Saini Surbhi, Speller-Brown Barbara, Wyse Emily, Meier Emily R, Carpenter Jessica, Fasano Ross M, Pearl Monica S
*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;76(5):531-8; discission 538-9; quiz 539. doi: 10.1227/NEU.0000000000000670.
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.
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.
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.
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%).
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.
颅内动脉瘤（IA）在普通儿科人群中较为罕见，占所有脑动脉瘤的比例不到2%。仅报告了7例患有镰状血红蛋白病和IA的儿童，其中大多数是在破裂后才被发现。
报告特定镰状细胞病（SCD）儿童人群中未破裂IA的患病率，并描述该患者群体中动脉瘤的形态、血液学特征及治疗情况。
对2002年1月至2013年8月在单一机构接受脑磁共振成像或血管造影的所有SCD儿童的电子数据库进行回顾性研究。审查记录中的IA、年龄、性别、镰状细胞基因型、神经系统症状、血液学指标、经颅多普勒检查结果及治疗情况。
179例接受脑磁共振成像或血管造影的SCD儿童中有5例（2.8%）被诊断为IA。均未出现蛛网膜下腔出血。4例患者（80%）患有HbSS病，1例患者患有血红蛋白镰状细胞HbSC病。共检测到18个动脉瘤；大多数患者有多个动脉瘤（80%）且双侧受累（60%）。
SCD儿童有发生多个颅内动脉瘤的风险，在解读脑部常规磁共振成像或血管造影时必须保持高度怀疑。
