Atlas of Nervous System Vascular Malformations: A Systematic Review

Abstract

Vascular malformations are frequent in the head and neck region, affecting the nervous system. The wide range of therapeutic approaches demand the correct anatomical, morphological, and functional characterization of these lesions supported by imaging. Using a systematic search protocol in PubMed, Google Scholar, Ebsco, Redalyc, and SciELO, the authors extracted clinical studies, review articles, book chapters, and case reports that provided information about vascular cerebral malformations, in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A total of 385,614 articles were grouped; using the inclusion and exclusion criteria, three of the authors independently selected 51 articles about five vascular cerebral malformations: venous malformation, brain capillary telangiectasia, brain cavernous angiomas, arteriovenous malformation, and leptomeningeal angiomatosis as part of Sturge-Weber syndrome. We described the next topics-"definition", "etiology", "pathophysiology", and "treatment"-with a focus on the relationship with the imaging approach. We concluded that the correct anatomical, morphological, and functional characterization of cerebral vascular malformations by means of various imaging studies is highly relevant in determining the therapeutic approach, and that new lines of therapeutic approaches continue to depend on the imaging evaluation of these lesions.

Keywords: approach; atlas; brain vascular anomalies; cerebral vascular malformations; neuroimaging.

Figure 1

Division of vascular anomalies with a focus on the sub-classifications of vascular malformations. Arteriovenous malformations are categorized in the group of simple malformations, according to ISSVA’s 2018 criteria. Note that the main topics covered in this systematic review are shown in green.

Figure 2

Studies included in the results of this study.

Figure 3

The main topics addressed in this systematic review from the points of view of the model, anatomy, magnetic resonance imaging, histopathology, clinical presentation, and common location [13]. The representative image of venous malformation shown in the figure is cavernoma; the representative image of arteriovenous malformation is a temporal lobe malformation; the representative capillary malformation is capillary telangiectasia.

Figure 4

Venous malformation in cerebellar vein, observed by T2 MRI [17].

Figure 5

Cavernous angioma in the right insula and putamen. Axial and coronal T2 magnetic resonance imaging [37].

Figure 6

(A) Cut surface of cavernous angioma showing cavity with blood debris and fibrous wall. (B) Axial T1-weighted magnetic resonance imaging with contrast showing hyperintense oval right parietal lesion in relation to the parenchyma and hypointense center with defined borders and irregular hypointense halo. (C) Histologic section of cavernous angioma showing thin-walled ectatic vessels. The white bar represents 1 cm.

Figure 7

Arteriovenous malformation of the right frontal lobe: (a) T2 MRI, axial image, shows multiple hypointense flow gaps, with a slight signal anomaly in the surrounding cerebral parenchyma (arrows); (b) with contrast, it shows an early enhancement of AVMs (arrows); (c) digital subtraction angiography of the left vertebral artery’s catheterization in the coronal plane shows the AVMs nidus (long arrow) and the drainage veins with opacification (short arrow) [8].

Figure 8

(A) Cut surface of arteriovenous malformation showing multiple vessels with variable lumens immersed in brain parenchyma. (B) Axial slice CT angiography showing a lesion composed of a vascular conglomerate that enhances the contrast medium. (C) Histological section showing vessels with variable lumens and mural thicknesses with arterialization and brain parenchyma between the vessels HE 10×. The white bar represents 1 cm.

Figure 9

CCT measuring 6 mm in right caudate nucleus, observed through T2 MRI [59].

Figure 10

(A) Simple T1-weighted axial slice MRI showing an oval lesion in the white matter of the right superior temporal gyrus, with a hyperintense center and a hypointense halo. (B) Histologic section shows telangiectasia with small, thin-walled vessels, luminal dilation, and white matter between them.

Figure 11

Leptomeningeal angiomas from ipsilateral SWS to port wine lesion (A), observed by contrast-enhanced MRI, is shown on the left side in leptomeningeal vascular malformation and gyrate atrophy. (B) CT without contrast shows calcifications and twist atrophy [66].