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BACKGROUND: The objectives of this study were to bring out the results of application of pleural wrap in primary repair of tracheo - esophageal fistula (TEF) with long-gap oesophageal atresia (LGEA) and also define the role of neonatologists and paediatric intensivists in post-operative management in these patients by laying down standard neonatal management protocol. MATERIALS AND METHODS: This was a retrospective descriptive observational study conducted between March 2011 and April 2019 on 23 cases of LGEA with TEF. The study was conducted at two tertiary care paediatric surgery centres in Northern part of India wherein these newborn babies were operated by two paediatric surgeons with variable experience of 8-12 years. It also describes the neonatal management protocol used in post-operative period. RESULTS: Out of 23 patients, 15 were boys and 8 were girls, with a mean age of 32.25 weeks and a mean birth weight of 2.02 kg. The mean hospital stay was 23.5 ± 8 days. Eleven cases had gap between 3 and 3.5 cm, 8 cases between 3.5 and 4 cm and 4 cases had gap more than 4 cm. The incidence of associated anomalies was 52%. Anastomotic leak rate was 8.69%, and 3 (13.04%) patients died in the post-operative period. All the operated patients were managed postoperatively as per strict neonatal management protocol exclusively by the team of neonatologists and neonatal intensivists. CONCLUSION: Application of pleural wrap over anastomosis following primary repair of LGEA with TEF significantly reduced the incidence of anastomotic leak in our study. Apart from the pleural wrap, the key to successful outcome also is contributed by the little prolonged, controlled ventilation and patience and perseverance in post-operative feeds. This post-operative management protocol that has been followed by us in our study is easily reproducible and can be adopted by paediatric surgeons working alongside neonatologists as a team.

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INTRODUCTION: Eating, drinking and swallowing difficulties are commonly reported morbidities for individuals born with OA/TOF. This study aimed to determine the nature and prevalence of eating, drinking and oro-pharyngeal swallowing difficulties reported in this population. METHOD: A systematic review and meta-proportional analysis were conducted (PROSPERO: CRD42020207263). MEDLINE, EMBASE, CINAHL, Pubmed, Scopus, Web of Science databases and grey literature were searched. Quantitative and qualitative data were extracted relating to swallow impairment, use of mealtime adaptations and eating and drinking-related quality of life. Quantitative data were summarised using narrative and meta-proportional analysis methods. Qualitative data were synthesised using a meta-aggregation approach. Where quantitative and qualitative data described the same phenomenon, a convergent segregated approach was used to synthesise data. RESULTS: Sixty-five studies were included. Six oro-pharyngeal swallow characteristics were identified, and pooled prevalence calculated: aspiration (24%), laryngeal penetration (6%), oral stage dysfunction (11%), pharyngeal residue (13%), nasal regurgitation (7%), delayed swallow initiation (31%). Four patient-reported eating/drinking difficulties were identified, and pooled prevalence calculated: difficulty swallowing solids (45%), difficulty swallowing liquids (6%), odynophagia (30%), coughing when eating (38%). Three patient-reported mealtime adaptations were identified, and pooled prevalence calculated: need for water when eating (49%), eating slowly (37%), modifying textures (28%). Mixed methods synthesis of psychosocial impacts identified 34% of parents experienced mealtime anxiety and 25% report challenging mealtime behaviours reflected in five qualitative themes: fear and trauma associated with eating and drinking, isolation and a lack of support, being aware and grateful, support to cope and loss. CONCLUSIONS: Eating and drinking difficulties are common in adults and children with repaired OA/TOF. Oro-pharyngeal swallowing difficulties may be more prevalent than previously reported. Eating, drinking and swallowing difficulties can impact on psychological well-being and quality of life, for the individual and parents/family members. Long-term, multi-disciplinary follow-up is warranted.

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BACKGROUND: This study aimed to analyze the results, feasibility and safety of the thoracoscopic approach for patients with esophageal atresia with tracheoesophageal fistula (EA/TEF) depending on the patient's birth weight. METHODS: The study involved only type C and D EA/TEF. Among the analyzed parameters were the patients' characteristics, surgical treatment and post-operative complications: early mortality, anastomosis leakage, anastomosis strictures, chylothorax, TEF recurrence, and the need for fundoplication or gastrostomy. RESULTS: 145 consecutive newborns underwent thoracoscopic EA with TEF repair. They were divided into three groups-A (N = 12 with a birth weight < 1500 g), B (N = 23 with a birth weight ≥ 1500 g but < 2000 g), and C-control group (N = 110 with a birth weight ≥ 2000 g). Primary one-stage anastomosis was performed in 11/12 (91.7%) patients-group A, 19/23 (82.6%)-group B and 110 (100%)-group C. Early mortality was 3/12 (25%)-group A, 2/23 (8.7%)-group B, and 2/110 (1.8%)-group C and was not directly related to the surgical repair. There were no significant differences in operative time and the following complications: anastomotic leakage, recurrent TEF, esophageal strictures, and chylothorax. There were no conversions to an open surgery. Fundoplication was required in 0%-group A, 4/21 (19.0%)-group B, and 2/108 (1.9%)-group C survivors. Gastrostomy was performed in 1/9 (11.1%)-group A, 3/21 (14.3%)-group B and 0%-group C. CONCLUSION: In an experienced surgeon's hands, even in the smallest newborns, the thoracoscopic approach may be safe, feasible, and worthy of consideration. Birth weight seems to be not a direct contraindication to the thoracoscopic approach.

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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.

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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.

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INTRODUCTION: Tracheoesophageal fistula (TEF) especially malignant TEF (mTEF) is an uncommon yet critical medical condition necessitating immediate intervention. This life-threatening condition frequently manifests in critically ill patients who are dependent on prolonged mechanical ventilation and are unsuitable candidates for thoracotomy due to their compromised health status. The Management of these mTEF patients remain a significant challenge.This study aimed to evaluate the safety and efficacy of using a cardiac septal occluder for the closure of mTEF. METHODS: 8 patients with mTEF underwent closure surgery using atrial/ventricular septal defect (ASD/VSD) septal occluders at the Respiratory Department of HuBei Yichang Central People's Hospital from 2021 to 2023. The procedure involved percutaneous placement of the occluder through the fistula to achieve closure. RESULTS: The placement of the cardiac septal occluder was successfully achieved with ease and efficiency in all patients. The study demonstrated that the use of cardiac septal occluder therapy in patients with mTEF can alleviate symptoms, improve quality of life, and enhance survival rates, with no significant complications observed. Furthermore, the study provided comprehensive details on surgical indications, preoperative evaluation and diagnosis, selection of occluder, methods of occlusion, and postoperative care. CONCLUSIONS: The application of cardiac septal occluder in the treatment of mTEF is a safe and effective palliative treatment. This approach may be particularly beneficial for patients with a high risk of complications and mortality associated with traditional surgical interventions.

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Tracheoesophageal fistulas (TOF) following esophagectomy for esophageal cancer are rare but potentially fatal. There is no consensus on treatment between stenting and surgical repair, although the latter is associated with better distant survival. In surgical repair, the interposition of a flap improves healing by providing well-vascularized tissue and reinforcing the repair zone. The flaps described are usually muscular and decaying. We present the case of a malnourished fifty-year-old man who underwent intrathoracic surgical repair of symptomatic recurrent TOF using a skin flap based on the perforators of the internal thoracic artery (IMAP). The perforator flap was completely de-epidermized and tunneled under the sternum by a proximal and limited resection of the 3rd costal cartilage and placed at the posterior aspect of the trachea, with the excess tissue rolled up on either side. At 9 months, the patient showed no recurrence and improved general condition. The de-epidermized IMAP tunneled under the sternum intrathoracically is a reliable alternative to the conventional muscle flaps described in TOF management and an attractive additional tool in the plastic surgeon's surgical arsenal.

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PURPOSE: The surgical indication of thoracoscopic primary repair for esophageal atresia with tracheoesophageal fistula is under debate. The current study aimed to investigate the outcome of thoracoscopic primary repair for esophageal atresia with tracheoesophageal fistula in patients weighing < 2000 g and those who underwent emergency surgery at the age of 0 day. METHODS: The surgical outcomes were compared between patients weighing < 2000 g and those weighing > 2000 g at surgery and between patients who underwent surgery at the age of 0 day and those who underwent surgery at age ≥ 1 day. RESULTS: In total, 43 patients underwent thoracoscopic primary repair for esophageal atresia with tracheoesophageal fistula. The surgical outcomes according to body weight were similar. Patients who underwent surgery at the age of 0 day were more likely to develop anastomotic leakage than those who underwent surgery at the age of ≥ 1 day (2 vs. 0 case, p = 0.02). Anastomotic leakage was treated with conservative therapy. CONCLUSION: Thoracoscopic primary repair is safe and useful for esophageal atresia with tracheoesophageal fistula even in newborns weighing < 2000 g. However, emergency surgery at the age of 0 day should be cautiously performed due to the risk of anastomotic leakage.

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OBJECTIVE: Tracheoesophageal puncture (TEP) is one of the most established methods for voice reacquisition following total laryngectomy. The most difficult complication following TEP is the management of saliva leakage or secretion into the trachea due to TE fistula enlargement. In this study, we devised a new strategy to close TE fistulas and confirmed its safety and effectiveness. METHODS: Skin incision: If the tracheal mucosa around the voice prosthesis appears intact and normal, an arcuate incision, from 10 to 2 o'clock, is made on the skin 5 mm superior to the edge of the stoma. However, if the surrounding tracheal mucosa is fragile because of leaking, the incision is made on the superior edge of the stoma, with later reconstruction of the posterior tracheal wall. Separation of the trachea and esophagus: If the esophagotracheal spatium appears normal and is easy to dissect, the connective pipes can be found easily. After cutting the pipe, a ligature alone is sufficient for the tracheal side; however, the esophageal wall is closed with Gambee sutures. If the esophagotracheal spatium is compromised and the posterior tracheal wall is fragile (due to saliva leakage), we remove the posterior wall and reconstruct the area using the superior skin flap. We performed our novel method on four patients with intractable conditions; postradiotherapy for laryngeal cancer, total pharyngo-laryngo-esophagectomy (TPLE) with jejunum reconstruction, TPLE with gastric lifting reconstruction, and in a patient who underwent cervicothoracic incisional drainage for descending necrotizing mediastinitis. RESULTS: None of the cases showed postoperative leakage from the fistula, and oral intake was resumed without difficulty. CONCLUSION: This study showed that this strategy based on TE fistula conditions is effective even in difficult-to-treat cases.

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OBJECTIVE: Validation of a contemporary International Classification of Diseases, 10th Revision, Clinical Modification (ICD-10-CM) congenital esophageal atresia/tracheoesophageal atresia (EA/TEF) cohort in the Pediatric Health Information System (PHIS) database. STUDY DESIGN: Database study, validation. SETTING: Tertiary care center. METHODS: Search methods used to validate an ICD-9-CM EA/TEF cohort in PHIS were modified for ICD-10-CM. A retrospectively and prospectively maintained clinical database at a single high-volume EA/TEF center was used for comparison. Patients treated between October 1, 2015 and July 31, 2022 were included. Searches progressively narrowed the cohort by ICD-10-CM diagnosis codes, expansion to include incorrectly coded as 'iatrogenic, age less than 30 days, and use of at least 1 ICD-10-CM procedure code. Results of PHIS data and institution data were compared for accuracy. RESULTS: The most refined search of PHIS and the EA/TEF clinical database yielded 93 and 84 patients, respectively. The sensitivity was 99% and positive predictive value was 94%. A PHIS search using these methods and encompassing 49 children's hospitals yielded an EA/TEF cohort of 2479 patients. CONCLUSION: We present a validated search method in the PHIS database to identify a high-fidelity cohort of EA/TEF patients for multi-institutional study. We have demonstrated that a carefully maintained clinical database may be used to validate cohorts in PHIS. This cohort allows for improved practice variability and outcomes study of EA/TEF patients. Similar methods may be employed to generate other rare disease cohorts in PHIS. LEVEL OF EVIDENCE: Level 4.

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BACKGROUND: The magnetic compression technique has been used to establish an animal model of tracheoesophageal fistula (TEF), but the commonly shaped magnets present limitations of poor homogeneity of TEF and poor model control. We designed a T-shaped magnet system to overcome these problems and verified its effectiveness via animal experiments. AIM: To investigate the effectiveness of a T-shaped magnet system for establishing a TEF model in beagle dogs. METHODS: Twelve beagles were randomly assigned to groups in which magnets of the T-shaped scheme (study group, n = 6) or normal magnets (control group, n = 6) were implanted into the trachea and esophagus separately under gastroscopy. Operation time, operation success rate, and accidental injury were recorded. After operation, the presence and timing of cough and the time of magnet shedding were observed. Dogs in the control group were euthanized after X-ray and gastroscopy to confirm establishment of TEFs after coughing, and gross specimens of TEFs were obtained. Dogs in the study group were euthanized after X-ray and gastroscopy 2 wk after surgery, and gross specimens were obtained. Fistula size was measured in all animals, and then harvested fistula specimens were examined by hematoxylin and eosin (HE) and Masson trichrome staining. RESULTS: The operation success rate was 100% for both groups. Operation time did not differ between the study group (5.25 min ± 1.29 min) and the control group (4.75 min ± 1.70 min; P = 0.331). No bleeding, perforation, or unplanned magnet attraction occurred in any animal during the operation. In the early postoperative period, all dogs ate freely and were generally in good condition. Dogs in the control group had severe cough after drinking water at 6-9 d after surgery. X-ray indicated that the magnets had entered the stomach, and gastroscopy showed TEF formation. Gross specimens of TEFs from the control group showed the formation of fistulas with a diameter of 4.94 mm ± 1.29 mm (range, 3.52-6.56 mm). HE and Masson trichrome staining showed scar tissue formation and hierarchical structural disorder at the fistulas. Dogs in the study group did not exhibit obvious coughing after surgery. X-ray examination 2 wk after surgery indicated fixed magnet positioning, and gastroscopy showed no change in magnet positioning. The magnets were removed using a snare under endoscopy, and TEF was observed. Gross specimens showed well-formed fistulas with a diameter of 6.11 mm ± 0.16 mm (range, 5.92-6.36 mm), which exceeded that in the control group (P < 0.001). Scar formation was observed on the internal surface of fistulas by HE and Masson trichrome staining, and the structure was more regular than that in the control group. CONCLUSION: Use of the modified T-shaped magnet scheme is safe and feasible for establishing TEF and can achieve a more stable and uniform fistula size compared with ordinary magnets. Most importantly, this model offers better controllability, which improves the flexibility of follow-up studies.

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BACKGROUND: Tracheal agenesis, or tracheal atresia, is a rare congenital anomaly. The presence of a tracheoesophageal fistula (TEF) can help with breathing for newborns with tracheal agenesis. In this article, we presented three unique cases and outcomes of neonates with tracheal agenesis along with a review of the literature. METHODS: This study consisted of a single center case series followed by a review of literature. Case reports were generated using both written and electronic medical records from a single hospital. We summarized three unique cases and outcomes of neonates with tracheal agenesis and performed a review of the literature. RESULTS: We identified three cases of tracheal agenesis presented with severe cyanosis without spontaneous crying upon birth. Experienced pediatricians attempted to intubate the babies but were unsuccessful. Endotracheal tubes were subsequently either accidentally or purposely placed into the esophagus, and oxygen saturation levels improved. This suggested tracheal agenesis with TEF. Two cases underwent surgical intervention after resuscitation with esophageal intubation. CONCLUSION: Esophageal intubation may be a life-sustaining ventilation support for patients with tracheal agenesis and TEF at initial resuscitation. Clinicians should suspect tracheal agenesis when a newborn presents with severe cyanosis and voiceless crying upon birth, and esophageal intubation should be immediately attempted.

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We developed a 3D-printed thoracoscopic surgery simulator for esophageal atresia with tracheoesophageal fistula (EA-TEF) and assessed its effectiveness in educating young pediatric surgeons. Prototype production and modifications were repeated five times before producing the 3-D printed final product based on a patient's preoperative chest computed tomography. A 24-item survey was used to rate the simulator, adapted from a previous report, with 16 young surgeons with an average of 6.2 years of experience in pediatric surgery for validation. Reusable parts of the thoracic cage were printed to combine with replaceable parts. Each structure was fabricated using diverse printing materials, and subsequently affixed to a frame. In evaluating the simulator, the scores for each factor were 4.33, 4.33, 4.27, 4.31, 4.63, and 4.75 out of 5, respectively, with the highest ratings in value and relevance. The global rating was 3.38 out of 4, with ten stating that it could be used with slight improvements. The most common comment from participants was that the esophageal anastomosis was close to the actual EA-TEF surgery. The 3D-printed thoracoscopic EA-TEF surgery simulator was developed and reflected the actual surgical environment. It could become an effective method of training young pediatric surgeons.

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OBJECTIVES: Recurrent and primary tracheoesophageal fistulas (TEFs) are a challenging surgical pathology to treat, as standard open surgical approaches are associated with high morbidity and mortality. As such, endoscopic modalities have gained interest as an alluring alternative, yet variable success rates have been reported in the literature. The aim of this study was to provide a contemporary update of the literature and describe our institutional experience with the bronchoscopic obliteration of recurrent and primary TEFs. METHODS: Retrospective chart review of all pediatric patients having undergone endoscopic TEF repair at two pediatric academic centers in Montreal, Canada and Lille, France between January 1, 2008 to December 31, 2020. RESULTS: 28 patients with TEFs (20 recurrent, 8 primary) underwent a total of 48 endoscopic procedures. TEF repair was performed under endoscopic guidance using various combinations of techniques, including fistula de-epithelialization (endoscopic brush, thulium laser, trichloroacetic acid-soaked pledgets or electrocautery), tissue adhesives, submucosal augmentation, esophageal clip and stenting. Successful closure was achieved in 16 patients (57 %), while 12 (43 %) required eventual open or thoracoscopic repair. The mean number of endoscopic procedures was 1.7. There were no major treatment-related complications such as pneumothorax, mediastinitis or death (mean follow-up 50.8 months). CONCLUSIONS: Endoscopic repair of recurrent or primary TEFs is a valuable component of our therapeutic armamentarium and may contribute to decreased surgical morbidity in this complex patient population. Families should be counselled that endoscopic results may be more modest than with open or thoracoscopic approaches, and multiple procedures may be required.

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INTRODUCTION: The number of patients with congenital disease living to adulthood continues to grow. Often undergoing surgical correction in infancy, they continue to require lifelong care. Their numbers are largely unknown. We sought to evaluate hospital admissions of adult patients with esophageal atresia with tracheoesophageal fistula (EA/TEF), congenital diaphragmatic hernia (CDH), and Hirschsprung disease (HD). METHODS: The Florida Agency for Healthcare Administration inpatient database was merged with the Distressed Communities Index and Centers for Medicare and Medicaid Services Hospital and Physician Compare datasets. The dataset was queried for adult patients (≥18 y, born after 1970) with EA/TEF, CDH, and HD in their problem list from 2010 to 2020. Patient demographics, hospitalization characteristics, and discharge information were obtained. RESULTS: In total, 1140 admissions were identified (266 EA/TEF, 135 CDH, 739 HD). Patients were mostly female (53%), had a mean age of 31.6 y, and often admitted to an adult internist in a general hospital under emergency. Principal diagnoses and procedures (when performed) varied with diagnosis and age at admission. EA patients were admitted with dysphagia and foregut symptoms and often underwent upper endoscopy with dilation. CDH patients were often admitted for diaphragmatic hernias and underwent adult diaphragm repair. Hirschsprung patients were often admitted for intestinal obstructive issues and frequently underwent colonoscopy but trended toward operative intervention with increasing age. CONCLUSIONS: Adults with congenital disease continue to require hospital admission and invasive procedures. As age increases, diagnoses and performed procedures for each diagnoses evolve. These data could guide the formulation of multispecialty disease-specific follow-up programs for these patients.

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Malignant central airway stenosis is treated with airway stent placement, but post-placement microbial characteristics remain unclear. We studied microbial features in 60 patients post-stent placement, focusing on changes during granulation tissue proliferation. Samples were collected before stent (N = 29), after stent on day 3 (N = 20), and after granulation tissue formation (AS-GTF, N = 43). Metagenomic sequencing showed significant respiratory tract microbiota changes with granulation tissue. The microbiota composition, dominated by Actinobacteria, Firmicutes, and Proteobacteria, was similar among the groups. At the species level, the AS-GTF group exhibited significant differences, with Peptostreptococcus stomatis and Achromobacter xylosoxidans enriched. Analysis based on tracheoesophageal fistula presence identified Tannerella forsythia and Stenotrophomonas maltophilia as the main differential species, enriched in the fistula subgroup. Viral and fungal detection showed Human gammaherpesvirus 4 and Candida albicans as the main species, respectively. These findings highlight microbiota changes after stent placement, potentially associated with granulation tissue proliferation, informing stent placement therapy and anti-infective treatment optimization. IMPORTANCE: Malignant central airway stenosis is a life-threatening condition that can be effectively treated with airway stent placement. However, despite its clinical importance, the microbial characteristics of the respiratory tract following stent insertion remain poorly understood. This study addresses this gap by investigating the microbial features in patients with malignant central airway stenosis after stent placement, with a specific focus on microbial changes during granulation tissue proliferation. The findings reveal significant alterations in the diversity and structure of the respiratory tract microbiota following the placement of malignant central airway stents. Notably, certain bacterial species, including Peptostreptococcus stomatis and Achromobacter xylosoxidans, exhibit distinct patterns in the after-stent granulation tissue formation group. Additionally, the presence of tracheoesophageal fistula further influences the microbial composition. These insights provide valuable references for optimizing stent placement therapy and enhancing clinical anti-infective strategies.

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AIM: Van der Zee (VdZ) described a technique to elongate the oesophagus in long-gap oesophageal atresia (LGOA) by thoracoscopic placement of external traction sutures (TPETS). Here, we describe our experience of using this technique. METHOD: Retrospective review of all LGOA + / - distal tracheo-oesophageal fistula (dTOF) cases where TPETS was used in our institutions. Data are given as medians (IQR). RESULTS: From 01/05/2019 to 01/03/2023, ten LGOA patients were treated by the VdZ technique. Five had oesophageal atresia (Gross type A or B, Group 1) and five had OA with a dTOF (type C, Group 2) but with a long gap precluding primary anastomosis. Age of first traction procedure was Group 1 = 53 (29-55) days and Group 2 = 3 (1-49) days. Median number of traction procedures = 3; time between first procedure and final anastomosis was 6 days (4-7). Four cases were converted to thoracotomy at the third procedure. Three had anastomotic leaks managed conservatively. Follow-up was 12-52 months. All patients achieved oesophageal continuity and were orally fed; no patient required an oesophagostomy. CONCLUSION: In this series, TPETS in LGOA facilitated delayed primary anastomoses and replicated the good results previously described but, in addition, was successful in cases with dTOF. We believe traction suture placement and tensioning benefit from being performed thoracoscopically because of excellent visualisation and the fact that the tension does not change when the chest is closed. Surgical and anaesthetic planning and expertise are crucial. It is now our management of choice in OA patients with a long gap with or without a distal TOF.

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