Long-term natural history of hemorrhagic moyamoya disease in 42 patients
Long-term natural history of hemorrhagic moyamoya disease in 42 patients
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DOI：
10.3171/jns.2000.93.6.0976
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发表时间：
2000-12-01
影响因子：
4.1
通讯作者：
Yamaura, A
中科院分区：
文献类型：
--
作者：
Kobayashi, E;Saeki, N;Yamaura, A
Object.本研究的目的是描述出血性烟雾病（MMD）的长期自然史。对42例出血性MMD患者进行了回顾性分析，这些患者接受了保守治疗，未接受旁路手术。该组包括四名在再出血事件后接受间接旁路手术的患者。平均随访80.6个月。分析首次出血和反复出血的临床特点，以确定再出血和预后不良的危险因素，首次出血时CT检查以脑室内出血或不伴脑出血为主29例（69%）。在随访期间，14例患者发生了第二次出血，其中5例（35.7%）发生在首次出血后10年或更长时间。年再出血率为7.09%/人/年。第二次出血事件的特征是3例（21.4%）发生半球出血，7例（50%）发生出血类型的变化。再出血后恢复良好率由45.5%下降到21.4%，死亡率由6.8%上升到28.6%。再出血和患者年龄是不良结局的统计学显著风险因素。所有4例在第二次出血后进行间接血运重建的患者均在8年内反复出血。首次出血后很长一段时间再出血的发生并不少见。此外，第一次发作后发生的半球和出血类型的变化表明在预防重复出血时遇到的困难。
Object. The purpose of this study was to delineate the long-term natural history of hemorrhagic moyamoya disease (MMD).Methods. A retrospective review was conducted among 42 patients suffering from hemorrhagic MMD who had been treated conservatively without bypass surgery. The group included four patients who had undergone indirect bypass surgery after an episode of rebleeding. The follow-up period averaged 80.6 months. The clinical features of the first bleeding episode and repeated bleeding episodes were analyzed to determine the risk factors of rebleeding and poor outcome.Intraventricular hemorrhage with or without intracerebral hemorrhage was a dominant finding on computerized tomography scans during the first bleeding episode in 29 cases (69%). During the follow-up period, 14 patients experienced a second episode of bleeding, which occurred 10 years or longer after the original hemorrhage in five cases (35.7%). The annual rebleeding rate was 7.09%/person/year. The second bleeding episode was characterized by a change in which hemisphere bleeding occurred in three cases (21.4%) and by the type of bleeding in seven cases (50%). After rebleeding the rate of good recovery fell from 45.5% to 21.4% and the mortality rate rose from 6.8% to 28.6%. Rebleeding and patient age were statistically significant risk factors of poor outcome. All four patients in whom there was indirect revascularization after the second bleeding episode experienced a repeated bleeding episode within 8 years.Conclusions. The occurrence of rebleeding a long time after the first hemorrhagic episode was not uncommon. Furthermore, the change in which hemisphere and the type of bleeding that occurred after the first episode suggested the difficulty encountered in the prevention of repeated hemorrhage.