tracheoesophageal fistula

Tracheoesophageal Fistula 

Definition

Tracheoesophageal fistula (TEF) is commonly a birth defect, with the trachea connected to the esophagus. In most cases, the esophagus is discontinuous, causing immediate feeding difficulties. TEFs may develop in adult life, secondary to the invasion of cancer in the area. In addition, TEFs may be deliberately constructed with surgery to aid talking in a patient who has the larynx removed (a laryngectomy).

Description

The trachea, or windpipe, carries air to the lungs. The esophagus carries food to the stomach. Sometimes during development, these two tubes do not separate completely, but remain connected by a short passage. When this happens, air enters the gastrointestinal system, causing the bowels to distend, and mucus is breathed into the lungs causing aspiration pneumonia and breathing problems.

Most tracheoesophageal fistulas are diagnosed when a child is born. There are three types. In 85-90% of tracheoesophageal fistulas, the top part of the esophagus ends in a blind sac, and the lower part inserts into the trachea. In the second type, the upper part of the esophagus is connected directly to the trachea, while the lower part ends in a pouch. In a rare type of fistula called an H type, both the esophagus and trachea are complete, but they are connected. This is the most difficult type of tracheoesophageal fistula to diagnose, because both eating and breathing are possible.

Causes and symptoms

Tracheoesophageal fistulas arise as a developmental abnormality. At birth, the infant has difficulty swallowing. Eating produces severe coughing spells that interfere with breathing. Aspiration pneumonia can develop from fluid breathed into the lungs.

Small H type fistulas may go undiagnosed until later in life. Symptoms of an H type fistula include frequent pulmonary infections and bouts of abdominal bloating.

Diagnosis

Diagnosis that the esophagus is interrupted is confirmed by the inability to insert a nasogastric suction tube into the stomach. The exact type and location of the fistula can be determined using a radiopaque catheter, which allows pictures to be taken of the esophagus. X rays may show air in the bowels. Endoscopy often fails to located the fistula if it is small.

Treatment

Babies with all but H type fistulas are unlikely to survive without surgical separation and repair of the trachea and the esophagus. Surgery cannot always be performed immediately because of prematurity, the presence of other birth defects, or complications from aspiration pneumonia. It is usually done at a hospital that has special facilities for treating seriously ill newborns.

While awaiting surgery, the infant's condition is stabilized. Preoperative care concentrates on avoiding aspiration pneumonia and includes:

• elevating the head to avoid reflux and aspiration of the stomach contents

• using a suction catheter to continuously removed mucus and saliva that could be inhaled
• when necessary, placement of a gastrostomy tube
• withholding feeding by mouth

When surgery is performed, the esophagus is reconnected to make it continuous and separate from the trachea. If the two ends of the esophagus are too far apart to be reattached, a piece of tissue from the large intestine is used to join the parts.

Prognosis

Infants who have tracheoesophageal fistula often have other birth defects that affect their recovery. Even when the esophagus is successfully separated and reattached, many infants have difficulty swallowing, because the contractility of the esophagus is impaired. Infants may also have problems with gastroesophageal reflux, in which the acidic contents of the stomach back up into the bottom of the esophagus and cause ulcers and scarring.

Prevention

Tracheoesophageal fistulas are not preventable birth defects.

Resources

Books

Berktow, Robert, editor. "Gastrointestinal Defects." In The Merck Manual of Diagnosis and Therapy. Rahway, NJ: Merck Research Laboratories, 2004.

Key terms

Endoscopy — A procedure in which an instrument containing a camera and a light source is inserted into the gastrointestinal tract so that the physician can visually inspect the gastrointestinal system.

Gastrostomy tube — Stomach tube for feeding.

Laryngectomy — Surgical removal of the larynx to treat cancer.

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fistula /fis·tu·la/ (fis´tu-lah) pl. fistulas, fis´tulae   [L.] an abnormal passage between two internal organs or from an internal organ to the body surface.

Various types of fistulae, designated according to site or to the organs with which they communicate. (A), Genitourinary fistulae; (B), anal fistulae.

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anal fistula  one from the anus to the skin, sometimes communicating with the rectum.

arteriovenous fistula 

1. one between an artery and a vein.

2. a surgically created arteriovenous connection that provides a site of access for hemodialysis tubing.

blind fistula  one open at one end only, opening on the skin (external blind f.) or on an internal mucous surface (internal blind f.) .

branchial fistula  a persistent pharyngeal groove (branchial cleft).

cerebrospinal fluid fistula  one between the subarachnoid space and a body cavity, with leakage of cerebrospinal fluid, usually as otorrhea or rhinorrhea.

colonic fistula  one connecting the colon with the body surface or another organ.

craniosinus fistula  one between the cerebral space and one of the sinuses, permitting escape of cerebrospinal fluid into the nose.

enterovesical fistula  one connecting the urinary bladder with some part of the intestines.

fecal fistula  a colonic fistula that discharges feces on the body surface.

gastric fistula  one communicating with the stomach, either pathologically or surgically created through the abdominal wall.

genitourinary fistula  one between two organs of the urogenital system or between one of those organs and some other system.

incomplete fistula  blind f.

intestinal fistula  one communicating with the intestine; sometimes surgically created through the abdominal wall.

perilymph fistula  rupture of the round window with leakage of perilymph into the middle ear, causing sensorineural hearing loss.

pulmonary arteriovenous fistula  a congenital fistula between the pulmonary arterial and venous systems, so that unoxygenated blood enters the systemic circulation.

salivary fistula  one communicating with a salivary duct.

tracheoesophageal fistula  one connecting the trachea and esophagus, either pathologically or created surgically to restore speech after laryngectomy.

umbilical fistula  one communicating with the colon or the urachus at the umbilicus.

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tracheoesophageal fistula

[trā′kē·ō·ē′səfā′jē·əl]

Etymology: Gk, tracheia + oisophagos, gullet

a congenital malformation in which there is an abnormal tubelike passage between the trachea and the esophagus. Also spelled tracheooesophageal fistula.

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tracheoesophageal

pertaining to or arising from the trachea and the esophagus.

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tracheoesophageal fistula

a communication between the trachea and the esophagus; can occur during fetal life due to faulty separation of the laryngotracheal groove from the foregut; often associated with esophageal stenosis or atresia, aspiration pneumonia.

tracheoesophageal ridge

primordium of the embryonic tracheoesophageal septum.

tracheoesophageal septum

the partition which develops by fusion of the tracheoesophageal ridges; completes the separation of the trachea and esophagus in the developing embryo.

tracheoesophageal stripe sign

in x-rays, the dorsal wall of the trachea and the adjacent ventral wall of the esophagus may be visualized because of air in the esophagus.

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tracheoesophageal fistula

Neonatology A congenital communication between the lower esophagus and trachea, often complicated by aspiration into the lungs, causing pneumonia, choking and possibly death; TEFs are usually detected after birth when feeding is attempted and the infant coughs, chokes and becomes cyanotic; they are a surgical emergency, requiring immediate repair to prevent permanent damage. See Esophageal atresia.