Radiologic-pathologic correlation
Life-threatening hemoptysis caused by chronic idiopathic pulmonary hilar fibrosis with unilateral pulmonary vein occlusion

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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.

Introduction

Mediastinal fibrosis (MF), also called fibrosing mediastinitis, is a rare chronic inflammatory condition resulting in an infiltrative tumor-like mass in the mediastinum that may compress vital structures with associated symptoms. The cause of MF, which usually runs a progressive cause, is generally unknown (idiopathic MF) although Histoplasma infection and, less frequently, tuberculosis have been shown to underlie a significant number of cases [1], [2], [3], [4]. Therapy for MF has been disappointing, with many cases having a fatal outcome. A limited variant of MF, called chronic idiopathic pulmonary hilar fibrosis (CIPHF), has been described [5]. In contrast to the more common form of MF, surgical intervention appears to be beneficial and the prognosis is more favorable in CIPHF.

We describe the case of a patient who presented with a life-threatening hemoptysis caused by complete occlusion of the right pulmonary vein. The features of the disease were consistent with the CIPHF variant of MF.

Section snippets

Case report

A 47-year-old woman was admitted for severe hemoptysis and atrial fibrillation with cardiac failure. Her previous medical history included 2 episodes of pleurisy of unknown cause 5 and 16 years before her current admission and hysterectomy 8 years before the said admission. Six years before the current admission, a regurgitant mitral valve had been replaced with a St. Jude prosthetic device. During the implantation, it was noted that the superior vena cava was markedly stenosed at its junction

Discussion

Compression of the large pulmonary vessels and bronchi is a well-recognized complication of MF [2], [6], [7], [8], [9], [10]. In a review by Berry et al [6], 11 of 140 patients had pulmonary vein stenosis, whereas tracheobronchial involvement was seen more commonly. However, the most common presentation of MF is stenosis of the superior vena cava with its associated syndrome [6]. Depending on the involved structures, other manifestations may include dyspnea, hemoptysis, and cough [6], [11].

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