Summmary : Teratomas are the most common histologic type of germ cell tumors in pediatrics. There are two types of teratomas, mature, benign and immature, malignant. Initial diagnosis is essential for optimal management. This work, based on a clinical case, aims to review the clinical, radiological, biological and histological characteristics allowing them to be differentiated.

Complete androgen insensitivity syndrome is the most frequent cause of disorder of sexual development in 46 XY patients. It is caused by mutations of the AR gene coding for the androgen receptor. Transmission is X-linked and mutations are most of the time inherited. It leads to a complete lack of response to androgen resulting in the presence of female external genitalia in 46 XY patients, normal but undescended testes and lack of female internal genitalia due to the secretion of anti-Müllerian hormone by male gonads. Traditionally, gonadectomy was proposed before puberty to decrease the risk of gonadal malignancy. However, more recent studies underlined the benefits of postponing gonadectomy until after pubertal development. Benefits of deferred gonadectomy are spontaneous pubertal development through peripheral aromatization of testosterone into oestrogens and the chance for the patient to have an active role in the decision-making process. After gonadectomy, hormone replacement therapy is required in order to prevent complications due to hypogonadism such as osteoporosis, cardiovascular diseases and a reduction of life expectancy.

The incidence of Barrett's esophagus, complication of gastroesophageal reflux disease, is rising in western countries. It is the same for esophageal adenocarcinoma, of which it is the main contributing factor. This retrospective study seeks to report the incidence of these pathologies observed in a regional hospital center and to describe their management. In 5 years, 354 Barrett's esophagus are detected and 34 of them are complicated by high-grade dysplasia or adenocarcinoma. Endoscopic resection is performed in 24 of these patients. The histological analysis of which leads to the conclusion of adenocarcinoma in 20 patients and high-grade dysplasia in the 14 others. The complications of endoscopic and surgical resections are detailed. Their frequency and severity remain low, comparable to data in the literature.

Cholelithiasis is rare in children and even more so in infants. We report the case of a 3-month-old patient with cholestatic jaundice secondary to an obstruction of the terminal portion of the bile duct. The treatment applied in this patient was a cholecystectomy with trans-cystic cholangiography and common bile duct clearance. The evolution was excellent. The current literature on biliary lithiasis in children and infants is poor in large cohort studies. The various treatments proposed, if necessary, include biliary lavage by percutaneous puncture, endoscopic retrograde cholangiopancreatography with sphincterotomy and laparoscopic or open cholecystectomy with intraoperative cholangiography. None of these procedures has shown superiority over the others. Therefore, no treatment algorithm is currently defined. Patients are treated on a case-by-case basis according to their symptoms, previous history and the level of expertise of each centre for these rare, difficult and specific procedures.

Tracheomalacia (TM) is characterized by tracheal collapse due to an intrinsic anomaly resulting in a lack of rigidity of the cartilaginous rings and/or the posterior membrane during expiration, coughing or crying. It may also be secondary to external compression or acquired during endobronchial diseases. TM is commonly associated with other syndromes or airway abnormalities. Tracheomalacia can be localized or diffused and if the main bronchi are involved, the term of tracheobronchomalacia (TBM) is used. The most common symptoms include expiratory stridor, barking cough and recurrent respiratory tract infections. If tracheal weakness is severe, Acute Life Threating Events (ALTE) or Brief Resolved Unexplained Event (BRUE) can occur. While mild forms usually do not require any treatment, severe TBM may require medical and/or surgical management. Amongst several possible treatments, including tracheostomy, noninvasive ventilation and airway stenting, the pexy surgical approach (posterior, anterior tracheopexy or aortopexy) is currently the favoured option.

We report the case of a child suffering from a neonatal cervicomediastinal neuroblastoma encasing the left subclavian artery and the left vertebral artery. There is only a few pediatric tumors extending from the neck to the upper part of the thorax. Because of the complex vascular and neurological anatomy of this area, the surgical excision of these cervicothoracic neuroblastomas is a real challenge. It is why, when we decided to propose a surgical management, we used the Transmanubrial Osteomuscular-Sparing Approach (TOSA), of which technique and benefits will be explained in this article.

We report the cases of two female newborns who present, at the first day, a interlabial mass. In both cases, the antenatal scans were normal, and the clinical examination in the delivery room didn't reveal any other abnormality. Similar features may suggest the same diagnosis. However, in the first case, it is a paraurethral cyst which causes the bulging of vaginal introitus whereas, in the second infant, it is an imperforated hymen. Paraurethral cysts are uncommon and even more so among infants. The diagnosis is primarily clinical, and ultrasound confirms the diagnosis and verifies the absence of other associated anomalies. These cysts usually resolve spontaneously. Rarely, they can lead to urinary retention. The second diagnosis, imperforate hymen, is the most common congenital malformation of the female genital tract. It can lead to hydrocolpos with the upstream accumulation of vaginal secretion and sometimes induce a hydrometrocolpos, with vaginal and uterine dilatation. The resulting mass can cause obstruction of surrounding urinary or digestive structures. Surgical management is generally necessary.

Cancers are rare pathologies in children. Improvement in survival rates has been obtained thanks to new therapeutic strategies based on the identification of risk factors. Targeted therapies in paediatric oncology are new treatments providing hope that cure is achievable without long-term sequelae.

OBJECTIVE:The aim of our retrospective study is to analyse the results of endoscopic macro-clips used for closing intestinal breaches in the real life. Post-endoscopic, spontaneous perforations and surgical fistulas are included. Our results are compared with principal published series. METHODS:Between 2010 and 2015, 25 consecutive patients underwent an endoscopic suture with macro-clips to seal a breach resulting from endoscopic perforation, surgical fistula, invasive medical procedure or spontaneous perforation. RESULTS:The endoscopic efficacy was immediate for all patients whose perforation was of endoscopic aetiology. None of them required surgery. Secondary complementary endoscopic treatment was only used in 6/25 patients. All of them had non-endoscopic aetiology for their perforation. The survival at 1 month was 84 % and at 3 months of 76 %. CONCLUSION:Our results confirm the high efficiency of macro-clips in the treatment of endoscopic breaches in a regional hospital center. The use of macro-clips seems less convincing for the closure of some postoperative fistula. The suture with macro-clips associated with endoscopic and medical treatments allowed to avoid surgical redo in some cases.

We report the story of an 11-year-old girl admitted to the emergency room for diplopia and divergent squint. Promptly apparead a fluctuating ptosis, a nasal voice and swallowing disorders, evoking the diagnosis of autoimmune myasthenia. The latter has been confirmed with electromyogram. Treatment with corticoids, plasmapheresis and pyridostigmine allowed symptoms control. Thymectomy by left thoracoscopy, non-robot assisted, was performed 6 months after the appearance of the first clinical signs for the purpose of remission.

A vascular mass localized in the face and the neck was displayed by ultrasonography in a 38-week-old male fetus. At birth, the mass was bulky and purplish. The newborn breathed spontaneously but with severe desaturation. During laryngoscopy, we observed an obstruction of the larynx with a left-shift caused by the hemorrhagic mass. Blood analysis revealed anemia, severe thrombocytopenia, and coagulation disorders. The diagnosis of kaposiform hemangioendothelioma (KHE) complicated by a Kasabach–Merritt phenomenon (KMP) was put forward and treatment with propranolol, corticoids, and vincristine was initiated. Platelets were transfused daily for 8 days but did not resolve the thrombocytopenia. At day 8, we added sirolimus to the treatment and noted a rapid response with the normalization of the platelet count within 1 week and a significant regression of the mass. In this paper, we review the clinical and biological features of hemangioendothelioma associated with KMP and discuss its current and future treatment. Sirolimus seems to be very promising.

Abstract Introduction No data are available on surgical management and outcomes of oesophageal atresia (OA) in Belgium. In collaboration with the Belgian Association of Paediatric Surgery (BELAPS) a retrospective study was organized to analyze data from children born with OA, with a 1-year follow-up. Materials and Methods Out of 17 centers in Belgium, 10 participated in this study. A novel checklist to collect data from OA patients born between the 1st of January 2010 and the 1st of January 2015 was created. Gross type A, B, C and D OA were included. Results One hundred children were included. Gross type C OA was seen in 86%. Associated malformations were present in 73%, most frequently cardiac malformations (52%). There was consensus regarding preoperative investigations, anastomotic technique, and transanastomotic tube placement. There was no consensus regarding genetic counseling, placement of a chest tube, and start of enteral feeding. There were six deaths in the first year of life. The majority of patients needed rehospitalization during the first year of life (85.9%) and there was a high occurrence of anastomotic strictures (51.6%). Conclusions This is the first study about management and outcome of OA in Belgium. Although the 1-year survival rate is high (93.8%), these data illustrate that OA has an important morbidity. Multidisciplinary follow-up and prospective studies/registries with longer follow-up are needed.

The persistence of the embryonic vitelline duct can lead to omphalomesenteric duct anomalies. A wide variety of anomalies, depending on the remnant segment and its degree of involution, may occur as a result of the omphalomesenteric duct failing to obliterate completely. The most frequent omphalomesenteric duct anomaly is Meckel's diverticulum, which is present in approximately 2-3 % of the population. Despite the progress in medical imaging, conventional abdominal computed tomography and endoscopy have limitations for the diagnosis of Meckel's diverticulum. The laparoscopic exploratory remains the best choice for diagnosis and treatment.

The Persistent Müllerian Ducts Syndrome (PMDS) is a rare congenital syndrome. It is one of abnormalities of genito-sexual development that is found on the normally virilized boy (46XY). It is characterized by the development of both Wolf structures and Müller duct. The pathophysiology can be explained by an action deficit of the anti-müllerian hormone (AMH). Its clinical presentations vary depending on the localization of the testis and the associated symptoms. Its discovery is mostly fortuitous and generally made in per-operative surgery of cryptorchidism or inguinal hernia. Treatment should be surgical. It relies on two aspects : ensuring the testicular descent and performing the excision of the müllerian duct. The follow-up is identical to the cryptorchid testes and the fertility problems will be influenced by the surgical procedure as well as the timing of the treatment.

Vascular anomalies (VAs) result from the defective development of the embryonic vascular system and feature dysplastic malformed vessels, which are not always apparent at birth. They do not regress over the patient's lifetime; they usually have commensurate growth during childhood and may worsen over time if not treated. VAs may cause chronic painful swelling, bleeding, functional deficits or vital structure obstruction. These patients' quality of life is usually impaired because of the chronicity and recurrence of the disease. We report on six cases of complicated VAs, refractory to current treatments, treated with rapamycin, an mTor inhibitor recently used in VAs. (C) 2017 Elsevier Masson SAS. All rights reserved.

The prevalence of liver cirrhosis progresses due to an increased occurrence of hepatitis C viral infection and of Non Alcoholic SteatoHepatopathy (NASH) related to the metabolic syndrome. Cirrhosis is a known risk factor for the development of hernia. The peri-operative morbidity and mortality after abdominal wall surgery is higher in the cirrhotic patient because of postoperative organ failure. The optimal timing for surgery and the method of repair remain controversial, but growing evidence orientates towards elective rather than emergency treatment regardless of the Child-Pugh score.

Superficial digestive tumours resection by endoscopic mucosal resection and sub-mucosal dissection causes very few complications according to the results obtained in expert centers. This work reports the rate of complications observed in a regional hospital, also comparing the results of their therapeutic management. The first 100 patients treated by endoscopic mucosal resection and sub-mucosal dissection, conducted by conventional techniques, were studied. The usual complications (stenosis, bleeding, perforation) were identified and details of their treatment reported. The overall complication rate was 16 %. Symptomatic stenoses are observed in 4 % of patients. Secondary bleeding occurs in 5 % of cases. Endoscopic management of these complications is effective in all cases, when it is attempted. Perforations complicate 7 % of the procedures. Two patients were assigned to surgery, successfully, the other 5 patients were treated by endoscopic sutures without damage. The complication rates observed in our department are comparable with results reported by the reference western centers. Their endoscopic management is usually successful and rescue surgery is unfrequent.

Préciser les indications de la vaginoplastie sigmoïdienne par cœlioscopie (VSC) et les détails techniques de cette chirurgie. Une enquête par questionnaire informatisé proposée au sein de la SFCP devait préciser la place de la vaginoplastie sigmoïdienne dans le traitement d'une atrésie vaginale et permettre le recueil de données opératoires des patientes opérées par VSC. Neuf centres ont répondu au questionnaire. Six centres utilisent la VSC dont un privilégie la technique de Davydov cœlioscopique. Trois réalisent une VS par laparotomie, deux privilégient la méthode de Franck. Onze VSC (moyenne d'âge 14 ans, de 5 à 28) ont été évaluées. L'utilisation du futur introït pour la pince à suture automatique sectionnant le transplant colique, pour mettre en place l'enclume de la pince circulaire du rétablissement de la continuité sont fréquentes (4 centres) et font référence aux techniques N.O.T.E.S. Une conversion. Les écoulements de mucus sont considérés peu ou pas gênants (3 patientes), et la vie sexuelle satisfaisante (cotée à 4/5). La VSC est peu décrite dans la littérature. Elle permet une approche non invasive efficace, avec un bon résultat rapidement obtenu pour une activité sexuelle satisfaisante.