Stenting of a Venous Stenosis in Vein of Galen Aneurysmal Malformation

The vein of Galen aneurysmal malformation (VGAM) is a high flow arteriovenous shunt at the choroidal level. In the neonatal period, it typically presents with cardiac failure. Venous stenoses, occlusions and anomalies are often present. In the absence of adequate venous outflow pathways, severe, irreversible cerebral parenchymal damage may occur due to intracranial venous hypertension, altered hydrodynamics and ischaemia. We present a case of deployment of a stent across a focal superior jugular bulb stenosis in an effort to avert this outcome.

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An intrathoracic arteriovenous malformation discovered as an extremely uncommon reason of neonatal congestive cardiac failure

Cardiology in the Young ◽

10.1017/s1047951109991375 ◽

2009 ◽

Vol 19 (5) ◽

pp. 530-533 ◽

Cited By ~ 2

Author(s):

Sigrun R. Hofmann ◽

Matthias Weise ◽

Katharina I. Nitzsche

Keyword(s):

Arteriovenous Malformation ◽

Cardiac Failure ◽

Arteriovenous Malformations ◽

Neonatal Period ◽

High Flow ◽

Congestive Cardiac Failure ◽

Vein Of Galen ◽

Prenatal Detection ◽

First Case

AbstractCongenital arteriovenous malformations are rare causes of congestive cardiac failure in neonates. The most common sites are in the head and liver, but other sites include the thorax, the abdomen and the limbs. The onset of failure is usually not in the immediate neonatal period, but later on in life, albeit that lesions such as the arteriovenous malformation of the vein of Galen, and other arteriovenous malformations in different locations which produce high flow can present early. We describe here the first case, to the best of our knowledge, of prenatal detection of an intrathoracic arteriovenous malformation producing neonatal cardiac failure, which was successfully treated by surgery postnatally.

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Management of Jugular Bulb Stenosis in Pediatric Vein of Galen Malformation: A Novel Management Paradigm

Pediatric Neurosurgery ◽

10.1159/000517653 ◽

2021 ◽

Vol 56 (6) ◽

pp. 584-590

Author(s):

Gaurav Gupta ◽

Michael S. Rallo ◽

David Y. Goldrich ◽

Vinayak Narayan ◽

Neil Majmundar ◽

...

Keyword(s):

Jugular Foramen ◽

Jugular Bulb ◽

Venous Hypertension ◽

Cerebral Injury ◽

Vein Of Galen ◽

Microsurgical Decompression ◽

Venous Stenosis ◽

Initial Reduction ◽

Treatment Paradigm ◽

Angioplasty And Stenting

Introduction: Pediatric vein of Galen malformations (VOGMs) are fistulous intracranial malformations arising congenitally within the choroidal fissure that can present with an array of neurological and cardiac sequelae. Associated venous stenosis may result in intracranial venous hypertension and ischemia leading to severe, irreversible cerebral injury. Management of neonatal VOGMs typically involves staged embolization and angioplasty/stenting for relief of venous stenosis. Rarely, jugular foraminal narrowing has been identified as causing jugular bulb stenosis. Case Presentation: We present the case of a 22-month-old female diagnosed with VOGM prenatally who displayed persistent intracranial venous hypertension despite multiple neuroembolization procedures during the neonatal period. Following initial reduction in arteriovenous shunting, she once again developed venous hypertension secondary to jugular bulb stenosis for which angioplasty was attempted. Failure of angioplasty to relieve the venous hypertension prompted skull base imaging, which revealed jugular foraminal ossification and stenosis. Microsurgical jugular foraminotomy followed by balloon angioplasty and stenting significantly reduced jugular pressure gradients. Restenosis requiring re-stenting developed postoperatively at 9 months, but the patient has remained stable with significant improvement in cortical venous congestion. Discussion/Conclusion: This case demonstrates the efficacy of microsurgical decompression of the jugular foramen and endovascular angioplasty/stenting as a novel treatment paradigm for the management of intracranial venous hypertension in the setting of VOGM.

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The Vein Of Galen Aneurysmal Malformation Associated With Sinus Venosus Defect And Anomalous Pulmonary Veins

Non Invasive Vascular Investigation ◽

10.24966/nivi-7400/100026 ◽

2020 ◽

Vol 5 (2) ◽

pp. 1-4

Author(s):

Merjema Karavdic ◽

Keyword(s):

Neonatal Period ◽

Pulmonary Veins ◽

Arteriovenous Shunt ◽

Sinus Venosus ◽

Vein Of Galen ◽

Cardiac Defect ◽

Congenital Cardiac Defect ◽

Sinus Venosus Defect

he Vein of Galen Aneurysmal Malformation (VGAM) is a rare cerebral arteriovenous shunt, which may be associated with a congenital cardiac defect. Embolisation of the VGAM is safer in infancy, even it may be undertaken in the neonatal period if necessary.

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Regression of cerebral calcifications after endovascular treatment in a case of vein of Galen arteriovenous malformation

Journal of Neurosurgery Pediatrics ◽

10.3171/2009.2.peds08419 ◽

2009 ◽

Vol 4 (1) ◽

pp. 17-20 ◽

Cited By ~ 2

Author(s):

Anshuman Bansal ◽

Philippe Gailloud ◽

Lori Jordan ◽

Diego San Millán Ruíz

Keyword(s):

Arteriovenous Malformation ◽

Endovascular Treatment ◽

Venous Hypertension ◽

Arteriovenous Shunt ◽

Vein Of Galen ◽

Cerebral Calcifications

The authors present the case of an infant harboring a vein of Galen arteriovenous malformation with conspicuous cerebral calcifications that progressively regressed after staged endovascular obliteration of the lesion. The role of venous hypertension and hydrocephalus secondary to the arteriovenous shunt are discussed to explain the formation and regression of the cerebral calcifications.

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Republished: Retreatment of a choroidal vein of Galen malformation with embolization 42 years after open surgical treatment in the neonatal period

Journal of NeuroInterventional Surgery ◽

10.1136/neurintsurg-2016-012709.rep ◽

2016 ◽

Vol 9 (5) ◽

pp. e19-e19

Author(s):

Lucas Elijovich ◽

Asim F Choudhri ◽

Adam S Arthur ◽

Paul Klimo ◽

Frederick A Boop ◽

...

Keyword(s):

Surgical Treatment ◽

Cerebral Artery ◽

Intraventricular Hemorrhage ◽

Open Surgery ◽

Anterior Cerebral Artery ◽

Neonatal Period ◽

Venous Hypertension ◽

Vein Of Galen ◽

Vein Of Galen Malformation ◽

Open Surgical Treatment

In 1976 an infant boy initially presented with a choroidal vein of Galen malformation (cVOGM). Craniotomy and clipping of the anterior cerebral artery and posterior choroidal arteries was performed, which was reported in the literature 40 years ago. The patient remained asymptomatic until age 42 when he re-presented with an isolated intraventricular hemorrhage. Angiography demonstrated cVOGM with venous sinus occlusive disease leading to venous hypertension and subsequent intraventricular hemorrhage. The angiogram also demonstrated a ‘pseudo-nidus’ composed of multiple arterial-to-arterial anastomoses that had developed as a result of the original surgical treatment. We embolized a portion of the lesion to reduce the venous hypertension. The patient has been asymptomatic for more than 2 years. To our knowledge, this is the first reported case of a symptomatic neonatal VOGM treated with open surgery that required embolization as an adult for a delayed hemorrhagic presentation.

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The Impact of Angiographic Geometry on the Clinical Presentation of True Vein of Galen Aneurysmal Malformations

Pediatric Neurosurgery ◽

10.1159/000512213 ◽

2020 ◽

Vol 55 (6) ◽

pp. 344-350

Author(s):

Tamer Hassan ◽

Ahmed Sultan ◽

Tamer Ibrahim ◽

Waleed F. El-Saadany

Keyword(s):

Heart Failure ◽

Clinical Presentation ◽

Transarterial Embolization ◽

High Flow ◽

Vein Of Galen ◽

Venous Outflow ◽

Significant Stenosis ◽

3 Dimensional ◽

Ct Angiogram ◽

The Impact

Background Purpose: Vein of Galen aneurysmal malformations (VGAM) may carry life-threatening and catastrophic sequelae in children. Their clinical presentations are differently variable between cardiac and cerebral syndromes. There may be a possible relationship between the Galenic angioarchitecture and the clinical presentation. We aimed to study the effect of the venous outflow impairment on the incidence of high-flow heart failure and the incidence of hydrocephalus. Materials and Methods: The angiographies of 21 patients with true VGAM have been studied. MR angiography, 3-dimensional CT angiogram (3D CTA), and conventional digital subtraction angiography were performed for all patients with true VGAM. Transarterial embolization was done in 1 or more sessions for all cases. Results: Among the 21 cases, 14 cases were mural type, 5 cases were choroidal type, and 2 cases were mixed. Hydrocephalus was observed more in infants (92.3%), mural types (92.9%), giant and supergiant (87.5%) aneurysms, and in cases with persistent limbic circle (90.91%). All cases of hydrocephalus were associated with significant stenosis (>70%) of the Falcine sinus draining system (100%). Conclusion: Significant stenosis (>70%) of the draining sinus is a significant factor for VGAM aneurysmal enlargement and occurrence of hydrocephalus. Probably, venous outflow impairment decreases the incidence of high-flow heart failure and increases the incidence of hydrocephalus.

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Surgical removal of a foreshortened right innominate vein Wallstent causing venous outflow obstruction

Vascular ◽

10.1177/17085381211068996 ◽

2022 ◽

pp. 170853812110689

Author(s):

Kristin Schafer ◽

Eric Goldschmidt ◽

Andrew Seiwert

Keyword(s):

Upper Extremity ◽

Venous Hypertension ◽

Complete Relief ◽

Surgical Removal ◽

Central Venous ◽

Outflow Obstruction ◽

Venous Outflow ◽

Central Venous Stenosis ◽

Venous Stenosis ◽

Venous Outflow Obstruction

Objectives: Stenting of central venous stenosis to preserve upper extremity hemodialysis access is well-described, though upper extremity complications secondary to these stents are less frequently discussed. Methods: We present the case of a 43-year-old male with a right brachiocephalic fistula who developed symptoms of venous hypertension following placement of a Wallstent for central venous stenosis. Workup demonstrated venous outflow obstruction secondary to stent foreshortening into the right subclavian vein. Results: The Wallstent was removed in a piecemeal fashion using an open surgical technique and a HeRO graft was placed for dedicated fistula outflow with complete relief of the patient’s symptoms. Conclusion: In situations where a stent has migrated and endovascular removal is not possible, individual Wallstent fibers can be removed through a limited venotomy.

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Quantified Partial Embolisation in a Vein of Galen Malformation with Congestive Cardiac Failure

Interventional Neuroradiology ◽

10.1177/159101999600200208 ◽

1996 ◽

Vol 2 (2) ◽

pp. 149-154 ◽

Cited By ~ 1

Author(s):

M. Moersdorf ◽

P. Lasjaunias

Keyword(s):

Cardiac Failure ◽

Guide Wire ◽

Paediatric Population ◽

High Flow ◽

Congestive Cardiac Failure ◽

Vein Of Galen ◽

Vein Of Galen Malformation ◽

Diastolic Velocity ◽

Morphological Criteria ◽

Feeding Vessels

The aim of the study was to quantify embolisation in a vein of Galen malformation by intraluminal measurements of flow velocities in the feeding vessels and to test a new 0.014-inch Doppler guide wire in the paediatric population. The examination was performed in a seven month old boy who presented with a vein of Galen malformation combined with congestive cardiac failure from birth. The measurements showed a decrease of the systolic and diastolic velocities and an increase in the effective downstream vascular resistance after closure of the high flow fistula. The decrease of the diastolic velocities after embolisation was more prominent because of the reduction of the sump effect responsible for the high diastolic velocity before embolisation. The reduction of about 40% of the vein of Galen malformation estimated with morphological criteria was associated with a decrease of the diastolic velocity in the basilar artery of about 50%. The clinical improvement led to the discontinuance of most of the cardiac treatment over a few days. These measurements seem to offer the possibility to assess the necessary reduction of the shunt to relieve CCF in a vein of Galen malformation.

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Intracranial Venous Hypertension and Venous Sinus Stenting in the Modern Management of Idiopathic Intracranial Hypertension

Life ◽

10.3390/life11060508 ◽

2021 ◽

Vol 11 (6) ◽

pp. 508

Author(s):

Robert K. Townsend ◽

Kyle M. Fargen

Keyword(s):

Intracranial Hypertension ◽

Young Women ◽

Idiopathic Intracranial Hypertension ◽

Venous Hypertension ◽

Venous Sinus ◽

Venous Outflow ◽

The Status ◽

Insight Into ◽

Symptom Recurrence

Idiopathic intracranial hypertension (IIH) is a debilitating condition that has traditionally been difficult to treat. In recent years, there has been increasing focus on the role of intracranial venous hypertension in the pathophysiology of IIH. Based on increased understanding of this pathophysiology, venous sinus stenting (VSS) has emerged as a safe and reliable treatment for a certain population of patients with IIH. Stratifying patients with IIH based on the status of their venous outflow can provide insight into which patients may enjoy reduction in their symptoms after VSS and provides information regarding why some patients may have symptom recurrence. The traditional view of IIH as a disease due to obesity in young women has been cast into doubt as the understanding of the role of intracranial venous hypertension has improved.

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Successful Surgical and Anesthetic Management of Vein of Galen Aneurysm in a Neonate in Congestive Heart Failure

Neurosurgery ◽

10.1227/00006123-198805000-00018 ◽

1988 ◽

Vol 22 (5) ◽

pp. 908-910 ◽

Cited By ~ 5

Author(s):

Jane Matjasko ◽

Walker Robinson ◽

Daniel Eudaily

Keyword(s):

Heart Failure ◽

Congestive Heart Failure ◽

Cardiac Failure ◽

Growth And Development ◽

Anesthetic Management ◽

Vascular Supply ◽

Vein Of Galen ◽

Vein Of Galen Malformation ◽

Vein Of Galen Aneurysm

Abstract A 12-day-old infant in intractable cardiac failure due to a vein of Galen malformation was treated successfully with serial ligation of the majority of the vessels feeding the malformation. Despite some residual vascular supply to the malformation, the congestive heart failure has disappeared and growth and development have been normal over a 3-year follow-up period.

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