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

Abstract

IntroductionUnderstanding 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.MethodsWe 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), I2, p-value)ResultsThirty-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); I2 < 0.01; p < 0.001] and incomplete embolization [OR = 10.87; 95% CI (1.86–63.55); I2 < 0.01; p < 0.001] were associated with mortality. First embolization as a neonate [OR = 3.24; 95% CI (1.47–7.15); I2 < 0.01; p < 0.001], incomplete embolization [OR = 5.26; 95% CI (2.06–13.43); I2 < 0.01; p < 0.001], and heart failure at presentation [OR = 3.10; 95% CI (1.03–9.33); I2 < 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.ConclusionsWe 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.

Neurodevelopmental and genetic findings in neonates with intracranial arteriovenous shunts: A case series

“…This implies that embolization plays an important role in managing cerebral AVSs: however, beyond the good success rate of the procedure, the neurodevelopmental outcome can be affected by the size of the malformation and the systemic consequences of prolonged heart failure. Recently, Savage et al performed an individual-participant meta-analysis to identify risk factors associated with all-cause mortality and clinical outcome after VGAM endovascular embolization, including 307 subjects from different small samples ( 12 ). They found that a good clinical outcome was achieved in 68% of them.…”

“…They found that a good clinical outcome was achieved in 68% of them. Furthermore, first embolization as a neonate [OR = 6.93; 95% CI (1.99–24.08)] and incomplete embolization [OR = 10.87; 95% CI (1.86–63.55)] were significantly associated with mortality, whereas first embolization as a neonate [OR = 3.24; 95% CI (1.47–7.15)], incomplete embolization [OR = 5.26; 95% CI (2.06–13.43)], and heart failure at presentation [OR = 3.10; 95% CI (1.03–9.33)] were also significantly associated with poor clinical outcomes ( 12 ). We confirm this trend: in our cohort, the infants who required more than an embolization had a poor outcome (P1 with a low score in fine and gross motor skills, P4 died, P5 had hydrocephalus and brain atrophy, P7 had focal seizures).…”

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

“…To date, there is no reliable tool for early prediction of clinical progress and prognosis in VGAM. Current best practice is to base therapy on the severity of heart failure without assessing further objective parameters [5, 16]. However, reliable early prediction of clinical progress would be of particular therapeutic importance, as interventional treatment in a decompensated state is associated with higher complication rates and higher mortality [4].…”

“…However, it is unsuitable for early postnatal assessment, as it relies on manifest organ dysfunction, which develops over time [5, 8, 14]. No other early, bedside marker is available to predict the severity of organ dysfunction, mortality, and short-term cerebral damage on magnetic resonance imaging (MRI) [16].…”

Endothelial samplingin situenables genetic characterization of vein of Galen Malformation

“…Sequelae of prolonged venous hypertension during this period lead to permanent disability or death in ∼40% of patients. 2,3 Intervention in the neonatal stage also carries a significant risk of peri-procedural stroke and death. 4 Thus, safer and more efficacious VOGM therapies are urgently needed.…”