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Introduction: Oesophageal atresia (EA) is a life-threatening congenital oesophageal deformity that causes considerable newborn morbidity and death. Many prognostic variables have been linked to the survival of infants with EA, although the results of the studies are still conflicting. Furthermore, studies on EA effects in developing countries still need to be included. Here, we aimed to determine the survival of children with EA and link it to prognostic variables in a particular developing country., Materials and Methods: A cross-sectional observational retrospective study was conducted using medical records of paediatric patients with EA at our institution from January 2014 to December 2020., Results: A total of 53 children with EA were included in the study. Log-rank analysis showed that definitive surgery and thrombocytopenia were significantly associated with the survival of children with EA, with a p-value of 0.007 and 0.002, respectively, whereas, sex, EA type, pneumonia and sepsis were not (p = 0.898, 0.919, 0.255, and 0.499, respectively). Multivariate analysis revealed that thrombocytopenia and definitive surgery were strongly associated with the survival of children with EA with a pvalue of 0.014 (hazard ratio (HR) = 2.67 [95% confidence interval (CI) = 1.22-5.85]) and 0.022 (HR =0.39 [95% CI = 0.17- 0.87]), respectively., Conclusion: Our study shows that thrombocytopenia might increase mortality, while definitive surgery might be beneficial for the survival of paediatric patients with EA. It implies that definitive surgery should be performed as early as necessary to prevent further morbidity and mortality. Our study comprehensively provides the survival of children with EA and links it to prognostic variables in a particular developing country. It serves as a potential research project that can be applied to the clinical setting to help clinicians manage EA better.

Introduction:  Esophageal atresia (EA) is associated with duodenal atresia (DA) in 3 to 6% of cases. The management of this association is controversial and literature is scarce on the topic., Materials and Methods:  We aimed to (1) review the patients with EA + DA treated at our institution and (2) systematically review the English literature, including case series of three or more patients., Results:  Cohort study: Five of seventy-four patients with EA had an associated DA (6.8%). Four of five cases (80%) underwent primary repair of both atresia, one of them with gastrostomy placement (25%). One of five cases (20%) had a delayed diagnosis of DA. No mortality has occurred. Systematic Review: Six of six-hundred forty-five abstract screened were included (78 patients). Twenty-four of sixty-eight (35.3%) underwent primary correction of EA + DA, and 36/68 (52.9%) underwent staged correction. Nine of thirty-six (25%) had a missed diagnosis of DA. Thirty-six of sixty-eight underwent gastrostomy placement. Complications were observed in 14/36 patients (38.9 ± 8.2%). Overall mortality reported was 41.0 ± 30.1% (32/78 patients), in particular its incidence was 41.7 ± 27.0% after a primary treatment and 37.0 ± 44.1% following a staged approach., Conclusion:  The management of associated EA and DA remains controversial. It seems that the staged or primary correction does not affect the mortality. Surgeons should not overlook DA when correcting an EA., Competing Interests: G.L. declares he received financial support for attending symposia from the Company Coloplast S.p.A. and from the Company AB Medica S.p.A. All others authors declared that they have no conflict of interest., (Thieme. All rights reserved.)

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.)

Introduction:  The data of the Turkish Esophageal Atresia Registry (TEAR) was evaluated to define the outcome of very low birth weight (VLBW) and low BW (LWB) infants with esophageal atresia (EA)., Materials and Methods:  The data registered by 24 centers between 2014 and 2018 were evaluated for demographic features, prenatal findings, associated anomalies, surgical treatment, and outcome. Patients were enrolled in three groups according to their BWs (VLBW <1,500 g), LWB = 1,500-2,500 g), and normal BW (NBW; >2,500 g)., Results:  Among the 389 cases, there were 37 patients (9.5%) in the VLBW group, 165 patients (42.4%) in the LBW group, and 187 patients (48.1%) in the NBW group. Prenatal diagnosis rates were similar among the three groups (29.7, 34.5, and 24.6%, respectively). The standard primary anastomosis was achieved at a significantly higher rate in NWB cases than in the other groups ( p  < 0.05). In patients with tracheoesophageal fistula (TEF), patients of the NBW group had significantly higher rates of full oral feedings, when compared with VLBW and LBW cases ( p  < 0.05). At the end of the first year, when we evaluate all patients, the number of cases with fistula recanalization and esophageal anastomotic strictures (AS) requiring esophageal dilatation was similar among the groups. The weight and height measurements at 6 months and 1 year of age of the survivors were similar in all the groups. The overall mortality rate was significantly higher in the VLBW and LBW groups, when compared with the NBW patients, even in patients with tension-free anastomosis ( p  < 0.05). The incidence of the associated anomalies was 90.6% in cases with mortality, which was significantly higher than in survivors (59.6%; p  < 0.05). According to Spitz's classification, the survival rate was 87.1% in class I, 55.3% in class II, and 16.7% in class III. The most common causes of mortality were associated with cardiovascular diseases, pneumonia, and sepsis., Conclusion:  The national data of TEAR demonstrates that the developmental and feeding parameters are better in NBW patients. Although VLBW patients have higher risk of developing fistula canalization than the LBW and NBW groups, long-term complications, such as anastomotic strictures, weight, and height values, after 1 year are similar in both groups. According to our results, associated anomalies and LBWs are still significant risk factors for mortality in cases with EA., Competing Interests: None declared., (Thieme. All rights reserved.)

Background and Objectives: Presence of a syndrome (or association) is predictive of poor survival in esophageal atresia (EA). However, most reports rely on historical patient outcomes, limiting their usefulness when estimating risk for neonates born today. We hypothesized improved syndromic EA survival due to advances in neonatal care., Methods: A retrospective single-center review of survival in 626 consecutive patients with EA from 1980 to 2017 was performed. Data were collected for recognized risk factors: preterm delivery; birth weight <1500 g; major cardiac disease; vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, and limb abnormalities (VACTERL); and non-VACTERL syndromes. Cox proportional hazards regression models were used to evaluate temporal trends in survival with respect to year of birth and syndromic EA., Results: Overall, 87% of 626 patients with EA survived, ranging from 82% in the 1980s to 91% in the 2010s. After adjusting for confounders, syndromic EA survival did not improve during the study, with no association found between year of birth and survival (hazard ratio [HR] 0.98, 95% confidence interval [CI]: 0.95-1.01). Aside from lethal non-VACTERL syndromes, patients with nonlethal non-VACTERL syndromes (HR 6.85, 95% CI: 3.50-13.41) and VACTERL syndrome (HR 3.02, 95% CI: 1.66-5.49) had a higher risk of death than those with nonsyndromic EA., Conclusions: Survival of patients with syndromic EA has not improved, and patients with non-VACTERL syndromes have the highest risk of death. Importantly, this is independent of syndrome lethality, birth weight, and cardiac disease. This contemporary survival assessment will enable more accurate perinatal counseling of parents of patients with syndromic EA., 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.)

Background/purpose: The Spitz classification for esophageal atresia with/without tracheoesophageal fistula (EA/TEF) predicts mortality. This study evaluates the contemporary relevance of the Spitz classification and investigates predictors of morbidity., Methods: EA/TEF patients born between 1995 and 2018 at two centers were retrospectively reviewed. Clinical variables including sex, prenatal diagnosis, birth weight, prematurity, major congenital heart disease (MCHD), and pre-operative mechanical ventilation (POMV) were collected. Index admission composite morbidity was considered positive if: length-of-stay >90th percentile (139 days), ventilation days >90th percentile (24 days), and/or gastrostomy was used for long-term feeding. Multivariable regression determined predictors of index admission mortality and composite morbidity. A composite morbidity predictive algorithm was created. ROC curves evaluated model discrimination., Results: Of 253 patients, 13 (5.1%) experienced index admission mortality. Of the patients not suffering mortality, 74 (31.6%) experienced composite morbidity. Only MCHD predicted mortality (p = 0.001); birth weight did not (p = 0.173). There was no difference between the Spitz classification and MCHD alone in predicting mortality risk (p = 0.198); both demonstrated very good discrimination. Prenatal diagnosis, POMV, prematurity, and male sex predicted composite morbidity risk (p < 0.001; p = 0.008; p = 0.009; p = 0.05). An algorithm incorporating these predictors demonstrated good discrimination (AUC = 0.784; 95% CI: 0.724, 0.844)., Conclusions: The Spitz classification maintains contemporary relevance for mortality risk, though birth weight can be de-emphasized. A new morbidity risk algorithm is proposed for early postnatal counseling., Type of Study: Prognosis study., Level of Evidence: Level IV., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Esophageal atresia is a developmental disorder in which the upper and lower esophagus fail to connect. It has an estimated prevalence of 1 in 2,500-4,500 live births and has poorer outcomes in low- and middle-income countries than in high-income countries. This study focused on the disorder's epidemiology, morbidity, and mortality in Jordan to address the lack of data regarding esophageal atresia in this country., Methods: This was a retrospective study covering a 16-year period at a tertiary care academic hospital. Data were extracted from archived medical records and operative notes. All patients who had complete congenital esophageal atresia data were included. In total, the records of 55 patients were analyzed., Results: Of the included patients, 9% were diagnosed prenatally and 47% were diagnosed with polyhydramnios. The mean gestational age was 37 weeks, the mean birthweight was 2,550 g, and 60% of patients were male. Isolated cases of esophageal atresia were reported in 58.2% of patients. There was a high rate of associated congenital anomalies (41.8%), with cardiac lesions the most common (20%), and 5.5% were syndromic. Parental consanguinity was found in 18.2% of patients. Postoperative surgical-related morbidities included stricture (18/24; 75%) and leakage (5/24; 20.8%). Fistula recurrence occurred in one patient (4.2%). The mortality rate was 12.8%., Conclusion: Esophageal atresia causes a high rate of mortality and exhibits post-operative morbidities. Moreover, associated anomalies were frequently observed. A high level of the malformation was found among offspring from consanguineous marriages., (© 2020 Japan Pediatric Society.)

Introduction: The optimal method of esophageal replacement remains controversial. The aim of this study was to evaluate 30-d outcomes of children in the National Surgical Quality Improvement Project Pediatric (NSQIP-P) database who underwent esophageal replacement from 2012 to 2018., Methods: Demographics, comorbidities, and procedural technique was identified in NSQIP-P and reviewed. Thirty-day outcomes were assessed and stratified by gastric pull-up or tube interposition versus small bowel or colonic interposition. Categorical and continuous variables were assessed by Pearson's chi-square, Fisher's exact, and Wilcoxon rank-sum tests, respectively. Multivariate logistic regression was performed to estimate the effects of procedure technique and clinical risk factors on patient outcomes., Results: Of the 99 cases of esophageal replacement included, 52 (52.5%) utilized a gastric conduit, whereas 47 (47.5%) involved small bowel/colonic esophageal interposition. Overall risk of complications was 52.5%, the most common of which were perioperative transfusion (30.3%), surgical site infection (11.1%), and sepsis (9.1%). Risk of unplanned reoperation was 17.2%, and risk of mortality was 3.0%. Risk for complications, reoperation, and readmission did not differ significantly between those who underwent gastric esophageal replacement and those who underwent small bowel or colonic interposition. Median operative time was shorter in the gastric esophageal replacement group (5.2 versus 8.1 h, P = 0.009)., Conclusions: Among children in NSQIP-P who underwent esophageal replacement from 2012 to 2018, the risk of 30-d complications, unplanned reoperation, and mortality was relatively frequent and was similar across operative techniques. Opportunities exist to improve preoperative optimization, utilization of blood transfusion services, and infectious complications in the perioperative period irrespective of operative technique., Level of Evidence: Level III, retrospective comparative study., (Copyright © 2020. Published by Elsevier Inc.)

Introduction: Oesophageal atresia ± tracheoesophageal fistula (EA/TEF) associated with congenital heart disease (CHD) carries a worse prognosis than EA/TEF alone. Though the Spitz classification takes major CHD into account, there are no data regarding survival with the specific combination of EA/TEF and Tetralogy of Fallot (TOF). With advances in postnatal care, we hypothesised that, survival is improving in these complex patients. This study reports morbidity and mortality outcomes of newborns with oesophageal atresia and TOF cardiac malformations METHODS: All patients with EA/TEF and TOF treated at Alder Hey Children's Hospital between the years 2000-2020, were identified. Data sets regarding gestation, birth weight, associated anomalies, operative intervention, morbidity, and mortality were analysed., Results: Of a total of 350, EA/TEF patients 9 (2.6%) cases had EA/TEF associated with TOF (M:F 4:5). The median gestational age was 35/40 (range 28-41 weeks) with a median birth weight of 1790 g (range 1060-3350 g). Overall survival was 56% (5/9 cases) and all survivors remain under follow up (range 37-4458 days). Surgical strategies for managing EA/TEF with Fallot's tetralogy included 6/9 primary repairs and 3/9 cases with TEF ligation only (+ gastrostomy ± oesophagostomy)., Conclusions: This study reports outcome data from one of the largest series of EA TEF patients with Fallot's tetralogy. Whilst outcomes may be challenging for this unique patient cohort, survival metrics provide important prognostic information that can be widely shared with health care teams and parents.

Purpose: The purpose of this study is to identify determinants of mortality and hospital readmission in infants born with esophageal atresia ± tracheoesophageal fistula., Methods: The Nationwide Readmissions Database (2010-2014) was queried for newborns with a diagnosis of esophageal atresia. Outcomes included mortality and readmissions at 30-day and 1-year., Results: 3157 patients were identified, of which 54% were male. 81% had an additional congenital anomaly, and 35% had VACTERL association. Overall mortality at index hospitalization was 11% (n = 360) and was significantly higher with additional congenital anomalies (13%), VACTERL (19%), and Spitz classification II/III (18%) vs. isolated esophageal atresia/tracheoesophageal fistula (4%), all p < 0.001. After esophageal atresia repair (n = 2179), 10% (n = 212) were readmitted within 30 days and 26% (n = 563) within 1 year, with 17% admitted to different hospitals. Common diagnoses during readmission were GERD (54%), infections (42%), failure to thrive (17%), tracheomalacia (14%), and esophageal stricture (10%). Unplanned readmissions accounted for 85% of readmissions. A large number underwent operative procedures, most commonly esophageal dilation (17%) and fundoplication/gastrostomy (12%)., Conclusion: Our study has uncovered a high likelihood of complications and unplanned readmission within the first year of life for newborns with esophageal atresia. Coordinated multidisciplinary care may help to decrease unnecessary readmissions and improve outcomes in this vulnerable population., Type of Study: Retrospective comparative analysis., Level of Evidence: Level III., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: To describe esophageal atresia mortality rates and their associations in our cohort., Study Design: Patients with esophageal atresia, managed at The Royal Children's Hospital, Melbourne (1980-2018), who subsequently died, were retrospectively identified from the prospective Nate Myers Oesophageal Atresia database. Data collected included patient and maternal demographics, vertebral anomalies, anorectal malformations, cardiovascular anomalies, tracheoesophageal fistula, renal anomalies, and limb defects (VACTERL) associations, mortality risk factors, and preoperative, operative, and postoperative findings. Mortality before discharge was defined as death during the initial admission., Results: A total of 88 of the 650 patients (13.5%) died during the study period; mortality before discharge occurred in 66 of the 88 (75.0%); mortality after discharge occurred in 22 of the 88 (25.0%). Common causes of mortality before discharge were palliation for respiratory anomalies (15/66 [22.7%]), associated syndromes (11/66 [16.7%]), and neurologic anomalies (10/66 [15.2%]). The most common syndrome leading to palliation was trisomy 18 (7/66 [10.6%]). Causes of mortality after discharge had available documentation for 17 of 22 patients (77.3%). Common causes were respiratory compromise (6/17 [35.3%]), sudden unexplained deaths (6/17 [35.3%]), and Fanconi anemia (2/17 [11.8%]). Of the patients discharged from hospital, 22 of 584 (3.8%) subsequently died. There was no statistical difference in VACTERL association between mortality before discharge (31/61 [50.8%]) and mortality after discharge (11/20 [55.0%]), nor in incidence of twins between mortality before discharge (8/56 [14.3%]) and mortality after discharge (2/18 [11.1%])., Conclusions: We identified predictors of mortality in patients with esophageal atresia in a large prospective cohort. Parents of children with esophageal atresia must be counselled appropriately as to the likelihood of death after discharge from hospital., (Crown Copyright © 2019. Published by Elsevier Inc. All rights reserved.)

Background: Esophageal atresia (EA) and tracheoesophageal fistula (TEF) are serious congenital anomalies with high morbidity and mortality. Diagnostic and therapeutic fiberoptic endoscopy has been used in children to evaluate and manage trachea-esophageal anomalies. This study aimed to evaluate the prognostic factors and the role of fiberoptic bronchoesophagoscopy (FB) in managing children with EA and TEF., Methods: From 2000 to 2017, hospitalized children with suspected EA and TEF were enrolled in the study. All associated medical records were retrospectively reviewed. Basic characteristics, diagnoses, age of surgical reconstruction, FB findings, associated anomalies, and survival durations were reviewed. Prognostic factors associated with the patients' mortality were analyzed., Results: A total of 33 children were enrolled, and 91% of them were type C. The median age at the time of hospitalization was 26 days (range, birth to 9 years), including 20 (61%) low-birth-weight infants and 26 (79 %) referred patients. FB was performed in patients preoperatively (39%) and postoperatively (96.8%). Among them, 28 patients (85%) had associated anomalies, including 17 (52%) cardiac and 23 (70%) airway anomalies. The median age of 31 patients who underwent surgical reconstruction was 3 (range, 0-39) days. Esophageal anastomotic stricture (21/31, 67.7%) was the most common postsurgical complication. Twenty-three patients (74.2%) received postoperative FB-guided interventions, including balloon dilatation, laser therapy, and stent implantation. Among the 9 mortality cases, the median age at death was 270 (range, 4-3246) days. Significant factor associated with mortality was delayed (> 48 h old) or no surgical reconstruction (p = 0.030)., Conclusion: Delayed (>48-hour old) or no surgical reconstruction was significantly related to mortality in children with congenital EA and TEF. Preoperative and postoperative FB evaluations helped to facilitate diagnoses and nonsurgical managements and resolve the patients' tracheoesophageal problems.

Background: Contemporary outcomes of infants with esophageal atresia with or without tracheoesophageal fistula (EA/TEF) from multi-gestational pregnancies compared to those of singleton pregnancies have not been reported., Methods: A single-center retrospective review of EA/TEF patients born from 1999 to 2013 was performed. Patient demographics, gestational age (GA), birth weight, associated anomalies, requirement for gastrostomy tube and mortality were reviewed., Results: Singleton EA/TEF patients outnumbered those from multi-gestational pregnancies nearly 10:1 (214 vs 22 patients). EA/TEF patients from multi-gestational pregnancies were more likely to be premature (77% vs. 32%), have lower birth weight (mean 1766 g vs. 2695 g), have associated duodenal atresia (18% vs. 6%) and require gastrostomy tube (41% vs. 33%) for feeding challenges compared to EA/TEF singletons. Mortality was also significantly greater for multi-gestational EA/TEF patients compared to singleton EA/TEF patients (18% vs. 6%)., Conclusion: EA/TEF infants from multi-gestational pregnancies have greater clinical complexity and mortality than singleton EA/TEF patients. Parents of EA/TEF multi-gestational infants should be appropriately counseled and supported., (Copyright © 2019 Elsevier Inc. All rights reserved.)

The aim of this study is to investigate management and outcome in esophageal atresia (EA) and to identify early predictive factors of morbidity and mortality in a developing country. Charts of neonates with repaired EA from 2007 to 2016 were reviewed. Patients' characteristics, operative details, and postoperative outcomes were collected. Statistical analyses were performed to identify predictors of complicated evolution. Forty-two cases were collected. There were 14 girls and 28 boys. Only one patient had antenatal diagnosis (2.3%). The mean gestational age was 38 weeks. Nine patients (21.4%) weighed less than 2.5 kg. Seventeen (40.4%) patients had associated malformations most commonly cardiac (9/17). Thirteen patients had delayed diagnosis (30.9%). Thirty-nine (92.8%) patients underwent primary esophageal anastomosis. Overall survival was 76.2%. Nineteen patients (57% of survivals) had complicated evolution before the age of one year and 15 patients (46.8% of survivals) developed complications after the age of one year. Perinatal variables associated with mortality were prematurity (p = 0.004, OR = 5.4, IC95% = [1.13-25.80]), low birth weight (p = 0.023, OR = 7, IC95% = [1.38-35.47]), cardiac malformations (p = 0.006, OR = 10.5, IC95% = [2.03-54.27]) and delayed diagnosis (p = 0.005, OR = 10.11, IC95% = [2.005-50.980]). Variables associated with short-term and middle-term complications were duration of intubation (p = 0.019, OR = 0.118, IC95% = [0.019-0.713]) and the presence of short-term complications (p = 0.016, OR = 7.33, IC95% = [1.467-36.664]) respectively. These factors may be used to identify patients who will benefit from more intensive follow-up program., (© The Author(s) 2019. Published by Oxford University Press on behalf of International Society for Diseases of the Esophagus.)

Aim:  Surgical expertise and advances in technical equipment and perioperative management have led to enormous progress in survival and morbidity of patients with esophageal atresia (EA) in the last decades. We aimed to analyze the available literature on surgical outcome of EA for the past 80 years., Materials and Methods:  A PubMed literature search was conducted for the years 1944 to 2017 using the keywords "esophageal/oesophageal atresia," "outcome," "experience," "management," and "follow-up/follow up." Reports on long-gap EA only, non-English articles, case reports, and reviews without original patient data were excluded. We focused on mortality and rates of recurrent fistula, leakage, and stricture., Results:  Literature search identified 747 articles, 118 manuscripts met the inclusion criteria. The first open end-to-end anastomosis and fistula ligation was reported in 1941. Thoracoscopic fistula ligation and primary anastomosis was performed first in 2000. Reported mortality rate decreased from 100% before 1941 to 54% in 1950 to 1959, 28% in 1970 to 1979, 16% in 1990 to 1999, and 9% nowadays. Rates of recurrent fistula varied over time between 4 and 9%. Leakage rate remained stable between 11 and 16%. However, stricture rate increased from 25 to 38%., Conclusion:  Including a full range of articles reflecting the heterogeneity of EA, mortality rate significantly decreased during the course of 80 years. Along with the decrease in mortality, there is a shift to the importance of major postoperative complications and long-term morbidity regardless of surgical technique., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Purpose: Most pediatric surgeons perform <2 esophageal atresia and tracheoesophageal fistula (EA/TEF) repairs annually. We aimed to determine whether higher surgeon and hospital volumes are associated with better outcomes after EA/TEF repair., Methods: Neonates with a diagnosis and repair of EA/TEF at their index hospital admission in the Pediatric Health Information System from 1/2000 to 9/2015 were included. For each patient, hospital and surgeon operative volumes were defined as the number of EA/TEF cases treated in the previous 365 days. Propensity score weighting was used to estimate relationships between operative volumes and rates of in-hospital mortality, readmission within 30 days, and readmission, reoperation, and dilation within one year., Results: Among 3085 patients, lower birth weight, earlier gestational age, the presence of congenital heart disease, and certain other anomalies were associated with higher mortality. In risk-adjusted analyses, there were no significant differences in mortality or any other outcome based on hospital or surgeon volume alone or when comparing low- or high-volume surgeons practicing at low- or high-volume hospitals., Conclusions: Neither surgeon nor hospital volume significantly impacted outcomes after EA/TEF repair. Our findings imply that selective referral and pediatric surgeon subspecialization in EA/TEF may not translate to improved outcomes., Type of Study: Retrospective comparative study LEVEL OF EVIDENCE: Level III., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Purpose: The aim of this study was to investigate the risk factors for in hospital mortality in infants with esophageal atresia (EA) without critical chromosome abnormality disorders and develop a new prognostic classification to assess the influence of external risk factors on the prognosis of EA, which could provide more effective treatment strategies in clinical practice., Methods: A retrospective chart review of all consecutive patients between January 1994 and December 2017, which included 65 EA infants who were admitted to Shizuoka Children's Hospital, was conducted. Based on multivariate analysis data and ROC analysis, the discrimination of the new prognostic classification was quantified and compared with that of the Spitz classification using the area under the ROC curve (AUC)., Results: Multiple logistic regression analysis showed that birth weight of < 1606 g (adjusted OR, 13.16; 95% CI, 1.16-352.75), and complex cardiac anomalies (adjusted OR 22.39; 95% CI 2.45-569.14) were significant risk factors for death. We have created a new classification close to Spitz classification using the presence of complex cardiac anomalies and birth weight. The mortality rates were 0% for class I (n = 0/40), 7.1% for class II (n = 1/14), 33.3% for class III (n = 3/9), and 100% for class IV (n = 2/2). The AUC of the new classification was better than that of the Spitz classification (0.939 vs 0.812, respectively; p = 0.04)., Conclusion: New prognostic classification can improve the stratification of EA patients and be a useful predictor of survival.

Background: Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) is a complex disorder, and most outcome data are confined to mortality and feeding-related morbidities. Our objective was to examine mortality, growth and neurodevelopmental outcomes in a large recent cohort of infants with EA/TEF., Methods: Single center study of EA/TEF infants referred from January 2000 to December 2015. Data collected included associated defects, neonatal morbidity and mortality and growth and neurodevelopmental outcomes at age 12-36months. Multiple regression analysis was used to determine variables associated with adverse outcome., Results: Of the 253 infants identified, 102 infants (40%) were preterm. Overall mortality was 8.3%, the majority from major cardiac malformations (p<0.001) Neurodevelopmental assessments (n=182) showed that 76% were within normal, while some delay was seen in 24%, most often in expressive and receptive language. Nine infants had hearing impairment and 5 had visual impairment. Gastrostomy tubes were required in 47 patients and 15% continued to have weight growth velocities less than the 10th centile. A number of specialist interventions were required, Speech/Language being frequent., Conclusion: Mortality in EA/TEF is primarily related to concomitant anomalies, especially cardiac. Multidisciplinary follow up is important for early identification and intervention for growth failure and developmental delay., Type of Study: Retrospective study LEVEL OF EVIDENCE: Level II., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objective: Evaluate the neonatal management and outcomes of neonates with prenatal diagnosis of esophageal atresia (EA) type A., Methods: This population-based study was conducted using data from the French National Register for infants with EA born from 2008 to 2014, including all cases of EA type A. We compared prenatal and neonatal characteristics and outcomes in children with prenatal diagnosis of EA type A with those with a postnatal diagnosis until the age of 1., Results: A total of 1118 live births with EA were recorded among which 88 (7.9%) were EA type A. Prenatal diagnoses were performed in 75 cases (85.2%), and counselling with a prenatal specialist was conducted in 84.8% of the prenatal group. Still within that group, the gestational age at delivery was significantly higher than in the postnatal group (36 [35-38] versus 34 [32-36] weeks; P = .048). Inborn births were more frequent in the prenatal group (86.1% vs 7.7%, P < .0001), and mortality and outcome were similar in both groups., Conclusion: Prenatal diagnosis is high in EA type A, which enables to offer an antenatal parental counseling and which avoids postnatal transfers. Prognosis of EA types A does not appear to be influenced by the prenatal diagnosis., (© 2018 John Wiley & Sons, Ltd.)

Background: Extracardiac birth defects are associated with worse outcomes in congenital heart disease (CHD). The impact of esophageal atresia/trachea-esophageal fistula (EA/TEF) on outcomes after surgery for ductal-dependent CHD is unknown., Methods: Retrospective matched cohort study using the Pediatric Health Information System database from 07/2004 to 06/2015. Hospitalizations with ductal-dependent CHD and EA/TEF, undergoing CHD surgery were included as cases. Admissions with ductal-dependent CHD without EA/TEF were matched 3:1 for age at admission and Risk Adjustment for Congenital Heart Surgery-1 classification. Comparisons were performed using generalized estimating equations., Results: There were 124 cases and 372 controls. Cases included 32 (25.8%) low-risk, 86 (69.3%) intermediate-risk, and 6 (4.8%) high-risk patients. Cases had more females compared to controls (53.2% vs 41.1%, P = .022). Cases were more likely to be premature (28.2% vs 13.7%, P = .001) and low birth weight (29.8% vs 11.8%, P < .001). Cases had a similar frequency of Down syndrome, and DiGeorge/Velocardiofacial syndrome, but a higher frequency of anorectal malformations (4.3% vs 2.4%, P < .001) and renal anomalies (27.4% vs 9.9%, P < .001) than controls. Cases had a higher mortality on univariate (22.0% vs 8.4%, P < .001) and multivariable analysis (odds ratio 2.45, 95%, confidence interval 1.34 - 4.49). Prematurity also was significantly associated with mortality on multivariable analysis. Cases had a longer duration of mechanical ventilation, longer hospital duration of stay, and higher total cost than controls (all P < .001)., Conclusion: In children with ductal-dependent CHD, EA/TEF is associated with increased morbidity, mortality and resource utilization. A majority of patients undergo EA/TEF repair prior to congenital heart disease surgery. (Surgery 2017;160:XXX-XXX.)., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background/purpose: The purpose of this study was to explore clinical characteristics and primary surgical diagnoses associated with in-hospital death in pediatric surgical patients admitted to the neonatal intensive care unit (NICU) of a tertiary hospital., Methods: This retrospective study includes all patients admitted to our NICU for pediatric surgical diseases between January 2001 and December 2015. Univariate and multivariate binary logistic regression were performed to assess independent factors associated with in-hospital death., Results: A total of 440 cases were included and 334 (83.5%) patients underwent one or more surgeries. Thirty six patients (8.2%) died while hospitalized in the NICU. The 5 most common surgical diagnoses were intestinal atresia/stenosis, anorectal malformation, congenital diaphragmatic hernia (CDH), esophageal atresia, and urinary system disorder. Necrotizing enterocolitis (NEC) had the highest mortality rate. Using logistic regression, in-hospital death was predicted by extremely low birth weight (ELBW) (odds ratio (OR)=6.594; P=0.006), CDH (OR=13.954; P<0.001), and NEC (OR=8.991; P=0.049)., Conclusions: This study describes CDH, NEC, and ELBW are independent predictive factors associated with in-hospital death of pediatric surgical patients in our NICU. Novel approaches for those conditions are required to improve the survival., Type of Study: Prognostic LEVELS OF EVIDENCE: II., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Aim:  No consensus exists about the optimal surgical technique for esophageal replacement. This study reports the surgical outcomes for the gastric pull-up and the colonic interposition procedures., Materials and Methods:  A retrospective review of children undergoing esophageal replacement surgery between January 2001 and June 2015 across four different pediatric surgery centers was conducted. Data collected included indications, epidemiology, surgical technique, complications, and outcomes. Patients were divided into group A, those that had a gastric pull-up procedure and group B, those that had a colonic interposition procedure., Results:  In total, 50 patients were included; 29 in group A and 21 in group B. Indications included esophageal atresia, caustic ingestion, and infective esophageal stricture. The median age at the time of surgery was 13 months. The mean length of follow-up was 5.2 years. Three patients died giving a mortality rate of 6%; 2 in group A and 1 in group B.In both groups, early postoperative complications included infective complications, such as wound infections, sepsis, and pneumonia (11), anastomotic leak (7), and respiratory complications (7). Late complications included adhesive bowel obstruction (2), anastomotic strictures (4), redundancy (1), and jejunostomy problems (1). Septic complications and anastomotic strictures occurred more frequently in group B. Further surgery was needed in eight patients; this was significantly higher in group B. Full oral feeding was achieved within 6 months in 91.5%., Conclusion:  The gastric pull-up and colonic interposition have comparable mortality and outcomes. The colonic interposition was associated with a higher rate of early septic complications, anastomotic strictures, and need for further surgery., Competing Interests: Conflict of Interest: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Objectives: Background and aim: This study has been conducted to determine the clinical presentations and surgical outcome of patients with OA admitted in a tertiary level hospital in Bangladesh., Methods: A prospective analysis was conducted for 32 patients with confirmed OA between July 2007 and June 2015. Of them surgical correction was done in 25 cases. The other 7, in whom surgery could not be done, were excluded from the study., Results: Of the 25 cases, that had been operated, 15 were full term and 52% were more than 48 hours old. Type-C OA with TOF was the commonest type (96%). 24 cases were operated through Right Thoracotomy. Of them 52% were done by Single Lung (left) ventilation. 'Division of the fistula' with 'end to end anastomosis' was done in all of them. Only 1 patient had a Gastric pull up surgery. Of the 25 cases, 16 survived (64%). In follow up all the patients were found thriving and showed no problem in swallowing., Conclusion: Our study which shows 64% survival is a great achievement for us but not satisfactory in international standard. However, the experience and encouragement that we are gathering, will guide us to achieve better result in near future.

Background: Transanastomotic feeding tube (TAFT) is commonly used for post-operative enteral nutrition after esophageal atresia (EA)/tracheoesophageal fistula (TEF) repairs. The purpose of this study is to analyze the therapeutic implications of avoiding a TAFT and its impact on the outcomes post-operatively., Methods: The medical data of 33 inpatients with EA/TEF type C repair from 2009 to 2014 were retrospectively reviewed. Patients were divided into two groups for comparison based on the usage of TAFT during the repair: TAFT- (without) and TAFT+ (with) groups, without randomization. Both groups were compared statistically for post-operative complications and outcomes., Results: Eighteen neonates were males and 15 females, with an average birth weight of 2.43±0.43 kg and a mean gestational age of 36.15 weeks. Nineteen (57.5%) in the TAFT- group and 14 (42.4%) were in the TAFT+ group. The post-operative complications, need for dilatations, duration of total parenteral nutrition (TPN) and length of hospital stay were similar in both groups. The incidence of pneumonitis was significantly higher in the TAFT+ group., Conclusions: By avoiding a TAFT, there is no increase in complication rates nor does it entail a prolonged period of TPN. Besides, not using a TAFT may have the advantage of reducing incidence of aspiration and consequent pneumonitis.

Background: Several classification systems exist to predict mortality in oesophageal atresia, the most widely quoted of these being over 20 years old. No classification system exists to predict morbidity. We sought to test whether these classification systems remain relevant and to determine whether they can be useful to predict morbidity. In addition, we aimed to identify independent risk factors for predicting mortality and morbidity., Methods: Neonates presenting with oesophageal atresia over a 20-year period (1990-2010) were retrospectively reviewed. Discriminative statistical analysis compared the performance of current classification systems. Stepwise logistic regression analysis of the influence of perioperative risk factors on mortality and duration of ventilatory support and intensive care unit stay were performed., Results: All classification systems predicted mortality in this series of 248 neonates. Birth weight, cardiac anomalies and pre-operative pneumonia were independent risk factors for predicting mortality in oesophageal atresia. The Waterston classification is the most useful classification for predicting post-operative morbidity in terms of length of hospital stay and time spent ventilated., Conclusion: Despite advances in the neonatal care of the very low birth weight infant and those with congenital cardiac disease, these conditions remain relevant in predicting mortality and morbidity in oesophageal atresia.

Esophageal atresia (EA) is a rare anomaly that mandates surgical intervention. Patients with EA often have complicated medical courses due to both esophageal anomalies and related comorbidities. Although several prognostic classification systems have been developed to decrease the mortality rate in EA, most systems focus only on the influence of the major anomaly, and external risk factors that could be influenced by the neonatal caregivers to a certain extent are not included. The aim of this study was to investigate the risk factors for in-hospital mortality in neonates with EA and develop a scoring model to predict mortality.In total, 198 infants with EA who were treated with surgical intervention at the Children's Hospital of Chongqing Medical University between March 2004 and June 2016 were included. The demographic information, clinical manifestations, laboratory testing, and outcomes during hospitalization were analyzed retrospectively. A predictive scoring model was developed according to the regression coefficients of the risk factors.The mortality rate was 18.1% (36/198). In the univariate analysis, higher incidences of prematurity, low birth weight, long gap, anastomotic leak, respiratory failure, postoperative sepsis, respiratory distress syndrome, pneumothorax, and septic shock were found in the nonsurvivor group than in the survivor group (P < .05). In the logistic regression analysis, anastomotic leak (OR: 10.75, 95% CI: 3.113-37.128), respiratory failure (OR: 4.104, 95% CI: 2.292-7.355), postoperative sepsis (OR: 3.564, 95% CI: 1.516-8.375), and low birth weight (OR: 8.379, 95% CI: 3.357-20.917) were associated with a high mortality rate. A scoring model for predicting death was developed with a sensitivity of 0.861, a specificity of 0.827, a positive predictive value of 0.524, and a negative predictive value of 0.963 at a cutoff of 2 points. The area under the receiver-operating characteristic curve of the score was 0.905 (95% CI, 0.863-0.948, P = .000) for death from EA. The mortality rate increased rapidly as the scores increased, and all patients with scores ≥5 died.Anastomotic leak, respiratory failure, postoperative sepsis, and low birth weight are independent risk factors for mortality in EA. Infants with a predictive score of 5 had a high risk of death.

Background: Gastric transposition is a relatively novel method of esophageal replacement. The purpose of this retrospective study was to assess the outcomes of long-gap esophageal atresia (LGEA) treated with esophageal replacement using primary gastric transposition in neonates., Methods: Between March 2008 and May 2015, 14 newborns with LGEA were treated in our hospital. They were all found to have gaps of over 3 cm at the time of the surgery and were diagnosed with LGEA. Primary gastric transposition was performed. They also underwent a gastric drainage procedure by pyloromyotomy. The nasogastric tube was removed if no anastomotic fistula was present and oral feeding was initiated. After initial recovery and discharge, the patients were evaluated with outpatient follow-ups or telephone follow-ups from 1 month after the surgery., Results: The mean age of the neonates at the time of the surgery was 32 hours (range, 4-96 h). The mean birth weight was 2550 g (range, 2100-3500 g). There were 2 deaths in this series of patients due to respiratory failure or withdrawal of treatment by the parents, with a mortality rate of 14.3%. Seven of the neonates developed unilateral or bilateral severe pneumonia. Early anastomotic leak occurred in 3 cases and anastomotic strictures occurred in 4 cases. These 4 neonates were able to eat a fairly normal diet after esophageal balloon dilation. Gastroesophageal reflux occurred in 7 of 12 cases. Feeding multiple small meals and postural support for positioning and feeding were instructed for these 7 cases. Subsequently, the symptoms alleviated and they had no additional surgical therapy. None of the neonates had delayed gastric emptying or gastric retention., Conclusion: Primary gastric transposition may be a rewarding reconstructive option in the treatment of LGEA.

Background: Esophageal atresia (EA) and tracheoesophageal fistula (TEF) represent major therapeutic challenges, frequently associated with serious morbidities following surgical repair. The aim of this longitudinal study was to assess temporal changes in morbidity and mortality of patients with EA/TEF treated in a tertiary-level center, focusing on postoperative complications and their impact on long-term gastroesophageal function., Methods: One hundred nine consecutive patients with EA/TEF born between 1975 and 2011 were followed for a median of 9.6 years (range, 3-27 years). Comparative statistics were used to evaluate temporal changes between an early (1975-1989) and late (1990-2011) study period., Results: Gross types of EA were A (n = 6), B (n = 5), C (n = 89), D (n = 7), and E (n = 2). Seventy (64.2%) patients had coexisting anomalies, 13 (11.9%) of whom died before EA correction was completed. In the remaining 96 infants, surgical repair was primary (n = 66) or delayed (n = 25) anastomosis, closure of TEF in EA type E (n = 2), and esophageal replacement with colon interposition (n=2) or gastric transposition (n=1). Long-gap EA was diagnosed in 23 (24.0%) cases. Postoperative mortality was 4/96 (4.2%). Overall survival increased significantly between the two study periods (42/55 vs. 50/54; P = 0.03). Sixty-nine (71.9%) patients presented postoperatively with anastomotic strictures requiring a median of 3 (range, 1-15) dilatations. Revisional surgery was required for anastomotic leakage (n = 5), recurrent TEF with (n = 1) or without (n=9) anastomotic stricture, undetected proximal TEF (n = 4), and refractory anastomotic strictures with (n = 1) or without (n = 2) fistula. Normal dietary intake was achieved in 89 (96.7%) patients, while 3 (3.3%) remained dependent on gastrostomy feedings. Manometry showed esophageal dysmotility in 78 (84.8%) infants at 1 year of age, increasing to 100% at 10-year follow-up. Fifty-six (60.9%) patients suffered from dysphagia with need for endoscopic foreign body removal in 12 (13.0%) cases. Anti-reflux medication was required in 43 (46.7%) children and 30 (32.6%) underwent fundoplication. The rate of gastroesophageal reflux increased significantly between the two study periods (29/42 vs. 44/50; P = 0.04). Twenty-two (23.9%) cases of endoscopic esophagitis and one Barrett's esophagus were identified., Conclusions: Postoperative complications after EA/TEF repair are common and should be expertly managed to reduce the risk of long-term morbidity. Regular multidisciplinary surveillance with transitional care into adulthood is recommended in all patients with EA/TEF.

The management of esophageal atresia is still limited due to the precariousness of technical equipments in Madagascar. Our case study aims to highlight possible therapeutic options and to describe the progresses to be made so as to optimize treatment of this congenital pathology. We collected the medical records of all patients hospitalized for esophageal atresia in the Department of Surgical Reanimation at the University Hospital-JRA Antananarivo. The first patients admitted who survived for a period of 42 months (between January 2011 and June 2014) were included in the study. Among the 17 patients admitted with esophageal atresia, three full-term newborns with type III esophageal atresia, successively admitted in Surgical Reanimation, were the first patients in Madagascar to survive after surgery. Only one patient had associated malformations. These three babies were extubated early in the operating room; they underwent oxygen therapy until normal spontaneous breathing. New-born babies underwent post-operatory physical therapy allowing to improve their respiratory status. Overall mortality rate from this pathology was 76.5% in 42 months. Despite these initial success, progresses still remain to be done in the treatment of this congenital anomaly, from diagnosis up to postoperative period. The improvement of technical equipments, especially ventilation and nutritional support equipments would allow more meaningful results, like in the countries where progresses has been made in the field of reanimation.

Aim: The aim of this study is to analyse the effect of delayed diagnosis on mortality rates, and evaluate the role of delayed diagnosis as a new prognostic factor in patients with oesophageal atresia (OA), especially in developing countries., Methods: The records of 80 consecutive patients with OA (2008-2013) were reviewed. Patients were divided into two groups according to the time of diagnosis. As we demonstrated the effect of delayed diagnosis on mortality, we decided to develop a new classification that will be utilised to predict the prognosis of OA. The discrimination ability of the new prognostic classification was compared with those of the Waterston, Montreal and Spitz classifications using the area under the curve., Results: The parameters of the new prognostic classification were birth weight less than 2000 g, the presence of major cardiac/life-threatening anomalies and delay in diagnosis. Class I consisted of patients with none or one of these parameters. Class II consisted of patients with two or three of these parameters. The area under the curve of the new classification was better than those of the other classifications in determining the prognosis of patients with OA., Conclusions: Delayed diagnosis of OA significantly led to morbidity and mortality. Although delayed diagnosis is not a characteristic of newborn or a marker of severity for OA and is a health care system issue in developing countries, we here point out that it is a prognostic factor in its own right. Our new classification has a superior discriminatory ability compared to the above-mentioned classifications., (© 2016 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

Background/purpose: A successful outcome in the repair of esophageal atresia (EA) is associated with a high quality pediatric surgical centre, however there are several controversies regarding the optimal management. The aim of this study was to investigate the outcome and management EA in a single pediatric surgical centre., Methods: Medical records of infants with repaired EA from 1994 to 2013 were reviewed., Results: 129 infants were included. Median follow-up was 5.3 (range 0.1-21) years. Overall survival was 94.6%, incidences of anastomotic leakage 7.0%, recurrent fistula 4.6% and anastomotic stricture 53.5% (36.2% within first year). In long gap EA (n=13), delayed primary anastomosis was performed in 9 (69.2%), gastric tube in 3 (23.1%) and gastric transposition in one (7.7%) infants. The incidences of anastomotic leakage and stricture in long gap EA were, 23.1% and 69.2%, respectively. Peroperative tracheobronchoscopy and postoperative esophagography were implemented as a routine during the study-period, but chest drains were routinely abandoned., Conclusion: The outcome in this study is fully comparable with recent international reports showing a low mortality but a significant morbidity, especially considering anastomotic strictures and LGEA. Multicenter EA registry with long-term follow up may help to establish best management of EA., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: The aim of the study was to evaluate the outcomes of initial surgical procedures for oesophageal atresia at our institution. Primary repair of oesophageal atresia at our centre was perceived to be associated with a high mortality rate. In view of this, almost all patients seen since January 2014 were offered initial surgery for staged repair., Materials and Methods: A retrospective review of records of infants with oesophageal atresia seen at the centre from January 2007 to December 2014 was used in this study., Results: Eighty-five cases of oesophageal atresia were seen over the study period. Of these, 74 (87.1) had surgery performed. Surgical outcome was, however, indicated in 67 of the 74, with overall survival rate of 40.3%. Among the 67 patients, early primary repair was performed in 24 (35.8%) with a survival rate of 45.8% (11 patients). Totally, 12 (17.9%) of the 67 had initial procedure for delayed primary repair, with a survival rate of 16.7% (2 patients). The remaining 31 (46.3%) patients had initial surgery for staged repair, with survival rate of 45.2% (14 patients). There was no association between the type of surgery and the surgical outcome (χ2 = 3.396, df = 2, P = 0.183)., Conclusion: The overall surgical survival rate of 40.3% for oesophageal atresia at our institution is low. This study did not show any difference in the survival rate of 45.8% and 45.2% associated with primary repair and staged repair respectively.

Aim of Study: We examined variables associated with survival for oesophageal atresia between 1996 and 2014., Methods: Possible explanatory variables: birth weight, gestation, cardiac anomalies (any or major), renal anomalies (any or severe), primary anastomosis, leak, secondary oesophageal surgery, tracheomalacia, aortopexy, tracheostomy, gastrostomy, fundoplication, karyotype, neurological status. Variables were assessed with logistic regression and a new model assessed with Kaplan-Meier graphs., Results: 104/120 (87%) babies survived. Median gestation 37weeks, 4 (3%) born before 28weeks. Mean birth weight 2.3 (SD 0.7) kg, 17 (14%) less than 1500g. Frequency (%) of explanatory variables: Major cardiac anomaly 21 (18%), any cardiac anomaly 48 (40%), severe renal anomaly 10 (8%), any renal anomaly 25 (21%), primary anastomosis 105 (88%), anastomotic leak 16 (13%), symptomatic tracheomalacia 28 (23%), aortopexy 17 (14%), tracheostomy 12 (10%), neurological anomaly 7 (6%), fundoplication 15 (13%), gastrostomy 30 (25%), secondary oesophageal surgery 8 (7%), abnormal karyotype 6 (5%). Multivariate analysis showed only renal (OR 0.04, 0.007 0.2) p=0.001, cardiac (OR 0.1, 0.002 0.6) p=0.01 and a primary anastomosis (OR 12.2, 1.8 81.6) p=0.01 (R2=0.48), or major cardiac (OR 0.04, 0.007 0.29) p=0.001 and severe renal anomalies (OR 0.009, 0.001 0.12) p<0.001 alone were significant (R2=0.57)., Conclusions: Survival is dependent on cardiac and renal anomalies. Birth weight is not significant. We propose a new classification system: 1: neither severe renal nor major cardiac anomaly, 2: either severe renal or major cardiac anomaly, 3: severe renal and major cardiac anomaly., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Esophageal atresia (EA) is a congenital malformation of the upper gastrointestinal tract with an estimated prevalence varying from 1 in 2500 to 1 in 4500 births. The aim of this study was to describe the epidemiology of EA between 1981 and 2012 and evaluate patients' survival., Methods: This study used data from a population-based Italian Congenital Malformation Registry. The survival status was ascertained by linking the registry records, vital records and the regional registries of patients. Kaplan-Meier methods were used to estimate survival probabilities up to 25 years and Cox proportional hazards regression was used to evaluate factors that affected survival., Results: A total of 407 cases of EA were identified among 1,417,724 total births. After the exclusion of cases with chromosomal anomalies, 49.9% of the patients presented with at least one associated congenital anomaly. The 25-year survival probability was 85.1% (95% confidence interval [CI], 80.8-89.4), with most deaths occurring during the first months of life. Patients' characteristics associated with decreased survival probability were low birth weight (hazard ratio, 3.7; 95% CI, 1.7-8.3) and presence of additional major defects (hazard ratio, 2.8; 95% CI, 1.3-6.0). A significant improvement in survival over the decades was observed for patients with nonisolated EA., Conclusion: This study detected a significant improvement in survival of individuals with EA over the past decades and identified the strongest predictors of mortality. These results will be important for the planning of the clinical management and formulation of prognosis when EA is diagnosed in a newborn. Birth Defects Research (Part A) 106:542-548, 2016. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

Introduction: Management of oesophageal atresia (OA) and trachea-oesophageal fistula (TOF) in babies of low birth weight is challenging especially when associated with other anomalies. Birth weight of <1500 g has previously formed part of a classification system designed to predict outcome, alongside the cardiac status of the patient. Improvements in neonatal care have led to increasing numbers of premature low birth weight infants surviving. The aim of this study was to look at the experience of our institution in the extremely low birth weight (ELBW) patients., Methods: A retrospective review of our institutions OA database was performed from 1993 to June 2015. Patients of birth weight less than 1000 g were included. A review of our OA/TOF clinical database and notes review established the following; gestation, birth weight, associated anomalies, operative procedures, morbidity and mortality., Results: Of 349 patients with OA across the 22-year period, 9 ELBW patients were identified (<1000 g). Six males and three females. Gestational age ranged from 23 to 34 weeks and median birth weight was 815 g ranging from 630 to 950 g. Overall survival was 56 % (5/9). There were double the numbers of ELBW OA/TOF patients seen in the second half of the study period presumably the result of improving neonatal care. Seven patients had type C OA with TOF and underwent emergency TOF ligation, two had concomitant oesophageal repair. One of these patients died from NEC; the other survived. Of the five who had isolated TOF ligation three died-two from cardiac disease and one from prematurity. Both type A patients survived and after initial gastrostomy placement one had a primary delayed repair, the other a gastric transposition. All three babies under 800 g died-one from cardiac disease the others from conditions indicative of their prematurity-necrotising enterocolitis and intraventricular haemorrhage., Conclusions: 50 % survival is achievable in OA/TOF under 1 kg and the Spitz classification is still applicable in this group as a whole. However, none of the current classification systems are applicable in infants <800 g who in our study all had poor outcomes. We suggest these should be considered as separate group when predicting outcomes.

Objectives Improved survival in infants with esophageal atresia (EA) with a birth weight < 1,500 g or a major cardiac anomaly has been reported when compared with the original Spitz classification proposed in 1994. Aim We reviewed outcome data for infants born over the last decade in our institution to update previously reported survival statistics. Materials and Methods The records of all neonates (n = 200) with a diagnosis of EA managed in a single institution between 2001 and 2011 were reviewed and compared with data from the original Spitz study and the subsequent reported cohort from the same institution. Data were obtained on birth weight, presence of a major cardiac anomaly, and survival. Differences in survival were compared using the Yates-corrected chi-square test. Local ethical study approval was obtained. Results Infants born over the last decade had a comparable overall survival rate of 93% (186/200) versus 92.6% (174/188) in the previously reported cohort (1993-2004). We demonstrate an improved survival as compared to the Spitz cohort (87.6%, 326 /372, p = 0.06) and a statistically significant improvement in survival in Group II (p = 0.01). Within this group, 12/51 neonates had a birth weight < 1,500 g and 39/51 had major cardiac anomalies. Of interest, of the nine deaths in Group II, eight were in the subgroup with major cardiac anomalies. Conclusion The survival of neonates in Group II has significantly improved. Mortalities within this group were predominantly in the subgroup with major cardiac anomalies suggesting birth weight is of less significance than in previous years reflecting recent advances in neonatal care. We propose an updated prognostic classification that makes a distinction between cardiac and low-birth-weight infants., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: The predictors of left ventricular outflow tract obstruction (LVOTO) after the repair of coarctation of the aorta or interruption of the aortic arch (CoA/IAA) with ventricular septal defect have been investigated. However, the predictors remain controversial., Methods: We performed primary repair of CoA/IAA with ventricular septal defect for 75 patients from 1996 to 2005. Four of the 75 patients died within 5 years after primary repair without relation to LVOTO. The morphology of the aortic valve of 71 survivors was bicuspid in 23 patients and tricuspid in 48 patients. The mean follow-up was 9.2 ± 2.6 years after primary repair., Results: There were 12 patients who showed LVOTO of 3.0 m/s or greater after primary repair. All of the 6 bicuspid patients demonstrated valvular aortic stenosis, and all of the 6 tricuspid patients showed discrete subvalvular LVOTO. In 5 of the 6 tricuspid patients, the aortic annular z-score before primary repair was -3.0 or less. A bicuspid aortic valve (p = 0.016) and the aortic annular z-score of -3.0 or less (p = 0.019) were significant risk factors for LVOTO after primary repair. At 10 years after primary repair, 82.6% and 95.6% of the bicuspid and tricuspid patients, respectively, were free from reoperation (p = 0.015)., Conclusions: The presence of a bicuspid aortic valve and an aortic valve annular z-score of -3.0 or less before primary repair are risk factors for LVOTO, and stenotic bicuspid valves and discrete subvalvular LVOTO are the main causes of LVOTO after primary repair of CoA/IAA with ventricular septal defect. The bicuspid patients more frequently required reoperation than the tricuspid patients., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Purpose: We hypothesize that weekend esophageal atresia and tracheoesophageal fistula (EA/TEF) repair has worse outcomes compared to procedures performed on weekdays., Methods: Kids' Inpatient Database (1997-2009) was searched for EA/TEF in infants admitted at <8days of life. Cases were limited to patients who underwent repair during their hospitalization. Risk-adjusted multivariate analysis (MVA) compared complications, mortality, and resource utilization (length of stay [LOS] total charges [TC]) between weekday and weekend procedures., Results: Overall, 861 EA/TEF cases with known day of repair were identified. Cohort survival was 96%. On risk-adjusted MVA, complication rates were higher with EA/TEF repair on a weekend (OR: 2.2) compared to a weekday. Additionally, complications (OR: 6.5) and LOS (OR: 9.3) were found to be higher among African American children compared to Caucasians. LOS was higher in patients with Medicaid (OR: 2.4) and repairs performed at non-teaching hospitals (OR: 3.2). Weekend vs. weekday procedure had no significant effect on mortality or resource utilization., Conclusion: By risk-adjusted MVA, increased complication rates for EA/TEF are seen in patients undergoing repair on weekends compared to weekdays. Additionally, African American children experienced higher complication rates compared to Caucasians. LOS after repair varies according to race, payer status, and hospital characteristics., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Purpose: Esophageal atresia (EA) is a rare congenital malformation requisite of surgical treatment. Survival rates have significantly increased in recent decades, but treated children are at risk of chronic morbidity. This study aims to review the literature on health-related quality of life (HRQOL) in patients with EA and describe the questionnaires used., Methods: A literature search was conducted in Pubmed, Cinahl and PsycINFO, from inception to January 2015. A meta-analysis of studies investigating HRQOL in patients with EA compared to healthy references was performed. The effect size was calculated as Cohen's d., Results: Twelve articles (published 1995-2014) describing HRQOL among children, young people and adults were identified. Only European studies were found, and these had variable sample sizes (8-128). Overall HRQOL was reported to be reduced in five of the seven studies that compared overall HRQOL with a healthy reference population. Impaired physical or general health was described in eight articles. In the meta-analysis, eight eligible studies provided 16 estimates of the effect of EA, six of which reached statistical significance for worse HRQOL (p < 0.05). Using Cohen's criteria, the pooled estimate of the effect of EA was small for overall and physical HRQOL, and <0.2 for the mental and social HRQOL components. Altogether, 15 different questionnaires were used, and none were condition specific for EA., Conclusions: Different findings are reported; however, this study suggests that patients with EA may have a reduced HRQOL. Moreover, HRQOL is not adequately measured in this group. Additional research is required.

Objective: The aim of this study was to evaluate the performance of prenatal diagnosis of esophageal atresia (EA) and its associated abnormalities., Methods: We conducted a retrospective study from a pediatric database of EA managed postnatally in a single center. Prenatal data included ultrasound and magnetic resonance imaging parameters including amniotic fluid (AF) volume, stomach visualization, AF biochemistry, and associated malformations. Postnatal data included type of EA, mortality, and postnatal diagnosis of associated malformations., Results: One hundred twenty-two cases were included. The diagnosis was suspected prenatally in 39/122 (32%) cases. Polyhydramnios was noted in 64/122 (52.4%), and the stomach was not visualized or small in 39 (32%). There was 14 (11.5%), 2 (1.6%), 101 (82.8%), 5 (4.1%), and 0 (0%) types I, II, III, IV, and V, respectively. EA was suspected prenatally in 12/14 (85.7%) in type I and in 27/108 (25%) in cases with tracheoesophageal fistula (II + III + IV + V). Magnetic resonance imaging was performed in 28 cases, which confirmed EA in 19/28 (sensitivity 67.8%). AF biochemistry was performed in 17 cases, which confirmed EA in 15/17 (sensitivity 88.2%) cases. Of the 69 syndromic associations, 41/69 (59.4%) cases were detected prenatally. Associated malformation was a strong predictor of postnatal death [19/69 vs 3/53, odds ratio 6.33 (1.76; 22.75), p < 0.01]., Conclusion: Prenatal diagnosis of EA remains challenging. MRI and AF biochemistry may prove useful in the diagnosis of EA. Prenatal ultrasound and MRI examination should also focus on associated anomalies. © 2015 John Wiley & Sons, Ltd., (© 2015 John Wiley & Sons, Ltd.)

Objective: The survival rate in newborns with congenital esophageal atresia (EA) is about 85-90 %, and it raises over 95 % in the developed countries. The survival rate in developing countries is much lower and the authors describe their experience with 60 newborns., Methods: Medical records of 60 newborns (40 boys and 20 girls) with congenital EA were reviewed for the prognostic factors and mortality., Results: The birth weight, mean Apgar score (AS) value, gestational age and birth-operative treatment time had significant influence on the mortality of treated patients (p < 0.05). Thirty five percent newborns had aspirational pneumonia at the moment of hospitalization and 86.7 % of them were operated during the first 48 h. The presence of associated anomalies considerably affected the death rate of treated patients (p < 0.05). The incidence of postoperative complications was similar to those in developed countries but the total mortality was higher (28.3 %); sepsis being the main cause of mortality. The postoperative complications and sepsis significantly influenced the mortality of patients (p < 0.05)., Conclusions: Total mortality in newborns with EA was high; sepsis being the most frequent cause of death. The high total mortality was also caused by prematurity, delay in diagnosis, increased incidence of the aspiration pneumonia and shortage of qualified nurses.

The purpose was to study the outcomes and factors affecting the survival of esophageal atresia in our center. A retrospective analysis of 86 cases of esophageal atresia (EA) over a 10-year period was performed with 46 boys and 42 girls. Demographic data, birth weight, gestational age, consanguinity, incidence of associated anomalies, place of delivery, history of feeding, and outcomes were studied. EA with distal tracheoesophageal fistula (TEF) was the commonest type with 58/86 (67%). The percentage of patients with at least one associated anomaly was 52/86 (60%), with 7/86 (8%) who are from consanguineous parents; most commonly associated anomalies were cardiac 13/86 (15%). The average gestational age and birth weight were 36 ± 2 weeks and 2300 ± 570 g, respectively. Survival rates for the patients according to the Waterston classification was 80% in group A, 58% in group B, and 25% in group C (three patients died before surgery). Prematurity, the gap between the two ends of the esophagus, and preoperative respiratory status were the most significant factors affecting the survival. Late complication of EA/TEF include respiratory symptoms, especially in the first year, associating tracheomalacia and bronchopulmonary infections in about 24/45 (53%), recurrence of TEF 3/45 (7%), esophageal stricture 26/45 (58%), and gastroesophageal reflux 22/45 (49%). The high incidence of delayed diagnosis, low birth weight, and lack of advanced neonatological management are important contributory factors to the poor outcome. The frequency of late complications highlights the need for multidisciplinary clinics to follow these children's., (© 2014 International Society for Diseases of the Esophagus.)

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., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Aim: The ideal repair of esophageal atresia (EA) is primary anastomosis with closure of the fistula if present. Long gap or local circumstances prompt other procedures that occasionally lead to disastrous complications. The aim of this study was to analyze the management of these complications in a tertiary referral center., Patients and Methods: A retrospective review of patients treated for EA between 1993 and 2013 was conducted. Both the patients were primarily treated by us, and referrals from elsewhere after two or more failed operations were included., Results: In total, 23 patients were included (3/176 cases of EA treated primarily by us and 20 referrals). Of the 23 patients, 6 had type I EA, 15 type III (four long gaps), 1 type IV, and 1 type V. Cardiac anomalies were associated in seven cases, duodenal atresia in three, and Down syndrome in two patients. Primary anastomosis was initially achieved in 12 patients. Primary or secondary Foker lengthening was used in seven cases. The causes of the failure were anastomotic leaks in nine, unmanageable strictures in seven, and refistulization in five patients. These patients required 66 reoperations (median of 3 [2-7]) before inclusion in the study. Radical tertiary treatment consisted of 15 esophageal replacements (11 colonic grafts and 4 gastric pull-ups), and 1 esophageal-gastric disconnection. Five patients previously treated with esophageal replacement and referred for graft problems required 13 interventions. Two families did not give consent for one replacement and one disconnection. Complications appeared in 12 patients, and 9 additional operations were required in 7 patients. With a follow-up of 31 months (range, 4-139 months) 15 patients take all their meals per os, 5 occasionally use the gastrostomy, and 2 and 1 are fed exclusively via gastrostomy or jejunostomy. All tracheoesophageal fistulas were closed, but 15 cases are below p3 for weight and 12 for height. Three patients (13%) ultimately died 32 months (range, 9-56 months) after the first operation (due to aspiration in one, and for causes unrelated to it in the other two [tracheostomy obstruction and Guillain-Barré syndrome])., Conclusions: When repeated complications appear after EA repair, radical surgical attitudes may be justified. If esophageal continuity cannot be reestablished, the native esophagus may have to be discarded and replaced. Many complications should be expected, but the end result can be good. These patients should be referred to centers with large experience in the management of this complex condition., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: The aim of this study was to examine national outcomes in newborn patients with esophageal atresia and tracheoesophageal fistula (EA/TEF) in the United Sates., Methods: Kids' Inpatient Database (KID) is designed to identify, track, and analyze national outcomes for hospitalized children in the United States. Inpatient admissions for pediatric patients with EA/TEF for kids' Inpatient Database years 2000, 2003, 2006, and 2009 were analyzed. Patient demographics, socioeconomic measures, disposition, survival and surgical procedures performed were analyzed using standard statistical methods., Results: A total of 4168 cases were identified with diagnosis of EA/TEF. The overall in-hospital mortality was 9%. Univariate analysis revealed lower survival in patients with associated acute respiratory distress syndrome, ventricular septal defect (VSD), birth weight (BW) < 1500 g, gestational age (GA), time of operation within 24 h of admission, coexisting renal anomaly, imperforate anus, African American race, and lowest economic status. Multivariate logistic regression identified BW < 1500 g (odds ratio [OR] = 4.5, P < 0.001), operation within 24 h (OR = 6.9, P < 0.001), GA <28 wk (OR = 2.2, P < 0.030), and presence of VSD (OR = 3.8, P < 0.001) as independent predictors of in-hospital mortality. Children's general hospital and children's unit in a general hospital were found to have a lower mortality rate compared with not identified as a children's hospital after excluding immediate transfers (P = 0.008)., Conclusions: BW < 1500 g, operation within 24 h, GA < 28 wk, and presence of VSD are the factors that predict higher mortality in EA/TEF population. Despite dealing with more complicated cases, children's general hospital and children's unit in a general hospital were able to achieve a lower mortality rate than not identified as a children's hospital., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background: This study reports national estimates of population characteristics and outcomes for patients with esophageal atresia with or without tracheoesophageal fistula (EA/TEF) and evaluates the relationships between hospital volume and outcomes., Methods: Patients admitted within 30 days of life who had International Classification of Diseases, 9th Edition, Clinical Modification diagnosis and procedure codes relevant to EA/TEF during 1999-2012 were identified with the Pediatric Health Information System database. Baseline demographics, comorbidities, and postoperative outcomes, including predictors of in-hospital mortality, were examined up to 2 years after EA/TEF repair., Results: We identified 3,479 patients with EA/TEF treated at 43 children's hospitals; 37% were premature and 83.5% had ≥1 additional congenital anomaly, with cardiac anomalies (69.6%) being the most prevalent. Within 2 years of discharge, 54.7% were readmitted, 5.2% had a repeat TEF ligation, 11.4% had a repeat operation for their esophageal reconstruction, and 11.7% underwent fundoplication. In-hospital mortality was 5.4%. Independent predictors of mortality included lower birth weight, congenital heart disease, other congenital anomalies, and preoperative mechanical ventilation. There was no relationship between hospital volume and mortality or repeat TEF ligation., Conclusion: This study describes population characteristics and outcomes, including predictors of in-hospital mortality, in EA/TEF patients treated at children's hospitals across the United States. Across these hospitals, rates of mortality or repeat TEF ligation were not dependent on hospital volume., (Copyright © 2014 Mosby, Inc. All rights reserved.)

Trisomy 18 is a common chromosomal aberration syndrome involving growth impairment, various malformations, poor prognosis, and severe developmental delay in survivors. Although esophageal atresia (EA) with tracheoesophageal fistula (TEF) is a potentially fatal complication that can only be rescued through surgical correction, no reports have addressed the efficacy of surgical intervention for EA in patients with trisomy 18. We reviewed detailed clinical information of 24 patients with trisomy 18 and EA who were admitted to two neonatal intensive care units in Japan and underwent intensive treatment including surgical interventions from 1982 to 2009. Nine patients underwent only palliative surgery, including six who underwent only gastrostomy or both gastrostomy and jejunostomy (Group 1) and three who underwent gastrostomy and TEF division (Group 2). The other 15 patients underwent radical surgery, including 10 who underwent single-stage esophago-esophagostomy with TEF division (Group 3) and five who underwent two-stage operation (gastrostomy followed by esophago-esophagostomy with TEF division) (Group 4). No intraoperative death or anesthetic complications were noted. Enteral feeding was accomplished in 17 patients, three of whom were fed orally. Three patients could be discharged home. The 1-year survival rate was 17%: 27% in those receiving radical surgery (Groups 3 and 4); 0% in those receiving palliative surgery (Groups 1 and 2). Most causes of death were related to cardiac complications. EA is not an absolute poor prognostic factor in patients with trisomy 18 undergoing radical surgery for EA and intensive cardiac management., (© 2013 Wiley Periodicals, Inc.)

Aim: Long-gap oesophageal atresia (LGOA) remains a rare variant of a relatively common congenital malformation. Objectives of this study were to review the short-term results including survival, length of stay and post-operative complications for infants with LGOA managed at a single centre in addition to their growth and neurodevelopmental assessment., Methods: Retrospective review of the case notes of all infants admitted with oesophageal atresia to our institution from January 2001 to May 2011. Infants with LGOA were selected based on pre-defined criteria. Demographic and clinical variables and details of follow-up visits including developmental assessments were extracted from their case notes., Results: Of 101 infants with oesophageal atresia, 15 fulfilled the criteria for LGOA. Overall survival was 80%. Median length of stay was 83 days. Additional congenital anomalies were present in nine (60%). A fall in weight centile during hospitalisation or outpatient follow-up signifying growth failure was seen in a majority with 11 of 13 patients showing this phenomenon. Follow-up at our institution ranged from 6 months to 9 years. Developmental assessments (Bayley-III) commenced in August 2006 were available in four patients (age 5-13 months) and were abnormal in all, with particular delay in the gross motor domain., Conclusions: Infants with LGOA spend a long time in hospital. They remain at significant risk of growth failure during hospitalisation and following discharge. There appears to be a risk of developmental delay that warrants close monitoring., (© 2013 The Authors. Journal of Paediatrics and Child Health © 2013 Paediatrics and Child Health Division (Royal Australasian College of Physicians).)

Background: The objective of this study was to assess effect of number of associated anomalies on outcome in oesophageal atresia with or without tracheoesophageal fistula patients., Materials and Methods: Retrospective analysis of records of neonates admitted with a diagnosis of oesophageal atresia (EA) with or without tracheoesophageal fistula during January 2005 to May 2011. Preoperative investigation included chest X-Ray, ultrasonography of abdomen and echocardiography. Associated anomalies were grouped as minor or major depending on whether life threatening or not. Major anomalies were further sub grouped according to the involvement of single, two or > 2 organ systems. Survival was correlated with the presence of anomalies and the number of systems involved., Results: Out of 301 patients with EA, 240 survived (79.7%). Of these 301, 117 (38.9%) had no associated anomalies. Of the total 61 deaths, 59% (36/61) were in patients with cardiac anomalies and 44% (27/61) were in those with >2 associated anomalies. The mortality rate was highest in those with >2 anomalies 27/34 (79.4%), whereas survival was best in those without any associated anomalies 104/117 (88.9%)., Conclusions: Apart from other factors, an association of more than two system anomalies influence the mortality in oesophageal atresia.

Surgical repair of oesophageal atresia may result in anastomotic strictures. These strictures are often treated by balloon dilatation (BD) and currently balloon dilatation (fluoroscopic or endoscopic) is the preferred primary treatment method. Here we review the current evidence of the outcomes of balloon dilatation of anastomotic strictures secondary to surgical repair of oesophageal atresia. We searched the standard databases (January, 1960-May, 2012) to identify all studies that reported outcomes of balloon dilatation of anastomotic strictures secondary to surgical repair of oesophageal atresia in children. Data, reported as median (range), were analysed and compared. Outcomes were success of BD, number of BD sessions, number of oesophageal perforations, need for other surgical interventions and mortality. Five studies were found to be relevant (n = 139; 81 [58%] male children). The total number of dilatation sessions was 401 (2.9 dilatations per child patient). General anaesthesia was used in two (40%) studies; sedation in a further two (40%) studies and one (20%) study used a combination of both. The size of balloon catheter ranged from 4 mm to 22 mm. Seven perforations were reported (1.8% per dilatation session), of which only one (14%) required surgery. No deaths were recorded. Balloon dilatation for anastomotic strictures post-EA repair is safe, and associated with a low perforation and mortality rates. Most perforations are amenable to conservative management.