Park Jee Ha, Lucaj Jon, Denchev Krassimir L
Anesthesiology, Wayne State University School of Medicine, Detroit, USA.
Cureus. 2022 Mar 31;14(3):e23700. doi: 10.7759/cureus.23700. eCollection 2022 Mar.
Fibrosing mediastinitis (FM) is an uncommon diagnosis that involves the proliferation of extensive, dense fibrous tissue in the mediastinum. FM accounts for less than 1% of people with prior infection by that develop hypersensitivity immune responses to antigens released during the initial exposure. Other causes include tuberculosis, blastomycosis, sarcoidosis, radiation, and idiopathic. We describe FM presenting with superior vena cava (SVC) syndrome. A 66-year-old Caucasian male presented with a one-week history of progressively worsening facial swelling associated with dysphonia, bilateral ptosis, dyspnea on exertion, and unintentional weight loss of 30 pounds within the past three months. He had a 40-pack-year smoking history and a past medical history of essential hypertension, peripheral vascular disease, and bilateral internal carotid artery stenosis. The CT chest demonstrated non-specific soft tissue extending throughout the mediastinum and towards the right hilar region, complicated by severe attenuation of the superior vena cava and a 2.4 cm × 1.6 cm necrotic lymph node. The mediastinum had hyperemic and desmoplastic changes heavily encased in venous collaterals. L4 lymph node pathological evaluation demonstrated sinus histiocytosis and reactive lymphoid hyperplasia without signs of malignancy or atypia. The patient was treated with corticosteroid and diuretic therapy to achieve intermittent symptomatic relief, but continued to decline clinically, ultimately leading to his demise. The diagnosis of FM is best obtained through CT chest with intravenous contrast to demonstrate abnormal mediastinal tissue and possible structural compromise. A biopsy of the mediastinal tissue may also help rule out malignancy. Only a few case reports have demonstrated mixed symptomatic and radiologic responses to anti-inflammatory and/or antifungal treatment. Even non-surgical and surgical interventions have shown inconsistent efficacy, with frequent restenosis warranting re-exploration.
纤维性纵隔炎（FM）是一种罕见的诊断，涉及纵隔内广泛、致密的纤维组织增生。FM在先前感染并对初次接触时释放的抗原产生超敏免疫反应的人群中占比不到1%。其他病因包括结核病、芽生菌病、结节病、放射和特发性。我们描述了以 Superior vena cava（SVC）综合征为表现的FM。一名66岁的白种男性，有一周面部肿胀逐渐加重的病史，伴有声音嘶哑、双侧上睑下垂、活动时呼吸困难，且在过去三个月内体重意外减轻30磅。他有40年的吸烟史，既往有原发性高血压、外周血管疾病和双侧颈内动脉狭窄病史。胸部CT显示非特异性软组织延伸至整个纵隔并朝向右侧肺门区域，伴有上腔静脉严重衰减和一个2.4厘米×1.6厘米的坏死淋巴结。纵隔有充血和纤维组织增生性改变，被大量静脉侧支包裹。L4淋巴结病理评估显示窦组织细胞增生和反应性淋巴样增生，无恶性或异型性迹象。患者接受了皮质类固醇和利尿剂治疗以实现间歇性症状缓解，但临床状况持续恶化，最终导致死亡。FM的诊断最好通过静脉注射造影剂的胸部CT来进行，以显示纵隔组织异常和可能的结构损害。纵隔组织活检也可能有助于排除恶性肿瘤。只有少数病例报告显示对抗炎和/或抗真菌治疗有混合的症状和放射学反应。即使是非手术和手术干预的疗效也不一致，频繁的再狭窄需要再次探查。
