PT - JOURNAL ARTICLE AU - M.S. Perez Akly AU - C. Vazquez AU - C.H. Besada AU - M.J. Rodriguez AU - M.F. Conde AU - A.R. Cajal AU - V.A. Peuchot AU - D. Dardik AU - M.M. Baccanelli AU - M.M. Serra TI - Prevalence of Intracranial Aneurysms in Hereditary Hemorrhagic Telangiectasia: Report from a Single Reference Center AID - 10.3174/ajnr.A7505 DP - 2022 Jun 01 TA - American Journal of Neuroradiology PG - 844--849 VI - 43 IP - 6 4099 - http://www.ajnr.org/content/43/6/844.short 4100 - http://www.ajnr.org/content/43/6/844.full SO - Am. J. Neuroradiol.2022 Jun 01; 43 AB - BACKGROUND AND PURPOSE: Neurologic manifestations in hereditary hemorrhagic telangiectasia include an increased incidence of brain abscesses and ischemic strokes due to paradoxic embolization in addition to a wide spectrum of symptoms and complications due to typical brain vascular malformations. Intracranial aneurysms are not part of this brain vascular malformation spectrum. The aim of this study was to determine their prevalence in patients with hereditary hemorrhagic telangiectasia.MATERIALS AND METHODS: This was a single-center, retrospective study. Adult patients from the institutional Hereditary Hemorrhagic Telangiectasia registry with a definitive diagnosis of hereditary hemorrhagic telangiectasia and an available report or angiographic imaging study were included and reviewed to determine the intracranial aneurysm prevalence. In addition, the morphologic characteristics of intracranial aneurysms and possible associated risk factors were collected.RESULTS: Two hundred twenty-eight patients were analyzed. Thirty-seven aneurysms in 33 patients (14.5%; 95% CI, 9.9%–19%) were found. The median diameter of intracranial aneurysms was 3.2 mm (interquartile range, 2.6–4.4 mm). No association between intracranial aneurysm and sex, age, or genetic background was noted. There were no subarachnoid hemorrhagic events due to intracranial aneurysm rupture.CONCLUSIONS: Due to the high prevalence of intracranial aneurysms in adult patients with hereditary hemorrhagic telangiectasia, further studies regarding bleeding risks and monitoring should be addressed.ACVRL1activin receptor-like kinase 1 or ALK-1BVMbrain vascular malformationENGendoglinHHThereditary hemorrhagic telangiectasiaIAintracranial aneurysmIQR25–75 interquartile rangeMADH4mothers against decapentaplegic drosophila homolog 4MMPmatrix metalloproteinaseTGFβ/BMPTransforming Growth Factorβ/bone morphogenetic protein