Proximal Tracheoesophageal Fistula Research Articles

Diagnose and treatment for Type D congenital esophageal atresia with tracheoesophageal fistula.

Type D esophageal atresia (EA) with tracheoesophageal fistula (TEF) is characterized by EA with both proximal and distal TEFs. It is a rare congenital anomaly with a very low incidence. To investigate diagnostic and treatment strategies for this rare condition. We retrospectively reviewed the clinicopathological features of patients with EA/TEF treated at our institution between January 2007 and September 2021. Among 386 patients with EA/TEF, 14 (3.6%) had type D EA/TEF. Only two patients were diagnosed with proximal TEF preoperatively. Seven patients were diagnosed intraoperatively. Five patients were missed for diagnosis during the initial surgery but was later confirmed by bronchoscopy. During the neonatal period, seven patients underwent a one-stage repair of proximal and distal TEF via thoracoscopy or thoracotomy. Due to missed diagnosis and other reasons, the other 7 patients underwent two-stage surgery for repair of the proximal TEF, including cervical incision and thoracoscopy. Ten of the 14 patients experienced postoperative complications including anastomotic leakage, pneumothorax, esophageal stricture, and recurrence. Patients who underwent one-stage repair of distal and proximal TEF during the neonatal period showed a higher incidence of anastomotic leak (4/7). In contrast, only one of seven patients with two-stage repair of the proximal TEF developed an anastomotic leak. Type D EA/TEF is a rare condition, and proximal TEFs are easily missed. Bronchoscopy may aim to diagnose and determine the correct surgical approach. A cervical approach may be more suitable for repairing the proximal TEF.

Esophageal atresia with tracheoesophageal fistula: A novel rare variant

Esophageal atresia (EA) is one of the most common congenital anatomical malformations that affect the alimentary tract and might be associated with the presence of tracheoesophageal fistula (TEF), and both occurs in about 1/2500 to 1/4000 live births. Depending on the anatomical description it is classifies into subtypes and there are many reported variants other than those subtypes like esophageal webs and congenital esophageal stenosis (CES). Here we report a rare variant of EA with proximal TEF which although presented clinically and radiologically as type C (EA) with (TEF) but at thoracotomy it is proved to be a different rare anatomical variant were the esophagus is found to be continuous down to the stomach with no gap resembling a type E (TEF) but there is an atretic segment just 1 cm above a (TEF). This is the first Case described in the literature. The knowledge and the anticipation of such types of variations remains the cornerstone for proper management and a high index of suspicion is needed to aid not to miss such rare anomalies.

1758 H-type Tracheoesophageal Fistula: A Rare Cause of Cough and Dysphagia in Adults

INTRODUCTION: Tracheoesophageal fistula (TEF) without associated esophageal atresia (H-type) is a rare congenital anomaly, accounting for ∼4% of esophageal malformations. It is defined as a connection between the posterior tracheal and anterior esophageal walls and is typically diagnosed before age 3. However, it can occasionally be seen in adults with chronic cough and respiratory infections. We present a 38-year-old female with a new diagnosis of H-type TEF. CASE DESCRIPTION/METHODS: A 38-year-old female (G16P12-0-3-12) with multiple prior admissions for hypoxia (presumed due to asthma and aspiration in the setting of achalasia) was admitted with HELLP syndrome. Prior pulmonary function tests showed severe airway obstruction and echocardiogram showed normal left and right ventricular systolic function. She reported coughing when supine and with meals, a history of congenital lung abnormality, severe infancy pneumonia, upper lobe resection, diagnosis of achalasia, and yearly admissions for pulmonary issues. After delivery, she was transferred to the pulmonary service for management of her hypoxia. A TEF without inflammation was noted on chest CT and confirmed by EGD and bronchoscopy. EGD showed a 3 mm proximal esophageal fistula. Bronchoscopy demonstrated a normal oropharynx and vocal cords (VC), erythematous larynx, inflamed arytenoids, a 4 mm proximal TEF without mucosal abnormalities, and right upper lobe (RUL) absence. A swallow study showed aspiration on the posterior side of the trachea presumed due to the TEF. Surgical repair rather than endoscopic clip or stent placement was determined to be the best treatment via cervical approach esophagoplasty, muscle flap reconstruction TEF repair, and laparoscopic Heller myotomy with Dor fundoplication. DISCUSSION: H-type TEF is rare and can mimic chronic lung disease due to aspiration, recurrent infection, and bronchiectasis. It must be considered in adults with recurrent respiratory infections, coughing with eating/drinking, and rarely, hemoptysis. A chest CT, bronchoscopy, esophagoscopy, and barium study are crucial to confirm the diagnosis. While endoscopic clip closure of fibrotic TEFs has been successful in 62%, it may obliterate surgical tissue planes. Surgery is the optimal treatment for H-type TEF and reported to be 75-94% successful. As H-type TEF disrupts myenteric plexus development with impaired esophageal peristalsis and lower sphincter dysfunction, a Heller myotomy and Dor fundoplication should be offered.

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A small proportion of infants with oesophageal atresia (OA) are thought to have a proximal tracheoesophageal fistula (TOF). Failure to recognize these can hamper mobilization of the upper pouch and lead to life-threatening episodes of aspiration once oral feeding starts. We reviewed our experience of upper pouch fistulae to identify characteristic features of proximal TOF.A retrospective review of TOF/OA patient notes and bronchoscopy photographs and videos, identified from our database from 01/01/2006 to 12/31/2015, was performed.Eight (6.1%) infants were identified (M:F 5:3) from a total population of 131 newly diagnosed TOF/OA infants during the period. Their median gestational age was 33 (range 28–39) weeks, and median birth weight was 1647g (range 1100–3400g). Five were initially diagnosed with pure OA and 3 with a distal TOF. All patients underwent rigid bronchoscopy at the initial surgery but only one proximal fistula was identified. The 7 missed proximal fistulae were subsequently found either during on-table oesophagograms for gap assessment (n = 2), at the time of thoracotomy when mobilizing the upper pouch (n = 3), or during subsequent bronchoscopy for symptoms post OA repair (n = 2). Two patients needed a further operation to divide the fistula. Review of the bronchoscopy videos identified four characteristic differences between upper and lower pouch fistulae. Proximal fistulae are found just distal to the vocal cords, are very small, often no more than a pit, do not open and close with ventilation, and are best identified by insufflation of the esophagus.Upper pouch fistulae are relatively easy to miss because of different characteristics compared with H-type or distal fistulae that have not previously been mentioned in the literature.Level of evidence: level IV.

Postoperative Complications and Functional Outcome after Esophageal Atresia Repair: Results from Longitudinal Single-Center Follow-Up

BackgroundEsophageal atresia (EA) and tracheoesophageal fistula (TEF) represent major therapeutic challenges, frequently associated with serious morbidities following surgical repair. The aim of this longitudinal study was to assess temporal changes in morbidity and mortality of patients with EA/TEF treated in a tertiary-level center, focusing on postoperative complications and their impact on long-term gastroesophageal function. MethodsOne hundred nine consecutive patients with EA/TEF born between 1975 and 2011 were followed for a median of 9.6 years (range, 3–27 years). Comparative statistics were used to evaluate temporal changes between an early (1975–1989) and late (1990–2011) study period. ResultsGross types of EA were A (n = 6), B (n = 5), C (n = 89), D (n = 7), and E (n = 2). Seventy (64.2%) patients had coexisting anomalies, 13 (11.9%) of whom died before EA correction was completed. In the remaining 96 infants, surgical repair was primary (n = 66) or delayed (n = 25) anastomosis, closure of TEF in EA type E (n = 2), and esophageal replacement with colon interposition (n=2) or gastric transposition (n=1). Long-gap EA was diagnosed in 23 (24.0%) cases. Postoperative mortality was 4/96 (4.2%). Overall survival increased significantly between the two study periods (42/55 vs. 50/54; P = 0.03). Sixty-nine (71.9%) patients presented postoperatively with anastomotic strictures requiring a median of 3 (range, 1–15) dilatations. Revisional surgery was required for anastomotic leakage (n = 5), recurrent TEF with (n = 1) or without (n=9) anastomotic stricture, undetected proximal TEF (n = 4), and refractory anastomotic strictures with (n = 1) or without (n = 2) fistula. Normal dietary intake was achieved in 89 (96.7%) patients, while 3 (3.3%) remained dependent on gastrostomy feedings. Manometry showed esophageal dysmotility in 78 (84.8%) infants at 1 year of age, increasing to 100% at 10-year follow-up. Fifty-six (60.9%) patients suffered from dysphagia with need for endoscopic foreign body removal in 12 (13.0%) cases. Anti-reflux medication was required in 43 (46.7%) children and 30 (32.6%) underwent fundoplication. The rate of gastroesophageal reflux increased significantly between the two study periods (29/42 vs. 44/50; P = 0.04). Twenty-two (23.9%) cases of endoscopic esophagitis and one Barrett’s esophagus were identified. ConclusionsPostoperative complications after EA/TEF repair are common and should be expertly managed to reduce the risk of long-term morbidity. Regular multidisciplinary surveillance with transitional care into adulthood is recommended in all patients with EA/TEF.

3D-CT検査で頸部気管食道瘻が術前診断されたGross D型食道閉鎖症の1例

3D-CT検査で頸部気管食道瘻が術前診断されたGross D型食道閉鎖症の1例

Characterization of the upper pouch tracheo–oesophageal fistula in oesophageal atresia

AimA small proportion of infants with oesophageal atresia (OA) are thought to have a proximal tracheoesophageal fistula (TOF). Failure to recognize these can hamper mobilization of the upper pouch and lead to life-threatening episodes of aspiration once oral feeding starts. We reviewed our experience of upper pouch fistulae to identify characteristic features of proximal TOF. MethodsA retrospective review of TOF/OA patient notes and bronchoscopy photographs and videos, identified from our database from 01/01/2006 to 12/31/2015, was performed. ResultsEight (6.1%) infants were identified (M:F 5:3) from a total population of 131 newly diagnosed TOF/OA infants during the period. Their median gestational age was 33 (range 28–39) weeks, and median birth weight was 1647g (range 1100–3400g). Five were initially diagnosed with pure OA and 3 with a distal TOF. All patients underwent rigid bronchoscopy at the initial surgery but only one proximal fistula was identified. The 7 missed proximal fistulae were subsequently found either during on-table oesophagograms for gap assessment (n=2), at the time of thoracotomy when mobilizing the upper pouch (n=3), or during subsequent bronchoscopy for symptoms post OA repair (n=2). Two patients needed a further operation to divide the fistula. Review of the bronchoscopy videos identified four characteristic differences between upper and lower pouch fistulae. Proximal fistulae are found just distal to the vocal cords, are very small, often no more than a pit, do not open and close with ventilation, and are best identified by insufflation of the esophagus. ConclusionUpper pouch fistulae are relatively easy to miss because of different characteristics compared with H-type or distal fistulae that have not previously been mentioned in the literature.Level of evidence: level IV.

Delayed Identification of Proximal Tracheoesophageal Fistula

Delayed Identification of Proximal Tracheoesophageal Fistula

Delayed diagnosis of high proximal tracheoesophageal fistula in esophageal atresia and a novel approach to the treatment of tracheomalacia by submanubrial tracheopexy

An infant with esophageal atresia (EA) had delayed diagnosis of proximal tracheoesophageal fistula (TEF) and severe tracheomalacia. We recommend bronchoscopy via laryngeal mask or rigid bronchoscopy to rule out associated TEF in infants diagnosed with esophageal atresia, as flexible bronchoscopy via endotracheal tube may not provide complete visualization of the trachea. We also describe a novel cervical approach to tracheopexy via neck incision for treatment of associated severe tracheomalacia in this infant.

Endoscopic Detection and Surgical Repair of Congenital Tracheo-Esophageal-Fistula (TEF) ± Esophageal Atresia (EA)

Purpose: This study was performed to evaluate the management of tracheoesophageal fistula (TEF) ± esophageal atresia (EA) under the guidance of preoperative tracheo-bronchoscopy (TrSc). Methods: Between 2007 and July 2014, a total of 26 consecutive newborns who underwent rigid TrSc for suspected TEF were identified. All associated charts and operation reports were retrospectively analyzed. Results: Distal TEF with EA (Gross C) predominated (n = 18). Furthermore, we managed 2 infants with proximal and distal TEF (Gross D) and 4 infants with isolated TEF (Gross E). In our hands, TrSc was feasible in infants with a birth weight above 1300 g. Twenty-five fistulas were identified by endoscopy in 23 patients. In one infant with a birth weight below 1000 g, an attempt to perform TrSc was interrupted, and urgent TEF closure was required. Fistula site at the carina was associated with a high rate of esophageal anastomosis under tension. During surgery, proximal TEF and isolated TEF were safely approached via right cervicotomy (n =5). Conclusion: This study supports the routine use of rigid TrSc at the time of surgery. Rigid TrSc allowed the surgical team to identify the number and location of TEFs, and the incidence of side effects was low.

Morbidity and mortality in esophageal atresia and tracheoesophageal fistula

Background: The outcome of newborns with esophageal atresia (EA) and tracheoesophageal fistula (TEF) has improved considerably. At present, the overall survival rates reported from developed countries are between 85 and 95%. This, however, is not the case from developing countries, where several factors contribute to higher morbidity and mortality rates. This study is an analysis of our experience with 158 patients of EA and TEF, with emphasis on factors contributing to morbidity and mortality. Patients and methods: This is a retrospective study of 158 patients with EA and TEF treated over a period of 20 years (between January 1992 and December 2011). Their records were reviewed, the study period was divided into two equal periods, and the results were analyzed accordingly. Results: A total of 158 patients (89 boys and 69 girls) with EA/TEF were treated. Their mean birth weight was 2.4 kg (700–3800 g). Their ages at diagnosis ranged from newborn to 8 months. Their gestational age ranged from 32 weeks to 41 weeks. Contrast study was used in 45 (28.5%) patients. Aspiration pneumonia was detected in 34.8% patients. The distribution of the number of patients according to the type of EA/TEF was as follows: 132 (83.5%) patients had EA with distal TEF, 14 (8.9%) had pure EA, five (3.2%) had EA with proximal and distal TEF, five (3.2%) had H-type TEF, and two (1.3%) had EA with proximal TEF. Forty-one (22.2%) patients had long-gap EA/TEF. Associated anomalies were seen in 82 (51.9%) patients, and 17 (10.8%) patients had severe anomalies. The postoperative complications included: anastomotic leak in 20 patients (12.7%), stricture in 22 (13.9%),gastroesophageal reflux in 18 (11.4%), tracheomalacia in two (1.3%), and recurrent TEF in one (0.6%). However, there was a marked decrease both in mortality and morbidity during the second period of the study. In our study, the overall mortality rate was 20.9%, but if those with severe associated anomalies were excluded, our postoperative mortality rate was 8.4%. Sepsis was the main cause of death. Conclusion: This is a relatively large series from a developing country. Although our postoperative complication rates were similar to those from developed countries, the overall mortality was higher. There was, however, a marked improvement during the second period of the study. This is attributed to a better understanding, early diagnosis, prompt referral, and better surgical techniques. Prematurity, sepsis, and associated severe malformations were the main causes of death. Once major anomalies responsible for death were excluded, our postoperative mortality rate was 8.4%. Sepsis continues to be a major cause of death, and several factors contribute to this, including prematurity and delayed diagnosis with increased incidence of aspiration pneumonia. To decrease the mortality rate, these factors should be addressed, and every effort should be made to overcome them. Patients with long-gap EA/TEF continue to be a management challenge, and every attempt should be made to preserve the native esophagus. Delayed primary repair and/or esophageal stretching are at present the preferred techniques with good long-term functional results. Keywords: Complications, Esophageal Atresia, Morbidity and Mortality, Tracheoesophageal Fistula

Use of a microporous polytetrafluoroethylene catheter balloon to treat refractory esophageal stricture: a novel technique for delivery of mitomycin C

PurposeEndoluminal application of mitomycin C shows promise as a nonsurgical approach to treating recalcitrant stricture but requires precise delivery to prevent mitomycin-mediated injury to adjacent normal mucosa. We describe a novel technique that uses a microporous polytetrafluoroethylene catheter balloon to endoluminally deliver mitomycin C to the target tissue while minimizing nontarget drug application. Materials and MethodsA newborn infant with proximal tracheoesophageal fistula and distal atresia underwent an uncomplicated repair. However, he developed recurrent esophageal stricture resistant to multiple attempts at pneumatic dilations. An image-guided endoluminal radiologic approach that uses microporous polytetrafluoroethylene catheter balloon was developed to precisely deliver mitomycin C to the mucosal lining of the stricture post-dilation. ResultsAfter uncomplicated pneumatic dilation under fluoroscopic guidance, we used a microporous balloon catheter to endoluminally deliver mitomycin C topically to the mucosa at the level of stricture. Three weeks post procedure, repeat esophagram showed resolution of the stricture with unobstructed flow of contrast material to the stomach. The patient had no observable side effects from mitomycin C application. ConclusionImage-guided therapies based on balloon dilation and drug-eluting microporous balloon techniques offer a safe, precise, and comprehensive approach to the treatment of recalcitrant esophageal strictures.

Tracheoesophageal fistula after primary repair of type C esophageal atresia in the neonatal period: recurrent or missed second congenital fistula

Purpose The aim of the study is to investigate whether a tracheoesophageal fistula (TEF) found after the primary repair of type C esophageal atresia (EA) is a recannulation of the original fistula, a missed proximal fistula, or other rare foregut malformation. Methods Between 2000 and 2009, 143 different types of patients with EA were admitted in our hospital. Seven patients (2 from our series, 5 referred to us by other hospitals with the history of primary repair of type C EA) had late presenting TEF. Esophagogram, 3-dimensional computed tomographic (CT) reconstruction, bronchoscopy, and reoperation were performed to confirm the TEF. Their medical records were reviewed and summarized. Results Persistent feeding or respiratory problems were the common symptom. The mean age of the first appearance was 17 ± 26 (1-63) months. Preoperative diagnosis was made by esophagograms and bronchoscopy in 6 patients. Reoperations were performed in all patients through thoracotomy. Missed proximal TEF shown as a distinct fistula above the primary anastomosis without much adhesion was confirmed in 5 cases. A recurrent TEF was found in 1 case. A case of communicating bronchopulmonary foregut malformation was confirmed by 3-dimensional CT reconstruction and reoperation. Conclusion A missed proximal TEF after repair of EA may be misdiagnosed as a recurrent TEF. Accurate preoperative diagnosis depends on combined evaluations of radiologic contrast study, 3-dimensional CT, and bronchoscopy.

Esophageal Atresia with Double Tracheoesophageal Fistula - A Case Report and Review of the Literature

Esophageal atresia with double tracheoesophageal fistula (TEF) is a very rare anomaly, and the accurate preoperative diagnosis of proximal TEF is very difficult. This paper describes a baby girl who presented with esophageal atresia with double, proximal, and distal TEF. The distal TEF was diagnosed before operation, whereas the proximal TEF was found intraoperatively. Overlooking the presence of proximal TEF can lead to increased morbidity and mortality due to severe respiratory infection and the necessity of a second operation. Great care must therefore be taken to not overlook the presence of proximal TEF in patients with this anomaly.

Diagnosis of a proximal tracheoesophageal fistula using three-dimensional CT scan: a case report

Missed proximal tracheoesophageal fistulas (TEF) after repair of an esophageal atresia (EA) with distal TEF are rare. The diagnosis usually is made on the basis of bronchoscopy and esophagoscopy, which can be inaccurate. Recent advances in computerized tomography (CT) have allowed saggital, coronal, and 3-dimensional reconstruction of any structure or organ. The authors report a case in which 3-dimensional CT was used to assist in the diagnosis of a missed proximal TEF in a 16-year-old girl.

Multistaged extrathoracic esophageal elongation procedure for long gap esophageal atresia: Experience with 12 patients

Purpose: This study evaluates the multistaged extrathoracic esophageal elongation procedure performed on 12 babies with long gap esophageal atresia over 15 years. Methods: Eight babies had pure esophageal atresia, 2 had proximal tracheoesophageal fistula (TEF), and 2 had distal TEF. The gaps ranged between 2 and 7 vertebral bodies. Proximal esophagostomy, TEF ligation, and gastrostomy were performed initially. The proximal esophagus is elongated 2 to 3 cm each time by translocating the esophagostomy distally along the anterior chest wall at 2- to 3-month intervals. Sham-fed milk is collected in an ostomy bag and refed via the gastrostomy. The definitive esophageal reconstruction is performed at 5 to 24 months of age. Results: Only one elongation was required in 4 babies, 2 were needed in 5, 3 in 2, and 5 in 1 patient. All patients tolerated sham feeding well. After esophageal restoration, 3 patients had minor leakage. All (12 of 12) patients had anastomotic stenosis requiring multiple dilatations, of which, 3 needed resection of stricture. Eleven patients had gastroesophageal reflux that required fundoplication. Follow-up was possible in 11 patients for 4 months to 14 years after esophageal restoration. Seven early patients are eating normally. Conclusion: Multistaged extrathoracic esophageal elongation is effective in stretching the proximal esophagus to bridge 2 to 7 vertebral bodies. J Pediatr Surg 36:1725-1727. Copyright © 2001 by W.B. Saunders Company.

Surgical management of tracheal agenesis

Tracheal agenesis is a rare congenital malformation that usually is fatal. This report describes our experience in two such cases. In both cases, the diagnosis was suspected at birth, because the patients had respiratory distress without an audible cry and were difficult to intubate. A gastrostomy and banding of the abdominal esophagus provided effective initial stabilization, in conjugation with respiratory management. The first patient also had complex cardiac malformations, and the infant died of cardiac failure 1 week after birth. The second infant, who had tracheal agenesis with a proximal tracheoesophageal fistula and a bronchoesophageal fistula, was managed successfully. At 9 months of age, a tracheotomy was performed, a long T tube was inserted to maintain the airway patency beyond the proximal tracheoesophageal fistula, and the patient was discharged. At 3 years of age, esophageal reconstruction was performed with a colonic interposition graft. The patient is thriving and developing normally at 4 years of age. Diagnosis at birth and maintenance of airway patency are essential for successful management of tracheal agenesis. Initial surgical interventions are palliative but lifesaving. Subsequent management focuses on improving the quality of life and allowing swallowing and speech.

Esophageal atresia. Prognostic factors and contribution of preoperative telescopic endoscopy.

In recent years, the surgical correction of esophageal atresia with distal tracheoesophageal fistula (TEF) has become increasingly successful. However, there remains a group of high-risk patients with specific anatomical abnormalities in whom the mortality remains appreciable. These associated disorders include cardiac, renal, and chromosomal anomalies as well as severe respiratory distress syndrome. These factors, rather than low birth weight or early gestational age, are primarily responsible for surgical mortality. Preoperative telescopic bronchoscopy has been a useful adjuvant confirming the diagnosis, identifying unusual variants, and permitting the proper anatomic placement of the endotracheal tube. A Fogarty balloon catheter can be passed bronchoscopically into the distal TEF in patients with severe respiratory distress syndrome to occlude the fistula and facilitate effective positive pressure ventilation. Thirty-two patients were treated for esophageal atresia among whom 28 had esophageal atresia with distal TEF, three had esophageal atresia alone, and one had esophageal atresia with proximal TEF. The higher-risk group comprised those with severe respiratory insufficiency as evidenced by a room air paO2 of less than 60 mmHg; this group accounted for nine of the ten deaths in the total series. There was one late death following surgical correction. In summary, in the absence of severe respiratory insufficiency or associated life-threatening congenital anomalies, the results of surgical correction for esophageal atresia are remarkably good and survival in this group approximates 100%.

Esophageal Atresia with the Proximal Tracheoesophageal Fistula (Gross's B type) : its Diagnosis and Treatment

Esophageal Atresia with the Proximal Tracheoesophageal Fistula (Gross's B type) : its Diagnosis and Treatment

Esophageal atresia with double tracheoesophageal fistula

Ninety cases of esophageal atresia (EA) with double tracheoesophageal fistula (TEF) from the literature and 4 new cases are examined. The incidence may be more common than is generally recognized with incidences up to 5.3% reported. The pre-, intra-, and postoperative diagnosis may be difficult, and half have been missed initially with almost half of these being first recognized at autopsy. During repair of EA and TEF the proximal esophagus should be mobilized looking for a proximal fistula. Many proximal TEF are missed at the initial operation and discovered in the postoperative period. The symptoms, diagnosis and treatment of the unrecognized proximal TEF are similar to that for isolated TEF. Results should be good since this problem occurs in large babies with fewer and less complex associated conditions.