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致命性张力性血胸合并失血性休克:神经纤维瘤病的一种罕见并发症

Fatal Tension Hemothorax Combined With Exanguination: A Rare Complication of Neurofibromatosis.

作者信息

Bidad Roz, Hall Caroline, Blohm Eike

机构信息

University of Vermont Medical Center, Department of Emergency Medicine, Burlington, Vermont.

出版信息

Clin Pract Cases Emerg Med. 2019 Sep 18;3(4):364-368. doi: 10.5811/cpcem.2019.7.43432. eCollection 2019 Nov.

Abstract

Neurofibromatosis (NF) is a common autosomal dominant disorder that can be subdivided into type 1, type 2, and schwannomatosis. Patients with NF1 typically develop café-au-lait spots, scoliosis, and benign neurofibromas. In addition, NF1 predisposes to vascular complications including stenosis, arterial ectasia, and aneurysms. Here, we report the case of an otherwise healthy 32-year-old man who developed a fatal tension hemothorax due to vertebral artery aneurysm rupture. Based on the available literature, we discuss the presentation, workup, and available therapeutic approaches to this complication of neurofibromatosis.

摘要

神经纤维瘤病(NF)是一种常见的常染色体显性疾病,可分为1型、2型和施万细胞瘤病。NF1患者通常会出现咖啡斑、脊柱侧弯和良性神经纤维瘤。此外,NF1易引发血管并发症,包括狭窄、动脉扩张和动脉瘤。在此,我们报告一例32岁健康男性因椎动脉动脉瘤破裂导致致命性张力性血胸的病例。基于现有文献,我们讨论了神经纤维瘤病这一并发症的表现、检查方法和可用的治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2388/6861020/98d404a8a09f/cpcem-03-364-g001.jpg

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