Types of esophageal atresia/tracheoesophageal fistula based on the Gross classification. (Reprinted with permission from Singh et al. [3])
Pathophysiology
For the congenital variant of TE fistula, an incomplete fusion of the tracheoesophageal fold allows for a defect in the tracheoesophageal septum [4]. This defect allows an open communication between the trachea and esophagus. Embryologically, the trachea and the esophagus both develop around 4–6 weeks from the caudal foregut. The longitudinal tracheoesophageal fold then divides the foregut into a ventral laryngotracheal tube and a dorsal esophagus. The tracheoesophageal fold fusion occurs around the 4th–6th week of embryonic development. The location of the incomplete fusion is where the TE fistula forms, hence the variability in presentation of TE fistulas.
There are several syndromes that are predisposed to incomplete fusion of the tracheoesophageal ridges and form TE fistulas. These include trisomy 13, 18, and 21 as well as VACTERL syndrome (Vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, and limb abnormalities) [1, 5, 6]. Other congenital anomalies are noted to have an association with TE fistulas or esophageal atresia. These include children with muscular/skeletal anomalies, kidney or genitourinary issues, gastrointestinal tract issues (imperforate anus, congenital diaphragmatic hernia), or heart deficits (tetralogy of Fallot, ventricular septal defect) [7, 8]. Also notable is that some patients with TE fistulas will also present with laryngeal cleft and may have further dysphagia or aspiration secondary to the cleft [9].
For the acquired variant of TE fistula, presentation can be equally variable as the cause can have a significant impaction on presentation. Typical causes for an acquired TE fistula include malignancy, trauma to the chest and neck, prolonged intubation in conjunction with a nasogastric/orogastric tube in place, infection (tuberculosis is most common), or ruptured diverticula [10–12]. Location is dependent on the area of disease or trauma.
Presentation
Patients typically present at birth or near birth with symptoms, including respiratory distress. The most common TE fistula, one in which the upper esophagus is a blind pouch, fills with food and saliva at first. Once full, liquids cannot pass to the stomach as it is a blind pouch and is regurgitated into the pharynx and aspirated into the airway, causing coughing, choking, and respiratory distress. This may lead to significant desaturations and result in a “blue baby” in moments of distress. Other cases may be more subtle in initial presentation but cause significant respiratory distress when the oral contents are aspirated.
Physical exam and imaging can be particularly helpful in diagnosis of a TE fistula. Passage of a small nasogastric tube into the esophagus in conjunction with an x-ray can determine the distal end of the esophagus which is particularly helpful in cases of proximal esophageal atresia/blind pouch or TE fistula. General x-rays of the chest and abdomen can be helpful in determining air column patterns in patients with concern for esophageal atresia. TE fistula can be further evaluated with cross-sectional imaging, including magnetic resonance imaging (MRI) or computed tomography (CT) [13], which will help clarify the location of the esophageal atresia and TE fistula and the nature of the fistula. CT has disadvantages of radiation exposure, and thus consideration of MRI instead is warranted depending on the clinical scenario. In general, Gastrografin swallow studies should not be performed on patients with suspected TE fistulas as it may predispose patients to chest infections or allergic reactions. While MRI or CT with thin cuts remains the mainstay of anatomic imaging and surgical planning, a diagnostic bronchoscopy and endoscopy helps to localize the TE fistula most accurately. There are some TE fistulas which are challenging to assess within the mucosal folds of the trachea/bronchus or esophagus. In these cases, during bronchoscopy and endoscopy, methylene blue dye can be placed in the esophagus via endoscopy, and the trachea can be visualized via bronchoscopy to see if there is any extravasation of dye within the airway. This helps to confirm the communication as well as identify its location.
Otolaryngologist Approach
History
Typical presentation of a TE fistula occurs shortly after birth, as children with TE fistula present with labored breathing, choking, and coughing. The pregnancy history should be elicited as polyhydramnios is a risk factor for TE fistula. Family history as well as other genetic syndromes such as trisomy 13, 18, and 21 or VACTERL may be picked up prenatally as well, and those patients should be monitored closely for possible TE fistula.
If there is any concern for a possible TE fistula in utero, a prenatal ultrasound and a prenatal MRI can be performed to better understand the area of concern.
Once born, the baby should be carefully assessed for signs of labored breathing, choking, and coughing. If they are unable to swallow their secretions, a nasogastric tube should be carefully placed to see if there is patency of the esophagus.
Exam
A general assessment of the patient and a head and neck exam are performed. From the general assessment, the patient should be observed to see if there are any signs of choking or coughing with their secretions that may represent aspiration or esophageal atresia. A full round abdomen may be a clinical sign of TE fistula as well. In addition, an endoscopic exam can be useful to assess vocal fold function (before any surgical intervention) as well as any other anatomic abnormalities within the upper airway (e.g., laryngeal cleft).
Imaging
Videofluoroscopic image demonstrating fistulous tract from the esophagus to the trachea. (Courtesy of J. Scott McMurray, MD)
Instrumented Assessment
Incomplete passage of a nasogastric tube can be helpful to determine esophageal patency. Definitive assessment with a preoperative diagnostic bronchoscopy and endoscopy is a mainstay of treatment. Methylene blue can be used to aid the assessment when direct visualization is challenging on endoscopy [14].
Management
Management of a TE fistula requires preoperative management, surgical management, and proper postoperative surveillance. Proper preoperative assessment with imaging and bronchoscopy/endoscopy is essential to understand the nature and type of TE fistula as well as to assess for any other concomitant lesions such as laryngeal cleft. Preoperative management of the patient’s general health is paramount as they are more prone to recurrent pneumonias secondary to aspiration events as well as poor nutrition secondary to the nature of the TE fistula. Once their nutritional and respiratory status are stabilized, successful surgical repair can be properly performed.
Operative Approach: Endoscopic and Open Repair of Tracheoesophageal Fistula
Indications
Surgical intervention is indicated for patients with TE fistula as the disorder adversely affects patient quality of life with concern for recurrent pneumonias, inability to tolerate secretions, and poor nutrition.
Endoscopic repair is indicated for smaller or more localized TE fistula and can be done via localized biological glue, laser/cauterization, or a combination of the two. Open repair is reserved for larger TE fistulas, TE fistulas which are difficult to expose endoscopically, or previously failed endoscopic repair.
Key Aspects of the Consent Process
Risks associated with bronchoscopy, endoscopy, and TE fistula repair should be discussed, including injury to the lips, gums, tongue, and teeth, as well as potential for transient or longer-lasting dysgeusia from the bronchoscopy/endoscopy. Furthermore, TE fistula repair can result in damage to the recurrent laryngeal nerves, causing vocal fold paresis or paralysis. This may lead to a poor voice, poor coordination in swallowing, and aspiration. Operative repair of the esophagus may lead to strictures at the area of repair, potentially causing prolonged dysphagia and need for further interventions on the stricture (dilation, additional open repair). Operative repair on the trachea may lead to stenosis or narrowing within the airway and predispose them to airway compromise, further recurrent pneumonias, and need for future surgical interventions on the airway (dilation, additional open repair). Poor healing may result in recurrence of the fistula and subsequent infections, including pneumonia or mediastinitis.
Equipment
A flexible bronchoscope and a gastrointestinal endoscope are used with their individualized endoscopic towers to visualize the areas of concern. If methylene blue is needed, no more than 1 cc can be placed through the biopsy port of the endoscope or bronchoscope during the procedure.
For definitive endoscopic repair, a rigid 3.5 bronchoscope can be used to evaluate the trachea, while an appropriately sized gastrointestinal endoscope is used to visualize the esophagus.
For definitive open repair, a rigid 3.5 bronchoscope can be used to provide endoscopic visualization while the open repair is being performed. A pediatric open head and neck set has all the basic equipment needed for open repair.
Endoscopic Repair Steps
- 1.
Patient positioning. The patient is placed supine, and a bite block is placed to prevent damage to the lips and teeth as well as damage to the endoscopic equipment. Anesthesia can be maintained by an inhalational anesthetic through a nasal approach or by total intravenous anesthesia (TIVA) with spontaneous ventilation and oxygenation via a nasal insufflation technique or via orotracheal intubation if necessary.
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