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: 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.

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.

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.

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.

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.

Implications of serial magnetic resonance imaging in the management of a newborn with vein of Galen aneurysmal malformation and a review of the relevant literature.

BACKGROUND: Despite advanced endovascular methods and comprehensive intensive care in the neonatal vein of Galen aneurysmal malformation, overall mortality ranges between 37-63% in treated patients with 37-50% of survivors possessing poor neurologic outcomes. These findings stress the need for more accurate and timely recognition of the patients who may and may not benefit from aggressive intervention. CASE: This case report presents a newborn with a vein of Galen aneurysmal malformation whom antenatal and postnatal follow-up included serial magnetic resonance imaging (MRI) including diffusion-weighted series. CONCLUSIONS: Given the experience from our current case and in light of the relevant literature, it is plausible that diffusion-weighted imaging studies may widen our perspective on dynamic ischemia and progressive injury occurring within the developing central nervous system of such patients. Meticulous identification of patients may favorably influence the clinical and parental decision on early delivery and prompt endovascular treatment versus aiding avoidance of further futile interventions both antenatally and postnatally.

Erratum: Pseudo-feeders as a red flag of impending or ongoing severe brain damage in Vein of Galen Aneurysmal Malformation.

[This corrects the article DOI: 10.3389/fped.2022.1066114.].

Outcomes of endovascular embolization for Vein of Galen malformations: An individual participant data meta-analysis.

Introduction: Understanding outcomes after Vein of Galen malformation (VOGM) embolization has been limited by small sample size in reported series and predominantly single center studies. To address these limitations, we perform an individual-participant meta-analysis (IPMA) to identify risk factors associated with all-cause mortality and clinical outcome after VOGM endovascular embolization. Methods: We performed a systematic review and IPMA of VOGM endovascular outcomes according to PRISMA guidelines. Individual patient characteristics including demographic, intra/post-operative adverse events, treatment efficacy (partial or complete occlusion), and clinical outcome were collected. Mixed-effects logistic regression with random effects modeling and Bonferroni correction was used (p ≤ 0.003 threshold for statistical significance). The primary and secondary outcomes were all-cause mortality and poor clinical outcome (moderate/severe developmental delay or permanent disabling injury), respectively. Data are expressed as (mean ± standard deviation (SD)) or (odds ratio (OR), 95% confidence interval (CI), I 2, p-value). Results: Thirty-five studies totaling 307 participants quantifying outcomes after endovascular embolization for VOGM were included. Follow up time was 42 (±57) months. Our analysis contained 42% neonates (<1 month) at first embolization, 45% infants (1 month ≤2 years), and 13% children (>2 years). Complete occlusion was reported in 48% of participants. Overall all-cause mortality was 16%. Overall, good clinical outcome was achieved in 68% of participants. First embolization as a neonate [OR = 6.93; 95% CI (1.99-24.08); I 2 < 0.01; p < 0.001] and incomplete embolization [OR = 10.87; 95% CI (1.86-63.55); I 2 < 0.01; p < 0.001] were associated with mortality. First embolization as a neonate [OR = 3.24; 95% CI (1.47-7.15); I 2 < 0.01; p < 0.001], incomplete embolization [OR = 5.26; 95% CI (2.06-13.43); I 2 < 0.01; p < 0.001], and heart failure at presentation [OR = 3.10; 95% CI (1.03-9.33); I 2 < 0.01; p = 0.002] were associated with poor clinical outcomes. Sex, angioarchitecture of lesion, embolization approach (transvenous vs. transarterial), and single or multistage embolization were not associated with mortality or clinical outcome. Conclusions: We identify incomplete VOGM embolization independently associated with mortality and poor clinical outcome. While this study provides the highest level of evidence for VOGM embolization to date, prospective multicenter studies are needed to understand the optimal treatment strategies, outcomes, and natural history after VOGM embolization.

Tratamiento endovascular a malformación aneurismática de la Vena de Galeno / Endovascular treatment of aneurysmal malformation of the Vein of Galen

Gestante de 19 años sin antecedentes de embarazos previos, presenta a las 27 semanas en ecografía del segundo trimestre hallazgo sugestivo en el feto de MAVG; a las 38 semanas se realiza parto por cesárea sin complicaciones, se planificó angiografía cerebral con embolización. Se abordó por la arteria femoral derecha utilizando lavado heparinizado continuo y sistema de navegación coaxial se estudió la MAVG de tipo mural, se canalizo de forma selectiva a las arterias coroideas postero medial y postero lateral derecha y a la arteria coroidea posteromedial izquierda y se embolizó bajo control radiológico utilizando espirales de coils y líquido embolizante Onix. Control inmediato mostro cierre completo de conexiones fistulosas y preservación de ramas sanas.

A 19-year-old pregnant woman with no history of previous pregnancies, presented at 27 weeks in a second-trimester ultrasound suggestive finding in the fetus of MAVG; At 38 weeks, cesarean delivery was performed without complications, cerebral angiography with embolization was planned. It was approached through the right femoral artery using continuous heparinized lavage and a coaxial navigation system. The mural-type AVM was studied, it was selectively channeled to the right posteromedial and posterolateral choroidal arteries and to the left posteromedial choroidal artery and it was embolized under control. radiological using coil spirals and Onix embolizing liquid. Immediate control showed complete closure of fistulous connections and preservation of healthy branches.

Arteriovenous Cerebral High Flow Shunts in Children: From Genotype to Phenotype.

Objective: To study the genotypes and phenotypes of cerebral arteriovenous fistulas that drain or do not drain through the vein of Galen, and true vein of Galen aneurysmal malformations, in order to determine whether genotyping could help improve classification of these malformations and their management. Methods: We carried out a retrospective review of genetic and phenotypic data in databases of four centers. All children with cerebral arteriovenous fistula or vein of Galen aneurysmal malformations aged below 18 years at onset were included. We recorded the nature of the genetic variant or absence of variant, age at onset, type of malformation, symptoms at onset (hemorrhage, neurological deficit, hydrocephalus, incidental, and heart failure), type of venous drainage and the long-term outcome. Results: One hundred and fifteen children were included. Autosomal dominant variants were identified in 39% of patients. The most frequent variant affected was the RASA1 gene (25%) followed by EPHB4 (8%) and the HHT-associated genes (5%). HHT gene variants were only observed in pial arteriovenous fistula not draining into the vein of Galen; on the contrary, EPHB4 variants were only seen in genuine vein of Galen aneurysmal malformation. RASA1 variants were identified in all types of shunts. Conclusions: EPHB4 variants seem specific to the vein of Galen aneurysmal malformation, RASA1 variants are associated with either pial arteriovenous fistulas or with genuine VGAM and HHT gene variants seem specific to pial arteriovenous fistulas. The genetic data helps to classify these malformations and to guide treatment toward lowest risk of post-operative cerebral ischemic-hemorrhagic complications.

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.

Microsurgical Resection of a Giant Posterior Fossa Aneurysmal Malformation in a 21-Month-Old.

Pediatric aneurysms commonly occur in the vertebrobasilar circulation with complex morphologies.1 "Aneurysmal malformations," or fistulous vessel dilations without a nidus, have also been described.2 Vessel friability and sensitivity to blood loss can complicate surgery. A 21-month-old male with motor and speech delay was found to have a giant posterior fossa aneurysmal malformation. He was lethargic, with minimal speech, and moved all extremities with mild hypotonia. Imaging demonstrated a 6.9 × 5.1 × 4.6 cm aneurysm arising from a fenestrated right V4 segment. This communicated via a single connection with the deep venous system, draining through the superior vermian cistern veins, posterior mesencephalic vein, basal vein of Galen, and inferior sagittal sinus, consistent with an arteriovenous fistula with secondary aneurysmal dilatation. Endovascular sacrifice was not feasible, in addition to concern for swelling after embolization. Three-dimensional modeling confirmed close proximity of the single inflow and outflow tracts. A suboccipital and left far lateral craniotomy for clip trapping and excision of the aneurysmal arteriovenous malformation was performed in a lateral position to completely decompress the brainstem (Video 1). Angiography before closure and postoperative vascular imaging demonstrated complete aneurysmal resection and fistula disconnection, with patency of normal vasculature. The postoperative course was notable for transient swallowing difficulties likely from lower cranial nerve irritation and refractory hydrocephalus requiring a shunt. The patient was meeting all developmental milestones at 2-year follow-up. This case highlights the complex vascular pathology often seen in pediatric patients, as well as the importance of presurgical planning and careful microsurgical technique in achieving a successful outcome.

Vein of Galen aneurysm, dilated cardiomyopathy, and slender habitus in a patient with a recurrent pathogenic variant in PACS2.

The PACS2 gene encodes a multifunctional sorting protein involved in nuclear gene expression and pathway traffic regulation that has been shown to be highly expressed during human prenatal brain development. Pathogenic variants in PACS2 have been recently shown to be implicated in a phenotype with global developmental delay/intellectual disability, seizures, autistic traits, facial dysmorphic features, and cerebellar dysgenesis. Here, we report a 25-year-old male with intellectual disability, epileptic encephalopathy, cerebellar dysgenesis, facial dysmorphism, and a previously reported pathogenic variant in PACS2. To our knowledge, this is the oldest patient reported who, in addition to the known phenotype described in PACS2 patients, presented with a vein of Galen malformation and dilated cardiomyopathy as previously unreported findings.

Recurrent Vein of Galen Aneurysmal Malformation as a Presentation of Hereditary Hemorrhagic Telangiectasia.

Introduction: Vein of Galen malformation (VGM) results from an aneurysmal aberration with an arteriovenous shunting of blood and is the most frequent arteriovenous malformation in infants and fetuses. The congenital malformation develops during weeks 6-11 of fetal development. Infants often die from high-output congestive heart failure. VGM is mostly considered as a sporadic condition with minimal recurrence risk in subsequent pregnancies. Mendelian forms of VGM have rarely been described as infrequent phenotypic presentations of 2 disorders: capillary malformation-arteriovenous malformation syndrome (RASA1, EPHB4) and hereditary hemorrhagic telangiectasia (ENG, ACVRL1, and SMAD4), both showing autosomal dominant inheritance. Case Presentation: Here, we report on a consanguineous couple with recurrent VGM in 2 pregnancies. Both partners were found to be affected by hereditary hemorrhagic telangiectasia due to a known pathogenic heterozygous c.790G>A (p.Asp264Asn) variant in ENG. Fetal DNA was unavailable, however in view of the mild phenotype in the couple, along with the severe prenatal presentation in 2 pregnancies, the fetus was presumed to be homozygous for the ENG variant. A subsequent pregnancy revealed a fetus heterozygous for the variant, which had an uneventful perinatal course. Conclusion: This report highlights a severe perinatal lethal phenotype due to biallelic variants in a gene hitherto known to cause an autosomal dominant disorder.

Vein of Galen aneurysmal malformation presenting as severe heart failure in a neonate.

The vein of Galen aneurysmal malformation (VGAM) is a rare cerebral arteriovenous malformation that can be life-threatening if not diagnosed and treated early. VGAM usually presents in the neonatal period with high-output cardiac failure. We report the case of a full-term male neonate who presented with respiratory distress, and a fontanel bruit soon after birth. A chest radiograph revealed marked cardiomegaly. Transthoracic echocardiography showed dilatation of all four cardiac chambers and a patent ductus arteriosus. Transfontanellar doppler ultrasound and brain computed tomography confirmed the diagnosis of a VGAM. Clinical worsening took place despite aggressive hemodynamic and ventilatory support. The patient's Bicêtre Neonatal Evaluation Score for embolization was 2. Endovascular treatment could not be performed. The patient regretfully passed away. VGAM should be considered in the differential diagnosis of neonatal congestive heart failure with a structurally normal heart. Early diagnosis and treatment improve prognosis considerably.

High Output Cardiovascular Physiology and Outcomes in Fetal Diagnosis of Vein of Galen Malformation.

Vein of Galen aneurysmal malformation (VGAM) is a rare anomaly associated with poor outcomes from high output cardiac failure and neurologic complications. Studies addressing fetal cardiovascular status and outcomes in this population are limited. A single-center retrospective review was conducted on patients with a prenatal diagnosis of VGAM who underwent a fetal echocardiogram between January 2015 and July 2019. Fetal echocardiographic data, brain magnetic resonance imaging (MRI) findings and outcomes were collected. Nine fetuses [median gestational age at echocardiogram 34 (1.1) weeks] were included. All patients had superior vena cava dilation and reversal of diastolic flow in the transverse aortic arch. Median cardiothoracic (CT) ratio was 0.39 (0.09). Right ventricular (RV) and left ventricular (LV) dysfunction was present in 66% and 11% fetuses, respectively. Four out of five patients that underwent postnatal endovascular neurosurgical interventions at our center were alive at follow-up (mean 2.7 years). Of the non-survivors (n = 5), 3 received comfort care because of severe brain damage and died in the neonatal period. Non-survivors more commonly had > mild tricuspid regurgitation (TR) (40% vs. 25%) and > mild RV dilation (60% vs. 25%). Combined cardiac index (CCI) was higher in non-survivors when compared to survivors (672.7 vs. 530.2 ml/kg/min, p = 0.016). Fetuses with significant parenchymal damage on brain MRI tended to have a higher CCI than those without (979.8 vs. 605.0 ml/kg/min, p = 0.047). RV dysfunction, TR and elevated CCI are more commonly seen in non-survivors with VGAM. A higher CCI is seen in those deemed untreatable due to significant parenchymal volume loss. Future multicenter studies are needed to assess for prenatal prediction of outcomes in this high-risk population.

Spontaneous regression of a vein of Galen aneurysmal malformation in a pediatric patient: illustrative case.

BACKGROUND: Vein of Galen aneurysmal malformations (VGAMs) are rare congenital intracranial vascular lesions that represent 30% of all pediatric vascular anomalies. These lesions are associated with severe manifestations, including congestive heart failure, hydrocephalus, and spontaneous hemorrhage. The mainstay of management is medical stabilization followed by endovascular embolization of the lesion. Although VGAM was first reported in 1937, there are few published cases demonstrating spontaneous regression of the lesion. OBSERVATIONS: The authors report the case of a 31-month-old female who presented with an incidentally found VGAM. After initial evaluation, including magnetic resonance imaging and angiography, the patient was lost to follow-up. Upon her return to the clinic at age 12 years, the previously identified VGAM was absent, indicative of involution of the lesion. The patient remained asymptomatic and met appropriate developmental milestones during this interval. LESSONS: This report adds a rare case of the spontaneous resolution of VGAM to the literature. This case may suggest the presence of VGAMs that are asymptomatic, undetected, and regress within the pediatric population. Future studies may benefit from identifying imaging and angiographic findings predictive of spontaneous regression. There may be a role for conservative management in particular cases of asymptomatic and medically stable children with VGAMs.