Idiopathic mediastinal fibrosis. A case report on its surgical treatment.
A case is presented in which mediastinal fibrosis had extended to involve the right lung, the diaphragm, the lower vena cava near the heart, and both atria including the interatrial septum. There is no previous report of this disease in such an extensive form. The relatively unusual clinical picture is discussed with regard to the pathophysiologic, clinical, diagnostic, and therapeutic problems it presents. The diagnosis could only be established postoperatively in our patient. In the course of a thoracotomy carried out to resect a neoplasm, an extensive, infiltrative tumorous process was discovered which, on the one hand, could not be classified histologically by immediate section under surgery and, on the other, showed no signs of malignancy. A radical operation was, therefore, performed in the form of a right-side pneumonectomy with partial resection of the diaphragm, lower vena cava, right and left atria and interatrial septum with plastic reconstruction of these structures. The patient survived surgery, and 3 years later, is in good health without any indication of residivation.
D000293:Adolescent; D001999:Bronchoscopy; D009202:Cardiomyopathies / Q000473:pathology / Q000601:surgery*; D006801:Humans; D008168:Lung / Q000473:pathology; D008297:Male; D008477:Mediastinal Diseases / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D011013:Pneumonectomy; D011184:Postoperative Period; D013902:Radiography, Thoracic; D012185:Retroperitoneal Fibrosis / Q000175:diagnosis; D014682:Vena Cava, Inferior