Résumé
We report a case of a 29-year-old female with a history of asthma, post-partumARDS, and pulmonary hypertension who presents with severe shortness of breath. The patient describes her shortness of breath as progressive over the past 10 years. Chest radiography and CT angiography of the thorax showed findings consistent with fibrosing mediastinitis with severe stenosis of the left main pulmonary artery. This resulted in appearance of unilateral absent left lung perfusion on quantitative Tc-99-MAA perfusion and Xe-133 ventilation (V/Q) scan.
Sujets)
Adulte , Femelle , Humains , Angiographie , Asthme , Sténose pathologique , Dyspnée , Histoplasma , Hypertension pulmonaire , Poumon , Médiastinite , Perfusion , Artère pulmonaire , Radiographie , Thorax , VentilationRésumé
Neste trabalho é relatado o caso de uma mulher de 51 anos de idade, com dor torácica, dispneia e engurgitamento de vasos do tórax superior, sem evidências clínicas de doença granulomatosa. O estudo por imagem do tórax revelou massa mediastinal com calcificações, abordada por mediastinoscopia com biópsia. A análise histopatológica mostrou padrão inflamatório crônico sem etiologia definida, compatível com mediastinite fibrosante.
The present study reports the case of a 51-year-old woman with chest pain, dyspnea and upper chest vessels engorgement, with no clinical evidence of granulomatous disease. Chest imaging study revealed a mediastinal mass with calcifications that was approached by mediastinoscopy with biopsy. Histopathological study demonstrated an etiologically undefined chronic inflammatory pattern compatible with fibrosing mediastinitis.
Sujets)
Humains , Femelle , Adulte d'âge moyen , Médiastinite/diagnostic , Radiographie thoracique/méthodes , Médiastinoscopie/méthodes , Tomographie/méthodesRésumé
Fibrosing mediastinitis is a rare disease that is characterized by the proliferation of dense fibrous tissue of the mediastinum. The pathogenesis of fibrosing mediastinitis is unknown in most cases. However, histoplasmosis, tuberculosis, autoimmune disease, radiation therapy, and other idiopathic fibroinflammatory diseases have been implicated in some cases. Most clinical features are related to an obstruction or compression of the mediastinal structure. Fibrosing mediastinitis is often progressive and occurs diffusely throughout the mediastinum. We encountered a case of fibrosing mediastinitis of a very focal lesion without evidence of mediastinal involvement. The condition was confirmed by biopsy and graft bypass surgery was performed because of SVC syndrome.
Sujets)
Maladies auto-immunes , Biopsie , Histoplasmose , Médiastinite , Médiastin , Maladies rares , Transplants , TuberculoseRésumé
Idiopathic fibrosing mediastinitis is, an uncommon cause of pulmonary hypertension this is characterized by excessive fibrosis of the mediastinum with an unknown etiology. Steroid therapy 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.
Sujets)
Biopsie , Échocardiographie , Fibrose , Hypertension pulmonaire , Médiastinite , Médiastin , Artère pulmonaire , Thoracotomie , TomodensitométrieRésumé
Fibrosing mediastinitis is a rare benign disorder caused by excessive proliferation of fibrous tissue within the mediastinum. Pulmonary artery stenosis is uncommon complication of fibrosing mediastinitis. We present a case of percutaneous stent deployment in a patient with severe pulmonary artery stenosis causing pulmonary hypertension secondary to fibrosing mediastinitis.
Sujets)
Humains , Sténose pathologique , Hypertension pulmonaire , Médiastinite , Médiastin , Artère pulmonaire , EndoprothèsesRésumé
A 68-year-old woman was admitted after suffering facial edema with neck vein engorgement ofr approximately 2 months. A chest X-ray showed a mild widening of the superior mediastinum and a luminal obliteration of the super ior vena cava (SVC) was noted on a computed tomograph. Venography showed that both subclavian veins were obstructed at the level of the poxima clavicle with a nonvisualization of the SVC. The SVC, both the innominate and the left inthrnal jugular veins were completely obstructed with extensive cord-like fibrotix changes despite the obsence of mediastinal involvement. The microscopic features showed a chronic granulomatous inflammation with a fibrosis minimally invading the mediastinal fat, which is cosistent with fibrosing mediastinitis.
Sujets)
Sujet âgé , Femelle , Humains , Clavicule , Oedème , Fibrose , Inflammation , Veines jugulaires , Médiastinite , Médiastin , Cou , Phénobarbital , Phlébographie , Veine subclavière , Thorax , VeinesRésumé
Fibrosing mediastinitis is a rare disease which is characterized by excessive fibrosis of mediastinum and symptoms caused by compression and obstruction of mediastinal structures. Afthough the pathogenesis of this disease is unknown, granulomatous infection is cinsidered to be the most common cause of this disease. Histoplasmosis is the most common etiology, especially in the endemic areas in United States. Tuberculosis is another etiology of fibrosing mediastinitis. We experienced two cases of fibrosing mediastinitis associated with tuberculous infection.