Primary clear cell sarcoma of the tongue and surgical reconstruction: About a rare case report.

Clear cell sarcomas (SCC), also called "soft-tissue melanoma", are rare and aggressive tumors that preferentially affect the lower limbs (tendons and fasciae) and which have also been described in head and neck localizations. Their clinical and immunohistochemical mimicry with melanoma makes it difficult to diagnose sarcomas. SCC treatment is mainly focused on large-scale resection surgery with adjuvant radiotherapy because of their low chemo-sensitivity and extreme lymphophilia. In case of head and neck localization, these treatments may lead to function and aesthetic sequelae thus requiring the use of modern techniques of reconstructive surgery. The authors describe the diagnosis, treatment and follow-up of large lingual SCC case using a DIEP free flap reconstruction according to an original technique developed in the department. Given the characteristics of patients with SCC (a high proportion of women between 20 and 40 years old) and its inherent qualities (low morbidity of the donor site, volume delivered and excellent plasticity), the fascio-cutaneous free flap type "DIEP" "taken according to the design of the" Cathedral triptych seems to be a viable choice among the range of reconstruction solutions.

Secondary bladder exstrophy repair with a bilateral gracilis muscle flap in an adult female patient: Case report of an original procedure.

Classical bladder exstrophy (CBE), affecting 1 birth out of 30,000, is characterized by an evaginated bladder plate through a defect in the lower abdominal wall, multiple abdominal wall anomalies including a pubic bone arch dehiscence. Numerous approaches from childhood to adulthood are thus required, depending on the severity of the deformity, including the associated genital anomalies. We report the case of a 19-year-old woman with CBE with a history of three-failed primary closure. We performed a secondary neck closure with a concomitant suspension of the bladder neck and reconstruction of the lower abdominal wall using a bilateral gracilis muscle flap transposition. The early postoperative course was uneventful. The patient was discharged at day ten postoperatively. The upper part of the genital sutures (labia minora) secondary healed in three weeks. Assessment at 2, 6 and 16 months postoperatively, respectively noticed a complete healing with successful sexual intercourses, perceived gracilis contraction by the patient, and finally, recent attempts to get pregnant. Neither urinary infection nor urinary leaks occurred. Bilateral crossed gracilis muscles transfer linking both rectus abdominis muscle in front of the reconstructed bladder neck might benefit to bladder exstrophy patients.

[Mastectomy and immediate reconstruction: Indications, techniques and decision algorithm]. / Mastectomie avec reconstruction mammaire immédiate : indications et techniques, algorithme de décision d'une reconstruction immédiate.

Immediate breast reconstruction indications extend to infiltrating carcinomas, due to new matrix implant coverage techniques and the development of perforator flaps. These techniques allow adjuvant treatments. However, the decision of immediate reconstruction must be discussed with the oncological multidisciplinary team and the benefits/risks must also be evaluated in relation to the morphology of the patients and their co-morbidities. The chosen type of mastectomy: conventional or skin sparing and/or nipple sparing depends on the shape and volume of the breast, the localization of the tumor in the breast and the distance from the nipple areola complex (NAC). We describe an algorithm to allow, in the case of therapeutic mastectomy with or without adjuvant radiotherapy, an immediate reconstruction with implants or free or pedicled flaps.

[Decision algorithm in immediate breast reconstruction]. / Algorithmes décisionnels en reconstruction mammaire immédiate.

Immediate breast reconstruction showed many advantages in terms of aesthetic and functional results and improvement of quality of life when compared to delayed breast reconstruction. Previous radiotherapy or the use of adjuvant treatments such as radiation therapy, or chemotherapy are no longer a contraindication for immediate breast reconstruction. However, it is important to respect certain rules in order to decrease the risk of complications: the choice of reconstruction technique, the management of the skin envelope according to the breast shape you want to create, the time delay between the first and the second stage of reconstruction depending on a possible adjuvant treatment.

The ongoing emergence of robotics in plastic and reconstructive surgery.

Robot-assisted surgery is more and more widely used in urology, general surgery and gynecological surgery. The interest of robotics in plastic and reconstructive surgery, a discipline that operates primarily on surfaces, has yet to be conclusively proved. However, the initial applications of robotic surgery in plastic and reconstructive surgery have been emerging in a number of fields including transoral reconstruction of posterior oropharyngeal defects, nipple-sparing mastectomy with immediate breast reconstruction, microsurgery, muscle harvesting for pelvic reconstruction and coverage of the scalp or the extremities.

[The sural medial perforator flap: Anatomical bases, surgical technique and indications in head and neck reconstruction]. / Le lambeau médial sural perforant : bases anatomiques, technique chirurgicale et indications en reconstruction cervicofaciale.

INTRODUCTION: The medial sural artery perforator (MSAP) flap is defined as a thin cutaneo-adipose perforator flap harvested on the medial aspect of the leg. The aims of this study were to describe the anatomical basis as well as the surgical technique and discuss the indications in head and neck reconstructive surgery. MATERIAL AND METHODS: We harvested 10 MSAP flap on 5 fresh cadavers. For each case, the number and the location of the perforators were recorded. For each flap, the length of pedicle, the diameter of source vessels and the thickness of the flap were studied. Finally, we performed a clinical application of a MSAP flap. RESULTS: A total of 23 perforators with a diameter superior than 1mm were dissected on 10 legs. The medial sural artery provided between 1 and 4 musculocutaneous perforators. Perforators were located in average at 10.3cm±2cm from the popliteal fossa and at 3.6cm±1cm from the median line of the calf. The mean pedicle length was 12.1cm±2.5cm. At its origin, the source artery diameter was 1.8mm±0.25mm and source veins diameters were 2.45mm±0.9mm in average. There was no complication in our clinical application. DISCUSSION: This study confirms the reliability of previous anatomical descriptions of the medial sural artery perforator flap. This flap was reported as thin and particularly adapted for oral cavity reconstruction and for facial or limb resurfacing. Sequelae might be reduced as compared to those of the radial forearm flap with comparable results.

Development of a universal double-digest RAD sequencing approach for a group of nonmodel, ecologically and economically important insect and fish taxa.

The generation of genome-scale data is critical for a wide range of questions in basic biology using model organisms, but also in questions of applied biology in nonmodel organisms (agriculture, natural resources, conservation and public health biology). Using a genome-scale approach on a diverse group of nonmodel organisms and with the goal of lowering costs of the method, we modified a multiplexed, high-throughput genomic scan technique utilizing two restriction enzymes. We analysed several pairs of restriction enzymes and completed double-digestion RAD sequencing libraries for nine different species and five genera of insects and fish. We found one particular enzyme pair produced consistently higher number of sequence-able fragments across all nine species. Building libraries off this enzyme pair, we found a range of usable SNPs between 4000 and 37 000 SNPS per species and we found a greater number of usable SNPs using reference genomes than de novo pipelines in STACKS. We also found fewer reads in the Read 2 fragments from the paired-end Illumina Hiseq run. Overall, the results of this study provide empirical evidence of the utility of this method for producing consistent data for diverse nonmodel species and suggest specific considerations for sequencing analysis strategies.

Paenibacillus larvae Phage Tripp Genome Has 378-Base-Pair Terminal Repeats.

Paenibacillus larvae bacteriophage Tripp was isolated from an American foulbrood diseased honey bee hive in North Carolina, USA. The 54,439-bp genome is 48.3% G+C, encodes 92 proteins, no tRNAs, and has 378-bp direct terminal repeats. It is currently unique in Genbank.

The feasibility of home polysomnographic recordings prescribed for sleep-related neurological disorders: a prospective observational study.

OBJECTIVE: Home polysomnography is being increasingly developed for sleep studies, with various grades of quality. This study aimed to determine the feasibility of affordable, high quality home polysomnographic recordings prescribed for suspected sleep-related neurological disorders. PATIENTS AND METHODS: We prospectively screened all patients referred to the specialist sleep disorders clinic in Nancy University Hospital between May 2011 and August 2011. Patients were eligible for inclusion if they required polysomnography for the diagnosis of a sleep-related neurological disorder. One-night, polysomnography was performed in each patient's home by a trained sleep technician. Financial cost was determined prior to inclusion. A recording was considered as satisfactory if all the following criteria were present: at least, one EEG channel with continuous signal allowing determination of sleep stages and wake during more than 66% of sleep time; at least, one usable respiratory channel (airflow or either band) during more than 66% of sleep time; and usable oximetry during more than 66% of sleep time. RESULTS: Forty-eight of the 139 screened patients were included. Among the 48 home polysomnography recordings, 35 (72.9%) were satisfactory. Thirteen (27.1%) tracings displayed an unsatisfactory loss of EEG data, including seven (14.6%) tracings with an unsatisfactory loss of respiratory data. CONCLUSION: Home polysomnography prescribed for suspected sleep-related neurological disorders is feasible, with affordable costs, whilst maintaining high quality recording. Further studies are needed to measure the real medico-economic impact of promoting outpatient domiciliary explorations for sleep-related neurological disorders.

Interferometry with non-classical motional states of a Bose-Einstein condensate.

The Ramsey interferometer is a prime example of precise control at the quantum level. It is usually implemented using internal states of atoms, molecules or ions, for which powerful manipulation procedures are now available. Whether it is possible to control external degrees of freedom of more complex, interacting many-body systems at this level remained an open question. Here we demonstrate a two-pulse Ramsey-type interferometer for non-classical motional states of a Bose-Einstein condensate in an anharmonic trap. The control sequences used to manipulate the condensate wavefunction are obtained from optimal control theory and are directly optimized to maximize the interferometric contrast. They permit a fast manipulation of the atomic ensemble compared to the intrinsic decay processes and many-body dephasing effects. This allows us to reach an interferometric contrast of 92% in the experimental implementation.

Integrated Mach-Zehnder interferometer for Bose-Einstein condensates.

Particle-wave duality enables the construction of interferometers for matter waves, which complement optical interferometers in precision measurement devices. This requires the development of atom-optics analogues to beam splitters, phase shifters and recombiners. Integrating these elements into a single device has been a long-standing goal. Here we demonstrate a full Mach-Zehnder sequence with trapped Bose-Einstein condensates confined on an atom chip. Particle interactions in our Bose-Einstein condensate matter waves lead to a nonlinearity, absent in photon optics. We exploit it to generate a non-classical state having reduced number fluctuations inside the interferometer. Making use of spatially separated wave packets, a controlled phase shift is applied and read out by a non-adiabatic matter-wave recombiner. We demonstrate coherence times a factor of three beyond what is expected for coherent states, highlighting the potential of entanglement as a resource for metrology. Our results pave the way for integrated quantum-enhanced matter-wave sensors.

[Hypothalamic involvement in multiple sclerosis]. / Atteintes hypothalamiques dans la sclérose en plaques.

Hypothalamic involvement is a rare condition in patients with multiple sclerosis (MS). We report two patients with a long history of MS who presented with severe acute hypothermia with associated thrombocytopenia and elevated transaminase levels. Several cases of hypothermia or hyperthermia in patients with MS have been reported in the literature. They could be linked with hypothalamic lesions, in particular in the pre-optic area. However, other anatomical locations seem to be involved in thermoregulation and can be affected by MS. Besides, some cases of syndrome of inappropriate antidiuretic hormone secretion have been reported in patients with MS. Finally, some sleep disorders, particularly hypersomnia or narcolepsy, could be related to hypothalamic lesions, through the fall in hypocretin-1 in the cerebrospinal fluid. Hypocretin-1 is a neuropeptide that is secreted by some hypothalamic cells. It plays a role in the sleep-awake rhythm. We report one patient with narcolepsy and cataplexy before the first symptoms of MS appeared. Hypothalamic signs are rare in MS. However, several series of autopsies have shown a high frequency of demyelinating lesions in the hypothalamic area. Among these lesions, the proportion of active lesions seems elevated. Yet only few of them have a clinical or biological translation such as thermoregulation dysfunction, sleep disorders or natremia abnormalities. Thus, it seems unlikely that inflammatory hypothalamic lesions alone, even when bilateral, could be the explanation of these signs. A sufficient number of inflammatory demyelinating lesions, which we can observe in patients with a long history of MS and an already severe disability, is probably necessary to develop such a rare symptomatology. Hypothalamic signs might be a factor of poor prognosis for the disease course and progression of the disability.

[Vaginal hysterectomy and bilateral adnexectomy for female to male transsexuals in an interdisciplinary concept]. / Vaginale Hysterektomie und beidseitige Adnexektomie in interdisziplinärem Konzept bei Frau zu Mann - Transsexualismus.

BACKGROUND: FtM reassignment surgeries are rare and require interdisciplinary cooperation of plastic surgeons and gynaecologists. There are only few homogeneous data and standardised recommendations about the operative access to hysterectomy and bilateral adnexectomy of FtM transsexual patients. PATIENTS AND METHODS: Between 2006 and 2009 106 FtM transsexuals were hysterectomised in an interdisciplinary concept of plastic surgeons and gynaecologists in the Frauenklinik des Rotkreuzklinikums München. Firstly plastic surgeons performed a complete colpectomy, after that a vaginal hysterectomy with bilateral adnexectomy was carried out by gynaecologists. Simultaneously plastic surgeons performed a bilateral subcutaneous adenomammectomy on the FtM patients, and the removed vaginal tissue was prepared for preforming a new urethra. In the next step the vagina was closed by plastic surgeons and the urethra preformed. RESULTS: In 103 of 106 cases (97.2%) hysterectomy and bilateral adnexectomy were performed through the vagina. The complication rate was 5.4%. The vaginal hysterectomy and the bilateral adnexectomy lasted 52 min on average. DISCUSSION: Vaginal, abdominal or laparoscopic approaches provide possible operative access for hysterectomy and bilateral adnexectomy in FtM transsexuals. A data comparison shows that the rate of complications with our FtM transsexuals operated through the vagina was not higher than that with non-transsexual patients operated through the vagina for benign illnesses of the uterus. There are advantages of the vaginal hysterectomy for patients concerning a reduced occurrence of scars and avoiding injuries of the rectus muscle as an important precondition for phalloplasty. The bilaterally performed subcutaneous adenomammectomy and the preparation of the removed vaginal tissue for the preformation of the urethra can be carried out simultaneously, meaning that the time for operation and the stay in hospital will be shortened and costs will be reduced as well. The problem of a relatively narrow field for the operation will be minimised or even solved by the preceding colpectomy. CONCLUSION: Realising the vaginal hysterectomy with bilateral vaginal adnexectomy after performing a total colpectomy from our point of view is the optimal choice concerning operative methods for reassignment surgeries.

[Operative management and techniques of mastectomy in female-to-male transsexuals]. / Operatives Management und Techniken der Mastektomie bei Frau-zu-Mann Transsexuellen.

BACKGROUND: Subcutaneous mastectomy in female to male transsexuals is one first important step in gender adjustment. Beside proper surgical technique, operative management of mastectomy procedures is important to allow for an optimised process of gender transformation including all necessary individual operations. METHODS: 126 female-to-male transsexuals were prospectively included in the observation period, all undergoing a step 1 operation of gender adjustment. The mastectomy procedure is conventionally embedded in an overall approach for gender transformation and was regularly combined with procedures like colpectomy, extension of the urethra as well as hysterectomy and ovariectomy. 4 different methods for mastectomy were introduced at our institution, depending on and adjusted to the breast volume, grade of breast ptosis, the breast size and envelope and skin elasticity. For patient evaluation a standardised survey was conducted using a semiquantitative score (1-4) for grading aesthetic results and nipple sensibility. RESULTS: Between 2006 and 2009 a total of 126 patients underwent a step 1 procedure of gender adjustment, 63 had mastectomy and 29 a revision procedure after external prior mastectomy. In 64/126 (50.8%) mastectomy was performed using a one-third edge cut, 30/126 (23.8%) were operated by a submammarian approach and pedicled nipples technique, in another 22/126 cases (17.5%) mastectomy was performed using circular tightening and 10/126 (7.9%) received freely transplanted nipples. For improving the aesthetic results, additional procedures were performed on 29 patients (46%). The patient survey revealed a high level of satisfaction with the aesthetic result, 42/50 (84%) of the patients rated results as good or very good. CONCLUSIONS: We newly introduced an operative concept of mastectomy being embedded and adjusted in an overall approach for gender adjustment (Munich Modular Operative Kit; MMOK). Using the presented individualised surgical techniques of mastectomy, all procedures were conducted in a very scar-saving manner, however, a higher rate of correction procedures was necessary to individually adjust skin shrinking and thereby optimising the prior aesthetic results. The new Munich Modular Operative Kit allows for gender reassignment including breast corrections usually using 4 operative steps.

[Perioperative anaesthetic management of an epileptic patient treated with a vagus nerve stimulation]. / Gestion périopératoire d'un patient épileptique porteur d'une neurostimulation du nerf vague.

The vagal nerve stimulation is approved for medically refractory epilepsy and major depression. We report the perioperative management of an epileptic patient with this indwelling device. This observation summarizes the physiologic implications and the specific anaesthetic considerations for procedures with this pre-existing device.

A method for release and multiple strand amplification of small quantities of DNA from endospores of the fastidious bacterium Pasteuria penetrans.

AIMS: To establish a reliable protocol to extract DNA from Pasteuria penetrans endospores for use as template in multiple strand amplification, thus providing sufficient material for genetic analyses. To develop a highly sensitive PCR-based diagnostic tool for P. penetrans. METHODS AND RESULTS: An optimized method to decontaminate endospores, release and purify DNA enabled multiple strand amplification. DNA purity was assessed by cloning and sequencing gyrB and 16S rRNA gene fragments obtained from PCR using generic primers. Samples indicated to be 100%P. penetrans by the gyrB assay were estimated at 46% using the 16S rRNA gene. No bias was detected on cloning and sequencing 12 housekeeping and sporulation gene fragments from amplified DNA. The detection limit by PCR with Pasteuria-specific 16S rRNA gene primers following multiple strand amplification of DNA extracted using the method was a single endospore. CONCLUSIONS: Generation of large quantities DNA will facilitate genomic sequencing of P. penetrans. Apparent differences in sample purity are explained by variations in 16S rRNA gene copy number in Eubacteria leading to exaggerated estimations of sample contamination. Detection of single endospores will facilitate investigations of P. penetrans molecular ecology. SIGNIFICANCE AND IMPACT OF THE STUDY: These methods will advance studies on P. penetrans and facilitate research on other obligate and fastidious micro-organisms where it is currently impractical to obtain DNA in sufficient quantity and quality.

Integrating BioPAX pathway knowledge with SBML models.

Online databases store thousands of molecular interactions and pathways, and numerous modelling software tools provide users with an interface to create and simulate mathematical models of such interactions. However, the two most widespread used standards for storing pathway data (biological pathway exchange; BioPAX) and for exchanging mathematical models of pathways (systems biology markup language; SBML) are structurally and semantically different. Conversion between formats (making data present in one format available in another format) based on simple one-to-one mappings may lead to loss or distortion of data, is difficult to automate, and often impractical and/or erroneous. This seriously limits the integration of knowledge data and models. In this paper we introduce an approach for such integration based on a bridging format that we named systems biology pathway exchange (SBPAX) alluding to SBML and BioPAX. It facilitates conversion between data in different formats by a combination of one-to-one mappings to and from SBPAX and operations within the SBPAX data. The concept of SBPAX is to provide a flexible description expanding around essential pathway data - basically the common subset of all formats describing processes, the substances participating in these processes and their locations. SBPAX can act as a repository for molecular interaction data from a variety of sources in different formats, and the information about their relative relationships, thus providing a platform for converting between formats and documenting assumptions used during conversion, gluing (identifying related elements across different formats) and merging (creating a coherent set of data from multiple sources) data.

Virtual Cell modelling and simulation software environment.

The Virtual Cell (VCell; http://vcell.org/) is a problem solving environment, built on a central database, for analysis, modelling and simulation of cell biological processes. VCell integrates a growing range of molecular mechanisms, including reaction kinetics, diffusion, flow, membrane transport, lateral membrane diffusion and electrophysiology, and can associate these with geometries derived from experimental microscope images. It has been developed and deployed as a web-based, distributed, client-server system, with more than a thousand world-wide users. VCell provides a separation of layers (core technologies and abstractions) representing biological models, physical mechanisms, geometry, mathematical models and numerical methods. This separation clarifies the impact of modelling decisions, assumptions and approximations. The result is a physically consistent, mathematically rigorous, spatial modelling and simulation framework. Users create biological models and VCell will automatically (i) generate the appropriate mathematical encoding for running a simulation and (ii) generate and compile the appropriate computer code. Both deterministic and stochastic algorithms are supported for describing and running non-spatial simulations; a full partial differential equation solver using the finite volume numerical algorithm is available for reaction-diffusion-advection simulations in complex cell geometries including 3D geometries derived from microscope images. Using the VCell database, models and model components can be reused and updated, as well as privately shared among collaborating groups, or published. Exchange of models with other tools is possible via import/export of SBML, CellML and MatLab formats. Furthermore, curation of models is facilitated by external database binding mechanisms for unique identification of components and by standardised annotations compliant with the MIRIAM standard. VCell is now open source, with its native model encoding language (VCML) being a public specification, which stands as the basis for a new generation of more customised, experiment-centric modelling tools using a new plug-in based platform.

[Modern concept for treatment of the female-to-male transsexual]. / Modernes Behandlungskonzept bei Frau-zu-Mann-Transsexualismus.

BACKGROUND: The experience of our multidisciplinary team in surgical treatment of female-to-male trans-sexualism is presented, and our treatment concepts described in detail. In addition, our preferred technique of neourethra formation using a prefabricated free fibula flap is described. PATIENTS AND METHOD: From 1996 to 2003, thirty-four patients underwent gender reassignment surgery as a staged procedure. The neourethra was constructed using an anterior vaginal flap and the prefabricated free fibular flap. RESULTS: There were four complete losses of the prefabricated fibular flap (11.7%). The neourethra stricture rate was 20.5% and the fistula formation rate was 14.7%. In 82.3% of the patients, the ability to micturate while standing was achieved. CONCLUSION: This modern concept reduces the complication rate and improves the quality of patient outcome.

Phalloplasty with free (septocutaneous) fibular flap sine fibula.

PURPOSE: The free prefabricated fibular flap has been used as a good alternative to the free radial forearm flap in female-to-male sex reassignment surgery. We describe a new technique of harvesting the flap without sacrificing the fibula. The neophallus is thinner, more elegant, less rigid and able to contain a hydraulic penile prosthesis. MATERIALS AND METHODS: Five biologically female patients underwent reassignment surgery using this technique. Average patient age was 30 years (range 24 to 37) and average followup was 25 months. All patients underwent total hysterectomy, salpingo-oophorectomies, vaginectomy and urethral prelamination 6 months before flap surgery. The lateral calf cutaneous island was raised on the peroneal artery septocutaneous perforators without disrupting the continuity of the fibula. Urethro-urethral anastomosis was done 3 months later. RESULTS: All patients sustained good results with no flap loss. Voiding from a standing position was achieved and the neophallus was esthetically acceptable. Donor morbidity was minimal with no disturbance to ambulation compared to the conventional method, in which the fibula is sacrificed. CONCLUSIONS: The free fibular flap without the fibula is a challenging refinement of neophallus construction that confers benefits to recipient and donor sites. It is a welcomed addition to the armamentarium for neophalloplasty.