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.

Esophageal atresia with tracheoesophageal fistula: two case reports.

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.

[Treatment of tracheoesophageal fistula and consequences of severe spinal injury]. / Lechenie trakheopishchevodnogo svishcha i posledstvii tyazheloi travmy pozvonochnika.

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 ThI-ThVII, and decompression laminectomy ThIV-ThVI. 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.

Perioperative anaesthetic management and short-term outcome of neonatal repair of oesophageal atresia with or without tracheo-oesophageal fistula in Europe: A sub-analysis of the neonate and children audit of anaesthesia practice in Europe (NECTARINE) prospective multicenter observational study.

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.

Diagnosis and management of congenital type D esophageal atresia.

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.

Recent Advances in the Genetic Pathogenesis, Diagnosis, and Management of Esophageal Atresia and Tracheoesophageal Fistula: A Review.

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.

Type III congenital pulmonary airway malformation associated with oesophageal atresia and tracheoesophageal fistula. A case report and review of literature.

Congenital pulmonary airway malformation (CPAM) together with oesophageal atresia and tracheoesophageal fistula (TOF) is a very rare condition in neonates. We presented a case of an infant with Gross type C oesophageal atresia with TOF coexisting with Stocker Type III CPAM in our centre. It is interesting to know that TOF associated with type III CPAM has never been reported in the literature. The child was delivered through caesarean section, and because of respiratory distress post-delivery, endotracheal intubation was carried out immediately. CPAM was diagnosed by a suspicious finding from the initial chest X-ray and the diagnosis was confirmed through computed tomography scan of the chest. The patient was initially stabilised in a neonatal intensive care unit (NICU), and after the successful ligation of fistula and surgical repair of TOF, lung recruitment was started by high flow oscillatory ventilation. The patient recovered well without complications and able to maintain good saturation without oxygen support through the stay in the neonatal unit. Early recognition of this rare association is essential for immediate transfer to NICU, the intervention of any early life-threatening complications, and for vigilant monitoring in the postoperative period.

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.

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.

[Combination of esophageal atresia with proximal tracheoesophageal fistula and meconium peritonitis: a case report]. / Sochetanie atrezii pishchevoda s proksimal'nym trakheopishchevodnym svishchom i mekonievogo peritonita.

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.

[Treatment of cicatricial tracheal stenosis and tracheoesophageal fistula in patients with COVID-19 pneumonia]. / Lechenie rubtsovogo stenoza trakhei i trakheopishchevodnogo svishcha u bol'nykh, perenesshikh COVID-19-pnevmoniyu.

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 zone¼, 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.

To assess the cell lines on esophagogastroduod enoscopies of children with feeding difficulty, esophageal fistula and asthma with histologic abnormality.

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.

Esophageal Atresia and Tracheoesophageal Fistula: Overview and Considerations for the General Surgeon.

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.

Perioperative management and outcomes of neonates undergoing anaesthesia for congenital tracheo-oesophageal fistula repair at Charlotte Maxeke Johannesburg academic hospital.

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.

Exome sequencing efficacy and phenotypic expansions involving esophageal atresia/tracheoesophageal fistula plus.

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.

Thoracoscopic Repair of Adult-Onset Congenital Tracheoesophageal Fistula Using a Polyglycolic Acid Sheet-Buttressed Stapler.

Congenital tracheoesophageal fistula (TEF) without esophageal atresia is usually diagnosed and treated in the neonatal period. It is uncommon to occur in adulthood. Conventional treatment of adult-onset TEF involves repair by either cervicotomy or thoracotomy. We reported the case of a 31-year-old male patient with clinical and radiographic evidence of congenital H-type TEF. Although this fistula was located at the level of the second thoracic vertebra, the repair of the anomaly was performed successfully using a thoracoscopic approach with the novel use of a polyglycolic acid sheet reinforcement.

A "Catlike Cry" as a Symptom of Congenital Tracheoesophageal Fistula.

Congenital tracheoesophageal fistula is usually diagnosed at an early age, but may remain undetected into adulthood if atresia is absent and if the fistula is small. A tracheoesophageal fistula should be suspected in patients with unexplained episodes of respiratory distress or pneumonia; however, more subtle signs can be an important symptom for early recognition of the disease. Ono's sign is a well-known symptom of tracheoesophageal fistula, characterized by paroxysmal coughing triggered by swallowing of fluids. In the present case, air movement between the esophagus and the trachea through the fistula caused a high-pitched sound, which the patient described as a "catlike cry." The high-pitched sound ceased after surgical closure of the fistula. We report here the symptom of "catlike cry" as one manifestation of tracheoesophageal fistula.

An overview of esophageal atresia and tracheoesophageal fistula.

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.