[White hemithorax in a young women carrying coffee and milk spots]. / Un hémithorax blanc chez une jeune portant des tâches café au lait.

INTRODUCTION: Neurofibromatosis type 1 (NF1) or Von Recklinghausen disease is an autosomal dominant genetic disorder with multivisceral manifestations. We report the case of a spontaneous haemothorax in a young lady wearing this genetic pathology. CASE REPORT: A 31-year-old woman with kyphoscoliosis developed acute chest pain and dyspnea. The physical examination revealed a right pleuritic syndrome, coffee and milk spots and neurofibromas of the trunk. Biological assessment showed anemia at 6.4g/dl. Chest x-ray revealed a right side opacification. The pleural puncture showed an incoagulable hemorrhagic fluid. The chest CT scan showed no vascular abnormalities. The diagnosis of spontaneous haemothorax, revealing NF1, was retained. Transfusion and thoracic drainage were performed followed by haemostasis surgery. Pleural exploration showed pleural hematoma with regard to the 5th intercostal space. Electrocoagulation and declogging were performed. The evolution of the patient was favorable. CONCLUSION: Haemothorax is a rare and serious complication which may reveal NF1. It must be suspected when sudden and spontaneous white haemithorax occurs in NF1.

[Tuberculous pneumothorax: Diagnosis and treatment]. / Pneumothorax tuberculeux : prise en charge diagnostique et thérapeutique.

BACKGROUND: Pneumothorax is a serious complication of cavitary pulmonary tuberculosis. The aim of this study was to describe clinical futures, to highlight challenges of its management. METHODS: A retrospective multicentric and descriptive study including 65 patients treated for PT (1999-2015) was conducted to figure out clinical futures and its work-up. RESULTS: The mean age was 37.8 years. The sex ratio was 3.6. Smoking history and incarceration were noted respectively in 67.6 and 15.3% of cases. Acute respiratory failure and cachexia were reported in 26.1 and 10.7% of cases. The PT was inaugural in 41.5% of cases. Pyo-pneumothorax was noted in 69.2% of cases. The duration of antituberculous treatment ranged from 6 to 15 months for susceptible TB and was at least 12 months for resistant TB (4 cases). Thoracic drainage was performed in 90.7% patients. Its average length was 47 days. The drain drop was noted in 20% of cases. Bronchopleural fistula was diagnosed in 6 cases and pleural infection in 5 of cases. Surgery treatment was necessary in 6 cases. Mean time to surgery was 171 days. Six patients had pleural decortication associated with pulmonary resection in 4 cases. Persistent chronic PT was noted in 12.6% and chronic respiratory failure in 3% of cases and death in 15.3% of cases. CONCLUSION: The diagnosis of the PT is often easy. Its treatment encounters multiples difficulties. Duration of thoracic drainage and anti-TB treatment are usually long. Surgery is proposed lately.

[Management of multidrug-resistant tuberculosis]. / Prise en charge de la tuberculose multirésistante.

The emergence of drug-resistant TB in many countries has become a major public health problem and an obstacle to effective tuberculosis control. Multidrug-resistant tuberculosis (MDR-TB), which is most often the result of poor adherence, is a particularly dangerous form of tuberculosis because it is caused by bacilli resistant to at least isoniazid and rifampicin, the two most effective anti-tuberculosis drugs. Techniques for rapid diagnosis of resistance have greatly improved the care of patients by allowing early treatment which remains complex and costly establishment, and requires skills and resources. The treatment is not standardized but it includes in all cases attack phase with five drugs (there must be an injectable agent and a fluoroquinolone that form the basis of the regimen) for eight months and a maintenance phase (without injectable agent) with a total duration of 20 months on average. Surgery may be beneficial as long as the lesions are localized and the patient has a good cardiorespiratory function. Evolution of MDR-TB treated is less favorable than tuberculosis with germ sensitive. The cure rate varies from 60 to 75% for MDR-TB, and drops to 30 to 40% for XDR-TB. Mortality remains high, ranging from 20 to 40% even up to 70-90% in people co-infected with HIV.

[Pleural complications of pulmonary hydatid disease]. / Kyste hydatique du poumon ouvert dans la plèvre.

BACKGROUND: Pulmonary hydatid disease encountered in Tunisia frequentely. Rupture of pulmonary cyst into the pleural cavity is rare, but represents the most serious complication of the hydatid disease. AIM: We analyse clinical, radiologic and outcome of intrapleural rupture of lung hydatic cyst and evaluate our experience in the surgical treatment. METHODS: We report 5 cases of rupture of rupture of pulmonary hydatid cyst into the pleural cavity hospitilazed during 1995 to 2010. RESULTS: Mean age of patients was 22,4 years. Three patients presented with hydropneumothorax, one with spontaneous pneumothorax and one with haemoptysis. The diagnosis was determined peropreratively in the first case and based on radiographic and serology findings in the other cases. Surgical treatment consist on decortication and cystotomy with capitonnage in three cases, pleurectomy one and lobectomy in the other case. Post operative course was unventful in four cases, one patient had bronchopleural fistula, pneumothorax and wound infection (parietal abssess) resolved with local treatment. After surgery, all patients were treated in post operative by 3 months course with Albendazole chemotherapy with a favourable outcome. CONCLUSION: Rupture of a pulmonary cyst into the pleural cavity is rare, but represents the most serious complication of the hydatid disease. The therapeutic progress has improved the prognosis of intrapleural ruptured hydatid cysts.

[Case report of pulmonary biphasic tumor with unusual follow-up]. / À propos d'une tumeur biphasique du poumon d'évolution inhabituelle.

BACKGROUND: Pulmonary blastoma, a rare primary lung malignancy is subdivided in 3 categories: well-differentiated fetal adenocarcinoma (WDFA), classic biphasic pulmonary blastoma (CBPB) and pleuropulmonary blastoma (PPB). Classic pulmonary blastoma is composed of a mixture of immature epithelial and mesenchymal tissue resembling fetal lung tissue. CASE REPORT: We described a case of a 48-year-old male, cigarette smoker, who presented with left thoracic pain and hemoptysis for 2 months. Chest radiography showed a well-delimited, homogeneous 4cm mass in the left lung periphery. Bronchoscopic examination revealed left endobronchial bleeding. Computed tomography of the chest revealed a tumor shadow measuring 7cm in the left upper lobe and bilateral nodules with no lymphadenopathy. A systemic evaluation demonstrated no metastatic lesion. Patient underwent a left upper lobectomy. The diagnosis of CBPB was affirmed on anatomopathology of the tumor resection. Immunohistochemical studies showed that tumor cells were positive for vimentin, desmin, actin, Pan Cytokeratin and TTF-1. The final diagnosis was BPB classified as pathological T3N0M0 and no adjuvant treatment was associated. The patient showed good objective response with no evidence of disease recurrence still in 5 years surgery resection. CONCLUSION: This case reiterates the importance of pathomorphological or immunohistochemical features in diagnosis of BPB.

[Primary mediastinal sarcomas]. / Les sarcomes primitifs du médiastin.

INTRODUCTION: Primary sarcomas of the mediastinum are rare and account for 2 to 8% of malignant mediastinal tumours. The aim of this study is to describe their clinical and pathological characteristics. PATIENTS AND METHODS: Fifteen cases of primary mediastinal sarcoma, diagnosed between 1993 and 2009, were reviewed retrospectively, noting the clinical, radiological and pathological findings, and the treatment given. RESULTS: The patient population consisted of eight females and seven males with mean age of 40 years (14 to 73 years). The symptomatology was predominately respiratory. Imaging showed a mediastinal mass invading adjacent organs in nine cases. The diagnosis was made in all cases by histological examination. These 15 mediastinal sarcoma comprised 12 malignant peripheral nerve tumours, two liposarcomas and one angiosarcoma. Three were grade III, six grade II and six grade I. Ten were treated surgically, of which seven had radical resections. Associated treatments were neoadjuvant (one case) or adjuvant (one case) chemotherapy and postoperative radiotherapy (five cases). Radiotherapy was undertaken alone in three inoperable tumours. Eight patients (53%) had died. CONCLUSION: Management of primary mediastinal sarcoma needs a multidisciplinary approach, and is based mainly on radical resection. The prognosis is poor and depends mainly on surgical excision and histological grade.

Desmoid-type chest wall fibromatosis. A six cases series.

PURPOSE OF THE STUDY: Desmoid-type fibromatosis is a fibroblastic proliferation that develops in the deep soft tissues and is characterized by infiltrative growth and a tendency towards local recurrence but does not metastasize. Fibromatosis of the chest wall is rare, representing only 10 to 20% of all deep fibromatoses. CASE REPORT: We report a six cases series of fibromatosis of the chest wall treated at our institution between 1996 and 2009. There were five men and one woman with a mean age of 42.2. Swelling of the chest wall was the most frequent symptom. Imaging showed an expansive homogeneous mass, invading the rib (four cases) or the sternum (two cases). Surgical resection was performed in all cases. The outcome was favorable (five cases) or marked by local recurrence (one case). DISCUSSION: Although it is considered to be a benign lesion, this tumor can be locally very aggressive, and has a high local recurrence rate depending upon the efficacy of surgical resection.

[Management of spontaneous pneumomediastinum in the adult: 14 cases and a review of the literature]. / Prise en charge du pneumomédiastin spontané chez l'adulte: à propos de 14 cas avec revue de la littérature.

The authors report a series of 14 patients hospitalized for spontaneous pneumomediastinium between 1992 and 2006. They included 10 men and four women with an average age of 27.84 years. Dyspnoea dominated the symptoms. The pneumomediastinum was idiopathic in five patients and secondary in the other nine patients, involving an attack of severe asthma in half of the patients, polymyositis in one patient and idiopathic pulmonary fibrosis in one patient, respectively. The patients presenting polymyositis and pulmonary fibrosis died due to respiratory distress. The other patients benefited from asthma treatment or thoracic drainage, and were kept at rest. Their outcome was good. No cases of relapse were observed after an average follow up of 2.11 years.

[Hydatid cyst of the chest wall mimicking metastatic colon cancer]. / Kyste hydatique de la paroi thoracique mimant une métastase de cancer colique.

Chest wall hydatidosis, rare even in the endemic countries, represents 0.09 to 0.3 % of all cases of thoracic echinococcosis. The authors report the case of a 76 year-old man presenting a chest wall mass 4 years after surgery for colic carcinoma. The mass presented both hypoechoic and hyperechoic structures in the ultrasound chest echography. Therefore, metastastic colon cancer was suspected. The pathological study of the mass revealed hydatid membranes. Thoracic tomodensitometry supported the diagnosis of costovertebral and soft tissue hydatid cysts. The patient underwent the surgical resection of two rib arches, a transverse apophysis and the neighbouring soft tissue associated with pre- and post-surgical albendazole. No clinical manifestations were noted in the follow-up after 1 year.