1型神经纤维瘤病患者自发性血胸1例报告

Q4 Medicine
Zohreh Kahramfar, O. Aloosh
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引用次数: 0

摘要

背景:1型神经纤维瘤病(NF-1)是一种常染色体显性遗传病,常伴有并发症,如良性和恶性肿瘤及血管受累,包括肺动脉高压、动脉狭窄和肺动脉动脉瘤。自发性血胸是NF-1的一种罕见且致命的并发症,由于血管病变,如胸内大血管狭窄或动脉瘤样改变,以及高度血管化的间充质肿瘤小血管的发育不良改变。最常见的是,肿瘤相关性血胸是由神经纤维瘤引起的,最常见的累及动脉是肋间动脉。病例介绍:一名48岁男性,一周前以呼吸困难主诉来我院就诊。他的症状开始是胸膜炎性胸痛,接着是呼吸困难。体格检查显示皮肤上有许多神经纤维瘤病变和卡萨梅-au-lait斑疹。在血胸检查时行开胸术。此外,胸膜和纤维胸的炎症均未见出血。结论:NF-1应考虑血管并发症。任何出现血流动力学紊乱或胸腔积液的患者都应及时检查和治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Spontaneous Hemothorax in a Patient with Neurofibromatosis Type 1: A Case Report
Background: Neurofibromatosis type1 (NF-1) is a hereditary autosomal dominant disease that is accompanied by complications, such as benign and malignant tumors and vascular involvement, including pulmonary hypertension, artery stenosis, and pulmonary artery aneurysm. Spontaneous hemothorax is a rare and lethal complication of NF-1 due to vasculopathy as stenosis or aneurysmal modifications of large intrathoracic vessels, and dysplastic alterations of small vessels in highly vascularized mesenchymal tumors. Most commonly, tumor-related hemothorax is due to neurofibroma, and the most frequently involved artery is the intercostal artery. Case Presentation: A 48-year-old male referred to our hospital with a chief complaint of dyspnea from a week ago. His symptoms began with pleuritic chest pain followed by dyspnea. The physical examination showed many neurofibromatosis lesions and café-au-lait macules on the skin. Thoracotomy was performed during the hemothorax examination. Moreover, inflammation of the pleura and fibrothorax were reported without any site of bleeding. Conclusion: Vascular complications should be regarded in NF-1. Any patient presents with hemodynamic disorder or pleural effusion should be examined and treated promptly.
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来源期刊
CiteScore
0.30
自引率
0.00%
发文量
16
审稿时长
8 weeks
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