• Surbhi Saini, Barbara Speller-Brown, Emily Wyse, Emily R Meier, Jessica Carpenter, Ross M Fasano, and Monica S Pearl.
• *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.
• Neurosurgery. 2015 May 1;76(5):531-8; discission 538-9; quiz 539.

BackgroundIntracranial 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.ObjectiveTo 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.MethodsA 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.ResultsFive 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%).ConclusionChildren 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.

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