Didier Aubert

Esophageal atresia: Data from a national cohort

PURPOSE A prospective national register was established in 2008 to record all new cases of live-birth newborns with esophageal atresia (EA). This epidemiological survey was recommended as part of a national rare diseases plan. METHODS All 38 national centers treating EA participated by completing for each patient at first discharge a questionnaire validated by a national committee of experts. Data were centralized by the national reference center for esophageal anomalies. Quantitative and qualitative analyses were performed, with P-values of less than 0.05 considered statistically significant. Results of the 2008-2009 data collection are presented in this report. RESULTS Three hundred seven new living cases of EA were recorded between January 1, 2008, and December 31, 2009. The male/female sex ratio was 1.3, and the live-birth prevalence of EA was 1.8 per 10,000 births. Major characteristics were comparable to those reported in the literature. Survival was 95%, and no correlation with caseload was noted. CONCLUSIONS Epidemiologic surveys of congenital anomalies such as EA, which is a rare disease, provide valuable data for public health authorities and fulfill one important mission of reference centers. When compared with previous epidemiological data, this national population-based registry suggests that the incidence of EA remains stable.

Tracheobronchial ruptures from blunt thoracic trauma in children

BACKGROUND/PURPOSE Tracheobronchial ruptures in blunt thoracic trauma in children are rare. The aim of this study was to suggest the means of an early diagnosis and a conservative management as often as possible. METHODS Sixteen cases of tracheobronchial ruptures by blunt thoracic trauma were observed over 26 years in 9 regional pediatric centers. RESULTS There were 12 boys and 4 girls, from ages 1 hour to 17 years. Nine children presented with associated lesions. Fibroscopy established the following diagnosis: 8 tracheal wounds and 8 bronchial wounds. Six children were operated on within 18 hours (on average) after installation of a thoracic drainage. Two lobectomies, 3 ideal tracheal sutures, and 1 bronchial suture were performed. Seven children were treated exclusively by thoracic drainage. Two of them were intubated through the lesion, leading to a transitory endoprothesis accompanied or not by an external thoracic drainage. One infant recovered spontaneously. There were no deaths in this series. Two recurrent postoperative nerve injuries were noted, one of which was a transitory spontaneously resolutive scar bud and one a granuloma treated by laser. Three times, a stenosis occurred after a conservative management. Two were operated on. CONCLUSIONS Tracheobronchial ruptures in children are rare. An early fibroscopy holds an important place in the approach of this pathology. Treatment is variable, based on thoracic lesions, their tolerance by the child, and associated lesions. Surgery is not the only therapy because conservative treatment by simple thoracic drainage or lesion intubation has proved effective.

Does prenatal diagnosis modify neonatal treatment and early outcome of children with esophageal atresia

OBJECTIVE Our study aimed at (1) evaluating neonatal treatment and outcome of neonates with either a prenatal or a postnatal diagnosis of esophageal atresia (EA) and (2) analyzing the impact of prenatal diagnosis on outcome based on the type of EA. STUDY DESIGN We conducted a population-based study using data from the French National Register for infants with EA born from 2008-2010. We compared prenatal, maternal, and neonatal characteristics among children with prenatal vs postnatal diagnosis and EA types I and III. We defined a composite variable of morbidity (anastomotic esophageal leaks, recurrent fistula, stenosis) and death at 1 year. RESULTS Four hundred sixty-nine live births with EA were recorded with a prenatal diagnosis rate of 24.3%; 82.2% of EA type I were diagnosed prenatally compared with 17.9% of EA type III (P < .001). Transfer after birth was lower in case of prenatal diagnosis (25.6% vs 82.5%; P < .001). The delay between birth and first intervention did not differ significantly among groups. The defect size was longer among the prenatal diagnosis group (2.61 vs 1.48 cm; P < .001). The composite variables were higher in prenatal diagnosis subset (44% vs 27.6%; P = .003) and in EA type I than in type III (58.1% vs 28.3%; P < .001). CONCLUSION Despite the excellent survival rate of EA, cases with antenatal detection have a higher morbidity rate related to the EA type (type I and/or long gap). Even though it does not modify neonatal treatment and the 1-year outcome, prenatal diagnosis allows antenatal parental counselling and avoids postnatal transfers.

CL041 - Registre National de l’Atrésie de l’Œsophage : Résultats année 2008

Introduction l’incidence de l’atresie de l’œsophage (AO) est mal connue et varie entre 1/2500 a 1/4600 naissances. Le but de ce travail est de presenter les donnees 2008 du registre national de l’AO (metropole plus DOM TOM). Methodes des fiches d’inclusion ont ete remplies par tous les centres prenant en charge une AO et saisies et analysees par le centre de reference. Les DIM de chaque centre ont ete contactes ainsi que 4 registres regionaux de malformations pour augmenter le nombre et l’exhaustivite des sources. Resultats 142 nouveaux cas ont ete declares pour l’annee 2008 (incidence = 1/5824 naissances). Il existait une predominance de garcons ( n = 83), le poids de naissance moyen etait de 2527 g avec un terme de 37 SA. Un diagnostic antenatal etait possible dans 17 % des cas. Il y avait 15 atresies de type I et 123 de type III. Le diagnostic etait pose dans les 24h dans 89 % des cas et la chirurgie initiale realisee avant 48h dans 94 % pour le type III. Le taux des malformations associees etait de 53 % et la mortalite globale de 5 %. Conclusions Ces premiers resultats montrent une incidence de l’AO similaire a celle observee par le registre Europeen des malformations. Le diagnostic antenatal reste rare mais la prise en charge dans notre pays est precoce et la mortalite faible.