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Background: Advances in surgical and neonatal care have led to improved survival of patients with œsophageal atresia (OA) over time. Morbidity remains significant, with one-third of patients being affected by a postoperative complication. Several aspects of management are not consensual, such as the use of œsophagogram before starting oral feeding., Methods: We conducted a multicenter retrospective study, including all children with OA that underwent a primary anastomosis in the first days of life, between 2012 and 2018 in five French centers, to determine the usefulness of postoperative œsophagogram during the 10 days after early primary repair of OA to diagnose the anastomotic leak and congenital œsophageal stenosis., Results: Among 225 included children, 90 (40%) had a routine œsophagogram and 25 (11%) had an anastomotic leak, clinically diagnosed before the scheduled œsophagogram in 24/25 (96%) children at median postoperative day 4. Ten patients had associated congenital œsophageal stenosis diagnosed on the œsophagogram in only 30% of cases., Conclusion: Early œsophagogram is rarely useful in the diagnosis of an anastomotic leak, which is clinically diagnosed before performing an œsophagogram in the majority of cases. The need for a postoperative œsophagogram should be evaluated on a case-by-case basis., Impact: Early œsophagogram is not helpful in the diagnosis of an anastomotic leak in the majority of cases. An anastomotic leak is most often diagnosed clinically before performing an œsophagogram. Early postoperative œsophagogram could be helpful for the diagnosis of congenital œsophageal stenosis. However, dysphagia occurs later and early diagnosis of congenital œsophageal stenosis has no impact on the management and outcome of asymptomatic children. Indication of postoperative œsophagogram has to be evaluated on a case-by-case basis., (© 2023. The Author(s), under exclusive licence to the International Pediatric Research Foundation, Inc.)

Objective: Despite recent progress in caring for patients born with esophageal atresia (EA), undernutrition and stunting remain common. Our study objective was to assess nutritional status in the first year after birth with EA and to identify factors associated with growth failure., Study Design: We conducted a population-based study of all infants born in France with EA between 2010 and 2016. Through the national EA register, we collected prenatal to 1 year follow-up data. We used body mass index and length-for-age ratio Z scores to define patients who were undernourished and stunted, respectively. Factors with P < 0.20 in univariate analyses were retained in a logistic regression model., Results: Among 1,154 patients born with EA, body mass index and length-for-age ratio Z scores at 1 year were available for about 61%. Among these, 15.2% were undernourished and 19% were stunted at the age of 1 year. There was no significant catch-up between ages 6 months and 1 year. Patients born preterm (41%), small for gestational age (17%), or with associated abnormalities (55%) were at higher risk of undernutrition and stunting at age 1 year ( P < 0.05). Neither EA type nor surgical treatment was associated with growth failure., Conclusion: Undernutrition and stunting are common during the first year after birth in patients born with EA. These outcomes are significantly influenced by early factors, regardless of EA type or surgical management. Identifying high-risk patient groups with EA (i.e., those born preterm, small for gestational age, and/or with associated abnormalities) may guide early nutritional support strategies., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential of interest., (Copyright © 2022 Depoortere, Lapillonne, Sfeir, Bonnard, Gelas, Panait, Rabattu, Guignot, Lamireau, Irtan, Habonimana, Breton, Fouquet, Allal, Elbaz, Talon, Ranke, Abely, Michel, Lirussi Borgnon, Buisson, Schmitt, Lardy, Petit, Chaussy, Borderon, Levard, Cremillieux, Tolg, Breaud, Jaby, Grossos, De Vries, Arnould, Pelatan, Geiss, Laplace, Kyheng, Nicolas, Aumar and Gottrand.)

Background and Objectives: Respiratory diseases are common in children with esophageal atresia (EA), leading to increased morbidity and mortality in the first year. The primary study objective was to identify the factors associated with readmissions for respiratory causes in the first year in EA children., Methods: A population-based study. We included all children born between 2008 and 2016 with available data and analyzed factors at birth and 1 year follow-up. Factors with a P value <.10 in univariate analyses were retained in logistic regression models., Results: Among 1460 patients born with EA, 97 (7%) were deceased before the age of 1 year, and follow-up data were available for 1287 patients, who constituted our study population. EAs were Ladd classification type III or IV in 89%, preterm birth was observed in 38%, and associated malformations were observed in 52%. Collectively, 61% were readmitted after initial discharge in the first year, 31% for a respiratory cause. Among these, respiratory infections occurred in 64%, and 35% received a respiratory treatment. In logistic regression models, factors associated with readmission for a respiratory cause were recurrence of tracheoesophageal fistula, aortopexy, antireflux surgery, and tube feeding; factors associated with respiratory treatment were male sex and laryngeal cleft., Conclusions: Respiratory morbidity in the first year after EA repair is frequent, accounting for >50% of readmissions. Identifying high risk groups of EA patients (ie, those with chronic aspiration, anomalies of the respiratory tract, and need for tube feeding) may guide follow-up strategies., Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2021 by the American Academy of Pediatrics.)

Objective: To identify the risk factors for early mortality and morbidity in a population with distal esophageal atresia (EA)-tracheoesophageal fistula., Study Design: Cohort study from a national register. Main outcomes and measures included early mortality, hospital length of stay (LoS), need for nutritional support at 1 year of age as a proxy measure of morbidity, and complications during the first year of life., Results: In total, 1008 patients with a lower esophageal fistula were included from January 1, 2008, to December 31, 2014. The survival rate at 3 months was 94.9%. The cumulative hospital LoS was 31.0 (17.0-64.0) days. Multivariate analysis showed that intrahospital mortality at 3 months was associated with low birth weight (OR 0.52, 95% CI [0.38-0.72], P < .001), associated cardiac abnormalities (OR 6.09 [1.96-18.89], P = .002), and prenatal diagnosis (OR 2.96 [1.08-8.08], P = .034). LoS was associated with low birth weight (-0.225 ± 0.035, P < .001), associated malformations (0.082 ± 0.118, P < .001), surgical difficulties (0.270 ± 0.107, P < .001), and complications (0.535 ± 0.099, P < .001) during the first year of life. Predictive factors for dependency on nutrition support at 1 year of age were complications before 1 year (OR 3.28 [1.23-8.76], P < .02) and initial hospital LoS (OR 1.96 [1.15-3.33], P < .01)., Conclusions: EA has a low rate of early mortality, but morbidity is high during the first year of life. Identifying factors associated with morbidity may help to improve neonatal care of this population., (Copyright © 2021 Elsevier Inc. All rights reserved.)

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., (Copyright © 2019 Elsevier Inc. All rights reserved.)

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., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objectives: The objective of the study was to assess the efficacy of reduced sufentanil doses for postoperative analgesia following surgical ductal closure in extremely premature infants., Methods: This was a retrospective, single-center, cohort study comparing 2 sufentanil dosing regimens used between 2001 and 2010 and included all infants born at <28 weeks of gestation with surgical ductal closure. Sufentanil doses were reduced in 2007 as a standard of care. Time was divided into 3 epochs to distinguish the effects of practice changes over time from the effects of sufentanil dose change: epoch 1 (2001 to 2004), epoch 2 (May 2005 to 2007), and epoch 3 (June 2007 to 2010)., Results: A total of 109 of 114 eligible infants were analyzed (mean [±SD], gestational age: 25.1 [±1.1] wk; mean [±SD], birth weight: 756 [±144] g). Median sufentanil doses were significantly higher during epochs 1 and 2 (0.1 to 0.2 µg/kg/h) than during epoch 3 (0.03 to 0.04 µg/kg/h) (P<0.0001). EDIN (Echelle de Douleur et d'Inconfort du Nouveau-né) pain scores were mostly ≤4 throughout the study period and their changes over time were not contemporaneous with the reduction in sufentanil doses; they were lower during epoch 1 versus epochs 2 and 3 (P<0.0001) and comparable between epochs 2 and 3. Midazolam doses and paracetamol use were not higher during epoch 3 as compared with epochs 1 and 2. No difference in opioid-related adverse events was observed between the 3 epochs., Conclusion: Our study supports the use of low continuous intravenous sufentanil doses, consistent with morphine doses currently recommended in this population.

Purpose: Thoracoscopic lung resection for congenital pulmonary airway malformation (CPAM) is a safe technique for children. Our purpose was to evaluate the feasibility of a fast-track protocol in such cases., Methods: From September 2007 to May 2016, 101 patients underwent a thoracoscopic pulmonary resection of which 83 for CPAM (lobectomy, wedge resection or sequestrectomy). We retrospectively reviewed the characteristics of surgical procedure, postoperative management and complications through three time periods (September 2007-December 2009: n=14, January 2010-March 2013: n=30, April 2013-May 2016: n=39) corresponding to management protocols modifications introducing fast-track pathways., Results: Through the 3 time periods, median postoperative hospital stay decreases (4, 3, 2days successively, P=0.02). In the third time period, 4 patients underwent surgery in day-case surgery. The overall and surgical complication rates, mainly related to air leakage, remain stable through the 3 time periods (14%, P=0.41 and 10%, P=0.52 respectively). Among the 13 patients without postoperative pleural drainage, one required secondary drainage after a partial resection of an emphysema., Conclusion: Fast-track protocol for children undergoing uncomplicated thoracic surgery for CPAM seems feasible without extra morbidity. Selected patient undergoing thoracoscopic resection of the lung may benefit from the absence of pleural drainage and can be operated on in day-case surgery., Level of Evidence: Level III., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objective: Evaluate neonatal management and outcome of neonates with either a prenatal or a post-natal diagnosis of EA type III., Study Design: Population-based study using data from the French National Register for EA from 2008 to 2010. We compared children with prenatal versus post-natal diagnosis in regards to prenatal, maternal and neonatal characteristics. We define a composite variable of morbidity (anastomotic esophageal leaks, recurrent fistula, stenosis) and mortality at 1 year., Results: Four hundred and eight live births with EA type III were recorded with a prenatal diagnosis rate of 18.1%. Transfer after birth was lower in prenatal subset (32.4% versus 81.5%, P<0.001). Delay between birth and first intervention was not significantly different. Defect size (2cm vs 1.4cm, P<0.001), gastrostomy (21.6% versus 8.7%, P<0.001) and length in neonatal unit care were higher in prenatal subset (47.9 days versus 33.6 days, P<0.001). The composite variables were higher in prenatal diagnosis subset (38.7% vs 26.1%, P=0.044)., Conclusion: Despite the excellent survival rate of EA, cases with antenatal detection have a higher morbidity related to the EA type (longer gap). Even if it does not modify neonatal management and 1-year outcome, prenatal diagnosis allows antenatal parental counseling and avoids post-natal transfer., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Background: The aim of the present national prospective population-based study was to assess the early morbidity of esophageal atresia (EA)., Methods: All 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., Results: From 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 164 days (range, 33-398 days), and 1% underwent an aortopexy for severe tracheomalacia. The weight/age Z-score was -0.8 (range, -5.5 to 3.7 months) at 12 months. Fifteen percent of patients were undernourished at 12 months 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 (p = .0009) and presence of a gastrostomy (p = .0002); exclusive oral feeding at discharge was associated with a decreased risk of complications (p = .007)., Conclusions: Digestive and respiratory morbidities remain frequent during the first year of life and are associated with difficult anastomosis and lack of full oral feeding.

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., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Purpose: The effectiveness of laparoscopic diagnosis of contralateral patent processus vaginalis (CPPV) in children with unilateral inguinal hernia was evaluated., Methods: Ninety-three consecutive children under the age of 1 year were operated on for a unilateral inguinal hernia. A contralateral CPPV was diagnosed by laparoscopy via the inguinal hernia sac before ligation. The laparoscopy results of this technique were correlated with those of herniography or inguinal exploration., Results: Laparoscopy was performed on 88 patients; sensitivity was 71% and specificity 89%. The only complication arising from the procedure was wound infection in two patients., Conclusion: This method is a simple, safe, and accurate procedure for selecting children for contralateral surgical exploration.

Study Aim: The aim of this prospective study was to evaluate the laparoscopic diagnosis of contralateral patent processus vaginalis in children with unilateral inguinal hernia., Patients and Method: Between November 1995 and February 1998, 91 consecutive children (78 boys, 13 girls) under the age of 1 year were operated on for a unilateral inguinal hernia. A contralateral hernia was diagnosed by a laparoscopy through the inguinal hernia sac before ligation. Results of this technique were correlated with those of herniography (79 cases) or with inguinal exploration (12 emergency)., Results: Laparoscopy was performed in 88 patients. Laparoscopy was impossible in three cases: one inguinal sac too thin, two cases of ectopic testis in the inguinal canal. Sensitivity was 73% and specificity 92%. The only complication arising from the procedure was wound infection in two patients., Conclusion: This method is a simple, safe and accurate procedure in order to select children for contralateral surgical exploration.

Background: Thirty children operated on for Crohn's disease (CD) were reviewed (1975-1994). The aim of the study was to assess their postoperative outcome., Patients: 19 boys and 11 girls, aged 15.3 (2) years (range 11.3-20) at surgery were studied., Results: Surgical indications were acute complications of CD and chronic intestinal illness. Six months after surgery, 11 of 12 patients had been weaned off steroids, and 22 of 23 patients were weaned off nutritional support; 17 patients without recurrence had a mean (SD) weight gain of 2.1 (8) kg and a height gain of 3.36 (3) cm. During 3.1 (2.7) years follow up, 12 patients (40%) had a recurrence of the disease after 19.4 (14) months (means (SD)): supra-anastomotic recurrence (six), severe perianal disease (two), and chronic illness (four). Six of 14 patients who were treated with mesalazine (13) or azathioprine (one) had recurrences. The postoperative recurrence rate was 50% at two years., Conclusion: Surgical treatment modifies the immediate outcome of severe or complicated CD, but does not prevent recurrence, despite localised resection or prophylactic postoperative treatment. Extension of the disease before surgery seems to be a major risk factor for postoperative recurrence in children.

The Duhamel abdominoperineal pull-through is the authors' preferred treatment for children with Hirschsprung's disease (HD). Advances in instrumentation and technique now make laparoscopic correction possible. This procedure was successfully performed in six children aged 5 weeks to 6 years. No colostomy was performed before or after the Duhamel procedure. The technique and its potential role in the treatment of HD are discussed.

Between 1989 and 1997, 23 children with primary obstructive megaureter underwent surgical repair, routinely using a vesico-psoas hitch ureteroneocystostomy. Ten of them had a antenatal diagnosis. Mean postoperative follow-up was 3 years. No postoperative obstruction and no vesicoureteric reflux occurred. Assessment of renal function on the operated side showed improvement in 7 cases and stabilization in all other cases. No bladder dysfunction was observed. Vesicopsoas hitch is an advantageous procedure in this indication. It is almost always unnecessary to perform ureteral tailoring and its specific complications can be avoided. Psoas hitch is easily performed in infants and young children and this technique is particularly appropriate in the subgroup of patients with antenatal diagnosis.

From 1979 to 1990 37 children with sickle cell disease underwent surgery for cholelithiasis. Mean age was 11,3 years. Twenty-four children had a history of recurrent abdominal pain; 8 of them were admitted for an acute gallstone complication. In 9 cases the stones were detected by systematic echography. The incidence of gallstones increasing with age, the rate of complications, the frequent resolution of recurrent abdominal pain after cholecystectomy justify elective surgery at the time of diagnostic of the stones. A transfusion preparation considerably reduces the risk of post-operative complications.