Interest of surgical vascular reconstructions aiming at preserving hemodialysis access.

BACKGROUND: Vascular hemodialysis access is a very precious asset for patients with end-stage renal failure. Ideally complications in these accesses should be detected early in order to treat them in time. The aim of this study was to evaluate the effectiveness of vascular reconstructions to preserve the vascular access for hemodialysis and to examine their benefits in terms of conserving the venous network, using hemodialysis catheters, time of cannulation after surgery, patency and postoperative morbi-mortality, by comparing them to those of newly created arterio-venous fistula (AVF). METHODS: This was a retrospective, descriptive and comparative study, performed from September 2012 to February 2015. It concerned patients operated for complications of their native AVF. Two groups were compared: reconstructed AVF (group 1) and newly created AVF (group 2). Demographic characteristics, comorbidities, preoperative clinical and paraclinical data, surgical techniques and outcomes were recorded. This data was analyzed and compared between the two groups. RESULTS: We collected 151 surgical interventions divided into two groups: 55 surgical reconstructions and 96 new AVF. The average age was 59.9±1.3 years [19.1-88.9], with a male predominance (59.6%). The two groups were comparable in terms of age, gender and comorbidities. Reconstructions were mostly indicated for stenosis (47.3%) and new vascular accesses for venous thrombosis (92.7%). Reconstruction had significantly better successful exploitation rates (95.7%) and time of cannulation (26.6 days) (respectively P=0.025 and P=0.000). Its primary failure rate was null. Its primary patency and primary functional patency were respectively 94.2% and 97.7% at 1 month and 82.2% and 87.1% at 2 years. Morbidity's global rates were comparable between both groups (41.8% vs. 43.8%). The rates of early morbidity and stenosis were significantly higher for reconstructions (respectively P=0.037 and P=0.047), while late morbidity and thrombosis' rates were significantly higher for new AVF (respectively P=0.021 and P=0.023). Mid-term permeabilities were better for reconstructions, but the differences were not statistically significant. CONCLUSION: Surgical reconstructions seem to be an effective alternative for the treatment of complicated AVF. Our results appear to be comparable to those of the literature. The benefits of these surgical techniques in terms of conserving venous network, use of hemodialysis catheters, time for cannulation after surgery, patency and postoperative morbi-mortality appear to be undeniable.

[A case of pulmonary arteriovenous malformation treated surgically]. / Bisegmentectomie pour une malformation artérioveineuse pulmonaire.

Pulmonary arteriovenous malformation is a rare abnormality consisting of a direct connection between the arteries and the pulmonary veins. Most of the malformations are related to hereditary hemorrhagic telangiectasia, although 10 to 20% cases are idiopathic. Clinical manifestations are due to right-to-left shunting. Embolization is the treatment of choice, when it is possible and accessible. Surgery continues to be appropriate in certain cases. We report the case of a woman who presented with an isolated complex arteriovenous malformation fed by two afferent arteries, a lingular one and an antero-basal one. Surgical treatment by lingual and antero-basal bisegmentectomy was undertaken with a good outcome.

A Delicate Surgical Resection to Treat a Proximal Pulmonary Adenoid Cystic Carcinoma.

Pulmonary adenoid cystic carcinoma (PACC) is an exceedingly rare tumor of low-grade malignancy. Diagnosis is often late, so the tumoral process may be huge at presentation. Surgical resection could be challenging, and the recurrence rate would be greater. We report, hereby, a case of proximal PACC with involvement of the carina in a young male adult, without respiratory distress. Surgical resection was performed through a left pneumonectomy followed by a complex trachea-bronchoplastic procedure. During the operative time, the assisted ventilatory mode was carefully chosen. No adjuvant treatment was needed. Our patient is still under clinicoradiological surveillance and remains disease-free.

[Primary benign schwannoma of the pleura: Report of 2 cases]. / Schwannome bénin primitif de la plèvre : à propos de deux cas opérés.

The preferred thoracic location of a schwannoma is the posterior mediastinum. A pleural location is very rare. To date there have been fewer than 20 cases in the literature. We report two operated cases of primary benign pleural schwannoma. The first occurred in a 52-year-old woman who presented with right-sided chest pain and chronic cough. The radiological appearance suggested a hydatid cyst of the lower right lobe. The second case concerned a 37-year-old, asymptomatic man with no past history, who presented with a left posterior mediastinal mass, discovered incidentally on imaging. Both patients underwent complete surgical resection via a posterolateral thoracotomy. The final anatomo-pathological investigation revealed two primary benign schwannomas of the pleura.

[Place of the microscopic examination in the diagnosis of costal fibrous dysplasia]. / Place de l'examen anatomopathologique dans le diagnostic de la dysplasie fibreuse costale.

BACKGROUND: Fibrous dysplasia of bone is a rare benign lesion characterized by the coexistence of a fibrous tissue and an immature osteogenesis. Costal localization is rare and may be monostotic or polyostotic. The diagnosis may be suspected based on clinical and radiological findings. Facing the development of radiological investigations, we tried to highlight the diagnostic role of the microscopic examination through the experience of our department. METHODS: We describe a retrospective study about 12 costal fibrous dysplasias diagnosed over a 17-year-period. Clinical records were retrieved from the department of thoracic surgery of the same hospital. RESULTS: Costal fibrous dysplasia is equally observed in men and women with predominance in the third and fourth decades. Clinical symptoms consist mainly in chest pain. Physical examination was normal in almost all cases. Based on the radiological findings, the diagnosis was suspected in 33% of the cases. Microscopic examination highlighted the diagnosis in all cases but it was challenging in one case and necessitated a multi-disciplinary approach. The difficulties encountered were due to artifact decalcification. CONCLUSION: Costal fibrous dysplasia is a benign lesion which diagnosis is based on microscopic features. Radiologic investigations show nonspecific features but allow to rule out a malignant tumor. The outcome of the patients is generally good except in rare cases with a malignant transformation.

Burkitt lymphoma in a child with Bloom syndrome.

BACKGROUND: Bloom syndrome is a rare disease characterized by chromosomal instability and increased risk of developing lymphoma. OBSERVATION: We report on a case of Bloom syndrome in a 5-year-old boy with Burkitt lymphoma. The diagnosis was suspected by growth retardation, repeated respiratory infections, facial telangiectasia, and a low immunoglobulin level, then confirmed cytogenetically by sister chromatid exchanges. Chemotherapy was poorly tolerated, which required reducing the doses. Unfortunately, it was not sufficient to control the neoplasm and the patient died 14 months after diagnosis. CONCLUSIONS: Cancers in Bloom syndrome are a challenge since the potentially life-threatening side effects of the chemotherapy may require modifications in standard treatment such as dose reduction, which can compromise the tumor prognosis.

[Bronchopulmonary carcinoid tumors: Tunisian experience with 115 surgically treated cases]. / Tumeurs carcinoïdes bronchopulmonaires : une série tunisienne de 115 cas opérés.

INTRODUCTION: Bronchial carcinoid tumors are rare well-differentiated, neuro-endocrine tumors, with low-grade malignancy. They are distinguished in two different groups: typical and atypical carcinoid. The purpose of this study was to review clinical, surgical and pathological characteristics in patients with primary bronchopulmonary carcinoid tumors. METHODS: This retrospective study included 115 bronchial carcinoid tumors surgically treated at a single institution during a twenty-one-year period, between 1992 and 2012. RESULTS: There were 56 men and 59 women, with a mean age of 43.73 years. Hundred patients had typical carcinoids and 15 atypical carcinoids. The majority of patients were symptomatic (96.52%). CT revealed a proximal mass in 51% which was obstructive leading to ventilator disorders in 80%. Bronchoscopy showed an endo-bronchial tumor in 83.48% of the cases. Anatomical resection had been achieved among 99 patients, and a conservative resection among 16 patients with typical carcinoid tumor. Lymph node metastases were present in 12.17% of the cases. Follow-up revealed recurrence in one patient and distant metastasis in 4 others. The 5-year survival rate was worse for atypical carcinoid with 45% than typical carcinoid with 95%. CONCLUSIONS: Carcinoids are rare malignant tumors, and their outcome is usually favorable after surgery. However, local recurrence and/or metastases can occur with both typical and atypical carcinoid tumors, justifying the need of prompt diagnosis and long-term follow-up. The factors influencing the survival included the pathological type, distant metastasis and mediastinal lymph node involvement.

[Surgery for thoracic tuberculosis]. / La chirurgie dans la tuberculose thoracique.

Tuberculosis is mainly a medical disease. Surgery has been the unique therapeutic tool for a long time before the advent of specific antituberculous drugs, and the role of surgery was then confined to the treatment of the sequelae of tuberculosis and their complications. The resurgence of tuberculosis and the emergence of multidrug-resistant TB combined to immunosuppressed patients represent a new challenge for tuberculosis surgery. Surgery may be indicated for a diagnostic purpose in patients with pulmonary, pleural, mediastinal or thoracic wall involvement, or with a therapeutic purpose (drainage, resection, residual cavity obliteration). Modern imaging techniques and the advent of video-assisted thoracic surgery allowed a new approach of this pathology; the majority of diagnostic interventions and selected cases requiring lung resection can be performed through a mini-invasive approach. Patients proposed for aggressive surgery may be treated with the best results thanks to a good evaluation of the thoracic lesions, of the patients' nutritional, infectious and general status combined with a good coordination between the specialized medical team for an optimal preparation to surgery.

[Congenital bronchial atresia in adults]. / Atrésie bronchique congénitale de l'adulte.

Congenital bronchial atresia is a rare congenital obliteration of a segmental or lobar bronchus resulting in distension of the corresponding parenchyma. It is seldom diagnosed in the adult. It may lead to infectious complications and, in the long term, to damage to the adjacent lung parenchyma. A surgical resection is necessary and it can be achieved by thoracoscopy. We report a recent series of six patients.

[Detection of the first strain of glycopeptide intermediary Staphylococcus aureus in Tunis Rabta hospital]. / Détection de la première souche de Staphylococcus aureus de sensibilité diminuée aux glycopeptides à l'hôpital la Rabta de Tunis.

Since the advent of the first glycopeptide intermediately susceptible Staphylococcus aureus (GISA) and its heterogeneous variant hGISA in 1997, debate still ensues as their clinical significance. We report here the first case of GISA in Rabta hospital of Tunisia. Antimicrobial resistance was determined by the disk diffusion method in accordance with CA-SFM (Comity of Antibiogramm of French society of Microbiology). The MIC of vancomycin and teicoplanin was determined by E-test. The detection of mec A gene, virulence factors genes and agr groups (1-4) was performed by multiplex PCR. spa types were determined with the assistance of Ridom of Staph Type software (Ridom GmbH, Wurburg, Germany). The allelic profiles of MRSA were assigned on the basis of their MLST type using the eBURST program. A MRSA bacteraemia patient was treated with teicoplanin for 14 days. S. aureus isolated from patient's blood culture was identified as MRSA and GISA with teicoplanin MIC of 16 mg/l. The molecular study of this strain showed that it belongs to the clonal complex CC8 and is attached to the iberian clone (agr1, enterotoxin A, ST 247, spa type t052). Clinicians and laboratories alike are increasingly aware that patients on long-term vancomycin therapy may signal the presence and potential spread of hGISA/GISA strains. hGISA/GISA strains emerged from lineages with agr types I and II. The multiresitance of the Iberian clone ST247 could be explained by the presence of several resistance genes.

[Profuse subcutaneous nodules in a Tunisian patient: Kaposi sarcoma revealing AIDS]. / Nodules sous cutanés profus chez un Tunisien: maladie de Kaposi révélatrice de SIDA.

The purpose of this report is to describe a case involving Kaposi sarcoma as the inaugural manifestation of HIV infection at the full-blown AIDS stage. The patient was a 59-year-old Tunisian man who presented with profuse subcutaneous nodules and multiple lymph nodes. Treatment was based on antiretroviral therapy in association with radiotherapy.

Histological characterization of Darier's disease in Tunisian families.

BACKGROUND: Darier's disease (OMIM 124200) is an autosomal-dominant skin disorder characterized by warty papules and plaques in seborreheic areas, palmo-plantar pits and distinctive nail abnormalities. The disease has complete penetrance in adults and variable expressivity. It is caused by mutations in the ATP2A2 gene, which encodes the sarco/endoplasmic reticulum Ca(2+) ATPase type 2 isoform (SERCA2). OBJECTIVE: We report histological investigations of six unrelated Tunisian families including 15 affected individuals with Darier's disease mutations. RESULTS: The typical histological features of Darier's disease have been observed in the 15 patients. Variable histological features have been observed among Tunisian patients ranging from mild to moderate lesions of Darier's disease. A significant correlation has been observed between the clinical presentation of the Darier's disease (mild or moderate) and the intensity of the histological features. Isolated acral form of Darier's disease was seen in one case. Two distinct original associations have been observed: Darier's disease/pemphigus vulgaris in one patient and Darier's disease/ichtyosis in the other patient. CONCLUSION: Our findings confirmed the clinical heterogeneity of Darier's disease on the basis of histological study. The intensity of the histological features could be closely correlated to the severity of Darier's disease clinical presentation.

[Darier's disease: an evaluation of its neuropsychiatric component]. / Les manifestations neuropsychiatriques de la maladie de Darier : résultat préliminaire d'une étude epidémioclinique et génétique de huit familles.

BACKGROUND: Darrier's disease is a rare genodermatosis with a dominant autosomic penetrating variable transmission. The association between Darier's disease and neuropsychiatric disorders has been reported since 1996. Moreover, associations with mental retardation, schizophrenia, mood disorders and suicide have been reported. The discovery in 1999 of the ATP2A2 gene of Darier's disease, localised on chromosome 12, allowed significant advances notably in understanding the pathogenicity of these symptoms. MATERIAL AND METHODS: In this article, we present the preliminary results of a clinical and genetic study of eight Tunisian families, involving dermatologists, psychiatrists and geneticians. Eight patients with Darier's disease and their first degree relatives were included in the study after they had given their written consent. Thirty-five subjects were examined, 23 of them had Darier's disease. All patients were submitted to a complete clinical examination; notably dermatological screening, genetic inquiry and blood tests for haplotype analysis. Only 13 of them underwent a psychiatric examination. RESULTS: The psychiatric examination was carried out only in 13 patients with Darier's disease, who revealed neuropsychiatric symptoms with a frequency of 61.1% (8/13). Two patients presented mild mental retardation; six patients had mood disorders, three of them belonged to the same family (two had recurrent depression, four belonged to the bipolar spectrum [2 bipolar disorder type 2, 2 cyclothymia]). The coexpression of the two distinct phenotypes (Darier's disease and bipolar disease), within the same three members' of the family of our study, suggests the existence of a genetic linkage between the two diseases, as has been reported in the literature.

[False aneurysm of left ventricle after nonpenetrating chest trauma (about one case)]. / Faux anévrisme du ventricule gauche après un traumatisme fermé du thorax (à propos d'un cas).

Ischemic heart disease is the most common etiology of aneurysms of the left ventricle. The latter can also result from trauma. We report a case of a patient operated for false aneurysm of the left ventricle revealed by dyspnea and occurring six years after a nonpenetrating chest trauma. Follow-up after surgery was good with no complication occurring. In conclusion, this lesion must be considered in case of chest trauma and can be easily detected by echocardiography or CT scan.