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Background: Genital malformations are frequently diagnosed in patients with VACTERL, but are currently not included in the acronym. This study aimed to analyze the frequency of genital anomalies in patients with esophageal atresia (EA) and/or anorectal malformation (ARM), with a subgroup analysis of children fulfilling the VACTERL criteria., Method: This was a cross-sectional retrospective analysis of two prospectively collected registries of patients operated on for ARM and EA between 2012 and 2022 at a specialized national center. Children were screened routinely for malformations according to the VACTERL acronym., Results: A total of 174 children were included in the study. VACTERL was diagnosed in 60 children (34%), while 114 children (66%) were defined as non-VACTERL. Genital malformations were diagnosed in 38% (23/60) of the children with VACTERL, and in 11% (13/114) of the children without VACTERL (p < 0.001). The presence of genital malformations correlated linearly with the number of diagnosed component features (CFs). In boys with VACTERL, the most common genital malformation was undescended testes present in 10/27 (21%) compared to 1/71 (1%) in non-VACTERL boys (p < 0.001). Müllerian duct anomalies were found in 26% of girls with VACTERL vs. 7% in non-VACTERL girls (p < 0.05)., Conclusion: There was a higher frequency of genital malformations in patients with VACTERL emphasizing the importance of genital assessment for these patients. We propose VACTERL-G as an extension of the current acronym aiming to reduce the risk of long-term morbidity due to delayed diagnosis of reproductive anomalies., Competing Interests: Conflicts of interest None., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Objective: To examine the association between nonsyndromic esophageal atresia with tracheoesophageal fistula (EA-TEF) and consanguinity., Study Design: A retrospective study comparing the incidence of EA-TEF between a low-consanguineous Jewish population and a high-consanguineous Bedouin population. All patients were treated at Soroka University Medical Center, the only tertiary medical center in southern Israel., Results: From 2000 to 2022, 579 130 children were born in southern Israel, and 386 915 (66.8%) were Jewish, and 192 215 were Bedouin Muslims. A total of 96 patients were diagnosed with EA-TEF; 83 of them were nonsyndromic. The incidence of EA-TEF was 1.66 cases per 10 000 live births and was statistically higher among the Bedouin population (3 vs 0.95 cases per 10 000 live births; P < .001). The consanguinity rate among the Bedouin group was higher compared with the Jewish (67.8% vs 0%; P < .001). There were no differences in other risk factors., Conclusions: The incidence of EA-TEF is higher among the Bedouin population that lives in the same geographic region and has the same medical access as the Jewish population, proposing consanguinity as a risk factor for EA-TEF., Competing Interests: Declaration of Competing Interest The authors declare no conflict of interest., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Objective:  Heterotopic pancreas (HP) is a condition in which there is well-differentiated pancreatic tissue that lacks any anatomic or vascular contact with the pancreatic gland. It normally arises from the stomach but can be found in other locations. Although it is usually asymptomatic, obstructive symptoms, bleeding, or malignant degeneration can occur. The incidence is very low, but it is significantly more common in patients with esophageal atresia (EA). The aim of this study is to evaluate the incidence of HP in patients with and without EA and to compare the results in both groups., Material and Methods:  We conducted a 2-year prospective study in pediatric patients who benefited from an upper gastrointestinal endoscopy. Patients were divided into two groups: group "A" comprised patients with EA and group "B" those without EA. The variables analyzed were the clinical presentation, presence of HP, location, associated malformations, genetic disorders, and management., Results:  A total of 192 consecutive patients were included in the study: 51 (26.6%) in group A and 141 (73.4%) in group B. Indications for endoscopy in group B were eosinophilic esophagitis in 37 (19.2%) patients, celiac disease in 23 (11.95%) patients, and other disorders in 81 (42.2%) patients. Gastric HP was found in seven patients, all of them in group A. All lesions were hosted in the prepyloric antrum. The prevalence of HP in groups A and B was 13.7 and 0%, respectively ( p  < 0.05). Female gender was predominant in patients with AE and HP, this result being statistically significant ( p  = 0.044). No other associated malformation or genetic syndrome studied showed association with HP. Only one patient debuted with upper gastrointestinal (GI) bleeding and required excision, while six patients were asymptomatic. The mean follow-up was 54 months (range: 45-78 months)., Conclusion:  The incidence of gastric HP is more common in patients with EA, with the female gender being a risk factor for their association. Active search and follow-up is recommended as it may become symptomatic anytime and need resection., Competing Interests: None declared., (Thieme. All rights reserved.)

Objective: To describe characteristics, treatments and clinical outcomes of patients with trisomy 18 and oesophageal atresia, using a nationwide database in Japan., Design: Descriptive study using a retrospective cohort., Setting: A nationwide inpatient database including 90% of hospitals with neonatal intensive care units in Japan., Patients: Patients hospitalised within a day after birth for both oesophageal atresia and trisomy 18 between July 2010 and March 2020., Interventions: Radical surgery for oesophageal atresia., Main Outcome Measures: Characteristics, treatment course and outcomes., Results: Among 271 patients with both oesophageal atresia and trisomy 18, 70 patients underwent radical surgery for oesophageal atresia. Patients who underwent radical surgery were less likely to have severe cardiac anomalies (17% vs 32%; p=0.020), but more likely to undergo cardiac surgery (21% vs 9.5%; p=0.012) than those who did not. The overall in-hospital mortality was lower (54% vs 79%; p<0.001) and the median age at death was higher (210 days vs 39 days; p<0.001) in patients who underwent radical surgery than the others. Postoperative mortality within 30 days after radical surgery was 5.7%. Patients who underwent radical surgery were likely to be discharged to home (50% vs 18%; p<0.001), whereas the age at home discharge (median 314 days vs 216 days; p=0.19) and the requirement for each home treatment did not differ significantly by radical surgery., Conclusion: This study provides information that will aid the clinical decision-making process for patients with oesophageal atresia and trisomy 18. Radical surgery may be a safe and feasible treatment option., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

This paper presented a national register for esophageal atresia (EA) started in January 2008. We report our experience about the conception of this database and its coordination. Data management and data quality are also detailed. In 2023, more than 2,500 patients with EA are included. Prevalence of EA in France was calculated at 1.8/10,000 live birth. Main clinical results are listed with scientific publications issued directly from the register., Competing Interests: None declared., (Thieme. All rights reserved.)

Background: Oesophageal atresia (OA) is one of the most common congenital gastrointestinal (GI) abnormalities. Due to advances in multidisciplinary care, early prognosis has improved with emphasis shifting to the long-term impact of this disease. Literature suggests a higher incidence of Barrett's and eosinophilic oesophagitis in these children, with an increased risk of oesophageal carcinoma. Guidelines for adults born with OA include routine endoscopy and lifelong screening of the upper gastrointestinal tract (GIT). Despite this, uncertainty remains regarding the necessity and frequency of endoscopic surveillance for children born with OA. We describe our endoscopic findings in children born with OA., Methods: A prospective analytic cohort study was undertaken, which included all children born with OA, that were followed-up in our unit between 2020 and 2022. History regarding feeding and GI symptoms were documented after which an endoscopy was performed., Results: During the study period, 37 endoscopies were performed in patients born with OA at a median age of 25 months. The most common clinical appearance on endoscopy was anastomotic strictures followed by oesophagitis. Twelve patients had biopsies taken, with abnormal histology in all but one patient. The most common histological finding was oesophagitis with lymphocytes and chronic gastritis. Two patients had Helicobacter Pylori infection, and one had findings suggestive of eosinophilic oesophagitis., Conclusion: All patients with a clinical indication for an endoscopy had abnormal clinical or histological findings, thus concurring with the literature in highlighting the need for regular endoscopy. We recommend regular clinical follow-up and endoscopic surveillance if clinically indicated for children born with OA., (Copyright© Authors.)

Introduction: To determine the incidence, management and outcomes of esophageal atresia/tracheo-esophageal fistula (EA/TEF) over a 15-y period in South Africa., Methods: A retrospective chart review of neonates with EA/TEF presenting at the main tertiary referral hospital in the KwaZulu-Natal province between 2002 and 2017 was conducted. Data collection comprised patient and maternal demographics, clinical presentations, laboratory and radiologic investigations, surgical procedures, and outcomes. A multivariate logistic regression determined the risk factors associated with mortality., Results: Among 180 neonates, mean (SD) age of diagnosis was four (three) days postnatal with Gross Type C (n = 165, 92%) being the most common and the incidence was one per 10,000 live births. Majority were born term (n = 95, 53%) at peripheral hospitals (n = 167, 93%) with a mean birth weight of 2369 (736) grams. Overall HIV exposure rate was 27% (n = 48). Most (n = 138, 77%) patients presented with established pneumonia, 44% (n = 61) of whom required prolonged (>7 d) ventilator support. The median (IQR) hospital stay was 11 (8-20) d. Overall survival rate was 70% (n = 126). Birth weight <1500 g, life threatening anomalies, ventilation >30 d and postoperative sepsis contributed to mortality., Conclusions: Incidence, disease types and presentations were similar to developed countries. Despite advances in technology and neonatal care in Africa, EA/TEF surgical outcomes remain suboptimal likely due to caregivers' inability to care for these infants in disadvantaged socioeconomic circumstances with poor sanitation, etc. Research is needed to identify strategies tailored for disadvantaged communities which may contribute to improved outcomes in the perioperative and postoperative period., (Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: An Oesophageal atresia (OA) affects almost 1 in 3500 newborn and its aetiology for development between 4th to 6th pregnancy weeks had not yet been clarified., Methods: This retrospective, observational control group-supported (n = 30) examination was performed with OA mothers (n = 20) who were interrogated via specialized questionnaires and interviews to evaluate data on medical pregnancy history including hormonal treatment, changing of life habits (nutrition, diet, medication), drug consumption (alcohol, smoking, illicit drugs) as well as exposure to nuclear waste or poisonous substance exposition. Aim was to assess risk factors during pregnancy and in particular in the first trimester., Results: The body mass index (BMI) prior pregnancy of OA mothers was significant lower (p = 0.022) and the number of mothers with obesity (BMI>30) did not differ to control group mothers (p = 0.081). They had additionally more pre-existing illnesses (p = 0.009) and allergies (p = 0.001). Control group mothers changed dietary habits with higher meat (p = 0.032) and caffeine intake (p = 0.012) compared to OA mothers, which had higher rates of abnormalities during pregnancy (p < 0.001) and 38.7% of them suffered of ≥1 abortion in the past. The ethnical background of OA mothers was more often German (p < 0.033), while OA fathers had a lower socioeconomic status (p = 0.039)., Conclusion: Maternal factors like previous abortions, obesity and immunological predispositions like existence of allergies combined with increased daily dairy consumption influenced obviously the occurrence of OA., Competing Interests: Declaration of competing interest The authors have no conflict of interest to declare (financial or non-financial)., (Copyright © 2023 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

Background: Children with long-gap esophageal atresia (LGEA) face a high risk of digestive and respiratory morbidity, but their mental health outcomes have not been investigated. We aimed to identify the prevalence of mental health problems in children with LGEA, associated factors and health-related quality of life (HRQOL)., Methods: Twenty-six children with LGEA aged 3-17 were recruited nationwide in Sweden. One of their parents and adolescents aged 11-17 completed information on the child's mental health (Strength and Difficulties Questionnaire), generic (PedsQL 4.0) and condition-specific HRQOL (EA-QOL). Parents gave information on current child symptomatology. Mental health level was determined using validated norms; abnormal≥90 percentile/borderline≥80 percentile/normal. Elevated levels were considered borderline/abnormal. Data were analyzed using descriptives, correlation and Mann-Whitney-U test. Significance level was p < 0.05., Results: Twelve children with LGEA aged 3-17 (46%) had elevated scores of ≥1 mental health domain in parent-reports, whereas 2 adolescents (15%) in self-reports. In parent-reports, 31% of the children had elevated levels of peer relationship problems, with associated factors being child sex male (p = 0.037), airway infections (p = 0.002) and disturbed night sleep (p = 0.025). Similarly, 31% showed elevated levels of hyperactivity/inattention, and associated factors were male sex (p = 0.005), asthma (p = 0.028) and disturbed night sleep (p = 0.036). Elevated levels of emotional symptoms, seen in 20%, were related to swallowing difficulties (p = 0.038) and vomiting problems (p = 0.045). Mental health problems correlated negatively with many HRQOL domains (p < 0.05)., Conclusions: Children with LGEA risk mental health difficulties according to parent-reports, especially peer relationship problems and hyperactivity/inattention, with main risk factors being male sex, airway problems and sleep disturbances. This should be considered in follow-up care and research, particularly since their mental health problems may impair HRQOL., Levels of Evidence: Prognosis study, LEVEL II., Competing Interests: Conflict of interest None., (Copyright © 2022 Elsevier Inc. All rights reserved.)

The combination of duodenal atresia (DA) and esophageal atresia (EA) is very rare. With improvements in prenatal sonography and the use of fetal magnetic resonance imaging (MRI), these malformations can be diagnosed in a more accurate and timely manner; polyhydramnios remains the most common sign despite having a low specificity. The high rate of associated anomalies (in 85% of cases) can also impact neonatal management and increase the morbidity rate; thus, it is of paramount importance to look for every possible associated malformation, such as VACTERL and chromosomic anomalies. The surgical management of this combination of atresias is not well defined and changes according to the patient's clinical status, the type of EA, and the other associated malformations. Management ranges from a primary approach for one of the atresias with delayed correction of the other (56.8%) to a simultaneous repair of both atresias (33.8%) with or without gastrostomy, or total abstention (9.4%). We suggest that a simultaneous approach can be safely performed on patients in good physical condition, with a birth weight over 1500 g, and with no major respiratory distress; this method begins by closing the tracheoesophageal fistula to protect the lung and then repairing the DA. The mortality rate has decreased over the years, dropping from 71% before 1980 to 24% after 2001. In this review, we present the available evidence on these conditions, focusing mostly on the epidemiology, prenatal diagnosis, neonatal management strategies, and outcome, with the aim of determining how the different clinical features and surgical approaches may impact on morbidity and mortality., Competing Interests: Declaration of Competing Interest None., (Copyright © 2023 French Society of Pediatrics. Published by Elsevier Masson SAS. All rights reserved.)

Knowledge of neurodevelopmental disorders such as attention deficit/hyperactivity disorder (ADHD), autism spectrum disorders (ASD) and intellectual disability (ID) in patients with esophageal atresia (EA) is scarce. The aims of this study were to investigate the prevalence and risk of ADHD, ASD and ID in individuals with EA. Data were obtained from four longitudinal population-based registries in Sweden and analyzed using Cox proportional hazards regression. Patients with EA born in Sweden in 1973-2018 were included together with five controls for each individual with the exposure matched on sex, gestational age at birth, birth year and birth county. Individuals with chromosomal aberrations and syndromes were excluded. In total, 735 individuals with EA and 3675 controls were included. Median age at time of the study was 20 years (3-48). ASD was found in 24 (3.9%), ADHD in 34 (5.5%) and ID in 28 (4.6%) individuals with EA. Patients with EA had a 1.66 times higher risk of ASD (95% confidence interval [CI], 1.05-2.64) and a 3.62 times higher risk of ID (95% CI, 2.23-5.89) compared with controls. The risk of ADHD was not significantly increased. ADHD medication had been prescribed to 88.2% of patients with EA and ADHD and to 84.5% of controls with ADHD. Individuals with EA have a higher risk of ASD and ID than individuals without the exposure. These results are important when establishing follow-up programs for children with EA to allow timely detection and consequentially an earlier treatment and support especially before school start., (© The Author(s) 2022. Published by Oxford University Press on behalf of International Society for Diseases of the Esophagus. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Introduction: Endoscopic surveillance guidelines for patients with repaired esophageal atresia (EA) rely primarily on expert opinion. Prior to embarking on a prospective EA surveillance registry, we sought to understand EA surveillance practices within the Eastern Pediatric Surgery Network (EPSN)., Methods: An anonymous, 23-question Qualtrics survey was emailed to 181 physicians (surgeons and gastroenterologists) at 19 member institutions. Likert scale questions gauged agreement with international EA surveillance guideline-derived statements. Multiple-choice questions assessed individual and institutional practices., Results: The response rate was 77%. Most respondents (80%) strongly agree or agree that EA surveillance endoscopy should follow a set schedule, while only 36% claimed to perform routine upper GI endoscopy regardless of symptoms. Many institutions (77%) have an aerodigestive clinic, even if some lack a multi-disciplinary EA team. Most physicians (72%) expressed strong interest in helping develop evidence-based guidelines., Conclusions: Our survey reveals physician agreement with current guidelines but weak adherence. Surveillance methods vary greatly, underscoring the lack of evidence-based data to guide EA care. Aerodigestive clinics may help implement surveillance schedules. Respondents support evidence-based protocols, which bodes well for care standardization. Results will inform the first multi-institutional EA databases in the United States (US), which will be essential for evidence-based care., Level of Evidence: This is a prognosis study with level 4 evidence., Competing Interests: Conflicts of interest Author has no competing interests or financial conflicts. This study has institutional financial support only that has allowed access to research staff., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Increased risk of scoliosis and musculoskeletal abnormalities in adolescents with esophageal atresia (EA) is reported, but the impact of these abnormalities on physical fitness and motor skills are not known., Methods: Scoliosis was assessed radiographically and shoulder and chest abnormalities by a standardized protocol. Physical fitness was evaluated with Grippit, Six-minute walk test, and International Physical Activity Questionnaire and motor skills by Motor Assessment Battery for Children., Results: Sixty-seven EA adolescents median 16 (13-20) years participated. The prevalence of significant scoliosis (≥ 20º) was 12% (8/67) whereas 22% (15/67) had mild scoliosis (10-19º). Vertebral anomalies occurred in 18/67 (27%), eight of them (44%) had scoliosis. The majority of adolescents (15/23) with scoliosis did not have vertebral anomalies. Musculoskeletal abnormalities were detected in 22-78%. Balance problems occurred three times more frequently than expected (44% vs. 15%, p = 0.004). Submaximal exercise capacity was significantly reduced compared to reference values (p < 0.001). Scoliosis ≥ 20º was related to reduced physical activity (p = 0.008), and musculoskeletal abnormalities to reduced physical activity and impaired motor skills (p = 0.042 and p < 0.038, respectively)., Conclusions: Significant scoliosis was diagnosed in 12% of the EA adolescents and related to reduced physical activity. Musculoskeletal abnormalities identified in more than half of the patients, were related to reduced physical activity and impaired motor skills, and exercise capacity was significantly below reference group. EA patients with and without vertebral anomalies need health-promoting guidance to prevent impaired motor skills and consequences of reduced physical activity., Level of Evidence: Prognostic Study, Level II., Competing Interests: Declaration of interest None, (Copyright © 2022 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Esophageal atresia (EA) is a rare malformation with a wide range of co-morbidity and associated malformations impairing weight gain and growth. The aim of this study was to calculate specific percentiles for body weight and height for children born with esophageal atresia according to sex from birth to the age of 6 years, accounting for prematurity and presence of congenital heart disease (CHD)., Methods: Data was extracted from an anonymized voluntary national registry for patients born with esophageal atresia between 2001 and 2021, from birth until the age of six years. Missing values were imputed using a multiple imputation approach. In premature infants, chronological age was corrected for gestational week until the age of one year. The impact of sex and additional congenital heart disease on weight gain and growth was analysed using quartile regression models., Results: In total, 1812 examinations of 485 patients were considered and 1232 examinations of 301 patients were finally included. Most data was available for children at birth and during the first year of life. Body weight was imputed for 3.3% and height for 12.5% of examinations. The mean body weight-for-age and length-for-age at birth according to gestational age was lower in EA patients and median body weight developed along the tenth percentile compared to the general population. Median height-for-age was at the 50th percentile during the first months of life, before crossing to lower percentiles before the age of one year. CHD had an additional negative impact on growth and weight gain, especially during the first year of life., Conclusions: Children with EA had a reduced bodyweight and -height compared to the general population. Therefore, specific percentile curves are helpful to evaluate growth and development. Especially during the first year of life, particular attention is necessary and complications leading to a reduced calorie intake should be treated without delay to promote timely development and growth. Cardiac co-morbidities may further compromise weight gain in these patients, implying that such patients should be under even closer surveillance., (© 2023. The Author(s).)

Objective: Tracheoesophageal fistula and esophageal atresia (TEA) and laryngeal cleft (LC) can coexist in some patients. The surgery-specific success rate of LC repair in children with associated TEA has not been well described. The aim of the study is to determine if the history of TEA alters the LC repair outcomes., Study Design: Case series with chart review., Setting: Single-institution academic medical center., Method: A retrospective review was conducted of patients with LC with and without TEA repair between January 2001 and November 2020. Data collected and analyzed included demographics and clinical characteristics, LC type, and LC with TEA timing of repairs., Results: An overall 282 patients met the inclusion criteria of LC repair: LC (n = 242, 85.8%) and LC + TEA (n = 40, 14.2%). Revision repair was required in 43 patients (15.2%) with 8 (2.8%) needing a second revision repair. The first LC revision rate in the LC group was 36/242 (14.9%) as compared with 7/40 (17.5%) in the LC + TEA group (P = .67). The second LC revision rate in the LC and LC + TEA groups was 7 (2.9%) and 1 (2.4%), respectively. The median time to revision was 5.1 months (interquartile range, 3.45-10.6) in the LC group as compared with 29.2 months (interquartile range, 4.8-44.2) in the LC + TEA group (P = .06)., Conclusion: The incidence of TEA and LC was 14.2% in our study. Based on our findings, history of TEA repair is not associated with a higher revision rate vs LC alone. The history of TEA repair did not alter the outcomes of LC repair., (© 2022 The Authors. Otolaryngology-Head and Neck Surgery published by Wiley Periodicals LLC on behalf of American Academy of Otolaryngology-Head and Neck Surgery Foundation.)

Background/Purpose Oesophageal Atresia (OA) is associated with co-existent anomalies. There is a controversy of literature pertaining to the risk (s) of intestinal malrotation. In order to guide management we critically evaluate the incidence of IM anomalies in OA newborns. Design MEDLINE and EMBASE databases were searched using keywords "(O)Esophageal Atresia and Malrotation/Associated Abnormalities/Associated Anomalies". Full texts of articles were screened if manuscripts exclusively reported patients with OA malrotation and/or associated anomalies. Larger case series (> 10patients) were included if abstract (s) showed that associated anomalies were systematically assessed. Full eligibility criteria required at least one case of malrotation in an OA index case. Data were collected on article type, number of patients and method (s) of diagnosis. Results 632 abstracts were screened of which 158 papers were analysed based on inclusion criteria-30 manuscripts documented the incidence (%) of malrotation. Incidence rate (s) were 0.5-13%. Malrotation was observed to have a higher incidence (10-44%) in OA babies with other gastrointestinal anomalies (VACTERL). Conclusion Newborns with OA appear to be at a higher risk (%) of having intestinal malrotation anomalies than healthy babies. Prospective studies are required to accurately quantify and define the ' true incidence ' of this association. Given the potential lethal consequences of midgut volvulus screening may be justified in OA babies. Consensus guidelines (DELPHI) exploring surgeons attitudes with regards management of ' asymptomatic malrotation ' disorders in OA newborns may further guide best practice., (Copyright © 2021. Published by Elsevier Inc.)

Background: Given the high prevalence and complication risks of acid gastroesophageal reflux (GERD) in the first months of life in infants with esophageal atresia, the ESPGHAN/NASPGHAN consensus statement recommends systematic treatment with proton pump inhibitors (PPIs) until the age of 1 year and checking for acid GERD thereafter. However, these recommendations have not been evaluated., Methods: This prospective study was conducted from 2007 to 2016. We evaluated the prevalence of acid GERD in 100 consecutive infants presenting with esophageal atresia/tracheoesophageal fistula after the age of 18 months when PPI treatment was stopped. The diagnosis of acid GERD was based on positive pH-metry and/or evidence of complications (e.g., peptic esophagitis, need for jejunal nutrition, or antireflux surgery). Those with acid GERD at a median age of 18 months received a control examination every year or adapted to their clinical situation., Results: The prevalence rates of acid GERD were 64.3% at 18 months and 22.8% at the last follow-up (median age 65 months).There is no risk factor for acid GERD identified., Conclusions: This study shows a high prevalence of acid GERD in late infancy and supports the recommendation of systematic checking for acid GERD when treatment with PPI is stopped., Impact: Acid gastroesophageal reflux disease (GERD) is a frequent complication of esophageal atresia in infants. The ESPGHAN/NASPGHAN consensus, which is based on expert opinion, recommends systematic treatment of children with PPI until the age of 1 year. The prevalence rates of acid GERD were 64.3% at 18 months and 22.8% at the last follow-up. This study shows a high prevalence of acid GERD in late infancy and supports the recommendation of systematic checking for acid GERD when treatment with PPI is stopped., (© 2021. The Author(s), under exclusive licence to the International Pediatric Research Foundation, Inc.)

Aim of the Study: An assessment of the clinical data and outcome of patients with oesophageal atresia (OA) with very low birth weight (VLBW) was completed., Method: With ethical consent, we reviewed the records of 327 successive patients with OA from 1980 to 2020. Main outcome measures survival and oesophageal repair were compared between patients with VLBW(≤1500 g) and with BW>1500 g., Results: Thirty-four (10%) patients had VLBW. Gross types of OA in VLBW were similar as in other patients: A (15%/7%), B (3%/3%), C (78%/82%), D (3%/4%), E (0%/7%), F (0%/1%) (p = 0.16-0.99). In VLBW the incidence of congenital heart disease (CHD) (47%) and trisomy 13/18 and Cri du Chat (15%) were higher than in BW>1500 g (23% and 1%), (p = 0.001 both). In VLBW one-month mortality was 35% vs 4% in patients with BW>1500 g (p < 0.001), overall mortality 56% and 8% (p < 0.001), respectively. Cause of one-month mortality in VLBW (12 patients) were CHD w/wo chromosomal abnormality (n = 7), cerebral hemorrhage (n = 2), gastric perforation (n = 1), anastomotic leakage (n = 1) and pulmonary hemorrhage (n = 1). Of VLBW patients 79% and of other patients 99% underwent oesophageal repair (p < 0.001). Repair in VLBW patients included early (n = 18) or delayed (n = 5) end-to-end anastomosis and reconstruction (n = 4). Anastomotic complications occurred in 24% of patients with VLBW and in 17% with BW>1500 g, (p = 0.31). From 1980-2000 to 2001-2020 survival in VLBW changed from 11% to 81% (p = 0.002). During 2001 - 2020 all VLBW patients underwent repair., Conclusion: OA with VLBW had high incidence of life-threatening associated anomalies and decreased survival. Recently survival and rate of oesophageal repair have improved significantly., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Introduction:  Esophageal atresia (EA) is a rare malformation that often requires a series of procedures, including surgical primary anastomosis, staged repair, and endoscopic procedures. Actual numbers and trends in interventions and variety in treatment strategies remain unclear., Materials and Methods:  Data from the German federal bureau of statistics containing all EA-related inpatient procedures encoded from 2005 until 2018 were analyzed for children during the first year of life. The sum of esophageal anastomoses and replacements was used to calculate an estimate of incidence of EA., Results:  Over 14 years, 12,627,888 inpatient cases were recorded in infants in Germany. The mean incidence of EA was 1 per 4,217 live births. On average, 163.3 (95% confidence interval [CI]: 150.8-176.1) esophageal anastomoses, 11.2 (95% CI: 8.7-13.7) esophageal lengthening procedures, and 6.7 (95% CI: 5.42-8.00) esophageal replacements were recorded annually. Overall, 187.8 (95% CI: 147.1-200.4) endoluminal treatments (ballon dilatation, bougienage, stent placement, or injection) were performed per 100 anastomoses. Over the years, bougienage was increasingly replaced by ballon dilatation as primary treatment. Boys had a significantly higher number of esophageal procedures than girls, but the incidence of endoscopic treatments in relation to anastomoses was the same for both genders., Conclusion:  The low incidence of EA in relation to a relatively large number of units treating those patients in Germany may pose challenges for maintaining competency and training of all specialists involved. The number of esophagoscopic treatments for esophageal stricture per anastomosis is lower than previously estimated., Competing Interests: O.J.M., reports grants from Innovationsfond GBA, Germany: TIC-PEA Study (Telemedical interdisciplinary care of patients with esophageal atresia), research grant; payment or honoraria for lectures from Holister-Dansac: lectures for pediatric stoma care—Honorarium; payment from several sources concerning medicolegal expert testimony: Bayerische Landesärztekammer, Landgericht Ansbach, Landgericht Koblenz; and Illuminati—patent pending., (Thieme. All rights reserved.)

Background: Esophageal atresia is a complex esophageal malformation with an incidence of 1 in 3,500-4,000 live births, and it usually occurs together with anomalies in other systems or chromosomes. This study aimed to investigate the short-term and long-term results of cases of esophageal atresia retrospectively in our institution and to analyze the factors affecting the outcome., Methods: Charts of the patients managed for esophageal atresia in our tertiary pediatric surgery department were investigated retrospectively. Statistical analysis was performed to determine the risk factors for morbidity and mortality., Results: One hundred and thirteen (95.8%) of 118 cases underwent a single-stage or staged esophagoesophagostomy procedure. In only five of the 40 patients with a long gap between the two atretic ends was an esophageal replacement procedure required. The most common early and late complications were anastomotic stenosis (41.6%) and gastroesophageal reflux (44.9%). In logistic regression analysis, the birthweight (OR [95% CI] = 0.998 [0.997, 0.999], P = 0.001) and preoperative inotrope requirement (OR [95% CI] = 13.8 [3.6-53.3], P < 0.001) were the two risk factors in the mortality prediction model obtained by multivariate analysis. The gap length between the two atretic ends (OR [95% CI] = 1.436 [1.010, 2.041], P = 0.044) and the number of sutures for anastomosis (OR [95% CI] = 1.313 [1.042, 1.656], P = 0.021) were the two risk factors in the gastroesophageal reflux prediction model obtained by multivariate analysis., Conclusions: Our study's early and late complication rates were like those found in other studies. Identifying risk factors would be beneficial and might help reduce the severity of potential complications in esophageal atresia patients. Prospective studies on large patient series would help develop registry-based, standardized management protocols., (© 2022 Japan Pediatric Society.)

Introduction: With advances in surgical and neonatal care, the survival of patients with oesophageal atresia (OA) has improved over time. Whereas a number of OA-related conditions (delayed primary anastomosis, anastomotic stricture and oesophageal dysmotility) may have an impact on feeding development and although children with OA experience several oral aversive events, paediatric feeding disorders (PFD) remain poorly described in this population. The primary aim of our study was to describe PFD in children born with OA, using a standardised scale. The secondary aim was to determine conditions associated with PFD., Methods: The Feeding Disorders in Children with Oesophageal Atresia Study is a national cohort study based on the OA registry from the French National Network. Parents of children born with OA between 2013 and 2016 in one of the 22 participating centres were asked to complete the French version of the Montreal Children's Hospital Feeding Scale., Results: Of the 248 eligible children, 145 children, with a median age of 2.3 years (Q1-Q3 1.8-2.9, min-max 1.1-4.0 years), were included. Sixty-one children (42%) developed PFD; 13% were tube-fed (n=19). Almost 40% of children with PFD failed to thrive (n=23). The presence of chronic respiratory symptoms was associated with the development of PFD. Ten children with PFD (16%) had no other condition or OA-related complication., Conclusion: PFD are common in children with OA, and there is no typical profile of patients at risk of PFD. Therefore, all children with OA require a systematic screening for PFD that could improve the care and outcomes of patients, especially in terms of growth., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: To know the cardiac malformations frequency associated with esophageal atresia and its type in patients of the Children's Specialties Hospital of Chihuahua as well as related sociodemographic characteristics., Method: The epidemiology, clinic and evolution of patients with esophageal atresia diagnosis who were admitted to this hospital for a period of two years were studied. Variables such as sex, gestational age, birth weight, Apgar score, atresia type, associated congenital malformations, hospital complications and parental related aspects were analyzed., Results: Twelve patients were studied, 50% of them were male, most of them were products of term pregnancies with adequate birth weight. There were mestizo ethnicity prevalence, young mothers children with a medium socio-economic level, without geographical predominance. 82% of the cases corresponded to type III esophageal atresia, the most frequent congenital malformations associated were cardiac in 83% of which 90% corresponded to atrial septum defects., Conclusions: Esophageal atresia is a relatively common congenital malformation of multifactorial etiology. A complete approach to patients with this pathology is necessary to identify a concomitant illness and provide adequate treatment., (Copyright: © 2022 Permanyer.)

Tracheoesophageal Fistula (TOF) is a congenital anomaly for which the cause is unknown in the majority of patients. OA/TOF is a variable feature in many (often mono-) genetic syndromes. Research using animal models targeting genes involved in candidate pathways often result in tracheoesophageal phenotypes. However, there is limited overlap in the genes implicated by animal models and those found in OA/TOF-related syndromic anomalies. Knowledge on affected pathways in animal models is accumulating, but our understanding on these pathways in patients lags behind. If an affected pathway is associated with both animals and patients, the mechanisms linking the genetic mutation, affected cell types or cellular defect, and the phenotype are often not well understood. The locus heterogeneity and the uncertainty of the exact heritability of OA/TOF results in a relative low diagnostic yield. OA/TOF is a sporadic finding with a low familial recurrence rate. As parents are usually unaffected, de novo dominant mutations seems to be a plausible explanation. The survival rates of patients born with OA/TOF have increased substantially and these patients start families; thus, the detection and a proper interpretation of these dominant inherited pathogenic variants are of great importance for these patients and for our understanding of OA/TOF aetiology.

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.)

Purpose: Caring for a chronically ill child can be stressful and requires additional care from parents. Parental mental health and stress may impact both parental and child wellbeing, leading to maladaptive parenting practices, and interference with illness management. The aim of the study was to explore the levels of anxiety and depression in parents of children born with esophageal atresia (EA) and to investigate whether parental anxiety and depression were associated with child's medical characteristics or with parental factors., Design and Methods: An international online survey was developed and disseminated to parents of children born with EA, aged 0-12 years, via EA patient charity social network sites. A one-way between subjects ANOVA and post hoc statistical analyses were used to examine differences in mean scores of parental anxiety and depression between sub-groups that described child's medical characteristics and parental characteristics., Results: A total of 240 parents completed the survey from 17 different countries. Of these, nearly 70% self-reported raised levels of anxiety, whilst 38% self-reported raised levels of depression. Statistically significant differences in mean scores of parental anxiety and depression were found between sub-groups that described the child's feeding problems, parental age, and perceived support for caring, caring stress and money matters., Conclusion: Child's feeding problems related to esophageal atresia, and parental factors, such as younger age, perceived lack of support for caring, caring stress, and money worries, may contribute to the increased levels of parental anxiety and depression in parents of children born with EA., Competing Interests: Declaration of Competing Interest We have no known conflict of interest to disclose., (Copyright © 2021 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Esophageal atresia (EA) affects around 2.3-2.6 per 10,000 births world-wide. Infants born with this condition require surgical correction soon after birth. Most survival studies of infants with EA are locally or regionally based. We aimed to describe survival across multiple world regions., Methods: We included infants diagnosed with EA between 1980 and 2015 from 24 birth defects surveillance programs that are members of the International Clearinghouse for Birth Defects Surveillance and Research. We calculated survival as the proportion of liveborn infants alive at 1 month, 1- and 5-years, among all infants with EA, those with isolated EA, those with EA and additional anomalies or EA and a chromosomal anomaly or genetic syndrome. We also investigated trends in survival over the decades, 1980s-2010s., Results: We included 6,466 liveborn infants with EA. Survival was 89.4% (95% CI 88.1-90.5) at 1-month, 84.5% (95% CI 83.0-85.9) at 1-year and 82.7% (95% CI 81.2-84.2) at 5-years. One-month survival for infants with isolated EA (97.1%) was higher than for infants with additional anomalies (89.7%) or infants with chromosomal or genetic syndrome diagnoses (57.3%) with little change at 1- and 5-years. Survival at 1 month improved from the 1980s to the 2010s, by 6.5% for infants with isolated EA and by 21.5% for infants with EA and additional anomalies., Conclusions: Almost all infants with isolated EA survived to 5 years. Mortality was higher for infants with EA and an additional anomaly, including chromosomal or genetic syndromes. Survival improved from the 1980s, particularly for those with additional anomalies., (© 2021 Wiley Periodicals LLC.)

Background: Concern exists that patients born with oesophageal atresia (OA) may be at high risk for Barrett's oesophagus (BO), a known malignant precursor to the development of oesophageal adenocarcinoma. Screening endoscopy has a role in early BO identification but is not universal in this population. This study aimed to determine prevalence of BO after OA repair surgery, to quantify the magnitude of this association and inform the need for screening and surveillance., Methods: A systematic review, undertaken according to PRISMA guidelines, was preregistered on PROSPERO (CRD42017081001). PubMed and EMBASE were interrogated using a standardized search strategy on 31 July 2020. Included papers, published in English, reported either: one or more patients with either BO (gastric/intestinal metaplasia) or oesophageal cancer in patients born with OA; or long-term (greater than 2 years) follow-up after OA surgery with or without endoscopic screening or surveillance., Results: Some 134 studies were identified, including 19 case reports or series and 115 single- or multi-centre cohort studies. There were 13 cases of oesophageal cancer (9 squamous cell carcinoma, 4 adenocarcinoma) with a mean age at diagnosis of 40.5 (range 20-47) years. From 6282 patients under long-term follow-up, 317 patients with BO were reported. Overall prevalence of BO was 5.0 (95 per cent c.i. 4.5 to 5.6) per cent, with a mean age at detection of 13.8 years (range 8 months to 56 years). Prevalence of BO in series reporting endoscopic screening or surveillance was 12.8 (95 per cent c.i. 11.3 to 14.5) per cent., Conclusion: Despite a limited number of cancers, the prevalence of BO in patients born with OA is relatively high. While limited by the quality of available evidence, this review suggests endoscopic screening and surveillance may be warranted, but uncertainties remain over the design and effectiveness of any putative programme., (© The Author(s) 2021. Published by Oxford University Press on behalf of BJS Society Ltd.)

The aim of this study was to evaluate the outcome of esophageal atresia in Germany in a retrospective observational study of a large cohort. Data from the major health insurance company in Germany, which covers approximately 30% of German patients, were analyzed. All patients born and registered between 2009 and 2013 with a diagnosis of esophageal atresia at first admission to the hospital were included. Mortality was analyzed during the first year of life. We identified 287 patients with esophageal atresia, including 253 with and 34 without tracheoesophageal fistula. Associated anomalies were found in 53.7% of the patients; the most frequent were cardiac anomalies (41.8%), anomalies of the urinary tract (17.4%), and atresia of the colon, rectum, and anus (9.4%). Forty-one patients (14.3%) had a birth weight <1500 g. Seventeen patients (5.9%) died before surgery. Gastrostomy was performed during the index admission in 70 patients (25.9%). The reconstruction of the esophageal passage was performed in 247 patients (93.9%). Forty-eight percent of the patients who underwent an operation required dilatation. The mortality rate in the patients who underwent an operation was 10.4%. These results from Germany correspond to the international results that have been reported. The number of dilatations was in the middle of the range of those reported in the literature; the overall mortality rate was in the upper portion of the range of the international rates. Efforts should be made to establish a clinical registry to measure and improve the quality of care for this and other rare conditions., (© The Author(s) 2020. Published by Oxford University Press on behalf of International Society for Diseases of the Esophagus. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Background: Esophageal atresia with tracheoesophageal fistula (EA/TEF) is associated with many congenital and vascular malformations; however, reports utilizing computed tomography (CT) and computed tomography angiography (CTA) are limited. The objective of this study is to review CT scans of the chest from patients with EA/TEF and report their pulmonary and vascular findings., Methods: We completed a retrospective chart review of children with congenital EA/TEF evaluated in the aerodigestive clinic at Children's Hospital Colorado. Results of the most recent CTA or CT of the chest were investigated. Demographics, medical conditions, and bronchoscopy findings were also recorded. The ratio of tracheal lumen area between inspiratory and expiratory CTA images was measured., Results: Of the patients with congenital EA/TEF seen in the program, 47 patients had a chest CT available for review. Eight patients (17%) had bronchiectasis. Of the contrast CT scans, 15 (58%) had a vascular abnormality and 16 (62%) demonstrated tracheal compression (38% at the level of the innominate artery, 35% from other structures). Nineteen of the CTAs had volumetric expiratory images of the trachea to evaluate tracheomalacia. The mean expiratory:inspiratory area was 0.57 (SD ± 0.23) at the level of the innominate., Conclusion: Patients with EA/TEF frequently have vascular abnormalities that may alter airway mechanics as well as pulmonary comorbidities that may affect long-term management. For patients experiencing persistent respiratory symptoms, CTA of the chest should be considered adjunct to bronchoscopy to help with medical and surgical management of these children., (© 2020 Wiley Periodicals LLC.)

Introduction:  We aimed to describe the prevalence of observable feeding difficulties during mealtimes among children with repaired esophageal atresia (EA) and to determine their early predictors., Materials and Methods:  A survey, based on parents' reports and concerning difficulties in EA children's nutritional intake, was performed with the help of 114 families of 2 to 17-year-old EA patients. Neonatal and clinical/surgical data were collected from medical records. Comparisons were made of the prevalence n (%) of feeding difficulties between children in three age groups (2 to 7 years, 8 to 12 years, or 13 to 17 years of age) using the Mantel-Haenszel chi-square test. Logistic regression identified outcome predictors (odds ratio: 95% confidence interval). Predictors with p ≤ 0.1 in the univariable analysis were included in multiple regression analysis ( p  < 0.05)., Results:  Seventy-five percent of the young children aged 2 to 7, (median number of feeding difficulties: 2), 61% of school-aged children aged 8 to 12 (median number of feeding difficulties: 1), and 60% patients in the teenage group, aged 13 to 17, (median number of feeding difficulties: 1), reported feeding difficulties. Surgical complications after EA repair independently predicted children having a gastrostomy ( p ≤ 0.01), using a food infusion pump ( p ≤ 0.01), taking small portions to facilitate eating ( p  = 0.01), and needing >30 minutes to finish a main meal ( p  = 0.02). Congenital independent predictors were VACTERL, low birth weight, and preterm birth., Conclusion:  Parentally observed feeding difficulties were commonly reported during early childhood, although prevalence decreases in older age groups. Several congenital and surgical factors were identified as independent predictors of complicated nutritional intake patterns., Competing Interests: None declared., (Thieme. All rights reserved.)

Esophageal atresia with/without tracheoesophageal fistula (EA/TEF) is a congenital digestive tract anomaly that represents a major therapeutic challenge. Postoperative digestive morbidities such as gastroesophageal reflux disease (GERD) and esophageal stricture are common. The aim of this study was to identify the incidence of and potential risk factors for digestive morbidities after EA/TEF repair. We retrospectively reviewed all EA/TEF patients who underwent repair at a single institution between January 1999 and December 2018, excluding patients who died prior to discharge. Patient demographics, perioperative management, and postoperative GERD and esophageal stricture rates were collected. We performed univariate and multivariate analyses to examine risk factors associated with postoperative GERD and esophageal stricture. The study enrolled 58 infants (58.6% male, 17.2% with type A EA/TEF, 62.1% with associated anomalies). Postoperative GERD occurred in 67.2% of patients and was the most common digestive morbidity. Esophageal stricture occurred in 37.9% of patients after EA/TEF repair. Multivariate analysis showed that long-gap EA/TEF and postoperative GERD were independent risk factors for esophageal stricture after repair surgery.Conclusion: The incidence of postoperative GERD and esophageal stricture was 67.2% and 37.9%, respectively. The risk factors for postoperative esophageal stricture were long-gap EA/TEF and postoperative GERD. What is Known: • EA/TEF is a congenital digestive tract anomaly with a high postoperative survival rate but can be complicated by many long-term morbidities. What is New: • Long-gap EA/TEF and postoperative GERD are risk factors of anastomotic stricture after repair. • Surgeons and pediatricians should be highly experienced in managing anastomotic tension and the GERD.

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: Tracheo-oesophageal anomaly is the abnormal communication between trachea and oesophagus. The most common type of tracheo-oesophageal anomaly is oesophageal atreasia with distal tracheo oesophageal fistula. Tracheo-oesophageal anomaly is a common neonatal problem requiring an urgent surgery. Tracheo-oesophageal anomaly can be diagnosed as an isolated malformation or as part of polymalformative syndrome with possible vertebrae anomalies also known as Vacterl syndrome. The aim of the study is to find out the prevalence of tracheo-oesophageal anomaly cases among neonates undergoing surgery in a tertiary care childrens hospital in Nepal., Methods: This is a descriptive cross sectional study conducted in a tertiary care children’s hospital in Nepal taking into account the medical records from period of 01 Jan, 2018 to Dec 31, 2019. Study population included the infants undergoing operative procedure in the operation theatre of Kanti Children’s Hospital. The calculated sample size was 306. Data was collected by retrospective chart review technique and listed in performa. Consecutive sampling technique was used. Thus collected data was entered in SPSS version 20 and necessary calculations were done., Results: The prevalence of tracheo-oesophageal anomalies was found out to be 30 (9.8%) at 95% confidence interval. The most common problem, with which infants were brought to Kanti Children’s Hospital, that required urgent neonatal surgical intervention was anorectal malformation constituting 94 (30.72%) of the surgeries followed by intestinal obstruction 76 (24.84%) which included duodenal atresia, jujunal atresia and ileal atresia., Conclusions: Tracheo-oesophageal anomaly constitutes a major portion of neonatal disease condition requiring surgery in Nepal.

Objective: Esophageal atresia with/without tracheo-esophageal fistula (EA/TEF) is more common among twins. The detection of polyhydramnios might be altered in twins, leading to EA/TEF underdiagnosis, prenatally. The aim of the study was to compare the prenatal manifestation of EA/TEF between twins and singletons., Methods: A 12-year study of EA/TEF cases was performed at a tertiary center. Cases exhibiting (a) small/absent stomach or (b) polyhydramnios were considered "suspected"; cases with (c) esophageal pouch were considered "detected." We compared the rate and timing of appearance of these signs between the groups., Results: There were 76 cases of EA/TEF, of which 17 were a co-twin. All twin pairs were EA/TEF discordant. The prevalence of EA/TEF at our center was 1:750 for twins (1:319 monochorionic and 1:1133 dichorionic) and 1:2399 for singletons. The rate of small/absent stomach, polyhydramnios and pouch in twins vs singletons was 23.5%, 47.1%, 29.4% and 39.7%, 72.4%,34.5%, respectively (P = .2, P = .09 and P = .7). Esophageal pouch was detected earlier in twins (P = .03). Twins were scanned more frequently (×1.8 times, P = .01)., Conclusion: EA/TEF is more prevalent in twins. Despite lower rate of polyhydramnios, twins were similarly detected prenatally as singletons, and this was accomplished earlier in pregnancy; perhaps reflecting more frequent scans., (© 2020 John Wiley & Sons Ltd.)

Purpose: To document the prevalence and variable appearance of gastric heterotopic pancreas (HP) in children undergoing upper gastrointestinal (GI) endoscopy., Methods: A prospective 4-year study of children undergoing flexible upper GI endoscopy in a single institution., Results: A total of 607 upper GI endoscopies were performed in 478 pediatric patients during the 4-year period. Eleven children (6 girls, 5 boys) aged 2.9 to 16.9 years had endoscopic features typical of gastric HP. All but one lesion was located in the gastric antrum and most appeared as an umbilicated submucosal nodule measuring 1-2 cm in diameter. Five of 13 children with repaired esophageal atresia (EA) and two of nine children with trisomy 21 had gastric HP. The prevalence of endoscopically visualized gastric HP in children without a history of EA or trisomy 21 was 1.1%., Conclusions: Gastric HP is present in about 1% of pediatric upper GI endoscopies. It is significantly more common in patients with EA and may also be associated with trisomy 21. Gastric HP typically appears as a single 1-2 cm antral submucosal nodule, usually with a central pit. Recognition of this lesion is important to avoid misdiagnosis and inappropriate treatment., Level of Evidence: Level II (diagnostic)., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Children with esophageal atresia (EA) have risk of gastroesophageal reflux disease (GERD), suggesting reflux monitoring for prompt management., Aim: To evaluate GERD in children with EA and specific symptom association from combined Video with Multichannel Intraluminal Impedance and pH (MII-pH) study., Methods: Children diagnosed with EA with suspected GERD and followed up at King Chulalongkorn Memorial Hospital between January 2000 and December 2018 were prospectively studied. All underwent esophagogastroduodenoscopy with esophageal biopsy and Video MII-pH study on the same day. Symptoms of GERD which included both esophageal and extra-esophageal symptom were recorded from video monitoring and abnormal reflux from MII-pH study based on the statement from the European Paediatric Impedance Group. Prevalence of GERD was also reported by using histopathology as a gold standard. Endoscopic appearance was recorded using Los Angeles Classification and esophagitis severity was graded using Esohisto criteria., Results: Fifteen children were recruited with age of 3.1 (2.2, 9.8) years (40%, male) and the common type was C (93.3%). The symptoms recorded were cough (75.2%), vomiting (15.2%), irritability or unexplained crying (7.6%) and dysphagia (1.9%) with the symptom-reflux association of 45.7%, 89%, 71% and 0%, respectively. There were abnormal endoscopic appearance in 52.9%, esophagitis in 64.7% and high reflux score in 47.1%. Video MII-pH study has high diagnostic value with the sensitivity, specificity and accuracy of 72.7%, 100% and 82.4%, respectively., Conclusion: Prevalence of GERD in children with EA was high. Video MII-pH study to detect GERD in children with EA had high diagnostic value with the trend of specific symptom association., Competing Interests: Conflict-of-interest statement: The authors of this manuscript having no conflict of interest to disclose., (©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.)

Context: Since the gap between two atretic segments of oesophagus is a critical determinant of prognosis for oesophageal atresia/tracheoesophageal fistula (EA/TEF), the search for a surrogate non-invasive pre-operative marker of long gap atresia continues., Aims: The purpose of the study was to compare the presence of normal and supernumerary ribs with length of EA and survival rates., Settings and Design: A prospective observational study was conducted at a tertiary care referral neonatal intensive care unit in North Karnataka, India, from January 2016 to June 2019., Subjects and Methods: Amongst babies with EA/TEF, pre-operative radiograph helped determine the number of ribs, and babies were divided into two groups; Group I: babies with 12 ribs and Group II: babies with supernumerary ribs., Statistical Analysis Used: Nominal variables were expressed as percentage and continuous variables as mean standard deviation. MedCalc software was used to compare proportions and means. A P < 0.05 was considered statistically significant., Results: Of the 61 cases, 51 were operated. Long gap EA was predominantly seen amongst babies in Group II (40% in Group II vs. 27% in Group I, P= 0.424). Survival rates by percentage were lower in babies in Group II (60% in Group II vs. 80% in Group I, P= 0.188). Both the above findings were proven statistically insignificant. The overall survival rate amongst the study population was 78.4% (39/51)., Conclusions: Supernumerary ribs were associated with a higher occurrence of long gap EA and lower survival rates, though statistically insignificant. Multicentre collaboration may provide significant input for strengthening or refuting the above hypothesis., Competing Interests: None

Aim: We had done this study for TEF with Anorectal malformation and TEF with no Anorectal malformation in terms of age, sex, surgical outcomes and mortality., Materials and Methods: This was a retrospective review of cases with clinical data (from April 2012 to April 2017). The participants of this study were 236 patients who had been diagnosed and managed for ARM. Among these patients, 25 patients associated with EA were selected as the subject patient group., Results: The incidence of tracheoesophageal fistula with ARM was 11.1%. The study has more male preponderance. All cases are of Type c except two cases of Type a. According to the classifications of ARMs, there were two cases with rectourethral fistula and eight cases with rectoperineal fistula and covered anus in the males. In females, there was a varied distribution of seven cases. There was one case (4%) presenting as a part of the Vertebral anorectal malformation cardiac tracheoesophageal renal and limb anomalies (VACTERL) association, which is the representative example of a complex anomaly. Most of the cases died due to cardiac problem and pneumonitis (due to delayed presentation)., Conclusion: The study concludes the experience of EA (±fistulae) with ARM, their distribution, incidence and outcome of the tertiary care centre., Competing Interests: None

Aim: Oesophageal atresia is frequently associated with other malformations, and our aim was to use computed tomography (CT) to explore intrathoracic malformations in patients with this condition., Method: This was retrospective study of children aged 0-16 with oesophageal atresia who were born in 1996-2013 and followed up at the French reference centre for rare oesophageal diseases at the University of Lille. Computed tomography scans were available for 48 of the 234 patients during follow-up visits, and these were reviewed by a thoracic radiologist., Results: More than two-thirds of the scans were performed to explore persistent respiratory symptoms. We found that six patients had a pulmonary malformations: four lobar agenesis, one right pulmonary aplasia and one congenital cystic adenomatoid malformation. Computed tomography enabled us to diagnose unexpected thoracic malformations in 16 patients: four lobar agenesis, six arteria lusoria, five persistent left superior vena cava and one partial anomalous pulmonary venous return. It also confirmed the diagnoses of suspected malformations in five patients: one congenital cystic adenomatoid malformation, one pulmonary hypoplasia, two right-sided aortic arches and one communicating bronchopulmonary foregut malformation., Conclusion: Intrathoracic anomalies were frequently associated with oesophageal atresia, and contrast-enhanced chest CT scans should be performed on patients with persistent respiratory symptoms., (© 2019 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

Esophageal atresia/tracheoesophageal fistula (EA/TEF) is one of the most common gastrointestinal birth defects. It can occur in isolation or in association with other birth defects or genetic syndromes. We retrospectively reviewed the EA/TEF cases evaluated at Rady Children's Hospital San Diego (San Diego, CA) between 2007 and 2016. Data were collected for 157 patients. The majority of patients (105, 66.8%) had an associated major malformation present, and 52 patients (33.1%) had isolated EA/TEF. The patients with associated malformations were distributed as follows: 16 patients (10.2%) had a known genetic syndrome (the most common being Trisomy 21 in 11 patients); six patients (3.8%) had a suspected genetic syndrome; one patient had a suspected teratogenic syndrome (diabetic embryopathy); 30 patients had VACTERL association (19.1%); 32 patients had a "partial VACTERL" association (only two VACTERL-type defects without other malformation); nine patients (5.7%) had one additional non-VACTERL-type birth defect, two patients had VACTERL-type defects plus auricular malformations; and nine patients (5.7%) were classified as "unknown syndrome." A classification of the patterns of malformation of patients with congenital EA/TEF is proposed based on reviewing the data of this relatively large and phenotypically diverse patient group., (© 2020 Wiley Periodicals, Inc.)

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.)

Background: Bronchoscopy is the gold standard for evaluating tracheomalacia; however, reliance on an invasive procedure limits understanding of normal airway dynamics. Self-gated ultrashort echo-time MRI (UTE MRI) can assess tracheal dynamics but has not been rigorously evaluated., Methods: This study was a validation of UTE MRI diagnosis of tracheomalacia in neonates using bronchoscopy as the gold standard. Bronchoscopies were reviewed for the severity and location of tracheomalacia based on standardized criteria. The percent change in cross-sectional area (CSA) of the trachea between end-inspiration and end-expiration was determined by UTE MRI, and receiver-operating curves were used to determine the optimal cutoff values to predict tracheomalacia and determine positive and negative predictive values., Results: Airway segments with tracheomalacia based on bronchoscopy had a more than threefold change in CSA measured from UTE MRI (54.4 ± 56.1% vs 14.8 ± 19.5%; P < .0001). UTE MRI correlated moderately with bronchoscopy for tracheomalacia severity (ρ = 0.39; P = .0001). Receiver-operating curves, however, showed very good ability of UTE MRI to identify tracheomalacia (area under the curve, 0.78). A "loose" definition (> 20% change in CSA) of tracheomalacia had good sensitivity (80%) but low specificity (64%) for identifying tracheomalacia based on UTE MRI, whereas a "strict" definition (> 40% change in CSA) was poorly sensitive (48%) but highly specific (93%)., Conclusions: Self-gated UTE MRI can noninvasively assess tracheomalacia in neonates without sedation, ionizing radiation, or increased risk. This technique overcomes major limitations of other diagnostic modalities and may be suitable for longitudinal population studies of tracheal dynamics., (Copyright © 2020. Published by Elsevier Inc.)

Objective: To describe the epidemiological characteristics, hospital follow-up, and course of patients who underwent surgery for esophageal atresia and its consequences on lung function., Population and Methods: Retrospective, longitudinal, and analytical study. The medical records of patients with esophageal atresia born between 1996 and 2017 were reviewed. Perinatal data, type of atresia, associated malformations, respiratory and gastrointestinal complications, and spirometry data were recorded over 3 years., Results: A total of 97 patients were included. The most common type of atresia was III, and the most frequent syndrome, trisomy 21; 13.4 % of patients died in the neonatal period; 23.8 % were followed up by the Department of Pulmonology, and their respiratory complications included exacerbations (46.4 %), wheezing or asthma (36 %), and pneumonia (26.8 %). Gastroesophageal reflux was a risk factor for wheezing (OR: 5.31; p = 0.002), exacerbations (OR: 4.00; p =0.009), and pneumonia (OR: 3.24; p = 0.02). In the first spirometry (n=20), the pattern was normal in 65 %; restrictive in 30 %; and mixed in 5 %. In the second spirometry (n = 19), the pattern was normal in 42.1 %; restrictive in 31.6 %; obstructive in 15.8 %, and mixed in 10.5 %. In the third spirometry (n = 14), the pattern was normal in 50 %; restrictive in 21.4 %; obstructive in 14.3 %, and mixed in 14.3 %., Conclusions: In our sample of patients, a large proportion had respiratory and gastrointestinal comorbidities. Lung function worsened progressively., Competing Interests: The authors report no conflicts of interest in this work., (Sociedad Argentina de Pediatría.)

Introduction: Esophageal atresia (EA) is associated with significant respiratory mortality. We aimed to assess incidence and predictive factors of EA associated pneumonia during the first 5 years of life., Materials and Methods:  Institutional ethical consent was obtained. Hospital records of patients with EA from 2002 to 2017 were reviewed. Episodes of pneumonia that were diagnosed in university or regional hospitals were included. For instance, respiratory infections other than pneumonia, anastomotic complications, aortopexy, fundoplication, major EA associated diseases, types of EA, and EA anastomosis dilatations were tested as potential risk factors for pneumonia., Results:  A total of 104 patients (56 males; type A 7, B 3, C 83, D 3, E 6, F 2) was included. Thirty-five (34%) patients had 94 episodes of pneumonia corresponding to median 2 (IQR [interquartile range]: 1-4) and 609 episodes per thousand patient years. Majority of pneumonias occurred before the age of 3 years. The cause of pneumonias could be identified as RS (respiratory syncytial) virus in 15(16%) and aspiration in seven (7.4%) episodes. In univariate analysis, pneumonia was predicted by occurrence and number of nonpneumonia respiratory infections, anastomotic reoperations, fundoplication, and number of EA anastomosis dilatations. In multivariate logistic regression analysis, significant risk factors for pneumonia were occurrence of nonpneumonia respiratory infections and number of anastomotic dilatations., Conclusion:  Episodes of pneumonia occurred in one-third of patients with EA. After the final repair, the incidence of pneumonia was highest during the first 3 years of life. Patients with other acute respiratory infections and high number of dilatations were at the greatest risk., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)