Harman Mustafa, Sayarlioglu Mehmet, Arslan Halil, Ayakta Hayati, Harman Ece
Department of Radiology, University of Yuzuncu Yil, Faculty of Medicine, Van, Turkey.
Eur J Radiol. 2003 Nov;48(2):209-12. doi: 10.1016/S0720-048X(03)00006-8.
We present CT, MRI and venography findings in 13-year boy with mediastinal fibrosis and superior vena cava (SVC) thrombosis associated with Behçet's disease. Fibrosing mediastinitis is an excessive fibrotic reaction that occurs in the mediastinum and may lead to compression of mediastinal structures (especially vascular). This condition is usually idiopathic, though many (and perhaps most) cases in the USA are thought to be caused by an abnormal immunologic response to Histoplasma capsulatum infection. SVC syndrome secondary to extrinsic compression by mediastinal fibrosis combined with Behçet's disease has rarely been described. Radiological investigations of this syndrome are necessary to avoid a useless anticoagulant therapy.
我们展示了一名13岁男孩的CT、MRI和静脉造影检查结果，该男孩患有与白塞病相关的纵隔纤维化和上腔静脉（SVC）血栓形成。纤维性纵隔炎是一种发生在纵隔的过度纤维化反应，可能导致纵隔结构（尤其是血管）受压。这种情况通常是特发性的，不过在美国，许多（可能是大多数）病例被认为是由对荚膜组织胞浆菌感染的异常免疫反应引起的。由纵隔纤维化的外在压迫合并白塞病导致的SVC综合征很少被描述。对该综合征进行放射学检查对于避免无效的抗凝治疗是必要的。
