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

High Output Cardiovascular Physiology and Outcomes in Fetal Diagnosis of Vein of Galen Malformation.

Vein of Galen aneurysmal malformation (VGAM) is a rare anomaly associated with poor outcomes from high output cardiac failure and neurologic complications. Studies addressing fetal cardiovascular status and outcomes in this population are limited. A single-center retrospective review was conducted on patients with a prenatal diagnosis of VGAM who underwent a fetal echocardiogram between January 2015 and July 2019. Fetal echocardiographic data, brain magnetic resonance imaging (MRI) findings and outcomes were collected. Nine fetuses [median gestational age at echocardiogram 34 (1.1) weeks] were included. All patients had superior vena cava dilation and reversal of diastolic flow in the transverse aortic arch. Median cardiothoracic (CT) ratio was 0.39 (0.09). Right ventricular (RV) and left ventricular (LV) dysfunction was present in 66% and 11% fetuses, respectively. Four out of five patients that underwent postnatal endovascular neurosurgical interventions at our center were alive at follow-up (mean 2.7 years). Of the non-survivors (n = 5), 3 received comfort care because of severe brain damage and died in the neonatal period. Non-survivors more commonly had > mild tricuspid regurgitation (TR) (40% vs. 25%) and > mild RV dilation (60% vs. 25%). Combined cardiac index (CCI) was higher in non-survivors when compared to survivors (672.7 vs. 530.2 ml/kg/min, p = 0.016). Fetuses with significant parenchymal damage on brain MRI tended to have a higher CCI than those without (979.8 vs. 605.0 ml/kg/min, p = 0.047). RV dysfunction, TR and elevated CCI are more commonly seen in non-survivors with VGAM. A higher CCI is seen in those deemed untreatable due to significant parenchymal volume loss. Future multicenter studies are needed to assess for prenatal prediction of outcomes in this high-risk population.

Repair of Sinus Venosus Defect and Anomalous Pulmonary Veins Associated With Vein of Galen Malformation.

Sinus venosus defect (SVD) is a deficiency in the sinus venosus portion of the atrium, often associated with partial anomalous pulmonary venous connections. In patients with vein of Galen malformation (VGAM), SVD provides a unique challenge with respect to embolization treatment. In our case, the child had percutaneous device closure of the SVD prior to VGAM embolization. Nine years later, he required surgical removal of the SVD device and Warden procedure due to progressive right-sided cardiac enlargement.

Bilateral carotid and vertebral rete mirabile with vein of Galen aneurysmal malformation: an unreported association.

Rete mirabile is a fine meshwork of anastomosing vessels that replace the parent artery. A 30-year-old woman complained of slurring of speech, right eye proptosis, recurrent vomiting, and loss of bladder and bowel control, followed by drowsiness lasting 30-40 min, for the past 6 months. On cross sectional imaging and angiography, the patient was found to have a vein of Galen aneurysmal malformation, with bilateral carotid and vertebral rete mirabile. The patient was offered both endovascular and open surgical options but she refused any form of surgical treatment and opted for conservative management. At the 6 month follow-up, she continued to have occasional episodes of headache and vomiting but was otherwise normal. We describe the clinical, cross sectional, and angiographic features of this patient. A comparison with other patients with bilateral carotid and vertebral rete mirabile is also reported.

Vein of Galen Arteriovenous Malformation in a Neonate.

The vein of Galen is the most common type of arteriovenous malformation in the fetus and neonate. Most vein of Galen arteriovenous malformations (VGAMs) are diagnosed in the neonatal period, with the remainder being identified in early childhood, typically via computed tomography scan. The VGAM is found in five different patterns where the vein of Galen and straight sinus extending to the torcula Herophili are dilated. This dilation can lead to significant compression of the posterior fossa structures. Clinically, the infant with this malformation can present with seizures or most commonly, high output cardiac failure. It is important, however, to keep a broad differential diagnosis as more prevalent neonatal conditions arise similarly. These conditions can include developmental delay, cerebral palsy, epilepsy, superior vena cava syndrome, hemangioendothelioma, and other arteriovenous fistulae. Treatment begins with early diagnosis and testing of initial sequelae. This is often accomplished in consultation with different pediatric subspecialists, particularly neurologists and cardiologists. The mainstay of therapy is with neurosurgical intervention. Although the mortality of a fetus or neonate with VGAM is very high, prognosis is dependent on the size of the malformation, age at diagnosis, and successful neurosurgical outcome.

Pediatric central nervous system vascular malformations.

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.

Vein of galen aneurysmal malformation: prognostic markers depicted on fetal MRI.

Fetal magnetic resonance imaging (MRI) serves a dual role in the prenatal diagnostic work up of a vein of Galen aneurysmal malformation (VGAM). First, it may confirm the prenatal ultrasound findings and secondly it may identify prognostically important secondary complications of the VGAM. Progressive heart failure with development of fetal hydrops and hemispheric white matter injuries are associated with a poor outcome in children with a VGAM. We present the prenatal findings using both ultrasound and MRI of a fetus with VGAM including bilateral injury of the cerebral hemispheres, severe dilatation of the jugular veins, cardiomegaly, and hydrops fetalis. The neonate died within 30 minutes after delivery. Moreover, fetal MRI revealed complete placenta praevia, uterine fibroids, and wrapping of the umbilical cord around the fetal neck. This additional information is unrelated to the fetal pathology, but could have been of importance to plan the delivery.

The significance of 3D power Doppler in prenatal diagnosis and the evaluation of the anatomical structure of vein of Galen aneurysmatic malformation: case report.

Prenatal diagnosis of vein of Galen aneurysmatic malformation (VGAM) is made with the use of color Doppler, while in B-mode it is seen as a centrally placed supratentorial cystic structure. 3D-power Doppler (3D-PD) is a method that enables precise visualization of the vascular anatomy of this complex malformation. In our case, VGAM was detected in the 33rd week of gestation with power Doppler, and the use of 3D-PD enabled better visualization of the angioarchitecture and detection of feeding and drainage vessels of aneurysmatic widening. The diagnosis was confirmed postnatally with the use of MRI. A prenatal study of the angioarchitecture could have prognostic significance as well as being important in the therapeutic approach during the postnatal period.

Teaching neuroimages: vein of Galen aneurysm mimicking pineal mass in a young adult.

A 22-year-old man presented with intractable progressive headaches over 2 months. Noncontrast head CT and contrast-enhanced MRI scan revealed a hyperdense pineal-based mass (figure, A and B). Arteriogram was normal (figure, C and D).

Vascular malformations of the brain.

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.

[A case of vein of Galen aneurysmal malformation with a newly developed dural arteriovenous fistula after successful embolization].

We report a case of vein of Galen aneurysmal malformation (VGAM) with a newly developed dural arteriovenous fistula (AVF) subsequent to successful embolization. A male neonate diagnosed as VGAM with prenatal ultrasonography and MRI presented severe cardiac and respiratory failure soon after birth. Five sessions of transarterial embolization using NBCA were performed during the first 6 months of his life. The shunt flow was effectively reduced and heart failure was resolved after the treatment. Follow-up angiography performed 2.5 years after the last embolization revealed complete obliteration of VGAM and newly developed small dural AVF on the wall of the thrombosed falcorial sinus. We believe that the dural AVF in this case was caused by local venous hypertension or induction of angiogenic factor during the thrombosing process of VGAM.

Endovascular treatment of vein of Galen aneurysmal malformation: management strategy and 21-year experience in Toronto.

OBJECT: The treatment of vein of Galen aneurysmal malformation (VGAM) is among the most challenging of all the neurovascular disorders. METHODS: Between 1984 and 2005, 26 consecutive patients with VGAMs were treated by the authors' group, and their data were prospectively collected; 12 patients presented with congestive heart failure (CHF) and 10 had hydrocephalus. Five patients did not undergo endovascular treatment because of minimal symptoms or severe comorbidities. Twelve patients underwent embolization for refractory CHF requiring ventilation: 7 of these 12 patients had the procedure in the neonatal stage (5 survived), and 5 were treated in infancy or childhood following successful aggressive medical treatment (4 survived). The other 9 patients underwent endovascular surgery to treat a progressively enlarging head circumference; there was imaging evidence of ventricular enlargement and/or signs of developmental delay (6 underwent surgery in infancy and 3 in childhood; 1 patient died). RESULTS: The survival rate in this series was 76.9% (20 of 26). Fourteen (66.7%) of 21 patients who underwent endovascular treatment had no developmental delay. An analysis of various factors demonstrated that comorbidities and embolization effect (procedure success and long-term effect) were prognostic factors of survival. In addition, the patient's age at embolization was significantly higher in those with developmental delay than in those without (559.86 ± 535.43 days vs 94.83 ± 95.77 days, respectively; p = 0.028). CONCLUSIONS: The authors conclude that neonatal VGAMs can be treated successfully with a strategic approach integrating antenatal diagnosis, endovascular surgery, treatment at intensive care facilities, and the cooperative efforts of different specialties. In the authors' experience, patients in whom VGAM was diagnosed and managed in infancy or childhood had more than 90% long-term survival.

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

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.

Diagnosis and treatment of vein of Galen aneurysmal malformations.

INTRODUCTION: Vein of Galen aneurysmal malformations (VGAM) are rare but clinically significant intracranial arteriovenous shunt lesions that most often present in neonates and infants. METHODS: Retrospective clinical data were collected for patients evaluated with a diagnosis of VGAM from 1994 to 2007. RESULTS: Thirteen patients with VGAM were evaluated from 1994 to 2007. Seven patients presented emergently with medically intractable cardiac failure, and six were treated in the first 2 weeks of life. Five children treated after this period (1.5-31 months of age) manifested enlarging head circumference, abnormal development, or subarachnoid hemorrhage. Eleven patients were managed endovascularly. Four disease or procedure-related complications occurred. Two complications were associated with poor outcome, both of which occurred in patients treated at less than 2 weeks of age. Two other patients experienced transient neurological deficits with no evidence of permanent sequelae. Outcome in the six patients treated emergently in the first 2 weeks of life included two patients who developed normally, one with mild to moderate neurological deficits, one with severe neurological deficits, and two deaths. Outcome in the five older patients (treated between 1.5 and 31 months) was considerably better than in the group treated early and included three with normal outcome and two with mild neurological deficits. CONCLUSIONS: Contemporary endovascular techniques remain the preferred treatment for VGAM in all age groups. Early diagnosis and multimodality treatment are essential for the best management and treatment of the complex constellation of clinical problems often arising from this disorder.

[Management and outcome of vein of galen malformation in childhood]. / Management und Outcome der Vena-Galeni-Malformation im Kindesalter.

The vein of Galen malformation (VGM) is a rare congenital cerebral vascular malformation. In most cases it already has been diagnosed prenatally. The impact of heart failure due to the arteriovenous shunt is relevant for the prognosis.Prognosis of neonatal symptomatic VGM with early heart failure is severe. Therefore an interdisciplinary cooperation is necessary and it is important to treat patients in a clinic with a perinatal center and a neuroradiology and neurosurgery that is experienced in treating such patients. The genesis, the pathophysiology, the diagnostic agents, the therapeutic agents and the prognosis of VGM will be described.

Vein of Galen malformation presenting in adulthood.

Vein of Galen malformations are rare vascular abnormalities, which develop during gestation and usually present in the neonatal period or early childhood. Symptoms are due to high output cardiac failure, hydrocephalus, seizures or haemorrhage. We report a case where a patient remained symptom free until presentation following a haemorrhage at the age of 47 years.