Intracranial vein of Galen malformation and its management: A case report.

Vein of Galen malformation (VOGM) is a rare congenital arteriovenous malformation affecting the pediatric population, characterized by a fistula between the diencephalon and a dilated vein of Galen. The present study reports the case of a 6-month-old male infant referred for developmental delays and abnormal head circumference. A clinical examination revealed macrocephaly, dilated scalp veins and neurological abnormalities. Neuroimaging confirmed a large VOGM with associated hydrocephalus. A multidisciplinary team devised a treatment plan involving endovascular embolization and ventriculoperitoneal shunt placement. The post-operative recovery exhibited an improvement in neurodevelopmental milestones and a reduced head circumference. Generally, the management of VOGM requires a comprehensive approach, including early diagnosis, precise imaging and timely intervention. The case described herein highlights the importance of interventional radiology in planning a management plan and the novel kissing microcatheter endovascular embolization technique.

Pediatric interventional neuroradiology: "How I do it" vol. II-Embolization.

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.

A Case of Vein of Galen Aneurysmal Malformation Diagnosed Prenatally in a Monochorionic-Diamniotic Twin Pregnancy.

We present a case of a vein of Galen aneurysmal malformation (VGAM), a rare congenital arteriovenous malformation, in one fetus of a monochorionic-diamniotic twin pregnancy. The diagnosis was made with color Doppler ultrasonography at 28 weeks and the affected fetus was found to have worsening cardiomegaly on subsequent fetal echocardiograms. She was emergently delivered at 32 weeks for abnormal fetal heart rate tracing of the affected twin. Magnetic resonance imaging of the brain findings after delivery demonstrated severe neurological injury; therefore, postnatal embolization was not performed. The neonate died on day of life 9. The cotwin survived without neurological complications. This is the first case in the literature of a VGAM diagnosed prenatally in a monochorionic-diamniotic twin pregnancy and demonstrates the challenge of delivery timing with prenatal diagnosis in a twin pregnancy.

A systematic review of prenatally diagnosed vein of Galen malformations: prenatal predictive markers and management from fetal life to childhood.

Introduction: Vein of Galen malformations (VGMs) account for less than 1% of all intracranial vascular malformations. However, in fetal and pediatric populations, they represent the most common vascular malformation of the brain. For the effective management of this condition, an optimal knowledge of its prenatal and postnatal clinical features is mandatory. Methods: Articles published between 1 January 2003 and 31 January 2024, reported in PubMed and EMBASE, were evaluated for a systematic review analyzing the prenatal and postnatal features and management of fetal VGMs. Results: Thirty-one papers reporting information on 51 prenatally diagnosed VGMs were included. The most common prenatal features were fetal hydrocephalus (39%) and cardiomegaly (56%). Postnatal data for 43 VGM cases are described. The overall mortality was 58.14%. In total, 77.78% of the survivors had normal development. Conclusions: Close follow-up and a multidisciplinary approach are mandatory to manage this condition. Our study aimed to provide a guide for gynecologists, neonatologists, cardiologists, and neuroradiologists.

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

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

Systemic venous anomalies in a child with a vein of Galen.

We describe our findings in a child with a vein of Galen malformation, in whom the right superior caval and the azygos veins drained into the roof of the morphologically left atrium. A persistent left superior caval vein drained into the morphologically right atrium through the coronary sinus. The additional presence of dual brachiocephalic veins permitted the deployment of a multifunctional ventricular septal defect occluder device to occlude the right superior caval vein, correcting the right-to-left shunt. This also prevented azygos venous drainage into the left atrium.

Neonatal intracranial haemorrhage secondary to vein of Galen aneurysmal dilatation.

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.

Literature review and case report: Rare case of successful prenatal detection of Vein of Galen Malformation (VGAM).

Vein of Galen aneurysmal malformation (VGAM) is a rare vascular anomaly originating during embryonic development, specifically between the 6th and 11th weeks of gestation. This malformation results from abnormal arteriovenous connections between primitive choroidal arteries and the median prosencephalic vein (MPV) of Markowski. Typically, the MPV regresses by the 11th week, but in VGAM, this regression is hindered, leading to persistent abnormal flow and the formation of arteriovenous shunts. We present a case of successful prenatal detection, as well as a comprehensive literature review that summarizes current knowledge, emphasizes the importance of prenatal detection, detailed imaging techniques, understanding clinical presentations, and outlines treatment options. Prenatal detection, crucial for early intervention, has become feasible through ultrasonography and MRI. Fetal MRI has emerged as the gold standard, offering detailed insights into arterial feeders, nidus presence, fistula position, venous drainage, and potential complications. The clinical presentation of VGAM varies with age, and neonates diagnosed in utero may exhibit signs of high-output cardiac failure. Early detection is critical for timely intervention, as untreated VGAMs often result in high mortality rates. Prognosis depends on the severity of heart failure, the number of arteriovenous shunts, and the presence of accompanying fetal abnormalities. Various imaging modalities, including CT angiography and digital subtraction angiography (DSA), aid in the assessment and treatment of VGAM. DSA remains the gold standard for evaluating angioarchitecture and guiding endovascular interventions. The optimal treatment for VGAM is transarterial embolization, offering significant improvements in prognosis. Surgical interventions are limited due to high morbidity and mortality. Management decisions should consider the balance between minimizing neurological damage and achieving maximum embolization effectiveness.

Cerebrofacial Venous Metameric Syndrome Mimicking Vein of Galen Aneurysmal Malformation.

Cerebrofacial venous metameric syndrome (CVMS) is a complex low-flow vascular malformation affecting bone and soft tissues including brain, dura mater, and eye. We show images of CVMS in an 18-month-old boy presenting facial venous malformations, developmental venous anomalies, dural sinus malformations, and dilated great cerebral vein, suggesting a vein of Galen aneurysmal malformation. Although Sturge-Weber syndrome is the most known form of CVMS, its presentations are variable and include several venous malformations. Recognizing the various manifestations of CVMS is necessary for adequate screening, treatment, and follow-up.

Multidisciplinary care of fetal vein of Galen arteriovenous malformation diagnosed via Doppler ultrasound and magnetic resonance imaging: A case report.

Fetal vein of Galen malformation (VOGM) represents a rare congenital anomaly affecting the fetal cerebral vasculature. A 27-year-old Middle Eastern woman was referred due to intrauterine growth restriction (IUGR) and fetal cardiac anomalies identified at 35 weeks of gestation. The diagnosis of fetal VOGM with dilated neck vessels was established through a combination of color Doppler ultrasonography and magnetic resonance imaging. A multidisciplinary approach involving maternal-fetal medicine, neonatology, pediatric cardiology, and interventional radiology was implemented. Given the grave prognosis for the baby, the patient received comprehensive counseling. Subsequent monitoring revealed a non-reassuring fetal heart trace, prompting the decision to perform a cesarean section. The newborn, a girl, was admitted to the neonatal intensive care unit for further management but she died shortly thereafter, with heart failure and intracranial hemorrhage identified as the probable causes of death. In summary, the diagnosis and management of VOGMs demand specialized expertise and a collaborative, multidisciplinary approach to optimize patient care and outcomes.

Spontaneous thrombosis of type II vein of Galen aneurysmal malformation: a case report.

Vein of Galen malformations (VGAMs) are rare and complex congenital brain vascular anomalies that pose significant diagnostic and treatment challenges. The natural history of this type of vascular anomaly is very poor, with many patients succumbing to complications such as congestive heart failure, hydrocephalus, and brain parenchymal injury. Although the clinical course of most VGAMs was considered unfortunate, with meticulous imaging, a group of lesions with a more placid presentation and course can be identified.

Chemical abscess post vein of Galen aneurysmal malformation embolisation with ethylene vinyl alcohol copolymer.

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.

Vein of Galen Malformations.

Significant efforts have been made over the last few decades to improve the diagnosis and management of patients with vein of Galen malformations (VOGMs). The mainstays of treatment remain focused on primary endovascular management by staged transarterial embolizations with adjunctive use of transvenous embolization, medical therapy, and neurosurgical intervention for symptom control in select patients. Innovation in endovascular technology and techniques as well as promising new genomic research elucidating potential therapeutic targets hold significant promise for the future of VOGM treatment.

Vein of Galen Aneurysmal Malformation: A Case Report.

Vein of Galen aneurysmal malformation is a relatively rare disease in which failure of the median prosencephalic vein of Markowski to involute early in gestation leads to a grossly dilated deep cerebral vein with multiple arterial feeders, causing a large arteriovenous shunt which leads to high-output cardiac failure. We describe a case of a term neonate who presented to a tertiary neonatal centre on day one of life with history, symptoms, and signs consistent with perinatal asphyxia; however, in the context of worsening multi-organ dysfunction and cardiomegaly, the infant was found to have a severe vein of Galen aneurysmal dilatation leading to high-output cardiac failure. The patient was transferred to a tertiary paediatric hospital and underwent a total of four coiling procedures to embolise the multiple feeder arteries supplying the aneurysmal malformation. This case highlights the difficulties in diagnosing this relatively uncommon condition, particularly in the context of a possible perinatal insult.

Vein of Galen aneurysmal malformation associated with brain abscess: A computed tomography case report.

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.

Epileptic Seizures in a Pediatric Patient With Vein of Galen Aneurysmal Malformation and Obstructive Hydrocephalus: A Rare Case Report.

The vein of Galen aneurysmal malformation (VGAM) is a rare congenital arteriovenous fistula of the embryonic median prosencephalic vein of Markowski, resulting in its pathological dilation. If left untreated, it can lead to multiple severe complications in the neonatal period, among which obstructive hydrocephalus. We present a case report of a six-year-old male patient with severe status epilepticus and a clinical history of VGAM and obstructive hydrocephalus, diagnosed via an MRI and an MR-angiography. The hydrocephalus was treated via a ventriculostomy at the age of six months, while the VGAM underwent a partial transarterial endovascular embolization when the patient was four years old. The procedures were successful, and there were no significant post-operative complications observed. The epileptic seizures began at a later point and were successfully medicated with valproate. However, they resumed due to a lowering of the medication dosage by the patient's parents. The patient was given a new valproic acid regimen with an appropriate dosage, and his parents reported no further seizures. This case report emphasizes the use of appropriate prenatal and neonatal diagnostic methods for VGAM and explores the nature of the multi-procedural therapy approach towards the pathology and its complications in relation to a possibly idiopathic co-pathology, namely epilepsy.

Vein of Galen aneurysmal malformation: does size affect outcome?

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

Update on the Vein of Galen Aneurysmal Malformation : Disease Concept and Genetics.

Vein of Galen aneurysmal malformation is one of important pediatric arteriovenous shunt diseases, especially among neonates and infants. Here, early history of the disease identification, basic pathoanatomy with a focus on the embryonic median prosencephalic vein, classification and differential diagnoses, and recent genetic studies are reviewed.

Use of greyscale and Doppler ultrasound in initial evaluation and follow-up of neurovascular malformations in children.

Pediatric intracranial arteriovenous shunts are rare vascular malformations that can be diagnosed prenatally or postnatally, as an incidental finding or due to complications. We propose a review of cerebral vascular malformations in newborns and infants with special emphasis on neurosonography and Doppler ultrasound as the first diagnostic method. Sonography can thus contribute in the planning of further studies that are always necessary, and in post-therapy follow-up.

Supracerebellar "Flyover" Approach to Dumbbell Falcotentorial Meningioma Encasing the Galenic Venous System.

Falcotentorial meningiomas involve the tentorial apex and straight sinus, posing challenges when encasing the galenic venous system.1 Microneurosurgery is considered the best treatment option for large falcotentorial meningiomas because it provides a definitive cure.2 In contrast, Gamma Knife surgery mainly allows the control of smaller or residual tumors after microsurgical resection.3 Approach selection between interhemispheric supratentorial versa supracerebellar transtentorial is dictated by the displacement of the Galen vein.1,4-8Video 1 describes the critical surgical steps of the supracerebellar "flyover" approach for a Bassiouni type II dumbbell falcotentorial meningiomas encasing the galenic venous system. Preoperative embolization was ruled out due to potential additional morbidity and mortality risks.9,10 A perimedian supracerebellar infratentorial transtentorial approach was performed with the patient in ¾ prone Concorde position. After early devascularization and division of the tentorium, the meningioma was internally debulked while preserving the arachnoid plane. The posterior choroidal arteries, internal cerebral veins, basal veins of Rosenthal, and vein of Galen were carefully dissected, and the tumor was completely resected. The patient was discharged on postoperative day 3 with no deficits. Postoperative magnetic resonance imaging confirmed a Simpson grade 1 resection. Pathology revealed a grade 2 meningioma. The patient remained asymptomatic with no recurrence at a 10-year follow-up. The reported case demonstrates that the most critical factor in the choice of approach to midline dumbbell falcotentorial meningiomas is the relationship of the tumor to the galenic venous system and its tributaries.