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.

Rome-Galen (129 to ca. 216).

Over the last 50 years the significance of Galen's contributions to the arts of medicine and surgery have been increasingly recognized. Despite his errors, his contributions to medical and surgical practice have been profound. In the present context, his teachings on cranial surgical instruments and technique would continue to be influential throughout one and a half millenia. His technical advice was sound. His error about the anatomy of blood vessels supplying CNS were not of much consequence since the CNS would remain surgically inaccessible until the end of the 19th century. He reclassified fractures as extending to the diploe or through the internal table. Moreover, they could be simple, comminuted, depressed, or elevated. He did not mention indications or clinical changes, on the other hand he had many sensible remarks to make on the instrumentation required for cranial surgery. As will be seen in what follows, he was much quoted in justification of the decisions of his successors. The major errors of significance related to his description of the anatomy of the cranium and its sutures. His concern about the cranial attachment, vascular components and excretory functions of the sutures added to Hippocrates' concerns about their inherent weakness resulted in the teaching that trepanation should avoid these structures. This was to have a limiting effect on the placement of trepanation openings which was of no benefit to the patients. Moreover, his enthusiasm for bloodletting would also serve to potentiate Hippocrates teaching on this matter, to the advantage of nobody.

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.

Pediatric Cerebral Vascular Malformations: Current and Future Perspectives.

Intracranial vascular malformations typically encountered by pediatric neurosurgeons include arteriovenous malformations, vein of Galen malformations and cavernous malformations. While these remain amongst some of the most challenging lesions faced by patients and caregivers, the past decade has produced marked advances in the understanding of the pathophysiology of these conditions, with concomitant innovations in treatment. This article will highlight present and future perspectives relevant to these diseases, with a focus on an emerging approach utilizing disease-specific mutations to develop a novel taxonomy for these conditions.

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.

Aortic Steal Correlates with Acute Organ Dysfunction and Short-Term Outcomes in Neonates with Vein of Galen Malformation.

INTRODUCTION: Vein of Galen aneurysmal malformation (VGAM) is a rare, congenital cerebrovascular malformation with high morbidity and mortality. Parameters to foresee clinical progression and allow individualized parent counseling are lacking. The aim of this study was to evaluate aortic steal measured by Doppler ultrasound as a prognostic parameter in these neonates. METHODS: A retrospective monocentric analysis of cardiac ultrasound exams before embolization in neonates with VGAM was conducted. Percentage of aortic steal measured by time-averaged maximum velocity above and below the zero flow baseline by pulsed Doppler ultrasound at the preductal aortic isthmus was calculated. Association of aortic steal with parameters of acute organ dysfunction (Bicêtre neonatal evaluation score [BNES], neonatal multiple organ dysfunction score [NeoMODS]) and mortality and determination of correlation between aortic steal and cerebral damage on initial and follow-up cerebral magnetic resonance imaging (cMRI) were evaluated. RESULTS: Twelve neonates were included, of which 3 died. Per 10 percentage point increase of aortic steal, BNES decreased by 1.64 (95% confidence interval [CI]: 1.28-2.0) points and the maximum observed NeoMODS increased by 1.25 (CI: 0.94-1.57) points. The odds for mortality increased by 2.3 (CI: 1.14-13.67) per 10 percentage point increase of aortic steal. There was a correlation between aortic steal and cerebral damage at baseline (white matter ρ [rho] = 0.34, gray matter ρ = 0.81) and follow-up (white matter ρ = 0.80, gray matter ρ = 0.72). CONCLUSION: The degree of aortic steal in neonates with VGAM was highly associated with the severity of organ dysfunction, disease progression on cMRI, and mortality.

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.

Revised concept of rete-like collateral formation: Rete mirabile does not exist in humans.

The carotid rete is a physiological network between the external and internal carotid arteries (ICA) in lower vertebrates. However, true carotid rete does not exist in humans. This review aimed to contrast the physiological function of human "rete-like collaterals" with that of lower vertebrate "rete mirabile". An explanation for the development of rete-like collaterals in human intracranial arteries was also discussed. The rete mirabile (carotid, vertebral, spinal, and thoracic) in lower vertebrates has a specific physiological role and does not form vasculature for the same purpose in humans. Therefore, the term "rete mirabile" should not be used for cases reported in humans. Instead, "rete-like collaterals" is preferred. In the literature, rete-like or arterial anastomosis was observed in the ICA cavernous portion and the intradural arteries. Based on the hypothesis of the segmental concept, it applies to the ICA and intracranial arteries. Whether in the ICA, middle cerebral artery, posterior cerebral artery, or posterior inferior cerebellar artery, the segmental concept is the same and should be considered to have formed secondary collaterals after segmental regress or dysgenesis of affected arteries. Summarily, the significance of this review lies in its reevaluation of vascular structures previously described as "carotid rete" in humans to a true and preferred term, "rete-like collaterals". It also provides insights into the historical context and potential genetic factors associated with the formation of arteries in humans, contributing to a better understanding of human vascular anatomy.

A Curious Case of Proptosis and Intracranial Calcifications Caused by a Vein of Galen Aneurysmal Malformation.

Vein of Galen aneurysmal malformation (VGAM) is a rare, congenital, intracerebral arteriovenous malformation with a poor prognosis. This disorder commonly presents during the neonatal period and rarely in infancy and childhood. Reported here is a case of VGAM in a three-month-old female baby who presented with proptosis and intracranial calcifications, which are rare presentations of this rare entity. The diagnosis was confirmed by magnetic resonance imaging (MRI).

Use of levosimendan in hemodynamic management of heart failure in two neonates with intracranial arteriovenous shunts: a case series.

BACKGROUND: The hemodynamic status of newborns with intracranial arteriovenous shunts (AVSs) may be extremely complex. Mini-invasive hemodynamic monitoring through innovative techniques such as Near-Infrared Spectroscopy (NIRS) and Pressure Recording Analytical Method (PRAM) may help in understanding hemodynamics in newborns with AVSs. Levosimendan is a calcium sensitizer and inodilator, and it is known to improve ventricular function, but its use in newborns is limited. In our cases, we evaluated the effect of levosimendan on hemodynamics through NIRS and PRAM. CASE PRESENTATION: Herein, we report the cases of two neonates with intracranial arteriovenous shunts, in whom we used levosimendan to manage cardiac failure refractory to conventional treatment. Levosimendan was used at a dosage of 0.1 mcg/kg/min for 72 h. Combined use of NIRS and PRAM helped in real-time monitoring of hemodynamic effects; in particular, levosimendan determined significant improvement in myocardium contractility as well as a reduction of heart rate. CONCLUSION: In two neonatal cases of AVSs, levosimendan led to an overall hemodynamic stabilization, documented by the combination of NIRS and PRAM. Our results suggest introducing levosimendan as a second-line treatment in cases of severe cardiac dysfunction due to AVSs without improvement using standard treatment strategies. Future prospective and larger studies are highly warranted.

Echocardiography evaluation of neonatal vein of Galen aneurysmal malformation.

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

Long-Term Outcomes of Endovascular Embolization in a Vein of Galen Aneurysmal Malformation: A Single-Center Experience.

BACKGROUND: A vein of Galen aneurysmal malformation (VGAM) is a rare congenital cerebral vascular condition with a high mortality rate if left untreated. This study describes the long-term outcomes of patients with VGAM, who were treated with endovascular embolization. METHODS: This retrospective analysis focused on VGAM patients who underwent one or more endovascular embolization sessions between January 2008 and December 2022. The study included newborns and children under 18 years. Data encompassed clinical and demographic characteristics, types of endovascular embolization, treatment complications, mortality rates, and long-term outcomes. RESULTS: Out of 22 VGAM cases, the majority were boys (86.36%), and the average age of the participants was 38 months, ranging from 25 days to 17 years. Endovascular embolization using liquid embolizing agents was the most common intervention (50%), and around 73% of patients underwent multiple sessions. Some patients underwent ventriculoperitoneal shunting (VPS) due to persistent hydrocephalus. In long-term outcomes, four patients (18.2%) showed developmental delays, and 16 patients (72.7%) had a positive outcome. CONCLUSIONS: Combining endovascular therapy with a comprehensive management strategy significantly reduces mortality rates and improves the possibility of normal neurological development in patients.

Meges of Sidon: The "Most Learned" of Surgery's Founding Fathers.

This paper aims to bring back to life an underrated, even forgotten surgeon of the late first century B.C.E., Meges of Sidon. He was an experienced surgeon of his time and was considered the most erudite Roman surgeon before Galen. He belonged to the Alexandria School of Medicine and later migrated to Rome to practice. Although most of his work did not survive, he was mentioned by notable ancient figures, such as Celsus and Galen. He excelled in various surgical specialties, not limited to neurosurgery, orthopedics, ophthalmology, and urology. Galen cited Meges in his surgical book on head injuries and cranial procedures. Meges was known to have invented a "double-edged" blade that he used to remove stones from the neck of the bladder. His treatment of anal fistulas was a reference through the Middle Ages. Celsus, a Roman encyclopedist of the first century, would later erroneously receive credit for ancient surgical innovations, such as the nonslipping cranial drill and the treatment of depressed skull fractures, even though he was not a surgeon. However, as Celsus was going over the history of surgery, he described Meges as the "most learned" of its prominent figures. Meges' neurosurgic techniques and teachings are deduced from Celsus, who shortly succeeded him, did not practice surgery, and acknowledged him as his primary source on surgical topics.

[The Galenic case reports on inflammatory diseases of the musculoskeletal system]. / Die Fallberichte des Galen von Pergamon über entzündliche Erkrankungen des Bewegungsapparats.

BACKGROUND: The case histories in the writings of the Greco-Roman physician Galen of Pergamum have so far been interpreted primarily in literary and socio-historic terms. Analysis focused on the medical aspects is still incomplete. QUESTION: Which competence for the treatment of inflammatory diseases of the musculoskeletal system do the Galenic case reports communicate? STUDY DESIGN AND RESEARCH METHODS: The 358 Galenic case histories were studied for anamnestic, clinical, therapeutic, and epidemiological statements on inflammatory diseases of the musculoskeletal system. RESULTS: Eight case reports could be identified in which inflammatory diseases of the musculoskeletal system are discussed. The descriptions are found in the writings On the Powers and Mixtures of Simple Drugs (n = 3), On the Composition of Drugs according to Kind (n = 2), Therapeutics to Glaucon (n = 1), How to detect Malingerers (n = 1) and On Hippocrates' 'Aphorisms' (n = 1). Seven male individuals and one group of patients are described; in one case a proper name is given. The descriptions do not follow a fixed structure. The texts are dominated by information on the anamnesis and catamnesis, the results of the physical examination and the choice of therapy. The author has repeatedly combined the case description with theoretical explanations. The most common inflammatory diseases of the musculoskeletal system that Galen was confronted with were soft tissue swelling without/with associated skin symptoms, gout, arthritis and sciatica. Knees and feet were affected more frequently than the hands. Galen himself was always the observing and treating physician. Therapy was dominated by ointments, fats and oils that should lead to the distraction or drying out of pathogenic juices. The statements on the prognosis were mostly favourable. DISCUSSION: The case reports illustrate the range of inflammatory diseases of the musculoskeletal system without being able to reliably identify individual nosological entities retrospectively. Equating 'arthritis' with chronic polyarthritis and 'podagra' with gout is also not undisputed. The doctor-patient-conversations are the most original element in terms of content and style. The explanations for the choice of therapy show that the numerous mixed agents disposable for the treatment of patients with inflammatory diseases of the musculoskeletal system were prescribed after critically weighing the advantages against the disadvantages.

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.

Understanding development of jugular bulb stenosis in vein of galen malformations: identifying metrics of complex flow dynamics in the cerebral venous vasculature of infants.

Introduction: Computational fluid dynamics (CFD) assess biological systems based on specific boundary conditions. We propose modeling more advanced hemodynamic metrics, such as core line length (CL) and critical points which characterize complexity of flow in the context of cerebral vasculature, and specifically cerebral veins during the physiologically evolving early neonatal state of vein of Galen malformations (VOGM). CFD has not been applied to the study of arteriovenous shunting in Vein of Galen Malformations but could help illustrate the pathophysiology of this malformation. Methods: Three neonatal patients with VOGM at Boston Children's Hospital met inclusion criteria for this study. Structural MRI data was segmented to generate a mesh of the VOGM and venous outflow. Boundary condition flow velocity was derived from PC-MR sequences with arterial and venous dual velocity encoding. The mesh and boundary conditions were applied to model the cerebral venous flow. We computed flow variables including mean wall shear stress (WSSmean), mean OSI, CL, and the mean number of critical points (nCrPointsmean) for each patient specific model. A critical point is defined as the location where the shear stress vector field is zero (stationary point) and can be used to describe complexity of flow. Results: The division of flow into the left and right venous outflow was comparable between PC-MR and CFD modeling. A high complexity recirculating flow pattern observed on PC-MR was also identified on CFD modeling. Regions of similar WSSmean and OSImean (<1.3 fold) in the left and right venous outflow channels of a single patient have several-fold magnitude difference in higher order hemodynamic metrics (> 3.3 fold CL, > 1.7 fold nCrPointsmean). Specifically, the side which developed JBS in each model had greater nCrPointsmean compared to the jugular bulb with no stenosis (VOGM1: 4.49 vs. 2.53, VOGM2: 1.94 vs. 0, VOGM3: 1 vs. 0). Biologically, these regions had subsequently divergent development, with increased complexity of flow associating with venous stenosis. Discussion: Advanced metrics of flow complexity identified in computational models may reflect observed flow phenomena not fully characterized by primary or secondary hemodynamic parameters. These advanced metrics may indicate physiological states that impact development of jugular bulb stenosis in VOGM.

Pial arteriovenous fistula associated with vein of Galen dilatation in adult: A case report and MRI findings.

Pial arteriovenous fistula (PAVF) is a rare intracranial vascular lesion where direct communication exists between one or more pial arteries and a cerebral vein, without an intervening nidus and located in the subpial meningeal space. When the drainage of PAVF involves a dilated, but already formed vein of Galen (VOG), it should be distinguished from other vascular lesions located in this area, because their angio-architecture, natural history and treatment options are different. A 33-year-old female was admitted to our hospital with a history of new-onset generalized tonic-clonic seizures. Clinical examination showed no neurological deficit. Magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) depicted an arteriovenous fistula that was fed by the pial branches from left posterior cerebral artery and drained into the medial atrial vein before joining the VOG confluence and causing VOG dilatation. No nidus between the feeding arteries and draining vein, dural feeding arteries, or anatomical variations commonly seen with true vein of Galen aneurysmal malformations (VOGM) were found. These finding suggested a diagnosis of a PAVF associated with vein of Galen dilatation, which was confirmed by digital subtraction angiography. The patient was treated with transarterial glue embolization in 1 section, resulting in nearly complete occlusion of the fistula. Conventional MRI and MRA are noninvasive modalities that can provide valuable information regarding the anatomic localization of the fistula point, the feeding arteries, the venous sac, and their relationship with surrounding structures. These techniques are helpful for accurate diagnosis and treatment planning.

Intracranial arteriovenous shunts in infants: A decade of experience from a quaternary pediatric center.

BACKGROUND AND PURPOSE: Intracranial arteriovenous shunts (IAVS) are rare vascular diseases in infants. They can be categorized into vein of Galen aneurysmal malformation (VGAM), pial arteriovenous fistula (PAVF), and dural arteriovenous fistula associated with dural sinus malformation (DAVF/DSM). We sought to review the clinical presentation, imaging characteristics, endovascular treatment (EVT), and outcomes of IAVS in infants presenting to a quaternary pediatric referral center over one decade. METHODS: A retrospective review of a prospectively maintained database was performed of all infants diagnosed with IAVS between January 2011 and January 2021 in a quaternary pediatric referral center. For each patient, demographic data, clinical presentation, imaging findings, management strategies, and outcomes were reviewed and discussed. RESULTS: Over the study period, 38 consecutive infants were diagnosed with IAVS. Patients with VGAM (23/38, 60.5%) presented with congenital heart failure (CHF) (14/23), hydrocephalus (4/23), and seizures (2/23), and three patients were asymptomatic. Eighteen patients with VGAM underwent EVT. Among those, 13 patients (72.2%) were successfully treated with an angiographic cure and three patients (3/18, 17%) died. Patients with PAVF (9/38, 23.7%) presented with CHF (5/9), intracranial hemorrhage (2/9), and seizures (2/9), and all of them were successfully treated endovascularly. Patients with Type I DAVF/DSM (4/6, 66.6%) presented with mass effect (2/4), cerebral venous hypertension (1/4), CHF (1/4), and cerebrofacial venous metameric syndrome (1/4). Patients with type II DAVF/DSM (2/6, 33.3%) presented with a thrill behind the ear. Patients with DAVF/DSM were treated endovascularly, five patients were cured, and one with type I DAVF/DSM died. CONCLUSION: Intracranial arteriovenous shunts are rare but potentially life-threatening neurovascular pathologies in infants. Endovascular treatment is challenging but feasible in carefully selected patients.

Vein of Galen malformations in the newborn: case series.

BACKGROUND: Vein of Galen malformations (VoGMs) in newborns often represent life-threatening emergencies. Outcome is difficult to predict. The authors review 50 VoGM cases to correlate anatomical types with treatment and outcome. OBSERVATIONS: Four distinct types of VoGMs are identified: mural simple (type I), mural complex (type II), choroidal (type III), and choroidal with deep venous drainage (type IV). Seven patients presented with mural simple VoGMs with a "single hole" fistula supplied by only one large feeder. These patients were treated electively at >6 months; development was normal. Fifteen patients presented with complex mural VoGMs. Multiple large feeders joined a single fistulous point within the wall of the varix. Patients typically presented with congestive heart failure (CHF) and required emergent transarterial intervention. Mortality was 7.7% with less than two-thirds developing normally. Twenty-five patients presented with choroidal VoGMs. Multiple large arterial feeders joined at multiple fistulous sites. Severe CHF in most patients required emergent transarterial and sometimes transvenous intervention. Mortality was 9.5%; two-thirds of the patients had a normal development. Three babies presented with choroidal VoGMs with deep intraventricular venous drainage. This phenomenon caused fatal "melting brain syndrome" in all three patients. LESSONS: Recognition of the specific VoGM type determines treatment options and sets outcome expectations.