Frederic Lavrand

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.

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.

Combined Nuss Procedure and Cardiac Procedure Through a Left Anterolateral Thoracotomy

Aortic root aneurysms associated with severe pectus excavatum caused by Marfans syndrome can be challenging to correct in children. Recent reports suggest single-stage correction of both cardiac and chest wall deformities through a median sternotomy. We report a 9-year-old girl with Marfans syndrome, an ascending aorta aneurysm, mitral regurgitation, and pectus excavatum, who underwent Davids procedure with mitral valve repair and the Nuss procedure to correct the thoracic deformity. This single-stage combined procedure was performed through a left anterolateral thoracotomy. This surgical approach offers easy access to the heart and aorta, has good cosmetic results, and avoids a midline skin incision.

One-Year Outcome for Congenital Diaphragmatic Hernia: Results From the French National Register

Objective To evaluate the status of congenital diaphragmatic hernia (CDH) management in France and to assess predictors of adverse outcomes. Study design We reviewed the first‐year outcome of all cases of CDH reported to the French National Register in 2011. Results A total of 158 cases were included. Of these, 83% (131) were prenatally diagnosed, with a mortality rate of 39% (44 of 112) for live born infants with a known outcome at hospital discharge. Mortality increased to 47% (60 of 128) including those with termination of pregnancy and fetal loss. This contrasts with the 7% (2 of 27) mortality rate of the patients diagnosed postnatally (P = .002). Mortality worsened with 1 prenatal marker of CDH severity (OR 3.38 [1.30‐8.83] P = .013) and worsened further with 2 markers (OR 20.64 [5.29‐80.62] P < .001). Classic postnatal risk factors of mortality such as side of hernia (nonleft P = .001), prematurity (P < .001), low birth weight (P = .002), and size of the defect (P < .001) were confirmed. Of the 141 live births (114 prenatal and 27 postnatal diagnosis) with known outcomes, 93 (67%) survived to hospital discharge, 68 (60%) with a prenatal diagnosis and 25 (93%) with a postnatal diagnosis. The median time to hospital discharge was 34 days (IQR, 19.25‐62). Of these survivors, 71 (76%) were followed up for 1 year. Conclusions Despite advances in management of CDH, mortality was high and associated with prenatal risk factors. Postnatally, severe persistent pulmonary hypertension was difficult to predict and presented persistent challenges in management.

Upper Sternal Cleft With a Complex Congenital Heart Defect: Repair in a Single Stage.

Sternal clefts are extremely rare in association with complex congenital heart defects, and their management can be challenging. Complete surgical repair in early infancy, of both anomalies in a single stage, is advocated. Different surgical techniques exist for isolated sternal cleft repair, but they do not consider the difficulties of combining sternal defect closure and a cardiac operation with the unavoidable postoperative cardiac edema. We describe a successful surgical technique to repair a sternal cleft in a 3-month-old infant in a concomitant cardiac operation using an absorbable polyglactin 910 mesh plate and sternal osseous autografting to avoid postoperative cardiac distention.

Séquelles des chirurgies thoraco-abdomino-pelviennes réalisées pour tumeurs chez l’enfant

Thoracic surgery for pediatric tumors may induce a large variety of sequelae that mainly concern skin, muscles and bones (ribs), thoracic nerve and spinal cord and respiratory system. Muscle-sparing thoracotomy, intrathoracic expanders inserted after right pneumonectomy, early orthopedic evaluation and follow-up are useful preventive proceedings able to decrease postoperative sequelae. Surgery for abdominal tumors in children is part of a sequence where each therapeutic treatment can induce its own sequelae possibly potentiated by other therapies. Scars and occlusions represent classic effects, others like diarrhea, intestinal ischemia may require specific and sometimes partially effective long-term treatment. The pelvic cavity is characterized by an anatomy dedicated to urinary and digestive continence and reproductive functions. The oncologic resection of tumors developed in this region exposed to significant risk of sequelae that may strongly affect the quality of life. The development of conservative approaches for local treatment and of fertility preservation techniques has significantly reduced this morbidity and must be known by the surgeon to optimize the therapeutic strategy.

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.