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.
image

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.

image Vertebral: Spine films
image Anorectal: Physical examination for patent anus
image 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.
image 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.
image Renal: Ultrasound
image 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:

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

• Infant weighs less than 1000 g

image Support with total parenteral nutrition (TPN) or G-tube feeds
image Constant suction on upper pouch

• Short-term cardiac or pulmonary problems (pneumonia) that can be resolved in 2 to 3 weeks
• “Long gap”

image TPN or G-tube feeds and bouginage with suction on upper pouch

image 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)

image Esophageal substitution

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