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Fibrosing Mediastinitis (Sarcoidosis-related) Causing Pulmonary Vein Obstruction

Fibrosing Mediastinitis (Sarcoidosis-related) Causing Pulmonary Vein Obstruction▶ Watch on YouTube — age-restricted, cannot embed here

Imaging findings

Prior CT from 2008 showed extensive calcified lymph node enlargement in the hilar and mediastinal regions, consistent with chronic granulomatous disease. Current CT demonstrated findings of interstitial lung edema (peribronchial fluid cuffs, septal lines, opaque background) in the right upper and left lower lobes, with a left pleural effusion. Detailed evaluation revealed severe narrowing of the left inferior pulmonary vein and the right superior pulmonary vein, progressing to complete occlusion of the left inferior pulmonary vein due to the fibrosing mediastinal tissue. SPECT perfusion scans confirmed absent perfusion in the affected lobes. Biopsy showed granulomatous disease, with features favoring sarcoidosis.

Key takeaways

Fibrosing mediastinitis, often a manifestation of sarcoidosis or histoplasmosis, can lead to severe pulmonary vein obstruction and resulting interstitial lung edema. The chronic, symmetric nature of the calcified lymphadenopathy often favors sarcoidosis. Stenting can be a therapeutic option for pulmonary vein stenosis/occlusion in such cases, with quantitative perfusion scans aiding in assessment.

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