Prenatal imaging presentation of Meckel diverticulum

International audienc

Prognosis of isolated congenital diaphragmatic hernia using lung-area-to-head-circumference ratio: variability across centers in a national perinatal network

Objective: Congenital diaphragmatic hernia (CDH) is a severe anomaly. The observed-to-expected lung-area-to-head-circumference ratio (o/e-LHR) has been shown to provide a useful prediction of subsequent survival of fetuses with CDH in referral centers with expertise and a large caseload. However, the accuracy of o/e-LHR measurements in general fetal medicine units with less expertise is not well known. The aim of this study was to evaluate the capacity of o/e-LHR to provide a useful prediction of mortality of fetuses with CDH when the measurement is performed in fetal medicine units with different levels of expertise. Methods: Between January 2008 and November 2013, 305 live births with expectantly managed left-sided isolated CDH were recorded in the database of the French National Center for Rare Diseases (31 centers) and followed up after birth. Centers were grouped into two categories according to their mean annual CDH caseload over the study period: large centers with an average of ≥ 14 cases and smaller centers with < 14 cases per year. The relationship between o/e-LHR and 28-day and 6-month mortality was modeled using fractional polynomials and the predictive value of o/e-LHR was quantified using the area under the receiver–operating characteristics curve. Comparisons between the two center categories were carried out. Analyses were adjusted for potential confounders such as thoracic herniation of the liver and gestational age at birth and at diagnosis. Results: During the study period, two large centers managed a total of 82 CDH cases and 29 smaller centers a total of 223 CDH cases. Overall, there was a significant inverse relationship between 28-day mortality rate and o/e-LHR, which decreased from 54% when o/e-LHR was 20% to 6% when o/e-LHR was 75% (P < 0.01). When the category of center was considered, adjusted associations between o/e-LHR and 28-day mortality were significantly different (P = 0.032) between large and smaller centers. The ability to predict survival at 28 days postpartum based on o/e-LHR was better in large centers; for a specificity of 0.30, the sensitivity was 0.71 in large centers and 0.55 in smaller ones. The results were similar for 6-month mortality. Conclusions: Our results show that o/e-LHR measured on two-dimensional ultrasound is a good indicator of neonatal prognosis in cases of CDH that may be used even in fetal medicine centers with a small caseload. However, our results also suggest that LHR measurement may be difficult to perform correctly. Therefore, appropriate training should be offered to professionals. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.SCOPUS: ar.jinfo:eu-repo/semantics/publishe

J Pediatr Surg

INTRODUCTION: Thoracotomy as surgical approach for esophageal atresia treatment entails the risk of deformation of the rib cage and consequently secondary thoracogenic scoliosis. The aim of our study was to assess these thoracic wall anomalies on a large national cohort and search for factors influencing this morbidity. MATERIALS AND METHODS: Pediatric surgery departments from our national network were asked to send recent thoracic X-ray and operative reports for patients born between 2008 and 2010 with esophageal atresia. The X-rays were read in a double-blind manner to detect costal and vertebral anomalies. RESULTS: Among 322 inclusions from 32 centers, 110 (34.2%) X-rays were normal and 25 (7.7%) displayed thoracic malformations, including 14 hemivertebrae. We found 187 (58.1%) sequelae of surgery, including 85 costal hypoplasia, 47 other types of costal anomalies, 46 intercostal space anomalies, 21 costal fusions and 12 scoliosis, with some patients suffering from several lesions. The rate of patients with these sequelae was not influenced by age at intervention, weight at birth, type of atresia, number of thoracotomy or size of the center. The rate of sequelae was higher following a classical thoracotomy (59.1%), whatever the way that thoracotomy was performed, compared to nonconverted thoracoscopy (22.2%; p=0.04). CONCLUSION: About 60 % of the patients suffered from a thoracic wall morbidity caused by the thoracotomy performed as part of surgical treatment of esophageal atresia. Minimally invasive techniques reduced thoracic wall morbidity. Further studies should be carried out to assess the potential benefit of minimally invasive approaches to patient pulmonary functions and on the occurrence of thoracogenic scoliosis in adulthood. LEVELS OF EVIDENCE: Level III retrospective comparative treatment study

J Pediatr

OBJECTIVE: To identify predictors of and factors associated with the performance of antireflux surgery during the first year of life in children born with esophageal atresia. STUDY DESIGN: All patients were included in a French registry for esophageal atresia. All 38 multidisciplinary French centers completed questionnaires about perinatal characteristics and one-year outcome for children born with esophageal atresia. RESULTS: Of 835 infants with esophageal atresia born in France from 2010 to 2014, 682 patients, excluding those with long-gap esophageal atresia, were included. Three patients had type I, 669 had type III, and 10 had type IV esophageal atresia. Fifty-three children (7.8%) received fundoplication during the first year of life. The median age at the time of the end-to-end esophageal anastomosis was 1.1 day (range 0-15). Multivariate analysis identified three perioperative factors that predicted the need for early antireflux surgery: anastomotic tension (P = .004), associated malformations (P = .019), and low birth weight (P = .018). Six other factors, measured during the first year of life, were associated with the need for antireflux surgery: gastroesophageal reflux (P < .001), anastomotic stricture (P < .001), gastrostomy (P < .001), acute life-threatening event (P = .002), respiratory complications (P = .045), and poor nutritional status (P < .001). CONCLUSIONS: Gastroesophageal reflux disease, low birth weight, poor nutrition, and surgical anastomosis difficulties predicted the performance of antireflux surgery in the first year of life in infants with esophageal atresia