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Competing Interests: Declaration of interests We declare no competing interests.

Background: Tracheal agenesis is a rare and often fatal congenital anomaly that occurs early in fetal development. Tracheal agenesis occurs in one in 50,000 to 100,000 live births. This case study describes the nursing aspect of caring for this type of patient., Clinical Findings: Airway anomalies in neonates can be diagnosed immediately at birth or later when the infant develops respiratory distress or respiratory failure. Diagnosis and management of tracheal agenesis is difficult and a complex problem requiring a multidisciplinary medical team's expert approach for its treatment., Primary Diagnosis: Respiratory distress syndrome versus tracheoesophageal fistula was suspected., Interventions: The infant quickly decompensated, requiring intubation and eventual transfer to our tertiary care center for further evaluation of a possible airway anomaly. Because of deteriorating status, surgery was performed, and it was discovered the patient had tracheal agenesis, requiring the development of a 3-dimensional trachea specific for this patient., Outcomes: This article describes the nursing aspect of caring for this type of patient., Practice Recommendations: This article describes the success of nursing interventions and teamwork among nursing and the multidisciplinary team for the successful discharge of this patient home to her family., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2023 by The National Association of Neonatal Nurses.)

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., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

This case study presents an infant with non-specific symptoms for esophageal atresia and tracheoesophageal fistula shortly after birth. A detailed examination of the infant provides clues to the potential diagnosis while the radiograph provides confirmation. This column provides the clinician with a detailed evaluation of the x-ray findings specific to the infant with esophageal atresia with and without tracheo esophageal fistula. Additionally, a description of the different types of this congenital anomaly, embryology, pathophysiology, testing, treatment, and nursing considerations is presented., (© Copyright 2022 Springer Publishing Company, LLC.)

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

Objective: To analyze the outcomes of an open anterior cervical approach and tospecifically describe a novel extended tracheotomy incision ("Key-hole technique") torepair H-type and other challenging tracheoesophageal fistulae (TOF) at a singletertiary pediatric center., Method: A retrospective chart analysis of pediatric patients (0-18 years old) who had undergone repair of TOF's between January 2006 and March 2020 were reviewed. A case series of patients who had undergone open cervical utilizing three different techniques were included. Patient demographics, surgical management and post-operative surgical outcomes including complications were evaluated., Results: During the study period, 117 pediatric patients were diagnosed and anaged with TOFs with or without oesophageal atresia. Within this group, 12 patients (10%) had anterior open cervical repair of congenital or persisting TOFs (6 males and 6 females). Eight cases (7%) had congenital Type E (known as H-type), two had type D, one type B and one type C TOF. Median gestational age was 37 weeks (range 28-41 weeks), age of presentation ranged from 1 day old to 3 years old with 67% being diagnosed within the first month of life. At the time of definitive surgery all patients had a bronchoscopy and oesophagoscopy to confirm the diagnosis, identify the level of the fistula and place a catheter through the fistula. This cases series of open anterior cervical repair of TOFs comprised of seven (58%) patients who had primary extraluminal tracheal approach, four (33%) with extended tracheotomy incision ('Key-hole' technique) and one (9%) patient with slide tracheoplasty for recurrent type C TOF in the presence of subglottic stenosis. Eleven of the twelve patients had successful open anterior cervical repair of TOF. One patient who had primary open anterior cervical repair with the 'Key-hole' technique had recurrence managed successfully with slide tracheoplasty. There were no cases of recurrent laryngeal nerve injury., Conclusion: This series demonstrates that open anterior cervical approach to correct TOFs is an effective and safe method in the majority of cases of congenital and acquired fistulae where there is no oesophageal atresia or the atresia is corrected (in the case of recurrent or second fistulae). We also present the outcomes of a novel surgical "Keyhole" technique to manage TOF fistulas via an extended-tracheotomy incision. We also found that slide tracheoplasty is an effective salvage operation in the case of complex recurrent fistulae., (Crown Copyright © 2020. Published by Elsevier B.V. All rights reserved.)

Background and Aim: Congenital malformations such as oesophageal atresia (OA) and tracheoesophageal fistula (TOF), congenital pulmonary airway malformations (CPAMs), congenital diaphragmatic hernia (CDH) and vascular rings (VRs) can affect lung development and respiratory function. This observational study describes our multidisciplinary approach and respiratory follow-up of children with such congenital malformations., Methods: Clinical data of children followed at the Pediatric Respiratory Unit of Parma University Hospital (Italy) between January 2015 and January 2020 were collected.  Results. Twenty-three patients with congenital malformation affecting lung development were identified. Almost half of our patients were diagnosed with fetal ultrasound.  Children attended the clinic at a mean age of 3 (3.7) years and follow-up visits were scheduled every 6 months average. More than half of our patients were hospitalized for lower respiratory tract infections. Six out of 9 children able to perform spirometry showed anomalies in lung function. Chest physiotherapy was recommended especially in children with OA., Conclusions: Children with congenital malformations affecting lung development are at risk of short and long-term respiratory complications, especially in the first years of life. OA was the malformation more associated to respiratory problems. Multidisciplinary approach and appropriate personalized follow-up are recommended for the best management of these children.   Abstract word count: 186.

Objective: Characterize patients with complete tracheal rings and tracheoesophageal fistula (TEF) and summarize management options., Methods: A systematic review of patients under 18 years of age with complete tracheal rings and TEF was conducted. Authors were contacted for additional patient information and new cases were added. Patients with iatrogenic TEF and tracheal stenosis due to other causes were excluded., Results: Sixteen patients with a median (IQR) follow-up of 10 months (3-12 months) were identified. All had a distal TEF with complete tracheal rings distal to the TEF. There were 10 (63%) type C esophageal atresia + TEF (EA/TEF), and 1 (6%) type D (5 missing data). Median (IQR) airway diameter was 2 mm (1.5-2.2 mm). Complete tracheal rings were diagnosed prior to TEF repair in 5 (31.3%) patients, after ≥1 failed extubation in 3 (12.5%) patients, and intra-operatively during respiratory distress in 1 patient. Ten patients (62.5%) were intubated with an endotracheal tube and one with a 6 Fr flexible aortic canula (5 missing data). Four patients with an endotracheal tube for TEF repair developed ventilatory problems. Complete tracheal rings were repaired in 9 (56%) patients (8 slide tracheoplasty, 1 pericardial patch) and followed conservatively in 3 (19%). One patient required tracheotomy. Four patients died., Conclusions: Complete tracheal rings with concurrent TEF is a rare entity that pose challenges for ventilatory management during operative repair. Bronchoscopy prior to TEF repair is critical to allow for proper preoperative planning., Competing Interests: Declaration of competing interest None of the authors have any conflicts of interest to declare., (Crown Copyright © 2020. Published by Elsevier B.V. All rights reserved.)

The thoracoscopic repair of esophageal atresia with tracheoesophageal fistula is a complex neonatal minimally invasive procedure. The thoracoscopic approach is now nearing its third decade of experience and but is overall still not widely utilized, only in skilled centers and by experienced surgeons. This article will summarize the recent advancements in technique and knowledge in the thoracoscopic approach to this challenging neonatal congenital abnormality., Competing Interests: None declared., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: We describe a technical modification to approach for dissection of proximal pouch in patients undergoing the primary repair of congenital Esophageal atresia with tracheoesophageal fistula (EA-TEF)., Methods: A prospective comparative study was performed from January 2016 to December, 2019 including the patients who were undergoing primary repair of EA-TEF (Type C). The patients were divided into Group A (upper pouch dissection by classical approach) and Group B (technical modification). The two groups were compared for operative outcome and complications. Modified OSATS score was used to rate the performance of operators in two groups and were compared., Results: Total of 70 patients were included. In Group B, the mean operating time was less compared to Group A. The incidence of tracheal injury was also less when compared to previous data (p = 0.042). Mean OSATS score for flow of operation and overall performance was better in Group B (p = 0.002, p = 0.005). The OSATS score was also better for fifth and seventh case in Group B, proving its faster learning by trainee fellows., Conclusion: The technical modification decreases chances of tracheal injury and shortens the learning curve for upper pouch dissection., Level of Evidence: Level III., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Congenital tracheoesophageal fistula not associated with esophageal atresia, known as H-type fistula, is an uncommon anomaly. It presents with cough, choking, and cyanosis during feeding and/or recurrent pneumonia. Although symptoms are usually present from birth, diagnosis is difficult. The rarity of this disease, non-specific symptoms, and the limitations of radiological and endoscopic confirmation of the fistula often result in a delay between presentation and diagnosis confirmation. Here we describe the clinical manifestations, assessment methods, and management of 3 newborn infants with H-type tracheoesophageal fistula, together with diagnosis recommendations to prevent unnecessary delays in the management of this condition., Competing Interests: The authors report no conflicts of interest in this work., (Sociedad Argentina de Pediatría.)

A tracheal pouch is a rare complication of successful repair of a congenital tracheoesophageal fistula (TEF). An 18-month-old child with a repaired congenital TEF was scheduled for esophageal dilation to treat his esophageal stricture. Migration of the distal end of the endotracheal tube into a previously undetected tracheal pouch caused an abrupt failure to ventilate at the end of surgery. Given our experience, we recommend to screen the trachea of every patient with corrected TEF for a tracheal pouch when they are scheduled for another procedure requiring general anesthesia.

We describe the case of a term baby boy born via vaginal delivery at 39 weeks gestation with oesophageal atresia, tracheaoesophageal fistula, situs inversus abdominalis and azygos continuation. The azygos continuation was diagnosed after cardiac echo and confirmed on cardiac catherisation after an unexpected umbilical line position on thoracoabdominal X-ray. The baby underwent a right-sided thoracotomy on day 1 of life for repair of the oesophageal atresia. A double fistula, of both the proximal and distal segments, of the oesophagus with short segment stenosis was confirmed. The tracheo-oesophageal fistulae were ligated and divided and the oesophageal atresia repaired by primary anastomosis without complications. The azygos vein was not ligated., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Oesophageal atresia causes a dysplasia of the oesophagus with or without a connection to the adjoining trachea. Prenatal ultrasound results are not specific enough to confirm a suspected diagnosis. In addition to polyhydramnios and a small or absent stomach, the so-called "pouch sign" reinforces the suspected diagnosis. An MRI increases the prenatal detection rate. Due to the lack of reliable sonografic markers, ultrasonic testing is advised during pregnancy. Particularly, further causes for the polyhydramnios should be categorically excluded. Postnatally, children present with classic symptoms. Surgical treatment results in a very high quality of life and a very good prognosis. Nevertheless lifelong monitoring and follow-up of the patient is required., Competing Interests: Die Autoren geben an, dass kein Interessenkonflikt besteht., (© Georg Thieme Verlag KG Stuttgart · New York.)

Congenital bronchial stenosis is a rare entity [1,2]. While there are some reports of congenital bronchial anomalies and their repair in the thoracic literature this is first report in the literature of a bronchial take off just below the larynx managed with surgical success. Here we present a case of a former 33-weeker born with a tracheoesophageal fistula, Tetralogy of Fallot, and a high bifurcation of the right mainstem bronchus with a concomitant long segment bronchial stenosis. Preoperative planning included printing 3-D reconstructed models of the airway to analyze various treatment options. At 3 months of age the patient was taken to the operating room for surgical repair of the Tetralogy of Fallot and side-to-side tracheobroncheoplasty. The patient was extubated on postoperative day 3 and was discharged home without need for any ventilatory support., (Copyright © 2018. Published by Elsevier B.V.)

Background/purpose: This study was designed to determine the volume, postoperative surgical outcomes and, if possible, the relationship between outcome and institutional / surgeon volume in neonates undergoing repair of esophageal atresia with tracheoesophageal fistula (EA-TEF) over the last 20years in Ontario., Methods: Using administrative databases, a population based cohort study of patients undergoing EA-TEF repair in Ontario between 1993 and 2012 was conducted., Results: 465 patients with the diagnosis of EA-TEF met inclusion criteria. The mean number of EA-TEF repairs per year per was 5.8. There was a significant difference in hospital annual volume between institutions (range 12.3-3.35: p<0.05). The average number of cases/surgeon for the last 10 study years ranged between 0.5 and 2 cases/year. Primary outcome revealed that repair of recurrent fistula or intestinal interposition was 5.3%, with no reportable difference between institutions. Secondary outcomes revealed that 45.6% underwent dilatation for esophageal strictures, and 19.8% underwent some type of drainage procedure of the chest. These rates were not significantly different between institutions., Conclusion: This study provides insight into the outcomes following EA-TEF repair in Ontario and the difficulty in determining surgeon or institution volume outcome relationships, as both primary and secondary outcome event rates are very low., Level of Evidence: 2., (Copyright © 2018. Published by Elsevier Inc.)

Background: Tracheoesophageal fistula (TEF) and esophageal atresia (EA) are rare anomalies in neonates. Up to 50% of neonates with TEF/EA will have Vertebral anomalies (V), Anal atresia (A), Cardiac anomalies (C), Tracheoesophageal fistula (T), Esophageal atresia (E), Renal anomalies (R), and Limb anomalies (L) (VACTERL) association, which has the potential to cause serious morbidity., Purpose: Timely management of the neonate can greatly impact the infant's overall outcome. Spreading latest evidence-based knowledge and sharing practical experience with clinicians across various levels of the neonatal intensive care unit and well-baby units have the potential to decrease the rate of morbidity and mortality., Methods/search Strategy: PubMed, CINAHL, Cochrane Review, and Google Scholar were used to search key words- tracheoesophageal fistula, esophageal atresia, TEF/EA, VACTERL, long gap, post-operative management, NICU, pediatric surgery-for articles that were relevant and current., Findings/results: Advancements in both technology and medicine have helped identify and decrease postsurgical complications. More understanding and clarity are needed to manage acid suppression and its effects in a timely way., Implications for Practice: Knowing the clinical signs of potential TEF/EA, clinicians can initiate preoperative management and expedite transfer to a hospital with pediatric surgeons who are experts in TEF/EA management to prevent long-term morbidity., Implications for Research: Various methods of perioperative management exist, and future studies should look into standardizing perioperative care. Other areas of research should include acid suppression recommendation, reducing long-term morbidity seen in patients with TEF/EA, postoperative complications, and how we can safely and effectively decrease the length of time to surgery for long-gap atresia in neonates.

Objectives: Recurrent congenital tracheoesophageal fistula (TEF) is well documented in infancy but may also present later in life. This study reviews our experience with the clinical presentation, diagnosis and management of 5 recurrent and 2 primary congenital tracheoesophageal fistulas (TEF) in adult patients. There are no literature series of late recurrence of TEF (repaired in childhood and recurring in adulthood) and relatively few reported cases of initial adult presentation of TEF. In this series, we aim to provide the first large series description of late recurrence of congenital TEF following repair in infancy or childhood. We also present management considerations for this unique group of patients., Methods: We performed a computer-based search of the adult thoracic surgery departmental operative database at our institution from 2002 to 2014. Patients with iatrogenic TEF or malignant TEF were excluded., Results: Seven patients are included in our series. Five patients (71%) had recurrent congenital TEF and 2 (29%) had initial diagnosis of congenital TEF. All presented with severe coughing symptoms, and most (86%) had a history of recurrent aspiration pneumonia. Repair technique was dictated by the location of the TEF and the specific tracheoesophageal pathology. Four patients underwent repair via cervical approach with or without a tracheal resection. Three patients with distal recurrence underwent repair via right thoracotomy with partial oesophagectomy for significant tissue compromise and cervical reconstruction. Patients were followed for at least 1 year. All 7 patients experienced full resolution of symptoms. One patient required postoperative placement of a retrievable tracheal stent for tracheomalacia. There were no mortalities., Conclusions: Adult presentation of congenital TEF is a rare but recognizable clinical entity. Recurrent TEF in adulthood is a possible late complication of TEF repair performed in childhood that has not previously been described. Adult patients experiencing symptoms of cough and recurrent aspiration pneumonia should be evaluated for congenital TEF. Surgical repair of congenital TEF in the adult is feasible and effective with acceptable morbidity., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: Intraoperative hypercapnia and acidosis have been associated with thoracoscopic repair of both congenital diaphragmatic hernia and esophageal atresia/tracheoesophageal fistula., Aim: The aim of the present study was to investigate whether thoracoscopic repair of congenital diaphragmatic hernia or esophageal atresia/tracheoesophageal fistula was associated with acidosis and hypercapnia in a large group of neonates, and to analyze the effects of acidosis and hypercapnia on early postoperative outcomes., Methods: We reviewed the charts of neonates who underwent open or thoracoscopic congenital diaphragmatic hernia or esophageal atresia/tracheoesophageal fistula repair (2004-2014). Patients with available intraoperative arterial gas values were included. Data (PaCO 2 : mm Hg) were compared using paired/unpaired tests and are reported as difference [95% confidence interval]., Results: Congenital diaphragmatic hernia: 187 neonates underwent open (n=153) or thoracoscopic (n=34) repair. Intraoperative arterial gas values were recorded in 96 open and in 23 thoracoscopic operations. Both groups had similar preoperative pH and PaCO 2 , and developed intraoperative acidosis (open -0.08 [-0.11, -0.05] P<.001, thoracoscopic -0.14 [-0.24, -0.04] P=.01) and hypercapnia (open: 7.8 [3.2, 12.4], P=.002; thoracoscopic: 20.2 [-2.5, 43, P=.07). Intraoperatively, neonates undergoing thoracoscopic repair developed lower pH than those having open surgery (-0.06 [-0.01, -0.10] P=.018), but maintained similar levels of PaCO 2 (-4.0 [-9.0, 4.4] P=.39). Esophageal atresia/tracheoesophageal fistula: 205 neonates underwent open (n=180) or thoracoscopic (n=25) repair. Intraoperative arterial gas values were recorded in 62 open and in 14 thoracoscopic operations. Both groups had similar preoperative pH and PaCO 2 , and developed intraoperative acidosis (open: -0.09 [-0.14, -0.04], P<.001; thoracoscopic: 0.21 [-0.28, -0.14], P<.001) and hypercapnia (open: 9.2 [2.6, 15.7] P=.008; thoracoscopic: 15.2 [1.6, 28.7], P=.03). Intraoperatively, neonates undergoing thoracoscopic repair developed lower pH than those having open surgery (difference 0.08 [0.01, 0.15], P=.02) but maintained similar levels of PaCO 2 (difference -1 [-9, 3], P=.35)., Conclusion: Neonates undergoing operative repair of congenital diaphragmatic hernia and esophageal atresia/tracheoesophageal fistula develop intraoperative acidosis and hypercapnia, regardless of the approach used. However, this phenomenon is more severe during thoracoscopic repair. Novel modalities to reduce intraoperative gas derangements, particularly during thoracoscopic repair, need to be established., (© 2017 John Wiley & Sons Ltd.)

Laryngotracheoesophageal clefts (LTECs) are rare congenital defects that are often accompanied by additional anomalies. The major issues in the treatment of these patients are intraoperative exposure insufficiency, technical difficulty of the operation, and anesthesia problems originating from the respiratory tract. Problems originating from mechanical ventilation and respiratory tract, eating disorders and relapse of fistula are among the problems encountered following surgery. Most of the time, concomitant additional anomalies also worsen the clinical picture. It was our aim with these case reports to report our experience in two cases with Type IV LTEC ranging from the inoperable type IV LTEC due to additional anomalies mounted up to severe respiratory distress to the carina that we operated on with a single stage anterior cervicothoracic approach on its fifth day on life., Competing Interests: The authors declare no competing interests.

Objective: To examine the prevalence of upper airway anomalies in patients diagnosed with congenital tracheoesophageal fistula and esophageal atresia (TEF/EA)., Methods: A retrospective review was conducted of all TEF/EA patients seen at a tertiary pediatric hospital between January 2008 and December 2013. Inclusion criteria included evaluation by the otolaryngology service. Exclusion criteria included age>18 years, acquired TEF/EA, subsequent rule out of TEF/EA, and otolaryngology evaluation for reasons not pertaining to the airway. Data collected and analyzed included demographics, comorbidities, presenting symptoms, surgical interventions, laryngoscopic and bronchoscopic examinations, and subsequent medical and surgical management., Results: Four hundred and thirty patients were diagnosed with TEF/EA at our institution. In all, 32.3%, or 139 children, were included in the analysis; 56.1% (n=78) male, 43.9% (n=61) female. Of the analyzed patients, 4.3% (n=6) were diagnosed with laryngomalacia. Eighteen patients (12.9%) were diagnosed with subglottic stenosis. Thirty (21.6%) had vocal fold paresis or immobility. Laryngeal cleft was diagnosed in 25.9% (n=36). Tracheomalacia was the most common airway finding, diagnosed in 37.4% (n=52) patients., Conclusion: Patients diagnosed with congenital TEF/EA have a high rate of secondary upper airway anomalies. Consideration should be given to perform a complete airway evaluation in all of these patients., (© The Author(s) 2015.)

Objective: The aim of this study was to evaluate the performance of prenatal diagnosis of esophageal atresia (EA) and its associated abnormalities., Methods: We conducted a retrospective study from a pediatric database of EA managed postnatally in a single center. Prenatal data included ultrasound and magnetic resonance imaging parameters including amniotic fluid (AF) volume, stomach visualization, AF biochemistry, and associated malformations. Postnatal data included type of EA, mortality, and postnatal diagnosis of associated malformations., Results: One hundred twenty-two cases were included. The diagnosis was suspected prenatally in 39/122 (32%) cases. Polyhydramnios was noted in 64/122 (52.4%), and the stomach was not visualized or small in 39 (32%). There was 14 (11.5%), 2 (1.6%), 101 (82.8%), 5 (4.1%), and 0 (0%) types I, II, III, IV, and V, respectively. EA was suspected prenatally in 12/14 (85.7%) in type I and in 27/108 (25%) in cases with tracheoesophageal fistula (II + III + IV + V). Magnetic resonance imaging was performed in 28 cases, which confirmed EA in 19/28 (sensitivity 67.8%). AF biochemistry was performed in 17 cases, which confirmed EA in 15/17 (sensitivity 88.2%) cases. Of the 69 syndromic associations, 41/69 (59.4%) cases were detected prenatally. Associated malformation was a strong predictor of postnatal death [19/69 vs 3/53, odds ratio 6.33 (1.76; 22.75), p < 0.01]., Conclusion: Prenatal diagnosis of EA remains challenging. MRI and AF biochemistry may prove useful in the diagnosis of EA. Prenatal ultrasound and MRI examination should also focus on associated anomalies. © 2015 John Wiley & Sons, Ltd., (© 2015 John Wiley & Sons, Ltd.)

Background: The coexistence of congenital diaphragmatic hernia (CDH) with esophageal atresia (EA) has only been reported occasionally in literature. Series of patients from a single institution with comparison of different postnatal therapeutic approaches have not been reported. We describe our management in this unique cohort of patients and discuss the procedures that can lead to successful outcomes in this association of congenital anomalies., Methods: The surgical approaches and outcome of six neonates with CDH associated with EA and distal tracheo-esophageal fistula (TEF) are discussed., Results: Five newborns were treated surgically, while one patient with trisomy 18 only received palliative treatment. In four patients TEF was ligated during laparotomy for CDH repair. Secondary surgery was performed for correction of EA via thoracotomy after 4-6 weeks (primary anastomosis in two patients, Foker's-technique in one patient, one patient deceased prior to secondary surgery). All three surviving patients required fundoplication due to severe gastro-esophageal reflux during the first year of life. Two patients also required dilatation for anastomotic stricture. In one preterm infant correction of both malformations was accomplished during one surgical intervention. The herniated organs were eventrated and temporarily placed into a silastic bag to allow a mediastinal shift to the left. Thus a continuous ventilation of the right lung with minimal compression and sufficient oxygenation was possible during esophageal repair via a right-sided thoracotomy and extrapleural approach. No further surgery was required so far., Conclusions: Definitive surgical correction in newborns with CDH and EA was so far accomplished with multiple surgical interventions. Ligation of TEF via an abdominal approach with repair of CDH followed by delayed repair of EA is prone to stenosis and gastro-esophageal reflux due to loss of esophageal length. With a new combination of established surgical methods a single-staged correction of both malformations is possible. This new approach might help to preserve sufficient length of esophagus to accomplish primary anastomosis without tension and therefore avoid long-term morbidity and repetitive surgeries., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Introduction: Esophageal atresia/tracheo-esophageal fistula (EA/TEF) has an incidence of approximately 1:3,500. The incidence of malrotation is thought to be 1:200-500. We attempted to define the incidence of a combination and discuss the implications., Methods: This was a retrospective review of all patients admitted to a single institution with a diagnosis of EA or EA/TEF or TEF between April 1981 and January 2013. Patients were included if the position of the duodeno-jejunal flexure (DJF) was determined by upper GI contrast study (UGIS), surgery or post-mortem., Results: Case notes were reviewed for 235 patients. In the EA type A group, 3/28 (11 %; 95 % CI 3.7-27.2 %) had malrotation, significantly higher than the reported incidence of malrotation in the general population (p = 0.0008). All three patients in this group were symptomatic with one patient found to have a volvulus at emergency surgery. In the type C group, 6/196 (3 %, 95 % CI 1.4-6.5 %) had malrotation, significantly higher than the incidence reported for the general population (p = 0.0033) but not significantly different to that of the type A group (p = 0.0878). There were no patients with malrotation identified in any other EA/TEF type. In total, 9/235 (3.8 %; 95 % CI 2.0-7.2 %) patients with EA had malrotation, significantly higher than the 5/1,050 (0.48 %) reported for the general population (p = 0.0002)., Conclusion: There is a high incidence of malrotation in patients with pure EA. In the type A group an attempt to identify the DJF position at gastrostomy siting and/or performance of UGIS in the neonatal period should be undertaken. There should also be a low threshold for UGIS in all EA/TEF patients.

Background/purpose: Existing prediction models for tracheo-esophageal fistula (TEF) and esophageal atresia (EA) are derived from small single-institution populations treated over long periods. A prediction rule developed in a contemporary, multicenter cohort is important for counseling, tailoring therapy, and benchmarking outcomes., Methods: Data were obtained from the 2003, 2006, and 2009 editions of the HCUP Kids' Inpatient Database. Subjects included patients with admission age

Background: We present a case report of a trachea-colonic fistula and demonstrate our unique approach to repair, which was efficient and effective., Methods: The patient was a 50-year-old man who had a congenital tracheoesophageal fistula repair with colonic interposition as a child who now developed a fistula between his colon and trachea., Results: We performed a transtracheal approach, with primary closure of redundant colon mucosa as well as direct repair of the trachea. An inferiorly based sternocleidomastoid muscle flap was interposed between these 2 layers to augment the repair. The patient had an uneventful recovery with an effective reconstitution of the alimentary tract and the airway., Conclusion: Tracheo-colonic fistula is an extremely rare pathology, and the scarring that develops after a prior esophagectomy makes a traditional lateral approach very difficult. The transtracheal approach is an effective method to obtain needed exposure in order to carry out the repair., (© 2014 Wiley Periodicals, Inc.)

Purpose: Esophageal atresia (EA), with or without tracheoesophageal fistula (TEF), is associated with postoperative gastroesophageal reflux (GER). We performed a systematic review of the literature regarding routine anti-reflux medication post EA-TEF repair and its impact on postoperative GER and associated complications., Methods: A comprehensive search was conducted using MEDLINE, EMBASE, CINHAL, CENTRAL (Cochrane library) electronic databases and gray literature. Full-text screening was performed in duplicate. Included articles reported a primary diagnosis of EA-TEF, a secondary diagnosis of postoperative GER, and primary treatment of GER with anti-reflux medications., Results: Screening of 2,910 articles resulted in 25 articles (1,663 patients) for analysis. Most were single-center studies (92%) and retrospective (76%); there were no randomized control trials. Fifteen studies named the class of anti-reflux agent used, 3 the duration of therapy, and none either the dose prescribed or number of doses. Complications were inconsistently reported. Anti-reflux surgery was performed in 433/1,663 (26.0%) patients. Average follow-up was 53.2 months (14 studies)., Conclusion: The quality of literature regarding anti-reflux medication for GER post EA-TEF repair is poor. There are no well-outlined algorithms for anti-reflux agents, doses, or duration of therapy. Standardized protocols and reliable reporting are necessary to develop guidelines to better manage postoperative GER in EA-TEF patients.

Purpose: Aim of this systematic review is to investigate the thoracic and cervical surgical approaches of H-type tracheo-esophageal fistula (TEF) according to the position of the fistula., Methods: The PubMed database was searched for original studies on H-type TEF treatment published between 1977 and 2012. Manuscripts finally included were divided into open and thoracoscopic surgery groups., Results: Seventeen studies were selected for open surgery group, and most of them agree on the importance of pre-operative diagnosis of the fistula by preliminary tracheoscopy. Right cervicotomy was used in 70 cases (76.9%), left cervicotomy in 12 (13.2%), and thoracotomy only in 9 (9.9%). Five studies were included in thoracoscopic group (6 patients). Indications for the surgical approach (cervical vs thoracic) according to the position of the TEF were clearly described in 10 manuscripts, and all stated differences in surgical technique details. Complications and mortality rates were not statistically correlated to the different surgical approaches., Conclusions: The evidence base in regard to the treatment of H-type fistula in children is poor and the skills and preferences of the surgeons guide the choice of the procedure. Surgical division of the fistula is curative, and the key to a successful repair is the pre-operatively identification of the level of the fistula with tracheoscopy. Right cervicotomy seems to be the approach of choice in the majority of case, with the thoracic approach appropriate only for fistulae opening below T2. Further well-designed prospective studies which take into account of selection and performance bias are strongly required., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Communicating bronchopulmonary foregut malformations are a diverse group of congenital anomalies characterized by a fistula between the respiratory and alimentary systems. Among these malformations, the association of an esophageal lung with esophageal atresia (EA) and tracheo-esophageal fistula (TEF) is extremely rare. We report the case of a neonate with esophageal lung detected following the primary repair of EA with TEF. Despite the rarity of esophageal lung with EA and TEF, it should be considered to develop in infants with abnormal findings on chest radiographs and an unusual preoperative and/or in the postoperative clinical course of EA and TEF. Swallowing study with water-soluble contrast medium is the investigation of choice to demonstrate the anatomic connection, if EA and TEF were already primarily repaired. If not, although the use of preoperative CT may be debatable in neonates due to the associated radiation exposure, in selected cases such as our patient, preoperative CT can be useful for early preoperative diagnosis.

Background: Tracheoesophageal fistula (TEF) in dogs is a rare disease with only few reports in the literature. This report aims to contribute to the current literature on suitable diagnostic methods for TEF and to provide follow-up information after successful surgical treatment., Case Presentation: A seven-month-old intact female Spanish Water Dog was presented for further investigation of recurrent respiratory symptom. Bronchoscopy revealed a small hole-like defect in the tracheal wall at the bifurcation. The finding of the contrast material swallow study under fluoroscopy was indicative of a TEF. To further evaluate the connection between the trachea and esophagus, a computed tomography scan was performed. The TEF was surgically approached by thoracotomy through the right lateral sixth intercostal space. The fistula was identified, double ligated and divided. Histopathology confirmed the process to originate from the esophagus and to be patent. The dog was re-examined two weeks and ten months after surgery, with no evidence of recurring clinical signs., Conclusions: Contrast material swallow study using fluoroscopy was the most reliable diagnostic method. Bronchoscopy may allow the fistula to be visualized, but due to a small fistular opening it can lead to a false negative result. Surgical correction by ligation and dividing of the fistula suggests a good prognosis for early diagnosed and operated TEF.

Background/purpose: Administrative databases include large multi-institutional cohorts of patients with rare congenital anomalies that can potentially be used to characterize these diseases and study variations in practice and outcomes. The purpose of this study was to develop a methodology to accurately identify a cohort of patients with a rare disease (esophageal atresia and tracheoesophageal fistula, EA/TEF) in the Pediatric Health Information System (PHIS) database., Methods: Patients with EA/TEF treated from 2001 to 2010 were identified by chart review at two institutions and then located within the PHIS database to find ICD-9-CM coding patterns unique to EA/TEF. Subsequently, a step-wise search strategy for PHIS was developed to identify patients with EA/TEF: this included searching the ICD-9-CM diagnosis code for congenital EA/TEF; adding the ICD-9-CM code for acquired TEF; limiting age to ≤ 30 days; and adding at least one of a number of specified ICD-9-CM procedure codes. The PHIS search results were subsequently validated by chart review at each institution., Results: The institutional chart reviews identified 207 patients with EA/TEF. The most refined PHIS search strategy identified 221 patients. The positive predictive value of the search increased incrementally from 65% with using only the correct ICD-9 code to 96% with the full methodology. A cohort of 2977 patients with EA/TEF is identified when this search strategy is applied to the entire PHIS database., Conclusion: Administrative databases such as PHIS can be utilized to identify cohorts of patients with rare congenital anomalies; however, cohort development requires a systematic search strategy and validation process to ensure correct identification of patients., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Purpose: The morbidity and mortality associated with esophageal atresia with or without a fistula make it a challenging congenital abnormality for the pediatric surgeon. Anatomic factors like inter-pouch gap and origin of fistula are not taken into consideration in various prognostic classifications. The preoperative evaluation of these cases with computerized tomography (CT) has been used by various investigators to delineate these factors. We reviewed these studies to evaluate the usefulness of this investigation in the intra operative and post operative period., Materials and Methods: A literature search was done on all peer-reviewed articles published on preoperative computed tomography (CT) in cases of tracheoesophageal fistula using the PUBMED and MEDLINE search engines. Key words included tracheoesophageal fistula, computerized tomography, virtual bronchoscopy, and 3D computerized tomography reconstruction. Further, additional articles were selected from the list of references obtained from the retrieved publications. A total of 8 articles were selected for analysis., Results: In most of the studies, comprising 96 patients, observations noted in preoperative CT were confirmed during surgery. In a study by Mahalik et al [Mahalik SK, Sodhi KS, Narasimhan KL, Rao KL. Role of preoperative 3D CT reconstruction for evaluation of patients with esophageal atresia and tracheoesophageal fistula. Pediatr Surg Int. 2012 Jun 22. [Epub ahead of print]], in 6 patients the fistula could not be identified pre-operatively by CT. One study found a difference of 0.43 cm in the intraoperative gap length compared to that given by CT when compared to +/- 2 mm shown by another [1. Mahalik SK, Sodhi KS, Narasimhan KL, Rao KL. Role of preoperative 3D CT reconstruction for evaluation of patients with esophageal atresia and tracheoesophageal fistula. Pediatr Surg Int. 2012 Jun 22. [Epub ahead of print], 3. Ratan SK, Varshney A, Mullick S, Saxena NC, Kakkar S, Sodhi PK. Evaluation of neonates with esophageal atresia using chest CT scan. Pediatr Surg Int. 2004 Oct; 20(10):757-61.]. Only three studies showed surgical utility of pre-operative CT, and the surgical plan changed based on the CT findings., Conclusion: The usefulness of pre-operative computerized tomography in cases of tracheoesophageal fistula is controversial. The safety of this technique is questionable due to limited facilities and associated radiation hazards. Further, limited information obtained which may help in changing the surgical plan can easily be managed intra-operatively by careful and meticulous surgery. Overall, the safety concerns outweigh its efficacy. Thus, the use of pre-operative CT scan cannot be generalized and protocolized as a standard of care in the management of tracheoesophageal atresia., (© 2013.)

Lesions at the thoracic inlet are difficult to access via a thoracic or cervical approach. The use of the anterior cervico-thoracic trapdoor incision has been reported to give good exposure to the anterior superior mediastinum in adults. We report our experience of four cases where a trapdoor incision was used to gain excellent access and exposure to thoracic inlet pathology in children., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Repair of recurrent tracheoesophageal fistula (TEF) after repair of congenital esophageal atresia continues to be a difficult problem. The most common re-operation for a recurrent TEF involves repair via a right thoracotomy and use of a flap as interposed tissue between the closure sites. Although several materials have been reported for recurrent fistula repair, natural pedicled flaps have been used in most previous reports. The harvesting of a pedicled flap can sometimes be difficult when the patient has had multiple surgeries. In this report, the successful use of a combined free autologous auricular cartilage and free fascia lata graft repair for a complex patient with multiple recurrent TEFs is described. We believe that our technique should be considered as an approach for patients with recurrent TEF.

A case of esophageal atresia associated with a foregut duplication cyst is reported and the literature reviewed. This is the first documented occurrence in conjunction with Down syndrome and the second case where both anomalies were treated at the initial surgery., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Introduction: Isolated tracheoesophageal fistula without esophageal atresia is a rare congenital malformation. Its etiology is obscure. Diagnosis is difficult but must be made early., Purpose: To study the clinical, radiological, and evolutionary sights of this malformation., Patients and Methods: We report 4 cases of tracheoesophageal fistula, collected in the department of pediatric surgery of Monastir Hospital and in the neonatology unit of Sousse Hospital during the period between January 2001 and December 2010., Results: The clinical picture consisted in a coughing bout and cyanosis after each feeding. Thoracic and abdominal imaging showed aspiration pneumonia, atelectasis, and gas within the colon. Gastrointestinal opacification demonstrated the fistula in 2 cases. Tracheoscopy visualized the tracheoesophageal fistula in the other 2 cases. Treatment was surgical and consisted in the section-ligation of the tracheoesophageal fistula with pleural interposition in all cases. The course was simple in two cases with a 3-year and 3.5-year follow up, respectively, but the infants died in the other 2 cases., Conclusion: Although a rare malformation, tracheoesophageal fistula should be suggested as a diagnosis when respiratory symptoms occur during feeding starting during the neonatal period., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)