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Esophageal Atresia and Tracheoesophageal Fistula

By

William J. Cochran

, MD, Geisinger Clinic

Last full review/revision Apr 2021| Content last modified Apr 2021
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Esophageal atresia is a birth defect Overview of Birth Defects Birth defects, also called congenital anomalies, are physical abnormalities that occur before a baby is born. They are usually obvious within the first year of life. The cause of many birth... read more in which the esophagus narrows or comes to an end. Most newborns with esophageal atresia also have an abnormal connection between the esophagus and the windpipe (trachea) called tracheoesophageal fistula.

The esophagus Throat and Esophagus The throat (pharynx—see also Throat) lies behind and below the mouth. When food and fluids leave the mouth, they pass through the throat. Swallowing of food and fluids begins voluntarily and... read more is the long tubelike organ that connects the mouth to the stomach. In esophageal atresia, the esophagus is narrow or develops as two separate sections that do not connect (atresia). Because of the defect, food and fluids are delayed or prevented from going from the esophagus to the stomach.

The windpipe Overview of the Respiratory System To sustain life, the body must produce sufficient energy. Energy is produced by burning molecules in food, which is done by the process of oxidation (whereby food molecules are combined with... read more (trachea) is the main airway to the lungs. Normally, the esophagus and the trachea are separate. However, in tracheoesophageal fistula, an abnormal connection (fistula) forms between the esophagus and the windpipe. Because of the defect, food and fluids go into the windpipe and lungs.

Esophageal atresia and tracheoesophageal fistula often occur together.

Doctors do not know what causes esophageal atresia and tracheoesophageal fistula, but many children with these birth defects have other abnormalities, such as defects of the spine Overview of Brain and Spinal Cord Birth Defects Birth defects of the brain and spinal cord can occur in early or late fetal development. Typical symptoms include intellectual disability, paralysis, incontinence, or loss of sensation in some... read more , heart Overview of Heart Defects About one in 100 babies is born with a heart defect. Some are severe, but many are not. Defects may involve abnormal formation of the heart's walls or valves or of the blood vessels that enter... read more , kidneys Kidney Defects There are several different birth defects that affect the kidneys (the two organs that filter waste from the blood to make urine). These defects are not usually apparent at the doctor's examination... read more , genitals Genital Birth Defects Defects of the genitals can involve the penis, scrotum, or testes (testicles) in boys and the vagina and labia in girls. Sometimes the genitals are ambiguous, that is, not clearly female or... read more Genital Birth Defects , ears Ear Defects Ears can be missing, deformed, or incompletely developed at birth. (See also Introduction to Birth Defects of the Face, Bones, Joints, and Muscles.) Birth defects of the ear include the following... read more Ear Defects , and limbs Missing or Incompletely Formed Limbs Limbs can be missing, deformed, or incompletely developed at birth. Limbs may form abnormally. For example, bones in the hand and forearm may be missing because of a genetic defect (see Chromosome... read more Missing or Incompletely Formed Limbs and retardation of mental development, physical development, or both.

Atresia and Fistula: Defects in the Esophagus

In esophageal atresia, the esophagus narrows or comes to a blind end. It does not connect with the stomach as it normally should. A tracheoesophageal fistula is an abnormal connection between the esophagus and the trachea (which leads to the lungs).

Atresia and Fistula: Defects in the Esophagus

Symptoms

A newborn with esophageal atresia coughs, chokes, and drools after attempting to swallow while feeding.

A tracheoesophageal fistula is dangerous because it allows swallowed food and saliva to travel through the fistula to the lungs, leading to coughing, choking, difficulty breathing, and possibly aspiration pneumonia Aspiration Pneumonia and Chemical Pneumonitis Aspiration pneumonia is lung infection caused by inhaling mouth secretions, stomach contents, or both. Chemical pneumonitis is lung irritation caused by inhalation of substances irritating or... read more (due to inhaling food or saliva). Food or fluid in the lungs may impair oxygenation of blood, leading to a bluish discoloration of the skin (cyanosis Cyanosis Cyanosis is a bluish discoloration of the skin resulting from an inadequate amount of oxygen in the blood. Cyanosis occurs when oxygen-depleted (deoxygenated) blood, which is bluish rather than... read more ).

Diagnosis

  • Before birth, prenatal ultrasonography

  • After birth, passage of a tube down the esophagus and x-rays

If doctors suspect these defects after birth, they try to pass a tube down the infant's esophagus. If passage of the tube is blocked, they take x-rays to confirm the diagnosis and to locate the problem.

Treatment

  • Surgery

Before surgery to repair the defects can be done, preparations are done to prevent complications such as aspiration pneumonia. First, feedings by mouth are stopped, and a tube is placed in the upper esophagus to continuously suction saliva before it can reach the lungs. Then the infant is fed by vein (intravenously).

Surgery needs to be done soon after birth to establish a normal connection between the esophagus and stomach and to close the fistula.

Some complications may develop after surgery. Leakage and scar tissue at the site where the esophagus was reconnected are the most common problems. Many children have feeding difficulties. About half of children who have feeding difficulties develop gastroesophageal reflux Gastroesophageal Reflux in Children Gastroesophageal reflux is the backward movement of food and acid from the stomach into the esophagus and sometimes into the mouth. Reflux may be caused by the infant’s position during feeding... read more (the backward movement of food and acid from the stomach into the esophagus), so doctors may give drugs that suppress stomach acid to newborns who have esophageal atresia. If the drugs do not control the reflux, a procedure called fundoplication is done. In fundoplication, the surgeon wraps the top of the stomach around the lower end of the esophagus to make that junction tighter and decrease reflux.

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