Galen Aneurysmal Malformation Research Articles

The Impact of Angiographic Geometry on the Clinical Presentation of True Vein of Galen Aneurysmal Malformations

Background Purpose: Vein of Galen aneurysmal malformations (VGAM) may carry life-threatening and catastrophic sequelae in children. Their clinical presentations are differently variable between cardiac and cerebral syndromes. There may be a possible relationship between the Galenic angioarchitecture and the clinical presentation. We aimed to study the effect of the venous outflow impairment on the incidence of high-flow heart failure and the incidence of hydrocephalus. Materials and Methods: The angiographies of 21 patients with true VGAM have been studied. MR angiography, 3-dimensional CT angiogram (3D CTA), and conventional digital subtraction angiography were performed for all patients with true VGAM. Transarterial embolization was done in 1 or more sessions for all cases. Results: Among the 21 cases, 14 cases were mural type, 5 cases were choroidal type, and 2 cases were mixed. Hydrocephalus was observed more in infants (92.3%), mural types (92.9%), giant and supergiant (87.5%) aneurysms, and in cases with persistent limbic circle (90.91%). All cases of hydrocephalus were associated with significant stenosis (>70%) of the Falcine sinus draining system (100%). Conclusion: Significant stenosis (>70%) of the draining sinus is a significant factor for VGAM aneurysmal enlargement and occurrence of hydrocephalus. Probably, venous outflow impairment decreases the incidence of high-flow heart failure and increases the incidence of hydrocephalus.

The Vein Of Galen Aneurysmal Malformation Associated With Sinus Venosus Defect And Anomalous Pulmonary Veins

he Vein of Galen Aneurysmal Malformation (VGAM) is a rare cerebral arteriovenous shunt, which may be associated with a congenital cardiac defect. Embolisation of the VGAM is safer in infancy, even it may be undertaken in the neonatal period if necessary.

Артеріовенозна мальформація вени Галена: пренатальна діагностика і постнатальні наслідки (аналіз даних літератури та власних спостережень)

Purpose — to assess ultrasound criteria and diagnostic value at vein of Galen malformation (VGAM) throughout perinatal period with possible further mortality rate and psychomotor development prognosis. Materials and methods. This was retrospective study involving 9 cases of VGAM diagnosed prenatally and managed at two institutions over a 5-year period (2014–2019). All cases had undergone detailed prenatal and perinatal cerebral, cardiac and fetoplacental unit assessment by grayscale ultrasound, color and pulsed–wave Doppler. In order to determine further treatment tactics neurosurgical consultation was involved into all confirmed VGAM cases. Results. Pregnancy and fetoneonatal outcome were known in all cases. Minor size supratentorial arachnoid cysts were detected in 6 VGAM cases. Vascular origin of formations was confirmed with Doppler scan. However, no signs of parenchymal abnormalities, liquor system of the brain damage and heart failure have been identified. All newborns were discharged with further outpatient follow-up. Vascular malformation with cardiomegaly correlation, tricuspid regurgitation, dilation of the right atrium and upper cava vein, severe brain abnormalities were considered by definition to be associated with poor outcome in 3 cases. Poor outcome was defined as death. Conclusions. VGAM diagnosis in newborns is highly determined by timely prenatal diagnosis and must involve postnatal neurosurgical assessment. Clarification of the diagnosis contributes to establishing the prognosis and inpatient care tactics. Color and pulsed+wave Doppler assessment is necessary for differential diagnosis with other midline cystic abnormalities of the brain. It is recommended to consider delivery within the perinatal clinic. Care must be provided by highly qualified perinatal team of obstetricians, neurosurgeons and neonatologists with an extensive experience in managing high risk pregnancies. Fetoneonatal outcome is poor due to congestive heart failure, severe brain damage and neurological impairment with tendency to worsen if diagnosed prenatally. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of these Institutes. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: vein of Galen aneurysmal malformation, fetus, newborn, diagnostic value, ultrasound, perinatal care, fetoneonatal outcome.

Prenatal Diagnosis of Vein of Galen Aneurysmal Malformation Allows Early Transumbilical Endovascular Treatment

AbstractNeonates with vein of Galen aneurysmal malformation (VGAM) presenting with severe cardiac failure and pulmonary hypertension represent a challenge for endovascular therapy. When early treatment is required, the small femoral arteries in this population are usually difficult to cannulate. Alternatively, the umbilical vessels offer a natural pathway to reach the lesion. Therefore, prenatal diagnosis of VGAM allows for delivery planning, perinatal management, and embolization through umbilical approach, thus leading to better outcomes.

Galenic Pial Arteriovenous Fistulas in Adults

BackgroundVein of Galen aneurysmal malformations (VOGMs) are pial arteriovenous fistulas possessing Galenic venous drainage most commonly presenting during the neonatal period and infancy, with initial discovery during adulthood quite rare. Objectives and methodsWe conducted a literature survey of the PubMed database in order to identify Galenic pial arteriovenous fistulas (GPAVFs) with major manifestation or initial presentation during adulthood. Inclusionary criteria included pial AVFs with Galenic drainage with major manifestation or initial presentation at, or older than, 18 years. Exclusionary criteria included exclusive pediatric onset of symptomatology attributable to GPAVFs without a new onset major presentation during adulthood, exclusive or major dural arterial supply, arteriovenous malformations with Galenic drainage, developmental venous anomalies with Galenic drainage, isolated varices or anomalies of the vein of Galen, and any lesions with uncertainty regarding true GPAVF nature. ResultsOur search generated 1589 articles. Excluding duplicates, 26 cases met criteria for evaluation. Mean age was 34.1 +/− 2.53 years. Clinical presentations of GPAVFs among adults included headache, intracranial hemorrhage, seizures, and focal neurologic deficits. Management strategies included observation (n = 5), emergent ventriculostomy or Torkildsen shunt (n = 3), cerebrospinal fluid diversion via ventriculoperitoneal shunting (n = 4), microsurgical obliteration or thrombectomy (n = 4), transarterial and/or transvenous embolotherapeutic obliteration (n = 7), and concurrent embolotherapy and radiosurgical irradiation (n = 1). ConclusionsGPAVFs in adults often present with symptomatology of mild severity and may be effectively managed conservatively, though occasionally present catastrophically or may be treated via cerebrospinal fluid diversion, microsurgical obliteration, or endovascular embolization. Severity sufficient to require emergent intervention portended a poor outcome.

Deep venous communication in vein of Galen malformations: incidence, Imaging, and Implications for treatment

BackgroundFailure to appreciate deep venous drainage pathways is a major cause of severe complications in the endovascular treatment of vein of Galen aneurysmal malformations (VOGMs).ObjectiveTo report deep venous drainage patterns...

Diagnosis of foetal vein of galen aneurysmal malformation by ultrasound combined with magnetic resonance imaging: a case series

BackgroundFoetal vein of Galen aneurysmal malformation (VGAM) is a very rare congenital malformation of the cerebral blood vessels. We sought to evaluate the diagnostic value of ultrasound in combination with magnetic resonance imaging (MRI) in foetal VGAM.Case presentationPrenatal ultrasound combined with MRI diagnosed five cases of VGAM. Two dimensional ultrasound images were used to find the echo-free cystic structure below the thalamus and above the cerebellum with five cases. Colour blood flow showed dilated VGAM in five cases, while the arteriovenous spectrum was explored in two cases and foetal heart failure was found in other three cases. MRI was manifested as a dilated VGAM found at the midline of the brain, demonstrating widening or dilation of the straight sinus in four cases, ventricular dilatation in one case, brain parenchyma bleeding in two cases, and grey matter softening in one case. One infant died on the day of its birth, while the other four infants died within one month to six months after birth.ConclusionsUltrasound combined with MRI can more accurately and comprehensively observe the pathological characteristics of VGAM, diagnose related complications early and determine its prognosis.

Pediatric intracranial arteriovenous shunts: Advances in diagnosis and treatment.

Pediatric intracranial arteriovenous shunts (IAVSs) comprise a wide range of lesions, including pial arteriovenous malformation (AVM) and arteriovenous fistula (AVF), dural arteriovenous fistula (DAVF) and vein of Galen aneurysmal malformation (VGAM). We provide an overview of pediatric IAVSs, encompassing both diagnosis and endovascular treatment. We include references from pertinent literature and representative cases from our hospital. Pediatric IAVS are frequently associated two broader conditions, such as capillary malformation-arteriovenous malformation (CM-AVM) and hereditary hemorrhagic telangiectasia (HHT). These conditions and the associated genetic mutations have only recently been described. Their impact on the brain will be different in the prenatal period, in neonates, in infants, and in children, with variable symptoms according to each age group and the current strategies of endovascular treatment in the management of these vascular lesions will be discussed. This review could improve the understanding of pediatric IAVSs and their diagnosis and treatment.

Vein of Galen aneurysmal malformation in a neonate presenting with congestive heart failure

Vein of Galen aneurysmal malformation in a neonate presenting with congestive heart failure

Late Discovery of a Vein of Galen Aneurysmal Malformation Complicated with Hydrocephalus: A Case Report

The aneurysmal malformation of the vein of Galen is a rare condition, corresponding to less than 1% of intracranial vascular malformations. It is a disease affecting mainly newborns and small children. We are reporting the case of an 11-year-old teenager who presented consciousness disorders associated to generalized convulsive seizures. The cerebral CT scan supplemented by an angioscanner, revealed an important expansive process in the pineal region, confirming the diagnosis of an aneurysmal malformation of the Galen’s ampulla with upstream obstructive hydrocephalus. The child benefited from a ventriculo-peritoneal shunt with favorable immediate operative sequels. His death occurred at home two months after surgery over the course of a convulsive seizure with post critical coma.

An alternative endovascular technique for the vein of galen aneurysmal malformation treatment: ethylene vinyl alcohol copolymer embolization via a double-lumen balloon microcatheter

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. In the first step, a mixture of N-butyl cyanoacrylate-lipiodol was used for embolization. In the second session of the treatment, ethylene-vinyl alcohol copolymer was administered through a double-lumen balloon microcatheter. Results Complete cure of the aneurysmal malformation was obtained by no filling was observed in arterial feeders, collapsed appearance of the vein of Galen, and arterial-venous shunts at the end of the five-year follow-up period with magnetic resonance imaging and angiography. Conclusion Ethylene-vinyl alcohol copolymer embolization via double-lumen balloon microcatheter provides an alternative and effective endovascular treatment option for the vein of Galen aneurysmal malformations using less contrast agent in less procedural time. Also, the possibility of spontaneous regression of residual aneurysmal malformations with small feeders should be considered.

Vein of Galen Aneurysmal Malformation Presenting with Obstructive Hydrocephalus: A Case Report

The prognosis for children harboring vein of Galen aneurysmal malformation (VGAM) has significantly improved over the last three decades due to refinements in the interventional endovascular techniques. It is now possible to obliterate the malformation with better results. Endovascular approaches evolved as the gold standard treatment option for this disorder. A few reports were published warning about the high rate of complications associated with ventriculoperitoneal shunting (VPS) in this setting. We present a pediatric case with VGAM where an emergency ventriculoperitoneal shunt procedure was required for symptomatic obstructive hydrocephalus (HCP) and management of the subsequent complications that followed it.

Vein of Galen Aneurysmal Malformation Presenting with Obstructive Hydrocephalus: A Case Report

The prognosis for children harboring vein of Galen aneurysmal malformation (VGAM) has significantly improved over the last three decades due to refinements in the interventional endovascular techniques. It is now possible to obliterate the malformation with better results. Endovascular approaches evolved as the gold standard treatment option for this disorder. A few reports were published warning about the high rate of complications associated with ventriculoperitoneal shunting (VPS) in this setting. We present a pediatric case with VGAM where an emergency ventriculoperitoneal shunt procedure was required for symptomatic obstructive hydrocephalus (HCP) and management of the subsequent complications that followed it.

Neuronavigation of the Galen vein Aneurysm.

The Vein of Galen aneurysmal malformation (VGAM) is a dilatation of the vein of Galen with multiply communications between of the vein of Galen and venous sinus and the cerebral arteries, occurring between 6 and 11 weeks of gestation

Long-term outcome of vein of Galen malformation.

To describe the long-term outcomes of children by the time they reached school age with vein of Galen aneurysmal malformation (VGAM). This was a retrospective observational study on a consecutive cohort of patients with VGAM. We included patients with at least one Francophone parent, aged between 6 and 11 years at the time of long-term evaluation. The neurological outcome was assessed with the King's Outcome Scale for Childhood Injury score and eight neurological and behavioural items from the Rivermead Postconcussion Symptoms questionnaire. All 52 patients (17 females, 32 males [data missing for n=3]) with at least one Francophone parent (5 fetuses and 47 children) were included. At the long-term evaluation time-point, 33 patients were alive and 19 patients had died. Risk of postnatal death was associated with severe neonatal cardiac failure (p=0.007) or isosystemic or suprasystemic pulmonary hypertension (p=0.014). Among survivors, 19 had a good outcome with normal schooling and 14 had a poor outcome. Moreover, among the good outcome patients, a large proportion had neurodevelopmental alterations. Long-term outcome of patients with VGAM appears to be less favourable than outcome described at the short- and medium-term, even in the absence of encephalomalacia at birth. Even patients with good outcome often have neuropsychological disorders that may have repercussions on learning and requiring appropriate rehabilitation or medical management. Long-term outcome appears to be less favourable than described at short- and medium-term follow-up. Even patients with good outcome at these time-points often have minor neuropsychological disorders.

EP08.03: The diagnosis of Vein of Galen aneurysmal malformation prenatally by colour Doppler flow imaging

EP08.03: The diagnosis of Vein of Galen aneurysmal malformation prenatally by colour Doppler flow imaging

A case study of galen aneurysmal malformation in Bamako

Galen aneurysmal malformation is a rare complex congenital vascular malformation of the brain, (less than 1% of intracranial arteriovenous malformations) characterized by pseudoaneurismal dilation of Galen's ampulla associated with one or several arteriovenous fistulas. In most cases, diagnosis is made in the neonatal or postnatal period, exceptionally in adulthood. We report the case of an 18-year male patient hospitalized for psychomotor retardation. Brain computed tomography (CT) scan using spontaneous contrast (A,B,C) showed voluminous isodense mass in the pineal region behind the third ventricle with peripheral calcifications, compatible with large vein of galen aneurysm. Its anteroposterior diameter was 8.5cm, its transverse diameter was 3cm in the axial plane (C) and its height was 3cm in the sagittal plane (G). Imaging objectified homogeneous contrast enhancement (D,E,F,G,H,I). The mass was associated with dilation of the superior sagittal sinus measured at 2.7cm (A,D), of transverse sinuses measured at 1.5cm to the right and at 1,1cm to the left (B,E). Multiple venous perianeurysmal derivations (I), hydrocephaly and small parenchymal cortical-subcortical calcifications were detected. Embolization can't be performed in Bamako, so the patient is waiting for transfer.

Vein of Galen Malformations: The Texas Children's Hospital Experience in the Modern Endovascular Era.

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

Outcome of vein of Galen malformation presenting in the neonatal period

ObjectiveVein of Galenaneurysmal malformation (VGAM) is a rare but important congenital malformation presenting to neonatal intensive care units (NICUs), and with a change from surgical to endovascular management, survival for...

Ethical Considerations in Multiple Pregnancy: Preterm Delivery in the Setting of Discordant Fetal Anomaly.

Planning for the preterm birth of a fetus with known anomalies can raise complex ethical issues. This is particularly true of multiple pregnancies, where the interests of each fetus and of the expectant parent(s) can conflict. In these complex situations, parental wishes and values can also conflict with the recommendations of treating clinicians. In this article, we consider the case of a dichorionic twin pregnancy complicated by the diagnosis of vein of Galen aneurysmal malformation (VGAM) in one of the twins at 28 weeks' gestation. Subsequent deterioration of the affected twin prompted the parents to request preterm delivery to prevent the imminent in-utero demise of the affected twin. However, given the associated risks of prematurity, complying with the parents' request may have disadvantaged the health and wellbeing of the unaffected twin. This article canvases the complex ethical issues raised when parents request preterm delivery of a multiple pregnancy complicated by a fetal anomaly in one twin, and the various ethical tools and frameworks that clinicians can draw on to guide their decision-making in such cases.