Interest of Thin Skin Flaps in the Treatment of Postburn Cervical Contractures: About Five Cases.

OBJECTIVES: The use of thin skin flaps in the reconstruction of postburn cervical contractures associated with functional impacts. MATERIAL AND METHODS: We conducted a descriptive observational study on about five patients who had reconstruction of postburn cervical contractures using various thin skin flaps in a plastic surgery department. The follow-up period was ranged from 12 to 18 months. We determined the characteristics of the retraction according to the Vandenbussche classification, the indications of the flaps, and the functional and aesthetic results. RESULTS: Most of our patients were young adults presenting segmental retraction of the neck associated with moderate functional impact. The occipito-cervico-dorsal flap was used in three cases. The occipito-cervico-shoulder flap was used in two cases. The aesthetic and functional results were satisfactory for all patients, but a "neck-collar" aspect of the cervical-chin angle was noticed. CONCLUSION: Postburn cervical contractures remain frequent. Their reconstruction is a difficult process as it is ideally done with a skin similar to the original one. The use of thin skin flaps seems to be an excellent option.

Epidemiological profile of burns in children in central and southern Tunisia: A 67-case series.

INTRODUCTION: Burns are among the most frequent injuries in children. They are a major cause of morbidity and mortality in low- and middle-income countries. This study aimed to describe the epidemiological aspects of burns in this environment and to propose preventive measures. PATIENTS AND METHODS: A retrospective study was conducted between 1 January 2012 and 31 December 2013. It included children less than 16 years old who were hospitalized for burn injuries in our department. We adopted the hospitalization criteria proposed by the French Society for the Study and Treatment of Burns. The data were collected from medical records and concerned both patients and burn characteristics. RESULTS: We recorded 67 cases, with 44.7% under 4 years old and boys (61%) more involved than girls (39%). The incidence of burns peaked in winter (31%) and summer (27%). Burns occurred at home in 95% of the cases. Hot liquids were the leading cause of children's burns. The time lapse between the accident and admission to the hospital was less than 24h in 57% of the cases. The average total body surface area (TBSA) burned was 8.8%. Only five patients presented a TBSA≥20%. The depth of the burns was superficial second-degree burns in 59% of cases. The burn involved mainly the upper limbs (65%). The average length of the hospital stay was 20.5 days. Only one patient died from severe sepsis. CONCLUSION: The lack of specialized burn centers in Tunisia associated with the low socioeconomic level of our population worsened the outcome of pediatric burns. The best solution lies in prevention.

La particularité de cicatrisation des pertes de substance cutanées dans les brûlures électriques: notre expérience.

Electrical burns are a major cause of bodily harm due to the mechanism and effect of the lesions. This prompts us to study these lesions and their management in order to reduce the morbidity caused by this type of accident. We conducted a retrospective descriptive observational study of patients hospitalized for electrical burns. This study includes 23 patients. The average age was 25.74 years. The most common occurrence was a domestic accident in 13 cases (56.5%), a work accident in 8 cases (34.8%), and a road traffic accident in 2 cases (8.7%). Fasciotomy was performed on six patients: five had high voltage injuries. Necrosis excision was performed at 8.26 days ± 5.55 days. Seven amputations were performed including 6 at the upper limb. Twenty flaps were used on 12 patients. Locoregional flaps were performed for 15 zones, and distant flaps as a solution in the event of locoregional flap failure. Five cases had immediate reconstruction because of exposure of noble elements, 3 showing necrosis of the flap. The average healing time was 45 days. Seven patients who underwent a reconstruction by flap healed after this period. The main results of the study show that conventional emergency decompression does not appear to reduce the amputation rate, the use of local and locoregional flaps in the initial phase (<21 days) carries a significant risk of suffering and necrosis, and that antithrombotic prevention or the use of flaps does not seem to have an impact on healing delays.

Les complications de l'expansion cutanée dans le traitement des séquelles de brulures.

Tissue expansion in burn sequelae has been associated with high complication rates. A critical analysis of 8 years (2008-2015) of experience with 45 expanders placed in 31 consecutive burns sequelae was performed to identify those factors that predispose to complications. Major and minor complications occurred in 33.4% of procedures. The factor associated with a statistically significant increase in complications was limbs localization. In addition, complications were significantly more likely to occur within the second stage. Factors that did not influence complication rate included patient age, the volume of the expander, and aesthetic or functional indications.

[Etiological profile of secondary Raynaud's phenomenon in an internal medicine department. About 121 patients]. / Profil étiologique d'un phénomène de Raynaud secondaire dans un service de médecine interne. À propos de 121 patients.

INTRODUCTION: Raynaud's phenomenon is a reversible episodic vasospastic disorder triggered by cold or emotion. Two types of Raynaud's phenomenon were distinguished: Raynaud's disease and secondary Raynaud's phenomenon. The purpose of this study was to determine the etiologic profile of secondary Raynaud's phenomenon in an internal medicine department. METHODS: A descriptive retrospective study including patients with secondary Raynaud's phenomenon followed in a tertiary internal medicine department between 2000 and 2013. RESULTS: We included 121 patients. The sex ratio M/F was 0.16. The mean age at the onset of Raynaud's phenomenon was 41.7 years. The average age of patients at the time of the etiologic diagnosis was 47.3 years. The mean delay between Raynaud's phenomenon onset and the first consultation was 41.33 months. Raynaud's phenomenon involved hands in all cases and feet in 16.10% of cases with a typical form in most cases (41.4%). Complications (digital ulcers and scars) were noted in 32.23% of cases. Nail fold capillaroscopy showed scleroderma pattern in 49.52% of patients. Antinuclear antibodies were positive in 88.49% of patients. Interstitial lung disease was reported in 54.04% of cases. Connective tissue diseases were diagnosed in 86.77% of patients. Other secondary Raynaud's phenomenon causes were vasculitis (6.61%), atherosclerosis (1.65%) and medical or professional causes (1.65%). The most frequent one cause systemic sclerosis (n=61, 98%) followed by systemic lupus erythematosus (11.57%) and primary Sjögren syndrome (6.61%). CONCLUSION: In our study, the Raynaud's phenomenon was most frequently secondary to connective tissue diseases. This may be a selection bias because our department is a third-line unit where patients are often referred for systemic disease suspicion.

Headache in a young woman: do not forget Susac's syndrome.

Susac syndrome is a rare disease characterized by the clinical triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. This underdiagnosed condition needs to be considered in the differential diagnosis of a broad variety of disorders. An early diagnosis is important as treatment can halt disease progression and prevent permanent disability. Herein, we report a case of Susac syndrome in a 31-year-old woman and we highlight how challenging an early diagnosis was and the importance of an aggressive therapeutic approach, including the combination of steroids and other cytotoxic drugs.

Granulomatosis with polyangiitis in Tunisia.

Granulomatosis with polyangiitis (GPA) is more frequent in Northern rather than Southern countries. Very few studies have been conducted in Africa. We have performed a retrospective descriptive study including clinical and laboratory profiles of 30 Tunisian GPA patients seen at the department of Internal Medicine of the University Hospital of la Rabta from 2000 to 2014. Mean age at initial GPA diagnosis was 46±12 years, and the average number of months between the onset of symptoms and diagnosis was 25. Seventeen (56%) were male, and 13 (44%) were female. Ear/nose/throat involvement occurred in 83%. Lung and renal involvement were observed in respectively 70% and 56% followed by mucocutaneous (50%), neurological (50%), ocular (33%), vascular (20%), ureteral (16%), and cardiac involvement in 10%. Cytoplasmic pattern-antineutrophil cytoplasmic antibodies (ANCA) was detected in 27 (90%) patients. Induction therapy consisted of intravenous cyclophosphamide pulses in 27 patients (90%) and oral methotrexate in 3 patients (10%). Trimethoprime-sulfamethoxazole was used in 26 patients (86%). Maintenance therapy consisted of azathioprine in 17 cases and methotrexate in 13 cases. Relapses occurred in 36%. Eighteen patients had favorable outcome and 12 died. Our patients had a distinct phenotype with high prevalence of pleural involvement, lymph node enlargement, sensorimotor neuropathy and ureter stenosis. ENT symptoms were less frequent as inaugural presentation. Overall 2-year survival was 60%.

Penconazole alters redox status, cholinergic function, and membrane-bound ATPases in the cerebrum and cerebellum of adult rats.

Pesticides exposure causes usually harmful effects to the environment and human health. The present study aimed to investigate the potential toxic effects of penconazole, a triazole fungicide, on the cerebrum and cerebellum of adult rats. Penconazole was administered intraperitoneally to male Wistar rats at a dose of 67 mg kg-1 body weight every 2 days during 9 days. Results showed that penconazole induced oxidative stress in rat cerebrum and cerebellum tissues. In fact, we have found a significant increase in malondialdehyde, hydrogen peroxide, and advanced oxidation protein product levels, as well as an alteration of the antioxidant status, enzymatic (superoxide dismutase and catalase) and nonenzymatic (glutathione), the cholinergic function, and membrane-bound ATPases (Na+/K+-ATPase and Mg2+-ATPase). Penconazole also provoked histological alterations marked by pyknotic and vacuolated neurons in the cerebrum and apoptosis and edema in the cerebellum Purkinje cells' layer. Therefore, the use of this neurotoxicant fungicide must be regularly monitored in the environment.

Intracardiac thrombus in Behçet's disease.

Behçet's disease (BD) is a multisystem inflammatory disorder. Intracardiac thrombus (ICT) formation is an uncommon but important complication of BD. Of the cases of Behçet's disease, we selected those with ICT. All patients fulfilled the diagnostic criteria of the International Study Group of Behçet's disease. The ICT in each case was confirmed by ultrasonography, computed tomography and MRI. Clinical features and laboratory parameters were determined. Among our 518 patients with BD, 8 were diagnosed as having intracardiac thrombus (ICT). All were male; the mean age at the time of the ICT diagnosis was 30.8 years. The main presenting symptoms were hemoptysis, chest pain, and dyspnea. It was associated with pulmonary artery aneurysm and vena cava thrombosis in 3 cases each, pulmonary embolism, and lower limbs deep venous thrombosis in 1 case each. The coexistence of other cardiac complications was as follows: pericarditis in 2 cases, myocarditis, endomyocardial fibrosis, and coronary arteritis with consequent myocardial infarction in one case each. In all cases, echocardiography was sufficient to reach the diagnosis. Chest computed tomography performed in all cases led to the diagnosis of associated pulmonary vasculo-Behçet lesions in 4 cases. All patients received colchicine, anticoagulation, and corticosteroids. Seven patients were on immunosuppressant agents (2 patients received azathioprine and 5 cyclophosphamide). Clinical remission with ICT resolution was observed in 5 cases. Combined immunosuppressive therapy with prednisone and cyclophosphamide might be needed to treat ICT due to BD.

Successful treatment of refractory adult onset Still's disease with rituximab.

Adult-onset Still's disease (AOSD) is an uncommon inflammatory condition of unknown origin. In chronic disease, joint involvement is often predominant and erosions are noted in one third of patients. Therapeutic strategies derive from observational data. Corticosteroids are usually the first-line treatment. With inadequate response to corticosteroids, methotrexate appears the best choice to control disease activity and allow for tapering of steroid use. For refractory disease, biological therapy seems the most promising. We report here the case of a 38-year-old female patient with AOSD refractory to cytotoxic agents, treated by rituximab infusion therapy with favorable outcome.

Lupus anticoagulant-hypoprothrombinemia syndrome presenting with co-existing cerebral venous thrombosis and subdural hemorrhage.

The lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) - the association of acquired factor II deficiency and lupus anticoagulant - is a rare disease that may cause a predisposition not only to thrombosis but also to severe bleeding. We are reporting on a 36-year-old female patient presenting with co-existing cerebral venous thrombosis and subdural hemorrhage. The coagulation screening showed a prolonged prothrombin time (PT), activated partial thromboplastin time (aPTT), and a normal fibrinogen level and platelet count. Evaluation of the clotting factors revealed decreased levels of factors II (37%). Factors V, VIII, IX and XI were normal. Lupus anticoagulant (LA) was demonstrated by the Dilute Russell's Viper Venom Test (DRVVT). Immunological work-up was positive for IgG type anticardiolipines antibodies (aCL). Successful management consisted first of oral prednisone (60mg/d). Thus, anticoagulation was introduced once factor II had stabilized.

Microscopic polyangiitis associated with primary biliary cirrhosis, Sjogren's syndrome and Hashimoto's thyroiditis.

The association between microscopic polyangiitis (MPA) and primary biliary cirrhosis (PBC) has seldom been reported. We describe here a patient who presented with sensorimotor neuropathy along with hypothyroidism, renal failure and liver dysfunction. Detection of antinuclear antibodies at a titer of 1/800, anti-SSA, anti-SSB, anti-GP210, anti-microsomial and p-ANCA anti-myeloperoxydase antibodies along with renal, salivary and liver biopsy led to a diagnosis of MPA associated with PBC, Sjogren's syndrome and Hashimoto's thyroiditis.

Polymorphisms of the NOS3 gene in Tunisian patients with Behçet's disease.

Behçet's disease (BD) is a multisystem inflammatory disease characterized by recurrent orogenital ulceration, ocular inflammation and skin lesions. Reduced plasma nitric oxide (NO) levels in patients with BD have been implicated in the development of the endothelial abnormalities and thrombotic complications occurring in these patients. Polymorphisms in the endothelial nitric oxide synthase gene (NOS3) have been inconsistently associated with BD. This inconsistency may derive from population stratification secondary to ethnic diversity, and consideration limited to only one rather than combinations of polymorphisms. We studied three genetic variations in the NOS3 gene: a single nucleotide polymorphism in the promoter region -786T>C, in exon 7 (Glu298Asp), and a variable number of tandem repeats in intron 4 (4a4b) of the NOS3 gene in 100 unrelated Tunisian patients with BD and 148 healthy controls. In addition, we also examined the association of NOS3 gene haplotypes with BD. Analyses of the Glu298Asp, -786T>C and 4a4b polymorphisms were made by the polymerase chain reaction (PCR) restriction fragment length polymorphism technique and PCR genotyping, respectively. The distribution of the Glu298Asp genotype differed significantly between patients with BD and controls (P = 0.01). Allele Asp298 was significantly more frequent in patients with BD than in controls (P = 0.005, OR = 1.70, 95% CI 1.14-2.54). In contrast, distribution of alleles and genotypes of -786T>C and 4a4b polymorphisms was not different between the control and BD group. However, the frequency of Asp-T-4b haplotype was significantly higher in patients with BD than in healthy controls. By gender, the signification remained only for heterozygous men (P = 0.03) and homozygous women (P = 0.02). These results suggest that Glu298Asp polymorphism of the NOS3 gene is associated with BD susceptibility in Tunisian patients.

Amyloidosis in Behcet's disease.

Behcet's disease (BD) is a multisystem vasculitis with protean manifestations. It is characterized by a heightened state of inflammation, although the factors that initiate and sustain this inflammation are not clear. We report some cases of BD-associated amyloidosis and have similar features. The patients developed nephrotic syndrome due to secondary amyloidosis, which was refractory to the immunosuppressive agents. Two patients expired and the third was lost to follow-up during the course. The BD complicated with amyloidosis is associated with high mortality despite the current aggressive therapy.

Inflammatory optic neuropathy in Behçet's disease.

Inflammatory optic neuropathy (ON) is a rare event in Behçet's disease (BD). We report herein a series of ten BD Tunisian patients with ON and describe its clinical features among them. A retrospective review of BD patients (International Study Group for BD criteria) was performed. The patients were divided into two groups: those presenting an inflammatory ON, and those none. The diagnosis of inflammatory ON was based on the clinical examination, visual field and visual evoked potentials. We analyzed the characteristics of the two groups. Ten patients (2.3%) presented an inflammatory ON among our 440 patients. Inflammatory ON was inaugural in 8 cases. Clinical manifestations were as follows: blurred vision (7 cases) and periorbital pain (3 cases). In two cases, the patients did not complain from ophthalmological symptoms. The fundus revealed a papilledema (2 cases), papillary pallor (4 cases), and was normal in 5 cases. Visual field realized in only three patients showed a scotoma in all cases. Visual evoked potentials revealed increased latency in all cases. All patients received corticosteroids associated to an immunosuppressive agent. The comparative study between the two groups revealed that inflammatory ON was significantly more associated to neurological involvement (p<0.0001) and that the disease was more severe in the ON group (p<0.0001). Inflammatory ON in BD is rare and may occur at an early stage of the clinical course of the disease. Its prevalence is certainly underestimated. A systematic visual evoked potential may be interesting as a screening tool.

Chest pain in patients with undiagnosed Behçet's disease.

Behçet's disease (BD) is a systemic inflammatory disease having a chronic and prolonged course with 4 major symptoms: oral and genital ulcerations, eye disease and cutaneous manifestations, as well as other multisystem involvements. Arterial involvement is a comparatively rare complication in BD and coronary lesions are extremely rare. We report here two cases of BD presenting as myocardial infarction (MI) with coronary artery aneurysm (CAA), with good improvement after immunosuppressive therapy.

Microscopic polyangiitis presenting with peripheral and central neurological manifestations.

Although peripheral neuropathy is a common complication of microscopic angiitis, manifestations involving the muscle and the central nervous system have been rarely reported. We describe a 48-year-old man who rapidly developed a clinical picture of mononeuritis multiplex. A month after the appearance of the primary symptoms, he became comatose and had left hemiplegia in relation with a massive cerebral haematoma. Laboratory data revealed signs of inflammation, glomerular dysfunction with microhaematuria, and positive myeloperoxidase-antineutrophil cytoplasmic antibodies. The neuromuscular biopsy disclosed a small-vessel vasculitis, consisting with microscopic angiitis, associated with myositis and extensive axonal loss. The patient had surgical evacuation of the haematoma and received immunosuppressive therapy with good outcome. Thus, microscopic angiitis should be considered as a differential diagnosis in cases of myositis and intracerebral haemorrhage.

[Chorea revealing a polycytemia vera]. / Chorée révélant une polyglobulie primitive.

Neurological manifestations in polycytemia vera are common. However, chorea is an exceptionally revealing feature of this disease. We report a 78-year-old man who presented with headache and an abnormal movement disorder corresponding to chorea. Laboratory findings showed increased levels of hemoglobin at 20 g/dl and hematocrit at 62.3%. An elevated erythrocyte mass to twice the normal value demonstrated the absolute erythrocytosis. A JAK2 V617F gene mutation was identified. A diagnosis of polycytemia vera-associated chorea was obtained. Clinical and biological outcomes were favorable after therapeutic phlebotomy and treatment with hydroxyurea. We recommend a complete blood cell count in elderly patient presenting with chorea to eliminate a diagnosis of polycytemia vera.

[Early breast cancer without axillary lymph node involvement]. / Cancer du sein sans atteinte ganglionnaire axillaire.

Breast cancer is the most common cancer in women. In France, breast cancer incidence was 88.9 per 100,000 women in 2000. Early detection of breast tumours by screening mammography allows a breast conserving treatment, i.e., breast irradiation preceded by a lumpectomy and a sentinel node biopsy. The standard irradiation of early breast cancers consists in delivering 50 Gy to the whole breast in 2 Gy fractions over a five-week period followed by an additional dose targeting the tumour bed of 16 Gy in eight fractions. The 3D-conformal treatment planning optimizes dose distribution to the whole breast and to the tumour bed and lessens the normal tissue irradiation (heart and ipsilateral lung). The aim of this article is to describe epidemiologic, radio anatomic and prognostic features of early stage breast cancer and to propose guidelines for 3D-conformal treatment planning in early breast cancers. This review is illustrated by a case report.

[Breast cancer with axillary lymph node involvement]. / Cancer du sein avec atteinte ganglionnaire axillaire.

Breast cancer is the most frequent cancer of women in western countries. There are one million new cases per year in the world which represents 22% of all female cancers, and more than 370,000 deaths due to breast cancer per year (14% of cancer mortality). More than half of breast cancers are associated with axillary nodal involvement. Post-operative radiation therapy (XRT) is a crucial part of locoregional treatment in axillary nodal involvement breast cancer owing to a 15-years risk reduction of locoregional recurrence of 70% and to a 5.4% risk reduction of specific mortality. In 3D-conformal irradiation in such breast cancers, target volumes are chest wall when mastectomy was performed or breast and boost of tumor bed in case of breast conservative surgery, and supra-clavicular and/or axillary and/or internal mammary node areas. The main organs at risk are ipsilateral lung, heart and brachial plexus. The aim of this article is to describe epidemiologic, radio anatomic and prognostic features of axillary nodal involvement breast cancer and to propose guidelines for 3D-conformal treatment planning in locally advanced breast cancers. This review is illustrated by a case report.