Vein of Galen Malformation

2019 ◽  
pp. 157-164
Author(s):  
Lissa C. Baird

Vein of Galen malformations consist of multiple arteriovenous fistulas shunting arterial blood into the persistent embryonic prosencephalic vein of Markowski. The mural type are characterized by fewer fistulas and commonly present with progressive hydrocephalus. The choroidal type malformations have more complex and numerous fistulas, have higher flow, and commonly present with severe high-output cardiac failure. Transfemoral transarterial embolization at the fistula sites of the malformation for complete obliteration of arteriovenous shunting is the optimal intervention. An interdisciplinary approach is critical from the time of diagnosis through the post-treatment follow-up period. Sequelae of cardiac failure and end-organ damage may be minimized with early endovascular intervention when indicated and complete embolization when feasible.

2012 ◽  
Vol 32 (5) ◽  
pp. E4 ◽  
Author(s):  
Ross C. Puffer ◽  
David J. Daniels ◽  
David F. Kallmes ◽  
Harry J. Cloft ◽  
Giuseppe Lanzino

Object The authors conducted a study to review their experience with tentorial dural arteriovenous fistulas (DAVFs) treated with transarterial endovascular embolization in which Onyx was used. Methods The authors reviewed prospectively collected data in 9 patients with tentorial DAVFs treated with Onyx embolization between 2008 and 2011. Information reviewed included clinical presentation, angiographic features, treatment, and clinical and radiologically documented outcome. Clinical follow-up was available in every patient. Radiological follow-up studies were available in 8 of 9 patients (mean follow-up 4.6 months). Results Six of 9 patients had complete angiographic obliteration (in 5 this was confirmed by a follow-up angiogram obtained 3–6 months later), and 2 patients had near-complete obliteration (faint filling of the venous drainage in the late venous phase). One patient had partial obliteration and required surgical disconnection. In all patients with complete obliteration, transarterial embolization was performed through the posterior branch of the middle meningeal artery. There were no procedural complications, and no morbidity or mortality resulted from Onyx embolization. Conclusions Transarterial Onyx embolization is a valid, effective, and safe alternative to surgical disconnection in many patients with tentorial DAVFs. The presence of an adequate posterior branch of the middle meningeal artery is critical to achieve a microcatheter position distal enough to increase the likelihood of complete obliteration.


2010 ◽  
Vol 113 (Special_Supplement) ◽  
pp. 21-27 ◽  
Author(s):  
Hyun Ho Jung ◽  
Jong Hee Chang ◽  
Kum Whang ◽  
Jin Soo Pyen ◽  
Jin Woo Chang ◽  
...  

Object The purpose of this study was to assess the efficacy of Gamma Knife surgery (GKS) for treating cavernous sinus dural arteriovenous fistulas (CSDAVFs). Methods Of the 4123 GKSs performed between May 1992 and March 2009, 890 procedures were undertaken to treat vascular lesions. In 24 cases, the vascular lesion that was treated was a dural arteriovenous fistula, and in 6 of these cases, the lesion involved the cavernous sinus. One of these 6 cases was lost to follow-up, leaving the other 5 cases (4 women and 1 man) to comprise the subjects of this study. All 5 patients had more than 1 ocular symptom, such as ptosis, chemosis, proptosis, and extraocular movement palsy. In all patients, CSDAVF was confirmed by conventional angiography. Three patients were treated by GKS alone and 2 patients were treated by GKS combined with transarterial embolization. The median follow-up period after GKS in these 5 cases was 30 months (range 9–59 months). Results All patients experienced clinical improvement, and their improvement in ocular symptoms was noticed at a mean of 17.6 weeks after GKS (range 4–24 weeks). Two patients received embolization prior to GKS but did not display improvement in ocular symptoms. An average of 20 weeks (range 12–24 weeks) was needed for complete improvement in clinical symptoms. There were no treatment-related complications during the follow-up period. Conclusions Gamma Knife surgery should be considered as a primary, combined, or additional treatment option for CSDAVF in selected cases, such as when the lesion is a low-flow shunt without cortical venous drainage. For those selected cases, GKS alone may suffice as the primary treatment method when combined with close monitoring of ocular symptoms and intraocular pressure.


Neurosurgery ◽  
1988 ◽  
Vol 22 (5) ◽  
pp. 908-910 ◽  
Author(s):  
Jane Matjasko ◽  
Walker Robinson ◽  
Daniel Eudaily

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.


2014 ◽  
Vol 20 (6) ◽  
pp. 781-790 ◽  
Author(s):  
Srinivasan Paramasivam ◽  
Yasunari Niimi ◽  
Dan Meila ◽  
Alejandro Berenstein

Dural arteriovenous fistulas (DAVF) associated with our series of patients with vein of Galen malformations (VOGM) are analyzed and discussed. We retrospectively analyzed 87 consecutive cases of VOGM treated between May 2002 and December 2011 and identified 26 patients with DAVF. We gathered information from the clinical case records, angiographic images, MRI on presentation and during follow-up. The findings were analyzed to aid discussion. Among 87 patients treated by multi-stage endovascular embolization, age range from newborn to 19 years, 26 (30%) had DAVF. In seven patients (8%), DAVF were found on initial angiogram and were all into the VOGM. Nineteen (21%) DAVF found on follow-up angiograms were all into the VOGM and distant locations. Sprouting and non-sprouting angiogenesis resulted in the formation of a network of vessels around partially thrombosed VOGM, recruiting blood from the surrounding dura mater resulting in a secondary network on the dura mater supplied by the blood vessels of dura mater in the region or from its natural collaterals. Embolization targeting DAVFs was done in 13 (52%) with complete cure in eight (32%) and recurrence in five (20%). Among 12 non-embolized patients (48%), eight (32%) had spontaneous regression with continued treatment of VOGM. In others, the DAVF either remained stable or progressed. DAVF associated with VOGM represent the dural response to angiogenic stimuli. They are observed to regress spontaneously or mature while continuing to treat the primary feeders of VOGM. It is important to include the external carotid system during angiograms. Persistent DAVF with residual VOGM that do not have access though the pial vessels are used as a conduit to treat the dural shunt and to achieve obliteration of residual VOGM at later stages of treatment.


2001 ◽  
Vol 94 (6) ◽  
pp. 886-891 ◽  
Author(s):  
Jonathan A. Friedman ◽  
Bruce E. Pollock ◽  
Douglas A. Nichols ◽  
Deborah A. Gorman ◽  
Robert L. Foote ◽  
...  

Object. Most dural arteriovenous fistulas (DAVFs) of the transverse and sigmoid sinuses do not have angiographically demonstrated features associated with intracranial hemorrhage and, therefore, may be treated nonsurgically. The authors report their experience using a staged combination of radiosurgery and transarterial embolization for treating DAVFs involving the transverse and sigmoid sinuses. Methods. Between 1991 and 1998, 25 patients with DAVFs of the transverse and/or sigmoid sinuses were treated using stereotactic radiosurgery; 22 of these patients also underwent transarterial embolization. Two patients were lost to follow-up review. Clinical data, angiographic findings, and follow-up records for the remaining 23 patients were collected prospectively. The mean duration of clinical follow up after radiosurgery was 50 months (range 20–99 months). The 18 women and five men included in this series had a mean age of 57 years (range 33–79 years). Twenty-two (96%) of 23 patients presented with pulsatile tinnitus as the primary symptom; two patients had experienced an earlier intracerebral hemorrhage (ICH). Cognard classifications of the DAVFs included the following: I in 12 patients (52%), IIa in seven patients (30%), and III in four patients (17%). After treatment, symptoms resolved (20 patients) or improved significantly (two patients) in 96% of patients. One patient was clinically unchanged. No patient sustained an ICH or irradiation-related complication during the follow-up period. Seventeen patients underwent follow-up angiographic studies at a mean of 21 months after radiosurgery (range 11–38 months). Total or near-total obliteration (> 90%) was seen in 11 patients (65%), and more than a 50% reduction in six patients (35%). Two patients experienced recurrent tinnitus and underwent repeated radiosurgery and embolization at 21 and 38 months, respectively, after the first procedure. Conclusions. A staged combination of radiosurgery and transarterial embolization provides excellent symptom relief and a good angiographically verified cure rate for patients harboring low-risk DAVFs of the transverse and sigmoid sinuses. This combined approach is a safe and effective treatment strategy for patients without angiographically determined risk factors for hemorrhage and for elderly patients with significant comorbidities.


2008 ◽  
Vol 109 (6) ◽  
pp. 1083-1090 ◽  
Author(s):  
Xianli Lv ◽  
Chuhan Jiang ◽  
Youxiang Li ◽  
Zhongxue Wu

Object Use of the Onyx liquid embolic system has become an option for treating dural arteriovenous fistulas (DAVFs) because of its advantageous nonadhesive and cohesive properties. However, the complication rates associated with the use of this system have not been reported. The authors present their initial experience of the risks related to transarterial embolization using this system. Methods Between February 2005 and February 2007, 31 patients with DAVFs were treated at Beijing Tiantan Hospital. Transarterial embolization using Onyx-18 was performed as a preoperative adjunct or as definitive therapy. The demographic characteristics, angiographic features, clinical presentation, treatment, and outcome of the patients were reviewed. Clinical follow-up status was supplemented by telephone interviews to determine Glasgow Outcome Scale scores. Results In 19 patients (61.3%) there was complete angiographic evidence of elimination of the shunts and resolution of the symptoms. The remaining 12 patients were treated successfully but did not attain complete embolization and had residual shunting. Adverse events occurred in 5 of 31 patients, with 3 DAVFs located at the tentorium, 1 at the inferior petrosal sinus, and 1 at the cavernous sinus. Complications included trigeminocardiac reflex in 2 patients (6.5%), hemifacial hypesthesia in 3 patients (9.7%), hemifacial palsy in 2 patients (6.5%), jaw pain in 1 patient (3.2%), posterior fossa infarction in 1 patient (3.2%), and microcatheter gluing in 1 patient (3.2%). At the last follow-up examination, all patients had returned to an independent clinical status. Conclusions Although a complete resolution of symptoms can be achieved with transarterial embolization using the Onyx liquid embolic system, the potential for serious complications exists with this procedure, necessitating the participation of a skilled neurointerventionalist.


2013 ◽  
Vol 19 (2) ◽  
pp. 207-216 ◽  
Author(s):  
Won-Sang Cho ◽  
Ki-Jeong Kim ◽  
O-Ki Kwon ◽  
Chi Heon Kim ◽  
Jiha Kim ◽  
...  

Object Spinal vascular diseases, such as spinal dural arteriovenous fistulas (DAVFs), perimedullary arteriovenous fistulas (AVFs), and spinal arteriovenous malformations (AVMs), are very rare. The authors analyzed the features and treatment outcomes of these conditions. Methods Data from 64 patients were retrospectively reviewed. There were 33 spinal DAVFs (1 patient had 2 lesions), 20 perimedullary AVFs, and 12 spinal AVMs. Clinical features, radiological findings, treatment results, and clinical outcomes were evaluated according to the diseases, subtypes, and treatment modalities. The median duration of follow-up was 20, 42, and 56 months for spinal DAVFs, perimedullary AVFs, and spinal AVMs, respectively. Results Spinal DAVFs showed faster progression of symptoms (median 5, 12, and 36 months for spinal DAVFs, perimedullary AVFs, and spinal AVMs, respectively) and worse neurological status at diagnosis (poor neurological status in 56%, 65%, and 33%, respectively). On MRI, signal voids were demonstrated in all except 1 spinal DAVF. At the last follow-up, 94% of spinal DAVFs, 68% of perimedullary AVFs, and 50% of spinal AVMs were completely obliterated. Favorable clinical outcomes were achieved in 91%, 95%, and 58%, respectively. In detail, the majority (78%) of spinal DAVFs were embolized, resulting in complete obliteration in 92% and favorable clinical outcomes in 92%. Most Type IVa and IVb perimedullary AVFs were surgically treated (71% and 88%), with complete obliterations of 86% and 71%, and favorable clinical outcomes in 100% and 86%, respectively. All Type IVc lesions were embolized with a low cure rate of 40%; however, clinical outcomes were satisfactory. Spinal AVMs were generally embolized (67%), and only glomus-type lesions attained a satisfactory cure rate (80%) and clinical outcome (100%). Conclusions Embolization produced satisfactory outcomes in spinal DAVFs and glomus-type spinal AVMs. Surgery is advantageous in Type IVa and IVb perimedullary AVFs. Palliative embolization can be effective in Type IVc perimedullary AVFs and juvenile spinal AVMs.


2019 ◽  
Vol 26 (2) ◽  
pp. 170-177
Author(s):  
Keisuke Yoshida ◽  
Shinsuke Sato ◽  
Tatsuya Inoue ◽  
Bikei Ryu ◽  
Shogo Shima ◽  
...  

Arteriovenous fistulas at the craniocervical junction are rare vascular malformations with frequent hemorrhagic presentations, which may have a concurrent pial feeder aneurysm. A 65-year-old man presented with subarachnoid hemorrhage and angiography showed an epidural arteriovenous fistula at the C-2 level with an anterior spinal feeder aneurysm without perimedullary venous drainage. Transarterial coil embolization of the ruptured aneurysm and partial Onyx embolization of the shunt led to thrombosis of the aneurysm. However, three years later angiography showed an increased shunt flow and recurrence of the aneurysm. Transvenous embolization of the shunt using coils and Onyx yielded complete obliteration of the shunt, thus leading to occlusion of the aneurysm. This case demonstrates that partial transarterial embolization of arteriovenous fistula leaves a risk of rebleeding, whereas complete obliteration of the shunt with a transvenous approach can lead to disappearance of the flow-related aneurysm without embolization of the aneurysm itself.


Neurosurgery ◽  
2011 ◽  
Vol 68 (2) ◽  
pp. E571-E574 ◽  
Author(s):  
Steven De Vleeschouwer ◽  
Charles-Albert Smets ◽  
Guido Wilms

Abstract BACKGROUND AND IMPORTANCE: Galenic dural arteriovenous fistulas (DAVFs) are a subtype of the rare falcotentorial DAVFs with a high risk of hemorrhage and an aggressive clinical course. Microsurgical treatment is often necessary because endovascular obliteration will rarely completely obliterate the DAVF. CLINICAL PRESENTATION: We present a unique case of a complex, ruptured galenic DAVF in which the key point of the fistula was formed by a large venous aneurysm of the vein of Galen. A session of embolization of the falcotentorial feeding vessels followed by additional surgical transsection of the remaining tentorial arterial feeders failed to exclude the galenic DAVF. Direct clipping of the venous aneurysm through a unilateral occipital craniotomy for a posterior interhemispheric transtentorial approach resulted in an ongoing radiological complete obliteration in this patient, who made an uneventful complete recovery after 2 subarachnoid hemorrhages, which he suffered before this definitive treatment. CONCLUSION: Direct interruption of the fistula key point by clipping of the venous aneurysm, rather than interruption of the feeding vessels, was mandatory for complete exclusion of this complex galenic DAVF.


2000 ◽  
Vol 93 (6) ◽  
pp. 1058-1061 ◽  
Author(s):  
Jonathan A. Friedman ◽  
Bruce E. Pollock ◽  
Douglas A. Nichols

✓ This 61-year-old man with a right-sided tentorial dural arteriovenous fistula (DAVF) was initially treated with staged stereotactic radiosurgery and transarterial embolization. Results of follow-up cerebral angiography performed 4 years later demonstrated complete obliteration of the dAVF and development of a previously undetected cerebellar arteriovenous malformation (AVM). The newly diagnosed AVM was treated with repeated stereotactic radiosurgery. This represents the first reported case of the development of a cerebral AVM documented in an adult by serial angiography.


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