title: Unruptured intracranial aneurysms in children with sickle cell disease: analysis of 18 aneurysms in 5 patients.
abstract: BACKGROUND
  Intracranial aneurysms (IAs) are rare in the general pediatric population and account for <2% of all cerebral aneurysms. Only 7 children with sickle hemoglobinopathy and IAs have been reported, the majority of which were discovered after rupture.
  
  
  OBJECTIVE
  To report the prevalence of unruptured IAs in a selected population of children with sickle cell disease (SCD) and to describe the aneurysm morphology, hematologic characteristics, and management in this patient population.
  
  
  METHODS
  A retrospective review of the electronic database for all children with SCD who underwent brain magnetic resonance imaging or angiography from January 2002 to August 2013 at a single institution was performed. Records were reviewed for IA, age, sex, sickle cell genotype, neurological symptoms, hematologic indexes, transcranial Doppler findings, and management.
  
  
  RESULTS
  Five of 179 children (2.8%) with SCD imaged by brain magnetic resonance imaging or angiography were diagnosed with IAs. None presented with subarachnoid hemorrhage. Four patients (80%) had HbSS disease, and 1 patient had hemoglobin sickle cell HbSC disease. A total of 18 aneurysms were detected; the majority of patients had multiple aneurysms (80%) and bilateral involvement (60%).
  
  
  CONCLUSIONS
  Children with SCD are at risk for developing multiple intracranial aneurysms, and a high index of suspicion must be maintained during the interpretation of routine magnetic resonance imaging or angiography of the brain.
authors.affiliation: *Center for Cancer and Blood Disorders, ¶Division of Neurology, and ‖Interventional Neuroradiology, Children's National Health System, Washington, District of Columbia; ‡Department of Pediatrics, George Washington University School of Medicine and Health Sciences, Washington, District of Columbia; §Division of Interventional Neuroradiology, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Surbhi
authors.initials: S
authors.lastname: Saini
authors.forename: Barbara
authors.initials: B
authors.lastname: Speller-Brown
authors.forename: Emily
authors.initials: E
authors.lastname: Wyse
authors.forename: Emily R
authors.initials: ER
authors.lastname: Meier
authors.forename: Jessica
authors.initials: J
authors.lastname: Carpenter
authors.forename: Ross M
authors.initials: RM
authors.lastname: Fasano
authors.forename: Monica S
authors.initials: MS
authors.lastname: Pearl
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications*; D002533:Cerebral Angiography; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D002532:Intracranial Aneurysm / Q000150:complications / Q000453:epidemiology* / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D015995:Prevalence; D012189:Retrospective Studies