Clinical characteristics and leptomeningeal collateral status in pediatric and adult patients with ischemic moyamoya disease
Clinical characteristics and leptomeningeal collateral status in pediatric and adult patients with ischemic moyamoya disease
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缺血性烟雾病儿童和成人患者的临床特征和软脑膜侧支状况
DOI：
10.1111/cns.13130
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发表时间：
2020-01-01
影响因子：
5.5
通讯作者：
Duan, Lian
中科院分区：
文献类型：
--
作者：
Liu, Zhi-Wen;Han, Cong;Duan, Lian
目的既往研究发现儿童烟雾病（moyamoya disease，MMD）与成人烟雾病（moyamoya disease，MMD）的临床特征存在显著差异，但对这些差异的影响因素研究较少。我们的目的是调查儿童和成人MMD患者之间的软脑膜侧支（LMC）状态的差异，并分析LMC对临床特征和治疗预后的影响。方法回顾性分析2014年1月至2016年1月214例MMD患者的临床资料。比较儿童和成人患者的临床特征和LMC状态。根据数字减影血管造影（DSA）显示的大脑后动脉（PCA）逆行血流，将LMC状态分为好或差。结果共分析了83例儿童和131例成人（1：1.6）MMD患者。儿童患者更容易发生短暂性脑缺血发作（81%），而成人患者更容易发生梗死（51%）。关于MMD的不同阶段（早期、中期和晚期分别对应于Suzuki分期1-2、3-4和5-6），在早期，儿童患者LMC状态良好的患病率高于成人患者（P = 0.047）和中期（P = 0.001），但在晚期（P = 0.547），这些患者组之间没有差异。在中期，成人患者的术后血管造影结果（P = 0.017）比儿科患者差。在儿童和成人患者中，LMC状态差与梗死（P = 0.017）和术后结局差（P = 0.003和P = 0.043）有很强的相关性。结论儿童MMD患者较成人患者具有更高的通畅率和建立良好LMC状态的能力，LMC状态差与严重的临床症状和不良的术后结局密切相关。LMC状态可能是儿童和成人MMD患者临床特征和预后差异的重要因素。
Aim Previous studies have found significant differences in clinical characteristics between pediatric and adult moyamoya disease (MMD) patients, but few studies have focused on the factors underlying these differences. We aimed to investigate the differences in leptomeningeal collateral (LMC) status between pediatric and adult MMD patients and to analyze the effects of LMCs on clinical characteristics and therapeutic prognosis. Methods We retrospectively analyzed 214 MMD patients from January 2014 to January 2016. Clinical characteristics and LMC status were compared between the pediatric and adult patients. LMC status was graded as good or poor depending on the retrograde flow from the posterior cerebral artery (PCA) on digital subtraction angiography (DSA). Results A total of 83 pediatric and 131 adult (1:1.6) MMD patients were analyzed. Pediatric patients were more likely to experience a transient ischemic attack (81%), whereas adult patients were more likely to experience infarction (51%). Regarding the different MMD stages (the early, medium, and advanced stages corresponded to Suzuki stages 1-2, 3-4, and 5-6, respectively), the prevalence of good LMC status was higher for pediatric patients than for adult patients in the early stage (P = 0.047) and the medium stage (P = 0.001), but there were no differences between these patient groups in the advanced stage (P = 0.547). Worse postoperative angiographic outcomes (P = 0.017) were found in adult patients than in pediatric patients in the medium stage. Poor LMC status had strong correlations with infarction (P P = 0.017) and poor postoperative outcomes (P = 0.003 and P = 0.043) in both pediatric and adult patients. Conclusions Pediatric MMD patients have greater patency and a greater ability to establish good LMC status than adult patients, and poor LMC status has a strong correlation with severe clinical symptoms and poor postoperative outcomes. LMC status may be an important factor in the differences in clinical characteristics and prognosis between pediatric and adult MMD patients.