Tracheoesophageal fistula

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Tracheoesophageal fistula
ICD-10 J95.0, Q39.1-Q39.2
ICD-9 530.84, 750.3
DiseasesDB 30034
eMedicine med/3416 
MeSH D014138

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List of terms related to Tracheoesophageal fistula

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]


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. [1]

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,[2][3] however, various pediatric surgical publications have attempted a classification system comprised of the below specified types.

Type Description
Type C 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.)
Type A Proximal and distal esophageal bud--a normal esophagus with a missing mid-segment.
Type D Proximal esophageal termination on the lower trachea or carina with distal esophagus arising from the carina.
Type H A variant of type D: if the two segments of esophagus communicate, this is termed an H-type fistula due to its resemblance to the letter H.
Type B Proximal esophageal termination on the lower trachea with distal esophageal bud.

The letter codes are usually associated with the system used by Gross,[4] while number codes are usually associated with Vogt.[5]

(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.

References

  1. "Esophageal Atresia and Tracheoesophageal Fistula - February 15, 1999 - American Academy of Family Physicians". Retrieved 2007-11-11.
  2. Spitz L (2007). "Oesophageal atresia". Orphanet journal of rare diseases. 2: 24. doi:10.1186/1750-1172-2-24. PMID 17498283.
  3. Kovesi T, Rubin S (2004). "Long-term complications of congenital esophageal atresia and/or tracheoesophageal fistula". Chest. 126 (3): 915–25. doi:10.1378/chest.126.3.915. PMID 15364774.
  4. Gross, RE. The surgery of infancy and chilhood. Philadelphia , WB Saunders; 1953.
  5. Vogt EC. Congenital esophageal atresia. Am J of Roentgenol. 1929;22:463–465.

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See Also


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