Tracheoesophageal Fistula Repair

Tracheoesophageal fistula (TEF) repair is performed for one of the most common congenital esophageal anomalies, occurring in approximately 1 in 3,000–4,500 live births. The Gross classification defines five anatomic patterns based on the relationship between esophageal atresia (EA) and the fistula. Type C — proximal esophageal atresia with a distal TEF arising from the trachea near the carina — accounts for approximately 84–85% of cases and is the prototypical lesion. Type A is pure esophageal atresia without a fistula (about 7–10%); Type B is proximal atresia with a proximal fistula (<1%); Type D is proximal atresia with both proximal and distal fistulas (rare); and Type E (or 'H-type') is an isolated TEF without atresia (about 4%).

All patients with EA/TEF require operative repair. Timing depends on patient stability and associated anomalies. Stable, term infants undergo definitive repair within the first 24–72 hours of life. Premature, septic, or unstable infants may have temporary gastrostomy and delayed primary repair. Long-gap esophageal atresia (typically defined as a gap of >2–3 vertebral bodies between the proximal and distal pouches) may require staged repair, traction lengthening (Foker or internal traction), or esophageal replacement.

Diagnosis is suspected at delivery when an attempt to pass a nasogastric or orogastric tube meets resistance at approximately 10–15 cm. The diagnosis is confirmed by a chest and abdominal radiograph showing the coiled tube in the proximal pouch. The presence of air in the stomach and bowel on the same film implies a distal TEF (Type C); the absence of bowel gas implies pure atresia (Type A). Contrast studies are not routinely required for Type C; the H-type fistula (Type E) often requires a tube esophagogram or bronchoscopy for diagnosis because the anatomy is otherwise normal on plain film.

Preoperative workup screens for the VACTERL association — Vertebral, Anorectal, Cardiac, TracheoEsophageal, Renal, and Limb anomalies — which is present in 20–30% of EA/TEF patients. Required tests include: echocardiogram (to identify cardiac anomalies AND to determine the side of the aortic arch, which determines the side of thoracotomy — a right aortic arch occurs in approximately 2.5% of cases and mandates a left thoracotomy because operating from the right would require crossing the arch); renal ultrasound (urinary tract anomalies in ~14%); spinal ultrasound or radiographs (vertebral anomalies); and limb radiographs as needed. Anorectal anomalies are identified by physical examination.

Preoperative care includes upright positioning to reduce gastroesophageal reflux through the fistula, continuous suction of the proximal pouch (via a Replogle tube), broad-spectrum antibiotics, and avoidance of bag-mask ventilation when possible (which inflates the stomach through the distal fistula and worsens respiratory mechanics). Bronchoscopy at induction of anesthesia is performed at many centers to characterize the fistula and detect tracheomalacia.

The standard approach for Gross Type C is right posterolateral thoracotomy via a fourth or fifth intercostal space incision. The thoracoscopic approach — described by Rothenberg and colleagues and now standard at many high-volume centers — uses three to four ports and provides similar exposure with markedly better cosmesis and less musculoskeletal sequelae. Both approaches follow the same operative principles.

Exposure begins with an extrapleural approach (the parietal pleura is reflected off the chest wall, preserving the pleural space for re-expansion of the lung if a leak develops; some surgeons use a transpleural approach instead). The azygos vein is identified and ligated to expose the underlying esophagus and trachea. (Some thoracoscopic approaches preserve the azygos vein when feasible.) The distal fistula at the membranous trachea is identified, ligated, and divided sharply, with care taken not to narrow the trachea. The tracheal end is closed with interrupted fine absorbable suture and a small leak test performed.

The proximal esophageal pouch is then mobilized to allow tension-free anastomosis. Gentle dissection up into the thoracic inlet is permitted; the proximal blood supply is segmental from the inferior thyroid arteries and is robust. The distal esophagus has a more tenuous blood supply (from intercostal and esophageal branches) and should not be mobilized extensively. The proximal pouch is opened, and an end-to-end esophageal anastomosis is created with single-layer interrupted absorbable suture (typically 5-0 PDS or Vicryl). A small transanastomotic feeding tube may be left across the anastomosis. A chest tube is placed and the wound is closed.

The infant is kept intubated and ventilated for at least 24–48 hours. Trophic feeds via the transanastomotic tube are started early. An esophagram is typically performed at postoperative day 5–7 to assess the anastomosis before initiating oral feeds; some centers proceed to oral feeds without routine contrast studies if the clinical course is uncomplicated. The chest tube is removed once the contrast study confirms no leak and drainage decreases.

Long-term, all EA/TEF patients require follow-up for the high incidence of associated gastroesophageal reflux (often requiring fundoplication in 15–25%), esophageal stricture at the anastomosis (very common — see below), tracheomalacia (15% with about 40% requiring intervention), and feeding difficulties. Lifelong surveillance is recommended given the small but real risk of late esophageal complications including dysplasia and (rare) carcinoma.

Anastomotic leak is the principal early complication. Rates range from 7–16% in modern series (lower with thoracoscopic approach in experienced hands — approximately 7.6% in the Holcomb multi-institutional series, vs 10–21% historical thoracotomy rates). Most leaks are contained and managed nonoperatively with continued chest drainage, antibiotics, NPO, and enteral feeds via the transanastomotic tube. Free leaks with mediastinitis or sepsis require reoperation.

Anastomotic stricture is the most common late complication, occurring in approximately 18–35% of patients depending on technique and definition. Symptoms include feeding intolerance, dysphagia, choking spells, and recurrent pneumonia. Diagnosis is by esophagram; management is with sequential dilations (balloon or bougie). Severe or refractory strictures may require resection and re-anastomosis.

Recurrent TEF — recurrence of communication between the trachea and the esophagus at or near the prior fistula site — occurs in 2–5% of patients, usually within the first year. Diagnosis is by tube esophagogram or bronchoscopy. Management is reoperation with re-division and interposition of healthy tissue (typically intercostal muscle or pericardium).

Tracheomalacia from the long-standing pressure of the dilated proximal pouch on the membranous trachea presents with stridor, expiratory wheezing, 'TEF cough,' and apneic spells. Mild cases resolve with growth; severe cases may require aortopexy or tracheal stenting.

On the oral boards, examiners commonly test: the Gross classification with the relative frequencies (Type C accounts for 85%); the diagnostic triad of inability to pass an NG tube, coiled NG on plain film, and air in the stomach implying a distal fistula; the VACTERL workup with the specific role of echocardiography to identify a right aortic arch (which mandates a left thoracotomy); the principles of operative repair (extrapleural approach, divide and close the fistula, primary esophageal anastomosis); and the recognition and management of the three classic complications — leak, stricture, and recurrent fistula.