Unruptured Intracranial Aneurysms in Children With Sickle Cell Disease: Analysis of 18 Aneurysms in 5 Patients

摘要

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%).CONCLUSION: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.ABBREVIATIONS:DSA, digital subtraction angiographyHbS, abnormal sickle hemoglobinHbSC, hemoglobin S-hemoglobin CHbSS, homozygous sickle cellIA, intracranial aneurysmMRA, magnetic resonance angiographySAH, subarachnoid hemorrhageSCD, sickle cell diseaseTCD, transcranial Doppler