[Does prenatal diagnosis modify neonatal management and early outcome of children with esophageal atresia type III?]. / Le diagnostic anténatal modifie-t-il la prise en charge néonatale et le devenir à 1 an des enfants suivis pour atrésie de l'œsophage de type III ?

OBJECTIVE: Evaluate neonatal management and outcome of neonates with either a prenatal or a post-natal diagnosis of EA type III. STUDY DESIGN: Population-based study using data from the French National Register for EA from 2008 to 2010. We compared children with prenatal versus post-natal diagnosis in regards to prenatal, maternal and neonatal characteristics. We define a composite variable of morbidity (anastomotic esophageal leaks, recurrent fistula, stenosis) and mortality at 1 year. RESULTS: Four hundred and eight live births with EA type III were recorded with a prenatal diagnosis rate of 18.1%. Transfer after birth was lower in prenatal subset (32.4% versus 81.5%, P<0.001). Delay between birth and first intervention was not significantly different. Defect size (2cm vs 1.4cm, P<0.001), gastrostomy (21.6% versus 8.7%, P<0.001) and length in neonatal unit care were higher in prenatal subset (47.9 days versus 33.6 days, P<0.001). The composite variables were higher in prenatal diagnosis subset (38.7% vs 26.1%, P=0.044). CONCLUSION: Despite the excellent survival rate of EA, cases with antenatal detection have a higher morbidity related to the EA type (longer gap). Even if it does not modify neonatal management and 1-year outcome, prenatal diagnosis allows antenatal parental counseling and avoids post-natal transfer.

[Epidemiology and management of rare paediatric tumours within the framework of the French Society for Children Cancer]. / Tumeurs rares en pédiatrie : particularités épidémiologiques et organisation de leur prise en charge dans le cadre de la Société française des cancers et des leucémies de l'enfant et de l'adolescent (SFCE).

Less than 1% of cancer occurs in children. With the progress made by national and international cooperative groups 75% of them are actually cured. However some entities have an incidence so weak that we can't actually establish standardized therapeutics guidelines. To improve our knowledge on these rare tumours a national organisation become necessary as well as an international collaboration. A French rare tumour committee was created within the French Society for Children Cancer (SFCE). Others European countries have such organisation. The objectives of these tasks groups are to enhance our knowledge of the real incidence of these rare tumours, their evolution, and to propose therapeutic recommendations for each of them. This article focuses on the specific French organization for rare tumours treatment. It also describes the draft for the creation of a new data base for prospective registry of clinical, therapeutics and follow up data. To provide a better understanding of these pathologies, the "Bulletin du Cancer's" editorial board decided to regularly publish an update on a rare paediatric tumour in a specific section.

Surgical management of neuroblastoma-related hepatomegaly: do material and method really count?

The authors report 2 cases of neuroblastoma-associated hepatomegaly, which were treated using a Silastic patch, and discuss in the light of recent reports, the technical aspects and outcome of these children. They were satisfied by the decompression achieved with the patch and believe there is no increased risk in using Silastic rather than other types of material. The outcome for these children depends more on the evolution of the underlying disease than the technical aspects of the abdominal decompression.

Intravesical instillation of ropivacaine reduces bladder spasms following paediatric ureteroneocystostomy.

OBJECTIVE: Bladder spasms are a common cause of pain after surgical procedures that call for postoperative catheter drainage. Several therapeutic methods have been used to lessen these spasms but none have received widespread success. PATIENTS AND METHODS: Twenty-six children were included in a prospective randomized trial to evaluate the safety and efficacy of daily intravesical instillation of ropivacaine as prophylactic treatment for bladder spasms following ureteroneocystostomy. RESULTS: Although six patients experienced mild transient pain during instillation, there was no systemic toxicity attributable to the ropivacaine. The average number of spasms per day fell by half in the instillation group (p<0.01). CONCLUSION: Intravesical instillation of ropivacaine is a feasible alternative prophylactic treatment for postoperative bladder spasms.

Complications occurring during conservative management of splenic trauma in children.

AIM: Conservative management of splenic rupture in haemodynamically stable children is now generally accepted. However, during follow-up, many complications can occur. The aim of this study was to describe the complications we observed and to propose a standardised follow-up adapted to them. METHODS: Between March 1992 and December 2002 we managed 65 children (aged between 3 and 15 years old) with accidental splenic rupture. Follow-up and treatment consisted of a 10-day bed rest with sonogram and Doppler controls on the 5th and 10th day and subsequently every month until complete healing. Complications included secondary haemorrhage in 3 cases, cystic evolution in 5 cases, and pseudo-aneurysm in 2 cases. All were treated by renewed bed rest. Three of the cysts resolved spontaneously, the other two underwent cystic resection and epiploplasty by laparotomy (n = 1) or laparoscopy (n = 1) and both pseudo-aneurysms were selectively embolised. There were no splenectomies. CONCLUSIONS: Complications essentially occurred in older children and were not related to gender, type of fracture, or extent of bleeding. Cystic evolution of the sub-capsular haematomas can appear up to 1 month after trauma. Peripheral pseudo-aneurysms which could be responsible for secondary haemorrhages were selectively embolised. We favour the use of the Doppler sonogram for follow-up until total recovery of these patients, even in low-grade traumas. Considering the number of complications encountered we do not believe the American evidence-based guidelines are suitable for our population.

[Thoracic epidural analgesia for a thrombocytopenic child]. / Analgésie péridurale thoracique chez un enfant thrombopénique.

Per- and postoperative analgesia of patients with chronic pain is a challenging issue for anaesthetists and can be achieved with locoregional anaesthesia, unless it is contraindicated. We report the case of a thrombocytopenic child presenting for thoracotomy and in whom intravenous analgesia failed after previous surgeries. Due to the central origin of the thrombocytopenia, thoracic epidural analgesia could be achieved, after platelet transfusions. When clinical advantage of thoracic epidural is unquestionable, central thrombocytopenia is a relative contraindication.

[Spontaneous rupture of a choledochal cyst in a 11-month-old girl]. / Rupture spontanée d'un kyste du cholédoque chez une enfant de 11 mois.

Choledochal cysts are rare congenital malformations of the biliary tract. Though most cysts are diagnosed incidentally, some present directly with complications. We report on the case of an 11-month-old girl admitted for abdominal pain, fever and vomiting. Ultrasonography revealed intraabdominal fluid and the absence of a choledochal cyst diagnosed 2 months earlier. Laparotomy for suspected rupture of a choledochal cyst was planned and a choledochojejunostomy with Roux-en-Y was performed. Spontaneous rupture of a choledochal cyst is rare and occurs most frequently in children under the age of 4. The exact cause is yet unknown and several factors have been implicated. The most probable cause is the combination of pancreatic reflux and epithelial irritation of a weakened cyst wall. Choledochal cysts should preferably be treated as soon as the child is 6 months old. Complete excision of the cyst is mandatory because of the risk of malignant transformation.

[Delayed discovery of congenital diaphragmatic hernia: diagnostic difficulties. A report of two cases]. / Les hernies diaphragmatiques congénitales de révélation tardive: difficultés diagnostiques. A propos de deux cas.

Delayed revelation of congenital diaphragmatic hernias (CDH) is not uncommon and can represent 5-30% of total CDHs. Time before diagnosis may be prolonged, sometimes to the adult period. Respiratory and gastrointestinal symptoms are frequent but not specific. The clinical presentation of delayed CDH may thus mislead the practitioner. Diagnosis can be approached and/or confirmed by plain radiography. Outcome is usually favorable after surgery. We report two cases of delayed CDH and we discuss the difficulty of diagnosis.

[Prognosis of neuroblastoma in childhood. Methods of assessment and clinical use]. / Le pronostic du neuroblastome de l'enfant. Méthodes d'étude et utilisation en pratique clinique.

Neuroblastoma and its benign counterpart, ganglioneuroma, are pediatric neuroblastic tumors arising in the sympathetic nervous system from neural-crest cells. Neuroblastoma, the most common extra-cranial solid tumour during childhood, is unique for its broad spectrum of clinical virulence from spontaneous remission to rapid and fatal progression despite intensive multimodality therapy. To a large extent, outcome could be predicted by the stage of disease and the age at diagnosis. However, a number of molecular events in neuroblastoma tumors, accounting for the variability of outcome and response to therapy, have been identified over the past decades. Among these, MYCN amplification is the most relevant prognostic factor and was the first genetic marker, in paediatric oncology, to be included in clinical strategies as a guide for therapeutic decision. This has allowed the most suitable intensity of therapy to be delivered according to a risk-stratified strategy, from observation to megadose chemotherapy with stem cell transplantation. Recent advances in understanding the biology and genetics of neuroblastoma will ultimately allow to select poor-risk patients for appropriate future biologically based therapies.

Treatment of post-appendectomy intra-abdominal deep abscesses.

The treatment of acute appendicitis in children is sometimes followed by complications including intra-abdominal abscess, for which the traditional treatment is surgical drainage. We evaluated the efficacy of antibiotic management compared to classic surgical treatment. This retrospective study investigated 22 children from 5 to 13 years of age with one or many abscesses after appendectomy, treated between 1992 and 2002. Eleven received surgery and the other 11 were treated with triple antibiotherapy. The two groups were comparable. Surgery was efficient in 36% of cases and complications occurred in 64% of cases (digestive fistula, intraperitoneal abscess, gaseous gangrene and septic shock). Average hospital stay in this group was 16.7 days. In the other group, medication was efficient in 91% of cases; a recurrent abscess was operated and a residual stercolith, which was maintaining the infection, was removed. The average hospital stay in this group was 10.4 days. Medical treatment of intraperitoneal abscess seems to be effective. Hospitalisation is shorter with medical management and complications are rare. Therefore, medication may be proposed in most cases, except when there are residual foreign bodies or stercoliths. Poor patient status and septic shock are the two other contraindications, because antibiotherapy is not immediately efficient.