INTRODUCTION:Fibrosing mediastinitis with bronchial artery hypervascularity is a reported but rare cause of massive hemoptysis. A patient with fibrosing mediastinitis presenting with massive hemoptysis treated with external-beam radiation therapy (XRT) is discussed.
CASE PRESENTATION:A 50 year old Caucasian man with a history of fibrosing mediastinitis presented to an outside emergency department with a three day history of intermittent submassive hemoptysis. Prior to presentation, his disease had been complicated by bilateral pulmonary vascular involvement requiring left pulmonary vein stenting and subsequent therapeutic warfarin therapy. While in the emergency department, the patient developed progressive hypoxia with an oxygen saturation (SaO2) dropping to 60%, requiring supplemental O2. The patient was transferred emergently to our institution’s medical intensive care unit. Initial vital signs were heart rate 114/min, blood pressure 102/52 mmHg, temperature 37.7 °C and respiratory rate 30/min. Examination revealed an acutely ill, pale man in respiratory distress in the tripod position. Lung auscultation revealed absent breath sounds over the left chest and bronchovesicular sounds over the right chest. Cardiac examination revealed tachycardia with normal heart sounds and no murmurs. Jugulovenous pressure estimate was <8 cm. The remaining physical examination was noncontributory. Complete blood count revealed a hemoglobin of 12.2 g/dL. Complete metabolic panel was normal. Prothrombin time was 30.7 seconds, activated partial thromboplastin time was 74.5 seconds and international normalized ratio (INR) was 2.9. SaO2 on 100% O2 non-rebreather was 93%. Following arrival to the ICU, the patient had continued hemoptysis and required endotracheal intubation. Chest roentgenography demonstrated left lung atelectasis with left hilar and mediastinal prominence and presence of a left pulmonary vein stent. Thoracic computed tomographic angiography demonstrated compression the superior vena cava, right superior pulmonary vein, and the right and left pulmonary arteries. Occlusion of the left superior pulmonary vein was noted and the left inferior pulmonary vein with stent remained patent. Pneumomediastinum and intraluminal obstruction of the left mainstem bronchus with complete atelectasis of the left lung were present. Radiographic enlargement of proximal and collateral bronchial arteries was not visualized and no target vessels for bronchial artery embolization were identified. Flexible bronchoscopy demonstrated a complex thrombus occluding the left mainstem bronchus and distal divisions. Careful evacuation of the thrombus allowed visualization of an intermittently spurting ectatic bronchial artery. Despite reversal of anticoagulation, attempted endobronchial balloon tamponade and initiation of mechanical ventilation, the patient continued to have significant bleeding and hypoxia. Given failure of conservative therapy, the inability to identify the bleeding vessel radiographically and poor surgical candidacy, radiation oncology was consulted for evaluation for XRT for severe hemoptysis. The patient received 2200 Gy of thoracic XRT in six fractions over six days with subsequent cessation of hemoptysis, resolution of left lung atelectasis and hypoxemia and successful extubation. Six months following discharge, the patient remained out of the hospital and free of hemoptysis.
DISCUSSIONS:Fibrosing mediastinitis is a rare complication of histoplasmosis infection that results in an invasive, dense fibrotic capsule that progressively invades and constricts structures within the mediastinum. Endobronchial hypervascularity and friability as a result central vascular occlusion been described and are associated with hemoptysis, sometimes severe and life-threatening. XRT has been employed for malignancy-associated hemoptysis however this is the first report of XRT for massive hemoptysis associated with fibrosing mediastinitis.
CONCLUSION:Life-threatening massive hemoptysis is a known complication of fibrosing mediastinitis. XRT may represent a potential therapy for refractory hemoptysis in fibrosing mediastinitis where angiographic intervention, endobronchial tamponade or surgical resection are ineffective or contraindicated.
DISCLOSURE:Peter Crossno, No Financial Disclosure Information; No Product/Research Disclosure Information