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Vascular anomalies of the pediatric orbit represent a heterogeneous group that include both vascular tumors and vascular malformations. The disorder may initially be silent and then associated with symptoms and/or function damage, depending on the type of vascular anomaly and its extension. Vascular tumors include benign, locally aggressive (or borderline) and malignant forms while vascular malformations are divided into “simple”, “combined” and syndromic, or “low flow” or “high flow”. Both entities can arise in isolation or as part of syndromes. In this review, we describe the imaging findings of the vascular lesions of the orbit in the pediatric population, which are key to obtain a correct diagnosis and to guide the appropriate treatment in the light of the new genetic and molecular discoveries, and the role of the radiologist in their multidisciplinary management. We will also touch upon the main syndromes associated with orbital vascular abnormalities.

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.

Vascular anomalies of the pediatric orbit represent a heterogeneous group that include both vascular tumors and vascular malformations. The disorder may initially be silent and then associated with symptoms and/or function damage, depending on the type of vascular anomaly and its extension. Vascular tumors include benign, locally aggressive (or borderline) and malignant forms while vascular malformations are divided into "simple", "combined" and syndromic, or "low flow" or "high flow". Both entities can arise in isolation or as part of syndromes. In this review, we describe the imaging findings of the vascular lesions of the orbit in the pediatric population, which are key to obtain a correct diagnosis and to guide the appropriate treatment in the light of the new genetic and molecular discoveries, and the role of the radiologist in their multidisciplinary management. We will also touch upon the main syndromes associated with orbital vascular abnormalities., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2022 Colafati, Piccirilli, Marrazzo, Carboni, Diociaiuti, El Hachem, Esposito, Zama, Rollo, Gandolfo and Tomà.)