Vein of Galen Aneurysmal Malformations in Pediatric Patients Submitted to Endovascular Treatment: Report of 3 Cases

Objective To report three cases of vein of Galen aneurysmalmalformation (VGAM) in pediatric patients treated at the hemodynamics lab of Hospital Santa Isabel (HSI) in Blumenau, state of Santa Catarina, Brazil, from 2006 to 2020. Clinical presentation, endovascular treatment, and postprocedure evolution to date are included. Case description Three children aged 5 to 12 months with cardiac, respiratory, or neurological damage in the neonatal stage, were referred to the neurosurgery service and diagnosed with VGAM. The three patients underwent endovascular embolization of themalformation, with different clinical evolution throughout outpatient follow-up. Conclusion Vein of Galen aneurysmal malformations are uncommon vascular abnormalities that, until the advent of endovascular embolization, were associated with high morbidity and mortality rates. Its prognosis is linked with initial clinic, early diagnosis, and timely surgical correction.

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.

Outcome of vein of Galen malformation presenting in the neonatal period.

OBJECTIVE: Vein 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 this condition has improved. However, there is little reported about the medical management decisions of infants with this condition and the associated long-term neurodevelopmental outcomes. We aim to report a single centre experience of both acute treatment and long-term outcomes of VGAM for those infants admitted to our NICU soon after birth. DESIGN: Retrospective cohort study over a 15-year period from 2001 to 2015 inclusive. SETTING: A quaternary NICU at The Royal Children's Hospital, Melbourne, Australia. PARTICIPANTS: 24 newborn infants referred for management of VGAM. There were no eligibility criteria set for this study; all presenting infants were included. INTERVENTIONS: None. MAIN OUTCOMES MEASURES: Clinical neuroimaging data were gathered. Surviving children were formally assessed with a battery of tests administered by a neuropsychologist and occupational therapist/physiotherapist at various ages across early to middle childhood. RESULTS: Fifteen neonates with VGAM did not survive beyond their NICU admission. 10 of these were not offered endovascular intervention. Of the nine surviving infants, only one had a normal neurodevelopmental outcome. CONCLUSIONS: The mortality of VGAM presenting in the neonatal period was high, and rates of normal neurodevelopmental outcome for survivors were low. These findings contribute to our understanding of which neonates should be treated and highlights the importance of providing clinical neurodevelopmental follow-up to survivors beyond their infant years.

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

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.

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.

Serial Quantitative and Qualitative Measurements of Flow in Vein of Galen Malformations Using 4-Dimensional Flow Magnetic Resonance Imaging (Phase Contrast Vastly undersampled Isotropic PRojection).

BACKGROUND: Vein of Galen malformations (VoGMs) induce cerebrovascular dysfunction through arterial steal and venous hypertension resulting, if untreated, in severe neurologic morbidity and mortality. Noninvasive techniques for quantitative, serial evaluation of cerebrovascular hemodynamics in VoGMs are lacking. This proof of concept study using quantitative blood flow measurements from 4-dimensional flow magnetic resonance imaging may be useful as a noninvasive biomarker to guide timing of intervention and assess disease progression and treatment outcomes. CASE DESCRIPTION: Between July 2016 and July 2018, 4 patients harboring VoGMs underwent Phase Contrast Vastly undersampled Isotropic PRojection (PCVIPR) imaging at the University of Wisconsin Hospitals and Clinics. We applied PCVIPR imaging to assess its potential for obtaining anatomic and physiologic flow data before and after surgical embolization of VoGMs. Hemodynamic pressure parameters obtained from PCVIPR imaging were compared with stump pressures recorded in vivo. We found a decrease in mean arterial pressure from 97 mm Hg pretreatment to 65 mm Hg post treatment. These findings corroborate the decrement in venous pressure gradients documented on PCVIPR imaging. For all patients, pressure gradient changes on PCVIPR imaging correlated with in vivo arterial pressures and aided in clinical decision related to cerebrovascular evaluation, treatment planning, and clinical course. CONCLUSIONS: Four-dimensional flow magnetic resonance imaging/PCVIPR imaging has a potential role in determining endovascular embolization and therapeutic outcomes on the basis of objective and reproducible hemodynamic characteristics of the vascular lesion. It represents a novel, noninvasive approach that may guide the extent and timing of therapeutic intervention and treatment of cerebrovascular diseases in pediatric patients.

Vein of Galen malformation treated with the Micro Vascular Plug system: case report.

This case report describes the use of the Micro Vascular Plug (MVP) system in a mural-type vein of Galen aneurysmal malformation, resulting in successful occlusion of the cerebrovascular fistula. This result was confirmed with immediate cessation in blood flow and 6-month follow-up angiography. The unique characteristics of the MVP provide potential advantages over current embolic techniques for occlusion of high-flow, fistulous, intracranial vessels such as those seen in the vein of Galen aneurysmal malformation, and may represent a valuable addition to the currently available options.

Cranial Doppler ultrasound in Vein of Galen malformation.

INTRODUCTION: Vein of Galen malformation (VGM) is the severest paediatric neurovascular disease usually requiring multiple staged embolisations. In the high-risk group of children with high-flow arteriovenous shunts, timing of treatment is uncertain. Low Doppler resistance index (RI) is known to be associated with adverse outcome in hypoxic-ischaemic brain injury in children. In this study, we want to present our long-term results of cranial transfontanellar Doppler ultrasound in children with VGM. METHODS: We identified and retrospectively analysed 264 transfontanellar Doppler measurements in 19 endovascular-treated true VGM (five females, 14 males) between 2000 and 2013. The recordings were obtained from the internal carotid arteries (ICA), the anterior cerebral arteries (ACA) and the basilar arteries (BA). Maximal systolic velocity (Vs), end-diastolic velocity (Ved) and the RI were measured before and after embolisation. RESULTS: Untreated, nearly all cases showed pathologic high systolic (up to >1.0 m/s), very high diastolic velocities (up to >0.5 m/s) and low RI (<0.6). There were statistically significant differences (p = 0.012) between the pre-embolisation RI and the post-embolisation RI with pathologic low RI before and nearly normal RI after successful shunt reduction. CONCLUSION: Successful endovascular shunt reduction in VGM leads to significant changes of cranial Doppler RI from pathologic low values to normal values. We propose cranial Doppler ultrasound as an adjunctive technique to other screenings in the management of VGM. Further research is warranted to evaluate the role of the RI in the treatment timing decision.

The use of adenosine in the treatment of a high-flow vein of Galen malformation in an adult.

The treatment of high-flow vein of Galen aneurysmal malformations (VGAM) remains a therapeutic challenge for the neurosurgeon and the neurointerventionalist, as it is associated with high morbidity and mortality rates despite recent advances in open cranial surgery and interventional neuroradiology. A 37-year-old patient presented with a history of non-specific headaches. He had a history of heart failure since birth that was caused by an untreated VGAM. Intravenous boluses of adenosine were injected as an attempt to slow down the arteriovenous shunting of a VGAM prior to endovascular treatment. Adenosine can be a very useful adjunct in patients with extremely high arteriovenous shunting. n-butyl cyanoacrylate (n-BCA) should be the embolic material of choice due to its quick polymerization and adhesive properties.

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).

Necrotising enterocolitis in a full-term infant with reversed diastolic flow in the descending aorta: what is the diagnosis?

Vein of Galen malformations (VGM) are rare intracranial vascular anomalies that constitute 1% of all intracranial vascular malformations. Untreated VGM have a very poor prognosis. A high proportion of patients who present in the neonatal period rapidly deteriorate and succumb to congestive cardiac failure. The prenatal diagnosis and possible in utero referral to highly specialised centre of postnatal care have resulted in considerable improvement in prognosis. The authors present a case of a postnatally diagnosed VGM in a full-term infant presenting with progressive cardiac failure and necrotising enterocolitis secondary to gut hypoperfusion. They emphasise the importance of prenatal diagnosis by pulsed wave Doppler and colour-velocity imaging for subsequent referral to a centre of excellence in a multidisciplinary approach involving neonatologists, paediatric cardiologists and interventional radiologists in order to optimise the management and limit the neurological sequelae in children with this type of malformation.

Superior vena cava flow and management of neonates with vein of Galen malformation.

OBJECTIVE: Vein of Galen malformation (VGAM) in neonates presents a complex management challenge. Measurement of superior vena cava (SVC) blood flow may provide insights into the haemodynamics of VGAM and the effects of therapeutic intervention. METHODS: SVC flow was assessed in 15 neonates with VGAM. SVC flow results, Bicêtre scores (clinical assessment), echocardiographic assessment and clinical outcomes are presented. RESULTS: SVC flows (166-581 ml/kg/min) were significantly elevated at presentation (p<0.001; normal range 55-111 ml/kg/min). Endovascular intervention was undertaken in 12 cases, with nine survivors. SVC flows decreased sequentially with each embolisation, with a median SVC flow at discharge of 124 ml/kg/min (IQR 79-155 ml/kg/min). All cases with SVC flow >400 ml/kg/min (n=5) had an adverse outcome (death or profound neurological damage). Cases with SVC flow <400 ml/kg (n=10) required embolisation before discharge at a median age of 6 days. There were no survivors with Bicêtre scores <8 (n=2) but the predictive value of early Bicêtre score was poor. CONCLUSIONS: SVC flow measurements provide insight into the haemodynamic challenges of VGAM and provide additional useful prognostic information.

Evolution of treatment options for vein of Galen malformations.

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.

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

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.

Introduction: venous brain circulation disorders.

Brain ischemia by arterial occlusion has been a focus of attention for decades, and cerebral venous disorders have been an underestimated condition of potentially good outcome if diagnosed and treated promptly. Recently, there has been considerable interest in cerebral injury following cerebral venous circulation disorders because diagnosis has improved as our understanding of the diseases and modern imaging technologies have advanced.

Regression of cerebral calcifications after endovascular treatment in a case of vein of Galen arteriovenous malformation.

The authors present the case of an infant harboring a vein of Galen arteriovenous malformation with conspicuous cerebral calcifications that progressively regressed after staged endovascular obliteration of the lesion. The role of venous hypertension and hydrocephalus secondary to the arteriovenous shunt are discussed to explain the formation and regression of the cerebral calcifications.

[Vein of Galen malformations in children].

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.

Repair of sinus venosus defect in a patient with untreated vein of Galen aneurysmal malformation.

The 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 may be undertaken in the neonatal period if necessary, but is safer in infancy. Recent advances in neuroradiology have changed the prognosis for this group with many patients achieving survival with normal development. This case report describes a patient with a sinus venosus defect (SVD) and a VGAM and considers both the optimal timing of treatment of the two malformations and the conduct of anaesthesia for open repair of the SVD in the presence of an untreated VGAM.