849 results match your criteria Vein of Galen Malformation


Arteriovenous Cerebral High Flow Shunts in Children: From Genotype to Phenotype.

Front Pediatr 2022 25;10:871565. Epub 2022 Apr 25.

Department of Diagnostic Radiology and Interventional Radiology, Lausanne University Hospital (CHUV), Lausanne, Switzerland.

Objective: To study the genotypes and phenotypes of cerebral arteriovenous fistulas that drain or do not drain through the vein of Galen, and true vein of Galen aneurysmal malformations, in order to determine whether genotyping could help improve classification of these malformations and their management.

Methods: We carried out a retrospective review of genetic and phenotypic data in databases of four centers. All children with cerebral arteriovenous fistula or vein of Galen aneurysmal malformations aged below 18 years at onset were included. Read More

View Article and Full-Text PDF

Outcome of Neonates Presenting With Severe Cardiac Failure due to Cerebral Arteriovenous Fistula.

Pediatr Neurol 2022 Apr 14;131:25-29. Epub 2022 Apr 14.

Department of Pediatrics and Benioff Children's Hospital, University of California San Francisco, San Francisco, California; Department of Neurology and Weill Institute for Neurosciences, University of California San Francisco, San Francisco, California. Electronic address:

Background: Congenital cerebral arteriovenous fistulas (AVFs), including vein of Galen malformations, presenting in infancy carry variable mortality and morbidity. This study aimed to describe the outcome of neonates with cerebral AVFs who present with refractory cardiac failure.

Methods: Retrospective chart review of neonates with refractory cardiac failure due to cerebral AVFs presenting before 28 days of age in a single-center neuro-intensive care nursery over a 12-year period (2008-2020) was conducted. Read More

View Article and Full-Text PDF

Vein of Galen aneurysmal malformation: rationalizing medical management of neonatal heart failure.

Pediatr Res 2022 Apr 14. Epub 2022 Apr 14.

Division of Neonatal-Perinatal Medicine, Department of Pediatrics, The University of Texas Southwestern Medical Center, Dallas, TX, USA.

Neonates who present in high output heart failure secondary to vein of Galen aneurysmal malformation can be difficult to manage medically due to the complex physiology that results from the large shunt through the malformation. Though the cardiac function is often normal, right ventricular dilation, severe pulmonary hypertension, and systemic steal can result in inadequate organ perfusion and shock. This report recommends medical management for stabilization of neonates prior to definitive management with endovascular embolization. Read More

View Article and Full-Text PDF

Vascular Lesions of the Pineal Region: A Comprehensive Review of the Therapeutic Options.

World Neurosurg 2022 Mar;159:298-313

Juha Hernesniemi International Center for Neurosurgery, Henan Provincial People's Hospital, Zhengzhou, China.

Objective: Vascular lesions of the pineal region comprise aneurysms of the pineal region, arteriovenous malformations, cavernous malformations, and vein of Galen malformations. In the present report, we have offered an extensive review of each vascular pineal region lesion.

Methods: We performed an extensive literature review, focusing on the current therapeutic options available for the different vascular lesions of the pineal region. Read More

View Article and Full-Text PDF

Rapid Ventricular Pacing Facilitates Transarterial Embolization in Vein of Galen Malformations.

Interv Neuroradiol 2022 Mar 2:15910199221082472. Epub 2022 Mar 2.

9968University of Alabama, Birmingham.

Mural type vein of Galen malformation (mVOGM) is a congenital high flow arteriovenous shunt between choroidal arteries and the prosencephalic vein of Markowski leading to heart failure and hydrovenous disorder in children. Embolizing fistulous connections can be challenging and typically requires adjunctive techniques such as induced hypotension, balloon-assisted flow control, and creation of a coil basket. These maneuvers add time, complexity, and unpredictability. Read More

View Article and Full-Text PDF

Cerebral neurovascular embryology, anatomic variations, and congenital brain arteriovenous lesions.

J Neurointerv Surg 2022 Feb 15. Epub 2022 Feb 15.

Division of Neuroradiology, Department of Medical Imaging and Division of Neurosurgery, Department of Surgery, Toronto Western Hospital, University Health Network, Toronto, Ontario, Canada.

Cerebral neurovascular development is a complex and coordinated process driven by the changing spatial and temporal metabolic demands of the developing brain. Familiarity with the process is helpful in understanding neurovascular anatomic variants and congenital arteriovenous shunting lesions encountered in endovascular neuroradiological practice. Herein, the processes of vasculogenesis and angiogenesis are reviewed, followed by examination of the morphogenesis of the cerebral arterial and venous systems. Read More

View Article and Full-Text PDF
February 2022

Pearls & Oy-sters: Arteriovenous Malformation With Sinus Thrombosis and Thalamic Hemorrhage: Unusual Cause of Parkinsonism and Dementia.

Neurology 2022 03 4;98(13):550-553. Epub 2022 Feb 4.

From the Departments of Neurology (P.R.A., S.K., S.G.L., M.E.I., J.C.G.-M.), Neurosurgery (E.V.-L.), and Radiology (L.A.M., J.J.G.M., E.S.M.D.), Osakidetza Basque Health Service, Basurto University Hospital, Bilbao, Spain; and University of the Basque Country (J.C.G.-M.).

Deep cerebral venous thrombosis is an uncommon condition, which usually produces headache, altered consciousness, and ocular movement abnormality. Parkinsonism occasionally occurs when there is basal ganglia involvement. We report a case of a 78-year-old man who presented with a rapidly progressive parkinsonism with poor response to dopaminergic therapy. Read More

View Article and Full-Text PDF

Hydrocephalus in Vein of Galen Malformations.

Neurol India 2021 Nov-Dec;69(Supplement):S376-S379

Institute of Neurosciences, Apollo Hospitals, Chennai, Tamil Nadu, India.

Vein of Galen malformation (VOGM) is a fistulous arteriovenous malformation presenting in the early childhood. Hydrocephalus with VOGM develops in one half of patients during the course of the disease. The concept of hydrodynamic disorders is the key to understand the development of hydrocephalus. Read More

View Article and Full-Text PDF
February 2022

Endoscopic third ventriculostomy in an infant with vein of Galen aneurysmal malformation treated by endovascular occlusion: Case report and a review of literature.

Neurochirurgie 2022 Jan 14. Epub 2022 Jan 14.

Department of Neurosurgery, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital, Prague, Czech Republic.

Introduction: Vein of Galen aneurysmal malformations (VGAMs) can, through multiple mechanisms, complicate with hydrocephalus (HCP). It is generally agreed that management strategies in this scenario should focus on endovascular embolizations. Treatment options for non-responders, however, have been only scarcely reported upon. Read More

View Article and Full-Text PDF
January 2022

Pulmonary hypertension associated with vein of Galen malformation. Fetal cardiac hemodynamic findings and physiological considerations.

J Perinatol 2022 Jan 12;42(1):143-148. Epub 2022 Jan 12.

Division of Fetal and Pediatric Cardiology, University of Montreal, Sainte-Justine University Hospital Center, Montreal, QC, Canada.

The management of newborns with vein of Galen aneurysmal malformation (VGAM) is clinically challenging for neonatologists and cardiologists. Hemodynamic profiles in four fetuses diagnosed with VGAM who subsequently developed neonatal cardiac failure and pulmonary hypertension were studied using two-dimensional and Doppler echocardiography. All four had an increased cardiothoracic ratio due to right ventricular dilatation on antenatal ultrasound. Read More

View Article and Full-Text PDF
January 2022

The outcome of the vein of Galen aneurysmal malformation cases diagnosed prenatally.

J Obstet Gynaecol 2022 Jan 12:1-5. Epub 2022 Jan 12.

Department of Obstetrics and Gynecology, Maternal-Fetal Medicine Unit, Sisli Memorial Hospital, Istanbul, Turkey.

Vein of Galen aneurysmal malformation (VGAM) is a rare foetal anomaly associated with neurodevelopment delay, cardiac failure, and even perinatal death. We aimed to assess prenatal features of VGAM and describe postnatal outcomes. This was a retrospective study involving six foetuses diagnosed with VGAM prenatally in two centres. Read More

View Article and Full-Text PDF
January 2022

Vein of Galen Malformation Manifesting as High-Output Heart Failure.

Cureus 2021 Dec 1;13(12):e20067. Epub 2021 Dec 1.

Pediatrics, University of Texas Health Science Center at Houston, Houston, USA.

The sudden onset of vomiting in a previously healthy term neonate has a broad differential requiring a thorough history and physical examination. When this does not reveal the underlying cause, a workup must be performed to rule out potentially devastating diagnoses that must be addressed in a timely fashion. In infants, this clinical presentation could be due to infections such as sepsis or meningitis, gastrointestinal causes such as anatomical abnormalities or ingestions, or cardiac causes such as congenital heart disease. Read More

View Article and Full-Text PDF
December 2021

Transvenous embolization of vein of galen aneurysmal malformations using the "Chapot pressure cooker" technique.

Interv Neuroradiol 2021 Dec 23:15910199211066986. Epub 2021 Dec 23.

Cerebrovascular Center, 22524Department of Neurosurgery, 5944Mount Sinai Health System, New York, USA.

Methods: Two patients, one 5-year-old and one 7-year-old, both presented with congestive heart failure in the newborn period and were subsequently treated in the newborn period with multiple, staged TAEs with n-BCA for choroidal VGAMs.

Results: We achieved progressive reduction in shunting and flow but were unable to accomplish complete closure of the malformation: in both patients, a small residual with numerous perforators persisted. The decision was made to perform TVE using the CHPC. Read More

View Article and Full-Text PDF
December 2021

Vein of Galen aneurysm, dilated cardiomyopathy, and slender habitus in a patient with a recurrent pathogenic variant in PACS2.

Am J Med Genet A 2022 03 11;188(3):991-995. Epub 2021 Dec 11.

Department of Clinical and Molecular Genetics and Rare Disease Unit, Valle Hebron Research Institute, Barcelona, Spain.

The PACS2 gene encodes a multifunctional sorting protein involved in nuclear gene expression and pathway traffic regulation that has been shown to be highly expressed during human prenatal brain development. Pathogenic variants in PACS2 have been recently shown to be implicated in a phenotype with global developmental delay/intellectual disability, seizures, autistic traits, facial dysmorphic features, and cerebellar dysgenesis. Here, we report a 25-year-old male with intellectual disability, epileptic encephalopathy, cerebellar dysgenesis, facial dysmorphism, and a previously reported pathogenic variant in PACS2. Read More

View Article and Full-Text PDF

Microsurgical Resection of a Giant Posterior Fossa Aneurysmal Malformation in a 21-Month-Old.

World Neurosurg 2021 Nov 27;158:165. Epub 2021 Nov 27.

Department of Neurosciences and Pediatrics, University of California San Diego, San Diego, California, USA. Electronic address:

Pediatric aneurysms commonly occur in the vertebrobasilar circulation with complex morphologies. "Aneurysmal malformations," or fistulous vessel dilations without a nidus, have also been described. Vessel friability and sensitivity to blood loss can complicate surgery. Read More

View Article and Full-Text PDF
November 2021

Vein of Galen aneurysmal malformation presenting as severe heart failure in a neonate.

Radiol Case Rep 2021 Dec 21;16(12):3961-3964. Epub 2021 Oct 21.

Radiology department, Childrens' hospital - Ibn Sina university hospital-Rabat, Lamfadel Cherkaoui street-10010 Rabat, MOROCCO.

The vein of Galen aneurysmal malformation (VGAM) is a rare cerebral arteriovenous malformation that can be life-threatening if not diagnosed and treated early. VGAM usually presents in the neonatal period with high-output cardiac failure. We report the case of a full-term male neonate who presented with respiratory distress, and a fontanel bruit soon after birth. Read More

View Article and Full-Text PDF
December 2021

Differentiation of Chiari malformation type 1 and spontaneous intracranial hypotension using objective measurements of midbrain sagging.

J Neurosurg 2021 Oct 29:1-8. Epub 2021 Oct 29.

1Department of Radiology, Division of Neuroradiology, Duke University Medical Center, Durham, North Carolina.

Objective: Chiari malformation type 1 (CM-1) and spontaneous intracranial hypotension (SIH) are causes of headache in which cerebellar tonsillar ectopia (TE) may be present. An accurate method for differentiating these conditions on imaging is needed to avoid diagnostic confusion. Here, the authors sought to determine whether objective measurements of midbrain morphology could distinguish CM-1 from SIH on brain MRI. Read More

View Article and Full-Text PDF
October 2021

Hirayama Disease in a Patient with a History of Late-Onset Symptomatic Vein of Galen Aneurysmal Malformation.

Pediatr Neurosurg 2021 11;56(6):555-562. Epub 2021 Oct 11.

Neurosurgery Division, Department of Pediatric Surgery, Montreal Children's Hospital, McGill University, Montreal, Québec, Canada.

Introduction: The purpose of this report is to present a rare case of Hirayama disease (HD) in a patient with a history of late-onset symptomatic vein of Galen aneurysmal malformation (VGAM). This report may provide new insights into the pathophysiology of HD, a rare disorder consisting of insidious onset of unilateral weakness and atrophy of the forearm and intrinsic hand muscles. These symptoms are believed to result from cervical myelopathy affecting the anterior horn cells due to abnormal compressive forces on the spinal cord from adjacent anatomical structures (i. Read More

View Article and Full-Text PDF
November 2021

Management of Jugular Bulb Stenosis in Pediatric Vein of Galen Malformation: A Novel Management Paradigm.

Pediatr Neurosurg 2021 6;56(6):584-590. Epub 2021 Oct 6.

Division of Otology and Neurotologic Skull Base Surgery, Department of Otolaryngology, Head and Neck Surgery, Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA.

Introduction: Pediatric vein of Galen malformations (VOGMs) are fistulous intracranial malformations arising congenitally within the choroidal fissure that can present with an array of neurological and cardiac sequelae. Associated venous stenosis may result in intracranial venous hypertension and ischemia leading to severe, irreversible cerebral injury. Management of neonatal VOGMs typically involves staged embolization and angioplasty/stenting for relief of venous stenosis. Read More

View Article and Full-Text PDF
November 2021

Initial experience with the Scepter Mini catheter for the embolization of vascular malformations in the pediatric population.

Neuroradiol J 2021 Oct 5:19714009211049084. Epub 2021 Oct 5.

Department of Neurosurgery, 5864The Mount Sinai Hospital, The Mount Sinai Hospital, USA.

Background: Achieving distal access and flow control are of significant importance for the treatment of intracerebral arteriovenous shunting lesions. The Scepter Mini catheter is a low-profile, dual-lumen balloon catheter, designed to provide navigability in small-caliber, tortuous intracranial vessels.

Objective: To describe the initial experience of the Scepter Mini catheter in the treatment of pediatric arteriovenous malformations and fistulas. Read More

View Article and Full-Text PDF
October 2021

Dural Sinus Arteriovenous Malformation in the Fetus. Case Report and Discussion of the Literature.

Diagnostics (Basel) 2021 Sep 9;11(9). Epub 2021 Sep 9.

Fetal Medicine and Surgery Service, Department of Clinical Sciences and Community Health, University of Milan, 20121 Milan, Italy.

Sonographic findings of cerebral arteriovenous malformations in the fetus are uncommon and usually regard aneurysm of the Galen vein. Outcome of arteriovenous malformations is usually severe. We report a case of a fetus at 21 weeks' gestation with a rarer arteriovenous malformation, referred to us for echocardiography on account of a suspicious cardiomegaly at obstetrical scan. Read More

View Article and Full-Text PDF
September 2021

Extra corporeal membrane oxygenation support for neonatal vein of Galen aneurysmal malformation: Case report.

J Neonatal Perinatal Med 2021 ;14(3):433-436

Department of Pediatric Critical Care, Cleveland Clinic, Cleveland, OH, USA.

Background: The vein of Galen aneurysm (VGAM) is the most common type of arteriovenous malformation in the neonate. These neonates commonly present with high output cardiac failure that may be associated with pulmonary hypertension. The medical management and stabilization of these neonates can be challenging before staged transarterial embolization of the aneurysm is undertaken. Read More

View Article and Full-Text PDF
December 2021

Review on the current treatment status of vein of Galen malformations and future directions in research and treatment.

Expert Rev Med Devices 2021 Oct 30;18(10):933-954. Epub 2021 Aug 30.

School of Medicine, European University of Cyprus, Nicosia, Cyprus.

Introduction: Vein of Galen malformations (VOGMs) represent a rare pathologic entity with often catastrophic natural history. The advances in endovascular treatment in recent years have allowed for a paradigm shift in the treatment and outcome of these high-flow shunts, even though their pathogenetic mechanisms and evolution remain in part obscure.

Areas Covered: The overall management of VOGMs requires a tailored case-to-case approach, starting with in utero detection and reserving endovascular treatment for indicated cases. Read More

View Article and Full-Text PDF
October 2021

Postpartum septic pelvic thrombophlebitis and ovarian vein thrombosis after caesarean section: a rare case report.

BMC Pregnancy Childbirth 2021 Aug 17;21(1):561. Epub 2021 Aug 17.

Department of Obstetrics and Gynecology, Affiliated Hospital of Nantong University, Nantong, People's Republic of China.

Background: Septic pelvic thrombophlebitis (SPT) is a well-recognized but rare puerperal complication that has two types: ovarian vein thrombophlebitis (OVT) and deep septic pelvic thrombophlebitis (DSPT). The present case report describes the clinical and imaging findings of a female patient diagnosed with right ovarian vein infectious thrombophlebitis after caesarean section (C-section).

Case Presentation: A 35-year-old G3P2 female who presented with a foetal vein Galen malformation at 41 weeks of gestation underwent C-section. Read More

View Article and Full-Text PDF

Endovascular management of anterior falcotentorial dural arteriovenous fistulas: importance of functionality of deep venous system and existence of accompanying choroidal arteriovenous malformation.

J Neurointerv Surg 2022 Jun 28;14(6):599-604. Epub 2021 Jul 28.

Department of Radiology Division of Neuroradiology, Istanbul University-Cerrahpasa Cerrahpasa Faculty of Medicine, Istanbul, Turkey.

Background: Anterior falcotentorial junction dural arteriovenous fistulas (AFDAVFs) are the most deeply located and most complex type of tentorial fistula due to their location and vascular anatomy. We aimed to make new angiographic definitions of AFDAVF nidus and functionality of the deep venous system of the brain and thereby provide a safer approach for endovascular treatment.

Methods: We retrospectively examined 18 patients with AFDAVF who received endovascular treatment at our neuroradiology department between June 2002 and May 2019. Read More

View Article and Full-Text PDF

Transvenous Pressure Monitoring Guides Endovascular Treatment of Vein of Galen Malformation: A Technical Note.

Pediatr Neurosurg 2021 9;56(4):401-406. Epub 2021 Jun 9.

Department of Neurosurgery, Cedars-Sinai Medical Center, Los Angeles, California, USA.

Introduction: Vein of Galen malformations (VGMs) are complex congenital arteriovenous malformations that generally require serial endovascular treatment sessions to slowly correct the high-flow fistulous connections that cause increased venous pressures and ultimately lead to the classic presentations of heart failure, hydrocephalus, and intracranial hemorrhages. Despite the advances in endovascular technology and embolic materials, the resolution of embolization is often limited to the subjective view of diminished flow on angiograms.

Case Report: An 8-month-old patient with a VGM developed clinical signs of heart failure and growing head circumference with ventriculomegaly. Read More

View Article and Full-Text PDF
October 2021

High-flow vascular malformations of the brain in pediatrics: Experience in a tertiary care children's hospital.

Arch Argent Pediatr 2021 06;119(3):152-161

Coordinación de Diagnóstico por Imágenes. Hospital de Pediatría "Prof. Dr. Juan P. Garrahan, Ciudad Autónoma de Buenos Aires.

Introduction: High-flow vascular malformations of the brain are uncommon in pediatrics.

Objective: The objective of this study is to establish the differences among these pathologies and group them by age at onset, clinical manifestations, and angioarchitecture.

Population And Method: This was a retrospective and observational study. Read More

View Article and Full-Text PDF

An Alternative Endovascular Technique for Vein of Galen Aneurysmal Malformation Treatment: Etylene Vinyl Alcohol Co-Polymer Embolization via Double-Lumen Balloon Microcatheter.

Turk Neurosurg 2021 ;31(4):661-664

Erciyes University, School of Medicine, Department of Radiology, Kayseri, Turkey.

Aim: To present an alternative endovascular treatment option for the vein of Galen aneurysmal malformation by ethylene-vinyl alcohol copolymer embolization via a double-lumen balloon microcatheter.

Material And Methods: A female patient was suspected with the vein of Galen aneurysmal malformation in the prenatal period and diagnosed choroidal type vein of Galen aneurysmal malformation. Once the patient was symptomatic with severe cardiac failure, an endovascular treatment decision was made. Read More

View Article and Full-Text PDF

Intracranial Dural Arteriovenous Fistulas: The Sinus and Non-Sinus Concept.

Acta Neurochir Suppl 2021 ;132:113-122

Department of Neurosurgery, ASST Grande Ospedale Metropolitano Niguarda, Milan, Italy.

Introduction: Dural arteriovenous fistulas (dAVFs) account for 10-15% of all intracranial arteriovenous lesions. Different classification strategies have been proposed in the course of the years. None of them seems to guide the treatment strategy. Read More

View Article and Full-Text PDF