Revascularisation surgery and long-term follow-up in juvenile Moyamoya syndrome: a retrospective analysis.

Revascularisation surgery and long-term follow-up in juvenile Moyamoya syndrome: a retrospective analysis.
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青少年烟雾病综合征的血运重建手术和长期随访:回顾性分析。

DOI:
10.1007/978-3-7091-0661-7_8
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发表时间:
2011
期刊:
Acta neurochirurgica. Supplement
影响因子:
--
通讯作者:
Elke Januschek
Elke Januschek
中科院分区:
--
文献类型:
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作者:
P. Ulrich;Elke Januschek

文献摘要

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由于其在西方国家的发病率较低,医生对青少年烟雾病的认识有待提高。血运重建手术的益处仅在青少年版本的疾病中得到证实。因此,早期血运重建可以防止不可逆的缺血性缺陷和迅速进行性智力迟钝的年轻patients.From 1984年至2009年,共19名儿童(平均年龄8岁,范围1-18岁,女性占优势2:1,17/19欧洲白色患者,2/19亚洲血统的至少一个父母)治疗少年烟雾病的手术血运重建。主要症状为癫痫(17/19),其次为短暂性脑缺血发作(TIA)或持续性可逆性脑缺血性神经功能缺损(PRIND)(15/19)和精神发育迟滞(11/19)。6个月后,血管造影显示大多数患者在间接旁路术后有明显的新生血管形成。平均随访17年3个月(最长25年,最短2年)。2例患者失访。根据文献,缺血症状消除了94%的患者的血运重建手术的并发症发生率非常低,并没有持久的发病率和死亡率在任何patients.Early诊断和手术治疗似乎增强了独立的血运重建程序的类型的好处。
Due to its low incidence in Western countries, physician awareness of juvenile Moyamoya disease should be improved. The benefits of revascularisation surgery have only been proven in the juvenile version of the disease. Therefore, early revascularisation may prevent irreversible ischaemic deficits and rapidly progressive mental retardation in young patients.From 1984 to 2009, a total of 19 children (mean age 8 years, range 1–18 years, female predominance 2:1, 17/19 European white patients, 2/19 Asian origin of at least one parent) were treated for juvenile Moyamoya disease by surgical revascularisation. The leading symptoms were epilepsy (17/19), followed by transient ischaemic attacks (TIA) or prolonged reversible ischaemic neurologic deficits (PRIND) (15/19) and mental retardation (11/19). Angiography showed a clear neovascularisation in the majority of patients after indirect bypasses after 6 months. The mean follow-up was 17 years and 3 months (maximum 25 years, minimum 2 years). Two patients were lost to follow-up. In accordance with the literature, ischaemic symptoms were eliminated by the revascularisation operation in 94% of our patients with a very low rate of complications, and no lasting morbidity and mortality in any of the patients.Early diagnosis and surgical treatment seem to potentiate the benefits independently of the type of revascularisation procedure.