Feng Zheng, Chang Yongquan, Fu Chao
Department of Pediatrics, The Third Bethune Hospital of Jilin University (China-Japan Union Hospital of Jilin University), Changchun, China.
Department of Neurosurgery, The Third Bethune Hospital of Jilin University (China-Japan Union Hospital of Jilin University), Changchun, China.
J Stroke. 2024 Sep;26(3):360-370. doi: 10.5853/jos.2024.02061. Epub 2024 Sep 30.
A ruptured peripheral cerebral aneurysm (PPCA) associated with moyamoya disease (MMD) is a rare but potentially life-threatening condition with controversial management strategies. We aim to summarize the clinical characteristics, treatment strategies, and prognostic factors of PPCAs in MMD.
We reviewed studies published in PubMed between 1980 and 2023 and used logistic regression analysis to identify the risk factors for adverse outcomes.
Of 425 identified studies, 48 eligible studies involving 121 participants were included in the current study. The mean age at diagnosis was 40.8±15.1 years, with a peak age of onset between 41 and 50 years. Among the identified participants, 59.6% were female, and 55.9% presented with impaired consciousness. Aneurysms were present in the posterior (35.5%) or anterior (30.6%) choroidal arteries in 66.1% of the cases, and 71.1% of the patients presented with intraventricular hemorrhage (IVH) with or without intracerebral hematoma (ICH). The treatment strategies were embolization (28.9%), direct surgery (21.5%), revascularization (22.3%), and conservation (27.3%). Favorable outcomes were achieved in 86.8% of all cases, with 97.1% for embolization, 65.4% for direct surgery, 96.3% for revascularization, and 84.8% for conservative treatment. Aneurysm rebleeding occurred in 11 (26.8%) of 41 patients managed conservatively, leading to worse outcomes in 7 patients (63.6%). Impaired consciousness (odds ratio [OR], 8.61; 95% confidence interval [CI], 2.06-36.00) and aneurysm rebleeding (OR, 16.54; 95% CI, 3.08-88.90) independently predicted poor outcomes.
PPCA should be considered in patients with hemorrhagic MMD, particularly those with IVH with or without ICH. Endovascular and bypass treatments are recommended as first-line options, with direct open surgery as an alternative in urgent hematoma evacuation cases. Detailed preoperative planning and intraoperative technical assistance are necessary to reduce procedure-related complications. Conservative management should be selected with caution because of the high risk of rebleeding and poor outcomes. Impaired consciousness and aneurysm rebleeding appeared to be independent risk factors for adverse prognoses. We emphasize that treatment selection should be personalized, and the potential benefits should be weighed against the associated risks.
与烟雾病（MMD）相关的外周脑动脉瘤破裂（PPCA）是一种罕见但可能危及生命的疾病，其治疗策略存在争议。我们旨在总结MMD中PPCA的临床特征、治疗策略和预后因素。
我们回顾了1980年至2023年在PubMed上发表的研究，并使用逻辑回归分析来确定不良结局的危险因素。
在425项已识别的研究中，本研究纳入了48项符合条件的研究，涉及121名参与者。诊断时的平均年龄为40.8±15.1岁，发病高峰年龄在41至50岁之间。在已识别的参与者中，59.6%为女性，55.9%有意识障碍。66.1%的病例中动脉瘤位于后脉络膜动脉（35.5%）或前脉络膜动脉（30.6%），71.1%的患者出现脑室内出血（IVH），伴或不伴有脑内血肿（ICH）。治疗策略包括栓塞（28.9%）、直接手术（21.5%）、血管重建（22.3%）和保守治疗（27.3%）。所有病例中86.8%取得了良好结局，其中栓塞治疗的良好结局率为97.1%，直接手术为65.4%，血管重建为96.3%，保守治疗为84.8%。41例接受保守治疗的患者中有11例（26.8%）发生动脉瘤再出血，其中7例（63.6%）结局更差。意识障碍（优势比[OR]，8.61；95%置信区间[CI]，2.06 - 36.00）和动脉瘤再出血（OR，16.54；95%CI，3.08 - 88.90）独立预测不良结局。
出血性MMD患者，尤其是伴有或不伴有ICH的IVH患者，应考虑PPCA。推荐血管内和搭桥治疗作为一线选择，在紧急血肿清除病例中，直接开放手术可作为替代方案。需要详细的术前规划和术中技术辅助以减少手术相关并发症。由于再出血风险高和结局差，应谨慎选择保守治疗。意识障碍和动脉瘤再出血似乎是不良预后的独立危险因素。我们强调治疗选择应个体化，且应权衡潜在益处与相关风险。
