mini (Microvention, Aliso Viejo, CA) in the treatment of vein of Galen malformations. Clinical presentation A 7 month old female presented with an enlarging VOGM that was initially identified on prenatal ultrasound. Given the enlarging size of the lesion and failure to meet developmental milestones, the patient underwent planned endovascular embolization of the VOGM. The novel Scepter Mini balloon catheter was used for treatment of this lesion affording easy access to the target pedicle and immediate flow arrest which allowed for immediate cure of the lesion. Conclusion The novel Scepter Mini Balloon (Microvention, Aliso Viejo, CA) afforded excellent distal access with subsequent immediate flow arrest therefore facilitating endovascular cure. Initially, a staged approach was favored for the treatment of the lesion, but the flow arrest achieved by the Scepter mini facilitated immediate occlusion from a single pedicle.


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