CTICasesFibrosing Mediastinitis with Right Pulmonary Artery and Vein Encasement
Imaging findings
A 68-year-old woman has a small, non-enhancing right lung with an enlarged left pulmonary artery. The right pulmonary artery is patent proximally but then abruptly terminates, encased by soft tissue extending into the right hilum along with the right main bronchus, and slow flow is seen within narrowed right-sided pulmonary veins. There is mosaic attenuation in the left lung reflecting redistributed pulmonary blood flow and pulmonary hypertension, narrowing of the left hilar structures without frank stenosis, and retrograde flow in the hemiazygos system from associated brachiocephalic vein compression.
Key takeaways
This constellation is characteristic of fibrosing mediastinitis, in which encasement and occlusion of the right pulmonary artery and veins has rendered the right lung functionally non-perfused, with compensatory pulmonary hypertension developing on the left side, which receives the entire cardiac output. There is no effective treatment for the right lung parenchyma at this stage, and the patient is functionally living with a single perfused lung.
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