Congenital Tracheoesophageal Fistula Research Articles

Management of a double H-type tracheoesophageal fistula without esophageal atresia: a case report

BackgroundDouble congenital tracheoesophageal fistula (TEF) without esophageal atresia, known as double H-type fistula, is an extremely uncommon malformation. Due to its nonspecific symptoms and the small size of the fistula in infancy, H-type tracheoesophageal fistula is rarely diagnosed in the neonatal period and is also challenging to repair.Case presentationWe report a rare case of double H-type tracheoesophageal fistula in a 2-day-old newborn, identified with a time-lapse and repaired separately. The diagnosis was established through repeated bronchoscopy, with methylene blue injected through an esophagoscope. A right thoracotomy was performed to undertake the surgical repair after cannulating the fistula with a vascular guide wire. Five months later, the infant, who had a history of recurrent episodes of coughing and shortness of breath, was diagnosed with acute respiratory distress, and a second TEF was detected by bronchoscopy. A cervical incision was used to repair the second H-type fistula.ConclusionsDouble H-type tracheoesophageal fistula should be considered while correcting a tracheoesophageal fistula, especially if the symptoms continue after the first operation. Repeated radiological contrast studies and bronchoscopy with methylene blue injected through an esophagoscope help the early diagnosis.

Endoscopic Chemocauterization with Trichloroacetic Acid for Congenital or Recurrent Tracheoesophageal Fistula in Children with Esophageal Atresia: Experience from a Tertiary Center

BackgroundRecurrent tracheoesophageal fistula (rTEF) is a well-known complication after surgery of EA, occurring in roughly 3–10% of the patients. Recent studies have highlighted safety and efficacy of endoscopic management of recurrent TEF. The aim of this study was to evaluate the efficacy of chemocauterization with trichloroacetic acid (TCA) in rTEF and congenital tracheoesophageal fistula (cTEF). MethodsRetrospective chart review of consecutive patients with recurrent or congenital TEF who underwent endoscopic chemo-cauterization between 2018 and 2022 at a tertiary center. Children diagnosed with TEF who underwent primary or secondary endoscopic treatment were included. Median follow up time was 19 months for rTEF and 33 months for cTEF. ResultsDuring the study period, 18 patients were treated endoscopically by chemocatuerization with TCA at our institution. Treatment of recurrent TEF was successful in 13 of 14 patients (93%) Treatment of congenital TEF was successful in 2 of 4 patients (50%). In 14 patients, closure was seen after 1–2 treatments. There were no serious adverse reactions or complications to the endoscopic treatment of TEF. ConclusionEndoscopic chemocauterization is a minimal invasive technique with low morbidity and high success rate and may be considered as primary treatment for recurrent TEF. Level of evidenceIII.

Congenital tracheoesophageal fistula: An anesthetic challenge

Managing a patient scheduled for congenital tracheoesophageal fistula (TEF) repair is challenging for the anesthetist. If an appropriate ventilation strategy is not employed, serious complications such as hypoxemia, gastric distension, and pulmonary aspiration can occur. We present the case of a 2-day-old male child, suffering from an isolated TEF without esophageal atresia (type H) scheduled for an open surgical repair performed by transthoracic approach (right thoracotomy). We successfully managed this intervention and herein report this case to demonstrate the multiple pre-operative, intraoperative, and post-operative complications regarding intubation that can occur while managing such a case.

Endoscopic interventional therapies for tracheoesophageal fistulas in children: A systematic review.

An electronic literature search was performed using the keywords "tracheoesophageal fistula," "endoscopic," and "children" in the four major medical databases (Ovid, Embase, PubMed, and Web of Science) right from inception to September 2022. All English language articles describing the endoscopic interventional therapies of TEF in children were reviewed. Two independent researchers screened eligible articles at the title and abstract level. Full texts of potentially relevant articles were then screened again, and reference lists were screened manually to identify additional studies. Relevant data were extracted and analyzed. A synthesis of the relevant data was presented in descriptive form because of the heterogeneity of the included articles. The Chi-Squared test was used with a significance level of 5% (P < 0.05). Among the 1,167 retrieved papers, a total of 46 studies describing 170 TEF patients with an age range of 0.3-175 months were included, including 11 cases of acquired tracheoesophageal fistula, 144 cases of recurrent tracheoesophageal fistula, and 15 cases of congenital tracheoesophageal fistula (H-type TEF). A total of 119 out of 170 fistulas were successfully blocked via endoscopic techniques with an overall success rate of 70.0%, while 48 fistulas failed to close by endoscopic interventions, following which the procedure was converted to open surgery. No obviously severe intraoperative/postoperative complications occurred during the follow-up period, but only a mild esophageal stricture was noticed in six patients and grade II tracheal stenosis in one patient. Two patients died from causes unrelated to endoscopic procedures, with a mortality rate of approximately 1.2%. A comparative assessment of different endoscopic interventional techniques for TEF that detected endotracheal stenting was performed in six patients and one fistula was successfully blocked (16.7%). De-epithelialization alone was performed in 65 patients and the fistula healed in 47 of them (72.3%), with the mean number of successful treatments required being 2.3 times. Chemical sealant injection was administered in 33 patients and success was achieved in 21 (63.6%). The average requirement for endoscopic procedures was 1.5 times. De-epithelialization, in combination with chemical sealant injection, was performed in 62 patients, achieving the highest success rate of 77.4% (48 patients). Other treatment methods were performed in four patients and successfully treatment outcomes were reported in two of them (50.0%). The mean number of successful treatments required was four times, and a treatment was converted to surgery in one patient (25.0%). An assessment of different TEF types showed that 9 out of 15 congenital TEFs, 7 out of 11 acquired TEFs, and 103 out of 144 recurrent TEFs were successfully occluded. A comparison of the success rate across multiple groups showed a significant difference with a score of P < 0.05, while there was no significant difference in the success rate of different TEF-type groups (P > 0.05). Endoscopic intervention is currently a preferred treatment modality for children with TEF because of its less-invasive nature, less complications, and high success rate. Among all interventional techniques, de-epithelialization, in combination with chemical sealant, has a higher success rate than other techniques. However, due to the limited number of cases reported for implementing many kinds of techniques, an ideal endoscopic interventional technique has yet to be devised, often necessitating more treatment applications and close follow-up.

Complete tracheal duplication with a communicating oesophageal foregut malformation in a dog

A complete tracheal duplication with a communicating foregut malformation has not been previously reported. We now describe the clinical presentation, computed tomography imaging features and pathological findings associated with this condition in a 12-week-old dog. The dog presented with recurrent episodes of bronchopneumonia and a soft tissue swelling in the ventral cervical region. The main findings were a congenital tracheoesophageal fistula connecting the tracheal and oesophageal anomalies, with secondary moderately neutrophilic bronchopneumonia. In addition, there was a suspected concurrent congenital hypertrophic cardiomyopathy and atrioventricular endocardiosis. This report highlights a novel foregut duplication and may assist in understanding the currently debated aetiopathogenesis of this condition in both humans and animals.

A systematic review of the thoracoscopic treatment of H-type congenital tracheoesophageal fistulas

A systematic review of the thoracoscopic treatment of H-type congenital tracheoesophageal fistulas

A case of the long time presence of a foreign body in esophagus in an infant

Introduction. Infants learn the world while playing through the taste of objects. If the parents neglect it, the object in the mouth can be swallowed. Foreign bodies in the esophagus are easily diagnosed, as a rule, since immediately after getting stuck they are manifested by salivation, choking, difficulty in passing food. If a foreign body passed into the esophagus being unnoticed, in 20% various complications develop, such as esophagitis, stenosis, organ perforation, esophageal-tracheobronchial fistula, mediastinitis, aortoesophageal fistula, cervical spondylitis.Material and methods. The article presents a clinical case of the long time presence of a large foreign body in the esophagus of an infant which was simulating respiratory disorders. The child was admitted to a surgical hospital with suspected congenital tracheoesophageal fistula because when swallowing liquid food he did not manifested any complaints typical for foreign bodies in the esophagus: pain in the neck and sternum with irradiation to the interscapular region, difficulty or inability to swallow, urge to vomit, hypersalivation. Mom and doctors were inclined to find other reasons for cough and wheezing which were intensified when the child was eating. The authors discuss features of extraesophageal manifestations of a foreign body in the esophagus as well as diagnostic and therapeutic tactics in such cases.Conclusion. Even small children can swallow large foreign bodies. While taking anamnesis, especially in children of the first year of life, a doctor should think not only about the syndrome of bronchial obstruction, but also about possible presence of a foreign body in the esophagus if wheezing and coughing attacks appear during meal. Diagnostic algorithm should include X-ray and endoscopic examinations which are effective and minimally invasive and which allow to remove a foreign body, even if it is in the esophagus for a long time.

The Phenotypical Profile and Outcomes of Neonates with Congenital Tracheoesophageal Fistula Associated with Congenital Cardiac Anomalies Presenting for Surgery.

(1) Background: Neonates born with oesophageal atresia and/or tracheooesophageal fistula (OA/TOF) are usually born with a multitude of other congenital anomalies, which may affect their anaesthetic care and survival to hospital discharge. We reviewed the profile and outcome of neonates with OA/TOF and associated congenital cardiac anomalies presenting for surgery at an academic hospital in South Africa. (2) Methods: A retrospective, cross-sectional analysis of all medical records of neonates who had undergone surgical repair of OA/TOF was conducted at an academic hospital between January 2015 and December 2019. Descriptive statistics were used to report the data. Comparisons in perioperative events and outcomes between those with cardiac lesions and those without were done. (3) Results: Neonates presenting for OA/TOF repair with congenital cardiac defects had an incidence of 62 [95% CI 38.5–99.8] per 1000 days since birth. In total, 45.9% had associated cardiac anomalies, with PDA and ASD as the most prevalent lesions. There were statistically significant differences in intraoperative adverse events seen in neonates with congenital cardiac lesions as compared to those without. (4) Conclusion: Despite advances in neonatal critical care and anaesthetic and surgical techniques, OA/TOF associated with congenital cardiac anomalies is still associated with high mortality rates in developing countries.

Congenital Tracheoesophageal Fistula Presenting in an Asymptomatic Adult- A Case Report

Introduction: Congenital tracheoseophageal fistula (TEF) involve an abnormal pathological connection between the oesophagus and trachea. Congenital TEF presenting in adults is extremely rare and literature is limited to case series of significantly symptomatic patients. Case presentation: An asymptomatic 41 year-old gentleman presented to clinic, having been diagnosed with a congenital tracheoesophageal fistula 10 years ago on a routine oesophagogastroduodenoscopy (OGD) in Brazil. He underwent a barium contrast swallow revealing contrast in the left main bronchi and significant pooling in the upper trachea. A computerised tomography (CT) scan of the neck and thorax with contrast revealed a small fistula measuring 6mm in diameter between the trachea and the oesophagus at the level of the second thoracic vertebral body. A microlaryngobronchoscopy and examination under anaesthesia visualised a fistula measuring 1.8cm to 2cm superior to inferiorly and similar in transverse dimension, 6-7cm below the level of the glottis. Management and outcome: He opted to have surgical treatment to prevent symptoms developing in the future. A trans-cervical approach was performed, and the oesophageal defect and tracheal defect underwent primary closure with sutures. The oesophageal defect repair was oversewn with the sternohyoid. He recovered well post-operatively but due to the considerable rotation and retraction of the trachea, he suffered a vocal cord palsy. This is improving at the latest follow-up. Discussion: Otolaryngologist (ENT) surgeons need to be aware of asymptomatic adult patients presenting with congenital tracheoesophageal fistulas and the investigation and management options available.

Anastomotic Stricture in End-to-End Anastomosis-Risk Factors in a Series of 261 Patients with Esophageal Atresia.

To assess the risk factors for anastomotic stricture (AS) in end-to-end anastomosis (EEA) in patients with esophageal atresia (EA). With ethical consent, hospital records of 341 EA patients from 1980 to 2020 were reviewed. Patients with less than 3 months survival (n = 30) with Gross type E EA (n = 24) and with primary reconstruction (n = 21) were excluded. Outcome measures were revisional surgery for anastomotic stricture (RSAS) and number of dilatations required for anastomotic patency without RSAS. The factors that were tested for risk of RSAS or dilatations were distal tracheoesophageal fistula (TEF) at the carina in C-type EA (congenital TEF [CTEF]), type A/B EA, antireflux surgery (ARS), anastomotic leakage, recurrent TEF, and Spitz group and congenital heart disease. A total of 266 patients, Gross type A (n = 17), B (n = 3), C (n = 237), or D (n = 9) underwent EEA (early n = 240, delayed n = 26). Early anastomotic breakdown required secondary reconstruction in five patients. Of the remaining 261 patients, 17 (6.1%) had RSAS, whereas 244 patients with intact end to end required a median of five (interquartile range: 2-8) dilatations for anastomotic patency. Main risk factors for RSAS or (> 8) dilatations were CTEF, type A/B, ARS, and anastomotic leakage that increased the risk of RSAS or dilatations from 4.6- to 11-fold. The risk of severe AS is associated with long-gap EA, significant gastroesophageal reflux, and anastomotic leakage.

Detail correction for Gross classification of esophageal atresia based on 434 cases in China.

Detail correction for Gross classification of esophageal atresia based on 434 cases in China.

Flexible endoscopic identification and catheterization of congenital H-type tracheoesophageal fistula using a laryngeal mask.

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.

Repeated aspiration pneumonia in a 7-year-old girl with undiagnosed congenital tracheoesophageal fistula

Repeated aspiration pneumonia in a 7-year-old girl with undiagnosed congenital tracheoesophageal fistula

Airway and ventilatory management in a premature neonate with congenital tracheoesophageal fistula

Esophageal atresia/tracheoesophageal fistula (TEF) is a congenital defect that often presents with respiratory distress in neonates. The anatomical defect in the form of connection between the esophagus and the trachea often affects respiratory function. Gastric distention often occurs due to large air leak into the stomach and may lead to respiratory distress. Surgical correction of the anatomical defect will improve the outcome in these patients. Preoperative preparation and intraoperative management is an essential point. Suboptimal preparation may lead to a life-threatening situation intraoperative. Focusing on airway and ventilatory management may help in reducing morbidity and mortality. We report a case of anesthesia management of TEF repair in a premature neonate with a complication during airway and ventilatory management.

H-type congenital tracheoesophageal fistula: Insights from 70 years of The Royal Children's Hospital experience

BackgroundThe long-term outcomes of H-type tracheoesophageal fistula (TOF), an uncommon variant of esophageal atresia/tracheoesophageal fistula (OA/TOF), are rarely described in the literature. We reviewed our institutional experience of 70 years. MethodsThe Nate Myers Oesophageal Atresia Database was queried for patients with an H-type TOF (1948–2017). Data included presentation, diagnostic workup, surgical management, and outcomes. ResultsOf 1088 patients with OA/TOF, 56 (5.1%) had an H-type TOF. The most common presenting symptoms were cyanotic episodes (68%), choking with feeds (52%), and aspiration pneumonitis (46%). The majority (82%) were symptomatic in the first week of life. Coexisting congenital anomalies were present in 46%: cardiac (13/56, 23%), genitourinary (10/56, 18%), and vertebral/skeletal (9/56, 16%). Patients were consistently diagnosed with prone contrast tube esophagogram (77% sensitivity on the first study and 96% after a second study). The fistula was most commonly approached through a right cervical collar incision. Right vocal cord palsy occurred in 22%, with one case of bilateral palsies. Other complications included leak (5.6%), recurrence (9.3%), stricture (1.9%), and diverticulum (1.9%). Although there was a trend towards a lower recurrence rate when interposition material was used, this was not statistically significant (3.3% vs 16.7%, p = 0.16). Survival in operative cases was 98.2%, and when all diagnosed cases were considered was 89.3%. ConclusionsWe have reported the largest single-center series of H-type TOF. Diagnosis is challenging, and surgical morbidity remains high. Despite this, long-term outcomes are favorable. Level of evidenceIV

Comparison of Detection Methods for Tracheoesophageal Fistulae With a Novel Method: Capnography With CO2 Insufflation.

Tracheoesophageal fistulae (TEF) are difficult to detect and require a high index of suspicion. We hypothesized that capnography to identify a spike in end-tidal carbon dioxide (etCO2) during esophagoscopy with carbon dioxide (CO2) insufflation would facilitate TEF diagnosis because of gas passage from the esophagus to the trachea. Medical records of 42 consecutive cases of recurrent, acquired, or missed congenital TEF diagnosed between January 2015 and November 2019 that underwent esophagoscopy with CO2 insufflation were reviewed. A control cohort of 97 similarly endoscopically evaluated patients with surgical confirmation of absence of recurrent TEF (eg, patients undergoing posterior tracheopexy) was also collected. All patients underwent pre-operative esophagoscopy, bronchoscopy, and capnography; diagnostic abilities of various combinations of modalities for TEF identification were calculated. Statistical analysis identified a maximum intra-esophagoscopy end-tidal CO2 level of 68 mmHg as the optimal discriminator between cases and controls, though in practice, we anecdotally find that recurrent TEFs typically permit rapid rise ≥90 mmHg. Increasing numbers of diagnostic modalities increased diagnostic sensitivity to detect recurrent TEF; the highest diagnostic sensitivity for TEF identification was achieved by the combination of intra-esophagoscopy fluoroscopy with bronchoscopy and capnography ≥68 mmHg (sensitivity = 88.1%). There were multiple cases of TEF (N = 7 for etCO2 ≥68 mmHg, N = 3 for etCO2 ≥90 mmHg) identified by capnography that were missed by esophagoscopy. There were 5 (for etCO2 ≥68 mmHg) or 6 (for etCO2 ≥90 mmHg) cases of recurrent TEF that were missed by all nonsurgical methods. Attention to etCO2 during esophagoscopy with CO2 insufflation represents a simple, novel way to detect TEF. Identification of TEF remains challenging, though combinations of diagnostic modalities improve diagnostic sensitivity.

Fiberoptic bronchoesophagoscopy-assisted evaluation and prognostic factor analysis in children with congenital esophageal atresia and tracheoesophageal fistula.

Esophageal atresia (EA) and tracheoesophageal fistula (TEF) are serious congenital anomalies with high morbidity and mortality. Diagnostic and therapeutic fiberoptic endoscopy has been used in children to evaluate and manage trachea-esophageal anomalies. This study aimed to evaluate the prognostic factors and the role of fiberoptic bronchoesophagoscopy (FB) in managing children with EA and TEF. From 2000 to 2017, hospitalized children with suspected EA and TEF were enrolled in the study. All associated medical records were retrospectively reviewed. Basic characteristics, diagnoses, age of surgical reconstruction, FB findings, associated anomalies, and survival durations were reviewed. Prognostic factors associated with the patients' mortality were analyzed. A total of 33 children were enrolled, and 91% of them were type C. The median age at the time of hospitalization was 26 days (range, birth to 9 years), including 20 (61%) low-birth-weight infants and 26 (79 %) referred patients. FB was performed in patients preoperatively (39%) and postoperatively (96.8%). Among them, 28 patients (85%) had associated anomalies, including 17 (52%) cardiac and 23 (70%) airway anomalies. The median age of 31 patients who underwent surgical reconstruction was 3 (range, 0-39) days. Esophageal anastomotic stricture (21/31, 67.7%) was the most common postsurgical complication. Twenty-three patients (74.2%) received postoperative FB-guided interventions, including balloon dilatation, laser therapy, and stent implantation. Among the 9 mortality cases, the median age at death was 270 (range, 4-3246) days. Significant factor associated with mortality was delayed (> 48 h old) or no surgical reconstruction (p = 0.030). Delayed (>48-hour old) or no surgical reconstruction was significantly related to mortality in children with congenital EA and TEF. Preoperative and postoperative FB evaluations helped to facilitate diagnoses and nonsurgical managements and resolve the patients' tracheoesophageal problems.

Esophageal Squamous Cell Carcinoma in a 31-Year-Old Woman with Chronic Congenital Tracheoesophageal Fistula

Esophageal Squamous Cell Carcinoma in a 31-Year-Old Woman with Chronic Congenital Tracheoesophageal Fistula

Acquired Tracheoesophageal Fistula After Esophageal Atresia Repair

Background:Recurrence of tracheoesophageal fistula is a frequent complication after esophageal atresia repair. Acquired tracheoesophageal fistulas are long new fistulas that are localized at sites that are not typical of the congenital tracheoesophageal fistula. We present four cases to discuss the diagnostic and management challenges concerning various acquired tracheoesophageal fistula localizations.Case Report:We retrospectively evaluated the medical records of patients admitted with acquired tracheoesophageal fistula in the last 5 years. Among the 16 postoperative tracheoesophageal fistulas, 4 were classified as acquired tracheoesophageal fistula. Patients’ admission age ranged from 1 to 8 years. The female to male ratio was 2:2. The presented cases were admitted with recurrent respiratory tract infections, choking, and coughing. The acquired tracheoesophageal fistulas were observed between the esophagus and cervical trachea, between the esophagus and the right bronchus passing through intrathoracic abscess cavity, in the right bronchus, and between the colon conduit and trachea. One of the acquired tracheoesophageal fistulas healed spontaneously, whereas others required surgical ligation.Conclusion:Acquired tracheoesophageal fistula most often occurs secondary to local or diffuse mediastinitis. Acquired tracheoesophageal fistula may appear at unusual sites not typical of congenital tracheoesophageal fistula, such as esophagus-to-right bronchus and conduit to trachea. Therefore, the unusual locations of acquired tracheoesophageal fistula should be borne in mind, and patients evaluated and managed more comprehensively.

Hurdles in managing tracheoesophageal fistula: case report

Congenital tracheoesophageal fistula (TOF) is a rare anomaly. With the development of endoscopic techniques, the diagnosis of TOF has made marked progress. It requires surgical repair in infancy by either open or thoracoscopic approach. Diagnosing and locating the location of fistula may be difficult particularly in smaller fistula. We report three cases of TOF, to highlight the role of bronchoscopy and endoscopy and also the challenges in managing the aforementioned cases, which may present with airway embarrassment.