Ineffective ventilation in a very low birth weight infant with H-type Tracheoesophageal Fistula with bilateral chemical pneumonitis. A lesson learned.

Tracheoesophageal fistula (TEF) with or without associated esophageal atresia (EA) in the neonate is challenging to diagnose and manage its complications like aspiration, respiratory distress, and other associated anomalies. To stabilize, ventilate and prepare for surgical correction, understanding the H-nature of disease and anticipation of problems and their management will improve survival. We present a newborn with tracheoesophageal fistula without atresia from resource-limited settings and lessons we learned from the case.

Mysterious Late Presentation of Congenital H-type Tracheoesophageal Fistula in Teenage - Teamwork and Operative Planning.

Congenital tracheoesophageal fistula (TEF) is a result of the failed fusion of tracheoesophageal ridges during the 3rd week of gestation. Commonly, TEF is associated with esophageal atresia, which is detected and treated in newborns. Isolated TEF is rare with an overall incidence of 1 in 80,000 live births. Very rarely, they can present in adulthood. This report is adding one such patient at the age of 17 years who presented with the first episode of aspiration pneumonia and a history of retrosternal discomfort and regurgitation since childhood to the limited world experience.

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.

Flexible endoscopic identification and catheterization of congenital H-type tracheoesophageal fistula using a laryngeal mask.

Endoscopy for revealing the orifice of congenital H-type tracheoesophageal fistula (cTEF) is important for diagnostics and therapeutics. To facilitate the identification and catheterization of cTEF, we developed a new modified flexible endoscopy technique using a laryngeal mask with intermittent airflow. A retrospective case series study was conducted from April 2016 to July 2019 at a national regional children's medical center. Twelve infants with cTEF underwent this flexible endoscopy technique. The intermittent positive pressure airflow through laryngeal mask was able to reveal the orifice of cTEF easily in tracheal lumen. Under the visual flexible endoscope, cannulation with a 3-Fr ureteral catheter in fistula was successfully used in all cases. There were no immediate or delayed complications. This case series shows that the flexible endoscopy technique is a safe, easy, and technically efficient approach for diagnosis and cannulation of cTEF.

Practical safety in the diagnosis and treatment of congenital isolated tracheoesophageal fistula.

The rareness of H-type tracheoesophageal fistula in conjunction with its unspecific clinical presentation and wide range of anatomical presentation makes its diagnosis and treatment a problematic topic for both ear, nose and throat (ENT) specialists and pediatric surgeons worldwide. Symptoms and clinical signs of H-TOF are easily misleading. Diagnostic methods, most of the times, are dependent on the physician's experience; therefore, various errors may be made. We analyzed our experience in managing H-TOF cases over the last 15 years. Advice and strategies of action for health professionals directly involved in the diagnosis and treatment were identified, but also errors and mistakes while managing 6 cases. We analyzed 'red flags' but also important steps in the practical safety concerning this rare congenital malformation. Choosing the surgical access for division of the fistula throughout the cervical or thoracic approach is sometimes difficult. A scrupulous perioperative planning is mandatory. A dynamic overview of the patient's presentation never underestimating the subtlety of H-TOF presentation should be conducted for its early recognition and achieving best outcomes.

Diagnostic and management strategies for congenital H-type tracheoesophageal fistula: a systematic review.

BACKGROUND: H type tracheoesophageal fistula (H-TEF) is a rare congenital anomaly. Management may be complicated by late diagnosis and variation(s) in the therapeutic strategy. A systematic review of published studies explores the utility of diagnostic studies, operations and postoperative complications. METHODS: Medline and PubMed database(s) were searched for ALL studies reporting H-TEF during 1997-2020. Using PRISMA methodology, manuscripts were screened for eligibility and reporting. RESULTS: Forty-seven eligible studies were analysed. Primary diagnosis varied widely with surgeons performing oesophagography and trachea-bronchoscopy. Preoperative localisation techniques included fluoroscopy, guidewire placement and catheterisation. A cervical approach (209 of 272 cases), as well as thoracotomy, thoracoscopy and endoscopic fistula ligation, were all described. Morbidity included fistula recurrence (1.7%), leak (2%), tracheomalacia (3.4%) and respiratory sequelae (1%). The major adverse complication in all studies was vocal cord palsy secondary to laryngeal nerve injury (18.5%) yet strikingly few centres routinely reported undertaking vocal cord screening pre or postoperatively. CONCLUSION: This study shows that paediatric surgeons record low volume activity with H type tracheoesophageal fistula. Variation(s) in clinical practice are widely evident. Laryngeal nerve injury and its subsequent management warrant special consideration. Care pathways may offset attendant morbidity and define 'best practice.'

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.

Experience of diagnosis and treatment of 31 H-type tracheoesophageal fistula in a single clinical center.

OBJECTIVE: To summarize the experience of the diagnosis and treatment of 31 H-type tracheoesophageal fistula (TEF) at the Children's Hospital of Chongqing Medical University, Chongqing, China. METHODS: A total of 31 patients with H-type TEF were enrolled in this retrospective study from January 2000 to July 2017, and the diagnosis and treatment of the disease were analyzed and summarized. RESULTS: Iodine oil examination of the esophagus, fiberoptic bronchoscopy combined with esophagoscopy and three-dimensional computed tomography (3-D CT) reconstruction of the trachea and esophagus was used separately in 31 patients. 1 patient who had his TEF ligatured directly experienced recurrence 1 month after surgery, but recovered after TEF suturing. Subsequently, the remaining 30 patients were treated by suturing after TEF excision. All patients were followed up for 3 months-2 years and did not suffer from anastomosis orifice stenosis, gastroesophageal reflux, and a recurrent respiratory tract infection. Three patients exhibited vocal cord paralysis and improved spontaneously after a follow-up of 3-6 months. CONCLUSION: H-type TEF is diagnosed by iodine oil examination of the esophagus, fiberoptic bronchoscopy combined with esophagoscopy, and 3-D CT reconstruction of the trachea and esophagus. Excision and suturing of the TEF separately are an effective treatment for the disease and are an excellent outcome.

Congenital H-type tracheoesophageal fistula: a national multicenter study.

BACKGROUND: Congenital H-type tracheoesophageal fistula (TEF) is very rare and represents <5 % of all congenital tracheoesophageal malformations. This is a national, multicenter review of our experience with isolated H-type TEF outlining clinical presentation, methods of diagnosis, associated anomalies, treatment and outcome PATIENTS AND METHODS: The medical records of all patients with the diagnosis of congenital H-type TEF treated at four pediatric surgery units in Saudi Arabia were retrospectively reviewed for: age at diagnosis, sex, presenting symptoms, associated anomalies, method of diagnosis, treatment and outcome. RESULTS: During the study period (January 1998-December 2013), 435 infants and children with the diagnosis of esophageal atresia with or without TEF were treated. Among these, 23 (5.3 %) had isolated TEF. There were 11 males and 12 females. Their age at presentation ranged from 5 days to 3 years and 7 months but the majority (90 %) were diagnosed during their first year of life. Their clinical presentation included: chocking and coughing during feeds in 12 (52.2 %), recurrent chest infection in 16 (69.6 %) and cyanosis in 10 (43.5 %). One presented with abdominal distension also. The diagnosis was made using esophagogram. In 11 (47.8 %), a single study confirmed the diagnosis, 8 (34.8 %) required two studies while 4 (17.4 %) required three studies. Nineteen (82.6 %) had preoperative bronchoscopy and in 13 (56.5 %), a catheter was used to cannulate the fistula. All were operated through a right cervical incision except one who underwent thoracoscopic ligation and division of the fistula. In one, the fistula was only transfixed and tied without being divided. This patient developed a recurrent fistula. Two patients developed postoperative stridor secondary to recurrent laryngeal nerve palsy. In both of them, there was complete recovery. CONCLUSIONS: H-type TEF is very rare and commonly presents with recurrent chest infection, chocking and coughing during feeds and cyanosis. Physicians caring for these patients should be aware of this and a high index of suspicion is of paramount importance to avoid delay in diagnosis with its associated morbidity. A contrast esophagogram is valuable in confirming the diagnosis. The study however may need to be repeated. Preoperative bronchoscopy is valuable to localize and cannulate the fistula for easier access during surgery. Surgical repair is the treatment of choice and this should be performed through a right cervical incision or thoracotomy for low fistulae. Thoracoscopic ligation and division of a low H-type fistula is an alternative and less invasive approach when compared to thoracotomy.

A case of acute respiratory distress syndrome associated with congenital H-type tracheoesophageal fistula and gastroesophageal reflux / 소아과

H-type tracheoesophageal fistula (TEF) is extremely rare in infants and children, and clinical manifestations of this condition are diverse based on its severity. Some cases of congenital TEF diagnosed in adulthood have been reported, which indicate the difficulty of early diagnosis of this disease. Gastroesophageal reflux (GER) may induce chronic aspiration, pulmonary aspiration, apparent life-threatening events, and failure to thrive. We report a 5-month- old boy whose recurrent pneumonia and wheezing did not improve under usual treatment and led to acute respiratory distress syndrome. He was found to have severe GER on the second-trial of the esophagogram and was eventually revealed to have congenital H-type TEF upon repeated evaluation.

A Case of H-type Tracheoesophageal Fistula Diagnosed by Esophagography in Prone Position

An H-type tracheoesophageal fistula is a rare congenital condition of which incidence is approximately 1: 50, 000-80, 000 births. Although patient usually has recurrent respiratory symptoms such as choking episodes and cyanotic spells, its rarity and atypical symptoms may delay its detection. Besides, conventional esophagogram often fail to find the defect. We reported a case of H-type tracheoesophageal fistula, diagnosed by tube esophagram with infant in the prone position. During the test, contrast medium was injected while the catheter is slowly withdraws from lower esophagus.

A case of agenesis of the right lung with H-type tracheoesophageal fistula

No abstract available.