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三叉神经分布区海绵状血管畸形:侵袭性表现及治疗的病例报告

Cavernous Malformation in the Trigeminal Distribution: A Case Report of Aggressive Presentation and Management.

作者信息

Frossard João Thiago, Domingues Flavio, Neves Pedro, Canhedo Nathalie, de Souza Jorge Marcondes

机构信息

Service of Neurosurgery and Post Graduation Program of Surgical Sciences, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil.

Department of Neuroradiology, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil.

出版信息

World Neurosurg. 2016 Feb;86:514.e19-22. doi: 10.1016/j.wneu.2015.10.086. Epub 2015 Nov 5.

Abstract

BACKGROUND

Cavernous malformation (CM) is a vascular malformation found in the encephalic parenchyma, spinal cord, nerve roots, and extraneural tissue. CM in the trigeminal distribution is exquisitely uncommon and its biological behavior not completely understood. The clinical picture might be diverse, depending on the affected sector of the trigeminal architecture, and literature debating its pathobiology is scarce.

CASE DESCRIPTION

We describe a case of 56-year-old woman who presented with left trigeminal neuralgia and a rapidly growing cavernous malformation of the entire distribution of the fifth nerve. The clinical picture evolved to a progressive gait ataxia and follow-up neuroimaging showed a large intracranial mass leading to a brainstem compression. After microsurgical resection, the mass proved to be a typical CM of the trigeminal root.

CONCLUSION

We present an uncommonly aggressive progression of a CM of the trigeminal root, Gasserian ganglion, and cavernous sinus evolving to severe brainstem compression. The documentation of this unique case as well as its management is presented is discussed.

摘要

背景

海绵状血管畸形(CM)是一种在脑实质、脊髓、神经根和神经外组织中发现的血管畸形。三叉神经分布区域的CM极为罕见,其生物学行为尚未完全明确。临床表现可能多种多样,取决于三叉神经结构受影响的部位,而关于其病理生物学的文献较少。

病例描述

我们报告一例56岁女性,她患有左侧三叉神经痛,且第五神经整个分布区域有一个快速生长的海绵状血管畸形。临床症状发展为进行性步态共济失调,后续神经影像学检查显示一个巨大的颅内肿块导致脑干受压。显微手术切除后,该肿块被证实为典型的三叉神经根海绵状血管畸形。

结论

我们呈现了一例三叉神经根、半月神经节和海绵窦的CM罕见的侵袭性进展,发展为严重的脑干受压。本文讨论了这一独特病例的记录及其治疗。

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