Is BRAF a prognostic factor in stage III skin melanoma? A retrospective study of 72 patients after positive sentinel lymph node dissection.

BACKGROUND: BRAF was identified as an oncogene in skin melanoma in 2002, and since 2011 has been a therapeutic target in the treatment of metastatic melanoma. The role of BRAF mutation in tumour initiation and the disease course remains to be elucidated. OBJECTIVES: The main objective of our study was to determine whether there is a relationship between BRAF status and overall survival in patients with a melanoma and a positive sentinel lymph node. We also sought an association between BRAF status and the clinicopathological features of the melanoma. Finally, we looked for a potential heterogeneity of BRAF status in primary and metastatic tumours. METHODS: All patients (n = 72) treated for melanoma and with a positive sentinel lymph node at the University Hospital of Clermont-Ferrand, France, between January 2000 and January 2010 were enrolled in the study. We investigated BRAF status in primary melanoma and lymph node metastatic tissue in our molecular pathology laboratory and collected the clinical and survival data. RESULTS: Of the 72 patients, 32 had at least one BRAF mutation. There was a statistically significant difference in overall survival between the BRAF-mutated and wild-type populations. The only clinical feature related to BRAF status was metastatic burden. Of the 25 patients in whom we obtained the status in both locations, five had a discordant result. CONCLUSIONS: BRAF mutation is an indicator of poor prognosis in patients with stage III melanoma with a positive sentinel lymph node. BRAF status could be used in the staging of this population. BRAF has a role not only in cellular immortalization but also in metastatic spread.

[Intermaxillary intraoperative fixation of mandibular fractures: arch bars or fixation screws?]. / Blocage maxillo-mandibulaire peropératoire des fractures de mandibule : arcs ou vis de blocage ?

INTRODUCTION: The gold standard management of mandibular fractures is open reduction and osteosynthesis associated with intermaxillary fixation. The use of intermaxillary fixation screws for 20 years has considerably reduced the number of intermaxillary fixation with arch bars. The aim of our review was to identify current indications and contraindications of each technique. INTERMAXILLARY FIXATION TECHNIQUES: We present a short history and compare the two techniques with recently published assets, drawbacks, and complications. DISCUSSION: The indications of intermaxillary fixation screws are uni- or bifocal fractures without or with minimal displacement. Their use is contraindicated in any other type of fracture, which should still be treated with arch bar fixation.

Bilateral cervical chondrocutaneous remnants: a familial observation.

Cervical chondrocutaneous remnants are extremely rare, and fewer than 40 unilateral, and only 11 bilateral, forms have been reported. The clinical and ultrasonographic criteria have been well characterised. Several associated anomalies may accompany the remnants and patients should therefore be investigated further. The treatment of choice is complete excision to confirm the diagnosis. Histological investigations define this lesion as heterotopic, composed of normal skin and fat with a strip of cartilage running through the middle. The embryological origin is not clear. We report what is to our knowledge the first familial form in 6 members of a Libyan family all of whom had unilateral or bilateral forms. We focus specifically on a 10-year-old girl with bilateral chondrocutaneous remnants who was admitted to our department.

[Conservative surgery for a mandibular condyle osteochondroma]. / Chirurgie conservatrice d'un ostéochondrome du condyle mandibulaire.

INTRODUCTION: Osteochondroma is a rare benign bone tumor of the mandibular condyle (90 cases reported). The symptoms are similar to those of temporomandibular joint disorders: painful temporomandibular joint, joint sounds, and modification of dental occlusion. CASE REPORT: A 37-year-old female patient consulted for a wide left laterodeviation of the mandible. CT scan revealed a 27 mm long bony growth on the condyle. The condyle neck was osteotomized, then the tumor and condyle head were split, the condyle was then remodeled and osteosynthesized on the condyle neck. The functional and esthetic results were satisfactory at 12 months without any recurrence or relapse. DISCUSSION: The authors present a novel conservative technique, slightly different from those reported in the literature. There are several therapeutic alternatives. They can be classified in three groups: invasive, non-invasive, and conservative techniques. Nevertheless, osteochondroma is a benign tumor and its management should be as little invasive as possible.

[Frontal sinusitis, mucocele and subperiosteal superolateral orbit abscess]. / Sinusite frontale, mucocèle et abcès orbitaire supérolatéral.

INTRODUCTION: Periorbital infection following pansinusitis is a rare complication which can lead to irreversible orbital and intracranial consequences. Chandler classified them in 1970. OBSERVATION: An eight-year-old child presented with a subperiosteal superolateral abscess. The initial flu-like syndrome developed into pansinusitis resisting to usual treatment. Palpebral edema and major exophthalmia then appeared requiring CT scan and MRI, which confirmed a subperiosteal abscess. DISCUSSION: A subperiosteal abscess is a serious condition requiring an early diagnosis and a medical or surgical treatment adapted to the severity. Chandler's classification gives the opportunity to adapt management to the infection's gravity. Ethmoiditis may be complicated by periorbital abscesses found usually in the superointernal quadrant. Medical imaging and surgical treatment confirmed a superolateral periorbital abscess and a frontal mucocele. This case illustrates the possibility of communication between frontal sinus and orbital cavity. During frontal sinus infection, pre-existing mucocele may become super-infected and induce a periorbital abscess.

[Acute dacryocystitis complicating primary mononucleosis infection]. / Dacryocystite aiguë sur une primo-infection mononucléosique.

INTRODUCTION: Infectious mononucleosis may lead to numerous complications. Tonsillar hyperplasia with risk of airway obstruction is well known. Dacryocystitis is a rare but potentially severe complication. OBSERVATION: A 6-year-old child with primary mononucleosis infectious diagnosed 8 days before, developed acute dacryocystitis, with rapid evolution to orbital cellulitis, despite adequate antibiotherapy. Emergency surgical drainage was required. DISCUSSION: Dacryocystitis is a rare and little documented complication of EBV infection. Its acute evolution to orbital cellulitis is possible and potentially severe. Its physiopathology is specific. Patients are initially free of chronic stenosis and epiphora, which express acute obstruction of the lachrymal sac due to general lymphoid hyperplasia.

Complete congenital agenesis of all major salivary glands: a case report and review of the literature.

Congenital agenesis of the salivary glands is uncommon. There are documented cases of partial or unilateral aplasia of the major salivary glands associated with the lacrimal puncta, but very few reports of the absence of all major salivary glands. We report the case of a 10-year-old girl with xerostomia and extensive teeth caries. Physical examination and imaging showed total and bilateral aplasia of the parotid, submandibular, and sublingual glands, with no involvement of the minor salivary glands or the lacrimal puncta. We describe the clinical presentation, important aspects in diagnosing partial forms of the condition, and patient management.

Calcium polyuronate dressing supplemented with zinc and manganese (trionic®) in necrotizing dermohypodermitis of the extremities: a randomized multicentre study.

Background. For the burn surgeon, the treatment of necrotizing soft tissue infections is one of the most demanding surgical emergencies, requiring "radical excisions" of the infected tissue and reconstruction. During the infection period, the excised sites are treated with application of gauzes soaked in saline solution. When the septic period is over, the excision sites are usually covered by sterile paraffin gauze dressing. Our aim was to evaluate a new calcium polyuronate dressing enriched with zinc and manganese ions (test group) versus the reference therapeutic combination (control group) from the septic period to the grafting of skin. Materials and methods. A multicentre, prospective, controlled, randomized clinical trial was conducted from November 2003 to July 2005. The primary endpoint was the waiting period for carrying out the skin graft and the percentage of grafted patients at 28 days after the last excision. The secondary endpoints were blood loss, exudates amounts, and pain during dressing changes. Results. Twenty-five patients were included, 14 with the new dressing and 11 with the reference therapeutic combination. The average waiting period for skin graft was 18 days in the test group versus 27.1 days in the control group (p = 0.128). All the patients in the test group received their grafts within 28 days after the last excision, compared with 60% (p = 0.043) in the control group. Bleeding during dressing change was statistically lower in the test group: 45.5% of the patients did not bleed compared with 0% in the control group (p = 0.045). Treatments were well tolerated. Conclusion. The properties of this new calcium polyuronate enriched with zinc and manganese ions seem to accelerate granulation tissue development, allowing skin grafting earlier in favourable conditions with less bleeding and less pain during dressing renewal.

[Giant cell tumors and pseudogiant cell tumors of the jaws]. / Tumeurs et pseudotumeurs des maxillaires riches en cellules géantes.

Giant cell tumors and pseudogiant cell tumors of the jaws are divided into cherubism, central giant cell granuloma (CGCG), aneurysmal cyst, giant cell tumor, and hyperparathyroidism-jaw tumor. These tumors have variable clinical characteristics, such as bilateral tumors in cherubism, disseminated tumors in hyperparathyroidism, or focal tumors in other types. Their radiological presentation is quite similar with presence of well-delimitated uni- or multilocular radiolucency. The primary surgical treatment must be conservative. Anatomopathology gives the histologic diagnosis, with presence of giant cells. Some tumors are locally aggressive leading to larger bone exeresis. The nosology of these giant-cell tumors remains unclear, especially for giant cell central granuloma and giant cell tumors.

[Velopharyngeal sequels in labial-alveolar-velopalatine clefts. Analysis, conclusions and speech therapy management]. / Les séquelles vélopharyngées dans les fentes labioalvéolopalatovélaires. Analyse, bilan et prise en charge orthophonique.

The association of voice and speech defines phonation. Phonation depends on various organs: the lungs for air, the larynx as a vibration generator, and cavities of resonance modeling articulation and tone. The role of the soft palate is important in French and for everyday conversation. Velopharyngeal incompetence is defined as a voice and articulation disruption by anatomic and/or functional deficiency between the soft palate and pharynx. Hypernasality is studied on an acoustic, phonetic, and vocal level and graded in various degrees of hypernasality. The various clinical and perceptive tests of velopharyngeal incompetence are described according to age. The difficulty to modelize hypernasality is discussed based on a recent study published in 2007. The differential diagnosis with hyper-closed nasality is discussed. The treatment for this condition is essentially speech therapy associated with pharyngoplasty in some cases. The assessment of children born with a labial-alveolar-velopalatine cleft is mainly clinical and perceptive. Therapeutic management is necessary for the child and his family. The follow-up requires a multidisciplinary approach.

[Regression in primary cutaneous melanoma is not predictive for sentinel lymph node micrometastasis]. / Absence de valeur prédictive des signes de régression histologique sur l'envahissement du ganglion sentinelle.

BACKGROUND: The predictive value of regression in melanoma is debated. AIM OF THE STUDY: A retrospective single-centre study to evaluate the correlation between regression in primary skin tumor and the presence of micrometastases in sentinel lymph nodes. PATIENTS AND METHODS: Histological signs of regression in 84 melanomas (>1 mm) with corresponding sentinel lymph nodes were studied by two independent pathologists. RESULTS: Regression was seen in 40 skin melanoma tumors while micrometastasis was seen in 24. Of the tumors with micrometastasis, only 10 were regressive (RR: 0.47, p=0.49). Breslow value>2 mm and male sex were predictive for node micrometastasis (RR: 4.6, p=0.03 and RR: 7.6, p=0.006, respectively). On multivariate analysis, these two factors were independent. COMMENTS: These data suggest that regression in primary cutaneous melanoma is not predictive for lymph node metastasis.

Comparison of a propofol target-controlled infusion and inhalational sevoflurane for fibreoptic intubation under spontaneous ventilation.

BACKGROUND: A propofol target-controlled infusion (TCI) is often proposed for the management of difficult airway intubation and fibreoptic intubation under anaesthesia and spontaneous ventilation. No data are available about sevoflurane. The aim of the present study was to compare propofol and sevoflurane as hypnotics during fibreoptic intubation under spontaneous ventilation. METHODS: After regional ethical committee approval, 52 ASA I-II patients without any predictors for difficult intubation gave their informed consent. They were randomly assigned to one of two groups. After 3 min of pre-oxygenation, patients received either propofol with a plasmatic target concentration of 4 mg/l (group P; n= 26) or sevoflurane 4% with tidal volume ventilation (group S; n= 26). After 2 min, propofol was increased by 1 mg/l and sevoflurane was increased by 1% every 2 min until there was no reaction during mandible translation. This concentration was maintained for 4 min before starting nasotracheal fibrescopy for intubation. During both induction and fibrescopy, pulse oximetry, bispectral index (BIS), heart rate, and arterial blood pressure were monitored. Quality of intubation and operator satisfaction were evaluated. Data were compared using Student's t-test, Mann-Withney U-test or chi-square test. A P-value < 0.05% was considered to be significant. RESULTS: During induction, no difference in pulse oximetry, BIS values at the end of induction, or duration of induction were noticed. Five episodes of desaturation under 90% occurred during fibreoptic intubation in group P compared with none in group S. CONCLUSION: Sevoflurane provides good fibreoptic intubation conditions to spontaneously breathing patients without any hypoxemic episodes such as those observed with propofol.

[Adenoid cystic carcinoma of the lacrimal gland in a 14-year-old girl]. / Carcinome adénoïde kystique de la glande lacrymale chez une enfant âgée de 14 ans.

The discovery of unilateral exophthalmia requires multidisciplinary care made all the more difficult in the case of a 14-year-old girl living in a tropical environment. The child, C.A., resident of the town of Djenné in Mali, was examined for nonpulsate, nonretractile, left lateral unilateral exophthalmia, painful upon palpation of a left upper-external mass under the orbital rim. The initial examination highlighted the existence of an intraorbital tumor with a moderate effect on visual function. In view of the slow and progressive development over 2 years, and with the assistance of an association, the child was transferred to a specialized medical structure where the tumorous nature was confirmed after surgical exeresis. The diagnosis was cylindroma, or adenoid cystic carcinoma. Neither chemotherapy nor radiation were used. At 15 months after surgery, the child showed no clinical sign of recurrence. Intraorbital tumors in children present diagnostic and therapeutic problems in countries with limited medical resources. Cylindromas or adenoid cystic carcinomas of the lachrymal gland are the second most common cause of epithelial tumors in this gland, which are characterized by a high degree of malignancy, a very high rate of recurrence, and a low survival rate at 5 years. In a tropical environment, diagnostic and therapeutic difficulties are compounded by limits to comprehensive care for the child.

[Congenital midline cervical cleft]. / Fente cervicale médiane congénitale.

INTRODUCTION: The midline cervical cleft is a rare congenital anomaly of the anterior part of the neck. CASE: We report the case of a 9-year old girl, presenting a congenital midline cervical cleft who underwent corrective surgery at the age of 7 months. Currently, outome is quite satisfactory two years post-operatively; no recurrence has developed. DISCUSSION: We discuss the clinical manifestation of this anomaly, the embryology, the surgical treatment and the clinical course. In its complete form, this rare disorder presents as a subcutaneous midline vertical cervical cord, associated with a cutaneous cleft. The cutaneous cleft consists of an ulceration with an overhanging cutaneous fibromatous protuberance which prolongs downwards to a blind-ended sinus tract. The embryologic mechanism is not formally established, but the majority of the authors agree on the imperfect midline fusion of the paired branchial arch tissue by anomaly of mesodermisation. Surgical treatment is required and must be carried out in the first month of the life in order to avoid neck and mandible functional and morphological disorders. Surgery involves complete excision of the cutaneous ulceration and subjacent fibrous cord followed by site closure using multiples "Z" plasties. Long postoperative monitoring is needed due to the frequency of the recurrence.

[Maximal isometric bite force and sports. Preliminary study]. / Forces occlusales maximales isométriques et sport. Etude préliminaire.

INTRODUCTION: The evaluation of the bite forces coupled with EMG activity of masseter muscles allows to point out temporo-mandibular joint disorders. The intense practice of sports induces stress which may affect the mandibular statics, due to an hyperfunction of the elevator masticatory muscles, especially the masseter muscles. This concept has led us to compare the maximum isometric bite forces of sedentary and physically trained subjects. MATERIAL AND METHODS: The aim of this experimentation is to study the maximum isometric bite forces of the premolars, with a force transducer, for two groups of physically trained and sedentary volunteers (25 subjects), with distinction between male and female subjects. Subjects with normal denture and no temporo-mandibular joint disorder were retained. EMG and force recording were synchronized and recorded during 10 seconds of maximal contraction. RESULTS: The male subjects developed a maximal bite-force significantly higher (p < 0.05) compared to the female subjects. There is no significant difference between subjects practising a sport (at least 6 hours a week) and sedentary ones. The evolution of force during the contraction is different between sexes. EMG activity allowed to control that subjects developed a maximal force and an increase in muscular fatigability of physically active females compared to sedentary ones was noticed. DISCUSSION: The difference of maximal force between men and women is similar to the one found for the locomotor muscles. The analysis of the evolution of the force according to the contraction duration, might be linked to a different distribution of muscular fibers according to the sex. The practice of a sport might increase the fatigability of the masseter muscles and might be a factor inducing a muscular imbalance of the mandibular posture. But the relatively low number of subjects and the absence of well defined distinctions between different kinds of sport limit our conclusions.

Gout of the temporomandibular joint: pitfalls in diagnosis.

Gout is a frequent benign disease that rarely affects the temporomandibular joint (TMJ) alone. When it does, the disease is usually confined to the joint space and leads to pain and limitation of jaw opening (acute gout). The case described in this report is atypical in so far as it extended beyond the joint capsule into the pterygoid muscle and destroyed the head of the mandible, the temporal bone and the greater wing of the sphenoid bone. This clinical behaviour in combination with the radiographic appearance created the appearance of a benign but osteolytic lesion. The clinical, radiographic and biological features of gout in the TMJ are reviewed and treatment options are discussed.

[Treatment of fractures of the orbital floor: reconstruction using Teflon sheets. Analysis of the results after 5 years]. / Le traitement des fractures du plancher de l'orbite: réfection par lame de Téflon. Analyse des résultats sur 5 ans.

We report our experience with fractures of the orbital floor over the past five years, reporting 80 cases. Different anatomic and clinical forms were defined, particularly trap-door fractures. After a clinical and computed tomography study, we recommend treatment with Teflon implants. Results obtained were analyzed and compared with other series in the literature.