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Esophageal atresia is a serious birth defect in which the esophagus, the long tube that connects the mouth to the stomach, is segmented and closed off at any point. This condition usually occurs with tracheoesophageal fistula, a condition in which the esophagus is improperly attached to the trachea, the nearby tube that connects the nasal area to the lungs. Esophageal atresia occurs in approximately 1 in 4,000 live births.
Failure of an unborn child (fetus) to develop properly results in birth defects. Many of these defects involve organs that do not function, or function only incidentally, before birth, and, as a result, go undetected until the baby is born. In this case, the digestive tract is unnecessary for fetal growth, since all nutrition comes from the mother through the placenta and umbilical cord.
During fetal development, the esophagus and the trachea arise from the same original tissue. Normally, the two tubes would form separately (differentiate); however, in cases of esphageal atresia and tracheoesophageal fistulas, they do not, resulting in various malformed configurations. The most common configuration is the "C" type, in which the upper part of the esophagus abruptly ends in a blind pouch, while the lower part attaches itself to the trachea. This configuration occurs in 85-90% of cases. Esophageal atresia without involvement of the trachea occurs in only 8% of cases.
Causes and symptoms
The cause of esophageal atresia, like that of most birth defects, is unknown.
An infant born with this defect will at first appear all right, swallowing normally. However, the blind pouch will begin to fill with mucus and saliva that would normally pass through the esophagus to the stomach. These secretions back up into the mouth and nasal area, causing the baby to drool excessively. When fed, the baby will also immediately regurgitate what he or she has eaten. Choking and coughing may also occur as the baby breaths in the fluid backing up from the esophagus. Aspiration pneumonia, an infection of the respiratory system caused by inhalation of the contents of the digestive tract, may also develop.
Physicians who suspect esophageal atresia after being presented with the above symptoms diagnose the condition using x-ray imaging or by passing a catheter through the nose and into the esophagus. Esophageal atresia is indicated if the catheter hits an obstruction 4-5 in (10-13 cm) from the nostrils.
Infants with esophageal atresia are unlikely to survive without surgery to reconnect the esophagus. The procedure is done as soon as possible; however, prematurity, the presence of other birth defects, or complications of apiration pneumonia may delay surgery. Once diagnosed, the baby will be fed intraveneously until he or she has recovered sufficiently from the operation. Mucus and saliva will also be continuously removed via a catheter until recovery has occured. When surgery is performed, the esophagus is reconnected and, if neccessary, separated from the trachea. If the two ends of the esophagus are too far apart to be reattached, tissue from the large intestine is used to join them.
Surgery to correct esophageal atresia is usually successful. Post-operative complications may include difficulty swallowing, since the esophagus may not contract efficiently, and gastrointestinal reflux, in which the acidic contents of stomach back up into the lower part of the esophagus, possibly causing ulcers.
Long, John D., and Roy Orlando. "Anatomy and Development and Acquired Anomalies of the Esophagus." In Sleisenger & Fordtran's Gastrointestinal and Liver Disease, edited by Mark Feldman, et al. Philadelphia: W. B. Saunders Co., 1998.
Fetal — Refers to the fetus, also known in the first two months after conception as an embryo.
Fistula — Unnatural connection between two hollow organs or one organ and the outside.
congenital absence or closure of a normal body opening or tubular structure; see also obstruction. adj., adj atret´ic.
anal atresia (atresia a´ni) imperforate anus.
1. congenital absence of the aortic orifice.
2. absence or closure of the aortic orifice, a rare congenital anomaly in which the left ventricle is hypoplastic, so that oxygenated blood passes from the left into the right atrium through a septal defect, and the mixed venous and arterial blood passes from the pulmonary artery to the aorta by way of a patent ductus arteriosus.
aural atresia absence of closure of the auditory canal.
biliary atresia congenital obliteration or hypoplasia of one or more components of the bile ducts, resulting in persistent jaundice and liver damage.
choanal atresia blockage of the posterior nares. When the blockage is bilateral in a newborn, it produces acute respiratory distress because neonates are nose-breathers. Diagnosis is confirmed if a catheter cannot be passed through the nares. Until surgery is done to relieve the obstruction, insertion of an airway may be necessary.
esophageal atresia congenital lack of continuity of the esophagus, commonly accompanied by tracheoesophageal fistula, and characterized by accumulations of mucus in the nasopharynx, gagging, vomiting when fed, cyanosis, and dyspnea. Treatment should begin with suction of the upper esophageal pouch, followed by surgical repair by esophageal anastomosis and division of the fistula as soon as the infant's general condition permits.
follicular atresia (atresia folli´culi) the normal death of the ovarian follicle when unfertilized.
laryngeal atresia congenital lack of the normal opening into the larynx.
mitral atresia congenital obliteration of the mitral orifice; it is associated with hypoplastic left heart syndrome and transposition of great vessels.
prepyloric atresia congenital membranous obstruction of the gastric outlet, characterized by vomiting of gastric contents only. Called also pyloric atresia.
pulmonary atresia congenital severe narrowing or obstruction of the pulmonary orifice, with cardiomegaly, reduced pulmonary vascularity, and right ventricular atrophy. It is usually associated with tetralogy of fallot, transposition of great vessels, or other cardiovascular anomalies.
pyloric atresia prepyloric atresia.
tricuspid atresia absence of the tricuspid orifice, circulation being made possible by an atrial septal defect.
urethral atresia imperforation of the urethra.
congenital failure of the full esophageal lumen to develop; often associated with tracheoesophageal fistula.
e·soph·a·ge·al a·tre·si·a(ĕ-sof'ă-jē'ăl ă-trē'zē-ă)
Neonatal condition in which the proximal end of the esophagus ends in a blind pouch. Food cannot enter the stomach through the esophagus.
e·soph·a·ge·al a·tre·si·a(ĕ-sof'ă-jē'ăl ă-trē'zē-ă)
Neonatal condition in which the proximal end of the esophagus ends in a blind pouch such that food cannot enter the stomach through the esophagus.