The esophagus Throat and Esophagus The throat (pharynx) 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 continues automatically... 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 or spinal cord cause a spectrum of neurologic problems; some may be barely noticeable, others may be fatal. Birth defects of the brain and spinal cord can occur in... 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 Birth 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... read more 
, ears Ear Defects Ears can be missing, deformed, or incompletely developed at birth. Birth defects, also called congenital anomalies, are physical abnormalities that occur before a baby is born. "Congenital"... read more 
, and limbs Missing or Incompletely Formed Limbs Limbs can be missing, deformed, or incompletely developed at birth. Birth defects, also called congenital anomalies, are physical abnormalities that occur before a baby is born. "Congenital"... read more 
and retardation of mental development, physical development, or both.
(See also Overview of Digestive Tract Birth Defects Overview of Digestive Tract Birth Defects A birth defect can occur anywhere along the digestive tract—in the esophagus, stomach, small intestine, large intestine, rectum, or anus. The digestive organs may be incompletely developed or... read more .)
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).  |
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 or grayish discoloration of the skin resulting from an inadequate amount of oxygen in the blood. Cyanosis occurs when oxygen-depleted (deoxygenated) blood, which is bluish... read more ).
Diagnosis
Before birth, prenatal ultrasonography
After birth, passage of a tube down the esophagus and x-rays
Doctors may suspect esophageal atresia and tracheoesophageal fistula before birth based on results of prenatal ultrasonography Ultrasonography Prenatal diagnostic testing involves testing the fetus before birth (prenatally) to determine whether the fetus has certain abnormalities, including certain hereditary or spontaneous genetic... read more or after birth based on symptoms.
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 medications that suppress stomach acid to newborns who had esophageal atresia. If the medications 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.
