Panagopoulos Nikolaos, Leivaditis Vasileios, Kraniotis Pantelis, Ravazoula Panagiota, Koletsis Efstratios, Dougenis Dimitrios
Department of Cardiothoracic Surgery, University Hospital of Patras, Patras, Greece.
Department of Cardiothoracic and Vascular Surgery, Westpfalz Klinikum, Kaiserslautern, Germany.
Braz J Cardiovasc Surg. 2019 Jan-Feb;34(1):85-92. doi: 10.21470/1678-9741-2018-0067.
Sclerosing mediastinitis (SM), previously named chronic fibrosing mediastinitis, is an inflammatory process that in its end-stage results to sclerosis around the mediastinal structures. SM is quite rare and has been correlated with inflammatory and autoimmune diseases, as well as malignancy. SM may either present in a mild form, with minor symptoms and a benign course or in a more aggressive form with severe pulmonary hypertension and subsequent higher morbidity and mortality. The diagnosis of SM may be difficult and quite challenging, as symptoms depend on the mediastinal structure that is mainly involved; quite often the superior vena cava. However, practically any mediastinal structure may be involved by the fibrotic process, such as the central airways, as well as the pulmonary arteries and veins, leading to obstruction or total occlusion. The latter may be impossible to undergo proper surgical excision of the lesion, and is considered to be a real challenge to the surgeon. We herein report a case of SM that presented with arterial and venous compression. The imaging appearance was that of unilateral pulmonary edema, associated with lung collapse. The case is supplemented by a non-systematic review of the relevant literature.
硬化性纵隔炎(SM),以前称为慢性纤维性纵隔炎,是一种炎症过程,在其终末期会导致纵隔结构周围硬化。SM非常罕见,与炎症性和自身免疫性疾病以及恶性肿瘤有关。SM可能以轻度形式出现,症状轻微,病程良性,也可能以更具侵袭性的形式出现,伴有严重的肺动脉高压,随后发病率和死亡率更高。SM的诊断可能困难且颇具挑战性,因为症状取决于主要受累的纵隔结构;通常是上腔静脉。然而,实际上任何纵隔结构都可能被纤维化过程累及,如中央气道以及肺动脉和静脉,导致梗阻或完全闭塞。后者可能无法进行病变的适当手术切除,被认为是外科医生面临的真正挑战。我们在此报告一例表现为动静脉受压的SM病例。影像学表现为单侧肺水肿,伴有肺萎陷。本文还对相关文献进行了非系统性综述。