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一例因纵隔纤维化导致的支气管纵隔肺静脉瘘罕见病例。

A Rare Case of Bronchomediastinal Pulmonary Vein Fistula due to Fibrosing Mediastinitis.

作者信息

Rawal Harsh, Mahajan Sugandhi, Brasch Andrea, Paul Vishesh

机构信息

Internal Medicine, University of Connecticut, Hartford, USA.

Internal Medicine, Carle Foundation Hospital, Urbana, USA.

出版信息

Cureus. 2020 Sep 14;12(9):e10439. doi: 10.7759/cureus.10439.

DOI:10.7759/cureus.10439
PMID:33072449
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7557110/
Abstract

Fibrosing mediastinitis (FM) is a rare condition with extensive proliferation of fibrous tissue in the mediastinum usually happens few years after Histoplasma infection. FM usually occurs years later after presentation of Histoplasma infection, and usually what makes patients seek medical attention are symptoms from compression and occlusion of vital mediastinal structures, such as the central airways, superior vena cava, pulmonary arteries, and veins. Rarely, heart, pericardium, coronaries, and aorta are involved. We report a case of 39-year-old-male who was admitted with fever and cough. The patient's condition worsened despite being on broad-spectrum antibiotics, with worsening encephalopathy and a new onset lower extremity weakness. Brain imaging showed multiple strokes suggestive of embolic event. CT chest/abdomen was suggestive of FM along with cavitary lung nodules and pneumomediastinum. Splenic and renal infarcts were also noted. Infective endocarditis was one of the top differential diagnosis due to multiple embolic infarcts, and hence a transesophageal echocardiography (TEE) was pursued. TEE showed a mass along with air bubbles entering the left atrium from the pulmonary vein. On re-evaluation of CT chest images, a fistula was seen extending from the mediastinum to the left main bronchus and the left upper pulmonary vein. This supported the diagnosis of FM with erosion of lymph node into the left main bronchus and left upper pulmonary artery, leading to fistula formation and subsequent systemic air embolization. The diagnosis of FM requires a multimodality approach, high clinical suspicion, and accurate history taking. Treatment mainly aims at managing the mechanical complications.

摘要

纤维性纵隔炎(FM)是一种罕见疾病,纵隔内纤维组织广泛增生,通常发生在组织胞浆菌感染数年之后。FM通常在组织胞浆菌感染出现数年之后发生,患者就医的常见原因是重要纵隔结构(如中央气道、上腔静脉、肺动脉和肺静脉)受压和阻塞所引起的症状。心脏、心包、冠状动脉和主动脉受累的情况较为罕见。我们报告一例39岁男性患者,因发热和咳嗽入院。尽管使用了广谱抗生素,患者病情仍恶化,出现脑病加重和新发下肢无力。脑部影像学检查显示多发性中风,提示栓塞事件。胸部/腹部CT提示FM,同时伴有空洞性肺结节和气纵隔。还发现了脾梗死和肾梗死。由于存在多个栓塞性梗死灶,感染性心内膜炎是主要鉴别诊断之一,因此进行了经食管超声心动图(TEE)检查。TEE显示有一个肿块,同时有气泡从肺静脉进入左心房。再次评估胸部CT图像时,发现一个瘘管从纵隔延伸至左主支气管和左上肺静脉。这支持了FM的诊断,即淋巴结侵蚀至左主支气管和左上肺动脉,导致瘘管形成及随后的系统性空气栓塞。FM的诊断需要采用多模式方法、高度的临床怀疑以及准确的病史采集。治疗主要针对处理机械性并发症。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a37/7557110/33cd069bca04/cureus-0012-00000010439-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a37/7557110/151291c1110b/cureus-0012-00000010439-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a37/7557110/66f2b6949214/cureus-0012-00000010439-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a37/7557110/b05cae15952f/cureus-0012-00000010439-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a37/7557110/33cd069bca04/cureus-0012-00000010439-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a37/7557110/151291c1110b/cureus-0012-00000010439-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a37/7557110/66f2b6949214/cureus-0012-00000010439-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a37/7557110/b05cae15952f/cureus-0012-00000010439-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7a37/7557110/33cd069bca04/cureus-0012-00000010439-i04.jpg

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