Life-threatening hemoptysis caused by chronic idiopathic pulmonary hilar fibrosis with unilateral pulmonary vein occlusion.
We present the case of a patient who has a life-threatening hemoptysis caused by occlusion of the right pulmonary vein owing to external compression by excessive fibrous tissue. Because the patient's lung was essentially nonfunctional and hemoptysis persisted, we performed a pneumonectomy on her. Pathological analysis of the lung showed severe hypertensive changes in the arterial and venous microvasculature of the lung. Parenchymal-pleural to intercostal systemic venous connections had developed, facilitating venous drainage of the lung circulation. Upon review of the patient's history and the pathological and radiological evidence, we concluded that the pathological changes were caused by a variant of mediastinal fibrosis termed chronic idiopathic pulmonary hilar fibrosis, of which only a few cases have been described in the literature. A detailed case history and review of the literature are presented.
den Bakker
Michael A
MA
Department of Pathology, Erasmus MC, PO Box 1738, 3000 DR Rotterdam, The Netherlands. m.denbakker@erasmusmc.nl
Thomeer
Maarten
M
Maat
Alexander P W M
AP
Groeninx van Zoelen
Christine E
CE
D003097:Collateral Circulation; D005260:Female; D006469:Hemoptysis / Q000209:etiology*; D006801:Humans; D008477:Mediastinal Diseases / Q000175:diagnosis; D008875:Middle Aged; D011658:Pulmonary Fibrosis / Q000150:complications* / Q000473:pathology*; D011668:Pulmonary Veno-Occlusive Disease / Q000150:complications*