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.

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.

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.

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.

A case of Gross E esophageal atresia discovered following a unique clinical course.

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.

A novel approach in managing challenging tracheoesophageal fistulae.

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.

Diagnosis and management of complete tracheal rings with concurrent tracheoesophageal fistula.

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.

The Current Thoracoscopic Management of Esophageal Atresia.

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.

Fístula traqueoesofágica en H en el período neonatal: dificultades en el diagnóstico y diferentes abordajes terapéuticos. Serie de casos / H-type tracheoesophageal fistula in neonatal period: Difficulties in diagnosis and different treatment approaches. A case series

La fístula traqueoesofágica congénita sin atresia esofágica asociada, conocida como tipo H, es una anomalía infrecuente. Se manifiesta con episodios de tos, ahogo y cianosis durante la alimentación y/o neumonía recurrente.Si bien los síntomas están usualmente presentes desde el nacimiento, el diagnóstico es difícil. La rareza de esta patología, los síntomas no específicos y las limitaciones en la demostración radiológica y endoscópica de la fístula contribuyen, a menudo, a la demora entre la presentación y la confirmación del diagnóstico.Se describen las manifestaciones clínicas, los métodos de evaluación y el tratamiento de 3 neonatos con esta patología, y se presentan recomendaciones para el diagnóstico a fin de evitar demoras innecesarias en el manejo de las fístulas en H.

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.

H-type tracheoesophageal fistula in the neonatal period: Difficulties in diagnosis and different treatment approaches. A case series. / Fístula traqueoesofágica en H en el período neonatal: dificultades en el diagnóstico y diferentes abordajes terapéuticos. Serie de casos.

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.

La fístula traqueoesofágica congénita sin atresia esofágica asociada, conocida como tipo H, es una anomalía infrecuente. Se manifiesta con episodios de tos, ahogo y cianosis durante la alimentación y/o neumonía recurrente. Si bien los síntomas están usualmente presentes desde el nacimiento, el diagnóstico es difícil. La rareza de esta patología, los síntomas no específicos y las limitaciones en la demostración radiológica y endoscópica de la fístula contribuyen, a menudo, a la demora entre la presentación y la confirmación del diagnóstico. Se describen las manifestaciones clínicas, los métodos de evaluación y el tratamiento de 3 neonatos con esta patología, y se presentan recomendaciones para el diagnóstico a fin de evitar demoras innecesarias en el manejo de las fístulas en H.

Congenital malformations potentially affecting respiratory function: multidisciplinary approach and follow-up.

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.

Ventilation Failure due to Endotracheal Tube Migration Into a Tracheal Pouch in a Toddler With a Repaired Tracheoesophageal Fistula: A Case Report.

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.

Novel approach for the dissection of upper pouch during primary repair of esophageal atresia with tracheoesophageal fistula: Technique and results.

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.

An interesting case of 'strange lines' a neonate with oesophageal atresia, tracheo-oesophageal fistula, situs inversus abdominalis and azygos continuation.

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.

[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.