Cerebral arteriopathy (CA) in children with sickle cell disease (SCD) is classically described as chronic stenosis of arteries in the anterior brain circulation, leading to ischemic stroke. Some studies have however reported strokes in children with SCD but without CA. In order to better understand the etiology and risk factors of these strokes, we retrospectively analyzed ischemic strokes occurring in a large cohort of children over a 13 year-period. Between 2007 and 2020, 25/1500 children with SCD had an ischemic stroke in our center. Among them, 13 (52%) had CA, described as anatomical arterial stenosis, while 12 (48%) did not. Patients with stroke without CA were older than patients with stroke attributed to SCD-CA (9.0 years old vs 3.6 years old, p=0.008), and had more frequently a SC genotype (25% vs 0% respectively). Their stroke involved posterior circulation more frequently, with cerebellar involvement in 42%. Retained stroke etiologies in patients without typical SCD-related CA were reversible cerebral vasoconstriction syndrome, cerebral fat embolism, arterial thrombosis or thromboembolism, hyperviscosity, vasculitis in a context of infectious meningoencephalitis, and severe hemodynamic failure. No recurrence was observed in the 24 months following stroke, even though 67% of the patients were no longer receiving exchange transfusions in this group. In conclusion, in a cohort of pediatric SCD patients with efficient stroke screening strategy, half of occurring ischemic strokes were related to causes other than CA. They affected a different population of SCD children and systematic long-term transfusion programs may not be necessary in these cases.

中文翻译：

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镰状细胞病儿童无脑动脉病的中风：原因和治疗。

镰状细胞病（SCD）儿童的脑动脉病（CA）通常被描述为前脑循环动脉慢性狭窄，导致缺血性中风。然而，一些研究报告了患有 SCD 但没有 CA 的儿童中风。为了更好地了解这些中风的病因和危险因素，我们回顾性分析了 13 年来发生在一大群儿童中的缺血性中风。2007 年至 2020 年间，我们中心有 25/1500 名 SCD 儿童发生缺血性中风。其中，13 人（52%）患有 CA，被描述为解剖性动脉狭窄，而 12 人（48%）则没有。无 CA 的卒中患者比 SCD-CA 引起的卒中患者年龄更大（9.0 岁 vs 3.6 岁，p=0.008），并且更常见 SC 基因型（分别为 25% vs 0%）。他们的中风更频繁地累及后循环，其中小脑受累的比例为 42%。在没有典型 SCD 相关 CA 的患者中，保留的卒中病因包括可逆性脑血管收缩综合征、脑脂肪栓塞、动脉血栓形成或血栓栓塞、高粘滞血症、感染性脑膜脑炎背景下的血管炎和严重血流动力学衰竭。尽管该组中 67% 的患者不再接受换血，但在卒中后 24 个月内没有观察到复发。总之，在采用有效卒中筛查策略的儿科 SCD 患者队列中，一半的缺血性卒中发生与 CA 以外的原因有关。它们影响了不同人群的 SCD 儿童，在这些病例中可能没有必要进行系统的长期输血计划。