- Tracheoesophageal fistula
Name = PAGENAME
DiseasesDB = 30034
ICD10 = ICD10|J|95|0|j|95, ICD10|Q|39|1|q|38-ICD10|Q|39|2|q|38
ICD9 = ICD9|530.84, ICD9|750.3
eMedicineSubj = med
eMedicineTopic = 3416
MeshID = D014138
A tracheoesophageal fistula (TEF) is an abnormal connection (
fistula) between the esophagusand the trachea. TEF is a common congenitalabnormality, but when occurring late in life is usually the sequela of surgical procedures such as a laryngectomy.
Congenital TEF can arise due to failed fusion of the
tracheoesophageal ridgesduring the third week of embryological development.cite journal |author=Clark DC |title=Esophageal atresia and tracheoesophageal fistula |journal=American family physician |volume=59 |issue=4 |pages=910–6, 919–20 |year=1999 |month=February |pmid=10068713 |doi= |url=http://www.aafp.org/afp/990215ap/910.html]
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-oesophageal fistula it runs between the trachea and the oesophagus. This connection may or may not have a central cavity; if it does, then food within the oesophagus may pass into the trachea (and on to the lungs) or alternatively, air in the trachea may cross into the oesophagus.
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
VACTERLassociation - a group of anomalies which often occur together, including heart, kidney and limb deformities.
Fistulae between the trachea and esophagus in the newborn can be of diverse morphology and anatomical location,cite journal |author=Spitz L |title=Oesophageal atresia |journal=Orphanet journal of rare diseases |volume=2 |issue= |pages=24 |year=2007 |pmid=17498283 |doi=10.1186/1750-1172-2-24] cite journal |author=Kovesi T, Rubin S |title=Long-term complications of congenital esophageal atresia and/or tracheoesophageal fistula |journal=Chest |volume=126 |issue=3 |pages=915–25 |year=2004 |pmid=15364774 |doi=10.1378/chest.126.3.915] 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.
The letter codes are usually associated with the system used by Gross, [Gross, RE. The surgery of infancy and chilhood. Philadelphia , WB Saunders; 1953.] while number codes are usually associated with Vogt. [Vogt EC. Congenital esophageal atresia. Am J of Roentgenol. 1929;22:463–465.]
An additional type, "blind upper segment only" has been described,cite book |author=Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. |title=Robbins and Cotran pathologic basis of disease |publisher=Elsevier Saunders |location=St. Louis, Mo |year=2005 |pages=800 |isbn=0-7216-0187-1 |oclc= |doi= |accessdate=] , 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.)
Tracheoesophageal fistula is suggested in a newborn by copious
salivationassociated with choking, coughing, and cyanosiscoincident with the onset of feeding.
It is surgically corrected, with resection of any fistula and
anastomosisof 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.
* [http://www.tofs.org.uk/ TOFS: The Tracheo Esophageal Support Group]
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