Delayed and incomplete treatment may result in dural fistula development in children with Vein of Galen malformation

The association of dural arteriovenous fistulas (DAVF) in children with Vein of Galen malformation (VGM) has recently been reported for the first time. In a larger series of cases treated with transarterial NBCA embolization, 30% had DAVF. We wanted to analyze the development of DAVF in our cohort of children with VGM and to evaluate whether their occurrence depends on different treatment timing and embolic materials. We analyzed 43 VGM cases treated with a combined transarterial and transvenous approach between 2003 and 2016. In our early series until 2011, we used coils solely in 21 children. Since 2012, 22 children were treated with the combination of coils and Onyx. In the early series treated with coils solely, no case presented initially with or developed DAVF over time on follow-up angiograms. In our recent series we found four cases (9%) with DAVF. In two patients (5%), DAVF were found on the initial angiogram. Both patients presented at our department at age >2 years and were not treated elsewhere before. One patient (2%) presented at our department with too proximal occlusion of arterial feeders performed at another institution before. Only one patient (2%) developed DAVF in our department after the transarterial use of Onyx. Interestingly, this child did not develop DAVF as long as we used coils solely and his DAVF was localized exactly where an Onyx cast was identified. In conclusion, delayed and incomplete treatment may have a considerable impact on the occurrence of DAVF in VGM.

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Dural and cavernous arteriovenous fistulas

The Neurosurgeon's Handbook ◽

10.1093/med/9780198570677.003.0488 ◽

2010 ◽

pp. 608-614

Author(s):

George Samandouras

Keyword(s):

Vein Of Galen ◽

Vein Of Galen Malformation ◽

Carotid Cavernous Fistula ◽

Cavernous Fistula ◽

Arteriovenous Fistulas

Chapter 9.14 covers dural and cavernous arteriovenous fistulas, including duralarteriovenous fistula (DAVF), carotid cavernous fistula (CCF), and vein of Galen malformation (VOGAM).

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Endovascular Treatment of Cervical Giant Perimedullary Arteriovenous Fistulas

Neurosurgery ◽

10.1227/neu.0b013e31822ec19e ◽

2011 ◽

Vol 70 (1) ◽

pp. 141-149 ◽

Cited By ~ 7

Author(s):

Alfredo Casasco ◽

Leopoldo Guimaraens ◽

Cagin Senturk ◽

Enrico Cotroneo ◽

Renato Gigli ◽

...

Keyword(s):

Endovascular Treatment ◽

Vascular Malformations ◽

Transarterial Embolization ◽

Cervical Region ◽

Motor Deficit ◽

Published Data ◽

Direct Puncture ◽

Arteriovenous Fistulas ◽

Proximal Occlusion ◽

Transvenous Approach

Abstract BACKGROUND Giant perimedullary arteriovenous fistulas (GPMAVFs) located in the cervical region are a rare pathology with distinctive characteristics. OBJECTIVE To evaluate clinical presentation and different endovascular treatment options of cervical GPMAVFs and review previously published data in the literature regarding cervical GPMAVFs. METHODS Six patients with cervical GPMAVFs were found in the spinal vascular malformations database of our group collected between 1990 and 2009. Endovascular techniques and treatment outcomes were evaluated and compared with other published series. RESULTS Clinical presentations were progressive motor deficit (5 patients), hematomyelia (1 patient), meningeal syndrome (1 patient), and respiratory arrest and gait apraxia (1 patient). Three patients were treated by the transarterial approach. One patient was treated by the transvenous approach due to previous embolizations resulting in a proximal occlusion and preventing a safe transarterial approach. A transvenous approach was used in another patient due to complex arterial anatomy. In 1 patient, direct percutaneous puncture of the venous pouch was necessary because of previous proximal occlusion of the arteries. All embolizations resulted in complete occlusions with clinical improvement, and there was no recanalization during a mean follow-up of 21 months. CONCLUSION Transarterial embolization of cervical GPMAVFs is safe and effective when it is done in highly experienced centers. Cervical GPMAVFs that cannot be accessed by the transarterial technique due to their complex angioarchitecture can be treated by transvenous embolization or direct puncture of the venous pouch.

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MR Perfusion Imaging in a Case of a Vein of Galen Malformation with Secondary Capillary Angioectasia

Interventional Neuroradiology ◽

10.1177/159101990300900110 ◽

2003 ◽

Vol 9 (1) ◽

pp. 57-63 ◽

Cited By ~ 1

Author(s):

Y.-C. Weon ◽

D. Ducreux ◽

H. Alvarez ◽

P. Lasjaunias

Keyword(s):

Perfusion Imaging ◽

Vascular Malformations ◽

Venous Hypertension ◽

Cerebral Veins ◽

Vein Of Galen ◽

Mr Perfusion ◽

Vein Of Galen Malformation ◽

Arteriovenous Fistulas ◽

Mr Perfusion Imaging ◽

Conventional Mri

Vein of Galen Aneurysmal Malformations (VAGMs) are uncommon vascular malformations associated with dilatation of the vein of Galen embryonic forerunner with single or multiple direct arteriovenous fistulas within its wall without direct reflux into normal cerebral veins. We describe a patient with a late neurological onset presenting a classic VGAM complicated by secondary thalamic capillary angioectasia imaged with MR perfusion. In our patient, abnormal MR perfusion parameters were not seen on conventional MRI; they probably reflect underlying venous hypertension. They were located in areas involved in motor neurological deficit.

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Transvenous microguidewire looping technique for breach of ipsilateral inferior petrosal sinus occlusions en route to cavernous sinus dural arteriovenous fistulas

Interventional Neuroradiology ◽

10.1177/1591019916653251 ◽

2016 ◽

Vol 22 (5) ◽

pp. 590-595 ◽

Cited By ~ 10

Author(s):

Young Dae Cho ◽

Jong Kook Rhim ◽

Dong Hyun Yoo ◽

Hyun-Seung Kang ◽

Jeong Eun Kim ◽

...

Keyword(s):

Cavernous Sinus ◽

Coil Embolization ◽

Inferior Petrosal Sinus ◽

Arteriovenous Fistulas ◽

Dural Arteriovenous Fistulas ◽

Embolic Materials ◽

Transvenous Approach ◽

Cure Rates ◽

Guiding Catheter

Background and purpose Transarterial access to dural arteriovenous fistulas (dAVFs) has been popularized by device improvements and novel embolic materials. However, this approach is limited in the cavernous sinus (CS) because of related complications and low cure rates. Although a transvenous approach, via ipsilateral inferior petrosal sinus (IPS), may be more suitable for CS-dAVFs, microcatheter delivery is occasionally impeded by ipsilateral IPS occlusion. Described herein is a microguidewire looping method to breach such occlusions, thus enabling access to CS lesions. Methods A microcatheter is initially advanced into the IPS orifice, and a microguidewire is passed into the occluded IPS. Looping is easily achieved through the resistance met. With greater support of the guiding catheter, the microguidewire (still looped) is then advanced into the CS. When nearing the CS, the microcatheter is further reinforced, and it is navigated along the microguidewire into the CS. Results This technique was applied in 10 instances of CS-dAVF with ipsilateral IPS occlusion, enabling ipsilateral access to the CS. In eight cases (80%), microdevice advancement was successful, culminating in effective transvenous coil embolization. Clinical and radiologic outcomes in all patients were excellent, with no delayed post-procedural cranial palsies. Conclusion This microguidewire looping technique enables safe and effective entry into the CS during transvenous coil embolization of CS-dAVFs with ipsilateral IPS occlusion.

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Transvenous Pressure Monitoring Guides Endovascular Treatment of Vein of Galen Malformation: A Technical Note

Pediatric Neurosurgery ◽

10.1159/000516446 ◽

2021 ◽

pp. 1-6

Author(s):

Daniel Chang ◽

Robin Babadjouni ◽

Peyton Nisson ◽

Julie Lynn Chan ◽

Miguel Quintero-Consuegra ◽

...

Keyword(s):

Heart Failure ◽

Endovascular Treatment ◽

Coil Embolization ◽

Clinical Signs ◽

Technical Note ◽

Vein Of Galen ◽

Pressure Monitoring ◽

Flow Reduction ◽

Embolic Materials ◽

Transvenous Approach

Introduction: Vein of Galen malformations (VGMs) are complex congenital arteriovenous malformations that generally require serial endovascular treatment sessions to slowly correct the high-flow fistulous connections that cause increased venous pressures and ultimately lead to the classic presentations of heart failure, hydrocephalus, and intracranial hemorrhages. Despite the advances in endovascular technology and embolic materials, the resolution of embolization is often limited to the subjective view of diminished flow on angiograms. Case Report: An 8-month-old patient with a VGM developed clinical signs of heart failure and growing head circumference with ventriculomegaly. The patient was treated endovascularly with a transvenous approach for coil embolization while undergoing continuous monitoring of the post-malformation venous pressures. The arterial and venous systolic blood pressures (SBP) were collected at serial time points and used to measure estimated 95% confidence interval bounds for arteriovenous SBP gradients and determine when sufficient coil embolization and flow reduction was thought to be achieved. Conclusion: The transvenous pressure monitoring demonstrated progressively increasing pressure gradients between the arterial and venous systems that correlated with the degree of flow reduction on angiographic runs. The patient underwent successful coil embolization of the VGM and had improvement of heart failure and ventricular size in follow-up at 8-month post-op. This provides a novel technique to introduce an objective measurement that can guide the embolization of a VGM.

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A cardiopulmonary bypass strategy to support a patient with vein of Galen malformation

Perfusion ◽

10.1177/02676591211001534 ◽

2021 ◽

pp. 026765912110015

Author(s):

Alex Robertson ◽

Nagarajan Muthialu ◽

Mike Broadhead

Keyword(s):

Cardiopulmonary Bypass ◽

Surgical Repair ◽

Circulatory Arrest ◽

Ductus Arteriosus ◽

Venous Drainage ◽

Deep Hypothermic Circulatory Arrest ◽

Vein Of Galen ◽

Vein Of Galen Malformation ◽

Neurological Sequelae ◽

Ph Stat

We present a dissection of the patent ductus arteriosus and pulmonary artery for surgical repair utilising cardiopulmonary bypass in the setting of vein of Galen malformation. Several strategies were employed to attenuate the cerebral shunt including pH-stat, high cardiac index, restrictive venous drainage, continuous ventilation and deep hypothermic circulatory arrest. The patient recovered from surgery with no apparent neurological sequelae.

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An unusual case of hypoplastic internal jugular vein in a vein of Galen malformation

Journal of Neuroanaesthesiology and Critical Care ◽

10.4103/2348-0548.182334 ◽

2016 ◽

Vol 03 (02) ◽

pp. 149-150

Author(s):

Prabhakar Prakash ◽

Virendra Jain ◽

Kavita Sandhu

Keyword(s):

Internal Jugular Vein ◽

Unusual Case ◽

Jugular Vein ◽

Vein Of Galen ◽

Vein Of Galen Malformation

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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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Pregnancy Causes Diminished Myogenic Tone and Outward Hypotrophic Remodeling of the Cerebral Vein of Galen

Journal of Cerebral Blood Flow & Metabolism ◽

10.1038/jcbfm.2012.199 ◽

2013 ◽

Vol 33 (4) ◽

pp. 542-542 ◽

Cited By ~ 6

Author(s):

Anne-Eva van der Wijk ◽

Malou PH Schreurs ◽

Marilyn J Cipolla

Keyword(s):

Cerebral Arteries ◽

Venous Blood ◽

Blood Stasis ◽

Wall Stress ◽

Myogenic Tone ◽

Cerebral Vein ◽

Cerebral Veins ◽

Vein Of Galen ◽

First Time ◽

Serotonergic Innervation

Pregnancy increases the risk of several complications associated with the cerebral veins, including thrombosis and hemorrhage. In contrast to the cerebral arteries and arterioles, few studies have focused on the effect of pregnancy on the cerebral venous side. Here, we investigated for the first time the effect of pregnancy on the function and structure of the cerebral vein of Galen in rats. Our major finding was that cerebral veins from late-pregnant (LP, n = 11) rats had larger lumen diameters and thinner walls than veins from nonpregnant (NP, n = 13) rats, indicating that pregnancy caused outward hypotrophic remodeling of the vein of Galen. Moreover, veins from NP animals had a small amount of myogenic tone at 10 mm Hg (3.9 ± 1.0%) that was diminished in veins during pregnancy (0.8 ± 0.3%; P < 0.01). However, endothelium-dependent and -independent vasodilation of the veins was unchanged during pregnancy. Using immunohistochemistry, we show that the vein of Galen receives perivascular innervation, and that serotonergic innervation of cerebral veins is significantly higher in veins from LP animals. Outward hypotrophic remodeling and diminished tone of cerebral veins during pregnancy may contribute to the development of venous pathology through elevated wall tension and wall stress, and possibly by promoting venous blood stasis.

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