esophageal atresia

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Esophageal Atresia

 

Definition

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.

Description

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.

Diagnosis

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.

Treatment

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.

Prognosis

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.

Resources

Books

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.

Key terms

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.

atresia

 [ah-tre´zhah]
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.
aortic atresia
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.
 Tricuspid atresia, here displaying a ventricular septal defect and normally related great arteries, the arrows showing the altered flow of blood through the heart. From Dorland's, 2000.
urethral atresia imperforation of the urethra.

e·soph·a·ge·al a·tre·si·a

congenital failure of the full esophageal lumen to develop; often associated with tracheoesophageal fistula.

esophageal atresia

[əsof′əjē′əl, es′ofā′jē·əl]
Etymology: Gk, oisophagos, gullet
an abnormal esophagus that ends in a blind pouch or narrows to a thin cord and thus does not provide a continuous passage to the stomach. It usually occurs as a congenital anomaly. Thoracotomy can affect repair.

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.

esophageal atresia

(isof´əjē´əl ətrē´zhə),
n an abnormal esophagus that ends in a blind pouch or narrows to a thin cord and thus fails to provide a continuous passage to the stomach. It is usually a congenital anomaly.

esophageal

of or pertaining to the esophagus.

esophageal achalasia
esophageal anomalies
very rare; include atresia, duplication, segmental aplasia, esophagorespiratory fistulae, diverticula, epithelial inclusion cysts.
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 is by surgical repair by esophageal anastomosis and division of the fistula.
esophageal distention
may result from acute or chronic obstruction of the esophagus, or from defective innervation. See also megaesophagus.
esophageal duplication
may be tubular and communicate with the effective esophagus, or cystic appearing as a cystic mass close to the functioning esophagus.
esophageal ectasia
esophageal enlargement
clinically visible enlargement as seen in esophageal diverticulum, stenosis, paralysis, cardial obstruction.
esophageal fibrosis
a cause of acquired megaesophagus; usually caused by trauma or spontaneous ulceration.
esophageal groove
see reticular groove.
esophageal groove lesion
includes granuloma, papilloma, foreign body lodgment; cause of obstructive bloat.
esophageal hyperkeratosis
hyperkeratotic thickening of the esophageal mucosa due usually to hypovitaminosis A or chlorinated naphthalene poisoning.
esophageal inflammation
esophageal motility disorders
esophageal neoplasm
very rare except for papilloma and fibropapilloma; causes chronic esophageal obstruction.
esophageal obstruction
acute obstruction is manifested by inability to swallow, regurgitation of saliva, food and water through the nose and much discomfort expressed by retching movements and pawing at the throat. Ruminants develop ruminal tympany. Chronic obstruction shows the same syndrome but with a gradual development and a tendency to develop aspiration pneumonia.
Enlarge picture
Palpating an esophageal obstruction in a cow. By permission from Blowey RW, Weaver AD, Diseases and Disorders of Cattle, Mosby, 1997
esophageal osteosarcoma
occurs in dogs in association with the parasite Spirocerca lupi.
esophageal papilloma
a cause of obstructive bloat.
esophageal paralysis
causes esophageal obstruction.
esophageal patching
see patch graft.
esophageal perforation
causes local cellulitis and compression-obstruction of esophagus.
esophageal pulsion diverticulum
a diverticulum that pushes outwards causing pressure on surrounding organs and tissues.
esophageal segmental aplasia
causes esophageal obstruction in neonates.
esophageal stenosis, esophageal stricture
causes esophageal obstruction; may be partial, permitting passage of liquids.
esophageal tube
see nasogastric tube.
esophageal ulcer
usually associated with pressure necrosis due to prolonged obstruction and injury by a solid foreign body or, rarely equine dysautonomia or Gasterophilus spp. infestation.
esophageal varices
distended veins at the gastric cardia causing dysphagia.