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Vein of Galen malformation (VOGM) is a congenital, intracranial vascular malformation, with an extracardiac shunt. Neonates can present with high output cardiac failure, pulmonary hypertension, or multiorgan failure and are at high risk of perioperative complications, especially in remote locations. We conducted a retrospective single-center analysis of the perioperative management of patients with VOGM presenting for embolization. Patients were identified by querying both the hospital billing dataset using International Classification of Diseases-10 diagnosis or billing code and the Neuro-interventional Radiology Database, from January 2011 to March 2020. As many as 14 patients were identified, 12 of which underwent definitive treatment. Six patients who underwent embolization in the neonatal period had pulmonary hypertension. Those children required varying degrees of hemodynamic and respiratory support preoperatively and experienced significant intraoperative events, including one intraoperative cardiac arrest. Caring for these critically ill patients in a remote location requires proper planning to prevent adverse outcomes.

Vein of Galen malformation (VOGM) is a congenital, intracranial vascular malformation, with an extracardiac shunt. Neonates can present with high output cardiac failure, pulmonary hypertension, or multiorgan failure and are at high risk of perioperative complications, especially in remote locations. We conducted a retrospective single-center analysis of the perioperative management of patients with VOGM presenting for embolization. Patients were identified by querying both the hospital billing dataset using International Classification of Diseases-10 diagnosis or billing code and the Neuro-interventional Radiology Database, from January 2011 to March 2020. As many as 14 patients were identified, 12 of which underwent definitive treatment. Six patients who underwent embolization in the neonatal period had pulmonary hypertension. Those children required varying degrees of hemodynamic and respiratory support preoperatively and experienced significant intraoperative events, including one intraoperative cardiac arrest. Caring for these critically ill patients in a remote location requires proper planning to prevent adverse outcomes. [ABSTRACT FROM AUTHOR]

Vein of Galen aneurysmal malformation (VGAM) is a rare vascular anomaly originating during embryonic development, specifically between the 6th and 11th weeks of gestation. This malformation results from abnormal arteriovenous connections between primitive choroidal arteries and the median prosencephalic vein (MPV) of Markowski. Typically, the MPV regresses by the 11th week, but in VGAM, this regression is hindered, leading to persistent abnormal flow and the formation of arteriovenous shunts. We present a case of successful prenatal detection, as well as a comprehensive literature review that summarizes current knowledge, emphasizes the importance of prenatal detection, detailed imaging techniques, understanding clinical presentations, and outlines treatment options. Prenatal detection, crucial for early intervention, has become feasible through ultrasonography and MRI. Fetal MRI has emerged as the gold standard, offering detailed insights into arterial feeders, nidus presence, fistula position, venous drainage, and potential complications. The clinical presentation of VGAM varies with age, and neonates diagnosed in utero may exhibit signs of high-output cardiac failure. Early detection is critical for timely intervention, as untreated VGAMs often result in high mortality rates. Prognosis depends on the severity of heart failure, the number of arteriovenous shunts, and the presence of accompanying fetal abnormalities. Various imaging modalities, including CT angiography and digital subtraction angiography (DSA), aid in the assessment and treatment of VGAM. DSA remains the gold standard for evaluating angioarchitecture and guiding endovascular interventions. The optimal treatment for VGAM is transarterial embolization, offering significant improvements in prognosis. Surgical interventions are limited due to high morbidity and mortality. Management decisions should consider the balance between minimizing neurological damage and achieving maximum embolization effectiveness.

Vein of Galen aneurysmal malformation (VGAM) is a rare congenital malformation characterised by arteriovenous fistulas between primitive choroidal arteries and the median prosencephalic vein, the embryonic precursor to the vein of Galen. Endovascular techniques have changed the management of these patients with improved prognosis. An eight-month-old with VGAM managed by endovascular embolisation using ethylene vinyl alcohol copolymer (EVOH) developed a chemical abscess - a rare complication. It was managed conservatively and showed promising clinical outcome. Contribution: Chemical abscesses following EVOH embolisation are scarce -- with imaging differentials, which include brain abscess and onyx granuloma. Knowledge and successful identification of this entity are essential as its management as prognoses differ. Chemical abscess is managed conservatively and has a good prognosis. [ABSTRACT FROM AUTHOR]

Background: Vein of Galen malformation (VGM) is a congenital intracranial vascular anomaly consisting of arteriovenous fistulas and/or malformations between various arterial feeders and the median prosencephalic vein of Markowski (MPV). Despite its rare occurrence, VGM is of particular clinical relevance, as the excessive intracranial shunt volume leads to high mortality without appropriate treatment. Methods: The objective of this article is to review the published data on neurointerventional treatment and compare outcome quality in the included studies. Eight studies were included and synthesized. One study was multicentric and the rest were retrospective monocentric (level 4 evidence studies according to the Oxford Centre for Evidence-based Medicine). Results: The total number of included patients was 480 and patient age ranged from 1 day to 18 years. Mild or severe heart failure, hydrocephalus, and other reasons led to the indication for neurointerventional treatment, which was performed in all studies in the form of embolization. Under consideration of the introduced semiquantitative multidimensional scoring system, the highest total score, i.e., the best outcome quality, was found for the study “Houston” 2002–2018 (19 points) and the study “Duisburg” 2001–2010 (19 points). Conclusions: Neurointerventional treatment represents the essential pillar in the interdisciplinary management of patients with VGM, although standardization is lacking—based on the results of the structured review. As complementary treatments, pediatric critical care is mandatory and includes medical hemodynamic stabilization.

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Children can have a variety of intracranial vascular anomalies ranging from small and incidental with no clinical consequences to complex lesions that can cause substantial neurologic deficits, heart failure, or profoundly affect development. In contrast to high-flow lesions with direct arterial-to-venous shunts, low-flow lesions such as cavernous malformations are associated with a lower likelihood of substantial hemorrhage, and a more benign course. Management of vascular anomalies in children has to incorporate an understanding of how treatment strategies may affect the normal development of the central nervous system. In this review, we discuss the etiologies, epidemiology, natural history, and genetic risk factors of three high-flow vascular malformations seen in children: brain arteriovenous malformations, intracranial dural arteriovenous fistulas, and vein of Galen malformations.

Introduction: Computational fluid dynamics (CFD) assess biological systems based on specific boundary conditions. We propose modeling more advanced hemodynamic metrics, such as core line length (CL) and critical points which characterize complexity of flow in the context of cerebral vasculature, and specifically cerebral veins during the physiologically evolving early neonatal state of vein of Galen malformations (VOGM). CFD has not been applied to the study of arteriovenous shunting in Vein of Galen Malformations but could help illustrate the pathophysiology of this malformation. Methods: Three neonatal patients with VOGM at Boston Children’s Hospital met inclusion criteria for this study. Structural MRI data was segmented to generate a mesh of the VOGM and venous outflow. Boundary condition flow velocity was derived from PC-MR sequences with arterial and venous dual velocity encoding. The mesh and boundary conditions were applied to model the cerebral venous flow. We computed flow variables including mean wall shear stress (WSSmean), mean OSI, CL, and the mean number of critical points (nCrPointsmean) for each patient specific model. A critical point is defined as the location where the shear stress vector field is zero (stationary point) and can be used to describe complexity of flow. Results: The division of flow into the left and right venous outflow was comparable between PC-MR and CFD modeling. A high complexity recirculating flow pattern observed on PC-MR was also identified on CFD modeling. Regions of similar WSSmean and OSImean (<1.3 fold) in the left and right venous outflow channels of a single patient have several-fold magnitude difference in higher order hemodynamic metrics (> 3.3 fold CL, > 1.7 fold nCrPointsmean). Specifically, the side which developed JBS in each model had greater nCrPointsmean compared to the jugular bulb with no stenosis (VOGM1: 4.49 vs. 2.53, VOGM2: 1.94 vs. 0, VOGM3: 1 vs. 0). Biologically, these regions had subsequently divergent development, with increased complexity of flow associating with venous stenosis. Discussion: Advanced metrics of flow complexity identified in computational models may reflect observed flow phenomena not fully characterized by primary or secondary hemodynamic parameters. These advanced metrics may indicate physiological states that impact development of jugular bulb stenosis in VOGM. [ABSTRACT FROM AUTHOR]

The Galenic venous system plays a vital role in the drainage of blood from deeper parts of the brain. This venous system is contributed by many major veins. These veins are located closer to the pineal gland making the surgical approach in this region difficult. Any accidental injury or occlusion of the vein of Galen could lead to devasting results. Thus, studying the dimensions of the vein of Galen is more important. Hence, we aimed to evaluate the morphometry and trajectory to the vein of Galen. About 100 computed tomographic venography records were evaluated and the length, diameter of vein of Galen, angle between straight sinus and vein of Galen and distance from internal occipital protuberance and roof of fourth ventricle to vein of Galen were studied. The mean length and diameter of vein of Galen were 9.8±2.7 and 4.08±1.04 respectively. The mean angle between straight sinus and vein of Galen was 64.2°. The mean distance between external occipital protuberance and roof of fourth ventricle to vein of Galen were 52±6.9 and 33.3±4.5 respectively. No significant morphometric differences were observed between the age groups as well as between the sexs. The results obtained from this study may be helpful for the neurosurgeons in better understanding of the anatomy of the Galenic venous system and to adopt a safe surgical approach to improve the efficacy of the surgeries of the pineal gland and also in the region of vein of Galen. [ABSTRACT FROM AUTHOR]

Background: Since the advent of endovascular treatment, the long-term prognosis of vein of Galen malformation (VOGM) has markedly improved; however, the nature of research leading to this point is unclear. The objective of this study was to define the composition of VOGM research to date, by means of a bibliometric analysis of the 100 most cited VOGM articles. Methods: An electronic search of Elsevier's Scopus database was performed to identify the 100 most cited articles on VOGM screened against predetermined criteria. Data were then compared. Results: The 100 most cited VOGM articles were published between 1974 and 2017 in 38 unique journals and originated from 16 unique countries. Mean citation count and rate were 59.4 citations and 2.9 citations/year, respectively. The USA (n = 42); Hôpital de Bicêtre, France (n = 15); and Dr. Pierre Lasjaunias (n = 16) were the largest individual country, institutional, and author contributors. Compared to the older articles (published < 2000), key differences for newer articles were statistically higher citation rates (P < 0.01), more authors (P < 0.01), higher proportion of endovascular treatment descriptions (P = 0.01), and more originating from Asia Pacific (P < 0.01). Conclusions: From the 100 most cited VOGM articles to date, there has been a noticeable shift from diagnosing VOGM based on the foundational work by Dr. Lasjaunias to understanding how we can model clinical outcomes now that endovascular treatment has become the standard of care. Significant shifts in prognosis are pending, and the current bibliometric data implicate we are on the precipice of more recent works making an impact in the near future. [ABSTRACT FROM AUTHOR]

Introduction: Computational fluid dynamics (CFD) assess biological systems based on specific boundary conditions. We propose modeling more advanced hemodynamic metrics, such as core line length (CL) and critical points which characterize complexity of flow in the context of cerebral vasculature, and specifically cerebral veins during the physiologically evolving early neonatal state of vein of Galen malformations (VOGM). CFD has not been applied to the study of arteriovenous shunting in Vein of Galen Malformations but could help illustrate the pathophysiology of this malformation.Methods: Three neonatal patients with VOGM at Boston Children’s Hospital met inclusion criteria for this study. Structural MRI data was segmented to generate a mesh of the VOGM and venous outflow. Boundary condition flow velocity was derived from PC-MR sequences with arterial and venous dual velocity encoding. The mesh and boundary conditions were applied to model the cerebral venous flow. We computed flow variables including mean wall shear stress (WSSmean), mean OSI, CL, and the mean number of critical points (nCrPointsmean) for each patient specific model. A critical point is defined as the location where the shear stress vector field is zero (stationary point) and can be used to describe complexity of flow.Results: The division of flow into the left and right venous outflow was comparable between PC-MR and CFD modeling. A high complexity recirculating flow pattern observed on PC-MR was also identified on CFD modeling. Regions of similar WSSmean and OSImean ( 3.3 fold CL, > 1.7 fold nCrPointsmean). Specifically, the side which developed JBS in each model had greater nCrPointsmean compared to the jugular bulb with no stenosis (VOGM1: 4.49 vs. 2.53, VOGM2: 1.94 vs. 0, VOGM3: 1 vs. 0). Biologically, these regions had subsequently divergent development, with increased complexity of flow associating with venous stenosis. Discussion: Advanced metrics of flow complexity identified in computational models may reflect observed flow phenomena not fully characterized by primary or secondary hemodynamic parameters. These advanced metrics may indicate physiological states that impact development of jugular bulb stenosis in VOGM.

Endovascular embolization is the primary strategy in the management for vein of Galen malformations (VOGM). However, despite significant advances in endovascular embolization technologies and techniques, VOGMs remain very technically challenging lesions largely due to the high-flow arteriovenous shunts present in these malformations. A variety of advanced flow-control techniques can be implemented to mitigate the risk of venous escape and increase the safety and efficacy of endovascular treatment. These techniques include regionally targeted strategies (transvenous embolization and balloon-assisted transarterial embolization) and global flow-control methods (pharmacologic cardiac arrest and rapid ventricular overdrive pacing). Each of these strategies are associated with unique advantages and disadvantages, highlighting the importance of a patient-specific approach when treating these challenging lesions.

Vein of Galen aneurysmal malformation (VGAM) is a rare intracranial vascular malformation which typically presents in a critically ill newborn with intractable heart failure, severe pulmonary hypertension, and right heart dilation. We report two unusual cases of neonates with VGAM and anomalous connection of right superior vena cava to the left atrium. Both neonates were diagnosed with VGAM in utero and were clinically stable after birth with dilation of the left atrium and left ventricle and no evidence of pulmonary hypertension. One case with hydrocephalus underwent transcatheter embolization at 1 week of age. The other case without hydrocephalus underwent elective transcatheter embolization at 4 months. We postulate that the presence of a right superior vena cava to the left atrium provides a physiological advantage and counters the left-to-right shunt from the arteriovenous malformation. This provides insight to a potential treatment strategy to improve outcomes in patients with severe heart failure and pulmonary hypertension secondary to VGAM. [ABSTRACT FROM AUTHOR]

During the first days of neonatal growth, the central nervous system (CNS) develops self-regulatory mechanisms to ensure constant cerebral perfusion. However, this vascular neogenesis takes place at a microscopic scale that cannot be observed with current clinical imaging techniques. Ultrasound localization microscopy (ULM) allows us to observe micro-vessels of the order of a few microns at depths of several centimeters. This can be done using conventional clinical ultrasound scanners and contrast sequences (CEUS). In this study, ULM is used to observe the human microvasculature in neonatal patients undergoing treatment for life-threatening malformations forming direct connections between the cerebral arterial and venous systems. It is observed that neuroendovascular treatment of neonatal arteriovenous malformations causes remodeling and reorganization of the cerebral vasculature by also activating corticomedullary vascular connections. ULM enables us to follow microvascular changes in human neonates with high spatio-temporal resolution. ULM may provide a novel clinical translatable tool, particularly including cerebral imaging in very young patients., (© 2025 The Author(s). Advanced Science published by Wiley‐VCH GmbH.)

Background: Achieving distal access and flow control are of significant importance for the treatment of intracerebral arteriovenous shunting lesions. The Scepter Mini catheter is a low-profile, dual-lumen balloon catheter, designed to provide navigability in small-caliber, tortuous intracranial vessels. Objective: To describe the initial experience of the Scepter Mini catheter in the treatment of pediatric arteriovenous malformations and fistulas. Methods: A single-institution, retrospective chart review identified all consecutive uses of the Scepter Mini catheter for endovascular embolization of vascular malformations in the pediatric population. Results: Three different arterial pedicles were embolized with the Scepter Mini catheter in two different patients. One patient was diagnosed with a vein of Galen malformation that had undergone multiple treatments and the other with a torcular dural arteriovenous fistula. All cases encompassed quite challenging tortuosity of small-caliber feeders which prevented the use of another microcatheter. The Scepter Mini catheter navigated into feeding arteries of diameters 0.65, 1.9, and 1.25 mm, and its balloon was inflated to achieve excellent blood flow control. Total obliteration (100%) of the shunting lesion was achieved in both cases. No reflux, pedicle rupture or other untoward effects were observed. Both patients had an uneventful recovery. Conclusion: The Scepter Mini catheter afforded fast and safe distal access, flow control, and treatment of arteriovenous malformations in this initial pediatric cohort. The catheter's low profile and easy navigability should support its use in tortuous and small arterial feeders, especially in the pediatric population. [ABSTRACT FROM AUTHOR]

Vein of Galen malformation (VOGM) is a rare type of arteriovenous malformation with majority presenting in neonatal period. The most common presentation is high output cardiac failure and association with persistent pulmonary hypertension (PPHN) is rarely reported. If left untreated mortality rate is very high. Endovascular embolization is the preferred treatment modality at present. We describe a full-term male baby presented at 1 h of life with respiratory distress, eventually diagnosed to have PPHN on echocardiography. Ultrasonography cranium done in view of cranial bruit revealed VOGM which later confirmed with magnetic resonance (MR) imaging brain and MR angio and venography. All supportive measures were provided. But because of hemodynamic instability endovascular embolization could not be attempted and baby succumbed due to severe refractory PPHN. We should consider the possibility of VOGM in all refractory cases of PPHN even though the association is very rare.

Objectives: Vein of Galen Malformation (VGAM) is a rare congenital cerebrovascular anomaly. Early detection and monitoring of concurrent fetal heart failure in VGAM are crucial for improving outcomes. The study aims to evaluate heart anatomy, systolic and diastolic heart function, and indicators of heart failure in fetuses referred to a tertiary center due to VGAM detected in the second or third trimester., Material and Methods: This single-center retrospective study of echocardiographic data from five fetuses with VGAM examined between 2008 and 2023. Parameters analyzed included gestational age, reason for referral, cardiovascular profile score (CVPS), systolic and diastolic heart function and selected cardiac parameters RESULTS: Cardiomegaly as a sign of congestive heart failure was the main reason for referral most of the fetuses (4/5 - 80%) diagnosed later as VGAM in our center. Abnormal cerebral vessel flow was visualized in all cases, with normal umbilical arterial flow. Three fetuses were treated prenatally with digoxin due to congestive heart failure. Monophasic tricuspid valve inflow and decreased shortening fraction (SF) were observed in three fetuses (60%) at the time of diagnosis. CVPS scores ranged from 2 to 10, correlating with the severity of cardiac compromise. All five patients demised, three died in utero, and two shortly after birth., Conclusions: This study emphasizes the severe cardiac implications of VGAM detected in utero and the need for early and comprehensive fetal assessment. Despite early diagnosis, outcomes remain poor, necessitating further research into effective prenatal treatments and management protocols to enhance survival for affected fetuses.

Vein of Galen malformations are the most common congenital neurovascular malformation and are a type of choroidal arteriovenous fistula involving the midline primitive choroidal venous circulation. The arteriovenous shunt zone of a VOGM may directly involve the embryonic precursor of the vein of Galen and/or its tributaries within the 3rd ventricle tela choroidea. Dural sinus malformations are characterized by dilated intracranial dural venous sinuses, some of which acquire multifocal arteriovenous shunts within the dural walls of these overgrown venous sinuses.Pial arteriovenous fistulae are high-flow shunts representing direct arterial to venous communication of pial blood vessels, with no definable nidus., Competing Interests: Disclosure The authors have no conflicts of interest to declare., (Crown Copyright © 2024. Published by Elsevier Inc. All rights reserved.)

Introduction: The purpose of this report is to present a rare case of Hirayama disease (HD) in a patient with a history of late-onset symptomatic vein of Galen aneurysmal malformation (VGAM). This report may provide new insights into the pathophysiology of HD, a rare disorder consisting of insidious onset of unilateral weakness and atrophy of the forearm and intrinsic hand muscles. These symptoms are believed to result from cervical myelopathy affecting the anterior horn cells due to abnormal compressive forces on the spinal cord from adjacent anatomical structures (i.e., dura and/or epidural veins), but this has not been proven. VGAM is a rare congenital cerebral vascular malformation, consisting of high-flow arteriovenous shunting between a persistent median prosencephalic vein and arterial feeders, which most frequently presents in the early neonatal period with congestive heart failure. Case Presentation: We report the case of an otherwise healthy boy who presented with heart failure due to VGAM at 7 years and subsequently presented at 14 years with left-sided HD. His cervical MRI with neck flexion revealed enlarged epidural veins at the C5–C6 spinal level with anterior, leftward displacement of the posterior dura and spinal cord as well as left hemicord flattening and/or atrophy at this level. He underwent successful surgical treatment by C5–C6–C7 osteoplastic laminotomies and tenting and expansile autologous duraplasty, during which enlarged, engorged epidural veins were confirmed and coagulated. This halted the progression of his left hand weakness and atrophy and allowed significant functional improvement. Postoperative catheter angiogram showed no anatomical connection between the persistent VGAM and the engorged epidural veins, and genetic testing revealed no genetic predisposition of vascular malformations. Discussion/Conclusion: In this patient, a combined surgical technique was performed that included epidural venous plexus coagulation with posterior autologous duraplasty and dural fixation using tenting sutures. This combined approach led to a favorable clinical and radiographic outcome with no complications or limitations and has not been previously proposed in the literature, to our knowledge. Although not completely ruled out, we found no angiographic connection or genetic predisposition to suggest there is a pathophysiological link between HD and VGAM. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Vein of Galen malformation (VOGM) is a fistulous arteriovenous malformation presenting in the early childhood. Hydrocephalus with VOGM develops in one half of patients during the course of the disease. The concept of hydrodynamic disorders is the key to understand the development of hydrocephalus. VOGM results in venous hypertension that secondarily disturbs cerebrospinal fluid (CSF) absorption leading to hydrocephalus and occurs frequently in infants and young children. The medullary veins are thought to be the main pathway for absorption of CSF by the cerebrofugal gradient act as the driving force. In neonates, the cavernous sinus is poorly developed and brain does not use it for venous drainage along with poor jugular bulb maturation results in poor venous drainage reserve. The presence of high flow vascular malformation with poor venous drainage reserve leads to hydrodynamic disorder, poor CSF absorption, and hydrocephalus. Apart from this, hydrocephalus secondary to intraventricular hemorrhage and physical obstruction of the enlarged VOGM at the aqueduct has been proposed. The management strategy is to perform timely endovascular treatment to correct the hydrodynamic disorder and avoid ventricular shunting. Trans-arterial embolization is the effective way, as it decreases flow in the malformation, secondarily the venous hypertension, and thereby improving the clinical symptoms related to hydrodynamic disorder. Ventricular diversion procedure is indicated in symptomatic hydrocephalus after exhausting our effort to reduce hydrodynamic pressure by endovascular embolization. [ABSTRACT FROM AUTHOR]

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

The vein of Galen malformation is caused by an abnormal shunting between choroidal arteries and the median prosencephalic vein during embryological development, leading to increased blood flow to the deep cerebral veins, intracranial damage, and systemic repercussions. Idiopathic spontaneous thrombosis of a vein of Galen malformation is rare, and its association with acute sinusitis has not been reported in the literature. We present the case of a girl with a postnatal diagnosis of a vein of Galen malformation at the age of 16 months, with secondary pulmonary hypertension that was adequately controlled with spironolactone. At 3 years old, while expecting elective endovascular treatment, the patient developed spontaneous thrombosis of the vein of Galen malformation, concomitant to an acute sinusitis episode, with complete resolution of the vascular malformation and secondary pulmonary hypertension. The patient continued with normal neurological development over a 5-year follow-up. We discuss the main pathophysiologic mechanisms that can explain spontaneous thrombosis of VOGMs and the patient's outcome. Awareness of different mechanisms that can lead to spontaneous thrombosis can help in the decision-making process and prompt targeted approaches to individual patients with a vein of Galen malformation. [ABSTRACT FROM AUTHOR]

We describe the case of a neonate with signs of heart failure. Echocardiography showed a structural normal heart shape with left ventricular dysfunction. At 2 months of age, a vein of Galen arteriovenous malformation was diagnosed through a brain magnetic resonance imaging. Embolization therapy was accomplished and a clinical and neurological follow-up was started. This clinical case highlights how important it is considering an intracranial cause in the differential diagnosis of neonatal congestive heart failure (CHF). We performed a narrative minireview of the literature about treatments and outcome of this malformation in association to CHF, to point out how complex the diagnosis of vein of Galen aneurysmal malformation (VGAM) may be and how an early diagnosis is important for its management. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Vein of Galen malformation (VGM) is a rare congenital intracerebral vascular malformation. The aim of this study was to gain reliable data about epidemiology; clinical manifestations; and pre-, peri- and postinterventional treatment strategies in Germany.Methods: This prospective epidemiologic study was conducted by Erhebungseinheit für Seltene Pädiatrische Erkrankungen in Deutschland, a hospital-based German nationwide surveillance unit for rare pediatric diseases. Patients aged upto 18 years with a new diagnosis of VGM were prospectively included between January 1, 2014 and December 31, 2015.Results: In the two-year study period, 30 individuals with newly diagnosed VGM were identified, resulting in an estimated annual incidence rate of VGM in Germany of at least 1: 58,100 live births. The main symptoms were age dependent. Neonates presented with mainly cardiac manifestations of high output failure; infants and older children mainly suffered from neurological complications. Of the patients with endovascular treatment 95% survived, but only 41% of these patients were discharged home without any complications. The Bicêtre Neonatal Evaluation Score was a good predictor for poor outcome but could have led to palliative care in some patients with good outcome.Conclusions: The incidence of VGM in Germany is estimated to be 1:58,100 live births. The majority of patients were eligible for endovascular treatment. Nevertheless, due to side effects and long-term sequelae in the majority of patients, further improvement in care of children with VGM is needed. [ABSTRACT FROM AUTHOR]

The endovascular approach is the standard treatment of vein of Galen malformations (VOGMs,) however the morbidity and mortality rates of VOGMs treated by embolization are still significant. Embolization with ethylene vinyl alcohol copolymer (EVOH) by a dual-lumen balloon (DLB) is a promising. In the present case we demonstrated two strategies of transarterial (TA) embolization of a mural type VOGM presenting with two direct shunts. The first shunt was embolized using coils to reduce the flow through the shunt, following by an EVOH injection which resulted in the complete occlusion of that shunt. The second shunt was embolized also with EVOH but through a DLB. In the second embolization a very precise cast of EVOH could be created resulting in a complete occlusion of the VOGM. This case demonstrated the advantages of DLB compared to other embolization techniques

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.)

Vein of Galen malformation (VOGM) is a rare type of arteriovenous malformation with majority presenting in neonatal period. The most common presentation is high output cardiac failure and association with persistent pulmonary hypertension (PPHN) is rarely reported. If left untreated mortality rate is very high. Endovascular embolization is the preferred treatment modality at present. We describe a full-term male baby presented at 1 h of life with respiratory distress, eventually diagnosed to have PPHN on echocardiography. Ultrasonography cranium done in view of cranial bruit revealed VOGM which later confirmed with magnetic resonance (MR) imaging brain and MR angio and venography. All supportive measures were provided. But because of hemodynamic instability endovascular embolization could not be attempted and baby succumbed due to severe refractory PPHN. We should consider the possibility of VOGM in all refractory cases of PPHN even though the association is very rare. [ABSTRACT FROM AUTHOR]

Background: We determined the frequency of cerebrovascular malformations in a pediatric cohort with hereditary hemorrhagic telangiectasia.Methods: Retrospective cohort study of 54 children diagnosed with hereditary hemorrhagic telangiectasia at a tertiary care center. All neuroimaging was reviewed to assess for number and types of cerebrovascular malformations and for intracerebral hemorrhage and arterial ischemic stroke. Clinical charts were reviewed for clinical manifestations, genetic mutation, and clinically evident intracerebral hemorrhages and arterial ischemic strokes.Results: Among 54 children with hereditary hemorrhagic telangiectasia with a median age of 3.5 years (interquartile range 0.4 to 7.9 years) at diagnosis, neuroimaging was performed in 52 (96.3%) at a median age of 5.2 years (interquartile range 1.8 to 9 years). Fourteen of 52 imaged children (26.9%) had cerebrovascular malformations. Cerebrovascular malformations included arteriovenous malformations, arteriovenous fistulas, vein of Galen malformations, and developmental venous anomalies. Six of the 14 children with cerebrovascular malformations (42.9%) had multiple malformations. Three children developed new cerebral arteriovenous malformations over time. Six children (11.1%) had clinically evident intracerebral hemorrhage, arterial ischemic stroke, or transient ischemic attack. The three children with intracerebral hemorrhage presented at young ages (4.3 to 7.7 years).Conclusions: More than a quarter of children with hereditary hemorrhagic telangiectasia who were imaged had cerebrovascular malformations, and overt stroke occurred in more than 10%. Intracerebral hemorrhages can occur in pediatric hereditary hemorrhagic telangiectasia patients at young ages, and new cerebral arteriovenous malformations may develop over time. Early screening with neuroimaging including neurovascular imaging as well as repeat neuroimaging may be warranted in children with hereditary hemorrhagic telangiectasia. [ABSTRACT FROM AUTHOR]

Cerebrovascular malformations are uncommon diverse group of dysmorphic vascular communications that may occur sporadically or as part of genetic syndromes. These include non-neoplastic lesions such as arteriovenous malformations (AVM), cavernous malformations (CM), developmental venous anomalies (DVA), and telangiectasias as well as others like arteriovenous fistulas (AVF), vein of Galen malformations (VOGM), and mixed or unclassified angiomas. These lesions often carry a high degree of morbidity and mortality often requiring surgical or endovascular interventions. The field of cerebrovascular anomalies has seen considerable advancement in the last few years. Treatment and management options of various types of brain anomalies have evolved in neurological, neurosurgical, and neuro-interventional radiology arena. The use of radiological imaging studies is a critical element for treatment of such neurosurgical cases. As imaging modalities continue to evolve at a rapid pace, it is imperative for neurological surgeons to be familiar with current imaging modalities essential for a precise diagnosis. Better understanding of these cerebrovascular lesions along with their associated imaging findings assists in determining the appropriate treatment options. In the current review, authors highlight various cerebrovascular malformations and their current imaging modalities. [ABSTRACT FROM AUTHOR]

Vein of Galen malformations (VoGMs) are rare vascular malformations resulting from persistent shunting of primitive choroidal vessels into the median prosencephalic vein of Markowski. VoGMs are associated with poor clinical outcome with a reported 76.7% mortality if left untreated. We present an exceedingly rare case of a giant, untreated VoGM measuring 7.8 × 5.5 × 7 cm in a 42-year-old man. The embryologic origin, classification, clinical manifestations, and treatment options of VoGMs are discussed with a review of pertinent literature.

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

Vein of Galen aneurysmal malformation (VGAM) is a rare congenital malformation characterised by arteriovenous fistulas between primitive choroidal arteries and the median prosencephalic vein, the embryonic precursor to the vein of Galen. Endovascular techniques have changed the management of these patients with improved prognosis. An eight-month-old with VGAM managed by endovascular embolisation using ethylene vinyl alcohol copolymer (EVOH) developed a chemical abscess - a rare complication. It was managed conservatively and showed promising clinical outcome., Contribution: Chemical abscesses following EVOH embolisation are scarce - with imaging differentials, which include brain abscess and onyx granuloma. Knowledge and successful identification of this entity are essential as its management as prognoses differ. Chemical abscess is managed conservatively and has a good prognosis., Competing Interests: The authors declare that they have no financial or personal relationships that may have inappropriately influenced them in writing this article., (© 2024. The Authors.)

Objective: Vein of Galenaneurysmal malformation (VGAM) is a rare but important congenital malformation presenting to neonatal intensive care units (NICUs), and with a change from surgical to endovascular management, survival for this condition has improved. However, there is little reported about the medical management decisions of infants with this condition and the associated long-term neurodevelopmental outcomes. We aim to report a single centre experience of both acute treatment and long-term outcomes of VGAM for those infants admitted to our NICU soon after birth.Design: Retrospective cohort study over a 15-year period from 2001 to 2015 inclusive.Setting: A quaternary NICU at The Royal Children's Hospital, Melbourne, Australia.Participants: 24 newborn infants referred for management of VGAM. There were no eligibility criteria set for this study; all presenting infants were included.Interventions: None.Main Outcomes Measures: Clinical neuroimaging data were gathered. Surviving children were formally assessed with a battery of tests administered by a neuropsychologist and occupational therapist/physiotherapist at various ages across early to middle childhood.Results: Fifteen neonates with VGAM did not survive beyond their NICU admission. 10 of these were not offered endovascular intervention. Of the nine surviving infants, only one had a normal neurodevelopmental outcome.Conclusions: The mortality of VGAM presenting in the neonatal period was high, and rates of normal neurodevelopmental outcome for survivors were low. These findings contribute to our understanding of which neonates should be treated and highlights the importance of providing clinical neurodevelopmental follow-up to survivors beyond their infant years. [ABSTRACT FROM AUTHOR]

Vein of Galen malformations (VoGMs) induce cerebrovascular dysfunction through arterial steal and venous hypertension resulting, if untreated, in severe neurologic morbidity and mortality. Noninvasive techniques for quantitative, serial evaluation of cerebrovascular hemodynamics in VoGMs are lacking. This proof of concept study using quantitative blood flow measurements from 4-dimensional flow magnetic resonance imaging may be useful as a noninvasive biomarker to guide timing of intervention and assess disease progression and treatment outcomes. Between July 2016 and July 2018, 4 patients harboring VoGMs underwent P hase C ontrast V astly undersampled I sotropic PR ojection (PCVIPR) imaging at the University of Wisconsin Hospitals and Clinics. We applied PCVIPR imaging to assess its potential for obtaining anatomic and physiologic flow data before and after surgical embolization of VoGMs. Hemodynamic pressure parameters obtained from PCVIPR imaging were compared with stump pressures recorded in vivo. We found a decrease in mean arterial pressure from 97 mm Hg pretreatment to 65 mm Hg post treatment. These findings corroborate the decrement in venous pressure gradients documented on PCVIPR imaging. For all patients, pressure gradient changes on PCVIPR imaging correlated with in vivo arterial pressures and aided in clinical decision related to cerebrovascular evaluation, treatment planning, and clinical course. Four-dimensional flow magnetic resonance imaging/PCVIPR imaging has a potential role in determining endovascular embolization and therapeutic outcomes on the basis of objective and reproducible hemodynamic characteristics of the vascular lesion. It represents a novel, noninvasive approach that may guide the extent and timing of therapeutic intervention and treatment of cerebrovascular diseases in pediatric patients. [ABSTRACT FROM AUTHOR]

Recent whole exome sequencing studies in humans have provided novel insight into the importance of the ephrinB2-EphB4-RASA1 signaling axis in cerebrovascular development, corroborating and extending previous work in model systems. Here, we aim to review the human cerebrovascular phenotypes associated with ephrinB2-EphB4-RASA1 mutations, including those recently discovered in Vein of Galen malformation: the most common and severe brain arteriovenous malformation in neonates. We will also discuss emerging paradigms of the molecular and cellular pathophysiology of disease-causing ephrinB2-EphB4-RASA1 mutations, including the potential role of somatic mosaicism. These observations have potential diagnostic and therapeutic implications for patients with rare congenital cerebrovascular diseases and their families. Highlights Genetic and functional studies in model organisms have revealed the importance of ephrinB2-EphB4-RASA1 signaling in multiple aspects of cerebrovascular development. Recent whole exome sequencing studies in humans have identified mutations in EFNB2 , EPHB4 , and RASA1 in multiple congenital cerebrovascular disorders. These diseases include capillary malformation-arteriovenous malformation (CM-AVM), Vein of Galen malformation (VOGM), and others. The localized nature and multifocality of lesions associated with ephrinB2-EphB4-RASA1 mutations implicates somatic mosaicism in disease pathogenesis. The ephrinB2-EphB4-RASA1 axis is a potentially targetable node for therapeutic intervention. [ABSTRACT FROM AUTHOR]

Congenital cerebrovascular anomalies in the pediatric age group are myriad with diverse etiologies. The purpose of this paper is to provide an imaging overview of congenital vascular malformations and vascular tumors, as these conditions are varied and the characteristic vascular abnormality may even suggest the underlying systemic condition in helping to guide further management. For example, the identification of an arterial anomaly such as agenesis/hypoplasia/duplication may warrant further evaluation for an associated syndrome. Recognition of other pertinent findings, such as persistent carotid-vertebrobasilar anastomoses for instance, would be particularly important for the planning of potential embolization/coiling of associated vascular malformation and/or aneurysm. Awareness of congenital dural sinus anomalies, including hypoplasia, aplasia, and or duplication, prevents the incorrect or overdiagnosis of sinus thrombosis. Moreover, multiple developmental venous anomalies in a patient may require further investigation for its familial association. In a similar context, there may be familial association with multiple cavernous malformations and children with central nervous system vascular malformations may have an association with other vascular conditions, eg, hereditary hemorrhagic telangiectasia, etc. An accurate description of vascular lesions is critical for understanding the natural history of the disease process and ultimately for guiding treatment. An important example of this includes Sturge-Weber syndrome, which is often incorrectly viewed as an arteriovenous malformation, but actually a type of venous malformation. Another example which is important to recognize includes the differentiation between an infantile hemangioma and a vascular malformation, particularly as the course of the pathology and the treatment is so different between the two. [ABSTRACT FROM AUTHOR]