Tracheoesophageal fistula

Overview
A tracheoesophageal fistula (TEF) is an abnormal connection (fistula) between the esophagus and the trachea. TEF is a common congenital abnormality, but when occurring late in life is usually the sequela of surgical procedures such as a laryngectomy.

Causes
Congenital TEF can arise due to failed fusion of the tracheoesophageal ridges during the third week of embryological development.

Associations
It is often found in association with coloboma of the iris, various heart abnormalities, choanal atresia, retardation, genital defects, and ear abnormalities-- an association known as the CHARGE syndrome.

TEF is also part of the VACTERL association.

Classification
Fistula between the trachea and esophagus in the newborn can be of diverse morphology and anatomical location, however, various pediatric surgical publications have attempted a classification system comprised of the below specified types.

The letter codes are usually associated with the system used by Gross, while number codes are usually associated with Vogt.

(For the purposes of this discussion, proximal esophagus indicates normal esophageal tissue arising normally from the pharynx, and distal esophagus indicates normal esophageal tissue emptying into the proximal stomach.)

Clinical presentation
Tracheoesophageal fistula is suggested in a newborn by copious salivation associated with choking, coughing, and cyanosis coincident with the onset of feeding.

Treatment
It is surgically corrected, with resection of any fistula and anastomosis of any discontinuous segments. Surgical repair is associated with complications, including
 * Stricture, due to gastric acid erosion of the shortened esophagus.
 * Leak of contents at the point of anastomosis.
 * Recurrence of fistula.