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Brain capillary telangiectasias: from normal variants to disease.
Acta Neurochirurgica ( IF 1.9 ) Pub Date : 2020-03-06 , DOI: 10.1007/s00701-020-04271-3
Anthony S Larson 1, 2 , Kelly D Flemming 3 , Giuseppe Lanzino 1, 2 , Waleed Brinjikji 1, 2
Affiliation  

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.

中文翻译:

脑毛细血管毛细血管扩张:从正常变异到疾病。

背景技术脑毛细血管毛细血管扩张(BCT)是脑中小的扩张的毛细血管网络,最通常是无症状的。尽管很少见,但有症状的BCT病例已经报道,因此了解这些病变的性质以促进正确识别很重要。相对于其他颅内血管畸形,已发表文献中有关BCT的各种流行病学,影像学和病理学特征的最新信息可能不足。方法我们在PubMed数据库中搜索了有症状的BCT的先前报告。此外,对Google Scholar和PubMed进行了搜索,以审查BCTs的当前流行病学,影像学,病理学和致病性特征。结果纳入了48篇已发表的研究,总共有99例有症状表现的BCT病例。33例有症状的BCT本质上是出血性的,而66例是非出血性的。出现出血性病变的平均年龄为25.5岁,最常见的部位是幕上中枢神经系统(54.5%),运动障碍代表最常出现的症状(26.1%)。手术切除治疗了15.2%的出血性病变。在非出血性病变中,出现时的平均年龄为39.8岁,其中脑桥是最常见的病变部位(78.5%),头痛是最常见的出现症状(22.2%)。非出血性病变患者的12.1%接受了手术切除。结论尽管症状性BCT非常罕见,出血性和非出血性均可引起破坏性的神经系统后遗症,可能通过多种机制引起。尽管已在某些情况下实现了手术切除并取得了良好的效果,但这些病变大多数不需要干预。为了进一步了解这些不那么了解的血管畸形的临床,手术和病原学意义,在有或没有手术干预的情况下进一步记录症状表现至关重要。
更新日期:2020-04-20
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