M S Perez Akly1,2,3, C Vazquez2,4, C H Besada5,2,3, M J Rodriguez5, M F Conde5, A R Cajal2,3,6,7, V A Peuchot2,8, D Dardik2,9, M M Baccanelli2,10,3,7, M M Serra2,4,3,7. 1. From the Department of Radiology (M.S.P.A., C.H.B., M.J.R., C.M.F.), Hospital Italiano de Buenos Aires, Buenos Aires, Argentina sliman.perez@hospitalitaliano.org.ar. 2. A.R.G. Argentine Rendu Study Group (M.S.P.A., C.V., C.H.B., A.R.C., VA.P., D.D., M.M.B., M.M.S.), Buenos Aires, Argentina. 3. Hereditary Hemorrhagic Telangiectasia Unit (M.S.P.A., C.H.B., A.R.C., M.M.B., M.M.S.) Hospital Italiano, Buenos Aires, Argentina. 4. Department of Internal Medicine (C.V., M.M.S.), Hospital Italiano, Buenos Aires, Argentina. 5. From the Department of Radiology (M.S.P.A., C.H.B., M.J.R., C.M.F.), Hospital Italiano de Buenos Aires, Buenos Aires, Argentina. 6. Translational Medicine and Biomedical Engineering Institute (A.R.C.), Buenos Aires, Argentina. 7. University Institute (A.R.C., M.M.B., M.M.S.), Hospital Italiano, Buenos Aires, Argentina. 8. Internal Medicine Research Area (V.A.P.), Hospital Italiano, Buenos Aires, Argentina. 9. From the Department of Radiology (D.D.), Clínica Instituto de Diagnóstico Sociedad Anónima (INDISA), Santiago, Chile. 10. Department of Neurosurgery (M.M.B.), Hospital Italiano, Buenos Aires, Argentina.
Abstract
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.
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.
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