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.

That which is unseen: 3D printing for pediatric cerebrovascular education.

INTRODUCTION: Pediatric cerebrovascular lesions are very rare and include aneurysms, arteriovenous malformations (AVM), and vein of Galen malformations (VOGM). OBJECTIVE: To describe and disseminate a validated, reproducible set of 3D models for optimization of neurosurgical training with respect to pediatric cerebrovascular diseases METHODS: All pediatric cerebrovascular lesions treated at our institution with adequate imaging studies during the study period 2015-2020 were reviewed by the study team. Three major diagnostic groups were identified: aneurysm, AVM, and VOGM. For each group, a case deemed highly illustrative of the core diagnostic and therapeutic principles was selected by the lead and senior investigators for printing (CSG/JM). Files for model reproduction and free distribution were prepared for inclusion as Supplemental Materials. RESULTS: Representative cases included a 7-month-old female with a giant left MCA aneurysm; a 3-day-old male with a large, complex, high-flow, choroidal-type VOGM, supplied from bilateral thalamic, choroidal, and pericallosal perforators, with drainage into a large prosencephalic vein; and a 7-year-old male with a left frontal AVM with one feeding arterial vessel from the anterior cerebral artery and one single draining vein into the superior sagittal sinus CONCLUSION: Pediatric cerebrovascular lesions are representative of rare but important neurosurgical diseases that require creative approaches for training optimization. As these lesions are quite rare, 3D-printed models and open source educational materials may provide a meaningful avenue for impactful clinical teaching with respect to a wide swath of uncommon or unusual neurosurgical diseases.

Spontaneous thrombosis of vein of Galen malformation managed with surgical resection: a case­based review.

INTRODUCTION: Vein of Galen malformation (VOGM) is an exceptionally uncommon form of congenital intracranial vascular malformations. It is highly unusual for this lesion to spontaneously thrombose. The clinical presentation of a patient may range from being asymptomatic to critically ill. The underlying pathophysiological mechanisms that cause spontaneous thrombosis are still poorly understood. METHODS AND RESULTS: The literature on spontaneous thrombosis of VOGM was systematically reviewed, analyzed, and summarized with a focus on its pathophysiology, types, clinical presentations, diagnosis, management, and outcomes. It was also illustrated with a case presentation. The case presents an unusual presentation and location of a VOGM in a 2-year-old boy who was successfully treated with surgical resection. CONCLUSIONS: A handful of cases of thrombosed VOGM have been reported worldwide where surgery was used to treat the condition. Low-flow fistulas of the mural type are prone to spontaneous thrombosis, have delayed clinical presentations, and are typically diagnosed in young children. Among the many possible manifestations, hydrocephalus is by far the most common. In the absence of blood flow, MRI is the diagnostic test of choice. Depending on the patient's symptoms, surgery to either remove the aneurysm or divert the cerebrospinal fluid usually results in a good prognosis.

Overcoming roadblocks in clinical innovation via high fidelity simulation: use of a phantom simulator to achieve FDA and IRB approval of a clinical trial of fetal embolization of vein of Galen malformations.

BACKGROUND: Vein of Galen malformation (VOGM) is a rare, life-threatening vascular malformation in neonates and is treated with embolization. However, even at the most experienced centers, patients face high mortality and morbidity. In utero treatment options have been limited by lack of animal models or simulations. OBJECTIVE: To create a novel ultrasound phantom simulator for a preclinical feasibility study of in utero fetal intervention for VOGM. METHODS: Novel phantoms were designed and built in two configurations of spherical and windsock shape from cryogel material to mimic the salient vasculature of the fetal VOGM, based on real-patient fetal MR imaging dimensions. Critical anatomy was realistically mimicked within this model and transtorcular ultrasound-guided coil deployment was simulated. Each phantom model was assessed before and after treatment to evaluate coil mass deposition within the target. RESULTS: The two phantoms underwent pretreatment T2-weighted MR imaging assessment, ultrasound-guided embolization, post-treatment MR and fluoroscopic imaging, and visual inspection of the sliced phantoms for target embolization verification. Postoperative MR scans confirmed realistic compact deposition of the coil masses within the central cavity. Phantom embolization results were submitted as part of the institutional review board and US Food and Drug Administration investigative device exemption approval for a first-in-humans clinical trial of fetal intervention for VOGM. CONCLUSIONS: A phantom simulator for fetal intervention of VOGM produces lifelike results during trial interventions, removing obstacles to feasibility and safety evaluations, typically precluded by non-availability of appropriate animal models. The study provides a proof of concept for potentially wider applications of medical simulation to enable novel procedural advancements in neurointerventions.

Vascular Lesions of the Pineal Region: A Comprehensive Review of the Therapeutic Options.

OBJECTIVE: Vascular lesions of the pineal region comprise aneurysms of the pineal region, arteriovenous malformations, cavernous malformations, and vein of Galen malformations. In the present report, we have offered an extensive review of each vascular pineal region lesion. METHODS: We performed an extensive literature review, focusing on the current therapeutic options available for the different vascular lesions of the pineal region. RESULTS: Vascular lesions of the pineal region are rare. Microneurosurgery remains a valid treatment of cavernomas, arteriovenous malformations, and aneurysms. Endovascular treatments seem to be the first option for the vein of Galen malformations, followed by microneurosurgery. Radiosurgery seems beneficial for small-size arteriovenous malformations. Complex and large vascular lesions will require a combination of multiple treatments. CONCLUSIONS: Vascular lesions of the pineal region are complex, uncommon diseases. Thus, definitive therapeutic modalities for these lesions require further research.

Endoscopic third ventriculostomy in an infant with vein of Galen aneurysmal malformation treated by endovascular occlusion: Case report and a review of literature.

INTRODUCTION: Vein of Galen aneurysmal malformations (VGAMs) can, through multiple mechanisms, complicate with hydrocephalus (HCP). It is generally agreed that management strategies in this scenario should focus on endovascular embolizations. Treatment options for non-responders, however, have been only scarcely reported upon. CASE PRESENTATION: We present a nine-month-old boy with a mural type VGAM complicated by HCP. Despite endovascular occlusion of the sole feeder, the child exhibited hydrocephalus progression prompting an Endoscopic Third Ventriculostomy (ETV). This procedure restored a cerebrospinal fluid (CSF) circulation otherwise impaired by aqueduct obstruction. Later, a new feeder arose and a second embolization was ultimately needed in order to achieve VGAM regression. Throughout four years of follow up, the child attained all developmental marks. DISCUSSION/CONCLUSION: VGAMs are prone to hydrocephalus development as there is both an underlying venous congestion and a mechanical, obstructive component. Although there is a rationale for addressing both components, the underlying AV shunts and subsequent venous pressure elevations usually determine failure of traditional CSF shunting strategies. It is therefore challenging to manage HCP in patients who failed to improve following endovascular embolizations. For such cases, ETV stands as an elegant minimal invasive alternative with potential to provide a more physiologic drainage route and thus better allow for neurological development.

A cardiopulmonary bypass strategy to support a patient with vein of Galen malformation.

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.

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

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

Hirayama Disease in a Patient with a History of Late-Onset Symptomatic Vein of Galen Aneurysmal Malformation.

INTRODUCTION: The purpose of this report is to present a rare case of Hirayama disease (HD) in a patient with a history of late-onset symptomatic vein of Galen aneurysmal malformation (VGAM). This report may provide new insights into the pathophysiology of HD, a rare disorder consisting of insidious onset of unilateral weakness and atrophy of the forearm and intrinsic hand muscles. These symptoms are believed to result from cervical myelopathy affecting the anterior horn cells due to abnormal compressive forces on the spinal cord from adjacent anatomical structures (i.e., dura and/or epidural veins), but this has not been proven. VGAM is a rare congenital cerebral vascular malformation, consisting of high-flow arteriovenous shunting between a persistent median prosencephalic vein and arterial feeders, which most frequently presents in the early neonatal period with congestive heart failure. CASE PRESENTATION: We report the case of an otherwise healthy boy who presented with heart failure due to VGAM at 7 years and subsequently presented at 14 years with left-sided HD. His cervical MRI with neck flexion revealed enlarged epidural veins at the C5-C6 spinal level with anterior, leftward displacement of the posterior dura and spinal cord as well as left hemicord flattening and/or atrophy at this level. He underwent successful surgical treatment by C5-C6-C7 osteoplastic laminotomies and tenting and expansile autologous duraplasty, during which enlarged, engorged epidural veins were confirmed and coagulated. This halted the progression of his left hand weakness and atrophy and allowed significant functional improvement. Postoperative catheter angiogram showed no anatomical connection between the persistent VGAM and the engorged epidural veins, and genetic testing revealed no genetic predisposition of vascular malformations. DISCUSSION/CONCLUSION: In this patient, a combined surgical technique was performed that included epidural venous plexus coagulation with posterior autologous duraplasty and dural fixation using tenting sutures. This combined approach led to a favorable clinical and radiographic outcome with no complications or limitations and has not been previously proposed in the literature, to our knowledge. Although not completely ruled out, we found no angiographic connection or genetic predisposition to suggest there is a pathophysiological link between HD and VGAM.

Management of Jugular Bulb Stenosis in Pediatric Vein of Galen Malformation: A Novel Management Paradigm.

INTRODUCTION: Pediatric vein of Galen malformations (VOGMs) are fistulous intracranial malformations arising congenitally within the choroidal fissure that can present with an array of neurological and cardiac sequelae. Associated venous stenosis may result in intracranial venous hypertension and ischemia leading to severe, irreversible cerebral injury. Management of neonatal VOGMs typically involves staged embolization and angioplasty/stenting for relief of venous stenosis. Rarely, jugular foraminal narrowing has been identified as causing jugular bulb stenosis. CASE PRESENTATION: We present the case of a 22-month-old female diagnosed with VOGM prenatally who displayed persistent intracranial venous hypertension despite multiple neuroembolization procedures during the neonatal period. Following initial reduction in arteriovenous shunting, she once again developed venous hypertension secondary to jugular bulb stenosis for which angioplasty was attempted. Failure of angioplasty to relieve the venous hypertension prompted skull base imaging, which revealed jugular foraminal ossification and stenosis. Microsurgical jugular foraminotomy followed by balloon angioplasty and stenting significantly reduced jugular pressure gradients. Restenosis requiring re-stenting developed postoperatively at 9 months, but the patient has remained stable with significant improvement in cortical venous congestion. DISCUSSION/CONCLUSION: This case demonstrates the efficacy of microsurgical decompression of the jugular foramen and endovascular angioplasty/stenting as a novel treatment paradigm for the management of intracranial venous hypertension in the setting of VOGM.

Vein of Galen Malformations: The Texas Children's Hospital Experience in the Modern Endovascular Era.

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.

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

We report the case of a 10-month-old boy with an enlarged head circumference and severe motor developmental delay. MRI showed a vein of Galen malformation (VGAM) with a heavily dilated median prosencephalic vein. Digital subtraction angiography confirmed a mural type VGAM with three feeding arteries arising from the posterior cerebral arteries. Due to the short length of the feeding arteries and the high flow, occlusion of the feeding vessels with detachable coils was not possible because of repeated coil dislocation into the dilated vein. Embolization of the three feeding vessels was then performed with a Woven EndoBridge single layer device (WEB SL17). In two arteries complete occlusion was accomplished with the WEB alone and in one artery additional deployment of two coils was necessary. Follow-up imaging at day 1 after treatment as well as 3 and 9 months after embolization showed persistent occlusion.

Cross-sectional study of a United Kingdom cohort of neonatal vein of galen malformation.

OBJECTIVE: Describe the course and outcomes in a UK national cohort of neonates with vein of Galen malformation identified before 28 days of life. METHODS: Neonates with angiographically confirmed vein of Galen malformation presenting to 1 of 2 UK treatment centers (2006-2016) were included; those surviving were invited to participate in neurocognitive assessment. Results in each domain were dichotomized into "good" and "poor" categories. Cross-sectional and angiographic brain imaging studies were systematically interrogated. Logistic regression was used to explore potential outcome predictors. RESULTS: Of 85 children with neonatal vein of Galen malformation, 51 had survived. Thirty-four participated in neurocognitive assessment. Outcomes were approximately evenly split between "good" and "poor" categories across all domains, namely, neurological status, general cognition, neuromotor skills, adaptive behavior, and emotional and behavioral development. Important predictors of poor cognitive outcome were initial Bicêtre score ≤ 12 and presence of brain injury, specifically white matter injury, on initial imaging; in multivariate analysis, only Bicêtre score ≤ 12 remained significant. INTERPRETATION: Despite modern supportive and endovascular treatment, more than one-third of unselected newborns with vein of Galen malformation did not survive. Outcome was good in around half of survivors. The importance of white matter injury suggests that abnormalities of venous as well as arterial circulation are important in the pathophysiology of brain injury. Ann Neurol 2018;84:547-555.

Vein of Galen Malformation Thrombosis by Single-Stage, 2-Coil Embolization.

Advances in endovascular embolization have improved morbidity and mortality among patients with vein of Galen malformations (VoGMs). The patient presented at 3 months of age with increased head circumference and a bruit over his anterior fontanelle. Diagnostic cerebral angiography confirmed the presence of a large mural-type VoGM. The decision was made to undergo a staged arterial embolization at 4 years of age after developing worsening right-sided hemiparesis. An attempt was made to occlude the posterior choroidal feeding vessel with a large 25 mm × 50 cm coil, followed by a 6 mm × 20 cm coil; however, the high flow of the lesion displaced both coils into the wall of the aneurysmal venous sac. Interval magnetic resonance imaging and angiography revealed partial occlusion of the VoGM at 7 months and complete thrombosis at 24 months post procedure, precluding a need for additional coiling. This case illustrates that a minimal change in intramural flow dynamics of VoGMs could lead to progressive thrombosis.

Presentation, course, and outcome of postneonatal presentations of vein of Galen malformation: a large, single-institution case series.

AIM: To describe presentation, clinical course, and outcome in postneonatal presentations of vein of Galen malformation (VGM). METHOD: Children older than 28 days presenting with VGM (from 2006-2016) were included. Notes/scans were reviewed. Outcome was dichotomized into 'good' or 'poor' using the Recovery and Recurrence Questionnaire. Logistic regression was performed to explore relationships between clinico-radiological features and outcome. RESULTS: Thirty-one children (18 males, 13 females) were included, presenting at a median age of 9.6 months (range 1.2mo-11y 7mo), most commonly with macrocrania (n=24) and prominent facial veins (n=9). Seven had evidence of cardiac failure. VGM morphology was choroidal in 19. Hydrocephalus (n=24) and loss of white matter volume (n=15) were the most common imaging abnormalities. Twenty-nine patients underwent glue embolization (median two per child). Angiographic shunt closure was achieved in 21 out of 28 survivors. Three children died of intracranial haemorrhage (1y, 6y, and 30d after embolization). Ten patients underwent neurosurgical procedures; to treat haemorrhage in four, and hydrocephalus in the rest. Outcome was categorized as good in 20 out of 28 survivors, but this was not predictable on the basis of the variables listed above. INTERPRETATION: Postneonatally presenting VGM has distinctive clinico-radiological features, attributable to venous hypertension. Endovascular treatment is associated with good outcomes, but more specific prognostic prediction was not possible within this cohort. WHAT THIS PAPER ADDS: Clinical and radiological features in older children with vein of Galen malformation relate to venous hypertension. Outcome is good in most cases with endovascular therapy. Mortality is low but is related to intracranial haemorrhage.