To determine whether extrauterine growth is associated with neurologic outcomes and if this association varies by prenatal growth profile.For 1493 preterms from the EPIPAGE (Étude Épidémiologique sur les Petits Âges Gestationnels [Epidemiological Study on Small Gestational Ages]) cohort, appropriate for gestational-age (AGA) was defined by birth weight >-2 SD and small for gestational-age (SGA) by birth weight ≤-2 SD. Extra-uterine growth was defined by weight gain or loss between birth and 6 months by z-score change. Growth following-the-curve (FTC) was defined as weight change -1 to +1 SD, catch-down-growth (CD) as weight loss ≥1 SD, and catch-up-growth (CU) as weight gain ≥1 SD. At 5 years, a complete medical examination (n = 1305) and cognitive evaluation with the Kauffman Assessment Battery for Children (n = 1130) were performed. Behavioral difficulties at 5 years and school performance at 8 years were assessed (n = 1095).Overall, 42.5% of preterms were AGA-FTC, 20.2% AGA-CD, 17.1% AGA-CU, 5.6% SGA-FTC, and 14.5% SGA-CU. Outcomes did not differ between CU and FTC preterm AGA infants. Risk of cerebral palsy was greater for AGA-CD compared with AGA-FTC (aOR 2.26 [95% CI 1.37-3.72]). As compared with children with SGA-CU, SGA-FTC children showed no significant increased risk of cognitive deficiency (aOR 1.41[0.94-2.12]) or school difficulties (aOR 1.60 [0.84-3.03]). Compared with AGA-FTC, SGA showed increased risk of cognitive deficiency (SGA-FTC aOR 2.19 [1.25-3.84]) and inattention-hyperactivity (SGA-CU aOR 1.65 [1.05-2.60]).Deficient postnatal growth was associated with poor neurologic outcome for AGA and SGA preterm infants. CU growth does not add additional benefits. Regardless of type of postnatal growth, SGA infants showed behavioral problems and cognitive deficiency.

OBJECTIVE:To estimate the prevalence of cerebral palsy at 2 years of age among children born very preterm, according to gestational age, infant gender, plurality, and neonatal cranial ultrasound abnormalities.METHODS:All infants born between 22 and 32 weeks of gestation in 9 regions of France in 1997 were included in this prospective, population-based, cohort study. The main outcome measure was cerebral palsy prevalence at 2 years. Of the 2364 survivors eligible for follow-up evaluation, 1954 (83%) were assessed at 2 years of age.RESULTS:Among the 1954 children assessed at 2 years, 8.2% had cerebral palsy. Bilateral spastic cerebral palsy, hemiplegia, and monoplegia accounted for 72%, 9%, and 10% of cases, respectively. Fifty percent of the children with cerebral palsy walked independently at the age of 2, 31% were unable to walk but could sit independently, and 19% could not sit (unable to maintain head and trunk control). The prevalence of cerebral palsy was 20% at 24 to 26 weeks of gestation, compared with 4% at 32 weeks. On the basis of ultrasound findings in the neonatal period, we found that 17% of children with isolated grade III intraventricular hemorrhage and 25% of children with white matter damage (ie, ventricular dilation, persistent echodensities, or cystic periventricular leukomalacia) had cerebral palsy, compared with 4% of children with normal ultrasound scans.CONCLUSIONS:Despite recent improvements in survival rates, cerebral palsy remains highly prevalent among very preterm children. Severe cranial ultrasound abnormalities predict motor disability strongly, but one third of infants with cerebral palsy had no ultrasound abnormalities.

Objective To analyze the main clinical features, genetic mutations, and outcomes of patients of the French Congenital Central Hypoventilation Syndrome (CCHS) Registry. Design A country-wide cohort established throughout a long-term multicenter effort. Patients Seventy French patients with CCHS (29 male patients and 41 female patients) Methods The following items were analyzed: the most important moments of the disease course; the main clinical characteristics; associated pathologic conditions; management; clinical outcome; and genetic mutations. Results An average of four new cases of CCHS per year was observed in the last 5 years. Thus, the incidence may be estimated to be 1 per 200,000 live births in France. The median age at diagnosis was 3.5 months (range, 0.5 to 15 months) before 1995 and < 2 weeks in the last 5 years (p = 0.01). CCHS occurred in isolation in 58 of 70 patients. In the remainder, it was associated with Hirschsprung disease (HSCR) [nine patients], Hirschsprung and neural crest tumor (two patients), and growth hormone deficiency (one patient). Among the 50 patients who lived beyond 1 year of age, all but one received nighttime ventilation, with 10 of them (20%) receiving it noninvasively. Three patients (6%) required daytime ventilatory support in addition to nighttime ventilation. The overall mortality rate was 38% (95% confidence interval [CI], 27 to 49%). The median age at death was 3 months (range, 0.4 months to 21 years). The 2-year mortality rate was greater in male patients than in female patients (p = 0.02; relative risk [RR], 2.71; 95% CI, 1.14 to 6.47) but was not affected by HSCR (p = 0.93; RR, 0.95; 95% CI, 0.28 to 3.2). The 43 patients who are currently alive (11 men; sex ratio, 0.4) have a mean age of 9 years (range, 2 months to 27 years). Among the 34 patients tested thus far, heterozygous mutations of the paired-like homeobox gene 2B (PHOX2B) gene were found in 31 patients (91%). Conclusion Our four major findings are the extreme rarity of CCHS, the improved recognition over time, the lack of effect of HSCR on the mortality rate, and the high frequency of PHOX2B mutations.

La plupart des recommandations concernant la réanimation du nouveau-né en salle de naissance reposent sur l’expérience et n’ont pas été scientifiquement établies. C’est le cas de l’utilisation de l’oxygène pur. Aujourd’hui, ces pratiques sont remises en question, à la lumière des conséquences de l’hyperoxie et de plusieurs méta-analyses récentes. En cas d’anoxie périnatale modérée chez le nouveau-né à terme, une ventilation avec de l’air peut ainsi être proposée en première intention. Cependant, de nouvelles études randomisées multicentriques, avec stratification selon la cause de l’asphyxie et l’âge gestationnel, sont nécessaires avant d’envisager des recommandations du même type pour les nouveau-nés présentant les formes les plus sévères d’asphyxie périnatale ou chez les prématurés. Dans les situations les plus graves, l’introduction secondaire d’oxygène, à une concentration mesurée et sous contrôle permanent de la saturation par oxymétrie pulsée, paraît pour l’instant de bonne pratique.

Most of the contemporary guidelines on newborn resuscitation are based on experience but lack scientific evidence. The use of 100% oxygen is one of the more evident. Today, these practices are questioned, particularly for the resuscitation of moderately depressed full term or near term newborns. Results of recent meta-analysis of trials that compared ventilation with air versus pure oxygen at birth suggests current practices should be revisited. On the basis of these data, air can be the initial gas to use for these babies. Large scale trials, including preterm and cause and/or severity of initial asphyxia, must now be undertaken before the publication of new guidelines for these populations. Particularly severely asphyxiated infants might require supplemental oxygen with titration of oxygen delivery and continuous monitoring of oxygen saturation.

La survenue d'un malaise anoxique grave en salle de naissance avant deux heures de vie chez un nouveau-né ayant présenté une adaptation à la vie extra-utérine parfaite, est une entité mal connue. Les auteurs rapportent six observations de malaises constatés chez des patients entre 20 et 120 minutes après la naissance. Ces enfants avaient un âge gestationnel d'au moins 36 SA. Ils étaient nés par voie basse après une grossesse normale et un accouchement eutocique. Le score d'Apgar était de 10 à cinq et à dix minutes et l'examen initial était normal dans tous les cas. Ils ont été découverts en arrêt cardiorespiratoire, la face enfouie sur le ventre, le sein ou dans le cou de leur mère. Toutes les mères étaient primipares. Cinq enfants sur six sont décédés. Les examens complémentaires étaient normaux (y compris l'autopsie des enfants décédés). La cause la plus probable de ces malaises est l'asphyxie par obstruction des voies aériennes supérieures du nouveau-né placé contre sa mère. La gravité de ces observations suggère de toujours veiller au dégagement de la face du nouveau-né lorsqu'il est placé après la naissance contre sa mère et d'être particulièrement vigilant chez les mères primipares.

Cardiorespiratory arrest occurring within the first two hours of life of a perfectly normal newborn is a very seldom event hitherto unreported. Six infants born after an uneventful pregnancy by normal vaginal delivery, with a normal Apgar score and physical examination, were found with unexpected cardiorespiratory arrest requiring cardiac and respiratory resuscitation early after birth. All were lying in the prone position, their face covered up while facing mother's abdomen, breast or neck. All mothers were primipara. All newborns but one died. Biological and bacteriological samples were normal and early onset neonatal sepsis was ruled out. Autopsy, performed in five infants, was not contributive. We hypothesize that the sudden and unexpected cardiorespiratory arrest occurring in these normal newborns was secondary to acute upper airway obstruction. To prevent this life threatening post-natal asphyxic episode, it is essential to ensure that the face of a newborn lying down upon mother's breast and abdomen is properly and continuously cleared.

Caring for extremely premature babies is difficult and costly. Mortality has been reduced with recent medical progress, but at the price of an increased number of surviving infants with handicaps. Should we then fix firm limits (gestational age and/or birthweight) for deciding on whether or not to take medical action? There is however the question of whether it is ethically acceptable to define human life solely on the basis of the length of gestation or birthweight. Moreover, what risk level for death or handicap is legitimate for treating or not a premature baby? The issue thus comes to the worthiness of trying first to save life, then accepting an interruption of curative treatments later on if severe cerebral injuries become evident. Who should make the decisions? Guidelines have been published by medical associations to help professionals to answer these important and puzzling questions.

Pour faire survivre des enfants nés très prématurément, il faut engager de très gros moyens. Les récents progrès techniques et thérapeutiques ont permis de diminuer leur mortalité, mais cet avantage s’accompagne d’une augmentation du nombre de survivants handicapés. Faut-il donc fixer des limites (en âge gestationnel et/ou en poids de naissance) à nos interventions médicales ? Cependant, au plan de l’éthique médicale, peut-on admettre de définir des êtres humains uniquement sur des critères de poids ou de durée de gestation ? Et quel pourcentage de risque de décès ou de handicap est-il légitime de retenir pour décider ou non une action thérapeutique ? Ne vaut-il pas mieux tenter d’abord de sauver la vie, au risque de devoir l’arrêter ultérieurement si apparaissent de graves lésions cérébrales ? Qui doit décider ? Telles sont quelques unes des questions, particulièrement complexes à résoudre, qui se posent aux équipes obstétrico-pédiatriques et pour lesquelles des recommandations ont été proposées par quelques sociétés savantes.

L’objectif de ce travail est d’analyser l’évolution des enfants infectés in utero par le CMV après primo-infection maternelle et a ou pauci-symptomatiques pendant la grossesseet à la naissance (c’est-à-dire présentant des anomalies mineures et isolées).

UNLABELLED:The setting up of the so-called "decrees on perinatal safety" on October 1998 has been associated with many difficulties which were apparently related to the lack of beds for intensive care units, special care units and neonatal medicine. This led to a national survey.OBJECTIVES:The aim of the survey was : (1) to collect the number of neonates requiring hospitalization in NICU and special care units over a 1-week period in metropolitan France and overseas departments and territories; (2) to assess the needs in equipments and care-givers.METHODS:The writs to be included in the survey were previously identified. Each day of hospitalization was classified as needing an intensive care unit, a special care unit or a neonatal unit. Then it was classified as well fitted or badly fitted.RESULTS:Two hundred and forty units (90% of the French units) from 204 hospitals participated in the survey and 3678 neonates were included and accumulated 17 583 days of hospitalization (NICU: 2728; special care: 5047; neonatal medicine: 9808). One thousand and five hundred and ninety hospitalization days did not fit well either with the technical level required by the neonate or/and with the location of the parents' home (9.2%): 23.1% in overseas departments and territories; 12% in metropolitan France. The main reasons for maladjustment were: a too high technical level: (59%); an insufficient technical level: (21%) (19 neonates could not be admitted in a NICU as they needed). The survey included 158 NICU and special care units. Taking into consideration the French law: the lack in equipment was: 294 ventilators, 231 cardio-respiratory monitors, 116 pulse oxymeters and 513 blood pressure monitors; 561 additional pediatricians were needed to allow a medical night duties including seven doctors in each NICU and each special care unit; 1878 additional nurses were also needed. Making the assumption that the mean occupation rate of the neonatal beds should be 70%, the needs were calculated for 1000 live births: metropolitan France: 0.76 (0.74; 0.78) in NICU; 1.45 (1.43-1.47) in special care units; overseas departments and territories: 2 (1.8-2.5) in NICU; 3.5 (3.2-3.8) in special care units.CONCLUSION:Finally, the main deficit was not related to the number of beds but to the equipment and number of care-givers. The status of overseas departments and territories was particularly worrying.

Les malformations congénitales de la main regroupent un nombre important de situations cliniques. Leur expression est, comme dans toutes les pathologies congénitales, variable. De ce fait, on peut presque considérer que chaque situation clinique est un peu unique. La difficulté, comme toute la pathologie congénitale, réside dans le fait que les tableaux cliniques rencontrés sont extrêmement divers, difficilement classifiables. Aussi, la conduite à tenir et les décisions chirurgicales sont souvent affaire d’« École ». Ceci est d’autant plus vrai que l’évaluation des résultats est rendue difficile en raison des faibles séries (donc de leur valeur statistique) et de l’évaluation fonctionnelle qui sera due en partie à la croissance de l’enfant. Il est impossible en quelques pages de décrire la totalité des malformations du membre supérieur, de résumer leur prise en charge et d’évaluer les résultats obtenus. Aussi, ce chapitre ne sera pas exhaustif et nous nous attacherons essentiellement aux pathologies les plus fréquemment rencontrées.Congenital deformities of the hand and upper limb include a significant number of clinical situations. Their expression is, as in all congenital diseases, variable. Therefore, we can almost consider that each clinical situation is a bit unique. The difficulty, as any congenital disease, is the fact that the clinical cases are extremely diverse and difficult to classify. So what to do and surgical strategies are often matter of “School”. This is even more true that the evaluation of results is very difficult due to low series (poor statistical value) and functional assessment to be partly due to the growth of the child. It is impossible in a few pages to describe all malformations of the hand and upper limb, summarize the indications and evaluate the results. Also, this chapter is not exhaustive and we will focus primarily on the most frequent pathologies.