Outcome of fetal Vein Galen aneurysmal malformations: a systematic review and meta-analysis

Objectives: To report the outcome of fetuses with a prenatal diagnosis of Vein of Galen (VOG) malformation. Methods: PubMed and Embase databases were searched. Random effect meta-analysis of proportions was used to analyze the data. The outcomes explored were prenatal ultrasound findings, mortality, preterm birth (PTB), abnormal neurological outcome, associated findings detected at post-natal brain imaging, need for anticonvulsant therapy, and the rate of children free from neurological impairment. Random effect meta-analysis of proportions were used to analyze the data. Results: Eleven studies (226 fetuses with a prenatal diagnosis of VOG malformation) were included. All cases were detected during the third trimester of pregnancy. Ventriculomegaly was detected in 31.8% (95% CI 27.6–47.7), cardiomegaly or other ultrasound signs of cardiac compromise in 23.1% (95% CI 14.9–32.5) and hydrops in 7.3% (95% CI 2.8–13.6) of cases. The incidence of IUD, NND, and PND was 1.5% (95% CI 0.2–4.3), 23.8% (95% CI 16.9–31.4), and 24.5% (95% CI 17.6–32.2), respectively, while 12.6% (95% CI 6.0–21.2) of pregnancies were complicated by PTB. Abnormal neurodevelopmental outcome was observed in 36.7% (95% CI 27.9–39.7) of cases, while 60.5% (95% CI 17.0–82.0) of children had abnormal findings on post-natal imaging and only 29.7% (95% CI 23.3–36.5) were free from neurological impairment after birth, although there was a wide heterogeneity in the time at follow-up between the included studies. Conclusion: VOG malformation diagnoses during fetal life is associated with a high incidence of brain damage, cardiac compromise, and abnormal neurodevelopmental outcome after birth.