Safety and efficacy of conventional compared to segmented esophageal fully covered self-expanding metal stents: a retrospective multicenter case-control study.

BACKGROUND: Segmented self-expanding metal stents (SEMS) are an alternative to conventional unsegmented SEMS in the treatment of esophageal strictures. Due to their segmented design, they may adapt better to the surrounding structures making them less likely to migrate or cause trauma. We examined if there are clinically relevant differences between segmented and conventional esophageal SEMS in benign and malignant stenosis in terms of their functionality and safety. PATIENTS AND METHODS: We performed a multicenter, retrospective case-control study of segmented and conventional SEMS implantations in esophageal stenosis. Outcome parameters were adverse events such as migration, occlusion, and severe complications (i.e., bleeding and perforation). RESULTS: 79 segmented SEMS were identified and compared to 79 conventional SEMS implantations. Groups were similar in terms of age, gender, and etiology. We observed 13.9% severe complications (SEMS-associated clinically significant bleeding or perforation) in the conventional SEMS group compared to 3.8% in the segmented SEMS group. This difference was statistically significant (p = 0.025). Rates of migration and occlusion were similar between both groups. Likewise, there was no significant difference in terms of short-term (30 days) clinical success. CONCLUSION: In this first controlled analysis, segmented SEMS were associated with fewer severe clinical complications compared to conventional SEMS.

Long-gap esophageal atresia: gastric transposition or esophageal lengthening with delayed primary anastomosis? A systematic review.

PURPOSE: This study aims to evaluate different surgical approaches to long-gap esophageal atresia (LGEA) with or without tracheoesophageal fistula (TEF) is unclear. METHODS: A systematic literature review was done comparing gastric transposition versus esophageal lengthening with delayed primary anastomosis in infants with LGEA+/-TEF. The primary outcome was time to full oral feeds. Secondary outcomes were time to full enteric feeds, need for further surgery, growth, mortality, and postoperative adverse events. RESULTS: No comparative studies were found. However, the literature was re-interrogated for non-comparative studies. Four hundred thirty-eight articles were identified and screened, and 18 met the inclusion criteria. All were case series. Forty-three infants underwent gastric transposition, and 106 had esophageal lengthening with delayed primary anastomosis. One study on gastric transposition reported time to full oral feeds, and one study in each group reported growth. Time to full enteric feeds was reported in one study in each group. 30% of infants had further surgery following gastric transposition, including hiatus hernia repair (5/43, 12%) and esophageal dilation (7/43, 16%). Following esophageal lengthening, 62/106 (58%) had anti-reflux surgery, 58/106 (55%) esophageal dilatation and 11/106 (10%) esophageal stricture resection. Anastomotic complications occurred in 13/43 (30%), gastrointestinal in 16/43 (37%), respiratory in 17/43 (40%), and nerve injury in 2/43 (5%) of the gastric transposition group. In the esophageal lengthening group, anastomotic complications occurred in 68/106 (64%), gastrointestinal in 62/106 (58%), respiratory in 6/106 (6%), and none sustained nerve injury. Each group had one death due to a cause not directly related to the surgical procedure. CONCLUSIONS: This systematic review highlights the morbidity associated with both surgical procedures and the variety in reporting outcomes.

Management and Outcomes of Esophageal Atresia With or Without Tracheo-Esophageal Fistula Over 15 Years in South Africa.

INTRODUCTION: To determine the incidence, management and outcomes of esophageal atresia/tracheo-esophageal fistula (EA/TEF) over a 15-y period in South Africa. METHODS: A retrospective chart review of neonates with EA/TEF presenting at the main tertiary referral hospital in the KwaZulu-Natal province between 2002 and 2017 was conducted. Data collection comprised patient and maternal demographics, clinical presentations, laboratory and radiologic investigations, surgical procedures, and outcomes. A multivariate logistic regression determined the risk factors associated with mortality. RESULTS: Among 180 neonates, mean (SD) age of diagnosis was four (three) days postnatal with Gross Type C (n = 165, 92%) being the most common and the incidence was one per 10,000 live births. Majority were born term (n = 95, 53%) at peripheral hospitals (n = 167, 93%) with a mean birth weight of 2369 (736) grams. Overall HIV exposure rate was 27% (n = 48). Most (n = 138, 77%) patients presented with established pneumonia, 44% (n = 61) of whom required prolonged (>7 d) ventilator support. The median (IQR) hospital stay was 11 (8-20) d. Overall survival rate was 70% (n = 126). Birth weight <1500 g, life threatening anomalies, ventilation >30 d and postoperative sepsis contributed to mortality. CONCLUSIONS: Incidence, disease types and presentations were similar to developed countries. Despite advances in technology and neonatal care in Africa, EA/TEF surgical outcomes remain suboptimal likely due to caregivers' inability to care for these infants in disadvantaged socioeconomic circumstances with poor sanitation, etc. Research is needed to identify strategies tailored for disadvantaged communities which may contribute to improved outcomes in the perioperative and postoperative period.

Preoperative laryngotracheobronchoscopy in infants with esophageal atresia: why is it not routine?

The value of laryngotracheobronchoscopy (LTB) immediately prior to repair of esophageal atresia with or without tracheo-esophageal fistula is contentious. Currently, there is a wide range of opinion on the utility of this investigation which is reflected by huge variation in clinical practice. This review is a critical analysis of the arguments for and against performing routine LTB prior to esophageal atresia repair. Reserving LTB for selected cases only is potentially disadvantageous since it limits the surgeon's and anesthetist's familiarity with the procedure, equipment, and range of potential findings. There is sufficient evidence to suggest that routine preoperative LTB becomes the standard of care.

Esophageal Atresia with Tracheoesophageal Fistula and Gastroesophageal Reflux in Children: Dental Considerations and Case Report.

BACKGROUND: Esophageal Atresia (EA) is defined as the congenital interruption of the continuity of the esophagus. Pediatric patients also have other congenital conditions, such as Tracheo-Esophageal Fistula (TEF). CASE REPORT: A 7-year-old male with TEF referred by a Pediatric Cardiologist, with the principal complaint of "severe and generalized tooth wearing". Considering that the patient was systemically stable, it was decided to perform the oral procedures under local anesthesia and rubber-dam isolation with an antimicrobial prophylaxis regimen. The treatment consisted of the extraction of all maxillary primary incisors and canines and both first molars; in the mandibular arch, only the lower second right molar was extracted, and a distal shoe was placed. Pulpotomies were performed and preformed metallic crowns were placed on the remaining second primary molars, on both lower first molars, and on lower canines and lateral incisors. Finally, a fixed prosthesis was positioned in the upper arch, and cemented through orthodontic bands adapted to both crowned second molars. The patient has been maintained under close medical and dental control. The child showed satisfactory oral conditions, and the vomiting episodes had decreased significantly. CONCLUSIONS: Dentists can learn and then participate in the integral health management of infants and young children affected with EA/TEF, particularly those with dental erosion.

Computed tomography and upper gastrointestinal series findings of esophageal bronchi in infants.

BACKGROUND: Esophageal bronchus is a rare form of communicating bronchopulmonary foregut malformation and a rare but important cause of an opaque hemithorax on chest radiography. A higher incidence of esophageal bronchus is associated with esophageal atresia, tracheo-esophageal fistula (TEF) and VACTERL (vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities) association. In the presence of these conditions, the pediatric radiologist may be the first to consider the diagnosis of esophageal bronchus or esophageal lung. OBJECTIVE: To describe the imaging features in five children with esophageal bronchus. MATERIALS AND METHODS: We reviewed hospital records and teaching files at two large pediatric tertiary referral centers over the 24-year period from January 1992 to January 2016. We reviewed all imaging studies and tabulated findings on radiography, fluoroscopic upper gastrointestinal (GI) series and CT. We then described the imaging features of esophageal bronchi with emphasis on CT and upper GI findings in four infants and one toddler. RESULTS: Three cases were identified from one institution (cases 2, 3, 4) and two from another (cases 1, 5). All five cases occurred in association with other midline malformations: four of the five had VACTERL association and three of the five had esophageal atresia and TEF. CONCLUSION: Lung opacification, ipsilateral mediastinal shift, and an abnormal carina and anomalous vascular anatomy suggest an esophageal bronchus or an esophageal lung on CT. While esophageal bronchus is a rare cause of an opaque hemithorax, CT and upper GI imaging play key roles in its diagnosis. Associations with esophageal atresia with tracheo-esophageal fistula and VACTERL association are particularly pertinent. Early diagnosis of esophageal bronchus might prevent complications such as aspiration and infection, which can allow for parenchymal sparing surgery as opposed to pneumonectomy.

Use of jet ventilation in thoracoscopic tracheo-esophageal fistula repair-can both surgeons and anesthesiologists be happy?

Laparoscopic and open thoracic surgery in the neonate typically results in hypercapnea and low cardiac output with often poor surgical visualization as the anesthesiologist attempts to correct the respiratory derangements usually seen. We describe three cases in which jet ventilation provided not only superior ventilation with a return to normocapnea but also ideal operating conditions. In addition, jet ventilation utilizes lower mean airway pressures which typically results in improved cardiac output.

Absent upper blind Pouch in a case of tracheo-esophageal fistula.

A common upper airway and digestive tract is a rare congenital anomaly that is usually fatal and its exact incidence is not known. It is a diagnostic challenge as it requires high index of suspicion. It should be considered in a neonate with respiratory distress in a non-vigorous baby requiring endotracheal intubation, which is difficult even in expert hand. We present a newborn with suspected tracheo-esophageal fistula that was diagnosed intraoperatively to have absent upper blind pouch of the esophagus and on autopsy found to have laryngeal atresia with absent vocal cords and a common aerodigestive tract continuing distally with trachea. The neonate was ventilated with endotracheal tube (ETT) placement which in retrospect we came to know that it was in the esophagus. The neonate also had associated multiple congenital anomalies of VACTERL association. The importance of teamwork between neonatologist, pediatric surgeon, anesthesiologist, and radiologist is highlighted for diagnosis and management of such rare cases.

Esophageal lung resection and prosthesis placement in a preterm neonate.

This report describes a successful outcome in a preterm baby with an esophageal atresia and tracheo-esophageal fistula, who initially underwent a primary esophageal repair; but a persistent nonexpanding lung on the side of surgery led to further investigations. A further diagnosis of an esophageal lung resulted in pneumonectomy and prophylactic placement of an intra-thoracic prosthesis to prevent post-pneumonectomy syndrome. To the best of our knowledge, this is the first report of a prophylactic placement of an intra-thoracic prosthesis in a neonate with the condition of esophageal atresia and tracheo-esophageal fistula and associated esophageal lung.

The Value of Preoperative Rigid Tracheobronchoscopy for the Diagnosis of Tracheomalacia in Oesophageal Atresia Patients.

BACKGROUND: Oesophageal atresia (OA) is often accompanied by tracheomalacia (TM). The aim of this study was to evaluate its presence in OA patients during routine rigid tracheobronchoscopy (TBS) before primary correction and compare this to postoperative TBS and clinical signs of TM. METHODS: This retrospective cohort study included patients born with OA between June 2013 and December 2022 who had received a TBS before OA correction and had been followed for at least twelve months. Definite TM was postoperatively diagnosed through TBS, and probable TM was defined as having symptoms of TM. RESULTS: We analysed data from 79 patients, of whom 87% with OA type C. Preoperatively, TM was observed in 33 patients (42% of all patients), seven of whom had severe TM. Definite TM was observed in 21 patients (27%), of whom 15 had severe TM. Forty-one patients (52% of all patients) had developed symptoms of TM within twelve months, including harsh barking cough (n = 15), stridor and/or wheezing (n = 20), recurrent respiratory insufficiency (n = 11), or needing airway surgery (n = 7). The sensitivity of preoperative TBS for the presence of postoperative (definite and probable combined) TM is 50.0%, 95% CI [35.2-64.8], and the specificity 67.6%, 95% CI [51.7-81.1]. Clinical characteristics did not differ between the patients with or without postoperative TM. CONCLUSIONS: More than half of the studied patients with OA experienced symptoms of TM. While preoperative TBS is routinely performed prior to surgical OA correction, its predictive value for the presence of postoperative TM remains limited. LEVEL OF EVIDENCE: Level II. TYPE OF STUDY: Study of Diagnostics Test.

Tracheal agenesis with broncho-esophageal fistula in VACTERL / TACRD association.

Tracheal agenesis (TA) is an extremely rare malformation. We report here autopsy findings in a case of TA with bronchoesophageal fistula of Floyd type III. The other malformations present included laryngeal atresia, Right lung hypolobulation, ventricular septal defect in membranous portion, bilateral cystic renal dysplasia, spleninculus, Meckel's diverticulum, and imperforate anus. The constellations of malformations present in our case have overlapping features with Vertebral anomalies, Anal atresia, Cardiovascular anomalies, Tracheo-esophageal fistula, Esophageal atresia, Renal anomalies, Limb anomalies and Tracheal atresia or laryngo tracheal atresia, Cardiac anomalies, Renal anomalies, Duodenal atresia association described previously in the literature.

It's a Bird, It's a Plane, It's a Vein: Jugular Vein Phlebectasia in a Pediatric Patient With Tracheomalacia.

Jugular vein phlebectasia is seen in the first decade of life and carries a high chance of misdiagnosis as it can often be mistaken for other conditions observed in pediatric populations. High clinical suspicion along with radiological studies can help to confirm the diagnosis. Treatment is usually conservative, with surgery reserved for unique circumstances. This is the first case to be reported with concomitant tracheomalacia and a history of tracheoesophageal fistula repair in a pediatric patient with external jugular vein phlebectasia.

Prophylactic Acid-suppression Medication to Prevent Anastomotic Strictures After Oesophageal Atresia Surgery: A Systematic Review and Meta-analysis.

BACKGROUND: Anastomotic stricture is a common postoperative complication of oesophageal atresia ± tracheoesophageal fistula (OA/TOF) repair. Acid gastro-oesophageal reflux disease (GORD) is considered to be a factor in stricture formation and acid suppression medication is recommended post-operatively in consensus guidance. We aimed to investigate whether patients who were treated prophylactically with acid suppression medication had a reduced incidence of strictures compared to those who did not receive it. METHODS: A systematic review of studies was performed, searching multiple databases without language or date restrictions. Multiple reviewers independently assessed study eligibility and literature quality. The primary outcome was anastomotic stricture formation, with secondary outcomes of GORD, anastomotic leak, and oesophagitis. Meta-analysis was performed using a random effects model, and the results were expressed as an odds ratio (OR) with 95% confidence intervals (CI). RESULTS: No randomised studies on the topic were identified. Twelve observational studies were included in the analysis with ten reporting the primary outcome. The quality assessment showed a high risk of bias in several papers, predominantly due to non-objective methods of assessment of oesophageal stricture and the non-prospective, non-randomised nature of the studies. Overall, 1395 patients were evaluated, of which 753 received acid suppression medication. Meta-analysis revealed a trend towards increased odds of anastomotic strictures in infants receiving prophylactic medication, but this was not statistically significant (OR 1.33; 95% CI 0.92, 1.92). No significant differences were found in secondary outcomes. CONCLUSIONS: This meta-analysis found no evidence of a statistically significant link between the prophylactic prescribing of acid suppression medication and the risk of developing anastomotic stricture after OA repair. The literature in this area is limited to observational studies and a randomised controlled trial is recommended to explore this question. LEVEL OF EVIDENCE: Level III.

A Rare Case of Conservative Management of Multiple Aerodigestive Fistulas in a Patient.

Acquired aerodigestive fistulas include tracheoesophageal fistulas (TEF) and bronchoesophageal fistulas (BEF). Common causes of acquired fistulas are usually malignant in origin. Tubercular tracheoesophageal fistula and bronchoesophageal fistula are rare. The limited availability of literature often presents a challenge in the treatment of tubercular TEF. We present the case of a 47-year-old woman who presented with complaints of progressive dysphagia and epigastric pain. Preliminary investigation showed raised erythrocyte sedimentation rate (ESR) of 65 mm/h and further evaluation by esophagogastroduodenoscopy for dysphagia revealed multiple ulcerated lesions in the esophagus, computed tomography (CT) revealed the presence of tracheoesophageal and bronchoesophageal fistulas with lung consolidation, and histological examination revealed granulomatous inflammation. The symptoms were managed conservatively with anti-tubercular medicine alone and showed good response.

Coin Aspiration Presenting as Chronic Cough and Tracheoesophageal Fistula.

Chronic cough can be a diagnostic challenge in the pediatric population. Foreign body aspiration without typical signs and symptoms can often be overlooked as a cause of chronic cough in children. Coin aspirations in the trachea typically have a sagittal orientation on an anteroposterior (AP) chest radiograph. We report a rare case of a previously healthy five-year-old girl presenting with a chronic cough for five months caused by a coin with a coronal orientation on an AP chest radiograph. The coin, initially presumed to be lodged in the esophagus, was actually lodged in the cervical trachea, leading to the development of a tracheoesophageal fistula (TEF). Her AP chest radiograph showed a coronal, circular radio-opaque shadow and the lateral view a tangential radio-opaque shadow, prompting an initial evaluation by esophagogastroduodenoscopy, which was normal. She then underwent rigid bronchoscopy, revealing a coin lodged in the trachea along with a TEF. Surgical removal was achieved through an external approach with a vertical tracheotomy and insertion of a tracheostomy tube. Five days later, a repeat rigid bronchoscopy showed a well-healed TEF, and she was successfully decannulated. She was ultimately discharged home on room air and oral feeds. TEF as a complication of a foreign body lodged in the trachea or esophagus is rare but life-threatening. Foreign body aspiration should always be considered in the differential diagnosis when evaluating younger children with chronic cough.

Anatomy and embryology of tracheo-esophageal fistula.

Anomalies in tracheo-esophageal development result in a spectrum of congenital malformations ranging from, most commonly, esophageal atresia with or without trachea-esophageal fistula (EA+/-TEF) to esophageal web, duplication, stricture, tracheomalacia and tracheal agenesis. Despite the relative frequency of EA, however, the underlying etiology remains unknown and is likely due to a combination of genetic, epigenetic and environmental factors. In recent years, animal models have dramatically increased our understanding of the molecular and morphological processes involved in normal esophageal development during the key stages of anterior-posterior regionalization, dorsal-ventral patterning and morphogenic separation. Moreover, the use of animal models in conjunction with increasingly advanced techniques such as genomic sequencing, sophisticated live imaging studies and organoid models have more recently cast light on potential mechanisms involved in EA pathogenesis. This article aims to unravel some of the mysteries behind the anatomy and embryology of EA whilst providing insights into future directions for research.

Surgery Complicated by Self-Expandable Metallic Stents (SEMS) Tracheal Stent in a Congenital H-type Tracheo-Esophageal Fistula.

A congenital tracheo-esophageal fistula of the H-type is a rare variant. The diagnosis is usually missed because of mild symptoms. A long history of coughing during liquid intake and nocturnal cough may aid in the diagnosis. A delay in the diagnosis may have a deleterious effect on the lung because of recurrent infections. Surgery is the cornerstone of management. Self-expandable metallic stents (SEMS) do not have a role in the management of these fistulae. We report a case of a missed diagnosis of a congenital H-type fistula managed as an acquired tracheo-esophageal fistula with two attempts at conservative management with a tracheal self-expandable metallic stent. The difficulties and disadvantages of using self-expandable metallic stents for the management of benign tracheo-esophageal fistulae are also discussed.

Acquired tracheo-esophageal fistula in adult-a classical case of 'what not to do'.

Acquired tracheo-esophageal fistulas (TEFs) are challenging. The most common causes are prolonged intubation, malignancy, and trauma whereas granulomatous infections like tuberculosis are rare. Endoscopic intervention with esophageal or tracheal stenting or clipping is of unproven benefit in the management of such lesions, where surgical repair is almost invariably required. We report a case of a 32-year-old man, with a case of multidrug-resistant pulmonary tuberculosis. He had no history of malignancy or trauma. The patient developed spontaneous TEF probably due to mediastinal lymph node necrosis. Multiple attempts were made using staplers, clips, and atrial septal defect (ASD) device closure but were unsuccessful. The nuanced complication leads to very individualized course of treatment which was optimal for this patient.

Management of Cervical Tracheo-Esophageal Fistula by Lateral Cervical Approach: Our Experience'.

Acquired Tracheo-esophageal fistula (TEF) is a challenging and complicated condition. The laryngeal protection is lost in acquired TEF cases due to the established connection between the esophagus and the airways leading to aspiration, pneumonia, and acute respiratory distress syndrome. Malignancy contributes to about 80% of acquired TEF. Nonmalignant causes for TEF include prolonged ventilation, trauma (iatrogenic, penetrating, or blunt injury), foreign bodies, corrosive burns, and granulomatous infections. With the advancements in critical care, the incidence of TEF post-ventilation is on the rise in recent decades. We would like to share our experience managing ten cases of nonmalignant acquired cervical TEF by the lateral cervical approach at our institute. Apart from the isolated TEF cases, one patient with concomitant tracheal stenosis was repaired simultaneously with good postoperative results. TEF was identified in two cases following removal of T-tube and solid stent respectively and was repaired successfully with lateral cervical approach with strap muscle flap interposition.