Endoscopic findings in children born with oesophageal atresia in an academic unit in South Africa.

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

CHRND variant in a paternally inherited esophageal atresia and tracheoesophgageal fistula: Report of a case.

BACKGROUND: The familial occurrence of esophageal atresia and tracheoesophageal fistula (EA-TEF) is very rare and the genetic basis behind the isolated familial cases have not been identified. A male infant born with EA-TEF and his affected father were evaluated with whole genome sequence to define a genetic causative variation in paternally inherited EA-TEF. CASE REPORT: A male infant was born to 29-years-old, gravida 1, para 1 women by normal vaginal delivery. The patient was diagnosed as Type-C EA-TEF. In his family history, his father was also operated for EA-TEF during neonatal period. He had no associated anomaly despite patent foramen ovale. Genomic DNAs were extracted from peripheral blood of the patient and the father. When causative genes responsible for EA-TEF were filtered out, four different variants in NOTCH2, SAMD9, SUPT20H and CHRND were found. Except the variant found in CHRND (NM_000751.2, c.381C>G, p.(Tyr127Ter)), other three variants were not found to be segregated with the father who has EA-TEF also. This nonsense variant was not found in GnomAD database. CONCLUSION: CHRND variant found in both EA-TEF patient and his affected father suggest that CHRND variant might possibly be considered as one of the causative genetic variants in familial isolated EA-TEF patients.

The French Experience with a Population-Based Esophageal Atresia Registry (RENATO).

This paper presented a national register for esophageal atresia (EA) started in January 2008. We report our experience about the conception of this database and its coordination. Data management and data quality are also detailed. In 2023, more than 2,500 patients with EA are included. Prevalence of EA in France was calculated at 1.8/10,000 live birth. Main clinical results are listed with scientific publications issued directly from the register.

Management and Outcomes of Esophageal Atresia With or Without Tracheo-Esophageal Fistula Over 15 Years in South Africa.

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

Combined esophageal and duodenal atresia: A review of the literature from 1950 to 2020.

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.

[Epidemiological, clinical and therapeutic profile of esophageal atresia at the Mother and Child University Hospital of the Jeanne Ebori Foundation from 2019 to 2020]. / Profil épidémiologique, clinique et thérapeutique des atrésies de l'œsophage au Centre Hospitalier Universitaire Mère-Enfant Fondation Jeanne Ebori de Libreville de 2019 à 2020.

Esophageal atresia is a congenital malformation incompatible with life. Its management in our context is difficult. The purpose of this study was to determine the clinical, therapeutic and epidemiological profile of esophageal atresia at the Mother and Child University Hospital of the Jeanne Ebori Foundation from 2019 to 2020. We conducted a retrospective study in the Neonatal Medicine and Pediatric Surgery Department of Mother and Child University Hospital of the Jeanne Ebori Foundation over a 2-year period from January 1, 2019 to December 31, 2020. We collected data from the medical records of 10 newborns diagnosed with esophageal atresia. The prevalence of neonatal surgical emergencies was 11% (n=10/89). Mean gestational age of patients was 37 WA+2, ranging from 34WA+2 days to 40WA+2 days; mean life span was 3.7 days with extremes of D0 and D7. Sex ratio was 1. The average birth weight was 2636 grams, ranging from 1460 g to 3425g; mean time between symptom onset and diagnosis was 4 days. Type III accounted for 70% of anatomical shapes. Mean waiting time for surgery was 0.8 days, ranging from D0 to D3. The average length of stay in hospital was 9.3 days, ranging from 1 to 38 days. Nine out of 10 patients underwent surgery. Only 2 patients survived, with a follow-up period of at least one year without sequelae. The management of esophageal atresia is still precarious in our context. Diagnostic delay is the main challenge. Improvement in prognosis involves improvement in anesthetic, surgical and neonatal technical equipment.

Parental risk factors for oesophageal atresia.

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.

Esophageal Surveillance Practices in Esophageal Atresia Patients: A Survey by the Eastern Pediatric Surgery Network.

INTRODUCTION: Endoscopic surveillance guidelines for patients with repaired esophageal atresia (EA) rely primarily on expert opinion. Prior to embarking on a prospective EA surveillance registry, we sought to understand EA surveillance practices within the Eastern Pediatric Surgery Network (EPSN). METHODS: An anonymous, 23-question Qualtrics survey was emailed to 181 physicians (surgeons and gastroenterologists) at 19 member institutions. Likert scale questions gauged agreement with international EA surveillance guideline-derived statements. Multiple-choice questions assessed individual and institutional practices. RESULTS: The response rate was 77%. Most respondents (80%) strongly agree or agree that EA surveillance endoscopy should follow a set schedule, while only 36% claimed to perform routine upper GI endoscopy regardless of symptoms. Many institutions (77%) have an aerodigestive clinic, even if some lack a multi-disciplinary EA team. Most physicians (72%) expressed strong interest in helping develop evidence-based guidelines. CONCLUSIONS: Our survey reveals physician agreement with current guidelines but weak adherence. Surveillance methods vary greatly, underscoring the lack of evidence-based data to guide EA care. Aerodigestive clinics may help implement surveillance schedules. Respondents support evidence-based protocols, which bodes well for care standardization. Results will inform the first multi-institutional EA databases in the United States (US), which will be essential for evidence-based care. LEVEL OF EVIDENCE: This is a prognosis study with level 4 evidence.

Prevalence of Mental Health Problems, Associated Factors, and Health-Related Quality of Life in Children with Long-Gap Esophageal Atresia in Sweden.

BACKGROUND: Children with long-gap esophageal atresia (LGEA) face a high risk of digestive and respiratory morbidity, but their mental health outcomes have not been investigated. We aimed to identify the prevalence of mental health problems in children with LGEA, associated factors and health-related quality of life (HRQOL). METHODS: Twenty-six children with LGEA aged 3-17 were recruited nationwide in Sweden. One of their parents and adolescents aged 11-17 completed information on the child's mental health (Strength and Difficulties Questionnaire), generic (PedsQL 4.0) and condition-specific HRQOL (EA-QOL). Parents gave information on current child symptomatology. Mental health level was determined using validated norms; abnormal≥90 percentile/borderline≥80 percentile/normal. Elevated levels were considered borderline/abnormal. Data were analyzed using descriptives, correlation and Mann-Whitney-U test. Significance level was p < 0.05. RESULTS: Twelve children with LGEA aged 3-17 (46%) had elevated scores of ≥1 mental health domain in parent-reports, whereas 2 adolescents (15%) in self-reports. In parent-reports, 31% of the children had elevated levels of peer relationship problems, with associated factors being child sex male (p = 0.037), airway infections (p = 0.002) and disturbed night sleep (p = 0.025). Similarly, 31% showed elevated levels of hyperactivity/inattention, and associated factors were male sex (p = 0.005), asthma (p = 0.028) and disturbed night sleep (p = 0.036). Elevated levels of emotional symptoms, seen in 20%, were related to swallowing difficulties (p = 0.038) and vomiting problems (p = 0.045). Mental health problems correlated negatively with many HRQOL domains (p < 0.05). CONCLUSIONS: Children with LGEA risk mental health difficulties according to parent-reports, especially peer relationship problems and hyperactivity/inattention, with main risk factors being male sex, airway problems and sleep disturbances. This should be considered in follow-up care and research, particularly since their mental health problems may impair HRQOL. LEVELS OF EVIDENCE: Prognosis study, LEVEL II.

Sex-specific percentiles for bodyweight and height in children born with esophageal atresia: a registry-based analysis 2001-2021.

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

Does the History of Tracheoesophageal Fistula Repair Alter Outcomes of Laryngeal Cleft Repair?

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

The risk of autism spectrum disorder and intellectual disability but not attention deficit/hyperactivity disorder is increased in individuals with esophageal atresia.

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

New insights in the prevalence of scoliosis and musculoskeletal asymmetries in adolescents with esophageal atresia.

BACKGROUND: Increased risk of scoliosis and musculoskeletal abnormalities in adolescents with esophageal atresia (EA) is reported, but the impact of these abnormalities on physical fitness and motor skills are not known. METHODS: Scoliosis was assessed radiographically and shoulder and chest abnormalities by a standardized protocol. Physical fitness was evaluated with Grippit, Six-minute walk test, and International Physical Activity Questionnaire and motor skills by Motor Assessment Battery for Children. RESULTS: Sixty-seven EA adolescents median 16 (13-20) years participated. The prevalence of significant scoliosis (≥ 20º) was 12% (8/67) whereas 22% (15/67) had mild scoliosis (10-19º). Vertebral anomalies occurred in 18/67 (27%), eight of them (44%) had scoliosis. The majority of adolescents (15/23) with scoliosis did not have vertebral anomalies. Musculoskeletal abnormalities were detected in 22-78%. Balance problems occurred three times more frequently than expected (44% vs. 15%, p = 0.004). Submaximal exercise capacity was significantly reduced compared to reference values (p < 0.001). Scoliosis ≥ 20º was related to reduced physical activity (p = 0.008), and musculoskeletal abnormalities to reduced physical activity and impaired motor skills (p = 0.042 and p < 0.038, respectively). CONCLUSIONS: Significant scoliosis was diagnosed in 12% of the EA adolescents and related to reduced physical activity. Musculoskeletal abnormalities identified in more than half of the patients, were related to reduced physical activity and impaired motor skills, and exercise capacity was significantly below reference group. EA patients with and without vertebral anomalies need health-promoting guidance to prevent impaired motor skills and consequences of reduced physical activity. LEVEL OF EVIDENCE: Prognostic Study, Level II.

Guía de Práctica Clínica en atresia esofágica / Clinical practice guide on esophageal atresia

La atresia esofágica es una de las anomalías congénitas más frecuentes en la práctica quirúrgica neonatal. Se estima que tiene una incidencia de 1 por cada 3500 recién nacidos vivos a nivel mundial. La preparación de guías de actuación y protocolos asistenciales es tendencia en la práctica médica actual. Esta Guía de Práctica Clínica se elaboró respondiendo a la necesidad de protocolizar la atención médico-quirúrgica de la atresia esofágica. En el Centro Territorial de Cirugía Neonatal de Holguín, donde se regionaliza la atención a neonatos de las cinco provincias orientales del país con afecciones congénitas y quirúrgicas de alta complejidad, la atresia esofágica fue la afección quirúrgica más frecuente en los últimos diez años, con una supervivencia ascendente que alcanzó 94,4 por ciento en 2019. La guía que se presenta se aprobó en el Primer Consenso Nacional de Guías de Prácticas Clínicas en Cirugía Pediátrica, en Varadero, Matanzas en 2019. Incluye las principales pautas para el diagnóstico, tratamiento y seguimiento de los pacientes afectados y se considera una herramienta eficiente para mejorar los resultados en la asistencia médica y quirúrgica neonatal(AU)

Esophageal atresia is one of the most common congenital anomalies in neonatal surgical practice. It is estimated to have an incidence of 1 per 3500 live newborns globally. The preparation of action guides and care protocols is a trend in current medical practice. This Clinical Practice Guide was prepared in response to the need to protocolize the medical-surgical care of esophageal atresia. In the Territorial Center for Neonatal Surgery of Holguín, where the care of neonates from the five eastern provinces of the country with congenital and surgical conditions of high complexity is regionalized, esophageal atresia was the most frequent surgical condition in the last ten years, with an ascending survival that reached 94.4 percent in 2019. The guideline presented was approved in the First National Consensus of Clinical Practice Guidelines in Pediatric Surgery, in Varadero, Matanzas in 2019. It includes the main guidelines for the diagnosis, treatment and follow-up of affected patients and is considered an efficient tool to improve outcomes in neonatal medical and surgical care(AU)

Can 18-years of data from a tertiary referral center help to identify risk factors in esophageal atresia?

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

Esophagic atresia type and its association with heart malformations in a Northern Mexico hospital.

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

OBJETIVO: Conocer la frecuencia de malformaciones cardiacas asociadas en pacientes con atresia de esófago y su tipo en el Hospital Infantil de Especialidades de Chihuahua, así como las características sociodemográficas relacionadas. MÉTODO: Se estudiaron la epidemiología, la clínica y la evolución de los pacientes con diagnóstico de atresia esofágica que ingresaron a dicho nosocomio durante un periodo de 2 años. Se analizaron variables como sexo, edad gestacional, peso al nacer, Apgar, tipo de atresia, malformaciones congénitas asociadas, complicaciones durante la estancia hospitalaria y aspectos relacionados con los padres. RESULTADOS: Se estudiaron 12 pacientes, de los cuales el 50% eran de sexo masculino, y la mayoría de ellos fueron producto a término con peso adecuado al nacimiento. Predominio de etnia mestiza, hijos de madres jóvenes con nivel socioeconómico medio, sin predominio geográfico. El 82% de los casos correspondían a atresia esofágica tipo III, y las malformaciones congénitas más frecuentes asociadas fueron las cardiacas en el 83% de los casos, de las cuales el 90% correspondían a defectos del tabique auricular. CONCLUSIONES: La atresia esofágica es una malformación congénita relativamente común y de etiología multifactorial. Es necesario realizar un abordaje completo de los pacientes con esta patología para poder identificar otra afección y brindar el tratamiento adecuado.

Feeding disorders in children with oesophageal atresia: a cross-sectional study.

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.

Prevalence of acid gastroesophageal reflux disease in infants with esophageal atresia/tracheoesophageal fistula.

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