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症状性大或巨大毛细血管扩张症:5 例管理和结果。

Symptomatic large or giant capillary telangiectasias: management and outcome in 5 cases.

机构信息

Departments of 1 Neurosurgery and.

Department of Pathology, Peking Union Medical College Hospital; and.

出版信息

J Neurosurg. 2016 Jul;125(1):160-6. doi: 10.3171/2015.5.JNS142805. Epub 2015 Nov 13.

DOI:10.3171/2015.5.JNS142805
PMID:26566211
Abstract

Brain capillary telangiectasias (BCTs) are usually small and benign with a predilection in the pons and basal ganglion. Reports of large and symptomatic BCTs are rare. Large BCTs have a much higher risk of causing uncontrolled bleeding and severe neurological defects, and they can be fatal if left untreated. Therefore, large BCTs should be managed with special caution. Because of the lack of reports, diagnosis of large BCTs has been difficult. Strategies of management are undefined for large or giant BCTs. The current study presents 5 cases of giant and large BCTs. To the authors' knowledge, this is the largest series of this disease ever reported. Radiological findings, histopathological characteristics, clinical presentations, and surgical management were analyzed in 5 symptomatic, unusually large BCTs (mean diameter 5.06 cm, range 1.8-8 cm). Four patients presented with focal or generalized seizures, and 1 patient presented with transient vision loss attributed to the lesions. Gross-total resection of the lesion was achieved in all patients. After surgery, the 4 patients with seizures were symptom free for follow-up periods varying from more than 1 to 5 years with no additional neurological deficits. The unique location, radiological characteristics, and clinical course suggest that giant BCTs could be a different entity from small BCTs. Surgery might be a good option for treatment of patients with intractable neurological symptoms, especially in those with surgically accessible locations. Complete removal would be anticipated to provide relief of the symptoms without causing new neurological deficits.

摘要

脑毛细血管扩张症(BCT)通常较小且良性,在脑桥和基底节中较常见。大型和有症状的 BCT 报告很少见。大型 BCT 出血失控和严重神经缺陷的风险更高,如果不治疗,可能会致命。因此,应特别小心地处理大型 BCT。由于缺乏报告,因此诊断大型 BCT 一直很困难。对于大型或巨大的 BCT,管理策略尚未确定。目前的研究介绍了 5 例巨大和大型 BCT。据作者所知,这是该疾病有史以来最大的系列报道。分析了 5 例有症状的异常大型 BCT(平均直径 5.06cm,范围 1.8-8cm)的影像学发现、组织病理学特征、临床表现和手术治疗。4 例患者表现为局灶性或全身性癫痫发作,1 例患者因病变而出现短暂性视力丧失。所有患者均实现了病变的全切除。手术后,4 例有癫痫发作的患者在随访期间没有任何其他神经功能缺损,症状也无复发,随访时间从 1 年以上到 5 年不等。独特的位置、影像学特征和临床过程表明,巨大的 BCT 可能与小的 BCT 不同。手术可能是治疗难治性神经症状患者的一个很好的选择,特别是在可手术部位的患者。预期完全切除将缓解症状而不会引起新的神经功能缺损。

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