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Vein of Galen Malformations HELP
Based on 145 articles published since 2009

These are the 145 published articles about Vein of Galen Malformations that originated from Worldwide during 2009-2019.
+ Citations + Abstracts
Pages: 1 · 2 · 3 · 4 · 5 · 6
1 Editorial Hydrocephalus in vein of Galen malformation. Another paradigm shift in neurosurgery. 2016

d'Avella, Domenico / Causin, Francesco. ·Academic Neurosurgery, Department of Neurosciences, University of Padova Medical School, Via Giustiniani, 235139, Padova, Italy. domenico.davella@unipd.it. · Neuroradiology, Azienda Ospedaliera, Padova, Italy. ·Acta Neurochir (Wien) · Pubmed #27216757.

ABSTRACT: -- No abstract --

2 Editorial Editorial. Vein of Galen malformation. 2011

Lanzino, Giuseppe / Taussky, Philipp. · ·J Neurosurg Pediatr · Pubmed #21194278.

ABSTRACT: -- No abstract --

3 Review Presentation, course, and outcome of postneonatal presentations of vein of Galen malformation: a large, single-institution case series. 2018

Gopalan, Vignesh / Rennie, Adam / Robertson, Fergus / Kanagarajah, Lakshmi / Toolis, Claire / Bhate, Sanjay / Ganesan, Vijeya. ·Developmental Neurosciences, UCL Great Ormond Street Institute of Child Health, London, UK. · Radiology Department, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK. · Neurology Department, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK. ·Dev Med Child Neurol · Pubmed #29359331.

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

4 Review Human genetics and molecular mechanisms of vein of Galen malformation. 2018

Duran, Daniel / Karschnia, Philipp / Gaillard, Jonathan R / Karimy, Jason K / Youngblood, Mark W / DiLuna, Michael L / Matouk, Charles C / Aagaard-Kienitz, Beverly / Smith, Edward R / Orbach, Darren B / Rodesch, Georges / Berenstein, Alejandro / Gunel, Murat / Kahle, Kristopher T. ·1Department of Neurosurgery. · 7Department of Genetics. · 2Department of Neurological Surgery, University of Wisconsin, Madison, Wisconsin; Departments of. · 3Neurosurgery and. · 4Neurointerventional Radiology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts. · 5Service de Neuroradiologie Diagnostique et Thérapeutique, Hôpital Foch, Suresnes, France; and. · 6Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, New York, New York. · 8Centers for Mendelian Genomics and Yale Program on Neurogenetics, and. · 9Department of Pediatrics and Cellular & Molecular Physiology, Yale School of Medicine, New Haven, Connecticut. ·J Neurosurg Pediatr · Pubmed #29350590.

ABSTRACT: Vein of Galen malformations (VOGMs) are rare developmental cerebrovascular lesions characterized by fistulas between the choroidal circulation and the median prosencephalic vein. Although the treatment of VOGMs has greatly benefited from advances in endovascular therapy, including technical innovation in interventional neuroradiology, many patients are recalcitrant to procedural intervention or lack accessibility to specialized care centers, highlighting the need for improved screening, diagnostics, and therapeutics. A fundamental obstacle to identifying novel targets is the limited understanding of VOGM molecular pathophysiology, including its human genetics, and the lack of an adequate VOGM animal model. Herein, the known human mutations associated with VOGMs are reviewed to provide a framework for future gene discovery. Gene mutations have been identified in 2 Mendelian syndromes of which VOGM is an infrequent but associated phenotype: capillary malformation-arteriovenous malformation syndrome ( RASA1) and hereditary hemorrhagic telangiectasia ( ENG and ACVRL1). However, these mutations probably represent only a small fraction of all VOGM cases. Traditional genetic approaches have been limited in their ability to identify additional causative genes for VOGM because kindreds are rare, limited in patient number, and/or seem to have sporadic inheritance patterns, attributable in part to incomplete penetrance and phenotypic variability. The authors hypothesize that the apparent sporadic occurrence of VOGM may frequently be attributable to de novo mutation or incomplete penetrance of rare transmitted variants. Collaboration among treating physicians, patients' families, and investigators using next-generation sequencing could lead to the discovery of novel genes for VOGM. This could improve the understanding of normal vascular biology, elucidate the pathogenesis of VOGM and possibly other more common arteriovenous malformation subtypes, and pave the way for advances in the diagnosis and treatment of patients with VOGM.

5 Review Galenic pial arteriovenous fistulas: Angioarchitecture, clinical presentation, and therapeutic considerations. 2018

George Zaki Ghali, Michael. ·Department of Neurological Surgery, Baylor College of Medicine, Houston, Texas. ·Clin Anat · Pubmed #29082570.

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

6 Review Endovascular Treatment of Vein of Galen Malformations: A Systematic Review and Meta-Analysis. 2017

Brinjikji, W / Krings, T / Murad, M H / Rouchaud, A / Meila, D. ·From the Departments of Radiology (W.B.) brinjikji.waleed@mayo.edu brinjikji.waleed@gmail.com. · Neurosurgery, (W.B.). · Division of Neuroradiology and Neurosurgery (W.B., T.K.), University of Toronto, Toronto Western Hospital and University Health Network, Toronto, Ontario, Canada. · Center for the Science of Healthcare Delivery (M.H.M.), Mayo Clinic, Rochester, Minnesota. · Neuroradiology Service (A.R.), Centre Hospitalier Universitaire Bicêtre, Le Kremlin Bicêtre, France. · Department of Radiology and Neuroradiology (D.M.), Klinikum Duisburg, Duisburg, Germany. · Department of Diagnostic and Interventional Neuroradiology (D.M.), Medical School Hannover, Hannover, Germany. ·AJNR Am J Neuroradiol · Pubmed #28982789.

ABSTRACT: BACKGROUND: Outcomes after endovascular embolization of vein of Galen malformations remain relatively poorly described. PURPOSE: We performed a systematic review of the literature to determine outcomes and predictors of good outcomes following endovascular treatment of vein of Galen malformations. DATA SOURCES: We used Ovid MEDLINE, Ovid Embase, and the Web of Science. STUDY SELECTION: Our study consisted of all case series with ≥4 patients receiving endovascular treatment of vein of Galen malformations published through January 2017. DATA ANALYSIS: We studied the following outcomes: complete/near-complete occlusion rates, technical complications, perioperative stroke, perioperative hemorrhage, technical mortality, all-cause mortality, poor neurologic outcomes, and good neurologic outcomes. Outcomes were stratified by age-group (neonate, infant, child). A random-effects meta-analysis was performed. DATA SYNTHESIS: A total of 27 series with 578 patients were included; 41.9% of patients were neonates, 45.0% of patients were infants, and 13.1% of patients were children. All-cause mortality was 14.0% (95% CI, 8.0%-22.0%). Overall good neurologic outcome rates were 62.0% (95% CI, 57.0%-67.0%). Overall poor neurologic outcome rates were 21.0% (95% CI, 17.0%-26.0%). Neonates were significantly less likely to have good neurologic outcomes than infants (48.0%; 95% CI, 35.0%-62.0% versus 77.0%; 95% CI, 70.0%-84.0%; LIMITATIONS: Limitations were selection and publication biases. CONCLUSIONS: Patients receiving endovascular embolization of vein of Galen malformations experienced good long-term clinical outcomes in >60% of cases. Appropriate patient selection is key as treatment guided by the Bicêtre neonatal evaluation score was associated with improved neurologic outcomes.

7 Review Antenatal diagnosis and prognostic factors of aneurysmal malformation of the vein of Galen: A case report and literature review. 2017

Herghelegiu, Doru / Ionescu, Cringu A / Pacu, Irina / Bohiltea, Roxana / Herghelegiu, Catalin / Vladareanu, Simona. ·aDepartment of Obstetrics Gynecology, "Sanador" Hospital bDepartment of Obstetrics Gynecolgy and Neonatalogy, "Sf Pantelimon" Clinical Emergency Hospital cDepartment of Obstetrics Gynecology and Neonatology University Emergency Hospital, University of Medicine and Pharmacy, "Carol Davila" dDepartment of Obstetrics Gynecology, Polizu Hospital eDepartment of Obstetrics Gynecology and Neonatology, Elias Emergency Clinical Hospital, University of Medicine and Pharmacy, Bucharest, Romania. ·Medicine (Baltimore) · Pubmed #28746188.

ABSTRACT: RATIONALE: Vein of Galen aneurysmal malformation (VGAM) is a rare complex malformation of the cerebral vascular system consisting of arteriovenous shunts between the vein of Galen and the cerebral arteries. PATIENT CONCERNS: We present the case of a 31-year-old pregnant woman, para 1, gravida 1. DIAGNOSES: At 26 weeks' gestation who was examined for an anechoic mass on the cerebral median midline with color and pulsed Doppler. She presented with positive flow on the color and pulsed Doppler test, associated with hydrocephalus, cortical hypoplasia, cardiomegaly, jugular vein distension. INTERVENTIONS: No intervention for VGAM was done. OUTCOMES: This case of a VGAM was associated with negative prognostic factors. LESSONS: The ultrasound color Doppler together with the 3D power Doppler allowed reconstruction of the vascular connections and of the relationship of these with other anatomical structures, which contributed to establishing the prognosis.

8 Review Endovascular treatment of pediatric intracranial arteriovenous shunt. 2017

Niimi, Yasunari. ·Department of Neuroendovascular Therapy, St Luke's International Hospital, Tokyo, Japan. ·Pediatr Int · Pubmed #27601322.

ABSTRACT: Intracranial arteriovenous shunts (ICAVS) in young children are characterized by frequent high-flow fistulas. In association with high-flow fistulas and the physiological condition of the developing brain and heart, each ICAVS type tends to present at a certain age with unique symptoms. Vein of Galen aneurysmal malformation (VGAM) and dural sinus malformation with arteriovenous (AV) shunt tend to present in the neonate with high output cardiac failure. In infancy, VGAM, pial arteriovenous fistula (AVF) and infantile dural AVF (DAVF) tend to present with hydrodynamic disorder such as macrocephaly, ventriculomegaly, prominent facial veins, and developmental delay. Pial AVF, AV malformation, and infantile DAVF can present with focal neurological signs such as seizure or hemorrhage at older ages. Endovascular treatment is currently the first choice of treatment for most pediatric ICAVS. The treatment goal should be defined on a patient-by-patient basis, according to the unique physiological condition of the child.

9 Review The natural progression of VGAMs and the need for urgent medical attention: a systematic review and meta-analysis. 2017

Yan, Jun / Gopaul, Roodrajeetsing / Wen, Jing / Li, Xi-Sheng / Tang, Jing-Feng. ·Department of Cerebrovascular Diseases, The Second Affiliated Hospital of Guilin Medical University, Guilin City, Guangxi Zhuang Autonomous Region, China. · Department of Neurosurgery, The First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi Zhuang Autonomous Region, China. · Department of Rheumatism, The First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi Zhuang Autonomous Region, China. ·J Neurointerv Surg · Pubmed #27091749.

ABSTRACT: BACKGROUND: Vein of Galen aneurysmal malformations (VGAMs) are congenital disorders that may require emergency treatment and some may cause sudden death before medical attention is provided. Some patients also have a spontaneous thrombosis. OBJECTIVE: To understand the natural progression of VGAMs through a systematic literature review. METHODS: We examined PubMed to identify studies published between July 1973 and March 2015. We determined the proportion of patients with VGAM who died before receiving medical attention, who received emergency treatment, or had a spontaneous thrombosis. We pooled estimates of proportions with corresponding 95% CIs calculated using the raw (ie, untransformed) proportions. RESULTS: The 31 studies obtained described the outcome of 754 patients with VGAM. The probability of sudden death risk was 4% (95% CI 1% to 7%; I CONCLUSIONS: Over time, the rate of preoperative sudden death in patients with VGAM gradually declined and the rate of emergency operations gradually increased. The outcome of patients with early spontaneous thromboses was good. Our study provides a definitive description of the natural progression of VGAMs and the need for urgent medical attention.

10 Review Pediatric central nervous system vascular malformations. 2015

Burch, Ezra A / Orbach, Darren B. ·Department of Radiology, Brigham and Women's Hospital, Boston, MA, USA. ·Pediatr Radiol · Pubmed #26346152.

ABSTRACT: Pediatric central nervous system (CNS) vascular anomalies include lesions found only in the pediatric population and also the full gamut of vascular lesions found in adults. Pediatric-specific lesions discussed here include infantile hemangioma, vein of Galen malformation and dural sinus malformation. Some CNS vascular lesions that occur in adults, such as arteriovenous malformation, have somewhat distinct manifestations in children, and those are also discussed. Additionally, children with CNS vascular malformations often have associated broader vascular conditions, e.g., PHACES (posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, eye anomalies and sternal anomalies), hereditary hemorrhagic telangiectasia, and capillary malformation-arteriovenous malformation syndrome (related to the RASA1 mutation). The treatment of pediatric CNS vascular malformations has greatly benefited from advances in endovascular therapy, including technical advances in adult interventional neuroradiology. Dramatic advances in therapy are expected to stem from increased understanding of the genetics and vascular biology that underlie pediatric CNS vascular malformations.

11 Review Outcome and complications of endovascular embolization for vein of Galen malformations: a systematic review and meta-analysis. 2015

Yan, Jun / Wen, Jing / Gopaul, Roodrajeetsing / Zhang, Chao-Yuan / Xiao, Shao-wen. ·Departments of 1 Neurosurgery and. · Rheumatism, The First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi, China. ·J Neurosurg · Pubmed #26230476.

ABSTRACT: OBJECT: There have been many multidisciplinary approaches to the treatment of vein of Galen malformations. Endovascular embolization is the first option for treatment. However, the effects of the treatment remain controversial. The aim of this study is to assess the efficacy and safety of endovascular embolization to treat patients with vein of Galen malformations. METHODS: This paper includes a retrospective analysis of a sample of 667 patients who underwent endovascular embolization to treat vein of Galen malformations. The data were obtained through a literature search of PubMed databases. The authors also evaluate the efficacy and safety of the treatment. Mortality within the follow-up period is analyzed. Pooled estimates of proportions with corresponding 95% CIs were calculated using raw (i.e., untransformed) proportions (PRAW). RESULTS: In the 34 studies evaluated, neonates accounted for 44% of the sample (95% CI 31%-57%; I(2) = 92.5%), infants accounted for 41% (95% CI 30%-51%; I(2) = 83.3%), and children and adults accounted for 12% (95% CI 7%-16%; I(2) = 52.9%). The meta-analysis revealed that complete occlusion was performed in 57% (95% CI 48%-65%; I(2) = 68.2%) of cases, with partial occlusion in 43% (95% CI 34%-51%; I(2) = 70.7%). The pooled proportion of patients showing a good outcome was 68% (95% CI 61%-76%; I(2) = 77.8%), while 31% showed a poor outcome (95% CI 24%-38%; I(2) = 75.6%). The proportional meta-analysis showed that postembolization mortality and complications were reported in 10% (95% CI 8%-12%; I(2) = 42.8%) and 37% (95% CI 29%-45%; I(2) = 79.1%), respectively. Complications included cerebral hemorrhage, cerebral ischemia, hydrocephalus, leg ischemia, and vessel perforation. CONCLUSIONS: The successful treatment of vein of Galen malformations remains a complex therapeutic challenge. The authors' analysis of clinical history and research literature suggests that vein of Galen malformations treated with endovascular embolization can result in an acceptable mortality rate, complications, and good clinical outcome. Future large-scale, multicenter, randomized trials are necessary to confirm these findings.

12 Review Vascular malformations of the brain. 2013

Toulgoat, Frederique / Lasjaunias, Pierre. ·Neuroradiology Department, Centre Hospitalier Universitaire Bicêtre, Paris, France. Electronic address: frederique.toulgoat@me.com. ·Handb Clin Neurol · Pubmed #23622310.

ABSTRACT: Pediatric neurovascular malformations are rare. However, proper diagnosis and management are mandatory to achieve a good neurocognitive outcome. Among them several types can be identified with specificities for each. In the newborn and infancy, the most frequent cerebral venous malformation is vein of Galen aneurysmal malformation. It can be discovered antenatally, in neonates (mainly in cases with hemodynamic impact), or in infants presenting with macrocrania and hydrocephalus. Treatment of choice is endovascular, by transarterial selective occlusion of pathological vessels. Interventions are staged with a first session at around 5 months, adjusted to neurological development. Late consequences, especially if left untreated or treated outside the therapeutic window, are delayed neurocognitive development and seizures. Pial arteriovenous malformation can also be diagnosed antenatally. Regional parenchymal destruction could occur in the first months of life, requiring early endovascular treatment. Dural sinus malformations are the third main type of neurovascular malformation, and are also diagnosed antenatally or in the first months of life. Cardiac tolerance is usually good. Adverse consequences are mainly neurocognitive delay due to chronic venous hyperpressure or acute hemorrhage due to thrombosis of the pathological sinuses. Nidal-type brain arteriovenous malformation and cavernous angioma are usually seen later in children, with hemorrhage often being the first presenting symptom.

13 Review Hidden mortality of prenatally diagnosed vein of Galen aneurysmal malformation: retrospective study and review of the literature. 2012

Deloison, B / Chalouhi, G E / Sonigo, P / Zerah, M / Millischer, A E / Dumez, Y / Brunelle, F / Ville, Y / Salomon, L J. ·Department of Obstetrics and Fetal Medicine and SFAPE (Société Française d'Amélioration des Pratiques Echographique), Paris Descartes University, Assistance Publique-Hôpitaux de Paris, Hôpital Necker Enfants, Paris, France. ·Ultrasound Obstet Gynecol · Pubmed #22605540.

ABSTRACT: OBJECTIVE: To evaluate the prognosis of prenatally diagnosed vein of Galen aneurysmal malformation (VGAM) in a large cohort with this condition and to review the literature on prenatally diagnosed VGAM. METHODS: This was a retrospective study of all cases of prenatally diagnosed VGAM managed in our referral center during a 12-year period. VGAM was categorized as being either isolated or associated with any other abnormality, based on fetal ultrasound and magnetic resonance imaging findings. Poor outcomes comprised termination of pregnancy with confirmation of antenatal findings, perinatal death and severe cardiac and/or neurological impairment in survivors. The literature was also reviewed for similar cases. RESULTS: Twenty-one cases of prenatally diagnosed VGAM were managed in our center. Four (19.0%) cases were isolated and 17 (81.0%) were associated with other anomalies. There were nine terminations (42.9%) and six neonatal deaths (28.6%). Six children (28.6%) were still alive at last follow-up, of whom three had abnormal neurological development. VGAM associated with other anomalies was strongly associated with a poor outcome compared with isolated forms (P < 0.0001). One hundred and nine cases from the literature were also reviewed. CONCLUSION: Fetuses with prenatally diagnosed VGAM have unexpectedly poor outcomes in the presence of cardiac or cerebral anomalies, while those with strictly isolated VGAM tend to have more favorable outcomes. Our literature review corroborates these findings.

14 Review Vein of Galen malformations: epidemiology, clinical presentations, management. 2012

Recinos, Pablo F / Rahmathulla, Gazanfar / Pearl, Monica / Recinos, Violette Renard / Jallo, George I / Gailloud, Philippe / Ahn, Edward S. ·Division of Pediatric Neurosurgery, The Johns Hopkins Hospital, 600 North Wolfe Street, Baltimore, MD 21287, USA. ·Neurosurg Clin N Am · Pubmed #22107867.

ABSTRACT: The vein of Galen aneurysmal malformation is a congenital vascular malformation that comprises 30% of the pediatric vascular and 1% of all pediatric congenital anomalies. Treatment is dependent on the timing of presentation and clinical manifestations. With the development of endovascular techniques, treatment paradigms have changed and clinical outcomes have significantly improved. In this article, the developmental embryology, clinical features and pathophysiology, diagnostic workup, and management strategies are reviewed.

15 Review Vein of Galen aneurysms: presentation and endovascular management. 2010

Hassan, Tamer / Nassar, Mahmoud / Elghandour, Mamdouh. ·Department of Neurosurgery, Alexandria University School of Medicine, Alexandria, Egypt. Tamer.Shihata@alexmed.edu.eg ·Pediatr Neurosurg · Pubmed #21540619.

ABSTRACT: BACKGROUND: We present our experience with managing 13 cases of vein of Galen aneurysm with a special focus on endovascular strategies. This clinical review deals with the multivariable clinical presentation of vein of Galen aneurysms and the role of transarterial endovascular treatment. METHODS: Thirteen patients diagnosed with vein of Galen aneurysms have been reviewed. Clinical presentation, diagnostic modalities and treatment strategies are also documented for each. MRI and three-dimensional CT angiogram (3D CTA) were performed for all patients. Transarterial embolization with Histoacryl was performed in 8 patients proved to have true vein of Galen aneurysmal malformation (VGAM). RESULTS: Clinical presentation was variable including hydrocephalus, headaches, heart failure, coma, epilepsy, and even left orbital swelling. MRI demonstrated large vein of Galen aneurysms in all the cases. 3D CTA demonstrated true VGAM in 10 patients. Two cases proved to have vein of Galen varices. Endovascular treatment was conducted successfully in 8 patients and resulted in arrest of head growth, resolution of headaches and improvement of cardiac condition. CONCLUSION: The transarterial approach proved to be a successful way of management of true VGAM that yielded better outcomes.

16 Review Adult presentation of a familial-associated vein of galen aneurysmal malformation: case report. 2010

Xu, David S / Usman, Asad A / Hurley, Michael C / Eddleman, Christopher S / Bendok, Bernard R. ·Department of Neurological Surgery, Feinberg School of Medicine, Northwestern University, Chicago, Illinois 60611, USA. ·Neurosurgery · Pubmed #21107153.

ABSTRACT: BACKGROUND AND IMPORTANCE: Vein of Galen aneurysmal malformations (VGAMs) arise from persistent arteriovenous shunting from primitive choroidal vessels into the median prosencephalic vein of Markowski, the embryonic precursor of the vein of Galen. VGAMs rarely present past infancy, and their natural history in adults is unknown. We report the first case of a familial-associated VGAM in an asymptomatic adult female patient. The clinical features of this case are presented alongside a systematic review of the literature on adult VGAM cases to assess the natural history, clinical management, and genetic basis of this rare neurovascular lesion. CLINICAL PRESENTATION: A previously healthy 44-year-old woman with a family history of a VGAM in a stillborn presented with an 8-week onset of dizziness and vertigo that spontaneously resolved. Time-resolved magnetic resonance angiography identified a choroidal VGAM. No intervention was undertaken at this time because of the patient's asymptomatic status after 9 months of follow-up. CONCLUSION: Based on our review of the literature, this is the first case report of a familial-associated VGAM in an adult patient and suggests that VGAM development can be genetically linked. Of 15 adult VGAM cases previously reported, all patients were either symptomatic or treated, thus precluding determination of VGAM natural history in adults. Patient outcomes correlated with the severity of presenting symptoms, which ranged from asymptomatic to immediately life-threatening. We hypothesize that self-selection may render VGAMs to be more benign for them to persist past childhood. Further investigation of the molecular biology underlying VGAM development is warranted.

17 Review Evolution of treatment options for vein of Galen malformations. 2010

Khullar, Dhruv / Andeejani, Ahmed M I / Bulsara, Ketan R. ·Department of Neurosurgery, Yale School of Medicine, New Haven, Connecticut 06520, USA. ·J Neurosurg Pediatr · Pubmed #21039167.

ABSTRACT: OBJECT: Vein of Galen aneurysmal malformations (VGAMs) continue to account for high morbidity and mortality rates in the pediatric population. Whereas in the past, mortality rates were nearly 100%, recent developments in endovascular embolization and improvements in neonatal care have improved prognoses. It is now possible that some patients can achieve normal neurological development following embolization of the VGAM. Access to the lesion can be gained via transarterial or transvenous routes. In this paper the authors review the pathophysiological characteristics of VGAM and discuss the evolution of treatment options. METHODS: A PubMed literature search was performed for vein of Galen malformation treatment options, beginning in the 1970s. A total of 22 papers were reviewed in full, and outcome data for 615 patients from 1983 to 2010 were compiled. Articles were reviewed if they focused primarily on the treatment of VGAM and reported outcomes for at least 5 treated patients. RESULTS: Of the 265 patient outcomes reported between 1983 and 2000, 200 received endovascular therapy. Of these patients 72% had a favorable outcome, and a 15% mortality rate was found. Microsurgery was found to have an 84.6% mortality rate. Furthermore, 76.7% of untreated patients died. More recently, endovascular embolization has become the mainstay of VGAM treatment. Of the 350 patients assessed between 2001 and 2010, 337 were treated endovascularly, mostly via the transarterial approach. Of these patients, 84.3% were found to have good or fair outcomes, and a 15.7% mortality rate was found. Neonates had the worst clinical outcomes following endovascular treatment, with a 35.6% mortality rate, whereas infants and children had significantly better outcomes, with mortality rates of 6.5% and 3.2%, respectively. CONCLUSIONS: Endovascular embolization has considerably improved outcomes in patients with VGAM. In the past, the prognosis for patients with VGAM was dismal, and successful procedures were considered to be those that partially or completely obliterated the lesion, but did not necessarily improve the patient's symptoms. More recently, with the continued development and improvement of endovascular techniques, many patients are found to be neurologically normal on follow-up, and mortality rates have dropped substantially when compared with microsurgical treatment.

18 Review Endovascular management of vein of Galen aneurysmal malformations. Influence of the normal venous drainage on the choice of a treatment strategy. 2010

Pearl, Monica / Gomez, Juan / Gregg, Lydia / Gailloud, Philippe. ·Division of Interventional Neuroradiology, The Johns Hopkins Hospital, Baltimore, MD, USA. ·Childs Nerv Syst · Pubmed #20725731.

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

19 Review The high risks of ventriculoperitoneal shunt procedures for hydrocephalus associated with vein of Galen malformations in childhood: case report and literature review. 2010

Jea, Andrew / Bradshaw, Tina J / Whitehead, William E / Curry, Daniel J / Dauser, Robert C / Luerssen, Thomas G. ·Division of Pediatric Neurosurgery, Texas Children's Hospital, Department of Neurosurgery, Baylor College of Medicine, Houston, TX 77030, USA. ajea @ bcm.edu ·Pediatr Neurosurg · Pubmed #20664304.

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

20 Review [Vein of Galen malformations in children]. 2009

Adaev, A R / Iakovlev, S B / Khukhlaeva, E A / Pilipenko, Iu V. · ·Zh Vopr Neirokhir Im N N Burdenko · Pubmed #20143614.

ABSTRACT: The literature review deals with historical stages of understanding of vein of Galen AVM pathogenesis in children up to state-of-art considerations. The authors analyzed existing classifications, clinical presentation, variants of natural course, problems of diagnostics and management of these patients. Causes of complications and possible ways for their prevention are also discussed.

21 Review Vein of Galen malformation. 2009

Hoang, Stanley / Choudhri, Omar / Edwards, Michael / Guzman, Raphael. ·Department of Neurosurgery, Division of Pediatric Neurosurgery, Lucile Packard Children's Hospital, Stanford University School of Medicine, Stanford, California, USA. ·Neurosurg Focus · Pubmed #19877798.

ABSTRACT: A vein of Galen malformation is a rare intracranial vascular lesion affecting the pediatric population. Its poor prognosis has been significantly improved with the development of endovascular embolization. In this paper the authors review the developmental mechanisms, clinical pathophysiology, and the available data on the management and outcome of the disease.

22 Review Pediatric cranial Doppler sonography in children: non-sickle cell applications. 2009

Soetaert, Amy M / Lowe, Lisa H / Formen, Christopher. ·Department of Radiology, St. Luke's Hospital, Kansas City, MO, USA. ·Curr Probl Diagn Radiol · Pubmed #19632499.

ABSTRACT: This article presents an overview of transcranial Doppler, well known for its use as a screening tool in children with sickle cell disease. However, there are many other pediatric applications in which cranial Doppler ultrasound can supply otherwise unavailable information regarding neurovascular flow dynamics. Images illustrate examples of normal anatomy and pathologic conditions that can be evaluated with cranial Doppler ultrasound. Characteristic imaging features of various pediatric applications of cranial Doppler ultrasound are discussed and illustrated. This image presentation discusses cranial Doppler ultrasound technique and normal findings and illustrates various pediatric disorders including benign enlargement of the subarachnoid space versus subdural hematomas, vasospasm, vasculitis, venous sinus thrombosis, vein of Galen varix, hydrocephalus, hypoxic ischemic injury, traumatic brain injury, and brain death.

23 Clinical Trial Superselective transvenous embolization with Onyx and n-BCA for vein of Galen aneurysmal malformations with restricted transarterial access: safety, efficacy, and technical aspects. 2017

Orlov, Kirill / Gorbatykh, Anton / Berestov, Vadim / Shayakhmetov, Timur / Kislitsin, Dmitry / Seleznev, Pavel / Strelnikov, Nikolay. ·Division of Interventional Neuroradiology and Neurosurgery, E.N. Meshalkin Siberian Federal Biomedical Research Center, Rechkunovskaya St. 15, Novosibirsk, Russia, 630055. · Division of Interventional Neuroradiology and Neurosurgery, E.N. Meshalkin Siberian Federal Biomedical Research Center, Rechkunovskaya St. 15, Novosibirsk, Russia, 630055. av_gorbatyh@meshalkin.ru. ·Childs Nerv Syst · Pubmed #28689342.

ABSTRACT: PURPOSE AND METHODS: Superselective transvenous embolization (TVE) with liquid embolic agents is a new concept in treatment of vein of Galen aneurysmal malformations (VGAM). We performed ten sessions of TVE in VGAM patients with restricted transarterial access. In this paper, we assessed clinical and angiographic outcomes of the proposed treatment and discussed three different TVE techniques with regard to morphology of the shunt and outflow tract. Safety and avoidance-of-complication tips were also discussed. RESULTS: Patient age ranged from 4 to 51 months. There were eight patients with choroidal VGAMs, seven of them were successfully treated with Onyx, and in one case, transvenous catheterization failed. In three cases, adjunctive coiling of draining vein was performed. In three cases, normal deep cerebral veins were connected to the outflow part of malformation; they were preserved during embolization in all cases. Six-month follow-up angiography demonstrated angiographic cure in six cases, and partial occlusion in one. There were two patients with mural VGAMs: both were treated with n-BCA. Partial occlusion was achieved in both cases. There was no procedure-related permanent morbidity or mortality. Oculomotor deficit due to quadrigeminal ischemia occurred in one case, and resolved completely after 3 weeks. CONCLUSION: TVE with liquid embolic agents is a safe and effective salvage method for VGAMs with restricted transarterial access, previously considered as poor candidates for endovascular treatment.

24 Clinical Trial Vein of Galen malformations in neonates: new management paradigms for improving outcomes. 2012

Berenstein, Alex / Fifi, Johanna T / Niimi, Yasunari / Presti, Salvatore / Ortiz, Rafael / Ghatan, Saadi / Rosenn, Barak / Sorscher, Michelle / Molofsky, Walter. ·Center for Endovascular Surgery, Hyman Newman Institute for Neurology and Neurosurgery, St. Luke's Roosevelt Hospital Center, New York, New York 10019, USA. ·Neurosurgery · Pubmed #22089754.

ABSTRACT: BACKGROUND: Untreated patients with symptomatic neonatal presentation of vein of Galen aneurismal malformations (VGAMs) carry almost 100% morbidity and mortality. Medical management and endovascular techniques for neonatal treatment have significantly evolved. OBJECTIVE: To evaluate the clinical and angiographic outcomes of modern management of neonates with refractory heart failure from VGAMs. METHODS: From 2005 to 2010, 16 neonatal patients with VGAM presented to our institution. Medical care from the prenatal to perinatal stages was undertaken according to specified institutional guidelines. Nine patients with refractory heart failure required neonatal endovascular intervention. All patients were treated by transarterial deposition of n-butyl cyanoacrylate into fistula sites. Short- and long-term angiographic studies and clinical outcomes were reviewed. RESULTS: Control of heart failure was achieved in 8 patients. One premature baby died shortly after treatment. Long-term angiographic follow-up shows total or near-total angiographic obliteration in all 8 patients. One patient has a mild hemiparesis from treatment. Another has a mild developmental delay. One patient developed a severe seizure disorder and developmental delay. Overall, 66.7% patients have normal neurological development with near-total or total obliteration of the malformation. CONCLUSION: Treatment of refractory heart failure in neonatal VGAM with modern prenatal, neurointensive, neuroanesthetic, and pediatric neuroendovascular care results in significantly improved outcomes with presumed cure and normal neurological development in most.

25 Article Occlusion of a mural type vein of Galen malformation in a 10-month-old boy with three Woven EndoBridge (WEB 17) and two coils. 2019

Runck, Frank / Maurer, Christoph J / Bode, Markus / Lochbihler, Harald / Kuršumović, Adisa / Berlis, Ansgar. ·Department of Diagnostic and Interventional Radiology and Neuroradiology, Universitätsklinikum Augsburg, Augsburg, Germany. · Department of Neurosurgery, Universitätsklinikum Augsburg, Augsburg, Germany. · Department of Pediatric Surgery, Universitätsklinikum Augsburg, Augsburg, Germany. · Department of Neurosurgery, Donauisar Klinikum Deggendorf, Deggendorf, Germany. ·BMJ Case Rep · Pubmed #30988102.

ABSTRACT: We report the case of a 10-month-old boy with an enlarged head circumference and severe motor developmental delay. MRI showed a vein of Galen malformation (VGAM) with a heavily dilated median prosencephalic vein. Digital subtraction angiography confirmed a mural type VGAM with three feeding arteries arising from the posterior cerebral arteries. Due to the short length of the feeding arteries and the high flow, occlusion of the feeding vessels with detachable coils was not possible because of repeated coil dislocation into the dilated vein. Embolization of the three feeding vessels was then performed with a Woven EndoBridge single layer device (WEB SL17). In two arteries complete occlusion was accomplished with the WEB alone and in one artery additional deployment of two coils was necessary. Follow-up imaging at day 1 after treatment as well as 3 and 9 months after embolization showed persistent occlusion.