Tracheal Agenesis: One Hospital's Nursing Experience With This Rare Airway Anomaly Case Report.

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.

Intravenous cannula for percutaneous rescue needle gastrostomy in a neonate with tracheoesophageal fistula-A case report.

Tracheoesophageal fistula offers concrete difficulties for anesthesiologists, which comprise associated congenital anomalies and more importantly the problems concerning ventilation and oxygenation. Among all the types of tracheoesophageal fistula, ventilatory problems are frequently encountered with type C fistula. Effective ventilation can be a challenge in such cases where the endotracheal tube invariably ventilates the fistula causing stomach inflation and respiratory compromise. Thorough knowledge and experience are of utmost importance when it comes to the successful airway management and better survival of neonates undergoing tracheoesophageal fistula repair. We report a case of a 3-day-old neonate, diagnosed with type C tracheoesophageal fistula and esophageal atresia posted for thoracoscopic repair. We want to highlight our experience of percutaneous needle gastrostomy done using an intravenous cannula, as a rescue measure for stomach decompression, to manage life-threatening hypoxia.

An Infant With Esophageal Atresia and Tracheoesophageal Fistula: What Does the X-Ray Reveal?

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.

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

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

[Oesophageal Atresia - from the Challenge in Prenatal Medicine until Surgical Care]. / Die Ösophagusatresie ­ von der Herausforderung in der Pränatalmedizin bis zur chirurgischen Versorgung.

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.

Basic Knowledge of Tracheoesophageal Fistula and Esophageal Atresia.

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.

Intraoperative acidosis and hypercapnia during thoracoscopic repair of congenital diaphragmatic hernia and esophageal atresia/tracheoesophageal fistula.

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 (PaCO2 : 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 PaCO2 , 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 PaCO2 (-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 PaCO2 , 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 PaCO2 (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.

Performance of prenatal diagnosis in esophageal atresia.

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.

Upper Airway Anomalies in Congenital Tracheoesophageal Fistula and Esophageal Atresia Patients.

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.

Esophageal atresia and malrotation: what association?

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.

Management of a congenital tracheoesophageal fistula in a young Spanish water dog.

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.

The management of postoperative reflux in congenital esophageal atresia-tracheoesophageal fistula: a systematic review.

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.

Esophageal lung diagnosed following the primary repair of esophageal atresia with tracheo-esophageal fistula in a neonate.

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.

Combined free autologous auricular cartilage and fascia lata graft repair for a recurrent tracheoesophageal fistula.

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.

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

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

An audit of anesthetic management and complications of tracheo-esophageal fistula and esophageal atresia repair.

BACKGROUND: Many different anesthetic techniques have been suggested for the management of tracheo-oesophageal fistula/oesophageal atresia (TOF/OA) although the incidence of ventilation difficulty is not well known and it is unclear which technique is best in managing this. The aim of our audit was to determine the incidence of ventilation difficulty during repair of TOF/OA. We also recorded the current practice for anesthesia and analgesia in these children as well as the incidence of comorbidities and surgical complications. METHODS: We retrospectively audited cases of TOF/OA repair over a 3-year period in four hospitals, recording demographics, comorbidities, surgical data, postoperative complications, and anesthetic technique, including ventilation difficulty and management strategy. RESULTS: A total of 111 patients were identified with TOF/OA, and 106 patient notes and 101 anesthetic records were found. 42% of patients were premature, and 57.5% had significant comorbidities. Death was most likely in infants with low birth weight and low gestational age at birth and in those with major cardiac comorbidity. A range of techniques were used for induction, maintenance, extubation, and pain control. There were ventilation difficulties recorded at induction in seven patients, and significant desaturations were recorded in 15 patients intraoperatively. CONCLUSIONS: This audit adds to the data already published about incidences of complications and comorbidities associated with TOF/OA repair. Defining anesthetic practice with regard to ventilation and analgesic strategies is important in comparing the adequacy and risk of techniques used. Our audit shows that a range of differing anesthetic techniques are still employed by different anesthetists and institutions and details some of the techniques being used for managing difficult ventilation.