[A rare cause of superior vena cava thrombosis: pulmonary arteriovenous malformations].
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Biomedical subjects
Publications and source records attributed to J Benamor.
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BACKGROUND: Sarcoïdosis is a benign systemic granulomatosis whose aetiology remains unknown. Lung is the most frequently involved organ. The pseudoalveolar form of this disease is known to have an acute onset and is quite uncommon. Therefore, diagnosing such a rare variety of sarcoidosis is rather often challenging. OBSERVATIONS: In the present article, the authors report two cases of pseudoalveolar sarcoidosis. The patients, both young adults, showed no suggestive signs of sarcoidosis at first presentation. This resulted in a considerable delay to diagnosis and to the corticosteroid therapy. CONCLUSION: The authors emphasize the rarity of the pseudoalveolar form of sarcoidosis. They insist on its roentgenographic characteristics and demonstrate the functional benefits allowed by the precocious medical management. They also propose a current review of the literature.
Sarcoidosis manifestations in the upper airways and pleura are exceptional, observed in 5% and 1% of cases. We report an exceptional observation of an association of these two localizations in addition to mediastinopulmonary involvement. After confirmation of the diagnosis obtained on bronchial biopsies and nasal mucosa biopsies, pleural sarcoidosis was retained because of the clinical context and the spontaneous resolution of the effusion. The major problem is the absence of a therapeutic consensus on proper management, oral corticosteroid therapy being proposed by some for these particular forms of sarcoidosis.
Chronic atrophic polychondritis is a rare systemic disease characterized by recurring inflammation of cartilage of the ear, nose, larynx and of the tracheobronchial tree. Nowadays, it is considered to be an autoimmune disease. The case of a 25-year-old female patient hospitalized in our department for treatment of a dyspnea is herein presented. ORL laryngoscopic examination revealed a small sized larynx with a pronounced narrowing of its lumen and very "floppy" cartilage. Bronchoscopic findings were a circumferential stenosis along the entire trachea and the right major bronchi. Results from biopsies performed on the trachea and thyroid cartilage were in favor of chronic atrophic polychondritis. The course of the disease was characterized by an aggravation of the dyspnea with episodes of asphyxia resulting in a tracheotomy. The tracheotomy has not been closed to this day, due to the underlined disease of the patient.
Cystic adenoid carcinoma (CAC) of the trachea, also called cylindroma, is a rare malignant tumor accounting for 1 per thousand of all respiratory tract cancers. We report three cases of cylindroma in patients hospitalized for inspiratory dyspnea due to a tracheal tumor. Bronchoscopy using a rigid tube revealed a smooth tumor of the trachea. Pathology analysis of the biopsy specimen diagnosed CAC. The three patients underwent resection with end-to-end anastomosis. Adjuvant radiotherapy was given in one patient. The short-term outcome was good but one patient developed local recurrence and lung metastasis at six years.
Intrathoracic textiloma is very rare and the transdiaphragmatic migration after abdominal surgery is exceptional. The diagnosis was suspected by history of surgery and radiology (CT), the treatment is only surgical. We report a case of intrathoracic textiloma after biliary surgery 30 years before and we describe the clinical, radiological features and treatment of this severe complication including clinical and medico-legal consequences.
We report 4 cases of malignant thoraco-pulmonary small-cell tumors (Askin tumor). Only two cases were operated. We emphasize the difficult histological diagnosis and demonstrate the importance of complete removal for survival. Prognosis remains poor.