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Congenital tracheoesophageal fistula (TEF) is a result of the failed fusion of tracheoesophageal ridges during the 3rd week of gestation. Commonly, TEF is associated with esophageal atresia, which is detected and treated in newborns. Isolated TEF is rare with an overall incidence of 1 in 80,000 live births. Very rarely, they can present in adulthood. This report is adding one such patient at the age of 17 years who presented with the first episode of aspiration pneumonia and a history of retrosternal discomfort and regurgitation since childhood to the limited world experience.

Congenital tracheoesophageal fistula (TEF) is a result of the failed fusion of tracheoesophageal ridges during the 3rd week of gestation. Commonly, TEF is associated with esophageal atresia, which is detected and treated in newborns. Isolated TEF is rare with an overall incidence of 1 in 80,000 live births. Very rarely, they can present in adulthood. This report is adding one such patient at the age of 17 years who presented with the first episode of aspiration pneumonia and a history of retrosternal discomfort and regurgitation since childhood to the limited world experience. [ABSTRACT FROM AUTHOR]

Advanced diagnostic and therapeutic flexible bronchoscopy in children is a rapidly evolving field. Recent advances in technology and awareness of indications, risks, and benefits by pediatric providers have greatly increased the use of advanced techniques in children. This review highlights advanced diagnostic procedures including assessment of endobronchial lesions, mediastinal/hilar masses, and peripheral lung nodules as well as therapeutic techniques for restoring airway lumen patency, managing persistent air leaks, and treating tracheoesophageal fistulas in children as well as the potential to spare these patients more invasive procedures., Competing Interests: Disclosure The authors have no conflicts of interest to disclose., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Objective: To evaluate genetic testing utilization and diagnostic yield in infants with esophageal atresia (EA)/tracheoesophageal fistula (TEF) over the past 12 years to inform future practices and individualize prognostication and management., Study Design: A retrospective cohort study was performed for all infants with EA or EA/TEF hospitalized between January 2011 and January 2023 at a quaternary children's hospital. For each infant, demographic information, prenatal and postnatal history, and genetic testing were reviewed., Results: There were 212 infants who were classified as follows: 1) complex/syndromic with EA/TEF plus an additional major anatomic anomaly (n = 114, of which 74 met VACTERL criteria); 2) isolated/nonsyndromic EA/TEF (n = 88) and 3) isolated/nonsyndromic EA (n = 10). A range of genetic tests were sent with varying diagnostic rates including karyotype analysis in 12 (all with complex/syndromic phenotypes and all positive), chromosomal microarray analysis in 189 (114 of whom were complex/syndromic with an overall diagnostic rate of 3/189), single gene testing for CHD7 in 18 (4 positive), and exome analysis in 37 complex/syndromic patients (8 positive)., Conclusions: EA/TEF with and without additional anomalies is genetically heterogeneous with a broad range of associated phenotypes. While the genetic etiology of EA/TEF with or without VACTERL remains largely unknown, genome wide testing (exome or genome) including copy number analysis is recommended over chromosomal microarray testing. We anticipate that expanded genetic/genomic testing modalities such as RNA sequencing and tissue specific molecular testing are needed in this cohort to improve our understanding of the genomic contributors to EA/TEF., Competing Interests: Declaration of Competing Interest Children's Hospital of Philadelphia Research Foundation (GRT-00003206). The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2024 Elsevier Inc. All rights reserved.)

H-type tracheo-oesophageal fistula is an uncommon type of tracheo-oesophageal malformation. Acute gastric volvulus is another infrequent pathology in children. They rarely present together.We report the case of a toddler with acute gastric volvulus possibly secondary to an undiagnosed H-type tracheo-oesophageal fistula. The fistula was suspected due to persistent gastric distention observed during volvulus detorsion. This kind of tracheo-oesophageal fistula often presents with subtle symptoms making early diagnosis difficult.Acute gastric volvulus is a life-threatening condition. Gastric distension caused by the passage of air into the stomach through the fistula could be a triggering factor for gastric volvulus., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

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., (© Copyright 2024 Springer Publishing Company, LLC.)

Treatment of patients with post-intubation (post-tracheostomy) stenotic laryngotracheal lesions combined with tracheoesophageal fistula is the most difficult problem for various specialists. A 20-year-old patient received a severe concomitant injury with necessary prolonged mechanical ventilation and tracheostomy. Decannulation was followed by shortness of breath and cough with discharge of sputum mixed with liquid and food. Post-tracheostomy total cicatricial atresia of the larynx and cervical trachea combined with cricoid cartilage fracture, as well as tracheoesophageal fistula of cervical trachea was diagnosed. Tracheostomy and gastrostomy were performed. After 3-month rehabilitation, the patient admitted to the Vishnevsky National Medical Research Center of Surgery. After additional examination, the patient underwent circular laryngotracheal resection, closure of tracheoesophageal fistula, laryngotracheal reconstruction (laryngotracheal anastomosis by Grillo with thyroid cartilage repair), re-tracheostomy. A favorable outcome was obtained. In such patients, treatment strategy cannot be standardized and require a personalized approach.

The purpose of this study is to evaluate the cell lines seen on esophagogastoduodenoscopy (EGD) of children who have difficulties feeding, esophageal fistula, and asthma that has some histologic abnormalities.  This is a study that looked back at the medical records of 100 children whose cells had been surgically restored after being impacted by EA or TEF. A review of the instrumental tests that were carried out at our facility has been conducted in order to identify any lingering anatomic or functional abnormalities of the airways and gastrointestinal system that would explain the pulmonary clinical images. Due to neurodevelopmental sequelae and the existence of tracheostomies, only 26 sets of pre and post-bronchodilator spirometry data were available for children. 100 children cells with esophageal atresia and tracheoesophageal fistula were included in the study. The average number of weeks spent in gestation was 37.01± 2.33, and the average weight of a newborn was 2614±77.69 grammes. Twenty percent of the children's cells exhibited a syndromic appearance, and the VACTERL connection was identified in seventeen percent of the patients. In all, 80% of the children cells in the sample reported having respiratory symptoms, with 85% of patients experiencing symptoms of the lower respiratory tract and 15% experiencing symptoms of the upper respiratory tract. It is common practice to attribute children's symptoms to reflux and esophageal anomalies, despite the presence of respiratory symptoms and aberrant findings discovered by flexible bronchoscopy and CT assessment.

Background: The incidence of esophageal atresia with tracheoesophageal fistula is 1 out of 3000-5000 live births. Its incidence in lower middle income countries is not known. The infants usually present with excessive secretions or choking while feeding and are at risk for aspiration. The outcome of these infants in lower middle income countries is not encouraging due to delays in referral, sepsis at presentation requiring preoperative stabilization, postoperative complications such as anastomosis leaks, pneumonia, and pneumothorax., Case Presentation: We present two African babies who were term infants at age 2 days (male) and 5 days (female) with diagnosis of esophageal atresia and tracheoesophageal fistula. The 5-day-old infant required preoperative stabilization due to sepsis and delayed surgery with a poor postoperative outcome. The 2-day-old infant was preoperatively stable and had a good postoperative outcome. The challenges faced in management of these two cases have been highlighted., Conclusion: Outcome of infants with esophageal atresia and tracheoesophageal fistula in lower middle income countries is not encouraging due to delays in referral and poor postoperative healing attributed to sepsis and recurrent pneumothorax. Timely referral, preoperative condition of the infant, and timely management has shown to be a contributory factor for an improved outcome., (© 2023. The Author(s).)

Infants born with esophageal atresia and tracheoesophageal fistula, a complex congenital malformation occurring in 1/2500-4000 live births, may suffer threats to their cardiac, respiratory, and digestive health in addition to anomalies that may exist in the genitourinary and musculoskeletal systems. Optimal care for these patients throughout their lives is best achieved through a coordinated, multidisciplinary approach that our health care system is not always well-equipped to provide. This review, though not exhaustive, highlights the components of care that pertain to initial surgical reconstruction and subsequent diagnosis and management of the complications that are most frequently encountered. Authors from among the many specialties involved in the care of these patients summarize the current best practice with attention to the most recent advances. Assessment and improvement of quality of life and transition to adult specialists as children grow to adulthood is also reviewed., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Background: Oesophageal atresia with or without a tracheo-oesophageal fistula is a congenital abnormality that usually requires surgical repair within the first days of life., Objective: Description of the perioperative anaesthetic management and outcomes of neonates undergoing surgery for oesophageal atresia with or without a tracheo-oesophageal fistula, included in the 'neonate and children audit of anaesthesia practice in Europe' (NECTARINE) database., Design: Sub-analyses of prospective observational NECTARINE study., Setting: European multicentre study., Patients: Neonates who underwent surgery for oesophageal atresia with or without a tracheo-oesophageal fistula in the NECTARINE cohort were selected., Main Outcome Measures: Incidence rates with 95% confidence intervals were calculated for peri-operative clinical events which required a predetermined intervention, postoperative complications, and mortality., Results: One hundred and three neonates undergoing a first surgical intervention for oesophageal atresia with or without a tracheo-oesophageal fistula repair were identified. Their median gestational age was 38 weeks with a median birth weight of 2840 [interquartile range 2150 to 3150] grams. Invasive monitoring was used in 66% of the procedures. The incidence of perioperative clinical events was 69% (95% confidence interval 59 to 77%), of 30-day postoperative complications 47% (95% confidence interval 38 to 57%) and the 30- and 90 days mortality rates were 2.1% and 2.6%, respectively., Conclusion: Oesophageal atresia with or without a tracheo-oesophageal fistula repair in neonates is associated with a high number of perioperative interventions in response to clinical events, a high incidence of postoperative complications, and a substantial mortality rate., (Copyright © 2023 European Society of Anaesthesiology and Intensive Care. Unauthorized reproduction of this article is prohibited.)

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., (© 2023. Springer Nature Limited.)

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., (© 2023. The Author(s).)

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

We report a patient with combination of esophageal atresia, proximal tracheoesophageal fistula and meconium peritonitis. These two rare disorders have different etiology, pathogenetic mechanisms and require different diagnostic manipulations and surgical treatments. The authors discuss the features of diagnosis and surgical treatment of this disease.

Modern approaches to intensive care and anesthesiology make it possible to save patients with various severe traumas. Prolonged mechanical ventilation allows stabilizing the patient's status, but can result severe complications including tracheoesophageal fistula. Our patient received severe combined injury after road accident, i.e. compression-comminuted fractures of DIV, DV, DVI vertebrae with spinal cord compression, ThIII compression fracture, compression-comminuted fracture of bodies and arches ThIV, V, VI with ThVI dislocation, lower paraplegia and pelvic organ dysfunction, left-sided laryngeal paresis, brain concussion and contusion of both lungs. Mechanical ventilation has been performed for 1.5 months. The patient suffered inflammatory complications: bilateral pneumonia complicated by right-sided pleural empyema, sepsis, tracheostomy suppuration followed by cervical soft tissue abscess. Pleural drainage and debridement, as well as drainage of abscess were performed. At the same time, we diagnosed external esophageal fistula at the CVII level. Tracheoesophageal fistula closure via cervical access was carried out at the first stage. Postoperative period was uneventful. After 20 days, we performed spondylosynthesis Th I -Th VII , and decompression laminectomy Th IV -Th VI . Spine stabilization by metal elements has a beneficial effect even without spinal cord recovery regarding better conditions for neurological and social rehabilitation. Thus, treatment was performed in a highly specialized multi-field hospital by thoracic surgeons and vertebrologists. We realized the treatment plan and obtained favorable results with minimal risk of postoperative complications.

Objective: To describe treatment of cicatricial tracheal stenosis and tracheoesophageal fistula in patients with COVID-19 pneumonia., Material and Methods: There were 91 patients with cicatricial tracheal stenosis for the period from August 2020 to April 2022 (21 months). Of these, 32 (35.2%) patients had cicatricial tracheal stenosis, tracheoesophageal fistula and previous coronavirus infection with severe acute respiratory syndrome. Incidence of iatrogenic tracheal injury following ventilation for viral pneumonia in the pandemic increased by 5 times compared to pneumonia of other genesis. Majority of patients had pneumonia CT grade 4 (12 patients) and grade 3 (8 patients). Other ones had pulmonary parenchyma lesion grade 2-3 or mixed viral-bacterial pneumonia. Isolated tracheoesophageal fistula without severe cicatricial stenosis of trachea or esophagus was diagnosed in 4 patients. In other 2 patients, tracheal stenosis was combined with tracheoesophageal fistula. Eight (25%) patients had tracheostomy at the first admission. This rate was almost half that of patients treated for cicatricial tracheal stenosis in pre-pandemic period., Results: Respiratory distress syndrome occurred in 1-7 months after discharge from COVID hospital. All patients underwent surgery. In 7 patients, we preferred palliative treatment with dilation and stenting until complete rehabilitation. In 5 patients, stent was removed after 6-9 months and these ones underwent surgery. There were 3 tracheal resections with anastomosis, and 2 patients underwent tracheoplasty. Resection was performed in 3 patients due to impossible stenting. Postoperative course in these patients was standard and did not differ from that in patients without viral pneumonia. In case of tracheoesophageal fistula, palliative interventions rarely allowed isolation of trachea. Four patients underwent surgery through cervical approach. There were difficult surgeries in 2 patients with tracheoesophageal fistula and cicatricial tracheal stenosis. One of them underwent separation of fistula and tracheal resection via cervical approach at primary admission. In another patient with thoracic fistula, we initially attempted to insert occluder. However, open surgery was required later due to dislocation of device., Conclusion: Absolute number of patients with tracheal stenosis, tracheoesophageal fistula and previous COVID-19 has increased by several times compared to pre-pandemic period. This is due to greater number of patients requiring ventilation with risk of tracheal injury, non-compliance with preventive protocol for tracheal injury including anti-ischemic measures during mechanical ventilation. The last fact was exacerbated by involvement of allied physicians with insufficient experience of safe ventilation in the «red zone», immunodeficiency in these patients aggravating purulent-inflammatory process in tracheal wall. The number of patients with tracheostomy was 2 times less that was associated with peculiarity of mechanical ventilation in SARS-CoV-2. Indeed, tracheostomy was a poor prognostic sign and physicians tried to avoid this procedure. Incidence of tracheoesophageal fistula in these patients increased by 2 times compared to pre-pandemic period. In subacute period of COVID-associated pneumonia, palliative measures for cicatricial tracheal stenosis and tracheoesophageal fistula should be preferred. Radical treatment should be performed after 3-6 months. Absolute indication for circular tracheal resection with anastomosis is impossible tracheal stenting and ensuring safe breathing by endoscopic methods, as well as combination of cicatricial tracheal stenosis with tracheoesophageal fistula and resistant aspiration syndrome. Incidence of postoperative complications in patients with cicatricial tracheal stenosis and previous mechanical ventilation for COVID-19 pneumonia and patients in pre-pandemic period is similar.

Purpose: Congenital tracheo-oesophageal fistula (TOF) occurs in 1 in 3000 births. Perioperative management for TOF repair requires co-ordination with a multi-disciplinary team, support from critical care units, and expertise in neonatal and cardiothoracic anaesthesia. Charlotte Maxeke Johannesburg academic hospital (CMJAH) is a quaternary referral centre that serves the regional community of Johannesburg, in Gauteng, South Africa. The aim of this research was to describe the perioperative outcomes of neonates undergoing surgical TOF repair at CMJAH. Factors in the preoperative, intra-operative, and postoperative management were considered to find relationships with the perioperative outcomes., Method: A retrospective single institution study was conducted with a population of 38 neonates who underwent congenital TOF repair from 1 January 2015 to 31 March 2020 at CMJAH. Descriptive statistics were used to describe the biodata using percentages, median, and inter-quartile ranges. An in-depth description of neonates that died was performed., Results: A total of 38 neonates diagnosed with TOF/OA were operated on during the study period. The mortality rate was 15.8%. No deaths occurred intraoperatively. In addition, 52.6% of the neonates had a prolonged stay in ICU, 44% had a delay in the initiation of feeds, 65% developed sepsis, and 36.8% had surgical related complications. CPR was required in 1 neonate, hypoxia leading to bradycardia in 10, and hyperlactataemia in 9 neonates. Inotropic support was required in 6 neonates, and vasopressor support in 4. Blood product transfusion were necessary for 9 neonates., Conclusion: The in-hospital mortality of TOF repair was better than that reported in other African countries and worse than international findings. In-hospital morbidity was burdened by respiratory illness and sepsis. Areas where management could be improved include widespread foetal anomaly scanning, incorporation of bronchoscopy, and preoperative respiratory optimisation., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Esophageal atresia/tracheoesophageal fistula (EA/TEF) is a life-threatening birth defect that often occurs with other major birth defects (EA/TEF+). Despite advances in genetic testing, a molecular diagnosis can only be made in a minority of EA/TEF+ cases. Here, we analyzed clinical exome sequencing data and data from the DECIPHER database to determine the efficacy of exome sequencing in cases of EA/TEF+ and to identify phenotypic expansions involving EA/TEF. Among 67 individuals with EA/TEF+ referred for clinical exome sequencing, a definitive or probable diagnosis was made in 11 cases for an efficacy rate of 16% (11/67). This efficacy rate is significantly lower than that reported for other major birth defects, suggesting that polygenic, multifactorial, epigenetic, and/or environmental factors may play a particularly important role in EA/TEF pathogenesis. Our cohort included individuals with pathogenic or likely pathogenic variants that affect TCF4 and its downstream target NRXN1, and FANCA, FANCB, and FANCC, which are associated with Fanconi anemia. These cases, previously published case reports, and comparisons to other EA/TEF genes made using a machine learning algorithm, provide evidence in support of a potential pathogenic role for these genes in the development of EA/TEF., (© 2022 Wiley Periodicals LLC.)

Esophageal atresia (EA) with tracheoesophageal fistula (TEF) is among the most common congenital anomalies requiring surgical intervention in infancy. General surgeons practicing in rural or austere environments may encounter emergency situations requiring their involvement. Respiratory emergencies can arise in the neonatal period; the recommended approaches are the ligation of the fistula through the chest or occlusion of the distal esophagus through the abdomen. As survivors of the condition reach late adulthood, general surgeons can anticipate encountering these patients. An understanding of risk factors, common symptoms, associated anomalies, and the appropriate diagnostic evaluation will facilitate care., Competing Interests: Disclosure The author has nothing to disclose., (Published by Elsevier Inc.)

Abstract: Esophageal atresia and tracheoesophageal fistula are often-concomitant pathologies that primarily afflict neonates. The complications of these anomalies may lead to increased morbidity and mortality, and clinicians should be familiar with the diagnosis and management of these pathologies. Clinicians can improve patient outcomes by having a thorough understanding of the signs and symptoms, classification systems, diagnostic workup, and surgical intervention options for these patients. Early recognition and treatment are imperative in providing patients with the best opportunity for recovery., (Copyright © 2022 American Academy of Physician Associates.)

Background: Interventional pediatric flexible bronchoscopy has many advantages over radiologic investigations in diagnosing uncommon congenital H-type tracheoesophageal fistula (TEF), dual TEF, bronchoesophageal fistula (BEF) and fistula recurrence including higher rates of identification and anatomic localization with guide wire cannulation. We compare the diagnostic utility of flexible bronchoscopy to radiologic techniques for congenital aerodigestive fistula., Methods: A single center retrospective review was completed of all cases of pediatric TEF and BEF diagnosed with flexible bronchoscopy between January 2000 and November 2020., Results: Fistulae were diagnosed 21 times in 18 patients at a median age of 1.22 years (interquartile range: 0.50 to 2.99). The median time from diagnosis to repair was 17.5 days (interquartile range: 5.5 to 43). Symptoms commonly related to fistula were found in all patients. Uncommon fistulae included single H-type TEF (n=10, 47.6%), dual H-type TEF (n=2, 9.5%), dual proximal and distal TEF with esophageal atresia (n=5, 23.8%), TEF recurrence (n=2, 14.3%), BEF (n=1, 4.8%), and a BEF recurrence (n=1, 4.8%). Flexible bronchoscopy confirmed the diagnosis in all fistulae using a guide wire cannulation or methylene blue dye injection. A combined procedure with simultaneous bronchoscopy and esophagoscopy was used for 6 fistulae. The positive examination rate was 75% for bronchoscopy compared with 2.6% for contrast swallow studies and 28.6% for tube esophagograms., Conclusions: Flexible bronchoscopy should be considered as a first line investigation in uncommon aerodigestive fistulae. In the absence of a skilled bronchoscopist, the best radiologic investigation is a pull-back tube esophagogram but may still require endoscopic confirmation at the time of fistula repair., Competing Interests: Disclosure: R.J.T. is supported by a NHMRC postgraduate scholarship (APP1190908). For the remaining authors there are no conflict of interest or other disclosures., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

We report a case of an adolescent girl presenting with acute respiratory distress syndrome (ARDS) requiring mechanical ventilation. Initial presentation during the ongoing COVID-19 pandemic was compatible with multisystem inflammatory response in children associated with COVID-19 (MIS-C). Subsequently a diagnosis of tuberculosis was made. During ventilation, she developed significant abdominal distension which was not relieved with nasogastric decompression. There was a high index of suspicion of bronchoenteric fistula. Bronchoscopy with adjunct oesophagoscopy demonstrated tracheo-oesophageal fistula (TEF). The classical presentation of TEF has been masked by onset of ARDS. During the pandemic the diagnosis of tuberculosis in high-burden countries decreased for multiple reasons leading to development of complications which are often confused with MIS-C. While diagnosing MIS-C, maintaining a high level of suspicion for concomitant or alternative aetiologies is essential., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

The patient was a 15 months-old boy who had been diagnosed CHARGE syndrome, which is a multiple congenital anomaly syndrome caused by mutations in the CHD7 gene. Mechanical ventilation management was initiated 2 hours after birth for dysphagia and respiratory failure, and tracheotomy was performed 3 months after birth for dysphagia and failed extubation. He was repeatedly hospitalized due to pneuomoniae. Approximately 1 year after birth, the boy had two consecutive episodes of sudden ventilatory insufficiency while replacing the tracheotomy cannula. A bronchoscopic examination under general anesthesia revealed a tracheoesophageal fistula directly below the tracheostomy. The patient was diagnosed with Gross E esophageal atresia, and we speculated that the cannula migrated to the esophagus via the fistula during tracheostomy cannula replacement. Gross E esophageal atresia is a rare disease. Its diagnosis is often delayed, and it is discovered by recurrent pneumonia in many cases. A tracheoesophageal fistula may also be found in children with deformities of the respiratory system. Furthermore, tracheoesophageal fistulae are often found in the neck. Therefore, when sudden ventilatory insufficiency occurs in a child with a tracheostomy after replacing the tracheostomy cannula, caution must be exercised since the cannula may have migrated to the esophagus via a fistula. J. Med. Invest. 69 : 141-144, February, 2022.

Objective: To determine the optimal treatment of patients with complications of esophageal electrochemical burns by batteries., Material and Methods: There were 75 children with esophageal electrochemical burns by batteries between 2010 and 2019. All children underwent X-ray of the cervical spine, chest and abdomen, esophagoscopy with removal of the battery, tracheoscopy. Complications occurred in 39 children: TEF - 21, esophageal stenosis - 19, laryngeal paresis - 14, esophageal perforation - 3. Patients with TEF were divided into 2 groups: clinically stable children without respiratory failure and severe illness with respiratory failure including mechanical ventilation. Group I consisted of 6 children, four of them underwent laparoscopic Nissen fundoplication and gastrostomy. Group II consisted of 15 children. In acute period, 3 children underwent laparoscopic fundoplication and gastrostomy, 8 ones - TEF ligation, 4 patients - tracheal repair with esophageal flap and esophageal extirpation. Patients with esophageal stenosis underwent bougienage. Patients with esophageal perforation required therapy. Tracheostomy was necessary for respiratory failure and bilateral laryngeal paresis. Lateralization procedures were performed in patients with negative course of disease., Results: In the 1st group, spontaneous closure of TEF was found in 3 children after fundoplication and gastrostomy. One child underwent thoracoscopic disconnection of TEF after reduction of fistula. In the 2nd group, fundoplication resulted spontaneous closure of fistula after 2-5 months. In 4 children, recanalization of the fistula or esophageal failure were observed in acute period after TEF ligation., Conclusion: Laparoscopic fundoplication and gastrostomy are optimal for TEF and can result complete or partial spontaneous closure of TEF. If radical procedure is necessary in acute period, tracheal repair with esophageal flap and extirpation of the esophagus with subsequent coloesophagoplasty should be considered.

Endoscopy for revealing the orifice of congenital H-type tracheoesophageal fistula (cTEF) is important for diagnostics and therapeutics. To facilitate the identification and catheterization of cTEF, we developed a new modified flexible endoscopy technique using a laryngeal mask with intermittent airflow. A retrospective case series study was conducted from April 2016 to July 2019 at a national regional children's medical center. Twelve infants with cTEF underwent this flexible endoscopy technique. The intermittent positive pressure airflow through laryngeal mask was able to reveal the orifice of cTEF easily in tracheal lumen. Under the visual flexible endoscope, cannulation with a 3-Fr ureteral catheter in fistula was successfully used in all cases. There were no immediate or delayed complications. This case series shows that the flexible endoscopy technique is a safe, easy, and technically efficient approach for diagnosis and cannulation of cTEF., (© 2021 Japan Gastroenterological Endoscopy Society.)

Objective: To describe 10-year experience of treating the cicatricial tracheal stenosis (CTS) in a regional multi-field hospital., Material and Methods: There were 120 CTS patients aged 13-75 years. In 8 (6.7%) patients, CTS was combined with tracheoesophageal fistula (TPF). Post-intubation stenosis was diagnosed in 16 (13.3%) cases, post-tracheostomy - in 102 (85%) ones, post-traumatic - in 2 (1.7%) patients. CTS length ranged from 1.2 to 8 cm. Fifty (41.7%) patients had cervical CTS, 40 (33.3%) patients - cervico-thoracic tracheal stenosis, 11 (9.2%) patients - tracheal stenosis at the thoracic level. Nineteen (15.8%) patients had multifocal stenoses. We used endoscopic techniques, circular tracheal resection (CTR) and laryngotracheal reconstruction., Results: Postoperative mortality rate was 0.83%. CTR was performed in 33 patients, laryngotracheal reconstruction - 77, endoscopic stenting - 6 patients. In 4 cases, local CTS was eliminated by bougienage and argon plasma exposure. CTS was successfully disconnected with TEF using CRT in 3 cases, laryngotracheoplasty and stenting - in 5 cases. The fenestrated tracheal defect was closed by a three-layer autologous flap in 59 patients. Of these, autologous flap was reinforced with porous nickel-titanium implants in 17 patients. Postoperative complications after CRT occurred in 6 (16.7%) patients (anastomotic leakage - 2, anastomositis - 1, restenosis - 2). No patients died. Postoperative complications after laryngotracheal reconstruction were observed in 18 (23.4%) patients including 5 ones with restenosis who underwent CTR with a favorable outcome., Conclusion: CTS treatment requires a multidisciplinary approach. Each surgery has certain indications and place in treatment algorithm. CTR is highly effective, but may be accompanied by complications associated with tracheal anastomosis. Decrease of postoperative morbidity will improve immediate and long-term results of CTS treatment. The chosen treatment algorithm ensured good and satisfactory results in 98% of patients.

Laryngotracheoesophageal clefts (LTECs) and tracheoesophageal fistulae (TEF) are important structural causes of aspiration requiring bronchoscopy for diagnosis. Determining which children are at greatest risk for LTEC and TEF would enable clinicians to be more selective in performing bronchoscopy., Methods: Medical records of children aged 0-18 years who underwent flexible and rigid bronchoscopy for evaluation of dysphagia with aspiration were collected and analyzed to identify predictors of LTEC and TEF., Results: Seventy-two children age 2 months to 9 years were identified. LTEC was identified in 19 (26%) and TEF was identified in 1 (1.3%). One-third of the cohort was born preterm (median gestational age 34 weeks). The proportion of LTEC in those born preterm was lower than that of those born full-term (12% vs. 34%, p = .03). There was no statistically significant difference in LTEC prevalence based on age, midline defects, laryngomalacia, tracheomalacia, history of TEF repair, silent aspiration, or viscosity of barium aspirated during videofluoroscopic swallowing studies. Bronchoalveolar lavage fluid cytology, lipid-laden macrophage proportions, and culture results were similar among those with and without LTEC., Conclusion: Children with dysphagia and tracheal aspiration born full-term are three times more likely to have LTEC than those born preterm. Dysphagia in children with a history of preterm birth is more likely to be functional as opposed to structural, however, LTEC was identified in approximately 10% of these children and must be considered. These results support the role of bronchoscopy in children with dysphagia with tracheal aspiration., (© 2021 Wiley Periodicals LLC.)

Background: Children born with esophageal atresia (EA) face comorbidities and complications often requiring surgery and anesthesia. We aimed to assess all procedures performed under general anesthesia during their first 12 years of life., Methods: We performed a retrospective cohort study about subsequent surgeries and procedures requiring general anesthesia in children born with type C EA between January 2007 and December 2017, with follow-up to March 2019., Results: Of 102 eligible patients, 63 were diagnosed with comorbidities, of whom 18 had VACTERL association. Follow-up time for all patients varied between 14 months and 12 years (median 7 years). The patients underwent total 637 procedures, median 4 [IQR2-7] per patient. In the first year of life, 464 procedures were performed, in the second year 69 and in the third year 29. Thirteen patients underwent no other procedures than primary EA repair. In 57 patients, 228 dilatations were performed. Other frequently performed procedures were esophagoscopy (n=52), urologic procedures (n=44) and abdominal procedures (n=33)., Conclusions: Patients with EA frequently require multiple anesthetics for a variety of procedures related to the EA, complications and comorbidities. This study can help care providers when counselling parents of a patient with an EA by giving them more insight into possible procedures they can be confronted with during childhood., Competing Interests: Declarations of Competing interest None, (Copyright © 2021 The Author(s). Published by Elsevier Inc. All rights reserved.)

Delayed presentation of oesophageal atresia (OA) with a tracheo-oesophageal fistula (TOF) is rare. Only a few case reports and two larger case series have been published. We present a neonate who was referred to our unit on day 14 of life with a missed OA and a TOF, having survived without any feeds or total parenteral nutrition up until referral.We concluded that although such a delayed presentation is rare and avoidable, it does occur. This case highlights the necessity of good feedback to the referral hospitals with education on how to prevent this from recurring again. It also emphasises the necessity of a comprehensive clinical examination of all newborn babies. A high index of suspicion for OA with or without a TOF is essential in all babies with clinical drooling, feeding problems (from the first feed) and/or respiratory symptoms especially if combined with antenatal polyhydramnios., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Esophageal atresia and tracheo-esophageal fistula (TEF), a well described congenital anomaly of the aero-digestive tract, commonly presents with inability to swallow and feed immediately after birth. However, diagnosis of recurrent or isolated TEF can be challenging and requires a combination of endoscopic and contrast studies. We describe a hitherto unreported technique of low flow intermittent oxygen insufflation into the suspicious tract and examine its safety and diagnostic yield for identification of occult TEF., Methods: A retrospective single center cohort study, analyzing case notes of patients with TEF who underwent bronchoscopic oxygen insufflation for suspected recurrent or isolated TEF between 2006 and 2019 at a tertiary pediatric hospital., Results: One-hundred and seven patients with TEF underwent 142 bronchoscopies during the study period. Of these, 22 patients underwent 28 bronchoscopies with oxygen insufflation. Twelve (43%) open fistulas were identified; of these, 9 (75%) were found using oxygen insufflation, revealing the fistula in 4/9 (44%) cases that had not been apparent using simple bronchoscopic visualization alone. One fistula was missed with multiple investigations, including bronchography and found only using oxygen insufflation. No complications were encountered., Conclusions: Recurrent or isolated TEF may be missed using ordinary flexible bronchoscopy and imaging studies. Low flow oxygen insufflation can be applied safely and may detect otherwise occult TEF., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Competing Interests: Disclosure The authors have nothing to disclose.

Background: Congenital H-type tracheo-oesophageal fistula (H-TOF ) accounts for 4%-5% of all congenital tracheo-oesophageal malformations. We present our experience in managing 18 cases with congenital H-TOF at a tertiary institute over a 10-year period., Methods: Records of all patients with congenital H-TOF managed from January 2009 to December 2018 in the Department of Paediatric Surgery at a tertiary institute were retrospectively analysed based on the age at presentation, gender, antenatal ultrasonography findings; birth history; details of previous hospitalisations, previous treatment details, presenting symptoms and associated anomalies; time to diagnosis; radiological investigations performed, bronchoscopy findings, intraoperative details, complications and postoperative follow-up., Results: Totally 18 patients with congenital H-TOF were managed over a 10-year period. There were 12 females and six males. Six patients had associated anomalies. There was wide variation in age at the start of symptoms (3 days-4 years) and presentation/referral to us (15 days-12 years). Four patients were diagnosed to have H-TOF at first admission. The most common presenting symptom was recurrent pneumonias (n=18). Bronchoscopy was done in all patients, and fistula was diagnosed and cannulated before surgery. The fistula was present at C8-T1 in 14 patients. The median age at surgery was 12 months. In 17 patients, the fistula was repaired by the cervical approach. There were two deaths, and 16 patients are doing well on median follow-up of 8 years., Conclusion: Congenital H-TOF should be considered in differential diagnosis while managing patients with recurrent lower respiratory tract infection and 'coughing and choking episodes'; early diagnosis and management of the associated H-TOF is important for improved survival and outcome., (© 2021 Charu Tiwari et al. published by Sciendo.)

Objective: To identify the risk factors for early mortality and morbidity in a population with distal esophageal atresia (EA)-tracheoesophageal fistula., Study Design: Cohort study from a national register. Main outcomes and measures included early mortality, hospital length of stay (LoS), need for nutritional support at 1 year of age as a proxy measure of morbidity, and complications during the first year of life., Results: In total, 1008 patients with a lower esophageal fistula were included from January 1, 2008, to December 31, 2014. The survival rate at 3 months was 94.9%. The cumulative hospital LoS was 31.0 (17.0-64.0) days. Multivariate analysis showed that intrahospital mortality at 3 months was associated with low birth weight (OR 0.52, 95% CI [0.38-0.72], P < .001), associated cardiac abnormalities (OR 6.09 [1.96-18.89], P = .002), and prenatal diagnosis (OR 2.96 [1.08-8.08], P = .034). LoS was associated with low birth weight (-0.225 ± 0.035, P < .001), associated malformations (0.082 ± 0.118, P < .001), surgical difficulties (0.270 ± 0.107, P < .001), and complications (0.535 ± 0.099, P < .001) during the first year of life. Predictive factors for dependency on nutrition support at 1 year of age were complications before 1 year (OR 3.28 [1.23-8.76], P < .02) and initial hospital LoS (OR 1.96 [1.15-3.33], P < .01)., Conclusions: EA has a low rate of early mortality, but morbidity is high during the first year of life. Identifying factors associated with morbidity may help to improve neonatal care of this population., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Tracheoesophageal fistulas (TEF) are an anomalous communication between airway and esophagus. There are several types of TEF. Congenital are mainly associated to an esophageal atresia. The type III or C, in which the upper segment of the esophagus ends in a blind pouch and there is distal tracheoesophageal fistula above the carina, accounts for 85% of esophageal atresias. The other are extremely infrequent. H-type or N-type TEF, classified as type 5 or E, is an uncommon variant and accounts for less than 4%. Recurrent TEF is a serious complication after first surgery of esophageal atresia and TEF. The rate of recurrence of TEF is estimated between 3-15%. The treatment is a challenge with a high rate of recurrence after surgery. Classical symptoms of RTEF include coughing especially after drinking, abdominal distension, repeated cyanosis, and respiratory infections. In the case of H-type fistula the symptoms are similar but appear during the first month of life. In this chapter we presented the management and alternative treatments of the congenital and acquired TEF., (Copyright © 2021. Published by Elsevier Inc.)

Esophagobronchial and esophagotracheal fistulas are rare but complex diseases with a heterogeneous spectrum of underlying etiologies. Common causes are locally advanced tumors of the esophagus and larynx, traumatic perforation from the esophageal or tracheal side as well as postoperative fistulas. The management of esophagotracheal and esophagobronchial fistulas always involves different health care providers and in most cases patients require a multidisciplinary treatment on the intensive care unit. The therapeutic concept primarily depends on the underlying cause, localization and size of the fistula but decision making is also influenced by the severity of the course of sepsis and the extent of the respiratory dysfunction. Endoscopic management with esophageal and/or tracheobronchial stenting is the most common treatment. Surgical reconstructive procedures are predominantly reserved for patients with a treatment refractory fistula or a septic multiple organ failure. The prognosis is particularly influenced by the underlying disease.

Background: H type tracheoesophageal fistula (H-TEF) is a rare congenital anomaly. Management may be complicated by late diagnosis and variation(s) in the therapeutic strategy. A systematic review of published studies explores the utility of diagnostic studies, operations and postoperative complications., Methods: Medline and PubMed database(s) were searched for ALL studies reporting H-TEF during 1997-2020. Using PRISMA methodology, manuscripts were screened for eligibility and reporting., Results: Forty-seven eligible studies were analysed. Primary diagnosis varied widely with surgeons performing oesophagography and trachea-bronchoscopy. Preoperative localisation techniques included fluoroscopy, guidewire placement and catheterisation. A cervical approach (209 of 272 cases), as well as thoracotomy, thoracoscopy and endoscopic fistula ligation, were all described. Morbidity included fistula recurrence (1.7%), leak (2%), tracheomalacia (3.4%) and respiratory sequelae (1%). The major adverse complication in all studies was vocal cord palsy secondary to laryngeal nerve injury (18.5%) yet strikingly few centres routinely reported undertaking vocal cord screening pre or postoperatively., Conclusion: This study shows that paediatric surgeons record low volume activity with H type tracheoesophageal fistula. Variation(s) in clinical practice are widely evident. Laryngeal nerve injury and its subsequent management warrant special consideration. Care pathways may offset attendant morbidity and define 'best practice.'

Background: A recurrent tracheo-esophageal fistula can complicate esophageal atresia and tracheo-esophageal fistula (TEF) repair in children. Therapeutic approaches and the rate of recurrence vary widely. Most reports are limited by small cohorts and short-term follow-up, and rates of re-recurrence are substantial, making it difficult to select the treatment of choice. We aimed to review our experience with the treatment of recurrent TEF using posterior tracheopexy, focusing on operative risks and long-term outcomes., Study Design: We conducted a retrospective review of patients with esophageal atresia TEF with recurrent TEF treated at 2 institutions from 2011 to 2020. We approach recurrent TEFs surgically. Once the TEF is divided and repaired, the membranous trachea is sutured to the anterior longitudinal ligament of the spine (posterior tracheopexy) and the esophagus is rotated into the right chest (rotational esophagoplasty), separating the suture lines widely. To detect re-recurrence, patients undergo endoscopic surveillance during follow-up., Results: Sixty-two patients with a recurrent TEF were surgically treated (posterior tracheopexy/rotational esophagoplasty) at a median age of 14 months. All had significant respiratory symptoms. On referral, 24 had earlier failed endoscopic and/or surgical attempts at repair. Twenty-nine required a concomitant esophageal anastomotic stricturoplasty or stricture resection. Postoperative morbidity included 3 esophageal leaks, and 1 transient vocal cord dysfunction. We have identified no recurrences, with a median follow-up of 2.5 years, and all symptoms have resolved., Conclusions: The surgical treatment of recurrent TEFs that incorporates a posterior tracheopexy and rotational esophagoplasty is highly effective for preventing re-recurrence with low perioperative morbidity., (Copyright © 2021 American College of Surgeons. Published by Elsevier Inc. All rights reserved.)

Rationale: Videofluoroscopic swallowing study (VFSS) is a noninvasive radiographic procedure that examines the oral, pharyngeal, and cervical esophageal stages of swallowing. Tracheoesophageal fistula (TEF) is difficult to diagnose depending on its size and location. However, how VFSS can be of benefit in the diagnosis of TEF has not been reported yet., Patient Concerns: A 64-year-old man who had been tracheostomized post spinal tumor resection surgery at the cervical level 1 to 2, had his tracheostomy tube removed approximately 25 years ago. After decannulation, he reported coughing while swallowing food, foreign sensation in the neck and repeated bouts of pneumonia ever since., Diagnosis: VFSS revealed, for the first time, acquired TEF after tracheostomy decannulation as the cause of repetitive aspiration pneumonia., Intervention: VFSS was performed in this case., Outcomes: In the background of suspected TEF based on VFSS results, the patient underwent a computed tomography scan of the chest and airway in the prone position, followed by bronchoscopy, which confirmed the existence of a TEF. He then underwent primary closure of the fistula. The patient had an uneventful recovery and is currently symptom-free 10 months after the surgery., Lessons: This case alerts clinicians to the possibility of TEF as a diagnosis when the aspirate leaks from the upper esophagus and through the posterior wall of trachea in the esophageal phase of VFSS., Competing Interests: Financial disclosure statements have been obtained, and no conflicts of interest have been reported by the authors or by any individuals in control of the content of this article. The authors have no funding and conflicts of interests to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Feingold syndrome 1 (FGLDS1) is an autosomal dominant malformation syndrome, characterized by skeletal anomalies, microcephaly, facial dysmorphism, gastrointestinal atresias and learning disabilities. Mutations in the MYCN gene are known to be the cause of this syndrome. Congenital absence of the flexor pollicis longus (CAFPL) tendon is a rare hand anomaly. Most cases are sporadic and no genetic variants have been described associated with this abnormality. We describe here a pedigree combining familial CAFPL tendon as a feature of FGLDS1. Molecular analyses of whole exome sequence data in five affected family members spanning three generations of this family revealed a novel mutation in the MYCN gene (c.1171C>T; p.Arg391Cys). Variants in MYCN have not been published in association with isolated or syndromic CAFPL tendon, nor has this been described as a skeletal feature of Feingold syndrome. This report expands on the clinical and molecular spectrum of MYCN-related disorders and highlights the importance of MYCN protein in normal human thumb and foramen development., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)