Previously published in Pearl M, Gomez J, Gregg L, Gailloud P: Endovascular management of vein of Galen aneurysmal malformations. G and H: The sagittal CT venogram (G) and concurrent CT bone windows image (H) demonstrate a plexiform pattern of PFS and atretic parietal cephalocele.Ĭlassification of vein of Galen malformations. A T2 hyperintense parietal subgaleal cystic lesion ( white arrow) can be seen with an associated anomalous vertical falcine vein pointing to the scalp mass ( black arrow). D–F: An atretic parietal cephalocele in a 2-month-old female infant with a scalp mass is demonstrated in sagittal T2-weighted (D), sagittal postcontrast T1-weighted (E), and axial T2-weighted (F) images. Sagittal T2-weighted (A) and postcontrast T1-weighted (B) MR images and 3D MR venogram (C) show a T2 hyperintense parietal subgaleal cystic lesion ( white arrow) with an associated anomalous vertical falcine vein pointing to the scalp mass ( black arrow). A–C: Atretic parietal cephalocele in a 3-month-old male infant with boggy scalp mass. Demonstration of PFS architecture (Manjila grade 2A, a common subtype) with three characteristic architectural patterns: straight or vertical (A–C), angulated (D–F), and plexiform (G and H). These rare subtypes could be considered crossover lesions of the eDVA family, and they require a more detailed physiological and anatomical characterization, like variants in Spetzler-Martin grading of arteriovenous malformations (AVMs). 1B- 5), and some cisternal venous varices or related arteriovenous pathologies with cortical venous reflux ( Fig. Of note, there are some types of sinus pericranii (SP) that are associated with an intraaxial DVA ( Fig. In the eDVAs described in this article, the lesion epicenter is extraaxial, located in the convexity dura, falx, tentorium, etc., even extending to intraosseous and subgaleal sites. We believe that persistent embryonic sinuses, sinus pericranii, venous varices or aneurysmal malformations, and enlarged emissary veins comprise a spectrum of eDVAs ( Fig. Classic developmental cranial venous anomalies drain the normal brain parenchyma and are located intraaxially. However, extraaxial DVAs (eDVAs) are discussed in relatively fewer publications, and their various subdivisions are often considered as separate entities, not as part of a spectrum. M any publications over the past few decades have identified the natural history and clinical significance of classic developmental venous anomalies (DVAs) of the brain that are located intraaxially. The authors highlight the broad concept of eDVAs and hope that this work will serve as a basis for future studies investigating the role of evolving focal venous hypertension/global intracranial hypertension and possibilities of fetal surgical intervention in these cases. In recent years, many of these symptomatic venous malformations have been treated with endovascular interventions, although these techniques are still being refined. Although VOGM is the most studied and classified of the above-mentioned eDVAs, the authors believe that grouping the former with the other venous anomalies/abnormalities listed above would enable the clinician to convey the exact morphophysiological configuration of these lesions, predict their natural history with respect to evolving venous hypertension or stroke, and extrapolate invaluable insights from VOGM treatment to the treatment of other eDVAs. Some patients, including those with vein of Galen malformations (VOGMs), can present with the added systemic morbidity of a high-output cardiac failure. The role of neurointervention and/or microsurgery in specific cases such as sinus pericranii and enlarged emissary veins of the skull is highlighted.Ī better understanding of the pathophysiology and developmental anatomy of these lesions can reduce treatment morbidity and mortality. The indications and surgical management options are discussed for these individual intracranial pathologies with relevant illustrations, and a novel classification is proposed for persistent falcine sinus (PFS). The article highlights the natural history, anatomy, embryology, imaging, clinical implications, and neurosurgical significance of these lesions, which the authors believe represent a continuum, with different entities characterized by distinct embryopathologic features. This paper is a narrative review of extraaxial developmental venous anomalies (eDVAs) of the brain involving dural venous flow or sinuses: persistent embryonic sinuses, sinus pericranii, enlarged emissary veins, and venous varices or aneurysmal malformations.
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