RT Journal Article
SR Electronic
T1 Arterial Spin-Labeling MR Imaging in the Detection of Intracranial Arteriovenous Malformations in Patients with Hereditary Hemorrhagic Telangiectasia
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
SP 1019
OP 1024
DO 10.3174/ajnr.A8281
VO 45
IS 8
A1 Alyafaie, Adam
A1 Han, Woody
A1 Li, Yi
A1 Vydro, Samuel A.
A1 Vella, Maya
A1 Truong, Torianna L.
A1 Park, Lindsay
A1 Langston, Daniel
A1 Kim, Helen
A1 Conrad, Miles B.
A1 Hetts, Steven W.
YR 2024
UL http://www.ajnr.org/content/45/8/1019.abstract
AB BACKGROUND AND PURPOSE: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease that causes vascular malformations in a variety of organs and tissues, including brain AVMs. Because brain AVMs have the potential to cause disabling or fatal intracranial hemorrhage, detection of these lesions before rupture is the goal of screening MR imaging/MRA examinations in patients with HHT. Prior studies have demonstrated superior sensitivity for HHT-related brain AVMs by using postcontrast MR imaging sequences as compared with MRA alone. We now present data regarding the incremental benefit of including arterial spin-labeling (ASL) perfusion sequences as part of MR imaging/MRA screening in patients with this condition.MATERIALS AND METHODS: We retrospectively analyzed 831 patients at the UCSF Hereditary Hemorrhagic Telangiectasia Center of Excellence. Of these, 42 patients had complete MR imaging/MRA, ASL perfusion scans, and criterion-standard DSA data. Two neuroradiologists reviewed imaging studies and a third provided adjudication when needed.RESULTS: Eight patients had no brain AVMs detected on DSA. The remaining 34 patients had 57 brain AVMs on DSA. Of the 57 identified AVMs, 51 (89.5%) were detected on ASL and 43 (75.4%) were detected on conventional MR imaging/MRA sequences (P = .049), with 8 lesions detected on ASL perfusion but not on conventional MR imaging.CONCLUSIONS: ASL provides increased sensitivity for brain AVMs in patients with HHT. Inclusion of ASL should be considered as part of comprehensive MR imaging/MRA screening protocols for institutions taking care of patients with HHT.ACVRL1activin A receptor type II-like 1 geneASLarterial spin-labelingENGendoglin geneHHThereditary hemorrhagic telangiectasiaPCASLpseudocontinuous arterial spin-labeling