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Review
. 2020;14(8):285-294.
doi: 10.5797/jnet.ra.2020-0020. Epub 2020 May 28.

Vascular Malformations of the Brain and Its Coverings

Dominik F Vollherbst  1 , Martin Bendszus  1 , Markus A Möhlenbruch  1
Affiliations
Review

Vascular Malformations of the Brain and Its Coverings

Dominik F Vollherbst et al. J Neuroendovasc Ther. 2020.

Abstract

Vascular malformations of the brain and its coverings encompass several different vascular pathologies of the brain and its coverings, which substantially differ in morphology, clinical presentation, and prognosis, reaching from incidental, asymptomatic vascular abnormalities to life-threatening diseases with high risks of morbidity, most frequently caused by intracranial hemorrhage. In this article, the most common vascular malformations of the brain with and without arteriovenous shunting of blood (e.g., arteriovenous malformations [AVMs], dural arteriovenous fistulas [DAVFs], and cavernous malformations) are explained with a focus on definition, diagnosis, classification, and management.

Keywords: arteriovenous malformation; dural arteriovenous fistula; hemorrhagic stroke; intracranial hemorrhage; vascular malformations.

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Conflict of interest statement

The following conflicts of interest are present: DFV has received travel support outside this work from MicroVention and Stryker GmbH & Co. KG; MB reports board membership: DSMB Vascular Dynamics; consultancy: Roche, Guerbet, Codman; grants/grants pending: DFG, Hopp Foundation, Novartis, Siemens, Guerbet, Stryker, Covidien; payment for lectures (including service on speakers bureaus): Novartis, Roche, Guerbet, Teva, Bayer, Codman; MAM has received consulting honoraria, speaker honoraria, and travel support outside this work from Codman, Covidien/Medtronic, MicroVention, Phenox, and Stryker. All other authors have nothing to disclosure.

Figures

Fig. 1
Fig. 1. Algorithm for morphology-based diagnosis of cerebral vascular malformations. AVF: arteriovenous fistula; AVM: arteriovenous malformation; DAVF: dural arteriovenous fistula; DVA: developmental venous anomaly; MRI: magnetic resonance tomography. 1“Caput medusa” or “palm tree” appearance, corresponding to several prominent vessels converging to a collecting vein; 2Contrast-enhancing patchy, brush-like lesion with blurry margins, hypointense in susceptibility-weighted imaging, typically located within the pons; 3“Popcorn” appearance with mixed signal in T2-weighted imaging and peripheral hypointensities in susceptibility-weighted imaging
Fig. 2
Fig. 2. Example of an arteriovenous malformation. Pre- (A, C) and post-interventional (B, D) images of a patient with a symptomatic, unruptured AVM. In the contrast-enhanced TOF angiography before treatment, the nidus of the AVM is visible as multiple contrast-enhancing small tubular structures, the so-called nidus (arrow in A). Pre-interventional DSA shows feeding arteries from a branch of the middle cerebral artery (white arrow in C) supplying the nidus (black arrow in C) and venous drainage into the superficial venous system (black arrowhead in C). After embolization with a liquid embolic agent, which is visible as hypointense material in MRI (arrow in B), the AVM is completely occluded (radiopaque cast of liquid embolic agent in D). AVM: arteriovenous malformation; DSA: digital subtraction angiography; MRI: magnetic resonance imaging; TOF: time-of-flight
Fig. 3
Fig. 3. Example of a DAVF. Pre- (A, C) and post-interventional (B, D) images of a patient with a symptomatic (pulsatile tinnitus) DAVF of the tentorium. MRI TOF angiography shows multiple hyperintense tubular structures around the left-sided tentorium, (arrows in A) and arterialized blood flow within adjacent dilated cortical veins (white arrowhead in A) and within the straight sinus (black arrowhead in A). In the pre-interventional DSA, multiple feeding arteries from the middle meningeal artery (white arrows in C) shunt into dilated cortical veins (black arrows in C), corresponding to a Cognard IV/Borden type III DAVF. After endovascular liquid embolization (radiopaque embolic agent: white arrows in D), the DAVF is completely occluded and the pulsatile tinnitus is no longer present. In post-interventional MRI (B), the embolic agent is visible as hypointense material (arrows in B). Pathological arterialized blood flow is now longer visible. DAVF: dural arteriovenous fistula; MRI: magnetic resonance imaging; TOF: time-of-flight
Fig. 4
Fig. 4. Example of a cavernous malformation MRI of a patient with a symptomatic (headache) cavernous malformation in the right parietal lobe. The cavernous malformation shows heterogeneous signal in T2-weighted images (A) with a popcorn-like appearance. Susceptibility-weighted imaging (B) shows the hypointense rim at the periphery of the malformation, which is typical for this disease. MRI: magnetic resonance imaging
Fig. 5
Fig. 5. Example of a developmental venous anomaly MRI of an asymptomatic patient. Contrast-enhanced T1-weighted image shows abnormal blood vessels passing through the right cerebellar hemisphere. Various blood vessels (white arrows) unite to a larger collecting vein (black arrow), resembling the head of the goddess medusa or a palm tree. MRI: magnetic resonance imaging
Fig. 6
Fig. 6. Example of a capillary telangiectasia MRI of an asymptomatic patient. In contrast-enhanced T1-weighted imaging (A) shows an area of brush-like hyperintensities in the right-sided pons, corresponding to a capillary telangiectasia. In susceptibility-weighted imaging (B) capillary telangiectasias are typically hypointense. In T2-weighted imaging (not shown), they are often invisible. MRI: magnetic resonance imaging
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