Esophageal Atresia With or Without Tracheoesophageal Fistula Workup

Updated: May 12, 2017
  • Author: Amulya K Saxena, MD, PhD; Chief Editor: Eugene S Kim, MD, FACS, FAAP  more...
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Workup

Laboratory Studies

In babies with esophageal atresia, samples should be obtained to determine baseline values for the following:

  • Complete blood count (CBC)
  • Electrolyte levels
  • Venous gas concentrations
  • Blood urea nitrogen (BUN) and serum creatinine levels
  • Blood glucose level
  • Serum calcium level
  • Arterial blood gas (ABG) concentrations, as necessary
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Imaging Studies

Ultrasonography

Prenatal ultrasonography may reveal the size of the gastric bubble, polyhydramnios, and VACTERL (vertebral defects, anorectal malformations, cardiovascular defects, tracheoesophageal defects, renal anomalies, and limb deformities) anomalies, all of which may indicate esophageal atresia in the fetus. The sensitivity of prenatal ultrasonography is approximately 40%. A prenatal diagnosis of esophageal atresia may be associated with a worse prognosis.

In infants and neonates, early renal ultrasonography is mandatory and is used to evaluate associated kidney anomalies, ureteral anomalies, or both.

Echocardiography is indicated early in the care of infants with esophageal atresia who have clinical signs of cardiovascular disease. [22] However, a 1-day-old neonate with significant congenital heart disease may have normal findings upon physical examination. Therefore, some argue that echocardiography should be performed in all infants with esophageal atresia. This examination also provides the surgeon with information regarding the side of the aortic arch. A right-side aortic arch is not uncommon in cases of esophageal atresia, and the surgeon should be aware of this finding.

Spinal ultrasonography is a simple test that takes advantage of the neonate's relatively transparent lumber lamina in the assessment of an associated tethered cord. This examination may be performed when the baby is younger than 1 month, though it is not critically important in the early care of the infant.

Radiography

Chest radiography (see the images below) is mandatory and should be performed as soon as possible if esophageal atresia is suspected.

This chest radiograph reveals esophageal atresia a This chest radiograph reveals esophageal atresia and distal tracheoesophageal fistula. Note the Replogle tube in the upper pouch and the GI air below the diaphragm.
This chest radiograph reveals esophageal atresia w This chest radiograph reveals esophageal atresia without tracheoesophageal fistula. Note the absence of gas below the diaphragm.

The value of chest radiography is enhanced if a Replogle tube is in place and if 5-10 mL of air is injected to distend the upper pouch. Great caution should be exercised if liquid contrast material is injected into the proximal pouch. First, to prevent spillage into the airway, only about 1 mL of isotonic water-soluble contrast should be used; a catheter with an end-hole should be employed. Second, if an upper-pouch fistula is present, the contrast material flows directly into the airway. Usually, a contrast-enhanced study is unnecessary.

The heart shadow and size should be assessed. Vertebral and rib anomalies should be assessed. The lung fields should be assessed for possible aspiration pneumonitis and for the rarely associated diaphragmatic hernia or congenital lung lesion.

The presence or absence of gastrointestinal (GI) air below the diaphragm is an important finding. Complete absence of gas in the GI tract denotes the absence of a distal tracheoesophageal fistula (TEF); however, distal fistulae simply occluded by mucous plugs have been rarely reported. In cases of esophageal atresia without fistula, assume that the distance between the ends of the atretic esophagus is too long for early single-stage primary repair. These infants require a delayed repair (see below).

Limb radiography (see the image below) is indicated if the limbs appear abnormal. The possibility of associated radial-ray deformities should be investigated.

This radiograph reveals a radius without a radial This radiograph reveals a radius without a radial ray deformity.

In cases where the distance between the two atretic ends of the esophagus is suspected to be too long for a primary repair, a "gapogram" (see the image below) is useful in assessing that distance.

Contrast material has been administered, and a pro Contrast material has been administered, and a probe has been placed through the gastrostomy in this child with pure esophageal atresia. The air-filled upper pouch can be observed superiorly, with a Replogle tube within it. This gap-o-gram reveals a very wide gap (>5 vertebral bodies), which requires esophageal replacement. This study is a dynamic investigation, one in which the surgeon and radiologist should be present to view the real-time fluoroscopic images.

A gastrostomy is created, and the upper pouch is intubated with a 10-French Replogle tube with radiopaque markings. A small-diameter Bakes dilator is introduced into the gastrostomy and directed superiorly under fluoroscopic guidance into the distal esophageal segment. With gentle but definite force on both the Bakes dilator and the Replogle tube, the two ends are pushed toward each other under fluoroscopic control.

At the point of least separation, an image is obtained, and the distance between the two ends is determined in terms of number of vertebral bodies, which provide an inherent reference for measurement. Generally, a separation distance of two (some say three) vertebral bodies or fewer is usually small enough to permit an anastomosis. If greater distances separate the ends, a delay of weeks to months may be required for the ends to grow closer together, for reassessment with gapograms every 4-6 weeks, or for esophageal replacement or lengthening surgery.

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