Which of the following interventions would be needed for a neonate diagnosed with a tracheoesophageal fistula?

The management of malignant TEF is aimed at palliation. As opposed to benign TEF, where surgical interventions restore the integrity and continuity of the airway and intestine, malignant fistulas represent the sequelae of advanced metastatic esophageal or bronchogenic carcinomas. Surgery, in many of these cases, is not within the initial oncologic principles of management. Furthermore, these patients are often significantly deconditioned and malnourished as a result of their disease burden, systemic chemotherapy, and local radiation. For these reasons, outcomes from radical and palliative surgery to address malignant TEF result in significant rates of morbidity and mortality.

In cases of incurable disease, airway and esophageal stenting has emerged as a viable option to achieve palliation. Either individual or a combination of stents across the fistula is placed to prevent further spillage of enteric contents across the fistula, thus minimizing pulmonary contamination and alleviating the symptoms of chronic aspiration and pulmonary sepsis.

The principals of preoperative planning in benign TEF also apply to patients with malignant disease. Preoperative imaging, including barium swallow, should be obtained to determine the anatomy and level of the fistula. Pulmonary sepsis should be treated with antibiotics, mechanical ventilation should be weaned as allowed, and nutrition should be optimized.

Most malignant TEF may be sealed using a self-expanding covered or partially covered metal or plastic esophageal stents. These stents are typically placed under direct endoscopic guidance with the assistance of bedside fluoroscopy. In some cases, tracheal stents may also be needed and should be placed under bronchoscopic vision before the esophageal stent. This technique decreases the incidence of tracheal narrowing from initial esophageal stenting.

For fistulas located at or above the cricopharyngeus, the placement of a stent is not recommended because a foreign body in the pharynx or larynx results in significant patient discomfort and the inability to swallow. In such cases, a definitive tracheostomy tube with inflatable cuff to protect the distal airways may be placed. Likewise, fistulas to the lobar or segmental bronchi are difficult to seal via bronchial stenting because there is a lack of healthy tissue proximally and distally. Typically treated with an esophageal stent alone, these peripheral fistulas may also be managed with the addition of a tracheobronchial stent.

The placement of self-expanding stents, whether in the esophagus or airway, can be safely accomplished with minimal intravenous sedation and the use of topical or nebulized anesthetic. Although well tolerated, patients should be counseled on the complications of esophageal and airway stenting, from minor to devastating. This includes bleeding, migration, erosion, and further progression of the fistula, leak, and occlusion.

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Tracheoesophageal Fistula

Christopher R. Morse MD, ... Douglas J. Mathisen MD, in Medical Management of the Thoracic Surgery Patient, 2010

Endoscopy (Bronchoscopy and Esophagoscopy)

Essential in the diagnosis and management of tracheoesophageal fistula

Bronchoscopy most valuable to locate fistula and determine extent

In ventilated patients, flexible bronchoscopy can be performed through endotracheal tube (tube pulled back under direct vision) (Fig. 12-3)

Rigid bronchoscopy provides complete inspection of airway

Measurements should be taken of fistula and remaining normal airway (distance from the vocal cords to the TEF, diameter of the TEF, and the distance from the TEF to the carina). Documention of the measurements in the bronchoscopy note are important for future comparison and for evaluation of possible operative intervention (resection or stenting).

Biopsies of membranous wall of the trachea should be performed if malignancy is suspected.

Entire tracheobronchial tree to be examined and cultures sent to guide antibiotic therapy

Tricks to identify the TEF: simultaneous bronchoscopy/esophagoscopy instill air, methylene blue

Esophagoscopy may reveal fistula, but less reliable than bronchoscopy

Difficult with smaller fistulas

Bulky tumor in esophagus may make identification complex

Essential in guiding any endoluminal interventions

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Pediatric Anesthesia

Michael A. Gropper MD, PhD, in Miller's Anesthesia, 2020

Tracheoesophageal Fistula

A tracheoesophageal fistula can have five or more configurations, most of which are diagnosed after an inability to swallow because of an associated esophageal atresia (the esophagus ends in a blind pouch). In these cases the characteristic diagnostic test is an inability to pass a suction catheter into the stomach. Neonates may have aspiration pneumonitis from a distal fistula connecting the stomach to the trachea through the esophagus or from a proximal connection of the esophagus with the trachea. Neonates with the rarer H-type fistulae have a fistula between esophagus and trachea; however the esophagus is patent with no atresia. These children present later, typically with respiratory distress and chest infections.

The tracheoesophageal fistula may be part of a larger constellation of anomalies known as the VATER association (V,vertebral;A, anal;TE, tracheoesophageal;R, renal) or the VACTERL association (VATER andC, cardiac; andL, limb). Any child with a tracheoesophageal fistula or esophageal atresia should be suspected of having the other anomalies. An echocardiogram to examine for a right-sided aortic arch and the presence of congenital heart disease should be performed before anesthesia.257 Abdominal ultrasound should also be performed to detect major renal abnormalities.

The major anesthetic issues include: (1) already compromised respiratory function due to aspiration pneumonia; (2) overdistention of the stomach from entry of air directly into the stomach through the fistula, particularly after administration of positive pressure ventilation by mask; (3) inability to ventilate the child’s lungs because of the large size of the fistula; (4) problems associated with other anomalies, particularly a patent ductus arteriosus and other forms of congenital heart disease.258 Prior to anesthesia, the infant’s feedings should be withheld, a catheter placed in the esophagus to drain saliva, and the infant placed prone in a head-up position. Anesthesia evaluation centers on the pulmonary and cardiovascular systems.

A major aim of anesthesia is to ensure adequate ventilation despite the presence of the fistula. Since positive pressure ventilation may inflate the stomach through the fistula and cause distension of the stomach, it should be avoided until an endotracheal tube is placed distal to the fistula and/or the fistula is occluded or ligated. The risk of abdominal distension and hypoventilation is greatest when the fistula is large or the lung compliance is poor. The distended stomach will further compromise ventilation of the lungs, exacerbating the situation. Several different strategies for anesthesia have been described.259 Usually an inhalational induction is preferred and spontaneous ventilation is maintained until the fistula is ligated. This is not always possible. Coordination with the surgeon is critical to defining the optimal way to ensure adequate ventilation until the fistula is occluded. Bronchoscopy is usually performed after induction to assess the size and location of the fistula. At bronchoscopy a Fogarty catheter or similar device may be placed directly in the fistula to occlude it. The endotracheal tube is ideally placed in the trachea distal to the origin of the fistula. This may be done blindly by advancing the tube into a main bronchus and then carefully pulling it back until equal air entry is heard. The endotracheal tube may be inadvertently placed into the fistula resulting in rapid gastric distension and arterial oxygen desaturation. If this occurs the tube should be withdrawn. Urgent transcutaneous gastric decompression may be needed or intraabdominal clamping of the distal esophagus through an abdominal incision.

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Esophagus

Courtney M. Townsend JR., MD, in Sabiston Textbook of Surgery, 2022

Foreign Body Ingestion, Benign Tracheoesophageal Fistula, and Schatzki Ring

The patient with foreign body ingestion can require technical expertise to prevent iatrogenic perforation. If the object is lodged in the esophagus, careful endoscopy under general anesthesia is preferred. Forceful pushing to move the object into the stomach can result in perforation. Full relaxation, lubrication with water, and gentle pressure can sometimes be enough. Bringing the object proximally requires special large endoscopic graspers, nets, or lassoes along with patience and full visualization as the object is removed to prevent injury in the esophagus and oropharynx. Over-tubes are frequently useful in this setting, as is rigid esophagoscopy. If the object is not retrievable, laparoscopy or laparotomy with gastrotomy may be necessary. Evaluation of the full gastrointestinal tract is recommended with radiographs and CT scan before an intervention. For patients with repeat foreign body ingestions, or those ingesting objects for the purpose of self-harm, inpatient psychiatric evaluation is warranted.

Benign tracheoesophageal fistula can be seen in patients with multiple procedures or foreign bodies in the upper mediastinum. A classic example of benign tracheoesophageal fistula is in the patient with endotracheal tube (or tracheostomy) and nasogastric tube. It is manifested most commonly with recurrent or persistent respiratory infection and bilious or salivary contents emanating from the tracheostomy. CT scan and barium swallow can be helpful in determining the diagnosis. Further evaluation is done with bronchoscopy and endoscopy, ensuring that bronchoscopy is performed such that the entire airway is evaluated. The tracheostomy balloon will have to be deflated and usually temporarily removed during the evaluation for visualization. If tracheoesophageal fistula is identified, treatment principles are (1) discontinuation of the causative agent, (2) consideration of exclusion of the fistula by stent or diversion, and finally (3) repair or delayed healing. In a stable patient, definitive repair may preclude the need for temporary exclusion or diversion. If the fistula was caused by a tracheostomy balloon, a longer or cuffless tracheostomy will be required. Antibiotics are usually employed as well. Enteral access and gastric decompression can be achieved with gastrostomy and jejunostomy tubes. Repair can be undertaken when the patient is medically suitable by either thoracotomy or cervical approach with resection of the fistula, possible primary repair or resection, and vascularized tissue interposition. Attempts at definitive repair in a compromised patient are not optimal. Delayed healing can occur if the offending agents are removed and diversion is successful. Esophageal stents can occasionally be used in this setting as well, although this must be determined on a case-by-case basis. Placement of simultaneous esophageal and airway stents (“kissing stents”) is typically reserved for only the most moribund patients as they have the potential to worsen the size of the fistula due to radial forces. EVT has also been described in the management of tracheoesophageal fistula, though clinical experience with this application is still relatively new.

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Pulmonary Manifestations of Genetic Diseases

Beth A. Pletcher, in Pulmonary Manifestations of Pediatric Diseases, 2009

Tracheoesophageal Fistula and Tracheal Agenesis

TEF is a common congenital anomaly of the respiratory tract, with an incidence of about 1 in 3,500 live births. TEF typically occurs with esophageal atresia. Esophageal atresia and TEF are classified according to their anatomic configuration. Type A consists of a proximal esophageal blind pouch, with the distal esophagus connecting directly to the trachea, and accounts for approximately 85% of cases. TEF occurs without esophageal atresia (H-type fistula) in only 5% of cases. These defects result from failure of the tracheoesophageal ridges to fuse fully during early embryonic life, with incomplete separation of the esophagus from the trachea.1 One third of infants born with a TEF have additional birth defects. TEFs may be seen as part of multiple anomaly associations and in numerous mendelian disorders (Table 14-9).

The clinical presentation of TEF depends on the presence or absence of esophageal atresia. In cases with esophageal atresia (95%), polyhydramnios occurs in approximately two thirds of pregnancies. Infants with esophageal atresia become symptomatic immediately after birth, with excessive secretions resulting in drooling, choking, respiratory distress, and the inability to feed. A fistula between the trachea and distal esophagus often leads to gastric distention and reflux of gastric contents through the TEF resulting in aspiration pneumonia. Children with H-type TEFs may present early if the defect is large, with coughing and choking associated with feeding as the milk is aspirated through the fistula. Smaller defects of this type may not be symptomatic in the newborn period. These patients typically have a prolonged history of mild respiratory distress associated with feedings or recurrent pneumonias. Treatment consists of surgical ligation of the fistula; patients with esophageal atresia require esophageal anastomosis.

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Neonatal Respiratory Disease

Waleed Maamoun, ... James J. Cummings, in Pediatric Critical Care (Fourth Edition), 2011

Tracheoesophageal Fistula

Tracheoesophageal fistula (TEF) occurs in approximately 1 in 4500 live births, making it one of the most common congenital malformations. Usually isolated, it can be associated with other anomalies including complex syndromes like VATER (vertebral defects, anal atresia, TEF, esophageal atresia, and renal anomalies), VACTERL (VATER plus cardiac and upper limb defects), and CHARGE (coloboma, heart defect, choanal atresia, mental retardation, genital hypoplasia, ear anomalies, and deafness). In the absence of a more generalized syndrome, TEF is also associated with isolated cardiac defects, which may present in up to 50% of cases. Whether as part of a syndrome or an isolated anomaly, TEF usually is associated with esophageal atresia; however, in 5% to 7% of cases there is no associated esophageal atresia (H-type TEF).

TEF and an associated esophageal atresia and TEF as part of a more general disorder invariably present in the immediate newborn period; infants with an isolated H-type fistula can remain clinically silent for many weeks or even months. Although relatively rare (approximately 1 in 100,000 live births), an H-type fistula must be suspected in any infant who coughs during feedings and has recurrent pneumonitis. In preterm infants it can present as apnea spells with no other signs. The diagnosis of an H-type fistula can be difficult but is best found by a cine esophagram; in cases in which this study is inconclusive, bronchoscopy may be necessary. Successful repair and preservation of pulmonary function depends on early diagnosis.

What is the treatment for tracheoesophageal fistula?

How is tracheoesophageal fistula treated? Tracheoesophageal fistula treatment involves corrective surgery. During this procedure, the connection between your baby's esophagus and trachea is repaired. This may be done using traditional or minimally invasive techniques.

Which of the following is a nursing interventions for a child with tracheoesophageal atresia?

Nursing interventions for a child with tracheoesophageal atresia are: Ensure safe swallowing. Place suction equipment at the bedside, and suction as needed; ensure proper nutrition by consulting with physician for enteral feedings, preferably a PEG tube in most cases. Prevent aspiration.

How will esophageal atresia with tracheoesophageal fistula affect an infant?

Almost 90 percent of babies born with esophageal atresia also have a tracheoesophageal fistula (TEF), in which the esophagus and the trachea are abnormally connected, allowing fluids from the esophagus to get into the airways and interfere with breathing.

What are the signs and symptoms of tracheoesophageal fistula?

Symptoms can include:.
Coughing or choking while nursing or taking a bottle..
Frothing or drooling from the mouth..
Vomiting..
Difficulty breathing while feeding..
Blue-tinged skin while feeding..
An unusually rounded abdomen..
Failure to gain weight..