Vascular Malformations



Vascular Malformations





ARTERIOVENOUS MALFORMATIONS


KEY FACTS



  • Congenital in nature, generally present in middle age (65% in patients >40 years of age).


  • Very rare, incidence of 1:100,000 individuals. Arteriovenous malformations (AVMs) represent 25% of all intracranial vascular malformations.


  • Symptoms: hemorrhage (usually parenchymal, 0.5% to 1% of these patients per year), seizures, and headaches. Increased chance of rupture during pregnancy.


  • Risk of bleeding is 2% to 3% per year, and mortality is approximately 20% to 30% per bleeding episode. Cumulative risk of bleeding is 70%.


  • Factors associated with increased risk of bleeding: deep or periventricular location, intranidal aneurysm(s), venous aneurysms, and deep venous drainage.


  • Location: more than 80% are supratentorial (especially parietal), over 80% are solitary, and 2% are multiple.


  • AVMs are solitary in 98% of patients, with multiple AVMs (2%) seen in Rendu-Osler-Weber syndrome, Wyburn-Mason syndrome, craniofacial arteriovenous metameric syndrome.


  • Major vascular supply is generally from internal carotid artery (ICA) (pial, 75%), but large AVMs may recruit external carotid artery (ECA) (dural, 15%) vessels or both (mixed, 10%) or even contralateral ICA and ECA.


  • Computed tomography (CT) shows calcification in 30% of intracranial AVMs; cyst may be seen especially after stereotactic radiosurgery.






FIGURE 12-1. Noncontrast axial T1 shows a wedge-shaped AVM in the right frontoparietal region.






FIGURE 12-2. Corresponding postcontrast T1 shows enhancement of most of the lesion.







FIGURE 12-3. Lateral DSA view, in a different patient, shows AVM nidus (arrow) being fed by branches of the MCA and immediate filling of a draining vein (arrowhead).






FIGURE 12-4. FrontalTOF MRAview, in a different patient, only faintly shows AVM nidus (arrow) due to fast flow and signal dephasing.






FIGURE 12-5. Frontal view of DSA in same patient as 12-4 shows feeding arteries, nidus, and draining vein (arrow) not seen on MRA due to fast and turbulent flow.






FIGURE 12-6. Three-dimensional DSA in a different patient shows a frontal AVM with multiple aneurysms (arrows) in feeding arteries. (See color insert)






FIGURE 12-7. Lateral DSA view, in a different patient, shows presence of AVM and an aneurysm (arrow) in draining vein.






FIGURE 12-8. Axial fluid attenuated inversion recovery (FLAIR) obtained 3 years after stereotactic radiosurgery shows no residual AVM but formation of cysts.



SUGGESTED READING

Gupta V Chugh M, Walia BS, Vaishya S, Jha AN. Use of CT angiography for anatomic localization of arteriovenous malformation nidal components. Am J Neuroradiol 2008;29:1837-1840.



CAVERNOUS MALFORMATIONS


KEY FACTS



  • Slow-flow low-pressure malformations with no normal intervening brain parenchyma.


  • 80% are supratentorial and 15% are multiple (often a familial component is present) and they may occur in the presence of developmental venous anomalies. Multiple (10% to 30%) ones maybe seen after irradiation or in a specific familial syndrome (autosomal dominant). Giant ones are more common in children.


  • Second most common intracranial vascular malformation after developmental venous anomalies.


  • Annual risk of bleeding is <1%; when it occurs, it tends to be self-limited and clinically not significant; however, risk of bleeding increases after one hemorrhage. Hemorrhagic ones typically have a surrounding rim of moderate T1 hyperintensity.


  • Most common clinical symptom is seizures (nearly 50%); most are asymptomatic.


  • Many have associated venous malformations (called “transitional” type); cavernous malformations may form from occlusion of a vein in developmental venous anomaly.


  • They are better visualized with T2* and SWI.






FIGURE 12-9. Axial noncontrast T1 shows typical “popcorn” or “mulberry” appearance of a cavernous malformation in the medial left frontal lobe.






FIGURE 12-10. Corresponding postcontrast T1 shows enhancement of the central part of the malformation.






FIGURE 12-11. Corresponding T2 shows a dark rim surrounding the lesion.






FIGURE 12-12. Corresponding T2* shows “blooming” of the rim due to magnetic susceptibility from chronic blood products.







FIGURE 12-13. Axial noncontrast CT, in a different patient, shows a dense left frontal cavernoma (arrow).






FIGURE 12-14. Axial T2*, in a different patient with familial cevernoma syndrome, shows multiple dark lesions.






FIGURE 12-15. Axial noncontrast T1, in a different patient, shows hemorrhagic cavernoma with typical hyperintense rim.






FIGURE 12-16. Axial noncontrast T1, in a different patient, shows a giant cavernoma.



SUGGESTED READING

de Souza JM, Domingues RC, Cruz LCH Jr, Domingues FS, Iasbeck T, Gasparetto EL. Susceptibility-weighted imaging for the evaluation of patients with familial cerebral cavernous malformations: a comparison with T2-weighted fast spin-echo and gradient-echo sequences. Am J Neuroradiol 2008;29:154-158.

Yun TJ, Na DG, Kwon BJ, Rho HG, Park S-H, Suh Y-L, et al. A T1 hyperintense perilesional signal aids in the differentiation of a cavernous angioma from other hemorrhagic masses. Am J Neuroradiol 2008;29:494-500.



DEVELOPMENTAL VENOUS ANOMALIES


KEY FACTS

Sep 8, 2016 | Posted by in NEUROLOGICAL IMAGING | Comments Off on Vascular Malformations

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