광범위한 섬유성 정맥 폐쇄를 동반한 특발성 섬유화성 종격동염 1예

Idiopathic Fibrosing Mediastinitis Causing Extensive Fibrotic Veno-occlusion with Minimal Mediastinal Involvement

특발성 섬유화성 종격동염은 일반적으로 일차적인 종격동의 섬유화 및 석회화가 종격동 기관을 침범하거나 압박함으로써 상대정맥증후군 등의 임상양상을 나타내는 질환이다. 그러나 저자등은 종격동의 침범은 아주 경미하면서도 광범위하고 심한 종격동 혈관의 섬유성 폐쇄를 동반한 특발성 섬유화성 종격동염 1예를 경험하였기에 보고하는 바이다.

A 68-year-old woman was admitted after suffering facial edema with neck vein engorgement for approximately 2 months. A chest X-ray showed a mild widening of the superior mediastinum and a luminal obliteration of the superior vena cava(SVC) was noted on a computed tomograph. Venography showed that both subclavian veins were obstructed at the level of the proximal clavicle with a nonvisualization of the SVC. The SVC, both the innominate and the left internal jugular veins were completely obstructed with extensive cord-like fibrotic changes despite the absence of mediastinal involvement. The microscopic features showed a chronic granulomatous inflammation with a fibrosis minimally invading the mediastinal fat, which is consistent with fibrosing mediastinitis.