Review shows that implementing a nationwide protocol for congenital diaphragmatic hernia was a key factor in reducing mortality and morbidity.

The French Rare Disease Reference Center for congenital diaphragmatic hernia (CDH) was created in 2008, to implement a national protocol for foetuses and children with this serious condition. Neonatal mortality from CDH is 30-40%, mainly due to pulmonary hypoplasia and persistent pulmonary hypertension, and half of those who live have high respiratory, nutritional and digestive morbidity. CDH management requires long-term and specialised multidisciplinary care. It has been well established that a standardised management protocol improves the prognosis of children with CDH. CONCLUSION: Organising health care and implementing a nationwide French protocol were key factors for reducing mortality and morbidity from CDH.

Impact of skin-to-skin contact on the autonomic nervous system in the preterm infant and his mother.

Before, during and after mother-newborn skin-to-skin contact (SSC), parasympathetic activity was evaluated by heart rate variability (HRV) analysis. SSC had a favorable impact on maternal and premature infant parasympathetic activities with a more pronounced response for neonates when the basal HRV values were lower, without modifications of EDIN scores, temperatures or oxygen saturation.

Safety and tolerability of subcutaneous treprostinil in newborns with congenital diaphragmatic hernia and life-threatening pulmonary hypertension.

BACKGROUND: Prolonged pulmonary hypertension (PH) is highly predictive for pulmonary morbidity and death in infants with congenital diaphragmatic hernia (CDH). OBJECTIVES: To report the effects and tolerability of subcutaneous treprostinil in newborns with severe CDH and late life-threatening PH. METHODS: We recorded clinical and echocardiography data before and after starting subcutaneous treprostinil, on patients with severe CDH and late PH, refractory to inhaled nitric oxide and oral sildenafil. RESULTS: 14 patients were treated with treprostinil (gestational age: 39.1±2.0weeks; birth weight: 3200±600g). Prior to treatment, the pre- and post-ductal SpO2 difference (Δ SpO2) was 14±10%. Treprostinil was initiated at a median age of 12days [5-157]. After starting treprostinil, ΔSpO2 decreased to 3% at day 7 (p<0.05), and the mean blood flow velocities in the right pulmonary arteries increased by 110% (p<0.05). 2 of the 14 patients died. At the age of follow up (12months to 3years), the 12 surviving infants were all weaned from respiratory support and discharged home. CONCLUSION: The subcutaneous treprostinil improves pulmonary hemodynamics and outcomes in infants with CDH and life-threatening PH. We suggest that the treatment should be considered in infants with severe CDH and late PH. TYPE OF STUDY: Case series with no comparison group. LEVEL OF EVIDENCE: Level IV.

[Infusion of vasoactive drugs at low flow rate: effects of the syringe switch and syringe volume (experimental study)]. / Perfusion des médicaments vasoactifs à très faible débit : influence des modalités du relais et du volume des seringues (étude expérimentale).

OBJECTIVE: The flow rate of intravenous administration of vasoactive agents should be steady in order to prevent changes in hemodynamics. In the newborns, because the flow rate is often lower than 1mL/h, it is difficult to switch of the syringe. The aim of our study was to compare the variations of concentration of active substance delivered after a manual or an automatic switch off the syringe with three different volumes (10mL, 20mL and 50mL). MATERIALS AND METHODS: Glucose solution (10g/L) was used to simulate the administered substance. Saline was administered in "Y" simultaneously with the glucose solution through a catheter. The infused substance was collected at the tip of the catheter. The glucose concentration was measured at 15min-interval for 3hours. RESULTS: The manual switch of the syringe was associated with a significant alteration of the flow rate, lasting more than 15min. In contrast, the automatic switch of syringes was associated with no change of the flow rate, especially with small-volume syringes (10mL). CONCLUSION: In newborns, in order to prevent the change in flow rate of drugs after a switch of syringes, our results suggest the use of syringe-pump with built-in automatic switch and small-volume syringes.

[The long-term follow-up of patients with a congenital diaphragmatic hernia: review of the literature]. / Hernie de coupole diaphragmatique: devenir à long terme.

The increased survival rate reached in infants with congenital diaphragmatic hernia (CDH) has shown a concomitant increase in late morbidity. Many complications including pulmonary damage, cardiovascular disease, gastro-intestinal disease, failure to thrive, neurocognitive defects and musculoskeletal abnormalities have been described. Long-term pulmonary morbidity in CDH consists of obstructive and restrictive lung function impairments due to altered lung structure and prolonged ventilatory support. Long-term consequences of pulmonary hypertension are unknown. Gastro-esophageal reflux disease (GERD) is also an important contributor to overall morbidity. Failure to thrive may be caused by GERD, insufficient intake due to oral aversion and increased caloric requirements due to pulmonary morbidity. Neurological outcome is determined by an increased risk of perinatal and neonatal hypoxemia in the first days of life of CDH patients. Many studies have addressed the substantial impact of the health problems described above, on the overall well-being of CDH patients, but only a few studies focus on the health-related quality of life. Considering the scattered data indicating substantial morbidity in long-term survivors of CDH, follow-up studies that systematically assess long-term sequelae are needed. Based on such studies, a more focused approach for routine multidisciplinary follow-up programs could be established. It is the goal of the French Collaborative Network to promote exchange of knowledge, future research and development of treatment protocols.

[Impact of cocooning and maternal voice on the autonomic nervous system activity in the premature newborn infant]. / Impact du cocooning et de la voix humaine sur le système nerveux autonome (SNA) de l'enfant grand prématuré

INTRODUCTION: Discomfort, pain, and stress have an adverse impact on the psychomotor development in the premature newborn infant. Recent studies indicate that pain and stress are associated with a reduction of parasympathetic outflow. We hypothesized that cocooning associated with the human voice has a favorable impact on parasympathetic activity in the premature newborn infant. METHOD: We compared heart rate variability (HRV) before and after standardized cocooning phases associated with the human voice and carried out: 1) by the mother and 2) by a third person. HRV was assessed and expressed as an index reflecting the parasympathetic tone. RESULTS: Ten children were included (median gestational age, 33 weeks (30(+4)-33(+2))). We observed a higher HRV index after the period of cocooning associated with the human voice compared with the baseline measurement (P<0.05), whether the procedure was carried out by the mother or a third person. CONCLUSION: This study shows that cocooning associated with the human voice enhances HRV in the preterm newborn infant, indicating an increase in parasympathetic activity after cocooning associated with the human voice. However, the impact is similar whether the cocooning associated with the human voice is performed by the mother or a third person. This result suggests that cocooning associated with the human voice carried out either by the mother or a third person contributes to decreasing stress and discomfort in the premature newborn infant.

Very preterm children free of disability or delay at age 2: predictors of schooling at age 8: a population-based longitudinal study.

AIM: To study the predictive value of a developmental assessment at 2 years corrected age (CA) for schooling at age 8 in children born very preterm and free of disability or delay; to identify other factors associated with schooling in this population. METHODS: 244 children born before 33 weeks in 1997, part of the population-based EPIPAGE cohort study, free of disability or delay, had their developmental quotient (DQ) evaluated with the Brunet-Lezine scale at 2 years CA. The mental processing composite (MPC) score was evaluated at age 5 with the K-ABC battery. Data on schooling were obtained at age 8 by postal questionnaire. Schooling was considered appropriate if the child was attending age-appropriate grade level in a regular classroom environment without support at school. RESULTS: Schooling was appropriate for 172 (70%) children. The predictive value of a DQ≥100 for appropriate schooling was 0.80 [0.75;0.85]. In children with a DQ at age 2<100, schooling varied significantly according to their MPC score at age 5 whereas it didn't in children with a DQ≥100. In multivariate analysis, the rate of appropriate schooling was significantly related to global DQ at age 2 (p<0.01), gestational age≥29 weeks (p<0.05), head circumference at age 2 (p<0.05) and mother's educational level (p<0.05). CONCLUSION: A DQ≥100 cannot be solely used for the prediction of appropriate schooling at age 8. Mother's educational level, gestational age and head circumference at age 2 could be taken account. These factors could be used to individualise follow-up.

[Management of severe respiratory failure in the newborn: indication of inhaled NO]. / Prise en charge de la détresse respiratoire sévère du nouveau-né: place du NO inhalé.

Severe respiratory failure results from large right to left shunting. In the newborn, both intrapulmonary and/or extrapulmonary shunting may cause severe respiratory failure. Right to left extrapulmonary shunting is associated with pulmonary hypertension (PPHN). Management depends on the main mechanism of the respiratory failure. Intrapulmonary shunting requires optimizing lung recrutment, whereas vascular recrutment is required to decrease extra-pulmonary shunting. Analysis of anamnesis, clinical examination, chest X-ray, and echocardiography allows distinguishing the mechanism of the respiratory failure. In case of PPHN, favorizing factors such as acidosis, stress, lung over distension, and polyglobuly should be treated. Inhaled NO is recommended in the treatment of PPHN after optimizing lung volume.