Arnaud Bonnard

Esophageal atresia: Data from a national cohort

PURPOSEnA 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.nnnMETHODSnAll 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.nnnRESULTSnThree 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.nnnCONCLUSIONSnEpidemiologic 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.

Indications for laparoscopy in the management of intussusception: A multicenter retrospective study conducted by the French Study Group for Pediatric Laparoscopy (GECI)

INTRODUCTIONnSurgical management of intussusception is required for cases where enema reduction fails. Some articles report an advantage of the laparoscopic over the open approach, but complications such as serosal tearing and frank perforation have been described. We aim to determine the best indication of laparoscopy in the decisional management tree of intussusception.nnnPATIENT AND METHODnThis is a retrospective chart review of all children with intussusception who failed hydrostatic enema reduction and who underwent immediate laparoscopic management. Cases were compiled from 7 pediatric surgical centers (French Study Group of Pediatric Laparoscopy) between 1992 and 2005. Data collected included age, duration of symptoms, findings on initial assessment, level of intussusceptum after attempted hydrostatic enema reduction, type of laparoscopic approach, operative time, conversion to open surgery, etiology, and postoperative complications. Two groups were analyzed-conversion to open surgery or not.nnnRESULTSnSixty-nine patients (48 males and 21 females) were reviewed. In total, 22 patients required a conversion to open surgery (31.9%). Eleven of these were converted because of failure of laparoscopic reduction. The risk for conversion to open surgery is directly linked to the length of time between onset of symptoms and diagnosis (1.6 vs 3.1 days for conversion group, P = .048), the presence of signs of peritonitis on the initial clinical assessment (10.6% vs 41% in conversion group, P = .003), and the presence or absence of a pathologic lead point (17% vs 50% in conversion group, P = .004).nnnCONCLUSIONnThe child seen early after the onset of symptoms (<1.5 days) with no signs of peritonitis is the best candidate for a laparoscopic approach in management of intussusception requiring surgery. Particular attention must be paid to not miss a pathologic lead point, especially as most tactile cues are lost.

Results from the French National Esophageal Atresia register: one-year outcome

BackgroundThe aim of the present national prospective population-based study was to assess the early morbidity of esophageal atresia (EA).MethodsAll 38 multidisciplinary French centers that care for patients with EA returned a specific questionnaire about the 1-year outcome for each patient. This information was centralized, checked, and entered into a database.ResultsFrom the total population of 307 EA patients born in 2008 and 2009, data about the 1-year outcome were obtained from 301 (98%) patients, of whom 4% were lost to follow-up and 5% died. Medical complications occurred in 34% of the patients: anastomotic leaks (8%), recurrent tracheoesophageal fistula (4%), and anastomotic stenosis (22%); all of the latter group needed dilation (median, 2 dilations/patient). A new hospitalization was required for 59% of patients (2.5 hospitalizations/patient) for digestive (52%) or respiratory (48%) reasons. Twelve percent of patients required antireflux surgery at a median age of 164xa0days (range, 33–398 days), and 1% underwent an aortopexy for severe tracheomalacia. The weight/age Z-score was −0.8 (range, −5.5 to 3.7xa0months) at 12xa0months. Fifteen percent of patients were undernourished at 12xa0months of age, whereas 37% presented with respiratory symptoms and 15% had dysphagia at the last follow-up. Significant independent factors associated with medical complications were anastomotic esophageal tension (pu2009=u2009.0009) and presence of a gastrostomy (pu2009=u2009.0002); exclusive oral feeding at discharge was associated with a decreased risk of complications (pu2009=u2009.007).ConclusionsDigestive and respiratory morbidities remain frequent during the first year of life and are associated with difficult anastomosis and lack of full oral feeding.

Non-operative management of gastroschisis: a case-matched study

Non-operative management of gastroschisis also known as plastic closure (PC) has been described as an alternative to conventional primary operative closure (POC) or staged silo closure (SSC). The aim of this study was to compare these techniques in neonates with gastroschisis. A retrospective review of neonates with gastroschisis who underwent PC was undertaken. The minimum follow-up was 1xa0year. Premature neonates (<35xa0weeks) and those with intestinal atresia or multiple congenital anomalies were excluded. Frequency matching of PC cases with two control groups with either POC or SSC was performed on 1:1 ratio, based on gestational age and birth weight. Statistical analysis using univariate analysis was performed. Three groups were assembled: PC, POC and SSC (nxa0=xa033). Median follow-up was 1,198xa0days. Groups were comparable with regard to: time to first feed, time to full enteral feeding, frequency of vascular access related infections, ventilation time, NICU LOS (length of stay) and hospital LOS. There was no difference observed in surgical complications. Almost all neonates in the PC group developed an umbilical hernia (83.8%). Umbilical hernias were highly associated with PC compared to the other two groups Pxa0=xa00.001. To date only one patient has had to have an operative repair of the umbilical hernia in the PC group. Plastic closure is safe and comparable to conventional closure techniques. However, PC is associated with the development of more umbilical hernias. The natural history of these hernias remains to be defined.

Characteristics and management of congenital esophageal stenosis: findings from a multicenter study.

BackgroundCongenital esophageal stenosis (CES) is a rare condition frequently associated with esophageal atresia (EA). There are limited data from small series about the presentation, treatment, and outcomes of CES.MethodsMedical records of all patients with CES included in the French Network on Esophageal Malformations and Congenital Diseases were reviewed retrospectively with regard to diagnosis, treatment, and outcome.ResultsOver 18xa0years, 61 patients (30 boys) had CES, and 29 (47%) of these patients also had EA. The mean age at diagnosis was 24xa0months (1xa0day to 14xa0years) and was younger in patients with CES and EA than in those with isolated CES (7 vs. 126xa0months, pu2009<u20090.05). Twenty-one of the 61 patients with CES had no clinical symptoms: in three patients, the findings were incidental, and in 18 of the 29 patients with associated EA, CES was diagnosed at the time of surgical repair of EA or during a postoperative systematic esophageal barium study. In the 40 other patients, at diagnosis, 50% presented with dysphasia, 40% with vomiting, 50% with food impaction, and 42% with respiratory symptoms. Diagnosis of CES was confirmed by esophageal barium study (56/61) and/or esophageal endoscopy (50/61). Sixteen patients had tracheobronchial remnants (TBR), 40 had fibromuscular stenosis (FMS), and five had membrane stenosis (MS). Thirty-four patients (56%) were treated by dilation only (13/34 remained asymptomatic at follow-up); 15 patients were treated by dilation but required later surgery because of failure (4/15 remained asymptomatic at follow-up); and nine patients had a primary surgical intervention (4/9 were asymptomatic at follow-up). Dilation was complicated by esophageal perforation in two patients (3.4%). At follow-up, dysphagia remained in 36% (21/58) of patients, but the incidence did not differ between the EA and the isolated CS groups (10/29 vs. 7/32, pu2009=u20090.27).ConclusionsCS diagnosis can be delayed when associated with EA. Dilation may be effective for treating patients with FMS and MS, but surgical repair is often required for those with TBR. Our results show clearly that, regardless of the therapeutic option, dysphagia occurs frequently, and patients with CES should be followed over the long term.

CARD15/NOD2 Is Not a Predisposing Factor for Necrotizing Enterocolitis

Multiple factors are incriminated in the etiopathogeny of necrotizing enterocolitis (NEC) in premature infants, including oral feeding, vascular abnormalities, increase in pro-inflammatory cytokines, and inappropriate response of the intestinal barrier to bacterial microflora. CARD15/NOD2 is a gene recently recognized as important in the innate response to gut flora and is involved in Crohns disease susceptibility. We thus tested its putative role in NEC. Ten children (seven boys and three girls) suffering from NEC who were admitted to Robert Debré hospital between 1999 and 2002 were retrospectively included in the study. Genetic screening of the 11 constant exons and the exon-intron junctions of CARD15/NOD2 by direct sequencing revealed no novel mutations of that gene in NEC patients. Furthermore, the three main mutations of CARD15/NOD2 (R702W, G908R, and 1007fs) associated with susceptibility to Crohns disease were not found in these patients. Our results suggest that CARD15/NOD2 does not play a major role in genetic susceptibility to NEC.

The use of the score for neonatal acute physiology-perinatal extension (SNAPPE II) in perforated necrotizing enterocolitis : could it guide therapy in newborns less than 1500 g?

BACKGROUNDnOnly a handful of clinical parameters other than body weight are used in managing LBW newborns with perforated necrotizing enterocolitis (NEC). Here, we determined clinical use of score for neonatal acute physiology-perinatal extension (SNAPPE II) score in the surgical decision, peritoneal drain (PD) vs PD + laparotomy in low birth weight (LBW) newborns with perforated NEC.nnnPATIENTS AND METHODnA retrospective study of all neonates weighing less than 1500 g with the diagnosis of perforated NEC between 2000 and 2006 was performed. Patients were categorized in 2 groups--PD alone vs PD + laparotomy. The SNAPPE score was calculated at various days of clinical evolution. The primary outcome of mortality was used, and comparisons using univariate and multivariate analyses were performed.nnnRESULTSnOf 39 patients identified, 20 were treated with PD alone, whereas 19 had PD and laparotomy. The mean gestational age (25.6 vs 26.6 weeks) and the mean birth weight (795 vs 910 g) were comparable (P > .05). There were no differences between PD group and LAP group with regard to SNAPPE scores calculated on the day of admission (P = .057), the day before the drain insertion (P = .167) and the day after the drain insertion (P = .66). When considering survival as the dependent variable while controlling for the treatment assignment, the modified SNAPPE score after PD drain insertion in group PD was significantly higher than in the PD + laparotomy group (21.4 vs 9.47; P = .009).nnnCONCLUSIONnThe modified SNAPPE score is a good predictor of mortality after the PD insertion. A post-PD insertion, high SNAPPE value was correlated with increased mortality regardless of an additional laparotomy. External validation of the modified SNAPPE score in a large patient population is required before its use in guiding treatment decisions.

Congenital diaphragmatic hernia: an evaluation of risk factors for failure of thoracoscopic primary repair in neonates

PURPOSEnPublications aiming to prove the feasibility and safety of thoracoscopic CDH-repair in neonates grow in numbers. Some teams use selection criteria, but none have proven statistical evidence. The aim of this study is to detect risk factors for failure of thoracoscopic primary closure of CDH in neonates.nnnMETHODSnIn 8 centers performing minimal access surgery (MAS), complete prenatal, postnatal, and operative data were evaluated for a retrospective study concerning patients with thoracoscopic congenital diaphragmatic hernia (CDH) repair. Most of the selection criteria and risk factors mentioned in the literature were analyzed. Two groups were defined: Group A - neonates who tolerated thoracoscopic primary repair, and Group B - neonates who required conversion or presented with major complications after thoracoscopic repair. Univariate and multivariate logistic regressions were used to compare these two groups.nnnRESULTSnFrom 2006 to 2010, thoracoscopy was performed in 40 neonates: Group A consisting of 28 neonates, and Group B 9 patients. Three patients were excluded because of insufficient data or major associated malformations. Significant statistical differences were found in Group B for postnatal PaCO2 >60 mmHg, need of iNO during postnatal stabilization, intrathoracic position of the stomach, pulmonary hypertension signs on the postnatal cardiac ultrasound, and preoperative OI >3.0. On multivariate analysis, only an OI >3.0 was significantly associated with conversion or major post-operative complication of thoracoscopic primary repair.nnnCONCLUSIONnCDH can be safely repaired in the neonatal period by thoracoscopy. The limiting factor for thoracoscopic CDH repair is PPHN. The best preoperative indicator for PPHN is OI. Prospective studies are nonetheless necessary to prove the effectiveness of using these risk factors as selection criteria to help design surgical management protocols for neonates presenting CDH.

Thoracoscopic repair of neonatal congenital diaphragmatic hernia (CDH): outcomes after a systematic quality improvement process.

BACKGROUNDnHigher recurrence rates have been reported for thoracoscopic repair (TR) of neonatal congenital diaphragmatic hernia (CDH) compared to open repair. Our centre initiated changes in surgical management following a quality review in order to improve outcome.nnnMETHODSnA retrospective review of TR patients from 2000 to 2011 at a single institution was performed. A review was done in 2007, and changes were implemented to decrease recurrence rates. These included use of pledgets, an extracorporeal corner stitch, liberal prosthetic patch use, lower insufflation pressures, and TR was limited to two experienced surgeons. Outcome data before and after this quality improvement process were compared. Non-TR patients from the same time period served as controls. Data are quoted as median (range) and non-parametric tests used to compare. P<0.05 was regarded as significant.nnnRESULTSnThere were 23 neonatal TR patients with median follow-up of 3.6 (range 0.4-7) years. Median age at repair was 2 (range 0-21) days. There were 5 patch repairs (22%), all after 2008. There were 9 recurrences (39%) at a median time of 162 days after TR, compared to 13 (10%) recurrences in the control cohort. For primary TR, there was a trend towards a decreased recurrence rate from 50% prior to 2008 to 25% after 2008 (P=0.26).nnnCONCLUSIONSnSystematic quality review was modestly effective in decreasing the recurrence rate for neonatal TR, but further outcome data are required.

Thoracoscopy in the management of congenital lung diseases in infancy

BackgroundThis study aimed to compare the results of thoracoscopic surgery for congenital lung diseases between infants younger than 6xa0months and those older than 6xa0months at the time of surgery in terms of operation duration, surgical complications, chest tube duration, and hospital stay.MethodsThe charts of 30 thoracoscopic resections for congenital lung diseases were retrospectively reviewed. This study compared 17 children younger than 6xa0months (mean, 3.94xa0months; range, 0.37–5.7xa0months; group 1) with 13 children older than 6xa0months (mean, 12.05xa0months; range, 6.2–24.63xa0months; group 2) at the time of surgery. The median follow-up period was 9xa0months (range, 1–41xa0months).ResultsLobectomy was performed in 27 cases, bilobectomy in 1 case, and nonanatomic excision in 2 cases. The mean operating time for group 1 (176xa0±xa054xa0min) was similar to that for group 2 (160xa0±xa046xa0min). The difference is not significant. The mean duration of chest tube drainage was similar in the two groups (4.4xa0days; range, 1–9xa0days for group 1 vs. 4.1xa0days; range, 3–8xa0days for group 2). The complications included 1 major and 10 minor complications, with no statistically significant difference between the two groups. Three surgical procedures in each group were converted. The hospital stay was not statistically different between the two groups (8xa0days; range, 3–20xa0days for group 1 vs. 6xa0days; range, 4–10xa0days for group 2).ConclusionsThe study findings showed no statistically significant difference between the two groups in terms of operation time, complication rate, conversion rate, or hospital stay. Lobectomy can be safely and successfully performed by thoracoscopy even for children younger than 6xa0months.