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Objectives: Posterior fossa anomalies (PFAs) are associated with a wide spectrum of neurodevelopmental disabilities, with presentation ranging from no obvious clinical symptoms to severe neurodevelopmental delay. The differential diagnosis of fetal PFAs using imaging is crucial for prenatal counseling and prognostic evaluation. Imaging of the tentorium cerebelli (TC) is critical for the differential diagnosis of fetal PFAs; however, achieving this using prenatal grayscale ultrasound is challenging. This study aimed to establish a reference range for a new measurement, the angle between the vein of Galen and the straight sinus (AVGS), measured using microvascular flow imaging, and to evaluate prospectively the effectiveness of AVGS for assessment of the position of the fetal TC. Methods: This was a single‐center prospective validation study including singleton pregnancies examined between 16 and 38 gestational weeks at Beijing Obstetrics and Gynecology Hospital, Capital Medical University, Beijing Maternal and Child Health Care Hospital, between January 2022 and July 2023. AVGS was measured in normal fetuses and used to establish a reference range. AVGS was then used to predict prospectively the position of the TC in 50 fetuses with one or more intracranial malformations, using cut‐offs of ≤ 5th centile or ≥ 95th centile to define abnormal AVGS. All intracranial malformations and the position of the TC in these fetuses were confirmed using fetal brain magnetic resonance imaging. The sensitivity, specificity, positive and negative predictive values and likelihood ratios were calculated to assess the performance of AVGS in the prediction of abnormal position of the fetal TC. Results: The study group comprised 602 singleton pregnancies, including 522 normal fetuses and 50 fetuses with an intracranial anomaly. A reference range for fetal AVGS was established. Fetal AVGS decreased with advancing gestational age. Ten of the 50 fetuses with an intracranial anomaly had an abnormally positioned TC. The sensitivity and specificity of AVGS for the prediction of abnormal position of the TC in fetuses with an intracranial malformation were 90.0% (95% CI, 71.4–100.0%) and 95.0% (95% CI, 88.2–100.0%), respectively. The positive and negative predictive values were 81.8% (95% CI, 47.8–96.8%) and 97.4% (95% CI, 84.9–99.9%), respectively, and the positive and negative likelihood ratios were 18.000 (95% CI, 4.590–70.592) and 0.105 (95% CI, 0.016–0.677), respectively. Conclusions: AVGS is a new and useful marker for the prenatal evaluation of fetal TC position. Increased AVGS (≥ 95th centile) suggests an abnormally elevated position of the TC, while decreased AVGS (≤ 5th centile) suggests an abnormally low TC. AVGS is helpful for differential diagnosis in fetuses with PFA and can inform appropriate prenatal counseling. © 2024 International Society of Ultrasound in Obstetrics and Gynecology. [ABSTRACT FROM AUTHOR]

Vein of Galen malformation (VGM) is a rare congenital, uncommon intracerebral vascular anomaly rarely complicated with the development of brain abscess as secondary to primary infection or after endovascular treatment. We report a very rare finding of a vein of Galen aneurysm associated with a large brain abscess at the time of diagnosis. A 12-year-old boy with a high-grade fever, severe headache, and recurrent episodes of convulsions came into the radiology department of Kassala Advanced Diagnostic Center. On a Siemens 16-slice scanner, brain non-contrast enhanced computed tomography (NECT) and contrast enhanced CT (CECT) was used to determine the source of the acute headache and convulsions which revealed a right frontal peripherally enhancing cystic lesion measuring 5.7 × 4.7 × 5.3 cm2 surrounded by massive vasogenic edema causing mass effect with midline shift to the left side by 1.5 cm suggestive of brain abscess. There is evidence of another avidly enhancing lesion seen within the third ventricle continuous with a straight sinus surrounded by extensive vascular loops consistent with an aneurysm of the vein of Galen, it was causing compression of the cerebral aqueduct with upstream mild hydrocephalus with dilated both lateral ventricles. Late presentation, diagnosis, and treatment also lead to an increase in the morbidities and mortalities of such case conditions. Urgent intervention should be considered for better outcomes.

Paediatric neurovascular anomalies associated with the vein of Galen (VG) comprise of a spectrum of rare, complex, and life-threatening conditions. In this group, the "vein of Galen aneurysmal dilatation" (VGAD) is a distinct entity that often presents with progressive neurological symptoms in older children. Acute haemorrhage in VGAD is uncommon. We present an unusual presentation of VGAD in a neonate and discuss the challenges faced in the management. [ABSTRACT FROM AUTHOR]

Abstract Background Vein of Galen aneurysmal malformation (VGAM) is considered a rare vascular malformation, constituting over 1% of all arteriovenous shunts. This malformation represents around 30% of congenital vascular malformations in the pediatric population. The focus of our study is to examine both the clinical and radiological outcomes following endovascular embolization in pediatric patients with Galenic arteriovenous shunts. Our aim is to evaluate the safety and efficacy of endovascular management. This research aims to contribute to understanding the management and outcomes of VGAM in pediatric patients, particularly in the context of endovascular embolization. Method This study included 19 patients with vein of Galen malformation evaluated clinically and radiologically and we treated them with endovascular embolization with aim to assess safety and efficacy of endovascular treatment of VGA in the period from May 2019 to December 2022. Results We evaluated 12 male patients 63.2% and 7 females 36.8% in pediatric age group with vein of Galen malformation. Patients were followed for 1 year. Our study included 3 neonates (15.8%), 10 infants (52.6%) and 6 children (2–10 years) (31.6%). Increase SC (skull circumference) was the most common presenting symptoms 8 (42.1%). We have reached total occlusion in 15 patients (78.9%) while 4 patients had residual (21.1%). 11 patients improved (57.9%) and 2 patients died (10.5%). Conclusion VGAM are not very rare and should be kept in mind in children with refractory heart failure. Introduction of endovascular embolization as the primary therapy has significantly improved prognosis. Good selection of cases based on their score and good timing of treatment has impact on prognosis with less morbidities.

Vein of Galen aneurysmal malformation (VGAM) is a rare congenital malformation characterised by arteriovenous fistulas between primitive choroidal arteries and the median prosencephalic vein, the embryonic precursor to the vein of Galen. Endovascular techniques have changed the management of these patients with improved prognosis. An eight-month-old with VGAM managed by endovascular embolisation using ethylene vinyl alcohol copolymer (EVOH) developed a chemical abscess - a rare complication. It was managed conservatively and showed promising clinical outcome. Contribution: Chemical abscesses following EVOH embolisation are scarce – with imaging differentials, which include brain abscess and onyx granuloma. Knowledge and successful identification of this entity are essential as its management as prognoses differ. Chemical abscess is managed conservatively and has a good prognosis.

Abstract Background Falcotentorial meningiomas are rare tumors that arise from the junction of the falx cerebri and the tentorium. Accurate preoperative imaging plays a crucial role in surgical planning and management. Magnetic resonance imaging (MRI) is the preferred imaging modality for meningiomas. In this report, we present a case of type 1 falcotentorial meningioma and discuss the role of preoperative imaging and management. Case presentation A 62-year-old female patient presented with gradually progressive bilateral lower limb weakness. Contrast-enhanced MRI showed a circumscribed lobulated extraaxial lesion near the falcotentorial junction with an adjacent dural tail, consistent with a type 1 falcotentorial meningioma. Based on these radiological features, the patient was diagnosed with a type 1 falcotentorial meningioma. The patient was referred for neurosurgical evaluation for further management. Conclusion Falcotentorial meningiomas are rare tumors that require careful preoperative imaging and surgical planning. Radiological reporting should include the relationship of the tumor to the vein of Galen to aid neurosurgical approach. Complete surgical resection is the preferred therapy, but subtotal resection can be considered in certain cases.

Background: Falcotentorial meningiomas are rare tumors that arise from the junction of the falx cerebri and the tentorium. Accurate preoperative imaging plays a crucial role in surgical planning and management. Magnetic resonance imaging (MRI) is the preferred imaging modality for meningiomas. In this report, we present a case of type 1 falcotentorial meningioma and discuss the role of preoperative imaging and management. Case presentation: A 62-year-old female patient presented with gradually progressive bilateral lower limb weakness. Contrast-enhanced MRI showed a circumscribed lobulated extraaxial lesion near the falcotentorial junction with an adjacent dural tail, consistent with a type 1 falcotentorial meningioma. Based on these radiological features, the patient was diagnosed with a type 1 falcotentorial meningioma. The patient was referred for neurosurgical evaluation for further management. Conclusion: Falcotentorial meningiomas are rare tumors that require careful preoperative imaging and surgical planning. Radiological reporting should include the relationship of the tumor to the vein of Galen to aid neurosurgical approach. Complete surgical resection is the preferred therapy, but subtotal resection can be considered in certain cases. [ABSTRACT FROM AUTHOR]

The falcine sinus is a normal embryonic structure that is situated between the 2 layers of the falx cerebri and drains the deep cerebral venous system into the superior sagittal sinus. It normally involutes after birth and is uncommon in adults. Although it is often an isolated and incidental finding, it can also be associated with a number of other conditions including but not limited to vein of Galen arterial malformations (VGAM), atretic parietal cephaloceles, acrocephalosyndactyly (Apert syndrome), absence of the corpus callosum, absence of the tentorium, osteogenesis imperfecta, or Chiari II malformations. We present a case series of 3 pediatric patients born with a persistent falcine sinus and an associated condition, including a VGAM, an APC, and a sinus thrombosis. The purpose of this article is to highlight the importance of understanding anatomic variations in the cerebral venous system to help aid in the proper diagnosis and treatment of associated pathologies.

Introduction: A vein of Galen aneurysmal malformation (VGAM) is a high-flow arteriovenous malformation that typically presents in early childhood. Complications associated with this condition include heart failure and hydrodynamic disorders resulting from high blood flow. Hydrocephalus is one of the hydrodynamic disorders. It can present in a chronic form due to a defect in the absorption of cerebrospinal fluid (CSF) resulting from venous hypertension or in an acute/subacute form due to obstruction of normal circulation routes caused by the mass effect of the VGAM. Currently, endovascular closure of the lesion is considered the treatment of choice. However, in acute/subacute presentations of obstructive hydrocephalus, progression may occur despite endovascular treatment, necessitating a cerebrospinal fluid diversion procedure. Prior to the advent of endoscopic treatment, ventricular shunts were utilized, but outcomes were poor due to the pathological hydrodynamic parameters associated with the malformation. The role of endoscopic third ventriculostomy as a treatment option in these situations remains unclear, and there is limited literature available. Therefore, we present the case of a patient with a vein of Galen aneurysmal malformation and obstructive hydrocephalus, which was treated via ETV. Furthermore, a literature review was conducted. Case description: We present a 5-month-old infant who was diagnosed at birth with VGAM. At 2 months of age, a partial closure of the malformation was performed via endovascular. Subsequently, the patient presented at the emergency department with symptoms of irritability, lethargy, vomiting, feeding refusal, and "setting sun" sign of several days' duration due to obstructive hydrocephalus. The patient was successfully treated via ETV. Following this, a new arteriography was performed and revealed occlusion of the VGAM. The child clinically improved and currently exhibits mild psychomotor impairment after 1-year follow-up. Conclusion: In the literature, there are a limited number of reports on the treatment of obstructive hydrocephalus associated with VGAM via ETV. These reports have demonstrated good outcomes with minimal surgical complications. Our patient also exhibited good results. As such, ETV is a viable and safe option for the treatment of obstructive hydrocephalus associated with VGAM in pediatric patients. However, more research is needed to establish the effectiveness of ETV in comparison to other treatment options and to understand the long-term outcomes. [ABSTRACT FROM AUTHOR]

Background: At present, there is a lack of normal magnetic resonance imaging (MRI) morphometric reference values for fetal internal jugular veins during middle and late pregnancy. Objective: We used MRI to assess the morphology and cross-sectional area of the internal jugular veins of fetuses during middle and late pregnancy and to explore the clinical value of these parameters. Materials and methods: The MRI images of 126 fetuses in middle and late pregnancy were retrospectively analysed to determine the optimal sequence for imaging the internal jugular veins. Morphological observation of the fetal internal jugular veins in each gestational week was carried out, lumen cross-sectional area was measured and the relationship between these data and gestational age was analysed. Results: The balanced steady-state free precession sequence was superior to other MRI sequences used for fetal imaging. The cross section of fetal internal jugular veins was predominantly circular in both the middle and late stages of pregnancy, however the prevalence of an oval cross section was significantly higher in the late gestational age group. The cross-sectional area of the lumen of the fetal internal jugular veins increased with increasing gestational age. Fetal jugular vein asymmetry was common, with the right jugular vein being dominant in the high gestational age group. Conclusion: We provide normal reference values for fetal internal jugular veins measured by MRI. These values may form the basis for clinical assessment of abnormal dilation or stenosis. [ABSTRACT FROM AUTHOR]

Introduction: Galenic dural arteriovenous fistulas (dAVFs) are a rare form of dAVF and rarely described in the literature. Their distinct location requires different surgical approaches than dAVFs occurring at the nearby sites of the straight sinus and torcular Herophili, and their high risk of hemorrhage makes these dAVFs very challenging to approach surgically. In this report, we present a unique case of Galenic dAVF. Case description: The patient is a 54-year-old female who presented with a 2-year history of progressive headaches, cognitive decline, and papilledema. A cerebral angiogram demonstrated a complex dAVF to the vein of Galen (VoG). She underwent transarterial embolization with Onyx-18 which resulted in minimal reduction in arterial venous shunting. She subsequently underwent a successful transvenous coil embolization resulting in complete occlusion of dAVF. The patient's postoperative course was complicated by interventricular hemorrhage; however, she had a remarkable clinical recovery with resolution of headaches and improvement in cognitive function. A follow-up angiogram completed 6 months post-embolization demonstrated very mild residual shunting. Conclusion: In the unique case presented here, we demonstrate the efficacy of transvenous embolization via an occluded straight sinus as an alternative therapeutic option to eliminate cortical venous reflux. [ABSTRACT FROM AUTHOR]

Background: 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. Rapid ventricular pacing (RVP) has been proposed as an alternative strategy with fewer drawbacks, but has not been well studied. The approach involves catheterizing the right ventricle with a pacing catheter connected to a temporary external pacemaker. Prior to embolization, RVP is initiated to lower cardiac output. Following embolization, pacing is discontinued, and the heart returns to sinus rhythm. Methods: We performed RVP in five mVOGM patients from 4/2020 through 7/2021. Accounting for multiple procedures, RVP was utilized in ten cases and twenty-six pedicles. Results: Ventricular capture was achieved in all instances and was well tolerated, without arrhythmia. Casting the arterial pedicle with liquid embolic immediately adjacent to, or traversing, the fistulous point was achieved in 9/10 cases. There were no procedural complications. In 1 case, creation of a coil basket in the venous pouch was required to achieve a stable arterial cast Conclusions: This report describes the largest case series utilizing RVP in mVOGM. The technique appears safe and well tolerated. [ABSTRACT FROM AUTHOR]

Perimesencephalic subarachnoid hemorrhage (PMSAH) is a group of diseases characterized by bleeding around the brainstem. In recent years, it has been suggested that PMSAH is associated with the venous system. We report a case of PMSAH caused by stenosis of the junction of the vein of Galen (VG) and the rectus sinus (RS). A 39-year-old man presented with restlessness at work. He was administered diazepam, and a few minutes later, his consciousness became clear. Imaging showed subarachnoid hemorrhage (SAH) around the right midbrain, occlusion of the right sigmoid sinus and the right transverse sinus, stenosis of the junction of the VG and RS, T2 hyperintensity in the right middle temporal gyrus, and obstruction of the right vein of Labbe. The location of the SAH coincided with stenosis of the junction of the VG and RS. PMSAH was diagnosed due to the increase in intracranial venous pressure caused by the patient’s sinus obstruction in addition to the stenosis of the junction of the VG and RS. It is necessary to pay attention to venous return when PMSAH is observed.

Vein of Galen aneurysmal malformations (VGAM) are arteriovenous fistulas between the choroidal or quadrigeminal arteries and the embryonic precursor of the vein of Galen – the median prosencephalic vein. VGAM represents 30% of arteriovenous malformations in the paediatric age group. They are associated with a high morbidity and 90% mortality if untreated but respond well to intervention with mortality reduced to below 40%. There are few documented cases of VGAM worldwide and scarce data about these lesions in sub-Saharan Africa with challenges in diagnosis and management. We present two cases of infants diagnosed with VGAM and treated with endovascular embolisation. We examine the clinical and radiological data at presentation, intervention and follow-up at one year. We report on some of the technical neuro-interventional details. Both patients had satisfactory radiological results and the interventions resulted notable clinical improvements from baseline. Physicians should maintain a high index of suspicion treating infants and toddlers presenting with macrocephaly, neurocognitive decline and cardiopulmonary symptoms. Upon diagnosis endovascular interventions are feasible and available.

IntroductionGalenic dural arteriovenous fistulas (dAVFs) are a rare form of dAVF and rarely described in the literature. Their distinct location requires different surgical approaches than dAVFs occurring at the nearby sites of the straight sinus and torcular Herophili, and their high risk of hemorrhage makes these dAVFs very challenging to approach surgically. In this report, we present a unique case of Galenic dAVF.Case descriptionThe patient is a 54-year-old female who presented with a 2-year history of progressive headaches, cognitive decline, and papilledema. A cerebral angiogram demonstrated a complex dAVF to the vein of Galen (VoG). She underwent transarterial embolization with Onyx-18 which resulted in minimal reduction in arterial venous shunting. She subsequently underwent a successful transvenous coil embolization resulting in complete occlusion of dAVF. The patient’s postoperative course was complicated by interventricular hemorrhage; however, she had a remarkable clinical recovery with resolution of headaches and improvement in cognitive function. A follow-up angiogram completed 6 months post-embolization demonstrated very mild residual shunting.ConclusionIn the unique case presented here, we demonstrate the efficacy of transvenous embolization via an occluded straight sinus as an alternative therapeutic option to eliminate cortical venous reflux.

Introduction: vein of Galen aneurysmal malformation (VOGM) is a low-frequency congenital anomaly, but with high lethality. Objective: we present a neonate with VOGM type II. Clinical case: male newborn with prenatal diagnosis by fetal sonography of VOGM. He was born at 37 weeks of gestation. He suffered intraventricular hemorrhage and hydrocephalus in the neonatal period. At two months of age, endovascular embolization was performed; However, he died at five months of age due to thrombosis of the superior longitudinal sinus, massive cerebral hemorrhage, and intracranial hypertension. Conclusions: patients with VOGM have a high risk of dying; early detection may improve their prognosis. [ABSTRACT FROM AUTHOR]

Objectives: To report the outcome of fetuses with a prenatal diagnosis of Vein of Galen (VOG) malformation. Methods: PubMed and Embase databases were searched. Random effect meta-analysis of proportions was used to analyze the data. The outcomes explored were prenatal ultrasound findings, mortality, preterm birth (PTB), abnormal neurological outcome, associated findings detected at post-natal brain imaging, need for anticonvulsant therapy, and the rate of children free from neurological impairment. Random effect meta-analysis of proportions were used to analyze the data. Results: Eleven studies (226 fetuses with a prenatal diagnosis of VOG malformation) were included. All cases were detected during the third trimester of pregnancy. Ventriculomegaly was detected in 31.8% (95% CI 27.6–47.7), cardiomegaly or other ultrasound signs of cardiac compromise in 23.1% (95% CI 14.9–32.5) and hydrops in 7.3% (95% CI 2.8–13.6) of cases. The incidence of IUD, NND, and PND was 1.5% (95% CI 0.2–4.3), 23.8% (95% CI 16.9–31.4), and 24.5% (95% CI 17.6–32.2), respectively, while 12.6% (95% CI 6.0–21.2) of pregnancies were complicated by PTB. Abnormal neurodevelopmental outcome was observed in 36.7% (95% CI 27.9–39.7) of cases, while 60.5% (95% CI 17.0–82.0) of children had abnormal findings on post-natal imaging and only 29.7% (95% CI 23.3–36.5) were free from neurological impairment after birth, although there was a wide heterogeneity in the time at follow-up between the included studies. Conclusion: VOG malformation diagnoses during fetal life is associated with a high incidence of brain damage, cardiac compromise, and abnormal neurodevelopmental outcome after birth. [ABSTRACT FROM AUTHOR]

In some vein of galen aneurysmal malformation (VGAM) patients, transvenous embolization (TVE) is an attractive option, but its safety is unclear. Here we report the first two VGAM patients treated using the Chapot "pressure cooker" technique (ChPC). 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. In this technique, a guiding catheter is placed transjugular into the straight sinus (SS). One or two detachable tip microcatheters are advanced to the origin of the SS. Another microcatheter is advanced and the tip placed between the distal marker and the detachment zone of the former. Coils and n-BCA are used to prevent reflux of Onyx. Conclusions: In this study, we recognized two important factors of traditional VGAM treatment that may cause interventionalists to consider the ChPC to treat VGAM: (1) without liquid embolic, deployed coils may not occlude the fistula entirely. (2) There is the concern of causing delayed bleeding should the arterial component of the fistula rupture. ChPC ameliorates these issues by offering complete closure of the fistula with liquid embolic material in TVE. [ABSTRACT FROM AUTHOR]

We present a dissection of the patent ductus arteriosus and pulmonary artery for surgical repair utilising cardiopulmonary bypass in the setting of vein of Galen malformation. Several strategies were employed to attenuate the cerebral shunt including pH-stat, high cardiac index, restrictive venous drainage, continuous ventilation and deep hypothermic circulatory arrest. The patient recovered from surgery with no apparent neurological sequelae. [ABSTRACT FROM AUTHOR]

Pediatric arteriovenous shunts can be challenging to embolize and the consequences of excess venous penetration disastrous. Moreover, the congenital nature of most shunts necessitates neonatal intervention whenever (medically refractory) heart failure occurs. Here, we describe current endovascular treatment strategies based on personal experience and literature review. While disparate, arteriovenous malformation, dural arteriovenous fistula (AVF), pial AVF, and vein of Galen aneurysmal malformation share unifying features such as high output heart failure and venous hypertension. This tutorial is divided into passages on Goals, Access, and Treatment which respect differences among pediatric arteriovenous shunts while maintaining a narrative economy. [ABSTRACT FROM AUTHOR]

Perimesencephalic subarachnoid hemorrhage (PMSAH) is a group of diseases characterized by bleeding around the brainstem. In recent years, it has been suggested that PMSAH is associated with the venous system. We report a case of PMSAH caused by stenosis of the junction of the vein of Galen (VG) and the rectus sinus (RS). A 39-year-old man presented with restlessness at work. He was administered diazepam, and a few minutes later, his consciousness became clear. Imaging showed subarachnoid hemorrhage (SAH) around the right midbrain, occlusion of the right sigmoid sinus and the right transverse sinus, stenosis of the junction of the VG and RS, T2 hyperintensity in the right middle temporal gyrus, and obstruction of the right vein of Labbe. The location of the SAH coincided with stenosis of the junction of the VG and RS. PMSAH was diagnosed due to the increase in intracranial venous pressure caused by the patient's sinus obstruction in addition to the stenosis of the junction of the VG and RS. It is necessary to pay attention to venous return when PMSAH is observed. [ABSTRACT FROM AUTHOR]

Straight sinus agenesis is a rare congenital anatomical variation that is usually accompanied by persistent falcine sinus. Concomitance of straight sinus agenesis and absence of falcine sinus are associated with thalamic arteriovenous malformation by the disturbance of the venous flow. In this report, a very rare case of aneurysmal dilatation of the vein of Galen associated with thalamic arteriovenous malformation and straight sinus agenesis is presented. [ABSTRACT FROM AUTHOR]

This paper aims to make simple the evaluation of the main veins related to the brainstem and cerebellum. Posterior fossa venous drainage is best understood in context with its three main collectors: superior: toward the Vein of Galen; posterior: toward the torcular complex; and anterior: toward the superior petrosal sinus. A fourth possible drainage path, often harder to distinguish, is directed toward the inferior petrosal sinus. Veins of these four systems are frequently connected to one another. Despite traditionally being considered less regular than its arterial disposition, posterior fossa venous anatomy follows specific patterns that are easy to identify. The three more representative veins of each venous confluent have been selected, to help in recognizing them angiographically. Since pial large veins are primarily located over the surface of the encephalon, most related anatomical structures can be confidently identified. This is of special interest when angiographic 2D or 3D studies are evaluated and provide fundamental assistance in locating precise structures. To better aid in understanding venous disposition, an overview of embryologic and fetal development is also discussed. [ABSTRACT FROM AUTHOR]

Background: The occipital transtentorial (OT) approach is well-established approach for pineal region tumors and can be of choice for the lesions located around the suboccipital part of tentorium such as the quadrigeminal plate, posterior part of thalamus, tentorial surface of cerebellum, splenial region, posterior falx, and lesions around the tentorial incisura. However, it is not very much extensively used in the above-mentioned locations other than the pineal region. Methods: Thirty-one patients of pineal region lesions were operated by OT approach, the role of conventional preoperative evaluation of the anatomy of the venous sinuses, deep venous system, and tentorial angle was investigated. Results: A variety of lesions were operated using this approach achieving gross and near total resection in majority of the cases (76.6%), with acceptable postoperative mean modified Rankin scales (1.8). Conclusion: The OT is a preferable approach for pineal region lesions for patients of all ages and can be tailored for achieving high resectability rates irrespective of the status of the deep venous system and tentorial angle, with reasonable postoperative surgical outcome. [ABSTRACT FROM AUTHOR]

Background: Even in the modern endovascular era, the treatment of Vein of Galen Malformations (VOGM) is extremely challenging. While their natural history is very poor, endovascular embolization has emerged as the standard of care. These lesions often require multiple treatment sessions to decrease shunting, with each treatment including multiple pedicles. Here we present the first reported use of the Scepter Mini (Microvention, Aliso Viejo, CA) in the treatment of vein of Galen malformations. Clinical presentation: A 7 month old female presented with an enlarging VOGM that was initially identified on prenatal ultrasound. Given the enlarging size of the lesion and failure to meet developmental milestones, the patient underwent planned endovascular embolization of the VOGM. The novel Scepter Mini balloon catheter was used for treatment of this lesion affording easy access to the target pedicle and immediate flow arrest which allowed for immediate cure of the lesion. Conclusion: The novel Scepter Mini Balloon (Microvention, Aliso Viejo, CA) afforded excellent distal access with subsequent immediate flow arrest therefore facilitating endovascular cure. Initially, a staged approach was favored for the treatment of the lesion, but the flow arrest achieved by the Scepter mini facilitated immediate occlusion from a single pedicle. [ABSTRACT FROM AUTHOR]

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. The overall management of VOGMs requires a tailored case-to-case approach, starting with in utero detection and reserving endovascular treatment for indicated cases. Lately, the advances in translational research with whole-genome sequencing and the coupling with cellular-level hemodynamics attempt to shed more light in the pathogenesis and evolution of these lesions. At the same time the advances in endovascular techniques allow for more safety and tailored technical strategy planning. Furthermore, the advances in MRI techniques allow a better understanding of their vascular anatomy. In view of these recent advances and by performing a PUBMED literature review of the last 15 years, we attempt a review of the evolutions in the imaging, management, endovascular treatment and understanding of underlying mechanisms for VOGMs. The progress in the fields detailed in this review appears very promising in better understanding VOGMs and expanding the available therapeutic arsenal. [ABSTRACT FROM AUTHOR]

This paper aims to make simple the evaluation of the main veins related to the brainstem and cerebellum. Posterior fossa venous drainage is best understood in context with its three main collectors: superior: toward the Vein of Galen; posterior: toward the torcular complex; and anterior: toward the superior petrosal sinus. A fourth possible drainage path, often harder to distinguish, is directed toward the inferior petrosal sinus. Veins of these four systems are frequently connected to one another. Despite traditionally being considered less regular than its arterial disposition, posterior fossa venous anatomy follows specific patterns that are easy to identify. The three more representative veins of each venous confluent have been selected, to help in recognizing them angiographically. Since pial large veins are primarily located over the surface of the encephalon, most related anatomical structures can be confidently identified. This is of special interest when angiographic 2D or 3D studies are evaluated and provide fundamental assistance in locating precise structures. To better aid in understanding venous disposition, an overview of embryologic and fetal development is also discussed.

Objective To report three cases of vein of Galen aneurysmal malformation (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 the malformation, 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.

Teaching Point: Typical imaging features of a vein of Galen aneurysmal malformation are enlarged intracranial arterial feeders to a dilated recipient vein.

Purpose: Arteriovenous fistulas of the Vein of Galen region in adults (Ad-VGAVF) are an uncommon entity with specific anatomic features. The aim of this article is to present our experience in the endovascular treatment of this pathology and to propose a therapeutic strategy based precisely on the angioarchitecture of these lesions. Materials and methods: During a 20-year period, 10 patients underwent endovascular treatment of Ad-VGAVF. They were nine men and one woman with a mean age of 50 years (23–66 years) treated with the same embolization strategy. Clinical presentation, angiographic characteristics, therapeutic strategy, and clinical outcomes were recorded. Results: All patients were treated exclusively by endovascular approach. Transarterial access was performed in eight patients and combined transvenous and transarterial access in two. Complete obliteration of the fistula was obtained in all patients. There were no intraprocedural complications. Post-embolization neurological symptoms occurred in 5 of 10 with complete resolution at six months in all of them. Conclusion: Arteriovenous fistulas of the Vein of Galen region in adults present uniform angioarchitecture despite their low prevalence. Based on this constant angioarchitecture and especially on the features of its venous drainage, judicious embolization strategy is feasible and effective. Ten cases treated entirely by endovascular approach with excellent clinical and angiographic outcomes show this treatment like a curative alternative for this entity of deep topography and severe prognosis. [ABSTRACT FROM AUTHOR]

Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant disease. The diagnostic criteria of HHT, or Curaçao criteria, include the following: recurrent epistaxis or nighttime nose bleeding, mucocutaneous telangiectases, visceral arteriovenous malformation, or an appropriate family history. The diagnosis is classified as definite if three criteria are present, possible if two criteria are present, and unlikely if only one is present. Nowadays, the confirmation of HHT diagnosis is based on molecular genetic studies. It has been showed that only mutations of genes encoding proteins within the transforming growth factor beta signaling pathway were responsible for the manifestation of the disease. The vein of Galen malformation (VOGM) as a presenting sign of HHT is rare. The prenatal diagnosis of HHT is even rarer. Herein, we present a case of prenatally diagnosed case of HHT based on the presence of VOGM in the fetus. To our knowledge, it is the first time that the gene mutation discovered in this case manifested as VOGM in the fetal life. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Isolated thrombosis of the vein of Galen is a rare and serious entity with few cases reported in the literature. We report the case of a previously healthy 18-year-old male who was admitted after developing headache and subsequently worsening mental status, requiring endotracheal intubation for airway protection. During his admission he developed symptoms of severe paroxysmal sympathetic hyperactivity and posturing. The computed tomography and magnetic resonance imaging of the brain showed bilateral thalamic lesions. The magnetic resonance angiography and digital arteriography revealed a thrombosis of the deep cerebral venous system (vein of Galen). We call attention to a case with rapid symptom progression and specific radiological findings, with atypical clinical course, characterized by paroxysmal sympathetic hyperactivity, but with good clinical functional outcome.

Pial arteriovenous fistulas are abnormal vascular connections between arteries and the cranial venous system on the surface of the brain in the pia mater. In contrast to vein of Galen aneurysmal malformations, they lack a true vascular nidus and may only indirectly involve the vein of Galen. An 18-month-old boy was referred to us with a history of seizures, drowsiness, bulging cephalic veins, and cranial nerves palsy identified as vein of Galen aneurysmal malformation according to initial magnetic resonance imaging. Digital subtraction angiography showed a vertebrobasilar junction pial high-flow fistula between the basilar artery and anterior pontomesencephalic vein, causing retrograde flow, dilation, and tortuosity of the lateral and posterior mesencephalic vein as well as the vein of Galen. Endovascular treatment, including coil embolization of fistula site, was done successfully without complication resulting in recovery from previous symptoms and signs. If cerebral venous dilations are encountered, one must keep in mind that the location of the fistula may not be in close proximity to the dilated veins, especially when there is a suspicion for vein of Galen aneurysmal malformation. Other pathologies, such as pial arteriovenous fistula, in a region away from the site of the vein of Galen must be considered. [ABSTRACT FROM AUTHOR]

Arteriovenous malformations (AVM) are congenital vascular lesions fed by arterial feeders originating from branches of the internal carotid artery (ICA) or vertebrobasilar artery. We experienced unique AVMs arising in the midline Galenic region, receiving blood supply from the ICA/vertebral artery systems and the external carotid artery system. We retrospectively reviewed data on eight patients who had an AVM arising in the Galenic region and were treated in the University of Tokyo Hospital between 1990 and 2019. The median age at diagnosis was 62 years. Three cases (38%) presented with obstructive hydrocephalus due to aqueduct obstruction caused by an engorged vein of Galen. In all cases, feeders from dural arteries were present and the vein of Galen was the primary drainer. All patients underwent stereotactic radiosurgery. Five patients were followed for > two years; nidus obliteration was confirmed in one, and > 75% shrinkage was confirmed in three, while one patient died due to hemorrhage. Altogether, AVMs arising in the Galenic region are rare and exhibit several peculiar characteristics including the presence of dural feeders, an older age at presentation and presentation with obstructive hydrocephalus.

A 4-year-old male presented with a large arachnoid cyst over the left temporal region causing displacement of adjacent structures. Cerebral angiography showed dilatation of the tentorial sinus without other apparent vascular alterations. The association of these two anomalies raises a therapeutic dilemma as no information is available about how the variants of the venous system can modify cerebrospinal fluid hydrodynamics and thus affect arachnoid cyst's prognosis. In this case, the patient was treated conservatively and has remained stable for 2 years. [ABSTRACT FROM AUTHOR]

Aneurism of the vein of Galen is a rare congenital anomaly, where complex arteriovenous malformation are identified between multiple choroidal arteries and the median prosencephalic vein of Markowski, which is a precursor of the vein of Galen, with subsequent aneurysmal enlargement of the respective arteriovenous system. The congenital malformation develops during week 6 to 11 of fetal development. Infants often die from highoutput congestive heart failure. We present a case of a 40 years old patient, presented the first time at hospital at 28 weeks of gestation for lower perception of fetal movements. The patient was referred to our Prenatal Diagnosis Unit for a suspected cardiac malformation. We diagnosed cardiomegaly along vein of Galen aneurysmal malformation (VGAM) with severe cardiac failure, ascites, and critical fetal distress. Fetal demise was noted 24 hours later, during the corticosteroid procedures for fetal maturation. A stillborn weighting 2000g with a severe hydropic aspect was born after labor induction. Autopsy was performed following injection of colored gelatin in carotid vessels. The aneurysmal defect was evident in the vein of Galen and straight sinus. We also found the transverse, sagittal and the occipital sinus seriously dilated. We could not highlight the communication with the middle cerebral artery, because of the degradation of the cerebral tissue, despite an optimal preparation. [ABSTRACT FROM AUTHOR]

Purpose: To present three fetal vein of Galen aneurysmal malformations (VGAMs), which were diagnosed through magnetic resonance imaging (MRI), and highlight these cardiovascular findings.Materials and Methods: We retrospectively reviewed three fetuses with VGAM at 31, 32, and 33 weeks of gestation. Feeding arteries and draining veins were observed by MRI. Secondary changes in the brain and high-output heart failure caused by high blood flow in the lesion were evaluated. Two fetuses were born, and neonatal MRI was performed. One fetus was terminated.Results: A characteristic dilated structure in the midline of the brain presented in each fetus. The arteriovenous fistula led to anatomical brain changes such as in the hydrocephalus, dilated feeding vessels (one or more), jugular vein, and/or superior vena cava. Substantial brachiocephalic vessel dilation was observed in two fetuses. Following parturition, one baby had neonatal asphyxia and sinus thrombosis, and MRI revealed hypoxic-ischemic encephalopathy. Cardiomegaly was detected in all three cases.Conclusion: With a large field of view, fetal MRI can observe brain VGAM, as well as the heart and affected large vessels. It can determine hydrocephalus, ischemia, intracranial hemorrhage, and sinus thrombosis. Providing such information on the infant's entire body can aid clinicians in determining the most appropriate treatment.Level Of Evidence: 4 J. Magn. Reson. Imaging 2017;46:1535-1539. [ABSTRACT FROM AUTHOR]

Postsurgical deep brain venous thrombosis has not been well described in children before. When approaching thalamic or intraventricular lesions, extra care should be taken to prevent injury to the internal cerebral veins (ICVs) and the vein of Galen. However, even when they are well preserved during surgery, postoperative hemodynamic changes, mainly in the first 24 h, or surgical manipulation can cause thrombosis of these veins. We report 2 children with unilateral postoperative ICV thrombosis; in 1 of the patients the vein of Galen was also thrombosed. Although both patients had altered sensorium initially, no anticoagulation therapy was given, and they both recovered well. When approaching thalamic or intraventricular lesions, extra care should be taken to prevent injury to the ICV and the vein of Galen. The surgeon should respect the deep brain venous system when approaching midline structures. Both the neurosurgeon and the neuroradiologist should be aware of this possible complication in order to make a prompt diagnosis and to offer proper treatment if needed. [ABSTRACT FROM AUTHOR]

Background: Galenic dural arteriovenous fistulas (DAVF) are rare; however, they are the most frequent type of DAVF to manifest aggressive clinical behavior and usually represent a diagnostic and therapeutic challenge for clinicians. Methods: We retrospectively reviewed clinical and imaging data of patients managed with neuroendovascular techniques for the treatment of galenic DAVFs from 2000 to 2016. We searched the 2000-2016 English-language literature for papers discussing neuroendovascular management of galenic DAVFs, with or without companion surgical procedures. Results: Five patients were treated for galenic DAVFs during the study period (four males; mean age, 61 years). Three presented with progressive neurological deterioration due to venous congestion, two with acute intracranial hemorrhage. Three were treated by staged transarterial embolization procedures (three procedures in two, four procedures in one); two underwent a single transvenous embolization procedure. Four out of five fistulas were completely occluded. All patients improved clinically; the patient whose fistula was partially occluded remains angiographically stable at 2-year follow-up. Six reports describing 17 patients are reviewed. Embolization was performed via transvenous approach in 1/17 and transarterial approach in 16/17 with additional open surgery in 9/16. The trend toward the use of transarterial approaches is based primarily on advances on embolization techniques that allow better and more controllable penetration of the embolizing agents with improved clinical and angiographic results, as well as the technical complexity of the transvenous approach. Conclusions: Although transarterial embolization is the preferred endovascular route for the management of most galenic DAVFs, selected cases can be successfully treated by transvenous approach. [ABSTRACT FROM AUTHOR]

Vein of Galen aneurysmal malformation (VGAM) is a rare congenital vascular malformation. We describe a unique case of VGAM diagnosed antenatally due to severe intrauterine growth restriction (IUGR) and heart failure. The neonate underwent two transarterial interventional embolization procedures on days 1 and 4 due to developing ventricular dilation and worsening cardiac failure. We emphazise the importance of a prenatal brain color-Doppler study in all cases of IUGR and the importance of tertiary center referral for a multidisciplinary team approach. Early embolization should be considered in the presence of antenatal heart failure, aiming to limit the neurological sequelae and the multiorgan failure. [ABSTRACT FROM AUTHOR]

Background: The coexistence of venous anomalies, such as vertical embryonic positioning of the straight sinus (VEP of SS), has been reported in patients with atretic cephalocele (AC). VEP of SS has been exclusively encountered when the AC is found above the torcular. Clinical presentation: We report a patient with suboccipital AC associated with venous anomalies analogous to VEP of SS, consisted of the Galenic venous system which did not drain into the straight sinus in the tentorium, but into the falcine sinus instead. Differences with VEP of SS in our case had no anatomical relationship between the falcine sinus and the suboccipital AC and no large cerebrospinal fluid space around the falcine sinus. A detailed neuroradiological examination was helpful for detecting these minute anomalies. [ABSTRACT FROM AUTHOR]

Vein of Galen aneurysmal malformation is a rare intracranial arteriovenous malformation .It accounts for up to 30% of intracranial vascular malformations in pediatric population and 1% of all pediatric congenital anomalies. It causes severe morbidity and mortality in neonates in the form of high output cardiac failure, nonimmune hydrops, hydrocephalus and intracranial hemorrhage. It is often diagnosed in postnatal period. Prenatal diagnosis of VGAMs may be accomplished with prenatal ultrasound (US) and fetal MRI which helps in early diagnosis and effective management.

Vein of Galen aneurysmal malformation is rare vascular developmental anomaly that arises prenataly and is ussually diagnosed during prenatal sonographic screening investigation or just in neonatal period. Ethiopathogenic factors of the disease are not well known and clinical features are various. Prenataly, congenital disease can be presented as cardiac failure or fetal hydrops. Postnatal clinical picture is usually asymptomatic and vascular anomaly is found during screening sonographic investigation of the brain or it is manifested as cardiac insufficiency in varying degrees. An important part of patient management is a multidisciplinary approach that allows early diagnosis of the disease, which is a prerequisite for successful treatment. Authors present two case reports of newborns with different clinical manifestation and management in early postnatal period. [ABSTRACT FROM AUTHOR]

The aim of this case report is to describe a rare non-hypoxic cause of pathological changes in fetal heart rate pattern during labor, and to determine management, including a description of important prenatal aspects when pathologic cardiotocographic recording is performed during labor. A fetus with rare arteriovenous malformation of the vein of Galen, which represents less than 1% of all intracranial arteriovenous malformations, was monitored by intrapartum external cardiotocography in the 37 + 5 gestational week. The baby was born by cesarean section because of signs of imminent intrauterine hypoxia on cardiotocography. However, metabolic acidosis was not confirmed in umbilical cord blood sampling. Despite intensive neonatal care management, the newborn died 31 h after delivery because of progressive cardiac decompensation, hypotension and multi-organ failure. Precise diagnosis of the abovementioned pathology, a pre-labor plan for delivery and postnatal prognosis assessment can significantly contribute to the avoidance of a misdiagnosis of fetal hypoxia and unnecessary operative delivery with marked medico-legal consequences. [ABSTRACT FROM AUTHOR]

Galenic dural arteriovenous fistula (DAVF) represents a unique, hard to treat subgroup of tentorial DAVFs. We present an unusual case of hemorrhagic Galenic DAVF in a 54-year-old woman. The fistula drained directly to the vein of Galen through multiple feeders. Complete occlusion of the fistula was achieved through transarterial embolization. Deep venous drainage remained intact and the patient recovered well. To our knowledge, this is the first report on complete closure of hemorrhagic Galenic DAVF using transarterial embolization with complete obliteration of vein of Galen. The presence of nonfunctioning straight sinus may have contributed to the success of treatment and it may be considered as a predictive marker for endovascular embolization. [ABSTRACT FROM AUTHOR]