Tracheoesophageal fistula (TEF) stands as a significant congenital anomaly frequently encountered in major pediatric surgical centers. Infants diagnosed with TEFs typically exhibit a range of symptoms including respiratory distress, challenges in feeding, choking, and an elevated risk of aspiration. It’s noteworthy that tracheoesophageal fistulas often present alongside other congenital anomalies, with cardiac defects being particularly common. A timely and accurate diagnosis of TEF is paramount for effective management and to ensure improved patient outcomes. Diagnostic approaches for TEF often begin with prenatal ultrasonography, which may reveal indicators such as polyhydramnios or the absence of a fetal stomach bubble. Postnatally, radiographic studies, notably chest x-rays with a nasogastric tube, play a crucial role in confirming the fistula’s presence. These diagnostic evaluations are instrumental in pinpointing the specific type of TEF, as variations in TEF anatomy can significantly influence clinical strategies and prognosis. The cornerstone of TEF management is surgical intervention aimed at closing the fistula and restoring esophageal continuity. The timing of surgical procedures is carefully determined based on the infant’s overall condition and the presence of any associated anomalies, such as cardiac defects. This resource is specifically designed for healthcare professionals to deepen their understanding and skills in the diagnosis and management of TEFs. Participants will gain insights into the pathogenesis, clinical presentations, and evidence-based diagnostic and management protocols for this condition. Enhanced expertise will empower healthcare providers to function effectively within interprofessional teams, thereby optimizing care and improving outcomes for patients with TEFs.
Objectives:
- Recognize the typical clinical presentation of infants with a tracheoesophageal fistula.
- Determine the most appropriate diagnostic methods for evaluating suspected tracheoesophageal fistula cases.
- Assess evidence-based management strategies for patients diagnosed with a tracheoesophageal fistula.
- Formulate strategies for effective interprofessional team collaboration to enhance treatment efficacy and outcomes for patients with tracheoesophageal fistulas.
Introduction
Tracheoesophageal fistula (TEF) is recognized as one of the prevalent congenital anomalies seen in major pediatric surgical centers. Infants with TEF commonly present with classic symptoms such as respiratory distress, feeding difficulties, choking episodes, and a significant risk of aspiration. The occurrence of TEF is frequently associated with other congenital anomalies, notably cardiac defects. Esophageal atresia, another congenital malformation, shares similar clinical presentations with TEF and can occur either in conjunction with or independently of a fistula. Understanding the nuances of Te Fistula Diagnosis is critical in these cases.
Etiology
While the precise mechanisms leading to the separation of the primitive trachea and esophagus are not fully elucidated, the prevailing hypothesis suggests that a defect in the lateral septation of the foregut, which divides into the trachea and esophagus, is the primary cause of TEF and esophageal atresia. The trachea and esophagus originate from a common primitive foregut. Around the fourth week of gestation, epithelial ridges begin to separate the developing respiratory and gastrointestinal tracts. The foregut subsequently divides into a ventral respiratory tract and a dorsal esophageal tract. It is believed that the fistula tract arises from an embryonic lung bud that fails to undergo proper branching. These disruptions in mesenchymal proliferation are thought to result in TEF formation.[1] Accurate te fistula diagnosis is the first step in addressing this developmental issue.
The VACTERL complex is a well-recognized association of congenital anomalies, encompassing vertebral defects (V), anal or gastrointestinal tract atresia (A), congenital cardiac defects (C), tracheoesophageal defects (TE), renal and distal urinary tract anomalies (R), and limb lesions (L). The Sonic Hedgehog (SHH) gene, which codes for an intracellular signaling molecule, has been implicated in esophageal atresia. Research has indicated that mice with deficiencies in the regulation of the SHH gene exhibit VACTERL-type anomalies.[2][3] Therefore, understanding genetic factors is becoming increasingly relevant in the context of te fistula diagnosis and related conditions.
Studies in rats have also utilized the Adriamycin-induced TEF model. Adriamycin, an anthracycline antibiotic, affects deoxyribonucleic acid integrity and synthesis. Introducing adriamycin into the peritoneal cavity of pregnant rats has resulted in a 40% to 90% incidence of esophageal atresia and TEF in developing embryos. Findings from these studies demonstrate that TEF and esophageal atresia can develop in rats, mirroring the condition in neonates, and VACTERL anomalies can also arise in rats.[4] Gli-2, a downstream signaling molecule for SHH, has been investigated in animals with TEF and control subjects. It was found that Gli-2 messenger ribonucleic acid was reduced in the fistula tract compared to the adjacent esophagus.[5] These findings underscore the complexity of the etiology and the importance of thorough te fistula diagnosis to understand the underlying mechanisms.
Epidemiology
Genetic factors appear to have a limited role in the pathogenesis of TEF. The concordance rate among twins is reported to be 2.5%.[6] The incidence of TEF is approximately 1 in 3500 live births.[7][8] Esophageal atresia and TEF are classified based on their anatomic configuration.[9] Type C, characterized by a proximal esophageal pouch and a distal TEF, is the most common, accounting for about 84% of cases. TEF without esophageal atresia, known as H-type fistula, occurs in only 4% of cases (refer to Image. Types of Tracheoesophageal Fistulas). [10] Depaepe and colleagues have observed a decreasing trend in the birth frequency of TEF across different European regions.[11] The occurrence of TEF does not show a correlation with maternal age, provided chromosomal anomalies are excluded. The reported rates of isolated TEF versus TEF associated with other congenital anomalies vary between 38.7% and 57.3%.[12] The incidence of trisomies and other chromosomal abnormalities in conjunction with TEF ranges from 6% to 10%, excluding neonates younger than 28 weeks of gestation. Though less frequent, trisomy 18 is more often associated with TEF than trisomy 21. Feingold syndrome, characterized by microcephaly, micrognathia, and digital anomalies, may also present with associated TEF or esophageal atresia. In 1962, Waterston and colleagues developed a risk stratification system for patients with TEF based on factors such as birth weight, presence of pneumonia, and associated anomalies.[13] Subsequently, Spitz and associates proposed a simplified system based on the presence of congenital heart defects and low birth weight.[14] Current survival rates for babies weighing less than 1500 g without significant cardiac anomalies approach 97%, but this rate decreases significantly to 22% if low birth weight is combined with cardiac anomalies. Acute morbidity and mortality are primarily attributed to cardiac and chromosomal defects, while late mortality is often due to ongoing respiratory complications.[15] These epidemiological factors highlight the importance of early te fistula diagnosis and risk assessment.
Pathophysiology
TEF arises from the abnormal septation of the caudal foregut during the fourth and fifth weeks of embryonic development. Under normal developmental conditions, the trachea forms as a diverticulum of the foregut and develops a complete septum that separates it from the esophagus. Fistula formation in conjunction with esophageal atresia occurs due to an abnormal posterior positioning of the tracheoesophageal septum, which results in a persistent connection between the trachea and esophagus. Isolated esophageal atresia without TEF typically occurs when the esophagus fails to recanalize during the 8th week of embryonic development.[16] Understanding these embryological processes is crucial for comprehending the variations in te fistula diagnosis and presentation.
History and Physical
Keckler and colleagues reported that in their series, TEF was most commonly associated with congenital heart disease, occurring in 32.1% of patients, excluding infants with patent foramen ovale and patent ductus arteriosus.[17] Ventricular septal defect was the most frequent cardiac anomaly, present in 22.3% of patients, often in combination with multiple cardiac defects. Isolated ventricular septal defect occurred in only 7.1%. Cyanotic heart disease was less common, observed in 4.5% of patients, and all of these cases involved tetralogy of Fallot. The second most common congenital defect associated with TEF was vertebral anomalies, seen in 24% of patients. Vertebral defects are often accompanied by other axial spine complications, such as rib anomalies and tethered cord. Axial spine abnormalities were associated with a higher rate of anastomotic leak, potentially due to increased tension at the surgical site. It is hypothesized that a wider gap may exist between the two ends of the esophagus because of a higher proximal pouch in patients with skeletal deformities.[18] These associations are important to consider during te fistula diagnosis, as they can influence management and prognosis.
The clinical presentation of TEF varies depending on the presence of esophageal atresia. Polyhydramnios is observed in approximately two-thirds of pregnancies, although many cases are not detected prenatally.[19] Newborns with esophageal atresia typically present immediately after birth with excessive secretions leading to drooling, choking, respiratory distress, and inability to feed. Gastric distension is a common complication of fistula between the trachea and distal esophagus. Reflux of gastric contents through the fistula tract can lead to aspiration pneumonia and increased morbidity. Patients with H-type TEFs may present early if the defect is significant, primarily with coughing and choking associated with aspiration of feeds through the fistula. However, some studies indicate that minor H-type defects may not be symptomatic in the newborn period, and diagnosis can be delayed from 26 days to 4 years.[20] These patients may have a prolonged history of mild respiratory distress associated with feeding or recurrent episodes of pneumonia. In rare instances, the diagnosis may be delayed into adulthood.[21] A high index of suspicion and thorough evaluation are crucial for timely te fistula diagnosis across different presentations.
Evaluation
Prenatal suspicion of TEF may arise from maternal polyhydramnios and the absence of the fetal stomach bubble on ultrasound. A study by Stringer and colleagues reported that prenatal scans diagnosing esophageal atresia had a sensitivity of 42% with a positive predictive value of 56%.[22] Karyotyping can be valuable in the prenatal assessment of suspected esophageal atresia due to the increased incidence of trisomy 18 in affected infants. Ultrasound imaging may also detect cardiac defects, which can indicate a poorer fetal prognosis.[23] If esophageal atresia or TEF is suspected prenatally, delivery should be planned at a medical center equipped with a neonatal intensive care unit and an operating room. Esophageal atresia and TEF are frequently associated with the VACTERL sequence, CHARGE syndrome (coloboma of the eye, heart defects, atresia of the choanae, retarded development, genital hypoplasia, and ear abnormalities), trisomies 18 and 21, and DiGeorge syndrome. An increased incidence of anomalies is associated with up to 65% of cases of pure esophageal atresia. The recurrence risk in subsequent pregnancies for non-syndromic esophageal atresia or TEF is approximately 1%.[24] Prenatal indicators can be the first step in te fistula diagnosis, prompting further postnatal evaluation.
Postnatally, esophageal atresia may be initially suspected when an orogastric or nasogastric catheter cannot be advanced beyond 10 to 15 cm into the esophagus. This finding can be confirmed with an anterior-posterior chest radiograph showing the catheter coiled in the upper esophageal pouch. A distal TEF may be visualized on a lateral chest radiograph. Both views typically reveal a gas-filled gastrointestinal tract. Water-soluble contrast can be carefully instilled into the esophageal pouch under fluoroscopic guidance to evaluate for TEF.[25] Barium contrast should be avoided due to the risk of pneumonitis if aspirated. Contrast material must be promptly removed to prevent regurgitation and aspiration. For the te fistula diagnosis of isolated TEF, an upper gastrointestinal series using thickened water-soluble contrast material is recommended. The distal esophagus is filled first, and then the catheter is withdrawn in a cephalad direction. Contrast swallow radiography can also be helpful, but the fistulous tract might be challenging to visualize in these studies.[26] Esophageal endoscopy and bronchoscopy are valuable tools for directly detecting the TEF. Methylene blue can be injected into the trachea, and the fistula can be identified by its appearance in the esophagus. Three-dimensional computed tomography (CT) scanning has also been successfully used for te fistula diagnosis.[27]
Treatment / Management
The first successful primary surgical repair of TEF was performed by Dr. Cameron Haight in 1941.[28] With pediatric surgical centers now reporting survival rates exceeding 90% for patients with TEF, current focus is on minimizing morbidity and improving patients’ quality of life. Open surgical repair of TEF and esophageal atresia typically involves a right posterolateral thoracotomy, fistula ligation, and primary esophageal anastomosis. Preoperative echocardiography is essential to rule out a right-sided aortic arch, which occurs in about 2.5% of cases. A right-sided aortic arch increases surgical complexity and often necessitates a left thoracotomy.[29] Renal ultrasound, spinal ultrasound, and limb radiographs may be performed to exclude other VACTERL anomalies.
Complications associated with primary repair include anastomotic leak, recurrent laryngeal nerve injury leading to vocal cord paralysis, esophageal stricture, persistent upper pouch fistula, recurrent fistula, and, in severe cases, death.[30] Spontaneous closure of a recurrent TEF is exceedingly rare.[31] Primary anastomosis may not be feasible when the gap between the upper and lower esophageal segments is greater than 2 vertebral bodies. In such instances, surgical options include Livaditis myotomy, mobilization of the distal esophageal segment to the diaphragmatic hiatus, and the Foker technique. Esophageal anastomosis performed under tension increases the risk of leak, stricture, and reflux disease.[32] Six decades after the first successful primary repair, Dr. Tom Lobe and Dr. Steve Rothenberg performed the first minimally invasive thoracoscopic TEF repair.[33] Minimally invasive techniques should be reserved for advanced pediatric surgical centers and have not been proven to reduce the risk of strictures and anastomotic leaks.[34] Thoracoscopic surgery offers excellent visualization of anatomical structures and can reduce morbidity by avoiding open thoracotomy. Avoiding open surgical repair also prevents potential chest wall deformity, scoliosis, rib fusion, muscle contractures, and chronic pain.[35] Successful te fistula diagnosis is crucial for guiding appropriate and timely surgical intervention.
Immediate surgical management often includes creating a gastrostomy for feeding and continuous suctioning of the blind esophageal pouch to prevent aspiration. Reconstruction options include primary repair using the native esophagus or replacement procedures using parts of the stomach or large intestine. Preserving the native esophagus is preferred, as replacement procedures increase the risk of recurrent aspiration and chronic respiratory complications. A staged procedure can be considered as the infant grows and the esophagus elongates if primary repair is not initially feasible.[36]
Mechanical elongation of the esophageal segment can be attempted using techniques like bougienage, electromagnetic stimulation, and graded tension applied to the disconnected esophageal segments with traction sutures, although the efficacy of these methods remains unproven.[37] A staged approach has improved outcomes in low birth weight infants. H-type fistula repair is typically performed via a cervical neck dissection to expose and repair the fistula. This procedure carries risks of recurrent laryngeal nerve injury and operative trauma.[38] The neodymium-doped yttrium aluminum garnet (Nd:YAG) laser has been used for H-type fistula repair, but experience with this technique is limited.[39]
Meier and colleagues [40] have described endoscopic repair of TEF using tissue adhesive (Histoacryl) and fibrin adhesive (TisseelTM), with success rates of 48% and 55%, respectively. In the tissue adhesive group, five patients also received a sclerosing agent (polidocanol or aethoxysklerol) during endoscopic repair, achieving a 100% success rate. Morbidity from endoscopic repair is minimal to none.[41] Hoelzer and colleagues also reported successful closure of recurrent TEFs using bronchoscopic application of fibrin glue, which promotes rapid granulation tissue formation and epithelialization.[42] Endoscopic repair of recurrent TEF was first reported in the 1970s using tissue adhesive (Histoacryl), where multiple attempts led to successful fistula closure.[43] A rigid bronchoscope is the preferred device for delivering the obliterating agent. Rod-lens telescopes are particularly useful for diagnosing H-type fistulas.
Laryngoscopy and bronchoscopy should be performed on all infants before open surgical repair of TEF or esophageal atresia. These procedures help identify the fistula level and detect tracheomalacia and tracheobronchitis prior to primary repair. Bronchoscopy can also reveal laryngeal abnormalities, including posterior laryngeal cleft, laryngomalacia, vocal cord dysfunction, the position of the aortic arch, and other fistulas.[44] Bronchoscopic findings aid in surgical planning. Carinal fistulas are associated with wide-gap atresia, while midtracheal fistulas are linked to minimal-gap atresia. Given the high prevalence of gastroesophageal reflux disease (GERD) post-repair, expert guidelines recommend routine treatment with a proton pump inhibitor (PPI) for at least one year post-repair and longer for those with ongoing GERD. Infants with TEF are also at increased risk for chronic feeding difficulties. GERD often persists and is associated with Barrett esophagitis. Expert panels recommend lifelong monitoring for pulmonary and gastrointestinal complications in children who have undergone TEF repair.[45] Effective management strategies are built upon accurate te fistula diagnosis and thorough preoperative assessment.
Differential Diagnosis
The differential diagnosis for TEF and esophageal atresia includes conditions such as esophageal stricture or diverticulum, pharyngeal pseudodiverticulum, severe GERD, vascular ring, iatrogenic esophageal perforation, laryngotracheoesophageal cleft, esophageal webs, esophageal duplication, congenital shortened esophagus, and tracheal agenesis or atresia. A comprehensive approach to te fistula diagnosis requires considering and excluding these alternative conditions.
Prognosis
The prognosis for isolated TEF is generally favorable.[46] However, infants with TEF and esophageal atresia have a more guarded prognosis, largely dependent on the presence of associated abnormalities. One study reported an 87% survival rate for patients with esophageal atresia or esophageal atresia with TEF, although 61% of early deaths were linked to cardiac and chromosomal anomalies.[47][48] Mortality rates for esophageal atresia and TEF were significantly higher in infants with associated cardiac disease (42% versus 12% in those without). Very low birth weight has also been identified as a significant factor in reduced patient survival.[49] The gap length in esophageal atresia can also influence prognosis.[50] Children with esophageal atresia and TEF often exhibit growth deficiencies. Poor in-utero development is common, with nearly one-third having birth weights below the fifth percentile.[51] Little and colleagues reported that up to 50% of children had weights below the 25th percentile in the first 5 years of life.[52] This trend is primarily attributed to significant respiratory and gastrointestinal morbidity associated with TEF. Long-term height and weight outcomes generally improve as these children age.[53] Feeding abnormalities are a significant cause of morbidity in early childhood. Some patients develop aversive feeding behaviors, refusing oral intake due to GERD, anastomotic strictures, and esophageal dysmotility. These children may require gastrostomy tube placement for nutritional support. Feeding aversion is more common in children with isolated esophageal atresia because they are often exclusively fed via gastrostomy in the initial months of life. Early and accurate te fistula diagnosis and management contribute significantly to long-term prognosis.
Respiratory complications are also frequently observed in children with TEF and esophageal atresia. Severe tracheomalacia and bronchomalacia occur in 10% to 20% of infants. Airway reactivity and instability can lead to life-threatening airway obstruction.[54] A subset of infants may require aortopexy for tracheal stabilization and weaning from mechanical ventilation. Children may exhibit a characteristic harsh, barking cough, indicative of iatrogenic airway issues. Recurrent bronchitis and pneumonia are common, affecting up to two-thirds of TEF patients in the first few years of life.[55] Untreated recurrent infections or frequent aspiration can result in irreversible lung damage, including bronchiectasis and persistent atelectasis. Wheezing is common in up to 40% of survivors and may persist with age. Recurrent respiratory symptoms are often caused by abnormal airway epithelium, which impairs mucociliary clearance. The severity of GERD further increases the risk of esophageal strictures and dysmotility, exacerbating aspiration risk. Recurrent TEF, though rare, should also be considered in cases of persistent or worsening symptoms. Respiratory morbidity generally decreases in frequency and severity as children reach late adolescence.[55] Hyperinflation of the lungs, reduced lung volumes, and overall abnormal pulmonary function are common in up to 40% of survivors, but these may not significantly impact daily activities. Management of pulmonary pathology includes tailored antibiotic use, physiotherapy, and GERD treatment to prevent aspiration. Inhaled bronchodilators and steroids are used to manage asthmatic symptoms. Serial pulmonary function tests and chest CT scans are valuable for monitoring patient progress.[56] Long-term follow-up is essential after te fistula diagnosis and treatment to manage potential respiratory complications.
Complications
Common complications following esophageal atresia and TEF repair, as seen in a series of 227 cases, include anastomotic leak (16%), esophageal stricture (35%), and recurrent fistulae (3%). Esophageal strictures are often successfully managed with endoscopic balloon dilation.[57] Tracheomalacia occurred in 15% of cases, with 40% of these requiring surgical repair. Disturbed peristalsis and delayed gastric emptying are typical and contribute to GERD and aspiration. Strictures at the anastomotic site are an early complication requiring dilatation in nearly half of all patients.[58] A small number of patients may require resection of the strictured esophageal segment. GERD can significantly increase the risk of stricture formation, and fundoplication may be beneficial. Esophageal dysmotility is a common finding, visualized on manometry in 75% to 100% of children post-primary repair.[59] Patients often experience dysphagia, obstruction by food particles, failure to thrive, and choking.[60] Dietary modifications, such as avoiding certain foods and frequent drinking during meals, can be helpful.
Open thoracotomy can lead to significant musculoskeletal morbidity. Vertebral defects associated with the VACTERL sequence can contribute to chest wall or spinal deformities. One study noted that 24% of patients had winged scapula due to latissimus dorsi muscle paralysis, while 20% exhibited chest wall asymmetry secondary to serratus anterior muscle atrophy.[61] Female infants may experience breast asymmetry and disfigurement.[62] Modified axillary incisions, as described by Bianchi and colleagues, or thoracoscopic techniques may reduce morbidity.[63] Motility disorders and respiratory function abnormalities are common after esophageal atresia and TEF repair and necessitate ongoing monitoring. A systematic review of long-term outcomes in adulthood following esophageal atresia repair in infancy reported the following pooled estimated prevalences:
- Dysphagia: 50.3%
- GERD with esophagitis: 40.2%
- GERD without esophagitis: 56.5%
- Respiratory tract infections: 24.1%
- Asthma: 22.3%
- Wheeze: 34.7%
- Persistent cough: 14.6%
- Barrett esophagus: 6.4%
- Squamous cell esophageal cancer: 1.4%
The prevalence of Barrett esophagus in adulthood was four times higher than in the general population, and it is a recognized risk factor for esophageal cancer.[64] The risk of esophageal cancer is approximately 50 times higher than in the general population over 40 years of age.[65] Long-term complications underscore the need for ongoing surveillance after te fistula diagnosis and treatment.
Postoperative and Rehabilitation Care
Postoperative laryngoscopy and bronchoscopy are important to assess the severity of tracheomalacia and to identify any missed or recurrent fistulae.[66] These follow-up procedures are crucial for managing potential complications detected post te fistula diagnosis and repair.
Enhancing Healthcare Team Outcomes
Persistent respiratory and gastrointestinal complications are common in patients who have undergone TEF repair. Long-term management focuses on early detection and aggressive management of these complications, led by an interprofessional team including pediatric surgeons, pulmonologists, gastroenterologists, otolaryngologists, and neonatal intensive care unit nurses.[67] Patients and families should be educated about these health risks and the importance of ongoing clinical and endoscopic surveillance into adulthood. Specific recommendations, primarily based on expert opinion, have been made by a panel:
- Routine monitoring for symptoms of GERD, dysphagia, and aspiration should be conducted in all children and adults.
- At least three surveillance endoscopies should be performed during childhood to detect early esophagitis. Routine endoscopic surveillance should continue into adulthood, every 5 to 10 years.
- Patients with GERD or dysphagia symptoms should undergo esophageal contrast studies and endoscopy. Esophagitis should be aggressively managed with PPIs, and esophageal strictures should be treated with dilation and PPIs. Asymptomatic patients do not require routine screening or dilation for strictures.
- Respiratory symptoms should be carefully evaluated to rule out anastomotic stricture, laryngeal cleft, vocal cord paralysis, congenital esophageal stenosis, recurrent fistula, or congenital vascular malformations. Acid suppression alone is unlikely to improve respiratory symptoms and may increase the risk of respiratory infection.
- Fundoplication has a limited role in patients with TEF due to underlying esophageal dysmotility, which predisposes them to post-fundoplication complications, including esophageal stasis and aspiration. However, fundoplication may be considered in patients with poorly controlled GERD despite maximal PPI therapy, long-term dependency on transpyloric feeding, or cyanotic spells due to GERD. Eosinophilic esophagitis should be excluded before considering fundoplication.
Effective management of TEF requires a well-coordinated interprofessional team to ensure comprehensive care and optimal patient outcomes. This collaborative approach ensures that all facets of the patient’s condition are addressed, from initial te fistula diagnosis and surgery to postoperative care and long-term follow-up, ultimately enhancing prognosis and quality of life for children with this condition.
Review Questions
Figure
Types of Tracheoesophageal Fistulas. This image illustrates the different subtypes of tracheoesophageal fistulas, aiding in understanding the variations in te fistula diagnosis. Source: Free-Ed.Net & The US Army
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Disclosure: Irim Salik declares no relevant financial relationships with ineligible companies.
Disclosure: Manju Paul declares no relevant financial relationships with ineligible companies.
