Vein of Galen Aneurysmal Malformations: Retrospective Evaluation of Endovascular Treatment Outcomes

Vein of Galen Aneurysmal Malformations: Retrospective Evaluation of Endovascular Treatment Outcomes | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Research Article Vein of Galen Aneurysmal Malformations: Retrospective Evaluation of Endovascular Treatment Outcomes Sanubar Nazarli, Cafer Ikbal Gulsever, Duran Sahin, Mehmet Barburoglu, and 2 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-6339339/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 18 Jul, 2025 Read the published version in Child's Nervous System → Version 1 posted 7 You are reading this latest preprint version Abstract Purpose: Vein of Galen Aneurysmal Malformations (VGAM) are rare congenital vascular anomalies that present significant management challenges due to their complex vascular anatomy and hemodynamic impact. This study retrospectively evaluates the clinical outcomes of VGAM patients treated with endovascular embolization, assessing procedural success, mortality, morbidity, and prognostic factors influencing long-term outcomes. Methods: A retrospective analysis was conducted on 26 patients diagnosed with VGAM and treated with endovascular embolization between 2005 and 2024. Patients were classified based on diagnosis timing, age at treatment (neonatal, infancy, childhood, or adulthood), and VGAM subtype (mixed, choroidal, or mural). Clinical, echocardiographic, and imaging data were collected. Primary outcomes included survival, neurological status, and the need for repeat embolization or surgical interventions. Results: Among 26 patients, 6 (23.1%) were female and 20 (76.9%) were male. Prenatal diagnosis was made in 15 cases (57.7%), while 11 (42.3%) were diagnosed postnatally. Endovascular embolization was performed in 24 patients (92.3%), with 11 (42.3%) requiring repeat procedures. Mortality was recorded in 6 patients (23%), all of whom had severe heart failure, and 4 had undergone embolization. Hydrocephalus was present in 14 patients (53.8%), with 6 requiring neurosurgical intervention. Among survivors, 19.2% exhibited developmental delay, while others maintained stable neurological function. Conclusion: Endovascular embolization is an effective treatment for VGAM, though complications such as heart failure and hydrocephalus significantly impact outcomes. Early intervention improves survival, but neonates with high-output cardiac failure remain at high risk. Further research is needed to refine VGAM classification systems and establish standardized treatment protocols. Figures Figure 1 Figure 2 Introduction Vein of Galen aneurysmal malformations (VGAM) are rare congenital vascular anomalies characterized by abnormal arteriovenous connections in the embryonic median prosencephalic vein [ 1 , 2 ]. These high-flow shunts can lead to severe complications, including congestive heart failure in neonates, hydrocephalus, and neurological deficits in infants and older children. The condition accounts for approximately 30% of pediatric vascular malformations and has historically been associated with high morbidity and mortality [ 3 ]. Advances in endovascular embolization techniques have significantly improved patient outcomes compared to traditional surgical approaches, offering a less invasive and more effective treatment option [ 4 ]. Despite the progress in treatment strategies, managing VGAM remains challenging due to its complex vascular anatomy and the variability in clinical presentation [ 5 ]. Neonates typically present with cardiac failure, while older children may develop hydrocephalus, seizures, or intracranial hemorrhage [ 6 ]. The success of endovascular therapy depends on multiple factors, including the timing of intervention, the type of VGAM, and the severity of hemodynamic compromise. Complete occlusion of the malformation must be achieved while preserving normal venous drainage, which requires expertise and careful patient selection [ 7 ]. This study aims to retrospectively evaluate the clinical outcomes of patients with VGAM who underwent endovascular embolization. The objective is to assess the efficacy and safety of endovascular treatment and identify key factors influencing patient prognosis. Methods Study Design This retrospective study was conducted after obtaining approval from the Clinical Research Ethics Committee of Istanbul University, Istanbul Faculty of Medicine (12.03.2024–24771163). Patient records from the Departments of Neurosurgery and Neuroradiology were reviewed to identify cases of VGAM that were treated with endovascular procedures between 2005 and 2024. Patients diagnosed with VGAM were included in the study, while those with incomplete or inaccessible medical records were excluded. Pre-Procedural Assessment Patients were categorized based on the timing of VGAM diagnosis into three groups: prenatal, neonatal (within the first six months), and later childhood or adulthood. Prenatal diagnosis was typically made in the third trimester using color Doppler ultrasound, with fetal MRI performed for differential diagnosis when necessary. Infants diagnosed prenatally were delivered in fully equipped medical centers, and their birth weight, head circumference, and gestational week at diagnosis were recorded. Patients were further classified into four groups based on the timing of endovascular intervention: neonatal (within the first 28 days), infancy (28 days to 2 years), early childhood (2–7 years), and late childhood/adulthood (7 years and older). Those diagnosed prenatally were assessed postnatally in the neonatal intensive care unit (NICU), where parents were informed about the procedure, and consent was obtained. Pre-procedural evaluations included neurological examination, routine blood tests, coagulation parameters, and echocardiography to assess cardiac function. Advanced imaging, including cranial CT, MRI, MR angiography, and digital subtraction angiography (DSA), was performed to assess the vascular anatomy and feeding arteries of VGAM. Endovascular procedures were conducted under general anesthesia by the Neuroradiology Department. Most patients underwent femoral artery cannulation using ultrasound-guided Seldinger technique, while some neonates had umbilical artery access. Embolic agents, including N-butyl cyanoacrylate (NBCA) and ethylene vinyl alcohol (EVA), were used. The procedure was closely monitored to avoid exceeding the maximum allowable contrast dose. Endovascular Treatment The primary approach for endovascular embolization in VGAM cases was transarterial femoral access, occasionally guided by Doppler ultrasound. In a small percentage of cases where arterial embolization was not feasible or required protection of venous reflux, a transvenous approach was utilized. Each patient typically required an average of two treatment sessions to achieve the desired therapeutic outcome. The embolization procedure prioritized occlusion of the most significant feeding artery first. Microcatheters used were a combination of flow-directed and wire-guided devices, selected based on the anatomy of the lesion and the embolic agent used. The embolization materials varied according to the hemodynamic characteristics of the malformation. Fast-setting adhesives were preferred for direct fistulas, while slower polymerizing agents were used for complex arteriovenous connections. Two main embolic agents were used: NBCA and EVA. NBCA facilitated permanent vessel occlusion and was effective in sealing small fistulas. EVA, on the other hand, was preferred for its non-adhesive properties and slower polymerization kinetics, allowing for more controlled application. The choice of embolic agent and delivery technique was tailored to each case, ensuring optimal occlusion while minimizing the risk of complications. VGAM occlusion was generally achieved in one or two sessions. However, the primary objective was not rapid lesion elimination but rather safe embolization to support normal cerebral maturation and neurocognitive development. Following embolization, neonates and infants were kept under sedation in the intensive care unit for 24 hours to prevent agitation and ensure hemodynamic stability. Patients were monitored closely, with blood pressure maintenance and neurological status assessments as part of post-procedural care. Endovascular treatment sessions were repeated every 3–6 months based on the patient’s clinical condition and response to embolization (Figs. 1 and 2 ). Post-Procedural Management Following embolization, all patients were transferred to the intensive care unit (ICU) for at least 48 hours of close monitoring. Standard post-procedural care included neurological assessments, hemodynamic stabilization, and respiratory support when necessary. Patients were monitored for signs of increased intracranial pressure, cardiac complications, and other potential adverse events. Continuous monitoring and multidisciplinary collaboration were emphasized to manage post-procedural complications effectively. Intensive care support, including ventilatory management and fluid resuscitation, was provided as needed. Patients with persistent neurological or systemic complications were closely followed for further interventions. Statistical Analysis Categorical variables were presented as frequencies and percentages. Numerical variables with a normal distribution, such as age, were expressed as mean ± standard deviation (SD). Ordinal data, including the Bicêtre score, were reported as median (25th–75th percentile). Since birth weight did not follow a normal distribution, it was also expressed as median (25th–75th percentile). Results Patient Characteristics and VGAM Classification Among the 26 patients included in the study, 6 (23.1%) were female and 20 (76.9%) were male. Prenatal diagnosis was made in 15 cases (57.7%), while 11 patients (42.3%) were diagnosed postnatally. The earliest prenatal diagnosis was made at 19 weeks of gestation, while the latest was at 38 weeks. Patients were classified into three groups based on VGAM type: 18 patients (69.2%) had the mixed type, three patients (11.5%) had the choroidal type, and five patients (19.2%) had the mural type. Endovascular Treatment and Timing Endovascular treatment was performed in 9 patients (34.6%) during the neonatal period, in 10 patients (38.4%) during infancy, in two patients (7.7%) in early childhood, in two patients (7.7%) in late childhood (9 and 11 years), and in one patient (3.8%) in adulthood (24 years), making them the oldest patient in the study. This patient required a second embolization one week after the initial procedure. Additionally, two patients (7.7%) with VGAM who were deemed unsuitable for intervention due to poor general condition died without undergoing any endovascular treatment. Among the nine neonatal patients who underwent embolization, seven (77.8%) were male and two (22.3%) were female. The mean gestational age at birth was 38 weeks and 4/7 days, with an average birth weight of 3,245 grams. These patients were assessed using the Bicêtre score, which was calculated as a median of 12 (interquartile range: 9–12). Cardiac and Neurological Findings Echocardiographic evaluations revealed normal cardiac function in 11 patients (42.3%), while 13 patients (50%) had heart failure. Other cardiac abnormalities included pulmonary hypertension in 9 cases (34.6%), patent foramen ovale in 3 cases (11.5%), patent ductus arteriosus in 4 cases (15.4%), tricuspid regurgitation in 8 patients (30.8%), cardiomegaly in 9 patients (34.6%), and right ventricular or atrial dilation in 4 patients (15.4%). Additionally, one patient (3.8%) had a right-to-left shunt, two patients (7.7%) had ventricular septal defects (VSD), and one patient (4%) had dextrocardia. Among the treated patients, developmental delay was observed in five (19.2%), epileptic seizures in four (15.3%), and hydrocephalus in 14 (53.8%). Mortality and Treatment Outcomes A total of 6 patients (23%) died during the study period. Of these, 2 patients (7.7%) were diagnosed with VGAM but did not undergo any interventional procedures before their death. The remaining four patients underwent embolization but succumbed despite treatment. Among these, two patients received a single embolization session, whereas the other two underwent three embolization procedures each. All deceased patients had severe heart failure, often accompanied by additional cardiac complications such as pulmonary hypertension and tricuspid valve abnormalities. Postnatally, these patients developed respiratory distress and required mechanical ventilation in the pediatric intensive care unit (PICU). Repeat Procedures and Surgical Interventions Among the 26 patients diagnosed with VGAM, 24 (92.3%) underwent endovascular embolization, while two patients (7.7%) were not eligible for the procedure. Four patients (15.4%) who underwent embolization later died. Repeat embolization was required in 11 patients (42.3%), of whom six (23.0%) underwent two sessions, four (15.4%) required three sessions, and one (3.8%) underwent four procedures. Surgical interventions were necessary in some cases: four patients (15.4%) underwent ventriculoperitoneal (V/P) shunt placement, while one patient (3.8%) underwent third ventriculostomy, and one patient (3.8%) required transient external ventricular drainage (EVD) (Table 1 ). Table 1 This table provides a comprehensive overview of VGAM patient characteristics, treatment, and outcomes. It includes demographics (gender, diagnosis timing, mortality), treatment details (embolization rates, repeat procedures, and mortality), and age at intervention (from neonatal to adulthood). VGAM classification is detailed into mixed, choroidal, and mural types. Procedure-related data highlights embolization success, multiple treatments, and mortality outcomes. Post-procedural findings include neurological complications and surgical interventions such as V/P shunts, ETV, and EVD placement. Additionally, neonatal characteristics (gestational age, birth weight) and echocardiographic findings (heart failure, pulmonary hypertension, and structural abnormalities) are documented. This table summarizes key clinical insights into VGAM management, guiding treatment decisions and prognosis assessment. Category Count and Percentage Patient Demographics and Diagnosis n = 26 Gender (Female/Male) 6 (23.1%) / 20 (76.9%) Prenatal Diagnosis 15 (57.7%) Postnatal Diagnosis 11 (42.3%) Mortality Rate (Total VGAM Patients) 6 (23%) Embolization Performed/Not Performed 24 (92.3%) / 2 (7.7%) Patients Who Died Without Embolization 2 (7.7%) Treatment and Mortality n = 26 Patients Who Underwent Embolization 24 (92.3%) Patients Without Treatment 2 (7.7%) Patients Requiring Repeat Embolization 11 (45.8%) Patients Who Died Despite Embolization 4 (16.7%) Patient Ages at Procedure Time n = 24 Neonatal Period (0–28 days) 9 (34.6%) Infancy Period (0–2 years) 10 (38.4%) Early Childhood (2–7 years) 2 (7.7%) Late Childhood (9–11 years) 2 (7.7%) Adult Age (24 years) 1 (3.8%) VGAM Type n = 26 Mixed Type 18 (69.2%) Choroidal Type 3 (11.5%) Mural Type 5 (19.2%) Procedure Details n = 24 Total Patients Who Underwent Procedure 24 (92.3%) Neonatal Period Procedures 9 (34.6%) Patients Without Procedure 2 (7.7%) Patients Who Died Without Procedure 2 (7.7%) Patients Who Died Despite Procedure 4 (15.4%) Patients Who Underwent Repeat Procedures 11 (42.3%) Patients Who Underwent Two Procedures 6 (23.0%) Patients Who Underwent Three Procedures 4 (15.4%) Patients Who Underwent Four Procedures 1 (3.8%) Post-Procedural Surgical and Neurological Findings n = 26 Developmental Delay 5 (19.2%) Epileptic Seizures 4 (15.3%) Hydrocephalus 14 (53.8%) Ventriculoperitoneal (V/P) Shunt 4 (15.4%) Shunt Revision 2 (7.7%) Endoscopic Third Ventriculostomy (ETV) 1 (3.8%) External Ventricular Drain (EVD) 1 (3.8%) Neonatal Patients' Characteristics n = 9 Neonatal Gender (Female/Male) 2 (22.3%) / 7 (77.8%) Mean Gestational Age (weeks/days) 38 (4/7) Mean Birth Weight (grams) 3245 g Mean Prenatal Diagnosis (weeks) 34 (2/7) Median Bicetre Score 12 (9–12) Echocardiographic Findings n = 26 Normal ECHO 11 (42.3%) Heart Failure 13 (50.0%) Pulmonary Hypertension 9 (34.6%) Patent Foramen Ovale 3 (11.5%) Patent Ductus Arteriosus 4 (15.4%) Tricuspid Regurgitation 8 (30.8%) Cardiomegaly 9 (34.6%) Right Ventricular/Atrial Dilation 4 (15.4%) Right-to-Left Shunt 1 (3.8%) Ventricular Septal Defect 2 (7.7%) Dextrocardia 1 (4.0%) Discussion This study highlights the clinical characteristics, treatment strategies, and outcomes of patients diagnosed with Vein of Galen Aneurysmal Malformation (VGAM), emphasizing the challenges and prognostic factors associated with endovascular management. The findings confirm that VGAM is more frequently observed in male patients, aligning with previous studies [ 8 ]. Although the reason for this gender disparity remains unclear, hormonal or genetic factors may play a role in the pathogenesis of the condition. The clinical presentation of VGAM varies significantly based on the age at diagnosis, with neonates primarily exhibiting cardiac manifestations such as high-output heart failure and pulmonary hypertension, while older infants and children tend to present with neurological symptoms, including hydrocephalus, developmental delay, and seizures [ 9 ]. Our study demonstrated that early diagnosis and intervention are crucial for improving patient outcomes. Of the 26 patients diagnosed with VGAM, 24 underwent endovascular embolization, while two patients, despite prenatal diagnosis, were in critical condition at birth and succumbed to multi-organ failure before any intervention could be performed. Among the 24 treated patients, four (16.7%) died despite undergoing embolization, all of whom were neonates with mixed-type VGAM and severe heart failure. These findings reinforce the well-documented correlation between early-onset high-output cardiac failure and increased mortality risk in neonates [ 10 , 11 ]. Compared to previously published reports, our study's mortality rates are consistent with findings from Fullerton et al., who reported a 15% mortality rate among treated VGAM patients [ 12 , 13 ]. Other studies have described widely varying mortality rates, ranging from 42–91%, reflecting differences in patient selection, anatomical complexity, and treatment protocols [ 14 , 15 ]. The primary goal of VGAM treatment extends beyond survival; it also aims to ensure normal neurodevelopmental outcomes. In our study, developmental delay was observed in 19.2% of patients, with hydrocephalus and epilepsy being the most common neurological complications [ 16 ]. This aligns with previous studies reporting developmental impairment in 16–40% of VGAM survivors [ 17 ]. A significant factor influencing long-term neurological outcomes is the need for cerebrospinal fluid (CSF) diversion procedures, particularly ventriculoperitoneal (V/P) shunt placement. While V/P shunts have been widely used in hydrocephalus management, they carry additional morbidity risks in VGAM patients, including increased susceptibility to encephalomalacia and secondary vascular complications. Our findings suggest that alternative approaches, such as endoscopic third ventriculostomy (ETV), should be considered when possible, as they may offer better long-term outcomes by preserving physiological CSF flow dynamics [ 15 , 17 , 18 ]. Age and VGAM type have been shown to influence treatment outcomes. In our study, all patients who died despite treatment had mixed-type VGAM, which has been previously associated with worse outcomes due to its complex vascular architecture and high-flow characteristics. Additionally, patients diagnosed and treated in the neonatal period had higher mortality rates compared to those diagnosed later in infancy or childhood. These findings support the need for a more functional classification system for VGAM that incorporates both angiographic and clinical parameters [ 19 ]. Current classification systems, such as those proposed by Lasjaunias and Yaşargil, primarily focus on anatomical and angiographic features but do not adequately account for age-related variations in venous maturation and physiological responses to treatment. Given that neonatal venous anatomy is significantly different from that of older children and adults, age should be a key factor in guiding treatment strategies [ 20 , 21 ]. The evolution of endovascular techniques has significantly improved VGAM management, allowing for both transarterial and transvenous embolization approaches tailored to individual patients. The choice of embolic agents, such as NBCA and EVA, plays a crucial role in procedural success, with EVA offering better control and slower polymerization kinetics compared to NBCA. However, despite technological advancements, the optimal timing and extent of embolization remain subjects of debate. While complete occlusion of the malformation is the ultimate goal, a stepwise approach prioritizing hemodynamic stabilization and gradual vascular remodeling may be safer in neonates with severe heart failure [ 22 ]. Another important consideration in VGAM management is the timing of CSF diversion procedures [ 23 , 24 ]. Our study, in agreement with previous reports, suggests that delaying V/P shunt placement until after endovascular treatment may reduce complications. This is because early shunt placement can exacerbate venous hypertension and lead to secondary brain damage. In cases where hydrocephalus persists despite embolization, ETV should be considered as a safer alternative. However, one should bear in mind that there might be anatomical restrictions in performing ETV, especially in large VGAMs [ 25 ]. Despite significant advancements in VGAM treatment, challenges remain in standardizing classification systems and optimizing management protocols [ 26 ]. The rarity of VGAM and the necessity for multidisciplinary expertise limit large-scale studies and prospective trials. Future research should focus on refining classification models that integrate clinical, angiographic, and neurodevelopmental factors to guide individualized treatment strategies. Additionally, advancements in imaging techniques and endovascular technologies will likely continue to improve patient outcomes, reducing both morbidity and mortality associated with this complex vascular anomaly. Limitations This study's retrospective and single-center design limits generalizability and may introduce selection bias. The small sample size restricts statistical power, making it difficult to capture the full variability of VGAM outcomes. Additionally, advancements in endovascular techniques over the 20-year study period may have influenced results. Long-term neurodevelopmental outcomes were not systematically assessed, highlighting the need for future studies with standardized follow-up. Conclusion VGAM is a complex pediatric vascular anomaly primarily affecting critically ill neonates. While endovascular embolization has improved outcomes, complication rates remain significant, emphasizing the importance of optimal patient selection and treatment timing. Severe heart failure unresponsive to medical therapy increases mortality risk, necessitating early intervention, though relying solely on the Bicêtre score for decision-making may be inadequate. A more comprehensive classification system integrating angiographic and clinical data is needed to guide treatment strategies effectively. Given the complexity of VGAM, specialized expertise in neurointervention and pediatric critical care is essential. Further research and standardized data collection in well-equipped centers could enhance patient outcomes, making multidisciplinary management in experienced centers the most appropriate approach. Declarations Disclosure The authors declare no conflicts of interest related to this study. No external funding was received for this research. Author Contribution Authorship Contribution StatementConception or design of the work: S.N., M.B., S.S., A.A.Data collection: S.N., C.İ.G., D.S.Data analysis and interpretation: S.N., M.B., S.S., A.A.Drafting the article: S.N., C.İ.G., D.S.Critical revision of the article: All authors reviewed the manuscript.Supervision: M.B., S.S., A.A. Acknowledgment The authors express their gratitude to the Neurosurgery, Neuroradiology, and Pediatric Intensive Care Units for their support and collaboration in data collection and patient management. Special thanks to the medical and technical staff involved in the treatment and follow-up of the patients. References Clarke NE, Shekhawat J, Popat H, Lord DJE, Abdel-Latif ME. Vein of Galen Aneurysmal Malformation: A Case Report. Healthcare (Basel) 2024;12. https://doi.org/10.3390/HEALTHCARE12070716. Halbach V V., Dowd CF, Higashida RT, Balousek PA, Ciricillo SF, Edwards MSB. Endovascular treatment of mural-type vein of Galen malformations. 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Cite Share Download PDF Status: Published Journal Publication published 18 Jul, 2025 Read the published version in Child's Nervous System → Version 1 posted Editorial decision: Revision requested 23 Jun, 2025 Reviews received at journal 20 Jun, 2025 Reviewers agreed at journal 20 May, 2025 Reviewers invited by journal 19 May, 2025 Editor assigned by journal 01 Apr, 2025 Submission checks completed at journal 01 Apr, 2025 First submitted to journal 30 Mar, 2025 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-6339339","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":459206287,"identity":"d4ca95fd-a9b8-4a0c-a444-43210e1ac9f1","order_by":0,"name":"Sanubar Nazarli","email":"","orcid":"","institution":"Aydin University","correspondingAuthor":false,"prefix":"","firstName":"Sanubar","middleName":"","lastName":"Nazarli","suffix":""},{"id":459206288,"identity":"6d79b5d4-c0b6-4cf0-839e-4c3d5b3970d5","order_by":1,"name":"Cafer Ikbal Gulsever","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAABA0lEQVRIiWNgGAWjYFACxgdgig/CswGJNB7Ar4XZAEyxQXhpIC0NJGk5DCbxauFvP8z4uIKhNrGN//AxiY97ztutbT8MtKXGJhqXFokzycyGZxiOJ7ZJpKVJznh2O3nbmUSglmNpuQ04tBgw5B+TbGA4BtTCY2zMc+B2stkBoBbGhsO4tfA/Zv8J1sJ//rPxnwPnks3OPySgRSKZjbGBoSaxjSGH8THDgQN2ZjcI2CJx4zGzZIPBAWOgXwwf9hxITjC7AbQlAY9f+PuTGT82VNTJ9vMffnDgxwE7e7Pz6Q8ffKixwakF6rzDcGYiWGUCXuVgUAdn2RNWPApGwSgYBSMNAADIM2KwzauLJQAAAABJRU5ErkJggg==","orcid":"","institution":"Istanbul University","correspondingAuthor":true,"prefix":"","firstName":"Cafer","middleName":"Ikbal","lastName":"Gulsever","suffix":""},{"id":459206289,"identity":"3c6618d7-6ace-4853-9fc3-969a6550110f","order_by":2,"name":"Duran Sahin","email":"","orcid":"","institution":"Istanbul University","correspondingAuthor":false,"prefix":"","firstName":"Duran","middleName":"","lastName":"Sahin","suffix":""},{"id":459206290,"identity":"08b463af-cbf3-459d-a10e-9e91384269ce","order_by":3,"name":"Mehmet Barburoglu","email":"","orcid":"","institution":"Istanbul University","correspondingAuthor":false,"prefix":"","firstName":"Mehmet","middleName":"","lastName":"Barburoglu","suffix":""},{"id":459206293,"identity":"99586249-6017-4869-ac4f-85a6d9042636","order_by":4,"name":"Serra Sencer","email":"","orcid":"","institution":"Istanbul University","correspondingAuthor":false,"prefix":"","firstName":"Serra","middleName":"","lastName":"Sencer","suffix":""},{"id":459206295,"identity":"492d8b39-64c7-41e0-a33f-a542df59cc14","order_by":5,"name":"Aydin Aydoseli","email":"","orcid":"","institution":"Istanbul University","correspondingAuthor":false,"prefix":"","firstName":"Aydin","middleName":"","lastName":"Aydoseli","suffix":""}],"badges":[],"createdAt":"2025-03-30 15:38:17","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-6339339/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-6339339/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1007/s00381-025-06901-6","type":"published","date":"2025-07-18T16:05:21+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":83281978,"identity":"540c1d49-24c9-4bb8-8ea5-2a7f72d33af2","added_by":"auto","created_at":"2025-05-22 10:29:49","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":409521,"visible":true,"origin":"","legend":"\u003cp\u003eImaging findings of a 5-year-old male patient diagnosed with an incidental Vein of Galen Aneurysmal Malformation (VGAM) following minor head trauma. (A-C) Cranial CT demonstrating triventricular hydrocephalus and a dilated Galen vein. (D-E) Pre-embolization Digital Subtraction Angiography (DSA) showing a large aneurysmal dilation of the Galen vein, supplied by bilateral posterior choroidal arteries and thalamoperforators, with significant arterial tortuosity. (F-G) Post-embolization DSA, revealing successful occlusion of feeding arteries and reduced arteriovenous shunting. (H-I) Post-embolization CT showing embolization material localized within the feeder arteries, with regression of ventricular dilation. Follow-up CT angiography at 2 years, demonstrating persistent obliteration of feeding arteries and thrombosis of the aneurysmal sac. The patient remained neurologically stable, and no additional neurosurgical intervention for hydrocephalus was required.\u003c/p\u003e","description":"","filename":"1.png","url":"https://assets-eu.researchsquare.com/files/rs-6339339/v1/5d53939a5047f37bececdcd3.png"},{"id":83282436,"identity":"c7703520-982e-46d8-93f4-40cf03400639","added_by":"auto","created_at":"2025-05-22 10:37:49","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":387478,"visible":true,"origin":"","legend":"\u003cp\u003eImaging findings of a newborn male diagnosed antenatally with Vein of Galen Aneurysmal Malformation (VGAM) at 37 weeks of gestation and delivered via cesarean section at 39 weeks. (A-C) Postnatal transfontanel ultrasound showing ventriculomegaly and a large arteriovenous malformation at the Galen vein level with high-velocity flow. (D-E) Cranial CT scans demonstrating severe hydrocephalus and an 84.8 × 55.3 mm aneurysmal malformation. (F-G) Pre-embolization Digital Subtraction Angiography (DSA) revealing anterior and posterior choroidal arteries as the primary feeders. (H-J) Post-embolization DSA showing successful occlusion of feeding arteries after coiling and embolization of the main feeders from the internal carotid and vertebral arteries. Follow-up MR angiography confirming thrombosis of the aneurysmal sac, with ventricular size regression after ventriculoperitoneal (V/P) shunt placement. The patient remained neurologically stable with normal development on follow-up.\u003c/p\u003e","description":"","filename":"2.png","url":"https://assets-eu.researchsquare.com/files/rs-6339339/v1/c3bc643ba7425b95d36f9125.png"},{"id":88506056,"identity":"630e0742-4689-4989-9b1e-b0e56b9fff61","added_by":"auto","created_at":"2025-08-07 07:30:05","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":1647776,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-6339339/v1/fed1035c-ef11-41c1-93ae-efd1e0f85b94.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Vein of Galen Aneurysmal Malformations: Retrospective Evaluation of Endovascular Treatment Outcomes","fulltext":[{"header":"Introduction","content":"\u003cp\u003eVein of Galen aneurysmal malformations (VGAM) are rare congenital vascular anomalies characterized by abnormal arteriovenous connections in the embryonic median prosencephalic vein [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. These high-flow shunts can lead to severe complications, including congestive heart failure in neonates, hydrocephalus, and neurological deficits in infants and older children. The condition accounts for approximately 30% of pediatric vascular malformations and has historically been associated with high morbidity and mortality [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e]. Advances in endovascular embolization techniques have significantly improved patient outcomes compared to traditional surgical approaches, offering a less invasive and more effective treatment option [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eDespite the progress in treatment strategies, managing VGAM remains challenging due to its complex vascular anatomy and the variability in clinical presentation [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. Neonates typically present with cardiac failure, while older children may develop hydrocephalus, seizures, or intracranial hemorrhage [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. The success of endovascular therapy depends on multiple factors, including the timing of intervention, the type of VGAM, and the severity of hemodynamic compromise. Complete occlusion of the malformation must be achieved while preserving normal venous drainage, which requires expertise and careful patient selection [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThis study aims to retrospectively evaluate the clinical outcomes of patients with VGAM who underwent endovascular embolization. The objective is to assess the efficacy and safety of endovascular treatment and identify key factors influencing patient prognosis.\u003c/p\u003e"},{"header":"Methods","content":"\u003cdiv id=\"Sec3\" class=\"Section2\"\u003e \u003ch2\u003eStudy Design\u003c/h2\u003e \u003cp\u003e This retrospective study was conducted after obtaining approval from the Clinical Research Ethics Committee of Istanbul University, Istanbul Faculty of Medicine (12.03.2024\u0026ndash;24771163). Patient records from the Departments of Neurosurgery and Neuroradiology were reviewed to identify cases of VGAM that were treated with endovascular procedures between 2005 and 2024. Patients diagnosed with VGAM were included in the study, while those with incomplete or inaccessible medical records were excluded.\u003c/p\u003e \u003c/div\u003e\n\u003ch3\u003ePre-Procedural Assessment\u003c/h3\u003e\n\u003cp\u003ePatients were categorized based on the timing of VGAM diagnosis into three groups: prenatal, neonatal (within the first six months), and later childhood or adulthood. Prenatal diagnosis was typically made in the third trimester using color Doppler ultrasound, with fetal MRI performed for differential diagnosis when necessary. Infants diagnosed prenatally were delivered in fully equipped medical centers, and their birth weight, head circumference, and gestational week at diagnosis were recorded.\u003c/p\u003e \u003cp\u003ePatients were further classified into four groups based on the timing of endovascular intervention: neonatal (within the first 28 days), infancy (28 days to 2 years), early childhood (2\u0026ndash;7 years), and late childhood/adulthood (7 years and older). Those diagnosed prenatally were assessed postnatally in the neonatal intensive care unit (NICU), where parents were informed about the procedure, and consent was obtained. Pre-procedural evaluations included neurological examination, routine blood tests, coagulation parameters, and echocardiography to assess cardiac function.\u003c/p\u003e \u003cp\u003eAdvanced imaging, including cranial CT, MRI, MR angiography, and digital subtraction angiography (DSA), was performed to assess the vascular anatomy and feeding arteries of VGAM. Endovascular procedures were conducted under general anesthesia by the Neuroradiology Department. Most patients underwent femoral artery cannulation using ultrasound-guided Seldinger technique, while some neonates had umbilical artery access. Embolic agents, including N-butyl cyanoacrylate (NBCA) and ethylene vinyl alcohol (EVA), were used. The procedure was closely monitored to avoid exceeding the maximum allowable contrast dose.\u003c/p\u003e\n\u003ch3\u003eEndovascular Treatment\u003c/h3\u003e\n\u003cp\u003eThe primary approach for endovascular embolization in VGAM cases was transarterial femoral access, occasionally guided by Doppler ultrasound. In a small percentage of cases where arterial embolization was not feasible or required protection of venous reflux, a transvenous approach was utilized. Each patient typically required an average of two treatment sessions to achieve the desired therapeutic outcome.\u003c/p\u003e \u003cp\u003eThe embolization procedure prioritized occlusion of the most significant feeding artery first. Microcatheters used were a combination of flow-directed and wire-guided devices, selected based on the anatomy of the lesion and the embolic agent used. The embolization materials varied according to the hemodynamic characteristics of the malformation. Fast-setting adhesives were preferred for direct fistulas, while slower polymerizing agents were used for complex arteriovenous connections.\u003c/p\u003e \u003cp\u003eTwo main embolic agents were used: NBCA and EVA. NBCA facilitated permanent vessel occlusion and was effective in sealing small fistulas. EVA, on the other hand, was preferred for its non-adhesive properties and slower polymerization kinetics, allowing for more controlled application. The choice of embolic agent and delivery technique was tailored to each case, ensuring optimal occlusion while minimizing the risk of complications.\u003c/p\u003e \u003cp\u003eVGAM occlusion was generally achieved in one or two sessions. However, the primary objective was not rapid lesion elimination but rather safe embolization to support normal cerebral maturation and neurocognitive development. Following embolization, neonates and infants were kept under sedation in the intensive care unit for 24 hours to prevent agitation and ensure hemodynamic stability. Patients were monitored closely, with blood pressure maintenance and neurological status assessments as part of post-procedural care. Endovascular treatment sessions were repeated every 3\u0026ndash;6 months based on the patient\u0026rsquo;s clinical condition and response to embolization (Figs.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e and \u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e).\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e\n\u003ch3\u003ePost-Procedural Management\u003c/h3\u003e\n\u003cp\u003eFollowing embolization, all patients were transferred to the intensive care unit (ICU) for at least 48 hours of close monitoring. Standard post-procedural care included neurological assessments, hemodynamic stabilization, and respiratory support when necessary. Patients were monitored for signs of increased intracranial pressure, cardiac complications, and other potential adverse events.\u003c/p\u003e \u003cp\u003eContinuous monitoring and multidisciplinary collaboration were emphasized to manage post-procedural complications effectively. Intensive care support, including ventilatory management and fluid resuscitation, was provided as needed. Patients with persistent neurological or systemic complications were closely followed for further interventions.\u003c/p\u003e \u003cdiv id=\"Sec7\" class=\"Section2\"\u003e \u003ch2\u003eStatistical Analysis\u003c/h2\u003e \u003cp\u003eCategorical variables were presented as frequencies and percentages. Numerical variables with a normal distribution, such as age, were expressed as mean\u0026thinsp;\u0026plusmn;\u0026thinsp;standard deviation (SD). Ordinal data, including the Bic\u0026ecirc;tre score, were reported as median (25th\u0026ndash;75th percentile). Since birth weight did not follow a normal distribution, it was also expressed as median (25th\u0026ndash;75th percentile).\u003c/p\u003e \u003c/div\u003e"},{"header":"Results","content":"\u003cdiv id=\"Sec9\" class=\"Section2\"\u003e \u003ch2\u003ePatient Characteristics and VGAM Classification\u003c/h2\u003e \u003cp\u003eAmong the 26 patients included in the study, 6 (23.1%) were female and 20 (76.9%) were male. Prenatal diagnosis was made in 15 cases (57.7%), while 11 patients (42.3%) were diagnosed postnatally. The earliest prenatal diagnosis was made at 19 weeks of gestation, while the latest was at 38 weeks. Patients were classified into three groups based on VGAM type: 18 patients (69.2%) had the mixed type, three patients (11.5%) had the choroidal type, and five patients (19.2%) had the mural type.\u003c/p\u003e \u003c/div\u003e\n\u003ch3\u003eEndovascular Treatment and Timing\u003c/h3\u003e\n\u003cp\u003eEndovascular treatment was performed in 9 patients (34.6%) during the neonatal period, in 10 patients (38.4%) during infancy, in two patients (7.7%) in early childhood, in two patients (7.7%) in late childhood (9 and 11 years), and in one patient (3.8%) in adulthood (24 years), making them the oldest patient in the study. This patient required a second embolization one week after the initial procedure. Additionally, two patients (7.7%) with VGAM who were deemed unsuitable for intervention due to poor general condition died without undergoing any endovascular treatment.\u003c/p\u003e \u003cp\u003eAmong the nine neonatal patients who underwent embolization, seven (77.8%) were male and two (22.3%) were female. The mean gestational age at birth was 38 weeks and 4/7 days, with an average birth weight of 3,245 grams. These patients were assessed using the Bic\u0026ecirc;tre score, which was calculated as a median of 12 (interquartile range: 9\u0026ndash;12).\u003c/p\u003e \u003cdiv id=\"Sec11\" class=\"Section2\"\u003e \u003ch2\u003eCardiac and Neurological Findings\u003c/h2\u003e \u003cp\u003eEchocardiographic evaluations revealed normal cardiac function in 11 patients (42.3%), while 13 patients (50%) had heart failure. Other cardiac abnormalities included pulmonary hypertension in 9 cases (34.6%), patent foramen ovale in 3 cases (11.5%), patent ductus arteriosus in 4 cases (15.4%), tricuspid regurgitation in 8 patients (30.8%), cardiomegaly in 9 patients (34.6%), and right ventricular or atrial dilation in 4 patients (15.4%). Additionally, one patient (3.8%) had a right-to-left shunt, two patients (7.7%) had ventricular septal defects (VSD), and one patient (4%) had dextrocardia.\u003c/p\u003e \u003cp\u003eAmong the treated patients, developmental delay was observed in five (19.2%), epileptic seizures in four (15.3%), and hydrocephalus in 14 (53.8%).\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec12\" class=\"Section2\"\u003e \u003ch2\u003eMortality and Treatment Outcomes\u003c/h2\u003e \u003cp\u003eA total of 6 patients (23%) died during the study period. Of these, 2 patients (7.7%) were diagnosed with VGAM but did not undergo any interventional procedures before their death. The remaining four patients underwent embolization but succumbed despite treatment. Among these, two patients received a single embolization session, whereas the other two underwent three embolization procedures each. All deceased patients had severe heart failure, often accompanied by additional cardiac complications such as pulmonary hypertension and tricuspid valve abnormalities. Postnatally, these patients developed respiratory distress and required mechanical ventilation in the pediatric intensive care unit (PICU).\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec13\" class=\"Section2\"\u003e \u003ch2\u003eRepeat Procedures and Surgical Interventions\u003c/h2\u003e \u003cp\u003eAmong the 26 patients diagnosed with VGAM, 24 (92.3%) underwent endovascular embolization, while two patients (7.7%) were not eligible for the procedure. Four patients (15.4%) who underwent embolization later died. Repeat embolization was required in 11 patients (42.3%), of whom six (23.0%) underwent two sessions, four (15.4%) required three sessions, and one (3.8%) underwent four procedures.\u003c/p\u003e \u003cp\u003eSurgical interventions were necessary in some cases: four patients (15.4%) underwent ventriculoperitoneal (V/P) shunt placement, while one patient (3.8%) underwent third ventriculostomy, and one patient (3.8%) required transient external ventricular drainage (EVD) (Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e).\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab1\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eThis table provides a comprehensive overview of VGAM patient characteristics, treatment, and outcomes. It includes demographics (gender, diagnosis timing, mortality), treatment details (embolization rates, repeat procedures, and mortality), and age at intervention (from neonatal to adulthood). VGAM classification is detailed into mixed, choroidal, and mural types. Procedure-related data highlights embolization success, multiple treatments, and mortality outcomes. Post-procedural findings include neurological complications and surgical interventions such as V/P shunts, ETV, and EVD placement. Additionally, neonatal characteristics (gestational age, birth weight) and echocardiographic findings (heart failure, pulmonary hypertension, and structural abnormalities) are documented. This table summarizes key clinical insights into VGAM management, guiding treatment decisions and prognosis assessment.\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"2\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003eCategory\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eCount and Percentage\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePatient Demographics and Diagnosis\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003en\u0026thinsp;=\u0026thinsp;26\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eGender (Female/Male)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e6 (23.1%) / 20 (76.9%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePrenatal Diagnosis\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e15 (57.7%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePostnatal Diagnosis\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e11 (42.3%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMortality Rate (Total VGAM Patients)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e6 (23%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eEmbolization Performed/Not Performed\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e24 (92.3%) / 2 (7.7%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePatients Who Died Without Embolization\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2 (7.7%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eTreatment and Mortality\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cb\u003en\u0026thinsp;=\u0026thinsp;26\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePatients Who Underwent Embolization\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e24 (92.3%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePatients Without Treatment\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2 (7.7%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePatients Requiring Repeat Embolization\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e11 (45.8%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePatients Who Died Despite Embolization\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e4 (16.7%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003ePatient Ages at Procedure Time\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cb\u003en\u0026thinsp;=\u0026thinsp;24\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eNeonatal Period (0\u0026ndash;28 days)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e9 (34.6%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eInfancy Period (0\u0026ndash;2 years)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e10 (38.4%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eEarly Childhood (2\u0026ndash;7 years)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2 (7.7%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eLate Childhood (9\u0026ndash;11 years)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2 (7.7%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAdult Age (24 years)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1 (3.8%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eVGAM Type\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cb\u003en\u0026thinsp;=\u0026thinsp;26\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMixed Type\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e18 (69.2%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eChoroidal Type\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e3 (11.5%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMural Type\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e5 (19.2%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eProcedure Details\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cb\u003en\u0026thinsp;=\u0026thinsp;24\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eTotal Patients Who Underwent Procedure\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e24 (92.3%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eNeonatal Period Procedures\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e9 (34.6%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePatients Without Procedure\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2 (7.7%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePatients Who Died Without Procedure\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2 (7.7%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePatients Who Died Despite Procedure\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e4 (15.4%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePatients Who Underwent Repeat Procedures\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e11 (42.3%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePatients Who Underwent Two Procedures\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e6 (23.0%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePatients Who Underwent Three Procedures\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e4 (15.4%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePatients Who Underwent Four Procedures\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1 (3.8%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003ePost-Procedural Surgical and Neurological Findings\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cb\u003en\u0026thinsp;=\u0026thinsp;26\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eDevelopmental Delay\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e5 (19.2%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eEpileptic Seizures\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e4 (15.3%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eHydrocephalus\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e14 (53.8%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eVentriculoperitoneal (V/P) Shunt\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e4 (15.4%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eShunt Revision\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2 (7.7%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eEndoscopic Third Ventriculostomy (ETV)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1 (3.8%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eExternal Ventricular Drain (EVD)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1 (3.8%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eNeonatal Patients' Characteristics\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cb\u003en\u0026thinsp;=\u0026thinsp;9\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eNeonatal Gender (Female/Male)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2 (22.3%) / 7 (77.8%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMean Gestational Age (weeks/days)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e38 (4/7)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMean Birth Weight (grams)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e3245 g\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMean Prenatal Diagnosis (weeks)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e34 (2/7)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eMedian Bicetre Score\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e12 (9\u0026ndash;12)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e\u003cb\u003eEchocardiographic Findings\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e\u003cb\u003en\u0026thinsp;=\u0026thinsp;26\u003c/b\u003e\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eNormal ECHO\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e11 (42.3%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eHeart Failure\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e13 (50.0%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePulmonary Hypertension\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e9 (34.6%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePatent Foramen Ovale\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e3 (11.5%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePatent Ductus Arteriosus\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e4 (15.4%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eTricuspid Regurgitation\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e8 (30.8%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eCardiomegaly\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e9 (34.6%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eRight Ventricular/Atrial Dilation\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e4 (15.4%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eRight-to-Left Shunt\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1 (3.8%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eVentricular Septal Defect\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2 (7.7%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eDextrocardia\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1 (4.0%)\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003c/div\u003e"},{"header":"Discussion","content":"\u003cp\u003eThis study highlights the clinical characteristics, treatment strategies, and outcomes of patients diagnosed with Vein of Galen Aneurysmal Malformation (VGAM), emphasizing the challenges and prognostic factors associated with endovascular management. The findings confirm that VGAM is more frequently observed in male patients, aligning with previous studies [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]. Although the reason for this gender disparity remains unclear, hormonal or genetic factors may play a role in the pathogenesis of the condition. The clinical presentation of VGAM varies significantly based on the age at diagnosis, with neonates primarily exhibiting cardiac manifestations such as high-output heart failure and pulmonary hypertension, while older infants and children tend to present with neurological symptoms, including hydrocephalus, developmental delay, and seizures [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eOur study demonstrated that early diagnosis and intervention are crucial for improving patient outcomes. Of the 26 patients diagnosed with VGAM, 24 underwent endovascular embolization, while two patients, despite prenatal diagnosis, were in critical condition at birth and succumbed to multi-organ failure before any intervention could be performed. Among the 24 treated patients, four (16.7%) died despite undergoing embolization, all of whom were neonates with mixed-type VGAM and severe heart failure. These findings reinforce the well-documented correlation between early-onset high-output cardiac failure and increased mortality risk in neonates [\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e, \u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e]. Compared to previously published reports, our study's mortality rates are consistent with findings from Fullerton et al., who reported a 15% mortality rate among treated VGAM patients [\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e, \u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e]. Other studies have described widely varying mortality rates, ranging from 42\u0026ndash;91%, reflecting differences in patient selection, anatomical complexity, and treatment protocols [\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e, \u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThe primary goal of VGAM treatment extends beyond survival; it also aims to ensure normal neurodevelopmental outcomes. In our study, developmental delay was observed in 19.2% of patients, with hydrocephalus and epilepsy being the most common neurological complications [\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e]. This aligns with previous studies reporting developmental impairment in 16\u0026ndash;40% of VGAM survivors [\u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e]. A significant factor influencing long-term neurological outcomes is the need for cerebrospinal fluid (CSF) diversion procedures, particularly ventriculoperitoneal (V/P) shunt placement. While V/P shunts have been widely used in hydrocephalus management, they carry additional morbidity risks in VGAM patients, including increased susceptibility to encephalomalacia and secondary vascular complications. Our findings suggest that alternative approaches, such as endoscopic third ventriculostomy (ETV), should be considered when possible, as they may offer better long-term outcomes by preserving physiological CSF flow dynamics [\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e, \u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e, \u003cspan citationid=\"CR18\" class=\"CitationRef\"\u003e18\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eAge and VGAM type have been shown to influence treatment outcomes. In our study, all patients who died despite treatment had mixed-type VGAM, which has been previously associated with worse outcomes due to its complex vascular architecture and high-flow characteristics. Additionally, patients diagnosed and treated in the neonatal period had higher mortality rates compared to those diagnosed later in infancy or childhood. These findings support the need for a more functional classification system for VGAM that incorporates both angiographic and clinical parameters [\u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e]. Current classification systems, such as those proposed by Lasjaunias and Yaşargil, primarily focus on anatomical and angiographic features but do not adequately account for age-related variations in venous maturation and physiological responses to treatment. Given that neonatal venous anatomy is significantly different from that of older children and adults, age should be a key factor in guiding treatment strategies [\u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e, \u003cspan citationid=\"CR21\" class=\"CitationRef\"\u003e21\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThe evolution of endovascular techniques has significantly improved VGAM management, allowing for both transarterial and transvenous embolization approaches tailored to individual patients. The choice of embolic agents, such as NBCA and EVA, plays a crucial role in procedural success, with EVA offering better control and slower polymerization kinetics compared to NBCA. However, despite technological advancements, the optimal timing and extent of embolization remain subjects of debate. While complete occlusion of the malformation is the ultimate goal, a stepwise approach prioritizing hemodynamic stabilization and gradual vascular remodeling may be safer in neonates with severe heart failure [\u003cspan citationid=\"CR22\" class=\"CitationRef\"\u003e22\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eAnother important consideration in VGAM management is the timing of CSF diversion procedures [\u003cspan citationid=\"CR23\" class=\"CitationRef\"\u003e23\u003c/span\u003e, \u003cspan citationid=\"CR24\" class=\"CitationRef\"\u003e24\u003c/span\u003e]. Our study, in agreement with previous reports, suggests that delaying V/P shunt placement until after endovascular treatment may reduce complications. This is because early shunt placement can exacerbate venous hypertension and lead to secondary brain damage. In cases where hydrocephalus persists despite embolization, ETV should be considered as a safer alternative. However, one should bear in mind that there might be anatomical restrictions in performing ETV, especially in large VGAMs [\u003cspan citationid=\"CR25\" class=\"CitationRef\"\u003e25\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eDespite significant advancements in VGAM treatment, challenges remain in standardizing classification systems and optimizing management protocols [\u003cspan citationid=\"CR26\" class=\"CitationRef\"\u003e26\u003c/span\u003e]. The rarity of VGAM and the necessity for multidisciplinary expertise limit large-scale studies and prospective trials. Future research should focus on refining classification models that integrate clinical, angiographic, and neurodevelopmental factors to guide individualized treatment strategies. Additionally, advancements in imaging techniques and endovascular technologies will likely continue to improve patient outcomes, reducing both morbidity and mortality associated with this complex vascular anomaly.\u003c/p\u003e \u003cdiv id=\"Sec15\" class=\"Section2\"\u003e \u003ch2\u003eLimitations\u003c/h2\u003e \u003cp\u003eThis study's retrospective and single-center design limits generalizability and may introduce selection bias. The small sample size restricts statistical power, making it difficult to capture the full variability of VGAM outcomes. Additionally, advancements in endovascular techniques over the 20-year study period may have influenced results. Long-term neurodevelopmental outcomes were not systematically assessed, highlighting the need for future studies with standardized follow-up.\u003c/p\u003e \u003c/div\u003e"},{"header":"Conclusion","content":"\u003cp\u003eVGAM is a complex pediatric vascular anomaly primarily affecting critically ill neonates. While endovascular embolization has improved outcomes, complication rates remain significant, emphasizing the importance of optimal patient selection and treatment timing. Severe heart failure unresponsive to medical therapy increases mortality risk, necessitating early intervention, though relying solely on the Bic\u0026ecirc;tre score for decision-making may be inadequate. A more comprehensive classification system integrating angiographic and clinical data is needed to guide treatment strategies effectively. Given the complexity of VGAM, specialized expertise in neurointervention and pediatric critical care is essential. Further research and standardized data collection in well-equipped centers could enhance patient outcomes, making multidisciplinary management in experienced centers the most appropriate approach.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e \u003ch2\u003eDisclosure\u003c/h2\u003e \u003cp\u003eThe authors declare no conflicts of interest related to this study. No external funding was received for this research.\u003c/p\u003e \u003c/p\u003e\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\u003cp\u003eAuthorship Contribution StatementConception or design of the work: S.N., M.B., S.S., A.A.Data collection: S.N., C.İ.G., D.S.Data analysis and interpretation: S.N., M.B., S.S., A.A.Drafting the article: S.N., C.İ.G., D.S.Critical revision of the article: All authors reviewed the manuscript.Supervision: M.B., S.S., A.A.\u003c/p\u003e\u003ch2\u003eAcknowledgment\u003c/h2\u003e \u003cp\u003eThe authors express their gratitude to the Neurosurgery, Neuroradiology, and Pediatric Intensive Care Units for their support and collaboration in data collection and patient management. Special thanks to the medical and technical staff involved in the treatment and follow-up of the patients.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n \u003cli\u003eClarke NE, Shekhawat J, Popat H, Lord DJE, Abdel-Latif ME. Vein of Galen Aneurysmal Malformation: A Case Report. Healthcare (Basel) 2024;12. https://doi.org/10.3390/HEALTHCARE12070716.\u003c/li\u003e\n \u003cli\u003eHalbach V V., Dowd CF, Higashida RT, Balousek PA, Ciricillo SF, Edwards MSB. Endovascular treatment of mural-type vein of Galen malformations. J Neurosurg 1998;89:74\u0026ndash;80. https://doi.org/10.3171/jns.1998.89.1.0074.\u003c/li\u003e\n \u003cli\u003eP L. Vein of Galen malformations. Neurosurgery 1989;25:666. https://doi.org/10.1097/00006123-198910000-00030.\u003c/li\u003e\n \u003cli\u003eBerenstein A, Ortiz R, Niimi Y, Elijovich L, Fifi J, Madrid M, et al. Endovascular management of arteriovenous malformations and other intracranial arteriovenous shunts in neonates, infants, and children. Childs Nerv Syst 2010;26:1345\u0026ndash;58. https://doi.org/10.1007/S00381-010-1206-Y.\u003c/li\u003e\n \u003cli\u003eHoffman HJ, Chuang S, Hendrick EB, Humphreys RP. Aneurysms of the vein of Galen. Experience at The Hospital for Sick Children, Toronto. J Neurosurg 1982;57:316\u0026ndash;22. https://doi.org/10.3171/JNS.1982.57.3.0316.\u003c/li\u003e\n \u003cli\u003eCiricillo SF, Edwards MS, Schmidt KG, Hieshima GB, Silverman NH, Higashida RT, et al. Interventional neuroradiological management of vein of Galen malformations in the neonate. Neurosurgery 1990;27:22. https://doi.org/10.1097/00006123-199007000-00003.\u003c/li\u003e\n \u003cli\u003eReichman A, Vi\u0026ntilde;uela F, Duckwiler GR, Peacock WJ, Vinters H V. Pathologic findings in a patient with a vein of Galen aneurysm treated by staged endovascular embolization. Childs Nerv Syst 1993;9:33\u0026ndash;8. https://doi.org/10.1007/BF00301934.\u003c/li\u003e\n \u003cli\u003eDuran D, Karschnia P, Gaillard JR, Karimy JK, Youngblood MW, DiLuna ML, et al. Human genetics and molecular mechanisms of vein of Galen malformation. J Neurosurg Pediatr 2018;21:367\u0026ndash;74. https://doi.org/10.3171/2017.9.PEDS17365.\u003c/li\u003e\n \u003cli\u003ePearl M, Gomez J, Gregg L, Gailloud P. Endovascular management of vein of Galen aneurysmal malformations. Influence of the normal venous drainage on the choice of a treatment strategy. Childs Nerv Syst 2010;26:1367\u0026ndash;79. https://doi.org/10.1007/S00381-010-1257-0.\u003c/li\u003e\n \u003cli\u003eLevrier O, Gailloud PH, Souei M, Manera L, Brunel H, Raybaud C. Normal galenic drainage of the deep cerebral venous system in two cases of vein of Galen aneurysmal malformation. Childs Nerv Syst 2004;20:91\u0026ndash;7. https://doi.org/10.1007/S00381-003-0841-Y.\u003c/li\u003e\n \u003cli\u003eDe Beritto T, Khan OA, Hageman JR, Schreiber M. Vein of Galen Arteriovenous Malformation in a Neonate. Pediatr Ann 2015;44:e243\u0026ndash;6. https://doi.org/10.3928/00904481-20151012-08.\u003c/li\u003e\n \u003cli\u003eWong FY, Mitchell PJ, Tress BM, Dargaville PA, Loughnan PM. Hemodynamic disturbances associated with endovascular embolization in newborn infants with vein of Galen malformation. J Perinatol 2006;26:273\u0026ndash;8. https://doi.org/10.1038/SJ.JP.7211479.\u003c/li\u003e\n \u003cli\u003eHeuer GG, Gabel B, Beslow LA, Stiefel MF, Schwartz ES, Storm PB, et al. Diagnosis and treatment of vein of Galen aneurysmal malformations. Childs Nerv Syst 2010;26:879\u0026ndash;87. https://doi.org/10.1007/S00381-009-1063-8.\u003c/li\u003e\n \u003cli\u003eRodesch G, Hui F, Alvarez H, Tanaka A, Lasjaunias P. Prognosis of antenatally diagnosed vein of Galen aneurysmal malformations. Childs Nerv Syst 1994;10:79\u0026ndash;83. https://doi.org/10.1007/BF00302765.\u003c/li\u003e\n \u003cli\u003eYan J, Wen J, Gopaul R, Zhang CY, Xiao SW. Outcome and complications of endovascular embolization for vein of Galen malformations: a systematic review and meta-analysis. J Neurosurg 2015;123:872\u0026ndash;90. https://doi.org/10.3171/2014.12.JNS141249.\u003c/li\u003e\n \u003cli\u003eHoang S, Choudhri O, Edwards M, Guzman R. Vein of Galen malformation. Neurosurg Focus 2009;27. https://doi.org/10.3171/2009.8.FOCUS09168.\u003c/li\u003e\n \u003cli\u003eAK G, VR R, DR V, TR K, C K, T K, et al. Evaluation, management, and long-term follow up of vein of Galen malformations. J Neurosurg 2006;105:26\u0026ndash;32. https://doi.org/10.3171/JNS.2006.105.1.26.\u003c/li\u003e\n \u003cli\u003eLylyk P, Vinuela F, Dion JE, Duckwiler G, Guglielmi G, Peacock W, et al. Therapeutic alternatives for vein of Galen vascular malformations. J Neurosurg 1993;78:438\u0026ndash;45. https://doi.org/10.3171/JNS.1993.78.3.0438.\u003c/li\u003e\n \u003cli\u003eFullerton HJ, Aminoff AR, Ferriero DM, Gupta N, Dowd CF. Neurodevelopmental outcome after endovascular treatment of vein of Galen malformations. Neurology 2003;61:1386\u0026ndash;90. https://doi.org/10.1212/01.WNL.0000094322.12621.02.\u003c/li\u003e\n \u003cli\u003eLi AH, Armstrong D, TerBrugge KG. Endovascular treatment of vein of Galen aneurysmal malformation: management strategy and 21-year experience in Toronto. J Neurosurg Pediatr 2011;7:3\u0026ndash;10. https://doi.org/10.3171/2010.9.PEDS0956.\u003c/li\u003e\n \u003cli\u003eLasjaunias P, Wuppalapati S, Alvarez H, Rodesch G, Ozanne A. Intracranial aneurysms in children aged under 15 years: review of 59 consecutive children with 75 aneurysms. Childs Nerv Syst 2005;21:437\u0026ndash;50. https://doi.org/10.1007/S00381-004-1125-X.\u003c/li\u003e\n \u003cli\u003eBrinjikji W, Krings T, Murad MH, Rouchaud A, Meila D. Endovascular Treatment of Vein of Galen Malformations: A Systematic Review and Meta-Analysis. AJNR Am J Neuroradiol 2017;38:2308\u0026ndash;14. https://doi.org/10.3174/AJNR.A5403.\u003c/li\u003e\n \u003cli\u003eStone McGuire L, Nikas D. Vein of Galen Malformation. N Engl J Med 2020;383. https://doi.org/10.1056/NEJMICM1913659.\u003c/li\u003e\n \u003cli\u003eLu VM, Luther EM, Silva MA, Rangwala SD, Starke RM, Smith ER, et al. The composition of landmark vein of Galen malformation research: the emergence of endovascular treatments. Childs Nerv Syst 2023;39. https://doi.org/10.1007/S00381-022-05687-1.\u003c/li\u003e\n \u003cli\u003eCory MJ, Durand P, Sillero R, Morin L, Savani R, Chalak L, et al. Vein of Galen aneurysmal malformation: rationalizing medical management of neonatal heart failure. Pediatr Res 2023;93:39\u0026ndash;48. https://doi.org/10.1038/S41390-022-02064-1.\u003c/li\u003e\n \u003cli\u003eShigematsu T, Bazil MJ, Fifi JT, Berenstein A. Fine, Vascular Network Formation in Patients with Vein of Galen Aneurysmal Malformation. AJNR Am J Neuroradiol 2022;43:1481\u0026ndash;7. https://doi.org/10.3174/AJNR.A7649.\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"childs-nervous-system","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"cnsy","sideBox":"Learn more about [Child's Nervous System](http://link.springer.com/journal/381)","snPcode":"381","submissionUrl":"https://submission.nature.com/new-submission/381/3","title":"Child's Nervous System","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false},"keywords":"","lastPublishedDoi":"10.21203/rs.3.rs-6339339/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-6339339/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003ePurpose: \u003c/strong\u003eVein of Galen Aneurysmal Malformations (VGAM) are rare congenital vascular anomalies that present significant management challenges due to their complex vascular anatomy and hemodynamic impact. This study retrospectively evaluates the clinical outcomes of VGAM patients treated with endovascular embolization, assessing procedural success, mortality, morbidity, and prognostic factors influencing long-term outcomes.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eMethods:\u003c/strong\u003e A retrospective analysis was conducted on 26 patients diagnosed with VGAM and treated with endovascular embolization between 2005 and 2024. Patients were classified based on diagnosis timing, age at treatment (neonatal, infancy, childhood, or adulthood), and VGAM subtype (mixed, choroidal, or mural). Clinical, echocardiographic, and imaging data were collected. Primary outcomes included survival, neurological status, and the need for repeat embolization or surgical interventions.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eResults: \u003c/strong\u003eAmong 26 patients, 6 (23.1%) were female and 20 (76.9%) were male. Prenatal diagnosis was made in 15 cases (57.7%), while 11 (42.3%) were diagnosed postnatally. Endovascular embolization was performed in 24 patients (92.3%), with 11 (42.3%) requiring repeat procedures. Mortality was recorded in 6 patients (23%), all of whom had severe heart failure, and 4 had undergone embolization. Hydrocephalus was present in 14 patients (53.8%), with 6 requiring neurosurgical intervention. Among survivors, 19.2% exhibited developmental delay, while others maintained stable neurological function.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConclusion:\u003c/strong\u003e Endovascular embolization is an effective treatment for VGAM, though complications such as heart failure and hydrocephalus significantly impact outcomes. Early intervention improves survival, but neonates with high-output cardiac failure remain at high risk. Further research is needed to refine VGAM classification systems and establish standardized treatment protocols.\u003c/p\u003e","manuscriptTitle":"Vein of Galen Aneurysmal Malformations: Retrospective Evaluation of Endovascular Treatment Outcomes","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-05-22 10:29:44","doi":"10.21203/rs.3.rs-6339339/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2025-06-23T15:43:13+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2025-06-20T13:10:18+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"164927418294825144753428174012602766573","date":"2025-05-20T08:58:37+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2025-05-19T13:50:21+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2025-04-01T12:14:44+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2025-04-01T12:13:11+00:00","index":"","fulltext":""},{"type":"submitted","content":"Child's Nervous System","date":"2025-03-30T15:29:11+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"childs-nervous-system","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"cnsy","sideBox":"Learn more about [Child's Nervous System](http://link.springer.com/journal/381)","snPcode":"381","submissionUrl":"https://submission.nature.com/new-submission/381/3","title":"Child's Nervous System","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false}}],"origin":"","ownerIdentity":"ef00d05f-7f2c-4792-a347-c9843e5b7658","owner":[],"postedDate":"May 22nd, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2025-08-07T07:11:42+00:00","versionOfRecord":{"articleIdentity":"rs-6339339","link":"https://doi.org/10.1007/s00381-025-06901-6","journal":{"identity":"childs-nervous-system","isVorOnly":false,"title":"Child's Nervous System"},"publishedOn":"2025-07-18 16:05:21","publishedOnDateReadable":"July 18th, 2025"},"versionCreatedAt":"2025-05-22 10:29:44","video":"","vorDoi":"10.1007/s00381-025-06901-6","vorDoiUrl":"https://doi.org/10.1007/s00381-025-06901-6","workflowStages":[]},"version":"v1","identity":"rs-6339339","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-6339339","identity":"rs-6339339","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}