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SUMMARY:
Four distinct vascular anomalies can be seen to affect the brain on fetal imaging: vein of Galen malformations, nongalenic arteriovenous pial fistulas, dural sinus malformations, and intracranial venous malformations. These congenital disorders affect the arteries and veins of the developing brain and are rarely seen beyond the neonatal stage. The 4 fetal cerebrovascular anomalies are associated with quite disparate natural histories and prognoses. MRI plays a pivotal role in the evaluation of fetuses with these conditions because of its ability to definitively establish the diagnosis, to detect subtle parenchymal injuries, to delineate the course of abnormal vessels in detail and to some extent the nature of vascular flow, and to identify ischemic, thrombotic, and hemorrhagic complications. Recently, an investigational transuterine embolization procedure targeted at treating fetuses with vein of Galen malformations who are at high risk for neonatal decompensation has emerged as a promising alternative to expectant management and postnatal embolization, with imaging being used to identify suitable patients for the intervention and in preprocedural planning. This manuscript reviews the essential imaging and clinical features of these 4 fetal neurovascular anomalies and underscores the practical aspects related to counseling, prognosis, and the multidisciplinary management of these entities.
ABBREVIATIONS:
- b-SSFP
- balanced steady-state free precession
- DSM
- dural sinus malformation
- icVM
- intracranial venous malformation
- RASA1
- RAS P21 protein activator 1
- SSFSE
- single-shot fast spin-echo
- VOGM
- vein of Galen malformation
Footnotes
This work was supported by the American Roentgen Ray Society Scholarship; Career Development award from the Office of Faculty Development at Boston Children’s Hospital; National Institute of Neurological Disorders and Stroke, grant/award Nos. R01EB031849, R01EB032366, R01HD109395, R01NS106030; National Institutes of Health Office of the Director, grant/award No. S10OD0250111; Rosamund Stone Zander Translational Neuroscience Center, Boston Children’s Hospital; National Institute of Biomedical Imaging and Bioengineering; and Eunice Kennedy Shriver National Institute of Child Health.
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