[Interventionnal bronchoscopy for the treatment of tracheobronchomalacia]. / Place de la bronchoscopie interventionnelle dans la prise en charge des trachéobronchomalacies.

Tracheobronchomalacia is usually characterized by more than 50% expiratory narrowing in diameter of the trachea and the bronchi. The expiratory collapse includes two entities: (1) the TBM related to the weakness of the cartilaginous rings, and (2) the Excessive Dynamic Airway Collapse (EDAC) due to the excessive bulging of the posterior membrane. Patients have nonspecific respiratory symptoms like dyspnea and cough. Diagnosis is confirmed by dynamic tests: flexible bronchoscopy and/or computed tomographic scan of the chest. There are different forms of tracheobronchomalacia in adults: primary (genetic, idiopathic) or secondary to trauma, tracheotomy, intubation, surgery, transplantation, emphysema, infection, inflammation, chronic bronchitis, extrinsic compression; or undiagnosed in childhood vascular rings. Some management algorithms have been proposed, but no specific recommendation was established. Only symptomatic patients should be treated. Medical treatments and noninvasive positive pressure ventilation should be the first line therapy, after evaluation of various quality measures (functional status, performance status, dyspnea and quality of life scores). If symptoms persist, therapeutic bronchoscopy permits: (1) patient's selection by stent trial to determine whether patient benefit for surgical airway stabilization; (2) malacic airways stenting in patients who are not surgical candidates, improving QOL despite a high complication rate; (3) the management of stent-related complication (obstruction, plugging, migration granuloma); (4) alternative therapeutics like thermo-ablative solution. Lasty, the development of new types of stents would reduce the complication rates. These different options remained discussed.

[Prenatal diagnosis of a giant cystic lymphangioma in the axillothoracic wall]. / Diagnostic anténatal d'un lymphangiome kystique géant de la paroi thoraco-axillaire.

Cystic lymphangiomas are benign malformative tumors of the lymphatic vessels, rare but potentially serious and usually located in the cervical region. The diagnosis is usually easy, but the treatment is sometimes difficult because of their location and extension into the surrounding tissue. We report a case of giant cystic lymphangioma of the right axillary-thoracic wall diagnosed by prenatal ultrasound at 20 weeks gestation. The male infant was born at 37 weeks gestation with a large, swelling, lateral parietal right chest. Ultrasound soft tissue and chest CT scan confirmed the diagnosis of giant cystic lymphangioma of the right axillary-thoracic wall. Surgical excision was performed on the 21st day after birth and the mass was excised incompletely. The postoperative course was uncomplicated. Ultrasound soft tissue follow-up showed the persistence of multiple cystic structures in the axilla. Prenatal diagnosis of these tumors is essential for planning multidisciplinary management in early postnatal care.

[Idiopathic intracranial hypertension and factor V Leiden mutation]. / Hypertension intracrânienne idiopathique et mutation du facteur V de Leiden.

Activated proteinC resistance is a frequent prothrombotic abnormality. In most cases it is due to factorV Leiden mutation by nucleotide G1691A substitution. This recently described thrombophilic defect of activated proteinC resistance has been postulated to be implicated in the pathogenesis of idiopathic intracranial hypertension (IIH). We report a case of factorV Leiden mutation in association with IIH and their likely link and implication in the management of IIH.

[Vertebral primary hydatid cyst with mediastinal involvement and paraplegia]. / Kyste hydatique vertébral primitif à extension médiastinale postérieure compliquée d'une paraplégie.

INTRODUCTION: The vertebrae are the most common localization of hydatid disease of bone. This can lead to fatal consequences. CASE REPORT: We report the case of a 40-year-old-man, from a rural area, who had symptoms of medullary compression. The diagnosis of primary vertebral hydatid cyst, already suspected on the imaging data, was confirmed. A right posterolateral thoracotomy allowed drainage of the cyst and relief of the medullary compression. Medical treatment with albendazole was continued for 4 months. The postoperative course was uneventful and the symptoms of medullary compression resolved progressively. No recurrence was observed during a follow-up of 24 months. CONCLUSION: Vertebral localization of hydatid disease is the most common and serious skeletal complication. Thoracotomy allows drainage of the cyst and the pleural cavity, and relieves the medullary compression.

Hashimoto's thyroiditis and severe hypothyroidism, associated with a single hot nodule.

We report the case of a 27 year-old man with symptoms of severe hypothyroidism that have evolved since his adolescence. He was found to have an 11 mm right lobe thyroid nodule. On thyroid ultrasound, the nodule was solid, heterogeneous with markedly atrophic hypoechoic surrounding tissue. Thyroid scintigraphy revealed increased (99m)Tc pertechnetate and (131)I uptake, with persistence of (131)I hyperfixation after 24 h. There was no fixation of the radiotracer in the remaining tissue. Thyroid function tests found TSH > 100 mIU/l, free thyroxine level 0.9 pmol/l (normal values 11.5-21.8), anti-thyroid peroxydase antibodies strongly positive > 1,000 IU/ml, and anti-thyroglobulin antibodies negative. One year after levothyroxine therapy, the nodule decreased to 40% of its original size. Hashimoto's thyroiditis may present as a single hot nodule and severe hypothyroidism. Data of reported cases suggest that the hot nodule corresponds to a localized hyperplasia of the less diseased portions of the thyroid. Chronic stimulation by TSH may have promoted nodular growth and isotopes uptake.

[Bilateral popliteal artery complications of multiple hereditary exostosis]. / Complications vasculaires poplitées bilatérales d'une exostose héréditaire multiple.

The osseous exostose is a rare benign tumor of the bone from which the vascular complications can be of venous or arterial order, are translated in various boards. We report the case of a young adult who presents a forgery aneurysm of the right popliteal artery with the popliteal artery booby-trapped to the left. The patient benefited from surgical treatment with good clinical and radiological evolution.

[Familial idiopathic pulmonary fibrosis associated with autoimmune polyendocrinopathy and epidermodysplasia verruciformis]. / Fibrose pulmonaire idiopathique familiale associée à une polyendocrinopathie auto-immune et à une épidermodysplasie verruciforme.

Familial idiopathic pulmonary fibrosis (IPF) is a very rare and progressively fatal disease. Its pathogenesis is not fully understood and involves damage to alveolar epithelial cells of possibly immunological, microbiological or chemical origin, leading to fibrosing healing. A genetic predisposition has been demonstrated. The authors report the case of a female patient whose brother died at the age of 29 from IPF. She had epidermodysplasia verruciformis since childhood, with the absence of pubertal development. At the age of 31, she presented diffuse interstitial pneumonia. A lung biopsy confirmed the diagnosis of IPF. Endocrine explorations detected hypogonadotropic hypogonadism, primary hypothyroidism and magnetic resonance imaging revealed an empty sella turcica. The association of familial IPF, autoimmune polyendocrinopathy and genetic dermatosis caused by a cellular immune deficiency supports the hypothesis of an immune dysfunction in the pathogenesis of IPF.

[Hereditary multiple exostosis revealed by deep vein and arterial popliteal thrombosis]. / Maladie des exostoses multiples révélée par une thrombose poplitée veineuse et artérielle.

We report a 38-year-old man who presented successively a thrombosis of the right popliteal vein and the right popliteal artery, responsible of a painful edema of the leg. There was no known thrombophilic predisposing condition. Radiologic assessment, guided by clinical findings, evidenced bilateral femoral and tibial exostosis resulting in a diagnosis of hereditary multiples exostosis. Although anticoagulant treatment with unfractioned heparin was instituted, distal ischemia of the right lower limb developed. Resection of the compressive osteochondroma and an arterial femorotibial bypass venous graft were performed in addition to prolonged anticoagulant therapy. Outcome was eventually favorable. Hereditary multiple exostosis may be asymptomatic for long time. Compression of adjacent vascular structures by an osteochondroma is a rare and potentially serious complication.

An 18-cm-large renal arteriovenous fistula treated by nephrectomy.

Idiopathic renal arteriovenous fistulas are extremely rare. They are believed to occur as the result of congenital renal artery aneurysm that erodes into an adjacent vein. We report a case of a 48-year-old man in whom we discovered fortuitously a painless mass of the right flank. Computed tomography revealed a huge renal artery aneurysm with giant arteriovenous fistula in the absence of any clinical stigmata. Given the size of the fistula and the partial destruction of the renal parenchyma, nephrectomy was successfully performed.

[Early surgery in patients with native valve endocarditis]. / Chirurgie de l'endocardite sur valves natives à la phase active.

Between January 1990 and December 2006, 93 patients with infective endocarditis on native valves were operated in the active phase of the disease. The average age of our patients was 32 years with a male ascendancy. The causal heart disorder was found in 89 % of the cases, dominated by rheumatoid arthritis. The germ in cause was isolated in 52.6 % of the cases. The operative indication was hemodynamic in 29 cases, infectious in nine cases, mixed in 29 cases and embolic in 26 cases. The average operating delay was of 13 days with regard to the beginning of the antibiotic treatment. We realized a valvular aortic replacement at 32 patients with reconstruction of the ring in six cases, a valvular mitral replacement at 29 patients, a mitroaortic replacement at 21 patients, a reconstructive mitral surgery in nine cases, a valvular tricuspid replacement in one case and a reconstructive tricuspid surgery in one case. The early mortality was 13 %. The follow-up was 89 % with an average recession of 3.1 years and a late mortality of 5 %. The aim of this study is to analyze the immediate and late results of the surgery of infective endocarditis in the active phase and to bring to light the prognostic factors of mortality.

[Surgery of 56 patients having a partial atrioventricular septal defect]. / Chirurgie du canal atrioventriculaire partiel : résultats à court et à moyen terme à propos de 56 patients opérés.

Between January 1991 and December 2006, 56 patients having a partial atrioventricular septal defect (AVSD) were operated. The purpose of this retrospective study is to analyze the immediate and long-term results of the surgery by granting of the importance to two main problems which are the disturbances of the rhythm and the conduction and the residual mitral regurgitation (MR). The mean age of our patients is of 10 and a half years with a net feminine ascendancy. Ninety-three percent of the patients were in regular sinus rhythm. No case of complete atrioventricular block (AVB) was noted. The MR was of grade I in 28.5% of the cases, grade II in 60% of the cases and grade III and IV in 7.5% of the cases. The MR was mild in 4% of the cases. The correction was made under cardiopulmonary bypass (CPB) and consisted of a suture of the mitral cleft in most of the cases with lock of the ostium primum by a patch of pericardium. The perioperative mortality was 1,8% of the cases. The disturbances of the rhythm and the conduction were noted in 34% of the cases. All the patients were controlled with a mean follow-up of six years and seven months. The secondary mortality was nil. The MR, at mid-term follow-up, was mild in 78% of the cases. The partial AVSD is a congenital heart disease, the spontaneous evolution of which can be burdened by complications, notably the disturbances of the rhythm and the conduction, as well as the heart failure. This justifies a premature surgical repair.

[Coronary artery fistula: case report and review of the literature]. / Fistule coronaire : rapport d'un cas opéré et revue de la littérature.

The coronary fistula is a rare abnormality making communicate a coronary artery with a cardiac cavity or a great vessel, so bypassing the myocardial capillary network. The majority of these fistulas are congenital but can nevertheless arise after a cardiac surgery. The right coronary artery and the left anterior descending coronary artery are mostly concerned. The circumflex coronary artery is rarely involved. The most frequent site of drainage is the right ventricle. We report the case of a 2-year-old child, brought by his parents for dyspnoea of effort. The diagnosis of coronary fistula was confirmed by the coronary angiography which showed an aneurysmal circumflex artery, draining into the right ventricle. The intervention was led under cardiopulmonary bypass. We proceeded to the longitudinal opening of the aneurysm then to the blindness of the fistula. The postoperative course was simple.

[Maxillo-facial location of hydatic cyst]. / Kyste hydatique du massif facial.

OBJECTIVE: A maxillofacial location of a hydatid cyst is rare, accounting 2% of cases. The purpose of this study was to review clinical and radiological features by emphasizing diagnostic difficulties. MATERIALS AND METHODS: Two observations of hydatid cyst with a maxillofacial location are presented. Two cases were investigated by panoramic dental radiography, an ultrasound and CT scan. MRI was done in one case. RESULTS: The first observation reports a 23-year-old woman who consulted for jugal tumefaction with pharyngeal extension evolving in spurts. The second observation was a 16-year-old girl, which consulted for a genian and masseter tumefaction with a tight trismus. In both cases, the imaging studies suggested the diagnosis of the hydatid cyst confirmed by the anatomopathologic exam. CONCLUSION: The hydatid cyst is an endemic parasitic pathology involving most often the lung and the liver. The maxillofacial location is exceptional. In cases of a cyst mass in an endemic zone, the diagnosis of the hydatid cyst must be entertained. CT scan and MRI provided a complete lesion workup. Treatment is surgical.

[Emboligenous hydatid cyst of the right heart]. / Kyste hydatique emboligène du coeur droit.

The cardiac location of the echinococcosis is rare. It is associated with complications potentially severe. Indeed, the break inside the cardiac chambers with pulmonary embolism is the inevitable complication of the echinococcosis of the right heart. Between January 1992 and January 2006, five patients were operated in the department of cardiac surgery of Sousse (Tunisia) for an emboligenous hydatid cyst of the right heart. The average age is of 30 years with extremes from 18 to 65 years. The cardio-pulmonary bypass is the technique of choice. We regretted a single death in immediate postoperative period. All the patients were controlled with an average recession of 36 months. A single late death was noticed. No recurrence was observed.