Intracranial Aneurysms in Sickle-Cell Disease Are Associated With the Hemoglobin SS Genotype But Not With Moyamoya Syndrome.
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.



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.



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.



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.
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.
Peter
P
Birkeland
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.).
Kate
K
Gardner
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.).
Rachel
R
Kesse-Adu
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.).
John
J
Davies
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.).
Jens
J
Lauritsen
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.).
Frantz
F
Rom Poulsen
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.).
Christos M
CM
Tolias
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.).
Swee Lay
SL
Thein
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000755:Anemia, Sickle Cell / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000235:genetics; D001921:Brain / Q000000981:diagnostic imaging; D002533:Cerebral Angiography; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D005260:Female; D020022:Genetic Predisposition to Disease; D005838:Genotype; D006451:Hemoglobin, Sickle / Q000235:genetics*; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D002532:Intracranial Aneurysm / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000235:genetics; D018810:Magnetic Resonance Angiography; D008297:Male; D008875:Middle Aged; D009072:Moyamoya Disease / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000235:genetics; D015995:Prevalence; D012189:Retrospective Studies; D055815:Young Adult
D006451:Hemoglobin, Sickle
anemia, sickle cell; intracranial aneurysm; moyamoya disease; stroke; subarachnoid hemorrhage