Life-threatening hemoptysis caused by chronic idiopathic pulmonary hilar fibrosis with unilateral pulmonary vein occlusion.

Abstract:

: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.

journal_name

Ann Diagn Pathol

authors

den Bakker MA,Thomeer M,Maat AP,Groeninx van Zoelen CE

doi

10.1016/j.anndiagpath.2005.07.006

subject

Has Abstract

pub_date

2005-12-01 00:00:00

pages

319-22

issue

6

eissn

1092-9134

issn

1532-8198

pii

S1092-9134(05)00112-7

journal_volume

9

pub_type

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