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脑毛细血管扩张症:从正常变异到疾病。

Brain capillary telangiectasias: from normal variants to disease.

机构信息

Mayo Clinic Department of Radiology, Mayo Clinic, 200 1st Street SW, Rochester, MN, 55905, USA.

Mayo Clinic Department of Neurosurgery, Mayo Clinic, 200 1st Street SW, Rochester, MN, 55905, USA.

出版信息

Acta Neurochir (Wien). 2020 May;162(5):1101-1113. doi: 10.1007/s00701-020-04271-3. Epub 2020 Mar 6.

DOI:10.1007/s00701-020-04271-3
PMID:32144484
Abstract

BACKGROUND

Brain capillary telangiectasias (BCTs) are small, dilated capillary networks in the brain that are most commonly asymptomatic. Though rare, symptomatic cases of BCTs have been reported, and it is therefore important to understand the nature of these lesions in order to facilitate proper recognition. Relative to other intracranial vascular malformations, updated information on the various epidemiologic, radiographic, and pathologic features of BCTs within the published literature may be inadequate.

METHODS

We searched the PubMed database for prior reports of symptomatically-manifested BCTs. Moreover, Google Scholar and PubMed were searched in order to review current epidemiologic, radiographic, pathologic, and pathogenetic features of BCTs.

RESULTS

Forty-eight published studies were included for a total of 99 individual cases of BCTs with symptomatic manifestations. Thirty-three symptomatic BCTs were hemorrhagic in nature, while 66 were non-hemorrhagic. The mean age at presentation of hemorrhagic lesions was 25.5 years, and the most common location was the supratentorial CNS (54.5%) with motor disturbance representing the most commonly encountered presenting symptom (26.1%). 15.2% of hemorrhagic lesions were treated with surgical removal. In non-hemorrhagic lesions, the mean age at presentation was 39.8 years with the pons being the most common lesion location (78.5%) and headache being the most common presenting symptom (22.2%). 12.1% of patients with non-hemorrhagic lesions were treated with surgical removal.

CONCLUSIONS

Despite their rarity, symptomatic BCTs, both hemorrhagic and non-hemorrhagic, can cause devastating neurological sequelae, potentially through multiple mechanisms. The large majority of these lesions do not require intervention, though surgical removal has been achieved with good outcome in select cases. Further documentation of symptomatic manifestations with or without surgical intervention is vital in order to further understand the clinical, surgical, and pathogenic implications of these less-appreciated vascular malformations.

摘要

背景

脑毛细血管扩张症(BCT)是脑内小的扩张毛细血管网络,通常无症状。虽然罕见,但已有症状性 BCT 的报道,因此了解这些病变的性质很重要,以便于正确识别。与其他颅内血管畸形相比,关于 BCT 的各种流行病学、影像学和病理学特征的最新信息在已发表的文献中可能不够充分。

方法

我们在 PubMed 数据库中搜索了有症状表现的 BCT 的既往报告。此外,还在 Google Scholar 和 PubMed 上进行了搜索,以回顾 BCT 的当前流行病学、影像学、病理学和发病机制特征。

结果

共纳入 48 项已发表的研究,共 99 例有症状表现的 BCT 病例。33 例有症状的 BCT 为出血性,66 例为非出血性。出血性病变的平均发病年龄为 25.5 岁,最常见的部位是幕上中枢神经系统(54.5%),最常见的首发症状是运动障碍(26.1%)。15.2%的出血性病变采用手术切除治疗。非出血性病变的平均发病年龄为 39.8 岁,最常见的病变部位是脑桥(78.5%),最常见的首发症状是头痛(22.2%)。12.1%的非出血性病变患者接受了手术切除治疗。

结论

尽管罕见,但有症状的 BCT(包括出血性和非出血性)可引起严重的神经后遗症,可能通过多种机制引起。这些病变中的绝大多数不需要干预,但在某些情况下,手术切除已取得良好效果。进一步记录有或无手术干预的症状表现对于进一步了解这些不太被重视的血管畸形的临床、手术和发病机制意义至关重要。

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