Purpose or Case Report: Fetal central nervous system (CNS) vascular malformations are rare, complex lesions requiring a structured, multidisciplinary approach. Fetal Magnetic Resonance Imaging (MRI) plays a pivotal role in lesion characterization, prognostic assessment, and perinatal planning.
This educational exhibit presents four representative entities—superior sagittal sinus thrombosis (SSST), vein of Galen malformation (VOGM), pial arteriovenous fistula (PAVF), and dural arteriovenous fistula (DAVF)—to illustrate a systematic MRI-based evaluation model integrating anatomic, hemodynamic, and prognostic data.
A vascular evaluation checklist is proposed for consistent interpretation, including:
Identification of abnormal arteriovenous connections (pial vs dural) and the presence or absence of a nidus.
Assessment of arterial feeders for hypertrophy or flow steal.
Evaluation of venous drainage patterns, sinus thrombosis, or aneurysmal venous dilation.
A prognostic checklist complements this approach, focusing on biomarkers of adverse outcome: parenchymal volume loss, white matter abnormalities (including Wallerian degeneration), irregular cortical contour, ventriculomegaly, intracranial hemorrhage, and signs of cardiac overload. In VOGM, a straight or falcine sinus diameter >7 mm identifies high-risk neonates requiring early intervention.
Dedicated fetal vascular MRI sequences—notably 3D time-of-flight (TOF) and angiographic/venographic acquisitions—are crucial for delineating arterial feeders, draining sinuses, and confirming arterialized flow. These sequences improve risk stratification, particularly in high-flow lesions such as VOGM and PAVF, where early recognition of parenchymal injury and hemodynamic impact is essential.
Finally, the integration of genetic testing (e.g., EPHB4, RASA1, ENG, COL4A1/2) refines classification, guides counseling, and identifies syndromic associations.
Overall, combining a structured MRI checklist with prognostic and genetic assessment enables accurate diagnosis, meaningful prenatal counseling, and standardized multidisciplinary management of fetal neurovascular malformations.
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