Comparison of MRI, MRA, and DSA for Detection of Cerebral Arteriovenous Malformations in Hereditary Hemorrhagic Telangiectasia

Abstract

Background and purpose: Patients with hereditary hemorrhagic telangiectasia (HHT) have a high prevalence of brain vascular malformations, putting them at risk for brain hemorrhage and other complications. Our aim was to evaluate the relative utility of MR imaging and MRA compared with DSA in detecting cerebral AVMs in the HHT population.

Materials and methods: Of 343 consecutive patients evaluated at the University of California, San Francisco HTT Center of Excellence, 63 met the study inclusion criteria: definite or probable hereditary hemorrhagic telangiectasia defined by meeting at least 2 Curacao criteria or positive genetic testing, as well as having at least 1 brain MR imaging and 1 DSA. MRIs were retrospectively reviewed, and the number of AVMs identified was compared with the number of AVMs identified on DSA.

Results: Of 63 patients, 45 (71%) had AVMs on DSA with a total of 92 AVMs identified. Of those, 24 (26%) were seen only on DSA; 68 (74%), on both DSA and MR imaging; and 5 additional lesions were seen only on MR imaging. Of the 92 lesions confirmed on DSA, 49 (53.3%) were seen on the 3D-T1 postgadolinium sequence, 52 (56.5%) were seen on the 2D-T1 postgadolinium sequence, 35 (38.0%) were seen on the SWI sequence, 24 (26.1%) were seen on T2 sequence, and 25 (27.2%) were seen on MRA. The sensitivity and specificity of MR imaging as a whole in detecting AVMs then confirmed on DSA were 80.0% and 94.4%, respectively, and the positive and negative predictive values were 97.3% and 65.4%, respectively.

Conclusions: This study reinforces the use of MR imaging as a primary screening tool for cerebral AVMs in patients with hereditary hemorrhagic telangiectasia and suggests that 3D-T1 postgadolinium and 2D-T1 postgadolinium performed at 3T are the highest yield sequences.

Fig 1.

Multiple brain AVMs in HHT: positive on MR imaging, MRA, and DSA. A 5-year-old boy with a frontopolar cerebral AVM (white arrow) demonstrated on SWI MR imaging (A), 3D postgadolinium T1 (B), time-resolved dynamic contrast-enhanced MRA (C), and DSA (D). A smaller AVM (white arrowhead) is suggested by hypointensity on SWI (A), enhancement on 3D postgadolinium T1 (B), and enhancement and subtle arteriovenous shunting on DSA (D). The smaller AVM is not seen on MRA (C).

Fig 2.

Small cerebellar AVM in HHT: positive on MR imaging, negative on MRA, and positive on DSA. A 49-year-old woman with a cerebellar hemispheric AVM (white arrow) not apparent on TOF-MRA (A) but apparent with microhemorrhage on SWI (B), on 3D postgadolinium T1 (C), and on DSA (D–F). DSA (D and F) additionally distinguishes the AVM nidus (white arrow) from the AVM draining vein (black arrowhead).

Fig 3.

Multiple brain AVMs in HHT: positive on MR imaging, MRA, and DSA. A 23-year-old woman with 2 brain AVMs identified on MRA, 7 brain AVMs identified on MR imaging, and 11 identified on DSA. Selected images demonstrate a left basal ganglia AVM (white arrow) on 3D-postgadolinium T1 (A), left ICA lateral DSA (B), left ICA anterior-posterior arterial phase DSA (E), and magnified left ICA anterior-posterior capillary phase DSA (F). Note that the angioarchitecture of the basal ganglia AVM is best seen on DSA (E and F), including a high-grade stenosis of the deep draining vein of the AVM (black arrow). Although 3D-postgadolinium T1 image of the right hemisphere (C) demonstrates 1 cortical AVM (black arrowhead), right ICA lateral DSA (D) demonstrates 2 cortical AVMs; the more anterior of these lesions (hashed arrow) was only apparent on DSA.

Fig 4.

Cerebral vascular lesion in HHT: positive on MR imaging and negative on MRA and DSA. A 72-year-old man previously treated with gamma knife radiosurgery for a left frontal AVM. T2 MR imaging (A) demonstrates gliosis in the superior frontal gyrus but no apparent AVM vessels, post-gadolinium 2D-T1 MR imaging (B) demonstrates an enhancing lesion in the superior frontal gyrus, and left ICA anterior-posterior (C) and lateral (D) DSA demonstrate no AVM.