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Necrotizing myositis is an extremely rare soft tissue infection, mainly caused by Group A Streptococci. Although its presentation is nonspecific and seems harmless, it quickly leads to death in almost all cases. Therefore, diagnosis and treatment of necrotizing myositis are considered as medical emergencies. The 27 years old patient we report benefited from early diagnosis and care. Necrotic tissues were surgically removed 24 hours after the appearance of the first clinical signs. Intravenous antibiotherapy as well as immunoglobulin therapy were also given on the first day. Starting from this clinical case, we present a brief explanation of the pathogenesis, the key clinical features and appropriate tools for diagnosis. Then, adequate antibiotherapy, role of immunoglobulin therapy and interest of hyperbaric oxygenotherapy will be discussed.

Background Solid pseudopapillary neoplasms of the pancreas (SPPN) can relapse very late, but little is known about risk factors for recurrence and optimal treatment. We aimed to identify risk factors for recurrence and to analyze treatment modalities in all French pediatric cases of SPPN over the past 20 years. Material and methods Data were collected from pediatric oncologists and surgeons, and also from adult pancreatic surgeons in order to identify late recurrences. Results Fifty-one patients (41 girls) were identified. Median age at diagnosis was 13.1 years [8.7–17.9]. Abdominal pain was the commonest presenting symptom (32/49, 65%). The tumor was located in the pancreatic head in 24 patients (47%). Preoperative biopsy or cytology was performed in 14 cases (28%). All patients were operated with a median of 23 days [0–163] after diagnosis. The rate of postoperative morbidity was 29%. With a median follow-up of 65 months [0.3–221], the overall and event-free survival was 100% and 71%, respectively. Seven patients (13.7%) relapsed with a median of 43 months [33–94] after initial surgery. Six were treated surgically, either alone (n = 3) or with perioperative chemotherapy (n = 2) or hyperthermic intraperitoneal chemotherapy (n = 1). One patient in whom further treatment was not feasible was still alive at last news. Risk factors for recurrence were positive surgical margins (P = 0.03) and age less than 13.5 years at diagnosis (P = 0.03). Conclusions SPPN recurrence in this pediatric series was a rare and late event that did not undermine overall survival. Complete surgical removal of recurrent tumors appears to be the best option.

International audience; BACKGROUND: Alimentary tract duplications (ATD) are a rare cause of intestinal obstruction in childhood. There are many case reports but few series about laparoscopy or thoracoscopy for ATD. The aim of our study was to report the outcome of minimally invasive surgery (MIS) for ATD.METHODS: This was a retrospective multicenter study from the GECI (Groupe d'Etude en Coeliochirurgie Infantile). We reviewed the charts of 114 patients operated on by MIS for ATD from 1994 to 2009.RESULTS: Sixty-two patients (54 %) had a prenatal diagnosis. Forty-nine patients (43 %) were symptomatic before surgery: 33 of those patients (63 %) with postnatal diagnosis compared to 16 (25 %) with prenatal diagnosis (P < 0.01). In this last group, the median age at onset of symptoms was 16 days (range = 0-972). One hundred and two patients had laparoscopy (esophageal to rectal duplications) and 12 patients had thoracoscopy for esophageal duplications. The mean operative time was 90 min (range = 82-98). There were 32 (28 %) resection anastomoses, 55 (48 %) enucleations, and 27 (24 %) unroofings. The conversion rate was 32 %, and in a multivariate analysis, it was significantly higher, up to 41 % for patients weighing CONCLUSION: This study showed that MIS for ATD is feasible with a low rate of complications. Patients with prenatal diagnosis should have prompt surgery to prevent symptoms, despite a high rate of conversion in small infants.

Objective This study evaluated the Trap-door button use (Cook Medical, Bloomington, IL) for antegrade enemas in children. Methods Since 2002, patients with fecal incontinence or encopresis and constipation underwent percutaneous cecostomy under laparoscopy using a button. Technical details are described. Age at surgery, operative time, hospital stay, diagnosis, indications for cecostomy, and duration of follow-up were recorded. A survey was proposed via a questionnaire that was sent to the patients. Patients wearing the button for less than 1 month were excluded from this evaluation. The survey concerned volume and frequency of enemas, difficulties encountered, benefits and disadvantages of this method, and assessment of the antegrade enemas in continence. Results Twenty-nine patients, 18 males and 11 females, aged 3 to 21 years (mean, 8.5 years) underwent laparoscopic Trap-door button placement. The indications for all the patients were intractable fecal incontinence in 24 cases and constipation with encopresis in 5 cases. Incontinence was because of myelomeningocele (n = 10), anorectal malformations (n = 11), caudal regression syndrome (n = 1), 22q11 syndrome (n= 1), and Hirschsprung disease with encephalopathy with convulsions (n = 1). Constipation with encopresis was because of sacrococcygeal teratoma (n = 1), cerebral palsy (n = 1), and acquired megarectum with psychiatric and social disorders (n = 3). A total of 26 cecostomy button placements and 3 sigmoidostomy button placements were successful with no intraoperative complication. The mean operative time was 25 minutes (10-40 minutes), and the hospital stay was 2.5 days (1-4 days). Twenty-two parents or patients answered the questionnaire. At the time of this survey, 2 patients had improved their fecal continence and had had the button removed. A mean of 4 weekly enemas was enough to improve fecal continence troubles (range, 1 daily to 1 for 2 weeks). The volume for enemas was 250 to 1000 mL (mean, 700 mL). The time required for the irrigation of the bowel by gravity took from 5 to 60 minutes (mean, 25 minutes) for 20 patients. Before surgery, 14 patients needed a diaper, day and night, and 6 needed sanitary protection. Soiling was a very significant inconvenience for all the patients. After surgery, only 5 patients needed a diaper (cerebral palsy, 22q11, cloacal malformation, myelomeningocele, bladder exstrophy) because of moderate results or urinary incontinence and continued soiling. Patients were asked to give an assessment (null = 0, bad=1, fair=2, good=3, very good=4). None of the patients felt there had been no changes or a bad result. There were 5 patients who felt they had an average result, 5 a good result, and 12 a very good result. The mean grade was 3.44 (17.2/20). A total of 3 patients had hypertrophic granulation tissue formation around the cecostomy button, and 12 had tiny leakage. Conclusion Percutaneous placement of a cecostomy button under laparoscopic control is an easy and major complication-free procedure. The use of the Trap-door device by the patients or with the help of the parents for antegrade enemas is effective and satisfactory. It improves the quality of life and is reversible.

Solid pseudopapillary neoplasms of the pancreas (SPPN) can relapse very late, but little is known about risk factors for recurrence and optimal treatment. We aimed to identify risk factors for recurrence and to analyze treatment modalities in all French pediatric cases of SPPN over the past 20 years.Data were collected from pediatric oncologists and surgeons, and also from adult pancreatic surgeons in order to identify late recurrences.Fifty-one patients (41 girls) were identified. Median age at diagnosis was 13.1 years [8.7-17.9]. Abdominal pain was the commonest presenting symptom (32/49, 65%). The tumor was located in the pancreatic head in 24 patients (47%). Preoperative biopsy or cytology was performed in 14 cases (28%). All patients were operated with a median of 23 days [0-163] after diagnosis. The rate of postoperative morbidity was 29%. With a median follow-up of 65 months [0.3-221], the overall and event-free survival was 100% and 71%, respectively. Seven patients (13.7%) relapsed with a median of 43 months [33-94] after initial surgery. Six were treated surgically, either alone (n = 3) or with perioperative chemotherapy (n = 2) or hyperthermic intraperitoneal chemotherapy (n = 1). One patient in whom further treatment was not feasible was still alive at last news. Risk factors for recurrence were positive surgical margins (P = 0.03) and age less than 13.5 years at diagnosis (P = 0.03).SPPN recurrence in this pediatric series was a rare and late event that did not undermine overall survival. Complete surgical removal of recurrent tumors appears to be the best option.

Recently developed quantitative and high-throughput technologies that allow automated and rapid screening of the whole genome, transcriptome and proteome have revolutionized the field of cancer genetics. At the same time, new challenges are met, e.g. the need for improved data analysis and standardization of tumor sample handling. Even if these issues are resolved, an 'old' problem in genetic tumor analysis remains, i.e. contamination of tumor samples by stromal and surrounding normal cells. To overcome this obstacle, laser capture microdissection (LCM) has been developed in order to procure the cells of interest from stained tissue sections with retention of morphology. In this review we describe the possible down-stream applications of LCM in the genetic analysis of neuroblastoma (NB). Special focus is given to MYCN copy number determination using real-time quantitative polymerase chain reaction (Q-PCR), analysis of 1p-, 3p- and 11q-deletions using loss of heterozygosity analysis and Q-PCR expression analysis of microdissected normal neuroblast cells and NB cells.

A 10-year-old girl with a family history of Hodgkin's lymphoma presented with a 2 month history of cervical lymphadenopathy and weight loss. Biopsy indicated concomitant nodal involvement by Langerhans cell histiocytosis and Hodgkin's lymphoma. Such an association is rare, especially so in children, but is not an isolated phenomenon, thereby prompting the question of whether Langerhans cell histiocytosis is a reactive or a neoplastic process.

Congenital absence of the left pericardium (allowing communication between pericardial and pleural cavities) is a rare developmental defect that results from faulty partitioning of the pleuropericardic cavity during the 5th week of development. It occurs sporadically in most instances, and may be associated with other malformations of the thoracic viscera. We report here two sibs born to consanguineous parents with absent left fibrous pericardium and developmental defects of the septum transversum: left posterolateral diaphragmatic hernia in one child, left diaphragmatic eventration in the other sib. This appears to be the first familial report of this rare association.

The laparoscopic pull-through for high imperforate anus in boys has recently been developed in France and Benelux. The purpose of this study was to evaluate technical difficulties and surgical complications of this approach.This report was based on a retrospective study from January 2002 to December 2007,including 34 patients from 10 centers. The rectourinary fistula found in 33/34 cases was bulbar in 10, prostaticin 20, and cervical in 3.Patients were 3.7 months old (range, 1.6-10.4) at surgery. Fistula was cut without closure in 11 cases or divided and closed on the genitourinary side with a suture in 15, with clips in five and linear stapler in two.Median operative duration was 152 mm (range, 80-410). The main per-operative complication, occurring in five cases, was leakage of stools from the rectum after the fistula section, requiring in one case a second laparoscopic procedure for washing and for all five cases intravenous antibiotics. Median hospital stay was 6 days(range, 3-15). During the follow-up, patients experienced eight anal stenoses and three prolapses, requiring in three cases perineal redo surgery.The laparoscopic approach of high imperforate anus allowed an easy control of the fistula and helped to determine the accurate passage through the levator ani. Nevertheless, technical points have to be improved,such as coprostasis before fistula section to avoid stool leakage, rectum tacking to prevent prolapse,and calibration of muscle channel to avoid stenosis.

Pseudo-papillary tumors of the pancreas are rare and usually occur in young women. We report a case with a very rare presentation (rupture of esogastric varices complicating biliary cirrhosis secondary to bile duct compression by a pancreatic tumor). After biological and radiological explorations, a duodenopancreatectomy was performed. Diagnosis was confirmed by conventional histology and immunohistochemistry. One year later, the patient remained asymptomatic.

Pseudo-papillary tumors of the pancreas are rare and usually occur in young women. We report a case with a very rare presentation (rupture of esogastric varices complicating biliary cirrhosis secondary to bile duct compression by a pancreatic tumor). After biological and radiological explorations, a duodenopancreatectomy was performed. Diagnosis was confirmed by conventional histology and immunohistochemistry. One year later, the patient remained asymptomatic.

Objectif Preciser les indications de la vaginoplastie sigmoidienne par cœlioscopie (VSC) et les details techniques de cette chirurgie. Materiel et Methode Une enquete par questionnaire informatise proposee au sein de la SFCP devait preciser la place de la vaginoplastie sigmoidienne dans le traitement d’une atresie vaginale et permettre le recueil de donnees operatoires des patientes operees par VSC. Resultats principaux Neuf centres ont repondu au questionnaire. Six centres utilisent la VSC dont un privilegie la technique de Davydov cœlioscopique. Trois realisent une VS par laparotomie, deux privilegient la methode de Franck. Onze VSC (moyenne d’âge 14 ans, de 5 a 28) ont ete evaluees. L’utilisation du futur introit pour la pince a suture automatique sectionnant le transplant colique, pour mettre en place l’enclume de la pince circulaire du retablissement de la continuite sont frequentes (4 centres) et font reference aux techniques N.O.T.E.S. Une conversion. Les ecoulements de mucus sont consideres peu ou pas genants (3 patientes), et la vie sexuelle satisfaisante (cotee a 4/5). Conclusion La VSC est peu decrite dans la litterature. Elle permet une approche non invasive efficace, avec un bon resultat rapidement obtenu pour une activite sexuelle satisfaisante.

Objectifs Determiner les resultats a court et moyen terme des duplications digestives de tout site operees par laparoscopie ou thoracoscopie chez l’enfant. Materiel et Methodes Etude retrospective multicentrique: 114 enfants provenant de 18 centres. P Resultats La localisation etait a l’œsophage(14 %), a l’estomac(12 %), au duodenum(6 %), a l’intestin grele(62 %), et au colon(6 %). Il y a eu 102 laparoscopies et 12 thoracoscopies. Le taux de conversion etait de 42 % chez les patients avec diagnostic antenatal(DAN) contre 20 % sans DAN (S). Il y a eu 29 % de patients symptomatiques avec DAN contre 63 % chez les patients sans DAN (S). Le taux de conversion etait de 48 % en cas d’intervention urgente contre 26 % si non urgente (S). L’abord a ete combine (exteriorisation de la duplication par orifice de trocart) pour 26 % des patients. Les complications post operatoires(7 %) ne dependaient pas de d’une conversion, de l’âge de l’enfant ou de l’urgence. Conclusion Le traitement des duplications digestives par thoraco/ laparoscopie comporte peu de complications. Le DAN permet de prevenir l’apparition des symptomes mais la thoraco/laparoscopie est plus difficile chez ces enfants. L’abord combine est une alternative a l’abord intracorporel pour les duplications du grele.