Vein of Galen aneurysmal malformation: does size affect outcome?

PURPOSE: To validate a semiautomated method for segmenting vein of Galen aneurysmal malformations (VGAM) and to assess the relationship between VGAM volume and other angioarchitectural features, cardiological findings, and outcomes. METHODS: In this retrospective study, we selected all subjects with VGAM admitted to the Gaslini Children's Hospital between 2009 and 2022. Clinical data were retrieved from electronic charts. We compared 3D-Slicer segmented VGAM volumes obtained by two independent observers using phase-contrast MR venography to those obtained with manual measurements performed on T2-weighted images. The relationship between VGAM volumes and clinical and neuroimaging features was then explored. RESULTS: Forty-three subjects with VGAM (22 males, mean age 6.56 days) were included in the study. Manual and semiautomated VGAM volumes were well correlated for both readers (r = 0.86 and 0.82, respectively). Regarding reproducibility, the inter-rater interclass correlation coefficients were 0.885 for the manual method and 0.992 for the semiautomated method (p < 0.001). The standard error for repeated measures was lower for the semiautomated method (0.04 versus 0.40 of manual method). Higher VGAM volume was associated with superior sagittal sinus narrowing, jugular bulb stenosis, and aqueductal stenosis (p < 0.05). A weak correlation was found between VGAM volume and straight sinus dilatation (r = 0.331) and superior sagittal sinus index (r = - 0.325). No significant associations were found with cardiac findings, post-embolization complications, and outcome (p > 0.05). CONCLUSIONS: Semiautomated VGAM volumetry is feasible and reliable with improved reproducibility compared to the manual method. VGAM volume is not a prognostic factor for clinical outcome, but it is related to other venous findings with potential hemodynamic effects.

Echocardiography evaluation of neonatal vein of Galen aneurysmal malformation.

BACKGROUND: In neonatal vein of Galen aneurysmal malformation, vein of Galen aneurysmal malformation echocardiography remains the mainstay for early detection and explains various haemodynamic changes occurring due to a large systemic arterio-venous shunt. However, there is limited evidence of echocardiography in risk stratifying neonatal vein of Galen aneurysmal malformation vein of Galen aneurysmal malformation. The objective of this study was to identify echocardiographic parameters that could be associated with major outcomes and guide timing of neuro-intervention. METHODS: In this retrospective chart review, infants < 28 days of age with the diagnosis of vein of Galen aneurysmal malformation vein of Galen aneurysmal malformation were included. Demographic, clinical, and echocardiographic parameters were compared in neonates who survived or died with neonatal presentation. A risk algorithm model based on key echocardiographic parameters was developed to determine those who are at risk of early death. RESULTS: Of the 19 neonates included, with median birth weight 3.1 kg (IQR 2.58-3.36), nine (47%) neonates died at median age of 5 days (IQR 4-17). All neonates showed retrograde diastolic flow at the level of descending aorta by colour Doppler on the first post-natal echocardiogram at median age of 2 days (IQR 1-5.5). An aortic antegrade-to-retrograde velocity time integral ratio of < 1.5 and supra-systemic pulmonary artery pressure had 100% positive predictive value of death (p = 0.029), whereas aortic antegrade-to-retrograde velocity time integral ratio of > 1.5 and sub-systemic pulmonary artery pressure had 100% positive predictive value of survival (p = 0.029). CONCLUSION: Combination of aorta antegrade-to-retrograde velocity time integral ratio and degree of pulmonary hypertension on the first post-natal echocardiogram may help stratify the severity of disease and guide optimal timing for neuro-intervention for neonatal vein of Galen aneurysmal malformation.

MV-flow in the assessment of fetal vein of Galen aneurysmal malformation.

MV-Flow is a tool enables to acquire spatially and temporally coherent data on low-speed blood flow information. In our case, it allowed a better definition of vein of Galen aneurysmal malformation morphology, venous drainage and feeder vessels than standard ultrasound techniques.

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

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. IMPACT: Vein of Galen aneurysmal malformation (VGAM) is a rare intracranial arteriovenous malformation, which can present in the neonatal period with high output heart failure. Heart failure secondary to VGAM is often difficult to manage and is associated with high mortality and morbidity. Despite optimal medical management, many patients require urgent endovascular embolization for stabilization of their heart failure. This report offers discrete recommendations that can be used by clinicians as guidelines for the medical management of heart failure in newborns with VGAM.

Cerebral Cavernous Malformation: From Mechanism to Therapy.

Cerebral cavernous malformations are acquired vascular anomalies that constitute a common cause of central nervous system hemorrhage and stroke. The past 2 decades have seen a remarkable increase in our understanding of the pathogenesis of this vascular disease. This new knowledge spans genetic causes of sporadic and familial forms of the disease, molecular signaling changes in vascular endothelial cells that underlie the disease, unexpectedly strong environmental effects on disease pathogenesis, and drivers of disease end points such as hemorrhage. These novel insights are the integrated product of human clinical studies, human genetic studies, studies in mouse and zebrafish genetic models, and basic molecular and cellular studies. This review addresses the genetic and molecular underpinnings of cerebral cavernous malformation disease, the mechanisms that lead to lesion hemorrhage, and emerging biomarkers and therapies for clinical treatment of cerebral cavernous malformation disease. It may also serve as an example for how focused basic and clinical investigation and emerging technologies can rapidly unravel a complex disease mechanism.

Cerebrovascular Anomalies: Perspectives From Immunology and Cerebrospinal Fluid Flow.

Appropriate vascular function is essential for the maintenance of central nervous system homeostasis and is achieved through virtue of the blood-brain barrier; a specialized structure consisting of endothelial, mural, and astrocytic interactions. While appropriate blood-brain barrier function is typically achieved, the central nervous system vasculature is not infallible and cerebrovascular anomalies, a collective terminology for diverse vascular lesions, are present in meningeal and cerebral vasculature supplying and draining the brain. These conditions, including aneurysmal formation and rupture, arteriovenous malformations, dural arteriovenous fistulas, and cerebral cavernous malformations, and their associated neurological sequelae, are typically managed with neurosurgical or pharmacological approaches. However, increasing evidence implicates interacting roles for inflammatory responses and disrupted central nervous system fluid flow with respect to vascular perturbations. Here, we discuss cerebrovascular anomalies from an immunologic angle and fluid flow perspective. We describe immune contributions, both common and distinct, to the formation and progression of diverse cerebrovascular anomalies. Next, we summarize how cerebrovascular anomalies precipitate diverse neurological sequelae, including seizures, hydrocephalus, and cognitive effects and possible contributions through the recently identified lymphatic and glymphatic systems. Finally, we speculate on and provide testable hypotheses for novel nonsurgical therapeutic approaches for alleviating neurological impairments arising from cerebrovascular anomalies, with a particular emphasis on the normalization of fluid flow and alleviation of inflammation through manipulations of the lymphatic and glymphatic central nervous system clearance pathways.

Pial arteriovenous fistula resembling the vein of Galen aneurysmal malformation, treated with staged endovascular embolization: a case report.

A 41-month-old boy was presented to our hospital because of an intracranial mass suspected of cerebrovascular malformation. He was admitted and received cerebral angiography. The angiography result confirmed the intracranial mass was the dilated vein of Galen resulting from a pial arteriovenous fistula, which quite resembling the vein of Galen aneurysmal malformation. Considering one-time embolization of the fistula may greatly change the distribution of intracranial blood flow, we decided to perform staged embolization. In the first stage, we partially embolized the fistula, resulting in a sharp decrease in blood flow to the lesion. The second intervention was performed one month later, and completely embolized the fistula. The boy recoverd well and returned to normal childhood without any neurological deficits. Follow-up MR images obtained at 10 months after the last procedure showing total obliteration of the pAVF, gradually shrinking of the varix, and remodeling of the vein of Galen.

Angioarchitecture of the hemorrhagic developmental venous anomaly with stenosis of the collecting vein and cavernous malformation: a case report.

We herein report a case of developmental venous anomaly (DVA) with venous congestion caused by stenosis of the collecting vein that presented with intracerebral hemorrhage (ICH). A 74-year-old woman was referred to our hospital a few days after the onset of motor aphasia. Computed tomography (CT) and magnetic resonance imaging (MRI) showed ICH in the left frontal lobe. Angiography revealed DVA in the left frontal lobe in the late venous phase. Stenosis of the collecting vein of DVA at the entrance to the superior sagittal sinus was detected and accompanied by cavernous malformation (CM) beside DVA. Cone-beam CT revealed the absence of the left septal vein and hypoplastic transverse caudate veins. The patient was treated by blood pressure management and no additional neurological symptoms were detected. DVA develops to compensate for the absence of pial or deep venous systems, and generally benign and clinically asymptomatic. However, the outflow restriction of DVA causes chronic venous hypertension and the formation of CM. These abnormalities are considered to occur during post-natal life and may result in ICH. The risk of hemorrhage needs to be considered in cases of DVA with restricted venous outflow or CM.

Giant, symptomatic mixed vascular malformation containing a cavernoma, developmental venous anomaly, and capillary telangiectasia in a 19-month-old infant.

Intracranial mixed vascular malformations (MVMs) are defined as any combination of a developmental venous anomaly (DVA), cerebral cavernous malformation (CCM), capillary telangiectasia (CTG), or arteriovenous malformation (AVM) within a single, contiguous lesion. However, most MVMs described in the literature contain only 2 pathologically discrete malformations; juxtaposition of 3 or more abnormalities in a single lesion remains exceedingly rare. We present the case of a 19-month-old female with new onset focal seizures and a 4-cm right basal ganglia lesion initially believed to be an embryonal neoplasm. She subsequently underwent gross total resection (GTR) of the lesion via a transsylvian-transinsular approach. Intraoperatively, the lesion appeared to be heterogenous and highly vascular, with areas of purplish-gray friable tissue. Pathology confirmed the lesion to be a MVM containing a CCM, CTG, and a DVA. This appears to be the first reported case of such a lesion confirmed on pathology in the literature.

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

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. All of the cases underwent foetal neurosonography and echocardiography. The presence of ventriculomegaly, intracranial haemorrhage and cardiac failure was recorded. Pregnancy and neonatal outcome information were obtained from medical records. The mean gestational age at diagnosis was 31.1 ± 5.1 weeks, and the mean size of VGAM was 29.2 ± 5.2 × 26.4 ± 3.3 mm. Ventriculomegaly was detected in five of six (83.3%) cases. Intracranial haemorrhage was present in five (83.3%) cases. Cardiac failure was shown in four (66.6%) foetuses. Three foetuses underwent termination of pregnancy (TOP); in two cases, neonatal death occurred. One patient was treated with endovascular embolisation, and there was no cardiac problem or neurodevelopment delay. Prenatally diagnosed VGAM have a poor prognosis, mainly if a cardiac failure or neurological consequences (intracranial haemorrhage, hydrocephaly) are present in utero.Impact StatementWhat is already known on this subject? VGAM is the most common cerebral arteriovenous malformation detected prenatally, and it can lead to severe consequences in the perinatal period.What do the results of this study add? The accuracy of foetal neurosonography is excellent for detecting VGAM and associated brain abnormalities. Foetal echocardiography is mandatory for the prediction of prognosisWhat are the implications of these findings for clinical practice and/or further research? VGAM is associated with severe brain injury, cardiac failure, and the prognosis is generally poor. We need predictors to identify those expected to benefit from postnatal therapy.

The Frequency and Associated Factors of Asymmetrical Prominent Veins: A Predictor of Unfavorable Outcomes in Patients with Acute Ischemic Stroke.

Objectives: The present study is aimed at investigating the frequency and associated factors of asymmetrical prominent veins (APV) in patients with acute ischemic stroke (AIS). Methods: Consecutive patients with AIS admitted to the Comprehensive Stroke Center of Shanghai Fourth People's Hospital between January 2013 and December 2017 were enrolled. MRI including diffusion-weighted imaging (DWI), perfusion-weighted imaging (PWI), and susceptibility-weighted imaging (SWI) was performed within 12 hours of symptom onset. The volume of asymmetrical prominent veins (APV) was evaluated using the Signal Processing In nuclear magnetic resonance software (SPIN, Detroit, Michigan, USA). Multivariate analysis was used to assess relationships between APV findings and medical history, clinical variables as well as cardio-metabolic indices. Results: Seventy-six patients met the inclusion criteria. The frequency of APV ≥ 10 mL was 46.05% (35/76). Multivariate analyses showed that proximal artery stenosis or occlusion (≥50%) (P < 0.001, adjusted odds ratio (OR) = 660.0, 95%CI = 57.28-7604.88) and history of atrial fibrillation (P < 0.001, adjusted OR = 10.48, 95%CI = 1.78-61.68) were independent factors associated with high APV (≥10 mL). Conclusion: Our findings suggest that the frequency of APV ≥ 10 mL is high in patients with AIS within 12 hours of symptom onset. History of atrial fibrillation and severe proximal artery stenosis or occlusion are strong predictors of high APV as calculated by SPIN on the SWI map.

Recurrent Transient Neurological Deficit Due to Intracerebral Steal Phenomenon in Association with a Developmental Venous Anomaly.

We report a symptomatic developmental venous anomaly (DVA) not causing parenchymal abnormality to provide a pathophysiologic clue in patients with recurrent transient neurologic deficit. A 32-year-old male presented with recurrent transient motor aphasia and headache in the left fronto-temporal region for three years. The symptoms usually lasted for one hour. Brain computed tomography (CT) angiography and magnetic resonance imaging using gradient recalled echo showed a prominent penetrating vein at the left frontal periventricular region. Brain CT perfusion imaging performed during the symptoms revealed increased perfusion in the corresponding area with relatively decreased perfusion in the left fronto-temporal cortices. Digital subtraction angiography revealed collecting venous blood from the left septal and thalamostriate veins draining into the left cavernous sinus without early arteriovenous shunting. In this patient, an inciting incident might have led to imbalance of the venous flow surrounding the DVA, causing venous hypertension and the intracerebral steal phenomenon in the surrounding area. The relatively hypoperfused cortical area adjacent to the DVA could be considered the cause of the transient motor aphasia, while venous hypertension could be the cause of the headache.

Changes in Angioarchitecture After Stereotactic Radiosurgery for Dural Arteriovenous Fistula.

INTRODUCTION: Dural arteriovenous fistulae (DAVF) are intracranial vascular abnormalities encountered in neurosurgery practice. Treatment options are microsurgical disconnection, endovascular embolization and/or radiosurgery. Past studies have reported the efficacy, safety, and predictors of success of radiosurgery. In this study, we investigated the angioarchitecture of fistulae at the time of radiosurgery and how the anatomy changed in the time after treatment based on angiogram follow-ups. METHODS: A retrospective analysis was performed on patients with angiographic diagnosis of DAVF treated with Gamma Knife radiosurgery (GKRS) between 2013 and 2018. Data collection included demographics, symptoms, grading scores, vascular anatomy, radiation data, treatment strategy, angiographic results, and length of patient follow-up. RESULTS: Our study reports data on 10 patients with a total of 14 fistulae. On follow-up angiography, 8 (57%) had complete occlusion of the fistula with a median time to follow up of 19.5 months. The remaining 6 (43%) were deemed as near-complete occlusion of fistula with a median time to follow up of 12.0 months. Time from radiosurgery to angiogram revealing incomplete vs. angiogram revealing complete obliteration was significantly different (p=0.045). Nearly all AVFs had decreased feeders over time after treatment with only one AVF developing an additional feeder post-treatment. Arterial feeders, drainage site, sex, Borden type, lesion volume and treatment volume had no predictive value of obliteration outcome. CONCLUSIONS: This study provides data on the angioarchitecture of fistulae treated with GKRS and also serves as an extension of previous studies reporting the safety and efficacy of GKRS treatment for DAVF in a specific patient population.

De Novo Dural Arteriovenous Fistula on Draining Veins of Previously Treated Pial Arteriovenous Malformation: a Case Report.

A 71-year-old man, with a pial micro-arteriovenous malformation (pAVM) draining into the confluence of the vein of Trolard and the vein of Labbé was surgically removed, sparing these cortical veins. 4-months MR and angiographic controls showed a de novo dural arteriovenous fistula (dAVF) draining into the previously spared cortical veins. It was removed using intraoperative motor evoked potentials (MEP). This is the first case of iatrogenic dAVF developing on the same draining vein of a previously treated pAVM. De novo dAVFs are generally iatrogenic. This case suggests that the unresected venous drainage of an AVM might be the substratum for neo-angiogenetic processes; moreover inflammation related to surgery might be the trigger factor for the development of the dAVF.

Association between draining vein diameters and intracranial arteriovenous malformation hemorrhage: a multicentric retrospective study.

PURPOSE: Intracranial arteriovenous malformations (AVMs) cause hemorrhage, and the role of draining vein diameters in rupture risk is controversial. The aims of the present study were to investigate the variables related with intracranial AVM rupture and to examine the association of draining vein diameters and AVM hemorrhage. METHODS: Two hundred three patients were included in this study, of which 117 (57%) had unruptured AVMs, and 86 (43%) had ruptured AVMs. RESULTS: In an adjusted (multivariate) analysis, the variables significantly associated with AVM hemorrhagic presentation were age (OR per year increase 0.97, 95%CI 0.95-0.99, p = 0.007), a deep nidus compared with superficial nidus (OR 3.21, 95%CI 1.13-9.06, p = 0.028), the nidus diameter (OR per each mm increase 0.95, 95%CI 0.92-0.97, p < 0.001), a single draining vein compared with multiple draining veins (OR 2.14, 95%CI 1.02-4.50, p = 0.044), the draining vein diameter (OR per mm increase 1.52, 95%CI 1.26-1.83, p < 0.001), and a draining vein diameter ≥ 5 mm compared with < 5 mm (OR 5.80, 95%CI 2.70-12.47, p < 0.001). CONCLUSION: In this study, after adjusted analysis, the variables associated with intracranial AVM hemorrhagic presentation were a young age, a small nidus diameter, a deeply located nidus, a single draining vein, and large draining vein diameters. A draining vein diameter cutoff ≥ 5 mm was positively associated with the risk of AVM rupture. A large and prospective study is now necessary to confirm if draining vein diameter is a risk factor for AVM hemorrhage.

Endovascular treatment of intracranial vascular malformations in children.

Paediatric intracranial vascular malformations are rare and different from adult ones in vascular anatomy, pathophysiology, and symptoms. Their impact on the brain and their symptoms will differ in the antenatal period, in neonates, infants, and children. Clinical presentation includes seizures, focal neurological deficit, haemorrhage, congestive heart failure, hydrovenous disorder, and developmental delays. These malformations are thus associated with a poor prognosis if left untreated. Therefore, aggressive management is generally recommended and must be performed by a multidisciplinary team with extensive experience. Endovascular treatment is the first-choice treatment for most paediatric intracranial vascular malformations. Indication and timing for treatment should be decided on the basis of a careful assessment of neurological symptoms, growth and development, cardiac and other systemic manifestations, and imaging of the malformation and the brain tissue. WHAT THIS PAPER ADDS: Paediatric intracranial vascular malformations are rare, but their prognosis is poor if left untreated. Improved clinical, anatomical, and pathophysiological understanding of these complex lesions has improved prognosis.

Cerebral Cavernous Malformations, Developmental Venous Anomaly, and Its Coexistence: A Review.

BACKGROUND: Cerebral cavernous malformations (CCMs) are intracranial vascular malformations that can exist as a single lesion or mixed vascular lesions. The most common mixed form is the coexistence of CCM with an associated developmental venous anomaly (DVA). In this paper, we aim to give a comprehensive review of CCM, DVA, and their coexistence as mixed lesions. A PubMed search using the keywords "Cerebral cavernous malformations, Developmental venous anomaly, Mixed Cerebral cavernous malformations with Developmental venous anomaly" was done. All studies in the English language in the past 10 years were analyzed descriptively for this review. SUMMARY: The search yielded 1,249 results for "Cerebral cavernous malformations," 271 results for "Developmental venous anomaly," and 5 results for "Mixed Cerebral cavernous malformations with Developmental venous anomaly." DVA is the most common intracranial vascular malformation, followed by CCM. CCM can have a wide array of clinical presentations like hemorrhage, seizures, or focal neurological deficits or can also be an incidental finding on brain imaging. DVAs are benign lesions by nature; however, venous infarction can occur in a few patients due to acute thrombosis. Mixed CCM with DVA has a higher risk of hemorrhage. CCMs are angiographically occult lesion, and cerebral digital subtraction angiography is the gold standard for the diagnosis of DVA. Mixed lesions, on the other hand, are best diagnosed with magnetic resonance imaging, which has also been effective in detecting specific abnormalities. Asymptomatic lesions are treated through a conservative approach, while clinically symptomatic lesions need surgical management. CONCLUSION: Individual CCM or DVA lesions have a benign course; however, when they coexist in the same individual, the hemorrhagic risk is increased, which prompts for rapid diagnosis and treatment.

Persistent falcine sinus with temporo-occipital schizencephaly: case report with a review of literature in relation to the undeveloped vein of Galen and/or straight sinus.

Falcine sinus is a normal midline embryonic venous sinus present in the fetal brain and usually disappears by birth. Persistent falcine sinus (PFS) has been reported as a normal variant or along with vein of Galen (VOG) malformation, encephalocele, and other abnormalities. Schizencephaly, either closed or open type, has been reported with other associated vascular anomalies. We report a 22-month-old child, who presented with delayed milestones and referred for magnetic resonance (MR) imaging, and the child was found to have PFS with associated bilateral temporo-occipital closed-lip schizencephaly, hippocampal abnormalities, falco-tentorial dehiscence, and white matter abnormalities. The vein of Galen and straight sinus were absent, and the internal cerebral veins were seen draining into superior sagittal sinus via the falcine sinus. These set of abnormalities are unique from abnormalities reported previously in association with the falcine sinus.

Evaluation of sporadic intracranial cavernous malformations for detecting associated developmental venous anomalies: added diagnostic value of C-arm contrast-enhanced cone-beam CT to routine contrast-enhanced MRI.

OBJECTIVE: Our purpose was to investigate the added diagnostic value of C-arm contrast-enhanced cone-beam CT (CE-CBCT) to routine contrast-enhanced MRI (CE-MRI) in detecting associated developmental venous anomalies (DVAs) in patients with sporadic intracranial cavernous malformations (ICMs). METHODS: Fifty-six patients (53 with single and three with double ICMs) met the inclusion criteria. All patients had routine CE-MRI scans performed at 1.5 Tesla. The imaging studies (CE-MRI and CE-CBCT) were retrospectively and independently reviewed by two observers, with consensus by a third. Group difference, intra- and interobserver agreement, and diagnostic performance of the modalities in detecting associated DVAs were calculated. Reference standard was CE-MRI. RESULTS: On CE-MRI and CE-CBCT, 37 (66%; of 56) and 47 patients (84%; of 56) had associated DVAs, respectively. In 10 patients (52.6%; of CE-MRI negatives [n=19]), CE-CBCT improved the diagnosis. Nine patients (16%; of 56) had no DVA on both imaging techniques. Difference in proportions of associated DVAs on CE-MRI and CE-CBCT was statistically significant, p < 0.05. Sensitivity, specificity, positive likelihood ratio, and area under the curve of CE-CBCT were 100% (95% confidence interval [CI]: 90.5-100%), 47.3% (95% CI: 24.4-71.1%), 1.9 (95%CI: 1.240-2.911), 0.737 (95%CI: 0.602-0.845), respectively. Intraobserver agreement was excellent for CE-MRI, kappa (κ) coefficient = 0.960, and CE-CBCT, κ=0.931. Interobserver agreement was substantial for CE-MRI, κ=0.803, and excellent for CE-CBCT, κ=0.810. CONCLUSIONS: CE-CBCT is a useful imaging technique especially in patients with negative routine CE-MRI in terms of detecting associated DVAs. In nearly half of these particular patients, it reveals an associated DVA as a new diagnosis. KEY POINTS: • Although it is known to be the gold standard, some of the DVAs associated with ICMs are underdiagnosed with CE-MRI. • In nearly half of the patients with negative routine CE-MRI, CE-CBCT reveals an associated DVA as a new diagnosis. • Intra- and interobserver agreement on CE-CBCT is excellent in terms of detecting associated DVAs.

Clinical Course and Management of Vein of Galen Varix of the Neonate: A Case Report and Literature Review.

A 28-year-old pregnant woman underwent an emergency caesarian section after 39 weeks of gestation because of decreased fetal movement and baseline fetal heart rate variability. The neonate was diagnosed with neonatal asphyxia and presented with right cardiac failure due to pulmonary hypertension. The neonate presented convulsion, and plane computed tomography (CT) showed dilation of the vein of Galen and sinuses on day 3. Magnetic resonance imaging and CT with contrast were performed after cardiac failure subsided, and there was no evidence of arteriovenous shunt and normalization of the vein of Galen. The patient was diagnosed with vein of Galen varix (VGV). In the previous literature, only 3 cases of VGV have been reported. VGV is considered to be caused by right cardiac failure without the presence of an arteriovenous shunt and requires treatment only for cardiac failure and its cause. Therefore, it is important to differentiate VGV from vein of Galen aneurysmal malformation.