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Esophageal atresia: Data from a national cohort
... The third study focused on the risk factors and natural history of gastrooesophageal reflux disease (GORD) at the age of 6 years, through a prospective Partie 1. État des connaissances 1. L'atrésie de l'oesophage 1.1 Définition et fréquence de l'atrésie de l'oesophage L'atrésie de l'oesophage (AO) est une maladie rare dont l'incidence en dehors de la France (Amérique du Sud, Australie, Canada, Etats-Unis, Europe, Irlande, Israël, Mexique, Pays de Galles) est estimée à environ 2,3 à 2,6 / 10 000 naissances vivantes (Lupo et al., 2017;Nassar et al., 2012)(Canfield et al. 2014 Charts and tables 2020b https://eu-rd-platform.jrc.ec.europa.eu/eurocat/eurocatdata/prevalence_en). En France, l'incidence de l'AO est estimée à environ 1,9/10 000 naissances vivantes, soit 160 à 170 nouveaux cas par an (Sfeir et al., 2013(Sfeir et al., , 2015. ...
... L'AO de type III selon la classification de Ladd est, de loin, la forme la plus fréquente (87%). L'AO de type I ou AO « pure » (11%), sans FOT, est la forme la plus sévère (Sfeir et al., 2013). (Schneider et al., 2014;Sfeir et al., 2021). ...
... Les formes associées à une FOT (AO/FOT) sont les formes les plus fréquentes, avec une anastomose le plus souvent possible dans les premiers jours de vie, et celles dont la prise en charge thérapeutique est la plus standardisée (Dingemann et al., 2021;Krishnan et al., 2016). (Bell et al., 2021;Deurloo et al., 2004;Schmedding et al., 2021;Sfeir et al., 2013Sfeir et al., , 2021. ...
Les progrès des dernières décennies dans la prise en charge néonatale et chirurgicale des enfants présentant une atrésie de l’oesophage (AO) ont permis une amélioration significative de leur espérance de vie. Cependant une morbidité importante persiste - en particulier digestive - à court, moyen et long terme, même si la prévalence de ces complications et leurs facteurs de risque restent actuellement mal identifiés. L’objectif de ce travail était d’étudier chez les enfants nés avec une AO, en population, trois complications digestives et leurs déterminants à trois périodes différentes de l’évolution d’une AO.Le premier travail s’est intéressé à la prévalence du dumping syndrome (DS) à l’âge de 3 mois, à l’aide d’une étude prospective multicentrique (n=38). Les nouveau-nés bénéficiaient systématiquement d’une hyperglycémie provoquée par voie orale entre l’âge de 2,5 et 3,5 mois. La glycémie était évaluée de façon discontinue pendant 4 heures, ce qui permettait de repérer les hyperglycémies précoces et les hypoglycémies tardives. Trente pour cent des patients avaient un DS, sans association identifiée avec les signes cliniques observés. Aucun facteur associé au DS n’a pu être mis en évidence dans cette étude.Le deuxième travail s’est intéressé aux facteurs de risque de sténose de l’anastomose (SA) à 1 an, par une étude prospective longitudinale multicentrique en population (n=1082). Les données à la naissance et à un an étaient relevées grâce au registre national français de l’AO (RENATO). Vingt-trois pour cent des enfants présentaient une SA à l’âge de 1 an, et les seuls facteurs de risque de SA identifiés étaient chirurgicaux (anastomose sous tension et anastomose retardée).Le troisième travail a porté sur les facteurs de risque et l’histoire naturelle du reflux gastro-oesophagien (RGO) à l’âge de 6 ans, par une étude prospective longitudinale multicentrique d’une cohorte nichée dans le registre RENATO (n=286). Les données à 6 ans étaient relevées grâce au réseau des centres participant au registre, avec la même méthodologie que le registre. Un tiers des enfants présentaient un RGO à l’âge de 6 ans, et 20% d’entre eux en avaient été opérés. La présence d’un RGO à l’âge de 1 an, un antécédent de gastrostomie avant l’âge d’un an, le sexe masculin et la dénutrition étaient les 4 facteurs prédictifs de RGO à l’âge de 6 ans. Le RGO pouvait disparaître, persister ou apparaître de novo à l’âge de 6 ans.Ce travail a permis la description du DS, nouvelle complication fréquente observée à court terme dans l’AO, d’individualiser des groupes à risque de complication digestives à court et moyen terme (1 an pour les SA et 6 ans pour le RGO) et de décrire l’évolution naturelle à moyen terme du RGO. Ses perspectives sont d’étudier la physiopathologie du DS dans l’AO et de poursuivre le suivi de la cohorte nichée à 12-13 ans, afin de mieux décrire l’histoire naturelle et d’aider à repérer les populations à risque pour adapter le suivi et le traitement de ces patients.
... We report nearly equal sex distribution for the entire cohort (n = 84), which is consistent with previous large retrospective reports (36)(37)(38)(39). Since sex distribution for EA patients was only reported for the entire cohort (34,(39)(40)(41), we indicate -for the first time -that there is nearly equal sex distribution in EA patients by anatomical types (Figure 1C). We also report equal sex distribution for longgap EA patients, which is in accordance with previous report in a larger cohort (9). ...
... This discrepancy could be explained, in part, by our exclusion criteria that eliminated extremely premature patients from the cohort. However, our findings of higher incidence of term-born patients are consistent with the national studies in France (41) and Italy (40) reporting similar results. While the incidence of gestational age in longgap EA patients are reported in the literature (6,42), our novel results outline incidence of long-gap EA by anatomical types (Figure 1B). ...
... It is well known that co-occurring cardiac anomalies can impact the length and complexity of care for EA patients (41). We identified that most EA patients had co-existing cardiac anomalies: 86% for the entire cohort (Table 2A) and 79% in the non-syndromic cases (Table 1C). ...
Esophageal atresia (EA), although a rare congenital anomaly, represents one of the most common gastrointestinal birth defects. There is a gap in our knowledge regarding the impact of perioperative critical care in infants born with EA. This study addresses EA types, disease severity stratification, and mortality in a retrospective cohort at a single institution. Institutional Review Board approved our retrospective cross-sectional study of term-born (n = 53) and premature infants (28–37 weeks of gestation; n = 31) that underwent primary surgical repair of EA at a single institution from 2009–2020. Demographic and clinical data were obtained from the electronic medical record, Powerchart (Cerner, London, UK). Patients were categorized by (i) sex, (ii) gestational age at birth, (iii) types of EA (in relation to respiratory tract anomalies), (iv) co-occurring congenital anomalies, (v) severity of disease (viz. American Society of Anesthesiologists (ASA) and Pediatric Risk Assessment (PRAm) scores), (vi) type of surgical repair for EA (primary anastomosis vs. Foker process), and (vii) survival rate classification using Spitz and Waterston scores. Data were presented as numerical sums and percentages. The frequency of anatomical types of EA in our cohort parallels that of the literature: 9.5% (8/84) type A, 9.5% (8/84) type B, 80% (67/84) type C, and 1% (1/84) type D. Long-gap EA accounts for 88% (7/8) type A, 75% (6/8) type B, and 13% (9/67) type C in the cohort studied. Our novel results show a nearly equal distribution of sex per each EA type, and gestational age (term-born vs. premature) by anatomical EA type. PRAm scoring showed a wider range of disease severity (3–9) than ASA scores (III and IV). The survival rate in our EA cohort dramatically increased in comparison to the literature in previous decades. This retrospective analysis at a single institution shows incidence of EA per sex and gestational status for anatomical types (EA type A-D) and by surgical approach (primary anastomosis vs. Foker process for short-gap vs. long-gap EA, respectively). Despite its wider range, PRAm score was not more useful in predicting disease severity in comparison to ASA score. Increased survival rates over the last decade suggest a potential need to assess unique operative and perioperative risks in this unique population of patients. Presented findings also represent a foundation for future clinical studies of outcomes in infants born with EA.
... По данным литературы, АП встречается с частотой 1,8-2,43 на 10 000 живорожденных. Благодаря развитию неонатальной хирургии в развитых странах выживаемость пациентов с данным пороком составляет от 86,9 до 95 % [3][4][5]. Частота антенатального выявления данного порока развития в Европе варьирует от 50 до 65 %, трудность пренатальной диагностики заключается в неопределенности косвенных признаков, позволяющих заподозрить АП. Несмотря на очевидность клинической картины у детей в 1-е сутки жизни, частота поздней диагностики достигает 16 % в развитых странах и более 30 % в развивающихся странах, что сопряжено с возникновением аспирации на фоне попыток кормлений и развитием вторичной инфекции. ...
... Более чем в половине случаев атрезия пищевода сочетается с другими врожденными пороками развития (ВПР), в том числе входит в состав ассоциации VACTERL и CHARGE-синдрома, что увеличивает риск неблагоприятного исхода. Послеоперационные осложнения, в конечном итоге, сводятся к двум основным проблемам: инфекционные (локальная и генерализованная инфекция) и осложнения, связанные с анатомией порока и способом хирургической коррекции (несостоятельность анастомоза, рубцовое сужение пищевода, гастроэзофагеальный рефлюкс) [3,4]. Дети успешно прооперированные по поводу атрезии пищевода чаще сверстников переносят заболевания желудочно-кишечного тракта, дыхательной системы, имеют задержку роста и развития [6][7][8]. ...
... Так же описаны отдаленные исходы у детей, оперированных по поводу атрезии пищевода [9]. Эпидемиология В литературе накоплен значительный объем данных, собранных в регистрах Европейских стран [4,[10][11][12]. В 2013 г. был опубликован анализ регистра новорожденных с атрезией пищевода, прооперированных в 38 медицинских центрах Франции в период 2008-2009 гг. ...
This literature review is devoted to the problem of predicting in-hospital mortality in newborns with esophageal atresia (EA). According to epidemiological study data, in developed countries, the mortality rate in newborns with EA ranges from 9% to 11% over the past 20 years. Three classifications were developed, Waterston 1962, Montreal 1993, and Spitz 1994, to assess the prognostic significance of risk factors. They considered birth weight, the presence of concomitant congenital malformations and pneumonia, and the need for mechanical ventilation. The choice of a model for predicting outcomes depends on the level of health care and other factors, such as prematurity, low birth weight, late diagnosis, and infectious complications. These factors have a greater impact on patient survival in developing countries than in developed ones, where insurmountable risk factors come out on top: combined congenital malformations and very low birth weight. Also, the magnitude of diastasis between segments of the esophagus creates difficulties in choosing surgical tactics and managing such patients in the postoperative period. In addition, the management of such patients in the intensive care unit, both preoperatively and postoperatively, has a significant impact on the outcome. The literature review underlined "pain points" in the treatment of newborns with EA in regions with different levels of medical care, the consideration of which will allow the achievement of better results.
... EA with/without TEF can occur in isolation or can be associated with various anomalies, most commonly VACTERL syndrome. [10][11][12][13] Other anomalies include CHARGE syndrome, complex cardiac lesions, and other chromosomal abnormalities such as Trisomy 13, 18, and 21. Wang et al. performed a nationwide (USA) analysis of the clinical outcome of 4168 neonates born with EA with/without a TEF in 2014. ...
... year of life. [12] They found EA with a distal TEF, prematurity, and associated anomalies to have strong associations with mortality. The large multicenter study by the Global PedSurg Research Collaborators reported an overall 30-day mortality rate of 25%. ...
Background: Despite great advances in the overall management of neonates with esophageal atresia (EA), many complications leading to morbidity still occurs. Most complications can be treated conservatively, but effective management is needed to reduce long-term morbidity.
Methods: A retrospective cohort study was performed on neonates treated for EA with/without a tracheoesophageal fistula (TEF) between 2001 and 2020. Data were collected from patient records, discharge summaries, and surgical notes. The information recorded included: maternal and neonatal demographics, information regarding the diagnosis, and details surrounding the surgery.
Results: During the 19-year study period, 53 neonates with a mean gestational age of 36.7 weeks were included for analysis. Forty-nine percent presented with an associated anomaly (most commonly, complex cardiac lesions). The majority (83%) had a primary repair on a median of day 3 of life. Nineteen neonates had a surgical complication 30 days post-repair: 7 minor (contained leaks and a chylothorax) and 12 major complications including anastomotic strictures, major anastomotic breakdowns, a recurrent TEF, and 5 surgery-related mortalities.
Conclusions: This study showed less morbidity and mortality of neonates born with EA, despite a high incidence of associated anomalies, in a resource-restricted hospital. It is important to highlight that even with limited resources, centers in low- or middle-income countries can have good outcomes.
... FEED-EASY is a cohort study based on the population-based registry of OA from the French National Network, coordinated by the National Reference Centre for OA. 8 All the multidisciplinary centres from the French National Network that treat OA (n=34) were invited to participate in FEED-EASY. Out of these 34 centres, 22 took part in this study. ...
... Prospective data were collected from the national populationbased registry and from medical files. 8 The French populationbased registry of OA prospectively collects data on all patients born with OA in French centres. Collected data included general demographics, neonatal history, OA-related and surgical characteristics, digestive complications or OA-related comorbidities (anastomotic leaks, recurrent tracheo-oesophageal fistula (TOF), anastomotic stenosis and requiring oesophageal dilatation), respiratory comorbidities (respiratory symptoms defined as recurrent lung infections, chronic wheezing, recurrent episodes of bronchiolitis, and need for nebulised corticosteroids and antibiotic prophylaxis), readmission within the first year of life, failure to thrive (defined as weight≤−2.0 ...
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.
... The incidence is 2.43/10,000 children and EA is often associated with other congenital malformations such as cardiac defects, other gastrointestinal anomalies, as well as limb and urinary tract malformations [1]. Survival rates has increased over the last decades to 90-95% today [2,3]. A child with EA needs surgery in the first days of life to gain access to the gastrointestinal tract for feeding orally. ...
Purpose
This study aimed to explore parents’ experience of sham feeding their baby born with esophageal atresia at home, waiting for reconstructive surgery.
Method
Semi-structured interviews were conducted with parents of six children born with esophageal atresia waiting for delayed reconstruction. The interviews were analyzed using qualitative content analysis.
Results
Parents experienced that sham feed reinforced the healthy abilities in their baby. They had faith in their own ability as parents to care for their child as well as to see to their baby’s strength to cope with difficulties. Parents expressed that the health care system can hinder as well as be a major support on their way to a more normal life at home while waiting for reconstructive surgery.
Conclusion
The experience of sham feeding at home while waiting for reconstructive surgery is characterized by positive aspects both for children born with esophageal atresia and their parents.
... Other congenital anomalies like a VACTERL association are present in up to 60% of OA cases [2], and surgery is needed to restore oesophageal continuity including a trachea-oesophageal fistula closure (if present) so that oral feedings can be established. Pre-operative complications such as aspiration and infections must be minimized to improve the survival rate and clinical outcome [3]. An optimal preoperative preparation includes oral food restriction, "slurping probe" in the upper blind sac to remove saliva, elevating the upper body and antibiotic prophylaxis [4,5]. ...
... When left untreated, esophageal continuity and the separation of the airway from the digestive tract are compromised, making (EA/TEF) a potentially fatal condition for newborns. Even though survival has significantly increased over the past few decades, EA/TEF-related morbidity and anomalies continue to be highly prevalent [3][4][5][6]. ...
With prevalence of about 1 in 3000 live births, pediatric surgeons commonly deal with esophageal abnormalities, which may provide substantial clinical complications. Surprisingly, the embryologic processes underlying esophageal atresia (EA) with or without tracheoesophageal fistula (TEF), one of the hallmark disease entities of pediatric surgery, have only lately been largely uncovered. When it comes to the treatment of congenital esophageal abnormalities, notably esophageal atresia and tracheoesophageal fistula, surgical methods are essential. In order to address the anatomical abnormalities and restore normal function, surgical correction is often necessary in the care of congenital esophageal anomalies, including esophageal atresia and tracheoesophageal fistula. In this review we are going to cover surgical approaches to repair those malformations, long-term outcomes, and latest developments in esophageal surgical approaches.
... Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is a congenital condition that occurs in approximately 1 in every 2,500 to 4,500 newborns [1] and can be repaired by primary anastomosis of the proximal and distal esophagus, with concurrent ligation of any TEF [2]. EA is associated with other congenital and chromosomal anomalies, such as deletions of 22q11, trisomy 18, trisomy 21, or a combination of these [3]. ...
Purpose:
Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is a congenital anomaly that can cause frequent digestive and nutritional problems, even after repair. The most common complication is anastomotic stricture, for which reoperation or balloon dilatation is performed. This study aimed to evaluate the postoperative complications of EA and the role of endoscopic balloon dilatation (EBD) in cases of anastomotic stricture.
Methods:
We retrospectively analyzed patients diagnosed with EA with or without TEF between January 2000 and February 2021. Patients' baseline characteristics, associated anomalies, and postoperative complications were reviewed.
Results:
Among 26 patients, 14 (53.8%) were male, 12 (46.2%) had coexisting anomalies, and the median follow-up was 6.1 years (range, 1.2-15.7 years). In univariate analysis, prematurity, low birth weight, and long-gap EA were associated with postoperative complications in 12 (46.2%) patients. Among the 10 (38.5%) patients with anastomotic stricture, nine (90.0%) required EBD. Regarding the first EBD, it was performed at a median of 3.3 months (range, 1.2-7.6 months) post-repair, while the average patient weight was 4.6 kg. The mean diameter ranged from 3.3 to 9.1 mm without major complications. In univariate analysis, long-gap EA alone was significantly associated with EBD.
Conclusion:
Approximately half of the patients experienced complications after EA repair. In particular, patients with a long-gap EA had a significantly increased risk of complications, such as anastomotic strictures. EBD can be safely used, even in infants.
... In most cases, a primary anastomosis can be performed during the first days of life [1]. Nowadays, due to increased experience in surgical management and neonatal intensive care, the survival rates of patients regularly exceed 90% [2]. Nevertheless, long-term complications after repair of EA, including dysphagia and gastroesophageal reflux (GER), as well as feeding difficulties such as choking, food retention and prolonged meals are still common in the children. ...
Aim:
This study reports the reliability and validity of the Polish version of the Esophageal Atresia Quality of Life (EA-QOL) questionnaires, which were originally developed in Sweden and Germany.
Methods:
A total of 50 families of children (23 aged 2 to 7, and 27 aged 8 to 17) with EA/TEF (esophageal atresia/tracheoesophageal fistula) participated in the study. The development and validation of the Polish version of the EA-QOL involved forward-backward translation of the survey items following the guidelines for cross-cultural translation, cognitive debriefing and evaluation of psychometric properties, including assessment of internal and retest reliability, linguistic validity, content validity, known-group validity and convergent validity. The medical records of patients and standardized questionnaires were used to obtain clinical data. The level of significance was p < 0.05.
Results:
The Polish versions of the EA-QOL questionnaires demonstrated strong linguistic and content validity, are slightly discriminative for esophageal and respiratory problems, but do not show convergent validity with the PedsQL 4.0 generic core scales. In terms of reliability, the internal consistency of the subscale and total scale of Polish versions as measured by Cronbach's alpha is good, and retest reliability is excellent.
Conclusions:
The Polish versions of the EA-QOL questionnaires meet most psychometric criteria that confirm the EA-QOL questionnaires' reliability and validity. This study enables application of these questionnaires in future research among children with EA in Poland and participation in international multicenter studies focusing on advancing knowledge of condition-specific QOL in this population. Future cross-cultural research using larger sample sizes is still needed to better address the relationship between condition-specific and generic QOL, as well as the discriminative ability of the EA-QOL questionnaires.
... [16] In most of the neonates with EA with or without a TEF, antenatal polyhydramnios has been reported to be 55%, but in our study, polyhydramnios was seen in 79.5% of pregnancies which is little higher. [17,18] In our setup, average age of presentation to the institute was 2.8 ± 2.5 days, 45.5% of neonates had respiratory distress at presentation, 18.2% neonates had shock and 27.5% were hypothermic at the time of presentation, hence delayed presentations established aspiration pneumonitis which further led to poor outcome in our study. Long gap EA with gap longer than 3 cm, approximately greater than the height of two vertebral bodies led to tension anastomosis which had higher chances of anastomotic leak and sepsis which decreased survival. ...
BACKGROUND: Esophageal atresia (EA) with or without tracheesophageal fistula (TEF) is a common congenital anomaly requiring urgent surgical intervention after birth. Due to improvement in antenatal diagnosis and postnatal management in neonates with EA, there are better overall survival rates in the developed countries. However, the outcome is still poor in developing countries where multiple factors contribute to higher morbidity and mortality.
AIM: To evaluate various factors affecting the perioperative management and their outcome in neonates with EA with or without TEF.
MATERIALS AND METHODS: This descriptive observational study was conducted for 4 years. Neonates who underwent surgery for EA with or without TEF at our institute were included. Age of the neonate at presentation, gender, birth weight, period of gestation, antenatal diagnosis records, clinical presentation, associated congenital anomalies, surgical interventions, gap between upper and lower esophageal ends, postoperative complications, and outcome was recorded.
RESULTS: Total, 44 neonates underwent surgery for esophageal atresia with or without TEF in our institute. Male-to-female ratio was 2.4:1. The mean birth weight of neonates was 2500 ± 900 g. The mean gestation age was 35 ± 5 weeks. Sepsis was the leading cause of postoperative morbidity seen in 45.5%, followed by anastomotic leak in 22.7%. Postoperative mortality was 59% with the survival rate of 41%.
CONCLUSION: Sepsis, hypothermia, delayed diagnosis, delayed referral, aspiration pneumonitis, anastomotic leaks were the preventable factors affecting the outcome.
... Oesophageal atresia (OA) with or without tracheoesophageal fistula (TOF) is a rare congenital anomaly characterized by failure of the oesophagus to develop normally [1]. The oesophagus ends blindly as a pouch, either in the neck or the thorax, and an abnormal communication between the trachea and the oesophagus often exists [2]. ...
(1) Background: Neonates born with oesophageal atresia and/or tracheooesophageal fistula (OA/TOF) are usually born with a multitude of other congenital anomalies, which may affect their anaesthetic care and survival to hospital discharge. We reviewed the profile and outcome of neonates with OA/TOF and associated congenital cardiac anomalies presenting for surgery at an academic hospital in South Africa. (2) Methods: A retrospective, cross-sectional analysis of all medical records of neonates who had undergone surgical repair of OA/TOF was conducted at an academic hospital between January 2015 and December 2019. Descriptive statistics were used to report the data. Comparisons in perioperative events and outcomes between those with cardiac lesions and those without were done. (3) Results: Neonates presenting for OA/TOF repair with congenital cardiac defects had an incidence of 62 [95% CI 38.5–99.8] per 1000 days since birth. In total, 45.9% had associated cardiac anomalies, with PDA and ASD as the most prevalent lesions. There were statistically significant differences in intraoperative adverse events seen in neonates with congenital cardiac lesions as compared to those without. (4) Conclusion: Despite advances in neonatal critical care and anaesthetic and surgical techniques, OA/TOF associated with congenital cardiac anomalies is still associated with high mortality rates in developing countries.
... It has a prevalence of approximately 1 in 2500-4000 live births (1,2). Children with EA experience gastrointestinal and respiratory morbidities, which may impact on their nutritional intake and growth (3)(4)(5). Studies have demonstrated that children with EA have a lower weight, height, and body mass index (BMI) compared with the reference pediatric population (6)(7)(8)(9). ...
Objectives:
Children with Esophageal Atresia (EA) often have feeding difficulties and dysphagia, which may compromise their nutritional status. This study aimed to compare dietary intake between children with EA and matched healthy controls (HC) and to investigate the relationship between dietary factors, growth, dysphagia and feeding difficulties in the EA cohort.
Methods:
This cross-sectional cohort study recruited children with EA and HC aged 2-17 years from a tertiary pediatric hospital in Australia. Growth parameters were measured. Dietary intake was assessed using the validated Australian Child and Adolescent Eating Survey. Dysphagia and feeding difficulties were assessed using objective questionnaires.
Results:
Twenty-one children with EA were matched for age and sex with 21 healthy controls (HC). Compared to HC, children with EA had lower mean z-scores for height-for-age, but mean weight-for-age and BMI-for-age z scores were similar. Energy intake was similar between the groups. The diet of children with EA consisted of a higher proportion of fats and lower proportion of carbohydrates compared to matched HC. Dysphagia severity in children with EA positively correlated with proportion of energy from fats and saturated fats.
Conclusions:
Children with EA have similar energy intake and growth parameters to HC, but their diet consists of a higher proportion of fats and lower proportion of carbohydrates compared to HC. Targeted dietary interventions and parental education is necessary.
... Esophageal atresia (EA) is characterized anatomically by a congenital obstruction of the esophagus with interruption of the continuity of the esophageal wall and is caused by an abnormal embryological development. Congenital EA has an incidence of 1.8/10,000 births in France (1). The treatment for EA is surgery to close the esotracheal fistula, if present, and to restore esophageal continuity. ...
Introduction: Scoliosis is a well-described complication of esophageal atresia (EA) caused by the associated spine malformations and/or thoracotomy. However, the sagittal posture abnormalities in patients with EA have not been described. The aim of this study was to evaluate the prevalence of and risk factors for sagittal posture abnormalities at the age of 6 years in patients operated on for EA.
Methods: A prospective cohort of 123 patients with EA was examined by the same rehabilitation doctor at the time of a multidisciplinary visit scheduled at the age of 6 years. Children presenting with scoliosis ( n = 4) or who missed the consultation ( n = 33) were excluded. Univariate and multivariate logistic regression models with Firth's penalized-likelihood approach were used to identify risk factors associated with sagittal posture anomalies. Candidate risk factors included neonatal characteristics, associated malformations, atresia type, postoperative complications, psychomotor development retardation, orthopedic abnormalities, and neurological hypotonia.
Results: The prevalence rates of sagittal posture abnormalities were 25.6% ( n = 22; 95% CI, 16.7–36.1%). Multivariate analysis showed that minor orthopedic abnormalities (OR: 4.02, 95% CI: 1.29–13.43, P = 0.021), and VACTERL (OR: 3.35, 95% CI: 1.09–10.71, P = 0.042) were significant risk factors for sagittal posture abnormalities.
Conclusion: This study shows that sagittal posture anomalies occur frequently in children operated on at birth for EA and are not directly linked to the surgical repair. These children should be screened and treated using postural physiotherapy, especially those with VACTERL and minor orthopedic abnormalities.
... El bajo peso al nacer es uno de los factores de riesgo sustanciales para la mortalidad en muchos estudios (19)(20)(21) . Un peso al nacer inferior a 1 500 g es uno de los indicadores de riesgo en el sistema de clasificación de pronóstico establecido por Spitz et al. (11) . ...
Objetivo
: Describir las características clínico-quirúrgicas y sociodemográficas de pacientes con atresia esofágica (AE) del Instituto Nacional de Salud del Niño San Borja (INSN-SB) 2015-2017.
Material y métodos
: Estudio observacional descriptivo de casos con AE 2015-2017. Los datos se procesaron y analizaron en el programa SPSS v20, las variables cualitativas fueron expresadas en frecuencias absolutas y relativas.
Resultados
: Se analizaron 74 casos con AE, 55,4% (n=41) fueron de sexo masculino. Según edad gestacional 45 a término, 28 pre-término y un post-término. El 59,5% tuvieron peso adecuado al nacer. 28,4% tuvieron control prenatal (CPN) completo y 68,9% no tuvieron diagnóstico prenatal de AE. La atresia con fistula traqueo-esofágica (FTE) distal fue la más frecuente con 74,3% (n=55). Se presentó sialorrea en 55 pacientes (74,3%) y disnea en 54 (72,9%). Predominaron las malformaciones cardiacas en el 27,1%, seguida por digestivas 20,3% y genitourinarias 17,6%. Solo 26 tuvieron síndromes asociados, VACTERL en 12 casos. La anastomosis termino-terminal y cierre de fistula se realizó en 55 pacientes (74,3%). 48 presentaron complicaciones tempranas, predominando sepsis (20,3%) y 46 complicaciones tardías, siendo reflujo gastroesofágico el más frecuente 29,7%. La mortalidad fue 10,8%.
Conclusiones
: La mayoría fue de sexo masculino, a término, con adecuado peso al nacer y sin CPN. La AE con FTE distal fue la más frecuente. Sialorrea y disnea fueron los síntomas predominantes y malformaciones cardiacas y digestivas las más asociadas. La anastomosis término-terminal y cierre de fístula fue la técnica más frecuente, como complicaciones sepsis y reflujo gastroesofágico. La mortalidad fue baja.
Gastroesophageal reflux disease (GERD) is frequent and prolonged in esophageal atresia (EA) pediatric patients requiring routine use of proton pump inhibitors (PPIs). However, there are still controversies on the prophylactic use of PPIs and the efficacy of PPIs on GERD and EA complications in this special condition. The aim of the study is to assess the prophylactic use of PPIs in pediatric patients with EA and its complications. We, therefore, performed a systematic review including all reports on the subject from 1980 to 2022. We conducted meta‐analysis of the pooled proportion of PPI—and no PPI groups using random effect model, meta‐regression, and estimate heterogeneity by heterogeneity index I ² . Thirty‐eight reports on the topic met the criteria selection, representing a cumulative 6044 patients with EA. Prophylactic PPI prescription during the first year of life does not appear to prevent GERD persistence at follow‐up and is not associated with a significantly reduced rate of antireflux surgical procedures (ARP). PPIs improve peptic esophagitis and induce remission of eosinophilic esophagitis at a rate of 50%. Their effect on other GERD outcomes is uncertain. Evidence suggests that PPIs do not prevent anastomotic stricture, Barrett's esophagus, or respiratory complications. PPI use in EA can improve peptic and eosinophilic esophagitis but is ineffective on the other EA complications. Side effects of PPIs in EA are almost unknown.
Objectives
This study aims to compare the intestinal microbiota and intestinal inflammation of children with esophageal atresia (EA) to matched healthy controls, and to investigate the relationship between these factors and clinical outcomes.
Methods
A cross‐sectional study of 35 children with EA and 35 matched healthy controls (HC) from a single tertiary pediatric hospital in Australia was conducted. Demographic and dietary data were collected using surveys. Stool samples were analyzed using 16S rRNA sequencing, and fecal calprotectin measurements were used to measure intestinal inflammation. Comparisons were made between the groups, and correlations between the microbiota and clinical factors were investigated in the EA cohort.
Results
Compared to HC, children with EA had similar alpha diversity, but beta diversity analysis revealed clustering of EA and HC cohorts. Children with EA had a significantly higher relative abundance of the order Lactobacillales , and a lower abundance of the genus uncultured Bacteroidales S24‐7 . Fecal calprotectin was significantly higher in children with EA compared to HC. In the EA cohort, children taking proton pump inhibitors (PPI's) had lower alpha diversity and higher calprotectin levels compared to those not taking PPI's. There was a negative correlation between calprotectin and length/height‐for‐age z scores, and children with higher calprotectin levels had a greater burden of gastrointestinal symptoms.
Conclusions
Children with EA have an altered intestinal microbiota compared to HC, which is likely related to PPI use, and may be impacting on growth and quality of life. It is important to rationalize PPI use in this cohort.
In this paper, is presented a national register for esophageal atresia (EA) started in January 2008. We report our experience about the conception of this database and its coordiantion. Data management and data quality are also detailed. In 2023, more then 2500 patients with esophageal atresia are included. Prevalence of EA in France was calculated at 1.8/10000 living birth. Main clinical results are listed with scientifc publications issued directly from the register.
Background and study aims
Esophageal mucosal bridge (EMB) may be diagnosed at the anastomotic site in children operated on for esophageal atresia (EA) but so far only a few cases ( n = 4) have been reported. This study aimed to characterize EMB in children with EA, risk factors, and treatment.
Patients and methods
This retrospective multicenter study recorded patient’s characteristics, EMB diagnosis circumstances, endoscopic management, follow-up, and EMB recurrence in children with EA aged less than 18 years, compared with paired EA patients without EMB.
Results
Thirty patients were included (60% male, 90% EA/tracheoesophageal fistula, 43% associated malformations). Compared to 44 paired controls, EMB was associated with a history of nasogastric tube feeding (31% vs. 9.1%, p = 0.02) and severe gastroesophageal reflux disease (history of fundoplication: 41.4% vs. 13.6%, p < 0.01). 77% had symptoms (food impaction and/or dysphagia). Endoscopic management was performed in 53% of patients (83% electrocoagulation) with no technical difficulties or complications. 80% of the symptomatic patients with EMB improved after endoscopic treatment, independently of anastomotic stricture dilatation or not.
Conclusion
EMB endoscopic management by electrocoagulation is safe and often leads to symptom improvement.
This study was performed to describe the current clinical practice and outcomes of type D esophageal atresia. We retrospectively analyzed 10 patients who were diagnosed with type D esophageal atresia and underwent esophageal atresia and tracheoesophageal fistula repair in the Capital Institute of Pediatrics and Beijing Children’s Hospital from January 2017 to May 2022. Ten patients include three newborns and seven non-newborns. Seven (70%) cases were misdiagnosed as type C esophageal atresia before the first operation. Three neonatal children underwent thoracoscopic distal tracheoesophageal fistula ligation and esophageal anastomosis: the proximal tracheoesophageal fistula was simultaneously repaired with thoracoscopy in one of these children, and the proximal tracheoesophageal fistula was not detected under thoracoscopy in the other two children. Among the seven non-neonatal children, one underwent repair of the proximal tracheoesophageal fistula through the chest and the other six underwent repair through the neck. Nine patients were cured, and one died of complications of severe congenital heart disease. Type D esophageal atresia lacks specific clinical manifestations. Misdiagnosis as type C esophageal atresia is the main cause of an unplanned reoperation. Patients without severe malformations have a good prognosis.
Introduction:
The Dynamic Imaging Grade of Swallowing Toxicity Scale (DIGEST) was developed to evaluate the safety, efficiency, and overall pharyngeal swallowing performance in patients with dysphagia. Although, various types of swallowing dysfunction are encountered in children with esophageal atresia (EA), oropharyngeal dysphagia poses risk for aspiration. Therefore, a retrospective study was performed to evaluate the safety and efficacy of swallowing by using DIGEST score in children with EA.
Methods:
Thirty-nine EA patients were included. The demographic features, respiratory problems, results and outcomes of surgical treatment were evaluated from medical records. The videofluoroscopic evaluation of swallowing (VFSE) investigated for both airway protection and bolus residuals at the level of vallecula, posterior pharyngeal wall and pyriform sinus at liquid and pudding consistencies. The penetration and aspiration scale (PAS) was used to define penetration and aspiration severity, and DIGEST was used to evaluate safety (DIGESTs), efficiency (DIGESTe), and overall pharyngeal swallowing performance (DIGESTt).
Results:
The median age of the patients were 13 months (7-39 months), and male to female ratio was 25:14. 67% of patients were type-C EA and 61% of them has associated anomalies. 38% of patients had aspiration (PAS= 6-8) in liquids and 10% in pudding consistency. Life threatening/profound swallowing dysfunction in DIGESTe (DIGEST=4) was seen in 13% (n=5) of patients. 40% of EA patients showed severe problems in DIGESTt.
Conclusion:
DIGEST is a valid and reliable tool to define the efficacy and safety of swallowing in children with EA.
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.
Background:
This study aimed to evaluate the contemporary surgical management of long-gap esophageal atresia, a rare and challenging problem managed by pediatric general surgeons.
Methods:
A retrospective review of the Pediatric Health Information System database for infants who underwent neonatal gastrostomy, followed by surgical reconstruction for long-gap esophageal atresia (2014-2021). Patients with birthweight less than 1.5 kg and those who received neonatal cardiac surgery were excluded. Outcomes were analyzed, including the need for further procedures, length of stay, and mortality.
Results:
Of 1,346 infants who underwent repair across 47 major children's hospitals, 100 (7%) met the inclusion criteria for long-gap esophageal atresia. Cardiac anomalies were identified in 43% of patients. The median age at repair was 87 days (interquartile range, 62-133). Ten percent of patients had a planned or unplanned reoperation ≤30 days after index surgery, and 4% underwent reoperation at >30 days. The median time to reoperation was 9 days (interquartile range, 7-60). Mortality during index admission was 5%, and the median hospital length of stay was 143 days (interquartile range, 101-192). Length of stay was significantly longer in patients with cardiac anomalies (cardiac: 179 days, non-cardiac: 125 days; P < .001), and 52% of patients required at least 1 postoperative dilation. The median time to the first dilation was 70 days (interquartile range, 42-173).
Conclusion:
This large multicenter study highlights the challenges of infants with long-gap esophageal atresia but suggests a high rate of successful delayed primary reconstruction. Hospitalizations are prolonged, and anastomotic stricture rates remain high. These data are useful for pediatric surgeons in counseling families on surgical repair strategy, timing, and postoperative outcomes.
The endoscopist plays a critical role in the management of patients with congenital esophageal defects. This review focuses on esophageal atresia and congenital esophageal strictures and, in particular, the endoscopic management of comorbidities related to these conditions, including anastomotic strictures, tracheoesophageal fistulas, esophageal perforations, and esophagitis surveillance. Practical aspects of endoscopic techniques for stricture management are reviewed including dilation, intralesional steroid injection, stenting, and endoscopic incisional therapy. Endoscopic surveillance for mucosal pathology is essential in this population, as patients are at high risk of esophagitis and its late complications such as Barrett's esophagus.
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.
Introduction:
There is currently no validated diagnostic modality to characterize the anatomy and predict outcomes of tracheal esophageal defects, such as esophageal atresia (EA) and tracheal esophageal fistulas (TEFs). We hypothesized that ultra-short echo-time MRI would provide enhanced anatomic information allowing for evaluation of specific EA/TEF anatomy and identification of risk factors that predict outcome in infants with EA/TEF.
Methods:
In this observational study, 11 infants had pre-repair ultra-short echo-time MRI of the chest completed. Esophageal size was measured at the widest point distal to the epiglottis and proximal to the carina. Angle of tracheal deviation was measured by identifying the initial point of deviation and the farthest lateral point proximal to the carina.
Results:
Infants without a proximal TEF had a larger proximal esophageal diameter (13.5 ± 5.1 mm vs. 6.8 ± 2.1 mm, p = 0.07) when compared to infants with a proximal TEF. The angle of tracheal deviation in infants without a proximal TEF was larger than infants with a proximal TEF (16.1 ± 6.1° vs. 8.2 ± 5.4°, p = 0.09) and controls (16.1 ± 6.1° vs. 8.0 ± 3.1°, p = 0.005). An increase in the angle of tracheal deviation was positively correlated with duration of post-operative mechanical ventilation (Pearson r = 0.83, p < 0.002) and total duration of post-operative respiratory support (Pearson r = 0.80, p = 0.004).
Discussion:
These results demonstrate that infants without a proximal TEF have a larger proximal esophagus and a greater angle of tracheal deviation which is directly correlated with the need for longer post-operative respiratory support. Additionally, these results demonstrate that MRI is a useful tool to assess the anatomy of EA/TEF.
Background
The estimated prevalence of esophageal atresia (EA) is 1 in 2500–4500 live births (LBs). Researchers have already identified risk factors, but the mechanisms are still unknown. The aim of this study is to identify EA prevalence trends and its risk factors in the São Paulo State (SPS) population database.
Methods
We conducted a population-based study using all EA cases identified by the Live Births Information System across 14 years (2005–2018) to estimate EA prevalence trends in recent years, stratified by maternal age and SPS geographical clusters. We calculated the prevalence trends, regression coefficient (β), annual percent change (APC), and 95% confidence interval (CI).
Results
We found 820 EA cases among 8,536,101 LBs with a prevalence of 1.0/10,000 LBs in SPS, Brazil. There was no significant difference in distribution by sex. Among all the cases, the majority (65%) were Caucasian; 51.8% were born at term; 43% had weight of ≥2500 g; 95.4% were singleton; and 73.4% of births were by cesarean section. From 2005 to 2018, there was an increasing trend of EA prevalence (APC=6.5%) with the highest APC of 12.2%. The highest EA prevalence rate (1.7/10,000 LB) was found in the group with maternal age of ≥35 years. No significant seasonal variation was found based on the conception month (p=0.061).
Conclusions
EA had an increasing prevalence trend in SPS, Brazil, in recent years, with the highest prevalence rate in the group with maternal age of ≥35 years. No seasonality was observed. This population-based study is the first to summarize the current epidemiology of EA in SPS LB.
Esophageal atresia with or without tracheoesophageal fistula and tracheobronchomalacia encompass 2 of the most common complex congenital intrathoracic anomalies. Tailoring interventions to address the constellation of problems present in each patient is essential. Due to advances in neonatology, anesthesia, pulmonary, gastroenterology, nutrition and surgery care for patients with complex congenital tracheoesophageal disorders has improved dramatically. Treatment strategies tailored to the individual patient needs are best implimented under the aegis of a comprehensive longitudinal multidisciplinary care team.
Developmental abnormalities provide a unique opportunity to seek for the molecular mechanisms underlying human organogenesis. Esophageal development remains incompletely understood and elucidating causes for esophageal atresia (EA) in humans would contribute to achieve a better comprehension. Prenatal detection, syndromic classification, molecular diagnosis, and prognostic factors in EA are challenging. Some syndromes have been described to frequently include EA, such as CHARGE, EFTUD2-mandibulofacial dysostosis, Feingold syndrome, trisomy 18, and Fanconi anemia. However, no molecular diagnosis is made in most cases, including frequent associations, such as Vertebral-Anal-Cardiac-Tracheo-Esophageal-Renal-Limb defects (VACTERL). This study evaluates the clinical and genetic test results of 139 neonates and 9 fetuses followed-up at the Necker-Enfants Malades Hospital over a 10-years period. Overall, 52 cases were isolated EA (35%), and 96 were associated with other anomalies (65%). The latter group is divided into three subgroups: EA with a known genomic cause (9/148, 6%); EA with Vertebral-Anal-Cardiac-Tracheo-Esophageal-Renal-Limb defects (VACTERL) or VACTERL/Oculo-Auriculo-Vertebral Dysplasia (VACTERL/OAV) (22/148, 14%); EA with associated malformations including congenital heart defects, duodenal atresia, and diaphragmatic hernia without known associations or syndromes yet described (65/148, 44%). Altogether, the molecular diagnostic rate remains very low and may underlie frequent non-Mendelian genetic models.
Esophageal atresia (EA) is a life-threatening congenital malformation of the esophagus. Despite considerable recent advances in perinatal resuscitation and neonatal care, EA remains an important cause of mortality and morbidity, especially in low-income countries. The aim of this study was to assess risk factors for adverse outcomes following surgical repair of EA at a single center in Tunisia. We performed a retrospective analysis using medical records of neonates with surgical management of EA at our institution from 1 January 2007 to 31 December 2021. In total, 88 neonates were included with a mortality rate of 25%. There were 29 girls and 59 boys. The diagnosis of EA was suspected prenatally in 19 patients. The most common associated anomalies were congenital heart diseases. Prematurity, low birth weight, outborn birth, age at admission >12 hours, congenital heart disease, postoperative sepsis, and anastomotic leak were risk factors for mortality following surgical repair of EA. Anastomotic tension was the only factor associated with short-term complications and the occurrence of short-term complications was predictive of mid-term complications. This study provides physicians and families with contemporary information regarding risk factors for adverse outcomes following surgical repair of EA. Thus, any effort to reduce these risk factors would be critical to improving patient outcomes and reducing cost. Future multi-institutional studies are needed to identify, investigate, and establish best practices and clinical care guidelines for neonates with EA.
Summary Background Data
EA is the most frequent congenital esophageal malformation. Long gap EA remains a therapeutic challenge for pediatric surgeons. A case case-control prospective study from a multi-institutional national French data base was performed to assess the outcome, at age of 1 and 6 years, of long gap esophageal atresia (EA) compared with non-long gap EA/tracheo-esophageal fistula (TEF). The secondary aim was to assess whether initial treatment (delayed primary anastomosis of native esophagus vs. esophageal replacement) influenced mortality and morbidity at ages 1 and 6 years.
Methods
A multicentric population-based prospective study was performed and included all patients who underwent EA surgery in France from January 1, 2008 to December 31, 2010. A comparative study was performed with non-long gap EA/TEF patients. Morbidity at birth, 1 year, and 6 years was assessed.
Results
Thirty-one patients with long gap EA were compared with 62 non-long gap EA/TEF patients. At age 1 year, the long gap EA group had longer parenteral nutrition support and longer hospital stay and were significantly more likely to have complications both early post-operatively and before age 1 year compared with the non-long gap EA/TEF group. At 6 years, digestive complications were more frequent in long gap compared to non-long gap EA/TEF patients. Tracheomalacia was the only respiratory complication that differed between the groups. Spine deformation was less frequent in the long gap group. There were no differences between conservative and replacement groups at ages 1 and 6 years except feeding difficulties that were more common in the native esophagus group.
Conclusions
Long gap strongly influenced digestive morbidity at age 6 years.
Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is one of the most common digestive malformations whose prevalence varies between 1.8 and 2.4/100,000 births. Although mortality decreased dramatically to about 5%, digestive problems remain frequent in children with EA both in early infancy and at long-term follow-up. These patients are at major risk of presenting gastroesophageal reflux disease (GERD) and its complications: anastomotic strictures, esophagitis, failure to thrive, and Barrett’s esophagus. Concerns in adults include esophageal adenocarcinoma and epidermoid carcinoma which have been occasionally reported. Recent recommendations help for the management of gastrointestinal complications, although they are mostly nonevidence-based. It is recommended that GERD be treated with proton pump inhibitors (PPI) in all EA patients from the neonatal period to the first year of life, or longer, depending on persistence of GERD. Endoscopy with biopsies is mandatory for routine monitoring of GERD in patients with EA. Every EA patient, including asymptomatic, should undergo monitoring of GERD (impedance/pH-metry, and/or endoscopy) at the time of discontinuation of anti-acid treatment and lifelong. Systematic endoscopy is recommended throughout childhood: one after stopping PPI therapy, one before the age of 10 years, and one at transition to adulthood. Thereafter, a regular clinical follow-up in every adult patient with EA is recommended with routine endoscopy every 5–10 years.KeywordsEsophageal atresiaGastroesophageal reflux diseaseBarrett’s esophagusAnastomotic stricture
Objetivo: Descrever as principais medidas epidemiológicas da Atresia de Esôfago em menores de 1 ano no estado do Rio de Janeiro de 2008 a 2021. Métodos: Estudo populacional no qual foram utilizados o Sistema de Informações Hospitalares (SIH/SUS), Sistema de Informação de Mortalidade (SIM) e Sistema de Informações de Nascidos Vivos (SINASC) e as taxas calculadas para 100 mil nascidos vivos. Resultados: Nesse período foram realizadas 273 internações. O ano com maior taxa de internações foi 2019, com 14,41 a cada 100.000 nascidos vivos e o com menor foi 2012, com 4,04. O número total de óbitos foi de 95. A maior taxa de mortalidade foi em 2018, de 4,98. Já a menor taxa de mortalidade foi vista no ano de 2013, com 1,78. Considerações finais: Sendo assim, foi visto que a taxa de internação aumentou ao longo do tempo, podendo-se entender que houve melhora ao diagnosticar esses pacientes, contudo a taxa de mortalidade não diminuiu, o que pode ser explicado por ainda haver a falta de recursos financeiros e tecnológicos, já que o Brasil é um país em desenvolvimento.
Objetivo: Descrever as principais medidas epidemiológicas da Atresia de Esôfago em menores de 1 ano no estado do Rio de Janeiro de 2008 a 2021. Métodos: Estudo populacional no qual foram utilizados o Sistema de Informações Hospitalares (SIH/SUS), Sistema de Informação de Mortalidade (SIM) e Sistema de Informações de Nascidos Vivos (SINASC) e as taxas calculadas para 100 mil nascidos vivos. Resultados: Nesse período foram realizadas 273 internações. O ano com maior taxa de internações foi 2019, com 14,41 a cada 100.000 nascidos vivos e o com menor foi 2012, com 4,04. O número total de óbitos foi de 95. A maior taxa de mortalidade foi em 2018, de 4,98. Já a menor taxa de mortalidade foi vista no ano de 2013, com 1,78. Considerações finais: Sendo assim, foi visto que a taxa de internação aumentou ao longo do tempo, podendo-se entender que houve melhora ao diagnosticar esses pacientes, contudo a taxa de mortalidade não diminuiu, o que pode ser explicado por ainda haver a falta de recursos financeiros e tecnológicos, já que o Brasil é um país em desenvolvimento.
Background:
Esophageal atresia is an upper gastrointestinal tract developmental abnormality in which the upper and lower esophagus do not connect. Esophageal atresia has a higher incidence of death in sub-Saharan Africa, ranging from 30% to 80%. In Ethiopia, infants with esophageal atresia had a higher mortality rate. The assessment of time to death and predictors of esophageal atresia can help to reduce newborn mortality.
Objective:
This study was aimed to investigate the time to death and predictors of neonates with esophageal atresia admitted to Tikur Anbessa Specialized Hospital, Ethiopia.
Methods:
An institutional-based retrospective follow-up study was conducted among 225 neonates diagnosed with esophageal atresia. The median survival time, Kaplan-Meier failure estimation curve, and Log rank test were computed. Bivariable and multivariable Cox regression hazards models were fitted to identify the predictors of time to death. Hazard ratio with a 95% confidence interval was calculated and p-values <0.05 were considered statistically significant.
Results:
In the study, the incidence density rate of neonates diagnosed with esophageal atresia was 5.5 (95% CI, 4.7-6.4) per 100-neonates day. The median time to death was 11 days (95% confidence interval (CI), 8.92-13.08). Birth weight <2500 g (adjusted hazard ratio (AHR)=1.49, 95% CI, 1.02 -2.21), having sepsis (AHR=1.67,95% CI, 1.15-2.44), being malnourished (AHR = 1.61, 95% CI, 1.03 -2.58), esophageal atretic neonates without surgery (AHR = 3.72, 95% CI, 1.34-10.38), diagnosis time at >48 hours of admission (AHR = 1.48, 95% CI, 1.01-2.15) and being dehydrated (AHR = 2.38, 95% CI, 1.63-3.46) were significant predictors of time to death among esophageal atretic neonates.
Conclusion:
The findings in this study highlighted the necessity of early diagnosis, proper comorbidity treatment, and timely surgical intervention to reduce infant deaths due to esophageal atresia.
Objectives
To identify the risk factors for anastomotic, refractory and recurrent strictures and to establish whether anastomotic stricture is associated with antireflux surgery.
Design
This prospective national multicentre study included all infants born with oesophageal atresia (OA) over an 8-year period. Data on OA and complications were collected at birth and at 1 year old. Univariate and multivariate analyses were conducted.
Results
1082 patients from 37 centres were included in the study. The prevalence of anastomotic stricture at 1 year old was 23.2%. Anastomosis under tension (defined by the surgeon at the time of repair) and delayed anastomosis (defined as anastomosis performed more than 15 days after birth, excluding delays due to prematurity or severe cardiac diseases) were found to be independent risk factors for anastomotic stricture (2.3 (1.42–3.74) and 4.02 (2.12–7.63), respectively). Patients with anastomotic stricture had a 2.3-fold higher rate of fundoplication compared with others (p=0.001). Anastomosis under tension and delayed anastomosis were found to be independent risk factors for recurrent stricture (1.92 (1.10–3.34) and 5.73 (2.71–12.14), respectively), while delayed anastomosis was the only risk factor for refractory stricture (8.30 (3.34–20.64)). There was a 2.39-fold (1.42–4.04) higher rate of fundoplication in the anastomotic stricture group than in the group without anastomotic stricture (p=0.001).
Conclusions
Patient-related anatomical factors leading to anastomosis under tension and delayed anastomosis increase the risk of anastomotic stricture.
Objective
: This study aims to quantify mortality rates and hospital lengths of stay (LOS) in neonates with esophageal atresia and tracheoesophageal fistula (EA/TEF), and to characterize the effects of birth weight (BW) and associated congenital anomalies upon these.
Methods
: Data regarding patients with EA/TEF were prospectively collected (2013-2019) at 298 North American centers. The primary outcome was mortality and secondary outcome was LOS. Risk factors affecting mortality and LOS were assessed.
Results
: EA/TEF was diagnosed in 3290 infants with a median BW of 2476 g (IQR 1897,2970). In-hospital mortality was 12.7%. Mortality was inversely correlated with BW. After adjustment, the risk of mortality decreased by approximately 11% with every 100g increase in BW. A significant congenital anomaly other than EA/TEF was diagnosed in 37.9% of patients. Risk of mortality increased in patients with associated congenital anomalies, most notably in those with a severe cardiac anomaly. Lower BW was associated with an increased mean LOS among survivors. Similar to mortality risk, additional anomalies were associated with prolonged LOS.
Conclusions
: This study demonstrates an in-hospital mortality of over 10%. Both increased mortality and prolonged LOS are highly associated with lower birth weight and the presence of concomitant congenital anomalies.
Introduction Adequate patient volume is essential for the maintenance of quality, meaningful research, and training of the next generation of pediatric surgeons. The role of university hospitals is to fulfill these tasks at the highest possible level. Due to decentralization of pediatric surgical care during the last decades, there is a trend toward reduction of operative caseloads. The aim of this study was to assess the operative volume of the most relevant congenital malformations at German academic pediatric surgical institutions over the past years.
Methods Nineteen chairpersons representing university-chairs in pediatric surgery in Germany submitted data on 10 index procedures regarding congenital malformations or neonatal abdominal emergencies over a 3-year period (2015 through 2017). All institutions were categorized according to the total number of respective cases into “high,” “medium,” and “low” volume centers by terciles. Some operative numbers were verified using data from health insurance companies, when available. Finally, the ratio of cumulative case load versus prevalence of the particular malformation was calculated for the study period.
Results From 2015 through 2017, a total 2,162 newborns underwent surgery for congenital malformations and neonatal abdominal emergencies at German academic medical centers, representing 51% of all expected newborn cases nationwide. The median of cases per center within the study period was 101 (range 18–258). Four institutions (21%) were classified as “high volume” centers, four (21%) as “medium volume” centers, and 11 (58%) as “low volume” centers. The proportion of patients operated on in high-volume centers varied per disease category: esophageal atresia/tracheoesophageal fistula: 40%, duodenal atresia: 40%, small and large bowel atresia: 39%, anorectal malformations: 40%, congenital diaphragmatic hernia: 56%, gastroschisis: 39%, omphalocele: 41%, Hirschsprung disease: 45%, posterior urethral valves: 39%, and necrotizing enterocolitis (NEC)/focal intestinal perforation (FIP)/gastric perforation (GP): 45%.
Conclusion This study provides a national benchmark for neonatal surgery performed in German university hospitals. The rarity of these cases highlights the difficulties for individual pediatric surgeons to gain adequate clinical and surgical experience and research capabilities. Therefore, a discussion on the centralization of care for these rare entities is necessary.
Œsophageal atresia is a rare neonatal malformation consisting in an interruption of the continuity of the œsophagus, with or without a tracheo-œsophageal fistula. Although mortality rate is now low and most cases can benefit from successful surgical repair soon after birth, morbidity –specially digestive and nutritional- remains high. Many of the adults born with œsophageal atresia will suffer from dysphagia, gastro-œsophageal reflux and/or œsophageal dysmotility, leading to nutritional consequences and quality of life impairment. Barrett’s œsophagus, potential risk of œsophageal cancer as well as risk of anastomotic stenosis and eosinophilic œsophagitis justify transition to adulthood and a lifelong prolonged follow-up.
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.
Background: Esophageal atresia (EA) is a rare congenital malformation of the esophagus. Surgical treatment is required to restore the continuity of the esophagus. This can be performed through thoracotomy. However, an increasing number of hospitals is performing minimal invasive surgery (MIS). In this article, we describe the technique of thoracoscopic repair of EA in neonates in more detail and show the outcome of a patient cohort operated by young pediatric surgeons in training. Methods: Between 2014 and 2019 correction was performed in 64 EA type C patients at the UMC Utrecht, Wilhelmina Children's Hospital, mainly by young pediatric surgeons in training. Results: All patients were corrected through MIS, 3 days after birth. The median operation duration was 181 (127-334) minutes. Nasogastric tube feeding was started on the first postoperative day, and oral feeding 6 days postop. Postoperative complications included leakage (14.1%), stenosis (51.1%), and recurrent tracheoesophageal fistula (7.8%). Conclusion: Thoracoscopic repair of EA can be performed safely, with good outcome and all the benefits of MIS. However, it remains a challenging procedure and should be performed only in pediatric centers with a vast experience in MIS, especially when training young pediatric surgeons. These centers must have access to a multidisciplinary team of neonatologists, pediatric anesthesiologists, surgeons, and ENT specialists to ensure the best possible care in hemodynamic, respiratory, and cerebral monitoring and gastrointestinal and developmental outcome.
Purpose. The aim of this study was to determine the influence of prognostic factors on the postoperative outcome of esophageal atresia patients, taking into consideration loco-regional particular features. Methods. A retrospective analysis of the medical records of 28 patients diagnosed with esophageal atresia was conducted, using a logistic regression model. Patients had been admitted in our hospital between 2009-2014. The survival rate was recorded separately from the independent factors, such as demographic features, weight at birth, the timing of surgical treatment, co-morbidities, postoperative prognosis and management of complications. Results. According to the Spitz prognostic classification, there were 21 patients in group I (birth weight over 1500 g with no major anomaly), 3 patients in group II (birth weight less than 1500 g or major cardiac anomaly) and 4 patients in group III (birth weight less than 1500 g plus major cardiac anomaly). The mortality rate was 33% in group I, 100% in group II and 100% in group III. The mean birth weight was 2282 g ±2SD, and the mean gestational age was 31 weeks. The age at initial presentation was over 24 hours in 15 patients, with fatal outcome in 13 of them. The cardiac malformations presented as the associated anomalies with the highest risk. Surgical treatment was as follows: primary anastomosis in 21 cases, cervicostomy and gastrostomy in 6 cases, and Foker technique in 1 case. Conclusions. The analysis of this series indicated a low survival rate for this pathology in our center. Besides the prognostic factors cited in literature (low birth weight and age at birth, associated cardiac malformations), we include, as risk factors for the increased mortality, the delayed diagnosis and presentation at our tertiary center. The further refinement of a multidisciplinary approach towards this pathology would contribute to a higher survival rate and an improved result of the therapeutic management.
Purpose: We sought to evaluate the impact of thoracoscopic repair on perioperative outcomes in infants with esophageal atresia and tracheoesophageal fistula (EA/TEF).
Methods: The American College of Surgeons National Surgical Quality Improvement Program pediatric database from 2014 to 2018 was queried for all neonates who underwent operative repair of EA/TEF. Operative approach based on intention to treat was correlated with perioperative outcomes, including 30-day postoperative adverse events, in logistic regression models.
Results: Among 855 neonates, initial thoracoscopic repair was performed in 133 (15.6%) cases. Seventy (53%) of these cases were converted to open. Those who underwent thoracoscopic repair were more likely to be full-term (p=0.03) when compared to those in the open repair group. There were no significant differences in perioperative outcome measures based on surgical approach except for operative time (thoracoscopic: 217 min vs. open: 180 min, p<0.001). A major cardiac comorbidity (OR 1.6, 95% CI 1.2-2.1; p=0.003) and preoperative ventilator requirement (OR 1.4, 95% CI 1.0-1.9; p=0.034) were the only risk factors associated with adverse events.
Conclusions: Thoracoscopic neonatal repair of EA/TEF continues to be used sparingly, is associated with high conversion rates, and has similar perioperative outcomes when compared to open repair.
Level of evidence: III
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.
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.
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, 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 length of stay was 31.0 (17.0–64.0) days. Multivariate analysis showed that intrahospital mortality at 3 months was associated with low birth weight [odds ratio (OR) 0.52 (95% confidence interval (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]. Length of stay 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 length of stay [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.
Esophageal atresia (EA) with or without tracheo-esophageal fistula (TEF) is a rare condition that can be nowadays succesfully treated. The current interest therefore is focused on the management of the difficult cases, on thoracoscopic approach, and on some aspects of the long-term results.
The current strategies for the difficult or impossible anastomoses in pure and long-gap EA, the introduction of thoracoscopic repair and the causes, mechanisms and management of post-operative gastro-esophageal reflux (GER) are reviewed.
Methods of esophageal elongation and multi-staged repair of pure and long-gap EA allow anastomosis but with functional results that are often poor. Esophageal replacement with colon or stomach achieves at least similar results and often requires less procedures. Thoracoscopic repair is a promising adjunct, but the difficulties for setting it as a gold-standard are pointed out. GER is a part of the disease and its surgical treatment, that is often required, is burdened by high failure rates.
EA with or without TEF can be successfully treated in most cases, but a number of unsolved issues remain and the current approach to difficult cases will certainly evolve in the future.
Pakarinen, MD, PhD, Associate Professor of Paediatric Surgery Hospital for children and adolescents, University of Helsinki Rintala RJ, Sistonen S, Pakarinen MP. Outcome of oesophageal atresia beyond childhood.
Esophageal atresia with/without tracheo-esophageal fistula is a relatively common malformation, occurring in around 1 in 3500 births. In around half of cases, additional malformations are present, forming either a syndrome of known genetic aetiology, or a recognised association, of which the VACTERL association (Vertebral anomalies, Anal atresia, Cardiac malformations, Tracheo-Esophageal fistula, Renal and Limb malformations) is the most recognised. Recently, microdeletions of the FOX gene cluster at 16q24.1, comprising four genes, FOXF1, MTHFSD, FOXC2 and FOXL1, were reported to cause a phenotype resembling VACTERL association, with vertebral anomalies, gastro-intestinal atresias (esophageal, duodenal and anal), congenital heart malformations, and urinary tract malformations, as well as a rare lethal developmental anomaly of the lung, alveolar capillary dysplasia. This article reviews these new data alongside other genetic causes of syndromic esophageal atresia, and also highlights information from relevant mouse models, particularly those for genes in the Sonic Hedgehog pathway.
Survivors of esophageal atresia are reaching their adulthood in large numbers for the first time enabling assessment of true long-term outcome among this group of patients. This review summarizes the current knowledge on the subject focusing on late symptoms and complications, esophageal pathology and pulmonary function. Relationships between esophageal dysmotility, gastroesophageal reflux, esophagitis and epithelial metaplastic changes including esophageal cancer are outlined. In addition to pertinent literature, institutional experience, and follow-up of patients with esophageal atresia for more than 60 years is included.
Oesophageal atresia (OA) encompasses a group of congenital anomalies comprising of an interruption of the continuity of the oesophagus with or without a persistent communication with the trachea. In 86% of cases there is a distal tracheooesophageal fistula, in 7% there is no fistulous connection, while in 4% there is a tracheooesophageal fistula without atresia. OA occurs in 1 in 2500 live births. Infants with OA are unable to swallow saliva and are noted to have excessive salivation requiring repeated suctioning. Associated anomalies occur in 50% of cases, the majority involving one or more of the VACTERL association (vertebral, anorectal, cardiac, tracheooesophageal, renal and limb defects). The aetiology is largely unknown and is likely to be multifactorial, however, various clues have been uncovered in animal experiments particularly defects in the expression of the gene Sonic hedgehog (Shh). The vast majority of cases are sporadic and the recurrence risk for siblings is 1%. The diagnosis may be suspected prenatally by a small or absent stomach bubble on antenatal ultrasound scan at around 18 weeks gestation. The likelihood of an atresia is increased by the presence of polyhydramnios. A nasogastric tube should be passed at birth in all infants born to a mother with polyhydramnios as well as to infants who are excessively mucusy soon after delivery to establish or refute the diagnosis. In OA the tube will not progress beyond 10 cm from the mouth (confirmation is by plain X-ray of the chest and abdomen). Definitive management comprises disconnection of the tracheooesophageal fistula, closure of the tracheal defect and primary anastomosis of the oesophagus. Where there is a "long gap" between the ends of the oesophagus, delayed primary repair should be attempted. Only very rarely will an oesophageal replacement be required. Survival is directly related to birth weight and to the presence of a major cardiac defect. Infants weighing over 1500 g and having no major cardiac problem should have a near 100% survival, while the presence of one of the risk factors reduces survival to 80% and further to 30-50% in the presence of both risk factors.
Although initial prognosis of oesophageal atresia is nowadays excellent with more than 95% of survival, the long-term complications are frequent. A gastro-oesophageal reflux is found in 26 to 75% of the cases, responsible for peptic oesophagitis, anastomotic stenosis and Barrett's oesophagus, risk factor of adenocarcinoma of the oesophagus. A dysphagia is frequently observed on these patients, sometimes several years after the surgery, observed in almost 45% of five-year-old children. Growth retardation is found in nearly a third of these children. Respiratory symptoms are particularly frequent, especially in the first years, associating tracheomalacia facilitating the bronchopulmonary infectious episodes (found in about 30% of 5-year-old children). Esotracheal fistula recurrence is very rare. A deformation of the rib cage is reported in 20%, and a scoliosis in 10% of the patients. However, the quality of life of these patients in the adulthood is good, and influenced by the existence of associated malformations. Even if the current prognosis of oesophageal atresia is good altogether, the frequency of the complications (digestive, respiratory, nutritional, orthopaedic) far from the initial intervention, and the necessity of a surveillance of the secondary oesophageal damages, justifies a systematic and multidisciplinary follow-up until adulthood.
To describe prevalence, prenatal diagnosis and epidemiological data on oesophageal atresia from 23 well-defined European regions and compare the prevalence between these regions.
Population-based study using data from a large European database for surveillance of congenital anomalies (EUROCAT) for two decades (1987-2006).
Twenty-three participating registries based on multiple sources of information including information about live births, fetal deaths with gestational age ≥20 weeks and terminations of pregnancy.
1222 cases of oesophageal atresia in a population of 5 019 804 births.
The overall prevalence was 2.43 cases per 10 000 births (95% CI 2.30 to 2.57). There were regional differences in prevalence ranging from 1.27 to 4.55. Prenatal detection rates varied by registry from >50% of cases to <10% of cases. A total of 546 cases (44.7%) had an isolated oesophageal anomaly, 386 (31.6%) were multiple malformed and 290 (23.7%) had an association or a syndrome. There were 1084 live born cases (88.7%), 43 cases were fetal deaths and 95 cases were terminations of pregnancy. One-week survival for live births was 86.9% and 99.2% if the gestational age was ≥38 weeks and isolated oesophageal atresia was present. Males accounted for 57.3% of all cases and 38.5% of live born cases were born with gestational age <37 weeks.
There were regional differences in prevalence of oesophageal atresia in Europe. Half of all cases had associated anomalies. Prenatal detection rate increased from 26% to 36.5% over the two decades. Survival in infants with isolated oesophageal atresia born at term is high.
Congenital heart disease is the most common congenital disorder in newborns. Advances in cardiovascular medicine and surgery have enabled most patients to reach adulthood. Unfortunately, prolonged survival has been achieved at a cost, as many patients suffer late complications, of which heart failure and arrhythmias are the most prominent. Accordingly, these patients need frequent follow-up by physicians with specific knowledge in the field of congenital heart disease. However, planning of care for this population is difficult, because the number of patients currently living with congenital heart disease is difficult to measure. Birth prevalence estimates vary widely according to different studies, and survival rates have not been well recorded. Consequently, the prevalence of congenital heart disease is unclear, with estimates exceeding the number of patients currently seen in cardiology clinics. New developments continue to influence the size of the population of patients with congenital heart disease. Prenatal screening has led to increased rates of termination of pregnancy. Improved management of complications has changed the time and mode of death caused by congenital heart disease. Several genetic and environmental factors have been shown to be involved in the etiology of congenital heart disease, although this knowledge has not yet led to the implementation of preventative measures. In this Review, we give an overview of the etiology, birth prevalence, current prevalence, mortality, and complications of congenital heart disease.
Clinical symptoms and ultrasound signs during pregnancy could suggest the presence of esophageal atresia (EA). However, most often EA is diagnosed postnatally. The aim of our study is to evaluate the course and outcome for prenatally and postnatally diagnosed EA. In addition, we studied the outcome of isolated versus nonisolated EA.
In a retrospective data analysis, ultrasound characteristics, maternal and neonatal variables as well as clinical outcome were compared for fetuses/neonates with prenatal (n = 30) or postnatal (n = 49) diagnosis of EA. Clinical outcome in terms of morbidity and mortality of isolated EA was compared with that of EA complicated by chromosomal or structural anomalies.
Prenatally diagnosed children were born 2 weeks earlier than postnatally diagnosed children (36.4 weeks vs 38.2 weeks; P = 0.02). The former had higher mortality rates (30 vs 12%; P = 0.05) and more associated anomalies (80 vs 59%; P = 0.04). In both subsets, there was a high morbidity rate in the survivors (not significant). Nonisolated EA was associated with greater occurrence of polyhydramnios (53 vs 27%; P = 0.04) and higher mortality rate (28 vs 0%; P = 0.002).
Mortality was significantly higher in prenatally diagnosed infants and in infants with additional congenital anomalies. Isolated EA is associated with good outcome.
Esophageal atresia and/or tracheoesophageal fistula (EA/TEF) are severe congenital anomalies. Although recent years have brought significant improvement in clinical treatment, our understanding of the etiology of these defects is lagging. Many genes and genetic pathways have been implicated in the development of EA/TEF, but only a few genes have been shown to be involved in humans, in animals, or in both. Extrapolating data from animal models to humans is not always straightforward. Environmental factors may also carry a risk, but the mechanisms are yet to be elucidated. This review gives an overview of the current state of knowledge about both genetic and environmental risk factors in the etiology of EA/TEF.
Although initial prognosis of oesophageal atresia is nowadays excellent with more than 95% of survival, the long-term complications are frequent. A gastro-oesophageal reflux is found in 26 to 75% of the cases, responsible for peptic oesophagitis, anastomotic stenosis and Barrett's oesophagus, risk factor of adenocarcinoma of the oesophagus. A dysphagia is frequently observed on these patients, sometimes several years after the surgery, observed in almost 45% of five-year-old children. Growth retardation is found in nearly a third of these children. Respiratory symptoms are particularly frequent, especially in the first years, associating tracheomalacia facilitating the bronchopulmonary infectious episodes (found in about 30% of 5-year-old children). Esotracheal fistula recurrence is very rare. A deformation of the rib cage is reported in 20%, and a scoliosis in 10% of the patients. However, the quality of life of these patients in the adulthood is good, and influenced by the existence of associated malformations. Even if the current prognosis of oesophageal atresia is good altogether, the frequency of the complications (digestive, respiratory, nutritional, orthopaedic) far from the initial intervention, and the necessity of a surveillance of the secondary oesophageal damages, justifies a systematic and multidisciplinary follow-up until adulthood.
The authors analyzed the outcome for 357 infants with oesophageal atresia and 15 with H-type tracheoesophageal fistula treated from 1980 through 1992. Survival according to Waterston risk categories was 99% for group A, 95% for group B, and 71% for group C. Presently, with optimal management, virtually all infants in groups A and B should survive. When examining the risk factors in the infants who died, two criteria were found to be important predictors of outcome: birth weight of less than 1,500 g and the presence of major congenital cardiac disease. A new classification for predicting outcome in oesophageal atresia is proposed: group I: birth weight > or = 1,500 g, without major cardiac disease, survival 97% (283 of 293); group II: birth weight < 1,500 g, or major cardiac disease, survival 59% (41 of 70); and group III: birth weight < 1,500 g, and major cardiac disease, survival 22% (2 of 9).
The total prevalence rate of tracheo-oesophageal fistula and oesophageal atresia in 15 EUROCAT registries covering 1,546,889 births during 1980-8 was 2.86 per 10,000. There was a decreasing prevalence rate over time (3.5 per 10,000 in 1980-2, 2.7 in 1983-5, 2.5 in 1986-8). Ten per cent of cases were associated with chromosomal anomalies and of the remaining cases, half were multiply malformed. Sixty two per cent of cases were males. There was a significantly increased risk for mothers of less than 20 years of age (odds ratio compared with mothers of 25-29 = 1.82, 95% confidence interval 1.23 to 2.67). There were no apparent epidemiological differences between isolated and multiply malformed cases in secular trend, sex ratio, or maternal age. Both isolated and multiply malformed cases tended to be premature and small for gestational age. There was variation between centres in survival of affected liveborn children up to 1 year of age.
Oesophageal atresia-tracheo-oesophageal fistula has featured in paediatric surgery since its beginnings. The first successful primary repair was in 1941. With overall survival now exceeding 90% in dedicated centres, the emphasis has changed to reducing morbidity and achieving improvements in the quality of life. An overview of current and emerging strategies in managing patients with this condition is presented. Advances in developmental biology and molecular genetics reflecting improved understanding of the pathogenesis are highlighted.
Ultrasonographic features suggestive of esophageal atresia with or without tracheo‐esophageal fistula (EA/TEF) are only in a small minority of fetuses with EA/TEF (<10%) identifiable on prenatal scans.
The prenatal diagnosis of EA/TEF relies in principle, on two nonspecific signs: polyhydramnios and absent or small stomach bubble. Polyhydramnios is associated with a wide range of fetal abnormalities, but most commonly it pursues a benign course. Similarly the sonographic absence of a stomach bubble may point to a variety of fetal anomalies.
The combination of polyhydramnios and absent stomach bubble in two small series offers a modest positive predictive value of 44 and 56% respectively. Prenatal scanning for EA/TEF identifies a larger proportion of fetuses with Edwards syndrome; there is also a higher proportion of isolated EA in comparison to postnatal studies.
Current ultrasound technology does not allow for a definite diagnosis of EA/TEF and therefore, counseling of parents should be guarded.
Postnatal diagnosis of EA is confirmed by the failure to pass a firm nasogastric tube into the stomach; on chest X‐ray, the tube is seen curling in the upper esophageal pouch. Corrective surgery for EA/TEF is well established and survival rates of over 90% can be expected. Copyright © 2008 John Wiley & Sons, Ltd.
Esophageal atresia and tracheaoesophageal fistula: newborn surgery
- Dpd Losty
- W B Jawaid
- B A Khalil
Losty DPD, Jawaid WB, Khalil BA. Esophageal atresia and tracheaoesophageal fistula: newborn surgery. 3rd ed. P Puri Hodder Arnold;
2011. p. 387-400.
EUROCAT Working Group: oesophageal atresia: prevalence, prenatal diagnosis and Fig. 1 Number of cases in 2008-2009 per center and survival. Each bar represents total cases during the 2-year study in each single center participating in the register
- R N Pedersen
- E Calzolari
- S Husby
Pedersen RN, Calzolari E, Husby S, et al. EUROCAT Working
Group: oesophageal atresia: prevalence, prenatal diagnosis and
Fig. 1 Number of cases in 2008-2009 per center and survival. Each bar represents total cases during the 2-year study in each single center
participating in the register. In white the number of alive patients, in black the dead patients.
associated anomalies in 23 European regions. Arch Dis Child
2012;97:227-32.
per center and survival. Each bar represents total cases during the 2-year study in each single center participating in the register In white the number of alive patients, in black the dead patients. associated anomalies in 23 European regions
- Fig
Fig. 1 Number of cases in 2008–2009 per center and survival. Each bar represents total cases during the 2-year study in each single center
participating in the register. In white the number of alive patients, in black the dead patients.
associated anomalies in 23 European regions. Arch Dis Child
2012;97:227-32.