Idiopathic Fibrosing Mediastinitis Research Articles

A Case Of Recurrent Constrictive Pericarditis With Diffuse Fibrosis

<h3>Background</h3> Evaluation for etiology of constrictive pericarditis is essential in guiding management. Uncommon etiologies include fibrosing mediastinitis, which is caused by proliferation of fibrous tissue in the mediastinum and Erdheim-Chester disease (ECD) which is characterized by inflammatory infiltration of lesions by histiocytes, often with involvement of the pericardium and aorta. <h3>Case</h3> A 46-year-old male with a history of idiopathic retroperitoneal fibrosis with thoracic and abdominal infiltrative masses, recurrent constrictive pericarditis and pericardial effusions, and recurrent admissions for heart failure with preserved ejection fraction (HFpEF) exacerbations presented with worsening shortness of breath and lower extremity edema. He previously underwent pericardial window and steroid therapy with minimal improvement. He was diagnosed with ECD. CT scan revealed large bilateral pleural effusions, soft tissue infiltration surrounding the heart and retroperitoneum, and diffuse pericardial thickening. Echocardiography findings were suggestive of constrictive pericarditis and reduced ejection fraction of 40%. Cardiac MRI confirmed these findings. Cardiac catheterization was consistent with constrictive physiology (Figure 1 A-H). Endomyocardial biopsies revealed scattered lymphocytes with no fibrosis or histiocyte infiltration. He underwent pericardiectomy, from the superior vena cava to the inferior vena cava and phrenic nerve to left anterior descending artery, with repeat biopsies revealing acute and chronic inflammation with extensive fibrosis. Follow up echocardiography showed improvement of septal motion variation and elimination of respiratory effect on mitral valve motions. <h3>Discussion</h3> Imaging was initially concerning for ECD given cardiac findings with retroperitoneal fibrosis. However, biopsies were inconsistent with ECD. Due to constrictive physiology, he underwent pericardiectomy with repeat biopsies revealing acute and chronic inflammation with extensive fibrosis, yielding a more likely diagnosis of idiopathic fibrosing mediastinitis. Treatment for idiopathic fibrosing mediastinitis is symptomatic relief with surgical resection. <h3>Conclusion</h3> Recurrent constrictive pericarditis with extensive multiorgan fibrosis is uncommon and ECD and fibrosing mediastinitis should be differential diagnoses in order to guide appropriate management.

Successful Stenting of Bilateral Pulmonary Veins Stenosis Secondary to Idiopathic Fibrosing Mediastinitis

An 18-year-old woman presented with dyspnea, cough, and hemoptysis for 2 years without pulmonary tuberculosis and recurrent lung infection. Computed tomography angiography revealed bilateral pulmonary veins (PVs) stenosis ([Figure 1A][1]) and multiple soft tissue shadows at posterior mediastinum ([

A Case of Pregnancy in a Patient with Idiopathic Fibrosing Mediastinitis

We report a case of a 36-years-old female with known Fibrosing Mediastinitis (FM) suspected from prior Histoplamosis infection who presented for medical induction of first pregnancy. The case centers on the outpatient and peripartum management of pregnant patients in the setting of FM with consideration given to method of delivery and anesthetic options during delivery.

Follicular dendritic sarcoma masquerading as fibrosing mediastinitis

Fibrosing mediastinitis (FM) is a rare disorder resulting from abnormal immunological-mediated fibro-proliferative reaction in the mediastinum. Here, we describe a case of a 46-year-old female with an incidentally found 11×9...

Idiopathic fibrosing mediastinitis: spectrum of imaging findings with emphasis on its association with IgG4-related disease

PurposeThe purpose of this study was to review the clinicoradiological features of idiopathic fibrosing mediastinitis (FM). MethodsThe records of six patients with idiopathic FM were retrospectively reviewed. ResultsThe most common presenting symptom was cough. There were three localized forms and three diffuse forms. Definitive histological diagnosis was most commonly made with mediastinoscopy. Interestingly, three of our patients had disease manifestations of IgG4-related disease (IgG4-RD) outside the mediastinum. ConclusionThe imaging findings of idiopathic FM vary depending on the involved mediastinal structures. Therapeutic options include medical treatment, stenting, or surgery. Radiologists should pay attention to the possible association of idiopathic FM with extrathoracic manifestations of the IgG4-RD spectrum.

Intracardiac Mass Due to Fibrosing Mediastinitis: The First Reported Case

We report a case of a woman, aged 53 years, presenting with a right atrial mass due to idiopathic fibrosing mediastinitis with periaortic involvement. This challenging diagnosis was confirmed by different imaging modalities and histopathologic analysis. The diagnosis of cardiac tumours is often difficult. To our knowledge, this is the first reported case of an intracavitary cardiac mass due to fibrosing mediastinitis. This rare disorder, which is characterized by invasive proliferation of fibrous tissue within the mediastinum, should be included in the differential diagnosis of intracardiac tumours.

Anaplastic Large Cell Lymphoma Mimicking Fibrosing Mediastinitis

Fibrosing mediastinitis is rare. One type of this disease is idiopathic fibrosing mediastinitis. It is necessary to rule out malignancy in order to accurately diagnose fibrosing mediastinitis. We herein report a case of anaplastic large cell lymphoma diagnosed three months after a preliminary diagnosis of fibrosing mediastinitis. Glucocorticoid therapy was not successful in controlling disease progression. Immediately after initiating chemotherapy for lymphoma, the patient's symptoms improved dramatically and the mediastinal lesion decreased in size. Although few similar cases have been reported, hidden malignancy may present as fibrosing mediastinitis. Therefore, physicians should consider the probability of malignancy in patients with fibrosing mediastinitis because treatments may vary accordingly.

特発性線維性縦隔炎による上大静脈症候群に合併したDownhill esophageal varicesの1例

症例は62歳，男性．特発性線維性縦隔炎による上大静脈狭窄に伴って発症した上大静脈症候群に対して，上大静脈内ステントを挿入し症状の改善を得たが，ステント挿入30ヵ月後に閉塞し，上大静脈症候群の再発が認められた．同時期に施行された上部消化管内視鏡検査にて食道全域に静脈の拡張と蛇行を認め，上大静脈症候群に伴い発症したDownhill esophageal varicesと診断した．内視鏡所見より経過観察可能と判断し慎重に経過を見たが，9年後の現在に至るまで大きな変化を認めていない．Downhill esophageal varicesは稀な疾患であるが，出血の報告もあり，上大静脈症候群症例では念頭に置く必要がある．今回，良性疾患による上大静脈症候群に発症したDownhill esophageal varicesに対して長期経過観察を行うことができたので報告する．

The Treatment of Superior Vena Cava Syndrome Secondary to Idiopathic Fibrosing Mediastinitis with Balloon Angioplasty and Stenting

The Treatment of Superior Vena Cava Syndrome Secondary to Idiopathic Fibrosing Mediastinitis with Balloon Angioplasty and Stenting

An Adolescent with Pulmonary Hypertension Secondary to Idiopathic Fibrosing Mediastinitis: Case Report

OZET Pulmonary hypertension is an important determinant of morbidity and mortality in many diseases, including congenital heart disease and respiratory disease. Fibrosing mediastinitis is a rare disorder caused by proliferation of acellular collagen. In fibrosing mediastinitis, clinical presentation is determined by compression to the vital mediastinal organs. Fibrosing mediastinitis may rarely lead to pulmonary veno-occlusive disease. In this case report, a 16 year old boy with pulmonary arterial hypertension was presented. His right pulmonary artery was completely occluded secondary to idiopathic fibrosing mediastinitis. The patient died after mediastinal biopsy. Herein, we discussed pulmonary arterial hypertension secondary to idiopathic fibrosing mediastinitis in children.

Octreotide, a Somatostatin Analogue, in the Treatment of Chylothorax Associated with Idiopathic Fibrosing Mediastinitis

Fibrosing mediastinitis (FM) is a rare benign disorder that is characterized by excessive fibrotic reactions in the mediastinum. FM is associated with various diseases, including Histoplasma capsulatum infection and IgG4-related disease, and may compromise the airways, great vessels, and other mediastinal structures. Chylothorax is not a common manifestation of FM, and there is no standard treatment for FM or chylothorax. Recently, however, somatostatin and octreotide, a somatostatin analogue, were successfully used for the treatment of chylothorax due to various causes, and they are considered as putative therapeutic interventions for chylothorax. Here, we present a 28-year-old Japanese man with chylothorax due to idiopathic FM, who was successfully treated with octreotide. The patient visited our hospital because of dyspnea on exertion. On admission, chest computed tomography revealed pericardial effusion, bilateral pleural effusion, and a mass in the mediastinum. The right pleural effusion appeared chylous, with the triglyceride level of 253 mg/dl. The biopsy specimen from the mediastinal mass showed collagenous fibers and fibroblasts with moderate infiltration of lymphocytes. Neither fungi nor bacteria were cultured from the biopsy specimen. Steroid therapy was not effective. The patient was then treated with subcutaneous octreotide (100 microg three times daily). Five days after starting the treatment, the drained pleural fluid was decreased to approximately 150 ml/day from approximately 1,000 ml/day. The mediastinal mass decreased in size 2 weeks after the initiation of octreotide treatment. After discharge, the patient has received octreotide treatment for 6 months without serious adverse events. We suggest octreotide as a treatment option for FM.

Effectiveness of Steroid Treatment for Hoarseness Caused by Idiopathic Fibrosing Mediastinitis: Report of a Case

A 65-year-old woman was referred to our department for investigation and treatment of hoarseness. A chest computed tomography (CT) scan showed a mediastinal mass spreading into the aortopulmonary window. This finding and that of flexible laryngoscopy suggested that the hoarseness was being caused by left recurrent nerve involvement resulting in left vocal cord paralysis. Thus, we performed a mediastinoscopic biopsy via a parasternal incision. Pathological examination revealed dense fibrous tissue infiltrated with varied inflammatory cells. Because no etiological pathogen or neoplastic lesion was identified, we diagnosed idiopathic fibrosing mediastinitis and began treating the patient with prednisolone. After a course of treatment, the mediastinal lesion showed a remarkable response. The hoarseness resolved as the lesion became smaller. Laryngoscopy confirmed recuperation of vocal cord function. This report shows that steroid therapy is a treatment option for hoarseness caused by recurrent laryngeal nerve involvement of fibrosing mediastinitis, if administered under close observation.

Idiopathic Fibrosing Mediastinitis Causing Pulmonary Hypertension with Improvement by Steroid Treatment

3 Idiopathic fibrosing mediastinitis is, an uncommon cause of pulmonary hypertension this is characterized by excessive fibrosis of the mediastinum with an unknown etiology. Steroid t herapy has been suggested for individuals with progressive symptoms, bu there is littlet data demonstrating the efficacy of such therapy are lacking. We present a case of pulmonary hypertension secondary to a compression of a main pulmonary artery by fibrosing mediastinitis which was confirmed by a biopsy with a thoracotomy. The chest CT scan and 2D echocardiography performed before and after a trial of steroid therapy demonstrated improvement after steroid therapy. (Tuberc Respir Dis 2006; 61: 74-79)

Fibrosing mediastinitis causing rapidly progressive dyspnea, pulmonary edema and death in a 16 yr old male

Idiopathic fibrosing mediastinitis is a rare entity involving more severely the more compliant structures within the mediastinum. In this report a rare case of simultaneous involvement of both the superior vena cava (SVC) and pulmonary veins is described in a 16--year old male with progressive dyspnea on exertion, cough and a three months' history of blood--tinged sputum. Physical examination and imaging studies revealed signs of pulmonary venous hypertension (PVH) and SVC stenosis. Fibrosing mediastinitis was confirmed by multiple biopsy samples.

Restenosis of superior vena cava after treatment using a self-expanding metallic stent in a patient with idiopathic fibrosing mediastinitis

The use of a self-expanding metallic stent (SEMS) for the treatment of superior vena cava (SVC) syndrome arising from non-neoplastic mediastinal disease is controversial, and data on long-term clinical observations for such have not yet been accumulated. We report a case of fibrosing mediastinitis with an obstruction of the SVC 30 months after the insertion of an SEMS. The patient had a headache and temporal facial swelling when the obstruction occurred, but these symptoms disappeared within 1 month. No further treatment was required in this case.

The possibilities of greater omentum usage in thoracic surgery.

To illustrate the wider role of an omental pedicle flap in surgical treatment for diverse thoracic organ disorders we have, retrospectively, reviewed our experience over the last 8 years. We used the greater omental pedicle flap in 68 patients. Bronchial stump omentopexy was performed in 35 patients with a high risk of impaired bronchial healing after right pneumonectomy as a preventive method and in two patients with an acute bronchial fistula. In 13 patients after circular tracheal or carinal resections and in four after esophago-respiratory disjoints of fistulas we applied anastomoses or circular omentopexy of the fistula zone. The omental coverage was performed in four patients with chronic empyema, in seven patients after extensive chest wall resection and in two patients with post-sternotomy mediastinites. In one patient with idiopathic fibrosing mediastinitis, one-stage allotransplantation of tracheal thoracic segment was conducted and greater omentum (GO) was used to wrap the allograft. Three patients developed major complications. The first, bronchial fistula after bronchial stump omentoplasty. In two patients circular wide tracheal resection (11-12 rings) was complicated by a tracheal divergence of anastomoses. These complications were cured in a conservative way since a displaced omental flap substituted the tracheal wall and, therefore, the trachea remained hermetically sealed. In other cases, the perfect adhesive properties of the omental tissue promoted perfect adhesive properties promoted prevention of incompetence and inflammatory complications. Immunological and bacteriological examinations showed that pediculated omental flap is a lymphocyte source and promotes a decrease in bacterial quantity and activity. We think the series demonstrates the value of the omental method, which offers excellent therapeutic results following an easy surgical procedure. This method extends indications for surgical treatment and decreases the postoperative complications.

Mediastinal Compression Syndromes Due to Idiopathic Fibrosing Mediastinitis - Report of Three Cases and Review of the Literature

Fibrosing mediastinitis is the most often observed benign cause of mediastinal compression syndromes, particularly the vena cava superior syndrome. We report 3 cases with such compression syndromes (2 x superior vena cava syndrome, 1 x symptomatic tracheal obstruction) due to fibrosing mediastinitis in which tumor resection led to a relief of symptoms. The operative procedures performed in our cases and in general as well as conservative treatment modalities reported for this rare disease are discussed with respect to our own experience and that of the recent publications in the European and American literature.

Idiopathic Fibrosing Mediastinitis Presenting as Asthma: Diagnostic and Management Considerations

A 10-year-old boy, previously in good health, experienced wheezing and exertional dyspnea. The symptoms had begun about 3 months before admission and progressed gradually to dyspnea at rest and orthopnea. On admission, the patient was in obvious respiratory distress with diffuse wheezing. There was no response to bronchodilators. Pulmonary function testing showed a fixed central airway obstruction, and chest x-ray showed mediastinal widening. Computed tomography (CT) demonstrated a mass displacing the esophagus and compressing the trachea. Surgical exploration revealed an inoperable mass surrounding the mediastinum. Histologic examination of the biopsy showed findings consistent with chronic fibrosing mediastinitis. No underlying disease process was identified. The patient was begun on steroid therapy and showed marked improvement. His pulmonary function tests and CT of the chest showed normal results after 2 months of therapy. He was gradually weaned off steroids and has been followed for 6 years, with no recurrence of disease. The etiology of chronic fibrosing mediastinitis and some points in the evaluation and treatment of this condition are discussed.

Tracheal stenosis: An unusual presenting complication of idiopathic fibrosing mediastinitis

Idiopathic fibrosing mediastinitis is a rare, obliterative, inflammatory process which is characterized by an abnormally exuberant proliferation of fibrous tissue within the superior mediastinum, resulting in the encroachment and occlusion of its contained structures. Usually, this is one of the low-pressure vascular structures, most often the superior vena cava. Airway obstruction as a primary presenting complication of this condition is rare with past treatment results usually being palliative at best. In this article, a patient with this complication is presented in whom emergent surgical treatment and tracheal decortication afforded permanent relief. The clinial presentation, morphology of the disease, and treatment are reviewed.