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Wednesday, May 13, 2009

Tracheoesophageal fistula



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.

A fistula, from the Latin meaning ‘a pipe,’ is an abnormal connection running either between two tubes or between a tube and a surface. In tracheo-esophageal fistula it runs between the trachea and the esophagus. This connection may or may not have a central cavity; if it does, then food within the esophagus may pass into the trachea (and on to the lungs) or alternatively, air in the trachea may cross into the esophagus.


Associations

Babies with TEF or esophageal atresia are unable to feed properly. Once diagnosed, prompt surgery is required to allow the baby to take in food. Many TEF children have few problems after surgery, however a number develop feeding difficulties and chest problems. Some TEF babies are also born with other abnormalities, most commonly those described in VACTERL association - a group of anomalies which often occur together, including heart, kidney and limb deformities.

Classification

Fistulae 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.

Not all types include both esophageal agenesis and tracheoesophageal fistula, but the most common types do.

Gross Vogt Description EA? TEF?
- Type 1 Esophageal agenesis. Very rare, and not included in the classification by Gross. Yes No
Type A Type 2 Proximal and distal esophageal bud--a normal esophagus with a missing mid-segment. Yes No
Type B Type 3A Proximal esophageal termination on the lower trachea with distal esophageal bud. Yes Yes
Type C Type 3B Proximal esophageal atresia (esophagus continuous with the mouth ending in a blind loop superior to the sternal angle) with a distal esophagus arising from the lower trachea or carina. (Most common, up to 90% of cases.) Yes Yes
Type D Type 3C Proximal esophageal termination on the lower trachea or carina with distal esophagus arising from the carina. Yes Yes
Type E (or Type H) - A variant of type D: if the two segments of esophagus communicate, this is sometimes termed an H-type fistula due to its resemblance to the letter H. TEF without EA. No Yes

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

An additional type, "blind upper segment only" has been described, but this type is not usually included in most classifications.

(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.

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