Case Reports

Endobronchial Findings of Fibrosing Mediastinitis

Effrosyni D Manali MD, Cynthia P Saad MD, Georgiann Krizmanich RN, and Atul C Mehta MD

Fibrosing mediastinitis is underdiagnosed because of the nonspecific character of the present- ing symptoms. The endobronchial findings obtained via flexible bronchoscopy are not defined in the literature. We describe 3 cases of fibrosing mediastinitis, most likely caused by histoplas- mosis. All 3 patients presented with hemoptysis and were found to have tracheobronchial concentric narrowing, severe hyperemia, and mucosal edema. The hyperemic blood vessels were treated with neodymium yttrium-aluminum-garnet (Nd:YAG) laser and argon plasma coagulation. We believe that recognition of specific endobronchial findings aids in prompt diagnosis of fibrosing mediastinitis. Key words: fibrosing mediastinitis, , bronchos- copy, laser, embolization. [Respir Care 2003;48(11):1038–1042. © 2003 Daedalus Enterprises]

Introduction late sequelae is several years and the mean interval be- tween onset of symptoms and death is approximately 6 Fibrosing mediastinitis is defined as “presence of ex- years.2 cessive fibrotic tissue in the that has the ten- The physical examination, routine laboratory tests, and dency to invade and obliterate normal structures.”1 Fibro- chest radiographs are usually noncontributory and, in as- sing mediastinitis is due to an autoimmune process or, sociation with the nonspecific character of the presenting more commonly, an idiosyncratic reaction to - symptoms, frequently lead to erroneous diagnoses such as tous mediastinal .1,2 , , and . The disease was first reported by Oulmont in 1856, but Thus the diagnosis is usually delayed and requires a the role of Mycobacterium (TB) and His- high degree of clinical suspicion, especially in the areas toplasma capsulatum in its pathogenesis was not demon- endemic for TB and histoplasmosis. Diagnosis is based on strated until the late 1950s.3 computed tomography (CT) findings of excessive medi- Fibrosing mediastinitis has a benign course and pro- astinal soft tissue and calcification.1,2 Mediastinoscopy or duces nonspecific symptoms until it constricts major me- thoracotomy may be necessary to exclude malignancy and diastinal structures, such as pulmonary arteries, veins, tra- to confirm the diagnosis.3 chea, main bronchi, the superior vena cava, and the Our review of the literature found that the role of flex- esophagus. Most patients present with symptoms such as ible bronchoscopy in fibrosing mediastinitis, especially cough, dyspnea, hemoptysis, pleuritic pain, recurrent pul- with regard to the endobronchial findings, has not been monary , wheezing, dysphagia, or superior vena defined. cava syndrome. The interval between initial infection and In this report we describe 3 patients with fibrosing me- diastinitis, most likely related to histoplasmosis, who pre- sented with symptoms suggestive of airway involvement. Effrosyni D Manali MD, Cynthia P Saad MD, Georgiann Krizmanich Flexible bronchoscopy was performed and patients were RN, and Atul C Mehta MD are affiliated with the Department of Pul- found to have endobronchial abnormalities that could be monary and Critical Care Medicine, The Cleveland Clinic Foundation, explained by the pathophysiology of the disease. Cleveland, Ohio.

This research received no financial support and the authors have no financial interest in the subject matter. Case 1

Correspondence: Atul C Mehta MD, Department of Pulmonary and Crit- ical Care Medicine, Desk A-90, The Cleveland Clinic Foundation, 9500 A 31-year-old woman was admitted for respiratory fail- Euclid Avenue, Cleveland OH 44195. E-mail: [email protected]. ure secondary to pulmonary hemorrhage following a dif-

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Fig. 1. Chest computed tomogram showing extensive calcified right lymphadenopathy (arrow), replacement of the normal fat of Fig. 2. Bronchoscopy view of hyperemic, oozing mucosa with a the right hilum by soft tissue, and narrowing of the right pulmonary spider-web-like microvasculature. artery.

In the 3-year follow-up period the patient has been re- ficult flexible bronchoscopy, which was performed for re- admitted to our hospital 6 times for recurrent hemoptysis. current hemoptysis in the 6 months prior to admission. The rest of her evaluation is unchanged. She has had re- A chest CT showed extensive calcified mediastinal peated bronchial artery embolizations and neodymium yt- lymphadenopathy, replacement of the normal fat of the trium-aluminum-garnet (Nd:YAG) laser coagulation of the right hilum by soft tissue, narrowing of the right pulmo- dilated endobronchial vessels, without complications. nary artery, and calcified involving the right lower lobe (Fig. 1). The initial echocardiographic and spi- Case 2 rometric data were nonspecific. The ventilation-perfusion scan, on the contrary, revealed substantial compromise of the ventilation and perfusion of the right lung. A 59-year-old male resident of the Ohio River Valley After extubation the patient was remarkable for mild area underwent radio-frequency ablation for chronic atrial anemia and flexible bronchoscopy findings consistent with fibrillation. Several weeks after the procedure the patient concentric narrowing of the bronchial tree bilaterally from developed hemoptysis and gradually worsening dyspnea. an extrinsic source and a highly hyperemic, oozing mu- The initial evaluation included a spiral CT that showed cosa with a diffuse spider-web-like microvasculature (Fig. thickening and narrowing of the pulmonary veins, espe- 2). cially on the left. In addition the mediastinum was remark- Because of the risk of recurrent hemorrhage, we per- able for enlarged lymph nodes, particularly on the left, and formed arteriography of the thoracic vessels, which re- increased soft-tissue density (Fig. 3). The rest of the ex- vealed 2 large hypertrophied bronchial arteries arising from amination was remarkable only for multiple calcifications the right side of the aorta and substantial intrapulmonary of the spleen and a negative PPD test. shunting into the right pulmonary artery. Selective bron- The clinical evaluation, pulmonary function tests, and chial artery embolization was successful. echocardiogram were unremarkable, but the quantitative The CT and bronchoscopy findings were both consid- lung perfusion scan revealed severely decreased flow to ered secondary to fibrosing mediastinitis, most likely from the left lung. The patient underwent balloon angioplasty of histoplasmosis. The mediastinoscopy reinforced the diag- pulmonary veins and had some improvement. nosis by revealing dense and lymphoid tissue neg- Three months later the patient was reevaluated for re- ative for neoplasm or granuloma. TB etiology was further current episodes of hemoptysis and was found to have excluded by a negative purified protein derivative (PPD) restenosis of the pulmonary veins. Balloon angioplasty of tuberculin test and bronchial cultures. was repeated.

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Case 3

A 51-year-old woman was admitted for massive hemop- tysis. Flexible bronchoscopy revealed marked extrinsic compression of the right main and the bronchus intermedius as well as active bleeding from the bronchus intermedius. The chest CT showed an infiltrative calcified process within the mediastinum, around the right main bronchus, and severe narrowing of the right main bronchus and bronchus intermedius. Bleeding was not controlled with conservative measures, so a right pneumonectomy was attempted but was impos- sible because of a large broncholith in the right hilum, encasing the pulmonary artery and both the pulmonary veins. Biopsies were negative for neoplasm. Postoperative course was unremarkable, and the patient was discharged under close follow-up orders. A few months later the patient was readmitted for an- Fig. 3. Chest computed tomogram showing enlarged mediastinal other episode of hemoptysis. Flexible bronchoscopy re- lymph nodes (long arrow) and increased soft tissue narrowing of vealed total obstruction of the right middle and lower lobes the pulmonary veins, especially on the left (short arrow). by an extrinsic process and an oozing mucosa (Fig. 5). The patient underwent embolization of right intercostal arter- Flexible bronchoscopy showed a severely hyperemic, ies, which were supplying collateral flow to the bronchial “bleed on touch” mucosa with diffuse dilated telangiec- arteries of the right lung. In the last 3 years the patient has tatic vessels involving the left main bronchus (Fig. 4). undergone 3 more bronchial artery embolizations for re- The bronchoscopy and CT findings were thought to be current hemoptysis, without complications. due to fibrosing mediastinitis from histoplasmosis. The Discussion evaluation was negative for TB, lymphoma, or neoplasm. He underwent argon plasma coagulation of the hyperemic Endoscopic findings of fibrosing mediastinitis have sel- blood vessels, without complications, and repeated bal- dom been described in the literature.4,5 In our patients they loon angioplasty of the occluded pulmonary veins.

Fig. 4. Bronchoscopy view of “bleed on touch” mucosa and dif- Fig. 5. Bronchoscopy view of complete extrinsic compression of fuse dilated vessels. the right middle lobe and oozing mucosa.

1040 RESPIRATORY CARE • NOVEMBER 2003 VOL 48 NO 11 ENDOBRONCHIAL FINDINGS OF FIBROSING MEDIASTINITIS consisted of tracheobronchial concentric narrowing with bolism and to study any compromise in the blood flow to severe hyperemia and mucosal edema. They mostly mim- either lung. Cardiac catheterization is performed in se- icked or chronic and extrinsic compres- lected cases, to look for .14,15 sion due to mediastinal lymphadenopathy of infectious or Management can be challenging, as the hemoptysis is neoplastic etiology, which can easily be ruled out by a usually severe and recurrent. Unfortunately, there is no proper history and physical examination. However, gran- evidence that complicated fibrosing mediastinitis due to ulomatous infection and neoplasm need further evaluation. histoplasmosis responds to any kind of antifungal or cor- TB is reported to cause fibrosing mediastinitis, especially ticosteroid treatment.16 Corticosteroids and tamoxifen have in areas with high prevalence of TB.6 Ruling out this con- been used to treat the idiopathic form of fibrosing medi- dition is very important if the patient is to be treated with astinitis, with various results.17–20 Surgical intervention is corticosteroids. All our patients had a negative PPD test, technically impossible and hazardous.12 Bronchial artery no history of exposure, and negative cultures for M. tu- embolization is useful for managing severe hemoptysis; it berculosis. On the other hand their epidemiologic charac- achieves immediate control of bleeding in 75–90% of pa- teristics strongly supported an etiology of histoplasmosis. tients.21–24 In complicated fibrosing mediastinitis due to They were all natives of the Ohio River Valley area, which histoplasmosis, the recurrence of hemoptysis remains ex- is among the highly endemic areas of the United States for tremely high, because the primary fibrotic process does H. capsulatum. In that area skin test reaction to histoplas- not respond to any treatment and nonbronchial collateral min is present in over 80% of the population.7 Serology vessels also contribute to bleeding.25,26 tests are helpful only with the active forms of the disease In our cases Nd:YAG laser coagulation of endobron- so they were not performed with our patients.8 chial vessels improved outcome. The Nd:YAG laser’s su- Biopsies of the mediastinal masses were performed in 2 perior hemostatic properties and its ability to operate of the 3 patients and were useful for excluding neoplasm, through a flexible bronchoscope were the major advan- lymphoma, or active granulomatous disease. H. capsula- tages of the system,27,28 but evidence of a sustained pos- tum was not found in our patients, as expected. Histologic itive effect remains weak. In the future, argon plasma co- stains and culture for fungi are negative in most lung and agulation could be more useful.29 lymph node specimens from such patients, probably be- cause of the inactivity of infection at the time of biopsy.9 Conclusion Mucosal biopsies were not obtained, because of the high risk of bleeding.10,11 Goodwin et al reported a single case Prompt evaluation of endobronchial and CT findings in which biopsy showed chronic mucosal , could be of value in diagnosing fibrosing mediastinitis and submucosal invasion, and destruction by fibrous tissue.3 avoiding erroneous diagnoses and potentially hazardous Although radiographs, pulmonary function tests, and interventions, especially with patients residing in areas echocardiogram findings were nonspecific and therefore highly endemic for histoplasmosis. We also believe that noncontributory, the chest CT was extremely valuable in endobronchial modalities such as Nd:YAG laser and argon the diagnosis of our patients. 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