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基于31例出血型烟雾病的临床特征

Clinical features of the haemorrhage type moyamoya disease based on 31 cases.

作者信息

Kawaguchi S, Sakaki T, Kakizaki T, Kamada K, Shimomura T, Iwanaga H

机构信息

Department of Neurosurgery, Nara Medical University, Japan.

出版信息

Acta Neurochir (Wien). 1996;138(10):1200-10. doi: 10.1007/BF01809751.

DOI:10.1007/BF01809751
PMID:8955440
Abstract

We evaluated and analysed our own 31 cases of the haemorrhagic type of moyamoya disease to clarify the clinical features of this disease. The cases were divided into three groups. Group A consisted of 12 cases with aneurysms. Aneurysms on the circle of Willis were treated as ordinary saccular aneurysms. Group B consisted of 14 cases with intracerebral haemorrhage (ICH) without aneurysms. These were managed almost as spontaneous ICH. Group C consisted of 5 cases with intraventricular haemorrhage (IVH) without aneurysms or ICH. Twenty-two surgical procedures for aneurysms, ICH and IVH were done in 19 cases (62%). Nineteen procedures for preventing future strokes were undertaken in 11 cases (35%). The overall initial outcome was excellent in 12 cases (39%), good in 7 cases (23%), poor in 7 cases (23%), and death in 5 cases (15%). During the follow-up period (mean: 6.5 years), rebleeding occurred in two cases (8%), and ischaemic attacks in two cases (8%). The rate of rebleeding or ischaemic attacks was 1.19% per patient-year during the follow-up period. There was no ischaemic or rebleeding episode in cases treated by STA-MCA bypass with encephalomyosynagiosis (EMS) during the follow-up period. Management of the primary haemorrhage should be according to the clinical condition, type of haemorrhage, and source of haemorrhage. When the patient needs to undergo revascularization surgery to prevent future strokes, we recommend STA-MCA bypass with EMS instead of encephaloduro-arteriosynangiosis (EDAS).

摘要

我们评估并分析了本院收治的31例出血型烟雾病患者,以明确该疾病的临床特征。将这些病例分为三组。A组由12例伴有动脉瘤的患者组成。Willis环上的动脉瘤按普通囊状动脉瘤进行治疗。B组由14例无动脉瘤的脑内出血(ICH)患者组成。这些患者几乎按自发性ICH进行处理。C组由5例无动脉瘤或ICH的脑室内出血(IVH)患者组成。19例患者(62%)接受了针对动脉瘤、ICH和IVH的22次手术。11例患者(35%)接受了19次预防未来中风的手术。总体初始结果为12例(39%)优,7例(23%)良,7例(23%)差,5例(15%)死亡。在随访期(平均6.5年)内,2例(8%)发生再出血,2例(8%)发生缺血性发作。随访期内每位患者每年的再出血或缺血性发作发生率为1.19%。在随访期内,采用颞浅动脉-大脑中动脉搭桥联合脑-肌-血管融合术(EMS)治疗的患者未发生缺血性或再出血事件。原发性出血的处理应根据临床情况、出血类型和出血来源而定。当患者需要接受血管重建手术以预防未来中风时,我们推荐采用颞浅动脉-大脑中动脉搭桥联合EMS,而非脑-硬脑膜-动脉血管融合术(EDAS)。

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