Management of congenital isolated H-type tracheoesophageal fistula

Congenital H -type tracheoesophageal fistula (TEF) without associated esophageal atresia (EA) is also called isolated TEF or N -type TEF. The fistula is oblique extending from a cephalad opening on the posterior wall of the trachea to a more caudal position on the anterior esophageal wall. H -type TEF is a rare anomaly comprising about 4% of all EA spectrum of anomalies. Characteristically, the fistula is located at a more cephalad level of the trachea than in TEF associated with EA cases. It is near or above the thoracic outlet in many cases. Coughing, choking and cyanosis during feeding are typical neonatal manifestations of the anomaly and are called the "three -Cs". A high index of suspicion is the key for an early diagnosis. It may be delayed in some cases although they almost always have suggestive pulmonary manifestations. The most commonly employed diagnostic tools are esophageal contrast studies and bronchoscopic evaluation. Contrast studies are preferably performed as a tube esophagogram with pullback technique. Bronchoscopy has a higher diagnostic accuracy and also enables catheterization of the fistula before a surgical intervention. The treatment is surgical. Surgery is usually performed by a lateral cervical approach especially if the level of the fistula is at or above the T2 vertebra level. More caudal fistulas are usually approached either by thoracotomy or thoracoscopy. The most common postoperative complications encountered are recurrent laryngeal nerve injury with resultant vocal cord dysfunction and fistula recurrence. The latter should be repaired by repeat surgery. Vocal cord dysfunction may be temporary and a conservative approach can be undertaken.