Tracheoesophageal Fistula (Congenital)

Incidence of EA + TEF: 1:3500.

EA/TEF in twins is 2.6 times higher than in singletons.

Respiratory distress

Stomach distention, rupture (1%) possible

Dehydration and hypoglycemia

Respiratory status

Congenital heart disease (23%): PDA, VSD, TOF, ASD, right-sided aortic arch

Location of fistula (typically unknown)

Fluid and metabolic status

Prematurity

Low birth weight (common)

Association with vertebral abnormalities (17%), anal and/or duodenal atresia (12%), cardiovascular anomalies (23%), tracheoesophageal fistula, esophageal atresia, renal (16%) and/or radial anomalies, and limb defects (10%) (VACTERL association).

Respiratory compromise is possible from aspiration pneumonitis, tracheomalacia (usually present but clinically significant in 10–20% of cases), gastric distention, prematurity, and congenital heart disease.

Gross classification: (A) EA without TEF (8%); (B) proximal TEF with distal EA (3%); (C) distal TEF with proximal EA (85%); (D) proximal and distal TEF (<1%); (E) TEF without EA or “H”-type TEF (4%). Gross type A is the only type without TEF and the attending risk of aspiration but the most likely to have associated anomalies (up to 65%).

Preop echocardiography is useful for identifying cardiac anomalies and the presence right aortic arch (2.5–5%); if present, left-sided approach is required.

In Gross C TEF, the fistula may be below the carina (11%) or within 1 cm proximal to the carina (22%), making the classic recommendation of positioning the ET tip between the carina and the fistula impossible or difficult. If margin between ET tip and carina is low, inadvertent endobronchial intubation of the compressed right lung may occur due to tube migration, and inadvertent ventilation of the fistula can occur due to tube migration. Repositioning of ET may lead to inadvertent endobronchial or fistula intubation.

Most Gross C type do not have a long esophageal gap.

Some fistula openings may be ≥3 mm with increased risk of stomach insufflation and inadvertent fistula intubation.

Having the ET bevel facing posteriorly reduces the risk of inadvertent intubation in the beginning and subsequent ET position adjustments, if required.

Avoiding high positive pressure ventilation reduces stomach insufflation.

The fistula subtends an angle not unlike those of the mainstem bronchi, not orthogonally as illustrated in many textbooks. This makes it plausible on the one hand to accidentally intubate with the ET, but makes it easy to insert a balloon-tipped Fogarty cath (Plan A, discussed later).

Genetic syndromes associated with EA/TEF include all full trisomies (Down syndrome, Edwards syndrome, Patau syndrome), single gene disorders (CHARGE syndrome, Feingold syndrome, Opitz syndrome, and Fanconi anemia).

Environmental factors implicated include maternal exposure to methimazole, exogenous sex hormones, alcohol, tobacco, diethylstilbestrol, infectious diseases, advanced maternal age, and working in agriculture or horticulture.

An adriamycin-induced EA/TEF rat model facilitates embryologic study of the disease.