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Vein of Galen malformations (VOGMs), also known as Vein of Galen Aneurysmal Malformations (VGAMs), are rare and complex cerebrovascular anomalies that pose significant diagnostic and therapeutic challenges. These malformations result from abnormal arteriovenous shunts during embryonic development, leading to a range of severe clinical manifestations, including high-output cardiac failure and hydrocephalus. Advances in prenatal imaging, particularly fetal MRI, have improved early detection, allowing for timely intervention. Endovascular techniques, especially transarterial embolization, have become the primary treatment modality, often preferred over surgical approaches due to their effectiveness and lower risk. However, challenges remain, particularly in managing these malformations in neonates and infants, where the risk of complications is high. Gamma Knife radiosurgery offers a non-invasive alternative for select cases, though its effects are gradual and may carry delayed risks. Despite advancements, the management of VOGMs continues to require a multidisciplinary approach, with ongoing research focused on improving outcomes through a better understanding of the genetic and molecular underpinnings of the disease. Future directions include the integration of genetic studies into clinical practice and the refinement of treatment strategies to optimize outcomes for this complex condition., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

The vein of Galen arteriovenous malformation is a rare abnormality of the fetal cerebral vascular system. It is caused by the persistence of a cerebral arteriovenous fistula of the median prosencephalic vein. Due to the high mortality rate of the surgical option in its treatment, the approach with embolization and radiosurgery combinations continues to be popular. We present a 3-year-old child patient who was accidentally detected by vein of Galen malformations during a screening for headache complaints with known heart disease. In the treatment of this patient, we performed radiosurgery therapy alone, rather than a combination. Our case is the third case of a and the youngest example child who was treated with only radiosurgery without embolization. Gamma knife therapy, which is a more convenient and effective treatment plan, is a method that can be safely used instead of open surgery with a high mortality rate., (Copyright © 2024 Copyright: © 2024 Neurology India, Neurological Society of India.)

Significant efforts have been made over the last few decades to improve the diagnosis and management of patients with vein of Galen malformations (VOGMs). The mainstays of treatment remain focused on primary endovascular management by staged transarterial embolizations with adjunctive use of transvenous embolization, medical therapy, and neurosurgical intervention for symptom control in select patients. Innovation in endovascular technology and techniques as well as promising new genomic research elucidating potential therapeutic targets hold significant promise for the future of VOGM treatment., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Vein of Galen malformation (VOGM) is a rare, life-threatening vascular malformation in neonates and is treated with embolization. However, even at the most experienced centers, patients face high mortality and morbidity. In utero treatment options have been limited by lack of animal models or simulations., Objective: To create a novel ultrasound phantom simulator for a preclinical feasibility study of in utero fetal intervention for VOGM., Methods: Novel phantoms were designed and built in two configurations of spherical and windsock shape from cryogel material to mimic the salient vasculature of the fetal VOGM, based on real-patient fetal MR imaging dimensions. Critical anatomy was realistically mimicked within this model and transtorcular ultrasound-guided coil deployment was simulated. Each phantom model was assessed before and after treatment to evaluate coil mass deposition within the target., Results: The two phantoms underwent pretreatment T2-weighted MR imaging assessment, ultrasound-guided embolization, post-treatment MR and fluoroscopic imaging, and visual inspection of the sliced phantoms for target embolization verification. Postoperative MR scans confirmed realistic compact deposition of the coil masses within the central cavity. Phantom embolization results were submitted as part of the institutional review board and US Food and Drug Administration investigative device exemption approval for a first-in-humans clinical trial of fetal intervention for VOGM., Conclusions: A phantom simulator for fetal intervention of VOGM produces lifelike results during trial interventions, removing obstacles to feasibility and safety evaluations, typically precluded by non-availability of appropriate animal models. The study provides a proof of concept for potentially wider applications of medical simulation to enable novel procedural advancements in neurointerventions., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction: Pediatric cerebrovascular lesions are very rare and include aneurysms, arteriovenous malformations (AVM), and vein of Galen malformations (VOGM)., Objective: To describe and disseminate a validated, reproducible set of 3D models for optimization of neurosurgical training with respect to pediatric cerebrovascular diseases METHODS: All pediatric cerebrovascular lesions treated at our institution with adequate imaging studies during the study period 2015-2020 were reviewed by the study team. Three major diagnostic groups were identified: aneurysm, AVM, and VOGM. For each group, a case deemed highly illustrative of the core diagnostic and therapeutic principles was selected by the lead and senior investigators for printing (CSG/JM). Files for model reproduction and free distribution were prepared for inclusion as Supplemental Materials., Results: Representative cases included a 7-month-old female with a giant left MCA aneurysm; a 3-day-old male with a large, complex, high-flow, choroidal-type VOGM, supplied from bilateral thalamic, choroidal, and pericallosal perforators, with drainage into a large prosencephalic vein; and a 7-year-old male with a left frontal AVM with one feeding arterial vessel from the anterior cerebral artery and one single draining vein into the superior sagittal sinus CONCLUSION: Pediatric cerebrovascular lesions are representative of rare but important neurosurgical diseases that require creative approaches for training optimization. As these lesions are quite rare, 3D-printed models and open source educational materials may provide a meaningful avenue for impactful clinical teaching with respect to a wide swath of uncommon or unusual neurosurgical diseases., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: Vein of Galen malformation (VOGM) is an exceptionally uncommon form of congenital intracranial vascular malformations. It is highly unusual for this lesion to spontaneously thrombose. The clinical presentation of a patient may range from being asymptomatic to critically ill. The underlying pathophysiological mechanisms that cause spontaneous thrombosis are still poorly understood., Methods and Results: The literature on spontaneous thrombosis of VOGM was systematically reviewed, analyzed, and summarized with a focus on its pathophysiology, types, clinical presentations, diagnosis, management, and outcomes. It was also illustrated with a case presentation. The case presents an unusual presentation and location of a VOGM in a 2-year-old boy who was successfully treated with surgical resection., Conclusions: A handful of cases of thrombosed VOGM have been reported worldwide where surgery was used to treat the condition. Low-flow fistulas of the mural type are prone to spontaneous thrombosis, have delayed clinical presentations, and are typically diagnosed in young children. Among the many possible manifestations, hydrocephalus is by far the most common. In the absence of blood flow, MRI is the diagnostic test of choice. Depending on the patient's symptoms, surgery to either remove the aneurysm or divert the cerebrospinal fluid usually results in a good prognosis., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: Vein of Galen aneurysmal malformations (VGAMs) can, through multiple mechanisms, complicate with hydrocephalus (HCP). It is generally agreed that management strategies in this scenario should focus on endovascular embolizations. Treatment options for non-responders, however, have been only scarcely reported upon., Case Presentation: We present a nine-month-old boy with a mural type VGAM complicated by HCP. Despite endovascular occlusion of the sole feeder, the child exhibited hydrocephalus progression prompting an Endoscopic Third Ventriculostomy (ETV). This procedure restored a cerebrospinal fluid (CSF) circulation otherwise impaired by aqueduct obstruction. Later, a new feeder arose and a second embolization was ultimately needed in order to achieve VGAM regression. Throughout four years of follow up, the child attained all developmental marks., Discussion/conclusion: VGAMs are prone to hydrocephalus development as there is both an underlying venous congestion and a mechanical, obstructive component. Although there is a rationale for addressing both components, the underlying AV shunts and subsequent venous pressure elevations usually determine failure of traditional CSF shunting strategies. It is therefore challenging to manage HCP in patients who failed to improve following endovascular embolizations. For such cases, ETV stands as an elegant minimal invasive alternative with potential to provide a more physiologic drainage route and thus better allow for neurological development., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

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.

Objective: Vascular lesions of the pineal region comprise aneurysms of the pineal region, arteriovenous malformations, cavernous malformations, and vein of Galen malformations. In the present report, we have offered an extensive review of each vascular pineal region lesion., Methods: We performed an extensive literature review, focusing on the current therapeutic options available for the different vascular lesions of the pineal region., Results: Vascular lesions of the pineal region are rare. Microneurosurgery remains a valid treatment of cavernomas, arteriovenous malformations, and aneurysms. Endovascular treatments seem to be the first option for the vein of Galen malformations, followed by microneurosurgery. Radiosurgery seems beneficial for small-size arteriovenous malformations. Complex and large vascular lesions will require a combination of multiple treatments., Conclusions: Vascular lesions of the pineal region are complex, uncommon diseases. Thus, definitive therapeutic modalities for these lesions require further research., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: Vein of Galen malformations (VOGM) comprise nearly a third of pediatric cerebrovascular anomalies, with potentially devastating neurological and systemic complications. Advances in endovascular therapies have dramatically improved outcomes compared to historical surgical treatments, and neurosurgeons are an essential component of the multidisciplinary critical care team., Objective: To retrospectively review pediatric patients with VOGM treated at Texas Children's Hospital (TCH), a quaternary referral center, over 15 yr, and present lessons learned in treating children with modern endovascular techniques., Methods: Charts from TCH were retrospectively reviewed for the past 15 yr. Patients with diagnosis including "Vein of Galen," "Vein of Galen malformation," "Vein of Galen aneurysmal malformation," or any abbreviations (ie, VOG, VOGM, VOGAM) were reviewed. Presentation, imaging, treatment specifics, and clinical outcomes were reported., Results: There were 18 patients with VOGM managed at TCH from 2002 to 2018 with a total of 29 embolizations. Seventeen were performed with a single embolisate (NBCA or Onyx), and 12 with a combination. A dual lumen balloon catheter was used as an adjunct in 3 embolizations. Complications occurred in 5 embolizations (24%), including hemorrhage, embolisate migration, and femoral vessel occlusion. Surviving patients were followed for a mean of 38 mo, with 12 having normal or near-normal neurological development., Conclusion: VOGM can present with a myriad of neurological and systemic symptoms, potentially in extremis. Neurosurgical involvement in these cases is critical, as urgent treatment can be lifesaving. Patients may require multiple treatment sessions using a variety of endovascular tools and techniques., (Copyright © 2019 by the Congress of Neurological Surgeons.)

We report the case of a 10-month-old boy with an enlarged head circumference and severe motor developmental delay. MRI showed a vein of Galen malformation (VGAM) with a heavily dilated median prosencephalic vein. Digital subtraction angiography confirmed a mural type VGAM with three feeding arteries arising from the posterior cerebral arteries. Due to the short length of the feeding arteries and the high flow, occlusion of the feeding vessels with detachable coils was not possible because of repeated coil dislocation into the dilated vein. Embolization of the three feeding vessels was then performed with a Woven EndoBridge single layer device (WEB SL17). In two arteries complete occlusion was accomplished with the WEB alone and in one artery additional deployment of two coils was necessary. Follow-up imaging at day 1 after treatment as well as 3 and 9 months after embolization showed persistent occlusion., Competing Interests: Competing interests: CJM received educational grants from Microvention and Stryker. AB is proctor for Sequent Medical, Microvention, Stryker and Medtronic and received lecture royalties from Penumbra. All other authors have no competing interests regarding the article., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Competing Interests: Competing interests: None declared.

Objective: Describe the course and outcomes in a UK national cohort of neonates with vein of Galen malformation identified before 28 days of life., Methods: Neonates with angiographically confirmed vein of Galen malformation presenting to 1 of 2 UK treatment centers (2006-2016) were included; those surviving were invited to participate in neurocognitive assessment. Results in each domain were dichotomized into "good" and "poor" categories. Cross-sectional and angiographic brain imaging studies were systematically interrogated. Logistic regression was used to explore potential outcome predictors., Results: Of 85 children with neonatal vein of Galen malformation, 51 had survived. Thirty-four participated in neurocognitive assessment. Outcomes were approximately evenly split between "good" and "poor" categories across all domains, namely, neurological status, general cognition, neuromotor skills, adaptive behavior, and emotional and behavioral development. Important predictors of poor cognitive outcome were initial Bicêtre score ≤ 12 and presence of brain injury, specifically white matter injury, on initial imaging; in multivariate analysis, only Bicêtre score ≤ 12 remained significant., Interpretation: Despite modern supportive and endovascular treatment, more than one-third of unselected newborns with vein of Galen malformation did not survive. Outcome was good in around half of survivors. The importance of white matter injury suggests that abnormalities of venous as well as arterial circulation are important in the pathophysiology of brain injury. Ann Neurol 2018;84:547-555., (© 2018 The Authors. Annals of Neurology published by Wiley Periodicals, Inc. on behalf of American Neurological Association.)

Advances in endovascular embolization have improved morbidity and mortality among patients with vein of Galen malformations (VoGMs). The patient presented at 3 months of age with increased head circumference and a bruit over his anterior fontanelle. Diagnostic cerebral angiography confirmed the presence of a large mural-type VoGM. The decision was made to undergo a staged arterial embolization at 4 years of age after developing worsening right-sided hemiparesis. An attempt was made to occlude the posterior choroidal feeding vessel with a large 25 mm × 50 cm coil, followed by a 6 mm × 20 cm coil; however, the high flow of the lesion displaced both coils into the wall of the aneurysmal venous sac. Interval magnetic resonance imaging and angiography revealed partial occlusion of the VoGM at 7 months and complete thrombosis at 24 months post procedure, precluding a need for additional coiling. This case illustrates that a minimal change in intramural flow dynamics of VoGMs could lead to progressive thrombosis., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Aim: To describe presentation, clinical course, and outcome in postneonatal presentations of vein of Galen malformation (VGM)., Method: Children older than 28 days presenting with VGM (from 2006-2016) were included. Notes/scans were reviewed. Outcome was dichotomized into 'good' or 'poor' using the Recovery and Recurrence Questionnaire. Logistic regression was performed to explore relationships between clinico-radiological features and outcome., Results: Thirty-one children (18 males, 13 females) were included, presenting at a median age of 9.6 months (range 1.2mo-11y 7mo), most commonly with macrocrania (n=24) and prominent facial veins (n=9). Seven had evidence of cardiac failure. VGM morphology was choroidal in 19. Hydrocephalus (n=24) and loss of white matter volume (n=15) were the most common imaging abnormalities. Twenty-nine patients underwent glue embolization (median two per child). Angiographic shunt closure was achieved in 21 out of 28 survivors. Three children died of intracranial haemorrhage (1y, 6y, and 30d after embolization). Ten patients underwent neurosurgical procedures; to treat haemorrhage in four, and hydrocephalus in the rest. Outcome was categorized as good in 20 out of 28 survivors, but this was not predictable on the basis of the variables listed above., Interpretation: Postneonatally presenting VGM has distinctive clinico-radiological features, attributable to venous hypertension. Endovascular treatment is associated with good outcomes, but more specific prognostic prediction was not possible within this cohort., What This Paper Adds: Clinical and radiological features in older children with vein of Galen malformation relate to venous hypertension. Outcome is good in most cases with endovascular therapy. Mortality is low but is related to intracranial haemorrhage., (© 2018 Mac Keith Press.)

Vein of Galen (VG) aneurysmal malformations (VGAMs) are complex vascular lesions. Their etiopathogenesis is extensively debated and remains poorly understood. Strictly speaking, true VGAMs are Galenic pial arteriovenous fistulas. They are believed to arise in utero and are contended to drain either into the true VG or the median prosencephalic vein of Markowski. Several classification systems have been proposed and are widely used. With the advent of endovascular therapy, precise understanding of the angioarchitecture is critical for management and therapeutic decision making. We review clinical presentation and diagnostic imaging findings, discussing angioarchitectural properties as they relate to treatment planning. Clin. Anat. 31:259-268, 2018. © 2017 Wiley Periodicals, Inc., (© 2017 Wiley Periodicals, Inc.)

Introduction: Vein 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., Objective: To 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., Methods: An 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'., Results: The 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., Conclusions: Our 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., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

This case report describes the use of the Micro Vascular Plug (MVP) system in a mural-type vein of Galen aneurysmal malformation, resulting in successful occlusion of the cerebrovascular fistula. This result was confirmed with immediate cessation in blood flow and 6-month follow-up angiography. The unique characteristics of the MVP provide potential advantages over current embolic techniques for occlusion of high-flow, fistulous, intracranial vessels such as those seen in the vein of Galen aneurysmal malformation, and may represent a valuable addition to the currently available options.

Background: Vein of Galen aneurysmal malformations (VGAMs) are congenital disorders that may require emergency treatment and some may cause sudden death before medical attention is provided. Some patients also have a spontaneous thrombosis., Objective: To understand the natural progression of VGAMs through a systematic literature review., Methods: We examined PubMed to identify studies published between July 1973 and March 2015. We determined the proportion of patients with VGAM who died before receiving medical attention, who received emergency treatment, or had a spontaneous thrombosis. We pooled estimates of proportions with corresponding 95% CIs calculated using the raw (ie, untransformed) proportions., Results: The 31 studies obtained described the outcome of 754 patients with VGAM. The probability of sudden death risk was 4% (95% CI 1% to 7%; I 2 =51.6%); of an emergency operation was 46% (95% CI 34% to 58%; I 2 =93.2%); and of spontaneous thromboses 1% (95% CI 0% to 2%; I 2 =0%) of cases. Differences between age and clinical outcomes of patients with spontaneous thromboses were significant according to the rank test (Mann-Whitney U test, Z=-2.398, p=0.016), both having a linear correlation (χ 2 test, p=0.022)., Conclusions: Over time, the rate of preoperative sudden death in patients with VGAM gradually declined and the rate of emergency operations gradually increased. The outcome of patients with early spontaneous thromboses was good. Our study provides a definitive description of the natural progression of VGAMs and the need for urgent medical attention., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

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., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

Background and Purpose: Vein of Galen aneurysmal malformation (VGAM) is a rare developmental intracranial vascular malformation. We analyzed the clinical presentations, imaging findings, angioarchitecture, management options, and outcome in a demographically heterogeneous set of VGAM patients., Methods: We retrospectively analyzed cases of VGAM from our departmental archive collected between 1988 and January 2015. Demographic, clinical, therapeutic, and follow-up details were obtained for each patient from the available records., Results: We identified 36 patients with VGAM including 6 neonates, 18 infants, 7 children aged 2-10 years, and 5 adults. Macrocrania was the commonest presenting feature. Type of fistulae was mural in 14 and choroidal in 18 patients while 4 had a thrombosed sac at presentation. In 3 cases the dilated venous sac had connection with the deep venous system. Bilateral jugular atresia and stenosis were seen in 9 and 6 patients, respectively. Giant venous sac (>4 cm) was significantly correlated with mural type (p=0.0001). Dural arterial recruitment was seen in 4 patients including 3 adults. Among the 23 patients treated by endovascular means, 14 had a good outcome, 5 had a poor outcome, and 4 died. A significant correlation was noted between jugular atresia and poor outcome (p=0.003)., Conclusions: We encountered a wide range of demographic, clinical, and angiographic features in VGAM. Mural type malformations were associated with giant venous sacs. Good outcome after embolization was seen in selected neonates and in most of the infants, children, and adults. Jugular atresia was significantly associated with poor outcome., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

A 5-month-old infant was to be treated with elective transarterial embolization for a vein of Galen aneurysmal malformation (VGAM). A team of endovascular surgical neuroradiologists, pediatric interventional radiologists, and pediatric cardiologists attempted conventional femoral arterial access, which was unsuccessful given the small caliber of the femoral arteries and superimposed severe vasospasm. Thereafter, eventual arterial access was achieved by navigating from the venous to the arterial system across the patent foramen ovale following a right femoral venous access. Embolization was then successfully performed. At a later date, the child underwent successful transvenous balloon-assisted embolization and eventual arterial embolization with cure of the VGAM.

Background: Vein of Galen aneurysmal malformation (VGAM) is a rare pediatric vascular malformation of the brain. Genetic backgrounds are not well elucidated. We report on a monozygotic twin with VGAM and his endovascular treatment, and the genetic analyses of the twins and their parents., Case Description: In a monochorionic, diamniotic pregnancy of a 28-year-old healthy woman, monozygotic twins were born by emergency caesarian section because of fetal distress of the smaller twin at 25 weeks' and 4 days' gestation. Although a postnatal cranial ultrasound failed to detect VGAM in the smaller twin, mild heart failure persisted. A brain magnetic resonance (MR) examination of this twin on day 82 revealed choroidal VGAM. The twin was treated successfully by two sessions of embolization at 6 and 8 months of age. An MR examination at 1 year showed minimal residual arteriovenous shunts. He developed normally similar to the normal co-twin, with a follow-up period of 1 year and 6 months. As for the affected twin, no germline mutation or copy number variations were identified in ENG, ALK1, SMAD4, BMPR2, PTEN, RASA1, KRIT1, Marcavernin, or PDCD10 through whole-exome sequencing (WES)., Conclusion: We have reported a rare combination of a monozygotic twin and VGAM and the successful endovascular treatment. Phenotypic discordance in monozygotic twins established early in embryogenesis could be attributable to environmental or epigenetic factors., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Up to now, only little is known about hydrocephalus (HC) in vein of Galen malformation (VGM). We want to present the different etiologies and our long-term experience (1992-2015) in the management of HC., Methods: Out of 44 treated children with VGM, we retrospectively reviewed all cases with HC. We analyzed the etiologies, our treatment results and complications., Results: Twenty-one children (48 %) presented either with HC or developed it over time. In 21 % of those cases, high venous pressure was presumably the sole cause. Until 2009, seven of them received ventriculoperitoneal (VP) shunting; six of those resulted in severe postoperative complications. The remaining children have been treated successfully by endovascular embolization. Five out of the 44 children (11 %) developed HC after intraventricular hemorrhage. In four cases, those children were treated with positive results by using transient external ventricular drainages. In one case a VP shunt with highest valve pressure was inserted. Another four children (9 %) presented with aqueductal stenosis-related HC caused by either dilated venous outflow or space-occupying coil masses after embolization. The latter case was successfully treated by ventriculocisternostomy, whereas endovascular treatment decreased the venous outflow in size and thus resolved the HC in the other cases. In the remaining cases (7 %), atrophy due to melting brain syndrome led to HC ex vacuo., Conclusions: HC in VGM is a common phenomenon with several etiologies requiring different treatments. In most cases, embolization of the VGM as sole treatment is completely sufficient in order to decrease high venous pressure. However, certain other causes of HC should be treated in an interdisciplinary setting by specialized neurosurgeons.

Purpose: We present a technical development of the endovascular treatment technique for vein of Galen aneurysmal malformation, using Onyx™ (ethylene vinyl alcohol copolymer) delivered under flow control through double-lumen balloon microcatheters., Materials and Methods: An 11-month-old patient that initially presented with increasing head circumference was diagnosed with mural type vein of Galen aneurysmal malformation. Complete cure was obtained in a single endovascular treatment session. Onyx was delivered simultaneously through two double-lumen balloon microcatheters (Scepter™, Microvention, Terumo Group). In both arterial feeders, balloon inflation occluded the high-flow arterial-venous shunting and thus facilitated the controlled propagation of the embolic product in the afferent artery and the shunt point while preventing distal migration into the venous system., Results: Two years post-procedure, MR imaging showed persistent occlusion of arterial-venous shunts with complete regression of the venous dilatation. On clinical examination, the patient had no neurological deficits, and no cognitive impairment was detected at neuropsychological testing., Conclusion: The use of double-lumen balloon microcatheters for flow control during delivery of Onyx represents a viable alternative for the endovascular treatment of vein of Galen aneurysmal malformations.

Vein of Galen aneurysmal malformations (VGAMs) are uncommon congenital malformations arising from fistulous communication with the median vein of the prosencephalon, a primitive precursor of midline cerebral venous structures. Angiographic embolization is the primary modality for treatment given historically poor microsurgical outcomes. Only a few reports of treatment by Gamma Knife radiosurgery (GKRS) exist in the literature, and the results are variable. The authors present 2 cases of VGAM in which GKRS provided definitive treatment with good outcome: one case involving antenatal presentation of a high-output, mural-type VGAM with complex clinical course refractory to multiple embolic procedures, and the other a choroidal-type VGAM presenting with hemorrhage in an adult and without a feasible embolic approach. With discussion of these cases and review of the literature, the authors advocate inclusion of GKRS as a therapeutic option for treatment of these complex lesions.

A 22-year-old man presented with intractable progressive headaches over 2 months. Noncontrast head CT and contrast-enhanced MRI scan revealed a hyperdense pineal-based mass (figure, A and B). Arteriogram was normal (figure, C and D).

We report a case of vein of Galen aneurysmal malformation (VGAM) with a newly developed dural arteriovenous fistula (AVF) subsequent to successful embolization. A male neonate diagnosed as VGAM with prenatal ultrasonography and MRI presented severe cardiac and respiratory failure soon after birth. Five sessions of transarterial embolization using NBCA were performed during the first 6 months of his life. The shunt flow was effectively reduced and heart failure was resolved after the treatment. Follow-up angiography performed 2.5 years after the last embolization revealed complete obliteration of VGAM and newly developed small dural AVF on the wall of the thrombosed falcorial sinus. We believe that the dural AVF in this case was caused by local venous hypertension or induction of angiogenic factor during the thrombosing process of VGAM.

Unlabelled: We performed retrospective and prospective analysis of surgical treatment of arteriovenous malformations of vein of Galen. Since 1987 till 2009 90 patients were operated in Burdenko Neurosurgical Institute. Age varied between 1 month and 38 years, 69 patients were children below 10. The paper describes main techniques for early diagnosis of the disease, its manifestation depending on age and type of malformation, treatment modalities, early postoperative and follow-up results. Endovascular treatment was applied in 75 (83.3%) patients. Complications with persistent neurological deficit after endovascular occlusion were observed in 10 (16%) patients, 2 patients died shortly after surgery., Conclusion: endovascular surgery is the treatment of choice in arteriovenous malformations of vein of Galen; optimal age for endovascular procedure is 4-5 months; observation in asymptomatic course is ineffective.

A prenatally diagnosed patient was referred to our hospital at 35 weeks of gestation. Antenatal ultrasonography demonstrated cardiomegaly and aneurysm of the vein of Galen. A 3,290 g male baby was delivered by elective cesarean section at 37 weeks of gestation. Physical examination was remarkable for tachypnea, hyperdynamic precordium with a continuous murmur, cranial bruit and bounding carotid pulses. Magnetic resonance angiography confirmed vein of Galen aneurysmal malformation and demonstrated the vessels feeding the aneurysm. Postnatal management included aggressive medical treatment of cardiac failure and transarterial embolization of the vessels feeding the aneurysm at 3 days of age. Posthemorrhagic hydrocephalus developed after embolization, and a ventriculo-peritoneal shunt was placed at 29 days of age. Presently, the patient is 4 years of age and has no neurological abnormality at follow-up. Vein of Galen aneurysmal malformations can cause severe morbidity and mortality in neonates. However, careful obstetric follow-up and early postnatal endovascular treatment of these malformations may lead to a favorable outcome., (Copyright © 2011. Published by Elsevier B.V.)

Object: The treatment of vein of Galen aneurysmal malformation (VGAM) is among the most challenging of all the neurovascular disorders., Methods: Between 1984 and 2005, 26 consecutive patients with VGAMs were treated by the authors' group, and their data were prospectively collected; 12 patients presented with congestive heart failure (CHF) and 10 had hydrocephalus. Five patients did not undergo endovascular treatment because of minimal symptoms or severe comorbidities. Twelve patients underwent embolization for refractory CHF requiring ventilation: 7 of these 12 patients had the procedure in the neonatal stage (5 survived), and 5 were treated in infancy or childhood following successful aggressive medical treatment (4 survived). The other 9 patients underwent endovascular surgery to treat a progressively enlarging head circumference; there was imaging evidence of ventricular enlargement and/or signs of developmental delay (6 underwent surgery in infancy and 3 in childhood; 1 patient died)., Results: The survival rate in this series was 76.9% (20 of 26). Fourteen (66.7%) of 21 patients who underwent endovascular treatment had no developmental delay. An analysis of various factors demonstrated that comorbidities and embolization effect (procedure success and long-term effect) were prognostic factors of survival. In addition, the patient's age at embolization was significantly higher in those with developmental delay than in those without (559.86 ± 535.43 days vs 94.83 ± 95.77 days, respectively; p = 0.028)., Conclusions: The authors conclude that neonatal VGAMs can be treated successfully with a strategic approach integrating antenatal diagnosis, endovascular surgery, treatment at intensive care facilities, and the cooperative efforts of different specialties. In the authors' experience, patients in whom VGAM was diagnosed and managed in infancy or childhood had more than 90% long-term survival.

Introduction: Vein of Galen arteriovenous malformations (VGAM) are rare intracranial vascular lesions mostly involving young children. Endovascular therapy is the current standard of care. Albeit interventional techniques have greatly reduced the once dismal vital and functional prognoses previously associated with these lesions, the treatment of VGAMs remains a complex therapeutic challenge., Discussions: This article reviews the available endovascular options for VGAM therapy, emphasizing three points that we have identified as critical in our practice for the establishment of a treatment strategy: (1) the importance of the deep cerebral venous anatomy, in particular the existence of normal drainage through the Galenic system in spite of the VGAM; (2) the concept of treatment staging, for arterial as well as for venous interventions; and (3) the definition of a therapeutic goal that can be attained at a reasonable cost in terms of complication risks and functional outcome.

Purpose: This study aims to analyze the clinical and radiological findings, timing and type of treatment, and outcome in children under 1 year of age that presented with neurosurgical vascular malformations., Methods: A retrospective review of 23 children under 1 year of age with neurosurgical vascular malformations was performed at a single institution between 1999 and 2009., Results: The lesions found in this age group were: 10 vein of Galen aneurysmal malformations (VGAMs), 5 arteriovenous malformations (AVM), 2 pial arteriovenous fistulas (AVF; 1 in the brain and 1 in the spinal cord), 2 cavernous malformations, 2 dural sinus malformations (DSMs) in the posterior fossa with negative angiography which after surgery turned out to be embryonal malformations of dural sinuses, 1 sacular aneurysm, and 1 dural arteriovenous fistula (DAVF) that drained toward the vein of Galen. Of the 10 patients with VGAM, 8 presented choroidal type and 2 mural type. Two patients with choroidal VGAM were born in dramatically severe clinical condition; therefore, we decided to withhold aggressive treatment, and they died within 48 h after birth. The other eight patients with choroidal VGAM received endovascular treatment between 1 and 3 staged embolizations. In four of them, total occlusion was achieved and subtotal and partial in the others. One patient had complications and evolved with severe developmental delay. Another patient with partial occlusion died, and another patient with previous developmental delay stopped treatment because of parental decision making. Five patients evolved with normal development and one with mild delay. Only one patient required shunt. Hydrocephalus was solved after endovascular treatment in the rest of the patients. Regarding the five AVMs, four were treated with microsurgical approach achieving total resection of the lesion and normal developmental milestones except for one patient with brainstem AVM that was admitted with quadriparesis and coma. In the patient with basal ganglia AVM, the lesion spontaneously disappeared. From the two pial AVFs, the frontal one was microsurgically treated and the spinal one received endovascular session achieving 50% of lesion occlusion for which further treatment is needed. The two cavernous malformations were operated achieving total resection with normal development. The two children with DSM received surgical resection without complications and normal development. The rare case of sacular aneurysm at this age was occluded at the time of diagnostic procedure due to the bad clinical condition of the patient and the hematoma was removed immediately in the operating room. The only case of DAVF died of respiratory intercurrence after three sessions of endovascular treatment., Conclusions: In this neonatal age group (first year of life), we can find the whole range of neurosurgical vascular pathology: VGAM, AVM, cavernous malformation, DSM, pial AVF, DAVF, and sacular aneurysm. VGAM is the most frequent vascular malformation during the first year of age. The early treatment of vascular malformations prevents its adverse effects on a developing brain. A multidisciplinary team composed by endovascular and surgical specialists is necessary to discuss and treat each case.

Aneurysm of the vein of Galen is a rare congenital vascular malformation of the central nervous system. There are controversies in the literature regarding the possible prognostic value of aneurysmal volume estimation with 3-dimensional US for the prediction of fetal outcome. In this report, we present a case of prenatally diagnosed large aneurysm of the vein of Galen complicated by heart failure in a fetus. The volume of the malformation was calculated as 17.8 cm(3) and the fetal outcome was poor., (© 2010 Wiley Periodicals, Inc.)

Background: A vein of Galen aneurysmal malformation (VGAM) is a relatively rare vascular malformation, often resulting in high morbidity and mortality. While surgical arterial clipping has been reported for decades, results in the literature have recently favored endovascular treatment., Methods: During a 10-year period, all children who were diagnosed with VGAM were included in our follow-up study. Clinical and radiological records of 5 consecutive patients were reviewed., Results: 5 children (4 infants and 1 child) who suffered from symptoms caused by VGAM were treated by means of transarterial embolization with N-butyl cyanoacrylate (NBCA) alone. Their age at the time of diagnosis ranged from 4 months to 3 years. 4 of the 5 patients presented with macrocranium, and 3 of those 4 patients were infants. The fifth patient presented with seizures. None of the patients presented with hemorrhage. 2 of the 5 VGAM patients were classified as the mural type, while the others 3 were of the choroidal type. Both mural type patients achieved total obliteration of lesions with good outcomes. One of the remaining 3 patients exhibited autistic behavior during late follow-up, whereas the other 2 had good outcomes. 2 patients suffered from asymptomatic ruptured arterial feeders during embolization, which were treated immediately with glue embolization., Conclusion: Our purpose in treating a patient with VGAM is to achieve normal brain development using minimally invasive interventions. Our treatment strategies were influenced by each patient's clinical status, their ages, and varying radiographic features. We achieved considerable successful in treating our patients by means of transarterial embolization alone., (© Georg Thieme Verlag KG Stuttgart · New York.)

Little to no pediatric or neurosurgical literature has been published about the complications of ventriculoperitoneal shunt procedures for hydrocephalus associated with vein of Galen malformations in childhood. The interventional neuroradiology literature, however, suggests that ventriculoperitoneal shunting as first-line treatment for hydrocephalus in children with vein of Galen malformations is fraught with short- and long-term dangers, including status epilepticus, intraventricular hemorrhage, subdural hematoma and hygroma, venous infarction, malignant dystrophic calcification, and worsening developmental delay. We present a single pediatric case where a ventriculoperitoneal shunt procedure for symptomatic hydrocephalus seemed to be the major contributing factor to the rapid neurological deterioration and eventual death of an infant with a vein of Galen malformation. Based on this experience and our review of the literature, we suggest the use of endovascular embolization of the vein of Galen malformation to reestablish a balance in hydrovenous dynamics as first-line treatment rather than directly addressing hydrocephalus with CSF diversion. The ventriculoperitoneal shunt procedure should be reserved for cases with symptomatic hydrocephalus in which the patient is a poor candidate for embolization, or for cases where endovascular therapy has already been maximized. The role of endoscopy in the treatment of hydrocephalus associated with vein of Galen malformations is not clear., ((c) 2010 S. Karger AG, Basel.)

Introduction: Vein of Galen aneurysmal malformations (VGAM) are rare but clinically significant intracranial arteriovenous shunt lesions that most often present in neonates and infants., Methods: Retrospective clinical data were collected for patients evaluated with a diagnosis of VGAM from 1994 to 2007., Results: Thirteen patients with VGAM were evaluated from 1994 to 2007. Seven patients presented emergently with medically intractable cardiac failure, and six were treated in the first 2 weeks of life. Five children treated after this period (1.5-31 months of age) manifested enlarging head circumference, abnormal development, or subarachnoid hemorrhage. Eleven patients were managed endovascularly. Four disease or procedure-related complications occurred. Two complications were associated with poor outcome, both of which occurred in patients treated at less than 2 weeks of age. Two other patients experienced transient neurological deficits with no evidence of permanent sequelae. Outcome in the six patients treated emergently in the first 2 weeks of life included two patients who developed normally, one with mild to moderate neurological deficits, one with severe neurological deficits, and two deaths. Outcome in the five older patients (treated between 1.5 and 31 months) was considerably better than in the group treated early and included three with normal outcome and two with mild neurological deficits., Conclusions: Contemporary endovascular techniques remain the preferred treatment for VGAM in all age groups. Early diagnosis and multimodality treatment are essential for the best management and treatment of the complex constellation of clinical problems often arising from this disorder.

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.

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.

The vein of Galen malformation (VGM) is a rare congenital cerebral vascular malformation. In most cases it already has been diagnosed prenatally. The impact of heart failure due to the arteriovenous shunt is relevant for the prognosis.Prognosis of neonatal symptomatic VGM with early heart failure is severe. Therefore an interdisciplinary cooperation is necessary and it is important to treat patients in a clinic with a perinatal center and a neuroradiology and neurosurgery that is experienced in treating such patients. The genesis, the pathophysiology, the diagnostic agents, the therapeutic agents and the prognosis of VGM will be described.