esophageal atresia

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.

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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.

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esophageal atresia

(isof´jē´l trē´zh),
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.

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esophageal

of or pertaining to the esophagus.

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esophageal achalasia

see megaesophagus.

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

see megaesophagus.

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

see esophagitis.

esophageal motility disorders

see megaesophagus.

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.

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.