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烟雾病合并豆纹动脉动脉瘤破裂:临床特征、治疗和转归

Moyamoya disease with lenticulostriate artery aneurysm rupture: clinical features, treatment and outcomes

摘要:

目的 探讨烟雾病合并豆纹动脉(lenticulostriate artery,LSA)动脉瘤破裂的临床特征、治疗策略和转归.方法 回顾性分析南京大学医学院附属鼓楼医院2012年10月至2015年3月收治的烟雾病合并LSA动脉瘤破裂患者.随访1年,应用改良Rankin量表(modified Rankin Scale,mRS)进行转归评价,0~2分定义为转归良好.总结患者的人口统计学特征、影像解剖学特征、治疗方案和转归.应用Pubmed数据库进行文献检索其他类似研究,并结合本组病例进行分析.结果 共纳入10例患者,男性2例,女性8例,年龄29~72岁,平均43.6岁.所有病例均为颅内出血,其中3例为脑叶出血,6例为脑室出血,1例为蛛网膜下腔出血.入院时7例存在意识障碍,3例进行急诊脑室外引流手术.其中,5例接受血管内栓塞,4例转归良好,1例残留轻度神经功能缺损(mRS评分3分);2例接受外科手术治疗,均转归良好;3例保守治疗,均发生再次出血,2例死亡,1例重度残疾(mRS评分4分).纳入符合要求文献14篇,总共18例患者纳入分析:外科治疗7例,血管内治疗6例,保守治疗5例(自愈1例).综合本组病例及文献报道数据,早期介入栓塞或开颅夹闭治疗患者转归良好率显著高于保守治疗患者(70.6% 对22.2%;P=0.038).结论 烟雾病合并LSA动脉瘤破裂患者保守治疗存在一定风险,需要积极治疗.介入栓塞载瘤动脉和外科夹闭动脉瘤均可有效改善该类患者的临床转归.载瘤血管条件允许时可选择同时介入栓塞载瘤动脉和动脉瘤,载瘤血管条件差且动脉瘤位于浅部时可选择外科手术.

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abstracts:

Objective To investigate the clinical features, treatment strategies and outcomes of patients with Moyamoya disease and lenticulostriate artery (LSA) aneurysm rupture. Methods Patients with moyamoya disease and LSA aneurysm rupture admitted to Nanjing Drum Tower Hospital, the Affiliated Hospital of Nanjing University Medicine School from October 2012 to March 2015 were analyzed retrospectively. They were followed up for 1 year. The modified Rankin Scale (mRS) was used to evaluate the outcomes, and 0-2 was defined as good outcome. The demographic characteristics, image anatomical features, treatment schemes, and outcomes of the patients were summarized. The Pubmed database was used to retrieve other similar studies, which combined with this group of cases for analysis. Results A total of 10 patients were enrolled, 2 males and 8 females, aged 29-72 years, with an average of 43. 6 years. All cases were intracranial hemorrhage, including 3 cases of cerebral hemorrhage, 6 cases of ventricular hemorrhage, and 1 case of subarachnoid hemorrhage. At the time of admission, 7 patients had disturbance of consciousness, and 3 patients underwent emergency extraventricular drainage. Among them, 5 patients received endovascular embolization, 4 had good outcome, 1 had mild neurological deficit (mRS score 3); 2 received surgical treatment, all had good outcome; 3 received conservative treatment, all had re-bleeding, 2 died, and 1 had severe disability (mRS score 4). Fourteen eligible articles were included, and a total of 18 patients were included in the analysis: surgical treatment in 7 cases, endovascular treatment in 6 cases, and conservative treatment in 5 cases (self-healing in 1 case). According to the data of this group of patients and literature reports, the good outcome rate of the patients with early interventional embolization or craniotomy clipping treatment was significantly higher than that of conservative treatment (70. 6%vs. 22. 2%; P = 0. 038). Conclusion There is a certain risk of conservative treatment of Moyamoya disease complicated with LSA aneurysm rupture and requires active treatment. Interventional embolization of the parent artery or surgically clipping of aneurysm can effectively improve the clinical outcome of such patients. Interventional embolization of the parent artery and aneurysm can be selected simultaneously if the condition of the parent artery is allowed. Surgery can be selected when the condition of parent artery is poor and the aneurysm is located in the superficial part.

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