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In seven of 255 consecutive patients (2.7%) who underwent pulmonary arteriovenous malformation (PAVM) embolization at our center between July 1, 1996, and July 1, 2000, the feeding artery was considered too short for safe occlusion with use of standard stainless-steel coils or detachable balloons. These patients were successfully treated with use of a modified vein of Galen technique similar to the one used for managing intracranial vein of Galen aneurysms. In this article, the authors report their experience with this technique in safely occluding the aneurysmal sacs of PAVMs in patients with short (<2 cm) feeding pulmonary arteries. [Copyright &y& Elsevier]

Summary. Objective and Importance: Vein of Galen aneurysm is a rare congenital anomaly of cerebral circulation and occurs mainly in newborns and children. The spontaneously thrombosed vein of Galen aneurysm is especially rare and an uncommonly reported event. Clinical Presentation: Two patients with a spontaneously thrombosed vein of Galen aneurysm were operated on at our institute. CT and MRI demonstrated space-occupying lesions of the pineal region and two other masses in the posterior fossa in the second case. The first described case should be referred to as the true type of aneurysm. The vascular malformation, revealed in the second case should be referred to as the false type. In this case the vein of Galen was enlarged to a gigantic size due to the blood drainage from the arteriovenous malformation supplying it from the inferior posterior cerebellar artery. Technique: Thrombosed aneurysmal sacs were resected totally by subtentorial supracerebellar and median suboccipital approaches. Conclusion: The authors report two cases of successful surgical resection of a thrombosed vein of Galen aneurysm in children. Diagnostic features are considered and the informative value of magnetic resonance imaging is stressed. The principles of choosing the right approach and dissection techniques of thrombosed aneurysms of the vein of Galen are described. [ABSTRACT FROM AUTHOR]

A fetus with a large supratentorial cyst and cardiomegaly was encountered at 33 weeks of gestation. The cyst appeared as an aneurysmal, fluid-filled structure extending posteriorly with a finger-like appendage. Using color flow mapping, we disclosed rapid in-and-out blood flow with marked turbulence within the cyst. For evaluation of its blood supply and venous drainage of the vascular malformation, a three-dimensional reconstruction of the power Doppler image was conducted. The results revealed that the vascular malformation was supplied by a small contralateral aneurysm from the branches of Willis' circle, draining posteriorly into an abnormal falcine sinus and then into the superior sagittal sinus. No other fetal abnormality such as hydrocephalus or hydrops was discovered. The prenatal diagnosis of an aneurysm of the vein of Galen was made on the basis of the gray-scale, color Doppler findings, and also the angioarchitecture obtained by three-dimensional power Doppler imaging. The woman was admitted at 37 weeks of gestation due to labor onset and delivered the baby via the vaginal route without complication. Postnatal angiography and magnetic resonance imaging confirmed the diagnosis of an aneurysm of the vein of Galen, and the angioarchitecture depicted it before birth. We suggest that three-dimensional power Doppler ultrasonography may assist in the diagnosis of an aneurysm of the vein of Galen, and precisely delineate the complicated corresponding vasculature. This may guide postnatal management and predict the prognosis more accurately. [ABSTRACT FROM AUTHOR]

Objective: The goals of this study were to evaluate the extent of resection (EOR) obtained with an occipital interhemispheric transtentorial approach (OITA) in a series of pediatric patients with pineal region tumors and to define preoperative radiological factors predictive of the EOR., Methods: This is a retrospective cohort study of a series of pediatric patients with pineal tumors who underwent surgery through a microsurgical OITA performed by the senior author during the period from January 2006 to January 2020. The tumor volume was measured preoperatively, and then on sagittal midline cuts the authors identified the most cranial point of the torcular Herophili (defined as the "Herophilus point") and the lowest point of the inferior profile of the vein of Galen (defined as the "Galen point"). The line joining these two points (defined as the "Herophilus-Galen line" [H-G line]) was used to identify the "Herophilus-Galen plane" (H-G plane) perpendicular to the sagittal plane. Tumor volumes located below and above this plane were measured. EOR was evaluated by measuring residual tumor volume visible on T1 volumetric injected sequences of immediate postoperative MRI., Results: Thirty patients were selected for study inclusion. The preoperative mean tumor volume was 15.120 cm3 (range 0.129-104.3 cm3). The mean volumes were 2.717 cm3 (range 0-31 cm3) above the H-G plane and 12.40 cm3 (median 5.27 cm3, range 0.12-72.87 cm3) below the H-G plane. Three patients underwent only biopsy. Of the remaining 27 patients, gross-total resection (GTR; 100% tumor volume) was achieved in 20 patients (74%). In the remaining 7 patients, the mean residual tumor volume was 7.3 cm3 (range 0.26-17.88 cm3). In 3 of these patients, GTR was accomplished after further surgical procedures (1 in 2 patients, 3 in 1 patient) for an overall GTR rate of 85.18%. Larger tumor volume was significantly associated with incomplete resection (p < 0.001). A tumor volume ≤ 2 cm3 above the H-G plane (p = 0.003), linear extension ≤ 1 mm above the H-G line, and pineal histology were predictive of GTR at first OITA procedure (p = 0.001)., Conclusions: The H-G line is an intuitive, easy-to-use, and reliable indicator of the superior anatomical limit of visibility during the microsurgical OITA. This anatomical landmark may be useful as a predictor of EOR for pineal tumors performed through this approach. The main limitations of this study are the small number of patients and the exclusively pediatric age of the patient population.

Over the past 10 years (1982-1992), we have been actively involved in the management of 179 cerebral arteriovenous malformations (CAVMs) in children and infants. Seventy-seven were true vein of Galen malformations (VGAMs) and 102 were pial AVMs (PAVMs), i.e., developed in the subpial space. Hemorrhage occurred as the first symptom in 50% of the children with pial AVMs, but was present in none of the VGAM cases. Only 31 children were found to be unsuitable for endovascular treatment, and in 124 cases embolization was indicated as the primary treatment (104 embolization performed). Only 21 children underwent a direct surgical approach (none in the VGAM group). In the embolized group in whom treatment has been completed ( n = 56), 8 children died, 39 have an anatomical cure, and 34 are clinically normal. In the group under treatment ( n = 56), 16 are not normal. The problems are timing and the aims (total or partial treatment) of the therapeutic procedures. In the nonembolized group ( n = 31), 8/13 of the pial lesions were operated on (no mortality, 2 patients with moderate neurological deficits). In the VGAM group 13/18 died and 4 had spontaneous thrombosis (only 1 is neurologically normal). In the nonembolized group 13 lesions have been completely excluded, but only 5 patients are neurologically normal. This fact again stresses the need for prognostic evaluation before treatment and a clear definition of the treatment aims. Analysis of a large number of published series on the management of children with AVMs (1017 cases) reveals inconsistencies that hamper proper evaluation and comparison. In our experience, endovascular treatment always seems to be the best primary treatment in both VGAMs and PAVMs. However, management of children with these lesions requires a large multidisciplinary team, which is the only way of offering the most suitable and effective treatment, the sole guarantee of a good result. [ABSTRACT FROM AUTHOR]

Vein of Galen aneurysms are uncommon malformations that can be treated with a combination of endovascular embolization and surgery. Since the relevant techniques are new and innovative, their pathologic sequelae are not yet well described. A patient treated with staged embolization over a 15-month period developed evidence of cerebral venous hypertension on angiography. At necropsy, we observed marked myointimal proliferation of the aneurysm wall, dura, and cerebral vessels in addition to severe widespread encephalomalacia, calcinosis, and rare mural thrombi. These vascular changes have not been previously described in cases of vein of Galen aneurysm, and may, in part, be a consequence of embolization therapy. [ABSTRACT FROM AUTHOR]

The extremely high morbidity and mortality associated with all forms of management of aneurysms of the vein of Galen continues to pose a tremendous challenge. Recent advances in neuroradiologic endovascular embolization techniques have offered some encouraging early results in the treatment of these vascular lesions. Our experience in managing two neonates and a 10-year-old boy with vein of Galen aneurysms is presented. The neonates underwent transtorcular embolization, while the boy was treated by standard transarterial balloon embolization. All patients had satisfactory outcomes. The combined surgical and radiologic approaches are simple when compared to previous surgical techniques, and early results suggest that they are preferable alternatives. The natural history of vein of Galen aneurysms and the various therapeutic modalities available are discussed. [ABSTRACT FROM AUTHOR]

Congestive heart failure is the usual complaint and cause of death in neonates with aneurysms of the great vein of Galen. Direct surgical obliteration of the feeding arteries cures the heart failure, but few such newborns survive the operation. The authors report a case of primary aneurysm of the great vein of Galen treated successfully by clipping the afferent arteries and reduction of the bulk of the aneurysm by bipolar coagulation. Five months after the operation the child's development is normal. [ABSTRACT FROM AUTHOR]

Using a Doppler Mark 500 of ATL we measured the systolic and diastolic velocity of the red cells into the main arteries of the neck (diastolic rate cm/sec) thus approaching the flow in two cases of upper brain stem AVM with a unique drainage into the superior longitudinal sinus through an enlarged vein of Galen and a falcine sinus. Because of the desperate clinical condition of the children it was decided to stop the high blood flow by closing the aspiration coming from the low pressure of the venous system above and below the site of union between the malformation and the superior longitudinal sinus. An intra-operative and post-operative measurement of the diastolic rate showed a radical effect of the closure of the superior longitudinal sinus. The demonstration of this aspirative factor has allowed us to classify an AVM as a haemodynamic push-pull malformation. [ABSTRACT FROM AUTHOR]

4 patients with acquired arteriovenous shunts located in the wall of an ectatic vein of Galen (VG) are reported. They represent so-called VG dural arterio-venous malformations (DAVM). These shunts involve the vasa vasorum normally present in the VG wall. Physiopathology, clinical signs and age groups are usually the same as those encountered in other DAVMs; conversely they are totally different from the other mural shunts of the VG, detected in the pediatric population (true VG arteriovenous malformations), and leading to different clinical symptoms. Venous approach in these VG DAVMs is almost always contraindicated unless secondary occlusion of VG afferent has spontaneously excluded the pouch from the cerebral venous circulation. [ABSTRACT FROM AUTHOR]

We report a neonate with congestive heart failure due to a huge arteriovenous malformation of the vein of Galen. Two dimensional ultrasound showed a large cystic structure behind the 3rd ventricle and the quadrigeminal plate. The cyst compressed the posterior part of the 3rd ventricle and the aqueduct and caused an obstructive hydrocephalus. The diagnosis was established by colour Doppler imaging (CDI). With the help of CDI, flow within the aneurysm could be shown as well as feeding arteries originating from the posterior and middle cerebral arteries. The straight sinus and the torcular Herophili were markedly dilated. [ABSTRACT FROM AUTHOR]

An aneurysm of the vein of Galen can have a varied CT appearance in infancy. The classical picture is a spherical posterior third ventricular mass with the density of circulating blood contiguous with a dilated straight sinus and with uniform contrast enhancement. With varying degrees of thrombosis of the aneurysm which may occur in late infancy, the mass can change in density and the dilated straight sinus disappear. With total thrombosis, a precontrast hyperdense rim develops with a low density center. The rim enhances but the low density center does not change with contrast infusion. Our experience in three patients with this spectrum of CT findings will be demonstrated and correlated with clinical findings and angiography. [ABSTRACT FROM AUTHOR]

An aneurysm of the vein of Galen is a rare midline arteriovenous malformation. The clinical signs and the prognosis strictly depend on age of presentation and on the amount of blood shunted into the malformation. In the newborn, cardiac failure is the most common presenting sign. In older children hydrocephalus, headache, focal neurological signs and subarachnoid bleeding may be the reason for admission. At the Hospital for Sick Children six patients with an aneurysm of the vein of Galen were studied by angiography and CT. Four of them were newborn in cardiac failure. Angiography was essential for the accurate demonstration of all the feeding vessels to the malformation and their exact location. Computed tomography also added useful information on associated hydrocephalus and ischemic brain damage. The dilated vein of Galen, straight sinus and torcular were clearly seen. Abnormal arterial vessels feeding the malformation were also recognized. [ABSTRACT FROM AUTHOR]

Two neonates with cardiovascular symptoms associated with intracranial AVM were initially considered to have ductal-dependent congenital heart disease. Prostaglandin E (PGE) infusion, initiated to achieve patency of the ductus arteriosus, produced improved oxygenation and cardiovascular status in both infants. Other vascular effects of PGE, including pulmonary and extracranial systemic vasodilation, likely accounted for these unique observations in the infants with intracranial AVM. Not only may the signs and symptoms of congenital heart disease be imitated by intracranial AVM, but improved oxygenation and cardiovascular status with PGE infusion used for suspected congenital heart disease may be observed as well. [ABSTRACT FROM AUTHOR]

Arteriovenous malformations of the vein of Galen, though rare, are associated with a significant mortality. The anaesthetic management of patients with this defect during surgery may be complicated by massive blood loss and congestive heart failure. Four patients under 18 months of age underwent craniotomies for arteriovenous malformations of the vein of Galen. Since two required a second craniotomy for ligation of residual feeding vessels, there were six procedures in the four patients. Average blood loss per procedure was estimated at 126 per cent of blood volume. One patient died during operation and one had an intra-operative cardiac arrest. Both of these patients had associated cardiac failure. In the presence of a compromised myocardium, sudden blood loss or attempts at induced hypotension may result in inadequate diastolic perfusion of the heart and precipitate cardiac arrest. Consequently, these patients should be maintained in a euvolaemic state with transfusion and attempts at controlled hypotension should be avoided. Les malformations artério-veineuses de l'ampoule de Galien, bien que rares, sont associées avec un taux de mortalité important. La conduite anesthésique nécessitée par la chirurgie de cette condition peut se compliquer d'hémorragie massive et d'insuffisance cardiaque congestive. Quatre jeunes patients de moins de 18 mois on subi des craniotomies pour des malformations artério-veineuses de l'ampoule de Galien. Comme deux de ces patients ont dû subir une deuxième craniotomie pour ligature de vaisseaux nourriciers superficiels on a compté six interventions sur quatre patients. La perte sanguine moyenne par intervention a été évaluée à 126 pour cent du volume sanguin. Un patient est mort pendant l'intervention et un autre a subi un arrêt cardiaque per-opératoire. Deux de ces patients souffraient également d'insuffisance cardiaque. En présence de myocarde défaillant, l'hémorragie subite ou des tentatives d'hypotension contrôlée peuvent causer une perfusion dyastolique inadéquate du coeur et précipiter un arrêt cardiaque. En conséquence ces malades devraient être maintenus dans un état euvolémique avec des transfusions et on devrait éviter toute tentative d'induire l'hypotension contrôlée. [ABSTRACT FROM AUTHOR]

A newborn with antenatal diagnosis of fetal hydrops at 36 wk of gestation, presented with congestive heart failure (CHF) and generalized edema. Computed tomographic angiography showed marked dilatation of cerebral duro-venous system including vein of Galen (VOG), straight sinus, torcula and transverse sinus without evidence of arteriovenous fistulae at the vein of Galen. Dilatation of duro-venous system resolved with concomitant improvement in biventricular function and CHF with decongestive therapy. Such entity should be differentiated from more serious conditions like VOG malformation and venous sinus thrombosis. [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. [ABSTRACT FROM AUTHOR]

We report a neonate with vein of Galen malformation (VGM) who developed congestive heart failure (CHF) early after birth. Serial changes in plasma B-type natriuretic peptide (BNP) following an endovascular embolization procedure for VGM were mirrored in his clinical CHF status. The plasma BNP level markedly increased to 1800 pg/ml (normal, <100 pg/ml) in accordance with the severity of CHF. It rapidly decreased to 356 pg/ml during the first week after endovascular embolization for VGM. In the following 3 weeks there was an unexpected upward trend in plasma BNP despite echocardiography revealing normal biventricular function. After additional evaluation and treatment for CHF, BNP decreased again and the patient’s clinical status concurrently improved. The patient was discharged with a normal BNP level. Monitoring serial plasma BNP provides valuable information regarding the need for additional evaluation or treatment of newborns with CHF and may be used to document improvement. [ABSTRACT FROM AUTHOR]

We report a rare case of developmental deep venous system anomaly. The great vein of Galen and the straight sinus were absent. Both internal cerebral veins and the basal veins of Rosenthal drained into a large frontal interhemispheric falcine vein, which eventually drained into the superior sagittal sinus. The patient also had an associated neuronal migration anomaly.Copyright © 2002 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Preeclampsia, a hypertensive disorder of pregnancy, complicates up to 10 % of all pregnancies and increases the risk for perinatal stroke in offspring. The mechanism of this increase is unknown, but may involve vascular dysfunction. The goal of this study was to evaluate the effect of experimental preeclampsia (ePE) on cerebrovascular function in offspring to eludciate a possible mechanism for this association. Dams were fed a high cholesterol diet beginning on day 7 of gestation to induce experimental preeclampsia. Middle cerebral arteries (MCA) and the Vein of Galen (VoG) were isolated from pups from ePE dams and compared to pups from normal pregnant (NP) dams at postnatal days 16, 23, and 30 and studied pressurized in an arteriograph chamber. Markers of inflammation and oxidative stress were measured in serum. Our results suggest altered structure and function in both MCA and VoG of ePE pups. We also found evidence of systemic inflammation and oxidative stress in ePE pups. These findings provide a potential link between preeclampsia and the occurrence or severity of perinatal stroke., (Copyright © 2021 Elsevier B.V. All rights reserved.)

A 5-year-old boy with macrocephaly and mental retardation was referred for radiologic evaluation. After cranial CT and MR imaging, the diagnosis of mural type vein of Galen aneurysmal malformation was established by angiography. Two weeks later, preembolization angiography revealed complete thrombosis of the malformation. Although it is a very rare event, vein of Galen aneurysmal malformation may spontaneously thrombose following diagnostic angiography. Possible effects of contrast media on thrombosis were discussed. [ABSTRACT FROM AUTHOR]

Research on the anatomy of cerebral deep veins (CDVs) around the vein of Galen (VG) is very important and has fundamental clinical significance. Large-scale anatomical studies of CDVs using computed tomography angiography (CTA) are rarely reported. A retrospective study of the CDVs around the VG was conducted in Chinese patients of Han nationality. One hundred cases were included in the final analysis. The patients were aged from 17 to 78 years (mean: 42.3 years). Also, 46% of the patients were female. The diameter of the internal cerebral vein (ICV) at its beginning and termination points ranged from 0.4 to 2.8 mm (1.49 ± 0.39 mm) and 0.4 to 3.5 mm (2.05 ± 0.47 mm), respectively. There was statistical significance regarding the diameter of the ICV at its beginning and termination points (P <0.01). The ICV length ranged from 28.5 to 47.9 mm (36.86 ± 3.74 mm). The length of the straight sinus (SS) ranged from 30.2 to 57.8 mm (43.6 ± 6.37 mm). The length of the VG ranged from 1.5 to 41.8 mm (9.30 ± 4.76 mm). The angle at the VG and SS transition area ranged from 25.4 to 110.6° (77.2 ± 18.0°). This study was a meaningful attempt to conduct anatomical research of CDVs using CTA. Preoperative familiarity with the normal venous structure and its variation around the VG would be helpful for endovascular treatment., Competing Interests: Competing Interests: The authors have declared that no competing interest exists., (© The author(s).)

The pediatric neurovascular disease runs the chronologic spectrum with dramatic changes in the presentation, evaluation, and treatment from the prenatal, perinatal, and infant periods through childhood and adolescence. These diseases are often dynamic throughout this period and the dynamic continues throughout life. There are four major categories: high-flow arteriovenous shunting lesions, arterial aneurysms, low-flow vascular lesions, and vascular occlusive disease. The high-flow lesions can be subdivided into a vein of Galen malformation, non-Galenic arteriovenous fistula, dural sinus malformations and fistula, and arteriovenous malformation. Low-flow vascular lesions include cerebral cavernous malformation, developmental venous anomaly, and capillary telangiectasia. The cerebrovascular occlusive disease can be divided between arterial occlusive disease and cerebral venous sinus thrombosis. The presentation of each of these entities can be very similar, especially in younger children; however, imaging and laboratory analysis can establish the diagnosis leading to the most appropriate therapy. A multidisciplinary team, dedicated to treating pediatric cerebrovascular disease, is important in delivering the best outcomes in these complex diseases. Given the relative rarity of pediatric presentation of cerebrovascular disease, many apply adult concepts to children. A better understanding of the diseases and their difference from adults makes a critical difference in selecting the correct approach., (Copyright © 2021 Elsevier B.V. All rights reserved.)

We describe spontaneous disappearance of an aneurysmal malformation of the vein of Galen (AMG), an anomaly that occurs most often in children, with a high mortality due to the accompanying severe cardiac failure. Spontaneous thrombosis of an AMG is rare. In this case, the AMG has been closed for about 5 years, without radiological evidence of thrombosis, and no active treatment. Proposed mechanisms of spontaneous thrombosis include slow flow shunts and severe obstruction of the venous outflow. However, in this case, the disappearance of the AMG without evidence of thrombosis indicates a different mechanisms, namely, obstruction of the feeding artery. [ABSTRACT FROM AUTHOR]

Two unique cases are presented of infants with signs of vein of Galen malformations, whose unsuspected associated sinus venosus atrial septal defects were detected during routine echocardiography. A conservative approach to cardiac treatment is advocated. [ABSTRACT FROM AUTHOR]

Two-dimensional color-coded Doppler examination revealed a cranial arteriovenous (AV) malformation in a critically ill newborn with intractable congestive heart failure. This case emphasizes the value of color-coded Doppler in the diagnosis of this rare malformation. [ABSTRACT FROM AUTHOR]

Background: Vein of Galen malformations (VOGM) comprise nearly a third of pediatric cerebrovascular anomalies, with potentially devastating neurological and systemic complications. Advances in endovascular therapies have dramatically improved outcomes compared to historical surgical treatments, and neurosurgeons are an essential component of the multidisciplinary critical care team., Objective: To retrospectively review pediatric patients with VOGM treated at Texas Children's Hospital (TCH), a quaternary referral center, over 15 yr, and present lessons learned in treating children with modern endovascular techniques., Methods: Charts from TCH were retrospectively reviewed for the past 15 yr. Patients with diagnosis including "Vein of Galen," "Vein of Galen malformation," "Vein of Galen aneurysmal malformation," or any abbreviations (ie, VOG, VOGM, VOGAM) were reviewed. Presentation, imaging, treatment specifics, and clinical outcomes were reported., Results: There were 18 patients with VOGM managed at TCH from 2002 to 2018 with a total of 29 embolizations. Seventeen were performed with a single embolisate (NBCA or Onyx), and 12 with a combination. A dual lumen balloon catheter was used as an adjunct in 3 embolizations. Complications occurred in 5 embolizations (24%), including hemorrhage, embolisate migration, and femoral vessel occlusion. Surviving patients were followed for a mean of 38 mo, with 12 having normal or near-normal neurological development., Conclusion: VOGM can present with a myriad of neurological and systemic symptoms, potentially in extremis. Neurosurgical involvement in these cases is critical, as urgent treatment can be lifesaving. Patients may require multiple treatment sessions using a variety of endovascular tools and techniques., (Copyright © 2019 by the Congress of Neurological Surgeons.)

Falcotentorial meningiomas originate in the junction of the falx cerebri and the tentorium. Due to its anatomic vicinity, these tumors have a close relationship with important neurovascular structures surrounding the pineal region including the deep venous system. Surgical approaches would normally consider posterior midline corridors, but decision between supra or infratentorial access should be considered by the size, anatomic displacement of structures, and the infiltration of the dural attachment. This surgical video1,2 demonstrates the surgical technique and pearls to achieve a stepwise resection of a complex falcotentorial meningioma. We present a case of a 42-yr-old female patient, neurologically intact at presentation. A semi-sitting position was used. Appropriate management of cerebrospinal fluid was obtained with an external ventricular drainage which is kept closed until the dura is opened. A suprainfratentorial craniotomy was done with adequate exposure of the superior sagittal sinus and torcula. The supracerebellar infratentorial corridor was used for inferior internal debulking and arachnoidal dissection of the tumor while the exposure obtained in the posterior interhemispheric allowed a corridor which exposed widely the tumor with transtentorial transfalcine extension. Adequate management of adjacent structures was done while preserving the straight sinus and vein of Galen. A gross total removal of the tumor was achieved and the patient was discharged without complications. After this surgical video, the viewer will have learned the steps to safely achieve a surgical removal of a falcotentorial meningioma taking care of its relationship with the venous and neural adjacent structures., (Copyright © 2018 by the Congress of Neurological Surgeons.)

Vein of Galen (VG) aneurysmal malformations (VGAMs) are complex vascular lesions. Their etiopathogenesis is extensively debated and remains poorly understood. Strictly speaking, true VGAMs are Galenic pial arteriovenous fistulas. They are believed to arise in utero and are contended to drain either into the true VG or the median prosencephalic vein of Markowski. Several classification systems have been proposed and are widely used. With the advent of endovascular therapy, precise understanding of the angioarchitecture is critical for management and therapeutic decision making. We review clinical presentation and diagnostic imaging findings, discussing angioarchitectural properties as they relate to treatment planning. Clin. Anat. 31:259-268, 2018. © 2017 Wiley Periodicals, Inc., (© 2017 Wiley Periodicals, Inc.)

Vein of Galen aneurysmal malformation (VGAM) is a rare angiopathy, which most commonly presents in infancy. Although very rare, it is associated with high morbidity and mortality rates. In order to minimize such morbid rates, a prompt diagnosis followed by a timely initiation of management is crucial. Multiple antenatal and postnatal imaging techniques for the diagnosis have been described and discussed in the literature. However, to our knowledge, a comprehensive review exploring such a list of imaging options for VGAM has never been established. We aim to review the diagnostic tools to aid in better understanding of the investigative modalities physicians may choose from when treating patients with a VGAM., Competing Interests: The authors have declared that no competing interests exist.

A large vein of Galen was diagnosed in a 9-month-old boy. This was not treated at birth, as there was no associated congestive heart failure. The patient was followed conservatively and follow-up magnetic resonance imaging showed increase in the size of the vein of Galen malformation. Subsequent cerebral angiogram demonstrated hypertrophied but thrombosed right posterior choroidal artery, suggesting spontaneous thrombosis of the arterial feeder and thus the embolization was not pursued. [ABSTRACT FROM AUTHOR]

OBJECTIVE A common approach to lesions of the pineal region is along the midline below the torcula. However, reports of how shifting the approach off midline affects the surgical exposure and relationships between the tributaries of the vein of Galen are limited. The purpose of this study is to examine the microsurgical and endoscopic anatomy of the pineal region as seen through the supracerebellar infratentorial approaches, including midline, paramedian, lateral, and far-lateral routes. METHODS The quadrigeminal cisterns of 8 formalin-fixed adult cadaveric heads were dissected and examined with the aid of a surgical microscope and straight endoscope. Twenty CT angiograms were examined to measure the depth of the pineal gland, slope of the tentorial surface of the cerebellum, and angle of approach to the pineal gland in each approach. RESULTS The midline supracerebellar route is the shortest and provides direct exposure of the pineal gland, although the culmen and inferior and superior vermian tributaries of the vein of Galen frequently block this exposure. The off-midline routes provide a surgical exposure that, although slightly deeper, may reduce the need for venous sacrifice at both the level of the veins from the superior cerebellar surface entering the tentorial sinuses and at the level of the tributaries of the vein of Galen in the quadrigeminal cistern, and require less cerebellar retraction. Shifting from midline to off-midline exposure also provides a better view of the cerebellomesencephalic fissure, collicular plate, and trochlear nerve than the midline approaches. Endoscopic assistance may aid exposure of the pineal gland while preserving the bridging veins. CONCLUSIONS Understanding the characteristics of different infratentorial routes to the pineal gland will aid in gaining a better view of the pineal gland and cerebellomesencephalic fissure and may reduce the need for venous sacrifice at the level of the tentorial sinuses draining the upper cerebellar surface and the tributaries of the vein of Galen.

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.

A 2-year-old boy with known sickle cell disease presented in acute coma. CT revealed bilateral thalamic infarcts and incomplete sinus thrombosis. Angiography confirmed thrombosis of the straight sinus and vein of Galen. [ABSTRACT FROM AUTHOR]

Tentorial dural arteriovenous fistulae (TDAVFs) are complex lesions with the arteriovenous fistula located between the leaves of the tentorium cerebelli. While a large portion of dural arteriovenous fistulae are treated endovascularly, TDAVF may require additional microsurgical treatment given their high risk of hemorrhage and multitude of feeders. We describe the case of a 65-year-old male who presented with hemorrhage from a straight sinus and galenic TDAVF. The straight sinus portion of the fistula was obliterated by 3 endovascular treatments and 1 microsurgical treatment. The galenic component of the TDAVF persisted and was approached via a posterior interhemispheric approach in a lateral position. This video demonstrates surgical technique and anatomy associated with this rarely seen dural arteriovenous fistula. The video can be found here: https://youtu.be/iOLzWOabLZ0 .

Background: Sphenoid wing region dural arteriovenous fistulas (DAVFs) are rare lesions that are typically fed by middle meningeal artery feeders and that drain via the sphenoparietal sinus or middle cerebral vein. We describe a unique case of a medial sphenoid wing fistula draining exclusively via the basal vein of Rosenthal. Methods: A 55-year-old man presented with progressive right temporal homonymous hemianopsia. Cerebral angiography revealed a DAVF that rapidly filled into the deep venous system via the basal vein of Rosenthal with a large venous varix compressing the optic nerve. The sphenoid wing DAVF was not amenable to endovascular embolization due to direct ophthalmic artery feeders and was therefore treated with surgical obliteration. A right pterional craniotomy with orbitozygomatic osteotomy was performed. Results: The fistula was clip ligated, and the venous varix was incised and drained. Intraoperative angiography demonstrated complete obliteration of the fistula. Conclusions: Sphenoid wing DAVFs may drain via the deep venous system and have a complex arterial feeding network. Key features of the fistula, including deep venous drainage, presence of venous varices, and retrograde leptomeningeal venous drainage, make this an aggressive lesion with a high risk of rupture based on the available natural history data. [ABSTRACT FROM AUTHOR]

Cognitive regression is a well-described presentation of vein of Galen aneurysmal malformations (VGAMs) in childhood. However, it remains unclear whether successful treatment of the malformation can reverse cognitive regression. Here, the authors present the case of a 5-year-old girl with a VGAM that was treated with staged endovascular embolization procedures. Comprehensive neurocognitive assessments were completed before intervention and approximately 6 years after initial presentation. There were significant age-matched improvements in this child's neurocognitive profile over this period. The authors believe that timely and successful treatment of VGAM in children may not only stabilize the associated cognitive deterioration but, in some cases, may ameliorate these deficits. Details of this case and a discussion of neurocognitive deficits related to VGAM are presented.