Tracheoesophageal Fistulas

Tracheoesophageal Fistulas

Jessica Van Beek–King, MD, and James W. Schroeder, Jr, MD, FACS, FAAP

Introduction/Etiology/Epidemiology

•A tracheoesophageal fistula (TEF) is an abnormal connection between the trachea and the esophagus.

—May be congenital or acquired

—No race or sex predilection

•May be associated with esophageal atresia (EA), whereby the esophagus ends in a blind pouch and does not connect to the stomach

•Congenital TEFs

—Incomplete separation of the esophagus from the laryngotracheal tube occurs during embryological development.

—Most cases have an unknown etiologic origin. EA and/or TEF associated with syndromes may have genetic associations. In isolation, EA and/or TEF is more likely multifactorial.

—Incidence ranges from 1 per 2,500 live births to 1 per 4,500 live births.

—There are 5 main types of EA and/or TEF. The first classification system, described by Vogt in 1929, included pure EA, which is extremely rare, as a sixth type. The system was modified by Gross in 1953 to the current 5-type classification. (See Figure 15-1.)

▪Type A: Proximal and distal esophageal bud, normal trachea, no TEF (8% of cases)

▪Type B: Proximal esophagus with TEF terminating at the lower trachea, distal esophageal bud (3% of cases)

▪Type C: Proximal EA, distal esophagus with TEF arising from the distal esophagus or carina (85% of cases)

▪Type D: Proximal esophagus forms a TEF that terminates in the distal trachea, and the distal esophagus arises as TEF from the carina (<1% of cases)

▪Type E (H-type): A variant of type D, whereby the proximal and distal esophagus is in continuity and a TEF is present near the distal tracheal (4% of cases)

•Acquired TEFs

—Nonmalignant

▪Blunt or open trauma, iatrogenic injury, irritation from endotracheal or tracheostomy tube, or cuff causing pressure necrosis

▪Foreign-body ingestion

~The most common foreign body associated with acquired TEF is button batteries.

—Malignant

▪Uncommon in the pediatric population

Figure 15-1. Gross classification of esophageal atresia with and without tracheoesophageal fistula. From Wootten CT, Myer CM. Congenital aerodigestive tract anomalies. In: Johnson JT, Rosen CA, eds. Bailey’s Head and Neck Surgery: Otolaryngology. 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2014: 1306–1327.

Pathophysiology

•The esophagus and trachea both develop from the primitive foregut.

—During weeks 4–6 of gestation, a ventral diverticulum forms that evolves into the trachea.

—A longitudinal fold fuses to form a septum. Posterior deviation of this septum yields incomplete separation, resulting in a TEF.

•Esophageal motility is always affected secondary to abnormal innervation. Dysmotility is most commonly seen in the distal segment.

•The trachea is deficient of cartilage at the diseased segment and has increased width of the trachealis muscle, leading to tracheomalacia.

—Mild to moderate tracheomalacia may yield chronic cough and retained pulmonary secretions. More severe tracheomalacia may lead to respiratory distress that necessitates positive pressure or tracheostomy tube placement. (See Chapter 14, Tracheomalacia, Vascular Rings and Slings, and Bronchomalacia.)

Clinical Features

•Copious oral and nasal secretions

•Episodes of coughing, choking, and cyanosis that worsen with oral feedings

•Abdominal distention secondary to air in the stomach

•H-type fistulas often have a much different presentation, whereby the child has a long-standing history of recurrent aspiration and pneumonias.

•Approximately 50% of congenital TEFs are associated with other anomalies.

—Cardiovascular anomalies are most common (ventricular septal defect, tetralogy of Fallot).

—Abnormalities may be musculoskeletal, anorectal and intestinal, genitourinary, head and neck, mediastinal, or chromosomal.

—Associated conditions occur most commonly with isolated EA and least commonly with TEF without EA.

—Anomalies may be associated with syndromes and/or sequences.

▪Vertebral, anal, cardiac, tracheal, esophageal, renal, and limb (“VACTERL” syndrome)

▪Coloboma of the eye, heart anomaly, choanal atresia, retardation, and genital and ear anomalies (“CHARGE” syndrome); trisomy 13, 14, and 18; and Potter syndrome have also been described.

Differential Diagnosis

•Esophageal stenosis

•Esophageal malignancy

•Pharyngeal pseudodiverticulum

•Aspiration pneumonia and/or pneumonitis

•Laryngopharyngeal reflux

•Zenker diverticulum

Diagnostic Considerations

•Prenatal ultrasonography: EA may initially manifest in utero with poly-hydramnios and absence of a gastric gas bubble. However, these findings only have a 44%–56% predictive value.

•Inability to pass a nasogastric tube (at birth): There is (a) an inability to pass a nasogastric tube beyond 10–12 cm or (b) coiling in the proximal esophageal pouch in TEF and/or EA.

•Chest imaging

—Chest radiography with air contrast allows visualization of the proximal esophageal pouch. Distal TEFs will display air in the stomach with distention (Figure 15-2).

—Contrast-enhanced esophagography or upper gastrointestinal series may be indicated.

▪Helpful in diagnosing H-type TEF, which is often diagnosed later than EA and/or TEF. Air is used instead of barium to avoid contrast material aspiration.

—Tracheobronchoscopy and esophagoscopy

▪Allow direct visualization of the trachea and esophagus for identification of level of TEF and other anomalies

▪Especially important for identifying H-type, acquired nonmalignant, or malignant TEFs

▪Important for identifying secondary airway lesions

▪Repeat endoscopies may be necessary throughout the patient’s life

Management

•Genetic counseling

•Multidisciplinary team management

—Preoperative management

▪Supportive care

▪Monitoring and support of nutrition, oxygenation, and ventilation

▪Suctioning of the proximal esophageal pouch to minimize aspiration of oral secretions

▪Semiprone positioning to avoid reflux from the distal esophagus into the trachea

—Surgical management

▪Urgent but not emergent, unless the infant is in respiratory distress

▪The goal of surgery is to establish reconnection of the esophagus and close the TEF.

~With short-segment EA, the ends of the esophagus may be repaired primarily.

~Native esophageal primary anastomosis is the preferred technique.

~Long-segment EA (>3 cm or 2 vertebral bodies) may require stretching prior to primary anastomosis. If stretching is inadequate, repair may be facilitated by using a colonic interposition graft, gastric pull-up, or jejunal free-flap procedure.

~Stretching is performed over a period of ≤3 months.

~Closure of the tracheal defect is performed primarily.

~With H-type fistulas, the fistula is identified with bronchoscopy. A Fogarty catheter is placed through the fistula to identify the defect in the esophagus. Esophageal and tracheal defects are closed primarily, and the local muscle flap is interposed.

~Endoscopic or open techniques are used, depending on the type and size of the fistula.

—Postoperative management

▪Intensive care unit monitoring is indicated.

▪The neck is flexed to avoid tension on the anastomosis; in some cases, the patient may be paralyzed and sedated for a number of days to further limit mobility of the anastomosis while it is healing.

▪Nasogastric tube feedings may begin after 48 hours, and oral feedings may begin once the infant is able to tolerate secretions.

▪Potential short- and long-term complications include reflux, anastomotic leak, esophageal stenosis or dysmotility, dysphagia, fistula recurrence, scoliosis, deformities of the chest wall, tracheomalacia, tracheal stenosis, persistent cough and wheeze, and Barrett esophagus.

Figure 15-2. Esophageal atresia (type C) with distal fistula in a 2-day-old female neonate with difficulty feeding. Anteroposterior chest radiograph demonstrates a distended esophageal pouch containing a nasogastral tube that could not be passed. Air in the stomach indicates a distal tracheoesopohageal fistula.

Treating Associated Conditions

•Once comorbid or associated conditions are identified, a multidisciplinary team approach should be used to treat these conditions as medically or surgically indicated.

•Esophageal dysmotility

—Swallow therapy may require video swallow or functional endoscopic evaluation of the swallow to determine the safety of oral feedings. Consistency variation of feedings is based on tolerance, aspiration, or penetration.

—A nasogastric or gastrostomy tube may be indicated if the patient is unable to safely take oral nutrition.

—Esophageal dilation for strictures may be indicated.

•Reflux

—Antisecretory therapy

•Tracheomalacia

—Mild to moderate symptoms should be treated with conservative therapy.

▪Humidified air, chest physical therapy, slow and careful feedings, and avoidance of infections

—Continuous positive airway pressure may be used for respiratory distress as a short-term intervention.

—Severe, diffuse tracheomalacia with failure of conservative therapy may necessitate tracheostomy tube placement or other treatment of the tracheomalacia.

•Chronic cough

—See the reflux and tracheomalacia discussion earlier in the chapter.

—Symptom management

Expected Outcomes/Prognosis

•Improved detection and advances in treatment have increased survival to >90%.

•If undetected, the TEF may lead to severe or fatal pulmonary complications.

•Acquired TEFs have higher mortality and morbidity rates.

When to Refer

•Once the diagnosis of EA and/or TEF is established, the patient should immediately be transferred to a facility with pediatric surgery specialists.

When to Admit

•Most diagnoses are established within the first 24 hours after birth, when the neonate is still an inpatient. The child should remain in the hospital or be transferred to a higher-level facility for monitoring and optimization prior to surgical correction.

Prevention

•Genetic counseling may be helpful in nonisolated or syndromic cases where there may be a genetic etiologic origin.

•In isolated EA and/or TEF, no specific gene or environmental factors have been conclusively identified as causative. Therefore, genetic counseling may be less beneficial.

•Acquired EA and/or TEF is preventable by being cautious of foreign- body ingestions, especially button batteries.

Resources for Families

•EA/TEF Esophageal Atresia/Tracheoesophageal Fistula Child and Family Support Connection. rarediseases.org/organizations/ eatef-child-and-family-support-connection-inc

•Family and Parent Resource Center (American Pediatric Surgical Society). www.eapsa.org/parents

Section 2. Developmental Anomalies of the Lung and Pulmonary Vessels

Chapter 16: Pulmonary Hypoplasia

Brian P. O’Sullivan, MD

Chapter 17: Pulmonary Sequestration

T. Bernard Kinane, MD

Chapter 18: Overinflation and Congenital Lobar Emphysema

Kevin Kuriakose, MD, FAAP

Chapter 19: Congenital Pulmonary Airway Malformation

Marianna M. Sockrider, MD, DrPH, FAAP

Chapter 20: Bronchogenic Cysts

Marianna M. Sockrider, MD, DrPH, FAAP