Objective To analyze the clinical and imaging features in patients with moyamoya disease with ≥ 2 attacks of hemorrhage. Methods The clinical data of 42 patients with moyamoya disease with ≥2 attacks of hemorrhage from December 2002 to December 2011 were analyzed retrospectively. Digital silhouette angiography (DSA) was performed in all the patients. According to the angiographic results, the differences of bleeding were compared according to the age, sites of rebleeding, modified Morioka classification (assessing the anterior choroidal artery [ AchA] on the bleeding hemispheres and the dilatation degree of the posterior communicating artery [PCoA]). Results ①Among 42 patients, bleeding occurred twice in 29 patients, 3 times in 8 patients, and ≥ 4 times in 5 patients. Seven patients were died in this group. The rebleeding mortality was 16.7% (1/42.). The mean age at rebleeding was 32. 1 ± 7. 9 years, the average rebleeding interval was 5.4 ± 1. 7 years. The age of rebleeding were 41-50 years old.Compared with other age stages, there were significant differences (P ＜0. 05) . ②The sites of rebleeding were intraventricular hemorrhage, parenchymal hemorrhage, and subarachnoid hemorrhage accounted for 47.6% , 40.5% , and 11.9% , respectively. The sites of rebleeding were exactly the same with the first bleeding in 20 patients (47. 6% ) , and different with the first sites in 22 patients (52. 4% ) . There were no significant differences (P＞0.05). ③ at the first bleeding, AchA did not develop on the bleeding he mispheres in 27 patients and 15(35.7% ) had mild or moderate vasodilation; AchA did not develop on the non-bleeding hemispheres in 34 patients, and 8 patients(19% ) had mild or moderate vasodilation. AchA-PCoA grade 1, 2, 3 on the bleeding hemispheres were 12 (28. 6% ) , 16 (38. 1% ) , and 14 (33. 3% ) , respectively; On the non-bleeding hemispheres were 18 (42.9%), 11 (26.2%), and 13 (30.9%), respectively. As compared with imaging of the bleeding hemispheres and non-bleeding hemispheres, the incidence of moderate vasodilation on the bleeding hemispheres was higher. The incidences of AchA-PCoA grade 2 -3 were higher, but there were no significant differences ( P ＞ 0. 05 ) . Conclusion The rebleeding of moyamoya disease mainly occurs in patients aged 31 to 50 years. The dilation of AchA-PCoA increases the risk of rebleeding in patients with hemorrhagic moyamoya disease, and both the bleeding and non-bleeding hemispheres may have the possibility of rebleeding. This group of patients does not show the regularity of rebleeding sites.%目的 分析烟雾病合并≥2次出血患者的临床及影像学特征.方法 回顾性分析2002年12月-2011年12月烟雾病合并≥2次出血42例患者的临床资料,对所有患者行DSA检查.根据造影结果,按其再出血的年龄、部位、改良的Morioka分级方法 [评估出血侧脉络膜前动脉(AchA)和后交通动脉(PCoA)扩张程度的分级]比较出血情况的差异.结果 ①42例患者中,有29例发生1次再出血,有8例发生 2次再出血,有5例发生≥3次再出血.全组有 7例死亡,再出血死亡率为16.7%(7/42).再出血时平均年龄为(32.1±7.9)岁,平均间隔(5.4±1.7)年后发生了再出血,发生再出血患者的年龄主要分布在41~50岁,与其他年龄段比较,差异有统计学意义(P＜ 0.05).②再出血部位在脑室的占47.6%,在脑实质的占40.5%,蛛网膜下腔出血占11.9%.再出血部位与首次出血完全相同的有20例(占47.6%),与首次出血不同有22例(占52.4%),是否合并动脉瘤差异无统计学意义(P＞ 0.05).③首次出血时,出血侧AchA未显影27例,轻度或者中度扩张15例,约占35.7%;非出血侧AchA未显影34例,轻度或者中度扩张8例,约占19.0%.出血侧AchA-PCoA扩张程度 1级12例(28.6%)、2级16例(38.1%)、3级14例(33.3%);非出血侧AchA-PCoA1级18例(42.9%)、2级11例(26.2%)、3级13例(30.9%).出血侧与非出血侧影像学比较,出血侧AchA中度扩张的发生率较高,AchA-PCoA分级2~3级发生率较高,但差异均无统计学意义(P＞0.05).结论 烟雾病再出血主要发生在31~50岁;AchA-PCoA的扩张增加了出血型烟雾病患者再出血的风险,并且出血半球和未出血半球均有再出血的可能.本组病例未显示再出血的部位有规律性.
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