Esophageal Atresia with Tracheoesophageal Fistula

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CHAPTER 5 Esophageal Atresia with Tracheoesophageal Fistula

Richard Ricketts

Step 1: Surgical Anatomy

♦ The proximal esophagus will be of variable length, and the distal tracheoesophageal fistula (TEF) is usually at or slightly above the carina.

♦ The vagus nerve courses along the trachea, tracheoesophageal groove, and the distal esophagus (Fig. 5-1).

♦ The proximal esophagus receives its blood supply from the thyroid arteries, and the distal esophagus from segmental branches directly off the aorta.

Figure 5-1

Step 2: Preoperative Considerations

♦ The diagnosis is frequently established in utero because of associated maternal polyhydramnios.

♦ The neonate may present with coughing, drooling, and respiratory distress shortly after delivery.

♦ Infants with pure esophageal atresia (EA) will be scaphoid because of absence of gas in the gastrointestinal tract.

♦ Neonates with a TEF may become distended, especially if they are placed on positive pressure ventilation.

♦ A babygram radiograph with a tube in the upper pouch establishes the diagnosis of EA or EA-TEF in a vast majority of neonates (Fig. 5-2). Contrast studies are rarely required.

♦ One must evaluate the baby for the VACTERL complex.

Vertebral: Spine films

Anorectal: Physical examination for patent anus

Cardiac: Echocardiogram to evaluate cardiac anatomy as well as identify position of the aortic arch. Surgical approach through the right or the left side of the chest is determined by the side of the aortic arch. In general, the arch is on the left and access for repair is through the right side of the chest.

TE: Plain babygram radiograph with a catheter (bougie or nasogastric [NG]) in upper pouch is sufficient. Many surgeons routinely perform a rigid bronchoscopy as part of the operative procedure before proceeding with the repair to assess for a possible proximal TEF.

Renal: Ultrasound

Limb: Physical examination for radial hypoplasia (Fig. 5-3)

♦ The distance between the two ends of the esophagus can be estimated by placing a blunt tipped bougie (Hurst dilator or small chest tube) into the upper pouch (with mild downward pressure) and taking a chest x-ray; assume that the distal esophagus is at or slightly above the level of the carina. In patients with pure EA who have already had a gastrostomy tube placed, instilling contrast into the stomach during the same maneuver will demonstrate the level of the distal esophagus (Fig. 5-4).

♦ The timing of repair depends on the infant’s size and condition:

Primary repair: Infant has no significant cardiac or pulmonary problems and weighs more than 1000 g.

Staged repair: Divide TEF and repair EA later

• Extremely premature infant

• Severe pneumonia

• Requirement for high-pressure ventilation

• Complex congenital heart disease (CHD) with cardiac decompensation (cyanotic CHD)

Esophageal substitution: Cervical esophagostomy with substitution at a later date

• Extremely long gap (EA with no distal esophagus in chest and upper pouch at the thoracic inlet)

• Failed repair

Figure 5-2

Figure 5-3

Figure 5-4

Step 3: Operative Steps

Anesthetic Considerations

♦ End of the endotracheal tube should be in the midtrachea to reduce likelihood of intubating the distal TEF.

♦ Use low ventilatory pressures to minimize distention of the stomach.

Positioning

♦ The infant is placed in an extreme lateral decubitus position (almost prone) with the side opposite the aortic arch up (Fig. 5-5).

Figure 5-5

Incision and Access

♦ The incision extends from just below the tip of the scapula posteriorly to a point midway between the scapula and vertebral column (Fig. 5-6).

♦ The 4th intercostal space is entered; the serratus anterior muscle is spared.

♦ An extrapleural approach is used. The plane is developed with a freer elevator initially and then with moistened “micro-Kittner” dissectors (folded neuropatties on a mosquito clamp) until a small Finochietto retractor can be placed.

♦ The parietal pleura is rolled off the azygos vein, which is then divided between suture ligatures. The TEF will usually be located at or slightly above the level of the azygos vein.

♦ The TEF, distal esophagus, vagus nerve, and proximal esophagus are then identified, and a determination is made about the feasibility of primary repair.

Figure 5-6

Division of the TEF and Mobilization of the Esophagus

♦ Encircle the distal esophagus near the TEF with a vessel loop, taking care not to injure the proximal bronchi (beware of the right mainstem bronchus going posteriorly) or vagus nerve (Fig. 5-7).

♦ Elevate the esophageal side of the TEF with a vessel loop. Pinch the fistula close with forceps, and have the anesthesiologist inflate the lungs to ensure that the anatomy has been correctly identified and that the bronchus or trachea has not been mistaken for the fistula.

♦ Start a 5-0 Prolene suture on the tracheal side of the TEF, and sequentially divide the TEF and sew the tracheal side closed with the 5-0 Prolene suture using a simple running stitch. Tie the suture, and then run it back and tie it to the original starting point, creating an air-tight tracheal closure.

♦ Mobilize the distal esophagus no more than 2 cm so as not to disrupt too much of the segmental blood supply.

♦ Mobilize the proximal esophagus after placing a stay stitch at its end, up to the thoracic inlet if necessary; the blood supply is intramural and will not be compromised. A combination of sharp and blunt dissection is used, taking care to stay directly on the esophageal wall so as not to enter the membranous portion of the trachea or damage the recurrent laryngeal nerves. This dissection may expose a proximal TEF, which if found is primarily repaired.

Figure 5-7

Anastomosis (Fig. 5-8)

♦ A single-layer, end-to-end anastomosis with fine suture is performed. Two “corner” stitches are placed with the knots on the outside. A center stitch and one stitch between the center and each “corner,” with knots on the inside, are then placed. The sutures are then pulled up and crossed to eliminate tension on the one being tied. Be sure that each stitch is full-thickness and includes mucosa. This completes the “back-row” anastomosis.

♦ For the anterior row of sutures, place a center stitch (including mucosa on each side) with the knot on the outside.

♦ Pull up on this stitch as the anesthesiologist passes down a transanastomotic 6 or 8 French NG feeding tube. Avoid creating a submucosal false channel.

♦ Complete the anterior row of the anastomosis with additional full-thickness stitches with the knots on the outside.

♦ Separate the suture lines on the esophagus and the trachea by interposing some mediastinal soft tissue held in place with 5-0 chromic suture.

Figure 5-8

♦ Place a 10 or 12 French chest tube near, but not touching, the anastomosis (Fig. 5-9). To keep it in the desired location, a 5-0 chromic suture may be used to sew it to the inside of the chest wall. Bring it out through a separate stab wound incision and connect to a 2-cm water seal (not to suction).

♦ Place saline into the chest, and inflate the lungs to ensure that the fistula closure on the tracheal side is complete. Close the chest in the standard fashion.

♦ Secure the transanastomotic feeding tube in place. Do not replace it “blindly” if it comes out.