Tracheoesophageal Fistula With Esophageal Atresia: A Case Series.

Tracheoesophageal fistula (TEF) with or without esophageal atresia (EA) results from maldevelopment of the trachea and esophagus during maturation of the primitive foregut. EA/TEF commonly presents shortly after birth because of increased oral secretions and the inability to advance a nasogastric or orogastric tube to the proper depth. Given that prenatal diagnosis is uncommon and early intervention is important to reduce morbidity and mortality risk, early recognition and diagnosis are imperative. We present a case series of two neonates diagnosed with EA/TEF, type "C" and type "E," born at low-acuity centers, who required transport to a tertiary center for surgical support. The pathophysiology as well as types of TEFs, symptomology, stabilization goals, corrective treatment, and long-term implications will be examined. Finally, the educational needs of parents and caregivers will be discussed.

The International Network on Oesophageal Atresia (INoEA) consensus guidelines on the transition of patients with oesophageal atresia-tracheoesophageal fistula.

Oesophageal atresia-tracheoesophageal fistula (EA-TEF) is a common congenital digestive disease. Patients with EA-TEF face gastrointestinal, surgical, respiratory, otolaryngological, nutritional, psychological and quality of life issues in childhood, adolescence and adulthood. Although consensus guidelines exist for the management of gastrointestinal, nutritional, surgical and respiratory problems in childhood, a systematic approach to the care of these patients in adolescence, during transition to adulthood and in adulthood is currently lacking. The Transition Working Group of the International Network on Oesophageal Atresia (INoEA) was charged with the task of developing uniform evidence-based guidelines for the management of complications through the transition from adolescence into adulthood. Forty-two questions addressing the diagnosis, treatment and prognosis of gastrointestinal, surgical, respiratory, otolaryngological, nutritional, psychological and quality of life complications that patients with EA-TEF face during adolescence and after the transition to adulthood were formulated. A systematic literature search was performed based on which recommendations were made. All recommendations were discussed and finalized during consensus meetings, and the group members voted on each recommendation. Expert opinion was used when no randomized controlled trials were available to support the recommendation. The list of the 42 statements, all based on expert opinion, was voted on and agreed upon.

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.

Clinician Knowledge of Societal Guidelines on Management of Gastrointestinal Complications in Esophageal Atresia.

OBJECTIVES: The aim of this study was to assess whether clinicians approached the management of children with esophageal atresia (EA) in accordance with the 2016 European Society of Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN)/North American Society of Paediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) guidelines on the management of gastrointestinal and nutritional complications in this cohort. METHODS: We invited expert physicians and surgeons closely involved in the care of children with EA (members of the International network on esophageal atresia [INoEA], ESPGHAN EA working group, French national EA registry, European pediatric surgical association (EUPSA), and European rare disease reference network [ERNICA]) to participate in an anonymous online survey containing 15 multiple choice questions concerning the management of gastrointestinal and nutritional complications in children with EA. Questions were based on the management of gastroesophageal reflux disease (GERD) dysphagia, cyanotic spells, feeding and nutrition, anastamotic strictures, and transition to adult care as detailed in the 2016 guidelines. RESULTS: Median concordance with ESPGHAN/NASPHAN EA Guidelines was 69% (16-100%, SD 16%) across all responders. Areas of greatest concordance were in the fields of surveillance endoscopy and medical management of GERD. Areas for potential educational opportunities include: the differential diagnosis and appropriate investigation of dysphagia and the diagnostic evaluation of extraesophageal symptoms. CONCLUSIONS: This survey highlights the importance of improving the understanding and adherence to the EA guidelines amongst clinicians involved in the care of these patients.

Care of the COVID-19 exposed complex newborn infant.

As we confront COVID-19, the global public health emergency of our times, new knowledge is emerging that, combined with information from prior epidemics, can provide insights on how to manage this threat in specific patient populations. Severe Acute Respiratory Syndrome (SARS) and Middle East Respiratory Syndrome (MERS), both caused by coronaviruses, caused serious respiratory illness in pregnant women that resulted in adverse perinatal outcomes. Thus far, COVID-19 appears to follow a mild course in the vast majority of pregnant women. A significant proportion of pregnant women appear to be asymptomatic carriers of SARS-CoV-2. However, there is limited information on how COVID-19 impacts the fetus and whether vertical transmission occurs. While these knowledge gaps are addressed, it is important to recognize the highly efficient transmission characteristics of SARS-C0V-2 and its potential for causing serious disease in vulnerable individuals, including health care workers. This review provides perspectives from a single center in New York City, the epicenter of the pandemic within the United States. It offers an overview of the preparations required for deliveries of newborns of mothers with COVID-19 and the management of neonates with particular emphasis on those born with complex issues.

"The Hopkins Mongol Case": The Dawn of the Bioethics Movement.

One of the earliest controversies in the modern history of bioethics was known at the time as "the Hopkins Mongol case," involving an infant with Trisomy 21 and duodenal atresia whose parents declined to consent to surgery. Fluids and feeding were withheld, and the infant died of dehydration after 15 days. The child's short life had a profound impact on the author's career and that of several others and ultimately led to changes in the care of children and adults with disabilities and the way difficult end-of-life decisions are made in US hospitals today. It also contributed to the growth of the modern bioethics movement and scholarship focused on pediatric bioethics issues.

Care recommendations for the respiratory complications of esophageal atresia-tracheoesophageal fistula.

Tracheoesophageal fistula (TEF) with esophageal atresia (EA) is a common congenital anomaly that is associated with significant respiratory morbidity throughout life. The objective of this document is to provide a framework for the diagnosis and management of the respiratory complications that are associated with the condition. As there are no randomized controlled studies on the subject, a group of experts used a modification of the Rand Appropriateness Method to describe the various aspects of the condition in terms of their relative importance, and to rate the available diagnostic methods and therapeutic interventions on the basis of their appropriateness and necessity. Specific recommendations were formulated and reported as Level A, B, and C based on whether they were based on "strong", "moderate" or "weak" agreement. The tracheomalacia that exists in the site of the fistula was considered the main abnormality that predisposes to all other respiratory complications due to airway collapse and impaired clearance of secretions. Aspiration due to impaired airway protection reflexes is the main underlying contributing mechanism. Flexible bronchoscopy is the main diagnostic modality, aided by imaging modalities, especially CT scans of the chest. Noninvasive positive airway pressure support, surgical techniques such as tracheopexy and rarely tracheostomy are required for the management of severe tracheomalacia. Regular long-term follow-up by a multidisciplinary team was considered imperative. Specific templates outlining the elements of the clinical respiratory evaluation according to the patients' age were also developed.

Tratamiento con adhesivo de fibrina asociado o no a diatermia para las fístulas traqueoesofágicas recurrentes: resultados tras más de 20 años de experiencia / Fibrin glue treatment associated or not with diathermy for recurrent tracheoesophageal fistula: our results after more than 20 years' experience

INTRODUCCIÓN: La fístula traqueoesofágica recurrente (FTER) representa una complicación frecuente (5-10%) en los pacientes con atresia de esófago (AE). La cirugía abierta de FTER implica una alta morbimortalidad, por lo que los abordajes endoscópicos suponen una alternativa prometedora. Presentamos los resultados a largo plazo de la aplicación broncoscópica de adhesivo de fibrina (AF) en pacientes con FTER secundaria a AE, técnica utilizada por primera vez en 1994 por nuestro equipo. MÉTODOS: Revisión retrospectiva de 1993 a 2019, incluyendo a todos los pacientes diagnosticados de FTER tras la reparación de AE y tratados con aplicación broncoscópica de AF. En la mayoría de los casos se aplicó diatermia previamente al sellado con AF. El número máximo de sesiones endoscópicas se estableció en cinco; en caso de persistir FTER tras la quinta sesión, se procedió a cirugía abierta. RESULTADOS: 14 pacientes con FTER fueron tratados con AF; en todos salvo los primeros 3 casos (11 pacientes, 78,6%) se aplicó diatermia concomitante. El día promedio del primer tratamiento fue el día 85 de vida (14 a 770). Los pacientes recibieron una media de 2,1 (1-5) sesiones endoscópicas. El seguimiento medio fue de 12,1 (10-20) años. El éxito global fue del 71,4%, sin apenas variar con la aplicación o no de diatermia concomitante (72,7% vs. 66,6%). CONCLUSIONES: La aplicación broncoscópica de adhesivo de fibrina asociado o no a diatermia representa una excelente opción para el tratamiento de FTER en pacientes con AE. El abordaje endoscópico debe considerarse como tratamiento de primera elección para FTER

INTRODUCTION: Recurrent tracheoesophageal fistula (RTEF) is a frequent complication (5-10%) in patients with esophageal atresia (EA). Open RTEF surgery has a high morbidity and mortality, so the endoscopic approach represents a promising alternative. We present the long-term results of fibrin glue (FG) bronchoscopic application in patients with RTEF secondary to EA, which was first used by our team in 1994. MATERIALS AND METHODS: A retrospective review of all patients diagnosed with RTEF following EA repair and treated with FG bronchoscopic application from 1993 to 2019 was carried out. In most cases, diathermy was applied prior to FG sealing. The maximum number of endoscopic sessions was 5. In case of persistent RTEF following the fifth session, open surgery was performed. RESULTS: 14 RTEF patients were treated with FG. In all but the first 3 cases (11 patients, 78.6%), diathermy was applied concomitantly. Mean first treatment day was day 85 of life (range: 14-770). Patients received a mean of 2.1 (1-5) endoscopic sessions. Mean follow-up was 12.1 (10-20) years. Overall success rate was 71.4%, without significant differences according to whether diathermy was concomitantly applied or not (72.7% vs. 66.6%). CONCLUSIONS: Fibrin glue bronchoscopic application associated or not associated with diathermy is an excellent option for RTEF treatment in EA patients. The endoscopic approach should be considered as the first-choice treatment for RTE

Evaluation of nebulized N-acetyl cysteine in outcome of esophageal atresia with tracheoesophegeal fistula.

AIM: To evaluate the role of nebulized N-acetyl cysteine (NAC) in liquefying the airway secretions and improving the outcome of patients of esophageal atresia with tracheoesophageal fistula (EA + TEF). METHODS: It was a non-randomized interventional study. Two milliliters of 10% NAC was given in a nebulized form (2:5 dilution, every six hourly) to patients of ET + TEF, along with regular suction of upper esophageal pouch. The group was compared with control, which comprised patients of EA + TEF receiving only saline nebulization. The consistency of the secretions was compared by hand held consistometer in unit of time (seconds) required to cross a predetermined distance along with gravity. RESULTS: Sixty patients were assessed. Of these, 30 patients were present in both groups. The study group showed significant (p = 0.01-0.0001) decrease in consistency of secretions from the control group after day 2 of NAC nebulization. Patients' discharge was significantly (p = 0.01) earlier in cases. There was no significant (p = 0.41) difference in mortality between the groups. No specific adverse effects were observed in the study group. CONCLUSION: It appears that nebulized NAC decreases the consistency of secretions in EA + TEF patients. It is interesting to note that the group of patients that received NAC was discharged earlier than the control group and had a higher survival rate than the control group. Whether this is directly attributable to the use of NAC is unknown. A prospective double-blinded randomized clinical trial is warranted to confirm these results. LEVEL OF EVIDENCE: Level II, prospective comparative study (non-randomized).

Nutritional Status in Adolescents with Esophageal Atresia.

OBJECTIVES: To examine factors that may affect nutritional status in adolescents with esophageal atresia. STUDY DESIGN: Anthropometric measurements, blood samples, pH measuring, mapping of dysphagia with a modified Easting Assessment Test questionnaire, 4-day dietary record, and a semistructured interview about eating habits and nutrition counseling were performed in a cross-sectional cohort of adolescents with esophageal atresia. RESULTS: Out of 102 eligible patients, 68 (67%) participated. The median height-for-age Z score was -0.6 (-4.6 to 1.8). Ten (15%) were classified as stunted (height-for-age Z score <-2). Fourteen (21%) were overweight. More than two-thirds reported symptoms of dysphagia (Easting Assessment Test score ≥3) and avoided specific foods. Forty-eight (71%) completed the 4-day dietary record, which showed daily intake of energy below age-appropriate recommendations. One-third reported an energy intake below their estimated basal metabolic rate. Only 24% had received counselling from a dietitian. CONCLUSIONS: Adolescents with a history of esophageal atresia have growth below reference values and energy intake below recommendations. Energy intake and its relation to stunting needs to be further studied in patients with esophageal atresia.

Fixed the gap, solved the problem? Eating skills in esophageal atresia patients at 3 years.

Although eating problems have been described as long-term morbidities of esophageal atresia (EA), there have been few studies exploring eating outcomes in children born with EA as primary aim. Parents of children operated on for EA in our Institution from January 2012 to January 2016, answered a telephone structured interview developed specifically to conduct the present study, assessing eating skills at 3 years of age. Clinical data were collected from children's medical records. Parents (45 mothers and 6 fathers) of 51 children (male = 34; female = 17) with a median age of 3.5 years form the object of the study. Considering eating problems, parents reported that 23 children (45%) still have episodes of choking during meals at 3 years of age, 9 (45%) of these have more than one episode a week, and 19 parents (39%) reported higher levels of anxiety during mealtimes. Forty-four children (86%) were described by their parents as able to eat alone, 32 (65%) accepted all food textures and 45 (90%) was described as curious about food (3 years). Forty-three (86%) parents let their children eat with other people. Correlations showed that weaning age was significantly associated with number of dilatations (rs = 0.35, P = 0.012), days of mechanical ventilation (rs = 0.40, P < 0.001), and presence of gastrostomy tube at discharge (rs = 0.45, P < 0.001). Chewing age resulted associated with number of dilatations (rs = 0.34, P < 0.01) and days of mechanical ventilation (rs = 0.38, P < 0.01). Presence of choking episodes was associated with curiosity about food (rs = 0.29, P < 0.05), while frequent choking episodes were associated with higher parental anxiety during mealtimes (rs = 0.45, P < 0.05). In order to prevent delay in the achievement of eating developmental milestones in children operated on of EA, we advocate a dedicated preventive intervention from birth to follow-up.

Use of Magnets as a Minimally Invasive Approach for Anastomosis in Esophageal Atresia: Long-Term Outcomes.

Introduction: The majority of esophageal atresia (EA) patients undergo surgical repair soon after birth. However, factors due to patient characteristics, esophageal length, or surgical complications can limit the ability to obtain esophageal continuity. A number of techniques have been described to treat these patients with "long-gap" EA. Magnets are a nonsurgical alternative for esophageal anastomosis. The purpose of this study was to report long-term outcomes for the use of magnets in EA. Materials and Methods: Between July 2001 and December 2017, 13 patients underwent placement of a magnetic catheter-based system under fluoroscopic guidance at six institutions. Daily chest radiographs were obtained until there was union of the magnets. Magnets were then removed and replaced with an oro- or nasogastric tube. Complications and outcomes were recorded. The average length of follow-up was 9.3 years (range 1.42-17.75). Results: A total of 85% of the patients had type A, pure EA, and 15% had type C with previous fistula ligation. The average length of time to achieve anastomosis was 6.3 days (range 3-13). No anastomotic leaks occurred, and all of the patients had an expected esophageal stenosis that required dilation given the 10F coupling surface of the magnets (average 9.8, range 3-22). Six patients (46%) had retrievable esophageal stents, and two underwent surgery; yet all maintained their native esophagus without interposition. A total of 92% were on full oral feeds at the time of follow-up. Conclusion: The use of magnets for treatment of long-gap EA is safe and feasible and accomplished good long-term outcomes. The main complication was esophageal stricture, although all patients maintained their native esophagus. A prospective observational study is currently enrolling patients to evaluate the safety and benefit of a catheter-based magnetic device for EA.

Esophageal Atresia: State of the Art in Translating Experimental Research to the Bedside.

Long-gap esophageal atresia is one of the most challenging diseases in the field of pediatric surgery. There is no optimal therapy, and thus many potential therapies and techniques are being actively explored, both in animal models and in neonates. This article will review the available experimental treatment options with a focus on novel techniques.

Oesophageal atresia with tracheo-oesophageal fistula, ileal atresia and Hirschsprung's disease: outcome of a rare phenotype.

Oesophageal atresia with or without tracheo-oesophageal fistula, ileal atresia and Hirschsprung's disease are surgical malformations of the gastrointestinal tract typically diagnosed early in the neonatal period and varying in severity and prognosis. This report describes a full-term male newborn presenting simultaneous oesophageal atresia with distal tracheo-oesophageal fistula, ileal atresia and Hirschsprung's disease. In addition to the complex types of gastrointestinal malformations involved, the combination of ileal atresia and Hirschsprung's disease, as well as ganglion cells distal to intestinal atresia, resulted in a challenging diagnosis. Despite a successful outcome, the patient presented increased morbidity and prolonged hospitalisation. We highlight some important findings that may aid the early diagnosis of Hirschsprung's disease in this clinical setting. To our knowledge, the association of oesophageal atresia/tracheo-oesophageal fistula, ileal atresia and Hirschsprung's disease has not been previously reported.

Prevalence and Predictive Factors of Histopathological Complications in Children with Esophageal Atresia.

OBJECTIVES: Endoscopic follow-up after esophageal atresia (EA) tracheoesophageal fistula (TEF) repair is recommended to detect esophageal histopathological complications. We investigated the prevalence of histopathologically proven esophageal complications (peptic esophagitis, gastric metaplasia, and eosinophilic esophagitis) and assessed the predictors of these complications in children with EA-TEF. MATERIALS AND METHODS: This is a prospective longitudinal cohort study performed between September 2005 and December 2014 comprising 77 children with EA-TEF followed-up until February 2017. Univariate analysis was performed using the Wilcoxon's rank-sum test for continuous variables and the Pearson's chi-square test for categorical variables. Multivariable analysis was performed using a Cox regression hazard model. The association between clinical factors and histopathologically proven complications was estimated using a Cox regression hazard model with time until the appearance of complications as the time scale. RESULTS: All 77 children received proton pump inhibitors (PPIs) (n = 73) or H2 receptor antagonists (H2RA). A total of 252 endoscopies were performed in 73 children (median 2.6/child, range: 1-29). Median age at study completion was 4.9 years (range: 2.3-11.5 years). Histopathologically proven complications occurred in 38 children (52%): peptic esophagitis (n = 32, 44%), eosinophilic esophagitis (n = 15, 21%), and gastric metaplasia (n = 9, 12%). A total of 82% patients were on PPI or H2RA at the time of diagnosis of histological complication. Multivariable Cox regression analysis showed that patients with recurrent anastomotic strictures (>3 dilations) had a higher risk of occurrence of histopathologically proven complications over time (hazard ratio: 3.11, 95% confidence interval [CI]: 1.53-6.34). On univariate analysis, the result of the first endoscopy was not associated with the occurrence of histopathologically proven complications (odds ratio: 0.8, 95% CI: 0.16-3.95). CONCLUSION: Histopathologically proven complications with potential long-term consequences occurred in approximately 50% of children after EA-TEF repair. A history of recurrent anastomotic strictures is associated with the occurrence of these complications. The result of the first endoscopy does not predict the histopathological outcome. Children with EA-TEF warrant close and systematic long-term follow-up at specialized multidisciplinary clinics with endoscopic evaluation.

Management and outcome of neonates with a prenatal diagnosis of esophageal atresia type A: A population-based study.

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

Oral feeding outcomes in infants with esophageal atresia and tracheoesophageal fistula.

PURPOSE: The purpose of this study was to explore oral feeding outcomes in infants born with type-C esophageal atresia and tracheoesophageal fistula (EA/TEF). METHODS: A retrospective cohort study of all infants born between January 2005 and December 2015 undergoing surgery for type-C EA/TEF at the University of Alberta Hospital was performed. RESULTS: Fifty-seven infants were identified, of which 61.4% were exclusively orally feeding at discharge home. Variables anticipated to predict oral feeding were explored. Only 46% of babies with a structural cardiac anomaly had exclusive oral feeding compared to 79% without cardiac anomaly, p=0.055. Logistic regression identified the presence of structural cardiac anomaly and corrected gestational age at discharge as significant negative predictor variables for exclusive oral feeding at discharge home. Additional regression analyses found early transanastomotic feeding to be a significant positive predictor for the discontinuation of PN. CONCLUSION: We report the rate of oral feeding at discharge for infants born with type-C EA/TEF and identify predictor variables. This information is important for health care professionals and the families of children born with EA/TEF, because a significant number will go home with supplemental nutrition by gavage tube or other routes. LEVEL OF EVIDENCE: Level 2.