Peter Birkeland1, Kate Gardner2, Rachel Kesse-Adu2, John Davies2, Jens Lauritsen2, Frantz Rom Poulsen2, Christos M Tolias2, Swee Lay Thein2. 1. From the Department of Neurosurgery, King's College Hospital, London, United Kingdom (P.B., C.M.T.); Departments of Neurosurgery (P.B., F.R.P.) and Orthopaedic Surgery (J.L.), Odense University Hospital, Denmark; Molecular Haematology, King's College London, London, United Kingdom (K.G., S.L.T.); Department of Haematology, Guy's and St. Thomas Hospital, London, United Kingdom (R.K.-A.); Department of Anaesthetics, Derriford Hospital, Plymouth, United Kingdom (J.D.); Institute of Clinical Research, University of Southern Denmark, Odense, Denmark (J.L., F.R.P.); and Sickle Cell Branch, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (S.L.T.). Peter@Birkeland.dk. 2. From the Department of Neurosurgery, King's College Hospital, London, United Kingdom (P.B., C.M.T.); Departments of Neurosurgery (P.B., F.R.P.) and Orthopaedic Surgery (J.L.), Odense University Hospital, Denmark; Molecular Haematology, King's College London, London, United Kingdom (K.G., S.L.T.); Department of Haematology, Guy's and St. Thomas Hospital, London, United Kingdom (R.K.-A.); Department of Anaesthetics, Derriford Hospital, Plymouth, United Kingdom (J.D.); Institute of Clinical Research, University of Southern Denmark, Odense, Denmark (J.L., F.R.P.); and Sickle Cell Branch, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD (S.L.T.).
Abstract
BACKGROUND AND PURPOSE: Intracranial aneurysms and aneurysmal subarachnoid hemorrhage may occur more frequently in sickle-cell disease (SCD), and this could be related to the sickle genotype and moyamoya syndrome seen in SCD. METHODS: Records from a total of 1002 patients with SCD attending 2 specialized adult hematologic services were retrospectively reviewed. We analyzed data of a cohort of 767 patients attending 1 SCD clinic between 2002 and 2013 and of 235 patients from the other clinic who have had neurovascular imaging between 2007 and 2014. RESULTS: We identified 4 patients in the cohort who had an aneurysmal subarachnoid hemorrhage during 9063 patient-years. The highest incidence rate was seen among women in the age group 30 to 39 years with the hemoglobin SS (HbSS) genotype (440 per 100 000 patient-years). Unruptured intracranial aneurysms were found in 20 of the 324 patients, who had imaging data; the prevalence was significantly higher in patients with HbSS genotype compared with other sickle genotypes with the highest prevalence (15%) observed in women in the age group 30 to 39 years. Fifty-one HbSS patients had a moyamoya vasculopathy, but only 3 of these had concomitant intracranial aneurysms. CONCLUSIONS: Intracranial aneurysms are common in HbSS SCD. There was also a trend toward more common occurrence of aneurysmal subarachnoid hemorrhage in HbSS; women in the age group 30 to 39 years were most at risk. There was no correlation between the occurrence of intracranial aneurysms and moyamoya syndrome.
BACKGROUND AND PURPOSE:Intracranial aneurysms and aneurysmal subarachnoid hemorrhage may occur more frequently in sickle-cell disease (SCD), and this could be related to the sickle genotype and moyamoya syndrome seen in SCD. METHODS: Records from a total of 1002 patients with SCD attending 2 specialized adult hematologic services were retrospectively reviewed. We analyzed data of a cohort of 767 patients attending 1 SCD clinic between 2002 and 2013 and of 235 patients from the other clinic who have had neurovascular imaging between 2007 and 2014. RESULTS: We identified 4 patients in the cohort who had an aneurysmal subarachnoid hemorrhage during 9063 patient-years. The highest incidence rate was seen among women in the age group 30 to 39 years with the hemoglobin SS (HbSS) genotype (440 per 100 000 patient-years). Unruptured intracranial aneurysms were found in 20 of the 324 patients, who had imaging data; the prevalence was significantly higher in patients with HbSS genotype compared with other sickle genotypes with the highest prevalence (15%) observed in women in the age group 30 to 39 years. Fifty-one HbSS patients had a moyamoya vasculopathy, but only 3 of these had concomitant intracranial aneurysms. CONCLUSIONS:Intracranial aneurysms are common in HbSS SCD. There was also a trend toward more common occurrence of aneurysmal subarachnoid hemorrhage in HbSS; women in the age group 30 to 39 years were most at risk. There was no correlation between the occurrence of intracranial aneurysms and moyamoya syndrome.
Authors: Hanne Stotesbury; Jamie M Kawadler; Patrick W Hales; Dawn E Saunders; Christopher A Clark; Fenella J Kirkham Journal: Front Neurol Date: 2019-08-13 Impact factor: 4.003
Authors: Sara B Keller; Jacob M Bumpus; J Christopher Gatenby; Elizabeth Yang; Adetola A Kassim; Carlton Dampier; John C Gore; Amanda K W Buck Journal: Cardiovasc Eng Technol Date: 2021-07-20 Impact factor: 2.305