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La transposition des gros vaisseaux est une cardiopathie congénitale cyanogène qui se manifeste en période néonatale par une cyanose sans détresse respiratoire, réfractaire à l'administration de l'oxygène. Elle nécessite toujours une prise en charge urgente avec un transfert rapide vers un centre expert. Dans la majorité des cas, le diagnostic est fait en prénatal permettant l'organisation de la naissance dans un centre susceptible de pratiquer une atrioseptostomie de Rashkind à tout moment. La prise en charge médicale préopératoire repose sur l'administration des prostaglandines et la manœuvre de Rashkind pour optimiser le mélange entre les deux circulations parallèles et donc l'oxygénation tissulaire. Le switch artériel est l'opération de choix habituellement réalisée dans les premières deux semaines de vie. L'intervention rétablit l'anatomie en remettant les gros vaisseaux au-dessus de leurs ventricules appropriés. La mortalité postopératoire est essentiellement liée aux problèmes de réimplantation coronaire dans des distributions complexes notamment celles avec un trajet intramural. Globalement, les résultats postopératoires et le pronostic fonctionnel à long terme sont excellents. Un sujet de préoccupation est l'émergence des troubles du développement neuropsychologique dans certains domaines d'apprentissage chez les enfants et adolescents opérés d'une transposition. Les enfants concernés nécessitent une prise en charge pluridisciplinaire en fonction de leurs déficits. This article aims to update general practitioners and pediatricians on neonatal management of transposition of the great arteries. This cardiac defect always requires urgent postnatal management and transfer to a tertiary care center. In France, prenatal detection rates are high reaching 80–90% in some regions. Prenatal diagnosis allows for scheduling of delivery in a specialist center with the possibility to perform a Rashkind manoeuvre at any time. Medical preoperative management relies on prostagladin infusion and Raskind manoeuvre in order to obtain a sufficient mixing of the two parallel circulations. Ideally minimal efficient doses of prostaglandin should be administered to obtain ductal patency with little side effects. The arterial switch is the surgery of choice mostly performed in the first two weeks of life. This intervention restores physiological anatomic conditions in putting the great arteries at their respective place on the appropriate ventricle. During the procedure the coronary arteries are reimplanted in the neoartic root. Postoperative morbidity and mortality are mostly due to complications after reimplantation of complex coronary pattern, especially those with an intramural course. In general, the postoperative results after arterial switch and the long-term functional prognosis are excellent. A recent concern lies in the growing detection of learning difficulties and altered neuropsychological development in some operated children and adolescents. Children with specific needs should benefit from early pluridisciplinary support. [ABSTRACT FROM AUTHOR]

Summary: The ureteric-pelvic junction (UPJ) is an uropathy that his management had profoundly evolved during these last thirty years, both from the point of view of the care and diagnosis. Purpose: The purpose of this study is to discuss a diagnostic and therapeutic strategy of this uropathy. Patients and methods: This study concerning 53 children (59 UPJ with antenatal diagnosis) brought together in the unity of paediatric surgery of 1999 in 2008. We classified the patients according to their care: group I: surgical at once; group II: medical then secondarily surgical; group III: purely medical. Results: This study was about 34 boys and 19 girls with a sex ratio of 1.8. The UPJ was noticed in 26 cases to the left. The vesico-ureteral reflux was the most frequent associated abnormality (five cases). The majority are a part of the group I (42%). The anteroposterieur diameter in the ultrasound was on average 32.65mm for the group I while it was respectively 25.86 and 14mm for the group II and III. The differential renal average function in the scintigraphy (DTPA or MAG III) was normal (46%) in the group III while was 35 and 38% respectively in the group I and II. The nephrogram was obstructive in 72 and 75 % of the cases respectively in the group I and II and only in 16 % of the cases in the group I. The final comparison of the differential renal function between the group I, and the group II, does not show a significant difference (P <0.005). Thirty-three patients benefited from an initial medical supervision. Only sixteen children (48%) had pyeloplasty. Conclusion: The big frequency of the spontaneous improvements at the infant by simple tissular maturation (30% in this series), and the low incidence of the postoperative complications of the patients operated late do not authorize any more the premature pyeloplasty in front of an antenatal diagnosis UPJ. [Copyright &y& Elsevier]

Summary: The malformative uropathy (MU) is frequent in paediatrics. Its gravity resides in the renal failure, that is often associated, hence the interest of an early diagnosis and a suitable management. The authors of this study wanted to determine the epidemiological and evolutionary features of the MU in a service of general paediatrics. It is a retrospective survey on eight and a half years (from January 2000 to June 2008), concerning 71 cases of MU, with 37 boys and 34 girls (sex ratio of 1.08). The mean age at diagnosis was 29.4 months, with extremes of one day to 13 years. The majority of urinary malformation was represented by the vesical urethral reflux (RVU) [45.3 %], the syndrome of the pyelo-ureteral junction (SJPU) [13.8 %] and the mega-ureter (MU) [12 %]. A feminine predominance was noticed in the RVU (63.3 %), but the children with SJPU or MU were mostly boys (80.1 %). The MU was bilateral in 33.8 % of the cases. An association of two MU or more different type was found for 12 of the patients (16.9 %), whose majority was of masculine sex (83.3 %). The diagnosis was mostly established after birth for an urinary infection (69 %), a growth delay (5.6 %) or a neonatal distress respiratory (4.2 %). The fever was the major physical sign found (70 %), and it was most often in association with an urinary infection. The renal ultrasonography was realised in first intention in nearly all cases (98.5 %), and it was abnormal in 47 cases. The pyelocalicial dilatation was predominant (29.5 %). The radiological retrograde uretrocytography (UCR) was indicated in 52 children to reveal anomalies of lower urinary tract; it permitted the diagnosis of 49 RVU and three valves of the posterior uretera. Four girls and 14 boys benefited from a surgical correction adapted to every type of MU. The mean age of the patients at surgery was 38.4 months. A spontaneous resolution of the MU was observed for 21 patients; it concerned 20 RVU, seven SJPU and four MU. Two of the patients evolved progressively towards a chronic renal failure. The antenatal diagnosis (DAN) should be currently the main circumstance of discovery of these anomalies whose majority is constituted of forms little or non complicated that present a prognosis and a favourable evolution spontaneously or after surgical correction. [Copyright &y& Elsevier]