Tracheoesophageal fistula
Encyclopedia
A tracheoesophageal fistula (TEF, or TOF; see spelling differences) is an abnormal connection (fistula
Fistula
In medicine, a fistula is an abnormal connection or passageway between two epithelium-lined organs or vessels that normally do not connect. It is generally a disease condition, but a fistula may be surgically created for therapeutic reasons.-Locations:Fistulas can develop in various parts of the...

) between the esophagus
Esophagus
The esophagus is an organ in vertebrates which consists of a muscular tube through which food passes from the pharynx to the stomach. During swallowing, food passes from the mouth through the pharynx into the esophagus and travels via peristalsis to the stomach...

 and the trachea
Vertebrate trachea
In tetrapod anatomy the trachea, or windpipe, is a tube that connects the pharynx or larynx to the lungs, allowing the passage of air. It is lined with pseudostratified ciliated columnar epithelium cells with goblet cells that produce mucus...

. TEF is a common congenital abnormality, but when occurring late in life is usually the sequela of surgical procedures such as a laryngectomy
Laryngectomy
Laryngectomy is the removal of the larynx and separation of the airway from the mouth, nose and esophagus. The laryngectomee breathes through an opening in the neck, a stoma. This procedure is usually performed in cases of laryngeal cancer...

.

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.

TEF can also occur due to pressure necrosis by a tracheostomy tube in apposition to a nasogastric tube (NGT). [Reference: Advanced Surgical Recall , 3rd Ed. By Dr. Lorne H. Blackbourne, pg. 206.]

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. Few children with TEF have problems after surgery, however a number develop feeding difficulties and chest problems. Some babies with TEF 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. 6% of babies with TEF also have a laryngeal cleft
Laryngeal cleft
A laryngeal cleft or laryngotracheoesophageal cleft is a rare congenital abnormality in the posterior laryngo-tracheal wall. It occurs in approximately 1 in 10,000 to 20,000 births...

.

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 based on 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 >-
| Type A
Type 2 Proximal and distal esophageal bud—a normal esophagus with a missing mid-segment. Yes >-
| Type B
Type 3A Proximal esophageal termination on the lower trachea with distal esophageal bud. Yes >-
| Type C
Type 3B Proximal esophageal atresia
Esophageal atresia
Esophageal atresia is a congenital medical condition which affects the alimentary tract. It causes the esophagus to end in a blind-ended pouch rather than connecting normally to the stomach. It comprises a variety of congenital anatomic defects that are caused by an abnormal embryological...

 (esophagus continuous with the mouth ending in a blind loop superior to the sternal angle
Sternal angle
The sternal angle or 'angle of Louis', from the Latin angulus Ludovici is the anterior angle formed by the junction of the manubrium and the body of the sternum in the form of a secondary cartilaginous joint . This is also called the manubriosternal joint or Angle of Louis...

) with a distal esophagus arising from the lower trachea or carina
Carina of trachea
In anatomy, the carina is a cartilaginous ridge within the trachea that runs anteroposteriorly between the two primary bronchi at the site of the tracheal bifurcation at the lower end of the trachea .The mucous membrane of the carina is the most sensitive area of the trachea and larynx...

. (Most common, up to 90% of cases.)
Yes >-
| Type D
Type 3C Proximal esophageal termination on the lower trachea or carina with distal esophagus arising from the carina. 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
Choking
Choking is the mechanical obstruction of the flow of air from the environment into the lungs. Choking prevents breathing, and can be partial or complete, with partial choking allowing some, although inadequate, flow of air into the lungs. Prolonged or complete choking results in asphyxia which...

, coughing, vomiting, and cyanosis
Cyanosis
Cyanosis is the appearance of a blue or purple coloration of the skin or mucous membranes due to the tissues near the skin surface being low on oxygen. The onset of cyanosis is 2.5 g/dL of deoxyhemoglobin. The bluish color is more readily apparent in those with high hemoglobin counts than it is...

 coincident with the onset of feeding. Esophageal atresia and the subsequent inability to swallow typically causes polyhydramnios
Polyhydramnios
Polyhydramnios is a medical condition describing an excess of amniotic fluid in the amniotic sac. It is seen in 0.2 to 1.6% of pregnancies,,...

 in utero.

Treatment

It is surgically corrected, with resection of any fistula and anastomosis
Anastomosis
An anastomosis is the reconnection of two streams that previously branched out, such as blood vessels or leaf veins. The term is used in medicine, biology, mycology and geology....

 of any discontinuous segments. Surgical repair is associated with complications, including
  • Stricture
    Stenosis
    A stenosis is an abnormal narrowing in a blood vessel or other tubular organ or structure.It is also sometimes called a stricture ....

    , due to gastric acid erosion of the shortened esophagus.
  • Leak of contents at the point of anastomosis.
  • Recurrence of fistula.

External links

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